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Biomedical subjects

A W Root

Publications and source records attributed to A W Root.

At least 73 records · Page 4Linked to original sources
PubMed1984

Evolution of the hyperandrogenism-polycystic ovary syndrome from isosexual precocious puberty: report of two cases.

Two girls who presented initially with isosexual precocity later developed the hyperandrogenism-polycystic ovary syndrome. We propose that the pathogenesis of the hyperandrogenism-polycystic ovary syndrome in these two children is related to an abnormal release of hypothalamic gonadotropin-releasing hormone, a subsequent sustained secretion of luteinizing hormone, and a consequent excessive secretion of ovarian androgens that results in hyperandrogenism. The clinical findings in these patients suggest that the dysfunction of the hypothalamic-pituitary regulation of luteinizing hormone secretion that leads to the hyperandrogenism-polycystic ovary syndrome may occur prior to puberty.

Adolescent↗
PubMed1984

The effect of magnesium depletion on thyroid function in rats.

The effects of dietary magnesium (Mg) depletion on thyroid function were studied in young male rats. The rats were fed a semipurified diet containing either 12 ppm Mg (deficient rats) or 662 ppm Mg (control rats) for 14 to 28 days. Results showed that the Mg-deficient rats had decreased body weight gain, lowered concentrations of plasma thyroxine (T4) and Mg, but increased weight of the thyroid gland when expressed in proportion to the body weight (milligrams/100 g). There was no difference in the accumulation (uptake) of 131I, 24 hours after Na131I injection, between the Mg-deficient and Mg-supplemented rats. The protein-bound 131I (PB131I) level and the ratio of PB131I to total 131I in plasma was significantly reduced in Mg-deficient rats. Serum thyroid-stimulating hormone (TSH) levels after thyrotropin-releasing hormone injection (TRH, 50 ng/100 g body weight) increased fivefold at 30 minutes, but declined to near the basal level at 2 hours in both groups. No consistent difference in TSH response was observed between the two treatments. Serum T4 response to TRH challenge was significantly reduced in Mg-deficient as compared to Mg-adequate rats at all time intervals. The reduction of T4 level could be due to an impaired T4 synthesis or release in Mg-deficient rats.

Animals↗
PubMed1983

The Kenny-Caffey syndrome: growth retardation and hypocalcemia in a young boy.

A 2-year-old black boy with the Kenny-Caffey syndrome was first evaluated because of growth retardation and hypocalcemia. Hypothalamic-pituitary function was normal. Basal serum somatomedin C levels were normal for age, but did not increase during short-term administration of human growth hormone. Serum immunoreactive parathyroid hormone levels remained inappropriately low during spontaneous and induced hypocalcemia, indicating that hypocalcemia was the consequence of hypoparathyroidism. The manifestations of 15 patients with this syndrome are tabulated.

Child, Preschool↗
PubMed1983

Anorexia nervosa presenting as growth retardation in adolescents.

Two adolescent males and one adolescent female were referred for evaluation of short stature. Extensive laboratory studies, often performed over several years, were unremarkable. After several years of observation, the diagnosis of anorexia nervosa was established. We emphasize the necessity to consider anorexia nervosa in the differential diagnosis of growth retardation in young adolescents.

Adolescent↗
PubMed1983

Human growth hormone blunts Na2EDTA-induced hypocalcaemia in hyposomatotrophic children.

An infusion of disodium ethylenediamine tetraacetate (Na2EDTA) (0.13 mmol/kg for 2 h) was administered to 10 hyposomatotrophic children prior to and after 6 and 12 months of treatment with human growth hormone (hGH). Total and ionized calcium and immunoreactive parathyroid hormone (iPTH) concentrations were determined. Mean basal total and ionized calcium concentrations did not change during the year of treatment with hGH. The nadir concentrations of total and ionized calcium increased progressively during hGH administration and after 12 months were significantly increased over pre-treatment values (total calcium: pretreatment 1.85 +/- 0.32 (SD) mmol/l, +12 months 2.10 +/- 0.15, P less than 0.01; ionized calcium: pre-treatment 0.55 +/- 0.31 mmol/l, +12 months 0.78 +/- 0.14, P less than 0.05). The mean basal concentration of iPTH increased slightly after 12 months of hGH administration (pre-treatment 72 +/- 18 pg/ml, +12 months 106 +/- 71, P less than 0.05), but Na2EDTA-evoked secretion of iPTH was not significant altered by hGH.

Calcium↗
PubMed1982

Encephalopathy and fatal myopathy in two siblings. Their association with partial deficiency of muscle carnitine.

Two brothers had intermittent episodes of muscle weakness, lethargy, hyperammonemia, rhabdomyolysis, and elevated activities of creatine phosphokinase (CPK), lactic dehydrogenase, and SGOT in serum associated with low muscle carnitine but normal serum carnitine concentrations. These siblings represent a "mixed" form of carnitine deficiency with the elements of both systemic and myopathic carnitine deficiency. The older sibling died suddenly after a 24-hour fast. The younger boy has received carnitine for three years. During this period, serum CPK activity has remained elevated and increased further during illnesses, but no clinical symptoms of encephalopathy or myopathy have appeared.

Carnitine↗
PubMed1982

Bilateral branchial cleft sinuses associated with intrauterine and postnatal growth retardation, premature aging, and unusual facial appearance: a new syndrome with dominant transmission.

A mother and son with bilateral branchial sinuses, intrauterine and postnatal growth retardation, unusual facial appearance, and premature aging in the mother are reported. No other members of the family are similarly affected. No hormonal or systemic cause of growth retardation was identified. Chromosomal studies with G-banding were normal. It is suggested that this syndrome is a dominant trait, the mother being the initial mutant.

Abnormalities, Multiple↗
PubMed1982

Chondrodystrophic myotonia (Schwartz-Jampel syndrome): report of a new case and follow-up of patients initially reported in 1969.

We report on a 9-year-old boy with chondrodystrophic myotonia (Schwartz-Jampel syndrome) and the progress of a brother and sister with this syndrome first described in 1969. This is an autosomal recessive trait characterized by mask-like face, narrow palpebral fissures (due to blepharophimosis, blepharospasm, and abnormal orbital configuration), microstomia, micrognathia, myotonia, muscular hypertrophy, osteochondrodysplasia, and growth retardation. Expressivity varies and in some sibships females are less severely affected than their brothers. The sexual development of the sibs with chondrodystrophic myotonia, who are now in the mid to late second decade, has been normal. Linear growth rate accelerated during puberty but the adult height of the male is less than normal. Administration of human growth hormone had no consistent effect on the growth pattern of this boy.

Abnormalities, Multiple↗
PubMed1982

Effect of human growth hormone on gastrin secretion in children with hyposomatotropism.

The effect of human growth hormone (hGH) on gastrin secretion was evaluated in 13 hyposomatotropic children and two subjects with bioinactive growth hormone (GH). Serum concentrations of gastrin were quantitated by radioimmunoassay after ingestion of a standard meal and following intravenous infusion of arginine, prior to and after 6 and 12 months of hGH administration. Although the meal provoked a significant increment in gastrin values at each point, hGH did not alter basal concentrations or or meal-evoked secretion of gastrin in these subjects. Arginine had no effect on gastrin levels. Serum immunoreactive insulin concentrations increased in response to eating and arginine, but hGH did not alter the insulin secretory response to either stimulus. It is concluded that hGH did not affect the secretion of gastrin or insulin under the conditions of this study.

Adolescent↗
PubMed1982

The reproductive endocrine system in cystic fibrosis: 2. Changes in gonadotrophins and sex steroids following LHRH.

Hypothalamic-pituitary-gonadal function was assessed in forty-seven patients with cystic fibrosis (CF) by the 3-hr infusion of 100 microgram of synthetic gonadotrophin-releasing factor. LHRH and the results compared with a group of children being evaluated for short stature and delayed puberty ('controls'). Levels of gonadotrophins and sex steroids were measured prior to and during the infusion. In prepubertal boys, LH and FSH release evoked by LHRH was significantly greater (P less than 0.001) in 'control' subjects than in CF patients. In pubertal boys, LH and FSH release was also greater in 'controls' than in CF, though to a lesser degree (P less than 0.05). In pubertal girls, responses to LHRH were comparable for LH and slightly greater (P less than 0.05) in 'controls' for FSH. In the earliest pubertal groups of both sexes (male-Tanner genitalia stage 2; females-Tanner breast stage 2), LH secretion was similar in patients with CF and 'control' subjects. Significant increments of testosterone and oestradiol in pubertal CF patients do not occur until 6 h after the LHRH infusion begins, in contrast to a rise at 3 h in 'control' subjects. These data suggest that prepubertal boys with CF, who are the most impaired in height, weight and skeletal maturation, also have measurable abnormalities of LHRH-releasable gonadotrophin secretion. Despite continued impaired weight growth, pubertal patients do attain essentially normal gonadotrophin secretory responses to LHRH administration and are similar to subjects with constitutional delayed adolescent development in reproductive endocrine physiology.

Adolescent↗
PubMed1981

The reproductive endocrine system in cystic fibrosis. I. Basal gonadotropin and sex steroid levels.

Serum gonadotropin and sex steroid levels were measured in 106 patients with cystic fibrosis (CF), 46 males and 60 females, aged 8 to 24 years. The finding of delayed pubertal increments of serum gonadotropin and sex steroid levels in CF patients suggests late maturation of the reproductive endocrine system. Although pubertal changes in reproductive endocrine hormones in patients with CF appear to be temporally delayed, generally appropriate levels of these hormones are finally attained in most patients by the late teenage years. Delayed maturation of the reproductive endocrine system probably is secondary to hypothalamic-pituitary dysfunction, the result of chronic inanition.

Adolescent↗
PubMed1981

Plasma free insulin concentrations: keystone to effective management of diabetes mellitus in children.

In patients with insulin-dependent diabetes, the therapeutic effect of prescribed insulin is routinely judged by the response of the blood glucose concentration. Measurement of both free and bound circulating insulin indicates that neither total nor free insulin concentrations correlate with the prescribed insulin dose. Errors of insulin administration may account for this discrepancy. Free insulin rather than total insulin values determine the plasma glucose concentration and the degree of long-term glycemic control as reflected by levels of hemoglobin A1C. Diabetic ketosis and ketoacidosis are strongly associated with absence of free-circulating insulin. Knowledge of plasma-free insulin values is useful in the management of insulin-dependent diabetes mellitus.

Blood Glucose↗
PubMed1981

Childhood thyromegaly: recent developments.

Evaluation of a child with goiter includes historical review, physical examination, and measurement of serum concentrations of PBI, T4 and T3RU, TSH, and titers of antithyroglobulin and antithyroid microsomal antibodies. If there are no indications for more intensive evaluation such as history of cervical irradiation, a palpable abnormality of the thyroid gland or unusual laboratory findings (e.g., a significant PBI-thyroxine iodine discrepancy in the absence of a positive antithyroid antibody titer), a trial of TSH-suppressive therapy with thyroxine is undertake, even if the cause of thyromegaly has not been identified. If thyroid size diminishes in the ensuing six to 12 months, treatment is maintained for approximately two years and then discontinued. If the goiter recurs, or if there is impaired thyroid function, treatment is resumed. Periodically, antithyroid antibody titers and indices of thyroid function are determined. If the goiter does not diminish after a reasonable trial of suppressive therapy with adequate amounts of thyroxine (i.e., those quantities which will inhibit TRH-induced secretion of TSH), subtotal thyroidectomy is recommended to be certain that an underlying neoplasm has not been overlooked. A biopsy of the thyroid is not performed routinely in such children prior to operative therapy. Almost invariably, examination of the surgical specimen reveals CLT. Postoperatively, suppressive doses of thyroxine are maintained indefinitely. Inasmuch as thyroxine suppression of TSH secretion is essential in the management of patients with thyroid neoplasms, a limited medical trial, as described, does not place the patient at undue risk.

Adolescent↗