Changes in the serum creatine kinase (CK) level according to the disease course were regressed to an exponential function Y = A e^-ex in each case of 28 Duchenne muscular dystrophy (DMD) through a long-term follow-up study. The regression to this formula implies that DMD muscle would decay by the leaking of a certain amount of CK into the circulation in proportion to the existing muscle. The diminution coefficient "r" was significantly reduced in puberty as compared with ages around ten. The cases of rapid progression showed a high rate of CK decrement in puberty. The diminution rate of CK is suspected to reflect the difference between the rate of muscle growth and primary muscle decay.

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In a study using a stop-flow technique in dog kidney, the existence of kalikrein and kinin was recognized in distal tubules. The presence of kininase I was seen in both distal and proximal tubules, and also partly in the distal tubules. The presence of kininase II in the distal tubules was again confirmed by pretreatment with SQ14225. No evidence of kinin formation, however, was obtained in the proximal nephrons in stop-flow method. From these results, it was suggested that kininase I and II localized in proximal tubules may destroy the kinin filtered from glomeruli at the proximal level, while kallikrein and kininogen and also kininase I and II in the distal tubules may regulate the activity of the renal kallikrein-kinin system in the distal nephrons.

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Using [125I] bTSH, labeled TSH binding (LTB) in sera from 203 patients with various thyroid disorders was studied. Four of them with known potent anti-TSH antibody showed extremely high LTB, as has been reported previously. Excluding these 4 sera, the mean ± s.d. of serum LTB from 199 patients was calculated to be 8.6 ± 2.1%. LTB exceeding 10.7% (mean + 1 s.d.) was observed in 16 sera; these weretaken as increased. LTB measured by polyethyleneglycol (PEG) precipitation correlated significantly with the serum IgG concentration; however, sera with increased LTB had high values irrespective of the serum IgG concentration. Specificities of increased LTB in 13 sera were further analyzed by means of binding to Protein A-sepharose and displacement studies using bTSH and nonradioactively iodinated bTSH. A significant correlation was observed between the LTB obtained by PEG and those by Protein A-sephrose. BTSH specificity was confirmed in 5 of the 13 sera; 7 of the remaining 8 sera showed displacement only by iodinated bTSH. None of the control Graves' sera showed any significant displacement. Comparisons of the results of measurement of LTB by Protein A-sephrose and those by the displacement studies disclosed that most of displaced sera had increased LTB to the IgG fraction. Disease distributions of 203 overall cases and 20 increased LTB cases revealed that apparently higher incidence (22.9%) of increased LTB in untreated Graves' patients than the others, though some increased LTB cases were also observed in patients with inactive Graves' or other thyroid disorders. In conclusion, increased LTB was observed in sera from approximately 10% of the patients with various thyroid disorders; most of them were found to be specific to either bTSH or iodinated bTSH. A frequent association of increased LTB with active Graves disease suggests a significance of these antibody like products to the disease process.

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Plasma retinol was determined by reverse-phase high-performance liquid chromatography in healthy subjects and patients with digestive diseases. The plasma retinol in the healthy control was independent of age (80.5 ± 17.16 μg/dl). On the other hand, the amount of retinol in patient groups was lower than in the healthy control group. It was 30.96 ± 7.16 μg/dl in patients with liver cancer, 38.02 ± 11.63 μg/dl in those with chronic pancreatitis and pancreatic cancer, 45.36 ± 12.63 μg/dl in a patient group with inflammatory bowel disease, 66.25 ± 7.27 μg/dl in the patients of early gastric cancer, and 66.44 ± 10.10 μg/dl in the group with biliary stone. These results and many biological aspects of retinol in human made it clear that, the special attention should be paid to the fluctuation of plasma retinol amount. This was considered to play an important role in causing complications associated with digestive diseases.

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Clinical trials of the hepatitis B vaccine developed in Japan were performed by the phase I, phase II and phase III studies. It was found that this vaccine did not only contained HBV itself but also other pathogens. It was ascertained to be adequately safe, since no major side effect was induced. After inoculation of the vaccine 3 times into 195 hospital employees, an HBs antibody seroconversion rate of 91.8% (97.0% in females and 86.3% in males) was obtained. In recipients aged more than 50 years, the rate was significantly lower. Although 8.2% of the recipients were non-responders to the vaccination, there was no significant correlation between the strength of the HBs antibody response and the lymphocyte subsets in the peripheral blood. It was observed, however, that among the non-responders, the incidence of low OKT4⁺/OKT8⁺ cell ratios tended to be somewhat increased. In follow-up studies of the antibody, majority of subjects, in whom the antibody level at 1 month after the 3 inoculations was below 50 RIA units, demonstrated negative conversion within 1 or 2 years. Namely, their value were reduced to below the minimal protective antibody value for prophylaxis of HBV infection.

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In order to clarify the mechanism of the hypotensive action of captopril, the acute and chronic effects of this drug on the kallikrein-kinin and renin-angiotensin systems were investigated respectively in 14 and 19 patients with hypertension. To determine the acute effect, a dose of 50 mg of captopril was administered once orally. For the chronic effect, 75-300 mg of the drug was administered daily for 14 days. In observations of the acute effect, blood pressure decreased significantly at 30 min. and maximally at 60-180 min. after administration with no change in heart rate. Significant increases in blood kinin levels and plasma renin activity (PRA), and a decrease in plasma angiotensin II levels were also observed. A marked augmentation was also found in urinary kinin excretion, but not in urinary kallikrein excretion. Moreover, the changes in blood pressure significantly correlated negatively with basal PRA, basal plasma angiotensin II and the changes in blood kinin levels, and positively with the changes in plasma angiotensin II. In our study of the chronic effect of captopril, similar changes in blood kinin levels, PRA, plasma angiotensin II levels, blood pressure and heart rate to the acute effect study were observed. Significant correlations of the changes in blood pressure were found negatively with basal PRA, basal plasma angiotensin II levels and the changes in blood kinin levels and positively with the changes in plasma angiotensin II levels. In addition, significant increases in urine volume and urinary sodium excretion occurred following administration of captopril for 14 days, and both increases negatively correlated with the changes in blood pressure. These findings suggest that the hypotensive effect of captopril might be caused, in part at least, by a decrease in plasma angiotensin II levels and an increase in blood kinin levels, and by augmentation of urine volume and urinary sodium excretion during long-term administration.

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We wished to investigate the pathophysiology of hypertriglyceridemia in chronic renal failure. Our subjects were 30 patients under hemodialysis for at least five years who had never taken vitamin D analogues or calcitonin preparations. During those five years, triglycerides were high, with no significant changes, although the cholesterol level decreased significantly 10 to 1 2 months after the first hemodialysis treatment. There was a significant correlation between alkaline phosphatase and triglycerides (p < 0.025) in these patients in the same assay. In the two patients with hypertriglyceridemia, serum triglycerides decreased to within the normal range by six months after parathyroidectomy, and remained there 30 months after surgery. These results suggested that secondary hyperparathyroidism maybe involved in the pathophysiological mechanism of hypertriglyceridemia seen in chronic renal failure.

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Natural killer (NK) cells have been implicated as first line of defence mechanism for carcinogenesis in humans. A lot of studies of depressed NK activity in patients with malignancies have supported this. Two major problems, however, in these studies are the choice of normal controls and target cells. To study this problem, peripheral mononuclear cells (PBMC) of sixty-six normal subjects from young and elderly, males and females were tested for NK function against twenty target cell lines with a microcytotoxicity assay. The result was shown that no sex or age difference existed with respect to NK function, except for a slight but significant decreased in NK activity of young female to K562 target cells. Target cells were divided into four groups by their NK sensitivity, namely, high, moderate, low and refractory sensitive. In general, NK activity of healthy persons is considered to remain stable and polyspecific in our results.

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Phytohemagglutinin-P (PHA-P) and concanavalin A (Con A) induced an increase in sialyltransferase (S-T) activity of the blood lymphocytes within 24 hours. PHA-P induced the maximal increase in S-T activity in the lymphocytes at 10 μg/ml and with Con A at 5 μg/ml. PHA-P induced a greater response than that by Con A. An additional effect of these mitogens was not observed. The exposure of cell extracts to these mitogens did not induce the increase in S-T activity. PHA-P or Con A had no effect on the induction of terminal deoxynucleotidyl transferase (TdT) activity, TdT-positive cells or peanut agglutinin (PNA)-positive cells from blood lymphocytes. Therefore, the change of the lymphocytes induced by these mitogens does not indicate a reversion to more immature state of lymphocyte differentiation, but represents a process of the functional differentiation to more activated lymphocytes. These mitogens had only a slight effect on S-T activity of human T-lymphoid leukemia cell lines. The decreased effects of the mitogens on S-T activity of leukemic cells may reflect the change of the membranes due to malignant transformation or the incomplete membranes tructure in undifferentiated leukemic cells.

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A rare instance of IgG-kappa monoclonal gammopathy in a patient with beta-thalassaemia trait is reported. The patient had a smoldering multiple myeloma with no apparent clinical symptoms pertaining to the disease and was a non-responder to conventional Melphalan and prednisolone therapy. Beta-thalassaemia trait was detected while investigating him for anaemia. A state of altered immunological reactivity, chronic infections and associated biliary tract disease are some of the pathogenetic mechanisms suggested. However in this patient none of these were operative.

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A 22-year-old female with Wilson's disease became pregnant and subsequently delivered a normal infant. At the age of 17 the patient presented evidence of liver cirrhosis and was diagnosed as having Wilson's disease. A regimen of d-penicillamine was started at this time and continued, although irregularly for the 10 months prior to her 9th week of pregnancy. At the 14th week of pregnancy she was started on 500 mg of D-penicillamine a day. She was stable during the remainder of her pregnancy and after delivery. Fetal development was normal and a normal infant was delivered at 35 weeks. It is suggested that pregnancy is not contraindicated in well treated Wilson's disease, and that a regimen of D-penicillamine during pregnancy can control the illness without harming the fetus.

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A 39-year-old male showed rapid progression of cerebral white matter involvement after 8 years' duration of adrenomyeloneuropathy. Increased ratio of C:26 to C:22 in the fatty acid composition of sphyngomyelin in the blood and needle-like cytoplasmic inclusions in the biopsied peripheral nerve were indicative of adrenoleucodystrophy or adrenoleucomyeloneuropathy. Plasma ACTH levels were extremely elevated, but most of ACTH immunoactivity was eluted at the molecular weight of 55000. Plasma cortisol levels were initially within normal range and were elevated in the final stage. Possible mechanism of elevated ACTH in this disorder was briefly discussed.

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Two autopsy cases of malignant lymphoma with marked hyperbilirubinemia (18.6 and 36.5 mg/dl at maximum) are presented. Histology revealed the portal area to be enlarged due to lymphoma cell infiltration and cholestasis in the hepatic lobules in both. Of 30 cases of malignant lymphoma treated in our department, hyperbilirubinemia was seen in 7 cases (23%) including these two cases (6.7%) which were severely jaundiced. The occurrence rate of jaundice in malignant lymphoma to all jaundiced diseases showing serum total bilirubin levels of more than 5 mg/dl was 0.7%.

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A patient of Hodgkin's disease with pulmonary involvement is described. Demonstration of Reed-Sternberg cells in the sputum confirmed the diagnosis of pulmonary involvement.

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A 89-year-old woman showed transient and mild hypercalcemia (11.0 mg/dl) at the phase of recovery from acute renal failure. Serum PTH level elevated to 2.1 ng/ml when hypercalcemia was observed, and decreased to 1.1 ng/ml. Autopsy study revealed the bleeding within parathyroid adenoma. The transient and mild hypercalcemia seen in the patient was considered as the result of the bleeding within parathyroid adenoma. This unusual manifestation of parathyroid adenoma, bleeding, usually causes hypercalcemic crisis. The mild hypercalcemia observed in this patient despite of bleeding into parathyroid adenoma might be due to old age of the patient and the existence of renal failure in the patient.

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A case of intravascular bronchiolo-alveolar tumor, a rare lung neoplasm which is often found among women, is reported. Our ultrastructural findings and existence of factor VHI-related antigen support the hypothesis of vascular origin. The relationship between IVBAT and epithelioid hemangioendothelioma is discussed. We also examined whether immunological abnormalities existed in our patient, because tuberculin test gave negative result in many cases reported previously and our case. No specific immunological change was found except for negative tuberculin test.

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