A prospective study of the development of growth hormone deficiency in children given cranial irradiation, and its relation to statural growth.

Although GH deficiency (GHD) is the most frequent hormonal abnormality that occurs after cranial radiation, the natural course of this complication and its relationship to growth in children are not known. Therefore, we undertook a 2-yr prospective study of 16 children, aged 1.7-15 yr at the time of treatment, who received cranial [31-42 Gy (1 Gy = 100 rads)] and spinal radiation for medulloblastoma or ependymoma (group I).

[Autoimmune polyendocrinopathy and chronic mucocutaneous candidiasis].

The authors report the case of a child who, at the age of 18 months showed signs of hypoparathyroidism together with gastrointestinal, then buccal, then ungual candidiasis. Acute adrenal failure occurred when he was 5 1/2 years' old. At the age of 10, the patient developed alopecia areata and interstitial keratitis.

Lack of adrenarche in two children with precocious puberty secondary to hypothalamic hamartoma.

Adrenarche, which occurs earlier than gonadarche in normal children, is marked by increases in plasma dehydroepiandrosterone and its sulfate (DHAS). Adrenarche and gonadarche can be dissociated in various situations, e.g.

Growth hormone response to growth hormone-releasing hormone (hp GHRH1-44) as an index of growth hormone secretory dysfunction after prophylactic cranial irradiation for acute lymphoblastic leukemia (24 grays).

The growth hormone response to growth hormone releasing hormone hp GHRH1-44 (2 micrograms/kg i.v.) was studied in 19 prepubertal children who had been irradiated with 24 Gy for acute lymphoblastic leukemia (ALL) or lymphosarcoma (LS) at a mean chronological age of 4 10/12 years (limits 10/12 to 9 years).

[Treatment of craniopharyngiomas in children. Retrospective analysis of 50 cases].

In order to evaluate the results of surgery and radiotherapy upon craniopharyngiomas in children, the authors review their own series of 50 cases treated from 1968 to 1985 and the literature. After subtotal removal, the recurrence-free survival rate, 10 years postoperatively, was 37%. This rate was significantly higher (72%) when the subtotal removal was followed by irradiation with a dose of 50 to 55 grays: however, deafness and severe neuro-psychological and intellectual sequelae were frequent in these patients.

Leydig cell insufficiency after testicular irradiation for acute lymphoblastic leukemia.

Leydig cell function in 21 boys with acute lymphoblastic leukemia who had been treated by bilateral direct testicular irradiation (12 X 2 Gy) at 8.4 +/- 0.7 years, was evaluated 3.

MRI of hypothalamic hamartomas in children.

Eight cases of hypothalamic hamartomas (HH) diagnosed in children by clinical, biological and CT studies have been explored by MRI and compared with a control group of twenty children without hypothalamohypophyseal signs. MRI revealed the hamartoma in all cases. Its location and position relative to the floor of the third ventricle was precisely determined.

[Treatment with growth hormone: results and new perspectives].

The use of human growth hormone is being extensively reevaluated in children with hypopituitarism, as we enter a new phase with sufficient supply of recombinant hGH. Simultaneously much emphasis has been placed on the methods of measurement of GH secretion in order to define a more appropriate cutoff level defining partial GH deficiency. Among non conventional indications of hGH, Turner syndrome has turned out to be most important with very encouraging short term results.

Somatomedin-C and growth in children with precocious puberty: a study of the effect of the level of growth hormone secretion.

This study was undertaken to investigate the role of GH secretion in the pubertal increase in plasma somatomedin-C (Sm-C) concentrations and its relation to growth in children with true precocious puberty (PP) and normal or deficient GH secretion. We studied 37 children (9 boys and 28 girls), divided into 3 groups according to their pubertal stages and their peak stimulated plasma GH concentration. Group I (n = 20) contained patients with PP and normal GH secretion.

[Craniopharyngioma in children. Endocrine evaluation and treatment. Apropos of 37 cases].

Endocrine function was studied in 37 children treated for craniopharyngioma by total (22 cases) or partial (12 cases) excision and complementary or isolated irradiation (9 cases). Height deficiency was the only revealing sign in only 20% of cases. Skull X-rays showed patent abnormalities at the first examination in 36 of 37 cases.

[Antenatal diagnosis of goiter by ultrasonography].

A case of foetal goiter diagnosed by ultrasonography is reported. A first child had been under treatment since the age of 4 months for goitrous hypothyroidism. A second pregnancy developed normally.

[Precocious puberty caused by a suprasellar arachnoid cyst. Analysis of 6 cases].

The authors report 6 cases (4 girls and 2 boys) with central precocious puberty associated with a suprasellar arachnoid cyst. Precocious puberty is rarely the presenting sign of arachnoid cysts. It was characterized by early onset, patent symptoms and frequent association (3 of 6 cases) with growth hormone deficiency.

[True precocious puberty in non-tumor hydrocephalus. An analysis of 16 cases].

True precocious puberty occurred in 16 children (15 girls and 1 boy) with non tumoral shunted hydrocephalus at a mean age of 6.8 years. They had mild clinical manifestations of precocious puberty, and the other pituitary functions were found to be normal.

[Treatment of central precocious puberty with an LHRH analog. Effect on growth and bone maturation after 2 years of treatment].

Eighteen children (15 girls and 3 boys) with true precocious puberty have been treated with an LHRH analogue (HOE 766, Buserelin suprefact) given subcutaneously during one (n = 11) or two (n = 7) years. Six of 18 children had organic precocious puberty, but their responses to therapy did not show any difference. A satisfactory suppression was achieved in 16 cases with plasma testosterone below 0.

Treatment of central precocious puberty with an LHRH agonist (Buserelin): effect on growth and bone maturation after three years of treatment.

The LHRH analog Buserelin was used to treat 27 children (21 girls, 6 boys) with central precocious puberty. Nineteen patients had idiopathic precocious puberty and 8 had organic lesions (hamartoma, hydrocephalus or suprasellar arachnoid cyst). All patients received 20 or 30 micrograms/kg/day s.

[Chemotherapy and ovarian function. Retrospective analysis in 17 girls treated for malignant tumor or hematologic disease].

Ovarian function was investigated in 17 patients aged 13 5/12 to 30 years who had received various types of combined chemotherapy without any irradiation. Ovarian insufficiency was found in 6 cases with amenorrhea (n = 5) or irregular menstruations (n = 1). There is a high risk of sterility in these cases although as described in one case, a normal pregnancy occurred in spite of evidence of ovarian failure.

[Role of pelvic ultrasonography in the diagnosis, therapeutic indications and surveillance of central precocious puberty].

The use of pelvic ultrasonography was evaluated as a diagnostic and follow-up tool in girls with precocious puberty. Before treatment 23 of 33 patients with central precocious puberty presented an increased size of the uterus. In 10 cases with prepubertal size of the uterus, the precocious puberty was only beginning or of mild severity.

Isolated growth hormone (GH) deficiency type 1A associated with a double deletion in the human GH gene cluster.

The gene deletions responsible for isolated GH deficiency type 1A were characterized by direct analysis of genomic DNA prepared from the leukocytes of two affected children. The probands had typical symptoms of severe isolated GH deficiency complicated by antibody development and growth arrest after human (h) GH treatment. DNA analysis using the restriction endonucleases Eco RI, Bam HI, and Hind III revealed that the restriction fragment containing the hGH-N gene was absent along with those bearing the human chorionic somatomammotropin (hCS)-A and -B and hGH-V sequences.

[Severe osteopenia in young children with hyperthyroidism. 2 cases].

The cases of 2 young children with Grave's disease and severe bone demineralization are reported. In one case, spontaneous fracture and a collapsed vertebra were noted. Increased serum alkaline phosphatase levels and normal 1.

Growth, growth hormone secretion and somatomedin C after cranial irradiation for acute lymphoblastic leukemia.

In a large group of 74 patients irradiated with 2400 rad for acute lymphoblastic leukemia an unusually high frequency of complete GH deficiency was observed (40%). Only 11 out of 46 prepubertal children had growth retardation and seven children received hGH treatment. On the contrary 11 other prepubertal cases had normal growth rates in spite of lack of response to AITT.

Long-term results of GnRH analogue (Buserelin) treatment in girls with central precocious puberty.

The GnRH analogue Buserelin was given for one year to six girls with central precocious puberty in a daily subcutaneous dose of 20 micrograms/kg/day. A decrease of plasma estradiol and vaginal maturation index to prepubertal values was obtained in 5 out of 6 cases. Bone maturation decreased and final predicted adult height improved significantly.