OBJECTIVES To provide a simple myomectomy technique for low-segment Hirschsprung disease and evaluate the efficacy of the new modification. DESIGN Case series of 19 patients followed up for 12 to 56 months (mean, 39.1 months). SETTING Tanta University Hospital, Tanta, Egypt. PARTICIPANTS Nineteen patients aged 4 months to 10 years complaining of chronic constipation, with radiological and...

Objective(s) Definite diagnosis of Hirschsprung’s disease (HD) is based on histopathological study, but there are limitations associated with standard histology and histochemistry in this regard. The aim of this study was to investigate calretinin immunostaining patterns in both ganglionic and aganglionic HD intestinal specimens and to compare them with control specimens. Materials and Method...

Hirschsprung disease is a serious disorder of enteric nervous system (ENS) development caused by the failure of ENS precursor migration into the distal bowel. We now demonstrate that retinoic acid (RA) is crucial for GDNF-induced ENS precursor migration, cell polarization and lamellipodia formation, and that vitamin A depletion causes distal bowel aganglionosis in serum retinol-binding-protein-...

References 1. Takahashi M, Buma Y, Iwamoto T, Inaguma Y, Ikeda H, Hiai H. Cloning and expression of the ret proto-oncogene encoding a tyrosine kinase with two potential transmembrane domains. Oncogene 1988;3:571–8. 2. Takahashi M, Buma Y, Hiai H. Isolation of ret proto-oncogene cDNA with an amino-terminal signal sequence. Oncogene 1989;4:805–6. 3. Airaksinen MS, Saarma M. The GDNF family: signa...

OBJECTIVES The purpose of this work was to examine changes in quality of life, disease-specific functioning, and psychosocial competencies of children and adolescents (8-16 years of age) with anorectal malformations or Hirschsprung disease and to identify predictors of change in quality of life by testing an explanatory model in which background variables explained changes in quality of life vi...

Cartilage hair hypoplasia (CHH), is a rare cause of metaphyseal chondrodysplasia and short stature. Other featuresincluded hair abnormality, immunodeficiency, anemia, gastrointestinal disorders (Hirschsprung disease,celiac, …) and increased risk of cancer. The disease is an autosomal recessive disorder and previously has notbeen reported in Iran. We report a 9-year-old boy diagnosed as cartilag...