[Two cases with primary pulmonary cryptococcosis diagnosed by transbronchial biopsy].
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Biomedical subjects
Publications and source records attributed to K Hara.
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Serial polygraphic recordings of two to three hours duration were made in five full-term newborns with trisomy 18 and one full-term newborn with trisomy 13 syndrome. The newborns with 18 trisomy syndrome were poor sleepers with long periods of wakefulness and/or drowsiness. There were no consistent abnormalities in the sleep profile characteristic of 18 or 13 trisomy syndrome. These infants had some difficulty in organizing a stable sleep cycle especially during the immediate neonatal period. There was poor correlation between EEG patterns and states. After one to two weeks, cyclic organization of sleep showed some tendency towards normalization, although there were more trace alternant and fewer high voltage slow patterns in quiet sleep even around and after 44 weeks conceptional age. Other abnormal features of sleep often observed were an increase of indeterminate sleep, a decrease of quiet sleep, and an increase or decrease of active sleep in some records.
Heart rate variability was studied in normal and abnormal newborn infants during sleep in relation to the EEG background. The background EEGs of the full-term newborn infants were classified into normal, minimal depression, mild depression, moderate depression, burst-suppression and flat patterns in order of increasing severity. Nonsequential and sequential histograms were produced with the aid of a computer from some 500 consecutive RR intervals of the ECG recorded on a magnetic tape during polygraphic recordings. The seuential curves mainly showed fast oscillation during quiet sleep and slow oscillation during active sleep in normal infants. The infants who had moderately depresed EEGs presented pronounced oscillation. In those whose EEGs displayed burst-suppression and flat patterns, the sequential curves showed very little heart rate variability. They were often flat (fixed heart rate). Thus, heart rate variability was found to be one of the valuable parameters in evaluating abnormality of the CNS in newborn infants.
The electroencephalographic features and their evolutional changes with age were described in three cases of lissencephaly syndrome diagnosed by CT scan. The case with more severe lissencephaly displayed very similar EEG findings. In early or middle infancy when infantile spasms began, EEG showed very high amplitude (more than 400 microV) slow waves mixed with sharp theta-waves. In their clinical course, they showed extreme spindles and in late infancy, the EEG revealed a tendency towards bilaterally synchronous discharges of high amplitude sharp and slow waves. On the other hand, milder forms of lissencephaly showed hypsarrhythmia in early infancy. In the late infancy the EEG showed bisynchronous sharp and slow waves of more than 200 microV. The anomaly ranging from agyria to pachygyria seems to be closely associated with varying EEG abnormalities from extremely high voltage hypsarrhythmia to focal spikes. The very high voltage of hypsarrhythmic patterns and the very low frequency of sharp wave discharges seem to be typical in the most severe lissencephaly or agyria
The correlation between EEG and CT scan was studied in 57 full-term newborn infants with various neurological abnormalities, in order to clarify pathological processes underlying EEG abnormalities during the neonatal period. The relation between EEG and CT findings changes with the time elapsing after the acute phase of the perinatal brain insult. The same EEG pattern was associated with different CT findings of perinatal brain injury were usually associated with complete obliteration of cerebrospinal fluid spaces, a phenomenon considered to reflect severe brain swelling. Milkdly and minimally depressed EEGs in the acute phase were usually associated with localized decreased brain density, which is thought to represent localized edema. Moderately depressed background EEGs in the first week were associated with normal CT scans in most cases, although mild localized edema did not produce significant changes in the background EEG in some cases. In contrast to perinatal brain lesions, CT scans were not always well correlated with background EEGs in congenital cerebral dysplasia and intracranial hemorrhage, although the EEG was an useful adjunct to the CT scan in the diagnosis and prognostication of these disorders.
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The biosynthetic activities of protein and collagen from the patient of kinky hair disease, were reduced to 70 and 50%, respectively, compared with control. In tissue cultures, fast-green FCF staining coarse granules were found in the cytoplasm of skin fibroblasts from the patient. Ultrastructurally, collagen fibrils by iliac cartilage biopsy were irregular in width. Ruthenium red staining granules, presumed to be proteoglycans in the cartilage matrix, were relatively small in size and few in number. Chondrocytes showed poor development of organelles and relatively small glycogen accumulation.
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