Management of neonatal cholestasis: Consensus statement of the pediatric gastroenterology chapter of Indian academy of pediatrics

Bhatia, Vidyut; Bavdekar, Ashish; Matthai, John; Waikar, Yogesh; Sibal, Anupam

doi:10.1007/s13312-014-0375-2

Cited by 39 publications

(45 citation statements)

References 33 publications

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“…We describe the case of a 10-year-old boy who presented with obstructive jaundice and other symptoms related to cholestasis from early infancy. Globally, only 500 cases [1] of AGS have been reported so far, with only five cases in the Indian subcontinent [5]. Rarely, AGS presents with skin manifestations and early-onset chronic liver disease (CLD), which was found in our case and is therefore worth reporting.…”

Section: Introductionmentioning

confidence: 54%

“…AGS is diagnosed if three or more of the following five major features are present: cardiac murmur, posterior embryotoxon, butterfly-like vertebrae, renal abnormalities, and characteristic facies, along with histological diagnosis of bile duct paucity [8]. The exact prevalence of this syndrome in the Indian subcontinent is not known; only five cases have been reported so far [5, 9–13]. This is an autosomal dominant syndrome, with the gene ( JAG1 ) being traced to chromosome 20 [13].…”

Section: Discussionmentioning

confidence: 99%

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A 10-year-old child presenting with syndromic paucity of bile ducts (Alagille syndrome): a case report

et al. 2016

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BackgroundAlagille syndrome, a rare genetic disorder with autosomal dominant transmission, manifests with five major features: paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects, and peripheral pulmonary stenosis. Globally, only 500 cases have so far been reported, with only five cases reported in the Indian subcontinent. Rarely, Alagille syndrome also presents with skin manifestations and early-onset chronic liver disease, which was found in our case. We believe that we report what could be the first case of Alagille syndrome presenting with café au lait spots, as no such published case report could be found in the literature.Case presentationWe report an unusual case of childhood cholestatic jaundice with neonatal onset of jaundice. A 10-year-old boy from the Indian subcontinent presented with obstructive jaundice from early infancy. He also had recurrent fractures of his upper limb bones, intermittent bleeding from his nose, productive cough, decreased night vision, hyperpigmented spots over his skin, and progressive enlargement of his abdomen. Histological examination of a liver biopsy specimen revealed a paucity of bile ducts and changes suggestive of chronic liver disease. Our patient was diagnosed with Alagille syndrome and managed conservatively but died 1 year after the final diagnosis.ConclusionsThis particular syndromic form of paucity of bile duct disorder has been rarely reported in the Indian literature so far. Our case is notable because the child had café au lait spots and very early onset of chronic liver disease, which is quite rare in Alagille syndrome. We believe this to be the first case report on Alagille syndrome manifesting with café au lait syndrome and such early onset of chronic liver disease.

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Section: Introductionmentioning

confidence: 54%

Section: Discussionmentioning

confidence: 99%

A 10-year-old child presenting with syndromic paucity of bile ducts (Alagille syndrome): a case report

et al. 2016

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“…Neonatal cholestasis (NC) has been documented in 19-33% of neonates with chronic liver disease attending tertiary care hospitals in India [1][2][3] . Of these, almost one third have been attributed to biliary atresia 3 .…”

Section: Introductionmentioning

confidence: 99%

“…Of these, almost one third have been attributed to biliary atresia 3 . Cytomegalovirus (CMV) is known to cause intrahepatic bile duct paucity and destruction and has been suggested to cause extra hepatic biliary atresia (EHBA) in a few case series 4 .…”

Section: Introductionmentioning

confidence: 99%

Cytomegalovirus in biliary atresia: coincidental or causal?

Mukherjee¹,

Kundu²,

Niyogi³

et al. 2016

Sri Lanka J Child Health

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“…Hasil penelitian ini memperlihatkan bahwa SGPT dan SGOT tidak ada perbedaan bermakna antara kolestasis IH dan EH, sedangkan pada beberapa penelitian sebelumnya dinyatakan bahwa hasil SGOT dan SGPT merupakan indikator yang sensitif terhadap kerusakan jaringan pada hepar, namun memiliki spesifisitas dan nilai prognosis yang kurang baik. 12,13 Hasil laboratorium ALP dan γ-GT serum pada penelitian ini tidak didapatkan perbedaan yang bermakna, sedangkan pada penelitian lain ALP dan γ-GT serum didapatkan lebih tinggi secara bermakna pada kolestasis EH. [13][14][15] Simpulan, didapatkan perbedaan manifestasi klinis asites, sedangkan manifestasi klinis yang lain dan hasil laboratorium tidak didapatkan perbedaan antara kolestasis IH dan EH.…”

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Perbedaan Manifestasi Klinis dan Laboratorium Kolestasis Intrahepatal dengan Ekstrahepatal pada Bayi

Prasetyo¹,

Ermaya²,

Martiza³

2016

mkb

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AbstrakIkterus fisiologis sering didapatkan pada bayi dan kebanyakan gejalanya ringan. Gejala ikterik biasanya akan menghilang dalam 2 minggu setelah lahir. Pada ikterus yang terkonjugasi terjadi defek produksi intrahepatal, transpor transmembran dari empedu, yaitu kolestasis intrahepatal (IH) atau obstruksi kolestasis ekstrahepatal (EH) yang mengakibatkan hambatan empedu. Penelitian ini dilakukan untuk melihat perbedaan manifestasi klinis dan laboratorium kolestasis IH dengan EH pada bayi. Studi potong lintang dilakukan pada 72 bayi dengan kolestasis yang datang ke Rumah Sakit Dr. Hasan Sadikin Bandung, periode Januari 2014-Desember 2015. Analisis data dilakukan dengan uji Pearson Chi-kuadrat dan Mann-Whitney. Subjek terdiri atas 43 (60%) laki-laki dan 29 (40%) perempuan, kolestasis IH sebanyak 61 orang (85%), dan EH sebanyak 11 (15%). Pada penelitian ini didapatkan perbedaan bermakna manifestasi klinis asites antara kolestasis IH dan EH (p=0,047), sedangkan venektasi, hepatomegali, dan splenomegali tidak didapatkan perbedaan bermakna. Pada pemeriksaan warna feses tidak didapatkan perbedaan bermakna (p=0,936), demikian juga hasil laboratorium bilirubin total, bilirubin direk, glutamat oksaloasetat transaminase, glutamat piruvat transaminase, alkali fosfatase, dan gama glutamil transferase serum tidak berbeda bermakna. Simpulan, didapatkan perbedaan manifestasi klinis asites, sedangkan manifestasi klinis yang lain dan hasil laboratorium tidak didapatkan perbedaan antara kolestasis IH dan EH. [MKB. 2016;48(1):45-50] Kata kunci: Kolestasis ekstrahepatal, kolestasis intrahepatal, laboratorium, manifestasi klinis Differences of Clinical Manifestation and Laboratory Findings in IntraHepatic and Extra-Hepatic Cholestasis in Infants AbstractPhysiological jaundice found in infants and most symptoms are often mild. Jaundice symptoms usually disappear within 2 weeks after birth. In conjugated jaundice defects in intra-hepatic production, transmembran transport from bile, i.e. cholestasis intra hepatic (IH), or extra-hepatic (EH) obstruction/cholestasis occur, resulting in bile barriers. This study was conducted to look at the differences in the clinical and laboratory manifestations of IH and EH cholestasis in infants. A cross-sectional study was performed on 72 infants with cholestasis who came to Dr. Hasan Sadikin General Hospital Bandung, during the period of January 2014-December 2015. Data analysis was performed with Pearson Chi-square test and Mann-Whitney. Subjects consisted of 43 (60%) infant boys and 29 (40%) infant girls, IH cholestasis were 61 (85%) and EH cholestasis were 11 (15.3%). Significant differences in the clinical manifestations of acites with IH and EH cholestasis were found (p=0.047), whereas insignificant differences in venectation, hepatomegaly and splenomegaly were observed. On examination of stool color, no significant difference was found (p=0.936). The same was true for laboratory results of total bilirubin, direct bilirubin, serum glutamic oxaloacetic transaminase, glutamic pyruvic transam...

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Management of neonatal cholestasis: Consensus statement of the pediatric gastroenterology chapter of Indian academy of pediatrics

Cited by 39 publications

References 33 publications

A 10-year-old child presenting with syndromic paucity of bile ducts (Alagille syndrome): a case report

A 10-year-old child presenting with syndromic paucity of bile ducts (Alagille syndrome): a case report

Cytomegalovirus in biliary atresia: coincidental or causal?

Perbedaan Manifestasi Klinis dan Laboratorium Kolestasis Intrahepatal dengan Ekstrahepatal pada Bayi

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