Lymphangioma of the jejunal mesentery and jejunal polyps presenting as an acute abdomen in a teenager.

PubMed

Jayasundara, Jasb; Perera, E; Chandu de Silva, M V; Pathirana, A A

2017-03-01

Cystic lymphangioma of the small bowel mesentery is a rare clinical entity, especially after childhood. Medical literature reveals a limited number of such cases presenting as acute abdomen due to bowel obstruction, small bowel volvulus and bleeding into the tumour. We present the management experience of an 18-year-old woman who presented with rapid onset diffuse peritonism and raised inflammatory markers. Computed tomography showed a mass in the small bowel mesentery with suspicion of segmental bowel ischaemia. Emergency laparotomy revealed a mass in the mid-jejunal mesentery close to the bowel wall with no bowel ischaemia. The patient made an uncomplicated recovery after segmental bowel resection and end-to-end anastomosis. Histology confirmed the mass as a cystic lymphangioma involving the jejunal mesentery and two small jejunal polyps. Lymphangioma could be considered in the differential diagnosis of an acute abdomen in a young adult when the presentation is atypical.

Management of orbital lymphangioma using intralesional injection of OK-432

PubMed Central

Suzuki, Y.; Obana, A.; Gohto, Y.; Miki, T.; Otuka, H.; Inoue, Y.

2000-01-01

AIM—To treat orbital lymphangioma with an intralesional injection of OK-432 (group A Streptococcus pyogenes of human origin).
METHOD—A 14 year old boy had a right orbital cystic lymphangioma. The visual acuity in the eye was 20/28. In an initial treatment, 0.02 mg of OK-432, was injected into the tumour after aspiration of the fluid contents, but no effect was seen. The second treatment was performed with 0.04 mg of OK-432.
RESULT—4 months later, the lesion had totally shrunk to fibrous tissue. The side effects were fever, a local inflammatory reaction lasting 3 days, and increased intraocular pressure, which was managed by draining the fluid contents. Visual acuity improved to 20/15, and the visual field defect and restriction of eye movement seen before treatment disappeared. No recurrence was noted 1 year after treatment.
CONCLUSION—An intralesional injection of OK-432 shrunk the lymphangioma without functional disturbance and scar in the facial skin. OK-432 may be useful for orbital lymphangioma, but further studies are still warranted to determine efficacy, complications, and the optimal dose for safe treatment.

 PMID:10837387

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.

Lymphangioma of vulva

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

Young, A.W. Jr.; Wind R.M.; Tovell, H.M.M.

1980-05-01

A second case with acquired lymphangioma of the vulva has been presented. The condition was diagnosed 22 years following radiation and lymph-node dissection for carcinoma of the cervix and a long history of recurring erysipelas involving the vulva and lymphedematous lower extremities. The pathogenesis of the lymphangioma and the possibility of a later development of a vascular cancer have been discussed.

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.

A Supernumerary Nipple-Like Clinical Presentation of Lymphangioma Circumscriptum.

PubMed

Taylor, Dustin; Kash, Natalie; Silapunt, Sirunya

2018-01-01

Lymphangioma circumscriptum is a superficially localized variant of lymphangioma. The characteristic clinical presentation is a "frogspawn" grouping of vesicles or papulovesicles on the proximal limb or limb girdle areas. Though most lymphangiomas develop congenitally, the lymphangioma circumscriptum subtype is known to present in adults. We report a case of lymphangioma circumscriptum on the left inframammary area of an African American female with an unusual supernumerary nipple-like clinical presentation. Our patient presented with a firm, smooth, hypopigmented papule, and the clinical diagnosis of keloid was made initially. However, she returned reporting growth of the lesion and was noted to have a firm, exophytic, lobulated, pink to skin-colored nodule. Histopathological examination demonstrated dilated lymphatic vessels, consistent with the diagnosis of lymphangioma. The presentation as a firm, hypopigmented papule and later exophytic, lobulated, skin-colored nodule in our case represents a clinical presentation of lymphangioma circumscriptum not previously described in the literature. Correct diagnosis in lymphangioma circumscriptum is vital, as recurrence following surgical resection and secondary development of lymphangiosarcoma and squamous cell carcinoma following treatment with radiation have been reported. Thus, it is important to consider lymphangioma circumscriptum in the differential of similar lesions in the future to allow appropriate diagnosis, treatment, and monitoring.

[Treatment of lymphangioma with OK-432 infiltration].

PubMed

Rodríguez, J; Cáceres, F; Vargas, P

2012-10-01

The management of lymphangioma using sclerotherapy has proven to be an effective therapeutic. Our aim was to evaluate the therapeutic efficacy of OK-432 (Picibanil) in patients with lymphagioma. The study was performed from November 2010 to July 2011. Fifteen patients of both genders were diagnosed with lymphangioma, 12 days to 12 years old. All patients were infiltrated with OK-432. The studied variables were: previous surgery, localization, type of lymphangioma, number of effective injections, reduction of mass valued as excellent (100% reduction), good (reduction > 50%) and bad (reduction < 50%), presence of recurrence and complications. 40% of pacients had prior surgery and 53.3% were located in the cervical-face region. The type of macrocystic lymphangioma was present in 40% of the series, mixed type in 46.6% and microcystic type in 13.4%. The number of effective infiltrations were 3. In 6 cases (40%) the result was excellent in 5 cases (33.4%) the result was good and in 4 cases (26.6%). We had 1 recurrence (6.6%) and we haven't had complications. Injection of OK-432 in macrocystic lymphangioma and mixed had a safe therapeutic modality with satisfactory results. So it is a valid alternative to conventional surgery.

Treatments of various otolaryngological cystic diseases by OK-4321: its indications and limitations.

PubMed

Ohta, Nobuo; Fukase, Shigeru; Suzuki, Yusuke; Ishida, Akihiro; Aoyagi, Masaru

2010-11-01

The aim of this study was to evaluate the indications for, and outcomes and limitations of, OK-432 therapy in various otolaryngological cystic diseases. A retrospective clinical study at Yamagata University School of Medicine and the Fukase Clinic in Japan. Between April 1996 and November 2009 we tried OK-432 therapy in 148 patients with otolaryngological cystic diseases. In cases of plunging ranulas, lymphangiomas, branchial cleft cysts, thyroglossal duct cysts, thyroid cysts, and cervical lymphocele, we aspirated as much of the fluid content of each cystic lesion as possible, and we then replaced the volume of aspirated fluid with about half the volume of OK-432 solution. Disappearance of the lesion was observed in 119 of 148 patients (80%). Marked reduction was observed in 20 of 148 patients (14%). Partial reduction was observed in four patients (3%), and no response was seen in five patients (3%). Plunging ranula, lymphangioma, thyroglossal duct cyst, thyroid cyst, auricular hematoma, and salivary mucocele showed better responses to OK-432 therapy than did branchial cleft cyst. Serious complications with OK-432 therapy were infrequent, and the therapy seemed to have no influence on future surgery. Our results confirmed that OK-432 therapy is simple, easy, safe, and effective and can be used as a substitute for surgery in the treatment of various otolaryngological cystic diseases.

Intralesional bleomycin and sodium tetradecyl sulphate for haemangiomas and lymphangiomas.

PubMed

Harjai, Man Mohan; Jha, Manvendu

2012-01-01

To compare the efficacy of intralesional bleomycin and sodium tetradecyl sulphate in treatment of haemangiomas and lymphangiomas. Between July 2007 and May 2009, 120 patients, sixty each of peripheral haemangiomas and lymphangiomas, were administered intralesional injection of bleomycin in a dose of 0.5-1 U/kg in children less than one year of age and 1 to 15 units in children more than one year of age and 1 to 3 ml of 2% sodium tetradecyl sulphate, depending on the size of the lesion at intervals of 14 days. Patients more than 20 years of age and those with diffuse or visceral lesions were excluded from the study. Complete resolution occurred in 16 patients (53%) of haemangiomas and 14 patients (47%) of lymphangiomas treated with bleomycin, while the results were 12 patients (40%) and 10 patients (33%), respectively, in sodium tetradecyl sulphate group. The satisfactory resolution (resolution more than 50%) occurred in eight patients (27%) of haemangiomas and lymphangiomas groups treated with bleomycin, while the results were six patients (20%) and eight patients (27%), respectively, in sodium tetradecyl sulphate group. Poor response rate was observed in six patients (20%) of haemangiomas and eight patients (27%) of lymphangiomas of bleomycin group and 12 patients (40%) of haemangiomas and lymphangiomas in sodium tetradecyl sulphate group. No pulmonary fibrosis or other serious side effects were found. Intralesional bleomycin and sodium tetradecyl sulphate are effective sclerosants in peripheral haemangiomas and lymphangiomas, but bleomycin was found to be more efficacious.

OK-432 (Picibanil) therapy for lymphangiomas in children.

PubMed

Laranne, J; Keski-Nisula, L; Rautio, Riitta; Rautiainen, Markus; Airaksinen, Mari

2002-05-01

Lymphangiomas are benign, soft tumors that most often affect the head and neck area, usually causing marked cosmetic and functional problems. Treatment options include surgery and a large number of different sclerotherapy agents. Surgical treatment is challenging because of the need for complete excision. The risk of damage to surrounding structures or poor cosmetic results is high. Various sclerotherapy agents have been shown to have minimal effects on lymphangiomas. Their use has been associated with severe systemic, local and cosmetic side effects. OK-432 (Picibanil) is a new and promising form of sclerotherapy. An intracystic injection of OK-432 produces a local inflammatory reaction, which leads to resolution of the lesion. We have treated 11 pediatric lymphangioma patients with OK-432 with excellent results: complete regression in six, marked regression in four and no response in one case. Local swelling should be anticipated, especially when treating lesions near the upper airway. We found OK-432 injections to be safe and effective as a first line of treatment for lymphangiomas.

3D-printing aided resection of intratracheal adenoid cystic carcinoma and mediastinal mature cystic teratoma in a 26-year-old female: a case report.

PubMed

Lu, Di; Feng, Siyang; Liu, Xiguang; Dong, Xiaoying; Li, Mei; Wu, Hua; Ren, Pengfei; Diao, Dingwei; Cai, Kaican

2018-02-01

Complete resection remains the ideal treatment for most patients with primary intratracheal tumors. Due to the limit in the resection length, the localization of the tumor during the surgery could be rather difficult if it hasn't invaded out of the trachea, which might be more difficult when there's another tumor in the anterior mediastinum. A 26-year-old woman with intratracheal adenoid cystic carcinoma (ACC) presented with dyspnea. CT scan revealed not only an ACC without extension out of trachea, but also a mediastinal teratoma (MT) near aorta, making the operation much more complicated than usual. To solve this problem, 3D-printing was applied for facilitating surgery plan making and intraoperative locating of ACC. And as we expected, the operation went successfully and the patient achieved full recovery without complications. To the best of our knowledge, the case of both intratracheal ACC and MT in one patient has not been previously reported.

[Sclerotization of orbital lymphangioma with OK-432].

PubMed

Lagrèze, W; Metzger, M; Rössler, J

2014-05-01

Orbital lymphangiomas are mostly congenital, apparent vascular space-occupying lesions, which can lead to disfiguring swelling of the periorbital soft tissues, ocular motility disorders, optic nerve compression and keratopathy. The treatment is challenging because the disease is principally incurable. Lymphangiomatous tissue can be surgically partially reduced or treated by intralesional injection of various sclerosants. In this review we report the successful use of OK-432 for destruction of a macrocystic orbital lymphangioma.

Orchitis reveals an extragonadal primary mediastinal thymic seminoma: a coincidence or not?

PubMed

Tampakis, Athanasios; Tampaki, Ekaterini Christina; Damaskos, Christos; Feretis, Themistoklis; Thymara, Irene; Kontzoglou, Konstantinos; Tomos, Periklis; Kouraklis, Gregory

2017-04-13

Mediastinal thymic seminomas are rare male germ cell tumors with extragonadal origin that appear predominately with a cystic appearance. A 22-year-old male was referred to our department for further investigation of a mediastinal mass discovered incidentally during routine chest X-ray. The patient has denied any symptoms including dyspnea, chest pain, cough, fever, dysphagia, hemoptysis, weight loss, and weakness. His past medical history was remarkable for orchitis, for which he had undergone a bilateral testicular biopsy, without the latter however, indicating the presence of a germ cell tumor or a premalignant lesion. Contrast-enhanced chest computed tomography revealed a lobulated and well-marginated cystic lesion in the anterior mediastinum. Differential diagnosis included mostly a multilocular thymic cyst, a lymphoma, a seminoma, or a soft tissue tumor. Resection of the mass revealed a primary thymic seminoma. A surgical approach for the management of these tumors might be reasonable considering that an extensive sampling is mandatory to gain an appropriate biopsy preoperatively in order to securely confirm or refute the presence of a mediastinal extragonadal tumor. Orchitis might be a sign of a general disorder of the germ cells which might transform in time.

Conjunctival Lymphangioma: A Case Report and Brief Review of the Literature

PubMed Central

Seca, Mariana; Borges, Pedro; Reimão, Pedro; Gomes, Miguel; Meireles, Angelina

2012-01-01

Background. Lymphangioma is a rare venolymphatic lesion, characterized by dilation of lymphatic vessels. It may occur as an isolated lesion or, more often, represent the surface component of a deep orbital lymphangioma. Case. We report a case of a conjunctival lymphangioma on a 58-year-old male that had simultaneously an upper respiratory tract infection. Excision and biopsy confirmed the nature of the lesion, and there has been no relapse to date. Conclusion. Conjunctival lymphangioma is a rare condition in which the diagnose, must be kept in mind in patients with a red eye resistance to topical therapy and in association with an upper respiratory tract infection. Finally, it is also necessary to be aware of possible recurrence of the lesion. PMID:22666617

Lymphangioma circumscriptum, angiokeratoma, or superficial vascular ectasia with epithelial hyperplasia?

PubMed

Katsoulas, Nikolaos; Tosios, Konstantinos I; Argyris, Prokopios; Koutlas, Ioannis G; Sklavounou, Alexandra

2014-08-01

We report a case of lymphangioma circumscriptum (cavernous lymphangioma with epithelial hyperplasia) in a 12-year-old girl, presenting as a papillary tumor on the right dorsal side of her tongue. Microscopic examination found cavernous vascular channels lined by a single layer of CD31(+), podoplanin-positive, CD34(-) endothelial cells that occupied the papillary lamina propria and were accompanied by epithelial hyperplasia. A review of the literature on oral vascular tumors with epithelial hyperplasia, namely, lymphangioma circumscriptum and angiokeratoma, provided information that draws into question the use of these terms. Copyright © 2014 Elsevier Inc. All rights reserved.

/sup 75/Se-selenomethionine scintigraphy in mediastinal diseases

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

Masaoka, A.; Kyo, S.

1978-03-01

Chest scanning with /sup 75/Se-selenomethionine was performed in 59 cases of mediastinal diseases. All cases of vascular diseases, cystic tumors, and benign neurogenic tumor were negatively scanned. Parenchymatous teratoma, thymoma, malignant lymphoma, Castleman's tumor, epithelial tumors, tuberculous lymphadenitis, and sarcoidosis showed high positive rates. In myasthenic thymus without thymoma two out of 15 cases were positive. The scan images of the resected specimens and preoperative chest scannings coincided.

High-grade squamous intraepithelial lesion arising adjacent to vulvar lymphangioma circumscriptum: a tertiary institutional experience

PubMed Central

Bae, Go Eun; Yoon, Gun; Song, Yong Jung; Kim, Hyun-Soo

2016-01-01

Lymphangioma circumscriptum of the vulva occurs in patients who have undergone radical hysterectomy, lymph node dissection, or radiation therapy for management of advanced uterine cancer. Since vulvar lymphangioma circumscriptum typically presents as multiple, grossly verrucous vesicles of various sizes, it may be impossible to clinically distinguish vulvar lymphangioma circumscriptum from other vulvoperineal cutaneous diseases. In the present study, 16 (1.6%) out of the 1,024 vulvar biopsy or excision specimens were diagnosed as lymphangioma circumscriptum. In two (12.5%) out of the 16 cases, unusual histopathological findings were observed. Both patients had previously undergone radical hysterectomy with lymph node dissection and postoperative radiation therapy or concurrent chemoradiation therapy for advanced cervical cancer. Microscopic examination revealed high-grade squamous intraepithelial lesions, which were located immediately adjacent to the normal squamous epithelium covering the dilated subepithelial lymphatic vessels. Further, human papillomavirus genotyping confirmed that both patients were infected with high-risk human papillomavirus. High-grade squamous intraepithelial lesion cannot be grossly distinguished from vulvar lymphangioma circumscriptum because the multiple, verrucous vesicles that constitute the characteristic gross appearance of vulvar lymphangioma circumscriptum hinder its distinction. In this regard, our cases of high-grade squamous intraepithelial lesion, located adjacent to vulvar lymphangioma circumscriptum, support the notion that active surgical excision is necessary for the treatment of vulvar lymphangioma circumscriptum. PMID:27329721

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

A rare case of acquired lymphangioma circumscriptum of the penis.

PubMed

Adikari, S; Philippidou, M; Samuel, M

2017-02-01

Acquired lymphangioma circumscriptum is a rare occurrence on the penis. We report a case of a 47-year-old man who presented with a single lesion of acquired lymphangioma circumscriptum on the penis resembling genital warts. We report the case to increase awareness of this rare condition which may mimic sexually transmitted infections such as genital warts.

Treatment of lymphangiomas with OK-432 (Picibanil).

PubMed

Rautio, Riitta; Keski-Nisula, Leo; Laranne, Jussi; Laasonen, Erkki

2003-01-01

To determine the efficacy of OK-432 sclerotherapy in the treatment of lymphangiomas. The treatment was begun for 14 patients with lymphangioma. The age range of the patients at the time of the first injection was from 10 months to 42 years. Eleven of the lesions involved the head and neck region, two the thorax and one was localized in the extremity. Prior to treatment all patients were investigated with either magnetic resonance imaging, computed tomography, ultrasound or a combination of these modalities. The injections were performed with ultrasound and/or fluoroscopic guidance. Eight patients received OK-432 as first-line treatment; five were treated after surgery and one after medical therapy. On average, 2.2 intracystic injections were performed per patient. Nine of the lesions were macrocystic and five were mixed lesions. Eleven patients showed complete or marked response to the OK-432 sclerotherapy, two patients had moderate shrinkage of their lesions and only one patient showed no response to therapy. Macrocystic lesions showed the best response to therapy. Those patients who received OK-432 as first-line treatment showed complete or marked response. It was found that treatment of lymphangiomas with OK-432 was safe and effective.

[Clinical aspects and therapy of lymphangiomas, hemangiomas and nevi in the area of the head and neck].

PubMed

Drepper, H

1985-07-01

The superficial angiomas and nevi arise from the endothelial cells, the pigment-cell-system or the epidermal cells. Hemangiomas are benign tumours of the endothelial cells appearing predominantly in female newborn infants. Depending on localisation, growth and proliferation there is a tendency for spontaneous involution. For irreversible dysfunction or anatomical deformities surgical resection during the growth phase of the hemangioma is indicated. Surgery may be necessary to improve the functional and esthetic appearance after spontaneous involution causing loose residual skin. Radiotherapy of the lesion is rarely indicated. Systemic steroid therapy in cooperation with the pediatrician should be reserved only for desperate cases such as Kasabach-Merrit-Syndrome. Port wine nevi without scars can be covered with skin tanning cosmetics. Argon-Laser-Therapy is not yet so selective that healing can be achieved with certainty and without scars. Clinical progression requires surgery, especially for racemose angiectasia. Arterial embolization should only be used under special conditions, and then only as pretreatment. Lymphangiomas are mostly angiectatic processes, especially of lymphatic vascular tissue and vessels. Even large cystic lymphangiomas can be treated quite well by surgery, but operations on large diffuse invasive lymphangiomas often cause lymphedema and infection similar to erysipelas leading to pseudorecurrence. The benign malformations of the pigment cell system require clear differentiation from malignant melanoma and its precursors. Malignant melanoma develops more frequently from congenital nevi of the deep type than from other pigmented lesions. Malignant melanomas arising from giant nevi are usually diagnosed too late so that almost all patients die. Removal of giant nevi as early as possible is recommended. The epidermal malformations, too, need accurate diagnosis. Multisymptomatic syndromes such as the Basal-Cell-Nevus-Syndrome, and vascular and

Treatment of lymphangiomas with OK-432 (Picibanil) sclerotherapy: a prospective multi-institutional trial.

PubMed

Giguère, Chantal M; Bauman, Nancy M; Sato, Yutaka; Burke, Diane K; Greinwald, John H; Pransky, Seth; Kelley, Peggy; Georgeson, Keith; Smith, Richard J H

2002-10-01

To describe and to determine the robustness of our study evaluating the efficacy of OK-432 (Picibanil) as a therapeutic modality for lymphangiomas. Prospective, randomized trial and parallel-case series at 13 US tertiary care referral centers. Thirty patients diagnosed as having lymphangioma. Ages in 25 ranged from 6 months to 18 years. Twenty-nine had lesions located in the head-and-neck area. Every patient received a 4-dose injection series of OK-432 scheduled 6 to 8 weeks apart unless a contraindication existed or a complete response was observed before completion of all injections. A control group was observed for 6 months. Successful outcome of therapy was defined as a complete or a substantial (>60%) reduction in lymphangioma size as determined by calculated lesion volumes on computed tomographic or magnetic resonance imaging scans. Overall, 19 (86%) of the 22 patients with predominantly macrocystic lymphangiomas had a successful outcome. OK-432 should be efficacious in the treatment of lymphangiomas. Our study design is well structured to clearly define the role of this treatment agent.

Excellent Result With the Use of Single-Dose OK-432 in Cervical Macrocystic Lymphangioma.

PubMed

Efe, Nihal; Altas, Enver; Mazlumoglu, Muhammet Recai; Aktan, Bulent; Ucuncu, Harun; Eren, Suat; Oner, Fatih

2016-10-01

Though the lymphangioma is a benign neoplasm, it may make an invasion to vital structures by progressively growing. For lymphangioma, which progressed in such a way, surgical treatment has high morbidity and recurrence risk. On these cases, OK-432 is a frequently used sclerotherapy agent. The authors report the result they obtained by the use of single-dose OK-432 on an inoperable pediatric cervical macrocystic lymphangioma case and also their experiences.

[Mediastinal Pancreatic Pseudocyst with Pancreatic Pleural Effusion].

PubMed

Sasajima, Motoko; Kawai, Hideki; Suzuki, Yohei; Saito, Yoshitaro; Eto, Takeshi

2017-06-01

A 72-year-old man with chronic alcohol related pancreatitis was admitted for dyspnea and pain at the upper body. Chest X-ray showed right massive pleural effusion. Chest and abdominal contrast enhanced thin slice computed tomography revealed the route from the pancreatic head reaching the right thoracic cavity via the esophagus hiatus and the communication between the cystic lesion and main pancreatic duct. We drained the pleural effusion that showed abnormally high amylase activity. We diagnosed his illness as mediastinal pancreatic pseudocyst with pancreatic pleural effusion. Endoscopic Nasopancreatic Drainage catheter was placed in the main pancreatic duct, and the pleural effusion disappeared.

[Unsuccessful treatment with OK-432 picibanil for orbital lymphangioma].

PubMed

Lanuza García, A; Bañón Navarro, R; Llorca Cardeñosa, A; Delgado Navarro, C

2012-01-01

Lymphangioma is a malformation of the lymphatic system. The classic approach is surgery. We report a case of orbital lymphangioma in a girl who was given OK-432 to avoid surgery and its complications. OK-432 is a lyophilized mixture of group A Streptococcus pyogenes which produces a fibrosis limited to the lesion with a high cure rate. The main advantages are the easy intra-lesional application. with no scars and or damage of closed areas. Its main disadvantage is a significant local inflammatory reaction. Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

Treatment of lymphangiomas in children: an update of Picibanil (OK-432) sclerotherapy.

PubMed

Greinwald, J H; Burke, D K; Sato, Y; Poust, R I; Kimura, K; Bauman, N M; Smith, R J

1999-10-01

Picibanil (OK-432) is a sclerosing agent derived from a low-virulence strain of Streptococcus pyogenes that induces regression of macrocystic lymphangiomas. This report describes a prospective, nonrandomized trial to evaluate the efficacy of Picibanil in the treatment of 13 affected children ranging in age from 1 to 94 months. On average, 4.1 fluoroscopically guided intracystic injections were performed per child, with an average total dose of 0.56 mg of Picibanil. As judged by physical examination and radiographic studies, 5 children (42%) showed a complete or substantial response, and 2 children (16%) showed an intermediate response. No response was seen in 5 children (42%), 2 of whom had massive craniofacial lymphangioma. Factors that contribute to failure with Picibanil sclerotherapy are the presence of a significant microcystic component to the lesion, massive craniofacial involvement, and previous surgical resection. Macrocystic lymphangiomas of the infratemporal fossa or cervical area have the best response to therapy.

Lymphangioma circumscriptum of the penis: a case report.

PubMed

Kokcam, Ibrahim

2007-06-01

We report a case of lymphangioma circumscriptum of the penis in a 19-year-old male. The lesions developed during puberty and resembled molluscum contagiosum and genital herpes. The case is presented because of its rarity and to increase diagnostic awareness and treatment with non-surgical intervention.

"Occult cutaneous lymphangiectasis": an unusual case of cutaneous lymphangioma.

PubMed

Kakinuma, Hiroshi

2002-01-01

An unusual case of cutaneous lymphatic abnormality is described and named as "occult cutaneous lymphangietasis". A 26-year-old man had noticed pigmented maculae in the left inguinofemoral region and waist, which had gradually increased in number for as long as he could remember. The peculiar distribution of the eruptions and their transient saccular dilatation due to infection suggested that they were of lymphatic origin. Lymphangiography showed the presence of dilated lymphatics in the left inguinofemoral area and correspondence of the distribution of dermal backflow with the locations of the pigmented maculae. The histology is consistent with a diagnosis of lymphangioma. We could find no other reports of cases of cutaneous lymphangioma featuring pigmented maculae as the sole manifestation, although whether the pigmentation is an original clinical feature of this type of lymphatic abnormality is still an open question.

Vulvar lymphangioma circumscriptum: a rare complication of therapy for squamous cell carcinoma of the cervix

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

LaPolla, J.; Foucar, E.; Leshin, B.

1985-11-01

The clinical and pathological features of a case of multifocal lymphangioma circumscriptum of the vulva are reported in a patient with chronic lymphedema of a lower extremity. Ten years previously the patient had been treated for squamous cell carcinoma of the cervix. Although lymphangioma circumscriptum is an extremely rare complication of altered lymphatic drainage, the presence of multiple noninflammatory vesicular appearing lesions in this setting should suggest the correct diagnosis.

Complete Resolution of Retroperitoneal Lymphangioma with a Single Trial of OK-432 in an Infant.

PubMed

Olivieri, Claudio; Nanni, Lorenzo; De Gaetano, Anna Maria; Manganaro, Lucia; Pintus, Claudio

2016-06-01

Retroperitoneal lymphangioma is extremely rare. Although these neoplasms are benign, they can grow progressively with subsequent compression and infiltration of the adjacent structures. Surgical excision is demanding when the lesion surrounds vital structures and it is generally fraught with a high recurrence and morbidity rate. We report the case of a huge retroperitoneal lymphangioma in a newborn treated successfully with intracystic injection of OK-432. Copyright © 2013. Published by Elsevier B.V.

OK-432 sclerotherapy in head and neck lymphangiomas: long-term follow-up result.

PubMed

Yoo, Jae Chul; Ahn, Youngjin; Lim, Yune Syung; Hah, J Hun; Kwon, Tack-Kyun; Sung, Myung-Whun; Kim, Kwang Hyun

2009-01-01

Nonsurgical treatments, such as sclerotherapy have been attempted for head and neck lymphagiomas. Of the available sclerosing agents, picibanil has shown satisfactory short-term treatment results in many studies, but no study has presented long-term treatment results. Accordingly, in the present study, the authors retrospectively reviewed the long-term treatment results of picibanil sclerotherapy. Fifty-five lymphangioma patients who underwent picibanil sclerotherapy were enrolled. Data about initial and long-term response, recurrence, and excision rate were collected. Initial response rates were 83.5 percent and long-term response rates were 76.3 percent. Initial and the long-term response rate were equally good for lymphangioma.

Treatment of lymphangiomas of the head and neck in children by intralesional injection of OK-432 (Picibanil).

PubMed

Brewis, C; Pracy, J P; Albert, D M

2000-04-01

The treatments previously used for lymphangiomas of the head and neck in children-surgery and intralesional injection of sclerosants-are associated with significant morbidity. A new treatment-intralesional injection of OK-432-was used for lymphangiomas of the head and neck in 11 children. The results were total shrinkage in two, marked shrinkage in two, slight shrinkage in five and no response in two. The results were not affected by previous surgery nor by whether aspiration prior to injection was possible. There were no recurrences in those children in whom shrinkage occurred and no child had subsequent surgery following injection. The results of this series support those of previous series showing that OK-432 injection is an effective and safe treatment for lymphangiomas of the head and neck in children.

Bacterial mediastinitis after heart transplantation.

PubMed

Baldwin, R T; Radovancevic, B; Sweeney, M S; Duncan, J M; Frazier, O H

1992-01-01

Bacterial mediastinal abscess or mediastinitis developed in nine (2.5%) of 361 consecutive patients who underwent isolated heart transplantation at the Texas Heart Institute. All nine patients had at least one predisposing factor that may have contributed to the development of mediastinitis. These included insulin-dependent diabetes mellitus, repeat operation for postoperative mediastinal hemorrhage, Staphylococcus aureus pneumonitis, and cardiac allograft rejection in the early postoperative period (less than 30 days), necessitating steroid pulse therapy alone or in combination with murine-derived monoclonal antibody (OKT3). In six of the nine patients, the diagnosis of mediastinitis was made on the basis of clinical findings (unstable sternum and incisional erythema, with or without gross purulence), and in the other three patients, diagnosis was confirmed by computed tomography of the chest. Culture data were unequivocal in all patients; S. aureus was the most frequent (five patients), followed by S. epidermidis (two patients), and Enterobacter cloacae (two patients). Computed tomography-directed percutaneous drainage and systemic antibiotics were successful in treating two of three patients who had stable sternums with mediastinal abscess. In the remaining seven patients, sternal and mediastinal debridement with rewiring of the sternum was successfully applied. No patient required muscle or omental flap coverage, and no patient experienced a recurrence of mediastinitis during an average follow-up period of 35 months (range, 12 to 46 months).

Branchial cysts: an unusual cause of a mediastinal mass: a case report.

PubMed

Kotecha, Vihar; Muturi, Alex; Ruturi, Josiah

2015-09-29

Complex embryological processes form the head and neck of humans. It is not flawless; remnants lead to sinuses or cysts, commonly in the head and neck region. We present the a case of an 8-year-old boy, a primary school pupil, from rural Kenya with chronic cough, wheezing, difficulty in breathing and dyspnea on exertion. He was treated with antibiotics and antitubercular drugs without improvement prior to referral to our hospital. A computed tomography scan of his chest revealed a superior mediastinal mass extending into his neck. A diagnosis of a brachial cleft cyst was made and our patient underwent a successful excision of the mass through a median strenotomy and neck dissection. Branchial cysts of the neck are common, accounting for 20% of pediatric neck masses. Usually they present as a neck mass but in our case it presented as a mediastinal mass, which is a very rare clinical presentation. Surgical excision is the mainstay of treatment. To the surgeon, the embryology and anatomy should be absolutely clear as dissection may be challenging due to the close proximity and variable course of the cystic stalk to major neck vessels and nerves.

Lymphangioma circumscriptum of the penis mimicking venereal lesions.

PubMed

Gupta, S; Radotra, B D; Javaheri, S M; Kumar, B

2003-09-01

Lymphangioma circumscriptum (LC) involving the penis is rare. We report two patients with penile LC. The lesions developed in early infancy in one patient, and during puberty in the other. The lesions resembled molluscum contagiosum in one and genital warts in the other. The first patient was previously treated with a diagnosis of venereal disease. A literature search found only 4 LC patients with penile lesions reported in the English literature. These cases are presented for their rarity, and to increase diagnostic vigilance and desirability of non-intervention.

Mediastinal hemorrhage: An evaluation of radiographic manifestations

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

Woodring, J.H.; Loh, F.K.; Kryscio, R.J.

1984-04-01

Eleven common radiographic signs of mediastinal hemorrhage were evaluated by two observers for the following three patient groups: normal subjects, patients with mediastinal hemorrhage and no arterial injury, and patients with major thoracic arterial injury. Supine chest radiographs were studied in all cases. Four major conclusions can be made based upon these finding. M/C ratio (mediastinal width to chest width), tracheal deviation, left hemothorax, paraspinal line widening, and aorto-pulmonary window opacification do not reliably separate these three groups of patients. The diagnosis of mediastinal hemorrhage may be made if the aortic contour is abnormal or if one of the followingmore » signs is positive: abnormal mediastinal width, apical cap, widening of the right paratracheal stripe, or deviation of the nasogastric tube. Due to interobserver variation, there is good agreement between observers for the following four signs only: transverse mediastinal width, tracheal deviation, nasogastric tube deviation, nasogastric tube deviation, and right paratracheal stripe widening.« less

Highly selective electrocoagulation therapy: an innovative treatment for lymphangioma circumscriptum.

PubMed

Yang, Xi; Jin, Yunbo; Chen, Hui; Li, Suolan; Ma, Gang; Hu, Xiaojie; Qiu, Yajing; Yu, Wenxin; Chang, Lei; Wang, Tianyou; Lin, Xiaoxi

2014-08-01

Lymphangioma circumscriptum (LC) is a type of microcystic lymphatic malformation involving the skin and mucosa that presents as translucent vesicles of varying size with a pink, red, or black hue. Lymphangioma circumscriptum causes not only cosmetic problems but also refractory rupture, infection, lymphorrhea, and bleeding. Various invasive methods, such as surgical excision, lasers, and sclerotherapy, have been used in the past to treat LC with varying success. Herein, we report a new treatment for the management of LC. This study reports the outcomes of 12 patients (aged 4-31 years) with LC treated by electrocoagulation using a special isolated needle. Patient demographics, lesion characteristics, radiologic findings, treatment course, and clinical responses are recorded. All 12 patients who were treated with the highly selective electrocoagulation therapy achieved near-complete clearance. Minimal intra- and postoperative sequelae were observed. The local complications included mild pain (n = 9), proliferous scarring (n = 1), and ulceration (n = 1) with no systemic side effects. The mean follow-up period was 8.25 months (3-14 months). Highly selective electrocoagulation therapy is an innovative, minimally invasive technique that seems to be safe and effective for the treatment of LC; the results from our limited study population seem promising, and the observed complications are acceptable.

Mediastinal pathology and the contributions of Dr. Juan Rosai.

PubMed

Wick, Mark R

2016-09-01

Dr. Juan Rosai is one of the most prolific contributors to the literature on mediastinal pathology, and he has added steadily to that body of work over a 50-year period. Rosai has written several landmark articles in this topical area, including articles on thymic epithelial lesions, mediastinal neuroendocrine tumors, mediastinal lymphoma and other hematopoietic lesions, thymolipoma, thymoliposarcoma, mediastinal solitary fibrous tumor, intrathymic langerhans-cell histiocytosis, mediastinal germ cell neoplasms, and multilocular thymic cyst. This review recounts his role as one of the principal figures in the surgical pathology of mediastinal diseases. Copyright © 2016 Elsevier Inc. All rights reserved.

Mediastinal impalement with a fibreglass sheet

PubMed Central

Wimalachandra, Welege Samantha Buddhika; Asmat, Atasha

2014-01-01

Mediastinal impalement injuries are uncommon and often fatal. There have been very few reported cases of survival following mediastinal impalement. Patients who present with these injuries always undergo operative intervention regardless of their underlying haemodynamic status or associated injuries. We herein present a case of mediastinal impalement injury, where a sheet of fibreglass had fractured the manubrium and entered thffige anterior mediastinum with no associated great vessel injury. The fibreglass sheet was removed via a partial sternotomy and the patient made an uneventful postoperative recovery. PMID:25273944

Mediastinal granuloma: a rare cause of dysphagia.

PubMed

Micic, Dejan; Hogarth, Douglas Kyle; Kavitt, Robert T

2016-06-14

Dysphagia is commonly attributed to disorders arising from dysfunction of the oesophageal mucosa or oesophageal motility. Mediastinal structures causing compression of the oesophagus remain a rare presenting cause of dysphagia. We report a case of a woman presenting with dysphagia to solid foods and associated symptoms of weight loss. Traditional evaluation for dysphagia was unrevealing until cross-sectional imaging suggested a mediastinal obstructive process. The finding of a mediastinal granuloma, distinct from mediastinal fibrosis, as the aetiology of dysphagia is a rare finding, with specific treatment implications. The patient was treated with itraconazole antifungal therapy with an improvement in her symptoms. 2016 BMJ Publishing Group Ltd.

Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review.

PubMed

Tan, Ronald; Martires, Joanne; Kamangar, Nader

2016-01-01

Fibrosing mediastinitis is a rare condition defined by the presence of fibrotic mediastinal infiltrates that obliterate normal fat planes. It is a late complication of a previous granulomatous infection, such as histoplasmosis or tuberculosis (TB). Due to its rarity, fibrosing mediastinitis is often under-recognized, and the clinical presentation is variable and dependent on the extent of infiltration or encasement of structures within the mediastinum. We present a case of fibrosing mediastinitis in a man with a prior history of TB, who presented with progressive dyspnea and was found to have chronic mediastinal soft tissue opacities and pulmonary hypertension. His diagnosis was delayed due to the lack of recognition of this clinical/radiographic entity. Fibrosing mediastinitis is a rare entity usually caused by granulomatous disease. Most cases develop as a late complication of histoplasmosis or TB. The presence of calcified mediastinal soft tissue infiltrates on advanced chest imaging can be diagnostic of fibrosing mediastinitis in patients with a prior history of a granulomatous infection once active processes such as malignancy are excluded.

Transarterial embolization of metastatic mediastinal hepatocellular carcinoma

PubMed Central

Chen, Chia-Chang; Yeh, Hong-Zen; Chang, Chi-Sen; Ko, Chung-Wang; Lien, Han-Chung; Wu, Chun-Ying; Hung, Siu-Wan

2013-01-01

This paper introduces an innovative treatment for extra-hepatic metastasis of hepatocellular carcinoma. A 71-year-old patient had a stable liver condition following treatment for hepatocellular carcinoma, but later developed symptomatic mediastinal metastasis. This rapidly growing mediastinal mass induced symptoms including cough and hoarseness. Serial sessions of transarterial embolization (TAE) successfully controlled this mediastinal mass with limited side effects. The patient’s survival time since the initial diagnosis of the mediastinal hepatocellular carcinoma was 32 mo, significantly longer than the 12 mo mean survival period of patients with similar diagnoses: metastatic hepatocellular carcinoma and a liver condition with a Child-Pugh class A score. Currently, oral sorafenib is the treatment of choice for metastatic hepatocellular carcinoma. Recent studies indicate that locoregional treatment of extra-hepatic metastasis of hepatocellular carcinomas might also significantly improve the prognosis in patients with their primary hepatic lesions under control. Many effective locoregional therapies for extrahepatic metastasis, including radiation and surgical resection, may provide palliative effects for hepatocellular carcinoma-associated mediastinal metastasis. This case report demonstrates that TAE of metastatic mediastinal hepatocellular carcinoma provided this patient with tumor control and increased survival time. This finding is important as it can potentially provide an alternative treatment option for patients with similar symptoms and diagnoses. PMID:23801848

Videothoracoscopy in the treatment of mediastinal cysts

PubMed Central

Brzeziński, Daniel; Kozak, Józef

2014-01-01

Introduction Progress in the development of surgical techniques has led to the growing use of video-assisted thoracoscopic surgery (VATS) techniques in mediastinal cyst treatment. Aim To present our experience of treating mediastinal cysts with the minimally invasive technique. Material and methods Fifty patients with mediastinal cysts were treated from 2001 to 2011. There were 32 women and 18 men. The age of the patients ranged from 17 to 72, the mean age being 42 years. All patients underwent basic preoperative diagnostic tests of the chest: X-ray, computed tomography (CT), bronchoscopy and spirometry; 4 patients underwent endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and 3 fine needle aspiration biopsy (FNAB) of the described lesions. Results The VATS was performed in each case. Conversion was carried out due to superior mediastinal location in 10 cases and pleural adhesions in 3 cases. The partial resection of a cyst was performed in 3 patients. One patient was treated conservatively due to heart failure. In that patient the transthoracic needle aspiration of a cyst under ultrasound guidance using alcoholisation with 76% ethanol with a good effect was performed twice. Cyst recurrence was observed in 1 case. Conclusions The surgical access depends on the location of a cyst. The VATS resection of a superior mediastinal cyst is not always feasible. Surgery of mediastinal cysts is both diagnostic and curative. PMID:25337163

Mediastinal hemangiopericytoma.

PubMed

Horikawa-Kyo, Youko; Tanaka, Takeo; Tanano, Hirofumi; Kitayama, Yasuhiro; Karakawa, Shuhei; Taniyama, Kiyomi

2009-08-01

Mediastinal hemangiopericytoma (HPC) was diagnosed in a 3-year-old female. The incidence of this tumor is rare in children, and few data are available to guide clinical management. The surgical resection was incomplete and she received adjuvant radiation therapy and chemotherapy. The patient is alive without adverse events 6 years after diagnosis. (c) 2009 Wiley-Liss, Inc.

Thymic cystic degeneration, pseudoepitheliomatous hyperplasia, and hemorrhage in a dog with brodifacoum toxicosis.

PubMed

Rickman, B H; Gurfield, N

2009-05-01

Thymic cysts with pseudoepitheliomatous hyperplasia are described in a 7-month-old female American Eskimo Dog that died of complications from brodifacoum poisoning. Grossly, there was hemothorax with marked cranial mediastinal hemorrhage. Histologically, thymic lobules were expanded and distorted by irregular cysts, lined by single to multiple layers of plump to slightly attenuated polygonal squamous epithelial cells supported by a basement membrane (pseudoepitheliomatous hyperplasia). The thymus had a paucity of lymphocytes and lacked corticomedullary differentiation. Extensive hemorrhage within the cysts and thymic parenchyma extended into the adjacent adipose tissue. To the authors' knowledge, this is the first report of cystic thymic degeneration with pseudoepitheliomatous hyperplasia in a nonhuman species.

Lymphatic endothelial cell line (CH3) from a recurrent retroperitoneal lymphangioma.

PubMed

Way, D; Hendrix, M; Witte, M; Witte, C; Nagle, R; Davis, J

1987-09-01

An endothelial cell line derived from a massive recurrent chyle-containing retroperitoneal lymphangioma was isolated in monolayer culture. Scanning and transmission electron microscopy and immunohistochemistry confirmed a close resemblance to blood vascular endothelium with typical cobblestone morphology, positive immunofluorescence staining for endothelial marker Factor VIII-associated antigen and fibronectin, and prominent Weibel-Palade bodies. The endothelial cells also exhibited other ultrastructural features characteristic of lymphatic endothelium, including sparse microvillous surface projections, overlapping intercellular junctions, and abundant intermediate filaments. This endothelial cell line represents a new source of proliferating lymphatic endothelium for future study, including structural and functional comparison to blood vascular endothelium.

Sick sinus syndrome as a complication of mediastinal radiation therapy

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

Pohjola-Sintonen, S.; Toetterman, K.J.K.; Kupari, M.

1990-06-01

A 33-year-old man who had received mediastinal radiation therapy for Hodgkin's disease 12 years earlier developed a symptomatic sick sinus syndrome requiring the implantation of a permanent pacemaker. The sick sinus syndrome and a finding of an occult constrictive pericarditis were considered to be due to the previous mediastinal irradiation. A ventricular pacemaker was chosen because mediastinal radiotherapy also increases the risk of developing atrioventricular conduction defects.

Rare mediastinal leiomyoma in a child.

PubMed

Hakeem, Zubair Ashraf; Rathore, Shyam Singh; Wahid, Abdul

2017-07-01

Leiomyoma of the mediastinum is rare. They are thought to arise from the smooth muscle cells of the media of mediastinal vascular structures and structure containing smooth muscle. Most mediastinal leiomyomas are seen in association with oesophagus in the posterior mediastinum. We present a case of huge leiomyoma in a 1.5 years old male child arising in the lower anterior mediastinum with compression of heart, liver and deformity of rib cage. Median sternotomy and a separate concomitant right mini-thoracotomy was done and tumor excised. Histopathology proved it to be a leiomyoma.

Congenital lymphangioma circumscriptum of vulva presenting as multiple giant mass lesions: a case report and literature review.

PubMed

Akhavan, Setareh; Agah, Jila; Nili, Fatemeh

2018-05-01

A G2L2 33-year-old woman presented to our clinic with large verrucous warty masses in labia major, perinea and the end portion of her spine measuring about 15 × 7, 9 × 7 and 8 × 8 cm, which had been enlarged following puberty and pregnancy. Her right upper and left lower limbs had gross congenital lymphedema. The masses were removed by superficial partial vulvectomy with a qualified margin and repaired without skin graft. Pathology report showed lymphangioma circumscriptum. Several months of follow up revealed normal healing and no recurrence. In conclusion, congenital vulvar lymphangioma, which is a rare disorder, can be highly triggered by hormonal-stimulating situations like puberty and pregnancy. Thus, it is better to visit the affected cases in a timely manner in order to excise these lesions before massive enlargement. In our experience, a superficial partial vulvectomy without skin graft can be a sufficient procedure. Obviously, an appropriate approach during and after surgery would be very important for obtaining a desirable healing. © 2018 Japan Society of Obstetrics and Gynecology.

Aortic Pseudoaneurysm Secondary to Mediastinitis due to Esophageal Perforation

PubMed Central

Zuluaga, Claudia Patricia; Aluja Jaramillo, Felipe; Velásquez Castaño, Sergio Andrés; Rivera Bernal, Aura Lucía; Granada, Julio Cesar; Carrillo Bayona, Jorge Alberto

2016-01-01

Esophageal perforation is a condition associated with high morbidity and mortality rates; it requires early diagnosis and treatment. The most common complication of esophageal rupture is mediastinitis. There are several case reports in the literature of mediastinitis secondary to esophageal perforation and development of aortic pseudoaneurysm as a complication. We report the case of a patient with an 8-day history of esophageal perforation due to foreign body (fishbone) with mediastinitis and aortic pseudoaneurysm. The diagnosis was made using Computed Tomography (CT) with intravenous and oral water-soluble contrast material. An esophagogastroduodenoscopy did not detect the perforation. PMID:26977330

Significance of mediastinal involvement in early stage Hodgkin's disease

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

Mauch, P.; Goodman, R.; Hellman, S.

1978-09-01

Between April 1969 and December 1974, 111 consecutive surgically staged I A and II A patients with supradiaphragmatic Hodgkin's disease were treated at the Joint Center for Radiation Therapy. Patients received 3600 to 4400 rad to mantle and para-aortic--splenic pedicle regions. Median follow-up was 56 months. Fourteen patients developed relapsing Hodgkin's disease and three patients died of possible treatment-related causes, two with acute myocardial infarctions and one with radiation pneumonitis. Patients with mediastinal enlargement greater than one third of the chest diameter have a significantly higher risk (p < 0.01) of developing relapse (9 of 18) than patients with lessermore » or no mediastinal disease (5 of 93). Of the 18 patients with large mediastinal disease, six relapsed in the mediastinum and two in the lung. There continue to be no pelvic extensions in the entire group. There is a 92% relapse-free and 97% overall survival in the 93 patients without extensive mediastinal disease. We continue to recommend mantle and para-aortic--splenic pedicle irradiation for these patients. In view of the large number of relapses in patients with extensive mediastinal disease, we are now treating this subgroup of patients with MOPP chemotherapy in addition to mantle and para-aortic irradiation.« less

Saccular aortic aneurysm that resembled a mediastinal neoplasm

PubMed Central

Nose, Naohiro; Kataoka, Hiroumi; Hamada, Masakatsu; Kosako, Yukio; Matsuno, Yasuji; Ishii, Takahiro

2012-01-01

INTRODUCTION Saccular aortic arch aneurysms in unusual sites may be misdiagnosed as a neoplasm. We present the case of a rare saccular aortic arch aneurysm between trachea and esophagus that resembled a mediastinal neoplasm in the preoperative findings. PRESENTATION OF CASE A 63-year-old male with an abnormal mediastinal shadow on chest X-ray was referred to the hospital. An axial plain computed tomogram of the chest revealed mediastinal soft tissue next to the right side of the aortic arch resembling a neoplasm originating from the gap between the trachea and the esophagus. The coronal view constructed by enhanced 64-row multi detector computed tomography revealed the soft tissue was an aneurysm arising from the inner side of the aortic arch. An aortic arch replacement was performed via a median sternotomy. DISCUSSION A thoracic aortic aneurysm sometimes behaves like a mediastinal neoplasm. The multiple cross-sectional image from multidetector computed tomography was useful for the correct diagnosis of such an aneurysm. CONCLUSION The possibility of an aneurysm should be considered whenever a mass in contact with the aortic wall is identified. PMID:22995656

Isolated mediastinal necrotizing granulomatous lymphadenopathy due to cat-scratch disease.

PubMed

Lovis, A; Clerc, O; Lazor, R; Jaton, K; Greub, G

2014-02-01

We report a patient suffering from cat-scratch disease limited to mediastinal lymphadenitis. Although rare, cat-scratch disease should be considered in the differential diagnosis of mediastinal lymphadenitis, especially when patients were exposed to cats.

A Prospective Multi-Institutional Cohort Study of Mediastinal Infections After Cardiac Operations.

PubMed

Perrault, Louis P; Kirkwood, Katherine A; Chang, Helena L; Mullen, John C; Gulack, Brian C; Argenziano, Michael; Gelijns, Annetine C; Ghanta, Ravi K; Whitson, Bryan A; Williams, Deborah L; Sledz-Joyce, Nancy M; Lima, Brian; Greco, Giampaolo; Fumakia, Nishit; Rose, Eric A; Puskas, John D; Blackstone, Eugene H; Weisel, Richard D; Bowdish, Michael E

2018-02-01

Mediastinal infections are a potentially devastating complication of cardiac operations. This study analyzed the frequency, risk factors, and perioperative outcomes of mediastinal infections after cardiac operations. In 2010, 5,158 patients enrolled in a prospective study evaluating infections after cardiac operations and their effect on readmissions and mortality for up to 65 days after the procedure. Clinical and demographic characteristics, operative variables, management practices, and outcomes were compared for patients with and without mediastinal infections, defined as deep sternal wound infection, myocarditis, pericarditis, or mediastinitis. There were 43 mediastinal infections in 41 patients (cumulative incidence, 0.79%; 95% confidence interval [CI] 0.60% to 1.06%). Median time to infection was 20.0 days, with 65% of infections occurring after the index hospitalization discharge. Higher body mass index (hazard ratio [HR] 1.06; 95% CI, 1.01 to 1.10), higher creatinine (HR, 1.25; 95% CI, 1.13 to 1.38), peripheral vascular disease (HR, 2.47; 95% CI, 1.21 to 5.05), preoperative corticosteroid use (HR, 3.33; 95% CI, 1.27 to 8.76), and ventricular assist device or transplant surgery (HR, 5.81; 95% CI, 2.36 to 14.33) were associated with increased risk of mediastinal infection. Postoperative hyperglycemia (HR, 3.15; 95% CI, 1.32 to 7.51) was associated with increased risk of infection in nondiabetic patients. Additional length of stay attributable to mediastinal infection was 11.5 days (bootstrap 95% CI, 1.88 to 21.11). Readmission rates and mortality were five times higher in patients with mediastinal infection than in patients without mediastinal infection. Mediastinal infection after a cardiac operation is associated with substantial increases in length of stay, readmissions, and death. Reducing these infections remains a high priority, and improving post-operative glycemic management may reduce their risk in patients without diabetes. Copyright © 2018 The

Risk score elaboration for mediastinitis after coronary artery bypass grafting.

PubMed

Magedanz, Ellen Hettwer; Bodanese, Luiz Carlos; Guaragna, João Carlos Vieira da Costa; Albuquerque, Luciano Cabral; Martins, Valério; Minossi, Silvia Daniela; Piccoli, Jacqueline da Costa Escobar; Goldani, Marco Antônio

2010-01-01

The mediastinitis is a serious postoperative complication of cardiac surgery, with an incidence of 0.4 to 5% and mortality between 14 and 47%. Several models were proposed to assess risk of mediastinitis after cardiac surgery. However, most of these models do not evaluate the postoperative morbidity. This study aims to develop a score risk model to predict the risk of mediastinitis for patients undergoing coronary artery bypass grafting. The study sample included data from 2,809 adult patients undergoing coronary artery bypass grafting between January 1996 and December 2007 at Hospital São Lucas -PUCRS. Logistic regression was used to examine the relationship between risk factors and the development of mediastinitis. Data from 1,889 patients were used to develop the model and its performance was evaluated in the remaining data (n=920). The definitive model was created with the data analysis of 2,809 patients. The rate of mediastinitis was 3.3%, with mortality of 26.6%. In the multivariate analysis, five variables remained independent predictors of the outcome: chronic obstructive pulmonary disease, obesity, surgical reintervention, blood transfusion and stable angina class IV or unstable. The area under the ROC curve was 0.72 (95% CI, 0.67-0.78) and P = 0.61. The risk score was constructed for use in daily practice to calculate the rate of mediastinitis after coronary artery bypass grafting. The score includes routinely collected variables and is simple to use.

Mediastinal fibrosis and superior vena cava syndrome.

PubMed

Novella Sánchez, Laura; Sanz Herrero, Francisco; Berraondo Fraile, Javier; Fernández Fabrellas, Estrella

2013-08-01

Superior vena cava syndrome is a clear sign for clinicians of infiltrative mediastinal involvement, usually caused by neoplasms in this location, and it is an indicator of poor prognosis. However, other diseases of benign origin can also cause these alterations. We present the case of a 34-year-old patient who debuted with symptoms of superior vena cava syndrome due to idiopathic mediastinal fibrosis, which presented a torpid evolution and few therapeutic alternatives. Copyright © 2012 SEPAR. Published by Elsevier Espana. All rights reserved.

Risks and benefits in treatment of mediastinal abscess by endobronchial ultrasound-guided transbronchial needle aspiration.

PubMed

Tian, Lei; Krimsky, William S; Wu, Qingchen; Sun, Jiayuan

2017-07-01

Mediastinal abscess is a fatal condition, treatment of mediastinal abscess is with antibiotics and sometimes surgery for debridement and drainage. Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a safe assessment and candidate treatment method of mediastinal lesions. This study aimed to HYPERLINK "javascript:void(0);" discuss risks and benefits in treatment of mediastinal abscess by EBUS-TBNA. We noticed a 56-year-old man with developed bilateral pneumonia and sepsis after puncture of mediastinal abscess by EBUS-TBNA. The patient was successfully treated with a combination of systemic anti-infection treatment and intracavitary administration of antibiotics, antifungal and repeated drainage and lavage via EBUS-TBNA, in 1 year follow-up without recurrence. This study indicated infection spread risk of mediastinal abscess after EBUS-TBNA, and mediastinal abscess was successfully cured by combination of systemic anti-infection and local intervention through EBUS-TBNA. EBUS-TBNA is a potential effective minimally invasive treatment for mediastinal abscess, and it is necessary to be aware of clinical complications after puncture of mediastinal infectious lesions by EBUS-TBNA. © 2015 John Wiley & Sons Ltd.

[Prevention of refractory cough with mediastinal fat to fill the residual cavity after radical systematic mediastinal lymphadenectomy in patients with right lung cancer].

PubMed

Huang, Jia; Luo, Qingquan; Shentu, Yang; Zhao, Xiaojing

2010-10-01

The aim of this study is to analyze the impact on the cough after radical systematic mediastinal lymphadenectomy and prevention of refractory cough with mediastinal fat to fill the residual cavity after radical systematic mediastinal lymphadenectomy. Sixty patients clinically diagnosed of lung cancer were selected according to the adopt standardization, from January 2008 to December 2008. All of the patients were divided into two groups randomly: the filling-fat group and the non-filling-fat group. The surgical information such as operation duration time bleeding volume during operation, post-operation bleeding volume were recorded. After one month, FACT-L and LCQ were completed. There are no remarkably differences between the operation duration time, bleeding volume in operation and 1st postoperation day's drainage volume of the two groups. There's significant difference in the scores of cough at night after taking off the chest tube, as well as in the scores of LCQ after one month and in the scores of last items of FACT-L. Filling the fat of the mediastinal to cover the residual cavity left by completely systematic mediastinal lymphadenectomy can reduce the refractory cough after surgery, and can also improve the quality of the life. It has no effect on the the operation duration time, bleeding volume in operation and 1st post-operation day's drainage volume of the patients.

Disseminated necrotic mediastinitis spread from odontogenic abscess: our experience

PubMed Central

Filiaci, Fabio; Riccardi, Emiliano; Mitro, Valeria; Piombino, Pasquale; Rinna, Claudio; Agrillo, Alessandro; Ungari, Claudio

2015-01-01

Summary Aims Deep neck infections are rare but potentially fatal complication of pulpal abscess of the teeth. If an infection can progress rapidly from a toothache to a life threatening infection, then it is critical that dentists be able to recognize the danger signs and identify the patients who are at risk. Mediastinitis is a severe inflammatory process involving the connective tissues that fills the intracellular spaces and surrounds the organs in the middle of the chest. This pathology has both an acute and a chronic form and, in most cases, it has an infectious etiology. This study want to expose the experience acquired in the Oral and Maxillo-facial Sciences Department, Policlinico Umberto I, “Sapienza” University of Rome, regarding two clinical cases of disseminated necrotizing mediastinitis starting from an odontogenic abscess. Methods We report two clinical cases of disseminated necrotic mediastinitis with two different medical and surgical approaches. The radiographic and photographic documentation of the patients was collected in the pre-and post-operatively. All patients underwent a CT scan and MRI. Results Mediastinitis can result from a serious odontogenic abscess, and the extent of its inflammation process must be never underestimated. Dental surgeons play a key role as a correct diagnosis can prevent further increasing of the inflammation process. Conclusions A late diagnosis and an inadequate draining represent the major causes of the elevated mortality rate of disseminated necrotizing mediastinitis. PMID:26330907

Can post-sternotomy mediastinitis be prevented by a closed incision management system?

PubMed Central

Dohmen, Pascal M.; Markou, Thanasie; Ingemansson, Richard; Rotering, Heinrich; Hartman, Jean M.; van Valen, René; Brunott, Maaike; Kramer, Axel; Segers, Patrique

2014-01-01

Post-sternotomy mediastinitis is a serious complication after cardiothoracic surgery and contribute significantly to post-operative morbidity, mortality, and healthcare costs. Negative pressure wound therapy is today’s golden standard for post-sternotomy mediastinitis treatment. A systematic literature search was conducted at PubMed until October 2012 to analyse whether vacuum-assisted closure technique prevents mediastinitis after clean surgical incisions closure. Today’s studies showed reduction of post-sternotomy mediastinitis including a beneficial socio-economic impact. Current studies, however included only high-risk patients, hence furthermore, larger randomised controlled trials are warranted to clarify the benefit for using surgical incision vacuum management systems in the general patient population undergoing sternotomy and clarify risk factor interaction. PMID:25285263

Primary mediastinal liposarcoma - computed tomography and pathological findings: a case report

PubMed Central

Thomaz, Fabiana Barroso; Guimarães, Anderson Nassar; de Magalhães, Isabela Fernandes; Magalhães, Fabio Vargas; Gonçalves, Letícia Pereira; Domingues, Romeu Cortes

2009-01-01

Liposarcomas are the most common soft tissue sarcoma of adults, and primary mediastinal liposarcomas are rare. We present a case of a 50-year-old man with primary mediastinal liposarcoma without any invasion into the surrounding structures, such as the esophagus, trachea, or left atrium of the heart. Following surgical removal of the liposarcoma, the patient has had no recurrence after one year. Surgical removal is the treatment of choice for a mediastinal liposarcoma; however, careful long-term follow-up is necessary because the recurrence rate is very high. PMID:19918396

Assessment of mediastinal tumors with diffusion-weighted single-shot echo-planar MRI.

PubMed

Razek, Ahmed Abdel; Elmorsy, Ahmed; Elshafey, Mohsen; Elhadedy, Tamer; Hamza, Osama

2009-09-01

To assess the role of diffusion-weighted single-shot echo-planar magnetic resonance imaging (MRI) in patients with mediastinal tumors. Prospective study was conducted on 45 consecutive patients (29 male, 16 female, age 22-66 years, mean 41 years) with mediastinal tumor. They underwent diffusion-weighted single-shot echo-planar MRI of the mediastinum with a b-factor of 0, 300, and 600 sec/mm(2). The apparent diffusion coefficient (ADC) value of the mediastinal tumor was correlated with the histopathological findings. The mean ADC value of malignant mediastinal tumors was 1.09 +/- 0.25 x 10(-3) mm(2)/sec, and of benign tumors was 2.38 +/- 0.56 x 10(-3) mm(2)/sec. There was a significant difference in the mean ADC value between malignant and benign tumors (P = 0.001) and within different grades of malignancy (0.001). When an ADC value of 1.56 x 10(-3) mm(2)/sec was used as a threshold value for differentiating malignant from benign tumor, the best results were obtained with an accuracy of 95%, sensitivity of 96%, specificity of 94%, positive predictive value of 94%, negative predictive value of 96%, and area under the curve of 0.938. The ADC value is a noninvasive parameter that can be used for differentiation of malignant from benign mediastinal tumors and grading of mediastinal malignancy.

A case of candida albicans mediastinitis after heart transplantation successfully treated with caspofungin.

PubMed

Garlicki, Mirosław; Czub, Paweł; Filczak, Krzysztof; Wojdyga, Ryszard; Puchniewicz, Maciej; Labuś, Krzysztof; Ehrlich, Marek P

2006-01-01

Reported here is a case of mediastinitis caused by candida albicans and Staphylococcus aureus following a heart transplantation that was successfully treated with caspofungin, antibiotics and mediastinal lavage. A review of the literature revealed that Candida albicans as a cause of mediastinitis has been rarely described. In the few existing reports, evolution was generally fatal, especially in immunocompromised patients, despite treatment with antifungal drugs and antibiotics.

Thyroid tuberculosis: presenting symptom of mediastinal tuberculous lymphadenitis--an unusual case.

PubMed

Chandanwale, Shirish S; Buch, Archana C; Vimal, Shruti S; Sachdeva, Punita

2014-01-01

Tuberculosis of thyroid gland is extremely rare. It spreads to thyroid by lymphogenous or heamatogenous route or from adjacent focus, either from larynx or cervical and mediastinal adenitis. We report an unusual case of a 33-year-old male with thyroid swelling. Fine needle aspiration (FNA) smears showed epithelioid cells without necrosis and acid fast bacilli (AFB). Subsequent investigation revealed mediastinal tuberculous lymphadenitis on Computerized Tomography (CT) scan. FNA confirmed the diagnosis of mediastinal tuberculous lymphadenitis. We conclude, when epithelioid cells are seen on FNA thyroid, tuberculosis must be ruled out especially in regions where there is high prevalence of tuberculosis.

Descending necrotizing mediastinitis: report of a case following steroid neck injection.

PubMed

Harar, R P S; Cranston, C; Warwick-Brown, N

2002-10-01

Cervical necrotizing fasciitis is a rare, rapidly progressive, severe bacterial infection of the soft tissues of the neck. Uncommonly, it may descend into the mediastinum. We describe a case of descending necrotizing mediastinitis in a young man, where there was diagnostic confusion and delay, with an eventual fatal outcome. A steroid injection for neck pain is thought to be the source of infection. In this case, the signs of mediastinitis were initially masked, and the diagnosis delayed until cardiopulmonary arrest occurred. Early recognition with a low threshold for computed tomography (CT) scanning is essential. Aggressive multidisciplinary therapy with mediastinal drainage is mandatory.

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- ...

Automatic mediastinal lymph node detection in chest CT

NASA Astrophysics Data System (ADS)

Feuerstein, Marco; Deguchi, Daisuke; Kitasaka, Takayuki; Iwano, Shingo; Imaizumi, Kazuyoshi; Hasegawa, Yoshinori; Suenaga, Yasuhito; Mori, Kensaku

2009-02-01

Computed tomography (CT) of the chest is a very common staging investigation for the assessment of mediastinal, hilar, and intrapulmonary lymph nodes in the context of lung cancer. In the current clinical workflow, the detection and assessment of lymph nodes is usually performed manually, which can be error-prone and timeconsuming. We therefore propose a method for the automatic detection of mediastinal, hilar, and intrapulmonary lymph node candidates in contrast-enhanced chest CT. Based on the segmentation of important mediastinal anatomy (bronchial tree, aortic arch) and making use of anatomical knowledge, we utilize Hessian eigenvalues to detect lymph node candidates. As lymph nodes can be characterized as blob-like structures of varying size and shape within a specific intensity interval, we can utilize these characteristics to reduce the number of false positive candidates significantly. We applied our method to 5 cases suspected to have lung cancer. The processing time of our algorithm did not exceed 6 minutes, and we achieved an average sensitivity of 82.1% and an average precision of 13.3%.

Angiography Before Posterior Mediastinal Tumor Resection: Selection Criteria and Patient Outcomes.

PubMed

Madariaga, Maria Lucia; Borges, Lawrence F; Rabinov, James D; Chang, David C; Lanuti, Michael; Mathisen, Douglas J; Gaissert, Henning A

2018-04-01

Resection of posterior mediastinal tumors may be complicated by bleeding or neurologic injury. Preoperative spinal angiography of mediastinal tumors has been underreported or not commonly practiced. This study evaluated the selection criteria and outcomes of patients with posterior mediastinal tumors who underwent preoperative angiography and embolization. This was a single-institution retrospective study of patients with posterior mediastinal tumors from 2002 to 2016. Multilevel spinal angiography was performed, with or without selective arterial embolization of vascular supply, in patients selected by thoracic or neurologic surgeons. Ten of 87 patients (11%) with posterior mediastinal tumors underwent preoperative angiography. A mean of 11 arteries (range, 2 to 25) were studied. Embolization in 7 of 10 patients successfully occluded 1 to 3 arteries. There was no significant difference in age, sex, body mass index, American Society of Anesthesiologists Physical Status Classification, operative time, operative blood loss, complications, or death between patients with or without angiography. Patients who underwent angiography had larger tumors (1,490 vs 97 cm 3 , p < 0.0001), involvement of the neuroforamen (44% vs 10%, p < 0.05), and a longer hospital stay (9 vs 4.5 days, p < 0.05). Angiography was complicated in 1 patient by vocal cord ulceration after intubation of a tumor-compressed trachea. Shared blood supply between the tumor and the spinal cord precluded embolization and tumor resection in 1 patient. Use of angiography increased over time. Selective preoperative angiography for evaluation of posterior mediastinal tumors identifies arterial variations, threatened spinal arteries, and targets for embolization. The specific role of angiography and embolization requires further investigation to standardize indications and protocols for the number of arteries examined. Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights

Giant mediastinal haemangiopericytoma: an uncommon case.

PubMed

Agrawal, D; Lahiri, T K; Lakhotia, S; Singh, Deepak

2014-01-01

Haemangiopericytoma is a rare soft tissue tumour characterised by tightly packed tumour cells situated around thin walled endothelial lined vascular channels, ranging from capillary sized vessels to large gaping sinusoidal spaces. The tumour cells are surrounded by reticulin and are negative for muscle, nerve and epithelial markers. The diagnosis of extra-pulmonary intra-thoracic, extra-pleural mediastinal mass is difficult. It constitutes only 6% of all primary tumours and cysts of the mediastinum. We report the rare occurrence of primary intra-thoracic, extra-pulmonary mediastinal haemangiopericytoma of mesenchymal origin with perivascular localisation. The patient underwent right postero-lateral thoracotomy and post-operatively received chemotherapy with adriamycin (60 mg/m2) on day 1 and ifosfamide (1.5 g/m2) on day 1 to 3. Thirty-seven months after the operation, the patient has been well with evidence of a single recurrence in the left lower lobe.

Giant Leiomyoma Arising from the Mediastinal Pleura: A Case Report.

PubMed

Haratake, Naoki; Shoji, Fumihiro; Kozuma, Yuka; Okamoto, Tatsuro; Maehara, Yoshihiko

2017-06-20

This report presents a rare case involving a patient with a giant leiomyoma originating from the mediastinal pleura. The patient underwent a medical examination, and chest radiography revealed a giant tumor. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a well demarcated, heterogeneous mass which seemed to originate from the posterior mediastinum. Positron emission tomography (PET) showed the uptake of this tumor with a standardized uptake value of 4.9. We suspected that this tumor was a solitary fibrous tumor, and the patient underwent a surgical resection. Intraoperative exploration revealed a well-encapsulated tumor measuring 15 × 11 cm that appeared to originate from the mediastinal pleura. Immunohistochemical findings revealed a benign leiomyoma. We finally diagnosed the patient with a mediastinal leiomyoma. The present report describes CT, MRI, and PET findings of leiomyoma, and presents a review of relevant literature.

Pulmonary and mediastinal metastases of a vaccination-site sarcoma in a cat.

PubMed

Rudmann, D G; Van Alstine, W G; Doddy, F; Sandusky, G E; Barkdull, T; Janovitz, E B

1996-07-01

Sarcomas at vaccination sites in cats were first reported in 1992. Recent retrospective studies have confirmed an association between these vaccination-site sarcomas (VSS) and feline leukemia virus (FeLV) and/ or rabies vaccines. In most cases, VSS are locally invasive fibrosarcomas that tend to recur but rarely metastasize. We report the mediastinal and pulmonary metastases of a VSS in a FeLV-and feline immunodeficiency virus-negative, 8-year-old, domestic short-haired cat. The primary sarcoma was removed from an interscapular vaccination site and diagnosed as a VSS 3 months prior to radiographic lesions suggestive of pulmonary and mediastinal metastases. At necropsy, there were multiple pulmonary and mediastinal nodules that histologically and ultrastructurally were fibrosarcomas, cytomorphologically similar to the VSS. In addition, immunohistochemical staining patterns of the VSS and metastatic sites were consistent with that described for VSS. Recent reports of pulmonary and mediastinal metastases of interscapular VSS emphasize the importance of early diagnosis and treatment of these tumors.

Descending necrotizing Mediastinitis caused by Kocuria rosea: a case report

PubMed Central

2013-01-01

Background Kocuria species are gram-positive, non-pathogenic commensals. However, in immunocompromised patients such as transplant recipients, cancer patients, or patients with chronic medical conditions, they can cause opportunistic infections. Case presentation We report the first case of descending necrotizing mediastinitis in a 58-year-old, relatively healthy woman caused by Kocuria rosea. Conclusion Descending necrotizing mediastinitis due to Kocuria rosea can be successfully treated with prompt surgical drainage combined with antimicrobial therapy. PMID:24112281

A three-dimensional mediastinal model created with rapid prototyping in a patient with ectopic thymoma.

PubMed

Akiba, Tadashi; Nakada, Takeo; Inagaki, Takuya

2015-01-01

Preoperative three-dimensional (3D) imaging of a mediastinal tumor using two-dimensional (2D) axial computed tomography is sometimes difficult, and an unexpected appearance of the tumor may be encountered during surgery. In order to evaluate the preoperative feasibility of a 3D mediastinal model that used the rapid prototyping technique, we created a model and report its results. The 2D image showed some of the relationship between the tumor and the pericardium, but the 3D mediastinal model that was created using the rapid prototyping technique showed the 3D lesion in the outer side of the extrapericardium. The patient underwent a thoracoscopic resection of the tumor, and the pathological examination showed a rare middle mediastinal ectopic thymoma. We believe that the construction of mediastinal models is useful for thoracoscopic surgery and other complicated surgeries of the chest diseases.

Sonographic findings in horses with mediastinal lymphosarcoma: 13 cases (1985-1992).

PubMed

Garber, J L; Reef, V B; Reimer, J M

1994-11-15

Sonographic findings correlated with necropsy findings in 8 of 9 horses in which the cranial portion of the mediastinum was evaluated by use of both methods. Cranial mediastinal masses were imaged as multilobular and homogeneously hypoechoic; a complex echogenic pattern was observed with necrosis within the mass. Pleural effusion was a common finding in horses with mediastinal lymphosarcoma. Cytologic evaluation of pleural fluid samples was useful in diagnosis of lymphosarcoma in 10 of 12 horses that had thoracentesis. Sonographic examination of the thorax and cranial portion of the mediastinum can aid in the diagnosis of mediastinal lymphosarcoma in horses. Such examination should be performed in horses with clinical signs of cranial vena cava obstruction in which pleural effusion is detected, or when thoracic lymphosarcoma is suspected.

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

A Three-Dimensional Mediastinal Model Created with Rapid Prototyping in a Patient with Ectopic Thymoma

PubMed Central

Nakada, Takeo; Inagaki, Takuya

2014-01-01

Preoperative three-dimensional (3D) imaging of a mediastinal tumor using two-dimensional (2D) axial computed tomography is sometimes difficult, and an unexpected appearance of the tumor may be encountered during surgery. In order to evaluate the preoperative feasibility of a 3D mediastinal model that used the rapid prototyping technique, we created a model and report its results. The 2D image showed some of the relationship between the tumor and the pericardium, but the 3D mediastinal model that was created using the rapid prototyping technique showed the 3D lesion in the outer side of the extrapericardium. The patient underwent a thoracoscopic resection of the tumor, and the pathological examination showed a rare middle mediastinal ectopic thymoma. We believe that the construction of mediastinal models is useful for thoracoscopic surgery and other complicated surgeries of the chest diseases. PMID:24633133

Mediastinal Adenopathy in India: Through the Eyes of Endobranchial Ultrasound.

PubMed

Dhamija, Amit; Basu, Arup; Sharma, Vishal; Bakshi, Pooja; Verma, Kusum

2015-09-01

Aetiology of mediastinal adenopathy is likely to vary with geographic location and socioeconomic status of a population. Whilst most of adenopathy in the West could be attributed to malignant disorders, causes of the same in a developing country like India has not been extensively studied earlier due to lack of less invasive tools to sample these nodes for cytological and microbiological analysis. Endobronchial ultrasound (EBUS) helps us reach these nodes as a minimally invasive procedure to take aspirations under real-time ultrasound guidance. The aim of the present study is to study the aetiology of mediastinal adenopathy in our population with the help of EBUS. This was a retrospective analysis of all EBUS procedures done by the authors and the diagnosis thus obtained at Sir Ganga Ram Hospital, New Delhi, India between April 2010 and December 2011. A total of 300 patients underwent EBUS in the above period. Most common aetiology encountered in our population was a granulomatous disorder (53% cases) like tuberculosis and sarcoidosis whilst malignancy was third in order of diagnosis (17% cases). Lymph node enlargement due to anthracosis was another uncommon aetiology encountered in the study (5% cases). Benign granulomatous disorders like tuberculosis and sarcoidosis are the most common causes of mediastinal adenopathy in our population. EBUS is proving its worth for diagnosing mediastinal adenopathy. © Journal of the Association of Physicians of India 2011.

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.

Post-surgical mediastinitis due to carbapenem-resistant Enterobacteriaceae: Clinical, epidemiological and survival characteristics.

PubMed

Abboud, C S; Monteiro, J; Stryjewski, M E; Zandonadi, E C; Barbosa, V; Dantas, D; Sousa, E E; Fonseca, M J; Jacobs, D M; Pignatari, A C; Kiffer, C; Rao, G G

2016-05-01

Invasive infections due to carbapenem-resistant Enterobacteriaceae (CRE), including polymyxin-resistant (PR-CRE) strains, are being increasingly reported. However, there is a lack of clinical data for several life-threatening infections. Here we describe a cohort of patients with post-surgical mediastinitis due to CRE, including PR-CRE. This study was a retrospective cohort design at a single cardiology centre. Patients with mediastinitis due to CRE were identified and were investigated for clinically relevant variables. Infecting isolates were studied using molecular techniques. Patients infected with polymyxin-susceptible CRE (PS-CRE) strains were compared with those infected with PR-CRE strains. In total, 33 patients with CRE mediastinitis were studied, including 15 patients (45%) with PR-CRE. The majority (61%) were previously colonised. All infecting isolates carried blaKPC genes. Baseline characteristics of patients with PR-CRE mediastinitis were comparable with those with PS-CRE mediastinitis. Of the patients studied, 70% received at least one agent considered active in vitro and most patients received at least three concomitant antibiotics. Carbapenem plus polymyxin B was the most common antibiotic combination (73%). Over 90% of patients underwent surgical debridement. Overall, in-hospital mortality was 33% and tended to be higher in patients infected with PR-CRE (17% vs. 53%; P=0.06). In conclusion, mediastinitis due to CRE, including PR-CRE, can become a significant challenge in centres with CRE and a high cardiac surgery volume. Despite complex antibiotic treatments and aggressive surgical procedures, these patients have a high mortality, particularly those infected with PR-CRE. Copyright © 2016. Published by Elsevier B.V.

Huge mediastinal liposarcoma resected by clamshell thoracotomy: a case report.

PubMed

Toda, Michihito; Izumi, Nobuhiro; Tsukioka, Takuma; Komatsu, Hiroaki; Okada, Satoshi; Hara, Kantaro; Ito, Ryuichi; Shibata, Toshihiko; Nishiyama, Noritoshi

2017-12-01

Liposarcoma is the single most common soft tissue sarcoma. Because mediastinal liposarcomas often grow rapidly and frequently recur locally despite adjuvant chemotherapy and radiotherapy, they require complete excision. Therefore, the feasibility of achieving complete surgical excision must be carefully considered. We here report a case of a huge mediastinal liposarcoma resected via clamshell thoracotomy. A 64-year-old man presented with dyspnea on effort. Cardiomegaly had been diagnosed 6 years previously, but had been left untreated. A computed tomography scan showed a huge (36 cm diameter) anterior mediastinal tumor expanding into the pleural cavities bilaterally. The tumor comprised mostly fatty tissue but contained two solid areas. Echo-guided needle biopsies were performed and a diagnosis of an atypical lipomatous tumor was established by pathological examination of the biopsy samples. Surgical resection was performed via a clamshell incision, enabling en bloc resection of this huge tumor. Although there was no invasion of surrounding organs, the left brachiocephalic vein was resected because it was circumferentially surrounded by tumor and could not be preserved. The tumor weighed 3500 g. Pathologic examination of the resected tumor resulted in a diagnosis of a biphasic tumor comprising dedifferentiated liposarcoma and non-adipocytic sarcoma with necrotic areas. The patient remains free of recurrent tumor 20 months postoperatively. Clamshell incision provides an excellent surgical field and can be performed safely in patients with huge mediastinal liposarcomas.

Stereotactic body radiation therapy for isolated hilar and mediastinal non-small cell lung cancers.

PubMed

Horne, Zachary D; Richman, Adam H; Dohopolski, Michael J; Clump, David A; Burton, Steven A; Heron, Dwight E

2018-01-01

The seminal phase II trial for pulmonary stereotactic body radiation therapy (SBRT) suggested that SBRT to central lesions resulted in unacceptable toxicity. Alternative dose-fractionation schemes have been proposed which may improve safety without compromise of efficacy. We report our institutional outcomes of SBRT for hilar/mediastinal non-small cell lung cancer (NSCLC). A retrospective review was conducted of patients with NSCLC in a hilar or mediastinal nodal station which was treated with SBRT. Patients presented with a lesion involving the hilum or mediastinum from primary or oligorecurrent NSCLC. Kaplan-Meier with log-rank testing and Cox analysis were utilized for outcomes analysis. From 2008-2015, 40 patients with median age of 70 were treated with SBRT for primary/oligorecurrent hilar/mediastinal NSCLC with median follow-up of 16.4 months. 85% presented with oligorecurrent disease at a median of 22.4 months following definitive therapy. The aortico-pulmonary window was the target in 40%, the hilum in 25%, lower paratracheal in 20%, subcarinal in 10%, and prevascular in 5%. The median dose was 48Gy in 4 fractions (range: 35-48Gy in 4-5 fractions). Median overall (OS) and progression-free (PFS) survivals were 22.7 and 13.1 months, respectively. Two-year local control was 87.7% and not significantly different between hilar and mediastinal targets. Median PFS was significantly improved in patients with hilar vs mediastinal nodal targets: 33.3 vs 8.4 months, respectively (p=0.031). OS was not statistically different between hilar and mediastinal targets (p=0.359). On multivariable analysis, hilar vs mediastinal target predicted for PFS (HR 3.045 95%CI [1.044-8.833], p=0.042), as did shorter time to presentation in patients with oligorecurrence (HR 0.983 [95%CI 0.967-1.000], p=0.049). Acute grade 3+ morbidity was seen in 3 patients (hemoptysis, pericardial/pleural effusion, heart failure) and late grade 3+ morbidity (hemoptysis) in 1 patient. Hilar/mediastinal

A Life-Threatening Mediastinal Hematoma After Central Venous Port System Implantation

PubMed Central

Sarach, Janine; Zschokke, Irin; Melcher, Gian A.

2015-01-01

Patient: Female, 68 Final Diagnosis: Mediastinal hematoma Symptoms: Agitation • severe hemodynamic instability • severe respiratory distress Medication: — Clinical Procedure: Cardiopulmonary resuscitation • reintubation • thoracic drain Specialty: Surgery Objective: Diagnostic/therapeutic accidents Background: We report a case of surgical central venous port system implantation using Seldinger’s technique with a life-threatening mediastinal hematoma due to the perforation of the superior vena cava. Case Report: A 68-year-old woman was admitted to our institution for port implantation. Open access to the cephalic vein and 2 punctures of the right subclavian vein were unsuccessful. Finally, the port catheter could be placed into the superior vena cava using Seldinger’s technique. As blood aspiration via the port catheter was not possible, fluoroscopy was performed, revealing mediastinal contrast extravasation without contrasting the venous system. A new port system could be placed in the correct position without difficulties. After extubation, the patient presented with severe respiratory distress and required consecutive cardiopulmonary resuscitation and reintubation. The CT scan showed a significant hematoma in the lower neck and posterior mediastinum with tracheal compression. We assumed a perforation of the superior vena cava with the tip of the guidewire using Seldinger’s technique. Long-term intensive treatment with prolonged ventilation and tracheotomy was necessary. The port system had to be subsequently explanted due to infection. Conclusions: Mediastinal hematoma is a rare but life-threatening complication associated with central venous catheterization using Seldinger’s technique. Perforation occurs most often during central venous catheterization in critical care. Mediastinal hematoma is an example of a mechanical complication occurring after central venous catheterization, which has been described only a few times in the literature to

Tracheal Compression Caused by a Mediastinal Hematoma After Interrupted Aortic Arch Surgery.

PubMed

Hua, Qingwang; Lin, Zhiyong; Hu, Xingti; Zhao, Qifeng

2017-08-03

Congenital abnormalities of the aortic arch include interrupted aortic arch (IAA), coarctation of the aorta (CoA), and double aortic arch (DAA). Aortic arch repair is difficult and postoperative complications are common. However, postoperative tracheobronchial stenosis with respiratory insufficiency is an uncommon complication and is usually caused by increased aortic anastomotic tension. We report here a case of tracheal compression by a mediastinal hematoma following IAA surgery. The patient underwent a repeat operation to remove the hematoma and was successfully weaned off the ventilator.In cases of tracheobronchial stenosis after aortic arch surgery, airway compression by increased aortic anastomotic tension is usually the first diagnosis considered by clinicians. Other causes, such as mediastinal hematomas, are often ignored. However, the severity of symptoms with mediastinal hematomas makes this an important entity.

Early experience using the da Vinci Surgical System for the treatment of mediastinal tumors.

PubMed

Kajiwara, Naohiro; Taira, Masahiro; Yoshida, Koichi; Hagiwara, Masaru; Kakihana, Masatoshi; Usuda, Jitsuo; Uchida, Osamu; Ohira, Tatsuo; Kawate, Norihiko; Ikeda, Norihiko

2011-10-01

The da Vinci Surgical System has been used in only a few cases for treating mediastinal tumors in Japan. Recently, we used the da Vinci Surgical System for various types of anterior and middle mediastinal tumors in clinical practice. We report our early experience using the da Vinci Surgical System. Seven patients gave written informed consent to undergo robotic surgery for mediastinal tumor dissection using the da Vinci Surgical System. We evaluated the safety and feasibility of this system for the surgical treatment of mediastinal tumors. Two specialists in thoracic surgery who are certified to use the da Vinci S Surgical System and another specialist acted as an assistant performed the tumor dissection. We were able to access difficult-to-reach areas, such as the mediastinum, safely. All the resected tumors were classified as benign tumors histologically. The average da Vinci setting time was 14.0 min, the average working time was 55.7 min, and the average overall operating time was 125.9 min. The learning curve for the da Vinci setup and manipulation time was short. Robotic surgery enables mediastinal tumor dissection in certain cases more safely and easily than conventional video-assisted thoracoscopic surgery and less invasively than open thoracotomy.

Near-infrared intraoperative imaging during resection of an anterior mediastinal soft tissue sarcoma.

PubMed

Predina, Jarrod D; Newton, Andrew D; Desphande, Charuhas; Singhal, Sunil

2018-01-01

Sarcomas are rare malignancies that are generally treated with multimodal therapy protocols incorporating complete local resection, chemotherapy and radiation. Unfortunately, even with this aggressive approach, local recurrences are common. Near-infrared intraoperative imaging is a novel technology that provides real-time visual feedback that can improve identification of disease during resection. The presented study describes utilization of a near-infrared agent (indocyanine green) during resection of an anterior mediastinal sarcoma. Real-time fluorescent feedback provided visual information that helped the surgeon during tumor localization, margin assessment and dissection from mediastinal structures. This rapidly evolving technology may prove useful in patients with primary sarcomas arising from other locations or with other mediastinal neoplasms.

Minimalist video-assisted thoracic surgery biopsy of mediastinal tumors.

PubMed

Tacconi, Federico; Rogliani, Paola; Cristino, Benedetto; Gilardi, Francesco; Palombi, Leonardo; Pompeo, Eugenio

2016-12-01

Mediastinal tumors often require surgical biopsy to achieve a precise and rapid diagnosis. However, subjects with mediastinal tumors may be unfit for general anesthesia, particularly when compression of major vessels or airways does occur. We tested the applicability in this setting of a minimalist (M) uniportal, video-assisted thoracic surgery (VATS) strategy carried out under locoregional anesthesia in awake patients (MVATS). We analyzed in a comparative fashion including propensity score matching, data from a prospectively collected database of patients who were offered surgical biopsy for mediastinal tumors through either MVATS or standard VATS. Tested outcome measures included feasibility, diagnostic yield, and morbidity. A total of 24 procedures were performed through MVATS. Diagnostic yield was 100%. Median hospital stay and time interval to oncologic treatment were 2 days (IQR, 2-3 days) and 7 days (IQR, 5.5-11.5 days), respectively. At overall comparison (MVATS, N=24 vs. VATS, N=23), there was a significant difference in both frequency and severity of postoperative complication as measured by Clavien-Dindo classification (P<0.006). In a propensity score matched comparison (8 patients per group), grade 3 or 4 complications requiring aggressive management were found only in the general anesthesia group. Global time spent in the operating room was shorter in the MVATS group (P=0.05). Time interval to oncological treatment was the same between groups. Other differences were also found in SIRS score (P=0.05) and PaO 2 /FiO 2 (P=0.04) thus suggesting better adaption to perioperative stress. MVATS biopsy appears to be a reliable tool to optimize diagnostic assessment in patients with mediastinal tumors. It can offer high diagnostic accuracy due to large tissue samples, while reducing morbidity rate compared to the same operation under general anesthesia. More robust evaluation is needed to define the appropriateness of MVATS in this specific clinical setting.

Surgical management of cervico-mediastinal goiters: Our experience and review of the literature.

PubMed

Di Crescenzo, V; Vitale, M; Valvano, L; Napolitano, F; Vatrella, A; Zeppa, P; De Rosa, G; Amato, B; Laperuta, P

2016-04-01

We analyze and discuss the clinical presentation, the diagnostic procedures and the surgical technique in relation to post-operative complications and results in cervico-mediastinal thyroid masses admitted in Thoracic Surgery Unit of AOU Second University of Naples from 1991 to 2006 and in Thoracic Surgery Unit of AOU "S. Giovanni di Dio & Ruggi D'Aragona" of Salerno over a period of 3 years (2011-2014). We reviewed 97 patients who underwent surgical treatment for cervico-mediastinal goiters. 47 patients (49.2%) had cervico-mediastinal goiter, 40 patients (40%) had mediastino-cervical goiter and 10 patients (10.8%) had mediastinal goiter. 73 cases were prevascular goiters and 24 were retrovascular goiters. We performed total thyroidectomy in 40 patients, subtotal thyroidectomy in 46 patients and in 11 cases the resection of residual goiter. In 75 patients we used only a cervical approach, in 21 patients the cervical incision was combined with median sternotomy and in 1 patient with transverse sternotomy. Three patients (3.1%) died in the postoperative period (2 cardio-respiratory failure and 1 pulmonary embolism). The histologic study revelead 8 (7.7%) carcinomas. Postoperative complications were: dyspnea in 9 cases (10.7%), transient vocal cord paralysis in 6 patients (9.2%), temporary hypoparathyroidism in 9 patients (9.2%) and kidney failure in 1 case (0.9%). The presence of a cervico-mediastinal thyroid mass with or without respiratory distress requires a surgical excision as the only treatment option. Thyroid masses extending to the mediastinum can be excised successfully by cervical incision. Bipolar approach (cervical incision and sternotomy) has an excellent outcome, achieving a safe resection, especially in large thyroid masses extending to the mediastinum with close relations to mediastinal structures and in some limited cases (carcinoma, thyroiditis, retrovascular goiter, ectopic goiter). Postoperative mortality and morbidity is very low, independent of

Supradiaphragmatic Hodgkin's disease: significance of large mediastinal masses

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

Prosnitz, L.R.; Curtis, A.M.; Knowlton, A.H.

In order to assess the significance of large mediastinal masses in patients with Hodgkin's disease, we analyzed all patients with pathological stage (PS) IA or IIA disease evaluated and treated at Yale between 1969 and 1978. There were 131 such patients treated initially with radical radiotherapy only, combination chemotherapy being reserved for those who failed radiation. Actuarial 5 and 10 year survivals were 95%. The presence of a mediastinal mass regardless of size did not affect survival. Relapse-free survival was 77% at 5 years, 74% at 10 years in the entire group. Patients with any mediastinal involvement had a 65%more » relapse-free survival, 72% if the mass was < 33% of transverse chest diameter, 55% if the mass was > 33%. These differences are suggestive of a greater tendency of such patients to fail radiotherapy but the differences were not statistically significant. Patients who did fail radiotherapy were for the most part successfully retreated with combined modality therapy (chemotherapy and radiation), accounting for the most part successfully retreated with combined modality therapy (chemotherapy and radiation), accounting for the overall survival of 95%. Only 6 patients died of causes related to Hodgkin's disease and 2 of these deaths were related to combined modality therapy complications. Because of the serious potential long term consequences of combined modality treatment, it should be used with great caution and on an individual basis only in PSIA and IIA patients.« less

Prognostic Significance of Tumor Size of Small Lung Adenocarcinomas Evaluated with Mediastinal Window Settings on Computed Tomography

PubMed Central

Sakao, Yukinori; Kuroda, Hiroaki; Mun, Mingyon; Uehara, Hirofumi; Motoi, Noriko; Ishikawa, Yuichi; Nakagawa, Ken; Okumura, Sakae

2014-01-01

Background We aimed to clarify that the size of the lung adenocarcinoma evaluated using mediastinal window on computed tomography is an important and useful modality for predicting invasiveness, lymph node metastasis and prognosis in small adenocarcinoma. Methods We evaluated 176 patients with small lung adenocarcinomas (diameter, 1–3 cm) who underwent standard surgical resection. Tumours were examined using computed tomography with thin section conditions (1.25 mm thick on high-resolution computed tomography) with tumour dimensions evaluated under two settings: lung window and mediastinal window. We also determined the patient age, gender, preoperative nodal status, tumour size, tumour disappearance ratio, preoperative serum carcinoembryonic antigen levels and pathological status (lymphatic vessel, vascular vessel or pleural invasion). Recurrence-free survival was used for prognosis. Results Lung window, mediastinal window, tumour disappearance ratio and preoperative nodal status were significant predictive factors for recurrence-free survival in univariate analyses. Areas under the receiver operator curves for recurrence were 0.76, 0.73 and 0.65 for mediastinal window, tumour disappearance ratio and lung window, respectively. Lung window, mediastinal window, tumour disappearance ratio, preoperative serum carcinoembryonic antigen levels and preoperative nodal status were significant predictive factors for lymph node metastasis in univariate analyses; areas under the receiver operator curves were 0.61, 0.76, 0.72 and 0.66, for lung window, mediastinal window, tumour disappearance ratio and preoperative serum carcinoembryonic antigen levels, respectively. Lung window, mediastinal window, tumour disappearance ratio, preoperative serum carcinoembryonic antigen levels and preoperative nodal status were significant factors for lymphatic vessel, vascular vessel or pleural invasion in univariate analyses; areas under the receiver operator curves were 0.60, 0.81, 0

Systematic mediastinal lymphadenectomy or mediastinal lymph node sampling in patients with pathological stage I NSCLC: a meta-analysis.

PubMed

Dong, Siyuan; Du, Jiang; Li, Wenya; Zhang, Shuguang; Zhong, Xinwen; Zhang, Lin

2015-02-01

To evaluate the evidence comparing systematic mediastinal lymphadenectomy (SML) and mediastinal lymph node sampling (MLS) in the treatment of pathological stage I NSCLC using meta-analytical techniques. A literature search was undertaken until January 2014 to identify the comparative studies evaluating 1-, 3-, and 5-year survival rates. The pooled odds ratios (OR) and the 95 % confidence intervals (95 % CI) were calculated with either the fixed or random effect models. One RCT study and four retrospective studies were included in our meta-analysis. These studies included a total of 711 patients: 317 treated with SML, and 394 treated with MLS. The SML and the MLS did not demonstrate a significant difference in the 1-year survival rate. There were significant statistical differences between the 3-year (P = 0.03) and 5-year survival rates (P = 0.004), which favored SML. This meta-analysis suggests that in pathological stage I NSCLC, the MLS can get the similar outcome to the SML in terms of 1-year survival rate. However, the SML is superior to MLS in terms of 3- and 5-year survival rates.

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.

Emerging biological insights and novel treatment strategies in primary mediastinal large B-cell lymphoma.

PubMed

Dunleavy, Kieron; Steidl, Christian

2015-04-01

While primary mediastinal large B-cell lymphoma (PMBCL) is considered to be a subtype of diffuse large B-cell lymphoma, it is a distinct clinicopathologic entity, with clinical and biological features closely resembling nodular sclerosing Hodgkin lymphoma. Recent studies have highlighted the shared biology of these two entities and identified novel critical pathways of lymphomagenesis, including the presence of distinct mutations. Mediastinal grey zone lymphomas with features in between PMBCL and nodular sclerosing Hodgkin lymphoma have been described as the missing link between the two parent entities. While the standard therapeutic approach to PMBCL has been immunochemotherapy followed by mediastinal radiation, strategies that obviate the need for radiation and thus eliminate its long-term toxicities have recently been developed. The identification of novel targets in PMBCL and mediastinal grey zone lymphomas have paved the way for testing of agents such as small molecule inhibitors of Janus kinase pathways and immune checkpoint inhibitors. Future directions in these diseases should focus on combining effective novel agents with immunochemotherapy platforms. Published by Elsevier Inc.

[Streptococcal mediastinitis after thyroidectomy. A literature review].

PubMed

Bures, C; Zielinski, V; Klatte, T; Swietek, N; Kober, F; Tatzgern, E; Bobak-Wieser, R; Gschwandtner, E; Gilhofer, M; Wechsler-Fördös, A; Hermann, M

2015-12-01

Surgical site infections after thyroid surgery are mostly superficial and can be well treated. Streptococcal mediastinitis in contrast is a rare but life-threatening complication. A 57-year-old female patient experienced septic fever, increase of inflammation parameters and erythema 2 days after thyroid surgery for Graves' disease. This process was triggered by a three-compartment infection by group A Streptococcus (GAS) with involvement of the mediastinum. Therapy over 6 weeks including seven wound revisions with the patient under general anesthesia, pathogen-adapted antibiotic treatment and cervical negative pressure treatment managed to control the infection. A total of 21 cases have been published on this phenomenon, 11 of which had a fatal outcome. High fever and surgical site erythema in the early postoperative period after thyroid surgery can be signs of a GAS infection, which might lead to necrotizing, descending, life-threatening mediastinitis. Early diagnosis with support of computed tomography (CT) scans, immediate therapy including wound opening, lavage, intravenous antibiotic treatment with penicillin and clindamycin are vital. If treatment resistance occurs, cervical negative pressure treatment should be considered.

[Treatment of lymphangiomas with picibanil in the first year of life].

PubMed

Subotic, U; Hosie, S; Waag, K L; Reinshagen, K

2008-01-01

The therapeutic gold standard of cystic hygroma is its complete resection. Because of its growth pattern and its main location in the head and neck region complete resection is not always possible. An alternative is the local injection of Picibanil, but only few cases have been published about its use in infants. We retrospectively analyzed the data of 8 infants (age: 2 weeks-12 months) who got Picibanil therapy because of cystic hygroma in the time period 2002 until 2006. Follow up ranged from 3 months up to 3 years. During the postoperative period all patients had local swelling, in 4 cases accompanied with local inflammation and fever. Tumor reduction of >50% was obtained in 7 of 8 patients. Local injection of Picibanil in infants with cystic hygroma seems to be a safe alternative to surgical therapy, especially when complete tumor resection means damage of important neighbouring structures. Prospective trials are necessary to confirm the better outcome after therapy with Picibanil compare to primary surgery.

Preoperative Localization of Mediastinal Parathyroid Adenoma with Intra-arterial Methylene Blue.

PubMed

Salman, Rida; Sebaaly, Mikhael G; Wehbe, Mohammad Rachad; Sfeir, Pierre; Khalife, Mohamad; Al-Kutoubi, Aghiad

2017-06-01

Ectopic parathyroid is found in 16% of patients with hyperparathyroidism. 2% of ectopic parathyroid adenomas are not accessible to standard cervical excision. In such cases, video-assisted thoracoscopic resection is the recommended definitive treatment. We present a case of mediastinal parathyroid adenoma localized preoperatively by injecting methylene blue within a branch of the internal mammary artery that is supplying the adenoma. Intra-arterial methylene blue injection facilitated visualization and resection of the adenoma. The preoperative intra-arterial infusion of methylene blue appears to be an effective and safe method for localization of ectopic mediastinal parathyroid adenomas and allows rapid identification during thoracoscopic resection.

Preoperative Localization of Mediastinal Parathyroid Adenoma with Intra-arterial Methylene Blue

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

Salman, Rida; Sebaaly, Mikhael G.; Wehbe, Mohammad Rachad

Ectopic parathyroid is found in 16% of patients with hyperparathyroidism. 2% of ectopic parathyroid adenomas are not accessible to standard cervical excision. In such cases, video-assisted thoracoscopic resection is the recommended definitive treatment. We present a case of mediastinal parathyroid adenoma localized preoperatively by injecting methylene blue within a branch of the internal mammary artery that is supplying the adenoma. Intra-arterial methylene blue injection facilitated visualization and resection of the adenoma. The preoperative intra-arterial infusion of methylene blue appears to be an effective and safe method for localization of ectopic mediastinal parathyroid adenomas and allows rapid identification during thoracoscopic resection.

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.

An unusual cause of an anterior mediastinal mass

PubMed Central

Hassan, I.; Goolam-Mahomed, A.

2014-01-01

Mesothelioma is a rare tumour and its radiological growth pattern varies. We report the case of a biopsy proven Malignant Pleural Mesothelioma (MPM) presenting as an anterior mediastinal mass in a platinum miner. The prognosis for this aggressive tumour remains poor, despite combination treatment modalities. PMID:26029542

Periorbital and Mediastinal Emphysema after Upper Gastrointestinal Endoscopy: Case Report of a Rare Complication.

PubMed

Lekha, Thankappan; Venkatakrishnan, Leelakrishnan; Divya, Karuppannasamy; Lavanya, Perumal

2017-01-01

To report a rare case of periorbital emphysema concurrent with cervicofacial and mediastinal emphysema in an elderly woman who underwent upper gastrointestinal endoscopy for chronic liver disease. An elderly woman with decompensated chronic liver disease presented with features of periorbital, facial, and mediastinal emphysema, characterized with crepitant swelling over the right periorbital area, face, neck, and mediastinum after undergoing upper gastrointestinal endoscopy. There was no history of trauma or Valsalva maneuver. Ocular findings were stable with no evidence of orbital compartmental syndrome. Urgent computed tomography scans of the orbit and chest were performed, and emergency systemic treatment with nasogastric decompression and antibiotics was initiated. However, she suddenly collapsed and succumbed despite all resuscitative efforts. Our case demonstrates that periorbital emphysema can occur following procedures such as upper gastrointestinal endoscopy, in the eyes without history of local trauma. This complication should be suspected especially if there is associated cervicofacial and mediastinal emphysema. Subcutaneous emphysema is usually self-resolving; however, extension of air into deeper planes can cause dangerous complications such as blindness due to orbital emphysema or mortality due to mediastinal emphysema. Hence, prompt diagnosis and urgent intervention are crucial to avoid vision and life threatening complications.

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.

A case of descending mediastinitis caused by Streptococcus pyogenes harboring genotype emm25 and sequence type 660.

PubMed

Ohya, Hiroaki; Mori, Nobuaki; Hayashi, Tetsuro; Minami, Shujiro; Higuchi, Akiko; Takahashi, Takashi

2017-06-01

Descending mediastinitis caused by Streptococcus pyogenes (group A streptococcus, GAS) is rare among cases of invasive GAS infection. In this report, we describe a case of a cervical abscess and secondary descending mediastinitis in a previously healthy 39-year-old Japanese man. The patient presented with a 2-week history of a sore throat, and subsequently developed an abscess and descending mediastinitis. We treated the cervical abscess using ampicillin/sulbactam and drainage, and GAS was subsequently isolated in two blood cultures from the patient's admission. Microbiological analyses revealed that the isolate harbored genotype emm25 and sequence type (ST) 660. This strain was susceptible to erythromycin (minimum inhibitory concentration [MIC]: ≤0.12 μg/mL), resistant to minocycline (MIC: >4 μg/mL), and possessed the tet(M) determinant. Although we have reviewed the literature regarding the clinical and microbiological characteristics of descending mediastinitis cause by GAS, little is known regarding epidemiological and clinical characteristics of emm25/ST660 GAS. Furthermore, to best of our knowledge, this is the first reported case of descending mediastinitis caused by emm25/ST660 GAS. Therefore, physicians should be aware of case with a cervical abscess and secondary descending mediastinitis caused by GAS infection, even if the patient is immunocompetent. Copyright © 2017 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

Mediastinal emphysema in mountain climbers: report of two cases and review.

PubMed

Vosk, A; Houston, C S

1977-01-01

Two cases of mediastinal emphysema occurring in healthy individuals climbing to the summit of Mount Rainier (4,393 m.) are described. In both, the condition was probably attributable to a voluntary, forced pressure breathing technique--a modified Valsalva maneuver. The various circumstances in which mediastinal emphysema may occur are discussed, along with its possible serious complication of cardiorespiratory compromise. The means of diagnosis are presented, emphasizing that cases in which the important finding of subcutaneous emphysema is absent may be those in which the danger is greatest. Therapeutic measures that occasionally may be necessary are outlined, though the condition is usually a benign one that subsides without specific treatment.

Surgical treatment for mediastinal abscess induced by endobronchial ultrasound-guided transbronchial needle aspiration: a case report and literature review.

PubMed

Yokoyama, Yujiro; Nakagomi, Takahiro; Shikata, Daichi; Higuchi, Rumi; Oyama, Toshio; Goto, Taichiro

2017-07-14

Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a useful and less invasive procedure for the definitive diagnosis of mediastinal and hilar lymph nodes. However, infectious complications can occur after EBUS-TBNA, although they are extremely rare. A 66-year-old man with necrotic and swollen lower paratracheal lymph nodes underwent EBUS-TBNA. A mediastinal abscess developed 9 days post-procedure. Surgical drainage and debridement of the abscess were performed along with lymph node biopsy followed by daily washing of the thoracic cavity. Surgical treatment was effective, leading to remission of the abscess. Biopsy revealed that the tumor was squamous cell carcinoma with no radiologically detected cancer elsewhere in the body. Mediastinal lung cancer was thus confirmed. Subsequent chemoradiotherapy led to the remission of the tumor. Mediastinitis after EBUS-TBNA is rare but should be considered, particularly if the target lymph nodes are necrotic. Mediastinitis can lead to serious and rapid deterioration of the patient's condition, for which surgical intervention is the treatment of choice.

Endobronchial Ultrasound in Suspected Non-Malignant Mediastinal Lymphadenopathy.

PubMed

Eickhoff, L; Golpon, H; Zardo, P; Suhling, H; Welte, T; Jonigk, D; Gottlieb, J; Fuehner, T

2018-05-22

 Endobronchial ultrasound (EBUS) bronchoscopy with transbronchial needle aspiration (TBNA) is a well-established tool in mediastinal staging in lung cancer and gains importance in exploration of non-malignant lymphadenopathy. The aim of this study was to evaluate the role of EBUS-TBNA in suspected non-malignant diseases.  A retrospective, single-center, observation analysis of endobronchial ultrasound bronchoscopy procedures was performed in a university medical center between March 2013 and July 2015. All patients with suspected non-malignant mediastinal lymphadenopathy were included. Cytopathological and microbiological results of EBUS were compared to clinical diagnosis 6 months after procedure and performance of EBUS was contrasted to malignant indications.  During study period, 333 EBUS bronchoscopies in 315 patients with mediastinal lymphadenopathy were performed. 111 out of 315 (35 %) patients had neither primary signs nor history of a malignant disease, categorised as patients with suspected non-malignant disease. 245 lymph nodes were sampled (median size 15 mm [IQR10 - 19]). Preferred station for TBNA was lymph node station 7 (38 %). Cytopathological findings revealed non-specific inflammation (n = 81; 70 %), carcinoma (n = 7; 6 %), epithelioid cell granulomas (n = 20; 17 %). 7 samples (6 %) were non-representative. Microbiologic testing of lymph nodes identified 3 infections (Mycobacteria tuberculosis [n = 2] and Nocardia nova [n = 1]) relevant to antibiotic therapy. Minor adverse events were observed in 9 out of 115 (8 %) patients. Sensitivity of EBUS-TBNA intervention in suspected non-malignant disease was 76 % and specificity 96 %.  EBUS-TBNA revealed a specific cause for suspected non-malignant lymphadenopathy in one-third of cases and was associated with excellent specificity. Predominant specific causes were granuloma, besides from tumor. In 3 patients pathogen could be isolated by TBNA. © Georg

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

Pancreatic mediastinal pseudocyst: report of two cases simulating intrathoracic disease.

PubMed

Ahmad, N; Auld, C D; Lawrence, J R; Watson, G D

1991-10-01

Mediastinal pseudocysts of the pancreas are extremely rare. Intra-thoracic symptoms such as dysphagia or dyspnoea due to compression or associated pleural effusions are common and urgent decompression by percutaneous or internal drainage is often necessary.

Incremental Value of Magnetic Resonance Imaging in Further Characterizing Hypodense Mediastinal and Paracardiac Lesions Identified on Computed Tomography

PubMed Central

Chaturvedi, Abhishek; Gange, Chris; Sahin, Hakan; Chaturvedi, Apeksha

2018-01-01

Mediastinal and paracardiac lesions are usually first diagnosed on a chest radiograph or echocardiogram. Often, a computed tomography is obtained to further delineate these lesions. CT may be suboptimal for evaluation of enhancement characteristics and direct extension into the adjacent mediastinal structures. With its intrinsic superior soft-tissue characterization, magnetic resonance imaging (MRI) can better delineate these lesions, their internal tissue characteristics, and identify adhesion/invasion into adjacent structures. This pictorial essay provides a brief synopsis of the key MRI sequences and their utility in further characterizing mediastinal and paracardiac lesions. PMID:29619281

Preclinical study of transcervical upper mediastinal dissection for esophageal malignancy by robot-assisted surgery.

PubMed

Mori, Kazuhiko; Yoshimura, Shuntaro; Yamagata, Yukinori; Aikou, Susumu; Seto, Yasuyuki

2017-06-01

Robotic surgical systems are potentially applicable to transcervical mediastinal lymph dissection for esophageal malignancy. Robot-assisted surgery was performed on a male fresh-frozen human cadaver. Devices for single-port laparoscopic surgery were deployed via one small incision in the left clavicular area. The task for the robot-assisted surgery was the upper mediastinal dissection to the level of the left main bronchus and en bloc harvest of the lymph nodes adherent to the left recurrent laryngeal nerve. An up-angled 30° scope in the 6 o'clock port and two robotic arms from the 3 and 9 o'clock ports worked effectively together. No collisions of the devices inside the cadaveric body or unexpected traumatic events occurred. The robotic surgical system can be used safely for the upper mediastinal dissection. Copyright © 2016 John Wiley & Sons, Ltd. Copyright © 2016 John Wiley & Sons, Ltd.

Post-tuberculous fibrosing mediastinitis: a review of the literature

PubMed Central

Wu, Zhe; Jarvis, Hannah; Howard, Luke S; Wright, Corrina; Kon, Onn Min

2017-01-01

Fibrosing mediastinitis (FM) is a rare disease where there is thickening of the fibrous tissue in the mediastinum. While histoplasmosis is the the most common recognised cause, the link with tuberculosis (TB) has been rarely documented. We review the link between TB and FM, and describe a case of probable TB-related FM.Our case is of a 74-year-old man who presented with breathlessness 3 years after fully treated TB. Scans revealed a calcified soft tissue mass within the mediastinum, and a diagnosis of fibrosing mediastinitis resulting in pulmonary hypertension was made. Tests for histoplasmosis and IgG4 staining were negative. Surgical intervention was not felt to be beneficial, and he was treated with prednisolone and mycophenolate mofetil.In the review, we highlight the two forms of mediastinitis—granulomatous versus fibrous, and how these two entities may be on a spectrum of disease progression. We also explore the prevalence, clinical presentation, pathogenesis, imaging techniques and treatment options of TB-related FM. PMID:28725444

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

Diagnostic performance of convex probe EBUS-TBNA in patients with mediastinal and coexistent endobronchial or peripheral lesions

PubMed Central

Verma, Akash; Goh, Kee San; Phua, Chee Kiang; Sim, Wen Yuan; Tee, Kuan Sen; Lim, Albert Y.H.; Tai, Dessmon Y.H.; Goh, Soon Keng; Kor, Ai Ching; Ho, Benjamin; Lew, Sennen J.W.; Abisheganaden, John

2016-01-01

Abstract To compare the performance of convex probe endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA) with conventional endobronchial biopsy (EBB) or transbronchial lung biopsy (TBLB) in patients with mediastinal, and coexisting endobronchial or peripheral lesions. Retrospective review of records of patients undergoing diagnostic EBUS-TBNA and conventional bronchoscopy in 2014. A total of 74 patients had mediastinal, and coexisting endobronchial or peripheral lesions. The detection rate of EBUS-TBNA for mediastinal lesion >1 cm in short axis, EBB for visible exophytic type of endobronchial lesion, and TBLB for peripheral lesion with bronchus sign were 71%, 75%, and 86%, respectively. In contrast, the detection rate of EBUS-TBNA for mediastinal lesion ≤1 cm in short axis, EBB for mucosal hyperemia type of endobronchial lesion, and TBLB for peripheral lesion without bronchus sign were 25%, 63%, and 38%, and improved to 63%, 88%, and 62% respectively by adding EBB or TBLB to EBUS-TBNA, and EBUS-TBNA to EBB or TBLB. Postprocedure bleeding was significantly more common in patients undergoing EBB and TBLB 8 (40%) versus convex probe EBUS-TBNA 2 patients (2.7%, P = 0.0004). EBUS-TBNA is a safer single diagnostic technique compared with EBB or TBLB in patients with mediastinal lesion of >1 cm in size, and coexisting exophytic type of endobronchial lesion, or peripheral lesion with bronchus sign. However, it requires combining with EBB or TBLB and vice versa to optimize yield when mediastinal lesion is ≤1 cm in size, and coexisting endobronchial and peripheral lesions lack exophytic nature, and bronchus sign, respectively. PMID:27977603

The Impact of the Medicaid Health Care-Associated Condition Program on Mediastinitis Following Coronary Artery Bypass Graft

PubMed Central

Hsu, Heather; Kawai, Alison Tse; Wang, Rui; Jentzsch, Maximilian S.; Rhee, Chanu; Horan, Kelly; Jin, Robert; Goldmann, Donald; Lee, Grace M.

2018-01-01

Objective In 2012, the Centers for Medicare and Medicaid Services expanded a 2008 program that eliminated additional Medicare payment for mediastinitis following coronary artery bypass graft (CABG) to include Medicaid. We aimed to evaluate the impact of this Medicaid program on mediastinitis rates reported by the National Healthcare Safety Network (NHSN) compared with rates of a condition not targeted by the program (deep space surgical site infection [SSI] after knee replacement). Design interrupted time series with comparison group. Methods We included surveillance data from non-federal acute care hospitals participating in NHSN and reporting CABG or knee replacement outcomes from 1/2009–6/2017. We examined the Medicaid program’s impact on NHSN-reported infection rates, adjusting for secular trends. Data analysis used generalized estimating equations with robust sandwich variance estimators. Results During the study period, 196 study hospitals reported 273,984 CABGs to NHSN, resulting in 970 mediastinitis cases (0.35%); 294 hospitals reported 555,395 knee replacements, with 1,751 resultant deep space SSIs (0.32%). There were no significant changes in incidence of either condition during the study. Mediastinitis models showed no effect of the 2012 Medicaid program on either secular trend during the post- vs. pre-program time periods (p-value=0.70) or immediate program effect (p-value=0.83). Results were similar in sensitivity analyses when adjusting for hospital characteristics, restricting to hospitals with consistent NHSN reporting, or incorporating a program implementation roll-in period. Knee replacement models also showed no program effect. Conclusions The 2012 Medicaid program to eliminate additional payments for mediastinitis following CABG had no impact on reported mediastinitis rates. PMID:29669607

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

Cystic Fibrosis and Pregnancy

MedlinePlus

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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.

Acute Leukemia and Concurrent Mediastinal Germ Cell Tumor: Case Report and Literature Review.

PubMed

Maese, Luke; Li, K David; Xu, Xinjie; Afify, Zeinab; Paxton, Christian N; Putnam, Angelica

2017-04-01

There is a known association of primary nonseminomatous mediastinal germ cell tumors (NSMGCT) and hematologic malignancy in younger males not linked to treatment. When combined these two rare entities convey a very poor prognosis. Here we report a 16-year-old male with an anterior mediastinal mass diagnosed as a malignant germ cell tumor based on elevation of serologic markers. He was found to have acute leukemia with megakaryocytic differentiation several days later. We focus our report on the pathologic findings, including a review of the literature, and a novel molecular analysis of the germ cell tumor.

Picibanil (OK-432) in the treatment of head and neck lymphangiomas in children

PubMed Central

Rebuffini, Elena; Zuccarino, Luca; Grecchi, Emma; Carinci, Francesco; Merulla, Vittorio Emanuele

2012-01-01

Background: Picibanil (OK-432) is a lyophilized mixture of group A Streptococcus pyogenes with antineoplastic activity. Because of its capacity to produce a selective fibrosis of lymphangiomas (LMs), it has been approved by Japanese administration in 1995 for the treatment of LMs. Materials and Methods: We treated 15 children (age range: 6-60 months) affected by head and neck macrocystic LMs with intracystic injections (single dose of 0.2 mL) of Picibanil (1-3 injections). Results: Complete disappearance of the lesion was noticed in eight (53.33%) cases, a marked (>50%) reduction of LMs was found five (33.33%) cases, while a moderate (<50%) response was recorded in two (13.33%) cases. Picibanil side effects included fever, local inflammation, and transitory increase of blood platelets’ concentration; a single case of anemia was resolved with concentrated red blood cells transfusion. Conclusions: Intracystic injection of Picibanil is an effective and safe treatment for macrocystic LMs in pediatric patients and may represent the treatment of choice in such cases, especially where surgical excision is associated with the risk of functional/cosmetic side effects. PMID:23814582

Picibanil (OK-432) in the treatment of head and neck lymphangiomas in children.

PubMed

Rebuffini, Elena; Zuccarino, Luca; Grecchi, Emma; Carinci, Francesco; Merulla, Vittorio Emanuele

2012-12-01

Picibanil (OK-432) is a lyophilized mixture of group A Streptococcus pyogenes with antineoplastic activity. Because of its capacity to produce a selective fibrosis of lymphangiomas (LMs), it has been approved by Japanese administration in 1995 for the treatment of LMs. We treated 15 children (age range: 6-60 months) affected by head and neck macrocystic LMs with intracystic injections (single dose of 0.2 mL) of Picibanil (1-3 injections). Complete disappearance of the lesion was noticed in eight (53.33%) cases, a marked (>50%) reduction of LMs was found five (33.33%) cases, while a moderate (<50%) response was recorded in two (13.33%) cases. Picibanil side effects included fever, local inflammation, and transitory increase of blood platelets' concentration; a single case of anemia was resolved with concentrated red blood cells transfusion. Intracystic injection of Picibanil is an effective and safe treatment for macrocystic LMs in pediatric patients and may represent the treatment of choice in such cases, especially where surgical excision is associated with the risk of functional/cosmetic side effects.

[Measurement of intrafraction displacement of the mediastinal metastatic lymph nodes of non-small cell lung cancer based on four-dimensional computed tomography (4D-CT)].

PubMed

Wang, Su-zhen; Li, Jian-bin; Zhang, Ying-jie; Li, Feng-xiang; Wang, Wei; Liu, Tong-hai

2012-09-01

To measure the intrafraction displacement of the mediastinal metastatic lymph nodes of non-small cell lung cancer (NSCLC) based on four-dimensional computed tomography (4D-CT), and to provide the basis for the internal margin of metastatic mediastinal lymph nodes. Twenty-four NSCLC patients with mediastinal metastatic lymph nodes confirmed by contrast enhanced CT (short axis diameter ≥ 1 cm) were included in this study. 4D-CT simulation was carried out during free breathing and 10 image sets were acquired. The mediastinal metastatic lymph nodes and the dome of ipsilateral diaphragma were separately delineated on the CT images of 10 phases of breath cycle, and the lymph nodes were grouped as the upper, middle and lower mediastinal groups depending on the mediastinal station. Then the displacements of the lymph nodes in the left-right, anterior-posterior, superior-inferior directions and the 3-dimensional vector were measured. The differences of displacement in three directions for the same group of metastatic lymph nodes and in the same direction for different groups of metastatic lymph nodes were compared. The correlation between the displacement of ipsilateral diaphragma and mediastinal lymph nodes was analyzed in superior-inferior direction. The displacements in left-right, anterior-posterior and superior-inferior directions were (2.24 ± 1.55) mm, (1.87 ± 0.92) mm and (3.28 ± 2.59) mm for the total (53) mediastinal lymph nodes, respectively. The vectors were (4.70 ± 2.66) mm, (3.87 ± 2.45) mm, (4.97 ± 2.75) mm and (5.23 ± 2.67) mm for the total, upper, middle and lower mediastinal lymph nodes, respectively. For the upper mediastinal lymph nodes, the displacements in left-right, anterior-posterior and superior-inferior directions showed no significant difference between each other (P > 0.05). For the middle mediastinal lymph nodes, the displacements merely in anterior-posterior and superior-inferior directions showed significant difference (P = 0

CT of chronic infiltrative lung disease: Prevalence of mediastinal lymphadenopathy

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

Niimi, Hiroshi; Kang, Eun-Young; Kwong, S.

1996-03-01

Our goal was to determine the prevalence of mediastinal lymph node enlargement at CT in patients with diffuse infiltrative lung disease. The study was retrospective and included 175 consecutive patients with diffuse infiltrative lung diseases. Diagnoses included idiopathic pulmonary fibrosis (IPF) (n = 61), usual interstitial pneumonia associated with collagen vascular disease (CVD) (n = 20), idiopathic bronchiolitis obliterans organizing pneumonia (BOOP) (n = 22), extrinsic allergic alveolitis (EAA) (n = 17), and sarcoidosis (n = 55). Fifty-eight age-matched patients with CT of the chest performed for unrelated conditions served as controls. The presence, number, and sites of enlarged nodesmore » (short axis {ge}10 mm in diameter) were recorded. Enlarged mediastinal nodes were present in 118 of 175 patients (67%) with infiltrative lung disease and 3 of 58 controls (5%) (p < 0.001). The prevalence of enlarged nodes was 84% (46 of 55) in sarcoidosis, 67% (41 of 61) in IPF, 70% (14 of 20) in CVD, 53% (9 of 17) in EAA, and 36% (8 of 22) in BOOP. The mean number of enlarged nodes was higher in sarcoidosis (mean 3.2) than in the other infiltrative diseases (mean 1.2) (p < 0.001). Enlarged nodes were most commonly present in station 10R, followed by 7, 4R, and 5. Patients with infiltrative lung disease frequently have enlarged mediastinal lymph nodes. However, in diseases other than sarcoid, usually only one or two nodes are enlarged and their maximal short axis diameter is <15 mm. 11 refs., 2 figs., 1 tab.« less

Fluoroscopically guided nose tube drainage of mediastinal abscesses in post-operative gastro-oesophageal anastomotic leakage.

PubMed

Xu, Q Y; Yin, G W; Chen, S X; Jiang, F; Bai, X J; Wu, J D

2012-11-01

The aim of this study was to retrospectively evaluate the technical success rates and clinical effectiveness of fluoroscopically guided nose tube drainage of mediastinal abscesses and a nasojejunum feeding tube in post-operative gastro-oesophageal anastomotic leakage (GEAL). From January 2006 to June 2011, 18 cases of post-operative GEAL with mediastinal abscesses after oesophagectomy with intrathoracic oesophagogastric anastomotic procedures for oesophageal and cardiac carcinoma were treated by insertion of a nose drainage tube and nasojejunum feeding tube under fluoroscopic guidance. We evaluated the feasibility of two-tube insertion to facilitate leakage site closure and complete resolution of the abscess, and the patients' nutritional benefit was also evaluated by checking the serum albumin level between pre- and post-enteral feeding via the feeding tube. The two tubes were placed successfully under fluoroscopic guidance in 18 patients (100%). The procedure time for two-tube insertion ranged from 20 to 40 min (mean 30 min). 17 patients (94%) achieved leakage site closure after two-tube insertion and had a good tolerance of two tubes in the nasal cavity. The serum albumin level was significant, increased from pre-enteral feeding (2.49 ± 0.42 g dl(-1)) to the post-enteral feeding (3.58 ± 0.47 g dl(-1)) via the feeding tube (p<0.001). The duration of follow-up ranged from 1 to 49 months (mean 19 months). The insertion of nose tube drainage and a nasojejunum feeding tube under fluoroscopic guidance is safe, and it provides effective relief from mediastinal abscesses in GEAL after oesophagectomy. Moreover, our findings indicate that two-tube insertion may be used as a selective procedure to treat mediastinal abscesses in post-operative GEAL. Advances in knowledge Directive drainage of mediastinal abscesses in post-operative GEAL may be an effective treatment.

Successful Treatment of Congenital Lymphangioma Circumscriptum of the Vulva with CO2 and Long-Pulsed Nd:YAG Lasers.

PubMed

Sasaki, Ryosuke; Negishi, Kei; Akita, Hirotaka; Suzuki, Kayoko; Matsunaga, Kayoko

2014-01-01

A 16-year-old girl presented with a 9-year history of vesicles on the vulva. She had initially taken a wait-and-see approach, but required treatment because of bleeding. Histological examination of a biopsied vesicle revealed dilated lymph channels in the upper dermis, suggesting lymphangioma circumscriptum (LC). The challenge for this pathology has been to find a conservative treatment with low morbidity and better results than those reported for surgical excision, which has been the mainstay of therapy. In this case, LC of the vulva was successfully treated using a 10,600-nm CO2 laser and long-pulsed Nd:YAG laser. Use of the 10,600-nm CO2 laser and long-pulsed Nd:YAG laser appeared effective for treating LC.

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.

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.

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.

Complete atrioventricular block following mediastinal irradiation: A report of six cases

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

Slama, M.S.; Le Guludec, D.; Sebag, C.

1991-07-01

Complete atrioventricular block (AVB) following radiotherapy has been reported rarely, usually after high dose mediastinal irradiation for Hodgkin's disease or lung or breast carcinoma. The authors report six new cases of episodic complete infranodal AVB, requiring permanent pacemaker implantation. The mean age was 48-years old (ranging from 25-60) at the first Adams Stokes attack, mean delay was 12 years after irradiation (10-18), and mean radiation dose was 5,200 rads (4,000-6,500). All patients had abnormal interval electrocardiograms (right bundle branch block in two, left bundle branch block in three, alternating left and right bundle branch block in one). Electrocardiograms during themore » episode of AVB or Holter recordings were consistent with infranodal block in all patients; electrophysiological study performed in five patients confirmed infranodal AVB in four, and one was normal. Pericardial disease was constant, which included pericardial constriction in four patients. Two patients died after failure of pericardiectomy to improve congestive heart failure, due to epicardial, myocardial, and endocardial involvement. Noncardiac mediastinal lesions were present in four cases. Since this delayed complication may occur in patients of such age that the relation between the AVB and the chest irradiation is questionable, they propose the following etiologic criteria; high radiation dose (over 4,000 rads); delay of 10 years or more; abnormal interval tracings; pericardial involvement; and associated cardiac or mediastinal radiation-induced lesions.« less

CT fluoroscopy-guided core needle biopsy of anterior mediastinal masses.

PubMed

Iguchi, T; Hiraki, T; Matsui, Y; Fujiwara, H; Sakurai, J; Masaoka, Y; Uka, M; Tanaka, T; Gobara, H; Kanazawa, S

2018-02-01

To retrospectively evaluate the safety, diagnostic yield, and risk factors of diagnostic failure of computed tomography (CT) fluoroscopy-guided biopsies of anterior mediastinal masses. Biopsy procedures and results of anterior mediastinal masses in 71 patients (32 women/39 men; mean [±standard deviation] age, 53.8±20.0years; range, 14-88years) were analyzed. Final diagnoses were based on surgical outcomes, imaging findings, or clinical follow-up findings. The biopsy results were compared with the final diagnosis, and the biopsy procedures grouped by pathologic findings into diagnostic success and failure groups. Multiple putative risk factors for diagnostic failure were then assessed. Seventy-one biopsies (71 masses; mean size, 67.5±27.3mm; range 8.6-128.2mm) were analyzed. We identified 17 grade 1 and one grade 2 adverse events (25.4% overall) according to the National Cancer Institute Common Terminology Criteria for Adverse Events version 4.0. Sixty-nine biopsies (97.2%) provided samples fit for pathologic analysis. Diagnostic failure was found for eight (11.3%) masses; the 63 masses diagnosed successfully included thymic carcinoma (n=17), lung cancer (n=14), thymoma (n=12), malignant lymphoma (n=11), germ cell tumor (n=3), and others (n=6). Using a thinner needle (i.e., a 20-gauge needle) was the sole significant risk factor for diagnostic failure (P=0.039). CT fluoroscopy-guided biopsy of anterior mediastinal masses was safe and had a high diagnostic yield; however, using a thinner biopsy needle significantly increased the risk of a failed diagnosis. Copyright © 2017 Éditions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

Surgical debridement, vacuum therapy and pectoralis plasty in poststernotomy mediastinitis.

PubMed

Ennker, I C; Pietrowski, D; Vöhringer, L; Kojcici, B; Albert, A; Vogt, P M; Ennker, J

2009-11-01

In cardiac surgery poststernotomy mediastinitis continues to be a serious cause of morbidity and mortality. We report our experience with vacuum-assisted closure (VAC) therapy followed by reconstruction with M. pectoralis muscle flaps as treatment for deep sternal wound infections. Our group performed a retrospective analysis of 3630 consecutive cardiac surgical patients using median sternotomy from 11/2004 to 11/2007. After removing sternal wires, necrotic debris and potentially infective material, restabilisation of the sternum was performed and VAC therapy was employed. Wound closure and subsequent reconstruction were performed using a bilateral pectoralis muscle plasty. Of the analysed patients 16 female and 29 male patients suffered from deep sternal wound infections and were treated with VAC. The most common risk factors were diabetes mellitus odds ratio (OR 3.5), chronic obstructive pulmonary disease (COPD) (OR 2.9), use of bilateral mammarian artery (OR 2.0) and obesity (1.8). The median age of patients with deep sternal infections was similar to control patients. Staphylococcus epidermis was the most common pathogen (37.8%) followed by Enterococcus faecilis (22.2%) and Staphylococcus aureus (17.8). In 22.2% no pathogen could be detected. The 30 day mortality was 0%, the in-hospital mortality was 15.6%. The results of our studies demonstrate that vacuum therapy in conjunction with early and aggressive debridement is an effective strategy for treating poststernotomy mediastinitis. We consider pectoralis major muscle flap reconstruction as a safe technique and regard it as the primary choice for wound closure in poststernotomy mediastinitis. (c) 2009 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Bilateral Video-Assisted Thoracoscopic Surgery Resection for Multiple Mediastinal Myelolipoma: Report of a Case

PubMed Central

Kohno, Tadasu; Mun, Mingyon; Yoshiya, Tomoharu

2014-01-01

Myelolipoma in the mediastinum is an extremely rare entity. In this report, we present the case of a 79-year-old asymptomatic man who had three bilateral paravertebral mediastinal tumors. The three tumors were resected simultaneously using bilateral three-port video-assisted thoracoscopic surgery (VATS). There has been no evidence of recurrence within four years after the operation. Multiple bilateral mediastinal myelolipomas are extremely rare. There are no reports in the English literature of multiple bilateral thoracic myelolipomas that were resected simultaneously using bilateral VATS. We also present characteristic features of myelolipomas, which are helpful for diagnosis. PMID:24782978

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

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

Percutaneous Image-Guided Cryoablation of Challenging Mediastinal Lesions Using Large-Volume Hydrodissection: Technical Considerations and Outcomes

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

Garnon, Julien, E-mail: juliengarnon@gmail.com; Koch, Guillaume, E-mail: Guillaume.koch@gmail.com; Caudrelier, Jean, E-mail: caudjean@yahoo.fr

ObjectiveThis study was designed to describe the technique of percutaneous image-guided cryoablation with large-volume hydrodissection for the treatment of challenging mediastinal lesions.MethodsBetween March 2014 and June 2015, three patients (mean age 62.7 years) with four neoplastic anterior mediastinal lesions underwent five cryoablation procedures using large-volume hydrodissection. Procedures were performed under general anaesthesia using CT guidance. Lesion characteristics, hydrodissection and cryoablation data, technical success, complications, and clinical outcomes were assessed using retrospective chart review.ResultsLesions (mean size 2.7 cm; range 2–4.3 cm) were in contact with great vessels (n = 13), trachea (n = 3), and mediastinal nerves (n = 6). Hydrodissection was performed intercostally (n = 4), suprasternally (n = 2), transsternally (n = 1), ormore » via the sternoclavicular joint (n = 1) using 1–3 spinal needles over 13.4 (range 7–26) minutes; 450 ml of dilute contrast was injected (range 300–600 ml) and increased mean lesion-collateral structure distance from 1.9 to 7.7 mm. Vulnerable mediastinal nerves were identified in four of five procedures. Technical success was 100 %, with one immediate complication (recurrent laryngeal nerve injury). Mean follow-up period was 15 months. One lesion demonstrated residual disease on restaging PET-CT and was retreated to achieve complete ablation. At last follow-up, two patients remained disease-free, and one patient developed distant disease after 1 year without local recurrence.ConclusionsCryoablation using large-volume hydrodissection is a feasible technique, enabling safe and effective treatment of challenging mediastinal lesions.« less

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.

Utility and safety of endobronchial ultrasound-guided transbronchial needle aspiration in patients with mediastinal and hilar lymphadenopathy: Western region experience.

PubMed

Aljohaney, Ahmed A

2018-01-01

The aim of the study was to evaluate the clinical utility and safety of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) in patients with mediastinal and hilar lymphadenopathy and to explicitly describe the utility of this procedure in patient's outcome. A retrospective review and analysis was conducted on 52 patients with mediastinal or hilar lymphadenopathy who underwent EBUS-TBNA from June 2012 to June 2016. All the patients were evaluated by computed tomography (CT) chest with contrast before EBUS examination. Enlarged mediastinal or hilar lymph node was defined as >1 cm short axis on the enhanced CT. Among the 52 patients studied, 57.7% were presented with mediastinal or hilar lymphadenopathy for diagnosis and 42.3% presented with suspected mediastinal malignancy. Paratracheal stations were the most common site for puncture in 33 lymph nodes (43%). The best diagnostic yield was obtained from subcarinal stations and the lowest yield from the hilar stations. Surgical biopsies confirmed lymphoma in six patients, tuberculosis (TB) in three, sarcoidosis in two and one had metastatic adenocarcinoma of unknown primary. The sensitivity, specificity, positive predictive value, and negative predictive value of EBUS-TBNA for diagnosis of mediastinal and hilar lymph node abnormalities were 78.6%, 100%, 100%, and 80%, respectively. The diagnostic yield of EBUS-TBNA in malignant and benign conditions was 79.0%. EBUS-TBNA is a safe and efficacious procedure which can be performed using conscious sedation with high yields. It can be used for the staging of malignancies as well as for the diagnosis of inflammatory and infectious conditions such as sarcoidosis and TB.

The diagnostic accuracy of the mediastinal width on supine anteroposterior chest radiographs with nontraumatic Stanford type A acute aortic dissection.

PubMed

Funakoshi, Hiraku; Mizobe, Michiko; Homma, Yosuke; Nakashima, Yoshiyuki; Takahashi, Jin; Shiga, Takashi

2018-03-01

Nontraumatic Stanford type A acute aortic dissection is a life-threatening condition; thus, the ability to make a precise diagnosis of nontraumatic Stanford type A acute aortic dissection is essential for the emergency physician. Several reports have shown that the mediastinal widening on a chest radiograph is useful for the diagnosis of nontraumatic Stanford type A acute aortic dissection; however, the exact cutoff value of the mediastinal width on plain radiographs is rarely defined. A single-center retrospective case-control study was conducted between October 1, 2013, and March 31, 2015. We evaluated the maximal mediastinal width of the anteroposterior chest X-ray at the level of the aortic knob in the supine position between patient groups with and without nontraumatic Stanford type A acute aortic dissection. We enrolled 72 patients (36 patients with nontraumatic Stanford type A acute aortic dissection and 36 patients without nontraumatic Stanford type A acute aortic dissection). The median mediastinal width of patients with nontraumatic Stanford type A acute aortic dissection was significantly larger than that of patients without nontraumatic Stanford type A acute aortic dissection (100.7 mm vs 77.7 mm, P  < .01). The optimal cutoff level was 87 mm (sensitivity, 81%; specificity, 89%). Using multivariable logistic regression, the odds ratio of a mediastinal width of >87 mm for a diagnosis nontraumatic Stanford type A acute aortic dissection was 57.1 (95% confidence interval, 11.2-290.2). A mediastinal width of >87 mm showed high sensitivity in the diagnosis of probable nontraumatic Stanford type A acute aortic dissection.

Copanlisib and Nivolumab in Treating Participants With Recurrent or Refractory Diffuse Large B-cell Lymphoma or Primary Mediastinal Large B-cell Lymphoma

ClinicalTrials.gov

2018-06-11

Recurrent Diffuse Large B-Cell Lymphoma; Recurrent Primary Mediastinal (Thymic) Large B-Cell Cell Lymphoma; Refractory Diffuse Large B-Cell Lymphoma; Refractory Primary Mediastinal (Thymic) Large B-Cell Cell Lymphoma

Mediastinal mature teratoma with coexistence of angiosarcoma, granulocytic sarcoma and a hematopoietic region in the tumor: a rare case of association between hematological malignancy and mediastinal germ cell tumor.

PubMed

Saito, A; Watanabe, K; Kusakabe, T; Abe, M; Suzuki, T

1998-09-01

An association between mediastinal germ cell tumors (MGCT) and hematological malignancies (e.g. acute leukemia, malignant histiocytosis) has been recognized since 1984. A rare case of mediastinal mature teratoma with angiosarcoma, a hematopoietic region and granulocytic sarcoma is reported in a 29-year-old male. The resected tumor was 9.0 x 6.5 cm, weighed 65 g and showed extensive necrosis, forming a cyst. The histological features of the tumor showed a mature teratoma, which contained a large gland lined by ciliated epithelium, hyalinous cartilage, a paraganglion-like structure, well-differentiated angiosarcoma with atypical hematopoiesis composed of CD34-positive cells, and malignant round cells. The malignant round cells did not stain for CD34 but were positive for leukocyte common antigen (LCA) and c-kit product. From these findings, the round cells were diagnosed as granulocytic sarcoma. The patient died of metastasis of the granulocytic sarcoma in the tonsils and cervical lymph nodes 8 months after surgery. A leukemic condition was not present throughout the clinical course. The association between MGCT and hematological malignancy is a distinctive syndrome. However, its pathogenesis is still obscure and the origin of the hematopoietic malignancy has not been fully elucidated. In this particular case, it is suggested that the granulocytic sarcoma might have arisen from the abnormal hematopoietic area in the mediastinal teratoma.

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

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.

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

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.

Two patients with TAFRO syndrome exhibiting strikingly similar anterior mediastinal lesions with predominantly fat attenuation on chest computed tomography.

PubMed

Ozawa, Yoko; Yamamoto, Hiroshi; Yasuo, Masanori; Takahashi, Hidekazu; Tateishi, Kazunari; Ushiki, Atsuhito; Kawakami, Satoshi; Fujinaga, Yasunari; Asaka, Shiho; Sano, Kenji; Takayama, Hiroshi; Imamura, Hiroshi; Hanaoka, Masayuki

2017-03-01

We herein report on two middle-aged men with TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis or renal failure, and organomegaly) syndrome, a unique clinicopathological variant of multicentric Castleman׳s disease recently proposed in Japan. Strikingly similar anterior mediastinal fat swellings with soft tissue density were observed in the patients on chest computed tomography. In TAFRO syndrome, bilateral pleural effusion and slight lymph node swelling are common in the thoracic region; however, anterior mediastinal lesions have not been previously observed. Although the mechanisms of anterior mediastinal lesions have not been defined, these lesions seem to have a close relationship with TAFRO syndrome. Copyright © 2016 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.

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 ...

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.

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? ...

Proposal for a recovery prediction method for patients affected by acute mediastinitis

PubMed Central

2012-01-01

Background An attempt to find a prediction method of death risk in patients affected by acute mediastinitis. There is not such a tool described in available literature for that serious disease. Methods The study comprised 44 consecutive cases of acute mediastinitis. General anamnesis and biochemical data were included. Factor analysis was used to extract the risk characteristic for the patients. The most valuable results were obtained for 8 parameters which were selected for further statistical analysis (all collected during few hours after admission). Three factors reached Eigenvalue >1. Clinical explanations of these combined statistical factors are: Factor1 - proteinic status (serum total protein, albumin, and hemoglobin level), Factor2 - inflammatory status (white blood cells, CRP, procalcitonin), and Factor3 - general risk (age, number of coexisting diseases). Threshold values of prediction factors were estimated by means of statistical analysis (factor analysis, Statgraphics Centurion XVI). Results The final prediction result for the patients is constructed as simultaneous evaluation of all factor scores. High probability of death should be predicted if factor 1 value decreases with simultaneous increase of factors 2 and 3. The diagnostic power of the proposed method was revealed to be high [sensitivity =90%, specificity =64%], for Factor1 [SNC = 87%, SPC = 79%]; for Factor2 [SNC = 87%, SPC = 50%] and for Factor3 [SNC = 73%, SPC = 71%]. Conclusion The proposed prediction method seems a useful emergency signal during acute mediastinitis control in affected patients. PMID:22574625

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.

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

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.

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 ...

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.

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.

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

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.

[Emergent Caesarean Section in a Patient with a Mediastinal Tumor and Von Recklinghausen Disease: A Case Report].

PubMed

Owada, Mayuko; Inomata, Shinichi; Danmura, Masato; Yamada, Kumiko; Tanaka, Makoto

2016-06-01

It is rare to encounter a pregnant patient with a mediastinal tumor, and if the tumor size increases as the pregnancy progresses, this increase can cause complications such as airway constriction and vascular occlusion. We report a case of a pregnant patient diagnosed with von Recklinghausen disease at the age of seven and diagnosed with a mediastinal tumors just after her present admission. The impending suffocation progressed and fetal heart rate decreased during her hospitalization. Her trachea was intubated and she was moved to an operating room for an emergent cesarean section under general anesthesia. With this rapid response, we could rescue both patient and infant. If the size of mediastinal tumor increases as pregnancy progresses, the tumor will cause suffocation by airway compression from the outside, in addition to specific airway edema on the inward side. The present case demonstrates that appropriate desisoins must be made for airway manegement and initiation of surgery.

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.

Tracer uptake in mediastinal and paraaortal thoracic lymph nodes as a potential pitfall in image interpretation of PSMA ligand PET/CT.

PubMed

Afshar-Oromieh, Ali; Sattler, Lars Peter; Steiger, Katja; Holland-Letz, Tim; da Cunha, Marcelo Livorsi; Mier, Walter; Neels, Oliver; Kopka, Klaus; Weichert, Wilko; Haberkorn, Uwe

2018-07-01

Since the introduction of 68 Ga-PSMA-11 PET/CT for imaging prostate cancer (PC) we have frequently observed mediastinal lymph nodes (LN) showing tracer uptake despite being classified as benign. The aim of this evaluation was to further analyze such LN. Two patient groups with biphasic 68 Ga-PSMA-11 PET/CT at 1 h and 3 h p.i. were included in this retrospective evaluation. Group A (n = 38) included patients without LN metastases, and group B (n = 43) patients with LN metastases of PC. SUV of mediastinal/paraaortal LN of group A (n = 100) were compared to SUV of LN metastases of group B (n = 91). Additionally, 22 randomly selected mediastinal and paraaortal LN of patients without PC were immunohistochemically (IHC) analyzed for PSMA expression. In group A, 7/38 patients (18.4%) presented with at least one PSMA-positive mediastinal LN at 1 h p.i. and 3/38 (7.9%) positive LN at 3 h p.i. with a SUVmax of 2.3 ± 0.7 at 1 h p.i. (2.0 ± 0.7 at 3 h p.i.). A total of 11 PSMA-positive mediastinal/paraaortal LN were detected in nine patients considering both imaging timing points. SUVmax of LN-metastases was 12.5 ± 13.2 at 1 h p.i. (15.8 ± 17.0 at 3 h p.i.). SUVmax increased clearly (> 10%) between 1 h and 3 h p.i. in 76.9% of the LN metastases, and decreased significantly in 72.7% of the mediastinal/paraaortal LN. By IHC, PSMA-expression was observed in intranodal vascular endothelia of all investigated LN groups and to differing degrees within germinal centers of 15/22 of them (68.1%). Expression was stronger in mediastinal nodes (p = 0.038) and when follicular hyperplasia was present (p = 0.050). PSMA-positive mediastinal/paraaortal benign LN were visible in a notable proportion of patients. PSMA-positivity on the histopathological level was associated with the activation state of the LN. However, in contrast to LN metastases of PC, they presented with significantly lower uptake, which, in addition, usually decreased

Tumor histology predicts mediastinal nodal status and may be used to guide limited lymphadenectomy in patients with clinical stage I non-small cell lung cancer.

PubMed

Cheng, Xinghua; Zheng, Difan; Li, Yuan; Li, Hang; Sun, Yihua; Xiang, Jiaqing; Chen, Haiquan

2018-06-01

Methods to minimize surgical trauma from mediastinal lymphadenectomy in patients with early-stage lung cancer are still immature. This study aimed to identify predictors of negative pathologic N2, which may be used to select patients for limited mediastinal lymphadenectomy. Clinicopathologic features of 1430 patients with resected clinical stage I non-small cell lung cancer and complete mediastinal lymphadenectomy were retrospectively analyzed for variables associated with negative N2 nodal metastasis (2008-2015). Overall and recurrence-free survival in patients after complete or limited mediastinal lymphadenectomy were assessed via Kaplan-Meier survival analysis and log-rank testing. The accuracy of frozen section diagnosis for predicting final pathology was retrospectively assessed in 126 randomly selected patients after the surgery. Multivariable analysis revealed that tumor size ≤2 cm, negative pN1, lymphovascular invasion, and lepidic adenocarcinoma were associated with negative mediastinal nodal metastasis. Notably, none of the patients with histology of adenocarcinoma in situ, minimally invasive adenocarcinoma, or lepidic pattern-predominant adenocarcinoma on final pathology had pN2 disease, and the 5-year overall and recurrence free-survival of these patients (99.3% and 99.3%, respectively) was not different from those after limited mediastinal lymphadenectomy (98.7% and 100%, P = .582 and .511, respectively). If these subtypes were classified together as the low-risk group, the concordance rate between frozen section and final pathology diagnosis was 88.9% in the retrospective test cohort. Tumor histology may predict negative mediastinal metastasis in patients with early-stage lung cancer. Future prospective studies are merited to validate the feasibility of using frozen section to select patients for limited mediastinal lymphadenectomy. Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

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.

Mediastinal germ cell tumour causing superior vena cava tumour thrombosis.

PubMed

Karanth, Suman S; Vaid, Ashok K; Batra, Sandeep; Sharma, Devender

2015-03-25

We report a rare case of a 35-year-old man who presented with a 1-week history of retrosternal chest pain of moderate intensity. A positron emission tomography CT (PET-CT) showed a large fluorodeoxy-glucose (FDG)-avid heterogeneously enhancing necrotic mass in the anterosuperior mediastinum with a focal FDG-avid thrombosis of the superior vena cava (SVC) suggestive of tumour thrombus and vascular invasion. α-Fetoprotein levels were raised (5690 IU/L). Image guided biopsy of the mediastinal mass was suggestive of non-seminomatous germ cell tumour (NSGCT). The patient received four cycles of BEP (bleomycin, etoposide and cisplatin) along with therapeutic anticoagulation with low-molecular-weight heparin. Follow-up whole body PET-CT revealed complete resolution of mediastinal mass and SVC tumour thrombosis. The documentation of FDG-PET-avid tumour thrombus resolving with chemotherapy supports the concept of circulating tumour cells being important not only in common solid tumours such as breast and colon cancer but also in relatively less common tumours such as NSGCT. The detection of circulating tumour cells could help deploy aggressive regimens upfront. 2015 BMJ Publishing Group Ltd.

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.

A pitfall during endobronchial ultrasound-guided transbronchial forceps biopsy of the mediastinal lymph nodes.

PubMed

Krenke, Rafal; Korczynski, Piotr; Gorska, Katarzyna; Chazan, Ryszarda

2014-03-01

The high diagnostic yield and favorable safety profile of endobronchial ultrasound-guided transbronchial forceps biopsy of the mediastinal lymph nodes have been recently demonstrated. We report an unusual technical problem during endobronchial ultrasound-guided transbronchial forceps biopsy that could be a prerequisite for severe complications. A rupture of the steering band precluded closure of the forceps jaws opened in the subcarinal lymph node. A solution to the problem is presented, together with other procedure-related complications reported in the literature. The report emphasizes that a dysfunction of the forceps steering band can result in severe complications when it occurs during transbronchial sampling of mediastinal lesions. Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

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

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

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.

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

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

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.

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.

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.

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.

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

Automatic identification of IASLC-defined mediastinal lymph node stations on CT scans using multi-atlas organ segmentation

NASA Astrophysics Data System (ADS)

Hoffman, Joanne; Liu, Jiamin; Turkbey, Evrim; Kim, Lauren; Summers, Ronald M.

2015-03-01

Station-labeling of mediastinal lymph nodes is typically performed to identify the location of enlarged nodes for cancer staging. Stations are usually assigned in clinical radiology practice manually by qualitative visual assessment on CT scans, which is time consuming and highly variable. In this paper, we developed a method that automatically recognizes the lymph node stations in thoracic CT scans based on the anatomical organs in the mediastinum. First, the trachea, lungs, and spines are automatically segmented to locate the mediastinum region. Then, eight more anatomical organs are simultaneously identified by multi-atlas segmentation. Finally, with the segmentation of those anatomical organs, we convert the text definitions of the International Association for the Study of Lung Cancer (IASLC) lymph node map into patient-specific color-coded CT image maps. Thus, a lymph node station is automatically assigned to each lymph node. We applied this system to CT scans of 86 patients with 336 mediastinal lymph nodes measuring equal or greater than 10 mm. 84.8% of mediastinal lymph nodes were correctly mapped to their stations.

Through the eye of the suprasternal notch: point-of-care sonography for tuberculous mediastinal lymphadenopathy in children.

PubMed

Moseme, Tsepo; Andronikou, Savvas

2014-06-01

Diagnosis of pulmonary tuberculosis (TB) remains a challenge in the pediatric population because of the lack of sputum production for laboratory analysis. Chest radiography is used in the diagnosis of pulmonary TB and the hallmark of diagnosis is the demonstration of hilar or mediastinal lymphadenopathy. Point-of-care sonography of the mediastinum is an alternative to chest radiography in detection of tuberculous lymph nodes. In the rural district health care setting US is often the most commonly available imaging modality and its mobility makes it possible to examine patients at the point of care, reducing the need for patients to travel to a regional hospital to acquire a chest radiograph. We developed and used a simplified technique for performing mediastinal sonography in a pilot study of 30 children (age 0 to 13 years) with proven or suspected TB. We can report that the procedure was successful in demonstrating the anterior mediastinal anatomy and predefined zones in all 30 children. We also recorded lymphadenopathy in 12 children. This report describes our procedural methods and initial results.

Total mechanical stapled oesophagogastric anastomosis on the neck in oesophageal cancer - prevention of postoperative mediastinal complications.

PubMed

Zieliński, Jacek; Jaworski, Radosław; Irga-Jaworska, Ninela; Haponiuk, Ireneusz; Jaśkiewicz, Janusz

2015-12-01

Oesophagogastric anastomosis after oesophagus resection is commonly performed on the neck. Even though a few different techniques of oesophagogastric anastomosis have been previously detailed, both manual and mechanical procedures have been burdened with leakages and strictures. Our simple technique of oesophagogastric anastomosis is a modification of mechanical anastomosis with the use of a circular stapler in order to prevent postoperative leak and concomitant mediastinal complications. Since 2008, we have performed nine oesophagogastric anastomoses following oesophagus resection. The mean age of the operated patients was 54 years. There was no mortality among the operated patients in the early post-operative period. The mean follow-up period for the patients operated on in our department was 17 months until the time of the analysis. None of the patients showed any leakage or stricture, and no mediastinal complications were reported in the group. Following our own experience, mechanical anastomosis with the use of a circular stapler seems to decrease the time of the operation as well as significantly reducing the incidence of leakages from the anastomosis. This type of anastomosis may decrease the number of postoperative strictures and the most dangerous mediastinal infections.

An Endoscopic Nasomediastinal Approach to a Mediastinal Abscess Developing after Zenker's Diverticulectomy

PubMed Central

Gundogdu, Kemal; Eminler, Ahmet Tarik; Parlak, Erkan; Cakmak, Guner

2017-01-01

Zenker's diverticulum is the most frequent symptomatic diverticulum of the esophagus, but the prevalence is <0.1%. The optimal treatment is surgery. Here, we present a nasomediastinal drainage approach to treatment of a mediastinal abscess, developing in the late postoperative period and attributable to leakage from the staple line. PMID:28831318

Utility and Safety of Endoscopic Ultrasound With Bronchoscope-Guided Fine-Needle Aspiration in Mediastinal Lymph Node Sampling: Systematic Review and Meta-Analysis.

PubMed

Dhooria, Sahajal; Aggarwal, Ashutosh N; Gupta, Dheeraj; Behera, Digambar; Agarwal, Ritesh

2015-07-01

The use of endoscopic ultrasound with bronchoscope-guided fine-needle aspiration (EUS-B-FNA) has been described in the evaluation of mediastinal lymphadenopathy. Herein, we conduct a meta-analysis to estimate the overall diagnostic yield and safety of EUS-B-FNA combined with endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA), in the diagnosis of mediastinal lymphadenopathy. The PubMed and EmBase databases were searched for studies reporting the outcomes of EUS-B-FNA in diagnosis of mediastinal lymphadenopathy. The study quality was assessed using the QualSyst tool. The yield of EBUS-TBNA alone and the combined procedure (EBUS-TBNA and EUS-B-FNA) were analyzed by calculating the sensitivity, specificity, positive likelihood ratio, negative likelihood ratio, and diagnostic odds ratio for each study, and pooling the study results using a random effects model. Heterogeneity and publication bias were assessed for individual outcomes. The additional diagnostic gain of EUS-B-FNA over EBUS-TBNA was calculated using proportion meta-analysis. Our search yielded 10 studies (1,080 subjects with mediastinal lymphadenopathy). The sensitivity of the combined procedure was significantly higher than EBUS-TBNA alone (91% vs 80%, P = .004), in staging of lung cancer (4 studies, 465 subjects). The additional diagnostic gain of EUS-B-FNA over EBUS-TBNA was 7.6% in the diagnosis of mediastinal adenopathy. No serious complication of EUS-B-FNA procedure was reported. Clinical and statistical heterogeneity was present without any evidence of publication bias. Combining EBUS-TBNA and EUS-B-FNA is an effective and safe method, superior to EBUS-TBNA alone, in the diagnosis of mediastinal lymphadenopathy. Good quality randomized controlled trials are required to confirm the results of this systematic review. Copyright © 2015 by Daedalus Enterprises.

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.

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

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

Cervical and mediastinal emphysema secondary to third molar extraction.

PubMed

Goodnight, J W; Sercarz, J A; Wang, M B

1994-01-01

A dramatic case of infected emphysema involving multiple deep cervical and mediastinal fascial planes following surgical extraction of a lower third molar in an otherwise healthy man is presented. The differential diagnosis and management of this condition are discussed, specifically contrasting it to necrotizing fasciitis. The direct cause of this complication was the use of an air turbine handpiece and air syringe for the tooth extraction. It is advised that air turbine handpieces and air syringes not be used during minor oral surgical procedures.

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.

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.

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.

Mediastinal lymph node detection and station mapping on chest CT using spatial priors and random forest

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

Liu, Jiamin; Hoffman, Joanne; Zhao, Jocelyn

2016-07-15

Purpose: To develop an automated system for mediastinal lymph node detection and station mapping for chest CT. Methods: The contextual organs, trachea, lungs, and spine are first automatically identified to locate the region of interest (ROI) (mediastinum). The authors employ shape features derived from Hessian analysis, local object scale, and circular transformation that are computed per voxel in the ROI. Eight more anatomical structures are simultaneously segmented by multiatlas label fusion. Spatial priors are defined as the relative multidimensional distance vectors corresponding to each structure. Intensity, shape, and spatial prior features are integrated and parsed by a random forest classifiermore » for lymph node detection. The detected candidates are then segmented by the following curve evolution process. Texture features are computed on the segmented lymph nodes and a support vector machine committee is used for final classification. For lymph node station labeling, based on the segmentation results of the above anatomical structures, the textual definitions of mediastinal lymph node map according to the International Association for the Study of Lung Cancer are converted into patient-specific color-coded CT image, where the lymph node station can be automatically assigned for each detected node. Results: The chest CT volumes from 70 patients with 316 enlarged mediastinal lymph nodes are used for validation. For lymph node detection, their system achieves 88% sensitivity at eight false positives per patient. For lymph node station labeling, 84.5% of lymph nodes are correctly assigned to their stations. Conclusions: Multiple-channel shape, intensity, and spatial prior features aggregated by a random forest classifier improve mediastinal lymph node detection on chest CT. Using the location information of segmented anatomic structures from the multiatlas formulation enables accurate identification of lymph node stations.« less

A Case of Ruptured Aneurysm of the Proper Esophageal Artery with Symptomatic Mediastinal Hematoma

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

Liu, Jiajia; Sato, Yusuke, E-mail: yusuke@doc.med.akita-u.ac.jp; Takahashi, Satoshi

Mediastinal aneurysms are rare but potentially life-threatening. Among these, bronchial artery aneurysms are most frequently reported, whereas up to now aneurysms of the proper esophageal artery had never been reported. A 69-year-old woman was referred to our hospital for treatment of a massive mediastinal hematoma. Enhanced computed tomography and selective proper esophageal arteriography revealed a 5-mm aneurysm in the proper esophageal artery that arises from the thoracic aorta at the Th8 level and has an anastomotic branch with the bronchial artery peripherally. Transcatheter arterial embolization was successfully performed using a mixture of N-butyl cyanoacrylate and lipiodol (1:3 ratio, 0.3 ml). Post-embolizationmore » angiography showed no filling into the aneurysm. The patient recovered with no complications and was discharged on the 25th post-procedure day.« less

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

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

Diagnostic yield of endobronchial ultrasound-guided transbronchial needle aspiration for mediastinal staging in lung cancer*

PubMed Central

Fernández-Bussy, Sebastián; Labarca, Gonzalo; Canals, Sofia; Caviedes, Iván; Folch, Erik; Majid, Adnan

2015-01-01

OBJECTIVE: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive diagnostic test with a high diagnostic yield for suspicious central pulmonary lesions and for mediastinal lymph node staging. The main objective of this study was to describe the diagnostic yield of EBUS-TBNA for mediastinal lymph node staging in patients with suspected lung cancer. METHODS: Prospective study of patients undergoing EBUS-TBNA for diagnosis. Patients ≥ 18 years of age were recruited between July of 2010 and August of 2013. We recorded demographic variables, radiological characteristics provided by axial CT of the chest, location of the lesion in the mediastinum as per the International Association for the Study of Lung Cancer classification, and definitive diagnostic result (EBUS with a diagnostic biopsy or a definitive diagnostic method). RESULTS: Our analysis included 354 biopsies, from 145 patients. Of those 145 patients, 54.48% were male. The mean age was 63.75 years. The mean lymph node size was 15.03 mm, and 90 lymph nodes were smaller than 10.0 mm. The EBUS-TBNA method showed a sensitivity of 91.17%, a specificity of 100.0%, and a negative predictive value of 92.9%. The most common histological diagnosis was adenocarcinoma. CONCLUSIONS: EBUS-TBNA is a diagnostic tool that yields satisfactory results in the staging of neoplastic mediastinal lesions. PMID:26176519

[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.

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)

Total mechanical stapled oesophagogastric anastomosis on the neck in oesophageal cancer – prevention of postoperative mediastinal complications

PubMed Central

Jaworski, Radosław; Irga-Jaworska, Ninela; Haponiuk, Ireneusz; Jaśkiewicz, Janusz

2015-01-01

Oesophagogastric anastomosis after oesophagus resection is commonly performed on the neck. Even though a few different techniques of oesophagogastric anastomosis have been previously detailed, both manual and mechanical procedures have been burdened with leakages and strictures. Our simple technique of oesophagogastric anastomosis is a modification of mechanical anastomosis with the use of a circular stapler in order to prevent postoperative leak and concomitant mediastinal complications. Since 2008, we have performed nine oesophagogastric anastomoses following oesophagus resection. The mean age of the operated patients was 54 years. There was no mortality among the operated patients in the early post-operative period. The mean follow-up period for the patients operated on in our department was 17 months until the time of the analysis. None of the patients showed any leakage or stricture, and no mediastinal complications were reported in the group. Following our own experience, mechanical anastomosis with the use of a circular stapler seems to decrease the time of the operation as well as significantly reducing the incidence of leakages from the anastomosis. This type of anastomosis may decrease the number of postoperative strictures and the most dangerous mediastinal infections. PMID:26855647

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

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

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.

[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.

Fiber 1.56-1.9 μm lasers in treatment of vascular malformations in children and adults

NASA Astrophysics Data System (ADS)

Abushkin, Ivan A.; Privalov, Valeriy A.; Lappa, Alexander V.; Minaev, Vladimir P.

2013-03-01

A few new low invasive fiber laser technologies for treatment of 1) capillary malformations (port-wine stains), 2) venous, arterial, and arteriovenous malformations, 3) lymphatic malformations of 3 types: micro, small and large-cystic lymphangiomas are presented in this work. There were applied 1.56 μm laser distant photocoagulation, 1.56 μm laser endovascular thermotherapy, 1.9 μm laser instant ablation, 1.9 μm laser interstitial thermotherapy, and 1.9 μm laser excision. The technologies were applied to about 300 patients. Good clinical and esthetic results have been achieved in great majority cases.

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.

[Application of trans-abdominal-mediastinal drainage tube in patients with high risk of esophagogastric or esophago-jejunal anastomotic leakage].

PubMed

Wang, Gang-cheng; Han, Guang-sen; Ren, Ying-kun

2012-08-01

To evaluate the therapeutic effects of trans-abdominal-mediastinal drainage tube on the prevention of esophagogastric or esophago-jejunal anastomotic leakage. A total of 79 patients underwent thoraco-abdominal radical resection for gastric cardia cancer, with high risk of leakage of the anatsomosis, from Aug. 2007 to Aug. 2011 were included in this study. They were assigned into 2 groups. Forty one patients had trans-abdominal-mediastinal drainage tube (improvement group) and 38 patients were without the mediastinal drainage tube (control group). The clinical data of all the 79 patients were reviewed and the therapeutic effects of the two treatment approaches were compared. There was anastomotic leakage in four patients of the improvement group. They were with stable vital signs and the median hospital stay was 29.3 days. There was anastomotic leakage in five cases of the contol group and all of them had high fever and chest tightness. One among those five patients had transdermal placement of thoracic drainage tube and was cured, and four among those five patients had second debridement operation, with 3 cured and one death case. Except the one death case, the median hospital stay of the control group was 53.4 days, significantly longer than that of the improvement group (P < 0.05). Although putting trans-abdominal-mediastinal drainage tube can not prevent the leakage of esophagogastric or esophago-jejunnal anastomosis, it can reduce the systemic inflammatory responses, death and painful suffering of the patients caused by anastomotic leakage.

Surgical outcomes in patients with primary mediastinal non-seminomatous germ cell tumours and elevated post-chemotherapy serum tumour markers.

PubMed

De Latour, Bertrand; Fadel, Elie; Mercier, Olaf; Mussot, Sacha; Fabre, Dominique; Fizazi, Karim; Dartevelle, Philippe

2012-07-01

Platinum-based chemotherapy followed by surgical resection of residual masses has become the standard treatment of patients with primary mediastinal non-seminomatous germ cell tumours (NSGCTs). Persistent serum tumour marker (STM) elevation after chemotherapy usually indicates a poor prognosis. We retrospectively assessed surgical outcomes in patients with high STM levels after chemotherapy for primary mediastinal NSGCT. Between 1983 and 2010, residual tumour excision was performed in 21 patients, 20 men and one woman with a median age of 30 years (range: 19-49 years), with primary mediastinal NSGCTs and high STM levels after platinum-based chemotherapy, followed by second-line chemotherapy in 11 patients. Alpha-fetoprotein was elevated in all 21 patients and β-human chorionic gonadotropin in three patients. Permanent histology demonstrated viable germ cell tumour (n=13), teratoma (n=3) or necrosis (n=5). After surgery, the STM levels returned to normal in 11 patients. Eight patients are alive with a median follow-up of 98 months. The 5-year survival rate was 36% and was not significantly affected by the use of preoperative second-line chemotherapy. At univariate analysis, only postoperative STM elevation and residual viable tumour, indicating incomplete resection, were significantly associated with lower survival (P=0.018 and P=0.04, respectively). In patients with primary mediastinal NSGCTs and elevated post-chemotherapy STMs, surgery is warranted when complete resection is deemed feasible. In specialized oncology centres, this aggressive approach can provide a cure in some patients.

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.

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

Repair of tracheomalacia with inflammatory defect and mediastinitis.

PubMed

Sandu, Kishore; Monnier, Yan; Hurni, Michel; Bernath, Marc-Andre; Monnier, Philippe; Wang, Yabo; Ris, Hans-Beat

2011-01-01

We describe a novel repair of an anterior inflammatory tracheal defect with mediastinitis, which occurred after external tracheal suspension of localized intrathoracic tracheomalacia. The malacic tracheal segment of 4-cm length containing the inflammatory tracheal defect was noncircumferentially resected. A temporary endotracheal silicone stent was introduced, and the trachea was closed by a pedicled pectoralis muscle flap reinforced with an embedded rib segment. Retrieval of the stent 5 months postoperatively resulted in a re-epithelialized, persistently stable, noncollapsible tracheal segment that showed the same diameter and configuration as the nonreconstructed part of the trachea. Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

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.

[A comparative study of Da Vinci robot system with video-assisted thoracoscopy in the surgical treatment of mediastinal lesions].

PubMed

Ding, Renquan; Tong, Xiangdong; Xu, Shiguang; Zhang, Dakun; Gao, Xin; Teng, Hong; Qu, Jiaqi; Wang, Shumin

2014-07-20

In recent years, Da Vinci robot system applied in the treatment of intrathoracic surgery mediastinal diseases become more mature. The aim of this study is to summarize the clinical data about mediastinal lesions of General Hospital of Shenyang Military Region in the past 4 years, then to analyze the treatment effect and promising applications of da Vinci robot system in the surgical treatment of mediastinal lesions. 203 cases of mediastinal lesions were collected from General Hospital of Shenyang Military Region between 2010 and 2013. These patients were divided into two groups da Vinci and video-assisted thoracoscopic surgery (VATS) according to the selection of the treatments. The time in surgery, intraoperative blood loss, postoperative drainage amount within three days after surgery, the period of bearing drainage tubes, hospital stays and hospitalization expense were then compared. All patients were successfully operated, the postoperative recovery is good and there is no perioperative death. The different of the time in surgery between two groups is Robots group 82 (20-320) min and thoracoscopic group 89 (35-360) min (P>0.05). The intraoperative blood loss between two groups is robot group 10 (1-100) mL and thoracoscopic group 50 (3-1,500) mL. The postoperative drainage amount within three days after surgery between two groups is robot group 215 (0-2,220) mL and thoracoscopic group 350 (50-1,810) mL. The period of bearing drainage tubes after surgery between two groups is robot group 3 (0-10) d and thoracoscopic group: 5 (1-18) d. The difference of hospital stays between two groups is robot group 7 (2-15) d and thoracoscopic group 9 (2-50) d. The hospitalization expense between two groups is robot group (18,983.6±4,461.2) RMB and thoracoscopic group (9,351.9±2,076.3) RMB (All P<0.001). The da Vinci robot system is safe and efficient in the treatment of mediastinal lesions compared with video-assisted thoracoscopic approach, even though its expense is higher.

Primary Pulmonary Mucinous Cystadenocarcinoma: A Case Report

PubMed Central

Efstathiou, Andreas; Asteriou, Christos; Barbetakis, Nikolaos; Miliaras, Dimosthenis; Kleontas, Athanassios; Karvelas, Christos; Lalountas, Miltiadis

2011-01-01

Primary pulmonary mucinous cystadenocarcinoma (PMCAC) is an extremely rare cystic neoplasm. A case of a 56-year-old male with a cystic lesion of the right lower lobe is described. Preoperative fine needle aspiration cytology and bronchoscopy were inconclusive. The patient underwent a formal right lower lobectomy and mediastinal lymph node dissection. Diagnosis was established intraoperatively. The biological behavior of primary PMCAC is unknown. Therefore, careful long-term follow-up is considered necessary because of lack of experience globally. PMID:21541179

Oral foregut cyst in a neonate.

PubMed

Rosa, Ana Cláudia Garcia; Hiramatsu, Daniel Martins; de Moraes, Fábio Roberto Ruiz; Passador-Santos, Fabrício; de Araújo, Vera Cavalcanti; Soares, Andresa Borges

2013-11-01

Oral foregut cysts are congenital choristomas that arise in the oral cavity during embryonic development from remnants of foregut-derived epithelium. This is an unusual report of a neonate with a large congenital sublingual cystic lesion, extending superficially from the left ventral tongue to the anterior floor of the mouth, impeding breast-feeding. The differential diagnosis included dermoid cyst, epidermoid cyst, mucous retention cyst, and oral lymphangioma. The treatment of choice was enucleation under general anesthesia. Histology showed a cystic lesion with a ciliated pseudostratified columnar epithelium with numerous goblet cells. Immunohistochemistry was positive for cytokeratin 7 and thyroid transcription factor 1 and negative for cytokeratin 20, resulting in a final diagnosis of an oral foregut cyst. Three weeks after surgery, the tongue had healed with good mobility, and breast-feeding could be established. No recurrence was present at 6 months of follow-up.

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

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.

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 ...

Mediastinal tuberculous lymphadenitis presenting as an esophageal intramural tumor: A very rare but important cause for dysphagia

PubMed Central

Pimenta, APA; Preto, JR; Gouveia, AMF; Fonseca, E; Pimenta, MML

2007-01-01

Dysphagia associated with esophageal mechanical obstruction is usually related to malignant esophageal diseases. Benign lesions are rarely a cause for this type of dysphagia, and usually occur either as an intramural tumor or as an extrinsic compression. Mediastinal tuberculous lymphadenitis is rare in adults, and even more rarely causes dysphagia. We report two cases of dysphagia in adult patients caused by mediastinal tuberculous lymphadenitis, presenting radiologically and endoscopically as an esophageal submucosal tumor. Based on the clinical and imaging diagnosis, the patients underwent a right thoracotomy, and excision of the mass attached to and compressing the esophagus. Pathological examination of the specimens showed a chronic granulomatous inflammation with caseous necrosis, which was consistent with tuberculous lymphadenitis. PMID:18023110

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.

Mediastinal mass following chemotherapy in patients with Ewing sarcoma and osteosarcoma.

PubMed

Panadero, M A; Cruz, J J; Gomez, A; Fonseca, E; Garcia, M J; Garcia, J; Martin, G; Sanchez, P; Dueñas, G A

1996-05-01

Thymic hyperplasia following combination chemotherapy for malignant disease is very uncommon in adolescents and adults. Our experience includes a thymic enlargement noted on the sequential computed tomography (CT) in three patients who were disease-free after chemotherapy for Ewing sarcoma (2) and osteosarcoma (1). The development of an anterior mediastinal mass after successful chemotherapy does not always imply relapse of malignant disease. To prevent inappropriate treatment, the possibility of benign aetiology must be considered.

Primary mediastinal hemangiopericytoma treated with preoperative embolization and surgery.

PubMed

Kulshreshtha, Pranjal; Kannan, Narayanan; Bhardwaj, Reena; Batra, Swati; Gupta, Srishti

2014-01-01

Hemangiopericytomas are rare tumors originating from vascular pericytes. The mediastinum is an extremely uncommon site with only a few cases reported. Diagnosis is based on histopathology and immunohistochemistry, which differentiates them from synovial sarcoma and solitary fibrous histiocytoma. They have a variable malignant potential. Treatment is mainly surgical extirpation as the role of adjuvant therapy is controversial. Preoperative embolization has been sparingly used. We report a case of primary mediastinal hemangiopericytoma in a 47-year-old man treated successfully with preoperative embolization and surgery. Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Management of mediastinal syndromes in pediatrics: a new challenge of ultrasound guidance to avoid high-risk general anesthesia.

PubMed

Sola, Chrystelle; Choquet, Olivier; Prodhomme, Olivier; Capdevila, Xavier; Dadure, Christophe

2014-05-01

Adverse events associated with anesthetic management of anterior mediastinal masses in pediatrics are common. To avoid an extremely hazardous general anesthesia, the use of real-time ultrasonography offers an effective alternative in high-risk cases. We report the anesthetic management including a light sedation and ultrasound guidance for regional anesthesia, surgical node biopsy, and placement of a central venous line in two children with an anterior symptomatic mediastinal mass. For pediatric patients with clinical and/or radiologic signs of airway compression, ultrasound guidance provides safety technical assistance to avoid general anesthesia and should be performed for the initial diagnostic and therapeutic procedures. © 2013 John Wiley & Sons Ltd.

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

(18)F-FDG and (18)F-FLT PET/CT imaging in the characterization of mediastinal lymph nodes.

PubMed

Rayamajhi, Sampanna Jung; Mittal, Bhagwant Rai; Maturu, Venkata Nagarjuna; Agarwal, Ritesh; Bal, Amanjit; Dey, Pranab; Shukla, Jaya; Gupta, Dheeraj

2016-04-01

There is currently no single modality for accurate characterization of enlarged mediastinal lymph nodes into benign or malignant. Recently (18)F-fluorothymidine (FLT) has been used as a proliferation marker. In this prospective study, we examined the role of (18)F-fluorodeoxyglucose ((18)F-FDG) positron emission tomography/computed tomography (PET/CT) and (18)F-FLT PET/CT in categorizing mediastinal lymph nodes as benign or malignant. A total of 70 consecutive patients with mediastinal lymphadenopathy detected on computed tomography (CT) or chest radiograph underwent whole body (18)F-FLT PET/CT and (18)F-FDG PET/CT (within 1 week of each other). Lymph nodal tracer uptake was determined by calculation of standardized uptake value (SUV) with both the tracers. Results of PET/CT were compared with histopathology of the lymph nodes. Histopathology results showed thirty-seven patients with sarcoidosis, seven patients with tuberculosis, nine patients with non-small cell lung cancer, five patients with Hodgkin's lymphoma and twelve patients with non-Hodgkin's lymphoma. The mean FDG SUVmax of sarcoidosis, tuberculosis, Hodgkin's and non-Hodgkin's lymphoma was 12.7, 13.4, 8.2, and 8.8, respectively, and the mean FLT SUVmax was 6.0, 5.4, 4.4, and 3.8, respectively. It was not possible to characterize mediastinal lymphadenopathy as benign or malignant solely based on FDG SUVmax values (p > 0.05) or FLT SUVmax values (p > 0.05). There was no significant difference in FDG uptake (p > 0.9) or FLT uptake (p > 0.9) between sarcoidosis and tuberculosis. In lung cancer patients, the FDG SUVmax and FLT SUVmax of those lymph nodes with tumor infiltration on biopsy was 6.7 and 3.9, respectively, and those without nodal infiltration was 6.4 and 3.7, respectively, and both the tracers were not able to characterize the nodal status as malignant or benign (p > 0.05). Though (18)F-FLT PET/CT and (18)F-FDG PET/CT reflect different aspects of biology, i.e., proliferation and metabolism

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.

Mediastinal irradiation in a patient affected by lung carcinoma after heart transplantation: Helical tomotherapy versus three dimensional conformal radiotherapy.

PubMed

Giugliano, Francesca M; Iorio, Vincenzo; Cammarota, Fabrizio; Toledo, Diego; Senese, Rossana; Francomacaro, Ferdinando; Muto, Matteo; Muto, Paolo

2016-04-26

Patients who have undergone solid organ transplants are known to have an increased risk of neoplasia compared with the general population. We report our experience using mediastinal irradiation with helical tomotherapy versus three-dimensional conformal radiation therapy to treat a patient with lung carcinoma 15 years after heart transplantation. Our dosimetric evaluation showed no particular difference between the techniques, with the exception of some organs. Mediastinal irradiation after heart transplantation is feasible and should be considered after evaluation of the risk. Conformal radiotherapy or intensity-modulated radiotherapy appears to be the appropriate treatment in heart-transplanted oncologic patients.

[Esophageal perforation and mediastinitis caused by fishbone ingestion: report of 3 cases].

PubMed

Vásquez, Julio C; Montesinos, Efraín; Castillo, Luis; Rojas, Luis; Peralta, Julio

2006-01-01

We present 3 consecutive cases of fishbone perforation of the esophagus that resulted in acute mediastinitis. All patients were successfully managed with drainage and debridement of the mediastinum and pleural cavity. However, temporary exclusion with external ligation of the esophagus was also performed in one patient, with spontaneous recanalization two weeks later. We discuss the possible role of this surgical maneuver in the management of esophageal perforation.

Anterior mediastinal haematoma and left haemothorax on well-controlled oral anticoagulant therapy.

PubMed Central

Abaskaron, M.; Peterson, G.; Huang, T. Y.

1983-01-01

An anterior mediastinal haematoma and left haemothorax developed in a hypertensive diabetic patient on oral anticoagulant therapy. This occurred in spite of well-controlled anticoagulation and the absence of other evidence of systemic bleeding. Angiography and daily chest X-ray follow-up were not only sufficient to confirm the diagnosis, but also avoided hazardous interventional procedures. Images Fig. 1a Fig. 1b PMID:6844193

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;…

Extramedullary plasmacytoma (EMP): Report of a case manifested as a mediastinal mass and multiple pulmonary nodules and review of literature.

PubMed

Luh, Shi-Ping; Lai, Yih-Shyong; Tsai, Chung-Hong; Tsao, Thomas Chang-Yao

2007-10-27

Extramedullary plasmacytoma (EMP) is a rare plasma cell neoplasm of soft tissue without bone marrow involvement or other systemic characteristics of multiple myeloma A 42 year-old woman presented with intermittent dry cough of 10 months duration. Her breathing sound was slightly coarse without rales or rhonchi on auscultation. CT scan revealed a right anterior mediastinal shadow with multiple pulmonary nodular lesions. A video-assisted thoracoscopic surgery (VATS) was performed. Histopathology showed it to be a myeloma. This is the first presentation of EMP with a mediastinal mass with multiple pulmonary nodules.

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.

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)

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

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

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.

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

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.

Uniportal transcervical video-assisted thoracoscopic surgery (VATS) approach for pulmonary lobectomy combined with transcervical extended mediastinal lymphadenectomy (TEMLA)

PubMed Central

Rybak, Mariusz; Solarczyk-Bombik, Katarzyna; Wilkojc, Michal; Czajkowski, Wojciech; Kosinski, Sylweriusz; Fryzlewicz, Edward; Nabialek, Tomasz; Pankowski, Juliusz

2017-01-01

Background To present the technique of uniportal transcervical video-assisted thoracoscopic surgery (VATS) approach for pulmonary lobectomy combined with transcervical extended mediastinal lymphadenectomy (TEMLA). Methods Transcervical extended approach utilizes a typical a 5–8 centimeters collar incision in the neck. The critical technical point enabling a wide access to the chest is an elevation of the sternal manubrium with a special retractor (modified Rochard frame, Asculap-Chifa Company). A bilateral visualization of the laryngeal recurrent and vagus nerves is usually performed to avoid injury of these structures. The uniportal transcervical VATS lobectomy for NSCLC is preceded by TEMLA to enable optimal intraoperative staging of the mediastinal nodes and perform extensive bilateral lymphadenectomy, which theoretically might affect survival. VATS lobectomy is the next step after obtaining results of intraoperative examination of the nodes. Ventilation of the operated lung is disconnected and the mediastinal pleura is opened. Pleural adhesions are divided. The branches of the pulmonary artery and vein and the lobar bronchus are sequentially dissected and managed with endo staplers. The fissure is divided with endo stapler and the resected lobe is removed in endobag. Results There were 9 patients operated on in the period 1.2.2016–30.7.2016. In one patient with left lower lobe tumor we had to convert to uniportal VATS left lower lobectomy due to extensive adhesions. There was no mortality and complications occurred in 2 patients. The mean operative time was 258.1 min (200–385 min) for the whole TEMLA procedure with imprint cytology and lobectomy and 168.1 min (110–295 min) for a lobectomy solely. Conclusions A uniportal transcervical video-assisted thoracoscopic surgery (VATS) approach for pulmonary lobectomy combined with TEMLA provides an opportunity for radical pulmonary resection and super radical extensive mediastinal lymphadenectomy. PMID:28523132

Mediastinal micro-vessels clipping during lymph node dissection may contribute to reduce postoperative pleural drainage

PubMed Central

Yan, Shi; Wang, Xing; Lv, Chao; Phan, Kevin; Wang, Yuzhao; Wang, Jia; Yang, Yue

2016-01-01

Background Postoperative pleural drainage markedly influences the length of postoperative stay and financial costs of medical care. The aim of this study is to retrospectively investigate potentially predisposing factors related to pleural drainage after curative thoracic surgery and to explore the impact of mediastinal micro-vessels clipping on pleural drainage control after lymph node dissection. Methods From February 2012 to November 2013, 322 consecutive cases of operable non-small cell lung cancers (NSCLC) undergoing lobectomy and mediastinal lymph node dissection with or without application of clipping were collected. Total and daily postoperative pleural drainage were recorded. Propensity score matching (1:2) was applied to balance variables potentially impacting pleural drainage between group clip and group control. Analyses were performed to compare drainage volume, duration of chest tube and postoperative hospital stay between the two groups. Variables linked with pleural drainage in whole cohort were assessed using multivariable logistic regression analysis. Results Propensity score matching resulted in 197 patients (matched cohort). Baseline patient characteristics were matched between two groups. Group clip showed less cumulative drainage volume (P=0.020), shorter duration of chest tube (P=0.031) and postoperative hospital stay (P=0.022) compared with group control. Risk factors significantly associated with high-output drainage in multivariable logistic regression analysis were being male, age >60 years, bilobectomy/sleeve lobectomy, pleural adhesion, the application of clip applier, duration of operation ≥220 minutes and chylothorax (P<0.05). Conclusions This study suggests that mediastinal micro-vessels clipping during lymph node dissection may reduce postoperative pleural drainage and thus shorten hospital stay. PMID:27076936

Origin and pharmacological response of atrial tachyarrhythmias induced by activation of mediastinal nerves in canines.

PubMed

Armour, J Andrew; Richer, Louis-Philippe; Pagé, Pierre; Vinet, Alain; Kus, Teresa; Vermeulen, Michel; Nadeau, Réginald; Cardinal, René

2005-03-31

We sought to determine the sites of origin of atrial tachyarrhythmias induced by activating mediastinal nerves, as well as the response of such arrhythmias to autonomic modulation. Under general anaesthesia, atrioventricular block was induced after thoracotomy in 19 canines. Brief trains of 5 electrical stimuli were delivered to right-sided mediastinal nerves during the atrial refractory period. Unipolar electrograms were recorded from 191 right and left atrial epicardial sites under several conditions, i.e. (i) with intact nervous systems and following (ii) acute decentralization of the intrathoracic nervous system or administration of (iii) atropine, (iv) timolol, (v) hexamethonium. Concomitant right atrial endocardial mapping was performed in 7 of these dogs. Mediastinal nerve stimulation consistently initiated bradycardia followed by atrial tachyarrhythmias. In the initial tachyarrhythmia beats, early epicardial breakthroughs were identified in the right atrial free wall (28/50 episodes) or Bachmann bundle region (22/50), which corresponded to endocardial sites of origin associated with the right atrial subsidiary pacemaker complex, i.e. the crista terminalis and dorsal locations including the right atrial aspect of the interatrial septum. Neuronally induced responses were eliminated by atropine, modified by timolol and unaffected by acute neuronal decentralization. After hexamethonium, responses to extra-pericardial but not intra-pericardial nerve stimulation were eliminated. It is concluded that concomitant activation of cholinergic and adrenergic efferent intrinsic cardiac neurons induced by right-sided efferent neuronal stimulation initiates atrial tachyarrhythmias that originate from foci anatomically related to the right atrial pacemaker complex and tissues underlying major atrial ganglionated plexuses.

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.

[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.

Metastatic mediastinal mature teratoma with malignant transformation in a young man with an adenocarcinoma in a Klinefelter's syndrome: Case report and review of the literature.

PubMed

Le Fèvre, C; Vigneron, C; Schuster, H; Walter, A; Marcellin, L; Massard, G; Lutz, P; Noël, G

2018-05-01

Malignant transformation of mediastinal mature teratoma is extremely rare and worsens the prognosis of the disease. Transformation can appear synchronously to or several years after the initial diagnosis. Clinical and radiological signs can orientate the clinician but the definitive diagnosis is obtained thanks to histology. An 11 year-old boy presented with a mediastinal mature teratoma and bone and pulmonary metastases. He received six cycles of chemotherapy combining etoposide, ifosfamide, cisplatin, followed by resection of a 16×14×9cm mediastinal mass. Karyotype analysis revealed the presence of an additional sex chromosome X (47 XXY) pathognomonic of Klinefelter's syndrome. Ten years later, sciatalgia revealed malignant transformation of a pre-existing sacral bone metastasis into gastrointestinal adenocarcinoma. The patient received four cycles of chemotherapy combining oxaliplatin, 5-fluorouracil and cetuximab. This treatment was followed by a complete resection of the sacral metastasis and completed with adjuvant irradiation of 54Gy in 30 daily fractions. Twelve months after the diagnosis of relapse, the patient remained alive without disease. To our knowledge, this is the first case of adenocarcinoma developed in bone metastases of a mediastinal mature teratoma in a boy with a Klinefelter's syndrome. We propose a review of the literature and an analysis of 20 others published cases of mediastinal teratoma with malignant transformation into adenocarcinoma. Copyright © 2018 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.

RESIDENTIAL RADON AND BIRTH DEFECTS: A POPULATION-BASED ASSESSMENT

PubMed Central

Langlois, Peter H; Lee, MinJae; Lupo, Philip J; Rahbar, Mohammad H; Cortez, Ruben K

2015-01-01

BACKGROUND Associations have been reported between maternal radiation exposure and birth defects. No such studies were found on radon. Our objective was to determine if there is an association between living in areas with higher radon levels and birth defects. METHODS The Texas Birth Defects Registry provided data on all birth defects from 1999–2009 from the entire state. Mean radon levels by geologic region came from the Texas Indoor Radon Survey. The association between radon and birth defects was estimated using multilevel mixed effect Poisson regression. RESULTS Birth defects overall were not associated with residential radon levels. Of the 100 other birth defect groups with at least 500 cases, 14 were significantly elevated in areas with high mean radon level in crude analyses, and 9 after adjustment for confounders. Cleft lip with/without cleft palate had an adjusted prevalence ratio (aPR) of 1.16 per 1 picoCurie/liter (pCi/l) increase in exposure to region mean radon, 95% confidence interval (CI) 1.08, 1.26. Cystic hygroma / lymphangioma had an aPR of 1.22 per 1 pCi/l increase, 95% CI 1.02, 1.46. Other associations were suggested but not as consistent: three skeletal defects, Down syndrome, other specified anomalies of the brain, and other specified anomalies of the bladder and urethra. CONCLUSIONS In the first study of residential radon and birth defects, we found associations with cleft lip w/wo cleft palate and cystic hygroma / lymphangioma. Other associations were suggested. The ecological nature of this study and multiple comparisons suggest that our results be interpreted with caution. PMID:25846606

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

[Mediastinal teratoma with malignant transformation of the somatic component. Clinical report].

PubMed

Gerardo, Rita; Morgado, Carolina; Calvo, Dolores; Pinto, Eugénia; Bravio, Ivan; Castelão, Nelson; Martelo, Fernando

2009-01-01

Mediastinal germ cell tumours (M-GCT) are rare forms of neoplasms compared with other tumours of the same location. They are classified in seminomas, malignant non-seminomatous GCT and teratomas. The malignant transformation of the somatic component of the teratoma, with sarcomatous or carcinomatous degeneration, is even more uncommon. We report the clinical case of a 32 year old man who presented with severe chest pain on the right hemithorax. The image exams revealed the existence of a large heterogeneous lesion with a diameter of 7.7 cm, with areas of lipomatous density and a calcic image with the appearance of a tooth, in the right projection of the anterior mediastinum, in the vicinity of the large vessels, compatible with teratoma. The transthoracic biopsy (CT guided) showed morphologic aspects of sarcoma. The patient was operated on with the en bloc resection of the mediastinal mass, right lung, a segment of the pericardium and the thymus. The pathological studies showed a teratoma with malignant transformation of the mesenquimatous component, with muscular differentiation into leiomiosarcoma and rabdomiosarcoma. After surgery, the patient was treated with a scheme of doxorubicin and ifosfamide. The most prominent concepts related to this clinical entity, as well as its treatment, are debated in this article, based on the most recent publications dedicated to the subject.

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

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.

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.

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.

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.

Hepatic lymphatics: anatomy and related diseases.

PubMed

Pupulim, Lawrence F; Vilgrain, Valérie; Ronot, Maxime; Becker, Christoph D; Breguet, Romain; Terraz, Sylvain

2015-08-01

The liver normally produces a large amount of lymph. It is estimated that between 25% and 50% of the lymph received by the thoracic duct comes from the liver. In normal conditions, hepatic lymphatics are not depicted on cross-sectional imaging. They are divided in lymphatics of deep system (lymphatics following the hepatic veins and the portal tract) and those of superficial system (convex surface and inferior surface). A variety of diseases may affect hepatic lymphatics and in general they manifest as lymphedema, lymphatic mass, or cystic lesions. Abnormal distended lymphatics are especially seen in periportal spaces as linear hypoattenuations on CT or strong linear hyperintensities on heavily T2-weighted MR imaging. Lymphatic tumor spread as in lymphoma and lymphangitic carcinomatosis manifests as periportal masses and regional lymph node enlargement. Lymphatic disruption after trauma or surgery is depicted as perihepatic fluid collections of lymph (lymphocele). Lymphatic malformation such as lymphangioma is seen on imaging as cystic spaces of variable size.

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

MRI-Guided Percutaneous Biopsy of Mediastinal Masses Using a Large Bore Magnet: Technical Feasibility

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

Garnon, J., E-mail: juliengarnon@gmail.com; Ramamurthy, N., E-mail: nitin-ramamurthy@hotmail.com; Caudrelier J, J., E-mail: caudjean@yahoo.fr

2016-05-15

ObjectiveTo evaluate the diagnostic accuracy and safety of magnetic resonance imaging (MRI)-guided percutaneous biopsy of mediastinal masses performed using a wide-bore high-field scanner.Materials and MethodsThis is a retrospective study of 16 consecutive patients (8 male, 8 female; mean age 74 years) who underwent MRI-guided core needle biopsy of a mediastinal mass between February 2010 and January 2014. Size and location of lesion, approach taken, time for needle placement, overall duration of procedure, and post-procedural complications were evaluated. Technical success rates and correlation with surgical pathology (where available) were assessed.ResultsTarget lesions were located in the anterior (n = 13), middle (n = 2), and posterior mediastinummore » (n = 1), respectively. Mean size was 7.2 cm (range 3.6–11 cm). Average time for needle placement was 9.4 min (range 3–18 min); average duration of entire procedure was 42 min (range 27–62 min). 2–5 core samples were obtained from each lesion (mean 2.6). Technical success rate was 100 %, with specimens successfully obtained in all 16 patients. There were no immediate complications. Histopathology revealed malignancy in 12 cases (4 of which were surgically confirmed), benign lesions in 3 cases (1 of which was false negative following surgical resection), and one inconclusive specimen (treated as inaccurate since repeat CT-guided biopsy demonstrated thymic hyperplasia). Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy in our study were 92.3, 100, 100, 66.7, and 87.5 %, respectively.ConclusionMRI-guided mediastinal biopsy is a safe procedure with high diagnostic accuracy, which may offer a non-ionizing alternative to CT guidance.« less

[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.

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.

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 .

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.

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.

Diagnostic Yield of CT-Guided Percutaneous Transthoracic Needle Biopsy for Diagnosis of Anterior Mediastinal Masses.

PubMed

Petranovic, Milena; Gilman, Matthew D; Muniappan, Ashok; Hasserjian, Robert P; Digumarthy, Subba R; Muse, Victorine V; Sharma, Amita; Shepard, Jo-Anne O; Wu, Carol C

2015-10-01

The purpose of this study was to evaluate the diagnostic yield and accuracy of CT-guided percutaneous biopsy of anterior mediastinal masses and assess prebiopsy characteristics that may help to select patients with the highest diagnostic yield. Retrospective review of all CT-guided percutaneous biopsies of the anterior mediastinum conducted at our institution from January 2003 through December 2012 was performed to collect data regarding patient demographics, imaging characteristics of biopsied masses, presence of complications, and subsequent surgical intervention or medical treatment (or both). Cytology, core biopsy pathology, and surgical pathology results were recorded. A per-patient analysis was performed using two-tailed t test, Fisher's exact test, and Pearson chi-square test. The study cohort included 52 patients (32 men, 20 women; mean age, 49 years) with mean diameter of mediastinal mass of 6.9 cm. Diagnostic yield of CT-guided percutaneous biopsy was 77% (40/52), highest for thymic neoplasms (100% [11/11]). Non-diagnostic results were seen in 12 of 52 patients (23%), primarily in patients with lymphoma (75% [9/12]). Fine-needle aspiration yielded the correct diagnosis in 31 of 52 patients (60%), and core biopsy had a diagnostic rate of 77% (36/47). None of the core biopsies were discordant with surgical pathology. There was no statistically significant difference between the diagnostic and the nondiagnostic groups in patient age, lesion size, and presence of necrosis. The complication rate was 3.8% (2/52), all small self-resolving pneumothoraces. CT-guided percutaneous biopsy is a safe diagnostic procedure with high diagnostic yield (77%) for anterior mediastinal lesions, highest for thymic neoplasms (100%), and can potentially obviate more invasive procedures.

Longterm effects of cardiac mediastinal nerve cryoablation on neural inducibility of atrial fibrillation in canines.

PubMed

Leiria, Tiago Luiz Luz; Glavinovic, Tamara; Armour, J Andrew; Cardinal, René; de Lima, Gustavo Glotz; Kus, Teresa

2011-04-26

In canines, excessive activation of select mediastinal nerve inputs to the intrinsic cardiac nervous system induces atrial fibrillation (AF). Since ablation of neural elements is proposed as an adjunct to circumferential pulmonary vein ablation for AF, we investigated the short and long-term effects of mediastinal nerve ablation on AF inducibility. Under general anesthesia, in 11 dogs several mediastinal nerve sites were identified on the superior vena cava that, when stimulated electrically during the atrial refractory period, reproducibly initiated AF. Cryoablation of one nerve site was then performed and inducibility retested early (1-2 months post Cryo; n=7) or late (4 months post Cryo; n=4). Four additional dogs that underwent a sham procedure were retested 1 to 2 months post-surgery. Stimulation induced AF at 91% of nerve sites tested in control versus 21% nerve sites early and 54% late post-ablation (both P<0.05). Fewer stimuli were required to induce AF in controls versus the Early Cryo group; this capacity returned to normal values in the Late Cryo group. AF episodes were longer in control versus the Early or Late Cryo groups. Heart rate responses to vagal or stellate ganglion stimulation, as well as to local nicotine infusion into the right coronary artery, were similar in all groups. In conclusion, focal damage to intrinsic cardiac neuronal inputs causes short-term stunning of neuronal inducibility of AF without major loss of overall adrenergic or cholinergic efferent neuronal control. That recovery of AF inducibility occurs rapidly post-surgery indicates the plasticity of intrathoracic neuronal elements to focal injury. Copyright © 2011 Elsevier B.V. All rights reserved.

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

The concept of negative pressure wound therapy (NPWT) after poststernotomy mediastinitis--a single center experience with 54 patients.

PubMed

Ennker, Ina C; Malkoc, Anita; Pietrowski, Detlef; Vogt, Peter M; Ennker, Juergen; Albert, Alexander

2009-01-12

Deep sternal infections, also known as poststernotomy mediastinitis, are a rare but often fatal complication in cardiac surgery. They are a cause of increased morbidity and mortality and have a significant socioeconomic aspect concerning the health system. Negative pressure wound therapy (NPWT) followed by muscular pectoralis plasty is a quite new technique for the treatment of mediastinitis after sternotomy. Although it could be demonstrated that this technique is at least as safe and reliable as other techniques for the therapy of deep sternal infections, complications are not absent. We report about our experiences and complications using this therapy in a set of 54 patients out of 3668 patients undergoing cardiac surgery in our institution between January 2005 and April 2007.

Spontaneous mediastinal myeloid sarcoma in a common marmoset (Callithrix jacchus) and review of the veterinary literature

PubMed Central

Morosco, Danielle T.; Cline, Curtis R.; Owston, Michael A.; Kumar, Shyamesh; Dick, Edward J.

2017-01-01

Background Myeloid sarcoma is a rare manifestation of myeloproliferative disorder defined as an extramedullary mass composed of myeloid precursor cells. A 9-month old, female, common marmoset (Callithrix jacchus) had increased respiratory effort. Methods A complete necropsy with histology and immunohistochemistry was performed. Results The thymus was replaced by a firm, grey-tan mass with a faint green tint, filling over 50% of the thoracic cavity. Sheets of granulocytes, lymphoid cells, nucleated erythrocytes, megakaryocytes, and hematopoietic precursors of indeterminate cell lineage replaced the thymus, perithymic connective tissue, mediastinal adipose tissues, epicardium, and much of the myocardium. The cells demonstrated diffuse strong cytoplasmic immunoreactivity for lysozyme, and strong, multifocal membranous immunoreactivity for CD117. Conclusion We report the first case of a myeloid sarcoma in a common marmoset (Callithrix jacchus), similar to reported human cases of mediastinal myeloid sarcoma, and present a review of myeloproliferative diseases from the veterinary literature. PMID:28145579

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.

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 ...

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.

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.

A rare constellation of empyema, lung abscess, and mediastinal abscess as a complication of endobronchial ultrasound-guided transbronchial needle aspiration.

PubMed

Huang, Chun-Ta; Chen, Chung-Yu; Ho, Chao-Chi; Yu, Chong-Jen

2011-07-01

The introduction of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) brought about significant advancement in the field of bronchoscopy. The major indications for EBUS-TBNA are lung cancer staging and diagnosis of mediastinal lymphadenopathy. This procedure is minimally invasive and cost saving, and no complications have been described in large-scale studies. In this report, we present a case of empyema, lung abscess, and mediastinal abscess that developed in a patient undergoing EBUS-TBNA; the patient subsequently recovered uneventfully after aggressive surgical debridement and antimicrobial therapy. Copyright © 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

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

Real-time prediction of mediastinal lymph node malignancy by endobronchial ultrasound.

PubMed

Shafiek, Hanaa; Fiorentino, Federico; Peralta, Alejandro David; Serra, Enrique; Esteban, Blanca; Martinez, Rocío; Noguera, Maria Angels; Moyano, Pere; Sala, Ernest; Sauleda, Jaume; Cosío, Borja G

2014-06-01

To evaluate the utility of different ultrasonographic (US) features in differentiating benign and malignant lymph node (LN) by endobronchial ultrasound (EBUS) and validate a score for real-time clinical application. 208 mediastinal LN acquired from 141 patients were analyzed. Six different US criteria were evaluated (short axis ≥10 mm, shape, margin, echogenicity, and central hilar structure [CHS], and presence of hyperechoic density) by two observers independently. A simplified score was generated where the presence of margin distinction, round shape and short axis ≥10 mm were scored as 1 and heterogeneous echogenicity and absence of CHS were scored as 1.5. The score was evaluated prospectively for real-time clinical application in 65 LN during EBUS procedure in 39 patients undertaken by two experienced operators. These criteria were correlated with the histopathological results and the sensitivity, specificity, positive and negative predictive values (PPV and NPV) were calculated. Both heterogenicity and absence of CHS had the highest sensitivity and NPV (≥90%) for predicting LN malignancy with acceptable inter-observer agreement (92% and 87% respectively). On real-time application, the sensitivity and specificity of the score >5 were 78% and 86% respectively; only the absence of CHS, round shape and size of LN were significantly associated with malignant LN. Combination of different US criteria can be useful for prediction of mediastinal LN malignancy and valid for real-time clinical application. Copyright © 2013 SEPAR. Published by Elsevier Espana. All rights reserved.

Mediastinal lymph node dissection versus mediastinal lymph node sampling for early stage non-small cell lung cancer: a systematic review and meta-analysis.

PubMed

Huang, Xiongfeng; Wang, Jianmin; Chen, Qiao; Jiang, Jielin

2014-01-01

This systematic review and meta-analysis aimed to evaluate the overall survival, local recurrence, distant metastasis, and complications of mediastinal lymph node dissection (MLND) versus mediastinal lymph node sampling (MLNS) in stage I-IIIA non-small cell lung cancer (NSCLC) patients. A systematic search of published literature was conducted using the main databases (MEDLINE, PubMed, EMBASE, and Cochrane databases) to identify relevant randomized controlled trials that compared MLND vs. MLNS in NSCLC patients. Methodological quality of included randomized controlled trials was assessed according to the criteria from the Cochrane Handbook for Systematic Review of Interventions (Version 5.1.0). Meta-analysis was performed using The Cochrane Collaboration's Review Manager 5.3. The results of the meta-analysis were expressed as hazard ratio (HR) or risk ratio (RR), with their corresponding 95% confidence interval (CI). We included results reported from six randomized controlled trials, with a total of 1,791 patients included in the primary meta-analysis. Compared to MLNS in NSCLC patients, there was no statistically significant difference in MLND on overall survival (HR = 0.77, 95% CI 0.55 to 1.08; P = 0.13). In addition, the results indicated that local recurrence rate (RR = 0.93, 95% CI 0.68 to 1.28; P = 0.67), distant metastasis rate (RR = 0.88, 95% CI 0.74 to 1.04; P = 0.15), and total complications rate (RR = 1.10, 95% CI 0.67 to 1.79; P = 0.72) were similar, no significant difference found between the two groups. Results for overall survival, local recurrence rate, and distant metastasis rate were similar between MLND and MLNS in early stage NSCLC patients. There was no evidence that MLND increased complications compared with MLNS. Whether or not MLND is superior to MLNS for stage II-IIIA remains to be determined.

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.

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.

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

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

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.

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.

Challenging posterior mediastinal mass resection via a minimally invasive approach with neurological monitoring.

PubMed

Smail, Hassiba; Baste, Jean Marc; Melki, Jean; Peillon, Christophe

2013-02-01

We report a novel surgical strategy for the resection of a rare type of posterior mediastinal tumour in a young patient. A melanotic schwannoma arose from the left thoracic sympathetic chain, adjacent to the origin of the artery of Adamkiewicz. Successful excision of this tumour via a minimally invasive approach without arterial or spinal cord injury was possible with the aid of neurological monitoring using spinal-evoked potentials.

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.

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.

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.

[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.

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…

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.

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

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.

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.

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. .

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.

The concept of negative pressure wound therapy (NPWT) after poststernotomy mediastinitis – a single center experience with 54 patients

PubMed Central

2009-01-01

Deep sternal infections, also known as poststernotomy mediastinitis, are a rare but often fatal complication in cardiac surgery. They are a cause of increased morbidity and mortality and have a significant socioeconomic aspect concerning the health system. Negative pressure wound therapy (NPWT) followed by muscular pectoralis plasty is a quite new technique for the treatment of mediastinitis after sternotomy. Although it could be demonstrated that this technique is at least as safe and reliable as other techniques for the therapy of deep sternal infections, complications are not absent. We report about our experiences and complications using this therapy in a set of 54 patients out of 3668 patients undergoing cardiac surgery in our institution between January 2005 and April 2007. PMID:19138422

"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.

Effectiveness of the Benign and Malignant Diagnosis of Mediastinal and Hilar Lymph Nodes by Endobronchial Ultrasound Elastography.

PubMed

Huang, Haidong; Huang, Zhiang; Wang, Qin; Wang, Xinan; Dong, Yuchao; Zhang, Wei; Zarogoulidis, Paul; Man, Yan-Gao; Schmidt, Wolfgang Hohenforst; Bai, Chong

2017-01-01

Background and Objectives: Endobronchial ultrasound elastography is a new technique for describing the stiffness of tissue during endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA). The aims of this study were to investigate the diagnostic value of Endobronchial ultrasound (EBUS) elastography for distinguishing the difference between benign and malignant lymph nodes among mediastinal and hilar lymph node. Materials and Methods: From June 2015 to August 2015, 47 patients confirmed of mediastinal and hilar lymph node enlargement through examination of Computed tomography (CT) were enrolled, and a total of 78 lymph nodes were evaluated by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA). EBUS-guided elastography of lymph nodes was performed prior to EBUS-TBNA. A convex probe EBUS was used with a new EBUS processor to assess elastographic patterns that were classified based on color distribution as follows: Type 1, predominantly non-blue (green, yellow and red); Type 2, part blue, part non-blue (green, yellow and red); Type 3, predominantly blue. Pathological determination of malignant or benign lymph nodes was used as the gold standard for this study. The elastographic patterns were compared with the final pathologic results from EBUS-TBNA. Results: On pathological evaluation of the lymph nodes, 45 were benign and 33 were malignant. The lymph nodes that were classified as Type 1 on endobronchial ultrasound elastography were benign in 26/27 (96.3%) and malignant in 1/27 (3.7%); for Type 2 lymph nodes, 15/20 (75.0%) were benign and 5/20 (25.0%) were malignant; Type 3 lymph nodes were benign in 4/31 (12.9%) and malignant in 27/31 (87.1%). In classifying Type 1 as 'benign' and Type 3 as 'malignant,' the sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy rates were 96.43%, 86.67%, 87.10%, 96.30%, 91.38%, respectively. Conclusion: EBUS elastography of mediastinal and

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.

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

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

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.

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.

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.

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

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

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

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 ...

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.

Soft tissue involvement, mediastinal pseudotumor, and venous thrombosis in pustulotic arthro-osteitis. A study of eight new cases.

PubMed

van Holsbeeck, M; Martel, W; Dequeker, J; Favril, A; Gielen, J; Verschakelen, J; Hoogmartens, M

1989-01-01

A syndrome of hyperostosis of the thoracic wall, nonspecific signs of inflammatory disease, and palmar and plantar pustulosis is described in eight patients (Table 1). Seven had intersternocostoclavicular ossification, and one had chronic recurrent multifocal osteomyelitis. This complex of findings has been called "pustulotic arthro-osteitis". This report emphasizes the periosseous soft tissue inflammation and the unexplained subclavian and mediastinal vein thrombosis seen in two patients. Inflammatory periosseous and mediastinal lesions were seen on plain films in all eight patients and on computed tomographic (CT) scans in seven. Radiographs of the spine showed a spondyloarthropathy in three patients. This was characterized by ossification of the vertebral ligaments and sclerosis of the vertebral bodies. Awareness of the radiologic features of pustulotic arthro-osteitis is important because the clinical, biochemical and pathologic findings are often nonspecific and misleading.

CT findings of descending necrotising mediastinitis via the carotid space ('Lincoln Highway').

PubMed

Kono, T; Kohno, A; Kuwashima, S; Iimura, F; Fujioka, M; Miyata, R; Mizusawa, Y; Kagawa, F; Kaneko, S; Yoshino, Y; Misawa, M; Ito, M; Otsuka, M; Seki, I

2001-02-01

A 3-year-old girl with fever and neck swelling showed widening of the superior mediastinum on chest radiographs. Contrast-enhanced CT of the neck revealed ill-defined low-attenuation areas with a thick, enhanced rim adjacent to the hypertrophied palatine tonsil. The abscess extended inferiorly within the carotid sheath between the carotid artery and the internal jugular vein into the anterior mediastinum. The carotid space is considered an important conduit of descending necrotising mediastinitis and is called the 'Lincoln Highway' as previously suggested.

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.

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.

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.

[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.

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.

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.

Therapeutic Outcomes of Pectoralis Major Muscle Turnover Flap in Mediastinitis.

PubMed

Bagheri, Reza; Tashnizi, Mohammad Abbasi; Haghi, Seyed Ziaollah; Salehi, Maryam; Rajabnejad, Ata'ollah; Safa, Mohsen Hatami Ghale; Vejdani, Mohammad

2015-08-01

This study aimed to evaluate the therapeutic results and safety of pectoralis major muscle turnover flaps in the treatment of mediastinitis after coronary artery bypass grafting (CABG) procedures. Data regarding 33 patients with post-CABG deep sternal wound infections (DSWIs) who underwent pectoralis major muscle turnover flap procedures in the Emam Reza and Ghaem Hospitals of Mashhad, Iran were reviewed in this study. For each patient, age, sex, hospital stay duration, remission, recurrence, and associated morbidity and mortality were evaluated. Of the 2,447 CABG procedures that were carried out during the time period encompassed by our study, DSWIs occurred in 61 patients (2.5%). Of these 61 patients, 33 patients (nine females [27.3%] and 24 males [72.7%]) with an average age of 63±4.54 years underwent pectoralis major muscle turnover flap placement. Symptoms of infection mainly occurred within the first 10 days after surgery (mean, 10.24±13.62 days). The most common risk factor for DSWIs was obesity (n=16, 48.4%) followed by diabetes mellitus (n=13, 39.4%). Bilateral and unilateral pectoralis major muscle turnover flaps were performed in 20 patients (60.6%) and 13 patients (39.4%), respectively. Complete remission was achieved in 25 patients (75.7%), with no recurrence in the follow-up period. Four patients (12.1%) needed reoperation. The mean hospitalization time was 11.69±6.516 days. Four patients (12.1%) died during the course of the study: three due to the postoperative complication of respiratory failure and one due to pulmonary thromboembolism. Pectoralis major muscle turnover flaps are an optimal technique in the treatment of post-CABG mediastinitis. In addition to leading to favorable therapeutic results, this flap is associated with minimal morbidity and mortality, as well as a short hospitalization time.

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

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.

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.

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

Reliable and valid assessment of competence in endoscopic ultrasonography and fine-needle aspiration for mediastinal staging of non-small cell lung cancer.

PubMed

Konge, L; Vilmann, P; Clementsen, P; Annema, J T; Ringsted, C

2012-10-01

Fine-needle aspiration (FNA) guided by endoscopic ultrasonography (EUS) is important in mediastinal staging of non-small cell lung cancer (NSCLC). Training standards and implementation strategies of this technique are currently under discussion. The aim of this study was to explore the reliability and validity of a newly developed EUS Assessment Tool (EUSAT) designed to measure competence in EUS - FNA for mediastinal staging of NSCLC. A total of 30 patients with proven or suspected NSCLC underwent EUS - FNA for mediastinal staging by three trainees and three experienced physicians. Their performances were assessed prospectively by three experts in EUS under direct observation and again 2 months later in a blinded fashion using digital video-recordings. Based on the assessments, intra-rater reliability, inter-rater reliability, and construct validity were explored. The intra-rater reliability was good (Cronbach's α = 0.80), but comparison of results based on direct observations and blinded video-recordings indicated a significant bias favoring consultants (P = 0.022). Inter-rater reliability was very good (Cronbach's α = 0.93). However, one rater assessing five procedures or two raters each assessing four procedures were necessary to secure a generalizability coefficient of 0.80. The assessment tool demonstrated construct validity by discriminating between trainees and experienced physicians (P = 0.034). Competency in mediastinal staging of NSCLC using EUS and EUS - FNA can be assessed in a reliable and valid way using the EUSAT assessment tool. Measuring and defining competency and training requirements could improve EUS quality and benefit patient care. © Georg Thieme Verlag KG Stuttgart · New York.

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.

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.

[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.

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.

Pneumomediastinum and Mediastinal Hematoma Secondary to Right Brachiocephalic Vein Thrombectomy Mimicking STEMI

PubMed Central

Shukla, Prem; Nivera, Noel

2017-01-01

A 50-year-old male with a history of hemodialysis dependent chronic kidney disease presented to our emergency department with acute midsternal crushing chest pain. Patient was diagnosed with acute anterolateral wall Myocardial Infraction due to the presence of corresponding ST segment elevations in EKG and underwent emergent cardiac catheterization which revealed normal patent coronaries without any disease. He continued to have chest pain for which CT of the chest was done which revealed pneumomediastinum with mediastinal hematoma, due to the recent attempted thrombectomy for thrombus in his right brachiocephalic vein. PMID:28804656

[Differential Diagnosis of Mediastinal and Hilar Lymphadenopathy with Focus on Occupational Diseases].

PubMed

Lux, H; Cavalcante, L Barreira; Baur, X

2018-06-01

Hilar and mediastinal lymphadenopathy may represent a diagnostic challenge in clinical practice. This article is intended to facilitate differential diagnosis by a systematic description of relevant pathologies, notably with occupational etiology. Clinical findings of relevant diseases, i. e. of tuberculosis, chronic beryllium disease, sarcoidosis, lung cancer, malignant lymphoma, Epstein-Barr virus infection, and histoplasmosis are compared.Case history, imaging and laboratory tests have important diagnostic impact. But also invasive methods can be necessary in order to exclude and prove malignancy, infection or autoimmune disease. © Georg Thieme Verlag KG Stuttgart · New York.

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

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

[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.

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.

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...

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...

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...

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...

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.

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

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

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

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

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

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.

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

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.

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.

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.

Oral calorie supplements for cystic fibrosis.

PubMed

Smyth, Rosalind L; Rayner, Oli

2017-05-04

Poor nutrition occurs frequently in people with cystic fibrosis and is associated with other adverse outcomes. Oral calorie supplements are used to increase total daily calorie intake and improve weight gain. However, they are expensive and there are concerns they may reduce the amount of food eaten and not improve overall energy intake. This is an update of a previously published review. To establish whether in people with cystic fibrosis, oral calorie supplements: increase daily calorie intake; and improve overall nutritional intake, nutritional indices, lung function, survival and quality of life. To assess adverse effects associated with using these supplements. We searched the Cochrane Cystic Fibrosis Trials Register comprising references from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. We contacted companies marketing oral calorie supplements.Last search: 18 October 2016. Randomised or quasi-randomised controlled trials comparing use of oral calorie supplements for at least one month to increase calorie intake with no specific intervention or additional nutritional advice in people with cystic fibrosis. We independently selected the included trials, assessed risk of bias and extracted data. We contacted the authors of included trials and obtained additional information for two trials. We identified 21 trials and included three, reporting results from 131 participants lasting between three months and one year. Two trials compared supplements to additional nutritional advice and one to no intervention. Two of the included trials recruited only children. In one trial the risk of bias was low across all domains, in a second trial the risk of bias was largely unclear and in the third mainly low. Blinding of participants was unclear in two of the trials. Also, in one trial the clinical condition of groups appeared to be unevenly balanced at baseline and in another trial there were

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

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.

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.

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

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.

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

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

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.

[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.

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

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

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

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.

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.

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.

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

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.

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.

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.

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

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.

Nearly total absence of pulmonary perfusion with corresponding technetium-99m MDP and gallium-67 uptake in a patient with mediastinal neuroblastoma

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

Garty, I.; Koren, A.; Moguilner, G.

1985-08-01

A case of unilateral nearly total hypoperfusion of the left lung in a 13-month-old girl is presented. The combination of the lung hypoperfusion and accumulation of the Tc-99m MDP and Ga-67 citrate in the same area suggested the preoperative diagnosis of mediastinal neuroblastoma. Explorative thoracotomy revealed the presence of a neuroblastoma compressing the left lung pedicle. The described scintigraphic appearance in the pediatric age group is suggested as typical of mediastinal neuroblastoma. This pathology should be included in the following gamuts in nuclear medicine: unilateral decrease or absent lung perfusion, unilateral diffuse chest uptake of Ga-67 citrate, and unilateral pulmonarymore » uptake in bone scintigraphy.« less

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

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

Recurrent Pneumonia due to Fibrosing Mediastinitis in a Teenage Girl: A Case Report with Long-Term Follow-Up

PubMed Central

Springer, Chaim; Wasser, Oren; Avital, Avraham; Koplewitz, Benjamin Z.

2018-01-01

A teenage girl was evaluated for recurrent right pneumonia. The evaluation revealed a calcified mediastinal mass that compressed the right intermediate and middle lobar bronchi, as well as the right pulmonary artery and veins. The clinical picture together with imaging studies and borderline positive serology testing suggested a diagnosis of fibrosing mediastinitis associated with histoplasmosis. This rare condition is characterized by the local proliferation of invasive fibrous tissue within the mediastinum due to a hyperimmune reaction to Histoplasma capsulatum. Antifungal and anti-inflammatory therapies are usually ineffective, and surgical intervention contains a high morbidity risk. Palliative surgery and stenting of the compressed airway have been suggested. In the past, the prognosis was thought to be poor, but recent studies demonstrate a more positive outcome. Our patient had been radiologically and functionally stable under follow-up for over thirteen years and has married and delivered two healthy children, both following an uneventful pregnancy. PMID:29744231

Occult Mediastinal Great Vessel Trauma: The Value of Aortography Performed During Angiographic Screening for Blunt Cervical Vascular Trauma

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

Ray, Charles E.; Bauer, Jason R.; Cothren, C. Clay

2005-05-15

Purpose. To determine the value of aortography in the assessment of occult aortic and great vessel injuries when routinely performed during screening angiography for blunt cerebrovascular injury (BCVI). Methods. One hundred and one consecutive patients who received both aortography and screening four-vessel angiography over 4 years were identified retrospectively. Angiograms for these patients were evaluated, and the incidence of occult mediastinal vascular injury was determined. Results. Of the 101 patients, 6 (6%) had angiographically documented traumatic aortic injuries. Of these 6 patients, one injury (17%) was unsuspected prior to angiography. Four of the 6 (67%) also had BCVI. One additionalmore » patient also had an injury to a branch of the subclavian artery. Conclusion. Routine aortography during screening angiography for BCVI is not warranted due to the low incidence (1%) of occult mediastinal arterial injury. However, in the setting of a BCVI screening study and no CT scan of the chest, aortography may be advantageous.« less

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

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.

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

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.

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.

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.

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

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

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.

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.

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.

Comparison of systematic mediastinal lymph node dissection versus systematic sampling for lung cancer staging and completeness of surgery.

PubMed

Wu, Nan; Yan, Shi; Lv, Chao; Feng, Yuan; Wang, Yuzhao; Zhang, Lijian; Yang, Yue

2011-12-01

This self-controlled prospective study was designed to investigate the efficacy of systematic sampling (SS), compared with systematic mediastinal lymph node dissection (SMLD), for pathologic staging and completeness of surgery. Over a period of 11 mo, 110 patients with lung cancer were enlisted and treated by pulmonary resection. Surgeons systematically sampled mediastinal lymph nodes prior to pulmonary resection, and after pulmonary resection SMLD was performed to each patient using Mountain's procedure [1]. After SMLD, pN status was classified as N0 in 57 cases, N1 in 27, and N2 in 26. SS detected 38.3% of pooled nodes and 37.6% of pooled positive nodes collected from SMLD. Pathologic diagnosis after SS was understaged in nine cases (8.2%) compared with staging after SMLD. However, surgery was incomplete in 24 cases (21.8%) if SMLD was not performed after sampling. Negative predictive value for SS was 86.8% on the right side, and 95.0% on the left. Three categories were generated according to pN status: negative nodes in SS and additional negative nodes from SMLD [S(-)D(-)], negative nodes in SS but additional positive nodes from SMLD [S(-)D(+)], and positive nodes in SS [S(+)D(+)]. cN2 (P=0.000) and CEA level (P=0.001) were correlated with pN status. There was significant overall survival difference between non-N2 group and N2 group (P=0.002). SMLD may harvest about three times of mediastinal lymph nodes compared with SS. SS is more likely to affect the completeness of surgery instead of underrating pathologic stage. Copyright © 2011 Elsevier Inc. All rights reserved.

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.

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

Fine-needle aspiration study of cystic papillary thyroid carcinoma: Rare cytological findings

PubMed Central

Mokhtari, Maral; Kumar, Perikala Vijayananda; Hayati, Kamran

2016-01-01

Background: Cystic papillary thyroid carcinoma (CPTC) is a variant of papillary carcinoma that has many mimickers in cytological grounds. Aim: To study the cytomorphologic features of CPTC and compare them to those of other cystic thyroid lesions using fine-needle aspiration cytology (FNAC). We also aimed to identify the cytomorphologic features that distinguish CPTC from other cystic thyroid lesions. Materials and Methods: Seventy-three cases of CPTC were included in the study. The cytomorphologic features of these cases were analyzed. The FNA smears of other thyroid lesions with cystic changes (300 colloid goiters, 290 adenomatoid nodules, 11 follicular neoplasms, and 9 hurtle cell neoplasm) were also studied. Results: The smears in CPTC revealed isolated follicular cells, small groups of cells with scalloped margins, cell swirls, small clusters with a cartwheel pattern, papillary clusters, intranuclear inclusions, nuclear grooves, sticky colloid, intracellular colloids, psammoma bodies, multinucleated giant cells, and foamy and hemosiderin laden macrophages. Small groups of cells with scalloped borders, cellular swirls, and small clusters with a cartwheel pattern were seen in CPTC, but not in other cystic lesions. Interestingly, mesothelial-like cells and hemophagocytic cells were seen in five and three cases of CPTC, respectively, but not in other cystic lesions. Conclusion: Mesothelial-like cells and hemophagocytic cells were observed in five and three cases of CPTC, respectively. Similar finding have not been previously reported in the literature. PMID:27756982

Cystic fibrosis identified by neonatal screening: incidence, genotype, and early natural history.

PubMed

Green, M R; Weaver, L T; Heeley, A F; Nicholson, K; Kuzemko, J A; Barton, D E; McMahon, R; Payne, S J; Austin, S; Yates, J R

1993-04-01

The incidence of cystic fibrosis over the last 10 years in East Anglia (a region of the United Kingdom with a population of 2.1 million) has halved. This has happened during the establishment of a neonatal screening programme, which has enabled early diagnosis, genetic counselling, and lately the option of prenatal diagnosis in subsequent pregnancies. One hundred and seven children were born with cystic fibrosis between 1981 and 1990, eight of whom were siblings. The Guthrie blood spots of 82 infants detected by neonatal immunoreactive trypsin screening between 1981 and 1990 were examined for the presence of the most common cystic fibrosis gene mutation (delta F508). It was present in 135 (82%) of the 164 cystic fibrosis genes analysed with 54 (66%) cases being homozygous and 27 (33%) heterozygous. Sixty nine per cent of infants were symptomatic at the time of diagnosis regardless of genotype. No association was found between the early clinical or biochemical features of the disease and homozygosity or heterozygosity for this mutation. Screening for cystic fibrosis using the blood immunoreactive trypsin assay alone remains an effective method of identifying infants with the disease soon after birth, thereby allowing early therapeutic intervention. Genetic counselling and prenatal diagnosis have contributed to a reduction in the number of children born with cystic fibrosis, but may not entirely explain the decreasing incidence of the disease.

Frequency of Undiagnosed Cystic Lung Disease in Patients With Sporadic Renal Angiomyolipomas

PubMed Central

Hartman, Thomas E.; Torres, Vicente E.; Decker, Paul A.

2012-01-01

Objective: The aim of this study was to assess the frequency of undiagnosed cystic lung lesions suggestive of pulmonary lymphangioleiomyomatosis (LAM) in patients who received a diagnosis of sporadic renal angiomyolipomas (AMLs). Methods: We conducted a retrospective review of CT scans of the chest or abdomen for cystic lung lesions on 176 adult patients who received a diagnosis of sporadic renal AML during a 10-year period, 1997 to 2006, and comparison with chest CT scans of 176 control subjects without renal AML but matched for age, sex, and smoking history. Patients presenting with suspected or known pulmonary LAM and those with underlying tuberous sclerosis were excluded. Results: Sporadic renal AML was diagnosed in 176 patients with a median age of 58 years (range, 20-91 years), the majority of whom were women (81.8%). Renal tumor was an incidental finding on imaging studies for most patients (90.3%). Nineteen patients (10.8%) had one or more cystic lung lesions and included nine patients (5.1%) with four or more cysts, all of whom were women. In comparison, eight control subjects (4.6%) had one to three cystic lung lesions and none of them exhibited four or more cysts. None of the patients with renal AML and cystic lung lesions, including six women with 10 or more cysts, had undergone an evaluation of their cystic lung disease. Conclusions: We conclude that a significant portion of women with sporadic renal AMLs exhibit cystic lung lesions suggestive of pulmonary LAM but may remain undiagnosed. Coexistence of pulmonary LAM should be considered in women incidentally found to have sporadic renal AMLs. PMID:21737494

The evaluation of selected insomnia predictors in adolescents and young adults with cystic fibrosis.

PubMed

Tomaszek, Lucyna; Cepuch, Grazyna; Pawlik, Lidia

2018-03-21

The purpose of the study was to assess the incidence of insomnia in adolescents and young adults with cystic fibrosis and its impact on the quality of life, and to examine whether demographic and clinical factors and negative emotional states are predictors of insomnia in these patients. The study was conducted among 95 cystic fibrosis patients aged 14-25 years. The study used a personal questionnaire survey, the Athens Insomnia Scale, the Cystic Fibrosis Quality of Life Questionnaire, the Hospital Anxiety and Depression Scale, and the Numeric Rating Scale. Insomnia was diagnosed in 38% of cystic fibrosis patients. In patients with insomnia, the level of anxiety (Me: 10 vs. 4; P=0.000) and depression (Me: 6.5 vs. 2; P=0.000) was significantly higher than in the good sleep quality group. The risk of insomnia increases as anxiety (OR: 4.31; 95% CI: 2.20 to 8.41) and depressive symptoms exacerbate (OR: 4.98; 95% CI: 1.84 to 13.43). Insomnia significantly worsens the quality of life in cystic fibrosis patients (ß =-0.5, P=0.000). Insomnia affects a large percentage of cystic fibrosis patients, and anxiety and depression are factors that increase the risk of insomnia. Insomnia decreases the quality of life in cystic fibrosis patients.

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

2018-02-01

Individuals with cystic fibrosis are at increased risk of colorectal cancer (CRC) compared with 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 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 if optimal screening strategies would change. Colonoscopy every 5 years, starting at an age of 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 this population is not clear. Using a Microsimulation Screening Analysis-Colon model, we found screening of patients with cystic fibrosis for CRC to be cost effective. Because of the higher risk of CRC in these patients, screening should start at an earlier age with a shorter screening interval. The findings of this study (especially those on FIT

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.

Mediastinal Bronchogenic Cyst With Acute Cardiac Dysfunction: Two-Stage Surgical Approach.

PubMed

Smail, Hassiba; Baste, Jean Marc; Melki, Jean; Peillon, Christophe

2015-10-01

We describe a two-stage surgical approach in a patient with cardiac dysfunction and hemodynamic compromise resulting from a massive and compressive mediastinal bronchogenic cyst. To drain this cyst, video-assisted mediastinoscopy was performed as an emergency procedure, which immediately improved the patient's cardiac function. Five days later and under video thoracoscopy, resection of the cyst margins was impossible because the cyst was tightly adherent to the left atrium. We performed deroofing of this cyst through a right thoracotomy. The patient had an uncomplicated postoperative recovery, and no recurrence was observed at the long-term follow-up visit. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Diagnosis of Adult Patients with Cystic Fibrosis.

PubMed

Nick, Jerry A; Nichols, David P

2016-03-01

The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis. Copyright © 2016 Elsevier Inc. All rights reserved.

Robot-assisted excision of cervical cystic hygroma through a retroauricular hairline approach: a case report.

PubMed

Lin, Frank Cheau-Feng; Yang, Tsung-Lin; Tung, Min-Che; Tsai, Stella Chin-Shaw

2016-06-09

Cystic hygroma is a rare benign abnormality of the lymphatic system generally occurring in young children less than 2 years old. The standard transcervical surgical treatment of cystic hygroma may often leave a permanent scar in the neck region. We report a case of cystic hygroma in a 19-month-old Asian baby girl successfully treated with robot-assisted excision through a hairline neck-lift approach. We present the use of the Yang's retractor as an instrumental advancement to this surgical approach. Treatment options for cystic hygroma may be surgical or nonsurgical. We report a case of cystic hygroma in a 19-month-old child successfully treated with robot-assisted excision through a small concealed retroauricular hairline approach. This is the first report in the medical literature of treating cystic hygroma with a minimally invasive robot-assisted excision via a small, concealed, hairline incision.

Recombinant Human DNase I Reduces the Viscosity of Cystic Fibrosis Sputum

NASA Astrophysics Data System (ADS)

Shak, Steven; Capon, Daniel J.; Hellmiss, Renate; Marsters, Scot A.; Baker, Carrie L.

1990-12-01

Respiratory distress and progressive lung destruction in cystic fibrosis can be attributed to bacterial persistence and the accumulation of viscous purulent secretions in the airways. More than 30 yr ago it was suggested that the large amounts of DNA in purulent secretions contribute to its viscosity and that bovine pancreatic DNase I could reduce the viscosity. To evaluate the potential clinical utility of recombinant human DNase I (rhDNase) in the treatment of cystic fibrosis, we have cloned, sequenced, and expressed rhDNase. Catalytic amounts of rhDNase greatly reduce the viscosity of purulent cystic fibrosis sputum, transforming it within minutes from a nonflowing viscous gel to a flowing liquid. The reduction in viscosity is associated with a decrease in size of DNA in the sputum. Inhalation of a rhDNase aerosol may be a simple direct approach that will help individuals with cystic fibrosis and other patients with pneumonia or bronchitis to clear their airways of purulent secretions.

Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum.

PubMed

Shak, S; Capon, D J; Hellmiss, R; Marsters, S A; Baker, C L

1990-12-01

Respiratory distress and progressive lung destruction in cystic fibrosis can be attributed to bacterial persistence and the accumulation of viscous purulent secretions in the airways. More than 30 yr ago it was suggested that the large amounts of DNA in purulent secretions contribute to its viscosity and that bovine pancreatic DNase I could reduce the viscosity. To evaluate the potential clinical utility of recombinant human DNase I (rhDNase) in the treatment of cystic fibrosis, we have cloned, sequenced, and expressed rhDNase. Catalytic amounts of rhDNase greatly reduce the viscosity of purulent cystic fibrosis sputum, transforming it within minutes from a nonflowing viscous gel to a flowing liquid. The reduction in viscosity is associated with a decrease in size of DNA in the sputum. Inhalation of a rhDNase aerosol may be a simple direct approach that will help individuals with cystic fibrosis and other patients with pneumonia or bronchitis to clear their airways of purulent secretions.

Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis.

PubMed

Elphick, Heather E; Southern, Kevin W

2012-06-13

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: 09 February 2012. 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. Two trials were identified by the searches; neither was judged eligible for inclusion in the review. No completed randomised controlled trials were

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

Maintenance of nutritional status in patients with cystic fibrosis: new and emerging therapies

PubMed Central

Kalnins, Daina; Wilschanski, Michael

2012-01-01

Poor clinical outcomes in cystic fibrosis are often associated with undernutrition. Normal growth and development should be achieved in cystic fibrosis, and nutritional counseling is paramount at all ages. Prevention and early detection of growth failure is the key to successful nutritional intervention. The advance in nutritional management is certainly one factor that has contributed to the improved survival in recent decades. This review outlines the major nutritional parameters in the management of the patient with cystic fibrosis, including recent advances in pancreatic enzyme replacement therapy and fat-soluble vitamin therapy. There are sections on complicated clinical situations which directly affect nutrition, for example, before and after lung transplantation, cystic fibrosis-related diabetes, and bone health. PMID:22787388

Rapid increase in cystic volume of an anaplastic astrocytoma misdiagnosed as neurocysticercosis: A case report

PubMed Central

Li, Hong-Jiang; Han, Hong-Xiu; Feng, Dong-Fu

2016-01-01

Reports describing a rapid increase in the cystic volume of anaplastic astrocytoma (AA) in a short time frame are rare. The present study reports the case of a 68-year-old male who was admitted to the No. 9 People's Hospital, Shanghai Jiaotong University School of Medicine (Shanghai, China), with a small cystic brain lesion and positive immunological testing for cysticercosis. Head magnetic resonance imaging (MRI) showed a cystic lesion, 6 mm in diameter, in the left frontal lobe. Neurocysticercosis was suspected and the patient was treated with a clinical trial of albendazole and steroids. A period of 25 days later, the patient's condition had deteriorated, and MRI revealed a cystic lesion in the left frontal lobe; thereafter, the cystic lesion was removed and a diagnosis of AA was established. The tumor was soft, ivory white and gelatinous due to myxoid degeneration. In this case, tumor-related angiogenesis and microvascular extravasation (blood-brain barrier disruption) may have been the main cause of the rapid increase in the cystic volume in such a short time frame. The similarity of the glioma and cysticercus antigens may have been the cause of the positive reactions in the cystic fluid. The present study reports the rare occurrence of a rapid increase of cystic volume and potential diagnostic difficulties. PMID:27698865

Ursodeoxycholic acid for cystic fibrosis-related liver disease.

PubMed

Cheng, Katharine; Ashby, Deborah; Smyth, Rosalind L

2014-12-15

Abnormal biliary secretion leads to the thickening of bile and the formation of plugs within the bile ducts; the consequent obstruction and abnormal bile flow ultimately results in the development of cystic fibrosis-related liver disease. This condition peaks in adolescence with up to 20% of adolescents with cystic fibrosis developing chronic liver disease. Early changes in the liver may ultimately result in end-stage liver disease with people needing transplantation. One therapeutic option currently used is ursodeoxycholic acid. To analyse evidence that ursodeoxycholic acid improves indices of liver function, reduces the risk of developing chronic liver disease and improves outcomes in general in cystic fibrosis. We searched the Cochrane CF and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. We also contacted drug companies.Date of the most recent search of the Group's trials register: 29 May 2014. Randomised controlled trials of the use of ursodeoxycholic acid for at least three months compared with placebo or no additional treatment in people with cystic fibrosis. Two authors independently assessed trial eligibility and quality. Ten trials have been identified, of which three trials involving 118 participants were included; the dose of ursodeoxycholic acid ranged from 10 to 20 mg/kg/day for up to 12 months. The complex design used in two trials meant that data could only be analysed for subsets of participants. There was no significant difference in weight change, mean difference -0.90 kg (95% confidence interval -1.94 to 0.14) based on 30 participants from two trials. Improvement in biliary excretion was reported in only one trial and no significant change after treatment was shown. There were no data available for analysis for long-term outcomes such as death or need for liver transplantation. There are few

Thoroughness of Mediastinal Staging in Stage IIIA Non-Small Cell Lung Cancer

PubMed Central

Vest, Michael T.; Tanoue, Lynn; Soulos, Pamela R.; Kim, Anthony W.; Detterbeck, Frank; Morgensztern, Daniel; Gross, Cary P.

2011-01-01

Introduction Guidelines recommend that patients with clinical stage IIIA non-small cell lung cancer (NSCLC) undergo histologic confirmation of pathologic lymph nodes. Studies have suggested that invasive mediastinal staging is underutilized, though practice patterns have not been rigorously evaluated. Methods We used the Surveillance, Epidemiology, and End Results-Medicare database to identify patients with stage IIIA NSCLC diagnosed from 1998 through 2005. Invasive staging and use of positron emission tomography (PET) scanning were assessed using Medicare claims. Multivariable logistic regression was used to identify patient characteristics associated with use of invasive staging. Results Of 7583 stage IIIA NSCLC patients, 1678 (22%) underwent invasive staging. Patients who received curative intent cancer treatment were more likely to undergo invasive staging than patients who did not receive cancer specific therapy (30% vs. 9.8%, adjusted odds ratio [OR} 3.31, 95% CI 2.78–3.95). The oldest patients (age 85–94) were less likely to receive invasive staging than the youngest ((age 67–69) (27.6 % vs. 11.9%, OR 0.46, 95% CI 0.34–0.61)). Sex, marital status, income and race were not associated with the use of the invasive staging. The use of invasive staging was stable throughout the study period, despite an increase in the use of PET scanning from less than 10% of patients prior to 2000 to almost 70% in 2005. Conclusion Nearly 80% of Medicare beneficiaries with stage IIIA NSCLC do not receive guideline adherent mediastinal staging; this failure cannot be entirely explained by patient factors or a reliance on PET imaging. Incentives to encourage use of invasive staging may improve care. PMID:22134069

The structure of tracheobronchial mucins from cystic fibrosis and control patients.

PubMed

Gupta, R; Jentoft, N

1992-02-15

Tracheobronchial mucin samples from control and cystic fibrosis patients were purified by gel filtration chromatography on Sephacryl S-1000 and by density gradient centrifugation. Normal secretions contained high molecular weight (approximately 10(7] mucins, whereas the cystic fibrosis secretions contained relatively small amounts of high molecular weight mucin together with larger quantities of lower molecular weight mucin fragments. These probably represent products of protease digestion. Reducing the disulfide bonds in either the control or cystic fibrosis high molecular weight mucin fractions released subunits of approximately 2000 kDa. Treating these subunits with trypsin released glycopeptides of 300 kDa. Trypsin treatment of unreduced mucin also released fragments of 2000 kDa that could be converted into 300-kDa glycopeptides upon disulfide bond reduction. Thus, protease-susceptible linkages within these mucins must be cross-linked by disulfide bonds so that the full effects of proteolytic degradation of mucins remain cryptic until disulfide bonds are reduced. Since various combinations of protease treatment and disulfide bond reduction release either 2000- or 300-kDa fragments, these fragments must represent important elements of mucin structure. The high molecular weight fractions of cystic fibrosis mucins appear to be indistinguishable from control mucins. Their amino acid compositions are the same, and various combinations of disulfide bond reduction and protease treatment release products of identical size and amino acid composition. Sulfate and carbohydrate compositions did vary considerably from sample to sample, but the limited number of samples tested did not demonstrate a cystic fibrosis-specific pattern. Thus, tracheobronchial mucins from cystic fibrosis and control patients are very similar, and both share the same generalized structure previously determined for salivary, cervical, and intestinal mucins.

[Measurement of nasal transepithelial potential difference: a diagnostic test for cystic fibrosis].

PubMed

Charfi, M R; Matran, R; Regnard, J; Lockhart, A

1996-01-01

Measurement of nasal transepithelial potential difference allows the exploration of transepithelial ionic transports in vivo. Cystic fibrosis is an interesting indication of this test. Indeed, this disease is characterized by a chloride and water secretion deficit across respiratory epithelium. We have measured nasal potential in 8 healthy volunteers. Measurements were repeated 3 times a day, during 3 days for each subject. The reproducibility of the data was analysed with factorial variance model. The mean nasal potential in the healthy volunteers group and in 10 patients with cystic fibrosis was compared. In the cystic fibrosis group, the nasal potential was measured 3 times with a 2 mn-interval between the measurements. No significant variation of the nasal potential values was found from day to day or in the same day from one measurement to another. Mean value was -19 +/- 3.5 mv in normal subjects and -42.6 +/- 5.1 mv in cystic fibrosis patients. We conclude that nasal potential measurement is an easy and reproducible test that might be a complementary tool routinely used along with the classical tests in the diagnosis of cystic fibrosis.

Death after cessation of treatment by cystic fibrosis patients: An international survey of clinicians.

PubMed

Pisaturo, Marisa; Deppen, Alain; Rochat, Isabelle; Robinson, Walter M; Hafen, Gaudenz M

2017-01-01

Little is known about cystic fibrosis patients, who are not considered to be terminally ill, and who die after voluntary cessation of treatment. This study was undertaken to provide an international snapshot of this issue. An online survey was distributed across three continents. Distribution to the medical directors of the cystic fibrosis centres affiliated with the US Cystic Fibrosis Foundation, Cystic Fibrosis Australia (inclusion of New Zealand) and to every clinician member of the European Cystic Fibrosis Society. More than 200 cystic fibrosis patients not considered to be terminally ill and, who voluntarily ceased treatment, were reported by the clinicians surveyed. Detailed data were reported in 102 patients (4 children, 25 adolescents and 73 adults). Only one child, six adolescents and one adult were judged by clinicians not to be competent to make the decision to stop treatment. Time-consuming and low immediate-impact therapies, such as respiratory physiotherapy, were most frequently discontinued. Resignation was the main reported reason for discontinuing treatment, followed by reactive depression and lack of familial support. A total of 69% of the patients received palliative care and 72% died in the 6 months following cessation of treatment. Death of cystic fibrosis patients, not considered to be terminally ill, is reported in Europe, the United States and Australia due to voluntary cessation of treatment.

Gallbladder and cystic duct absence. An infrequent malformation in laparoscopic surgery.

PubMed

Cabajo Caballero, M A; Martin del Olmo, J C; Blanco Alvarez, J I; Atienza Sanchez, R

1997-05-01

Gallbladder absence is an infrequent anomaly normally accompanied by lack of the cystic duct. Of unknown etiology, in general it is accepted to be a congenital malformation. A male patient (age 59) diagnosed with nonfunctional symptomatic scleroatrophic gallbladder by echography and ERCP was operated on using a laparoscopic approach. Gallbladder and cystic absence was diagnosed during the procedure. A new case of gallbladder and cystic duct absence, diagnosed by laparoscopy, is presented. This type of extrahepatic bile duct malformation is quite rare, but it must be taken into account due to the generalization of laparoscopic surgery in biliar pathology because of the high risk of serious lesions to the hepatocholedochal system.

Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis.

PubMed

Elphick, Heather E; Southern, Kevin W

2016-11-08

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. This is an update of a previously published review. 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: 29 September 2016. 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

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

US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis.

PubMed

Floto, R Andres; 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. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Cutaneous metastatic tuberculous abcess in a patient with cervico-mediastinal lymphatic tuberculosis.

PubMed

Dekeyzer, S; Moerman, F; Callens, S; Vandekerckhove, L

2013-01-01

Metastatic tuberculous abcess or tuberculous gumma is a rare form of cutaneous tuberculosis resulting from haematogenous spread from a non-cutaneous tuberculous focus. A 26-year old patient of Pakistani origin presented at our clinic with an abcess on his right thigh that had slowly grown over a period of two months to a total size of 30 cm. Based on clinical findings, microbiology, CT thigh and CT chest, our patient was diagnosed with a tuberculous abcess and cervico-mediastinal tuberculous lymphadenitis. Antituberculosis drugs were initiated. Cutaneous tuberculosis should be included in the differential diagnosis of chronic cutaneous abcesses, especially in patients from tuberculosis endemic nations.

[Vascular neoplasms of the mediastinum].

PubMed

Biriukov, Iu V; Charnetskiĭ, R I; Godzhello, E A

1991-04-01

During a period of 42 years the authors had 44 patients with various vascular neoplasms of the mediastinum--angiomas. Experience in the treatment of 6 of them is discussed in B. V. Petrovsky's monograph Surgery of the Mediastinum (1960). The other 38 cases were distributed as follows: hemangiomas 28 (14 mature and 14 immature) and lymphangiomas 10 (all mature). The patients' ages ranged from 11 to 63 years, the duration of the anamnesis from 4 weeks to 40 years. Mediastinal angiomas are mainly encountered in children and young people, with equal frequency among females and males. Their clinico-radiological picture is diverse and unspecific, the diagnosis is established with difficulty. Surgery is the principal method of treatment. Radical removal of the tumor must be attempted. Radiologically-guided endovascular occlusion of the vessel supplying the tumor is possible in some cases. The late results of surgical treatment are good in cases of mature angiomas and depend on the radicalism of the operation in immature forms.

The Swiss Cystic Fibrosis Infant Lung Development (SCILD) cohort.

PubMed

Korten, Insa; Kieninger, Elisabeth; Yammine, Sophie; Regamey, Nicolas; Nyilas, Sylvia; Ramsey, Kathryn; Casaulta, Carmen; Latzin, Philipp; For The Scild Study Group

2018-04-26

The Swiss Cystic Fibrosis Infant Lung Development (SCILD) cohort is a prospective birth cohort study investigating the initiating events of cystic fibrosis lung disease during infancy, and their influence on the trajectory of disease progression throughout early childhood. Infants with cystic fibrosis are recruited throughout Switzerland after diagnosis by new-born screening. It is the first European population-based prospective cohort study of infants with cystic fibrosis taking advantage of a nationwide new-born screening programme. The study was established in 2011 and recruitment is ongoing. The cohort study is currently divided into three study phases (phase 1: diagnosis to age 1 year; phase 2: age 1 to 3 years; and phase 3: age 3 to 6 years). Study participants have weekly telephone interviews, weekly anterior nasal swab collection and two study visits in the first year of life. They also complete follow-up study visits at 3 and 6 years of age. Data for this study are derived from questionnaires, lung function measurements, telephone interviews, nasal swab material and magnetic resonance imaging. To date, 70 infants have been recruited into the study and 56 have completed phase 1, including a baseline study visit at 6 weeks of age, weekly surveillance and a study visit at one year of age. More than 2500 data points on respiratory health and almost 2000 nasal samples have been collected. Phases 2 and 3 will commence in 2018. The dataset of the SCILD cohort combines lung function data, the collection of environmental and sociodemographic factors, documentation of respiratory symptoms, and microbiological analyses. The design not only allows tracking of the cystic fibrosis lung disease independent of clinical status, but also surveillance of early disease prior to severe clinical symptoms. This cohort profile provides details on the study design and summarizes the first published results of the SCILD cohort.

[Cystic renal neoplasms. New entities and molecular findings].

PubMed

Moch, H

2010-10-01

Renal neoplasms with dominant cysts represent a broad spectrum of known as well as novel renal tumor entities. Established renal tumors with dominant cysts include cystic nephroma, mixed epithelial and stromal tumor, synovial sarcoma and multilocular cystic renal cancer (WHO classification 2004). Novel tumor types have recently been reported, which are also characterized by marked cyst formation. Examples are tubulocystic renal cancer and renal cancer in end-stage renal disease. These tumors are very likely to be included in a future WHO classification due to their characteristic phenotype and molecular features. Cysts and clear cell renal cell carcinoma frequently coexist in the kidneys of patients with von Hippel-Lindau disease. Cysts are also a component of many sporadic clear cell renal cell carcinomas. Multilocular cystic renal cell carcinoma is composed almost exclusively of cysts and is regarded as a specific subtype of clear cell renal cancer. Recent molecular findings suggest that clear cell renal cancer may develop via a cyst-dependent mechanism in von Hippel-Lindau syndrome as well as via cyst-independent molecular pathways in sporadic clear cell renal cancer.

Penetration of ciprofloxacin into heart valves, myocardium, mediastinal fat, and sternal bone marrow in humans.

PubMed Central

Mertes, P M; Voiriot, P; Dopff, C; Scholl, H; Clavey, M; Villemot, J P; Canton, P; Dureux, J B

1990-01-01

The penetration of ciprofloxacin into heart tissue (valve and myocardium), mediastinal fat, and sternal bone marrow was the object of a prospective nonrandomized study involving 36 patients undergoing mitral and/or aortic valve replacement. Patients were divided into two groups of 18. Group 1 patients were administered a single 400-mg intravenous dose of ciprofloxacin over a 1-h period. Group 2 patients received a 750-mg dose of ciprofloxacin orally every 12 h over the 48-h period preceding surgery. In this group, the last dose of ciprofloxacin consisted of an intravenous infusion of 400 mg. Concentrations of ciprofloxacin in plasma and tissue were assayed by high-performance liquid chromatography. Peak and trough levels in plasma were, respectively, 6.19 +/- 1.73 and 0.54 +/- 0.25 micrograms/ml in group 1 patients and 11.59 +/- 3.95 and 0.89 +/- 0.57 micrograms/ml in group 2 patients. Levels of ciprofloxacin in plasma remained significantly higher in group 2 than in group 1 until 12 h postinfusion (P less than 0.05). Concentrations of ciprofloxacin in heart valves and myocardia rose rapidly by 1 h postinfusion and remained greater than the MICs for usually susceptible pathogens for at least 5 h. Peak concentrations in myocardia were achieved by hour 1 and were 31.6 +/- 25.0 micrograms/g for group 1 and 21.8 +/- 13.0 micrograms/g for group 2. Peak concentrations in heart valves, achieved between hours 1 and 3, were 5.8 +/- 3.2 and 8.3 +/- 3.1 micrograms/g for groups 1 and 2, respectively. In both groups, peak concentrations in mediastinal fat were lower and achieved later. These were 3.1 +/- 3.8 micrograms/g in group 1 and 2.0 +/- 1.8 micrograms/gram in group 2 and were achieved between hours 3 and 5 and hours 1 and 3, respectively. In conclusion, the good diffusion of ciprofloxacin into heart tissue warrants its use for the treatment of bacterial endocarditis. On the other hand, low and delayed concentrations in mediastinal fat could limit its value as an

Adenoid cystic carcinoma of the buccal mucosa: A case report and review of the literature

PubMed Central

Ajila, Vidya; Hegde, Shruthi; Nair, Gopakumar R; Babu, Subhas G

2012-01-01

Adenoid cystic carcinomas are deceptive malignancies that show slow growth and local invasion with recurrences seen many years after diagnosis. Upto 50% of these tumors occur in the intraoral minor salivary glands usually in the hard palate. Buccal mucosal tumors are relatively rare. We determined the incidence of buccal mucosal adenoid cystic carcinoma by reviewing the number of reported cases in the literature. This is the first article to analyze the occurrence of adenoid cystic carcinomas in the buccal mucosa through a review of 41 articles. Our review revealed 178 buccal mucosal adenoid cystic carcinomas among a total of 2,280 reported cases. We present a case of adenoid cystic carcinoma occurring in the left buccal mucosa of a 45-year-old female. PMID:23559934

Acoustic radiation force impulse (ARFI) ultrasound imaging of pancreatic cystic lesions.

PubMed

D'Onofrio, M; Gallotti, A; Salvia, R; Capelli, P; Mucelli, R Pozzi

2011-11-01

To evaluate the ARFI ultrasound imaging with Virtual Touch tissue quantification in studying pancreatic cystic lesions, compared with phantom fluid models. Different phantom fluids at different viscosity or density (water, iodinate contrast agent, and oil) were evaluated by two independent operators. From September to December 2008, 23 pancreatic cystic lesions were prospectively studied. All lesions were pathologically confirmed. Non-numerical values on water and numerical values on other phantoms were obtained. Inter-observer evaluation revealed a perfect correlation (rs=1.00; p<0.0001) between all measurements achieved by both operators per each balloon and fluid. Among the pancreatic cystic lesions, 14 mucinous cystadenomas, 4 pseudocysts, 3 intraductal papillary-mucinous neoplasms and 2 serous cystadenomas were studied. The values obtained ranged from XXXX/0-4,85 m/s in mucinous cystadenomas, from XXXX/0-3,11 m/s in pseudocysts, from XXXX/0-4,57 m/s in intraductal papillary-mucinous neoplasms. In serous cystadenomas all values measured were XXXX/0m/s. Diagnostic accuracy in benign and non-benign differentiation of pancreatic cystic lesions was 78%. Virtual Touch tissue quantification can be applied in the analysis of fluids and is potentially able to differentiate more complex (mucinous) from simple (serous) content in studying pancreatic cystic lesions. Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.

∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis.

PubMed

Pankow, Sandra; Bamberger, Casimir; Calzolari, Diego; Martínez-Bartolomé, Salvador; Lavallée-Adam, Mathieu; Balch, William E; Yates, John R

2015-12-24

Deletion of phenylalanine 508 of the cystic fibrosis transmembrane conductance regulator (∆F508 CFTR) is the major cause of cystic fibrosis, one of the most common inherited childhood diseases. The mutated CFTR anion channel is not fully glycosylated and shows minimal activity in bronchial epithelial cells of patients with cystic fibrosis. Low temperature or inhibition of histone deacetylases can partly rescue ∆F508 CFTR cellular processing defects and function. A favourable change of ∆F508 CFTR protein-protein interactions was proposed as a mechanism of rescue; however, CFTR interactome dynamics during temperature shift and inhibition of histone deacetylases are unknown. Here we report the first comprehensive analysis of the CFTR and ∆F508 CFTR interactome and its dynamics during temperature shift and inhibition of histone deacetylases. By using a novel deep proteomic analysis method, we identify 638 individual high-confidence CFTR interactors and discover a ∆F508 deletion-specific interactome, which is extensively remodelled upon rescue. Detailed analysis of the interactome remodelling identifies key novel interactors, whose loss promote ∆F508 CFTR channel function in primary cystic fibrosis epithelia or which are critical for CFTR biogenesis. Our results demonstrate that global remodelling of ∆F508 CFTR interactions is crucial for rescue, and provide comprehensive insight into the molecular disease mechanisms of cystic fibrosis caused by deletion of F508.

Telomerase activity is a useful marker to distinguish malignant pancreatic cystic tumors from benign neoplasms and pseudocysts.

PubMed

Yeh, T S; Cheng, A J; Chen, T C; Jan, Y Y; Hwang, T L; Jeng, L B; Chen, M F; Wang, T C

1999-12-01

Pancreatic serous cystadenoma, mucinous cystic neoplasms, ductal adenocarcinoma with cystic change, and pseudocysts are a spectrum of pancreatic cystic lesions. Their management strategy and prognosis are extremely diverse. Imaging study, cytology, and analysis of the tumor markers of cyst fluid are not always reliable in differentiation of these disease entities. Fifteen patients with pancreatic cystic neoplasms (including six mucinous cystadenocarcinomas, two mucinous cystic neoplasms with borderline malignancy, two mucinous cystadenomas, and five serous cystadenomas), 4 patients with pancreatic ductal adenocarcinomas with cystic change, and 10 patients with pseudocysts were studied. Echo-guided or computed tomography-guided biopsies of pancreatic cystic lesions and their normal counterparts were conducted on all patients prior to operation or other management. The specimens were assayed for telomerase activity by using TRAP (telomere repeat amplification protocol). The level of telomerase activity in each specimen was semiquantitated as strong, moderate, weak, and none. The final diagnoses were made from histopathological examination of surgically resected or biopsied specimens. The efficacy of telomerase activity as a tumor marker to predict malignancy of pancreatic cystic lesions was evaluated. Three of the four pancreatic ductal adenocarcinomas with cystic change had strong or moderate telomerase activity; four of the six mucinous cystadenocarcinomas had moderate or weak telomerase activity; one of the two mucinous cystadenomas with borderline malignancy had weak telomerase activity; and none of their normal counterparts had detectable telomerase activity. In contrast, none of the two mucinous cystadenomas, five serous cystadenomas, and 10 pseudocysts had detectable telomerase activity. Based on these results, the sensitivity of telomerase activity for prediction of malignancy or premalignancy of pancreatic cystic lesions was 67%, the specificity was 100

Loss of Cystic Fibrosis Transmembrane Regulator Impairs Intestinal Oxalate Secretion.

PubMed

Knauf, Felix; Thomson, Robert B; Heneghan, John F; Jiang, Zhirong; Adebamiro, Adedotun; Thomson, Claire L; Barone, Christina; Asplin, John R; Egan, Marie E; Alper, Seth L; Aronson, Peter S

2017-01-01

Patients with cystic fibrosis have an increased incidence of hyperoxaluria and calcium oxalate nephrolithiasis. Net intestinal absorption of dietary oxalate results from passive paracellular oxalate absorption as modified by oxalate back secretion mediated by the SLC26A6 oxalate transporter. We used mice deficient in the cystic fibrosis transmembrane conductance regulator gene (Cftr) to test the hypothesis that SLC26A6-mediated oxalate secretion is defective in cystic fibrosis. We mounted isolated intestinal tissue from C57BL/6 (wild-type) and Cftr -/- mice in Ussing chambers and measured transcellular secretion of [ 14 C]oxalate. Intestinal tissue isolated from Cftr -/- mice exhibited significantly less transcellular oxalate secretion than intestinal tissue of wild-type mice. However, glucose absorption, another representative intestinal transport process, did not differ in Cftr -/- tissue. Compared with wild-type mice, Cftr -/- mice showed reduced expression of SLC26A6 in duodenum by immunofluorescence and Western blot analysis. Furthermore, coexpression of CFTR stimulated SLC26A6-mediated Cl - -oxalate exchange in Xenopus oocytes. In association with the profound defect in intestinal oxalate secretion, Cftr -/- mice had serum and urine oxalate levels 2.5-fold greater than those of wild-type mice. We conclude that defective intestinal oxalate secretion mediated by SLC26A6 may contribute to the hyperoxaluria observed in this mouse model of cystic fibrosis. Future studies are needed to address whether similar mechanisms contribute to the increased risk for calcium oxalate stone formation observed in patients with cystic fibrosis. Copyright © 2016 by the American Society of Nephrology.

Na and K Dependence of the Na/K Pump in Cystic Fibrosis Fibroblasts

NASA Astrophysics Data System (ADS)

Reznik, Vivian M.; Schneider, Jerry A.; Mendoza, Stanley A.

1981-11-01

The Na and K dependence of the Na/K pump was measured in skin fibroblasts from patients with cystic fibrosis and age/sex-matched controls. Under basal conditions, there was no difference between control and cystic fibrosis cells in protein per cell, intracellular Na and K content, or Na/K pump activity (measured as ouabain-sensitive 86Rb uptake). There was no difference in the Na dependence of the Na/K pump between cystic fibrosis cells and control cells. In cells from patients with cystic fibrosis, the Na/K pump had a significantly lower affinity for K (Km = 1.6 mM) when compared to normals (Km = 0.9 mM). This difference was demonstrated by using two independent experimental designs.

Doxycycline improves clinical outcomes during cystic fibrosis exacerbations.

PubMed

Xu, Xin; Abdalla, Tarek; Bratcher, Preston E; Jackson, Patricia L; Sabbatini, Gina; Wells, J Michael; Lou, Xiang-Yang; Quinn, Rebecca; Blalock, J Edwin; Clancy, J P; Gaggar, Amit

2017-04-01

Matrix metalloprotease-9 (MMP-9) plays a role in progression of cystic fibrosis, and doxycycline can reduce MMP-9 in vitro Here, we explore the effect of doxycycline during cystic fibrosis exacerbation treatment on MMP-9 related readouts and clinical end-points.This randomised, double-blind, placebo-controlled study enrolled hospitalised patients with cystic fibrosis undergoing exacerbation. In total, 20 participants were given doxycycline and 19 participants were given placebo over an 8-day period during hospitalisation. Biospecimens were collected at the beginning and the end of the study period. Primary end-points were total MMP-9 levels in the sputum and safety/tolerability. Secondary end-points included change in lung function, time to next exacerbation, and markers of MMP-9-related protease activity (active MMP-9 and TIMP-1). Nonparametric testing was used for within-group and between-group analyses.Doxycycline was well tolerated, with no treatment discontinuations or serious adverse events. Doxycycline reduced total sputum MMP-9 levels by 63.2% (p<0.05), and was also associated with a 56.5% reduction in active MMP-9 levels (p<0.05), a 1.6-fold increase in sputum TIMP-1 (p<0.05), improvement in forced expiratory volume in 1 s (p<0.05), and an increase in time to next exacerbation (p<0.01).Adjunctive use of doxycycline improved dysregulated MMP-9 levels in sputum, along with biomarkers consistent with a reduced proteolytic pulmonary environment. Improvement in clinical outcome measures suggests an important therapeutic benefit of doxycycline for individuals with cystic fibrosis. Copyright ©ERS 2017.

Variants in mannose‐binding lectin and tumour necrosis factor α affect survival in cystic fibrosis

PubMed Central

Buranawuti, Kitti; Boyle, Michael P; Cheng, Suzanne; Steiner, Lori L; McDougal, Kathryn; Fallin, M Daniele; Merlo, Christian; Zeitlin, Pamela L; Rosenstein, Beryl J; Mogayzel, Peter J; Wang, Xinjing; Cutting, Garry R

2007-01-01

Background Patients with cystic fibrosis with the same mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene differ widely in survival suggesting other factors have a substantial role in mortality. Objective To determine if the genotype distribution of variants in three putative cystic fibrosis modifier genes (tumour necrosis factor α (TNFα), transforming growth factor β1 (TGFβ1) or mannose‐binding lectin (MBL2)) differed among patients with cystic fibrosis grouped according to age and survival status. Methods Genotypes of four variants (TNFα‐238, TNFα‐308, TGFβ1‐509 and MBL2 O) were determined in three groups of Caucasians from a single medical centre: 101 children with cystic fibrosis (aged <17 years; mean age 9.4 years), 115 adults with cystic fibrosis (aged ⩾17 years; mean age 30.8 years) and 38 non‐surviving adults with cystic fibrosis (21 deceased and 17 lung transplant after 17 years of age). Genotypes of 127 healthy Caucasians in the same geographical region were used as controls. Kaplan–Meier and Cox hazard regression were used to evaluate the genotype effect on cumulative survival. Results Genotype frequencies among adults and children with cystic fibrosis differed for TNFα‐238 (G/G vs G/A; p = 0.022) and MBL2 (A/A vs O/O; p = 0.016). When adults with cystic fibrosis were compared to non‐surviving adults with cystic fibrosis, genotype frequencies of both genes differed (TNFα‐238G/G vs G/A; p =  0.0015 and MBL2: A/A vs O/O; p = 0.009). The hazard ratio for TNFα‐238G/G vs G/A was 0.25 (95% CI 0.06 to 1.0, p = 0.04) and for MBL2 O/O vs A/A or A/O was 2.5 (95% CI 1.3 to 4.9, p = 0.007). Conclusions TNFα‐238 G/A and MBL2 O/O genotypes appear to be genetic modifiers of survival of cystic fibrosis. PMID:17158822

Cervical lymph node metastasis in adenoid cystic carcinoma of the major salivary glands.

PubMed

2017-02-01

To verify the prevalence of cervical lymph node metastasis in adenoid cystic carcinoma of major salivary glands, and to establish recommendations for elective neck treatment. A search was conducted of the US National Library of Medicine database. Appropriate articles were selected from the abstracts, and the original publications were obtained to extract data. Among 483 cases of major salivary gland adenoid cystic carcinoma, a total of 90 (18.6 per cent) had cervical metastasis. The prevalence of positive nodes from adenoid cystic carcinoma was 14.5 per cent for parotid gland, 22.5 per cent for submandibular gland and 24.7 per cent for sublingual gland. Cervical lymph node metastasis occurred more frequently in patients with primary tumour stage T3-4 adenoid cystic carcinoma, and was usually located in levels II and III in the neck. Adenoid cystic carcinoma of the major salivary glands is associated with a significant prevalence of cervical node metastasis, and elective neck treatment is indicated for T3 and T4 primary tumours, as well as tumours with other histological risk factors.

Macrolide antibiotics for cystic fibrosis.

PubMed

Southern, Kevin W; Barker, Pierre M; Solis-Moya, Arturo; Patel, Latifa

2012-11-14

Macrolide antibiotics may have a modifying role in diseases which involve airway infection and inflammation, like cystic fibrosis. To test the hypotheses that, in people with cystic fibrosis, macrolide antibiotics: 1. improve clinical status compared to placebo or another antibiotic; 2. do not have unacceptable adverse effects. If benefit was demonstrated, we aimed to assess the optimal type, dose and duration of macrolide therapy. 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.We contacted investigators known to work in the field, previous authors and pharmaceutical companies manufacturing macrolide antibiotics for unpublished or follow-up data (May 2010).Latest search of the Group's Cystic Fibrosis Trials Register: 29 February 2012. Randomised controlled trials of macrolide antibiotics compared to: placebo; another class of antibiotic; another macrolide antibiotic; or the same macrolide antibiotic at a different dose. Two authors independently extracted data and assessed risk of bias. Seven groups were contacted and provided additional data which were incorporated into the review. Ten of 31 studies identified were included (959 patients). Five studies with a low risk of bias examined azithromycin versus placebo and demonstrated consistent improvement in forced expiratory volume in one second over six months (mean difference at six months 3.97% (95% confidence interval 1.74% to 6.19%; n = 549, from four studies)). Patients treated with azithromycin were approximately twice as likely to be free of pulmonary exacerbation at six months, odds ratio 1.96 (95% confidence interval 1.15 to 3.33). With respect to secondary outcomes, there was a significant reduction in need for oral antibiotics and greater weight gain in those taking azithromycin. Adverse events were uncommon

Intranasal Live Influenza Vaccine Priming Elicits Localized B Cell Responses in Mediastinal Lymph Nodes.

PubMed

Jegaskanda, Sinthujan; Mason, Rosemarie D; Andrews, Sarah F; Wheatley, Adam K; Zhang, Ruijun; Reynoso, Glennys V; Ambrozak, David R; Santos, Celia P; Luke, Catherine J; Matsuoka, Yumiko; Brenchley, Jason M; Hickman, Heather D; Talaat, Kawsar R; Permar, Sallie R; Liao, Hua-Xin; Yewdell, Jonathan W; Koup, Richard A; Roederer, Mario; McDermott, Adrian B; Subbarao, Kanta

2018-05-01

Pandemic live attenuated influenza vaccines (pLAIV) prime subjects for a robust neutralizing antibody response upon subsequent administration of a pandemic inactivated subunit vaccine (pISV). However, a difference was not detected in H5-specific memory B cells in the peripheral blood between pLAIV-primed and unprimed subjects prior to pISV boost. To investigate the mechanism underlying pLAIV priming, we vaccinated groups of 12 African green monkeys (AGMs) with H5N1 pISV or pLAIV alone or H5N1 pLAIV followed by pISV and examined immunity systemically and in local draining lymph nodes (LN). The AGM model recapitulated the serologic observations from clinical studies. Interestingly, H5N1 pLAIV induced robust germinal center B cell responses in the mediastinal LN (MLN). Subsequent boosting with H5N1 pISV drove increases in H5-specific B cells in the axillary LN, spleen, and circulation in H5N1 pLAIV-primed animals. Thus, H5N1 pLAIV primes localized B cell responses in the MLN that are recalled systemically following pISV boost. These data provide mechanistic insights for the generation of robust humoral responses via prime-boost vaccination. IMPORTANCE We have previously shown that pandemic live attenuated influenza vaccines (pLAIV) prime for a rapid and robust antibody response on subsequent administration of inactivated subunit vaccine (pISV). This is observed even in individuals who had undetectable antibody (Ab) responses following the initial vaccination. To define the mechanistic basis of pLAIV priming, we turned to a nonhuman primate model and performed a detailed analysis of B cell responses in systemic and local lymphoid tissues following prime-boost vaccination with pLAIV and pISV. We show that the nonhuman primate model recapitulates the serologic observations from clinical studies. Further, we found that pLAIVs induced robust germinal center B cell responses in the mediastinal lymph node. Subsequent boosting with pISV in pLAIV-primed animals resulted in

Radiation therapy in adenoid-cystic carcinoma

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

Vikram, B.; Strong, E.W.; Shah, J.P.

1984-02-01

Between 1949-1977, 74 patients with adenoid-cystic carcinoma of various head and neck sites were treated by radiation therapy at Memorial Sloan-Kettering Cancer Center. Radiation therapy alone was employed in 49 patients for recurrent, unresectable disease, and in 25 patients it was given as an adjunct to surgical resection. Among the 49 patients treated with radiation therapy alone, tumor regression was seen in 47 (96%). However, 44 of the 47 (93.5%) subsequently relapsed locally. Relapse occurred within 18 months in one-half of the patients and within 5 years in all of them. Of the 25 patients who received adjunctive radiation therapymore » about one-half relapsed locally within five years. There were 9 patients in this group, however, whose field size exceeded 8x8 cm and the dose of radiation also exceeded 4500 rad: 88% of these patients remained relapse-free at 5 years, compared with only 22% of the other 16 whose dose, or field size, or both, were inadequate by comparison. These data suggest that when irradiation is employed for advanced, inoperable adenoid-cystic carcinoma, it offers useful palliation but is rarely, if ever, curative. Postoperative irradiation, on the other hand, might improve the local control and the survival in patients with operable adenoid-cystic carcinoma who are at high risk for relapse, but only if the field size and the dose are adequate.« less

Adenoid cystic carcinoma of child: a rare case.

PubMed

Mathai, Meera; Sherubin, J Eugenia; Agnihotri, Pg; Sangeetha, Gs

2014-01-01

Adenoid cystic carcinoma (ACC) is the second most common malignant tumor affecting both major and minor salivary glands. Clinically, it is a slowly growing tumor with high propensity for local invasion, recurrence and distant metastasis. It is predominantly seen in the ffith and sixth decades of life. Here, we report a rare case of ACC affecting the right maxilla of a 12-year-old girl. How to cite this article: Mathai M, Sherubin JE, Agnihotri PG, Sangeetha GS. Adenoid Cystic Carcinoma of Child: A Rare Case. Int J Clin Pediatr Dent 2014;7(3):206-208.

Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis.

PubMed

Derichs, Nico

2013-03-01

Cystic fibrosis (CF) is caused by genetic mutations that affect the cystic fibrosis transmembrane conductance regulator (CFTR) protein. These mutations can impact the synthesis and transfer of the CFTR protein to the apical membrane of epithelial cells, as well as influencing the gating or conductance of chloride and bicarbonate ions through the channel. CFTR dysfunction results in ionic imbalance of epithelial secretions in several organ systems, such as the pancreas, gastrointestinal tract, liver and the respiratory system. Since discovery of the CFTR gene in 1989, research has focussed on targeting the underlying genetic defect to identify a disease-modifying treatment for CF. Investigated management strategies have included gene therapy and the development of small molecules that target CFTR mutations, known as CFTR modulators. CFTR modulators are typically identified by high-throughput screening assays, followed by preclinical validation using cell culture systems. Recently, one such modulator, the CFTR potentiator ivacaftor, was approved as an oral therapy for CF patients with the G551D-CFTR mutation. The clinical development of ivacaftor not only represents a breakthrough in CF care but also serves as a noteworthy example of personalised medicine.

Cystic fibrosis swine fail to secrete airway surface liquid in response to inhalation of pathogens.

PubMed

Luan, Xiaojie; Belev, George; Tam, Julian S; Jagadeeshan, Santosh; Hassan, Noman; Gioino, Paula; Grishchenko, Nikolay; Huang, Yanyun; Carmalt, James L; Duke, Tanya; Jones, Teela; Monson, Bev; Burmester, Monique; Simovich, Tomer; Yilmaz, Orhan; Campanucci, Veronica A; Machen, Terry E; Chapman, L Dean; Ianowski, Juan P

2017-10-05

Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) channel, which can result in chronic lung disease. The sequence of events leading to lung disease is not fully understood but recent data show that the critical pathogenic event is the loss of the ability to clear bacteria due to abnormal airway surface liquid secretion (ASL). However, whether the inhalation of bacteria triggers ASL secretion and whether this is abnormal in cystic fibrosis has never been tested. Here we show, using a novel synchrotron-based in vivo imaging technique, that wild-type pigs display both a basal and a Toll-like receptor-mediated ASL secretory response to the inhalation of cystic fibrosis relevant bacteria. Both mechanisms fail in CFTR -/- swine, suggesting that cystic fibrosis airways do not respond to inhaled pathogens, thus favoring infection and inflammation that may eventually lead to tissue remodeling and respiratory disease.Cystic fibrosis is caused by mutations in the CFTR chloride channel, leading to reduced airway surface liquid secretion. Here the authors show that exposure to bacteria triggers secretion in wild-type but not in pig models of cystic fibrosis, suggesting an impaired response to pathogens contributes to infection.

Sjögren's syndrome with multiple cystic lesions and pulmonary arteriovenous fistulae.

PubMed

Taniguchi, Hirokazu; Miwa, Atsuo; Abo, Hitoshi; Demachi, Hiroshi; Izumi, Saburo

2008-01-01

A patient presented with Sjögren's syndrome associated with pulmonary multiple cystic lesions and a pulmonary arteriovenous fistulae. A histological examination of the lungs during the autopsy revealed the stenosis of the bronchiole lumens with hyperplasia of goblet cells, proliferation of smooth muscles in the inner wall of the bronchioli and retention of mucus in the airway lumens. These small airway changes were accompanied with chronic inflammatory changes of the airways in Sjögren's syndrome and led to the formation of cystic lesions via a ball-valve mechanism. Arteriovenous fistulae were situated around the cystic lesions. There may have been a correlation between the formation of the fistulae and cysts, but no mechanism was indicated in the histological findings. This report reveals that chronic inflammatory changes of the airways in Sjögren's syndrome are sufficient to cause the formation of cystic lesions.

Low-Grade Astrocytoma within a Mature Cystic Teratoma in an Adolescent Patient.

PubMed

Yoder, Nicole; Marks, Asher; Hui, Pei; Litkouhi, Babak; Cron, Julia

2018-06-01

Mature cystic teratomas are the most common ovarian neoplasm in adolescents. They are typically benign, however, malignant transformation rarely occurs. We report a low-grade astrocytoma arising from a mature cystic teratoma in an adolescent patient. The patient was a 12-year-old girl with an asymptomatic ovarian cyst and subsequent cystectomy. Final pathology identified a solid tumor with glial tissue within the cyst, reported as low-grade astrocytoma arising in a mature cystic teratoma. There are few data on astrocytomas in the gynecologic tract. Risk factors for malignant transformation in a mature cystic teratoma include increased age, postmenopausal status, elevated carcinoma antigen 125, and large tumor size. Interestingly, this patient had a history of partial trisomy 20, which has been associated with teratoma formation in a mouse model. Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

Trypsin level in gallbladder bile and ductitis and width of the cystic duct.

PubMed

Vracko, J; Wiechel, K L

2000-01-01

The change from laparotomy to laparoscopy for cholecystectomy has raised the question of how to manage concomitant bile duct stones. The present-day interest--and controversy--has focused on a transcystic approach reported to be feasible in 66-96% of cases, but without explaining the necessary prerequisite: the widening of the cystic duct. The cystic duct, wide mainly in patients with bile duct stones, has been reported to be highly variable: from strictured to very wide. The present study aims at comparing the trypsin level in the gallbladder bile and the cystic duct morphology and width in patients with and without bile duct stones. A prospective series of 63 gallstone patients, 30 with and 33 without bile duct stones (controls), underwent cholecystectomy and bile duct clearance. The study includes the trypsin level in the gallbladder bile, the width and morphology of the cystic duct, and the size of the gallstones. The patients with bile duct stones had, in contrast to the controls, higher trypsin levels in the gallbladder bile (P < 0.001) and wider cystic ducts (P < 0.001) with more pronounced signs of chronic ductitis. The obtained results strongly suggest that the increased trypsin level, a sign of reflux of pancreatic juice, caused changes in the cystic duct that facilitate gallstone migration, which also ought to render a transcystic stone extraction feasible.

Impact of cystic fibrosis disease on archaea and bacteria composition of gut microbiota.

PubMed

Miragoli, Francesco; Federici, Sara; Ferrari, Susanna; Minuti, Andrea; Rebecchi, Annalisa; Bruzzese, Eugenia; Buccigrossi, Vittoria; Guarino, Alfredo; Callegari, Maria Luisa

2017-02-01

Cystic fibrosis is often associated with intestinal inflammation due to several factors, including altered gut microbiota composition. In this study, we analyzed the fecal microbiota among patients with cystic fibrosis of 10-22 years of age, and compared the findings with age-matched healthy subjects. The participating patients included 14 homozygotes and 14 heterozygotes with the delF508 mutation, and 2 heterozygotes presenting non-delF508 mutations. We used PCR-DGGE and qPCR to analyze the presence of bacteria, archaea and sulfate-reducing bacteria. Overall, our findings confirmed disruption of the cystic fibrosis gut microbiota. Principal component analysis of the qPCR data revealed no differences between homozygotes and heterozygotes, while both groups were distinct from healthy subjects who showed higher biodiversity. Archaea were under the detection limit in all homozygotes subjects, whereas methanogens were detected in 62% of both cystic fibrosis heterozygotes and healthy subjects. Our qPCR results revealed a low frequency of sulfate-reducing bacteria in the homozygote (13%) and heterozygote (13%) patients with cystic fibrosis compared with healthy subjects (87.5%). This is a pioneer study showing that patients with cystic fibrosis exhibit significant reduction of H 2 -consuming microorganisms, which could increase hydrogen accumulation in the colon and the expulsion of this gas through non-microbial routes. © FEMS 2016.

Host response to Staphylococcus aureus cytotoxins in children with cystic fibrosis.

PubMed

Chadha, Ashley D; Thomsen, Isaac P; Jimenez-Truque, Natalia; Soper, Nicole R; Jones, Lauren S; Sokolow, Andrew G; Torres, Victor J; Creech, C Buddy

2016-09-01

Staphylococcus aureus is one of the earliest bacterial pathogens to colonize the lungs of children with cystic fibrosis and is an important contributor to pulmonary exacerbations. The adaptive host response to S. aureus in cystic fibrosis remains inadequately defined and has important implications for pathogenesis and potential interventions. The objectives of this study were to determine the functional antibody response to select staphylococcal exotoxins (LukAB, alpha-hemolysin, and PVL) in children with cystic fibrosis and to evaluate the relationship of this response with pulmonary exacerbations. Fifty children with cystic fibrosis were enrolled and followed prospectively for 12months. Clinical characteristics and serologic profiles were assessed at routine visits and during pulmonary exacerbations, and functional antibody assessments were performed to measure neutralization of LukAB-mediated cytotoxicity. For each antigen, geometric mean titers were significantly higher if S. aureus was detected at the time of exacerbation. For LukAB, geometric mean titers were significantly higher at exacerbation follow-up compared to titers during the exacerbation, consistent with expression during human disease, and the humoral response capably neutralized LukAB-mediated cytotoxicity. Moreover, the presence of a positive S. aureus culture during a pulmonary exacerbation was associated with 31-fold higher odds of having a LukA titer ≥1:160, suggesting potential diagnostic capability of this assay. The leukotoxin LukAB is expressed by S. aureus and recognized by the human adaptive immune response in the setting of pulmonary infection in cystic fibrosis. Anti-LukAB antibodies were not only predictive of positive staphylococcal culture during exacerbation, but also functional in the neutralization of this toxin. Copyright © 2016 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

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.

Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections.

PubMed

Hisert, Katherine B; Heltshe, Sonya L; Pope, Christopher; Jorth, Peter; Wu, Xia; Edwards, Rachael M; Radey, Matthew; Accurso, Frank J; Wolter, Daniel J; Cooke, Gordon; Adam, Ryan J; Carter, Suzanne; Grogan, Brenda; Launspach, Janice L; Donnelly, Seamas C; Gallagher, Charles G; Bruce, James E; Stoltz, David A; Welsh, Michael J; Hoffman, Lucas R; McKone, Edward F; Singh, Pradeep K

2017-06-15

Previous work indicates that ivacaftor improves cystic fibrosis transmembrane conductance regulator (CFTR) activity and lung function in people with cystic fibrosis and G551D-CFTR mutations but does not reduce density of bacteria or markers of inflammation in the airway. These findings raise the possibility that infection and inflammation may progress independently of CFTR activity once cystic fibrosis lung disease is established. To better understand the relationship between CFTR activity, airway microbiology and inflammation, and lung function in subjects with cystic fibrosis and chronic airway infections. We studied 12 subjects with G551D-CFTR mutations and chronic airway infections before and after ivacaftor. We measured lung function, sputum bacterial content, and inflammation, and obtained chest computed tomography scans. Ivacaftor produced rapid decreases in sputum Pseudomonas aeruginosa density that began within 48 hours and continued in the first year of treatment. However, no subject eradicated their infecting P. aeruginosa strain, and after the first year P. aeruginosa densities rebounded. Sputum total bacterial concentrations also decreased, but less than P. aeruginosa. Sputum inflammatory measures decreased significantly in the first week of treatment and continued to decline over 2 years. Computed tomography scans obtained before and 1 year after ivacaftor treatment revealed that ivacaftor decreased airway mucous plugging. Ivacaftor caused marked reductions in sputum P. aeruginosa density and airway inflammation and produced modest improvements in radiographic lung disease in subjects with G551D-CFTR mutations. However, P. aeruginosa airway infection persisted. Thus, measures that control infection may be required to realize the full benefits of CFTR-targeting treatments.

Bilateral Breast Enlargement: An Unusual Presentation of Superior Vena Cava Obstruction in a Hemodialysis Patient with Fibrosing Mediastinitis

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

Goo, Dong Erk, E-mail: degoo@hosp.sch.ac.kr; Kim, Yong Jae; Choi, Deuk Lin

2011-02-15

A 67-year-old woman with end-stage renal disease presented with profound edema of both breasts. The presence of a patent hemodialysis basilic transposition fistula and superior vena cava obstruction (SVC), due to fibrosing mediastinitis, was demonstrated by the use of fistulography. Endovascular treatment with a balloon and stent caused immediate resolution of the breast edema.

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.

An evaluation of different steam disinfection protocols for cystic fibrosis nebulizers.

PubMed

Hohenwarter, K; Prammer, W; Aichinger, W; Reychler, G

2016-01-01

Contamination is a key element in cystic fibrosis. For this reason, nebulizer hygiene is an important, but complex and time-consuming task for cystic fibrosis patients. The aim of this study was to compare different steam disinfection and drying protocols. One hundred nebulizer parts were inoculated with cystic fibrosis-related bacteria in high concentrations (Burkholderia multivorans 3.9 × 10(10)/ml, Staphylococcus aureus 8.9 × 10(8/)ml and Pseudomonas aeruginosa 2.1 × 10(9)/ml). Tubes with Mycobacterium abscessus complex were additionally tested. Six steam disinfectors were compared. Different methods of drying were examined. All tested bacteria were efficiently killed by the different steam disinfectors tested. The risk of contamination depended on the method of drying. Steam disinfection is a safe disinfection method. It is better to leave the nebulizers wet after steam disinfection than to manipulate them by active drying, which seems to be a source of recontamination. Copyright © 2015 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Early respiratory infection is associated with reduced spirometry in children with cystic fibrosis.

PubMed

Ramsey, Kathryn A; Ranganathan, Sarath; Park, Judy; Skoric, Billy; Adams, Anne-Marie; Simpson, Shannon J; Robins-Browne, Roy M; Franklin, Peter J; de Klerk, Nick H; Sly, Peter D; Stick, Steve M; Hall, Graham L

2014-11-15

Pulmonary inflammation, infection, and structural lung disease occur early in life in children with cystic fibrosis. We hypothesized that the presence of these markers of cystic fibrosis lung disease in the first 2 years of life would be associated with reduced lung function in childhood. Lung function (forced expiratory volume in the first three-quarters of a second [FEV0.75], FVC) was assessed in individuals with cystic fibrosis diagnosed after newborn screening and healthy subjects during infancy (0-2 yr) and again at early school age (4-8 yr). Individuals with cystic fibrosis underwent annual bronchoalveolar lavage fluid examination, and chest computed tomography. We examined which clinical outcomes (pulmonary inflammation, infection, structural lung disease, respiratory hospitalizations, antibiotic prophylaxis) measured in the first 2 years of life were associated with reduced lung function in infants and young children with cystic fibrosis, using a mixed effects model. Children with cystic fibrosis (n = 56) had 8.3% (95% confidence interval [CI], -15.9 to -6.6; P = 0.04) lower FEV0.75 compared with healthy subjects (n = 18). Detection of proinflammatory bacterial pathogens (Pseudomonas aeruginosa, Staphylococcus aureus, Haemophilus influenzae, Aspergillus species, Streptococcus pneumoniae) in bronchoalveolar lavage fluid was associated with clinically significant reductions in FEV0.75 (ranging between 11.3 and 15.6%). The onset of lung disease in infancy, specifically the occurrence of lower respiratory tract infection, is associated with low lung function in young children with cystic fibrosis. Deficits in lung function measured in infancy persist into childhood, emphasizing the need for targeted therapeutic interventions in infancy to maximize functional outcomes later in life.

Atomic Structure of the Cystic Fibrosis Transmembrane Conductance Regulator.

PubMed

Zhang, Zhe; Chen, Jue

2016-12-01

The cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel evolved from the ATP-binding cassette (ABC) transporter family. In this study, we determined the structure of zebrafish CFTR in the absence of ATP by electron cryo-microscopy to 3.7 Å resolution. Human and zebrafish CFTR share 55% sequence identity, and 42 of the 46 cystic-fibrosis-causing missense mutational sites are identical. In CFTR, we observe a large anion conduction pathway lined by numerous positively charged residues. A single gate near the extracellular surface closes the channel. The regulatory domain, dephosphorylated, is located in the intracellular opening between the two nucleotide-binding domains (NBDs), preventing NBD dimerization and channel opening. The structure also reveals why many cystic-fibrosis-causing mutations would lead to defects either in folding, ion conduction, or gating and suggests new avenues for therapeutic intervention. Copyright © 2016 Elsevier Inc. All rights reserved.

CT differentiation of mucin-producing cystic neoplasms of the liver from solitary bile duct cysts.

PubMed

Kim, Hyoung Jung; Yu, Eun Sil; Byun, Jae Ho; Hong, Seung-Mo; Kim, Kyoung Won; Lee, Jong Seok; Kim, So Yeon

2014-01-01

The purpose of this study was to identify the CT features required for differentiating mucin-producing cystic neoplasms of the liver (mucinous cystic neoplasms and cyst-forming intraductal papillary neoplasms of the bile duct) from solitary bile duct cysts. CT images of pathologically confirmed mucinous cystic neoplasms (n = 15), cyst-forming intraductal papillary neoplasms of the bile duct (n = 16), and solitary bile duct cysts (n = 31) were reviewed. Analysis of the CT findings included shape, presence of septa, location of septa (peripheral vs central), thickness of septa (thin vs thick), mosaic pattern, mural nodules, intracystic debris, calcification, upstream bile duct dilatation, downstream bile duct dilatation, and communication between a cystic lesion and the bile duct. The maximum size of a cystic lesion and the maximum size of the largest mural nodule were measured. The presence of septa, central septa, mural nodules, upstream bile duct dilatation, and downstream bile duct dilatation were found to be significant CT findings for differentiating mucinous cystic neoplasms and cyst-forming intraductal papillary neoplasms of the bile duct from solitary bile duct cysts (p < 0.05 for each finding). When two of these five criteria were used in combination, the sensitivity and specificity for diagnosing mucin-producing cystic neoplasms and cyst-forming intraductal papillary neoplasms of the bile duct were 87% (27 of 31) and 87% (27 of 31), respectively. When two of these five criteria were used in combination, the sensitivity and specificity for diagnosing mucinous cystic neoplasms and cyst-forming intraductal papillary neoplasms of the bile duct were 87% (27 of 31) and 87% (27 of 31), respectively [corrected]. With the use of specific CT criteria, mucin-producing cystic neoplasms of the liver can be differentiated from solitary bile duct cysts with a high degree of accuracy.

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.

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

Cystic Fibrosis-Related Diabetes (CFRD): Daily Management

MedlinePlus

Cystic Fibrosis-Related Diabetes (CFRD): Daily Management September 20, 2011 This Web cast is supported by an unrestricted educational grant from Genentech, Inc. Antoinette, Moran, MD Professor, Pediatric Endocrinology ...

Negative pressure wound treatment improves Acute Physiology and Chronic Health Evaluation II score in mediastinitis allowing a successful elective pectoralis muscle flap closure: six-year experience of a single protocol.

PubMed

Salica, Andrea; Weltert, Luca; Scaffa, Raffaele; Guerrieri Wolf, Lorenzo; Nardella, Saverio; Bellisario, Alessandro; De Paulis, Ruggero

2014-11-01

Optimal management of poststernotomy mediastinitis is controversial. Negative pressure wound treatment improves wound environment and sternal stability with low surgical invasiveness. Our protocol was based on negative pressure followed by delayed surgical closure. The aim of this study was to provide the results at early follow-up and to identify the risk factors for adverse outcome. In 5400 cardiac procedures, 44 consecutive patients with mediastinitis were enrolled in the study. Mediastinitis treatment was based on urgent debridement and negative pressure as the first-line approach. After wound sterilization, chest closure was achieved by elective pectoralis muscle advancement flap. Each patient's hospital data were collected prospectively. Variables included patient demographics and clinical and biological data. Acute Physiology and Chronic Health Evaluation (APACHE) II score was calculated at the time of diagnosis and 48 hours after debridement. Focus outcome measures were mediastinitis-related death and need for reintervention after pectoralis muscle closure. El Oakley type I and type IIIA mediastinitis were the most frequent types (63.6%). Methicillin-resistant Staphylococcus aureus was present in 25 patients (56.8%). Mean APACHE II score was 19.4±4 at the time of diagnosis, and 30 patients (68.2%) required intensive care unit transfer before surgical debridement. APACHE II score improved 48 hours after wound debridement and negative pressure application (mean value, 19.4±4 vs 7.2±2; P=.005) independently of any other variables included in the study. One patient in septic shock at the time of diagnosis died (2.2%). Negative pressure promotes a significant improvement in clinical status according to APACHE II score and allows a successful elective surgical closure. Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Altered steady state pharmacokinetics of levofloxacin in adult cystic fibrosis patients receiving calcium carbonate.

PubMed

Pai, Manjunath P; Allen, Sarah E; Amsden, Guy W

2006-08-01

Levofloxacin is used in adult patients with cystic fibrosis but its pharmacokinetics is not well characterized in this population. Patients with cystic fibrosis use calcium routinely to prevent osteoporosis. A slower intestinal transit time is common in cystic fibrosis implying that the standard 2-h spacing of minerals and levofloxacin to prevent a chelation interaction may be insufficient. The objectives of this study were to characterize the steady state pharmacokinetics of oral levofloxacin 750 mg with and without 2-h spaced calcium carbonate in patients with cystic fibrosis compared to matched healthy volunteers. In an open-label, randomized, cross-over study of five patients with cystic fibrosis and five age, sex, race, and serum creatinine matched healthy volunteers received 750 mg of oral levofloxacin alone daily for 5 days and the same dose of levofloxacin with 2-h spaced calcium carbonate supplementation 500 mg po thrice daily with meals in random sequence. Blood was collected for plasma assay of levofloxacin pre-dose, 0.5, 1, 1.5, 2, 4, 8, 12, and 24h after the fifth levofloxacin dose. There was no significant interaction in healthy volunteers, however, when cystic fibrosis patients were given levofloxacin with 2-h spaced calcium, the maximum plasma concentration (Cmax) decreased by 19% and time to Cmax increased by 37% (p<0.05). This difference in peak concentrations resulted in a lack of bioequivalence (Cmax geometric mean ratio 81.6%, 90% confidence intervals: 71.8%, 91.4%) even when levofloxacin and calcium supplements were spaced by the standard 2h administration instruction in patients with cystic fibrosis. These results indicate that multivalent cations such as calcium should be maximally separated from oral levofloxacin administration in adult patients with cystic fibrosis to prevent this drug interaction, thereby better optimizing antibiotic efficacy and decreasing the potential for resistance development.

Efficacy and Safety of Ultrasound-Guided Percutaneous Polidocanol Sclerotherapy in Benign Cystic Thyroid Nodules: Preliminary Results.

PubMed

Gong, Xiaohua; Zhou, Qi; Wang, Fang; Wu, Wenjun; Chen, Xiaojun

2017-01-01

To evaluate the efficacy and safety of percutaneous polidocanol injection (PPI) in treating cystic thyroid nodules. A total of 158 cystic or predominantly cystic thyroid nodules (>80% cystic component) in 143 patients were evaluated. 114 patients with compressive symptoms or aesthetic complaints were offered PPI. 44 individuals without compressive symptoms and aesthetic complaints who were only followed up clinically were used as the control group. The efficacy and safety of PPI were evaluated for 1 month, 3 months, 6 months, 9 months, and 12 months of follow-up. In the PPI group, the mean baseline volume of 15.6 ± 18.9 cm 3 reduced at the 1-month follow-up to 5.1 ± 5.6 cm 3 ( p < 0.001) and 0.6 ± 0.9 ( p < 0.001), and nodules shrunk according to the time after PPI ( p < 0.001). A complete response (if ≥70% decrease) to PPI at the 12-month follow-up occurred in 100% of the cystic or predominant cystic nodules. None of the nodules recurred at the 12-month follow-up after PPI. The side effects were mild. Twenty patients (17.5%) developed mild localized pain, and fourteen cases (12.3%) experienced mild or moderate fever after PPI. PPI is a safe and effective alternative to treat benign cystic or predominant cystic thyroid nodules.

Omega-3 fatty acids for cystic fibrosis.

PubMed

Oliver, Colleen; Watson, Helen

2016-01-05

Studies suggest that a diet rich in omega-3 essential fatty acids may have beneficial anti-inflammatory effects for chronic conditions such as cystic fibrosis. This is an updated version of a previously published review. To determine whether there is evidence that omega-3 polyunsaturated fatty acid supplementation reduces morbidity and mortality and to identify any adverse events associated with supplementation. 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. Authors and persons interested in the subject of the review were contacted.Date of last search: 13 August 2013. Randomised controlled trials in people with cystic fibrosis comparing omega-3 fatty acid supplements with placebo. Two authors independently selected studies for inclusion, extracted data and assessed the risk of bias of the studies. The searches identified 15 studies; four studies with 91 participants (children and adults) were included; duration of studies ranged from six weeks to six months. Two studies were judged to be at low risk of bias based on adequate randomisation but this was unclear in the other two studies. Three of the studies adequately blinded patients, however, the risk of bias was unclear in all studies with regards to allocation concealment and selective reporting.Two studies compared omega-3 fatty acids to olive oil for six weeks. One study compared a liquid dietary supplement containing omega-3 fatty acids to one without for six months. One study compared omega-3 fatty acids and omega-6 fatty acids to a control (capsules with customised fatty acid blends) for three months. Only one short-term study (19 participants) comparing omega-3 to placebo reported a significant improvement in lung function and Shwachman score and a reduction in sputum volume in the omega-3 group. Another

[A case showing a complete response by weekly paclitaxel associated with severe empyema and mediastinal abscess caused by reduction of a recurrent lung metastatic tumor originating from adenocarcinoma of the esophagogastric junction after primary operation].

PubMed

Kimura, Akiharu; Hiramatsu, Kiyoshi; Sakuragawa, Tadayuki; Ito, Takaaki; Otsuji, Hidehiko; Tsuchiya, Tomonori; Hara, Tomohiro; Maeda, Takao; Tanaka, Hiroshi; Machiki, Yuichi; Hosoya, Jun; Kojima, Tsuyoshi; Kato, Kenji

2010-02-01

The patient was a 57-year-old man who presented with cancer of the esophagogastric junction. He underwent total gastrectomy, lower esophagectomy, distal pancreatectomy and splenectomy with para-aortic lymphnode dissection by the transthoracoabdominal approach. He was given a daily dose of 100 mg of S-1 as adjuvant chemotherapy. About one year after the operation, lung metastasis was recognized by enhanced CT examination. He began weekly paclitaxel as second-line chemotherapy. Paclitaxel was infused once a week. About two weeks after the first infusion therapy, he was admitted to our hospital with fever and dyspnea. A chest enhanced CT revealed remarkable empyema and mediastinal abscess. Chest drainage and mediastinal drainage were performed.After one month of drainage, the empyema and mediastinal abscess had improved. The metastastic tumor of the lung disappeared at the time of discharge. CR has been maintained for more than a year without chemotherapy.This case suggests that remarkable reduction of the tumor induced by chemotherapy may have caused the empyema and mediastinal abscess.

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

Prevalence of cystic macular lesions in patients with Usher II syndrome.

PubMed

Walia, S; Fishman, G A; Hajali, M

2009-05-01

To evaluate the prevalence of cystic macular lesions in patients with Usher II syndrome. All Usher type II patients seen in the inherited eye disease clinic at the University of Illinois at Chicago between January 2002 and December 2007 were included (n=76). Each participating patient underwent a detailed clinical examination, including best-corrected visual acuity, slit-lamp biomicroscopy and dilated fundus examination. The presence of cystoid lesions was determined by optical coherence tomography (OCT), fundus fluorescein angiogram (FFA), fundus photographs and/or clinical examination. A cystic-appearing macular change was observed in at least one eye in 19 out of the 76 patients (25%), 13 on the basis of OCT, five using FFA (two solely with the use of FFA and three based on clinical notes and FFA findings) and one based solely on clinical notes. Of the 18 patients with CME, determined by OCT or FFA, five (27.8%) showed either a funduscopically normal-appearing macula (n=4) or an atrophic appearing macular change (n=1). One-fourth of our total cohort of Usher II patients had cystic macular lesions. Moreover, a funduscopically normal-appearing macula was observed in 22% (n=4) of our 18 patients with cystic-appearing macular lesions on OCT and/or FFA testing. On the basis of the reasonably high prevalence of cystic macular lesions in our cohort, it would seem prudent to evaluate Usher II patients for the presence of cystoid macular oedema.

A sticky situation: myocardial infarction in a young woman with immune thrombocytopenia on eltrombopag and a history of mediastinal radiation.

PubMed

Teichman, Jennifer; Taher, Ahmed; Hashi, Abdulaziz; Bagai, Akshay; Sholzberg, Michelle

2018-01-01

More recent immune thrombocytopenia (ITP) treatment strategies enhance platelet production with the use of thrombopoietin receptor agonists (TPO-RA) such as eltrombopag. Patients receiving TPO-RA agents may be at an increased risk of thromboembolism, however the pathophysiology and common underlying risk factors are not well understood. We present the case of a young asplenic woman on eltrombopag for chronic ITP with acute myocardial infarction involving the right coronary artery. Past medical history was significant for remote mediastinal radiation for lymphoma and splenectomy for ITP. She had no other risk factors for coronary artery disease. She underwent coronary catheterization and balloon angioplasty to the culprit lesion, although stenting was deferred due to concerns with dual antiplatelet therapy. She was discharged from hospital on single antiplatelet therapy with acetylsalicylic acid. We believe that the patient's ITP, recent eltrombopag use, surgical asplenia and history of mediastinal radiation synergistically contributed to her myocardial infarction. The risks of bleeding and thromboembolism must be carefully weighed in patients receiving TPO-RA therapy.

Diagnosis of malignancy of adult mediastinal tumors by conventional and transesophageal echocardiography.

PubMed

Zhou, Wei-Wei; Wang, Hong-Wei; Liu, Nan-Nan; Li, Jing-Jing; Yuan, Wei; Zhao, Rui; Xiang, Liang-Bi; Qi, Miao

2015-04-20

Transesophageal echocardiography (TEE) is a well-established method for detecting and diagnosing heart tumors. In contrast, its role in assessing the presence, growth and evidence of malignant tumors originating from mediastinal sites remains unclear. The aim of this study was to compare the diagnostic impact of TEE and transthoracic echocardiography (TTE) for determining the localization, growth and malignancy of adult mediastinal tumors (MTs). In a prospective and investigator-blinded study, we evaluated 144 consecutive patients with MT lesions to assess the diagnostic impact of TEE and TTE for detecting the presence of tumors spreading both inside and outside of the heart and for determining infiltration and invasion using pathological examination results as a reference. All tumor lesions were diagnosed and carefully evaluated by biopsy. Biopsy revealed malignant tumors in 79 patients and benign tumors in 65 patients. When compared to histological findings, TEE predicted malignancy from the presence of tumors spreading both inside and outside of the heart and from infiltration and invasion in 49/79 patients (62.0%). TTE predicted malignancy in only 8/79 patients (10.1%, P < 0.005). TEE visualized tumor lesions in 130 patients (90.3%) while the TTE visualized tumor lesions in 110 patients (76.4%) and was less effective at detecting MT lesions (P < 0.001). TTE and TEE could detect anterior MTs and adequately verified MTs (P > 0.05); TEE detected medium MTs better than TTE (P < 0.001). TEE is effective and superior to TTE for predicting the localization and growth of MTs as well as for accessing evidence of tumor malignancy. TTE and TEE were able to detect anterior MTs; TEE was able to detect medium MT better than TTE.

Diagnosis of Malignancy of Adult Mediastinal Tumors by Conventional and Transesophageal Echocardiography

PubMed Central

Zhou, Wei-Wei; Wang, Hong-Wei; Liu, Nan-Nan; Li, Jing-Jing; Yuan, Wei; Zhao, Rui; Xiang, Liang-Bi; Qi, Miao

2015-01-01

Background: Transesophageal echocardiography (TEE) is a well-established method for detecting and diagnosing heart tumors. In contrast, its role in assessing the presence, growth and evidence of malignant tumors originating from mediastinal sites remains unclear. The aim of this study was to compare the diagnostic impact of TEE and transthoracic echocardiography (TTE) for determining the localization, growth and malignancy of adult mediastinal tumors (MTs). Methods: In a prospective and investigator-blinded study, we evaluated 144 consecutive patients with MT lesions to assess the diagnostic impact of TEE and TTE for detecting the presence of tumors spreading both inside and outside of the heart and for determining infiltration and invasion using pathological examination results as a reference. Results: All tumor lesions were diagnosed and carefully evaluated by biopsy. Biopsy revealed malignant tumors in 79 patients and benign tumors in 65 patients. When compared to histological findings, TEE predicted malignancy from the presence of tumors spreading both inside and outside of the heart and from infiltration and invasion in 49/79 patients (62.0%). TTE predicted malignancy in only 8/79 patients (10.1%, P < 0.005). TEE visualized tumor lesions in 130 patients (90.3%) while the TTE visualized tumor lesions in 110 patients (76.4%) and was less effective at detecting MT lesions (P < 0.001). TTE and TEE could detect anterior MTs and adequately verified MTs (P > 0.05); TEE detected medium MTs better than TTE (P < 0.001). Conclusions: TEE is effective and superior to TTE for predicting the localization and growth of MTs as well as for accessing evidence of tumor malignancy. TTE and TEE were able to detect anterior MTs; TEE was able to detect medium MT better than TTE. PMID:25881598

Lung transplantation for cystic fibrosis.

PubMed

Coloni, G F; Venuta, F; Ciccone, A M; Rendina, E A; De Giacomo, T; Filice, M J; Diso, D; Anile, M; Andreetti, C; Aratari, M T; Mercadante, E; Moretti, M; Ibrahim, M

2004-04-01

Lung transplantation is a robust therapeutic option to treat patients with cystic fibrosis. Since 1996, 109 patients with cystic fibrosis were accepted onto our waiting list with 58 bilateral sequential lung transplants performed in 56 patients and two patients retransplanted for obliterative bronchiolitis syndrome. Preoperative mean FEV(1) was 0.64 L/s, mean PaO(2) with supplemental oxygen was 56 mm Hg, and the mean 6-minute walking test was 320 m. Transplantation was performed through a "clam shell incision" in the first 29 patients and via bilateral anterolateral thoracotomies without sternal division in the remaining patients. Cardiopulmonary bypass was required in 14 patients. In 21 patients the donor lungs had to be trimmed by wedge resections with mechanical staplers and bovine pericardium buttressing to fit the recipient chest size. Eleven patients were extubated in the operating room immediately after the procedure. Hospital mortality of 13.8% was related to infection (n = 5), primary graft failure (n = 2), and myocardial infarction (n = 1). Acute rejection episodes occurred 1.6 times per patient/year; lower respiratory tract infections occurred 1.4 times per patient in the first year after transplantation. The mean FEV(1) increased to 82% at 1 year after operation. The 5-year survival rate was 61%. A cyclosporine-based immunosuppressive regimen was initially employed in all patients; 24 were subsequently switched to tacrolimus because of central nervous system toxicity, cyclosporine-related myopathy, or renal failure, obliterative bronchiolitis syndrome, gingival hyperplasia, or hypertrichosis. Ten patients were subsequently switched to sirolimus. Freedom from bronchiolitis obliterans at 5 years was 60%. Our results confirm that bilateral sequential lung transplantation is a robust therapeutic option for patients with cystic fibrosis.

Vitamin K supplementation for cystic fibrosis.

PubMed

Jagannath, Vanitha A; Fedorowicz, Zbys; Thaker, Vidhu; Chang, Anne B

2015-01-18

Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction. Malabsorption of fat and fat-soluble vitamins (A, D, E, K) may occur and can cause subclinical deficiencies of some of these vitamins. Vitamin K is known to play an important role in both blood coagulation and bone formation. Supplementation with vitamin K appears to be one way of addressing the deficiency, but there is very limited agreement on the appropriate dose and frequency of use of these supplements. To assess the effects of vitamin K supplementation in people with cystic fibrosis and to determine the optimal dose and route of administration of vitamin K for both routine and therapeutic use. 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.Most recent search: 08 October 2014. Randomised and quasi-randomised controlled trials of all preparations of vitamin K used as a supplement compared to either no supplementation (or placebo) at any dose or route and for any duration, in children or adults diagnosed with cystic fibrosis (by sweat test or genetic testing). Two authors independently screened papers, extracted trial details and assessed their risk of bias. Two trials (total of 32 participants) each lasting one month were included in the review and were assessed as having a moderate risk of bias. One was a dose-ranging parallel group trial in children (aged 8 to 18 years); and the other (with an older cohort) had a cross-over design comparing supplements to no treatment, but no separate data were reported for the first intervention period. Neither of the trials addressed any of the primary outcomes (coagulation, bone formation and quality of life). Both trials reported the restoration of serum vitamin K and undercarboxylated osteocalcin levels to the normal range after one month of

Vitamin K supplementation for cystic fibrosis.

PubMed

Jagannath, Vanitha A; Fedorowicz, Zbys; Thaker, Vidhu; Chang, Anne B

2011-01-19

Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction. Malabsorption of fat and fat-soluble vitamins (A, D, E, K) may occur and can cause subclinical deficiencies of some of these vitamins. Vitamin K is known to play an important role in both blood coagulation and bone formation. Supplementation with vitamin K appears to be one way of addressing the deficiency, but there is very limited agreement on the appropriate dose and frequency of use of these supplements. To assess the effects of vitamin K supplementation in people with cystic fibrosis and to determine the optimal dose and route of administration of vitamin K for both routine and therapeutic use. 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.Most recent search: 15 April 2010. Randomised and quasi-randomised controlled trials of all preparations of vitamin K used as a supplement compared to either no supplementation (or placebo) at any dose or route and for any duration, in children or adults diagnosed with cystic fibrosis (by sweat test or genetic testing). Two authors independently screened papers, extracted trial details and assessed their risk of bias. Two trials (total of 32 participants) were included in the review and were assessed as having a moderate risk of bias. One was a dose-ranging parallel group trial; and the other had a cross-over design, but no separate data were reported for the first intervention period. Neither of the trials addressed any of the primary outcomes (coagulation, bone formation and quality of life). Both trials reported the restoration of serum vitamin K and undercarboxylated osteocalcin levels to the normal range after one month of daily supplementation with 1 mg of vitamin K. Evidence from randomised controlled trials on the benefits of routine

Vitamin K supplementation for cystic fibrosis.

PubMed

Jagannath, Vanitha A; Fedorowicz, Zbys; Thaker, Vidhu; Chang, Anne B

2013-04-30

Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction. Malabsorption of fat and fat-soluble vitamins (A, D, E, K) may occur and can cause subclinical deficiencies of some of these vitamins. Vitamin K is known to play an important role in both blood coagulation and bone formation. Supplementation with vitamin K appears to be one way of addressing the deficiency, but there is very limited agreement on the appropriate dose and frequency of use of these supplements. To assess the effects of vitamin K supplementation in people with cystic fibrosis and to determine the optimal dose and route of administration of vitamin K for both routine and therapeutic use. 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.Most recent search: 11 October 2012. Randomised and quasi-randomised controlled trials of all preparations of vitamin K used as a supplement compared to either no supplementation (or placebo) at any dose or route and for any duration, in children or adults diagnosed with cystic fibrosis (by sweat test or genetic testing). Two authors independently screened papers, extracted trial details and assessed their risk of bias. Two trials (total of 32 participants) were included in the review and were assessed as having a moderate risk of bias. One was a dose-ranging parallel group trial; and the other had a cross-over design, but no separate data were reported for the first intervention period. Neither of the trials addressed any of the primary outcomes (coagulation, bone formation and quality of life). Both trials reported the restoration of serum vitamin K and undercarboxylated osteocalcin levels to the normal range after one month of daily supplementation with 1 mg of vitamin K. Evidence from randomised controlled trials on the benefits of routine

Analysis of cystic fibrosis gene mutations in children with cystic fibrosis and in 964 infertile couples within the region of Basilicata, Italy: a research study.

PubMed

Dell'Edera, Domenico; Benedetto, Michele; Gadaleta, Gemma; Carone, Domenico; Salvatore, Donatello; Angione, Antonella; Gallo, Massimiliano; Milo, Michele; Pisaturo, Maria Laura; Di Pierro, Giuseppe; Mazzone, Eleonora; Epifania, Annunziata Anna

2014-10-10

Cystic fibrosis is the most common autosomal recessive genetic disease in the Caucasian population. Extending knowledge about the molecular pathology on the one hand allows better delineation of the mutations in the CFTR gene and the other to dramatically increase the predictive power of molecular testing. This study reports the results of a molecular screening of cystic fibrosis using DNA samples of patients enrolled from January 2009 to December 2013. Patients were referred to our laboratory for cystic fibrosis screening for infertile couples. In addition, we identified the gene mutations present in 76 patients affected by cystic fibrosis in the pediatric population of Basilicata. In the 964 infertile couples examined, 132 subjects (69 women and 63 men) resulted heterozygous for one of the CFTR mutations, with a recurrence of carriers of 6.85%. The recurrence of carriers in infertile couples is significantly higher from the hypothetical value of the general population (4%). This study shows that in the Basilicata region of Italy the CFTR phenotype is caused by a small number of mutations. Our aim is to develop a kit able to detect not less than 96% of CTFR gene mutations so that the relative risk for screened couples is superimposable with respect to the general population.

Aspergillus infections in cystic fibrosis.

PubMed

King, Jill; Brunel, Shan F; Warris, Adilia

2016-07-05

Patients with cystic fibrosis (CF) suffer from chronic lung infection and airway inflammation. Respiratory failure secondary to chronic or recurrent infection remains the commonest cause of death and accounts for over 90% of mortality. Bacteria as Staphylococcus aureus, Pseudomonas aeruginosa and Burkholderia cepacia complex have been regarded the main CF pathogens and their role in progressive lung decline has been studied extensively. Little attention has been paid to the role of Aspergillus spp. and other filamentous fungi in the pathogenesis of non-ABPA (allergic bronchopulmonary aspergillosis) respiratory disease in CF, despite their frequent recovery in respiratory samples. It has become more apparent however, that Aspergillus spp. may play an important role in chronic lung disease in CF. Research delineating the underlying mechanisms of Aspergillus persistence and infection in the CF lung and its link to lung deterioration is lacking. This review summarizes the Aspergillus disease phenotypes observed in CF, discusses the role of CFTR (cystic fibrosis transmembrane conductance regulator)-protein in innate immune responses and new treatment modalities. Copyright © 2016. Published by Elsevier Ltd.

The lower airway microbiota in early cystic fibrosis lung disease: a longitudinal analysis.

PubMed

Frayman, Katherine B; Armstrong, David S; Carzino, Rosemary; Ferkol, Thomas W; Grimwood, Keith; Storch, Gregory A; Teo, Shu Mei; Wylie, Kristine M; Ranganathan, Sarath C

2017-12-01

In infants and young children with cystic fibrosis, lower airway infection and inflammation are associated with adverse respiratory outcomes. However, the role of lower airway microbiota in the pathogenesis of early cystic fibrosis lung disease remains uncertain. To assess the development of the lower airway microbiota over time in infants and young children with cystic fibrosis, and to explore its association with airway inflammation and pulmonary function at age 6 years. Serial, semi-annual bronchoscopies and bronchoalveolar lavage (BAL) procedures were performed in infants newly diagnosed with cystic fibrosis following newborn screening. Quantitative microbiological cultures and inflammatory marker (interleukin 8 and neutrophil elastase) measurements were undertaken contemporaneously. 16S ribosomal RNA gene sequencing was conducted on stored BAL samples. Spirometry results recorded at 6 years of age were extracted from medical records. Ninety-five BAL samples provided 16S ribosomal RNA gene data. These were collected from 48 subjects aged 1.2-78.3 months, including longitudinal samples from 27 subjects and 13 before age 6 months. The lower airway microbiota varied, but diversity decreased with advancing age. Detection of recognised cystic fibrosis bacterial pathogens was associated with reduced microbial diversity and greater lower airway inflammation. There was no association between the lower airway microbiota and pulmonary function at age 6 years. In infants with cystic fibrosis, the lower airway microbiota is dynamic. Dominance of the microbiota by recognised cystic fibrosis bacterial pathogens is associated with increased lower airway inflammation, however early microbial diversity is not associated with pulmonary function at 6 years of age. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Ursodeoxycholic acid treatment in patients with cystic fibrosis at risk for liver disease.

PubMed

Siano, Maria; De Gregorio, Fabiola; Boggia, Bartolo; Sepe, Angela; Ferri, Pasqualina; Buonpensiero, Paolo; Di Pasqua, Antonio; Raia, Valeria

2010-06-01

Meconium ileus has been detected as a risk factor for development of liver disease in cystic fibrosis, with influence on morbidity and mortality. To evaluate the effect of early treatment with ursodeoxycholic acid in patients with cystic fibrosis and meconium ileus to prevent chronic hepatic involvement and to explore the potential role of therapy on clinical outcomes. 26 cystic fibrosis patients with meconium ileus (16 M, mean age 8,4 years, range 3,5-9) were assigned to two groups: group 1 (14 patients) treated early with ursodeoxycholic acid (UDCAe); group 2 (12 patients) treated with ursodeoxycholic acid at the onset of cystic fibrosis liver disease (UDCAd). Anthropometric data, pulmonary function tests, pancreatic status, complications such as diabetes, hepatic involvement and Pseudomonas aeruginosa colonisation were compared among groups. A higher prevalence of cystic fibrosis chronic liver disease was observed in the UDCAd group with a statistically significant difference at 9 years of age (p<0.05). Chronic infection by P. aeruginosa was found in 7% of UDCAe and 33% of UDCAd (p<0.05). No differences were observed in nutritional status and other complications. Early treatment with ursodeoxycholic acid may be beneficial in patients at risk of developing cystic fibrosis chronic liver disease such as those with meconium ileus. Multicentre studies should be encouraged to confirm these data. Copyright 2009 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

Human papillomavirus-related carcinoma with adenoid cystic-like features of the inferior turbinate: a case report.

PubMed

Hwang, Sun Jin; Ok, Sohea; Lee, Heung-Man; Lee, Eunjung; Park, Il-Ho

2015-02-01

Sinonasal malignancies are uncommon, but are of many different histologic types. Recently, Human papillomavirus (HPV)-related carcinoma with adenoid cystic features was reported as a new histologic form. Although this histologic type resembles an adenoid cystic carcinoma, it differs from adenoid cystic carcinomas with regard to its association with HPV. Here, we present a case of HPV-related carcinoma with adenoid cystic features in the nasal cavity. We also review the histological characters of the tumor. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

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.

[Diagnostic value of MYB protein expression in adenoid cystic carcinoma and status of MYB gene copy number].

PubMed

Huo, Zhen; Zeng, Xuan; Wu, Shafei; Wu, Huanwen; Meng, Yunxiao; Liu, Yuanyuan; Luo, Yufeng; Cao, Jinling; Liang, Zhiyong

2015-08-01

To explore the diagnostic value of MYB protein expression for adenoid cystic carcinoma and its differential diagnosis from other salivary gland tumors, and to further investigate the status of MYB gene copy number. MYB expression was studied by immunohistochemistry in 34 adenoid cystic carcinomas, 55 non-adenoid cystic carcinomas (other salivary gland tumors) including 10 pleomorphic adenomas, 10 basal cell adenomas, 10 epithelial-myoepithelial carcinomas, 9 basal cell adenocarcinomas, 8 mucoepidermoid carcinomas, 4 carcinoma in pleomorphic adenomas, and 4 polymorphous low-grade adenocarcinoma. MYB gene copy number status was detected by FISH in MYB protein-positive cases. 82.4% (28/34) of adenoid cystic carcinomas were MYB protein-positive, compared with 9.1% (5/55) of non-adenoid cystic carcinomas, and the difference between the two groups was statistically significant (P < 0.01). 2/18 of adenoid cystic carcinomas had duplication of MYB gene by FISH, and all non-adenoid cystic carcinomas were negative although the difference was not statistically significant (P = 0.435). MYB protein expression is a useful diagnostic marker for adenoid cystic carcinomas in its separation from other salivary gland tumors. In addition, duplication of MYB gene is no a major mechanism for the MYB protein overexpression.

Failure of the Cystic Fibrosis Transmembrane Conductance Regulator to Conduct ATP

NASA Astrophysics Data System (ADS)

Reddy, M. M.; Quinton, P. M.; Haws, C.; Wine, J. J.; Grygorczyk, R.; Tabcharani, J. A.; Hanrahan, J. W.; Gunderson, K. L.; Kopito, R. R.

1996-03-01

The cystic fibrosis transmembrane conductance regulator (CFTR) is chloride ion channel regulated by protein kinase A and adenosine triphosphate (ATP). Loss of CFTR-mediated chloride ion conductance from the apical plasma membrane of epithelial cells is a primary physiological lesion in cystic fibrosis. CFTR has also been suggested to function as an ATP channel, although the size of the ATP anion is much larger than the estimated size of the CFTR pore. ATP was not conducted through CFTR in intact organs, polarized human lung cell lines, stably transfected mammalian cell lines, or planar lipid bilayers reconstituted with CFTR protein. These findings suggest that ATP permeation through the CFTR is unlikely to contribute to the normal function of CFTR or to the pathogenesis of cystic fibrosis.

Highlights from the 2017 North American Cystic Fibrosis Conference.

PubMed

Martiniano, Stacey L; Toprak, Demet; Ong, Thida; Zemanick, Edith T; Daines, Cori L; Muhlebach, Marianne S; Esther, Charles R; Dellon, Elisabeth P

2018-04-16

The 31st annual North American Cystic Fibrosis Conference (NACFC) was held in Indianapolis, IN on November 2-4, 2017. Abstracts of presentations from the conference were published in a supplement to Pediatric Pulmonology [2017; Pediatr Pulmonol Suppl. 52: S1-S776]. The current review summarizes several major topic areas addressed at the conference: the pathophysiology and basic science of cystic fibrosis (CF) lung disease, clinical trials, clinical management issues, and quality improvement (QI). In this review, we describe emerging concepts in several areas of CF research and care. © 2018 Wiley Periodicals, Inc.

Rehabilitation of an Advanced Case of Adenoid Cystic Carcinoma

PubMed Central

Volpato, Luiz Evaristo Ricci; Caldas, Lorena Frange; Castro, Paulo Henrique de Souza; de Carvalhosa, Artur Aburad; Volpato, Maria Carmen Palma Faria; Bandéca, Matheus Coelho; Borges, Álvaro Henrique

2015-01-01

Adenoid cystic carcinoma is a cancer of the salivary gland that primarily affects the parotid, submandibular, and accessory salivary glands. Its growth is slow and it has infiltrative nature. A 46-year-old female patient coming from the rural area presented a lesion on the palate and reported pain in the region for three years. After incisional biopsy, and histopathological diagnosis of adenoid cystic carcinoma of the cribriform type of minor salivary gland, superior hemimaxillectomy and adjuvant treatment with radiotherapy and maxillofacial prosthetic rehabilitation were performed. PMID:25709844

Incidental late diagnosis of cystic fibrosis following AH1N1 influenza virus pneumonia: a case report.

PubMed

Iadevaia, Carlo; Iacotucci, Paola; Carnovale, Vincenzo; Calabrese, Cecilia; Rea, Gaetano; Ferrara, Nicola; Perrotta, Fabio; Mazzarella, Gennaro; Bianco, Andrea

2017-10-01

Cystic fibrosis is an autosomal recessive disorder characterized by chronic progressive multisystem involvement. AH1N1 virus infections caused classic influenza symptoms in the majority of cystic fibrosis patients while others experienced severe outcomes. We report a case of late incidental cystic fibrosis diagnosis in a young Caucasian man suffering from respiratory failure following infection due to AH1N1 influenza virus. The patient was admitted to our department with fever, cough, and dyspnea at rest unresponsive to antibiotics CONCLUSIONS: Late diagnosis of cystic fibrosis in uncommon. This report highlights the importance of early cystic fibrosis diagnosis to minimize risk of occurrence of potential life-threatening complications.

Imprinted expression in cystic embryoid bodies shows an embryonic and not an extra-embryonic pattern

PubMed Central

Kulinski, Tomasz M.; Casari, M. Rita T.; Guenzl, Philipp M.; Wenzel, Daniel; Andergassen, Daniel; Hladik, Anastasiya; Datlinger, Paul; Farlik, Matthias; Theussl, H. -Christian; Penninger, Josef M.; Knapp, Sylvia; Bock, Christoph; Barlow, Denise P.; Hudson, Quanah J.

2015-01-01

A large subset of mammalian imprinted genes show extra-embryonic lineage (EXEL) specific imprinted expression that is restricted to placental trophectoderm lineages and to visceral yolk sac endoderm (ysE). Isolated ysE provides a homogenous in vivo model of a mid-gestation extra-embryonic tissue to examine the mechanism of EXEL-specific imprinted gene silencing, but an in vitro model of ysE to facilitate more rapid and cost-effective experiments is not available. Reports indicate that ES cells differentiated into cystic embryoid bodies (EBs) contain ysE, so here we investigate if cystic EBs model ysE imprinted expression. The imprinted expression pattern of cystic EBs is shown to resemble fetal liver and not ysE. To investigate the reason for this we characterized the methylome and transcriptome of cystic EBs in comparison to fetal liver and ysE, by whole genome bisulphite sequencing and RNA-seq. Cystic EBs show a fetal liver pattern of global hypermethylation and low expression of repeats, while ysE shows global hypomethylation and high expression of IAPEz retroviral repeats, as reported for placenta. Transcriptome analysis confirmed that cystic EBs are more similar to fetal liver than ysE and express markers of early embryonic endoderm. Genome-wide analysis shows that ysE shares epigenetic and repeat expression features with placenta. Contrary to previous reports, we show that cystic EBs do not contain ysE, but are more similar to the embryonic endoderm of fetal liver. This explains why cystic EBs reproduce the imprinted expression seen in the embryo but not that seen in the ysE. PMID:25912690

[Endocrine complications of cystic fibrosis in childhood].

PubMed