FONG DISEASE

Also, called the nail-patella syndrome or onychoosteodysplasia. Fong disease, an hereditary condition with abnormally formed (dysplastic) or absent nails and absent or underdeveloped (hypoplastic) kneecaps (patellae). Other features include iliac horns, 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. Fong disease is inherited as dominant gene. This means that the disease can be transmitted by one affected parent. This condition is named after the physician who in 1946 discovered it in a patient on whom he performed intravenous pyelography while investigating hypertension and albuminuria related to pregnancy. On X-ray Dr. Fong saw the ‘iliac horns’ (symmetrical bilateral central posterior iliac processes) which are now known to be a characteristic feature of nail-patella syndrome.