Three-year growth hormone treatment in short children with X-linked hypophosphatemic rickets: effects on linear growth and body disproportion.

CONTEXT: Children with X-linked hypophosphatemic rickets (XLH) are prone to progressive disproportionate stunting despite oral phosphate and vitamin D treatment. OBJECTIVE: Our objective was to analyze the effects of GH treatment on stature and lengths of linear body segments in short children with XLH. DESIGN, SETTINGS, AND PATIENTS: A 3-yr randomized controlled open-label GH study in short prepubertal children with XLH (n = 16) on phosphate and calcitriol treatment was conducted. A cohort of XLH patients (n = 76) on conservative treatment served as an XLH reference population. MAIN OUTCOME MEASURES: Changes in SD scores (SDS) of stature and linear body segments, i.e. sitting height, leg and arm length, and sitting height index (i.e. ratio between sitting height and stature) were the main outcome measures. RESULTS: XLH patients presented at time of enrollment with significant impairments of stature (-3.3 SDS) and linear body segments compared with healthy children. Leg length (-3.8 SDS) was most impaired, whereas sitting height (-1.7 SDS) was best preserved. The markedly elevated mean sitting height index (+3.3 SDS) reflected severe body disproportion. GH resulted in a sustained increase in linear growth (stature, +1.1 SDS; sitting height, +1.3 SDS; leg length, +0.8 SDS; arm length, +1.1 SDS; each P < 0.05 vs. baseline), whereas no significant changes were observed in controls. Mean height SDS at 3 yr did not significantly differ between groups. Sitting height index remained stable in both the GH-treated patients and in study controls but increased further in the XLH-reference population. CONCLUSIONS: The 3-yr GH treatment improved linear growth without progression of body disproportion in short children with XLH.

Living related kidney donation as an advantage for growth of children independent of glomerular filtration rate.

Seventy-two pediatric kidney recipients of living related donors (LRD) and 145 of cadaveric donors (CAD) were analyzed for height standard deviation scores (Ht-SDS) and glomerular filtration rates (GFR) directly after transplantation and over the following 5 years. GFR was significantly higher immediately after transplantation in LRD compared with CAD recipients; however, GFR was not different during the 5-year follow-up period. Although Ht-SDS was comparable at the time of transplantation in both groups, it was significantly higher among LRD recipients over the next 5 years. Multivariate and covariate analyses showed that Ht-SDS after 5 years was mainly influenced only by CAD vs LRD and not by GFR or other factors, namely, donor age, rejections, time of dialysis, preemptive transplantation, age at transplantation, or immunosuppression. Thus, children receiving grafts from LRD showed a better catch-up growth independent of the GFR than those after CAD transplantation. We concluded that the period of donor death and prolonged cold ischemia in CAD grafts may lead to changes in gene expression of cytokines and other mediator molecules that affect bone metabolism. Better growth seems to be an additional factor supporting the policy of LRD kidney transplantation as the best option in children.

Growth in children after kidney transplantation with living related donor graft or cadaveric graft.

The extent to which growth after renal transplantation differs between children with a living related donor graft (LRD) and those with a cadaveric donor graft (CAD) is unclear. We retrospectively studied growth in the 5 years after transplantation in 30 boys who received LRD and 21 who received CAD. Height was similar in both groups after transplantation but was greater in LRD than in CAD recipients during follow-up. LRD recipients were taller at all ages, and had greater growth velocity in infancy and during puberty. Glomerular filtration rate (GFR) was higher immediately after transplantation in LRD than in CAD recipients, but did not differ between the groups during follow-up. GFR and other factors did not affect height 5 years after transplantation. These findings support use of LRD as the preferred option in children.

Analysis of cognitive and motor functioning during pubertal development: a new approach.

We investigated cognitive-motor abilities in 303 (156 female) school children from Zagreb, Croatia, in the age span 10 to 14 years using a newly developed chronometrical reactionmeter system (CRD). The following tests were applied: CRD-311 (simple visual discrimination of signal location), CRD-324 (short-term memory actualisation), CRD-21 (simple convergent visual orientation), and CRD-11 (arithmetically conceptualised/operationalised convergent thinking). In both gender a statistically significant age related improvement of the performance for time related parameters (minimum time of test item solving (MT), total ballast (TB), and total time of test solving (TT) was observed. In contrast, the number of errors (NE), which was the only non-time related parameter tested, did not significantly change with age. Significant differences between boys and girls were observed for the time related parameters TB and MT. TB was significantly lower in girls, whereas boys tended to be faster in MT measurements. In TT as a composed measure of the mentioned parameters, no major differences were observed. We conclude that the CRD system is a new useful tool for investigating the complexity of cognitive-motor abilities in children. Our cross-sectional study demonstrated that the time-related parameters were significantly affected by age and gender during puberty.

Does growth hormone therapy harmonize distorted morphology and body composition in chronic renal failure?

Recombinant human growth hormone (rhGH) therapy of growth retardation in chronic renal failure (CRF) has become well established. While there are ample data about its effectiveness in restoring longitudinal growth delay, data on complex anthropometry are scarce. Twenty-three children with CRF (6 after renal transplantation) were investigated using 24 different parameters. The analysis revealed disproportionate growth in CRF. While parameters of the trunk and transverse dimension of the head were preserved, the extremities were affected more severely. Bone dimensions of the legs and arms as well as muscle mass were affected the most. RhGH therapy was effective in restoring impaired longitudinal dimensions of the body in CRF. The restoration of growth retardation occurred predominantly in the extremities. This was accompanied by an increase in transversal dimensions, circumferences and soft tissue of the extremities, as well as an increase in bone and muscle volume, indicating harmonization of the total body shape. The dimensions of trunk, forehead diameter and morphological face height remained within the normal range. There was no evidence of acromegaly in children with CRF and rhGH therapy. We conclude that rhGH therapy at a dose of 28 iU/m2/week is effective in restoring previously disproportional body structure and shape in children with CRF.

Sonomorphologic evaluation of goiter in an iodine deficiency area in the Ivory Coast.

OBJECTIVES: This study evaluated the extent of thyroid abnormalities in a remote iodine-deficient area of the Ivory Coast. METHODS: Ultrasonography was used in detecting the presence of goiter. RESULTS: The overall prevalence rates of goiter were 64.7% among females and 53.3% among males. In children aged 6 to 15 years (n = 314), the prevalence of goiter was 62% regardless of sex. Thyroid volume increased steadily with age, with significantly larger goiters in women 25 years and older. Frequency of cysts and calcifications did not correlate with sex. CONCLUSIONS: Especially in developing countries, prophylaxis of iodine deficiency disorders must be improved in iodine-deficient areas to prevent substantial morbidity, which is more severe in women and elderly persons.

Body morphology in adolescent girls with anorexia nervosa: a short-term longitudinal study.

Changes in body morphology of anorectic girls during illness as well as during the treatment period were studied using data on 23 anthropometric traits. The sample consisted of 20 adolescent girls, aged 16.05 +/- 2.21 years. Girls with shorter duration of anorexia nervosa have larger amounts of fat and muscle tissue on the upper arm, as well as thicker subcutaneous fat tissue of the trunk, than the girls with longer duration of illness. These differences remain even after the treatment period. Growth rate of girls with longer duration of illness has been decreased. Slight changes in the latent structure of body morphology in anorectic girls at the end of hospitalisation in comparison with those at the beginning of hospitalisation were observed. The above-mentioned changes could have resulted from greater relative increase of fat than muscular tissue mass during hospitalisation and possible difference in the sequence of fat gain between the trunk and the extremities. Further studies are needed.

The aging process--an analysis of the latent structure of body morphology (in males).

The morphological characteristics (20 anthropometric variables) of a total of 2,351 examinees (from the age of 18 to 90) were analyzed by a model of the principal components of the factor analysis. Four factors were extracted that explain 71.4% of the total variance. The factors-"general body voluminosity", "subcutaneous fat tissue", "longitudinal body dimensionality" and "upper body voluminosity"-were analyzed within the context of their appearance in different age-determined cohorts. The differences between cohorts (groups per ten years of age) were studied by the canonical discriminant analysis. The first two discriminant functions (describing mostly the variability of cohorts-96.11%) indicate a constant decrease of body and sitting height, and an increase of upper body voluminosity till the fourth age cohort, which is the most crucial one in the change of latent morphological structure. Results of the correct classification of cohort members show that only 48.45% of probands were correctly placed (the best classification determined was in the age between 46 and 55 years) indicating that in males, at least three different groups exist according to the specificity of morphological aging in human organisms.