SYNDROME, NAIL-PATELLA

Hereditary dominant condition with abnormally formed (dysplastic) or absent nails and absent or underdeveloped (hypoplastic) kneecaps (patellae). Other features include iliac horns (symmetrical bilateral central posterior iliac processes), abnormality of the elbows interfering with full range of motion (pronation and supination) and kidney disease resembling glomerulonephritis which.is often mild but can be progressive and lead to renal failure. The nail-patella gene locus found linked genetically to the ABO blood group in1965 is now known to be in chromosome region 9q34. Also called onychoosteodysplasia, Turner-Kieser syndrome, and Fong disease.