Glucose phosphate isomerase deficiency with hereditary nonspherocytic hemolytic anemia

Eight children (5 living, 3 deceased) with severe hereditary nonspherocytic hemolytic anemia caused by glucose phosphate isomerase deficiency have been observed in two Kentucky and Indiana families. All affected children were severely anemic in early life. Three deaths occurred in young patients who did not receive adequate transfusions of blood or whose parents refused to permit splenectomy. Splenectomy generally abolishes the requirement for blood transfusion. No patient has required regular transfusion of blood after puberty. Growth and development have been surprisingly normal and no patient has died of infection. The anemia is expressed as an autosomal recessive trait, but the enzyme variant can be detected in hematologically normal heterozygotes. The abnormal isomerase molecule is heat labile and is contained in neutrophils and lymphocytes as well as in erythrocytes.     

The role of adenoviruses in the pertussis syndrome.

To define the role of adenoviruses in the pertussis syndrome, a study was done of a group of 134 children with clinical pertussis and a healthy control population of similar age, race, sex, and socioeconomic status. Adenovirus infections occurred in 30 (22.4%) of 134 patients with the pertussis syndrome and 5 (4.9%) of 101 control subjects (p smaller than 0.001). B. pertussis was recovered from 46 (34.3%) patients, and from 18 (39.1%) of these patients adenoviruses were also isolated. Although adenovirus infections also occurred in patients with the pertussis syndrome with negative cultures for B. pertussis, the rate, 12 of 88 patients (13.6%), was significantly lower (p smaller than 0.001). The clinical course was similar irrespective of the results of bacterial or viral cultures. These data substantiate the frequent association of adenoviruses with the pertussis syndrome, It would appear that adenoviruses do not usually have an independent role in the pathogenesis of the pertussis syndrome since we found them so commonly to be one agent in a mixed infection.     

Infant care: cache or carry.

To test the hypothesis that a characteristic infant-care pattern existed during most of human history, contemporary hunter-gatherers in a representative sample of world cultures were examined. Numerically coded measures of infant care revealed a uniform pattern. Mothers are the principal caregivers, providing extensive body contact day and night and prolonged breast-feeding. When not carried, the baby of hunter-gatherers has complete freedom of movement. Care is consistently affectionate, with immediate nurturant response to crying. Nonetheless, in most groups, children achieve early independence and by 2 to 4 years spend more than half the time away from the mother. In the United States this pattern of carrying that endured for one to three million years has been replaced by one resembling nesting or caching. Infants spend little time in body contact with caregivers and their movements are restricted by playpens, high chairs, or cribs. Of the minority who are breast-fed, half are weaned within a few weeks. Separate sleeping arrangements and delayed response to crying are regularly recommended. These remarkable transformations may profoundly alter infant development and maternal involvement.     

Reactions and serologic responses in young children and infants after administration of inactivated monovalent influenza A vaccine.

Infants, two years of age and under, received zonally purified whole-virus A/Aichi/2/68 (H3N2) influenza vaccine. Febrile responses, including one febrile convulsion, were observed in nine of 13 (69%). There was a fourfold rise in serum-neutralizing antibody in nine of 11 (82%) of those lacking initial antibody titers. Purity of the vaccine was documented by electron microscopy, absence of egg protein, and very low levels of endotoxin (0.048mug/dose). It is concluded that the toxicity of vaccine is related to the virus itself and that, despite the good antibody response, influenza vaccine should be administered only to children two years and under at high-risk of complications from influenza, if at all.     

Respiratory syncytial virus infections in infants: quantitation and duration of shedding.

Infants hospitalized with respiratory syncytial virus infection were studied to delineate the quantitative shedding patterns and duration of shedding of RSV. Nasal wash specimens collected daily from 19 infants contained a mean maximal titer of 4.34 log10 50% tissue culture infective doses per milliliter. On admission, the mean titer was 4.14 log10 TCID50, with no consistent decline until after Day 6. The mean duration of shedding for 23 patients until they were virus negative was 6.7 days with a range of 1 to 21 days. Quantities of RSV shed were significantly greater in infants less than one month of age and in infants with evidence of pulmonary consolidation on chest roentgenogram. Shedding extended for a significantly longer time in infants with lower respiratory tract disease than in those with clinical manifestations limited to the upper respiratory tract.