Pseudohypoparathyreoidismus Und Pseudo-Pseudohypoparathyreoidismus: Hereditärer Brachymetacarpaler Kleinwuchs
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Noch vor wenigen Jahren herrschte die Ansicht, daB Pseudohypopara- thyreoidismus und Pseudo-Pseudohypoparathyreoidismus seltene Erkran- kungen seien, deren Kenntnis einigen Spezialisten auf dem Gebiet der Knochen- und Nebenschilddrusenkrankheiten vorbehalten bleiben konnte. Oberblickt man jedoch das neuere Schrifttum, so kann man feststellen, daB es sich keineswegs urn Raritaten handelt, sondern daB sie jetzt haufiger beobachtet werden, weil ihre Symptomatologie bekannter wird. Die jahrelange Beschaftigung mit dem Krankheitsbild und die Beob- achtung einer relativ groBen Zahl von Kranken bilden die Grundlage dieser zusammenfassenden Darstellung. Wir konnen nicht entscheiden, ob aus genetischen Grunden eine zufallige Akkumulation von Fallen in unserem Raum entstand oder ob das Interesse fUr diese Krankheit ihre Erkennung forderte. Die umstandlichen Namen Pseudohypoparathyreoidismus und Pseudo- Pseudohypoparathyreoidismus sind Bezeichnungen fUr verschieden schwere Verlaufsformen der gleichen Erkrankung. Die Nomenklatur hat zwar vor- wiegend historische Grunde, sie ist aber letzten Endes so gut gewahlt, daB es sich als schwierig erweist, sie zu vereinfachen. Es ist zwar auch von anderen Autoren betont worden, daB Pseudohypo- parathyreoidismus und Pseudo-Pseudohypoparathyreoidismus eine nosolo- gisch einheitliche Erkrankung bilden, in der folgenden Darstellung ist aber erstmals der Versuch unternommen worden, diese Erkenntnis auf die Patho- physiologie und Pathogenese zu ubertragen. Es erwies sich dabei als not- wen dig, die Theorie FULLER ALBRIGHTS - des Erstbeschreibers - durch eine neue zu ersetzen. Seit ALBRIGHTS Entdeckung und Abgrenzung der Erkrankung sind Befunde beschrieben worden, die seine eigenen Beobach- tungen zwar bestatigten, aber neue hinzufUgten, die eine andere Inter- pretation erfordern.
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