PLASMA RENIN ACTIVITY AND ALDOSTERONE CONCENTRATION IN NORMAL SUBJECTS AND PATIENTS WITH SALT-LOSING TYPE OF CONGENITAL ADRENAL HYPERPLASIA DURING INFANCY

Plasma renin activity (PRA) and aldosterone concentration (PAC) were determined in normal infants aged 20 days to 1 year as well as in normal neonates and older children. The responses of PRA and PAC to five intramuscular injections of synthetic ACTH-Z at 6-hour intervals while on replacement cortisol therapy were also studied in nine infants with salt-losing type of congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH) of varying severity, using the data obtained in normal infants as control. The mean PRA and PAC decreased with age from the neonatal period, but in infants aged 20 days to 3 months these variables remained as high as those in newborns. They were also remarkably higher in infants aged 4 to 12 months than in older children. In two cases of CAH (cases 1 and 2) where salt wasting symptoms had been only transiently observed during the neonatal period, PAC was markedly elevated in response to ACTH administration, while in the remaining seven infants with CAH (cases 3–9) with persistent salt wasting symptoms, PAC was not elevated at all or only slightly so. PRA, on the other hand, increased with time during the ACTH stimulation in cases 3–9, while it increased and then decreased in cases 1 and 2. These findings suggest that the salt-wasting symptoms in CAH may arise where there is a tendency toward renal salt loss due to the overproduction of ACTH-dependent steroids characteristic of this disease, and cannot be compensated for by sufficient secretion of aldosterone. In cases 3–9, the maximal PAC under the ACTH stimulation was 17–44 ng/dl. These levels are comparable to or even higher than the normal range for control older children aged 2–8 years, but the levels were inappropriately low for the marked elevation of PRA in all of them when compared with the mean level in normal infants. Therefore, these data also seem to explain at least in part the well known clinical fact that the salt wasting symptoms in CAH are severe during infancy, requiring the administration of mineralocorticoids in addition to cortisol. However, beyond infancy only the replacement cortisol therapy is necessary to remit the symptoms.     

Non-effective Group with L-DOPA on Parkinsonism

(L-DOPA was supplied from Kyowa Hakko Co., Ltd. and from Dai-Ichi Pharmaceutical Co., Ltd.) An effectiveness of L-DOPA on 41 cases; 39 with idiopathic parkinsonism and 2 with olivopontocerebellar atrophy with parkinsonism, was observed. A comparison was made on several factors between 10 markedly and 9 non-effective cases. It was found that there is a relationship between the effectiveness of L-DOPA and an appearance of pyramidal signs which observed in all non-effective whereas only in two out of 10 markedly effective cases. Discussions were made on the idiopathic parkinsonisms which accompany pyramidal signs and are refractive to L-DOPA.     

Effect of Arterial Infusion of Bleomycin on Esophageal Carcinoma -- An Evaluation by Nuclear Cytophotometry --

In order to determine the histological changes after a singleinfusion of bleomycin (BLM) into the aortic esophageal artery(BLM A.I.), resected specimens from 58 patients with esophagealcarcinoma (27 cases with BLM A.I., 14 untreated cases, 13 caseswith radiation therapy and four cases with combined therapyof radiation and BLM) were examined and evaluated by Feulgenmicrospectrometry for nuclear deoxyribonucleic acid (DNA) content.The histological changes following the BLM A.I. were characterizedby degeneration and necrosis at the front of the invading carcinomatissue accompanied by inflammatory cell infiltration with foreignbody giant cells and fibrosis. In comparison with untreatedcases, BLM-treated cases showed an increase in nuclear DNA contentand a wide dispersion of the DNA values especially at the perinecroticarea and at the front of advancing carcinoma nests. Therefore,a histological effect of the BLM A.I. was shown by the measurementof the nuclear DNA content of carcinoma cells.     

Dynamic Changes of 12-Lead Electrocardiograms in a Patient with Brugada Syndrome

Dynamic ECG Changes in Brugada Syndrome. We present a patient with Brugada syndrome in whom 12-lead ECXis were recorded just before and after an episode of ventricular fibrillation (VF). A progressive elevation of both the RS-T segment and J waves just preceding and following the VF, and a close relationship between the amplitude of the RS-T segment and the preceding R-R intervals during atrial fibrillation, were documented. These findings support the hypothesis that RS-T elevation and a subsequent VF are related to a transient outward current-mediated spike-and-dome morphology of the epicardial action potential.     

Case Report: Primary splenic non-Hodgkin's B cell lymphoma in a patient with chronic hepatitis C

A case of primary splenic lymphoma in a patient with chronic hepatitis C is reported. A 69-year-old man with chronic hepatitis C was admitted to Fukuoka City Hospital for evaluation of an enlarging splenic tumour. In the spleen, ultrasonographic examination revealed a hypoechoic tumour and computed tomography demonstrated a non-enhancing low density area measuring 7 cm in diameter; coeliac angiography revealed a hypovascular tumour. Gallium scintigraphy showed uptake of the radioisotope in the splenic tumour. A splenectomy was performed and the morphological and immunohistochemical findings of this tumour were compatible with those of non-Hodgin's B cell lymphoma. Recently, cases of malignant B cell lymphoma associated with hepatitis C virus infection have been reported. Lymphotropism of hepatitis C virus may play a pathological role in the development of non-Hodgkin's lymphoma. We emphasize the importance of considering lymphoma in the differential diagnosis of extrahepatic disorders during the course of chronic hepatitis C virus infections.