电镜检查对小儿遗传性进行性肾炎诊断的意义(附8例报告)

目的：评价电镜检查对小儿遗传性进行性肾炎的诊断意义。方法：对８例遗传性进行性肾炎患儿进行活检组织电镜结合临床表现及随访观察，结果：该病在小儿时期常表现为复发性血尿，很少有眼和耳异常及肾功能不全。部分患儿可无家族史，肾活检组织电镜检查肾小球基底膜（ＧＢＭ），变薄或增厚和分层，结论：电镜检查对遗传性进行性肾炎具有确诊意义。     

Study of light-, electron- and immunofluorescence microscopy of urinary sediment in amyloidosis

Summary Urinary sediment of 11 patients with amyloidosis and 12 without (with proteinuria or in good health) have been studied by different morphological techniques.By light microscopy, an amyloid-related substance was occasionally demonstrated both in patients with amyloidosis and in control subjects. Immunofluorescence (IF) showed substance A (amyloid component) to be present in some cases of amyloidosis and in controls. On electron-microscopy, fibrils with characteristic appearance of amyloid substance were found in some cases of amyloidosis (4 out of 11), but were also found in controls.It therefore seems difficult to establish the diagnosis of amyloidosis by microscopic studies of the urinary sediment.Attachée de recherche INSERM     

In Vitro Microscopic Study of Fungus Cells Treated with Econazole:* Mikroskopische in vitro-Untersuchung an Pilzzellen,die mit Econazol behandelt wurden

Summary: The influence of econazole at concentrations of 0.01%, 0.1% and 1.0% on Candida albicans cell was studied by electron microscopy. The various effects of the drug were evaluated quantitatively in order to obtain unbiased results. Changes could be observed already after six hours of incubation. These consist in the development of invaginations of the cytoplasmic membrane, of lomasomes and of mesosomes and vacuoles. Mesosomes and lomasomes form a tubular system which is apt to destroy the drug by hydrolytic enzymes as indicated by the presence of acid phosphatase activity. Zusammenfassung: Der Einfluß von Econazol in Konzentrationen von 0,01%, 0,1% und 1,0% auf Candida albicans-Zellen wurde elektronenmikroskopisch untersucht. Die verschiedenartigen Auswirkungen des Medikamentes wurden quantitativ erfaßt, um möglichst objektive Ergebnisse zu erhalten. Strukturelle Veränderungen konnten bereits nach 6-stündiger Inkubation beobachtet werden. Hierbei war das Auftreten von Invaginationen der cytoplasmatischen Membran sowie von Lomasomen und Mesosomen zu beobachten. Bei höheren Konzentrationen treten große einzelne Mesosomen und Vacuolen auf. Mesosomen und Lomasomen bilden ein tubuläres System, in dem das Medikament durch hydrolytische Enzyme zerstört werden kann, wie die Gegenwart von saurer Phosphatase anzeigt.     

Die Viskoelastizität parallelsträngigen Bindegewebes und ihre Bedeutung für die Funktion

Zusammenfassung Eine unter Zugbelastung begrenzte Parallelverschieblichkeit sich überlappender Untereinheiten (Fibrillen und Subfibrillen) wird als ein wesentliches Merkmal des Ordnungsprinzips kollagener Fasern angegeben.Das viskoelastische Verhalten von menschlichem und tierischem Sehnenkollagen wird anhand kombinierter mechanischer, röntgenographischer und morphologischer Meßdaten beschrieben und mit dem Ordnungsprinzip der Fasern korreliert.Das Vorliegen parallel verschieblicher Untereinheiten wird durch Retardations- und Relaxationsversuche in Kombination mit Kurzzeitbeugungsmessungen unter Einsatz der Synchrotronstrahlung bewiesen und mit der dämpfenden Eigenschaft der Sehnenfasern in Zusammenhang gebracht.Für die elastische Eigenschaft der Sehnenfasern wird die Rückstellkraft deformierter helikaler und nichthelikaler Molekülketten verantwortlich gemacht.Das Längenwachstum von Sehnenfasern wird mit dem Gleitvermögen von Untereinheiten korreliert.Der auf eine Verbesserung der Zueinanderausrichtung potentieller Bindungspartner beruhende Konditionierungseffekt einer zyklischen Faserbelastung ist an weniger gut formierten Einheiten besonders auffällig und entsprechend an jugendlichen Sehnenfasern größer als an alten.Die stoßartige Belastung einer Sehnenfaser aus der Ruhephase führt zu größeren molekularen Deformationen als nachfolgende gleichhohe Belastungen und kann fibrilläre Gefügestörungen hervorrufen.Es wird eine Modellkonstruktion zur Simulation mechanischer Eigenschaften von Kollagen beschrieben und für einige Beispiele durchgerechnet.

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Functional properties of parallel fibred connective tissue with special regard to viscoelasticity

Summary A limited glide of overlapping subunits of collagen fibres under stretching forces represents a characteristic feature of the system. The viscoelastic behaviour of human and animal tendon collagen is described simultaneously by mechanical, morphological and X-ray data and correlated with the structural principle of the fibres.The existence of gliding subunits is proven by retardation- and relaxation-experiments in combination with short-time-x-ray-measurements by synchrotron-radiation. This behaviour may explain the damping properties of tendons. The retractive force of deformed helical and nonhelical molecular chains is generally thought to be responsible for the elasticity of the system.Length-increase of tendons during growth may be also correlated with the gliding processes.The so called conditioning-effect is due to an improved arrangement of possible reacting groups by cyclic stretch which can be well demonstrated on low ordered samples and is therefore greater in the case of juvenile specimens than in older ones.A quick first stretch causes greater molecular deformations than further stretches of the same strength, and may lead to disordered domains in the structure.A mathematical model for the simulation of the mechanical properties is given and was used for the calculation of some examples.

Frau B. Noetzel und Frau U. Pretzsch danken wir für ihre Mitarbeit, Frau K. Bienert für die Ausführung photographischer Arbeiten. Mit Unterstützung durch die Deutsche Forschungsgemeinschaft (Ne 102/10) und das Bundesministerium für Forschung und Technologie.     

Ultrastructural evidence for the development of adrenal medullary grafts in the brain

Summary This study shows that mouse mature chromaffin cells can elaborate neurite-like fibers and became integrated with the host brain. A piece of adrenal medulla, with or without attached adrenal cortical tissue, was implanted into the subarachnoid space or the hippocampal formation and examined using the electron microscopy. One week after transplantation, chromaffin cells could be observed surrounded by a basal lamina, containing many densecored vesicles 100–280 nm in diameter, including synaptic-like vesicles, which tended to gather in the cytoplasmic area or processes. The cells were irregularly shaped and bore cytoplasmic processes which sometimes ended with thick growth cone-like structures. The Golgi complex seemed to be well developed, suggesting the synthesis of new storage vesicles. One month after transplantation, the vast majority of chromaffin cells showed the noradrenaline phenotype typical of noradrenaline-storing cells in the normal gland, irrespective of graft components used or implantation sites. Some cells, presumably corresponding to the adrenaline phenotype, had secretory vesicles (140–210 nm in diameter) with denser cores than in the adrenaline-storing cells of normal gland. In the subarachnoid space, both types of graft had mostly cuboid chromaffin cells which bore a few, short, blung cytoplasmic processes. In the intracerebral transplants, the chromaffin cells of cortex-free adrenal medullary grafts developed processes having the characteristics of neurites extending from the chromaffin cells, in contrast to their counterparts with attached adrenocortical tissue. Thin sections through both types of graft showed isolated nerve cells, morphologically similar to sympathetic neurons, in the neighbourhood of the chromaffin cells. Reinnervation of the chromaffin cells was frequently observed in cortex-free implants. The integration of these grafts in the host brain is strongly suggested.     

Ultrastructural demonstration of abnormal multiaxonal Schwann-cell units in Hirschsprung's disease

The pathophysiology of Hirschsprung's disease is not fully understood. Using light microscopy we have previously demonstrated the absence of a unique Schwann-cell antigen in the circular muscle of aganglionic colon identified by D₇ monoclonal antibody. In an attempt to characterise the morphological changes in neuronal cells at subcellular level, we studied innervation patterns in normal and aganglionic colon by electron microscopy. The most striking observation on ultrastructural serial examination of the entire resected specimen of colon from patients with Hirschsprung's disease was the presence of grossly swollen monoaxonal or oligoaxonal Schwann cell units with loss of cellular contents in the circular muscle of aganglionic colon. The extent of subcellular changes in Schwann cells and axons corresponded with a diminution of immunoreactivity with a panel of neuronal cell antibodies. These ultrastructural findings suggest that degenerative changes in Schwann cells and axons within the circular muscle coat of aganglionic segment may be a significant factor in the pathogenesis of Hirschsprung's disease. Offprint requests to: P. Puri     

The mild form of pseudoachondroplasia

A 9-year-old boy from an incestuous union presented with mild clinical and roentgenological manifestations of pseudoachondroplasia. Tibial growth cartilage was obtained during a routine orthopedic procedure. Microscopic, histochemical and biochemical analysis showed changes identical to those of 6 sporadic cases of typical pseudoachondroplasia. The results support a concept of pathogenetic homogeneity of pseudoachondroplasia.Dedicated to Prof. Dr. H.-R. Wiedemann on the occasion of his 65th birthday     

Tumour of Wagner-Meissner touch corpuscles*

Summary Two benign tumours composed mainly or exclusively of Wagner-Meissner corpuscles are described. In the first case the touch corpuscles are composed of closely piled laminar cells and surrounded by argyrophilic fibres. In the second case some Schwann cells are observed in between the tactile corpuscles. The light microscopic, electron-microscopic and immunohistochemical results demonstrate that these corpuscles are comparable with the tactile end organs of the skin. Immunohistochemically, neuron-specific enolase, vimentin and protein S-100 could be demonstrated in the tactile corpuscles. Neural processes present in normal Meissner corpuscles are absent and immunohistochemically no nerve fibres or nerve endings can be demonstrated using antibodies to neurofilaments as they are observed in normal touch corpuscles of the skin.Tumours which consist mainly of multiple touch corpuscles have not been described in the literature. It is suggested to call these tumours Wagner-Meissner neurilemmoma.Dedicated to Professor Dr. Dr. h.c. K. Lennert in honor of his 65th birthday     

Elektronenmikroskopische Befunde während hypothermer Lagerungskonservierung der Leber

Zusammenfassung Schweinelebern wurden durch einfache hypotherme Lagerung mit Plasma-Protein-Fraktion 5% (PPF), Albumin 5% (A), Collins C2 (C) und Ringer-Lactat (R) über 6 h konserviert. Die elektronenmikroskopischen Veränderungen bestehen in erster Linie im Auftreten von Chromatinverdichtungen des Zellkernes, Kernwandhyperchromatose, Schwellung und Cristaedestruktion der Mitochondrien, Schwellung und Degranulierung des endoplasmatischen Reticulums. Diese Schädigungszeichen sind für die verschiedenen Konservierungsverfahren unterschiedlich stark ausgeprägt. Den besten Zustand der Hepatocyten insgesamt gewährleistet die Collins-C2-Lösung.

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Electron microscopic findings during hypothermic storagePreservation of the liver. I.

Summary Porcine livers were preserved by simple hypothermic storage for 6 h using plasma-protein-fraction 5%, albumin 5%, Collins C2, and Ringer-lactate. Electron-microscopic alterations primarily consist of aggregations of nuclear chromatin, marginal chromatin condensation, swelling and destruction of cristae mitochondriales, swelling and degranulation of the endoplasmic reticulum. These signs of cell damage are of varying intensity depending on the different preservation methods. The best condition of hepatocytes on the whole is guaranteed by using Collins C2-solution.

     

鼓室硬化豚鼠中耳黏膜电镜下超微结构特点

目的了解豚鼠鼓室硬化中耳黏膜电镜下的超微结构特点,以探讨豚鼠鼓室硬化的发病机理。方法健康杂色豚鼠8只为研究对象,其中,6只（8耳）行鼓室硬化造模,2只（4耳）为正常对照。鼓室硬化的造模方法为显微镜下经豚鼠鼓膜紧张部后下象限向鼓室内注射1×10^8个／L金黄色葡萄球菌液100μl,观察6个月以上,观察期间不给任何治疗。以显微镜下可见鼓膜穿孔边缘或鼓室腔有钙化斑为造模成功标准。取鼓室硬化造模成功豚鼠不同部位的中耳黏膜及正常豚鼠相应部位的中耳黏膜分别在电镜下观察其超微结构。结果正常豚鼠中耳黏膜胶原纤维少,纤维细胞形态大小正常,粗面内质网、线粒体形态正常,有线粒体嵴,无溶酶体。鼓室硬化中耳黏膜纤维细胞呈不规则变形、伸长、退变,细胞核深染呈卵圆形,核周的胞浆内有肿胀线粒体和变性溶酶体聚集,内质网扩张成囊状,黏膜下层细胞基质中含大量的胶原纤维,在基质胶原纤维束间有大量无定形的富含高密度电子的小体。结论豚鼠鼓室硬化中耳黏膜电镜下见胶原纤维显著增生,细胞基质小囊泡、胞内溶酶体、脂质颗粒内的磷脂结构均有钙化,但主要发生在细胞基质小囊泡。