Between June 1977 and May 1986, 310 patients with Kawasaki disease were admitted to Shizuoka Children's 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 pseudo-obstruction radiologically, and discuss the treatment of this type of intestinal involvement.

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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.

Aspergillus infection is the most frequent fungal infection associated with chronic granulomatous disease (CGD), and often results in a life-threatening situation. This report describes the use of high-dose fluconazole, a new antifungal agent, for invasive Aspergillus infection in a patient with CGD. A 27-month-old boy was sent to our hospital because of unknown fever in October, 1988. He was then admitted for pneumonia and pleural effusion of the right lung in February, 1989. Treatment with antibiotics was ineffective, and cultures of throat and pleural fluid were negative. In May, 1989, Aspergillus fumigatus was cultured from a subcutaneous abscess at the point of pleural puncture. Therefore we speculated that Aspergillus might have been the cause of pneumonia. The patient was diagnosed as having CGD by NBT test. Treatment with miconazole, flucitocin and amphotericin-B syrup was ineffective. From July, 1989, he was given 100 mg/day fluconazole d.i.v., but the drug did not reach an effective serum concentration to combat Aspergillus. However, an effective concentration of fluconazole was reached at a dose of 250 mg/day, and the chest X-ray findings subsequently improved, despite occasional high fever and continued high CRP. In July, 1990, the route of fluconazole administration was changed from d.i.v. to p.o. at the same dose, resulting in a serum concentration of fluconazole higher than that achieved with d.i.v. treatment. Both the clinical and laboratory findings showed improvement thereafter. Therapy for Aspergillus infection associated with CGD was found to necessitate high doses of anti-fungal drugs over a long period, although treatment with previously employed anti-fungal drugs could not be continued due to their adverse side effects.(ABSTRACT TRUNCATED AT 250 WORDS)

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. Heterozygous deficiency of C7 was found in 10 of her family members. Genetic studies showed that the mode of inheritance might be an autosomal codominant trait. No genetic linkage between deficiency of C7 and the HLA system was found.The biological significance of the complement system has been fully elucidated with the discovery of complement deficiencies associated with disease (9, 25). Deficiencies of the early-acting complement components (Cl, C4, and C2) are frequently associated with systemic lupus erythematosus (SLE) or other rheumatic diseases (2). In patients with C3 deficiency, recurrent bacterial infection has been shown to be the predominant complication (24) and lupus-like symptoms have also been reported as complications (26). It has been reported that the deficiencies Hamamatsu 430.