Clinical and pathological features of eight fatal cases with Sjögren's syndrome (all female, aged 43-61) were studied from the prognostic viewpoint. Chronic active hepatitis, primary biliary cirrhosis, diffuse interstitial pneumonitis and primary pulmonary hypertension were fatal illness in seven of them, and remaining one died of accidental infection. The clinical and pathological findings of these cases suggested that such hepatic or pulmonary diseases might be the manifestations of Sjögren's syndrome. Interstitial nephritis mayalso be a manifestation of the syndrome, possibly with a fatal outcome. Fromthese observations Sjögren's syndrome with liver, lung or kidney involvement may be a possible clinical type with poor prognosis.

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Changes in plasma cyclic AMPinduced by glucagon injection was investigated in 9 healthy controls, 18 patients with acute hepatitis, 16 patients with chronic inactive hepatitis, 31 patients with chronic active hepatitis, and 29 patients with liver cirrhosis. A P/B ratio, calculated from the peak value of plasma cyclic AMPat 10 min after glucagon injections divided by the base value, wasfound to be a convenient index of the response to glucagon. Compared with the controls the ratio was significantly lower in liver cirrhosis and higher in chronic hepatitis. A hyperresponse of plasma cyclic AMPto glucagon was observed in acute hepatitis phase I (the stage of elevated serum GPT and GOT), however the response in acute hepatitis phase II (the stage of normalized serum GPT and GOT)was almost the same as that in controls. These results indicate that the glucagon test, using the P/B ratio, is useful for a diagnosis of the reserve functions of the liver disease. The drop in the P/B ratio during acute hepatitis suggests that the illness is on a normal recovery course.

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Selective aldosterone deficiency is a rare but well described entity characterized by reduced or absent aldosterone production with otherwise normal adrenal function. A middleaged Japanese womanwith such a deficiency is reported on. The evidence is in favor of an acquired defect of C-18 dehydrogenasing enzyme, the pathogenesis of which is probably due to the autoimmunemechanismbecause of the existence of autoantibodies against the adrenal cortex. Noteworthy in tjiis patient besides selective lack of aldosterone is that, Hashimoto thyroiditis and deficient states of growth hormone, insulin and probably adrenocorticotropin were present. The hyperkalemia presumably caused by selective hypoaldosteronism was corrected by small doses of 9α-fluorohydrocortisone. It is suggested that selective aldosterone deficiency should be considered in all cases with disturbances based on autoimmune mechanisms.

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A twenty-one-year-old male subject had been complaining of anemic symptoms and bleeding tendency of several years duration, and was incidentally found to have mild hepatomegaly at the age of nineteen. The clinical picture of aplastic anemia was associated with foam cells infiltrating into the bone marrow and lymph nodes, and peripheral leukocytes having vacuolization in the cytoplasm. A slight degree of mental retardation was noted, but otherwise normal neurologically. The characteristic foam histiocytes were filled with many PAS-negative granules which fluoresced strongly a greenish-yellow in ultraviolet light. However, biochemical analysis of lipids accumulated in the cytoplasm of foam cells remains to be conclusive. Total sphingomyelinase activity was slightly below that of normal control. These findings were compatible in every way with those described with adult Niemann-Pick disease. The relationship of foam cells to various lipid storage diseases, both inherited and acquired, was discussed.

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Acute intermittent porphyria (AIP) is linked to a genetic defect in uroporphyrinogen I synthase. In pursuit of the question whether this disturbance of heme synthesis is due to an abnormal quality or quantity of this enzyme, we purified uroporphyrinogen I synthase, separated into isoenzymes and tested the stability of them in five porphyric patients; three AIP and two porphyria cutanea tarda (PCT). Six isoenzymes were found in all these porphyric patients as well as in normals. No lack of the isoenzymes was found in AIP. In normals and in PCT, the isoenzymes with the highest percent activity were found to be the most labile ones, but this instability was even more pronounced in AIP patients. This suggests that the enzymatic defect in AIP is more likely a qualitative than a quantitative one, suggesting a structural gene defect.

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Furosemide is now commonlyprescribed for the patients with idiopathic edema and is known as a powerful stimulus to the renin-angiotensin-aldosterone system. We report here two patients with idiopathic edema who had episodes of furosemide-induced edema. In these patients exaggerated increase in the activity of reninangiotensin-aldosterone system was observed in response to the combined stimuli of furosemide administration and erect posture. This could be explained as a cause of fusosemide-induced edema. Therefore attention should be paid that administration of furosemide increases edema in somepatients with idiopathic edemaand makes the patients dependent on its continued administration.

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We recorded two cases of endocrine disturbance following tuberculous meningitis associated with intracranial calcification in the suprasellar region. Both patients showed hypogonadism, delayed bone-maturation, obesity, polydipsia and polyuria. A giant gynecomastia was seen in one of them. In discussion we considered the relation of gynecomastia and endocrinological disturbance following hypopituitarism. Prolonged administration of clomiphene citrate and synthetic LH-RHwas not effective in promoting sexual maturity in these patients.

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