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In neonates, infants, children and adolescents serum concentrations of the thyroid hormones show a clear age dependency. Currently, age-dependent thyroid hormone reference ranges for the new ADVIA Centaur immunoanalyzer are not available. Thyroid-stimulating hormone (TSH), free thyroxine (FT4), total thyroxine (T4), free triiodothyronine (FT3) and total triiodothyronine (T3) were determined in the sera of 460 healthy children aged 0 daysto 18 years from an inland (n=308) and a seaside city (n=152) using the Bayer ADVIA Centaur analyzer. Square root functions defining the upper and lower limit of the continuous age-dependent reference range and the median line for each thyroid hormone were estimated applying a parametric regression technique. Continuous age-dependent reference ranges show a better fit to the obtained data than discontinuous reference ranges. The median TSH and FT4 concentrations decrease within the first year of life and are nearly constant until 18 years of age. The median T4 decreases within the entire age interval, whereas the median FF3 is constant. The median T3 increases within the first year of life and decreases afterwards. The calculated continuous reference ranges for T4, T3 and partly FT4 are consistent with published reference ranges. However, the reference ranges obtained for FT3 and partly TSH differed considerably, compared to published reference ranges. No significant residence- or sex-specific effects on age-adjusted hormone concentrations were found except for a slight residence-specific effect on age-adjusted FT4, which was probably due to assay imprecision. Due to the lack of international standardization, the assay-specific evaluation of reference ranges is very important. Continuous age-dependent reference ranges comply better with the biological reality and are more reliable than discontinuous reference ranges.

In a cross-sectional study dating from april 94 to march 96 we have investigated length and weight of 2610 girls and 2865 boys (age 4-18 years) at nursery schools and schools in Saarland. Thus we could provide first normative data for this region of Germany. Compared with data that have been collected between 1968 and 1989 at Dortmund, Bonn, Hamm and Jena, we found an increase in height of 2 cm for girls and boys aged 15-18 years. Apart from this finding, no further differences have been observed. Compared with international data, 18-year-old females and males in Saarland show large body heights: their average heights were 169 and 182 cm, respectively. Unfortunately, body weight of children of the Saarland is high, too. Children in Saarland were on average 1.5-6.6 kg heavier than those of the Dortmund study or other international studies. Studies have revealed that, according to the ECOG criteria, people of the Saarland are more obese than in other European countries. As a consequence of our study we request an institution for auxology to be founded which enables immediate reaction. This not only implies medical view points but also practical aspects of daily life (e.g. height of chairs or tables at schools). It is important to note that no changes have been made so far. Standards for height and weight should be updated every ten years. This should be a collaborative task between public health services and pediatric endocrinologists.

The use of reference data to evaluate the physical development of children and adolescents is part of the daily routine in the paediatric ambulance. The construction of such reference data is based on the collection of extensive reference data. There are different kinds of reference data: cross sectional references, which are based on data collected from a big representative cross-sectional sample of the population, longitudinal references, which are based on follow-up surveys of usually smaller samples of individuals from birth to maturity, and mixed longitudinal references, which are a combination of longitudinal and cross-sectional reference data. The advantages and disadvantages of the different methods of data collection and the resulting reference data are discussed. The Saarland Growth Study was conducted for several reasons: growth processes are subject to secular changes, there are no specific reference data for children and adolescents from this part of the country and the growth charts in use in the paediatric praxis are possibly not appropriate any more. Therefore, the Saarland Growth Study served two purposes a) to create actual regional reference data and b) to create a database for future studies on secular trends in growth processes of children and adolescents from Saarland. The present contribution focusses on general remarks on the sampling design of (cross-sectional) growth surveys and its inferences for the design of the present study.

This study aimed to set up current reference charts of anthropometric data in the Saarland. Only national and international data were available to be compared but no former Saarland charts could be found. In the period between 1994 and 1995 we investigated children of 3 to 11 years in a cross-sectional study. Therefore we measured body height, weight, circumferences, skinfolds and bioelectrical impedance (BIA). No significant gender differences were found for body height and weight. Boys of all groups of age showed bigger abdominal circumferences than girls of the same age. On the other hand upper-arm and calf-girth of younger girls were larger than that from boys. In higher age groups circumferences become rather equal. The skinfolds of Saarland girls are thicker than those of boys. The urban rural comparison indicated no significant differences. Nor was any social divergence found among the aforementioned parameters. Regarding height Saarland children are seen to be similar or somewhat shorter than those examined in national or international studies. By the way, in higher percentiles the children in our study were heavier. Thus high BMI values of our study are bigger compared with former studies. According to the definition of obesity by the ECOG almost 20 to 30% of our children are obese. The older children become the higher is the percentage of obesity. Comparing girls and boys, bioelectrical impedance shows higher values for girls. In higher age classes resistance levels gets smaller, in boys more so than in girls. Body fat estimated by a formula based on BIA test parameters yielded negative values. So we propose the use of sex- and age-specific raw charts of BIA test parameters.

A female infant was seen at the age of 2 months because of hypotonia, delayed motor development, and lactic acidosis, and she died at age 13 months due to respiratory failure. In a muscle specimen taken at 11 months and in a liver specimen obtained 1.5 hours postmortem, we found decreased activities of cytochrome c oxidase and long-chain acyl coenzyme A dehydrogenase. Neuropathological changes were typical for Leigh's subacute necrotizing encephalomyelopathy. To our knowledge, this is the first report of a combined defect of complex IV of the respiratory chain and of the long-chain specific acyl coenzyme A dehydrogenase of beta-oxidation in muscle and liver.

In a group of 216 healthy children (age: 3 days-16 years) normal values of alpha-amylase in serum were found out (substrate: p-nitrophenyl-alpha, D-maltoheptaosid). Infants (age: 3-353 days) mostly showed lower catalytic concentrations than older children: alpha-amylase (25 degrees C) up to 30 U/L; alpha-amylase 37 degrees C): up to 60 U/L. But, the collective was too small (n = 30) to give any statistical statement, on the other hand biological and analytical deviations of catalytic concentrations were big. In children (n = 186; age: 1-16 years) the values were not dependent on sex or age. As normal range we propose for children aged 1-16 years alpha-amylase (25 degrees C): 20-110 U/L; alpha-amylase (37 degrees C): 40-200 U/L.

Two children with abnormally long eyelashes and bushy brows ("trichomegaly") present at birth and associated with pigmentary degeneration of the retina and general growth retardation are described. This combination of findings has been observed previously in three cases. Mental retardation, sparse scalp hair, endocrinologic deficiencies and (in one of our cases) koilonychia may be found in addition, but not necessarily.

Detailed discussion of diseases of the adrenals in children where surgery may be indicated, seen from the paediatric point of view. Following differentiation between adrenal insufficiency and adrenal hyperfunction, as well as adrenal haemorrhage--where differential diagnosis is often rather difficult--the tumours of the zona glomerulosa, fasciculata and reticularis as well as of the adrenal medulla are presented and their signs and symptoms, their clinical hormonal diagnosis, localisation diagnosis and therapy are described.