Development of height and weight in children with diabetes mellitus: report on two prospective multicentre studies, one cross-sectional, one longitudinal.

Optimal regimen for insulin therapy should lead to normal longitudinal growth and weight gain in children with diabetes mellitus. However, reports published so far indicate that this goal of paediatric diabetology is currently not achieved in a considerable number of patients. In a cross-sectional sample of 89 children with insulin dependent diabetes mellitus (IDDM) for more than 3 years, we found the relation of height to weight to be significantly different compared to 102 healthy school children of similar age. Using bivariate analysis, body shape in these children with diabetes was shifted towards small and obese (P less than 0.05) compared to control children. We subsequently initiated a longitudinal study and followed children from the onset of diabetes for the following 3 years, recording height, weight and bone age as well as glycosylated haemoglobin and daily insulin requirement. At diagnosis, height SDS was identical in children with IDDM (+0.04 +/- 0.10) compared to control children (-0.07 +/- 0.10; M +/- SE), while weight SDS was -0.26 +/- 0.10 in children with diabetes (controls: + 0.01 +/- 0.01). Bone age was identically retarded in newly diagnosed IDDM children (-0.73 +/- 0.12 SDS) and in our control group of children from the same regional background (-0.50 +/- 0.12; n.s.). In this group of children with diabetes mellitus followed prospectively, height to weight relationship differed from controls after 2 and after 3 years of the disease (P less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

[Hydrogen (H2) breath test following lactose loading in children with recurrent abdominal pain]. / Wasserstoff (H2)-Atemtest nach Laktosebelastung bei Kindern mit rezidivierenden Bauchschmerzen.

To define the practical diagnostic value of H2 breath testing after lactose load in children with chronic abdominal pain, we measured end-expiratory H2 every 30 minutes for 2 hours after peroral lactose (2 g/kg body weight, Lactoscreen). H2 testing was done in 62 children (28 female, 32 male), aged between 2 and 16 years. Abdominal pain had been observed in these children for more than 2 months. Causes of abdominal pain other than lactose intolerance had been excluded. In 2 out of 62 children, H2 testing was impossible for lack of cooperation. 17 out of the remaining 60 children showed a positive test result (28.3%). In 15 patients, the rise in H2 exceeded 40 ppm, and was thus easily distinguished from a nonspecific rise (less than 25 ppm). Stool pH and Clini-test results were not correlating well with H2 findings, In 16 out of 17 children with positive H2 tests, abdominal pain had ceased on a diet free of lactose after a month. After 6 months 15 out of 17 children were symptom free. These data confirm early indication of non-invasive H2 testing in children with chronic abdominal pain.

[Urine glucose testing in children with diabetes mellitus--obsolete as a daily control measure?]. / Uringlucosediagnostik bei Kindern mit Diabetes mellitus--als tägliche Kontrollmassnahme veraltet?

Sixty-four children with type 1 diabetes (33 girls and 31 boys with a mean age of 11 1/4 years) were studied in order to ascertain whether urine sugar measurements performed by the patients or their parents can provide adequate monitoring of treatment. The results of two home-testing methods for urine glucose were compared with subsequent glucose dehydrogenase assays in the laboratory. Over the range from 0 to 5 g/dl the results of home testing disagreed with the laboratory checks by an average of one concentration grade, and displayed wide scatter. Only in the concentration range of 0.5 g/dl were there differences between the nonspecific reduction test (Clinitest) and the specific enzymatic assay. Glucose concentrations in urine specimens passed at 7 am, 12 noon and 6 pm (home testing) were compared with blood glucose concentrations checked at the same times. Correlation was not very close (correlation coefficients between 0.3 and 0.64). Correlation between pooled urine collections and 24 hour blood sugar profiles was equally poor (0.5-0.6). The author concludes that urine sugar testing is unsatisfactory for home monitoring of children with diabetes and the results are inadequate for diagnostic or therapeutic purposes.

[Juvenile form of tyrosinemia type I]. / Juvenile Form der Tyrosinämie Typ I.

A 1-3/12-year-old Turkish boy born of consanguineous parents was hospitalized in poor general condition with disorientation, hepatosplenomegaly, and rickets. Laboratory tests showed pronounced symptoms of hepatic dysfunction, rickets, and Fanconi's syndrome with acidosis. The diagnosis juvenile type I tyrosinemia was based on the anamnesis, hepatorenal symptoms, and elevated tyrosine and methionine blood levels as well as the pathognomic findings of heavy succinylacetonuria and absent fumarylacetoacetase activity in the fibroblasts. Etiology, pathobiochemistry, clinical symptoms, differential diagnosis, and therapy of this rare autosomal-recessive inherited metabolic disease were discussed.

[Intensification of insulin substitution in adolescents and children with type I diabetes mellitus]. / Intensivierung der Insulinsubstitution bei Jugendlichen und Kindern mit Typ-I-Diabetes mellitus.

In order to approach the therapeutic goal of normoglycemia in diabetic children and adolescents, new forms of insulin substitution have been developed and subsequently tested as "intensified conventional insulin therapy" or "multiple daily insulin injections". Typical for this therapy are unmodified insulin injections before each main meal, administration of basal delayed insulin at night and if necessary also during the day, regular daily blood sugar analyses as an aid to decisions regarding therapy, and variation of the unmodified doses according to certain general or individual rules. The article reviews the principles of this therapy, indications for it and the method employed, as well as the authors' own results and those published in the literature. It concludes with a discussion of complications in therapy, of controversial aspects of practical management and of the long-term perspectives.

[Biotinidase deficiency: a congenital metabolic disease which can be successfully treatment with vitamin H]. / Biotinidasemangel: eine angeborene Stoffwechselerkrankung, die mit Vitamin H erfolgreich behandelt werden kann.

At the age of 13 months a patient developed muscular hypotonia, deafness of the inner ear and cutaneous symptoms (alopecia; skin rash, complicated by superinfection with monilia). Biochemical assays revealed compensated metabolic acidosis, pathologically high lactate and pyruvate concentrations in the blood and cerebro-spinal fluid, as well as increased urinary excretion of 3-OH-isovaleric acid, 3-methylcrotonylglycine and lactate. The patient was diagnosed as suffering from autosomal recessive biotinidase deficiency on the basis of severely reduced biotinidase activity in plasma (0.05 nmol/min/ml). In both his parents and brother heterozygosity was found. Institution of therapy with a daily dose of 10 mg biotin rapidly removed most of the symptoms; after six months of treatment the deafness had improved significantly.

[Discitis in children and adolescents]. / Discitis bei Kindern und Adoleszenten.

This study reported on the anamnesis, clinical and instrumental findings as well as therapy in a girl with discitis. The described diagnostic problems and course are characteristic of this frequently unrecognized disease, the cause of which has not yet been fully clarified. Diagnostic and therapeutic procedures suggested in the literature were critically evaluated.

Familial occurrence of dermatomyositis and progressive scleroderma after injection of a local anaesthetic for dental treatment.

In an 11-year-old boy severe dermatomyositis occurred a few days after injection in the jaw of a local anaesthetic of the amide type for tooth extraction. The patient's grandmother was affected by progressive scleroderma at the age of 34 years, also after injection of a local anaesthetic for dental treatment. Both cases are discussed with special reference to the immuno-genetic aspects of the origin of dermatomyositis.

Alcohol embryopathy and diabetic fetopathy in the same newborn.

Both alcohol embryopathy and diabetic fetopathy were observed in the same female child. The mother was known to be alcoholic as well as diabetic. At birth the signs of diabetic fetopathy predominated: the child showed edematous subcutaneous fat, birth weight was 3650 g. The heart was enlarged. The patient's blood sugar levels ranged from 0 to 1.4 mMol/1 (0-25 mg/dl). Features of alcohol embryopathy were typical craniofacial dysmorphy, hypotonia of muscles and hyperexcitability. Later on the features of alcohol embryopathy predominated: the child became dystrophic with pronounced microcephaly, and the craniofacial dysmorphy clearly resembled other patients with alcohol embryopathy. This observation is in favour of the hypothesis, that alcohol induces cell hypoplasia in the embryo resulting in postnatal growth retardation. Maternal and consequently embryonic and fetal hyperglycemia induced cell hypertrophy in the embryo and fetus, which compensated the effect of alcohol on birth weight in our patient.

[Alteration of TSH secretion in children with goitre after short-term treatment with potassium iodide (author's transl)]. / Veränderungen der TSH-Sekretion bei Kindern mit Strumen nach Kurzzeitbehandlung mit Kaliumjodid.

300 microgram of potassium iodide daily were administered over a period of two weeks to 12 children with goitre. Basal TSH concentrations, maximal TSH increase after TRH and integrated TSH secretion after TRH were significantly decreased in this group whereas the increase of total thyroxine (T4-RIA) did not reach significance. It appears that in the development of iodine deficiency goitre in children a sensitive feed-back system is predominant. Early administration of iodine or even better general iodinated salt prophylaxis seem to be the pathogenetically correct approach in solving the common goitre problem.

[Cogenital goiter in a premature infant after amniography (author's transl)]. / Kongenitale Struma bei einem Frühgeborenen nach Amniographie.

A premature infant was born with goiter and hypothyroidism 3 weeks after amniography. The correlation between excessive iodide exposure and thyroid function of discussed. The danger of using of idodine containing contrast media for amniography are emphasized.

[Two cases of cold hemagglutinin syndrome in infants (author's transl)]. / Zwei Fälle von Kältehämagglutinin-Syndrom im Säuglingsalter.

Two cases of acute, short-term cold agglutinin disease in infants were presented. This rare disease must be considered in infants when the BSR is distinctly elevated without any corresponding clinical symptoms.