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Biomedical subjects

S L Drop

Publications and source records attributed to S L Drop.

At least 109 records · Page 6Linked to original sources
PubMed1990

Twenty-four-hour plasma growth hormone (GH) profiles, urinary GH excretion, and plasma insulin-like growth factor-I and -II levels in prepubertal children with chronic renal insufficiency and severe growth retardation.

We studied 24-h plasma GH profiles, maximal GH responses to arginine provocation and insulin-like growth factor-I (IGF-I) and IGF-II levels in plasma in 22 euthyroid prepubertal children (mean age, 9.5 yr) with chronic renal insufficiency (glomerular filtration rate, less than 20 mL/min.1.73 m2) and severe growth retardation [mean (+/- SD) height SD score (SDS), -2.8 (1.1)]. The 24-h GH profiles were analyzed using the Pulsar program. Girls had significantly higher 24-h GH secretion than boys (P less than 0.004). Children with end-stage nephrotic syndrome had higher baseline GH levels and total area under the curve (AUCo) than patients with dysplastic kidneys (P less than 0.05), while the area under the curve above baseline (AUCb) was similar in all types of renal diseases. The type of treatment (conservative, peritoneal, hemodialysis) did not significantly influence the 24-h GH secretion. No correlation was found between 24-h GH profiles and age, height SDS for chronological age, height velocity SDS for bone age, and weight for height. Fourteen children showed a normal 24-h GH profile, defined as a GH profile with well defined, regular GH peaks returning to baseline GH levels and a distinct day and night pattern (AUCb, 90-300 micrograms/L.24 h). Four children had low profiles, with GH peaks below 10 micrograms/L, returning to baseline GH levels and occurring almost exclusively during the night (AUCb, less than 90 micrograms/L.24 h). The remaining four children had elevated 24-h GH profiles, with GH peaks on top of elevated baseline GH levels of more than 3 micrograms/L (AUCb, 35-205 micrograms/L.24 h; AUCo greater than 300 micrograms/L.24 h). In all patients 24-h urinary GH and beta 2-globulin excretion was 100-1000 times higher than that in controls. The urinary GH excretion correlated significantly with all characteristics of the 24-h GH profiles (r = 0.57-0.59; P less than 0.05). The maximal GH response during the arginine tolerance test was normal in 66% of the children. The mean (+/- SD) SDS for bone age for the IGF-I plasma levels was +1.1 (1.9), and that for IGF-II was +3.6 (3.4). IGF-I levels correlated significantly with the AUCb, maximum GH, and GH peak characteristics of the 24-h GH profiles (r = 0.05-0.73; P less than 0.02-0.001). IGF-II levels did not show any correlation with the characteristics of the endogenous GH secretion.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1989

Congenital hypothyroidism and partial thyroid hormone unresponsiveness of the pituitary in a patient with congenital thyroxine binding albumin elevation.

We describe a girl who presented at the age of 6 weeks with cardiogenic shock due to congenital hypothyroidism (serum thyroxine (T4) less than 12 nmol/l). Thyroxine replacement therapy was instituted. In spite of high total serum T4 levels, thyroid stimulating hormone (TSH) serum values remained elevated. The raised serum T4 levels were the result of congenital elevation of thyroid binding albumin (TBA). Toxic doses of both T4 and triiodothyronine (T3) normalized the elevated TSH levels indicating that the pituitary is responsive to thyroid hormone, albeit at a higher threshold. In patients with congenital TBA elevation and an altered T4 pituitary response requiring thyroid replacement therapy, the measurement of serum free T4 levels is the parameter of choice to monitor treatment.

Congenital Hypothyroidism↗
PubMed1989

A controlled trial of methionyl growth hormone therapy in prepubertal children with short stature, subnormal growth rate and normal growth hormone response to secretagogues. Dutch Growth Hormone Working Group.

Thirty short and slowly growing children with normal plasma growth hormone (GH) responses to standard provocation tests were randomly assigned to either a group (n = 20) undergoing treatment with methionyl GH (somatrem), 2 IU per m2 body surface s.c. daily, or a control group (n = 10). Twelve out of 18 children who completed the first year of treatment showed a height velocity increment of more than 2 cm/year. The mean (SD) growth velocity of the treatment group increased by 3.0 (1.9) cm/year over the first year, compared with -0.2 (0.7) cm/year in the control group. Neither parameters of endogenous GH secretion nor plasma IGF-I levels showed a significant correlation with the growth response. Of the auxological variables studied, pre-treatment growth velocity (r = -0.8) and the short-term height velocity increment (r = 0.7-0.9) showed significant correlations with the growth response in the first year of treatment. Somatrem therapy was without side effects, except in one child who developed anti-GH antibodies in combination with a poor growth response.

Body Height↗
PubMed1988

Organization of the gene encoding the insulin-like growth factor binding protein IBP-1.

Human genomic clones encompassing the tissue specific expressed gene IBP-1, an insulin-like growth factor binding protein were isolated and characterized. The gene is organized in four exons and spans 5.9 kb. S1 nuclease analysis determined a single transcription start site. The first exon and 5' flanking region are highly GC rich and located in a CpG island. The CpG island enclose the CAAT box, the TATA box, the transcription start site and a potential SP1 transcription factor binding site. The presumptive promoter region is characteristic for genes expressed in a tissue specific fashion. All signals required for cleavage/polyadenylation are located within exon IV, predicting a mRNA of 1.5 kb which is consistent with the size seen on RNA blots.

Amino Acid Sequence↗
PubMed1988

Isolation and characterization of a cDNA encoding the low molecular weight insulin-like growth factor binding protein (IBP-1).

IGF-I and IGF-II are growth-stimulating peptides with strong mitogenic properties. These polypeptide growth factors circulate in serum bound to specific binding proteins. We report the cloning and complete sequence of a cDNA encoding a low mol. wt IGF-binding protein from a human placenta cDNA library. We propose the designation IGF-binding protein 1 (IBP-1) for the gene and corresponding protein. Expression of the cDNA encoding IBP-1 in COS cells resulted in the synthesis of a 30-kd protein which binds IGF-I and is immunologically indistinguishable from the IGF-binding protein isolated from amniotic fluid or human serum. Northern blotting analysis demonstrated that expression of the IBP-1 gene is highly tissue specific and limited to placental membranes and fetal liver suggesting a rigid control. The IBP-1 gene is a single copy gene, located on chromosome 7. The results obtained suggest that most, if not all, lower mol. wt IGF-binding proteins originate from this gene.

Amino Acid Sequence↗
PubMed1988

Methionyl human growth hormone in Turner's syndrome.

Sixteen girls with Turner's syndrome aged 7.9-15.2 years (bone ages 7.0-11.8 years) were given methionyl growth hormone (somatrem) 4 IU/m2 body surface daily, corresponding to 0.9 IU/kg/week. During one year of treatment their mean (SD) height velocity increased from 3.4 (0.9) to 7.2 (1.7) cm/year and height prediction from 148.2 (4.4) to 150.0 (4.4) cm. All the girls except one had a height velocity increment of more than 2 cm/year and these velocities are above the age references for girls with Turner's syndrome. The girl with a low growth response had antibodies against growth hormone with high binding capacity (3.7 U/l). The height velocity increment was inversely correlated with age and bone age, but this might be partly due to the somewhat higher dosage/m2 body surface and kg body weight that the younger patients were given because of the rounding off of the dose. The better results of our study compared with those of other workers who used similar dosages but did not give the drug as often suggest that giving it daily might have increased the growth response as it does in children deficient in growth hormone.

Adolescent↗
PubMed1988

Hormonal evaluation of boys born with undescended testes during their first year of life.

We studied pituitary-gonadal function during the first year of life in 48 boys born with 56 undescended testes in order to test the hypotheses that functional insufficiency of the hypothalamo-pituitary-gonadal axis and disorders of testosterone (T) biosynthesis occur in such boys. Cryptorchidism persisted for longer than 1 yr in 29 boys (30 testes; group I), whereas spontaneous descent occurred in 19 boys (20 testes; group II), in 6 after the sixth month. A control group (group III) included 160 boys. Basal and peak LHRH-stimulated serum LH and FSH and basal serum T values were determined at 3, 6, and 12 months. Serum T, dihydrotestosterone (DHT), progesterone (P), 17-hydroxypregnenolone, 17-hydroxyprogesterone, dehydroepiandrosterone sulfate, and androstenedione before and after hCG administration were determined at age 1 yr. Comparing the 3 groups, cross-sectional evaluation revealed no significant differences in basal or peak LHRH-stimulated serum LH and FSH levels, except that basal serum LH levels were slightly higher in group II than in group III. Comparing groups I and II, longitudinal evaluation revealed similar basal and peak LHRH-stimulated serum LH and FSH values, with comparable changes with time. Basal serum T, DHT, and T precursor levels were similar in all three groups, with similar rises of T and DHT and variable minimal increases in androstenedione and dehydroepiandrosterone sulfate after hCG stimulation. We conclude that during the first year of life, boys with cryptorchidism have no functional insufficiency of the hypothalamo-pituitary-gonadal axis or disorders in T biosynthesis.

17-alpha-Hydroxyprogesterone↗
PubMed1987

Evidence of 11 beta-hydroxylase deficiency in childhood adrenocortical tumors. The plasma corticosterone/11-deoxycorticosterone ratio as a possible marker for malignancy.

In search for a biochemical marker to differentiate between adrenocortical carcinoma (AC) and adenoma (AA), plasma levels of the following steroids were studied preoperatively and postoperatively: 11-deoxycorticosterone (DOC), corticosterone (B), 11-deoxycortisol (S), and cortisol (F). Levels were measured by Sephadex LH-20 chromatography and specific radioimmunoassays. The subjects included eight children ages 2 years, 5 months to 9 years, 10 months. There were three girls and 5 boys with pseudoprecocious puberty due to adrenocortical tumors (histologically, four were AC and four, AA). The preoperative showed that DOC and S levels were elevated in all patients, F levels were elevated in four of eight children when compared with age-matched controls, whereas B was normal. Postoperatively, all levels returned to normal. The ratios of B/DOC and F/S as an index of adrenal 11 beta-hydroxylase activity were calculated. The preoperative ratios of B/DOC were markedly decreased in all patients with AC compared to controls (7.7,4.1,5.9,1.9 versus 23.5, median), but normal in three of four patients with AA (16.2, 29.6, 16.1). The F/S ratios were significantly lower in AC and AA when compared with controls. The data indicate a deficiency in 11 beta-hydroxylation in cases of adrenocortical tumors. Despite a still limited number of patients, the decreased B/DOC ratios may possibly indicate malignancy and could be helpful in distinguishing by biochemical means between benign and malignant adrenocortical tumors.

Adenoma↗
PubMed1987

LH-RH nasal spray treatment for cryptorchidism. A double-blind, placebo-controlled study.

A double-blind, placebo-controlled study of the efficacy of LH-RH nasal spray in treatment of cryptorchidism involving 252 prepuberal boys with 301 undescended testes, showed a success rate of 9% (14 testes) for LH-RH and 8% (10 testes) for placebo. Including a subsequent open study and a second course of LH-RH as required, the rate for LH-RH rose to 18% (48 testes). The follow-up period saw late descent in another 5% (14 testes), in a few cases coinciding with the onset of puberty. Retrospective evaluation revealed a previous scrotal position of the testes for 43% of the boys with success of treatment and even for 17% of the boys with failure of treatment. In many of these cases surgery revealed an anatomical anomaly that might have caused testicular ascent. Hormonal evaluation revealed no abnormalities in cryptorchid boys compared with control subjects, nor was there a divergence in values before and after treatment. The lower the pretreatment testicular position, the better the rate of success.

Administration, Intranasal↗
PubMed1987

The effect of treatment with an LH-RH agonist (Buserelin) on gonadal activity growth and bone maturation in children with central precocious puberty.

Twenty-five children (23 girls and 2 boys) with central precocious puberty were treated with the LH-RH agonist D-Ser (TBU)6-LHRH (1-9) EA (HOE 766, Buserelin) by daily subcutaneous injection for a period of 11-18 months. Eight girls and 2 boys previously treated with cyproterone acetate (CPA, 100-150 mg/m2 body surface per day) and the first seven newly diagnosed patients received 2 X 10 micrograms Buserelin/kg bodyweight per day for 1 week, followed by a maintenance therapy of 1 X 10 micrograms/kg per day. After an initial marked increase, oestrogen (E2) serum levels in girls and testosterone (T) values in boys decreased. After a treatment period of 6-20 weeks the patients received 2 X 20 micrograms Buserelin/kg per day for 1 week and thereafter a maintenance dosage of 20 micrograms/kg per day to obtain full suppression (i.e. E2 less than 50 pmol/l; T less than 1 nmol/l). The remaining eight patients started directly on 2 X 20 micrograms Buserelin/kg per day followed by 1 X 20 micrograms/kg per day. All eight girls with menarche before therapy had no further menses. In all girls there was a reduction of palpable breast tissue. Decrease of pubic hair development was observed in 3 girls, an increase was seen in 5 girls, whereas in the remaining 15 girls no change was observed. Both boys had a reduction of testicular volume and of pubic hair.(ABSTRACT TRUNCATED AT 250 WORDS)

Bone Development↗
PubMed1986

Double-blind, placebo-controlled study of luteinising-hormone-releasing-hormone nasal spray in treatment of undescended testes.

In a double-blind, placebo-controlled study, 252 prepubertal boys with 301 undescended testes were treated with luteinising-hormone-releasing-hormone (LHRH), 1.2 mg/day intranasally. After the 8-week double-blind period 10 placebo-treated (8%) and 14 LHRH-treated (9%) testes had completely descended. After a second LHRH course, involving all the subjects in an open study, 48 testes (18%) had descended completely. The lowest success rate (7%) occurred in the youngest age group (1-2 years). Of the successfully treated testes, 75% could be manipulated at least to the scrotal entrance before treatment. In comparison with age-matched controls, the cryptorchid boys' responses to LHRH and human chorionic gonadotropin before treatment did not suggest a deficient hypothalamo-pituitary-gonadal axis or deficient Leydig cell function. After treatment there was no evidence of stimulation of the hypothalamo-pituitary-gonadal axis; serum testosterone did not increase. Surgery was required in 170 patients (196 testes) revealing various anatomical anomalies.

Administration, Intranasal↗
PubMed1986

Primary adrenocortical insufficiency in childhood.

Seven patients with primary glucocorticoid or glucocorticoid and mineralocorticoid deficiency are described with emphasis on the clinical presentation and laboratory investigations. Two patients presented with irreversible shock and at autopsy adrenal tissue was recognized only microscopically. In 3 patients adrenal antibodies were present. One girl had the polyglandular autoimmune disorder type I and one boy had glucocorticoid deficiency only. The histories of the patients illustrate that the presenting symptoms of primary adrenocortical insufficiency are very insidious and that it may take several years before the correct diagnosis is made and hydrocortisone substitution therapy is instituted.

Adrenal Glands↗