504 overweight children admitted to hospital between 1921 and 1947 were followed up for 40 years by questionnaires at 10 year intervals. The mean weight for height (W/H) standard deviation score (SDS) reached a maximum in puberty (+3.5). The SDS fell to about +1 in adulthood. 47% patients were still obese (SDS greater than +1) in adulthood; 84.6% of these had SDS more than +2 in childhood. The degree of obesity in the family (parents and grandparents) and the degree of overweight in puberty were the most important factors for weight level in adulthood. Even when their food intake was in accordance with recommended levels, obese children had higher than normal weight as adults. Excessive overweight in puberty (SDS greater than +3) was associated with higher than expected morbidity and mortality in adult life. Weight-reducing measures should be started early in life to improve the unfavourable long-term prognosis for very obese children.
Over a 10-year period, from 1984-1995, in the Norwegian county of Vest-Agder, five patients in a paediatric clinic were diagnosed as having chromosome constitution 47,XYY. There are 1,250 males born a year in Vest-Agder. The patients were identified with bias, and not in a routine or prospective screening programme. All patients except one, a child who was diagnosed by chance at the age of one week; were admitted because of moderate conduct disorders or problems at school and striking tallness of stature. The half-brother of one of the 47,XYY boys had Klinefelter's syndrome 47,XYY. We conclude that identification of 47,XYY syndrome and information about it were of significance and help in counselling the patients and their families.
To construct new Danish growth charts for 0- to 20-year-olds and to compare them with Danish references from 1982 and with World Health Organization (WHO) standards for children aged 0-5 years from 2006, by applying similar inclusion and exclusion criteria.
Anthropometric data from three contemporary Danish population-based studies were combined. References for height were based on healthy Caucasian children born at term. A total of 12,671 height measurements (8055 in boys and 4616 in girls) were included. Reference charts were developed using the generalised additive models for location, scale and shape.
From prepubertal ages, a secular increase in height was observed for both genders. The differences were most pronounced in puberty, and final heights were increased by 1.4 cm in boys and 2.9 cm in girls compared to 1982 references. In boys, but not girls an upward shift in body mass index (BMI) above median levels was found. Reference curves for height were superimposable with standard curves based on the selective WHO criteria. Danish children were longer/taller and heavier and they had larger head circumferences than those reported in the recent multiethnic WHO standards.
We recommend national implementation of these contemporary 2014 Danish references for anthropometric measurements.
We present a 9.1-year-old girl of Calabrian (Italy) ancestry, with clinical features (cranio-facial dysmorphism, short stature with delayed bone age and speech delay) suggesting the diagnosis of Floating-Harbor syndrome (FHS). Physical examination showed: height 113.9 cm (-2.9 SD), with a parent's target of 156.2 cm (+1.0 SD), weight 20.7 kg, BMI 16.0 (-0.04 SD), and many phenotypic abnormalities: long eyelashes, large bulbous nose with broad nasal bridge, short philtrum, moderately broad mouth, tooth folding and malocclusion, posteriorly rotated ears, low posterior hair line, short neck, clinodactyly of the 5th finger and hyperextensible finger joints. Diffused hyperpigmentation and hypertrichosis with sporadic pubic terminal hairs, but neither clitoromegaly nor other signs of hyperandrogenism and/or precocious puberty, were observed (T1, P1). Carpal bone evaluation showed a delayed bone age (TW2: 5-5/10, - 3.6 yr) and the statural age/bone age ratio was 1.1. Other dysmorphic syndromes were excluded on the basis of clinical evidence, also evaluated by a computer-assisted search (P.O.S.S.U.M. version 3.5, 1992). Analysis of chromosome 22 by the FISH method, using specific probes Cos29 and Tuple1, excluded microdeletions in the region 22q11.2, typical of Velo-cardio-facial syndrome. In this case, we report the impairment of serum GH responsiveness (GH baseline values: 0.2-1.9 ng/ml) to the administration of oral 150 microg clonidine [peak 4.7 ng/ml, normal values (nv)>10 ng/ml] and oral 4 mg dexamethasone (8.1 ng/ml, nv>10 ng/ml). Moreover, the evaluation of spontaneous 24-h GH secretion (Carmeda AB, Stockholm, Sweden) showed low mean GH levels (1.75 ng/ml, nv>3.0 ng/ml), with a maximum sleep-related peak of 2.8 ng/ml. Serum IGF-1 values were in the low-normal range (80-176 ng/ml, nv 133-626 ng/ml). While in FHS the cranio-facial features minimize with advancement of age, the impairment of growth velocity is permanent and results in severe dwarfism. In our case, treatment with recombinant GH (0.10 U/kg/day), administered by a needle-free device, induced a dramatic increase of growth velocity, increasing the height from -2.8 to -1.9 SD after 18 months, thus indirectly confirming a role of GH deficiency in the pathogenesis of FHS dwarfism.
The influence of acculturation to a sedentary lifestyle upon the growth and development of lung volumes has been studied in Inuit children aged 9-19 years. Surveys were conducted in the circumpolar community of Igloolik (69 degrees 40'N, 81 degrees W) in 1969/70, 1979/80 and 1989/90. Over this period, the children showed little change of height or body mass at any given age, but a progressive loss of what initially had been a high level of health-related fitness. The sample for each survey comprised about 70% of children in the chosen age range: in the most recent study 87 males and 65 females. Respiratory data included forced vital capacity, one-second forced expiratory volume, maximal mid-expiratory flow rate (second and third surveys only), smoking habits and respiratory health. In each of the 3 surveys, many of the older children in the community were regular smokers. The average cigarette consumption currently rises progressively to 13 +/- 8 cigarettes/day in 87% of males and 11 +/- 7 cigarettes/day in 95% of females over 17 years of age. Nevertheless, lung volumes show the anticipated increase as a logarithmic function of stature. Furthermore, statistically fitted curves show only minor inter-survey differences in volumes for a given standing height. We thus conclude that the deterioration in other aspects of health-related fitness has not yet influenced the growth and development of respiratory function within this Inuit population.