Summer camp.

The first summer camp in Japan was held at a site near Tokyo in 1963. The guide book was edited by Japan Diabetes Society in 1984. In 1992, 1012 children with diabetes participated at 36 sites all across Japan. The programme is characterized by the smooth combination between education for the children and their experience of recreation. The term of the camp, staff member issues, camp operating fund are discussed. A pan-pacific international diabetes summer camp was successfully held at the Ehime prefecture site in 1989 with 56 patients from eight countries. The summer camp is evaluated for the achievement of good psycho-physical growth of the participating children and the maintenance of good control of diabetes. The problems include the term length of the camp, finding a camp site and the national and local governmental subsidy. Finally, a more effective operation with a minimum number of educators should be another concern in the future.

A multicenter study on HLA and autoimmunity in Japanese patients with early-onset insulin-dependent diabetes mellitus (IDDM): the JDS Study.

The Japan Diabetes Society (JDS) conducted a multicenter study on HLA and autoimmunity in Japanese patients with early-onset insulin-dependent diabetes mellitus (IDDM). HLA, immunoglobulin heavy-chain complex (Gm), properdin factor B (BF), and glyoxalase of erythrocytes (GLO) were typed, and organ-specific autoantibodies including islet cell antibodies (ICA) were assayed in 159 IDDM patients and their relatives and in 258 healthy Japanese subjects. The HLA-DRw9 phenotype and HLA-Bw61/DRw9 haplotype were significantly increased among the patients with autoantibodies other than ICA, whereas the DR4 phenotype and Bw54/DR4 haplotype were significantly increased in those without the autoantibodies. The DR4 phenotype was significantly increased in the patients with autoimmune thyroid diseases. The relative risk of the HLA-DRw9/DR4 genotype was highest among all DR genotypes. The Gm phenotype of g and gft were significantly increased in the patients with the autoantibodies. The BF-F phenotype was significantly decreased in the patients either with or without the autoantibodies. There was no association of GLO types with IDDM. The prevalence of ICA among IDDM patients was decreased with duration of IDDM. No significant association was found between the prevalence of ICA and sex, age at onset, or HLA type. On the other hand, the prevalence of the autoantibodies was not significantly changed with duration of the disease, and was significantly higher in females than in males.

ICA and organ-specific autoantibodies among Japanese patients with early-onset insulin-dependent diabetes mellitus--the JDS study.

The Japan Diabetes Society (JDS) conducted a multicenter study on the immunogenetics of insulin-dependent diabetes mellitus (IDDM) among Japanese. The previous report of the JDS study described HLA types and other immunogenetic markers in Japanese patients with IDDM. In the present report, the autoimmunity of Japanese patients was studied by measuring ICA and other organ-specific autoantibodies in patients with different durations of IDDM. The prevalences of ICA were the highest in the first year after diagnosis (73.1%) and decreased to 58.0%, 18.3% and 2.8% in 1-5 years, 5-10 years and 10 years or more after diagnosis, respectively (P < 0.01), while the prevalences of the other organ specific autoantibodies increased gradually with duration of IDDM from 20% in the first year to 35% in 10 years or more after diagnosis (P < 0.05). There were no sex differences in the prevalences of ICA but those of other organ-specific autoantibodies were significantly higher in female patients than in male patients (P < 0.01). The prevalence of ICA was not correlated with sex, age at onset or HLA types. In one of the subjects, a girl, the titers of ICA increased in parallel with a decrease in insulin secretion before the development of overt IDDM and declined thereafter. These findings suggest that IDDM might develop when the autoimmunity specific to pancreatic islets is triggered in people with underlying autoimmunity as shown by the presence of organ-specific autoantibodies other than ICA.

Immunogenetics of early-onset insulin-dependent diabetes mellitus among the Japanese: HLA, Gm, BF, GLO, and organ-specific autoantibodies--the J.D.S. study.

The Japan Diabetes Society (JDS) conducted a multicenter study on the immunogenetics of early-onset insulin-dependent diabetes mellitus (IDDM) of the Japanese. Human leukocyte antigen (HLA), properdin factor B (BF), immunoglobulin heavy-chain complex (Gm), and glyoxalase of erythrocytes (GLO) were typed, and organ-specific autoantibodies, including islet cell antibody (ICA), were assayed in 159 Japanese IDDM patients and their family members and in 258 healthy Japanese controls. The HLA-DRw9 phenotype and HLA-Bw61/DRw9 haplotype were significantly increased among the patients with autoantibodies other than ICA but with no autoimmune diseases (RR = 5.84, cP less than 0.001; and RR = 7.45, P less than 0.001), whereas the HLA-DR4 phenotype and HLA-Bw54/DR4 haplotype were significantly increased in those without either the autoantibodies or autoimmune diseases (RR = 2.64, cP less than 0.001; and RR = 4.55, P less than 0.001). The HLA-DR4 phenotype was significantly increased in the patients with autoimmune thyroid diseases (RR = 6.21, cP less than 0.05). In all groups of patients, the HLA-DR2 phenotype was significantly decreased, and the relative risk of the HLA-DRw9/DR4 genotype was highest among all HLA-DR genotypes. No significant association was found between HLA type and the duration or incidence of ICA. Gm types of g and gft were significantly increased in the patients with the autoantibodies (RR = 2.11, P less than 0.05; and RR = 34.11, P less than 0.05), whereas the BF-F phenotype was significantly decreased in the patients either with or without autoantibodies (RR = 0.43, P less than 0.05; and RR = 0.46, P less than 0.05). There was no association between IDDM and GLO type. These data indicate that immunogenetic bases underlying IDDM of the Japanese are heterogeneous, as are those in Caucasians.

Familial predisposition of type 1 diabetes mellitus in Japan, a country with low incidence. Japan Diabetes Society Data Committee for Childhood Diabetes.

The study group of the Japan Diabetes Society (JDS) carried out nationwide hospital-based and population-based surveys of childhood type 1 diabetes mellitus (DM) in Japan. According to the nationwide population-based survey, the incidence of childhood type 1 DM in Japan was 1.5 (1.4-1.6)/10(5), which did not differ for the 5 years from 1986-1990. Predisposition for DM and autoimmunity were studied in the first-degree relatives of the patients, including older and later cohorts. The prevalence of type 1 DM was 3.3% (12/369) among siblings and 2.2% (8/369) among parents, while the prevalence of type 2 DM was 0% among siblings and 4.3% (16/369) among parents. The risk of type 1 DM among siblings of the patients was 330 times higher than that among the general population in the Japanese population. The rate of positivity for autoantibodies, including ICA, IAA, GAD and IA-2, was 1.4-2.9% in parents (n=140) and 2.0-3.9% in siblings (n=203). The genetic susceptibility for type 1 DM is far lower in Japanese children than in Caucasian children, but predisposition to the disease and positive autoimmunity are almost the same in Japanese families of patients as in Caucasian families. The quality of life of Japanese parents of children with type 1 DM was less satisfactory that that of the Caucasian parents previously reported, which might be a result of the low incidence of type 1 DM in Japan.

Diurnal variation and increase of disaccharidase activity in diabetic rats.

The small intestinal disaccharidase activity and its daily variation in the diabetic rat have not been well described. Therefore, the small intestinal disaccharidase (maltase, lactase and sucrase) activity and its daily profile were studied in streptozotocin-induced diabetic rats under physiological conditions. In diabetic rats, a similar pattern of diurnal variation of disaccharidase activity to control rats was observed, while the relationships between daily change of disaccharidase activity and that of food consumption suggested that there was a different mechanism of diurnal variation in diabetic rats. On the other hand, a significant increase of mean 24-h lactase and sucrase activities was noted in diabetic rats, while that of maltase was not significant. Using the in vitro incubation method, a significant correlation between glucose concentration and lactase or sucrase activity but not maltase activity was observed. However, insulin showed no effect on disaccharidase activity. Thus we clarified the presence of a diurnal variation of disaccharidase activity and an increase in its activity in diabetic rats. This change was suggested to be derived from high plasma glucose level.

Nutritional factors for longevity in Okinawa--present and future.

Several factors, such as environment and heredity, are presumed to be related to longevity. Of these nutrition is believed to function as a regulatory factor. Okinawa prefecture is well known as the leading area for longevity in the world. We therefore examined present and past nutrition records together with the background of all the 88 centenarians (18 male, 70 female) who are living in Okinawa in 1991. Their leading occupation was agriculture, and they were in work until the 8th decade. They took rice or potato as carbohydrate with abundant vegetables and vegetable protein or fish protein. Although they did not take a rich diet it was well balanced, and was assumed to be related to longevity because of the decreased incidence of atherosclerosis; together with a good genetic background, suggested by the accumulation of longevity in their siblings.

Long term prognosis: juvenile onset IDDM in Japan.

The incidence of IDDM in children in Japan was found to be approximately 6 out of 100,000 in the child population of 6 to 15 years old. This prevalence is very low compared with those in Caucasian countries. Therefore, the history of management of childhood diabetes in Japan is short compared with that of western countries. Three studies which were carried out in Japan are reported and discussed in this report. The complications and prognosis of Type-1 Diabetes in Japan concluded as follows, 1) The long-term outcome of childhood diabetes was very poor. 2) The prevalence of microvascular complications were strongly related to the age of patients and the duration of diabetes. 3) The prevalence of retinopathy was dependent on the degree of diabetic control. But now, in Japan, we are using the intensive insulin therapy universally for childhood diabetes, and during recent ten years, the management and education of patients progressed rapidly. Therefore, the prognosis of childhood diabetes in Japan will improve.

[An autopsy case of AIDS with disseminated cytomegalovirus infection and neurological disturbance].

We report a case of acquired immunodeficiency syndrome (AIDS) complicated by disseminated CMV infection and neurological disturbance. A 21 years old male with hemophilia A was diagnosed as having AIDS in Feb. 1986 because of interstitial pneumonia and esophageal candidiasis. Since Jan. 1987 he had complained of hypesthesia in the legs. On Mar. 14 he was admitted due to diarrhea. The laboratory data revealed that WBC was 4,000/microliters including 29% of lymphocytes, 1.6% of OKT4+-, 71.6% of OKT8+-lymphocytes, T4/T8 ratio 0.02 and positive HIV antibody and HTLV-1 antibody. After the admission, sensory disturbance exacerbated to complicate paraplegia. He developed acute hepatitis associated with leukopenia, thrombocytopenia, pneumonia and melena, and eventually died on May 29. The autopsy findings disclosed CMV infection in the lungs, colons, and adrenal glands, suggesting that the primary cause of death was adrenal insufficiency. Degeneration of cerebro-spinal nerve cells and peripheral neuritis were thought to result from direct HIV infection to the nervous system.

[Adult T-cell leukemia with massive melena due to marked gastrointestinal involvement].

A 60-year-old man born in Okinawa was admitted to our hospital because of epigastralgia. Physical examination revealed general lymphadenopathy, mild hepatomegaly and skin eruption. The peripheral blood leukocyte count was 168,600/microliters, with 93% abnormal lymphocytes showing convoluted or lobulated nuclei. Anti HTLV-1 antibody was positive with titer of 1: 1280 (PA). Leukemic cells had typical ATL cells' surface markers (OKT3; 97.2%, T4; 93.3%, T8; 2.8%, OKIA1; 39.6%, IL-2R; 41.8%) and complete monoclonal HTLV-1 provirus DNA. Endoscopic examination with biopsy revealed massive involvement of ATL cells into gastric mucosa. In the course of the treatment, he had extremely massive melena, and was saved by emergency operation. Multiple ulcers were found in the resected colon. Histological examination showed the marked infiltration of the ATL cells into the mucous or submucous membrane. Thereafter, he was treated well with ALG (Anti Lymphocyte Globulin), until hypercalcemia occurred. He died of acute renal failure after hypercalcemia.

[An elderly case of idiopathic retroperitoneal fibrosis].

An elderly case of idiopathic retroperitoneal fibrosis (IRPF) with bilateral ureteral obstruction was reported. A 74-year-old man was admitted with complaints of general fatigue and loss of appetite. An elastic hard mass with a smooth surface was palpated in the left upper quadrant of the abdomen. Abnormal ESR and CRP were noticed. Abdominal ultrasonography showed both a homoechoic mass which surrounded the aorta in the retroperitoneal space and bilateral hydronephrosis. Computed tomography revealed a retroperitoneal mass involving bilateral ureters. Magnetic resonance imaging demonstrated a mass with a sharp image and signal intensity. Furthermore the morphological relationship between the mass and the ureters and major vessels clearly indicated the characteristics of benign retroperitoneal fibrosis. Based upon the above findings, a diagnosis of IRPF was made. After confirming the histological diagnosis by biopsy, treatment consisting of bilateral ureterolysis, intra-abdominal transposition of ureters and oral administration of prednisolone was performed, resulting in a normalization of laboratory findings. Analysis of 86 previously cases of IRPF reported up to 1990 in Japan, revealed the peak age to be in the 7th decade with predominance in males (males: females = 57:29).