joint flexion syndrome in children
Introduction
Introduction to pediatric joint flexion syndrome Joint flexion syndrome, Guerin-Stern syndrome, also known as congenital multiple constochrogosis, congenital myodystrophy, arthromyodysplastic syndrome, congenital multiple joint stiffness syndrome, Post-natal fixed joint contracture syndrome, etc., this disease is a congenital malformation, clinically divided into three types: flexion type, straightening contracture type and mixed type. basic knowledge The proportion of sickness: 0.01% Susceptible people: children Mode of infection: non-infectious Complications: congenital heart disease
Cause
Causes of pediatric joint flexion syndrome
Cause:
The condition is a congenital malformation and the cause is unknown.
Pathogenesis
Pathologically, the joint capsule is inelastic, the bone is atrophy, the spinal motor is nerve-free, and there are some changes in the muscle fibers that are distributed in the muscle fibers and fat infiltration.
Prevention
Prevention of joint flexion syndrome in children
The disease belongs to congenital malformation, and the prevention methods are as follows:
1, early pregnancy, avoid fever and cold. Women who have had high fever in early pregnancy, even if the child does not have obvious appearance deformity, brain tissue development may be adversely affected, manifested as mental retardation, poor learning and reaction ability, this mental retardation can not be restored. Of course, fetal fever caused by high fever is also related to the sensitivity of pregnant women to high fever and other factors.
2. Avoid getting close to dogs and cats. Few people know that cats with bacteria are also a source of infectious diseases that are a great threat to fetal malformation, and cat feces are the main route of transmission of this malignant infectious disease.
3. Avoid women who wear makeup every day. The survey shows that the incidence of fetal malformations in heavy makeup is less than 1.25 times that of heavy makeup. The main adverse effects on fetal malformation are arsenic, lead, mercury and other toxic substances contained in cosmetics, which affect the normal development of the fetus. Secondly, some of the ingredients in the cosmetics are exposed to ultraviolet rays in the sun to produce teratogenic aromatic amine compounds.
4, to avoid mental stress during pregnancy. Human emotions are controlled by the central nervous system and the endocrine system. One of the endocrine corticosteroids is closely related to human mood changes. When pregnant women are emotionally stressed, adrenocortical hormone may block the fusion of a certain tissue of the embryo. If it occurs during the first 3 months of pregnancy, it will cause malformations such as cleft lip or palate.
5. Avoid drinking alcohol. Pregnant women drink alcohol, alcohol can enter the developmental embryo through the placenta, causing serious damage to the fetus. Such as a small head, very small ear and nose and a wide upper lip.
6, avoid eating mold and vegetarian food. Experts pointed out that if pregnant women eat food contaminated with mycotoxin (mildew food), mycotoxins can cause fetal chromosome breaks in the fetus through the placenta.
Complication
Complications of pediatric flexion syndrome Complications congenital heart disease
This disease can coexist with other malformations such as congenital heart disease, cleft palate.
Symptom
Symptoms of pediatric joint flexion syndrome common symptoms joint deformity joint stiffness muscle atrophy hip dislocation expression indifference
The disease can be divided into three types: flexion type, straightening contracture type and mixed type. It can occur in both men and women. After birth, there are symmetrical multiple joint flexion or straight contracture deformity of the upper and lower limbs. A few can only invade a single limb, and the lower limbs are affected. In the upper limbs, even the spine can be violated. The violation of the spine is often delayed rather than present at birth.
The muscles of the affected limbs are atrophic, the skin lines of the joints disappear, the skin is tense and bright, and the flexion deformity often has the formation of skin sacs. The elbows are straight and the knee joints can be columnar or fusiform, and the range of contraction of the contracture is small. Even stiff, but no painful performance, due to poor facial muscle development, children often appear indifferent.
Examine
Examination of pediatric joint flexion syndrome
1. Blood, urine, and routine examinations generally have no special findings, and may have the characteristics of infected blood when infected.
2. Muscle biopsy muscle fibers in the distribution of some degenerative muscle fibrosis and fat infiltration and other changes, muscle biopsy can be seen in its pathological features are denervated and nerve re-dominant phenomenon.
3. The muscle electrical stimulation response of the child is extremely low, and the electromyogram has no degenerative changes. In the soft tissue level of the X-ray examination, the muscle tissue is reduced and the subcutaneous fat is relatively thickened. In some cases, the wrist bone may be seen. Distance fusion, increased joint shadow density, poor development of the femoral head, etc., for reference when diagnosed.
Diagnosis
Diagnosis and differential diagnosis of pediatric joint flexion syndrome
diagnosis
According to the typical clinical joint birth defects, it can be diagnosed. Only a single joint special deformity such as forearm pronation contracture deformity, stiff congenital hip dislocation and difficult to correct scoliosis are difficult to confirm. Muscle biopsy is difficult. Help with diagnosis.
Differential diagnosis
Muscular atrophy, attention to differentiation with progressive spinal muscular atrophy, clinical manifestations and laboratory, auxiliary examination can help diagnose.
