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Encyclopedia > Cenani Lenz syndactylism

Cenani Lenz syndactylism (also known as Cenani's syndactyly) is a congenital malformation syndrome involving both, upper and lower extremity. It is thought to be inhereted autosomal recessive. The syndrome is named after Turkish (Asim Cenani) and German (Widukind Lenz) medical geneticists. In genetics, the term recessive gene refers to an allele that causes a phenotype (visible or detectable characteristic) that is only seen in a homozygous genotype (an organism that has two copies of the same allele). ... == A geneticist is a scientist who studies genes, or a physician who diagnoses, treats, and counsels patients with genetic disorders or syndromes. ...


It is characterised by a nearly symmetrical presence of a spoon hand (classical type) or, more frequently, an oligodactylous hand. All individuals with this syndrome had the following symptoms: carpal, metacarpal and digital synostoses, disorganisation of carpal bones, numeric reduction of digital rays and syndactyly of toes. Additionaly some individuals have some of the following symptoms: radio-ulnar synostosis, brachymesomelia, radius head dislocation, metatarsal synostoses and numeric reduction of rays.


References

  • Cenani A, Lenz W (1967). "[Total syndactylia and total radioulnar synostosis in 2 brothers. A contribution on the genetics of syndactylia]". Z Kinderheilkd 101 (3): 181-90. PMID 4298043.
  • Nezarati MM, McLeod DR (2002). "Cenani-Lenz syndrome: report of a new case and review of the literature". Clin Dysmorphol 11 (3): 215-8. PMID 12072805.
  • Harpf C, Pavelka M, Hussl H (2005). "A variant of Cenani-Lenz syndactyly (CLS): review of the literature and attempt of classification". Br J Plast Surg 58 (2): 251-7. PMID 15710123.

  Results from FactBites:
 
Cenani Lenz syndactylism - Wikipedia, the free encyclopedia (189 words)
Cenani Lenz syndactylism (also known as Cenani's syndactyly) is a congenital malformation syndrome involving both, upper and lower extremity.
The syndrome is named after Turkish (Asim Cenani) and German (Widukind Lenz) medical geneticists.
It is characterised by a nearly symmetrical presence of a spoon hand (classical type) or, more frequently, an oligodactylous hand.
Lenz - Wikipedia, the free encyclopedia (111 words)
Lenz is a place in the Grisons, Switzerland
Lenz is a literary fragment by Georg Büchner
This is a disambiguation page: a list of articles associated with the same title.
  More results at FactBites »


 
 

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