Banki syndrome

Banki syndrome
Other names	Fusion of the lunate and cuneiform bones of the wrist, clinodactyly, clinometacarpy, brachymetacarpy and leptometacarpy (thin diaphysis)

Banki syndrome is a rare disorder in which two or more bones are fused. The symptoms may include: abnormality of the long bone of hand; short fingers or toes; permanent curving of the pinkie finger; fusion of wrist bones. The disorder has been reported in three generations of a single Hungarian family. ^{[1] [2]} First described by Z. Banki in a 1965 paper, ^[3] it has been noted as being similar to Liebenberg syndrome, featuring lunatotriquetral fusion of the lunate bone with the triquetral bone, clinodactyly of the fingers, overall short metacarpals, and thin diaphysis of the longer bones, but unlike Liebenberg, no elbow dysplasia is observed. ^{[4] [5]}

Sources

 This article incorporates public domain material from Banki Syndrome. National Institutes of Health.

References

1. "Banki syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 2021-03-20.
2. McKusick, Victor Almon; Osler, William (1983). Mendelian Inheritance in Man: Catalogs of Autosomal Dominant, Autosomal Recessive, and X-linked Phenotypes. Johns Hopkins University Press. p. 60. ISBN   9780801827440.
3. Banki, Z (1965). "Kombination erblicher Gelenk- und Knochenanomalien an der Hand Zwei neue Röntgenzeichen" [Combination of hereditary joint and bone abnormalities in the hand with two new x-ray signs]. RöFo: Fortschritte auf dem Gebiete der Röntgenstrahlen und der Nuklearmedizin (in German). 103 (11): 598–604. doi:10.1055/s-0029-1227792. S2CID   73398723 . Retrieved April 17, 2021.
4. Castriota-Scanderbeg, Alessandro; Dallapiccola, Bruno (2006). Abnormal Skeletal Phenotypes: From Simple Signs to Complex Diagnoses. Physica-Verlag. p. 435. ISBN   9783540303619.
5. Tiberio G, Digilio MC, Graziani M, Testa F, Giannotti A (2000). "Liebenberg syndrome: brachydactyly with joint dysplasia (MIM 186550): a second family". Journal of Medical Genetics . 37 (7): 548–551. doi: 10.1136/jmg.37.7.548 . PMC   1734641 . PMID   10970192.