Prevalence of coeliac disease in Turner syndrome.

This study was undertaken to investigate the prevalence of coeliac disease in children and adolescents with Turner syndrome. Eighty-seven children and adolescents with Turner syndrome were screened for IgA-antiendomysium antibodies (EMA) and IgA-antigliadin antibodies (AGA), 5% (4/87) being found to be EMA-positive, and 15% (13/87) to have AGA levels above normal. Of the 10 patients who were either AGA- or EMA-positive and further investigated with intestinal biopsy, four manifested villous atrophy (i.e. all three of the EMA-positive patients, but only one of the seven AGA-positive patients). The results suggest EMA-positivity to be a good immunological marker for use in screening for coeliac disease, and such screening to be justified in patients with Turner syndrome.

Effect of growth hormone (GH) during puberty in GH-deficient children: preliminary results from an ongoing randomized trial with different dose regimens.

This paper reports results from an ongoing, randomized, multicentre national trial. The aim is to elucidate whether a dose of growth hormone (GH) of 0.2 IU/kg (0.07 mg/kg), given either as once-daily or twice-daily injections during puberty, is more effective than a once-daily dose of 0.1 IU/kg/day (0.03 mg/kg/day) in improving final height in children with GH deficiency (GHD). The twice-daily regimen comes closer to the spontaneous GH secretion pattern in puberty. Ninety-two children with GHD who had been receiving GH therapy for at least 1 year, and with spontaneous puberty or who were prepubertal and due to be started on replacement therapy to induce puberty, were randomly assigned to receive GH as follows: group A, 0.1 IU/kg/day (0.03 mg/kg/day), administered once daily; group B, 0.2 IU/kg/day (0.07 mg/kg/day), administered once daily; and group C, 0.2 IU/kg/day (0.07 mg/kg/day), divided into two equal injections given at 12-hour intervals. Pubertal height gain was 0.7, 0.7 and 1.3 SDS for groups A, B and C, respectively. The gain in height during puberty was thus most marked in group C. Mean final height, when corrected for parental height, was between 0 and 1 SDS in all treatment groups. All but seven children reached a final height within +/- 2 SD of the general population. There was a wide range of final heights in all three treatment groups. This variation in response suggests the need to individualize treatment in order to achieve an appropriate final height for most individuals.

Children with nocturnal upper airway obstruction: postoperative orthodontic and respiratory improvement.

UNLABELLED: Twenty children, aged 4-9 years, underwent adeno/tonsillectomy because of unequivocal anamnestic nocturnal obstructive breathing. Preoperatively, apnea-hypopnea index was > 5 in 10 cases only, AI > 1 in 17. Nineteen children had signs of increased respiratory labour in movement recordings and inspiratory EMG-activity. Oxygen desaturation index was 0 in 7 children, and nadir SaO2 was > or = 90% in 10. Cephalometry and dentition study models initially revealed significant changes, chiefly lateral cross-bite (n = 11) and vertical growth direction of the mandible. Tonsillar size or duration of disease was not correlated with the severity of polysomnographic findings, nor were orthodontic variables. Symptoms disappeared promptly postoperatively. After one year, respiratory recordings were normalized or improved in the majority of children, and orthodontic variables normalized or improved in all children. CONCLUSION: Oximetry and airflow recordings may be normal in children who benefit from treatment of anamnestic nocturnal obstruction. Craniofacial deformities are common and improve significantly with surgical treatment of the airway obstruction.

Growth hormone treatment of short children born small-for-gestational-age: the Nordic Multicentre Trial.

The aims of this study were to evaluate the efficacy and safety of different doses of growth hormone (GH) treatment in prepubertal short children born small-for-gestational-age (SGA). Forty-eight children born SGA from Sweden, Finland, Denmark and Norway were randomly allocated to three groups: a control group of 12 children received no treatment for 2 y, one group was treated with GH at 0.1 IU/kg/d (n=16), and one group was treated with GH at 0.2 IU/kg/d (n=20). In total 42 children completed 2 y of follow-up, and 24 children from the treated groups completed 3 y of treatment. Their mean (SD) age at the start of the study was 4.69 (1.61) y and their mean (SD) height was -3.16 (0.70) standard deviation scores (SDS). The children remained prepubertal during the course of the study. No catch-up growth was observed in the untreated group, but a clear dose-dependent growth response was found in the treated children. After the third year of treatment, the group receiving the higher dose of GH, achieved their target height. The major determinants of the growth response were the dose of GH used, the age at the start of treatment (the younger the child, the better the growth response) and the family-corrected individual height deficit (the higher the deficit, the better the growth response). Concentration of insulin-like growth factor-I (IGF-I) and IGF-binding protein-3 increased during treatment. An increase in insulin levels was found without negative effects on fasting glucose levels or glycosylated haemoglobin levels. GH treatment was well tolerated. In conclusion, short prepubertal children born SGA show a dose-dependent growth response to GH therapy, and their target height SDS can be achieved within 3 y of treatment given GH at 0.2 IU/kg/d. However, the long-term benefit of different regimens of GH treatment in children born SGA remains to be established.

Mutans streptococci and lactobacilli in saliva from children with insulin-dependent diabetes mellitus.

The quantitative distribution of mutans streptococci and lactobacilli in saliva of insulin-dependent diabetic children was compared with a group of healthy children and related to the metabolic control of the disease. The study group, consisting of 94 boys and girls (age 4-19) with type 1 diabetes was matched by sex and age with a non-diabetic control group. Stimulated whole saliva was collected and flow rate, buffer capacity and the levels of mutans streptococci and lactobacilli were analysed in all children. In the diabetic group, total salivary proteins and glucose content of saliva were determined. Data on caries experience were recorded from the dental cards of all children. There were no difference in the distribution or number of mutans streptococci between the groups, but significantly (p less than 0.05) lower levels of lactobacilli were found among the diabetic children. The number of lactobacilli was positively correlated (p less than 0.05) to glucose concentration in saliva. There was no difference in the prevalence of caries between the groups. The present findings suggest that the dietary treatment of young insulin dependent diabetics gives rise to a reduced number of lactobacilli in saliva but does not affect the mutans streptococci.

Corticosteroid treatment of maternal ITP and risk of neonatal thrombocytopenia.

Maternal ITP is associated with a high risk of thrombocytopenia in the fetus and newborn infant. Methods of predicting this risk include determinations of maternal platelet count and platelet-associated IgG prior to delivery. In this report we show that prednisolone treatment normalized maternal platelet count and platelet-associated IgG without eliminating circulating platelet antibodies, which thus crossed the placenta and rendered the infant severely thrombocytopenia. Determination of maternal circulating platelet antibodies may be the best way of predicting infant thrombocytopenia, especially if the mother has received corticosteroids.

On the structural and phyletic origin of the aminergic nerves of the hypophysis of frog tadpoles (Rana temporaria) with special reference to pars distalis.

With the aid of lesion experiments and the Falck-Hillarp fluorescence technique, it is shown that the aminergic nerves of the hypophysis, including pars distalis, originate in the caudal hypothalamus and reach the gland via a fibre tract in the floor of tuber cinereum. However, some contribution to the eminentia mediana from the preoptic area could not be excluded. A vasomotor function of the pars distalis nerves is unlikely. Based on a comparative survey the hypothesis is put forward, that the pars distalis nerves, which disappear during the metamorphic climax, are remnants of an aminergic innervation existing together with a hypophyseal portal system as a functional link between brain and pituitary in the fish-like ancestor of amphibians. Aspects are given on the problem of why a direct pars distalis innervation has neither been retained nor evolved during evolution of tetrapods.

The ontogenesis of monoaminergic nerve fibres in the hypophysis of Rana temporaria with special reference to the pars distalis.

In Rana temporaria tadpoles, fluorescent fibres appear in the prospective eminentia mediana, the pars intermedia and the pars distalis at Gosner's stage 25. During prometamorphosis the amount of fluorescent material increases around the developing primary capillary plexus in the eminentia mediana. In the pars intermedia the fibres form a dense fluorescent network but in the pars distalis the fibres are few and delicate. At stages 42-43, the onset of climax, the pars distalis fibres disappear. The possible functional significance of the pars distalis fibres is discussed. The background adaptation ability appears at stages 28-29, while the fluorescent pars intermedia innervation is observable at stage 25.