Bochdalek Hernia Causing Sigmoid Colon Strangulation in an Elderly Patient.

Diaphragmatic hernia with bowel strangulation is a fatal condition requiring a prompt diagnosis. Bochdalek hernia is a common type of diaphragmatic hernia that rarely but occasionally occurs in adults. We herein report a case of Bochdalek hernia causing sigmoid colon strangulation in an elderly patient whose condition was initially misdiagnosed as empyema. The early diagnosis of strangulated bowel stemming from diaphragmatic hernia can be challenging because of its rarity and the nonspecificity of its symptoms. However, tracing the mesenteric arteries on computed tomography can enable a quick diagnosis.

Enhanced T-cell response to mosquito extracts by NK cells in hypersensitivity to mosquito bites associated with EBV infection and NK cell lymphocytosis.

Hypersensitivity to mosquito bites is characterized by severe systemic as well as local symptoms, and associated with chronic active EBV infection and NK cell lymphocytosis. In this HEN disease, we investigated the response of PBMC to MSG extracts. PBMC were taken from three defined cases of HEN disease, three borderline cases, five individuals with simple exaggerated reactions to mosquito bites without systemic symptoms (simple responders), and eight healthy donors. PBMC, or purified CD4+, CD8+ or CD56+ cells, were cultured with MSG extracts prepared from each of five mosquito species to examine their proliferation and cytokine secretion. The patients with HEN disease had high stimulation indices with variations in responses to the extracts from Aedes albopictus, Aedes aegypti, Anopheles sinensis and Culex pipiens pallens. However, a non-Japan-habitant species Anopheles stephensi did not stimulate the patients' PBMC. Some borderline or simple responders showed moderate proliferation, and healthy donors had no reactive PBMC. In HEN disease, both CD56+ NK cells (producing IFN-gamma) and CD4+ Th0 cells (producing IL-4 and IFN-gamma) were increased in the blood. CD4+ cells, but not CD56+ NK cells or CD8+ cells, propagated in response to MSG extracts. However, this response of CD4+ cells and their IL-4 production were strongly enhanced by coexisting CD56+ cells. We suggest that the CD4+ T cell serving as the primary responder to MSG antigen and the NK cell functioning as the enhancer are both pathogenic in the development of HMB.

Mizoribine as an effective combined maintenance therapy with prednisolone in child-onset systemic lupus erythematosus.

BACKGROUND: Systemic lupus erythematosus (SLE) is one of the major collagen diseases in childhood. However, the pathogenesis of this disease still remains unknown. The disease is known as a chronic inflammatory disease. Since oral and intravenous corticosteroid therapy has been introduced into the treatment of SLE, the prognosis of patients has improved significantly. However, it has now become clear that there are limitations in the effectiveness, as well as adverse reactions when corticosteroids therapy is administered for a long-term period. Therefore, we have been attempting to improve the maintenance therapy of child-onset SLE. METHODS: We have proposed and tested a new type of combination therapy using prednisolone (PSL) and mizoribine (MZR) in pediatric patients with SLE for maintenance therapy after the induction of remission. RESULTS: Our results showed that this combination therapy is more effective than the previous regimen. In addition, no significant side-effects were observed in our study. CONCLUSION: This combination therapy is still not perfect. Efforts should be continued to establish an optimal therapy regimen for child-onset SLE.

[Intravenous cyclophosphamide pulse therapy for refractory juvenile dermatomyositis].

We described three children with juvenile dermatomyositis (JDM) refractory to the conventional therapy. They were successfully treated with intravenous cyclophosphamide (IVCY) pulses, and two of them were administered plasma exchange (PE) before IVCY. Case 1. A 17-year-old girl with JDM was previously treated for 2 years with the combination of prednisolone, intravenous gamma-globulin, methotrexate, and azathioprine. However, muscle weakness gradually progressed. She failed to hold her sitting position and to rise her arms, but both serum CK and aldolase were stable. After the episode of aspiration pneumonia the follow-up muscle biopsy was performed, which revealed muscle degeneration and massive mononuclear cell infiltration in perivascular area. The erythrocyte sedimentation rate (ESR) and fibrin degradation product E (FDP-E) levels were gradually increased. Because the active inflammation of muscle and muscle vasculature was suspected, the PE and IVCY combination therapy was administered. During the 6 courses of the therapy, muscle weakness was markedly improved so that she could hold herself at the sitting position and could have meals by herself. Case 2. A 5-year-old boy with JDM was treated for 8 months with prednisolone p.o., but his muscle strength became worse. The muscle enzyme levels, such as serum CK and aldolase, were not reflecting his status of the disease, but FDP-E levels were increased. Muscle MRI and biopsy revealed the inflammatory changes of perivascular area of muscle. The PE and IVCY combination therapy was effective, and he became able to walk and run by himself. Case 3. A 14-year-old boy was diagnosed as having JDM when he was 10 years of age, and treated with prednisolone p.o., and subsequently with intravenous methylprednisolone pulses and azathioprine. Three years later the flares were observed accompanied with the elevations of serum CK and FDP-E. The administration of IVCY improved muscle strength as well as serum muscle enzyme and FDP-E levels. These findings indicated that the clinical manifestations of JDM should be closely monitored, that the serum levels of muscle enzymes including CK and aldolase were sometimes not indicative for the flares of JDM, and that muscle MRI and re-biopsy examination were needed for the children with progressive muscle weakness. In addition, determination of ESR and FDP-E was not specific but helpful to detect flares of the disease in some cases.