Genotype correlates with the natural history of severe bile salt export pump deficiency Highlights NAPPED is the largest global database of genotyped patients with BSEP deficiency. The genotype of patients with BSEP deficiency predicts survival with native liver. Genotype predicts long-term benefit of interruption of enterohepatic circulation. Serum bile acids can be a surrogate marker for long-term outcome. Treatment of patients with BSEP deficiency should be based on genotype.

No abstract

Background An inverse association has been suggested between celiac disease (CD) and Helicobacter pylori (Hp) infection in children; however, there are inconsistent data. The purpose of this multi‐center study is to evaluate the association between Hp and CD in childhood. Methods Children who underwent endoscopy between July 2016 and November 2017 in four pediatric gastroenterology centers were included in the study. Patients with a history of previous Hp eradication, antibiotic or acid‐suppressive drug therapy in the last 4 weeks, and any underlying chronic disease were excluded. The presence of Hp infection was confirmed by both histopathology and the rapid urease test. The ones who had the diagnosis of CD were compared with the children who underwent endoscopy during the same period and had another diagnosis. Duodenal histopathology of children with CD was categorized according to the modified Marsh classification. Results Of 3056 endoscopies performed in the study period, 2484 cases were eligible for the study. A total of 482 CD patients (mean age: 9.71 ± 4.63 years, 58.5% girls) and 2060 controls (mean age: 9.92 ± 4.66 years, 54.6% girls) were included in the study. The rate of Hp infection was significantly lower in CD group (26.3% vs 50.1%, P < .01). The difference was prominent even in children younger than 6 years old (P < .01). There was no correlation between Hp infection and the modified Marsh scores in CD (P > .05). Conclusion In this cross‐sectional study, where Hp infection is common even in the pediatric population, the frequency of Hp infection was significantly lower in children with CD compared with the controls. Systematic cohort studies are necessary to clarify causal association between Hp infection and the development of celiac disease.

OBJECTIVE:The aim of this study was to review the management of pediatric cases of vitelline duct pathology (VDP) detected surgically or incidentally during the neonatal period and the outcomes.METHODS:The data of newborns who were symptomatic and underwent VDP resection or who were incidentally diagnosed with VDP at a single institution between 1985 and 2015 were retrospectively analyzed in terms of age, sex, clinical features, treatment, perioperative findings, ectopic tissue pathology, and postoperative follow-up information.RESULTS:Among the 36 newborns enrolled in this study, 26 were male and 10 were female (2.6:1). The median weight was 2400 g (range: 800–3090 g). In 16 cases (14 males and 2 females; 7:1) the VDP was surgically repaired. Pathological evaluation indicated that 43% (n=7) of the cases had ectopic gastric mucosa. VDP was incidentally discovered in 12 males and 8 females (1.5:1). VDP was removed in 10 cases (50%) and left intact in others. Ectopic gastric mucosa was observed in 10% of the VDP removal cases. Ectopic gastric tissue was more prevalent in the surgical VDP cases than in the incidentally discovered and VDP removal cases (p<0.05). Male predominance was greater in the surgically repaired cases than in the incidentally discovered cases (p<0.05). One patient whose VDP was discovered incidentally was admitted 3 years later with obstruction due to intussusception caused by Meckel’s diverticulum, and 1 patient was admitted with rectal bleeding at 11 years of age.CONCLUSION:Symptomatic VDP in the newborn demonstrates a significant gender difference. Symptomatic cases are more likely to have ectopic gastric tissue than non-symptomatic cases. Incidentally detected cases without removal should be followed closely for future complications.

No abstract

No abstract

Abstract:Background: Hepatic artery aneurysm (HAA) is a rare clinical entity that can lead to potentially life threatening complications. We reported our personal experience of 4 cases, in which we used different procedures. Methods: The fi rst case had a pseudo-aneurysm involving the right hepatic artery. The second case had a pseudoaneurysm, which was localized distal to the accidentally ligated right hepatic artery from the previous cholecystectomy operation. The third case had multiple aneurysms with accompanying dissecting abdominal aortic aneurysm. The fourth case had a pseudo-aneurysm originating from the proper hepatic artery. A covered stent was successfully placed in the case 1. In the second case, the right hepatic artery was ligated distal to the aneurysm. In the third case, vascular structures were not appropriate for vascular reconstruction, and a covered stent placement and embolization were unsuccessful. In the fourth case, ligation of the proper hepatic artery and cholecystectomy was performed.Results: The third case with multiple aneurysms died from multi-organ failure due to sepsis. The remaining cases (case 1, 2, and 4) are disease free and alive. Conclusion: HAAs are more commonly observed clinical entities, and their treatment should be handled for each patient separately. Computerized tomography-Angiography and intraoperative Doppler ultrasound are useful radio-diagnostics for determination of aneurysm and planning the operative procedure (Fig. 5, Ref. 15). Full Text in PDF www.elis.sk.

Heterotopic gastric mucosa (HGM) can be found anywhere from the mouth to the rectum. Moreover, anecdotal presence at airways, umbilicus, gallbladder, biliary tree, urinary bladder, and scrotum was reported (1-3). Here we present a 15-year-old girl referred to our clinic with recurrent hematemesis, because of duodenojejunal HGM. Her physical examination was normal except pallor (serum hemoglobin level: 7.2 g/dL). Gastroduodenoscopy revealed a huge, snake-like polypoid lesion obliterating the lumen of the distal duodenum and extending farther (Fig. 1A). Oxyntic mucosa was detected on biopsy specimens by hematoxylin-eosin staining (Fig. 1B). Computed tomography showed a 12 Â 5 cm mobile mass in the lumen of the fourth part of the duodenum. At laparotomy, a mass was found starting approximately from 4 cm proximal to the ligament of Treitz and continued to 8 cm distal of Treitz, and was resected (Fig. 2).HGM is a rare hereditary or acquired condition, usually seen in adults. Children would present with recurrent hematemesis or melena (4). Residual acid and gastrin-secreting tissues are commonly located on the duodenum; however, presence on jejunum, colon, and esophagus has all been described previously (5). Pediatric gastroenterologists should be aware of HGM in patients with upper gastrointestinal (GI) bleeding.