Growth and rate of increase in stature.
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Biomedical subjects
Publications and source records attributed to R Rappaport.
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Dexamethasone has been proposed as therapy of congenital adrenal hyperplasia in adolescents with menstrual abnormalities. Seven patients previously treated with hydrocortisone (mean dosage of 26 mg/m2/day) or prednisone (6 mg/m2/day), received dexamethasone doses between 0.3 and 0.73 mg/m2/day during at least 4 months. Regular menses and satisfactory adrenal suppression were obtained in most cases, but the occurrence of severe striae, sometimes with excessive weight gain, led to the cessation of this treatment. Analysis of data indicate that glucocorticoid activity of dexamethasone is 50-90 times more potent than that of hydrocortisone. Its use, after cessation of growth, should be carefully monitored, starting with much lower doses when necessary.
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Somatomedins and vitamin D metabolites have been shown to interact with cartilage metabolism. In the present work their stimulating effect on the growth of rabbit cultured chondrocytes is studied. These cells possess specific binding sites for an insulin like preparation (ILA) distinct from insulin binding sites. They also possess specific nuclear binding sites for one vitamin D metabolite : 24,25- (OH)2D3. In the absence of seric factors, these two types of hormone stimulate the proteoglycan synthesis but do not increase DNA polymerase activities in chondrocytes. Another type of growth factor enhances the cellular multiplication but inhibits the specific protein synthesis (Fibroblast growth factor or Retinal factor). When DNA polymerase activities are induced by fetal calf serum, ILA or vitamin D metabolites may increase the rate of DNA synthesis.
Female pseudo-hermaphroditism is due, most frequently, to the presence of androgens during early fetal life as observed in congenital adrenal hyperplasia, or hormone administration to pregnant patients. In this report 121 cases are reviewed. The importance of a genitographic assessment of the internal genitalia, principally the urogenital sinus is documented. The occurrence of fetal masculinisation is due to androgen production as early as the 9th week of gestation. This process is very likely ACTH dependant, however the role of HCG still remains likely at this very early stage of adrenal activity. It is of interest that adrenal androgens are not capable of stabilizing the Wolfian derivatives. Mullerian derivatives develop normally, as well as ovaries. Progestins have been shown to masculinize the fetus in a few cases, probably by interfering with the 3 beta hydroxysteroid deshydrogenase system. The same agents are associated with a large number of non specific malformations.
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The present study was designated to investigate causes of hGH treatment failure in 59 prepubertal children, with idiopathic or organic GH deficiency. Children with height gain of less than 6 cm during the first year of treatment were the oldest, those with tallest stature, and had a lower growth rate prior to treatment. Increase of growth rate was negatively correlated with pre-treatment growth rate. Poor results were chiefly observed in children with tumoral organic hypopituitarism or after irradiation of the brain. Finally hGH doses appeared to be similar when two groups with respectively poor and good results were compared, indicating a major role of endogenous factors.
In a seven year old boy a chordoma of the clivus has been treated by X-ray therapy after partial neurochirurgical resection. A recurrence of the tumor is noted 10 years after at the the border of the X-ray field. The final size of the sexually mature boy is -- 4 DS below the mean with a complete defect in GH secretion after arginin and insulin stimulation. TSH secretion insuficiency and high HPr levels are shown by TRH stimulation test. The length of survey in this particular case has allowed the demonstration of severe stunting growth which is in part a consequence of hypothalamo-pituitary axis irradiation.
We report the case of a patient with a case of cytomegalovirus (CMV) colitis, which presented as a flare-up of her ulcerative colitis. Standard treatment for the flare-up, which included intravenous corticosteroids, bowel rest, topical salicylates and ultimately colectomy were not effective. The patient did not improve until therapy with intravenous ganciclovir was initiated. There have been 26 previous reports of CMV colitis complicating inflammatory bowel disease (IBD). The diagnosis is not frequently entertained and, if not made, leads to a high rate of colectomy (67 percent) and mortality (33 percent). Appropriate antiviral therapy appears to eliminate these complications, thus a high index of suspicion for CMV superinfection in cases of IBD refractory to traditional therapy is warranted.