本文選題：慢性炎性脫髓鞘性多發(fā)性神經(jīng)病　切入點(diǎn)：臨床分析　出處：《中國循證兒科雜志》2017年03期

【摘要】：目的探討兒童慢性炎性脫髓鞘性多發(fā)性神經(jīng)病(CIDP)的臨床特點(diǎn)和預(yù)后。方法收集2008年1月至2017年1月復(fù)旦大學(xué)附屬兒科醫(yī)院收治的CIDP患兒的臨床資料,檢索PubMed數(shù)據(jù)庫1991年1月至2017年2月的兒童CIDP病例,將文獻(xiàn)檢索和本文的病例匯總,根據(jù)到達(dá)疾病高峰期時(shí)間分組,4~8周為亞急性起病組,8周為慢性起病組,比較兩組的臨床表現(xiàn)、實(shí)驗(yàn)室檢查、治療和預(yù)后。結(jié)果 (1)符合本文納入和排除標(biāo)準(zhǔn)的CIDP患兒15例,男6例,女9例;1~3歲4例,~7歲4例,~15歲7例;起病年齡1歲1月至11歲9月;7例有前驅(qū)感染史;到達(dá)疾病高峰期時(shí)間為4周至13月,平均(12.9±13.6)周。15例的首發(fā)癥狀均為運(yùn)動(dòng)障礙,4例有感覺障礙;疾病高峰期改良Rankin量表(MRS)評(píng)分:3分8例,4分6例,5分1例;病程呈單向型3例,進(jìn)展型5例,復(fù)發(fā)型7例;15例神經(jīng)傳導(dǎo)檢查均有神經(jīng)源性損害,腦脊液檢查均有細(xì)胞蛋白分離現(xiàn)象。14例予糖皮質(zhì)激素治療,1例予靜脈丙種球蛋白治療;1例家屬放棄治療出院,其余14例住院時(shí)間7~17 d,出院時(shí)臨床癥狀均有不同程度好轉(zhuǎn)。隨訪中7例患兒復(fù)發(fā)。(2)共有14篇文獻(xiàn)報(bào)道87例CIDP患兒與本文15例合并行文獻(xiàn)分析(n=102),亞急性起病組38例,慢性起病組64例。兩組性別、起病年齡和運(yùn)動(dòng)障礙差異無統(tǒng)計(jì)學(xué)意義,感覺障礙(57.1%vs 23.5%,P=0.002)和顱神經(jīng)異常(25.7%vs 7.8%,P=0.023)亞急性起病組高于慢性起病組,且更易反復(fù)發(fā)作(62.2%vs 34.0%,P=0.010),慢性起病組單相發(fā)作形式多(50.8%vs 27.8%,P=0.026)。結(jié)論 CIDP亞急性起病患兒感覺障礙和顱神經(jīng)異常多于慢性起病患兒,并且疾病發(fā)展過程中容易復(fù)發(fā)。兒童CIDP運(yùn)動(dòng)障礙常見,較少出現(xiàn)顱神經(jīng)損害和呼吸衰竭等,腦脊液細(xì)胞蛋白分離現(xiàn)象多見,電生理改變明顯,早期治療則預(yù)后較好。
[Abstract]:Objective to investigate the clinical features and prognosis of chronic inflammatory demyelinating polyneuropathy (CIDP) in children. Methods the clinical data of children with CIDP were collected from January 2008 to January 2017 in the Pediatrics Hospital of Fudan University. From January 1991 to February 2017, children with CIDP were searched in PubMed database. The literature was searched and the cases collected in this paper. According to the time of reaching the peak of disease, the patients were divided into subacute onset group for 8 weeks and chronic onset group for 8 weeks. The clinical manifestations of the two groups were compared. Results 1) Fifteen children with CIDP, including 6 males and 9 females, 1 / 3 years old, 4 / 7 years old and 7 / 7 years old, who met the criteria of inclusion and exclusion of CIDP, had a history of prodromal infection from 1 year old to 11 years old, and 7 cases from 1 year old to 11 year old, 7 cases were diagnosed by laboratory examination, treatment and prognosis. The initial symptoms of the 15 cases were all dyskinesia in 4 cases with sensory disturbance, and the scores of 3 points in 8 cases, 4 cases in 4 cases and 5 points in 1 case, and the course of disease was unidirectional in 3 cases. Neurogenic damage was found in 5 cases of progressive type, 7 cases of recurrent type and 15 cases of nerve conduction. Cerebrospinal fluid (CSF) examination showed the phenomenon of cell protein separation. 14 cases were treated with glucocorticoid, 1 case was treated with intravenous immunoglobulin and 1 case was discharged from hospital. The remaining 14 cases were hospitalized for 7 days and their clinical symptoms were improved in varying degrees at the time of discharge. There were 14 articles about 87 cases of CIDP and 15 cases of this article combined with literature analysis, 38 cases in subacute onset group. There was no significant difference in sex, onset age and motor disorder between the two groups. The subacute onset group was higher than the chronic onset group (57.1 vs 23.5P0. 002) and the cranial nerve abnormality 25. 7 vs 7. 8 Pn0. 023). And it is more likely to repeat attack 62.2% vs 34.0% P0.010%, and 50.8% vs 27.8% P0.026 in chronic onset group. Conclusion the sensory disorders and cranial nerve abnormalities in children with subacute onset of CIDP are more than those in patients with chronic onset, and it is easy to recur in the course of disease development. CIDP motility disorder is common in children. There were few cranial nerve damage and respiratory failure, and the separation of cerebrospinal fluid cell proteins was more common, electrophysiological changes were obvious, and early treatment had a better prognosis.
【作者單位】： 復(fù)旦大學(xué)附屬兒科醫(yī)院神經(jīng)科;
【分類號(hào)】：R744.5

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本文編號(hào)：1672652