The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence

Wilkins, Lawson

doi:10.1097/00007611-195105000-00033

Cited by 96 publications

(132 citation statements)

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“…Testicular biopsy of these Poststreptococcal meningitis Cyst of the cavum velum interpositum skeletal maturation was consistent with chronologic age, vaginal cytology revealed no evidence of estrogen effect, and there was no progression of sexual development during prolonged periods of observation. Levels of LH were below the limits of sensitivity of the assay in all of 10 children with premature pubarche [49].…”

Section: Plasma Lh Concentrationsmentioning

confidence: 78%

“…Normal males (N = 61), 1 month-19 years of age, without evidence of disease of the central nervous system or of the pituitary-testicular axis; males with constitutional delay in growth and development [49] (N = 44), 6-16 years of age; normal females (N = 48), 2 months-15 years of age; all were premenarchal and without evidence of disease of the central nervous system or of the pituitary-ovarian axis; patients with hypopituitarism and documented lack of growth hormone response to hypoglycemia [35] (N = 29), 6-20 years of age; phenotypic females with gonadal dysgenesis (N = 18), 3 weeks-18 years of age; anorchic males (N = 6), 11-22 years of age; male pseudohermaphrodites (N = 5), 3 months-15 years of age; and subjects with precocious sexual development (N = 32), 1-11 years of age.…”

Section: Subjectsmentioning

confidence: 99%

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Concentrations of Plasma Luteinizing Hormone in Infants, Children, and Adolescents with Normal and Abnormal Gonadal Function

et al. 1970

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ExtractThis communication reports the development of a sensitive, reproducible, and specific radioimmunoassay for luteinizing hormone (LH) in plasma and the application of this method to a cross-sectional survey of plasma LH concentratior~s in 243 infants, children, and adolescents with normal and abnormal growth and sexual development. One nanogram of Hartree purified human pituitary LH standard was immunologically equivalent to 13.7 mIU of the Second International Reference Preparation of Human Menopausal Gonadotropin. The lower limit of sensitivity of the assay varied between 0.025 and 0.05 ng/ml. Specimens of plasma containing t0.25 ng/ml (t3.7 mIU/ml) have been considered indistinguishable from 'zero'.Plasma LH concentrations increased after 9 years of age in normal boys and after 6 years of age in normal girls. In males with constitutional delay in growth and development, LH levels were generally lower than in normal boys of comparable ages (figs. 3 and 4). The plasma LH concentration also increased with advancing sexual maturation ( fig. 5).Levels of LH were t0.25 nglml in 15 of 18 males (6-20 years old) and in 10 of 11 females (6-19 years old) with hypopituitarism and documented lack of human growth hormone response to hypoglycemia. In three such patients, plasma LH concentrations correlated with their degrees of sexual maturation, but in a 16-year-old clinically prepubertal male, patient SA, with hypopituitarism secondary to a craniopharyngioma, LH levels higher than normal male values were noted.Plasma LH concentrations were 1.2 and 1.45 ng/ml in 2 of 3 girls less than 4 years old, with gonadal dysgenesis, and ranged between 0.33 and 3.6 ng/ml in 10 of 15 older patients with this diagnosis (table 111). The plasma LH concentration was 1.7 ng/ml in a 3-month-old male ~seudohermaphrodite. Plasma LH concentrations ranged between 0.38 and 0.88 ng/ml in five of seven children with true sexual precocity. In 13 of 15 girls with precocious thelarche, and in all of 10 patients with premature pubarche, LH levels were t0.25 ng/ml. The intravenous infusion of conjugated equine estrogens was followed by a prompt decline in plasma LH levels in patients with gonadal dysgenesis and Klinefelter's syndrome, but failed to alter significantly LH values in patient SA ( fig. 6 A, B, C). Administration of clomiphene citrate and cisclomiphene citrate increased plasma LH concentrations in normal males, but failed to do so in patients with hypopituitarism, including patient SA ( fig. 7 A, B). The intramuscular administration of medroxyprogesterone acetate was associated with suppression of plasma LH levels in two patients with true sexual precocity ( fig. 8). ROOT, MOSHANG, BONGIOVANNI and EBERLEIN SpeculationThe hypothalamic-pituitary-gonadal axis is active in the prepubertal human subject, and is responsive to abnormal function in one segment of the system.

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Section: Plasma Lh Concentrationsmentioning

confidence: 78%

Section: Subjectsmentioning

confidence: 99%

Concentrations of Plasma Luteinizing Hormone in Infants, Children, and Adolescents with Normal and Abnormal Gonadal Function

et al. 1970

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“…1 Today, short stature and macrocephaly are well-recognized traits of this disorder. 2 -9 Another form of growth anomaly associated with NF1 was reported by van Asperen et al, who observed the combination of NF1 with overgrowth and features resembling Weaver syndrome in a mother and her son.…”

Section: Introductionmentioning

confidence: 99%

Childhood overgrowth in patients with common NF1 microdeletions

et al. 2005

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While growth retardation and short stature are well-known features of patients with classical neurofibromatosis type 1 (NF1), we found advanced height growth and accelerated carpal bone age in patients with an NF1 microdeletion. Our analysis is based on growth data of 21 patients with common 1.4/1.2 Mb microdeletions, including three patients with a Weaver-like appearance. Overgrowth was most evident in preschool children (2-6 years, n ¼ 10, P ¼ 0.02). We conclude that childhood overgrowth is part of the phenotypic spectrum in patients with the common 1.4/1.2 Mb NF1 microdeletions and assume that the chromosomal region comprised by the microdeletions contains a gene whose haploinsufficiency causes overgrowth.

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“…These anaemias were all described separately, but Beutler (1957) showed that in the people who were sensitive to primaquin there was diminution of glutathione in the blood, due to deficiency of glucose-6-phosphate dehydrogenase. T h e situation was similar in favism and in moth-ball anaemia (Zinkham & Childs, 1957;Childs, Zinkham, Browne, Kimbro & Torbert, 1958). As a result of their work and that of others (reviewed by Beutler, 1960) we now know that there is a type of acute haemolytic anaemia which is due to abnormality of the red blood corpuscles caused by deficiency of a particular enzyme.…”

Section: The Variety Of Inborn Errorsmentioning

confidence: 86%

“…It was found in January I950 by Lawson Wilkins that giving the patient I 9 VOl. 21 cortisone caused a reduction of androgen production (Wilkins, 1957). Presumably the high cortisone level in the blood causes the pituitary gland to cut down production of ACTH which is no longer needed for one purpose, but in so doing the gland also removes the stimulus to production of androgen.…”

Section: Vol 21mentioning

confidence: 99%