The short arm deletion syndrome of chromosome 4 (4p- syndrome).

Abstract

Partial deletion of the short arm of chromosome 4 (4p-) represents another (rare) cause of cleft lip and cleft palate. Further characteristic manifestations of the syndrome (also called Wolf or Wolf-Hirschhorn syndrome) are growth failure, microcephaly, prominent glabella, hypertelorism, beaked nose, poorly differentiated and low set ears, cardiac and renal malformation and hypospadias. Life expectancy is often shortened. The 4p- syndrome has many features in common with another deletion syndrome, the cri-du-chat syndrome, and also with the Smith-Lemli-Opitz syndrome. The latter is a hereditary condition with normal karyotype. The cri-du-chat syndrome is characterized by a peculiar high-pitched, mewing cry and can be differentiated from the Wolf syndrome by the different staining characteristics (banding) of chromosomes 4 and 5.

• Authors: H Zellweger, J Bardach, J Bordwell, K Williams
• Journal: Archives of otolaryngology (Chicago, Ill. : 1960) (Arch Otolaryngol) Vol. 101 Issue 1 Pg. 29-32 (Jan 1975) ISSN: 0003-9977 [Print] United States
• PMID: 1119985 (Publication Type: Journal Article, Research Support, U.S. Gov't, Non-P.H.S.)

Topics

• Abnormalities, Multiple (diagnosis)
• Chromosome Aberrations (diagnosis)
• Chromosome Disorders
• Chromosomes, Human, 4-5
• Cleft Lip (genetics)
• Cleft Palate (genetics)
• Face
• Female
• Humans
• Infant, Newborn
• Karyotyping
• Syndrome