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Biomedical subjects

C Pegelow

Publications and source records attributed to C Pegelow.

11 recordsLinked to original sources
PubMed1986

Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial.

Children with sickle cell anemia have an increased susceptibility to bacterial infections, especially to those caused by Streptococcus pneumoniae. We therefore conducted a multicenter, randomized, double-blind, placebo-controlled clinical trial to test whether the regular, daily administration of oral penicillin would reduce the incidence of documented septicemia due to S.pneumoniae in children with sickle cell anemia who were under the age of three years at the time of entry. The children were randomly assigned to receive either 125 mg of penicillin V potassium (105 children) or placebo (110 children) twice daily. The trial was terminated 8 months early, after an average of 15 months of follow-up, when an 84 percent reduction in the incidence of infection was observed in the group treated with penicillin, as compared with the group given placebo (13 of 110 patients vs. 2 of 105; P = 0.0025), with no deaths from pneumococcal septicemia occurring in the penicillin group but three deaths from the infection occurring in the placebo group. On the basis of these results, we conclude that children should be screened in the neonatal period for sickle cell hemoglobinopathy and that those with sickle cell anemia should receive prophylactic therapy with oral penicillin by four months of age to decrease the morbidity and mortality associated with pneumococcal septicemia.

Administration, Oral↗
PubMed1986

Erythrocyte glutathione synthetase deficiency leads not only to glutathione but also to glutathione-S-transferase deficiency.

Glutathione synthetase (GSH-S) is one of the two known hereditary causes of glutathione deficiency. We describe a family whose two children have hemolytic anemia. The children's erythrocytes lack GSH and are severely deficient in GSH-S activity. No neurologic findings or 5-oxoprolinuria were present. A concurrent deficiency of glutathione-S-transferase (GST) was also detected in the erythrocytes. Residual glutathione could be detected in the erythrocytes using a sensitive cycling assay. The deficiency was found to be most severe in reticulocyte-depleted preparations. The GSH-S activity of the erythrocytes of the parents was one-half normal, while the glutathione S-transferase activity was normal. We conclude that the primary defect is one of GSH-S. Glutathione stabilizes GST in vitro, and it is assumed that the deficiency of GST in the erythrocytes of the patients is due to the instability of this enzyme in the absence of adequate intracellular GSH levels.

Adolescent↗
PubMed1977

Leukemia following Reye's syndrome treated with exchange transfusions: search for type-C viral infection.

A 5-year-old child developed acute lymphoblastic leukemia during convalescence from an episode of Reye's syndrome that was treated with multiple exchange transfusions. Routine laboratory, histology, and viral serology were unable to establish a common etiology for the 2 illnesses. Cultural and immunologic methods to search for evidence of infection with type-C viruses or viral genes in lymphoblasts from the buffy coat and bone marrow failed to reveal these agents. Although no common infectious etiology was defined for the close temporal occurrence of 2 rare diseases, the possibility of an iatrogenically induced malignancy was considered.

Blood Transfusion↗
PubMed1977

Severe coagulation abnormalities in Reye syndrome.

Coagulation studies were performed on 13 children with clinical diagnoses of Reye syndrome. Four had abnormalities so severe that transfusion of coagulation factors and platelets were required prior to percutaneous liver biopsy. In one of these, abnormalities frequently found in association with acute hepatic failure were present. Disseminated intravascular coagulation was noted in two, while in the fourth child laboratory data were most consistent with that process. Although the coagulation defect associated with Reye syndrome is most often due to decreased production of all coagulation factors except VIII, these patients demonstrate that consumption can also occur. The severity of the coagulopathies found indicates that bleeding can become a major complication in the diagnosis and management of Reye syndrome.

Blood Coagulation Disorders↗
PubMed1977

On the birth of an infant with sickle cell anemia.

This is the first report in the English literature of the birth of an obligate SS infant whose parents both had sickle cell anemia. The young adult SS parents of this infant and their families did not wish to prevent the birth of this child but had, in fact, many positive reasons to continue the pregnancy to term even in the face of the increased personal risk of pregnancy for this mother. This unusual circumstance initiated a re-appraisal of the realistic expectations with respect to reproduction in patients with homozygous recessive genetic disorders. No definitive studies could be found which attempted to evaluate the influence of genetic counseling on reproductive behavior in patients with sickle cell anemia. The questions raised about the 'right to reproduce' and the realistic aims of genetic counseling in adult homozygote patients have been discussed. However, no conclusions seem warranted because of the paucity of available information.

Adult↗
PubMed1977

Congenital malaria: a rare cause of splenomegaly and anemia in an American infant.

A 38-day-old infant had fever, jaundice, hepatosplenomegaly, and a hemolytic anemia. A peripheral blood smear demonstrated intraerythrocytic malarial parasites identified as Plasmodium vivax. Maternal and infant sera contained antibodies to this species. A directed history revealed the mother had suffered several febrile illnesses in Mexico during her pregnancy. Malaria had not been diagnosed nor was it considered at the time of her delivery at this hospital. Review of this and six other cases of congenital malaria reported in this country since 1950 indicates clinical manifestations seldom appear before 3 weeks of age. Although these signs are more frequently associated with other transplacental infections, their occurrence in an infant whose mother is from or who has traveled in an endemic area should prompt consideration of the diagnosis of congenital malaria.

Adult↗
PubMed1977

Severe iron deficiency anemia.

Severe iron deficiency anemia remains a continuing major health hazard among inner city children in Los Angeles. Over a 24-month period, 60 children in whom hemoglobin values were below 7 grams per dl were admitted to hospital; 11 (18 percent) of them were in overt congestive heart failure. Contrary to the popular conceptions, two thirds of the anemic children were undernourished, approximating the 16th percentile for weight on the Iowa growth chart, and the frequency of premature birth was not greater than in the general population. There were no deaths in this series. A management protocol which included partial exchange transfusion of children in congestive heart failure and supportive transfusion for children with hemoglobin levels below 5 grams per dl was employed.

Anemia, Hypochromic↗