Masashi Hokonohara

Clinical features of Japanese children and adolescents with systemic lupus erythematosus: results of 1980-1994 survey.

Marked advances have been made in the past decade in the management of adults with systemic lupus erythematosus (SLE). Therefore, a nationwide retrospective survey was conducted between 1980 and 1994 to investigate the clinical manifestations of SLE in Japanese children and adolescents. Questionnaires were sent to 340 hospitals. Of 405 patients reported by 176 hospitals, 373 patients, diagnosed by the criteria established by the Pediatric Study Group of the Japanese Ministry of Health and Welfare in 1985, were enrolled in the study. Forty‐nine of the 354 patients (13.8%) had relatives with a connective tissue disease within the third degree of consanguinity. The frequent manifestations in 373 patients were the presence of antinuclear antibody (98.9%), immunologic disorders (93.0%), hypocomplementemia (87.1%), malar rash (79.6%) and fever (74.0%). Lupus nephritis was present in 148 of the 309 patients (47.9%) at their first visit to a clinic, and 261 of the 373 patients (70.0%) developed renal involvement during the observation period. Of 370 patients, 92 patients (24.9%) exhibited central nervous system lupus. Of 368 patients, 192 patients (52.2%) were treated by methylprednisolone pulse therapy and 148 patients (40.2%) received immunosuppressants in combination with steroid therapy at some stage during the observation period. Survival rate at 5 years from onset was 95.9%. Management of infection, coagulopathies, and central nervous system involvement is essential to improve the prognosis of SLE in Japanese children and adolescents.

Impaired granulocyte chemotaxis and increased circulating immune complexes in Kawasaki disease

Our study was carried out to clarify the changes in granulocyte functions and circulating immune complexes in 32 children with Kawasaki disease. Patients were divided into two groups, those with or without coronary aneurysm. In the group with coronary aneurysm, impairment of both granulocyte chemotaxis and phagocytosis was found, together with higher circulating immune complexes and normal intracellular killing activity. In the group without coronary aneurysm, impaired phagocytosis was observed, with normal granulocyte chemotaxis, circulating immune complexes, and intracellular killing activity. No correlation was observed between granulocyte chemotaxis and circulating immune complexes. Impairment of granulocyte chemotaxis and circulating immune complexes may yield pertinent information as to the degree of severity of vasculitis in Kawasaki disease.

Diagnostic significance of increased serum hyaluronic acid in juvenile rheumatoid arthritis

Background : We have previously reported that serum levels of hyaluronic acid (HA) objectively reflect the severity of arthritis in juvenile rheumatoid arthritis (JRA). However, clear diagnostic standards do not exist for JRA; it is difficult to evaluate arthritis in children, particularly in small children and the diagnostic criteria for JRA requires an exclusion of several diseases. Therefore, if a specific test finding associated with JRA could be established, it would enable general pediatricians to make an objective diagnosis.

Acute rheumatic fever and poststreptococcal acute glomerulonephritis caused by T serotype 12 Streptococcus

We present a rare case of a 10 year old Japanese boy with acute rheumatic fever accompanied with poststreptococcal acute glomerulonephritis. We isolated group A Streptococcus serotype T 12, a strain that was thought to be nephritogenic but not rheumatogenic, from throat culture. Although rare, physicians should be aware that acute renal disease may accompany rheumatic fever.

Scanning Electron Microscopic Studies on Streptococcal Aerosol Infection

The initial focus of streptococcal invasion and histological changes of rabbit tonsils infected by aerosolinduced streptococci was investigated by scanning electron microscopic studies of the free and fractured surface of the tonsils.

Experimental studies on the initial focus of invasion of group A streptococci.

Rabbits were infected with group A streptococci type 12 by an aerosol method, that seems to resemble closely the human natural infection, in order to investigate the mechanism of invasion, especially the initial focus of invasion of streptococci. Living streptococci and FITC-labelled streptococci were exposed to several groups of rabbits, which were sacrificed and divided according to the duration after exposure. The specimens of organs were examined by light microscopy, fluorescent microscopy, scanning electron microscopy (SEM), and transmission electron microscopy (TEM). Histologically, the adherence of streptocossi was found on the pharyngeal mucosa and tonsils and the mucocilial clearance in the larynx and the trachea. Phagocytosis was also found in the bronchus and lung. By SEM, invading streptococci and cell debris-like masses were observed in the micropore of the tonsillar crypt of the rabbit. From these results it was supposed that there should be differences of tissue tropisms and defence mechanisms against streptococci among the mucosa and that micropores of the tonsillar crypts should play an important role in the initial focus of invasion of group A streptococci.

Transmission Electron Microscopic Studies on StreptococcaI Aerosol Infection

To investigate the initial focus of streptococcal invasion, aerosol‐induced infection in rabbits that resembled natural infection was examined by transmission electron microscopy (TEM). Group A type 12 streptococci were adjusted to a concentration of 108‐109 colony forming units (CFU)/ml in saline. The rabbits were exposed to the aerosolized suspension for about two hours and sacrificed at two hours to 14 days after the exposure. Pharyngeal mucous membranes, palatine tonsils and tracheas were observed by TEM.

Quality of life and daily management of children with rheumatic disease

Appropriate management of the daily life of patients with rheumatic diseases is an important part of therapy for favorable results and a high quality of life.

Bone Evaluation by Microdensitometry in Patients with Juvenile Reumatoid Arthritis

To evaluate the hand bones of patients with juvenile rheumatoid arthritis (JRA) objectively, 30 patients with JRA were examined by microdensitometric analysis (MD). The results were as follows: 1) Among the nine indices of MD, %σGS/D, reflecting the bone mineral content, correlated well with the stage of JRA determined by roentgenological findings of the hand bones. 2) %σGS/D showed remarkable improvement after the remission of JRA. 3) Rheumatoid factor (RF) positive JRA showed statistically low %σGS/D compared with RF negative JRA, and kept a low %σGS/D for a long period from the onset in contrast to RF negative JRA. 4) %σGS/D in the patients with JRA treated with steroid decreased more rapidly according to the dosage of steroid than in the patients with SLE. Based on these results it is suggested that MD seems to be useful in evaluating the bone objectively, and not only steroid but also RF seems to be a factor leading to bone destruction in patients with JRA.