1147 LIVER ULTRASTRUCTORE IN NEUROLOGICAL GRADE I (SO-CALLED MILD) REYE'S SYNDROME (RS)
1147 LIVER ULTRASTRUCTORE IN NEUROLOGICAL GRADE I (SO-CALLED MILD) REYE'S SYNDROME (RS)
复制标题
1147 神经系统 I 级（所谓的轻度）雷氏综合征 (RS) 肝脏超微结构
DOI：
10.1203/00006450-197804001-01153
复制
发表时间：
1978
影响因子：
3.6
通讯作者：
W. Schubert
中科院分区：
文献类型：
--
作者：
J. Partin;J. Partin;W. Schubert
Of 106 consecutive cases of RS, 23 were neurological Grade I upon admission. Fourteen cases, including 2 who progressed to coma, received diagnostic liver biopsy for examination by lipid histochemistry, and electron microscopy (EM). Initial clinical chemical measures were SGOT 130-1524 IU (mean 609), SGOT 50-2300 IU (mean 761), blood NH3 15-339 μgm/dl (mean 103), CPK 0-10 Bioscience units (mean 2.3). By light microscopy, all liver specimens were typical of RS, demonstrating heavy panlobular micro-vesicular neutral fat. There was substantial variation in organelle damage from patient to patient; all demonstrated universal mitochondrial matrix expansion and pleomorphism in osmium-fixed liver, but matrix dense bodies were not universally absent, being present in most mitochondria of least damaged livers and absent in 2 livers demonstrating the most severe EM changes. Peroxisomes and smooth endoplasmic reticulum (ER) were greatly increased; glycogen was reduced and golgi lipoprotein particles were reduced or absent in all but 1 case. Rough ER was normal. Bile stasis was absent. Some disintegrating liver cells were present in all samples.Conclusion: In RS, Grade I neurological status is not synonymous with “mild” disease because: 1) some Grade I children have unexpected severe alteration of liver ultrastructure; and 2) certain children unexpectedly progress to severe encephalopathy. All Grade I cases should be hospitalized for glucose infusion and careful observation.
