Membranous glomerulonephritis and hepatitis B surface antigen in children.

Of 33 children with membranous nephropathy screened for HBs Ag, 14 were found to be HBs Ag carriers, whereas HBs Ag was detected in 3 of 170 and 4 of 100 children with glomerular and nonglomerular kidney diseases, respectively. HBs Ag was often associated with acute hepatitis at onset (five patients) or with elevated transminases values. This high incidence and the prevalence of an unusual subtype (ayw2) suggest a relationship between HBs Ag and the glomerular lesions. Using immunofluorescence, however, HBs Ag could not be detected within the deposits, so that the nature of the relationship cannot be considered as established. The clinical outcome (50% remission), the plasma complement component disturbances, and findings by immunofluorescence did not differ from those observed in children with MGN without detectable HBs Ag.     

Diabetes insipidus in children. III. Anterior pituitary dysfunction in idiopathic types

Seventeen patients with idiopathic diabetes insipidus occurring in childhood were observed from 4 to 26 years (mean duration 15 1/2 years). The diagnosis of idiopathic diabetes insipidus was based on routine clinical examination and careful, repeated neuroradiologic investigations. Anterior pituitary dysfunction was present in some of these patients. Growth hormone deficiency was present in six children, insufficient thyroid stimulating hormone secretion after thyrotropin-releasing hormone stimulation was demonstrated in one, and abnormal response to a metyrapone test in two. Elevated prolactin and TSH values were present in three and two patients, respectively. Some of these abnormalities were transitory. The presence of anterior pituitary dysfunction in idiopathic diabetes insipidus indicates that the destructive process is not localized to vasopressin synthesizing cells but may also involve other parts of the hypothalamus.     

Acute hemorrhagic, hepatic, and neurologic manifestations in juvenile rheumatoid arthritis: possible relationship to drugs or infection

Seven children with juvenile rheumatoid arthritis had a syndrome characterized by hemorrhage and neurologic, hepatic, hematologic, and metabolic manifestations. The disease did not seem to conform clearly to the characteristics of Reye syndrome or any other well-known entity. This severe complication may be induced by macrophage activation secondary to a drug or intercurrent infection. Our data suggest that a sudden fall in erythrocyte sedimentation rate or in platelet and fibrinogen levels may mark the start of this complication and may be an indication for rapid steroid therapy.     

Impaired defense against vaccinia in a child with T-lymphocyte deficiency associated with inosine phosphorylase defect.

A case of progressive vaccinia associated with a profound deficiency of cellular immunity and a defect in inosine phosphorylase is described. A striking contrast was observed between humoral immunity, which showed little if any impairment, and a severe cellular defect affecting both markers and functions of T lymphocytes. The child died despite treatment with methosazone, levamisole, transfer factor, irradiated blood transfusions, and a thymus graft. An adequate serum level of antibody to vaccinia virus was obtained by transfer of specific immunoglobulins, but this failed to stop the progression of the disease. This observation suggests that host defense against vaccinia infection is mainly mediated by cellular immunity.     

Endocrine aspects and tumoral markers in intracranial germinoma: an attempt to delineate the diagnostic procedure in 14 patients

This work outlines the endocrine abnormalities associated with intracranial germinomas (14 patients before treatment). Diabetes insipidus of various intensity was present in all cases. Adipsia is often present as well. In six patients, the first neuroradiologic examination after the onset of DI was normal. Growth hormone secretion was deficient in 11 of 13 patients. Abnormalities of TSH response to TRH were present in nine of ten (four insufficient and five exaggerated responses to TRH). Anterior pituitary dysfunction could not be predicted by the tumor site as determined by radiologic criteria. Tumoral markers have also been studied. Elevated plasma tumoral markers were found in four patients of 11 studied. Tumoral germinoma cells were present in CSF in five patients of ten, in one of them before radiologic confirmation. Pertinent endocrine evaluation and search for tumoral markers was of great value in systematic follow-up of patients with central diabetes insipidus, and could lead to early diagnosis and treatment of the tumor.     

Metabolism of theophylline to caffeine in premature newborn infants.

Plasma concentrations of theophylline and caffeine in seven premature neonates receiving theophylline orally for treatment of apnea at age one to 9 days were measured by high performance liquid chromatography, ultraviolet spectrophotometry, and mass spectrometry. Plasma concentrations of caffeine increased from 1.8 mg/l (range = 0.1 to 3.7) at day one to 3.7 mg/l (1.3 to 8.0) seven days after initiation of theophylline therapy. Similarly, plasma concentrations of theophylline were 4.6 mg/l (1.5 to 7.5) and 11.0 mg/l (4.0 to 19.0) on days one and 7 of theophylline therapy, respectively. In contrast, four normal adult volunteers given theophylline orally for eight to ten days had plasma theophylline concentrations ranging from 3 to 14 mg/l but no measurable caffeine. This indicates that caffeine is a biotransformation product of theophylline in premature neonates and that the metabolic pathway followed by theophylline in premature infants includes a methylation reaction producing caffeine, whereas in adults, the major metabolic pathway involves oxidative reactions (demethylation and oxidation). Some pharmacologic effects attributed to theophylline during chronic therapy for apnea may in part be due to caffeine. Routine monitoring during theophylline therapy in premature neonates with apnea should include plasma concentrations of both theophylline and caffeine in order to assess the total methylxanthine load.     

Plasma levels, half-life values, and correlation with physiologic assays for growth and immunity

To assess the gonadal effects of chlorambucil given to prepubertal and pubertal boys, testicular function of 21 adolescents or young men, treated with chlorambucil for nephrotic syndrome before or during puberty, have been evaluated. Evaluations have been based on penis and testicular size, serum FSH, LH and testosterone values, and sperm count. Nine patients have testicular hypotrophy, 13 have significantly elevated FSH, 17 have azoospermia, two have severe oligospermia, and repetitive sperm counts show no improvement. A quiescent state of the epithelium of the seminiferous tubules does not protect against chlorambucil toxicity, which seems to be related to the cumulative dose. Chlorambucil should be used with caution, even in children.