Acute glomerulonephritis secondary to lobar pneumonia

We report 4 cases of children who developed lobar pneumonia and subsequently acute glomerulonephritis, all within a period of six weeks. In one case streptococcus pneumonia type 5 was isolated, a strain not previously described in the literature as being nephritogenic.     

Neutrophil function response to aerobic and anaerobic exercise in female judoka and untrained subjects

OBJECTIVES: Recent studies have indicated reduced immunity in trained athletes. AIM: To assess the effects of aerobic and anaerobic exercise on the phagocytic process in 18-26 year old trained female judoka (n = 8) and untrained controls (n = 7). METHODS: Each subject participated randomly in two different testing sessions (aerobic, 20 minutes of treadmill running at 70-80% of maximal heart rate; anaerobic, Wingate anaerobic test). Venous blood samples were drawn before, immediately after, and 24 hours after each session. RESULTS: There were no significant differences in basal values of net chemotaxis (chemotaxis--random migration), bactericidal activity, and superoxide anion release between the judoka and the untrained women. There was a significant decrease in net chemotaxis 24 hours after the aerobic exercise in both the judoka (from 64 (19) to 39 (13) cells/field, p < 0.02) and the untrained controls (from 60 (7) to 47 (12) cells/field, p < 0.05). Bactericidal activity and superoxide anion release did not change significantly after aerobic exercise in either group. There were no significant changes in net chemotaxis, bactericidal activity, and superoxide anion release after anaerobic exercise in either the judoka or untrained women. CONCLUSIONS: The decrease in net chemotaxis after aerobic, but not after anaerobic, exercise, suggests that net chemotaxis is affected by the combination of exercise intensity and duration, and not by the exercise intensity itself. Similar effects of both exercise sessions in the judoka and the untrained women suggest that training had no effect on neutrophil function response to aerobic and anaerobic exercises.     

Should we treat elevated thyroid stimulating hormone levels in obese children and adolescents?

OBJECTIVE: To examine the prevalence of abnormal thyroid function tests among obese children and adolescents, and to study the effect of thyroid hormone supplementation on body weight, linear growth and lipid profiles in these children. DESIGN: Thyroid function tests and lipid profiles were measured in 196 obese children and adolescents. Thyroid auto-antibodies were measured in children with hyperthyrotropinemia (elevated thyroid stimulating hormone (TSH) and normal free thyroxine-FT4). All children with hyperthyrotropinemia participated in a combined dietary-behavioral-physical activity weight management intervention. Fifteen of the obese children with hyperthyrotropinemia were also treated with thyroid hormone substitution for 6 months and were compared to non-treated subjects (n = 26). RESULTS: Forty-one obese children had hyperthyrotropinemia (20.9%). Positive thyroid auto-antibodies were only found in 19.5% of these children. Treatment had no significant effect on body weight, linear growth and lipid profile, except for causing a greater decrease in triglyceride levels. TSH levels returned to normal ranges in the majority of children with hyperthyrotropinemia who participated in the combined intervention, irrespective of thyroxine treatment. CONCLUSIONS: Hyperthyrotropinemia is relatively common in obese children, but autoimmune thyroid disease accounts for a minority of the cases. TSH levels returned to normal in the majority of patients even without thyroid hormone administration. No beneficial effects on body weight, body mass index, linear growth and body lipids were found in treated subjects, suggesting that thyroid substitution is not necessary in most cases.     

Unusual late presentation of X-linked chronic granulomatous disease in an adult female with a somatic mosaic for a novel mutation in CYBB

Most patients with chronic granulomatous disease (CGD) have mutations in the X-linked CYBB gene that encodes gp91phox, a component of the phagocyte NADPH oxidase. The resulting X-linked form of CGD is usually manifested in boys. Rarely, X-CGD is encountered in female carriers with extreme expression of the mutated gene. Here, we report on a woman with a novel mutation in CYBB (CCG[90-92]-->GGT), predicting Tyr30Arg31-->stop, Val in gp91phox, who presented with clinical symptoms at the age of 66. The mutation was present in heterozygous form in genomic DNA from her leukocytes but was fully expressed in mRNA from these cells, indicating that in her leukocytes the X chromosome carrying the nonmutated CYBB allele had been inactivated. Indeed, only 0.4% to 2% of her neutrophils showed NADPH oxidase activity. This extreme skewing of her X-chromosome inactivation was not found in her cheek mucosal cells and is thus not due to a general defect in gene methylation on one X chromosome. Moreover, the CYBB mutation was not present in the DNA from her cheek cells and was barely detectable in the DNA from her memory T lymphocytes. Thus, this patient shows a somatic mosaic for the CYBB mutation, which probably originated during her lifetime in her bone marrow.     

Polycythemia of the preterm and full-term newborn infant: relationship between hematocrit and gestational age, total blood solutes, reticulocyte count, and blood pH.

The umbilical venous hematocrit at birth (Hct 1) and the peripheral venous hematocrit at 2 h of life (Hct 2) were determined in 78 healthy full-term and 14 healthy preterm newborn infants. Hct 1 was 51.6 +/- 4.1% in full-term infants and 50.8 +/- 4% in preterm infants. Hct 2 was 60.9 +/- 2 and 58.6 +/- 6.1% in full-term and preterm infants, respectively. Significant differences between Hct 1 and Hct 2 were found in both groups of infants (p less than 0.01). The blood viscosity increased significantly in both groups from birth to 2 h of life. Neonatal polycythemia (Hct higher than 70% at 2 h of life) was detected in only 3 full-term infants (3.8%). They received partial exchange transfusion. There was a positive linear correlation of Hct 1 with Hct 2 in full-term newborns (r = 0.71, p less than 0.001) and preterm infants (r = 0.57, p less than 0.02). No infants with Hct 1 equal to or below 50% had Hct 2 higher than 65%. None with Hct 1 between 51 and 54% had Hct 2 higher than 70%. Neither Hct 1 nor Hct 2 correlated with birth weight, gestational age, total blood solutes, or reticulocyte counts at birth in either group. An inverse linear correlation was found between blood pH at birth and Hct 2 in preterm newborn infants (r = 0.66, p less than 0.02).     

Early immunological defects in comatose patients after acute brain injury

OBJECT: The aim of this prospective study was to evaluate the phagocytic, humoral, and cellular arms of the immune system in comatose patients shortly after severe brain injury and to compare the findings with those reported earlier in patients in a persistent vegetative state. The study was conducted in intensive care units and immunology laboratories of university-affiliated hospitals in central Israel. METHODS: The study group consisted of 14 men aged 16 to 65 years who were comatose as a result of acute brain injury due to mechanical trauma. All were studied within 72 hours of injury. Brain damage was severe in all cases (Glasgow Coma Scale score < 8). Healthy age- and sex-matched volunteers served as simultaneous controls. Infections arose in nine (75%) of the 12 patients in whom data were available; the cumulative mortality rate was 38% (five of 13 patients in whom outcome data were available). Every patient exhibited one or more defects in at least one arm of the immune system. Significant deficiencies were noted in neutrophil superoxide release, immunoglobulin (Ig)G, IgG1, IgM, C1q, C2, properdin, alternate C pathway, T cells, T helper cells, T suppressor cells, and natural killer cells. In an earlier series of patients examined by the authors months after the primary insult, these impairments were absent in most of the patients in the vegetative state. CONCLUSIONS: Significant deficiencies of the immune system, particularly the cellular arm, are precipitated by severe brain injury within 72 hours of the event. These impairments probably play a role in the high rate of complicating infections and multiple organ failure. Together with earlier findings, the results of this study indicate that if brain-injured patients survive these hazards, their immune system will eventually recover.     

Paediatric Behçet disease manifested as recurrent myositis: from an incomplete to a full-blown form

A 12-year-old boy presented with a limp and findings suggesting localised myositis of his right calf and a working diagnosis of Behçet disease was made. During 3 years of follow-up, he had another three episodes of calf myositis, all responsive to corticosteroids within days. Conclusion A case of recurrent localised myositis as a main manifestation of Behçet disease is reported. The evolution of incomplete Behçet disease, which is common in children, to the full blown form, with the emphasis on muscle involvement and the importance of early diagnosis of Behçet disease, is discussed.     

Clinical and laboratory findings of spotted fever in Israeli children

In a prospective study of 70 Israeli children with spotted fever the major clinical features were fever (100%), skin rash (98.5%), myalgia (54%) and vomiting (40%). Thrombocytopenia (75%) and hyponatremia (62.5%) were common, but were not associated with increased mortality. Antibodies to Rickettsia conorii were detected by the indirect immunofluorescent antibody assay. In one patient Rickettsia was grown from blood. Contacts with dogs were reported in 17 of 40 patients questioned, and in only 2 was a canine tick bite obvious. Hospitalization was required in 11 (16%) patients. There was 1 fatality. The rickettsia responsible for spotted fever in Israel appears to be an antigenic variant of R. conorii. Early recognition and treatment of this disease permits rapid eradication of the rickettsiae and facilitates complete recovery.