Sample records for abdominal cystic masses

  1. Giant mesenteric cystic lymphangioma presenting with abdominal pain and masquerading as a gynecologic malignancy

    PubMed Central

    Maa, John; Wa, Christianne; Jaigirdir, Adnan; Cho, Soo-Jin; Corvera, Carlos U

    2009-01-01

    Lymphangiomas are congenital malformations of the lymphatic system that account for about 5% of all benign tumors in infants and children.1 The most common sites are the neck and axilla, which account for 95% of cases.2 Abdominal cystic lymphangiomas are quite rare, and can arise from either the retroperitoneum, gastrointestinal tract, or the mesentery of the abdominal viscera.3 The presenting symptoms are painless abdominal distension, a palpable mass, or secondary complications in the abdomen such as intestinal obstruction, volvulus, intestinal infarction, or bleeding.4 Typically diagnosed during childhood, these tumors prompt surgical intervention. We describe an atypical case of an abdominal cystic lymphangioma, which did not manifest until adulthood, with atypical symptoms of a rapidly expanding and symptomatic mass. PMID:21139927

  2. An unusual abdominal cystic mass in the round ligament of the uterus: a case report.

    PubMed

    Açmaz, Gökhan; Aksoy, Hüseyin; Aksoy, Ülkü; Özyurt, Sezin; Esin, Erkan; Gökahmetoğlu, Günhan

    2014-10-01

    Cystic tumor of the round ligament is extremely rare. To our knowledge, this is the first case reported in the literature of a round ligament giant serous cystadenoma. A 17-year-old adolescent who presented with massive abdominal swelling and was later identified as a serous cystadenomas of the round ligament with a size of approximately 50 cm. A multidisciplinary team including gynecologist, surgeon, and urologist performed the surgical cyst excision and she has recovered well without any complications. Round ligament serous cystadenomas could grow to any size when there is a diagnosis. In addition, even in unusual locations, round ligament serous cysts should be kept in the differential diagnosis of abdominal masses. Copyright © 2014 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  3. Abdominal Cystic Echinococcosis Treated with Albendazole. A Pediatric Cohort Study.

    PubMed

    Moroni, Samanta; Moscatelli, Guillermo; Bournissen, Facundo García; González, Nicolás; Ballering, Griselda; Freilij, Héctor; Salgueiro, Fabián; Altcheh, Jaime

    2016-01-01

    Cystic echinococcosis is endemic in Argentina. The standard pharmacological treatment for the disease is albendazole, but surgery is a common alternative. Even though primary infection occurs mainly in the pediatric population, the optimal therapeutic option in pediatrics is not clearly defined and few pediatric cohorts with cystic echinococcosis treated with albendazole have been described to date. To describe therapeutic response to albendazole in a cohort of pediatric patients with abdominal cystic echinococcosis. Patients (0-18 years old) with abdominal cystic echinococcosis who were treated with albendazole between January 1998 and August 2013. Diagnosis of abdominal cystic echinococcosis was made by ultrasound. All patients received albendazole, 10-15 mg/kg/day. Epidemiological data, symptoms, number, location and outcome of the cysts, serology and treatment received were analyzed. The parameter used to assess treatment response was cyst changes evaluated by ultrasound follow up using the WHO-IWGE classification. A total of 28 patients (with 46 abdominal cysts) were included in the cohort. Mean age at enrolment was 9.4 years and mean duration of follow-up, 23.8 months. All patients resided in rural areas and had had contact with dogs. The asymptomatic form of the disease was the most common presentation. All patients received albendazole (mean duration: 142.5 days), with low incidence of adverse events. Albendazole had a positive effect on most of the cysts. Surgery was performed in 13 patients. Treatment with albendazole for uncomplicated cystic echinococcosis cysts is safe and effective, and can potentially reduce the need for surgical intervention.

  4. Multifocal hepatic cystic mass as first manifestation of metastatic spinal hemangiopericytoma

    PubMed Central

    Balibrea, José M.; Rovira-Argelagués, Montserrat; Otero-Piñeiro, Ana M.; Julián, Juan F.; Carrato, Cristina; Navinés, Jordi; Sánchez, M. Carmen; Fernández-Llamazares, Jaime

    2012-01-01

    INTRODUCTION Hemangiopericytomas (HPCs) are rare vascular tumors with a high malignant potential. Hepatic metastases from HPC are very infrequent and usually show a distinctive solid aspect with a surrounding pseudocapsule. PRESENTATION OF CASE A 37-year-old man with a previous medical history of recurrent spinal hemangiopericytoma with a 9 cm × 7 cm cystic hepatic mass detected on follow-up. Contrast enhanced US and MRI confirmed the presence the lesion showing mixed (solid and cystic) content. Parasitic and viral serology plus serum tumoral markers (CEA, ca 19.9, ca 125, AFP) tests, upper and lower endoscopy and general laboratory tests were normal and extended left lobectomy was performed. Histopathologic study confirmed the diagnosis of multifocal metastasic hemangiopericytoma with moderate CD-34, CD-99 and Bcl-2 positivity after immunohistochemical staining. After 1-year follow-up the patient does not present any evidence of abdominal recurrence but a skull base recurrence has been detected. DISCUSSION Liver metastasis from spinal HPC are uncommon and do not have cystic appearance so radiologic diagnosis can be challenging. In spite of the presence of previously diagnosed HPC context, the presence of a liver cystic mass in a young patient makes necessary to discard a number much more frequent benign and malignant diagnosis before metastatic disease can be confirmed. CONCLUSION The presence of a cystic hepatic mass makes it mandatory to rule out a number neoplasms other than metastasic HPC before a definitive diagnosis is made. In addition to local radiotherapy and antiangiogenic agents, surgery can be useful to treat liver dissemination. PMID:23103627

  5. A Case Report of Cystic Pheochromocytoma.

    PubMed

    Junejo, Shoaib Z; Tuli, Sandeep; Heimann, David M; Sachmechi, Issac; Reich, David

    2017-07-25

    BACKGROUND Pheochromocytoma is a rare catecholamine-producing tumor with an estimated incidence of less than 0.1% in the global population. We present a case of cystic pheochromocytoma that was diagnosed as an incidental finding. The patient presented with abdominal pain and had a history of hypertension. CASE REPORT A 64-year-old man with hypertension presented with a clinical history of intermittent abdominal pain for one year. He denied sweating, palpitations, headache or back pain. He was found to have an elevated blood pressure of 170/90 and no palpable abdominal mass. Contrast-enhanced computed tomography (CT) imaging of the abdomen and pelvis were performed that showed cystic mass measuring 9 cm in diameter arising from the left adrenal gland with contrast-enhancing mural nodules. Magnetic resonance imaging (MRI) confirmed the cystic nature of the mass. Laboratory analysis showed an elevated plasma normetanephrine (NMN) of 1,087 pg/ml and metanephrine (MN) of 372 pg/ml; 24-hour urine showed elevated levels of NMN and MN, 3,002 mg/24 h and 1,596 mg/24 h, respectively. Given the laboratory and radiologic findings, a diagnosis of cystic pheochromocytoma was made. After controlling blood pressure with the alpha-blocker, doxazosin, the patient was hydrated and scheduled for an elective adrenalectomy. The histopathology of the excised adrenal gland was consistent with a cystic pheochromocytoma. CONCLUSIONS Cystic pheochromocytoma is a very rare tumor that may present without symptoms. The clinical course of cystic pheochromocytoma is similar to that of solid pheochromocytoma. Early surgical intervention is recommended, following blood pressure control with an alpha-blocker, and adequate hydration.

  6. An unusual cause of paediatric abdominal pain: Mesenteric masses accompanied with volvulus.

    PubMed

    Yang, Chao; Wang, Shan; Zhang, Jun; Kong, Xiang Ru; Zhao, Zhenzhen; Li, Chang Chun

    2016-07-01

    Volvulus caused by mesenteric masses is rare and may result in serious consequences. This study aimed to better characterize volvulus caused by mesenteric masses in children. A retrospective study was conducted in 24 patients who underwent surgical treatment between January 1994 and January 2014 in one single institution. There were 10 boys and 14 girls. The most frequent findings were abdominal pain (100%), emesis (91.7%) and nausea (83.3%). Physical examination showed positive ileus signs in majority cases, and palpable mass was found in half of the patients. Ultrasound and CT scans revealed mesenteric masses in 21 and 24 patients, and 'whirlpool sign' was observed in 19 and 22 patients, respectively. Emergency laparotomy was performed in all patients. Histological examination revealed that 18 cystic masses were lymphangioma, 5 solid cases were lipoma and the remaining one was lipoblastoma. The postoperative course was uneventful in 22 patients, and postoperative obstruction and incision infection occurred in 2 patients. There was no evidence of recurrence at follow-up. Volvulus caused by mesenteric masses is a rare but potentially life-threatening cause of abdominal pain, which should be considered in the differential diagnosis of paediatric acute abdominal pain.

  7. Large Omental Cystic Lymphangioma Masquerading as Mucinous Ovarian Neoplasia in an 8-Year-Old Premenarchal Girl: The Findings from Diagnostic Imaging and Laparoscopic-Assisted Excision.

    PubMed

    Takeda, Akihiro; Ito, Hiroaki; Nakamura, Hiromi

    2017-12-01

    Omental cystic lymphangioma is an extremely rare abdominal mass caused by congenital malformation. An 8-year-old premenarchal girl reported abdominal pain. Diagnostic imaging revealed a large multicystic mass measuring 22 cm in diameter, which occupied the entire abdominal cavity with ascites. Emergency laparoscopy revealed a ruptured large cystic mass originating from the greater omentum; this was followed by successful laparoscopic-assisted excision. The pathological diagnosis was omental cystic lymphangioma. The present findings show that omental cystic lymphangioma masquerading as mucinous ovarian neoplasia was a rare cause of acute abdominal events in a young girl. The present case shows that minimally invasive surgery can be a feasible option, which might achieve a favorable outcome in a young patient with an omental cystic lymphangioma. Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  8. Congenital cystic neck masses: embryology and imaging appearances, with clinicopathological correlation.

    PubMed

    Gaddikeri, Santhosh; Vattoth, Surjith; Gaddikeri, Ramya S; Stuart, Royal; Harrison, Keith; Young, Daniel; Bhargava, Puneet

    2014-01-01

    Congenital cystic masses of the neck are uncommon and can present in any age group. Diagnosis of these lesions can be sometimes challenging. Many of these have characteristic locations and imaging findings. The most common of all congenital cystic neck masses is the thyroglossal duct cyst. The other congenital cystic neck masses are branchial cleft cyst, cystic hygroma (lymphangioma), cervical thymic and bronchogenic cysts, and the floor of the mouth lesions including dermoid and epidermoid cysts. In this review, we illustrate the common congenital cystic neck masses including embryology, clinical findings, imaging features, and histopathological findings. Copyright © 2014 Elsevier, Inc. All rights reserved.

  9. Cystic mediastinal masses and the role of MRI.

    PubMed

    Madan, Rachna; Ratanaprasatporn, Lisa; Ratanaprasatporn, Linda; Carter, Brett W; Ackman, Jeanne B

    2017-12-27

    While some cystic masses can be definitively diagnosed on CT, others remain indeterminate. Because of its intrinsic superior soft tissue resolution, MR is an important tool in the evaluation of select mediastinal masses that are incompletely characterized on CT. This review describes how non-vascular MR provides greater diagnostic precision in the evaluation of indeterminate cystic mediastinal masses on CT. It also emphasizes key MR pulse sequences for optimal evaluation of problematic mediastinal masses. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. Clinics in diagnostic imaging (133). Retained placenta from an intra-abdominal pregnancy.

    PubMed

    Win, T; Tang, P H; Lim, T Y

    2011-01-01

    A 29-year-old Indonesian woman presented with abdominal pain seven months after an intra-abdominal pregnancy. Ultrasonography revealed a cystic mass in the pelvis and magnetic resonance imaging showed an umbilical stump within it, indicating a retained placenta. This was removed surgically, and on histology, an infarcted placenta was confirmed.

  11. Primary omental gastrointestinal stromal tumour (GIST) presenting with a large abdominal mass and spontaneous haemoperitoneum.

    PubMed

    Seow-En, Isaac; Seow-Choen, Francis; Lim, Tony Kiat Hon; Leow, Wei Qiang

    2014-11-03

    A 60-year-old Indonesian woman presented with a 9-day history of increasing abdominal distension, pain and tiredness. Physical examination revealed significant pallor with a palpable mass in the abdomen. CT of the abdomen reported a 22 cm complex mass in the peritoneal cavity with free intra-abdominal fluid. Laboratory results showed anaemia with a raised serum CA 125 level. At laparotomy a large haemorrhagic tumour with blood filled cystic cavities was found attached to both greater omentum and the transverse mesocolon with 2.2 L of blood in the peritoneal cavity. There was no invasion of any part of the stomach or intestines and there were no metastases seen. Histopathology of the resected specimen was consistent with that of a gastrointestinal stromal tumour arising from the omentum. Immunohistochemical studies revealed the tumour to be strongly positive for discovered on GIST-1 (DOG1) but negative for both CD117 and CD34. Platelet-derived growth factor receptor α (PDGFRA) exon 18 mutation D842V was detected. 2014 BMJ Publishing Group Ltd.

  12. Twisted intra-abdominal cyst in a neonate: a surprise revelation.

    PubMed

    Mohamed, Ferzine; Telugu, Ramesh Babu; Karl, Immanuel Sampath

    2017-08-08

    We, herein, present a male neonate with an antenatally detected intra-abdominal cyst who presented at 18 days of life at which time, the ultrasound revealed a 5×4 cm cyst. Since he was asymptomatic, we planned to repeat the ultrasound a month later and operate if the cyst showed no regression. However, a week later, he presented with an acute abdomen, irritable cry and a repeat ultrasound showing a larger (8×6 cm) cystic mass with debris within. He was taken up for an emergency laparotomy. Intraoperatively, the cyst was found arising from the left lateral abdominal wall free from all structures with a twisted pedicle. Histopathology surprisingly revealed seminiferous tubules within the cyst wall with the vas deferens, thus confirming the diagnosis of a torsion of intra-abdominal testis. Hence, we emphasise the importance of examining for an undescended testis when dealing with a male neonate presenting with a cystic intra-abdominal mass. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  13. Laparoscopic Removal of a Large Ovarian Mass Utilizing Planned Trocar Puncture

    PubMed Central

    2012-01-01

    Background: Large cystic ovarian masses pose technical challenges to the laparoscopic surgeon. Removing large, potentially malignant specimens must be done with care to avoid the leakage of cyst fluid into the abdominal cavity. Case: We present the case of a large ovarian cystic mass treated laparoscopically with intentional trocar puncture of the mass to drain and remove the mass. Discussion: Large cystic ovarian masses can be removed laparoscopically with intentional trocar puncture of the mass to facilitate removal without leakage of cyst fluid. PMID:22906344

  14. Prenatal diagnosis of intra-abdominal cystic lesions by fetal ultrasonography: diagnostic agreement between prenatal and postnatal diagnosis.

    PubMed

    Marchitelli, Giulia; Stirnemann, Julien; Acanfora, Marta Maddalena; Rousseau, Veronique; Salomon, Laurent J; Ville, Yves

    2015-09-01

    The aim of this study was to assess the diagnostic agreement between the prenatal diagnosis of intra-abdominal cystic lesions made by ultrasound examination and the postnatal diagnosis. We reviewed all consecutive cases referred for an anechoic abdominal cyst from 2009 to 2013. Prenatal ultrasound diagnosis was compared with postnatal diagnosis. Prenatal diagnosis was defined as 'correct' if a specific prenatal diagnosis or one of the possible diagnoses was confirmed postnatally, as 'not confirmed' if the postnatal examination revealed no abnormalities and as 'incorrect' if the postnatal diagnosis was different from those suggested prenatally. Seventy-three cases were included, and prenatal diagnoses were made at a median gestational age of 27 weeks (range: 13-36). Correct diagnoses were made in 66 cases (90.4%), including four in which the lesion resolved spontaneously in utero; two diagnoses were 'not confirmed' postnatally, and one was incorrect (a prenatal diagnosis of intestinal duplication was in fact an anorectal malformation). Postnatal diagnosis was not achieved in four cases: None of them required surgery, and clinical follow-up was favorable. The abdominal cysts were isolated in 52 cases (71%) and associated with other anomalies in 21 cases (29%). Aneuploidies were diagnosed in three cases (all trisomy 21). Eight cases underwent termination of pregnancy; there were no fetal deaths and one neonatal death. Postnatal surgery was performed in 30 out of 65 liveborn infants (46.1%). Overall diagnostic agreement between prenatal and postnatal diagnosis of fetal intra-abdominal cystic lesions is high. © 2015 John Wiley & Sons, Ltd.

  15. Nutritional status and body fat distribution in children and adolescentes with Cystic Fibrosis.

    PubMed

    Chaves, Célia Regina Moutinho de Miranda; Cunha, Ana Lúcia Pereira da; Costa, Ana Carolina da; Costa, Roseli de Souza Santos da; Lacerda, Speranza Vieira

    2015-11-01

    assessing the nutritional status and body fat distribution in children and adolescents with cystic fibrosis. Fifty-six (56) 8-18 year old patients were assessed for fat distribution by dual energy X-ray absorptiometry, for nutritional status by height/age, and for body mass index to age and dietary intake by 24-hour dietary recall. Approximately 50% of the sample showed adequate nutritional status. Most of it showed inadequate caloric and lipid intake. BMI/age was the nutritional indicator that best showed the increased percentage of trunk fat, android/gynecoidratio and trunk fat/total fat ratio. Patients with Pancreatic Insufficiency and eutrophic individuals showed higher median android/gynecoidratio. Increased abdominal adiposity was evidenced by DXA. The BMI did not identify decreased lean body mass. However, when body mass was high, it was significant for abdominal adiposity. The anthropometric assessment of patients with cystic fibrosis should be associated with body composition and body fat distribution to obtain an earlier malnutrition and cardiometabolic risk factor diagnosis.

  16. Mucinous cystic neoplasms of the mesentery: a case report and review of the literature

    PubMed Central

    Metaxas, Georgios; Tangalos, Athanasios; Pappa, Polyxeni; Papageorgiou, Irene

    2009-01-01

    Background Mucinous cystic neoplasms arise in the ovary and various extra-ovarian sites. While their pathogenesis remains conjectural, their similarities suggest a common pathway of development. There have been rare reports involving the mesentery as a primary tumour site. Case presentation A cystic mass of uncertain origin was demonstrated radiologically in a 22 year old female with chronic abdominal pain. At laparotomy, the mass was fixed within the colonic mesentery. Histology demonstrated a benign mucinous cystadenoma. Methods and results We review the literature on mucinous cystic neoplasms of the mesentery and report on the pathogenesis, biologic behavior, diagnosis and treatment of similar extra-ovarian tumors. We propose an updated classification of mesenteric cysts and cystic tumors. Conclusion Mucinous cystic neoplasms of the mesentery present almost exclusively in women and must be considered in the differential diagnosis of mesenteric tumors. Only full histological examination of a mucinous cystic neoplasm can exclude a borderline or malignant component. An updated classification of mesenteric cysts and cystic tumors is proposed. PMID:19454018

  17. Selected Adnexal Cystic Masses in Postmenopausal Women Can be Safely Managed by Laparoscopy

    PubMed Central

    Lee, Jeong-Won; Kim, Chul Jung; Lee, Ji Eun; Lee, Sun-Joo; Kim, Byoung-Gie; Lee, Je-Ho; Bae, Duk-Soo

    2005-01-01

    The aim of this study was to assess the efficacy and safety of laparoscopic treatment for adnexal cystic masses that were predicted to be benign in postmenopausal women. Postmenopausal women found to have an adnexal cystic mass were retrospectively evaluated with transvaginal ultrasonography, and serum CA-125 levels. The selection criteria were adnexal cystic masses greater than 3 cm but less than 10 cm, the masses were in the benign range (4-8) of Sassone's scoring system for transvaginal ultrasonography, and the patients had serum CA-125 levels less than 65 IU/mL. Two hundred nineteen women fulfilled the criteria and underwent operative laparoscopy. Almost all the masses (99.5%) were accurately predicted to be benign except for one borderline ovarian tumor. Two hundreds thirteen (97.3%) women were successfully managed by operative laparoscopy and six (2.7%) required laparotomy. For the patients managed by laparoscopy, the mean operative time was 51.3 min; the mean hospital stay was 2.5 days. There was no significant morbidity and surgery-related mortality. The combination of the Sassone's scoring system for transvaginal ultrasonography and serum CA-125 level can accurately predict benign cystic masses, and operative laparoscopy is technically feasible and safe for the management of adnexal mass in postmenopausal women. PMID:15953871

  18. Preferential reduction of quadriceps over respiratory muscle strength and bulk after lung transplantation for cystic fibrosis.

    PubMed

    Pinet, C; Scillia, P; Cassart, M; Lamotte, M; Knoop, C; Mélot, C; Estenne, M

    2004-09-01

    In the absence of complications, recipients of lung transplants for cystic fibrosis have normal pulmonary function but the impact of the procedure on the strength and bulk of respiratory and limb muscles has not been studied. Twelve stable patients who had undergone lung transplantation for cystic fibrosis 48 months earlier (range 8-95) and 12 normal subjects matched for age, height, and sex were studied. The following parameters were measured: standard lung function, peak oxygen uptake by cycle ergometry, diaphragm surface area by computed tomographic (CT) scanning, diaphragm and abdominal muscle thickness by ultrasonography, twitch transdiaphragmatic and gastric pressures, quadriceps isokinetic strength, and quadriceps cross section by CT scanning, and lean body mass. Diaphragm mass was computed from diaphragm surface area and thickness. Twitch transdiaphragmatic and gastric pressures, diaphragm mass, and abdominal muscle thickness were similar in the two groups but quadriceps strength and cross section were decreased by nearly 30% in the patients. Patients had preserved quadriceps strength per unit cross section but reduced quadriceps cross section per unit lean body mass. The cumulative dose of corticosteroids was an independent predictor of quadriceps atrophy. Peak oxygen uptake showed positive correlations with quadriceps strength and cross section in the two groups, but peak oxygen uptake per unit quadriceps strength or cross section was reduced in the patient group. The diaphragm and abdominal muscles have preserved strength and bulk in patients transplanted for cystic fibrosis but the quadriceps is weak due to muscle atrophy. This atrophy is caused in part by corticosteroid therapy and correlates with the reduction in exercise capacity.

  19. Juvenile granulosa cell tumor arising from intra-abdominal testis in newborn: case report and review of the literature.

    PubMed

    Partalis, Nikolaos; Tzardi, Maria; Barbagadakis, Sophia; Sakellaris, George

    2012-05-01

    In the present case, the neonate presented with a left-sided abdominal mass and an empty left scrotum. Abdominal ultrasonography showed well-defined cystic formation, and laparotomy revealed a tumor arising from an intra-abdominal left testis. The carcinoembryonic antigen and neuron-specific enolase levels were within normal limits, and the serum β-human chorionic gonadotropin and α-fetoprotein levels were within age-related normal values. The findings from the immunochemistry tests confirmed the diagnosis. Copyright © 2012 Elsevier Inc. All rights reserved.

  20. Costs Associated with Surgically Treated Cases of Abdominal Cystic Echinococcosis: A Single Center's Experience from 2008 to 2014, Pavia, Italy

    PubMed Central

    Narra, Roberta; Maestri, Marcello; Budke, Christine M.; Tamarozzi, Francesca; Mariconti, Mara; Nicoletti, Giovanni J.; Rinaldi, Francesca; Brunetti, Enrico

    2016-01-01

    Cystic echinococcosis (CE) is a globally distributed zoonosis caused by the Echinococcus granulosus sensu lato species complex. Four approaches are available for treatment of abdominal CE: surgery, percutaneous aspiration, chemotherapy with albendazole, and watch-and-wait. Allocation of patients to these different treatment options mainly depends on the stage of the cystic lesion. However, as available guidelines are not widely followed, surgery is often applied even without the correct indication outside referral centers. This is not only a disadvantage for the patient, but also a waste of money. In this study, we evaluated the cost of the surgical approach for abdominal CE by analyzing hospitalization costs for 14 patients admitted to the General Surgery Ward at the “San Matteo” Hospital Foundation in Pavia, Italy, from 2008 through 2014. We found that the total cost of a single hospitalization, including hospital stay, surgical intervention, personnel, drugs, and administrative costs ranged from €5,874 to 23,077 (median €11,033) per patient. Our findings confirm that surgery can be an expensive option. Therefore, surgical intervention should be limited to cyst types that do not benefit from nonsurgical therapies and appropriate case management can best be accomplished by using a cyst stage-specific approach. PMID:27273641

  1. Costs Associated with Surgically Treated Cases of Abdominal Cystic Echinococcosis: A Single Center's Experience from 2008 to 2014, Pavia, Italy.

    PubMed

    Narra, Roberta; Maestri, Marcello; Budke, Christine M; Tamarozzi, Francesca; Mariconti, Mara; Nicoletti, Giovanni J; Rinaldi, Francesca; Brunetti, Enrico

    2016-08-03

    Cystic echinococcosis (CE) is a globally distributed zoonosis caused by the Echinococcus granulosus sensu lato species complex. Four approaches are available for treatment of abdominal CE: surgery, percutaneous aspiration, chemotherapy with albendazole, and watch-and-wait. Allocation of patients to these different treatment options mainly depends on the stage of the cystic lesion. However, as available guidelines are not widely followed, surgery is often applied even without the correct indication outside referral centers. This is not only a disadvantage for the patient, but also a waste of money. In this study, we evaluated the cost of the surgical approach for abdominal CE by analyzing hospitalization costs for 14 patients admitted to the General Surgery Ward at the "San Matteo" Hospital Foundation in Pavia, Italy, from 2008 through 2014. We found that the total cost of a single hospitalization, including hospital stay, surgical intervention, personnel, drugs, and administrative costs ranged from €5,874 to 23,077 (median €11,033) per patient. Our findings confirm that surgery can be an expensive option. Therefore, surgical intervention should be limited to cyst types that do not benefit from nonsurgical therapies and appropriate case management can best be accomplished by using a cyst stage-specific approach. © The American Society of Tropical Medicine and Hygiene.

  2. Cystic pancreatic tumors (CPT): predictors of malignant behavior.

    PubMed

    Javle, Milind; Shah, Pankaj; Yu, Jihnhee; Bhagat, Vishal; Litwin, Alan; Iyer, Renuka; Gibbs, John

    2007-03-01

    Due to widespread use of imaging studies, increasing cystic pancreatic tumor (CPT) cases are being detected. The diagnosis of malignancy in CPT cases requires pancreatectomy. Clinical and laboratory characteristics of CPT may predict underlying malignancy. CPT cases treated between 1994 and 2004 at our institution were included. Pseudocysts were excluded. Serous cystadenoma (SCA), mucinous cystadenoma (MCA), intrapapillary mucinous tumor, cystic endocrine tumor, and lymphoepithelial cysts were classified as benign or pre-malignant. Serous cystadenocarcinoma (SCACA), mucinous cystadenocarcinoma (MCACA), and adenocarcinoma (ACA) were classified as malignant. Thirty-five patients had histological confirmation. Median age was 65 years. Male/female ratio was higher in malignant group (P = 0.0284). Weight loss and abdominal mass were more prevalent in malignant group (P = 0.042 and 0.028, respectively). Malignant lesions were larger, associated with local invasion (superior mesenteric artery (SMA), superior mesenteric vein (SMV), portal vein (PV) complex or celiac encasement) and CA 19-9 elevation. On univariate analyses, local invasion (P = 0.0029), negative surgical intervention (P = 0.0010), presence of ACA (P = 0.0044), or malignant CPT (P = 0.0018) were associated with shorter survival. On a multivariate analysis, local invasion was associated with shorter survival [Hazard ratio (HR) = 4.322, P = 0.0218], while surgical intervention was associated with improved survival (HR = 0.179, P = 0.0124). Male sex, abdominal mass, weight loss, larger tumor size, local invasion, and elevated CA 19-9 were associated with malignant CPT.

  3. A Huge Mature Cystic Teratoma in a Nulliparous Patient

    PubMed Central

    Gunduz, Melih; Pata, Ozlem; Unlu, Cihat

    2015-01-01

    Here we report a case of a giant mature cystic teratoma of the ovary in a 38-year-old nullipaous woman. The patient presented with abdominal distension and abdominal pain. Laparotomy and cystectomy yielded satisfactory results. Histologic evaluation confirmed a benign cystic teratoma of the ovary. The patient underwent surgery for rupture of corpus luteum six years ago and no gross lesion was seen at the operation. This case demonstrates that dermoid cysts can grow to enormous sizes within a short duration. PMID:26500962

  4. Can a Modified Bosniak Classification System Risk Stratify Pediatric Cystic Renal Masses?

    PubMed

    Saltzman, Amanda F; Carrasco, Alonso; Colvin, Alexandra N; Meyers, Mariana L; Cost, Nicholas G

    2018-03-20

    We characterize and apply the modified Bosniak classification system to a cohort of children with cystic renal lesions and known surgical pathology. We identified all patients at our institution with cystic renal masses who also underwent surgery for these lesions. Patients without available preoperative imaging or pathology were excluded. All radiological imaging was independently reviewed by a pediatric radiologist blinded to pathological findings. Imaging characteristics (size, border, septations, calcifications, solid components, vascularity) were recorded from the most recent preoperative ultrasounds and computerized tomograms. The modified Bosniak classification system was applied to these scans and then correlated with final pathology. A total of 22 patients met study criteria. Median age at surgery was 6.1 years (range 11 months to 16.8 years). Of the patients 12 (54.5%) underwent open nephrectomy, 6 (27.3%) open partial nephrectomy, 2 (9.1%) laparoscopic cyst decortication, 1 (4.5%) open renal biopsy and 1 (4.5%) laparoscopic partial nephrectomy. Final pathology was benign in 9 cases (41%), intermediate in 6 (27%) and malignant in 7 (32%). All malignant lesions were modified Bosniak class 4, all intermediate lesions were modified class 3 or 4 and 8 of 9 benign lesions (89%) were modified class 1 or 2. Cystic renal lesions in children with a modified Bosniak class of 1 or 2 were most often benign, while class 3 or 4 lesions warranted surgical excision since more than 90% of masses harbored intermediate or malignant pathology. The modified Bosniak classification system appears to allow for a reasonable clinical risk stratification of pediatric cystic renal masses. Copyright © 2018 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  5. Mucinous Cystadenocarcinoma Co-Existing with Mature Cystic Teratoma : A Rare Case Report

    PubMed Central

    Yeshvanth, Sunil Kumar; Shrinivas, Teerthanath; Agrawal, Tanu; Shetty, Jayaprakash K

    2015-01-01

    Co-existence of mucinous cystadenocarcinoma with mature cystic teratoma in the same ovary is very rare. We report a case in a 48-year-old female who presented with left abdominal mass. CT scan revealed a heterogeneous enhancing left ovarian mass lesion. Clinical diagnosis of complex ovarian cyst was made, later underwent laparotomy and histologically diagnosed as mucinous cystadenocarcinoma (grade 2) co-existing with benign cystic teratoma, stage Ia (FIGO) of the left ovary. Six months after surgery, the patient is doing well without any recurrence or metastasis. Hence, histopathological examination plays a significant role in accurate diagnosis and management of the patient. So, we should be aware of these rare co-existent tumours and meticulous dissection should be done to look for any synchronous tumours or malignant areas; since management and prognosis will vary significantly depending upon the microscopic type and stage. PMID:26393136

  6. Ultrasound in the diagnosis of palpable abdominal masses in children.

    PubMed

    Annuar, Z; Sakijan, A S; Annuar, N; Kooi, G H

    1990-12-01

    Ultrasound examinations were done to evaluate clinically palpable abdominal masses in 125 children. The examinations were normal in 21 patients. In 15 patients, the clinically palpable masses were actually anterior abdominal wall abscesses or hematomas. Final diagnosis was available in 87 of 89 patients with intraabdominal masses detected on ultrasound. The majority (71%) were retroperitoneal masses where two-thirds were of renal origin. Ultrasound diagnosis was correct in 68 patients (78%). All cases of hydronephrosis were correctly diagnosed based on characteristic ultrasound appearances. Correct diagnoses of all cases of adrenal hematoma, psoas abscess, liver hematoma, liver abscess and one case of liver metastases were achieved with correlation of relevant clinical information.

  7. Castleman Disease Presenting as an Abdominal Mass.

    PubMed

    Shariati, Farnaz; Verter, Elizabeth; Chang, Wendy; Huang, Li; Joshi, Virendra

    2017-01-01

    Unicentric Castleman disease is a rare condition of lymphoid hyperplasia, of which only 15% of cases occur in the abdomen. We report a 66-year-old man who presented with complaints of abdominal pain. Computed tomography scans revealed nephrolithiasis and a homogeneous calcified mass between the pancreas and stomach and several para-pancreatic nodes. Direct visualization during exploratory laparotomy revealed a mass on the lesser curvature of the stomach. Pyloromyotomy and mass resection were performed. Biopsy showed reactive lymphoid hyperplasia consistent with the hyaline vascular variant of Castleman disease.

  8. Castleman Disease Presenting as an Abdominal Mass

    PubMed Central

    Verter, Elizabeth; Chang, Wendy; Huang, Li; Joshi, Virendra

    2017-01-01

    Unicentric Castleman disease is a rare condition of lymphoid hyperplasia, of which only 15% of cases occur in the abdomen. We report a 66-year-old man who presented with complaints of abdominal pain. Computed tomography scans revealed nephrolithiasis and a homogeneous calcified mass between the pancreas and stomach and several para-pancreatic nodes. Direct visualization during exploratory laparotomy revealed a mass on the lesser curvature of the stomach. Pyloromyotomy and mass resection were performed. Biopsy showed reactive lymphoid hyperplasia consistent with the hyaline vascular variant of Castleman disease. PMID:28584844

  9. Gastric trichobezoar: abdominal mass in a child with sickle cell disease.

    PubMed

    Sciarretta, Jason D; Bond, Sheldon J

    2011-11-01

    Abdominal pain is a frequent occurrence among the pediatric population and can be a diagnostic challenge. Trichobezoar is a differential diagnosis that is often neglected. Different from previously reported cases, we present a 3-year-old girl with sickle cell disease with complaints of acute abdominal pain, suspecting sickle cell splenic sequestration. The child presented to the emergency department with sharp epigastric pain and an associated palpable upper abdominal mass. This case illustrates a large obstructing gastric trichobezoar and summarizes both the diagnostic modalities and treatment.

  10. Omentalization of cystic sublumbar lymph node metastases for long-term palliation of tenesmus and dysuria in a dog with anal sac adenocarcinoma.

    PubMed

    Hoelzler, M G; Bellah, J R; Donofro, M C

    2001-12-15

    A 13-year-old castrated male Bassett Hound was examined because of a 2-week history of severe constipation and tenesmus. Radiography revealed a large cystic mass in the caudal portion of the abdomen that was compressing the urethra and obstructing the pelvic canal. A small perianal mass was also noticed in the region of the left anal sac. Exploratory surgery was performed, but the mass was deemed unresectable. Instead, the mass was incised, drained, and omentalized in an attempt to establish continuous drainage after surgery. Cytologic evaluation of the perianal mass was consistent with a diagnosis of anal sac adenocarcinoma. Histologic evaluation of the abdominal mass revealed it was a lymph node effaced by adenocarcinoma. Despite the poor prognosis for anal sac adenocarcinoma with metastatic spread to the sublumbar lymph nodes, tenesmus and dysuria in this dog remained palliated until the dog's death 18 months after surgery. Omentalization was successful in providing a continuous method of fluid drainage for this cystopapillary abdominal tumor.

  11. Bowel ultrasound imaging in patients with cystic fibrosis: Relationship with clinical symptoms and CFTR genotype.

    PubMed

    Fraquelli, Mirella; Baccarin, Alessandra; Corti, Fabiola; Conti, Clara Benedetta; Russo, Maria Chiara; Della Valle, Serena; Pozzi, Roberta; Cressoni, Massimo; Conte, Dario; Colombo, Carla

    2016-03-01

    Ultrasound imaging is used to assess bowel abnormalities in gastrointestinal diseases. We aimed to assess the rate of predefined bowel ultrasound signs and their relationship with gastrointestinal symptoms and the cystic fibrosis transmembrane conductance regulator (CFTR) genotype in cystic fibrosis patients in regular follow-up. Prospective study of 70 consecutive patients with cystic fibrosis and 45 controls who underwent abdominal ultrasound; pertinent findings were related to gastrointestinal symptoms and, in cystic fibrosis patients, to pancreatic status, malabsorption degree, lipase intake, CFTR genotype (classified as severe or mild against functional class of CFTR mutations). 96% patients showed at least one abnormal bowel ultrasound sign. Most frequent signs were lymph node enlargement (64%), bowel loop dilatation (55%), thick corpuscular intraluminal content (49%), bowel wall hypervascularization (26%), thickened bowel wall (22%) and intussusception (17%). Patients with recurrent abdominal pain showed more bowel wall hypervascularization than patients without recurrent pain (47% vs. 19%, respectively; p = 0.02) and intussusception (58% vs. 17%, respectively; p < 0.01). Genotype was not associated to specific bowel ultrasound signs. Patients with bowel loop intussusception showed greater lipase intake than those without intussusception (8.118 ± 2.083 vs. 5.994 ± 4.187, respectively; p < 0.01). Cystic fibrosis patients present a higher rate of bowel ultrasound abnormalities than controls. Bowel ultrasound abnormalities are associated with abdominal symptoms. Copyright © 2015 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  12. Waist Circumference Adjusted for Body Mass Index and Intra-Abdominal Fat Mass

    PubMed Central

    Berentzen, Tina Landsvig; Ängquist, Lars; Kotronen, Anna; Borra, Ronald; Yki-Järvinen, Hannele; Iozzo, Patricia; Parkkola, Riitta; Nuutila, Pirjo; Ross, Robert; Allison, David B.; Heymsfield, Steven B.; Overvad, Kim; Sørensen, Thorkild I. A.; Jakobsen, Marianne Uhre

    2012-01-01

    Background The association between waist circumference (WC) and mortality is particularly strong and direct when adjusted for body mass index (BMI). One conceivable explanation for this association is that WC adjusted for BMI is a better predictor of the presumably most harmful intra-abdominal fat mass (IAFM) than WC alone. We studied the prediction of abdominal subcutaneous fat mass (ASFM) and IAFM by WC alone and by addition of BMI as an explanatory factor. Methodology/Principal Findings WC, BMI and magnetic resonance imaging data from 742 men and women who participated in clinical studies in Canada and Finland were pooled. Total adjusted squared multiple correlation coefficients (R2) of ASFM and IAFM were calculated from multiple linear regression models with WC and BMI as explanatory variables. Mean BMI and WC of the participants in the pooled sample were 30 kg/m2 and 102 cm, respectively. WC explained 29% of the variance in ASFM and 51% of the variance in IAFM. Addition of BMI to WC added 28% to the variance explained in ASFM, but only 1% to the variance explained in IAFM. Results in subgroups stratified by study center, sex, age, obesity level and type 2 diabetes status were not systematically different. Conclusion/Significance The prediction of IAFM by WC is not improved by addition of BMI. PMID:22384179

  13. Giant multilocular cystadenoma of the prostate: a rare cause of huge cystic pelvic mass.

    PubMed

    Olgun, Deniz Cebi; Onal, Bulent; Mihmanli, Ismail; Kantarci, Fatih; Durak, Haydar; Demir, Hale; Cetinel, Bulent

    2012-03-01

    Giant multilocular prostatic cystadenoma is a rare benign tumor that evolves from the prostate gland. Obstructive voiding symptoms occur in all reported cases. These lesions do not invade adjacent structures. Preoperative radiologic evaluation can define the benign nature of the lesion. Here we report a case of large cystic lesions identified by magnetic resonance imaging and sonographic findings that caused an extensive mass effect in the pelvis. When retrovesical, huge cystic lesions fill the pelvis completely in young men, with high levels of serum prostate-specific antigen, giant multilocular prostatic cystadenoma should be considered as a differential diagnosis. To our knowledge, this is the youngest case of prostatic cystadenoma reported in the literature.

  14. Abdominal Lipomatosis with Secondary Self-Strangulation of Masses in an Adult Rhesus Macaque (Macaca mulatta)

    PubMed Central

    Chum, Helen H; Long, C Tyler; McKeon, Gabriel P; Chang, Angela G; Luong, Richard H; Albertelli, Megan A

    2014-01-01

    An 10-y-old, intact male rhesus macaque (Macaca mulatta) presented for bilateral scrotal swelling and a distended abdomen. A soft mass in the left upper quadrant of the abdomen was palpated. A barium study did not reveal any gastrointestinal abnormalities. Exploratory laparotomy revealed a large (1.25 kg, 15.0 × 13.0 × 9.5 cm), red and tan, soft, circumscribed, spherical mass within the greater omentum and 10 to 20 smaller (diameter, 1 to 4 cm), soft to firm masses in the mesentery and greater omentum. The resected mass was a self-strangulating abdominal lipoma, a pedunculated neoplasm composed of white adipocytes arising from peritoneal adipose tissue undergoing secondary coagulation necrosis after strangulation of the blood supply due to twisting of the mass around the peduncle. The smaller masses were histologically consistent with simple or self-strangulating pedunculated abdominal lipomas. The macaque presented again 9 mo later with a firm, 5.0-cm mass in the midabdomen, with intestinal displacement visible on radiographs. Given this animal's medical history and questionable prognosis, euthanasia was elected. Necropsy revealed numerous, multifocal to coalescing, 1.0- to 15.0-cm, pale tan to yellow, circumscribed, soft to firm, spherical to ellipsoid, pedunculated masses that were scattered throughout the mesentery, greater omentum, lesser omentum, and serosal surfaces of the gastrointestinal tract. All of the masses were pedunculated abdominal lipomas, and most demonstrated coagulation necrosis due to self-strangulation of the blood supply. To our knowledge, this report is the first to describe abdominal lipomatosis with secondary self-strangulation of masses in a rhesus macaque. PMID:25402181

  15. Associations of infant subcutaneous fat mass with total and abdominal fat mass at school-age. The Generation R Study

    PubMed Central

    Santos, Susana; Gaillard, Romy; Oliveira, Andreia; Barros, Henrique; Abrahamse-Berkeveld, Marieke; van der Beek, Eline M; Hofman, Albert; Jaddoe, Vincent WV

    2017-01-01

    Background Skinfold thickness enables the measurement of overall and regional subcutaneous fatness in infancy and may be associated with total and abdominal body fat in later childhood. We examined the associations of subcutaneous fat in infancy with total and abdominal fat at school-age. Methods In a population-based prospective cohort study among 821 children, we calculated total subcutaneous fat (sum of biceps, triceps, suprailiacal and subscapular skinfold thicknesses) and central-to-total subcutaneous fat ratio (sum of suprailiacal and subscapular skinfold thicknesses/total subcutaneous fat) at 1.5 and 24 months. At 6 years, we measured fat mass index (total fat/height3), central-to-total fat ratio (trunk fat/total fat) and android-to-gynoid fat ratio (android fat/gynoid fat) by dual-energy X-ray absorptiometry and preperitoneal fat mass area by abdominal ultrasound. Results Central-to-total subcutaneous fat ratio at 1.5 months was positively associated with fat mass index and central-to-total fat ratio at 6 years, whereas both total and central-to-total subcutaneous fat ratio at 24 months were positively associated with all childhood adiposity measures (p<0.05). A 1-standard-deviation scores higher total subcutaneous fat at 24 months was associated with an increased risk of childhood overweight (Odds Ratio 1.70 [95% Confidence Interval 1.36, 2.12]). These associations were weaker than those for body mass index and stronger among girls than boys. Conclusions Subcutaneous fat in infancy is positively associated with total and abdominal fat at school-age. Our results also suggest that skinfold thicknesses add little value to estimate later body fat, as compared to body mass index. PMID:27225335

  16. Associations of Infant Subcutaneous Fat Mass with Total and Abdominal Fat Mass at School-Age: The Generation R Study.

    PubMed

    Santos, Susana; Gaillard, Romy; Oliveira, Andreia; Barros, Henrique; Abrahamse-Berkeveld, Marieke; van der Beek, Eline M; Hofman, Albert; Jaddoe, Vincent W V

    2016-09-01

    Skinfold thickness enables the measurement of overall and regional subcutaneous fatness in infancy and may be associated with total and abdominal body fat in later childhood. We examined the associations of subcutaneous fat in infancy with total and abdominal fat at school-age. In a population-based prospective cohort study among 821 children, we calculated total subcutaneous fat (sum of biceps, triceps, suprailiacal, and subscapular skinfold thicknesses) and central-to-total subcutaneous fat ratio (sum of suprailiacal and subscapular skinfold thicknesses/total subcutaneous fat) at 1.5 and 24 months. At 6 years, we measured fat mass index (total fat/height(3) ), central-to-total fat ratio (trunk fat/total fat), and android-to-gynoid fat ratio (android fat/gynoid fat) by dual-energy X-ray absorptiometry and preperitoneal fat mass area by abdominal ultrasound. Central-to-total subcutaneous fat ratio at 1.5 months was positively associated with fat mass index and central-to-total fat ratio at 6 years, whereas both total and central-to-total subcutaneous fat ratio at 24 months were positively associated with all childhood adiposity measures. A 1-standard-deviation scores higher total subcutaneous fat at 24 months was associated with an increased risk of childhood overweight (odds ratio 1.70, 95% confidence interval 1.36, 2.12). These associations were weaker than those for body mass index and stronger among girls than boys. Subcutaneous fat in infancy is positively associated with total and abdominal fat at school-age. Our results also suggest that skinfold thicknesses add little value to estimate later body fat, as compared with body mass index. © 2016 John Wiley & Sons Ltd.

  17. Effect of High-Intensity Interval Training on Total, Abdominal and Visceral Fat Mass: A Meta-Analysis.

    PubMed

    Maillard, Florie; Pereira, Bruno; Boisseau, Nathalie

    2018-02-01

    High-intensity interval training (HIIT) is promoted as a time-efficient strategy to improve body composition. The aim of this meta-analysis was to assess the efficacy of HIIT in reducing total, abdominal, and visceral fat mass in normal-weight and overweight/obese adults. Electronic databases were searched to identify all related articles on HIIT and fat mass. Stratified analysis was performed using the nature of HIIT (cycling versus running, target intensity), sex and/or body weight, and the methods of measuring body composition. Heterogeneity was also determined RESULTS: A total of 39 studies involving 617 subjects were included (mean age 38.8 years ± 14.4, 52% females). HIIT significantly reduced total (p = 0.003), abdominal (p = 0.007), and visceral (p = 0.018) fat mass, with no differences between the sexes. A comparison showed that running was more effective than cycling in reducing total and visceral fat mass. High-intensity (above 90% peak heart rate) training was more successful in reducing whole body adiposity, while lower intensities had a greater effect on changes in abdominal and visceral fat mass. Our analysis also indicated that only computed tomography scan or magnetic resonance imaging showed significant abdominal and/or visceral fat-mass loss after HIIT interventions. HIIT is a time-efficient strategy to decrease fat-mass deposits, including those of abdominal and visceral fat mass. There was some evidence of the greater effectiveness of HIIT running versus cycling, but owing to the wide variety of protocols used and the lack of full details about cycling training, further comparisons need to be made. Large, multicenter, prospective studies are required to establish the best HIIT protocols for reducing fat mass according to subject characteristics.

  18. Distal intestinal obstruction syndrome and colonic pathologies in cystic fibrosis.

    PubMed

    Canny, J D; Brookes, A; Bowley, D B

    2017-01-02

    The management of abdominal pain in cystic fibrosis can be complicated. Distal intestinal obstruction syndrome is a common cause of pain and obstruction in these patients. Knowledge of the diagnosis and management and of similar presenting symptoms is essential for the hospital doctor.

  19. Epidural Cystic Spinal Meningioma

    PubMed Central

    Zhang, Ji; Chen, Zheng-he; Wang, Zi-feng; Sun, Peng; Jin, Jie-tian; Zhang, Xiang-heng; Zhao, Yi-ying; Wang, Jian; Mou, Yong-gao; Chen, Zhong-ping

    2016-01-01

    Abstract Cystic spinal meningioma (CSM) is an uncommon meningioma variant. Extradural CSMs are particularly rare and difficult to distinguish from other intraaxial tumors. This study presents a case of a 36-year-old woman with intraspinal extradual CSM at the thoracolumbar spine. She experienced persistent weakness, progressive numbness, and sensory disturbance in the right lower limb. Magnetic resonance imaging (MRI) of the patient revealed an irregular cystic mass at the thoracic 11 to lumbar 3 levels dorsally. This case was misdiagnosed as other neoplasms prior to surgery because of the atypical radiographic features and location of the tumor. Extradural CSMs should be considered in the differential diagnosis of intraspinal extradural cystic neoplasms. Complete removal of cystic wall provides an optimal outcome, rendering the lesion curable. PMID:26986119

  20. Body Mass Normalization for Lateral Abdominal Muscle Thickness Measurements in Adolescent Athletes.

    PubMed

    Linek, Pawel

    2017-09-01

    To determine the value of allometric parameters for ultrasound measurements of the oblique external (OE), oblique internal (OI), and transversus abdominis (TrA) muscles in adolescent athletes. The allometric parameter is the slope of the linear regression line between the log-transformed body mass and log-transformed muscle size measurement. The study included 114 male adolescent football players between the ages of 10 and 19 years. All individuals with no surgical procedures performed on the trunk area and who had played a sport for at least 2 years were included. A real-time B-mode ultrasound scanner with a linear array transducer was used to obtain images of the lateral abdominal muscles from both sides of the body. A stabilometric platform was used to assess the body mass value. The correlations between body mass and the OE, OI, and TrA muscle thicknesses were r = 0.73, r = 0.79, and r = 0.64, respectively (in all cases, P < .0001). The allometric parameters were 0.77 for the OE, 0.67 for the OI, and 0.61 for the TrA. Using these parameters, no significant correlations were found between body mass and the allometric-scaled thickness of the lateral abdominal muscles. Significant positive correlations exist between body mass and lateral abdominal muscle thickness in adolescent athletes. Therefore, it is reasonable to advise that the values of the allometric parameters for the OE, OI, and TrA muscles obtained in this study should be used, and the allometric-scaled thicknesses of those muscles should be analyzed in future research on adolescent athletes. © 2017 by the American Institute of Ultrasound in Medicine.

  1. A colon-associated cystic mass occurring in conjunction with cecal dilatation in a Holstein cow

    PubMed Central

    Garrett, Edgar F.; Singh, Kuldeep

    2012-01-01

    A 7-year-old Holstein cow was presented for reduced appetite and decreased milk production. Based on physical examination, cecal dilatation was the primary differential diagnosis and was confirmed at surgery. However, in addition to the dilated cecum, 2 large cystic masses were found firmly attached to the proximal loop of the ascending colon. PMID:23729831

  2. Prevalence and Risk Factors for Human Cystic Echinococcosis in the Cusco Region of the Peruvian Highlands Diagnosed Using Focused Abdominal Ultrasound

    PubMed Central

    Tamarozzi, Francesca; Hou, Amy; Morales, Maria Luisa; Giordani, Maria Teresa; Vilca, Freddy; Mozo, Karen; Bascope, Ruben; White, A. Clinton; Brunetti, Enrico; Chen, Lin; Cabada, Miguel M.

    2017-01-01

    Latin America is among the highly endemic regions for cystic echinococcosis (CE). In Peru, an estimated 1,139 disability-adjusted life years are lost annually from surgical treatment of CE. This is comparable with the combined total for Argentina, Brazil, Uruguay, and Chile. The prevalence of human infection has been investigated in the central Peruvian Andes, but there are no community-based screening data from other regions of Peru. We carried out a population survey in January 2015 using abdominal ultrasound to estimate the prevalence of abdominal CE in the Canas and Canchis provinces, in the Cusco region of Peru. Among 1,351 subjects screened, 41 (3%) had CE. There was significant variation between communities with similar socioeconomic features in a small geographical area. A history of CE was reported by 4.1% of the screened subjects, among whom 30.3% still had CE on ultrasound. Among patients reporting previous CE treatment, 14.9% had CE in active stages. Limited education, community of residence, and knowing people with CE in the community were associated with CE. These results demonstrate a significant burden of CE in the region and suggest the need for further investigations, control activities, and optimization of clinical management for CE in this area. PMID:28719254

  3. [CLINICAL AND EPIDEMIOLOGICAL PECULIARITIES OF CYSTIC ECHINOCOCCOSIS IN CHILDREN].

    PubMed

    Melia, Kh; Kokaia, N; Manjgaladze, M; Kelbakiani-Kvinikhidze, T; Sulaberidze, G

    2017-04-01

    The postoperative period of cystic echinococcosis was studied in 13 children. Demographic, epidemiological, clinical diagnosis, treatment, number location, and development of cysts and serologic data were analyzed. Age of children at diagnosis range 5 to 17 years. All patients with cystic echinococcosis had abdominal cysts. The liver was the main organ involved in ten patients (76,9%) - they had cysts located in the liver, two patients (15,4%) had lung cyst, one patient had concomitant lung and liver cysts. Twelve patients had single cysts and one had more than one abdominal cysts. Surgical treatment was performed in 23,1% cases. Ultrasound studies (US) were performed during the monitoring period. Evaluation of cysts was assessed by monitoring US changes. Positive dynamics was revealed in all patients; relapse of the disease was not noticed. Proceeding from the fact that in all patients echoarchitectonics of the hepatic tissue was lumped with a non-uniform structure and uneven ultrasound distribution, it is assumed that these changes are indicative of the development of connective tissue in the liver.

  4. Functional cystic thyroid adenoma in a cat.

    PubMed

    Hofmeister, E; Kippenes, H; Mealey, K L; Cantor, G H; Löhr, C V

    2001-07-15

    A 9-year-old cat with hyperthyroidism was referred for radioactive iodine treatment. The cat also had a ventral cervical mass that the owners reported had been present for several years and had increased in size during the past few weeks. On physical examination, the mass was found to have caused lateral displacement of the trachea, esophagus, jugular vein, and common carotid artery. The mass was aspirated and was determined to be cystic in nature. Concentrations of thyroid hormones in the cystic fluid were similar to serum concentrations, and nuclear scintigraphy revealed thyroactive tissue lining the cyst wall. Magnetic resonance imaging suggested that the cyst originated from the right lobe of the thyroid gland. The cat was treated with sodium iodide I 131 but died 4 days later, presumably as a result of aspiration of gastric or esophageal contents. A necropsy was not performed, but histologic examination of a biopsy specimen of the mass indicated that it was a cystic thyroid adenoma.

  5. A huge ovarian mucinous cystadenoma associated with contralateral teratoma and polycystic ovary syndrome in an obese adolescent girl.

    PubMed

    Thaweekul, Patcharapa; Thaweekul, Yuthadej; Mairiang, Karicha

    2016-12-01

    A 13-year-old, obese girl presented with acute abdominal pain with abdominal distension for a year. The physical examination revealed marked abdominal distension with a large well-circumscribed mass sized 13×20 cm. Her body mass index (BMI) was 37.8 kg/m2. An abdominal CT scan revealed a huge multiloculated cystic mass and a left adnexal mass. She had an abnormal fasting plasma glucose and low HDL-C. Laparotomy, right salpingooophorectomy, left cystectomy, lymph node biopsies and partial omentectomy were performed. The left ovary demonstrated multiple cystic follicles over the cortex. The histologic diagnosis was a mucinous cystadenoma of the right ovary and a matured cystic teratoma of the left ovary. Both obesity and polycystic ovary syndrome (PCOS) are associated with a greater risk of ovarian tumours, where PCOS could be either the cause or as a consequence of an ovarian tumour. We report an obese, perimenarchal girl with bilateral ovarian tumours coexistent with a polycystic ovary and the metabolic syndrome.

  6. Mass screening of multiple abdominal solid organs using mobile helical computed tomography scanner--a preliminary report.

    PubMed

    Ishikawa, Susumu; Aoki, Jun; Ohwada, Susumu; Takahashi, Toru; Morishita, Yasuo; Ueda, Keisuke

    2007-04-01

    The possibility of a new screening procedure for multiple abdominal solid organs using a mobile helical computed tomography (CT) scanner was evaluated. A total of 4,543 residents, who were 40 years of age or older, received CT scanning without contrast medium. The mean age of participants was 64 years including 2,022 males and 2,521 females. A total of 2,105 abnormal findings were uniquely detected in 1,594 participants. Liver and kidney diseases including ureter occupied around 30% of total abnormal findings, respectively. Besides frequent cystic or calcified lesions, solid tumours were suspected in 56 lesions, which received further examination by specialized physicians. Five (9%) of them were confirmed as being malignant tumours including pancreatic cancer in two patients, and liver, lung and ovary cancers in one patient each, respectively. All five patients with each malignant lesion received curative operations. Small-sized abdominal aortic aneurysms and heart valve diseases were uniquely found in 22 and two patients, respectively. Qualitative diagnoses of solid tumours were difficult using CT findings without contrast medium. CT screening procedures require further investigation in aspect of the selection of examinees, CT scanning procedure, sensitivity and specificity, and cost-effectiveness.

  7. Body Mass Normalization for Ultrasound Measurements of Adolescent Lateral Abdominal Muscle Thickness.

    PubMed

    Linek, Pawel; Saulicz, Edward; Wolny, Tomasz; Myśliwiec, Andrzej

    2017-04-01

    The purpose of this study was to determine the value of the allometric parameter for ultrasound measurements of the thickness of the oblique external (OE), internal (OI), and transversus abdominis (TrA) muscles in the adolescent population. The allometric parameter is the slope of the linear regression line between the log transformed body mass and log transformed muscle size measurement. The study included 321 adolescents between the ages of 10 and 17, consisting of 160 boys and 161 girls. The participants were recruited from local schools and attended regular school classes at normal grade levels. All individuals with no signs of scoliosis (screening with use of a scoliometer), and no surgical procedures performed on the trunk area were included. A real-time ultrasound B-scanner with a linear array transducer was used to obtain images of the lateral abdominal muscles from both sides of the body. The correlation between body mass and the OE muscle was r = 0.69; the OI muscle r = 0.68; and the TrA muscle r = 0.53 (in all cases, P < .0001). The allometric parameter for the OE was 0.88296; the OI 0.718756; and the TrA 0.60986. Using these parameters, no significant correlations were found between body mass and the allometric-scaled thickness of the lateral abdominal muscles. Significant positive correlations exist between body mass and lateral abdominal muscle thickness assessed by ultrasound imaging. Therefore, it is reasonable to advise that the values of the allometric parameters for OE, OI, and TrA obtained in this study should be used in other studies performed on adolescents. © 2016 by the American Institute of Ultrasound in Medicine.

  8. Normal Weight but Low Muscle Mass and Abdominally Obese: Implications for the Cardiometabolic Risk Profile in Chronic Obstructive Pulmonary Disease.

    PubMed

    Beijers, Rosanne J H C G; van de Bool, Coby; van den Borst, Bram; Franssen, Frits M E; Wouters, Emiel F M; Schols, Annemie M W J

    2017-06-01

    It is well established that low muscle mass affects physical performance in chronic obstructive pulmonary disease (COPD). We hypothesize that combined low muscle mass and abdominal obesity may also adversely influence the cardiometabolic risk profile in COPD, even in those with normal weight. The cardiometabolic risk profile and the responsiveness to 4 months high-intensity exercise training was assessed in normal-weight patients with COPD with low muscle mass stratified by abdominal obesity. This is a cross-sectional study including 81 clinically stable patients with COPD (age 62.5 ± 8.2 years; 50.6% males; forced expiratory volume in 1 second 55.1 ± 19.5 percentage predicted) with fat-free mass index <25th percentile eligible for outpatient pulmonary rehabilitation. Body composition, blood biomarkers, blood pressure, physical activity level, dietary intake, and physical performance were assessed at baseline and in a subgroup after 4 months of exercise training. Mean body mass index was 22.7 ± 2.7 kg/m 2 , and 75% of patients had abdominal obesity. Abdominally obese patients had higher glucose, insulin, homeostatic model assessment for insulin resistance (HOMA-IR), branched chain amino acids and a higher prevalence of metabolic syndrome compared with those without abdominal obesity. Exercise training improved cycling endurance time and quadriceps strength, but did not yield a clinically meaningful improvement of the cardiometabolic risk profile. Triglycerides showed a significant decrease, while the HOMA-IR increased. Abdominal obesity is highly prevalent in normal-weight patients with COPD with low muscle mass who showed an increased cardiometabolic risk compared with patients without abdominal obesity. This cardiometabolic risk profile was not altered after 4 months of exercise training. Copyright © 2017 AMDA – The Society for Post-Acute and Long-Term Care Medicine. Published by Elsevier Inc. All rights reserved.

  9. Simultaneous liver mucinous cystic and intraductal papillary mucinous neoplasms of the bile duct: a case report.

    PubMed

    Budzynska, Agnieszka; Hartleb, Marek; Nowakowska-Dulawa, Ewa; Krol, Robert; Remiszewski, Piotr; Mazurkiewicz, Michal

    2014-04-14

    Cystic hepatic neoplasms are rare tumors, and are classified into two separate entities: mucinous cystic neoplasms (MCNs) and intraductal papillary mucinous neoplasms of the bile duct (IPMN-B). We report the case of a 56-year-old woman who presented with abdominal pain and jaundice due to the presence of a large hepatic multilocular cystic tumor associated with an intraductal tumor. Partial hepatectomy with resection of extrahepatic bile ducts demonstrated an intrahepatic MCN and an intraductal IPMN-B. This is the first report of the simultaneous occurrence of these two histologically distinct entities in the liver.

  10. Congenital cystic lesions of the head and neck.

    PubMed

    Ibrahim, Mohannad; Hammoud, Khaled; Maheshwari, Mohit; Pandya, Amit

    2011-08-01

    This article presents clinical characteristics and radiologic features of congenital cervical cystic masses, among them thyroglossal duct cysts, cystic hygromas, branchial cleft cysts, and the some of the rare congenital cysts, such as thymic and cervical bronchogenic cysts. The imaging options and the value of each for particular masses, as well as present clinical and radiologic images for each, are discussed. Copyright © 2011 Elsevier Inc. All rights reserved.

  11. Pulmonary cystic echinococcosis.

    PubMed

    Santivanez, Saul; Garcia, Hector H

    2010-05-01

    Pulmonary cystic echinococcosis, a zoonosis caused by the larvae of the dog tapeworm Echinococcus granulosus, is considered as a major public health problem in countries where dogs are used to care for large herds. Despite its frequency and widespread endemicity, the literature on pulmonary cystic echinococcosis is scarce and not systematic. We aimed to summarize currently available information to provide a comprehensive overview for clinicians facing cases of pulmonary cystic echinococcosis. Despite discrepancies in the literature, some patterns can be discerned. The ratio of lung:liver involvement is higher in children than in adults. Most pulmonary cases are discovered incidentally on routine radiograph evaluation; also most infected individuals remain asymptomatic until the cyst enlarges sufficiently to cause symptoms. Symptoms are usually caused by mass effect from the cyst. Complications (cyst rupture, aggregated infection) change the clinical presentation, producing cough, chest pain, hemoptysis, or vomica. Diagnosis is obtained by chest radiographs or computed tomography, and supported by serology. Presurgical chemotherapy reduces the chances of seeding and recurrence. Treatment with benzimidazoles is an option when surgery is unavailable or complete removal is unfeasible. Diagnosis of pulmonary cystic echinococcosis is primarily made by imaging, and surgery remains the main therapeutic approach.

  12. Tracking of abdominal subcutaneous and preperitoneal fat mass during childhood. The Generation R Study.

    PubMed

    Vogelezang, S; Gishti, O; Felix, J F; van der Beek, E M; Abrahamse-Berkeveld, M; Hofman, A; Gaillard, R; Jaddoe, V W V

    2016-04-01

    Overweight and obesity in early life tends to track into later life. Not much is known about tracking of abdominal fat. Our objective was to examine the extent of tracking of abdominal fat measures during the first six years of life. We performed a prospective cohort study among 393 Dutch children followed from the age of 2 years (90% range 1.9; 2.3) until the age of 6 years (90% range 5.7; 6.2). At both ages, we performed abdominal ultrasound to measure abdominal subcutaneous and preperitoneal fat distances and areas, and we calculated the preperitoneal/subcutaneous fat distance ratio. High abdominal fat measures were defined as values in the upper 15%. Abdominal subcutaneous fat distance and area, and preperitoneal fat area at 2 years were correlated with their corresponding measures at 6 years (all P-values <0.01), with the strongest coefficients for abdominal subcutaneous fat measures. Preperitoneal fat distance at the age of 2 years was not correlated with the corresponding measure at 6 years. The tracking coefficient for preperitoneal/subcutaneous fat distance ratio from 2 to 6 years was r=0.36 (P<0.01). Children with high abdominal subcutaneous fat measures at 2 years had increased risk of having high abdominal subcutaneous fat measures at 6 years (odds ratios 9.2 (95% confidence interval (CI) 4.1-20.8) and 12.4 (95% CI 5.4-28.6) for subcutaneous fat distance and area, respectively). These associations were not observed for preperitoneal fat measures. Our findings suggest that both abdominal subcutaneous and preperitoneal fat mass measures track during childhood, but with stronger tracking for abdominal subcutaneous fat measures. An adverse abdominal fat distribution in early life may have long-term consequences.

  13. Cystic urogenital anomalies in ferrets (Mustela putorius furo).

    PubMed

    Li, X; Fox, J G; Erdman, S E; Lipman, N S; Murphy, J C

    1996-03-01

    Single or multiple semispherical to bilobulated fluid-filled cystic structures of variable size were observed on the dorsal aspects of the urinary bladder of four male and two female ferrets (Mustela putorius furo). All ferrets had been neutered. On physical examination, the cysts were palpated as caudal abdominal masses. Three of the six ferrets presented with dysuria, and two ferrets had signs compatible with endocrine dysfunction. Adrenal cortical hyperplasia or neoplasia were observed in all of the five ferrets examined. Sex hormones assayed in one of the six ferrets revealed elevated levels of serum estrodiol. The posterior aspect of the cysts was located on and/or attached to the trigone or neck of the bladder, with variable intraluminal communication with the bladder and/or the urethra. The anterior aspect of the cysts projected dorsally or dorsocranially into the caudal abdomen. The cysts were thin walled and contained urinelike fluid (n = 5) or viscous yellow fluid (n = 1). Histologically, the cyst walls were composed of three layers, epithelium, muscle, and serosa, with fibrovascular stroma between layers. The epithelium consisted of simple to stratified transitional, columnar, or squamous epithelial cells. The muscular layer consisted of intermittent bundles and/or single to double layers of continuous to discontinuous smooth muscle. The serosal layer consisted of loose fibrous stroma covered by flattened mesothelial cells. The cystic anomalies in these ferrets were most likely derived from the urogenital glands/ducts or other remnants.

  14. Agenesis of the gallbladder with hypoplastic cystic duct diagnosed at laparoscopy.

    PubMed

    Kwon, A-Hon; Yanagimoto, Hiroaki; Matsui, Yoichi; Imamura, Atsushi

    2006-08-01

    An 86-year-old man was admitted to our department with complaints of intermittent upper abdominal pain. Ultrasonography of the abdomen showed dilated extrahepatic bile ducts containing stones; however, the gallbladder was not clearly identified. Magnetic resonance cholangiopancreatography showed dilated extrahepatic ducts and choledocholithiasis without gallbladder visualization. The stone extraction was performed with endoscopic sphincterotomy. Three-dimensional images using spiral-computed tomography after intravenous-infusion cholangiography clearly demonstrated an obstruction of the cystic duct. The patient was scheduled for laparoscopic cholecystectomy. At laparoscopy, the gallbladder fossa was not identified on the undersurface of the liver. Despite a thorough examination of the intrahepatic (left-sided within the lesser omentum), retroperitoneal, retrohepatic (within the falciform ligament), retroduodenal, and retropancreatic areas using laparoscopic ultrasonography, the gallbladder was not found. After careful dissection of the hepatoduodenal ligament, the dilated extrahepatic bile duct and a 1-cm length of hypoplastic cystic duct were found. Gallbladder agenesis is usually accompanied by the lack of the cystic duct. The present case is the third report of gallbladder agenesis with a patent or hypoplastic cystic duct.

  15. [Panhypopituitarism caused by an intrasellar cystic mass in late-onset hypogonadism clinic].

    PubMed

    Yamamoto, Yoshiyuki; Takada, Shingo; Kinjo, Takanori; Nonomura, Daichi; Yoneda, Suguru; Nomura, Hironori; Tei, Norihide; Matsumiya, Kiyomi; Okusu, Takahiro

    2013-10-01

    A 74-year-old man who was referred to our late onset hypogonadism clinic presented with sweating and loss of appetite. His aging males' symptoms (AMS) and international index of erectile function (IIEF-5) scores were 59 and 2, respectively. His hormonal examination revealed extremely low free testosterone values. The patient was started on androgen replacement therapy, but his symptoms did not improve. Additional hormonal examinations revealed low values for other anterior pituitary hormones. Magnetic resonance imaging revealed an intrasellar cystic mass with suprasellar extension. We considered this mass caused hypothalamic hypopituitarism. A load test for anterior pituitary hormones revealed panhypogonadism. His symptoms improved after administration of adrenal and thyroid hormones and androgen. Five months after start of drug administration, his AMS score improved to 29, but IIEF-5 score showed little change. As a matter of course, not only androgen but all pituitary-related hormones are needed for hypopituitarism patients.

  16. Mature cystic teratoma with high proportion of solid thyroid tissue: a controversial case with unusual imaging findings.

    PubMed

    Gil, Rui; Cunha, Teresa Margarida; Rolim, Ines

    2017-07-01

    We describe a case of a mature cystic teratoma of the ovary with high proportion of solid thyroid tissue (< 50% of the entire tumor) in a childbearing woman. The patient presented with non-specific abdominal bloating. Pelvic ultrasound and magnetic resonance imaging revealed a complex cystic-solid tumor confined to the left ovary with an anterior fat-containing locus compatible with mature cystic teratoma and a posterior predominantly solid component with low signal intensity on T2-weighted images that was histopatologically diagnosed as benign thyroid tissue. Thyroglobulin levels were in normal range. Although thyroid tissue is present in up to 20% of mature cystic teratomas, with exception of struma ovarii, it is not usually macroscopically nor radiologically identified. The differential diagnosis should include T2-hypointense adnexal lesions associated with mature cystic teratoma, malignant transformation of mature teratoma, and immature teratoma.

  17. Pancreatic neuroendocrine tumor with complete replacement of the pancreas by serous cystic neoplasms in a patient with von Hippel-Lindau disease: a case report.

    PubMed

    Maeda, Shimpei; Motoi, Fuyuhiko; Oana, Shuhei; Ariake, Kyohei; Mizuma, Masamichi; Morikawa, Takanori; Hayashi, Hiroki; Nakagawa, Kei; Kamei, Takashi; Naitoh, Takeshi; Unno, Michiaki

    2017-09-25

    von Hippel-Lindau disease is a dominantly inherited multi-system syndrome with neoplastic hallmarks. Pancreatic lesions associated with von Hippel-Lindau include serous cystic neoplasms, simple cysts, and neuroendocrine tumors. The combination of pancreatic neuroendocrine tumors and serous cystic neoplasms is relatively rare, and the surgical treatment of these lesions must consider both preservation of pancreatic function and oncological clearance. We report a patient with von Hippel-Lindau disease successfully treated with pancreas-sparing resection of a pancreatic neuroendocrine tumor where the pancreas had been completely replaced by serous cystic neoplasms, in which pancreatic function was preserved. A 39-year-old female with von Hippel-Lindau disease was referred to our institution for treatment of a pancreatic neuroendocrine tumor. Abdominal computed tomography demonstrated a well-enhanced mass, 4 cm in diameter in the tail of the pancreas, and two multilocular tumors with several calcifications, 5 cm in diameter, in the head of the pancreas. There was complete replacement of the pancreas by multiple cystic lesions with diameters ranging from 1 to 3 cm. Magnetic resonance cholangiopancreatography showed innumerable cystic lesions on the whole pancreas and no detectable main pancreatic duct. Endoscopic ultrasound-guided fine-needle aspiration of the mass in the pancreatic tail showed characteristic features of a neuroendocrine tumor. A diagnosis of pancreatic neuroendocrine tumor in the tail of the pancreas and mixed-type serous cystic neoplasms replacing the whole pancreas was made and she underwent distal pancreatectomy while avoiding total pancreatectomy. The stump of the pancreas was sutured as firm as possible using a fish-mouth closure. The patient made a good recovery and was discharged on postoperative day 9. She is currently alive and well with no symptoms of endocrine or exocrine pancreatic insufficiency 8 months after surgery. A pancreas

  18. Fetus-in-Fetu: An Unusual Cause for Abdominal Mass in Infancy

    PubMed Central

    Grosfeld, Jay L.; Stepita, Donald S.; Nance, Walter E.; Palmer, Catherine G.

    1974-01-01

    Fetus-in-fetu is an unusual cause of retroperitoneal abdominal mass in infants, which most likely arises from inclusion of a monozygotic, diamniotic twin. This entity is distinguished from teratoma by its embryological origin, its unusual location in the retroperitoneal space, its invariable benignity, and by the presence of vertebral organization with limb buds and well-developed organ systems. Diagnosis is made radiographically by the finding of a diminutive vertebral column on a plain abdominal film. The treatment of choice is total excision with special attention being given to the fetus' blood supply which may be directly from the host's superior mesenteric vessels. A well-documented example of this unusual entity that occurred in a six-week old infant male is presented. ImagesFig. 1.Fig. 2.Fig. 3. PMID:4471720

  19. A case report of an unusual presentation of ocular rhinosporidiosis as a conjunctival cystic mass.

    PubMed

    Shah, Sangeeta; Lavaju, Poonam; Bharati, Priyanka; Joshi, Icchya

    2017-02-01

    Ocular involvement of rhinosporidiosis is seen in about 15% of cases and clinically appears as a freely mobile, granular, pink, fleshy sessile or pedunculated mass. The conjunctiva is the most common site of origin. A 25-year-old male presented with a painless mass in the lower part of the left eye for 6 months which was gradually progressive. Examination revealed a pedunculated mass of 15 mm x 15 mm arising from the lower palpebrae of the left eye retracting the lower lid. It appeared to be vascular with few white spots at the apex. Rest of the ocular examinations was within the normal limit. The presentations of ocular rhinosporidiosis vary. Though the conjunctival origin is very common, it may not have a classic pink fleshy appearance at all times. A vascular/cystic painless conjunctival mass should also be considered as a case of rhinosporidiosis in prone areas.

  20. The suitability of matrix assisted laser desorption/ionization time of flight mass spectrometry in a laboratory developed test using cystic fibrosis carrier screening as a model.

    PubMed

    Farkas, Daniel H; Miltgen, Nicholas E; Stoerker, Jay; van den Boom, Dirk; Highsmith, W Edward; Cagasan, Lesley; McCullough, Ron; Mueller, Reinhold; Tang, Lin; Tynan, John; Tate, Courtney; Bombard, Allan

    2010-09-01

    We designed a laboratory developed test (LDT) by using an open platform for mutation/polymorphism detection. Using a 108-member (mutation plus variant) cystic fibrosis carrier screening panel as a model, we completed the last phase of LDT validation by using matrix-assisted laser desorption/ionization time of flight mass spectrometry. Panel customization was accomplished via specific amplification primer and extension probe design. Amplified genomic DNA was subjected to allele specific, single base extension endpoint analysis by mass spectrometry for inspection of the cystic fibrosis transmembrane regulator gene (NM_000492.3). The panel of mutations and variants was tested against 386 blinded samples supplied by "authority" laboratories highly experienced in cystic fibrosis transmembrane regulator genotyping; >98% concordance was observed. All discrepant and discordant results were resolved satisfactorily. Taken together, these results describe the concluding portion of the LDT validation process and the use of mass spectrometry to detect a large number of complex reactions within a single run as well as its suitability as a platform appropriate for interrogation of scores to hundreds of targets.

  1. Retrospective evaluation of ultrasound-indeterminate renal multilocular cystic masses by using neutrophil-lymphocyte ratio and computed tomography.

    PubMed

    Wang, Hanfeng; Gu, Liangyou; Jia, Rui; Zeng, Jing; Liu, Xiaoxia; Zhang, Dawei; Wu, Yongjie; Luo, Guangda; Zhang, Xu

    2017-01-01

    To evaluate the clinical usefulness of neutrophil-lymphocyte ratio (NLR) in differentiating the ultrasound-indeterminate renal multilocular cystic masses (RMCM) in comparison with computed tomography (CT) and whether NLR has additional benefits to CT on sensitivity of detecting the malignant. Overall, 93 patients who underwent normal ultrasound with a conclusion of indeterminate RMCM were examined by NLR and CT within 30 days before surgery or follow-up from March to September 2014 at PLA General Hospital and enrolled in this retrospective study. Logistic regression model was performed to find independent predictors for differentiating true nature of RMCM; differences in the validity parameters and diagnostic power of CT, NLR, and their combination were compared using McNemar tests and AUC model, respectively. The final diagnoses of the 93 patients consisted of 36 patients with benign complex cysts, 16 with multilocular cystic renal cell carcinoma, 9 with multilocular cystic nephroma, and 32 with clear cell renal cell carcinoma. Higher NLR were strongly associated with malignant masses. Multivariate logistic regression analysis revealed that NLR could be an independent predictor for differentiating true nature of these masses (OR = 3.617; 95% CI: 1.219-10.727; P = 0.020). For detecting the malignant masses, the sensitivity, specificity, and accuracy were 71.9%, 80.6%, and 75.3% for CT and 57.9%, 88.9%, and 69.9% for NLR under cutoff value of 2.31, respectively, whereas those of CT+NLR were 89.5%, 69.4%, and 81.7%. No significant difference was found between CT and NLR in sensitivity (P = 0.185), specificity (P = 0.549), and accuracy (P = 0.428). But the sensitivity of CT+NLR was significantly higher than those of CT (P = 0.002) and NLR (P<0.001), respectively; AUC model analysis indicated that CT+NLR got the largest area of 0.795 (P<0.001, 95% CI: 0.693-0.896) in comparison with those of CT (area = 0.795, P<0.001, 95% CI: 0.661-0.864) and NLR (area = 0.734, P<0

  2. The challenging image-guided abdominal mass biopsy: established and emerging techniques 'if you can see it, you can biopsy it'.

    PubMed

    Sainani, Nisha I; Arellano, Ronald S; Shyn, Paul B; Gervais, Debra A; Mueller, Peter R; Silverman, Stuart G

    2013-08-01

    Image-guided percutaneous biopsy of abdominal masses is among the most commonly performed procedures in interventional radiology. While most abdominal masses are readily amenable to percutaneous biopsy, some may be technically challenging for a number of reasons. Low lesion conspicuity, small size, overlying or intervening structures, motion, such as that due to respiration, are some of the factors that can influence the ability and ultimately the success of an abdominal biopsy. Various techniques or technologies, such as choice of imaging modality, use of intravenous contrast and anatomic landmarks, patient positioning, organ displacement or trans-organ approach, angling CT gantry, triangulation method, real-time guidance with CT fluoroscopy or ultrasound, sedation or breath-hold, pre-procedural image fusion, electromagnetic tracking, and others, when used singularly or in combination, can overcome these challenges to facilitate needle placement in abdominal masses that otherwise would be considered not amenable to percutaneous biopsy. Familiarity and awareness of these techniques allows the interventional radiologist to expand the use of percutaneous biopsy in clinical practice, and help choose the most appropriate technique for a particular patient.

  3. Effects of testosterone supplementation on whole body and regional fat mass and distribution in human immunodeficiency virus-infected men with abdominal obesity.

    PubMed

    Bhasin, Shalender; Parker, Robert A; Sattler, Fred; Haubrich, Richard; Alston, Beverly; Umbleja, Triin; Shikuma, Cecilia M

    2007-03-01

    Whole body and abdominal obesity are associated with increased risk of diabetes mellitus and heart disease. The effects of testosterone therapy on whole body and visceral fat mass in HIV-infected men with abdominal obesity are unknown. The objective of this study was to determine the effects of testosterone therapy on intraabdominal fat mass and whole body fat distribution in HIV-infected men with abdominal obesity. IN this multicenter, randomized, placebo-controlled, double-blind trial, 88 HIV-positive men with abdominal obesity (waist-to-hip ratio > 0.95 or mid-waist circumference > 100 cm) and total testosterone 125-400 ng/dl, or bioavailable testosterone less than 115 ng/dl, or free testosterone less than 50 pg/ml on stable antiretroviral regimen, and HIV RNA less than 10,000 copies per milliliter were randomized to receive 10 g testosterone gel or placebo daily for 24 wk. Fat mass and distribution were determined by abdominal computerized tomography and dual energy x-ray absorptiometry during wk 0, 12, and 24. We used an intention-to-treat approach and nonparametric statistical methods. Baseline characteristics were balanced between groups. In 75 subjects evaluated, median percent change from baseline to wk 24 in visceral fat did not differ significantly between groups (testosterone 0.3%, placebo 3.1%, P = 0.75). Total (testosterone -1.5%, placebo 4.3%, P = 0.04) and sc (testosterone-7.2%, placebo 8.1%, P < 0.001) abdominal fat mass decreased in testosterone-treated men, but increased in placebo group. Testosterone therapy was associated with significant decrease in whole body, trunk, and appendicular fat mass by dual energy x-ray absorptiometry (all P < 0.001), whereas whole body and trunk fat increased significantly in the placebo group. The percent of individuals reporting a decrease in abdomen (P = 0.01), neck (P = 0.08), and breast size (P = 0.01) at wk 24 was significantly greater in testosterone-treated than placebo-treated men. Testosterone-treated men

  4. Local anesthesia and hydro-distension to facilitate cystic calculus removal in horses.

    PubMed

    Russell, Tom; Pollock, Patrick J

    2012-07-01

    To report a technique for improving surgical access to the bladder for removal of cystic calculi in male horses. Retrospective case series. Geldings (8) with cystic calculi. After catheterization of the bladder through the penis, 100 mL 2% lidocaine hydrochloride solution was instilled. After 10 minutes, the bladder was distended with sterile, warmed Hartmann's solution to a pressure of ≈ 40 cm H(2) O, using gravity feed. This was left in place until abdominal access was gained at surgery, then the fluid siphoned off via the catheter. Calculi were 3-11 cm in diameter (median, 6 cm). Bladder capacity ranged from 1.4 to 2.5 L (median, 1.8 L). Exteriorization time from placing a hand in the abdomen to having the bladder in a surgically accessible position was <5 minutes. The described technique facilitates exteriorization of the bladder for removal of cystic calculi. Copyright 2012 by The American College of Veterinary Surgeons.

  5. A giant cystic pheochromocytoma mimicking liver abscess an unusual presentation - a case report.

    PubMed

    Sarveswaran, Venugopal; Kumar, Surees; Kumar, Amit; Vamseedharan, Muthukumar

    2015-01-01

    Giant cystic pheochromocytoma is a rare neuroendocrine tumor. The possibility of cystic pheochromocytoma should be considered for any peri-adrenal mass even in absence of characteristic symptoms and negative biochemical analysis. The key in the management of a case of cystic pheochromocytoma is the preoperative suspicion and the intraoperative crisis management.

  6. Journal club: Renal masses detected at abdominal CT: radiologists' adherence to guidelines regarding management recommendations and communication of critical results.

    PubMed

    Maehara, Cleo K; Silverman, Stuart G; Lacson, Ronilda; Khorasani, Ramin

    2014-10-01

    The purpose of this study was to assess radiologists' adherence to published guidelines for managing renal masses detected at abdominal CT at one institution and to a critical results communication policy. A validated natural language processing tool supplemented by manual review was used to randomly assemble a cohort of 97 radiology reports from all abdominal CT reports (n = 11,952) generated from July 2010 to June 2011. Critical renal mass findings warranted consideration for surgery, intervention, or imaging follow-up and required direct, separate, and timely communication to the referrer in addition to the radiology report. Primary outcomes were adherence to guidelines and institutional policy for communicating critical results. Sample size allowed a 95% CI ± 5% for primary outcome. Pearson chi-square test was performed to assess whether radiology subspecialization was predictive of the primary outcome. Of all abdominal CT reports, 35.6% contained at least one renal mass finding (4.3% critical). Guideline adherence was lower for patients with critical than for those with noncritical findings (48/57 [84.2%] vs 40/40 [100%]; p = 0.01). Adherence to critical result communication policy was 73.7% (42/57). For critical findings, abdominal radiologists had higher guideline adherence (40/43 [93.0%] vs 8/14 [57.1%]; p = 0.001) and critical result communication policy adherence (36/43 [83.7%] vs 6/14 [42.9%]; p = 0.002) than nonabdominal radiologists. In reporting renal masses detected at abdominal CT, radiologists largely adhered to management guidelines but did not adhere to the critical results communication policy in one of four reports. Subspecialization improved adherence to both management guidelines and the institution's critical result communication policy.

  7. Cystic lesion around the hip joint

    PubMed Central

    Yukata, Kiminori; Nakai, Sho; Goto, Tomohiro; Ikeda, Yuichi; Shimaoka, Yasunori; Yamanaka, Issei; Sairyo, Koichi; Hamawaki, Jun-ichi

    2015-01-01

    This article presents a narrative review of cystic lesions around the hip and primarily consists of 5 sections: Radiological examination, prevalence, pathogenesis, symptoms, and treatment. Cystic lesions around the hip are usually asymptomatic but may be observed incidentally on imaging examinations, such as computed tomography and magnetic resonance imaging. Some cysts may enlarge because of various pathological factors, such as trauma, osteoarthritis, rheumatoid arthritis, or total hip arthroplasty (THA), and may become symptomatic because of compression of surrounding structures, including the femoral, obturator, or sciatic nerves, external iliac or common femoral artery, femoral or external iliac vein, sigmoid colon, cecum, small bowel, ureters, and bladder. Treatment for symptomatic cystic lesions around the hip joint includes rest, nonsteroidal anti-inflammatory drug administration, needle aspiration, and surgical excision. Furthermore, when these cysts are associated with osteoarthritis, rheumatoid arthritis, and THA, primary or revision THA surgery will be necessary concurrent with cyst excision. Knowledge of the characteristic clinical appearance of cystic masses around the hip will be useful for determining specific diagnoses and treatments. PMID:26495246

  8. Abdominal mass

    MedlinePlus

    ... lumpy mass in the right upper quadrant. Liver enlargement (hepatomegaly) can cause a firm, irregular mass below ... the kidney (usually only affects one kidney). Spleen enlargement (splenomegaly) can sometimes be felt in the left- ...

  9. Short- and Long-Term Effects of Abdominal Lipectomy on Weight and Fat Mass in Females: a Systematic Review.

    PubMed

    Seretis, Konstantinos; Goulis, Dimitrios G; Koliakos, Georgios; Demiri, Efterpi

    2015-10-01

    Adipose tissue is considered as an endocrine organ, which is developed in specific depots, distinguished either as subcutaneous or visceral. Lipectomy, by means of liposuction or abdominoplasty, is a common plastic surgery procedure, which can remove substantial amounts of subcutaneous fat. This systematic review aims to evaluate the impact of surgical removal of abdominal subcutaneous adipose tissue on body weight and fat mass in females in the short- and long-term. A systematic review was conducted using a predetermined protocol established according to the Cochrane Handbook's recommendations. PubMed, Scopus, CENTRAL, and the Cochrane Library were searched from inception to December 2014. Eligible studies were prospective studies with ≥1 month of follow-up that included female only individuals who underwent lipectomy of the abdominal region and reported on body weight, body mass index (BMI), or fat mass. Ten studies were included in this systematic review with a total of 231 individuals. A significant weight loss and BMI improvement were reported in 4 out of 5 studies with a mean follow-up of 1-2 months, but in none of the 5 studies with a longer follow-up (3-20 months). Fat mass showed a similar to weight change. The risk of bias was low for the two clinical trials but high for the observational studies included in the review. This systematic review revealed only a transient effect of abdominal lipectomy in body fat and weight in women, which fades a few months after the operation. These results corroborate the evidence from experimental and clinical studies, which support fat redistribution and compensatory fat growth, as a result of feedback mechanisms, triggered by fat removal. Additional clinical studies, with adequate follow-up, may further elucidate the long-term effects of abdominal lipectomy in body weight and composition. Systematic review registration PROSPERO CRD42015017564 ( www.crd.york.ac.uk/PROSPERO ).

  10. Cystic fibrosis - resources

    MedlinePlus

    Resources - cystic fibrosis ... The following organizations are good resources for information on cystic fibrosis : Cystic Fibrosis Foundation -- www.cff.org March of Dimes -- www.marchofdimes.org/baby/cystic-fibrosis-and- ...

  11. Adenoid cystic carcinoma of the larynx presenting with unusual subglottic mass: Case report.

    PubMed

    Kashiwagi, Takashi; Kanaya, Hiroaki; Konno, Wataru; Goto, Kazutaka; Hirabayashi, Hideki; Haruna, Shin-Ichi

    2016-10-01

    A 33-year-old woman presented with an unusual subglottic bulging mass accompanied by prolonged cough and wheeze. Laryngeal endoscopy revealed a bilateral, symmetrical mass immediately below the vocal cords with marked airway obstruction. Chronic subglottic laryngitis with inflammation or another condition such as amyloidosis was initially suspected. Cervicothoracic computed tomography revealed an obvious reduction of laryngeal caliber caused by an engulfing mass extending from just under the vocal cords to the cricoid ring, which was associated with thyroid, arytenoid, and cricoid cartilage destruction. Histopathological diagnosis of a biopsy specimen collected via a tracheotomy revealed that the lesion was a cT4aN0M0 adenoid cystic carcinoma (ACC) originating from the laryngeal minor salivary glands. The patient was treated by total laryngectomy with elective bilateral neck dissection under general anesthesia. Gross inspection of resected tissue confirmed yellowish-white, solid tumor mainly circumferentially encompassing the lumina of the cricoid ring. The histopathological findings confirmed typical ACC accompanied by a predominant cribriform appearance with no evidence of lymph node metastasis. The patient remains well and free of recurrence or metastasis. We herein describe laryngeal ACC and discuss radiological images and the surgical pathology. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  12. [Analyze prescription rules of Professor Jiang Liangduo treatment for abdominal mass based on traditional Chinese medicine inheritance platform].

    PubMed

    Lian, Xiao-Xiao; Guo, Xiao-Xia

    2018-01-01

    To investigate the herbal prescription rules of Professor Jiang Liangduo in the treatment of abdominal mass based on the traditional Chinese medicine inheritance support system software (TCMISS) of version 2.5, find out new herbal formulas for the treatment of abdominal mass, and then provide new reference to its traditional Chinese medicine therapy. By the method of retrospective study, one hundred and thirty-two outpatient prescriptions of Professor Jiang for the treatment of abdominal mass were collected to establish a typical database with TCMISS. Four properties, five tastes, channel tropism, frequency count, Chinese herbal prescriptions rules and the new prescriptions were analyzed so as to dig out the prescription rules. There were 57 herbs with a frequency>=15, and then 91 core combinations of 2-5 herbs were evolved and 9 new prescriptions were created. It was found out that these drugs mainly had the effects of liver nourishing and soothing, soft-moist and dredging-tonifying, supporting right and dispeling evil, cooperating with the method of calming the liver and resolving hard lump according to the actual situation. It reflected the thought of treatment based on syndrome differentiation in TCM, and provided a new reference for its clinical treatment and research. Copyright© by the Chinese Pharmaceutical Association.

  13. A correlation study of diagnostic fine-needle aspiration with histologic diagnosis in cystic neck lesions.

    PubMed

    Moatamed, Neda A; Naini, Bita V; Fathizadeh, Payman; Estrella, Julie; Apple, Sophia K

    2009-10-01

    The clinical diagnosis of a mass in the neck region encompasses a wide spectrum of differential diagnosis. Fine-needle aspiration is a quick and safe technique, which can provide useful information for initial assessment and further therapeutic measures. The aim of this retrospective study was to evaluate the performance characteristics of the fine-needle aspiration (FNA) in cystic neck lesions. Of 142 patients with FNA for cystic neck masses during 2002-2007, 92 cases were selected with a follow-up histologic diagnosis, excluding the cystic colloid nodule of the thyroid. The cases were divided into salivary gland cystic neck (37 patients) and non-salivary cystic neck (55 patients) mass groups. False-positive and false-negative diagnoses were applied only to the malignant lesions after confirmation by histopathology. In the first group, nine malignant and 28 benign diagnoses were made by FNA; of which three were false-negative. In the second group, there were nine malignant and 46 benign diagnoses with three false negatives. The overall performance of the FNA showed 76% sensitivity and 100% specificity. In conclusion, FNA of the cystic neck lesions offers an invaluable and highly specific initial diagnostic approach for the management of the patients. (c) 2009 Wiley-Liss, Inc.

  14. Rapid Loss of Bone Mass and Strength in Mice after Abdominal Irradiation

    PubMed Central

    Jia, Dan; Gaddy, Dana; Suva, Larry J.; Corry, Peter M.

    2011-01-01

    Localized irradiation is a common treatment modality for malignancies in the pelvic-abdominal cavity. We report here on the changes in bone mass and strength in mice 7–14 days after abdominal irradiation. Male C57BL/6 mice of 10–12 weeks of age were given a single-dose (0, 5, 10, 15 or 20 Gy) or fractionated (3 Gy × 2 per day × 7.5 days) X rays to the abdomen and monitored daily for up to 14 days. A decrease in the serum bone formation marker and ex vivo osteoblast differentiation was detected 7 days after a single dose of radiation, with little change in the serum bone resorption marker and ex vivo osteoclast formation. A single dose of radiation elicited a loss of bone mineral density (BMD) within 14 days of irradiation. The BMD loss was up to 4.1% in the whole skeleton, 7.3% in tibia, and 7.7% in the femur. Fractionated abdominal irradiation induced similar extents of BMD loss 10 days after the last fraction: 6.2% in the whole skeleton, 5.1% in tibia, and 13.8% in the femur. The loss of BMD was dependent on radiation dose and was more profound in the trabecula-rich regions of the long bones. Moreover, BMD loss in the total skeleton and the femurs progressed with time. Peak load and stiffness in the mid-shaft tibia from irradiated mice were 11.2–14.2% and 11.5–25.0% lower, respectively, than sham controls tested 7 days after a single-dose abdominal irradiation. Our data demonstrate that abdominal irradiation induces a rapid loss of BMD in the mouse skeleton. These effects are bone type- and region-specific but are independent of radiation fractionation. The radiation-induced abscopal damage to the skeleton is manifested by the deterioration of biomechanical properties of the affected bone. PMID:21859327

  15. Peripheral calcifying cystic odontogenic tumour of the maxillary gingiva.

    PubMed

    de Lima, Ana Paula; Kitakawa, Dárcio; Almeida, Janete Dias; Brandão, Adriana Aigotti Haberbeck; Anbinder, Ana Lia

    2012-08-23

    Odontogenic tumors are lesions that are derived from remnants of the components of the developing tooth germ. The calcifying cystic odontogenic tumor or calcifying odontogenic cyst is a benign cystic neoplasm of odontogenic origin that is characterized by an ameloblastoma-like epithelium and ghost cells. Calcifying cystic odontogenic tumor may be centrally or peripherally located, and its ghost cells may exhibit calcification, as first described by Gorlin in 1962. Most peripheral calcifying cystic odontogenic tumors are located in the anterior gingiva of the mandible or maxilla. Authors report a rare case of a peripheral calcifying cystic odontogenic tumor of the maxillary gingiva. A 39-year-old male patient presented with a fibrous mass on the attached buccal gingiva of the upper left cuspid teeth. It was 0.7-cm-diameter, painless and it was clinically diagnosed as a peripheral ossifying fibroma. After an excisional biopsy, the diagnosis was peripheric calcifying cystic odontogenic tumor. The patient was monitored for five years following the excision, and no recurrence was detected. All biopsy material must be sent for histological examination. If the histological examination of gingival lesions with innocuous appearance is not performed, the frequency of peripheral calcifying cystic odontogenic tumor and other peripheral odontogenic tumors may be underestimated.

  16. Peripheral calcifying cystic odontogenic tumour of the maxillary gingiva

    PubMed Central

    2012-01-01

    Background Odontogenic tumors are lesions that are derived from remnants of the components of the developing tooth germ. The calcifying cystic odontogenic tumor or calcifying odontogenic cyst is a benign cystic neoplasm of odontogenic origin that is characterized by an ameloblastoma-like epithelium and ghost cells. Calcifying cystic odontogenic tumor may be centrally or peripherally located, and its ghost cells may exhibit calcification, as first described by Gorlin in 1962. Most peripheral calcifying cystic odontogenic tumors are located in the anterior gingiva of the mandible or maxilla. Case presentation Authors report a rare case of a peripheral calcifying cystic odontogenic tumor of the maxillary gingiva. A 39-year-old male patient presented with a fibrous mass on the attached buccal gingiva of the upper left cuspid teeth. It was 0.7-cm-diameter, painless and it was clinically diagnosed as a peripheral ossifying fibroma. After an excisional biopsy, the diagnosis was peripheric calcifying cystic odontogenic tumor. The patient was monitored for five years following the excision, and no recurrence was detected. Conclusions All biopsy material must be sent for histological examination. If the histological examination of gingival lesions with innocuous appearance is not performed, the frequency of peripheral calcifying cystic odontogenic tumor and other peripheral odontogenic tumors may be underestimated. PMID:22917449

  17. Adenoid Cystic Carcinoma of Buccal Mucosa: A Rare Case Report.

    PubMed

    Garg, Vipul; Roy, Swati; Khanna, Kaveri Surya; Bakshi, Preeti Sethi; Chauhan, Isha

    2016-09-01

    Adenoid cystic carcinoma is a malignant neoplasm most commonly originating in salivary glands of head and neck region. Among intra oral adenoid cystic carcinoma, buccal mucosa is among the rarest sites. We report a case of adenoid cystic of buccal mucosa in a 40-year old female. We have discussed the clinical features, histopathology, diagnosis and treatment along with a brief review of the relevant literature. Although the buccal mucosa is an uncommon site for adenoid cystic carcinoma, the relatively indolent growth pattern of this case and its location which is rather atypical for this type of salivary gland malignancy primarily warrants the necessity behind reporting of this case. Secondly, adenoid cystic carcinoma should be considered in the differential diagnosis of mass of buccal mucosa. It is important to identify such cases rather early and surgical removal with adequate margins is the treatment of choice .

  18. Duodenum-preserving total pancreatic head resection for benign cystic neoplastic lesions.

    PubMed

    Beger, Hans G; Schwarz, Michael; Poch, Bertram

    2012-11-01

    Cystic neoplasms of the pancreas are diagnosed frequently due to early use of abdominal imaging techniques. Intraductal papillary mucinous neoplasm, mucinous cystic neoplasm, and serous pseudopapillary neoplasia are considered pre-cancerous lesions because of frequent transformation to cancer. Complete surgical resection of the benign lesion is a pancreatic cancer preventive treatment. The application for a limited surgical resection for the benign lesions is increasingly used to reduce the surgical trauma with a short- and long-term benefit compared to major surgical procedures. Duodenum-preserving total pancreatic head resection introduced for inflammatory tumors in the pancreatic head transfers to the patient with a benign cystic lesion located in the pancreatic head, the advantages of a minimalized surgical treatment. Based on the experience of 17 patients treated for cystic neoplastic lesions with duodenum-preserving total pancreatic head resection, the surgical technique of total pancreatic head resection for adenoma, borderline tumors, and carcinoma in situ of cystic neoplasm is presented. A segmental resection of the peripapillary duodenum is recommended in case of suspected tissue ischemia of the peripapillary duodenum. In 305 patients, collected from the literature by PubMed search, in about 40% of the patients a segmental resection of the duodenum and 60% a duodenum and common bile duct-preserving total pancreatic head resection has been performed. Hospital mortality of the 17 patients was 0%. In 305 patients collected, the hospital mortality was 0.65%, 13.2% experienced a delay of gastric emptying and a pancreatic fistula in 18.2%. Recurrence of the disease was 1.5%. Thirty-two of 175 patients had carcinoma in situ. Duodenum-preserving total pancreatic head resection for benign cystic neoplastic lesions is a safe surgical procedure with low post-operative morbidity and mortality.

  19. Management of cystic echinococcosis complications and dissemination: where is the evidence?

    PubMed

    Dziri, Chadli; Haouet, Karim; Fingerhut, Abe; Zaouche, Abdeljelil

    2009-06-01

    This systematic review was designed to provide "evidence-based" answers to identify the best treatment for a complicated hydatid cyst of the liver and the appropriate management of disseminated cystic echinococcosis. An extensive electronic search of the relevant literature was performed using Medline and the Cochrane Library. This systematic review enabled us make to determine the best treatment options for the following conditions. Liver hydatid cysts ruptured into the biliary tract: Common bile duct exploration should be conducted using intraoperative cholangiography and choledoscopy. When the biliary tract is cleared of all cystic content, T-tube drainage should be sufficient. The principal difficulty concerned the management of the large biliocystic fistula: suture or internal transfistulary drainage or fistulization. Medical treatment is indicated in association with surgery for 3 months postoperatively. During the preoperative period, endoscopic retrograde cholangiopancreatography (ERCP) combined with preoperative endoscopic sphincterotomy (ES) may decrease the incidence of postoperative external fistula. Liver hydatid cysts involving the thorax: An abdominal approach is mandatory when common bile duct drainage is required, and it may be sufficient to treat a direct rupture into bronchi. An acute abdomen, owing to Liver hydatid cysts ruptured into peritoneum, requires an emergent operation. Medical treatment should be associated. Cystic echinococcosis of the lung: Surgery is still the main therapeutic option to remove the cyst, suture bronchial fistula if necessary, followed by capitonnage. Osseous cystic echinococcosis: Wide surgical excision is recommended. Cystic echinococcosis of the heart: Cystopericystectomy is the "gold standard" procedure but is sometimes unsuitable for particular sites. Cystic echinococcosis of the kidney: Cystectomy with pericystectomy is feasible in 75% of cases; nephrectomy must be reserved for destroyed kidney. Multiple

  20. High-intensity interval training reduces abdominal fat mass in postmenopausal women with type 2 diabetes.

    PubMed

    Maillard, F; Rousset, S; Pereira, B; Traore, A; de Pradel Del Amaze, P; Boirie, Y; Duclos, M; Boisseau, N

    2016-12-01

    This study compared the effect of high-intensity interval training (HIIT) and moderate-intensity continuous training (MICT) for 16 weeks on whole-body and abdominal fat mass (FM) in postmenopausal women with type 2 diabetes (T2D). Seventeen women (69±1 years; BMI: 31±1kg.m -2 ) were randomly assigned to either a HIIT [60×(8s at 77-85% HR max , 12s of active recovery)] or MICT (40min at 55-60% of their individual HRR) cycling program for 16 weeks, 2 days/week. Dual-energy X-ray absorptiometry was used to measure whole-body and regional FM content, including abdominal adiposity and visceral adipose tissue. Plasma cholesterol, HDL, LDL, triglycerides, glucose and HbA 1c levels were measured. Levels of nutritional intake and physical activity were evaluated by 7-day self-reports. Dietary energy (caloric) intake, physical activity level and total body mass did not vary in either group from the beginning to the end of the training intervention. Overall, total FM decreased and total fat-free mass significantly increased over time (by around 2-3%). Total FM reduction at the end of the intervention was not significantly different between groups. However, significant loss of total abdominal (-8.3±2.2%) and visceral (-24.2±7.7%) FM was observed only with HIIT. Time effects were noted for HbA 1c and total cholesterol/HDL ratio. With no concomitant caloric restriction, an HIIT program in postmenopausal women with T2D (twice a week for 16 weeks) appeared to be more effective for reducing central obesity than MICT, and could be proposed as an alternative exercise training program for this population. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  1. Non-neoplastic cystic and cystic-like lesions of the pancreas: may mimic pancreatic cystic neoplasms.

    PubMed

    Goh, Brian K P; Tan, Yu-Meng; Chung, Yaw-Fui A; Chow, Pierce K H; Ong, Hock-Soo; Lim, Dennis T H; Wong, Wai-Keong; Ooi, London L P J

    2006-05-01

    Cystic lesions of the pancreas consist of a broad range of pathological entities. With the exception of the pancreatic pseudocyst, these are usually caused by pancreatic cystic neoplasms. Non-neoplastic pancreatic cystic and cystic-like lesions are extremely rare. In the present article, the surgical experience with these unusual entities over a 14-year period is reported. Between 1991 and 2004, all patients who underwent surgical exploration for a cystic lesion of the pancreas were retrospectively reviewed. Patients with a pancreatic pseudocyst were excluded. There were 106 patients of whom 8 (7.5%) had a final pathological diagnosis consistent with a non-neoplastic pancreatic cystic or cystic-like lesion, including 3 patients with a benign epithelial cyst, 2 with a pancreatic abscess (one tuberculous and one foreign body), 2 with mucous retention cysts and 1 with a mucinous non-neoplastic cyst. These eight patients are the focus of this study. There were six female and two male patients with a median age of 61.5 years (range, 41-71 years). All the patients were of Asian origin including seven Chinese and one Indian. Four of the patients were asymptomatic and their pancreatic cysts were discovered incidentally on radiological imaging for other indications. All the patients underwent preoperative radiological investigations, including ultrasonography, computed tomography or magnetic resonance imaging, which showed a cystic lesion of the pancreas. Three patients, all of whom were symptomatic, were diagnosed preoperatively with a malignant cystic neoplasm on the basis of radiological imaging. Two patients were eventually found to have a pancreatic abscess, one tuberculous and the other, secondary to foreign body perforation. The third patient was found on final histology to have chronic pancreatitis with retention cysts. The remaining five patients had a preoperative diagnosis of an indeterminate cyst; on pathological examination, they were found to have a benign

  2. [A Case of Cystic Cervical Lymph Node Metastasis of HPV-positive Tonsil Cancer, Being Discriminated as the Branchiogenic Carcinoma].

    PubMed

    Kambara, Rumi; Tamai, Masamitsu; Horii, Arata

    2016-02-01

    In recent years, human papillomavirus (HPV)-positive oropharyngeal carcinomas have been increasing. The first manifestation of these tumors is frequently as cystic metastasis to cervical lymph nodes that may precede recognition of the primary tumor, so, they often result in misdiagnosis as branchial cleft cysts. We report a case of cystic cervical lymph node metastasis of HPV-positive tonsil cancer. The patient was a 70-years-old man who noticed a mass on his left neck. The tumor was large and soft, and it was diagnosed as benign in fine-needle aspiration cytology. We diagnosed the tumor as a branchial cleft cyst and undertook surgery. The histopathological diagnosis was squamous cell carcinoma arising from a branchiogenic cyst. However, because it did not satisfy the diagnostic criteria, we diagnosed the tumor as an unknown primary tumor. One year later, left tonsil cancer was suspected based on PET-CT imaging and a left tonsillectomy was undertaken, whereafter tonsil cancer was found. In p16 immunostaining, it was positive in both cystic mass and tonsil. The cervical mass was cystic lymph node metastasis of HPV-positive tonsil cancer. It is important to investigate the oropharynx, when we found cystic cervical mass, because HPV-positive oropharyngeal carcinoma frequently results in cystic neck metastasis.

  3. [Solid cystic papillary tumor of the pancreas].

    PubMed

    Bahri, I; Njim, L; Khabir, A; Mahmoudi, H; Ghorbel, A; Zakhama, A; Jlidi, R

    2001-11-01

    Solid cystic papillary tumors of the pancreas are rare; they occur most commonly in young women. Despite their characteristic microscopic appearance, their immunophenotype is not specific. Their prognosis is excellent after complete surgical resection. The study aim was to report two cases in female patients who were 15 and 20 years old; the first tumor was discovered fortuitously and the second girl presented with abdominal pain and vomiting. Both tumors were encapsulated and located in the tail of the pancreas. The histological study showed the papillary architecture mixed with solid areas. Immunohistochemical staining was positive only for vimentin in one case and positive for cytokeratin, chromogranin, synaptophysin, neuron specific enolase, vimentin and protein S100 in the second case.

  4. Multimodality imaging of ovarian cystic lesions: Review with an imaging based algorithmic approach

    PubMed Central

    Wasnik, Ashish P; Menias, Christine O; Platt, Joel F; Lalchandani, Usha R; Bedi, Deepak G; Elsayes, Khaled M

    2013-01-01

    Ovarian cystic masses include a spectrum of benign, borderline and high grade malignant neoplasms. Imaging plays a crucial role in characterization and pretreatment planning of incidentally detected or suspected adnexal masses, as diagnosis of ovarian malignancy at an early stage is correlated with a better prognosis. Knowledge of differential diagnosis, imaging features, management trends and an algorithmic approach of such lesions is important for optimal clinical management. This article illustrates a multi-modality approach in the diagnosis of a spectrum of ovarian cystic masses and also proposes an algorithmic approach for the diagnosis of these lesions. PMID:23671748

  5. Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?

    PubMed

    Labombarda, Fabien; Saloux, Eric; Brouard, Jacques; Bergot, Emmanuel; Milliez, Paul

    2016-09-01

    Cystic fibrosis is a complex multi-systemic chronic disease characterized by progressive organ dysfunction with development of fibrosis, possibly affecting the heart. Over the last four decades pathological, experimental, and clinical evidence points towards the existence of a specific myocardial involvement in cystic fibrosis. Multi-modality cardiac imaging, especially recent echocardiographic techniques, evidenced diastolic and/or systolic ventricular dysfunction in cystic fibrosis leading to the concept of a cystic fibrosis-related cardiomyopathy. Hypoxemia and inflammation are among the most important factors for heart involvement in cystic fibrosis. Cystic Fibrosis Transmembrane Regulator was found to be involved in the regulation of cardiomyocyte contraction and may also account for cystic fibrosis-related myocardial dysfunction. This review, mainly focused on echocardiographic studies, seeks to synthesize the existing literature for and against the existence of heart involvement in cystic fibrosis, its mechanisms and prognostic implications. Careful investigation of the heart function may be helpful for risk stratification and therapeutic decisions in patients with cystic fibrosis. Copyright © 2016 Elsevier Ltd. All rights reserved.

  6. Controlled longitudinal study of bone mass accrual in children and adolescents with cystic fibrosis.

    PubMed

    Buntain, H M; Schluter, P J; Bell, S C; Greer, R M; Wong, J C H; Batch, J; Lewindon, P; Wainwright, C E

    2006-02-01

    A study was undertaken to observe the gains in bone mass in children and adolescents with cystic fibrosis (CF) over 24 months and to examine the relationship between areal bone mineral density (aBMD) and associated clinical parameters including physical activity, nutrition, and 25-hydroxyvitamin D (25OHD). Areal BMD of the total body (TB), lumbar spine (LS), and total femoral neck (FNt) were repeatedly measured in 85 subjects aged 5-18 years with CF and 100 age and sex matched controls over 2 years. At each visit anthropometric variables, nutritional parameters, pubertal status, disease severity, physical activity, dietary calcium, caloric intake, and serum 25OHD were assessed and related to aBMD. After adjusting for age, sex, and height Z-score, gains in LS aBMD in children (5-10 years) and TB and FNt aBMD in adolescents (11-18 years) with CF were significantly less than in controls. Lean tissue mass was significantly associated with TB and LS aBMD gains in children and adolescents and explained a significant proportion of the aBMD deficit observed. Lung function parameters were significantly associated with aBMD gains in adolescents with CF. Inadequate bone mass accrual during childhood and adolescence contributes to the low bone mass observed in adults with CF. Accounting for the height discrepancy which is frequently observed in those with CF, in addition to age and sex, is important when assessing low bone mass in children and adolescents with CF. To optimise an individual's potential to acquire maximal bone mass, it is necessary to maximise nutritional status and limit the progression of chronic suppurative lung disease.

  7. Pregnancy outcome in women with cystic fibrosis-related diabetes.

    PubMed

    Reynaud, Quitterie; Poupon-Bourdy, Stéphanie; Rabilloud, Muriel; Al Mufti, Lina; Rousset Jablonski, Christine; Lemonnier, Lydie; Nove-Josserand, Raphaële; Touzet, Sandrine; Durieu, Isabelle

    2017-10-01

    With increasing life expectancy, more women with cystic fibrosis and diabetes mellitus become pregnant. We investigated how pre-gestational diabetes (cystic fibrosis-related diabetes) influenced pregnancy outcome and the clinical status of these women. We analyzed all pregnancies reported to the French cystic fibrosis registry between 2001 and 2012, and compared forced expiratory volume (FEV 1 ) and body mass index before and after pregnancy in women with and without pre-gestational diabetes having a first delivery. A total 249 women delivered 314 infants. Among these, 189 women had a first delivery and 29 of these had pre-gestational diabetes. There was a trend towards a higher rate of assisted conception among diabetic women (53.8%) than non-diabetic women (34.5%, p = 0.06), and the rate of cesarean section was significantly higher in diabetic women (48% vs. 21.4%, p = 0.005). The rate of preterm birth and mean infant birthweight did not differ significantly between diabetic and non-diabetic women. Forced expiratory volume before pregnancy was significantly lower in the diabetic group. The decline in forced expiratory volume and body mass index following pregnancy did not differ between the women with and those without pre-gestational diabetes. Pre-gestational diabetes in women with cystic fibrosis is associated with a higher rate of cesarean section but does not seem to have a clinically significant impact on fetal growth or preterm delivery. The changes in maternal pulmonary and nutritional status following pregnancy in women with cystic fibrosis were not influenced by pre-gestational diabetes. © 2017 Nordic Federation of Societies of Obstetrics and Gynecology.

  8. Sealing of the cystic and appendix arteries with monopolar electrocautery during laparoscopic combined cholecystectomy and appendectomy.

    PubMed

    Liu, Gui-Bao; Mao, Yuan-Yuan; Yang, Chang-Ping; Cao, Jin-Lin

    2018-03-01

    The best method to ligate the arteries during laparoscopic cholecystectomy or appendectomy remains controversy. The aim of this study is to introduce a new approach during laparoscopic combined cholecystectomy and appendectomy using a monopolar electrocautery to seal the cystic and appendix arteries. We retrospectively reviewed data from 57 patients who underwent laparoscopic combined cholecystectomy and appendectomy between December 2006 and June 2016. Each laparoscopic combined cholecystectomy and appendectomy was performed by coagulating and sealing the cystic and appendix arteries. Absorbable clip or coils were then used to ligate the proximal of cystic duct and the stump of appendix. The other side of the cystic duct and appendix which subsequently were to be removed from abdomen were used titanium clips or silk ligature. Of the 57 patients, 3 patients (5.3%) were converted to open surgery due to severe abdominal adhesions or gallbladder perforation. The mean operative time was 56 minutes (range, 40-80 minutes). Mean blood loss was 12 mL (range, 5-120 mL), and the mean postoperative hospital stay was 3.0 days (range, 2-5 days). No postoperative bleeding, biliary leakage, infection, or mortality occurred. Monopolar electrocautery to seal the cystic and appendix arteries is a safe, effective, and economical surgical procedure during laparoscopic combined cholecystectomy and appendectomy. Further randomized controlled clinical trials are required to validate our findings.

  9. Sealing of the cystic and appendix arteries with monopolar electrocautery during laparoscopic combined cholecystectomy and appendectomy

    PubMed Central

    Liu, Gui-Bao; Mao, Yuan-Yuan; Yang, Chang-Ping; Cao, Jin-Lin

    2018-01-01

    Abstract The best method to ligate the arteries during laparoscopic cholecystectomy or appendectomy remains controversy. The aim of this study is to introduce a new approach during laparoscopic combined cholecystectomy and appendectomy using a monopolar electrocautery to seal the cystic and appendix arteries. We retrospectively reviewed data from 57 patients who underwent laparoscopic combined cholecystectomy and appendectomy between December 2006 and June 2016. Each laparoscopic combined cholecystectomy and appendectomy was performed by coagulating and sealing the cystic and appendix arteries. Absorbable clip or coils were then used to ligate the proximal of cystic duct and the stump of appendix. The other side of the cystic duct and appendix which subsequently were to be removed from abdomen were used titanium clips or silk ligature. Of the 57 patients, 3 patients (5.3%) were converted to open surgery due to severe abdominal adhesions or gallbladder perforation. The mean operative time was 56 minutes (range, 40–80 minutes). Mean blood loss was 12 mL (range, 5–120 mL), and the mean postoperative hospital stay was 3.0 days (range, 2–5 days). No postoperative bleeding, biliary leakage, infection, or mortality occurred. Monopolar electrocautery to seal the cystic and appendix arteries is a safe, effective, and economical surgical procedure during laparoscopic combined cholecystectomy and appendectomy. Further randomized controlled clinical trials are required to validate our findings. PMID:29595660

  10. Cystic echinococcosis: a disease mimicking cancer in a non-endemic country report of two cases.

    PubMed

    Comrnejo-Juárez, P; Espinosa-Altamirano, A; Ibarra-del-Río, M; Pacheco-Bravo, I; Volkow-Fernández, P

    2013-03-01

    Echinococcosis is a parasitic disease that involves dogs as definitive host and sheep as intermediate host. Humans become infected incidentally through fecal-oral contact, particularly in the course of playful and close contact with an infected dog. Mexico is considered a region that is virtually free of cystic echinoccocosis. This manuscript describes two cases that were referred to a tertiary-care oncology hospital with a diagnosis of cancer. In one case, the presumptive diagnosis was liver cancer because abdominal ultrasonography revealed a low-density mass in the right hepatic lobe. Drainage was performed and cytologic examination of the fluid showed multiple Echinococcus cyst as well as prostoscolex. The case was resolved with percutaneous drainage and administration of albendazole for two months. In the second case, the patient was referred with a diagnosis of disseminated cervical cancer A cyst was identified in the upper right lung lobe; a diagnostic puncture was performed showing an Echinococcus cyst. This resolved solely with two months of albendazole administration.

  11. Adenoid Cystic Carcinoma of the Trachea Resulting in Fatal Asphyxia.

    PubMed

    Huston, Butch; Froloff, Victor; Mills, Kelly; McGee, Michael

    2017-01-01

    Primary malignant tumors of the trachea are uncommon. The authors report a case of a 72-year-old female who died from asphyxia due to an undiagnosed obstructing adenoid cystic carcinoma of the trachea. The decedent became unresponsive while eating cereal and was pronounced upon arrival at the local hospital. The autopsy revealed a near occlusive tumor mass just superior to the bifurcation of the distal trachea. There was no evidence of aspiration. The death was the result of asphyxia due to obstruction of the trachea by an adenoid cystic carcinoma. This case demonstrates that an obstructive tumor mass may remain undiagnosed until an obstructive episode results in a sudden death. © 2016 American Academy of Forensic Sciences.

  12. Early detection of cystic fibrosis lung disease: multiple‐breath washout versus raised volume tests

    PubMed Central

    Lum, Sooky; Gustafsson, Per; Ljungberg, Henrik; Hülskamp, Georg; Bush, Andrew; Carr, Siobhán B; Castle, Rosemary; Hoo, Ah‐fong; Price, John; Ranganathan, Sarath; Stroobant, John; Wade, Angie; Wallis, Colin; Wyatt, Hilary; Stocks, Janet

    2007-01-01

    Background Lung clearance index (LCI), a measure of ventilation inhomogeneity derived from the multiple‐breath inert gas washout (MBW) technique, has been shown to detect abnormal lung function more readily than spirometry in preschool children with cystic fibrosis, but whether this holds true during infancy is unknown. Objectives To compare the extent to which parameters derived from the MBW and the raised lung volume rapid thoraco–abdominal compression (RVRTC) techniques identify diminished airway function in infants with cystic fibrosis when compared with healthy controls. Methods Measurements were performed during quiet sleep, with the tidal breathing MBW technique being performed before the forced expiratory manoeuvres. Results Measurements were obtained in 39 infants with cystic fibrosis (mean (SD) age 41.4 (22.0) weeks) and 21 controls (37.0 (15.1) weeks). Infants with cystic fibrosis had a significantly higher respiratory rate (38 (10) vs 32 (5) bpm) and LCI (8.4 (1.5) vs 7.2 (0.3)), and significantly lower values for all forced expiratory flow‐volume parameters compared with controls. Girls with cystic fibrosis had significantly lower forced expiratory volume (FEV0.5 and FEF25–75 ) than boys (mean (95% CI girls–boys): –1.2 (–2.1 to −0.3) for FEV0.5 Z score; FEF25–75: –1.2 (–2.2 to −0.15)). When using both the MBW and RVRTC techniques, abnormalities were detected in 72% of the infants with cystic fibrosis, with abnormalities detected in 41% using both techniques and a further 15% by each of the two tests performed. Conclusions These findings support the view that inflammatory and/or structural changes in the airways of children with cystic fibrosis start early in life, and have important implications regarding early detection and interventions. Monitoring of early lung disease and functional status in infants and young children with cystic fibrosis may be enhanced by using both MBW and the RVRTC. PMID:17121870

  13. Diagnostic ultrasonography in cattle with abdominal fat necrosis.

    PubMed

    Tharwat, Mohamed; Buczinski, Sébastien

    2012-01-01

    This study describes the ultrasonographic findings in 14 cows with abdominal fat necrosis. Ultrasonography of the abdomen revealed the presence of heterogeneous hyperechoic masses and hyperechoic omentum with localized masses floating in a hypoechoic peritoneal fluid. A hyperechogenic rim was imaged around both kidneys. The intestines were coated with hyperechoic capsules and the intestinal lumens were constricted. Ultrasonographic examination of the pancreatic parenchyma showed an overall increased echogenicity which was homogenously distributed in 3 cases. A diagnosis of abdominal fat necrosis was made with ultrasound-guided biopsy of the echogenic masses, and thereafter at postmortem examination. Results from this study demonstrate the efficacy of ultrasonography as an imaging modality for antemortem diagnosis of abdominal lipomatosis in cattle. To the authors' knowledge, this study is the first that illustrates ultrasonographic findings in cattle affected with abdominal lipomatosis.

  14. Controlled longitudinal study of bone mass accrual in children and adolescents with cystic fibrosis

    PubMed Central

    Buntain, H M; Schluter, P J; Bell, S C; Greer, R M; Wong, J C H; Batch, J; Lewindon, P; Wainwright, C E

    2006-01-01

    Background A study was undertaken to observe the gains in bone mass in children and adolescents with cystic fibrosis (CF) over 24 months and to examine the relationship between areal bone mineral density (aBMD) and associated clinical parameters including physical activity, nutrition, and 25‐hydroxyvitamin D (25OHD). Methods Areal BMD of the total body (TB), lumbar spine (LS), and total femoral neck (FNt) were repeatedly measured in 85 subjects aged 5–18 years with CF and 100 age and sex matched controls over 2 years. At each visit anthropometric variables, nutritional parameters, pubertal status, disease severity, physical activity, dietary calcium, caloric intake, and serum 25OHD were assessed and related to aBMD. Results After adjusting for age, sex, and height Z‐score, gains in LS aBMD in children (5–10 years) and TB and FNt aBMD in adolescents (11–18 years) with CF were significantly less than in controls. Lean tissue mass was significantly associated with TB and LS aBMD gains in children and adolescents and explained a significant proportion of the aBMD deficit observed. Lung function parameters were significantly associated with aBMD gains in adolescents with CF. Conclusions Inadequate bone mass accrual during childhood and adolescence contributes to the low bone mass observed in adults with CF. Accounting for the height discrepancy which is frequently observed in those with CF, in addition to age and sex, is important when assessing low bone mass in children and adolescents with CF. To optimise an individual's potential to acquire maximal bone mass, it is necessary to maximise nutritional status and limit the progression of chronic suppurative lung disease. PMID:16384878

  15. [Multiple choristoma and a myelolipoma in a Sheltie].

    PubMed

    Cubillos, Claudette C; Köhler, Claudia; Fromme, Vivian; Leitner, Susanne; Hauber, Elke; Schwede, Maartje; Alef, Michaele; Kiefer, Ingmar

    2018-06-01

    An 11-year-old female Sheltie was presented with inappetence and a progressive increase in abdominal distention. Abdominal ultrasound revealed a large cystic mass in the midabdomen and cystic lesions in the right liver lobe and in the caudal pole of the left kidney. Histopathologic examination of the resected tissue revealed a myelolipoma of the spleen, dispersed splenic tissue in the liver and dispersed uterine and salpinx tissues in the kidney. This report describes the clinical, ultrasonographic and computed tomographic features and the results of histopathology. In addition to the abnormally large and cystic myelolipoma of the spleen, the great number of choristomas is remarkable, which has not previously been documented in a dog. Schattauer GmbH.

  16. [Four Cases Report on Primary Lung Adenoid Cystic Carcinoma].

    PubMed

    He, Xilan; Chen, Jianhua

    2017-11-20

    Lung adenoid cystic carcinoma is a kind of rare lung cancer. Diagnosis and treatment is not enough understandable for them. We collected and analyzed 4 cases of lung adenoid cystic carcinoma for broadening the sight of this disease. Retrospectively analysed the 4 cases we collected from Hunan Cancer Hospital Between January 2012 and December 2016. We depicted the pathology, immunohistochemical, epidermal growth factor receptor (EGFR) mutation and anaplastic lymphoma kinase (ALK) arrangement in these cases. And the methods of the diagnosis and treatment were analyzed. Lung adenoid cystic carcinoma is usually located in the airway, EGFR mutation and ALK arrangement is rare in this disease. Generally the metastasis of the lung cancer occurred in the advanced stage. The prognosis is good if the mass could be resected completely. Diagnosis of the lung adenoid cystic carcinoma depends on pathological experiments, surgery is the main treatment in the early stage, radiotherapy and chemotherapy is an advisable therapy in the advanced stage. And the prognosis of this kind of lung cancer is better than small cell lung cancer and non-small cell lung cancer.

  17. Abdominal hernias: Radiological features

    PubMed Central

    Lassandro, Francesco; Iasiello, Francesca; Pizza, Nunzia Luisa; Valente, Tullio; Stefano, Maria Luisa Mangoni di Santo; Grassi, Roberto; Muto, Roberto

    2011-01-01

    Abdominal wall hernias are common diseases of the abdomen with a global incidence approximately 4%-5%. They are distinguished in external, diaphragmatic and internal hernias on the basis of their localisation. Groin hernias are the most common with a prevalence of 75%, followed by femoral (15%) and umbilical (8%). There is a higher prevalence in males (M:F, 8:1). Diagnosis is usually made on physical examination. However, clinical diagnosis may be difficult, especially in patients with obesity, pain or abdominal wall scarring. In these cases, abdominal imaging may be the first clue to the correct diagnosis and to confirm suspected complications. Different imaging modalities are used: conventional radiographs or barium studies, ultrasonography and Computed Tomography. Imaging modalities can aid in the differential diagnosis of palpable abdominal wall masses and can help to define hernial contents such as fatty tissue, bowel, other organs or fluid. This work focuses on the main radiological findings of abdominal herniations. PMID:21860678

  18. Effect of abdominal resistance exercise on abdominal subcutaneous fat of obese women: a randomized controlled trial using ultrasound imaging assessments.

    PubMed

    Kordi, Ramin; Dehghani, Saeed; Noormohammadpour, Pardis; Rostami, Mohsen; Mansournia, Mohammad Ali

    2015-01-01

    The aim of this study was to compare the effect of diet and an abdominal resistance training program to diet alone on abdominal subcutaneous fat thickness and waist circumference of overweight and obese women. This randomized clinical trial included 40 overweight and obese women randomly divided into 2 groups: diet only and diet combined with 12 weeks of abdominal resistance training. Waist and hip circumferences and abdominal skin folds of the subjects were measured at the beginning and 12 weeks after the interventions. In addition, abdominal subcutaneous fat thickness of the subjects was measured using ultrasonography. Percentage body fat and lean body mass of all the subjects were also measured using a bioelectric impedance device. After 12 weeks of intervention, the weight of participants in both groups decreased; but the difference between the 2 groups was not significant (P = .45). Similarly, other variables including abdominal subcutaneous fat, waist circumference, hip circumference, body mass index, body fat percentage, and skin fold thickness were reduced in both groups; but there were no significant differences between the groups. This study found that abdominal resistance training besides diet did not reduce abdominal subcutaneous fat thickness compared to diet alone in overweight or obese women. Copyright © 2015 National University of Health Sciences. Published by Elsevier Inc. All rights reserved.

  19. Changes in abdominal obesity in Chilean university students stratified by body mass index.

    PubMed

    Cossio-Bolaños, Marco; Vilchez-Avaca, Catalina; Contreras-Mellado, Victor; Andruske, Cynthia Lee; Gómez-Campos, Rossana

    2016-01-13

    Studies based on Body Mass Index (BMI) and waist circumference (WC) are generally used to examine the prevalence and tendency of overweight and obesity. These studies help determine the socioeconomic development of a country and improve public health policies. Therefore, the goal of this research was to determine the trend of change in abdominal obesity of Chilean university students according to the Body Mass Index (BMI) measured in intervals of three and six years. For this study, a total of 1598 students of both sexes ranging in age from 18 to 26 from a Chilean university were evaluated. Students were assessed commencing in 2007 (372 males and 315 females), 2010 (250 males and 330 females), and ending in 2013 (153 males and 178 females). During the three transversal assessments, weight, height, and waist circumference were evaluated. BMI was calculated for both sexes. No significant differences were found in age and BMI during the three years evaluated (2007, 2010, and 2013). In 2013, waist circumference (WC) increased significantly (p < 0.001 for both sexes). Moreover, in 2013, in all the percentiles evaluated, high values of WC were compared in relation to previous years. Furthermore, in 2013, in all four BMI categories (underweight, normal, overweight, and obese), the university students showed significant increases in WC (Females: p = 0.004; Males: p = 0.035) whereas in 2007 and 2010, the values remained relatively stable. BMI remained constant during 2007, 2010, and 2013. However, the university students of both sexes showed greater risk of abdominal obesity as a result of increased WC in 2013.

  20. Abdominal elephantiasis: a case report.

    PubMed

    Hanna, Dominique; Cloutier, Richard; Lapointe, Roch; Desgagné, Antoine

    2004-01-01

    Elephantiasis is a well-known condition in dermatology usually affecting the legs and external genitalia. It is characterized by chronic inflammation and obstruction of the lymphatic channels and by hypertrophy of the skin and subcutaneous tissues. The etiology is either idiopathic or caused by a variety of conditions such as chronic filarial disease, leprosy, leishmaniasis, and chronic recurrent cellulites. Elephantiasis of the abdominal wall is very rare. A complete review of the English and French literature showed only two cases reported in 1966 and 1973, respectively. We report a third case of abdominal elephantiasis and we briefly review this entity. We present the case of a 51-year-old woman who had progressively developed an enormous pediculated abdominal mass hanging down her knees. The skin was thickened, hyperpigmented, and fissured. She had a history of multiple abdominal cellulites. She underwent an abdominal lipectomy. Histopathology of the specimen confirmed the diagnosis of abdominal elephantiasis. Abdominal elephantiasis is a rare disease that represents end-stage failure of lymph drainage. Lipectomy should be considered in the management of this condition.

  1. Cystic fibrosis.

    PubMed

    Elborn, J Stuart

    2016-11-19

    Cystic fibrosis is a common life-limiting autosomal recessive genetic disorder, with highest prevalence in Europe, North America, and Australia. The disease is caused by mutation of a gene that encodes a chloride-conducting transmembrane channel called the cystic fibrosis transmembrane conductance regulator (CFTR), which regulates anion transport and mucociliary clearance in the airways. Functional failure of CFTR results in mucus retention and chronic infection and subsequently in local airway inflammation that is harmful to the lungs. CFTR dysfunction mainly affects epithelial cells, although there is evidence of a role in immune cells. Cystic fibrosis affects several body systems, and morbidity and mortality is mostly caused by bronchiectasis, small airways obstruction, and progressive respiratory impairment. Important comorbidities caused by epithelial cell dysfunction occur in the pancreas (malabsorption), liver (biliary cirrhosis), sweat glands (heat shock), and vas deferens (infertility). The development and delivery of drugs that improve the clearance of mucus from the lungs and treat the consequent infection, in combination with correction of pancreatic insufficiency and undernutrition by multidisciplinary teams, have resulted in remarkable improvements in quality of life and clinical outcomes in patients with cystic fibrosis, with median life expectancy now older than 40 years. Innovative and transformational therapies that target the basic defect in cystic fibrosis have recently been developed and are effective in improving lung function and reducing pulmonary exacerbations. Further small molecule and gene-based therapies are being developed to restore CFTR function; these therapies promise to be disease modifying and to improve the lives of people with cystic fibrosis. Copyright © 2016 Elsevier Ltd. All rights reserved.

  2. Adenoid cystic carcinoma of cervix: two cases report and review of the literature.

    PubMed

    Benhayoune, Khadija; El Fatemi, Hinde; Bannani, Abdelaziz; Melhouf, Abdelilah; Harmouch, Toufik

    2015-01-01

    Adenoid cystic carcinoma of the cervix is a rare and aggressive tumor with fatal outcome. In this paper we report two cases of primary adenoid cystic carcinoma and a review of literature. A 80 years old woman, admitted to our hospital with postmenopausal bleeding and hydrorrhea. Gynealogical examination showed a cervical stenotic with the presence of a tumor processus. Biopsy of cervical growth was done. 80-year-old woman presented with vaginal bleeding with pelvic pain. Physical examination revealed a friable mass in the cervix. Incisional biopsy was performed. In the both cases the diagnosis of adenoid cystic carcinoma of the cercix was confirmed. Adenoid cystic carcinoma of the cervix is clinically and radiologically similar to other tumors of the cervix but the diagnosis can only be made by histological examination.

  3. Adenoid cystic carcinoma of cervix: two cases report and review of the literature

    PubMed Central

    Benhayoune, Khadija; El Fatemi, Hinde; Bannani, Abdelaziz; Melhouf, Abdelilah; Harmouch, Toufik

    2015-01-01

    Adenoid cystic carcinoma of the cervix is a rare and aggressive tumor with fatal outcome. In this paper we report two cases of primary adenoid cystic carcinoma and a review of literature. A 80 years old woman, admitted to our hospital with postmenopausal bleeding and hydrorrhea. Gynealogical examination showed a cervical stenotic with the presence of a tumor processus. Biopsy of cervical growth was done. 80-year-old woman presented with vaginal bleeding with pelvic pain. Physical examination revealed a friable mass in the cervix. Incisional biopsy was performed. In the both cases the diagnosis of adenoid cystic carcinoma of the cercix was confirmed. Adenoid cystic carcinoma of the cervix is clinically and radiologically similar to other tumors of the cervix but the diagnosis can only be made by histological examination. PMID:26090035

  4. Nasopharyngeal Carcinoma with Cystic Cervical Metastasis Masquerading as Branchial Cleft Cyst: A Potential Pitfall in Diagnosis and Management.

    PubMed

    Sai-Guan, Lum; Min-Han, Kong; Kah-Wai, Ngan; Mohamad-Yunus, Mohd-Razif

    2017-03-01

    Most metastatic lymph nodes from head and neck malignancy are solid. Cystic nodes are found in 33% - 61% of carcinomas arise from Waldeyer's ring, of which only 1.8% - 8% originate are from the nasopharynx. Some cystic cervical metastases were initially presumed to be branchial cleft cyst. This case report aims to highlight the unusual presentation of cystic cervical metastasis secondary to nasopharyngeal carcinoma in a young adult. The histopathology, radiological features and management strategy were discussed. A 36-year-old man presented with a solitary cystic cervical swelling, initially diagnosed as branchial cleft cyst. Fine needle aspiration yielded 18 ml of straw-coloured fluid. During cytological examination no atypical cells were observed. Computed tomography of the neck showed a heterogeneous mass with multiseptation medial to the sternocleidomastoid muscle. Histopathological examination of the mass, post excision, revealed a metastatic lymph node. A suspicious mucosal lesion at the nasopharynx was detected after repeated thorough head and neck examinations and the biopsy result confirmed undifferentiated nasopharyngeal carcinoma. Cystic cervical metastasis may occur in young patients under 40 years. The primary tumour may not be obvious during initial presentation because it mimicks benign branchial cleft cyst clinically. Retrospective review of the computed tomography images revealed features that were not characteristic of simple branchial cleft cyst. The inadequacy of assessment and interpretation had lead to the error in diagnosis and subsequent management. Metastatic head and neck lesion must be considered in a young adult with a cystic neck mass.

  5. Nutrient Status of Adults with Cystic Fibrosis

    PubMed Central

    GORDON, CATHERINE M.; ANDERSON, ELLEN J.; HERLYN, KAREN; HUBBARD, JANE L.; PIZZO, ANGELA; GELBARD, RONDI; LAPEY, ALLEN; MERKEL, PETER A.

    2011-01-01

    Nutrition is thought to influence disease status in patients with cystic fibrosis (CF). This cross-sectional study sought to evaluate nutrient intake and anthropometric data from 64 adult outpatients with cystic fibrosis. Nutrient intake from food and supplements was compared with the Dietary Reference Intakes for 16 nutrients and outcomes influenced by nutritional status. Attention was given to vitamin D and calcium given potential skeletal implications due to cystic fibrosis. Measurements included weight, height, body composition, pulmonary function, and serum metabolic parameters. Participants were interviewed about dietary intake, supplement use, pulmonary function, sunlight exposure, and pain. The participants’ mean body mass index (±standard deviation) was 21.8±4.9 and pulmonary function tests were normal. Seventy-eight percent used pancreatic enzyme replacement for malabsorption. Vitamin D deficiency [25-hydroxyvitamin D (25OHD)<37.5 nmol/L] was common: 25 (39%) were deficient despite adequate vitamin D intake. Lipid profiles were normal in the majority, even though total and saturated fat consumption represented 33.0% and 16.8% of energy intake, respectively. Reported protein intake represented 16.9% of total energy intake (range 10%–25%). For several nutrients, including vitamin D and calcium, intake from food and supplements in many participants exceeded recommended Tolerable Upper Intake Levels. Among adults with cystic fibrosis, vitamin D deficiency was common despite reported adequate intake, and lipid profiles were normal despite a relatively high fat intake. Mean protein consumption was adequate, but the range of intake was concerning, as both inadequate or excessive intake may have deleterious skeletal effects. These findings call into question the applicability of established nutrient thresholds for patients with cystic fibrosis. PMID:18060897

  6. Intra-abdominal fungal pseudomycetoma in two cats.

    PubMed

    Bianchi, Matheus V; Laisse, Cláudio J M; Vargas, Thainã P; Wouters, Flademir; Boabaid, Fabiana M; Pavarini, Saulo P; Ferreiro, Laerte; Driemeier, David

    Pseudomycetomas are deep cutaneous to subcutaneous lesions caused by Microsporum canis mainly described in Persian cats, with few reports of intra-abdominal location. This report describes the clinical signs and lesions of intra-abdominal pseudomycetomas caused by M. canis in two Persian cats. Two Persian cats with a history of previous laparotomy (ovariohysterectomy and nephrostomy) and fecal impaction were examined. Cat #1 was euthanized and subjected to necropsy, histopathology and mycological evaluation. Cat #2 presented with chronic dermatophytosis, and an intra-abdominal mass, that was subjected to histopathology evaluation. Cat #1 presented at necropsy a white-grayish, firm mass (6cm×3.5cm×2.8cm) in the uterine cervix. Cat #2 presented a firm whitish mass (6.5cm×1.5cm×0.5cm) located close to the left kidney. Histologically, both masses contained multifocal granules with hyphae and spores surrounded by Splendore-Hoeppli reaction, with a pyogranulomatous inflammatory infiltrate and fibrous connective tissue proliferation in the periphery. Hyphae and spores exhibited marked Grocott and periodic acid-Schiff staining. M. canis was identified by fungal isolation in cat #1. Pseudomycetoma should be considered as a differential diagnosis in cats, especially in Persian cats presenting with an intra-abdominal mass. Entrance of the agent into the cavity can occur during laparotomy. Copyright © 2016 Asociación Española de Micología. Publicado por Elsevier España, S.L.U. All rights reserved.

  7. Peritoneal mucinous cystadenocarcinoma of probable urachal origin: a challenging diagnosis

    PubMed Central

    Gore, D M; Bloch, S; Waller, W; Cohen, P

    2006-01-01

    This report describes the case of a mucinous cystadenocarcinoma of probable urachal origin that presented with mass effect, precipitating deep venous thrombosis and pulmonary embolism. The patient presented with acute symptoms of leg swelling, pain and dyspnoea, and a vague awareness of lower abdominal distension. Computer tomography showed a cystic mass closely related to the anterior abdominal wall and the superior aspect of the bladder. A 1500 cm3 cyst adherent to the dome of the urinary bladder was resected on laparotomy. Partial cystectomy was not carried out in the belief that the cyst represented a benign lesion. Subsequent imaging has shown cystic changes in the anterior bladder wall, and the patient has been referred for partial cystectomy. PMID:17021133

  8. Spectrum of magnetic resonance imaging findings in pancreatic and other abdominal manifestations of Von Hippel-Lindau disease in a series of 23 patients: a pictorial review.

    PubMed

    Graziani, Rossella; Mautone, Simona; Vigo, Mario; Manfredi, Riccardo; Opocher, Giuseppe; Falconi, Massimo

    2014-01-10

    Von Hippel Lindau disease is a rare autosomal dominantly inherited multisystem disorder characterized by development of benign and malignant tumors. The abdominal manifestation of the syndrome are protean. Magnetic resonance plays an important role in identification of abdominal abnormalities and follow-up of lesions. To describe magnetic resonance imaging findings and patterns of pancreatic and other principal abdominal manifestations in a series of von Hippel-Lindau (VHL) disease patients and to review literature. We retrospectively reviewed abdominal magnetic resonance studies performed in 23 patients (10 males, 13 females) diagnosed of VHL. In all examined patients abdominal involvement was present. The pancreatic imaging findings detected were: unilocular cystic lesions (6/23: 26.1%); serous cystadenomas (11/23: 47.8%), including diffuse lesions (8/23: 34.8%); solid neuroendocrine tumors (8/23: 34.8%); cystic neuroendocrine tumors (1/23: 4.3%). The renal findings detected were: simple renal cysts (18/23: 78.3%); complex renal cysts (13/23: 56.5%), including benign lesions (10/23: 43.5%) and malignant lesions (3/23: 13.0%); renal carcinomas (11/23: 47.8%) and 5 of these (45.5%) were multiple and bilateral. Five patients (21.7%) presented pheochromocytoma (4 of these were bilateral; 80.0%) and 1 patient (4.3%) presented cystadenoma of the epididymis. In VHL disease patients, magnetic resonance imaging plays an essential role in the identification of pancreatic and other abdominal lesions, in their follow-up, in the screening of asymptomatic gene carriers, and in their long-term surveillance.

  9. Longitudinal cystic fibrosis care.

    PubMed

    Antunovic, S S; Lukac, M; Vujovic, D

    2013-01-01

    Cystic fibrosis is a complex disease entity that presents considerable lifelong challenges. Implementation of medical and surgical treatment options involves multisystem interventions to prevent and treat lung and gastrointestinal manifestations of cystic fibrosis and associated comorbidities. From birth through adulthood, cystic fibrosis care entails a longitudinal regimen aimed at achieving relief of disease symptoms and enhanced life expectancy. With increased knowledge of the molecular behavior of the cystic fibrosis transmembrane conductance regulator (CFTR) in health and disease, clinical practice has been enriched by the prospect of novel strategies, including mutation-specific drug and gene therapy targeting restoration of corrupted transepithelial ion transport. Emerging paradigms of comprehensive care increasingly enable personalized solutions to address the root cause of disease-transforming management options for individuals with cystic fibrosis.

  10. Spontaneous extraskeletal osteosarcoma with various histological growth patterns in the abdominal wall of an ICR mouse

    PubMed Central

    Ito, Tsuyoshi; Katoh, Yoshitaka; Shimada, Yuko; Ohnuma-Koyama, Aya; Takahashi, Naofumi; Kuwahara, Maki; Harada, Takanori

    2015-01-01

    Extraskeletal osteosarcoma is extremely rare in mice. This case report demonstrates a spontaneous murine extraskeletal osteosarcoma that exhibited various histological growth patterns in an ICR mouse. At necropsy, the tumor mass was located in the abdominal wall and was 45 × 30 × 25 mm in size. Histopathologically, the tumor showed the following four growth patterns: a solid pattern of polygonal cells embedded in an osteoid eosinophilic matrix with calcification, an irregular sheet pattern of short spindle cells accompanying some eosinophilic multinucleated cells, a fascicular pattern of spindle cells and a cystic pattern lined by short spindle cells. Immunohistochemically, most of the tumor cells were positive for vimentin, proliferating cell nuclear antigen and osterix. The multinucleated cells mentioned above were desmin positive and were regarded as regenerative striated muscles but not tumor cells. Since no clear continuity with normal bone tissues was observed, the tumor was diagnosed as an “extraskeletal osteosarcoma.” PMID:26989300

  11. Cystic fibrosis.

    PubMed

    O'Sullivan, Brian P; Freedman, Steven D

    2009-05-30

    Cystic fibrosis is the most common lethal genetic disease in white populations. The outlook for patients with the disease has improved steadily over many years, largely as a result of earlier diagnosis, more aggressive therapy, and provision of care in specialised centres. Researchers now have a more complete understanding of the molecular-biological defect that underlies cystic fibrosis, which is leading to new approaches to treatment. One of these treatments, hypertonic saline, is already in use, whereas others are in advanced stages of development. We review clinical care for cystic fibrosis and discuss recent advances in the understanding of its pathogenesis, implementation of screening of neonates, and development of therapies aimed at treating the basic defect.

  12. [Abdominal wall actinomycosis. A report of a case].

    PubMed

    Rojas Pérez-Ezquerra, Beatriz; Guardia-Dodorico, Lorena; Arribas-Marco, Teresa; Ania-Lahuerta, Aldonza; González Ballano, Isabel; Chipana-Salinas, Margot; Carazo-Hernández, Belén

    2015-01-01

    Abdominal wall Actinomycosis is a rare disease associated with the use of intrauterine device and as a complication of abdominal surgery. Diagnosis is difficult because it is unusual and behaves like a malignant neoplasm. A case report is presented of a patient who had used an intrauterine device for four years and developed a stony tumour in the abdominal wall associated with a set of symptoms that, clinically and radiologically, was simulating a peritoneal carcinomatosis associated with paraneoplastic syndrome, even in the course of an exploratory laparotomy. The patient attended our hospital with a two-month history of abdominal pain and symptoms that mimic a paraneoplastic syndrome. The diagnosis of abdominal actinomycosis was suspected by the finding of the microorganism in cervical cytology together with other cultures and Actinomyces negative in pathological studies, confirming the suspicion of a complete cure with empirical treatment with penicillin. Actinomycosis should be considered in patients with pelvic mass or abdominal wall mass that mimics a malignancy. Antibiotic therapy is the first treatment choice and makes a more invasive surgical management unnecessary. Copyright © 2015 Academia Mexicana de Cirugía A.C. Published by Masson Doyma México S.A. All rights reserved.

  13. Characteristics of Resting Metabolic Rate in Critically Ill, Mechanically Ventilated Adults With Cystic Fibrosis.

    PubMed

    Frankenfield, David C; Ashcraft, Christine M; Drasher, Tammy L; Reid, Elizabeth K; Vender, Robert L

    2017-05-01

    Critically ill patients with cystic fibrosis may be especially sensitive to the negative consequences of overfeeding and underfeeding, yet there is almost no information available about the energy needs of these patients. The purpose of this study was to characterize the metabolic rate of critically ill adult patients with cystic fibrosis requiring mechanical ventilation. This was an observational study in which the resting metabolic rate, oxygen consumption, and carbon dioxide production of adult patients with cystic fibrosis requiring critical care, sedation, and mechanical ventilation were measured with indirect calorimetry. This group was compared with a cohort of adult critical care patients without cystic fibrosis. Twelve patients with cystic fibrosis were identified and measured. These were compared with a control group of 25 critically ill patients. Both groups were underweight (body mass index, 17.4 ± 4.0 kg/m 2 in cystic fibrosis and 18.4 ± 2.3 kg/m 2 in control). Adjusting for differences in age, sex, height, and weight, there was no difference in resting metabolic rate between the cystic fibrosis and control groups (1702 ± 193 vs 1642 ± 194 kcal/d, P = .388). Measured resting metabolic rate matched predicted values 58% of the time in cystic fibrosis and 60% of the time in control. The resting metabolic rate of sedated adult patients with cystic fibrosis being assisted with mechanical ventilation is not different from that of adult critical care patients without cystic fibrosis. In both these underweight groups, accurate prediction of resting metabolic rate is difficult to obtain.

  14. Cystic renal cell carcinoma carries an excellent prognosis regardless of tumor size.

    PubMed

    Winters, Brian R; Gore, John L; Holt, Sarah K; Harper, Jonathan D; Lin, Daniel W; Wright, Jonathan L

    2015-12-01

    Cystic renal cell carcinoma (cystic RCC) is thought to carry an improved prognosis relative to clear cell RCC (CCRCC); however, this is based on small case series. We used a population-based tumor registry to compare clinicopathologic features and cancer-specific mortality (CSM) of cystic RCC with those of CCRCC. The Surveillance, Epidemiology, and End Results database was queried for all patients diagnosed and treated for cystic RCC and CCRCC between 2001 and 2010. Clinical and pathologic factors were compared using t tests and chi-square tests as appropriate. Kaplan-Meier survival analysis compared CSM differences between cystic RCC and CCRCC. A total of 678 patients with cystic RCC and 46,677 with CCRCC were identified. The mean follow-up duration was 52 and 40 months, respectively. When compared with CCRCC patients, those with cystic RCC were younger (mean age 58 vs. 61 y, P < 0.001), more commonly black (22% vs. 9%, P < 0.001), and female (45% vs. 41%, P = 0.02). Cystic RCCs were more commonly T1a tumors (66% vs. 55%, P < 0.001), well differentiated (33% vs. 16%, P < 0.001), and smaller (mean size = 3.8 vs. 4.5 cm, P < 0.001). Cystic RCC was associated with a reduction in CSM when compared with CCRCC (P = 0.002). In a subset analysis, this reduction in CSM was seen only for those with T1b/T2 tumors (P = 0.01) but not for those with T1a RCCs lesions (P = 0.31). We report the largest series of cystic RCC and corroborate the findings of improved CSM when compared with CCRCC for larger tumors; however, no difference was noted in smaller tumors, suggesting that tumor biology becomes more relevant to prognosis with increasing size. These data may suggest a role for active surveillance in appropriately selected patients with small, cystic renal masses. Copyright © 2015 Elsevier Inc. All rights reserved.

  15. Cystic renal cell carcinoma carries an excellent prognosis regardless of tumor size

    PubMed Central

    Winters, Brian R.; Gore, John L.; Holt, Sarah K.; Harper, Jonathan D.; Lin, Daniel W.; Wright, Jonathan L.

    2016-01-01

    Introduction Cystic renal cell carcinoma (cystic RCC) is thought to carry an improved prognosis relative to clear cell RCC (CCRCC); however, this is based on small case series. We used a population-based tumor registry to compare clinicopathologic features and cancer-specific mortality (CSM) of cystic RCC with those of CCRCC. Materials and methods The Surveillance, Epidemiology, and End Results database was queried for all patients diagnosed and treated for cystic RCC and CCRCC between 2001 and 2010. Clinical and pathologic factors were compared using t tests and chi-square tests as appropriate. Kaplan-Meier survival analysis compared CSM differences between cystic RCC and CCRCC. Results A total of 678 patients with cystic RCC and 46,677 with CCRCC were identified. The mean follow-up duration was 52 and 40 months, respectively. When compared with CCRCC patients, those with cystic RCC were younger (mean age 58 vs. 61 y, P < 0.001), more commonly black (22% vs. 9%, P < 0.001), and female (45% vs. 41%, P = 0.02). Cystic RCCs were more commonly T1a tumors (66% vs. 55%, P < 0.001), well differentiated (33% vs. 16%, P < 0.001), and smaller (mean size 3.8 vs. 4.5 cm, P < 0.001). Cystic RCC was associated with a reduction in CSM when compared with CCRCC (P = 0.002). In a subset analysis, this reduction in CSM was seen only for those with T1b/T2 tumors (P = 0.01) but not for those with T1a RCCs lesions (P = 0.31). Conclusions We report the largest series of cystic RCC and corroborate the findings of improved CSM when compared with CCRCC for larger tumors; however, no difference was noted in smaller tumors, suggesting that tumor biology becomes more relevant to prognosis with increasing size. These data may suggest a role for active surveillance in appropriately selected patients with small, cystic renal masses. PMID:26319351

  16. [Giant splenic cyst in a teenager girl: Case report].

    PubMed

    Martínez Torres, Beatriz; Medina García, Manuel; Zafra Anta, Miguel Ángel; García Muñoz-Najar, Alejandro José; Tardío Dovao, Juan C

    2017-06-01

    Giant nonparasitic splenic epidermoid cysts are relatively uncommon. These lesions can lead abdominal pain, but most of then are asymptomatic, and they are discovered incidentally. We report a 13-y old female with a giant splenic epidermoid cystic, given the special interest of diagnostic and therapeutic decision-making of this rare entity. A 13-y old female with clinical history of abdominal pain since the last two months. On physical examination a firm, tender mass was palpable in left hypochondrium. Diagnosis of a large cystic splenic mass was made based on ultrasound and abdominal computed tomography scan. Splenectomy was performed, and histopathological-immunohistochemistry studies revealed findings suggestive of primary epithelial cyst. The post-operative clinical course was satisfactory and uneventful. Treatment of giant nonparasitic splenic cysts is surgical. Preserve splenic parenchyma must be the aim in an individualized decision-making. The different types of surgical modalities will be according to the diagnosis and clinical situation (cyst size, age, comorbidities).

  17. Unusual fast-growing ovarian cystic teratoma during pregnancy presenting with intracystic fat "floating balls" appearance.

    PubMed

    Donnadieu, Anne Claire; Deffieux, Xavier; Le Ray, Camille; Mordefroid, Marie; Frydman, René; Fernandez, Hervé

    2006-12-01

    A large ovarian cyst was diagnosed at 22 weeks' of gestation in a 32-year-old woman. The ultrasonographic appearance of the ovarian cyst was unusual with multiple mobile, spherical echogenic structures floating in the cystic mass, called intracystic "fat balls." Right adnexectomy was performed by laparotomy at 28 weeks' of gestation, because of rapid growth and overall size exceeding 20 cm. Pathological examination confirmed ovarian cystic teratoma. Usually, dermoid cysts are slow-growing, even in premenopausal women. The exact mechanism related to the fast growth during pregnancy is unknown. It could be related to an unusual pattern of estrogen (E)/P receptors expression in the cystic teratoma. This case shows that a fast-growing, mature ovarian cystic teratoma may occur during pregnancy.

  18. Diagnostic utility of abdominal ultrasonography in dogs with chronic diarrhea.

    PubMed

    Leib, M S; Larson, M M; Grant, D C; Monroe, W E; Troy, G C; Panciera, D L; Rossmeisl, J H; Werre, S R

    2012-01-01

    Chronic diarrhea is common in dogs and has many causes. Ultrasonographic descriptions of many gastrointestinal diseases have been published, but the diagnostic utility of ultrasonography in dogs with chronic diarrhea has not been investigated. Diagnostic utility of abdominal ultrasound will be highest in dogs with GI neoplasia and lowest in those with inflammatory disorders. 87 pet dogs with chronic diarrhea. Prospective study in which medical records were reviewed and contribution of abdominal ultrasound toward making diagnosis was scored. In 57/87 (66%) of dogs, the same diagnosis would have been reached without ultrasonography. In 13/87 (15%) of dogs, the ultrasound examination was vital or beneficial to making the diagnosis. Univariable analysis identified that increased diagnostic utility was associated with weight loss (P = .0086), palpation of an abdominal or rectal mass (P = .0031), diseases that commonly have mass lesions visible on ultrasound examination (P < .0001), and a final diagnosis of GI neoplasia. Multivariable regression indicated that utility of abdominal ultrasonography would be 30 times more likely to be high in dogs in which an abdominal or rectal mass was palpated (odds ratio 30.5, 95% CI 5.5-169.6) (P < .0001) versus dogs without a palpable mass. In 15/87 (17%) of dogs, additional benefits of ultrasonography to case management, independent of the contribution to the diagnosis of diarrhea, were identified. Overall, the diagnostic utility of abdominal ultrasonography was low in dogs with chronic diarrhea. Identification of factors associated with high diagnostic utility is an indication to perform abdominal ultrasonography in dogs with chronic diarrhea. Copyright © 2012 by the American College of Veterinary Internal Medicine.

  19. Intra-Abdominal Hypertension and Abdominal Compartment Syndrome after Abdominal Wall Reconstruction: Quaternary Syndromes?

    PubMed

    Kirkpatrick, A W; Nickerson, D; Roberts, D J; Rosen, M J; McBeth, P B; Petro, C C; Berrevoet, Frederik; Sugrue, M; Xiao, Jimmy; Ball, C G

    2017-06-01

    Reconstruction with reconstitution of the container function of the abdominal compartment is increasingly being performed in patients with massive ventral hernia previously deemed inoperable. This situation places patients at great risk of severe intra-abdominal hypertension and abdominal compartment syndrome if organ failure ensues. Intra-abdominal hypertension and especially abdominal compartment syndrome may be devastating systemic complications with systematic and progressive organ failure and death. We thus reviewed the pathophysiology and reported clinical experiences with abnormalities of intra-abdominal pressure in the context of abdominal wall reconstruction. Bibliographic databases (1950-2015), websites, textbooks, and the bibliographies of previously recovered articles for reports or data relating to intra-abdominal pressure, intra-abdominal hypertension, and the abdominal compartment syndrome in relation to ventral, incisional, or abdominal hernia repair or abdominal wall reconstruction. Surgeons should thus consider and carefully measure intra-abdominal pressure and its resultant effects on respiratory parameters and function during abdominal wall reconstruction. The intra-abdominal pressure post-operatively will be a result of the new intra-peritoneal volume and the abdominal wall compliance. Strategies surgeons may utilize to ameliorate intra-abdominal pressure rise after abdominal wall reconstruction including temporizing paralysis of the musculature either temporarily or semi-permanently, pre-operative progressive pneumoperitoneum, permanently removing visceral contents, or surgically releasing the musculature to increase the abdominal container volume. In patients without complicating shock and inflammation, and in whom the abdominal wall anatomy has been so functionally adapted to maximize compliance, intra-abdominal hypertension may be transient and tolerable. Intra-abdominal hypertension/abdominal compartment syndrome in the specific setting of

  20. Abdominal Wall Desmoid during Pregnancy: Diagnostic Challenges

    PubMed Central

    Awwad, Johnny; Hammoud, Nadine; Farra, Chantal; Fares, Farah; Abi Saad, George; Ghazeeri, Ghina

    2013-01-01

    Background. Desmoids are benign tumors, with local invasive features and no metastatic potential, which have rarely been described to be pregnancy associated. Case. We described the rapid growth of an anterior abdominal wall mass in a 40-year-old pregnant woman. Due to its close proximity to the enlarged uterus, it was misdiagnosed to be a uterine leiomyoma by ultrasound examination. Final tissue diagnosis and radical resection were done at the time of abdominal delivery. Conclusion. Due to the diagnostic limitations of imaging techniques, desmoids should always be considered when the following manifestations are observed in combination: progressive growth of a solitary abdominal wall mass during pregnancy and well-delineated smooth tumor margins demonstrated by imaging techniques. This case emphasizes the importance of entertaining uncommon medical conditions in the differential diagnosis of seemingly common clinical manifestations. PMID:23346436

  1. Cystic lesions of the pancreas

    PubMed Central

    Karoumpalis, Ioannis; Christodoulou, Dimitrios K.

    2016-01-01

    Different types of benign or malignant cystic lesions can be observed in the pancreas. Pancreatic cystic lesions are classified under pathology terms into simple retention cysts, pseudocysts and cystic neoplasms. Mucinous cystic neoplasm is a frequent type of cystic neoplasm and has a malignant potential. Serous cystadenoma follows in frequency and is usually benign. Intraductal papillary mucinous neoplasms are the most commonly resected cystic pancreatic neoplasms characterized by dilated segments of the main pancreatic duct and/or side branches, the wall of which is covered by mucus secreting cells. These neoplasms can occupy the pancreatic head or any part of the organ. Solid pseudopapillary tumor is rare, has a low tendency for malignancy, and is usually located in the pancreatic body or tail. Endoscopic ultrasound with the use of fine-needle aspiration and cytology permits discrimination of those lesions. In this review, the main characteristics of those lesions are presented, as well as recommendations regarding their follow up and management according to recent guidelines. PMID:27065727

  2. Mullerian papilloma-like proliferation arising in cystic pelvic endosalpingiosis.

    PubMed

    McCluggage, W Glenn; O'Rourke, Declan; McElhenney, Clodagh; Crooks, Michael

    2002-09-01

    This report describes an unusual epithelial proliferation occurring in pelvic cystic endosalpingiosis. A cyst mass lined by a layer of ciliated epithelial cells involved the posterior surface of the cervix and vagina. The epithelial proliferation within the wall resembled a mullerian papilloma with fibrous and fibrovascular cores lined by bland cuboidal epithelial cells. Other areas had a microglandular growth pattern resembling cervical microglandular hyperplasia, and focally there was a solid growth pattern. Foci of typical endosalpingiosis involved the surface of both ovaries and pelvic soft tissues. The cystic lesion recurred after partial cystectomy and drainage and was followed up radiologically and with periodic fine-needle aspiration. Part of the wall of the cyst removed 11 years after the original surgery showed an identical epithelial proliferation. MIB1 staining showed a proliferation index of less than 5%, contrasting with the higher proliferation index of a typical serous borderline tumor. The differential diagnosis is discussed. As far as we are aware, this is the first report of such a benign epithelial proliferation involving cystic endosalpingiosis. Copyright 2002, Elsevier Science (USA). All rights reserved.

  3. Adenoid cystic carcinoma of cervix in younger women: report of two new cases.

    PubMed

    Sinaa, Mohamed; Oukabli, Mohamed; Albouzidi, Abderahmane

    2014-01-01

    Adenoid cystic carcinoma is a malignant epithelial neoplasm derived from the salivary glands. Primary adenoid cystic carcinoma of the cervix is extremely rare, accounting for less than 1% of all cervical carcinomas. Its origin is debatable. It generally presents in elderly age group, however only twenty three cases have been reported in women less than age 45 years old. In this paper we report two new cases of primary adenoid cystic carcinoma in younger women and include the cytopathology and histopathology findings. A 36, 41 year-old women were admitted with signs and symptoms suggestive of a cervical cancer. Speculum examination showed a firm mass in the uterine cervix. The diagnosis of adenoid cystic carcinoma was confirmed by the histopathology examination of the biopsy specimen in the two patients, which characteristically revealed the presence of a cribriform pattern and cylindromatous structures of the tumor cells, adhered around hyaline stoma. They were managed successfully by concurrent radiotherapy. Adenoid cystic carcinoma of the cervix is a rare disease. It usually occurs in an elderly age group. We report these two cases because of its rarity in young patients, with description of illustrative pathology and discussion on the histological diagnosis.

  4. Annual Review Clinic improves care in children with cystic fibrosis.

    PubMed

    Chuang, Sandra; Doumit, Michael; McDonald, Rebecca; Hennessy, Erika; Katz, Tamarah; Jaffe, Adam

    2014-03-01

    It is unclear whether annual multidisciplinary reviews in cystic fibrosis (CF) patients should be conducted in dedicated annual review (AR) clinics or during continuous assessments throughout the year. Our aim was to assess the effect of introducing an AR clinic. A retrospective written and electronic record review of CF patients was carried out for 2007 (no AR Clinic) and 2010 (established AR Clinic) calendar years. An internet-based satisfaction survey was distributed to families attending the AR clinic. In total, 123 children (mean age 9.5 years, range 1.32-18.8 years) and 141 children (8.3 years, 1.1-18.3 years) were included in 2007 and 2010 respectively. There was a significant increase in multidisciplinary reviews (documented annual review 28% vs 85%, P < 0.001; dietary assessment 46% vs 92%, P < 0.001) and investigations (OGTT 2% vs 74%, P < 0.001; abdominal ultrasound 35% vs 85%, P < 0.001) conducted after the introduction of AR clinic. The majority of the families surveyed (85%) were satisfied or very satisfied with the AR clinic. CF AR clinic significantly improves the number of annual investigations and multidisciplinary reviews performed. Families were satisfied with this new process. © 2013. Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society. All rights reserved.

  5. Cystic Echinococcosis

    PubMed Central

    Brunetti, Enrico; McCloskey, Cindy

    2015-01-01

    Echinococcosis is one of the 17 neglected tropical diseases (NTDs) recognized by the World Health Organization. The two major species of medical importance are Echinococcus granulosus and Echinococcus multilocularis. E. granulosus affects over 1 million people and is responsible for over $3 billion in expenses every year. In this minireview, we discuss aspects of the epidemiology, clinical manifestations, and diagnosis of cystic echinococcosis or cystic hydatid disease caused by E. granulosus. PMID:26677245

  6. Comparison of Ambient and Atmospheric Pressure Ion Sources for Cystic Fibrosis Exhaled Breath Condensate Ion Mobility-Mass Spectrometry Metabolomics

    NASA Astrophysics Data System (ADS)

    Zang, Xiaoling; Pérez, José J.; Jones, Christina M.; Monge, María Eugenia; McCarty, Nael A.; Stecenko, Arlene A.; Fernández, Facundo M.

    2017-08-01

    Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the gene that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The vast majority of the mortality is due to progressive lung disease. Targeted and untargeted CF breath metabolomics investigations via exhaled breath condensate (EBC) analyses have the potential to expose metabolic alterations associated with CF pathology and aid in assessing the effectiveness of CF therapies. Here, transmission-mode direct analysis in real time traveling wave ion mobility spectrometry time-of-flight mass spectrometry (TM-DART-TWIMS-TOF MS) was tested as a high-throughput alternative to conventional direct infusion (DI) electrospray ionization (ESI) and atmospheric pressure chemical ionization (APCI) methods, and a critical comparison of the three ionization methods was conducted. EBC was chosen as the noninvasive surrogate for airway sampling over expectorated sputum as EBC can be collected in all CF subjects regardless of age and lung disease severity. When using pooled EBC collected from a healthy control, ESI detected the most metabolites, APCI a log order less, and TM-DART the least. TM-DART-TWIMS-TOF MS was used to profile metabolites in EBC samples from five healthy controls and four CF patients, finding that a panel of three discriminant EBC metabolites, some of which had been previously detected by other methods, differentiated these two classes with excellent cross-validated accuracy.

  7. The cystic fibrosis gene: Medical and social implications for heterozygote detection

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Wilfond, B.S.; Fost, N.

    1990-05-23

    The primary goal of mass screening programs for cystic fibrosis carriers should be to allow people to make more informed reproductive decisions. However, previous experience with genetic screening programs, including those for phenylketonuria and sickle cell disease, have revealed complex problems including error, confusion, and stigmatization. These problems could be greater with cystic fibrosis, since more than 8 million Americans may be carriers and entrepreneurial interests can be expected to promote screening in what could become a billion-dollar industry. The present frequency of the detectable mutation ({Delta}F{sub 508}), 75%, will complicate the counseling process. The sensitivity of the test tomore » detect at-risk couples would be 56%. The cost of screening could be as much as $2.2 million for each cystic fibrosis birth avoided. Regardless of improvements in the detection rate, implementation of population screening should be delayed until pilot studies that demonstrate its safety and effectiveness are completed. While studies are in progress, preconception testing should be offered to adult relatives of cystic fibrosis patients as part of a comprehensive program following institutional review board approval for compassionate use.« less

  8. Abdominal Subcutaneous Mass After Laser-Assisted Lipolysis and Immediate Multiple Treatments with a Dual-Wavelength Laser, Vacuum and Massage Device.

    PubMed

    Gentileschi, Stefano; Servillo, Maria; D'Ettorre, Marco; Salgarello, Marzia

    2016-04-01

    Body contouring by means of minimally invasive procedures is a growing trend. Current approaches to body contouring often involve a combination of surgical techniques (eg, laser-assisted liposuction) and a series of noninvasive device-based treatments aimed at accelerating recovery and improving aesthetic outcomes. In this case study, we describe a 38-year-old woman who presented with an abdominal-wall mass that resembled a tumor when assessed with magnetic resonance imaging. Twenty-six months before presenting to our office, the patient had undergone laser lipolysis and a series of treatments with a device that delivered dual-wavelength laser energy and vacuum-assisted massage. To address the patient's concerns, we removed the mass and performed abdominal dermolipectomy. No postoperative complications occurred, and the patient was highly satisfied with the aesthetic outcome. The results of histologic studies indicated that the mass was pseudocystic and fluid-filled, surrounded by a fibrous capsule, and characterized as a foreign-body granuloma. Further analysis is warranted regarding the safety of laser lipolysis without aspiration combined with a device delivering dual-wavelength laser energy and vacuum-assisted massage. 5 Risk. © 2015 The American Society for Aesthetic Plastic Surgery, Inc. Reprints and permission: journals.permissions@oup.com.

  9. [Undifferentiated (embryonal) sarcoma of the liver. Report of a case].

    PubMed

    Orozco, H; Mercado, M A; Takahashi, T; Chan, C; Quintanilla, L; Jiménez, M; Sosa, R; Esquivel, E

    1991-01-01

    A 15-year-old woman who was studied because an abdominal mass at the Instituto Nacional de la Nutricion Salvador Zubiran (INNSZ) is reported. The history revealed only malaise and mild abdominal pain. At physical exploration, an abdominal mass in the upper right quadrant was found. Liver function tests were normal. Abdominal ultrasound and computerized tomography revealed a large cystic mass of the right hepatic lobe. She underwent exploratory laparotomy. Intraoperative frozen sections of the biopsies demonstrated undifferentiated sarcoma of the liver, and an extended right trisegmentectomy was performed. Postoperative outcome was uneventful. Adjuvant treatment with doxorubicin and dacarbazine was given, and at six months of follow-up, the patient is alive without any evidence of recurrence. Clinical and histopathologic features of this rare malignant tumor are discussed, as well as the therapeutic choices.

  10. Cystic Fibrosis and Pregnancy

    MedlinePlus

    ... Global Map Premature Birth Report Cards Careers Archives Pregnancy Before or between pregnancies Nutrition, weight & fitness Prenatal ... complications > Cystic fibrosis and pregnancy Cystic fibrosis and pregnancy E-mail to a friend Please fill in ...

  11. Abdominal Cysticercosis in a Red Fox ( Vulpes vulpes ).

    PubMed

    Whipp, Christopher James; Daoust, Pierre-Yves; Conboy, Gary; Gelens, Hans

    2017-01-01

    A large abdominal mass containing numerous cysticerci identified as those of Taenia crassiceps (=Cysticercus longicollis) was found in the pelvic region of the abdominal cavity of a severely constipated and emaciated red fox ( Vulpes vulpes ) in Prince Edward Island, Canada. Cysticercosis has not previously been reported in a wild canid in North America.

  12. The L441P mutation of cystic fibrosis transmembrane conductance regulator and its molecular pathogenic mechanisms in a Korean patient with cystic fibrosis.

    PubMed

    Gee, Heon Yung; Kim, Chang Keun; Kim, So Won; Lee, Ji Hyun; Kim, Jeong-Ho; Kim, Kyung Hwan; Lee, Min Goo

    2010-01-01

    Cystic fibrosis (CF) is an autosomal recessive disorder usually found in populations of white Caucasian descent. CF is caused by mutations in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene. A 5-yr-old Korean girl was admitted complaining of coughing and greenish sputum. Chest radiographs and computed tomographic (CT) scan revealed diffuse bronchiectasis in both lungs. The patient had chronic diarrhea and poor weight gain, and the abdominal pancreaticobiliary CT scan revealed atrophy of the pancreas. Finally, CF was confirmed by the repeated analysis of the quantitative pilocarpine iontophoresis test. The chloride concentration of sweat samples taken from both forearms of the pateint was an average of 88.7 mM/L (normal value <40 mM/L). After a comprehensive search for mutations in the CFTR gene, the patient was found to carry the non-synonymous L441P mutation in one allele. Molecular physiologic analysis of the L441P mutation of CFTR revealed that the L441P mutation completely abolished the CFTR Cl(-) channel activity by disrupting proper protein folding and membrane trafficking of CFTR protein. These results confirmed the pathogenicity of the L441P mutation of CFTR circulating in the Korean population. The possibility of CF should be suspected in patients with chronic bronchiectasis, although the frequency of CF is relatively rare in East Asia.

  13. Pediatric Cystic Nephroma Is Morphologically, Immunohistochemically, and Genetically Distinct From Adult Cystic Nephroma.

    PubMed

    Li, Yunjie; Pawel, Bruce R; Hill, Dana A; Epstein, Jonathan I; Argani, Pedram

    2017-04-01

    The term cystic nephroma has traditionally been used to refer to 2 neoplasms, a lesion in adults that is now thought to be part of the spectrum of mixed epithelial stromal tumor (MEST) and a pediatric lesion that has been associated with mutations in the DICER1 gene. A direct detailed morphologic, immunohistochemical, and genetic comparison of these 2 lesions has not been performed. In this study, we compare the morphologic features, immunoreactivity for estrogen receptor and inhibin, and DICER1 genetic status of 12 adult cystic nephroma/MEST (median age 50.5 y, all females) and 7 pediatric cystic nephroma (median age 1.3 y, male:female=6:1). Both lesions (11 of 12 adult cases, 6 of 7 pediatric cases) frequently demonstrated subepithelial accentuation of stromal cellularity, though the increased cellularity frequently included inflammatory cells in the pediatric cases. All adult and pediatric cases labeled for estrogen receptor; however, whereas most (83%) of adult cases labeled for inhibin at least focally, no pediatric case labeled for inhibin. Most adult cases (58%) demonstrated wavy, ropy collagen in association with cellular stroma, whereas this was not found in pediatric cases. 86% of pediatric cases demonstrated DICER1 mutations, whereas only 1 of 10 adult cases demonstrated a DICER1 mutation. In summary, although cellular stroma and estrogen receptor immunoreactivity are commonly present in both adult and pediatric cystic nephroma, ropy collagen and inhibin immunoreactivity are far more common in adult cystic nephroma/MEST, whereas DICER1 mutations are far more prevalent in pediatric cystic nephroma. These results support the current World Health Organization Classification's separation of adult and pediatric cystic nephromas as distinct entities.

  14. Unexpected Abscess Localization of the Anterior Abdominal Wall in an ADPKD Patient Undergoing Hemodialysis.

    PubMed

    Sabanis, Nikos; Paschou, Eleni; Gavriilaki, Eleni; Mourounoglou, Maria; Vasileiou, Sotirios

    2015-01-01

    Autosomal Dominant Polycystic Kidney Disease (ADPKD) is one of the most common monogenic disorders and the leading inheritable cause of end-stage renal disease worldwide. Cystic and noncystic extrarenal manifestations are correlated with variable clinical presentations so that an inherited disorder is now considered a systemic disease. Kidney and liver cystic infections are the most common infectious complications in ADPKD patients. Furthermore, it is well known that ADPKD is commonly associated with colonic diverticular disease which recently has been reported to be linked to increased risk of infection on hemodialysis patients. Herein, we present a case of anterior abdominal wall abscess caused by Enterococcus faecalis in a patient with ADPKD undergoing hemodialysis. Although the precise pathway of infection remains uncertain, the previous medical history as well as the clinical course of our patient led us to hypothesize an alternative route of infection from the gastrointestinal tract through an aberrant intestinal barrier into the bloodstream and eventually to an atypical location.

  15. Neonatal ovarian torsion complicated by intestinal obstruction and perforation, and review of the literature.

    PubMed

    Jeanty, Cerine; Frayer, Elizabeth A; Page, Renee; Langenburg, Scott

    2010-06-01

    We present a case of neonatal ovarian torsion complicated by bowel obstruction and perforation and review the literature regarding the incidence of bowel obstruction in neonatal ovarian cysts, the presentation, and treatment. A term neonate was prenatally diagnosed with a cystic abdominal mass palpable on physical examination. A postnatal abdominal x-ray showed paucity of gas in the left hemiabdomen with rightward displacement of bowel loops. Exploratory laparotomy on day 2 of life revealed a large cystic mass in the left lower quadrant consistent with a torsed left ovary, an omental band causing strangulation of the bowel mesentery, and a perforation of the distal ileum. Our literature search revealed 19 reported cases of neonatal ovarian cysts resulting in bowel obstruction. Infants may present with a palpable abdominal mass, respiratory distress, as well as signs and symptoms of intestinal obstruction. Two mechanisms exist for bowel obstruction: adhesions caused by a torsed necrotic ovary and mass effect of a large ovarian cyst, often measuring 9 to 10 cm in diameter. Options to treat ovarian cysts include antenatal or postnatal aspiration, laparoscopy, and laparotomy. Cysts less than 4 to 5 cm can be observed, whereas operative intervention is indicated in symptomatic cases and in persistent or enlarging ovarian cysts. Copyright 2010 Elsevier Inc. All rights reserved.

  16. BMI, total and abdominal fat distribution, and cardiovascular risk factors in school-age children.

    PubMed

    Gishti, Olta; Gaillard, Romy; Durmus, Busra; Abrahamse, Marieke; van der Beek, Eline M; Hofman, Albert; Franco, Oscar H; de Jonge, Layla L; Jaddoe, Vincent W V

    2015-05-01

    More specific total body and abdominal fat mass measures might be stronger associated with cardiovascular risk factors in childhood, than BMI. We examined the independent associations of total and abdominal fat measures with cardiovascular risk factors in school age children. We performed a population-based cohort study among 6,523 children. At the age of 6 y, we measured childhood BMI, and general and abdominal fat mass, using dual-energy X-ray absorptiometry, and ultrasound and cardiovascular risk factors. Conditional on BMI, higher fat mass percentage and abdominal fat mass were associated with higher blood pressure, total- and low-density lipoprotein (LDL)-cholesterol, insulin and c-peptide levels, but with lower left ventricular mass and high-density lipoprotein (HDL)-cholesterol (P values < 0.05). These associations differed between underweight, normal weight, overweight, and obese children. Higher childhood adiposity measures were associated with increased odds of cardiovascular risk factors clustering, with the strongest effect for fat mass percentage (odds ratios: 3.01 (95% confidence interval: 2.67, 3.9). Our results suggest that general and abdominal fat measures are associated with cardiovascular risk factors in childhood, independent from BMI. These measures may provide additional information for identification of children with an adverse cardiovascular profile.

  17. Reproducibility of regional DEXA examinations of abdominal fat and lean tissue.

    PubMed

    Tallroth, Kaj; Kettunen, Jyrki A; Kujala, Urho M

    2013-01-01

    The aim of this study was to develop and test the validity of a new repeatable method to delimit abdominal areas for follow-up of fat mass (FM) and lean tissue mass (LM) in DEXA examinations. 37 male volunteers underwent two DEXA examinations. Total body FM and LM measurements and corresponding abdominal measurements in a carefully defined region were calculated from the first scan. After repositioning of the subjects and a second scan, the delimited region was copied and the abdominal tissues re-calculated. The mean LM of the abdominal area was 2.804 kg (SD 0.556), and the mean FM was 1.026 kg (SD 0.537). The intra-class correlation coefficient for the repeated abdominal LM, FM, and LM/FM ratio measurements was 0.99. The mean difference (bias) for the repeated abdominal LM measurements was -13 g (95% confidence interval (CI) -193.0 to 166.8), and for the repeated abdominal FM measurements it was -35 g (95% CI -178.9 to 108.5). The results indicate that regional DEXA is a sensitive method with excellent reproducibility in the measurements of the abdominal fat and lean tissues. The method may serve as a useful tool for evaluation and follow-up of various dietary and training programmes.

  18. Post-Treatment Follow-Up Study of Abdominal Cystic Echinococcosis in Tibetan Communities of Northwest Sichuan Province, China

    PubMed Central

    Li, Tiaoying; Ito, Akira; Pengcuo, Renqing; Sako, Yasuhito; Chen, Xingwang; Qiu, Dongchuan; Xiao, Ning; Craig, Philip S.

    2011-01-01

    diagnosis, seven (53.8%) of 13 cases with CE1 type cysts without any previous intervention showed negative specific IgG antibody response to E. granulosus recombinant antigen B (rAgB). However, following 3 months to 18 months albendazole therapy, six of these 7 initially seronegative CE1 cases sero-converted to be specific IgG antibody positive, and concurrently ultrasound scan showed that cysts changed to CE3a from CE1 type in all the six CE cases. Two major profiles of serum specific IgG antibody dynamics during albendazole treatment were apparent in CE cases: (i) presenting as initial elevation followed by subsequent decline, or (ii) a persistent decline. Despite a decline, however, specific antibody levels remained positive in most improved or cured CE cases. Conclusions This was the first attempt to follow up community-screened cystic echinococcosis patients after albendazole therapy using ultrasonography and serology in an endemic Tibetan region. Cyclic albendazole treatment proved to be effective in the great majority of CE cases in this resource-poor area, but periodic abdominal ultrasound examination was necessary to guide appropriate treatment. Oral albendazole for over 18 months was more likely to result in CE cure. Poor drug compliance resulted in less good outcomes. Serology with recombinant antigen B could provide additional limited information about the effectiveness of albendazole in CE cases. Post-treatment positive specific IgG antibody seroconversion, in initially seronegative, CE1 patients was considered a good indication for positive therapeutic efficacy of albendazole. PMID:22039558

  19. Characteristics and Treatments of Large Cystic Brain Metastasis: Radiosurgery and Stereotactic Aspiration

    PubMed Central

    Kim, Moinay; Cheok, Stephanie; Chung, Lawrance K.; Ung, Nolan; Thill, Kimberly; Voth, Brittany; Kwon, Do Hoon; Kim, Jeong Hoon; Kim, Chang Jin; Tenn, Stephen; Lee, Percy

    2015-01-01

    Brain metastasis represents one of the most common causes of intracranial tumors in adults, and the incidence of brain metastasis continues to rise due to the increasing survival of cancer patients. Yet, the development of cystic brain metastasis remains a relatively rare occurrence. In this review, we describe the characteristics of cystic brain metastasis and evaluate the combined use of stereotactic aspiration and radiosurgery in treating large cystic brain metastasis. The results of several studies show that stereotactic radiosurgery produces comparable local tumor control and survival rates as other surgery protocols. When the size of the tumor interferes with radiosurgery, stereotactic aspiration of the metastasis should be considered to reduce the target volume as well as decreasing the chance of radiation induced necrosis and providing symptomatic relief from mass effect. The combined use of stereotactic aspiration and radiosurgery has strong implications in improving patient outcomes. PMID:25977901

  20. A laparoscopic approach to treating torsion of a nonpregnant uterus accompanied by a large ovarian cyst in a premenarchal girl.

    PubMed

    Dutra, Robson Azevedo; Perez-Bóscollo, Adriana Cartafina; Ribeiro, Fernanda Cristina Silva Alves; Vietez, Nádia Bicego

    2008-03-01

    A 10-year-old premenarchal girl was admitted to our hospital with moderate abdominal pain, although presenting no vomiting or abdominal rebound tenderness. A large abdominal mass was visible and palpable in the periumbilical and epigastric regions. Results of physical examination revealed that the general health status was satisfactory. Computed tomographic scan revealed a large, thin-walled cyst occupying nearly the entire peritoneal cavity. The other viscera were of normal aspect. A laparoscopic approach revealed a left ovarian cystic tumor that was twisted 360 degrees in conjunction with the uterine corpus with hemorrhagic infarction. A partial hysterectomy and a left salpingo-oophorectomy were carried out. The tumor was classified as mature cystic teratoma of the ovary accompanied by hemorrhagic necrosis, not only of the cyst but also of the left uterine tube and the uterine corpus.

  1. Effects of canola and high-oleic-acid canola oils on abdominal fat mass in individuals with central obesity.

    PubMed

    Liu, Xiaoran; Kris-Etherton, Penny M; West, Sheila G; Lamarche, Benoît; Jenkins, David J A; Fleming, Jennifer A; McCrea, Cindy E; Pu, Shuaihua; Couture, Patrick; Connelly, Philip W; Jones, Peter J H

    2016-11-01

    To determine the effect of diets low in saturated fatty acids and high in monounsaturated fatty acids (MUFA) or polyunsaturated fatty acids on body composition in participants at risk for metabolic syndrome (MetS). This study was a randomized, crossover, controlled feeding study. Participants (n = 101, ages 49.5 ± 1.2, BMI 29.4 ± 0.4 kg/m 2 ) were randomized to five isocaloric diets containing treatment oils: Canola, CanolaOleic, CanolaDHA, Corn/Safflower, and Flax/Safflower. Each diet period was 4 weeks followed by a 2- to 4-week washout period. Canola (3.1 kg, P = 0.026) and CanolaOleic oil diets (3.09 kg, P = 0.03) reduced android fat mass compared with the Flax/Saff oil diet (3.2 kg), particularly in men. The decrease in abdominal fat mass was correlated with the reduction in blood pressure after the Canola (systolic blood pressure: r = 0.26, P = 0.062; diastolic blood pressure: r = 0.38, P = 0.0049) and CanolaOleic oil diets (systolic blood pressure: r = 0.39 P = 0.004; diastolic blood pressure: r = 0.45, P = 0.0006). The decrease in abdominal fat mass also was associated with a reduction in triglyceride levels after the CanolaOleic oil diet (r = 0.42, P = 0.002). Diets high in MUFA (compared with PUFA) reduced central obesity with an accompanying improvement in MetS risk factors. Diets high in MUFA may be beneficial for treating and perhaps preventing MetS. © 2016 The Obesity Society.

  2. Effects of Canola and High-Oleic Acid Canola Oils on Abdominal Fat Mass in Individuals with Central Obesity

    PubMed Central

    Liu, Xiaoran; Kris-Etherton, Penny M.; West, Sheila G.; Lamarche, Benoît; Jenkins, David J. A.; Fleming, Jennifer A.; McCrea, Cindy E.; Pu, Shuaihua; Couture, Patrick; Connelly, Philip W.; Jones, Peter J. H.

    2016-01-01

    Objective To determine the effect of diets low in saturated fatty acids (SFA), high in monounsaturated fatty acids (MUFA) or polyunsaturated fatty acids (PUFA) on body composition in participants at risk for MetS. Methods; The present study is a randomized, crossover, controlled feeding study. Participants (n = 101, ages 49.5 ± 1.2, BMI 29.4 ± 0.4 kg/m2) were randomized to five isocaloric diets contained treatment oils: Canola, CanolaOleic, CanolaDHA, Corn/Safflower and Flax/Safflower. Each diet period was 4-week followed by a 2–4 week washout period. Results Canola (3.1 kg, p=0.026) and CanolaOleic oil diets (3.1 kg, p=0.03) reduced android fat mass compared with the Flax/Saff oil diet (3.2 kg), particularly in males. The decrease in abdominal fat mass was correlated with the reduction in blood pressure after the Canola (SBP r = 0.26, p=0.062; DBP r=0.38, p=0.0049) and CanolaOleic oil diets (SBP r = 0.39 p=0.004; DBP r=0.45, p=0.0006). The decrease in abdominal fat mass also was associated with a reduction in TG levels after the CanolaOleic oil diet (r = 0.42, p=0.002). Conclusion Diets high in MUFA (compared with PUFA) reduced central obesity with an accompanying improvement in MetS risk factors. Our findings demonstrate that diets high in MUFA may be beneficial for treating and perhaps preventing MetS. PMID:27804268

  3. Evolving Mechanistic Views and Emerging Therapeutic Strategies for Cystic Fibrosis–Related Diabetes

    PubMed Central

    2017-01-01

    Diabetes is a common and important complication of cystic fibrosis, an autosomal recessive genetic disease due to mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Cystic fibrosis–related diabetes (CFRD) is associated with profound detrimental effects on the disease course and mortality and is expected to increase in prevalence as the survival of patients with cystic fibrosis continues to improve. Despite progress in the functional characterization of CFTR molecular defects, the mechanistic basis of CFRD is not well understood, in part because of the relative inaccessibility of the pancreatic tissue and the limited availability of representative animal models. This review presents a concise overview of the current understanding of CFRD pathogenesis and provides a cutting-edge update on novel findings from human and animal studies. Potential contributions from paracrine mechanisms and β-cell compensatory mechanisms are highlighted, as well as functional β-cell and α-cell defects, incretin defects, exocrine pancreatic insufficiency, and loss of islet cell mass. State-of-the-art and emerging treatment options are explored, including advances in insulin administration, CFTR modulators, cell replacement, gene replacement, and gene editing therapies. PMID:29264462

  4. Mucocele of the appendix: An unusual cause of lower abdominal pain in a patient with ulcerative colitis-. A case report and review of literature

    PubMed Central

    Lakatos, Peter Laszlo; Gyori, Gabriella; Halasz, Judit; Fuszek, Peter; Papp, Janos; Jaray, Balazs; Lukovich, Peter; Lakatos, Laszlo

    2005-01-01

    The authors report the case of a 60-year-old male patient. In November 2001 he developed intestinal symptoms of bloody diarrhea and abdominal pain. Colonoscopy and biopsy established the diagnosis of ulcerative colitis (proctosigmoiditis). The disease activity was moderate at the beginning. No significant laboratory alterations were found (including CEA, CA19-9), and mesalazine was started orally. He was in remission until November 2003, when he was admitted to our Outpatient Clinic for upper and right lower abdominal pain and bloody diarrhea. Colonoscopy found proctosigmoiditis with a moderate activity, gastroscopy revealed chronic gastritis, laboratory data was normal. Treatment was amended with mesalazine clysma and methylprednisolone (16 mg) orally. Symptoms ameliorated; however, right lower abdominal pain persisted. US and CT examination demonstrated a pericecal cystic mass (11 cm×3.5 cm). At first pericecal abscess was suspected, as the previous US examination (6 mo earlier) had revealed normal findings. Fine needle aspiration was performed. Cytology confirmed the diagnosis of mucocele. The patient underwent partial cecum resection and extirpation of the mucocele. He recovered well and the final histology revealed a cystadenoma of the appendix. Follow up was started. The patient is now free of symptoms. Although primary adenocarcinoma of the appendix is uncommon, the authors emphasize that preoperative diagnosis of an underlying malignancy in a mucocele is important for patient management; however, it is difficult on imaging studies. PMID:15637769

  5. Effect of endurance exercise on respiratory muscle function in patients with cystic fibrosis.

    PubMed

    Reilly, Charles C; Ward, Katie; Jolley, Caroline J; Frank, Lucy A; Elston, Caroline; Moxham, John; Rafferty, Gerrard F

    2012-03-15

    During exercise, patients with cystic fibrosis (CF) dynamically hyperinflate, which imposes both elastic and threshold loads on the inspiratory muscles and places them at a mechanical disadvantage due to muscle shortening. Conversely, dynamic hyperinflation imposes a progressively resistive load and lengthens the expiratory muscles potentially increasing their susceptibility to develop low frequency fatigue (LFF). The aim of the study was to determine whether high intensity endurance exercise leads to the development of LFF in either the diaphragm or expiratory abdominal wall muscles in patients with CF. Ten patients and ten healthy individuals were studied. Twitch transdiaphragmatic pressure (TwP(di)) and twitch abdominal pressure (TwT(10)) were measured before and after exhaustive endurance cycle exercise at 80% of their previously determined maximum work rate. There was no difference in TwP(di) or TwT(10) at 20, 40 or 60 min post exercise compared to pre-exercise resting values in any of the participants, indicating that overt LFF of the respiratory muscles did not develop. Copyright © 2012 Elsevier B.V. All rights reserved.

  6. [Intraoperative ultrasonography, dissection with ultra-shears and trans-cystic choledochoscopy during laparoscopic approach in obstructive lithiasis icterus case].

    PubMed

    Alecu, L; Corodeanu, G; Costan, I; Lungu, C; Staicu, A

    2001-01-01

    The authors present the case of a 48 years old male with lithiasis obstructive icterus who was operated by laparoscopic transcystic approach with choledochoscopy. This minimally invasive technique was very spectacular trough the using of laparoscopic ultrasonography, dissection with Ultra-Shears device and transcystic choledochoscopy. Careful watching of abdominal laparoscopic view and transcystic choledochoscopic view is the particularity of laparoscopic transcystic management of choledocholithiasis. This metod don't let us to explore the biliary tract before the junction between common bile duct and cystic duct.

  7. Molecular Diagnosis of Cystic Fibrosis.

    PubMed

    Deignan, Joshua L; Grody, Wayne W

    2016-01-01

    This unit describes a recommended approach to identifying causal genetic variants in an individual suspected of having cystic fibrosis. An introduction to the genetics and clinical presentation of cystic fibrosis is initially presented, followed by a description of the two main strategies used in the molecular diagnosis of cystic fibrosis: (1) an initial targeted variant panel used to detect only the most common cystic fibrosis-causing variants in the CFTR gene, and (2) sequencing of the entire coding region of the CFTR gene to detect additional rare causal CFTR variants. Finally, the unit concludes with a discussion regarding the analytic and clinical validity of these approaches. Copyright © 2016 John Wiley & Sons, Inc.

  8. Evaluation of Abdominal Ultrasonography Mass Screening for Hepatocellular Carcinoma in Taiwan

    PubMed Central

    Yeh, Yen-Po; Hu, Tsung-Hui; Cho, Po-Yuan; Chen, Hsiu-Hsi; Yen, Amy Ming-Fang; Chen, Sam Li-Sheng; Chiu, Sherry Yueh-Hsia; Fann, Jean Ching-Yuan; Su, Wei-Wen; Fang, Yi-Jen; Chen, Shih-Tien; San, Hsiao-Ching; Chen, Hung-Pin; Liao, Chao-Sheng

    2014-01-01

    Mass screening with abdominal ultrasonography (AUS) has been suggested as a tool to control adult hepatocellular carcinoma (HCC) in individuals, but its efficacy in reducing HCC mortality has never been demonstrated. This study aimed to assess the effectiveness of reducing HCC mortality by mass AUS screening for HCC based on a program designed and implemented in the Changhua Community-based Integrated Screening (CHCIS) program with an efficient invitation scheme guided by the risk score. We invited 11,114 (27.0%) of 41,219 eligible Taiwanese subjects between 45 and 69 years of age who resided in an HCC high-incidence area to attend a risk score-guided mass AUS screening between 2008 and 2010. The efficacy of reducing HCC mortality was estimated. Of the 8,962 AUS screening attendees (with an 80.6% attendance rate), a total of 16 confirmed HCC cases were identified through community-based ultrasonography screening. Among the 16 screen-detected HCC cases, only two died from HCC, indicating a favorable survival. The cumulative mortality due to HCC (per 100,000) was considerably lower in the invited AUS group (17.26) compared with the uninvited AUS group (42.87) and the historical control group (47.51), yielding age- and gender-adjusted relative mortality rates of 0.69 (95% confidence interval [CI]: 0.56-0.84) and 0.63 (95% CI: 0.52-0.77), respectively. Conclusion: The residents invited to community-based AUS screening for HCC, compared with those who were not invited, showed a reduction in HCC mortality by ∼31% among subjects aged 45-69 years who had not been included in the nationwide vaccination program against hepatitis B virus infection. (Hepatology 2014;59:1840–1849) PMID:24002724

  9. Computed tomography of cystic nerve root sleeve dilatation.

    PubMed

    Neave, V C; Wycoff, R R

    1983-10-01

    A case of cystic nerve root sleeve dilatation in the lumbar area associated with a chronic back pain syndrome is presented. Prominent computed tomography (CT) findings include: (a) rounded masses in the region of the foramina isodense with cerebrospinal fluid in the subarachnoid space; (b) associated asymmetry of epidural fat distribution; (c) enlargement of the neural foramina in axial sections with scalloped erosion of the adjacent posteriolateral vertebral body, pedicle, and pedicular-laminar junction with preservation of cortex and without bony sclerosis or infiltrative appearance; (d) prominent or ectatic dural sac with lack of usual epidural landmarks between the sac and vertebral body; and (e) multilevel abnormalities throughout the entire lumbar region. Myelographic and CT correlations are demonstrated with a review of the literature. A discussion of the various cystic abnormalities involving nerve root sheaths is undertaken in an attempt to clarify the confusing nomenclature applied to nerve root sleeve pathology.

  10. Hyperandrogenism Accompanies Increased Intra-Abdominal Fat Storage in Normal Weight Polycystic Ovary Syndrome Women.

    PubMed

    Dumesic, Daniel A; Akopians, Alin L; Madrigal, Vanessa K; Ramirez, Emmanuel; Margolis, Daniel J; Sarma, Manoj K; Thomas, Albert M; Grogan, Tristan R; Haykal, Rasha; Schooler, Tery A; Okeya, Bette L; Abbott, David H; Chazenbalk, Gregorio D

    2016-11-01

    Normal weight polycystic ovary syndrome (PCOS) women may have altered adipose structure-function underlying metabolic dysfunction. This study examines whether adipose structure-functional changes exist in normal weight PCOS women and correlate with hyperandrogenism and/or hyperinsulinemia. This is a prospective cohort study. The setting was an academic medical center. Six normal weight PCOS women and 14 age- and body mass index-matched normoandrogenic ovulatory (NL) women were included. All women underwent circulating hormone and metabolic measurements; frequently sampled intravenous glucose tolerance testing; total body dual-energy x-ray absorptiometry; abdominal magnetic resonance imaging; and SC abdominal fat biopsy. Circulating hormones and metabolites, body fat and its distribution, and adipocyte size were compared between PCOS and NL women, and were correlated with each other in all women. Circulating LH and androgen levels were significantly greater in PCOS than NL women, as were fasting insulin levels, pancreatic β-cell responsiveness to glucose, and total abdominal fat mass. Intra-abdominal fat mass also was significantly increased in PCOS women and was positively correlated with circulating androgen, fasting insulin, triglyceride, and non-high-density lipoprotein cholesterol levels in all women. SC abdominal fat mass was not significantly increased in PCOS women, but contained a greater proportion of small SC abdominal adipocytes that positively correlated with serum androgen levels in all women. Hyperandrogenism in normal weight PCOS women is associated with preferential intra-abdominal fat deposition and an increased population of small SC abdominal adipocytes that could constrain SC adipose storage and promote metabolic dysfunction.

  11. Cystic neoplasms of the exocrine pancreas.

    PubMed

    Campbell, F; Azadeh, B

    2008-04-01

    The increasing use of radiological imaging has led to greater detection of small and asymptomatic cystic lesions of the pancreas. Most are resectable, but not all are neoplastic. This review provides an update on the histopathology, immunohistochemistry, molecular biology, pathogenesis and management of cystic neoplasms of the exocrine pancreas. These include the serous, the mucinous cystic, the intraductal papillary mucinous and the solid pseudopapillary neoplasms. Recently reported variants are described and very rare cystic variants of other pancreatic epithelial and mesenchymal neoplasms are briefly mentioned.

  12. Longitudinal changes in abdominal fat distribution with menopause.

    PubMed

    Franklin, Ruth M; Ploutz-Snyder, Lori; Kanaley, Jill A

    2009-03-01

    Increases in abdominal fat have been reported with menopause, but the impact of menopause on abdominal fat distribution (visceral vs subcutaneous) is still unclear. The objective of the study was to determine if abdominal fat content (volume) or distribution is altered with menopause. Magnetic resonance imaging was used to quantify total abdominal, subcutaneous, and visceral fat in 8 healthy women, both in the premenopausal state and 8 years later in the postmenopausal state. Physical activity (PA) and blood lipids were also measured. Body weight and waist circumference did not change with menopause (pre- vs postmenopause: body weight, 63.2 +/- 3.1 vs 63.9 +/- 2.5 kg; waist circumference, 92.1 +/- 4.6 vs 93.4 +/- 3.7 cm); however, total abdominal fat, subcutaneous fat, and visceral fat all significantly (P < .05) increased with menopause (pre- vs postmenopause: total, 27 154 +/- 4268 vs 34 717 +/- 3272 cm(3); subcutaneous, 19 981 +/- 3203 vs 24 918 +/- 2521 cm(3); visceral, 7173 +/- 1611 vs 9798 +/- 1644 cm(3)). Although absolute adiposity changed with menopause, relative fat distribution was not significantly different after menopause (pre- vs postmenopause: subcutaneous, 73% +/- 3% vs 71% +/- 3%; visceral, 26% +/- 3% vs 28% +/- 3%). Lean mass, fat mass, and PA, along with total cholesterol and triglyceride levels, did not change with menopause. High-density lipoprotein and low-density lipoprotein both increased (P < .05), and the ratio of total cholesterol to high-density lipoprotein decreased (P < .05) with menopause. As measured longitudinally with magnetic resonance imaging, total abdominal fat content increased with menopause despite no change in PA, body weight, or waist circumference; however, menopause did not affect the relative abdominal fat distribution in these women.

  13. Extraskeletal Ewing sarcoma of the abdominal wall

    PubMed Central

    Farhat, L. Ben; Ghariani, B.; Rabeh, A.; Dali, N.; Said, W.; Hendaoui, L.

    2008-01-01

    Abstract Ewing sarcoma is most commonly a bone tumour which has usually extended into the soft tissues at the time of diagnosis. Exceptionally, this tumour can have an extraskeletal origin. Clinical or imaging findings are non-specific and diagnosis is based on histology. We report a case of an extraskeletal Ewing sarcoma developed in the soft tissues of the abdominal wall in a 35-year-old woman who presented a painful abdominal wall tumefaction. Ultrasongraphy and computed tomography showed a large, well-defined soft tissue mass developed in the left anterolateral muscle group of the abdominal wall. Surgical biopsy was performed and an extraskeletal Ewing sarcoma was identified histologically. PMID:18818133

  14. Magnetic resonance imaging as a diagnostic tool in case of ovarian masses in girls and young women.

    PubMed

    Bekiesińska-Figatowska, Monika; Jurkiewicz, Elzbieta; Iwanowska, Beata; Uliasz, Maria; Romaniuk-Doroszewska, Anna; Bragoszewska, Hanna; Ceran, Alicja; Olszewski, Andrzej

    2007-05-01

    Gynecological examination and transvaginal ultrasound are difficult or impossible in girls and young women who have not started their sexual life. CT is not a method of choice in this age group because of the ionizing radiation and iodine-containing contrast media. MRI is chosen then. Pelvic MRI was performed in 15 patients aged 9-19 years with suspected ovarian mass after they had had unclear gynecological and sonographic examinations. 1.5 T MRI systems were used. SE,T(F)SE and SPIR sequences were applied in T1- and T2-weighted images in three planes. Contrast media were administered in 7 patients. In a group of 3 girls with acute abdominal pain, polycystic ovaries, ovarian hemorrhagic cyst, and fibroma of the ovary were diagnosed. In a group of 11 patients with chronic abdominal pain, dermoid cysts of the ovaries were found in 7 cases, in one bilateral and accompanied by ectopic kidney. In 2 patients, serous cysts were diagnosed. In 2 cases an ovarian origin of the mass was excluded: multilocular cystic lesion in the presacral region and a hydatid mole were revealed. A neoplastic ovarian mass was diagnosed in a girl with increasing circumference of the abdomen. The accuracy of MRI in localizing lesions was 100%. Its accuracy in precisely characterizing lesions was 83.3%. Magnetic resonance imaging, with its noninvasiveness, high spatial resolution, and tissue specificity, is a method of choice in the diagnosis or exclusion of ovarian pathology in children and adolescents.

  15. Reproducibility of Regional DEXA Examinations of Abdominal Fat and Lean Tissue

    PubMed Central

    Tallroth, Kaj; Kettunen, Jyrki A.; Kujala, Urho M.

    2013-01-01

    Objective The aim of this study was to develop and test the validity of a new repeatable method to delimit abdominal areas for follow-up of fat mass (FM) and lean tissue mass (LM) in DEXA examinations. Methods 37 male volunteers underwent two DEXA examinations. Total body FM and LM measurements and corresponding abdominal measurements in a carefully defined region were calculated from the first scan. After repositioning of the subjects and a second scan, the delimited region was copied and the abdominal tissues re-calculated. Results The mean LM of the abdominal area was 2.804 kg (SD 0.556), and the mean FM was 1.026 kg (SD 0.537). The intra-class correlation coefficient for the repeated abdominal LM, FM, and LM/FM ratio measurements was 0.99. The mean difference (bias) for the repeated abdominal LM measurements was −13 g (95% confidence interval (CI) −193.0 to 166.8), and for the repeated abdominal FM measurements it was −35 g (95% CI −178.9 to 108.5). Conclusions The results indicate that regional DEXA is a sensitive method with excellent reproducibility in the measurements of the abdominal fat and lean tissues. The method may serve as a useful tool for evaluation and follow-up of various dietary and training programmes. PMID:23615566

  16. Prophylactic Embolization of the Cystic Artery Prior to Radioembolization of Liver Malignancies—An Evaluation of Necessity

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Powerski, Maciej, E-mail: maciej.powerski@med.ovgu.de; Busse, Anke, E-mail: anke.busse@med.ovgu.de; Seidensticker, Max, E-mail: max.seidensticker@med.ovgu.de

    PurposePrior to radioembolization (RE) of hepatic tumors, many centers prophylactically occlude the cystic artery (CA) during evaluation angiography (EVA) to prevent radiation-induced cholecystitis. There is no conclusive evidence for the protective effect of CA embolization and it bears the risk of inducing ischemic cholecystitis. The aim of this study is to evaluate the justification for CA embolization by comparing clinical and morphologic imaging parameters between patients undergoing coil occlusion of the cystic artery (COCA) and those with uncoiled CA (UCCA).Materials and MethodsRetrospective comparison of 37 patients with UCCA versus 68 patients with COCA in terms of clinical findings (CRP, leukocytemore » count, body temperature, upper abdominal pain) and morphologic imaging parameters associated with cholecystitis (gallbladder (GB) wall thickness, free fluid in GB bed, bremsstrahlung SPECT) after EVA, after RE, and at 6-week follow-up.ResultsAt none of the 3 time points (EVA, RE, 6-week follow-up) was there any significant difference in CRP, leukocyte count, body temperature, or upper abdominal pain between the UCCA and COCA group. There was also no significant difference between the two groups with regard to GB wall thickness, fluid in the GB bed, and bremsstrahlung in SPECT. One patient of the UCCA group and two patients of the COCA developed cholecystitis requiring treatment.ConclusionComparison of clinical and imaging findings between patients with and without CA embolization prior to RE identified no predictors of radiogenic or ischemic cholecystitis after RE. Our study provides no evidence for a benefit of prophylactic CA embolization before RE.« less

  17. Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases

    PubMed Central

    Zhang, Youming; Xiao, Desheng; Yin, Hongling; Long, Xueying; Li, Li; Zai, Hongyan; Chen, Minfeng; Li, Wenzheng; Sun, Lunquan

    2017-01-01

    Objectives To characterize the imaging and clinicopathological features of primitive neuroectodermal tumors (PNETs) arising in intra-abdominal and retroperitoneal regions. Methods Eighteen patients with histopathologically proven intra-abdominal and retroperitoneal PNET were enrolled; computed tomography was performed for all cases, and magnetic resonance imaging was performed for a single case. Typical computed tomography and magnetic resonance imaging findings, including morphology, texture and enhancement features, as well as clinicopathological characteristics and prognosis data were retrospectively analyzed. Results Of eighteen PNET patients, fifteen were male and three were female, with a median age of 36 years (range, 2–65 years). The onset of symptoms was most often nonspecific and insidious. The mean tumor diameter was 7.2 cm (range, 3.0–12.1 cm), with necrosis in fifteen cases, cystic changes in eight, partition structure in five, calcification in five, hemorrhage in two, and mural nodules in one. Contrast enhanced computed tomography showed multiple tiny feeding arteries within the masses in six cases, resulting in a crab-like appearance, and mild ring enhancement pattern in five cases. Eleven cases showed surrounding invasion and metastasis. Of the eighteen PNET cases, nine cases showed smooth, well-defined margins, and nine cases had irregular, ill-defined margins. A median survival was 10.0±1.6 months. However, chemotherapy had efficacy on patients even those with advanced disease. Conclusions Primary intra-abdominal and retroperitoneal PNETs are rare, and imaging features documented here may help the diagnosis of this severe disease. Notably, two signs present in retroperitoneal PNET tumors, including a mild ring enhancement pattern and a crab-like appearance of the tiny feeding arteries, may have the potential to help us improve the ability to make a relatively reliable diagnosis. PMID:28319177

  18. Voice Disorder in Cystic Fibrosis Patients

    PubMed Central

    Lourenço, Bruna Mendes; Costa, Kauê Machado; da Silva Filho, Manoel

    2014-01-01

    Cystic fibrosis is a common autosomal recessive disorder with drastic respiratory symptoms, including shortness of breath and chronic cough. While most of cystic fibrosis treatment is dedicated to mitigating the effects of respiratory dysfunction, the potential effects of this disease on vocal parameters have not been systematically studied. We hypothesized that cystic fibrosis patients, given their characteristic respiratory disorders, would also present dysphonic symptoms. Given that voice disorders can severely impair quality of life, the identification of a potential cystic fibrosis-related dysphonia could be of great value for the clinical evaluation and treatment of this disease. We tested our hypothesis by measuring vocal parameters, using both objective physical measures and the GRBAS subjective evaluation method, in male and female cystic fibrosis patients undergoing conventional treatment and compared them to age and sex matched controls. We found that cystic fibrosis patients had a significantly lower vocal intensity and harmonic to noise ratio, as well as increased levels of jitter and shimmer. In addition, cystic fibrosis patients also showed higher scores of roughness, breathiness and asthenia, as well as a significantly altered general grade of dysphonia. When we segregated the results according to sex, we observed that, as a group, only female cystic fibrosis patients had significantly lower values of harmonic to noise ratio and an abnormal general grade of dysphonia in relation to matched controls, suggesting that cystic fibrosis exerts a more pronounced effect on vocal parameters of women in relation to men. Overall, the dysphonic characteristics of CF patients can be explained by dysfunctions in vocal fold movement and partial upper airway obstruction, potentially caused by the accumulation of mucus and chronic cough characteristic of CF symptomatology. Our results show that CF patients exhibit significant dysphonia and suggest they may

  19. Vitamin A supplementation for cystic fibrosis.

    PubMed

    Bonifant, Catherine M; Shevill, Elizabeth; Chang, Anne B

    2014-05-14

    People with cystic fibrosis and pancreatic insufficiency are at risk of fat soluble vitamin deficiency as these vitamins (A, D, E and K) are co-absorbed with fat. Thus, some cystic fibrosis centres routinely administer these vitamins as supplements but the centres vary in their approach of addressing the possible development of deficiencies in these vitamins. Vitamin A deficiency causes predominantly eye and skin problems while supplementation of vitamin A to excessive levels may cause harm to the respiratory and skeletal systems in children. Thus a systematic review on vitamin A supplementation in people with cystic fibrosis would help guide clinical practice. To determine if vitamin A supplementation in children and adults with cystic fibrosis:1. reduces the frequency of vitamin A deficiency disorders;2. improves general and respiratory health;3. increases the frequency of vitamin A toxicity. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 07 April 2014. All randomised or quasi-randomised controlled trials comparing all preparations of oral vitamin A used as a supplement compared to either no supplementation (or placebo) at any dose and for any duration, in children or adults with cystic fibrosis (defined by sweat tests or genetic testing) with and without pancreatic insufficiency. No relevant studies for inclusion were identified in the search. No studies were included in this review. As there were no randomised or quasi-randomised controlled trials identified, we cannot draw any conclusions on the benefits (or otherwise) of regular administration of vitamin A in people with cystic fibrosis. Until further data are available, country or region specific guidelines on the use of

  20. Mature Cystic Teratoma with Co-existent Mucinous Cystadenocarcinoma in the same Ovary-A Diagnostic Dilemma

    PubMed Central

    Roy, Sanjeet; Gupta, Mayank; Chandramohan, Anuradha

    2016-01-01

    Mature cystic teratoma co-existing with a mucinous cystadenocarcinoma is an infrequently encountered entity with only a handful of cases reported till date. The possibilities in such a case are either a malignant transformation of a benign teratoma into adenocarcinoma or a collision tumor between a mature cystic teratoma and a mucinous tumour of either a primary ovarian surface epithelial-stromal origin or a secondary from a primary gastrointestinal tract tumour. The importance of distinguishing between the two entities has significant bearing on subsequent therapeutic management. We report a rare case of a mature cystic teratoma co-existing with a mucinous cystadenocarcinoma in the same ovary in a 44-year-old lady. Contrast Enhanced Computed Tomography (CECT) imaging of the left ovarian mass was suggestive of a teratoma but an intra-operative frozen section examination was reported as an adenocarcinoma with a cystic teratoma. Gross examination of the surgical specimen revealed a dermoid cyst with another multi-septated cystic lesion containing mucoid material. Histopathological examination showed a mature cystic teratoma and an associated well differentiated mucinous cystadenocarcinoma. The latter displayed a CK7-ve/CK20+ve immunoprofile. In absence of clinical, biochemical or radiological findings of a primary lower gastrointestinal tract tumour, the immunoprofile suggested the possibility of adenocarcinomatous transformation in a benign teratoma. PMID:28208869

  1. Pseudotumors after primary abdominal lipectomy as a new sequela in patients with abdominal apron.

    PubMed

    Dragu, Adrian; Bach, Alexander D; Polykandriotis, Elias; Kneser, Ulrich; Horch, Raymund E

    2009-11-01

    Malnutrition and overweight is a common problem in modern societies. Primary abdominal lipectomy is a standard surgical tool in patients with these problems. However, unknown secondary problems result from recent advances in obesity surgery. Plication of the anterior musculoaponeurotic wall is a widely and commonly used operative technique during abdominoplasty. Many different plication techniques have been published. So far no common standard and long-term effectiveness is proven. In addition, there is no sufficient literature dealing with the postoperative risks of plication of the musculoaponeurotic wall. Four patients with development of pseudotumors were reviewed. All four patients received 12 months in advance a primary abdominal lipectomy including a vertical plication of the musculoaponeurotic wall. All four patients were females with mean age of 61 years and mean body mass index (BMI) of 37 kg/m(2). All four patients had developed a pseudotumor of the abdomen as a long-term complication more than 12 months after primary abdominal lipectomy including a vertical plication of the anterior rectus sheath. One should be aware of the potential long-term risk of secondary postoperative hematoma formation, with or without partial necrosis of the anterior rectus sheath after vertical plication of the anterior musculoaponeurotic wall. Viewed clinically and radiologically, such sequelas may appear as pseudotumor like masses and require immediate revision.

  2. Traumatic abdominal hernia complicated by necrotizing fasciitis.

    PubMed

    Martínez-Pérez, Aleix; Garrigós-Ortega, Gonzalo; Gómez-Abril, Segundo Ángel; Martí-Martínez, Eva; Torres-Sánchez, Teresa

    2014-11-01

    Necrotizing fasciitis is a critical illness involving skin and soft tissues, which may develop after blunt abdominal trauma causing abdominal wall hernia and representing a great challenge for physicians. A 52-year-old man was brought to the emergency department after a road accident, presenting blunt abdominal trauma with a large non-reducible mass in the lower-right abdomen. A first, CT showed abdominal hernia without signs of complication. Three hours after ICU admission, he developed hemodynamic instability. Therefore, a new CT scan was requested, showing signs of hernia complication. He was moved to the operating room where a complete transversal section of an ileal loop was identified. Five hours after surgery, he presented a new episode of hemodynamic instability with signs of skin and soft tissue infection. Due to the high clinical suspicion of necrotizing fasciitis development, wide debridement was performed. Following traumatic abdominal wall hernia (TAWH), patients can present unsuspected injuries in abdominal organs. Helical CT can be falsely negative in the early moments, leading to misdiagnosis. Necrotizing fasciitis is a potentially fatal infection and, consequently, resuscitation measures, wide-spectrum antibiotics, and early surgical debridement are required. This type of fasciitis can develop after blunt abdominal trauma following wall hernia without skin disruption.

  3. Intra-cystic concentrations of albendazole-sulphoxide in human cystic echinococcosis: a systematic review and analysis of individual patient data.

    PubMed

    Lötsch, Felix; Naderer, Judith; Skuhala, Tomislava; Groger, Mirjam; Auer, Herbert; Kaczirek, Klaus; Waneck, Fredrik; Ramharter, Michael

    2016-08-01

    Cystic echinococcosis (CE) is a widespread zoonosis caused by the species complex Echinococcus granulosus. Albendazole (ABZ)-the first-line anthelminthic drug for medical treatment of CE-is metabolized in vivo to the active derivative ABZ-sulphoxide (ABZ-SO). Target-site ABZ-SO concentrations in the hydatid cyst mediate the anthelminthic effect in CE. Primary outcome of this systematic review of individual patient data was the intra-cystic ABZ-SO concentration stratified by cyst size, location, calcification status and use of praziquantel. Studies reporting intra-cystic ABZ-SO concentrations in humans were identified by a systematic search. A pooled analysis of individual patient data was performed to assess intra-cystic concentrations. Pharmacokinetic data of 121 individual cysts were analysed. There was no correlation between plasma and intra-cystic ABZ-SO concentrations (rho = -0.03, p = 0.76). Intra-cystic drug concentrations were also not associated with sex and treatment duration. Use of praziquantel in combination with ABZ was associated with higher plasma (median 540 vs. 240 μg/L; p = 0.04) but not intra-cystic ABZ-SO concentrations (median 220 vs. 199 μg/L; p = 0.36). Relative drug concentrations in hepatic cysts were higher than in other cysts (0.8 vs. 0.4; p = 0.05). Intra-cystic concentrations were higher in calcified than non-calcified cysts (median 897 vs. 245 μg/L; p = 0.03). There was a trend towards higher intra-cystic concentrations in smaller sized cysts (β = -17.2 μg/L/cm; 95th CI, -35.9 to 1.6; p = 0.07). This study demonstrates that mean intra-cystic drug concentrations are similar to plasma concentrations on a population level. However, in individual patients plasma concentrations are not directly predictive for intra-cystic concentrations. The use of booster drugs was not associated with higher intra-cystic ABZ-SO concentrations in this analysis.

  4. Does intra-abdominal fluid increase the resting energy expenditure?

    PubMed

    Zarling, E J; Grande, A; Hano, J

    1997-10-01

    In patients with intra-abdominal fluid collection, caloric needs are based on an estimated dry weight. This is done because intra-abdominal fluid has been assumed to be metabolically inactive. One recent study of patients with slowly resolving ascites suggested otherwise. In our study, the effect of intra-abdominal fluid on the resting energy expenditure (REE) and apparent lean body mass was determined in 10 stable patients requiring peritoneal dialysis. For each subject, in both the empty and full state, we measured REE by indirect calorimetry, and body composition by the bioelectric impedance method. In the full state, the VCO2 was significantly increased (210 +/- 11 versus 197 +/- 9 mL/min, P < 0.02) compared with the empty state. This caused an increase in the calculated resting energy expenditure (1531 +/- 88 kcal/d empty versus 1593 +/- 94 kcal/d full, P < 0.05). The magnitude of increase in REE was similar to the expected calories derived from glucose absorbed out of the dialysate. Estimates of body fat, lean body mass, and total water also were not affected by the intra-abdominal fluid. We conclude that intra-abdominal fluid will not affect the measured REE and hence may be considered to be metabolically inactive.

  5. Hyperandrogenism Accompanies Increased Intra-Abdominal Fat Storage in Normal Weight Polycystic Ovary Syndrome Women

    PubMed Central

    Akopians, Alin L.; Madrigal, Vanessa K.; Ramirez, Emmanuel; Margolis, Daniel J.; Sarma, Manoj K.; Thomas, Albert M.; Grogan, Tristan R.; Haykal, Rasha; Schooler, Tery A.; Okeya, Bette L.; Abbott, David H.; Chazenbalk, Gregorio D.

    2016-01-01

    Context: Normal weight polycystic ovary syndrome (PCOS) women may have altered adipose structure-function underlying metabolic dysfunction. Objective: This study examines whether adipose structure-functional changes exist in normal weight PCOS women and correlate with hyperandrogenism and/or hyperinsulinemia. Design: This is a prospective cohort study. Setting: The setting was an academic medical center. Patients: Six normal weight PCOS women and 14 age- and body mass index-matched normoandrogenic ovulatory (NL) women were included. Intervention(s): All women underwent circulating hormone and metabolic measurements; frequently sampled intravenous glucose tolerance testing; total body dual-energy x-ray absorptiometry; abdominal magnetic resonance imaging; and SC abdominal fat biopsy. Main Outcome Measure(s): Circulating hormones and metabolites, body fat and its distribution, and adipocyte size were compared between PCOS and NL women, and were correlated with each other in all women. Results: Circulating LH and androgen levels were significantly greater in PCOS than NL women, as were fasting insulin levels, pancreatic β-cell responsiveness to glucose, and total abdominal fat mass. Intra-abdominal fat mass also was significantly increased in PCOS women and was positively correlated with circulating androgen, fasting insulin, triglyceride, and non-high-density lipoprotein cholesterol levels in all women. SC abdominal fat mass was not significantly increased in PCOS women, but contained a greater proportion of small SC abdominal adipocytes that positively correlated with serum androgen levels in all women. Conclusion: Hyperandrogenism in normal weight PCOS women is associated with preferential intra-abdominal fat deposition and an increased population of small SC abdominal adipocytes that could constrain SC adipose storage and promote metabolic dysfunction. PMID:27571186

  6. [Giant cystic lymphangioma breast. Report of a case with 20-year follow-up and review of the literature].

    PubMed

    Vargas-Hernández, Víctor Manuel; Tovar-Rodríguez, José María; Moreno-Eutimio, Mario Adán; Acosta-Altamirano, Gustavo

    2014-01-01

    Lymphangiomas are congenital malformations or acquired (secondary to trauma, infection or neoplasia) in the mammary gland, are extremely rare. These lesions tend to infiltrate surrounding tissues and malignant degeneration is extremely rare. Clinically manifests as benign masses, slow growing; diagnosed clinically and by imaging studies. Suegery with removal of the mass is performed for aesthetic reasons and to make differential diagnosis with other common injuries. Women 45 years of age with progressive increase in size of the left breast, breast trauma concerns and has no other symptoms.The histologic diagnosis was cystic lymphangioma giant left mammary gland. Lymphangiomas are uncommon lesions and extremely rare in the mammary gland, locally aggresive behavior and are benign, where abnormal lymphatic tissue has some ability to proliferate and accumulate large amounts of liquid, representing cystic appearance, as presented in our case. Local surgical excision is the treatment. In this first case of giant breast cystic lymphangioma reported in Mexico, which corroborates the benignity of the lesion.

  7. Pancreatic cystic tumors.

    PubMed

    Salvia, R; Festa, L; Butturini, G; Tonsi, A; Sartori, N; Biasutti, C; Capelli, P; Pederzoli, P

    2004-04-01

    Cystic tumors of the pancreas are less frequent than other tumors in neoplastic pancreatic pathology, but in recent years the literature has reported an increasing number. After the first report by Becourt in 1830, cystic tumors were classified into 2 different types by Compagno and Oertel in 1978: benign tumors with glycogen-rich cells and mucinous cystic neoplasms with overt and latent malignancy. The WHO classification of exocrine tumors of the pancreas, published in 1996, is based on the histopathological features of the epithelial wall, which are the main factor in differential diagnosis with cystic lesions of the pancreas. Thanks to the knowledge acquired up to now, a surgical procedure is not always required because the therapeutic choice is conditioned by the correct classification of this heterogeneous group of tumors. Clinical signs are not really useful in the clinical work up, most patients have no symptoms and when clinical signs are present, they may help us to pinpoint the organ of origin but never to identify the type of pathology. In the last few years, the great improvement in imaging has enabled us not only to discriminate cystic from solid lesions, but also to identify the features of the lesions and label them preoperatively. More invasive diagnostic procedures such as fine needle aspiration and intracystic fluid tumor marker level are not really useful because they are not sensitive and the cystic wall can show different degrees of dysplasia and de-epithelialization. These are the reasons for sending the entire specimen to pathology. Good cooperation between surgeons, pathologists, radiologists and gastroenterologists is mandatory to increase the chances of making a proper diagnosis. Therefore, we must analyze all the information we have, such as age, sex, clinical history, location of the tumor and radiological features, in order to avoid the mistake of treating a cystic neoplasm as a benign lesion or as a pseudocyst, as described in the

  8. Differential tumor microenvironment in human ovarian cystic tumors.

    PubMed

    Tavares Murta, Beatriz Martins; Cunha, Fernando de Queiróz; Miranda, Rodrigo; Adad, Sheila Jorge; Murta, Eddie Fernando Candido

    2004-01-01

    Cells and soluble mediators obtained from tumor effusions are useful in evaluating the tumor microenvironment. Our aim was to examine cytologically and to quantify the leukocyte infiltrate, nitric oxide, cytokines and tumor markers in the intracystic fluid from patients with a cystic adnexal mass, for a possible differentiation between benign and malignant findings. Sixty-six women who had their cystic fluids collected were prospectively divided into benign tumor (22, 33.3%), malignant tumor (10, 15.2%) or other gynecological alterations (34, 51.5%). Cytology, total and differential leukocyte counts were determined by light microscopy. Tumor markers, cytokines and nitric oxide were assayed in the supernatants using the Immulite system, ELISA and Griess reaction, respectively. The sensitivity and specificity of the cytological analysis was 66.7% and 97.7%, respectively. The levels of CA 19.9, CA 15.3, alpha-fetoprotein, carcinoembryonic antigen, progesterone and beta-HCG were significantly higher in the benign and/or malignant group than in the other gynecological alterations. Also, the local concentrations of CA 15.3 and beta-HCG were significantly higher in malignant than in benign tumors. In malignant tumors, increased leukocyte counts and higher concentrations of IL-6, IL-10 and nitric oxide were detected than in benign tumors or other gynecological alterations. In malignant tumors, the microenvironment could be differentiated from benign tumors or other gynecological alterations by cystic fluid analysis.

  9. Cystic fibrous dysplasia in the long bone.

    PubMed

    Bahk, Won-Jong; Kang, Yong-Koo; Rhee, Seung-Koo; Chung, Yang-Guk; Lee, An-Hee; Bahk, Yong-Whee

    2007-10-01

    Prominent osteolysis associated with "ground glass" density of fibrous dysplasia may indicate cystic change or sarcomatous transformation. This complication has been reported only sporadically in the long bones. This article presents clinical, radiographic, and pathologic findings, and outcome of simple curettage and bone graft observed in a series of 8 patients with prominent cystic fibrous dysplasia of the long bone. Magnetic resonance imaging features provide a basis for separation of benign cystic change from malignant transformation. However, biopsy is necessary to distinguish nonspecific cystic degeneration from secondary aneurysmal bone cyst. Simple curettage with allo-chip-bone graft is an effective treatment for cystic fibrous dysplasia.

  10. Retroperitoneal dedifferentiated liposarcoma with huge cystic degeneration: A case report.

    PubMed

    Uchihashi, Kazuyoshi; Matsuyama, Atsuji; Shiba, Eisuke; Kimura, Yoshizo; Ogata, Toshiro; Yabuki, Kei; Harada, Hiroshi; Kubo, Chisachi; Tsuda, Yojiro; Jotatsu, Mao; Hisaoka, Masanori

    2017-05-01

    Prominent cyst formation is an unusual feature of liposarcoma. We report here a case of dedifferentiated liposarcoma with huge cystic change without preoperative chemo- or radiation therapy. The lesion arose in the retroperitoneum juxtaposed to the right kidney of a 67-year-old woman. She underwent a surgical removal of the retroperitoneal cyst. The cystic tumor contained 1600 mL of old bloody fluid, and its wall was composed of edematous, inflamed or sclerosing fibrous tissue with fatty tissue containing abundant atypical stromal cells, which were immunohistochemically positive for MDM2 and CDK4, and demonstrated MDM2 gene amplification by fluorescence in situ hybridization. The wall was contiguous to an atypical lipomatous nodule located in the mesentery. The following surgical specimens of the right hemicolectomy and right nephrectomy revealed atypical cells infiltrating into the subserosa of the colon and the perirenal fat tissue or that in the renal sinus. This case indicates that well differentiated or dedifferentiated liposarcoma should be also considered as a differential diagnosis of perirenal cystic mass. © 2017 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.

  11. Gastrointestinal Manifestations of Cystic Fibrosis

    PubMed Central

    2016-01-01

    Cystic fibrosis has historically been considered a pulmonary disease, but with the increasing life expectancy of these patients, gastrointestinal manifestations are becoming more important. Furthermore, nutritional status is closely linked to pulmonary function and, thus, overall mortality. This article discusses gastrointestinal manifestations (which involve nutritional, pancreatic, hepatobiliary, and, in particular, gastrointestinal tract issues) of cystic fibrosis as well as management of the disease. In addition, the article discusses studies that have been critical to our understanding of gastrointestinal manifestations of cystic fibrosis. PMID:27330503

  12. Abnormal Ion Permeation through Cystic Fibrosis Respiratory Epithelium

    NASA Astrophysics Data System (ADS)

    Knowles, M. R.; Stutts, M. J.; Spock, A.; Fischer, N.; Gatzy, J. T.; Boucher, R. C.

    1983-09-01

    The epithelium of nasal tissue excised from subjects with cystic fibrosis exhibited higher voltage and lower conductance than tissue from control subjects. Basal sodium ion absorption by cystic fibrosis and normal nasal epithelia equaled the short-circuit current and was amiloride-sensitive. Amiloride induced chloride ion secretion in normal but not cystic fibrosis tissue and consequently was more effective in inhibiting the short-circuit current in cystic fibrosis epithelia. Chloride ion-free solution induced a smaller hyperpolarization of cystic fibrosis tissue. The increased voltage and amiloride efficacy in cystic fibrosis reflect absorption of sodium ions across an epithelium that is relatively impermeable to chloride ions.

  13. Giant Follicular Cyst of Ovary in an Adolescent Girl

    PubMed Central

    Gedam, Jaya; Bhalerao, Minal; Nadar, Ponambalaganpathi A

    2014-01-01

    Cystic abdominal lesions are extremely common in adolescent girls and are now diagnosed more frequently due to the availability of better imaging modalities. Presentations as huge cysts have become rare as most of them are diagnosed and treated early. Adolescent girls presenting with huge benign abdominal cysts is not uncommon, most of them due to serious cystadenomas of the ovary, but large follicular cysts are rare. We present a 13-year-old girl who presented with a large abdominal mass which was subsequently diagnosed as juvenile follicular cyst of the ovary. PMID:25177606

  14. Abdominal Adhesions

    MedlinePlus

    ... Clearinghouse What are abdominal adhesions? Abdominal adhesions are bands of fibrous tissue that can form between abdominal ... Esophagus Stomach Large intestine Adhesion Abdominal adhesions are bands of fibrous tissue that can form between abdominal ...

  15. Abdominal Pain

    MedlinePlus

    ... and Recurrent or Functional Abdominal Pain (RAP or FAP) What is abdominal pain? Abdominal pain , or stomachache, ... recurrent abdominal pain (RAP) or functional abdominal pain (FAP)? If your health care provider has ruled out ...

  16. Genetics Home Reference: cystic fibrosis

    MedlinePlus

    ... Foundation) Genetic Testing (1 link) Genetic Testing Registry: Cystic fibrosis Other Diagnosis and Management Resources (5 links) American Society for Reproductive Medicine: Male Infertility Baby's First Test GeneReview: Cystic Fibrosis and Congenital Absence of the Vas Deferens Genomics ...

  17. Induction of ovarian cystic follicles in sheep.

    PubMed

    Christman, S A; Bailey, M T; Head, W A; Wheaton, J E

    2000-10-01

    Cystic follicles are a significant cause of infertility in women, dairy cattle and sheep. Sheep were used as a model to identify factors that may elicit formation of cystic follicles. Insulin resistance and elevated LH activity were tested in overweight ewes because of associations among these factors and the formation of cystic follicles. Sheep were synchronized using a progesterone-releasing pessary and insulin resistance was induced during the synchronization period through administration of bovine somatotropin. Following removal of pessaries follicular growth was stimulated by treatment with eCG or eCG and hCG (PG-600). Follicular growth was monitored via daily transrectal ultrasonography and blood samples were collected for hormonal analyses. Six of 18 ewes had a subnormal or absent preovulatory gonadotropin surge and developed cystic follicles. Neither insulin resistance nor elevated LH activity were associated with formation of cystic follicles. Ewes that developed cystic follicles were heavier (93 +/- 4 kg) than ewes that ovulated (81 +/- 3 kg; P = 0.02). Furthermore, following pessary removal and initiation of daily ultrasonography, ewes that developed cystic follicles lost body weight (-3 +/- 1%), while ovulatory ewes continued to gain body weight (1 +/- 1%; P = 0.005). It is speculated that in heavy ewes metabolic factors associated with acute body weight loss inhibit the positive feedback of estradiol and thereby suppress the preovulatory gonadotropin surge leading to formation of cystic follicles.

  18. What's it Like to Have Cystic Fibrosis?

    MedlinePlus

    ... deal with cystic fibrosis. What Is CF? Cystic fibrosis (CF) is a disease that causes the body to make thick, sticky mucus (say: ... special protein. This protein is defective in cystic fibrosis, producing the thick, sticky mucus that causes problems for people with CF. What Causes CF? ...

  19. The relationship of body mass index and abdominal fat on the radiation dose received during routine computed tomographic imaging of the abdomen and pelvis.

    PubMed

    Chan, Victoria O; McDermott, Shaunagh; Buckley, Orla; Allen, Sonya; Casey, Michael; O'Laoide, Risteard; Torreggiani, William C

    2012-11-01

    To determine the relationship of increasing body mass index (BMI) and abdominal fat on the effective dose acquired from computed tomography (CT) abdomen and pelvis scans. Over 6 months, dose-length product and total milliamp-seconds (mAs) from routine CT abdomen and pelvis scans of 100 patients were recorded. The scans were performed on a 64-slice CT scanner by using an automatic exposure control system. Effective dose (mSv) based on dose-length product, BMI, periumbilical fat thickness, and intra-abdominal fat were documented for each patient. BMI, periumbilical fat thickness, and intra-abdominal fat were compared with effective dose. Thirty-nine men and 61 women were included in the study (mean age, 56.3 years). The mean BMI was 26.2 kg/m(2). The mean effective dose was 10.3 mSv. The mean periumbilical fat thickness was 2.4 cm. Sixty-five patients had a small amount of intra-abdominal fat, and 35 had a large amount of intra-abdominal fat. The effective dose increased with increasing BMI (P < .001) and increasing amounts of intra-abdominal fat (P < .001). For every kilogram of weight, there is a 0.13 mSv increase in effective dose, which is equal to 6.5 chest radiographs per CT examination. For an increase in BMI by 5 kg/m(2), there is a 1.95 mSv increase in effective dose, which is equal to 97.5 chest radiographs per CT examination. Increasing BMI and abdominal fat significantly increases the effective dose received from CT abdomen and pelvis scans. Copyright © 2012 Canadian Association of Radiologists. Published by Elsevier Inc. All rights reserved.

  20. Endoscopic treatment of intraventricular cystic tumors.

    PubMed

    Margetis, Konstantinos; Souweidane, Mark M

    2013-02-01

    Intraventricular cystic tumors constitute a surgical challenge, because of their deep location and the histologically benign nature of most of them. We aim to present concisely, yet comprehensively, the role of neuroendoscopy in the treatment of intraventricular cystic tumors. A literature review searching for applications of endoscopy in the treatment of intraventricular cystic tumors is presented. Our experience is added to the presented data. In controversial issues, a comparison is made with traditional treatment methods. Intraventricular endoscopy has been successfully used in the treatment of the whole range of intraventricular cystic tumors. The most common indication is the treatment of colloid cysts. In the treatment of colloid cysts, a comparison with microsurgical techniques showed that endoscopy is advantageous in regard to operative morbidity and postoperative shunt dependency but is associated with a slightly higher recurrence rate. Intraventricular endoscopy has emerged as a viable option in the treatment of intraventricular cystic tumors. Copyright © 2013 Elsevier Inc. All rights reserved.

  1. Vitamin A supplementation for cystic fibrosis.

    PubMed

    Bonifant, Catherine M; Shevill, Elizabeth; Chang, Anne B

    2012-08-15

    People with cystic fibrosis and pancreatic insufficiency are at risk of fat soluble vitamin deficiency as these vitamins (A, D, E and K) are co-absorbed with fat. Thus, some cystic fibrosis centres routinely administer these vitamins as supplements but the centres vary in their approach of addressing the possible development of deficiencies in these vitamins. Vitamin A deficiency causes predominantly eye and skin problems while supplementation of vitamin A to excessive levels may cause harm to the respiratory and skeletal systems in children. Thus a systematic review on vitamin A supplementation in people with cystic fibrosis would help guide clinical practice. To determine if vitamin A supplementation in children and adults with CF: 1. reduces the frequency of vitamin A deficiency disorders; 2. improves general and respiratory health; 3. increases the frequency of vitamin A toxicity. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 23 May 2012. All randomised or quasi-randomised controlled trials comparing all preparations of oral vitamin A used as a supplement compared to either no supplementation (or placebo) at any dose and for any duration, in children or adults with cystic fibrosis (defined by sweat tests or genetic testing) with and without pancreatic insufficiency. No relevant studies for inclusion were identified in the search. No studies were included in this review. As there were no randomised or quasi-randomised controlled trials identified, we cannot draw any conclusions on the benefits (or otherwise) of regular administration of vitamin A in people with cystic fibrosis. Until further data are available, country or region specific guidelines on the use of vitamin A in

  2. Fetal and infant growth patterns associated with total and abdominal fat distribution in school-age children.

    PubMed

    Gishti, Olta; Gaillard, Romy; Manniesing, Rashindra; Abrahamse-Berkeveld, Marieke; van der Beek, Eline M; Heppe, Denise H M; Steegers, Eric A P; Hofman, Albert; Duijts, Liesbeth; Durmuş, Büşra; Jaddoe, Vincent W V

    2014-07-01

    Higher infant growth rates are associated with an increased risk of obesity in later life. We examined the associations of longitudinally measured fetal and infant growth patterns with total and abdominal fat distribution in childhood. We performed a population-based prospective cohort study among 6464 children. We measured growth characteristics in the second and third trimesters of pregnancy, at birth, and at 6, 12, and 24 months. Body mass index, fat mass index (body fat mass/height(2)), lean mass index (body lean mass/height(2)), android/gynoid fat ratio measured by dual-energy x-ray absorptiometry, and sc and preperitoneal abdominal fat measured by ultrasound at the median age of 6.0 years (90% range, 5.7-7.4). We observed that weight gain in the second and third trimesters of fetal life and in early, mid, and late infancy were independently and positively associated with childhood body mass index (P < .05). Only infant weight gain was associated with higher fat mass index, android/gynoid fat ratio, and abdominal fat in childhood (P < .05). Children with both fetal and infant growth acceleration had the highest childhood body mass index, fat mass index, and sc abdominal fat, whereas children with fetal growth deceleration and infant growth acceleration had the highest value for android/gynoid fat ratio and the lowest value for lean mass index (P < .05). Growth in both fetal life and infancy affects childhood body mass index, whereas only infant growth directly affects measured total body and abdominal fat. Fetal growth deceleration followed by infant growth acceleration may lead to an adverse body fat distribution in childhood.

  3. Association of the Myostatin gene with obesity, abdominal obesity and low lean body mass and in non-diabetic Asian Indians in north India.

    PubMed

    Bhatt, Surya Prakash; Nigam, Priyanka; Misra, Anoop; Guleria, Randeep; Luthra, Kalpana; Jain, S K; Qadar Pasha, M A

    2012-01-01

    To determine the association of the A55T and K153R polymorphisms of the Myostatin gene with obesity, abdominal obesity and lean body mass (LBM) in Asian Indians in north India. A total of 335 subjects (238 men and 97 women) were assessed for anthropometry, % body fat (BF), LBM and biochemical parameters. Associations of Myostatin gene polymorphisms were evaluated with anthropometric, body composition and biochemical parameters. In A55T polymorphism, BMI (p=0.04), suprailiac skinfold (p=0.05), total skinfold (p=0.008), %BF (p=0.002) and total fat mass (p=0.003) were highest and % LBM (p=0.03) and total LBM (Kg) were lowest (p=0.04) in subjects with Thr/Thr genotype as compared to other genotypes. Association analysis of K153R polymorphism showed that subjects with R/R genotype had significantly higher BMI (p=0.05), waist circumference (p=0.04), %BF (p=0.04) and total fat mass (p=0.03), and lower %LBM (p=0.02) and total LBM [(Kg), (p=0.04)] as compared to other genotypes. Using a multivariate logistic regression model after adjusting for age and sex, subjects with Thr/Thr genotype of A55T showed high risk for high %BF (OR, 3.92, 95% Cl: 2.61-12.41), truncal subcutaneous adiposity (OR, 2.9, 95% Cl: 1.57-6.60)] and low LBM (OR, 0.64, 95% CI: 0.33-0.89) whereas R/R genotype of K153R showed high risk of obesity (BMI; OR, 3.2, 95% CI: 1.2-12.9; %BF, OR, 3.6, 95% CI: 1.04-12.4), abdominal obesity (OR, 2.12, 95% CI: 2.71-14.23) and low LBM (OR, 0.61, 95% CI: 0.29-0.79). We report that variants of Myostatin gene predispose to obesity, abdominal obesity and low lean body mass in Asian Indians in north India.

  4. Appetite stimulants for people with cystic fibrosis.

    PubMed

    Chinuck, Ruth; Dewar, Jane; Baldwin, David R; Hendron, Elizabeth

    2014-07-27

    Chronic loss of appetite in cystic fibrosis concerns both individuals and families. Appetite stimulants have been used to help cystic fibrosis patients with chronic anorexia attain optimal body mass index and nutritional status. However, these may have adverse effects on clinical status. The aim of this review is to systematically search for and evaluate evidence on the beneficial effects of appetite stimulants in the management of CF-related anorexia and synthesize reports of any side-effects. Trials were identified by searching the Cochrane Cystic Fibrosis and Genetic Disorders Group's Cystic Fibrosis Trials Register, MEDLINE, Embase, CINAHL, handsearching reference lists and contacting local and international experts.Last search of online databases: 01 April 2014.Last search of the Cystic Fibrosis Trials Register: 08 April 2014. Randomised and quasi-randomised controlled trials of appetite stimulants, compared to placebo or no treatment for at least one month in adults and children with cystic fibrosis. Authors independently extracted data and assessed the risk of bias within eligible trials. Meta-analyses were performed. Three trials (total of 47 recruited patients) comparing appetite stimulants (cyproheptadine hydrochloride and megesterol acetate) to placebo were included; the numbers of adults or children within each trial were not always reported. The risk of bias of the included trials was graded as moderate.A meta-analysis of all three trials showed appetite stimulants produced a larger increase in weight z score at three months compared to placebo, mean difference 0.61 (95% confidence interval 0.29 to 0.93) (P < 0.001) (n = 40) with no evidence of a difference in effect between two different appetite stimulants. One of these trials also reported a significant weight increase with megesterol acetate compared to placebo at six months (n = 17). The three trials reported no significant differences in forced expiratory volume at one second (per cent predicted

  5. Primary intra-abdominal malignant fibrous histiocytoma: a highly aggressive tumor.

    PubMed

    Salemis, Nikolaos S; Gourgiotis, Stavros; Tsiambas, Evangelos; Panagiotopoulos, Nikolaos; Karameris, Andreas; Tsohataridis, Efstathios

    2010-12-01

    Malignant fibrous histiocytoma (MFH) is the most common soft-tissue sarcoma of late adult life occurring predominantly in the extremities. Primary intra-abdominal MFH is a very rare occurrence. The aim of this study is to describe a very rare case of an intra-abdominal MFH with a highly aggressive clinical course. A 67-year-old male was referred to our department with a 2-week history of dull lower abdominal pain and a gradually enlarging right lower abdominal mass, which he first noticed 2 months prior to admission. Computed tomography (CT) scan demonstrated a mass in the right iliac fossa. On exploratory laparotomy, a tumor was found in the right iliac fossa attached to the parietal lateral peritoneum without any evidence of invasion into the adjacent structures. Complete excision of the tumor with clear margins was performed. Histological and immunohistochemical examinations showed a MFH. One month after surgery, while on adjuvant chemotherapy, the patient was readmitted with dyspnea and a slightly palpable mass in the area of the previous radical resection. CT scan revealed local tumor recurrence along with multiple pulmonary metastatic deposits. Unfortunately, despite treatment, the patient died of progressive disease 5 weeks later. Primary intra-abdominal MFH is a very rare but aggressive malignancy with a high tendency of local recurrence and metastatic spread. Early detection and complete surgical excision with clear margins is the treatment of choice. In some cases, however, the tumor can exhibit a highly aggressive clinical course despite radical surgery and adjuvant therapy.

  6. Chloride impermeability in cystic fibrosis

    NASA Astrophysics Data System (ADS)

    Quinton, Paul M.

    1983-02-01

    Cystic fibrosis is the most common fatal genetic disease affecting Caucasians and is perhaps best characterized as an exocrinopathy involving a disturbance in fluid and electrolyte transport1. A high NaCl concentration in the sweat is characteristic of patients with this disease; the basic physiological reason for this abnormality is unknown. We have microperfused isolated sweat ducts from control subjects and cystic fibrosis patients, and report here results which suggest that abnormally low Cl- permeability in cystic fibrosis leads to poor reabsorption of NaCl in the sweat duct, and hence to a high concentration of NaCl in the sweat.

  7. Oral calorie supplements for cystic fibrosis.

    PubMed

    Smyth, Rosalind L; Rayner, Oli

    2017-05-04

    concerns surrounding allocation concealment. There were no significant differences between people receiving supplements or dietary advice alone for change in weight, height, body mass index, z score or other indices of nutrition or growth. Changes in weight (kg) at three, six and 12 months respectively were: mean difference (MD) 0.32 (95% confidence interval (CI) -0.09 to 0.72); MD 0.47 (95% CI -0.07 to 1.02 ); and MD 0.16 (-0.68 to 1.00). Total calorie intake was greater in people taking supplements at 12 months, MD 265.70 (95% CI 42.94 to 488.46). There were no significant differences between the groups for anthropometric measures of body composition, lung function, gastro-intestinal adverse effects or activity levels. Moderate quality evidence exists for the outcomes of changes in weight and height and low quality evidence exists for the outcomes of change in total calories, total fat and total protein intake as results are applicable only to children between the ages of 2 and 15 years and many post-treatment diet diaries were not returned. Evidence for the rate of adverse events in the treatment groups was extremely limited and judged to be of very low quality AUTHORS' CONCLUSIONS: Oral calorie supplements do not confer any additional benefit in the nutritional management of moderately malnourished children with cystic fibrosis over and above the use of dietary advice and monitoring alone. While nutritional supplements may be used, they should not be regarded as essential. Further randomised controlled trials are needed to establish the role of short-term oral protein energy supplements in people with cystic fibrosis and acute weight loss and also for the long-term nutritional management of adults with cystic fibrosis or advanced lung disease, or both.

  8. Cystic Fibrosis (CF) Respiratory Screen: Sputum

    MedlinePlus

    ... for Educators Search English Español Cystic Fibrosis (CF) Respiratory Screen: Sputum KidsHealth / For Parents / Cystic Fibrosis (CF) Respiratory Screen: Sputum What's in this article? What It ...

  9. CXCR4+ granulocytes reflect fungal cystic fibrosis lung disease.

    PubMed

    Carevic, Melanie; Singh, Anurag; Rieber, Nikolaus; Eickmeier, Olaf; Griese, Matthias; Hector, Andreas; Hartl, Dominik

    2015-08-01

    Cystic fibrosis airways are frequently colonised with fungi. However, the interaction of these fungi with immune cells and the clinical relevance in cystic fibrosis lung disease are incompletely understood.We characterised granulocytes in airway fluids and peripheral blood from cystic fibrosis patients with and without fungal colonisation, non-cystic fibrosis disease controls and healthy control subjects cross-sectionally and longitudinally and correlated these findings with lung function parameters.Cystic fibrosis patients with chronic fungal colonisation by Aspergillus fumigatus were characterised by an accumulation of a distinct granulocyte subset, expressing the HIV coreceptor CXCR4. Percentages of airway CXCR4(+) granulocytes correlated with lung disease severity in patients with cystic fibrosis.These studies demonstrate that chronic fungal colonisation with A. fumigatus in cystic fibrosis patients is associated with CXCR4(+) airway granulocytes, which may serve as a potential biomarker and therapeutic target in fungal cystic fibrosis lung disease. Copyright ©ERS 2015.

  10. Calcium Stone Growth in Urine from Cystic Fibrosis Patients and Healthy Controls

    NASA Astrophysics Data System (ADS)

    McSorley, Anita; Jones, Andrew M.; Webb, A. Kevin; Rao, P. Nagaraj; Kavanagh, John P.

    2007-04-01

    Cystic fibrosis patients have an increased risk of renal stone disease. There is some evidence that this may be related to a different excretory pattern of stone risk factors, but an alternative hypothesis, that the urine of cystic fibrosis patients is deficient in urinary inhibitors of crystallization and stone formation has not been tested. Here we have grown calcium stones, in vitro, in the presence of urine from healthy controls and compared this with growth in the presence of urine from cystic fibrosis patients. A stone farm was used to grow twelve calcium stones simultaneously, firstly in artificial urine for about 200 hours and then in 90% whole human urine for another 500 hours. Six of the stones received urine from healthy controls and six received urine from adult cystic fibrosis patients. There were no significant differences in stone mass at any of the key time points or in the overall growth pattern (p>0.05) between stones destined for, or treated with, urine from CF patients and the controls. Human urine greatly inhibited stone growth in vitro but there was no difference in the growth rate in urine from healthy controls and CF patients. This refutes the hypothesis that a tendency for a higher prevalence of urinary stones in CF patients is related to a deficiency in inhibitory activity.

  11. Abdominal tap

    MedlinePlus

    Peritoneal tap; Paracentesis; Ascites - abdominal tap; Cirrhosis - abdominal tap; Malignant ascites - abdominal tap ... abdominal cavity ( most often cancer of the ovaries ) Cirrhosis of the liver Damaged bowel Heart disease Infection ...

  12. Staged abdominal re-operation for abdominal trauma.

    PubMed

    Taviloglu, Korhan

    2003-07-01

    To review the current developments in staged abdominal re-operation for abdominal trauma. To overview the steps of damage control laparotomy. The ever increasing importance of the resuscitation phase with current intensive care unit (ICU) support techniques should be emphasized. General surgeons should be familiar to staged abdominal re-operation for abdominal trauma and collaborate with ICU teams, interventional radiologists and several other specialties to overcome this entity.

  13. Molecular diagnosis of cystic fibrosis.

    PubMed

    Shrimpton, Antony E

    2002-05-01

    A review of the current molecular diagnosis of cystic fibrosis including an introduction to cystic fibrosis, the gene function, the phenotypic variation, who should be screened for which mutation, newborn and couple screening, quality assurance, phenotype-genotype correlation, methods and method limitations, options, statements, recommendations, useful Websites and treatments.

  14. Cystic precursors to invasive pancreatic cancer

    PubMed Central

    Matthaei, Hanno; Schulick, Richard D.; Hruban, Ralph H.; Maitra, Anirban

    2011-01-01

    Improvements in the sensitivity and quality of cross-sectional imaging have led to increasing numbers of patients being diagnosed with cystic lesions of the pancreas. In parallel, clinical, radiological, pathological and molecular studies have improved the systems for classifying these cysts. Patients with asymptomatic serous cystic neoplasms can be managed conservatively with regular monitoring; however, the clinical management of patients with intraductal papillary mucinous neoplasms and mucinous cystic neoplasms is far more challenging, as it is difficult to determine whether these lesions will progress to malignancy. Fortunately, prospective studies have helped to establish that proposed clinical and radiological criteria (the Sendai guidelines) can be used to guide the care of patients with cystic lesions of the pancreas. Despite this progress in imaging and clinical guidelines, sensitive and specific tests have not yet been developed that can reliably predict the histology and biological properties of a cystic lesion. Such biomarkers are urgently needed, as noninvasive precursors of pancreatic cancer are curable, while the vast majority of invasive pancreatic adenocarcinomas are not. PMID:21383670

  15. Cystic fibrosis screening in assisted reproduction.

    PubMed

    Gazvani, Rafet; Lewis-Jones, Iwan

    2006-06-01

    The purpose of this review is to discuss the incidence of cystic fibrosis in the general population, in ethnically diverse populations and specifically in couples needing assisted reproduction caused by male factor subfertility. We review the current understanding of risks for reproductive couples and discuss ideal screening strategies. In ethnically diverse populations, a large difference in clinical sensitivity and birth prevalence exists between the broad racial/ethnic groups examined. Extensive data clearly demonstrate the cost-effectiveness of cystic fibrosis screening. Testing for cystic fibrosis gene mutations is reliable and, with a 26-mutation panel, nearly 90% of possible severe mutations can be detected. To halve the incidence of cystic fibrosis in the community, by offering genetic testing of the fetus if both partners are carrier positive, may also be possible. Recent guidelines suggest that all couples contemplating pregnancy should be informed of molecular screening for cystic fibrosis carrier status for purposes of genetic counselling. In ethnically diverse populations, ethnic-specific mutations should be included in the mutation panels.

  16. Estimating abdominal adipose tissue with DXA and anthropometry.

    PubMed

    Hill, Alison M; LaForgia, Joe; Coates, Alison M; Buckley, Jonathan D; Howe, Peter R C

    2007-02-01

    To identify an anatomically defined region of interest (ROI) from DXA assessment of body composition that when combined with anthropometry can be used to accurately predict intra-abdominal adipose tissue (IAAT) in overweight/obese individuals. Forty-one postmenopausal women (age, 49 to 66 years; BMI, 26 to 37 kg/m(2)) underwent anthropometric and body composition assessments. ROI were defined as quadrilateral boxes extending 5 or 10 cm above the iliac crest and laterally to the edges of the abdominal soft tissue. A single-slice computed tomography (CT) scan was measured at the L3 to L4 intervertebral space, and abdominal skinfolds were taken. Forward step-wise regression revealed the best predictor model of IAAT area measured by CT (r(2) = 0.68, standard error of estimate = 17%) to be: IAAT area (centimeters squared) = 51.844 + DXA 10-cm ROI (grams) (0.031) + abdominal skinfold (millimeters) (1.342). Interobserver reliability for fat mass (r = 0.994; coefficient of variation, 2.60%) and lean mass (r = 0.986, coefficient of variation, 2.67%) in the DXA 10-cm ROI was excellent. This study has identified a DXA ROI that can be reliably measured using prominent anatomical landmarks, in this case, the iliac crest. Using this ROI, combined with an abdominal skinfold measurement, we have derived an equation to predict IAAT in overweight/obese postmenopausal women. This approach offers a simpler, safer, and more cost-effective method than CT for assessing the efficacy of lifestyle interventions aimed at reducing IAAT. However, this warrants further investigation and validation with an independent cohort.

  17. Cystic lymph node enlargement of the neck: filariasis as a rare differential diagnosis in MRI.

    PubMed

    Schick, Christoph; Thalhammer, Axel; Balzer, Jörn O; Abolmaali, Nasreddin; Vogl, Thomas J

    2002-09-01

    Cervical lymph node enlargement is a common feature of most inflammatory and neoplastic entities of the head and neck. Filariasis can lead to lymphangiectasis resembling lymph node enlargement; however, this is a rare differential diagnosis in European patients. As ethnic minorities are increasing throughout Europe and personal mobility, e.g. during holidays, is increasing, such rare differential diagnoses have to be taken into consideration. We present the case of an Iraqi patient referred to as for a suspected cystic lymph node mass that was verified histologically by open MRI biopsy and proved to be a cystic manifestation of filariasis.

  18. Cystic renal tumors: new entities and novel concepts.

    PubMed

    Moch, Holger

    2010-05-01

    Cystic renal neoplasms and renal epithelial stromal tumors are diagnostically challenging and represent some novel tumor entities. In this article, clinical and pathologic features of established and novel entities are discussed. Predominantly cystic renal tumors include cystic nephroma/mixed epithelial and stromal tumor, synovial sarcoma, and multilocular cystic renal cell carcinoma. These entities are own tumor entities of the 2004 WHO classification of renal tumors. Tubulocystic carcinoma and acquired cystic disease-associated renal cell carcinoma are neoplasms with an intrinsically cystic growth pattern. Both tumor types should be included in a future WHO classification as novel entities owing to their characteristic features. Cysts and clear cell renal cell carcinoma frequently coexist within the kidneys of patients with von Hippel-Lindau disease. Sporadic clear cell renal cell carcinomas often contain cysts, usually as a minor component. Some clear cell renal cell carcinomas have prominent cysts, and multilocular cystic renal cell carcinoma is composed almost exclusively of cysts. Recent molecular findings suggest that clear cell renal cancer may develop through cyst-dependent and cyst-independent molecular pathways.

  19. Ovarian mature cystic teratoma with fistula formation into the rectum: a case report.

    PubMed

    Kizaki, Yuichiro; Nagai, Tomonori; Ohara, Ken; Gomi, Yosuke; Akahori, Taichi; Ono, Yoshihisa; Matsunaga, Shigetaka; Takai, Yasushi; Saito, Masahiro; Baba, Kazunori; Seki, Hiroyuki

    2016-01-01

    While ovarian mature cystic teratomas are benign ovarian germ-cell tumors and the most common type of all ovarian tumors, the formation of fistulas into surrounding organs such as the bladder and the intestinal tract is extremely rare. This report documents a case of ovarian mature cystic teratoma with a rectal fistula, thought to be caused by local inflammation. A pelvic mass was diagnosed as an ovarian mature cystic teratoma of approximately 10 cm in diameter on transvaginal ultrasound and magnetic resonance examinations. Endoscopic examination of the lower gastrointestinal tract to investigate diarrhea revealed an ulcerative lesion with hair in the rectal wall adjacent to the ovarian cyst, and formation of a fistula from the ovarian teratoma into the rectum was suspected. Laparotomy revealed extensive inflammatory adhesions between a left ovarian tumor and the rectum. Left salpingo-oophorectomy and upper anterior resection of the rectum were performed. The final pathological diagnosis was ovarian mature cystic teratoma with no malignant findings, together with severe rectal inflammation and fistula formation with no structural disorders such as diverticulitis of the colon or malignant signs. The formation of fistulas and invasion into the neighboring organs are extremely rare complications for ovarian mature cystic teratomas. The invasion of malignant cells into neighboring organs due to malignant transformation of the tumor is reported as the cause of fistula formation into the neighboring organs. A review of 17 cases including the present case revealed that fistula formation due to malignant transformation comprised only 4 cases (23.5 %), with inflammation as the actual cause in the majority of cases (13 cases, 76.5 %). Although malignancy is the first consideration when fistula formation is observed between ovarian tumors and surrounding organs, in mature cystic teratoma, local inflammation is more likely than malignant transformation.

  20. Hepatic and pulmonary cystic echinococcosis in a patient from the Central African Republic.

    PubMed

    Develoux, Michel; Enache-Angoulvant, Adela; Gounant, Valerie; Brian, Emmanuel; Khalil, Antoine; Bazelly, Bernard; Hennequin, Christophe

    2011-03-01

    Apical lung opacity was diagnosed in an asymptomatic 30 year-old woman native of Central African Republic by routine chest X-ray. CT scan demonstrated an excavated pulmonary mass and revealed a simple hepatic cyst. Tuberculosis was suspected but mycobacterial cultures remained negative. Three months later, ultrasonography showed septations within the hepatic lesion suggestive of cystic echinococcosis. The detection of seric anti-Echinococcus antibodies was positive. Hepatic and pulmonary cysts were removed surgically and association with three-month course of albendazole resulted in a favorable outcome. Cystic echinococcosis is exceptional in Central Africa and to our knowledge never reported from the Central African Republic. Copyright © 2011 Elsevier Ltd. All rights reserved.

  1. An oblique muscle hematoma as a rare cause of severe abdominal pain: a case report.

    PubMed

    Shimodaira, Masanori; Kitano, Tomohiro; Kibata, Minoru; Shirahata, Kumiko

    2013-01-18

    Abdominal wall hematomas are an uncommon cause of acute abdominal pain and are often misdiagnosed. They are more common in elderly individuals, particularly in those under anticoagulant therapy. Most abdominal wall hematomas occur in the rectus sheath, and hematomas within the oblique muscle are very rare and are poorly described in the literature. Here we report the case of an oblique muscle hematoma in a middle-aged patient who was not under anticoagulant therapy. A 42-year-old Japanese man presented with a painful, enlarging, lateral abdominal wall mass, which appeared after playing baseball. Abdominal computed tomography and ultrasonography showed a large soft tissue mass located in the patient's left internal oblique muscle. A diagnosis of a lateral oblique muscle hematoma was made and the patient was treated conservatively. Physicians should consider an oblique muscle hematoma during the initial differential diagnosis of pain in the lateral abdominal wall even in the absence of anticoagulant therapy or trauma.

  2. Cystic Fibrosis Associated with Worse Survival After Liver Transplantation.

    PubMed

    Black, Sylvester M; Woodley, Frederick W; Tumin, Dmitry; Mumtaz, Khalid; Whitson, Bryan A; Tobias, Joseph D; Hayes, Don

    2016-04-01

    Survival in cystic fibrosis patients after liver transplantation and liver-lung transplantation is not well studied. To discern survival rates after liver transplantation and liver-lung transplantation in patients with and without cystic fibrosis. The United Network for Organ Sharing database was queried from 1987 to 2013. Univariate Cox proportional hazards, multivariate Cox models, and propensity score matching were performed. Liver transplant and liver-lung transplant were performed in 212 and 53 patients with cystic fibrosis, respectively. Univariate Cox proportional hazards regression identified lower survival in cystic fibrosis after liver transplant compared to a reference non-cystic fibrosis liver transplant cohort (HR 1.248; 95 % CI 1.012, 1.541; p = 0.039). Supplementary analysis found graft survival was similar across the 3 recipient categories (log-rank test: χ(2) 2.68; p = 0.262). Multivariate Cox models identified increased mortality hazard among cystic fibrosis patients undergoing liver transplantation (HR 2.439; 95 % CI 1.709, 3.482; p < 0.001) and liver-lung transplantation (HR 2.753; 95 % CI 1.560, 4.861; p < 0.001). Propensity score matching of cystic fibrosis patients undergoing liver transplantation to non-cystic fibrosis controls identified a greater mortality hazard in the cystic fibrosis cohort using a Cox proportional hazards model stratified on matched pairs (HR 3.167; 95 % CI 1.265, 7.929, p = 0.014). Liver transplantation in cystic fibrosis is associated with poorer long-term patient survival compared to non-cystic fibrosis patients, although the difference is not due to graft survival.

  3. Unilocular cystic lymphangioma of thigh-an extremely rare clinical entity.

    PubMed

    Thakur, Sudhir Kumar

    2010-10-01

    Majority of cystic lymphangiomas are multilocular and occur in neck and axilla. The cystic lymphangioma is also known as cystic hygroma. Unilocular cystic hygroma of neck and breast have been reported in literature and have been termed hydrocele of neck and breast respectively. However unilocular cystic hygroma of thigh is probably being reported for the first time.

  4. Secondary Hypertension, Erythrocytosis, and Unilateral Renal Cystic Disease in a Submariner: A Case Report.

    PubMed

    Forbes, Angela S; Yeo, Fred E

    Erythrocytosis, or increased red blood cell mass, may be primary as in the case of polycythemia vera (PV), or secondary due to a variety of causes related to erythropoietin (EPO) secretion and hypoxia. Chronic pulmonary disease and certain EPO-secreting tumors should be addressed and excluded early during the course of evaluation for a patient presenting with increased red blood cell mass. Inclusion of the JAK2 V617F gene mutation in the recent World Health Organization criteria for the diagnosis of PV allows for facilitated diagnosis and guides therapy. EPO levels can be helpful in diagnosis and guiding therapy, but in the case of cystic renal diseases, EPO levels are often not elevated, creating diagnostic uncertainty. This report describes a case of symptoms directly attributable to erythrocytosis in the setting of negative JAK2 mutation and normal EPO levels. The subsequent discovery of a large cystic renal kidney and PV were the leading diagnostic considerations. 2016.

  5. A painful pulsatile abdominal mass in a young man with elevated blood pressures: an unusual presentation of phaeochromocytoma.

    PubMed

    Lee, B M K; Ti, L K

    2002-08-01

    We report an unusual presentation of phaeochromocytoma in a young man with a painful, pulsatile abdominal mass and elevated blood pressures. This led to a delay in diagnosis and resulted in the administration of triggers of catecholamine release, possibly causing a catecholamine surge. This caused the development of catecholamine-induced cardiomyopathy and multiple organ failure, requiring inotropic and ventilatory support, intra-aortic balloon pump and dialysis. Fortunately, his condition reversed with supportive treatment and alpha-adrenergic blockade. This illustrates the importance of having a high index of suspicion of phaeochromocytoma, especially in young patients with elevated blood pressures.

  6. Intestinal bile acid malabsorption in cystic fibrosis.

    PubMed

    O'Brien, S; Mulcahy, H; Fenlon, H; O'Broin, A; Casey, M; Burke, A; FitzGerald, M X; Hegarty, J E

    1993-08-01

    This study aimed at examining the mechanisms participating in excessive faecal bile acid loss in cystic fibrosis. The study was designed to define the relation between faecal fat and faecal bile acid loss in patients with and without cystic fibrosis related liver disease; to assess terminal ileal bile acid absorption by a seven day whole body retention of selenium labelled homotaurocholic acid (SeHCAT); and to determine if small intestinal bacterial overgrowth contributes to faecal bile acid loss. The study population comprised 40 patients (27 men; median age 18 years) with cystic fibrosis (n = 8) and without (n = 32) liver disease and eight control subjects. Faecal bile acid excretion was significantly higher in cystic fibrosis patients without liver disease compared with control subjects (mean (SEM) 21.5 (2.4) and 7.3 (1.2) micromoles/kg/24 hours respectively; p < 0.01) and patients with liver disease (7.9 (1.3) micromoles/kg/24 hours; p < 0.01). No correlation was found between faecal fat (g fat/24 hours) and faecal bile acid (micromoles 24 hours) excretion. Eight (33%) of cystic fibrosis patients had seven day SeHCAT retention < 10% (normal retention > 20%). SeHCAT retention in cystic fibrosis patients with liver disease was comparable with control subjects (30.0 (SEM) 8.3% v 36.8 (5.9)%; p = NS) while SeHCAT retention in cystic fibrosis patients who did not have liver disease was significantly reduced (19.9 (3.8); p < 0.05). Although evidence of small bowel bacterial overgrowth was present in 40% of patients no relation was found between breath hydrogen excretion, faecal fat, and faecal bile acid loss. The results are consistent with the presence of an abnormality in terminal ideal function in patients with cystic fibrosis who do not have liver disease and that a defect in the ileal absorption of bile acids may be a contributory factor to excessive faecal bile acid loss. Faecal bile acid loss in cystic fibrosis is unrelated to the presence of intraluminal fat

  7. Intestinal bile acid malabsorption in cystic fibrosis.

    PubMed Central

    O'Brien, S; Mulcahy, H; Fenlon, H; O'Broin, A; Casey, M; Burke, A; FitzGerald, M X; Hegarty, J E

    1993-01-01

    This study aimed at examining the mechanisms participating in excessive faecal bile acid loss in cystic fibrosis. The study was designed to define the relation between faecal fat and faecal bile acid loss in patients with and without cystic fibrosis related liver disease; to assess terminal ileal bile acid absorption by a seven day whole body retention of selenium labelled homotaurocholic acid (SeHCAT); and to determine if small intestinal bacterial overgrowth contributes to faecal bile acid loss. The study population comprised 40 patients (27 men; median age 18 years) with cystic fibrosis (n = 8) and without (n = 32) liver disease and eight control subjects. Faecal bile acid excretion was significantly higher in cystic fibrosis patients without liver disease compared with control subjects (mean (SEM) 21.5 (2.4) and 7.3 (1.2) micromoles/kg/24 hours respectively; p < 0.01) and patients with liver disease (7.9 (1.3) micromoles/kg/24 hours; p < 0.01). No correlation was found between faecal fat (g fat/24 hours) and faecal bile acid (micromoles 24 hours) excretion. Eight (33%) of cystic fibrosis patients had seven day SeHCAT retention < 10% (normal retention > 20%). SeHCAT retention in cystic fibrosis patients with liver disease was comparable with control subjects (30.0 (SEM) 8.3% v 36.8 (5.9)%; p = NS) while SeHCAT retention in cystic fibrosis patients who did not have liver disease was significantly reduced (19.9 (3.8); p < 0.05). Although evidence of small bowel bacterial overgrowth was present in 40% of patients no relation was found between breath hydrogen excretion, faecal fat, and faecal bile acid loss. The results are consistent with the presence of an abnormality in terminal ideal function in patients with cystic fibrosis who do not have liver disease and that a defect in the ileal absorption of bile acids may be a contributory factor to excessive faecal bile acid loss. Faecal bile acid loss in cystic fibrosis is unrelated to the presence of intraluminal fat

  8. Anatomical variations of the cystic artery.

    PubMed

    Mlakar, Bostjan; Gadzijev, Eldar M; Ravnik, Dean; Hribernik, Marija

    2003-02-01

    Thorough knowledge about the origin of the cystic artery is surgically important, especially when intraoperative or post-operative bleeding occurs in the gallbladder fossa. The arterial supply of the gallbladder was studied in 81 livers. The gallbladder was supplied by one cystic artery in 86% and by two arteries in 14% of cases. When a single artery was present, it originated from the right hepatic artery in 53% of livers. Other origins included the anterior or the posterior sectional hepatic artery, the replacing right hepatic artery, and in 5% of cases, segmental arteries for segments 4, 5, 6 and 8. When two cystic arteries supplied the gallbladder, both most commonly originated from the right hepatic artery (7% incidence). In 1% of cases, a subsegmental branch for segment 6 and a subsegmental branch for segment 5 respectively, originated from the cystic artery.

  9. Pregnancy and cystic fibrosis: Approach to contemporary management

    PubMed Central

    Tay, George; Callaway, Leonie; Bell, Scott C

    2014-01-01

    Over the previous 50 years survival of patients with cystic fibrosis has progressively increased. As a result of improvements in health care, increasing numbers of patients with cystic fibrosis are now considering starting families of their own. For the health care professionals who look after these patients, the assessment of the potential risks, and the process of guiding prospective parents through pregnancy and beyond can be both challenging and rewarding. To facilitate appropriate discussions about pregnancy, health care workers must have a detailed understanding of the various important issues that will ultimately need to be considered for any patient with cystic fibrosis considering parenthood. This review will address these issues. In particular, it will outline pregnancy outcomes for mothers with cystic fibrosis, issues that need to be taken into account when planning a pregnancy and the management of pregnancy for mothers with cystic fibrosis or mothers who have undergone organ transplantation as a result of cystic fibrosis. PMID:27512443

  10. Cystic Duct Closure by Sealing With Bipolar Electrocoagulation

    PubMed Central

    Damgaard, B.; Jorgensen, L. N.; Larsen, S. S.; Kristiansen, V. B.

    2010-01-01

    Background: Cystic duct leakage after cholecystectomy is not uncommon and is a potentially serious complication. The aim of this study was to assess a bipolar sealing system (LigaSure®) for closure of the cystic duct. Methods: The records from consecutive laparoscopic cholecystectomies performed in 2 hospitals with closure of the cystic duct with LigaSure after informed consent were recorded and complications and morbidity registered. The records were compared with those of patients undergoing laparoscopic cholecystectomy with closure of the cystic duct with clips during the same period. Results: During the study period, 218 laparoscopic cholecystectomies were performed; 102 of these were performed with the LigaSure. One patient was excluded due to violation of the protocol. We experienced no cases of cystic duct leakage, but in one patient, bile leakage from the gallbladder bed was observed probably due to a small aberrant duct. Conclusion: The LigaSure system was safe and effective for closure and division of the cystic duct in laparoscopic cholecystectomy. PMID:20412641

  11. Efficacy of electrocoagulation in sealing the cystic artery and cystic duct occluded with only one absorbable clip during laparoscopic cholecystectomy.

    PubMed

    Yang, Chang-Ping; Cao, Jin-Lin; Yang, Ren-Rong; Guo, Hong-Rong; Li, Zhao-Hui; Guo, Hai-Ying; Shao, Yin-Can; Liu, Gui-Bao

    2014-02-01

    Even though laparoscopic cholecystectomy (LC) emerged over 20 years ago, controversies persist with regard to the best method to ligate the cystic duct and artery. We proposed to assess the effectiveness and safety of electrocoagulation to seal the cystic artery and cystic duct after their occlusion with only one absorbable clip. We retrospectively compared the clinical data for 635 patients undergoing LC using electrocoagulation to seal the cystic artery and cystic duct that were occluded with only one absorbable clip (Group 1) and 728 patients undergoing LC using titanium clips (Group 2). In parallel, 30 rabbits randomized into six groups underwent cholecystectomy. After cystic duct ligation with absorbable or titanium clips, the animals were sacrificed 1, 3, or 6 months later, and intraabdominal adhesions were assessed after celiotomy. The mean operative time was significantly shorter (41.6 versus 58.9 minutes, P<.01) in Group 1 than in Group 2. No cystic duct leaks occurred in any patients from Group 1, compared with seven leaks among the 728 (0.96%) patients from Group 2 (P<.05). The morbidity was significantly higher in Group 2 than in Group 1 (3.43% versus 1.58%). Mean intraoperative blood loss and hospitalization length were not significantly different between the two groups, and no deaths occurred in either group. In animal experiments, adhesion was tighter for absorbable than for titanium clips, but fibrous tissue encapsulation was thinner at the site of titanium clips. Electrocoagulation of the cystic artery and cystic duct that were occluded with only one absorbable clip is safe and effective during LC. This approach is associated with shortened operative times and reduced leakage, compared with the standard method using metal clips.

  12. Diabetes in cystic fibrosis.

    PubMed

    Bridges, Nicola

    2013-05-01

    Cystic fibrosis related diabetes (CFRD) is a common complication of cystic fibrosis, caused by a fall in insulin secretion with age in individuals with pancreatic insufficiency. CFRD is associated with worse clinical status and increased mortality. Treatment of CFRD with insulin results in sustained improvements in lung function and nutrition. While clinical experience with insulin treatment in CF has increased, the selection of who to treat and glycaemic targets remain unclear. Copyright © 2013. Published by Elsevier Ltd.

  13. Evaluation of factors related to bone disease in Polish children and adolescents with cystic fibrosis.

    PubMed

    Sands, Dorota; Mielus, Monika; Umławska, Wioleta; Lipowicz, Anna; Oralewska, Beata; Walkowiak, Jarosław

    2015-09-01

    The aim of the study was to evaluate factors related to bone formation and resorption in Polish children and adolescents with cystic fibrosis and to examine the effect of nutritional status, biochemical parameters and clinical status on bone mineral density. The study group consisted of 100 children and adolescents with cystic fibrosis with a mean age 13.4 years old. Anthropometric measurements, included body height, body mass and body mass index (BMI); bone mineral densitometry and biochemical testing were performed. Bone mineral density was measured using a dual-energy X-ray absorption densitometer. Biochemical tests included serum calcium, phosphorus, parathyroid hormone and vitamin D concentrations, as well as 24-h urine calcium and phosphorus excretion. Pulmonary function was evaluated using FEV1%, and clinical status was estimated using the Shwachman-Kulczycki score. Standardized body height, body mass and BMI were significantly lower than in the reference population. Mean serum vitamin D concentration was decreased. Pulmonary disease was generally mild, with a mean FEV1% of 81%. Multivariate linear regression revealed that the only factors that had a significant effect on bone marrow density were BMI and FEV1%. There were no significant correlations between bone mineral density and the results of any of the biochemical tests performed. Nutritional status and bone mineral density were significantly decreased in children and adolescents with cystic fibrosis. In spite of abnormalities in biochemical testing, the factors that were found to have the strongest effect on bone mineral density were standardized BMI and clinical status. Copyright © 2015. Published by Elsevier Urban & Partner Sp. z o.o.

  14. Singing for children and adults with cystic fibrosis.

    PubMed

    Irons, Jung Yoon; Kenny, Dianna Theadora; Chang, Anne B

    2010-05-12

    Cystic fibrosis is a genetically inherited, life-threatening condition that affects major organs. The management of cystic fibrosis involves a multi-faceted daily treatment regimen that includes airway clearance physiotherapy, taking pancreatic enzymes and other medications. Previous studies identified that compliance with this intensive treatment especially among adolescents with cystic fibrosis is poor. Because of both the nature and consequences of the illness and the relentless demands of treatments, many individuals with cystic fibrosis are likely to have a poor quality of life. Anecdotal evidence suggests that singing may provide rigorous exercises for the whole respiratory system as well as a means for emotional expression, which may enhance quality of life. To evaluate the effects of a singing intervention in addition to usual therapy on the quality of life, morbidity, respiratory muscle strength and pulmonary function of children and adults with cystic fibrosis. We searched the Group's Cystic Fibrosis Trials Register, the Cochrane Central Register of Controlled Trials, major allied complementary data bases, and clinical trial registers. Hand searching for relevant conference proceedings and journals was also carried out.Date of search of Trials Register: 02 September 2009.Date of additional searches: 17 September 2009. Randomised controlled trials in which singing (as an adjunctive intervention) is compared with either a sham intervention or no singing in people with cystic fibrosis. No trials were found that met the selection criteria. No meta-analysis could be performed. As no studies that met the criteria were found, this review is unable to support or refute the benefits of singing as a therapy for people with cystic fibrosis. Future randomised controlled trials are required to evaluate singing therapy for people with cystic fibrosis.

  15. Diagnosing cystic fibrosis-related diabetes: current methods and challenges.

    PubMed

    Prentice, Bernadette; Hameed, Shihab; Verge, Charles F; Ooi, Chee Y; Jaffe, Adam; Widger, John

    2016-07-01

    Cystic fibrosis-related diabetes (CFRD) is the end-point of a spectrum of glucose abnormalities in cystic fibrosis that begins with early insulin deficiency and ultimately results in accelerated nutritional decline and loss of lung function. Current diagnostic and management regimens are unable to entirely reverse this clinical decline. This review summarises the current understanding of the pathophysiology of CFRD, the issues associated with using oral glucose tolerance tests in CF and the challenges faced in making the diagnosis of CFRD. Medline database searches were conducted using search terms "Cystic Fibrosis Related Diabetes", "Cystic Fibrosis" AND "glucose", "Cystic Fibrosis" AND "insulin", "Cystic Fibrosis" AND "Diabetes". Additionally, reference lists were studied. Expert commentary: Increasing evidence points to early glucose abnormalities being clinically relevant in cystic fibrosis and as such novel diagnostic methods such as continuous glucose monitoring or 30 minute sampled oral glucose tolerance test (OGTT) may play a key role in the future in the screening and diagnosis of early glucose abnormalities in CF.

  16. Radiological analysis of cystic lesion in osteonecrosis of the femoral head.

    PubMed

    Gao, Fuqiang; Han, Jun; He, Zike; Li, Zirong

    2018-04-27

    Cystic lesions are a common complication in osteonecrosis of the femoral head (ONFH). This study will discuss the cause of cystic lesion formation and the feature of cystic lesion distribution in ONFH. According to the feature of cystic lesion in ONFH, we will discuss the possible mechanisms of cystic lesions and their  influence on collapse of the femoral head. We retrospectively gathered 102 ONFH patients (168 hips) from November in 2015 to August in 2016 on China-Japan Friendship Hospital. Three categories of patients' medical information were collected: demographic characteristics, bone cystic lesion location, and pathological finding on CT and MRI imaging (microfracture, collapse, crescent sign). On mid-coronal and mid-axial CT section, the femoral head was divided into four quadrants for locating the cystic lesion. And we classified the location relationship of cystic lesion and sclerosis rim as G1 type, G2 type, and G3 type on coronal CT section. A significant difference was found between ONFH group with cystic lesion and ONFH group without cystic lesion in terms of microfracture (P < 0.001), collapse (P < 0.001), and crescent sign (P < 0.001). Forty-four cystic lesions (70%) are located in anterior hip area and 19 cystic lesions (30%) are located in posterior hip area. There were 14, 24, and seven cystic lesions (31, 53, 16%) locating in lateral, central, and medial pillars of the femoral head. G2 type was the most common pattern of location relationship between cystic lesion and sclerosis rim. Cystic lesions are often found near sclerosis rim in ONFH. The femoral head with osteonecrosis complicating by cystic lesions is more likely to accompany microfracture, collapse, and crescent sign which indicate structural instability in the femoral head. Cystic lesion in ONFH plays an important role in aggravating the progression of femoral head collapse. The peak stress from sclerosis rim may be a main factor inducing the formation of cystic lesion in

  17. Modified Multivisceral Transplant After Acute Abdominal Trauma.

    PubMed

    Nikeghbalian, Saman; Alaa Eldin, Ahmed; Aliakbarian, Mohsen; Kazemi, Kourosh; Shamsaeefar, Alireza; Gholami, Siavash; Malekhosseini, Seyed Ali

    2016-04-01

    A 50-year-old man sustained blunt abdominal trauma in a motor vehicle accident. He underwent exploratory laparotomy on the day of trauma, and severe bleeding from the base of the small bowel mesentery was controlled by mass ligation and through-and-through suturing. After transfer to our center, repeat exploratory laparotomy showed ischemic small intestine, ischemic right colon, and severe pancreatic trauma. The severely injured organs were excised including the entire small bowel, pancreas, spleen, stomach, and right hemicolon. The next day, a modified multivisceral transplant was performed including stomach, pancreaticoduodenal complex, and small bowel transplant. Postoperative complications included an intra-abdominal collection that was drained percutaneously with ultrasonographic guidance and severe rejection that was treated with anti-thymocyte globulin. In summary, for select patients who have severe abdominal trauma may be treated with acute multivisceral transplant.

  18. CT fluoroscopy-assisted puncture of thoracic and abdominal masses: a randomized trial.

    PubMed

    Kirchner, Johannes; Kickuth, Ralph; Laufer, Ulf; Schilling, Esther Maria; Adams, Stephan; Liermann, Dieter

    2002-03-01

    We investigated the benefit of real-time guidance of interventional punctures by means of computed tomography fluoroscopy (CTF) compared with the conventional sequential acquisition guidance. In a prospective randomized trial, 75 patients underwent either CTF-guided (group A, n = 50) or sequential CT-guided (group B, n = 25) punctures of thoracic (n = 29) or abdominal (n = 46) masses. CTF was performed on the CT machine (Somatom Plus 4 Power, Siemens Corp., Forchheim, Germany) equipped with the C.A.R.E. Vision application (tube voltage 120 kV, tube current 50 mA, rotational time 0.75 s, slice thickness 10 mm, 8 frames/s). The average procedure time showed a statistically significant difference between the two study groups (group A: 564 s, group B 795 s, P = 0.0032). The mean total mAs was 7089 mAs for the CTF and 4856 mAs for the sequential image-guided intervention, respectively. The sensitivity was 71% specificity 100% positive predictive value 100% and negative predictive value 60% for the CTF-guided puncture, and 68, 100, 100 and 50% for sequential CT, respectively. CTF guidance realizes a time-saving but increases the radiation exposure dosage.

  19. Drug therapies for reducing gastric acidity in people with cystic fibrosis.

    PubMed

    Ng, Sze May; Francini, Angelo J

    2012-04-18

    Malabsorption of fat and protein contributes to poor nutritional status in people with cystic fibrosis. Impaired pancreatic function may also result in increased gastric acidity, leading in turn to heartburn, peptic ulcers and the impairment of oral pancreatic enzyme replacement therapy. The administration of gastric acid-reducing agents has been used as an adjunct to pancreatic enzyme therapy to improve absorption of fat and gastro-intestinal symptoms in people with cystic fibrosis. It is important to establish the evidence regarding potential benefits of drugs that reduce gastric acidity in people with cystic fibrosis. To assess the effect of drug therapies for reducing gastric acidity for: nutritional status; symptoms associated with increased gastric acidity; fat absorption; lung function; quality of life and survival; and to determine if any adverse effects are associated with their use. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches, handsearches of relevant journals, abstract books and conference proceedings.Most recent search of the Group's Trials Register: 15 February 2012. All randomised and quasi-randomised trials involving agents that reduce gastric acidity compared to placebo or a comparator treatment. Both authors independently selected trials, assessed trial quality and extracted data. Thirty-eight trials were identified from the searches. Sixteen trials, with 256 participants, were suitable for inclusion. Seven trials were limited to children and three trials enrolled only adults. Meta-analysis was not performed. However, one trial found that drug therapies that reduce gastric acidity improved gastro-intestinal symptoms such as abdominal pain; seven trials reported significant improvement in measures of fat malabsorption; and two trials reported no significant improvement in nutritional status. Only one trial reported measures

  20. Factors associated with abdominal obesity in children

    PubMed Central

    Melzer, Matheus Ribeiro Theodósio Fernandes; Magrini, Isabella Mastrangi; Domene, Semíramis Martins Álvares; Martins, Paula Andrea

    2015-01-01

    Objective: To identify the association of dietary, socioeconomic factors, sedentary behaviors and maternal nutritional status with abdominal obesity in children. Methods: A cross-sectional study with household-based survey, in 36 randomly selected census tracts in the city of Santos, SP. 357 families were interviewed and questionnaires and anthropometric measurements were applied in mothers and their 3-10 years-old children. Assessment of abdominal obesity was made by maternal and child's waist circumference measurement; for classification used cut-off points proposed by World Health Organization (1998) and Taylor et al. (2000) were applied. The association between variables was performed by multiple logistic regression analysis. Results: 30.5% of children had abdominal obesity. Associations with children's and maternal nutritional status and high socioeconomic status were shown in the univariate analysis. In the regression model, children's body mass index for age (OR=93.7; 95%CI 39.3-223.3), female gender (OR=4.1; 95%CI 1.8-9.3) and maternal abdominal obesity (OR=2.7; 95%CI 1.2-6.0) were significantly associated with children's abdominal obesity, regardless of the socioeconomic status. Conclusions: Abdominal obesity in children seems to be associated with maternal nutritional status, other indicators of their own nutritional status and female gender. Intervention programs for control of childhood obesity and prevention of metabolic syndrome should consider the interaction of the nutritional status of mothers and their children. PMID:26298655

  1. [Factors associated with abdominal obesity in children].

    PubMed

    Melzer, Matheus Ribeiro Theodósio Fernandes; Magrini, Isabella Mastrangi; Domene, Semíramis Martins Álvares; Martins, Paula Andrea

    2015-12-01

    To identify the association of dietary, socioeconomic factors, sedentary behaviors and maternal nutritional status with abdominal obesity in children. A cross-sectional study with household-based survey, in 36 randomly selected census tracts in the city of Santos/SP. 357 families were interviewed and questionnaires and anthropometric measurements were applied in mothers and their 3-0 years-old children. Assessment of abdominal obesity was made by maternal and child's waist circumference measurement; for classification used cut-off points proposed by World Health Organization (1998) and Taylor et al. (2000) were applied. The association between variables was performed by multiple logistic regression analysis. 30.5% of children had abdominal obesity. Associations with children's and maternal nutritional status and high socioeconomic status were shown in the univariate analysis. In the regression model, children's body mass index for age (OR=93.7; 95%CI 39.3-223.3), female gender (OR=4.1; 95%CI 1.8-9.3) and maternal abdominal obesity (OR=2.7; 95%CI 1.2-6.0) were significantly associated with children's abdominal obesity, regardless of the socioeconomic status. Abdominal obesity in children seems to be associated with maternal nutritional status, other indicators of their own nutritional status and female gender. Intervention programs for control of childhood obesity and prevention of metabolic syndrome should consider the interaction of the nutritional status of mothers and their children. Copyright © 2015 Sociedade de Pediatria de São Paulo. Publicado por Elsevier Editora Ltda. All rights reserved.

  2. Chloride and potassium channels in cystic fibrosis airway epithelia

    NASA Astrophysics Data System (ADS)

    Welsh, Michael J.; Liedtke, Carole M.

    1986-07-01

    Cystic fibrosis, the most common lethal genetic disease in Caucasians, is characterized by a decreased permeability in sweat gland duct and airway epithelia. In sweat duct epithelium, a decreased Cl- permeability accounts for the abnormally increased salt content of sweat1. In airway epithelia a decreased Cl- permeability, and possibly increased sodium absorption, may account for the abnormal respiratory tract fluid2,3. The Cl- impermeability has been localized to the apical membrane of cystic fibrosis airway epithelial cells4. The finding that hormonally regulated Cl- channels make the apical membrane Cl- permeable in normal airway epithelial cells5 suggested abnormal Cl- channel function in cystic fibrosis. Here we report that excised, cell-free patches of membrane from cystic fibrosis epithelial cells contain Cl- channels that have the same conductive properties as Cl- channels from normal cells. However, Cl- channels from cystic fibrosis cells did not open when they were attached to the cell. These findings suggest defective regulation of Cl- channels in cystic fibrosis epithelia; to begin to address this issue, we performed two studies. First, we found that isoprenaline, which stimulates Cl- secretion, increases cellular levels of cyclic AMP in a similar manner in cystic fibrosis and non-cystic fibrosis epithelial cells. Second, we show that adrenergic agonists open calcium-activated potassium channels, indirectly suggesting that calcium-dependent stimulus-response coupling is intact in cystic fibrosis. These data suggest defective regulation of Cl- channels at a site distal to cAMP accumulation.

  3. [Abdominal trauma].

    PubMed

    Sido, B; Grenacher, L; Friess, H; Büchler, M W

    2005-09-01

    Blunt abdominal trauma is much more frequent than penetrating abdominal trauma in Europe. As a consequence of improved quality of computed tomography, even complex liver injuries are increasingly being treated conservatively. However, missed hollow viscus injuries still remain a problem, as they considerably increase mortality in multiply injured patients. Laparoscopy decreases the rate of unnecessary laparotomies in perforating abdominal trauma and helps to diagnose injuries of solid organs and the diaphragm. However, the sensitivity in detecting hollow viscus injuries is low and the role of laparoscopy in blunt abdominal injury has not been defined. If intra-abdominal bleeding is difficult to control in hemodynamically unstable patients, damage control surgery with packing of the liver, total splenectomy, and provisional closure of hollow viscus injuries is of importance. Definitive surgical treatment follows hemodynamic stabilization and restoration of hemostasis. Injuries of the duodenum and pancreas after blunt abdominal trauma are often associated with other intra-abdominal injuries and the treatment depends on their location and severity.

  4. New animal models of cystic fibrosis: what are they teaching us?

    PubMed Central

    Keiser, Nicholas W.; Engelhardt, John F.

    2013-01-01

    Purpose of review Cystic fibrosis is the first human genetic disease to benefit from the directed engineering of three different species of animal models (mice, pigs, and ferrets). Recent studies on the cystic fibrosis pig and ferret models are providing new information about the pathophysiology of cystic fibrosis in various organ systems. Additionally, new conditional cystic fibrosis transmembrane conductance regulator (CFTR) knockout mice are teaching unexpected lessons about CFTR function in surprising cellular locations. Comparisons between these animal models and the human condition are key to dissecting the complexities of disease pathophysiology in cystic fibrosis. Recent findings Cystic fibrosis pigs and ferrets have provided new models to study the spontaneous development of disease in the lung and pancreas, two organs that are largely spared overt spontaneous disease in cystic fibrosis mice. New cystic fibrosis mouse models are now interrogating CFTR functions involved in growth and inflammation at an organ-based level using conditional knockout technology. Together, these models are providing new insights on the human condition. Summary Basic and clinical cystic fibrosis research will benefit greatly from the comparative pathophysiology of cystic fibrosis mice, pigs, and ferrets. Both similarities and differences between these three cystic fibrosis models will inform pathophysiologically important mechanisms of CFTR function in humans and aid in the development of both organ-specific and general therapies for cystic fibrosis. PMID:21857224

  5. Enteral tube feeding for cystic fibrosis.

    PubMed

    Conway, S P; Morton, A; Wolfe, S

    2008-04-16

    Enteral tube feeding is routinely used in many cystic fibrosis centres when weight for height percentage is less than 85%, when there has been weight loss for longer than a two-month period or when there has been no weight gain for two to three months (under five years old) or for six months (over five years old). To examine the evidence that in people with cystic fibrosis supplemental enteral tube feeding improves nutritional status, respiratory function, and quality of life without significant adverse effects. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also contacted the companies that market enteral feeds and reviewed their databases. Date of the most recent search of the Group's Cystic Fibrosis Trials Register: November 2007. All randomised controlled trials comparing supplemental enteral tube feeding for one month or longer with no specific intervention in people with cystic fibrosis. Thirteen trials were identified by the search; however, none were eligible for inclusion in this review. There are no trials included in this review. Supplemental enteral tube feeding is widely used throughout the world to improve nutritional status in people with cystic fibrosis. The methods mostly used, nasogastric or gastrostomy feeding, are invasive, expensive, and may have a negative effect on self-esteem and body image. Reported use of enteral tube feeding suggests that it results in nutritional and respiratory improvement and it is disappointing that their efficacy has not been fully assessed by randomised controlled trials. With the more frequent recommendations to use enteral tube feeding as an early rather than a late intervention, this systematic review identifies the need for a multicentre, randomised controlled trial assessing both efficacy and possible

  6. Fatal Airway Obstruction in a Man With a Cystic Hygroma.

    PubMed

    Wygant, Cassandra Maria; Cohle, Stephen D

    2018-05-03

    We describe a 24-year-old man with a cystic hygroma of the left side of the lower neck that led to sudden death. Cystic hygroma (cystic lymphangioma) is a congenital malformation of the lymphatic system. The patient, who had a tracheostomy because of airway obstruction from the cystic hygroma, was found dead with his tracheostomy tube on the floor next to him. Complications of cystic hygroma include infiltration of the neck causing airway obstruction, dysphagia, pain, and obstructive sleep apnea.

  7. Chronic abdominal wall pain misdiagnosed as functional abdominal pain.

    PubMed

    van Assen, Tijmen; de Jager-Kievit, Jenneke W A J; Scheltinga, Marc R; Roumen, Rudi M H

    2013-01-01

    The abdominal wall is often neglected as a cause of chronic abdominal pain. The aim of this study was to identify chronic abdominal wall pain syndromes, such as anterior cutaneous nerve entrapment syndrome (ACNES), in a patient population diagnosed with functional abdominal pain, including irritable bowel syndrome, using a validated 18-item questionnaire as an identification tool. In this cross-sectional analysis, 4 Dutch primary care practices employing physicians who were unaware of the existence of ACNES were selected. A total of 535 patients ≥18 years old who were registered with a functional abdominal pain diagnosis were approached when they were symptomatic to complete the questionnaire (maximum 18 points). Responders who scored at least the 10-point cutoff value (sensitivity, 0.94; specificity, 0.92) underwent a diagnostic evaluation to establish their final diagnosis. The main outcome was the presence and prevalence of ACNES in a group of symptomatic patients diagnosed with functional abdominal pain. Of 535 patients, 304 (57%) responded; 167 subjects (31%) recently reporting symptoms completed the questionnaire. Of 23 patients who scored above the 10-point cutoff value, 18 were available for a diagnostic evaluation. In half of these subjects (n = 9) functional abdominal pain (including IBS) was confirmed. However, the other 9 patients were suffering from abdominal wall pain syndrome, 6 of whom were diagnosed with ACNES (3.6% prevalence rate of symptomatic subjects; 95% confidence interval, 1.7-7.6), whereas the remaining 3 harbored a painful lipoma, an abdominal herniation, and a painful scar. A clinically relevant portion of patients previously diagnosed with functional abdominal pain syndrome in a primary care environment suffers from an abdominal wall pain syndrome such as ACNES.

  8. Cystic hygroma of the neck: single center experience and literature review.

    PubMed

    Damaskos, C; Garmpis, N; Manousi, M; Garmpi, A; Margonis, G-A; Spartalis, E; Doula, C; Michail-Strantzia, C; Patelis, N; Schizas, D; Arkoumanis, P-T; Andreatos, N; Tsourouflis, G; Zavras, N; Markatos, K; Kontzoglou, K; Antoniou, E A

    2017-11-01

    Malformations of the lymphatic system are recognized as benign congenital tumors that affect infant and children in the perinatal era. In children, these abnormalities usually found in the neck and the axillary region, but they can present in other parts of the body such as mediastinum, pelvis, retroperitoneum as well as in solid organs (e.g., adrenal glands, pancreas, stomach). Our aim is to report our experience on cystic hygromas via two cases and review the literature. Herein we present two cases of cystic hygroma, the first of female children and the second of a female adult patient respectively. Both of these patients underwent surgical excision of the masses. After the procedure, both patients have recovered well, and no recurrence of the lesion has been noted during the follow-up period. Surgical treatment remains the gold-standard treatment for these tumors, while other modalities have been used with mixed results.

  9. Desmoplastic fibroma of the rib with cystic change: a case report and literature review.

    PubMed

    Okubo, Taketo; Saito, Tsuyoshi; Takagi, Tatsuya; Suehara, Yoshiyuki; Kaneko, Kazuo

    2014-05-01

    Desmoplastic fibroma (DF) is a rare, locally aggressive, solitary tumor microscopically composed of well-differentiated myofibroblasts with abundant dense collagen deposition. The most common sites are the long tubular bones and mandible. To our knowledge, only five cases of DF in the ribs have been reported. Here, we report a case of DF in this rare location with unusual radiological findings. A 40-year-old man presented with a 4-year history of swelling of the right chest wall. Radiographs revealed a mass at the right 9th rib, and computed tomography demonstrated a mass of 14 × 12 × 8 cm at the right 9th rib with expanded cystic change and marked calcification that appeared to have arisen from the bone. Open biopsy suggested DF. Total excision was performed, and the chest wall was reconstructed. The surgical specimen was a yellowish tumor with multilocular cystic change containing a viscous liquid. The tumor was composed of a proliferation of less-atypical spindle-shaped cells in a collagenous background. The cystic change was observed in the extra-osseous lesion. No β-catenin cytoplasmic/nuclear accumulation was detected, and no β-catenin or GNAS genetic mutations were detected. A final diagnosis of DF was made on the basis of the pathological and radiological findings. The patient was successfully treated with total excision of the tumor with no evidence of recurrence 6 months after surgery.

  10. Metastatic Neuroblastoma in Adult Patient, Presenting as a Super Scan on 68Ga-DOTANOC PET/CT Imaging.

    PubMed

    Malik, Dharmender; Jois, Abhiram; Singh, Harmandeep; Bora, Girdhar S; Basher, Rajender Kumar; Mittal, Bhagwant Rai

    2017-09-01

    We report a case of 23-year-old man who presented with complaints of progressive abdominal distension for the past 3 months along with the loss of appetite and weight and had a large solid cystic mass in the left half of the abdominal cavity revealed on ultrasonography and contrast-enhanced CT of the abdomen. Subsequent biopsy and histopathology revealed it to be neuroblastoma. Ga-DOTANOC PET/CT scan performed to rule out distant metastasis showed intense radiotracer uptake distributed throughout the skeleton, mimicking a super scan.

  11. Acute Scedosporium apiospermum Endobronchial Infection in Cystic Fibrosis.

    PubMed

    Padoan, Rita; Poli, Piercarlo; Colombrita, Domenico; Borghi, Elisa; Timpano, Silviana; Berlucchi, Marco

    2016-06-01

    Fungi are known pathogens in cystic fibrosis patients. A boy with cystic fibrosis boy presented with acute respiratory distress. Bronchoscopy showed airways obstruction by mucus plugs and bronchial casts. Scedosporium apiospermum was identified as the only pathogen. Bronchoalveolar lavage successfully resolved the acute obstruction. Plastic bronchitis is a new clinical picture of acute Scedosporium endobronchial colonization in cystic fibrosis patients.

  12. Diagnosis and surgical management of abdominal cocoon: results from 12 cases.

    PubMed

    Liu, Hai-yan; Wang, Yong-sheng; Yang, Wan-guang; Yin, Sheng-lu; Pei, Hui; Sun, Tong-wen; Wang, Lexin

    2009-01-01

    This study was designed to describe the characteristics, diagnostic and therapeutic methods of abdominal cocoon. Twelve patients with abdominal cocoon were surgically treated. The clinical findings from these patients were analyzed. All patients presented with acute complete intestinal obstruction, and 10 had a previous history of abdominal mass. In nine patients, the whole or part of the small intestines were covered by an ash gray, dense and tough fibrous membrane. The capsule was surgically excised, and the adhesion was released. Partial resection of the small intestines was performed. In the other three patients, the small intestines were only partially covered by a membrane, and there was an extensive adhesion of intestinal tract, forming a large mass which could not be relieved by surgical lysis. Intestinal tube was put in, and fistulation procedures were performed. All patients recovered fully after the surgery. There are four types of surgical findings in abdominal cocoon. The most common type is that the small intestines are fully covered by a thick white membrane, causing intestinal obstruction. Surgical excision of the membrane and the release of adhesion is the treatment of choice.

  13. [Results of nutritional intervention in children and adolescents with cystic fibrosis].

    PubMed

    Gaspar, Maria Cristina A; Chiba, Sônia M; Gomes, Clóvis E T; Juliano, Yara; Novo, Neil F; Ancona-Lopez, Fabio

    2002-01-01

    Few studies have verified longitudinally the evolution of the nutritional status of patients with cystic fibrosis. The objective of this study is to follow the evolution of the nutritional status, body composition and energy consumption, macronutrients and micronutrients ingested by children and adolescents by means of nutritional interventions at the Clinic of Cystic Fibrosis/Pediatric Pneumology of the Department of Pediatrics of Universidade Federal de São Paulo. 18 patients were involved in this study, thirteen males and five females with ages ranging from 0.3 to 18.4 years. We performed three evaluations: evaluation 1 (M1--prenutritional intervention), M2 after 6 months, and M3 after 12 months. In these three instances we verified: the z score for weight/age, weight/height and height/age and the calculation of a 3-day diet record. We verified the body composition (anthropometry) in M1 and M3. The nutritional interventions were hypercaloric, hyperproteic, with adequate amount of ingested macronutrients and micronutrients. We observed an increase in the z score for height/age (M1=-1.07; M2=-0.69; M3=-0.50) and fat-free mass after the nutritional interventions, without improvement in the z score for weight/height and fat mass. We verified an increase in the energy intake during M2 (139%) and M3 (132%) compared to M1 (106%). Remarkable increase in the intake of protein, calcium, iron and vitamin C by the patients was found. The occurrence of anemia was found in 44% (8/18) of the patients. The improvement of the z score in height/age and fat-free mass was probably due to the increase in energy consumption after the nutritional intervention. A significant improvement in the z score for weight/height and fat mass was not found, probably due to a gain in height and fat-free mass.

  14. Cystic fibrosis-related diabetes: a distinct condition.

    PubMed

    Cano Megías, Marta; González Albarrán, Olga

    2015-01-01

    Cystic fibrosis is the most common fatal inherited autosomal recessive disease in Caucasians, affecting approximately one out of every 2,000 births. Survival of patients with cystic fibrosis has significantly improved due to advances in respiratory and nutritional care, and their current average life expectancy is 30-40 years. Development of cystic fibrosis-related diabetes is a comorbidity that increases with age and may reach a prevalence up to 50% in adults. Its development is associated to impaired lung function and nutritional status, and early diagnosis and treatment are therefore essential to improve quality of life and performance status. Insulin therapy for diabetes and other early carbohydrate metabolism disorders may improve lung function and nutritional status of patients with cystic fibrosis. Copyright © 2014 SEEN. Published by Elsevier Espana. All rights reserved.

  15. Genomics of mucoepidermoid and adenoid cystic carcinomas.

    PubMed

    Yan, Kenneth; Yesensky, Jessica; Hasina, Rifat; Agrawal, Nishant

    2018-02-01

    To report on the current state of the literature on the genetics of mucoepidermoid and adenoid cystic carcinomas of the salivary glands with a focus on genomic screens and recently discovered genetic translocations. A PubMed based literature review was performed to query for genetics related basic science and preclinical studies about mucoepidermoid and adenoid cystic carcinomas of the salivary glands. Genetic translocations between CRTC1 and MAML2 in mucoepidermoid carcinoma and between MYB and NFIB in adenoid cystic carcinoma have been recently discovered and have therapeutic implications. Key signaling pathways such as the EGFR pathway in mucoepidermoid carcinoma and the Notch pathway, chromatin regulation, and c-kit mediated epithelial-mesenchymal transitions in adenoid cystic carcinoma have recently been elucidated, pointing to possible therapeutic targets in both cancers.

  16. The use of ultrasound in the diagnosis of abdominal wall hernias.

    PubMed

    Young, J; Gilbert, A I; Graham, M F

    2007-08-01

    The diagnosis of abdominal wall hernias is not always straightforward and may require additional investigative modalities. Real-time ultrasound is accurate, non-invasive, relatively inexpensive, and readily available. The value of ultrasound as an adjunctive tool in the diagnosis of abdominal wall hernias in both pre-operative and post-operative patients was studied. Retrospective analysis of 200 patients treated at the Hernia Institute of Florida was carried out. In these cases, ultrasound had been used to assist with case management. Patients without previous hernia surgery and those with early and late post-herniorrhaphy complaints were studied. Patients with obvious hernias were excluded. Indications for ultrasound examination included patients with abdominal pain without a palpable hernia, a palpable mass of questionable etiology, and patients with inordinate pain or excessive swelling during the early post-operative period. Patients were treated with surgery or conservative therapy depending on the results of the physical examination and ultrasound studies. Cases in which the ultrasound findings influenced the decision-making process by confirming clinical findings or altering the diagnosis and changing the treatment plan are discussed. Of the 200 patients, 144 complained of pain alone and on physical exam no hernia or mass was palpable. Of these 144 patients with pain alone, 21 had a hernia identified on the US examination and were referred for surgery. The 108 that had a negative ultrasound were treated conservatively with rest, heat, and anti-inflammatory drugs, most often with excellent results. Of the 56 remaining patients who had a mass, with or without pain, 22 had hernias identified by means of ultrasound examination. In the other 34, the etiology of the mass was not a hernia. Abdominal wall ultrasound is a valuable tool in the scheme of management of patients in whom the diagnosis of abdominal wall hernia is unclear. Therapeutic decisions can be

  17. Management of the Upper Airway in Cystic Fibrosis

    PubMed Central

    Illing, Elisa A.; Woodworth, Bradford A.

    2015-01-01

    Purpose of Review Upper airway disease engenders significant morbidity for patients with cystic fibrosis and is increasingly recognized as having a much greater role in pulmonary outcomes and quality of life than originally believed. Widespread disparate therapeutic strategies for cystic fibrosis chronic rhinosinusitis underscore the absence of a standardized treatment paradigm. This review outlines the most recent evidence-based trends in the management of upper airway disease in cystic fibrosis. Recent Findings The unified airway theory proposes that the sinuses are a focus of initial bacterial colonization which seeds the lower airway and may play a large role in maintaining lung infections. Mounting evidence suggests more aggressive treatment of the sinuses may confer significant improvement in pulmonary disease and quality of life outcomes in cystic fibrosis patients. However, there is a lack of high-level evidence regarding medical and surgical management of cystic fibrosis chronic rhinosinusitis that makes generalizations difficult. Summary Well designed clinical trials with long-term follow-up concerning medical and surgical interventions for cystic fibrosis sinus disease are required to establish standardized treatment protocols, but increased interest in the sinuses as a bacterial reservoir for pulmonary infections has generated considerable attention. PMID:25250804

  18. Association of visceral fat area with abdominal skeletal muscle distribution in overweight Japanese adults.

    PubMed

    Tanaka, Noriko I; Murakami, Haruka; Ohmori, Yumi; Aiba, Naomi; Morita, Akemi; Watanabe, Shaw; Miyachi, Motohiko

    2016-07-20

    Quantitative evaluation of visceral fat mass and skeletal muscle mass is important for health promotion. Recently, some studies suggested the existence of adipocyte-myocyte negative crosstalk. If so, abdominal skeletal muscles may easily and negatively affected not only by the age but also the visceral fat because age-related reduction in abdominal region is greater compared with limbs. We cross-sectionally examined the existence of quantitative associations between visceral fat area and abdominal skeletal muscle distribution in overweight people. A total of 230 Japanese males and females who aged 40-64 years and whose body mass index (BMI) was 28.0-44.8kg/m 2 participated in this study. The cross-sectional area (CSA) of the visceral fat, subcutaneous fat, and abdominal skeletal muscles, namely, the rectus abdominis, abdominal oblique, erector spinae, and iliopsoas muscles were measured by the computed tomography images. Stepwise regression analyses revealed the existence of sex difference in the relation between visceral fat CSA and other morphological variables. In males, BMI was a positive, and the iliopsoas muscle group CSA was a negative contributor of the visceral fat CSA. In females, both age and BMI were selected as positive contributors. These data suggested that the visceral fat CSA may negatively associated with iliopsoas muscle group CSA in males. In females, the visceral fat CSA was not significantly related to the distribution of the abdominal skeletal muscle groups. Copyright © 2016 Asia Oceania Association for the Study of Obesity. Published by Elsevier Ltd. All rights reserved.

  19. Challenges in pulmonary fibrosis · 3: Cystic lung disease

    PubMed Central

    Cosgrove, Gregory P; Frankel, Stephen K; Brown, Kevin K

    2007-01-01

    Cystic lung disease is a frequently encountered problem caused by a diverse group of diseases. Distinguishing true cystic lung disease from other entities, such as cavitary lung disease and emphysema, is important given the differing prognostic implications. In this paper the features of the cystic lung diseases are reviewed and contrasted with their mimics, and the clinical and radiographic features of both diffuse (pulmonary Langerhans' cell histiocytosis and lymphangioleiomyomatosis) and focal or multifocal cystic lung disease are discussed. PMID:17726170

  20. The ΔF508 Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator Is Associated With Progressive Insulin Resistance and Decreased Functional β-Cell Mass in Mice.

    PubMed

    Fontés, Ghislaine; Ghislain, Julien; Benterki, Isma; Zarrouki, Bader; Trudel, Dominique; Berthiaume, Yves; Poitout, Vincent

    2015-12-01

    Cystic fibrosis (CF) is the result of mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). CF-related diabetes affects 50% of adult CF patients. How CFTR deficiency predisposes to diabetes is unknown. Herein, we examined the impact of the most frequent cftr mutation in humans, deletion of phenylalanine at position 508 (ΔF508), on glucose homeostasis in mice. We compared ΔF508 mutant mice with wild-type (WT) littermates. Twelve-week-old male ΔF508 mutants had lower body weight, improved oral glucose tolerance, and a trend toward higher insulin tolerance. Glucose-induced insulin secretion was slightly diminished in ΔF508 mutant islets, due to reduced insulin content, but ΔF508 mutant islets were not more sensitive to proinflammatory cytokines than WT islets. Hyperglycemic clamps confirmed an increase in insulin sensitivity with normal β-cell function in 12- and 18-week-old ΔF508 mutants. In contrast, 24-week-old ΔF508 mutants exhibited insulin resistance and reduced β-cell function. β-Cell mass was unaffected at 11 weeks of age but was significantly lower in ΔF508 mutants versus controls at 24 weeks. This was not associated with gross pancreatic pathology. We conclude that the ΔF508 CFTR mutation does not lead to an intrinsic β-cell secretory defect but is associated with insulin resistance and a β-cell mass deficit in aging mutants. © 2015 by the American Diabetes Association. Readers may use this article as long as the work is properly cited, the use is educational and not for profit, and the work is not altered.

  1. [Genetic counseling in cystic fibrosis].

    PubMed

    Julia, S; Bieth, E

    2000-08-01

    Genetic counseling is an important part of health care in patients with cystic fibrosis or respiratory diseases associated with the CFTR (cystic fibrosis transmembrane conductance regulator) gene, including certain types of allergic bronchopulmonary aspergilloses or bronchial diseases (diffuse bronchiectasia). The basic goal is to provide patients with information on the transmission of cystic fibrosis and to asses the risk of recurrence. This risk is determined from molecular biology analyses examining the CFTR gene. Genotyping is the only means of screening for the heterozygous state, frequent in the French population (about 1/30). Because of the large number of mutated alleles not covered entirely by the genetic tests, there remains a question of probability expressed as a residual risk of a heterozygous state. A prenatal genotype diagnosis should be proposed to heterozygous couples who have a 25% risk of having a diseased child. Technically, this is almost always possible and the results are highly reliable. Nevertheless, there remains the risks related to sample taking and the ethical issue about which the patients must be informed. Management of these at risk couples who desire a child must be based on a multidisciplinary approach, particularly important when one of the parents has overt cystic fibrosis.

  2. Isolated torsion of fallopian tube in a post-menopausal patient: a case report.

    PubMed

    Ozgun, Mahmut Tuncay; Batukan, Cem; Turkyilmaz, Cagdas; Serin, Ibrahim Serdar

    2007-07-20

    Isolated fallopian tube torsion after menopause is a rare condition. Here we report the second case of isolated fallopian tube torsion in a post-menopausal woman. A 55-year-old post-menopausal woman presented with right lower abdominal pain. Sonography depicted a simple cystic mass adjacent to the right uterine border. Laparatomy revealed torsion of the right fallopian tube together with a paraovarian cyst. Total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed. Histopathological examination revealed a simple paraovarian cyst with severe congestion, necrosis and hemorrhage. Tubal torsion should be considered in the differential diagnosis of acute lower abdominal pain, even in post-menopausal women.

  3. Knee joint loading in knee osteoarthritis: influence of abdominal and thigh fat.

    PubMed

    Messier, Stephen P; Beavers, Daniel P; Loeser, Richard F; Carr, J Jeffery; Khajanchi, Shubham; Legault, Claudine; Nicklas, Barbara J; Hunter, David J; Devita, Paul

    2014-09-01

    Using three separate models that included total body mass, total lean and total fat mass, and abdominal and thigh fat as independent measures, we determined their association with knee joint loads in older overweight and obese adults with knee osteoarthritis (OA). Fat depots were quantified using computed tomography, and total lean and fat mass were determined with dual energy x-ray absorptiometry in 176 adults (age, 66.3 yr; body mass index, 33.5 kg·m) with radiographic knee OA. Knee moments and joint bone-on-bone forces were calculated using gait analysis and musculoskeletal modeling. Higher total body mass was significantly associated (P ≤ 0.0001) with greater knee compressive and shear forces, compressive and shear impulses (P < 0.0001), patellofemoral forces (P < 0.006), and knee extensor moments (P = 0.003). Regression analysis with total lean and total fat mass as independent variables revealed significant positive associations of total fat mass with knee compressive (P = 0.0001), shear (P < 0.001), and patellofemoral forces (P = 0.01) and knee extension moment (P = 0.008). Gastrocnemius and quadriceps forces were positively associated with total fat mass. Total lean mass was associated with knee compressive force (P = 0.002). A regression model that included total thigh and total abdominal fat found that both were significantly associated with knee compressive and shear forces (P ≤ 0.04). Thigh fat was associated with knee abduction (P = 0.03) and knee extension moment (P = 0.02). Thigh fat, consisting predominately of subcutaneous fat, had similar significant associations with knee joint forces as abdominal fat despite its much smaller volume and could be an important therapeutic target for people with knee OA.

  4. Primary cystic echinococcosis in the subcutaneous gluteal region - a case report.

    PubMed

    Steurer, Stefan; Auer, Herbert

    2008-01-01

    Cystic echinococcosis (hydatidosis, hydatid disease) belongs to the group of the most severe parasitic diseases of man and is caused by metacestodes (larvae) of Echinococcus granulosus. Dogs and other canid mammals are the definitive hosts harbouring the adult stages of the tape worm in their intestines. Proglottids containing eggs are passed in the faeces of the definitive host. The eggs are ingested by intermediate hosts (i.e. sheep, cattle, swine etc.), in which the metacestode stage develops. Humans are accidental hosts and are infected by ingestion of eggs upon contact with contaminated water, vegetables, fur (of the dogs) or contaminated hands, the subsequently developing metacestodes are usually localized in the liver, the lungs and many other organs. We report on a case of a 57 year old female patient of Turkish origin suffering from a multicystic gluteal lesion diagnosed by CT- and MRT scan. Surgical intervention revealed a subcutaneous hydatid cyst of 15 cm in diameter. Parasitological-serological examinations revealed a highly positive specific antibody level against E. granulosus arc 5 antigen. The surgically resected cysts could be identified as E. granulosus sheep strain (genotype G1). Additionally to the surgical treatment the patient received albendazole postoperatively. Primary subcutaneous cystic echinococcosis without involving thoracic or abdominal organs is extremely rare and it has not been reported so far. Since soft tissue tumors may resemble hydatid cysts, careful preoperative evaluation is critical for proper handling during surgery to avoid possible anaphylactic reactions or spillage of protoscoleces, particularly when patients are deriving from endemic areas.

  5. Cystic meningiomas.

    PubMed

    Borovich, B; Guilburd, J N; Doron, Y; Soustiel, J F; Zaaroor, M; Braun, J; Gruszkiewicz, J; Feinsod, M

    1988-01-01

    Four cases of cystic meningiomas were found among 194 meningiomas diagnosed by computed tomography (CT) and operated on during a 7 year period, an incidence of 2%. The cysts were in all cases peritumoral. The cyst's wall was the brain itself, and the ependymal ventricular wall was part of their medial boundary. They contained xanthochromic fluid with a high protein content. Three meningiomas were parasagittal and one was adjacent to the pteryon and the external part of the sphenoid ridge. The mural nodules were in 2 cases apparent single nodes although in one it was part of multiple distant and regional growths, in another the tumour was built by the aggregation of 2 nodes, the remaining case was an "en plaque" meningioma. All were definitely attached to the dura. Histological pattern was different in every case. Those parasagittal were: one pure meningotheliomatous, one mixed meningotheliomatous with pseudo psammomatous and lipoblastic sections and one highly vascular angioblastic; the pteryonal case was psammomatous and microcystic. CT diagnosis is difficult because glial, metastatic and other tumours may look cystic and resemble cystic meningiomas. Nevertheless in 3 cases the correct diagnosis was suspected preoperatively because the solid portion of the tumour showed intense and homogeneous contrast enhancement with a sharp edge and was located adjacent to the dura. On the other hand in the remaining case, the parasagittal solid tumour was not readily apparent on CT (the "en plaque", case), and the tentative preoperative diagnosis was of an epidermoid tumour.(ABSTRACT TRUNCATED AT 250 WORDS)

  6. Anabolic agent use in adults with cystic fibrosis.

    PubMed

    Green, Heather D; Barry, Peter J; Jones, Andrew M

    2015-10-01

    The use of non-prescribed anabolic agents amongst non-athletes is increasing with young, adult males with cystic fibrosis (CF) in the highest risk demographic. There is evidence that anabolic agents increase weight and muscle mass in adults with a variety of catabolic conditions but there is no evidence for their use in hormone sufficient adults with CF. We report a case of anabolic agent use in a male adult with CF and review the clinical features of anabolic agent use with a focus on adults with CF. Copyright © 2015 Elsevier Ltd. All rights reserved.

  7. Genomics of mucoepidermoid and adenoid cystic carcinomas

    PubMed Central

    Yan, Kenneth; Yesensky, Jessica; Hasina, Rifat

    2018-01-01

    Objective To report on the current state of the literature on the genetics of mucoepidermoid and adenoid cystic carcinomas of the salivary glands with a focus on genomic screens and recently discovered genetic translocations. Methods A PubMed based literature review was performed to query for genetics related basic science and preclinical studies about mucoepidermoid and adenoid cystic carcinomas of the salivary glands. Results and conclusions Genetic translocations between CRTC1 and MAML2 in mucoepidermoid carcinoma and between MYB and NFIB in adenoid cystic carcinoma have been recently discovered and have therapeutic implications. Key signaling pathways such as the EGFR pathway in mucoepidermoid carcinoma and the Notch pathway, chromatin regulation, and c‐kit mediated epithelial‐mesenchymal transitions in adenoid cystic carcinoma have recently been elucidated, pointing to possible therapeutic targets in both cancers. PMID:29492469

  8. Detection of cystic structures using pulsed ultrasonically induced resonant cavitation

    NASA Technical Reports Server (NTRS)

    Bar-Cohen, Yoseph (Inventor); Kovach, John S. (Inventor)

    2002-01-01

    Apparatus and method for early detection of cystic structures indicative of ovarian and breast cancers uses ultrasonic wave energy at a unique resonance frequency for inducing cavitation in cystic fluid characteristic of cystic structures in the ovaries associated with ovarian cancer, and in cystic structures in the breast associated with breast cancer. Induced cavitation bubbles in the cystic fluid implode, creating implosion waves which are detected by ultrasonic receiving transducers attached to the abdomen of the patient. Triangulation of the ultrasonic receiving transducers enables the received signals to be processed and analyzed to identify the location and structure of the cyst.

  9. [News in cystic fibrosis].

    PubMed

    Delaisi, B

    2013-08-01

    The improvement over the last two decades in the treatment of cystic fibrosis led to an increase in life expectancy approaching 40 years at birth. Logically, the population of adult patients has been increasing and is currently 50% of patients followed in France. These therapeutic advances have justified the establishment in 2003 of a generalized neonatal screening for cystic fibrosis. The latest data of this screening show an incidence of CF of 1/5359 live births, far below the incidence of 1/2500 which was widely accepted twenty years ago. The performance of this screening is currently based on the dosage of trypsin immuno reactive, followed in case of exceeding the threshold of a search of the 30 most common mutations, can detect around 96% of 150 to 200 CF cases every year. Therefore, the possibility of a false negative of the screening cannot be excluded and evocative symptoms of cystic fibrosis, even for children born after 2003, will lead to prescribe a sweat test. While treatments available so far goal consequences of cystic fibrosis, a new therapeutic class to correct the functional defect of the mutated protein, called CFTR modulators, is emerging. Ivacaftor, leader of this new class, belonging to the category of "CFTR potentiator" got its access on the market in September 2012 for patients carrying the G551D mutation. New other molecules, named "CFTR correctors" which can have synergistic effect with ivacaftor and concern patients carrying the most common mutation--DF 508--are under development. Copyright © 2013. Published by Elsevier Masson SAS.

  10. Leisure-time physical activity and intra-abdominal fat in young adulthood: A monozygotic co-twin control study.

    PubMed

    Rottensteiner, Mirva; Leskinen, Tuija; Järvelä-Reijonen, Elina; Väisänen, Karoliina; Aaltonen, Sari; Kaprio, Jaakko; Kujala, Urho M

    2016-05-01

    To investigate differences in abdominal fat compartments between young adult monozygotic twin pairs discordant for leisure-time physical activity. Ten young adult male monozygotic twin pairs (age range 32-36 years) discordant for leisure-time physical activity during the past 3 years were systematically selected from a population-based Finnish twin cohort. Magnetic resonance image at the level of the L2-L3 intervertebral disc was used to predict intra-abdominal and subcutaneous abdominal fat masses. Dietary intake was assessed with a 4-day food diary. Inactive twins had 31% more intra-abdominal fat than their active co-twins (mean difference 0.52 kg, 95% CI 0.12 to 0.91, P = 0.016), whereas the difference in subcutaneous abdominal fat was only 13% (P = 0.21) and 3% in body mass index (P = 0.28). Intraperitoneal fat mass was 41% higher among inactive twins compared to their active co-twins (mean difference 0.41 kg, 95% CI 0.11 to 0.70, P = 0.012). Dietary intake did not differ between co-twins. A lower level of physical activity is related to greater accumulation of intra-abdominal fat among healthy adult males in their mid-30s. The findings highlight the importance of leisure-time physical activity independent of genes and diet in the prevention of intra-abdominal fat accumulation from early adulthood onward. © 2016 The Obesity Society.

  11. Unusual growth rate during cystic echinococcosis.

    PubMed

    Valour, Florent; Khenifer, Safia; Della-Schiava, Nellie; Cotte, Eddy; Guibert, Benoit; Wallon, Martine; Durupt, Stéphane; Durieu, Isabelle

    2014-04-01

    Cystic echinococcosis is a world wild zoonosis caused by Echinococcus granulosus, leading to hepatic and lung cysts with a usually slight growth rate. We report the case of an 82year-old Algerian woman with hepatic and lung cystic echinococcosis with a 10-fold size increase in 6months. Copyright © 2013. Published by Elsevier Ireland Ltd.

  12. Effect of Gender on the Total Abdominal Fat, Intra-Abdominal Adipose Tissue and Abdominal Sub-Cutaneous Adipose Tissue among Indian Hypertensive Patients.

    PubMed

    Sahoo, Jaya Prakash; Kumari, Savita; Jain, Sanjay

    2016-04-01

    Abdominal obesity is a better marker of adverse metabolic profile than generalized obesity in hypertensive subjects. Further, gender has effect on adiposity and its distribution. Effect of gender on obesity and the distribution of fat in different sub-compartments of abdomen among Indian hypertensive subjects. This observational study included 278 adult subjects (Males-149 & Females-129) with essential hypertension from a tertiary care centre in north India over one year. A detailed history taking and physical examination including anthropometry were performed in all patients. Total Abdominal Fat (TAF) and abdominal adipose tissue sub-compartments like Intra-Abdominal Adipose Tissue (IAAT) and Sub-Cutaneous Adipose Tissue (SCAT) were measured using the predictive equations developed for Asian Indians. Female hypertensive subjects had higher Body Mass Index (BMI) with more overweight (BMI ≥ 23kg/m(2)), and obesity (BMI≥ 25 kg/m(2)). Additionally, they had higher prevalence of central obesity based on both Waist Circumference (WC) criteria (WC≥ 90 cm in males and WC≥ 80 cm in females) and TAF criteria {≥245.6 cm(2) (males) and ≥203.46 cm(2) (females)} than male patients. But there was no difference in the prevalence of central obesity based on Waist Hip Ratio (WHR) criteria (WHR ≥0.90 in males and WHR ≥ 0.85 in females) between two genders. High TAF & IAAT were present in more females although there was no difference in the distribution of high SCAT between two genders. Female hypertensive subjects were more obese with higher abnormal TAF & IAAT compared to male patients. However, there was no difference in the distribution of high SCAT among them.

  13. Management of intra-abdominal hypertension and abdominal compartment syndrome: a review

    PubMed Central

    2014-01-01

    Patients in the intensive care unit (ICU) are at risk of developing of intra abdominal hypertension (IAH) and abdominal compartment syndrome (ACS). Aim: This review seeks to define IAH and ACS, identify the aetiology and presentation of IAH and ACS, identify IAP measurement techniques, identify current management and discuss the implications of IAH and ACS for nursing practice. A search of the electronic databases was supervised by a health librarian. The electronic data bases Cumulative Index of Nursing and Allied Health Literature (CINAHL); Medline, EMBASE, and the World Wide Web was undertaken from 1996- January 2011 using MeSH and key words which included but not limited to: abdominal compartment syndrome, intra -abdominal hypertension, intra-abdominal pressure in adult populations met the search criteria and were reviewed by three authors using a critical appraisal tool. Data derived from the retrieved material are discussed under the following themes: (1) etiology of intra-abdominal hypertension; (2) strategies for measuring intra-abdominal pressure (3) the manifestation of abdominal compartment syndrome; and (4) the importance of nursing assessment, observation and interventions. Intra-abdominal pressure (IAP) and abdominal compartment syndrome (ACS) have the potential to alter organ perfusion and compromise organ function. PMID:24499574

  14. Insulin and oral agents for managing cystic fibrosis-related diabetes.

    PubMed

    Onady, Gary M; Stolfi, Adrienne

    2016-04-18

    The Cystic Fibrosis Foundation recommends both short-term and long-acting insulin therapy when cystic fibrosis-related diabetes has been diagnosed. Diagnosis is based on: an elevated fasting blood glucose level greater than 6.94 mmol/liter (125 mg/deciliter); or oral glucose tolerance tests greater than 11.11 mmol/liter (200 mg/deciliter) at two hours; or symptomatic diabetes for random glucose levels greater than 11.11 mmol/liter (200 mg/deciliter); or glycated hemoglobin levels of at least 6.5%. To establish the effectiveness of insulin and oral agents for managing diabetes in people with cystic fibrosis in relation to blood sugar levels, lung function and weight management. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also handsearched abstracts from pulmonary symposia and the North American Cystic Fibrosis Conferences.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 18 February 2016. Randomized controlled trials comparing all methods of diabetes therapy in people with diagnosed cystic fibrosis-related diabetes. Two authors independently extracted data and assessed the risk of bias in the included studies. The searches identified 22 trials (34 references). Four trials (200 participants) are included: one short-term single-center trial (n = 7) comparing insulin with oral repaglinide and no medication in people with cystic fibrosis-related diabetes and normal fasting glucose; one long-term multicenter trial (n = 100, 74 of whom had cystic fibrosis-related diabetes) comparing insulin with oral repaglinide and placebo; one long-term multicenter trial (n = 73) comparing insulin with oral repaglinide; and one 12-week single-center trial (n = 20) comparing the long-acting insulin glargine to short-term neutral protamine Hagedorn

  15. Prevalence of Mycobacterium lentiflavum in cystic fibrosis patients, France.

    PubMed

    Phelippeau, Michael; Dubus, Jean-Christophe; Reynaud-Gaubert, Martine; Gomez, Carine; Stremler le Bel, Nathalie; Bedotto, Marielle; Prudent, Elsa; Drancourt, Michel

    2015-10-26

    Mycobacterium lentiflavum is rarely isolated in respiratory tract samples from cystic fibrosis patients. We herein describe an unusually high prevalence of M. lentiflavum in such patients. M. lentiflavum, isolated from the respiratory tract of cystic fibrosis patients, was identified using both rpoB partial sequencing and detected directly in the sputum by using real-time PCR targeting the smpB gene. M. lentiflavum emerged as the third most prevalent nontuberculous mycobacterial species isolated in cystic fibrosis patients in Marseille, France. Six such patients were all male, and two of them may have fulfilled the American Thoracic Society clinical and microbiological criteria for M. lentiflavum potential lung infection. M. lentiflavum was the third most common mycobacteria isolated in cystic fibrosis patients, particularly in six male patients. M. lentiflavum outbreaks are emerging particularly in cystic fibrosis patients.

  16. Laparoscopic excision of an epidermoid cyst arising from the deep abdominal wall.

    PubMed

    Ishikawa, Hajime; Nakai, Takuya; Ueda, Kazuki; Haji, Seiji; Takeyama, Yoshifumi; Ohyanagi, Harumasa

    2009-10-01

    Epidermoid cysts are the most common type of cutaneous cyst. However, their occurrence in the deep abdominal wall has not yet been reported. Here, we present the case of a 60-year-old woman who developed an epidermoid cyst in the deep abdominal wall, which was resected laparoscopically. The patient presented with right upper quadrant abdominal pain on admission to our hospital. Computed tomography revealed cholecystolithiasis and an incidentally identified well-defined hypoattenuating mass (62 x 47 x 65 mm) in the deep abdominal wall on the left side of the navel. We performed laparoscopic complete resection of the abdominal wall tumor followed by cholecystectomy. The excised specimen was a cyst covered with a smooth thin membrane and contained sludge. Histopathologic examination revealed an epidermoid cyst. This is a very rare case with no previous reports on a similar type of epidermoid cyst.

  17. Cystic fibrosis-related diabetes compared with type 1 and type 2 diabetes in adults.

    PubMed

    Konrad, Katja; Scheuing, Nicole; Badenhoop, Klaus; Borkenstein, Martin H; Gohlke, Bettina; Schöfl, Christof; Seufert, Jochen; Thon, Angelika; Holl, Reinhard W

    2013-10-01

    With increasing life expectancy of patients with cystic fibrosis (CF), secondary diabetes becomes more prevalent. It appears to be the most common co-morbidity in persons with cystic fibrosis. Therefore, the objective of our study was to describe characteristics of cystic fibrosis-related diabetes compared with type 1 and 2 diabetes (T1DM/T2DM) in adults. Data from 218 436 patients >18 years with cystic fibrosis (n = 401), T1DM (n = 32,409) or T2DM (n = 185 626) in the multicenter Diabetes-Patienten-Verlaufsdokumentation or prospective documentation of diabetes patients registry were analysed. Diabetes onset [median (interquartile range)] in cystic fibrosis [18.70 (15.50-25.30) years] was between T1DM [16.40 (10.50-31.80) years] and T2DM [58.50 (48.80-68.00) years], with female preponderance. Body mass index (BMI) and glycosylated haemoglobin (HbA1c ) were lowest (19.6 [18.1-21.5] kg/m(2) )/50 mmol/mol (6.73%) versus T1DM (24.4 [22.1-27.4])/62 mmol/mol (7.83%) vs. T2DM (29.6 [26.1-33.9])/54 mmol/mol (7.06%); all p < 0.01. A total of 78.6% of cystic fibrosis patients with diabetes received insulin. Insulin dose (0.74 IE/kg bodyweight) was not significantly different from T1DM (0.73) and T2DM (0.76). Frequency of vascular complications, adjusted for confounding effects, across the groups was different: Hypertension (CFRD 16.1% vs. T1DM 24.0% vs. T2DM 32.2%; all p < 0.01), retinopathy (CFRD 10.7% vs. T1DM 10.4% vs. T2DM 10.5%, not significant), nephropathy (CFRD 25.2% vs. T1DM 17.2% vs. T2DM 24.7%; only T1DM/T2DM; p < 0.01). CFRD is a uniquely complex entity with clear differences from T1DM and T2DM in adults. Copyright © 2013 John Wiley & Sons, Ltd.

  18. Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.

    PubMed

    Lee, T; Southern, K W

    2007-04-18

    Cystic fibrosis is caused by a defective gene encoding a protein called the cystic fibrosis transmembrane conductance regulator (CFTR), and is characterised by chronic lung infection resulting in inflammation and progressive lung damage that results in a reduced life expectancy. To determine whether topical CFTR gene replacement therapy to the lungs in people with cystic fibrosis is associated with improvements in clinical outcomes, and to assess any adverse effects. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings. Date of most recent search: February 2007 Randomised controlled trials comparing topical CFTR gene delivery to the lung, using either viral or non-viral delivery systems, with placebo or an alternative delivery system in people with confirmed cystic fibrosis. The authors independently extracted data and assessed study quality. Authors of included studies were contacted and asked for any available additional data. Meta-analysis was limited due to differing study designs. Three randomised controlled trials met the inclusion criteria for this review, involving a total of 155 participants. Thirteen studies were excluded. The included studies differed in terms of CFTR gene replacement agent and study design, which limited the meta-analysis. Although the first Moss study reported a significant improvement in respiratory function (FEV(1)) 30 days after participants had received their first dose of gene therapy agent, this finding was not confirmed in their larger second study or in our meta-analysis.In participants who received the CFTR gene transfer agents in the Alton study, "influenza-like" symptoms were found (relative risk 7.00 (95% confidence interval (CI) 1.10 to 44.61)). There were no other significant increases in adverse events in any of the studies. Alton

  19. Abdominal Wall Endometriosis Eleven Years After Cesarean Section: Case Report

    PubMed

    Djaković, Ivka; Vuković, Ante; Bolanča, Ivan; Soljačić Vraneš, Hrvojka; Kuna, Krunoslav

    2017-03-01

    Endometriosis is a common chronic disease characterized by growth of the endometrial gland and stroma outside the uterus. Symptoms affect physical, mental and social well-being. Extrapelvic location of endometriosis is very rare. Abdominal wall endometriosis occurs in 0.03%-2% of women with a previous cesarean section or other abdominopelvic operation. The leading symptoms are abdominal nodular mass, pain and cyclic symptomatology. The number of cesarean sections is increasing and so is the incidence of abdominal wall endometriosis as a potential complication of the procedure. There are cases of malignant transformation of abdominal wall endometriosis. Therefore, it is important to recognize this condition and treat it surgically. We report a case of a 37-year-old woman with abdominal wall endometriosis 11 years after cesarean section. She had low abdominal pain related to menstrual cycle, which intensified at the end of menstrual bleeding. A nodule painful to palpation was found in the medial part of previous Pfannenstiel incision. Ultrasound guided biopsy was performed and the diagnosis of endometriosis confirmed. Surgery is the treatment of choice for abdominal wall endometriosis. Excision with histologically proven free surgical margins of 1 cm is mandatory to prevent recurrence. A wide spectrum of mimicking conditions is the main reason for late diagnosis and treatment of abdominal wall endometriosis. In our case, the symptoms lasted for eight years and had intensified in the last six months prior to surgery.

  20. Diagnosis and treatment of endocrine comorbidities in patients with cystic fibrosis.

    PubMed

    Siwamogsatham, Oranan; Alvarez, Jessica A; Tangpricha, Vin

    2014-10-01

    The aim of this review is to provide an update on various relevant endocrine aspects of care in adolescents and adults with cystic fibrosis. As life expectancy in cystic fibrosis has continuously improved, endocrine complications have become more apparent. The common endocrine complications include cystic fibrosis related diabetes, cystic fibrosis related bone disease, vitamin D deficiency and poor growth and pubertal development. Thyroid and adrenal disorders have also been reported, although the prevalence appears to be less common. Endocrine diseases are an increasingly recognized complication that has a significant impact on the overall health of individuals with cystic fibrosis. This review summarizes the updated screening and management of endocrine diseases in the cystic fibrosis population.

  1. About Cystic Fibrosis

    MedlinePlus

    ... Testing for Cystic Fibrosis CFTR-Related Metabolic Syndrome (CRMS) How Babies Are Screened in IRT-Only vs. ... Guidelines Infant Care Clinical Care Guidelines Management of CRMS in First 2 Years and Beyond Clinical Care ...

  2. Percutaneous Image-Guided Aspiration and Sclerosis of Adventitial Cystic Disease of the Femoral Vein

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Johnson, Jason M.; Kiankhooy, Armin; Bertges, Daniel J.

    2009-07-15

    Adventitial cystic disease (ACD), also known as cystic mucoid or myxomatous degeneration, is a rare vascular disease mainly seen in arteries. Seventeen cases have been reported in the world literature. We report the first known case of ACD successfully treated with percutaneous image-guided ethanol sclerosis. Computed tomography showed a cystic mass adherent to the wall of the common femoral vein. An ultrasound examination revealed a deep venous thrombosis of the leg, secondary to extrinsic compression of the common femoral vein. Three years prior to our procedure, the cyst was aspirated, which partially relieved the patient's symptoms. Over the following 3more » years the patient's symptoms worsened and a 10-cm discrepancy in thigh size developed, in addition to the deep venous thrombosis associated with lower-extremity edema. Using ultrasound guidance and fluoroscopic control, the cyst was drained and then sclerosed with absolute ethanol. The patient's symptoms and leg swelling resolved completely within several weeks. Follow-up physical examination and duplex ultrasound 6 months following sclerosis demonstrated resolution of the symptoms and elimination of the extrinsic compression effect of the ACD on the common femoral vein.« less

  3. Pancreatic enzyme replacement therapy for people with cystic fibrosis.

    PubMed

    Somaraju, Usha Rani; Solis-Moya, Arturo

    2014-10-13

    adults with cystic fibrosis were included in the review. The number of participants in each trial varied between 14 and 129 with a total of 426 participants included in the review. All the included trials were for a duration of four weeks. The included trials had mostly an unclear risk of bias from the randomisation process as the details of this were not given; they also mostly had a high risk of attrition bias and reporting bias.We could not combine data from all the trials as they compared different formulations. Findings from individual studies provided insufficient evidence to determine the size and precision of the effects of different formulations. Ten studies reported information on the review's primary outcome (nutritional status); however, we were only able to combine data from two small cross-over studies (n = 41). The estimated gain in body weight was imprecise, 0.32 kg (95% confidence interval -0.03 to 0.67, P = 0.07). Combined data from the same studies gave statistically significant results favouring enteric-coated microspheres over enteric-coated tablets for our secondary outcomes stool frequency, abdominal pain and fecal fat excretion. Data from another single small cross-over study also favoured enteric-coated microspheres over non-enteric-coated tablets with adjuvant cimetidine in terms of stool frequency. There is limited evidence of benefit from enteric-coated microspheres when compared to non-enteric coated pancreatic enzyme preparations up to one month. In the only comparison where we could combine any data, the fact that these were cross-over studies is likely to underestimate the level of inconsistency between the results of the studies due to over-inflation of confidence intervals from the individual studies.There is no evidence on the long-term effectiveness and risks associated with pancreatic enzyme replacement therapy. There is also no evidence on the relative dosages of enzymes needed for people with different levels of severity of

  4. Evaluation of Patients with Cystic Echinococcosis.

    PubMed

    Türkoğlu, Emine; Demirtürk, Neşe; Tünay, Havva; Akıcı, Murat; Öz, Gürhan; Baskin Embleton, Didem

    2017-03-01

    Cystic echinococcosis (CE) is a globally prevalent zoonotic disease. Demographic, clinical, laboratory, and follow-up data of patients between May 2009 and 2015 were retrospectively analyzed by screening data from a hospital automation system. A total of 238 (females, n=139 and males, n=99) patients with a mean age of 40.6±20.58 years were included. Less than half (40.8%) of the patients were living in the countryside. Hepatic involvement of CE was most frequently (72.2%) seen. A majority (75.6%) of the patients were symptomatic, but abdominal pain was the most frequently seen symptom. For diagnosis, in all patients, imaging modalities were used, while in 66% of the patients, serological methods were also employed. The patients received both medical and surgical treatments (78.5%, n=187), only surgical treatment (10.5%, n=25), or only medical treatment (8.8%, n=21). Surgical treatment was performed for patients with hepatic (n=139/176, 80.6%), pulmonary (n=78/94, 82.9%), splenic (n=7/9; 77.7%), and mesenteric (n=6/7, 85.1%) cysts, and patients cases with brain, bone, muscle, omentum, bladder, and adrenal cysts had undergone surgical intervention. Publication of regional data is important in terms of epidemiological considerations and may aid in the formulation of standard treatment approaches.

  5. Air trapping and airflow obstruction in newborn cystic fibrosis piglets.

    PubMed

    Adam, Ryan J; Michalski, Andrew S; Bauer, Christian; Abou Alaiwa, Mahmoud H; Gross, Thomas J; Awadalla, Maged S; Bouzek, Drake C; Gansemer, Nicholas D; Taft, Peter J; Hoegger, Mark J; Diwakar, Amit; Ochs, Matthias; Reinhardt, Joseph M; Hoffman, Eric A; Beichel, Reinhard R; Meyerholz, David K; Stoltz, David A

    2013-12-15

    Air trapping and airflow obstruction are being increasingly identified in infants with cystic fibrosis. These findings are commonly attributed to airway infection, inflammation, and mucus buildup. To learn if air trapping and airflow obstruction are present before the onset of airway infection and inflammation in cystic fibrosis. On the day they are born, piglets with cystic fibrosis lack airway infection and inflammation. Therefore, we used newborn wild-type piglets and piglets with cystic fibrosis to assess air trapping, airway size, and lung volume with inspiratory and expiratory X-ray computed tomography scans. Micro-computed tomography scanning was used to assess more distal airway sizes. Airway resistance was determined with a mechanical ventilator. Mean linear intercept and alveolar surface area were determined using stereologic methods. On the day they were born, piglets with cystic fibrosis exhibited air trapping more frequently than wild-type piglets (75% vs. 12.5%, respectively). Moreover, newborn piglets with cystic fibrosis had increased airway resistance that was accompanied by luminal size reduction in the trachea, mainstem bronchi, and proximal airways. In contrast, mean linear intercept length, alveolar surface area, and lung volume were similar between both genotypes. The presence of air trapping, airflow obstruction, and airway size reduction in newborn piglets with cystic fibrosis before the onset of airway infection, inflammation, and mucus accumulation indicates that cystic fibrosis impacts airway development. Our findings suggest that early airflow obstruction and air trapping in infants with cystic fibrosis might, in part, be caused by congenital airway abnormalities.

  6. Abdominal epilepsy as an unusual cause of abdominal pain: a case report.

    PubMed

    Yunus, Yilmaz; Sefer, Ustebay; Dondu, Ulker Ustebay; Ismail, Ozanli; Yusuf, Ehi

    2016-09-01

    Abdominal pain, in etiology sometimes difficult to be defined, is a frequent complaint in childhood. Abdominal epilepsy is a rare cause of abdominal pain. In this article, we report on 5 year old girl patient with abdominal epilepsy. Some investigations (stool investigation, routine blood tests, ultrasonography (USG), electrocardiogram (ECHO) and electrocardiograpy (ECG), holter for 24hr.) were done to understand the origin of these complaints; but no abnormalities were found. Finally an EEG was done during an episode of abdominal pain and it was shown that there were generalized spikes especially precipitated by hyperventilation. The patient did well on valproic acid therapy and EEG was normal 1 month after beginning of the treatment. The cause of chronic recurrent paroxymal abdominal pain is difficult for the clinicians to diagnose in childhood. A lot of disease may lead to paroxysmal gastrointestinal symptoms like familial mediterranean fever and porfiria. Abdominal epilepsy is one of the rare but easily treatable cause of abdominal pain. In conclusion, abdominal epilepsy should be suspected in children with recurrent abdominal pain.

  7. Bosniak classification system: a prospective comparison of CT, contrast-enhanced US, and MR for categorizing complex renal cystic masses.

    PubMed

    Graumann, Ole; Osther, Susanne Sloth; Karstoft, Jens; Hørlyck, Arne; Osther, Palle Jörn Sloth

    2016-11-01

    Background The Bosniak classification was originally based on computed tomographic (CT) findings. Magnetic resonance (MR) and contrast-enhanced ultrasonography (CEUS) imaging may demonstrate findings that are not depicted at CT, and there may not always be a clear correlation between the findings at MR and CEUS imaging and those at CT. Purpose To compare diagnostic accuracy of MR, CEUS, and CT when categorizing complex renal cystic masses according to the Bosniak classification. Material and Methods From February 2011 to June 2012, 46 complex renal cysts were prospectively evaluated by three readers. Each mass was categorized according to the Bosniak classification and CT was chosen as gold standard. Kappa was calculated for diagnostic accuracy and data was compared with pathological results. Results CT images found 27 BII, six BIIF, seven BIII, and six BIV. Forty-three cysts could be characterized by CEUS, 79% were in agreement with CT (κ = 0.86). Five BII lesions were upgraded to BIIF and four lesions were categorized lower with CEUS. Forty-one lesions were examined with MR; 78% were in agreement with CT (κ = 0.91). Three BII lesions were upgraded to BIIF and six lesions were categorized one category lower. Pathologic correlation in six lesions revealed four malignant and two benign lesions. Conclusion CEUS and MR both up- and downgraded renal cysts compared to CT, and until these non-radiation modalities have been refined and adjusted, CT should remain the gold standard of the Bosniak classification.

  8. L206W mutation of the cystic fibrosis gene, relatively frequent in French Canadians, is associated with atypical presentations of cystic fibrosis

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Rozen, R.; Ferreira-Rajabi, L.; Robb, L.

    Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Over 400 mutations have been reported at this locus. Although severe forms of cystic fibrosis are usually associated with pancreatic insufficiency, pulmonary dysfunction, and elevated sweat chloride, there is a wide range of phenotypes, including congenital absence of the vas deferens, observed with some of the milder mutations. The L206W mutation, which was first identified in patients from South France, is relatively frequent in French Canadians from Quebec. In this report, we document the atypical form of cystic fibrosis associated with this mutation in amore » cohort of 7 French Canadian probands. 20 refs.« less

  9. A universal array-based multiplexed test for cystic fibrosis carrier screening.

    PubMed

    Amos, Jean A; Bridge-Cook, Philippa; Ponek, Victor; Jarvis, Michael R

    2006-01-01

    Cystic fibrosis is a multisystem autosomal recessive disorder with high carrier frequencies in caucasians and significant, but lower, carrier frequencies in other ethnicities. Based on technology that allows high detection of mutations in caucasians and significant detection in other ethnic groups, the American College of Medical Genetics (ACMG) and American College of Obstetricians and Gynecologists (ACOG) have recommended pan-ethnic cystic fibrosis carrier screening for all reproductive couples. This paper discusses carrier screening using the Tag-It multiplex mutation platform and the Cystic Fibrosis Mutation Detection Kit. The Tag-It cystic fibrosis assay is a multiplexed genotyping assay that detects a panel of 40 cystic fibrosis transmembrane conductance regulator mutations including the 23 mutations recommended by the ACMG and ACOG for population screening. A total of 16 additional mutations detected by the Tag-It cystic fibrosis assay may also be common. The assay method is described in detail, and its performance in a genetics reference laboratory performing high-volume cystic fibrosis carrier screening is assessed.

  10. Arnebia euchroma ointment can reduce abdominal fat thickness and abdominal circumference of overweight women: A randomized controlled study.

    PubMed

    Siavash, Mansour; Naseri, Mohsen; Rahimi, Mojgan

    2016-01-01

    Obesity is a worldwide health problem which is associated with a lot of complications. One of these comorbidities is the metabolic syndrome that is in correlation with abdominal fat thickness and waist circumference. Various methods were used to reduce abdominal fat thickness such as liposuction. A noninvasive method is the topical agent. In this study, we investigated the effectiveness of Arnebia euchroma (AE) ointment on the abdominal fat thickness. This study was a double-blind clinical trial which was done at the endocrinology clinic in Khorshid Hospital, Isfahan, Iran, in 2014. After explaining the procedure and obtaining informed consent, the candidates were randomly divided into the case and control groups. The participants of the case and control groups applied AE ointment or placebo for 6 weeks on their abdominal area. Body mass index, waist and buttock circumference, and abdominal fat thickness were measured in both case and control groups at their first visit and then at the next 2, 4, and 6 weeks. We used t -test for comparing parametric variables between groups, paired t -test for changes from baseline to final, and repeated measure ANOVA for changes at different steps. Sixty female candidates participated in this study (thirty in each group). Ten patients left the study and fifty participants finished the trial. At the end of the study, participants had a significant weight loss (2.96 ± 1.6 kg, P < 0.001) that was slightly more in the case group (3.15 ± 1.5 kg vs. 2.75 ± 1.7, P = 0.375). Abdominal circumference also decreased significantly in the participants (11.3 ± 6.7 cm, P < 0.001), but the changes were more significant in the case group (13.9 vs. 6.5 cm, P = 0.004). Similarly, abdominal fat thickness decreased significantly in the participants (2.3 ± 1.1 cm, P < 0.001), although changes were not significantly different between two groups (2.53 vs. 2.04 cm, P = 0.139). Topical AE ointment can reduce the abdominal fat thickness as well as the

  11. KNEE-JOINT LOADING IN KNEE OSTEOARTHRITIS: INFLUENCE OF ABDOMINAL AND THIGH FAT

    PubMed Central

    Messier, Stephen P.; Beavers, Daniel P.; Loeser, Richard F.; Carr, J. Jeffery; Khajanchi, Shubham; Legault, Claudine; Nicklas, Barbara J.; Hunter, David J.; DeVita, Paul

    2014-01-01

    Purpose Using three separate models that included total body mass, total lean and total fat mass, and abdominal and thigh fat as independent measures, we determined their association with knee-joint loads in older overweight and obese adults with knee osteoarthritis (OA). Methods Fat depots were quantified using computed tomography and total lean and fat mass determined with dual energy x-ray absorptiometry in 176 adults (age = 66.3 yr., BMI = 33.5 kg·m−2) with radiographic knee OA. Knee moments and joint bone-on-bone forces were calculated using gait analysis and musculoskeletal modeling. Results Higher total body mass was significantly associated (p ≤ 0.0001) with greater knee compressive and shear forces, compressive and shear impulses (p < 0.0001), patellofemoral forces (p< 0.006), and knee extensor moments (p = 0.003). Regression analysis with total lean and total fat mass as independent variables revealed significant positive associations of total fat mass with knee compressive (p = 0.0001), shear (p < 0.001), and patellofemoral forces (p = 0.01) and knee extension moment (p = 0.008). Gastrocnemius and quadriceps forces were positively associated with total fat mass. Total lean mass was associated with knee compressive force (p = 0.002). A regression model that included total thigh and total abdominal fat found both were significantly associated with knee compressive and shear forces (p ≤ 0.04). Thigh fat was associated with the knee abduction (p = 0.03) and knee extension moment (p = 0.02). Conclusions Thigh fat, consisting predominately of subcutaneous fat, had similar significant associations with knee joint forces as abdominal fat despite its much smaller volume and could be an important therapeutic target for people with knee OA. PMID:25133996

  12. Impaired rib bone mass and quality in end-stage cystic fibrosis patients.

    PubMed

    Mailhot, Geneviève; Dion, Natalie; Farlay, Delphine; Rizzo, Sébastien; Bureau, Nathalie J; Jomphe, Valérie; Sankhe, Safiétou; Boivin, Georges; Lands, Larry C; Ferraro, Pasquale; Ste-Marie, Louis-Georges

    2017-05-01

    Advancements in research and clinical care have considerably extended the life expectancy of cystic fibrosis (CF) patients. However, with this extended survival come comorbidities. One of the leading co-morbidities is CF-related bone disease (CFBD), which progresses with disease severity and places patients at high risk for fractures, particularly of the ribs and vertebrae. Evidence that CF patients with vertebral fractures had higher bone mineral density (BMD) than the nonfracture group led us to postulate that bone quality is impaired in these patients. We therefore examined rib specimens resected at the time of lung transplant in CF patients to measure parameters of bone quantity and quality. In this exploratory study, we analysed 19 end-stage CF and 13 control rib specimens resected from otherwise healthy lung donors. BMD, bone microarchitecture, static parameters of bone formation and resorption and microcrack density of rib specimens were quantified by imaging, histomorphometric and histological methods. Variables reflecting the mineralization of ribs were assessed by digitized microradiography. The degree of bone mineralization (g/cm 3 ) and the heterogeneity index of the mineralization (g/cm 3 ) were calculated for trabecular and cortical bone. Compared to controls, CF ribs exhibited lower areal and trabecular volumetric BMD, decreased trabecular thickness and osteoid parameters, and increased microcrack density, that was particularly pronounced in specimens from patients with CF-related diabetes. Static parameters of bone resorption were similar in both groups. Degree of mineralization of total bone, but not heterogeneity index, was increased in CF specimens. The combination of reduced bone mass, altered microarchitecture, imbalanced bone remodeling (maintained bone resorption but decreased formation), increased microdamage and a small increase of the degree of mineralization, may lead to decreased bone strength, which, when coupled with chronic coughing

  13. Pyometra in childhood.

    PubMed

    Barry, Carole

    2015-05-01

    Pyometra, an accumulation of pus in the uterine cavity, occurs rarely in children but should be considered in the differential diagnosis of an intra-abdominal or pelvic abscess. A 10-month-old infant presented with an increasing abdominal mass. She had previous respiratory and diarrheal illnesses. She was underweight, febrile, and tachycardic with an 8×6-cm mobile tender mass in her lower abdomen. Investigations demonstrated leukocytosis and ultrasonogram confirmed a cystic mass. At laparotomy a pyometra was found. Needle aspiration and washout of the uterine cavity were performed but a second procedure was required to place a drain into the cavity. Pyometra occurs rarely in childhood. A review of cases associates a hypoestrogenized endometrium exposed to transient bacteremia with obstruction to uterine drainage. Treatment should include continuous drainage of the uterus.

  14. Living with Cystic Fibrosis: A Guide for the Young Adult.

    ERIC Educational Resources Information Center

    Cystic Fibrosis Foundation, Atlanta, GA.

    Intended for the young adult with cystic fibrosis, the booklet provides information on dealing with problems and on advances in treatment and detection related to the disease. Addressed are the following topics: description of cystic fibrosis; inheritance of cystic fibrosis; early diagnosis; friends, careers, and other matters; treatment;…

  15. Intra-Abdominal Hypertension and Abdominal Compartment Syndrome in Association with Ruptured Abdominal Aortic Aneurysm in the Endovascular Era: Vigilance Remains Critical

    PubMed Central

    Bozeman, Matthew C.; Ross, Charles B.

    2012-01-01

    Intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS) are common complications of ruptured abdominal aortoiliac aneurysms (rAAAs) and other abdominal vascular catastrophes even in the age of endovascular therapy. Morbidity and mortality due to systemic inflammatory response syndrome (SIRS) and multiple organ failure (MOF) are significant. Recognition and management of IAH are key critical care measures which may decrease morbidity and improve survival in these vascular patients. Two strategies have been utilized: expectant management with prompt decompressive laparotomy upon diagnosis of threshold levels of IAH versus prophylactic, delayed abdominal closure based upon clinical parameters at the time of initial repair. Competent management of the abdominal wound with preservation of abdominal domain is also an important component of the care of these patients. In this review, we describe published experience with IAH and ACS complicating abdominal vascular catastrophes, experience with ACS complicating endovascular repair of rAAAs, and techniques for management of the abdominal wound. Vigilance and appropriate management of IAH and ACS remains critically important in decreasing morbidity and optimizing survival following catastrophic intra-abdominal vascular events. PMID:22454763

  16. Intra-abdominal hypertension and abdominal compartment syndrome in association with ruptured abdominal aortic aneurysm in the endovascular era: vigilance remains critical.

    PubMed

    Bozeman, Matthew C; Ross, Charles B

    2012-01-01

    Intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS) are common complications of ruptured abdominal aortoiliac aneurysms (rAAAs) and other abdominal vascular catastrophes even in the age of endovascular therapy. Morbidity and mortality due to systemic inflammatory response syndrome (SIRS) and multiple organ failure (MOF) are significant. Recognition and management of IAH are key critical care measures which may decrease morbidity and improve survival in these vascular patients. Two strategies have been utilized: expectant management with prompt decompressive laparotomy upon diagnosis of threshold levels of IAH versus prophylactic, delayed abdominal closure based upon clinical parameters at the time of initial repair. Competent management of the abdominal wound with preservation of abdominal domain is also an important component of the care of these patients. In this review, we describe published experience with IAH and ACS complicating abdominal vascular catastrophes, experience with ACS complicating endovascular repair of rAAAs, and techniques for management of the abdominal wound. Vigilance and appropriate management of IAH and ACS remains critically important in decreasing morbidity and optimizing survival following catastrophic intra-abdominal vascular events.

  17. Network for Early Onset Cystic Kidney Diseases-A Comprehensive Multidisciplinary Approach to Hereditary Cystic Kidney Diseases in Childhood.

    PubMed

    König, Jens Christian; Titieni, Andrea; Konrad, Martin

    2018-01-01

    Hereditary cystic kidney diseases comprise a complex group of genetic disorders representing one of the most common causes of end-stage renal failure in childhood. The main representatives are autosomal recessive polycystic kidney disease, nephronophthisis, Bardet-Biedl syndrome, and hepatocyte nuclear factor-1beta nephropathy. Within the last years, genetic efforts have brought tremendous progress for the molecular understanding of hereditary cystic kidney diseases identifying more than 70 genes. Yet, genetic heterogeneity, phenotypic variability, a lack of reliable genotype-phenotype correlations and the absence of disease-specific biomarkers remain major challenges for physicians treating children with cystic kidney diseases. To tackle these challenges comprehensive scientific approaches are urgently needed that match the ongoing "revolution" in genetics and molecular biology with an improved efficacy of clinical data collection. Network for early onset cystic kidney diseases (NEOCYST) is a multidisciplinary, multicenter collaborative combining a detailed collection of clinical data with translational scientific approaches addressing the genetic, molecular, and functional background of hereditary cystic kidney diseases. Consisting of seven work packages, including an international registry as well as a biobank, NEOCYST is not only dedicated to current scientific questions, but also provides a platform for longitudinal clinical surveillance and provides precious sources for high-quality research projects and future clinical trials. Funded by the German Federal Government, the NEOCYST collaborative started in February 2016. Here, we would like to introduce the rationale, design, and objectives of the network followed by a short overview on the current state of progress.

  18. Abdominal Assessment.

    PubMed

    Fritz, Deborah; Weilitz, Pamela Becker

    2016-03-01

    Abdominal pain is one of the most common complaints by patients, and assessment of abdominal pain and associated symptoms can be challenging for home healthcare providers. Reasons for abdominal pain are related to inflammation, organ distention, and ischemia. The history and physical examination are important to narrow the source of acute or chronic problems, identify immediate interventions, and when necessary, facilitate emergency department care.

  19. Low-Grade Myxofibrosarcoma of the Rectus Abdominus Muscle Infiltrating into Abdominal Cavity: A Case Report.

    PubMed

    Nomura, Tadashi; Sakakibara, Shunsuke; Moriwaki, Aya; Kawamoto, Teruya; Suzuki, Satoshi; Ishimura, Takeshi; Hashikawa, Kazunobu; Terashi, Hiroto

    2017-01-01

    Objective: Myxofibrosarcoma (MFS) is a relatively rare tumor that is histologically characterized by myxoid stroma and spindle cell proliferation. This tumor most commonly arises as a slow growing, enlarging painless mass in the extremities of elderly patients. Methods: We report a case of a primary, low-grade MFS in the rectus abdominis muscle infiltrating the abdominal cavity of a 75-year-old man. Results: The patient underwent a wide excision of the right abdominal wall mass with a 3-cm surgical margin from the scar due to a biopsy. The tumor infiltrated the urinary bladder, peritoneum, and external iliac vessels. Twenty-six months after the initial operation, he had recurrences in his abdominal wall, urinary bladder, and right iliac vessels. Conclusions: To our knowledge, primary MFS of the muscle in the abdomen has not been documented previously. Although this case was histopathologically classified as a low-grade tumor, it infiltrated the abdominal cavity. The tumor is suspected to have penetrated the abdominal cavity below the linea arcuata, which lacks the posterior sheath of the rectus abdominis muscle; from there, it could easily spread without being blocked by any biological barriers.

  20. Rehabilitation with Cystic Fibrosis: From Utopia to Reality.

    ERIC Educational Resources Information Center

    Goldberg, Richard T.; And Others

    1980-01-01

    The paper dispels some of the myths regarding cystic fibrosis (a genetic metabolism disorder), provides information on the latest developments in rehabilitation, summarizes research in the field, and projects future needs of the patient with cystic fibrosis. (SBH)

  1. Milk of calcium in abdomen.

    PubMed

    Huang, Yu-Sen; Huang, Kuo-How; Chang, Chin-Chen; Liu, Kao-Lang

    2011-03-01

    A 45-year-old woman had an asymptomatic abnormality on a screening abdominal radiograph. The radiopaque mass in her right upper abdomen was surrounded by numerous "pearls" and resembled an abalone on the supine abdominal radiograph. We advised an additional upright abdominal radiograph, which showed a calcium fluid level. We also clarified the location of the cystic lesion at the right floating kidney, which changed its location between the supine and upright positions. Computed tomography of the abdomen revealed a right renal cyst with a calcium-fluid interface owing to the milk of calcium. The patient was then followed up without additional investigation or the need for intervention. Copyright © 2011 Elsevier Inc. All rights reserved.

  2. Abdominal pythiosis in a Bengal tiger (Panthera tigris tigris).

    PubMed

    Buergelt, Claus; Powe, Joshua; White, Tamara

    2006-06-01

    An adult Bengal tiger (Panthera tigris tigris) housed in an outdoor sanctuary in Florida exhibited vomiting, diarrhea, and weight loss. A clinical workup did not reveal the source of the clinical signs and antibiotic therapy was unrewarding. Radiographs revealed the presence of an abdominal mass. The tiger died during an immobilization for a follow-up clinical examination. A necropsy was performed and tissue samples of intestine and mesenteric lymph nodes were submitted for histopathologic diagnosis. A pyogranulomatous panenteritis and lymphadenitis with intralesional hyphae led to a presumptive etiologic diagnosis of intestinal/abdominal pythiosis. The diagnosis of pythiosis was confirmed by serology and immunoblotting.

  3. Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.

    PubMed

    Lee, Tim W R; Southern, Kevin W

    2013-11-26

    Cystic fibrosis is caused by a defective gene encoding a protein called the cystic fibrosis transmembrane conductance regulator (CFTR), and is characterised by chronic lung infection resulting in inflammation and progressive lung damage that results in a reduced life expectancy. To determine whether topical CFTR gene replacement therapy to the lungs in people with cystic fibrosis is associated with improvements in clinical outcomes, and to assess any adverse effects. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings.Date of most recent search: 22 August 2013.An additional search of the National Institutes for Health (NIH) Genetic Modification Clinical Research Information System (GeMCRIS) was also performed for the years 1992 to 2013.Date of most recent search: 04 September 2013. Randomised controlled trials comparing topical CFTR gene delivery to the lung, using either viral or non-viral delivery systems, with placebo or an alternative delivery system in people with confirmed cystic fibrosis. The authors independently extracted data and assessed study quality. Authors of included studies were contacted and asked for any available additional data. Meta-analysis was limited due to differing study designs. Three randomised controlled trials met the inclusion criteria for this review, involving a total of 155 participants. Fourteen studies were excluded. The included studies differed in terms of CFTR gene replacement agent and study design, which limited the meta-analysis.Although the first Moss study reported a significant improvement in respiratory function (forced expiratory volume at one second) 30 days after participants had received their first dose of gene therapy agent, this finding was not confirmed in their larger second study or in our meta-analysis.In participants

  4. Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.

    PubMed

    Lee, Tim W R; Southern, Kevin W

    2012-10-17

    Cystic fibrosis is caused by a defective gene encoding a protein called the cystic fibrosis transmembrane conductance regulator (CFTR), and is characterised by chronic lung infection resulting in inflammation and progressive lung damage that results in a reduced life expectancy. To determine whether topical CFTR gene replacement therapy to the lungs in people with cystic fibrosis is associated with improvements in clinical outcomes, and to assess any adverse effects. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings.Date of most recent search: 19 July 2012.An additional search of the National Institutes for Health (NIH) Genetic Modification Clinical Research Information System (GeMCRIS) was also performed for the years 1992 to 2012.Date of most recent search: 25 July 2012. Randomised controlled trials comparing topical CFTR gene delivery to the lung, using either viral or non-viral delivery systems, with placebo or an alternative delivery system in people with confirmed cystic fibrosis. The authors independently extracted data and assessed study quality. Authors of included studies were contacted and asked for any available additional data. Meta-analysis was limited due to differing study designs. Three randomised controlled trials met the inclusion criteria for this review, involving a total of 155 participants. Fourteen studies were excluded. The included studies differed in terms of CFTR gene replacement agent and study design, which limited the meta-analysis.Although the first Moss study reported a significant improvement in respiratory function (forced expiratory volume at one second) 30 days after participants had received their first dose of gene therapy agent, this finding was not confirmed in their larger second study or in our meta-analysis.In participants who

  5. Abdominal Aortic Aneurysm

    MedlinePlus

    ... Kids and Teens Pregnancy and Childbirth Women Men Seniors Your Health Resources Healthcare Management End-of-Life ... familydoctor.org editorial staff Categories: Family Health, Men, Seniors, WomenTags: abdominal aorta, abdominal aortic aneurysm, abdominal pain, ...

  6. A cystic fibrosis handbook for teachers.

    PubMed

    Ryan, L L; Williams, J K

    1996-01-01

    The purposes of this project were to (1) develop a handbook on cystic fibrosis for elementary school teachers and to (2) pilot this handbook with a group of teachers and school nurses. The project used a descriptive survey design in which parents, teachers, and school nurses of 14 elementary-age children with cystic fibrosis were recruited from one cystic fibrosis clinic. Interest in using the handbook with their child's teachers was elicited from parents; also, interest in using the handbook was obtained by open-ended questions in a mailed survey sent to teachers and school nurses. Levels of teacher and school nurse knowledge were measured with a true/false pretest and posttest instrument. All parents expressed a desire to use the handbook with their child's teachers. Sixty-seven percent of the teachers and 89% of the school nurses returned the survey, and all endorsed the use of the handbook in their classrooms or schools. Comparison of the pretest and posttest scores from the teachers revealed an increase in teachers' knowledge. Scores on pretest and posttest measures from school nurses were high at each testing time. Results support the use of a printed handbook to promote knowledge of cystic fibrosis in teachers and to support communication among nurses, parents, and teachers.

  7. Liver Disease in Cystic Fibrosis: an Update

    PubMed Central

    Parisi, Giuseppe Fabio; Di Dio, Giovanna; Franzonello, Chiara; Gennaro, Alessia; Rotolo, Novella; Lionetti, Elena; Leonardi, Salvatore

    2013-01-01

    Context Cystic fibrosis (CF) is the most widespread autosomal recessive genetic disorder that limits life expectation amongst the Caucasian population. As the median survival has increased related to early multidisciplinary intervention, other manifestations of CF have emergedespecially for the broad spectrum of hepatobiliary involvement. The present study reviews the existing literature on liver disease in cystic fibrosis and describes the key issues for an adequate clinical evaluation and management of patients, with a focus on the pathogenetic, clinical and diagnostic-therapeutic aspects of liver disease in CF. Evidence Acquisition A literature search of electronic databases was undertaken for relevant studies published from 1990 about liver disease in cystic fibrosis. The databases searched were: EMBASE, PubMed and Cochrane Library. Results CF is due to mutations in the gene on chromosome 7 that encodes an amino acidic polypeptide named CFTR (cystic fibrosis transmembrane regulator). The hepatic manifestations include particular changes referring to the basic CFTR defect, iatrogenic lesions or consequences of the multisystem disease. Even though hepatobiliary disease is the most common non-pulmonary cause ofmortalityin CF (the third after pulmonary disease and transplant complications), only about the 33%ofCF patients presents clinically significant hepatobiliary disease. Conclusions Liver disease will have a growing impact on survival and quality of life of cystic fibrosis patients because a longer life expectancy and for this it is important its early recognition and a correct clinical management aimed atdelaying the onset of complications. This review could represent an opportunity to encourage researchers to better investigate genotype-phenotype correlation associated with the development of cystic fibrosis liver disease, especially for non-CFTR genetic polymorphisms, and detect predisposed individuals. Therapeutic trials are needed to find strategies of

  8. Computed tomography diagnosis of a thoracic and abdominal penetrating foreign body in a dog

    PubMed Central

    Appleby, Ryan; zur Linden, Alex; Singh, Ameet; Finck, Cyrielle; Crawford, Evan

    2015-01-01

    A 1.5-year-old, spayed female, mixed-breed dog was presented for hemoabdomen associated with an abdominal mass. Upon presentation bicavitary effusion was diagnosed. A penetrating intra-abdominal wooden foreign body was identified using computed tomography. This case describes a thoracic penetrating wooden foreign body causing bicavitary effusion following migration into the retroperitoneal space. PMID:26538669

  9. Dental treatment for people with cystic fibrosis.

    PubMed

    Harrington, N; Barry, P J; Barry, S M

    2016-06-01

    To describe the nature and consequences of the multi-system genetic condition cystic fibrosis with a view to ensuring optimal dental treatment planning for these patients. A literature search was conducted to identify the key medical and dental manifestations of cystic fibrosis. These findings are discussed and utilised to create recommendations for treatment planning in patients with cystic fibrosis for the practising dental practitioner. Cystic fibrosis is a complex, lethal, multisystem autosomal recessive disorder resulting from mutations on chromosome 7 which result in dysfunction of an ion channel that sits on epithelial surfaces. Respiratory disease remains the leading cause of mortality. Survival has greatly increased in recent decades secondary to improved treatment and specialist care. Specific dental manifestations of the disease may result from the condition itself or complications of treatment. Modification of patient management may be necessary to provide optimum patient care. The pathophysiology and clinical manifestations are relevant to practicing dental practitioners and inform recommendations to be utilised to ensure optimal treatment planning for these patients.

  10. Protein Sequencing with Tandem Mass Spectrometry

    NASA Astrophysics Data System (ADS)

    Ziady, Assem G.; Kinter, Michael

    The recent introduction of electrospray ionization techniques that are suitable for peptides and whole proteins has allowed for the design of mass spectrometric protocols that provide accurate sequence information for proteins. The advantages gained by these approaches over traditional Edman Degradation sequencing include faster analysis and femtomole, sometimes attomole, sensitivity. The ability to efficiently identify proteins has allowed investigators to conduct studies on their differential expression or modification in response to various treatments or disease states. In this chapter, we discuss the use of electrospray tandem mass spectrometry, a technique whereby protein-derived peptides are subjected to fragmentation in the gas phase, revealing sequence information for the protein. This powerful technique has been instrumental for the study of proteins and markers associated with various disorders, including heart disease, cancer, and cystic fibrosis. We use the study of protein expression in cystic fibrosis as an example.

  11. Cardiovascular risk factors in middle age obese Indians: a cross-sectional study on association of per cent body fat and intra-abdominal fat mass.

    PubMed

    Sandhu, Jaspal Singh; Esht, Vandana; Shenoy, Shweta

    2012-01-01

    To determine the association of per cent total body fat (TBF), intra-abdominal fat (IAF) mass and subcutaneous abdominal fat with cardiovascular risk factors in middle age obese Indians. Cross-sectional study. Hydrostatic Laboratory, Department of Sports Medicine and Physiotherapy, Guru Nanak Dev University, India. 51 subjects aged 30-55 years with a body mass index value 23 and above. In all the participants, TBF was estimated by underwater weighing machine and IAF and subcutaneous fat were measured by ultrasonography. Lipid profile was determined by a semiautomated analyser. Main outcome measures were: IAF, per cent body fat to TBF ratio, lipid profile and risk of developing cardiovascular diseases. IAF was found to be significantly associated with lipid variables (95% CI, p<0.01) and risk of developing cardiovascular diseases (95% CI, p≤0.05) in both male and female subjects. TBF and subcutaneous fat thickness showed no significant results (95% CI, p>0.05) with either lipid variables or risk of developing cardiovascular diseases (tables 1 and 2). IAF mass showed significant association with age (95% CI, p<0.01) and significant negative association with physical activity (95% CI, p<0.05) in male subjects (tables 3 and 4). An ultrasonic measurement of IAF is a better predictor of the risk of developing cardiovascular diseases in middle aged Indian population. In male subjects, physical activity of 5 or more days a week showed lesser amount of IAF as compared with those with physical activity <5 days a week.

  12. Cardiovascular risk factors in middle age obese Indians: a cross-sectional study on association of per cent body fat and intra-abdominal fat mass

    PubMed Central

    Sandhu, Jaspal Singh; Esht, Vandana; Shenoy, Shweta

    2012-01-01

    Objectives To determine the association of per cent total body fat (TBF), intra-abdominal fat (IAF) mass and subcutaneous abdominal fat with cardiovascular risk factors in middle age obese Indians. Design Cross-sectional study. Setting Hydrostatic Laboratory, Department of Sports Medicine and Physiotherapy, Guru Nanak Dev University, India. Participants: 51 subjects aged 30–55 years with a body mass index value 23 and above. Methodology In all the participants, TBF was estimated by underwater weighing machine and IAF and subcutaneous fat were measured by ultrasonography. Lipid profile was determined by a semiautomated analyser. Main outcome measures were: IAF, per cent body fat to TBF ratio, lipid profile and risk of developing cardiovascular diseases. Results IAF was found to be significantly associated with lipid variables (95% CI, p<0.01) and risk of developing cardiovascular diseases (95% CI, p≤0.05) in both male and female subjects. TBF and subcutaneous fat thickness showed no significant results (95% CI, p>0.05) with either lipid variables or risk of developing cardiovascular diseases (tables 1 and 2). IAF mass showed significant association with age (95% CI, p<0.01) and significant negative association with physical activity (95% CI, p<0.05) in male subjects (tables 3 and 4). Conclusion An ultrasonic measurement of IAF is a better predictor of the risk of developing cardiovascular diseases in middle aged Indian population. In male subjects, physical activity of 5 or more days a week showed lesser amount of IAF as compared with those with physical activity <5 days a week. PMID:27326015

  13. Body image, nutritional status, abdominal strength, and cardiorespiratory fitness in children and adolescents practicing sports.

    PubMed

    Schubert, Alexandre; Januário, Renata Selvatici B; Casonatto, Juliano; Sonoo, Christi Noriko

    2013-01-01

    To verify the association between nutritional status, physical fitness, and body image in children and adolescents. This cross-sectional study included 401 students (236 boys and 165 girls) aged between 8 and 16 years that were regularly enrolled in sports in the local clubs. The nutritional status was evaluated by the body mass index. Students were assessed for satisfaction with body image, abdominal strength resistance, and cardiorespiratory fitness. The variables were assessed on the same day following a standardized order. In order to verify relationships between variables, the chi-square test was used. Afterwards, the binary logistic regression was applied to identify the magnitude of the associations, considering p<0.05 as significant. Association was found between body image and body mass index (p=0.001), abdominal strength resistance (p=0.005) and cardiorespiratory fitness (p=0.001). The Odds Ratio for presenting the body image insatisfaction for those who have not achieved the expected values for the health criteria in abdominal strength resistance and cardiorespiratory fitness were 2.14 and 2.42 times respectively, and for those with overweight and obesity, 2.87 times. Insatisfaction with body image is associated with body mass index and also with physical fitness, abdominal strength resistance, and cardiorespiratory fitness variables.

  14. Laparoscopic resection of adult colon duplication causing intussusception

    PubMed Central

    Kyo, Kennoki; Azuma, Masaki; Okamoto, Kazuya; Nishiyama, Motohiro; Shimamura, Takahiro; Maema, Atsushi; Shirakawa, Motoaki; Nakamura, Toshio; Koda, Kenji; Yokoyama, Hidetaro

    2016-01-01

    Gastrointestinal duplications are uncommon congenital malformations that can occur anywhere along the gastrointestinal tract. Most cases are recognized before the age of 2 years, and those encountered in adults are rare. We describe here a case of ascending colon duplication in a 20-year-old male that caused intussusception and was treated laparoscopically. Although computed tomography revealed a cystic mass filled with stool-like material, the preoperative diagnosis was a submucosal tumor of the ascending colon. We performed a laparoscopic right colectomy, and the postoperative pathological diagnosis was duplication of the ascending colon, both cystic and tubular components. We conclude that gastrointestinal duplications, although rare, should be considered in the differential diagnosis of all abdominal and submucosal cystic lesions and that laparoscopy is a preferred approach for the surgical treatment of gastrointestinal duplications. PMID:26900303

  15. How the airway smooth muscle in cystic fibrosis reacts in proinflammatory conditions: implications for airway hyper-responsiveness and asthma in cystic fibrosis.

    PubMed

    McCuaig, Sarah; Martin, James G

    2013-04-01

    Among patients with cystic fibrosis there is a high prevalence (40-70%) of asthma signs and symptoms such as cough and wheezing and airway hyper-responsiveness to inhaled histamine or methacholine. Whether these abnormal airway responses are due to a primary deficiency in the cystic fibrosis transmembrane conductance regulator (CFTR) or are secondary to the inflammatory environment in the cystic fibrosis lungs is not clear. A role for the CFTR in smooth muscle function is emerging, and alterations in contractile signalling have been reported in CFTR-deficient airway smooth muscle. Persistent bacterial infection, especially with Pseudomonas aeruginosa, stimulates interleukin-8 release from the airway epithelium, resulting in neutrophilic inflammation. Increased neutrophilia and skewing of CFTR-deficient T-helper cells to type 2 helper T cells creates an inflammatory environment characterised by high concentrations of tumour necrosis factor α, interleukin-8, and interleukin-13, which might all contribute to increased contractility of airway smooth muscle in cystic fibrosis. An emerging role of interleukin-17, which is raised in patients with cystic fibrosis, in airway smooth muscle proliferation and hyper-responsiveness is apparent. Increased understanding of the molecular mechanisms responsible for the altered smooth muscle physiology in patients with cystic fibrosis might provide insight into airway dysfunction in this disease. Copyright © 2013 Elsevier Ltd. All rights reserved.

  16. Bartter syndrome presenting as poor weight gain and abdominal mass in an infant.

    PubMed

    Heffernan, Annie; Steffensen, Thora S; Gilbert-Barness, Enid; Perlman, Sharon

    2008-01-01

    Bartter syndrome, a group of disorders that encompasses multiple genetic defects with similar clinical presentation, has been divided into six different genotypes, according to different genetic defects, and into three main clinical variants (or phenotypes). Classic laboratory findings in all variants include hypochloremia, hypokalemia, and metabolic alkalosis with excessive excretion of chloride and potassium. Classic Bartter syndrome, neonatal Bartter syndrome, and Gitelman syndrome are the three main clinical variants. Classic Bartter syndrome and neonatal Bartter syndrome have defects in genes that affect transport channels in the ascending loop of Henle, where as in Gitleman syndrome the defect occurs in the transport channels of the distal convoluted tubule. Classic Bartter syndrome and neonatal Bartter syndrome have similar presenting symptoms, potential outcomes, and treatment, but different ages at presentation. Gitelman syndrome, a more benign condition than the other clinical variants, has the classic hallmark finding of hypomagnesemia and low to normal excretion of calcium. This differentiates it from the classic and neonatal variants of the disease. With early diagnosis and proper treatment, Bartter syndrome has a good prognosis. But failure to identify it can lead to tubulointerstitial nephritis and renal failure. We present a case of a 6-month-old boy with Bartter syndrome who presented with poor weight gain and an abdominal mass.

  17. [Recurrent benign cystic peritoneal mesothelioma].

    PubMed

    Stroescu, C; Negulescu, Raluca; Herlea, V; David, L; Ivanov, B; Nitipir, Cornelia; Popescu, I

    2008-01-01

    The benign cystic peritoneal mesothelioma (BCPM) is a rare neoplasm affecting mainly females at reproductive age. The natural history and physiopathology of the BCPM are not entirely known. It is mainly characterized by the lack of malignant elements, no tendency to metastasis and by a pervasive tendency to generate local recurrences after surgical removal. The clinical manifestations are insidious, uncharacteristic; the benign cystic peritoneal mesothelioma is often discovered during a surgical procedure addressing another condition. Imaging tests can raise the suspicion of BCPM but the diagnostic can only be confirmed by histopathological examination corroborated with an immunohistochemical analysis. There are no long term studies dictating a single therapeutic attitude but a high risk of local recurrences and the possibility of transformation into malignant mesothelioma have lead to the current tendency towards an aggressive treatment of the tumor. We present the case of a recurrent benign cystic peritoneal mesothelioma in a 40 years old female patient, emphasizing the therapeutic approach and the role of radical surgery in the treatment of BPCM.

  18. Physical exercise training for cystic fibrosis.

    PubMed

    Radtke, Thomas; Nevitt, Sarah J; Hebestreit, Helge; Kriemler, Susi

    2017-11-01

    Physical exercise training may form an important part of regular care for people with cystic fibrosis. This is an update of a previously published review. To assess the effects of physical exercise training on exercise capacity by peak oxygen consumption, pulmonary function by forced expiratory volume in one second, health-related quality of life and further important patient-relevant outcomes in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 04 May 2017.We searched ongoing trials registers (clinicaltrials.gov and the WHO ICTRP). Date of most recent search: 10 August 2017. All randomised and quasi-randomised controlled clinical trials comparing exercise training of any type and a minimum duration of two weeks with conventional care (no training) in people with cystic fibrosis. Two authors independently selected studies for inclusion, assessed methodological quality and extracted data. The quality of the evidence was assessed using the GRADE system. Of the 83 studies identified, 15 studies which included 487 participants, met the inclusion criteria. The numbers in each study ranged from nine up to 72 participants; two studies were in adults, seven were in children and adolescents and six studies included all age ranges. Four studies of hospitalised participants lasted less than one month and 11 studies were outpatient-based, lasting between two months and three years. The studies included participants with a wide range of disease severity and employed differing levels of supervision with a mixture of types of training. There was also wide variation in the quality of the included studies.This systematic review shows very low- to low-quality evidence from both short- and long-term studies that in people

  19. PULMONARY CYSTIC ECHINOCOCCOSIS

    PubMed Central

    Santivanez, Saul; Garcia, Hector H.

    2012-01-01

    Pulmonary cystic echinococosis, a zoonosis caused by the larvae of the dog tapeworm Echinococcus granulosus, is considered as a major public health problem in those countries where dogs are used to care for large herds because of the incapacitating effects produced in affected population. The ratio lung:liver involvement is higher in children than in adults. A higher proportion of lung cases are discovered incidentally on a routine x-ray evaluation; the majority of infected people remain asymptomatic until the cyst enlarges sufficiently to cause symptoms. The majority of symptoms are caused by mass effect from the cyst volume; the presence of complications caused by cysts broke changes the clinical presentation; the principal complication is cyst rupture, producing cough, chest pain, hemoptysis, or vomica. Diagnosis is obtained by imaging evaluation (Chest X-ray or CT scan), supported by serology in the majority of cases. Surgery is the main therapeutic approach, having as principal objective, the removal of the parasite, preventing intraoperative dissemination; the use of pre surgical chemotherapy reduces the chances of seeding and recurrence; treatment using benzimidazoles is the preferred treatment when surgery is not available, or complete removal is not feasible PMID:20216420

  20. Leiomyoma of broad ligament mimicking ovarian malignancy- report of a unique case.

    PubMed

    Mallick, D; Saha, M; Chakrabarti, S; Chakraborty, J

    2014-01-01

    Tumors of the broad ligament are uncommon. Leiomyoma, which is the commonest female genital neoplasm, is also the most common solid tumor of the broad ligament. Leiomyomas affect 30% of all women of reproductive age but the incidence of broad-ligament leiomyoma is <1%. These benign tumors are usually asymptomatic. A case is being described where a 52 year old presented with gradual abdominal swelling which was clinically and radiologically diagnosed as ovarian malignancy. On abdominal and bimanual palpation a soft cystic mass was noted in the right pelvic region. CA 125 was mildly raised. CEA, CA 19.9 levels were within normal limit. The radiological diagnosis was ovarian cyst with possibility of malignant changes. Staging laparotomy and histopathological examination of the resected specimen revealed a right sided broad ligament leiomyoma with cystic changes. The degenerative changes in the leiomyoma lead to the clinical and radiological diagnostic confusion. Thus, though uncommon, broad ligament leiomyoma should be considered during evaluation of adnexal masses for optimal patient management. The above description of leiomyoma in the broad ligament is a highly unique case and thus deserves appropriate attention.

  1. CFTR Genotype and Maximal Exercise Capacity in Cystic Fibrosis: A Cross-sectional Study.

    PubMed

    Radtke, Thomas; Hebestreit, Helge; Gallati, Sabina; Schneiderman, Jane E; Braun, Julia; Stevens, Daniel; Hulzebos, Erik Hj; Takken, Tim; Boas, Steven R; Urquhart, Don S; Lands, Larry C; Tejero, Sergio; Sovtic, Aleksandar; Dwyer, Tiffany; Petrovic, Milos; Harris, Ryan A; Karila, Chantal; Savi, Daniela; Usemann, Jakob; Mei-Zahav, Meir; Hatziagorou, Elpis; Ratjen, Felix; Kriemler, Susi

    2018-02-01

    relevant confounders, lung function and body mass index were associated with V.O2peak. CFTR functional genotype class was not associated with maximal exercise capacity in patients with cystic fibrosis overall, but those with at least one copy of a F508del-CFTR mutation and a single class V mutation had lower maximal exercise capacity.

  2. Network for Early Onset Cystic Kidney Diseases—A Comprehensive Multidisciplinary Approach to Hereditary Cystic Kidney Diseases in Childhood

    PubMed Central

    König, Jens Christian; Titieni, Andrea; Konrad, Martin; Bergmann, C.

    2018-01-01

    Hereditary cystic kidney diseases comprise a complex group of genetic disorders representing one of the most common causes of end-stage renal failure in childhood. The main representatives are autosomal recessive polycystic kidney disease, nephronophthisis, Bardet–Biedl syndrome, and hepatocyte nuclear factor-1beta nephropathy. Within the last years, genetic efforts have brought tremendous progress for the molecular understanding of hereditary cystic kidney diseases identifying more than 70 genes. Yet, genetic heterogeneity, phenotypic variability, a lack of reliable genotype–phenotype correlations and the absence of disease-specific biomarkers remain major challenges for physicians treating children with cystic kidney diseases. To tackle these challenges comprehensive scientific approaches are urgently needed that match the ongoing “revolution” in genetics and molecular biology with an improved efficacy of clinical data collection. Network for early onset cystic kidney diseases (NEOCYST) is a multidisciplinary, multicenter collaborative combining a detailed collection of clinical data with translational scientific approaches addressing the genetic, molecular, and functional background of hereditary cystic kidney diseases. Consisting of seven work packages, including an international registry as well as a biobank, NEOCYST is not only dedicated to current scientific questions, but also provides a platform for longitudinal clinical surveillance and provides precious sources for high-quality research projects and future clinical trials. Funded by the German Federal Government, the NEOCYST collaborative started in February 2016. Here, we would like to introduce the rationale, design, and objectives of the network followed by a short overview on the current state of progress. PMID:29497606

  3. Intra-abdominal hypertension and abdominal compartment syndrome in pediatrics. A review.

    PubMed

    Thabet, Farah Chedly; Ejike, Janeth Chiaka

    2017-10-01

    To consolidate pediatric intensivists' understanding of the pathophysiology, definition, incidence, monitoring, and management of intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS); and to highlight the characteristics related to the pediatric population. This is a narrative review article that utilized a systematic search of the medical literature published in the English language between January 1990 and august 2016. Studies were identified by conducting a comprehensive search of Pub Med databases. Search terms included "intra-abdominal hypertension and child", "intra-abdominal hypertension and pediatrics", "abdominal compartment syndrome and child", and "abdominal compartment syndrome and pediatrics". Intra-abdominal hypertension and ACS are associated with a number of pathophysiological disturbances and increased morbidity and mortality. These conditions have been well described in critically ill adults. In children, the IAH and the ACS have a reported incidence of 13% and 0.6 to 10% respectively; they carry similar prognostic impact but are still under-diagnosed and under-recognized by pediatric health care providers. Intra-abdominal hypertension and ACS are conditions that are regularly encountered in critically ill children. They are associated with an increased morbidity and mortality. Early recognition, prevention and timely management of this critical condition are necessary to improve its outcome. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. Inadequate erythroid response to hypoxia in cystic fibrosis.

    PubMed

    Vichinsky, E P; Pennathur-Das, R; Nickerson, B; Minor, M; Kleman, K; Higashino, S; Lubin, B

    1984-07-01

    An increase in hemoglobin concentration characterizes the normal compensatory response to chronic tissue hypoxia. We observed no such increase in 42 chronically hypoxic patients with cystic fibrosis, in whom the mean concentration was 12.6 gm/dl; one third of the patients were anemic. Compared with patients with cyanotic heart disease, patients with cystic fibrosis did not have a compensatory increase in P50 or 2,3-diphosphoglycerate. Despite anemia, erythropoietin levels in patients with cystic fibrosis were not significantly different from normal control values. The growth of colony-forming units-erythroid in patients with cystic fibrosis was similar to that in control subjects, and there was no inhibition of growth with the addition of autologous serum. Erythropoietin sensitivity, determined by measuring the CFUe dose response curve, was normal in both patients and controls. Results of iron studies were consistent with iron deficiency in the majority of patients. Impaired absorption of iron was observed in six of 13 iron-deficient patients with cystic fibrosis. An inverse correlation between erythrocyte sedimentation rate and peak serum iron was obtained during the iron absorption study. Eight patients who underwent a therapeutic trial of iron demonstrated a 1.8 gm/dl rise in hemoglobin concentration. Two patients with previously documented iron malabsorption responded to parenteral iron therapy after failure to respond to oral supplementation. These studies demonstrate that patients with cystic fibrosis not only have an impaired erythroid response to hypoxia, but are frequently anemic. Their inadequate erythroid response to hypoxia results in part from disturbances in erythropoietin regulation and iron availability.

  5. Plain abdominal radiography in acute abdominal pain; past, present, and future

    PubMed Central

    Gans, Sarah L; Stoker, Jaap; Boermeester, Marja A

    2012-01-01

    Several studies have demonstrated that a diagnosis based solely on a patient’s medical history, physical examination, and laboratory tests is not reliable enough, despite the fact that these aspects are essential parts of the workup of a patient presenting with acute abdominal pain. Traditionally, imaging workup starts with abdominal radiography. However, numerous studies have demonstrated low sensitivity and accuracy for plain abdominal radiography in the evaluation of acute abdominal pain as well as various specific diseases such as perforated viscus, bowel obstruction, ingested foreign body, and ureteral stones. Computed tomography, and in particular computed tomography after negative ultrasonography, provides a better workup than plain abdominal radiography alone. The benefits of computed tomography lie in decision-making for management, planning of a surgical strategy, and possibly even avoidance of negative laparotomies. Based on abundant available evidence, major advances in diagnostic imaging, and changes in the management of certain diseases, we can conclude that there is no place for plain abdominal radiography in the workup of adult patients with acute abdominal pain presenting in the emergency department in current practice. PMID:22807640

  6. Adult cystic hygroma: successful use of OK-432 (Picibanil).

    PubMed

    Woolley, S L; Smith, D R K; Quine, S

    2008-11-01

    We report an adult case of cystic lymphangioma treated with OK-432 (Picibanil). A case report and review of the literature concerning the use of OK-432 to treat cystic lymphangioma is presented. A 31-year-old woman developed a cystic lymphangioma four weeks post-partum. This was treated initially by aspiration, for diagnostic purposes. Investigation suggested that surgery would be challenging. A review of the literature demonstrated success with OK-432 in the treatment of this condition, although primarily in the paediatric population. This patient was successfully treated thus, and at the time of writing remained symptom free. A suggested management plan is outlined. Treatment with OK-432 is useful in the management of cystic lymphangiomas in adults and should be considered as first line treatment.

  7. Normal sweat chloride test does not rule out cystic fibrosis.

    PubMed

    Başaran, Abdurrahman Erdem; Karataş-Torun, Nimet; Maslak, İbrahim Cemal; Bingöl, Ayşen; Alper, Özgül M

    2017-01-01

    Başaran AE, Karataş-Torun N, Maslak İC, Bingöl A, Alper ÖM. Normal sweat chloride test does not rule out cystic fibrosis. Turk J Pediatr 2017; 59: 68-70. A 5-month-old patient presented with complaints of fever and cough. He was hospitalized with the diagnosis of bronchopneumonia and pseudo-Bartter's syndrome. Patient was further investigated for diagnosis of cystic fibrosis. The chloride (Cl) level in sweat was determined within the normal range (25.1 mmol/L, 20.3 mmol/L). CFTR (Cystic Fibrosis Transmembrane Regulator gene; NM_000492.2) genotyping results were positive for p.E92K; p.F1052V mutations. The patient was diagnosed with cystic fibrosis. In our patient, with features of CF and normal sweat test, mutation analysis was helpful for the diagnosis of cystic fibrosis.

  8. Serum lipoprotein concentrations in cystic fibrosis

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Vaughan, W.J.; Lindgren, F.T.; Whalen, J.B.

    1978-02-17

    Two major classes of lipoproteins, low density and high density, are decreased in the serum of patients with cystic fibrosis; major apoproteins are also decreased. Since essential fatty acids and certain fat-soluble vitamins depend on lipoproteins for transport in the serum, knowledge of lipoprotein levels in cystic fibrosis patients could prove valuable in understanding (i) the basis for the abnormally low serum levels of these fatty acids and vitamins and (ii) the effects of therapies involving these molecules.

  9. Laparoscopic management of cystic disease of the liver.

    PubMed

    Albrink, M H; McAllister, E W; Rosemurgy, A S; Karl, R C; Carey, L C

    1994-04-01

    Laparoscopic surgical procedures are increasing in scope and in variety. The benefits of decreased wound morbidity and pain have been well documented for multiple procedures that have traditionally required laparotomy. Although there are few controlled studies to document them, these benefits may be evident from simple clinical observation. Cystic disease of the liver is a condition that is treated largely for symptomatic reasons. The so-called noninvasive or radiographic guided methods of treatment for cystic disease of the liver are fraught with high recurrence rates. We present four cases of cystic disease of the liver treated laparoscopically, followed with pertinent discussion.

  10. Abdominal Trauma Revisited.

    PubMed

    Feliciano, David V

    2017-11-01

    Although abdominal trauma has been described since antiquity, formal laparotomies for trauma were not performed until the 1800s. Even with the introduction of general anesthesia in the United States during the years 1842 to 1846, laparotomies for abdominal trauma were not performed during the Civil War. The first laparotomy for an abdominal gunshot wound in the United States was finally performed in New York City in 1884. An aggressive operative approach to all forms of abdominal trauma till the establishment of formal trauma centers (where data were analyzed) resulted in extraordinarily high rates of nontherapeutic laparotomies from the 1880s to the 1960s. More selective operative approaches to patients with abdominal stab wounds (1960s), blunt trauma (1970s), and gunshot wounds (1990s) were then developed. Current adjuncts to the diagnosis of abdominal trauma when serial physical examinations are unreliable include the following: 1) diagnostic peritoneal tap/lavage, 2) surgeon-performed ultrasound examination; 3) contrast-enhanced CT of the abdomen and pelvis; and 4) diagnostic laparoscopy. Operative techniques for injuries to the liver, spleen, duodenum, and pancreas have been refined considerably since World War II. These need to be emphasized repeatedly in an era when fewer patients undergo laparotomy for abdominal trauma. Finally, abdominal trauma damage control is a valuable operative approach in patients with physiologic exhaustion and multiple injuries.

  11. The Molecular Biology of Adenoid Cystic Carcinoma

    PubMed Central

    Liu, Jia; Shao, Chunbo; Tan, Marietta L.; Mu, David; Ferris, Robert L.; Ha, Patrick K.

    2011-01-01

    Background Adenoid cystic carcinoma (ACC) is an unusual salivary gland malignancy that remains poorly understood. Standard treatment, including surgery with postoperative radiation therapy have attained reasonable local control rates, but the propensity for distant metastases has limited any improvement in survival over time. Our understanding of the molecular mechanisms driving adenoid cystic carcinoma is quite rudimentary, due to the infrequent nature of its occurrence. Methods An extensive literature review was performed on salivary gland adenoid cystic carcinoma and basic science research findings. Results This review highlights many findings that are emerging about the carcinogenesis of ACC including cytogenetics, tumor suppressor genes, oncogenes, epigenetic alterations, mitochondrial alterations, and biomarker studies. Conclusions While there have been many discoveries, much still remains unknown about this rare malignancy. PMID:22006498

  12. Abdominal war wounds--experiences from Red Cross field hospitals.

    PubMed

    Leppäniemi, Ari K

    2005-01-01

    The traditional approach to abdominal war wounds consists of triage, eche-loned care, and mandatory laparotomy for penetrating abdominal injuries, and it remains valid in modern conventional wars with well-organized evacuation and surgical services. Expectant management of abdominal casualties can be considered under difficult circumstances with a high influx of patients exhausting the available resources. This can occur in regional conflicts associated with mass movements of people and with collapsed infrastructure. While always combined with adequate fluid resuscitation, antibiotic treatment, and other supportive care, the expectant approach in patients with penetrating abdominal injuries could be indicated for asymptomatic patients with multiple fragment wounds or for patients presenting several days post-injury in good condition. The focus of surgical resources and competence should be on the majority of patients with intestinal perforation only, who need surgery to save life--but not necessarily on an urgent basis--and who have a good chance of survival. The limited availability of blood products to correct blood loss and coagulation factor deficiencies, and the lack of sophisticated monitoring of hemodynamic variables that call into question the value of a damage-control approach for the most severely injured. Even if the bleeding could be temporarily controlled, the subsequent need for adequate resuscitation before returning the patient to the operating room could be difficult to achieve and would result in incompletely resuscitated patients being reoperated while acidotic, coagulopathic, and even hypothermic. Perhaps, in mass casualty situations these patients should be recognized during triage or at least early during operation, and aggressive surgery should be replaced with adequate expectant management with sedation and analgesics.

  13. [Benign multicystic peritoneal mesothelioma in a patient with Crohn disease].

    PubMed

    Fluxá, Daniela; Kronberg, Udo; Lubascher, Jaime; O'Brien, Andrés; Las Heras, Facundo; Ibáñez, Patricio; Quera, Rodrigo

    2016-12-01

    Benign multicystic peritoneal mesothelioma is an uncommon lesion arising from the peritoneal mesothelium. It is asymptomatic or presents with unspecific symptoms. Imaging techniques may reveal it, however the final diagnosis can only be made by histopathology. Surgery is the only effective treatment considering its high recurrence rate. We report a 19 years old male with Crohn’s disease. Due to persistent abdominal pain, an abdominal magnetic resonance imaging was performed, showing a complex cystic mass in the lower abdomen. The patient underwent surgery and the lesion was completely resected. The pathological study reported a benign multicystic peritoneal mesothelioma.

  14. Management of complex abdominal wall defects associated with penetrating abdominal trauma.

    PubMed

    Arul, G Suren; Sonka, B J; Lundy, J B; Rickard, R F; Jeffery, S L A

    2015-03-01

    The paradigm of Damage Control Surgery (DCS) has radically improved the management of abdominal trauma, but less well described are the options for managing the abdominal wall itself in an austere environment. This article describes a series of patients with complex abdominal wall problems managed at the UK-led Role 3 Medical Treatment Facility (MTF) in Camp Bastion, Afghanistan. Contemporaneous review of a series of patients with complex abdominal wall injuries who presented to the Role 3 MTF between July and November 2012. Five patients with penetrating abdominal trauma associated with significant damage to the abdominal wall were included. All patients were managed using DCS principles, leaving the abdominal wall open at the end of the first procedure. Subsequent management of the abdominal wall was determined by a multidisciplinary team of general and plastic surgeons, intensivists and specialist nurses. The principles of management identified included minimising tissue loss on initial laparotomy by joining adjacent wounds and marginal debridement of dead tissue; contraction of the abdominal wall was minimised by using topical negative pressure dressing and dermal-holding sutures. Definitive closure was timed to allow oedema to settle and sepsis to be controlled. Closure techniques include delayed primary closure with traction sutures, components separation, and mesh closure with skin grafting. A daily multidisciplinary team discussion was invaluable for optimal decision making regarding the most appropriate means of abdominal closure. Dermal-holding sutures were particularly useful in preventing myostatic contraction of the abdominal wall. A simple flow chart was developed to aid decision making in these patients. This flow chart may prove especially useful in a resource-limited environment in which returning months or years later for closure of a large ventral hernia may not be possible. Published by the BMJ Publishing Group Limited. For permission to use

  15. Copy Number Variations in Candidate Genes and Intergenic Regions Affect Body Mass Index and Abdominal Obesity in Mexican Children

    PubMed Central

    Burguete-García, Ana Isabel; Bonnefond, Amélie; Peralta-Romero, Jesús; Froguel, Philippe

    2017-01-01

    Introduction. Increase in body weight is a gradual process that usually begins in childhood and in adolescence as a result of multiple interactions among environmental and genetic factors. This study aimed to analyze the relationship between copy number variants (CNVs) in five genes and four intergenic regions with obesity in Mexican children. Methods. We studied 1423 children aged 6–12 years. Anthropometric measurements and blood levels of biochemical parameters were obtained. Identification of CNVs was performed by real-time PCR. The effect of CNVs on obesity or body composition was assessed using regression models adjusted for age, gender, and family history of obesity. Results. Gains in copy numbers of LEPR and NEGR1 were associated with decreased body mass index (BMI), waist circumference (WC), and risk of abdominal obesity, whereas gain in ARHGEF4 and CPXCR1 and the intergenic regions 12q15c, 15q21.1a, and 22q11.21d and losses in INS were associated with increased BMI and WC. Conclusion. Our results indicate a possible contribution of CNVs in LEPR, NEGR1, ARHGEF4, and CPXCR1 and the intergenic regions 12q15c, 15q21.1a, and 22q11.21d to the development of obesity, particularly abdominal obesity in Mexican children. PMID:28428959

  16. [MALIGNANT TUMORS IN OVARIAN MATURE CYSTIC TERATOMAS INTRAOPERATIVE DIAGNOSTIC BASIS].

    PubMed

    Khachatryan, A

    2016-11-01

    Extremely rare ovarian primary tumors formed in a mature cystic teratomaare described in the literature. This research work studies the frequency of malignant mature cystic teratoma, as well as their clinical and morphological features and necessity of intraoperative histological examination of all teratomas. Cases histories of 56 patients, suffering from ovarian mature cystic teratomahave been studied in MC Shengavit in the period of 2003 - 2015. Among them 4 patients with the somatic malignancies were identified. Morphological methods, which are considered to be "gold standard" of tumor investigation, were used in staining the slides with hematoxylin - eosin. According to the literature the secondary malignant transformation rarely occurs and is typical in postmenopausal women, with a frequency of 0.17-3%. According to the results of our study, malignant tumors in mature cystic teratomas were observed in 4 (7,14%) from the total number of mature cystic teratomas (n=56). There was not revealed a correlation between the duration of the complaints, age of the patients, sizes of ovarian mature teratoma and malignization degree. Thus, the greatest difficulties of clinical diagnosis of malignant tumors in the ovarian mature cystic teratomas were in the early stage of the disease, because of a variety of clinical manifestations, not pathognomonic for malignization. All mentioned symptoms may be observed in the patients with usual mature cystic teratomas. Тhis cases confirm the necessity to take tissue samples from the other ovary for intraoperative histopathological evaluation in each case of mature cystic teratomas. It is necessary to examine a large number of tumor sites, to prevent errors in the assessment of the maturity degree of teratoma.

  17. Lipoma of round ligament on the intraperitoneal portion (abdominal site): a case report.

    PubMed

    Ozdemır, Ozhan; Sarı, Mustafa Erkan; Sakar, Vefa Selimova; Nebıoglu, Mehriban; Atalay, Cemal Resat

    2017-09-01

    Primary tumors of round ligament are rare, and when found are typically leiomyomas. Endometrioma, and mesothelial cysts are the benign lesions recognized as involving the round ligament. We report a case of lipoma of the round ligament in a 48-year-old premenopausal woman. Round ligament lipoma on the intraperitoneal portion (abdominal site) is very rare and it should be kept in the differential diagnosis of ovarian and abdominal masses.

  18. The role of anaerobic bacteria in the cystic fibrosis airway.

    PubMed

    Sherrard, Laura J; Bell, Scott C; Tunney, Michael M

    2016-11-01

    Anaerobic bacteria are not only normal commensals, but are also considered opportunistic pathogens and have been identified as persistent members of the lower airway community in people with cystic fibrosis of all ages and stages of disease. Currently, the role of anaerobic bacteria in cystic fibrosis lower airway disease is not well understood. Therefore, this review describes the recent studies relating to the potential pathophysiological role(s) of anaerobes within the cystic fibrosis lungs. The most frequently identified anaerobic bacteria in the lower airways are common to both cystic fibrosis and healthy lungs. Studies have shown that in cystic fibrosis, the relative abundance of anaerobes fluctuates in the lower airways with reduced lung function and increased inflammation associated with a decreased anaerobic load. However, anaerobes found within the lower airways also produce virulence factors, may cause a host inflammatory response and interact synergistically with recognized pathogens. Anaerobic bacteria are potentially members of the airway microbiota in health but could also contribute to the pathogenesis of lower airway disease in cystic fibrosis via both direct and indirect mechanisms. A personalized treatment strategy that maintains a normal microbial community may be possible in the future.

  19. Bilateral Cystic Lymphangioma of Ovary Associated with Chylous Ascites.

    PubMed

    Nerune, Savitri Mallikarjun; Arakeri, Surekha Ulhas; Patil, Vijaya L; Mulay, Himanshu Dilip

    2015-08-01

    Intraabdominal cystic lymphangiomas are rare and are located in retroperitoneum, mesentery, omentum and other visceral organs. Lymphangiomas of the ovary are rare and are usually unilateral. Cases with bilateral cystic lymphangiomas of the ovary are reported very rarely in literature. We report a rare case of bilateral cystic lymphangioma of ovary associated with chylous ascites in a 35-year-old lady who presented with complaints of severe dysmenorrhoea and oligomenorrhoea since 6 months with history of chyluria for the past 3 years.

  20. The value of plain abdominal radiographs in management of abdominal emergencies in Luth.

    PubMed

    Ashindoitiang, J A; Atoyebi, A O; Arogundade, R A

    2008-01-01

    The plain abdominal x-ray is still the first imaging modality in diagnosis of acute abdomen. The aim of this study was to find the value of plain abdominal x-ray in the management of abdominal emergencies seen in Lagos university teaching hospital. The accurate diagnosis of the cause of acute abdominal pain is one of the most challenging undertakings in emergency medicine. This is due to overlapping of clinical presentation and non-specific findings of physical and even laboratory data of the multifarious causes. Plain abdominal radiography is one investigation that can be obtained readily and within a short period of time to help the physician arrive at a correct diagnosis The relevance of plain abdominal radiography was therefore evaluated in the management of abdominal emergencies seen in Lagos over a 12 month period (April 2002 to March 2003). A prospective study of 100 consecutively presenting patients with acute abdominal conditions treated by the general surgical unit of Lagos University Teaching Hospital was undertaken. All patients had supine and erect abdominal x-ray before any therapeutic intervention was undertaken. The diagnostic features of the plain films were compared with final diagnosis to determine the usefulness of the plain x-ray There were 54 males and 46 females (M:F 1.2:1). Twenty-four percent of the patients had intestinal obstruction, 20% perforated typhoid enteritis; gunshot injuries and generalized peritonitis each occurred in 13%, blunt abdominal trauma in 12%, while 8% and 10% had acute appendicitis and perforated peptic ulcer disease respectively. Of 100 patients studied, 54% had plain abdominal radiographs that showed positive diagnostic features. Plain abdominal radiograph showed high sensitivity in patients with intestinal obstruction 100% and perforated peptic ulcer 90% but was less sensitive in patients with perforated typhoid, acute appendicitis, and blunt abdominal trauma and generalized peritonitis. In conclusion, this study

  1. Metabolomic responses to lumacaftor/ivacaftor in cystic fibrosis.

    PubMed

    Kopp, Benjamin T; McCulloch, Scott; Shrestha, Chandra L; Zhang, Shuzhong; Sarzynski, Lisa; Woodley, Frederick W; Hayes, Don

    2018-05-01

    Cystic fibrosis (CF) is a life-limiting disease caused by a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Lumacaftor/Ivacaftor is a novel CFTR modulator approved for patients that are homozygous for Phe508del CFTR, but its clinical effectiveness varies amongst patients, making it difficult to determine clinical responders. Therefore, identifying biochemical biomarkers associated with drug response are clinically important for follow-up studies. Serum metabolomics was performed on twenty patients with CF pre- and 6-month post-Lumacaftor/Ivacaftor response via Ultrahigh Performance Liquid Chromatography-Tandem Mass Spectroscopy (UPLC-MS/MS). Correlation with clinical variables was performed. Metabolomics analysis demonstrated 188 differentially regulated metabolites between patients pre- and post-Lumacaftor/Ivacaftor initiation, with a predominance of lipid and amino acid alterations. The top 30 metabolites were able to differentiate pre- and post-Lumacaftor/Ivacaftor status in greater than 90% of patients via a random-forest confusion matrix. Alterations in bile acids, phospholipids, and bacteria-associated metabolites were the predominant changes associated with drug response. Importantly, changes in metabolic patterns were associated with clinical responders. Selected key lipid and amino acid metabolic pathways were significantly affected by Lumacaftor/Ivacaftor initiation and similar pathways were affected in clinical responders. Targeted metabolomics may provide useful and relevant biomarkers of CFTR modulator responses. © 2018 Wiley Periodicals, Inc.

  2. Evaluation of the levels of metalloproteinsase-2 in patients with abdominal aneurysm and abdominal hernias.

    PubMed

    Antoszewska, Magdalena

    2013-05-01

    Abdominal aortic aneurysms and abdominal hernias become an important health problems of our times. Abdominal aortic aneurysm and its rupture is one of the most dangerous fact in vascular surgery. There are some theories pointing to a multifactoral genesis of these kinds of diseases, all of them assume the attenuation of abdominal fascia and abdominal aortic wall. The density and continuity of these structures depend on collagen and elastic fibers structure. Reducing the strength of the fibers may be due to changes in the extracellular matrix (ECM) by the proteolytic enzymes-matrix metalloproteinases (MMPs) that degrade extracellular matrix proteins. These enzymes play an important role in the development of many disease: malignant tumors (colon, breast, lung, pancreas), cardiovascular disease (myocardial infarction, ischemia-reperfusion injury), connective tissue diseases (Ehler-Danlos Syndrome, Marfan's Syndrome), complications of diabetes (retinopathy, nephropathy). One of the most important is matrix metalloproteinase-2 (MMP-2). The aim of the study was an estimation of the MMP-2 blood levels in patients with abdominal aortic aneurysm and primary abdominal hernia, and in patients with only abdominal aortic aneurysm. The study involved 88 patients aged 42 to 89 years, including 75 men and 13 women. Patients were divided into two groups: patients with abdominal aortic aneurysm and primary abdominal hernia (45 persons, representing 51.1% of all group) and patients with only abdominal aortic aneurysm (43 persons, representing 48,9% of all group). It was a statistically significant increase in MMP-2 blood levels in patients with abdominal aortic aneurysm and primary abdominal hernia compared to patients with only abdominal aortic aneurysm. It was a statistically significant increase in the prevalence of POCHP in patients with only abdominal aortic aneurysm compared to patients with abdominal aortic aneurysm and primary abdominal hernia. Statistically significant

  3. Frequent NFIB-associated Gene Rearrangement in Adenoid Cystic Carcinoma of the Vulva.

    PubMed

    Xing, Deyin; Bakhsh, Salwa; Melnyk, Nataliya; Isacson, Christina; Ho, Julie; Huntsman, David G; Gilks, C Blake; Ronnett, Brigitte M; Horlings, Hugo M

    2017-05-01

    Adenoid cystic carcinoma is a rare malignant tumor that usually arises in the major and minor salivary glands and other locations containing secretory glands, including the lower female genital tract. Lower female genital tract carcinomas with adenoid cystic differentiation can be subclassified into 2 distinct groups based on the presence or absence of high-risk HPV. Cervical mixed carcinomas with some adenoid cystic differentiation are high-risk HPV-related but pure adenoid cystic carcinomas of vulvar and cervical origin appear to be unrelated to high-risk HPV. Mechanisms by which normal cells give rise to an HPV-unrelated adenoid cystic carcinoma remain largely unknown. Studies demonstrate that chromosomal translocation involving the genes encoding the transcription factors MYB and NFIB functions as a driving force of adenoid cystic carcinomas development regardless of anatomic site. The current study used fluorescence in situ hybridization with 3 different probes including MYB break-apart probe, NFIB break-apart probe, and MYB-NFIB fusion probe to assess for the presence of gene rearrangements in adenoid cystic carcinomas of the vulva. Six (66.7%) of 9 vulvar adenoid cystic carcinomas demonstrated NFIB rearrangement. Of these 6 cases with a disturbed NFIB, only 2 cases (33.3%) were positive for a MYB rearrangement that was also confirmed by a positive MYB-NFIB fusion pattern. NFIB-associated gene rearrangement is a frequent genetic event in vulvar adenoid cystic carcinomas. Chromosome translocations involving NFIB but with an intact MYB indicate the presence of novel oncogenic mechanisms for the development of adenoid cystic carcinomas of the vulva.

  4. Drug therapies for reducing gastric acidity in people with cystic fibrosis.

    PubMed

    Ng, Sze May; Franchini, Angelo J

    2014-07-13

    Malabsorption of fat and protein contributes to poor nutritional status in people with cystic fibrosis. Impaired pancreatic function may also result in increased gastric acidity, leading in turn to heartburn, peptic ulcers and the impairment of oral pancreatic enzyme replacement therapy. The administration of gastric acid-reducing agents has been used as an adjunct to pancreatic enzyme therapy to improve absorption of fat and gastro-intestinal symptoms in people with cystic fibrosis. It is important to establish the evidence regarding potential benefits of drugs that reduce gastric acidity in people with cystic fibrosis. To assess the effect of drug therapies for reducing gastric acidity for: nutritional status; symptoms associated with increased gastric acidity; fat absorption; lung function; quality of life and survival; and to determine if any adverse effects are associated with their use. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches, handsearches of relevant journals, abstract books and conference proceedings.Most recent search of the Group's Trials Register: 17 March 2014. All randomised and quasi-randomised trials involving agents that reduce gastric acidity compared to placebo or a comparator treatment. Both authors independently selected trials, assessed trial quality and extracted data. The searches identified 39 trials; 17 of these, with 273 participants, were suitable for inclusion, but the number of trials assessing each of the different agents was small. Seven trials were limited to children and four trials enrolled only adults. Meta-analysis was not performed, 14 trials were of a cross-over design and we did not have the appropriate information to conduct comprehensive meta-analyses. The included trials were generally not reported adequately enough to allow judgements on risk of bias.However, one trial found that drug therapies

  5. Effect of supplementary zinc on body mass index, pulmonary function and hospitalization in children with cystic fibrosis.

    PubMed

    Ataee, Pedram; Najafi, Mehri; Gharagozlou, Mohammad; Aflatounian, Majid; Mahmoudi, Maryam; Khodadad, Ahmad; Farahmand, Fatemeh; Motamed, Farzaneh; Fallahi, Glolam Hossein; Kalantari, Najmoddin; Soheili, Habib; Modarresi, Vajiheh; Modarresi, Mozhgan Sabbaghian; Rezaei, Nima

    2014-01-01

    Zinc deficiency, which is common in patients with cystic fibrosis (CF), can lead to several complications that may increase the number of hospital admissions in this group of patients. As supplementary zinc can prevent such complications, this study was performed to evaluate the effect of supplementary zinc on body mass index (BMI), forced expiratory volume in one second (FEV1) and number of hospitalizations in CF patients. In this study, 30 children with CF, who were referred to the Digestive Diseases Clinic of the Children's Medical Center in Tehran, were enrolled. Supplementary zinc of 2 mg/kg per day was administered to all patients. Serum level of zinc, alkaline phosphatase, and albumin as well as BMI, FEV1, and number of hospitalizations were compared before and after zinc administration. Height (p<0.001), weight (p<0.001) and BMI (p=0.001) were significantly increased after zinc, while the number of hospitalizations was significantly decreased (p=0.023). In contrast to patients with normal pulmonary function tests who received supplement therapy, BMI was not increased in those with abnormal pulmonary function after supplementary zinc. Supplementary zinc can increase BMI in CF patients, mostly in those with normal pulmonary function. While supplementary zinc may decrease the number of hospitalizations, other factors can also influence the hospitalization number.

  6. Festival food coma in cystic fibrosis.

    PubMed

    Pandit, Chetan; Graham, Christie; Selvadurai, Hiran; Gaskin, Kevin; Cooper, Peter; van Asperen, Peter

    2013-07-01

    Children with cystic fibrosis liver disease and portal hypertension are at risk of developing acute hepatic encephalopathy. Even in the presence of normal synthetic liver function these children may have porto-systemic shunting. We report a case of an adolosecent who had cystic fibrosis liver disease and presented with life threatening hepatinc encephalopathy. This case illustrates that it is necessary to consider an appropriate dietary regimen in adolosecents with liver disease to prevent hepatic decompensation. Copyright © 2012 Wiley Periodicals, Inc.

  7. [A commonly seen cause of abdominal pain: abdominal cutaneous nerve entrapment syndrome].

    PubMed

    Solmaz, Ilker; Talay, Mustafa; Tekindur, Şükrü; Kurt, Ercan

    2012-01-01

    Although abdominal cutaneous nerve entrapment syndrome (ACNES) is accepted as a rare condition, it is a syndrome that should be diagnosed more commonly when the clinical signs cannot explain the cause of abdominal pain. Abdominal pain is commonly considered by physicians to be based on intra-abdominal causes. Consequently, redundant tests and consultations are requested for these patients, and unnecessary surgical procedures may be applied. Patients with this type of pain are consulted to many clinics, and because their definitive diagnoses cannot be achieved, they are assessed as psychiatric patients. Actually, a common cause of abdominal wall pain is nerve entrapment on the lateral edge of the rectus abdominis muscle. In this paper, we would like to share information about the diagnosis and treatment of a patient who, prior to presenting to us, had applied to different clinics for chronic abdominal pain and had undergone many tests and consultations; abdominal surgery was eventually decided.

  8. Prevalence of abdominal obesity in Abia State, Nigeria: results of a population-based house-to-house survey.

    PubMed

    Chukwuonye, Innocent Ijezie; Chuku, Abali; Onyeonoro, Ugochukwu Uchenna; Okpechi, Ikechi Gareth; Madukwe, Okechukwu Ojoemelam; Umeizudike, Theophilus Ifeanyichukwu; Ogah, Okechukwu Samuel

    2013-01-01

    Abdominal obesity is associated with the risk of developing disorders, such as diabetes and hypertension. The objective of this study was to investigate the prevalence of abdominal obesity in Abia State, Nigeria. We carried out a cross-sectional study aimed at ascertaining the prevalence of abdominal obesity in Abia State, Nigeria. Participants in the study were recruited from communities in the three senatorial zones in the state. Screening for abdominal obesity was carried out in these subjects using waist circumference (the National Cholesterol Education Program Third Adult Treatment Panel criteria were used). The World Health Organization Stepwise Approach to Surveillance of chronic disease risk factors was used. Body mass index, anthropometric measurements, and other relevant data were also collected. Data on waist circumference were obtained from 2,807 subjects. The prevalence of obesity using body mass index in the population was 11.12%. In men and women, it was 7.73%, and 14.37%, respectively. The prevalence of abdominal obesity in the population was 21.75%. In men and women, it was 3.2% and 39.2%, respectively. The prevalence of abdominal obesity is high in Nigeria, and needs to be monitored because it is associated with increased cardiovascular risk.

  9. Prevalence of abdominal obesity in Abia State, Nigeria: results of a population-based house-to-house survey

    PubMed Central

    Ijezie, Innocent Chukwuonye; Chuku, Abali; Onyeonoro, Ugochukwu Uchenna; Okpechi, Ikechi Gareth; Madukwe, Okechukwu Ojoemelam; Umeizudike, Theophilus Ifeanyichukwu; Ogah, Okechukwu Samuel

    2013-01-01

    Background and objective Abdominal obesity is associated with the risk of developing disorders, such as diabetes and hypertension. The objective of this study was to investigate the prevalence of abdominal obesity in Abia State, Nigeria. Materials and methods We carried out a cross-sectional study aimed at ascertaining the prevalence of abdominal obesity in Abia State, Nigeria. Participants in the study were recruited from communities in the three senatorial zones in the state. Screening for abdominal obesity was carried out in these subjects using waist circumference (the National Cholesterol Education Program Third Adult Treatment Panel criteria were used). The World Health Organization Stepwise Approach to Surveillance of chronic disease risk factors was used. Body mass index, anthropometric measurements, and other relevant data were also collected. Results Data on waist circumference were obtained from 2,807 subjects. The prevalence of obesity using body mass index in the population was 11.12%. In men and women, it was 7.73%, and 14.37%, respectively. The prevalence of abdominal obesity in the population was 21.75%. In men and women, it was 3.2% and 39.2%, respectively. Conclusion The prevalence of abdominal obesity is high in Nigeria, and needs to be monitored because it is associated with increased cardiovascular risk. PMID:23946664

  10. Anthropometry of height, weight, arm, wrist, abdominal circumference and body mass index, for Bolivian adolescents 12 to 18 years: Bolivian adolescent percentile values from the MESA study.

    PubMed

    Baya Botti, A; Pérez-Cueto, F J A; Vasquez Monllor, P A; Kolsteren, P W

    2009-01-01

    Anthropometry is important as clinical tool for individual follow-up as well as for planning and health policy-making at population level. Recent references of Bolivian Adolescents are not available. The aim of this cross sectional study was to provide age and sex specific centile values and charts of Body Mass Index, height, weight, arm, wrist and abdominal circumference from Bolivian Adolescents. Data from the MEtabolic Syndrome in Adolescents (MESA) study was used. Thirty-two Bolivian clusters from urban and rural areas were selected randomly considering population proportions, 3445 school going adolescents, 12 to 18 y, 45% males; 55% females underwent anthropometric evaluation by trained personnel using standardized protocols for all interviews and examinations. Weight, height, wrist, arm and abdominal circumference data were collected. Body Mass Index was calculated. Smoothed age- and gender specific 3rd, 5th, 10th, 25th, 50th, 75th, 85th, 90th, 95th and 97th Bolivian adolescent percentiles(BAP) and Charts(BAC) where derived using LMS regression. Percentile-based reference data for the antropometrics of for Bolivian Adolescents are presented for the first time.

  11. Autonomously hyperfunctioning cystic nodule harbouring thyroid carcinoma - Case report and literature review.

    PubMed

    Lima, Maria João; Soares, Virgínia; Koch, Pedro; Silva, Artur; Taveira-Gomes, António

    2018-01-01

    Hyperthyroidism is rarely associated with malignancy, but it cannot rule out thyroid cancer. Although there is published data describing this coexistence, thyroid carcinomas inside autonomously functioning nodules are uncommon. A 49-year-old woman presented with a cervical mass, unexplained weight loss and anxiousness, sweating and insomnia. On physical examination, she had a palpable left thyroid nodule. Thyroid function tests showed suppressed TSH (<0,1 uUI/mL), thyroxine 1,44 ng/dL (normal range 0,70-1,48) and triiodothyronine 4,33 pg/mL (normal range 1,71-3,71). Ultrasound imaging revealed a left lobe, 4 cm partial cystic nodule. 99mTC thyroid scintigraphy showed a hyperfunctioning nodule with suppression of the remainder parenchyma. Fine-needle aspiration cytology was nondiagnostic (cystic fluid). The patient was started on thiamazole 5 mg daily with subsequent normalization of thyroid function, but she developed cervical foreign body sensation and a left hemithyroidectomy was performed. Histology showed a 4 cm cystic nodule with a follicular variant papillary carcinoma and the patient underwent completion thyroidectomy, followed by radio-iodine ablation. Published literature showed an increased prevalence of autonomously functioning nodules, harbouring thyroid carcinomas in adults. Papillary carcinoma is the most frequently described but the follicular variant is rare. Although rare, thyroid cancer is not definitively excluded in hyperthyroid patients and it should always be considered as differential diagnosis. Copyright © 2018. Published by Elsevier Ltd.

  12. Vorinostat in Treating Patients With Locally Advanced, Recurrent, or Metastatic Adenoid Cystic Carcinoma

    ClinicalTrials.gov

    2018-05-23

    Recurrent Oral Cavity Adenoid Cystic Carcinoma; Recurrent Salivary Gland Carcinoma; Salivary Gland Adenoid Cystic Carcinoma; Stage III Major Salivary Gland Cancer AJCC v7; Stage III Oral Cavity Adenoid Cystic Carcinoma AJCC v6 and v7; Stage IVA Major Salivary Gland Cancer AJCC v7; Stage IVA Oral Cavity Adenoid Cystic Carcinoma AJCC v6 and v7; Stage IVB Major Salivary Gland Cancer AJCC v7; Stage IVB Oral Cavity Adenoid Cystic Carcinoma AJCC v6 and v7; Stage IVC Major Salivary Gland Cancer AJCC v7; Stage IVC Oral Cavity Adenoid Cystic Carcinoma AJCC v6 and v7; Tongue Carcinoma

  13. Bilateral Cystic Lymphangioma of Ovary Associated with Chylous Ascites

    PubMed Central

    Arakeri, Surekha Ulhas; Patil, Vijaya L.; Mulay, Himanshu Dilip

    2015-01-01

    Intraabdominal cystic lymphangiomas are rare and are located in retroperitoneum, mesentery, omentum and other visceral organs. Lymphangiomas of the ovary are rare and are usually unilateral. Cases with bilateral cystic lymphangiomas of the ovary are reported very rarely in literature. We report a rare case of bilateral cystic lymphangioma of ovary associated with chylous ascites in a 35-year-old lady who presented with complaints of severe dysmenorrhoea and oligomenorrhoea since 6 months with history of chyluria for the past 3 years. PMID:26435959

  14. Cystic brain metastasis is associated with poor prognosis in patients with advanced breast cancer.

    PubMed

    Sun, Bing; Huang, Zhou; Wu, Shikai; Ding, Lijuan; Shen, Ge; Cha, Lei; Wang, Junliang; Song, Santai

    2016-11-08

    Brain metastasis (BM) with a cystic component from breast cancer is rare and largely uncharacterized. The purpose of this study was to identify the characteristics of cystic BM in a large cohort of breast cancer patients. A total of 35 eligible patients with cystic BM and 255 patients with solid BM were analyzed. Three factors were significantly associated with an increased probability of developing cystic lesions: age at diagnosis ≤ 40 years, age at BM ≤ 45 years, and poor histological grade (p < 0.05). Patients with cystic metastasis were also characterized by a larger metastasis volume, a shorter progression-free survival (PFS) following their first treatment for BM, and poor overall survival after BM (p < 0.05). Multivariate analysis further demonstrated that local control of cystic BM was only potentially achieved for HER2-negative primary tumors (p = 0.084). Breast cancer patients with parenchymal BM were reviewed from consecutive cases treated at our institution. Cystic BM was defined when the volume of a cystic lesion was greater than 50% of the aggregated volume of all lesions present. Clinicopathologic and radiographic variables were correlated with development of cystic lesions and with prognosis of cystic BM. This study shows that cystic BM from breast cancer, a special morphological type of BM, had worse prognosis than the more commonly observed solid BM. Younger age and low tumor grade were associated with the development of cystic lesions. Further comprehensive research and management of cystic BM are warranted to improve its poor prognosis.

  15. Unusual case of iron overload with cancer-mimicking abdominal splenosis.

    PubMed

    Marchi, Giacomo; Avesani, Giacomo; Zamò, Alberto; Girelli, Domenico

    2018-05-16

    A 48-year-old man, former alcohol abuser and drug addicted, was referred to our tertiary referral centre for iron disorders because of marked hyperferritinaemia. His clinical history revealed chronic hepatitis C, ß-thalassaemia trait and post-traumatic splenectomy at age of 22. MRI-estimated liver iron content was markedly elevated, while first-line genetic test for haemochromatosis was negative. Alpha-fetoprotein was increased but liver ultrasonography did not reveal focal liver lesions. Multiphasic contrast-enhanced CT confirmed this result but showed two abdominal masses (diameter of 9 cm and 7 cm, respectively) among bowel loops, strongly suspicious for cancer. However, biopsy of one of the masses led to the final diagnosis of abdominal splenosis. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  16. Factors Predictive of Improved Abdominal Ultrasound Visualization after Oral Administration of Simethicone.

    PubMed

    Marsico, Maria; Gabbani, Tommaso; Casseri, Tommaso; Biagini, Maria Rosa

    2016-11-01

    Ultrasonography is a non-invasive, accurate and low-cost technique used to study the upper abdomen, but it has reduced reliability in the study of the pancreas and retroperitoneum. Simethicone is a well-known emulsifying agent that has been used to improve ultrasonographic visualization. The aim of this study was to identify anthropometric parameters that are able to predict a good response to simethicone in improving ultrasonographic visualization of abdominal structures. One hundred twenty-seven patients were recruited. After basal examination, their anthropometric parameters were collected. Patients with an incomplete upper abdominal examination because of gastrointestinal gas have greater body mass index, waist circumference and abdominal wall thickness. In our study, the best anthropometric parameter for identifying patients with poor visualization at abdominal ultrasound examination is waist circumference. Using a cutoff of 84 cm, we can identify patients with poor visibility at abdominal ultrasound examination (group B) with a sensitivity of 90%. Copyright © 2016 World Federation for Ultrasound in Medicine & Biology. Published by Elsevier Inc. All rights reserved.

  17. Therapies for Cystic Fibrosis

    MedlinePlus

    ... Testing for Cystic Fibrosis CFTR-Related Metabolic Syndrome (CRMS) How Babies Are Screened in IRT-Only vs. ... Guidelines Infant Care Clinical Care Guidelines Management of CRMS in First 2 Years and Beyond Clinical Care ...

  18. Renal Complex Cystic Masses: Usefulness of Contrast-Enhanced Ultrasound (CEUS) in Their Assessment and Its Agreement with Computed Tomography.

    PubMed

    Sanz, Enrique; Hevia, Vital; Gómez, Victoria; Álvarez, Sara; Fabuel, Jose-Javier; Martínez, Laura; Rodriguez-Patrón, Rafael; González-Gordaliza, Cristina; Burgos, Francisco-Javier

    2016-12-01

    Around 10 % of renal cell carcinomas (RCC) are cystic, while some benign cysts have complex appearance in conventional diagnostic tests such as computed tomography (CT) or magnetic resonance imaging (MRI). These renal complex cystic masses (RCCMs) are a challenging entity in urological practice and sometimes have a difficult management, requiring surgical removal. Contrast-enhanced ultrasound (CEUS) is a very sensitive test detecting microvascularization in real time, and it has been used in the diagnostic workup of these kinds of lesions. The aim of our study was to assess the diagnostic power of CEUS in the evaluation of RCCM. This is a prospective observational study between April 2011 and July 2014. A total of 66 patients with 67 RCCMs were enrolled (Bosniak 2-4). Twenty-four patients underwent surgical removal of the RCCM. All participants underwent CEUS (experimental) and CT (control). All CEUS procedures were performed by a single high-experienced observer (urologist). Benign lesions were defined as those Bosniak 2-2F, and malignant were Bosniak 3-4. Statistical analysis was made measuring consistency (kappa index and Landis-Koch scale) and validity (sensitivity, specificity, positive and negative predictive values) of the study. Median size of RCCM measured by CEUS and CT was 3.8 cm (interquartile range (AIQ) 3.2-4.6) and 3.9 cm (AIQ 3.2-4.5), respectively. Kappa index shows good agreement between both tests (0.71; 95 % CI 0.57-0.85), both overall and stratified by categories according to Bosniak classification. CEUS has a sensitivity 100 %, specificity 81.4 %, positive predictive value 70.4 %, and negative predictive value 100 %. A total of eight RCCMs were discordant, and seven of eight classified as malignant by CEUS and not by CT. Of those seven lesions classified as malignant by CEUS, six (six of seven, 85.7 %) were malignant in the pathological exam. CEUS is a very useful tool for assessing RCCM, with good results in terms of consistency

  19. Genetic counselling issues in cystic fibrosis.

    PubMed

    Culling, Bronwyn; Ogle, Robert

    2010-06-01

    Cystic fibrosis is a chronic condition for which genetic testing offers much for the individuals affected in terms of an early diagnosis and offers timely additional information for families with regard to family planning and prenatal testing. Genetic counselling encompasses a range of clinical issues for families and forms a complementary resource for clinicians caring for people with cystic fibrosis. This review will discuss the range of genetic information readily available to patients and families through genetic counselling. Copyright 2010 Elsevier Ltd. All rights reserved.

  20. Cystic fibrosis carrier screening in a North American population.

    PubMed

    Zvereff, Val V; Faruki, Hawazin; Edwards, Marcia; Friedman, Kenneth J

    2014-07-01

    The aim of this study was to compare the mutation frequency distribution for a 32-mutation panel and a 69-mutation panel used for cystic fibrosis carrier screening. Further aims of the study were to examine the race-specific detection rates provided by both panels and to assess the performance of extended panels in large-scale, population-based cystic fibrosis carrier screening. Although genetic screening for the most common CFTR mutations allows detection of nearly 90% of cystic fibrosis carriers, the large number of other mutations, and their distribution within different ethnic groups, limits the utility of general population screening. Patients referred for cystic fibrosis screening from January 2005 through December 2010 were tested using either a 32-mutation panel (n = 1,601,308 individuals) or a 69-mutation panel (n = 109,830). The carrier frequencies observed for the 69-mutation panel study population (1/36) and Caucasian (1/27) and African-American individuals (1/79) agree well with published cystic fibrosis carrier frequencies; however, a higher carrier frequency was observed for Hispanic-American individuals (1/48) using the 69-mutation panel as compared with the 32-mutation panel (1/69). The 69-mutation panel detected ~20% more mutations than the 32-mutation panel for both African-American and Hispanic-American individuals. Expanded panels using race-specific variants can improve cystic fibrosis carrier detection rates within specific populations. However, it is important that the pathogenicity and the relative frequency of these variants are confirmed.

  1. Abdominal obesity, ethnicity and gastro‐oesophageal reflux symptoms

    PubMed Central

    Corley, Douglas A; Kubo, Ai; Zhao, Wei

    2007-01-01

    Objective To evaluate the associations between abdominal obesity and gastro‐oesophageal reflux disease (GORD), and their interactions with ethnicity and gender. Design A cross‐sectional study. Participants completed detailed symptom questionnaires and underwent a standardised examination, including anthropometric measurements. Setting A large integrated healthcare system. Patients 80 110 members of the Kaiser Permanente multiphasic health check‐up cohort. Main outcome measures Gastro‐oesophageal reflux‐type symptoms. Results Recent reflux‐type symptoms were present in 11% of the population. The multivariate OR for symptoms with an abdominal diameter (adjusted for body mass index (BMI)) of ⩾26 vs <16.3 cm was 1.85 (95% CI 1.55 to 2.21) for the white population, 0.95 (95% CI 0.61 to 1.48) for the black population and 0.64 (95% CI 0.18 to 2.30) for Asians. The mean abdominal diameter was greater in men (22.0 cm, 95% CI 21.9 to 22.0) than in women (20.1 cm, 95% CI 20.0 to 20.1, p<0.01), but the risk of symptoms for any given diameter did not differ markedly by gender. The association between increasing BMI and symptoms was also much stronger among the white population than among the black population. The association between BMI and reflux‐type symptoms was partially mediated through abdominal diameter. Conclusions There was a consistent association between abdominal diameter (independent of BMI) and reflux‐type symptoms in the white population, but no consistent associations in the black population or Asians. The BMI association was also strongest among the white population. These findings, combined with the increased prevalence of abdominal obesity in male subjects, suggest that an increased obesity may disproportionately increase GORD‐type symptoms in the white population and in male subjects. PMID:17047097

  2. Focused abdominal sonography for trauma (FAST) in blunt paediatric abdominal trauma.

    PubMed

    Faruque, Ahmad Vaqas; Qazi, Saqib Hamid; Khan, Muhammad Arif Mateen; Akhtar, Wassem; Majeed, Amina

    2013-03-01

    To evaluate the role of focussed abdominal sonography for trauma in blunt paediatric abdominal trauma patients, and to see if the role of computed tomography scan could be limited to only those cases in which sonography was positive. The retrospective study covered 10 years, from January 1,2000 to December 31,2009, and was conducted at the Department of Radiology and Department of Emergency Medicine, Aga Khan University Hospital, Karachi. It comprised cases of 174 children from birth to 14 years who had presented with blunt abdominal trauma and had focussed abdominal sonography for trauma done at the hospital. The findings were correlated with computed tomography scan of the abdomen and clinical follow-up. Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of focussed abdominal sonography for trauma were calculated for blunt abdominal trauma. SPSS 17 was used for statistical analysis. Of the total 174 cases, 31 (17.81%) were later confirmed by abdominal scan. Of these 31 children, sonography had been positive in 29 (93.54%) children. In 21 (67.74%) of the 31 children, sonograpy had been true positive; 8 (25%) (8/31) were false positive; and 2 (6%) (2/31) were false negative. There were 6 (19.3%) children in which sonography was positive and converted to laparotomy. There was no significant difference on account of gender (p>0.356). Focussed abdominal sonography for trauma in the study had sensitivity of 91%, specificity of 95%, positive predictive value of 73%, and negative predictive value of 73% with accuracy of 94%. All patients who had negative sonography were discharged later, and had no complication on clinical follow-up. Focussed abdominal sonography for trauma is a fairly reliable mode to assess blunt abdominal trauma in children. It is a useful tool to pick high-grade solid and hollow viscous injury. The results suggest that the role of computed tomography scan can be limited to those cases in which focussed

  3. CT of abdominal tuberculosis

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Epstein, B.M.; Mann, J.H.

    1982-11-01

    Intraabdominal tuberculosis (TB) presents with a wide variety of clinical and radiologic features. Besides the reported computed tomographic (CT) finding of high-density ascites in tuberculous peritonitis, this report describes additional CT features highly suggestive of abdominal tuberculosis in eight cases: (1) irregular soft-tissue densities in the omental area; (2) low-density masses surrounded by thick solid rims; (3) a disorganized appearance of soft-tissue densities, fluid, and bowel loops forming a poorly defined mass; (4) low-density lymph nodes with a multilocular appearance after intravenous contrast administration; and (5) possibly high-density ascites. The differential diagnosis of these features include lymphoma, various forms ofmore » peritonitis, peritoneal carcinomatosis, and peritoneal mesothelioma. It is important that the CT features of intraabdominal tuberculosis be recognized in order that laparotomy be avoided and less invasive procedures (e.g., laparoscopy, biopsy, or a trial of antituberculous therapy) be instituted.« less

  4. Vitamin D supplementation for cystic fibrosis.

    PubMed

    Ferguson, Janet H; Chang, Anne B

    2014-05-14

    Cystic fibrosis (CF) is a genetic disorder with multiorgan effects. In a subgroup with pancreatic insufficiency malabsorption of the fat soluble vitamins (A, D, E, K) may occur. Vitamin D is involved in calcium homeostasis and bone mineralisation and may have extraskeletal effects. This review examines the evidence for vitamin D supplementation in cystic fibrosis. To assess the effects of vitamin D supplementation on the frequency of vitamin D deficiency, respiratory outcomes and vitamin D toxicity in the cystic fibrosis population. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 08 July 2013. Randomised and quasi-randomised controlled studies of vitamin D supplementation compared to placebo in the cystic fibrosis population regardless of exocrine pancreatic function. Both authors independently assessed the risk of bias of each included study and extracted outcome data (from published study information) for assessment of bone mineralization, growth and nutritional status, frequency of vitamin D deficiency, respiratory status, quality of life and adverse events. Six studies (239 participants) are included, although only three studies provided data from 69 adults and children with cystic fibrosis for analysis. One study compared a single high dose of vitamin D (250,000 IU) to placebo at the time of hospital admission with a respiratory exacerbation in 30 pancreatic insufficient adults with cystic fibrosis. The second study compared supplemental 800 international units (IU) vitamin D and placebo for 12 months in 30 osteopenic pancreatic insufficient adults; both groups continued 900 IU vitamin D daily. The third study compared supplemental 1 g calcium alone, 1600 IU vitamin D alone, 1600 IU vitamin D and 1 g calcium and placebo in a double

  5. Dynamics of albumin synthetic response to intra-abdominal abscess in patients with gastrointestinal fistula.

    PubMed

    Zhou, Bo; Ren, Jianan; Han, Gang; Chen, Yu; A, Jiye; Gu, Guosheng; Chen, Jun; Wang, Gefei; Li, Jieshou

    2014-04-01

    Low serum albumin concentration is a predictor of failure of source control for intra-abdominal infection. However, data on dynamics of albumin synthesis in these patients and to what extent these changes contribute to hypoalbuminemia are relatively scarce. We investigated in a group of patients with gastrointestinal fistula the dynamic response of liver albumin synthesis to intra-abdominal abscess and how these related to hypoalbuminemia and circulating endocrine hormone profiles. Eight gastrointestinal fistula patients scheduled to undergo percutaneous abscess sump drainage were enrolled prospectively to measure albumin synthesis rates at different stages of the inflammatory response (immediately after diagnosis and 7 d following sump drainage when clinical signs of intra-abdominal sepsis had been eradicated). Eight age-, sex-, and body mass index-matched intestinal fistula patients were studied as control patients. Consecutive arterial blood samples were drawn during a primed-constant infusion (priming dose: 4 micromol·kg(-1), infusion rate: 6 micromol·kg(-1)·min(-1)) to determine the incorporation rate of L-[ring-(2)H5]-phenylalanine directly into plasma albumin using gas chromatography/mass spectrometry analysis. Patients suffering from intra-abdominal infection had reduced plasma albumin and total plasma protein concentrations, compared with control patients. Albumin fractional synthesis rates in patients with intra-abdominal abscess were decreased, compared with those in the control group. When the source of infection was removed, albumin synthesis rates returned to control values, whereas albumin concentrations did not differ significantly from the corresponding concentrations in control subjects and patients with intra-abdominal abscess. Despite nutritional intervention, albumin synthesis rate is decreased in intestinal fistula patients with intra-abdominal abscess; albumin synthesis returns to control values during convalescence.

  6. Dynamics of Albumin Synthetic Response to Intra-Abdominal Abscess in Patients with Gastrointestinal Fistula

    PubMed Central

    Zhou, Bo; Han, Gang; Chen, Yu; A, Jiye; Gu, Guosheng; Chen, Jun; Wang, Gefei; Li, Jieshou

    2014-01-01

    Abstract Background: Low serum albumin concentration is a predictor of failure of source control for intra-abdominal infection. However, data on dynamics of albumin synthesis in these patients and to what extent these changes contribute to hypoalbuminemia are relatively scarce. We investigated in a group of patients with gastrointestinal fistula the dynamic response of liver albumin synthesis to intra-abdominal abscess and how these related to hypoalbuminemia and circulating endocrine hormone profiles. Methods: Eight gastrointestinal fistula patients scheduled to undergo percutaneous abscess sump drainage were enrolled prospectively to measure albumin synthesis rates at different stages of the inflammatory response (immediately after diagnosis and 7 d following sump drainage when clinical signs of intra-abdominal sepsis had been eradicated). Eight age-, sex-, and body mass index–matched intestinal fistula patients were studied as control patients. Consecutive arterial blood samples were drawn during a primed-constant infusion (priming dose: 4 micromol·kg−1, infusion rate: 6 micromol·kg−1·min−1) to determine the incorporation rate of L-[ring-2H5]-phenylalanine directly into plasma albumin using gas chromatography/mass spectrometry analysis. Results: Patients suffering from intra-abdominal infection had reduced plasma albumin and total plasma protein concentrations, compared with control patients. Albumin fractional synthesis rates in patients with intra-abdominal abscess were decreased, compared with those in the control group. When the source of infection was removed, albumin synthesis rates returned to control values, whereas albumin concentrations did not differ significantly from the corresponding concentrations in control subjects and patients with intra-abdominal abscess. Conclusion: Despite nutritional intervention, albumin synthesis rate is decreased in intestinal fistula patients with intra-abdominal abscess; albumin synthesis returns to

  7. Giant hydronephrosis mimicking progressive malignancy

    PubMed Central

    Schrader, Andres Jan; Anderer, Georgia; von Knobloch, Rolf; Heidenreich, Axel; Hofmann, Rainer

    2003-01-01

    Background Cases of giant hydronephroses are rare and usually contain no more than 1–2 litres of fluid in the collecting system. We report a remarkable case of giant hydronephrosis mimicking a progressive malignant abdominal tumour. Case presentation A 78-year-old cachectic woman presented with an enormous abdominal tumour, which, according to the patient, had slowly increased in diameter. Medical history was unremarkable except for a hysterectomy >30 years before. A CT scan revealed a giant cystic tumour filling almost the entire abdominal cavity. It was analysed by two independent radiologists who suspected a tumour originating from the right kidney and additionally a cystic ovarian neoplasm. Subsequently, a diagnostic and therapeutic laparotomy was performed: the tumour presented as a cystic, 35 × 30 × 25 cm expansive structure adhesive to adjacent organs without definite signs of invasive growth. The right renal hilar vessels could finally be identified at its basis. After extirpation another tumourous structure emerged in the pelvis originating from the genital organs and was also resected. The histopathological examination revealed a >15 kg hydronephrotic right kidney, lacking hardly any residual renal cortex parenchyma. The second specimen was identified as an ovary with regressive changes and a large partially calcified cyst. There was no evidence of malignant growth. Conclusion Although both clinical symptoms and the enormous size of the tumour indicated malignant growth, it turned out to be a giant hydronephrosis. Presumably, a chronic obstruction of the distal ureter had caused this extraordinary hydronephrosis. As demonstrated in our case, an accurate diagnosis of giant hydronephrosis remains challenging due to the atrophy of the renal parenchyma associated with chronic obstruction. Therefore, any abdominal cystic mass even in the absence of other evident pathologies should include the differential diagnosis of a possible hydronephrosis. Diagnostic

  8. Mucous solids and liquid secretion by airways: studies with normal pig, cystic fibrosis human, and non-cystic fibrosis human bronchi

    PubMed Central

    Martens, Chelsea J.; Inglis, Sarah K.; Valentine, Vincent G.; Garrison, Jennifer; Conner, Gregory E.

    2011-01-01

    To better understand how airways produce thick airway mucus, nonvolatile solids were measured in liquid secreted by bronchi from normal pig, cystic fibrosis (CF) human, and non-CF human lungs. Bronchi were exposed to various secretagogues and anion secretion inhibitors to induce a range of liquid volume secretion rates. In all three groups, the relationship of solids concentration (percent nonvolatile solids) to liquid volume secretion rate was curvilinear, with higher solids concentration associated with lower rates of liquid volume secretion. In contrast, the secretion rates of solids mass and water mass as functions of liquid volume secretion rates exhibited positive linear correlations. The y-intercepts of the solids mass-liquid volume secretion relationships for all three groups were positive, thus accounting for the higher solids concentrations in airway liquid at low rates of secretion. Predictive models derived from the solids mass and water mass linear equations fit the experimental percent solids data for the three groups. The ratio of solids mass secretion to liquid volume secretion was 5.2 and 2.4 times higher for CF bronchi than for pig and non-CF bronchi, respectively. These results indicate that normal pig, non-CF human, and CF human bronchi produce a high-percent-solids mucus (>8%) at low rates of liquid volume secretion (≤1.0 μl·cm−2·h−1). However, CF bronchi produce mucus with twice the percent solids (∼8%) of pig or non-CF human bronchi at liquid volume secretion rates ≥4.0 μl·cm−2·h−1. PMID:21622844

  9. Next-generation technologies for spatial proteomics: Integrating ultra-high speed MALDI-TOF and high mass resolution MALDI FTICR imaging mass spectrometry for protein analysis.

    PubMed

    Spraggins, Jeffrey M; Rizzo, David G; Moore, Jessica L; Noto, Michael J; Skaar, Eric P; Caprioli, Richard M

    2016-06-01

    MALDI imaging mass spectrometry is a powerful analytical tool enabling the visualization of biomolecules in tissue. However, there are unique challenges associated with protein imaging experiments including the need for higher spatial resolution capabilities, improved image acquisition rates, and better molecular specificity. Here we demonstrate the capabilities of ultra-high speed MALDI-TOF and high mass resolution MALDI FTICR IMS platforms as they relate to these challenges. High spatial resolution MALDI-TOF protein images of rat brain tissue and cystic fibrosis lung tissue were acquired at image acquisition rates >25 pixels/s. Structures as small as 50 μm were spatially resolved and proteins associated with host immune response were observed in cystic fibrosis lung tissue. Ultra-high speed MALDI-TOF enables unique applications including megapixel molecular imaging as demonstrated for lipid analysis of cystic fibrosis lung tissue. Additionally, imaging experiments using MALDI FTICR IMS were shown to produce data with high mass accuracy (<5 ppm) and resolving power (∼75 000 at m/z 5000) for proteins up to ∼20 kDa. Analysis of clear cell renal cell carcinoma using MALDI FTICR IMS identified specific proteins localized to healthy tissue regions, within the tumor, and also in areas of increased vascularization around the tumor. © 2016 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  10. Pancreatic enzyme replacement therapy for people with cystic fibrosis.

    PubMed

    Somaraju, Usha Rani; Solis-Moya, Arturo

    2016-11-23

    parallel trial and 12 cross-over trials of children and adults with cystic fibrosis were included in the review. The number of participants in each trial varied between 14 and 129 with a total of 512 participants included in the review. All the included trials were for a duration of four weeks. The included trials had mostly an unclear risk of bias from the randomisation process as the details of this were not given; they also mostly had a high risk of attrition bias and reporting bias.We could not combine data from all the trials as they compared different formulations. Findings from individual studies provided insufficient evidence to determine the size and precision of the effects of different formulations. Ten studies reported information on the review's primary outcome (nutritional status); however, we were only able to combine data from two small cross-over studies (n = 41). The estimated gain in body weight was imprecise, 0.32 kg (95% confidence interval -0.03 to 0.67; P = 0.07). Combined data from the same studies gave statistically significant results favouring enteric-coated microspheres over enteric-coated tablets for our secondary outcomes stool frequency, mean difference -0.58 (95% confidence interval -0.85 to -0.30; P < 0.0001); proportion of days with abdominal pain, mean difference -7.96% (95% confidence interval -12.97 to -2.94; P = 0.002); and fecal fat excretion, mean difference -11.79 g (95% confidence interval -17.42 to -6.15; P < 0.0001). Data from another single small cross-over study also favoured enteric-coated microspheres over non-enteric-coated tablets with adjuvant cimetidine in terms of stool frequency, mean difference -0.70 (95% confidence interval -0.90 to -0.50; P < 0.00001). There is limited evidence of benefit from enteric-coated microspheres when compared to non-enteric coated pancreatic enzyme preparations up to one month. In the only comparison where we could combine any data, the fact that these were cross-over studies is likely to

  11. Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells

    NASA Astrophysics Data System (ADS)

    Rich, Devra P.; Anderson, Matthew P.; Gregory, Richard J.; Cheng, Seng H.; Paul, Sucharita; Jefferson, Douglas M.; McCann, John D.; Klinger, Katherine W.; Smith, Alan E.; Welsh, Michael J.

    1990-09-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) was expressed in cultured cystic fibrosis airway epithelial cells and Cl- channel activation assessed in single cells using a fluorescence microscopic assay and the patch-clamp technique. Expression of CFTR, but not of a mutant form of CFTR (ΔF508), corrected the Cl- channel defect. Correction of the phenotypic defect demonstrates a causal relationship between mutations in the CFTR gene and defective Cl- transport which is the hallmark of the disease.

  12. Child with Abdominal Pain.

    PubMed

    Iyer, Rajalakshmi; Nallasamy, Karthi

    2018-01-01

    Abdominal pain is one of the common symptoms reported by children in urgent care clinics. While most children tend to have self-limiting conditions, the treating pediatrician should watch out for underlying serious causes like intestinal obstruction and perforation peritonitis, which require immediate referral to an emergency department (ED). Abdominal pain may be secondary to surgical or non-surgical causes, and will differ as per the age of the child. The common etiologies for abdominal pain presenting to an urgent care clinic are acute gastro-enteritis, constipation and functional abdominal pain; however, a variety of extra-abdominal conditions may also present as abdominal pain. Meticulous history taking and physical examination are the best tools for diagnosis, while investigations have a limited role in treating benign etiologies.

  13. Initiating transitional care for adolescents with cystic fibrosis at the age of 12 is both feasible and promising.

    PubMed

    Skov, M; Teilmann, G; Damgaard, I N; Nielsen, K G; Hertz, P G; Holgersen, M G; Presfeldt, M; Dalager, A M S; Brask, M; Boisen, K A

    2018-05-05

    Adolescence is a vulnerable period in cystic fibrosis, associated with declining lung function. This study described, implemented and evaluated a transition programme for adolescents. We conducted a single centre, non-randomised and non-controlled prospective programme at the cystic fibrosis centre at Copenhagen University Hospital Rigshospitalet from 2010-2011, assessing patients aged 12 to 18 at baseline and after 12 months. Changes implemented included staff training on communication, a more youth friendly feel to the outpatient clinic, the introduction of youth consultations partly alone with the adolescent, and a parents' evening focusing on cystic fibrosis in adolescence. Lung function and body mass index (BMI) were measured monthly and adolescents were assessed for their readiness for transition and quality of life at baseline and 12 months. We found that 40 (98%) of the eligible patients participated and youth consultations were successfully implemented with no dropouts. The readiness checklist score increased significantly over the one-year study period, indicating increased readiness for transfer and self-care. Overall quality of life, lung function and BMI remained stable during the study period. A well structured transition programme for cystic fibrosis patients as young as 12 years of age proved to be both feasible and sustainable. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  14. Mucocele of the cystic duct remnant after orthotopic liver transplant: a problem revisited.

    PubMed

    Chatterjee, Suvadip; Das, Debasish; Hudson, Mark; Bassendine, Margaret Fiona; Scott, John; Oppong, Kofi Ernest; Sen, Gourab; French, Jeremy J

    2011-06-01

    Mucocele of the cystic duct remnant is an uncommon hepatobiliary complication of a liver transplant. Current practice usually involves either excising the cystic duct, or incorporating the distal end of the transected cystic duct into the suture line of the biliary anastomosis to ensure drainage. We report a patient who developed cystic duct remnant mucocele after the latter approach was adopted. We believe that this is likely related to delayed anastomotic stricturing, which prevented draining from the remnant cystic duct. We also discuss the incidence, pathology, investigations, and treatment of this condition.

  15. Adenoid cystic carcinoma: An unusual presentation.

    PubMed

    Pushpanjali, M; Sujata, D Naga; Subramanyam, S Bala; Jyothsna, M

    2014-05-01

    The adenoid cystic carcinoma is a relatively rare epithelial tumor of the major and minor salivary glands, accounting for about 1% of all malignant tumor of the oral and maxillofacial regions. Peak incidence occurs between the 5(th) and 6(th) decades of life. The clinical and pathological findings typical of this tumor include slow growth, peri-neural invasion, multiple local recurrences and distant metastasis. Herein, we report a case of adenoid cystic carcinoma of oropharynx with unusual clinical presentation. The diagnosis of this case and importance of cytology in diagnosing such cases is discussed.

  16. Ovariectomy due to a dermoid cyst in an orangutan (Pongo pygmaeus).

    PubMed

    Gyimesi, Zoltan S; Forrester, James W; Doering, David L; Burns, Roy B; McManamon, Rita

    2015-03-01

    A large abdominal mass was identified during an elective preventative health examination in a 25-yr-old female hybrid orangutan (Pongo pygmaeus). Sonographically, the mass was fluid-filled and a presumptive diagnosis of a dermoid cyst or cystic teratoma of an ovary was made. Exploratory laparotomy, after assembly of a surgical team, confirmed that the 2.5-kg cystic mass was associated with the left ovary. Following ovariectomy, perioperative dissection of the mass revealed hair components, confirming the working diagnosis. Because the right ovary was slightly nodular and firmer than expected, and these germ cell tumors sometimes occur bilaterally, excision of the contralateral ovary was elected. Histopathology confirmed the left ovarian mass was a dermoid cyst; the right ovary contained a corpus hemorrhagicum but no evidence of neoplasia. Recovery was uneventful and the orangutan was prescribed hormone replacement therapy to mitigate potential problems associated with a chronic lack of estrogen and progesterone. This case report demonstrates the importance of elective examinations under anesthesia, even in well-trained great apes.

  17. Episodic seasonal Pseudo-Bartter syndrome in cystic fibrosis.

    PubMed

    Kintu, Brett; Brightwell, Alex

    2014-06-01

    Pseudo-Bartter syndrome (PBS) describes an uncommon but well recognised complication of cystic fibrosis leading to hypochloraemic, hypokalaemic metabolic alkalosis. Pseudo-Bartter syndrome is usually seen at initial presentation or within the first two years of life in children with cystic fibrosis. Risk factors for development of PBS include warm weather conditions, severe respiratory or pancreatic disease and gastrointestinal losses (e.g. vomiting and diarrhoea). PBS is rare in older children and adolescents although epidemics have been associated with heat wave conditions in warmer climates. In this era of climate change, it is crucial that clinicians consider Pseudo-Bartter syndrome when patients with cystic fibrosis present unwell during summer. Copyright © 2014 Elsevier Ltd. All rights reserved.

  18. Galectin-3 levels relate in children to total body fat, abdominal fat, body fat distribution, and cardiac size.

    PubMed

    Dencker, Magnus; Arvidsson, Daniel; Karlsson, Magnus K; Wollmer, Per; Andersen, Lars B; Thorsson, Ola

    2018-03-01

    Galectin-3 has recently been proposed as a novel biomarker for cardiovascular disease in adults. The purpose of this investigation was to assess relationships between galectin-3 levels and total body fat, abdominal fat, body fat distribution, aerobic fitness, blood pressure, left ventricular mass, left atrial size, and increase in body fat over a 2-year period in a population-based sample of children. Our study included 170 children aged 8-11 years. Total fat mass and abdominal fat were measured by dual-energy x-ray absorptiometry (DXA). Body fat distribution was expressed as abdominal fat/total fat mass. Maximal oxygen uptake was assessed by indirect calorimetry during a maximal exercise test and scaled to body mass. Systolic and diastolic blood pressure and pulse pressure were measured. Left atrial size, left ventricular mass, and relative wall thickness were measured by echocardiography. Frozen serum samples were analyzed for galectin-3 by the Proximity Extension Assay technique. A follow-up DXA scan was performed in 152 children 2 years after the baseline exam. Partial correlations, with adjustment for sex and age, between galectin-3 versus body fat measurements indicated weak to moderate relationships. Moreover, left atrial size, left ventricular mass, and relative wall thickness and pulse pressure were also correlated with galectin-3. Neither systolic blood pressure nor maximal oxygen uptake was correlated with galectin-3. There was also a correlation between galectin-3 and increase in total body fat over 2 years, while no such correlations were found for the other fat measurements. More body fat and abdominal fat, more abdominal body fat distribution, more left ventricular mass, and increased left atrial size were all associated with higher levels of galectin-3. Increase in total body fat over 2 years was also associated with higher levels of galectin-3. What is Known: • Galectin-3 has been linked to obesity and been proposed to be a novel biomarker

  19. Abdominal wall fat pad biopsy

    MedlinePlus

    Amyloidosis - abdominal wall fat pad biopsy; Abdominal wall biopsy; Biopsy - abdominal wall fat pad ... most common method of taking an abdominal wall fat pad biopsy . The health care provider cleans the ...

  20. A retrospective investigation of abdominal visceral fat, body mass index (BMI), and active smoking as risk factors for donor site wound healing complications after free DIEP flap breast reconstructions.

    PubMed

    Timmermans, Floyd W; Westland, Pèdrou B; Hummelink, Stefan; Schreurs, Joep; Hameeteman, Marijn; Ulrich, Dietmar J O; Slater, Nicholas J

    2018-06-01

    The deep inferior epigastric artery perforator (DIEP) flap is one of the most common techniques for breast reconstruction. Body mass index (BMI) is considered as an important predictor of donor site healing complications such as wound dehiscence. The use of computed tomography (CT) proved to be a precise and objective method to assess visceral adipose tissue. It remains unclear whether quantification of visceral fat provides more accurate predictions of abdominal wound healing complications than BMI. A total of 97 patients with DIEP flap were retrospectively evaluated. Patients' abdominal visceral fat (AVF) was quantified on CT angiography (CTA). The patients were postoperatively assessed for abdominal wound healing complications. We analyzed for the correlations between AVF, BMI, and dehiscence and established a logistic regression model to assess the potential high-profile predictors in anatomic and patient characteristics such as weight, smoking, and diabetes. We included 97 patients, and of them, 24 patients (24.7%) had some degree of abdominal dehiscence. No significant differences were observed between the dehiscence group and the non-dehiscence group, except for smoking (p = 0.002). We found a significant correlation between AVF and BMI (R = 0.282, p = 0.005), but neither was significant in predicting donor site dehiscence. Smoking greatly increased the likelihood of developing wound dehiscence (OR = 11.4, p = < 0.001). AVF and BMI were not significant predictors of abdominal wound healing complications after DIEP flap reconstruction. This study established active smoking (OR = 11.4, p = < 0.001) as the significant risk factor that contributed to the development of abdominal wound dehiscence in patients with DIEP. Copyright © 2018 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

  1. Abdominal actinomycosis masquerading as an omental tumor in a 12-year-old female.

    PubMed

    Hirayama, Yutaka; Iinuma, Yasushi; Hashizume, Naoki; Yoshida, Motomu; Iida, Hisataka; Shibuya, Hiroyuki; Naito, Shinichi; Nitta, Koju

    2013-02-01

    We herein report a case of abdominal actinomycosis in a 12-year-old girl in whom an omental primary tumor was suspected before surgery. The patient began to experience intermittent lower left abdominal pain. Abdominal computed tomography (CT) scans were inconclusive at this time, but 6 months later, CT and magnetic resonance imaging (MRI) examinations showed a 7-cm, tumor-like lesion in the left abdominal cavity; malignancy could not be ruled out. The tumor, which originated in the omentum and adhered strongly to the left abdominal wall, was resected along with approximately 90 % of the omentum, the peritoneum in contact with the mass, and the posterior layer of the rectus abdominal sheath, under suspicion of a malignant tumor. However, omental actinomycosis was the final pathological diagnosis. The patient's antibiotic treatment was changed to a penicillin-series oral antibiotic to prevent recurrence of the actinomycosis. The patient was discharged from our hospital 16 days after the first surgery, but she developed three episodes of ileus; the first two required surgery. The patient has had no further recurrences of actinomycosis or postoperative ileus 20 months after discharge.

  2. Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis.

    PubMed

    Elborn, J Stuart; Bell, Scott C; Madge, Susan L; Burgel, Pierre-Regis; Castellani, Carlo; Conway, Steven; De Rijcke, Karleen; Dembski, Birgit; Drevinek, Pavel; Heijerman, Harry G M; Innes, J Alistair; Lindblad, Anders; Marshall, Bruce; Olesen, Hanne V; Reimann, Andreas L; Solé, Ampara; Viviani, Laura; Wagner, Thomas O F; Welte, Tobias; Blasi, Francesco

    2016-02-01

    The improved survival in people with cystic fibrosis has led to an increasing number of patients reaching adulthood. This trend is likely to be maintained over the next decades, suggesting a need to increase the number of centres with expertise in the management of adult patients with cystic fibrosis. These centres should be capable of delivering multidisciplinary care addressing the complexity of the disease, in addition to addressing the psychological burden on patients and their families. Further issues that require attention are organ transplantation and end of life management.Lung disease in adults with cystic fibrosis drives most of the clinical care requirements, and major life-threatening complications, such as respiratory infection, respiratory failure, pneumothorax and haemoptysis, and the management of lung transplantation require expertise from trained respiratory physicians. The taskforce therefore strongly reccommends that medical leadership in multidisciplinary adult teams should be attributed to a respiratory physician adequately trained in cystic fibrosis management.The task force suggests the implementation of a core curriculum for trainees in adult respiratory medicine and the selection and accreditation of training centres that deliver postgraduate training to the standards of the HERMES programme. Copyright ©ERS 2016.

  3. Renal diseases in adults with cystic fibrosis: a 40 year single centre experience.

    PubMed

    Wilcock, M J; Ruddick, A; Gyi, K M; Hodson, M E

    2015-10-01

    There is a sizable literature describing renal disease in patients with cystic fibrosis. Previous studies have focused on single disease processes alone, most commonly renal stone disease or acute kidney injury. In this study we report for the first time on the prevalence of all forms of renal disease in a cystic fibrosis population. A retrospective review of adult patients with cystic fibrosis attending the Adult Cystic Fibrosis Department at the Royal Brompton Hospital was carried out by searching the department's database to identify patients with renal problems and subsequently retrieving clinical information from medical notes. The prevalence of all renal diseases in our population was 5.1 %. The most commonly identified problem was renal stones. At 2.0 % the prevalence of renal stones in adult patients with cystic fibrosis was comparable to the general population. A range of other renal diseases were identified, the next most common being drug-induced acute kidney injury. A range of cystic fibrosis independent and attributable diseases has been identified but no cystic fibrosis specific disease. In contrast to other cystic fibrosis centres no increased prevalence of renal stones was found.

  4. Health-related quality of life of Spanish children with cystic fibrosis.

    PubMed

    Groeneveld, Iris F; Sosa, Elena S; Pérez, Margarita; Fiuza-Luces, Carmen; Gonzalez-Saiz, Laura; Gallardo, Cristian; López-Mojares, Luis M; Ruiz, Jonatan R; Lucia, Alejandro

    2012-12-01

    To investigate (1) the contributions of sex, age, nutritional status- and physical-fitness-related variables on health-related quality of life (HRQOL) in Spanish children with cystic fibrosis, and (2) the agreement on HRQOL between children and their parents. In 28 children aged 6-17 years, body mass index percentile, percentage body fat, physical activity, pulmonary function, cardiorespiratory fitness, functional mobility, and dynamic muscle strength were determined using objective measures. HRQOL was measured using the revised version of the cystic fibrosis questionnaire. Simple and multiple linear regression analyses were performed to determine the variables associated with HRQOL. To assess the agreement on HRQOL between children and parents, intra-class correlation coefficients (ICCs) were calculated. Girls reported worse emotional functioning, a higher treatment burden, and more respiratory problems than boys. Greater functional mobility appeared associated with a less favourable body image and more eating disturbances. Agreement on HRQOL between children and parents was good to excellent, except for the domain of treatment burden. Sex and age were stronger predictors of HRQOL than nutritional status- or physical-fitness-related variables. Children reported a lower treatment burden than their parents perceived them to have.

  5. Survey of abdominal obesities in an adult urban population of Kinshasa, Democratic Republic of Congo

    PubMed Central

    Kasiam Lasi On’kin, JB; Longo-Mbenza, B; Okwe, A Nge; Kabangu, N Kangola

    2007-01-01

    Summary Background The prevalence of overweight/obesity, which is an important cardiovascular risk factor, is rapidly increasing worldwide. Abdominal obesity, a fundamental component of the metabolic syndrome, is not defined by appropriate cutoff points for sub-Saharan Africa. Objective To provide baseline and reference data on the anthropometry/body composition and the prevalence rates of obesity types and levels in the adult urban population of Kinshasa, DRC, Central Africa. Methods During this cross-sectional study carried out within a random sample of adults in Kinshasa town, body mass index, waist circumference and fatty mass were measured using standard methods. Their reference and local thresholds (cut-off points) were compared with those of WHO, NCEP and IFD to define the types and levels of obesity in the population. Results From this sample of 11 511 subjects (5 676 men and 5 835 women), the men presented with similar body mass index and fatty mass values to those of the women, but higher waist measurements. The international thresholds overestimated the prevalence of denutrition, but underscored that of general and abdominal obesity. The two types of obesity were more prevalent among women than men when using both international and local thresholds. Body mass index was negatively associated with age; but abdominal obesity was more frequent before 20 years of age and between 40 and 60 years old. Local thresholds of body mass index (≥ 23, ≥ 27 and ≥ 30 kg/m2) and waist measurement (≥ 80, ≥ 90 and ≥ 94 cm) defined epidemic rates of overweight/general obesity (52%) and abdominal obesity (40.9%). The threshold of waist circumference ≥ 94 cm (90th percentile) corresponding to the threshold of the body mass index ≥ 30 kg/m2 (90th percentile) was proposed as the specific threshold of definition of the metabolic syndrome, without reference to gender, for the cities of sub-Saharan Africa. Conclusion Further studies are required to define the

  6. [Historical compilation of cystic fibrosis].

    PubMed

    Navarro, Salvador

    2016-01-01

    Cystic fibrosis is the most common life-shortening recessively inherited disorder in the Caucasian population. The genetic mutation that most frequently provokes cystic fibrosis (ΔF508) appeared at least 53,000years ago. For many centuries, the disease was thought to be related to witchcraft and the "evil eye" and it was only in 1938 that Dorothy H. Andersen characterized this disorder and suspected its genetic origin. The present article reviews the pathological discoveries and diagnostic and therapeutic advances made in the last 75 years. The review ends with some considerations for the future. Copyright © 2015 Elsevier España, S.L.U. and AEEH y AEG. All rights reserved.

  7. Caring for Children with Cystic Fibrosis: A Collaborative Clinical and School Approach

    ERIC Educational Resources Information Center

    Strawhacker, MaryAnn Tapper; Wellendorf, Joyce

    2004-01-01

    Earlier diagnosis and more effective treatments have improved both morbidity and mortality associated with cystic fibrosis, making regular school attendance a reality. School nurses have a unique opportunity to assist students with cystic fibrosis successfully manage their disease. Medical treatment for cystic fibrosis can be complex, leaving…

  8. Cystic fibrosis respiratory tract salt concentration: An Exploratory Cohort Study.

    PubMed

    Grandjean Lapierre, Simon; Phelippeau, Michael; Hakimi, Cyrine; Didier, Quentin; Reynaud-Gaubert, Martine; Dubus, Jean-Christophe; Drancourt, Michel

    2017-11-01

    In cystic fibrosis patients, electrolytic and osmolality imbalance secondary to cystic fibrosis transmembrane conductance regulator mutations may impact on mucoid secretion accumulation and secondary colonization by opportunistic pathogens such as nontuberculous mycobacteria.We performed a noninvasive exploratory prospective controlled clinical study comparing sputum salinity and acid-base characteristics of cystic fibrosis and noncystic fibrosis control patients. A total of 57 patients and 62 controls were included.Sputum salt concentrations were 10.5 g/L (95% CI: 7.7-13.3) in patients and 7.4 g/L (95% CI: 5.9-8.9) in aged-matched controls, a difference that was found to be statistically significant (P < .05). No difference in pH was observed between patients and controls.These differences in respiratory secretions salt concentrations could influence host-pathogen interactions in the context of cystic fibrosis respiratory infections. We propose to include respiratory secretion salt measurement as a routine analysis on cystic fibrosis patients' sputum submitted for bacterial culture. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  9. Intrinsic pro-angiogenic status of cystic fibrosis airway epithelial cells

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Verhaeghe, Catherine; Tabruyn, Sebastien P.; Oury, Cecile

    Cystic fibrosis is a common genetic disorder characterized by a severe lung inflammation and fibrosis leading to the patient's death. Enhanced angiogenesis in cystic fibrosis (CF) tissue has been suggested, probably caused by the process of inflammation, as similarly described in asthma and chronic bronchitis. The present study demonstrates an intrinsic pro-angiogenic status of cystic fibrosis airway epithelial cells. Microarray experiments showed that CF airway epithelial cells expressed several angiogenic factors such as VEGF-A, VEGF-C, bFGF, and PLGF at higher levels than control cells. These data were confirmed by real-time quantitative PCR and, at the protein level, by ELISA. Conditionedmore » media of these cystic fibrosis cells were able to induce proliferation, migration and sprouting of cultured primary endothelial cells. This report describes for the first time that cystic fibrosis epithelial cells have an intrinsic angiogenic activity. Since excess of angiogenesis is correlated with more severe pulmonary disease, our results could lead to the development of new therapeutic applications.« less

  10. Cystic adventitial degeneration: ectopic ganglia from adjacent joint capsules.

    PubMed

    Ortmann, J; Widmer, M K; Gretener, S; Do, D D; Willenberg, T; Daliri, A; Baumgartner, I

    2009-11-01

    Cystic adventitial degeneration is a rare non-atherosclerotic cause of peripheral arterial occlusive disease, mainly seen in young men without other evidence of vascular disease. Diagnosis will be established by clinical findings and by ultrasound or angiography and can be treated by excision or enucleation of the affected arterial segment or by percutaneous ultrasound-guided aspiration. However, the etiology of adventitial cysts remains unknown. We report a case of cystic adventitial degeneration showing a connection between the joint capsule and the adventitial cyst, supporting the theory that cystic adventitial degeneration may represent ectopic ganglia from adjacent joint capsules.

  11. A retrospective analysis of human cystic echinococcosis in Hamedan province, an endemic region of Iran.

    PubMed

    Ahmadi, N A; Hamidi, M

    2008-10-01

    In a retrospective investigation, the medical files of the patients who, between 1992 and 2006, underwent surgery for the treatment of cystic echinococcosis (CE) in one of the three university hospitals in Hamedan province, in western Iran, were explored. Of the 179 patients involved, 44.1% were male and 55.9% female, giving a male/ female ratio of 0.79. The mean annual surgical incidence of CE in the catchment areas of the study hospitals was estimated to be 1.33 cases/100,000. Although most of Hamedan's population is urban (53.02% at the last census, in 1997), most (57.5%) of the patients lived in rural areas (P<0.05). Among the CE cases, housewives (47.3%), farmers (16.6%) and labourers (16.0%) were over-represented compared with the frequencies of these occupations in the general population of the region (P<0.05 for each). Most of the CE cases were aged 20-39 years (35.8%) or 40-59 years (29.0%) and most had liver cysts, either alone (60.9%) or with cysts in other sites (9.5%), although 19.6% of the cases appeared only to have lung cysts. Overall, 90.5% of the cases showed single-organ involvement. Liver involvement was 3.3-fold more common than lung involvement, the ratio between cases with liver cysts and those with lung cysts generally increasing with age (from 1.6 for the cases aged <20 years to 5.6 among the cases aged 40-59 years). The most common clinical symptoms were abdominal pain and/or a sensation of abdominal mass in those with hepatic CE, and chest pain, cough, dyspnoea and/or haemoptysis in those with pulmonary CE. Although 72% of the cases each had a single cyst, 19.6% had two cysts each and 8.4% each had at least three cysts. Only three (1.7%) of the cases had had previous surgery for CE. Diagnosis of CE in Hamedan province is largely dependent on ultrasonography and computed tomography. Together, these results confirm that CE is endemic in the province and indicate that certain occupations (housewife, farmer and labourer) and/or rural life are

  12. Predicting the intrauterine fetal death of fetuses with cystic hygroma in early pregnancy.

    PubMed

    Shimura, Mai; Ishikawa, Hiroshi; Nagase, Hiromi; Mochizuki, Akihiko; Sekiguchi, Futoshi; Koshimizu, Naho; Itai, Toshiyuki; Odagami, Mizuha

    2018-01-11

    We investigated whether it was possible to predict the prognosis of fetuses with cystic hygroma in early pregnancy based on the degree of neck thickening. We retrospectively analyzed 57 singleton pregnancies with fetuses with cystic hygroma who were examined before the 22nd week of pregnancy. The fetuses were categorized according to the outcome, structural abnormalities at birth, and chromosomal abnormalities. Here, we proposed a new sonographic predictor with which we assessed neck thickening by dividing the width of the neck thickening by the biparietal diameter, which is expressed as the cystic hygroma width/biparietal diameter ratio. The median cystic hygroma width/biparietal diameter ratio in the intrauterine fetal death group (0.51) was significantly higher than that in the live birth group (0.27). No significant difference in the median cystic hygroma width/biparietal diameter ratio was found between the structural abnormalities group at birth and the no structural abnormalities group, and no significant difference in the median cystic hygroma width/biparietal diameter ratio was found between the chromosomal abnormality group and the no chromosomal abnormality group. We used receiver operating characteristic analysis to evaluate the cystic hygroma width/biparietal diameter ratio to predict intrauterine fetal death. When the cystic hygroma width/biparietal diameter ratio cut-off value was 0.5, intrauterine fetal death could be predicted with a sensitivity of 52.9% and a specificity of 100%. It is possible to predict intrauterine fetal death in fetuses with cystic hygroma in early pregnancy if cystic hygroma width/biparietal diameter ratio is measured. However, even if cystic hygroma width/biparietal diameter ratio is measured, predicting the presence or absence of a structural abnormality at birth or a chromosomal abnormality is difficult. © 2018 Japanese Teratology Society.

  13. Review of surgical management of cystic hydatid disease in a resource limited setting: Turkana, Kenya.

    PubMed

    Cooney, Rachel M; Flanagan, Kevin P; Zehyle, Ebehard

    2004-11-01

    Cystic hydatid disease is a zoonotic infection of humans caused by infection with the larval stage of Echinococcus granulosus. The prevalence rate (5-10%) in Turkana, Northern Kenya is among the highest worldwide. With an increase in foreign travel and migration of peoples, practitioners need to be aware of diseases common in these countries and their rarer manifestations. The objective of this study was to review the management of this disease in a poorly resourced high-prevalence area. The surgical records of Kakuma Mission Hospital, Turkana from July 1981 to May 2002 were reviewed. A total of 710 (female : male, 3 : 2) surgical procedures for hydatid disease were recorded, the average age being 27 years (range, 3-65 years). There were 663 patients, and therefore 47 patients had repeat procedures. A total of 52.8% (n = 375) had hepatic cysts (248 right sided, 30 left sided, 97 site undefined), and 10.1% (n = 72) had multiple abdominal cysts. Other intra-abdominal sites included 8.16% mesenteric (n = 58), 2.9% retroperitoneal (n = 21), 3.5% spleen (n = 25), the abdominal wall (n = 4). There was no information on the site of disease in 5.9% of cases (n = 42). More unusual sites were retro-orbital, gluteal, zygomatic, brachial, parotid, uterine, tibial and foot. The largest volume of cyst fluid was 7 l. There was one intraoperative death and one postoperative death recorded. Endocystectomy +/- a scolicidal was the commonest procedure performed. However, 165 percutaneous aspiration injection of a scolicidal agent and reaspiration procedures were performed, six being on pregnant women. This is one of the largest series of surgical hydatid patients from Africa and demonstrates the wide diversity in cyst location.

  14. Destructive granuloma derived from a liver cyst: A case report

    PubMed Central

    Kawashita, Yujo; Kamohara, Yukio; Furui, Junichiro; Fujita, Fumihiko; Miyamoto, Shungo; Takatsuki, Mitsuhisa; Abe, Kuniko; Hayashi, Tomayoshi; Ohno, Yasuharu; Kanematsu, Takashi

    2006-01-01

    We herein report the case of an idiopathic liver cystic mass which aggressively infiltrated the thoraco-abdominal wall. A 74-year-old woman who had a huge cystic lesion in her right hepatic lobe was transferred to our hospital for further examinations. Imaging studies revealed a simple liver cyst, and the cytological findings of intracystic fluid were negative. She was followed up periodically by computed tomography (CT) scans. Seven years later, she complained of a prominence and dull pain in her right thoraco-abdominal region. CT revealed an enlargement of the cystic lesion and infiltration into the intercostal subcutaneous tissue. We suspected the development of a malignancy inside the liver cyst such as cystadenocarcinoma, and she therefore underwent surgery. A tumor extirpation was performed, including the chest wall, from the 7th to the 10th rib, as well as a right hepatic lobectomy. Pathologically, the lesion consisted of severe inflammatory change with epithelioid cell granuloma and bone destruction without any malignant neoplasm. No specific pathogens were evident based on further histological and molecular examinations. Therefore the lesion was diagnosed to be a destructive granuloma associated with a long-standing hepatic cyst. Since undergoing surgery, the patient has been doing well without any signs of recurrence. PMID:16586558

  15. Diagnostic problems in cystic fibrosis - specific characteristics of a group of infants and young children diagnosed positive through neonatal screening, in whom cystic fibrosis had not been diagnosed.

    PubMed

    Woś, Halina; Sankiewicz-Szkółka, Magda; Więcek, Sabina; Kordys-Darmolińska, Bożena; Grzybowska-Chlebowczyk, Urszula; Kniażewska, Maria

    2015-01-01

    Neonatal cystic fibrosis screening contributes to an early diagnosis of cystic fibrosis and to implementing appropriate therapeutic management. Long-standing screening tests have made it possible to identify a group of newborns in whom the diagnosis was ambiguous and required further specialised tests. The aim is to present cases of patients with a positive result of newborn screening for cystic fibrosis who were found to be carriers of the mutation in both alleles, however the lack of clinical symptoms and correct sweat testing values did not lead doctors to diagnosing cystic fibrosis and by the same token implementing the treatment. The analysis encompassed a group of 22 infants and children 3 months to 3 years of age, in whom, in spite of a positive result of newborn screening for cystic fibrosis and the presence of 2 mutations in the CFTR gene, the diagnosis of cystic fibrosis was not made, and appropriate treatment was not administered because of diagnostic doubts (due to correct concentration of chlorides in sweat, correct IRT level and lack of clinical signs of cystic fibrosis). The control group consisted of 55 children treated in our centre, in whom neonatal screening for cystic fibrosis was positive and the diagnosis was confirmed by genetic testing, sweat chloride testing and IRT concentration. There were no differences in birth body weight between the groups. The differences in chlorideion levels in sweat secretion tests and mean IRT values were statistically significant and were: 97.5 for the control group and 26.4 for the test group. At the present time there are no clinical symptoms to give a diagnosis of cystic fibrosis and start treatment in the test group. Newborn screening contributes not only to an early diagnosis of cystic fibrosis but also to CFTR-related metabolic syndromes (CRMS), which is a phenomenon requiring further observation. This fact constitutes a definite psychological problem for the parents of these patients. .

  16. Perivascular epithelioid cell tumor (PEComa) of abdominal cavity from falciform ligament: a case report.

    PubMed

    Choi, Cheol Woong; Kim, Tae Oh; Kim, Kyung Yeob; Lee, Sun Mi; Kim, Gwang Ha; Kang, Dae Hwan; Song, Geun Am; Kim, Suk; Kim, Dae Hwan

    2009-04-01

    We present a case of perivascular epithelioid cell tumors (PEComas) in the abdominal cavity at the falciform ligament. A 30-yr-old Korean man visited to hospital for the evaluation of a growing, palpable abdominal mass. He had felt the mass growing over 6 months. There was no family or personal history of tuberous sclerosis. The resected specimen showed a mass of 8.0x7.0x5.5 cm in size. Histological examination showed sheets of spindle-to-epithelioid cells with clear-to-eosinophilic cytoplasm. Immunohistochemically, tumor cells were positive for HMB-4 (gp100) and smooth muscle actin. They were also positive for the S-100, which is a marker of neurogenic and melanocytic tumors. Patient was treated with radical resection of tumor without any adjuvant therapy. He is well and on follow-up visits without tumor recurrence.

  17. Perivascular Epithelioid Cell Tumor (PEComa) of Abdominal Cavity from Falciform Ligament: A Case Report

    PubMed Central

    Choi, Cheol Woong; Kim, Kyung Yeob; Lee, Sun Mi; Kim, Gwang Ha; Kang, Dae Hwan; Song, Geun Am; Kim, Suk; Kim, Dae Hwan

    2009-01-01

    We present a case of perivascular epithelioid cell tumors (PEComas) in the abdominal cavity at the falciform ligament. A 30-yr-old Korean man visited to hospital for the evaluation of a growing, palpable abdominal mass. He had felt the mass growing over 6 months. There was no family or personal history of tuberous sclerosis. The resected specimen showed a mass of 8.0×7.0×5.5 cm in size. Histological examination showed sheets of spindle-to-epithelioid cells with clear-to-eosinophilic cytoplasm. Immunohistochemically, tumor cells were positive for HMB-4 (gp100) and smooth muscle actin. They were also positive for the S-100, which is a marker of neurogenic and melanocytic tumors. Patient was treated with radical resection of tumor without any adjuvant therapy. He is well and on follow-up visits without tumor recurrence. PMID:19399284

  18. Hormonal abnormalities of the pancreas and gut in cystic fibrosis.

    PubMed

    Adrian, T E; McKiernan, J; Johnstone, D I; Hiller, E J; Vyas, H; Sarson, D L; Bloom, S R

    1980-09-01

    We have investigated the effect of cystic fibrosis on alimentary hormones in 10 children by measuring the pancreatic and gut hormone rsponse to a milk drink. Plasma insulin and gastric inhibitory peptide were both significantly reduced (P < 0.05 and P < 0.005, respectively, at 15 min) in the patients with cystic fibrosis, compared with controls, even though the early glucose rise was greater in the former group (P < 0.05 at 15 min). Fasting levels of pancreatic polypeptide were significantly lower in the fibrocystic children (P < 0.01), and the normal response to milk was completely abolished in these patients (P < 0.001). Fasting plasma enteroglucagon concentrations were grossly abolished in the cystic fibrosis patients (P < 0.001) and these remained elevated throughout the test. No significant differences were seen in basal or postmilk responses of plasma glucagon, gastrin, secretin, vasoactive intestinal peptide, or motilin in cystic fibrosis. It would thus appear that the pancreatic polypeptide cell is more susceptible to the effects of the disease process than the beta or alpha cell in cystic fibrosis. Some aspects of the abnormalities in the gastrointestinal endocrine system were similar to those seen in celiac disease and tropical sprue and may, therefore, effect a similar hormonal response in these patients with cystic fibrosis to those with mucosal damage.

  19. Bone disease in cystic fibrosis: new pathogenic insights opening novel therapies.

    PubMed

    Jacquot, J; Delion, M; Gangloff, S; Braux, J; Velard, F

    2016-04-01

    Mutations within the gene encoding for the chloride ion channel cystic fibrosis transmembrane conductance regulator (CFTR) results in cystic fibrosis (CF), the most common lethal autosomal recessive genetic disease that causes a number of long-term health problems, as the bone disease. Osteoporosis and increased vertebral fracture risk associated with CF disease are becoming more important as the life expectancy of patients continues to improve. The etiology of low bone density is multifactorial, most probably a combination of inadequate peak bone mass during puberty and increased bone losses in adults. Body mass index, male sex, advanced pulmonary disease, malnutrition and chronic therapies are established additional risk factors for CF-related bone disease (CFBD). Consistently, recent evidence has confirmed that CFTR plays a major role in the osteoprotegerin (OPG) and COX-2 metabolite prostaglandin E2 (PGE2) production, two key regulators in the bone formation and regeneration. Several others mechanisms were also recognized from animal and cell models contributing to malfunctions of osteoblast (cell that form bone) and indirectly of bone-resorpting osteoclasts. Understanding such mechanisms is crucial for the development of therapies in CFBD. Innovative therapeutic approaches using CFTR modulators such as C18 have recently shown in vitro capacity to enhance PGE2 production and normalized the RANKL-to-OPG ratio in human osteoblasts bearing the mutation F508del-CFTR and therefore potential clinical utility in CFBD. This review focuses on the recently identified pathogenic mechanisms leading to CFBD and potential future therapies for treating CFBD.

  20. Sonographic assessment of abdominal fat distribution during the first year of infancy.

    PubMed

    Brei, Christina; Much, Daniela; Heimberg, Ellen; Schulte, Verena; Brunner, Stefanie; Stecher, Lynne; Vollhardt, Christiane; Bauer, Jan S; Amann-Gassner, Ulrike; Hauner, Hans

    2015-09-01

    Longitudinal data regarding the fat distribution in the early postnatal period is sparse. We performed ultrasonography (US) as a noninvasive approach to investigate the development of abdominal subcutaneous (SC) and preperitoneal (PP) fat depots in infants ≤1 y and compared longitudinal US data with skinfold thickness (SFT) measurements and anthropometry in 162 healthy children at 6 wk, 4 mo, and 1 y postpartum. US was found to be a reproducible method for the quantification of abdominal SC and PP adipose tissue (AT) in this age group. Thickness of SC fat layers significantly increased from 6 wk to 4 mo and decreased at 1 y postpartum, whereas PP fat layers continuously increased. Girls had a significantly higher SC fat mass compared to boys, while there was no sex-specific difference in PP fat thickness. SC fat layer was strongly correlated with SFT measurements, while PP fat tissue was only weakly correlated with anthropometric measures. US is a feasible and reproducible method for the quantification of abdominal fat mass in infants ≤1 y of age. PP and SC fat depots develop differentially during the first year of life.

  1. "Cystic fibrotics could survive cholera, choleraics could survive cystic fibrosis"; hypothesis that explores new horizons in treatment of cystic fibrosis.

    PubMed

    Azimi, Arsalan

    2015-12-01

    Cystic fibrosis, the most common inherited disease of white population, is a disease of CFTR channels, in which mucosal function of many organs especially respiratory tract is impaired. Decreased mucociliary clearance and accumulation of mucus in airways facilitates colonization of infectious microorganisms, followed by infection. Following chronic infection, persistent inflammation ensues, which results in airway remodeling and deterioration of mucociliary clearance and result in a vicious cycle. Here, it is hypothesized that cholera toxin (CT) could ameliorate symptoms of cystic fibrosis as CT could dilute the thickened mucus, improve mucociliary clearance and alleviate airway obstruction. CT strengthens immunity of airway mucosa and it could attenuates bacterial growth and reduce persistency of infection. CT also modulates cellular immune response and it could decrease airway inflammation, hinder airway remodeling and prevent respiratory deterioration. Thereby it is hypothesized that CT could target and ameliorate many of pathophysiologic steps of the disease and it explores new horizons in treatment of CF. Copyright © 2015 Elsevier Ltd. All rights reserved.

  2. 68Ga-PSMA PET-CT Imaging of Metastatic Adenoid Cystic Carcinoma.

    PubMed

    de Keizer, Bart; Krijger, Gerard C; Ververs, F Tessa; van Es, Robert J J; de Bree, Remco; Willems, Stefan

    2017-12-01

    A patient with a history of adenoid cystic carcinoma of the nasal cavity presented himself with bone pain and an elevated PSA level. On suspicion of metastatic prostate cancer a 68 Ga-PSMA PET-CT was performed. The PET-CT showed numerous lung and non-sclerotic bone metastasis. Biopsy of a bone metastasis was performed and pathology showed adenoid cystic carcinoma instead of prostate cancer. Immunohistochemical PSMA staining of the primary tumour showed intense PSMA expression in adenoid cystic carcinoma tumour cells. Because of the high PSMA expression of adenoid cystic carcinoma, 68 Ga-PSMA PET-CT might be a promising imaging modality for this malignancy.

  3. Self-reported exercise and longitudinal outcomes in cystic fibrosis: a retrospective cohort study.

    PubMed

    Collaco, Joseph M; Blackman, Scott M; Raraigh, Karen S; Morrow, Christopher B; Cutting, Garry R; Paranjape, Shruti M

    2014-10-06

    Cystic fibrosis (CF) is characterized by recurrent respiratory infections and progressive lung disease. Whereas exercise may contribute to preserving lung function, its benefit is difficult to ascertain given the selection bias of healthier patients being more predisposed to exercise. Our objective was to examine the role of self-reported exercise with longitudinal lung function and body mass index (BMI) measures in CF. A total of 1038 subjects with CF were recruited through the U.S. CF Twin-Sibling Study. Questionnaires were used to determine exercise habits. Questionnaires, chart review, and U.S. CF Foundation Patient Registry data were used to track outcomes. Within the study sample 75% of subjects self-reported regular exercise. Exercise was associated with an older age of diagnosis (p = 0.002), older age at the time of ascertainment (p < 0.001), and higher baseline FEV1 (p = 0.001), but not CFTR genotype (p = 0.64) or exocrine pancreatic function (p = 0.19). In adjusted mixed models, exercise was associated with both a reduced decline in FEV1 (p < 0.001) and BMI Z-score (p = 0.001) for adults, but not children aged 10-17 years old. In our retrospective study, self-reported exercise was associated with improved longitudinal nutritional and pulmonary outcomes in cystic fibrosis for adults. Although prospective studies are needed to confirm these associations, programs to promote regular exercise among individuals with cystic fibrosis would be beneficial.

  4. Unsupervised quantification of abdominal fat from CT images using Greedy Snakes

    NASA Astrophysics Data System (ADS)

    Agarwal, Chirag; Dallal, Ahmed H.; Arbabshirani, Mohammad R.; Patel, Aalpen; Moore, Gregory

    2017-02-01

    Adipose tissue has been associated with adverse consequences of obesity. Total adipose tissue (TAT) is divided into subcutaneous adipose tissue (SAT) and visceral adipose tissue (VAT). Intra-abdominal fat (VAT), located inside the abdominal cavity, is a major factor for the classic obesity related pathologies. Since direct measurement of visceral and subcutaneous fat is not trivial, substitute metrics like waist circumference (WC) and body mass index (BMI) are used in clinical settings to quantify obesity. Abdominal fat can be assessed effectively using CT or MRI, but manual fat segmentation is rather subjective and time-consuming. Hence, an automatic and accurate quantification tool for abdominal fat is needed. The goal of this study is to extract TAT, VAT and SAT fat from abdominal CT in a fully automated unsupervised fashion using energy minimization techniques. We applied a four step framework consisting of 1) initial body contour estimation, 2) approximation of the body contour, 3) estimation of inner abdominal contour using Greedy Snakes algorithm, and 4) voting, to segment the subcutaneous and visceral fat. We validated our algorithm on 952 clinical abdominal CT images (from 476 patients with a very wide BMI range) collected from various radiology departments of Geisinger Health System. To our knowledge, this is the first study of its kind on such a large and diverse clinical dataset. Our algorithm obtained a 3.4% error for VAT segmentation compared to manual segmentation. These personalized and accurate measurements of fat can complement traditional population health driven obesity metrics such as BMI and WC.

  5. Gamma Knife Radiosurgery for the Treatment of Cystic Cerebral Metastases

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Ebinu, Julius O.; Lwu, Shelly; Monsalves, Eric

    Purpose: To assess the role of Gamma Knife radiosurgery (GKRS) in the treatment of nonsurgical cystic brain metastasis, and to determine predictors of response to GKRS. Methods: We reviewed a prospectively maintained database of brain metastases patients treated at our institution between 2006 and 2010. All lesions with a cystic component were identified, and volumetric analysis was done to measure percentage of cystic volume on day of treatment and consecutive follow-up MRI scans. Clinical, radiologic, and dosimetry parameters were reviewed to establish the overall response of cystic metastases to GKRS as well as identify potential predictive factors of response. Results:more » A total of 111 lesions in 73 patients were analyzed; 57% of lesions received prior whole-brain radiation therapy (WBRT). Lung carcinoma was the primary cancer in 51% of patients, 10% breast, 10% colorectal, 4% melanoma, and 26% other. Fifty-seven percent of the patients were recursive partitioning analysis class 1, the remainder class 2. Mean target volume was 3.3 mL (range, 0.1-23 mL). Median prescription dose was 21 Gy (range, 15-24 Gy). Local control rates were 91%, 63%, and 37% at 6, 12, and 18 months, respectively. Local control was improved in lung primary and worse in patients with prior WBRT (univariate). Only lung primary predicted local control in multivariate analysis, whereas age and tumor volume did not. Lesions with a large cystic component did not show a poorer response compared with those with a small cystic component. Conclusions: This study supports the use of GKRS in the management of nonsurgical cystic metastases, despite a traditionally perceived poorer response. Our local control rates are comparable to a matched cohort of noncystic brain metastases, and therefore the presence of a large cystic component should not deter the use of GKRS. Predictors of response included tumor subtype. Prior WBRT decreased effectiveness of SRS for local control rates.« less

  6. Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis.

    PubMed

    Lavie, Moran; Manovitz, Tzipora; Vilozni, Daphna; Levy-Mendelovich, Sarina; Sarouk, Ifat; Weintraubv, Ilana; Shoseyov, David; Cohen-Cymberknoh, Malena; Rivlin, Joseph; Efrati, Ori

    2015-01-07

    To investigate the long-term follow-up of distal intestinal obstruction syndrome (DIOS) in Israeli cystic fibrosis (CF) patients. This is a multi-center, comparative, retrospective study in which we reviewed the medical records of all CF patients from three major CF centers in Israel who were treated in the period from 1980 to 2012. Patients diagnosed with DIOS were defined as the study group. The patients were diagnosed with DIOS based on their clinical presentation and typical findings on either abdominal X-ray or computerized tomography scan. For the control group, CF patients with no DIOS were matched to the patients in the study group for age, sex, and cystic fibrosis transmembrane conductance regulator (CFTR) mutations. For both groups, the collected data included age, sex, CFTR genotype, weight, height, and body mass index. Clinical data included respiratory function tests in the last five years prior to the study, respiratory function test immediately before and after the DIOS event, number of hospitalizations, sputum culture results, and CF-related conditions diagnosed according to the CF clinical practice guidelines. In the study group, data on the DIOS treatment and tendency for DIOS recurrence were also analyzed. The medical charts for a total of 350 CF patients were reviewed. Of the 350 CF patients, 26 (7.4%) were diagnosed with DIOS. The control group included 31 CF patients with no DIOS diagnosis. The mean follow-up period was 21.6 ± 8.2 years. The total of DIOS episodes in the follow-up period was 60. The distribution of DIOS episodes was as follows: 6/26 (23.1%) study patients had one episode of DIOS in their lifetime, 7/26 (26.9%) had two episodes, 7/26 (26.9%) had three episodes, and 6/26 (23.1%) had four or more episodes. Compared to the control group, DIOS patients had a significantly higher incidence of meconium ileus in the past (65.4% vs 0%, respectively, P < 0.02), more Aspergillus spp. colonization (34.6% vs 3.2%, respectively, P < 0

  7. Breakthrough Therapies: Cystic Fibrosis (CF) Potentiators and Correctors

    PubMed Central

    Solomon, George M.; Marshall, Susan G.; Ramsey, Bonnie W.; Rowe, Steven M.

    2015-01-01

    Cystic Fibrosis is caused by mutations in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene resulting in abnormal protein function. Recent advances of targeted molecular therapies and high throughput screening have resulted in multiple drug therapies that target many important mutations in the CFTR protein. In this review, we provide the latest results and current progress of CFTR modulators for the treatment of cystic fibrosis, focusing on potentiators of CFTR channel gating and Phe508del processing correctors for the Phe508del CFTR mutation. Special emphasis is placed on the molecular basis underlying these new therapies and emerging results from the latest clinical trials. The future directions for augmenting the rescue of Phe508del with CFTR modulators is also emphasized. PMID:26097168

  8. Benign cystic lesions of the vagina: a literature review.

    PubMed

    Eilber, Karyn Schlunt; Raz, Shlomo

    2003-09-01

    Knowledge of the etiology, evaluation and treatment of cystic lesions of the vagina is essential as these lesions are often encountered in urological and gynecological practices. We searched MEDLINE and MeSH for literature from the last 50 years referring to cysts of the vagina. Review of the literature regarding etiology, clinical and pathological diagnosis, prognosis and treatment identified 18 journal articles and 6 books. Benign cystic lesions of the vagina present a spectrum, from small asymptomatic lesions to cysts large enough to cause urinary obstruction. History, physical examination and radiological imaging, including voiding cystourethrogram and magnetic resonance imaging, are useful in diagnosis. Treatment is determined by the severity of symptoms. Cystic lesions of the vagina are relatively common and usually represent benign conditions. A vaginal cyst may be an embryological derivative, ectopic tissue or urological abnormality. Awareness of the various diagnoses of benign cystic lesions of the vagina and associated abnormalities will aid in evaluation and treatment.

  9. Intimal cell masses in the abdominal aortas of swine fed a low-fat, low-cholesterol diet for up to twelve years of age.

    PubMed

    Kim, D N; Schmee, J; Lee, K T; Thomas, W A

    1985-05-01

    The normal subendothelial intima of large arteries in man, swine and most other species is a variegated structure from birth onwards. In some regions it contains only a few scattered cells; in others there may be a continuous single layer of cells; and in still others the cells pile up to form what we have called intimal cell masses (ICM). The cells in the normal ICM are mostly smooth muscle cells although there is also a small resident population of monocyte-like cells. We have been studying the ICM in swine with emphasis on the abdominal aorta. We have found that atherosclerotic lesions in the abdominal aorta of swine induced by high-fat high-cholesterol diets begin by a hyperplastic reaction of the smooth muscle cells in the ICM and progress to form large lesions characterized by extensive regions of lipid-rich calcific necrotic debris similar to advanced lesions in man. Because of the putative key role of the ICM in atherogenesis we think that it is important to learn as much as possible about their natural history under conditions as normal as possible. In this report we present data on ICM in the abdominal aortas of 34 male and female Hormel miniature swine maintained on a low-fat low-cholesterol diet for up to 12 years of age. The ICM grow slowly with aging and in the distal portion of the aorta account for an average of 9% in the male and 15% in the female of the total cells in the aortic wall (intima + media).(ABSTRACT TRUNCATED AT 250 WORDS)

  10. Cystic Benign Metastasizing Leiomyoma of the Lung Mimicking Hydatid Cyst.

    PubMed

    Alimi, Faouzi; El Hadj Sidi, Chighali; Ghannouchi, Chams

    2016-12-01

    A 60-year-old woman, with history of a resected uterine benign leiomyoma, is operated for a cystic lesion in the left lung, mimicking an hydatid cyst, and confirmed histologically as Cystic Benign Metastasizing Leiomyoma.

  11. Recent progress in translational cystic fibrosis research using precision medicine strategies.

    PubMed

    Cholon, Deborah M; Gentzsch, Martina

    2018-03-01

    Significant progress has been achieved in developing precision therapies for cystic fibrosis; however, highly effective treatments that target the ion channel, CFTR, are not yet available for many patients. As numerous CFTR therapeutics are currently in the clinical pipeline, reliable screening tools capable of predicting drug efficacy to support individualized treatment plans and translational research are essential. The utilization of bronchial, nasal, and rectal tissues from individual cystic fibrosis patients for drug testing using in vitro assays such as electrophysiological measurements of CFTR activity and evaluation of fluid movement in spheroid cultures, has advanced the prediction of patient-specific responses. However, for precise prediction of drug effects, in vitro models of CFTR rescue should incorporate the inflamed cystic fibrosis airway environment and mimic the complex tissue structures of airway epithelia. Furthermore, novel assays that monitor other aspects of successful CFTR rescue such as restoration of mucus characteristics, which is important for predicting mucociliary clearance, will allow for better prognoses of successful therapies in vivo. Additional cystic fibrosis treatment strategies are being intensively explored, such as development of drugs that target other ion channels, and novel technologies including pluripotent stem cells, gene therapy, and gene editing. The multiple therapeutic approaches available to treat the basic defect in cystic fibrosis combined with relevant precision medicine models provide a framework for identifying optimal and sustained treatments that will benefit all cystic fibrosis patients. Copyright © 2017 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

  12. Classification of malnutrition in cystic fibrosis: implications for evaluating and benchmarking clinical practice performance2

    PubMed Central

    HuiChuan, J Lai; Suzanne, M Shoff

    2008-01-01

    Background In 2005, the Cystic Fibrosis Foundation (CFF) revised the nutrition classification guidelines to eliminate the use of percentage of ideal body weight (%IBW) to define “nutritional failure”; the CFF also recommended that children with cystic fibrosis maintain a body mass index percentile (BMIp) ≥ 50th. Objective We assessed the effect of the 2005 CFF nutrition classification guidelines on evaluating the performance of nutritional care practices. Design Data from 14 702 children reported to the 2002 CFF Patient Registry were analyzed to compare malnutrition rates in 113 cystic fibrosis centers in the United States. Nutritional failure was defined according to the 2002 CFF criteria—ie, height < 5th percentile, %IBW < 90%, or BMIp < 10th. “Below BMI goal” was defined according to the 2005 CFF criterion, ie BMIp < 50th. Results Eliminating %IBW resulted in a 6% reduction (from 33% to 27%) in the nutritional failure rate in the United States. The use of BMIp < 50th led to the classification of 57% of children as below the BMI goal. Misclassification of nutritional failure according to %IBW ranged from 1% to 16% among 113 centers and was greater in the centers with a larger proportion of tall patients. After the elimination of %IBW, one-third of centers changed to a different tertile ranking for nutritional failure rates (kappa = 0.50, moderate-to-poor agreement). More than half the centers changed to a different tertile ranking, from nutritional failure to below BMI goal (kappa = 0.22, poor agreement). Conclusion Eliminating misclassification by %IBW and implementing the new BMI goal led to profound and unequal changes in malnutrition rates across cystic fibrosis centers. PMID:18614737

  13. Adenoid cystic carcinoma: An unusual presentation

    PubMed Central

    Pushpanjali, M; Sujata, D Naga; Subramanyam, S Bala; Jyothsna, M

    2014-01-01

    The adenoid cystic carcinoma is a relatively rare epithelial tumor of the major and minor salivary glands, accounting for about 1% of all malignant tumor of the oral and maxillofacial regions. Peak incidence occurs between the 5th and 6th decades of life. The clinical and pathological findings typical of this tumor include slow growth, peri-neural invasion, multiple local recurrences and distant metastasis. Herein, we report a case of adenoid cystic carcinoma of oropharynx with unusual clinical presentation. The diagnosis of this case and importance of cytology in diagnosing such cases is discussed. PMID:25328314

  14. Don't Forget the Abdominal Wall: Imaging Spectrum of Abdominal Wall Injuries after Nonpenetrating Trauma.

    PubMed

    Matalon, Shanna A; Askari, Reza; Gates, Jonathan D; Patel, Ketan; Sodickson, Aaron D; Khurana, Bharti

    2017-01-01

    Abdominal wall injuries occur in nearly one of 10 patients coming to the emergency department after nonpenetrating trauma. Injuries range from minor, such as abdominal wall contusion, to severe, such as abdominal wall rupture with evisceration of abdominal contents. Examples of specific injuries that can be detected at cross-sectional imaging include abdominal muscle strain, tear, or hematoma, including rectus sheath hematoma (RSH); traumatic abdominal wall hernia (TAWH); and Morel-Lavallée lesion (MLL) (closed degloving injury). These injuries are often overlooked clinically because of (a) a lack of findings at physical examination or (b) distraction by more-severe associated injuries. However, these injuries are important to detect because they are highly associated with potentially grave visceral and vascular injuries, such as aortic injury, and because their detection can lead to the diagnosis of these more clinically important grave traumatic injuries. Failure to make a timely diagnosis can result in delayed complications, such as bowel hernia with potential for obstruction or strangulation, or misdiagnosis of an abdominal wall neoplasm. Groin injuries, such as athletic pubalgia, and inferior costochondral injuries should also be considered in patients with abdominal pain after nonpenetrating trauma, because these conditions may manifest with referred abdominal pain and are often included within the field of view at cross-sectional abdominal imaging. Radiologists must recognize and report acute abdominal wall injuries and their associated intra-abdominal pathologic conditions to allow appropriate and timely treatment. © RSNA, 2017.

  15. Relationship of abdominal obesity with cardiovascular disease, diabetes and hyperlipidaemia in Spain.

    PubMed

    Casanueva, Felipe F; Moreno, Basilio; Rodríguez-Azeredo, Rosario; Massien, Christine; Conthe, Pedro; Formiguera, Xavier; Barrios, Vivencio; Balkau, Beberly

    2010-07-01

    To evaluate the relevance of obesity and abdominal obesity in the prevalence of cardiovascular disease (CVD), diabetes mellitus, hyperlipidaemia and hypertension in primary care patients and to ascertain whether waist circumference (WC) measurement should be included in routine clinical practice in addition to body mass index (BMI). As part of the IDEA study, primary care physicians from Spain recruited patients aged 18-80 years. WC and BMI and the presence of CVD, diabetes mellitus, hyperlipidaemia and hypertension were recorded. Finally, 17 980 were analysed. An age-related increase in adiposity was observed. Overall 33% were obese by BMI, and 51% of subjects presented abdominal obesity by the National Cholesterol Education Program Adult Treatment Panel III (NCEP-ATPIII) (WC > 102 cm for men and > 88 cm for women). Although there was a correlation between BMI and WC, they presented different distribution patterns. Women, but not men, with a high level of education, professional activity and smoking were associated with a lower WC. Abdominal obesity was significantly associated with CVD. Some subjects with abdominal obesity but lean by BMI, showed an increased prevalence of CVD and diabetes. Furthermore, abdominal obesity was strongly associated with dyslipidaemia and hypertension. Half of the primary care patients studied showed abdominal obesity as measured by WC, whereas one-third was obese by BMI. Abdominal obesity was strongly associated with CVD and diabetes, even in patients lean by BMI. WC should be included in the routine clinical practice in addition to BMI.

  16. Correlation between intra-abdominal pressure and pulmonary volumes after superior and inferior abdominal surgery.

    PubMed

    Cleva, Roberto de; Assumpção, Marianna Siqueira de; Sasaya, Flavia; Chaves, Natalia Zuniaga; Santo, Marco Aurelio; Fló, Claudia; Lunardi, Adriana C; Jacob Filho, Wilson

    2014-07-01

    Patients undergoing abdominal surgery are at risk for pulmonary complications. The principal cause of postoperative pulmonary complications is a significant reduction in pulmonary volumes (FEV1 and FVC) to approximately 65-70% of the predicted value. Another frequent occurrence after abdominal surgery is increased intra-abdominal pressure. The aim of this study was to correlate changes in pulmonary volumes with the values of intra-abdominal pressure after abdominal surgery, according to the surgical incision in the abdomen (superior or inferior). We prospectively evaluated 60 patients who underwent elective open abdominal surgery with a surgical time greater than 240 minutes. Patients were evaluated before surgery and on the 3rd postoperative day. Spirometry was assessed by maximal respiratory maneuvers and flow-volume curves. Intra-abdominal pressure was measured in the postoperative period using the bladder technique. The mean age of the patients was 56 ± 13 years, and 41.6% 25 were female; 50 patients (83.3%) had malignant disease. The patients were divided into two groups according to the surgical incision (superior or inferior). The lung volumes in the preoperative period showed no abnormalities. After surgery, there was a significant reduction in both FEV1 (1.6 ± 0.6 L) and FVC (2.0 ± 0.7 L) with maintenance of FEV1/FVC of 0.8 ± 0.2 in both groups. The maximum intra-abdominal pressure values were similar (p=0.59) for the two groups. There was no association between pulmonary volumes and intra-abdominal pressure measured in any of the groups analyzed. Our results show that superior and inferior abdominal surgery determines hypoventilation, unrelated to increased intra-abdominal pressure. Patients at high risk of pulmonary complications should receive respiratory care even if undergoing inferior abdominal surgery.

  17. Cystic tumors of the liver: A practical approach

    PubMed Central

    Poggio, Paolo Del; Buonocore, Marco

    2008-01-01

    Biliary cyst tumors (cystadenoma and cystadeno-carcinoma) are an indication for liver resection. They account for only 5% of all solitary cystic lesions of the liver, but differential diagnosis with multiloculated or complicated biliary cysts, atypical hemangiomas, hamartomas and lymphangiomas may be difficult. The most frequent challenge is to differentiate biliary cyst tumors from hemorrhagic cysts. Computerized tomography (CT) and magnetic resonance imaging (MRI) are often not diagnostic and in these cases fine needle aspiration (FNA) is used to confirm the presence of atypical biliary cells. FNA, however, lacks adequate sensitivity and specificity and should always be used in conjunction with imaging. Pre-operative differentiation of cystadenoma from cystadenocarcinoma is impossible and surgery must be performed if a biliary cyst tumor is suspected. When multiple cystic lesions are observed throughout the liver parenchyma, it is important to exclude liver metastasis, of which colonic cancer is the most common primary site. Multiple biliary hamartomas (von Meyenburg complex) can appear as a mixture of solid and cystic lesions and can be confused with cystic metastasis. Strong and uniform T2 hyperintensity on MRI is usually diagnostic, but occasionally a percutaneous biopsy may be required. PMID:18595127

  18. Predictive 5-Year Survivorship Model of Cystic Fibrosis

    PubMed Central

    Liou, Theodore G.; Adler, Frederick R.; FitzSimmons, Stacey C.; Cahill, Barbara C.; Hibbs, Jonathan R.; Marshall, Bruce C.

    2007-01-01

    The objective of this study was to create a 5-year survivorship model to identify key clinical features of cystic fibrosis. Such a model could help researchers and clinicians to evaluate therapies, improve the design of prospective studies, monitor practice patterns, counsel individual patients, and determine the best candidates for lung transplantation. The authors used information from the Cystic Fibrosis Foundation Patient Registry (CFFPR), which has collected longitudinal data on approximately 90% of cystic fibrosis patients diagnosed in the United States since 1986. They developed multivariate logistic regression models by using data on 5,820 patients randomly selected from 11,630 in the CFFPR in 1993. Models were tested for goodness of fit and were validated for the remaining 5,810 patients for 1993. The validated 5-year survivorship model included age, forced expiratory volume in 1 second as a percentage of predicted normal, gender, weight-for-age z score, pancreatic sufficiency, diabetes mellitus, Staphylococcus aureus infection, Burkerholderia cepacia infection, and annual number of acute pulmonary exacerbations. The model provides insights into the complex nature of cystic fibrosis and supplies a rigorous tool for clinical practice and research. PMID:11207152

  19. New and emerging targeted therapies for cystic fibrosis

    PubMed Central

    Rowe, Steven M

    2016-01-01

    Cystic fibrosis (CF) is a monogenic autosomal recessive disorder that affects about 70 000 people worldwide. The clinical manifestations of the disease are caused by defects in the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The discovery of the CFTR gene in 1989 has led to a sophisticated understanding of how thousands of mutations in the CFTR gene affect the structure and function of the CFTR protein. Much progress has been made over the past decade with the development of orally bioavailable small molecule drugs that target defective CFTR proteins caused by specific mutations. Furthermore, there is considerable optimism about the prospect of gene replacement or editing therapies to correct all mutations in cystic fibrosis. The recent approvals of ivacaftor and lumacaftor represent the genesis of a new era of precision medicine in the treatment of this condition. These drugs are having a positive impact on the lives of people with cystic fibrosis and are potentially disease modifying. This review provides an update on advances in our understanding of the structure and function of the CFTR, with a focus on state of the art targeted drugs that are in development. PMID:27030675

  20. What is Cystic Fibrosis?

    MedlinePlus

    ... pass the faulty CFTR gene to their children. Example of an Inheritance Pattern for Cystic Fibrosis The image shows how CFTR genes are inherited. A person inherits two copies of the CFTR gene—one from each parent. If each parent has a ...

  1. Early bronchiectasis in cystic fibrosis detected by surveillance CT.

    PubMed

    Pillarisetti, Naveen; Linnane, Barry; Ranganathan, Sarath

    2010-08-01

    There is emerging evidence that cystic fibrosis lung disease begins early in infancy. Newborn screening allows early detection and surveillance of pulmonary disease and the possibility of early intervention in this life-shortening condition. We report two children with cystic fibrosis who underwent a comprehensive assessment from diagnosis that included measurement of lung function, limited-slice high-resolution CT and BAL performed annually. Early aggressive surveillance enabled significant lung disease and bronchiectasis to be detected during the first few years of life and led to a change in management, highlighting a clinical role for CT scanning during the preschool years in children with cystic fibrosis.

  2. Drug therapies for reducing gastric acidity in people with cystic fibrosis.

    PubMed

    Ng, Sze May; Moore, Helen S

    2016-08-22

    Malabsorption of fat and protein contributes to poor nutritional status in people with cystic fibrosis. Impaired pancreatic function may also result in increased gastric acidity, leading in turn to heartburn, peptic ulcers and the impairment of oral pancreatic enzyme replacement therapy. The administration of gastric acid-reducing agents has been used as an adjunct to pancreatic enzyme therapy to improve absorption of fat and gastro-intestinal symptoms in people with cystic fibrosis. It is important to establish the evidence regarding potential benefits of drugs that reduce gastric acidity in people with cystic fibrosis. This is an update of a previously published review. To assess the effect of drug therapies for reducing gastric acidity for: nutritional status; symptoms associated with increased gastric acidity; fat absorption; lung function; quality of life and survival; and to determine if any adverse effects are associated with their use. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches, handsearches of relevant journals, abstract books and conference proceedings.Most recent search of the Group's Trials Register: 12 May 2016. All randomised and quasi-randomised trials involving agents that reduce gastric acidity compared to placebo or a comparator treatment. Both authors independently selected trials, assessed trial quality and extracted data. The searches identified 39 trials; 17 of these, with 273 participants, were suitable for inclusion, but the number of trials assessing each of the different agents was small. Seven trials were limited to children and four trials enrolled only adults. Meta-analysis was not performed, 14 trials were of a cross-over design and we did not have the appropriate information to conduct comprehensive meta-analyses. All the trials were run in single centres and duration ranged from five days to six months. The

  3. Pharmacogenetics of cystic fibrosis treatment.

    PubMed

    Carter, Suzanne C; McKone, Edward F

    2016-08-01

    Cystic fibrosis (CF) is genetic autosomal recessive disease caused by reduced or absent function of CFTR protein. Treatments for patients with CF have primarily focused on the downstream end-organ consequences of defective CFTR. Since the discovery of the CFTR gene that causes CF in 1989 there have been tremendous advances in our understanding of the genetics and pathophysiology of CF. This has recently led to the development of new CFTR mutation-specific targeted therapies for select patients with CF. This review will discuss the characteristics of the CFTR gene, the CFTR mutations that cause CF and the new mutation specific pharmacological treatments including gene therapy that are contributing to the dawning of a new era in cystic fibrosis care.

  4. Respiratory infections in patients with cystic fibrosis undergoing lung transplantation.

    PubMed

    Lobo, Leonard J; Noone, Peadar G

    2014-01-01

    Cystic fibrosis is an inherited disease characterised by chronic respiratory infections associated with bronchiectasis. Lung transplantation has helped to extend the lives of patients with cystic fibrosis who have advanced lung disease. However, persistent, recurrent, and newly acquired infections can be problematic. Classic cystic fibrosis-associated organisms, such as Staphylococcus aureus and Pseudomonas aeruginosa, are generally manageable post-transplantation, and are associated with favourable outcomes. Burkholderia cenocepacia poses particular challenges, although other Burkholderia species are less problematic. Despite concerns about non-tuberculous mycobacteria, especially Mycobacterium abscessus, post-transplantation survival has not been definitively shown to be less than average in patients with these infections. Fungal species can be prevalent before and after transplantation and are associated with high morbidity, so should be treated aggressively. Appropriate viral screening and antiviral prophylaxis are necessary to prevent infection with and reactivation of Epstein-Barr virus and cytomegalovirus and their associated complications. Awareness of drug pharmacokinetics and interactions in cystic fibrosis is crucial to prevent toxic effects and subtherapeutic or supratherapeutic drug dosing. With the large range of potential infectious organisms in patients with cystic fibrosis, infection control in hospital and outpatient settings is important. Despite its complexity, lung transplantation in the cystic fibrosis population is safe, with good outcomes if the clinician is aware of all the potential pathogens and remains vigilant by means of surveillance and proactive treatment. Copyright © 2014 Elsevier Ltd. All rights reserved.

  5. Lung function imaging methods in Cystic Fibrosis pulmonary disease.

    PubMed

    Kołodziej, Magdalena; de Veer, Michael J; Cholewa, Marian; Egan, Gary F; Thompson, Bruce R

    2017-05-17

    Monitoring of pulmonary physiology is fundamental to the clinical management of patients with Cystic Fibrosis. The current standard clinical practise uses spirometry to assess lung function which delivers a clinically relevant functional readout of total lung function, however does not supply any visible or localised information. High Resolution Computed Tomography (HRCT) is a well-established current 'gold standard' method for monitoring lung anatomical changes in Cystic Fibrosis patients. HRCT provides excellent morphological information, however, the X-ray radiation dose can become significant if multiple scans are required to monitor chronic diseases such as cystic fibrosis. X-ray phase-contrast imaging is another emerging X-ray based methodology for Cystic Fibrosis lung assessment which provides dynamic morphological and functional information, albeit with even higher X-ray doses than HRCT. Magnetic Resonance Imaging (MRI) is a non-ionising radiation imaging method that is garnering growing interest among researchers and clinicians working with Cystic Fibrosis patients. Recent advances in MRI have opened up the possibilities to observe lung function in real time to potentially allow sensitive and accurate assessment of disease progression. The use of hyperpolarized gas or non-contrast enhanced MRI can be tailored to clinical needs. While MRI offers significant promise it still suffers from poor spatial resolution and the development of an objective scoring system especially for ventilation assessment.

  6. [Treatment of autonomous and cystic thyroid nodules with intranodular ethanol injection].

    PubMed

    Braga-Basaria, Milena; Trippia, Marcus Adriano; Stolf, Anderson Ravy; Mesa, Cléo; Graf, Hans

    2002-01-01

    Intranodular ethanol injection has been used for the past 10 years as an efficient modality for treating patients with thyroid nodules. Several studies have reported the success of this therapy in autonomous and cystic nodules and, more recently, in cold benign nodules. To evaluate the efficacy of this therapeutic modality on the treatment of autonomous and cystic thyroid nodules. 42 patients (26 with cystic and 16 with autonomous nodules) were treated with ultrasound guided intranodular 99% ethanol injection and followed for 6 months. No major complications were observed during or after treatment, however, most of the patients reported slight to moderate pain and/or discomfort after the injection. Most of the nodules showed reduction after the treatment. Autonomous nodules had a mean reduction of 50.3% and cystic nodules of 69.3%. No significant differences in pretreatment serum total T3, total T4 or TSH were observed among the patients in the cystic group. Patients in the autonomous group with hyperfunctioning nodules showed a decrease in serum total T3, total T4 and an increase in serum TSH levels, hence, proving the effectiveness of this therapy. Intranodular ethanol injection is a safe and efficient treatment for autonomous and cystic nodules of the thyroid.

  7. The subcutaneous abdominal fat and not the intraabdominal fat compartment is associated with anovulation in women with obesity and infertility.

    PubMed

    Kuchenbecker, Walter K H; Groen, Henk; Zijlstra, Tineke M; Bolster, Johanna H T; Slart, Riemer H J; van der Jagt, Erik J; Kobold, Anneke C Muller; Wolffenbuttel, Bruce H R; Land, Jolande A; Hoek, Annemieke

    2010-05-01

    Abdominal fat contributes to anovulation. We compared body fat distribution measurements and their contribution to anovulation in obese ovulatory and anovulatory infertile women. Seventeen ovulatory and 40 anovulatory women (age, 30 +/- 4 yr; body mass index, 37.7 +/- 6.1 kg/m(2)) participated. Body fat distribution was measured by anthropometrics, dual-energy x-ray absorptiometry, and single-sliced abdominal computed tomography scan. Multiple logistic regression analysis was applied to determine which fat compartments significantly contributed to anovulation. Anovulatory women had a higher waist circumference (113 +/- 11 vs. 104 +/- 9 cm; P < 0.01) and significantly more trunk fat (23.0 +/- 5.3 vs. 19.1 +/- 4.2 kg; P < 0.01) and abdominal fat (4.4 +/- 1.3 kg vs. 3.5 +/- 0.9 kg; P < 0.05) on dual-energy x-ray absorptiometry scan than ovulatory women despite similar body mass index. The volume of intraabdominal fat on single-sliced abdominal computed tomography scan was not significantly different between the two groups (203 +/- 56 vs. 195 +/- 71 cm(3); P = 0.65), but anovulatory women had significantly more sc abdominal fat (SAF) (992 +/- 198 vs. 864 +/- 146 cm(3); P < 0.05). After multiple logistic regression analysis, only trunk fat, abdominal fat, and SAF were associated with anovulation. Abdominal fat is increased in anovulatory women due to a significant increase in SAF and not in intraabdominal fat. SAF and especially abdominal and trunk fat accumulation are associated with anovulation.

  8. Different optical spectral characteristics in a necrotic transmissible venereal tumor and a cystic lesion in the same canine prostate observed by triple-band trans-rectal optical tomography under trans-rectal ultrasound guidance

    NASA Astrophysics Data System (ADS)

    Jiang, Zhen; Holyoak, G. Reed; Ritchey, Jerry W.; Bartels, Kenneth E.; Rock, Kendra; Ownby, Charlotte L.; Slobodov, Gennady; Bunting, Charles F.; Piao, Daqing

    2011-03-01

    Different optical spectral characteristics were observed in a necrotic transmissible venereal tumor (TVT) and a cystic lesion in the same canine prostate by triple-wavelength trans-rectal optical tomography under trans-rectal ultrasound (TRUS) guidance. The NIR imager acquiring at 705nm, 785nm and 808nm was used to quantify both the total hemoglobin concentration (HbT) and oxygen saturation (StO2) in the prostate. The TVT tumor in the canine prostate as a model of prostate cancer was induced in a 7-year old, 27 kg dog. A 2 mL suspension of 2.5x106 cells/mL of homogenized TVT cells recovered from an in vivo subcutaneously propagated TVT tumor in an NOD/SCID mouse were injected in the cranial aspect of the right lobe of the canine prostate. The left lobe of the prostate had a cystic lesion present before TVT inoculation. After the TVT homogenate injection, the prostate was monitored weekly over a 9-week period, using trans-rectal NIR and TRUS in grey-scale and Doppler. A TVT mass within the right lobe developed a necrotic center during the later stages of this study, as the mass presented with substantially increased [HbT] in the periphery, with an area of reduced StO2 less than the area of the mass itself shown on ultrasonography. Conversely, the cystic lesion presented with slightly increased [HbT] in the periphery of the lesion shown on ultrasound with oxygen-reduction inside and in the periphery of the lesion. There was no detectable change of blood flow on Doppler US in the periphery of the cystic lesion. The slightly increased [HbT] in the periphery of the cystic lesion was correlated with intra-lesional hemorrhage upon histopathologic examination.

  9. Bone Mineral Density of Indian Children and Adolescents with Cystic Fibrosis.

    PubMed

    Gupta, Sumita; Mukherjee, Aparna; Khadgawat, Rajesh; Kabra, Madhulika; Lodha, Rakesh; Kabra, Sushil K

    2017-07-15

    To document bone mineral density of children and adolescents with cystic fibrosis. Cross-sectional study. Tertiary-care center of Northern India, July 2012 to August 2015. 52 children aged 6-18 years with cystic fibrosis and 62 healthy controls of similar age and sex. Both patients and controls were stratified into two groups, as pre-pubertal and peri-/post-pubertal, and compared for whole body bone mineral density, measured using dual energy X-ray absorptiometry. Serum levels of calcium, phosphate, alkaline phosphatase, 25-hydroxyvitamin D and parathyroid hormone were measured in children with cystic fibrosis. Compared with controls, the mean (SD) bone mineral density of children with cystic fibrosis was significantly lower in both the pre-pubertal (0.7 (0.1) g/cm2 vs 0.9 (0.1) g/cm2; P<0.001)) and peri-/post-pubertal groups (0.9 (0.1) g/cm2 vs 1.1 (0.1) g/cm2; P<0.001). Also, the mean (SD) bone mineral apparent density of pre-pubertal and peri-/post-pubertal cystic fibrosis patients was lower than the controls (P <0.001 and P= 0.01, respectively). Thirty-seven (71.2%) cystic fibrosis patients had serum 25-hydroxyvitamin D level below 15 ng/mL. Bone mineral density of children with cystic fibrosis was significantly lower than controls; majority of them were vitamin-D deficient. Intervening at an early stage of the disease and providing optimal therapy involving simultaneous management of the several factors affecting bone mineral accretion may be beneficial in improving bone health of these patients.

  10. Chronic Abdominal Wall Pain.

    PubMed

    Koop, Herbert; Koprdova, Simona; Schürmann, Christine

    2016-01-29

    Chronic abdominal wall pain is a poorly recognized clinical problem despite being an important element in the differential diagnosis of abdominal pain. This review is based on pertinent articles that were retrieved by a selective search in PubMed and EMBASE employing the terms "abdominal wall pain" and "cutaneous nerve entrapment syndrome," as well as on the authors' clinical experience. In 2% to 3% of patients with chronic abdominal pain, the pain arises from the abdominal wall; in patients with previously diagnosed chronic abdominal pain who have no demonstrable pathological abnormality, this likelihood can rise as high as 30% . There have only been a small number of clinical trials of treatment for this condition. The diagnosis is made on clinical grounds, with the aid of Carnett's test. The characteristic clinical feature is strictly localized pain in the anterior abdominal wall, which is often mischaracterized as a "functional" complaint. In one study, injection of local anesthesia combined with steroids into the painful area was found to relieve pain for 4 weeks in 95% of patients. The injection of lidocaine alone brought about improvement in 83-91% of patients. Long-term pain relief ensued after a single lidocaine injection in 20-30% of patients, after repeated injections in 40-50% , and after combined lidocaine and steroid injections in up to 80% . Pain that persists despite these treatments can be treated with surgery (neurectomy). Chronic abdominal wall pain is easily diagnosed on physical examination and can often be rapidly treated. Any physician treating patients with abdominal pain should be aware of this condition. Further comparative treatment trials will be needed before a validated treatment algorithm can be established.

  11. Oedema is associated with clinical outcome following emergency abdominal surgery.

    PubMed

    Vaughan-Shaw, P G; Saunders, J; Smith, T; King, A T; Stroud, M A

    2013-09-01

    Oedema is observed frequently following surgery and may be associated with worse outcomes. To date, no study has investigated the role of oedema in the emergency surgical patient. This study assesses the incidence of oedema following emergency abdominal surgery and the value of early postoperative oedema measurement in predicting clinical outcome. A prospective cohort study of patients undergoing emergency abdominal surgery at a university unit over a two-month period was undertaken. Nutritional and clinical outcome data were collected and oedema was measured in the early postoperative period. Predictors of oedema and outcomes associated with postoperative oedema were identified through univariate and multivariate analysis. Overall, 55 patients (median age: 66 years) were included in the study. Postoperative morbidity included ileus (n=22) and sepsis (n=6) with 12 deaths at follow-up. Postoperative oedema was present in 19 patients and was associated with prolonged perioperative fasting (107 vs 30 hours, p=0.009) but not with body mass index (24 kg/m(2) vs 27 kg/m(2), p=0.169) or preadmission weight loss (5% vs 3%, p=0.923). On multivariate analysis, oedema was independently associated with gastrointestinal recovery (B=6.91, p=0.038), artificial nutritional support requirement (odds ratio: 6.91, p=0.037) and overall survival (χ(2) =13.1, df=1, p=0.001). Generalised oedema is common after emergency abdominal surgery and appears to independently predict gastrointestinal recovery, the need for artificial nutritional support and survival. Oedema is not associated with commonly applied markers of nutritional status such as body mass index or recent weight loss. Measurement of oedema offers utility in identifying those at risk of poor clinical outcome or those requiring artificial nutritional support following emergency abdominal surgery.

  12. Oedema is associated with clinical outcome following emergency abdominal surgery

    PubMed Central

    Vaughan-Shaw, PG; Saunders, J; Smith, T; King, AT

    2013-01-01

    Introduction Oedema is observed frequently following surgery and may be associated with worse outcomes. To date, no study has investigated the role of oedema in the emergency surgical patient. This study assesses the incidence of oedema following emergency abdominal surgery and the value of early postoperative oedema measurement in predicting clinical outcome. Methods A prospective cohort study of patients undergoing emergency abdominal surgery at a university unit over a two-month period was undertaken. Nutritional and clinical outcome data were collected and oedema was measured in the early postoperative period. Predictors of oedema and outcomes associated with postoperative oedema were identified through univariate and multivariate analysis. Results Overall, 55 patients (median age: 66 years) were included in the study. Postoperative morbidity included ileus (n=22) and sepsis (n=6) with 12 deaths at follow-up. Postoperative oedema was present in 19 patients and was associated with prolonged perioperative fasting (107 vs 30 hours, p=0.009) but not with body mass index (24kg/m2 vs 27kg/m2, p=0.169) or preadmission weight loss (5% vs 3%, p=0.923). On multivariate analysis, oedema was independently associated with gastrointestinal recovery (B=6.91, p=0.038), artificial nutritional support requirement (odds ratio: 6.91, p=0.037) and overall survival (χ2=13.1, df=1, p=0.001). Conclusions Generalised oedema is common after emergency abdominal surgery and appears to independently predict gastrointestinal recovery, the need for artificial nutritional support and survival. Oedema is not associated with commonly applied markers of nutritional status such as body mass index or recent weight loss. Measurement of oedema offers utility in identifying those at risk of poor clinical outcome or those requiring artificial nutritional support following emergency abdominal surgery. PMID:24025285

  13. Insulin therapy in patients with cystic fibrosis in the pre-diabetes stage: a systematic review.

    PubMed

    Pu, Mariana Zorrón Mei Hsia; Christensen-Adad, Flávia Corrêa; Gonçalves, Aline Cristina; Minicucci, Walter José; Ribeiro, José Dirceu; Ribeiro, Antonio Fernando

    2016-09-01

    To elucidate whether insulin is effective or not in patients with cystic fibrosis before the diabetes mellitus phase. The study was performed according to the Prisma method between August and September 2014, using the PubMed, Embase, Lilacs and SciELO databases. Prospective studies published in English, Portuguese and Spanish from 2002 to 2014, evaluating the effect of insulin on weight parameters, body mass index and pulmonary function in patients with cystic fibrosis, with a mean age of 17.37 years before the diabetes mellitus phase were included. Eight articles were identified that included 180 patients undergoing insulin use. Sample size ranged from 4 to 54 patients, with a mean age ranging from 12.4 to 28 years. The type of follow-up, time of insulin use, the dose and implementation schedule were very heterogeneous between studies. There are theoretical reasons to believe that insulin has a beneficial effect in the studied population. The different methods and populations assessed in the studies do not allow us to state whether early insulin therapy should or should not be carried out in patients with cystic fibrosis prior to the diagnosis of diabetes. Therefore, studies with larger samples and insulin use standardization are required. Copyright © 2016 Sociedade de Pediatria de São Paulo. Publicado por Elsevier Editora Ltda. All rights reserved.

  14. Cystic fibrosis: a mucosal immunodeficiency syndrome

    PubMed Central

    Cohen, Taylor Sitarik; Prince, Alice

    2013-01-01

    Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that regulates the transport of ions and the movement of water across the epithelial barrier. Mutations in CFTR, which form the basis for the clinical manifestations of cystic fibrosis, affect the epithelial innate immune function in the lung, resulting in exaggerated and ineffective airway inflammation that fails to eradicate pulmonary pathogens. Compounding the effects of excessive neutrophil recruitment, the mutant CFTR channel does not transport antioxidants to counteract neutrophil-associated oxidative stress. Whereas mutant CFTR expression in leukocytes outside of the lung does not markedly impair their function, the expected regulation of inflammation in the airways is clearly deficient in cystic fibrosis. The resulting bacterial infections, which are caused by organisms that have substantial genetic and metabolic flexibility, can resist multiple classes of antibiotics and evade phagocytic clearance. The development of animal models that approximate the human pulmonary phenotypes—airway inflammation and spontaneous infection—may provide the much-needed tools to establish how CFTR regulates mucosal immunity and to test directly the effect of pharmacologic potentiation and correction of mutant CFTR function on bacterial clearance. PMID:22481418

  15. Mutation spectrum and differential gene expression in cystic and solid vestibular schwannoma.

    PubMed

    Zhang, Zhihua; Wang, Zhaoyan; Sun, Lianhua; Li, Xiaohua; Huang, Qi; Yang, Tao; Wu, Hao

    2014-03-01

    We sought to characterize the mutation spectrum of NF2 and the differential gene expression in cystic and solid vestibular schwannomas. We collected tumor tissue and blood samples of 31 cystic vestibular schwannomas and 114 solid vestibular schwannomas. Mutation screening of NF2 was performed in both tumor and blood DNA samples of all patients. cDNA microarray was used to analyze the differential gene expression between 11 cystic vestibular schwannomas and 6 solid vestibular schwannomas. Expression levels of top candidate genes were verified by quantitative reverse transcription PCR. NF2 mutations were identified in 34.5% of sporadic vestibular schwannomas, with all mutations being exclusively somatic. No significant difference was found between the mutation detection rates of cystic vestibular schwannoma (35.5%) and solid vestibular schwannoma (34.2%). cDNA microarray analysis detected a total of 46 differentially expressed genes between the cystic vestibular schwannoma and solid vestibular schwannoma samples. The significantly decreased expression of four top candidate genes, C1orf130, CNTF, COL4A3, and COL4A4, was verified by quantitative reverse transcription PCR. NF2 mutations are not directly involved in the cystic formation of vestibular schwannoma. In addition, the differential gene expression of cystic vestibular schwannoma reported in our study may provide useful insights into the molecular mechanism underlying this process.

  16. Solitary Hepatocellular Carcinoma Fed by the Cystic Artery: Limitation of Transcatheter Arterial Embolization

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hirota, Shozo; Matsumoto, Shinichi; Fukuda, Tetsuya

    1999-05-15

    Purpose: To clarify the limitations of transcatheter treatment for hepatocellular carcinoma (HCC) with parasitic feeders from the cystic artery. Methods: Three male patients had a solitary HCC (average diameter 3 cm) fed by the cystic artery among 221 patients with HCC from 1994 to 1997. One tumor was nourished entirely from the cystic artery arising from the medial branch of the left hepatic artery, and two tumors were fed partially by the cystic arteries arising from the anterior inferior branch of the right hepatic artery. We analyzed the indications for transcatheter treatment for these three patients. Results: We chose notmore » to embolize the cystic artery for fear of necrosis of the gallbladder. Although embolization of the anterior branch of the right hepatic artery was performed in one patient with a tumor fed partially by the cystic artery, only half the tumor was embolized. Two patients underwent hepatic resection, and one received percutaneous ethanol injection therapy. At follow-up of 28-40 months (average 33 months) all patients are alive. Conclusion: Feeding by the cystic artery represents a limitation of TAE for HCC.« less

  17. Clinicopathological review and survival characteristics of adenoid cystic carcinoma.

    PubMed

    Binesh, Fariba; Akhavan, Ali; Masumi, Omid; Mirvakili, Abbas; Behniafard, Nasim

    2015-03-01

    To study the clinical characters, the outcomes of treatments and the factors affecting treatment results of adenoid cystic carcinomas at Shahid Sadoughi Hospital and Shahid Ramazanzadeh radiotherapy center, Yazd, Iran. The clinical data of 31 patients with adenoid cystic carcinoma of any anatomic site diagnosed over an 8 year period (2004-2012), were investigated retrospectively. Data regarding patients' characteristics, pathological features and follow-up were obtained from patients records. Survival rate, local recurrence and distant metastasis were analyzed using Kaplan-Meier method. Prognosis factors were analyzed by Log-rank test and Cox regression. The study included31 patients with adenoid cystic carcinoma. The mean age at presentation was 50.2 ± 24.8 years. There were 11 (35.5 %) males and 20 (64.5 %) females with a female predilection (M:F = 0.55:1). Parotid gland was the most common site (8/31, 25.7 %) followed by submandibular gland (7/31, 22.6 %). Perineural invasion was detected in 67.7 % of the cases. Positive surgical status was reported in 48.4 % of the specimens. Metastasis was detected in 25.8 % of the patients and the most common site of distant metastasis was lung. Overall survival rates at 2, 5, and 7 years were 95, 75, and 57 % respectively. Margin status showed significant effect on survival (P value = 0.01). Positive surgical margin is an important factor affecting the prognosis of the patients with adenoid cystic carcinoma. Surgery with negative surgical margin is the first choice of treatment for the patients with adenoid cystic carcinoma. Our findings show that the prognosis of patients with adenoid cystic carcinoma in our center is fair.

  18. Exopolysaccharides produced by Inquilinus limosus, a new pathogen of cystic fibrosis patients: novel structures with usual components.

    PubMed

    Herasimenka, Yury; Cescutti, Paola; Impallomeni, Giuseppe; Rizzo, Roberto

    2007-11-26

    The major cause of morbidity and mortality in patients with cystic fibrosis, an autosomal recessive disorder, is chronic microbial colonisation of the major airways that leads to exacerbation of pulmonary infection. Several different microbes colonise cystic fibrosis lungs, and Pseudomonas aeruginosa is one of the most threatening, since the establishment of mucoid (alginate producing) strains is ultimately associated with the patient's death. Very recently a new bacterium, named Inquilinus limosus, was repeatedly found infecting the respiratory tract of cystic fibrosis patients. Its multi-resistance characteristic to antibiotics might result in the spreading of I. limosus infection among the cystic fibrosis community, as recently happened with strains of the Burkholderia cepacia complex. Since exopolysaccharides are recognised as important virulence factors in lung infections, the primary structure of the polysaccharide produced by I. limosus strain LMG 20952(T) was investigated as the first step in understanding its role in pathogenesis. The structure was determined by means of methylation analysis, acid degradations, mass spectrometry and NMR spectroscopy. The results showed that the bacterium produced a mixture constituted of the following polymers: [3)-[4,6-O-(1-carboxyethylidene)]-beta-D-Glcp(1-->]n; [2)-[4,6-O-(1-carboxyethylidene)]-alpha-D-Manp(1-->]n. Both polymers were completely substituted with pyruvyl ketal groups, a novel structural characteristic not previously found in bacterial polysaccharides. The absolute configuration of all pyruvyl groups was S. Inspection of possible local conformations assumed by the two polysaccharide chains showed features, which might provide interesting clues for understanding structure-function relationships.

  19. Identical twins with mature cystic teratomas treated with laparoscopic surgery: Two case reports.

    PubMed

    Mabuchi, Yasushi; Ota, Nami; Kobayashi, Aya; Tanizaki, Yuko; Minami, Sawako; Ino, Kazuhiko

    2017-02-01

    Mature cystic teratomas are the most common among all ovarian neoplasms, representing 30-40% of the cases. However, to the best of our knowledge, there have been only two reports of mature cystic teratomas occurring in identical twins to date. We herein report a case of identical twins with mature cystic teratomas who were treated with laparoscopic surgery. A 32-year-old woman was referred to our hospital due to a tumor in the right ovary. The patient underwent laparoscopic resection of the ovarian tumor and the pathological diagnosis was benign mature cystic teratoma. Two years later, the identical twin of the abovementioned patient was referred to our hospital also due to a right ovarian tumor. The patient underwent laparoscopic resection of the ovarian tumor and the pathological diagnosis was benign mature cystic teratoma. Therefore, for early diagnosis, it may be important to consider the possibility of mature cystic teratoma in the identical twin of a patient, even in the absence of symptoms.

  20. Non-functioning parathyroid cystic tumour: malignant or not? Report of a case.

    PubMed

    Cocorullo, G; Scerrino, G; Melfa, G; Raspanti, C; Rotolo, G; Mannino, V; Richiusa, P; Cabibi, D; Giannone, A G; Porrello, C; Gulotta, G

    2017-01-01

    Parathyroid carcinoma (PC) is a very rare endocrine tumour, usually characterized by symptoms such as a neck mass, dysphonia, severe hypercalcemia exceeding 140 mg/L and elevated serum parathyroid hormone levels, even more than 5 times the upper limit of normal. Non-functioning parathyroid cancer is extremely rare and, in this case, its pre-operative diagnosis is often difficult. A 54-year old female patient, referring dysphagia and dysphonia, underwent neck ultrasound and neck CT. A left thyroid nodule, probably cystic, was found. It presented caudal extent on anterior mediastinum causing compression of the left lateral wall of the trachea. The preoperative calcemia was into the normal range. The patient underwent left thyroid lobectomy. Histological exam showed a cystic lesion, immunohistochemically originating from parathyroid that oriented for carcinoma. The 18 months follow-up did not show a residual-recurrent disease. The parathyroid origin of a neck lesion could not be suspected before surgery when specific laboratory tests are not available and clinical effects of hyperparathyroidism syndrome are not present. Histological features are not always sufficient for the differential diagnosis between the parathyroid adenoma and carcinoma. The immunohistochemistry is an useful tool that can aid to reach the definite diagnosis.

  1. Vitamin A absorption in cystic fibrosis: risk of hypervitaminosis A.

    PubMed Central

    James, D R; Owen, G; Campbell, I A; Goodchild, M C

    1992-01-01

    Vitamin A status was examined in nine adult cystic fibrosis patients and six adult control subjects, together with an assessment of their ability to absorb 10,000 IU of retinyl palmitate from a test meal, taken with appropriate pancreatic enzyme supplements. Median baseline values for plasma retinol and carotene, as well as median serum retinol binding protein concentrations, were significantly lower in cystic fibrosis patients than in control subjects. One cystic fibrosis patient had a raised fasting plasma retinyl ester concentration suggestive of chronic hypervitaminosis A, but no symptoms of toxicity. Measures of vitamin A absorption were also significantly lower in cystic fibrosis patients, although there was considerable overlap with control values. No correlation was observed between measures of baseline status and vitamin A absorption. Measurement of plasma retinyl esters may be an appropriate investigation in those patients considered to be at risk of chronic hypervitaminosis A. PMID:1612491

  2. A Rare Case: Struma Ovarii in a 14-Year-Old Girl.

    PubMed

    Iltar, Elif; Ureyen, Isin; Toptas, Tayfun; Savas, Melike; Çekiç, Sema; Uysal, Aysel

    2018-02-01

    Ovarian tumors presented with ovarian mass in childhood and adolescence are uncommon but an important part of gynecological cases. Struma ovarii is one of the rare cystic benign ovarian tumors that is observed predominantly in women who are between the ages of 40 and 60 years old. It is extremely rare in adolescents. Herein, we present a 14-year-old adolescent girl with struma ovarii who presented to the emergency room with abdominal pain.

  3. Respiratory muscle training for cystic fibrosis.

    PubMed

    Hilton, Nathan; Solis-Moya, Arturo

    2018-05-24

    Cystic fibrosis is the most common autosomal recessive disease in white populations, and causes respiratory dysfunction in the majority of individuals. Numerous types of respiratory muscle training to improve respiratory function and health-related quality of life in people with cystic fibrosis have been reported in the literature. Hence a systematic review of the literature is needed to establish the effectiveness of respiratory muscle training (either inspiratory or expiratory muscle training) on clinical outcomes in cystic fibrosis. This is an update of a previously published review. To determine the effectiveness of respiratory muscle training on clinical outcomes in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials register comprising of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of most recent search: 17 April 2018.A hand search of the Journal of Cystic Fibrosis and Pediatric Pulmonology was performed, along with an electronic search of online trial databases up until 07 May 2018. Randomised controlled studies comparing respiratory muscle training with a control group in people with cystic fibrosis. Review authors independently selected articles for inclusion, evaluated the methodological quality of the studies, and extracted data. Additional information was sought from trial authors where necessary. The quality of the evidence was assessed using the GRADE system MAIN RESULTS: Authors identified 19 studies, of which nine studies with 202 participants met the review's inclusion criteria. There was wide variation in the methodological and written quality of the included studies. Four of the nine included studies were published as abstracts only and lacking concise details, thus limiting the information available. Seven studies were parallel studies and two of a cross-over design. Respiratory

  4. [Cystic echinococcosis in the Mersin province (119 cases)].

    PubMed

    Aksu, Müzeyyen; Sevimli, Feride Kırcalı; Ibiloğlu, Ibrahim; Arpacı, Rabia Bozdoğan

    2013-01-01

    This study was made to emphasize the importance of cystic echinococcosis in the Mersin province and to contribute to informarion regarding the geographic distribution of infection in Turkey. Seven different pathology laboratory records in the Mersin province between the period 2011-2012 were evaluated according to age, sex and organ involvement. In study period, a total of 119 cases of cystic echinococcosis were determined. Male patients constituted 52.94% of the cases, and female, 47.05%. The age distribution of these cases was between 5 and 76 years. Most of the cases appeared in 41-50 (22.68%) year old patients. The most common localizations for cystic echinococcosis was determined in the liver (n: 53, 44.53%) and lung (n: 39, 32.77%), followed by the kidney (n: 4, 3.36%), spleen (n: 3, 2.52%) and gall bladder (n: 3, 2.52%). In 3 cases (2.52%), multiorgan involvement was seen. Cystic echinococcosis is an important health problem in Turkey. The stray dogs, which play an important role in the contagiousness of the disease, should be kept under control and treated. Also, slaughtering of animals must be preformed under the supervision of veterinary surgeons and destruction of infected organs should be carried out under proper conditions in slaughterhouses.

  5. Abdominal Circumference Versus Body Mass Index as Predictors of Lower Extremity Overuse Injury Risk.

    PubMed

    Nye, Nathaniel S; Kafer, Drew S; Olsen, Cara; Carnahan, David H; Crawford, Paul F

    2018-02-01

    Abdominal circumference (AC) is superior to body mass index (BMI) as a measure of risk for various health outcomes. Our objective was to compare AC and BMI as predictors of lower extremity overuse injury (LEOI) risk. Retrospective review of electronic medical records of 79,868 US Air Force personnel over a 7-year period (2005-2011) for incidence of new LEOI. Subjects were stratified by BMI and AC. Injury risk for BMI/AC subgroups was calculated using Kaplan-Meier curves and Cox proportional-hazards regression. Receiver operating characteristic curves with area under the curve were used to compare each model's predictive value. Cox proportional-hazards regression showed significant risk association between elevated BMI, AC, and all injury types, with hazard ratios ranging 1.230-3.415 for obese versus normal BMI and 1.665-3.893 for high-risk versus low-risk AC (P < .05 for all measures). Receiver operating characteristic curves with area under the curve showed equivalent performance between BMI and AC for predicting all injury types. However, the combined model (AC and BMI) showed improved predictive ability over either model alone for joint injury, overall LEOI, and most strongly for osteoarthritis. Although AC and BMI alone performed similarly well, a combined approach using BMI and AC together improved risk estimation for LEOI.

  6. 21 CFR 866.5910 - Quality control material for cystic fibrosis nucleic acid assays.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 8 2013-04-01 2013-04-01 false Quality control material for cystic fibrosis... Test Systems § 866.5910 Quality control material for cystic fibrosis nucleic acid assays. (a) Identification. Quality control material for cystic fibrosis nucleic acid assays. A quality control material for...

  7. 21 CFR 866.5910 - Quality control material for cystic fibrosis nucleic acid assays.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Quality control material for cystic fibrosis... Test Systems § 866.5910 Quality control material for cystic fibrosis nucleic acid assays. (a) Identification. Quality control material for cystic fibrosis nucleic acid assays. A quality control material for...

  8. 21 CFR 866.5910 - Quality control material for cystic fibrosis nucleic acid assays.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Quality control material for cystic fibrosis... Test Systems § 866.5910 Quality control material for cystic fibrosis nucleic acid assays. (a) Identification. Quality control material for cystic fibrosis nucleic acid assays. A quality control material for...

  9. 21 CFR 866.5910 - Quality control material for cystic fibrosis nucleic acid assays.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Quality control material for cystic fibrosis... Test Systems § 866.5910 Quality control material for cystic fibrosis nucleic acid assays. (a) Identification. Quality control material for cystic fibrosis nucleic acid assays. A quality control material for...

  10. LDL-cholesterol and insulin are independently associated with body mass index in adult cystic fibrosis patients.

    PubMed

    Coderre, Lise; Fadainia, Christophe; Belson, Linda; Belisle, Virginie; Ziai, Sophie; Maillhot, Geneviève; Berthiaume, Yves; Rabasa-Lhoret, Rémi

    2012-09-01

    The median life expectancy of cystic fibrosis (CF) patients has increased dramatically over the last few years and we now observe a subset of patients with a body mass index (BMI) exceeding 25 kg/m(2). The aim of this study was to characterize these individuals and to identify factors associated with higher BMI. This is a cross sectional study including 187 adult CF subjects. Percent predicted forced expiratory volume in 1s (%FEV(1)), blood lipid profiles as well as fasting glucose and insulin levels were evaluated. Subjects also had an oral glucose tolerance test (OGTT) and the area under the curve (AUC) for glucose and insulin was calculated. CF subjects were then stratified according to the following BMI categories: underweight: BMI≤18.5 kg/m(2); normal weight: 18.5 kg/m(2)

  11. Atypical postcesarean epithelioid trophoblastic lesion with cyst formation: a case report and literature review.

    PubMed

    Zhou, Feng; Lin, Kaiqing; Shi, Haiyan; Qin, Jiale; Lu, Bingjian; Huang, Lili

    2015-07-01

    We report an extremely rare case of atypical postcesarean epithelioid trophoblastic lesion with cyst formation. A 41-year-old Chinese woman presented with lower abdominal pain and menstrual disorder. Her serum human chorionic gonadotropin (hCG) was low (0.373 IU/L), and her urine hCG was negative. Ultrasound images showed a 3.7×2.8×2.5 cm(3) mass on the surface of the lower uterine segment, and a laparoscopy indicated a cystic mass in the serosal surface of the lower uterine segment. Histology indicated a cystic lesion consisting of epithelioid trophoblastic cells with an intermediate pattern between a classical placental site nodule and an epithelioid trophoblastic tumor; thus, the term atypical postcesarean epithelioid trophoblastic lesion with cyst formation was appropriate. As in atypical placental site nodule, serum hCG monitoring after treatment is necessary. Copyright © 2015. Published by Elsevier Inc.

  12. Fetal laser ablation of feeding artery of cystic lung lesions with systemic arterial blood supply.

    PubMed

    Cruz-Martinez, R; Martínez-Rodríguez, M; Bermúdez-Rojas, M; Magaña-Abarca, C; Narvaez-Dominguez, V; Rojas-Macedo, A; Bautista-García, N; Alcocer-Alcocer, M

    2017-06-01

    To assess the effectiveness of laser surgery in fetuses with a cystic lung lesion with systemic arterial blood supply (hybrid lung lesion) at risk of perinatal death. A cohort of five consecutive fetuses with a large hybrid lung lesion associated with hydrops and/or pleural effusion with severe lung compression was selected for percutaneous ultrasound-guided fetal laser ablation of the feeding artery (FLAFA) before 32 weeks' gestation in a single tertiary national referral center in Queretaro, Mexico. The primary outcomes were survival and need for postnatal surgery. FLAFA was performed successfully in all cases at a median gestational age of 24.9 (range, 24.4-31.7) weeks. After fetal intervention, dimensions in both lungs increased and fluid effusions resolved in all cases. All cases were delivered liveborn at term at a median gestational age of 39.6 (range, 38.0-39.7) weeks, without respiratory morbidity or need for oxygen support, resulting in perinatal survival of 100%. During follow-up, three (60%) cases showed progressive regression of the entire lung mass and did not require postnatal surgery, whereas in two (40%) cases a progressive decrease in size of the mass was observed but a cystic portion of the lung mass persisted and postnatal lobectomy was required. In fetuses with large hybrid lung lesions at risk of perinatal death, FLAFA is feasible and could improve survival and decrease the need for postnatal surgery. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

  13. Retrospective comparison of abdominal ultrasonography and radiography in the investigation of feline abdominal disease

    PubMed Central

    Won, Wylen Wade; Sharma, Ajay; Wu, Wenbo

    2015-01-01

    Abdominal radiography and ultrasonography are commonly used as part of the initial diagnostic plan for cats with nonspecific signs of abdominal disease. This retrospective study compared the clinical usefulness of abdominal radiography and ultrasonography in 105 feline patients with signs of abdominal disease. The final diagnosis was determined more commonly with ultrasonography (59%) compared to radiography (25.7%). Ultrasonography was also able to provide additional clinically relevant information in 76% of cases, and changed or refined the diagnosis in 47% of cases. Based on these findings, ultrasonography may be sufficient as an initial diagnostic test for the investigation of feline abdominal disease. PMID:26483582

  14. The Tribolium homeotic gene Abdominal is homologous to abdominal-A of the Drosophila bithorax complex

    NASA Technical Reports Server (NTRS)

    Stuart, J. J.; Brown, S. J.; Beeman, R. W.; Denell, R. E.; Spooner, B. S. (Principal Investigator)

    1993-01-01

    The Abdominal gene is a member of the single homeotic complex of the beetle, Tribolium castaneum. An integrated developmental genetic and molecular analysis shows that Abdominal is homologous to the abdominal-A gene of the bithorax complex of Drosophila. abdominal-A mutant embryos display strong homeotic transformations of the anterior abdomen (parasegments 7-9) to PS6, whereas developmental commitments in the posterior abdomen depend primarily on Abdominal-B. In beetle embryos lacking Abdominal function, parasegments throughout the abdomen are transformed to PS6. This observation demonstrates the general functional significance of parasegmental expression among insects and shows that the control of determinative decisions in the posterior abdomen by homeotic selector genes has undergone considerable evolutionary modification.

  15. The Tribolium homeotic gene Abdominal is homologous to abdominal-A of the Drosophila bithorax complex.

    PubMed

    Stuart, J J; Brown, S J; Beeman, R W; Denell, R E

    1993-01-01

    The Abdominal gene is a member of the single homeotic complex of the beetle, Tribolium castaneum. An integrated developmental genetic and molecular analysis shows that Abdominal is homologous to the abdominal-A gene of the bithorax complex of Drosophila. abdominal-A mutant embryos display strong homeotic transformations of the anterior abdomen (parasegments 7-9) to PS6, whereas developmental commitments in the posterior abdomen depend primarily on Abdominal-B. In beetle embryos lacking Abdominal function, parasegments throughout the abdomen are transformed to PS6. This observation demonstrates the general functional significance of parasegmental expression among insects and shows that the control of determinative decisions in the posterior abdomen by homeotic selector genes has undergone considerable evolutionary modification.

  16. [Differential diagnosis of abdominal pain].

    PubMed

    Frei, Pascal

    2015-09-02

    Despite the frequency of functional abdominal pain, potentially dangerous causes of abdominal pain need to be excluded. Medical history and clinical examination must focus on red flags and signs for imflammatory or malignant diseases. See the patient twice in the case of severe and acute abdominal pain if lab parameters or radiological examinations are normal. Avoid repeated and useless X-ray exposure whenever possible. In the case of subacute or chronic abdominal pain, lab tests such as fecal calprotectin, helicobacter stool antigen and serological tests for celiac disease are very useful. Elderly patients may show atypical or missing clinical signs. Take care of red herrings and be skeptical whether your initial diagnosis is really correct. Abdominal pain can frequently be an abdominal wall pain.

  17. Cystic pulmonary hydatidosis

    PubMed Central

    Sarkar, Malay; Pathania, Rajnish; Jhobta, Anupam; Thakur, Babu Ram; Chopra, Rajesh

    2016-01-01

    Cystic echinococcosis (CE) is a zoonotic parasitic disease caused by the larval stages of the cestode Echinococcus granulosus. Worldwide, pulmonary hydatid cyst is a significant problem medically, socially, and economically. Surgery is the definitive therapy of pulmonary hydatidosis. Benzimidazoles may be considered in patients with a surgical contraindication. This review will focus on pathogenesis, lifecycle, clinical features, and management of pulmonary hydatid disease. PMID:27051107

  18. Cost-Effectiveness of Screening Individuals With Cystic Fibrosis for Colorectal Cancer.

    PubMed

    Gini, Andrea; Zauber, Ann G; Cenin, Dayna R; Omidvari, Amir-Houshang; Hempstead, Sarah E; Fink, Aliza K; Lowenfels, Albert B; Lansdorp-Vogelaar, Iris

    2017-12-27

    Individuals with cystic fibrosis are at increased risk of colorectal cancer (CRC) compared to the general population, and risk is higher among those who received an organ transplant. We performed a cost-effectiveness analysis to determine optimal CRC screening strategies for patients with cystic fibrosis. We adjusted the existing Microsimulation Screening Analysis-Colon microsimulation model to reflect increased CRC risk and lower life expectancy in patients with cystic fibrosis. Modeling was performed separately for individuals who never received an organ transplant and patients who had received an organ transplant. We modeled 76 colonoscopy screening strategies that varied the age range and screening interval. The optimal screening strategy was determined based on a willingness to pay threshold of $100,000 per life-year gained. Sensitivity and supplementary analyses were performed, including fecal immunochemical test (FIT) as an alternative test, earlier ages of transplantation, and increased rates of colonoscopy complications, to assess whether optimal screening strategies would change. Colonoscopy every 5 years, starting at age 40 years, was the optimal colonoscopy strategy for patients with cystic fibrosis who never received an organ transplant; this strategy prevented 79% of deaths from CRC. Among patients with cystic fibrosis who had received an organ transplant, optimal colonoscopy screening should start at an age of 30 or 35 years, depending on the patient's age at time of transplantation. Annual FIT screening was predicted to be cost-effective for patients with cystic fibrosis. However, the level of accuracy of the FIT in population is not clear. Using a Microsimulation Screening Analysis-Colon microsimulation model, we found screening of patients with cystic fibrosis for CRC to be cost-effective. Due to the higher risk in these patients for CRC, screening should start at an earlier age with a shorter screening interval. The findings of this study

  19. Hypervitaminosis D: case report of pediatric osteoporosis secondary to cystic fibrosis.

    PubMed

    Cialdella, Pietro; Carella, Francesco

    2011-09-01

    The objective of the study is to evaluate alterations of bone metabolism in adolescence and adult CF, determining the rate of osteoporosis, osteopenia and vertebral and non-vertebral fractures. We took into account the clinical case of a child who right from the age of seven years has presented joint pain.The little girl was diagnosed with osteopenia taken with therapy of calcium and vitamin D; after few years despite treatment nephrocalcinosis and osteoporosis take over.It was examined a cohort of patients with cystic fibrosis of the southern Italy, 24 patients aged between 12 and 44 years, 12 females and 12 males with BMD assessment methods like dual energy X-rays (DXA) and calcaneal ultrasound densitometry in a few cases, ultrasonography was used jointly.From this case study we tried to establish the relationship between cystic fibrosis and osteoporosis etiopathogenetic, the adoptive therapy and the impact of therapies on patients.It was concluded that, given the high number of unrecognized patients with impaired bone mineralization, we must implement and integrate a more aggressive treatment with bisphosphonates and prevention programs that can combat the lifestyle and new eating habits of our young people that facilitate the loss of bone mass.

  20. [Rapid diagnostic tests for the serodiagnosis of human cystic echinococcosis].

    PubMed

    Tamarozzi, F; Mariconti, M; Covini, I; Brunetti, E

    2017-02-01

    Cystic echinococcosis (CE) is a parasitic zoonosis especially affecting resource-poor populations in livestock raising areas. Imaging, in particular ultrasound (US), is crucial for the diagnosis, staging, and clinical management of abdominal CE in humans. Serology is a valuable complement to imaging, especially when ultrasound features of CE are absent or unclear. In rural endemic areas, where expertise in US is scant, and conventional serology techniques are unavailable due to lack of laboratory equipment, rapid diagnostic tests (RDTs) may be very useful. Several reports have described the performance of commercial and experimental RDTs in the diagnosis of CE, including a recent study by our group that compared the diagnostic performances of three commercial RDTs for the diagnosis of hepatic CE. To put RDTs for CE in context, we reviewed the available literature in English on this topic. Overall, RDTs appear to be useful in resourcepoor settings where they may replace conventional serodiagnostic tests. However, like other serodiagnostic tests, RDTs lack standardization and show unsatisfactory sensitivity and specificity. An important issue that needs to be addressed is that studies on the diagnostic performance of RDTs fail to take into account the variables known to influence results such as anatomical location and cyst stage.

  1. Imaging of benign and malignant cystic pancreatic lesions and a strategy for follow up

    PubMed Central

    Bhosale, Priya; Balachandran, Aparna; Tamm, Eric

    2010-01-01

    Cystic lesions in a variety of organs are being increasingly recognized as an incidental finding on cross-sectional imaging. These lesions can be benign, premalignant or malignant. When these cystic lesions are small it can be difficult to characterize them radiologically. However, with appropriate clinical history and knowledge of typical imaging features of cystic pancreatic lesions this can enable accurate diagnosis and thus guide appropriate treatment. In this review, we provide an overview of the most common types of cystic lesions and their appearance on computer tomography, magnetic resonance imaging and ultrasound. We will also discuss the follow up and management strategies of these cystic lesions. PMID:21160696

  2. Variation in Cilia Protein Genes and Progression of Lung Disease in Cystic Fibrosis.

    PubMed

    Blue, Elizabeth; Louie, Tin L; Chong, Jessica X; Hebbring, Scott J; Barnes, Kathleen C; Rafaels, Nicholas M; Knowles, Michael R; Gibson, Ronald L; Bamshad, Michael J; Emond, Mary J

    2018-04-01

    Cystic fibrosis, like primary ciliary dyskinesia, is an autosomal recessive disorder characterized by abnormal mucociliary clearance and obstructive lung disease. We hypothesized that genes underlying the development or function of cilia may modify lung disease severity in persons with cystic fibrosis. To test this hypothesis, we compared variants in 93 candidate genes in both upper and lower tertiles of lung function in a large cohort of children and adults with cystic fibrosis with those of a population control dataset. Variants within candidate genes were tested for association using the SKAT-O test, comparing cystic fibrosis cases defined by poor (n = 127) or preserved (n = 127) lung function with population controls (n = 3,269 or 3,148, respectively). Associated variants were then tested for association with related phenotypes in independent datasets. Variants in DNAH14 and DNAAF3 were associated with poor lung function in cystic fibrosis, whereas variants in DNAH14 and DNAH6 were associated with preserved lung function in cystic fibrosis. Associations between DNAH14 and lung function were replicated in disease-related phenotypes characterized by obstructive lung disease in adults. Genetic variants within DNAH6, DNAH14, and DNAAF3 are associated with variation in lung function among persons with cystic fibrosis.

  3. Isolation, motivation and balance: living with type 1 or cystic fibrosis-related diabetes.

    PubMed

    Tierney, Stephanie; Deaton, Christi; Webb, Kevin; Jones, Andrew; Dodd, Mary; McKenna, Diane; Rowe, Rachel

    2008-04-01

    To explore patients' responses to developing and managing cystic fibrosis-related diabetes and to contrast their views with those of individuals with type 1 diabetes mellitus. The incidence of diabetes among people with cystic fibrosis has increased with improvement in life expectancy. However, little is known about how patients respond to and manage cystic fibrosis-related diabetes, and how this compares with people living with type 1 diabetes mellitus. Qualitative research was undertaken in order to fully explore meanings and views. Semi-structured telephone or face-to-face interviews were conducted with patients who had cystic fibrosis-related diabetes or type 1 diabetes mellitus, during which, they discussed diagnosis and management of diabetes. Framework analysis was employed to identify themes and to consider similarities and differences between the two groups. Eleven cystic fibrosis-related diabetes and 12 type 1 diabetes mellitus patients were interviewed in 2006. Patients with cystic fibrosis-related diabetes described their diabetes diagnosis as a progression of their primary illness, management of which was important owing to the benefits it brought to their cystic fibrosis. Those with type 1 diabetes mellitus were more likely to report feeling psychologically low because of diabetes and to list long-term complications as a key factor motivating self-management. Both groups struggled to balance the demands of diabetes with other life and health obligations, and experienced isolation because of diabetes. Conclusions. Variation in perceptions recalled during interviews stemmed from diabetes being part of an existing life-threatening chronic illness in people with cystic fibrosis-related diabetes. Similarities and differences in attitudes and management practices were found, with less urgency regarding glucose monitoring and fewer information resources available for those with cystic fibrosis-related diabetes. Both groups need support for optimal diabetes

  4. Complementary and alternative medicine use in children with cystic fibrosis.

    PubMed

    Giangioppo, Sandra; Kalaci, Odion; Radhakrishnan, Arun; Fleischer, Erin; Itterman, Jennifer; Lyttle, Brian; Price, April; Radhakrishnan, Dhenuka

    2016-11-01

    To estimate the overall prevalence of complementary and alternative medicine use among children with cystic fibrosis, determine specific modalities used, predictors of use and subjective helpfulness or harm from individual modalities. Of 53 children attending the cystic fibrosis clinic in London, Ontario (100% recruitment), 79% had used complementary and alternative medicine. The most commonly used modalities were air purifiers, humidifiers, probiotics, and omega-3 fatty acids. Family complementary and alternative medicine use was the only independent predictor of overall use. The majority of patients perceived benefit from specific modalities for cystic fibrosis symptoms. Given the high frequency and number of modalities used and lack of patient and disease characteristics predicting use, we recommend that health care providers should routinely ask about complementary and alternative medicine among all pediatric cystic fibrosis patients and assist patients in understanding the potential benefits and risks to make informed decisions about its use. Copyright © 2016 Elsevier Ltd. All rights reserved.

  5. Mycobacterium chimaera pulmonary infection complicating cystic fibrosis: a case report.

    PubMed

    Cohen-Bacrie, Stéphan; David, Marion; Stremler, Nathalie; Dubus, Jean-Christophe; Rolain, Jean-Marc; Drancourt, Michel

    2011-09-22

    Mycobacterium chimaera is a recently described species within the Mycobacterium avium complex. Its pathogenicity in respiratory tract infection remains disputed. It has never been isolated during cystic fibrosis respiratory tract infection. An 11-year-old boy of Asian ethnicity who was born on Réunion Island presented to our hospital with cystic fibrosis after a decline in his respiratory function over the course of seven years. We found that the decline in his respiratory function was correlated with the persistent presence of a Mycobacterium avium complex organism further identified as M. chimaera. Using sequencing-based methods of identification, we observed that M. chimaera organisms contributed equally to respiratory tract infections in patients with cystic fibrosis when compared with M. avium subsp. hominissuis isolates. We believe that M. chimaera should be regarded as an emerging opportunistic respiratory pathogen in patients with cystic fibrosis, including young children, and that its detection warrants long-lasting appropriate anti-mycobacterial treatment to eradicate it.

  6. [Italian Cystic Fibrosis Registry. Report 2011-2014].

    PubMed

    Giordani, Barbara; Amato, Annalisa; Majo, Fabio; Ferrari, Gianluca; Quattrucci, Serena; Minicucci, Laura; Padoan, Rita; Floridia, Giovanna; Puppo Fornaro, Gianna; Taruscio, Domenica; Salvatore, Marco

    2018-01-01

    centre refer only to 2014. The present Report has been organized into 10 sections. 1. Demography - number of Italian patients with cystic fibrosis (CF) in 2014 was 4,981 and their median age was 20.4 years; estimated 2014 CF prevalence was 8.2/100,000 residents in Italy; on average, 52.1% of the patients were male and CF distribution showed higher frequency in patients aged from 7 to 35 years. On average, 53.7% of CF patients are aged more than 18 years. 2. Diagnoses - most of the CF patients were diagnosed before two years of age (around 66%); a significant proportion of patients (on average, 12%) was diagnosed in adult age. 3. New diagnoses - new diagnoses were 187 in 2011, 200 in 2012, 160 in 2013, and 135 in 2014. Estimated incidence was 1/4,052 live births in 2011; 1/4,313 in 2012; 1/5,189 in 2013 and 1/8,243 in 2014. 4. Genetics - 99.5% of patients was studied at the molecular level, with identification of 90.1% of Cystic Fibrosis Transmembrane Regulator CFTR mutations; [delta]508F was the most frequent mutation (44.8% in 2014). 5. Lung function - FEV₁ (Forced Expiratory Volume in the first second) scores progressively decreased shortly before the start of adult age, in accordance with the natural history of the disease. Most of the patients between 6 and 17 years of age reported a FEV₁ % ≥ 70% of the predicted value, while the proportion of patients with severe lung disease (FEV₁ % <40% of the predicted value) is <2% over the period 2011-2014. 6. Nutrition - most critical periods come out during the first 6 months of life and during adolescence. Prevalence of malnourished male aged 12-17 years decreases over the period 2011-2014; an increasing percentage of patient (both male and female) with a suboptimal body mass index value is observed among patients aged more than 18 years 7. Complications - the presence of missing data represents an obstacle in the correct evaluation of prevalence value of complications related to Italian patients within ICFR

  7. Not All Children with Cystic Fibrosis Have Abnormal Esophageal Neutralization during Chemical Clearance of Acid Reflux.

    PubMed

    Woodley, Frederick W; Moore-Clingenpeel, Melissa; Machado, Rodrigo Strehl; Nemastil, Christopher J; Jadcherla, Sudarshan R; Hayes, Don; Kopp, Benjamin T; Kaul, Ajay; Di Lorenzo, Carlo; Mousa, Hayat

    2017-09-01

    Acid neutralization during chemical clearance is significantly prolonged in children with cystic fibrosis, compared to symptomatic children without cystic fibrosis. The absence of available reference values impeded identification of abnormal findings within individual patients with and without cystic fibrosis. The present study aimed to test the hypothesis that significantly more children with cystic fibrosis have acid neutralization durations during chemical clearance that fall outside the physiological range. Published reference value for acid neutralization duration during chemical clearance (determined using combined impedance/pH monitoring) was used to assess esophageal acid neutralization efficiency during chemical clearance in 16 children with cystic fibrosis (3 to <18 years) and 16 age-matched children without cystic fibrosis. Duration of acid neutralization during chemical clearance exceeded the upper end of the physiological range in 9 of 16 (56.3%) children with and in 3 of 16 (18.8%) children without cystic fibrosis ( p =0.0412). The likelihood ratio for duration indicated that children with cystic fibrosis are 2.1-times more likely to have abnormal acid neutralization during chemical clearance, and children with abnormal acid neutralization during chemical clearance are 1.5-times more likely to have cystic fibrosis. Significantly more (but not all) children with cystic fibrosis have abnormally prolonged esophageal clearance of acid. Children with cystic fibrosis are more likely to have abnormal acid neutralization during chemical clearance. Additional studies involving larger sample sizes are needed to address the importance of genotype, esophageal motility, composition and volume of saliva, and gastric acidity on acid neutralization efficiency in cystic fibrosis children.

  8. Cystic echinococcosis in Southern Israel.

    PubMed

    Ben-Shimol, Shalom; Sagi, Orli; Houri, Ohad; Bazarsky, Elina; Berkowitz, Anat; Bulkowstein, Shlomi; Barrett, Chiya; Greenberg, David

    2016-01-01

    The aim of this retrospective, population-based study was to characterize demographically and clinically cystic-echinococcosis (CE) in southern Israel, between 2005 and 2012. Newly-diagnosed (nd-CE) and past-diagnosed (pd-CE, diagnosed before the study) cases were defined. Two populations live in southern-Israel, receiving medical treatment at a single hospital: the Jewish and the Bedouin populations (resembling resource-rich and resource-poor populations, respectively). 126 CE cases were identified; 55 nd-CE and 71 pd-CE. Mean annual nd-CE incidence per 100,000 in the Bedouin and Jewish populations were 2.7 ± 1.2 and 0.4 ± 0.3, respectively (P<0.001). None of the Bedouin and 86.5% of the Jewish patients were born outside Israel. Liver and lung involvement were recorded in 85.7% and 15.1% of overall-CE, respectively. Abdominal pain, cough, fever, eosinophilia and asymptomatic disease were documented in 63.6%, 32.7%, 27.3%, 41.5% and 12.7% of nd-CE, respectively. Serology sensitivity for first test and any positive test were 67.3% and 83.3%, respectively. Computed tomography, ultrasonography and X-ray diagnosis were documented in 79.2%, 58.4% and 17.0% of overall-CE, respectively, with ultrasonography mainly used in liver-CE and X-ray in lung-CE. Treatment included surgery and albendazole in 50.0% and 55.3% of CE, respectively. We conclude that CE is endemic in southern-Israel among the Bedouin population, while disease is probably mainly imported in the Jewish population. Liver involvement and eosinophilia rates were high compared with those of other endemic regions, possibly due to differences in the timing of diagnosis. These findings may help developing treatment and prevention strategies.

  9. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis

    PubMed Central

    Olivier, Kenneth N; Saiman, Lisa; Daley, Charles L; Herrmann, Jean-Louis; Nick, Jerry A; Noone, Peadar G; Bilton, Diana; Corris, Paul; Gibson, Ronald L; Hempstead, Sarah E; Koetz, Karsten; Sabadosa, Kathryn A; Sermet-Gaudelus, Isabelle; Smyth, Alan R; van Ingen, Jakko; Wallace, Richard J; Winthrop, Kevin L; Marshall, Bruce C; Haworth, Charles S

    2016-01-01

    Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease such as cystic fibrosis (CF). Pulmonary disease caused by NTM has emerged as a major threat to the health of individuals with CF but remains difficult to diagnose and problematic to treat. In response to this challenge, the US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society (ECFS) convened an expert panel of specialists to develop consensus recommendations for the screening, investigation, diagnosis and management of NTM pulmonary disease in individuals with CF. Nineteen experts were invited to participate in the recommendation development process. Population, Intervention, Comparison, Outcome (PICO) methodology and systematic literature reviews were employed to inform draft recommendations. An anonymous voting process was used by the committee to reach consensus. All committee members were asked to rate each statement on a scale of: 0, completely disagree, to 9, completely agree; with 80% or more of scores between 7 and 9 being considered ‘good’ agreement. Additionally, the committee solicited feedback from the CF communities in the USA and Europe and considered the feedback in the development of the final recommendation statements. Three rounds of voting were conducted to achieve 80% consensus for each recommendation statement. Through this process, we have generated a series of pragmatic, evidence-based recommendations for the screening, investigation, diagnosis and treatment of NTM infection in individuals with CF as an initial step in optimising management for this challenging condition. PMID:26666259

  10. Energy-matched moderate and high intensity exercise training improves nonalcoholic fatty liver disease risk independent of changes in body mass or abdominal adiposity - A randomized trial.

    PubMed

    Winn, Nathan C; Liu, Ying; Rector, R Scott; Parks, Elizabeth J; Ibdah, Jamal A; Kanaley, Jill A

    2018-01-01

    Exercise training is commonly prescribed for individuals diagnosed with nonalcoholic fatty liver disease (NAFLD); however, consensus regarding the volume and intensity of exercise for optimal benefits is lacking. Thus, we determined whether high intensity interval exercise training (HIIT) produced greater reductions in intrahepatic lipid (IHL) content and NAFLD risk factors compared with energy-matched moderate intensity continuous exercise training (MICT) in obese adults with liver steatosis. Eighteen obese adults were randomized to either 4weeks of HIIT (4min 80% VO 2 peak/3min, 50% VO 2 peak) or MICT (55% VO 2 peak, ~60min), matched for energy expenditure (~400kcal/session) and compared to five non-exercising age-matched control subjects. IHL was measured by 1 H-MRS and frequent blood samples were analyzed for glucose, insulin, c-peptide, and NEFA levels during a liquid meal test (180min) to characterize metabolic phenotype. Baseline body weight, visceral abdominal adiposity, and fasting insulin concentrations were greater in the MICT vs HIIT group (P<0.05), while IHL was tightly matched between MICT and HIIT subjects (P>0.05), albeit higher than control subjects (P<0.01). Visceral abdominal adiposity, body mass, liver aminotransferases (ALT, AST), and hepatic apoptotic/inflammatory markers (cytokeratin 18 and fetuin a) were not reduced with either exercise training intervention (P>0.05). Both HIIT and MICT lowered IHL (HIIT, -37.0±12.4%; MICT, -20.1±6.6%, P<0.05); however, the reduction in IHL was not statistically different between exercise intensities (P=0.25). Furthermore, exercise training decreased postprandial insulin, c-peptide, and lipid peroxidation levels (iAUC, P<0.05). Collectively, these findings indicate that energy-matched high intensity and moderate intensity exercise are effective at decreasing IHL and NAFLD risk that is not contingent upon reductions in abdominal adiposity or body mass. Copyright © 2017 Elsevier Inc. All rights reserved.

  11. Preimplantation genetic diagnosis for cystic fibrosis: a case report.

    PubMed

    Biazotti, Maria Cristina Santoro; Pinto Junior, Walter; Albuquerque, Maria Cecília Romano Maciel de; Fujihara, Litsuko Shimabukuro; Suganuma, Cláudia Haru; Reigota, Renata Bednar; Bertuzzo, Carmen Sílvia

    2015-01-01

    Cystic fibrosis is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. This disorder produces a variable phenotype including lung disease, pancreatic insufficiency, and meconium ileus plus bilateral agenesis of the vas deferens causing obstructive azoospermia and male infertility. Preimplantation genetic diagnosis is an alternative that allows identification of embryos affected by this or other genetic diseases. We report a case of couple with cystic fibrosis; the woman had the I148 T mutation and the man had the Delta F508 gene mutation. The couple underwent in vitro fertilization, associated with preimplantation genetic diagnosis, and with subsequent selection of healthy embryos for uterine transfer. The result was an uneventful pregnancy and delivery of a healthy male baby.

  12. Parental care and overprotection of children with cystic fibrosis.

    PubMed

    Cappelli, M; McGrath, P J; MacDonald, N E; Katsanis, J; Lascelles, M

    1989-09-01

    Parental overprotection has often been clinically associated with the psychological maladjustment of children with a chronic disease. The purpose of this study was to examine parental care and overprotection in children with cystic fibrosis compared to healthy controls. Results indicated no differences in the level of parental care or overprotection between controls and children with cystic fibrosis. However, a number of significant correlations were found between parental care and overprotection and children's psychosocial functioning. In particular, positive correlations were found between parental overprotection and poor psychosocial functioning in children with cystic fibrosis, whereas, poor psychosocial functioning in healthy children was associated with lack of parental care. Parental overprotection and care appear to play important roles in the emotional and psychological functioning of healthy and chronically ill children.

  13. Preimplantation genetic diagnosis for cystic fibrosis: a case report

    PubMed Central

    Biazotti, Maria Cristina Santoro; Pinto, Walter; de Albuquerque, Maria Cecília Romano Maciel; Fujihara, Litsuko Shimabukuro; Suganuma, Cláudia Haru; Reigota, Renata Bednar; Bertuzzo, Carmen Sílvia

    2015-01-01

    Cystic fibrosis is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. This disorder produces a variable phenotype including lung disease, pancreatic insufficiency, and meconium ileus plus bilateral agenesis of the vas deferens causing obstructive azoospermia and male infertility. Preimplantation genetic diagnosis is an alternative that allows identification of embryos affected by this or other genetic diseases. We report a case of couple with cystic fibrosis; the woman had the I148 T mutation and the man had the Delta F508 gene mutation. The couple underwent in vitro fertilization, associated with preimplantation genetic diagnosis, and with subsequent selection of healthy embryos for uterine transfer. The result was an uneventful pregnancy and delivery of a healthy male baby. PMID:25993078

  14. Pediatric heart-lung transplantation for cystic fibrosis.

    PubMed

    Maynard, L C

    1994-01-01

    To describe the preoperative and postoperative experience of children who have undergone heart-lung transplantation for cystic fibrosis. Retrospective descriptive study. Paediatric Surgical Unit, Harefield Hospital, Middlesex, Great Britain. Twelve children less than 15 years of age (mean age 11 years 10 months; range 7 to 14 years), six boys and six girls, who received heart-lung transplants between September 1987 and March 1991. All 12 children were alive and well 1 year after surgery, although one girl had undergone retransplantation in the eighth postoperative month. Actuarial survival rate was 66% at 2 years. Early results suggest that heart-lung transplantation is a successful therapeutic option for children with cystic fibrosis. Cystic fibrosis-related postoperative complications were malabsorption of immunosuppressive drugs, meconium ileus equivalent, and persisting infection in the upper respiratory tract. These and general complications of acute rejection and infection did not prevent 66% of the group from returning to their normal schooling within the first postoperative year. Obliterative bronchiolitis remains the most serious late complication after lung transplantation, and further research is needed into treatment and prevention.

  15. Recent advances in understanding and managing cystic fibrosis transmembrane conductance regulator dysfunction

    PubMed Central

    Alton, Eric W.F.W.

    2015-01-01

    Cystic fibrosis is the most common autosomal recessive genetic disease in Caucasians and has been extensively studied for many decades. The cystic fibrosis transmembrane conductance regulator gene was identified in 1989. It encodes a complex protein which has numerous cellular functions. Our understanding of cystic fibrosis pathophysiology and genetics is constantly expanding and being refined, leading to improved management of the disease and increased life expectancy in affected individuals. PMID:26097737

  16. Young patients with cystic fibrosis demonstrate subtle alterations of the cardiovascular system.

    PubMed

    Eising, Jacobien B; van der Ent, Cornelis K; Teske, Arco J; Vanderschuren, Maaike M; Uiterwaal, Cuno S P M; Meijboom, Folkert J

    2018-02-02

    As life expectancy increases in patients with cystic fibrosis, it is important to pay attention to extra-pulmonary comorbidities. Several studies have shown signs of myocardial dysfunction in adult patients, but little is known about onset and development of these changes over time. In this prospective study, cardiac function in children with cystic fibrosis was compared to that of healthy children. 33 children, aged 3-12years, with cystic fibrosis were recruited from the Wilhelmina Children's hospital and 33 age-matched healthy children were selected from the WHISTLER study, a population-based cohort study. Measurements of lung function, arterial stiffness, and echocardiography (conventional measures and myocardial deformation imaging) were performed. There were no differences in anthropometrics, lung function and blood pressure between the two groups. The cystic fibrosis children had a higher arterial stiffness compared to the healthy children (pulse wave velocity respectively 5.76±0.57m/s versus 5.43±0.61m/s, p-value 0.049). Using conventional echocardiographic parameters for right ventricular function, Tricuspid Annular Plane Systolic Excursion) and Tissue Doppler Imaging, cystic fibrosis children had a reduced right ventricular systolic function when compared to the healthy children. After adjustment for lung function, global strains of both right and left ventricles were significantly lower in the cystic fibrosis group than in healthy children (linear regression coefficient 1.45% left ventricle, p-value 0.022 and 4.42% right ventricle, p-value <0.01). Systolic strain rate of basal segment of the left ventricle, the mid segment of the right ventricle and the apical septum were significantly lower in the cystic fibrosis children than in healthy controls. Our study suggests that already at a very young age, children with cystic fibrosis show an increased arterial stiffness and some signs of diminished both right and left ventricular function. Copyright © 2018

  17. Chronic abdominal wall pain and ultrasound-guided abdominal cutaneous nerve infiltration: a case series.

    PubMed

    Kanakarajan, Saravanakumar; High, Kristina; Nagaraja, Ravi

    2011-03-01

    Chronic abdominal wall pain occurs in about 10-30% of patients presenting with chronic abdominal pain. Entrapment of abdominal cutaneous nerves at the lateral border of the rectus abdominis muscle has been attributed as a cause of abdominal wall pain. We report our experience of treating such patients using ultrasound-guided abdominal cutaneous nerve infiltration. We conducted a retrospective audit of abdominal cutaneous nerve infiltration performed in the period between September 2008 to August 2009 in our center. All patients had received local anesthetic and steroid injection under ultrasound guidance. The response to the infiltration was evaluated in the post-procedure telephone review as well as in the follow-up clinic. Brief pain inventory (BPI) and numerical rating scale pain scores were collated from two points: the initial outpatient clinic and the follow up clinic up to 5 months following the injection. Nine patients had abdominal cutaneous nerve injections under ultrasound guidance in the period under review. Six patients reported 50% pain relief or more (responders) while three patients did not. Pain and BPI scores showed a decreasing trend in responders. The median duration of follow-up was 12 weeks. Ultrasound can reliably be used for infiltration of the abdominal cutaneous nerves. This will improve the safety as well as diagnostic utility of the procedure. Wiley Periodicals, Inc.

  18. Serum galactomannan in cystic fibrosis patients colonized with Aspergillus species.

    PubMed

    Warren, Thomas A; Yau, Yvonne; Ratjen, Felix; Tullis, Elizabeth; Waters, Valerie

    2012-08-01

    Cystic fibrosis patients are often colonized by Aspergillus species. Sera from 138 pediatric and adult cystic fibrosis patients were tested for the presence of galactomannan. All serum samples were negative for galactomannan and there was no difference among patients who were chronically, intermittently, and never colonized with Aspergillus.

  19. [Cystic echinococcosis mimicking tuberculosis in childhood].

    PubMed

    Pekcan, Sevgi; Kiper, Nural; Köse, Mehmet; Cobanoğlu, Nazan; Yalçın, Ebru; Doğru, Deniz; Ozçelik, Uğur

    2011-01-01

    Both cystic echinococcosis (CE) and tuberculosis (Tbc) are important health problems in developing countries. Pulmonary CE and Tbc have to be differentiated from other diseases as they have increased the risk of morbidity and mortality. Besides, these two diseases can mimic each other. Here, we discuss a 7 year-old patient admitted with fever, cough who was unresponsive to nonspecific antibiotic treatment given for pneumonia, had Tbc treatment due to a positive tuberculin skin test and radiologic appearance consistent with Tbc and on follow-up. He was eventually diagnosed as CE based on a cystic lesion consistent with CE in the liver and echinococcosis specific IgE positivity and was succesfully treated with anti-parasitic therapy.

  20. TESTS TO ASSESS SENSITIZATION TO ASPERGILLUS FUMIGATUS IN CYSTIC FIBROSIS

    PubMed Central

    Aguiar, Simone Santana; Damaceno, Neiva; Forte, Wilma Carvalho Neves

    2017-01-01

    ABSTRACT Objective: To evaluate the results of the tests used to identify the IgE mediated sensitization to Aspergillus fumigatus in patients with cystic fibrosis. Methods: This is a cross-sectional descriptive study with a convenience sample of 86 patients diagnosed with cystic fibrosis in the Reference Service in Cystic Fibrosis at a tertiary teaching hospital. The following tests were performed to assess the sensitization to A. fumigatus in patients with cystic fibrosis: Total serum IgE, eosinophil count, fungus detection through oropharyngeal swab or sputum culture, serum-specific IgE, and immediate-type hypersensitivity (IgE) skin tests. We compared the results of the different tests performed. Results: In 33 (38.4%) patients with cystic fibrosis, with ages ranging from 1 to 33 years (median of 8 years), the IgE-mediated A. fumigatus sensitization test results were: in 16 patients, there was an increase in serum-specific IgE (>0.35 kU/L); in 23, skin tests were positive; and six had sensitization in both tests. We observed two patients with eosinophilia (>1,000 eosinophils/mm3) and seven with increasing total serum IgE (>1,000 IU/mL), all of whom obtained negative results in skin tests and had no IgE increase specific to A. fumigatus. A. fumigatus was not detected in oropharyngeal swabs and/or sputum culture of any patients. Conclusions: We conclude that, among the tests used to assess sensitization to A. fumigatus in cystic fibrosis patients, both serum-specific IgE and immediate-type hypersensitivity (IgE) skin tests are required. Serum eosinophilia and respiratory secretion culture were not essential in this study. PMID:28977288

  1. Is size the only determinant of delayed abdominal closure in pediatric liver transplant?

    PubMed

    Khorsandi, Shirin Elizabeth; Day, Arthur William Raven; Cortes, Miriam; Deep, Akash; Dhawan, Anil; Vilca-Melendez, Hector; Heaton, Nigel

    2017-03-01

    The aim was to determine the factors associated with the use of delayed abdominal closure in pediatric liver transplantation (LT) and whether this affected outcome. From a prospectively maintained database, transplants performed in children (≤18 years) were identified (October 2010 to March 2015). Primary abdominal closure was defined as mass closure performed at time of transplant. Delayed abdominal closure was defined as mass closure not initially performed at the same time as transplant; 230 children underwent LT. Of these, 176 (76.5%) had primary closure. Age was similar between the primary and delayed groups (5.0 ± 4.9 versus 3.9 ± 5.0 years; P = 0.13). There was no difference in the graft-to-recipient weight ratio (GRWR) in the primary and delayed groups (3.4 ± 2.8 versus 4.1 ± 2.1; P = 0.12). Children with acute liver failure (ALF) were more likely to experience delayed closure then those with chronic liver disease (CLD; P < 0.001). GRWR was similar between the ALF and CLD (3.4 ± 2.4 versus 3.6 ± 2.7; P = 0.68). Primary closure children had a shorter hospital stay (P < 0.001), spent fewer days in pediatric intensive care unit (PICU; P = 0.001), and required a shorter duration of ventilation (P < 0.001). Vascular complications (arterial and venous) were similar (primary 8.2% versus delayed 5.6%; P = 0.52). Graft (P = 0.42) and child survival (P = 0.65) in the primary and delayed groups were similar. Considering timing of mass closure after transplant, patients in the early delayed closure group (<6 weeks) were found to experience a shorter time of ventilation (P = 0.03) and in PICU (P = 0.003). In conclusion, ALF was the main determinant of delayed abdominal closure rather than GRWR. The optimal time for delayed closure is within 6 weeks. The use of delayed abdominal closure does not adversely affect graft/child survival. Liver Transplantation 23 352-360 2017 AASLD. © 2016 by the

  2. Cystic mucinous adenocarcinoma of the lung: a case report

    PubMed Central

    2011-01-01

    Mucinous cystic tumors of the lung are uncommon, the preoperative pathologic diagnosis is difficult and their biological behavior is still controversial. We report the case of a patient with a clinically benign cystic lesion that post-operatively showed to be consistent with an invasive adenocarcinoma arising in a mucinous cystadenoma of the lung, We underline the difficulty of the clinical pre-operative diagnosis of this cystic neoplasia radiologically mimicking a hydatid cyst, and we report the negative TTF1 immunostaining potentially misleading in the differential diagnosis with metastatic mucinous carcinomas. Finallly, we evidence the presence of a pre-existing mucinous benign lesion suggesting early and complete resection of benign appearing lung cysts because they can undergo malignant transformation if left untreated or they can already harbor foci of invasive carcinoma at the time of the presentation. Even if a good prognosis, better than in other lung carcinomas, with no recurrrence or metastasis after complete surgical exicision, has been reported for cystic mucinous cystoadenocarcinomas, the follow-up showed an aggressive biological behaviour, with the early onset of metastasis, in keeping with P53 positive immunostaining and high Ki-67 proliferation index. PMID:21970610

  3. Treatment of cystic craniopharyngioma with phosphorus-32 intracavitary irradiation

    PubMed Central

    Zhao, Rong; Deng, Jinglan; Liang, Xiaoyan; Zeng, Jin; Chen, Xiaoyuan

    2013-01-01

    Purpose The aim of the study was to evaluate the effect of phosphorus-32 colloid ([32P]) intracavitary irradiation on the treatment of patients with cystic craniopharyngiomas. Methods Twenty patients with predominantly cystic craniopharyngiomas were admitted from 1981 to 2006. Eleven patients had [32P] intracavitary irradiation by stereotactic injection or Ommaya cyst instillation as the primary treatment, and the remaining nine had the same internal irradiation as an adjuvant treatment after tumor resection. A calculated irradiation dose of 400~500 Gy per once was delivered to the cyst wall. Conclusion The patients were followed up ranging from 36 to 336 months; no operative morbidity or mortality was found from [32P] intracavitary irradiation. Fourteen patients (70%) had tumor progression and required further two to four times intracavitary irradiation. All 20 cases achieved tumor shrinkage or stabilization with effective outcome 3–6 months after the last [32P] therapy. For patients with cystic craniopharyngioma, [32P] administration by stereo-tactic injection or Ommaya cyst instillation is a safe and helpful option, which could improve the life quality, prolong the life span, and enhance the survival rate of cystic craniopharyngioma patients. PMID:19904543

  4. Cystic fibrosis-related diabetes mellitus: etiology, evaluation, and management.

    PubMed

    Fischman, Daniel; Nookala, Vinod K

    2008-12-01

    To review the pathophysiology, diagnosis, and management of cystic fibrosis-related diabetes mellitus (CFRD). We performed a MEDLINE search of the literature, using the search terms "cystic fibrosis-related diabetes, "CFRD," and "cystic fibrosis and diabetes," to identify pertinent articles available in English. In patients with cystic fibrosis (CF), CFRD is a major cause for an accelerated decline in health. It is the result of multiple pathophysiologic mechanisms, including destruction of pancreatic islet cells, impaired hepatic response to the antigluconeogenic effects of insulin, and impaired insulin sensitivity. Nutritional management and adequate caloric intake are paramount to successful management of CF. Although insulin remains the standard of care for treating CFRD in conjunction with fasting hyperglycemia, a small but growing body of literature supports the use of oral therapies. In this article, we discuss the benefits of and possible adverse reactions to the various classes of oral and injectable agents used in the treatment of diabetes mellitus, with special attention to the population of patients with CF. Orally administered agents can have a role in the treatment of CFRD. Further study is needed to determine the optimal combination of therapeutic modalities for CFRD.

  5. Cystic fibrosis liver disease - from diagnosis to risk factors.

    PubMed

    Ciucă, Ioana Mihaiela; Pop, Liviu; Tămaş, Liviu; Tăban, Sorina

    2014-01-01

    Cystic fibrosis (CF) is the most frequent monogenic genetic disease, autosomal recessive transmitted, characterized by an impressive clinical polymorphism and appreciative fatal prospective. Liver disease is the second non-pulmonary cause of death in cystic fibrosis, which, with increasing life expectancy, became an important management problem. Predisposing factors like male gender, pancreatic insufficiency, meconium ileus and severe mutation are incriminated to influence the occurrence of cystic fibrosis associated liver disease (CFLD). Our study included 174 patients with CF, monitored in the National Cystic Fibrosis Centre, Timisoara, Romania. They were routinely followed-up by clinical assessment, liver biochemical tests, ultrasound examinations and other methods like transient elastography, biopsy, in selected cases. Sixty-six patients, with median age at diagnosis 4.33 years, diagnosed with CFLD, without significant gender gap. CFLD was frequent in patients aged over eight years, with meconium ileus history, carriers of severe mutations (p=0.002). Pancreatic insufficiency, although present in 75% of patients with CFLD was not confirmed as risk factor, not male gender, in our study. CF children older than eight years, carriers of a severe genotype, with a positive history of meconium ileus, were more likely predisposed to CFLD.

  6. Beware of spontaneous reduction "en masse" of inguinal hernia.

    PubMed

    Berney, C R

    2015-12-01

    Reduction 'en masse' of inguinal hernia is a rare entity defined as manual reduction of an external hernia sac back through the abdominal wall but where its content still remains incarcerated or strangulated into a displaced position, most often in the pre-peritoneal space. Small bowel obstruction habitually follows requiring urgent repair, preferentially via a trans-abdominal approach. Pre-operative clinical diagnosis is difficult and abdominal CT-scan imaging is the investigation of choice.

  7. How can the cystic fibrosis respiratory microbiome influence our clinical decision-making?

    PubMed

    Rogers, Geraint B; Bruce, Kenneth D; Hoffman, Lucas R

    2017-11-01

    Almost 15 years have now passed since bacterial community profiling techniques were first used to analyse respiratory samples from people with cystic fibrosis. Since then, many different analytical approaches have been used to try to better understand the contribution of the cystic fibrosis lung microbiota to disease, with varying degrees of success. We examine the extent to which cystic fibrosis respiratory microbiome research has been successful in informing clinical decision-making, and highlight areas that we believe have the potential to yield important insight. Recent research on the cystic fibrosis lung microbiome can be broadly divided into efforts to better characterize microbiota composition, particularly relative to key clinical events, and attempts to understand the cystic fibrosis lung microbiology as an interactive microbial system. The latter, in particular, has led to the development of a number of models in which microbiome-mediated processes precipitate clinical events. Growing technological sophistication is enabling increasingly detailed microbiological data to be generated from cystic fibrosis respiratory samples. However, translating these data into clinically useful measures that accurately predict outcomes and guide treatments remains a formidable challenge. The development of systems biology approaches that enable the integration of complex microbiome and host-derived data provide an exciting opportunity to address this goal.

  8. Trichosporon mycotoxinivorans, a Novel Respiratory Pathogen in Patients with Cystic Fibrosis▿

    PubMed Central

    Hickey, Patrick W.; Sutton, Deanna A.; Fothergill, Annette W.; Rinaldi, Michael G.; Wickes, Brian L.; Schmidt, Howard J.; Walsh, Thomas J.

    2009-01-01

    This report describes the molecular epidemiology, in vitro susceptibility, colonial and microscopic morphologies, and biochemical features of Trichosporon mycotoxinivorans, a newly recognized pathogen that appears to have a propensity for patients with cystic fibrosis. The index patient died with histologically documented Trichosporon pneumonia complicating cystic fibrosis. This is also the first report of disease caused by a Trichosporon species in a nontransplant patient with cystic fibrosis. As T. mycotoxinivorans has not previously been recognized as a respiratory pathogen, the significance of its recovery from sputum samples was not initially appreciated. Genetic analysis of archived clinical samples found three additional cases of T. mycotoxinivorans infection which had previously been identified as other members of the genus. An additional isolate of T. mycotoxinivorans was identified from a clinical sample on initial testing. Three of these four cases were also patients with cystic fibrosis. All isolates had MICs at 48 h of amphotericin B of ≥1 μg/ml and of echinocandins of ≥16 μg/ml, but they displayed various susceptibilities to the triazoles. In summary, Trichosporon mycotoxinivorans is a newly recognized human pathogen that is associated with cystic fibrosis. PMID:19656976

  9. Antimicrobial resistance in the respiratory microbiota of people with cystic fibrosis.

    PubMed

    Sherrard, Laura J; Tunney, Michael M; Elborn, J Stuart

    2014-08-23

    Cystic fibrosis is characterised by chronic polymicrobial infection and inflammation in the airways of patients. Antibiotic treatment regimens, targeting recognised pathogens, have substantially contributed to increased life expectancy of patients with this disease. Although the emergence of antimicrobial resistance and selection of highly antibiotic-resistant bacterial strains is of major concern, the clinical relevance in cystic fibrosis is yet to be defined. Resistance has been identified in recognised cystic fibrosis pathogens and in other bacteria (eg, Prevotella and Streptococcus spp) detected in the airway microbiota, but their role in the pathophysiology of infection and inflammation in chronic lung disease is unclear. Increased antibiotic resistance in cystic fibrosis might be attributed to a range of complex factors including horizontal gene transfer, hypoxia, and biofilm formation. Strategies to manage antimicrobial resistance consist of new antibiotics or localised delivery of antimicrobial agents, iron sequestration, inhibition of quorum-sensing, and resistome analysis. Determination of the contributions of every bacterial species to lung health or disease in cystic fibrosis might also have an important role in the management of antibiotic resistance. Copyright © 2014 Elsevier Ltd. All rights reserved.

  10. Heterogeneity of the cystic fibrosis phenotype in a large kindred family in Qatar with cystic fibrosis mutation (I1234V).

    PubMed

    Abdul Wahab, A; Al Thani, G; Dawod, S T; Kambouris, M; Al Hamed, M

    2001-04-01

    Twenty-nine subjects (17 families) with cystic fibrosis belonging to the same Bedouin tribe were screened for cystic fibrosis transmembrane regulator gene mutations (CFTR). Homozygous I1234V mutation in exon 19 was identified in all families with a relatively high rate of consanguinity (96.6 per cent). The homozygous I1234V mutation tended to present with a variable degree of pulmonary disease, pancreatic insufficiency and electrolyte imbalance. Homozygous I1234V was found to be a common mutation in the studied Bedouin tribe in Qatar.

  11. Hyponatremia-associated rhabdomyolysis following exercise in an adolescent with cystic fibrosis.

    PubMed

    Kaskavage, Jillian; Sklansky, Daniel

    2012-07-01

    Adolescents with well-controlled cystic fibrosis, including good lung function and appropriate growth, commonly participate in competitive athletic activities. We present the case of an adolescent male with cystic fibrosis, hyponatremia, dehydration, and rhabdomyolysis after participating in football practice on a summer morning. The patient presented with severe myalgia and serum sodium of 129 mmol/L, chloride 90 mmol/L, and creatine phosphokinase 1146 U/L. Aggressive hydration with intravenous 0.9% saline resulted in clinical improvement with no renal or muscular sequelae. Health care providers need to educate patients with cystic fibrosis about maintaining adequate hydration and sodium repletion during exercise. Research is needed regarding the appropriate amount and composition of oral rehydration fluids in exercising individuals with cystic fibrosis, as the physiology encountered in these patients provides a unique challenge to maintaining electrolyte balance and stimulation of thirst.

  12. European evidence-based guidelines on pancreatic cystic neoplasms

    PubMed Central

    Del Chiaro, Marco

    2018-01-01

    Evidence-based guidelines on the management of pancreatic cystic neoplasms (PCN) are lacking. This guideline is a joint initiative of the European Study Group on Cystic Tumours of the Pancreas, United European Gastroenterology, European Pancreatic Club, European-African Hepato-Pancreato-Biliary Association, European Digestive Surgery, and the European Society of Gastrointestinal Endoscopy. It replaces the 2013 European consensus statement guidelines on PCN. European and non-European experts performed systematic reviews and used GRADE methodology to answer relevant clinical questions on nine topics (biomarkers, radiology, endoscopy, intraductal papillary mucinous neoplasm (IPMN), mucinous cystic neoplasm (MCN), serous cystic neoplasm, rare cysts, (neo)adjuvant treatment, and pathology). Recommendations include conservative management, relative and absolute indications for surgery. A conservative approach is recommended for asymptomatic MCN and IPMN measuring <40 mm without an enhancing nodule. Relative indications for surgery in IPMN include a main pancreatic duct (MPD) diameter between 5 and 9.9 mm or a cyst diameter ≥40 mm. Absolute indications for surgery in IPMN, due to the high-risk of malignant transformation, include jaundice, an enhancing mural nodule >5 mm, and MPD diameter >10 mm. Lifelong follow-up of IPMN is recommended in patients who are fit for surgery. The European evidence-based guidelines on PCN aim to improve the diagnosis and management of PCN. PMID:29574408

  13. New and emerging targeted therapies for cystic fibrosis.

    PubMed

    Quon, Bradley S; Rowe, Steven M

    2016-03-30

    Cystic fibrosis (CF) is a monogenic autosomal recessive disorder that affects about 70,000 people worldwide. The clinical manifestations of the disease are caused by defects in the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The discovery of the CFTR gene in 1989 has led to a sophisticated understanding of how thousands of mutations in the CFTR gene affect the structure and function of the CFTR protein. Much progress has been made over the past decade with the development of orally bioavailable small molecule drugs that target defective CFTR proteins caused by specific mutations. Furthermore, there is considerable optimism about the prospect of gene replacement or editing therapies to correct all mutations in cystic fibrosis. The recent approvals of ivacaftor and lumacaftor represent the genesis of a new era of precision medicine in the treatment of this condition. These drugs are having a positive impact on the lives of people with cystic fibrosis and are potentially disease modifying. This review provides an update on advances in our understanding of the structure and function of the CFTR, with a focus on state of the art targeted drugs that are in development. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  14. Blunted perception of neural respiratory drive and breathlessness in patients with cystic fibrosis

    PubMed Central

    Jolley, Caroline J.; Elston, Caroline; Moxham, John; Rafferty, Gerrard F.

    2016-01-01

    The electromyogram recorded from the diaphragm (EMGdi) and parasternal intercostal muscle using surface electrodes (sEMGpara) provides a measure of neural respiratory drive (NRD), the magnitude of which reflects lung disease severity in stable cystic fibrosis. The aim of this study was to explore perception of NRD and breathlessness in both healthy individuals and patients with cystic fibrosis. Given chronic respiratory loading and increased NRD in cystic fibrosis, often in the absence of breathlessness at rest, we hypothesised that patients with cystic fibrosis would be able to tolerate higher levels of NRD for a given level of breathlessness compared to healthy individuals during exercise. 15 cystic fibrosis patients (mean forced expiratory volume in 1 s (FEV1) 53.5% predicted) and 15 age-matched, healthy controls were studied. Spirometry was measured in all subjects and lung volumes measured in the cystic fibrosis patients. EMGdi and sEMGpara were recorded at rest and during incremental cycle exercise to exhaustion and expressed as a percentage of maximum (% max) obtained from maximum respiratory manoeuvres. Borg breathlessness scores were recorded at rest and during each minute of exercise. EMGdi % max and sEMGpara % max and associated Borg breathlessness scores differed significantly between healthy subjects and cystic fibrosis patients at rest and during exercise. The relationship between EMGdi % max and sEMGpara % max and Borg score was shifted to the right in the cystic fibrosis patients, such that at comparable levels of EMGdi % max and sEMGpara % max the cystic fibrosis patients reported significantly lower Borg breathlessness scores compared to the healthy individuals. At Borg score 1 (clinically significant increase in breathlessness from baseline) corresponding levels of EMGdi % max (20.2±12% versus 32.15±15%, p=0.02) and sEMGpara % max (18.9±8% versus 29.2±15%, p=0.04) were lower in the healthy individuals compared to the cystic fibrosis

  15. Blunted perception of neural respiratory drive and breathlessness in patients with cystic fibrosis.

    PubMed

    Reilly, Charles C; Jolley, Caroline J; Elston, Caroline; Moxham, John; Rafferty, Gerrard F

    2016-01-01

    The electromyogram recorded from the diaphragm (EMG di ) and parasternal intercostal muscle using surface electrodes (sEMG para ) provides a measure of neural respiratory drive (NRD), the magnitude of which reflects lung disease severity in stable cystic fibrosis. The aim of this study was to explore perception of NRD and breathlessness in both healthy individuals and patients with cystic fibrosis. Given chronic respiratory loading and increased NRD in cystic fibrosis, often in the absence of breathlessness at rest, we hypothesised that patients with cystic fibrosis would be able to tolerate higher levels of NRD for a given level of breathlessness compared to healthy individuals during exercise. 15 cystic fibrosis patients (mean forced expiratory volume in 1 s (FEV 1 ) 53.5% predicted) and 15 age-matched, healthy controls were studied. Spirometry was measured in all subjects and lung volumes measured in the cystic fibrosis patients. EMG di and sEMG para were recorded at rest and during incremental cycle exercise to exhaustion and expressed as a percentage of maximum (% max) obtained from maximum respiratory manoeuvres. Borg breathlessness scores were recorded at rest and during each minute of exercise. EMG di % max and sEMG para % max and associated Borg breathlessness scores differed significantly between healthy subjects and cystic fibrosis patients at rest and during exercise. The relationship between EMG di % max and sEMG para % max and Borg score was shifted to the right in the cystic fibrosis patients, such that at comparable levels of EMG di % max and sEMG para % max the cystic fibrosis patients reported significantly lower Borg breathlessness scores compared to the healthy individuals. At Borg score 1 (clinically significant increase in breathlessness from baseline) corresponding levels of EMG di % max (20.2±12% versus 32.15±15%, p=0.02) and sEMG para % max (18.9±8% versus 29.2±15%, p=0.04) were lower in the healthy individuals compared to the cystic

  16. Systemic mastocytosis: CT and US features of abdominal manifestations.

    PubMed

    Avila, N A; Ling, A; Worobec, A S; Mican, J M; Metcalfe, D D

    1997-02-01

    To study the imaging findings in patients with systemic mastocytosis and to correlate the findings with the severity of disease on the basis of an established classification system. Pathologic findings, when available, were correlated with imaging findings. Computed tomographic (CT) and ultrasound (US) scans and corresponding pathologic findings, when available, were retrospectively reviewed in 27 patients with systemic mastocytosis. Only five (19%) of the patients in our series had normal abdominal CT and/or US examination results. Common abdominal imaging findings associated with systemic mastocytosis were hepatosplenomegaly, retroperitoneal adenopathy, periportal adenopathy, mesenteric adenopathy, thickening of the omentum and the mesentery, and ascites. Less common findings included hepatofugal portal venous flow, Budd-Chiari syndrome, cavernous transformation of the portal vein, ovarian mass, and complications such as chloroma. The findings were more common in patients with category II and those with category III disease. Abdominal findings at CT and US are common in patients with systemic mastocytosis. Although the findings in patients with systemic mastocytosis are not specific to the disease, they are useful in directing further studies for diagnostic confirmation and in estimating the extent of systemic involvement.

  17. In vitro comparison of intra-abdominal hypertension development after different temporary abdominal closure techniques.

    PubMed

    Benninger, Emanuel; Labler, Ludwig; Seifert, Burkhardt; Trentz, Otmar; Menger, Michael D; Meier, Christoph

    2008-01-01

    To compare volume reserve capacity (VRC) and development of intra-abdominal hypertension after different in vitro temporary abdominal closure (TAC) techniques. A model of the abdomen was designed. The abdominal wall was simulated with polychloroprene, a synthetic rubber compound. A lentil-shaped defect of 150 cm(2) was cut into the anterior aspect of the abdominal wall. TAC of this defect was performed by a zipper system (ZS), a bag silo closure (BSC), or a vacuum assisted closure (VAC) with subatmospheric pressures ranging from 0- to 200 mmHg. The model with intact abdominal wall served as reference. The model was filled with water to baseline level. The intra-abdominal pressure was increased in 2 mmHg steps from baseline level (6 mmHg) to 40 mmHg by adding volume to the system according to a standardized protocol. VRC with corresponding intra-abdominal pressure were analyzed and compared for the different TAC techniques. VRC was the highest after BSC at all pressure levels studied (P < 0.05). VAC and ZS resulted in significantly lower VRC compared with BSC and reference (P < 0.05). The magnitude of negative pressure on the VAC did not significantly influence the VRC. In the present in vitro model, BSC demonstrated the highest VRC of all evaluated TAC techniques. Different levels of subatmospheric pressures applied to the VAC did not affect VRC. The results for ZS and VAC indicate that these TAC techniques may increase the risk for recurrent intra-abdominal hypertension and should therefore not be used in high-risk patients during the initial phase after abdominal decompression.

  18. Change in Intra-Abdominal Fat Predicts the Risk of Hypertension in Japanese Americans.

    PubMed

    Sullivan, Catherine A; Kahn, Steven E; Fujimoto, Wilfred Y; Hayashi, Tomoshige; Leonetti, Donna L; Boyko, Edward J

    2015-07-01

    In Japanese Americans, intra-abdominal fat area measured by computed tomography is positively associated with the prevalence and incidence of hypertension. Evidence in other populations suggests that other fat areas may be protective. We sought to determine whether a change in specific fat depots predicts the development of hypertension. We prospectively followed up 286 subjects (mean age, 49.5 years; 50.4% men) from the Japanese American Community Diabetes Study for 10 years. At baseline, subjects did not have hypertension (defined as blood pressure ≥140/90 mm Hg) and were not taking blood pressure or glucose-lowering medications. Mid-thigh subcutaneous fat area, abdominal subcutaneous fat area, and intra-abdominal fat area were directly measured by computed tomography at baseline and 5 years. Logistic regression was used to estimate odds of incident hypertension over 10 years in relation to a 5-year change in fat area. The relative odds of developing hypertension for a 5-year increase in intra-abdominal fat was 1.74 (95% confidence interval, 1.28-2.37), after adjusting for age, sex, body mass index, baseline intra-abdominal fat, alcohol use, smoking status, and weekly exercise energy expenditure. This relationship remained significant when adjusted for baseline fasting insulin and 2-hour glucose levels or for diabetes mellitus and pre-diabetes mellitus classification. There were no significant associations between baseline and change in thigh or abdominal subcutaneous fat areas and incident hypertension. In conclusion, in this cohort of Japanese Americans, the risk of developing hypertension is related to the accumulation of intra-abdominal fat rather than the accrual of subcutaneous fat in either the thigh or the abdominal areas. © 2015 American Heart Association, Inc.

  19. Brain and lung metastasis of alveolar echinococcosis in a refugee from a hyperendemic area.

    PubMed

    Tappe, Dennis; Weise, David; Ziegler, Uwe; Müller, Andreas; Müllges, Wolfgang; Stich, August

    2008-11-01

    Alveolar echinococcosis (AE) of the liver with cerebral and pulmonary metastasis was diagnosed in a Tibetan monk who initially presented with severe headache to an emergency department in Germany. Multiple lesions with perifocal oedema and severe compression of the third ventricle were seen with computed tomography (CT) of the brain. Glioma or cerebral metastasis of a hitherto undiagnosed abdominal or pulmonary malignancy was suspected. CT scans of the lung and liver demonstrated further tumorous masses. Magnetic resonance imaging of the brain revealed the cystic nature of the cerebral lesions and the patient had a highly positive serology for AE. The echinococcal aetiology of the brain lesions was confirmed by PCR for this refugee from an area where two disease entities, AE and cystic echinococcosis, are hyperendemic.

  20. [Pancreatic mucinous cystadenoma doubly complicated by acute pancreatitis and retroperitoneal rupture].

    PubMed

    Maghrebi, Houcine; Makni, Amine

    2017-01-01

    Mucinous cystadenomas are benign tumors with malignant potential. They are often revealed by non-specific abdominal pain, jaundice or an episode of acute pancreatitis. We here report an exceptional case of mucinous cystadenoma doubly complicated by acute pancreatitis and retroperitoneal rupture. The study involved a 30-year old non-weighted female patient, presenting with epigastric pain associated with left hypochondrium evolving over the last three months and which had intensified without fever or jaundice in the last 3 days. Clinical examination showed impingement on palpation of the epigastrium and of the left hypochondrium. There was no palpable mass. Laboratory tests were without abnormalities, except for lipasemia that was 8-times the upper normal. Abdominal CT scan showed bi-loculated cystic mass in the pancreas tail, measuring 111 mm * 73 mm, with a thin wall and a fluid content, associated with an infiltration of the left perirenal fascia. MRI (Panel A) showed mucinous cystadenoma with retroperitoneal rupture. The caudal portion of the main pancreatic duct was slightly dilated and communicated with the pancreatic cyst. The patient underwent surgery via bi-sub-costal approach. A cystic mass in the pancreas tail with retroperitoneal rupture associated with acute pancreatitis (outflow of necrotic content from left anterior prerenal space) was found. Caudal splenopancreatectomy was performed (Panel B). The postoperative course was uneventful. The anatomo-pathological examination of the surgical specimen showed pancreatic mucinous cystadenoma with low-grade dysplasia.

  1. Measurement of superficial and deep abdominal muscle thickness: an ultrasonography study.

    PubMed

    Tahan, Nahid; Khademi-Kalantari, Khosro; Mohseni-Bandpei, Mohammad Ali; Mikaili, Saeed; Baghban, Alireza Akbarzadeh; Jaberzadeh, Shapour

    2016-08-23

    Real-time ultrasound imaging is a valid method in the field of rehabilitation. The ultrasound imaging allows direct visualization for real-time study of the muscles as they contract over the time. Measuring of the size of each abdominal muscle in relation to the others provides useful information about the differences in structure, as well as data on trunk muscle activation patterns. The purpose of this study was to assess the size and symmetry of the abdominal muscles at rest in healthy adults and to provide a reference range of absolute abdominal muscle size in a relatively large population. A total 156 healthy subjects with the age range of 18-44 years were randomly recruited. The thickness of internal oblique, external oblique, transverse abdominis, and rectus abdominis muscles was measured at rest on both right and left sides using ultrasound. Independent t test was used to compare the mean thickness of each abdominal muscle between males and females. Differences on side-to-side thicknesses were assessed using paired t test. The association between abdominal muscle thicknesses with gender and anthropometric variables was examined using the Pearson correlation coefficient. A normal pattern of increasing order of mean abdominal muscle thickness was found in both genders at both right and left sides: transverse abdominis < external oblique < internal oblique < rectus abdominis. There was a significant difference on the size of transverse abdominis, internal oblique, and external oblique muscles between right and left sides in both genders. Males had significantly thicker abdominal muscles than females. Age was significantly correlated with the thickness of internal oblique, external oblique, and rectus abdominis muscles. Body mass index was also positively correlated with muscle thickness of rectus abdominis and external oblique. The results provide a normal reference range for the abdominal muscles in healthy subjects and may be used as an index to

  2. Cystic echinococcosis in immigrant from Peru: first case treated with percutaneous treatment in Japan.

    PubMed

    Sakaguchi, Hiroshi; Tanaka, Toshihiro; Marugami, Nagaaki; Kichikawa, Kimihiko; Horiuchi, Hazuki; Morioka, Chie; Toyohara, Masahisa; Moriya, Kei; Nishiofuku, Mariko; Mitoro, Akira; Fukui, Hiroshi; Hirai, Toshiko; Yamashita, Namiko; Ouji, Yukiteru; Ishizaka, Shigeaki; Yoshikawa, Masahide

    2007-09-01

    We report a case of cystic echinococcosis (CE) caused by Echinococcusgranulosus, for which a modified percutaneous evacuation (PEVAC) treatment was applied. The patient had immigrated from Peru to Japan and had 2 hydatid cystic masses, 1 located in segment (S)5 of the liver and the other in S3 (5.3 and 3.5 cm in diameter, respectively), both of which were visualized as pseudotumors by ultrasound (US) examinations. Albendazole treatment showed no effects and surgical treatment was refused. After punctuation of the S5 cyst under US guidance and S3 with CT guidance, 10- and 12-French gauge catheters, respectively, with multiple side holes were inserted. About 60 ml of the cyst contents was drawn out from the S5 lesion and 2 ml from the S3 lesion. Using repetitive manual injections and aspiration of small amounts of hypertonic saline, the remaining cyst content was removed as much as possible, after which 20 and 10 ml of 98% ethanol was injected into the S5 and S3 lesions, respectively. A short-term evaluation during the 4 month-period following the procedure using US revealed nearly complete evacuation of the S5 lesion, whereas that at S3 remained as a pseudo-solid mass. We consider that percutaneous treatment is a safe therapeutic modality for hydatid cysts. This is the first case report of CE treated percutaneously in Japan.

  3. Antenatal testing for cystic fibrosis in Cuba, 1988-2011.

    PubMed

    Collazo, Teresa; López, Ixchel; Clark, Yulia; Piloto, Yaixa; González, Laura; Gómez, Manuel; García, Marileivis; Reyes, Lidice; Rodríguez, Fidel

    2014-01-01

    INTRODUCTION Cystic fibrosis is a multisystem autosomal recessive disease with wide variability in clinical severity. It is incurable and characterized by elevated and premature mortality, as well as poor quality of life. Its frequency, lethality and devastating impact on both the physical and psychological wellbeing of patients and their families, make it a serious health problem. Its frequency in Cuba is 1 in 9862 live births, where marked molecular heterogeneity of the CFTR gene makes molecular diagnosis difficult. Six mutations have been identified that together enable molecular characterization of only 55.5% of cystic fibrosis chromosomes. This paper presents national results of antenatal diagnostic testing, using direct and indirect methods, for detection of cystic fibrosis. OBJECTIVE Characterize the Cuban public health system's experience with antenatal molecular testing for cystic fibrosis from 1988 through 2011. METHODS A retrospective descriptive study was conducted with results of antenatal diagnostic testing of amniotic fluid, performed nationwide from 1988 through 2011, for 108 fetuses of couples with some risk of having children affected by cystic fibrosis, who requested testing. Polymerase chain reaction detected mutations p.F508del, p.G542X, p.R1162X, p.R334W, p.R553X and c.3120+1G>A, and markers XV2C and KM19. Data were analyzed using absolute frequencies and percentages, and presented in tables. RESULTS For 93 cases (86.1%), testing for cystic fibrosis was done using direct analysis of mutations p.F508del, p.G542X, p.R1162X, p.R334W, p.R553X and c.3120+1G>A; five cases (4.6%) were tested indirectly using markers XV2C/Taq I and KM19/Pst I; and 10 (9.3%) were tested using a combination of the two methods. A total of 72 diagnoses (66.7% of studies done) were concluded, of which there were 20 healthy fetuses, 16 affected, 27 carrier, and 9 who were either healthy or carriers of an unknown mutation. CONCLUSIONS Direct or indirect molecular study was

  4. Outcome of Preterm Infants with Transient Cystic Periventricular Leukomalacia on Serial Cranial Imaging Up to Term Equivalent Age.

    PubMed

    Sarkar, Subrata; Shankaran, Seetha; Barks, John; Do, Barbara T; Laptook, Abbot R; Das, Abhik; Ambalavanan, Namasivayam; Van Meurs, Krisa P; Bell, Edward F; Sanchez, Pablo J; Hintz, Susan R; Wyckoff, Myra H; Stoll, Barbara J; Carlo, Waldemar A

    2018-04-01

    To determine the outcome of preterm infants whose cystic periventricular leukomalacia "disappeared" on serial screening cranial imaging studies. Infants ≤26 weeks of gestation born between 2002 and 2012 who had cranial imaging studies at least twice, the most abnormal study at <28 days of age and another closest to 36 weeks, were reviewed. The outcome of late death (after 36 weeks postmenstrual age) or neurodevelopmental impairment (NDI) in surviving infants at 18-26 months corrected age was compared between the infants with no cystic periventricular leukomalacia on both studies and cystic periventricular leukomalacia that disappeared (cystic periventricular leukomalacia at <28 days but not at 36 weeks), persisted (cystic periventricular leukomalacia on both studies), or appeared late (cystic periventricular leukomalacia only at 36 weeks). Predictors of NDI were evaluated by logistic regression. Of 7063 eligible infants, 433 (6.1%) had cystic periventricular leukomalacia. Among the 433 infants with cystic periventricular leukomalacia, cystic periventricular leukomalacia disappeared in 76 (18%), persisted in 87 (20%), and 270 (62%) had late cystic periventricular leukomalacia. Loss to follow-up ranged between 3% and 13%. Death or NDI was more common in infants with disappeared cystic periventricular leukomalacia compared with those with no cystic periventricular leukomalacia (38 of 72 [53%] vs 1776 of 6376 [28%]; OR [95% CI] 2.8 [1.8-4.6]). Disappeared, persistent, and late cystic periventricular leukomalacia were all also independently associated with NDI (OR 1.17, 1.21, and 1.16, respectively). Infants with "disappeared" cystic periventricular leukomalacia are at increased risk of adverse outcome similar to infants with persistent or late cystic periventricular leukomalacia. Copyright © 2017 Elsevier Inc. All rights reserved.

  5. Pancreatic cystic neoplasms: Review of current knowledge, diagnostic challenges, and management options

    PubMed Central

    Jana, Tanima; Shroff, Jennifer; Bhutani, Manoop S.

    2015-01-01

    Pancreatic cystic lesions are being detected with increasing frequency, largely due to advances in cross-sectional imaging. The most common neoplasms include serous cystadenomas, mucinous cystic neoplasms, intraductal papillary mucinous neoplasms, solid pseudopapillary neoplasms, and cystic pancreatic endocrine neoplasms. Computed tomography (CT), magnetic resonance imaging (MRI), and endoscopic ultrasound (EUS) are currently used as imaging modalities. EUS-guided fine needle aspiration has proved to be a useful diagnostic tool, and enables an assessment of tumor markers, cytology, chemistries, and DNA analysis. Here, we review the current literature on pancreatic cystic neoplasms, including classification, diagnosis, treatment, and recommendations for surveillance. Data for this manuscript was acquired via searching the literature from inception to December 2014 on PubMed and Ovid MEDLINE. PMID:25821410

  6. Pulmonary function outcomes for assessing cystic fibrosis care.

    PubMed

    Wagener, Jeffrey S; Elkin, Eric P; Pasta, David J; Schechter, Michael S; Konstan, Michael W; Morgan, Wayne J

    2015-05-01

    Assessing cystic fibrosis (CF) patient quality of care requires the choice of an appropriate outcome measure. We looked systematically and in detail at pulmonary function outcomes that potentially reflect clinical practice patterns. Epidemiologic Study of Cystic Fibrosis data were used to evaluate six potential outcome variables (2002 best FVC, FEV(1), and FEF(25-75) and rate of decline for each from 2000 to 2002). We ranked CF care sites by outcome measure and then assessed any association with practice patterns and follow-up pulmonary function. Sites ranked in the top quartile had more frequent monitoring, treatment of exacerbations, and use of chronic therapies and oral corticosteroids. The follow-up rate of pulmonary function decline was not predicted by site ranking. Different pulmonary function outcomes associate slightly differently with practice patterns, although annual FEV(1) is at least as good as any other measure. Current site ranking only moderately predicts future ranking. Copyright © 2014 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

  7. Update on fat-soluble vitamins in cystic fibrosis.

    PubMed

    Maqbool, Asim; Stallings, Virginia A

    2008-11-01

    We review and critique recent scientific advances in the understanding of fat-soluble vitamins and the care of people with cystic fibrosis. A shift in the conceptual approach to fat-soluble vitamin status has occurred. Vitamin status in cystic fibrosis had previously been discussed in terms of sufficiency versus insufficiency as compared with healthy populations. The discussion of vitamin status has now shifted to that of suboptimal versus optimal with respect to health outcomes. This is best illustrated by advances in the study of vitamin D. Newer metabolic and immunological roles and biomarkers have been identified. With supplementation of water-miscible formulations of preformed vitamin A, increased serum retinol has been observed, and may increase the risk for toxicity. A paradigm shift has occurred in defining fat-soluble vitamin status by utilizing different biomarkers and associations with health outcomes. Identification of additional biomarkers, redefining definitions of adequacy, optimal surveillance for toxicity as well as adequacy is needed for care of patients with cystic fibrosis.

  8. Prenatal diagnosis of cystic fibrosis: 10-years experience.

    PubMed

    Hadj Fredj, S; Ouali, F; Siala, H; Bibi, A; Othmani, R; Dakhlaoui, B; Zouari, F; Messaoud, T

    2015-06-01

    We present in this study our 10years experience in prenatal diagnosis of cystic fibrosis performed in the Tunisian population. Based on family history, 40 Tunisian couples were selected for prenatal diagnosis. Fetal DNA was isolated from amniotic fluid collected by transabdominal amniocentesis or from chronic villi by transcervical chorionic villus sampling. The genetic analysis for cystic fibrosis mutations was performed by denaturant gradient gel electrophoresis and denaturing high-pressure liquid phase chromatography. We performed microsatellites analysis by capillary electrophoresis in order to verify the absence of maternal cell contamination. Thirteen fetuses were affected, 21 were heterozygous carriers and 15 were healthy with two normal alleles of CFTR gene. Ten couples opted for therapeutic abortion. The microsatellites genotyping showed the absence of contamination of the fetal DNA by maternal DNA in 93.75%. Our diagnostic strategy provides rapid and reliable prenatal diagnosis at risk families of cystic fibrosis. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  9. Sweat Chlorides in Salt-Deprived Cystic Fibrosis Heterozygotes

    PubMed Central

    Myers, Michael F.

    1965-01-01

    Sweat chlorides of 10 sets of parents of children with cystic fibrosis and 11 controls were studied in an attempt to develop a test for the diagnosis of cystic fibrosis heterozygotes by subjecting both the parents and controls to a low sodium diet and comparing sweat chloride values as the diet progressed. It was hoped that the sweat chloride levels of the parents, the heterozygotes, would remain stationary throughout the diet, since their children, the homozygotes, reveal this finding under similar conditions of salt deprivation. The sweat chloride levels of the controls, because of effects of aldosterone, were expected to decrease steadily from the commencement of the diet to its termination. A decrease in sweat chloride values of similar magnitude was found in both parents and controls as the diet continued. It is concluded that the study of sweat electrolyte levels in salt-deprived subjects is of no value in the diagnosis of cystic fibrosis heterozygotes. PMID:14289142

  10. Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis.

    PubMed

    Elphick, Heather E; Southern, Kevin W

    2014-11-28

    Allergic bronchopulmonary aspergillosis (ABPA) is an allergic reaction to colonisation of the lungs with the fungus Aspergillus fumigatus and affects around 10% of people with cystic fibrosis. ABPA is associated with an accelerated decline in lung function. High doses of corticosteroids are the main treatment for ABPA; although the long-term benefits are not clear, their many side effects are well-documented. A group of compounds, the azoles, have activity against Aspergillus fumigatus and have been proposed as an alternative treatment for ABPA. Of this group, itraconazole is the most active. A separate antifungal compound, amphotericin B, has been employed in aerosolised form to treat invasive infection with Aspergillus fumigatus, and may have potential for the treatment of ABPA. Antifungal therapy for ABPA in cystic fibrosis needs to be evaluated. The review aimed to test the hypotheses that antifungal interventions for the treatment of ABPA in cystic fibrosis:1. improve clinical status compared to placebo or standard therapy (no placebo);2. do not have unacceptable adverse effects.If benefit was demonstrated, we aimed to assess the optimal type, duration and dose of antifungal therapy. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings.In addition, pharmaceutical companies were approached.Date of the most recent search of the Group's Trials Register: 17 March 2014. Published or unpublished randomised controlled trials, where antifungal treatments have been compared to either placebo or no treatment, or where different doses of the same treatment have been used in the treatment of ABPA in people with cystic fibrosis. Four trials were identified by the searches; none of which was judged eligible for inclusion in the review. No completed randomised controlled trials

  11. Adenoid cystic carcinoma associated with mucous retention cyst of the parotid gland.

    PubMed

    Hebbale, Manjula Advisha; Halli, Rajshekhar C; Kini, Yogesh K; Kharkar, Viraj R; Metgud, Rashmi

    2011-09-01

    Mucous retention cysts of the parotid gland are rare, and a coexistent adenoid cystic carcinoma is even an unusual occurrence. Such coexistent adenoid cystic carcinomas with mucous retention cyst of the parotid gland are difficult to diagnose clinically and, at times, stage difficulty in their management. We report a rare case of adenoid cystic carcinoma associated with mucous retention cyst of the parotid gland with its diagnostic and management dilemma in a 14-year-old adolescent girl.

  12. The lipid A of Burkholderia multivorans C1576 smooth-type lipopolysaccharide and its pro-inflammatory activity in a cystic fibrosis airways model.

    PubMed

    Ieranò, Teresa; Cescutti, Paola; Leone, Maria Rosaria; Luciani, Alessandro; Rizzo, Roberto; Raia, Valeria; Lanzetta, Rosa; Parrilli, Michelangelo; Maiuri, Luigi; Silipo, Alba; Molinaro, Antonio

    2010-12-01

    Cystic fibrosis is an autosomal recessive disorder and it is characterised by chronic bacterial airway infection which leads to progressive lung deterioration, sometimes with fatal outcome. Burkholderia multivorans and Burkholderia cenocepacia are the species responsible for most of the infections of cystic fibrosis patients. Lipopolysaccharide endotoxins (LPSs) are among the foremost factors of pathogenesis of Gram-negative infection and, in particular, lipid A is the endotoxic portion of LPS responsible for eliciting host innate immune response. In this work, the complete primary structure of the lipid A from B. multivorans C1576 has been defined and, further, its pro-inflammatory activity in a cystic fibrosis airways model is shown. The structure of B. multivorans lipid A was attained by chemical, mass spectrometry and nuclear magnetic resonance analyses whereas its biological activity was assessed on the intestinal epithelial cell line CACO-2 cells, on the airway epithelial IB3-1 cells, carrying the ΔF508/W1282X CFTR mutation and on an ex vivo model of culture explants of nasal polyps.

  13. Abdominal binders may reduce pain and improve physical function after major abdominal surgery - a systematic review.

    PubMed

    Rothman, Josephine Philip; Gunnarsson, Ulf; Bisgaard, Thue

    2014-11-01

    Evidence for the effect of post-operative abdominal binders on post-operative pain, seroma formation, physical function, pulmonary function and increased intra-abdominal pressure among patients after surgery remains largely un-investigated. A systematic review was conducted. The PubMed, EMBASE and Cochrane databases were searched for studies on the use of abdominal binders after abdominal surgery or abdominoplasty. All types of clinical studies were included. Two independent assessors evaluated the scientific quality of the studies. The primary outcomes were pain, seroma formation and physical function. A total of 50 publications were identified; 42 publications were excluded leaving eight publications counting a total of 578 patients for analysis. Generally, the scientific quality of the studies was poor. Use of abdominal binder revealed a non-significant tendency to reduce seroma formation after laparoscopic ventral herniotomy and a non-significant reduction in pain. Physical function was improved, whereas evidence supports a beneficial effect on psychological distress after open abdominal surgery. Evidence also supports that intra-abdominal pressure increases with the use of abdominal binders. Reduction of pulmonary function during use of abdominal binders has not been revealed. Abdominal binders reduce post-operative psychological distress, but their effect on post-operative pain after laparotomy and seroma formation after ventral hernia repair remains unclear. Due to the sparse evidence and poor quality of the literature, solid conclusions may be difficult to make, and procedure-specific, high-quality randomised clinical trials are warranted.

  14. A service evaluation of an integrated model of palliative care of cystic fibrosis.

    PubMed

    Bourke, Stephen J; Booth, Zoe; Doe, Simon; Anderson, Alan; Rice, Sarah; Gascoigne, Alistair; Quibell, Rachel

    2016-07-01

    Patients with advanced cystic fibrosis have severe symptoms with a complex trajectory of exacerbations and recovery. They are often awaiting lung transplantation, and many die without receiving specialist palliative care. We introduced an integrated model whereby palliative specialists joined the cystic fibrosis team to provide palliative care in parallel with standard care. A service evaluation of this model of care was undertaken in a prospective case series documenting symptoms and outcomes, the views of the cystic fibrosis team and the experience of the palliative specialists. Over 3 years, 28 (10%) of 282 patients attending the cystic fibrosis centre had specialist palliative care. They had advanced lung disease (mean forced expiratory volume in 1 s (FEV1) = 0.86 L (25% predicted)), and 17 died: 6 were on a transplant waiting list at death; 10 were unsuitable and 1 died post transplantation. All who died over these 3 years had specialist palliative care. Four patients had successful transplants. Assessment showed a high prevalence of breathlessness, cough, pain, vomiting and fatigue, with a significant impact on daily life. The cystic fibrosis team rated this model of care highly, felt that palliative care should be members of the team, and thought that patients had found it helpful. The palliative specialists gained knowledge of cystic fibrosis, found it beneficial to meet patients earlier in the disease, and identified unmet needs in managing bereavement and the effects of deaths on other patients with cystic fibrosis. This model has been successful in overcoming the difficulties in access to specialist palliative care for patients with cystic fibrosis. © The Author(s) 2016.

  15. Uneven Distribution of Regional Blood Supply Prompts the Cystic Change of Pituitary Adenoma.

    PubMed

    Zhang, Jianhe; Gu, Jianjun; Ma, Yiming; Huang, Yinxing; Wang, Jiaxing; Wu, Zhifeng; Zhong, Qun; Wang, Shousen

    2017-07-01

    Previous studies have suggested that the cystic change of pituitary adenoma might be related to the blood supply and metabolism of the tumor; however, the exact pathologic mechanism underlying the cystic change remains unknown. We aimed to assess the features of regional blood supply of pituitary adenoma and examine its relationship with the cystic change of pituitary adenoma. Patients (N = 79) with pituitary adenoma admitted to our hospital were divided into the parenchyma group (n = 40) or the cystic change group (n = 39). Dynamic contrast-enhanced magnetic resonance imaging of the pituitary adenoma was conducted for the parenchyma group and the steepest slopes (SS max , reflecting regional blood supply) at different areas were calculated. The location of cystic change of the pituitary adenoma was recorded and analyzed for the cystic change group. The parenchyma group showed an upper SS max of 2.52 ± 1.18, a lower SS max of 2.89 ± 1.46, a left SS max of 2.71 ± 1.31, and a right SS max of 2.66 ± 1.29. The difference between the upper and lower SS max was statistically significant (P < 0.001), with no difference between the left and right regions (P = 0.668). The location of cystic change of the pituitary adenoma was mainly in the upper region, accounting for 48.7% of cases. Regional blood supply is unevenly distributed in the parenchymal pituitary adenoma, with reduced blood supply in the upper than the lower region. Cystic change mainly occurs in the upper region of pituitary adenoma. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Cystic dysplasia of the testis: a very rare paediatric tumor of the testis.

    PubMed

    Eberli, Daniel; Gretener, Heini; Dommann-Scherrer, Corina; Pestalozzi, Dietegen; Fehr, Jean-Luc

    2002-01-01

    To describe a case of cystic dysplasia of the testis (CDT), an uncommon cause of scrotal swelling in the pediatric patient. Clinic, therapy, fertility, and radiographic and pathologic findings are discussed and the 30 previously reported cases are reviewed. A 9-year-old boy presented with asymptomatic scrotal swelling. A scrotal ultrasound showed a multicystic scrotal mass in the rete testis and an ipsilateral renal agenesis. The growth in size of the mass forced the authors to perform an operative exploration. Intraoperative findings included a multicystic mass in the rete testis of the right testicle. Testicle-sparing total removal of the multicystic mass was performed and the pathologic examination revealed a benign, multilobulated configuration of the cysts in the region of the rete testis. These findings were similar to those found in previously reported cases of CDT. Ipsilateral renal agenesis is the most common associated anomaly. As a pathogenetic factor, mal-junction of the Wolffian duct in the 5th week of gestation is most creditable. CDT is a rare cause of pediatric scrotal mass. When feasible, a testicle-sparing approach should be considered and all patients should undergo evaluation for associated urologic anomalies.

  17. The non-resolving lung cavity: a case of pulmonary cystic echinococcosis.

    PubMed

    Mifsud, Simon; Schembri, Emma Louise; Gauci, Jonathan; Mizzi, Adrian; Mallia Azzopardi, Charles; Micallef, Josef

    2017-07-31

    The authors report a case of a 20-year-old woman who was diagnosed with pulmonary cystic echinococcosis. She was admitted to hospital with a 1-week history of unresolving cough, coloured sputum with occasional haemoptysis and fever despite oral antibiotics. Radiology revealed a cavitating right lower lobe lung abscess. After 4 weeks of treatment, follow-up radiology showed incomplete resolution. Bronchoscopy revealed a white, avascular cystic lesion in the right lower lobe and serology testing for Echinococcus granulosus was positive. Repeat imaging eventually confirmed the cystic lesion with the 'air bubble'sign. A thorough travel history, a high index of clinical suspicion and close follow-up are essential in making a diagnosis of pulmonary cystic echinococcosis. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  18. Safety of Chemotherapeutic Infusion or Chemoembolization for Hepatocellular Carcinoma Supplied Exclusively by the Cystic Artery

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Kang, Beomsik, E-mail: kangbs98@gmail.com; Kim, Hyo-Cheol, E-mail: angiointervention@gmail.com; Chung, Jin Wook, E-mail: chungjw@snu.ac.kr

    Purpose: This study was designed to evaluate the safety of chemotherapeutic infusion or chemoembolization by way of the cystic artery in patients with hepatocellular carcinoma (HCC) supplied exclusively by the cystic artery. Methods: Between Jan 2002 and Dec 2011, we performed chemotherapeutic infusion or chemoembolization using iodized oil for the treatment of 27 patients with HCC supplied exclusively by the cystic artery. Computed tomography (CT) scans, digital subtraction angiograms, and medical records were retrospectively reviewed by consensus. Results: The cystic artery originated from the main right hepatic artery in 24 (89 %) patients, from the right anterior hepatic artery inmore » 2 (7 %) patients, and from the left hepatic artery in 1 (4 %) patient. Selective catheterization of the cystic artery was achieved in all patients. Superselection of tumor-feeding vessels from the cystic artery was achieved in 7 patients (26 %). Chemotherapeutic infusion was performed in 18 patients (67 %), and chemoembolization was performed in 9 patients (33 %). There were no major complications and only 2 minor complications, including vasovagal syncope and nausea with vomiting. Individual tumor response supplied exclusively by the cystic artery at the follow-up enhanced CT scan were complete response (n = 16), partial response (n = 3), and stable disease (n = 8). Conclusion: HCC supplied exclusively by the cystic artery can be safely treated without severe complications by chemotherapeutic infusion or chemoembolization using iodized oil through the cystic artery.« less

  19. Abdominal Sepsis.

    PubMed

    De Waele, Jan J

    2016-08-01

    Abdominal infections are an important challenge for the intensive care physician. In an era of increasing antimicrobial resistance, selecting the appropriate regimen is important and, with new drugs coming to the market, correct use is important more than ever before and abdominal infections are an excellent target for antimicrobial stewardship programs. Biomarkers may be helpful, but their exact role in managing abdominal infections remains incompletely understood. Source control also remains an ongoing conundrum, and evidence is increasing that its importance supersedes the impact of antibiotic therapy. New strategies such as open abdomen management may offer added benefit in severely ill patients, but more data are needed to identify its exact role. The role of fungi and the need for antifungal coverage, on the other hand, have been investigated extensively in recent years, but at this point, it remains unclear who requires empirical as well as directed therapy.

  20. Widespread co-endemicity of human cystic and alveolar echinococcosis on the eastern Tibetan Plateau, northwest Sichuan/southeast Qinghai, China.

    PubMed

    Li, Tiaoying; Chen, Xingwang; Zhen, Ren; Qiu, Jiamin; Qiu, Dongchuan; Xiao, Ning; Ito, Akira; Wang, Hu; Giraudoux, Patrick; Sako, Yasuhito; Nakao, Minoru; Craig, Philip S

    2010-03-01

    Cystic echinococcosis (CE) or hydatid disease is known to be cosmopolitan in its global distribution, while alveolar echinococcosis (AE) is a much rarer though more pathogenic hepatic parasitic disease restricted to the northern hemisphere. Both forms of human echinococcosis are known to occur on the Tibetan Plateau, but the epidemiological characteristics remain poorly understood. In our current study, abdominal ultrasound screening programs for echinococcosis were conducted in 31 Tibetan townships in Ganze and Aba Tibetan Autonomous Prefectures of northwest Sichuan Province during 2001-2008. Hospital records (1992-2006) in a major regional treatment centre for echinococcosis in Sichuan Province were also reviewed. Of 10,186 local residents examined by portable ultrasound scan, 645 (6.3%) were diagnosed with echinococcosis: a prevalence of 3.2% for CE, 3.1% for AE and 0.04% for dual infection (both CE and AE). Human cystic and alveolar echinococcosis in pastoral areas was highly co-endemic, in comparison to much lower prevalences in semi-pastoral or farming regions. The high ultrasound prevalence in these co-endemic areas in northwest Sichuan Province was also reflected in the hospital study, and hospital records furthermore indicated another possible highly co-endemic focus in Guoluo Prefecture of Qinghai Province, located at the border of northwest Sichuan. These chronic cestode zoonoses constitute an unparalleled major public health problem for pastoral Tibetan communities, and pose great difficulties for adequate treatment access and effective transmission control in such remote regions. Copyright 2009 Elsevier B.V. All rights reserved.