1/10. Laron dwarfism: growth and immunoreactive insulin following treatment with human growth hormone.A 13 1/2-year-old boy with features of growth hormone deficiency had elevated fasting plasma GH levels (5.7 to 66 ng/ml). serum somatomedin values remained low despite treatment with human growth hormone. plasma GH values were suppressed following oral administration of glucose and increased following insulin-induced hypoglycemia, L-dopa, and arginine. chlorpromazine suppressed GH, both fasting and during IIH. These results suggest that the neuroendocrine mechanisms mediating GH secretion seemed to be intact. Peak plasma insulin levels increased in response to glucose administration after HGH suggesting that GH has a direct effect on the pancreatic beta cell which is not mediated by Sm. plasma testosterone values increased to adult male levels, but there was inadequate secondary sexual response. Growth was enhanced by HGH and may have been due to testosterone and/or insulin. Although Laron dwarfism may result from a receptor defect, an abnormality in GH structure is also possible.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/10. Suprahypophyseal dysfunction in a patient with asexual ateleiosis.The neuroendocrine function of a 19 years old female dwarf with primary amenorrhoea and lack of sexual development (asexual ateleiosis) was studied. Undetectable fasting plasma levels of growth hormone (GH) and a lack of response to three different provocative stimuli was observed. Oestrogen administratin did not modify the GH response. Thyroid and adrenal function were within normal limits. Undetectable plasma levels of immunoreactive oestradiol and lack of oestrogenic activity in vaginal smears indicated absence of ovarian function. Low levels of circulating gonadotrophins with a significant rise after synthetic LH-RH administration was demonstrated, while clomiphene citrate failed to induce ovulation. Following 6 months of continuous GH administration a significant increase in the growth rate was evident, whereas no pubertal development was observed. These data are interpreted as demonstrating the suprahypophyseal origin of the sexual infantilism in a patient with inappropriate GH secretion. It is suggested that a combined deficiency of LH-RH and GH-RH may account for the aetiology of this disorder.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
3/10. Acute lymphoblastic leukemia in a patient with pituitary dwarfism under treatment with growth hormone.An 18-year-old male with pituitary dwarfism, who had been on replacement of growth hormone (GH) and thyroxine for 3.5 years, developed acute lymphoblastic leukemia (ALL). The GH replacement was discontinued, and he was treated with a conventional protocol for ALL. A complete remission was obtained after 10 weeks. maintenance chemotherapy was given with reduced doses (1/4 to 1/2) of cytotoxic drugs. The platelet count soon reached 200,000/microL, but the hemoglobin level and white blood cell count improved only slowly, reaching 10.0 g/dL and 1,500/microL, respectively, after five months. He has been in complete remission with a hypocellular bone marrow for nearly 15 months. Since GH can stimulate the proliferation of some normal and leukemic hemato-lymphoid cells, the slow remission induction and the prolonged anemia and leukocytopenia after remission, may have been related to the absence of GH in this patient.- - - - - - - - - - ranking = 0.010915978705586keywords = bone (Clic here for more details about this article) |
4/10. Growth in a case of Russell-silver syndrome treated for hypopituitarism.The growth characteristics of Russell-silver syndrome (RSS) include dwarfism of prenatal onset, moderate retardation of bone age and normal postnatal height velocity. We describe a case of hypopituitarism in a girl with typical RSS who suffered from a severe trauma at birth. Signs of hypopituitarism appeared during childhood. Before substitutive treatment, a short course of human growth hormone (hGH) induced a moderate rise in plasma IGF-I levels which was within the range observed in other pituitary dwarfs. Under replacement therapy, catch-up growth was similar to what is observed in other growth hormone deficient children. However, bone age matured much faster than chronological age. This observation appears to be a particular feature of RSS, possibly enhanced by hGH therapy. An improvement of adult height beyond the final height usually observed in RSS children without endocrine disturbances should therefore not be expected from hGH therapy. Growth hormone deficiency and RSS do not appear to be causally related. However, in each child with RSS, a particular attention should be given to a decreased height velocity, a severely delayed bone age as well as a history of major perinatal problems. Should one of these factors be found, a careful evaluation of the hypothalamo-pituitary axis ought to be performed with, accordingly, an appropriate substitutive therapy.- - - - - - - - - - ranking = 1.0327479361168keywords = endocrine, bone (Clic here for more details about this article) |
5/10. Growth hormone and androgen effects in the third decade.Two male hypopituitary dwarfs were treated with human pituitary growth hormone (hGH) from the ages of 24.3 and 22.7 years, at bone ages of 13-14 years TW2-RUS. Height, weight, upper arm circumference and skinfolds, and skeletal maturation were studied during 1.7 and 2.2 years of hGH alone, followed by 3.1 and 3.0 years of hGH combined with exogenous androgen. Although height growth rate was doubled by hGH alone, actual growth during first year was only 2.2 and 3.5 cm. Addition of androgen caused growth rate of about 8 cm in first year, and total height gains of 17 and 16 cm. These figures are similar to those of normal late maturers, of hyposomatotropism with spontaneous puberty, and of hypogonadotropic hypogonadists receiving androgen. So at pubertal bone age hGH effect on height is largely dependent on presence of androgen, irrespective of chronological age. arm fat and muscle underwent profound changes under influence of hGH alone (particularly fat) and with androgen (particularly muscle).- - - - - - - - - - ranking = 0.021831957411173keywords = bone (Clic here for more details about this article) |
6/10. Pseudopituitary dwarfism due to resistance to somatomedin: a new syndrome.The case of an infant is described who at birth was already small and postnatally grew extremely slowly. At age 3 the girl's height was 65 cm, weight 5.6 kg, bone age 21 months. Basal plasma GH was 36-66 ng/ml, basal SM activity was rather high, being around 2.0 U/ml. RIA- and RRA-SM were also increased. Prolonged GH administration did not raise plasma SM. There was a tendency for hypoglycemic episodes in the presence of low insulin levels. Receptor studies with skin fibroblasts showed a diminution of the specific binding of SM-C by 50%. Apparently only the specific IGF-receptor is defective whereas the insulin receptor responds to the increased SM with hypoglycemia. The observation that the high plasma SM did not suppress the enhanced GH-secretion suggests that perhaps the hypothalamic IGF-receptor was also defective.- - - - - - - - - - ranking = 0.010915978705586keywords = bone (Clic here for more details about this article) |
7/10. Dysplastic optic discs in association with transsphenoidal encephalocele and hypopituitary dwarfism: a case report.A patient with a basal encephalocele, dysplastic optic discs and hypopituitary dwarfism is described. The importance of identifying associated structural defects and endocrine dysfunction in patients with dysplastic optic discs is emphasised.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
8/10. Globular-like deformities of the cornea in a patient with dwarfism.A family affected with pituitary dwarfism transmitted probably as an X-linked recessive or autosomal recessive condition, is described. The propositus had globular-like changes in his cornea, this latter as a sequela of endocrine disturbances, is suggested and discussed.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
9/10. Short stature in a patient with klinefelter syndrome and growth hormone deficiency.We report on a child with klinefelter syndrome and short stature due to idiopathic growth hormone deficiency (IGHD). His height was below the mid-parental height, with a significant delay in bone age. Height velocity increased from 4.5 to 8.2 cm/year during 1 year of GH therapy and typical catch-up growth was observed. No adverse reactions to the treatment were observed. We wish to emphasize the rare association between klinefelter syndrome and IGHD and the apparent effectiveness and safety of recombinant somatotropin treatment in aneuploid patients.- - - - - - - - - - ranking = 0.010915978705586keywords = bone (Clic here for more details about this article) |
10/10. Morning glory syndrome in children. association with endocrine and central nervous system anomalies.OBJECTIVE: To identify morning glory syndrome, an uncommon optic disc anomaly. Generally, it is an isolated ocular abnormality; however, some cranial facial and neurologic associations have been reported. patients: We herein report two patients with morning glory syndrome and associated pituitary dwarfism. In one patient, the pituitary insufficiency was secondary to compression of the pituitary gland by a basal encephalocele; in the second patient, causative factors were not identified. CONCLUSIONS: patients with morning glory syndrome should have a complete general physical examination and growth evaluation so that early recognition and treatment of the patient with pituitary dwarfism can occur.- - - - - - - - - - ranking = 4keywords = endocrine (Clic here for more details about this article) |
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