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Clinics and Practice
Volume 6
Issue 4
10.4081/cp.2016.865
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Clinics and Practice is published by MDPI from Volume 11 Issue 1 (2021). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
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Case Report

A Child with Debilitating Pruritus

by
Nikhil Sonthalia
1,*,
Sami S. Jain
1,
Vinay B. Pawar
1,
Vinay G. Zanwar
1,
Ravindra G. Surude
1,
Pravin M. Rathi
1,
Kshitij K. Munde
2 and
Sandeep Bavdekar
2
1
Department of Gastroenterology, Topical National Medical College and BYL Nair Ch Hospital, Dr. A.L Nair Road, Mumbai 400008, Maharashtra, India
2
Department of Pediatric Medicine, Topical National Medical College and BYL Nair Ch Hospital, Mumbai 400008, Maharashtra, India
*
Author to whom correspondence should be addressed.
Clin. Pract. 2016, 6(4), 865; https://doi.org/10.4081/cp.2016.865
Submission received: 21 June 2016 / Revised: 28 August 2016 / Accepted: 22 September 2016 / Published: 24 November 2016

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.
Keywords: progressive familial intrahepatic cholestasis; bland cholestasis; granular bile; pruritus; alopecia progressive familial intrahepatic cholestasis; bland cholestasis; granular bile; pruritus; alopecia

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MDPI and ACS Style

Sonthalia, N.; Jain, S.S.; Pawar, V.B.; Zanwar, V.G.; Surude, R.G.; Rathi, P.M.; Munde, K.K.; Bavdekar, S. A Child with Debilitating Pruritus. Clin. Pract. 2016, 6, 865. https://doi.org/10.4081/cp.2016.865

AMA Style

Sonthalia N, Jain SS, Pawar VB, Zanwar VG, Surude RG, Rathi PM, Munde KK, Bavdekar S. A Child with Debilitating Pruritus. Clinics and Practice. 2016; 6(4):865. https://doi.org/10.4081/cp.2016.865

Chicago/Turabian Style

Sonthalia, Nikhil, Sami S. Jain, Vinay B. Pawar, Vinay G. Zanwar, Ravindra G. Surude, Pravin M. Rathi, Kshitij K. Munde, and Sandeep Bavdekar. 2016. "A Child with Debilitating Pruritus" Clinics and Practice 6, no. 4: 865. https://doi.org/10.4081/cp.2016.865

APA Style

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), 865. https://doi.org/10.4081/cp.2016.865

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