Severe Gastric Outlet Obstruction in a Child with Ectopic Pancreas

Ertem, Deniz; Tutar, Engin; Cam, Sebahat; Uğraş, Meltem; Pehlivanoğlu, Ender

doi:10.1097/01.mpg.0000221895.51748.44

Cited by 33 publications

(16 citation statements)

References 13 publications

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“…The age at presentation ranged from less than 16 weeks to 10 years ( 18 , 22 ) with the majority of patients diagnosed within the first year of life ( 12 , 13 , 17 , 21 , 22 ). The presenting features included jaundice ( 11 , 12 , 18 ), growth retardation or failure to thrive ( 10 – 12 , 16 , 18 , 19 , 21 , 22 ), cholestatic disease ( 10 – 14 , 17 – 19 , 21 ), cardiovascular complications ( 10 – 13 , 16 – 19 , 21 , 22 ), hepatomegaly ( 11 , 18 , 22 ), splenomegaly ( 18 , 22 ), pruritus ( 10 – 13 , 16 , 18 , 19 , 21 ), xanthomas ( 10 – 13 , 18 , 21 ), abnormal liver function results ( 10 – 12 , 17 , 18 , 21 , 22 ), abnormal facies ( 10 – 13 , 16 , 19 , 21 , 22 ), and renal disease ( 10 , 12 – 14 , 16 , 21 , 22 ). The most commonly presented features are shown in Table 1 .…”

Section: Resultsmentioning

confidence: 99%

Systematic Review

Kamath

Baker

Houwen

et al. 2018

J. pediatr. gastroenterol. nutr.

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Background and Aim:Alagille syndrome (ALGS) is an inherited multisystem disorder typically manifesting as cholestasis, and potentially leading to end-stage liver disease and death. The aim of the study was to perform the first systematic review of the epidemiology, natural history, and burden of ALGS with a focus on the liver component.Methods:Electronic databases and proceedings from key congresses were searched in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2009 guidelines. This analysis included publications reporting epidemiology, natural history, economic burden or health-related quality of life (HRQoL) outcomes in patients with ALGS.Results:Of 525 screened publications, 20 met the inclusion criteria. Liver-related features included cholestasis (87%–100% of patients), jaundice (66%–85%), and cirrhosis (44%–95%). Between 15% and 47% of patients underwent liver transplantation and 4% to 14% received partial biliary diversion. Pruritus affected the majority of patients (59%–88%, of whom up to 45% had severe pruritus) and manifested during the first 10 years of life. Children with ALGS had significantly impaired HRQoL compared with healthy controls and those with other diseases. Itching was the symptom that most affected children with ALGS. No study assessed the economic burden of ALGS.Conclusions:Our findings consolidate information on the clinical course of ALGS, and highlight gaps in knowledge, most notably the absence of any research on the economic consequences of the disease. Further research is needed to establish the incidence of genetically confirmed ALGS. Disease-specific tools are also needed to improve the measurement of symptoms, such as itching, and better understand the impact of ALGS on HRQoL.

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Section: Resultsmentioning

confidence: 99%

Systematic Review

Kamath

Baker

Houwen

et al. 2018

J. pediatr. gastroenterol. nutr.

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“…The current investigation highlights the complexity of this question. Because LT can be problematic for many of these disorders, identifying the appropriate patient for the appropriate operation would be ideal . A prospective, multicenter, multinational registry would be most likely to provide further insights into this important issue.…”

Section: Discussionmentioning

confidence: 99%

“…Because LT can be problematic for many of these disorders, identifying the appropriate patient for the appropriate operation would be ideal. (33)(34)(35)(36) A prospective, multicenter, multinational registry would be most likely to provide further insights into this important issue. Such a registry will need to be multicentered and will need to carefully account for the type of surgery, genotype of the disease, and stage of disease at the time of surgery.…”

Section: Discussionmentioning

confidence: 99%

Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis

et al. 2017

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To evaluate the efficacy of non-transplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 Alagille syndrome (ALGS), 16 Familial Intrahepatic Cholestasis-1 (FIC1), 18 Bile Salt Export Pump (BSEP) disease, & 4 others with low γ-glutamyl transpeptidase disease (levels <100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBD), 11 ileal exclusions (IE) and 7 gallbladder-to-colon diversion (GBC). Serum total bilirubin decreased after PEBD in FIC1 (8.1±4.0 vs. 2.9±4.1 mg/dL, preop vs. 12–24 months postop respectively, p=0.02) but not in ALGS or BSEP. Total serum cholesterol decreased after PEBD in ALGS patients (695±465 vs. 457±319 mg/dL; preop vs. 12–24 months postop respectively; p=0.0001). Alanine aminotransferase levels increased in ALGS after PEBD (182±70 vs. 260±73 IU/L, preop vs. 24 mos. p=0.03) but not in FIC1 or BSEP. ALGS, FIC1, and BSEP patients experienced less severely scored pruritus after PEBD (ALGS: 100% vs. 9% severe, FIC1: 64% vs. 10%; BSEP: 50% vs. 20%, preop vs. >24 mos. postop respectively, p<0.001). ALGS patients experienced a trend toward greater freedom from xanthomata after PEBD. There was a trend toward decreased pruritus in FIC1 after IE and GBC. Vitamin K supplementation increased in ALGS after PEBD (33% vs. 77%, p=0.03. Overall, there were 15 major complications after surgery. 12 patients (3 ALGS, 3 FIC1, 6 BSEP) subsequently underwent liver transplantation. Conclusion This is the first multi-center analysis of non-transplant surgical approaches to intrahepatic cholestasis. Approaches vary, are well tolerated, and generally although not uniformly result in improvement of pruritus and cholestasis.

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“…Recently, AGS patients have been described as having optic pits (Fea et al. 2006) and being at risk of developing optic atrophy caused by idiopathic intracranial hypertension (Narula et al. 2006).…”

Section: Discussionmentioning

confidence: 99%

Occurrence and pattern of ocular disease in children with cholestatic disorders

Fahnehjelm

Fischler

Martin

et al. 2011

Acta Ophthalmologica

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ABSTRACT.Purpose: To describe visual function and ocular manifestations in patients with onset of cholestasis during the neonatal period. Methods: Patients with neonatal cholestasis, either transitory or chronic, who came for assessment to our tertiary referral centre were included in a crosssectional study and underwent ophthalmological examinations including fundus photography. A total of 57 patients (24 girls and 33 boys), aged 0.4-18.0 years, were included. Of these, 28 patients had biliary atresia, 11 had Alagille's syndrome, five had progressive familiar intrahepatic cholestasis and nine had different disorders such as pituitary insufficiency, alpha-1-antitrypsin deficiency, mitochondriopathy, congenital infections or cholestasis caused by unknown reasons. Results: Visual dysfunction and ⁄ or one or several ocular manifestations occurred in 39 out of 57 patients. Major ocular malformations occurred in five patients. Out of three patients with biliary atresia, one patient had severe visual impairment caused by microphthalmia and chorioretinal coloboma, one patient with Cat Eye syndrome had bilateral uveochorioretinal coloboma and one patient had Rieger's anomaly. Two patients, both with pituitary deficiency and transient cholestasis, had severe unilateral visual impairment caused by optic nerve hypoplasia. Conclusion: The majority (68%) of the patients with cholestasis had ocular manifestations. Although the severity of ocular complications varied with diagnosis, and was most apparent among patients with biliary atresia or pituitary deficiency, no conclusion can be drawn regarding the connections between these conditions from the present study. Nevertheless, ocular assessment is important for diagnostic purposes and for early intervention in patients with cholestasis.

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Severe Gastric Outlet Obstruction in a Child with Ectopic Pancreas

Abstract: We recommend annual fundoscopy in the follow-up of children with AGS to facilitate early detection and appropriate management of IIH to prevent visual loss.

Cited by 33 publications

References 13 publications

Systematic Review

Systematic Review

Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis

Occurrence and pattern of ocular disease in children with cholestatic disorders

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