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Biomedical subjects

S L Drop

Publications and source records attributed to S L Drop.

At least 127 records · Page 7Linked to original sources
PubMed1986

[Pseudo- and central precocious puberty due to adrenogenital syndrome].

We describe three patients in whom at the age of 4-7 yrs the diagnosis adrenogenital syndrome (21-hydroxylase deficiency) was made. There was no salt-loss. Two boys presented with precocious pubic hair development and increased growth velocity and bone maturation. The third patient was a severely virilized girl raised as a boy until she was 4 yrs old. Following an extensive psychiatric evaluation, it was decided to raise her as a girl and feminoplasty was performed. Clinical and biochemical evidence of central precocious puberty was present in one boy at the time of diagnosis at age 7 and developed under hydrocortisone substitution therapy in the two other patients. The precocious puberty was treated in two patients with the anti-androgen cyproterone acetate. In one boy suppression of pituitary gonadotropin secretion was obtained by LH-RH analogue (Buserelin) treatment.

Adrenal Hyperplasia, Congenital↗
PubMed1986

[Hypercalcemia in childhood. A boy with asymptomatic hypocalciuric hypercalcemia].

The diagnostic work-up of a patient with (familial) hypocalciuric hypercalcemia (FHH) is discussed. The patient showed no clinical signs of hypercalcemia. There were no indications of vitamin D intoxication. In the first degree family members no hypercalcemia was found. Physical examination was normal. In contrast to hyperparathyroidism, FHH is usually symptomless. Furthermore, FHH is characterized by a normal chloride/phosphate ratio, a normal, but relatively high, serum parathyroid hormone level, a relatively low urinary calcium excretion, a calcium-creatinine clearance ratio less than 0.01 and a normal cyclic AMP excretion.

Calcium↗
PubMed1986

[Central precocious puberty].

Precocious puberty is a stressful event for patient and environment. The diagnostic evaluation will be discussed. LHRH analogs will give a new approach in the treatment of central precocious puberty.

Age Determination by Skeleton↗
PubMed1985

Somatomedin-C/insulin-like growth factor I-binding proteins in human amniotic fluid and in fetal and postnatal blood: evidence of immunological homology.

By using the chemical cross-linking agent dis-succinimidyl suberate and [125I]somatomedin-C/insulin-like growth factor I (Sm-C/IGF-I) to affinity label Sm-binding proteins, we identified a 30,000- to 40,000-dalton (30-40K) [125I] Sm-C-binding protein complex in midterm amniotic fluid and cord blood. An antibody raised against a Sm-binding protein purified from midterm amniotic fluid recognized this labeled complex not only in amniotic fluid but also in fetal serum and term cord and several postnatal human plasmas, indicating that the 30-40K Sm-binding proteins in each are similar or identical. The binding proteins in amniotic fluid appear to possess binding sites specific for Sm-C, because under the conditions employed, unlabeled Sm-C was at least 10-fold more potent than IGF-II or multiplication-stimulating activity in competing with [125I]Sm-C for binding. Although [125I]GF-II could be cross-linked to similarly sized proteins, unlabeled Sm-C also competed for this binding better than either unlabeled IGF-II or MSA (at least 5-fold greater potency). These findings suggest that this amniotic fluid Sm-binding protein is primarily a carrier of Sm-C, but does not exclude the possibility that a binding site or a distinct binding protein exists which is specific for IGF-II but not amenable to cross-linking by the procedure used. Because unlabeled Sm-C was less potent in inhibiting the binding of [125I]Sm-C to amniotic fluid than to cord plasma proteins, the amniotic fluid binding protein is either more abundant or less avidly binds [125I]Sm-C than the cord plasma binding protein.

Affinity Labels↗
PubMed1985

[Hernia inguinalis uteri in the male].

Persistent Müllerian duct syndrome (also known as uterine inguinal hernia) is described and two cases are reported. In this rare type of male pseudohermaphroditism there is no disturbance of either primary or secondary external masculinization. Consequently, this anomaly is always diagnosed incidentally, generally at operation for cryptorchidism or inguinal hernia. In addition to bilateral normal testes, these patients possess a uterus with two uterine tubes. The persistence of the internal female genitalia is caused by the insufficiency of the MIF hormone (Müllerian Inhibiting Factor). Early orchiopexy is recommended. Removal of the female internal genitalia is seldom required.

Anti-Mullerian Hormone↗
PubMed1984

Linkage and segregation analysis of HLA and congenital hypoaldosteronism due to corticosterone methyl-oxydase deficiency type I and type II.

To determine the genetic relations between HLA and deficiencies of steroidogenic enzymes other than 21-hydroxylase, we investigated HLA and congenital hypoaldosteronism in two families with corticosterone methyl-oxidase type 2 (CMO2) and one family with type 1 (CMO1) deficiency, respectively. Apart from a first documentation of HLA in CMO1 deficiency, our results, combined with those reported previously, excluded close linkage of HLA and CMO2 deficiency. However, loose linkage could not be encluded and the segregation of HLA haplotypes in sibships with CMO2 deficiency deviated significantly from random segregation. We suggest that HLA and CMO2 deficiency may not be independent.

Aldosterone↗
PubMed1984

Isolation of a somatomedin-binding protein from preterm amniotic fluid. Development of a radioimmunoassay.

Amniotic fluid binding protein (AFBP) is a heat and acid stable somatomedin (Sm)-binding protein with a mol wt of 35-40,000 and an isoelectric point of +/- 4.7. It is reactive in RRAs for Sm and inhibits Sm activity in Sm bioassays. AFBP was purified from midgestational human amniotic fluid (AF) using acid-ethanol extraction, Sephadex G-150 chromatography, high speed gel filtration chromatography, and disc gel-electrophoresis. Specific binding activity (microgram equivalents per mg protein) was quantitated by incubation with 125I-insulin-like growth factor II and dextran-coated charcoal separation. Protein recovery was less than 1%. AFBP antiserum was produced by immunizing rabbits with purified AFBP. The antiserum was cleared of human serum albumin antibodies by affinity chromatography. Immunoelectrophoresis of 20x concentrated preterm AF and fetal serum resulted in one precipitin line. AFBP was labeled by the chloramine-T method. The AFBP antiserum specifically bound +/- 35% of added 125I-AFBP at a final dilution of 1:5000. A double antibody RIA was developed. The AFBP level measured by RIA in midgestation AF (n = 30) was 148 +/- 18 (SEM) and in term AF (n = 12) 72 +/- 36 mu geq/ml. Insulin-like growth factor I/Sm-C values (determined by RIA) in the same samples were uniformly very low (less than 0.10 U/ml). When serum was chromatographed on Sephadex G-200 at pH 2.2, AFBP-RIA activity eluted in one peak corresponding to a mol wt of 35-40,000. Highest activity was found in fetal serum (gestational age +/- 20 weeks) and lowest in serum from adults. The development of the AFBP-RIA may contribute to further elucidation of the physiological importance of Sm and the Sm-binding proteins in pre- and postnatal growth.

Adult↗
PubMed1984

Immunoassay of a somatomedin-binding protein from human amniotic fluid: levels in fetal, neonatal, and adult sera.

We developed a specific RIA for a somatomedin (Sm)-binding protein, with an approximate mol wt of 35-40,000, purified from midgestational human amniotic fluid (AF) and termed AF-binding protein (AFBP). After Sephadex G-200 chromatography AFBP-RIA activity was found in fractions of fetal and cord serum only at a kav corresponding to a mol wt of +/- 40,000. Whole serum or plasma dilutions in a range of 1:20 to 1:600 showed parallelism with the standard curve. Sm-binding activity in fetal serum was found solely at a mol wt of 30-40,000; in cord serum additionally at a mol wt range of 150-200,000. AFBP serum or plasma concentrations determined by RIA were influenced by several factors: AFBP values in eight adults were highest in the morning (mean +/- SEM, 0.7 +/- 0.1 mu geq/ml) and lowest at night (0.3 +/- 0.1). AFBP values in pre- and postnatal serum showed a gradual decline with increasing age: fetal serum: mean +/- SEM, 36.7 +/- 15.7 (n = 17); adults: 0.6 +/- 0.07 mu geq/ml (n = 19). In serum from GH-deficient children AFBP concentrations were significantly higher than in an age-matched control group (P less than 0.05). Elevated values also were found in serum of children with end-stage renal failure and in serum of pregnant women at 36 weeks of gestation. AFBP was found in urine of preterm infants (mean +/- SEM, 0.04 +/- 0.005 mu geq/ml; n = 31). AFBP immunoreactivity was demonstrable in serum of three orangoutan mothers and their three children and in medium of a hepatoma cell line (PLC/PRF/5) but not in bovine, porcine, rabbit, or rat serum or in medium of cell cultures of (pre-)term placentae. We conclude that AFBP immunoreactivity is present in pre- and postnatal serum and has striking similarity to an unsaturated serum Sm-binding protein with a mol wt of +/- 40,000.

Adolescent↗
PubMed1983

Pituitary-dependent Cushing disease and primary adrenocortical nodular dysplasia in childhood. Presentation of 4 cases.

Four patients with Cushing syndrome were followed for 1.5-12 years. The main clinical symptoms were marked growth retardation and truncal obesity. Two patients had pituitary-dependent Cushing disease, while the other two had primary adrenocortical nodular dysplasia. The treatment of choice of pituitary-dependent Cushing disease is transsphenoidal resection of the microadenoma while in primary adrenocortical nodular dysplasia bilateral adrenalectomy with subsequent steroid replacement is recommended. In our pre- or early pubertal patients catch-up growth occurred following surgical therapy and in all cases pubertal development proceeded normally.

Adenoma↗
PubMed1982

The effect of puberty and short-term oral administration of testosterone undecanoate on GH tests and sex-steroid related plasma compounds in GH deficient patients.

In twelve boys and two girls, with idiopathic (partial) growth hormone deficiency diagnosed at prepubertal age, we studied the effect of spontaneous puberty (six patients) as well as the effect of sex-steroid priming (eight patients). Six boys received testosterone undecanoate and two girls ethinyloestradiol for 5 days. Two pubertal patients showed a normal GH response, the patients primed with testosterone or oestrogen did not, despite a distinct effect on sex-steroid related plasma compounds. Reevaluation of GH status appears to be worthwhile in GH deficient patients presenting with short stature, growth deceleration at an early pubertal age and delayed sexual maturation. Sex-steroid priming is unlikely to alter the outcome of GH testing, whenever marked growth deceleration is evident at prepubertal age.

Adolescent↗
PubMed1982

The effect of ACTH stimulation on plasma steroids in two patients with congenital hypoaldosteronism and in their relatives.

In two children with isolated congenital hyperreninaemic hypoaldosteronism, as well as in their relatives, plasma levels of aldosterone (Aldo), corticosterone (B), deoxycorticosterone (DOC), 18-OH-B and 18-OH-DOC were measured before and after an iv bolus of 0.25 mg Synacthen (Ciba). A corticosterone methyl oxidase deficiency type II was demonstrated in one child. Her normoreninaemic parents (no consanguinity) had plasma values consistent with heterozygosity. The results in the other child and one asymptomatic sib were compatible with a partial corticosterone methyl oxidase deficiency type I. His parents were consanguine but had normal Aldo levels. Overnight dexamethasone administration did not suppress any of the steroids measured except cortisol, suggesting synthesis of these steroids by the zona glomerulosa.

18-Hydroxycorticosterone↗
PubMed1981

Prolonged galactorrhoea in a 6-year-old girl with isosexual precocious puberty due to a feminizing adrenal tumour.

A large cystic adrenal carcinoma was removed in toto from a 6 1/2-year-old girl with isosexual precocious puberty treated with cyproterone-acetate. Immediately following surgery, sever galactorrhoea developed, but was successfully treated with bromocriptine. The tumour fluid and pre-operative plasma samples contained highly elevated levels of both oestrogens and androgens. In addition prolactin values were high. One year after adrenalectomy all plasma hormonal values were normal for age, but galactorrhoea could still be elicited. The prolonged galactorrhoea is most likely the result of excessive oestrogen 'priming', whereas the hyperprolactinaemic effect of cyproterone acetate therapy remains uncertain.

Adrenal Gland Neoplasms↗