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Intestinal atresia

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https://www.readbyqxmd.com/read/28407396/a-further-family-of-stromme-syndrome-carrying-cenpf-mutation
#1
Ferda Ozkinay, Tahir Atik, Esra Isik, Zeliha Gormez, Mahmut Sagiroglu, Ozlem Atan Sahin, Nergul Corduk, Huseyin Onay
Stromme syndrome is a rare genetic disorder characterized by microcephaly, anterior ocular chamber anomalies, and "apple peel" type jejunal atresia. Here, we report a Stromme syndrome family with two affected siblings with a homozygous truncating frameshift mutation in CENPF. A 3-month-old girl was hospitalized due to prenatally diagnosed microcephaly, microphthalmia, and dysmorphological features. The history of a previous child with the same findings in addition to "apple peel" intestinal atresia had been noted...
April 13, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28381137/jejunal-occlusion-caused-by-heterotopic-gastric-and-duodenal-mucosa
#2
Olivia Seyde, Giacomo Puppa, Philippe Morel, Barbara E Wildhaber, Anne-Laure Rougemont
We report a rare case of late complication of a complex intestinal malformation. At day 1 of life, a baby girl underwent resection of an atretic jejunal segment, associated with an enteric duplication harboring foci of gastric and duodenal heterotopia. After an asymptomatic period of 19 years, the patient presented with acute bowel obstruction. Recurrence of the jejunal occlusion at the previous anastomotic site was caused by mucosa hyperplasia in association with heterotopic gastric and duodenal tissue. A Wnt/β-catenin pathway deregulation was hypothesized but not confirmed by CTNNB1 exon 3 mutation analysis...
February 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28371283/outcomes-in-patients-undergoing-robotic-reconstructive-uterovaginal-anastomosis-of-congenital-cervical-and-vaginal-atresia
#3
Ying Zhang, Yisong Chen, Keqin Hua
OBJECTIVE: To introduce our experience of robotic surgery of reconstructive uterovaginal anastomosis and operative outcomes in congenital cervical and vaginal atresia patients. METHODS: Clinical observation and follow-up of four patients with congenital cervical and vaginal atresia who underwent robotic reconstruction of cervix and vagina by SIS (small intestinal submucosa, SIS) graft. RESULTS: Average patient age was 13.8 ± 2.2. Patients complained of severe periodic abdominal pain...
March 29, 2017: International Journal of Medical Robotics + Computer Assisted Surgery: MRCAS
https://www.readbyqxmd.com/read/28365103/race-and-outcomes-in-gastroschisis-repair-a-nationwide-analysis
#4
Ye Kyung Song, Omar Nunez Lopez, Hemalkumar B Mehta, Fredrick J Bohanon, Yesenia Rojas-Khalil, Kanika A Bowen-Jallow, Ravi S Radhakrishnan
BACKGROUND: The incidence of gastroschisis has increased 30% between the periods 1995-2005 and 2006-2012, with the largest increase in Black neonates born to Black mothers younger than 20years old. OBJECTIVE: Racial disparities in peri- and post-operative outcomes have been previously identified in several types of adult and pediatric surgical patients. Is there an association between race and clinical outcomes and healthcare resource utilization in neonates with gastroschisis? METHODS: Retrospective study using national administrative data from the Kid's Inpatient Database (KID) from 2006, 2009, and 2012 for neonates (age<28days) with gastroschisis...
March 11, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28286566/gastrointestinal-manifestations-of-mitochondrial-disorders-a-systematic-review
#5
REVIEW
Josef Finsterer, Marlies Frank
Mitochondrial disorders (MIDs) due to respiratory-chain defects or nonrespiratory chain defects are usually multisystem conditions [mitochondrial multiorgan disorder syndrome (MIMODS)] affecting the central nervous system (CNS), peripheral nervous system, eyes, ears, endocrine organs, heart, kidneys, bone marrow, lungs, arteries, and also the intestinal tract. Frequent gastrointestinal (GI) manifestations of MIDs include poor appetite, gastroesophageal sphincter dysfunction, constipation, dysphagia, vomiting, gastroparesis, GI pseudo-obstruction, diarrhea, or pancreatitis and hepatopathy...
January 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/28248794/intestinal-metaplasia-of-the-esophagus-in-children-with-esophageal-atresia
#6
Helen Hsieh, Adam Frenette, Laurent Michaud, Usha Krishnan, Dorothée Bouron Dal-Soglio, Frederic Gottrand, Christophe Faure
OBJECTIVES: Patients with esophageal atresia/tracheo-esophageal fistula (EA-TEF) can develop Barrett's esophagus as a long-term consequence of their condition. Intestinal metaplasia (IM), a risk factor for developing adenocarcinoma of the esophagus, has not been well characterized in the pediatric population. METHODS: Retrospective review of EA-TEF patients followed at three academic pediatric centers between the years 1997 and 2014. RESULTS: Among 542 children and adolescents, 1...
February 28, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28241003/-duodenal-diaphragm-diagnosis-in-a-school-aged-child-and-minimally-invasive-treatment-case-report
#7
Andrea Barrueto Barrera, Sofia Santelices Baeza, Francisco Miranda Labra, David Schnettler Rodríguez
Duodenal atresia is the third cause of intrinsic intestinal obstruction in the neonatal period. Typical presentation includes early-onset vomiting of gastric or bilious content, abdominal distension and poor weight gain. If the obstruction is incomplete, as in a perforated duodenal diaphragm, presenting symptoms tend to appear later and be nonspecific, so diagnosis is usually delayed. We present the case of a 9-year-old girl with a history of biliary postprandial vomiting from the infancy period, without any impact on the nutritional status, managed symptomatically...
January 31, 2017: Medwave
https://www.readbyqxmd.com/read/28239322/intestinal-barrier-integrity-and-function-in-infants-with-cholestasis
#8
Nagla H Abu Faddan, Tahra M K Sherif, Omnia A Mohammed, Khalid A Nasif, Ebtesam M El Gezawy
BACKGROUND/AIMS: The safety of the human body is maintained by effective monitoring of the mucosal surface integrity and protection against potentially harmful compounds. This function of the gut called intestinal barrier function can be affected by cholestasis and the absence of bile in the intestinal lumen. We aimed to determine whether the gut barrier integrity is impaired in infants with cholestasis by evaluation of the intestinal fatty acid binding proteins (I-FABP) and ileal bile acid binding protein (I-BABP) as markers of intestinal epithelial cell damage and plasma D-lactate level as a marker of gut wall permeability...
January 2017: Intestinal Research
https://www.readbyqxmd.com/read/28234123/the-development-of-total-parenteral-nutrition
#9
Don K Nakayama
The first patient to receive complete nourishment of a patient by intravenous infusion independent of the alimentary tract was an infant girl born with near-total small bowel atresia. Total parenteral nutrition, the intravenous infusion of nutrients, has been attempted since Harvey's description of the circulatory system in the early 17th century. The modern era of parenteral nutrition began in the early 20th century, when infusions of glucose, plasma, and emulsified fat into humans proved feasible. Robert Elman, working in the 1930s and 1940s, demonstrated that carefully prepared protein hydrolysates could be safely infused intravenously and incorporated by the body...
January 1, 2017: American Surgeon
https://www.readbyqxmd.com/read/28225489/immunoregulation-effects-of-different-%C3%AE-%C3%AE-t-cells-and-toll-like-receptor-signaling-pathways-in-neonatal-necrotizing-enterocolitis
#10
Lei Hui, Yi Dai, Zhi Guo, Jiahui Zhang, Fang Zheng, Xiangli Bian, Zhimin Wu, Qin Jiang, Miaomiao Guo, Ke Ma, Jinping Zhang
The aim of the study was to observe cytokine and T-cell-related toll-like-receptor (TLR) changes in intestinal samples of neonatal necrotizing enterocolitis patients.Four necrotic bowels were collected from neonatal NEC patients with gestational ages of 28 to 29 weeks in our hospital, whereas 4 neonatal patients who underwent intestinal atresia surgery served as the controls. Intestinal flora was examined and IL-1, IL-2, IL-4, IL-6, IL-8, IL-10, TNF-α, IFN-γ, and IL-17 expressions in resected intestine samples, as well as in isolated gamma delta T (γδT) cells, were analyzed immunohistochemically and via quantitative RT-PCR...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28224094/cystic-meconium-peritonitis-with-jejunoileal-atresia-is-it-associated-with-unfavorable-outcome
#11
Kin Wai Edwin Chan, Kim Hung Lee, Hei Yi Vicky Wong, Siu Yan Bess Tsui, Yuen Shan Wong, Kit Yi Kristine Pang, Jennifer Wai Cheung Mou, Yuk Him Tam
AIM: To compare the outcome between patients with jejunoileal atresia (JIA) associated with cystic meconium peritonitis (CMP) and patients with isolated JIA (JIA without CMP). METHODS: A retrospective study was conducted for all neonates with JIA operated in our institute from January 2005 to January 2016. Demographics including the gestation age, sex, birth weight, age at operation, the presence of associated syndrome was recorded. Clinical outcome including the type of operation performed, operative time, the need for reoperation and mortality were studied...
February 8, 2017: World Journal of Clinical Pediatrics
https://www.readbyqxmd.com/read/28209955/-the-complications-of-intestinal-stoma-in-children
#12
S V Minaev, N I Bykov, A V Isaeva, A V Kachanov, E A Tovkan, N V Filip'yeva, I N Gerasimenko
AIM: To investigate the complications of intestinal stoma in children and to develop measures for decrease of their incidence. MATERIAL AND METHODS: The study included 152 children with congenital and acquired gastrointestinal pathology requiring surgical treatment with the imposition of intestinal stoma. Atresia of intestinal tube was observed in 28 (18.4%) children, meconium ileus - in 10 (6.6%) cases, Hirschsprung's disease - in 11 (7.2%)cases, anorectal malformations - in 39 (25...
2017: Khirurgiia
https://www.readbyqxmd.com/read/28205719/p-27-development-of-intestinal-metaplasia-in-children-with-esophageal-atresia-tracheoesophageal-fistula
#13
H H Hsieh, A Frenette, A Aspirot, C Faure
No abstract text is available yet for this article.
April 1, 2016: Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus
https://www.readbyqxmd.com/read/28188037/determinants-of-outcomes-in-patients-with-simple-gastroschisis
#14
Fouad Youssef, Jean-Martin Laberge, Pramod Puligandla, Sherif Emil
PURPOSE: We analyzed the determinants of outcomes in simple gastroschisis (GS) not complicated by intestinal atresia, perforation, or necrosis. METHODS: All simple GS patients enrolled in a national prospective registry from 2005 to 2013 were studied. Patients below the median for total parenteral nutrition (TPN) duration (26days) and hospital stay (34days) were compared to those above. Univariate and multivariate logistic and linear regression analyses were employed using maternal, patient, postnatal, and treatment variables...
January 28, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28185632/risk-factors-and-outcomes-of-tapering-surgery-for-small-intestinal-dilatation-in-pediatric-short-bowel-syndrome
#15
Maria Hukkinen, Reetta Kivisaari, Antti Koivusalo, Mikko P Pakarinen
BACKGROUND: In remains unclear why in some short bowel syndrome (SBS) patients, the remaining small bowel (SB) dilates excessively leading to requirement of tapering surgery. METHODS: Among SBS children, we retrospectively analyzed risk factors for tapering surgery with logistic regression and compared the outcome of operated patients (n=16) to those managed conservatively (n=44) with Cox proportional hazards regression. RESULTS: SBS was caused by necrotizing enterocolitis (NEC) (n=31), SB atresia (SBA) (n=13), midgut volvulus (n=12), or gastroschisis (n=4)...
January 31, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28168985/predicting-time-to-full-enteral-nutrition-in-children-after-significant-bowel-resection
#16
Jessica Gonzalez-Hernandez, Purvi Prajapati, Gerald Ogola, Nandini Channabasappa, Barbara Drews, Hannah G Piper
PURPOSE: Parenteral nutrition (PN) contributes to considerable morbidity in children after significant bowel resection. This study evaluates the utility of clinical variables in predicting time to independence from PN. METHODS: After IRB approval, a retrospective review (1999-2012) of 71 children who were on PN for >6weeks after intestinal resection and successfully weaned was performed. Clinical characteristics were evaluated to determine the relationship to time to full enteral nutrition...
January 29, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28083491/gastrointestinal-duplication-presenting-as-neonatal-intestinal-obstruction-an-experience-of-15-years-at-tertiary-care-centre
#17
Kamal Nain Rattan, Shruti Bansal, Aastha Dhamija
BACKGROUND: Gastrointestinal tract (GIT) duplications are one of the rare congenital anomalies and can occur in any portion of the gastrointestinal tract but are more commonly encountered in small intestine. The duplication cysts cause symptoms like abdominal mass and intestinal obstruction requiring surgery or may remain asymptomatic. We are reporting our 15 years' experience duplication cysts presenting in neonates. METHODS: It is a retrospective study undertaken in the department of pediatric surgery between 2001 and 2015 for GIT duplications in neonates...
January 2017: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/28060781/detection-of-an-infant-s-duodenal-atresia-by-milk-scan
#18
Ha Wu, Zhiheng Huang, Min Ji, Yiwei Li, Ruifang Zhao
Duodenal membranous atresia is a rare congenital disease. Here, we present a case of 6-month-old girl referred to us for repeated vomiting since birth. Milk scan was performed, and the results revealed a typical "double-bubble" sign. In addition, only very little activity was shown in the small intestine even at the end of the scintigraphy. These results led to the diagnosis of duodenal atresia, which was subsequently confirmed by gastroenterography and laparotomy. Our case demonstrated the potential value of milk scan in the diagnosis of this congenital disorder...
February 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28051043/experience-with-neonatal-hydrometrocolpos-in-the-niger-delta-area-of-nigeria-upsurge-or-increased-recognition
#19
Philemon Ekemenye Okoro, C Obiorah, C E Enyindah
BACKGROUND: Hydrometrocolpos (HMC) is a clinical condition in which there is a cystic distension of the vagina (hydrocolpos), uterus and sometimes, fallopian tubes (hydrosalpinx) with fluid. This study described our experience with cases of HMC seen in our practice, and highlighted the flare in our practice. PATIENTS AND METHODS: A retrospective study of cases of HMC managed at the University of Port Harcourt Teaching Hospital between September 2010 and August 2012...
October 2016: African Journal of Paediatric Surgery: AJPS
https://www.readbyqxmd.com/read/28003230/prenatal-intestinal-volvulus-look-for-cystic-fibrosis
#20
Taieb Chouikh, Nicolas Mottet, Christelle Cabrol, Yann Chaussy
Intestinal volvulus is a life-threatening emergency requiring prompt surgical management. Prenatal intestinal volvulus is rare, and most are secondary to intestinal atresia, mesenteric defect or without any underlying cause. Cystic fibrosis (CF) is known to cause digestive tract disorders. After birth, 10-15% of newborns with CF may develop intestinal obstruction within a few days of birth because of meconial ileus.(1) This obstruction is a result of dehydrated thickened meconium obstructing the intestinal lumen...
December 21, 2016: BMJ Case Reports
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