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Die multiple endokrine Neoplasie Typ I

Clinical manifestations, diagnostic procedures and therapeutic modalities in multiple endocrine neoplasia type I (MEN I) syndrome

Diagnostik und Therapie am Beispiel einer klassischen Familienanamnese

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An Erratum to this article was published on 01 September 1999

Zusammenfassung

□ Hintergrund

Am Beispiel der charakteristischen Anamnese einer Familie mit multipler endokriner Neoplasie Typ I (MEN I) werden die Klinik, Diagnostik und Therapie der einzelnen endokrinen Manifestationen dieser Erkrankung besprochen.

□ Diagnose

Genetische Untersuchungen ermöglichen heute eine sichere Identifizierung der Genträger. Bei diesen sind regelmäßige Kontrolluntersuchungen einschließlich Screening aller beteiligten endokrinen Organe (Nebenschilddrüse, Hypophyse, Pankreas) erforderlich, da auch in höherem Lebensalter noch weitere Krankheitsmanifestationen hinzukommen können. Aufgrund konsequent durchgeführter Screening-Untersuchungen konnte das Erstdiagnosealter um ein bis zwei Jahrzehnte herabgesetzt werden. In der Folge sank die Malignitätsrate endokriner Pankreastumoren als prognosedeterminierender Faktor auf etwa ein Drittel. Es ist zur Zeit noch ungeklärt, ob die Überlebensrate der MEN-I-Patienten durch das hier aufgezeigte Vorgehen in ähnlicher Weise wie bei der MEN II positiv beeinflußt werden kann.

Abstract

□ Background

A classical family history, representative of the multiple endocrine neoplasia type I (MEN I) syndrome, is reported to illustrate the clinical manifestations, diagnostic procedures and therapeutic modalities of the various endocrine disorders of this syndrome.

□ Diagnosis

Today genetic analysis clearly identifies gene carriers. In these patients screening of all involved endocrine organs (parathyroid, pituitary, pancreas) at regular time intervals is necessary because also at higher ages additional endocrine manifestations may develop. Due to consequent screening the age at time of diagnosis was reduced by 10 to 20 years. As a consequence the rate of malignancy of pancreatic endocrine tumors which is the limiting factor with regard to long-term prognosis was reduced to about one third. At present it is not known whether this procedure can also increase the survival rate of MEN I patients as could be demonstrated in the MEN II syndrome.

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  1. Medizinische Klinik I, Abteilung Gastroenterologie, Eberhard-Karls-Universität Tübingen, Otfried-Müller-Straße 10, D-72076, Tübingen

    Regina Lamberts & Micheal Gregor

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  1. Regina Lamberts
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  2. Micheal Gregor
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Correspondence to Regina Lamberts.

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Ein Erratum zu diesem Beitrag ist unter http://dx.doi.org/10.1007/BF03044945 zu finden.

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Lamberts, R., Gregor, M. Die multiple endokrine Neoplasie Typ I. Med Klin 94, 447–452 (1999). https://doi.org/10.1007/BF03044730

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