The Finnish type of Congenital Nephrotic Syndrome is characterized by large placenta, early manifestation, growth and developmental delay and resistance to treatment. Authors experienced a case of characteristic Finnish type of Congenital Nephrotic Syndrome in a girl, who was admitted to the Pediatric Department of KHUH at 2 1/1 months of age because of generalized edema and abdominal distension and died of pneumonia at 5 1/2 months of age. The diagnosis was made by birth history, clinical manifestation, laboratory findings and finally by the autopsy findings, Literatures are reviewed briefly.
Autopsy ; Diagnosis ; Edema ; Female ; Growth and Development ; Humans ; Nephrotic Syndrome* ; Placenta ; Pneumonia ; Reproductive History

Mild cognitive impairment (MCI) has been defined as a transitional state between normal aging and Alzheimer disease. Diffusion tensor imaging (DTI) can estimate the microstructural integrity of white matter tracts in MCI. We evaluated the microstructural changes in the white matter of MCI patients with DTI. We recruited 11 patients with MCI who met the working criteria of MCI and 11 elderly normal controls. The mean diffusivity (MD) and fractional anisotropy (FA) were measured in 26 regions of the brain with the regions of interest (ROIs) method. In the MCI patients, FA values were significantly decreased in the hippocampus, the posterior limb of the internal capsule, the splenium of corpus callosum, and in the superior and inferior longitudinal fasciculus compared to the control group. MD values were significantly increased in the hippocampus, the anterior and posterior limbs of the internal capsules, the splenium of the corpus callosum, the right frontal lobe, and in the superior and the inferior longitudinal fasciculus. Microstructural changes of several corticocortical tracts associated with cognition were identified in patients with MCI. FA and MD values of DTI may be used as novel biomarkers for the evaluation of neurodegenerative disorders.
Aged ; Aged, 80 and over ; Aging/*pathology ; Anisotropy ; Biological Markers ; Cerebral Cortex/*pathology ; Cognition Disorders/*pathology ; Diffusion Magnetic Resonance Imaging/*methods ; Female ; Humans ; Male ; Middle Aged ; Neural Pathways/*pathology ; Severity of Illness Index

BACKGROUND: Progressing stroke (PS) variably develops from initially the same size and severity, and is most frequently observed in lacunar infarctions. We investigated fractional anisotrophy (FA), mean diffusivity (MD) and infarct volume by using diffusion tensor imaging during the acute phase of ischemic stroke to determine whether these parameters are useful in characterizing and predicting PS. METHODS: In this study, 55 consecutive patients admitted within 24 hours of the onset of their first ischemic stroke were included. NIH stroke scale (NIHSS) and Canadian Neurological scale (CNS) were performed upon admission, twice a day, and at discharge. Modified Rankin scale and Barthel index were also evaluated. PS was defined as a 2-point drop in NIHSS and a 1-point drop in CNS from admission to day 3. A correlation analysis was performed between clinical scale scores and imaging parameters, and the distribution of those values was compared between the two groups with and without PS. RESULTS: Significant correlations were observed between clinical scale scores and infarct volumes. The FA ratio in 14 patients with PS was lower than the patients without PS (p=0.004). Other characteristics including infarct volume and MD ratio were not different. The FA ratio remained as an independent predictor of PS (OR, 1.055; p=0.011). CONCLUSIONS: In acute ischemic stroke within the first 24 hours, only infarct volume was correlated with clinical status. However, patients with PS showed lower FA values, which accounts for rapid and severe vasogenic edema involving the disruption of the cell membrane and axonal fibers. Moreover, FA may be a predictor of PS.
Anisotropy* ; Axons ; Cell Membrane ; Diffusion Tensor Imaging* ; Diffusion* ; Edema ; Humans ; Stroke* ; Stroke, Lacunar