Metabolic disease with autoimmune phenomena: 2 cases of SLE-like disease in young children diagnosed with lysinuric protein intolerance
نویسندگان
چکیده
The second son of consanguineous parents was repeatedly evaluated for failure to thrive, intermittent vomiting, diarrhea, hepatosplenomegaly and panzytopenia since his first year of life. Specific diets had no effect. Bone marrow aspiration showed hemophagocytosis, but no signs of malignancy. At the age of 6 years he developed a hemolytic uremic syndrome-like disease. Renal biopsy revealed mesangioproliferative glomerulonephritis, classified as Lupus nephritis IIa, consistent with positive findings for ANA (1:320), La-, Sm-, RNP-, histoneand phospholipid antibodies, so he received immunosuppressive therapy.
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