wrist sign
Introduction
Introduction The patient held the contralateral wrist at the proximal end of the contralateral humeral head with one hand, with the thumb and little fingers around for 1 week, and if the thumb and the little finger could overlap each other without pressure, it was positive.
Cause
Cause
Cause of wrist sign
More common in Ma Fan syndrome, this congenital malformation is autosomal dominant and genetic mutation. The accumulation of mucopolysaccharides such as chondroitin sulfate A or C, the structural and functional defects of elastic fibers are the basis for the pathological changes of this deformity.
Examine
an examination
Related inspection
Bone and joint MRI examination urine routine examination
Diagnosis of wrist signs:
Seen in Ma Fan syndrome, its clinical manifestations are very different, mainly in the following aspects.
The body is slender, the face is long and narrow, the limbs are slender, especially the limbs are more prominent, the finger distance is greater than the height, and the length of the lower part of the body (from the upper edge of the pubic symphysis to the heel) is larger than the upper section (height minus the length of the lower section). The hand and foot are slender, the joint is slack; the thumb sign (4 fingers pressed on the thumb when the fist is on, the tip of the thumb is white) is positive; the wrist sign is positive, the knee sign (one knee on the other tip of the toe can touch the ground) Positive, loose hands, wrists, and joints, dislocation of the ankle, hip, shoulder joint or subluxation. Arch arch depression, high bow varus, and flat foot eversion may occur. The combination of joint contractures is called congenital spider finger (toe) contracture, which can show finger, knee joint, elbow joint, shoulder joint contracture, can display scales, elbows; scoliosis, kyphosis, individual cases There is a lordosis, with the largest single curve, the right chest, and the left waist. It can also be hyperbolic or multi-curved. Severe scoliosis can be combined with spinal spondylolisthesis, causing pain due to pulling the cauda equina.
Chicken breast, funnel chest, spontaneous pneumothorax, ascending aorta dilatation caused aortic insufficiency. From childhood to adulthood, ascending aortic dilation is aggravated at any time, eventually forming an ascending aortic aneurysm, ascending aortic dissection, mitral valve prolapse, left ventricular hypertrophy, leading to a series of cardiac symptoms.
The ectopic lens is caused by the relaxation or rupture of the suspensory ligament, and its incidence is as high as 50% to 80%, resulting in subluxation of the lens. This change originates in the embryo. In addition, due to the long eyeball, the lens has poor concentrating ability, and high myopia is more common.
Congenital spider finger (toe) contracture can express large ears, double ears, windward ears, high sacral arches, mental retardation, testicular hypotonia, due to defects in the larynx, trachea, bronchial cartilage stent, wheezing after upper respiratory tract infection, The pronunciation is hoarse and presents "public ducks."
McKusick divides the signs of Marfan syndrome into two categories: general signs (hard signs) and small signs (soft signs). Lens ectopic, ascending aorta dilatation, severe kyphosis, and chest deformity are major signs. Myopia, fine height, mitral valve prolapse, ligament relaxation, spider finger (toe), thumb sign, wrist sign, knee sign positive for small signs. Only a major sign is a true interstitial tissue defect, and small signs can cross in the normal population.
Must have 2 or more general signs and some small signs to be sure of the Marfan syndrome. There is only one general sign and some small signs, which may be Marfan syndrome. Only some small signs without major signs should be classified as Ma Fan.
These diseases manifest themselves in the physical, ocular and vascular specific signs. Generally speaking, the diagnosis is not difficult. However, due to the great difference in clinical manifestations, the hidden Marfan syndrome often causes difficulties in diagnosis and controversy. Modern diagnostic criteria were established at the 7th International Congress of Human Genetics in 1986 and were clearly defined at the first International Marfan Syndrome Symposium in 1988.
Diagnosis
Differential diagnosis
Differential diagnosis of wrist signs:
Must have 2 or more general signs and some small signs to be sure of the Marfan syndrome. There is only one general sign and some small signs, which may be Marfan syndrome. Only some small signs without major signs should be classified as Ma Fan.
Seen in Ma Fan syndrome, its clinical manifestations are very different, mainly in the following aspects.
The body is slender, the face is long and narrow, the limbs are slender, especially the limbs are more prominent, the finger distance is greater than the height, and the length of the lower part of the body (from the upper edge of the pubic symphysis to the heel) is larger than the upper section (height minus the length of the lower section). The hand and foot are slender, the joint is slack; the thumb sign (4 fingers pressed on the thumb when the fist is on, the tip of the thumb is white) is positive; the wrist sign is positive, the knee sign (one knee on the other tip of the toe can touch the ground) Positive, loose hands, wrists, and joints, dislocation of the ankle, hip, shoulder joint or subluxation. Arch arch depression, high bow varus, and flat foot eversion may occur. The combination of joint contractures is called congenital spider finger (toe) contracture, which can show finger, knee joint, elbow joint, shoulder joint contracture, can display scales, elbows; scoliosis, kyphosis, individual cases There is a lordosis, with the largest single curve, the right chest, and the left waist. It can also be hyperbolic or multi-curved. Severe scoliosis can be combined with spinal spondylolisthesis, causing pain due to pulling the cauda equina. Chicken breast, funnel chest, spontaneous pneumothorax, ascending aorta dilatation caused aortic insufficiency. From childhood to adulthood, ascending aortic dilation is aggravated at any time, eventually forming an ascending aortic aneurysm, ascending aortic dissection, mitral valve prolapse, left ventricular hypertrophy, leading to a series of cardiac symptoms. The ectopic lens is caused by the relaxation or rupture of the suspensory ligament, and its incidence is as high as 50% to 80%, resulting in subluxation of the lens. This change originates in the embryo. In addition, due to the long eyeball, the lens has poor concentrating ability, and high myopia is more common.
Congenital spider finger (toe) contracture can express large ears, double ears, windward ears, high sacral arches, mental retardation, testicular hypotonia, due to defects in the larynx, trachea, bronchial cartilage stent, wheezing after upper respiratory tract infection, The pronunciation is hoarse and presents "public ducks." McKusick divides the signs of Marfan syndrome into two categories: general signs (hard signs) and small signs (soft signs). Lens ectopic, ascending aorta dilatation, severe kyphosis, and chest deformity are major signs. Myopia, fine height, mitral valve prolapse, ligament relaxation, spider finger (toe), thumb sign, wrist sign, knee sign positive for small signs. Only a major sign is a true interstitial tissue defect, and small signs can cross in the normal population.
