A study of beta 2-microglobulin skin deposits in dialyzed patients and healthy controls

This study reports on beta 2-microglobulin (beta 2M) deposits in the skin of 12 uremic patients and three kidney transplant recipients compared with eight healthy controls. Uremic patients were treated by hemodialysis (HD), hemofiltration (HF), hemodiafiltration (HDF), or continuous ambulatory peritoneal dialysis (CAPD) for a period lasting from 1 to 19 years. Congo red staining of the skin was negative in patients and controls. However, immunofluorescent staining with an anti-beta 2-microglobulin monoclonal antibody was positive in the skin of all patients and of six of the eight controls. Beta 2M skin deposition is more intense in patients than in controls and increases with patient age and the duration of dialysis. A stron correlation is observed between the extent of skin beta 2M deposits and clinical manifestations due to beta 2M deposits in internal organs. However, no correlation is found between beta 2M skin deposits and sex or beta 2M serum levels.     

Hereditary Cerebral Hemorrhage with Amyloidosis-Dutch Type (HCHWA-D): I - A Review of Clinical, Radiologic and Genetic Aspects

Hereditary cerebral hemorrhage with amyloidosis - Dutch type (HCHWA-D) is an autosomal dominant disease caused by deposition of β-amyloid in the leptomeningeal arteries and cortical arterioles, in addition to preamyloid deposits and amyloid plaques in the brain parenchyma.
The disease is due to a point mutation at codon 693 of the amyloid precursor protein (βPP) gene at chromosome 21. Since this point mutation is diagnostic for HCHWA-D, presymptomatic testing is feasible and offered, together with genetic counselling and psychological support, to subjects at risk. HCHWA-D is clinically characterized by recurrent strokes, in addition to dementia, which can occur after the first stroke but also preceding it. Radiological studies revealed focal lesions (hemorrhages, hemorrhagic and non-hemorrhagic infarctions) and diffuse white matter damage. Diffuse white matter hyperintensities on MRI are an early symptom of HCHWA-D since they have been found on MRI scans of subjects who had not suffered a stroke.
The presence of the diagnostic point mutation makes HCHWA-D a useful model to study the effects of cerebral amyloid angiopathy in vivo. The characteristic pathological abnormalities and its implications for Alzheimer's disease will be discussed in Part II of this article     

Erfassung muskuloskeletaler Beschwerden mittels Fragebogen

Zusammenfassung Der standardisierte Fragebogen zur Erfassung muskuloskeletaler Beschwerden des japanischen Komitees zum Studium arbeitsbedingter Gesundheitsstörungen im Hals-Arm-Gebiet wurde auf Deutsch übertragen und seine Reliabilität und Validität überprüft. Im Fragebogen wird anhand eines Körperschemas nach der Häufigkeit (nie/selten, gelegentlich, fast täglich) von Beschwerden (Schmerzen, Steifigkeit, Müdigkeit) in 12 Körperregionen gefragt. Der Fragebogen wurde gleichzeitig mit ärztlichen Palpationsuntersuchungen bei 644 Personen aus zwölf Berufsgruppen im Dienstleistungssektor eingesetzt. Auf Grund einer Faktoranalyse der Fragebogenresultate kann festgestellt werden, dass Beschwerden regional getrennt (nämlich im Nacken-Schultergebiet, dem Rücken und je der rechten und linken oberen Extremität) auftreten und für diese Regionen die Symptome zu Indizes zusammengefasst werden können. Diese Indizes sind reliabel (Cronbach 0.8). Mit zunehmenden Indexwerten nehmen schmerzhafte Palpationsbefunde stetig zu. Beschwerden korrelieren mit vermehrtem Medikamentenkonsum und Arztkonsultationen. Der Fragebogen stellt folglich ein valides Untersuchungsinstrument für muskuloskeletale Beschwerden am Arbeitsplatz dar.

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Summary The standardized illustrated questionnaire on musculoskeletal disorders of the Japanese Committee on Occupational Cervico-brachial Disorders was translated into German. This questionnaire, composed of 37 items about the occurrence (never/seldom, occasionally, almost daily) of symptoms (pain, stiffness, fatique) in twelve body regions, was used together with medical examinations (pressure points), and further information was gathered on the consumption of analgetics and medical visits prompted by musculoskeletal symptoms. The questionnaire's reliability and validity were tested in 644 persons from twelve occupational groups within the service sector. Factoranalyses showed that symptoms can be grouped into four distinct regions of occurrence: neck/shoulder/area, back/low back, and both left and right upper extremities. Indices based on these regions are consistent (Cronbach 0.8). Palpation findings steadily increase with increasing index values. The consumption of medicaments and medical visits positively correlate with the indices. Consequently the questionnaire is judged to be a valid instrument for studying musculoskeletal disorders at the workplace.

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Résumé Le questionnaire illustré et standardisé sur les symptômes musculaires et osseux du comité japonais sur la cervico-brachialgie professionelle a été traduit en allemand et sa reliabilité et validité ont été testées. Le questionnaire est composé de 37 variables sur la fréquence (jamais/rarement, par occasion, presque toujours) des symptômes (douleur, raideur, fatigue) dans douze régions du corps. Il était distribué simultanément aux examens médicaux parmi 644 personnes de douze groupes d'employés dans le secteur de service. D'autres informations concernent la fréquence de la consommation des analgésiques et des visites médicales pour des douleurs musculaires. Une analyse factorielle a montré que les symptômes peuvent être groupés en quatre régions du corps: nuque/épaules, dos et les extrémités supérieurs. Les indices sur ces régions sont consistant (Cronbach 0.8). Les douleurs provoquées par la palpation augmentent avec la valeur de l'index. La consommation des analgésiques et les consultations sont correlées avec les indices. Par conséquent, le questionnaire illustré est un instrument valable pour étudier les problèmes musculaires sur les lieux de travail.

     

Heterogeneity of glomerular size in normal donor kidneys: impact of race

Glomerular size has been the subject of many studies and, in a number of settings, has a direct association with the development of glomerular sclerosis. However, the normal distribution of glomerular size has not been thoroughly evaluated in the general population in the United States. To address this issue, we analyzed the baseline biopsy specimens of 103 human donor kidneys to determine the maximal planar area (MPA) of the glomerular tuft in a heterogeneous human population. The MPA of each glomerulus was determined by measurement of sections through the vascular pole and/or origin of the proximal tubule, and was determined on each section by two methods: point counting and computer planimetry. There was very high agreement between these two methods. Multivariate analysis was used to identify significant correlates with MPA. Overall, younger donors had smaller glomeruli (P < 0.0001). Black donors had a larger MPA (23.4+/-8.6 mm2 x 10(-3)) than white donors (17.9+/-6.7 mm2 x 10(-3); P < 0.001), independent of donor age. MPA was not significantly different between genders. This heterogeneity in glomerular size may confound clinical studies if not recognized and may help explain differences in glomerular structure and function in response to injurious processes.