Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

1. Thirty-two patients with hemoglobin SC (Hb SC) disease from Rio de Janeiro, Brazil, are described. Mean patient age at the time of study was 20 years, and average age at onset nine years. 2. The main complaint at presentation was bone or joint pains. Symptoms involved bone or joint pains in 72% of the patients, while 59% presented hepatomegaly and 50% splenomegaly. Mean hemoglobin level was 10.6 g/dl, erythrocyte count, 3.9 X 10(6)/mm3, and serum bilirubin, 1.4 g/dl. Reticulocytes and Hb F were 3.0% and 2.2%, respectively. 3. The characteristics of Hb SC disease observed in the present study did not differ significantly from those of other series studied elsewhere. This relative homogeneity of data is probably related to the physiopathological mechanism that promotes sickling in this condition.
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PMID:Clinical and hematological features of hemoglobin SC disease in Rio de Janeiro, Brazil. 365 26

Twenty-seven patients were seen and followed at our Sickle Cell Center over a period of seven years. Their clinical, hematological, and biochemical features were determined and compared to those of patients with sickle cell anemia who were concurrently investigated. The data indicate that the mild anemia of hemoglobin (Hb) SC disease is slightly microcytic and hyperchromatic. Parameters of hemolysis and the complications of chronic hemolytic anemia (cholelithiasis, leg ulcers, hepatomegaly, and cardiomegaly) are milder in Hb SC disease than in sickle cell anemia. Asplenia and its sequelae (increased platelet count and reduced serum IgM levels) are less frequent in Hb SC disease. Cerebrovascular accidents and the decreased leukocyte alkaline phosphatase scores are similar in both diseases. Thromboembolic complications, retinopathy, and renal papillary necrosis are more frequent in Hb SC disease.
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PMID:Clinical, hematological, and biochemical features of Hb SC disease. 713 65

Acute sickle hepatic crisis (ASHC) has been observed in approximately 10% of patients with sickle cell disease. It occurs predominantly in patients with homozygous (Hb SS) sickle cell anemia and to a lesser degree in patients with Hb SC disease, sickle cell trait, and Hb S beta thalassemia. Patients commonly present with jaundice, right upper quadrant pain, nausea, low-grade fever, tender hepatomegaly, and mild to moderate elevations in serum AST, ALT, and bilirubin. We describe the case of a patient with a history of hemoglobin SC disease and cirrhosis caused by hepatitis C presenting approximately 1 year after liver transplantation with an ASHC. The diagnosis was confirmed by liver biopsy. Our patient was treated with RBC exchange transfusions, IV hydration, and analgesia and made a complete recovery. Only a limited number of patients with sickle cell disease have received liver transplants, and, to our knowledge, this is the first case of ASHC after transplantation in a patient with Hb SC disease.
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PMID:Acute Sickle Hepatic Crisis after Liver Transplantation in a Patient with Hb SC Disease. 2578 94