Evaluation of glucose metabolism in hepatitis serology negative beta thalassemia major patients

To evaluate the impaired glucose metabolism and their possible risk factors in beta thalassemia major [TM] patients negative for hepatitis serology and PCR. Prospective descriptive study. Department of Pediatric Hematology, Pamukkale University Faculty of Medicine, Denizli, Turkey. Patients with history of familial diabetes mellitus [DM] and hepatitis serology and polymerase chain reaction [PCR] positive TM patients were excluded. An oral glucose tolerance test [OGTT] was done on 32 TM patients. Insulinogenic index, beta-cell function index and insulin resistance index were calculated. Glucose metabolism in beta TM patients negative for hepatitis serology and PCR. Seven patients [1.8%] had impaired glucose metabolism [IGM]. Three patients [9.3%] were diagnosed with DM, one [3.1%] patient with impaired glucose tolerance [IGT] and three [9.3%] patients with impaired fasting glucose [IFG]. Cases with IGM had significantly higher, annual erythrocyte consumption rate [ml/kg/year], ferritin, alaninaminotransferase [ALT], postsplenectomy period, age at first transfusion when compared with normal glucose metabolism [NGM] patients [p < 0.05]. Insulinogenic index decreased in IGM patients compared to NGM patients [p < 0.005]. Our results show that annual erythrocyte consumption rate, ferritin, post-splenectomy period, insulinogenic index and ALT values are predictive of IGM in TM patients negative for hepatitis serology and PCR

A new association: Dandy-Walker's variant and tatralogy of Fallot with atrial septal defect and patent ductus arteriosus and primary hypothyroidy.

An 11-month-old girl who has Dandy-Walker's variant (DWV) associated with tetralogy of Fallot (TOF), atrial septal defect (ASD), patent ductus arteriosus (PDA), and primary hypothyroidy is presented. There has been no report describing a case of DWV associated with TOF, ASD and PDA and primary hypothyroidy. The first case of Dandy-Walker malformation associated with TOF was reported by Kohyama et al in 1988, since then, a few cases were reported in the literature. Our patient is the first reported case.