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Wednesday, February 17, 2021

Laband-Zimmermann syndrome

An extremely rare inherited syndrome of gingival fibromatosis, associated with hypoplasia of the distal phalanges, nail dysplasia, joint hypermobility, and sometimes hepatosplenomegaly. The nose and pinnas are usually large and poorly structures, giving the affected persons peculiar facial characteristics.

Laband-Zimmermann syndrome
Laband-Zimmermann syndrome 

Both sexes affected. Fibromatosis of the gingiva is usually present at birth or appears shortly after. Skeletal defects include clubbed tree frog-like fingers and toes, and hyperextensibility of the metacarpophalangeal joints. The nails are usually absent or dysplastic. Occasionally, mental retardation. Inheritance is autosomal dominant.

More details: 📖 ABC of Clinical Genetics 3th Edition

P. F. Laband, G. Habib, G. S. Humphreys: Hereditary gingival fibromatosis. Report of an affected family with associated splenomegaly and skeletal and sof- tissue abnormalities. Oral Surgery, Oral Medicine, Oral Pathology, St. Louis, March 1964, 17: 339-351.

Karl Wilhelm Zimmermann (1861 - 1935), German anatomist and histologist.

Karl Wilhelm Zimmermann
Karl Wilhelm Zimmermann 

Karl Wilhelm Zimmermann studied in Berlin from 1882 and received his doctorate in 1887. Turning to anatomy, Zimmermann from 1894 to 1927 was an assistant in the anatomical institutes at Greifswald and Berlin, as well as prosector at Giessen and in Bern, Switzerland. He became Dozent of anatomy at Bern in 1894. From 1898 he was professor extraordinary, and in 1927 he was appointed ordinary professor as well as director of the anatomical institute in Bern.


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