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Biomedical subjects

K E Petersen

Publications and source records attributed to K E Petersen.

At least 37 records · Page 2Linked to original sources
PubMed1982

X-linked congenital adrenal hypoplasia. A study of five generations of a Greenlandic Family.

In a Greenlandic family comprising 171 descendants in 5 generations, 11 boys had died with a clinical picture of adrenocortical insufficiency within three weeks of birth. Three treated male patients, who survived, were closely studied. The adrenal glands could not be identified by computed tomography. The clinical picture and biochemical studies were consistent with congenital adrenal hypoplasia. The pedigree indicates X-linked recessive inheritance. The pubertal development was delayed in two patients aged 14 years. LHRH stimulation yielded a significant rise in LH and FSH in at least one of the patients, adrenal androgen values were very low. A family instruction programme has been set up.

Adolescent↗
PubMed1982

Heterozygotes and cryptic patients in families of patients with congenital adrenal hyperplasia (21-hydroxylase deficiency). HLA and glyoxalase I typing and hormonal studies.

In a group of 18 unrelated Danish children with 21-hydroxylase deficiency (21-OH def.), human leukocyte antigen (HLA) typing revealed a significant increase of Bw47 and a significant decrease of B8. HLA studies of the families of 14 probands predicted among the siblings 11 heterozygote carriers and 3 genetically unaffected. Glyoxalase studies showed a recombination fraction of 8%. ACTH-stimulated 17-OH progesterone is the only hormone value useful in the discrimination between heterozygotes and normals. Two families are described in detail. In one family, one of two HLA-identical brothers had classical virilizing congenital adrenal hyperplasia (CAH), while the other was a normal boy without 21-OH def. In another family with 3 girls, one had classical, salt-wasting CAH, one had "late onset' CAH, and the third sister and the father shared the HLA-B14 antigen and were shown to have "cryptic' 21-OH def.

Adolescent↗
PubMed1981

ACTH in normal children and children with pituitary and adrenal diseases. I. Measurement in plasma by radioimmunoassay--basal values.

Plasma ACTH was measured by radioimmunoassay including preliminary extraction onto adsorbent glass particles. Normal basal values for children 4-16 years of age were 11-82 microgram/ml, corresponding to values found in the literature. Normal values were found in girls with precocious puberty, children with McCune-Albright and Beckwith-Wiedemann's syndrome. Values between 233 and 305 pg/ml were found in untreated children with congenital adrenal hyperplasia. In primary Addison's disease, very high levels (654-1 413 pg/ml) before cortisone therapy and their decline to nearly normal values during therapy are described. ACTH values in a case of pituitary Cushing's syndrome was just above the normal range (104-163 microgram/ml).

Adolescent↗
PubMed1980

Treatment of girls with excessive height prediction. Follow-up of forty girls treated with intramuscular estradiol and progesterone.

In a follow-up study of 40 tall girls treated with intramuscular estradiol and progesterone, the final height, bone age maturation, side effects and acceptance of treatment were evaluated. The mean duration of treatment was 18 months. During treatment, mean height increase was 6.5 cm (height velocity 3.7 cm/year), which is nearly 50% reduction of normal growth rate. The mean increase in bone age was 2.7 years (bone age velocity 1.8 years/year), which approximates twice the normal maturation rate. The mean reduction in final height was 5.0 cm as evaluated by the method of Bayley. Pinneau (BP), 2.9 cm by the method of Tanner et al. (TW) and 3.0 cm by the method of Roche et al. (RWT). The reduction was greatest when treatment was started before menarche, according to all three prediction methods. When treatment was started after menarch the calculated height reduction was greatest according to the BP method. There was good agreement between the three prediction methods in girls with a bone age below 12 years before treatment. In girls with a bone age above 12 years the height reduction by the BP method was much greater than when measured by the other methods. Side effects evaluated at follow-up were minimal and first menstruation occurred within 3 months (mean) after cessation of treatment. The number of pregnancies was estimated to be normal for age. All but three accepted the treatment. It is concluded that this type of treatment must be restricted to girls with severe psychological problems due to excessive height prognosis and selection for treatment must be based on an individualized evaluation.

Adolescent↗
PubMed1979

17-Hydroxyprogesterone in normal children and congenital adrenal hyperplasia. Measurement in serum by radioimmunoassay after thin-layer chromatography.

Serum 17alpha-hydroxyprogesterone (17 OH-P) was measured by a specific radioimmunoassay technique combined with thin-layer chromatography. Normal values for children are less than 1.1 microgram/1 (less than 3.3 nmol/l)--corresponding to values found in the literature. In congenital adrenal hyperplasia (CAH) values up to several hundred microgram/l are found. The values rise after ACTH stimulation and are suppressed by decadrone or cortisone treatment. The rise in 17 ketosteroids and pregnanetriol in untreated CAH is relatively smaller (15--25 fold). This clinical sensitivity of 17 OH-P is thus valuable for the diagnosis of CAH (21 hydroxylase deficiency). Furthermore it is easier to take a blood sample than to collect urine for 24 hours. The usefulness in therapeutic monitoring is being studied.

17-Ketosteroids↗
PubMed1979

Combined test of hypothalamic-pituitary function in growth-retarded children treated with growth hormone. II. Secretion of LH, FSH, TSH, prolactin and ACTH.

A total number of 23 patients treated with human growth hormone were retested by use of a combined pituitary stimulation test. Plasma concentrations of GH, FSH, LH, TSH, T4, T3, prolactin (PRL), ACTH and cortisol were measured before and after stimulation with hypoglycemia, TRH and LHRH. The test was performed in patients with persistent GH deficiency (group A) and patients with transitory GH deficiency (group B). In group A a normal pubertal development was found in three patients, whereas in prepubertal subjects the FSH/LH responses were smaller than those of prepubertal patients in group B. Also plasma ACTH increase was less pronounced in group A patients than in group B. In contrast, the plasma TSH and PRL responses were more sustained in group A than in group B. The secretory pattern of TSH and PRL was comparable in the two groups of patients. Thus, in patients with persistent GH deficiency additional multiple disturbances of the hypothalamic-pituitary function often appeared whereas in most patients with transitory GH deficiency the combined pituitary test was normal at the reinvestigation.

Adolescent↗
PubMed1979

Combined test of hypothalamic-pituitary function in growth retarded children treated with growth hormone. I. Secretion of growth hormone and somatomedin before and after treatment.

In 23 growth retarded children two consecutive insulin tolerance tests (ITT) were performed to establish a diagnosis of growth hormone (GH) deficiency. Nine children did not respond (GH peak value less than 8 mU/1), whereas 14 were classified as having partial GH deficiency (GH peak value less than 20 mU/1). All were treated for an average period of 40 months with human growth hormone (HGH). In a combined stimulation test at the end of the treatment period 9 children demonstrated a persistent GH deficiency, whereas a normal response was found in 14 of the previous partial GH deficient children. During treatment the monthly growth rate rose from 0.21 cm 0.58 cm in the GH deficient children and from 0.31 cm to 0.70 cm in the partial deficient children, in most of whom spontaneous pubertal development occurred during treatment. Somatomedin (SM) values were decreased in the GH deficient children before and after treatment but increased to normal levels during treatment. Growth velocity in these children during treatment was correlated to SM values before treatment. In the partial GH deficient children SM values were subnormal before but normal after treatment. This supports the assumption that in some children with constitutional delay in puberty a reversible functional hypopituitarism exists, which is normalized after the onset of puberty, due to androgens sensitizing growth hormone releasing mechanisms. Treatment with HGH may induce increased growth velocity in some of these patients.

Body Height↗
PubMed1977

Familial adrenocortical hypoplasia with early clinical and biochemical signs of mineralocorticoid deficiency (hypoaldosteronism).

Biochemical investigations in a boy with a salt losing syndrome revealed a very low secretion of aldosterone which did not rise during salt deprivation, in spite of a normal rise in plasma renin activity. Cortisol secretion was normal - but subsequently decreased, while the corticosterone secretion was high. The patient was studied at the age of 5 weeks, 3 months and also at the age of 8 months. He survived until the age of 18 months on treatment with sodium chloride and DOCA, but did not receive glucocorticoids. At autopsy the adrenal glands were absent, but in fat tissue from the upper renal poles foetal adrenal cortex tissue was found. The histological picture agrees well with other cases which could be designated as "foetal-cortex-only" adrenal hypoplasia. The same histological changes were demonstrated in the boy's brother who died suddenly at the age of 6 weeks. The boy's testes were advanced in maturation to a stage of about ten years: spermatocytes and Leydig cells were present.

17-Hydroxycorticosteroids↗