Congenital Dyserythropoietic Anemia and the Dubin-Johnson Syndrome
نویسندگان
چکیده
منابع مشابه
Dubin-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 کاملCongenital dyserythropoietic anemia type III.
BACKGROUND AND OBJECTIVES Congenital dyserythropoietic anemia type III (CDA-III) is a group of very rare disorders characterized by similar bone marrow morphology. The clinical picture is characterized by hemolytic anemia and dramatic bone marrow changes dominated by active erythropoiesis with big multinucleated erythroblasts. The aim of this review is to describe the clinical manifestations, l...
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.
متن کاملDubin-Johnson syndrome in a Saudi neonate.
There are many disorders associated with direct hyperbilirubinemia in the neonatal period. These usually need urgent referral to identify treatable diseases. DubinJohnson Syndrome (DJS), which results from impaired hepatic excretory function, is an uncommon cause of neonatal jaundice and is only sporadically reported in neonates. We report here a neonate who presented with direct hyperbilirubin...
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ژورنال
عنوان ژورنال: Gastroenterology
سال: 1974
ISSN: 0016-5085
DOI: 10.1016/s0016-5085(19)32795-7