[The early detection of hearing disorders in infancy as a result of interdisciplinary cooperation]. / Die Früherkennung von Hörstörungen im Kleinkindalter als Ergebnis interdisziplinärer Zusammenarbeit.

The early perception of hearing loss in children is problematic yet, but to improve, if a screening is inclusive in basis tend of crèches.

Morphometric analysis of normal skeletal muscles in infancy, childhood and adolescence. An autopsy study.

The deltoid and lateral vastus muscles of 113 subjects aged from 1 week to 20 years who had suddenly died from accidents were studied at autopsy 8-48 h after death (mean 22 h). All muscles studied gave a positive histochemical reaction for ATPase after alkaline preincubation and for NADH-TR; the fact that the dehydrogenase was still active indicated that the material was reasonably well preserved. The muscles of children shortly after birth contained about 40% type 1 fibres; the percentage of these fibres increased to about 60% within the first 2 postnatal years and then remained constant. This indicates that the normal type 2 to type 1 transformation during development is not completed at birth. The mean diameter of the muscle fibres shortly after birth was 10-12 micron, and at age 15-20 years it was 40-60 micron. The standard deviation of the diameters within a sample never exceeded 20% of the mean value. The 5-fold increase in diameter corresponded to an increase in cross-sectional area by a factor of 25. The largest increase was observed in type 2 fibres of males (31-fold) which at age 15-20 years tended to be thicker than type 1 fibres while in females of that age type 2 fibres tended to be of smaller diameter than type 1 fibres. Before the age of 15, there was no difference between muscles from males and females, and type 1 fibres were usually thicker than type 2 fibres.

Myopathy with mitochondrial abnormalities and rimmed vacuoles.

A man of 44 years suffering from an exercise-induced neuromuscular disease with mitochondrial abnormalities and rimmed vacuoles is reported. The mitochondrial abnormalities and rimmed vacuoles (autophagic vacuoles) are interpreted as sequential changes of the same pathogenetic process depending on the degree of energy deficiency.

Changes in human skeletal muscles due to ageing. Histological and histochemical observations on autopsy material.

Morphological changes in human skeletal muscle with ageing are reported. Samples from the deltoid and the vastus lateralis muscles from 126 subjects, aged 20-80 years, were studied by light microscopy. The patients died suddenly due to accidents or from fatal diseases. Until their death, they had preserved normal physical activity corresponding to their age. Chronic diseases, inactivity or neuromuscular diseases which are known to lead to changes in the muscles were excluded. The frequency of neurogenic changes of muscles increased with increasing age. These results correlated with electrophysiological and morphological changes in the peripheral nerves due to ageing reported by other investigators. The neurogenic changes in persons over 70 years were overlapped by a type-2 fibre atrophy.

[Sporadic occurrence of benign myopathy with early contractures (Emery, Dreifuss and Rotthauwe)]. / Sporadisches Auftreten der benignen Myopathie mit Frühkontrakturen (Emergy-Dreifuss und Rotthauwe).

A myopathy which sporadically appeared in a young male corresponds clinically, electromyographically, histologically and cardiologically to the rare picture of the X-chromosomally recessive benign myopathy with early contractures. The coordination to the clinical picture mentioned and the genetic advice, taking into consideration the X-chromosomally recessive heredity, are discussed and proved.

[An unusual myopathy: the so-called congenital fiber-type disproportion]. / Eine seltene Myopathie: die sog. kongenitale Fasertyp-Disproportion.

It is reported on a 19-year-old female patient with a congenital myopathic syndrome with deformities of the skeleton which could be recognized myopathologically as a so-called congenital fibre type disproportion. It is referred to the importance of the modern method of histochemistry.

[The caries inhibiting action of fluoride gels in animal experiments]. / Zur Karieshemmwirkung von Fluorid-Gelen und -Gelée im Tierexperiment

In a short-term animal test, 70 rats were classified into five groups, two fluoride gel groups with and without phosphate addition, one Elmex Gelée group and one placebo gel group. Another group served as a control group. The results found in the active substance groups were statistically significant, as compared to the placebo and control groups. The question as to why the phosphate addition used inhibited caries more successfully, could not be answered.

Effects of exogenous dehydroepiandrosterone sulphate on various enzymes and on steroid metabolism in the guinea-pig.

Treatment of male guinea-pigs daily with an oral dose of 2 mg dehydroepiandrosterone (DHA) sulphate/100 g body weight for 2 weeks significantly reduced the glucose-6-phosphate dehydrogenase (G-6-PDH) activity of erythrocytes, liver, kidney and testis. Lactate dehydrogenase activity in plasma also decreased, but L-aspartate: 2-oxoglutarate aminotransferase (GOT) and L-alanine:2-oxoglutarate aminotransferase (GPT) activity in plasma remained unaffected. In liver and kidney, however, a significant rise in GOT and GPT was observed. A 2- to 3-7-fold increase of C19-steroids was observed in plasma, liver and kidney. In extracts of liver and kidney more than 60% of steroids were isolated from the sulphatide fraction. Only minor changes were detected in the metabolic pattern of C19-steroids, 17-hydroxysteroids prevailing in the free and sulphatide fractions, while 17-oxosteroids predominated in the sulphate and glucuronide fractions. A slight rise of cyclic AMP concentrations in liver and kidney tissue was attributed to the inhibition of phosphodiesterase by the DHA/G-6-PDH system

Free and sulfoconjugated dehydroepiandrosterone, cyclic adenosime-3',5'-monophosphate, and free estriol in maternal and cord blood.

When free DHEA, its sulfatide, and sulfate were assayed in maternal plasma as well as in umbilical cord arterial and venous plasma, rather high concentrations were found in either fraction from cord arterial plasma, reflecting the fetal contribution not only of free DHEA and DHEA sulfate, but also of the lipophile steroid sulfatide. Since high DHEA levels were associated with elevated c-AMP concentrations, a certain interrelationship of both parameters is indicated. In the course of delivery, a rapid decrease of free estriol in maternal plasma was observed. Higher concentration of free estriol in umbilical venous plasma pointed at its placental biosynthesis from fetal precursors.

Interaction between dehydroepiandrosterone, glucose-6-phosphate dehydrogenase, and cyclic adenosine-3',5'-monophosphate in neoplastic and normal human mammary tissue.

When total DHEA, G-6-PDH activity, and c-AMP were determined in human neoplastic mammary tissue and corresponding normal tissue the G-6-PDH activity in the former tissue greatly exceeded that found in normal tissue. On the other hand, a remarkable decrease of total DHEA and c-AMP could be detected in cancer tissue, hinting at the participation of DHEA in the intracellular regulation of G-6-PDH and c-AMP levels.

[Family studies for detection of latent psoriatics by assay of alterations in steroid metabolism (author's transl)]. / Familienuntersuchungen zur Erfassung latenter Psoriatiker aufgrund von Veränderungen im Steroidstoffwechsel

In psoriasis changes of the DHEA metabolism could be demonstrated. These genetically determined basic alterations may represent an important factor for the manifestation of psoriasis. They consist of a decreased penetration of DHEA through the cell membrane and an increased reduction of DHEA to ADIOL, leading to a reduced DHEA/ADIOL ratio. In the present investigations the question was whether the assay of these parameters may aid in the detection of latent psoriasis. For this purpose 31 members form 6 unselected families of psoriatics (8 manifested psoriatics, 18 potential psoriatics, 5 clinically and anamnestically healthy family members by marriage) as well as 6 further controls were examined. It could be shown that those changes in the DHEA metabolism, which are evident in manifested psoriatics also occur at least in part of potential psoriatics. However, both parameters investigated do not always behave the same way. Hence, from the present data a satisfactory diagnosis of latent psoriasis does not seem possible. Still, the estimation of the above parameters may aid in the attempts to define latent psoriasis. Further investigations of such families are warranted for final elucidation of this problem.