A Child with Debilitating Pruritus
| Autor: | Kshitij K Munde, Vinay B Pawar, Vinay Zanwar, Ravindra Surude, Sandeep B Bavdekar, Nikhil Sonthalia, Pravin Rathi, Samit S Jain |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2016 |
| Předmět: |
medicine.medical_specialty
medicine.drug_class Progressive familial intrahepatic cholestasis Case Report bland cholestasis Gastroenterology 03 medical and health sciences 0302 clinical medicine Cholestasis alopecia granular bile Internal medicine medicine lcsh:R5-920 Cholestyramine Bile acid medicine.diagnostic_test business.industry General Medicine Jaundice pruritus medicine.disease Ursodeoxycholic acid 030220 oncology & carcinogenesis Liver biopsy 030211 gastroenterology & hepatology medicine.symptom business lcsh:Medicine (General) Rifampicin medicine.drug |
| Zdroj: | Clinics and Practice Clinics and Practice, Vol 6, Iss 4 (2016) |
| ISSN: | 2039-7283 2039-7275 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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