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Biomedical subjects

S D Frasier

Publications and source records attributed to S D Frasier.

At least 37 records · Page 2Linked to original sources
PubMed1983

Ullrich-Turner syndrome associated with interstitial deletion of Xp11.4 leads to p22.31.

The full phenotype of the Ullrich-Turner syndrome (UTS) is thought to be due to loss of the short arm of X. We report a 16-year-old girl with lack of secondary sexual development, amenorrhea, and short stature. She had thyroiditis and numerous other UTS manifestations and was found to have a non-mosaic 46,X,del(Xp) chromosome abnormality. Breakpoints occurred at p11.4 and p22.31, with a loss of the intervening segment.

Adolescent↗
PubMed1983

Importance and accuracy of bone age ratings in a computerized growth evaluation system.

Bone age ratings according to Greulich and Pyle and to Tanner and co-workers (RUS) of 88 children from two pediatric endocrine centers were compared with ratings from 2 trained technicians and independent ratings from a consulting pediatric endocrinologist. Considering the mean of all ratings as 'true bone age', the mean errors of the individual estimations were small (0.15-0.38 years for the Greulich and Pyle method, 0.12-0.27 years for the method according to Tanner and co-workers). Only in 2 of 338 ratings were differences larger than 1 year observed. In these 2 cases, there was marked dissociation between the maturation of carpal and phalangeal bones. It is concluded that estimations of bone maturation can be carried out reliably by properly trained technicians.

Age Determination by Skeleton↗
PubMed1983

Human pituitary growth hormone (hGH) therapy in growth hormone deficiency.

This review has attempted to answer a number of questions regarding human growth hormone therapy in growth hormone deficiency. I believe that the available data support several conclusions which form a suggested current approach to the clinical use of hGH. While these conclusions are derived from data obtained using pituitary growth hormone, it is likely that they are applicable to growth hormone manufactured by recombinant DNA technology, as well. Treatment should be begun as early as the diagnosis can be made in anticipation of a better initial and long-term response in younger patients. Growth hormone should be administered on the basis of body weight in an initial dose of 0.06-0.10 unit/kg 3 times a week. Growth hormone may be administered either intramuscularly or subcutaneously. Therapy should be continuous whenever possible. Treatment should be given until there is no further response which generally will reflect closure of the epiphyses. Associated hormone deficiencies should be adequately treated, and patients should be periodically evaluated for the development of additional deficiencies. Concomitant therapy is not indicated unless deficiencies are clearly demonstrated. Thyroid replacement should be at full dosage, while glucocorticoid replacement should probably not exceed 10-15 mg/m2 x day. Gonadal steroids should be used at the bone age when puberty is expected, and hGH should be continued during pubertal development. There is no general indication for giving anabolic/androgenic steroid in combination with hGH in prepubertal patients. If a waning effect of therapy is observed, the dose of hGH should be incrementally increased, and/or the addition of anabolic/androgenic steroid therapy should be considered. While most reports have focused on the effect of hGH on linear growth, changes in weight, bone age, body proportions, and body composition have also been observed. The effect on bone age is variable, but there is greater enhancement of linear growth than of epiphyseal development in the majority of treated patients. Bone age must be monitored during hGH administration whether or not anabolic/androgenic steroids are used concurrently. Growth hormone administration is remarkably free of side effects. However, neutralizing antibodies to hGH may develop and they should be sought in patients in whom an unexplained decrease in response is observed. Certainly the available incomplete data allow for different conclusions. The expanding supply of hGH should lead to a more systematic evaluation and provide more definite answers to the questions which this review has considered.

Body Composition↗
PubMed1983

Thyroid-stimulating hormone and thyroglobulin levels decrease with chronological age in children and adolescents.

TSH, thyroglobulin (Tg), and the free T4 index were determined and related to chronological age in 24 normal boys (aged 7.6-17.6 yr; mean, 12.6 +/- 1.5 yr) and 20 normal girls (aged 7.8-17.0 yr; mean, 12.2 +/- 1.4 yr). There was no significant (P greater than 0.1) difference in mean serum TSH (3.06 +/- 2.07 vs. 2.97 +/- 2.07 microunits/ml) and Tg levels (25.3 +/- 10.0 vs. 28.0 +/- 14.8 ng/ml) or free T4 index (9.6 +/- 1.6 vs. 9.4 +/- 1.3 micrograms/dl) between boys and girls. Serum TSH levels correlated negatively with chronological age (rs = -0.518; r = -0.562; P less than 0.01), as did serum Tg levels (rs = -0.618; r -0.545; P less than 0.01). On the other hand, a positive correlation was found between serum TSH and Tg levels (rs = 0.455; r = 0.421; P less than 0.01). Free T4 index levels did not correlate with chronological age, TSH levels, or Tg levels. We conclude that both serum TSH and Tg levels vary inversely with chronological age in children and adolescents. Also, a direct correlation was noted between serum TSH and Tg levels. These data as well as those from earlier studies may indicate that pituitary secretion of TSH contributes to serum levels of Tg. The decline in TSH with age may be the result of an increased response of the thyroid gland to TSH or a decline in the MCR of T4 with age.

Adolescent↗
PubMed1982

Histocompatability antigens and immunoglobulin G insulin antibodies in Mexican-American insulin-dependent diabetic patients.

To determine the association of histocompatability antigens (HLA) with insulin-dependent diabetes (IDD) in Mexican-Americans, we determined HLA-A, -B, and -C specificities in 112 unrelated patients and 332 controls, and HLA-DR specificities in 85 patients and 209 controls. We also studied immunoglobulin G (IgG) insulin antibody formation in 56 Mexican-Americans with IDD, and the relationship between antibody formation and HLA-DR antigens. IDD patients have a significant increase in HLA-DR4 compared to the control population (chi 2 = 14.75; corrected P less than 0.0001). HLA-DR2 was not detected in any patient with IDD. A significant association between HLA-Aw30 and HLA-B18 was found in IDD patients (chi 2 = 9.39; P less than 0.05) as compared to controls. IgG insulin antibody formation was significantly increased in HLA-DR3- and -DR4-negative patients compared to that in patients positive for both antigens (P less than 0.05). These findings support previous observations in caucasians and black Americans indicating that HLA-DR specificities are associated with IDD and may play a role in determining its mode of inheritance, and perhaps its pathogenesis, independent of ethnic differences. HLA-DR immune-associated antigens are also of importance in determining IgG insulin antibody formation.

Adolescent↗
PubMed1982

Conversion of progesterone from the adrenal or ovary to deoxycorticosterone in plasma.

The in vivo conversion of progesterone to deoxycorticosterone (DOC) by peripheral 21 hydroxylation was studied in normal men and women. The technique used was to infuse labeled progesterone under steady state conditions and isolate the precursor and product from blood by multiple chromatography steps. DOC was chromatographed as both the steroid and its acetylated derivative. The conversion rate was found to be 1.3 +/- 0.1% in men and women despite differences in the concentration of progesterone during the cycle. Since progesterone was very low in men and women in the follicular phase of the cycle, we conclude that DOC is derived from direct secretion. However, progesterone rises to 1073 +/- 101 ng per dl in the luteal phase and the calculations indicate that DOC increases from 8.3 +/- 0.4 (SE) to 15.6 +/- 1.3 ng per dl. 3/4 of luteal phase DOC is from conversion of progesterone. Acute ACTH increases both progesterone and DOC, and almost all of the DOC in plasma is from direct secretion. However, chronic ACTH excess and adrenal 21 hydroxylase deficiency is associated with a fraction (1/4-1/5) of DOC from progesterone indicating that the peripheral conversion rate is unrelated to the adrenal enzyme defect.

Adrenal Glands↗
PubMed1981

A dose-response curve for human growth hormone.

Although human GH (hGH) has been administered to GH-deficient patients for over 20 yr, there are minimal published data on the relationship of response to dose. We have given hGH on the basis of body weight to 93 prepubertal GH-deficient patients over an initial 12 months of therapy. Their annual growth rate while receiving hGH was 5.58 +/- 2.30 (+/- SD) cm at a dose of 30 mIU/kg, three times a week (tiw; n = 27); 7.31 +/- 1.75 (+/- SD) cm at a dose of 60 mIU/kg, tiw (n = 38); 7.22 +/- 3.12 (+/- SD) cm at a dose of 80 mIU/kg, tiw (n = 12); and 8.94 +/- 1.19 cm (+/- SD) at a dose of 100 mIU/kg, tiw (n = 16). Doubling the dose from 30 to 60 mIU increased the mean rate of growth 1.3 times, and increasing the 30 mIU dose by a factor of 3.3 increased the mean rate of growth 1.6 times. The response (y) as a function of the log-dose (x) is defined by the equation y = -3.12 + 5.80 log x. When the effect of hGH is expressed as the increase in growth rate while receiving therapy, the log-dose relationship is defined by the equation y = -6.09 + 5.67 log x. This dose-response curve provides data which are useful in choosing the best dose of hGH for an individual patient. It also allows a more accurate projection of the costs and benefits of hGH therapy.

Adolescent↗
PubMed1981

Successful outcome of pregnancy in women with hypothyroidism.

Published data on the influence of hypothyroidism on fertility, gestation, and the offspring are controversial. We studied nine hypothyroid women during 11 pregnancies. Mean serum values for thyroxine, triiodothyronine (T3), resin T3 uptake ratio, and thyroid-stimulating hormone were 2.3 microgram/dL, 82 ng/dL, 0.64, and 105 mU/mL, respectively. Four patients had iatrogenic hypothyroidism (three remote thyroidectomy, one remote 131I therapy), two Hashimoto's thyroiditis, and three idiopathic primary hypothyroidism. Seven patients first presented untreated after the 24th week of gestation. Two patients needed cesarean section; seven delivered vaginally. There was one stillborn infant in the only patient with pre-eclampsia. Another infant had Down's syndrome and an ostium primum defect (mother's age, 41 years). The remaining nine infants were normal at birth. All placentas were normal. Follow-up in seven infants up to 2.7 years showed normal thyroid function and somatic development. Infants of hypothyroid mothers may be normal because their hypothalamic-pituitary thyroid axis develops independently from the mother.

Adult↗
PubMed1980

Elevated serum concentrations of triiodothyronine in hypothyroid patients. Values for patients receiving USP thyroid.

Serum concentrations of thyroxine (T4), triiodothyronine (T3), and thyroid-stimulating hormone (TSH) were measured for 14 hypothyroid patients while they were receiving USP desiccated thyroid and after six weeks of therapy with comparable doses of levothyroxine sodium (Na-l-thyroxine). Therapy with USP thyroid caused supraphysiologic elevations in the concentration of T3 in 13 patients. Treatment with levothyroxine resulted in serum concentrations of T3 that were within the range of normal. We believe the preferable therapy for hypothyroidism is levothyroxine.

Adolescent↗
PubMed1980

HLA-DRw antigens in Mexican-American and Black-American diabetic patients.

HLA-A, - B, and -C antigens were studied in 67 Mexican-American and 38 black-American diabetic patients who had the onset of their disease before age 31 yr. Control populations consisted of 322 Mexican-American and 367 black-American subjects for HLA-A, -B, and -C antigens. In addition, HLA-DRw antigens were studied in 60 Mexican-American and 34 black-American diabetic patients. Control populations for HLA-DRw antigens consisted of 189 Mexican-American and 145 black-American subjects. We found that juvenile-onset--diabetic patients of Mexican-American origin who had the onset of their disease before age 19 demonstrated a significant increase in HLA-DRw4. HLA-DRw4 was also significantly increased in black-American patients with juvenile-onset diabetes mellitus. HLA-DRw2 was not detected in any patient with juvenile-onset diabetes in either ethnic group. A significant association was found between HLA-B18 and HLA-DRw3 in Mexican-American juvenile-diabetic patients. These findings, which are comparable to those in similar Caucasian patients, provide additional information to support the hypothesis that HLA-DRw antigens play a major role in determining the susceptibility to juvenile-onset diabetes mellitus.

Adult↗
PubMed1979

Use of growth hormone-gel.

We evaluated the efficacy of a depot preparation of growth hormone (GH) in a 15% gelatin solution (GH-gel) in the treatment of 15 growth hormone-deficient children. The studies were designed to see if prolonging absorption of GH to achieve lower more physiological concentrations of GH in plasma would decrease the frequency of injection, reduce the amount of GH needed for effective therapeutic response, and improve the response to long-term treatment. We found that after a single dose of GH-gel the plasma concentrations of GH were lower than those achieved after the standard aqueous preparation. The preparation was efficacious in promoting growth and our 1st study of 6 patients suggested that GH-gel given twice a week had a growth response equal to that of the three-times a week aqueous schedule. However both schedules resulted in the frequently observed decreased growth rate during the second treatment year. Our 2nd study, attempting to ameliorate this waning effect by using the GH-gel preparation twice a week in a weight-adjusted dose during the entire second year did not resolve the problem. Thus, GH in depot gel results in more physiological plasma concentrations of GH and may be beneficial in reducing the quantity of hormone needed and the injection frequency but it does not offer a solution to the waning response to the long-term administration of GH.

Adolescent↗
PubMed1979

Testosterone and estradiol concentrations in paired maternal and cord sera and their correlation with the concentration of chorionic gonadotropin.

Testosterone (T) and estradiol (E2) concentrations were determined and correlated with beta human chorionic gonadotropin (beta-HCG) concentrations in 43 paired maternal and cord sera (22 female and 21 male infants). Mean (+/- SD) maternal E2 concentrations were significantly (P less than .005) higher when the sex of the fetus was male than when the sex of the fetus was female (20.6 +/- 3.9 vs 13.5 +/- 3.2 ng/ml). Maternal T concentrations were not significantly different when related to the sex of the fetus (males, 114.8 +/- 60.7 vs females, 113.8 +/- 54.5 ng/100 ml, P greater than .1). Regression analysis did not show a significant correlation between maternal T or E2 concentrations and maternal beta-HCG concentrations. Mean cord serum T and E2 concentrations of male infants were significantly greater than that of female infants (T, 38.8 +/- 8.5 vs 25.8 +/- 7.1 ng/100 ml, P less than .005; E2, 9.1 +/- 3.3 vs 6.6 +/- 2.0 ng/ml, P less than .005). Regression analysis showed a significant (P less than .005) correlation between cord beta-HCG concentrations and E2 concentrations for male infants (r = .7) and female infants (r = .6). A significant correlation between cord beta-HCG concentrations and T concentrations was found for male infants (r = .5; P less than .01) but not for female infants (r = .3; P greater than .05). There was no correlation between maternal and infant E2 concentrations (males, r = .3, P greater than .05; females, r = .3, P greater than .2) or T concentrations (males, r = .02, P greater than 0.4; females, r = .06, P greater than .3). These data (1) confirm the sex difference in cord serum T and E2 concentrations, (2) indicate that the lower beta-HCG concentrations in mothers of male infants are associated with E2 concentrations which are greater than those in mothers of female infants, and (3) are consistent with an influence of beta-HCG on fetal T and E2 secretion.

Chorionic Gonadotropin↗
PubMed1978

Collaborative study of the effects of human growth hormone in growth hormone deficiency. V. Treatment with growth hormone administered once a week.

Twenty-two GH-deficient patients received 6 IU GH weekly for 6 months. They increased their growth rate from 1.0 +/- 0.2 to 2.9 +/- 0.3 (SE) cm/6 months (P less than 0.01). However, their rate of growth was significantly less (P less than 0.05) than the rate [4.1 +/- 0.5 (SE) cm/6 months] observed earlier when they had received 2 IU GH three times a week. Seven patients received weekly GH for 18 months and they also grew significantly less (P less than 0.01) than when they had received GH divided over the week. These results suggest that once a week GH does not provide the most effective therapy for GH deficiency.

Drug Administration Schedule↗