Volume 12, Issue 1 (5-1998)                   Med J Islam Repub Iran 1998 | Back to browse issues page

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SABRI M R, ALAVIAN-GHAVANINI A. BENIGN EXTREME HYPERBILIRUBINEMIA IN A 9 YEAR-OLD GIRL WITH SICKLE-THALASSEMIA AND THE PROBABLE ROLE OF HBF IN PREDICTING THE OUTCOME. Med J Islam Repub Iran 1998; 12 (1) :75-78
URL: http://mjiri.iums.ac.ir/article-1-1051-en.html
BENIGN EXTREME HYPERBILIRUBINEMIA IN A 9 YEAR-OLD GIRL WITH SICKLE-THALASSEMIA AND THE PROBABLE ROLE OF HBF IN PREDICTING THE OUTCOME
MOHAMMAD REZA SABRI , AHMAD ALAVIAN-GHAVANINI
Assistant Professor
Abstract:   (4914 Views)
Hepatic dysfunction is a frequent manifestation in patients with sickle cell anemia. It is usually a multifactorial process. A rare benign form of extreme hyperbilirubinemia, presumably due to intrahepatic sickling, may be the cause. We report a 9 year old girl with sickle-thalassemia hemoglo binopathy, presenting with profound jaundice. Sickle cell disease is often mild in the Iranian population due to relatively higher levels of HbF, suggesting that the βs gene is associated with a gene capable of producing high levels of HbF. Moreover, sickle thalassemia disease is generally milder than sickle cell disease. In this patient, the previous electrophoresis had shown a relatively high HbF level (34.3%). This may account for the benign course of hyperbilirubinemia and no need for blood transfusion in this case, despite the majority of previous reports
Keywords: Sickle-thalassemia, Intrahepatic sickling, Fetal hemoglobin.
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Type of Study: case report | Subject: Pediatric
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