A Study On Damage Of Peripheral Nerves And Its Pathogenesis In Patients With Syndrome Of Infantile Hepatitis

Part ⅠA Clinical Study on Damage of Peripheral Nerves in Patients with Syndrome of Infantile Hepatitis【Objective】To explore the influence of vitamin E(VitE) concentration in serum to peripheral nerve conduction in patients with syndrome of infantile hepatitis.【Methods】 A retrospective study was carried out in 58 infants suffered from syndrome of infantile hepatitis without congenital biliary atresia and 31 of them were followed up. The level of VitE in serum was detected with high performance liquid chromatography(HPLC) and nerve conductions 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 lowest limit of the normal value(<10.61μmol/L) in 62.07%(36/58) of patients, and lower than 3 standard deviation of normal level (<6.20μmol/L) in 34.48%(20/58) of patients and less than 2.79mol/L in 17.24% (10/58) of patients. It was indicated that the level of VitE was decreased in more than 60% of the patients with the syndrome and was decreased seriously in 1/3 of them. (2) The level of DB was more than 27.7μmol/L in 86.2%(50/58) of the patients and was more than 102.6μmol/L in 46.55% of patients(27/57). Severe conjugated hyperbilirubinemia with cholestasis was demonstrated in most patients. (3) One abnormal parameter in nerve conduction test was found in 86.2%(50/58) patients at least. In 144 nerves tested, 60.42%(87/144) hadat least one abnormal parameter. It could be well shown that patients with syndrome of infantile hepatitis were always complicated with abnormal nerve conduction. The processes of axonal degeneration and demyelination in the pathological nerves were almost equal according to106 abnormal parameters of the nerve conduction tests from the patients. (4) Analysis for the association between bilirubin levels and VitE concentration in serum: Decreased VitE concentrations were shown in 70%(35/50) of 50 patients with high level of DB but only in 12.5%(1/8) of 8 patients with normal DB level. It could be indicated that low level of VitE was predominantly related to conjugated hyperbilirubinemia with cholestasis(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: There were 83.3%(30/36) of patients with abnormal nerve conduction in 36 patients with low level of VitE but only 27.2%(6/22) of patients with abnormal nerve conduction in 22 patients with normal VitE level, (P<0.01). Abnormal nerve conductions were more likely to low VitE level in serum. (6) Analysis for the association between abnormal nerve conduction and bilirubin in serum: There were no significant association between abnormal nerve conduction serum level either DB or TB. (7) There were 8 patients dead and 9 patients with motor development delay in 31 patients in follow up. In these 17 patients with poor outcome, there was 94.1%(16/17) of them with low VitE levels (<10.61μmol/L) and 76.47%(13/17) of them with very low levels of VitE (<6.20μmol/L). It was indicated that poor prognosis was more like to the patients with much lower serum VitE levels. 【Conclusions】(1) Low VitE serum levels were presented almost in the two third of patients with infantile hepatitis syndrome and severe decerased levels in the one third of them.(2) A conjugated hyperbilirubinemia with cholestasis could be found in most patients (86.2%) suffered from infantile hepatitis syndrome without congenital biliary atresia and about half of them with cholesasis seriously.(3) Conjugated hyperbilirubinemia with cholestasis should be the main cause to decrease the serum VitE level in this study.(4) Abnormal nerve conduction could be found in most patients with infantile hepatitis syndrome. A abnormal electrophysiological examination...