Normalization of serum bile acids after partial external biliary diversion indicates an excellent long-term outcome in children with progressive familial intrahepatic cholestasis

Schukfeh, Nagoud; Metzelder, Martin L.; Petersen, Claus; Reismann, Marc; Pfister, Eva Doreen; Ure, Benno M.; Kuebler, Joachim F.

doi:10.1016/j.jpedsurg.2011.08.010

Cited by 59 publications

(69 citation statements)

References 24 publications

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“…Partial biliary diversion (PBD) is currently regarded as the therapy of first choice for patients with progressive familial intrahepatic cholestasis (PFIC) associated with a low serum gammaglutamyl transpeptidase (GGT) [1][2][3][4][5]. This group comprises both PFIC type I (ATP8B1 deficiency) and PFIC type II (ABCB11 deficiency) [4].…”

Section: Accepted Manuscriptmentioning

confidence: 99%

“…Even better results were reported when PEBD was performed in non-cirrhotic patients [3,5]. In some individuals, PEBD was able to reverse hepatic fibrosis and reduce disease progression [1][2][3]5,7,15]. Therefore, PBD is now considered to be the first treatment in non-cirrhotic individuals with low-GGT PFIC (type I and II) [5].…”

Section: Accepted Manuscriptmentioning

confidence: 99%

“…In some individuals, PEBD was able to reverse hepatic fibrosis and reduce disease progression [1][2][3]5,7,15]. Therefore, PBD is now considered to be the first treatment in non-cirrhotic individuals with low-GGT PFIC (type I and II) [5]. In contrast, most patients with Alagille syndrome can be treated conservatively, although PBD has been used for patients with intractable pruritus [2,6,16].…”

Section: Accepted Manuscriptmentioning

confidence: 99%

See 2 more Smart Citations

Total biliary diversion as a treatment option for patients with progressive familial intrahepatic cholestasis and Alagille syndrome

Woerd

Kokke

Zee

et al. 2015

Journal of Pediatric Surgery

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Section: Accepted Manuscriptmentioning

confidence: 99%

Section: Accepted Manuscriptmentioning

confidence: 99%

Section: Accepted Manuscriptmentioning

confidence: 99%

See 1 more Smart Citation

Total biliary diversion as a treatment option for patients with progressive familial intrahepatic cholestasis and Alagille syndrome

Woerd

Kokke

Zee

et al. 2015

Journal of Pediatric Surgery

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“…Previous reports [3,[5][6][7][9][10][11][12][13] have noted therapeutic effect from PEBD in patients with PFIC1. However, such benefits are not seen in all PFIC1 patients.…”

Section: Discussionmentioning

confidence: 87%

Clinical Course Following Partial External Biliary Diversion for Progressive Familial Intrahepatic Cholestasis 1: A Report of Two Patients

Kimura

Abukawa²,

Takei³

et al. 2016

Journal of Gastroenterology and Hepatology Research

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AIM:To better understand the effect of partial external biliary diversion (PEBD) in patients with progressive familial intrahepatic cholestasis 1 (PFIC1). MATERIALS AND METHODS:We analyzed quantitative changes in the concentrations of individual bile acids in serum, urine, and biliary bile, and concomitant changes in symptoms such as pruritus, in 2 patients whose PFIC1 clinically evolved to resemble the course of benign recurrent intrahepatic cholestasis (BRIC) upon treatment with PEBD. RESULTS: Patient 1 showed good therapeutic effect after PEBD including improved liver function test results and decreased total bile acids in serum, as well as decreased pruritus. The cholic acid (CA)/chenodeoxycholic acid (CDCA) ratio in biliary bile also decreased. Patient 2, however, experienced little decrease in pruritus after PEBD, and showed no clear change in the CA/CDCA ratio. Nonetheless, this patient's course did not rapidly worsen. CONCLUSION: In PFIC1, increased bile secretion and a decreased CA/CDCA ratio in biliary bile may be the most important responses after PEBD. Relief of pruritus is also an important sign of therapeutic effect. Moreover, progression of symptoms in PFIC1 sometimes can be delayed by PEBD, resulting in a relatively mild BRIC-like course, even when the CA/CDCA ratio and pruritus show little change. However, mechanisms underlying clinical benefits remain obscure. Abbreviations PFIC1: progressive familial intrahepatic cholestasis 1; GGT: γ-glutamyltransferase;

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“…UDCA helps to postpone biliary cirrhosis in the PFIC conditions. An early partial external biliary diversion normalizes serum bile acids and relieves pruritus for children with PFIC1 and 2 (56). This procedure decelerates disease progression until liver transplantation is needed.…”

Section: Introductionmentioning

confidence: 99%

Neonatal Cholestasis â€“ Differential Diagnoses, Current Diagnostic Procedures, and Treatment

et al. 2015

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Cholestatic jaundice in early infancy is a complex diagnostic problem. Misdiagnosis of cholestasis as physiologic jaundice delays the identification of severe liver diseases. In the majority of infants, prolonged physiologic jaundice represent benign cases of breast milk jaundice, but few among them are masked and caused by neonatal cholestasis (NC) that requires a prompt diagnosis and treatment. Therefore, a prolonged neonatal jaundice, longer than 2 weeks after birth, must always be investigated because an early diagnosis is essential for appropriate management. To rapidly identify the cases with cholestatic jaundice, the conjugated bilirubin needs to be determined in any infant presenting with prolonged jaundice at 14 days of age with or without depigmented stool. Once NC is confirmed, a systematic approach is the key to reliably achieve the diagnosis in order to promptly initiate the specific, and in many cases, life-saving therapy. This strategy is most important to promptly identify and treat infants with biliary atresia, the most common cause of NC, as this requires a hepatoportoenterostomy as soon as possible. Here, we provide a detailed work-up approach including initial treatment recommendations and a clinically oriented overview of possible differential diagnoses in order to facilitate the early recognition and a timely diagnosis of cholestasis. This approach warrants a broad spectrum of diagnostic procedures and investigations including new methods that are described in this review.

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Normalization of serum bile acids after partial external biliary diversion indicates an excellent long-term outcome in children with progressive familial intrahepatic cholestasis

Cited by 59 publications

References 24 publications

Total biliary diversion as a treatment option for patients with progressive familial intrahepatic cholestasis and Alagille syndrome

Total biliary diversion as a treatment option for patients with progressive familial intrahepatic cholestasis and Alagille syndrome

Clinical Course Following Partial External Biliary Diversion for Progressive Familial Intrahepatic Cholestasis 1: A Report of Two Patients

Neonatal Cholestasis â€“ Differential Diagnoses, Current Diagnostic Procedures, and Treatment

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