Cytomegalovirus-associated hemophagocytic syndrome in a patient with adult onset Still's disease.

Reactive hemophagocytic syndrome (HPS) is characterized by hemophagocytosis by activated histiocytes, resulting in pancytopenia and liver dysfunction. We describe a patient with adult onset Still's disease (AOSD) in whom HPS developed. An 80-year-old Japanese woman with high fever, arthralgia, skin rash, and pleuritis was admitted to our hospital for further examination. She was diagnosed with AOSD and steroid therapy was initiated. During the course of steroid therapy, a re-elevation of serum ferritin levels and a marked increase in serum transaminase were observed. Bone marrow aspiration revealed an increase in the number of histiocytes with hemophagocytosis and cytomegalovirus (CMV)-positive leukocytes were detected. At this time we diagnosed the patient as having virus-associated hemophagocytic syndrome (VAHS) and elevated levels of trasaminase and ferritin were normalized by ganciclovir treatment. Reactive HPS occurs in cases of active AOSD. However, it should be noted that HPS may be accompanied by opportunistic infections during immunosuppressive therapy requiring prompt antibiotic therapy.

Thyroid lymphoma of mucosa-associated lymphoid tissue with monoclonal gammopathy occurring in an atomic bomb survivor: report of a case.

We describe herein a case of thyroid lymphoma of the mucosa-associated lymphoid tissue (MALT) with monoclonal gammopathy (IgG kappa), occurring in a woman who was exposed to atomic-bomb (A-bomb) radiation 1.5 km from the explosion of the hypocenter when she was 31 years old. Her estimated radiation dose was 0.2 Gy. A total thyroidectomy resulted in the disappearance of monoclonal gammopathy, thus indicating extramedullary monoclonal gammopathy of the thyroid. Three patterns of protein electrophoresis, i.e., normal, the appearance of monoclonal immunoglobulins, and their disappearance after the total thyroidectomy, were consecutively recorded. The utility of checking of the monoclonal immunoglobulin levels after a thyroidectomy due to lymphoma of the MALT to monitor recurrence is therefore suggested. This lymphoma is most likely not related to the patient's A-bomb radiation exposure.

Long-term effects of dietary alpha-linolenic acid from perilla oil on serum fatty acids composition and on the risk factors of coronary heart disease in Japanese elderly subjects.

Although important roles of dietary n-3 fatty acids in the prevention of coronary heart disease (CHD) have been suggested, long-term effects of dietary alpha-linolenic acid (ALA, 18:3n-3) have not yet been established under controlled conditions. We tested whether a moderate increase of dietary ALA affects fatty acids composition in serum and the risk factors of CHD. Oxidized LDL (OxLDL) was directly measured by ELISA using antibody specific to OxLDL. By merely replacing soybean cooking oil (SO) with perilla oil (PO) (i.e., increasing 3 g/d of ALA), the n-6/n-3 ratio in the diet was changed from 4:1 to 1:1. Twenty Japanese elderly subjects were initially given a SO diet for at least 6 mo (baseline period), a PO diet for 10 mo (intervention period), and then returned to the previous SO diet (washout period). ALA in the total serum lipid increased from 0.8 to 1.6% after 3 mo on the PO diet, but EPA and DHA increased in a later time, at 10 mo after the PO diet, from 2.5 to 3.6% and 5.3 to 6.4%, respectively (p<0.05), and then returned to baseline in the washout period. In spite of increases of serum n-3 fatty acids, the OxLDL concentration did not change significantly when given the PO diet. Body weight, total serum cholesterol, triacylglycerol, glucose, insulin and HbA1c concentrations, platelet count and aggregation function, prothrombin time, partial thromboplastin time, fibrinogen and PAI-1 concentration, and other routine blood analysis did not change significantly when given the PO diet. These data indicate that, even in elderly subjects, a 3 g/d increase of dietary ALA could increase serum EPA and DHA in 10 mo without any major adverse effects.

Prevalence rate of thyroid diseases among autopsy cases of the atomic bomb survivors in Hiroshima, 1951-1985.

To examine the radiogenic risk of latent thyroid cancer, thyroid adenoma, colloid/adenomatous goiter and chronic thyroiditis, the data for 3821 subjects collected in the course of autopsies of atomic bomb survivors in Hiroshima from 1951 to 1985 by the Radiation Effects Research Foundation (RERF) were analyzed using a logistic model. About 80% of the autopsies were performed at RERF and the remainder at local hospitals. The frequencies of the above diseases were not associated with whether the underlying cause of death was cancer. However, note that our results may be influenced by potentially biasing factors associated with autopsy selection. The relative frequency of latent thyroid cancer (greatest dimension < or = 1.5 cm but detectable on a routine microscopic slide of the thyroid gland) increased as the radiation dose increased and was about 1.4-fold greater at 1 Gy than in the 0-Gy dose group. The relative occurrence of thyroid adenoma also increased as radiation dose increased, and was about 1.5-fold greater at 1 Gy than in the 0-Gy dose group. Sex, age at the time of the bombing or period of observation did not significantly modify the radiogenic risks for thyroid adenoma or latent thyroid cancer. No statistically significant association was found between radiation exposure and the rates of colloid/adenomatous goiter and chronic thyroiditis. The possible late effect of atomic bomb radiation on the frequency of benign thyroid diseases is discussed on the basis of these data.

Colorectal cancer incidence among atomic bomb survivors, 1950-80.

Colorectal cancer incidence in the LSS sample during 1950-80 was investigated. A total of 730 incidence cases of colorectal cancer were confirmed from a variety of sources. Sixty-two percent of the cancers were microscopically verified and 12% were ascertained through death certificate only. The risk of colon cancer increased significantly with intestinal dose, but no definite increase of risk was observed for rectal cancer. Relative risk at 1 Sv and excess risk per 10(4) PY-Sv for colon cancer are 1.80 (90% confidence internal 1.37-2.36) and 0.36 (90% confidence interval 0.06-0.77) respectively. City and sex did not significantly modify the dose-response of colon cancer, but the risk decreased with age at the time of bombings (ATB). The relative risk of colon cancer does not vary substantially over time following exposure. A non-linear dose response did not significantly improve the fit. Further, the anatomic location of the tumors indicate that the cecum and ascending, transverse and descending, and sigmoid colon seem equally sensitive to radiation. No difference in the distribution of tumor histological types could be observed by radiation dose.

[Mitral valve replacement with concomitant coronary bypass for the papillary muscle rupture after acute myocardial infarction in situs inversus].

A 53-year-old man, who was known to have situs inversus totalis all of his life, had acute myocardial infarction complicated by partial rupture of the posterior papillary muscle causing mitral regurgitation and pulmonary edema. The patient underwent mitral valve replacement (Omnicarbon 27 mm) with concomitant aortocoronary saphenous vein bypass, and he is now doing well 9 months following the operation. To our knowledge, this is the first case of the successful mitral valve replacement and concomitant aortocoronary saphenous vein bypass on a patient with situs inversus totalis (mirror-image dextrocardia) in Japan.

Analysis of thyroid carcinoma based on material registered in Japan during 1977-1986 with special reference to predominance of papillary type.

BACKGROUND: As geographic differences have been observed in the characteristics of thyroid carcinoma, an analysis was made on thyroid carcinoma in the iodine rich country of Japan. METHODS: A total of 10,973 patients with histologically confirmed thyroid carcinoma registered in Japan from 1977-1986 were analyzed. Cases detected incidentally at autopsy and cases of nonepithelial tumor were excluded. This series included approximately 27% of all thyroid carcinoma cases in Japan. RESULTS AND CONCLUSIONS: Histologic distribution showed that papillary carcinoma accounted for 78.4% of cases, follicular carcinoma accounted for 17.2%, medullary carcinoma for 1.4%, squamous cell carcinoma for 0.3%, and anaplastic carcinoma for 2.7%. There is a tendency in Japan to diagnose papillary carcinoma as follicular carcinoma and to diagnose malignant lymphoma as anaplastic carcinoma. It was considered that the percentage of papillary carcinoma was higher and the percentage of follicular carcinoma and anaplastic carcinoma was lower than foregoing values. The characteristics of thyroid carcinoma in Japan were described, and the low incidence of nonpapillary carcinoma compared with papillary carcinoma was discussed in relation to iodine excess as an etiologic factor.

Hyperparathyroidism among atomic bomb survivors in Hiroshima.

To determine the effect of exposure to atomic bomb radiation on the occurrence of hyperparathyroidism, the prevalence was determined among a population of 3,948 atomic bomb survivors and their controls in Hiroshima. The diagnosis of hyperparathyroidism was based upon histopathological findings or the presence of consistent hypercalcemia and elevated levels of serum parathyroid hormone. Primary hyperparathyroidism was diagnosed in 19 persons (3 males, 16 females). Females had approximately a threefold higher overall prevalence of hyperparathyroidism than males (P less than 0.05). The prevalence rates of hyperparathyroidism increased with radiation dose (chi2(1) = 12, P less than 0.001) after adjusting for sex and age at the time of the bombing. The estimated relative risk was 4.1 at 1 Gy (95% confidence limits 1.7 to 14). There was some evidence that the effect of radiation was greater for individuals who were younger at the time of the bombing. In conclusion, exposure to atomic bomb radiation affected the occurrence of hyperparathyroidism, suggesting that doses of radiation lower than those used in radiotherapy may also induce this disorder.

[Four cases of spontaneous pneumothorax with no reexpansion of collapsed lung despite chest tube drainage].

We report four cases of spontaneous pneumothorax with no reexpansion of the collapsed lung despite chest tube placement and adequate suction. The cause of absence of reexpansion was endobronchial obstruction by bronchogenic carcinoma in one patient and by sputum plug in another. In the other two patients, the collapsed lung had pulmonary fibrosis and did not reexpand at all because of reduced pulmonary compliance.

[Effect of intravenous administration of coagulation factor XIII concentrate on persistent pleural air leak].

We administered coagulation factor XIII concentrate intravenously to six patients suffering from persistent air leak with no evidence of bronchopleural fistulae They are those who had either failed to be healed in response to pleurodesis, intrapleural fibrin glue injection and surgery or had not received such treatment. Their blood coagulation factor XIII activity levels were less than 70% of the standard plasma level. In four of the six patients, air leak stopped within 10 days after the treatment begun. Because factor XIII plays an important role in wound healing, reduction in the activity level can be a cause of insufficient healing of pulmonary surface fistulae and may lead to persistent air leak. In such cases, intravenous administration of factor XIII concentrate increases the activity levels and may put an end to persistent air leak.

[Respiratory surgery through a median sternotomy utilizing the IMA-retractor].

A new utilization of the IMA-retractor for respiratory surgery is described. Following linear division of the sternum, the IMA-retractor is applied to tip upward the sternal edge on the operated side. The IMA-retractor provides excellent exposure of the lung without the need for surgical assistance and greatly facilitates respiratory operations through the median sternotomy.

A review of forty-five years study of Hiroshima and Nagasaki atomic bomb survivors. Thyroid cancer: reports up to date and a review.

Evidence to date from a number of studies, indicates that there is an increased incidence of thyroid cancer among A-bomb survivors, and that the incidence rises with increase of dose and is especially predominant in females exposed at younger ages. Although 45 years have elapsed since A-bomb exposure, there are still some questions about the development of thyroid cancer: 1) Is the incidence now decreasing? 2) What factors, other than radiation exposure, are involved? 3) What is the incidence of multiple cancer, including thyroid cancer? With these questions in mind, it is apparent that studies in this field must continue for some time to come.

Thyroid cancer: epidemiological study of thyroid cancer in A-bomb survivors from extended life span study cohort in Hiroshima.

We analysed thyroid cancers which were diagnosed clinically or detected at autopsy during 22 years (1958 to 1979) in a sample of 75,493 study subjects exposed in Hiroshima belonging to the extended LSS sample of the Radiation Effects Research Foundation. One hundred and twenty-five cases of clinical thyroid cancer (15 in males and 110 in females) were confirmed, giving a crude incidence rate per 100,000 person-years of 2.7 for males, 12.4 for females and 8.6 for both sexes combined. There was a significant increase of thyroid cancer with increase of atomic bomb radiation dose (thyroid tissue dose based on T65D) in females and in the sexes combined. This tendency was predominant in those exposed at less than 19 years of age. Compared to the control group, the relative risk in the greater than or equal to 0.50 Gy group was higher at 4.0 for males (not significant) and at 4.3 for females (p less than 0.01). Latent thyroid cancer was detected in 155 cases or 3.5% (2.5% for males and 4.5% for females) of the 4,425 cases that came to autopsy during the same period. Compared to the control group, the relative risk in the 50+ rad group was 1.7 for males (not significant), 2.0 for females (p less than 0.05) and 1.9 for both sexes combined (p less than 0.05). New data obtained from autopsy cases between 1950 and 1985 have been added.

X-ray sensitivity of fibroblast cell strains derived from atomic bomb survivors with and without breast cancer.

Fibroblasts were established in vitro from skin biopsies obtained from 55 women and 1 man with or without breast cancer and with or without exposure to radiations from the atomic bomb (A-bomb) explosion in Hiroshima. The radiosensitivity of these cells was evaluated by clonogenic assays after exposure to X-rays. Dose-response curves were fitted to a multitarget model, S/S0 = A[1-(1-e-D/D0)N]. There was no difference in the means or variances of radiosensitivity between exposed and nonexposed groups, or between groups with and without breast cancer.

Radiosensitivity of skin fibroblasts from atomic bomb survivors with and without breast cancer.

Fibroblasts were established in vitro from skin biopsies obtained from 55 women and 1 man with or without breast cancer and with or without exposure to radiation from the atomic bomb explosion in Hiroshima. The radiosensitivity of these cells was evaluated by clonogenic assays after exposure to X-rays or to fission neutrons from a 252Cf source. Data were fitted to a multitarget model, S/S0 = A [1 - (1 - ekD)N], for both X-ray and neutron dose-survival curves. A single hit model, S/S0 = AekD, fits the neutron dose-survival responses as well. There were no differences in the means or variances of radiosensitivity between exposed and nonexposed groups or between patients with or without breast cancer. Hence, although the sample is not large, it provides no support for the hypothesis that atomic bomb radiation preferentially induces breast cancer in women whose cells in vitro are sensitive to cell killing by radiation.

A case of patent ductus arteriosus in an 80 years old woman.

A case of patent ductus arteriosus (PDA) in an 80 years old female is presented. This is the oldest case reported in Japan and the most elderly case of newly diagnosed PDA in the world. Features of PDA in the elderly are reviewed.