Abstract

Referring to an earlier case report by Corchia et al., 1Corchia C Balata A Meloni GF Meloni T Favism in a female newborn infant whose mother ingested fava beans before delivery.J Pediatr. 1995; 127: 807-808Abstract Full Text Full Text PDF PubMed Scopus (40) Google Scholar we wish to report on a 14-hour-old girl who was admitted to the neonatal unit because of anemia and hyperbilirubinemia. She was born at 40 weeks of gestation, after an uncomplicated pregnancy. Physical examination revealed diffuse pallor and yellow sclerae. The abdomen was distended by an enlarged liver and spleen. Laboratory data were as follows: hemoglobin concentration, 12.8 gm/dl; hematocrit, 0.40; leukocyte count, 47 × 10/μl with 50 nucleated red cells per 100 leukocytes; reticulocyte count, 10%; bilirubin concentration, 12.5 mg/dl; Coombs test results, negative; blood group, B Rh + ; erythrocyte glucose-6-phosphate dehydrogenase (G6PD) activity, 1.4 units/gm hemoglobin (normal range, 3.3 to 5.5 units/gm hemoglobin); father's G6PD activity, 0.06 unit/gm hemoglobin; and mother's G6PD activity, 2.8 units/gm hemoglobin. The mother revealed that she had inhaled naphthalene during the twenty-eighth week of gestation. The baby underwent transfusion twice with 50 and 70 ml of compatible packed red blood cells (group B, Rh + ) on the second and eighteenth days of life, respectively. On day 24 from birth, the infant's hematocrit was 0.40, the liver and spleen were normal, and the baby was discharged home. Twenty days later the patient was readmitted to the hospital because of anemia. Physical examination revealed an enlarged liver and spleen. Hematocrit was 0.23; hemoglobin concentration, 7 gm/dl; reticulocyte count, 1.5%; and bilirubin concentration, 0.4 mg/dl. The patient underwent transfusion with 65 ml of compatible packed red cells (blood group, B Rh + ), after which the hematocrit was 0.36. The liver and spleen were normal 7 days later. At this writing the patient has been under medical care for 2 years; her clinical condition is very good, and laboratory data are within normal limits. We believe that her hemolytic crisis occurred in utero because the clinical signs were evident at birth. We agree with the writer's recommendation that all women living in high prevalence areas be counseled to avoid exposure to oxidizing substances, such as naphthalene and fava beans, during pregnancy.

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