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Unlabelled: Early puberty is frequently observed in adopted children. This randomized trial treated 30 adopted children with early puberty and short stature with either gonadotropin-releasing hormone agonist (GnRHa) alone or in combination with growth hormone (GH) for 3 y. Before the start of treatment (T1) in the trial and at discontinuation (T2) the children and their parents underwent a psychological evaluation. At the start of treatment the children did not have increased levels of behavioural or emotional problems as assessed by the Child Behaviour Checklist (CBCL). During treatment the CBCL scores did not increase. Self-perception of the children appeared to be normal, and after 3 y a significantly higher score for acceptance by peers was observed. At T1, an overestimation of future height was present in 80% of the children and 17% of the parents. Lower family stress was observed at T1 and T2 compared with reference values. Intelligence quotient levels decreased significantly during treatment. The findings are discussed with reference to the reported levels of behavioural and emotional problems in adopted children and the psychosocial effects of precocious puberty.Conclusion: This psychological evaluation did not reveal any consistent abnormalities in adopted children with early puberty. Treatment with GnRHa with or without GH did not increase emotional and behavioural problems in adopted children, nor was their self-perception decreased. [ABSTRACT FROM AUTHOR]

Objectives: To study final height in girls with Turner's syndrome treated with once or twice daily injections of growth hormone (GH) in combination with low dose ethinyl oestradiol.Design: Until final height was reached, the effect of fractionated subcutaneous injections given twice daily was compared with once daily injections of a total GH dose of 6 IU/m2/day. Twice daily injections were given as one third in the morning and two thirds at bedtime. All girls concurrently received low dose oestradiol (0.05 microgram ethinyl oestradiol/kg/day, increased to 0.10 microgram/kg/day after 2.25 years).Patients: Nineteen girls with Turner's syndrome aged > or = 11 years (mean (SD) 13.6 (1.7) years).Measurements: To determine final height gain, we assessed the difference between the attained final height and the final height predictions at the start of treatment. These final height predictions were calculated using the Bayley-Pinneau (BP) prediction method, the modified projected adult height (mPAH), the modified index of potential height (mIPHRUS), and the Turner's specific prediction method (PTSRUS).Results: The gain in final height (mean (SD)) was not significantly different between the once daily and the twice daily regimens (7.6 (2.3) v 5.1 (3.2) cm). All girls exceeded their adult height prediction (range, 1.6-12.3 cm). Thirteen of the 19 girls had a final height gain > 5.0 cm. Mean (SD) attained final height was 155.5 (5.4) cm. A "younger bone age" at baseline and a higher increase in height standard deviation score for chronological age (Dutch-Swedish-Danish references) in the first year of GH treatment predicted a higher final height gain after GH treatment.Conclusions: Division of the total daily GH dose (6 IU/m2/day) into two thirds in the evening and one third in the morning is not advantageous over the once daily GH regimen with respect to final height gain. Treatment with a GH dose of 6 IU/m2/day in combination with low dose oestrogens can result in a significant increase in adult height in girls with Turner's syndrome, even if they start GH treatment at a relatively late age. [ABSTRACT FROM AUTHOR]

Background: Osteoporosis is a major public health problem, and its prevention is of great importance. It is known that bone mass later in life is determined by the peak bone mass acquired during adolescence and the subsequent rate of bone loss. Therefore we should give special attention to children that are 'at risk' of low bone mass, and we must seek simple yet reliable methods to measure their bone mineral density (BMD) regularly. Aim: We investigated the value of a quantitative ultrasound device (QUS), the Sahara clinical bone sonometer (Hologic), in screening of low bone mass in children. In contrast to dual energy X-ray absorptiometry (DEXA), the most commonly used technique for measurement of BMD today, the QUS method is free of ionizing radiation, easy to handle and inexpensive. Subjects and methods: Intra- and inter-observer variability of the QUS method was assessed using replicate measurements by two observers in 15 randomly chosen children. QUS parameters were measured in 226 healthy schoolchildren (121 boys, 105 girls) as well as in 41 children at risk for low bone mass (15 boys, 26 girls) between 7 and 18 years old. For comparison we also determined BMD by DEXA in those children at risk. Results: Reproducibility of the QUS device was moderate, as well as the correlation between QUS and DEXA (r = 0.14-0.50). The QUS device was not able to recognize children with low bone mass as determined by DEXA. Although it is well known that BMD increases with age and pubertal stage, we could not find significant differences in QUS parameters between age and pubertal stage groups. Conclusion: We conclude that there is enough evidence that the Sahara clinical bone sonometer is not useful in screening of low bone mass in children. [ABSTRACT FROM AUTHOR]

Objective: To study the resumption of puberty and the final height achieved in children with central precocious puberty (CPP) treated with the GnRH agonist triptorelin.Patients: 31 girls and five boys with CPP who were treated with triptorelin 3.75 mg intramuscularly every four weeks. Girls were treated for a mean (SD) of 3.4 (1.0) years and were followed up for 4.0 (1.2) years after the treatment was stopped.Results: The rate of bone maturation decreased during treatment and the predicted adult height increased from 158.2 (7.4) cm to 163.9 (7.5) cm at the end of treatment (p < 0.001). When treatment was stopped bone maturation accelerated, resulting in a final height of 161.6 (7.0) cm, which was higher than the predicted adult height at the start of treatment (p < 0.001). Height at the start of treatment was the most important factor positively influencing final height (r = 0.75, p < 0.001). Bone age at cessation of treatment negatively influenced final height (r = -0.52, p = 0.03). A negative correlation between bone age and height increment after discontinuation of treatment was observed (r = -0.85, p = 0.001). Residual growth capacity was optimal when bone age on cessation of treatment was 12 to 12.5 years. Body mass index increased during treatment and remained high on cessation. At final height, the ratio of sitting height to subischial leg length was normal. Menarche occurred at 12.3 (1.1) years, and at a median (range) of 1.1 (0.4 to 2.6) years after treatment was stopped. The ovaries were normal on pelvic ultrasonography.Conclusions: Treatment of CPP with triptorelin increases final height, with normal body proportions, and seems to increase body mass index. The best results were achieved in girls who were taller at the start of treatment. Puberty was resumed after treatment, without the occurrence of polycystic ovaries. [ABSTRACT FROM AUTHOR]

Indications and results are presented for nearly all the cardiac pacemaker implantations in children in The Netherlands from 1963 through mid-1979. Five infants and 71 older children received a pacemaker for a total of 184 patient years. Two of the infants died within a week of birth; four children died after the pacemaker failed to control a sick sinus syndrome, which in three of them developed after a Mustard operation for transposition of the great arteries. A seventh death was due to a myocardial infarction soon after the implantation. In addition to the initial implantations a further 82 operations were necessary in 32 children for various reasons, intolerance to wires or to the pacing unit being the most frequent. [ABSTRACT FROM AUTHOR]

We report very large hepatic peroxisomes ( d-circle >1 Μm) in a patient with rhizomelic chondrodysplasia punctata and a patient with acyl-CoA oxidase deficiency. The effects of peroxisomal enlargement on the enzymatic activity are discussed. As increase in peroxisomal size is also reported in at least 12 other patients with peroxisomal disorders, we propose, a relationship between the enlargement of the organelles and their functional deficiency. [ABSTRACT FROM AUTHOR]