Munehiko Tomisawa

Accessory diaphragm: Report of a case associated with atrial septal defect

Summary An accessory diaphragm associated with atrial septal defect is described in a 6-yr-old boy. The patient was asymptomatic but showed chest x-ray abnormalities; accessory diaphragm associated with atrial septal defect was suspected on angiography and bronchography. Operation confirmed the presence of an accessory diaphragm and resection was successful.

Mucocutaneous lymph node syndrome presenting as acute cholecystitis

Mucocutaneous lymph node syndrome (MLNS) is not uncommon in Japan and it is well recognized that the dominant lesion is located in the coronary artery. We experienced recently a particular case which was diagnosed at first as acute cholecystitis, but a definite diagnosis of MLNS was later made from clinical manifestations and laboratory findings, especially ECG tracings, selective coronary angioram and selective celiac angiogram. We discussed the significant relationship between acute cholecystitis and MLNS.

Ultrastructural Observations of Functional Neural Tumor

We studied electron-microscopically the fine structures of sixteen masses of the functional neural tumor, consisting of 14 neuroblastomas and ganglioneuroblastomas, 1 ganglioneuroma and 1 pheochromocytoma. The ultrastructural findings of these tumors were compared with the observed values of the catecholamine content (norepinephrine and epinephrine) in these tumor masses and the urinary VMA (vanilmandelic acid) and HVA (homovanillic acid) levels in patients with these tumors. The cells of an undifferentiated type of neuroblastoma were revealed to be round or ovoid in shape and their nuclei occupied a greater portion of the cells. Cytoplasmic organelles were scarce. The cell of a differentiated type were observed to be larger and richer in cytoplasmic organelles than the undifferentiated cells. In the spaces of neuroblastoma cells, were often observed cellular processes, in which cored vesicles (osmiophilic granules) measuring about 1,000 A in diameter were found, but no typical neurofilaments and neurotubules were found. Schwann’s cell was not observed, either. In the cells of ganglioneuroblastoma, cytoplasmic organelles were well developed, and Schwann’s cells, similar to those found in ganglioneuroma cells, were sometimes detected. Large cored vesicles were numerous in cytoplasm and processes of the cell. Ganglioneuroma cells electron-microscopically resembled normal sympathetic ganglion cells. The cells and processes were always surrounded with Schwann’s cells. Pheochromocytoma cells resembled adrenal medullary cells in the ultrastructure. Their osmiophilic granules were observed to resemble those in the cells of the adrenal medulla, but they were fewer in number, and the size of the granules varied more widely. The relationship between the catecholamine content of these tumor tissues and the number of the cored vesicles in these tumor cells was investigated, but no apparent parallelism was found between them in these functional neural tumors. We thought that the catecholamine content of the tumor depended on the number of the cored vesicles in a tumor cell and the density of the cells in the tumor tissue. Therefore, we compared the number of vesicles with the catecholamine content only in tumor tissues of neuroblastomas in which the density of tumor cells was nearly the same. Consequently, the number of cored vesicles in the tumor cells was closely related to the catehcolamine content in tumor tissues. No correlationship was observed between the number of cored vesicles in the tumor cells and the urinary VMA and HVA excretion levels of the patients with neuroblastoma.

The study of Pulmonary Hypertension in Heart Diseases of Children

Considering the significance of the height of peak pressure (a) and dicrotic notch (c) of the main pulmonary artery, we regarded the main pulmonary artery as a single compression chamber, including both the left and the right main pulmonary branches, and we found that c/a in a congenital heart disease group with L-R shunt was larger than that in the control group. In order to find out the factors determining c/a, we injected intravenously phentolamineHC1 and observed its effect. Cia was higher in the QP/QS<P-group than in the QP/ QS 2 2-group The sympathetic vascular tonus took part largely in the QP/QS<2-group. We also found that the pre-loading value of cia was high in QP/QS22-group in spite of low reactivity to phentolamine-HC1. We think that it was caused primarily by the lowering of distensibility resulting from pulmonary dilatation.