Takeshi Miyake

Scoring method for identifying patients with Kawasaki disease at high risk of coronary artery aneurysms

The beneficial effect of immunoglobulin in preventing coronary artery aneurysms in patients with Kawasaki disease is recognized, but immunoglobulin should be selectively given to patients at high risk for coronary lesions. The present study was designed to present a scoring method to evaluate the severity of Kawasaki disease in terms of coronary artery disease based on clinical and early laboratory findings. Seventy-eight patients who were admitted at 4 to 7 days of illness and who had received aspirin alone during the acute febrile period were separated into 2 groups according to significant coronary artery involvement, and possible related factors to the discrimination of the 2 groups were studied using multivariate analysis. Age at onset, C-reactive protein and platelet count contributed significantly to the discrimination, and from these 3 items a simple scoring system was devised to predict patients at high risk for coronary lesions. Retrospective study using this scoring method indicated that significant coronary artery disease was more often seen in patients predicted to be at high risk treated with aspirin, while no patients who received immunoglobulin had coronary lesions of moderate or severe degree. Thus, the present scoring method provides simple but clinically useful criteria for predicting patients at high risk of coronary artery disease, and immunoglobulin therapy for selected patients may reduce the significant coronary artery complications of Kawasaki disease.

Small bowel pseudo-obstruction in Kawasaki disease

Between June 1977 and May 1986, 310 patients with Kawasaki disease were admitted to Shizuoka Childrens Hospital. Among these patients, seven showed the symptoms of intestinal paralysis during the acute stage. We present three of these cases who showed typical intestinal pseudoobstruction radiologically, and discuss the treatment of this type of intestinal involvement.

Inherited Deficiency of the Seventh Component of Complement Associated with Meningococcal Meningitis: Lack of Serum Bactericidal Activity against Neisseria meningitidis in a Girl with C7 Deficiency and HLA Studies of a C7-Deficient Japanese Family

An 8‐year‐old girl with meningococcal meningitis lacked serum complement activity. The seventh component of complement (C7) could not be detected in her serum by either functional or immunochemical analysis. The levels of the other components were within the normal range. Her serum complement activity was restored by the addition of purified C7. Her fresh serum showed a total absence of bactericidal activity against Neisseria meningitidis, group Y, but her serum bactericidal activity was restored by the addition of purified C7. The restoration of her serum bactericidal activity was completely inhibited in the presence of Mg2+ EGTA. These findings suggest that restoration of the bactericidal activity of her serum against N. meningitidis might be mediated by the specific antibody against N. meningitidis and the reconstituted complement system in her serum.

B-Cell Function in Kawasaki Disease and the Effect of High-Dose Gamma-Globulin Therapy

We studied in vino B‐cell function in Kawasaki disease (KD). By plaque‐forming assay, IgG‐, IgA‐ and IgM‐secreting cells in the first week of KD were markedly increased, and recovered to a normal level in the second week in many cases. Lymphocyte blast formation with Staphylococcus aureus Cowan I (SAC), a B‐cell‐specific mitogen, was suppressed in the acute phase, and recovered to a nod level in the convalescent phase. By flow cytometry, HLA‐DR‐ and HLA‐DQ‐positive cells were increased in the acute phase of KD. CD3‐and CD4‐positive cells were also decreased. CD8‐positive cells showed no significant change. In five patients, CD4‐positive cells with HLA‐DR positivity neither increased in the acute phase nor changed during the course of illness. From our results, it can be considered that pathogenic microorganisms or toxins activate B cells directly in KD without the association of T cells. We also studied the effect of high‐dose gamma‐globulin therapy on B‐cell function in KD. However, the results indicated that this form of therapy had no significant effect on B‐cell functions.