Medizin - Open Access LMU - Teil 05/22 Archiv

Androstenedione and testosterone were measured in whole adrenal glands of 56 previously healthy boys who died suddenly between birth and 2 yr of age. In each adrenal gland, the concentration of androstenedione considerably exceeded that of testosterone. The highest concentrations were found during the first week of life (median, 295 ng/g; range, 98- 320 ng/g). Thereafter, values decreased rapidly until the end of the first year of life (median, 10 ng/g; range, 4.4-22.7 ng/g). Adrenal testosterone concentrations averaged 15% of those of androstenedione in the same gland and similarly decreased until the end of the first year. The decrease of adrenal androgen concentrations paralleled the involution of the fetal adrenal zone. A close correlation existed between the concentration of androstenedione in adrenal tissue and plasma. However, no correlation existed between adrenal and plasma testosterone. When the adrenals and testes of the same infant were compared, there was 10 times more androstenedione in the adrenals than in the testes during the first 2 yr of life. The testes contained more testosterone than the adrenals only during the first 4 months. Thus, in infant boys the adrenals are the main source of androstenedione during the first 2 yr. After the sixth month of life, they also are the main source of testosterone.

The most commonly used techniques for the in vivo evaluation of the cellular immune response include intracutaneous testing with microbial recall antigens or sensitization with neoantigens. The reliability of these tests for the individual patient usually is low due to the lack of standardization and quantification. Moreover only the efferent branch of the immune response can be judged. The dinitrochlorobenzene-contact allergy time (DNCB-CAT) is a quantitative approach for the assessment of the cellular immune response. 2% DNCBointment is applied on the upper arm in a 1 cm2 area. On the following days patch-testing with 0.05% DNCB-ointment is done on the homolateral forearm in alternating localizations till an allergic contact dermatitis reaction appears. As assessed in patients with malignant melanoma (MM, n=\\5) and with lymphoproliferative disorders (LD, η = 25), the DNCB-CAT correlates with the age of the patients and can be expressed by a formula given by the age (years) χ factor (MM = 0.16; LD = 0.17) + constant figure (MM = 5.5; LD = 4.3). There was no significant difference between the two groups or subgroups investigated. By DNCB-CAT quantitative analysis of the cellular immune response in vivo is possible. It is an appropriate model for further investigations of the cellular immunity under different clinical, histological, prognostic, and therapeutic aspects.

Bei elf von 43 nichtimmunen Patienten mit Malaria tropica traten eine oder mehrere Organkomplikationen auf: zerebrale Malaria, akute respiratorische Insuffizienz, akutes Nierenversagen, Sekundärinfektion, Autoimmunhämolyse, spontane Milzruptur und akute Pankreatitis. Die Parasitämie betrug 0,1 bis 60 %. Initiale antiparasitäre Therapie mit Chinin parenteral führte in neun Fällen zu rascher Rückbildung der Parasitämie. Zusätzlich wurde ein zweites schizontozides Mittel entsprechend der Resistenzlage gegeben. Die supportive Therapie umfaßte intensivmedizinische Überwachung mit Bilanzierung von Elektrolyt- und Wasserhaushalt sowie gegebenenfalls eine frühzeitige Hämodialyse und (oder) endotracheale Intubation mit PEEP-Beatmung. In einem Fall mit exzessiver Parasitämie wurde eine Austauschtransfusion durchgeführt. Heparin wurde nur bei nachgewiesener disseminierter intravasaler Gerinnung gegeben, Corticosteroide nur bei persistierender Autoimmunhämolyse. Alle Patienten überlebten ohne zurückbleibende Defekte. Die retrospektive Analyse zeigt, daß neben einer raschen spezifischen Therapie die supportive Behandlung der einzelnen Organkomplikationen für Verlauf und Prognose der komplizierten Malaria tropica mitentscheidend ist.

The Fe/S protein of complex III is encoded by a nuclear gene, synthesized in the cytoplasm as a precursor with a 32 residue amino-terminal extension, and transported to the outer surface of the inner mitochondrial membrane. Our data suggest the following transport pathway. First, the precursor is translocated via translocation contact sites into the matrix. There, cleavage to an intermediate containing an eight residue extension occurs. The intermediate is then redirected across the inner membrane, processed to the mature subunit, and assembled into complex III. We suggest that the folding and membrane-translocation pathway in the endosymbiotic ancestor of mitochondria has been conserved during evolution of eukaryotic cells; transfer of the gene for Fe/S protein to the nucleus has led to addition of the presequence, which routes the precursor back to its “ancestral” assembly pathway.

Eine seltene Ursache der primären Nebennierenrindeninsuffizienz ist die X-chromosomal vererbte Adrenomyeloneuropathie. Neurologisch ist sie durch Zeichen der zentralen und peripheren Demyelinisierung, endokrinologisch durch einen M. Addison und eine zusätzliche primäre Hodeninsuffizienz gekennzeichnet. Bei zwei Patienten mit diesem Krankheitsbild konnte der Stoffwechseldefekt im Abbau langkettiger Fettsäuren durch Bestimmung eines erhöhten Hexakosansäure-Spiegels (C 26) im Blut gesichert werden. Bei einem Patienten (Fall 1) lag ein isolierter Ausfall der Zona fasciculata vor, beim anderen (Fall 2) fand sich eine klinisch manifeste komplette Nebennierenrindeninsuffizienz. Beide Patienten haben einen beginnenden Hypogonadismus. Die neurologische Symptomatik ging im Fall 2 der Entwicklung der endokrinen Ausfälle voraus, im Fall 1 führten Familienanamnese und Nebennierenrindeninsuffizienz zur Diagnosestellung. Beim Nachweis einer peripheren Neuropathie bei männlichen Jugendlichen oder jüngeren Erwachsenen sollte auf die Zeichen einer beginnenden Nebennierenrindeninsuffizienz geachtet werden.

In a retrospective study the reports of 211 cases of cardiogenic cerebral embolism - diagnosed on the base of neurological and cardiological findings - were analyzed in view of signs and findings of prognostic value. There were 21 patients with TIA, 39 cases of RIND and 151 patients with cerebral infarction, 60 of which showed mild and 91 severe neurological symptoms. 38 patients died during the period of hospitalization. While sex of the patients as well as vascular risk factors (hypertension, diabetes mellitus, cigarette smoking) did not influence the clinical course of the disease, patients with TIA or RIND in general were younger (about 5 years) than those with severe stroke. Prognosis of cardiogenic cerebral embolism depended to a great degree on the underlying heart disease. Cerebral embolism after myocardial infarction showed a better remission of symptoms than embolism in atrial fibrillation. In the group of valvular diseases the course of embolic strokes in mitral lesions was worse than in aortal valve disease. Prognosis was worst in endocarditis, both in view of neurological deficit and of mortality. Mostly, the cardiogenic emboli lead to infarctions of the middle cerebral artery territory (78 per cent) with a predilection for the left hemisphere. In media-syndromes the clinical course was significantly worse in patients with additional homonymous visual defect compared to incomplete infarctions. Initial disturbance of conscience reduced prognosis quoad vitam et restitutionem significantly. Of the neuroradiological findings, the detection of arterial occlusion or circulatory disturbance in angiography as well as the finding of an ischemic lesion in computed axial tomography (CAT) was correlated with a severe course of the embolic stroke. While 7 patients with hemorrhagic infarction in CAT-Scan showed no differences in the clinical course, the 14 patients with pathological cerebral spinal fluid findings in embolism had an unfavourable prognosis. The development of epileptic seizures did not influence the further course of the infarction to a significant extent. Results are compared with the current world literature.