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.     

The mucocutaneous lymph node syndrome or Kawasaki disease]

A case of "mucocutaneous lymph node syndrome" is presented. This syndrome is a nosological entity with clearly defined diagnostic criteria. The disease has mainly been observed in Japan, rarely in other countries. It is named according to its main manifestations on skin, lymph nodes and mucous membranes. We discuss the differential diagnosis and mention the danger of a severe course of this syndrome.     

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.     

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     

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.     

Thallium 201 scintigraphy in an infant with myocardial infarction following mucocutaneous lymph node syndrome

A 4-month-old boy with myocardial infarction following mucocutaneous lymph node syndrome is described. — Angiocardiogram revealed multiple aneurysms of the right and left main coronary arteries, complete occlusion of the left anterior descending and left circumflex coronary arteries, and post-infarction aneurysm of the left ventricle. — Thallium 201 scintigram demonstrated the infarcted area precisely and repeat examinations showed the improvement of the myocardial perfusion, which was probably due to the development of the collateral circulation.