Masakatsu Kubo

Characteristics of juvenile dermatomyositis in Japan

Questionnaires were sent to 1290 hospitals in Japan asking for data on patients with juvenile dermatomyositis (JDM) diagnosed between June 1984 and May 1994. Of the 204 patients identified by these questionnaires, 102 met the criteria for JDM. JDM is categorized into three subtypes: Banker‐type JDM, Brunsting‐type and fulminant‐type; patients with the latter exhibit markedly elevated serum levels of creatinine phosphokinase (> 10 000 U/mL) and appear to be at risk of renal failure. Cutaneous manifestations were present in 98% of patients and preceded the appearance of other symptoms. This tendency is one of the reasons for the difficulty in some cases in diagnosing the onset of JDM. Better criteria for early treatment of JDM are needed. The results of the present study suggest that itching and calcinosis are factors that indicate a poor prognosis in patients with JDM. Muscle enzyme levels do not always reflect disease activity, suggesting that methods other than measurement of muscle enzymes, such as measurement of the levels of neoprerin and von Willebrand factor antigen, as well as magnetic resonance imaging should be used to be evaluate disease severity. Patients with Brunsting‐type JDM who exhibit dysphagia and antinuclear antibody positivity and patients with Banker‐type JDM should be treated aggressively. Pulse therapy should be selected as the initial therapy in patients with fulminant‐type JDM.

Antibodies to native type III collagen in the serum of patients with Kawasaki disease.

Pathological studies have revealed that the vasculitis in Kawasaki disease (KD) begins in the intima and the adventitia of blood vessels resulting in panvasculitis. In the present study we investigated the possibility that auto-antibodies to types III and II collagen are involved in the vascular injury. Seven out of 38 patients (18%) with KD had elevated serum levels of IgG anti-body to type III collagen. None had increased serum antibodies to type II collagen. Specificity of the antibody to type III collagen in patients with KD was 86%. Three out of seven KD patients (42.9%) with coronary complications had elevated serum levels of the antibody. It is conceivable that the more severe the inflammation of systemic blood vessels, the more frequently antibodies to type III collagen are detected and coronary complications develop. Accordingly, this antibody may be secondarily detected in the serum of KD when systemic blood vessels are severely damaged. Detection of antibodies to type III collagen might be a risk factor predicting coronary complications.

A case of isolated retrograde amnesia following brain concussion

Retrograde amnesia is typically accompanied by anterograde amnesia. However, several recent reports describe isolated retrograde amnesia in which associated anterogarde amnesia was not present. 1–7 In fact, retrograde amnesia has various etiologies (e.g. encephalitis, traumatic, psychiatric) and likely represents a heterogeneous entity that is dependent on multiple memory systems within discrete neural structures. 2,4

Human Parvovirus B19感染症が発症に関与したと考えられた全身型若年性関節リウマチの1女児例

We encountered a case of a girl where Human Parvovirus B19 infection was considered to have been concerned with the development of systemic type juvenile rheumatoid arthritis (JRA). While the affected child did not show any evident infectious erythema-like findings, changes in the serum antibody titer by the EIA method presented the pattern of first infection. During the clinical course the condition of the disease as JRA was serious and hemophagocytic syndrome developed concurrently. Furthermore, the resistance to the treatment was also noted. So the patient was treated with prednisolone combined with low dose weekly MTX therapy. The possibility of Human parvovirus B19 being concerned with the development of rheumatoid arthritis in one form or another has been suggested in recent years. In the disease type with systemic angititis as main pathophysiology, which is called systemic JRA we encountered this time, it is not clear how Human Parvovirus B19 was concerned with the development of this disease, but it appeared to hold a key position in studying pathophysiology of the development.

A boy with erosive arthritis during treatment of severe group-A streptococcal infection

Abstract The patient was admitted to our department with major complaints of fever, swelling of the left thigh, and a cognition disorder. A blood culture yielded type T1, toxin-B-positive group-A streptococcus. Magnetic resonance imaging of the left foot showed fasciitis. Severe group-A streptococcal infection was diagnosed, and the administration of antibiotics, a massive dosage of gamma-globulin, and a steroid preparation were prescribed, which resulted in a general improvement in the symptoms and reductions in the swelling of the left foot and the second finger of the left hand without any sequelae. Following discharge from the hospital, however, destruction of the joint of the first toe of the right foot was recognized. A number of cases of post-streptococcal reactive arthritis (PSRA) have recently been reported, and the pathogenicity of this arthritis, which is different from that of rheumatic fever, has been noted. Severe group-A streptococcal infections, such as the one described here, may develop into erosive arthritis after the symptoms, that affect several organs, have disappeared. We believe that because it is possible for post-streptococcal reactive arthritis to develop, one should be aware of this when dealing with all clinical patients who have streptococcal infections.

Study on Septicaemia in Infants and Children in the Past 20 Years

Underlying diseases, complications, clinical findings, and laboratory findings were evaluated in 158 cases of septicaemia admitted to Jikei University Hospital from 1975 to 1994, in order to conjectured factors that prescribe for the prognosis. 50% of the patients had underlying diseases. Malignancy including leukaemia (31 cases, 39.2%) was the most common underlying disease, followed by low birth weight infant (17 cases, 21.5%), aplastic anemia (9 case, 11.4%), and congenital heart disease (7 cases, 8.9%). The death rate for patients with underlying disease (27.8%) was significantly greater than the mortality for normal patients with septicaemia (8.9%) (p < 0.05). Meningitis (24.7%) was the most common complication, followed by DIC (19.6%), shock (15.2%), and pneumonia (10.8%). The mortality rate of septicaemia complicated by shock was 66.7% (p < 0.01), and that complicated by DIC was 45.2% (p < 0.01). The mortality rate for patients with the clinical findings of respiratory distress, cough, abdominal distention, cyanosis, splenomegaly, or peripheral coldness was more than 40% and significantly greater (p < 0.01). Mortality rate in patients with granulocyte counts of < 4.000/mm3, platelet counts of < 5 x 10(4)/ mm3, total protein of < 5.0 g/dl, or ESR of < 20 mm/hr were significantly greater (p < 0.01) than those in patients with normal laboratory findings. Coincidence rate of blood and stool cultures was 57.9% for E. coli, and 28.6% for Klebsiella sp., and that of blood and throat cultures was more than 30% for Pseudomonas sp., Haemophilus influenzae, and Staphylococcus aureus. In the study of antimicrobial susceptibility for microorganisms isolated, the number of drug resistant S. aureus had increased in the last 10 years.

Studies on Human Lymphocyte Subpopulation

Cyclic nucleotide is considered as one of the regulators of immune response. 98 healthy subjects and 22 infants with bronchial asthma were subjected to the study on the effects of cyclic nucleotide and related drugs on SRBC receptor and complement receptor of lymphocytes. Lymphocytes were isolated by conray-ficoll gradient method. The isolated lymphocytes were incubated together with individual drugs at 37°C for 30 minutes. Each lymphocyte preparation was washed three times with gelatine veronal buffer. SRBC receptor and complement receptor of lymphocytes was examined by the methods of Yata et al.1). Active rosette test was performed using the methods described by Wybran et al.2). The results were as follows: The binding affinity of lymphocytes to SRBC was signiflcantly inhibited by the addition of iso, DbcAMP or PGEl. Propranolol could inhibit the inhibitory action of iso, but not that of DbcAMP. Active rosette test revealed that both is0 and DbcAMP inhibited the lymphocyte binding affinity to SRBC. The results obtained from E rosette test and active rosette test in patients with brochial 1)