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Biomedical subjects

R K Danish

Publications and source records attributed to R K Danish.

9 recordsLinked to original sources

Urea synthesis, nitrogen balance, and glucose turnover in growth-hormone-deficient children before and after growth hormone administration.

We measured the effect of human growth hormone (hGH) on urea synthesis, nitrogen retention, and glucose turnover in ten euthyroid growth hormone (GH)-deficient children before and after seven daily injections of 0.1 U/kg hGH. The patients were fed a weight-maintaining diet with 9% of energy derived from protein. Following an overnight fast, urea synthesis and glucose turnover were determined using a primed constant infusion of [15N2] urea and a constant infusion of [6,6-2H2] glucose. Human growth hormone produced a decrease in urea nitrogen synthesis from 6.8 +/- 0.5 to 4.2 +/- 0.4 mg/kg.h; (P less than .01), while plasma urea nitrogen decreased from 13.1 +/- 0.8 to 7.4 +/- 0.8 mg/dL; (P less than .01). The decrease in urea synthesis was reflected in a corresponding decrease in urine urea nitrogen excretion (-2.8 mg/kg.h). There was a significant correlation between plasma urea nitrogen and urea synthesis rate both before (r = 0.85, P less than .01) and after (r = 0.79, P less than .01) hGH treatment. In response to hGH, there was a rise in both plasma glucose (81.4 +/- 2.2 v 89.8 +/- 2.3 mg/dL; P less than .05) and insulin (5.7 +/- 0.8 v 13.1 +/- 3.0 microU/mL; P less than .05), however, glucose turnover remained unchanged (4.7 +/- 0.3 v 4.6 +/- 0.6 mg/kg.min). After seven days of growth hormone treatment, the patients were placed on 0.1 U/kg of hGH three times a week for 6 months, and their growth rate was calculated.(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Glucose↗

Rapid simultaneous assay of serum estrone, estradiol, and estriol in pregnant women using methyl ether acetate derivatives by capillary gas chromatography and electron-impact mass spectrometry.

A simple gas chromatographic--mass spectrometric method capable of measuring estrone, estradiol, and estriol simultaneously with a sensitivity close to that of radioimmunoassay has been developed. The estrogens in serum were extracted with diethyl ether, and internal standards (3-O-C2H3-estrone, 3-O-C2H3-estradiol, and 3-O-C2H3-estriol) were added, followed by converting to methyl ether compounds with an extractive alkylation procedure. The methyl ethers were then acetylated. Analyses were performed using a SP-2250 capillary column gas chromatograph coupled with an electron-impact mass spectrometer. The estrogen methyl ether acetate derivatives were more stable chemically and gave less fragmentation upon electron impact than the conventional trimethylsilyl derivatives. The use of selected ion monitoring of molecular ions and that of the corresponding internal standards (M + 3) provides a sensitivity down to 10 pg for estrone and estradiol and to 200 pg for estriol. The time required for the preparation of multiple samples is within 4 hours.

Dehydroepiandrosterone↗

Treatment of Cushing's disease with bilateral adrenalectomy and autotransplantation.

2 Cases of childhood Cushing's disease have been treated with bilateral adrenalectomy and autotransplantation of adrenal tissue. Transplantation was unsuccessful in 1 case. In the other patient, replacement therapy was discontinued without any symptoms of hypo- or hyperadrenocorticism. Her urinary 17-hydroxycorticosteroids, free cortisol and aldosterone remain in the low normal range indicating functional adrenal tissue, probably a result of the transplant.

Adolescent↗

Micropenis. II. Hypogonadotropic hypogonadism.

This paper documents the case histories of 14 patients with hypogonadotropic hypogonadism and micropenis (penile length below 2.5 SD from the mean for the patient's chronlogical age, or for age corresponding to stage of sexual development). Nine of the patients were raised as males. Five of them received androgen for the purpose of stimulating penile growth: two realized normal adult penile length (-0.2 and -2.1 SD from normal mean length) whereas the other three had penile lengths significantly below the mean (-3.6, -4.6 and -5.2 SD from normal mean length). These data suggest that among hypogonadotropic patients with micropenis, those with prepubertal penile lengths between 2.5 and 3 SD below the mean may develop an adult penis of a length within the normal range. However, for those presenting with a shorter phallus, the expectation is that penile length will remain greater than 2 SD below the mean.

Child, Preschool↗

Micropenis. III. Primary hypogonadism, partial androgen insensitivity syndrome, and idiopathic disorders.

This paper, the third in a series, presents data from 28 patients with micropenis categorized as primary hypogonadism (11 subjects), partial androgen insensitivity (1 subject), idiopathic (6 subjects), and etiology undetermined (10 subjects). Among the 11 patients with primary hypogonadism, 8 presented with various degrees of gonadal dysgenesis, 1 was a true hermaphrodite and 2 had the Robinow syndrome. Nine of the 28 patients were raised as females: 5 with primary hypogonadism and 4 with undetermined etiology. Eleven of the 19 patients raised as males received androgen stimulation during prepubertal years and responded with penile growth. However, this growth response was temporary and did not appear to be predictive of eventual adult size of the penis. Generally, the prestimulation size of this group of patients is more predictive of adult penile size. Only 7 of the patients raised as males have attained adult somatic growth. Three out of the three with primary hypogonadism, the subject with partial androgen insensitivity, and one of three with idiopathic micropenis have below-normal adult penile length. These limited data suggest that growth potential of the micropenis may be greater among the patients with an idiopathic state than among those with primary hypogonadism and partial androgen insensitivity.

Adolescent↗

Studies of androgen production and binding in 13 male pseudohermaphrodites and 13 males with micropenis.

We studied androgen production by morphologic and biochemical criteria plus androgen binding by skin fibroblasts in 13 male pseudohermaphrodites and 13 males with micropenis, none of whom had evidence of androgen insensitivity. Seventeen of the 26 patients had evidence of deficient androgen production, suggesting this as the cause of their incomplete virilization in utero. Fibroblasts from all 26 patients demonstrated normal androgen binding and affinity of the steroid for the receptor. Although these results exclude a deficiency of androgen binding in these individuals, other end organ defects are possible. Guidelines for diagnosis and management of such patients are presented.

Adolescent↗

Diencephalic syndrome revisited.

This report details the histories of five patients with clinical diencephalic syndrome who collectively demonstrate the variability found in the syndrome with respect to: (1) clinical course, (2) site of the tumor, and (3) ease of obtaining radiologic confirmation of the presence of a tumor. A review of an additional 67 patients indicates that the observations are not unique. The anatomic variability combined with the fact that the course of those who are treated is infinitely better than those left untreated adds urgency to the establishment of precise anatomic diagnosis. These considerations led to a critical review of the histories of the 72 patients. From this it can be stated that anteriorly and posteriorly placed tumors do exhibit subtle but significant differences in their clinical course, and roentgenograms of the optic foramina and analysis of the CSF cell and protein content appear warranted early in the investigation of emaciation from unknown cause. Further, an evaluation is made of the role of various radiologic techniques and of endocrine studies in establishing the diagnosis. Similarly, the relative merits of radiotherapy and/or surgery in the treatment of the disease are defined. Finally, the adequacy of the term diencephalic syndrome is discussed.

Astrocytoma↗