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Biomedical subjects

K Iinuma

Publications and source records attributed to K Iinuma.

243 records · Page 14Linked to original sources

The histology of the lung in neonates with tricuspid valve disease and gross cardiomegaly due to severe regurgitation.

Lung autopsies from four neonates with Ebstein's anomaly or tricuspid valve dysplasia and gross cardiomegaly were examined. The mean cardiothoracic ratio was 92 +/- 0.5%. The degrees of pulmonary hypoplasia and pulmonary artery muscularization were assessed and were compared with those in lungs from four controls and four patients with diaphragmatic hernias. Lung and body weight ratios and radial alveolar counts, which reflect pulmonary hypoplasia and immaturity, were significantly decreased only in patients with diaphragmatic hernia. The thickness of the medical muscle layer in small pulmonary arteries was greater in patients with diaphragmatic hernia; however, in patients with tricuspid valve disease, it was relatively small. Abnormal vascular muscle extension was seen only in patients with diaphragmatic hernia. In the cases of tricuspid valve disease, although cardiomegaly and lung compression were severe, lung hypoplasia and immaturity were not, and neither abnormal medial thickening nor extension were found. Our results suggest that, at least in full-term infants with tricuspid valve disease, surgical relief of lung compression may improve respiratory function, even if the cardiomegaly is severe.

Birth Weight↗

Cytoplasmic inclusion bodies and minimal hepatitis: fibrinogen storage without hypofibrinogenemia.

A 12-year-old Japanese boy had chronic elevation and fluctuation of serum transaminase levels since infancy, with no signs or symptoms of liver failure. Usual infections or metabolic disorders were eliminated from consideration. No coagulopathy or abnormality in plasma concentrations of clotting factors was found. Light microscopy of liver biopsy specimens obtained at ages 2, 5, and 7 years showed slight hepatocyte disarray and minimal mononuclear-leukocyte lobular inflammation, with eosinophilic inclusion bodies in the cytoplasm of hepatocytes throughout the lobule. These bodies stained with the periodic acid-Schiff (PAS) technique; the PAS-positive material was partly diastase digestible and on immunostaining marked for fibrinogen but not for alpha 1-antitrypsin. On transmission electron microscopy, the bodies were represented by finely granular material contained within membranes and were interpreted as tentatively endoplasmic reticulum. Fibrinogen storage may be manifest as minimal hepatitis without coagulopathy.

Afibrinogenemia↗

Cerebral glucose metabolism in five patients with Lennox-Gastaut syndrome.

Regional cerebral glucose metabolic rates were estimated by positron emission tomography, in parallel with electroencephalography and cranial computed tomography in 5 patients with Lennox-Gastaut syndrome. The 5 patients, 3 boys and 2 girls, ranged in age from 10-15 years. Computed tomography revealed no gross abnormalities. Each patient received 2-5 mCi of 2-(18F)-fluoro-2-deoxy-D-glucose (18F-FDG) intravenously. Averaged cerebral glucose metabolic rates were reduced in each cerebral region as compared with controls. Unilateral hypometabolism was present in 4 patients: one in the inferior frontal gyrus as well as the posterior portion of the superior temporal gyrus; one in the inferior frontal gyrus; one in the posterior portion of the superior temporal gyrus; and one demonstrated diffuse hemispheric hypometabolism including the inferior frontal and posterior portion of the superior temporal gyrus. The side of hypometabolism was the same as the epileptogenic focus on the electroencephalogram. No focal changes were demonstrated on the electroencephalogram of a patient whose positron emission tomography revealed hemispheric hypometabolism. Hypometabolism of the inferior frontal and posterior portion of the superior temporal gyrus may relate to the possible pathogenesis of Lennox-Gastaut syndrome. Positron emission tomography has the potential to reveal a latent focal or lateralized abnormality in some patients with non-localized electroencephalographic changes.

Blood Glucose↗

Cytochrome C oxidase-deficient mitochondria in mitochondrial myopathy.

Electron microscopic cytochemistry was used to evaluate the behavior of cytochrome c oxidase (COX) in cultured skin fibroblasts from 4 patients with decreased COX activity (Leigh encephalopathy, fatal infantile COX deficiency). In patients with Leigh encephalopathy, all mitochondria reacted to COX staining either equivocally or negatively, indicating that all mitochondria were abnormal in these patients. In 1 patient with fatal infantile COX deficiency, intercellular heterogeneity of mitochondria was observed by COX staining. In another patient with fatal infantile COX deficiency, intracellular heterogeneity of mitochondria was observed. Patients with Leigh encephalopathy appeared to have a different type of mitochondrial COX deficiency than those with fatal infantile COX deficiency. Our result suggest that these 2 diseases may result from different genetic mechanisms.

Acidosis, Lactic↗

Quantitative measurement of volume flow rate (cardiac output) by the multibeam Doppler method.

A new method has been developed for measuring the volume flow rate of blood flowing through large vessels or outflow tracts of the heart. In this article we describe the principle of a method that can reduce the dependence of the Doppler angle of flow measurement by setting the sample points along a line to which every ultrasound beam is perpendicular. To evaluate the accuracy of this method, flow phantom experiments were made for both steady and pulsatile flows. The volume flow rate measured by this method agrees well with that observed by an ultrasound flowmeter (r = 0.99) when the vessel diameter is large (25 mm). However, this method overestimates by 40% when the vessel diameter is small (8 mm). To make this method applicable to small vessels, an improvement in the lateral resolution of Doppler measurement is necessary. It has been concluded that this method can be used to measure the cardiac output or volume flow rates in large vessels.

Algorithms↗

Visual abnormalities and occipital EEG discharges: risk factors for West syndrome.

Seventeen infants (7 boys and 10 girls) with visual abnormalities (such as poor tracking and following and nystagmoid eye movement) and occipital slow activities with irregular polyspikes on EEG were studied. Two thirds of the children showed development of West syndrome with hypsarrhythmia on follow-up EEG. In 1 child, hypsarrhythmia was evident on the follow-up EEG, but no clinical seizures occurred. Because most of the children in our series developed West syndrome, patients with visual abnormalities associated with occipital EEG discharges in early infancy may be at high risk for West syndrome. Occipital abnormalities in early infancy may relate to the occurrence of West syndrome.

Child, Preschool↗