An Immunological Study on Mucocutaneous Lymph Node Syndrome: Activation of Tg Suppressor Cells

We analyzed the lymphocyte function of MCLS in pokeweed mitogen-induced immunoglobulin production. T cell suppressor activity was activated in the acute phase adn normalized in convalescence. T cells showing suppressor activity in this system were identified to be Tg cells, and they seemed to be activated as suppressor by a certain factor in the serum. the sera of patients whose T cells showed suppressor activity could induce the suppresoor activity of normal control Tg cells. The suppressor inducing factor in sera is likely to be circulating immune complexes, since T_g cells usually show suppressor activity when they interact with immune complexes. The induction of the suppressor activity of Tg cells by immune complexes may provide a kind of negative feedback suppression mechanism feedback mechanism on the antibody formation and it may be related to the self-limiting characteristics of MCLS. MCLS is different from collagen diseases such as rheumatoid arthritis in that Tg cells do not usually work as suppressor cells in the later diseases.     

川崎病并巨噬细胞活化综合征1例

患儿,女,1岁1个月,以反复发热1个月,唇皲裂、指(趾)肿胀6 d,黄疸4 d入院。体检:全身重度黄染,散在皮疹,股静脉穿刺处有0.8 cm×1.0 cm瘀斑,球结膜充血,巩膜黄染,唇红皲裂,杨梅舌,肝肋下4 cm,质中等边缘钝,脾未及,指(趾)肿胀明显,脱屑少。血WBC 2.0×109L-1,N0.8,L0.2,Hb67 g/L,     

Cytochrome B558 Component of O-2M Generating Oxidase in the Neutrophils From Patients With Chronic Granulomatous Disease

Cytochrome b₅₅₈ is probably a component of an electron transport chain which is called NADPH oxidase. Cytochrome b₅₅₈ from the neutrophils of two male patients with chronic granulomatous disease (CGD) and their parents was studied. One of the CGD patients had decreased amounts of cytochrome b₅₅₈, The α-band of light absorption of cytochrome b₅₅₈ at 558 nm was found as a trace, and the peak of the γ-band was shifted to 420 nm, whereas the normal γ-band is at 426 nm. Intermediate amounts of cytochrome b₅₅₈ were present in his mother, whereas his father had a normal amount. Another patient had normal peaks of α- and γ-bands of cytochrome b₅₅₈. Thus, abnormalities in NADPH oxidase of the two patients were different.     

The Potential Role of Cytokine-Mediated Vascular Endothelial Activation in the Pathogenesis of Kawasaki Disease

Kawasaki disease (KD) is an acute febrile illness of infancy and early childhood characterized by diffuse vasculitis. Although the disease is generally self-limited, up to 30% of untreated patients with KD may develop coronary artery (CA) abnormalities. The acute phase of KD is characterized by marked activation of the immune system leading to increased cytokine production by immune effector cells, the induction of activation antigens on their vascular endothelium and the generation of lytic antibodies directed against vascular endothelial cells (EC) stimulated with cytokines. Treatment with intravenous gammaglobulin (IVGG) usually rapidly reduces acute clinical symptoms and prevents CA abnormalities. Immunologically, successful IVGG treatment is associated with decreased lymphocyte activation, reduced cytokine secretion and the loss of cytokine induced expression of leukocyte adhesion molecules on vascular endothelium. The association between improvement of clinical symptoms with the reduction of cytokine secretion, and reversal of EC activation supports a role for immune mediated injury to cytokine induced EC antigens in the pathogenesis of this disorder.     

Concentrations of LTB4, LTC4, LTD4 and LTE4 in bronchiolitis due to respiratory syncytial virus

Fifty-five infants with bronchiolitis due to respiratory syncytial virus were evaluated for the presence of leukotriene B₄, C₄, D₄ and E₄ in nasopharyngeal secretions. An attempt was made to correlate concentrations of leukotrienes to arterial oxygen tension. Forty participants received conventional therapy consisting primarily of aerosolized albuterol and occasional aminophylline therapy. The other 15 individuals received ribavirin therapy in addition to conventional therapy, and leukotriene concentrations were compared among individuals in these groups. RSV infection was documented by standard methods, and leukotrienes were measured by reverse-phase high pressure liquid chromatography. The leukotriene detected most commonly was LTC₄ (up to 83% of subjects); LTD₄ and LTB₄ were present in approximately 30% of individuals. The mean partial pressure of oxygen was found to be lower in those individuals with detectable LTB₄ than in those without detectable LTB₄ (p < 0.025), and an overall inverse correlation of LTB₄ concentrations with initial pO₂ values was observed (r = 0.318, p < 0.05). The presence and quantity of other leukotrienes did not correlate with the severity of illness. During the first week of illness, the concentration of leukotrienes declined sharply in ribavirin recipients. Individuals receiving conventional therapy during the same time interval exhibited stable or increasing leukotriene concentrations. These observations suggest that LTB₄ may be important in the pathogenesis of bronchiolitis, and that ribavirin therapy may inhibit leukotriene release in the respiratory tract.     

Interventions in Kawasaki Disease

During the past 10 years, the clinical experience of catheter interventional treatment in Kawasaki disease has gradually increased. These treatments include balloon angioplasty, stent implantation, rotational ablation, and transluminal coronary revascularization. Because coronary artery stenosis in Kawasaki disease commonly involves severe calcification, in contrast with adult atherosclerotic coronary artery lesions, the indication or technique of catheter intervention for adult patients cannot be directly determined. Satisfactory acute results for coronary balloon angioplasty were obtained in patients in a relatively short interval from the onset of disease, especially within 6 years. However, the incidence of restenosis after angioplasty was still high. Rotational ablation may be the most appropriate catheter intervention for Kawasaki disease. The advantage of rotational ablation is the high success rate, even in patients with calcified coronary artery stenosis. Stent implantation requires larger arterial access and is not possible in younger children. Care should be paid to the detection of new aneurysm formation because this was associated with the use of additional balloon angioplasty using high-pressure balloon inflation. Anticoagulation or antiplatelet regimens are essential for long-term management.     

Co-morbidity of Kawasaki Disease

Despite more than four decades of investigation, the etiology of Kawasaki disease remains obscure, and none of the proposed etiologic theories for the disease have achieved independent confirmation. Clinical and epidemiologic features support an infectious cause, but the etiology remains unclear. The authors present a case of Kawasaki disease associated with Epstein-Barr virus and Mycoplasma pneumoniae infection in a 3.5-y-old boy. He received two doses of intravenous immunoglobulin due to prolonged course of Kawasaki disease but later had complicated autoimmune haemolytic anaemia. His prolonged fever subsided after azithromycin administration. Epstein-Barr virus infection was confirmed by molecular microbiological pathology of cervical lymph node and serological tests. The serological tests for Mycoplasma pneumoniae also revealed a positive result. Thus, it is concluded that Mycoplasma pneumoniae and Epstein-Barr virus infections may occur simultaneously in a child with Kawasaki disease. In addition, autoimmune hemolytic anaemia may be noted in Kawasaki disease patients after high-dose IVIG administration. To the authors' knowledge, this is the first report of Kawasaki disease with Epstein-Barr virus and Mycoplasma pneumoniae in the English-language literature.     

Spectrum of Kawasaki Disease

The authors report 22 patients of Kawasaki disease, diagnosed and treated over a period of 3 years at a tertiary care centre in New Delhi. Ten cases fullfiled the criteria of a “classical” case while 12 cases were “incomplete” cases. Echocardiography was performed in all cases and coronary artery involvement was found in 8 (36%) cases. All cases received high dose Intravenous Immunoglobulins (IVIG) as standard therapy. In two cases, a repeat dose of IVIG was required for defervescence to occur. All cases recovered after therapy. Those with coronary artery involvement were planned for a 2 years follow up from the time of diagnosis.     

川崎病的诊断

美国多学科委员会(美国风湿热、心内膜炎和川崎病委员会、青少年心血管疾病委员会、美国心脏协会)的专家被召集修订美国心脏协会关于川崎病的诊断、治疗和长期管理的建议,最后由美国儿科学会签署.该文对川崎病的诊断标准,尤其对不完全川崎病的诊断进行了必要的补充和修订.     

Symptomatic Myocarditis in Kawasaki Disease

Kawasaki disease (KD) is an acute febrile illness of infants and young children that is characterized by a medium vessel vasculitis, most commonly involving the coronary arteries. Though subclinical myocarditis is rather common in KD, symptomatic congestive heart failure is extremely uncommon. The authors report a 9-y-old boy who developed heart failure (ejection fraction 28%) in the acute phase of KD. He was initially treated with intravenous immunoglobulin (2 g/kg) without much clinical improvement. He was then given 5 daily pulses of intravenous methylprednisolone followed by tapering doses of oral prednisolone. The child showed prompt clinical recovery and remains well on follow-up. The present case serves to highlight the fact that methylprednisolone can be considered upfront as rescue therapy in children with KD who have symptomatic congestive cardiac failure during the acute stage of the disease.     

Mite Antigen in Kawasaki Disease

The relation between house dust mite antigen and Kawasaki disease (KD) wasexamined immunologically in 60 patients with KD.

• 1) In most patients, the serum IgE was significantly raised during the acute phase of the disease and fell gradually during convalescence. 2) Although anti-mite (Dermatophagoides pteronyssinus) specific IgE (RAST) values during the illness were significantly raised in only 15 patients (25%) when expressed as RAST scores, there was a correlation between serum IgE and anti-mite specific IgE (expressed as counts/min) (r=0.56; p<0.01). 3) An intradermal skin test on reactivity to mite antigen gave positive or false-positive in 34 of 60 patients (57%) transiently or persistently during the illness. 4) The serum levels of anti-mite (D.pteronyssinus) specific IgG were estimated by the IgG RAST method. In 15 of 20 patients (75%) RAST values were persistently or transiently high. 5) The presence of mite antigen in circulating immune complexes in the patients was examined by Raji cell assay and the direct fluorescein isothiocyanate labelled anti-mite rabbit gammaglobulin method. In three of eight patients specific fluorescence was seen on the surface of cells. 6) The proliferative responses of lymphocytes to mite ryltigen and phytohaemaglutinin (PHA) were examined in six patients. The response to mite antigen of lymphocytes from the patients was significantly higher than those of age-matched controls (p<0.0.1). 7) More mite (Dermatophagoides spp) were found in house dust collected from the homes of 12 patients than in that of dust from 10 control homes (p<0.0 1).

These results support the hypotheses that mite antigen may invade the body via the upper respiratory tract and act as a respiratory allergen and form circulating immune complexes, resulting in an immune copledx disease such as vasculitis.     

Pulmonary Hypertension in Kawasaki Disease

This report describes the case of two pediatric patients who demonstrated echocardiographic evidence of pulmonary hypertension (PH) during the acute phase of Kawasaki disease. The etiology of PH development in this setting is currently unknown, but the authors hypothesize that pulmonary vasculitis may play a significant role. Fortunately, the PH appeared to be self-limited and resolved in both cases with routine treatment of Kawasaki disease.