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Sample records for congenital pouch colon

  1. Congenital pouch colon in girls: Genitourinary abnormalities and their management

    PubMed Central

    Chadha, Rajiv; Khan, Niyaz Ahmed; Shah, Shalu; Pant, Nitin; Gupta, Amit; Choudhury, Subhasis Roy; Debnath, Pinaki Ranjan; Puri, Archana

    2015-01-01

    Aims: To discuss the assessment and management of genitourinary (GU) tract abnormalities in 21 girls with Types I-III congenital pouch colon (CPC), studied over a period of 10 years. Materials and Methods: Assessment included clinical and radiological assessment, examination under anesthesia (EUA), endoscopy of the lower GU tract, and evaluation of the surgical findings, operative procedures for the GU anomalies, and the results of management. Results: Initial examination of the external genitalia showed a “clover-leaf” appearance (n = 6) and a single perineal opening (n = 6). In 9 patients, the openings of the urethra and double vagina were seen, of which a vestibular fistula was seen in 5 and an anterior perineal fistula in 1. Seventeen patients (81%) had urinary incontinence (UI) - partial in 10, and complete in 7. Renal function tests, X-ray sacrum, and abdominal US were normal in all patients. Micturating cystourethrogram (n = 9) showed a wide, bladder neck incompetence (BNI) with reduced bladder capacity in seven patients. EUA and endoscopy revealed a septate vagina in all patients and the urethral opening at a “high” position (n = 14) or at a relatively normal or “low” position (n = 7). In 8 patients, the intervaginal septum was thick and fleshy. Endoscopy showed a short, wide urethra, an open incompetent bladder neck, poorly developed trigone, and reduced bladder capacity in the patients with UI. The fistula from the colonic pouch opened in the proximal urethra (n = 4), high in the vestibule (n = 3), low in the vestibule (n = 8), perineum just posterior to the vestibule (n = 1), and undetermined (n = 5). Vaginoscopy (n = 8) showed normal cervices in all and cervical mucus in 4 patients. The subtypes of CPC were Type I CPC (n = 4), Type II CPC (n = 16), and Type III CPC (n = 1). All 21 patients had uterus didelphys. In four patients with UI, during tubular colorraphy, a segment of the colonic pouch was preserved for later bladder augmentation if

  2. Single stage management of a unique variant of congenital pouch colon with triplet fistula and normal anus.

    PubMed

    Pandey, Vaibhav; Gangopadhyay, Ajay Narayan; Gupta, Dinesh Kumar; Sharma, Shiv Prasad

    2015-01-01

    Congenital pouch colon (CPC) in the female patient presents with highly variable and anomalous anatomy. We herein report the first case of CPC with uterus didelphys having normal anal opening, H-type vestibular fistula, two other fistulous communications between pouch colon and two vagina managed in a single stage with excellent postoperative outcome. PMID:26166988

  3. Single stage management of a unique variant of congenital pouch colon with triplet fistula and normal anus

    PubMed Central

    Pandey, Vaibhav; Gangopadhyay, Ajay Narayan; Gupta, Dinesh Kumar; Sharma, Shiv Prasad

    2015-01-01

    Congenital pouch colon (CPC) in the female patient presents with highly variable and anomalous anatomy. We herein report the first case of CPC with uterus didelphys having normal anal opening, H-type vestibular fistula, two other fistulous communications between pouch colon and two vagina managed in a single stage with excellent postoperative outcome. PMID:26166988

  4. Congenital pouch colon in a girl associated with bilateral atresia of cervix uteri and uterus didelphys.

    PubMed

    Chadha, Rajiv; Puri, Manju; Saxena, Rahul; Agarwala, Surendrakumar; Puri, Archana; Choudhury, Subhasis Roy

    2013-04-01

    This report describes a girl with congenital pouch colon (CPC), uterus didelphys with septate vagina, and a cloacal anomaly. The girl underwent cloacal reconstruction at the age of 15 months. Subsequently, at puberty, the child had primary amenorrhea with severe cyclic abdominal pain due to endometriosis of both the uteruses and adnexal cysts with hematometra and hematosalpinx. Laparotomy with removal of both uteri and the left fallopian tube was performed. Both uteri had atresia of the cervix uteri. This report emphasizes the need for comprehensive evaluation and a long-term management strategy for associated gynecologic anomalies in girls with CPC, especially with regard to patency of the outflow tract. PMID:23798813

  5. Congenital pouch colon in a girl associated with bilateral atresia of cervix uteri and uterus didelphys

    PubMed Central

    Chadha, Rajiv; Puri, Manju; Saxena, Rahul; Agarwala, Surendrakumar; Puri, Archana; Choudhury, Subhasis Roy

    2013-01-01

    This report describes a girl with congenital pouch colon (CPC), uterus didelphys with septate vagina, and a cloacal anomaly. The girl underwent cloacal reconstruction at the age of 15 months. Subsequently, at puberty, the child had primary amenorrhea with severe cyclic abdominal pain due to endometriosis of both the uteruses and adnexal cysts with hematometra and hematosalpinx. Laparotomy with removal of both uteri and the left fallopian tube was performed. Both uteri had atresia of the cervix uteri. This report emphasizes the need for comprehensive evaluation and a long-term management strategy for associated gynecologic anomalies in girls with CPC, especially with regard to patency of the outflow tract. PMID:23798813

  6. Malabsorption and pouch ulcerations following the Martin repair for total colonic aganglionosis.

    PubMed

    Perrault, J; Stockwell, M; Stephens, C; Forstner, G

    1979-08-01

    A late complication of the Martin repair for total colonic aganglionosis is described. Pouch malfunction resulted in stasis and ulceration, excessive blood and protein loss, and bacterial overgrowth of the small intestine. The severity and nature of the lesion was identified by colonoscopy. PMID:490294

  7. Multiple Congenital Colonic Stenosis: A Rare Gastrointestinal Malformation

    PubMed Central

    Elisa, Zambaiti; Cinzia, Chiaramonte; Sergio, Salerno; Giuseppe, Li Voti; Fortunato, Siracusa

    2016-01-01

    Congenital colonic stenosis is a rare pediatric condition. Since 1968, only 16 cases have been reported in the literature. To the authors' knowledge, multiple congenital colonic stenosis has not been previously reported in the literature. We report the case of a 2-month-old male, presented at our Neonatal Intensive Care Unit with a suspicion of intestinal malrotation. Clinical examination revealed persistent abdominal distension. During the enema examination, the contrast medium appeared to fill the lumen of the colon up to three stenotic segments and could not proceed further. Intraoperatively we confirmed the presence of four types of colonic atresia, located in the ascending, transverse, and descending colon, respectively, plus appendix atresia. First surgical steps consisted in resection of proximal stenotic segment, appendix removal, proximal cecostomy, and distal colostomy on ascending colon in order to preserve colonic length. Histopathological examination confirmed the diagnosis of colonic stenosis. Final surgical step consisted in multiple colocolostomy and enteroplasty. A planned two-stage procedure, consisting of resection with colostomy for decompression as the first step and a later anastomosis, is recommended in order to allow bowel length preservation. PMID:27066287

  8. Multiple Congenital Colonic Stenosis: A Rare Gastrointestinal Malformation.

    PubMed

    Elisa, Zambaiti; Cinzia, Chiaramonte; Sergio, Salerno; Giuseppe, Li Voti; Fortunato, Siracusa

    2016-01-01

    Congenital colonic stenosis is a rare pediatric condition. Since 1968, only 16 cases have been reported in the literature. To the authors' knowledge, multiple congenital colonic stenosis has not been previously reported in the literature. We report the case of a 2-month-old male, presented at our Neonatal Intensive Care Unit with a suspicion of intestinal malrotation. Clinical examination revealed persistent abdominal distension. During the enema examination, the contrast medium appeared to fill the lumen of the colon up to three stenotic segments and could not proceed further. Intraoperatively we confirmed the presence of four types of colonic atresia, located in the ascending, transverse, and descending colon, respectively, plus appendix atresia. First surgical steps consisted in resection of proximal stenotic segment, appendix removal, proximal cecostomy, and distal colostomy on ascending colon in order to preserve colonic length. Histopathological examination confirmed the diagnosis of colonic stenosis. Final surgical step consisted in multiple colocolostomy and enteroplasty. A planned two-stage procedure, consisting of resection with colostomy for decompression as the first step and a later anastomosis, is recommended in order to allow bowel length preservation. PMID:27066287

  9. Huge Congenital Segmental Dilatation of the Sigmoid Colon in a Neonate: A “Rarity to Meet” and a “Challenge to Treat”

    PubMed Central

    Kaiser, Margarita; Ratschek, Manfred; Till, Holger

    2016-01-01

    Only ten cases of neonatal congenital segmental dilatation (CSD) of the colon have been described so far. We present a full-term female newborn with trisomy 21, ventricular septal defect, and gross abdominal distension. Plain abdominal radiographs revealed a huge cystic lesion occupying the left hemiabdomen. Upon laparotomy on day 4 a CSD of the distal sigmoid and proximal rectum was confirmed and resected. The proximal colon was exteriorized and the distal part closed as a Hartmann pouch. Histology confirmed a huge segmental dilatation of the sigmoid without dysganglionosis or pseudodiverticula, but normal intestinal architecture. After correction of the ventricular septal defect a low rectal end-to-end anastomosis could be performed at an age of 5 months. The postoperative course was uneventful. CSD of the sigmoid colon is extremely “rare to meet” and a “challenge to treat” in the newborn period, but clinical awareness of this entity prompts pediatric surgical success. PMID:27239360

  10. Colonization of congenitally immunodeficient mice with probiotic bacteria.

    PubMed Central

    Wagner, R D; Warner, T; Roberts, L; Farmer, J; Balish, E

    1997-01-01

    We assessed the capacity of four probiotic bacteria (Lactobacillus acidophilus, Lactobacillus reuteri, Lactobacillus casei GG, and Bifidobacterium animalis) to colonize, infect, stimulate immune responses in, and affect the growth and survival of congenitally immunodeficient gnotobiotic beige-athymic (bg/bg-nu/nu) and beige-euthymic (bg/bg-nu/+) mice. The bacteria colonized and persisted, in pure culture, in the alimentary tracts of both mouse strains for the entire study period (12 weeks). Although all adult and neonatal beige-euthymic mice survived probiotic colonization, some infant mortality occurred in beige-athymic pups born to mothers colonized with pure cultures of L. reuteri or L. casei GG. The probiotic bacteria manifested different capacities to adhere to epithelial surfaces, disseminate to internal organs, affect the body weight of adult mice and the growth of neonatal mice, and stimulate immune responses. Although the probiotic species were innocuous for adults, these results suggest that caution and further studies to assess the safety of probiotic bacteria for immunodeficient hosts, especially neonates, are required. PMID:9234796

  11. Gastric-type extremely well-differentiated adenocarcinoma arising in the blind pouch of a bypassed stomach, presenting as colonic pseudo-obstruction.

    PubMed

    McFarland, Sarah; Manivel, Carlos J; Ramaswamy, Archana; Mesa, Hector

    2015-01-01

    Gastric carcinoma after gastric bypass is rare. Extremely well-differentiated adenocarcinoma (EWDA) of the stomach is a rare variant that has been mostly reported in Japan. We present a case of a 68-year-old man with EWDA arising in the bypassed stomach that presented as a colonic pseudo-obstruction (CPO). Several imaging, endoscopic and pathologic studies performed in the course of 2 months were non-diagnostic. An iatrogenic duodenal perforation during a diagnostic procedure led to an emergent exploratory laparotomy in which the dilated colonic segment was resected. Pathologic examination showed metastatic EWDA in the colonic wall. Post-operative complications led to the patient's demise. At autopsy the primary tumor was identified in the blind pouch of the bypassed stomach. A literature review on gastric EWDA and carcinomas arising in bypassed stomachs is discussed. EWDA of the stomach is rare, difficult to diagnose, and shows an aggressive clinical course discordant with its near-benign histology. Gastric cancer arising in a bypassed stomach is uncommon; when it occurs it is usually diagnosed at advanced stage. Surveillance of the blind pouch is not currently recommended. Malignant infiltration of the colonic wall should be included in the differential diagnosis of CPO of unclear etiology. PMID:26424554

  12. Congenital membrane causing duodenal obstruction and malpositioning of the descending colon.

    PubMed

    Koh, Chee-Chee; Tseng, Sheng-Hong; Weng, Chia-Chi; Chen, Yun

    2013-08-01

    A congenital membrane without intestinal malrotation is a rare cause of duodenal obstruction. Here we present an 11-year-old girl who had suffered from intermittent abdominal cramping pain and vomiting for more than 5 years. The image studies, including a plain abdomen roentgenogram and sonogram, showed no definite diagnosis. The upper gastrointestinal series and small bowel series showed the contrast was static over the third portion of the duodenum and the descending colon pulled up toward the epigastric area. Laparoscopic exploration revealed a congenital membrane extending from the right-side paraduodenal peritoneum through the third portion of the duodenum to the descending colon, which had caused obstruction of the third portion of the duodenum and malpositioning of the descending colon. To the best of our knowledge, this is the first case report in the literature where a congenital membrane caused both duodenal obstruction and malpositioning of the descending colon. PMID:23597513

  13. Colonization of congenitally athymic, gnotobiotic mice by Candida albicans.

    PubMed Central

    Balish, E; Balish, M J; Salkowski, C A; Lee, K W; Bartizal, K F

    1984-01-01

    Colony counts, scanning electron microscopy, and light microscopy were used to assess the capacity of Candida albicans to colonize (naturally) and infect the alimentary tract of adult and neonatal (athymic [nu/nu] or heterozygous [+/nu] littermates) germfree BALB/c mice. When exposed to yeast-phase C. albicans, the alimentary tract of adult germfree mice (nu/nu or +/nu) is quickly (within 24 to 48 h) colonized with yeast cells. Neither morbidity nor mortality was evident in any mice that were colonized with a pure culture of C. albicans for 6 months. Yeast cells of C. albicans predominated on mucosal surfaces in the oral cavities and vaginas of adult athymic and heterozygous mice. In both genotypes, C. albicans hyphae were observed in keratinized tissue on the dorsal posterior tongue surface and in the cardial-atrium section of the stomach. Conversely, neonatal athymic or heterozygous mice, born to germfree or C. albicans-colonized mothers, do not become heavily colonized or infected with C. albicans until 11 to 15 days after birth. Although yeast cells adhered to some mucosal surfaces in vivo, neither widespread mucocutaneous candidiasis, i.e., invasion of mucosal surfaces with C. albicans hyphae, nor overwhelming systemic candidiasis was evident in neonatal (nu/nu or +/nu) mice. Thus, even in the absence of functional T-cells and a viable bacterial flora, athymic and heterozygous littermate mice (adult or neonatal BALB/c) that are colonized with a pure culture of C. albicans manifest resistance to extensive mucocutaneous and systemic candidiasis. Images PMID:6372689

  14. Changing your ostomy pouch

    MedlinePlus

    The bathroom is a good place to change your pouch. Empty your used pouch into the toilet first, if it needs emptying. Gather your supplies. If you have a 2-piece pouch, be sure you have the special ...

  15. Heterotopic nephrogenic rests in the colon and multiple congenital anomalies: possibly related association.

    PubMed

    Jain, Dhanpat; Martel, Maritza; Reyes-Múgica, Miguel; Parkash, Vinita

    2002-01-01

    Heterotopic renal tissue (HRT) in the wall of the colon is a very rare occurrence, with only five cases published. Our patient is only the second patient reported to have this abnormality in the absence of sirenomelia. We describe colonic HRT in a child, associated with multiple congenital anomalies. The congenital abnormalities were of the VACTERL type, accompanied by valvular cardiac anomalies that were clinically diagnosed as Shone syndrome. The HRT was not apparent clinically or grossly. Microscopically, multifocal islands of renal tissue consisting of glomeruli, cystically dilated tubules, and blastema were seen in all layers of the bowel, and simulated "cystic partially differentiated nephroblastoma." Our case provides further support to the belief that VACTERL association and sirenomelia represent related entities. PMID:12375130

  16. Radiolocalization of monoclonal antibodies in hepatic metastases from human colon cancer in congenitally athymic mice

    SciTech Connect

    Yoshida, K.; Rivoire, M.; Divgi, C.; Welt, S.; Cohen, A.M.; Sigurdson, E.R. )

    1990-02-01

    Intrasplenic injection of the HT-29 LMM metastatic human colon cancer line reproducibly results in hepatic metastasis formation in congenitally athymic mice. HT-29-15, a murine monoclonal antibody (mAb) of the IgG1 class reactive with the HT-29 LMM line, and BL-3, an isotype-matched control antibody, were labeled with 125I. Labeled mAbs were injected i.v. in mice with hepatic metastases, and animals were sacrificed on days 3, 5, and 7. Specific mAb uptake by tumor was significantly greater than nonspecific mAb uptake, as evidenced by specific/nonspecific tumor/blood ratios (radiolocalization indices) of 3.47/1-25.6/1. Relative mAb uptake was greater by the hepatic tumors than by the splenic tumors from day 3 to day 7, although this was significant (P less than 0.05) only on day 7 (5.12 {plus minus} 2.97 versus 1.79 {plus minus} 0.71). Tumor/uninvolved tissue ratios were also significantly greater (P less than 0.05) for the hepatic metastases than for the splenic tumors on day 7 (12.23 {plus minus} 3.85 versus 6.63 {plus minus} 2.63). This murine hepatic metastasis model appears useful for evaluation of localization of mAbs to hepatic metastases from human colon carcinoma.

  17. Pharyngeal pouch carcinoma.

    PubMed

    Saunders, M W; Murty, G E; Bradely, P J

    1993-02-01

    Malignant change occurs in 0.3-10% of pharyngeal pouches, with longstanding pouches most at risk. Contrast radiology and endoscopy can detect large and medium sized tumors but are inadequate for small lesions and carcinoma in situ. Expectant treatment and conservative surgery fail to provide an excised pouch for histological analysis and small lesions may be missed. Consequently, careful consideration must be given to radical excision, particularly if the pouch has been longstanding. The role of radiotherapy in conjunction with surgery remains unproven. PMID:8482256

  18. Changing your ostomy pouch

    MedlinePlus

    American College of Surgeons, Division of Education. Ostomy skills: Emptying and changing the pouch. Available at: www. ... Perry AG, Potter PA, Ostendorf W. Clinical Nursing Skills and Techniques . 8th ed. Philadelphia, PA: Elsevier Mosby; ...

  19. Crohn's Disease of the Ileoanal Pouch.

    PubMed

    Lightner, Amy L; Pemberton, John H; Loftus, Edward J

    2016-06-01

    Crohn's disease (CD) of the pouch is an increasingly recognized diagnosis after ileal pouch-anal anastomosis. This post-ileal pouch-anal anastomosis diagnosis in conjunction with pouchitis remains the leading reason for pouch excision. Unfortunately, CD of the pouch remains a difficult diagnosis with lack of a uniform definition largely because of its similarity to common postoperative pouch complications, including pouchitis, abscess formation, or stricture at the anastomosis. Once diagnosed, treatment algorithms largely include multimodal therapy including biologics. This review focuses on the definition, etiology, diagnosis, and treatment for CD of the pouch, a postoperative de novo diagnosis of CD. PMID:27057684

  20. Subsequent Adenomas of Ileal Pouch and Anorectal Segment after Prophylactic Surgery for Familial Adenomatous Polyposis

    PubMed Central

    M'Koma, A.E.; Herline, A.J.; Adunyah, S.E.

    2014-01-01

    Familial adenomatous polyposis (FAP) is an autosomally dominant disease characterized by the early development of colorectal adenomas and carcinoma in untreated patients. Patients with FAP may develop rectal cancer at their initial presentation (primary) or after prophylactic surgery (secondary). Controversies exist regarding which surgical procedure represents the best first-line treatment. The options for FAP are ileorectal anastomosis (IRA) or a restorative proctocolectomy (RPC) with either a handsewn or a stapled ileal pouch-anal anastomosis (IPAA), with or without mucosectomy. The purpose of these surgeries is to stop progression to an adenoma-cancer sequence by eradicating the colon, a disease prone organ. Unfortunately, these surgical procedures, which excise the entire colon and rectum while maintaining transanal fecal continence, do not guarantee that patients still won't develop adenomas. Based on the available literature, we therefore reviewed reported incidences of pouch-related adenomas that occurred post prophylactic surgery for FAP. The review consists of a collection of case, descriptive, prospective and retrospective reports. Objectives To provide available data on the natural history of subsequent adenomas after prophylactic surgery (by type) for FAP. Methods A review was conducted of existing case, descriptive, prospective and retrospective reports for patients undergoing prophylactic surgery for FAP (1975 – August, 2013). In each case, the adenomas were clearly diagnosed in one of the following: the ileal pouch mucosa (above the ileorectal anastomosis), within the anorectal segment (ARS) below the ileorectal anastomosis, or in the afferent ileal loop. Results A total of 515 (36%) patients with pouch-related adenomas have been reported. Two hundred and eleven (211) patients had adenomas in the ileal pouch mucosa, 295 had them in the ARS and in 9 were in the afferent ileal loop. Patients with pouch adenomas without dysplasia or cancer were

  1. Laparoscopic restorative proctocolectomy ileal pouch anal anastomosis: How I do it?

    PubMed Central

    Madnani, Manish A; Mistry, Jitendra H; Soni, Harshad N; Shah, Atul J; Patel, Kantilal S; Haribhakti, Sanjiv P

    2015-01-01

    Surgery for ulcerative colitis is a major and complex colorectal surgery. Laparoscopy benefits these patients with better outcomes in context of cosmesis, pain and early recovery, especially in young patients. For surgeons, it is a better tool for improving vision and magnification in deep cavities. This is not the simple extension of the laparoscopy training. Starting from preoperative preparation to post operative care there are wide variations as compared to open surgery. There are also many variations in steps of laparoscopic surgery. It involves left colon, right colon and rectal mobilisation, low division of rectum, pouch creation and anastomosis of pouch to rectum. Over many years after standardisation of this technique, it takes same operative time as open surgery at our centre. So we present our standardized technique of laparoscopic assisted restorative proctocolectomy and ileal pouch anal anastomosis (IPAA). PMID:26195886

  2. Novel isolated cecal pouch model for endoscopic observation in rats

    PubMed Central

    Koshino, Kurodo; Kanai, Nobuo; Yamato, Masayuki; Okano, Teruo; Yamamoto, Masakazu

    2015-01-01

    AIM: To create a new rat model for drug administration, cell transplantation, and endoscopic examination for the treatment of intestinal diseases. METHODS: F344/NJc l-rnu/rnu rats (10-wk-old males, 350-400 g) were used in this study. The rats were anesthetized via 2% isoflurane inhalation. The rat’s cecum was isolated from the intestines, and a pouch was created. The remainder of the intestines was rejoined to create an anastomosis. The “side-to-side” anastomosis (SSA) technique initially involves the creation of a 2-cm longitudinal incision into each intestinal wall. To create an anastomosis along the ileal and colonic walls, both intestines were cut, and a continuous suture procedure was performed that included all layers of both intestines. The serous membrane was sutured along the edge and on the anterior wall of the anastomosis. The “end-to-end” anastomosis (EEA) technique was compared with the SSA technique. In the EEA technique, the frontal surfaces of both cut intestinal lumens were joined together by continuous sutures. Additional sutures were made at the serosa. After the anastomotic intestine was successfully constructed, the two intestinal lumens that were cut at the isolated cecum were managed. In addition, one luminal side of the pouch remained open to create an artificial anus on the dorsum as a passage for the residual substances in the pouch. Finally, small animal endoscopy was used to observe the inside of the pouch. RESULTS: In this animal model, mucus and feces are excreted through the reconstructed passage. Accordingly, the cecal pouch mucosa was not obstructed or contaminated by feces, thus facilitating observations of the luminal surface of the intestine. The endoscopic observation of the cecal pouch provided clear visualization given the absence of feces. The membrane surface of the cecum was clearly observed. Two methods of creating an anastomotic intestine, the “SSA” and “EEA” techniques, were compared with regard to

  3. A suprasellar subarachnoid pouch; aetiological considerations.

    PubMed Central

    Binitie, O; Williams, B; Case, C P

    1984-01-01

    A child with hydrocephalus treated by a valved shunt was reinvestigated after developing a shunt infection. A pouch was discovered invaginating the floor of the third ventricle and filling slowly with CSF from the region of the interpeduncular cistern. Histology and mechanisms of this pouch formation are discussed. Images PMID:6502163

  4. Tuberculosis Detection by Giant African Pouched Rats

    ERIC Educational Resources Information Center

    Poling, Alan; Weetjens, Bart; Cox, Christophe; Beyene, Negussie; Durgin, Amy; Mahoney, Amanda

    2011-01-01

    In recent years, operant discrimination training procedures have been used to teach giant African pouched rats to detect tuberculosis (TB) in human sputum samples. This article summarizes how the rats are trained and used operationally, as well as their performance in studies published to date. Available data suggest that pouched rats, which can…

  5. Experimental Infection of Sheep at 45 and 60 Days of Gestation with Schmallenberg Virus Readily Led to Placental Colonization without Causing Congenital Malformations

    PubMed Central

    Dal Pozzo, Fabiana; De Regge, Nick; Cay, Brigitte; Saegerman, Claude

    2015-01-01

    Background Main impact of Schmallenberg virus (SBV) on livestock consists in reproductive disorders, with teratogenic effects, abortions and stillbirths. SBV pathogenesis and viral placental crossing remain currently poorly understood. Therefore, we implemented an experimental infection of ewes, inoculated with SBV at 45 or 60 days of gestation (dg). Methodology “Mourerous” breed ewes were randomly separated in three groups: eight and nine ewes were subcutaneously inoculated with 1 ml of SBV infectious serum at 45 and 60 dg, respectively (G45 and G60). Six other ewes were inoculated subcutaneously with sterile phosphate buffer saline as control group. All SBV inoculated ewes showed RNAemia consistent with previously published studies, they seroconverted and no clinical sign was reported. Lambs were born at term via caesarian-section, and right after birth they were blood sampled and clinically examined. Then both lambs and ewes were euthanatized and necropsied. Principal Findings/Significance No lambs showed any malformation suggestive of SBV infection and none of them had RNAemia or anti-SBV antibodies prior to colostrum uptake. Positive SBV RNA detection in organs was rare in both G45 and G60 lambs (2/11 and 1/10, respectively). Nevertheless most of the lambs in G45 (9/11) and G60 (9/10) had at least one extraembryonic structure SBV positive by RTqPCR. The number of positive extraembryonic structures was significantly higher in G60 lambs. Time of inoculation (45 or 60 dg) had no impact on the placental colonization success rate but affected the frequency of detecting the virus in the offspring extraembryonic structures by the time of lambing. SBV readily colonized the placenta when ewes were infected at 45 or 60 dg but infection of the fetuses was limited and did not lead to congenital malformations. PMID:26418420

  6. Postnatal development of Blake's pouch cyst: a case report and new insight for its pathogenesis.

    PubMed

    Hirono, Seiichiro; Ito, Daisuke; Murai, Hisayuki; Kobayashi, Masayoshi; Suyama, Maiko; Fujii, Katsunori; Saeki, Naokatsu

    2014-10-01

    Blake's pouch cyst (BPC), a rare cystic malformation in the posterior fossa, is believed to be caused by the congenital expansion of the posterior membranous area that normally regresses during embryogenesis. However, due to the wide spectrum of the onset pattern and age of patients, the natural history and the pathogenesis are poorly understood. The authors describe the case of a girl who admitted with headache and right abducens nerve paresis at the age of 3 years and 10 months. Magnetic resonance (MR) imaging demonstrated a tetraventricular hydrocephalus, an open aqueduct, and a posterior fossa cyst compatible with BPC. Multiple tumors were also noticed in the ventricular wall. Tumor biopsy and an endoscopic third ventriculostomy were performed. Intraoperative observation confirmed the BPC, and pathological diagnosis was pilomyxoid astrocytoma. In retrospect, MR imaging was performed twice in the past, at the age of 8 months and again at 22 months, and no anomaly was detected, suggesting that Blake's pouch was once regressed. Therefore, a BPC in this patient was certainly developed after her second or third year of life. The ventricular tumors may influence the cerebrospinal fluid (CSF) absorption, which triggered the re-expansion of BPC from the possible remnant of Blake's pouch. This is a rare but important report providing evidence that in addition to the classic congenital BPC in which the remnant of Blake's pouch remains persistent, there could be postnatal or secondary BPC, which develops after birth. Possible mechanisms include that the remnant of Blake's pouch, which originally disappears, may re-expand postnatally in association with unknown trigger or a change in CSF dynamics or absorption. PMID:24907110

  7. Models of Inflammation: Carrageenan Air Pouch.

    PubMed

    Duarte, Djane B; Vasko, Michael R; Fehrenbacher, Jill C

    2016-01-01

    The subcutaneous air pouch is an in vivo model that can be used to study the components of acute and chronic inflammation, the resolution of the inflammatory response, the oxidative stress response, and potential therapeutic targets for treating inflammation. Injection of irritants into an air pouch in rats or mice induces an inflammatory response that can be quantified by the volume of exudate produced, the infiltration of cells, and the release of inflammatory mediators. The model presented in this unit has been extensively used to identify potential anti-inflammatory drugs. © 2016 by John Wiley & Sons, Inc. PMID:26995549

  8. Local regulation of postprandial motor responses in ileal pouches

    PubMed Central

    Mularczyk, A; Contessini-Avesan..., E; Cesana, B; Bianchi, P; Basilisco, G

    1999-01-01

    BACKGROUND—Local mechanisms are involved in the postprandial regulation of ileal tone in healthy subjects, but whether these mechanisms affect the postprandial tonic response of ileal pouches has not yet been investigated.
AIMS—To study the effect of a meal on pouch tone and phasic motor activity in patients with gut continuity or ileostomy and, in the latter group, the effect of a pouch perfusion with chyme or saline.
PATIENTS—Twenty patients with ileal pouches: 10 with gut continuity and 10 with ileostomy.
METHODS—Pouch tone and the frequency of phasic volume events were recorded with a barostat under fasting and postprandial conditions and after perfusion of the isolated pouch with chyme or saline.
RESULTS—The meal increased pouch tone and the frequency of phasic volume events in the patients with gut continuity, but not in those with ileostomy. Pouch perfusion with chyme induced a greater increase in pouch tone than saline.
CONCLUSIONS—The meal stimulated pouch tone and phasic motor activity. These effects were at least partially related to local pouch stimulation by intraluminal contents.


Keywords: ileal pouches; postprandial motor responses; ileal tone; ileostomy; motor activity; barostat PMID:10486368

  9. A Case of In-Bore Transperineal MRI-Guided Prostate Biopsy of a Patient with Ileal Pouch-Anal Anastomosis

    PubMed Central

    Kongnyuy, Michael; Frye, Thomas; George, Arvin K.; Kilchevsky, Amichai; Iyer, Amogh; Kadakia, Meet; Muthigi, Akhil; Turkbey, Baris; Wood, Brad J.; Pinto, Peter A.

    2015-01-01

    Ulcerative colitis (UC) is an inflammatory disease that specifically affects the colon. Ulcerative colitis is primarily treated medically and refractory disease is treated with proctocolectomy and ileal pouch-anal anastomosis (IPAA). Gastroenterologists advise against digital rectal exams, pelvic radiation therapy, and transrectal ultrasound (TRUS) biopsies of the prostates of ileal pouch-anal anastomosis patients. Any form of pouch manipulation can lead to severe bleeding, inflammation, and pain. Urologists are therefore faced with the challenge of doing a prostate biopsy without a transrectal ultrasound. We report the rare case of a patient with an ileal pouch-anal anastomosis who underwent in-bore transperineal MRI-guided biopsy of the prostate. PMID:26844005

  10. 31 CFR 538.516 - Diplomatic pouches.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... under the Export Administration Regulations (15 CFR parts 730 et seq.). ... authorized: (a) The importation into the United States from Sudan, or the exportation from the United States to Sudan, of diplomatic pouches and their contents; and (b) The exportation or...

  11. 31 CFR 538.516 - Diplomatic pouches.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... under the Export Administration Regulations (15 CFR parts 730 et seq.). ... authorized: (a) The importation into the United States from Sudan, or the exportation from the United States to Sudan, of diplomatic pouches and their contents; and (b) The exportation or...

  12. 31 CFR 538.516 - Diplomatic pouches.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... under the Export Administration Regulations (15 CFR parts 730 et seq.). ... authorized: (a) The importation into the United States from Sudan, or the exportation from the United States to Sudan, of diplomatic pouches and their contents; and (b) The exportation or...

  13. 31 CFR 538.516 - Diplomatic pouches.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... under the Export Administration Regulations (15 CFR parts 730 et seq.). ... authorized: (a) The importation into the United States from Sudan, or the exportation from the United States to Sudan, of diplomatic pouches and their contents; and (b) The exportation or...

  14. 31 CFR 538.516 - Diplomatic pouches.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... under the Export Administration Regulations (15 CFR parts 730 et seq.). ... authorized: (a) The importation into the United States from Sudan, or the exportation from the United States to Sudan, of diplomatic pouches and their contents; and (b) The exportation or...

  15. 31 CFR 560.521 - Diplomatic pouches.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... Commerce under the Export Administration Regulations (15 CFR parts 730 through 774). ... authorized: (a) The importation into the United States from Iran, or the exportation from the United States to Iran, of diplomatic pouches and their contents; and (b) The exportation, reexportation, sale,...

  16. 31 CFR 560.521 - Diplomatic pouches.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... Commerce under the Export Administration Regulations (15 CFR parts 730 et seq.). ... authorized: (a) The importation into the United States from Iran, or the exportation from the United States to Iran, of diplomatic pouches and their contents; and (b) The exportation, reexportation, sale,...

  17. 31 CFR 560.521 - Diplomatic pouches.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... Commerce under the Export Administration Regulations (15 CFR parts 730 through 774). ... authorized: (a) The importation into the United States from Iran, or the exportation from the United States to Iran, of diplomatic pouches and their contents; and (b) The exportation, reexportation, sale,...

  18. 31 CFR 560.521 - Diplomatic pouches.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... Commerce under the Export Administration Regulations (15 CFR parts 730 et seq.). ... authorized: (a) The importation into the United States from Iran, or the exportation from the United States to Iran, of diplomatic pouches and their contents; and (b) The exportation, reexportation, sale,...

  19. 31 CFR 560.521 - Diplomatic pouches.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... Commerce under the Export Administration Regulations (15 CFR parts 730 et seq.). ... authorized: (a) The importation into the United States from Iran, or the exportation from the United States to Iran, of diplomatic pouches and their contents; and (b) The exportation, reexportation, sale,...

  20. Characterization of Commercial Li-ion Cells in Pouch Format

    NASA Technical Reports Server (NTRS)

    Jeevarajan, Judith

    2014-01-01

    The li-ion pouch design cells exhibit similar behavior under off-nominal conditions as those in metal cans that do not have the internal safety devices. Safety should be well characterized before batteries are designed. Some of the li-ion pouch cell designs studied in this program reacted most violently to overcharge conditions at the medium rates but were tolerant to overcharge at very low rates. Some pouch cell designs have higher tolerance to vacuum exposures than some others. A comparison of the pouch material itself does not show a correlation between this tolerance and the number of layers or composition of the pouch indicating that this is a property of the electrode stack design inside the pouch. Reduced pressure (8 to 10 psi) test environments show that the extent of capacity degradation under reduced pressure environments is much less than that observed under vacuum conditions. Lithium-ion Pouch format cells are not necessarily true polymer cells.

  1. Congenital Hypothyroidism

    MedlinePlus

    ... Body in Balance › Congenital Hypothyroidism Fact Sheet Congenital Hypothyroidism March, 2012 Download PDFs English Espanol Editors Rosalind S. ... MD Susan R. Rose, MD What is congenital hypothyroidism? Newborn babies who are unable to make enough ...

  2. Absent upper blind Pouch in a case of tracheo-esophageal fistula.

    PubMed

    Harjai, Man Mohan; Badal, Sachendra; Khanna, Sangeeta; Singh, Ajit Kumar

    2015-01-01

    A common upper airway and digestive tract is a rare congenital anomaly that is usually fatal and its exact incidence is not known. It is a diagnostic challenge as it requires high index of suspicion. It should be considered in a neonate with respiratory distress in a non-vigorous baby requiring endotracheal intubation, which is difficult even in expert hand. We present a newborn with suspected tracheo-esophageal fistula that was diagnosed intraoperatively to have absent upper blind pouch of the esophagus and on autopsy found to have laryngeal atresia with absent vocal cords and a common aerodigestive tract continuing distally with trachea. The neonate was ventilated with endotracheal tube (ETT) placement which in retrospect we came to know that it was in the esophagus. The neonate also had associated multiple congenital anomalies of VACTERL association. The importance of teamwork between neonatologist, pediatric surgeon, anesthesiologist, and radiologist is highlighted for diagnosis and management of such rare cases. PMID:25552830

  3. Solitary Pouch Ulcer: A New Clinical Entity?

    PubMed

    Pricolo, Victor E

    2016-07-01

    Solitary rectal ulcer syndrome is a well-known clinical entity, likely secondary to a defecatory dysfunction. In patients who have undergone restorative proctocolectomy with ileoanal reservoir, it is conceivable that a similar pathophysiology may lead to "solitary pouch ulcer," but such a syndrome has not been reported to date. This article reports 2 such cases and clinical success with lasting symptomatic relief through local therapy and behavior modification rather than anti-inflammatory. PMID:26859123

  4. Granuloma pouch assay for mutagenicity testing.

    PubMed

    Maier, P

    1980-11-01

    The Granuloma Pouch Assay (GPA) is an animal model in which mutagenic and carcinogenic effects of a testcompound can be detected in rapidly dividing fibroblasts of a granulation tissue in adult male rats. Growth of this tissue was initiated with a small amount of croton oil at the inside wall of a subcutaneous air pouch on the back of the animals. The test compound can be injected either into the pouch (local) or administered by systemic routes. Alkali labile DNA-lesions, chromosome aberrations, sister chromatid exchanges, point mutations and tumor development in situ were determined. The comparison of mutation frequencies after local and systemic administration of testcompounds, provide an estimation of the pharmacokinetic characteristics and the mutagenic potency of the chemical. The local application route allows the detection of locally active mutagens and of compounds which require activation by P-448 dependent mono-oxygenases. Liver mediated proximate metabolites are detectable when they are transformed into ultimate carcinogens in extrahepatic cells whereas chemicals with a strong organ specific activity are not. PMID:7235991

  5. Analysis of autonomic nerve preservation and pouch reconstruction influencing fragmentation of defecation after sphincter-preserving surgery for rectal cancer.

    PubMed

    Katsumata, K; Sumi, T; Enomoto, M; Mori, Y; Aoki, T

    2010-01-01

    Our questionnaire survey on defecation disorders after rectal cancer surgery revealed that 66.7% of postoperative patients were most annoyed with fragmentation of defecation. Therefore, we performed a change-over-time analysis on the relationship of fragmentation and factors including location of rectal cancer, surgical technique, anastomosis method, pouch reconstruction, extent of lymph node dissection, and degree of pelvic and colonic nerve preservation surrounding the superior mesenteric artery. The fragmentation decreased over time at the postoperative time points of 6 months, 2 and 5 years. A statistical analysis of factors influencing fragmentation revealed that location of cancer, reconstruction technique, anastomosis method and degree of pelvic nerve preservation were significant factors for the entire patient population and that colonic nerve preservation was a significant factor 5 years after surgery. Analysis of patients with lower rectal cancer only showed that in addition to surgical technique and anastomosis method, pouch reconstruction was effective and autonomic nerve preservation was effective 5 years after surgery. As a result, when the anastomotic site was closer to the anus, the frequency of fragmentation increased; we concluded that pouch reconstruction was an effective surgical technique and colonic nerve preservation was effective in the longer term. PMID:21051900

  6. Why do central arachnoid pouches expand?

    PubMed Central

    Williams, Bernard; Guthkelch, A. N.

    1974-01-01

    Three cases of progressive hydrocephalus, two occurring in infants and one in a 12 year old girl who also exhibited precocious puberty, were found to be associated with large arachnoid pouches originating within the posterior fossa. The pathogenesis of such cysts is discussed with special reference to the possibility that their progressive distension results from CSF pulsations of venous origin. Both a direct method of treatment (opening the cyst into the adjacent subarachnoid space) and an indirect one (insertion of a ventriculo-atrial shunt) have been used with success. Images PMID:4548436

  7. Guttural pouch mycosis in a 6-month-old filly

    PubMed Central

    2006-01-01

    Abstract A 6-month-old filly was presented with unilateral epistaxis. Based on clinical signs, endoscopic examination, and postmortem examination, guttural pouch mycosis was diagnosed. The young age of the filly and the fact that this was the 2nd diagnosis of guttural pouch mycosis on this farm was unusual. PMID:16604984

  8. Pouch Method for the Isolation and Enumeration of Clostridia

    PubMed Central

    Bladel, B. O.; Greenberg, Richard A.

    1965-01-01

    An anaerobic film-pouch method has been developed for the isolation and enumeration of clostridia. Fabrication of the pouch is described. Counts of spore suspensions of Putrefactive Anaerobe 3679 and of Clostridium botulinum strains 41-B and 33-A in pouches were compared with those obtained by anaerobic-jar and agar-deep techniques. Statistical analysis revealed a significant difference in favor of the pouch over the tube and anaerobic-jar methods. Tests performed with C. welchii, both in spore suspension and added to chicken pot pie in culture form, also demonstrated the pouch to be at least as proficient as the other, more cumbersome, techniques. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 Fig. 6 PMID:14325895

  9. Santosh PGI pouch: A new innovation in urinary diversion

    PubMed Central

    Devana, Sudheer Kumar; Sharma, Aditya Prakash; Singh, Shrawan Kumar

    2015-01-01

    Introduction To know the feasibility and outcome of the Santosh PGI pouch as a new innovative technique of continent cutaneous diversion (CCD) following cystectomy. Material and methods Twelve patients (eleven with carcinoma of the bladder and one with an exstrophy of the bladder) underwent CCD using the Santosh PGI pouch after cystectomy. A 50-cm segment of terminal ileum was isolated 15-20 cm proximal to the ileocecal junction. The ileal segment was folded into the form of an ‘S’ configuration. On the antimesenteric border three longitudinal incisions were performed of about 7 cm in length. The terminal 8 cm portion of the distal part of the pouch was used for creating the intussuscepted nipple valve. Demucosalization of the interior of the nipple, fixing the nipple valve with the serosa of the pouch wall and wrapping of the catheterizable channel with a pouch wall for providing continence was done. The uretero-pouch anastomosis was done using the serosal lined tunnel technique. The catheterizable channel was brought out through the right rectus muscle. Results Median follow-up of the patients was 13.5 months. No significant complications were noted in the pouch reconstruction. Duration of the pouch reconstruction was around 75-110 min. Postoperatively, one patient had a UTI and another had paralytic ileus on the follow-up. All patients were doing regular CIC with acceptable continence of up to 400 ml. No ureteroileal anastomotic stricture or difficulty in catheterizing the pouch was seen. Conclusions The Santosh PGI pouch, which is a type of CCD, is technically feasible, easy to reconstruct with acceptable continence and offers minimal morbidity. PMID:26251752

  10. Embryo oxygenation in pipefish brood pouches: novel insights.

    PubMed

    Goncalves, Ines Braga; Ahnesjö, Ingrid; Kvarnemo, Charlotta

    2015-06-01

    The pipefish brood pouch presents a unique mode of parental care that enables males to protect, osmoregulate, nourish and oxygenate the developing young. Using a very fine O2 probe, we assessed the extent to which males of the broad-nosed pipefish (Syngnathus typhle) oxygenate the developing embryos and are able to maintain pouch fluid O2 levels when brooding in normoxia (100% O2 saturation) and hypoxia (40% O2 saturation) for 24 days. In both treatments, pouch fluid O2 saturation levels were lower compared with the surrounding water and decreased throughout the brooding period, reflecting greater offspring demand for O2 during development and/or decreasing paternal ability to provide O2 to the embryos. Male condition (hepatosomatic index) was negatively affected by hypoxia. Larger males had higher pouch fluid O2 saturation levels compared with smaller males, and levels were higher in the bottom section of the pouch compared with other sections. Embryo size was positively correlated with O2 availability, irrespective of their position in the pouch. Two important conclusions can be drawn from our findings. First, our results highlight a potential limitation to brooding within the pouch and dismiss the notion of closed brood pouches as well-oxygenated structures promoting the evolution of larger eggs in syngnathids. Second, we provide direct evidence that paternal care improves with male size in this species. This finding offers an explanation for the documented strong female preference for larger partners because, in terms of oxygenation, the brood pouch can restrict embryo growth. PMID:26041030

  11. Congenital hemangiomas.

    PubMed

    Boull, Christina; Maguiness, Sheilagh M

    2016-03-01

    Congenital hemangiomas are rare solitary vascular tumors that do not proliferate after birth. They are characterized as either rapidly involuting congenital hemangiomas (RICHs) or noninvoluting congenital hemangiomas (NICHs) based on their clinical progression. NICHs have no associated complications, but are persistent. RICH, while usually asymptomatic, may ulcerate or bleed early in their presentation, but involute quickly during the first few months of life. Hepatic RICHs are not associated with cutaneous RICHs, but may result in high-output cardiac failure due to arteriovenous or portovenous shunting. In the following review, the clinical characteristics and current management specific to congenital hemangiomas is discussed. PMID:27607320

  12. Occurrence and structure of epipharyngeal pouches in bears (Ursidae)

    PubMed Central

    WEISSENGRUBER, G. E.; FORSTENPOINTNER, G.; KÜBBER-HEISS, A.; RIEDELBERGER, K.; SCHWAMMER, H.; GANZBERGER, K.

    2001-01-01

    The infrequent mention of epipharyngeal pouches occurring in some species of bears indicates the scarcity of morphological and functional knowledge about these structures. In order to provide precise morphological data on the structure of these remarkable formations and to verify their taxonomic utility, the pharyngeal regions of 1 spectacled bear and 3 brown bears were examined. All these individuals possessed epipharyngeal pouches, which are tubular, blind-ending outpouchings of the caudodorsal pharyngeal wall equipped with respiratory epithelium and a thick layer of elastic fibres. While the spectacled bear and Ursus arctos syriacus possessed a single pouch on the caudodorsal wall of the nasopharynx, in Ursus arctos and Ursus arctos beringianus 2 unequally sized pouches were present. Two additional sacs of smaller size, representing outpouchings of the lateral pharyngeal wall, occurred in the spectacled bear. These findings prove epipharyngeal pouches to be constant and unique morphological features of the family Ursidae, the anatomical features suggesting involvement in the respiratory system most probably in important aspects of ursid phonation. This is the first description of epipharyngeal pouches in the spectacled bear. PMID:11322723

  13. Colonic Polyps

    MedlinePlus

    ... Colonic polyps grow in the large intestine, or colon. Most polyps are not dangerous. However, some polyps ... member with polyps Have a family history of colon cancer Most colon polyps do not cause symptoms. ...

  14. [Congenital thrombophilia].

    PubMed

    Kojima, Tetsuhito

    2016-03-01

    Congenital thrombophilia is a thrombotic diathesis caused by a variety of genetic abnormalities in blood coagulation factors or their inhibitory factors associated with physiological thrombus formation. Patients with congenital thrombophilia often present with unusual clinical episodes of venous thrombosis (occasionally combined with pulmonary embolism, known as venous thromboembolism) at a young age and recurrence in atypical vessels, such as the mesenteric vein and superior sagittal sinus, often with a family history of this condition. Studies in Japan as well as in western countries have shown congenital thrombophilia to be caused by a wide variety of genetic abnormalities in natural anticoagulant proteins, such as antithrombin, protein C, and protein S. However, there may still be many unknown causes of hereditary thrombosis. We recently reported a case of hereditary thrombosis induced by a novel mechanism of antithrombin resistance, that is, congenital thrombophilia caused by a gain-of-function mutation in the gene encoding the coagulation factor prothrombin. PMID:27076244

  15. Congenital toxoplasmosis

    MedlinePlus

    Congenital toxoplasmosis is a group of symptoms that occur when an unborn baby (fetus) is infected with the parasite ... Toxoplasmosis infection can be passed to a developing baby if the mother becomes infected while pregnant. The ...

  16. Congenital Myopathy

    MedlinePlus

    ... arms and legs, droopy eyelids, and problems with eye movements. Weakness often gets worse with time. Central core ... difficulties occur as well. Some children have weakened eye movements. Congenital fiber-type disproportion myopathy is a rare ...

  17. Congenital cataract

    MedlinePlus

    ... of the following birth defects: Chondrodysplasia syndrome Congenital rubella Conradi-Hünermann syndrome Down syndrome (trisomy 21) Ectodermal ... Images Eye Cataract - close-up of the eye Rubella syndrome Cataract References Dahan E. Pediatric cataract surgery. ...

  18. Congenital syphilis

    MedlinePlus

    ... fact that this disease can be cured with antibiotics if caught early, rising rates of syphilis among pregnant women in the United States have increased the number of infants born with congenital syphilis.

  19. Congenital rubella

    MedlinePlus

    ... mother is infected with the virus that causes German measles. Congenital means the condition is present at ... Gershon AA. Rubella virus (German measles). In: Mandell GL, Bennett JE, ... of Infectious Diseases . 8th ed. Philadelphia, PA: Elsevier ...

  20. Congenital rubella

    MedlinePlus

    ... is infected with the virus that causes German measles. Congenital means the condition is present at birth. ... Gershon AA. Rubella virus (German measles). In: Mandell GL, Bennett JE, ... . 8th ed. Philadelphia, PA: Elsevier Churchill Livingstone; ...

  1. Ileal pouch-anal anastomosis: Points of controversy.

    PubMed

    Trigui, A; Frikha, F; Rejab, H; Ben Ameur, H; Triki, H; Ben Amar, M; Mzali, R

    2014-09-01

    Restorative proctocolectomy with ileal pouch-anal anastomosis has become the most commonly used procedure for elective treatment of patients with ulcerative colitis and familial adenomatous polyposis. Since its original description, the procedure has been modified in order to obtain optimal functional results with low morbidity and mortality, and yet provide a cure for the disease. In this review of the literature of restorative proctocolectomy with ileal pouch-anal anastomosis, we discuss these technical modifications, limiting our discussion to the current points of controversy. The current "hot topics" for debate are: indications for ileal pouch-anal or ileo-rectal anastomosis, indications for pouch surgery in the elderly, indeterminate colitis and Crohn's disease, the place of the laparoscopic approach, transanal mucosectomy with hand-sewn anastomosis vs. the double-stapled technique, the use of diverting ileostomy and the issue of the best route for delivery of pregnant women. Longer follow-up of patients and increased knowledge and experience with pouch surgery, coupled with ongoing prospective evaluation of the procedure are required to settle these issues. PMID:24999229

  2. Comparative morphometric analysis of the gastrointestinal tract of the captive greater cane rat (Thryonomys swinderianus) and African giant pouched rat (Cricetomys gambianus).

    PubMed

    Byanet, Obadiah; Abayomi, Akileye O; Aondohemba, Tyagher J

    2015-01-01

    The greater cane rats (Thryonomys swinderianus) and African giant pouched rats (Cricetomys gambianus) are among the largest rodents in Africa, undergoing domestications for meat and research purposes. The aim of this study was to explore whether there are any quantitative anatomical gastrointestinal adaptations associated with their omnivorous or herbivorous diets. In the African giant rat, the mean gastrointestinal tract length and colon width of the males were significantly higher than their females counterpart (P < 0.05 and P < 0.01, respectively). In a similar way, the mean gastrointestinal tract weight, stomach length and jejunal width in males greater cane rat were significantly higher than in the females (P < 0.05, P < 0.01 and P < 0.01 respectively). The monogastric, omnivores African giant pouched rats had mean significant stomach length and width than greater cane rat (P < 0.01 and P < 0.01 respectively). Also, the duodenal length, jejunal and ileal widths were higher in the former than in the latter (P < 0.05, P < 0.05 and P < 0.01 respectively). The monogastric, herbivore greater cane rats had higher mean cecal width and colon length than the African giant pouched rat (P < 0.01 and P < 0.01 respectively). In conclusion, the African giant pouched rat had larger stomach and longer and wider small intestine, compared to the greater cane rat, which instead had more prominent cecum and wider and longer colon. This suggests that greater cane rats are hindgut fermenting herbivores (cecal fermenter), as is the case in most rodent species. PMID:26738259

  3. [Dorso-ventral continuous irrigation using the Incise Pouch].

    PubMed

    Dittrich, K; Kriwanek, S; Armbruster, C

    1993-01-01

    Dorso-ventral lavage procedures in the treatment of abdominal sepsis are often complicated by technical and nursing problems. With the help of the Incise Pouch, which consists of an adhesive foil with a plastic bag around it, we have succeeded in solving the main problems of the dorso-ventral lavage procedure. (1) All the lavage solution is collected in the bag and drawn into a vacuum pump. The suction drains are placed directly in the bag. This enables us to measure the lavage solution exactly. (2) There is no leakage of liquid into the bed, so that the patient remains dry. (3) The danger of intestinal fistulas or intraabdominal bleeding caused by suction drains is eliminated (by putting them into the bag of the Incise Pouch). If reexploration of the abdominal cavity ("on demand" or planned) is done, the Incise Pouch remains in position. This method makes early diagnosis and treatment of postoperative complications possible. PMID:8326812

  4. Ileo-anal pouch procedure: experience in the Chinese population.

    PubMed

    Lau, P W; Boey, J; Lorentz, T G

    1991-11-01

    The ileo-anal pouch procedure is now a well-established method for dealing with ulcerative colitis and familial polyposis in many centres in the West. Experience in the Chinese population is not well documented, mainly due to the rarity of inflammatory bowel disease. This report documents the experience of a university teaching hospital in Hong Kong. Despite being a small series, the low complication rates and good functional results show that the pelvic pouch procedure has now evolved to a stage where it can be performed safely even in centres with infrequent experience. PMID:1661110

  5. The Hamster Buccal Pouch Model of Oral Carcinogenesis.

    PubMed

    Nagini, Siddavaram; Kowshik, Jaganathan

    2016-01-01

    The hamster buccal pouch (HBP) carcinogenesis model is one of the most well-characterized animal tumor models used as a prelude to investigate multistage oral carcinogenesis and to assess the efficacy of chemointervention. Hamster buccal pouch carcinomas induced by 7,12-dimethylbenz[a]anthracene (DMBA) show extensive similarities to human oral squamous cell carcinomas. The HBP model offers a number of advantages including a simple and predictable tumor induction procedure, easy accessibility for examination and follow-up of lesions, and reproducibility. This model can be used to test both chemopreventive and chemotherapeutic agents. PMID:27246045

  6. The superior mesenteric artery. The critical factor in the pouch pull-through procedure.

    PubMed

    Smith, L; Friend, W G; Medwell, S J

    1984-11-01

    The critical factor in ileal pouch pull-through operations is the length of the superior mesenteric artery. The pouch must reach the dentate line and have adequate blood supply. A series of cadaver and morgue studies were performed to evaluate the two most popular pouches of the "J" and "S" configurations. The "S" pouch generally reaches 2 to 4 cm more caudad than the "J" pouch. If necessary, however, the "J" pouch can be lengthened 2 to 4 cm by cutting the branch vessel under tension to the pouch, sparing vessels to either side and the marginal arcade. The "S" pouch procedure always sacrifices the ileocecal artery, but the "J" pouch procedure does not necessarily do so. The greatest caudad reach is available when the ileum is cut flush with the cecum. To verify a rule of thumb for reaching the dentate line with the pouch, the length of the superior mesenteric artery (SMA) origin to the inferior margin of the symphysis pubis and the SMA to dentate line were compared. If the tip of the pouch or conduit reached 6 cm below the symphysis pubis, all pouches reached the dentate line. PMID:6499610

  7. [Congenital syphilis].

    PubMed

    Tabák, Réka; Tabák, Adám; Várkonyi, Viktória

    2010-01-10

    Syphilis has been a re-emerging disease in the past few decades. As a consequence, the prevalence of congenital syphilis is expected to be on the rise. Maternal syphilis may be related to several pathologies, such as miscarriage, stillbirth, or congenital syphilis in the child. Infants that acquire syphilis in utero are frequently asymptomatic, and the organ damage caused by the infection may be apparent only years later. Syphilis is a curable disease, and most of its complications in the infant can be prevented by screening and treating the mother. Every newborn potentially infected should be treated with penicillin immediately starting on the day of birth. PMID:20061233

  8. Value of histopathology for predicting the post-operative complications of ileo-anal anastomosis (J-pouch) procedure in children with refractory ulcerative colitis.

    PubMed

    El Demellawy, Dina; El Hallani, Soufiane; de Nanassy, Joseph; Lee, James Young; Chan, Emily; Sullivan, Katrina; Bass, Juan; Mack, David; Nasr, Ahmed

    2016-06-01

    The J-pouch is a surgical procedure offered to children with refractory ulcerative colitis (UC) who have undergone subtotal colectomy to reconstruct a reservoir function with ileo-anal anastomosis. Unfortunately, post-operative complications may occur and can compromise the pouch function. We assessed rectal histopathology to determine whether severity of inflammation in the rectum prior to the creation of the J-Pouch was associated with post-operative complications. We retrospectively reviewed the histopathology of all J-pouch procedure specimens from paediatric patients during the period 2000-2013 using an objective grading system that assesses the chronicity and activity of the UC disease. We analysed the parameters for association with the post-operative complications. A classification tree algorithm was generated to predict the risk of complication based on histopathological parameters. A total of 28 paediatric patients were identified, among whom 10 developed post-operative complications (35%). The activity score at the recto-anal margin was higher among the patients with post-operative complications (mean 7.3±3.1 versus 4.8±3.1; p=0.04). The involvement of more than 5% colonic crypts with epithelial neutrophilic infiltration at the recto-anal margin was found to be an independent parameter that would stratify the patients into low-risk or high-risk group for developing complications (17% versus 64%; p=0.04). An association between UC disease activity at the recto-anal margin and post-operative J-pouch complications was determined. Potentially, this association suggests that a histopathological assessment of the recto-anal transitional zone may have value in guiding the surgeon on the risk of post-operative complications. PMID:27130833

  9. Long Upper Pouch in Esophageal Atresia: A Rare Variant

    PubMed Central

    Yhoshu, Enono; Mahajan, Jai Kumar; Dash, Vedarth

    2016-01-01

    The earliest clinical sign of esophageal atresia (EA) is excessive salivation and the diagnosis is made by failure to pass an infant feeding tube (IFT) into the stomach. The diagnostic errors may occur due to presence of an unusually long upper pouch, when the IFT seems to pass into the stomach. We describe one such case and review the relevant literature. PMID:26793598

  10. 21 CFR 876.5900 - Ostomy pouch and accessories.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... attached to the patient's skin by an adhesive material and that is intended for use as a receptacle for... generic type of device and its accessories includes the ostomy pouch, ostomy adhesive, the disposable... bag, ostomy drainage bag with adhesive, stomal bag, ostomy protector, and the ostomy size...

  11. 21 CFR 876.5900 - Ostomy pouch and accessories.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... attached to the patient's skin by an adhesive material and that is intended for use as a receptacle for... generic type of device and its accessories includes the ostomy pouch, ostomy adhesive, the disposable... bag, ostomy drainage bag with adhesive, stomal bag, ostomy protector, and the ostomy size...

  12. 21 CFR 876.5900 - Ostomy pouch and accessories.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... attached to the patient's skin by an adhesive material and that is intended for use as a receptacle for... generic type of device and its accessories includes the ostomy pouch, ostomy adhesive, the disposable... bag, ostomy drainage bag with adhesive, stomal bag, ostomy protector, and the ostomy size...

  13. Microbiological shelf life of pasteurized milk in bottle and pouch.

    PubMed

    Petrus, R R; Loiola, C G; Oliveira, C A F

    2010-01-01

    Shelf life of pasteurized milk in Brazil ranges from 3 to 8 d, mainly due to poor cold chain conditions that prevail throughout the country and subject the product to repeated and/or severe temperature abuse. This study evaluated the influence of storage temperature on the microbiological stability of homogenized whole pasteurized milk (75 degrees C/15 s) packaged in high-density polyethylene (HDPE) bottle and low-density polyethylene (LDPE) pouch, both monolayer materials pigmented with titanium dioxide (TiO(2)). The storage temperatures investigated were 2, 4, 9, 14, and 16 degrees C. Microbiological evaluation was based on mesophilic and psychrotrophic counts with 7 log CFU/mL and 6 log CFU/mL, respectively, set as upper limits of acceptability for maintaining the quality of milk. The microbiological stability for pasteurized milk packaged in HDPE bottle and stored at 2, 4, 9, 14, and 16 degrees C was estimated at 43, 36, 8, 5, and 3 d, respectively. For milk samples packaged in LDPE pouch, shelf life was estimated at 37, 35, 7, 3, and 2 d, respectively. The determination of Q(10) and z values demonstrated that storage temperature has a greater influence on microbiological shelf life of pasteurized milk packaged in LDPE pouch compared to HDPE bottle. Based on the results of this study, HDPE bottle was better for storing pasteurized milk as compared to LDPE pouch. PMID:20492183

  14. Using Giant African Pouched Rats ("Cricetomys Gambianus") to Detect Landmines

    ERIC Educational Resources Information Center

    Poling, Alan; Weetjens, Bart J.; Cox, Christophe; Beyene, Negussie W.; Sully, Andrew

    2010-01-01

    Within the past decade, giant pouched rats have been used successfully to detect landmines. This manuscript summarizes how these rats are trained and used operationally. The information provided is intended to be of practical value toward strengthening best practices in using "Cricetomys" for humanitarian purposes while simultaneously ensuring the…

  15. 21 CFR 876.5900 - Ostomy pouch and accessories.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... attached to the patient's skin by an adhesive material and that is intended for use as a receptacle for... generic type of device and its accessories includes the ostomy pouch, ostomy adhesive, the disposable... bag, ostomy drainage bag with adhesive, stomal bag, ostomy protector, and the ostomy size...

  16. 21 CFR 876.5900 - Ostomy pouch and accessories.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... attached to the patient's skin by an adhesive material and that is intended for use as a receptacle for... generic type of device and its accessories includes the ostomy pouch, ostomy adhesive, the disposable... bag, ostomy drainage bag with adhesive, stomal bag, ostomy protector, and the ostomy size...

  17. Improving Growth of Calibrachoa x Hybrida (Cerv.) in Hanging Pouches

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Unique growing containers and non-traditional types of plant presentation applications can cause new production problems for growers. This study was conducted to evaluate the growth of a popular new container plant, Calibrachoa, produced in hanging flower pouches using different growing media compo...

  18. Congenital Defects.

    ERIC Educational Resources Information Center

    Goldman, Allen S.; And Others

    There are two general categories (not necessarily mutually exclusive) of congenital defects: (1) abnormalities that have an hereditary basis, such as single and multiple genes, or chromosomal abberration; and (2) abnormalities that are caused by nonhereditary factors, such as malnutrition, maternal disease, radiation, infections, drugs, or…

  19. Congenital amusias.

    PubMed

    Tillmann, B; Albouy, P; Caclin, A

    2015-01-01

    In contrast to the sophisticated music processing reported in the general population, individuals with congenital amusia show deficits in music perception and production. Congenital amusia occurs without brain damage, sensory or cognitive deficits, and has been suggested as a lifelong deficit with genetic origin. Even though recognized for a long time, this disorder has been systematically studied only relatively recently for its behavioral and neural correlates. The currently most investigated hypothesis about the underlying deficits concerns the pitch dimension, notably with impaired pitch discrimination and memory. Anatomic and functional investigations of pitch processing revealed that the amusic brain presents abnormalities in the auditory and inferior frontal cortices, associated with decreased connectivity between these structures. The deficit also impairs processing of pitch in speech material and processing of the time dimension in music for some of the amusic individuals, but does not seem to affect spatial processing. Some studies suggest at least partial dissociation in the disorder between perception and production. Recent studies revealed spared implicit pitch perception in congenital amusia, supporting the power of implicit cognition in the music domain. Current challenges consist in defining different subtypes of congenital amusia as well as developing rehabilitation programs for this "musical handicap." PMID:25726292

  20. Colon cancer

    MedlinePlus

    Colorectal cancer; Cancer - colon; Rectal cancer; Cancer - rectum; Adenocarcinoma - colon; Colon - adenocarcinoma ... In the United States, colorectal cancer is one of the leading causes of deaths due to cancer. Early diagnosis can often lead to a complete cure. Almost ...

  1. Characterization of the Gut-Associated Microbiome in Inflammatory Pouch Complications Following Ileal Pouch-Anal Anastomosis

    PubMed Central

    Tyler, Andrea D.; Knox, Natalie; Kabakchiev, Boyko; Milgrom, Raquel; Kirsch, Richard; Cohen, Zane; McLeod, Robin S.; Guttman, David S.; Krause, Denis O.; Silverberg, Mark S.

    2013-01-01

    Introduction Inflammatory complications following ileal pouch-anal anastomosis (IPAA) for ulcerative colitis (UC) are common and thought to arise through mechanisms similar to de novo onset inflammatory bowel disease. The aim of this study was to determine whether specific organisms in the tissue-associated microbiota are associated with inflammatory pouch complications. Methods Patients having previously undergone IPAA were recruited from Mount Sinai Hospital. Clinical and demographic information were collected and a pouchoscopy with biopsy of both the pouch and afferent limb was performed. Patients were classified based on post-surgical phenotype into four outcome groups: familial adenomatous polyposis controls (FAP), no pouchitis, pouchitis, and Crohn’s disease-like (CDL). Pyrosequencing of the 16S rRNA V1-V3 hypervariable region, and quantitative PCR for bacteria of interest, were used to identify organisms present in the afferent limb and pouch. Associations with outcomes were evaluated using exact and non-parametric tests of significance. Results Analysis at the phylum level indicated that Bacteroidetes were detected significantly less frequently (P<0.0001) in the inflammatory outcome groups (pouchitis and CDL) compared to both FAP and no pouchitis. Conversely, Proteobacteria were detected more frequently in the inflammatory groups (P=0.01). At the genus level, organisms associated with outcome were detected less frequently among the inflammatory groups compared to those without inflammation. Several of these organisms, including Bacteroides (P<0.0001), Parabacteroides (P≤2.2x10-3), Blautia (P≤3.0x10-3) and Sutterella (P≤2.5x10-3), were associated with outcome in both the pouch and afferent limb. These associations remained significant even following adjustment for antibiotic use, smoking, country of birth and gender. Individuals with quiescent disease receiving antibiotic therapy displayed similar reductions in these organisms as those with active

  2. Congenital Hydrocephalus.

    PubMed

    Estey, Chelsie M

    2016-03-01

    There are several types of hydrocephalus, which are characterized based on the location of the cerebrospinal fluid (CSF) accumulation. Physical features of animals with congenital hydrocephalus may include a dome-shaped skull, persistent fontanelle, and bilateral ventrolateral strabismus. Medical therapy involves decreasing the production of CSF. The most common surgical treatment is placement of a ventriculoperitoneal shunt. Postoperative complications may include infection, blockage, drainage abnormalities, and mechanical failure. PMID:26704658

  3. Dietary habits after ileal pouch-anal anastomosis.

    PubMed

    Chartrand-Lefebvre, C; Heppell, J; Davignon, I; Dubé, S; Pomp, A

    1990-04-01

    Dietary habits of patients who had undergone ileal pouch-anal anastomosis were assessed and correlated with bowel function. Twenty-four well-adapted patients (11 women, 13 men; mean age 32 years) voluntarily entered the study 30 +/- 4 months after closure of the diverting ileostomy. A standardized questionnaire on 108 food items and a 3-day food journal were used in the assessment. Twenty-one patients had no difficulty in selecting an appropriate diet. Caloric intake was adequate. Specific symptoms associated with several foods were as follows: increased stool frequency (beer, spirits, chinese food), decreased stool consistency (beer, wine, fried fish), perianal irritation (spicy foods), undigested particles (grapefruit, lettuce), odours (eggs). Pasta and bananas were associated with increased stool consistency. The authors believe that these observations may help in dietary counselling after ileal pouch-anal anastomosis. PMID:2268807

  4. Congenital Adrenal Hyperplasia

    MedlinePlus

    MENU Return to Web version Congenital Adrenal Hyperplasia Overview What is congenital adrenal hyperplasia? Congenital adrenal hyperplasia, or CAH, is a disorder that affects the adrenal glands. The adrenal ...

  5. A Portable Analyzer for Pouch-Actuated, Immunoassay Cassettes

    PubMed Central

    Qiu, Xianbo; Liu, Changchun; Mauk, Michael G.; Hart, Robert W.; Chen, Dafeng; Qiu, Jing; Kientz, Terry; Fiene, Jonathan; Bau, Haim H.

    2011-01-01

    A portable, small footprint, light, general purpose analyzer (processor) to control the flow in immunoassay cassettes and to facilitate the detection of test results is described. The durable analyzer accepts disposable cassettes that contain pouches and reaction chambers for various unit operations such as hydration of dry reagents, stirring, and incubation. The analyzer includes individually controlled, linear actuators to compress the pouches in the cassette, which facilitates the pumping and mixing of sample and reagents, and to close diaphragm-based valves for flow control. The same types of actuators are used to compress pouches and actuate valves. The analyzer also houses a compact OEM scanner/reader to excite fluorescence and detect emission from labels. The analyzer is hydraulically isolated from the cassette, reducing the possibility of cross-contamination. The analyzer facilitates programmable, automated execution of a sequence of operations such as pumping and valving in a timely fashion, reducing the level of expertise required from the operator and the possibility for errors. The analyzer’s design is modular and expandable to accommodate cassettes of various complexities and additional functionalities. In this paper, the utility of the analyzer has been demonstrated with the execution of a simple, consecutive, lateral flow assay of a model biological system and the test results were detected with up converting phosphor labels that are excited at infrared frequencies and emit in the visible spectrum. PMID:22125359

  6. Reconstruction with Jejunal Pouch after Gastrectomy for Gastric Cancer.

    PubMed

    Namikawa, Tsutomu; Munekage, Eri; Munekage, Masaya; Maeda, Hiromichi; Kitagawa, Hiroyuki; Nagata, Yusuke; Kobayashi, Michiya; Hanazaki, Kazuhiro

    2016-06-01

    The construction of a gastric substitute pouch after gastrectomy for gastric cancer has been proposed to help ameliorate postprandial symptoms and nutritional performance. Adequate reconstruction after gastrectomy is an important issue, because postoperative patient quality of life (QOL) primarily depends on the reconstruction method. To this end, jejunal pouch (JP) reconstructions were developed to improve the patient's eating capacity and QOL by creating large reservoirs with improved reflux barriers to prevent esophagitis and residual gastritis. It is important that such reconstructions also preserve blood and extrinsic neural integrity for maintaining pouch function, because JP motility is associated directly with QOL. Some problems remain to be resolved with the JP reconstructions method including gastrointestinal motility, which plays a major role in food transfer, digestion, and absorption of nutrients. Further studies including basic research and larger prospective randomized control trials are also needed to obtain definitive results. With persistent innovations in surgical techniques, JP after gastrectomy could become a safe and preferable reconstructive modality to improve patient QOL after gastrectomy. PMID:27305882

  7. The Kock pouch reconsidered: an alternative surgical technique.

    PubMed

    Crawshaw, Alison; Williams, Julia; Woodhouse, Fran

    2014-09-24

    The psychological impact stoma surgery can have on an individual is well documented within the literature ( White and Hunt, 1997 ; Borwell, 2009 ; Williams, 2005 ; Brown, 2005 ). For many years, surgeons have explored and developed innovations in surgical techniques, in particular restorative procedures with a view of preventing permanent stoma formation; ileal anal pouch (IAP) now being the surgical procedure of choice for treatment of ulcerative colitis (UC) and familial adenomatous polyposis (FAP). However, high morbidity rates are associated with pouch longevity ( Castillo et al 2005 ; Nessar and Wu, 2012 ) and once removed can lead to a high-output ileostomy with risks of electrolyte imbalance and malabsorption. This then creates the dilemma of whether the Kock pouch (KP) should be offered as a surgical option. This article offers a historical perspective of the KP and its place in the surgical management of UC and FAP. This article also presents results from a recent audit funded by the Ileostomy Association (IA), highlighting how patients manage their KP and the importance of maintaining bowel control and being free of an incontinent stoma as a means of coming to terms with their condition. PMID:25251313

  8. Long-term followup of the Kock and Indiana pouch procedures.

    PubMed

    Arai, Y; Kawakita, M; Terachi, T; Oishi, K; Okada, Y; Takeuchi, H; Yoshida, O

    1993-07-01

    Between 1984 and 1991, 115 consecutive patients underwent cutaneous continent urinary diversion comprising 76 Kock and 39 Indiana pouch procedures. The 2 different forms of achieving continent urinary diversion were subsequently compared in a long-term followup that evaluated complications, including pouch function and the need for revisions. In the Kock pouch group there were 14 (18.4%) early postoperative complications (3 months), which required 4 subsequent reoperations (5.3%). The Indiana pouch group had a similar incidence of early complications (17.9%) but there were no reservoir related problems. The long-term study group comprised 68 Kock and 37 Indiana pouch patients who were observed for 12 months or longer (mean followup 53 and 34 months, respectively). Of 9 efferent nipple valve malfunctions observed in the Kock pouch group 5 required surgical revision. Of 16 complications related to afferent limb function 15 were caused by the use of polyester fiber fabric for the anchoring collar and 8 of these 15 complications required surgical revision. The first 2 Indiana pouch patients had pouch deformities due to incomplete detubularization of the cecum that required surgical repair. Overall, surgical revisions, including minor repairs, were performed on 15 Kock pouch patients (22.1%) and 4 Indiana pouch patients (10.8%). Both forms of the procedure preserved continence to a satisfactory degree. Urinary tract stones developed in 18 patients (26.5%) from the Kock pouch group, usually on the exposed staples or the eroded, nonabsorbable collar used to construct the nipple valves. Stone formation was rare (5.4%) in the Indiana pouch group. The incidence of ureteral implantation stricture was low in both procedures. There was no significant difference in the incidence of bacteriuria between the 2 methods of urinary diversion. These data demonstrate that the Kock pouch and Indiana pouch procedures can be accomplished with the same early postoperative complication rate

  9. Congenital Anomalies in Infant with Congenital Hypothyroidism

    PubMed Central

    Razavi, Zahra; Yavarikia, Alireza; Torabian, Saadat

    2012-01-01

    Objective Congenital hypothyroidism is characterized by inadequate thyroid hormone production in newborn infants. Many infants with CH have co-occurring congenital malformations. This is an investigation on the frequency and types of congenital anomalies in infants with congenital hypothyroidism born from May 2006-2010 in Hamadan, west province of Iran. Methods The Iranian neonatal screening program for congenital hypothyroidism was initiated in May 2005. This prospective descriptive study was conducted in infants diagnosed with congenital hypothyroidism being followed up in Pediatric Endocrinology Clinic of Besat Hospital, a tertiary care centre in Hamadan. Cases included all infants with congenital hypothyroidism diagnosed through newborn screening program or detected clinically. Anomalies were identified by clinical examination, echocardiography, and X-ray of the hip during the infant’s first year of life. Results A total of 150 infants with biochemically confirmed primary congenital hypothyroidism (72 females and 78 males) were recruited during the period between May 2006-2010. Overall, 30 (20%) infants had associated congenital anomalies. The most common type of anomaly was Down syndrome. Seven infants (3.1%) had congenital cardiac anomalies such as: ASD (n=3), VSD (n=2), PS (n =1), PDA (n=1). Three children (2.6%) had developmental dysplasia of the hip (n=3). Conclusion The overall frequency of Down syndrome, cardiac malformation and other birth defect was high in infants with CH. This reinforces the need to examine all infants with congenital hypothyroidism for the presence of associated congenital anomalies. PMID:23074545

  10. Congenital Toxoplasmosis

    PubMed Central

    McAuley, James B.

    2014-01-01

    Toxoplasmosis is caused by infection with the parasite Toxoplasma gondii. It is one of the most common parasitic infections in humans and is most typically asymptomatic. However, primary infection in a pregnant woman can cause severe and disabling disease in the developing fetus. Recent developments have included increased understanding of the role of parasite genotype in determining infectivity and disease severity. Risk factors for acquisition of infection have been better defined, and the important role of foodborne transmission has been further delineated. In addition, strategies have emerged to decrease mother-to-child transmission through prompt identification of acutely infected pregnant women followed by appropriate treatment. Refined diagnostic tools, particularly the addition of immunoglobulin G avidity testing, allow for more accurate timing of maternal infection and hence better decision making during pregnancy. Congenitally infected children can be treated, beginning in utero and continuing through the first year of life, to ameliorate the severity of disease. However, despite these many advances in our understanding of congenital toxoplasmosis prevention and treatment, significant areas of study remain: we need better drugs, well defined strategies for screening of pregnant women, improved food safety, and improved diagnostic tests. PMID:25232475

  11. Safety and Long-Term Performance of Lithium-ion Pouch Cells

    NASA Technical Reports Server (NTRS)

    Jeevarajan, Judith

    2012-01-01

    Lithium-ion batteries have the highest energy density of the batteries available in the commercial market today. Although most lithium-ion cell designs use a metal can design, this has changed significantly in recent years. Cell designs are offered in the pouch format as they offer better volumetric and gravimetric energy densities and in some cases, higher tolerance to abuse or off-nominal conditions. In the past decade, several state-of-the-art lithium-ion pouch cell designs have been tested. The pouch cell designs have become more robust in the past two years but there are still a few issues that need to be looked into for optimization. The pouch cells seem to have a tendency to swell when left in storage under ambient conditions. The cells also swell under overvoltage and undervoltage conditions. A significant issue that has been observed is the swelling of the cells under a vacuum condition which could lead to deformation of the cell pouch after this exposure. This last factor would be very critical in the use of these cell designs for space applications as vacuum exposure is used to check for cell and battery leaks before it is flown into space. In rare cases, corrosion of the aluminum layer of the pouches has been observed in stored cells. Pouch material analysis has been carried out in an effort to understand the strength of the pouches and determine if this is a factor in the corrosion as well as unsafe condition of the cells as deformation of the inner layers of the pouch could occur when the cells swell under the various conditions described above. Pouch materials are typically aluminized plastic, made up of a layer of Al sandwiched between one or more layers of polymeric material. Deformations or cell manufacturing processes could lead to a compromise of the inner polymeric layer/s of the pouch leading to the corrosion of the Al layer in the aluminized pouch material. The safety of the pouch cell designs has been determined for cells from various

  12. Congenital neuroblastoma

    PubMed Central

    Evans, A. R.

    1965-01-01

    The clinical histories and post-mortem findings in five cases of neuroblastoma are described, and an account given of the microscopic characteristics of the tumours. In four of the cases the tumour was present at birth and was probably so in the fifth case. In only one case was the presence of the malignant tumour a significant factor in causing death. The differential diagnosis of such tumours is discussed. The accumulated evidence of many recorded cases suggests that neuroblastoma, becoming manifest in the early months or weeks of life, and congenital tumour, would be included in such a group, and has an appreciably better prognosis than has this same tumour when it becomes manifest in later childhood. The literature is briefly reviewed to illustrate this aspect of prognosis and possible reasons for it are indicated. Images PMID:14247705

  13. Congenital Cholesteatoma.

    PubMed

    Walker, David; Shinners, Michael J

    2016-05-01

    Congenital cholesteatoma is one of the more common causes of the onset of childhood conductive hearing loss unrelated to middle ear effusion. If undiagnosed, the disease can progress to irreversibly destroy the conductive hearing architecture, as well as the surrounding skull base of the lateral temporal bone. When diagnosed early, the growth can be removed and the conductive hearing mechanism preserved in the vast majority of patients. Because most children are asymptomatic, the burden falls on primary care providers to perform pneumatic otoscopy and visualize all quadrants of the tympanic membrane even in young children who frequently resist attempts to conduct a thorough examination to rule out suspicious lesions. [Pediatr Ann. 2016;45(5):e167-e170.]. PMID:27171804

  14. Congenital amusia.

    PubMed

    Williamson, Victoria J; Stewart, Lauren

    2013-01-01

    For most people, music, like language, is acquired effortlessly in early life. But a few percent of the population have lifelong difficulties in the perception and production of music. In this chapter we discuss psycho-acoustic and behavioral studies that have attempted to delineate the nature of the auditory perceptual deficits in this group and consider whether these difficulties extend outside the musical domain. Finally, we review structural imaging studies in this group which point to subtle anomalies in temporal and frontal areas. We suggest that amusia can be considered a disorder of neural development, which has relatively specific consequences at the behavioral level. Studies of congenital amusia provide a unique window on the neurocognitive architecture of music processing. PMID:23622169

  15. Congenital hypothyroidism

    PubMed Central

    Agrawal, Pankaj; Philip, Rajeev; Saran, Sanjay; Gutch, Manish; Razi, Mohd Sayed; Agroiya, Puspalata; Gupta, Keshavkumar

    2015-01-01

    Congenital hypothyroidism (CH) is the one of the most common preventable cause of mental retardation. In the majority of patients, CH is caused by an abnormal development of the thyroid gland (thyroid dysgenesis) that is a sporadic disorder and accounts for 85% of cases and the remaining 15% of cases are caused by dyshormonogenesis. The clinical features of congenital hypothyroidism are so subtle that many newborn infants remain undiagnosed at birth and delayed diagnosis leads to the most severe outcome of CH, mental retardation, emphasizing the importance of neonatal screening. Dried capillary blood is used for screening and it is taken from heel prick optimally between 2 and 5 days of age. Blood spot TSH or thyroxine (T4) or both are being used for CH screening in different programs around the world. Neonates with abnormal thyroid screening tests should be recalled immediately for examination and a venipuncture blood sample should be drawn for confirmatory serum testing. Confirmatory serum should be tested for TSH and free T4, or total T4. Serum TSH and T4 undergo dynamic changes in the first weeks of life; it is important to compare serum results with age-normal reference ranges. Treatment should be started promptly and infant should be rendered euthyroid as early as possible, as there is an inverse relationship between intelligence quotient (IQ) and the age at diagnosis. Levothyroxine (l-thyroxine) is the treatment of choice and American academy of pediatrics and European society of pediatric endocrinology recommend 10-15μgm/kg/day as initial dose. The immediate goal of therapy is to normalize T4 within 2 weeks and TSH within one month. The overall goal of treatment is to ensure growth and neurodevelopmental outcomes as close as possible to their genetic potential. PMID:25729683

  16. The hamster cheek pouch model for field cancerization studies.

    PubMed

    Monti-Hughes, Andrea; Aromando, Romina F; Pérez, Miguel A; Schwint, Amanda E; Itoiz, Maria E

    2015-02-01

    External carcinogens, such as tobacco and alcohol, induce molecular changes in large areas of oral mucosa, which increase the risk of malignant transformation. This condition, known as 'field cancerization', can be detected in biopsy specimens using histochemical techniques, even before histological alterations are identified. The efficacy of these histochemical techniques as biomarkers of early cancerization must be demonstrated in appropriate models. The hamster cheek pouch oral cancer model, universally employed in biological studies and in studies for the prevention and treatment of oral cancer, is also an excellent model of field cancerization. The carcinogen is applied in solution to the surface of the mucosa and induces alterations that recapitulate the stages of cancerization in human oral mucosa. We have demonstrated that the following can be used for the early detection of cancerized tissue: silver staining of nucleolar organizer regions; the Feulgen reaction to stain DNA followed by ploidy analysis; immunohistochemical analysis of fibroblast growth factor-2, immunohistochemical labeling of proliferating cells to demonstrate an increase of epithelial cell proliferation in the absence of inflammation; and changes in markers of angiogenesis (i.e. those indicating vascular endothelial growth factor activity, endothelial cell proliferation and vascular density). The hamster cheek pouch model of oral cancer was also proposed and validated by our group for boron neutron capture therapy studies for the treatment of oral cancer. Clinical trials of this novel treatment modality have been performed and are underway for certain tumor types and localizations. Having demonstrated the efficacy of boron neutron capture therapy to control tumors in the hamster cheek pouch oral cancer model, we adapted the model for the long-term study of field cancerized tissue. We demonstrated the inhibitory effect of boron neutron capture therapy on tumor development in field

  17. Tunguska, 1908: the gas pouch and soil fluidization hypothesis

    NASA Astrophysics Data System (ADS)

    Nistor, I.

    2012-01-01

    The Siberian taiga explosion of 30 June 1908 remains one of the great mysteries of the 20th century: millions of trees put down over an area of 2200 km2 without trace of a crater or meteorite fragments. Hundred years of failed searches have followed, resulting in as many flawed hypothesis which could not offer satisfactory explanations: meteorite, comet, UFO, etc. In the author's opinion, the cause is that the energy the explorers looked for was simply not there! The author's hypothesis is that a meteoroid encountered a gas pouch in the atmosphere, producing a devastating explosion, its effects being amplified by soil fluidization.

  18. Hermetic packaging of drugs: optimized sealing of foil pouches.

    PubMed

    Auslander, D E; Gilbert, S G

    1976-07-01

    Factors affecting the sealing of foil packages were studied in the sealing of foil packages were studied in three laboratories. The relationship of sealing temperature (with machine speed and pressure kept constant) to the incidence of defective packages was determined. The maximum acceptable limit for defective pouches was 1%. Three tests were employed to detect defects: vacuum-dye, seal strength, and pressurized ammonia vapor. Only the last was sensitive enough to determine the optimum sealing conditions. This test also was capable of detecting leakage sites. Replacement of the cellophane layer of the foil laminate with polyvinylidene chloride-coated polyester improved the barrier properties of the package. PMID:957113

  19. Tubular Colonic Duplication Presenting as Rectovestibular Fistula

    PubMed Central

    Bendre, Pradnya; D'souza, Flavia; Ramchandra, Mukunda; Nage, Amol; Palse, Nitin

    2015-01-01

    Complete colonic duplication is a very rare congenital anomaly that may have different presentations according to its location and size. Complete colonic duplication can occur in about 15% of all gastrointestinal duplications. Double termination of tubular colonic duplication in the perineum is even more uncommon. We present a case of a Y-shaped tubular colonic duplication which presented with a rectovestibular fistula and a normal anus. Radiological evaluation and initial exploration for sigmoidostomy revealed duplicated colons with a common vascular supply. Endorectal mucosal resection of theduplicated distal segment till the colostomy site with division of the septum of the proximal segment and colostomy closure proved curative without compromise of the continence mechanism. Tubular colonic duplication should always be ruled out when a diagnosis of perineal canal is considered in cases of vestibular fistula alongwith a normal anus. PMID:26473141

  20. Tubular Colonic Duplication Presenting as Rectovestibular Fistula.

    PubMed

    Karkera, Parag J; Bendre, Pradnya; D'souza, Flavia; Ramchandra, Mukunda; Nage, Amol; Palse, Nitin

    2015-09-01

    Complete colonic duplication is a very rare congenital anomaly that may have different presentations according to its location and size. Complete colonic duplication can occur in about 15% of all gastrointestinal duplications. Double termination of tubular colonic duplication in the perineum is even more uncommon. We present a case of a Y-shaped tubular colonic duplication which presented with a rectovestibular fistula and a normal anus. Radiological evaluation and initial exploration for sigmoidostomy revealed duplicated colons with a common vascular supply. Endorectal mucosal resection of theduplicated distal segment till the colostomy site with division of the septum of the proximal segment and colostomy closure proved curative without compromise of the continence mechanism. Tubular colonic duplication should always be ruled out when a diagnosis of perineal canal is considered in cases of vestibular fistula alongwith a normal anus. PMID:26473141

  1. New treatment for ileal pouch-anal or coloanal anastomotic stenosis.

    PubMed

    Benoist, S; Panis, Y; Berdah, S; Hautefeuille, P; Valleur, P

    1998-07-01

    Persistent anastomotic stricture following ileal pouch-anal or coloanal anastomoses can be treated by transanal resection using a stapler or a more complex procedure, such as transanal pouch advancement with neoanastomosis. We propose an easier and faster technique, which does not require any particular device. Its long-term functional results are satisfactory in most patients. PMID:9678384

  2. Congenital hypothyroidism.

    PubMed

    Rastogi, Maynika V; LaFranchi, Stephen H

    2010-01-01

    Congenital hypothyroidism (CH) occurs in approximately 1:2,000 to 1:4,000 newborns. The clinical manifestations are often subtle or not present at birth. This likely is due to trans-placental passage of some maternal thyroid hormone, while many infants have some thyroid production of their own. Common symptoms include decreased activity and increased sleep, feeding difficulty, constipation, and prolonged jaundice. On examination, common signs include myxedematous facies, large fontanels, macroglossia, a distended abdomen with umbilical hernia, and hypotonia. CH is classified into permanent and transient forms, which in turn can be divided into primary, secondary, or peripheral etiologies. Thyroid dysgenesis accounts for 85% of permanent, primary CH, while inborn errors of thyroid hormone biosynthesis (dyshormonogeneses) account for 10-15% of cases. Secondary or central CH may occur with isolated TSH deficiency, but more commonly it is associated with congenital hypopitiutarism. Transient CH most commonly occurs in preterm infants born in areas of endemic iodine deficiency. In countries with newborn screening programs in place, infants with CH are diagnosed after detection by screening tests. The diagnosis should be confirmed by finding an elevated serum TSH and low T4 or free T4 level. Other diagnostic tests, such as thyroid radionuclide uptake and scan, thyroid sonography, or serum thyroglobulin determination may help pinpoint the underlying etiology, although treatment may be started without these tests. Levothyroxine is the treatment of choice; the recommended starting dose is 10 to 15 mcg/kg/day. The immediate goals of treatment are to rapidly raise the serum T4 above 130 nmol/L (10 ug/dL) and normalize serum TSH levels. Frequent laboratory monitoring in infancy is essential to ensure optimal neurocognitive outcome. Serum TSH and free T4 should be measured every 1-2 months in the first 6 months of life and every 3-4 months thereafter. In general, the prognosis

  3. Motility of the jejunum after proctocolectomy and ileal pouch anastomosis.

    PubMed Central

    Chaussade, S; Merite, F; Hautefeuille, M; Valleur, P; Hautefeuille, P; Couturier, D

    1989-01-01

    Proctocolectomy with ileal pouch anastomosis could modify motility of the small intestine through two mechanisms: obstruction or bacterial overgrowth. Motility of the jejunum was measured in 11 patients with ileoanal anastomosis six (n = 6), or 12 (n = 5) months after closure of the loop ileostomy. Manometric recording from the jejunum were made during fasting (four hours) and after a liquid meal (one hour). These findings were compared with those of six healthy volunteers. Motor events were classified as follows: migrating motor complex (MMC), propagated contractions, or discrete clustered contractions. All patients were investigated for bacterial overgrowth (D-glucose breath test). Only two patients had bacterial overgrowth. The frequency of MMC remained unchanged after ileo-anal anastomosis (2.83 (0.37)/four hours) compared with normal volunteers (2.81 (0.29)/four hours). During fasting, four patients had numerous propagated contractions in the jejunum. This condition was associated in two with bacterial overgrowth and in two with intubation of the reservoir. Discrete clustered contractions were found in the seven patients studied postprandially (7.6 (2.5)/h), but not in volunteers. These seven patients emptied their pouch spontaneously and bacterial overgrowth was found in only one. As this motility pattern was previously described in partial small intestinal obstruction, it is postulated that discrete clustered contractions could be the consequence of a functional obstruction as a result of anastomosis of the small intestine to the high pressure zone of the anal sphincters. Images Fig. 1 Fig. 2 PMID:2707637

  4. Congenital hypothyroidism.

    PubMed

    Abduljabbar, Mohammad A; Afifi, Ashraf M

    2012-01-01

    Congenital hypothyroidism (CH) is defined as thyroid hormone deficiency present at birth. Babies with CH who are not identified and treated promptly develop severe mental retardation. Most of the babies with CH do not manifest the typical known signs and symptoms of hypothyroidism, and this is most likely due to transplacental passage of some maternal thyroid hormone in addition to some residual neonatal thyroid function, as might be seen with thyroid hypoplasia, an ectopic gland, or mild dyshormonogenesis. Screening for CH has enabled the virtual eradication of the devastating effects of mental retardation due to sporadic CH in most developed countries of the world. CH is classified into permanent and transient forms, which in turn can be divided into primary, secondary, or peripheral etiologies. Permanent CH refers to a persistent deficiency of thyroid hormone that requires life-long treatment. Transient CH refers to a temporary deficiency of thyroid hormone that is discovered at birth but recovers to normal in the first few months or years of life. In the last several decades, there have been exciting advances in our understanding of fetal and neonatal thyroid physiology. In addition, advances in molecular biology have helped in understanding the early events in thyroid gland embryogenesis, mechanisms of thyroid action in the brain, the molecular basis for many of the inborn errors of thyroid hormonogenesis, and thyroid hormone action. However, many questions and challenges are still not answered. For example, the increasing numbers of surviving small and premature neonates with abnormalities in thyroid function need definite diagnostic criteria and whether they require medical therapy. Another challenge is the dilemma of finding the best screening methodology that is sensitive and cost effective. PMID:22570946

  5. Congenital myopathies

    PubMed Central

    Colombo, Irene; Scoto, Mariacristina; Manzur, Adnan Y.; Robb, Stephanie A.; Maggi, Lorenzo; Gowda, Vasantha; Cullup, Thomas; Yau, Michael; Phadke, Rahul; Sewry, Caroline; Jungbluth, Heinz

    2015-01-01

    Objective: To assess the natural history of congenital myopathies (CMs) due to different genotypes. Methods: Retrospective cross-sectional study based on case-note review of 125 patients affected by CM, followed at a single pediatric neuromuscular center, between 1984 and 2012. Results: Genetic characterization was achieved in 99 of 125 cases (79.2%), with RYR1 most frequently implicated (44/125). Neonatal/infantile onset was observed in 76%. At birth, 30.4% required respiratory support, and 25.2% nasogastric feeding. Twelve percent died, mainly within the first year, associated with mutations in ACTA1, MTM1, or KLHL40. All RYR1-mutated cases survived and did not require long-term ventilator support including those with severe neonatal onset; however, recessive cases were more likely to require gastrostomy insertion (p = 0.0028) compared with dominant cases. Independent ambulation was achieved in 74.1% of all patients; 62.9% were late walkers. Among ambulant patients, 9% eventually became wheelchair-dependent. Scoliosis of variable severity was reported in 40%, with 1/3 of (both ambulant and nonambulant) patients requiring surgery. Bulbar involvement was present in 46.4% and required gastrostomy placement in 28.8% (at a mean age of 2.7 years). Respiratory impairment of variable severity was a feature in 64.1%; approximately half of these patients required nocturnal noninvasive ventilation due to respiratory failure (at a mean age of 8.5 years). Conclusions: We describe the long-term outcome of a large cohort of patients with CMs. While overall course is stable, we demonstrate a wide clinical spectrum with motor deterioration in a subset of cases. Severity in the neonatal/infantile period is critical for survival, with clear genotype-phenotype correlations that may inform future counseling. PMID:25428687

  6. Giant congenital nevus

    MedlinePlus

    ... A congenital pigmented or melanocytic nevus is a dark-colored, often hairy, patch of skin. A congenital ... rare. Symptoms A nevus will appear as a dark-colored patch with any of the following: Brown ...

  7. Congenital heart disease

    MedlinePlus

    Congenital heart disease is a problem with the heart's structure and function that is present at birth. ... Congenital heart disease (CHD) can describe a number of different problems affecting the heart. It is the most common ...

  8. Adult Congenital Heart Association

    MedlinePlus

    ... to ACHA Search The futures of adults with congenital heart disease made brighter by their pasts Get Involved 2016 ... conference theme is "The Changing Landscape of Adult Congenital Heart Disease." Join Us Help us improve the quality of ...

  9. Congenital Heart Information Network

    MedlinePlus

    ... heart defects. Important Notice The Congenital Heart Information Network website is temporarily out of service. Please join ... and Uwe Baemayr for The Congenital Heart Information Network Exempt organization under Section 501(c)3. Copyright © ...

  10. Congenital Intralabyrinthine Cholesteatoma

    PubMed Central

    Prasad, Sanjay; Prasad, Kiran; Azadarmaki, Roya

    2014-01-01

    A patient with a congenital intralabyrinthine cholesteatoma is presented. High-resolution computerized tomographic scans and intraoperative photomicrographs display features of intralabyrinthine extension. We discuss pathogenetic theories for the development of congenital intralabyrinthine cholesteatoma. The distinction of this condition from congenital cholesteatoma with labyrinthine erosion is discussed. PMID:25057421

  11. Congenital sternoclavicular dermoid sinus.

    PubMed

    Willaert, Annelore; Bruninx, Liesje; Hens, Greet; Hauben, Esther; Devriendt, Koen; Vander Poorten, Vincent

    2016-02-01

    We report a case series of 8 patients, presenting with a congenital sinus in the region of the sternoclavicular joint. This rare malformation has only been reported in the Japanese dermatological literature under the name of "congenital dermoid fistula of the anterior chest region". It has to be distinguished from other congenital anomalies and requires complete excision. PMID:26810293

  12. Effect of DMSO and DMBA hamster pouch carcinogenesis

    SciTech Connect

    Rivera-Hidalgo, F.; Miller, E.G.; Binnie, W.H.

    1987-01-01

    The penetration of mucosal surfaces by chemical carcinogens is required for tumor induction. The effectiveness of dimethyl sulfoxide (DMSO) as a carrier for carcinogen is controversial. The purpose of this study was to determine what effect DMSO would have on the 9,10-dimethyl- 1,2-benzanthracene (DMBA)-induced carcinogenesis in the hamster cheek pouch. Thirty Syrian golden hamsters were divided into two groups: the control group received a topical application of 0.5% DMBA in mineral oil three times per week for 16 weeks, while the experimental group received a topical application of DMSO previous to each DMBA application. At autopsy, both groups had developed tumors, the tumor ratio of control to experimental was 3.5:1.9 and the average size of tumors was 2.2 to 1.9 mm sq. The results suggest that DMSO interfered with the usual DMBA induction mechanism.

  13. A Novel Lithium-ion Laminated Pouch Cell Tested For Performance And Safety

    NASA Technical Reports Server (NTRS)

    Jeevarajan, Judith A.; Inoue, Takefumi

    2006-01-01

    A new Li-ion 4.0 Ah pouch cell from GS Yuasa has been tested to determine its performance and safety. The cell is of a laminate pouch design with liquid electrolyte. The rate, thermal and vacuum performance capabilities have been tested to determine the optimum parameters. Under vacuum conditions, the cells were cycled under restrained and unrestrained configurations. The burst pressure of the laminate pouch was also determined. The overcharge, overdischarge into reversal and external short circuit safety tests were also performed to determine the cell s tolerance to abuse. Key Words: Li-ion, safety, vacuum test, abuse, COTS batteries, rate capability

  14. Gastric Pouch After Simple or Radical Cystectomy for Benign and Malignant Bladder Disease

    PubMed Central

    Shamsa, Ali

    2014-01-01

    Background: There is a controversy regarding the use of gastric pouch for benign and malignant bladder diseases. Objectives: The aim of this retrospective study was to report our experience, mostly on gastric neobladder (not gastrocystoplasty) for benign and malignant bladder diseases, its complications, outcomes and follow-up results. Materials and Methods: In this retrospective case series, we described our experience on seven gastric pouches (2 gastrocystoplasty and 5 gastric neobladders). Results: Postoperative complications were rare. Continence was defined as bladder capacity over 400 mL. Their follow-up period ranged from five months up to writing the article. One of the studied cases is still alive and awaiting renal transplantation. Conclusions: Gastric pouch is a suitable segment for bladder cystoplasty and neobladder. Continence is mostly related to the capacity of pouch. PMID:25738108

  15. Colon cancer

    MedlinePlus

    ... red or processed meats Have colorectal polyps Have inflammatory bowel disease ( Crohn disease or ulcerative colitis ) Have a family history of colon cancer Have a personal history of breast cancer Some inherited diseases also increase the risk ...

  16. Complete colonic duplication in children

    PubMed Central

    Khaleghnejad Tabari, Ahmad; Mirshemirani, Alireza; Khaleghnejad Tabari, Nasibeh

    2012-01-01

    Background: Complete colonic duplication is a very rare congenital anomaly that may have different presentations according to its location and size. Complete colonic duplication can occur in 15% of gastrointestinal duplication. We report two cases of complete colonic duplications, and their characteristics. Case Presentation: We present two patients with complete colonic duplication with different types and presentations. Case 1: A 2- year old boy presented to the clinic with abdominal protrusion, difficulty to defecate, chronic constipation and mucosal prolaps covered bulging (rectocele) since he was 6 months old. The patient had palpable pelvic mass with doughy consistency. Rectal exam confirmed perirectal mass with soft consistency. The patient underwent a surgical operation that had total tubular colorectal duplication with one blind end and was treated with simple fenestration of distal end, and was discharged without complication. After two years follow up, he had normal defecation and good weight gain. Case 2: A 2 –day old infant was referred with imperforate anus and complete duplication of recto-sigmoid colon, diphallus, double bladder, and hypospadiasis. After clinical and paraclinical investigations, he underwent operations in several stages in different periods, and was discharged without complications. After four years follow up, he led a normal life. Conclusion: The patients with complete duplication have to be examined carefully because of the high incidence of other systemic anomalies. Treatment includes simple resection of distal common wall, fenestration, and repair other associated anomalies. PMID:24358440

  17. [Gangrenous pyoderma and enterocutaneous fistulas after ileal pouch-anal anastomosis].

    PubMed

    Fadrique, Alfonso García; Ferrer, Francisco Villalba; Esteban, Marcos Bruna; Vila, José Vicente Roig

    2007-05-01

    We describe the medical-surgical management of a patient with a complex inflammatory bowel disease who developed 2 acute episodes of pyoderma gangrenosum and enterocutaneous fistulas after ileal pouch-anal anastomosis for ulcerative colitis. The rarity of this postsurgical complication is emphasized. A good response to topical tacrolimus was achieved in cutaneous wounds. A less favorable response to infliximab was achieved in the abdominal fistulas, requiring surgical excision of the pouch. PMID:17498458

  18. Congenital aganglionosis in a 3-day-old Holstein calf

    PubMed Central

    2005-01-01

    Abstract Necropsy of a 3-day-old Holstein heifer revealed proximal megacolon and distal colorectal hypoplasia. Histologically, the hypoplastic distal colon and rectum lacked submucosal and myenteric ganglia. Clinical history, physical examination, and pathologic findings were consistent with intestinal aganglionosis, a congenital anomaly well documented in humans and foals but not previously reported in cattle. PMID:15943121

  19. Partially hydrolyzed guar gum attenuates the severity of pouchitis in a rat model of ileal J pouch-anal anastomosis.

    PubMed

    Atila, Koray; Terzi, Cem; Canda, Aras Emre; Akhisaroglu, Serpil Tanriverdi; Avci, Hatice Sanli; Sarioglu, Sulen; Oktay, Gulgun; Gulay, Zeynep

    2009-03-01

    We aimed to evaluate the efficacy of treatment with partially hydrolyzed guar gum (PHGG) using a rat model of ileal pouch-anal anastomosis and pouchitis. In the J pouch groups, tissue myeloperoxidase activities were significantly higher than native myeloperoxidase activities (P = 0.020; P = 0.015; P = 0.004, respectively). A statistically significant difference in total histological score was detected in the J pouch + 5% dextran sulfate sodium (DSS) group, compared to the J pouch control and the J pouch + 5% DSS + PHGG groups (P < 0.01 and P < 0.01, respectively). There was a significant overgrowth of aerobes and anaerobes in the J pouch + 5% DSS group. This study demonstrated that rectal administration of PHGG attenuates the severity of pouchitis in a rat model. In conclusion, PHGG may be an additional therapeutic strategy for the treatment of pouchitis. PMID:18594969

  20. Genetics Home Reference: congenital hypothyroidism

    MedlinePlus

    ... Understand Genetics Home Health Conditions congenital hypothyroidism congenital hypothyroidism Enable Javascript to view the expand/collapse boxes. Download PDF Open All Close All Description Congenital hypothyroidism is a partial or complete loss of function ...

  1. Experimental modal analysis of lithium-ion pouch cells

    NASA Astrophysics Data System (ADS)

    Hooper, James Michael; Marco, James

    2015-07-01

    If future electric and hybrid electric vehicle batteries are to be designed such that the impact of vibration induced resonance is minimized, engineers tasked with the design of the vehicle's energy storage system must have a rigorous understanding of key system attributes such as the natural frequencies of the cell, the level of damping present and the mode shapes induced within the battery under mechanical load. This paper describes the underpinning theory and experimental method employed when using the impulse excitation technique to quantify the natural frequencies and mode shapes of a commercially available 25 Ah Nickel Manganese Cobalt Oxide (NMC) Laminate Pouch Cell. Experimental results are presented for fifteen cells at five different values of state of charge (SOC). The results indicate that irrespective of the energy content within the cell, the same four modes of vibration (torsion and bending) exist within a frequency range of 191 Hz-360 Hz. This is above the frequency range (0-150 Hz) typically associated with road-induced vibration. The results also indicate that the cell's natural frequencies of vibration and damping do not vary with changing values of SOC.

  2. Non-Surgical Management of Critically Compromised Airway Due to Dilatation of Interposed Colon

    PubMed Central

    Min, Jinsoo

    2016-01-01

    We present a rare case of critically compromised airway secondary to a massively dilated sequestered colon conduit after several revision surgeries. A 71-year-old male patient had several operations after the diagnosis of gastric cancer. After initial treatment of pneumonia in the pulmonology department, he was transferred to the surgery department for feeding jejunostomy because of recurrent aspiration. However, he had respiratory failure requiring mechanical ventilation. The chest computed tomography (CT) scan showed pneumonic consolidation at both lower lungs and massive dilatation of the substernal interposed colon compressing the trachea. The dilated interposed colon was originated from the right colon, which was sequestered after the recent esophageal reconstruction with left colon interposition resulting blind pouch at both ends. It was treated with CT-guided pigtail catheter drainage via right supraclavicular route, which was left in place for 2 weeks, and then removed. The patient remained well clinically, and was discharged home. PMID:27066087

  3. Intraepithelial lymphocytosis is a frequent finding in biopsies from ileal pouch-anal anastomoses.

    PubMed

    Schaeffer, David F; Walsh, Joanna C; Tyler, Andrea D; Ben-Bassat, Ofer; Silverberg, Mark S; Riddell, Robert H; Kirsch, Richard

    2016-08-01

    Following restorative proctocolectomy with an ileal pouch-anal anastomosis, the small bowel mucosa undergoes several specific histologic adaptions, which may be unrelated to the underlying disease or symptoms of pouchitis. An increase in intraepithelial lymphocytes (IELs) has not been described as part of this spectrum. Mucosal biopsies of the ileal pouch and afferent limb of 230 patients (mean age: 45.7y [18.3-74.7], gender [female/male]: 117/113) with a functioning ileal pouch-anal anastomosis (mean time since ileostomy closure: 10.8months) and associated clinically annotated outcome data were assessed for IELs/100 enterocytes. Forty-two patients (18.3%) showed an increase in IELs (≥20 IELs/100 enterocytes [range 20-39]), in pouch and/or afferent limb biopsies. Intraepithelial lymphocytosis was more commonly observed in afferent limb compared to pouch biopsies (18.8% vs 8.3%; P = .42) and in familial adenomatous polyposis compared to ulcerative colitis patients (16% vs 8%; P = 0.36), but neither difference reached statistical significance. No cases with increased IELs displayed severe villous blunting. Increased IELs were not significantly associated with age, sex, ethnicity, smoking history, time since ileostomy, use of antibiotics, biologic agents, anti-diarrheal agents or probiotics, C-reactive protein levels or differential white cell count. None of the 42 patients with increased IELs had positive celiac serology (anti-human tissue transglutaminase IgA [ELISA] with corresponding total serum IgA). Intraepithelial lymphocytosis in pouch biopsies may represent a subclinical response to an altered bacterial microenvironment. Pathologists should be aware that intraepithelial lymphocytosis is part of the spectrum of changes in pouch biopsies, and only rarely is due to celiac disease. PMID:27063473

  4. A Foilized Polyethylene Pouch for the Prevention of Transmission of HIV from Mother to Child

    PubMed Central

    Malkin, Robert; Howard, Caroline

    2012-01-01

    Many children become HIV+ due to mother-to-child transmission, a risk that can be largely eliminated if infants ingest antiretroviral (ARV) medications immediately after birth. As most mothers in Africa deliver at home, the ARV must be provided at their last antenatal visit, sometimes months before birth. No current drug delivery system allows the mother to store the medication at home long enough to be effective. We propose a preserving, foilized, polyethylene pouch to be pre-dosed and sealed by a pharmacist for later delivery to the newborn. Pouches were filled with 0.6 ml of Nevirapine (NVP). Thirty-three pouches were immediately studied to measure the impact of medication handling (oxygen, light, filling and sealing the pouches). The remaining samples were stored for up to one year at three storage conditions (25°C/60%RH, 30°C/65%RH, and 40°C/75%RH). Every two months, moisture loss, preservative concentration, impurity concentrations and NVP concentration were measured. Flora and fauna challenges were conducted. The pouch nearly eliminated moisture loss: pouches lost less than 0.7% of their weight over twelve months. As expected, exposing the medication to light, oxygen, and handling significantly affected the sacrificial preservative concentrations (Propyl paraben dropped 38%, Methyl paraben dropped 12% at time point zero). However, after the initial time point, preservative levels were stable in the package over twelve months under all storage conditions (4.1% average concentration drop), leaving sufficient preservatives to protect the medication. The concentration of NVP changed an average of only 1.3% over all storage conditions and times points (maximum 1.4%). We conclude that the foilized polyethylene pouch can preserve NVP, and perhaps other ARV’s, for up to one year. PMID:23028405

  5. Giant congenital nevus

    MedlinePlus

    A congenital pigmented or melanocytic nevus is a dark-colored, often hairy, patch of skin. A congenital ... A nevus will appear as a dark-colored patch with any of the ... Hair Regular or uneven borders Smaller affected areas near the ...

  6. Congenital CMV Infection

    MedlinePlus

    ... CMV Babies Born with CMV (Congenital CMV Infection) Language: English Español (Spanish) Recommend on Facebook Tweet Share Compartir ... Detection and Intervention Helping Children With Congenital CMV Language: English Español (Spanish) File Formats Help: How do I ...

  7. Congenital heat disease

    SciTech Connect

    Higgins, C.B.; Silverman, N.H.; Kersting-Somerhoff, B.A.

    1990-01-01

    The book covers the tomographic anatomy of the normal and congenitally malformed heart and tomographic imaging of the normal heat. It then compares echocardiographic evaluation and the use of MR imaging in the diagnosis and evaluation of individual congenital cardiac malformations.

  8. Space colonization.

    PubMed

    2002-12-01

    NASA interest in colonization encompasses space tourism; space exploration; space bases in orbit, at L1, on the Moon, or on Mars; in-situ resource utilization; and planetary terraforming. Activities progressed during 2002 in areas such as Mars colonies, hoppers, and biomass; space elevators and construction; and in-situ consumables. PMID:12506926

  9. Reiterative expression of pax1 directs pharyngeal pouch segmentation in medaka.

    PubMed

    Okada, Kazunori; Inohaya, Keiji; Mise, Takeshi; Kudo, Akira; Takada, Shinji; Wada, Hiroshi

    2016-05-15

    A striking characteristic of vertebrate development is the pharyngeal arches, which are a series of bulges on the lateral surface of the head of vertebrate embryos. Although each pharyngeal arch is segmented by the reiterative formation of endodermal outpocketings called pharyngeal pouches, the molecular network underlying the reiterative pattern remains unclear. Here, we show that pax1 plays crucial roles in pouch segmentation in medaka (Oryzias latipes) embryos. Importantly, pax1 expression in the endoderm prefigures the location of the next pouch before the cells bud from the epithelium. TALEN-generated pax1 mutants did not form pharyngeal pouches posterior to the second arch. Segmental expression of tbx1 and fgf3, which play essential roles in pouch development, was almost non-existent in the pharyngeal endoderm of pax1 mutants, with disturbance of the reiterative pattern of pax1 expression. These results suggest that pax1 plays a key role in generating the primary pattern for segmentation in the pharyngeal endoderm by regulating tbx1 and fgf3 expression. Our findings illustrate the crucial roles of pax1 in vertebrate pharyngeal segmentation and provide insights into the evolutionary origin of the deuterostome gill slit. PMID:27034424

  10. The transmissions of antibodies across the gut of pouch-young marsupials

    PubMed Central

    Yadav, M.

    1971-01-01

    The transmission of antibodies across the gut of suckling pouch-young was investigated in three species of marsupials (Setonix brachyurus, Macropus eugenii and Trichosurus vulpecula) from Australia. Mother Setonix, immunized against Salmonella adelaide flagella and Bacteriophage Φ × 174, transmitted the antibodies in milk to their young. In sucrose density gradient runs, the antibody activity in milk whey and in serum of pouch-young, of Setonix and Macropus was found to be in the 7S region only; antibody in the 11S and 19S regions was not detected. Chromatographic preparations of IgM antibodies were fed to pouch-young Setonix which were later bled and their serum titrated for anti-S. adelaide agglutinins and antiphage Φ × 174 activity. The IgM antibodies were not transmitted across the gut in detectable amounts. Antibodies were present in the blood of pouch-young Setonix within 15–60 minutes of gavage (feeding by stomach tube) of immune serum. In Setonix the capacity to absorb antibodies in the intestine was lost at an age between 170 and 200 days and in Trichosurus it was lost at an age between 98 and 145 days. At these ages the pouch-young were able to leave the marsupium for varying lengths of time. Antibodies did not traverse the rumen wall in a young Setonix whose rumen was isolated from the intestine with ligatures before immune serum was gavaged. PMID:5115612

  11. Bmp signaling mediates endoderm pouch morphogenesis by regulating Fgf signaling in zebrafish.

    PubMed

    Lovely, C Ben; Swartz, Mary E; McCarthy, Neil; Norrie, Jacqueline L; Eberhart, Johann K

    2016-06-01

    The endodermal pouches are a series of reiterated structures that segment the pharyngeal arches and help pattern the vertebrate face. Multiple pathways regulate the complex process of endodermal development, including the Bone morphogenetic protein (Bmp) pathway. However, the role of Bmp signaling in pouch morphogenesis is poorly understood. Using genetic and chemical inhibitor approaches, we show that pouch morphogenesis requires Bmp signaling from 10-18 h post-fertilization, immediately following gastrulation. Blocking Bmp signaling during this window results in morphological defects to the pouches and craniofacial skeleton. Using genetic chimeras we show that Bmp signals directly to the endoderm for proper morphogenesis. Time-lapse imaging and analysis of reporter transgenics show that Bmp signaling is necessary for pouch outpocketing via the Fibroblast growth factor (Fgf) pathway. Double loss-of-function analyses demonstrate that Bmp and Fgf signaling interact synergistically in craniofacial development. Collectively, our analyses shed light on the tissue and signaling interactions that regulate development of the vertebrate face. PMID:27122171

  12. Lateral congenital anomalies of the pharyngeal apparatus: part I. Normal developmental anatomy (embryogenesis) for the surgeon.

    PubMed

    Mirilas, Petros

    2011-09-01

    Knowledge of the embryogenesis of the pharyngeal apparatus is the only means of understanding the "architecture" of the neck. The embryonic pharynx (which includes future oral and nasal cavities) is a much more extensive area than the adult pharynx. The main feature of the developing pharynx is a series of arches, internal pouches, and external clefts, which together comprise the pharyngeal apparatus. This structure is associated with other developing splanchna of the neck, e.g., the thyroid and parathyroid glands, tonsils, and thymus. Within each of the pharyngeal arches are the developing aortic arches and, specific for each arch, cranial nerves. The complex relations of the mesenchymal derivatives of arches (muscles, cartilage, bones) with the neurovascular bundles within each arch are presented and explained. The pharyngeal apparatus undergoes dramatic transformations: pouches and clefts disappear without interruption (interruption would produce gills and support the misnomer "branchial apparatus"). In addition, in the lateroventral neck, somites migrate to produce other muscles such as sternocleidomastoid and trapezius innervated by spinal nerves. Lateral congenital anomalies largely rely on persistence of a cleft/and or pouch or communication between the two. Their tracts have a "crooked" course among other entities generated by alterations that take place during embryogenesis. PMID:21944634

  13. Morphological characteristics of the temporomandibular joint in the pouch young of the Tasmanian devil.

    PubMed

    Hayashi, K; Sugisaki, M; Kino, K; Ishikawa, T; Kawashima, S; Amemiya, T

    2015-04-01

    We recently reported the absence of the articular disc, which is a constant structure in mammals, in the temporomandibular joint of the adult Tasmanian devil. However, whether the articular disc disappears with growth of the animal was unknown. The aim of this study was to determine whether a pouch young of the Tasmanian devil has the articular disc. The temporomandibular joint of a fresh carcass of the pouch young, whose crown-rump length was 43 mm, was examined microscopically and by microcomputed tomography. The absence of the articular disc in the pouch young temporomandibular joint was histologically confirmed. It is suggested that the articular disc of the Tasmanian devil is naturally absent. PMID:24809824

  14. A Novel Model of Surgical Injury in Adult Rat Kidney: A “Pouch Model”

    PubMed Central

    Litbarg, Natalia O.; Vujicic, Snezana; Setty, Suman; Sethupathi, Periannan; Dunea, George; Arruda, Jose A.; Singh, Ashok K.

    2013-01-01

    Regenerative mechanisms after surgical injury have been studied in many organs but not in the kidney. Studying surgical injury may provide new insights into mechanisms of kidney regeneration. In rodent models, extrarenal tissues adhere to surgical kidney wound and interfere with healing. We hypothesized that this can be prevented by wrapping injured kidney in a plastic pouch. Adult rats tolerated 5/6 nephrectomy with pouch application well. Histological analysis demonstrates that application of the pouch effectively prevented formation of adhesions and induced characteristic wound healing manifested by formation of granulation tissue. Additionally, selected tubules of the wounded kidney extended into the granulation tissue forming branching tubular epithelial outgrowths (TEOs) without terminal differentiation. Tubular regeneration outside of renal parenchyma was not previously observed, and suggests previously unrecognized capacity for regeneration. Our model provides a novel approach to study kidney wound healing. PMID:24100472

  15. Energy and process substitution in the frozen-food industry: geothermal energy and the retortable pouch

    SciTech Connect

    Stern, M.W.; Hanemann, W.M.; Eckhouse, K.

    1981-12-01

    An assessment is made of the possibilities of using geothermal energy and an aseptic retortable pouch in the food processing industry. The focus of the study is on the production of frozen broccoli in the Imperial Valley, California. Background information on the current status of the frozen food industry, the nature of geothermal energy as a potential substitute for conventional fossil fuels, and the engineering details of the retortable pouch process are covered. The analytical methodology by which the energy and process substitution were evaluated is described. A four-way comparison of the economics of the frozen product versus the pouched product and conventional fossil fuels versus geothermal energy was performed. A sensitivity analysis for the energy substitution was made and results are given. Results are summarized. (MCW)

  16. Development and Evaluation of a Reflective Solar Disinfection Pouch for Treatment of Drinking Water

    PubMed Central

    Walker, D. Carey; Len, Soo-Voon; Sheehan, Brita

    2004-01-01

    A second-generation solar disinfection (SODIS) system (pouch) was constructed from food-grade, commercially available packaging materials selected to fully transmit and amplify the antimicrobial properties of sunlight. Depending upon the season, water source, and challenge organism, culturable bacteria were reduced between 3.5 and 5.5 log cycles. The system was also capable of reducing the background presumptive coliform population in nonsterile river water below the level of detection. Similar experiments conducted with a model virus, the F-specific RNA bacteriophage MS2, indicated that the pouch was slightly less efficient, reducing viable plaques by 3.5 log units in comparison to a 5.0 log reduction of enterotoxigenic Escherichia coli O18:H11 within the same time period. These results suggest that water of poor microbiological quality can be improved by using a freely available resource (sunlight) and a specifically designed plastic pouch constructed of food-grade packaging materials. PMID:15066858

  17. The Quechua Manta Pouch: A Caretaking Practice for Buffering the Peruvian Infant against the Multiple Stressors of High Altitude.

    ERIC Educational Resources Information Center

    Tronick, E. Z.; And Others

    1994-01-01

    Describes the caretaking practices employed by the Peruvian Quechua mountain people, who swaddle infants in cloths and blankets (known as a manta pouch) to protect them from the harsh and frigid environment. The practices of 14 mother-infants pairs are examined in detail, and the benefits and drawbacks of the manta pouch are examined. (MDM)

  18. Giant African Pouched Rats as Detectors of Tuberculosis in Human Sputum: Comparison of Two Techniques for Sputum Presentation

    ERIC Educational Resources Information Center

    Wray, Alisha M.; Mahoney, Amanda; Weetjens, Bart J.; Cox, Christophe; Jubitana, Maureen; Kazwala, Rudovic; Mfinanga, Godfrey S.; Durgin, Amy; Poling, Alan

    2013-01-01

    Previous studies have shown that pouched rats can detect the presence of "Mycobacterium tuberculosis," which causes tuberculosis, in human sputum samples obtained from clinical facilities. Although pouched rats evaluate sputum samples quickly, preparing the samples is relatively slow. The present study evaluated whether the rats can detect…

  19. Seasonal energy requirements and thermoregulation of growing pouched mice, Saccostomus campestris (Cricetidae)

    NASA Astrophysics Data System (ADS)

    Ellison, G. T. H.; Skinner, J. D.

    1991-06-01

    Pouched mice ( Saccostomus campestris) were born in captivity during January and March and subsequently maintained under long photoperiod (14 h light: 10 h dark) at 25°C. During their first winter (July) and the following summer (January) the pouched mice were exposed to natural photoperiod in an unheated laboratory for 3 weeks prior to measurement. The pouched mice continued to grow during the study, and were significantly heavier after summer exposure than after winter exposure 6 months earlier. Although this increase in body mass would result in a decline in their surface area to volume ratio there was no significant decline in minimal thermal conductance ( C m) and winter-exposed pouched mice had a relatively lower C m than expected. Meanwhile the smaller, winter-exposed animals displayed a significantly higher capacity for non-shivering thermogenesis, together with higher levels of basal metabolism than summer individuals. These differences were not solely attributable to the contrasting body mass of each group and it is therefore clear that S. campestris can increase thermoregulatory heat production, and modify heat loss following exposure to short photoperiod and cold during their first winter. Despite the significant increase in metabolism, the overall energy requirements of small, winter-exposed animals were significantly lower than those for heavier pouched mice following exposure to summer conditions. These results suggest that growing pouched mice can effectively adapt to lower temperature conditions during their first winter, yet accrue considerable overall savings in total energy requirements as a result of their smaller body mass.

  20. Congenital myasthenia gravis.

    PubMed

    Nizamani, Noor Bakht; Talpur, Khalid Iqbal; Memon, Mariya Nazish

    2013-07-01

    Congenital myasthenia gravis is caused by genetic mutations affecting neuromuscular transmission, characterized by muscle weakness usually starting in childhood. A two and a half years old male child presented with bilateral ptosis and hoarseness of voice. The symptoms progressed giving the clinical impression of congenital myasthenia gravis. A series of tests were done including Ice Pack Test, acetylcholine receptor antibody test, trial of steroids and finally neostigmine test which confirmed the diagnosis. This case illustrates the challenges in diagnosing congenital myasthenia gravis and highlights the potential benefits of neostigmine test in its diagnosis. PMID:23823963

  1. Giant African pouched rats (Cricetomys gambianus) that work on tilled soil accurately detect land mines.

    PubMed

    Edwards, Timothy L; Cox, Christophe; Weetjens, Bart; Tewelde, Tesfazghi; Poling, Alan

    2015-09-01

    Pouched rats were employed as mine-detection animals in a quality-control application where they searched for mines in areas previously processed by a mechanical tiller. The rats located 58 mines and fragments in this 28,050-m(2) area with a false indication rate of 0.4 responses per 100 m(2) . Humans with metal detectors found no mines that were not located by the rats. These findings indicate that pouched rats can accurately detect land mines in disturbed soil and suggest that they can play multiple roles in humanitarian demining. PMID:25962550

  2. Localization of gamma-glutamyl transpeptidase in hamster buccal pouch epithelium treated with 7,12-dimethylbenz(a)anthracene

    SciTech Connect

    Solt, D.B.

    1981-07-01

    The utility of gamma-glutamyl transpeptidase (GGT) was explored as a histochemical marker for chemical carcinogenesis in hamster buccal pouch mucosa. One or both buccal pouches of 18 noninbred male Syrian golden hamsters were treated topically with 0.5% 7,12-dimethylbenz(a)anthracene (DMBA) in mineral oil over 16 weeks to produce numerous epithelial lesions at various stages of neoplastic development. Both buccal pouches of 4 control animals were similarly treated with mineral oil alone. GGT activity was not detectable in untreated pouches or pouches treated with mineral oil alone. With this technique, multiple discrete GGT-stained areas were visible in wholemounts prepared at 1 and 6 weeks after the final application of DMBA. The experimental results were consistent with the hypothesis that the early GGT-stained cell populations are preneoplastic in nature.

  3. Congenital heart disease

    MedlinePlus

    ... about genetic counseling and screening if you have a family history of cogenital heart disease. ... Fraser CD, Carberry KE. Congenital heart disease. In: Townsend CM ... Textbook of Cardiovascular Medicine . 10th ed. Philadelphia, PA: ...

  4. Adult Congenital Heart Association

    MedlinePlus

    ... survivable, manageable, yet in the routine years between infancy and adulthood, sometimes forgettable. The Adult Congenital Heart ... understand the continuum of the disease from its infancy. The Adult Congential Heart Association brings together valuable ...

  5. Congenital nephrotic syndrome

    MedlinePlus

    ... may be high. There may be signs of malnutrition. A urinalysis reveals fat and large amounts of ... The disorder often leads to infection, malnutrition, and kidney failure. ... die within the first year. Congenital nephrotic syndrome ...

  6. A colonic duplication cyst causing bowel ischaemia in a 74-year-old lady

    PubMed Central

    Fenelon, Christopher; Boland, Michael R; Kenny, Brian; Faul, Peter; Tormey, Shona

    2016-01-01

    Colonic duplication cysts are rare congenital malformations that predominantly present before the age of 2 years. We report the case of a 74-year-old lady who presented with sudden onset abdominal pain. A computed tomography scan noted a calcified structure adjacent to abnormal loops of bowel. Intraoperative findings revealed an ischaemic loop of small bowel wrapped around a mass in the mesentery adjacent to the sigmoid colon. Final histology revealed a colonic duplication cyst. Colonic duplication cysts are rare entities that most commonly cause obstruction or perforation. We present the very rare case of a colonic duplication cyst causing bowel ischaemia in an elderly female. PMID:27572680

  7. A Case of Extragonadal Teratoma in the Pouch of Douglas and Literature Review.

    PubMed

    Kakuda, Mamoru; Matsuzaki, Shinya; Kobayashi, Eiji; Yoshino, Kiyoshi; Morii, Eiichi; Kimura, Tadashi

    2015-01-01

    Mature cystic teratoma is a germ cell tumor of the ovaries and is often observed in clinical practice. However, extragonadal teratomas are rare tumors and have been reported outside the ovaries, (e.g., in the greater omentum). The mechanism underlying the development of extragonadal teratomas remains unknown. We encountered a case of extragonadal teratoma in the pouch of Douglas that appeared to be a parasitic dermoid cyst. From our experience and the literature review, we discuss the potential mechanism leading to the development of extragonadal teratomas. A 41-year-old nonpregnant woman was referred to our department due to myoma and anemia. A 4-cm asymptomatic mass in the pouch of Douglas was observed, and the patient was diagnosed with ovarian mature cystic teratoma. She underwent laparoscopic surgery, and intraoperative findings revealed that the fallopian tube was injured and torn, and a residual small ovary was observed in the left side of the ovary. A tumor measuring approximately 4 cm observed in the pouch of Douglas was extracted without rupturing. The tumor was diagnosed as a parasitic dermoid cyst by macroscopic and histopathological findings. Auto-amputation could be the underlying mechanism that leads to an isolated parasitic dermoid cyst in the pouch of Douglas. PMID:26205577

  8. Ethanolic leaf extract of neem (Azadirachta indica) inhibits buccal pouch carcinogenesis in hamsters.

    PubMed

    Subapriya, R; Bhuvaneswari, V; Ramesh, V; Nagini, S

    2005-01-01

    We evaluated the chemopreventive effects of ethanolic neem leaf extract in the initiation and post-initiation phases of 7,12-dimethylbenz[a]anthracene (DMBA)-induced hamster buccal pouch (HBP) carcinogenesis. The frequency of bone marrow micronuclei as well as the concentrations of lipid peroxides, ratio of reduced to oxidized glutathione (GSH/GSSG), and the activities of the GSH-dependent enzymes glutathione peroxidase (GPx) and glutathione-S-transferase (GST) in the buccal pouch, liver and erythrocytes were used as biomarkers of chemoprevention. All the hamsters painted with DMBA alone for 14 weeks developed buccal pouch carcinomas that showed diminished lipid peroxidation and enhanced antioxidant status associated with increased frequencies of bone marrow micronuclei. In the liver and erythrocytes of tumour-bearing animals, enhanced lipid peroxidation was accompanied by compromised antioxidant defences. Administration of ethanolic neem leaf extract effectively suppressed DMBA-induced HBP carcinogenesis as revealed by the absence of tumours in the initiation phase and reduced tumour incidence in the post-initiation phase. In addition, ethanolic neem leaf extract modulated lipid peroxidation and enhanced antioxidant status in the pouch, liver and erythrocytes and reduced the incidence of bone marrow micronuclei. The results of the present study, demonstrate that ethanolic neem leaf extract inhibits the development of DMBA-induced HBP tumours by protecting against oxidative stress. PMID:15473007

  9. Mycotic encephalitis, sinus osteomyelitis, and guttural pouch mycosis in a 3-year-old Arabian colt.

    PubMed

    Hunter, Barbara; Nation, Patrick N

    2011-12-01

    Mycotic encephalitis caused severe ataxia and other neurologic deficits in a horse. The finding of a single, large focus of cerebral malacia, with histopathologic evidence of fungal elements, suggested infection was a result of direct transfer from the frontal sinuses, rather than hematogenous spread from the guttural pouch. PMID:22654140

  10. Using Trained Pouched Rats to Detect Land Mines: Another Victory for Operant Conditioning

    ERIC Educational Resources Information Center

    Poling, Alan; Weetjens, Bart; Cox, Christophe; Beyene, Negussie W.; Bach, Harvard; Sully, Andrew

    2011-01-01

    We used giant African pouched rats ("Cricetomys gambianus") as land mine-detection animals in Mozambique because they have an excellent sense of smell, weigh too little to activate mines, and are native to sub-Saharan Africa, and therefore are resistant to local parasites and diseases. In 2009 the rats searched 93,400 m[superscript 2] of land,…

  11. Using Trained Pouched Rats (Cricetomys Gambianus) to Detect Land Mines: Another Victory for Operant Conditioning

    ERIC Educational Resources Information Center

    Poling, Alan; Weetjens, Bart; Cox, Christophe; Beyene, Negussie W.; Bach, Harvard; Sully, Andrew

    2011-01-01

    We used giant African pouched rats ("Cricetomys gambianus") as land mine-detection animals in Mozambique because they have an excellent sense of smell, weigh too little to activate mines, and are native to sub-Saharan Africa, and therefore are resistant to local parasites and diseases. In 2009 the rats searched 93,400 m[superscript 2] of land,…

  12. Patterning of the third pharyngeal pouch into thymus/parathyroid by Six and Eya1.

    PubMed

    Zou, Dan; Silvius, Derek; Davenport, Julie; Grifone, Raphaelle; Maire, Pascal; Xu, Pin-Xian

    2006-05-15

    Previous studies have suggested a role of the homeodomain Six family proteins in patterning the developing vertebrate head that involves appropriate segmentation of three tissue layers, the endoderm, the paraxial mesoderm and the neural crest cells; however, the developmental programs and mechanisms by which the Six genes act in the pharyngeal endoderm remain largely unknown. Here, we examined their roles in pharyngeal pouch development. Six1-/- mice lack thymus and parathyroid and analysis of Six1-/- third pouch endoderm demonstrated that the patterning of the third pouch into thymus/parathyroid primordia is initiated. However, the endodermal cells of the thymus/parathyroid rudiments fail to maintain the expression of the parathyroid-specific gene Gcm2 and the thymus-specific gene Foxn1 and subsequently undergo abnormal apoptosis, leading to a complete disappearance of organ primordia by E12.5. This thus defines the thymus/parathyroid defects present in the Six1 mutant. Analyses of the thymus/parathyroid development in Six1-/-;Six4-/- double mutant show that both Six1 and Six4 act synergistically to control morphogenetic movements of early thymus/parathyroid tissues, and the threshold of Six1/Six4 appears to be crucial for the regulation of the organ primordia-specific gene expression. Previous studies in flies and mice suggested that Eya and Six genes may function downstream of Pax genes. Our data clearly show that Eya1 and Six1 expression in the pouches does not require Pax1/Pax9 function, suggesting that they may function independently from Pax1/Pax9. In contrast, Pax1 expression in all pharyngeal pouches requires both Eya1 and Six1 function. Moreover, we show that the expression of Tbx1, Fgf8 and Wnt5b in the pouch endoderm was normal in Six1-/- embryos and slightly reduced in Six1-/-;Six4-/- double mutant, but was largely reduced in Eya1-/- embryos. These results indicate that Eya1 appears to be upstream of very early events in the initiation of thymus

  13. Changes in the absorption of bile acids after total colectomy in patients with an ileostomy or pouch-anal anastomosis

    SciTech Connect

    Nasmyth, D.G.; Johnston, D.; Williams, N.S.; King, R.F.; Burkinshaw, L.; Brooks, K.

    1989-03-01

    Bile acid absorption was investigated using /sup 75/Se Taurohomocholate (SeHCAT) in controls and patients who had undergone total colectomy with either conventional ileostomy or pouch-anal anastomosis for ulcerative colitis or adenomatous polyposis. Whole-body retention of SeHCAT after 168 hours was greater in the controls than the patients who had undergone colectomy (P less than .05). Retention of SeHCAT did not differ significantly between patients with an ileostomy and patients with pouch-anal anastomosis, but patients with an ileostomy and ileal resection of more than 20 cm retained less SeHCAT than patients with a pouch-anal anastomosis (P less than .01). Analysis of fecal bile acids from ileostomies and pouches showed that bacterial metabolism of primary conjugated bile acids was greater in patients with a pouch. It was concluded that bile acid absorption was not significantly impaired by construction of a pouch compared with conventional ileostomy, but bacterial metabolism of bile acids was greater in the pouches.

  14. Colon cancer - resources

    MedlinePlus

    Resources - colon cancer ... The following organizations are good resources for information on colon cancer : American Cancer Society -- www.cancer.org/cancer/colonandrectumcancer/index Colon Cancer Alliance -- www.ccalliance.org National ...

  15. Measuring neoplastic transformation in the hamster cheek pouch using Fourier domain low-coherence interferometry

    NASA Astrophysics Data System (ADS)

    Graf, Robert N.; Chen, Xiaoxin; Brown, William; Wax, Adam

    2008-02-01

    Fourier Domain Low Coherence Interferometry (fLCI) is a promising technique which combines the depth resolution of low coherence interferometry with the sensitivity of light scattering spectroscopy for probing the health of epithelial tissue layers. Our new fLCI system configuration utilizes a white light Xe arc lamp source and a 4-f interferometer which re-images light scattered from the sample onto the detection plane. The system employs an imaging spectrometer at the detection plane to acquire depth resolved profiles from 252 adjacent spatial points without the need for any scanning. The limited spatial coherence of the light source requires the resolution of adjacent spatial points for the generation of depth information. Depth-resolved spectral information is recovered by performing a short-time Fourier transform on the detected spectra, similar to spectroscopic optical coherence tomography. Wavelength dependent variations in scattering intensity are analyzed as a function of depth to obtain information about the neoplastic transformation of the probed cells. Previous studies have demonstrated fLCI as an excellent technique for probing the scatterer morphology of simple phantoms and of in vitro cancer cell monolayers. We now seek to assess the ability of the new fLCI system to measure the health of subsurface tissue layers using the hamster cheek pouch model. Seven hamsters will have one cheek pouch treated with the known carcinogen DMBA. At the conclusion of the 24 week treatment period the animals will be anesthetized and the cheek pouches will be extracted. We will use the fLCI optical system to measure the neoplastic transformation of the in situ subsurface tissue layers in both the normal and DMBA-treated cheek pouches. Traditional histological analysis will be used to verify the fLCI measurements. We expect our results to establish the feasibility of fLCI to distinguish between healthy and dysplastic epithelial tissues in the hamster cheek pouch.

  16. The gene for congenital chloride diarrhea maps close to but is distinct from the gene for cystic fibrosis transmembrane conductance regulator

    SciTech Connect

    Kere, J.; de la Chapelle, A.; Holmberg, C. ); Sistonen, P. Finnish Red Cross Blood Transfusion Service, Helsinki )

    1993-11-15

    Congenital chloride diarrhea (CLD) is characterized by watery stools with high chloride content beginning prenatally and is inherited as an autosomal recessive trait. Perfusion studies have established a basic defect in ileal and colonic Cl[sup [minus

  17. Genetics of Congenital Cataract.

    PubMed

    Pichi, Francesco; Lembo, Andrea; Serafino, Massimiliano; Nucci, Paolo

    2016-01-01

    Congenital cataract is a type of cataract that presents at birth or during early childhood, and it is one of the most easily treatable causes of visual impairment and blindness during infancy, with an estimated prevalence of 1-6 cases per 10,000 live births. Approximately 50% of all congenital cataract cases may have a genetic cause, and such cases are quite heterogeneous. Although congenital nuclear cataract can be caused by multiple factors, genetic mutation remains the most common cause. All three types of Mendelian inheritance have been reported for cataract; however, autosomal dominant transmission seems to be the most frequent. The transparency and high refractive index of the lens are achieved by the precise architecture of fiber cells and homeostasis of the lens proteins in terms of their concentrations, stabilities, and supramolecular organization. Research on hereditary congenital cataract has led to the identification of several classes of candidate genes that encode proteins such crystallins, lens-specific connexins, aquaporin, cytoskeletal structural proteins, and developmental regulators. In this review, we highlight the identified genetic mutations that account for congenital nuclear cataract. PMID:27043388

  18. Totally Transanal Laparo-Endoscopic Single-Site ProctoColectomy-Ileoanal J-Pouch (TLPC-J): An Experimental Study of a Novel Approach

    PubMed Central

    Vahdad, Mohammad Reza; Rahmanian, Ehsan; Moslemi, Sam; Najafi, Sultan Mohsen; Foroutan, Hamid Reza

    2015-01-01

    Background: The natural orifice transluminal endoscopic surgery (NOTES) has become a commonly considered novel approach in the surgical field. The NOTES provide possibility of operation through the natural orifice and decreases the intentional puncture of the systemic organ and subsequent complications. Totally transanal laparo-endoscopic single-site proctoColectomy-Ileoanal J-Pouch (TLPC-J) is a novel method in minimally invasive surgery for total colectomy. The main goal of this study is to perform this new method on an animal model, to assess probable complication and to resolve probable issues by using patients that are candidate for total colectomy. Method: Five dogs were prepared in lithotomy position. The TLPC-I procedure consists of endorectal technique with full thickness rectal dissection starting 1 cm orally from the dentate line above the peritoneal reflection and the proximal bowel was replaced into the abdominal cavity. Afterwards, the TriPort system was inserted in the anal canal and mesentrial resection of the total colon, mobilization of a distal ileal segment and intracorporeal suture of an ileal J-loop was accomplished by this system. An incision in the J-loop was conducted transanally. The J-pouch was created with an Endo-GIA® and sutured to the rectal wall. Results: All animals survived and passed stool with clear post operation situation. There was no infection in site of anastomosis. Conclusion: The TLPC-I provides the possibility of surgery without abdominal wall incision and decreases post operation complication such as pain, abdominal wound infection and wound dehiscence. This technique increases the quality of life and surgeons can discharge the patients early. PMID:26379349

  19. Adults with Congenital Heart Defects

    MedlinePlus

    ... Pressure High Blood Pressure Tools & Resources Stroke More Web Booklet: Adults With Congenital Heart Defects Updated:Apr ... topic from the list below to learn more. Web Booklet: Adults With Congenital Heart Defects Introduction Introduction: ...

  20. Congenital heart defect - corrective surgery

    MedlinePlus

    ... born with one or more heart defects has congenital heart disease . Surgery is needed if the defect could harm ... 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/ ...

  1. Impact of Congenital Heart Defects

    MedlinePlus

    ... complex lesions, limitations are common. Some children with congenital heart disease have developmental delay or other learning difficulties. What ... defects? Successful treatment requires highly specialized care. Severe congenital heart disease requires extensive financial resources both in and out ...

  2. Congenital tracheobronchial stenosis.

    PubMed

    Hewitt, Richard J; Butler, Colin R; Maughan, Elizabeth F; Elliott, Martin J

    2016-06-01

    Congenital tracheobronchial stenosis is a rare disease characterized by complete tracheal rings that can affect variable lengths of the tracheobronchial tree. It causes high levels of morbidity and mortality both due to the stenosis itself and to the high incidence of other associated congenital malformations. Successful management of this complex condition requires a highly individualized approach delivered by an experienced multidisciplinary team, which is best delivered within centralized units with the necessary diverse expertise. In such settings, surgical correction by slide tracheoplasty has become increasingly successful over the past 2 decades such that long-term survival now exceeds 88%, with normalization of quality of life scores for patients with non-syndrome-associated congenital tracheal stenosis. Careful assessment and planning of treatment strategies is of paramount importance for both successful management and the provision of patients and carers with accurate and realistic treatment counseling. PMID:27301600

  3. Congenital hyperinsulinism with hyperammonaemia

    PubMed Central

    Pschibul, Alex; Müller, Jörg; Fahnenstich, Hubert

    2010-01-01

    Congenital hyperinsulinism is considered to be the most frequent cause of persistent recurrent hypoglycaemia in infants. The clinical presentation and response to pharmacological treatment may vary significantly depending on the underlying pathology. We report a case of a female infant with mild but early onset of recurrent hypoglycaemia. Metabolic workup revealed hyperinsulinism combined with mild hyperammonaemia as well as elevation of α-ketoglutarate in urine. Genetic testing demonstrated a de novo mutation in exon 7 of the glutamate dehydrogenase gene on chromosome 10. Episodes of hypoglycaemia responded to treatment with diazoxide. The differential diagnosis, pathophysiology and treatment of congenital hyperinsulinism is discussed. PMID:22315648

  4. Congenital Adrenal Hyperplasia

    PubMed Central

    Speiser, Phyllis W.

    2015-01-01

    Congenital adrenal hyperplasia associated with deficiency of steroid 21-hydroxylase is the most common inborn error in adrenal function and the most common cause of adrenal insufficiency in the pediatric age group. As patients now survive into adulthood, adult health-care providers must also be familiar with this condition. Over the past several years, F1000 has published numerous commentaries updating research and practical guidelines for this condition. The purposes of this review are to summarize basic information defining congenital adrenal hyperplasia and to highlight current knowledge and controversies in management. PMID:26339484

  5. Congenital midline nasofrontal masses.

    PubMed

    Saettele, Megan; Alexander, Alan; Markovich, Brian; Morelli, John; Lowe, Lisa H

    2012-09-01

    Congenital midline nasal masses are uncommon anomalies including nasal dermoids/epidermoids, nasal glial heterotopias and encephaloceles. These lesions can occur at the nasal bridge, extend intranasally and have intracranial extension with communication to the subarachnoid space. Therefore, accurate diagnosis of these lesions is critically important for presurgical planning and prevention of potentially fatal complications. Neuroimaging is essential in the evaluation of congenital midline nasal masses to identify the specific type of lesion, evaluate for the presence of intracranial extension and allow for appropriate presurgical planning. PMID:22648391

  6. Congenital muscular torticollis.

    PubMed

    Nilesh, Kumar; Mukherji, Srijon

    2013-07-01

    Congenital muscular torticollis (CMT) is a rare congenital musculoskeletal disorder characterized by unilateral shortening of the sternocleidomastoid muscle (SCM). It presents in newborn infants or young children with reported incidence ranging from 0.3% to 2%. Owing to effective shortening of SCM on the involved side there is ipsilateral head tilt and contralateral rotation of the face and chin. This article reports a case of CMT in a 3½-year-old male child successfully managed by surgical release of the involved SCM followed by physiotherapy. PMID:24205484

  7. Congenital muscular torticollis

    PubMed Central

    Nilesh, Kumar; Mukherji, Srijon

    2013-01-01

    Congenital muscular torticollis (CMT) is a rare congenital musculoskeletal disorder characterized by unilateral shortening of the sternocleidomastoid muscle (SCM). It presents in newborn infants or young children with reported incidence ranging from 0.3% to 2%. Owing to effective shortening of SCM on the involved side there is ipsilateral head tilt and contralateral rotation of the face and chin. This article reports a case of CMT in a 3½-year-old male child successfully managed by surgical release of the involved SCM followed by physiotherapy. PMID:24205484

  8. Surgical considerations in FAP-related pouch surgery: Could we do better?

    PubMed

    Möslein, Gabriela

    2016-07-01

    The ileoanal pouch has become the standard restorative procedure of choice for patients with the classical phenotype in FAP (familial adenomatous polyposis) and also for ulcerative colitis (UC). Whilst we tend to encounter descriptive analyses comparing functional outcome, fertility and quality of life (QOL) between series in literature, there may be an urgent need to discuss the subtle technical modifications that may be pivotal for improving long-term QOL in FAP patients. Our aim is to review the current literature and discuss the aspects of ileal pouch-anal anastomosis that may require specific reevaluation for FAP. Surgical strategies aimed at minimizing post-interventional desmoid growth is one of the most important aspects. For this study, the following topics of interest were selected: Timing of surgery, IRA or ileoanal pouch for classical FAP, laparoscopic or conventional surgery, TME or mesenteric dissection, preservation of the ileocolic vessels, handsewn or double-staple anastomosis, shape and size of pouch, protective ileostomy, Last and definitely not least: how to manage desmoid plaques or desmoids at the time of prophylactic surgery. For the depicted technicalities of the procedure, a review of recent literature was performed and evaluated. For the topics selected, only sparse reference in literature was identified that was focused on the specific condition situation of FAP. Almost all pouch literature focusses on the procedural aspects, and FAP patients are always a very minor number. Therefore it becomes obvious that the specific entity is not adequately taken into account. This is a serious bias for identification of important steps in the procedure that may be beneficial for patients with either of the diseases. The results of this study demonstrate that several technical differences for construction of ileoanal pouches in FAP patients deserve more attention and prospective evaluation-perhaps even randomized trials. The role, importance and

  9. Imaging of congenital pulmonary malformations.

    PubMed

    Praticò, Francesco Emanuele; Corrado, Michele; Della Casa, Giovanni; Parziale, Raffaele; Russo, Giuseppe; Gazzani, Silvia Eleonora; Rossi, Enrica; Borgia, Daniele; Mostardi, Maurizio; Bacchini, Emanuele; Cella, Simone; De Filippo, Massimo

    2016-01-01

    Congenital pulmonary malformations represent a broad spectrum of anomalies that may result in varied clinical and pathologic pictures, ranging from recurrent pulmonary infections and acute respiratory distress syndrome, which require timely drug therapy, up to large space-occupying lesions needing surgical treatment. This classification includes three distinct anatomical and pathological entities, represented by Congenital Cystic Adenomatoid Malformation, Bronchopulmonary Sequestration and Congenital Lobar Emphysema. The final result in terms of embryological and fetal development of these alterations is a Congenital Lung Hypoplasia. Since even Bronchial Atresia, Pulmonary Bronchogenic Cysts and Congenital Diaphragmatic Hernias are due to Pulmonary Hypoplasia, these diseases will be discussed in this review (1, 2). PMID:27467867

  10. The congenital cranial dysinnervation disorders.

    PubMed

    Gutowski, N J; Chilton, J K

    2015-07-01

    Congenital cranial dysinnervation disorders (CCDD) encompass a number of related conditions and includes Duane syndrome, congenital fibrosis of the external ocular muscles, Möbius syndrome, congenital ptosis and hereditary congenital facial paresis. These are congenital disorders where the primary findings are non-progressive and are caused by developmental abnormalities of cranial nerves/nuclei with primary or secondary dysinnervation. Several CCDD genes have been found, which enhance our understanding of the mechanisms involved in brain stem development and axonal guidance. PMID:25633065

  11. Congenital alopecia universalis.

    PubMed

    Saraswat, P K; Laha, N N

    1989-09-01

    A case of congenital alopecia universalis without any other ectodermal defect and mental abnormality is described in a girl of eight years. There was no family history in any of the members. The child was born of a non-consanguineous marriage. PMID:2632563

  12. OPERATION FOR CONGENITAL CATARACT

    PubMed Central

    Barkan, Otto

    1949-01-01

    The traditional treatment of needling or discission of congenital cataract or membrane is open to many serious objections. Removal of the cataract by a modified form of linear extraction is recommended. The technique, with recent improvements which further assure extraction without hazard in early infancy, is described. PMID:18125222

  13. Congenital adrenal hyperplasia

    MedlinePlus

    ... or inappropriately). Congenital adrenal hyperplasia can affect both boys and girls. About 1 in 10,000 to 18,000 ... penis but normal testes Well-developed muscles Both boys and girls will be tall as children, but much shorter ...

  14. Congenital Midline Cervical Cleft

    PubMed Central

    Villanueva-Meyer, Javier; Glastonbury, Christine; Marcovici, Peter

    2015-01-01

    Congenital midline cervical cleft is a rare anomaly that typically presents in the neonatal period as a thin suprasternal vertical band of erythematous skin with a nipple-like projection superiorly, which may exude fluid. We present the clinical and pathophysiologic features and the imaging findings of this uncommon, and rarely described entity in a newborn girl. PMID:25926928

  15. Gastric-jejunum pouch side-to-end anastomosis: a novel and safe operation of gastrojejunostomy for preventing reflux gastritis

    PubMed Central

    Cao, Yongkuan; Gong, Jiaqing; Gan, Wei; Zhou, Jun; Huang, Ling; Wang, Yonghua; Luo, Guode; Song, Yaning

    2015-01-01

    Purpose: This study aims to introduce a simple operation method of gastrojejunostomy for preventing reflux esophagitis --gastric-jejunum pouch side-to-end anastomosis. Methods: Based on Billroth II anastomosis (Billroth II) method, we designed a new technique of anastomosis between stomach wall and jejunal pouch. The technique was named gastric-jejunum pouch side-end anastomosis. We retrospectively analyzed the clinical data which was collected from March 2012 to February 2014. Among all the recruited 66 patients, 51 gastric cancer patients and 7 pyloric obstruction patients were implemented with hand-assisted laparoscopic distal gastrectomy plus D2 lymph node dissection. The remaining 8 patients were malignant pyloric obstruction; they were treated with small exploratory incision on the upper abdomen and distal gastric partial transection. All the patients were treated with gastric-jejunum pouch side-to-end anastomosis. Results: The surgical incision was small, the operative time was short, their bleeding volume was little, the recovery time of the bowel function and hospitalization time was relatively short. Postoperatively, there was neither mortality and gastroparesis, nor anastomotic leakage and jejunal pouch leakage. Minor postoperative complications were detected followed up for 12 months, no acid reflux and reflux esophagitis symptoms was reported. Conclusions: Gastric -jejunum pouch side-to-end anastomosis is a simple operation technique with highly surgical safety. PMID:26131135

  16. Indiana pouch continent urinary reservoir in patients with previous pelvic irradiation

    SciTech Connect

    Mannel, R.S.; Braly, P.S.; Buller, R.E. )

    1990-05-01

    Little information exists on the use of continent urinary reservoirs in patients with previous pelvic irradiation. We report the use of the Indiana pouch urinary reservoir in ten women with a history of pelvic irradiation for cervical cancer, of whom eight underwent a total pelvic exenteration for recurrent pelvic tumor and two had diversion for radiation-induced vesicovaginal fistula. All ten women achieved daytime continence, with a median time between catheterizations of 4.5 hours and a median pouch capacity of 500 mL. There was no evidence of leakage from the reservoir or significant ureteral reflux or obstruction on postoperative radiographic evaluation. No patient has required reoperation or had significant postoperative complications with the technique described.

  17. The relationship between hatching rate and number of embryos of the brood pouch in Littorina saxatilis

    NASA Astrophysics Data System (ADS)

    Conde-Padín, P.; Carballo, M.; Caballero, A.; Rolán-Alvarez, E.

    2008-10-01

    Littorina saxatilis is an ovoviviparous organism in which shelled embryos can be directly observed in nearly all mature females captured in the wild. This characteristic has been used a number of times as an indirect estimate for fecundity of natural populations. However, there is no experimental corroboration that the number of embryos within a female brood pouch is actually related to the rate of hatching per unit of time, a more realistic estimate of female fecundity. In order to make this corroboration we estimated the correlation between the number of embryos in the brood pouch and the hatching rate of isolated females grown in the laboratory. We also compared the hatching rates of females from two sympatric ecotypes (RB and SU) of this species, which differ significantly in the number of embryos. We found a high agreement between hatching rate and number of embryos, concluding that the latter is a good proxy for fecundity.

  18. [Necrosis of the pouch due to nontraumatic rupture of the pile during permanent endocardial electrostimulation].

    PubMed

    Circo, A; Mangiameli, S; Vanaria, D; Lombardo, D; Evola, R

    1977-09-30

    Necrosis of the pouch containing the generator, due to non-traumatic breakage of the batteries, was observed in a patient with a permanent pacemaker, followed by rapid ingravescence. The cause of this complication is discussed. Chemical inflammation was noted soon after the pacemaker ceased to function. Initially this was similar to the not uncommon bacterial inflammation noted in such patients. Here, of course, the pacemaker usually continues to work, and its replacement is optional, whereas in the reported case is an emergency matter. In situations where tissue distress in the pouch is accompanied by inflammation, therefore, thought should be given to the possibility that, even if the batteries are still working, the cause may be chemical or electrochemical rather than septic, and than sudden breakdown of the generator may be expected. PMID:917334

  19. Nd:YAG photovaporization of residual equine guttural-pouch mycotic lesions after internal carotid occlusion

    NASA Astrophysics Data System (ADS)

    Tate, Lloyd P.; Tudor, R. A.; Little, Edwina D. E.

    1998-07-01

    Hospital records reviewed over a 12-year period determined that 6 horses had been presented for guttural pouch mycosis. All had a history of epistaxis and several had accompanying neurologic signs, including dysphagia. Initially, conformation of the disease was obtained by radiographic and endoscopic examinations. Surgical treatment was directed at controlling and preventing additional hemorrhage by occluding the arterial source of the hemorrhage. Direct treatment of the mycotic plaque present within the guttural pouch was conducted using transendoscopic technique to direct irradiation from the Nd:YAG laser. The protocol described for the laser application was successful in resolving the lesions and was less complicated and stressful than previously reported topically applied chemical protocols.

  20. The Primary Implantation of Human Tumours to the Hamster Cheek Pouch

    PubMed Central

    Williams, Dorothy E.; Evans, D. M. D.; Blamey, R. W.

    1971-01-01

    The hamster cheek pouch is an immunologically privileged site. The present study is of simple implantation of human tumours direct from operative specimen to cheek pouch, in particular to determine whether tumour type influences the rate of successful implant. All implants were studied 10 or 20 days later. The use of cortisone significantly improved the number of implants growing. Carcinomas of the cervix were found to show growth in 55% of implants, in animals conditioned with cortisone. Growth from tumours of the uterine body, or from colorectal carcinomas, occurred in 25-30% of implants. Breast cancer gave poor results. ImagesFig. 1Fig. 2Fig. 3Fig. 4Fig. 5 PMID:5144526

  1. Teaching Giant African Pouched Rats to Find Landmines: Operant Conditioning With Real Consequences

    PubMed Central

    Poling, Alan; Weetjens, Bart J; Cox, Christophe; Beyene, Negussie; Bach, Håvard; Sully, Andrew

    2010-01-01

    Giant African pouched rats recently have been used as mine-detection animals in Mozambique. To provide an example of the wide range of problems to which operant conditioning procedures can be applied and to illustrate the common challenges often faced in applying those procedures, this manuscript briefly describes how the rats are trained and used operationally. To date, the rats have performed well and it appears they can play a valuable role in humanitarian demining. PMID:22532890

  2. Teaching giant african pouched rats to find landmines: operant conditioning with real consequences.

    PubMed

    Poling, Alan; Weetjens, Bart J; Cox, Christophe; Beyene, Negussie; Bach, Håvard; Sully, Andrew

    2010-01-01

    Giant African pouched rats recently have been used as mine-detection animals in Mozambique. To provide an example of the wide range of problems to which operant conditioning procedures can be applied and to illustrate the common challenges often faced in applying those procedures, this manuscript briefly describes how the rats are trained and used operationally. To date, the rats have performed well and it appears they can play a valuable role in humanitarian demining. PMID:22532890

  3. Detecting neoplastic development in the hamster cheek pouch using Fourier domain low coherence interferometry

    NASA Astrophysics Data System (ADS)

    Graf, Robert N.; Robles, Francisco; Chen, Xiaoxin; Wax, Adam

    2009-02-01

    Fourier Domain Low Coherence Interferometry (fLCI) is an optical technique that recovers depth-resolved spectroscopic information about scatterers. The current fLCI system utilizes a white light Xe arc lamp source, a 4-f interferometer, and an imaging spectrometer at the detection plane to acquire spectra from 256 adjacent spatial points. This configuration permits the acquisition of ultrahigh depth resolution Fourier domain OCT images without the need for any beam scanning. fLCI has traditionally obtained depth-resolved spectral information by performing a short-time Fourier transform (STFT) on the detected spectra, similar to the processing techniques of spectroscopic OCT. We now employ a dual Gaussian window processing method which simultaneously obtains high spectral and temporal resolution, thus avoiding the resolution trade-off normally associated with the STFT. Wavelength dependent variations in scattering intensity are analyzed as a function of depth to obtain structural information about the probed scatterers. We now verify fLCI's ability to distinguish between normal and dysplastic epithelial tissue using the hamster cheek pouch model. Thirty hamsters will have one cheek pouch treated with the known carcinogen DMBA. At the conclusion of the 24 week treatment period the animals will be anesthetized and the cheek pouches will be extracted. We will use the fLCI optical system to measure the neoplastic transformation of the in situ subsurface tissue layers in both the normal and DMBA-treated cheek pouches. Traditional histological analysis will be used to verify the fLCI measurements. Our results will further establish fLCI as an effective method for distinguishing between normal and dysplastic epithelial tissues.

  4. Modulation of xenobiotic-metabolizing enzymes by ethanolic neem leaf extract during hamster buccal pouch carcinogenesis.

    PubMed

    Subapriya, R; Velmurugan, B; Nagini, S

    2005-06-01

    Chemoprevention by medicinal plants is a promising approach for controlling cancer. There is substantial evidence to indicate that chemopreventive agents exert their anticarcinogenic effects by modulation of phase I and phase II xenobiotic-metabolizing enzymes. Therefore, we examined the chemopreventive potential of ethanolic neem leaf extract (ENLE) on 7,12-dimethylbenz[a]anthracene (DMBA)-induced hamster buccal pouch (HBP) carcinogenesis. Hamsters were divided into four groups of six animals each. The right buccal pouches of animals in Group I were painted with 0.5 per cent DMBA in liquid paraffin three times per week. Animals in Group 2 painted with DMBA as in group 1, received in addition, intragastric administration of ENLE at a concentration of 200 mg/kg bw three times per week on days alternate to DMBA application. Group 3 was given ENLE alone. Animals in Group 4 served as controls. All animals were killed after an experimental period of 14 weeks. Five out of six hamsters painted with DMBA alone developed squamous cell carcinomas in the buccal pouch. The HBP tumours showed an increase in phase I carcinogen activation (cytochrome P450 and b5) and phase II detoxification enzyme (glutathione-S-transferase, DT-diaphorase and NADPH-diaphorase) activities. In the liver of tumour-bearing animals, enhanced cytochrome P450 and b5 levels were accompanied by a decrease in phase II detoxification enzyme activities. Administration of ENLE effectively suppressed DMBA-induced HBP tumours, decreased cytochrome P450 and b5 levels, and enhanced phase II enzyme activities in the pouch and liver. Our results suggest that the modulation of DMBA metabolism is a possible mechanism for the chemopreventive effects of ethanolic neem leaf extract. PMID:16110755

  5. Modeling Lithium Ion Battery Safety: Venting of Pouch Cells; NREL (National Renewable Energy Laboratory)

    SciTech Connect

    Santhanagopalan, Shriram.; Yang, Chuanbo.; Pesaran, Ahmad

    2013-07-01

    This report documents the successful completion of the NREL July milestone entitled “Modeling Lithium-Ion Battery Safety - Complete Case-Studies on Pouch Cell Venting,” as part of the 2013 Vehicle Technologies Annual Operating Plan with the U.S. Department of Energy (DOE). This work aims to bridge the gap between materials modeling, usually carried out at the sub-continuum scale, and the

  6. Effects of chlorhexidine on the structure and permeability of hamster cheek pouch mucosa

    SciTech Connect

    Harvey, B.V.; Squier, C.A.; Hall, B.K.

    1984-10-01

    This study examined the effects of chlorhexidine (CHD) on the clinical appearance, morphology, and in vitro permeability of hamster cheek pouch mucosa. The cheek pouches were treated daily for 3 weeks with topical applications of saline, 0.2% CHD, or 2.0% CHD. Treatment with 2.0% CHD resulted in the formation of discrete white lesions in every animal in the group, whereas no changes were identified in any animal treated with 0.2% CHD or saline. Upon microscopic examination it was determined that treatment with 2.0% CHD resulted in a statistically significant increase in epithelial thickness, when compared to the other groups, and the lesions were found to consist of hyperplastic areas of epithelium with associated inflammatory cell accumulations. Daily treatments with 2.0% CHD, 0.2% CHD or saline had no effect on the very low permeability of cheek pouch mucosa to /sup 14/C-CHD. However, treatment with 2.0% CHD resulted in decreased permeability to /sup 3/H/sub 2/O when compared to the other groups. Treatment with 2.0% CHD also resulted in a thickened permeability barrier, as determined using a tracer, horseradish peroxidase. It is concluded that topical applications of 0.2% T CHD have no detectable effect on cheek-pouch mucosa while applications of 2.0% CHD result in hyperplasia and a decrease in mucosal permeability. Results suggest that CHD should be used with caution clinically and at a concentration of 0.2% or less.

  7. Pouched Rats' Detection of Tuberculosis in Human Sputum: Comparison to Culturing and Polymerase Chain Reaction

    PubMed Central

    Mahoney, Amanda; Weetjens, Bart J.; Cox, Christophe; Beyene, Negussie; Reither, Klaus; Makingi, George; Jubitana, Maureen; Kazwala, Rudovick; Mfinanga, Godfrey S.; Kahwa, Amos; Durgin, Amy; Poling, Alan

    2012-01-01

    Setting. Tanzania. Objective. To compare microscopy as conducted in direct observation of treatment, short course centers to pouched rats as detectors of Mycobacterium tuberculosis. Design. Ten pouched rats were trained to detect tuberculosis in sputum using operant conditioning techniques. The rats evaluated 910 samples previously evaluated by smear microscopy. All samples were also evaluated through culturing and multiplex polymerase chain reaction was performed on culture growths to classify the bacteria. Results. The patientwise sensitivity of microscopy was 58.0%, and the patient-wise specificity was 97.3%. Used as a group of 10 with a cutoff (defined as the number of rat indications to classify a sample as positive for Mycobacterium tuberculosis) of 1, the rats increased new case detection by 46.8% relative to microscopy alone. The average samplewise sensitivity of the individual rats was 68.4% (range 61.1–73.8%), and the mean specificity was 87.3% (range 84.7–90.3%). Conclusion. These results suggest that pouched rats are a valuable adjunct to, and may be a viable substitute for, sputum smear microscopy as a tuberculosis diagnostic in resource-poor countries. PMID:22848808

  8. Comparative rice seed toxicity tests using filter paper, growth pouch-tm, and seed tray methods.

    PubMed

    Wang, W

    1993-02-01

    Paper substrate, especially circular filter paper placed inside a Petri dish, has long been used for the plant seed toxicity test (PSTT). Although this method is simple and inexpensive, recent evidence indicates that it gives results that are significantly different from those obtained using a method that does not involve paper, especially when testing metal cations. The study compared PSTT using three methods: filter paper, Growth Pouch-TM, and seed tray. The Growth Pouch-TM is a commercially available device. The seed tray is a newly designed plastic receptacle placed inside a Petri dish. The results of the Growth Pouch-TM method showed no toxic effects on rice for Ag up to 40 mg L(-1) and Cd up to 20 mg L(-1). Using the seed tray method, IC50 (50% inhibitory effect concentration) values were 0.55 and 1.4 mg L(-1) for Ag and Cd, respectively. Although results of filter paper and seed tray methods were nearly identical for NaF, Cr(VI), and phenol, the toxicities of cations Ag and Cd were reduced by using the filter paper method; IC50 values were 22 and 18 mg L(-1), respectively. The results clearly indicate that paper substrate is not advisable for PSTT. PMID:24227383

  9. Comparative rice seed toxicity tests using filter paper, growth pouch-tm, and seed tray methods

    USGS Publications Warehouse

    Wang, W.

    1993-01-01

    Paper substrate, especially circular filter paper placed inside a Petri dish, has long been used for the plant seed toxicity test (PSTT). Although this method is simple and inexpensive, recent evidence indicates that it gives results that are significantly different from those obtained using a method that does not involve paper, especially when testing metal cations. The study compared PSTT using three methods: filter paper, Growth Pouch-TM, and seed tray. The Growth Pouch-TM is a commercially available device. The seed tray is a newly designed plastic receptacle placed inside a Petri dish. The results of the Growth Pouch-TM method showed no toxic effects on rice for Ag up to 40 mg L-1 and Cd up to 20 mg L-1. Using the seed tray method, IC50 (50% inhibitory effect concentration) values were 0.55 and 1.4 mg L-1 for Ag and Cd, respectively. Although results of filter paper and seed tray methods were nearly identical for NaF, Cr(VI), and phenol, the toxicities of cations Ag and Cd were reduced by using the filter paper method; IC50 values were 22 and 18 mg L-1, respectively. The results clearly indicate that paper substrate is not advisable for PSTT.

  10. Congenital hemifacial hyperplasia.

    PubMed

    Deshingkar, S A; Barpande, S R; Bhavthankar, J D

    2011-07-01

    Congenital hemifacial hyperplasia (CHH) is a rare congenital malformation characterized by marked unilateral overdevelopment of hard and soft tissues of the face. Asymmetry in CHH is usually evident at birth and accentuated with age, especially at puberty. The affected side grows at a rate proportional to the nonaffected side so that the disproportion is maintained thr oughout the life. Multisystem involvement has resulted in etiological heterogeneity including heredity, chromosomal abnormalities, atypical forms of twinning, altered intrauterine environment, and endocrine dysfunctions; however, no single theory explains the etiology adequately. Deformities of all tissues of face, including teeth and their related tissues in the jaw, are key findings for correct diagnosis of CHH. Here an attempt has been made to present a case of CHH with its archetypal features and to supplement existing clinical knowledge. PMID:22090778

  11. Congenital hemophagocytic reticulosis.

    PubMed

    Koto, A; Morecki, R; Santorineou, M

    1976-04-01

    A fatal case of an apparently congenital form of hemophagocytic reticulosis is reported. The onset was manifested by hyperbilirubinemia and hepatosplenomegaly which were present at birth and persisted throughout life. Fever, anemia and pancytopenia developed at 1 month of age and became progressively worse. A splenectomy was performed at the age of 3 months, but the child died one day later with disseminated intravascular coagulation and pulmonary hemorrhage. The literature is reviewed with regard to the relationship of this case to (familial) hemophagocytic reticulosis and malignant histiocytosis (histiocytic medullary reticulosis). It is suggested that congenital hemophagocytic reticulosis, as described here, (familial) hemophagocytic reticulosis in infants, and malignant histiocytosis in adults all represent the same basic disorder with different ages of onset and clinicopathologic manifestations. PMID:1266810

  12. Update on congenital glaucoma

    PubMed Central

    Mandal, Anil K; Chakrabarti, Debasis

    2011-01-01

    Congenital glaucoma is a global problem and poses a diagnostic and therapeutic challenge to the ophthalmologist. A detailed evaluation under general anesthesia is advisable to establish the diagnosis and plan for management. Medical therapy has a limited role and surgery remains the primary therapeutic modality. While goniotomy or trabeculotomy ab externo is valuable in the management of congenital glaucoma, primary combined trabeculotomy–trabeculectomy offers the best hope of success in advanced cases. Trabeculectomy with antifibrotic agent and glaucoma drainage devices has a role in the management of refractory cases, and cyclodestructive procedures should be reserved for patients where these procedures have failed. Early diagnosis, prompt therapeutic intervention and proper refractive correction are keys to success. Management of residual vision and visual rehabilitation should be an integral part of the management of children with low vision and lifelong follow-up is a must. PMID:21150027

  13. Congenital scoliosis - Quo vadis?

    PubMed

    Debnath, Ujjwal K; Goel, Vivek; Harshavardhana, Nanjanduppa; Webb, John K

    2010-04-01

    Congenital spinal vertebral anomalies can present as scoliosis or kyphosis or both. The worldwide prevalence of the vertebral anomalies is 0.5-1 per 1000 live births. Vertebral anomalies can range from hemi vertebrae (HV) which may be single or multiple, vertebral bar with or without HV, block vertebrae, wedge shaped or butterfly vertebrae. Seventy per cent of congenital vertebral anomalies result in progressive deformities. The risk factors for progression include: type of defect, site of defect (junctional regions) and patient's age at the time of diagnosis. The key to success in managing these spinal deformities is early diagnosis and anticipation of progression. One must intervene surgically to halt the progression of deformity and prevent further complications associated with progressive deformity. Planning for surgery includes a preoperative MRI scan to rule out spinal anomalies such as diastematomyelia. The goals of surgical treatment for congenital spinal deformity are to achieve a straight growing spine, a normal standing sagittal profile, and a short fusion segment. The options of surgery include in situ fusion, convex hemi epiphysiodesis and hemi vertebra excision. These basic surgical procedures can be combined with curve correction, instrumentation and short segment fusion. Most surgeons prefer posterior (only) surgery for uncomplicated HV excision and short segment fusion. These surgical procedures can be performed through posterior, anterior or combined approaches. The advocates of combined approaches suggest greater deformity correction possibilities with reduced incidence of pseudoarthrosis and minimize crankshaft phenomenon. We recommend posterior surgery for curves involving only an element of kyphosis or modest deformity, whereas combined anterior and posterior approach is indicated for large or lordotic deformities. In the last decade, the use of growing rods and vertebral expandable prosthetic titanium rib has improved the armamentarium of the

  14. Congenital midline cervical cleft.

    PubMed

    Agag, Richard; Sacks, Justin; Silver, Lester

    2007-01-01

    Congenital midline cervical cleft (CMCC) is a rare disorder of the ventral neck that is clinically evident at birth and must be differentiated from the more common thyroglossal duct cyst. The case of CMCC presented here was associated with chromosomes 13/14 de novo Robertsonian translocations as well as midline deformities including a sacral tuft and a minor tongue-tie. The case is presented as well as discussion of histopathology, embryology, and surgical treatment. PMID:17214531

  15. Other congenital abnormalities.

    PubMed

    Cobbett, J R

    1974-06-29

    The plastic surgeon is not a miracle worker, as so many of his patients believe. Nevertheless, he can do much to minimize the functional and cosmetic effect of many congenital deformities. If a moral can be drawn from this article it must be that the plastic surgeon should be given an early opportunity to see and assess the patients described here, if only to ease the anxiety in the minds of their parents by appropriate reassurance and discussion. PMID:4853507

  16. Congenital Cataract Screening.

    PubMed

    Rajavi, Zhale; Sabbaghi, Hamideh

    2016-01-01

    Congenital cataract is a leading cause of visual deprivation which can damage the developing visual system of a child; therefore early diagnosis, management and long-term follow-up are essential. It is recommended that all neonates be screened by red reflex examination at birth and suspected cases be referred to ophthalmic centers. Early surgery (<6 weeks of age, based on general neonatal health) is important for achieving the best visual outcome particularly in unilateral cases. In bilateral cases, surgery is highly recommended before appearance of strabismus or nystagmus (<10 weeks of age) with no longer than a one-week interval between the fellow eyes. Parents should be informed that surgery is a starting point and not the endpoint of treatment. Appropriate postoperative management including immediate optical correction in the form of aphakic glasses or contact lenses, or intraocular lens (IOL) implantation at the appropriate age (>1 year) is highly recommended. After surgery, amblyopia treatment and periodic follow-up examinations should be started as soon as possible to achieve a satisfactory visual outcome. Practitioners should consider the possibility of posterior capsular opacity, elevated intraocular pressure and amblyopia during follow-up, especially in eyes with microphthalmia and/or associated congenital anomalies. All strabismic children should undergo slit lamp examination prior to strabismus surgery to rule out congenital lens opacities. From a social point of view, equal and fair medical care should be provided to all children regardless of gender. PMID:27621790

  17. Congenital Cataract Screening

    PubMed Central

    Rajavi, Zhale; Sabbaghi, Hamideh

    2016-01-01

    Congenital cataract is a leading cause of visual deprivation which can damage the developing visual system of a child; therefore early diagnosis, management and long-term follow-up are essential. It is recommended that all neonates be screened by red reflex examination at birth and suspected cases be referred to ophthalmic centers. Early surgery (<6 weeks of age, based on general neonatal health) is important for achieving the best visual outcome particularly in unilateral cases. In bilateral cases, surgery is highly recommended before appearance of strabismus or nystagmus (<10 weeks of age) with no longer than a one-week interval between the fellow eyes. Parents should be informed that surgery is a starting point and not the endpoint of treatment. Appropriate postoperative management including immediate optical correction in the form of aphakic glasses or contact lenses, or intraocular lens (IOL) implantation at the appropriate age (>1 year) is highly recommended. After surgery, amblyopia treatment and periodic follow-up examinations should be started as soon as possible to achieve a satisfactory visual outcome. Practitioners should consider the possibility of posterior capsular opacity, elevated intraocular pressure and amblyopia during follow-up, especially in eyes with microphthalmia and/or associated congenital anomalies. All strabismic children should undergo slit lamp examination prior to strabismus surgery to rule out congenital lens opacities. From a social point of view, equal and fair medical care should be provided to all children regardless of gender.

  18. Liver Colonization Competence Governs Colon Cancer Metastasis

    NASA Astrophysics Data System (ADS)

    Kuo, Tsong-Hong; Kubota, Tetsuro; Watanabe, Masahiko; Furukawa, Toshiharu; Teramoto, Tatuso; Ishibiki, Kyuya; Kitajima, Masaki; Rahim Moosa, A.; Penman, Sheldon; Hoffman, Robert M.

    1995-12-01

    Tumors that metastasize do so to preferred target organs. To explain this apparent specificity, Paget, >100 years ago, formulated his seed and soil hypothesis; i.e., the cells from a given tumor would "seed" only favorable "soil" offered by certain organs. The hypothesis implies that cancer cells must find a suitable "soil" in a target organ-i.e., one that supports colonization-for metastasis to occur. We demonstrate in this report that ability of human colon cancer cells to colonize liver tissue governs whether a particular colon cancer is metastatic. In the model used in this study, human colon tumors are transplanted into the nude mouse colon as intact tissue blocks by surgical orthotopic implantation. These implanted tumors closely simulate the metastatic behavior of the original human patient tumor and are clearly metastatic or nonmetastatic to the liver. Both classes of tumors were equally invasive locally into tissues and blood vessels. However, the cells from each class of tumor behave very differently when directly injected into nude mouse livers. Only cells from metastasizing tumors are competent to colonize after direct intrahepatic injection. Also, tissue blocks from metastatic tumors affixed directly to the liver resulted in colonization, whereas no colonization resulted from nonmetastatic tumor tissue blocks even though some growth occurred within the tissue block itself. Thus, local invasion (injection) and even adhesion to the metastatic target organ (blocks) are not sufficient for metastasis. The results suggest that the ability to colonize the liver is the governing step in the metastasis of human colon cancer.

  19. Radiology of congenital heart disease

    SciTech Connect

    Amplatz, K.

    1986-01-01

    This is a text on the radiologic diagnosis of congenital heart disease and its clinical manifestations. The main thrust of the book is the logical approach which allows an understanding of the complex theory of congenital heart disease. The atlas gives a concise overview of the entire field of congenital heart disease. Emphasis is placed on the understanding of the pathophysiology and its clinical and radiological consequences. Surgical treatment is included since it provides a different viewpoint of the anatomy.

  20. Colon diverticula - slideshow

    MedlinePlus

    ... this page: //medlineplus.gov/ency/presentations/100158.htm Colon diverticula - series To use the sharing features on ... 6 out of 6 Normal anatomy Overview The colon, or large intestine, is a muscular tube that ...

  1. Colon cancer - slideshow

    MedlinePlus

    ... this page: //medlineplus.gov/ency/presentations/100157.htm Colon cancer - Series To use the sharing features on ... 5 out of 5 Normal anatomy Overview The colon, or large intestine, is a muscular tube that ...

  2. Genetics Home Reference: congenital hepatic fibrosis

    MedlinePlus

    ... Home Health Conditions congenital hepatic fibrosis congenital hepatic fibrosis Enable Javascript to view the expand/collapse boxes. ... PDF Open All Close All Description Congenital hepatic fibrosis is a disease of the liver that is ...

  3. Genetics Home Reference: Leber congenital amaurosis

    MedlinePlus

    ... Registry: Leber congenital amaurosis 9 National Eye Institute: Gene Therapy for Leber Congenital Amaurosis These resources from MedlinePlus ... Additional NIH Resources (1 link) National Eye Institute: Gene Therapy for Leber Congenital Amaurosis Educational Resources (3 links) ...

  4. Colon cancer - Series (image)

    MedlinePlus

    Colon cancer is the third most common cancer in the United States. Risk factors include a diet low ... The treatment of colon cancer depends on the stage of the disease. Stage I cancer is limited to the inner lining of the colon; ...

  5. A quantitative study of the morphological development and bacterial colonisation of the gut of the tammar wallaby Macropus eugenii eugenii and brushtail possum Trichosurus vulpecula during in-pouch development.

    PubMed

    Lentle, R G; Dey, D; Hulls, C; Mellor, D J; Moughan, P J; Stafford, K J; Nicholas, K

    2006-11-01

    We compared the rates of change of various morphological parameters of the stomach, small intestine, caecum and colon of tammar wallabies and brushtail possums with body mass during in-pouch development. These were correlated with changes in the numbers of bacterial species in the various gut segments. In the pouch-young of both species, the wet tissue masses of all gut segments increased with body mass in a positively allometric manner (i.e. with a body mass exponent > 1), suggesting that the mass of each component was disproportionately low at birth, but increased disproportionately rapidly postnatally. However, the lengths of the wallaby stomach and small intestine scaled isometrically with respect to body mass (i.e. with a body mass exponent around 0.33), which may indicate that the shape of these components changes to the adult form during early neonatal development. Conversely, the length of the caecum and colon of both wallabies and possums scaled in a positively allometric manner with respect to body mass, showing area to volume compensation. This may indicate a more general pattern of disproportionately rapid postnatal enlargement in areas that are distal to the principal sites of neonatal digestion (i.e. the stomach). The numbers of bacterial species present in the various gastrointestinal segments of both species were low in animals aged 100 days or less but there was a significant increase in microbial diversity in the caecum of brushtail possums aged over 100 days. The possum caecum also showed the greatest rate of increase in wet tissue mass relative to body mass. It is postulated that caecal development may act as a nidus for establishment of communities of commensal microflora in the developing marsupial. PMID:16819652

  6. Black tea polyphenols protect against 7,12-dimethylbenz[a]anthracene-induced hamster buccal pouch carcinogenesis.

    PubMed

    Letchoumy, P Vidjaya; Chandra Mohan, K V P; Kumaraguruparan, R; Hara, Y; Nagini, S

    2006-01-01

    Dietary chemoprevention has emerged as a cost-effective approach for cancer control. We evaluated the chemopreventive effects of black tea polyphenols (Polyphenon-B) administration during the preinitiation phase of 7,12-dimethylbenz[a]anthracene (DMBA)-induced hamster buccal pouch (HBP) carcinogenesis. The expression of proliferating cell nuclear antigen (PCNA) in the buccal pouch and the concentration of lipid peroxides, protein carbonyl, and the antioxidant status in the buccal pouch, liver and erythrocytes were used as biomarkers of chemoprevention. All the hamsters painted with DMBA alone for 14 weeks developed buccal pouch carcinomas associated with increased expression of PCNA, diminished lipid and protein oxidation, and enhanced antioxidant status. In the liver and erythrocytes of tumor-bearing animals, enhanced oxidation of lipids and proteins was accompanied by compromised antioxidant defenses. Dietary administration of Polyphenon-B effectively suppressed DMBA-induced HBP carcinogenesis as revealed by decreased incidence of tumours and PCNA expression. In addition, Polyphenon-B modulated lipid and protein oxidation and enhanced the antioxidant status in the pouch, liver, and erythrocytes. We suggest that Polyphenon-B exerts its chemopreventive effects by inhibiting cell proliferation in the target tissue and modulating the oxidant-antioxidant status in the target as well as in host tissues. PMID:17120615

  7. Molecular and Genetic Studies of Congenital Myopathies

    ClinicalTrials.gov

    2015-10-26

    Central Core Disease; Centronuclear Myopathy; Congenital Fiber Type Disproportion; Multiminicore Disease; Myotubular Myopathy; Nemaline Myopathy; Rigid Spine Muscular Dystrophy; Undefined Congenital Myopathy

  8. Currarino triad associated with malrotation of the colon.

    PubMed

    Daoud, Faiez S; Aburub, Mohammad A; Hadidy, Azmy M

    2007-02-01

    Currarino triad is a rare congenital condition characterized by a sacral bony defect, presacral mass, and anorectal malformations. We describe an unusual case of complete Currarino triad in a 22-year-old female with sacral bony defect, anal stenosis, recto-vaginal fistula, and dual pathology meningocele and teratoma in the pre sacral mass associated with malrotation of the colon. This combination has not been reported previously. The clinical presentation, surgical management and review of literature are discussed. PMID:17268712

  9. Congenital limb deficiency disorders.

    PubMed

    Wilcox, William R; Coulter, Colleen P; Schmitz, Michael L

    2015-06-01

    Congenital limb deficiency disorders (LDDs) are birth defects characterized by the aplasia or hypoplasia of bones of the limbs. Limb deficiencies are classified as transverse, those due to intrauterine disruptions of previously normal limbs, or longitudinal, those that are isolated or associated with certain syndromes as well as chromosomal anomalies. Consultation with a medical geneticist is advisable. Long-term care should occur in a specialized limb deficiency center with expertise in orthopedics, prosthetics, and occupational and physical therapy and provide emotional support and contact with other families. With appropriate care, most children with LDDs can lead productive lives. PMID:26042905

  10. [Congenital myasthenic syndrome].

    PubMed

    Araga, Shigeru

    2008-06-01

    Congenital myasthenic syndromes (CMS) are rare heterogeneous disorders in which neuromuscular transmission is compromised by one or more specific mechanisms. CMS are clinically diagnosed by a history of fatigability and muscle weakness since infancy or early childhood, a decremental EMG response and the absence of acetylcholine receptor antibodies. CMS form a heterogeneous group of disorders which are classified as originating from presynaptic, synaptic or postsynaptic defects. Molecular genetic studies reveal a various type of mutations in synapse-associated genes. However, the genetic abnormalities of many CMS are still unresolved. This article outlines the classification of CMS and etiology of individual forms. PMID:18540366

  11. Precalcaneal Congenital Fibrolipomatous Hamartoma

    PubMed Central

    Yang, Ji-Hye; Park, Oun-Jae; Kim, Jeong-Eun; Won, Chong-Hyun; Chang, Sung-Eun; Choi, Jee-Ho; Moon, Kee-Chan

    2011-01-01

    Precalcaneal congenital fibrolipomatous hamartomas (PCFHs) are characterized clinically by the presence of unilateral or bilateral, asymptomatic nodules in the medial precalcaneal plantar region of the heel. They are skin colored and usually painless nodules. In most patients, the lesions appear within the first few months of life, but they may also be present at birth. Generally PCFHs are benign, but they can grow in proportion to the growth of the infants. Here, we report the case of a 4-month-old boy with a solitary, localized skin-colored nodule on the precalcaneal plantar region of his right heel, diagnosed as a PCFH. PMID:21738373

  12. Nonclassic Congenital Adrenal Hyperplasia

    PubMed Central

    Witchel, Selma Feldman; Azziz, Ricardo

    2010-01-01

    Nonclassic congenital adrenal hyperplasia (NCAH) due to P450c21 (21-hydroxylase deficiency) is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and “on-time” puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH. PMID:20671993

  13. Congenital nephrotic syndrome.

    PubMed

    Begolli, Mirije; Begolli, Ilir; Gojani, Xhenane; Arenliu-Qosaj, Fatime; Berisha, Merita

    2011-01-01

    The aim of this case is to present a case of a two month old female with congenital nephritic syndrome, which is very rare. On admission, the baby showed marked edema and distended abdomen. She was diagnosed and treated with daily albumin infusions, antibiotics, diuretic, gamma globulin replacement, ACEI and NSAIDs. Parents were informed about the nature of the disease, prognosis, and advised for further medical care in a more advanced kidney transplantation centre. This was the first treatment of this condition in the Pediatric Clinic in Kosovo and it presented a challenge for us. PMID:22299306

  14. CONGENITAL DIAPHRAGMATIC HERNIA

    PubMed Central

    Adams, Burton E.

    1954-01-01

    Treatment of congenital diaphragmatic hernia in infants is a matter of semi-emergency and should be done as soon as adequate preparations can be made because sometimes fatal complications develop swiftly. In preoperative preparation there is great advantage in thorough decompression of the abdominal viscera, stomach, bowel and bladder. As to operation, the author believes the abdominal approach has most to recommend it. In the postoperative period, continued gastric suction for a brief time, parenteral administration of fluids and use of a Mistogen tent with a high moist oxygen content will facilitate rapid recovery. ImagesFigure 1. PMID:13209363

  15. Congenital protein hypoglycosylation diseases

    PubMed Central

    Sparks, Susan E

    2012-01-01

    Glycosylation is an essential process by which sugars are attached to proteins and lipids. Complete lack of glycosylation is not compatible with life. Because of the widespread function of glycosylation, inherited disorders of glycosylation are multisystemic. Since the identification of the first defect on N-linked glycosylation in the 1980s, there are over 40 different congenital protein hypoglycosylation diseases. This review will include defects of N-linked glycosylation, O-linked glycosylation and disorders of combined N- and O-linked glycosylation. PMID:23776380

  16. Congenital anterior urethral diverticulum.

    PubMed

    Singh, Sanjeet Kumar; Ansari, Ms

    2014-09-01

    Congenital anterior urethral diverticulum (CAUD) may be found all along the anterior urethra and may present itself at any age, from infant to adult. Most children with this condition present with difficulty in initiating micturition, dribbling of urine, poor urinary stream, or urinary tract infection. A careful history will reveal that these children never had a good urinary stream since birth, and the telltale sign is a cystic swelling of the penile urethra. In this paper, we present two cases of CAUD that were managed by excision of the diverticulum with primary repair. PMID:26328174

  17. Primary congenital bladder diverticula: Where does the ureter drain?

    PubMed Central

    Macedo, Antonio; Garrone, Gilmar; Ottoni, Sérgio Leite; Oliveira, Diego Estevam; do Rosário Souza, Geórgia Rubiane Meira; da Cruz, Marcela Leal

    2015-01-01

    Background: Primary congenital bladder diverticulum (PCBD) is related to a deficient detrusor layer allowing out-pouching of the bladder mucosa through the inadequate muscularis wall. We aimed to review our experience with symptomatic PCBD in order to correlate clinical findings with anatomical aspects and to present late outcome. Materials and Methods: We reviewed all patients operated in our institution since 2004. We evaluated the charts for complaints, radiological exams, method of treatment, complications and length of follow-up. Results: We treated 10 cases (11 renal units - [RU]), predominantly males (9/10), mean age at surgery of 5.3 years. All patients had significant urological complaints presenting either with antenatal hydronephrosis (4) or febrile urinary tract infection (5) and urinary retention in one. The ureter was found implanted inside the diverticulum in 8/11 RU. An extravesical psoas-hitch ureteroneocystostomy and diverticulum resection was performed in 10/11 cases, whereas 1 case was treated intravesically based on surgeon's preference without performing cystoscopy. Mean follow-up was 34.1 months (1-120) without complications. Conclusions: PCBD is an uncommon diagnosis and has a high probability of drainage inside the diverticulum (72.7%). We recommend the extravesical approach associated with diverticulectomy and ureteroneocystostomy as the preferred technique to treat this abnormality. PMID:26712296

  18. Association between gastro-intestinal symptoms and menstruation in patients with ileal pouches

    PubMed Central

    Bharadwaj, Shishira; Wu, Xian-rui; Barber, Matthew D.; Queener, Elaine; Graff, Lesley; Shen, Bo

    2014-01-01

    Background and aims: Gastro-intestinal (GI) symptoms are often experienced by healthy women during menstruation. An increased frequency of GI symptoms during menses has also been reported in women with irritable bowel syndrome or inflammatory bowel disease (IBD); however, IBD patients with restorative proctocolectomy and ileal pouch-anal anastomoses (IPAA) have not been studied. We aimed to examine the association between GI symptoms before and during menses in patients with IPAA, and to assess factors for exacerbation of GI symptoms in those patients. Methods: Adult women recorded in the Pouchitis Registry were invited to participate in a mailed survey. Participants reported on GI symptoms 1–5 days prior to- (pre-menses) and during the days of their menses in recent months. Demographic and clinical variables were obtained through the survey and chart review. Results: One hundred and twenty-eight (21.3%) out of 600 women with IPAA responded to the survey questionnaire. Forty-three (33.5%) were excluded for reasons including post-menopausal (n = 25), hysterectomy (n = 14) and use of contraceptives (n = 4). Abdominal pain (P = 0.001), diarrhea (P = 0.021), and urgency (P = 0.031) were more commonly reported during menses than pre-menses by the participants. Only a history of painful menses was significantly associated with increased GI symptoms during menses for patients with ileal pouch (odds ratio = 5.67; 95% confidence interval: 1.41–22.88; P = 0.015). Conclusion: GI symptoms such as abdominal pain, diarrhea, and urgency are commonly associated with menses in patients with ileo-anal pouch. Painful menses may be associated with worsening of GI symptoms. PMID:25016379

  19. Congenital Median Upper Lip Fistula

    PubMed Central

    al Aithan, Bandar

    2012-01-01

    Congenital median upper lip fistula (MULF) is an extremely rare condition resulting from abnormal fusion of embryologic structures. We present a new case of congenital medial upper lip fistula located in the midline of the philtrum of a 6 year old girl. PMID:22953305

  20. Use of the Anaerobic Pouch in Isolating Clostridium botulinum Spores from Fresh Meats

    PubMed Central

    Greenberg, Richard A.; Bladel, Bendt O.; Zingelmann, Walter J.

    1966-01-01

    The anaerobic film pouch was demonstrated to be an effective device for the primary isolation of Clostridium botulinum types A and B spores from raw pork, beef, and chicken. Optimal pasteurization of these meats (for reduction of nonspore microflora without affecting indigenous putrefactive anaerobic spore levels) was 50 min at 60 C. C. botulinum spores were recovered with good precision from meat samples inoculated with mixtures of C. botulinum and Putrefactive Anaerobe 3679 at 1:1 and at 1:99 ratios. Verification of C. botulinum isolates was accomplished by protection testing of subcultures in mice. PMID:5335387

  1. Using Giant African Pouched Rats to Detect Tuberculosis in Human Sputum Samples: 2009 Findings

    PubMed Central

    Poling, Alan; Weetjens, Bart J.; Cox, Christophe; Mgode, Georgies; Jubitana, Maureen; Kazwala, Rudovic; Mfinanga, Godfrey S.; Huis in ‘t Veld, Diana

    2010-01-01

    In 2009, giant African pouched rats trained to detect tuberculosis (TB) evaluated sputum samples from 10,523 patients whose sputum had previously been evaluated by smear microscopy. Microscopists found 13.3% of the patients to be TB-positive. Simulated second-line screening by the rats revealed 620 new TB-positive patients, increasing the case detection rate by 44%. These data suggest that the rats may be useful for TB detection in developing countries, although further research is needed. PMID:21118940

  2. Coverage of Gingival Fenestration Using Modified Pouch and Tunnel Technique: A Novel Approach

    PubMed Central

    Pendor, Sunil; Baliga, Vidya; Muthukumaraswamy, A.; Dhadse, Prasad V.; Ganji, Kiran Kumar; Thakare, Kaustubh

    2013-01-01

    Gingival fenestration defects are a rare phenomenon. Gingival fenestration means the exposure of the tooth due to loss of the overlying bone and gingiva. Though treatment of mucosal fenestration occurring in association with chronic periapical inflammation has been reported previously, the occurrence and treatment of gingival fenestration have not been documented in great detail. This report describes the occurrence of a gingival fenestration that developed secondarily to a gutka chewing habit. Treatment of the fenestration along with coverage of an adjacent recession defect in a single-step procedure using a pouch and tunnel technique is described. PMID:23936687

  3. Controversies in J Pouch Surgery for Ulcerative Colitis: A Focus on Handsewn Versus Stapled Anastomosis.

    PubMed

    Nobel, Tamar; Khaitov, Sergey; Greenstein, Alexander J

    2016-09-01

    The accepted current standard for treatment of medically refractory ulcerative colitis is total proctocolectomy with an ileal pouch-anal anastomosis for restoration of continence. There are 2 techniques by which the anastomosis can be performed, including handsewn and stapled. Handsewn anastomosis with mucosectomy was the first method described; however, it has been associated with significant incontinence. The double-stapled anastomosis was developed in response to improve postoperative function. Controversy remains as to which technique is superior as both have disadvantages. This review article addresses differences between the 2 methodologies in relation to postoperative complications, anorectal physiology, functional outcomes, and oncological safety. PMID:27542137

  4. A Case Report: A Third/Fourth Branchial Pouch Anomaly Presented by Solid Thyroid and Lateral Cervical Neck Masses.

    PubMed

    Nasreldin, Magda H A; Ibrahim, Eman A; Saad El-Din, Somaia A

    2016-01-01

    Branchial pouch-derived anomalies may arise from remnants of the first, second, or third/fourth branchial arches. Branchial pouch-related structures are found within the thyroid gland in the form of solid cell rests, epithelial lined cyst with or without an associated lymphoid component, thymic and/or parathyroid tissue, and less commonly in the form of heterotopic cartilage. We present a rare case of left solid thyroid swelling nearby two cervical nodules in a seven-year-old female with a clinical diagnosis suggestive of malignant thyroid tumor with metastasis to the cervical lymph nodes. Histopathological examination revealed that it was compatible with third/fourth branchial pouch-derived anomaly composed of mature cartilage and thymic and parathyroid tissues for clinical and radiological correlations. PMID:26819565

  5. Histology of the pouch epithelium and the mammary glands during chemically induced oestrus in the brushtail possum (Trichosurus vulpecula)

    PubMed Central

    Old, Julie M; Irving, M; Deane, Elizabeth M

    2005-01-01

    Changes in the epithelium of the maternal pouch and the mammary gland of brushtail possums (Trichosurus vulpecula) were examined after animals were treated to induce ovulation with follicle-stimulating hormone (FSH), luteinizing hormone (LH), pregnant mares’ serum gonadotrophin (PMSG) and oestradiol. The mammary glands were similar in appearance to those described in eutherian mammals and in previous studies on other marsupials. Exposure of possums to these compounds, particularly PSMG, appeared to result in changes in the mammary glands that could be associated with milk/secretion production. In contrast, the pouch epithelium had a similar histological appearance to that of epithelium from other parts of the body regardless of whether the animal was exposed to stimulants. These preliminary observations are discussed in the context of the purported role of the pouch epithelium and the mammary gland in production of secretions at oestrus and provision of immunological protection to the neonatal marsupial. PMID:16011549

  6. [Genetics of congenital lipodystrophies].

    PubMed

    Buffet, A; Lombes, M; Caron, P

    2015-10-01

    Congenital lipodystrophies are heterogeneous genetic diseases, leading to the loss of adipose tissue. This loss of adipose tissue can be generalized or partial, thus defining different phenotypes. These lipodystrophies have a major metabolic impact, secondary to lipotoxicity. This lipotoxicity is responsible for insulin resistance, dyslipidemia and hepatic steatosis. The severity of the metabolic impact correlates with the severity of the loss of adipose tissue. Mutations in 15 predisposition genes are currently described; BSCL2 and AGPT2 genes are the major genes in the generalized forms. On the contrary, LMNA and PPARG gene mutations are recovered in partial lipodystrophies forms. These different genes encode for proteins involved in adipocyte physiology, altering adipocyte differentiation, triglycerides synthesis and lysis or playing a major role in the lipid droplet formation. Congenital lipodystrophies treatment is based on the management of metabolic comorbidities but recombinant leptin therapy appears to have promising results. These different points have been recently discussed during the 2015 Endocrine Society Congress, notably by S. O'Rahilly and are highlighted in this review. PMID:26776286

  7. Congenital fiber type disproportion.

    PubMed

    Kissiedu, Juliana; Prayson, Richard A

    2016-04-01

    Type I muscle fiber atrophy in childhood can be encountered in a variety of neuromuscular disorders. Congenital fiber type disproportion (CFTD) is one such condition which presents as a nonprogressive muscle weakness. The diagnosis is often made after excluding other differential diagnostic considerations. We present a 2-year-9-month-old full term boy who presented at 2 months with an inability to turn his head to the right. Over the next couple of years, he showed signs of muscle weakness, broad based gait and a positive Gower's sign. He had normal levels of creatine kinase and normal electromyography. A biopsy of the vastus lateralis showed a marked variation in muscle fiber type. The adenosine triphosphate (ATP)-ase stains highlighted a marked type I muscle atrophy with rare scattered atrophic type II muscle fibers. No abnormalities were observed on the nicotinamide adenine dinucleotide (NADH), succinate dehydrogenase (SDH) or cytochrome oxidase stained sections. Ragged red fibers were not present on the trichrome stain. Abnormalities of glycogen or lipid deposition were not observed on the periodic acid-Schiff or Oil-Red-O stains. Immunostaining for muscular dystrophy associated proteins showed normal staining. Ultrastructural examination showed a normal arrangement of myofilaments, and a normal number and morphology for mitochondria. A diagnosis of CFTD was made after excluding other causes of type I atrophy including congenital myopathy. The lack of specific clinical and genetic disorder associated with CFTD suggests that it is a spectrum of a disease process and represents a diagnosis of exclusion. PMID:26526626

  8. [Congenital defects and incapacity].

    PubMed

    Jouve de la Barreda, Nicolás

    2009-01-01

    As a whole the congenital defects constitute an important section of the medical attention affecting near 3% of the population. A 15% of spontaneous abortions take place of which the greater frequency corresponds to the chromosome anomalies (25%) and the monogenic mutations (20%) and in a lesser extent to the effects of teratogenic agents. Between the genetic causes determining the congenital defects the mutations that affect genes acting in the early stages of development occupy a main place. These alterations can affect to homeotic genes or monogenic systems that act during the critical phases of the organogenesis. It seems evident that an alteration in the expression of a necessary gene for the appearance of a morphogenetic change constitutes the angular stone to understand resurging of a malformation or discapacity. In the last years has been demonstrated the importance of the teratogenic or environmental agents on the delicate internal physiological balance during the critical stages of the development. In this context must be included the inductive environmental factors inducing epigenetic modifications in the early stage of the development of the embryos produced by fertilization in vitro. PMID:19799481

  9. [Congenital multiple arthrogryposis].

    PubMed

    Parsch, Klaus; Pietrzak, Szymon

    2007-03-01

    From 1975 to 2004 a total of 38 children handicapped by congenital multiple arthrogryposis were cared for. The congenital joint contractures demand a major effort in terms of surgical reconstruction. In the case of distal arthrogryposis the chances that patients will be able to walk without help are good, while those with amyoplasia are likely to be dependent on mobility aids throughout their lives. The ultimate goal of treatment for patients is to develop into self-confident adults who can cope with life despite their handicaps. The hip in arthrogryposis shows variable forms of pathology, ranging from the almost normal hip to hip contractures with dislocation. Its treatment has some limited advantages, but hardly improves mobility. The knee contractures are actively treated to allow patients to sit, stand and walk better. The club foot and the rocker-bottom foot need sophisticated conservative and operative treatments. If conservative manipulation of bilateral extension contractures of the elbow fails operative treatment is carried out on the dominant side. For shoulder, hand and finger contractures conservative manipulation brings about little improvement, and surgical approaches help hardly at all. PMID:17323063

  10. Cataracts in Congenital Toxoplasmosis

    PubMed Central

    Arun, Veena; Noble, A. Gwendolyn; Latkany, Paul; Troia, Robert N.; Jalbrzikowski, Jessica; Kasza, Kristen; Karrison, Ted; Cezar, Simone; Sautter, Mari; Greenwald, Mark J.; Mieler, William; Mets, Marilyn B.; Alam, Ambereen; Boyer, Kenneth; Swisher, Charles N.; Roizen, Nancy; Rabiah, Peter; Del Monte, Monte A.; McLeod, Rima

    2008-01-01

    Purpose To determine the incidence and natural history of cataracts in children with congenital toxoplasmosis. Methods Children referred to the National Collaborative Chicago-based Congenital Toxoplasmosis Study (NCCCTS) between 1981 and 2005 were examined by ophthalmologists at predetermined times according to a specific protocol. The clinical course and treatment of patients who developed cataracts was reviewed. Results In the first year of life, 134 of 173 children examined were treated with pyrimethamine, sulfadiazine, and Leucovorin, while the remaining 39 were not treated. Cataracts occurred in 27 eyes of 20 patients (11.6%, 95% confidence interval [7.2%, 17.3%]). Fourteen cataracts were present at birth, and 13 developed postnatally. Locations of the cataracts included anterior polar (3 eyes), anterior subcapsular (6), nuclear (5), posterior subcapsular (7), and unknown (6). Thirteen cataracts were partial, 9 total, and 5 with unknown complexity. Twelve cataracts remained stable, 12 progressed, and progression was not known for 3. Five of 27 eyes had cataract surgery, with 2 of these developing glaucoma. Sixteen eyes of 11 patients had retinal detachment and cataract. All eyes with cataracts had additional ocular lesions. Conclusions In the NCCCTS cohort, 11.6% of patients were diagnosed with cataracts. There was considerable variability in the presentation, morphology, and progression of the cataracts. Associated intraocular pathology was an important cause of morbidity. PMID:18086432

  11. [Enzymopathic congenital hyperlactacidemia].

    PubMed

    Leroux, J P; Marsac, C; Saudubray, J M

    1976-01-01

    Congenital enzymopathic hyperlactacidemia results from a defect of utilisation of pyruvate either at the level of the pyruvate junction (pyruvate-carboxylase, pyruvate-dehydrogenase and Kreb's cycle), or at the level of the unidirectional enzymes on neo-glucogenesis and of neo-glycogenogenesis, e.g. glucose-6-phosphatase, phosphoenol-pyruvate-carboxykinase and glycogen synthetase. The enzymopathies which affect neoglucogenesis associate hyper-lactacidemia and fasting hypoglycemia and more or less marked hepatomegaly. Type I glycogenesis (von Gierke's disease) is the best known example. Enzymopathies which affect the pyruvate junction and the Krebs cycle, may be manifested in addition by: --either chronic neuropathies, e.g. Leigh's disease, recurrent ataxia, and moderate hyperalactacidemia,--or, as in congenital lactic acidoses, which have a rapid and severe prognosis with major hyperlactacidemia. Functional investigation, in particular, loading tests are of great value in orientation and justify the practice of tissue biopsy which permits the enzyme diagnosis. Recent, still unconfirmed knowledge of the pathogenesis of these diseases emphasizes the considerable importance of estimation of blood lactic acid in the investigation of metabolic acidoses of hereditary origin. PMID:184725

  12. An unusual colon atresia in a calf: at the junction of the distal loop and transverse colon. A brief overview

    PubMed Central

    Lombardero, Matilde; Yllera, María del Mar

    2014-01-01

    Congenital defects are those abnormalities present at birth. During embryogenesis, many anomalies can occur. The primitive gut tube lengthens quickly and rotates, allowing the gastrointestinal tract acquire its final position and orientation. Because the colon of large animals is complex, most changes occur in this segment. Thus, in ruminants, colon atresia is the most frequent malformation, affecting mainly ascending colon, at the level of the spiral loop. There are no previous references about a very atypical colon atresia at the junction of distal loop and transverse colon, such we have described in a 5-day-old calf, after a history of abdominal distention and absence of feces at birth, even with a patent anal opening. Atresia coli was detected at distal position of the typical colon atresia, at the junction of distal loop and transverse colon. In addition, the distal blind end was bent into a U-shape supported by the mesocolon. Besides the anatomical findings of this worthwhile atresia coli we discuss its possible etiology, in which local factors, such as a compromised blood supply during embryogenesis, are more consistent than genetic factors. Finding out the causes of atresia coli would help to reduce its incidence, lessen animal suffering and economic loss. PMID:25495264

  13. Endoscopy-assisted transanal repair of afferent limb obstruction and long efferent limb with ileopexy after ileal J-pouch-anal anastomosis.

    PubMed

    Araki, Toshimitsu; Okita, Yoshiki; Kawamura, Mikio; Kondo, Satoru; Kobayashi, Minako; Ohi, Masaki; Toiyama, Yuji; Tanaka, Koji; Inoue, Yasuhiro; Uchida, Keiichi; Mohri, Yasuhiko; Kusunoki, Masato

    2016-05-01

    Afferent limb obstruction can be a persistent complication after restorative proctocolectomy with ileal pouch-anal anastomosis. We present a case of afferent limb obstruction complicated by a long efferent limb of the ileal pouch that we successfully treated with side-to-side anastomosis of the afferent and efferent limbs. The procedure involved using a transanal endoscopic stapling device assisted by transanal endoscopy with a thin intestinal video endoscope. This allowed reliable, safe visualization of the lesion from the tight pouch-anal anastomosis and facilitated repair with an endoscopic stapling device. Because the technique was performed without enterotomy, it reduced the risks of contamination and leakage from transabdominal small bowel anastomosis. Laparotomy view also prevented of injury to the pouch itself and entrapment of the mesentery of the afferent and efferent limbs of the pouch between the stapler anvils. PMID:27117971

  14. Accurate Dosing of Antiretrovirals at Home Using a Foilized, Polyethylene Pouch to Prevent the Transmission of HIV From Mother to Child

    PubMed Central

    Choy, Alexa; Ortiz, Mercedes; Malkin, Robert

    2015-01-01

    Abstract Mother-to-child HIV transmission rates remain elevated in countries with high home birth rates. This risk can be dramatically reduced if infants receive antiretroviral (ARV) medication within 24 hours after birth. However, many barriers prevent access to these medications immediately after delivery, for example, there is currently no suitable mechanism to preserve predosed ARVs in the home during the months before birth. In response to this, students of the Duke University developed the Pratt pouch, a foilized polyethylene packet designed to preserve predosed ARVs. This cross-sectional study presents the data from the first clinical trials of the Pratt pouch in Guayaquil, Ecuador. Fourteen HIV-positive mothers and nurses were observed using the pouch to deliver a dose of ARVs to an infant. Weight measurements, time, and notes on spillage were taken at each observation period. Successful usage was quantitatively assessed through the calculation of dosing accuracy based on the volume of liquid medication emptied from the pouch. Additionally, mothers were surveyed after a month of using the device at home to assess their perception of the accuracy, acceptability, and ease of use of the pouch. Used pouches were collected for physical analysis of tearing. Observed users delivered accurate doses (M = 101.1%, standard deviation = 8.2%) in an average time of 2.6 minutes. A total of 2869 used pouches were recovered. No seal failures or failed attempts at opening/delivering the pouches were observed or detected. Forty-three mothers were surveyed. All mothers (100%) reported that they were able to follow their physician's treatment plan, all pouches were received in good condition and the pictorial sheets provided clear instructions. We conclude that the Pratt pouch is a highly accurate and easy-to-use device for delivering liquid oral ARVs to infants and is appropriate for prepackaging ARVs for home use. PMID:26107673

  15. Sources and severity of self-reported food intolerance after ileal pouch-anal anastomosis.

    PubMed

    Steenhagen, Elles; de Roos, Nicole M; Bouwman, Carolien A; van Laarhoven, Cees J H M; van Staveren, Wija A

    2006-09-01

    Data on food intolerance after ileal pouch-anal anastomosis are scarce. The aim of this study was to identify foods causing intolerance and to determine the nature and severity of reported symptoms. Patients from the Dutch Crohn's and Ulcerative Colitis Association were mailed a survey on food intolerance; 105 (31% men) of 137 patients took part. They all reported intolerance to one or more foods. Common symptoms (scored from 0=absent to 10=severe), included diarrhea (mean score=5.8), fatigue (mean score=5.5), and thirst (mean score=4.6). Spicy foods, cabbage, and citrus fruits (or juice) were most likely to decrease stool consistency, increase stool frequency, or cause perianal irritation. Onions, cabbage, or leeks were reported by 28% of the patients to cause flatulence. The urge to defecate was stronger after a cooked meal (45% within (1/2) hour) than after sandwiches (15% within (1/2) hour). Foods reported to increase stool consistency were potato products, bread, and bananas. This study demonstrates that food intolerance is a common, albeit mild, problem after ileal pouch-anal anastomosis. Food and nutrition professionals should encourage patients to base their food choices on individual tolerance as long as no (patho-) physiological-based evidence to the contrary is available. PMID:16963353

  16. Thermal behavior and electrochemical heat generation in a commercial 40 Ah lithium ion pouch cell

    NASA Astrophysics Data System (ADS)

    Schuster, Elke; Ziebert, Carlos; Melcher, Andreas; Rohde, Magnus; Seifert, Hans Jürgen

    2015-07-01

    Quantitative data on the thermal behavior of lithium ion batteries under charging and discharging conditions are essential for designing thermal management systems and improving battery safety. In this work, commercial 40 Ah lithium ion pouch cells with Li(Ni1/3Mn1/3Co1/3)O2 cathodes were tested under isoperibolic and adiabatic conditions in an Accelerating Rate Calorimeter at different charging/discharging currents from 5 A to 40 A. Adiabatic tests simulate the worst-case scenario of a battery pack without cooling. For charging and discharging an overall exothermic behavior was found and a total temperature increase for one half cycle between 3 and 11 K. Isoperibolic tests simulate a single cell under constant environmental temperature. Here an exothermic behavior for discharging and an endothermic behavior for charging were observed. To transfer the measured temperature changes into heat data, the effective specific heat capacity and the heat transfer coefficient were determined. For the first time the heat generation data for a large format pouch cell have been determined using both isoperibolic and adiabatic conditions. These data were compared with the total heat data calculated as the sum of reversible and irreversible heat that were measured by potentiometric and current interruption techniques respectively. A good agreement was found between all three heat generation determination methods.

  17. Altered cytokeratin expression during chemoprevention of hamster buccal pouch carcinogenesis by S-allylcysteine.

    PubMed

    Balasenthil, Seetharaman; Rao, Kunchala S; Nagini, Siddavaram

    2003-01-01

    We examined the effect of S-allylcysteine (SAC), a water-soluble garlic constituent, on cytokeratin expression, a sensitive and specific marker for differentiation status during 7,12-dimethylbenz[a]anthracene (DMBA)-induced hamster buccal pouch (HBP) carcinogenesis in male Syrian hamsters. Hamsters were divided into four groups of six animals each. Animals in group 1 were painted with a 0.5% solution of DMBA in liquid paraffin on the right buccal pouches three times a week for 14 weeks. Group 2 animals were painted with DMBA as in group I, and in addition they received orally 200 mg/kg of SAC on days alternate to DMBA application. Group 3 animals received SAC as in group 2. Group 4 animals received neither DMBA nor SAC and served as the control. The hamsters were killed after an experimental period of 14 weeks. Cytokeratin expression was detected by Western blot analysis using monoclonal antibodies AE1 and AE3. In DMBA-induced HBP tumors, the decreased expression of high molecular weight cytokeratins of molecular mass between 55-70 kDa was observed. Administration of SAC (200 mg/kg) to animals painted with DMBA suppressed the incidence of DMBA-induced carcinomas and was associated with restoration of normal cytokeratin expression. The results of the present study suggest that inhibition of HBP tumorigenesis by SAC may be due to its regulatory effects on differentiation, tumor invasiveness, and its ability to migrate and form metastases. PMID:14704476

  18. Congenital Chloride Diarrhea - Novel Mutation in SLC26A3 Gene.

    PubMed

    Bhardwaj, Swati; Pandit, Deepti; Sinha, Aditi; Hari, Pankaj; Cheong, Hae Il; Bagga, Arvind

    2016-08-01

    The authors report a case of congenital chloride diarrhea with molecular confirmation of diagnosis. A 10-mo-old boy presented with failure to thrive, voluminous diarrhea, dehydration, hyponatremia, hypokalemia, metabolic alkalosis and history of maternal polyhydramnios. The diagnosis of congenital chloride diarrhea was based on high fecal and low urinary chloride excretion, in addition to biochemical abnormalities. Genetic testing revealed a novel homozygous mutation in exon 4 of the SLC26A3 gene that encodes the protein regulating chloride bicarbonate absorption in distal ileum and colon. Therapy with oral fluids and electrolytes led to decrease in stool frequency and improvement in growth parameters. PMID:26637435

  19. Congenital sensorineural hearing loss

    SciTech Connect

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-02-01

    The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems.

  20. Congenital adrenal hyperplasia

    PubMed Central

    Dessinioti, Cleo; Katsambas, Andreas

    2009-01-01

    Congenital adrenal hyperplasia consists of a heterogenous group of inherited disorders due to enzymatic defects in the biosynthetic pathway of cortisol and/or aldosterone. This results in glucocorticoid deficiency, mineralocorticoid deficiency, and androgen excess. 95% of CAH cases are due to 21-hydroxylase deficiency. Clinical forms range from the severe, classical CAH associated with complete loss of enzyme function, to milder, non-classical forms (NCAH). Androgen excess affects the pilosebaceous unit, causing cutaneous manifestations such as acne, androgenetic alopecia and hirsutism. Clinical differential diagnosis between NCAH and polycystic ovary syndrome may be difficult. In this review, the evaluation of patients with suspected CAH, the clinical presentation of CAH forms, with emphasis on the cutaneous manifestations of the disease, and available treatment options, will be discussed. PMID:22523607

  1. Multicystic congenital mesoblastic nephroma.

    PubMed

    Drut, Ricardo

    2002-01-01

    This report describes an unusual example of congenital mesoblastic nephroma cellular variant that presented in a 1-week-old neonate as a multicystic tumor of the kidney. Extensive pseudocystic cavitation resulted from progressive accumulation of ground substance in a loosely myxoid tissue composed of stellate- and spindle-shaped cells that compressed and infiltrated renal tissue. The cells of the tumor were positive for vimentin and smooth muscle actin. The patient is alive and well 16 years after surgery. Differential diagnosis from segmental cystic dysplasia, cystic intralobar nephrogenic rest, cystic nephroma, cystic partially differentiated nephroblastoma, cystic nephroblastoma, and cystic clear cell sarcoma of the kidney, all of which may present at this age, is discussed. PMID:11927972

  2. Congenital mirror movements.

    PubMed Central

    Schott, G D; Wyke, M A

    1981-01-01

    In this report are described seven patients assessed clinically and neuropsychologically in whom mirror movements affecting predominantly the hands occurred as a congenital disorder. These mirror movements, representing a specific type of abnormal synkinesia, may arise as a hereditary condition, in the presence of a recognisable underlying neurological abnormality, and sporadically, and the seven patients provide more or less satisfactory examples of each of these three groups. Despite the apparent uniformity of the disorder, the heterogeneity and variability may be marked, examples in some of our patients including the pronounced increase in tone that developed with arm movement, and the capacity for modulation of the associated movement by alteration of neck position and bio-feedback. Various possible mechanisms are considered; these include impaired cerebral inhibition of unwanted movements, and functioning of abnormal motor pathways. Emphasis has been placed on the putative role of the direct, crossed corticomotoneurone pathways and on the unilateral and bilateral cerebral events that precede movement. PMID:7288446

  3. Congenital vertical talus: a review.

    PubMed

    McKie, Janay; Radomisli, Timothy

    2010-01-01

    Congenital vertical talus, also known as congenital convex pes valgus, is an uncommon disorder of the foot, manifested as a rigid rocker-bottom flatfoot. Radiographically, it is defined by dorsal dislocation of the navicular on the talus. This condition requires surgical correction. If left untreated, this foot deformity results in a painful and rigid flatfoot with weak push-off power. This article provides an overview of this rare foot deformity, outlines appropriate workup of the disorder, and details current treatment options, with emphasis on the evolution of treatment of congenital vertical talus. PMID:19963176

  4. Laparoscopic resection of adult colon duplication causing intussusception

    PubMed Central

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

    2016-01-01

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

  5. A case of sigmoid colon duplication in an adult woman.

    PubMed

    Al-Jaroof, Abdulla Hassan; Al-Zayer, Faisal; Meshikhes, Abdul-Wahed Nasir

    2014-01-01

    Colonic duplication is a rare congenital anomaly that is often diagnosed in childhood, but may go unrecognised until adulthood. It often presents with chronic abdominal pain and constipation, and the preoperative diagnosis may be difficult. We present a case of sigmoid duplication in a 33-year-old Indonesian woman who presented with right-sided colicky abdominal pain and vomiting. Clinical examination was unremarkable and radiological investigations raised the possibility of a giant colon diverticulum. The patient underwent exploratory laparotomy that revealed a tubular sigmoid duplication. A sigmoid colectomy with end-to-end anastomosis was performed. She was discharged a week later and remained well at 1 year follow-up. Colon duplications rarely present in adult life and the accurate diagnosis is often made at laparotomy. PMID:25096653

  6. Laparoscopic resection of adult colon duplication causing intussusception.

    PubMed

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

    2016-02-21

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

  7. Correlation between fluorescein flowmetry and laser Doppler flowmetry. A study in the intestine (ileoanal pouch) in man.

    PubMed

    Perbeck, L; Lindquist, K; Proano, E; Liljeqvist, L

    1990-05-01

    A study was undertaken to compare two new methods of capillary blood flow measurement, namely fluorescein flowmetry (FF) and laser Doppler flowmetry (LDF). The blood flow was measured in a pelvic pouch during its construction and in the completed ileoanal anastomosis in 12 patients. There was a high correlation between the two methods (correlation coefficient, 0.78) (p less than 0.01) when the blood flow was measured in the pelvic pouch. The correlation coefficient between the two methods for the difference between the blood flow in the pelvic pouch at the site of the planned anastomosis when the pouch resided in the abdomen and that in the completed ileoanal anastomosis was r = 0.99 (n = 12, p less than 0.001); the reduction amounted to 25% as measured by FF and 27% as measured by LDF (n = 12, p less than 0.01). All ileoanal anastomoses healed perfectly, the lowest FF and LDF values being 0.004 density units/sec and 0.3 V, respectively. The results indicate that either method can be considered for measuring capillary blood flow. PMID:2163097

  8. A Case of Sigmoid Colon Tuberculosis Mimicking Colon Cancer

    PubMed Central

    Yu, Seong-Min; Kim, Min-Dae; Lee, Hee-Ryong; Jung, Peel; Ryu, Tae-Hyun; Choi, Seung-Ho; Lee, Il-Seon

    2012-01-01

    Tuberculosis of the sigmoid colon is a rare disorder. An 80-year-old man visited Bongseng Memorial Hospital for medical examination. A colonoscopy was performed, and a lesion in the sigmoid colon that was suspected to be colon cancer was found. A biopsy was performed, and tuberculous enteritis with chronic granulomatous inflammation was diagnosed. Intestinal tuberculosis is most frequent in the ileocecal area, followed by the ascending colon, transverse colon, duodenum, stomach, and sigmoid colon, in descending order. Hence, we report a case of intestinal tuberculosis in the sigmoid colon, which is rare and almost indistinguishable from colon cancer. PMID:23185709

  9. Label-free vascular imaging in a spontaneous hamster cheek pouch carcinogen model for pre-cancer detection (Conference Presentation)

    NASA Astrophysics Data System (ADS)

    Hu, Fangyao; Morhard, Robert; Liu, Heather; Murphy, Helen; Farsiu, Sina; Ramanujam, Nimmi

    2016-03-01

    Inducing angiogenesis is one hallmark of cancer. Tumor induced neovasculature is often characterized as leaky, tortuous and chaotic, unlike a highly organized normal vasculature. Additionally, in the course of carcinogenesis, angiogenesis precedes a visible lesion. Tumor cannot grow beyond 1-2 mm in diameter without inducing angiogenesis. Therefore, capturing the event of angiogenesis may aid early detection of pre-cancer -important for better treatment prognoses in regions that lack the resources to manage invasive cancer. In this study, we imaged the neovascularization in vivo in a spontaneous hamster cheek pouch carcinogen model using a, non-invasive, label-free, high resolution, reflected-light spectral darkfield microscope. Hamsters' cheek pouches were painted with 7,12-Dimethylbenz[a]anthracene (DMBA) to induce pre-cancerous to cancerous changes, or mineral oil as control. High resolution spectral darkfield images were obtained over the course of pre-cancer development and in control cheek pouches. The vasculature was segmented with a multi-scale Gabor filter with an 85% accuracy compared with manually traced masks. Highly tortuous vasculature was observed only in the DMBA treated cheek pouches as early as 6 weeks of treatment. In addition, the highly tortuous vessels could be identified before a visible lesion occurred later during the treatment. The vessel patterns as determined by the tortuosity index were significantly different from that of the control cheek pouch. This preliminary study suggests that high-resolution darkfield microscopy is promising tool for pre-cancer and early cancer detection in low resource settings.

  10. Dose-response effect of tomato paste on 7,12-dimethylbenz[a]anthracene-induced hamster buccal pouch carcinogenesis.

    PubMed

    Bhuvaneswari, V; Velmurugan, B; Nagini, S

    2004-06-01

    We evaluated the dose-response effect of tomato paste on 7,12-dimethylbenz[a]anthracene (DMBA)-induced hamster buccal pouch (HBP) carcinogenesis using lipid peroxidation, reduced glutathione (GSH) and the GSH-dependent enzymes; glutathione peroxidase (GPx), glutathione-S-transferase (GST) and gamma-glutamyltranspeptidase (GGT) as biomarkers of chemoprevention. Hamsters were divided into eight groups of six animals each. The right buccal pouches of animals in group 1 were painted with a 0.5 per cent DMBA in liquid paraffin three times per week. Animals in groups 2 to 4 painted with DMBA as in group 1, received in addition, intragastric administration of tomato paste containing lycopene at concentrations of 2.5, 5 and 10 mgkg(-1)bw, respectively three times per week on days alternate to DMBA application. Groups 5 through 7 were given tomato paste alone. Animals in group 8 served as controls. All animals were killed after an experimental period of 14 weeks. Lipid peroxidation and GSH-dependent antioxidants were measured in the buccal pouch, liver and erythrocytes. Diminished lipid peroxidation in the HBP tumours was associated with enhanced levels of GSH and GSH-dependent enzymes. In contrast to the buccal pouch, the liver and erythrocytes of tumour-bearing hamsters exhibited elevated lipid peroxidation accompanied by compromised antioxidant status. Administration of tomato paste significantly reduced the incidence of HBP tumours, modulated lipid peroxidation and enhanced GSH and GSH-dependent enzymes in the pouch, liver and erythrocytes. Among the three doses used, tomato paste containing 5 mgkg(-1)bw lycopene showed the optimum effect. It is suggested that tomato paste exerts its chemopreventive effects by modulating lipid peroxidation and enhancing antioxidants in the target organ as well as in the liver and erythrocytes. PMID:15354408

  11. Carvedilol alleviates adjuvant-induced arthritis and subcutaneous air pouch edema: Modulation of oxidative stress and inflammatory mediators

    SciTech Connect

    Arab, Hany H.; El-Sawalhi, Maha M.

    2013-04-15

    Rheumatoid arthritis (RA) is a systemic inflammatory disease with cardiovascular complications as the leading cause of morbidity. Carvedilol is an adrenergic antagonist which has been safely used in treatment of several cardiovascular disorders. Given that carvedilol has powerful antioxidant/anti-inflammatory properties, we aimed to investigate its protective potential against arthritis that may add further benefits for its clinical usefulness especially in RA patients with concomitant cardiovascular disorders. Two models were studied in the same rat; adjuvant arthritis and subcutaneous air pouch edema. Carvedilol (10 mg/kg/day p.o. for 21 days) effectively suppressed inflammation in both models with comparable efficacy to the standard anti-inflammatory diclofenac (5 mg/kg/day p.o.). Notably, carvedilol inhibited paw edema and abrogated the leukocyte invasion to air pouch exudates. The latter observation was confirmed by the histopathological assessment of the pouch lining that revealed mitigation of immuno-inflammatory cell influx. Carvedilol reduced/normalized oxidative stress markers (lipid peroxides, nitric oxide and protein thiols) and lowered the release of inflammatory cytokines (TNF-α and IL-6), and eicosanoids (PGE{sub 2} and LTB{sub 4}) in sera and exudates of arthritic rats. Interestingly, carvedilol, per se, didn't present any effect on assessed biochemical parameters in normal rats. Together, the current study highlights evidences for the promising anti-arthritic effects of carvedilol that could be mediated through attenuation of leukocyte migration, alleviation of oxidative stress and suppression of proinflammatory cytokines and eicosanoids. - Highlights: ► Carvedilol possesses promising anti-arthritic properties. ► It markedly suppressed inflammation in adjuvant arthritis and air pouch edema. ► It abrogated the leukocyte invasion to air pouch exudates and linings. ► It reduced/normalized oxidative stress markers in sera and exudates of

  12. Small Bowel Obstruction due to Anomalous Congenital Bands in Children

    PubMed Central

    Soysal, Feryal Gun; Ozbey, Huseyin; Keskin, Erbug; Celik, Alaattin; Karadag, Aslı; Salman, Tansu

    2016-01-01

    Introduction. The aim of the study was to evaluate our children who are operated on for anomalous congenital band while increasing the awareness of this rare reason of intestinal obstruction in children which causes a diagnostic challenge. Patients and Methods. We retrospectively reviewed the records of fourteen children treated surgically for intestinal obstructions caused by anomalous congenital bands. Results. The bands were located between the following regions: the ascending colon and the mesentery of the terminal ileum in 4 patients, the jejunum and mesentery of the terminal ileum in 3 patients, the ileum and mesentery of the terminal ileum in 2 patients, the ligament of Treitz and mesentery of the jejunum in one patient, the ligament of Treitz and mesentery of the terminal ileum in one patient, duodenum and duodenum in one patient, the ileum and mesentery of the ileum in one patient, the jejunum and mesentery of the jejunum in one patient, and Meckel's diverticulum and its ileal mesentery in one patient. Band excision was adequate in all of the patients except the two who received resection anastomosis for intestinal necrosis. Conclusion. Although congenital anomalous bands are rare, they should be considered in the differential diagnosis of patients with an intestinal obstruction. PMID:27478432

  13. Enhanced Contaminated Human Remains Pouch: initial development and preliminary performance assessments

    SciTech Connect

    Iseli, A.M.; Kwen, H.D.; Ul-Alam, M.; Balasubramanian, M.; Rajagopalan, S.

    2011-11-07

    The objective is to produce a proof of concept prototype Enhanced Contaminated Human Remains Pouch (ECHRP) with self-decontamination capability to provide increased protection to emergency response personnel. The key objective was to decrease the concentration of toxic chemicals through the use of an absorbent and reactive nanocellulose liner. Additionally, nanomaterials with biocidal properties were developed and tested as a 'stand-alone' treatment. The setting was a private company research laboratory. The main outcome measures were production of a functional prototype. A functional prototype capable of mitigating the threats due to sulfur mustard, Soman, and a large variety of liquid and vapor toxic industrial chemicals was produced. Stand-alone biocidal treatment efficacy was validated. The ECHRP provides superior protection from both chemical and biological hazards to various emergency response personnel and human remains handlers.

  14. USING TRAINED POUCHED RATS TO DETECT LAND MINES: ANOTHER VICTORY FOR OPERANT CONDITIONING

    PubMed Central

    Poling, Alan; Weetjens, Bart; Cox, Christophe; Beyene, Negussie W; Bach, Harvard; Sully, Andrew

    2011-01-01

    We used giant African pouched rats (Cricetomys gambianus) as land mine-detection animals in Mozambique because they have an excellent sense of smell, weigh too little to activate mines, and are native to sub-Saharan Africa, and therefore are resistant to local parasites and diseases. In 2009 the rats searched 93,400 m2 of land, finding 41 mines and 54 other explosive devices. Humans with metal detectors found no additional mines. On average, the rats emitted 0.33 false alarm for every 100 m2 searched, which is below the threshold given by International Mine Action Standards for accrediting mine-detection animals. These findings indicate that Cricetomys are accurate mine-detection animals and merit continued use in this capacity. PMID:21709791

  15. Cytological and bacteriological evaluation of transendoscopic guttural pouch lavages in clinically healthy horses.

    PubMed

    Dobesova, Olga; Bezdekova, Barbora

    2016-06-01

    Thirty-eight guttural pouch lavages from 19 clinically healthy horses were collected transendoscopically. Cytological examination and bacteriological culture of the samples were carried out. All 38 lavages assessed for cytology contained less than five per cent neutrophils and could be termed as cytologically 'normal'. In none of the lavages did the neutrophil count vary between five and 25 per cent or exceed 25 per cent, which are the borderlines for being termed 'reactive' or 'pathological', respectively. Epithelial cells were the most commonly represented cell type in all lavages. Bacteriological culture was positive in 16 out of 38 lavages, but specific pathogenic bacteria were not cultivated in any of them, and only transient microflora was present. Although the relationship between the presence of specific bacteria, neutrophil count and total cell count could not be statistically evaluated due to the absence of 'reactive' and 'pathological' lavages, transendoscopic lavage appears to be a reliable method for obtaining a sample for bacteriological culture. PMID:27342089

  16. Combination chemoprevention of hamster buccal pouch carcinogenesis by bovine milk lactoferrin and black tea polyphenols.

    PubMed

    Mohan, K V P Chandra; Letchoumy, P Vidjaya; Hara, Y; Nagini, S

    2008-03-01

    Combination chemoprevention is a promising approach for oral cancer prevention. The authors evaluated the combined chemopreventive effects of bovine milk lactoferrin (bLF) and black tea polyphenols (Polyphenon-B) in a clinically relevant in vivo model of 7,12-dimethylbenz[a]anthracene (DMBA)-induced hamster buccal pouch (HBP) carcinogenesis. Although dietary administration of bLF and Polyphenon-B alone significantly reduced the tumor incidence, combined administration of bLF and polyphenon-B was more effective in inhibiting DMBA-induced genotoxicity and development of HBP carcinomas by modulation of carcinogen-metabolizing enzymes and cellular redox status. These results suggest that a "designer item" approach will be useful for human oral cancer prevention strategies. PMID:18259952

  17. Anti-inflammatory effects of Houttuynia cordata supercritical extract in carrageenan-air pouch inflammation model.

    PubMed

    Kim, Dajeong; Park, Dongsun; Kyung, Jangbeen; Yang, Yun-Hui; Choi, Ehn-Kyoung; Lee, Yoon-Bok; Kim, Hyun-Kyu; Hwang, Bang Yeon; Kim, Yun-Bae

    2012-06-01

    Anti-inflammatory effects of Houttuynia cordata supercritical extract (HSE) were investigated in rat carrageenan-air pouch model. Oral administration of HSE (50-200 mg/kg) suppressed carrageenan-induced exudation and albumin leakage, as well as inflammatory cell infiltration at a high dose (200 mg/kg). Intraperitoneal injection of dexamethasone (2 mg/kg) only decreased exudation and cell infiltration, while indomethacin (2 mg/kg, i.p.) reduced exudate volume and albumin content without influence on the cell number. HSE lowered tumor-necrosis factor-α (TNF-α) and nitric oxide (NO), as well as prostaglandin E(2) (PGE(2)). Dexamethasone only reduced TNF-α and NO, while indomethacin decreased PGE(2). The results indicate that HSE exhibits anti-inflammatory effects by inhibiting both TNF-α-NO and cyclooxygenase-2-PGE(2) pathways. PMID:22787488

  18. Surgical treatment of ulcerative colitis: Ileorectal vs ileal pouch-anal anastomosis

    PubMed Central

    Scoglio, Daniele; Ahmed Ali, Usama; Fichera, Alessandro

    2014-01-01

    Total proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the current gold standard in the surgical treatment of ulcerative colitis (UC) refractory to medical management. A procedure of significant magnitude carries its own risks including anastomotic failure, pelvic sepsis and a low rate of neoplastic degeneration overtime. Recent studies have shown that total colectomy with ileorectal anastomosis (IRA) has been associated with good long-term functional results in a selected group of UC patients amenable to undergo a strict surveillance for the relatively high risk of cancer in the rectum. This manuscript will review and compare the most recent literature on IRA and IPAA as it pertains to postoperative morbidity and mortality, failure rates, functional outcomes and cancer risk. PMID:25309058

  19. Ultrasonography of wallaby prenatal development shows that the climb to the pouch begins in utero.

    PubMed

    Drews, Barbara; Roellig, Kathleen; Menzies, Brandon R; Shaw, Geoff; Buentjen, Ina; Herbert, Catherine A; Hildebrandt, Thomas B; Renfree, Marilyn B

    2013-01-01

    Marsupials have a functional placenta for a shorter period of time compared to that of eutherian species, and their altricial young reach the teats without any help from the mother. We have monitored the short intrauterine development of one marsupial, the tammar wallaby, with high-resolution ultrasound from reactivation of the 100-cell diapausing blastocyst to birth. The expanding blastocyst could be visualized when it had reached a diameter of 1.5 mm. From at least halfway through pregnancy, there are strong undulating movements of the endometrium that massage the expanding vesicle against the highly secretory endometrial surface. These unique movements possibly enhance exchange of uterine secretions and gases between the mother and embryo. There was a constant rate of development measured ultrasonographically from mid-gestation, regardless of when the blastocyst reactivated. Interestingly climbing movements by the fetus began in utero about 3 days before birth, mimicking those required to climb to the pouch. PMID:23492830

  20. Using giant african pouched rats to detect human tuberculosis: a review

    PubMed Central

    Poling, Alan; Mahoney, Amanda; Beyene, Negussie; Mgode, Georgies; Weetjens, Bart; Cox, Christophe; Durgin, Amy

    2015-01-01

    Despite its characteristically low sensitivity, sputum smear microscopy remains the standard for diagnosing tuberculosis (TB) in resource-poor countries. In an attempt to develop an alternative or adjunct to microscopy, researchers have recently examined the ability of pouched rats to detect TB-positive human sputum samples and the microbiological variables that affect their detection. Ten published studies, reviewed herein, suggest that the rats are able to detect the specific odor of Mycobacterium tuberculosis, which causes TB, and can substantially increase new-case detections when used for second-line TB screening following microscopy. Further research is needed to ascertain the rats’ ability to detect TB in children and in HIV-positive patients, to detect TB when used for first-line screening, and to be useful in broad-scale applications where cost-effectiveness is a major consideration. PMID:26587178

  1. [Congenital lumbar hernia].

    PubMed

    Peláez Mata, D J; Alvarez Muñoz, V; Fernández Jiménez, I; García Crespo, J M; Teixidor de Otto, J L

    1998-07-01

    Hernias in the lumbar region are abdominal wall defects that appear in two possible locations: the superior lumbar triangle of Grynfelt-Lesshaft and the inferior lumbar triangle of Petit. There are 40 cases reported in the pediatric literature, and only 16 are considered congenital, associated with the lumbocostovertebral syndrome and/or meningomyelocele. A new case is presented. A premature newborn with a mass in the left flank that increases when the patient cries and reduces easily. The complementary studies confirm the diagnosis of lumbar hernia and reveal the presence of lumbocostovertebral syndrome associated. At the time of operation a well defined fascial defect at the superior lumbar triangle of Grynfelt-Lesshaft is primarily closed. The diagnosis of lumbar hernia is not difficult to establish but it is necessary the screening of the lumbocostovertebral syndrome. We recommend the surgical treatment before 12 months of age; the objective is to close the defect primarily or to use prosthetic material if necessary. PMID:12602034

  2. [Congenital Esophageal Atresia].

    PubMed

    Suzuki, Makoto; Kuwano, Hiroyuki

    2015-07-01

    In this report, we describe the esophageal atresia in terms of current surgical management on the basis of our experience and literatures. Traditionally, infants with esophageal atresia have presented shortly after birth because of an inability to pass an orogastric tube, respiratory distress, or an inability to tolerate feeding. And also, an isolated trachea-esophageal fistula (TEF) usually cases coughing, recurrent pneumonia, or choking during feedings. To ignore these symptoms is to risk a delayed diagnosis. The condition may be associated with other major congenital anomalies such as those seen in the vertebral, anal, cardiac, tracheo-esophageal, renal/radial (VACTER) association, or it may be an isolated defect. Therapeutic strategies for esophageal atresia are a prevention of pulmonary complication by TEF closing and an early establishment of enteral alimentation. We promptly repair healthy infants without performing a gastrostomy and delay repair in infants with high-risk factors such as associated severe cardiac anomaly and respiratory insufficiency. Esophageal atresia has been classically approached through a thoracotomy. The disadvantages of such a thoracotomy have been recognized for a long time, for example winged scapula, elevation of fixation of shoulder, asymmetry of the chest wall, rib fusion, scoliosis, and breast and pectoral muscle maldevelopment. To avoid such disadvantages, thoracoscopic repair was recently reported. PMID:26197921

  3. Congenital parotid fistula.

    PubMed

    Natasha, Shiggaon

    2014-01-01

    Parotid fistula is a cause of great distress and embarrassment to the patient. Parotid fistula is most commonly a post-traumatic situation. Congenital parotid salivary fistulas are unusual entities that can arise from accessory parotid glands or even more infrequently, from normal parotid glands through an aberrant Stensen's duct. The treatment of fistulous tract is usually surgical and can be successfully excised after making a skin incision along the skin tension line around the fistula opening. This report describes a case of right accessory parotid gland fistula of a 4-year-old boy with discharge of pus from right cheek. Computed tomography (CT) fistulography and CT sialography demonstrated fistulous tract arising from accessory parotid gland. Both CT fistulography and CT sialography are very helpful in the diagnosis and surgical planning. In this case, superficial parotidectomy is the treatment of choice. A detailed history, clinical and functional examination, proper salivary gland investigations facilitates in correct diagnosis followed by immediate surgical intervention helps us to restore physical, psychological health of the child patient. PMID:25231049

  4. Congenital Diaphragmatic Hernia

    PubMed Central

    2012-01-01

    Congenital Diaphragmatic Hernia (CDH) is defined by the presence of an orifice in the diaphragm, more often left and posterolateral that permits the herniation of abdominal contents into the thorax. The lungs are hypoplastic and have abnormal vessels that cause respiratory insufficiency and persistent pulmonary hypertension with high mortality. About one third of cases have cardiovascular malformations and lesser proportions have skeletal, neural, genitourinary, gastrointestinal or other defects. CDH can be a component of Pallister-Killian, Fryns, Ghersoni-Baruch, WAGR, Denys-Drash, Brachman-De Lange, Donnai-Barrow or Wolf-Hirschhorn syndromes. Some chromosomal anomalies involve CDH as well. The incidence is < 5 in 10,000 live-births. The etiology is unknown although clinical, genetic and experimental evidence points to disturbances in the retinoid-signaling pathway during organogenesis. Antenatal diagnosis is often made and this allows prenatal management (open correction of the hernia in the past and reversible fetoscopic tracheal obstruction nowadays) that may be indicated in cases with severe lung hypoplasia and grim prognosis. Treatment after birth requires all the refinements of critical care including extracorporeal membrane oxygenation prior to surgical correction. The best hospital series report 80% survival but it remains around 50% in population-based studies. Chronic respiratory tract disease, neurodevelopmental problems, neurosensorial hearing loss and gastroesophageal reflux are common problems in survivors. Much more research on several aspects of this severe condition is warranted. PMID:22214468

  5. Congenital diaphragmatic hernia.

    PubMed

    Tovar, Juan A

    2012-01-01

    Congenital Diaphragmatic Hernia (CDH) is defined by the presence of an orifice in the diaphragm, more often left and posterolateral that permits the herniation of abdominal contents into the thorax. The lungs are hypoplastic and have abnormal vessels that cause respiratory insufficiency and persistent pulmonary hypertension with high mortality. About one third of cases have cardiovascular malformations and lesser proportions have skeletal, neural, genitourinary, gastrointestinal or other defects. CDH can be a component of Pallister-Killian, Fryns, Ghersoni-Baruch, WAGR, Denys-Drash, Brachman-De Lange, Donnai-Barrow or Wolf-Hirschhorn syndromes. Some chromosomal anomalies involve CDH as well. The incidence is < 5 in 10,000 live-births. The etiology is unknown although clinical, genetic and experimental evidence points to disturbances in the retinoid-signaling pathway during organogenesis. Antenatal diagnosis is often made and this allows prenatal management (open correction of the hernia in the past and reversible fetoscopic tracheal obstruction nowadays) that may be indicated in cases with severe lung hypoplasia and grim prognosis. Treatment after birth requires all the refinements of critical care including extracorporeal membrane oxygenation prior to surgical correction. The best hospital series report 80% survival but it remains around 50% in population-based studies. Chronic respiratory tract disease, neurodevelopmental problems, neurosensorial hearing loss and gastroesophageal reflux are common problems in survivors. Much more research on several aspects of this severe condition is warranted. PMID:22214468

  6. Congenital Triangular Alopecia.

    PubMed

    Yin Li, Vincent Chum; Yesudian, Paul Devakar

    2015-01-01

    Congenital triangular alopecia (CTA) also known as temporal triangular alopecia is a benign noncicatricial pattern of hair loss. It typically affects the frontotemporal region and rarely involves the temporoparietal or occipital scalp. It is a nonprogressive disorder that presents as a triangular, oval or lancet-shaped patch of alopecia. CTA can manifest at birth or develop later in life. The exact etiology of this condition remains unknown. Rarely, it may be associated with other disorders such as Down's syndrome and phakomatosis pigmentovascularis. The diagnosis is based on its distinct clinical appearance. Histologically, hair follicles are miniaturized and replaced by sparse vellus hair follicles. Tricoscopy using a polarized light handheld dermatoscope can be a useful diagnostic tool. CTA is often asymptomatic and remains unchanged throughout the life. No treatment is required. Surgical intervention with follicular unit hair transplantation can provide a satisfactory cosmetic result. In this paper, we have identified 126 cases of CTA in the published literature cited on PubMed between 1905 and 2015. From the available evidence, 79% of patients with CTA presented with unilateral hair loss, 18.5% with bilateral involvement and rarely, with occipital alopecia (2.5%). There was no gender predilection. These figures are entirely consistent with previously published data. Physicians should remember to consider CTA as a potential diagnosis in any patient presenting with a nonscarring alopecia in order to avoid unnecessary investigations and treatments. PMID:26180448

  7. Congenital Rhabdomyosarcoma of Shoulder

    PubMed Central

    Khaleghnejad-Tabari, Ahmad; Mirshemirani, Alireza; Rouzrokh, Mohsen; Nariman, Shahin; Hassas-Yeganeh, Shaghayegh; Gharib, Atoosa; Khaleghnejad-Tabari, Nasibeh

    2012-01-01

    A 16-day-old female was referred with congenital swelling on her right shoulder. On examination, there was a hard, round, ecchymotic, nontender, slightly movable, warm and shiny 10x15 cm mass on the right axillary pits which was extended to the right side of neck and chest wall. The mass separated the shoulder from the chest wall causing paralysis of right hand. Chest X-ray, ultrasound and MRI with contrast demonstrated a soft tissue mass suspected to be a hemangioma. The mass rapidly increased in size despite aggressive steroid therapy with rupture and bleeding. On the 45th post natal day the baby was taken to operating room to control the bleeding and if possible total excision of the mass. The mass was separated easily from the surrounding tissue and was excised along with right upper extremity. At the end of surgery the baby had cardiac arrest, and apparently died of Disseminated Intravascular Coagulation (DIC). The final pathology report was Rhabdomyosarcoma (RMS). PMID:25628836

  8. [Congenital foot abnormalities].

    PubMed

    Delpont, M; Lafosse, T; Bachy, M; Mary, P; Alves, A; Vialle, R

    2015-03-01

    The foot may be the site of birth defects. These abnormalities are sometimes suspected prenatally. Final diagnosis depends on clinical examination at birth. These deformations can be simple malpositions: metatarsus adductus, talipes calcaneovalgus and pes supinatus. The prognosis is excellent spontaneously or with a simple orthopedic treatment. Surgery remains outstanding. The use of a pediatric orthopedist will be considered if malposition does not relax after several weeks. Malformations (clubfoot, vertical talus and skew foot) require specialized care early. Clubfoot is characterized by an equine and varus hindfoot, an adducted and supine forefoot, not reducible. Vertical talus combines equine hindfoot and dorsiflexion of the forefoot, which is performed in the midfoot instead of the ankle. Skew foot is suspected when a metatarsus adductus is resistant to conservative treatment. Early treatment is primarily orthopedic at birth. Surgical treatment begins to be considered after walking age. Keep in mind that an abnormality of the foot may be associated with other conditions: malposition with congenital hip, malformations with syndromes, neurological and genetic abnormalities. PMID:25524290

  9. Singing in congenital amusia.

    PubMed

    Dalla Bella, Simone; Giguère, Jean-François; Peretz, Isabelle

    2009-07-01

    Congenital amusia is a musical disorder characterized by impaired pitch perception. To examine to what extent this perceptual pitch deficit may compromise singing, 11 amusic individuals and 11 matched controls were asked to sing a familiar tune with lyrics and on the syllable /la/. Acoustical analysis of sung renditions yielded measures of pitch accuracy (e.g., number of pitch errors) and time accuracy (e.g., number of time errors). The results revealed that 9 out of 11 amusics were poor singers, mostly on the pitch dimension. Poor singers made an anomalously high number of pitch interval and contour errors, produced pitch intervals largely deviating from the score, and lacked pitch stability; however, more than half of the amusics sang in-time. Amusics' variability in singing proficiency was related to their residual pitch perceptual ability. Thus, their singing deficiency might be a consequence of their perceptual deficit. Nevertheless, there were notable exceptions. Two amusic individuals, despite their impoverished perception, sang proficiently. The latter findings are consistent with the existence of separate neural pathways for auditory perception and action. PMID:19603898

  10. ASSESSMENT OF THE GASTRO-JEJUNO-DUODENAL TRANSIT AFTER JEJUNAL POUCH INTERPOSITION

    PubMed Central

    da SILVA, Alcino Lázaro; GOMES, Célio Geraldo de Oliveira

    2015-01-01

    Background : The jejunal pouch interposition between the gastric body and the duodenum after the gastrectomy, although not frequent in the surgical practice today, has been successfully employed for the prevention and treatment of the postgastrectomy syndromes. In the latter, it is included the dumping syndrome, which affects 13-58% of the patients who undergo gastrectomy. Aim : Retrospective assessment of the results of this procedure for the prevention of the dumping syndrome. Methods : Fourty patients were selected and treatetd surgically for peptic ulcer, between 1965 and 1970. Of these, 29 underwent vagotomy, antrectomy, gastrojejunalduodenostomy at the lesser curvature level, and the 11 remaining were submitted to vagotomy, antrectomy, gastrojejunal-duodenostomy at the greater curvature level. The gastro-jejuno-duodenal transit was assessed in the immediate or late postoperative with the contrasted study of the esophagus, stomach and duodenum. The clinical evolution was assessed according to the Visick grade. Results : Of the 40 patients, 28 were followed with the contrast evaluation in the late postoperative. Among those who were followed until the first month (n=22), 20 (90%) had slow gastro-jejuno-duodenal transit and in two (10%) the transit was normal. Among those who were followed after the first month (n=16), three (19%) and 13 (81%) had slow and normal gastric emptying, respectively. None had the contrasted exam compatible with the dumping syndrome. Among the 40 patients, 22 underwent postoperative clinical evaluation. Of these, 19 (86,5%) had excellent and good results (Visick 1 and 2, respectively). Conclusions : The jejunal pouch interposition showed to be a very effective surgical procedure for the prevention of the dumping syndrome in gastrectomized patients. PMID:26734789

  11. Thoracoscopic repair of a large neonatal congenital diaphragmatic hernia using Gerota's fascia.

    PubMed

    Fukuzawa, Hiroaki; Tamaki, Akihiko; Takemoto, Jyunkichi; Morita, Keiichi; Endo, Kosuke; Iwade, Tamaki; Yuichi, Okata; Bitoh, Yuko; Yokoi, Akiko; Maeda, Kosaku

    2015-05-01

    A large congenital diaphragmatic hernia needing patch repair has a high risk of recurrence. Thus, managing these large congenital diaphragmatic hernias under thoracoscopy has become a problem. Here, a large congenital diaphragmatic hernia that was repaired using Gerota's fascia under thoracoscopy is reported. In the present case, it was impossible to close the hernia directly under thoracoscopy because the hernia was too large. Gerota's fascia was raised up by the left kidney and used for the repair. The left colon adhering to Gerota's fascia was mobilized, and a large space was made under thoracoscopy. Gerota's fascia was fixed to the diaphragmatic defect. The patient's postoperative course was good, and there was no recurrence. This technique could be one option for repairing a large hernia under thoracoscopy. PMID:25913592

  12. [Congenital gastrointestinal tract obstructions (pictorial essay)].

    PubMed

    Coşkun, Abdulhakim; Sevinç, Halil

    2004-03-01

    A wide spectrum of congenital anomalies may cause obstruction in the upper and lower gastrointestinal tract. Neonates with complete upper intestinal obstruction do not usually require further radiological evaluation after radiography. Barium studies are sometimes needed. Barium studies and other comprehensive methods such as ultrasonography, computed tomography and magnetic resonance imaging are usually complementary procedures which are not usually helpful and may even delay surgery, resulting in some complications and death. The decision to perform a given imaging examination should be considered carefully to avoid unnecessary radiation exposure to the patient. The diagnosis of low intestinal obstruction is usually apparent at abdominal radiography because of the presence of many dilated loops. The differentiation between ileal and colonic obstruction can be made with a contrast enema study. Dilute ionic, water-soluble contrast agents and non-balloon tip catheter of appropriate size is preferred for neonatal contrast enemas. Barium sulphate suspensions typically should not be used because of their potential to exacerbate the impaction of meconium plugs in meconium ileus, whereas water-soluble enemas can be therapeutic. PMID:15054709

  13. Congenital Cytomegalovirus Infection: Audiologic Outcome

    PubMed Central

    Fowler, Karen B.

    2013-01-01

    The association between congenital cytomegalovirus (CMV) infection and sensorineural hearing loss (SNHL) was first described almost 50 years ago. Studies over the intervening decades have further described the relationship between congenital CMV infection and SNHL in children. However, congenital CMV infection remains a leading cause of SNHL in children in the United States and the world today. As more CMV infections are identified, it is important to recognize that infants who are born to seroimmune mothers are not completely protected from SNHL, although their hearing loss is often milder than that seen in CMV-infected infants following primary maternal infections. Late-onset and progressive hearing losses occur following congenital CMV infection, and CMV-infected infants should be evaluated regularly to provide for early detection of hearing loss and appropriate intervention. Fluctuating hearing loss that is not explained by concurrent middle ear infections is another characteristic of CMV-related hearing loss in children. Challenges still remain in predicting which children with congenital CMV infection will develop hearing loss and, among those who do develop loss, whether or not the loss will continue to deteriorate. PMID:24257423

  14. Congenital Scoliosis (Mini-review).

    PubMed

    Weiss, Hans-Rudolf; Moramarco, Marc

    2016-01-01

    Congenital scoliosis is a lateral deformity of the spine with a disturbance of the sagittal profile caused by malformations of vertebra and ribs. Typically, early surgical intervention is the suggested treatment (before three-years-old) for young patients with congenital scoliosis. While a previous study was conducted in 2011 to investigate long-term studies supporting the necessity for this recommendation and no evidence was found, this current review, is an updated search for evidence published from 2011 through March 2015. This also failed to find any prospective or randomized controlled studies to support the hypothesis that spinal fusion surgery in patients with congenital scoliosis should be considered as evidence-based treatment. Contradictory results exist on the safety of hemivertebra resection and segmental fusion using pedicle screw fixation. When using the VEPTR (vertical expandable prosthetic titanium rib) device, studies show a high rate of complications exist. It is difficult to predict the final outcome for patients with congenital scoliosis. However, it is possible that many patients with congenital scoliosis may be able to avoid spinal surgery with the application of advanced bracing technology. Therefore, it is only prudent to advocate for conservative management first before spinal surgery is considered. PMID:26769614

  15. Treatment of congenital malformations.

    PubMed

    Brucker, Sara Yvonne; Rall, Katharina; Campo, Rudi; Oppelt, Peter; Isaacson, Keith

    2011-03-01

    The prevalence of müllerian malformations is 1 in 200, or 0.5%. A third of the anomalies are septate, a third bicornuate uteri, 10% arcuate uterus, 10% didelphis and unicornuate uterus, and < 5% uterine and vaginal aplasia. Correct diagnosis of the malformation is most important but often very difficult. Correct treatment can only be performed if the malformation is clear. Longitudinal vaginal septums have to be removed due to potential obstetric problems. Transverse vaginal septums can cause hematocolpos and pain and have to be incised crosswise and excised so as not to shorten the vagina at the same time. Congenital vaginal agenesis occurs in Mayer-Rokitansky-Kuster-Hauser syndrome patients and in androgen insensitivity syndrome. The first choice for surgical treatment should be the new laparoscopic-assisted creation of a neovagina. Septate uterus has to be distinguished from a bicornuate uterus. Even if it is not proven to be a cause for infertility, the chance of miscarriage can be diminished by performing hysteroscopic metroplasty. Repair of a uterine septum in infertility patients often improves pregnancy rates. In contrast, surgical repair of a bicornuate uterus requires an abdominal metroplasty. This should only be performed if the patient has recurrent fetal loss due to the uterine structural defect. In a unicornuate uterus it is most important to determine if there is a second uterine horn that can cause cyclic pain if it has functioning endometrium. The only surgical option in these cases is to remove the rudimentary uterus with endometrium and hematometra, respectively. PMID:21437824

  16. Common Tests for Congenital Heart Defects

    MedlinePlus

    ... Heart Defect - Fetal Circulation • Care & Treatment • Tools & Resources Web Booklets on Congenital Heart Defects These online publications ... to you or your child’s defect and concerns. Web Booklet: Adults With Congenital Heart Defects Web Booklet: ...

  17. Genetics Home Reference: congenital dyserythropoietic anemia

    MedlinePlus

    ... Understand Genetics Home Health Conditions CDA congenital dyserythropoietic anemia Enable Javascript to view the expand/collapse boxes. ... PDF Open All Close All Description Congenital dyserythropoietic anemia ( CDA ) is an inherited blood disorder that affects ...

  18. Care and Treatment for Congenital Heart Defects

    MedlinePlus

    ... Physical Activity Recommendations for Heart Health • Tools & Resources Web Booklets on Congenital Heart Defects These online publications ... to you or your child’s defect and concerns. Web Booklet: Adults With Congenital Heart Defects Web Booklet: ...

  19. Genetic Counseling for Congenital Heart Defects

    MedlinePlus

    ... Pressure High Blood Pressure Tools & Resources Stroke More Genetic Counseling for Congenital Heart Defects Updated:Oct 26, ... person with congenital heart disease considers having children. Genetic counseling can help answer these questions and address ...

  20. Understand Your Risk for Congenital Heart Defects

    MedlinePlus

    ... health problems than their parents. Learn more about genetic counseling . Single gene: Rarely, congenital heart defects are caused ... of Congenital Heart Defects • Understand Your Risk Introduction Genetic Counseling • Symptoms & Diagnosis • Care & Treatment • Tools & Resources Related Sites ...

  1. Magnetic resonance imaging of congenital heart disease

    SciTech Connect

    Fletcher, B.D.; Jacobstein, M.D.

    1988-01-01

    Focusing primarily on MR imaging of the heart, this book covers other diagnostic imaging modalities as well. The authors review new technologies and diagnostic procedures pertinent to congenital heat disease and present each congenital heat abnormality as a separate entity.

  2. Volvulus of the ascending colon in a non-rotated midgut: Plain film and MDCT findings

    PubMed Central

    Camera, Luigi; Calabrese, Milena; Mainenti, Pier Paolo; Masone, Stefania; Vecchio, Walter Del; Persico, Giovanni; Salvatore, Marco

    2012-01-01

    Colonic volvulus is a relatively uncommon cause of large bowel obstruction usually involving mobile, intra-peritoneal, colonic segments. Congenital or acquired anatomic variation may be associated with an increased risk of colonic volvulus which can occasionally involve retro-peritoneal segments. We report a case of 54-year-old female who presented to our Institution to perform a plain abdominal film series for acute onset of cramping abdominal pain. Both the upright and supine films showed signs of acute colonic obstruction which was thought to be due to an internal hernia of the transverse colon into the lesser sac. The patient was therefore submitted to a multi-detector contrast-enhanced computed tomography (CT). CT findings were initially thought to be consistent with the presumed diagnosis of internal hernia but further evaluation and coronal reformatting clearly depicted the presence of a colonic volvulus possibly resulting from a retro-gastric colon. At surgery, a volvulus of the ascending colon was found and a right hemi-colectomy had to be performed. However, a non rotated midgut with a right-sided duodeno-jejunal flexure and a left sided colon was also found at laparotomy and overlooked in the pre-operative CT. Retrospective evaluation of CT images was therefore performed and a number of CT signs of intestinal malrotation could be identified. PMID:23150768

  3. [The congenital afibrinogenemia: case report].

    PubMed

    Brahem, Imen; Charfeddine, Bassem; Chraiti, Haythem; Ben Abdallah, Jihene; Ben Othmen, Leila; Neffati, Souhir; Ali Smach, Mohamed; Ltaief, Affef; Ksourri, Monia; Dridi, Hedi; Limem, Khalifa

    2010-01-01

    The deficiency in factor I or fibrinogen is a largely unknown genetic disease. It is a rare condition inherited as an autosomal recessive, whose clinical events are variable, ranging from moderate to minimal bleeding or cataclysmic hemorrhage. We report a case of congenital afibrinogenemia in a 17 years-old patient hospitalized in surgical ICU for hemoperitoneum medium abundance discovered by abdominal ultrasound performed before a picture of abdominopelvic pain lasting for 24 hours. Exploration led to the diagnosis of congenital afibrinogenemia with favorable evolution with a contribution of factor deficient. Through this case we raise the problem of congenital afibrinogenemia in diagnosis and the peculiarities of its management. PMID:20870582

  4. Culture - colonic tissue

    MedlinePlus

    ... from the large intestine. The cause may be bacteria, fungi, or viruses. ... bacteria Cytomegalovirus Mycobacterium tuberculosis bacteria Salmonella bacteria Shigella bacteria These organisms may lead to diarrhea or infections involving the colon.

  5. Transverse colon conduit diversion

    SciTech Connect

    Schmidt, J.D.; Buchsbaum, H.J.

    1986-05-01

    The versatility and other advantages of the transverse colon conduit for urinary diversion have been described and implemented in 50 patients. Because most patients considered for this procedure will be at high risk because of a history of significant pelvic irradiation, underlying malignancy, poor renal function, fistula, and so forth, the technical details of surgery and patient selection cannot be minimized. The transverse colon segment is indicated for primary supravesical diversion as well as for salvage of problems related to ileal conduits. Adenocarcinoma of the colon is an unlikely long-term complication of this form of diversion because the fecal stream is absent. Now that the transverse colon conduit has been used for more than 10 years, meaningful comparisons with ileal segments should soon be available.

  6. [Angiodysplasia of the colon].

    PubMed

    Bruni, R; Rossodivita, I; Santoro, M; La Banca, G; Rollo, R; Putti, R

    1990-01-01

    The Authors report their experience with a case of angiodysplasia of the colon. It is outlined how these lesions can be demonstrated by angiography and colonoscopy. The pathophysiology, diagnosis and management are discussed as well. PMID:2223469

  7. Laparoscopic Colon Resection

    MedlinePlus

    ... inches to complete the procedure. What are the Advantages of Laparoscopic Colon Resection? Results may vary depending ... type of procedure and patient’s overall condition. Common advantages are: Less postoperative pain May shorten hospital stay ...

  8. Colon cancer screening

    MedlinePlus

    ... screening; Sigmoidoscopy - screening; Virtual colonoscopy - screening; Fecal immunochemical test; Stool DNA test; sDNA test ... death and complications caused by colorectal cancer. SCREENING TESTS There are several ways to screen for colon ...

  9. Paternal transmission of congenital myotonic dystrophy.

    PubMed Central

    Bergoffen, J; Kant, J; Sladky, J; McDonald-McGinn, D; Zackai, E H; Fischbeck, K H

    1994-01-01

    The congenital form of myotonic dystrophy is reported to be almost exclusively, if not exclusively, maternally transmitted. We present a case of congenital myotonic dystrophy which was inherited from a mildly affected father. This family illustrates that the congenital form of myotonic dystrophy can occur without intrauterine or other maternal factors related to the disease. The possibility of paternal transmission of the congenital form of myotonic dystrophy could be considered when counselling myotonic dystrophy patients and their families. Images PMID:7966187

  10. Intestinal colonization resistance

    PubMed Central

    Lawley, Trevor D; Walker, Alan W

    2013-01-01

    Dense, complex microbial communities, collectively termed the microbiota, occupy a diverse array of niches along the length of the mammalian intestinal tract. During health and in the absence of antibiotic exposure the microbiota can effectively inhibit colonization and overgrowth by invading microbes such as pathogens. This phenomenon is called ‘colonization resistance’ and is associated with a stable and diverse microbiota in tandem with a controlled lack of inflammation, and involves specific interactions between the mucosal immune system and the microbiota. Here we overview the microbial ecology of the healthy mammalian intestinal tract and highlight the microbe–microbe and microbe–host interactions that promote colonization resistance. Emerging themes highlight immunological (T helper type 17/regulatory T-cell balance), microbiota (diverse and abundant) and metabolic (short-chain fatty acid) signatures of intestinal health and colonization resistance. Intestinal pathogens use specific virulence factors or exploit antibiotic use to subvert colonization resistance for their own benefit by triggering inflammation to disrupt the harmony of the intestinal ecosystem. A holistic view that incorporates immunological and microbiological facets of the intestinal ecosystem should facilitate the development of immunomodulatory and microbe-modulatory therapies that promote intestinal homeostasis and colonization resistance. PMID:23240815

  11. Congenital granular-cell myoblastoma.

    PubMed

    Cussen, L J; MacMahon, R A

    1975-04-01

    The clinical and pathologic features of congenital granular-cell myoblastoma in five infant girls are reported. One lesion, treated expectantly, progressively decreased in size and after 3 yr and 9 mo could not be detected, while two lesions which were imcompletely excised did not recur. It is suggested that congenital granular-cell myoblastoma is caused by an intrauterine stimulus, and that this stimulus may possible be production of estrogen by the fetus. Congential granular-cell myoblastoma should be treated expectantly or by limited excision, and has an excellent prognosis. PMID:164527

  12. [Congenital lumbar hernia and bilateral renal agenesis].

    PubMed

    Barrero Candau, R; Garrido Morales, M

    2007-04-01

    We report a new case of congenital lumbar hernia. This is first case reported of congenital lumbar hernia and bilateral renal agenesis. We review literature and describe associated malformations reported that would be role out in every case of congenital lumbar hernia. PMID:17650728

  13. Spontaneous colon perforations associated with a vascular type of ehlers-danlos syndrome.

    PubMed

    Yoneda, Akira; Okada, Kazuya; Okubo, Hitoshi; Matsuo, Mitsutoshi; Kishikawa, Hiroki; Naing, Banyar Than; Watanabe, Atsushi; Shimada, Takashi

    2014-05-01

    Ehlers-Danlos syndrome, vascular type (vEDS) (MIM #130050) is an autosomal dominant disorder caused by mutation in the type III collagen gene, COL3A1, leading to fragility of blood vessels, bowel and uterus that leads to spontaneous rupture. We report a previously undiagnosed vEDS patient with bowel complications. A 20-year-old female patient was referred to our hospital with abdominal pain. Computed tomography showed notable dilatation of the sigmoid colon with intraperitoneal fluid. Laparotomy revealed dilatation of the sigmoid colon, breakdown of serosa and muscularis propria of the sigmoid colon with impending perforation, and intra-abdominal hemorrhage caused by breakdown of the mesenterium. Resection of the sigmoid colon with Hartmann's pouch and an end colostomy were performed. Physical examination showed joint hypermobility, translucent skin with venous prominence and facial structure abnormalities. Genetic analysis using cDNA extracted from the patient's fibroblasts by reverse transcriptase polymerase chain reaction direct sequencing showed a missense mutation within the triple helix region of COL3A1 (c.2150 G>A; Gly717Asp). PMID:24932165

  14. Congenital long QT syndrome

    PubMed Central

    Crotti, Lia; Celano, Giuseppe; Dagradi, Federica; Schwartz, Peter J

    2008-01-01

    Congenital long QT syndrome (LQTS) is a hereditary cardiac disease characterized by a prolongation of the QT interval at basal ECG and by a high risk of life-threatening arrhythmias. Disease prevalence is estimated at close to 1 in 2,500 live births. The two cardinal manifestations of LQTS are syncopal episodes, that may lead to cardiac arrest and sudden cardiac death, and electrocardiographic abnormalities, including prolongation of the QT interval and T wave abnormalities. The genetic basis of the disease was identified in the mid-nineties and all the LQTS genes identified so far encode cardiac ion channel subunits or proteins involved in modulating ionic currents. Mutations in these genes (KCNQ1, KCNH2, KCNE1, KCNE2, CACNA1c, CAV3, SCN5A, SCN4B) cause the disease by prolonging the duration of the action potential. The most prevalent LQTS variant (LQT1) is caused by mutations in the KCNQ1 gene, with approximately half of the genotyped patients carrying KCNQ1 mutations. Given the characteristic features of LQTS, the typical cases present no diagnostic difficulties for physicians aware of the disease. However, borderline cases are more complex and require the evaluation of various electrocardiographic, clinical, and familial findings, as proposed in specific diagnostic criteria. Additionally, molecular screening is now part of the diagnostic process. Treatment should always begin with β-blockers, unless there are valid contraindications. If the patient has one more syncope despite a full dose β-blockade, left cardiac sympathetic denervation (LCSD) should be performed without hesitation and implantable cardioverter defibrillator (ICD) therapy should be considered with the final decision being based on the individual patient characteristics (age, sex, clinical history, genetic subgroup including mutation-specific features in some cases, presence of ECG signs – including 24-hour Holter recordings – indicating high electrical instability). The prognosis of the

  15. Detection of vesicant-induced upper airway mucosa damage in the hamster cheek pouch model using optical coherence tomography

    NASA Astrophysics Data System (ADS)

    Hammer-Wilson, Marie J.; Nguyen, Vi; Jung, Woong-Gyu; Ahn, Yehchen; Chen, Zhongping; Wilder-Smith, Petra

    2010-01-01

    Hamster cheek pouches were exposed to 2-chloroethyl ethyl sulfide [CEES, half-mustard gas (HMG)] at a concentration of 0.4, 2.0, or 5.0 mg/ml for 1 or 5 min. Twenty-four hours post-HMG exposure, tissue damage was assessed by both stereomicrography and optical coherence tomography (OCT). Damage that was not visible on gross visual examination was apparent in the OCT images. Tissue changes were found to be dependent on both HMG concentration and exposure time. The submucosal and muscle layers of the cheek pouch tissue showed the greatest amount of structural alteration. Routine light microscope histology was performed to confirm the OCT observations.

  16. The mitogenomes of the pouched lamprey (Geotria australis) and least brook lamprey (Lampetra aepyptera) with phylogenetic considerations.

    PubMed

    Ren, Jianfeng; Pu, Jiafei; Buchinger, Tyler; Zhu, Xinyun; Baker, Cindy; Li, Weiming

    2016-09-01

    We report the mitogenomes of the pouched lamprey (Geotria australis) and least brook lamprey (Lampetra aepyptera) in the families Geotriidae and Petromyzontidae, respectively. Both of the mitogenomes contain the 37 typical vertebrate genes. Their gene order and contents are identical to those of previously described lamprey mitogenomes. The mitogenome of G. australis (17 080 bp) is the largest among the 10 reported lamprey mitogenomes, owed to two long noncoding regions. The mitogenome of L. aepyptera is 77 bp longer (16 236 bp) than that of the congeneric European river lamprey L. fluviatilis, a size difference mostly due to different copy numbers of tandem repeats in the noncoding regions. The phylogenetic analysis supports that the pouched lamprey (Geotriidae) diverged earlier from the common ancestor of lampreys than the Petromyzonids, and the placement of the least brook lamprey in the genus Lampetra. PMID:26330185

  17. Detection of vesicant-induced upper airway mucosa damage in the hamster cheek pouch model using optical coherence tomography.

    PubMed

    Hammer-Wilson, Marie J; Nguyen, Vi; Jung, Woong-Gyu; Ahn, Yehchen; Chen, Zhongping; Wilder-Smith, Petra

    2010-01-01

    Hamster cheek pouches were exposed to 2-chloroethyl ethyl sulfide [CEES, half-mustard gas (HMG)] at a concentration of 0.4, 2.0, or 5.0 mg/ml for 1 or 5 min. Twenty-four hours post-HMG exposure, tissue damage was assessed by both stereomicrography and optical coherence tomography (OCT). Damage that was not visible on gross visual examination was apparent in the OCT images. Tissue changes were found to be dependent on both HMG concentration and exposure time. The submucosal and muscle layers of the cheek pouch tissue showed the greatest amount of structural alteration. Routine light microscope histology was performed to confirm the OCT observations. PMID:20210463

  18. Optimization of process conditions for Rohu fish in curry medium in retortable pouches using instrumental and sensory characteristics.

    PubMed

    Majumdar, Ranendra K; Dhar, Bahni; Roy, Deepayan; Saha, Apurba

    2015-09-01

    'Kalia', a popular preparation of Rohu fish, packed in four-layered laminated retort pouch was processed in a steam/air mixture over-pressure retort at 121.1 °C to three different F 0 values of 7, 8 and 9 min. Time-temperature data were collected during heat processing using an Ellab Sterilization Monitoring System. Texture profile such as hardness, springiness, gumminess and chewiness decreased as the F 0 value increased. The L* values decreased whereas a* and b* values increased with increasing F 0 value. Based on the commercial sterility, sensory evaluation, colour and texture profile analysis, F 0 value of 8 min and cook value of 66 min, with a total process time of 41.7 min at 121.1 °C was found satisfactory for the preparation of Rohu fish curry (Kalia) in retort pouches. PMID:26344980

  19. Topical photosan-mediated photodynamic therapy for DMBA-induced hamster buccal pouch premaligant lesions: an in vivo study

    NASA Astrophysics Data System (ADS)

    Hsu, Yih-Chih; Chiang, Chun-Pin; Chen, Jian Wen; Chen, Ying-Ru; Lee, Jeng-Woei

    2010-02-01

    One of the best strategies to prevent the occurrence of oral cancer is to eliminate oral precancers and block their further malignant transformation. Previous studies showed that photosan-mediated photodynamic therapy (photosan-PDT) is very effective for human head and neck cancers. To avoid the systemic photodynamic toxicity of photosan, this study was designed to use a topical photosan-PDT for treatment of DMBA-induced hamster buccal pouch precancerous lesions. Twelve 10-week-old male Syrian golden hamsters were used in this study. DMBA was applied to the left buccal pouches thrice a week for 8 to 10 weeks and mineral oil was painted on the right buccal pouches thrice a week for 8 to 10 weeks as the normal controls. Six hamsters were euthanized for tissue harvest. Precancerous lesions of moderate to severe dysplasia were consistently induced and proven by histological examination. These induced precancerous lesions in the remaining 6 hamsters were used for testing the efficacy of topical photosan-PDT. Before PDT, fluorescence spectroscopy was used to determine when protoporphyrine IX (PpIX) reached its peak level in the lesional epithelial cells after topical application of photosan-gel. We found that PpIX reached its peak level in precancerous lesions about 13.5 min after topical application of photosan-gel. The precancerous lesions in 4 hamsters were treated with topical photosan-PDT using the 635-nm LED light once or twice a week. Complete regression of the precancerous lesions was found after 2-4 PDT treatments by visual and histological examination. Our findings indicate that topical photosan-PDT is a very effective treatment modality for DMBA-induced hamster buccal pouch precancerous lesions.

  20. Further Assessment of Monkeypox Virus Infection in Gambian Pouched Rats (Cricetomys gambianus) Using In Vivo Bioluminescent Imaging.

    PubMed

    Falendysz, Elizabeth A; Lopera, Juan G; Lorenzsonn, Faye; Salzer, Johanna S; Hutson, Christina L; Doty, Jeffrey; Gallardo-Romero, Nadia; Carroll, Darin S; Osorio, Jorge E; Rocke, Tonie E

    2015-01-01

    Monkeypox is a zoonosis clinically similar to smallpox in humans. Recent evidence has shown a potential risk of increased incidence in central Africa. Despite attempts to isolate the virus from wild rodents and other small mammals, no reservoir host has been identified. In 2003, Monkeypox virus (MPXV) was accidentally introduced into the U.S. via the pet trade and was associated with the Gambian pouched rat (Cricetomys gambianus). Therefore, we investigated the potential reservoir competence of the Gambian pouched rat for MPXV by utilizing a combination of in vivo and in vitro methods. We inoculated three animals by the intradermal route and three animals by the intranasal route, with one mock-infected control for each route. Bioluminescent imaging (BLI) was used to track replicating virus in infected animals and virological assays (e.g. real time PCR, cell culture) were used to determine viral load in blood, urine, ocular, nasal, oral, and rectal swabs. Intradermal inoculation resulted in clinical signs of monkeypox infection in two of three animals. One severely ill animal was euthanized and the other affected animal recovered. In contrast, intranasal inoculation resulted in subclinical infection in all three animals. All animals, regardless of apparent or inapparent infection, shed virus in oral and nasal secretions. Additionally, BLI identified viral replication in the skin without grossly visible lesions. These results suggest that Gambian pouched rats may play an important role in transmission of the virus to humans, as they are hunted for consumption and it is possible for MPXV-infected pouched rats to shed infectious virus without displaying overt clinical signs. PMID:26517839

  1. Further Assessment of Monkeypox Virus Infection in Gambian Pouched Rats (Cricetomys gambianus) Using In Vivo Bioluminescent Imaging

    PubMed Central

    Falendysz, Elizabeth A.; Lopera, Juan G.; Lorenzsonn, Faye; Salzer, Johanna S.; Hutson, Christina L.; Doty, Jeffrey; Gallardo-Romero, Nadia; Carroll, Darin S.; Osorio, Jorge E.; Rocke, Tonie E.

    2015-01-01

    Monkeypox is a zoonosis clinically similar to smallpox in humans. Recent evidence has shown a potential risk of increased incidence in central Africa. Despite attempts to isolate the virus from wild rodents and other small mammals, no reservoir host has been identified. In 2003, Monkeypox virus (MPXV) was accidentally introduced into the U.S. via the pet trade and was associated with the Gambian pouched rat (Cricetomys gambianus). Therefore, we investigated the potential reservoir competence of the Gambian pouched rat for MPXV by utilizing a combination of in vivo and in vitro methods. We inoculated three animals by the intradermal route and three animals by the intranasal route, with one mock-infected control for each route. Bioluminescent imaging (BLI) was used to track replicating virus in infected animals and virological assays (e.g. real time PCR, cell culture) were used to determine viral load in blood, urine, ocular, nasal, oral, and rectal swabs. Intradermal inoculation resulted in clinical signs of monkeypox infection in two of three animals. One severely ill animal was euthanized and the other affected animal recovered. In contrast, intranasal inoculation resulted in subclinical infection in all three animals. All animals, regardless of apparent or inapparent infection, shed virus in oral and nasal secretions. Additionally, BLI identified viral replication in the skin without grossly visible lesions. These results suggest that Gambian pouched rats may play an important role in transmission of the virus to humans, as they are hunted for consumption and it is possible for MPXV-infected pouched rats to shed infectious virus without displaying overt clinical signs. PMID:26517839

  2. Further assessment of Monkeypox Virus infection in Gambian pouched rats (Cricetomys gambianus) using in vivo bioluminescent imaging

    USGS Publications Warehouse

    Falendysz, Elizabeth; Lopera, Juan G.; Faye Lorenzsonn; Salzer, Johanna S.; Hutson, Christina L.; Doty, Jeffrey; Gallardo-Romero, Nadia; Carroll, Darin S.; Osorio, Jorge E.; Rocke, Tonie E.

    2015-01-01

    Monkeypox is a zoonosis clinically similar to smallpox in humans. Recent evidence has shown a potential risk of increased incidence in central Africa. Despite attempts to isolate the virus from wild rodents and other small mammals, no reservoir host has been identified. In 2003,Monkeypox virus (MPXV) was accidentally introduced into the U.S. via the pet trade and was associated with the Gambian pouched rat (Cricetomys gambianus). Therefore, we investigated the potential reservoir competence of the Gambian pouched rat for MPXV by utilizing a combination of in vivo and in vitro methods. We inoculated three animals by the intradermal route and three animals by the intranasal route, with one mock-infected control for each route. Bioluminescent imaging (BLI) was used to track replicating virus in infected animals and virological assays (e.g. real time PCR, cell culture) were used to determine viral load in blood, urine, ocular, nasal, oral, and rectal swabs. Intradermal inoculation resulted in clinical signs of monkeypox infection in two of three animals. One severely ill animal was euthanized and the other affected animal recovered. In contrast, intranasal inoculation resulted in subclinical infection in all three animals. All animals, regardless of apparent or inapparent infection, shed virus in oral and nasal secretions. Additionally, BLI identified viral replication in the skin without grossly visible lesions. These results suggest that Gambian pouched rats may play an important role in transmission of the virus to humans, as they are hunted for consumption and it is possible for MPXV-infected pouched rats to shed infectious virus without displaying overt clinical signs.

  3. Electrochemical properties of large-sized pouch-type lithium ion batteries with bio-inspired organic cathode materials

    NASA Astrophysics Data System (ADS)

    Yeo, Jae-Seong; Yoo, Eun-Ji; Ha, Sang-Hyeon; Cheong, Dong-Ik; Cho, Sung-Baek

    2016-05-01

    To investigate the feasibility of scaling up bio-inspired organic materials as cathode materials in lithium ion batteries, large-sized pouch cells are successfully prepared via tape casting using lumichrome with an alloxazine structure and aqueous styrene butadiene rubber-carboxymethyl cellulose (SBR-CMC) binders. A battery module with a two-in-series, six-in-parallel (2S6P) configuration is also successfully fabricated and is able to power blue LEDs (850 mW). Lumichrome shows no structural changes during the fabrication processes used to produce the positive electrode. The large-sized pouch cells show two sets of cathodic and anodic peaks with average potentials of 2.58 V and 2.26 V vs. Li/Li+, respectively. The initial discharge capacities are 142 mAh g-1 and 148 mAh g-1 for ethylene carbonate-dimethyl carbonate (EC-DMC) and tetraethylene glycol dimethyl ether (TEGDME) electrolytes, respectively, similar to that of a coin cell (149 mAh g-1). The EC-DMC-injected pouch cells exhibit higher rate performance and cyclability than the TEGDME-injected ones. The TEGDME electrolyte is not suitable for lithium metal anodes because of electrolyte decomposition and subsequent cell swelling.

  4. Methanolic Extract of Ficus carica Linn. Leaves Exerts Antiangiogenesis Effects Based on the Rat Air Pouch Model of Inflammation

    PubMed Central

    Eteraf-Oskouei, Tahereh; Allahyari, Saeideh; Akbarzadeh-Atashkhosrow, Arezu; Delazar, Abbas; Pashaii, Mahdiyeh; Gan, Siew Hua; Najafi, Moslem

    2015-01-01

    The antiangiogenesis effect of Ficus carica leaves extract in an air pouch model of inflammation was investigated in rat. Inflammation was induced by injection of carrageenan into pouches. After antioxidant capacity and total phenolic content (TPC) investigations, the extract was administered at 5, 25, and 50 mg/pouch, and then the volume of exudates, the cell number, TNFα, PGE2, and VEGF levels were measured. Angiogenesis of granulation tissues was determined by measuring hemoglobin content. Based on the DPPH assay, the extract had significant antioxidant activity with TPC of 11.70 mg GAE/100 g dry sample. In addition, leukocyte accumulation and volume of exudate were significantly inhibited by the extract. Moreover, it significantly decreased the production of TNFα, PGE2, and VEGF, while angiogenesis was significantly inhibited by all administered doses. Interestingly, attenuation of angiogenesis and inflammatory parameters (except leukocyte accumulation) by the extract was similar to that shown by diclofenac. The extract has anti-inflammatory effects and ameliorated cell influx and exudation to the site of the inflammatory response which may be related to the local inhibition of TNFα, PGE2, and VEGF levels as similarly shown by diclofenac. The antiangiogenesis and anti-VEGF effects of Ficus carica may be correlated with its significant antioxidant potentials. PMID:25977699

  5. Topical photosan-mediated photodynamic therapy for DMBA-induced hamster buccal pouch early cancer lesions: an in vivo study

    NASA Astrophysics Data System (ADS)

    Hsu, Yih-Chih; Chang, Walter Hong-Shong; Chang, Junn-Liang; Liu, Kuang-Ting; Chiang, Chun-Pin; Liu, Chung-Ji; Chen, Chih-Ping

    2011-03-01

    Oral cancer has becomes the most prominent cancer disease in recent years in Taiwan. The reason is the betel nut chewing habit combing with smoking and alcohol-drinking lifestyle of people results in oral cancer becomes the fastest growth incident cancer amongst other major cancer diseases. In previous studies showed that photosan, haematoporphyrin derivative (HPD), has demonstrated effective PDT results on human head and neck disease studies. To avoid the systemic phototoxic effect of photosan, this study was designed to use a topical photosan-mediated PDT for treatment of DMBA-induced hamster buccal pouch cancerous lesions. DMBA was applied to one of the buccal pouches of hamsters thrice a week for 10 to 12 weeks. Cancerous lesions were induced and proven by histological examination. These DMBA-induced cancerous lesions were used for testing the efficacy of topical photosan-mediated PDT. Before PDT, fluorescence spectroscopy was used to determine when photosan reached its peak level in the lesional epithelial cells after topical application of photosan gel. We found that photosan reached its peak level in cancerous lesions about 13.5 min after topical application of photosan gel. The cancerous lesions in hamsters were then treated with topical photosan-mediated PDT (fluence rate: 600 mW/cm2; light exposure dose 200 J/cm2) using the portable Lumacare 635 nm fiber-guided light device. Visual examination demonstrated that topical photosan-mediated PDT was an applicable treatment modality for DMBA-induced hamster buccal pouch cancerous lesions.

  6. Combination therapies in adjuvant with topical ALA-mediated photodynamic therapy for DMBA-induced hamster buccal pouch premalignant lesions

    NASA Astrophysics Data System (ADS)

    Yang, Deng-Fu; Hsu, Yih-Chih

    2012-03-01

    In Taiwan, oral cancer has becomes the fastest growth male cancer disease due to the betel nut chewing habit combing with smoking and alcohol-drinking lifestyle of people. In order to eliminate the systemic phototoxic effect of 5-aminolevulinic acid (ALA), this study was designed to use a topical ALA-mediated PDT for treatment of DMBA-induced hamster buccal pouch precancerous lesions. DMBA was applied to one of the buccal pouches of hamsters thrice a week for 10 to 12 weeks. Cancerous lesions were induced and proven by histological examination. These DMBA-induced cancerous lesions were used for testing the efficacy of topical ALA-mediated PDT. Before PDT, fluorescence spectroscopy was used to determine when ALA reached its peak level in the lesional epithelial cells after topical application of ALA gel. We found that ALA reached its peak level in precancerous lesions about 2.5 hrs after topical application of ALA gel. The cancerous lesions in hamsters were then treated with topical ALA -mediated PDT with light exposure dose of 150 J/cm2 using LED 635 nm fiber-guided light device. Visual examination demonstrated that adjuvant topical ALA -mediated PDT group has shown better therapeutic results in compared to those of non-adjuvant topical ALA-mediated PDT group for DMBA-induced hamster buccal pouch precancerous lesions.

  7. Development and on-orbit operation of lithium-ion pouch battery for small scientific satellite “REIMEI”

    NASA Astrophysics Data System (ADS)

    Uno, Masatoshi; Ogawa, Keita; Takeda, Yasuo; Sone, Yoshitsugu; Tanaka, Koji; Mita, Makoto; Saito, Hirobumi

    2011-10-01

    A lithium-ion battery was developed using off-the-shelf pouch cells and launched with a small scientific satellite "REIMEI." The cells were potted with polyurethane or epoxy resin to protect the battery from vacuum in space. Preliminary experimental test results of pouch cells potted in a soft aluminum cap suggested that the cells tended to swell in vacuum, although they had been reinforced with the resins. Bread board models (BBMs), in which pouch cells were potted with resins in a hard aluminum case, were fabricated for cycle life performance tests in the laboratory. The test results indicated that the performance of epoxy-potted BBM was superior to that of the polyurethane-potted BBM. The measured cell resistance implied that the electrolyte solution leaked through the polyurethane resin, resulting in premature deterioration. The epoxy resin was used for the flight battery. The end-of-discharge-voltage (EoDV) trend of the flight battery on orbit was compared with the laboratory test results corrected based on a post-launch cycle test using a fresh cell. The corrected EoDV trend in the laboratory was in good agreement with the on-orbit trend for the early cycle period, indicating that the on-orbit battery was not inadvertently affected by conditions in space.

  8. Methanolic Extract of Ficus carica Linn. Leaves Exerts Antiangiogenesis Effects Based on the Rat Air Pouch Model of Inflammation.

    PubMed

    Eteraf-Oskouei, Tahereh; Allahyari, Saeideh; Akbarzadeh-Atashkhosrow, Arezu; Delazar, Abbas; Pashaii, Mahdiyeh; Gan, Siew Hua; Najafi, Moslem

    2015-01-01

    The antiangiogenesis effect of Ficus carica leaves extract in an air pouch model of inflammation was investigated in rat. Inflammation was induced by injection of carrageenan into pouches. After antioxidant capacity and total phenolic content (TPC) investigations, the extract was administered at 5, 25, and 50 mg/pouch, and then the volume of exudates, the cell number, TNFα, PGE2, and VEGF levels were measured. Angiogenesis of granulation tissues was determined by measuring hemoglobin content. Based on the DPPH assay, the extract had significant antioxidant activity with TPC of 11.70 mg GAE/100 g dry sample. In addition, leukocyte accumulation and volume of exudate were significantly inhibited by the extract. Moreover, it significantly decreased the production of TNFα, PGE2, and VEGF, while angiogenesis was significantly inhibited by all administered doses. Interestingly, attenuation of angiogenesis and inflammatory parameters (except leukocyte accumulation) by the extract was similar to that shown by diclofenac. The extract has anti-inflammatory effects and ameliorated cell influx and exudation to the site of the inflammatory response which may be related to the local inhibition of TNFα, PGE2, and VEGF levels as similarly shown by diclofenac. The antiangiogenesis and anti-VEGF effects of Ficus carica may be correlated with its significant antioxidant potentials. PMID:25977699

  9. Chemopreventive and antioxidant efficacy of (6)-paradol in 7,12-dimethylbenz(a)anthracene induced hamster buccal pouch carcinogenesis.

    PubMed

    Suresh, Kathiresan; Manoharan, Shanmugam; Vijayaanand, Mariadoss Arokia; Sugunadevi, Govindasamy

    2010-01-01

    The present study evaluated the chemopreventive potential of (6)-paradol, a pungent phenolic constituent of ginger, on 7,12-dimethylbenz(a)anthracene (DMBA)-induced hamster buccal pouch carcinogenesis. The mechanistic pathway for the chemopreventive potential of (6)-paradol was evaluated by measuring the status of tumor incidence, volume and burden as well as by analyzing the status of phase II detoxification agents, lipid peroxidation and antioxidants. Oral squamous cell carcinoma was induced in hamster buccal pouches by painting them with 0.5% DMBA in liquid paraffin three times a week for 14 weeks. We observed 100% tumor formation with marked biochemical abnormalities in tumor-bearing animals compared to control animals. Oral administration of 30 mg/kg b.w. (6)-paradol to DMBA-treated hamsters on alternate days from DMBA painting for 14 weeks, significantly reduced the formation of tumors and improved the status of detoxification agents, lipid peroxidation and antioxidants. Therefore, the present study suggests that (6)-paradol has potent chemopreventive, anti-lipid peroxidative and antioxidant potentials as well as a modulating effect on phase II detoxification enzyme and reduced glutathione (GSH) in DMBA-induced hamster buccal pouch carcinogenesis. PMID:21273675

  10. Lipopolysaccharide-Activated Leukocytes Enhance Thymic Stromal Lymphopoietin Production in a Mouse Air-Pouch-Type Inflammation Model.

    PubMed

    Segawa, Ryosuke; Mizuno, Natsumi; Hatayama, Takahiro; Jiangxu, Dong; Hiratsuka, Masahiro; Endo, Yasuo; Hirasawa, Noriyasu

    2016-08-01

    Thymic stromal lymphopoietin (TSLP) is a key cytokine that exacerbates allergic and fibrotic reactions. Several microbes and virus components have been shown to induce TSLP production, mainly in epithelial cells. TLR4 activators, such as lipopolysaccharide (LPS), induce TSLP production in vivo, although the underlying mechanisms remain unclear. In this study, we investigated the contribution of LPS-activated leukocytes to the production of TSLP in a mouse air-pouch-type inflammation model. LPS induced the production of TSLP in this model but not in the mouse keratinocyte cell line PAM212. Transfer of the infiltrated leukocytes collected from an LPS-injected air pouch to the air pouch of another mouse enhanced TSLP production. Further, the LPS-activated leukocytes produced tumor necrosis factor alpha (TNF-α) and interleukin-1 beta (IL-1β); a deficiency in these cytokines attenuated the LPS-induced production of TSLP. TSLP production was induced by TNF-α and enhanced by IL-1β and LPS in the PAM212 cells. These results demonstrated that TNF-α and IL-1β, which are partly produced by LPS-activated leukocytes, contribute to TSLP production via TLR4 activation in vivo. PMID:27271511

  11. Low-level X-radiation effects on functional vascular changes in Syrian hamster cheek pouch epithelium during hydrocarbon carcinogenesis

    SciTech Connect

    Lurie, A.G.; Coghill, J.E.; Rippey, R.M.

    1985-07-01

    Effects of repeated low-level X radiation on functional microvascular changes in hamster cheek pouch epithelium during and following carcinogenesis by 7,12-dimethylbenz(a)anthracene (DMBA) were studied. Hamsters were treated with either radiation, DMBA, radiation + DMBA, or no treatment. Animals were sacrificed at 3-week intervals from 0 to 39 weeks after treatments began. Pouch vascular volume and permeability changes were studied by fractional distributions of radiotracers and were analyzed by a variety of statistical methods which explored the vascular parameters, treatment types, elapsed time, presence of the carcinogen, and histopathologic changes. All treatments resulted in significant changes in vascular volume with time, while only DMBA treatments alone resulted in significant changes in vascular permeability with time. As in prior studies, there were significant vascular volume differences between DMBA and DMBA + radiation groups of tumor-bearing cheek pouches. Radiation significantly affected DMBA-associated vascular volume and permeability changes during carcinogenesis. Several possible explanations for the relationship of these changes to the enhancement of DMBA carcinogenesis are discussed.

  12. Health in adults with congenital heart disease.

    PubMed

    Cuypers, Judith A A E; Utens, Elisabeth M W J; Roos-Hesselink, Jolien W

    2016-09-01

    Since the introduction of cardiac surgery, the prospects for children born with a cardiac defect have improved spectacularly. Many reach adulthood and the population of adults with congenital heart disease is increasing and ageing. However, repair of congenital heart disease does not mean cure. Many adults with congenital heart disease encounter late complications. Late morbidity can be related to the congenital heart defect itself, but may also be the consequence of the surgical or medical treatment or longstanding alterations in hemodynamics, neurodevelopment and psychosocial development. This narrative review describes the cardiac and non-cardiac long-term morbidity in the adult population with congenital heart disease. PMID:27451323

  13. Experimental model of congenital syphilis.

    PubMed Central

    Kajdacsy-Balla, A; Howeedy, A; Bagasra, O

    1993-01-01

    Female LSH hamsters infected with Treponema pallidum subsp, endemicum before pregnancy or during early pregnancy transmit a form of syphilis to the fetus that is similar to human congenital syphilis. The offspring develops rhinitis, skin rash, failure to thrive, and hepatosplenomegaly. T. pallidum is detectable in their livers, spleens, and nasal secretions. Immunoglobulin M antibodies are detected in the serum. Images PMID:8335390

  14. Congenital Chagas disease: an update

    PubMed Central

    Carlier, Yves; Sosa-Estani, Sergio; Luquetti, Alejandro O; Buekens, Pierre

    2015-01-01

    Congenital infection with Trypanosoma cruzi is a global problem, occurring on average in 5% of children born from chronically infected mothers in endemic areas, with variations depending on the region. This presentation aims to focus on and update epidemiological data, research methods, involved factors, control strategy and possible prevention of congenital infection with T. cruzi. Considering that etiological treatment of the child is always effective if performed before one year of age, the diagnosis of infection in pregnant women and their newborns has to become the standard of care and integrated into the surveillance programs of syphilis and human immunodeficiency virus. In addition to the standard tests, polymerase chain reaction performed on blood of neonates of infected mothers one month after birth might improve the diagnosis of congenital infection. Recent data bring out that its transmission can be prevented through treatment of infected women before they become pregnant. The role of parasite genotypes and host genetic factors in parasite transmission and development of infection in foetuses/neonates has to be more investigated in order to better estimate the risk factors and impact on health of congenital infection with T. cruzi. PMID:25760448

  15. Advances in understanding colonic function.

    PubMed

    Milla, Peter J

    2009-04-01

    The colon is an organ of conservation that salvages water, electrolytes, and energy. The organization of colonic function is determined by the roles played by the luminal flora, the function of the different mucosal epithelial cell types, immunocompetent cells, and the neuromusculature. These different components of the colon interact with one another and with the colonic flora, and different areas of the colon serve different functions. In the normal adult during the course of a day the colon absorbs approximately 1.5 L of fluid, but under the influence of aldosterone increases up to 5 to 6 L. Diarrhoea occurs when secretion exceeds absorptive processes by either small intestinal secretion overwhelming colonic salvage or salvage being impaired by reduced colonic absorption or increased colonic secretion. PMID:19300122

  16. Evolutive leukoencephalopathy in congenital cytomegalovirus infection.

    PubMed

    Krakar, Goran; Đaković, Ivana; Delin, Sanja; Bošnjak, Vlatka Mejaški

    2015-01-01

    Congenital cytomegalovirus infection is the most common infectious cause of congenital brain injury. Type and severity of congenital cytomegalovirus infection-related brain abnormalities depend on the developmental stage of the central nervous system at the time of fetal infection. The aim of this study was to follow the course of leukoencephalopathy in a patient with congenital cytomegalovirus infection. We describe brain magnetic resonance imaging (MRI) findings of a boy with symptomatic congenital cytomegalovirus infection performed at the age of 3 weeks, 13 months, and 4 and 7 years. Neonatal brain MRI showed most of characteristic findings in congenital cytomegalovirus infection with most prominent white matter abnormalities and cortical dysplasia. MRI follow-up images showed that cortical dysgenesis remained unchanged and static, whereas white matter abnormalities evolved over the years. We propose that leukoencephalopathy in congenital cytomegalovirus infection is not only nonprogressive or static but even evolutive and suggests both underlying disruption and delay of myelination. PMID:24453153

  17. Coping with Congenital Hand Differences

    PubMed Central

    Franzblau, Lauren E.; Chung, Kevin C.; Carlozzi, Noelle; Chin, Autumn Y. T.; Nellans, Kate W.; Waljee, Jennifer F.

    2015-01-01

    Purpose Although functional outcomes following reconstruction for congenital hand differences are frequently described, much less is known regarding children’s ability to cope with psychosocial effects of these conditions. We qualitatively explored stress and coping mechanisms among children following reconstructive surgery for congenital hand differences. Methods Forty patients and their parents participated in semi-structured interviews examining stress related to hand functioning and appearance, emotional responses to stress, and coping strategies. Interviews were audio-taped, transcribed, and analyzed thematically. A consensus taxonomy for classifying content evolved from comparisons of coding by two reviewers. Themes expressed by participants were studied for patterns of connection and grouped into broader categories. Results In this sample, 58% of children and 40% of parents reported stress related to congenital hand differences, attributed to functional deficits (61%), hand appearance (27%), social interactions (58%), and emotional reactions (46%). Among the 18 children who reported stress, 43% of parents were not aware of the presence of stress. Eight coping strategies emerged, including humor (12%), self-acceptance (21%), avoidance (27%), seeking external support (30%), concealment (30%), educating others (9%), support programs (21%) and religion (24%). Conclusions Although children with congenital hand differences often experience emotional stress related to functional limitations and aesthetic deformities, many apply positive coping mechanisms that enhance self-esteem and self-esteem. Clinicians caring for children with congenital hand differences should inform families about potential sources of stress in order to direct resources toward strengthening coping strategies and support systems. Level of Evidence Level IV-Case series PMID:25502854

  18. Multi-analyte approach for determining the extraction of tobacco constituents from pouched snus by consumers during use

    PubMed Central

    2013-01-01

    Background Snus is a smokeless oral tobacco product with a significant history of use in Sweden, where it is regulated under food legislation. Users place a small porous sachet or a pinch of loose snus between the upper jaw and cheek for approximately one hour, leading to partial intake of tobacco constituents. To understand user exposure to tobacco, a multi-analyte approach based on the extraction of pouches by methanol, ethanol and water was validated and applied to the measurement of various constituents, including nicotine, four tobacco-specific nitrosamines (TSNAs), propylene glycol, water, ammonium, nitrate, sodium, chloride, linalool, citronellol, linalyl acetate and geraniol, extracted from snus pouches during use by human consumers. Results After validation against established single-analyte methods, the multi-analyte approach was used to determine constituent levels in snus pouches before and after one hour of use. Although the concentrations in the snus pouches varied from nanogram (e.g. TSNAs) to milligram (e.g. nicotine, sodium and propylene glycol) quantities (25.1 ng to 35.3 mg per 1 g pouch), the mean percentage extracted varied only from 19.2% for linalyl acetate to 37.8% for the TSNA 4-(methylnitrosamino)-1-(3-pyridyl)-1-butanone (NNK) among all constituents analyzed. The TSNAs, some of which are known carcinogens, showed the highest percentage extraction (range 34.6%–37.8%). Measurement variability was low for all analytes, ranging from 2.4% (total TSNAs, NAT) to 9.5% (geraniol). By contrast, inter-subject variability ranged from 6.7% (NAB) to 52.2% (linalyl acetate), and was greater than 20% for eight of the constituents analyzed. Intra-subject variability ranged from 3.4% (citronellol) to 29.7% (geraniol). Conclusions Generally, less than a third of each constituent tested was extracted during one hour of snus use, independent of constituent concentration. The variable nature of in-use extraction was shown to be driven by inter

  19. Noncontact point spectroscopy guided by two-channel fluorescence imaging in a hamster cheek pouch model

    NASA Astrophysics Data System (ADS)

    Yang, Victor X.; Yeow, Jenny; Lilge, Lothar D.; Kost, James; Mang, Thomas S.; Wilson, Brian C.

    1999-07-01

    A system for in vivo, fluorescence image-guided, non-contact point fluorescence spectroscopy is presented. A 442 nm HeCd laser is used as the fluorescence excitation source. An intensified CCD serves as the detector for both imaging and spectroscopy, on which two regions of 300 X 300 pixels were used for green (500 +/- 18 nm) and red (630 +/- 18 nm) imaging channels, and a strip of 600 X 120 pixels are used for emission spectroscopy (450 - 750 nm). At a working distance of 40 mm, the system has a spatial resolution of 0.16 mm and a spectral resolution of 5 nm. System performance is demonstrated in a carcinogenesis model in hamsters, where tumors were induced by painting DMBA in the cheek pouch. Autofluorescence and Photofrin-induced fluorescence measurements were performed every 2 weeks during the 18 weeks of tumor induction. Punch biopsies on selected animals were taken for histological staging. The results show that autofluorescence fluorescence can distinguish dysplasia from normal mucosal tissue model, utilizing the peak red intensity (or the red-to-green intensity ratio). Photofrin-induced fluorescence was superior to autofluorescence for differentiating high grade dysplasia from invasive cancer.

  20. Effect of 3-methoxybenzamide on the induction and development of hamster-pouch tumors

    SciTech Connect

    Miller, E.G.; Rivera-Hidalgo, F.; Binnie, W.H.

    1987-01-01

    Data from this laboratory and others has suggested that inhibitors of poly(ADP-ribose) polymerase might be able to augment the action of chemical carcinogens. The purpose of this study was to see if one of these inhibitors, 3-methoxybenzamide (MBA), could enhance the carcinogenic effects of 7,12-dimethylbenz(a)anthracene (DMBA). Thirty-two female Syrian hamsters were divided into two equal experimental groups. The left buccal pouches of the animals in Group I were painted three times weekly, first with a solution of dimethylsulfoxide (DMSO) and then with a 0.5% solution of DMBA in mineral oil. In Group II the basic treatment was the same except that the DMSO contained MBA (2.5%). After a total of 50 treatments (16 1/2 weeks), the animals were sacrificed and autopsied. The data indicated that the hamsters in Group II had approximately twice as many tumors as the animals in Group I. The average size of the tumors in the two groups was essentially the same. Using the two-stage mechanism for tumorigenesis it would appear the MBA affected the initiating phase of DMBA-induced carcinogenesis.

  1. The flavonoid quercetin modulates the hallmark capabilities of hamster buccal pouch tumors.

    PubMed

    Priyadarsini, Ramamurthi Vidya; Vinothini, Govindarajah; Murugan, Ramalingam Senthil; Manikandan, Palrasu; Nagini, Siddavaram

    2011-01-01

    Epidemiological studies have consistently demonstrated the protective effects of dietary phytochemicals against cancer risk. Quercetin, a ubiquitous dietary flavonoid, has attracted considerable attention owing to its potent antioxidant and antiproliferative activities. The present study was designed to investigate the chemopreventive as well as the therapeutic ability of quercetin to modulate the key hallmark capabilities of 7,12-dimethylbenz[a]anthracene (DMBA)-induced hamster buccal pouch (HBP) carcinomas. We analyzed the expression of markers associated with cell proliferation and survival (PCNA, p21, p53, cyclin D1, GST-P), apoptosis (Fas, Fas-L, Bcl-2 family proteins, cytochrome-C, Apaf-1, caspases, PARP, survivin, cFLIP, API1), invasion (MMPs, TIMP-2, RECK), angiogenesis (PlGF, VEGF, VEGF receptors, HIF-1α), as well as the epigenetic markers (HDAC-1, DNMT1) by immunohistochemical, Western blot, and RT-PCR analyses. Simultaneous administration of quercetin to DMBA-painted hamsters reduced tumor incidence and tumor burden, while posttreatment of quercetin resulted in a significant tumor growth delay. In addition, quercetin administration induced cell cycle arrest and apoptosis and blocked invasion and angiogenesis. We found a positive correlation between the inhibition of HDAC-1 and DNMT1 by quercetin and its anticancer properties. A dietary phytochemical such as quercetin that modulates a plethora of molecules offers promise as an ideal candidate for multitargeted cancer prevention and therapy. PMID:21294050

  2. Heat penetration characteristics and physico-chemical properties of in-pouch processed dairy dessert (kheer).

    PubMed

    Jha, Alok; Patel, A A; Gopal, T K Srinivasa; Ravishankar, C N

    2014-10-01

    Kheer, a traditional milk product of South East Asia, containing cooked rice grains in a creamy sweetened concentrated milk, has no large-scale production due to its poor shelf life. Shelf-life was improved by development of a process based on in-pouch thermal processing employing a rotary retort. Product development included optimization of rice-to-milk solids ratio (0.18-0.52) and total milk solids levels (16-26 %) to simulate the conventional product in taste, appearance and textural attributes. Various Fo values (12.4-14.8) were examined with regard to product quality. While the TBA value tended to increase (0.073-0.081) the reflectance value (35.3-43.4) declined with increasing Fo. The pH of the product (6.04-6.10) showed a slight tendency to increase with Fo. Sensorily, the product was found to be acceptable for a period of 150 days at 37 °C. PMID:25328196

  3. Carcinogenic effects of MGP-7 and B[a]P on the hamster cheek pouch.

    PubMed

    Brandon, Jimi Lynn; Conti, Claudio J; Goldstein, Lawrence S; DiGiovanni, John; Gimenez-Conti, Irma B

    2009-10-01

    This study was performed to examine the carcinogenic effects of benzo[a]pyrene (B[a]P) and manufactured gas plant (MGP) residues on the hamster cheek pouch (HCP). Syrian hamsters were treated topically with a suspension of 2%, 10%, or 20% B[a]P or 50% or 100% MGP-7 (a mixture of residues from 7 MGP sites) in mineral oil for eight (short-term study) and sixteen, twenty, twenty-eight, and thirty-two weeks (long-term study). The short-term study showed that B[a]P induced p53 protein accumulation, indicative of genotoxic damage, as well as increased cell proliferation, hyperplasia, and inflammation, which is usually associated with promotional activity. In contrast, the MGP-7 presented only marginal p53 accumulation and induction of BrdU incorporation. In the long-term experiments, animals treated with 2% and 10% of B[a]P continued to show p53 protein accumulation as well as hyperplasia and increased cell proliferation and inflammation. By thirty weeks, all the animals treated with B[a]P had a 100% incidence of squamous cell carcinoma (SCC). Animals treated with 50% and 100% MGP-7 showed only weak hyperplasia and a low proliferation rate and accumulation of p53 protein through thirty-two weeks. Benzo[a]pyrene was highly carcinogenic when used at adequate doses. Manufactured gas plant residue, however, was not carcinogenic in this model. PMID:19679887

  4. Classifying sex biased congenital anomalies

    SciTech Connect

    Lubinsky, M.S.

    1997-03-31

    The reasons for sex biases in congenital anomalies that arise before structural or hormonal dimorphisms are established has long been unclear. A review of such disorders shows that patterning and tissue anomalies are female biased, and structural findings are more common in males. This suggests different gender dependent susceptibilities to developmental disturbances, with female vulnerabilities focused on early blastogenesis/determination, while males are more likely to involve later organogenesis/morphogenesis. A dual origin for some anomalies explains paradoxical reductions of sex biases with greater severity (i.e., multiple rather than single malformations), presumably as more severe events increase the involvement of an otherwise minor process with opposite biases to those of the primary mechanism. The cause for these sex differences is unknown, but early dimorphisms, such as differences in growth or presence of H-Y antigen, may be responsible. This model provides a useful rationale for understanding and classifying sex-biased congenital anomalies. 42 refs., 7 tabs.

  5. [Congenital myasthenic syndromes; French experience].

    PubMed

    Eymard, Bruno; Hantaï, Daniel; Fournier, Emmanuel; Nicole, Sophie; Sternberg, Damien; Richard, Pascale; Fardeau, Michel

    2014-02-01

    Congenital myasthenic syndromes CMS) form a heterogeneous group of genetic diseases characterized by abnormal neuromuscular transmission. The associated muscular weakness is exacerbated by exertion and usually starts during infancy/childhood In 2002 a national Congenital Myasthenic Syndromes Network was created in France, composed of neurologists, neuropediatricians, pathologists, molecular geneticists and neurobiologists. The network has now identified nearly 300 cases of CMS, as well as three new culprit genes. Based on our personal experience and data from the most recent studies, we describe the 18 principal culprit genes so far identified, along with diagnostic pitfalls, the disease course, prognosis and treatment. The underlying genetic defect remains to be identified in nearly half of CMS patients. PMID:26263703

  6. Surgical correction of congenital megaprepuce.

    PubMed

    Shenoy, M U; Rance, C H

    1999-01-01

    Congenital megaprepuce (CMP) is a rare entity. Two infant boys presented with a tight congenital phimosis resulting in an excessively baggy, urine-filled prepuce and a swollen scrotum. Compression of the scrotum resulted in drainage of urine. We feel this to be a separate entity from a buried penis and recommend early surgery. The phimotic tip of the foreskin was excised and the inner layer preserved to cover the full length of the penile shaft. The outer layer of the foreskin, in reality the penoscrotal junction, was anchored to the base of the penile shaft. A V-shaped edge of ventral skin was excised and the edges approximated, giving the appearance of median raphe. The final appearance was that of a circumcised penis. A third patient awaits operation. PMID:10631746

  7. [Radiological evaluation of congenital tumors].

    PubMed

    Aguado del Hoyo, A; Ruiz Martín, Y; Lancharro Zapata, Á; Marín Rodríguez, C; Gordillo Gutiérrez, I

    2015-01-01

    In this article, we consider tumors that are diagnosed during pregnancy or in the first three months of life. This is a heterogeneous group of neoplasms with special biological and epidemiological characteristics that differentiate them from tumors arising in children or adults. In the last two decades, the prenatal detection of congenital tumors has increased due to the generalized use of prenatal sonographic screening. Advances in imaging techniques, especially in fetal magnetic resonance imaging, have enabled improvements in the diagnosis, follow-up, clinical management, and perinatal treatment of these tumors. This image-based review of the most common congenital tumors describes their histologic types, locations, and characteristics on the different imaging techniques used. PMID:26115799

  8. Congenital Anomalies of the Nose.

    PubMed

    Funamura, Jamie L; Tollefson, Travis T

    2016-04-01

    Congenital anomalies of the nose range from complete aplasia of the nose to duplications and nasal masses. Nasal development is the result of a complex embryologic patterning and fusion of multiple primordial structures. Loss of signaling proteins or failure of migration or proliferation can result in structural anomalies with significant cosmetic and functional consequences. Congenital anomalies of the nose can be categorized into four broad categories: (1) aplastic or hypoplastic, (2) hyperplastic or duplications, (3) clefts, and (4) nasal masses. Our knowledge of the embryologic origin of these anomalies helps dictate subsequent work-up for associated conditions, and the appropriate treatment or surgical approach to manage newborns and children with these anomalies. PMID:27097134

  9. [Congenital ranula in a newborn].

    PubMed

    Bernhard, M K; Hückel, D; Hamala, D

    2007-05-01

    Ranulas are cystic lesions in the floor of the mouth. They are either retention cysts of the excretory duct of the sublingual gland or pseudocysts formed by excretory duct rupture followed by extravasation and accumulation of mucus in the surrounding tissue. We report the case of a premature newborn with a congenital ranula in the floor of mouth. The ranula caused no discomfort or complications, so that immediate intervention was not necessary. The cyst resolved completely by the age of 4 months. Complications in newborns especially include airway obstruction and feeding difficulties. Surgical treatment options are needle aspiration, excision of the ranula, marsupialization, cryosurgery, and--in addition to excision of the cyst--removal of the ipsilateral sublingual gland. Sclerotherapy has shown good results as well. As many congenital cysts resolve or rupture spontaneously, they should be observed for potential resolution for several months in uncomplicated cases. PMID:16770600

  10. Are parenchymal AVMs congenital lesions?

    PubMed

    Morales-Valero, Saul F; Bortolotti, Carlo; Sturiale, Carmelo; Sturiale, Carmelo L; Lanzino, Giuseppe

    2014-09-01

    A long-held dogma in neurosurgery is that parenchymal arteriovenous malformations (AVMs) are congenital. However, there is no strong evidence supporting this theory. An increasing number of documented cases of de novo formation of parenchymal AVMs cast doubt on their congenital nature and suggest that indeed the majority of these lesions may form after birth. Further evidence suggesting the postnatal development of parenchymal AVMs comes from the exceedingly rare diagnosis of these lesions in utero despite the widespread availability of high-resolution imaging modalities such as ultrasound and fetal MRI. The exact mechanism of AVM formation has yet to be elucidated, but most likely involves genetic susceptibility and environmental triggering factors. In this review, the authors report 2 cases of de novo AVM formation and analyze the evidence suggesting that they represent an acquired condition. PMID:25175439

  11. Colonization, mouse-style

    PubMed Central

    2010-01-01

    Several recent papers, including one in BMC Evolutionary Biology, examine the colonization history of house mice. As well as background for the analysis of mouse adaptation, such studies offer a perspective on the history of movements of the humans that accidentally transported the mice. See research article: http://www.biomedcentral.com/1471-2148/10/325 PMID:20977781

  12. Serotonin and colonic motility.

    PubMed

    Kendig, D M; Grider, J R

    2015-07-01

    The role of serotonin (5-hydroxytryptamine [5-HT]) in gastrointestinal motility has been studied for over 50 years. Most of the 5-HT in the body resides in the gut wall, where it is located in subsets of mucosal cells (enterochromaffin cells) and neurons (descending interneurons). Many studies suggest that 5-HT is important to normal and dysfunctional gut motility and drugs affecting 5-HT receptors, especially 5-HT3 and 5-HT4 receptors, have been used clinically to treat motility disorders; however, cardiovascular side effects have limited the use of these drugs. Recently studies have questioned the importance and necessity of 5-HT in general and mucosal 5-HT in particular for colonic motility. Recent evidence suggests the importance of 5-HT3 and 5-HT4 receptors for initiation and generation of one of the key colonic motility patterns, the colonic migrating motor complex (CMMC), in rat. The findings suggest that 5-HT3 and 5-HT4 receptors are differentially involved in two different types of rat CMMCs: the long distance contraction (LDC) and the rhythmic propulsive motor complex (RPMC). The understanding of the role of serotonin in colonic motility has been influenced by the specific motility pattern(s) studied, the stimulus used to initiate the motility (spontaneous vs induced), and the route of administration of drugs. All of these considerations contribute to the understanding and the controversy that continues to surround the role of serotonin in the gut. PMID:26095115

  13. Colonic interposition: radiographic evaluation.

    PubMed

    Agha, F P; Orringer, M B

    1984-04-01

    This report reviews the clinical and radiographic features of 40 patients who underwent visceral esophageal substitution with colon for benign or malignant lesions of the esophagus. The incidence and radiographic identification of complications are discussed. All patients were routinely examined with barium esophagrams on postoperative day 10. If an anastomotic leak was suspected clinically before this time, studies were performed using water-soluble iodinated contrast material. Follow-up barium esophagrams were obtained 1-96 months after operation (average, 60 months) in 24 patients. Eight patients (21%) demonstrated asymptomatic "jejunization" of the colonic mucosa with no attributable clinical manifestations; this finding resolved in 1-3 months, without sequelae, and has not been reported before. The spectrum of ischemic changes in the colonic segment included mucosal edema, spasm, ulceration, loss of haustration, and frank necrosis. Radiographically detectable early postoperative complications included anastomotic leak in six (three pharyngocolic, three cervical esophagocolic) and aspiration of barium into the tracheobronchial tree due to incoordinated swallowing in eight. Late postoperative complications included anastomotic narrowing (12) malfunctioning of the colon due to impaired emptying (five), recurrent aspiration pneumonia (three), small bowel obstruction (three), transhiatal herniation of small bowel through the diaphragmatic hiatus (one), and reflux into the retained bypassed esophagus (one). PMID:6608225

  14. Congenital scoliosis – Quo vadis?

    PubMed Central

    Debnath, Ujjwal K; Goel, Vivek; Harshavardhana, Nanjanduppa; Webb, John K

    2010-01-01

    Congenital spinal vertebral anomalies can present as scoliosis or kyphosis or both. The worldwide prevalence of the vertebral anomalies is 0.5-1 per 1000 live births. Vertebral anomalies can range from hemi vertebrae (HV) which may be single or multiple, vertebral bar with or without HV, block vertebrae, wedge shaped or butterfly vertebrae. Seventy per cent of congenital vertebral anomalies result in progressive deformities. The risk factors for progression include: type of defect, site of defect (junctional regions) and patient's age at the time of diagnosis. The key to success in managing these spinal deformities is early diagnosis and anticipation of progression. One must intervene surgically to halt the progression of deformity and prevent further complications associated with progressive deformity. Planning for surgery includes a preoperative MRI scan to rule out spinal anomalies such as diastematomyelia. The goals of surgical treatment for congenital spinal deformity are to achieve a straight growing spine, a normal standing sagittal profile, and a short fusion segment. The options of surgery include in situ fusion, convex hemi epiphysiodesis and hemi vertebra excision. These basic surgical procedures can be combined with curve correction, instrumentation and short segment fusion. Most surgeons prefer posterior (only) surgery for uncomplicated HV excision and short segment fusion. These surgical procedures can be performed through posterior, anterior or combined approaches. The advocates of combined approaches suggest greater deformity correction possibilities with reduced incidence of pseudoarthrosis and minimize crankshaft phenomenon. We recommend posterior surgery for curves involving only an element of kyphosis or modest deformity, whereas combined anterior and posterior approach is indicated for large or lordotic deformities. In the last decade, the use of growing rods and vertebral expandable prosthetic titanium rib has improved the armamentarium of the

  15. Congenital malformations in diabetic offspring.

    PubMed

    Temesio, P; Belitzky, R; Gallego, L; Martell, M; Pose, S V

    1977-01-01

    A retrospective study of 215 deliveries in diabetic mothers at Hospital de Clínicas (Montevideo, Uruguay) has been performed. Presence of congenital malformations (CM) was considered in relation to age of the mothers, class of maternal diabetes, maternal angiopathy, treatment and metabolic control. The prevalence of CM in the series was 9.8%. None of the factors analyzed seems to be statistically linked to the prevalence of CM. PMID:613685

  16. Congenital Portosystemic Shunt: Our Experience

    PubMed Central

    Timpanaro, Tiziana; Passanisi, Stefano; Sauna, Alessandra; Trombatore, Claudia; Pennisi, Monica; Petrillo, Giuseppe; Smilari, Pierluigi; Greco, Filippo

    2015-01-01

    Introduction. Congenital portosystemic venous malformations are rare abnormalities in which the portal blood drains into a systemic vein and which are characterized by extreme clinical variability. Case Presentations. The authors present two case reports of a congenital extrahepatic portosystemic shunt (Type II). In the first patient, apparently nonspecific symptoms, such as headache and fatigue, proved to be secondary to hypoglycemic episodes related to the presence of a portosystemic shunt, later confirmed on imaging. During portal vein angiography, endovascular embolization of the portocaval fistula achieved occlusion of the anomalous venous tract. In the second patient, affected by Down's syndrome, the diagnosis of a portosystemic malformation was made by routine ultrasonography, performed to rule out concurrent congenital anomalies. Because of the absence of symptoms, we chose to observe this patient. Conclusions. These two case reports demonstrate the clinical heterogeneity of this malformation and the need for a multidisciplinary approach. As part of a proper workup, clinical evaluation must always be followed by radiographic diagnosis. PMID:25709849

  17. Streptococcus Adherence and Colonization

    PubMed Central

    Nobbs, Angela H.; Lamont, Richard J.; Jenkinson, Howard F.

    2009-01-01

    Summary: Streptococci readily colonize mucosal tissues in the nasopharynx; the respiratory, gastrointestinal, and genitourinary tracts; and the skin. Each ecological niche presents a series of challenges to successful colonization with which streptococci have to contend. Some species exist in equilibrium with their host, neither stimulating nor submitting to immune defenses mounted against them. Most are either opportunistic or true pathogens responsible for diseases such as pharyngitis, tooth decay, necrotizing fasciitis, infective endocarditis, and meningitis. Part of the success of streptococci as colonizers is attributable to the spectrum of proteins expressed on their surfaces. Adhesins enable interactions with salivary, serum, and extracellular matrix components; host cells; and other microbes. This is the essential first step to colonization, the development of complex communities, and possible invasion of host tissues. The majority of streptococcal adhesins are anchored to the cell wall via a C-terminal LPxTz motif. Other proteins may be surface anchored through N-terminal lipid modifications, while the mechanism of cell wall associations for others remains unclear. Collectively, these surface-bound proteins provide Streptococcus species with a “coat of many colors,” enabling multiple intimate contacts and interplays between the bacterial cell and the host. In vitro and in vivo studies have demonstrated direct roles for many streptococcal adhesins as colonization or virulence factors, making them attractive targets for therapeutic and preventive strategies against streptococcal infections. There is, therefore, much focus on applying increasingly advanced molecular techniques to determine the precise structures and functions of these proteins, and their regulatory pathways, so that more targeted approaches can be developed. PMID:19721085

  18. Use of a laparoscopic specimen retrieval pouch to facilitate removal of intact or fragmented cystic calculi from standing sedated horses: 8 cases (2012-2015).

    PubMed

    Katzman, Scott A; Vaughan, Betsy; Nieto, Jorge E; Galuppo, Larry D

    2016-08-01

    OBJECTIVE To evaluate the use of a laparoscopic specimen retrieval pouch for removal of intact or fragmented cystic calculi from standing horses. DESIGN Retrospective case series. ANIMALS 8 horses (5 geldings and 3 mares) with cystic calculi. PROCEDURES Physical examination and cystoscopic, ultrasonographic, and hematologic evaluations of urinary tract function were performed for each horse. A diagnosis of cystic calculus was made on the basis of results of cystoscopy and ultrasonography. Concurrent urolithiasis or other urinary tract abnormalities identified during preoperative evaluation were recorded. Horses were sedated and placed in standing stocks, and the perineum was aseptically prepared. Direct access to the urinary bladder was gained in geldings via perineal urethrotomy or in mares by a transurethral approach. Calculi were visualized endoscopically, manipulated into the retrieval pouch, and removed intact or fragmented (for larger calculi). RESULTS For 4 geldings and 1 mare, fragmentation was necessary to facilitate calculus removal. Mean duration of surgery was 125 minutes, and trauma to the urinary bladder and urethra was limited to areas of hyperemia and submucosal petechiation. No postoperative complications were encountered for any horse. When lithotripsy was required, the retrieval pouch provided an effective means of stabilizing calculi and containing the fragments for removal. CONCLUSIONS AND CLINICAL RELEVANCE Use of the laparoscopic specimen retrieval pouch was an effective, minimally traumatic method for retrieving cystic calculi from standing horses. The pouch protected the urinary bladder and urethra from trauma during calculus removal and allowed for stabilization, containment, and fragmentation of calculi when necessary. PMID:27439348

  19. Redo Ileal pouch-anal anastomosis combined with anti-TNF-α maintenance therapy for Crohn's disease with pelvic fistula: report of two cases.

    PubMed

    Araki, Toshimitsu; Okita, Yoshiki; Fujikawa, Hiroyuki; Ohi, Masaki; Tanaka, Koji; Inoue, Yasuhiro; Uchida, Keiichi; Mohri, Yasuhiko; Kusunoki, Masato

    2014-10-01

    Pouch failure has been reported to occur after ileal pouch-anal anastomosis for Crohn's disease. We report two cases of patients with Crohn's disease, who underwent redo ileal pouch-anal anastomosis (redo-IPAA) combined with anti-TNF-α maintenance therapy, with good functional results. The first patient, a man with presumed ulcerative colitis, suffered pelvic fistula recurrence and anastomotic dehiscence. He underwent redo-IPAA, at which time longitudinal ulcers were found. Infliximab was started 4 days postoperatively and continued. The second patient, a woman treated for ulcerative colitis, underwent laparoscopic IPAA 8 years later. After the development of a pelvic fistula, twisted mesentery of the ileal pouch was found intraoperatively and Crohn's disease was diagnosed. Adalimumab therapy resulted in fistula closure. Redo-IPAA was performed to normalize the twisted mesentery of the ileal pouch. No complications have been observed in either patient, both of whom have experienced good functional results after closure of the covering stomas. PMID:24442570

  20. Modulating Effect of Enicostemma littorale on the Expression Pattern of Apoptotic, Cell Proliferative, Inflammatory and Angiogenic Markers During 7, 12-Dimethylbenz (a) Anthracene Induced Hamster Buccal Pouch Carcinogenesis

    PubMed Central

    Manoharan, Shanmugam; Rajasekaran, Duraisamy; Prabhakar, Murugaraj Manoj; Karthikeyan, Sekar; Manimaran, Asokan

    2015-01-01

    Enicostemma littorale leaves are traditionally used for the treatment of several diseases, including inflammation and cancer. This study has taken effort to explore the antitumor initiating potential of E. littorale leaves (ElELet) by analyzing the expression pattern of apoptotic (p53, Bcl-2 and Bcl-2 associated X-protein), cell-proliferative (cyclin D1 and proliferating cell nuclear antigen), angiogenic (vascular endothelial growth factor), invasive (matrix metalloproteinase-2 and 9), and inflammatory (NF-κB and cyclooxygenase-2) markers during 7, 12-dimethylbenz (a) anthracene (DMBA) induced hamster buccal pouch carcinogenesis. Oral tumors were induced in the buccal pouches of hamsters using the potent site and organ specific carcinogen, DMBA. DMBA application 3 times a week for 14 weeks resulted in tumor formation in the buccal pouches. Hundred percent tumor formations with dysregulation in the expression pattern of apoptotic, cell proliferative, inflammatory, angiogenic, and invasive markers were observed in the buccal pouches of hamsters treated with DMBA alone. ElELet at a dose of 250 mg/kg body weight orally to DMBA treated hamsters significantly prevented the tumor formation as well as corrected the abnormalities in the expression pattern of above mentioned molecular markers. ElELet thus modulated the expression pattern of all the above mentioned molecular markers in favor of the suppression of cell proliferation occurring in DMBA induced hamster buccal pouch carcinogenesis. PMID:26862274

  1. Modulating Effect of Enicostemma littorale on the Expression Pattern of Apoptotic, Cell Proliferative, Inflammatory and Angiogenic Markers During 7, 12-Dimethylbenz (a) Anthracene Induced Hamster Buccal Pouch Carcinogenesis.

    PubMed

    Manoharan, Shanmugam; Rajasekaran, Duraisamy; Prabhakar, Murugaraj Manoj; Karthikeyan, Sekar; Manimaran, Asokan

    2015-01-01

    Enicostemma littorale leaves are traditionally used for the treatment of several diseases, including inflammation and cancer. This study has taken effort to explore the antitumor initiating potential of E. littorale leaves (ElELet) by analyzing the expression pattern of apoptotic (p53, Bcl-2 and Bcl-2 associated X-protein), cell-proliferative (cyclin D1 and proliferating cell nuclear antigen), angiogenic (vascular endothelial growth factor), invasive (matrix metalloproteinase-2 and 9), and inflammatory (NF-κB and cyclooxygenase-2) markers during 7, 12-dimethylbenz (a) anthracene (DMBA) induced hamster buccal pouch carcinogenesis. Oral tumors were induced in the buccal pouches of hamsters using the potent site and organ specific carcinogen, DMBA. DMBA application 3 times a week for 14 weeks resulted in tumor formation in the buccal pouches. Hundred percent tumor formations with dysregulation in the expression pattern of apoptotic, cell proliferative, inflammatory, angiogenic, and invasive markers were observed in the buccal pouches of hamsters treated with DMBA alone. ElELet at a dose of 250 mg/kg body weight orally to DMBA treated hamsters significantly prevented the tumor formation as well as corrected the abnormalities in the expression pattern of above mentioned molecular markers. ElELet thus modulated the expression pattern of all the above mentioned molecular markers in favor of the suppression of cell proliferation occurring in DMBA induced hamster buccal pouch carcinogenesis. PMID:26862274

  2. Development of functional canned and pouched tuna products added inulin for commercial production.

    PubMed

    Rueangwatcharin, U; Wichienchot, S

    2015-08-01

    Four formulas of canned tuna in spring water and tuna in mayonnaise and pouched tuna in salad cream and tuna in thousand island cream with added inulin were developed for commercial production. The effects of the addition of a prebiotic (inulin, Orafti®-HP) on the color and sensory properties of these products were studied. For inulin concentrations studied (3, 5, 7 and 10 %, w/w) caused more intensed yellow and red colors. Hedonic sensory values of tuna packed in spring water and in mayonnaise showed no significant differences (p > 0.05) among products with different inulin addition levels (3, 5, 7 and 10 %, w/w) in terms of color, flavor, sweetness and overall characteristics. However, when packed in thousand island cream, significant differences (p < 0.05) in color and overall characteristics were found when inulin was added higher than 7 %. Tuna in salad cream showed significant differences (p < 0.05) in color, flavor, sweetness and overall characteristics at higher than 7 % inulin. The panelists showed acceptable overall liking scores at upto 7 % inulin of all tuna products. The thermal sterilization process resulted in approximately 20 % decrease in final inulin content. The calculated residual fructans of finished products at shelf life of 3 years were 3.01, 2.78, 2.90 and 2.84 % for tuna in spring water, tuna in mayonnaise, tuna in thousand island and tuna in salad cream, respectively. Considering formula cost in a commercial production and the recommended daily intake (RDI) of inulin in the finished product at end of shelf life (≥3 g/d), an addition of 5 % inulin for tuna in spring water and 7 % inulin for tuna in mayonnaise, tuna in thousand island and tuna in salad cream are recommended. PMID:26243930

  3. fras1 shapes endodermal pouch 1 and stabilizes zebrafish pharyngeal skeletal development

    PubMed Central

    Talbot, Jared Coffin; Walker, Macie B.; Carney, Thomas J.; Huycke, Tyler R.; Yan, Yi-Lin; BreMiller, Ruth A.; Gai, Linda; DeLaurier, April; Postlethwait, John H.; Hammerschmidt, Matthias; Kimmel, Charles B.

    2012-01-01

    Lesions in the epithelially expressed human gene FRAS1 cause Fraser syndrome, a complex disease with variable symptoms, including facial deformities and conductive hearing loss. The developmental basis of facial defects in Fraser syndrome has not been elucidated. Here we show that zebrafish fras1 mutants exhibit defects in facial epithelia and facial skeleton. Specifically, fras1 mutants fail to generate a late-forming portion of pharyngeal pouch 1 (termed late-p1) and skeletal elements adjacent to late-p1 are disrupted. Transplantation studies indicate that fras1 acts in endoderm to ensure normal morphology of both skeleton and endoderm, consistent with well-established epithelial expression of fras1. Late-p1 formation is concurrent with facial skeletal morphogenesis, and some skeletal defects in fras1 mutants arise during late-p1 morphogenesis, indicating a temporal connection between late-p1 and skeletal morphogenesis. Furthermore, fras1 mutants often show prominent second arch skeletal fusions through space occupied by late-p1 in wild type. Whereas every fras1 mutant shows defects in late-p1 formation, skeletal defects are less penetrant and often vary in severity, even between the left and right sides of the same individual. We interpret the fluctuating asymmetry in fras1 mutant skeleton and the changes in fras1 mutant skeletal defects through time as indicators that skeletal formation is destabilized. We propose a model wherein fras1 prompts late-p1 formation and thereby stabilizes skeletal formation during zebrafish facial development. Similar mechanisms of stochastic developmental instability might also account for the high phenotypic variation observed in human FRAS1 patients. PMID:22782724

  4. Gastric pouches and the mucociliary sole: setting the stage for nervous system evolution.

    PubMed

    Arendt, Detlev; Benito-Gutierrez, Elia; Brunet, Thibaut; Marlow, Heather

    2015-12-19

    Prerequisite for tracing nervous system evolution is understanding of the body plan, feeding behaviour and locomotion of the first animals in which neurons evolved. Here, a comprehensive scenario is presented for the diversification of cell types in early metazoans, which enhanced feeding efficiency and led to the emergence of larger animals that were able to move. Starting from cup-shaped, gastraea-like animals with outer and inner choanoflagellate-like cells, two major innovations are discussed that set the stage for nervous system evolution. First, the invention of a mucociliary sole entailed a switch from intra- to extracellular digestion and increased the concentration of nutrients flowing into the gastric cavity. In these animals, an initial nerve net may have evolved via division of labour from mechanosensory-contractile cells in the lateral body wall, enabling coordinated movement of the growing body that involved both mucociliary creeping and changes of body shape. Second, the inner surface of the animals folded into metameric series of gastric pouches, which optimized nutrient resorption and allowed larger body sizes. The concomitant acquisition of bilateral symmetry may have allowed more directed locomotion and, with more demanding coordinative tasks, triggered the evolution of specialized nervous subsystems. Animals of this organizational state would have resembled Ediacarian fossils such as Dickinsonia and may have been close to the cnidarian-bilaterian ancestor. In the bilaterian lineage, the mucociliary sole was used mostly for creeping, or frequently lost. One possible remnant is the enigmatic Reissner's fibre in the ventral neural tube of cephalochordates and vertebrates. PMID:26554050

  5. Etanercept administration prevents the inflammatory response induced by carrageenan in the murine air pouch model.

    PubMed

    Mattei, Rodrigo Antônio; Dalmarco, Eduardo Monguilhott; Fröde, Tânia Silvia

    2015-12-01

    Rheumatoid arthritis (RA) is one of several inflammatory and autoimmune diseases that affect approximately 1% of world's population. The development of TNF inhibitors in the last decade represents a great advance in the treatment of mild and severe forms of RA. Etanercept is one of these drugs that is useful for RA treatment, but the mechanism of inhibition of the signaling pathway of inflammation was not completely elucidated. This study was conducted to evaluate the anti-inflammatory effect of etanercept in comparison to reference drugs (dexamethasone and indomethacin). Inflammation was induced by subcutaneal administration of carrageenan in the Swiss albino mice using the murine air pouch model. Exudation; leukocytes; myeloperoxidase (MPO); adenosine deaminase (ADA); nitric oxide metabolites (NOx); tumor necrosis factor (TNF); interferon gamma (IFN-γ); interleukins (IL) IL-6, IL-17, IL-10, IL-4, and IL-2; nuclear transcription factor kappa B (NF-κB) activation and apoptosis were evaluated 24 h after the induction of inflammation. Treatment with etanercept significantly inhibited exudate concentrations; leukocyte count; MPO and ADA activities; NOx, TNF, IFN-γ, and IL-17 levels; and NF-kappa B activation (p < 0.05). Etanercept induced apoptosis, reducing the number of viable neutrophils without increasing necrosis (p < 0.05). Our results suggest that the anti-inflammatory mechanism of action of etanercept may be via decrease of NF-κB activation. This effect promoted the reduction of pro-inflammatory cytokines and NOx and the induction of neutrophil apoptosis. The effect of etanercept upon neutrophils apoptosis may indicate the use of this drug therapy in the early stage of rheumatoid arthritis disease. PMID:26255064

  6. Gastric pouches and the mucociliary sole: setting the stage for nervous system evolution

    PubMed Central

    Arendt, Detlev; Benito-Gutierrez, Elia; Brunet, Thibaut; Marlow, Heather

    2015-01-01

    Prerequisite for tracing nervous system evolution is understanding of the body plan, feeding behaviour and locomotion of the first animals in which neurons evolved. Here, a comprehensive scenario is presented for the diversification of cell types in early metazoans, which enhanced feeding efficiency and led to the emergence of larger animals that were able to move. Starting from cup-shaped, gastraea-like animals with outer and inner choanoflagellate-like cells, two major innovations are discussed that set the stage for nervous system evolution. First, the invention of a mucociliary sole entailed a switch from intra- to extracellular digestion and increased the concentration of nutrients flowing into the gastric cavity. In these animals, an initial nerve net may have evolved via division of labour from mechanosensory-contractile cells in the lateral body wall, enabling coordinated movement of the growing body that involved both mucociliary creeping and changes of body shape. Second, the inner surface of the animals folded into metameric series of gastric pouches, which optimized nutrient resorption and allowed larger body sizes. The concomitant acquisition of bilateral symmetry may have allowed more directed locomotion and, with more demanding coordinative tasks, triggered the evolution of specialized nervous subsystems. Animals of this organizational state would have resembled Ediacarian fossils such as Dickinsonia and may have been close to the cnidarian–bilaterian ancestor. In the bilaterian lineage, the mucociliary sole was used mostly for creeping, or frequently lost. One possible remnant is the enigmatic Reissner's fibre in the ventral neural tube of cephalochordates and vertebrates. PMID:26554050

  7. Congenital talipes equinovarus associated with hereditary congenital common peroneal nerve neuropathy: a literature review.

    PubMed

    Matar, Hosam E; Garg, Neeraj K

    2016-03-01

    We present a unique case of a congenital hereditary common peroneal nerve neuropathy with congenital idiopathic congenital talipes equinovarus that had been treated with the Ponseti method with satisfactory outcome at 5-year follow-up, along with a literature review. PMID:26588839

  8. Comparative evaluation of antiproliferative, antiangiogenic and apoptosis inducing potential of black tea polyphenols in the hamster buccal pouch carcinogenesis model

    PubMed Central

    Letchoumy, Paramasivame Vidjaya; Mohan, Kurapathy Venkata Poorna Chandra; Prathiba, Duvuru; Hara, Yukihiko; Nagini, Siddavaram

    2007-01-01

    Background To evaluate the relative chemopreventive efficacy of two black tea polyphenols, Polyphenon-B [P-B] and BTF-35 on 7,12-dimethylbenz [a]anthracene (DMBA)-induced hamster buccal pouch (HBP) carcinogenesis. Methods Hamsters were divided into 6 groups. The right buccal pouches of animals in groups 1–3 were painted with 0.5% of DMBA three times a week for 14 weeks. While hamsters in group 1 received no further treatment, animals in groups 2 and 3 received diet containing 0.05% P-B and BTF-35 respectively, four weeks before DMBA painting that was continued until the end of the experiments. Animals in groups 4 and 5 were given P-B and BTF-35 alone respectively as in groups 2 and 3. Group 6 animals served as the untreated control. All the animals were sacrificed after 18 weeks. The expression of p21, cyclin D1, glutathione S-transferase pi (GST-P), nuclear factor kappa B (NF-κB), Bcl-2, Bax, cytochrome C, caspase-3, caspase-9, poly(ADP-ribose) polymerase (PARP), cytokeratins and vascular endothelial growth factor (VEGF) was analysed by RT-PCR, immunohistochemical and Western blot analyses. Results DMBA treated animals developed buccal pouch carcinomas that displayed increased expression of p21, cyclin D1, GST-P, NF-κB, cytokeratins, VEGF and Bcl-2 with decreased expression of Bax, cytochrome C, caspase-3, caspase-9, and PARP. Dietary administration of both P-B and BTF-35 reduced the incidence of DMBA-induced HBP carcinomas by modulating markers of cell proliferation, cell survival, tumour infiltration, angiogenesis, and apoptosis. Conclusion The results of the present study provide a mechanistic basis for the chemopreventive potential of black tea polyphenols. The greater efficacy of BTF-35 in inhibiting HBP carcinogenesis and modulating multiple molecular targets may have a potential role in the prevention of oral cancer. PMID:18053169

  9. Alveolar ridge augmentation by connective tissue grafting using a pouch method and modified connective tissue technique: A prospective study

    PubMed Central

    Agarwal, Ashish; Gupta, Narinder Dev

    2015-01-01

    Background: Localized alveolar ridge defect may create physiological and pathological problems. Developments in surgical techniques have made it simpler to change the configuration of a ridge to create a more aesthetic and more easily cleansable shape. The purpose of this study was to compare the efficacy of alveolar ridge augmentation using a subepithelial connective tissue graft in pouch and modified connective tissue graft technique. Materials and Methods: In this randomized, double blind, parallel and prospective study, 40 non-smoker individuals with 40 class III alveolar ridge defects in maxillary anterior were randomly divided in two groups. Group I received modified connective tissue graft, while group II were treated with subepithelial connective tissue graft in pouch technique. The defect size was measured in its horizontal and vertical dimension by utilizing a periodontal probe in a stone cast at base line, after 3 months, and 6 months post surgically. Analysis of variance and Bonferroni post-hoc test were used for statistical analysis. A two-tailed P < 0.05 was considered to be statistically significant. Results: Mean values in horizontal width after 6 months were 4.70 ± 0.87 mm, and 4.05 ± 0.89 mm for group I and II, respectively. Regarding vertical heights, obtained mean values were 4.75 ± 0.97 mm and 3.70 ± 0.92 mm for group I and group II, respectively. Conclusion: Within the limitations of this study, connective tissue graft proposed significantly more improvement as compare to connective tissue graft in pouch. PMID:26759591

  10. Blake's pouch cyst and Werdnig-Hoffmann disease: Report of a new association and review of the literature

    PubMed Central

    Shohoud, Sherien A.; Azab, Waleed A.; Alsheikh, Tarek M.; Hegazy, Rania M.

    2014-01-01

    Background: We report a case of a neonate with proximal spinal muscular atrophy (SMA) type 1 (also known as Werdnig-Hoffmann disease or severe infantile acute SMA) associated with a Blake's pouch cyst; a malformation that is currently classified within the spectrum of Dandy-Walker complex. The association of the two conditions has not been previously reported in the English literature. A comprehensive review of the pertinent literature is presented. Case Description: A male neonate was noted to have paucity of movement of the four limbs with difficulty of breathing and poor feeding soon after birth. Respiratory distress with tachypnea, necessitated endotracheal intubation and mechanical ventilation. Pregnancy was uneventful except for decreased fetal movements reported by the mother during the third trimester. Neurological examination revealed generalized hypotonia with decreased muscle power of all limbs, nonelicitable deep tendon jerks, and occasional tongue fasciculations. Molecular genetic evaluation revealed a homozygous deletion of both exons 7 and 8 of the survival motor neuron 1 (SMN1) gene, and exon 5 of the neuronal apoptosis inhibitory protein (NAIP) gene on the long arm of chromosome 5 consistent with Werdnig-Hoffmann disease (SMA type 1). At the age of 5 months, a full anterior fontanelle and abnormal increase of the occipito-frontal circumference were noted. Computed tomographic (CT) scan and magnetic resonance imaging (MRI) of the brain revealed a tetraventricular hydrocephalus and features of Blake's pouch cyst of the fourth ventricle. Conclusions: This case represents a previously unreported association of Blake's pouch cyst and SMA type 1. PMID:25225621

  11. The LPS-induced neutrophil recruitment into rat air pouches is mediated by TNFα: likely macrophage origin

    PubMed Central

    Arreto, C-D.; Dumarey, C.; Nahori, M-A.; Vargaftig, B. B.

    1997-01-01

    The role of resident cells during the lipopolysaccharide (LPS)-induced neutrophil recruitment into rat air pouches was investigated. In this model, LPS (Escherichia coli, O55: B5 strain; 2–2000 ng) induced a dose– and time-dependent neutrophil recruitment accompanied by the generation of a tumour necrosis factor-α (TNFα)-like activity. Dexamethasone (0.05–5 mug) and cycloheximide (6 ng), injected 2 h before LPS into the pouches, inhibited the neutrophil recruitment and the generation of the TNFα-like activity, while the H1-receptor antagonist mepyramine (1 and 4 mg/kg, i.p., 0.5 h before LPS) and the PAF-receptor antagonist WEB 2170 (0.05 and 1 mg/kg, i.p., 0.5 h before LPS) had no effect. Purified alveolar macrophages (AM) were used to replenish the pouches of cycloheximide-treated recipient rats. AM provided by PBS-treated animals led to the recovery of the LPS-induced neutrophil recruitment and of the TNFα-like formation contrasting with those from cycloheximide-treated animals (1 mg/kg, i.p.). When delivered in situ, liposome-encapsulated clodronate, a macrophage depletor, significantly impaired both the LPSinduced neutrophil recruitment and the TNFα-like activity. An anti-murine TNFα polyclonal antibody (0.5 h before LPS) was also effective. These results emphasize the pivotal role of macrophages for LPS-induced neutrophil recruitment via the formation of TNFα. PMID:18472868

  12. An iminosugar-based heparanase inhibitor heparastatin (SF4) suppresses infiltration of neutrophils and monocytes into inflamed dorsal air pouches.

    PubMed

    Sue, Mayumi; Higashi, Nobuaki; Shida, Hiroaki; Kogane, Yusuke; Nishimura, Yoshio; Adachi, Hayamitsu; Kolaczkowska, Elzbieta; Kepka, Magdalena; Nakajima, Motowo; Irimura, Tatsuro

    2016-06-01

    Local infiltration of inflammatory cells is regulated by a number of biological steps during which the cells likely penetrate through subendothelial basement membranes that contain heparan sulfate proteoglycans. In the present study, we examined whether administration of heparastatin (SF4), an iminosugar-based inhibitor of heparanase, could suppress local inflammation and degradation of heparan sulfate proteoglycans in basement membranes. In a carrageenan- or formyl peptide-induced dorsal air pouch inflammation model, the number of infiltrated neutrophils and monocytes was significantly lower in mice after topical administration of heparastatin (SF4). The concentration of chemokines MIP-2 and KC in pouch exudates of drug-treated mice was similar to control. In a zymosan-induced peritonitis model, the number of infiltrated cells was not altered in drug-treated mice. To further test how heparastatin (SF4) influences transmigration of inflammatory neutrophils, its suppressive effect on migration and matrix degradation was examined in vitro. In the presence of heparastatin (SF4), the number of neutrophils that infiltrated across a Matrigel-coated polycarbonate membrane was significantly lower, while the number of neutrophils passing through an uncoated membrane was not altered. Lysate of bone marrow-derived neutrophils released sulfate-radiolabeled macromolecules from basement membrane-like extracellular matrix, which was suppressed by heparastatin (SF4). Heparan sulfate degradation activity was almost completely abolished after incubation of lysate with protein G-conjugated anti-heparanase monoclonal antibody, strongly suggesting that the activity was due to heparanase-mediated degradation. Taken together, in a dorsal air pouch inflammation model heparastatin (SF4) potentially suppresses extravasation of inflammatory cells by impairing the degradation of basement membrane heparan sulfate. PMID:27015605

  13. Genetics Home Reference: congenital sucrase-isomaltase deficiency

    MedlinePlus

    ... and other compounds made from these sugar molecules (carbohydrates). Congenital sucrase-isomaltase deficiency usually becomes apparent after ... isomaltase deficiency, congenital Merck Manual for Healthcare Professionals: Carbohydrate ... Congenital sucrase-isomaltase deficiency The American ...

  14. Blister pouches for effective reagent storage and release for low cost point-of-care diagnostic applications

    NASA Astrophysics Data System (ADS)

    Smith, Suzanne; Sewart, Rene; Land, Kevin; Roux, Pieter; Gärtner, Claudia; Becker, Holger

    2016-03-01

    Lab-on-a-chip devices are often applied to point-of-care diagnostic solutions as they are low-cost, compact, disposable, and require only small sample volumes. For such devices, various reagents are required for sample preparation and analysis and, for an integrated solution to be realized, on-chip reagent storage and automated introduction are required. This work describes the implementation and characterization of effective liquid reagent storage and release mechanisms utilizing blister pouches applied to various point-of-care diagnostic device applications. The manufacturing aspects as well as performance parameters are evaluated.

  15. Boron biodistribution for BNCT in the hamster cheek pouch oral cancer model: Combined administration of BSH and BPA

    SciTech Connect

    D.W. Nigg; William Bauer; Various Others

    2014-06-01

    Sodium mercaptoundecahydro-closo-dodecaborate (BSH) is being investigated clinically for BNCT. We examined the biodistribution of BSH and BPA administered jointly in different proportions in the hamster cheek pouch oral cancer model. The 3 assayed protocols were non-toxic, and showed preferential tumor boron uptake versus precancerous and normal tissue and therapeutic tumor boron concentration values (70–85 ppm). All 3 protocols warrant assessment in BNCT studies to contribute to the knowledge of (BSH+BPA)-BNCT radiobiology for head and neck cancer and optimize therapeutic efficacy.

  16. Associated noncardiac congenital anomalies among cases with congenital heart defects.

    PubMed

    Stoll, Claude; Dott, Beatrice; Alembik, Yves; Roth, Marie-Paule

    2015-02-01

    Cases with congenital heart defects (CHD) often have other associated anomalies. The purpose of this investigation was to assess the prevalence and the types of associated anomalies in CHD in a defined population. The anomalies associated with CHD were collected in all live births, stillbirths and terminations of pregnancy during 26 years in 346,831 consecutive pregnancies of known outcome in the area covered by our population based registry of congenital anomalies. Of the 4005 cases with CHD born during this period (total prevalence of 115.5 per 10,000), 1055 (26.3%) had associated major anomalies. There were 354 (8.8%) cases with chromosomal abnormalities including 218 trisomies 21, and 99 (2.5%) nonchromosomal recognized dysmorphic conditions. There were no predominant recognized dysmorphic conditions, but VACTERL association. However, other recognized dysmorphic conditions were registered including Noonan syndrome, fetal alcohol syndrome, and skeletal dysplasias. Six hundred and two (15.0%) of the cases had non syndromic, non chromosomal multiple congenital anomalies (MCA). Anomalies in the urinary tract, the musculoskeletal, the digestive, and the central nervous systems were the most common other anomalies. Prenatal diagnosis was obtained in 18.7% of the pregnancies. In conclusion the overall prevalence of associated anomalies, which was one in four infants, emphasizes the need for a thorough investigation of cases with CHD. A routine screening for other anomalies may be considered in infants and in fetuses with CHD. One should be aware that the anomalies associated with CHD can be classified into a recognizable anomaly, syndrome or pattern in one out of nine cases with CHD. PMID:25497206

  17. Sphingolipids in colon cancer

    PubMed Central

    García-Barros, Mónica; Coant, Nicolas; Truman, Jean-Philip; Snider, Ashley J.

    2013-01-01

    Colorectal cancer is one of the major causes of death in the western world. Despite increasing knowledge of the molecular signaling pathways implicated in colon cancer, therapeutic outcomes are still only moderately successful. Sphingolipids, a family of N-acyl linked lipids, have not only structural functions but are also implicated in important biological functions. Ceramide, sphingosine and sphingosine-1-phosphate are the most important bioactive lipids, and they regulate several key cellular functions. Accumulating evidence suggests that many cancers present alterations in sphingolipids and their metabolizing enzymes. The aim of this review is to discuss the emerging roles of sphingolipids, both endogenous and dietary, in colon cancer and the interaction of sphingolipids with WNT/β-catenin pathway, one of the most important signaling cascades that regulate development and homeostasis in intestine PMID:24060581

  18. Giant colon lipoma

    PubMed Central

    Yaman, İsmail; Derici, Hayrullah; Demirpolat, Gülen

    2015-01-01

    Colon lipomas are rare, non-epithelial tumors. They are generally smaller than two centimeters and asymptomatic, they are incidentally diagnosed and do not require treatment. Large and symptomatic colon lipomas are rather rare. Its differential diagnosis is generally made by histopathological examination of the resected specimen. A fifty-year-old female patient presented with the symptoms of abdominal pain, swelling in the abdomen and loss of weight. During colonoscopy, there was a submucosal mass of 8×6 cm, which almost completely obstructed the lumen in the hepatic flexure and was covered by a mucosa that was sporadically ulcerated and necrotic in nature. In magnetic resonance imaging, an ovoid mass with a diameter of 8.5 cm at its widest dimension was detected, which had signal intensity similar to that of adipose tissue. Since the patient was symptomatic and differential diagnosis could not be made, she underwent laparoscopic right hemicolectomy. A submucosal lipoma was detected on histopathological examination of the specimen. The patient was discharged without any problems on post-operative day 7. Definite diagnosis of lipomas before surgery is challenging; they may be mistaken for malignancy, especially if the lesion is large and ulcerated. For large and symptomatic colon lipomas, surgery is required to both prevent complications and rule out malignancy. PMID:26170744

  19. CONGENITAL PSEUDARTHROSIS OF THE CLAVICLE

    PubMed Central

    de Figueiredo, Marina Juliana Pita Sassioto Silveira; dos Reis Braga, Susana; Akkari, Miguel; Prado, José Carlos Lopes; Santili, Cláudio

    2015-01-01

    Congenital pseudarthrosis of the clavicle (PCC) is a rare affection, that can be diagnosed at birth and represent a disturbance of union of the ossification centers. It's more common in girls and in the right side. This study objectives to proceed a revision about the subject, that was searched in online database of LILACS and MEDLINE. We found 56 articles till present data. Besides be a bit infrequent, the PCC must not be missed or even forgotten, especially as differential diagnosis with acute fracture of the clavicle at birth by trauma in the childbirth. The diagnostic is relatively easy and the treatment can be just observation or even surgical. PMID:27047839

  20. Presence of PAF-acether in stool of patients with pouch ileoanal anastomosis and pouchitis.

    PubMed

    Chaussade, S; Denizot, Y; Valleur, P; Nicoli, J; Raibaud, P; Guerre, J; Hautefeuille, P; Couturier, D; Benveniste, J

    1991-06-01

    Platelet-activating factor is an endogenous phospholipid produced by a wide variety of inflammatory cells. Platelet-activating factor induces severe pathological changes in various organs and, among numerous potent effects, causes bowel necrosis. Pouchitis is a poorly understood complication of ileoanal pouch anastomosis which occurs in patients who undergo surgery for ulcerative colitis. The aim of this study was to measure ileal or fecal platelet-activating factor and lyso platelet-activating factor contents in normal volunteers (n = 12), in patients with terminal ileostomy (n = 7), and in patients with ileoanal anastomosis (n = 15) (8 patients have pouchitis defined by the presence of ulcerations on the reservoir). Fecal samples were processed and assessed for platelet-activating factor by platelet aggregation assay. The aggregating material was further characterized as platelet-activating factor by the following: inhibition of the platelet aggregation it induced by specific platelet-activating factor receptor antagonist (BN 52021; IHB, Le Plessis Robinson, France); abolition of platelet aggregation after incubation with phospholipase A2 but not with lipase A1; and retention time on high-performance liquid chromatography. Stool platelet-activating factor content (in nanograms per gram of stool, mean +/- 1SD) was significantly increased in patients with pouchitis (22.2 +/- 16 ng/g) compared with patients with normal reservoir (1.59 +/- 0.63 ng/g, P less than 0.01), terminal ileostomy (0.59 +/- 0.43 ng/g, P less than 0.01), and healthy controls (0 +/- 0 ng/g of stool, P less than 0.001). Lyso platelet-activating factor (nanograms per gram of stool) was increased in patients with pouchitis (10,704 +/- 5499 ng/g) compared with patients with normal reservoir (4721 +/- 4549 ng/g of stool, P less than 0.05), terminal ileostomy (3042 +/- 4019 ng/g, P less than 0.02), and healthy volunteers (128 +/- 107 ng/g, P less than 0.001). In patients with ileoanal anastomosis and

  1. Congenital duodenal obstruction: causes and imaging approach.

    PubMed

    Brinkley, Michael F; Tracy, Elisabeth T; Maxfield, Charles M

    2016-07-01

    Bilious emesis is a common cause for evaluation in pediatric radiology departments. There are several causes of congenital duodenal obstruction, most of which require elective surgical correction, but the potential of malrotation with midgut volvulus demands prompt imaging evaluation. We review the various causes of congenital duodenal obstruction with an emphasis on the approach to imaging evaluation and diagnosis. PMID:27324508

  2. Arrhythmias in Complex Congenital Heart Disease

    PubMed Central

    Hayward, Robert M.; Tseng, Zian H.

    2014-01-01

    Late after surgical repair of complex congenital heart disease, atrial arrhythmias are a major cause of morbidity, and ventricular arrhythmias and sudden cardiac death are a major cause of mortality. The six cases in this article highlight common challenges in the management of arrhythmias in the adult congenital heart disease population. PMID:25197326

  3. Diffuse lymphoid follicles of the colon associated with colonic carcinoma.

    PubMed

    Bronen, R A; Glick, S N; Teplick, S K

    1984-01-01

    In four patients aged 59-75 years, colonic carcinoma was associated with diffuse lymphoid follicles in the colon. In one case, the prominence and distribution of the lymphoid follicles corresponded to the progression and regression of the tumor bulk. It is extremely unusual to demonstrate lymphoid follicles, particularly diffuse, on barium enema in patients in this age range. The colonic carcinomas and lymphoid follicles are directly related, possibly representing an immune response. PMID:6606941

  4. Comparsion of light dose on topical ALA-mediated photodynamic therapy for DMBA-induced hamster buccal pouch premalignant lesions

    NASA Astrophysics Data System (ADS)

    Yang, Deng-Fu; Tseng, Meng-Ke; Liu, Chung-Ji; Hsu, Yih-Chih

    2012-03-01

    Oral cancer has becomes the most prominent male cancer disease due to the local betel nut chewing habit combing with smoking and alcohol-drinking lifestyle. In order to minimize the systemic phototoxic effect of 5-aminolevulinic acid (ALA), this study was designed to use a topical ALA-mediated PDT for treatment of DMBA-induced hamster buccal pouch cancerous lesions. DMBA was applied to one of the buccal pouches of hamsters thrice a week for 8 to 10 weeks. Precancerous lesions were induced and proven by histological examination. These DMBA-induced cancerous lesions were used for testing the efficacy of topical ALA -mediated PDT. We found that ALA reached its peak level in cancerous lesions about 2.5 hrs after topical application of ALA gel. The precancerous lesions in hamsters were then treated with topical ALA -mediated PDT with light exposure dose of 75 and 100 J/cm2 using LED 635 nm Wonderlight device. It is suggesting that optimization of the given light dose is critical to the success of PDT results.

  5. Gene expression signature of DMBA-induced hamster buccal pouch carcinomas: modulation by chlorophyllin and ellagic acid.

    PubMed

    Vidya Priyadarsini, Ramamurthi; Kumar, Neeraj; Khan, Imran; Thiyagarajan, Paranthaman; Kondaiah, Paturu; Nagini, Siddavaram

    2012-01-01

    Chlorophyllin (CHL), a water-soluble, semi-synthetic derivative of chlorophyll and ellagic acid (EA), a naturally occurring polyphenolic compound in berries, grapes, and nuts have been reported to exert anticancer effects in various human cancer cell lines and in animal tumour models. The present study was undertaken to examine the mechanism underlying chemoprevention and changes in gene expression pattern induced by dietary supplementation of chlorophyllin and ellagic acid in the 7,12-dimethylbenz[a]anthracene (DMBA)-induced hamster buccal pouch (HBP) carcinogenesis model by whole genome profiling using pangenomic microarrays. In hamsters painted with DMBA, the expression of 1,700 genes was found to be altered significantly relative to control. Dietary supplementation of chlorophyllin and ellagic acid modulated the expression profiles of 104 and 37 genes respectively. Microarray analysis also revealed changes in the expression of TGFβ receptors, NF-κB, cyclin D1, and matrix metalloproteinases (MMPs) that may play a crucial role in the transformation of the normal buccal pouch to a malignant phenotype. This gene expression signature was altered on treatment with chlorophyllin and ellagic acid. Our study has also revealed patterns of gene expression signature specific for chlorophyllin and ellagic acid exposure. Thus dietary chlorophyllin and ellagic acid that can reverse gene expression signature associated with carcinogenesis are novel candidates for cancer prevention and therapy. PMID:22485181

  6. Antiproliferative and apoptosis inducing effect of lactoferrin and black tea polyphenol combination on hamster buccal pouch carcinogenesis.

    PubMed

    Chandra Mohan, Kurapathy Venkata Poorna; Devaraj, Halagowder; Prathiba, Duvuru; Hara, Yukihiko; Nagini, Siddavaram

    2006-10-01

    Combination chemoprevention using tea polyphenols as one of the components has received growing consideration in recent years. The present study was designed to evaluate the antiproliferative and apoptosis inducing effects of bovine lactoferrin (bLF) and black tea polyphenol (Polyphenon-B: P-B) combination on 7,12-dimethylbenz[a]anthracene (DMBA)-induced hamster buccal pouch (HBP) carcinogenesis. Topical application of DMBA for 14 weeks induced buccal pouch tumours that showed aberrant expression of cytokeratins, a marker for epithelial carcinomas. This was associated with increased cell proliferation and evasion of apoptosis as revealed by upregulation of proliferating cell nuclear antigen, NF-kappaB, mutant p53, Bcl-2 and downregulation of Bax, Fas and caspase 3 protein expression. Although dietary administration of bLF and Polyphenon-B alone significantly reduced tumour incidence, combined administration of bLF and Polyphenon-B was more effective in inhibiting HBP carcinogenesis by restoring normal cytokeratin expression, inhibiting cell proliferation and inducing apoptosis. These findings suggest that a "designer item" approach will be useful for human oral cancer prevention strategies. PMID:16905260

  7. Gene Expression Signature of DMBA-Induced Hamster Buccal Pouch Carcinomas: Modulation by Chlorophyllin and Ellagic Acid

    PubMed Central

    Vidya Priyadarsini, Ramamurthi; Kumar, Neeraj; Khan, Imran; Thiyagarajan, Paranthaman; Kondaiah, Paturu; Nagini, Siddavaram

    2012-01-01

    Chlorophyllin (CHL), a water-soluble, semi-synthetic derivative of chlorophyll and ellagic acid (EA), a naturally occurring polyphenolic compound in berries, grapes, and nuts have been reported to exert anticancer effects in various human cancer cell lines and in animal tumour models. The present study was undertaken to examine the mechanism underlying chemoprevention and changes in gene expression pattern induced by dietary supplementation of chlorophyllin and ellagic acid in the 7,12-dimethylbenz[a]anthracene (DMBA)-induced hamster buccal pouch (HBP) carcinogenesis model by whole genome profiling using pangenomic microarrays. In hamsters painted with DMBA, the expression of 1,700 genes was found to be altered significantly relative to control. Dietary supplementation of chlorophyllin and ellagic acid modulated the expression profiles of 104 and 37 genes respectively. Microarray analysis also revealed changes in the expression of TGFβ receptors, NF-κB, cyclin D1, and matrix metalloproteinases (MMPs) that may play a crucial role in the transformation of the normal buccal pouch to a malignant phenotype. This gene expression signature was altered on treatment with chlorophyllin and ellagic acid. Our study has also revealed patterns of gene expression signature specific for chlorophyllin and ellagic acid exposure. Thus dietary chlorophyllin and ellagic acid that can reverse gene expression signature associated with carcinogenesis are novel candidates for cancer prevention and therapy. PMID:22485181

  8. The diameter of the ileal J-pouch-anal anastomosis as an important risk factor of pouchitis – clinical observations

    PubMed Central

    Banasiewicz, Tomasz; Marciniak, Ryszard; Kaczmarek, Elżbieta; Meissner, Wiktor; Krokowicz, Piotr; Paszkowski, Jacek; Walkowiak, Jarosław; Majewski, Przemysław; Marszałek, Andrzej; Drews, Michał

    2011-01-01

    Summary Background Patients’ quality of life after restorative proctocolectomy depends on the potential complications. Stricture of the ileal pouch-anal anastomosis is one of the complications following restorative proctocolectomy. Material/Methods We analyzed the correlation between the diameter of the anastomosis and clinical parameters, including pouchitis disease activity index (PDAI), the activity of fecal M2-pyruvate kinase and maximum tolerable volume of the pouch. The study group consisted of 31 patients in whom covering ileostomy had been closed 72±50 months before enrolement to the study. Restorative proctocolectomy for ulcerative colitis or familial adenomatous polyposis coli had been performed in this group. Results The study did not show any correlation between the diameter of the anastomosis and primary indication for surgery, the time elapsed after restoration of the bowel continuity, the activity of fecal M2-pyruvate kinase, or maximum tolerable volume. However, meaningful correlations between the stricture of the anastomosis and the presence and activity of pouchitis, together with the ileal villi atrophy, were detected. Conclusions Stricture of the anastomosis appears to be an important factor increasing the incidence of pouchitis, and is independent of the underlying condition and time after the operation. Dilation of the anastomosis and prevention of stricture should constitute a permanent element of postoperative follow-up. PMID:21278694

  9. Thermal characterization of large size lithium-ion pouch cell based on 1d electro-thermal model

    NASA Astrophysics Data System (ADS)

    Vertiz, G.; Oyarbide, M.; Macicior, H.; Miguel, O.; Cantero, I.; Fernandez de Arroiabe, P.; Ulacia, I.

    2014-12-01

    Thermal management is one of the key factors to keep lithium-ion cells in optimum electrical performance, under safe working conditions and into a reasonably low ageing process. This issue is becoming particularly relevant due to the heterogeneous heat generation along the cell. Cell working temperature is determined by ambient temperature, heat generation and evacuation capacity. Therefore, thermal management is established by: i) the intrinsic thermal properties (heat capacity & thermal conductivity) and ii) the heat generation electro-thermal parameters (internal resistance, open circuit voltage & entropic factor). In this research, different methods - calculated and experimental - are used to characterize the main heat properties of a 14Ah -LiFePO4/graphite-commercial large sizes pouch cell. In order to evaluate the accuracy of methods, two comparisons were performed. First, Newman heat generation estimations were compared with experimental heat measurements. Secondly, empirical thermal cell behaviour was match with 1D electro-thermal model response. Finally, considering the results, the most adequate methodology to evaluate the key thermal parameters of a large size Lithium-ion pouch cell are proposed to be: i) pulse method for internal resistance, ii)heat loss method for entropic factor; and iii)experimental measurement (ARC calorimeter and C-177-97 standard method) for heat capacity and thermal conductivity.

  10. Application of a spontaneously closed protective stoma in an ileal pouch-anal anastomosis: a preliminary study

    PubMed Central

    Wang, Jinhai; Ke, Bingxin; Lin, Jianjiang; Xu, Jiahe; Chen, Wenbin

    2015-01-01

    Background: To evaluate the application value of a spontaneously closed protective stoma (SCPS) in an ileal pouch-anal anastomosis, which is a novel procedure first performed in our hospital in 2008. Materials and methods: Two males cases with ulcerative colitis and one female with familial adenomatous polyposis were treated with colorectal surgery at the First Affiliated Hospital of Zhejiang University since March 2010. The surgery was designed as total proctocolectomy with an ileal pouch-anal anastomosis and SCPS. The surgical plan and procedure was determined with the patients after analyzing their hospitalized records and follow-up information. Results: No operation-induced death or anastomotic leakage occurred. One patient had a persistent fever and another patient presented with postoperative urinary retention. The average time until flatulence occurred post-SCPS was 26 days, and the average time until the removal of the postoperative stomal tube was 46 days that healed well. Conclusions: An SCPS can effectively protect the anastomosis with a simple operation and avoid the second surgery. Patients with ulcerative colitis require a two-stage operation, those who were in poor health and had a long history of hormone treatment even requiring a three-stage operation. However, a one- or two-stage operation could help alleviate pain for patients who require multiple surgeries and reduce economic burden. PMID:25785126

  11. A flexible and low-cost polypropylene pouch for naked-eye detection of herpes simplex viruses.

    PubMed

    Nahar, Sharifun; Ahmed, Minhaz Uddin; Safavieh, Mohammadali; Rochette, Annie; Toro, Carla; Zourob, Mohammed

    2015-02-01

    Effective viral detection is a key goal in the development of point of care (POC) diagnostic devices. Loop-mediated isothermal amplification (LAMP) could potentially be a valuable tool for rapid viral detection and diagnosis in commercial and hospital laboratories and resource limited settings. Here, we present a novel polypropylene pouch (PP) for detection of HSV-1 and HSV-2. With this plastic pouch we could detect up to 6.08 × 10(1) copies per μL of HSV-1 DNA and 0.598 copies per μL of HSV-2 DNA within 45 minutes. Since LAMP itself is less sensitive to inhibitory substances present in the real sample, we could also detect viral DNA without the need for viral DNA extraction and purification. The result from LAMP could be evaluated by naked eye due to the addition of hydroxy naphthol blue (HNB) dye in the reaction mixture. Since this proposed device is easy to handle, portable, user friendly and low cost, it offers a tremendous potential to be a perfect candidate for POC diagnostic device for use in resource limited settings. PMID:25529059

  12. Cardiac Arrhythmias In Congenital Heart Diseases

    PubMed Central

    Khairy, Paul; Balaji, Seshadri

    2009-01-01

    Arrhythmias figure prominently among the complications encountered in the varied and diverse population of patients with congenital heart disease, and are the leading cause of morbidity and mortality. The incidence generally increases as the patient ages, with multifactorial predisposing features that may include congenitally malformed or displaced conduction systems, altered hemodynamics, mechanical or hypoxic stress, and residual or postoperative sequelae. The safe and effective management of arrhythmias in congenital heart disease requires a thorough appreciation for conduction system variants, arrhythmia mechanisms, underlying anatomy, and associated physiology. We, therefore, begin this review by presenting the scope of the problem, outlining therapeutic options, and summarizing congenital heart disease-related conduction system anomalies associated with disorders of the sinus node and AV conduction system. Arrhythmias encountered in common forms of congenital heart disease are subsequently discussed. In so doing, we touch upon issues related to risk stratification for sudden death, implantable cardiac devices, catheter ablation, and adjuvant surgical therapy. PMID:19898654

  13. Optimum treatment of congenital cytomegalovirus infection.

    PubMed

    Leruez-Ville, Marianne; Ville, Yves

    2016-01-01

    Congenital cytomegalovirus infection affects 0.7% of live births and is the leading cause of congenital neurological handicaps of infectious origin. However, systematic screening of this infection has not been implemented in pregnancy or at birth in any country. This apparent paradox has been justified by the unavailability of an efficient vaccine and by the scarcity of data available on the treatment of congenital CMV. However, in the last decade interesting new data on the management of this congenital infection has emerged including new results on both neonatal and postnatal treatments. This review provides an update on the potential benefits of antiviral treatment and on passive immunisation both in the neonatal and the antenatal periods. These suggest a benefit to a proactive approach for neonatal and prenatal congenital infections. PMID:27043943

  14. [Maternal imagination and congenital malformations].

    PubMed

    Van Heiningen, Teunis Willem

    2011-01-01

    Since antiquity philosophers and scientists tried to explain the cause of congenital malformations. In early modern medicine maternal imagination was largely accepted as their true cause, This concept was rejected by Blondel, a London physician. Around 1750 Wolff introduced the Hemmungsbildung as the cause of congenital malformations, a concept adopted in 1781 by Blumenbach. Later on Soemmerring (1784), Crichton (1785) and Meckel the younger adopted Blumenbach's concept. In 1824 Suringar further developed it. More and more the excessive development of fetal blood vessels or nerves was rejected as a possible cause, although from time to time these ideas were adopted again. In the early 1800s Etienne Geoffroy Saint-Hilaire (1811) and Vrolik (1817) developed a classification of monstra. These attempts urged Isidore Geoffroy Saint-Hilaire (Etienne's son) and Vrolik the younger (Gerard's son) to develop it further. Nevertheless, around 1840 Vrolik had to admit that although we are well acqainted with the various malformations, we are still ignorant of the primary cause of these phenomena. Meanwhile the dispute between the adherents of the theory of preformation and those who had adopted the concept of epigenesis exercised many minds. In the second half of the eighteenth century the latter theory became more and more adopted and this fact cleared the way for the ideas introduced by Wolff and Blumenbach, because it was consistent with the idea of a gradual development of fetal structures. PMID:22073754

  15. Congenital PCB poisoning: a reevaluation

    SciTech Connect

    Miller, R.W.

    1985-05-01

    A review of the literature reveals a need to clarify the pathologic physiology of congenital polychlorinated biphenyl (PCB) poisoning, which is characterized by intrauterine growth retardation, brown staining of the skin and mucous membranes as in Addison's disease, natal teeth, widely open fontanelles and sagittal suture and apparent overgrowth of the gingiva. The skull abnormalities may represent irregular calcification, with natal teeth appearing because the bone of the mandible is penetrated more easily than usual. Some fetuses were poisoned at the time the mothers ingested the oil; others were affected in the subsequent years from residual contamination in the mothers' bodies. The misadventure in Japan was repeated in Taiwan in 1979. The seven congenital cases in Taiwan reported thus far seem to differ from those in Japan. In Taiwan the noses were somewhat black, two of the infants did not have low birth weight and the osseous abnormalities of the skull and gingival hyperplasia were not observed. Systematic followup studies should be made in Taiwan of the children born within 2 years of maternal poisoning with PCBs. Special attention should be given to age at first dentition and skull-X-rays for spotty calcification, among other measures of physical, neurologic and intellectual development.

  16. Colonic lymphangiomatosis associated with anemia

    PubMed Central

    Chung, Woo Chul; Kim, Hye-Kang; Yoo, Jin Young; Lee, Jeong Rok; Lee, Kang-Moon; Paik, Chang Nyol; Jang, U-Im; Yang, Jin Mo

    2008-01-01

    Lymphangioma is an uncommon malformation of lymphatic system. Multiple colonic lymphangioma named as lymphangiomatosis is considered an extremely rare disease. Although lymphangioma is a benign tumor and most colonic lymphangiomas do not cause symptoms and do not require treatment, resection of lymphangioma is necessary in the presence of symptoms such as abdominal pain, bleeding, intussusceptions. We report a case of colonic lymphangiomatosis in a man who presented with abdominal discomfort and anemia, which was diagnosed and treated with endoscopic snare polypectomy. PMID:18837097

  17. Fungal infection of the colon

    PubMed Central

    Praneenararat, Surat

    2014-01-01

    Fungi are pathogens that commonly infect immunocompromised patients and can affect any organs of the body, including the colon. However, the literature provides limited details on colonic infections caused by fungi. This article is an intensive review of information available on the fungi that can cause colon infections. It uses a comparative style so that its conclusions may be accessible for clinical application. PMID:25364269

  18. Paternal transmission of congenital myotonic dystrophy.

    PubMed Central

    de Die-Smulders, C E; Smeets, H J; Loots, W; Anten, H B; Mirandolle, J F; Geraedts, J P; Höweler, C J

    1997-01-01

    We report a rare case of paternally transmitted congenital myotonic dystrophy (DM). The proband is a 23 year old, mentally retarded male who suffers severe muscular weakness. He presented with respiratory and feeding difficulties at birth. His two sibs suffer from childhood onset DM. Their late father had the adult type of DM, with onset around 30 years. Only six other cases of paternal transmission of congenital DM have been reported recently. We review the sex related effects on transmission of congenital DM. Decreased fertility of males with adult onset DM and contraction of the repeat upon male transmission contribute to the almost absent occurrence of paternal transmission of congenital DM. Also the fathers of the reported congenitally affected children showed, on average, shorter CTG repeat lengths and hence less severe clinical symptoms than the mothers of children with congenital DM. We conclude that paternal transmission of congenital DM is rare and preferentially occurs with onset of DM past 30 years in the father. Images PMID:9391889

  19. Pathways to Colonization

    NASA Technical Reports Server (NTRS)

    Smitherman, David V., Jr.

    2003-01-01

    The steps required for space colonization are many to grow from our current 3-person International Space Station, now under construction, to an infrastructure that can support hundreds and eventually thousands of people in space. This paper will summarize the author's findings from numerous studies and workshops on related subjects and identify some of the critical next steps toward space colonization. Findings will be drawn from the author s previous work on space colony design, space infrastructure workshops, and various studies that addressed space policy. In conclusion, this paper will note that significant progress has been made on space facility construction through the International Space Station program, and that significant efforts are needed in the development of new reusable Earth to Orbit transportation systems. The next key steps will include reusable in space transportation systems supported by in space propellant depots, the continued development of inflatable habitat and space elevator technologies, and the resolution of policy issues that will establish a future vision for space development.

  20. Evaluation of Giant African Pouched Rats for Detection of Pulmonary Tuberculosis in Patients from a High-Endemic Setting

    PubMed Central

    Reither, Klaus; Jugheli, Levan; Glass, Tracy R.; Sasamalo, Mohamed; Mhimbira, Francis A.; Weetjens, Bart J.; Cox, Christophe; Edwards, Timothy L.; Mulder, Christiaan; Beyene, Negussie W.; Mahoney, Amanda

    2015-01-01

    Background This study established evidence about the diagnostic performance of trained giant African pouched rats for detecting Mycobacterium tuberculosis in sputum of well-characterised patients with presumptive tuberculosis (TB) in a high-burden setting. Methods The TB detection rats were evaluated using sputum samples of patients with presumptive TB enrolled in two prospective cohort studies in Bagamoyo, Tanzania. The patients were characterised by sputum smear microscopy and culture, including subsequent antigen or molecular confirmation of Mycobacterium tuberculosis, and by clinical data at enrolment and for at least 5-months of follow-up to determine the reference standard. Seven trained giant African pouched rats were used for the detection of TB in the sputum samples after shipment to the APOPO project in Morogoro, Tanzania. Results Of 469 eligible patients, 109 (23.2%) were culture-positive for Mycobacterium tuberculosis and 128 (27.3%) were non-TB controls with sustained recovery after 5 months without anti-TB treatment. The HIV prevalence was 46%. The area under the receiver operating characteristic curve of the seven rats for the detection of culture-positive pulmonary tuberculosis was 0.72 (95% CI 0.66–0.78). An optimal threshold could be defined at ≥2 indications by rats in either sample with a corresponding sensitivity of 56.9% (95% CI 47.0–66.3), specificity of 80.5% (95% CI 72.5–86.9), positive and negative predictive value of 71.3% (95% CI 60.6–80.5) and 68.7% (95% CI 60.6–76.0), and an accuracy for TB diagnosis of 69.6%. The diagnostic performance was negatively influenced by low burden of bacilli, and independent of the HIV status. Conclusion Giant African pouched rats have potential for detection of tuberculosis in sputum samples. However, the diagnostic performance characteristics of TB detection rats do not currently meet the requirements for high-priority, rapid sputum-based TB diagnostics as defined by the World Health

  1. Critical congenital heart disease screening

    PubMed Central

    Chamsi-Pasha, Mohammed A.; Chamsi-Pasha, Hassan

    2016-01-01

    Critical congenital heart disease (CCHD) is a heart lesion for which neonates require early surgical intervention to survive. Without intervention, the rates of mortality and survival with significant disability are extremely high. Early diagnosis can potentially improve health outcomes in newborns with CCHD. Until recent years, no routine screening protocol existed. In the last few years, pulse oximetry screening for CCHD in newborns has been added to the list of recommended uniform screening panels and advocated by several health-care authorities. A positive screening test result warrants an echocardiogram to evaluate for CCHD. Newborn screens do not usually require parental consent. However, most of the states mandates in the United States include a statement allowing exemption from the screen on the basis of parental religious or personal beliefs. PMID:27390667

  2. HLA typing in congenital toxoplasmosis.

    PubMed Central

    Meenken, C; Rothova, A; de Waal, L P; van der Horst, A R; Mesman, B J; Kijlstra, A

    1995-01-01

    HLA-A, HLA-B, HLA-C, and HLA-D typing was performed in 47 mothers of patients suffering from ocular toxoplasmosis to investigate whether an immunogenetic predisposition exists for developing congenital toxoplasmosis in their offspring. No significant association between any HLA antigen was observed in the mothers of patients with ocular toxoplasmosis, although a total absence of the HLA-B51 antigen was found in this group. HLA-A, HLA-B, and HLA-C typing was also performed in their children (52 patients with ocular toxoplasmosis), to investigate a possible relation between the severity of ocular toxoplasmosis and an eventual immunogenetic factor. In the patients with ocular toxoplasmosis an increased frequency of the HLA-Bw62 antigen was observed in correlation with severe ocular involvement. PMID:7612565

  3. Critical congenital heart disease screening.

    PubMed

    Chamsi-Pasha, Mohammed A; Chamsi-Pasha, Hassan

    2016-01-01

    Critical congenital heart disease (CCHD) is a heart lesion for which neonates require early surgical intervention to survive. Without intervention, the rates of mortality and survival with significant disability are extremely high. Early diagnosis can potentially improve health outcomes in newborns with CCHD. Until recent years, no routine screening protocol existed. In the last few years, pulse oximetry screening for CCHD in newborns has been added to the list of recommended uniform screening panels and advocated by several health-care authorities. A positive screening test result warrants an echocardiogram to evaluate for CCHD. Newborn screens do not usually require parental consent. However, most of the states mandates in the United States include a statement allowing exemption from the screen on the basis of parental religious or personal beliefs. PMID:27390667

  4. Prenatal sonographic diagnosis of congenital varicella syndrome.

    PubMed

    Tongsong, Theera; Srisupundit, Kasemsri; Traisrisilp, Kuntharee

    2012-01-01

    Congenital varicella syndrome is a rare disorder occurring in less than 1% of maternal varicella during early pregnancy but is associated with high fetal morbidity and mortality. This case report aimed to describe the sonographic features of congenital varicella syndrome following maternal varicella. Well-documented maternal chicken pox was made at 12 weeks of gestation and prenatal ultrasound was performed at 16 weeks. Striking sonographic features included hydropic changes and disseminated calcifications in multiple organs, especially liver and myocardium. Elective termination of pregnancy was done at 17 weeks. The presence of disseminated calcifications could suggest the diagnosis of congenital varicella syndrome. PMID:22323269

  5. Ventricular Tachycardia in Congenital Pulmonary Stenosis.

    PubMed

    Ruckdeschel, Emily Sue; Schuller, Joseph; Nguyen, Duy Thai

    2016-03-01

    With modern surgical techniques, there is significantly increased life expectancy for those with congenital heart disease. Although congenital pulmonary valve stenosis is not as complex as tetralogy of Fallot, there are many similarities between the 2 lesions, such that patients with either of these conditions are at risk for ventricular arrhythmias and sudden cardiac death. Those patients who have undergone surgical palliation for congenital pulmonary stenosis are at an increased risk for development of ventricular arrhythmias and may benefit from a more aggressive evaluation for symptoms of palpitations or syncope. PMID:26920196

  6. The Pratt Pouch Provides a Three-Fold Access Increase to Antiretroviral Medication for Births outside Health Facilities in Southern Zambia

    PubMed Central

    Dahinten, Alexander P.; Malkin, Robert A.

    2016-01-01

    Introduction: Modern day antiretroviral therapy allows HIV+ pregnant women to lower the likelihood of viral transmission to their infants before, during, and after birth from 20-45% to less than 5%. In developing countries, where non-facility births may outnumber facility births, infant access to safe antiretroviral medication during the critical first three days after birth is often limited. A single-dose, polyethylene pouch (“Pratt Pouch”) addresses this challenge by allowing the medication to be distributed to mothers during antenatal care. Methods: The Pratt Pouch was introduced as part of a one year clinical feasibility study in two districts in Southern Province, Zambia. Participating nurses, community health workers, and pharmacists were trained before implementation. Success in achieving improved antiretroviral medication access was assessed via pre intervention and post intervention survey responses by HIV+ mothers. Results: Access to medication for HIV-exposed infants born outside of a health facility increased from 35% (17/51) before the introduction of the pouch to 94% (15/16) after (p<0.05). A non-significant increase in homebirth rates from 33% (pre intervention cohort) to 50% (post intervention cohort) was observed (p>0.05). Results remained below the national average homebirth rate of 52%. Users reported minimal spillage and a high level of satisfaction with the Pratt Pouch. Conclusion: The Pratt Pouch enhances access to infant antiretroviral medication in a rural, non-facility birth setting. Wide scale implementation could have a substantial global impact on HIV transmission rates from mother to child. PMID:27073584

  7. Clinical and physiological study of anal sphincter and ileal J pouch before preileostomy closure and 6 and 12 months after closure of loop ileostomy.

    PubMed

    Chaussade, S; Michopoulos, S; Hautefeuille, M; Valleur, P; Hautefeuille, P; Guerre, J; Couturier, D

    1991-02-01

    Spontaneous evolution of pouch and anal function, and absorption features has been assessed in 15 patients who underwent proctocolectomy with J ileal pouch anastomosis without conservation of a rectal muscular cuff. All the patients were studied before preileostomy closure and six and 12 months after the closure of the protection loop ileostomy. Stool frequency was identical at six and 12 months (mean +/- SEM: 5.0 +/- 0.4 and 5.3 +/- 0.5/day, respectively). Sixty-six percent of patients at six months and 40% of patients at 12 months need to defecate at least one time during night. Stool weight as well as steatorrhea decreased significantly six months after the closure of loop ileostomy (P less than 0.05). Mean resting anal pressure remained unchanged six and 12 months after closure of the loop ileostomy (41 +/- 6 and 45 +/- 5 cm H2O, respectively). Maximum squeeze anal pressures increased significantly at six (P less than 0.05) and 12 months (P less than 0.05). The rectoanal inhibitory reflex was always absent at the same period. The maximum pouch capacity increased significantly during the first six months (P less than 0.01) from 142 +/- 17 to 279 +/- 27 ml. The maximum infused volume during a saline continence test was not significantly different at six and 12 months; the percentage of evacuation of the reservoir and the volume at which the first ileal contraction appeared in the reservoir increased significantly (P less than 0.05) at six and 12 months. In conclusion, in patients with ileoanal anastomosis and pouch reservoir, the closure of the loop ileostomy is associated with spontaneous modifications of the anal and pouch parameters.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:1988259

  8. Hereditary and Familial Colon Cancer

    PubMed Central

    Jasperson, Kory W.; Tuohy, Thérèse M.; Neklason, Deborah W.; Burt, Randall W.

    2011-01-01

    Between 2% to 5% of all colon cancers arise in the setting of well defined inherited syndromes, including Lynch syndrome, familial adenomatous polyposis, MUTYH-associated polyposis, and certain hamartomatous polyposis conditions. Each is associated with a high risk of colon cancer. In addition to the syndromes, up to one-third of colon cancers exhibit increased familial risk, likely related to inheritance. A number of less penetrant, but possibly more frequent susceptibility genes have been identified for this level of inheritance. Clarification of predisposing genes allows for accurate risk assessment and more precise screening approaches. This review examines the colon cancer syndromes, their genetics and management, and also the common familial colon cancers with current genetic advances and screening guidelines. PMID:20420945

  9. Schwannoma of the sigmoid colon.

    PubMed

    Çakır, Tuğrul; Aslaner, Arif; Yaz, Müjgan; Gündüz, Umut rıza

    2015-01-01

    Colonic schwannomas are very rare gastrointestinal tumours originating from Schwann cells, which form the neural sheath. Primary schwannomas of the lower gastrointestinal tract are very rare and usually benign in nature. However, if they are not surgically removed, malign degeneration can occur. We report a case of a 79-year-old woman who presented to our clinic with rectal bleeding and constipation. She underwent a lower gastrointestinal tract endoscopy. A mass subtotally obstructing the lumen of the sigmoid colon was seen and biopsies were taken. Histopathological examination indicated a suspicion of gastrointestinal tumour and the patient underwent sigmoid colon resection after preoperative evaluation by laboratory analysis, abdominal ultrasonography and CT. Her postoperative course was uneventful and she was discharged on the fifth day for outpatient control. The histopathology report revealed schwannoma of the sigmoid colon. This was a case of schwannoma of the sigmoid colon that was successfully treated with total resection. PMID:25976197

  10. Diclazuril Protects against Maternal Gastrointestinal Syndrome and Congenital Toxoplasmosis

    PubMed Central

    Oz, Helieh S.; Tobin, Thomas

    2014-01-01

    Background Toxoplasmosis is a common cause of foodborne, gastrointestinal and congenital syndrome with particularly severe or unknown health consequences. There is no safe and effective preventive or therapeutic modality against congenital toxoplasmosis or to eliminate the persistent chronic infection. Hypothesis Diclazuril to be safe in pregnancy and effective against gastrointestinal toxoplasmosis. Methods CD1 programmed pregnant mice were divided into groups and administered a diet containing diclazuril, or sham control. Treatments were initiated on Day 5 of pregnancy and continued until Day 16 when dams were euthanatized. On Day 8 of pregnancy dams were infected intraperitoneally with escalating doses of tachyzoites (0, 100, 300, 600) from Type II strain. Dams were monitored daily for distress, pain, and abortion and samples collected at the end of the experiments. Results Infected dams developed moderate to severe Toxoplasma related complications in tachyzoites dose dependent manner. Animals became anemic and showed hydrothorax, and ascities. Diclazuril effectively protected dams from ascities and anemia (p < 0.05). Infected dams showed splenomegaly, with massive infiltration of epithelioid cells compared with the protective effect of diclazuril in treated animals. Infected dams exhibited severe hepatitis (score 0 to 4 scale = 3.5 ± 0.01) with influx of inflammatory and plasma cells, dysplastic hepatocytes, multinucleated giant cell transformation and hepatic cells necrosis. Diclazuril treatment significantly protected dams from hepatitis, also in tachyzoites dose (100, 300, 600) dependent manner (respectively infected-treated versus infected controls, p < 0.001, p < 0.01 and p < 0.05). Colonic tissues were significantly shortened in length, with infiltration of lymphocytes, and macrophages and microabscess formations in the cryptic structures, with significant improvement in diclazuril treated animals. Additionally, the number of fetuses, fetal length and

  11. Congenital retrosternal hernias of Morgagni: Manifestation and treatment in children

    PubMed Central

    Slepov, Oleksii; Kurinnyi, Sergii; Ponomarenko, Oleksii; Migur, Mikhailo

    2016-01-01

    Background: Due to scarcity of congenital diaphragmatic hearnias of Morgagni (CDHM), non-specific clinical presentation in the pediatric age group, we aimed to investigate the incidence, clinical manifestations, anatomical characteristics, and develop diagnostic algorithm and treatment of CDHM in children. Materials and Methods: The patients’ records of children with CDHM treated in our hospital during past 20 years were retrospectively reviewed for the age at diagnosis, gender, clinical findings, anatomical features, operative details and outcome. Results: Since 1995 to 2014 we observed 6 (3 boys, 3 girls) patients with CDHM, that comprise 3.2% of all congenital diaphragmatic hernia cases (n = 185). Age at diagnosis varied from 3 mo. to 10y.o. Failure to thrive was main symptom in 4 patients, followed by recurrent respiratory infections (n = 3), dyspnea (n = 3), and gastrointestinal manifestations: constipation (n = 2), abdominal pain (n = 1). Work-up consisted of plain X-ray for all (n = 6), upper GI (n = 3), barium enema (n = 2), sonography (n = 6) and CT (n = 2). Abdominal approach used in 5 patients, and thoracotomy in one. Herniated contents were: liver lobes (n = 4), transverse colon (n = 3) and greater omentum (n = 1). 5 had right-sided lesion, 1- left-sided. Defect repaired using local tissues. Post-operative course was uneventful; all patients appeared well during follow-up. Conclusion: CDHM is very uncommon anomaly, very occasionally diagnosed at the early age. Failure to thrive and recurrent respiratory infections are most frequent clinical manifestations. In suspected CDHM we advocate the following work-up: plain chest and abdominal X-ray, contrast study (upper GI series or barium enema), ultrasonographic screen and CT scan. Surgical repair via abdominal approach, using local tissues and hernia sac removal is preferred. PMID:27251653

  12. Colonize, evade, flourish

    PubMed Central

    Rubin, Erica J; Trent, M Stephen

    2013-01-01

    Helicobacter pylori is an adapted gastric pathogen that colonizes the human stomach, causing severe gastritis and gastric cancer. A hallmark of infection is the ability of this organism to evade detection by the human immune system. H. pylori has evolved a number of features to achieve this, many of which involve glyco-conjugates including the lipopolysaccharide, peptidoglycan layer, glycoproteins, and glucosylated cholesterol. These major bacterial components possess unique features from those of other gram-negative organisms, including differences in structure, assembly, and modification. These defining characteristics of H. pylori glycobiology help the pathogen establish a long-lived infection by providing camouflage, modulating the host immune response, and promoting virulence mechanisms. In this way, glyco-conjugates are essential for H. pylori pathogenicity and survival, allowing it to carve out a niche in the formidable environment of the human stomach. PMID:23859890

  13. [Irritable colon and constipation].

    PubMed

    Meyenberger, C

    1993-04-20

    Irritable bowel syndrome is a very common clinical problem with a broad spectrum of severity. The management includes a combination of positive diagnosis of typical symptoms with limited investigations to exclude underlying structural or biochemical disorders. Therapeutic trials focus on the relief of predominant symptoms. Identification and modification of factors exacerbating symptoms, behavioural techniques and pharmacologic agents directed to the presumed gastrointestinal motor dysfunction are required. Psychological support by the physician is the most important part of treatment. Chronic constipation may be the predominant symptom of irritable bowel syndrome. Underlying organic disorders must be excluded by clinical examination and endoscopy. Severe chronic constipation requires further investigation of colonic motility and defecation. High fibre diet, osmotic laxatives and procinetic agents may lead to an improvement. In rare cases surgery may be indicated. PMID:8488351

  14. Living with a Congenital Heart Defect

    MedlinePlus

    ... well the heart’s chambers and valves are working. Health Insurance and Employment Adults who have congenital heart defects ... carefully consider how changing jobs will affect their health insurance coverage. Some health plans have waiting periods or ...

  15. Congenital urethrocutaneous fistula in an adolescent male

    PubMed Central

    Kale, Satish M.; Mody, Nikunj B.; Patil, Surendra B.; Sadawarte, Pranam

    2015-01-01

    A urethrocutaneous fistula is a common complication after hypospadias repair, but congenital fistula is a rare anomaly. We present a 16-year-old boy with this unusual anomaly. Its etiology, embryology, and management are discussed in brief. PMID:26424989

  16. Congenital and Genetic Disease in Domestic Animals

    ERIC Educational Resources Information Center

    Mulvihill, John J.

    1972-01-01

    Reviews observations on domestic animals that have led to the identification of environmental teratogens, and have provided insight into the pathogenesis of congenital defects and genetic diseases in man." (Author/AL)

  17. Missed congenital hypothyroidism in an identical twin.

    PubMed

    Azam, Anita; Cutfield, Wayne; Mouat, Fran; Hofman, Paul L; Jefferies, Craig; Webster, Dianne; Gunn, Alistair Jan

    2012-10-01

    Newborn screening for congenital hypothyroidism has been remarkably effective, although rare cases of false negative screening have been reported in same sex twins, presumptively due to fetal blood exchange. We report a case in which the diagnosis of congenital hypothyroidism due to thyroid ectopia in a monozygotic twin was delayed by 8 months, with a normal newborn screening TSH level of 11 mIU/L blood (normal < 15 mIU/L) at 2 days of life. This is the first such case since the national New Zealand newborn screening programme introduced screening for congenital hypothyroidism in 1981 (30 years ago). Repeating thyroid studies at 14 days of age in same-sex twins has been advocated to avoid delayed diagnosis, but given the low risk, may not be cost effective. It is important to maintain a high index of suspicion in same-sex twin pregnancies of potential congenital hypothyroidism. PMID:22970836

  18. Genetics Home Reference: Fukuyama congenital muscular dystrophy

    MedlinePlus

    ... and walking. Fukuyama congenital muscular dystrophy also impairs brain development. People with this condition have a brain abnormality ... cobblestones). These changes in the structure of the brain lead to significantly delayed development of speech and motor skills and moderate to ...

  19. Genetics Home Reference: autosomal recessive congenital methemoglobinemia

    MedlinePlus

    ... congenital methemoglobinemia is caused by mutations in the CYB5R3 gene. This gene provides instruction for making an ... isoforms) of this enzyme are produced from the CYB5R3 gene. The soluble isoform is present only in ...

  20. Genetics Home Reference: congenital mirror movement disorder

    MedlinePlus

    ... throughout life, without other associated signs and symptoms. Intelligence and lifespan are not affected. People with congenital ... movement. Normally, signals from each half of the brain control movements on the opposite side of the ...

  1. Genetics Home Reference: congenital insensitivity to pain

    MedlinePlus

    ... have a complete loss of the sense of smell (anosmia). Congenital insensitivity to pain is considered a ... to cells that detect sensations such as touch, smell, and pain. Related Information What does it mean ...

  2. Digenic mutations in severe congenital neutropenia

    PubMed Central

    Germeshausen, Manuela; Zeidler, Cornelia; Stuhrmann, Manfred; Lanciotti, Marina; Ballmaier, Matthias; Welte, Karl

    2010-01-01

    Severe congenital neutropenia a clinically and genetically heterogeneous disorder. Mutations in different genes have been described as causative for severe neutropenia, e.g. ELANE, HAX1 and G6PC3. Although congenital neutropenia is considered to be a group of monogenic disorders, the phenotypic heterogeneity even within the yet defined genetic subtypes points to additional genetic and/or epigenetic influences on the disease phenotype. We describe congenital neutropenia patients with mutations in two candidate genes each, including 6 novel mutations. Two of them had a heterozygous ELANE mutation combined with a homozygous mutation in G6PC3 or HAX1, respectively. The other 2 patients combined homozygous or compound heterozygous mutations in G6PC3 or HAX1 with a heterozygous mutation in the respective other gene. Our results suggest that digenicity may underlie this disorder of myelopoiesis at least in some congenital neutropenia patients. PMID:20220065

  3. Congenital esophageal stenosis owing to tracheobronchial remnants

    PubMed Central

    Rebelo, Priscila Guyt; Ormonde, João Victor C.; Ormonde, João Baptista C.

    2013-01-01

    OBJECTIVE To emphasize the need of an accurate diagnosis of congenital esophageal stenosis due to tracheobronchial remnants, since its treatment differs from other types of congenital narrowing. CASE DESCRIPTION Four cases of lower congenital esophageal stenosis due to tracheobronchial remnants, whose definitive diagnosis was made by histopathology. Except for the last case, in which a concomitant anti-reflux surgery was not performed, all had a favorable outcome after resection and anastomosis of the esophagus. COMMENTS The congenital esophageal stenosis is an intrinsic narrowing of the organâ€(tm)s wall associated with its structural malformation. The condition can be caused by tracheobronchial remnants, fibromuscular stenosis or membranous diaphragm and the first symptom is dysphagia after the introduction of solid food in the diet. The first-choice treatment to tracheobronchial remnants cases is the surgical resection and end-to-end anastomosis of the esophagus. PMID:24142326

  4. Genetics Home Reference: congenital diaphragmatic hernia

    MedlinePlus

    ... Center: Congenital Diaphragmatic Hernia University of Michigan Health System These resources from MedlinePlus offer information about the diagnosis and management of various health conditions: Diagnostic Tests Drug Therapy ...

  5. Genetics Home Reference: congenital generalized lipodystrophy

    MedlinePlus

    ... an abnormal heart rhythm (arrhythmia) that can cause sudden death. People with congenital generalized lipodystrophy have a distinctive ... stenosis), and severe arrhythmia that can lead to sudden death. Related Information What does it mean if a ...

  6. Genetics Home Reference: congenital central hypoventilation syndrome

    MedlinePlus

    ... central hypoventilation syndrome: PHOX2B genotype determines risk for sudden death. Pediatr Pulmonol. 2008 Jan;43(1):77-86. ... Rand CM. Congenital central hypoventilation syndrome (CCHS) and sudden infant death syndrome (SIDS): kindred disorders of autonomic regulation. Respir ...

  7. METROPOLITAN ATLANTA CONGENITAL DEFECTS PROGRAM (MACDP)

    EPA Science Inventory

    The Metropolitan Atlanta Congenital Defects Program (MACDP) was established in 1967 by the Centers for Disease Control and Prevention with Emory University and the Georgia Mental Health Institute as the nation's first population-based active ascertainment birth defects surveillan...

  8. Genetics Home Reference: congenital myasthenic syndrome

    MedlinePlus

    ... Advocacy Resources (2 links) Muscular Dystrophy Association Myasthenia Gravis Foundation of America: Congenital Myasthenia GeneReviews (1 link) ... for professional medical care or advice. Users with questions about a personal health condition should consult with ...

  9. The "harlequin" sign and congenital Horner's syndrome.

    PubMed Central

    Morrison, D A; Bibby, K; Woodruff, G

    1997-01-01

    When trying to establish the likely anatomical site (preganglionic or postganglionic) of a lesion causing congenital Horner's syndrome, the distribution of facial flushing (the "harlequin" sign), may be seen. In babies and young children, facial flushing is a relatively simple clinical sign to demonstrate, compared with facial sweating. In unilateral facial flushing the areas that do not flush are almost always identical to the anhidrotic areas. However, neither facial flushing nor testing the pupil reactions with pholedrine or hydroxyamphetamine can be relied on to predict the probable site of any lesion causing congenital Horner's syndrome. Two patients with congenital Horner's syndrome are presented which demonstrated the "harlequin" sign and in whom clinical examination and pharmacological testing gave conflicting evidence for localisation of the site of the causative lesion. The presentation of congenital Horner's syndrome should be investigated and include MRI or CT to exclude a serious underlying cause. Images PMID:9219751

  10. Congenital anomalies after assisted reproductive technology.

    PubMed

    Pinborg, Anja; Henningsen, Anna-Karina Aaris; Malchau, Sara Sofie; Loft, Anne

    2013-02-01

    Worldwide, more than 5 million children have been born after assisted reproductive technology (ART), and in many developed countries ART infants represent more than 1% of the birth cohorts. It is well known that ART children are at increased risk of congenital malformations even after adjustment for known confounders such as maternal age. The proportion of ART children is not negligible, and knowledge about the causes of the higher risk of congenital malformations is crucial to develop prevention strategies to reduce the future risk in ART children. The aim of this review is to summarize the literature on the association between ART and congenital anomalies with respect to subfertility, fertility treatment other than ART, and different ART methods including intracytoplasmic sperm injection, blastocyst culture, and cryotechniques. Trends over time in ART and congenital anomalies will also be discussed. PMID:23290686

  11. Prospective radionuclide renal function evaluation and its correlation with radiological findings in patients with Kock pouch urinary diversion

    SciTech Connect

    Chen, K.K.; Chang, L.S.; Chen, M.T.; Yeh, S.H. )

    1991-05-01

    In an attempt to understand better the status of renal function after Kock pouch urinary diversion we conducted a prospective evaluation of renal function in 25 patients using the radionuclide 131iodine-hippurate. Studies were done before, and at 1 month and every 6 months for 30 months postoperatively. The radionuclide results were then compared to excretory urography and contrast study of the reservoir. Our renal function study included the determination of individual and total effective renal plasma flow (ml. per minute), the time to maximal radioactivity over the kidney (peak time in minutes) and a renogram. The mean total (both kidneys) effective renal plasma flow rates before (25 patients) and at month 1 (19), month 6 (14), month 12 (12), month 18 (6), month 24 (6) and month 30 (7) after operation were 385.5 +/- 112.2, 310.5 +/- 109.9, 362.7 +/- 69.2, 442.0 +/- 97.5, 468.2 +/- 82.5, 405.7 +/- 70.6 and 414.0 +/- 65.1, respectively. A comparison of individual and total effective renal plasma flow before and after operation revealed that only the change of the flow at each or both sides of the kidney before and at 1 month after the operation reached statistically significant differences, respectively (p less than 0.05, paired t test). Postoperatively 5 of 6 patients with hydronephrosis had abnormal peak time and a third segment on the renogram was performed on the corresponding side of the kidney. No reflux was noted on contrast study of the reservoir of any patient followed for up to 30 months. In conclusion, the radionuclide renal function evaluation showed a significant decrease of renal function 1 month after Kock pouch diversion, then it resumed and remained stable (neither improved nor deteriorated) for 30 months. Also the abnormal peak time and third segment on the renogram usually implicated a dilated upper urinary tract.

  12. Colon interposition for oesophageal replacement.

    PubMed

    Thomas, Pascal A; Gilardoni, Adrian; Trousse, Delphine; D'Journo, Xavier B; Avaro, Jean-Philippe; Doddoli, Christophe; Giudicelli, Roger; Fuentes, Pierre

    2009-01-01

    The choice of the colon as an oesophageal substitute results primarily from the unavailability of the stomach. However, given its durability and function, colon interposition keeps elective indications in patients with benign or malignant oesophageal disease who are potential candidates for long survival. The choice of the colonic portion used for oesophageal reconstruction depends on the required length of the graft, and the encountered colonic vascular anatomy, the last being characterised by the near-invariability of the left colonic vessels, in contrast to the vascular pattern of the right side of the colon. Accordingly, the transverse colon with all or part of the ascending colon is the substitute of choice, positioned in the isoperistaltic direction, and supplied either from the left colic vessels for long grafts or middle colic vessels for shorter grafts. Technical key points are: full mobilisation of the entire colon, identification of the main colonic vessels and collaterals, and a prolonged clamping test to ensure the permeability of the chosen nourishing pedicle. Transposition through the posterior mediastinum in the oesophageal bed is the shortest one and thereby offers the best functional results. When the oesophageal bed is not available, the retrosternal route is the preferred alternative option. The food bolus travelling mainly by gravity makes straightness of the conduit of paramount importance. The proximal anastomosis is a single-layer hand-fashioned end-to-end anastomosis to prevent narrowing. When the stomach is available, the distal anastomosis is best performed at the posterior part of the antrum for the reasons of pedicle positioning and reflux prevention, and a gastric drainage procedure is added when the oesophagus and vagus nerves have been removed. In the other cases, a Roux-en-Y jejunal loop is preferable to prevent bile reflux into the colon. Additional procedures include re-establishment of the colonic continuity, a careful closure of

  13. Screening programme for congenital toxoplasmosis in France.

    PubMed

    Thulliez, P

    1992-01-01

    The high prevalence of Toxoplasma gondii infection in France led to the establishment of a national screening programme. Preventive measures were progressively introduced, and these became compulsory in 1978 with the result that the incidence of congenital toxoplasmosis is now markedly reduced. Further improvements may include more systematic sampling from women before pregnancy, better and adequate health education and centralized notification of both maternal and congenital cases of toxoplasmosis. PMID:1290073

  14. Cutaneous mastocytosis associated with congenital alopecia.

    PubMed

    Kim, Cho Rok; Kim, Hyun-Je; Jung, Mi-Young; Lee, Jae-Hyung; Park, Ji-Hye; Lee, Dong-Youn; Lee, Joo-Heung; Yang, Jun-Mo

    2012-07-01

    Mastocytosis is a rare disorder that shows accumulation of mast cells in tissues. Atypical clinical features may mimic impetigo, Langerhans cell histiocytosis, and carcinoid syndrome; however, only 1 case of scarring alopecia associated with mastocytosis has been reported. We present the first case of cutaneous mastocytosis associated with congenital alopecia areata in a 3-year-old Korean girl. This case showed an atypical clinical presentation of congenital alopecia areata, but histopathological results confirmed the diagnosis of cutaneous mastocytosis. PMID:22356916

  15. Congenital diseases of the gastrointestinal tract.

    PubMed

    Lentze, M

    2014-05-01

    With the rapid increase in knowledge on the genetic origin of diseases within the gastrointestinal tract the number of congenital diseases, which already manifest during childhood have drastically increased. Due to the large application of molecular genetics the number is steadily increasing. To make the access to these rare diseases fast and efficient the data base of the National Library of Medicine (Online Mendelian Inheritance of Man - OMIN) is a very helpful online tool, with which all these disease entities can be found easily (http://www.ncbi.nlm.nih.gov/omim). Detailed tables are given to find most of the congenitally inherited disease, which affect the gastrointestinal tract. A variety of congenital diarrheas with disturbances of digestion, hydrolysis, absorption and secretion is described in detail: lactose intolerance, sucrose intolerance, glucose-galactose malabsorption, fructose malabsorption, trehalase and enterokinase deficiency, congenital chloride and sodium diarrhea, congenital hypomagnesaemia, primary bile acid malabsorption, acrodermatitis enteropathica and Menke's syndrome. Also described in detail are diseases with structural anomalies of the intestine like microvillous inclusion disease, congenital tufting enteropathy and IPEX syndrome. The diagnosis in the disturbances of carbohydrate hydrolysis or absorption can be established by H2-breath tests after appropriate sugar challenge. Treatment consists of elimination of the responsible sugar from the diet. The diagnosis of the congenital secretory diarrheas is established by investigation of electrolytes in blood and stool. Substitution of high doses of the responsible mineral can improve the clinical outcome. In acrodermatitis enteropathica low serum zinc level together with the typical skin lesions guide to the diagnosis. High doses of oral zinc aspartate can cure the symptoms of the disease. The diagnosis of structural congenital lesions of the intestine can be established by histology and

  16. An unusual presentation of congenital bronchoesophageal fistula.

    PubMed

    Atalabi, O M; Falade, A G; Obajimi, O M; Akinyinka, O O; Lagundoye, S B; Ibinaiye, P O

    2004-01-01

    We present the case of a 5-week-old neonate with multiple congenital abnormalities including a broncho-oesophageal fistula, which showed radiological features suggestive of congenital diaphragmatic hernia. Emergency limited barium swallow done was initially reported as a case of diaphragmatic hernia. Autopsy revealed pus within the right lung, and a fistulous connection between the oesophagus and an intralobar sequestrated lung. No diaphragmatic hernia or intra-abdominal organ abnormality were seen, and an occipital meningomyelocoele was also confirmed. PMID:15171538

  17. Management of adolescents with congenital adrenal hyperplasia

    PubMed Central

    Merke, Deborah P; Poppas, Dix P

    2014-01-01

    The management of congenital adrenal hyperplasia involves suppression of adrenal androgen production, in addition to treatment of adrenal insufficiency. Management of adolescents with congenital adrenal hyperplasia is especially challenging because changes in the hormonal milieu during puberty can lead to inadequate suppression of adrenal androgens, psychosocial issues often affect adherence to medical therapy, and sexual function plays a major part in adolescence and young adulthood. For these reasons, treatment regimen reassessment is indicated during adolescence. Patients with non-classic congenital adrenal hyperplasia require reassessment regarding the need for glucocorticoid drug treatment. No clinical trials have compared various regimens for classic congenital adrenal hyperplasia in adults, thus therapy is individualised and based on the prevention of adverse outcomes. Extensive patient education is key during transition from paediatric care to adult care and should include education of females with classic congenital adrenal hyperplasia regarding their genital anatomy and surgical history. Common issues for these patients include urinary incontinence, vaginal stenosis, clitoral pain, and cosmetic concerns; for males with classic congenital adrenal hyperplasia, common issues include testicular adrenal rest tumours. Transition from paediatric to adult care is most successful when phased over many years. Education of health-care providers on how to successfully transition patients is greatly needed. PMID:24622419

  18. Approach to the diagnosis of congenital myopathies.

    PubMed

    North, Kathryn N; Wang, Ching H; Clarke, Nigel; Jungbluth, Heinz; Vainzof, Mariz; Dowling, James J; Amburgey, Kimberly; Quijano-Roy, Susana; Beggs, Alan H; Sewry, Caroline; Laing, Nigel G; Bönnemann, Carsten G

    2014-02-01

    Over the past decade there have been major advances in defining the genetic basis of the majority of congenital myopathy subtypes. However the relationship between each congenital myopathy, defined on histological grounds, and the genetic cause is complex. Many of the congenital myopathies are due to mutations in more than one gene, and mutations in the same gene can cause different muscle pathologies. The International Standard of Care Committee for Congenital Myopathies performed a literature review and consulted a group of experts in the field to develop a summary of (1) the key features common to all forms of congenital myopathy and (2) the specific features that help to discriminate between the different genetic subtypes. The consensus statement was refined by two rounds of on-line survey, and a three-day workshop. This consensus statement provides guidelines to the physician assessing the infant or child with hypotonia and weakness. We summarise the clinical features that are most suggestive of a congenital myopathy, the major differential diagnoses and the features on clinical examination, investigations, muscle pathology and muscle imaging that are suggestive of a specific genetic diagnosis to assist in prioritisation of genetic testing of known genes. As next generation sequencing becomes increasingly used as a diagnostic tool in clinical practise, these guidelines will assist in determining which sequence variations are likely to be pathogenic. PMID:24456932

  19. [Congenital thoracic defects demonstrated by radiophotography].

    PubMed

    Jonescu, N; Ionescu, G C

    1991-01-01

    Costal congenital defects are malformations or anomalies of dimension, location or structure of the ribs, generated by disturbances of prenatal development. These anomalies appear in mesoderm which also gives rise to skeleton, muscles, serums, conjunctive tissues, circulatory system and urogenital apparatus. Of the 59,225 persons examined, 502 had costal congenital defects. The presence of azygos lobe (Wrisberg's lobe) in 158 cases (0.26%), of right aortic (arcs high dextroposition of cross) in 2 cases and (3%000) dextroposition of heart in 6 cases (10%000), out of which a situs inversus (complete cardiovascular transposition) were noticed as congenital defects with common origin in embryonic mesoderm. Medical radiophotography can be used as a method in detecting congenital costal defects. Other defects in other organs can be also detected. The congenital costal defects found in an rf examined population, representing a part of the total of congenital malformations, may be one of their indirect epidemiologic indices. Their existence may lead to the explanation of the symptomatology of the nervous compressive type on brachiocephalic vascular, etc. plexus. As some of the defects are an excess of osseous and cartilaginous matter, they may be used, when indicated, as autografts. PMID:1823198

  20. [Aftereffects of congenital infections in infants].

    PubMed

    Burdzenidze, E; Zhvania, M

    2006-12-01

    Congenital infections are among the most pressing health care problems. Congenital infections are not reason of congenital malformation and perinatal mortality only, but also pathologies that can be revealed during first year of life. Frequency for congenital viral infection displayed from birth varies between 23% and 92%. The aim of the study was the investigation of inherent infection consequences (citomegaloviral infection, herpes infection and chlamidia) in children in different age groups. Under our observation were 81 children with congenital infections. Among them 29 were with citomegaloviral infection, 17 with herpes infection; 15 chlamidia infection and 22 infections mix (citomegalovirus + herpes, citomegalovirus + chlamidia and chlamidia + herpes). In all observed children neurological simptomatic such as neuro-reflectory hyperexcitability syndrom, hypertension-hydrocephalic syndrom, musculary dystonia syndrom, hydrocephaly, retardation of psychomotor development etc. were present. After birth the worst prevalent are pathologies of cardiovascular system: functional cardiopathy, carditis, congenital heart disease (among them multivalvular disease), affection of hepatobilliar system, organs of vision and hearing etc are present also. PMID:17261890

  1. Functional Assessment for Congenital Heart Disease

    PubMed Central

    2014-01-01

    Significant improvement in survival of children with congenital cardiac malformations has resulted in an increasing population of adolescent and adult patients with congenital heart disease. Of the long-term cardiac problems, ventricular dysfunction remains an important issue of concern. Despite corrective or palliative repair of congenital heart lesions, the right ventricle, which may be the subpulmonary or systemic ventricular chamber, and the functional single ventricle are particularly vulnerable to functional impairment. Regular assessment of cardiac function constitutes an important aspect in the long-term follow up of patients with congenital heart disease. Echocardiography remains the most useful imaging modality for longitudinal monitoring of cardiac function. Conventional echocardiographic assessment has focused primarily on quantification of changes in ventricular size and blood flow velocities during the cardiac cycles. Advances in echocardiographic technologies including tissue Doppler imaging and speckle tracking echocardiography have enabled direct interrogation of myocardial deformation. In this review, the issues of ventricular dysfunction in congenital heart disease, conventional echocardiographic and novel myocardial deformation imaging techniques, and clinical applications of these techniques in the functional assessment of congenital heart disease are discussed. PMID:24653734

  2. Treatment and prevention of pouchitis after ileal pouch-anal anastomosis for chronic ulcerative colitis

    PubMed Central

    Singh, Siddharth; Stroud, Andrea M; Holubar, Stefan D; Sandborn, William J; Pardi, Darrell S

    2016-01-01

    Background Pouchitis occurs in approximately 50% of patients following ileal pouch-anal anastomosis (IPAA) for chronic ulcerative colitis. Objectives The primary objective was to determine the efficacy and safety of medical therapies (including antibiotics, probiotics, and other agents) for prevention or treatment of acute or chronic pouchitis. Search methods We searched MEDLINE, EMBASE and the Cochrane Library from inception to October 2014. Selection criteria Randomized controlled trials of prevention or treatment of acute or chronic pouchitis in adults who underwent IPAA for ulcerative colitis were considered for inclusion. Data collection and analysis Two authors independently screened studies for eligibility, extracted data and assessed study quality. Methodological quality was assessed using the Cochrane risk of bias tool. The overall quality of the evidence supporting the outcomes was evaluated using the GRADE criteria. The primary outcome was the proportion of patients with clinical improvement or remission of pouchitis in patients with acute or chronic pouchitis, or the proportion of patients with no episodes of pouchitis after IPAA. The proportion of patients who developed at least one adverse event was a secondary outcome. We calculated the risk ratio (RR) and corresponding 95% confidence interval (CI) for each dichotomous outcome. Main results Thirteen studies (517 participants) were included in the review. Four studies assessed treatment of acute pouchitis. One study (16 participants) compared ciprofloxacin and metronidazole; another (26 participants) compared metronidazole to budesonide enemas; another (18 participants) compared rifaximin to placebo; and the fourth study (20 participants) compared Lactobacillus GG to placebo. Four studies assessed treatment of chronic pouchitis. One study (19 participants) compared glutamine to butyrate suppositories; another (40 participants) compared bismuth enemas to placebo; and two studies (76 participants

  3. Congenital Cystic Lesions of the Lung: Congenital Cystic Adenomatoid Malformation and Bronchopulmonary Sequestration

    PubMed Central

    Sfakianaki, Anna K; Copel, Joshua A

    2012-01-01

    Congenital cystic lesions of the lung in fetuses are rare. The most common malformations of the lower respiratory tract are congenital cystic adenomatoid malformation and bronchopulmonary sequestration. With the increased use of obstetric ultrasound, cystic lung lesions are detected more often antenatally, which allows for proper planning of peripartum and neonatal management. This article discusses a range of diagnostic and management options. PMID:22866187

  4. Congenital cholesteatoma of the infratemporal fossa with congenital aural atresia and mastoiditis: a case report

    PubMed Central

    2012-01-01

    Background Congenital cholesteatoma may be expected in abnormally developed ear, it may cause bony erosion of the middle ear cleft and extend to the infratemporal fossa. We present the first case of congenital cholesteatoma of the infratemporal fossa in a patient with congenital aural atresia that has been complicated with acute mastoiditis. Case presentation A sixteen year old Egyptian male patient presented with congenital cholesteatoma of the infratemporal fossa with congenital aural atresia complicated with acute mastoiditis. Two weeks earlier, the patient suffered pain necessitating hospital admission, magnetic resonance imaging revealed a soft tissue mass in the right infratemporal fossa. On presentation to our institute, Computerized tomography was done as a routine, it proved the diagnosis of mastoiditis, pure tone audiometry showed an air-bone gap of 60 dB. Cortical mastoidectomy was done for treatment of mastoiditis, removal of congenital cholesteatoma was carried out with reconstruction of external auditory canal. Follow-up of the patient for 2 years and 3 months showed a patent, infection free external auditory canal with an air-bone gap has been reduced to 35db. One year after the operation; MRI was done and it showed no residual or recurrent cholesteatoma. Conclusions Congenital cholesteatoma of the infratemporal fossa in cases of congenital aural atresia can be managed safely even if it was associated with mastoiditis. It is an original case report of interest to the speciality of otolaryngology. PMID:22731118

  5. Congenital neuroblastoma with placental involvement.

    PubMed

    Kume, Ayako; Morikawa, Teppei; Ogawa, Makiko; Yamashita, Aki; Yamaguchi, Shunichi; Fukayama, Masashi

    2014-01-01

    We describe an extremely rare case of congenital neuroblastoma with placental involvement. A fetus with a left abdominal mass detected during ultrasonography at 23 weeks' gestation developed hydrops fetalis by 26 weeks' gestation. The mother developed hypertension at 26 5/7 weeks' gestation. Based on a clinical diagnosis of pregnancy-induced hypertension, labor was induced at 26 6/7 weeks. However, intrauterine fetal death was diagnosed during delivery. Postmortern examination revealed a solid tumor at the site of the left adrenal gland. Histological examination of the tumor revealed dense proliferation of small round tumor cells with sparse cytoplasm and hyperchromatic nuclei. Some tumor-cell complexes contained abundant neurofibrils and Hormer-Wright rosettes were observed. A diagnosis of neuroblastoma of the left adrenal gland was made. The liver was markedly enlarged and was extensively replaced by neuroblastoma cells. In addition, small nests of tumor cells were detected in the blood vessels of various organs including the heart, lung, spleen, kidneys, stomach, small and large intestine, thyroid gland, testis, spinal cord, and bone marrow. Histological examination of the enlarged placenta revealed numerous neuroblastoma cells in the villous fetal capillary spaces. The present case was unusual in that the tumor cells were found not only in the chorionic villi, but also in the intervillous space of the maternal vascular system. However, there was no clinical evidence of maternal metastasis. PMID:25550872

  6. Clinical genetics of congenital hypothyroidism.

    PubMed

    Szinnai, Gabor

    2014-01-01

    Congenital hypothyroidism (CH) is a state of insufficient thyroid hormone supply to the organism, starting in utero. Two forms of permanent primary or thyroidal CH are known. Thyroid dysgenesis (TD) describes a spectrum of defects of thyroid organogenesis. Five monogenetic forms due to mutations in TSHR, PAX8, NKX2-1, FOXE1 and NKX2-5 have been identified so far. Thyroid dyshormonogenesis comprises defects at every step of thyroid hormone synthesis. Mutations in 7 genes are well described causing iodine transport defect (SLC5A5), iodine organification defect (TPO, DUOX2, DUOXA2, SLC26A4), thyroglobulin (TG) synthesis or transport defect or iodotyrosine deiodinase (IYD/DEHAL1) deficiency. The new consensus guidelines for CH recommend genetic counseling for each family with an affected child. Mode of inheritance, recurrence rate and possible associated malformations in the context of syndromic forms should be outlined. Molecular genetic studies should be preceded by a detailed phenotypic description of the patient's thyroid disease and a detailed family history. This review summarizes clinical, biochemical and radiological phenotypes and molecular aspects of the known genetic forms of TD and thyroid dyshormonogenesis relevant for genetic counseling and molecular studies. PMID:25231445

  7. Congenital Adrenal Hyperplasia: Unresolved Issues.

    PubMed

    Yau, Mabel; Khattab, Ahmed; Poppas, Dix; Ghizzoni, Lucia; New, Maria

    2016-01-01

    Congenital adrenal hyperplasia (CAH) describes a family of disorders that comes from enzymatic deficiencies in cortisol production, with 21-hydroxylase deficiency causing ∼90% of cases. Distinction is made between the severe classical form and milder nonclassical form of CAH. Molecular genetic analysis is used to confirm the hormonal diagnosis. A high rate of genotype-phenotype disconcordance has been found in 21-hydroxylase deficiency. The goal of treatment is to replace with synthetic glucocorticoids and mineralocorticoids and suppress adrenal androgen production. The treatment of patients affected with nonclassical CAH, particularly males, remains controversial. Variable synthetic glucocorticoids are used and new modes of glucocorticoid delivery are under investigation. To improve height, growth hormone and other adjuvant therapies are employed. Long-term outcomes of genital surgery using modern techniques in females affected with classical CAH continue to be investigated. Prenatal treatment with dexamethasone is available to avoid ambiguous genitalia in these females. Although studies have shown its safety to mother and fetus, prenatal treatment is still regarded as experimental. Currently, prenatal diagnosis of CAH can only be obtained through invasive methods. Recently, the detection of cell-free fetal DNA in maternal plasma has made it possible to make this diagnosis earlier and noninvasively. PMID:27211889

  8. Symptoms of autism among children with congenital deafblindness.

    PubMed

    Dammeyer, Jesper

    2014-05-01

    Associations between congenital deafness or blindness and autism have been found. The main consequences of congenital sensory impairment, being barriers for communication, language and social interaction development, may lead to symptoms of autism. To date only few studies have been reported concerning individuals with congenital deafblindness. This study examines symptoms of autism among 71 children with congenital deafblindness using the Autism Behavior Checklist. The cohort of children with congenital deafblindness was found to have symptoms of autism on a level similar to children with another developmental disorder than autism for example intellectual disability. No association was found between severity of congenital sensory impairment and severity or type of symptoms of autism. PMID:24127166

  9. Congenital von Willebrand's disease and clinical hypothyroidism.

    PubMed

    Hassan, S; Qureshi, W; Donthireddy, V; Kuriakose, P

    2013-03-01

    Data from case reports and systematic reviews suggest an association of Hypothyroidism and Acquired von Willebrand's syndrome. It is not known if congenital von Willebrand's disease is associated with hypothyroidism in a similar way. The aim of this study was to identify the association of congenital von Willebrand's disease (VWD) with clinical hypothyroidism. A total of 350 cases of congenital VWD were initially screened from our institution database from 1985 to 2010. A careful review of patient records was carried out to see if patients truly had congenital VWD and coexisting clinical hypothyroidism. Patients with uncertain diagnoses or other bleeding disorders were excluded, leading to 197 patients remaining in the final sample. A random age- and sex-matched parallel control group was also obtained from the hospital database. Of 197 patients (mean age 43.8 ± 17.5 years, women 72%) of congenital VWD, 32/197 (16%) were diagnosed with clinical hypothyroidism, while only 11/197 (5.6%) of the matched controls were clinically hypothyroid. Univariate and multivariate analysis demonstrated that VWD was an independent predictor of developing clinical hypothyroidism (OR 3.45; 95% CI 1.65-7.22, P = 0.001). The proportion of patients diagnosed with clinical hypothyroidism was more in the VWD group (P < 0.0001). Our analysis shows a strong association of clinical hypothyroidism in patients with congenital VWD, but future studies will be required to delineate a pathological mechanism. In our opinion, clinicians should consider checking thyroid function in the newly diagnosed and established cases of congenital VWD. PMID:23171382

  10. Congenital malaria in Urabá, Colombia

    PubMed Central

    2011-01-01

    Background Congenital malaria has been considered a rare event; however, recent reports have shown frequencies ranging from 3% to 54.2% among newborns of mothers who had suffered malaria during pregnancy. There are only a few references concerning the epidemiological impact of this entity in Latin-America and Colombia. Objective The aim of the study was to measure the prevalence of congenital malaria in an endemic Colombian region and to determine some of its characteristics. Methods A prospective, descriptive study was carried out in the mothers who suffered malaria during pregnancy and their newborns. Neonates were clinically evaluated at birth and screened for Plasmodium spp. infection by thick smear from the umbilical cord and peripheral blood, and followed-up weekly during the first 21 days of postnatal life through clinical examinations and thick smears. Results 116 newborns were included in the study and 80 umbilical cord samples were obtained. Five cases of congenital infection were identified (four caused by P. vivax and one by P. falciparum), two in umbilical cord blood and three in newborn peripheral blood. One case was diagnosed at birth and the others during follow-up. Prevalence of congenital infection was 4.3%. One of the infected newborns was severely ill, while the others were asymptomatic and apparently healthy. The mothers of the newborns with congenital malaria had been diagnosed with malaria in the last trimester of pregnancy or during delivery, and also presented placental infection. Conclusions Congenital malaria may be a frequent event in newborns of mothers who have suffered malaria during pregnancy in Colombia. An association was found between congenital malaria and the diagnosis of malaria in the mother during the last trimester of pregnancy or during delivery, and the presence of placental infection. PMID:21846373

  11. Colon polyps and cancer.

    PubMed

    Kronborg, O

    2004-01-01

    Screening for colorectal neoplasia still is the best method of reducing the mortality due to colorectal cancer, and it is to be hoped that fecal occult blood test programs will expand in the near future and be combined with appropriate endoscopy. There are substantial problems with compliance in large programs with occult blood tests as well as endoscopy. Colonography and DNA testing in feces are not yet suitable for population screening. Diagnostic strategies in symptomatic patients are becoming more selective, in the hope of avoiding many superfluous examinations without increasing the risk of missing cancers. New results have confirmed the preventive effect of long-term aspirin use on adenoma recurrence, but the most cost-effective dosage is not clear; the mechanism of action is also uncertain, but seems to involve cyclooxygenase-2. The risk of adenomas does not appear to be associated with low consumption of folate, but with low intake of fiber. A number of biomarkers have been evaluated in polyp patients, but so far surveillance is still based on endoscopic experience, which is less than optimal. Attempts have been made to restrict the number of surveillance endoscopies and reduce the pathologist's workload. The place of argon plasma coagulation has been clearly defined in connection with piecemeal removal of large sessile adenomas. Advances have been achieved in surgery and radiotherapy for rectal cancer, and acute surgery for colonic cancer with severe obstruction will be less common after the introduction of the metal stent. PMID:14722849

  12. Characterization of pig colonic mucins.

    PubMed Central

    Fogg, F J; Hutton, D A; Jumel, K; Pearson, J P; Harding, S E; Allen, A

    1996-01-01

    Pig colonic mucins isolated from the adherent mucus gel in the presence of proteinase inhibitors were solubilized by homogenization and the component mucins fractionated by CsC1 density-gradient centrifugation. Polymeric and reduced pig colonic mucin were both largely excluded on Sepharose CL-2B, papain-digested colonic mucin was included. The M(r) values of polymeric, reduced and digested mucins were 5.5 x 10(6), 2.1 x 10(6) and 0.6 x 10(6) respectively. This suggests that pig colonic mucin is comprised of 2-3 subunits, each subunit containing 3-4 glycosylated regions. The intrinsic viscosities of polymeric, reduced and digested mucin were 240 ml.g-1, 100 ml.g-1 and 20 ml.g-1 respectively. Polymeric pig colonic mucin comprised 16% protein per mg of glycoprotein and was rich in serine, threonine and proline (43% of total amino acids). There were approx. 150 disulphide bridges and 53 free thiol groups per mucin polymer. A seventh of the protein content was lost on reduction. This protein was particularly rich in proline and the hydrophobic amino acids. Papain-digested pig colonic mucin contained 11% protein per mg of glycoprotein and was rich in serine, threonine, glutamate and aspartate. All types of amino acids with the exception of aspartate were lost on digestion. The amino acid analysis of the proteolytically digested regions of pig colonic mucin are markedly different to the tandem repeat regions of the human mucin genes shown to be expressed in the colon. PMID:8670173

  13. Genetic Syndromes associated with Congenital Heart Disease

    PubMed Central

    2015-01-01

    Recent research has demonstrated that genetic alterations or variations contribute considerably to the development of congenital heart disease. Many kinds of genetic tests are commercially available, and more are currently under development. Congenital heart disease is frequently accompanied by genetic syndromes showing both cardiac and extra-cardiac anomalies. Congenital heart disease is the leading cause of birth defects, and is an important cause of morbidity and mortality during infancy and childhood. This review introduces common genetic syndromes showing various types of congenital heart disease, including Down syndrome, Turner syndrome, 22q11 deletion syndrome, Williams syndrome, and Noonan syndrome. Although surgical techniques and perioperative care have improved substantially, patients with genetic syndromes may be at an increased risk of death or major complications associated with surgery. Therefore, risk management based on an accurate genetic diagnosis is necessary in order to effectively plan the surgical and medical management and follow-up for these patients. In addition, multidisciplinary approaches and care for the combined extra-cardiac anomalies may help to reduce mortality and morbidity accompanied with congenital heart disease. PMID:26413101

  14. Congenital Scoliosis in Smith–Magenis Syndrome

    PubMed Central

    Li, Zheng; Shen, Jianxiong; Liang, Jinqian; Sheng, Lin

    2015-01-01

    Abstract The Smith–Magenis syndrome (SMS) is a complex and rare congenital condition that is characterized by minor craniofacial anomalies, short stature, sleep disturbances, behavioral, and neurocognitive abnormalities, as well as variable multisystemic manifestations. Little is reported about spinal deformity associated with this syndrome. This study is to present a case of scoliosis occurring in the setting of SMS and explore the possible mechanisms between the 2 diseases. The patient is a 13-year-old Chinese female with congenital scoliosis and Tetralogy of Fallot, mental retardation, obstructive sleep apnea, hypertrophy of tonsil, conductive hearing loss, and agenesis of the epiglottis. An interphase fluorescent in situ hybridization at chromosome 17p11.2 revealed a heterozygous deletion, confirming a molecular diagnosis of SMS. She underwent a posterior correction at thoracic 1-lumbar 1 (T1-L1) levels, using the Moss-SI spinal system. At 6-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of correction. Congenital cardiac disease, immunodeficiency, and severe behavioral problems can affect the surgical outcome following spine fusion and need to be taken into consideration for the surgeon and anesthesiologist. Scoliosis is not uncommon among patients with SMS, and there is a potential association between congenital scoliosis and SMS. The potential mechanisms in the pathogenesis of congenital scoliosis of SMS included retinoic acid-induced 1 (RAI1) microdeletion and RAI1 gene point mutation. PMID:25929900

  15. Congenital lacrimal fistula: A major review.

    PubMed

    Chaung, Jia Quan; Sundar, Gangadhara; Ali, Mohammad Javed

    2016-08-01

    The purpose of this article is to review and summarize the etiopathogenesis, symptomatology, systemic associations, management, complications and clinical outcomes of congenital lacrimal fistulae. The authors performed an electronic database (PubMed, MEDLINE, EMBASE and Cochrane Library) search of all articles published in English on congenital lacrimal fistulae. Congenital subsets of patients from series of mixed lacrimal fistulae were included in the review. These articles were reviewed along with their relevant cross-references. Data reviewed included demographics, presentations, investigations, management, complications and outcomes. The prevalence of congenital lacrimal fistulae is reported to be around 1 in 2000 live births. They are frequently unilateral, although familial cases tend to be bilateral. Lacrimal and systemic anomalies have been associated with lacrimal fistulae. Exact etiopathogenesis is unknown but mostly believed to be an accessory out budding from the lacrimal drainage system during embryogenesis. Treatment is indicated when significant epiphora or discharge is present and is mostly achieved by various fistulectomy techniques with or without a dacryocystorhinostomy. Congenital lacrimal fistulae are a distinct clinical entity with unique features. Surgical management can be challenging and successful outcomes are usually achieved with widely accepted protocols. PMID:27191932

  16. Congenital scoliosis: an up-to-date

    PubMed Central

    Burnei, G; Gavriliu, S; Vlad, C; Georgescu, I; Ghita, RA; Dughilă, C; Japie, EM; Onilă, A

    2015-01-01

    Congenital scoliosis represents a spinal malformation due to defects of formation, segmentation or mixed ones. It is characterized by a longitudinal and rotational imbalance. 54 patients were analyzed and 39 out of them were operated by various approaches with anterior and posterior instrumentations during 2000 and 2012. The impossibility to appoint some patients encountered in the daily practice into the known classifications, allowed us to purpose two categories of congenital scoliosis related to the predominance of spinal deviances in the coronal and transversal views. No certain etiology of congenital scoliosis has been identified until today. The susceptibility of some polygenic defects is obvious due to the presence of a sum of defects associated to most of the congenital scoliosis cases and the rarity of the presence of a unique defect. The diagnosis requires a thorough clinical and imaging examination in order to establish an individualized therapeutic strategy. The treatment of congenital scoliosis is different from the adolescent idiopathic one. Therapeutic criteria are significantly different. It is essential to assess the difference in growth of the concavity related to the convexity when choosing a particular procedure. The magnitude of the curve and the progressive rate are fundamental issues to the surgeon PMID:26351546

  17. Congenital basis of posterior fossa anomalies

    PubMed Central

    Cotes, Claudia; Bonfante, Eliana; Lazor, Jillian; Jadhav, Siddharth; Caldas, Maria; Swischuk, Leonard

    2015-01-01

    The classification of posterior fossa congenital anomalies has been a controversial topic. Advances in genetics and imaging have allowed a better understanding of the embryologic development of these abnormalities. A new classification schema correlates the embryologic, morphologic, and genetic bases of these anomalies in order to better distinguish and describe them. Although they provide a better understanding of the clinical aspects and genetics of these disorders, it is crucial for the radiologist to be able to diagnose the congenital posterior fossa anomalies based on their morphology, since neuroimaging is usually the initial step when these disorders are suspected. We divide the most common posterior fossa congenital anomalies into two groups: 1) hindbrain malformations, including diseases with cerebellar or vermian agenesis, aplasia or hypoplasia and cystic posterior fossa anomalies; and 2) cranial vault malformations. In addition, we will review the embryologic development of the posterior fossa and, from the perspective of embryonic development, will describe the imaging appearance of congenital posterior fossa anomalies. Knowledge of the developmental bases of these malformations facilitates detection of the morphological changes identified on imaging, allowing accurate differentiation and diagnosis of congenital posterior fossa anomalies. PMID:26246090

  18. Newborn screening for congenital hypothyroidism.

    PubMed

    Büyükgebiz, Atilla

    2006-11-01

    Most neonates born with congenital hypothyroidism (CH) have normal appearance and no detectable physical signs. Hypothyroidism in the newborn period is almost always overlooked and delayed diagnosis leads to the most severe outcome of CH, mental retardation, emphasizing the importance of neonatal screening. Blood spot T4 or TSH or both can be used in neonatal screening for CH. The latter, which is more sensitive, is not cost effective, so the first two are used in different programs in the world. TSH screening was shown to be more specific in the diagnosis of CH; T4 screening is more sensitive in detecting newborns especially with rare hypothalamic-pituitary hypothyroidism, but less specific with a high frequency of false positives mainly in low birth weight and premature infants. The time at which the sample is taken may vary between centers, with the majority taking blood from a heel prick after 24 hours of age to minimize the false positive high TSH due to the physiological neonatal TSH surge that elevates TSH levels and causes dynamic T4 and T3 changes in the first 1 or 2 days after birth. Early discharge of mothers postpartum has increased the ratio of false positive TSH elevations. Although transient hypothyroidism may occur frequently, all suspected infants should be treated as having CH for the first 3 years of life, taking into account the risks of mental retardation. A reevaluation after 3 years is needed in such patients. The goal of initial therapy in CH is to minimize neonatal central nervous system exposure to hypothyroidism by normalizing thyroid function, as reflected by T4 and TSH levels, as rapidly as possible. Iodine deficiency is the most important cause of CH worldwide. Iodine is essential for thyroid hormone synthesis and is present in soil, water and air. Prevention of iodine deficiency can be by iodized salt, iodized oil, iodized bread or iodine tablets. PMID:17220056

  19. Flying and congenital heart disease.

    PubMed

    Macartney, F J

    1984-03-01

    Only those congenital defects carrying a very low risk of complication (either before or after surgical correction) were considered. Atrial Septal Defects--(a) Ostium primum defects should be treated with caution either before or after surgical correction because of the risk of progressive conduction disorders and mitral regurgitation. (b) Ostium secundum defects could be considered for licensing (if the defect is small) or with surgical repair if the right ventricular systolic pressure is normal. (c) Sinus venosus defects--if too small to require surgical repair, licensing may be considered provided ambulatory electrocardiographic monitoring shows no evidence of arrhythmias. Surgery increases the risk of sino-atrial disease, thus licensing should be permitted only where there is no evidence of arrhythmia and adequate cardiological follow-up is possible. Ventricular Septal Defects--Subjects with very small defects not requiring surgical closure may be considered for licensing. Subjects who have had surgical closure have a risk of arrhythmias and should be carefully evaluated. Pulmonary Stenosis--If mild (either before or after surgery) may be licensed, but regular assessment perhaps including right heart catheterization is needed to demonstrate stability of the lesion. Persistent Ductus Arteriosus--Surgical closure should be recommended on diagnosis and need not affect licensing. Isolated Bicuspid Aortic Valve--Need not debar from licensing, but careful annual examination (with electrocardiogram 2-D echocardiography and fluroscopy ) is required to detect calcification, stenosis or regurgitation. Coarctation of aorta--Subjects who have had a repair before the age of 12 years may be considered for licensing after examination of other risk factors (blood pressure at rest and on exercise in particular). Those repaired over the age of 12 may be considered for restricted licensing if normotensive. These recommendations will need review in the light of further long

  20. MicroRNAs Expression in the Ileal Pouch of Patients with Ulcerative Colitis Is Robustly Up-Regulated and Correlates with Disease Phenotypes

    PubMed Central

    Sherman Horev, Hadas; Elad, Hofit; Baram, Liran; Issakov, Ofer; Tulchinsky, Hagit; Pasmanik-Chor, Metsada; Shomron, Noam; Dotan, Iris

    2016-01-01

    Background Gene expression alterations are associated with disease behavior in inflammatory bowel disease (IBD). microRNAs (miRNAs) are dominant in the regulation of gene expression, and may affect IBD phenotype. Our aim was to assess mucosal miRNA expression in IBD and the correlation with intestinal inflammation. Methods We performed a large-scale analysis of ileal mucosal miRNA. Biopsies were retrieved from patients with ileal Crohn’s disease (CD), unoperated ulcerative colitis (UC) patients, UC patients after pouch surgery, and normal controls (NC). Pouch UC patients were classified as having a normal pouch (NP), chronic pouchitis (CP), and Crohn’s-like disease of the pouch (CLDP). miRNA expression was analyzed by parallel massive (next-generation) sequencing (NGS). Bioinformatics tools were applied for clustering and the detection of potential targets. Results Sixty-one subjects were recruited. The ileum of unoperated UC patients was comparable with NC. There were significant miRNA expression alterations (fold change ≥2, corrected P ≤.05) in NP (n = 6), CP (n = 40) and CLDP (n = 139), but only two expression alterations were noted in CD. More than 90% of the altered miRNAs were up-regulated, and many were predicted to be associated with significantly decreased transcripts. miRNAs alterations were generally clustered with disease phenotypes. Conclusions Ileal inflammation causes increased miRNA expression. miRNA alterations correlate with IBD phenotype, apparently by controlling the down-regulation of specific mRNAs. PMID:27536783

  1. Handicapping Conditions Associated with the Congenital Rubella Syndrome.

    ERIC Educational Resources Information Center

    Vernon, McCay; And Others

    1980-01-01

    The authors discuss the incidence of impairments diagnosed among children with congenital rubella syndrome. Approximately 73 percent are hearing impaired, at least 35 percent have congenital heart disorders, and 33 percent have visual defects. (Author)

  2. Prenatal detection of congenital high airway obstruction syndrome with encephalocele

    PubMed Central

    Padmanabhan, Laxmi Devi; Nampoothiri, Sheela

    2016-01-01

    Congenital high airway obstruction syndrome (CHAOS) causes secondary morphological changes which can be detected on ultrasound. Here we report a case of congenital high airway obstruction with an occipital encephalocele detected at 23 weeks of gestation. PMID:27081227

  3. Genetics Home Reference: autosomal dominant congenital stationary night blindness

    MedlinePlus

    ... stationary night blindness autosomal dominant congenital stationary night blindness Enable Javascript to view the expand/collapse boxes. ... Close All Description Autosomal dominant congenital stationary night blindness is a disorder of the retina , which is ...

  4. Genetics Home Reference: congenital bile acid synthesis defect type 1

    MedlinePlus

    ... bile acid synthesis defect type 1 congenital bile acid synthesis defect type 1 Enable Javascript to view ... PDF Open All Close All Description Congenital bile acid synthesis defect type 1 is a disorder characterized ...

  5. Genetics Home Reference: congenital bile acid synthesis defect type 2

    MedlinePlus

    ... bile acid synthesis defect type 2 congenital bile acid synthesis defect type 2 Enable Javascript to view ... PDF Open All Close All Description Congenital bile acid synthesis defect type 2 is a disorder characterized ...

  6. What proportion of congenital abnormalities can be prevented?

    PubMed Central

    Czeizel, A E; Intôdy, Z; Modell, B

    1993-01-01

    OBJECTIVE--To estimate the proportion of preventable congenital abnormalities in Hungary. DESIGN--Analysis of available Hungarian data-bases and of the effectiveness of primary, secondary, and tertiary preventive methods. SETTING--Databases of ad hoc epidemiological studies and of the Hungarian congenital abnormality registry. MAIN OUTCOME MEASURES--Prevalence at birth and prevalence after prevention in 73 congenital abnormality types or groups. RESULTS--Preventive methods are available for 51 (70%) of the 73 congenital abnormality types or groups evaluated. The birth prevalence of all congenital abnormalities could be reduced from 65 to 26 per 1000; thus 39 per 1000 (60%) are preventable. Without congenital dislocation of the hip, which is unusually common in Hungary, the preventable proportion of congenital abnormalities is 52%. CONCLUSION--Many congenital abnormalities can be prevented, but as they do not represent a single pathological category there is no single strategy for their prevention. Images p502-a p503-a PMID:8448464

  7. Ethanolic neem (Azadirachta indica) leaf extract induces apoptosis in the hamster buccal pouch carcinogenesis model by modulation of Bcl-2, Bim, caspase 8 and caspase 3.

    PubMed

    Subapriya, R; Bhuvaneswari, V; Nagini, S

    2005-01-01

    Induction of apoptosis is one of the most active strategies in cancer chemoprevention and the ability of medicinal plants in this regard has attracted major research interest. The present study was designed to investigate the apoptosis inducing capacity of an ethanolic neem leaf extract (ENLE) during 7,12-dimethylbenz[a]anthracene (DMBA)-induced hamster buccal pouch carcinogenesis using the apoptosis-associated proteins Bcl-2, Bim, caspase 8 and caspase 3 as markers. Topical application of DMBA to the hamster cheek pouch for 14 weeks resulted in well developed squamous cell carcinomas associated with increased expression of Bcl-2 and decreased expression of Bim, caspase 8 and caspase 3. Administration of ENLE inhibited DMBA-induced hamster buccal pouch (HBP) carcinogenesis, as revealed by the absence of neoplasms, with induction of Bim and caspases 8 and 3 and inhibition of Bcl-2 expression. Our results suggest that the chemopreventive effects of ENLE may be mediated by induction of apoptosis. PMID:16436003

  8. Prenatal diagnosis of congenital mesoblastic nephroma.

    PubMed

    Do, A Young; Kim, Jung-Sun; Choi, Suk-Joo; Oh, Soo-Young; Roh, Cheong-Rae; Kim, Jong-Hwa

    2015-09-01

    Congenital mesoblastic nephroma is a rare renal tumor that is diagnosed during pregnancy and is associated with polyhydramnios, prematurity, and neonatal hypertension. Differential diagnoses include Wilms tumor, adrenal neuroblastoma, and other abdominal tumors. We report a case of congenital mesoblastic nephroma detected by prenatal ultrasonography as a large fetal renal mass with polyhydramnios at 32 weeks of gestation. Ultrasonography showed a 6×6-cm complex, solid, hyperechoic, round mass in the right kidney. At 35 weeks of gestation, the patient was admitted with preterm premature rupture of membranes and the baby was delivered vaginally. Postnatal ultrasonography and computed tomography showed a heterogeneous solid mass on the right kidney. At the end of the first week of life, a right nephrectomy was performed and subsequent pathological examination confirmed a cellular variant of congenital mesoblastic nephroma with a high mitotic count. Postoperative adjuvant chemotherapy was administered. The newborn was discharged in good condition. PMID:26430667

  9. Congenital myopathies: clinical and immunohistochemical study.

    PubMed

    Thaha, Fazil; Gayathri, N; Nalini, A

    2011-01-01

    Congenital myopathies (CMs), a group of relatively non-progressive disorders presents with weakness and hypotonia of varying severity, morphologically recognized by specific structural abnormalities within the myofiber. This report presents the clinical and Histopathological features of 40 patients with CMs. Centronuclear myopathy was the commonest (40%) followed by congenital fiber type disproportion (37.5%). Other less common CMs included: myotubular myopathy (5%), nemaline myopathy (5%), central core disease (5%), multicore disease (2.5%) and congenital myopathy with tubular aggregate (5%). Immunolabeling to desmin corresponded to morphological changes within the myofibers while vimentin was negative in all the patients. There is no combined role of these proteins in the disease process. PMID:22234203

  10. Congenital cholesteatoma: delayed diagnosis and its consequences.

    PubMed

    Goh, B S; Faizah, A R; Salina, H; Asma, A; Saim, L

    2010-09-01

    This is a retrospective review of congenital cholesteatoma cases that were managed surgically. There were 5 cases. The age of presentation ranged from 5 to 18 year old. Three patients presented with complication of the disease. Three patients had intact tympanic membrane, two had perforation at the anterior superior quadrant. All patients had cholesteatoma medial to tympanic membrane. Four cases had extensive ossicular erosion with preoperative hearing worse than 40 dB. Four cases underwent canal wall down mastoid surgery and one underwent canal wall up surgery. One patient had recurrence which required revision surgery. In conclusion, congenital cholesteatoma presented late due to the silent nature of disease in its early stage. Extensive disease, ossicular destruction with risk of complication at presentation were marked in our study. Hence, more aggressive surgical intervention is recommended in the management of congenital cholesteatoma. PMID:21939167

  11. Congenital narrowing of the cervical spinal canal.

    PubMed Central

    Kessler, J T

    1975-01-01

    The clinical and laboratory findings in six patients with congenital narrowing of the cervical spinal canal and neurological symptoms are described. A variable age of onset and an entirely male occurrence were found. Signs and symptoms of spinal cord dysfunction predominated in all but one patient. Symptoms were produced in five patients by increased physical activity alone. Congenital narrowing of the cervical spinal canal may result in cord compression without a history of injury and occasionally without evidence of significant bony degenerative changes. The clinical features may be distinguishable from those found in cervical spondylosis without congenital narrowing. Intermittent claudication of the cervical spinal cord appears to be an important feature of this syndrome. Surgery improved four out of five people. PMID:1219087

  12. Deprivation amblyopia and congenital hereditary cataract.

    PubMed

    Mansouri, Behzad; Stacy, Rebecca C; Kruger, Joshua; Cestari, Dean M

    2013-01-01

    Amblyopia is a neurodevelopmental disorder of vision associated with decreased visual acuity, poor or absent stereopsis, and suppression of information from one eye.(1,2) Amblyopia may be caused by strabismus (strabismic amblyopia), refractive error (anisometropic amblyopia), or deprivation from obstructed vision (deprivation amblyopia). 1 In the developed world, amblyopia is the most common cause of childhood visual impairment, 3 which reduces quality of life 4 and also almost doubles the lifetime risk of legal blindness.(5, 6) Successful treatment of amblyopia greatly depends on early detection and treatment of predisposing disorders such as congenital cataract, which is the most common cause of deprivational amblyopia. Understanding the genetic causes of congenital cataract leads to more effective screening tests, early detection and treatment of infants and children who are at high risk for hereditary congenital cataract. PMID:24138041

  13. Male genitoplasty for 46 XX congenital adrenal hyperplasia patients presenting late and reared as males

    PubMed Central

    Sharma, Shilpa; Gupta, Devendra K.

    2012-01-01

    Aim: To evaluate the clinical profile and management of 46 XX Congenital Adrenal Hyperplasia (CAH) patients presenting with severe virilization and assigned a male gender. Materials and Methods: Of 173 children diagnosed with CAH at the Pediatric Intersex Clinic since 1980, seven children with CAH presented late with severe virilization and were reared as males. All of them were assigned the male sex with removal of the female adnexa. Six were treated with male genitoplasty. Appropriate hormonal supplementation was offered after puberty. Results: The mean age at presentation was 14.2 years (7 – 21). Six patients had presented after puberty, only one at seven years of age. Staged male genitoplasty comprising of chordee correction, male urethroplasty, and bilateral testicular prosthesis was performed. The female adnexa (uterus, ovaries, most of the upper vagina, and the fallopian tubes) were removed. The mental makeup was masculine in six and bigender in one. Bilateral mastectomy was performed at puberty in all. Hormonal treatment comprised of glucocorticoids and testosterone. Six patients were comfortable with the outcome of the masculinizing genitoplasty. One had a short-sized phallus. One had repeated attacks of urinary tract infection arising from the retained lower vaginal pouch. Social adjustments were good in all, except in one who had a bigender mental makeup. Conclusion: CAH patients with severe virilization presenting late and reared as males are extremely rare. However, the assigned gender can be retained adequately as males, meeting the socioeconomic compulsions of the society. The results are satisfactory following appropriate surgical procedures and hormonal supplementation. PMID:23226638

  14. Pulmonary Hypertension in Congenital Heart Disease: Beyond Eisenmenger Syndrome.

    PubMed

    Krieger, Eric V; Leary, Peter J; Opotowsky, Alexander R

    2015-11-01

    Patients with adult congenital heart disease have an increased risk of developing pulmonary hypertension. There are several mechanisms of pulmonary hypertension in patients with adult congenital heart disease, and understanding them requires a systematic approach to define the patient's hemodynamics and physiology. This article reviews the updated classification of pulmonary hypertension in patients with adult congenital heart disease with a focus on pathophysiology, diagnostics, and the evaluation of pulmonary hypertension in special adult congenital heart disease populations. PMID:26471823

  15. Air pollution and congenital heart defects.

    PubMed

    Agay-Shay, Keren; Friger, Michael; Linn, Shai; Peled, Ammatzia; Amitai, Yona; Peretz, Chava

    2013-07-01

    Environmental factors such as ambient air pollution have been associated with congenital heart defects. The aim of this study was to investigate the association between gestational exposure to air pollution and the risk of congenital heart defects. We conducted a registry-based cohort study with a total of 135,527 live- and still-births in the Tel-Aviv region during 2000-2006. We used a Geographic Information System-based spatiotemporal approach with weekly inverse distance weighting modeling to evaluate associations between gestational exposure to ambient air pollution during weeks 3-8 of pregnancy and the risk for congenital heart defects. The following pollutants were studied: carbon monoxide, nitrogen-dioxide, ozone, sulfur-dioxide and particulate matter with aerodynamic diameter smaller than 10 μm and 2.5 μm (PM10, PM2.5 respectively). Logistic models, adjusted for socio-demographic covariates were used to evaluate the associations. We found that maternal exposure to increased concentrations of PM10 was associated with multiple congenital heart defects (adjusted OR 1.05, 95% CI: 1.01 to 1.10 for 10 μg/m(3) increment). An inverse association was observed between concentrations of PM2.5 and isolated patent ductus arteriosus (adjusted OR 0.78, 95% CI: 0.68 to 0.91 for 5 µg/m(3) increment). Sensitivity analyses showed that results were consistent. Generally there were no evidence for an association between gaseous air pollutants and congenital heart defects.Our results for PM10 and congenital heart defects confirm results from previous studies. The results for PM2.5 need further investigations. PMID:23623715

  16. Congenital Absence of the Internal Carotid Artery

    SciTech Connect

    Florio, Francesco; Balzano, Silverio; Nardella, Michele; Strizzi, Vincenzo; Cammisa, Mario; Bozzini, Vincenzo; Catapano, Giuseppe; D'Angelo, Vincenzo

    1999-01-15

    We report three cases of congenital absence of an internal carotid artery (ICA), diagnosed incidentally by digital subtraction angiography. The analysis of the cases is based on the classification of segmental ICA agenesis proposed by Lasjaunias and Berenstein. Usually the patients with this rare vascular anomaly are asymptomatic; some may have symptoms related to cerebrovascular insufficiency, compression by enlarged intracranial collateral vessels, or complications associated with cerebral aneurysms. Diagnosis of congenital absence of ICA is made by skull base computed tomography (CT) scan, CT and magnetic resonance angiography, and conventional or digital subtraction angiography.

  17. Congenital angiomyoma of the tongue: case report

    PubMed Central

    Kim, Y-H; Jang, Y-W; Pai, H; Kim, S-G

    2010-01-01

    Angiomyomas of the oral cavity are rare benign vascular neoplasms. In particular, the congenital form has not been reported before in the English language literature. We present a congenital angiomyoma of the tongue that was found on the posterior middle of the tongue in an infant. On MRI, the mass showed an isointense signal to muscle on the T1 weighted image and a slightly hyperintense signal on the T2 weighted image. Immunohistochemically, tumour cells were positive to desmin and smooth muscle actin, but negative to vimentin and S100. The treatment was surgical excision and no recurrence was found during the 26 month follow-up period. PMID:20841464

  18. Recurrent congenital fibrosarcoma with heart metastases.

    PubMed

    Lohi, Olli; Vornanen, Martine; Kähkönen, Marketta; Vettenranta, Kim; Parto, Katriina; Arola, Mikko

    2012-07-01

    Congenital fibrosarcomas are malignant tumors that arise in soft tissues. In infants this unique tumor does not commonly metastasize, even though there may be local recurrences. We report here a boy who had congenital fibrosarcoma in his right foot, which was completely excised at the age of 3 days. Four months later, a solitary encapsulated metastasis emerged in thoracic chest wall, which was operated. During adjuvant chemotherapy he developed histologically confirmed fibrosarcoma metastases in the heart. After extended treatment with cyclophosphamide/topotecan and gemcitabine/docetaxel, the heart tumors disappeared and he has been in complete remission for 3 years. PMID:22217490

  19. Pediatric congenital vertebral artery arteriovenous malformation.

    PubMed

    Shownkeen, Harish; Bova, Davide; Chenelle, Andrew G; Origitano, Thomas C

    2003-05-01

    Vertebral arteriovenous fistulas are rare in children and the congenital form has been seldom reported in the literature. Prior to using endovascular therapy techniques, only surgery was the main treatment. The most common endovascular treatment is through the use of detachable balloons. This report describes the clinical and radiological findings of a congenital vertebral artery fistula in a 20-month-old child. Balloons could not be safely employed; therefore, embolization was performed with Guglielmi detachable microcoils. We review the history and treatment of these lesions, their clinical presentation, and imaging features, including their outcome, with particular attention to the pediatric population. PMID:12695869

  20. Congenital myasthenic syndromes and transient myasthenia gravis.

    PubMed

    Gajda, Anna; Szabó, Hajnalka; Gergev, Gyurgyinka; Karcagi, Veronika; Szabó, Nóra; Endreffy, Emoke; Túri, Sándor; Sztriha, László

    2013-05-30

    Hypotonia in the neonatal period and early infancy is a common clinical finding. It can be caused by various heterogeneous disorders of different origin which might lead to diagnostic difficulties. Disorders of the neuromuscular junction, such as congenital myasthenic syndromes and neonatal transient myasthenia gravis are among the aetiologies. We report on a case of congenital myasthenia caused by mutation in the long cytoplasmic loop of the epsilon subunit of the acetylcholine receptor and a neonate of a myasthenic mother diagnosed with transient myasthenia gravis. PMID:23909021

  1. Congenital nephrotic syndrome. Gallium-67 imaging

    SciTech Connect

    Trepashko, D.W.; Gelfand, M.J.; Pan, C.C.

    1988-11-01

    Congenital nephrotic syndrome is a rare disorder. Heavy proteinuria, hypoalbuminemia, and edema occur during the first 3 months of life. Initial cases were reported from Finland and sporadic cases have occurred elsewhere. Finnish cases demonstrated an autosomal recessive inheritance pattern; currently, Finnish and non-Finnish types are recognized. The clinical course consists of failure to thrive, frequent infections, declining renal function, and early death by age 4 years from sepsis or uremia. Recently renal transplantation has improved the prognosis of patients with this disease. An abnormal Ga-67 scan in a case of congenital nephrotic syndrome is presented.

  2. Mediastinal bronchogenic cyst mimicking congenital lobar emphysema.

    PubMed

    Arun, Sumitha; Kumar, Manish; Ross, Benjamin Jeyanth

    2016-01-01

    Bronchogenic cyst (BC) is a rare congenital malformation of the lung. Most patients remain asymptomatic until adulthood while some are symptomatic in the first few years of life. However, symptoms in newborn period are rare. We report a case of a 3-day-old preterm baby with respiratory distress diagnosed as congenital lobar emphysema on chest X-ray. A CT scan revealed a mediastinal cyst causing obstructive lobar emphysema. The cyst was excised and pathological examination was suggestive of BC. PMID:27609589

  3. [Congenital hip dysplasia, screening and therapy].

    PubMed

    Kolb, A; Windhager, R; Chiari, C

    2015-11-01

    Congenital hip dysplasia and hip dislocation are relatively common pathological conditions of the musculoskeletal system in infants. An early and certain diagnosis can now be achieved by sonographic hip screening within the framework of screening examination programs. This early diagnostic procedure in infants is essential particularly for a conservative treatment strategy. Therefore, apart from possessing in-depth knowledge, training of the examiner in specialist courses is of central importance. This article presents an overview of the entity of congenital hip dysplasia and hip dislocation, the diagnostics and treatment with special emphasis on recent developments. PMID:26489825

  4. NEW BORN SCREENING IN PREVENTING CONGENITAL HYPOTHYROIDISM.

    PubMed

    Ali, Mazahir; Zia, Aisha; Siddiqui, Saad Ebrahim

    2015-01-01

    Congenital Hypothyroidism is one of the most common preventable causes of mental retardation which is highly prevalent in our society due to lack of a national neonatal screening program, lack of education of the parents, increased consanguinity, and lack of suspicion from doctor's leads to delayed diagnosis and an increased incidence of congenital hypothyroidism in our society. This Menace can be easily tackled with a mass neonatal screening program and effective legislation which would make sure that serious efforts are being made to eradicate this preventable disease from Pakistan. PMID:27004363

  5. Congenital syphilis: subtle presentation of fulminant disease.

    PubMed

    Bennett, M L; Lynn, A W; Klein, L E; Balkowiec, K S

    1997-02-01

    The incidence of congenital syphilis has experienced a fourfold to fivefold increase in 6 years. It is a completely preventable disease whose clinical spectrum ranges from asymptomatic infection, to fulminant sepsis, to death. Congenital syphilis was diagnosed in a 6-week-old infant whose mother was negative for the disease by prenatal screen. The otherwise well child presented with a generalized, papulosquamous eruption of 3 weeks' duration but within hours multisystem failure developed from overwhelming treponemal sepsis. Factors related to increased incidence, problems in serodiagnosis, manifestations of the early versus late forms of the disease, and recommendations for evaluation and treatment are illustrated by this patient and are discussed. PMID:9039217

  6. Toward postnatal reversal of ocular congenital malformations

    PubMed Central

    Sahel, José-Alain; Marazova, Katia

    2013-01-01

    Aniridia is a panocular disorder that severely affects vision in early life. Most cases are caused by dominantly inherited mutations or deletions of the PAX6 gene, which encodes a transcription factor that is essential for the development of the eye and the central nervous system. In this issue of the JCI, Gregory-Evans and colleagues demonstrate that early postnatal topical administration of an ataluren-based formulation reverses congenital malformations in the postnatal mouse eye, providing evidence that manipulation of PAX6 after birth may lead to corrective tissue remodeling. These findings offer hope that ataluren administration could be a therapeutic paradigm applicable to some major congenital eye defects. PMID:24355915

  7. Pulmonary Hypertension and Congenital Heart Disease.

    PubMed

    Roth, Todd S; Aboulhosn, Jamil A

    2016-08-01

    Pulmonary arterial hypertension in congenital heart disease (PAH-CHD) is a frequent complication in adults with congenital heart disease. Regardless of etiology, the optimal treatment strategy for this difficult population is challenging. The new frontier of targeted PAH therapies has demonstrated improved functional capacity in the various phenotypes of PAH-CHD, with work currently in progress scrutinizing outcomes. In those who fail conventional medical therapy, heart and heart-lung (block) transplantation become the final therapeutic options, with the role of ventricular assist devices and the total artificial heart still under investigation in this group. PMID:27443136

  8. Canine congenital portosystemic shunts: Disconnections dissected.

    PubMed

    Van den Bossche, L; van Steenbeek, F G

    2016-05-01

    Canine congenital portosystemic shunts (CPSS) are vascular anomalies that connect the portal vein with the systemic circulation, therefore bypassing the hepatic parenchyma. Portosystemic shunts exist in two different subtypes: extrahepatic and intrahepatic. This congenital disorder is also described in mice, cat, sheep and man. Research has been focused on pathophysiology, diagnostics and treatment of CPSS and this has resulted in increased knowledge, although the aetiology of the disease remains unclear. This review focuses on the aetiology and genetic basis of both intra- and extrahepatic shunts. PMID:27061656

  9. Acquired Congenital Malalignment of the Great Toenails

    PubMed Central

    Decker, Ashley; Scher, Richard K.; Avarbock, Andrew

    2016-01-01

    Congenital malalignment is the lateral deviation of the nail plate along the longitudinal axis due to the lateral rotation of the nail matrix. The nail plate grows out in ridges caused by repeated microtrauma to the nail. Common complications include onychomycosis, Pseudomonas infection and acute or chronic paronychia. Treatment options range from conservative management to surgical options including realignment and nail matrixectomy. Congenital malalignment usually presents in infancy or childhood, but we present two cases of acquired malalignment occurring in the teenage years. PMID:27171597

  10. Radiographic findings in congenital lead poisoning

    SciTech Connect

    Pearl, M.; Boxt, L.M.

    1980-07-01

    Because lead crosses the placenta throughout pregnancy, the fetus is at risk for lead poisoning. A full term, asymptomatic child was born with congenital lead poisoning secondary to maternal pica. Radiographic findings of a dense cranial vault, lead lines, and delayed skeletal and deciduous dental development were noted at birth. After chelation therapy, when the patient was seven months old, radiographs revealed normal skeletal maturation. Tooth eruption did not occur until 15 months of age. Newborn infants with these radiographic findings should be screened for subclinical, congenital lead poisoning.

  11. Acquired Congenital Malalignment of the Great Toenails.

    PubMed

    Decker, Ashley; Scher, Richard K; Avarbock, Andrew

    2016-02-01

    Congenital malalignment is the lateral deviation of the nail plate along the longitudinal axis due to the lateral rotation of the nail matrix. The nail plate grows out in ridges caused by repeated microtrauma to the nail. Common complications include onychomycosis, Pseudomonas infection and acute or chronic paronychia. Treatment options range from conservative management to surgical options including realignment and nail matrixectomy. Congenital malalignment usually presents in infancy or childhood, but we present two cases of acquired malalignment occurring in the teenage years. PMID:27171597

  12. Adult colonic intussusception: a case report.

    PubMed

    Sasatomi, T; Oriishi, T; Nakano, R; Nozoe, Y; Tanaka, T; Horiuchi, H; Noake, T; Takeuchi, K; Tsuji, Y; Shirouzu, K

    2001-01-01

    Intussusception accounts for almost all cases of intestinal obstruction in children. In contrast, intussusception in adults is relatively rare. An 86-year-old Japanese female with rectal bleeding came to our hospital via ambulance. At first, colonoscopy findings revealed the sigmoid colon cancer. Ultrasonography showed a hypoechoic mass with a multiple concentric ring sign. Computed tomography showed a round fluid-filled cystic structure. Colon contrast studies demonstrated stenosis in the rectosigmoid colon. A laparotomy was performed. The sigmoid colon was intussuscepted to the rectosigmoid colon. We employed both rectosigmoid and sigmoid colon resection. The resected specimen showed that the disease was advanced sigmoid colon cancer with ulcer formation due to an ischemic change. Tumor was 4.5 cm x 2.0 cm in size. The disease was histopathologically diagnosed as advanced sigmoid colon cancer, well-differentiated adenocarcinoma. We report here a case of adult intussusception due to the sigmoid colon cancer. PMID:11501502

  13. Symptoms of Autism among Children with Congenital Deafblindness

    ERIC Educational Resources Information Center

    Dammeyer, Jesper

    2014-01-01

    Associations between congenital deafness or blindness and autism have been found. The main consequences of congenital sensory impairment, being barriers for communication, language and social interaction development, may lead to symptoms of autism. To date only few studies have been reported concerning individuals with congenital deafblindness.…

  14. 38 CFR 4.9 - Congenital or developmental defects.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 false Congenital or developmental defects. 4.9 Section 4.9 Pensions, Bonuses, and Veterans' Relief DEPARTMENT OF VETERANS AFFAIRS SCHEDULE FOR RATING DISABILITIES General Policy in Rating § 4.9 Congenital or developmental defects. Mere congenital or developmental defects,...

  15. Undiagnosed congenital hypothyroidism in a newborn treated with dopamine infusion.

    PubMed

    Shi, Xuanxing; Sun, Yueling; Qiang, Rong

    2015-06-01

    Medications administered during the neonatal period may mask the diagnosis of congenital hypothyroidism. Herein, we report a case of undiagnosed congenital hypothyroidism while the infant was on treatment with dopamine. Given the inhibitory effect of dopamine on thyroid-stimulating hormone, a high index of suspicion for potential congenital hypothyroidism is needed in such neonates. PMID:25724212

  16. Colon volvulus displaced into the chest – right-sided posttraumatic hernia or congenital malformation?

    PubMed Central

    Dębek, Wojciech; Matuszczak, Ewa

    2016-01-01

    We present the case of a 13.5-year-old girl who was admitted to the Pediatric Surgery Department from the Pediatric Department of a district hospital, where she stayed because of stomachache and vomiting. Interview revealed blunt injury of the epigastrium a week ago. Chest X-ray revealed a loss of the right diaphragmatic outline, irregular radiolucency on the right side of the chest, collapsed right lung and mediastinal displacement to the left. The patient was operated on, and the surgery revealed herniation of the intestines and half of the stomach into the defect of the right dome of the diaphragm. The patient made an uneventful postoperative recovery. A small innate defect of the diaphragm can remain asymptomatic and undiagnosed as long as there is no herniation of the abdominal organs into the chest.

  17. Automated classification of optical coherence tomography images for the diagnosis of oral malignancy in the hamster cheek pouch

    PubMed Central

    Pande, Paritosh; Shrestha, Sebina; Park, Jesung; Serafino, Michael J.; Gimenez-Conti, Irma; Brandon, Jimi; Cheng, Yi-Shing; Applegate, Brian E.; Jo, Javier A.

    2014-01-01

    Abstract. Most studies evaluating the potential of optical coherence tomography (OCT) for the diagnosis of oral cancer are based on visual assessment of OCT B-scans by trained experts. Human interpretation of the large pool of data acquired by modern high-speed OCT systems, however, can be cumbersome and extremely time consuming. Development of image analysis methods for automated and quantitative OCT image analysis could therefore facilitate the evaluation of such a large volume of data. We report automated algorithms for quantifying structural features that are associated with the malignant transformation of the oral epithelium based on image processing of OCT data. The features extracted from the OCT images were used to design a statistical classification model to perform the automated tissue diagnosis. The sensitivity and specificity of distinguishing malignant lesions from benign lesions were found to be 90.2% and 76.3%, respectively. The results of the study demonstrate the feasibility of using quantitative image analysis algorithms for extracting morphological features from OCT images to perform the automated diagnosis of oral malignancies in a hamster cheek pouch model. PMID:25162909

  18. Diabetes Is Reversed in a Murine Model by Marginal Mass Syngeneic Islet Transplantation Using a Subcutaneous Cell Pouch Device

    PubMed Central

    Pepper, Andrew R.; Pawlick, Rena; Gala-Lopez, Boris; MacGillivary, Amanda; Mazzuca, Delfina M.; White, David J. G.; Toleikis, Philip M.; Shapiro, A. M. James

    2015-01-01

    Background Islet transplantation is a successful β-cell replacement therapy for selected patients with type 1 diabetes mellitus. Although high rates of early insulin independence are achieved routinely, long-term function wanes over time. Intraportal transplantation is associated with procedural risks, requires multiple donors, and does not afford routine biopsy. Stem cell technologies may require potential for retrievability, and graft removal by hepatectomy is impractical. There is a clear clinical need for an alternative, optimized transplantation site. The subcutaneous space is a potential substitute, but transplantation of islets into this site has routinely failed to reverse diabetes. However, an implanted device, which becomes prevascularized before transplantation, may alter this equation. Methods Syngeneic mouse islets were transplanted subcutaneously within Sernova Corp's Cell Pouch (CP). All recipients were preimplanted with CPs 4 weeks before diabetes induction and transplantation. After transplantation, recipients were monitored for glycemic control and glucose tolerance. Results Mouse islets transplanted into the CP routinely restored glycemic control with modest delay and responded well to glucose challenge, comparable to renal subcapsular islet grafts, despite a marginal islet dose, and normoglycemia was maintained until graft explantation. In contrast, islets transplanted subcutaneously alone failed to engraft. Islets within CPs stained positively for insulin, glucagon, and microvessels. Conclusions The CP is biocompatible, forms an environment suitable for islet engraftment, and offers a potential alternative to the intraportal site for islet and future stem cell therapies. PMID:26308506

  19. Fast and slow ion diffusion processes in lithium ion pouch cells during cycling observed with fiber optic strain sensors

    NASA Astrophysics Data System (ADS)

    Sommer, Lars Wilko; Kiesel, Peter; Ganguli, Anurag; Lochbaum, Alexander; Saha, Bhaskar; Schwartz, Julian; Bae, Chang-Jun; Alamgir, Mohamed; Raghavan, Ajay

    2015-11-01

    Cell monitoring for safe capacity utilization while maximizing pack life and performance is a key requirement for effective battery management and encouraging their adoption for clean-energy technologies. A key cell failure mode is the build-up of residual electrode strain over time, which affects both cell performance and life. Our team has been exploring the use of fiber optic (FO) sensors as a new alternative for cell state monitoring. In this present study, various charge-cycling experiments were performed on Lithium-ion pouch cells with a particular class of FO sensors, fiber Bragg gratings (FBGs), that were externally attached to the cells. An overshooting of the volume change at high SOC that recovers during rest can be observed. This phenomenon originates from the interplay between a fast and a slow Li ion diffusion process, which leads to non-homogeneous intercalation of Li ions. This paper focuses on the strain relaxation processes that occur after switching from charge to no-load phases. The correlation of the excess volume and subsequent relaxation to SOC as well as temperature is discussed. The implications of being able to monitor this phenomenon to control battery utilization for long life are also discussed.

  20. Laparoscopic restorative proctocolectomy with ileal pouch-anal anastomosis for Peutz-Jeghers syndrome with synchronous rectal cancer.

    PubMed

    Zhong, Min-Er; Niu, Bei-Zhan; Ji, Wu-Yang; Wu, Bin

    2016-06-14

    We report on a patient diagnosed with Peutz-Jeghers syndrome (PJS) with synchronous rectal cancer who was treated with laparoscopic restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA). PJS is an autosomal dominant syndrome characterized by multiple hamartomatous polyps in the gastrointestinal tract, mucocutaneous pigmentation, and increased risks of gastrointestinal and nongastrointestinal cancer. This report presents a patient with a 20-year history of intermittent bloody stool, mucocutaneous pigmentation and a family history of PJS, which together led to a diagnosis of PJS. Moreover, colonoscopy and biopsy revealed the presence of multiple serried giant pedunculated polyps and rectal adenocarcinoma. Currently, few options exist for the therapeutic management of PJS with synchronous rectal cancer. For this case, we adopted an unconventional surgical strategy and ultimately performed laparoscopic restorative proctocolectomy with IPAA. This procedure is widely considered to be the first-line treatment option for patients with ulcerative colitis or familial adenomatous polyposis. However, there are no previous reports of treating PJS patients with laparoscopic IPAA. Since the operation, the patient has experienced no further episodes of gastrointestinal bleeding and has demonstrated satisfactory bowel control. Laparoscopic restorative proctocolectomy with IPAA may be a safe and effective treatment for patients with PJS with synchronous rectal cancer. PMID:27298573

  1. Laparoscopic restorative proctocolectomy with ileal pouch-anal anastomosis for Peutz-Jeghers syndrome with synchronous rectal cancer

    PubMed Central

    Zhong, Min-Er; Niu, Bei-Zhan; Ji, Wu-Yang; Wu, Bin

    2016-01-01

    We report on a patient diagnosed with Peutz-Jeghers syndrome (PJS) with synchronous rectal cancer who was treated with laparoscopic restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA). PJS is an autosomal dominant syndrome characterized by multiple hamartomatous polyps in the gastrointestinal tract, mucocutaneous pigmentation, and increased risks of gastrointestinal and nongastrointestinal cancer. This report presents a patient with a 20-year history of intermittent bloody stool, mucocutaneous pigmentation and a family history of PJS, which together led to a diagnosis of PJS. Moreover, colonoscopy and biopsy revealed the presence of multiple serried giant pedunculated polyps and rectal adenocarcinoma. Currently, few options exist for the therapeutic management of PJS with synchronous rectal cancer. For this case, we adopted an unconventional surgical strategy and ultimately performed laparoscopic restorative proctocolectomy with IPAA. This procedure is widely considered to be the first-line treatment option for patients with ulcerative colitis or familial adenomatous polyposis. However, there are no previous reports of treating PJS patients with laparoscopic IPAA. Since the operation, the patient has experienced no further episodes of gastrointestinal bleeding and has demonstrated satisfactory bowel control. Laparoscopic restorative proctocolectomy with IPAA may be a safe and effective treatment for patients with PJS with synchronous rectal cancer. PMID:27298573

  2. Ontogenetic shifts in brain scaling reflect behavioral changes in the life cycle of the pouched lamprey Geotria australis

    PubMed Central

    Salas, Carlos A.; Yopak, Kara E.; Warrington, Rachael E.; Hart, Nathan S.; Potter, Ian C.; Collin, Shaun P.

    2015-01-01

    Very few studies have described brain scaling in vertebrates throughout ontogeny and none in lampreys, one of the two surviving groups of the early agnathan (jawless) stage in vertebrate evolution. The life cycle of anadromous parasitic lampreys comprises two divergent trophic phases, firstly filter-feeding as larvae in freshwater and secondly parasitism as adults in the sea, with the transition marked by a radical metamorphosis. We characterized the growth of the brain during the life cycle of the pouched lamprey Geotria australis, an anadromous parasitic lamprey, focusing on the scaling between brain and body during ontogeny and testing the hypothesis that the vast transitions in behavior and environment are reflected in differences in the scaling and relative size of the major brain subdivisions throughout life. The body and brain mass and the volume of six brain structures of G. australis, representing six points of the life cycle, were recorded, ranging from the early larval stage to the final stage of spawning and death. Brain mass does not increase linearly with body mass during the ontogeny of G. australis. During metamorphosis, brain mass increases markedly, even though the body mass does not increase, reflecting an overall growth of the brain, with particularly large increases in the volume of the optic tectum and other visual areas of the brain and, to a lesser extent, the olfactory bulbs. These results are consistent with the conclusions that ammocoetes rely predominantly on non-visual and chemosensory signals, while adults rely on both visual and olfactory cues. PMID:26283894

  3. Of humans and hamsters: the hamster buccal pouch carcinogenesis model as a paradigm for oral oncogenesis and chemoprevention.

    PubMed

    Nagini, S

    2009-10-01

    Oral squamous cell carcinoma (OSCC), a common malignancy worldwide, is an important contributor to the overall international cancer burden. Squamous cell carcinomas (SCCs) induced by 7,12-dimethylbenz[a]anthracene (DMBA) in the HBP reiterate many of the features observed in human OSCCs. The major risk factors associated with human oral cancer such as tobacco, betel quid and alcohol promote HBP carcinogenesis. SCCs induced by DMBA in the cheek pouch of Syrian hamsters are morphologically and histologically similar to human OSCC. Like human oral carcinogenesis, HBP carcinogenesis is a multistep process that involves sequential progression from hyperplasia to invasive carcinoma through varying degrees of dysplasia. In addition, HBP tumours express several biochemical and molecular markers that are also expressed in human OSCC. Multiple signaling pathways are dysfunctional in both human and hamster OSCCs. In particular, cell proliferation, apoptosis and angiogenesis are intricately interlinked in malignant transformation of the HBP mucosa by DMBA. The HBP carcinogenesis model is the best-known animal system for intervention by chemopreventive agents because of easy accessibility for examination, and follow-up of lesions. A number of synthetic and natural products have been documented to exhibit chemopreventive efficacy in the HBP model. Chemoprevention studies in the HBP model can serve as a crucial link in the potential efficacy assessment of candidate agents for oral cancer prevention and therapy. PMID:19538166

  4. Raman spectroscopic detection of early stages in DMBA-induced tumor evolution in hamster buccal pouch model: an exploratory study

    NASA Astrophysics Data System (ADS)

    Ghanate, Avinash D.; Kumar, G.; Talathi, Sneha; Maru, G. B.; Krishna, C. Murali

    2010-12-01

    Oral cancers are the serious health problem in developing as well as developed world, and more so in India and other south Asian countries. Survival rate of these cancers, despite advances in treatment modalities are one of the poorest which is attributed to lack of reliable screening and early detection methods. The hamster buccal pouch (HBP)carcinogenesis model closely mimics human oral cancers. Optical spectroscopy methods are sensitive enough to detect subtle biochemical changes and thus hold great potential in early detection of cancers. However, efficacy of these techniques in classifying of sequential evolution of tumors has never been tested. Therefore, in this study, we have explored the feasibility of Raman spectroscopic classification of different stages of cancers in hamster model. Strong vibrational modes of lipids (1440, 1654, and 1746 cm-1) are seen in control tissue spectra, whereas strong protein bands are seen in spectra of DMBA treated tissues. These differences were exploited to classify control and treated tissues using Linear Discriminant Analysis (LDA), Principle Component Analysis (PCA)-Limit test, Factorial Discriminant Analysis (FDA), Quadratic Discriminant Analysis (QDA), PLS-DA and non- linear decision tree methods. All these techniques have shown good classification between spectra of different stages of tumor evolution and results were further successfully verified by leave-one-out and single blinded methods. Thus findings of this study, first of its kind,demonstrate the feasibility of Raman spectroscopic detection of early changes in tumor evolution.

  5. Raman spectroscopic detection of early stages in DMBA-induced tumor evolution in hamster buccal pouch model: an exploratory study

    NASA Astrophysics Data System (ADS)

    Ghanate, Avinash D.; Kumar, G.; Talathi, Sneha; Maru, G. B.; Krishna, C. Murali

    2011-08-01

    Oral cancers are the serious health problem in developing as well as developed world, and more so in India and other south Asian countries. Survival rate of these cancers, despite advances in treatment modalities are one of the poorest which is attributed to lack of reliable screening and early detection methods. The hamster buccal pouch (HBP)carcinogenesis model closely mimics human oral cancers. Optical spectroscopy methods are sensitive enough to detect subtle biochemical changes and thus hold great potential in early detection of cancers. However, efficacy of these techniques in classifying of sequential evolution of tumors has never been tested. Therefore, in this study, we have explored the feasibility of Raman spectroscopic classification of different stages of cancers in hamster model. Strong vibrational modes of lipids (1440, 1654, and 1746 cm-1) are seen in control tissue spectra, whereas strong protein bands are seen in spectra of DMBA treated tissues. These differences were exploited to classify control and treated tissues using Linear Discriminant Analysis (LDA), Principle Component Analysis (PCA)-Limit test, Factorial Discriminant Analysis (FDA), Quadratic Discriminant Analysis (QDA), PLS-DA and non- linear decision tree methods. All these techniques have shown good classification between spectra of different stages of tumor evolution and results were further successfully verified by leave-one-out and single blinded methods. Thus findings of this study, first of its kind,demonstrate the feasibility of Raman spectroscopic detection of early changes in tumor evolution.

  6. A new species of pouched octopus, Cistopus Gray, 1849 (Cephalopoda: Octopodidae) from the southwest coast of India.

    PubMed

    Sreeja, Vijayamma; Norman, Mark D; Kumar, Appukuttannair Biju

    2015-01-01

    Octopuses of the genus Cistopus Gray, 1849 are commercially valuable catches in the cephalopod fisheries of India. The primary and unique diagnostic character of this genus is the possession of eight small mucous pouches embedded in the oral faces of the webs between the bases of each arm. Historically only a single species of Cistopus, C. indicus, had been reported from Indian waters. In reviewing the octopod fauna off the Kerala coast, we have detected three species of Cistopus, of which one is described here as a new species. Cistopus platinoidus sp. nov. is distinct from Cistopus species described to date (C. indicus, C. taiwanicus and C. chinensis) on the basis of sucker counts, the number and position of enlarged suckers in males, and presence/absence of a calamus. Our studies of catch composition of Kerala octopod fisheries indicate a higher diversity of target species than previously suspected, including a number of undescribed species. Taxonomic resolution and collation of biological and distributional data are required for effective monitoring and management of these valuable fisheries. PMID:26701522

  7. Characterizing and modeling mechanical properties and onset of short circuit for three types of lithium-ion pouch cells

    NASA Astrophysics Data System (ADS)

    Sahraei, Elham; Meier, Joseph; Wierzbicki, Tomasz

    2014-02-01

    Three types of lithium ion pouch cells ranging from small consumer electric cells with LiCoO2 cathode to large (electric vehicle size) cells with nanophosphate chemistry were tested under several local and global compression scenarios, including compression between two flat plates and local indentation with a flat cylindrical punch, a conical punch, and three hemispherical punches. Load, displacement, temperature, and voltage were recorded in all tests. The punch displacements were stopped when a drop in force and voltage of the cell, as well as a rise in temperature indicated a short circuit in the cell. Finite element models were developed for each cell type. Two tests were used for calibration of the constitutive properties of each type of cell, and the remaining tests served for the validation of the computational model. The models successfully predicted the load displacement relation and contour of deformations in the cells. Additionally, the models closely predict the force and punch displacement corresponding to the onset of short circuit in the cell. The current results are building confidence in robustness and accuracy of the present calibration and modeling approach.

  8. Predicting heat generation in a lithium-ion pouch cell through thermography and the lumped capacitance model

    NASA Astrophysics Data System (ADS)

    Bazinski, S. J.; Wang, X.

    2016-02-01

    A technique is developed that combines a new closed-form analytical form of the lumped capacitance model (LCM) and thermography to predict the internal heat generation of a lithium-ion pouch cell. A series of tests is conducted to validate the new technique using a polyimide film heater mounted as a vertical plate in front of an infrared camera. A programmable power supply is used to subject the heater to three types of pre-defined heat profiles. The technique performs very well in reconstructing each known heat profile. The technique is then used to predict the rate of heat generation of a 14.5 Ah lithium iron phosphate cell under different rates of discharge. Compared to the heat rate measured by a calorimeter, the model predicts an average heat rate within 10% for moderate C-rates and lower. For C-rates higher than 2C, a simple correction algorithm is applied due to cell temperature gradients becoming more pronounced.

  9. Detection of oral squamous-cell cancer and precancerous lesions by fluorescence imaging in a hamster cheek-pouch model

    NASA Astrophysics Data System (ADS)

    Lam, Stephen; Kluftinger, A. M.; Hung, J.; Davis, N. L.; Quenville, N. F.; Palcic, Branko

    1993-03-01

    The role of non-skin phototoxic dose of Photofrin in the detection of dysplasia and carcinoma in situ was assessed in a small animal model of oral squamous cell cancer (SCC). Nine,10-dimethyl 1,2-benzanthracene (DMBA) impregnated cotton sutures, covered with a silicone sheath, were sewn into the hamster cheek pouch to produce dysplasia, carcinoma in situ, and invasive cancer. The yield of SCC was 83% by 20 weeks. Fluorescence imaging was performed using a specially designed device that exploits differences of fluorescence properties of normal, precancerous, and cancerous tissues with and without Photofrin. The fluorescence was induced by a helium-cadmium laser (442 nm) and then measured at two different wavelengths by an image intensified camera. Computed images using a mathematical transformation of fluorescence data were then displayed on a video monitor. Areas with dysplasia and both in situ and invasive cancers could be clearly delineated from the adjacent normal tissues. Lesions as small as 2 mm in diameter could be identified. Because of the presence of endogenous porphyrins, the addition of a non-skin phototoxic dose of Photofrin (0.25 mg/kg iv) did not enhance the signal to noise ratio. Our results suggest that fluorescence imaging can accurately detect both precancerous and cancerous lesions in the oral mucosa without exogenous porphyrins. It may have an important role as a non-invasive, clinical diagnostic tool in oropharyngeal cancer.

  10. Comparison of proctocolectomy and ileal pouch-anal anastomosis to colectomy and ileorectal anastomosis in familial adenomatous polyposis.

    PubMed

    Koskenvuo, L; Mustonen, H; Renkonen-Sinisalo, L; Järvinen, H J; Lepistö, A

    2015-06-01

    Prophylactic surgical options for familial adenomatous polyposis (FAP) are either colectomy and ileorectal anastomosis (IRA) or proctocolectomy and ileal pouch-anal anastomosis (IPAA). The aim of this study was to analyse the short-term and long-term outcomes of these two operative techniques. All patients with FAP in Finland have been prospectively recorded in a database since 1963 were retrospectively reviewed in this analysis. Altogether 140 (61%) colectomies with IRA and 88 (39%) proctocolectomies with IPAA have been performed. Complications occurred in 28 (21%) patients after IRA and in 26 (30%) patients after IPAA. There were 15 (11%) severe complications for IRA and 5 (6%) for IPAA. Twenty-one (15%) patients of the IRA group ended up in conventional ileostomy whereas 3 (3.4%) patients of the IPAA group had their ileal reservoir converted to an ileostomy (p = 0.01). Cumulative survival for IRA was lower than for the IPAA (p = 0.03), but if accounting only for operations made after the IPAA era had commenced, there was no significant difference. IPAA was associated with improved long-term survival without an increase in postoperative complications. The risk of death after colectomy and IRA seemed to be predominantly related to the remaining risk of rectal cancer. Therefore, we favour proctocolectomy with IPAA as the prophylactic surgical procedure for FAP with intermediate or severe polyposis. PMID:25504366

  11. Ultrasonographic appearance of colon taeniasis.

    PubMed

    Fabijanić, D; Giunio, L; Ivani, N; Fabijanić, A; Mirić, D; Kardum, D

    2001-03-01

    We present the case of a 50-year-old woman with abdominal pain, nausea, loss of appetite, and frequent stools in whom the routine ultrasonographic examination demonstrated a double-reflective, ribbon-like structure in the lumen of the initial segment of the ascending colon, which suggested colon taeniasis. Because the initial parasitologic analysis yielded negative results and application of albendazol did not have any therapeutic effect, the diagnosis was confirmed by barium enema and subsequently by parasitologic examination of proglottids passed in the stool after application of niclosamide. The double-reflective, ribbon-like structure in the lumen of the intestine seems to be specific to the ultrasonographic appearance of intestinal taeniasis. Transcutaneous ultrasonography of the gastrointestinal tract, performed as a screening method before conventional radiologic or endoscopic examination, can point to the ultimate diagnosis of colon taeniasis. PMID:11270533

  12. Subclinical Congenital Cytomegalovirus Infection and Hearing Impairment

    ERIC Educational Resources Information Center

    Dahle, Arthur J.; And Others

    1974-01-01

    When the hearing sensitivity of children with subclinical congenital cytomegalovirus infection was evaluated and compared with that of a group of matched control subjects, nine of the 18 infected subjects were found to have some hearing loss, ranging from slight high-frequency impairments to a severe-to-profound unilateral loss. (MYS)

  13. Genetics Home Reference: severe congenital neutropenia

    MedlinePlus

    ... Alter BP, Link DC, Stein S, Rodger E, Bolyard AA, Aprikyan AA, Bonilla MA, Dror Y, Kannourakis G, Newburger PE, ... PubMed or Free article on PubMed Central Schäffer AA, Klein C. Genetic heterogeneity in severe congenital neutropenia: ...

  14. Gastroesophageal reflux and congenital gastrointestinal malformations.

    PubMed

    Marseglia, Lucia; Manti, Sara; D'Angelo, Gabriella; Gitto, Eloisa; Salpietro, Carmelo; Centorrino, Antonio; Scalfari, Gianfranco; Santoro, Giuseppe; Impellizzeri, Pietro; Romeo, Carmelo

    2015-07-28

    Although the outcome of newborns with surgical congenital diseases (e.g., diaphragmatic hernia; esophageal atresia; omphalocele; gastroschisis) has improved rapidly with recent advances in perinatal intensive care and surgery, infant survivors often require intensive treatment after birth, have prolonged hospitalizations, and, after discharge, may have long-term sequelae including gastro-intestinal comorbidities, above all, gastroesophageal reflux (GER). This condition involves the involuntary retrograde passage of gastric contents into the esophagus, with or without regurgitation or vomiting. It is a well-recognized condition, typical of infants, with an incidence of 85%, which usually resolves after physiological maturation of the lower esophageal sphincter and lengthening of the intra-abdominal esophagus, in the first few months after birth. Although the exact cause of abnormal esophageal function in congenital defects is not clearly understood, it has been hypothesized that common (increased intra-abdominal pressure after closure of the abdominal defect) and/or specific (e.g., motility disturbance of the upper gastrointestinal tract, damage of esophageal peristaltic pump) pathological mechanisms may play a role in the etiology of GER in patients with birth defects. Improvement of knowledge could positively impact the long-term prognosis of patients with surgical congenital diseases. The present manuscript provides a literature review focused on pathological and clinical characteristics of GER in patients who have undergone surgical treatment for congenital abdominal malformations. PMID:26229394

  15. NATIONAL CONGENITAL RUBELLA SYNDROME REGISTRY (NCRSR)

    EPA Science Inventory

    The system compiles information on all U.S. congenital rubella syndrome cases. The registry includes cases reported to CDC since 1969. Information collected includes demographic data, laboratory results, and disease symptoms. These data are used to calculate incidence rates and o...

  16. Why prevent, diagnose and treat congenital toxoplasmosis?

    PubMed Central

    McLeod, Rima; Kieffer, Francois; Sautter, Mari; Hosten, Tiffany; Pelloux, Herve

    2009-01-01

    Evidence that prevention, diagnosis and treatment of toxoplasmosis is beneficial developed as follows: antiparasitic agents abrogate Toxoplasma gondii tachyzoite growth, preventing destruction of infected, cultured, mammalian cells and cure active infections in experimental animals, including primates. They treat active infections in persons who are immune-compromised, limit destruction of retina by replicating parasites and thereby treat ocular toxoplasmosis and treat active infection in the fetus and infant. Outcomes of untreated congenital toxoplasmosis include adverse ocular and neurologic sequelae described in different countries and decades. Better outcomes are associated with treatment of infected infants throughout their first year of life. Shorter intervals between diagnosis and treatment in utero improve outcomes. A French approach for diagnosis and treatment of congenital toxoplasmosis in the fetus and infant can prevent toxoplasmosis and limit adverse sequelae. In addition, new data demonstrate that this French approach results in favorable outcomes with some early gestation infections. A standardized approach to diagnosis and treatment during gestation has not yet been applied generally in the USA. Nonetheless, a small, similar experience confirms that this French approach is feasible, safe, and results in favorable outcomes in the National Collaborative Chicago-based Congenital Toxoplasmosis Study cohort. Prompt diagnosis, prevention and treatment reduce adverse sequelae of congenital toxoplasmosis. PMID:19430661

  17. Congenital cardiac anomalies in an English bulldog.

    PubMed

    McConkey, Marina J

    2011-11-01

    A 4-year-old male castrated English bulldog was referred to the Atlantic Veterinary College for evaluation of exercise intolerance, multiple syncopal episodes, and a grade IV/VI heart murmur. The dog was shown to have 3 congenital cardiac anomalies: atrial septal defect, mitral valve dysplasia, and subaortic stenosis. Medical management consisted of exercise restriction, atenolol, pimobendan, and taurine. PMID:22547849

  18. Prevalence of congenital muscular dystrophy in Italy

    PubMed Central

    Graziano, Alessandra; Bianco, Flaviana; D'Amico, Adele; Moroni, Isabella; Messina, Sonia; Bruno, Claudio; Pegoraro, Elena; Mora, Marina; Astrea, Guja; Magri, Francesca; Comi, Giacomo P.; Berardinelli, Angela; Moggio, Maurizio; Morandi, Lucia; Pini, Antonella; Petillo, Roberta; Tasca, Giorgio; Monforte, Mauro; Minetti, Carlo; Mongini, Tiziana; Ricci, Enzo; Gorni, Ksenija; Battini, Roberta; Villanova, Marcello; Politano, Luisa; Gualandi, Francesca; Ferlini, Alessandra; Muntoni, Francesco; Santorelli, Filippo Maria; Bertini, Enrico; Pane, Marika

    2015-01-01

    Objective: We provide a nationwide population study of patients with congenital muscular dystrophy in Italy. Methods: Cases were ascertained from the databases in all the tertiary referral centers for pediatric neuromuscular disorders and from all the genetic diagnostic centers in which diagnostic tests for these forms are performed. Results: The study includes 336 patients with a point prevalence of 0.563 per 100,000. Mutations were identified in 220 of the 336 (65.5%). The cohort was subdivided into diagnostic categories based on the most recent classifications on congenital muscular dystrophies. The most common forms were those with α-dystroglycan glycosylation deficiency (40.18%) followed by those with laminin α2 deficiency (24.11%) and collagen VI deficiency (20.24%). The forms of congenital muscular dystrophy related to mutations in SEPN1 and LMNA were less frequent (6.25% and 5.95%, respectively). Conclusions: Our study provides for the first time comprehensive epidemiologic information and point prevalence figures for each of the major diagnostic categories on a large cohort of congenital muscular dystrophies. The study also reflects the diagnostic progress in this field with an accurate classification of the cases according to the most recent gene discoveries. PMID:25653289

  19. Congenital Tuberculosis Complicated by Hemophagocytic Lymphohistiocytosis.

    PubMed

    Osowicki, Joshua; Wang, Shiqi; McKenzie, Christopher; Marshall, Carolyn; Gard, Jye; Ke Juin, Wong; Steer, Andrew C; Connell, Tom G

    2016-01-01

    We present the case of a male infant with congenital tuberculosis in a nonendemic setting complicated by hemophagocytic lymphohistiocytosis, who was treated successfully with antituberculous therapy and corticosteroids. We review the pediatric literature concerning the unusual association of these 2 rare conditions. PMID:26398869

  20. Genetics of Nonsyndromic Congenital Hearing Loss.

    PubMed

    Egilmez, Oguz Kadir; Kalcioglu, M Tayyar

    2016-01-01

    Congenital hearing impairment affects nearly 1 in every 1000 live births and is the most frequent birth defect in developed societies. Hereditary types of hearing loss account for more than 50% of all congenital sensorineural hearing loss cases and are caused by genetic mutations. HL can be either nonsyndromic, which is restricted to the inner ear, or syndromic, a part of multiple anomalies affecting the body. Nonsyndromic HL can be categorised by mode of inheritance, such as autosomal dominant (called DFNA), autosomal recessive (DFNB), mitochondrial, and X-linked (DFN). To date, 125 deafness loci have been reported in the literature: 58 DFNA loci, 63 DFNB loci, and 4 X-linked loci. Mutations in genes that control the adhesion of hair cells, intracellular transport, neurotransmitter release, ionic hemeostasis, and cytoskeleton of hair cells can lead to malfunctions of the cochlea and inner ear. In recent years, with the increase in studies about genes involved in congenital hearing loss, genetic counselling and treatment options have emerged and increased in availability. This paper presents an overview of the currently known genes associated with nonsyndromic congenital hearing loss and mutations in the inner ear. PMID:26989561