Benign recurrent intrahepatic cholestasis in a Saudi child

Drees, K al; Zaben, Abdullah Al; Amir, Abdulrazack; Abdulla, A.

doi:10.1080/02724939992563

Cited by 5 publications

(2 citation statements)

References 10 publications

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“…There are conflicting reports regarding the use of cholestyramine (bind to BS in the intestine and reduce their re-absorption), UDCA, and opioid antagonists (reduce the pruritogenic effect of endogenous opioids). One report suggested that cholestyramine therapy shortened the duration of icteric phase and that UDCA ameliorated the symptoms of pruritus [25]. Some recent reports have shown a beneficial role of rifampicin in remission of cholestasis [9].…”

Section: Discussionmentioning

confidence: 99%

Nasobiliary Drainage for Benign Recurrent Intrahepatic Cholestasis in Patients Refractory to Standard Therapy

Yakar¹,

Demır²,

Gokturk³

et al. 2016

CIM

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Purpose: Benign recurrent intrahepatic cholestasis (BRIC) is characterized by episodic cholestasis and pruritus without anatomical obstruction. The aim of this study was to evaluate the safety and efficacy of nasobiliary drainage (NBD) in patients with BRIC refractory to medical therapy and to determine whether the use of NBD prolongs the episode duration. Methods: This was a multicenter retrospective study consisting of 33 patients suffering from BRIC. All patients were administrated medical treatment and 16 patients who were refractory to standard medical therapies improved on treatment with temporary endoscopic NBD. Duration of treatment response and associated complications were analyzed. Results: Sixteen patients (43% females) underwent 25 NBD procedures. The median duration of NBD was 17 days. There were significant improvements in total and direct bilirubin and alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase and gamma-glutamyl transpeptidase on the 3rd day of NBD. Longer clinical remission was monitored in the NBD group. Post-endoscopic retrograde cholangiopancreatography pancreatitis was observed in one of 16 cases. Conclusion: NBD effectively eliminates BRIC in all patients and improves biomarkers of cholestasis. It can be suggested that patients with attacks of BRIC can be treated with temporary endoscopic NBD; however, the results of this study should be confirmed by prospective studies in the future.

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Section: Discussionmentioning

confidence: 99%

Nasobiliary Drainage for Benign Recurrent Intrahepatic Cholestasis in Patients Refractory to Standard Therapy

Yakar¹,

Demır²,

Gokturk³

et al. 2016

CIM

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“…For patients with BRIC the results are variable, varying from shortening of the cholestatic episodes [24,124,125] to no effect at all [19,24,115,116,126] (Table 2C). Consequently cholestyramine seems to have no place in the treatment of PFIC, but it may be beneficial in patients with BRIC.…”

Section: Cholestyraminementioning

confidence: 95%

Liver disease associated with canalicular transport defects: Current and future therapies

Stapelbroek

Erpecum

Klomp

et al. 2010

Journal of Hepatology

157

152

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Bile formation at the canalicular membrane is a delicate process. This is illustrated by inherited liver diseases due to mutations in ATP8B1, ABCB11, ABCB4, ABCC2 and ABCG5/8, all encoding hepatocanalicular transporters. Effective treatment of these canalicular transport defects is a clinical and scientific challenge that is still ongoing. Current evidence indicates that ursodeoxycholic acid (UDCA) can be effective in selected patients with PFIC3 (ABCB4 deficiency), while rifampicin reduces pruritus in patients with PFIC1 (ATP8B1 deficiency) and PFIC2 (ABCB11 deficiency), and might abort cholestatic episodes in BRIC (mild ATP8B1 or ABCB11 deficiency). Cholestyramine is essential in the treatment of sitosterolemia (ABCG5/8 deficiency). Most patients with PFIC1 and PFIC2 will benefit from partial biliary drainage. Nevertheless liver transplantation is needed in a substantial proportion of these patients, as it is in PFIC3 patients. New developments in the treatment of canalicular transport defects by using nuclear receptors as a target, enhancing the expression of the mutated transporter protein by employing chaperones, or by mutation specific therapy show substantial promise. This review will focus on the therapy that is currently available as well as on those developments that are likely to influence clinical practice in the near future.

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