Tag Archives: partial external biliary diversion

What Works for Itching?

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Two recent articles delve into the issue of pruritus associated with cholestasis:

  • JE Squires et al. JPGN 2017; 64: 425-430.
  • Thebaut et al. JPGN 2017; 64: 431-35.

In the first study, a single-center retrospective review of 8 patients with FIC1 disease who underwent partial exernal biliary diversion (PEBD) showed that all patients had resolution of chronic cholestasis (T bili <2 mg/dL) but 7 of 8 experience episodic cholestatic events. Pruritus improved but did not resolve.  PEBD did not obviate the need for aggressive fat-soluble vitamin supplements.

In the second study, the authors added sertraline to patients who had ongoing pruritus despite ursodeoxycholic acid and rifampin therapy.  Patients had either Alagille syndrome or PFIC (progressive familial intrahepatic cholestasis). Two patients had undergone PEBD. Sertraline was started at 1 mg/kg/day and increased as needed every two weeks to max of 4 mg/kg/day (median daily dose 2.2 mg/kg/day).  6 patients had adverse effects, including agitation (2), skin reactions (2), alopecia (1) and vomiting (1). Key finding:

  • 14 of 20 children had improved “itching score” from 8/10 to 5/10.  This correlated with improved sleep and less skin scratch marks.

 

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications/diets (along with potential adverse effects) should be confirmed by prescribing physician/nutritionist.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.

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Clinical Features of Byler Disease

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A recent article (Morris AL, et al. JPGN 2015; 60: 460-6) provides a detailed analysis of six cases of Byler disease during their first two years of life.  These cases were strictly defined and defined by homozygous c.923G>T mutation of ATP8b1.

Presenting features:

  • 2 with newborn direct hyperbilirubinemia
  • 2 with complications of coagulopathy. “Bleeding diathesis is a particular issue in the Amish community where home delivery is common and vitamin K may not be administered perinatally.”
  • 1 with failure to thrive and rickets
  • 1 was a sibling identified with newborn genetic testing

Key features:

  • Intensive fat-soluble vitamin supplementation was needed. “Vitamin K deficiency can be lethal.”
  • Poor growth was frequent (Figure 2): “growth trajectories were generally at the low end of percentiles and did not reflect parental size.” It was “typically responsive to supplementation with medium-chain triglyceride-based formula. and/or use of 30 cal per ounce formulae.”
  • Elevated serum bile acids and low normal GGT (Υ-glutamyltranspeptidase)
  • Diarrhea was commonly reported
  • Intractable pruritus in 4 of 6 children which developed between 6-12 months of age;  in two patients rifampin therapy was effective.
  • Partial external biliary diversion was used in 4 children during 2nd year of life; there was a “generally favorable response to PEBD.”
  • There were not issues noted with portal hypertension

Bottomline: This report shares some practical experience with this rare disorder.

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