Partielle Lipodystrophie mit Osteomyelitis

Böhles, Hansjosef; Pm, Kövary

doi:10.1055/s-2008-1033788

Cited by 2 publications

(1 citation statement)

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“…Bei MCGN Typ 11, der "dense deposit disease" mit intramembranösen "dense deposits", deren Assoziation mit der progressiven Lipodystrophie gut belegt ist, bestehen deutlich erniedrigte C 3 -Spiegel (Aktivierung des "alternative path. way") bei meist normalen frühen Komponenten C1q, C 2 und C 4 ; im Serum der Patienten findet man häufig eine C 3 -spaltende Aktivität, den sogenannten C 3 -Nephritisfaktor (C 3 NeF), ein Immunglobulin der IgG-Klasse, das die C 3 -Konvertase des "alternative pathway" vor ihrer Inaktivierung schützt (9,14,25). .…”

Section: Einführungunclassified

,,Lipodystrophia progressiva" (Barraquer-Simon-Syndrom): Differentialdiagnose und Klinik

Böcker¹,

Weitbrecht²,

Neundörfer³

1986

Fortschr Neurol Psychiatr

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"Progressive lipodystrophy" or "partial lipodystrophy of the cephalothoracic type" is a rare, acquired condition of unknown aetiology with onset in childhood and a complete loss of subcutaneous fat of face, neck, trunk and upper extremities. The disease is more common in females than males and causes a disfigurement of the face; that it cannot be regarded only as a harmless variation, is above all due to a typical concomitant disease, membranoproliferative glomerulonephritis with hypocomplementaemia. Laboratory investigations nearly always reveal a complement-activation by the "alternative pathway" with consumption and lack of complement-component C3, a finding which allows a clear distinction between partial lipodystrophy and congenital or acquired forms of total lipodystrophy ("Berardinelli-Seip-Syndrom", lipoatrophic diabetes) and other circumscript lipodystrophies.

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Section: Einführungunclassified