書誌事項
- タイトル別名
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- Histopathological study on the fetus of sanfilippo B disease.
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抄録
Light and electron microscopic studies were carried out in a 22-week-old fetus with Sanfilippo B disease. The neuronal and visceral maturation was the same as that of an age matched control fetus. In the central nervous system (CNS) electron-lucent vacuoles were found in the well-developed neurons and glial cells of the pons and spinal cord. A few membranous bodies with a dense material were demonstrated in the neuronal processes of the cerebellar white matter and pons. Electron-lucent vacuoles were observed in the liver, kidney, lung, spleen and pancreas. Nonspecific membranous bodies were present in all organs of the affected and control fetuses. The amounts of uronic acid were increased 1.5-, 3-and 5-fold in the cerebral cortex, liver and kidney of the affected fetus, respectively, as compared to those in the control fetus.<BR>These results suggested that the number of electron-lucent vacuoles and membranous dense bodies increased with the maturation in the CNS and vacuolar inclusion bodies were caused by mucopolysaccharide accumulation.
収録刊行物
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- 脳と発達
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脳と発達 16 (6), 481-486, 1984
THE JAPANESE SOCIETY OF CHILD NEUROLOGY
