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Biomedical subjects

R Brauner

Publications and source records attributed to R Brauner.

At least 127 records · Page 7Linked to original sources
PubMed1988

[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. After radical excision the rate of recurrence was the lowest, with a 10 year-recurrence-free survival rate of 88%. The postoperative mortality was low in case of pre-chiasmatic craniopharyngiomas, but high in case of retro-chiasmatic ones. Nevertheless, it appears from the recent literature data that impressive surgical improvements are to be expected from new surgical routes, associated with the use of the most recent technologies. The conclusions of this study are: 1. Radical excision is the treatment of choice; 2. If radical excision is not possible, surgery should be followed by irradiation to lower the risk of recurrence; 3. However, in view of the dangers of radiotherapy to the growing brain, it should be delayed as long as possible, particularly in the case of young children, and used only when tumor recurrence has been demonstrated.

Adolescent↗
PubMed1987

[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. However, at 27 weeks a first ultrasonography showed hypertrophy of the foetus' thyroid gland, and this was confirmed by a second ultrasonic examination performed at 34 weeks. The femoral ossification centre, which usually appears between 31 and 33 weeks of amenorrhoea, was absent. The child was born at 41 weeks. Additional examinations confirmed the presence of hypothyroidism with goiter and disorders of organification. This is the third case of foetal goiter discovered in utero by ultrasonography. The important therapeutic implications of such a diagnosis (appropriate neonatal intensive care in case of compressive goiter, very early treatment of hypothyroidism) open new possibilities of monitoring in pregnant women whose history suggests a risk of foetal goiter.

Amniotic Fluid↗
PubMed1987

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.c. of Buserelin, and we obtained plasma E2 less than 20 pg/ml, vaginal maturation index less than 30 in girls or plasma testosterone less than 0.3 ng/ml in boys. The mean growth rate decreased from 9.3 +/- 0.5 to 4.6 +/- 1.3 cm/year after 3 years. The velocity of skeletal maturation decreased so that the final height prediction improved by a mean value of 1.6 SD. As the follow-up increases, this study confirms that LHRHa therapy is effective and potentially improves the final height of children presenting active and severe central precocious puberty.

Aging↗
PubMed1987

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. In group II (n = 8), PP was accompanied by GH deficiency. Group III (n = 9) patients were GH deficient and prepubertal. The mean plasma Sm-C (RIA) levels in groups I and II were 2.01 +/- 0.17 (+/- SEM) and 0.59 +/- 0.21 U/mL, respectively (P less than 0.001), and it was 0.09 +/- 0.01 U/mL in group III (P less than 0.001 compared to group II). The higher mean plasma Sm-C level in group II compared to that in group III could be related to a significantly higher GH response to arginine-insulin stimulation (P less than 0.02), although this value was in the hypopituitary range. The mean growth rate in group II (6.8 +/- 0.9 cm/yr) was also much higher than the rate in group III (1.9 +/- 0.5 cm/yr; P less than 0.001) and only slightly lower than that in group I (90 +/- 0.8 cm/yr; P less than 0.05). These data indicate that plasma Sm-C values are closely correlated with even small changes in GH secretion. The observed growth rates could, in general, be linked to plasma GH and Sm-C levels, as modulated by sex steroids, in these patients with precocious puberty.

Adolescent↗
PubMed1987

[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 0.5 ng/ml (boys) or estradiol below 25 pg/ml and vaginal maturation index below 35 (girls). The mean annual height gain diminished from 9.5 +/- 0.8 cm during the control year to 7.7 +/- 0.7 cm and 5.1 +/- 0.7 cm during the first and second years of therapy respectively (p less than 0.05). Simultaneously, the mean bone age of 10.4 +/- 0.4 yr at onset of treatment, was 11.4 +/- 0.4 yr after one year and 11.8 +/- 0.3 yr after two years. These changes explain an average increase of predicted height of 5.7 cm after two years of treatment with the LHRH analogue. At least on the basis of these data with two years follow-up, this treatment seems satisfactory. We did not find anti-Buserelin antibodies in any of these patients.

Body Height↗
PubMed1987

[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. Analysis of 24 children before and after surgery helps defining the part played by surgery in endocrine deficiencies: the frequency of thyroid and GH deficiencies is poorly changed after surgery as they are most often already present before surgery. On the other hand, the frequency of corticotropin and antidiuretic deficiencies is highly increased after surgery. Gonadotropic deficiency is almost constant after surgery. The final height is greater than 2 SD in 9 of the 14 patients whose growth is completed.

Adolescent↗
PubMed1986

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. A total of about 40 kilobases DNA were absent due to two separate deletions flanking the hCS-L gene. The two affected siblings are homozygous for this rearrangement of the hGH/hCS gene cluster, which could have been generated by homologous crossing over between two different chromosomes, one bearing one of the previously described deletions of the hGH-N gene, and one bearing a deletion of DNA containing the hCS-A, hCS-B, and hGH-V sequences. Alternatively, this abnormality could have been generated by a complex intrachromosomal rearrangement. The parents, who are consanguinous, have DNA restriction patterns consistent with heterozygosity for this double deletion. This type of deletional mutation is the first involving multiple deletion of the hGH and hCS gene cluster.

Adolescent↗
PubMed1986

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. Plasma SmC values were correlated with growth rates but were discrepant with GH responses to AITT in some cases. Because of this high frequency of GH dysregulation further growth at time of puberty should be carefully documented.

Body Height↗
PubMed1986

[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. During treatment with a LHRH analogue, changes in uterine size were slow in spite of a satisfactory and rapid control of estrogen secretion. At onset of treatment, transient ovarian cysts were seen in 2 patients. In our experience, pelvic ultrasonography did not provide significant information on the control of the disease by LHRH analogue therapy. Of 16 girls with presumed premature thelarche, 3 presented signs of estrogenic stimulation of the uterus. It remains a useful technique to rule out the presence of ovarian cysts or tumors at time of diagnosis.

Buserelin↗
PubMed1986

[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 1.25-(OH)2D and 24-25 (OH)2D levels were observed when 25(OH)D levels were decreased. These anomalies, not described previously in young hyperthyroid children, should be kept in mind. Bone X-ray and calcium phosphorus metabolism investigations should be part of routine check-ups of young children with Grave's disease.

Calcium↗
PubMed1986

[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. Cyclophosphamide seemed to be less harmful when given before puberty. Great variations in individual susceptibility for relatively low doses were observed with this drug. The combination with other drugs in some protocols might play a role in these cases. At variance with results reported in adults, the MOPP chemotherapy used in children with Hodgkin's disease did not induce ovarian dysfunction.

Adolescent↗
PubMed1985

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.

Adolescent↗
PubMed1985

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. This analogue of GnRH appears to be an effective medication for gonadotropin dependent precocious puberty in girls.

Buserelin↗
PubMed1985

Precocious puberty secondary to cranial irradiation for tumors distant from the hypothalamo-pituitary area.

This retrospective study is the first report of the occurrence of central precocious puberty in 6 children having received cranial irradiation. Pubertal development took place at a mean age of 7 5/12 years (6 10/12-7 10/12 years in 5 girls and at 9 years in 1 boy). They had received 2,400-4,500 rad at a mean age of 5 2/12 years. In addition, 5 children had GH deficiency so that their growth spurt was blunted and 3 of them were left with an extremely short stature. This condition would require a therapeutic approach combining the use of an LHRH analogue and hGH therapy when necessary in order to protect from too rapidly progressing bone maturation.

Adolescent↗