A child with debilitating pruritus

  • Nikhil Sonthalia | nikhil_zenith@yahoo.co.in Department of Gastroenterology, Topical National Medical College and BYL Nair Ch Hospital, Mumbai, Maharashtra, India.
  • Sami S. Jain Department of Gastroenterology, Topical National Medical College and BYL Nair Ch Hospital, Mumbai, Maharashtra, India.
  • Vinay B. Pawar Department of Gastroenterology, Topical National Medical College and BYL Nair Ch Hospital, Mumbai, Maharashtra, India.
  • Vinay G. Zanwar Department of Gastroenterology, Topical National Medical College and BYL Nair Ch Hospital, Mumbai, Maharashtra, India.
  • Ravindra G. Surude Department of Gastroenterology, Topical National Medical College and BYL Nair Ch Hospital, Mumbai, Maharashtra, India.
  • Pravin M. Rathi Department of Gastroenterology, Topical National Medical College and BYL Nair Ch Hospital, Mumbai, Maharashtra, .
  • Kshitij K. Munde Department of Gastroenterology, Topical National Medical College and BYL Nair Ch Hospital, Mumbai, Maharashtra, India.
  • Sandeep Bavdekar Department of Pediatric Medicine, Topical National Medical College and BYL Nair Ch Hospital, Mumbai, Maharashtra, India.

Abstract

We describe a case of two-year-old boy presenting with debilitating pruritus, patchy alopecia and jaundice since the age of 6 months. On evaluation he had intrahepatic cholestasis with persistently raised serum alkaline phosphatase, normal Gamma glutamyl transferase and raised serum bile acid levels. His liver biopsy showed bland cholestasis and electron microscopy showed granular bile suggestive of progressive familial intrahepatic cholestasis type I. Medical therapy with ursodeoxycholic acid, cholestyramine, rifampicin with nutritional modification was successful in alleviating the symptoms and correcting the nutritional status. To our knowledge this is only the sixth case of progressive familial intrahepatic cholestasis type I reported from India. Herein we discuss the diagnostic and therapeutic hurdles that one encounters in managing progressive familial intrahepatic cholestasis and also review the literature regarding this rare disorder.

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Published
2016-11-24
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Section
Case Reports
Keywords:
Progressive familial intrahepatic cholestasis, bland cholestasis, granular bile, pruritus, alopecia.
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How to Cite
Sonthalia, N., Jain, S. S., Pawar, V. B., Zanwar, V. G., Surude, R. G., Rathi, P. M., Munde, K. K., & Bavdekar, S. (2016). A child with debilitating pruritus. Clinics and Practice, 6(4). https://doi.org/10.4081/cp.2016.865