传染性单核细胞增多症与皮肤黏膜淋巴结综合征的实验室检测对比分析

目的　分析传染性单核细胞增多症 (IM)和皮肤黏膜淋巴结综合征 (MCLS)的实验室检测结果 ,并对比分析 ,以明确二者的鉴别诊断要点 ,指导治疗。方法　收集我院近 10年住院IM患儿 2 2 5例 ,MCLS患儿 2 80例。实验室指标包括 :EB病毒 (EBV)抗体、嗜异凝集试验、外周血白细胞总数及分类、异形淋巴细胞、血小板计数等进行对比分析。结果　IM及MCLS患儿EBV抗体阳性率均高 ,无显著差异。嗜异凝集试验及异形淋巴细胞检查 ,阳性率差异甚大 ,IM患儿中可见明显增高。外周血白细胞总数均增高 ,MCLS白细胞分类以中性粒细胞升高更明显 ,而IM则以淋巴细胞升高明显 ;而血小板计数则以MCLS增高明显 ,且当白细胞总数趋于正常时血小板持续升高。结论　IM及MCLS病因似与EBV感染有连带关系 ,在实验室检查中各有特点 ,掌握其鉴别诊断要点 ,对早期明确诊断 ,指导治疗有重要意义     

Complement and protease inhibitors in the mucocutaneous lymph node syndrome

Total hemolytic activity of serum (CH50), complement components C3 and C4, alpha 1antitrypsin (alpha 1AT), alpha 1antichymotrypsin (alpha 1X), antithrombin III (AT III), alpha 2 macroglobulin (alpha 2M), and inter-alpha-inhibitor (I-alpha-I) were measured in 23 Japanese and 19 European children with the Mucocutaneous Lymph node Syndrome (MCLS) during the acute phase of disease. Second sera, obtained after day 20 were available from 18 Japanese and 10 European children. In 28 out of 31 children with mild disease, as assessed by the coronary risk score of Asai and Kusakawa, complement was normal or elevated during the acute phase. In 10 out of 11 children with high risk scores, CH50 was below the normal range. One child in this group had ECG changes during the acute phase, one patient died and two others developed coronary aneurysms. C1I was elevated in all 42 cases, alpha 1AT in 40, and alpha 1X in 38 patients. alpha 1AT was depressed in two children, one of whom developed an aneurysm. One of the four children with depression of alpha 1X died of myocardial infarction. Decreased concentrations of AT III, alpha 2M and I-alpha-I were frequent and tended to mark the more severe courses of the disease. A third group of 20 children was evaluated 5 weeks to 6 months after the acute illness. Mean concentrations of all five protease inhibitors were completely normalized in this group. The results of this study indicate that consumption of complement and of protease inhibitors occurs in many cases of MCLS during the acute phase. Determination of CH50 appears to be useful to identify high risk patients early in the course of their illness. Transient deficiency of substances for control of inflammation may in part be responsible for the severe vascular lesions seen in some patients.     

Morphological observations on the vasculitis in the mucocutaneous lymph node syndrome

Twenty-seven skin biopsies were obtained from the exanthemata of patients in the acute stage of the mucocutaneous lymph node syndrome (MCLS). The three vascular systems of different caliber size—the intrapapillary capillary loops (ICL), the superficial arteriolar or venular plexus (SAP, SVP) and the small subcutaneous vessels—were examined to investigate the characteristics of the vasculitis in MCLS and differentiate it from infantile polyarteritis nodosa (IPN). Significant papillary edema and dilatation of ICL, SAP and SVP were observed on the 4th day after the onset of the illness, and then gradually decreased. In the subcutaneous regions, vasculitis began which endothelial necrosis, and subendothelial edema and degenerative changes in the muscle cells followed. These changes in the small subcutaneous vessels were observed for a longer period than in the ICL, SAP and SVP. Moderate mononuclear cell infiltrations were observed. Both arteries and veins were affected.     

Cardiovascular complications of mucocutaneous lymph node syndrome

The clinical and pathologic changes seen in patients with Kawasaki disease are discussed. Emphasis is placed on the cardiovascular manifestations and the present treatment and suggested long-term follow-up of this disorder.     

Plasma fibronectin concentrations in mucocutaneous lymph node syndrome.

Plasma fibronectin concentrations were measured in 41 patients suffering from mucocutaneous lymph node syndrome (MCLS, Kawasaki disease). Plasma fibronectin concentrations were decreased significantly in the early days of the disease, but increased gradually and reached significantly high concentrations by the fourth week of the disease. In nine of the 41 patients, coronary involvement was found by echocardiographic examination. These patients showed significantly lower plasma fibronectin concentrations in the first and second weeks of the disease than those without coronary involvement. As the underlying pathology of MCLS is known to be vasculitis, the lower plasma fibronectin concentrations might be partly the result of injury inflicted upon the endothelial cells, which are thought to be the major site of synthesis of circulating plasma fibronectin concentrations.     

Major histocompatibility complex antigens and immune mechanisms in steroid-responsive nephrotic syndrome

Although the pathogenesis of steroid-responsive nephrotic syndrome (SRNS) is obscure, involvement of an immune mechanism is often suggested. Further evidence of an immune basis for this disorder is an increased frequency of specific major histocompatibility complex (MHC) antigens. In the first part of this study, the phenotypic frequency of HLA-A, -B, -C, -DR antigens were investigated in 30 children with SRNS and in 630 controls. In the second part, total T (CD3⁺ cells) and B lymphocytes (CD19⁺ cells) and the lymphocyte subsets (CD4⁺, CD8⁺ cells and their ratio) were studied in the same patients and in 30 healthy children. The investigations of all the patients were performed during the acute stage and 14 of 30 during remission stage. Human leukocyte antigens (HLA) were determined by standard microlymphocytotoxicity assay and lymphocytes were analyzed by flow cytometry. Human leukocyte antigens A3, DR4, DR7 and the haplotype HLA-A2/B12 showed the strongest association with SRNS. In the studies for cellular immune disorder, CD3⁺ and CD8⁺ cells were found to be decreased significantly in the acute stage before beginning steroid therapy. No significant difference in lymphocyte subsets was observed in the remission stage without steroid or immunosuppressive therapy.     

Circulating immune complexes in leukaemias and lymphomas

Circulating immune complexes (CIC) were estimated in 78 patients of leukaemias and lymphomas by CIq deviation ELISA and PEG assay. In all leukaemias a significant elevation in CIC was seen at the time of first presentation. While in ALL a decrease occurred on therapy as partial or complete remission was achieved, no such fall was seen in AML or CML-BC when treated. CIC levels were much higher in non-Hodgkins lymphoma than in Hodgkins disease and showed a direct correlation with B symptoms and activity of the disease. The CIC levels were highest in null-ALL followed by those in common ALL and T-ALL. The mean duration of remission in patients of ALL without elevation in CIC was much longer than in those with CIC.     

Elucidation on sudden death in children with the mucocutaneous lymph node syndrome

Atrial pacing was carried out in six children aged one year to eleven years with the mucocutaneous lymph node syndrome (MCLS) during cardiac catheterization. The cardiac index (CI) was measured before pacing and at pacing rates of 150 and 180/min. The CI increased in cases which did not show any pathological findings on the coronary artery angiograms. Conversely, CI decreased at a pacing rate of 150/min, in the case which showed arterial stenosis. Atrial pacing with measurement of CI may be a good method for detecting and evaluating coronary artery lesions in children with MCLS.     

Intrahepatic cholangitis associated with mucocutaneous lymph node syndrome

Although gallbladder hydrops occurs frequently in patients with mucocutaneous lymph node syndrome (MCLS), the etiology of the gallbladder lesion is unclear. Recently we performed a liver biopsy on a child with severe MCLS and demonstrated selective invasion of biliary ductular epithelial cells by polymorphonuclear leukocytes with sparing of the hepatocytes. The possible role of the selective destruction of biliary cells in the pathogenesis of biliary disease in MCLS is discussed.     

Possible polyclonal B cell activation in mucocutaneous lymph node syndrome

Immunoserological studies on polyclonal B cell activation were carried out on 39 patients with mucocutaneous lymph node syndrome (MCLS) and in age-matched healthy individuals. The incidence of anti-mite, P. acnes (Kato) and EB virus antibodies, recently proposed as aetiological agents by some investigators, was increased in the patient group. Serum immunoglobulin (Ig) M level and IgM-anti-dinitrophenyl (DNP) antibodies, which are considered to be parameters of polyclonal B cell activation, were determined in MCLS cases. The level of serum IgM in MCLS was significantly elevated (0.02<P<0.05). Levels of anti-DNP antibodies in seven cases of MCLS (18%) were significantly higher than those of the controls (P<0.01). Nine of the ten pair sera in MCLS showed a stage-dependent decrease in anti-DNP antibodies. These results suggest that polyclonal B cell activation occurs in MCLS.Abbreviations MCLS mucocutaneous lymph node syndrome - P. acnes Propionibacterium acnes - EB virus Epstein-Barr virus - D. farinae Dermatophagoides farinae - PBS phosphatebuffered saline - Ig immunoglobulin - RT room temperature - FITC fluorescein isothiocyanate - DNP dinitrophenyl - BSA bovine serum albumin - OD optical density     

Pulmonary disease in children with acquired immune deficiency syndrome and AIDS-related complex

Two major pulmonary diseases were defined on the basis of lung biopsies in 15 children with acquired immune deficiency syndrome (AIDS) or AIDS-related complex. Pneumocystis carinii pneumonia was observed in eight children, and pulmonary lymphoid hyperplasia in six. One child had nonspecific interstitial pneumonitis. Children with P. carinii pneumonia had more severe hypoxemia, with higher alveolar-arterial oxygen gradients, and higher isomorphic elevations of serum lactate dehydrogenase. Clinically, children with pulmonary lymphoid hyperplasia were older, and had digital clubbing, parotid gland enlargement, and elevated serum IgG levels. Results of serologic assays and lung tissue analysis were suggestive of persistent Epstein-Barr virus infection exclusively in patients with pulmonary lymphoid hyperplasia. Recognition of the clinical and laboratory findings characteristic of each entity may assist in the differential diagnosis without the need of surgical biopsy.