Continuation of the Pedegree of Hereditary Potato Nose (Benjamins-Stibbe)

Nieuwenhuijse, Adriana C.; Gilse, P. H. G. van

doi:10.3109/00016485009118366

Cited by 5 publications

(1 citation statement)

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“…Anyane-Yeboa et a1 [I9841 reported on five individuals in three generations with bifid nose and suggested that this trait was an autosomal dominant condition. Benjamins and Stibbe [I9271 and later Nieuwenhuijse [1944] reported on a family in which 16 members in three generations had the potato nose trait. There was only one instance of apparent nonpenetrance in an obligate gene carrier.…”

Section: Discussionmentioning

confidence: 99%

Familial occurrence of a developmental defect of the medial nasal processes

et al. 1985

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We report on a family in which the propositus and two distant relatives have a wide nose, broad philtrum, and short columella, a fusion defect of the medial nasal processes. This anomaly resembles potato nose [Benjamins and Stibbe, Acta Otolaryngol 11:274-284, 1927] and bifid nose [Anyane-Yeboa et al, Am J Med Genet 17:561-563, 1984], which are also fusion anomalies of the medial nasal processes. Potato nose is an autosomal dominant trait, whereas bifid nose is likely heterogeneous. In this family autosomal recessive inheritance is likely, therefore suggesting that anomalies of the medial nasal processes are causally heterogeneous and represent a developmental field defect.

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Section: Discussionmentioning

confidence: 99%