Acute Hemolytic Anemia in the Newborn Infant due to Naphthalene Poisoning: Report of Two Cases, with Investigations into the Mechanism of the Disease

Author:

DAWSON JEAN P.1,THAYER WILLIAM W.1,DESFORGES JANE F.1,Manchester Alice C.1,Lendraitis Reda1

Affiliation:

1. Tufts Hematology Laboratory, the First and Third Tufts Medical Services and Department of Pediatrics, Boston City Hospital, Boston, Mass.

Abstract

Abstract 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.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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