?616?
论
著
文章绩号:1005-2224(2007}08—0616—05
儿童慢性马兜铃酸肾病3例临床
病理分析及文献复习
‘
张焱,姚勇,黄建萍,刘景城,肖慧捷,杨霁云
【摘要】
目的通过报告3例慢性马兜铃酸肾病,增加对其临床与病理特征的认识。方法‘对北京大学第一
医院儿科收治的3例慢性马兜铃酸肾病患儿进行回顾性分析,总结其临床和病理特点,并复习相关文献。结果3例慢性马兜铃酸肾病患儿,男1例,女2例,年龄为lO岁、14岁和16岁;分别因乙型肝炎、颅咽管瘤术后脑水肿和紫癜性肾炎服用舍马兜铃酸成分的中草药或中成药4~8个月,于开始服用2个月互6年后发病;3例息儿均以不同程度的肾功能损害伴近一远端肾小管受损为主要表现。其中2例以贫血为首发症状,肾功能损害严重,均已达到肾衰竭尿毒症期,另l例以糖尿为首发表现,肾功能损害较轻,同时表现继发性Fanconi综合征。3例肾病理均以广泛性或多灶状寡细胞性问质纤维化和肾小管萎缩为特征,肾小球病变轻微,免疫荧光除2例因基础疾病见IgA(++一+++)外.均未见明显免疫复合物沉积。2例惠儿停用舍有马兜铃酸的药物后肾功能仍呈不同程度的进行惶恶化,其中l饲放弃治疗半年后死于消化道大出血,l例等待肾替代治疗;另l例肾功能损伤较轻者,停用舍有马兜铃酸的药物后经小剂量糖皮质激素和营养肾小管治疗病情改善,肾功能及肾小管功能恢复,仍在临床随访中。结论儿童慢性马兜铃酸肾病临床特点是渐进性肾功能损害和肾小管功能受损,肾脏病理特征为广泛的寡细胞性阉质纤维化和肾小管萎缩;在停服舍马兜铃酸药物后紧功纯恶化常不可逆转,最终导致终末期紧病;小剂量糖皮质激素对于缓解早期轻症者的病情可能有益。预防该痛的关键在于提高对舍马兜铃酸中药肾毒性的认识。l关键词】
马兜铃酸;肾损害;儿童
文献标志码:A
中图分类号:R72
Clinicalandpathologicalanalysisof3childrenWithchronicaristolochicacidnephropathyandreviewoftheliter-ature.
ZHANGYah.YAO100034,China
yo愕,HUANGJian-ping,et
a1.Department
ofPediatrics.PekingUniversityFirstHospital,
&ijing
Abstractworld。but
Objective
Many
eases
ofaristolochieacid
nephropathy(AAN)in
adultshadbeenreportedthroughoutthe
chronicaristolochieacidnephropathy(CAAN)hadseldombeendescribedinchildren.Wereported3children
to
withCAANdiagnosedrecently
otis
The3
esse8
investigatetheclinicalandpathologicalcharacteristicsofthediseasein
children.Meth-
with
werestudied
retrospectively。andrelevant
literatures
wag
reviewed.Results
to
our
Threechildren
CAAN,including1boyand2They
girls。aged10。14and16yearsrespectively。werereferreddepartmentofpediatrics.
received
traditionalChineseherbmedicine
containingaristolochieacid4
to
to
8monthsfordifferentbasaldiseasesin-
chdingchronicaggressivehepatitisB.hydrocephalussecondary
the
operationofcraniopharyngioma
and
purpuranephri-
tisIandsufferedfromCAANfrom2mont{措to6yearsaftertheyfestationofthem
beganreceiving
theChinesemedication.The/nainmanl?
of
our
patients岫renal
failureofvariousdegreeaccompaniedwithproximalanddistaltubulardisfunction.Two
onset
beganwithanemia
andsufferedfromseriousrenalfailure。whichhadachievedUremia.Theofanother
ca6e
脚glueosnria
withrenalfunctionimpairedmild}y。andshepresentedsecondaryFanconi
syndrome
simultaneously,Their
glomemlar
pathologicalcharacteristicswerediffusepaucicellularinterstitialfibrosis
andmarkedtubular
atrophywithmild
two
impairment.AfterwithdrawaloftheChineseherbmedicinecontainingaristolochicacid。renalfailureintientsattenuatedprogressively.Oneofthemgoing
to
ofthethreepa-
one
diedofgastrointestinalmassivehacmorrbagehalf
a
yearlater.another
was
receiverenalreplacementtreatment.Anothercase-wbese
renalfunctionwas
impaired
mildly-received
low
d∞nge
steroidandrenaltubularnutritiontherapyafterwithdrawalof
作者单位:北京大学第一医院儿科,北京100034通讯作者:姚勇,E—mail:yongya02003@yah∞.com.cn
theChinese
herb
medicine
containing
aristolochic
acid,her
renalandtubularfunction
ameliorated
gradually.This
patient
is
万方数据
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