Both metacarpal index and phalanx index increased
Introduction
Introduction Metacarpal index = sum of the length of the 2nd and 5th metacarpal bones / the sum of the 2nd and 5th metacarpal transverse diameters. The metacarpal bone, the bone of the palm, starts with the thumb and is the first, second, third, fourth, and fifth metacarpals. The clinical manifestations of Marfan syndrome are an increase in both the metacarpal index and the phalanx index. Manfan syndrome is a congenital mesodermal dysplastic disease, a hereditary connective tissue disease, an autosomal dominant hereditary disease.
Cause
Cause
May be suffering from Marfan syndrome. Manfan syndrome is a congenital mesodermal dysplastic disease, a hereditary connective tissue disease, an autosomal dominant hereditary disease, and individual autosomal recessive inheritance. The specific cause of the disease is unknown. It is believed to be associated with abnormalities in congenital protein metabolism.
Examine
an examination
Related inspection
Bone and joint plain M-mode echocardiography (ME) of the extremities
1. Bone changes: the main change in Marfan syndrome.
Body bone changes: long bones such as sputum, femur, sacral, and sacral bones are slender, the patient is tall, thin, and the chest is flat. The height is more than 180cm. The hands are flat and the middle finger is more than the height.
Skull changes: head length, head index
Limb changes: the fingers are slender as a spider's finger (toe), visible to the clubbing, fingering. Algae, flat feet, increased metacarpal index and phalanx index.
2. Eye changes: mainly ectopic lens, accounting for 86.8%, other blurred vision, refractive error, tremor iris, rupture of the iris, anterior chamber, narrow pupil.
3. Cardiovascular changes: mainly aortic root dilatation with aortic regurgitation, followed by ascending aortic aneurysm, aortic dissection, pulmonary artery dilatation, meningioma. The basis of vascular disease is cystic necrosis of the middle layer of the artery. Heart disease becomes mitral valve prolapse, atrial septal defect, cardiac hypertrophy, and arrhythmia.
Diagnosis
Differential diagnosis
Ma Fang syndrome must be differentiated from the following diseases:
1 homocycyuria.
2 rheumatic aortic valve insufficiency.
3 familial mitral valve prolapse or aortic valve prolapse.
4 familial aortic annulus dilatation.
5 congenital contracture spider-like finger (toe) and other identification.
1. Bone changes: the main change in Marfan syndrome.
Body bone changes: long bones such as sputum, femur, sacral, and sacral bones are slender, the patient is tall, thin, and the chest is flat. The height is more than 180cm. The hands are flat and the middle finger is more than the height.
Skull changes: head length, head index
Limb changes: the fingers are slender as a spider's finger (toe), visible to the clubbing, fingering. Algae, flat feet, increased metacarpal index and phalanx index.
2. Eye changes: mainly ectopic lens, accounting for 86.8%, other blurred vision, refractive error, tremor iris, rupture of the iris, anterior chamber, narrow pupil.
3. Cardiovascular changes: mainly aortic root dilatation with aortic regurgitation, followed by ascending aortic aneurysm, aortic dissection, pulmonary artery dilatation, meningioma. The basis of vascular disease is cystic necrosis of the middle layer of the artery. Heart disease becomes mitral valve prolapse, atrial septal defect, cardiac hypertrophy, and arrhythmia.
