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中华临床医师杂志(电子版) ›› 2017, Vol. 11 ›› Issue (11) : 1906 -1911. doi: 10.3877/cma.j.issn.1674-0785.2017.11.009

所属专题: 经典病例 文献

临床病例分析

丙基硫氧嘧啶相关性血管炎合并急性肾损伤一例并文献复习
魏素玲1, 高春林1, 夏正坤1,()   
  1. 1. 210000 南京总医院儿科
  • 收稿日期:2017-03-08 出版日期:2017-06-01
  • 通信作者: 夏正坤
  • 基金资助:
    国家自然科学基金资助项目(81270800)

Propylthiouracil induced vasculitis associated with acute kidney injury children: a case report and literature review

Suling Wei1, Chunlin Gao1, Zhengkun Xia1,()   

  1. 1. Department of Pediatrics, Nanjing General Hospital, Nanjing 210000, China
  • Received:2017-03-08 Published:2017-06-01
  • Corresponding author: Zhengkun Xia
  • About author:
    Correspondling author: Xia Zhengkun,
引用本文:

魏素玲, 高春林, 夏正坤. 丙基硫氧嘧啶相关性血管炎合并急性肾损伤一例并文献复习[J]. 中华临床医师杂志(电子版), 2017, 11(11): 1906-1911.

Suling Wei, Chunlin Gao, Zhengkun Xia. Propylthiouracil induced vasculitis associated with acute kidney injury children: a case report and literature review[J]. Chinese Journal of Clinicians(Electronic Edition), 2017, 11(11): 1906-1911.

目的

报告1例由于服用丙基硫氧嘧啶(PTU)导致抗中性粒细胞胞浆抗体(ANCA)阳性的血管炎合并急性肾损伤。

方法

收集患者的病史、实验室血清学及肾活组织病理检查结果,分析其诊断及治疗,并对PTU相关性血管炎文献进行综述。

结果

15岁女性患者,以突发少尿、蛋白尿起病,迅速进展至肾功能衰竭,血MPO-ANCA检查阳性,肾活检病理提示:(1)肾小球节段坏死性病变及新月体(5/7),结合病史考虑ANCA相关性血管炎(PTU相关);(2)肾小球节段毛细血管内增生性病变,免疫荧光以C3沉积为主。结合患儿有原发性甲状腺功能亢进病史,服用PTU治疗3年,明确诊断为"①PTU相关性血管炎;②急性肾损伤",立即停用PTU,给予血浆置换4次及甲泼尼龙冲击治疗3个疗程后,患儿肾功能恢复,尿量正常。随访4个月各项检查指标恢复正常。

结论

PTU可导致ANCA阳性的血管炎合并急性肾损伤,及时诊治可改善预后。

Objective

A report of 1 case of antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis complicated with acute kidney injury caused by taking propylthiouracil (PTU).

Methods

The medical history, laboratory serology and renal biopsy were collected. The diagnosis and treatment were analyzed. The literature of Propylthiouracil induced ANCA-associated vasculitis was reviewed.

Results

A 15-year-old female patients presenting with sudden oliguria and proteinuria onset developed renal failure and positive blood MPO-ANCA rapidly. Renal biopsy revealed: (1) glomerular segment necrotic lesions and crescents (5/7), considering ANCA-associated vasculitis (propylthiouracil related), (2) glomerular capillary hypertrophy lesions, immunofluorescence showed mass C3 deposition. In light of a history of hyperthyroidism treated with PTU for 3 years, of the patient was diagnosed as: 1. propylthiouracil (PTU) induced vasculitis; 2. acute kidney injury. After treatment with immediate PTU withdrawal, 4 cycles of continuous renal replacement therapy and 3 cycles of methylprednisolone, the child recovered renal function and normal urine output. Follow-up of at 4th month showed the indicators returned to normal.

Conclusion

PTU can lead to ANCA-positive vasculitis with acute kidney injury, early diagnosis and treatment can improve the prognosis.

表1 实验室检查数据(治疗前)
变量 入院检查 治疗后复查 正常范围
血红蛋白 97 101 115~150 g/L
血小板 141 158 (125~350)×109/L
白细胞计数 10.2 10.8 (3.5~9.5)×109/L
尿蛋白定量 1.15 1.02 <0.4g/24 h
尿沉渣(/HP) 3729.6 669 0~12/μl
肌酐清除率(ml/min) 8.77 127 80~120 ml/min
血白蛋白(g/L) 30.3 36.4 35~50 g/L
血肌酐(μmol/L) 897 54 46~92 μmol/L
尿素氮(mmol/L) 29.3 8.7 2.5~6.1 mmol/L
尿酸(μmol/L) 454 439 149~369 μmol/L
尿NAG酶 106.9 14.3 <17.8 μ/g?cr
尿RBP 2.5 0.1 <0.5 mg/L
免疫球蛋白G(g/L) 11.0 7.44 7~16 g/L
免疫球蛋白A(g/L) 2.25 1.82 0.7~4.0 g/L
免疫球蛋白M(g/L) 1.41 1.49 0.4~2.3 g/L
补体C3 0.169 0.949 0.9~1.8 g/L
补体C4 0.155 0.141 0.1~0.4 g/L
T3 0.5 0.75 1.23~3.07 mol/L
T4 107.67 74.85 71~161 nmol/L
TSH 1.12 2.61 0.3~4.5 mIU/L
抗甲状腺球蛋白抗体 56.87 / <115 IU/ml
抗甲状腺过氧化物酶抗体 75.57 / <34.0 IU/ml
促甲状腺激素受体抗体 0.47 / <0.75 IU/L
抗GBM抗体 阴性 / 阴性
抗TBM抗体 阴性 / 阴性
抗髓过氧化物酶抗体 (A~MPO) 阳性 阴性 阴性
抗蛋白酶抗体(A~PR3) 阴性 / 阴性
胞浆型抗中性粒细胞胞浆抗体 阴性 / 阴性
核周型抗中性粒细胞胞浆抗体 (甲醛抗性) 阳性 阳性 阴性
核周型抗中性粒细胞胞浆抗体 (甲醛敏感) 阴性 阴性 阴性
C3肾炎因子 阴性 / 阴性
补体H因子 阴性 / 阴性
抗补体H因子抗体 阴性 / 阴性
淋巴细胞免疫分型CD4 125.0 617 (651.3±273.6)个/μl
图3 电镜放大倍数:×20 000
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