Are needle-free injections a useful alternative for growth hormone therapy in children? Safety and pharmacokinetics of growth hormone delivered by a new needle-free injection device compared to a fine gauge needle.

The clinical safety, use and pharmacokinetics of a new needle-free device for delivery of growth hormone (GH) were compared with those of conventional needle injection devices. In an open-label, randomized, 4-period crossover study, 18 healthy adults received single subcutaneous injections of Genotropin administered by the Genotropin ZipTip needle-free device and by conventional injection. Bioequivalence was established between the devices. In a separate open-label, randomized, multicenter, 2-period crossover study, pediatric patients underwent 2-weeks Genotropin treatment administered by the Genotropin ZipTip and by a fine-gauge needle device (>95% used the Genotropin Pen). In total, 128/133 patients who were treated completed the study. Genotropin ZipTip was well tolerated and >50% of patients found no difference between the devices for all parameters assessed. After study completion, >20% patients preferred to continue using Genotropin ZipTip. Although statistical analyses demonstrated superiority of the Genotropin Pen versus Genotropin ZipTip for bleeding, pain, soreness, and bruising, Genotropin ZipTip was considered to provide a safe and bioequivalent alternative to needle injection.

[Saarland Growth Study: sampling design]. / Saarländische Wachstumsstudie: Sampling Design.

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.

[Saarland Growth Study: analyses of body composition of children, aged 3 to 11 years. Measurement of height, weight, girth (abdomen, upper arm, calf) and skinfolds (triceps, biceps, subscapular,suprailiacal, abdominal) and bioelectric impedance (BIA)]. / Saarländische Wachstumsstudie: Analysen zur Körperzusammensetzung bei Kindern im Alter zwischen 3 und 11 Jahren. Messung von Körperhöhe, Körpergewicht, Umfängen (Abdomen, Oberarmen und Waden), Hautfettfalten (Trizeps, Bizeps, subskapular, suprailiakal und abdominal) und bioelektrischer Impedanzanalyse (BIA).

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.

[Saarland Growth Study: percentile charts for height, weight, and body mass index (BMI) for boys and girls, 4-18 years old, in Saarland]. / Saarländische Wachstumsstudie: Perzentilenkurven für Körperhöhe, Körpergewicht und Body Mass Index (BMI) für saarländische Jungen und Mädchen im Alter zwischen 4 und 18 Jahren.

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.

Cytochrome c oxidase deficiency and long-chain acyl coenzyme A dehydrogenase deficiency with Leigh's subacute necrotizing encephalomyelopathy.

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.

[Surgical diseases of the adrenal glands in childhood--pediatric aspects]. / Chirurgische Erkrankungen der Nebennieren imKindesalter. Pädiatrische Aspekte.

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.

[alpha-Amylase standard values in children]. / alpha-Amylase: Richtwerte bei Kindern.

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.

[The long-eyelash syndrome (trichomegaly syndrome, Oliver-McFarlane)]. / Das Syndrom der langen Wimpern ("Trichomegaliesyndrom", Oliver-McFarlane).

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.

Hypophosphatasia congenita letalis.

Hypophosphatasia is a serious enzymatic defect, where the serum alkaline phosphatase is considerably diminished or completely absent. The urine or serum contain excessive quantities of phosphoethanolamine.. The illness manifests itself with severe disorder of mineralisation in the skeletal system. We report a child with extremely severe manifestations. There was no bony cranial vault and all the extremities were shortened and thick. The alkaline phosphatase was extremely low and the secretion of phosphoethanolamine and proline was considerably increased. The differential diagnosis and the prenatal diagnostics will be mentioned in this case report.

[LH-RH nasal spray (Kryptocur), a new aspect in the hormonal treatment of undescended testes (author's transl)]. / LH-RH-Nasalspray (Kryptocur), ein neuer Aspekt in der hormonellen Behandlung des Hodenhochstandes.

New aspects in the hormonal treatment of undescended testes have been introduced with the LH-RH nasal spray. There are 188 references on boys with 232 retained testes. A four-weeks' course with LH-RH nasal spray (1.2 mg in 24 hours) resulted in complete descension in 52% of the cases (32%-87%). In 21% of the cases, the position of the testes was improved. No success was recorded in 27% of the cases only. The author's own patients material included 40 boys with 50 maldescended testes, success being recorded in 78% of the cases. Administration of LH-RH nasal spray did not produce any side effects. Today, this method can be considered as the method of first choice in the hormonal treatment of undescended testes.

[Optimal TRH-testdose in children: 1 microgram TRH/kg (i. v.) (author's transl)]. / Optimale TRH-Testdosis bei Kindern: 1 microgram TRH/kg (i. v.). Normalwerte bei euthyreoten Kindern.

In a previous study the optimal TRH test dose of 1 microgram/kg (i v.) was determined in 67 infants between the ages of 2 weeks and 2 years old. At that time the following doses were tested: 0.5 microgram, 1 microgram, 1 microgram/kg, 2 micrograms/kg and 200 micrograms. In the present study the clinical application of this low dose TRH administered to children between the ages of 25 months and 16 years will be reported. There was a significant increase in TSH in all of the 70 euthyroidic children. The 30 minute values after intravenous injection of 1 microgram TRH/kg were 11.7 +/- 4.5 microU/ml (400% increase, upper threshold value 20 microU/ml). The dose of 200 micrograms TRH resulted in a 30 min value of 13.0 +/- 6.6 microU/ml in 30 of the children (430% increase, upper threshold value 20 microU/ml). In children two years old and younger these values were significantly higher. After administering 1 micrograms TRH/kg, the value 17.0 +/- 8.5 microU TRH/ml was measured in 20 children (735--400% increase) and after 200 micrograms TRH, the value 21.0 +/- 7.5 microU/ml (690--200%). The upper threshold value is 36 microU TSH/ml. The TSH basal secretion did not depend on the age of the child. The upper threshold value of 7.5 microU TSH/ml applies to children two weeks of age and older. 1 microgram TRH/kg (i. v.) is the optimal test dose for children. The higher doses do not provide any additional clinical information.