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Congenital hyperplasia of the adrenal cortex is manifested in boys as macrogenitosomia praecox and in girls as female pseudohermaphroditism, with or without concomitant adrenocortical insufficiency.

Some tendency to familial occurrence of congenital adrenocortical hyperplasia has previously been observed. According to a survey by Bentinck et al. (1952) a total of 97 cases of familial somatosexual aberrations affecting 42 families were reported between 1852 and 1952. Eighty of these children were either definitely or probably female pseudohermaphrodites and 17 had either demonstrable or probable macrogenitosomia praecox. In no family, however. was it possible to demonstrate virilizing adrenocortical hyperplasia in more than one generation. Wilkins et al. (1950, 1955) have accordingly assumed that congenital hyperplasia of the adrenal cortex is due to a recessive hereditary genital defect, clinically manifest only in homozygotes.

In this paper a description will be given of the clinical findings in a family with six children, including three

ABSTRACT

A longitudinal study was made of the daily urinary excretion, on or near each birthday, of a number of C₁₉ and C₂₁ steroids in 9 healthy girls and 5 healthy boys aged 3 to 7 years. The amount of androsterone excreted by each individual increased slowly during the period of study but the absolute amounts varied greatly between individuals. The excretion of aetiocholanolone was greater than that of androsterone, contrary to reported findings in older children. Small amounts of DHA were found. Testosterone was found in only about 40% of samples; epitestosterone in 70 % and 11β-OH-androsterone in only 62 %. Cortisol metabolites were excreted in amounts which increased with age and all three metabolites of corticosterone were present in most specimens. 11-Deoxycortisol was found in about 50 % of the samples and THS in 63 %. The mean trend in the ratio of glucuronides to sulphates of the 11-deoxy-17-oxosteroids decreased with increasing age, but the 11-deoxy-11-oxy ratio of 17-oxosteroids increased as did the 5α/5β ratio of the C₁₉ and C₂₁ steroids.

No sex differences were observed. The excretion of cortisol metabolites showed a positive correlation with height and weight. 11-Deoxy-17-oxosteroids were positively correlated with the weight. No significant relationships between steroid excretion and skeletal maturity were found.

ABSTRACT

The influence of ovine follicle stimulating hormone (NIH-FSH-S-3) on ovine luteinizing hormone (NIH-LH-S-8) in the Ventral Prostate Weight method (VPW) was studied. Adding of NIH-FSH-S-3 to NIH-LH-S-8 at ratios of 1:1, 4:1 and 10:1 gave no significantly higher responses than did NIH-LH-S-8 alone.

Urinary extracts from 2 women, hypophysectomized for metastasizing mammary carcinoma and from 3 children between 2 and 5 years old (1 boy, 2 girls) gave no positive response with the doses employed (1/4 of a 24 hours urine sample total per rat). It is concluded that the Ventral Prostate Weight method in hypophysectomized rats is specific for the assay of luteinizing hormone.

Abstract. The steroid binding capacity and concentration of plasma sex hormone binding globulin have been compared in 116 children aged between 2 and 14 years. Concentration was measured by electroimmunodiffusion standardised with reference to the mass of the pure protein and binding capacity by quantitating the binding of radiolabelled 5α-dihydrotestosterone. Binding capacity correlated highly with concentration in all subjects and neither differed significantly between the sexes before or during puberty. However, both were significantly lower in pubertal than in pre-pubertal children. These findings suggest the metabolism of the protein is similar in boys and girls and that the fall in its steroid binding capacity at puberty in fact is due to a fall in its concentration rather than to changes in its physicochemical properties.

Abstract.

In 4 girls and 1 boy with pseudo-hypoparathyroidism growth and physical maturation were followed longitudinally for 7 – 13 years until adult height had been reached. As a result of early puberty and cessation of growth all patients were relatively shorter as adults than in their childhood years. The difference between average height at the age of 8.0 years and average adult height was 2.25 sd. This observation offers an explanation for the finding in the literature that short stature is more common in adults with this disease than in children. Skeletal age was advanced in all cases and the development of the tubular bones of the hand was more advanced than the development of the round bones. It is possible that this difference resulted from inappropriately early closure of the epiphyseal discs of disproportionally short metacarpals and phalanges. On the other hand it may be an aspecific phenomenon of advanced skeletal maturation.

Abstract.

Serum somatomedin B levels were determined by radioimmunoassay in 209 healthy boys and girls from one month to 16 years of age.

Low values were found up to the second year life. In the first year the mean level was 13.8 mg/l in girls and 11.5 mg/l in boys. In older children the values increased to levels between 13 and 22 mg/l in boys and between 13 and 18.5 mg/l in girls. They were independent of the stage of pubertal development.

Somatomedin B levels were normal in 71 children with constitutional growth delay, primordial dwarfism, familial dwarfism and other forms of growth disturbance. The mean levels were between 12.1 and 14.4 mg/l.

Values below 6 mg/l were present only in children with hGH deficiency. In these patients we could find an increase of the mean level from 4.3 mg/l without therapy to 9.4 mg/l under treatment.

Thus the determination of somatomedin B seems to be useful for the diagnosis of hGH deficiency.

Abstract. Luteinizing hormone (LH) bioactivity was determined by an in vitro microbioassay in 65 plasma samples from 26 infants and young children between 10 days and 6.5 years of age. In addition LH was measured by radioimmunoassay. For both, LH preparation LER-907 was used as standard.

Biologically measured LH values (bio-LH) were always higher than radioimmunologically determined LH values (RIA-LH) as reflected by bio/RIA ratios greater then 1.0.

In male infants bio-LH was elevated up to 7 months of age. Thereafter, it decreased and remained low over the age range of our study. In female infants bio-LH was also elevated, but decreased after 1 month and showed a slight tendency to rise in the age group 3–6.5 years.

The results of RIA-LH showed approximately the same pattern, but at a lower level. In boys RIA-LH levels were highest around 1 month of age, decreased steadily between 2 and 7 months and remained constant thereafter. Girls had rather constant RIA-LH values between 2 months and 3 years of age. In contrast to bio-LH, there is a clear-cut drop of RIA-LH in the age group 3–6.5 years, resulting in a statistically significant increase of the bio/RIA ratio.

In boys the time course of the bio-LH changes coincides with the known elevation of testosterone during the first months of life.

In girls the elevated bio-LH levels observed during the first month are not so far associated with a known steroid correlate.

Our study shows an increased biological activity of circulating LH and a marked dissociation of biologically and radioimmunologically active LH during early infancy, analogous to observations during puberty.

Abstract. The effect of more frequent (daily) injections of human growth hormone (hGH) on growth rate was studied in 16 growth hormone deficient children (12 boys, 4 girls) during 2 years. All had previously been treated with im injection of hGH 2–3 times weekly and in the majority of the patients a waning growth response was observed.

For a total weekly dose of 12 IU hGH a daily dose of 2 IU was injected sc at night before sleep. This dosage has been shown by us to imitate the average nocturnal hGH profile in plasma.

Growth response on the im treatment was 5.2 ± 1.2 cm/year (sd) in boys and 5.4 ± 0.9 cm/year in girls. A significant increase was seen during the first year of sc treatment to 7.9 ± 2.7 cm in boys and 6.3 ± 2cm in girls. During the second year the growth response was still significantly increased in boys (7.2 ± 1.9 cm). Bone age was more advanced and the period of previous im treatment was longer in girls (6.7 vs 3.6 years) which may be the main cause of the waning second year response (4.7 ±1.3 cm/year). Pubertal development occurred in 9 children during treatment. However, the highest growth rates were not found in these children.

Absence of antibodies against hGH and local reactions at the injection site is evidence of the safety of the treatment, which was very well accepted by the children.

Daily sc injections thus represent an effective alternative to conventional im injections ensuring high acceptance in children with growth hormone deficiency.

The aim of the present work was to obtain bone mass estimates of healthy Greek children aged 6–18 years. This work was considered worthwhile since similar data are very few in the world litterature (Bonnard 1968, Gryfe et al. 1971), while those for Greece (Livadas et al. 1975) refer to 902 children only (462 boys and 440 girls) aged 5–13 years.

The material of the present study consists of 2.406 schoolboys and 2.451 schoolgirls aged 6–18 years, of whom 864 boys and 1.189 girls were living in Attica, while the remaining 1.542 boys and 1.262 girls were living in communities outside Attica (i.e. Atalanti, Arnea, Elatia and Karpenisi). Standing body height and body weight was measured in all subjects. Furthermore, a plain x–ray of the left hand was taken in all children, using a focal distance of 80 cm.

By means of a micrometer apparatus (Taschenmessloupe TM4, C. Zeiss) the

Abstract. The availability of iodinated salt containing 20 mg of iodine as iodate/kg salt consumed on a voluntary basis enabled us to investigate its effect on goitre prevalence and iodine excretion in urine in a longitudinal, prospective, randomized study over 4 years. With this salt, under the assumption of a consumption of 5 g salt per day and person, an additional intake of 100 μg of iodine can be achieved. The study was performed on initially 334 children (168 boys, 166 girls) at the age of 10 years living in an area of iodine deficiency. After 4 years, 286 children still participated in the study. Initially, goitre prevalence as assessed by palpation was found to be 30.5% (37.4% in girls and 23.8% in boys). Neck circumference was found to be significantly higher in children with goitre compared with those without (30.2 ± 1.4 vs 29.4 ± 1.4 cm; P < 0.001). Iodine excretion in the urine was significantly lower in children with goitre compared with those without (40.4 ± 16.7 μg/g creatinine vs 46.1 ± 24.9 μg/g creatinine; x ± sd; P < 0.05). The children were randomly assigned to two different groups: group A (N = 146) was asked to use iodinated salt, group B (N = 188) non-iodinated salt. Over the 4 years, a continuous increase in iodine excretion in urine could be demonstrated in group A. After 1 year, it was significantly higher than in the control group that used non-iodinated salt. After 4 years, the mean iodine excretion in children using iodinated salt was 60.1 ± 24.1 μg/g creatinine in contrast to 45.1 ± 18.6 μg/g in the control group (x ± sd; P< 0.0001). However, no decrease in goitre prevalence could be documented: after 4 years, 23.8% of the children belonging to the group using iodinated salt and 22.5% of those in the group taking non-iodinated salt had a goitre. From these observations we conclude: 1. The voluntary use of a commercially available iodinated salt containing 20 mg iodate/kg leads to a significant increase in iodine intake, measured by urinary iodine excretion. Even after 4 years, the value is far below the daily iodine intake recommended by the WHO. No decrease in goitre frequency could be assessed. 2. An increase in iodine ingestion can be achieved either by increasing the iodine content of the salt or by application of iodine by alternative measures. The safest way would be to use iodinated salt exclusively, i.e. also in the food industry and restaurants. An increase in the iodine content of the salt and its continuous voluntary use would lead to a large variation in iodine intake. A higher risk of adverse reaction, e.g. iodine-induced thyrotoxicosis, cannot be excluded in susceptible persons.