A case of HTLV-I-associated myelopathy with IgA nephropathy and pseudohypoparathyroidism type 1.

Y Yoshida, S Takenaga, S Noguchi, K Sonoda, Y Arimura, K Niina, Y Yasumoto, M Osame
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Abstract

We report a case of HAM/TSP presenting with short stature, mental retardation, skin eruptions, uterine and ovarian hypogenesis and nephropathy. Skin erythema was noted since from the age of three years old and spasticity of lower extremities from elementary school age. Serum calcium level showed 4.1 mEq/l. Recombinant human PTH infusion resulted in no response of phosphate excretion. The persistent proteinuria prompted renal needle biopsy, which revealed IgA and C1q deposits in glomerular mesangium. A diagnosis of pseudohypoparathyroidism and IgA nephropathy was entertained. This patient with pseudohypoparathyroidism who has a deficient immune system was seized with the early onset of HAM/TSP and IgA nephropathy.

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htlv - 1相关性脊髓病合并IgA肾病和1型假性甲状旁腺功能减退1例。
我们报告一例HAM/TSP表现为身材矮小,智力迟钝,皮肤疹,子宫和卵巢发育不全和肾病。三岁起出现皮肤红斑,小学起出现下肢痉挛。血钙水平为4.1 mEq/l。重组人PTH输注导致磷酸盐排泄无反应。持续蛋白尿提示肾穿刺活检,发现肾小球系膜有IgA和C1q沉积。诊断为假性甲状旁腺功能低下和IgA肾病。该患者具有免疫系统缺陷的假性甲状旁腺功能减退症被早期发作的HAM/TSP和IgA肾病所捕获。
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