Resolution of precocious puberty following resection of fourth ventricular medulloblastoma: case report.

Medulloblastoma is a malignant embryonal tumor that arises in the cerebellum and invades the fourth ventricle, often resulting in obstructive hydrocephalus. Patients typically present with symptoms related to increased intracranial pressure and cerebellar dysfunction. The authors report a rare case of classic medulloblastoma with central precocious puberty (CPP) as its only presenting symptom.

Fludrocortisone in the management of an infant with a single ventricle and an overly tightened pulmonary artery band.

We describe a 2-month-old female infant with a univentricular heart and overly tightened pulmonary artery band (PAB) resulting in hypercyanotic episodes. Despite adequate doses of morphine and a beta-blocker, she continued to have multiple hypercyanotic events per day. Treatment with fludrocortisone resulted in resolution of these episodes and allowed performance of a Glenn shunt at 3 half months of age.

Low prevalence of autoantibodies to the insulin-like growth factor I receptor in children with short stature.

Inhibition of IGF-I action by circulating IGF-I receptor autoantibodies is a potential mechanism of IGF-I resistance in growing children. To define the prevalence of IGF-I receptor antibodies in short-statured children, we have examined serum and plasma samples from a well-characterized group of 34 short, prepubertal, growth hormone-sufficient children and three growth hormone-deficient children. IGF-I receptor purified from human placental membranes was radioiodinated by the solid phase radioiodination method.

Whole body nitrogen kinetics and their relationship to growth in short children treated with recombinant human growth hormone.

We studied the effects of growth hormone on retention of 15N-labeled amino acids in 34 short, prepubertal, growth hormone-sufficient children and three growth hormone-deficient subjects. All 34 non-growth hormone-deficient children had apparently normal circulating growth hormone molecules and no mutations were detected in the growth hormone or IGF-I genes of any subjects. Fibroblasts from 34 children responded normally when challenged with recombinant human IGF-I.

Low prevalence of insulin-like growth factor-I gene mutations in human growth disorders.

In an attempt to identify genetic lesions contributing to human growth disorders, we evaluated a prospectively recruited group of children with growth failure for mutations in the insulin-like growth factor-I (IGF-I) gene. Two complementary approaches were used: Southern blot analysis to examine the large scale organization of the gene, and a solution hybridization, nuclease protection assay to identify small alterations, such as point mutations. From a total of 61 subjects studied, 52 had no organic basis for their short stature.