Inheritance of the Dubin-Johnson-Sprinz Syndrome
نویسندگان
چکیده
منابع مشابه
Lysosomal changes in liver tissue from patients with the Dubin-Johnson-Sprinz syndrome.
1. Clinical, morphological and biochemical data, including data obtained from the application of subcellular fractionation techniques to liver biopsy specimens, are presented for two patients with the Dubin-Johnson-Sprinz (DJS) syndrome. 2. Subcellular fractionation experiments demonstrate that the lysosomes, which have strikingly reduced equilibrium densities, accumulate melanin. Morphological...
متن کاملDubin-Johnson syndrome.
A young man presented with recurrent episodes of mild jaundice. Apart from conjugated hyperbilirubinemia, other liver function tests were always normal. Clinical suspicion of Dubin-Johnson syndrome was raised. Liver biopsy showed diffuse deposition of coarse granular dark brown pigment in hepatocytes. Dubin-Johnson syndrome is a benign condition, which results from a hereditary defect in biliar...
متن کاملCholescintigraphy in Dubin-Johnson syndrome.
Hereditary or familial hiperbilirubinemia comprises a group of syndromes (Dubin-Johnson’s, Rotor’s, hepatic storage disease) in which hyperbilirubinemia, predominantly unconjugated or conjugated, occurs as an isolated biochemical abnormality without evidence of either hepatocellular necrosis or cholestasis. We present a patient with Dubin-Johnson syndrome, one of the familial disorders associat...
متن کاملThe organelle pathology and demonstration of mitochondrial superoxide dismutase deficiency in two patients with Dubin--Johnson--Sprinz syndrome.
1. Liver biopsies from two patients with the Dubin-Johnson-Sprinz syndrome were subjected to analytical subcellular fractionation by sucrose-density-gradient centrifugation and enzymic microassays. 2. A selective deficiency of mitochondrial superoxide dismutase has been demonstrated in these patients. 3. The significance of this enzyme deficiency is discussed in relation to the organelle pathol...
متن کاملDubin-Johnson syndrome presenting with neonatal cholestasis.
We report a case of Dubin-Johnson syndrome presenting with neonatal cholestasis. Liver histology was studied during the neonatal period and at 6 years of age. Distinct brownish pigment granules in hepatocytes were noted. This case confirms that Dubin-Johnson syndrome is a cause of neonatal cholestasis.
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ژورنال
عنوان ژورنال: Gastroenterology
سال: 1975
ISSN: 0016-5085
DOI: 10.1016/s0016-5085(75)80285-x