Background: Neonates affected by hereditary spherocytosis may suffer from significant jaundice. This study was conducted on neonates with jaundice hospitalized at the Children’s Hospital in Bandar Abbas, South Iran, to determine the frequency of hereditary spherocytosis among them. Patients and Methods: In this cross-sectional study, 814 neonates with jaundice hospitalized at the Children’s ...

background: neonates affected by hereditary spherocytosis may suffer from significant jaundice. this study was conducted on neonates with jaundice hospitalized at the children’s hospital in bandar abbas, south iran, to determine the frequency of hereditary spherocytosis among them. patients and methods: in this cross-sectional study, 814 neonates with jaundice hospitalized at the children’s hos...

BACKGROUND The laboratory diagnosis of hereditary spherocytosis commonly relies on NaCl-based or glycerol-based red cell osmotic fragility tests; more recently, an assay directly targeting the hereditary spherocytosis molecular defect (eosin-5'-maleimide-binding test) has been proposed. None of the available tests identifies all cases of hereditary spherocytosis. DESIGN AND METHODS We compare...

Hereditary spherocytosis is a familial hemolytic anemia. Immunoglobulin A (IgA) nephropathy associated with hereditary spherocytosis has not been reported in children. Here, we report a case of a 17-year-old boy with IgA nephropathy and hereditary spherocytosis. The patient was diagnosed with hereditary spherocytosis at the age of 12 years and splenectomy was done at the age of 15 years. Later,...

A rare case of hereditary spherocytosis (HS) and rheumatic mitral stenosis coexisting in a patient having severe stenosis, atrial fibrillation, symptoms the left ventricular dysfunction, along with hemolytic anemia attributed to HS. We present 58-year-old lady who presented emergency department complaints increasing shortness breath for past week. She was examined have fibrillation fast rate. O...

BACKGROUND Hereditary spherocytosis is a genetic, frequently familial hemolytic blood disease characterized by varying degrees of hemolytic anemia, splenomegaly, and jaundice. There are few reports on adult open-heart surgery for patients with hereditary spherocytosis. CASE PRESENTATION We report a rare case of an adult open-heart surgery associated with hereditary spherocytosis. A 63-year-ol...

The rate of phosphoenolpyruvate transport in erythrocytes from patients with hereditary spherocytosis and from healthy individuals was examined in a 0.1 M citrate buffer (pH 6.1 at 37 degrees C) containing 10 mM phosphoenolpyruvate and 10 mM NaF. The rate in erythrocytes from patients with hereditary spherocytosis was 0.09 +/- 0.02 mumol/min/ml of cells (mean +/- SD, n = 8), whereas the rate in...

We experienced a case of a 19-year-old man with Gilbert syndrome with concomitant hereditary spherocytosis. The patient presented with moderate unconjugated hyperbilirubinemia, and inherited etiology was strongly suspected. The diagnosis of Gilbert syndrome was confirmed by the genetic analysis of the UGT1A1 gene, demonstrating UGT1A1*28 and compound heterozygote UGT1A1*6. In addition, since th...

Studies during the last three decades have enabled the development of detailed molecular insights into the structural basis of altered function in various inherited red cell membrane disorders. This review highlights our current understanding of molecular and mechanistic insights into various inherited red cell membrane disorders involving either altered membrane structural organization (heredi...