Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Homozygous alpha-thalassaemia Hb Bart is a very rare illness in North America and Western Europe, but is a common cause of NIHF especially in South East Asia. It causes severe hydrops fetalis with lethal outcome and maternal complications as preterm labour, pre-eclampsia and retained placenta. A case report of an immigrant from Laos with preterm labour and fetal ascites, but without signs of pre-eclampsia is demonstrated. Fetal heart rate (FHR) trace and biophysical profile were pathological, while umbilical Doppler flow was normal due to hypoxaemia, but with hypervolumina and a high cardiac output. An amniocentesis and puncture of the ascites were performed, but FBS was not successful. Before receiving the results a caesarean section had to be done for deterioration of the FHR trace. A 1,370-g female infant was born who died of cardiovascular failure due to an alpha-thalassaemia Hb Bart and respiratory distress 5 days post partum. The parents had both an alpha-zero-thalassaemia. alpha-Thalassaemia is still a very unknown illness in Germany, but with the increasing numbers of immigrants from South East Asia it should be considered at the differential diagnosis of hydrops fetalis. A prenatal diagnosis should be offered to parents with alpha-zero-thalassaemia.
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PMID:Perinatal case report of unexpected thalassemia Hb Bart. 828 33

Thalassemia is common in Iran. Appropriate therapy for this disease includes a regular blood transfusion and chelation therapy. However, in this approach patients will inevitably experience side effects, particularly iron overloads in critical organs, including heart, ductless glands, and liver. This study attempted to determine prevalence of adenoidal abnormality between Iranian thalassemia patients for prediagnosis and to offer necessary medical measures. This is a descriptive nonrandomized study and included all the patients suffering from thalassemia major referring to medical centers linked with the Iranian blood transfusion organization from January 2004 to January 2005. All patients were sampled for CBC, FBS, 2-h BS, HbAlC, liver function, renal function, and endocrine disease. Initially, reports of adenoidal experiments as well as other associated parameters were provided from medical records. A total of 437 patients enrolled in the study: 5.4% suffered from diabetes, 1% had hypothyroid, and 1 person showed hypoparathyroidism. The mean levels of ferritin in diabetic and nondiabetic groups and hypothyroid and nonhypothyroid groups were not significantly different. The mean levels of ferritin among various age groups also were not significantly different. Results of a comparison between present research and similar studies conducted throughout Iran and those performed abroad on adenoidal abnormalities in thalassemia patients show that subject patients of this study statistically suffered from fewer difficulties than diabetes patients in other studies.
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PMID:Endocrinopathies in patients with transfusion-dependent beta-thalassemia. 1843 1