The purpose of this article is to review the current status of research on the nephrotic syndrome in Japan. In this country, there two study groups for the nephrotic syndrome and a systematic research is now under way. One of these study groups is the Research Council for the Treatment of Adult Nephrotic Syndrome in Japan, which initiated a study in 1968 mainly by investigators in the field of internal medicine, and the other is the Nephrotic Syndrome Research Committee based on the Research Grant for Specific Diseases from the Ministry of Health and Welfare. The latter group consists of researchers in the field of epidemiology, internal medicine, pediatrics and pathology, and it has conducted a continuous study from 1973.
From abundant research activities of these two groups, I wish to describe here only the epidemiological and therapeutic aspects of the nephrotic syndrome in Japan. In order to provide as objective and accurate data as possible, these groups established the diagnostic criteria (Table 1).

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The kidney and intestine are considered to hold definite positions in the bilirubin metabolism, especially during obstructive jaundice. In this study, renal and intestinal cytoplasmic binding proteins of organic anions, as well as hepatic ones, were analyzed by Sephadex G 75 column chromatography, using 131-I sulfobromophthalein. The enzyme activities of glutathione S-transferases in those organs were also measured using di-nitrochloro- benzene as a substrate, since some of the cytoplasmic binding proteins are glutathions S-transferases synonyms. Hepatic, renal and intestinal ligandins were compared, by these methods, with each other between two groups: control and bile duct ligated rats. In the bile duct ligated rats, the binding increased in the kidney, and remained unchanged in the intestine and liver. But enzyme activity had a tendency to decrease in all the three organs.

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In 25 of 44 patients with acute leukemia admitted to our clinic during the 3-year period ending in 1975, 32 episodes of septicemia in total were found. Septicemia developed in connection with severe granulocytopenia either during remission-induction or at hematologically terminal stages. Multiple organism septicemia was seen in 8 episodes at the terminal stages. The mortality was higher at the terminal stages than during remissioninduction. From the blood were isolated 44 strains of 11 species, of which about twothirds were distributed among 3 species: Escherichia coli, Klebsiella and Pseudomonas aeruginosa. Gentamicin was the most potent drug to gram-negative isolates. The majority of gram-negative septicemias were preceded by isolation of identical strains of the same species from surveillance cultures of the throat and stools. Most of the causative organisms had a close correlation with the preceding antibiotic treatment. The great serologic diversity of the isolates strongly suggested that most septicemias would indeed arise from the patient's ownflora. Serumantibodies to quite a numberof blood strains were demonstrated at the onset or 2 weeks later. Lesions on the respiratory and gastrointestinal tracts were inferred to have provided probable portals of entry for gram-negative bacilli. Thus, the importance of bacteriological surveillance of the endogenous flora was stressed to prevent and control gram-negative septicemia in acute leukemia.

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The relationship between the electrical impedance fluctuation (ΔZ) and the chest cage movement(ΔTD) associated with ventilation on either upper and lower zones of the hemithorax or the bilateral sides of the thorax were discussed in the range of the vital capacity which was measured by the conventional spirometry (ΔV) in fifteen normal adults. The ventilatory variation of the impedance in the two regions were smaller at the top and larger at the bottom. The dorso-ventral diameter changes, ΔZ/ΔV and ΔTD/ΔV had a similar tendency. Despite correlatibe change between ΔZ and ΔTD in the normal adults, the impedance fluctuated without evident TD change in the abdominal ventilation, and the chest cage moved without evident impedance change in the area under which thoracoplasty was carried out. These results suggest that the ventilatory impedance variation is evoked not by the chest wall movement but by distribution of the inspired air into the region under the electrode, and the impedance method may detect the regional ventilation evidently.

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A patient is reported with X-linked hypoxanthine-guanine phosphoribosyltransferase (HGPRT) deficiency. 1. He had a juvenile gout with excessive urate excretion but lacked any neurological symptoms usually present in the Lesch-Nyhan syndrome. 2. The patient had negligible HGPRT activity (0.07% of normal) in erythrocytes. 3. Autoradiography of fibloblast cultures revealed no uptake of ³H-hypoxanthine. The fibloblasts of his mother and two sisters were all mosaic in uptake of labelled hypoxanthine and shown to be heterozygotes. 4. This case is the first reported case of complete deficiency of HGPRT without neurological disorders, which is proved to be inherited as a X-linked recessive trait by an autoradiographic study of cultured skin fibloblasts of his family.

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An autopsy case of primary acquired sideroblastic anemia (PASA) is described. Cytogenetic studies showed an isochromosome 17 (17qi) not only in diploid but also in octaploid cells from the bone marrow, whereas the karyo-type of peripheral lymphocytes was normal. Postmortem examinations revealed hyperplasia of the three myeloid cell lines in the bone marrow and extramedullary hematopoiesis in the liver and spleen, but no evidence of leukemia. The present case provides cytogenetic evidence that PASA is a disorder derived from an uncommitted stem cell mutation and is clonal in nature.

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