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Tracheobronchial stenosis in Keutel syndrome

¹ Clinic of Pneumology, Immenhausen, Germany and ² Dept of Pathology, Klinikum Kassel, Kassel, Germany

CORRESPONDENCE: M. Meier, Lungenfachklinik Immenhausen, Robert-Koch-Str. 3, D 34376, Immenhausen, Germany. Fax: 49 5673 501101

Keywords: Keutel syndrome, matrix GLA protein, tracheobronchial stenosis

Received: May 29, 2000
Accepted July 27, 2000

Abstract

In 1971 Keutel et al. described a new syndrome in two siblings presenting with peripheral pulmonary stenoses, brachytelephalangism, neural hearing loss and abnormal cartilage calcification. Recent investigations provided evidence that mutations in the gene encoding the human matrix GLA protein cause Keutel syndrome. With these new insights in the disease the symptomatology of Keutel syndrome was reassessed. The follow-up of the two siblings was studied by clinical and post mortem examination.

As a new feature of Keutel syndrome tracheobronchial stenosis and concentric calcification of pulmonary, coronary, hepatic, renal, meningeal and cerebral arteries were described.

Complementary to the results in molecular genetics the symptomatology of Keutel syndrome could be revised by clinical and post mortem examination.

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