-thalassemia major is a disease caused by  polypeptide chain synthesis disorder which is inherited as an autosomal recessive from both parents which is marked by little or no  globin chain synthesis. Medication for  thalassemia major patients is by repeated blood transfusions, which causes hemochromatosis. Hemochromatosis can occur in various organs including the pancreas. The aim of the st...

We report the first surviving case of neonatal hemochromatosis with renal tubular dysgenesis. Renal failure was treated with peritoneal dialysis. Although hepatic failure from neonatal hemochromatosis was progressive, repeated exchange transfusions improved jaundice and coagulopathy. The patient gained weight and received a liver transplantation from her father.

BACKGROUND AND OBJECTIVE Porphyria cutanea tarda (PCT) is a disorder of porphyrin metabolism associated with decreased activity of uroporphyrinogen decarboxylase (URO-D) in the liver. The relevance of iron in the pathogenesis of PCT is well established: iron overload is one of the factors that trigger the clinical manifestations of the disease and iron depletion remains the cornerstone of thera...

TESTOSTERONE TREATMENT OF HEMOCHROMATOSIS In recent years there have been reports suggesting that in patients with hemochromatosis the use of testosterone may be of benefit, at least symptomatically, with apparent improvement in strength and sense of wellbeing. Such beneficial results have been thought to be related to the gonadal hypoplasia commonly seen in hemochromatosis and to the anabolic ...

BSJFW, et al. Automated measurement of unsaturated iron binding capacity is an effective screening strategy for C282Y homozygous hemochromatosis. Gut 2000;46:405–9. 14. Witte DL, Crosby WH, Edwards CQ, Fairbanks VF, Mitros FA. Hereditary hemochromatosis. Clin Chim Acta 1996;245:139–200. 15. Adams PC, Bhayana V. Unsaturated iron binding capacity: a screening test for hemochromatosis? Clin Chem 2...

A case of neonatal hemochromatosis is reported in a premature 35-week infant who presented at birth with coffee ground vomiting and gradual appearance of grayish icter and colorless stool. Neonatal hemochromatosis was confirmed by elevated ferritin levels and extrahepatic siderosis detected in liver biopsy.