婴儿肝炎综合征患儿周围神经损害的临床研究

Decreased peripheral nerve conduction velocity may be associated with lower serum level of vitamin E in patients with infantile hepatitis syndrome

目的 探讨婴儿肝炎综合征 (简称婴肝 )患儿血清维生素E(VitE)对周围神经传导功能的影响。方法 收集 5 8例确诊为非胆道闭锁婴儿肝炎患儿的临床资料 ,并对其中的 31例进行了 2～ 14个月的随访。对照组为同龄 32名健康婴儿。回顾性比较、分析两组婴儿血清VitE、总胆红素(TB)、结合胆红素 (DB)和神经传导功能测试结果及其相互关系。结果 (1) 4 1例患儿血清VitE浓度低于同龄健康组 90 %正常下界 (<13 78μmol/L) ,其中 2 8例患儿低于 99%正常下界 (<9 17μmol/L) ;(2 ) 5 0例患儿血清DB >2 5 7μmol/L ,2 7例达严重高结合胆红素血症DB含量 (>10 2 6 μmol/L) ;(3) 5 0例患儿及 6 0 4 %被检神经存在至少 1项周围神经传导功能异常 ;(4 )DB≥ 2 5 7μmol/L组血清VitE浓度降低率 (78% )高于DB <2 5 7μmol/L组 (2 5 % ) (P <0 0 1) ;(5 )血清VitE降低组患儿周围神经传导功能异常率 (93% )高于VitE正常组 (71% ) (P <0 0 5 ) ;(6 )无论血清TB或DB浓度 ,与患儿周围神经传导功能异常未见明显关系 ;(7) 31例随访发现 :死亡和运动发育落后患儿病初血清VitE浓度严重降低(<9 17μmol/L)率 (88% )高于预后相对良好的患儿 (4 3% ) ,P <0 0 1。 结论 (1)超过 2 / 3的婴肝患儿存在血清VitE浓度降低 。

Objective To explore the influence of vitamin E (VitE) concentration in serum on peripheral nerve conduction in patients with infantile hepatitis syndrome (IHS). Methods A retrospective study was carried out in 58 infants suffered from IHS without congenital biliary atresia and 31 of them were followed up. Thirty-two healthy infanis were as control. The level of VitE in serum was detected with high performance liquid chromatography and nerve conduction was tested with surface electrodes along the nerves of limbs. The relationship between the level of VitE or total bilirubin (TB) or direct bilirubin (DB) and the nerve conduction velocity was analyzed comparatively. Results (1) The serum level of VitE was bellow the lower limit of 90% the normal value (13.78 μmol/L) in 71%(41/58)of patients, and was bellow the lower limit of 99% the normal level (9.17 μmol/L)in 48%(28/58)of patients. (2) The level of DB was more than 25.7 μmol/L in 86% (50/58) of the patients and was more than 102.6 μmol/L in 47% (27/58) of patients. Severe conjugated hyperbilirubinemia with cholestasis was demonstrated in most patients. (3) At least one abnormal parameter in nerve conduction test was found in 86% (50/58) patients. In 144 nerves tested, 60.4% (87/144) had at least one abnormal parameter.(4)Analysis for the association between bilirubin levels and VitE concentration in serum: in groups of DB≥25.7 μmol/L and DB <25.7 μmol/L, the percentage of decreased VitE concentrations was 78%(39/50)and 25%(2/8), respectively, and the difference was significant (P<0.01). Similar association between low VitE concentration and increased level of TB in serum could not be demonstrated. (5) Analysis for the association between abnormal nerve conduction and VitE concentration in serum: in the two groups with low and normal level of VitE, the percentage of abnormal nerve conduction was 93%(38/41)and 71%(12/17), respectively (χ c 2=4.93, P<0.05). (6) Analysis for the association between abnormal nerve conduction and bilirubin in serum: There was no significant association between abnormal nerve conduction and serum level of either DB or TB. (7) Eight patients died and 9 patients had motor development delay in 31 patients during follow up. In these 17 patients with poor outcome, 88% (15/17) had very low VitE levels (<9.17 μmol/L),which was markedly higher than the proportion of cases (43%,6/14) with better prognosis(χ c 2=7.235, P<0.01). Conclusions (1) Low VitE serum levels were found in excess of the two thirds of patients with IHS and severely decerased levels in nearly a half of them. (2) A conjugated hyperbilirubinemia with cholestasis could be found in most patients (86%) suffered from IHS without congenital biliary atresia and about a half of them had serious cholestasis. (3) Conjugated hyperbilirubinemia with cholestasis could be the predominant cause of decreased serum VitE level in this study. (4) Abnormality of nerve conduction in patients with IHS might be related to VitE deficiency.