Classification of late myopathies based on observation of 15 new cases

Summary The author observed 15 cases of chronic progredient myopathy in which the first symptoms appeared after the age of 40. 7 of them belonged to the group of primary, non-purulent polymyositis and the possibility of a polymyositic origin was also present in the 8th case.Cases 9 to 15 appeared to be differentiable both from polymyositis and muscular dystrophy. Thus the author had no case which could have been regarded unequivocally as progressive muscular dystrophy. These 7 cases of unknown etiology do not constitute a uniform group nor do they represent even an independent nosological entity.In the classification of myopathies beginning after the 30th year, the author tried to follow etio-pathogenetic principles. With modern diagnostic techniques, e.g., with the biochemical, histochemical and electron microscopic examination of the muscle biopsy, it is possible to shed light on some conditions underlying late myopathies, such as endocrine changes, dysmetabolic changes, deficiency of muscle enzymes, muscle tuberculosis or sarcoidosis, etc.The residual cases may be grouped under 3 main headings: they are classified as progressive muscular dystrophy or as chronic polymyositis or may be considered as an independent disease entity which can be distinguished from both above conditions. Several authors have an extremely uniform view on these 3 possibilities.Based on literary data and on the author's experience, it can be stated that the most important group of late myopathies consists of polymyositides.Only an insignificant part of late myopathies may be regarded as progressive muscular dystrophy.In the third group, constituted by the remaining late myopathy cases, the only common feature is their unknown etiology. From the standpoint of clinical picture, histopathologic findings and therapeutic responsiveness, we are faced with a heterogenous syndrome.

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Zusammenfassung Es wurde über die Beobachtung von 15 progredienten, chronischen Myopathie-Fällen berichtet, bei denen die ersten Symptome über das 40. Lebensjahr erschienen. 7 Fälle gehörten zu den primären, nicht eitrigen Polymyositiden, und auch im Fall 8 bestand die Möglichkeit eines polymyositischen Ursprungs.Die Fälle 9 bis 15 schienen von den Polymyositiden und auch von der progressiven Muskeldystrophie abtrennbar. Der Autor hatte also keinen Spätmyopathiefall, der als Dystrophia musculorum progressiva betrachtet werden konnte. Die 7 Fälle unbekannter Ätiologie bilden keine einheitliche Gruppe und stellen auch keine selbständige nosologische Einheit dar.In der Klassifikation der nach dem 30. Lebensjahr beginnenden Myopathien wurde versucht, ein ätio-pathogenetisches Prinzip einzuleiten. Moderne diagnostische Verfahren, unter anderem biochemische, histochemische und elektronenmikroskopische Untersuchungen der Muskelbiopsie, vermögen den Hintergrund einiger Spätmyopathien aufzuklären, so z. B. Endokrinopathien, dysmetabolische Veränderungen, Mangel an Muskelenzymen, Muskeltuberkulose, Muskelsarkoidose usw.Die zurückbleibenden Fälle werden in 3 Gruppen eingeteilt: 1. Progressive Muskeldystrophien. 2. Chronische Polymyositiden. 3. Fälle, die eine selbständige nosologische Einheit darstellen und welche von den zwei obenerwähnten Untergruppen abgrenzbar sind. Die Meinung einiger Autoren ist eine äußerst uniformisierte über diese 3 Möglichkeiten.Auf Grund der Literaturangaben und eigener Erfahrungen war es feststellbar, daß die Polymyositiden die wichtigste Gruppe der Spätmyopathien darstellen.Nur einen unbedeutenden Teil der Spätmyopathien kann man für eine Dystrophia musculorum progressiva betrachten.In der dritten Gruppe, welche sich aus den zurückbleibenden Spätmyopathie-Fällen zusammensetzt, ist die einzige gemeinsame Eigenschaft der Fälle ihre unbekannte Ätiologie. Diese Gruppe stellt vom Standpunkt des klinischen Bildes, der histopathologischen Befunde und der therapeutischen Beeinflußbarkeit ein heterogenes Syndrom dar.

     

Necrotizing fasciitis in a child: a rare complication of idiopathic nephrotic syndrome

In nephrotic syndrome there is an increased tendency for bacterial infections due to immunological changes secondary to proteinuria, treatment (including steroids), and other as yet unknown causes. However, necrotizing fasciitis (NF) is an uncommon complication of the disease and has rarely been reported in nephrotic children. We report a 14-month-old boy with nephrotic syndrome who developed sepsis and NF as a complication. He was treated successfully with intensive medical and surgical treatment.     

A retrospective drug utilization evaluation of vancomycin usage in paediatric patients

Objective: To evaluate the appropriateness of use of vancomycin in paediatric patients at KK Women's and Children's Hospital, the major paediatric hospital in Singapore to identify potential problems in prescribing practices that may necessitate intervention to optimize vancomycin usage. Methods: A retrospective drug utilization evaluation was performed for paediatric patients who received intravenous vancomycin from 1 June 1998 to 31 June 1999. The outcome measures were consistency of vancomycin indication with recommended guidelines, dosing regimens, microbiological data, monitoring of serum drug levels, renal function, clinical outcomes and adverse drug reactions (ADRs). Results: A total of 96 cases was available for evaluation. Sixty‐two (64·6%) courses of vancomycin were consistent with guidelines for indication of therapy. Eighty‐six (89·6%) of the dosing regimen were consistent. All infusion times that were recorded (56·3%) were consistent with criteria. Of the patients treated with vancomycin for more than 1 day, peak and/or trough serum vancomycin levels were ordered for 70 cases. Of the 56 cases with paired levels ordered, 46 cases had at least one level that fell outside the therapeutic range. Nineteen (19·8%) cases of ADRs were documented. Fifty‐eight (60·4%) cases received concurrent nephrotoxic drugs. However, a substantial portion of vancomycin courses were apparently not prescribed for appropriate indications, and there was poor recording of vancomycin administration information and sampling time. Conclusion: The majority of dosing regimens of vancomycin was consistent with guideline criteria. The most evident problem was the sub‐optimal use of the monitoring of vancomycin serum levels. The information derived from this study may be used as a for further study and for the development of strategies for optimize vancomycin usage.     

Early detection of diabetes retinopathy by new algorithms for automatic recognition of vascular changes

Diabetes mellitus often results in diabetic retinopathy caused by pathological changes of the retinal vessel tree. Early detection of these changes can delay the disease. Image processing can reduce the workload of screeners and can play a central role in quality assurance tasks. Therefore we aimed at the refinement and development of image processing algorithms to improve the quality and cost effectiveness of screening and diagnosis of diabetic retinopathy. In order to support ophthalmologists in their routine and to enable the quantitative assessment of vascular changes in colour fundus photographs a multi-resolution approach was developed which segments the vessel tree efficiently and precisely into digital images of the retina. The vessel tracker aims at determining as correctly as possible the retinal vascular network captured on a digital image irrespective of its origin. In addition to the tracker, algorithms were developed to detect the optic disk, bright lesions such as cotton wools spots, and dark lesions such as haemorrhages. The following classification of veins and arteries identifies arteries in 78.4 % and veins in 66.5% correctly. This helps selecting conspicuous images from a great number of patients.