Pediatric Wright Syndrome
Introduction
Introduction to children's Wright syndrome Reiters syndrome (RS) has three clinical features: urethritis, conjunctivitis, arthritis, so it is also called urethra, eye, joint syndrome. Infection and genetic factors are associated with the onset of the disease. Children with Wright syndrome are rare in clinical practice. basic knowledge Sickness ratio: 0.1% Susceptible people: children Mode of infection: non-infectious Complications: cystitis balanitis iridocyclitis peripheral neuritis
Cause
The cause of children's Wright syndrome
(1) Causes of the disease
The etiology of this disease is still unclear, but infection and genetic factors have a strong correlation with the incidence of this disease. Most cases are caused by intestinal infection. The intestinal infections are mostly Gram-negative bacilli, including Shigella , Salmonella, Helicobacter pylori and Yersinia, mainly after the Shigella dysentery, Paronen reported that 344 of the 150,000 cases of Shigella infection occurred in the disease, Noer in 602 cases of Shigella dysentery Among them, 6 cases of Reiter syndrome were found. In China, 90% of patients with Reiter syndrome had a history of dysentery or diarrhea before the onset of illness. Those who were positive for stool culture were F. dysenteriae. The authors found a case of typhoid fever. Later, some people have proposed infectious diseases such as spirochetes, gonococcus, mycoplasma, and chlamydia trachomatis.
Patients with Rett syndrome have a family onset trend. If there are reports of brothers suffering from this disease, and the relatives of the patients, arthritis, ankylosing spondylitis and increased incidence of psoriasis, all suggest that the disease has a genetic predisposition, patients The positive rate of HLA-B27 is more than 75%, and it is more supportive of genetic factors. The disease is more common in male elderly children, and the ratio of male to female is 10:1 to 20:1.
(two) pathogenesis
The pathogenesis of this disease is still not clear, the patient's ESR increases, C-reactive protein is positive, serum globulin is increased, suggesting that immune factors may play a role.
Prevention
Pediatric Wright Syndrome Prevention
The etiology and pathogenesis of this disease are still not clear enough, but the infection is common after intestinal infection. Therefore, extensive health campaigns are carried out. Before and after meals, wash hands, eliminate mosquitoes and flies, avoid eating unclean and deteriorating foods, and actively prevent intestinal infection. Diseases such as F. falciparum, Salmonella, Helicobacter pylori and Yersinia should have positive effects.
Complication
Pediatric Wright syndrome complications Complications cystitis balanitis iridocyclitis peripheral neuritis
Complicated cystitis, balanitis, permanent deformity of the residual foot joint; musculoskeletal lesions can be complicated with the calcaneus and plantar fasciitis, ankle arthritis, ligamentous epiphysis; ocular iridocyclitis, cornea Ulcers, etc.; can also occur multiple peripheral neuritis, temporary hemiplegia and meningoencephalitis, myocarditis or pericarditis, pulmonary infiltrates and pleurisy, diarrhea, parotid swelling and so on.
Symptom
Symptoms of children's Wright syndrome Common symptoms Back pain Joint pain Eye pain Urethral secretion of mucus or... Keratitis Corneal ulcers Dachshund obesity periostitis conduction block
90% of patients have 3 to 30 days after the infection (mostly within 2 weeks), the first symptoms are urethritis, followed by conjunctivitis and arthritis, 80% of patients have moderate to high fever, 1 to 2 per day The peak is not affected by antipyretics, and usually lasts for 10 to 40 days to relieve itself. After heat retreat, arthritis subsides.
1. Urinary, reproductive system shows that the incidence of this system symptoms is more than 90%, the most common is urethritis, there may be serous or serous purulent secretions, burning sensation or severe pain when urinating, urethral redness and swelling, can be concurrent Cystitis can also be seen in balanitis, the incidence rate is 23% to 50%, which is characterized by painless superficial erosion, surrounded by ring-shaped erythema, which can only be a small blister on the onset, and forms a superficial erosion after rupture. Coronal and glans are also affected. Sometimes balanitis can occur before other symptoms, or only balanitis and no urethritis. Female patients with urethritis are less common.
2. Joint symptoms Joint symptoms are most prominent in the triad, the incidence rate is more than 90%, there is severe pain and burning sensation, and then swelling, arthritis occurs in the second week of the disease, from single joint involvement to multiple joints, good hair Lower limb joints, such as the knee and ankle joints, are also found in the fingers, toes, wrists, hips, and spinal joints. The pain can be relieved within a few days after the application of analgesics. The general prognosis is good. It is reported that there are 5% residual foot joints. Permanent deformity, joint exudate culture sterile growth.
3. Musculoskeletal Lesions In addition to arthritis, Reiter syndrome also shows three typical musculoskeletal lesions:
(1)Sausage digit toes: The diffuse swelling of the entire finger or toe, called the sausage digit toes.
(2) periostitis: especially in the swelling or tenderness of the attachment point of the Achilles tendon or tendon, the calcaneus of the calcaneus and the plantar fascia often cause "painful heel syndrome".
(3) Ankle arthritis: lower back pain, caused by arthritis, often asymmetrical, often with ligaments.
4. 2/3 cases of eye symptoms have ocular symptoms. Ocular conjunctivitis is one of the triads of this disease. Generally, it starts from the lower eyelid and is evenly flat and smooth with a purple color. Mild can be asymptomatic and moderate. Patients with conjunctivitis have a gritty sensation and a thin serous secretion. They have uniform inflammation in the conjunctiva and the iliac crest. Local hyperemia can extend to the bulbous membrane. In severe cases, there are eye pain, photophobia and other symptoms and conjunctiva. Edema, lesions are mostly bilateral, 80% of patients with conjunctivitis relieved within 1 to 4 weeks, in addition, keratitis, scleritis, iritis, iridocyclitis, corneal ulcer, etc., keratitis The incidence is 3% to 8%, and the symptoms of the eye can disappear within a few days or weeks.
5. Other symptoms of skin mucosal lesions are common, rash from the beginning of the pale yellow blisters, and then ulceration after ulceration, mostly occurs in the genitals, palmar, upper jaw, pharynx, rash often scarring and epithelial keratosis, oral lesions also Visible in the mucous membranes of the tongue, cheeks, lips and soft palate, visible fusion erythema around the uvula and tonsils, red nipples or superficial erosion on the tongue, about 10% of patients may have cardiac abnormalities, such as myocarditis or pericardium Inflammation, electrocardiogram showed conduction block in various parts, about 1% of patients had neurological abnormalities, common in multiple peripheral neuritis, temporary hemiplegia and meningoencephalitis, respiratory lesions accounted for 8%, can be expressed as pulmonary infiltrates And pleurisy, see diarrhea, parotid swelling and other performance.
Examine
Examination of children's Wright syndrome
1. Blood examination: The number of white blood cells in peripheral blood is normal or slightly increased, mild anemia, increased erythrocyte sedimentation rate, rheumatoid factor, anti-nuclear antibody is negative, and 2/3 patients may be positive for circulating immune complex.
2. Synovial fluid examination: the synovial fluid is turbid, some cases are obviously purulent, white blood cell count is often (2 ~ 5) × 109 / L, even up to 10 × 109 / L, most of the early multinucleated white blood cells, inflammation Lymphocytes are predominant when regressed.
3. Immunofluorescence: Immunofluorescence examination of synovial tissue showed immunoglobulin deposition, IgM and C3 deposition in the perivascular and interstitial, HLA-B27 positive has important reference value.
4. Urine and urethral secretion examination: urethral secretions contain a large number of white blood cells, mostly polynuclear white blood cells, often pyuria, also visible hematuria.
X-ray examination is often normal in early and mild cases, followed by osteoporosis near the common joint, narrowing and erosive changes in the joint cavity, periostitis near the affected joint, and periosteal hyperosteogeny are characteristic X-ray findings.
Diagnosis
Diagnosis and diagnosis of children's Wright syndrome
The diagnosis of this disease includes: urethritis, arthritis, conjunctivitis triad at the same time or in a short period of time; characteristic damage of skin and mucous membranes; fever, leukocytosis, increased erythrocyte sedimentation rate, elevated serum immunoglobulin, C reaction Protein positive; urethral secretion, conjunctival secretion, synovial fluid and stool pathogen test positive; X-ray characteristic manifestations; except rheumatoid arthritis, ankylosing spondylitis, Behcet's disease.
Diagnosis can be determined when there is triad, sometimes mainly keratitis instead of conjunctivitis, or manifested as balanitis rather than urethritis, sometimes manifested by skin mucosa, so it needs to be differentiated from other diseases, joint disease should be associated with rheumatic fever , rheumatoid arthritis, infection and infection-related arthritis identification, skin mucosal lesions, should be identified with Sjoon syndrome and skin mucosal lymph node syndrome.
