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mixed connective tissue disease pediatric


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Children, female, 12 years old, due to rash associated with muscle pain, weakness 1 month hospitalization. 1 month before admission, a rash to the face, hands, legs as the forward, no itching. With lower limb muscle pain, whole body fatigue, loss of appetite appetite, weight loss. examination: body temperature 36.2 ℃, pulse 120 beats / min, breathing 20 times / min, blood pressure 100/60 mm Hg. development, nutrition medium. cheeks and nose butterfly at dull red stain visible, clear boundary; scattered within hair red rash; hands and fingers at the occasion of the size of fish of different sizes visible erythema; double-leg of varying sizes scattered purpura. no time superficial lymph nodes. cardiopulmonary abdomen were normal. limb proximal muscle tenderness evident. neurological examination was normal. laboratory: anti-nuclear antibody: anti-double stranded (ds)-DNA (), anti-smooth muscle antibodies (-), anti-soluble nuclear protein antibody (UIRNP) (); immune globulin and complement the normal; ESR 69 mm / 1 h; blood WBC 3.9 × 109 / L, RBC 3.36 × 1012 / L, Hb 94 g / L; urinary WBC 8.1/HP, RBC 3.8/HP, BLD (occult blood), PRO ( protein); liver function: ALT 125 U / L, AST 228 U / L, TP (total protein) 53.8 g / L, A / G 0.92; enzymes: CK 1770 U / L, CK-MB 36.6 U / L, HDBH 591 U / L; renal function: BUN 9.29 mmol / L; immunoglobulin and complement were normal. pathologic diagnosis: (gastrocnemius) of inflammatory myopathy (in accordance with dermatomyositis). diagnosis of mixed connective tissue disease. hospitalized fever after the children, the readme, as the material is unclear, difficulty breathing, when orthopnea. Examination: irritability, breathing 60 times / min, heart rate 180 times / min, blood pressure 90/70 mm Hg, smell and wet lung  sound, right lung breath sounds low, low heart sound blunt, gallop, liver Youlei of about 4.0 cm. ophthalmologic examination: blood vessels near the posterior pole of both eyes see a lot of lint scattered spots, center right eye macular reflex (), left ocular reflex is unclear. B Ultra showed right pleural effusion. review of renal function, BUN gradually increased to 20.69 mmol / L, creatinine 105.8 mmol / L. On the Sun Yunxiao (Binzhou Medical College Hospital, Pediatrics, Shandong, Binzhou, 256603 ); Li Youjie (Binzhou Medical College Hospital, Department of Biochemistry, Shandong, Binzhou, 256603); Ranhuan Ying (fat city hospital of Chinese medicine, pediatrics, Shandong, Feicheng, 271600); Title Clinical 2006 21 22 English Journal Name JOURNAL OF APPLIED CLINICAL PEDIATRICS Keywords children of mixed connective tissue disease with multiple organ damage case report Keywords R729 part names similar to the literature references cited literature similar degree in foreign language similar to similar meetings
mixed connective tissue disease pediatric

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Mixed connective tissue disease in children is what causes?
(A) etiology
MCTD cause is unknown, its incidence may be related to the immune disorder, the evidence for the p
ersistence of high titers of serum anti-RNP antibodies were significantly higher C polyclonal hypergammaglobulinemia, B cell activity is too high, inhibition of T cell deficiency, active disease increase blood circulation immune complexes, 25% of patients with low complement hyperlipidemia, found the vessel wall, the muscle fiber membrane, the glomerular basement membrane and the binding site of skin epidermis and dermis are IgG, IgM, IgA and complement deposition, between the disease and HLA relationship under study, some people think he has a genetic background of specific immunization with HLA-DR4,-DR5 closely related to the independence that MCTD is a disease.
(B) of the pathogenesis
The pathogenesis of MCTD is not known, the following evidence that its pathogenesis may be related to immune-mediated related: circulating immune complex deposition in glomeruli, serum anti-RNP antibody positive, many organizations have a wide range of lymphocytes, plasma cells infiltration, high
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