A 19-year-old Patient with Recurrent Pruritus and Jaundice

نویسندگان

چکیده

А im: to highlight the importance of broad differential diagnosis and possibility conversion benign recurrent intrahepatic cholestasis type 2 into more aggressive clinical phenotype. Key points. A 19-year-old female patient was admitted Clinic with skin pruritus, jaundice, dark urine, clay-colored stool, general fatigue. Past medical history significant for aforementioned symptoms since 3 years old, that relapsed every 1–2 were usually ameliorated conservative therapy. During recent years, frequency relapses recovery period increased, at same time effectiveness therapy decreased. Blood chemistry results revealed an elevation total bilirubin (up 634 μmol/L), direct 354 bile acids 510 μmol/L) normal gamma glutamyl transferase level. Workup negative viral hepatitis, autoimmune liver diseases, obstructive choledochal lesions, storage although mutation in gene ABCB11 found. Benign diagnosed. Following plasmapheresis, jaundice pruritus significantly diminished, levels normalized. Regular follow up, biopsy measures relapse prevention given features phenotype recommended. Conclusion. Identification etiology cholestatic diseases requires diagnosis. Clinical course patients may transform phenotype, reminiscent progressive familial cholestasis.

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ژورنال

عنوان ژورنال: ?????????? ?????? ?????????????????, ???????????, ???????????????

سال: 2023

ISSN: ['2658-6673', '1382-4376']

DOI: https://doi.org/10.22416/1382-4376-2023-33-3-66-75