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fong disease
<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.
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