Nephrocalcinosis in children: a retrospective survey. Members of the Arbeitsgemeinschaft für pädiatrische Nephrologie.

We carried out a retrospective survey on 152 children and adolescents with nephrocalcinosis (NC) in 22 German centers of pediatric nephrology. Etiology, clinical manifestations, growth and development, sonographic appearance of NC and renal function were analyzed. The median age at the time of diagnosis was 3.3 (range 0.1-21) years and the median duration of follow-up was 4.1 years. In 34% of children NC was associated with idiopathic hypercalciuria (IHC) and in 32% with various hereditary tubular disorders. In 9% NC was observed subsequent to prophylactic bolus administration of vitamin D in infancy. A positive family history was found in 36%. Clinical manifestations were mainly failure to thrive during the 1st year of life (46%), psychomotor/mental retardation (28%) and urinary tract infection (34%). In 14% nephrolithiasis was associated. During the follow-up the proportion of patients with the most severe degree of NC (stages 2b or 3) increased from 40% to 55% and that of hypercalciuria decreased from 79% to 52%. Body height was <2 standard deviation scores (SDS) of normal in 41% at the time of diagnosis and in 32% at the last observation; the increase in relative height was significant only for IHC. Glomerular filtration rate (GFR) and urinary concentration capacity changed only slightly with time. At the last investigation GFR was <50 ml/min/1.73 m2 in 6% and concentration capacity <800 mosmol/kg in 48% of patients. The degree of NC was negatively correlated with GFR and concentration capacity.

[Therapy of iodine deficiency goiter in adolescents with iodine or a combination of iodine and levothyroxine with special reference to lipid parameters]. / Therapie der Jodmangelstruma bei Jugendlichen mit Jodid oder einer Kombination von Jodid und Levothyroxin unter besonderer Berücksichtigung der Lipidparameter.

The are various opinions about the optimal therapy of endemic goiter in adolescents. From 106 patients (75 girls) suffering from endemic goiter 50 patients (group A) were treated with 300 micrograms iodine per day and 56 patients (group B) with 100 micrograms iodine plus 100 micrograms levothyroxine per day. Before therapy and after 172 days of therapy on average the thyroid volume, the thyroid hormones (TSH, T3, T4) and the lipidparameters (cholesterol, LDL-cholesterol, HDL-cholesterol, triglycerides) were measured. Within the period of treatment the thyroid volume (measured by ultrasound) decreased by 11.3% in group A and by 23% in group B. The iodine deficiency was corrected completely in both groups. However, investigations in both groups proved that patients undergoing combined therapy excrete the iodine administered additionally. Analogously to studies of the intrathyreoidal iodine metabolism it can be concluded that combined therapy on the basis of the dose mentioned above does either not result in an increase in the intrathyreoidal iodine contents or in a minor increase only. Iodine treatment as well as combined treatment cause a decrease in TSH-levels and an increase in T4-levels. The changes are only significant in group B. In the total group the percentage of T3-increases was reduced from 45% to 33% during treatment. The initially elevated plasma concentration of cholesterol, LDL-cholesterol and triglycerides - compared to the control group - decreased significantly in both groups during therapy. Both groups does not differ significantly with respect to the decrease in lipids. It is to be concluded that combination therapy is preferable to iodine therapy with respect to the SD-volume reduction as well as the lipid metabolism. The primary cause of endemic goiter, i.e. the intrathyreoidal iodine deficiency, can presumably not be eliminated by means of the dose of 100 micrograms iodine combined with 100 micrograms LT4.

[Euthyroid goiter in puberty--a harmless illness?]. / Euthyreote Pubertätsstruma--eine harmlose Erkrankung?

An investigation was carried out in to thyroid hormones (TSH, T3, T4) and lipid parameters (total cholesterol, LDL cholesterol, HDL cholesterol, triglyceride) in 136 adolescents (94 femals, average age 13 years). An iodine deficiency (grade II-II) with respect to the daily urine excretion per 1,73 m2 BSA was found in 75%. With few exceptions the serum levels of TSH and T4 were in the normal range. In 36% of the patients we noticed compensatory elevated T3 levels. Correlations between thyroid hormones TSH, T4, renal iodine excretion and the volume of thyroid glands were not detectable, only T3 showed a dignificant positive correlation to the thyroid gland volume. The average values of lipids in patients were found to be higher than in normals. We consider the changed lipids as a sign of a disturbed efficacy of thyroid hormones. The regional insufficient iodine supply causes goiters and to a high degree the observed hyperchole-sterolemia, too. Our results underline the necessity of a common iodine salt prophylaxis as well as the treatment of "harmless" goiters in puberty.

[Effect of organized general preventive administration of iodized salt on struma incidence and ioduria in 11-16-year-old children in the Jena endemic area]. / Einfluss der organisierten allgemeinen Iodsalzprophylaxe auf die Strumafrequenz und Iodurie bei 11-16jährigen Kindern im Jenaer Endemiegebiet.

In 1988 and in 1991, i.e. two and five years after the introduction of a general iod salt supply (32 mg KJO2/kg salt) in our region, we investigated the frequency of goiters and the level of iodine excretion in urine in 504 and 336 school children of 11 to 16 years of age. In 1988 the assessment of goiter size was made by inspection and palpation according to the recommendation of WHO. 36% of the school children were found to have goiters of type I b or II. The average urine excretion of iodine amounted to 46.2 +/- 37.2 micrograms Iod/g creatinine (n = 353). In 1991 we selected school children with goiters by the same criteria and found frequency (34%) to be nearly unchanged compared to 1988. Furthermore we investigated these goiters with ultrasound and confirmed the diagnosis in 100 from 107 children. The average daily urine excretion of iodine was significantly higher in 1991 (57.7 +/- 24.8 micrograms/Iod/g creatinine, n = 328) than in 1988. However this value is below the level recommended by the WHO (minimum 75 micrograms Iod/g creatinine). Despite the increase of the iodine intake over the last years, the frequency of goiters in pubertal school children in the Jena region is endemic. This fact supports the demand for a general and sufficient iodine supply.

[Contamination of breast milk with organochlorine compounds in comparison with cow's milk and selected milk products in the new federal districts]. / Zur Kontamination von Humanmilch mit chlororganischen Verbindungen im Vergleich zu Kuhmilch und ausgewählten Milchprodukten in den neuen Bundesländern.

400 human milk, 280 cowmilk, 56 milkpowder and 305 butter samples were examined concerning there content of chloroorganic compounds. In human milk the highest concentration had DDE, followed by PCB, DDT and HCB, the lowest values were measured for HCH. Only total DDT in human milk but no compound in the milk product samples was above the maximal tolerated limit. There is a strong reduction in comparison to the values measured in 1979 (ten times for total HCH, one half for total DDT and to one sixth for HCB) but the PCB concentration didn't change.