1. Two cases of naphthalene hemolytic anemia in the newborn period are reported.
2. Both exhibited glutathione instability upon incubation with acetyl phenylhydrazine and naphthol months to years later. Several members of their families exhibited a similar defect with evidence that it is inherited as a simple dominant.
3. In those individuals with glutathione instability there was deficient TPNH2 generation by their hemolysates in the presence of glucose-6-phosphate and TPN, indicating a deficiency in glucose-6-phosphate dehydrogenase activity. Glutathione reductase activity was normal or decreased.
4. TPNH2-linked reduction of methemoglobin by erythrocyte suspensions in the presence of glucose and methylene blue was also decreased in those subjects tested, a finding consistent with the deficiency in glucose-6-phosphate dehydrogenase.
With the Technical Assistance of Frances DeLuca, B.S., and Alice Manchester, B.S. Several factors are thought to contribute to the iron-deficiency anemia of infancy. Included among these are decreased dietary iron, inadequate iron stores at birth, and excessively rapid growth. This study was undertaken to evaluate the relative importance of these factors.
Methods and MaterialsThe peripheral blood was studied in all infants, as previously described,1 with the exception of some hemoglobin determinations, which were measured with use of a modification of the cyanomethemoglobin method.2 Serum iron and iron-binding capacity were measured by the method
1) A case of hypoplastic anemia with multiple congenital anomalies (Fanconi's type) is reported. The patient exhibited pancytopenia associated with skin pigmentation, small stature and anomalies of the skeletal and genito-urinary systems.
2) The literature is reviewed and the characteristics of the disease discussed.
3) Management and therapy are discussed.
I . The effects of iron overloading and unsaturation of dietary lipid on the metabolism of a-tocopherol in the rat were studied.2. Young adult male vitamin E-deficient rats were given 1000 i.u. of vitamin A and 100 pg of [14C-5-Me]~-a-tocopherol and then given diets containing 5 yo methyl oleate or 5 yo cod-liver oil fatty-acid methyl esters. Rats from each group were given intramuscular injections of iron-dextran (50 mg Fe/kg rat) at 48 h intervals for 15 days, and compared with controls given dextran. After this time, liver, kidney and the remainder of the carcass were analysed for[14C]a-tocopherol, and liver and kidney were also analysed for vitamin A.3. There was no evidence that Fe overloading caused any increase in the destruction of either tocopherol or vitamin A in vivo, whether or not the diet contained polyunsaturated fatty acids. Indeed, treatment with Fe significantly decreased the metabolism of the radioactive tocopherol dose in all three tissues studied.
4.These experiments show that the stress effect of Fe in the vitamin E-deficient animal is unrelated to an increase in oxidative reactions. They provide further evidence that ' lipid peroxidation' is not causally concerned in ' anti-vitamin E' stress conditions and that a-tocopherol does not function, in vivo, as an antioxidant.
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