Masahiko Okuni

A nationwide surveillance study of rheumatic diseases among Japanese children.

To estimate the number of children with rheumatic diseases, a questionnaire was distributed to the pediatrics department of 1290 hospitals in Japan in June 1994. From this survey, 1606 cases with juvenile rheumatoid arthritis (JRA), 906 cases with systemic lupus erythematosus (SLE), 320 cases with dermatomyositis/polymyositis (DM/PM), 28 cases with scleroderma (PSS), 70 cases with Sjögrens syndrome (Sjs), 93 cases with mixed connective tissue disease (MCTD), 25 cases with aortitis syndrome, 20 cases with polyarteritis (PN) and 51 cases with Behçet disease were reported. The crude annual incidence rates per 100 000 among the childhood population were estimated as JRA, 0.83; SLE, 0.47; DM/PM, 0.16; PSS, 0.01; Sjs, 0.04; MCTD, 0.05; aortitis syndrome, 0.01; PN, 0.01; and Behçet disease, 0.03. The present study reveals that there are more children with rheumatic diseases than are estimated from the reported cases in the literature and the number of children who are receiving Assistance Medical Costs Insurance covered by the Japanese government.

Clinical analysis of 570 cases with juvenile rheumatoid arthritis: Results of a nationwide retrospective survey in Japan

The purpose of the present study was to investigate the incidence of juvenile rheumatoid arthritis (JRA) among Japanese children and to evaluate the clinical features of this disease. A questionnaire was sent to the department of pediatrics of 1290 hospitals in Japan, in 1994, asking for the number of rheumatic patients during the past 10 years. Subsequently, a second questionnaire was sent asking for the type of onset, clinical features, treatment, and other details. The results of 570 cases were obtained. Of these, 310 cases (54%) were the systemic onset type, 140 cases were the polyarticular onset type (25%), and 120 cases (21%) were the pauciarticular onset type. Hence, in the present series of children, the proportion of the pauciarticular type was less than the other two types of JRA. In the laboratory findings of the systemic onset type, hyperferritinemia and thrombocytosis were noted, in addition to leukocytosis, positive C‐reactive protein (CRP) and accentuated erythrocyte sedimentation rate (ESR). The rheumatoid factor was positive in 50% of patients with the polyarticular onset type. Chronic uveitis was recognized in 13 cases (10.8%) of the pauciarticular onset type. In four girls, uveitis started before the onset of arthritis. Nonsteroidal anti‐inflammatory drugs were used in almost one‐third of cases, and methotrexate (MTX) was used in 12.8% of cases. The quality of life of children with JRA was disturbed in almost 20% of cases. Therefore, for the early and definitive diagnosis of the systemic type of JRA, diagnostic procedures including thrombocyte counts and serum ferritin level, should be performed. In order to obtain good results and to avoid side effects, a protocol for the use of disease modifying anti‐rheumatic drugs and immunosuppressants, especially for the use of MTX, must be established.

AUDITORY EVENT‐RELATED POTENTIALS AND REACTION TIME IN CHILDREN EVALUATION OF COGNITIVE DEVELOPMENT

The authors studied age‐related correlations of auditory event‐related potentials (ERPs) using the conventional ‘oddball’ paradigm and reaction time in 175 normal subjects, aged between four and 21 years. From four years, the latencies of N100, N200 and P300 in ERP progressively shortened until about 16 to 17 years, after which they reached adult level, but with a slight tendency to prolongation towards 20 years; there was no trend for amplitude changes. The reaction time was more rapid than that of P300 latency. These findings indicate that the cognitive function of children improves rapidly until 16 to 17 years. A reduced difference between P300 latency and reaction time with age implies additional progress in motor skill. Combining P300 latency and reaction‐time studies may provide more detailed information for the evaluation of the development of cognitive function and motor skill.

A multicenter, randomized, controlled trial of intravenous gamma globulin therapy in children with acute Kawasaki disease

We studied the effect of intravenous, polyethyleneglycol‐treated, human immunoglobulin, administered at 200 mg/kg per day (group A: n = 147; male 86, female 61; age < 1 year, 50) or 400 mg/kg per day (group B: n = 152; male 87, female 65; age < l year, 52) for five consecutive days and compared it with freeze‐dried, sulfonated human immunoglobulin [group C: n = 152; male 87, female 65; age < 1 year, 51), administered at 200 mg/kg per day for five consecutive days, on the prevention of coronary artery abnormalities in Kawasaki disease. Echocardiograms were interpreted blindly and independently. Proportions of 87.1%, 95.4%, and 82.3% in groups A, B, and C, respectively, had no coronary artery abnormalities. The confidence limits of difference between the proportions of groups A and C, groups B and C, and groups B and A were −4.4% and 10.4%, 7.8% and 15.9%, and 4.0% and 10.8%, respectively. Duration of fever and serum immunoglobulin G (IgG) levels were correlated with the prevalence of coronary artery abnormalities. We concluded that intravenous, polyethyleneglycol‐treated, human immunoglobulin and freeze‐dried, sulfonated human immunoglobulin had clinically equivalent effects on coronary artery abnormalities, and that five daily doses of 400 mg/kg of intravenous, polyethyleneglycol‐treated, human immunoglobulin is more effective than that of 200 mg/kg gamma globulin.

Immunoreactive Polymorphonuclear Leukocyte Elastase in Complex with Alphal‐Antitrypsin in Kawasaki Disease

Immunoreactive polymorphonuclear leukocyte elastase (iPMN elastase) was measured by ELISA in 36 patients with Kawasaki disease and in 41 patients with anaphylactoid purpura and other infectious diseases of childhood. The iPMN elastase level was very high in Kawasaki disease compared with other diseases. There was no significant correlation in iPMN elastase levels in Kawasaki disease between the complicated and non‐complicated groups in the acute period. The iPMN elastase in the non‐complicated group fell below 250 4g/1 at 20 days from the onset of the illness. However, iPMN elastase in the complicated group (coronary aneurysm or dilatation) tended to remain high above 500 ligjl at 20 days from the onset. We suggest that increased iPMN elastase levels in Kawasaki disease reflect stronger enhancement and stimulation of PMN than in other diseases.

Coronary risks after high-dose γ-globulin in children with Kawasaki disease

Abstract Objectives: The goals of the present study were to develop a predictive coronary risk scoring system after intravenous γ‐globulin (IVGG) therapy of any dose for the different preparations currently used in the treatment of children with Kawasaki disease and to determine the predictive value of the system. The previously reported scoring systems were based on treatment with high‐dose IVGG therapy at limited doses and were determined using investigative methods.

Autologous bone marrow transplantation in children with advanced neuroblastoma.

Background. Encouraging results have been reported with high dose chemotherapy and total body radiation followed by bone marrow autotransplantation in children with advanced neuroblastoma; however, relapse remains a significant problem. Methods. The authors treated 22 children with advanced neuroblastoma with high dose chemotherapy, surgery, intraoperative radiation, and a bone marrow autotransplant (treated in vitro to remove tumor cells) followed by 13-cis-retinoic acid. Results. The 3-year relapse rate was 25% (95% confidence interval [CI], 6-44%). The 3-year disease free survival rate was 72% (95% CI, 52-92%). Toxicities included hemolytic uremic syndrome, herpes infection, and hepatic venoocclusive disease. Conclusion. These data suggest that this treatment strategy offers an increased rate of 3-year disease free survival. The nonrandomized nature of this study and its use of multiple modalities precludes the analysis of the specific contribution of each treatment component and comparison with conventional therapy. Cancer 1994; 74: 972-7.

Findings on magnetic resonance imaging of the spine and femur in a case of McCune-Albright syndrome.

Polyostotic fibrous dysplasia, a major osseous change in McCune-Albright syndrome, is seen in the cranium, facial bones, bones of the extremities, and ribs, but rarely in the spine. Spinal X-rays revealed no abnormalities in an 8-year-old girl with this syndrome, but99mTc-methylene diphosphonate bone scintigraphy disclosed high-density areas in the thoracic and lumbar vertebrae. Multiple well-circumscribed areas of low signal intensity were seen on T1-weighted magnetic resonance imaging (MRI) of the spine. Although MRI spine scans in this disease have never been reported, our findings in this case proved interesting for evaluating osseous lesions. MRI made it possible to differentiate between fibrous lesions (low signal intensity on T1- and T2-weighted MRI) and cartilaginous lesions (low signal intensity on T1-weighted MRI and high signal intensity on T2-weighted MRI).

Lipoprotein (a) and apolipoprotein A‐1 and B in schoolchildren whose grandparents had coronary and cerebrovascular events: A preliminary study of 12–13 year old Japanese children

The aim of this study was to evaluate the relationship between the serum levels of lipoprotein (a) [Lp (a)] and apolipoproteins (apo A‐1 and apo B) in schoolchildren with a history of coronary and cerebrovascular events in their grandparents.

Treadmill exercise test in children with cardiomyopathy and postmyocarditic myocardial hypertrophy

SummaryThe treadmill exercise test with the Bruce protocol was performed in three patients with postmyocarditic myocardial hypertrophy (PMH) and ten patients with cardiomyopathy, including three with dilated cardiomyopathy (DCM), five with hypertrophic obstructive cardiomyopathy (HOCM), and two with hypertrophic and nonobstructive cardiomyopathy (HCM). The endurance time was below the normal level in all but one case and was normal or near normal in the three cases with PMH. ST depression was observed in five cases, none of which were of HCM. A marked increase in amplitude of the negative phase of the P wave in V1 was observed in one patient with DCM. The response of blood pressure during the exercise was abnormal in patients with DCM and HCM but was normal in PMH.