Kawasaki Disease Complicated by Peripheral Gangrene

An 8.5-month-old male infant with Kawasaki disease (KD) received high-dose intravenous immunoglobulin (IVIG) therapy on the fifth day after fever onset. However, multiple peripheral limb ischemias occurred 2 days later. Accordingly, heparin followed by dipyridamole was administered. Aside from a small amputation at the tip of the right middle finger, all other digital ischemias resolved. This presentation demonstrates that early recognition and management of peripheral gangrene in KD may keep its sequela to a minimum.     

Immunological Abnormalities and Endothelial Cell Injury in Kawasaki Disease

Kawasaki disease is an acute febrile disease causing vasculitis, which may lead to as severe a complication as myocardial infarction. This disease is characterized by marked activation of the immune system, including elevation of various cytokines, polyclonal B cell activation, and decrease of CD8+ cells. The high levels of cytokines induce adhesion molecules and a new antigen on endothelial ceils. As a result, the immune cells bind to the endothelium and the activated immune cells produce cytotoxic antibodies against the new antigen on the endothelial cells. Thus, the activated immune system is involved in the endothelial cell injury. IVIG (intravenous immunoglobulin) therapy suppresses the activated immune system and reduces the endothelial cell damage.     

Kawasaki Disease update

Kawasaki Disease is rapidly becoming the most common cause of acquired heart disease in children in both the developed and developing world. Its etiology remains a mystery but important progress has been made in characterizing the features of the arterial wall and myocardial pathology and long-term clinical consequences. New treatments aimed at modifying the host immune response are currently under study. The genetic influence on susceptibility and disease outcome is an area of active research.     

Para-aortic Lymphadenopathy Associated with Kawasaki Disease

Background

Kawasaki disease is an acute vasculitis that occurs mainly in children. Cervical lymphadenopathy is one of the major presenting manifestations of Kawasaki disease. We report a case of Kawasaki disease with para aortic lymphadenopathy, as an unusual feature in this disease.

Case Presentation

This 2.5 year old girl presented with persistent high grade fever, erythematous rash, bilateral non purulent conjunctivitis, red lips, and edema of extremities. Laboratory results included an elevated erythrocyte sedimentation rate, leukocytosis, anemia, and positive C-reactive protein. On second day after admission she developed abdominal pain. Ultrasonography of abdomen revealed multiple lymph nodes around para aortic area, the largest measuring 12mm×6mm. Treatment consisted of aspirin and high dose intravenous γ-globulin. Ultrasonography and CT scan of abdomen performed one week later showed disappearance of the lymph nodes.

Conclusion

There are few previous reports of lymphadenopathy in unusual sites such as mediastinum in Kawasaki disease. Para aortic lymph nodes enlargement might be an associated finding with acute phase of Kawasaki disease. In these patients a close observation and ultrasonographic follow up will prevent unnecessary further investigation.     

Recognizing Kawasaki disease

Kawasaki disease (KD) occurs most often in children from one to three years of age. It is a common systemic vasculitis that is rare in children older than eight years of age or younger than six months of age; however, it may occur in these age groups. Boys are affected more often than girls by a ratio of 1.5 to 1. The mean annual incidence rate reported in a recent Canadian study was 13/100,000 children younger than five years of age. The highest incidence rates occur in Japan. The purpose of the present paper is to help the clinician to recognize KD in children. In particular, the paediatrician must be aware of situations in which KD poses a diagnostic challenge, such as KD in an infant younger than six months of age and the child who presents with a prolonged, unexplained fever but insufficient criteria to make a diagnosis of KD.     

A Case of Refractory Kawasaki Disease Complicated by Peripheral Ischemia

Kawasaki disease (KD), an acute systemic vasculitis that primarily affects infants and young children, is occasionally refractory to initial high-dose intravenous immunoglobulin (IVIG). Some patients with KD develop peripheral gangrene as a fatal complication, though this is rare. There has been no case reported in the literature of complicated peripheral gangrene that completely resolved without sequelae. Recently, we encountered a patient with KD which was refractory to IVIG, later developing peripheral gangrene. For the treatment of a peripheral gangrene dexamethasone, methotrexate, and an anticoagulant were administered, resulting in resolution of the gangrenous lesions without sequelae. Early diagnosis and treatment can reduce permanent sequelae and mortality from refractory KD complicated by peripheral ischemia.     

Kawasaki disease--Indian perspective

Kawasaki disease is a syndrome of unknown etiology affecting children below 5 years of age and is a leading cause of acquired heart disease in many developed countries. Incidence of this disease in India is extremely low as evidenced by the meagre case reports. Complications due to this disease in Indian patients are still rarer. Here we report two cases of Kawasaki disease both of whom had a benign course. A comparison of this disease in Indian and Western literature shows that the incidence of cardiac complications in the Indian patients is about 10% while in the west it is reported at around 30%. This paucity of complications in the Indian patients may be the reason of poor reporting of this disease in our country.     

INFANTILE POLYARTERITIS AND KAWASAKI DISEASE

Abstract. Polyarteritis in infancy is very rare, difficult to diagnose and invariably fatal. A 6-month-old girl who presented with a prolonged unexplained fever and was subsequently demonstrated at post-mortem examination to have polyarteritis is described. The combination of polyarteritis with some unusual presenting features suggests that the case described is one of the Mucocutaneous Lymph Node Syndrome (M.L.N.S.) or Kawasaki Disease. Polyarteritis and Kawasaki Disease are discussed with reference to the case described.     

Kawasaki Disease and Epstein-Barr Virus

We report the results of virological (serological and molecular biological) studies of Epstein-Barr virus (EBV) in patients with Kawasaki disease (KD). Forty-nine (86%) of 57 Kawasaki disease patients and 15 (68%) of 22 patients with recurrent Kawasaki disease had serological evidence of primary Epstein-Barr virus infection during the first month after the onset of their disease based on the results of a sensitive method of detecting antibody to viral capsid antigen (VCA). The serological response to EBV was significantly low and transient. EBV sequences were identified directly in peripheral blood mononuclear cell (PBMC) DNA samples from 23 (56%) of 41 KD patients within 2 weeks after onset by means of the polymerase chain reaction (PCR). EBV sequences were also detected in 10 (83%) of 12 repeatedly tested KD patients within 3 months after onset. In contrast, only 7 (18%) of 40 control DNA samples were PCR-positive. These virological studies indicate that an unusual EBV-cell interaction may occur in KD.     

Infecciones previas o coincidentes con la sospecha de enfermedad de Kawasaki ¿debemos cambiar nuestra actitud?

Introduction

Kawasaki disease (KD) is a multisystem vasculitis associated with coronary artery abnormalities. Infections could be a trigger of the inflammation. The main aim of this study was to describe the presence of infections in children with KD, and to analyse the clinical characteristics and the presence of coronary abnormalities in these cases.

Kawasaki Disease and Agranulocytosis

Kawasaki disease associated with agranulocytosis was observed in a one-year-old girl. The agranulocytic state continued for a month, and granulocyte transfusions were required. We discuss the relation between agranulocytosis and drugs, viral infections, Kawasaki disease, etc. The systemic vasculitis of Kawasaki disease seems to be the cause of the bone marrow disturbance.     

Anticardiolipin Response in Kawasaki Disease

ABSTRACT. Antibodies against cardiolipin are formed in many different infectious diseases, and high levels are associated with susceptibility to thrombosis, especially in patients with systemic lupus erythematosus. In view of the postulated infectious etiology of Kawasaki disease and its association with thrombosis, we have studied the occurrence of anticardiolipin antibodies in this disease. Serial serum specimens from 36 patients were tested, using a solid-phase enzyme immunoassay. A change of at least 0.3 optical density units between two consecutive specimens for at least one immunoglobulin class was observed in 47% of cases. Peak levels occurred one to two weeks after the onset of symptoms. Four patients developed coronary artery aneurysms, and they all showed a clear anticardiolipin response. Anticardiolipin antibodies might be one factor contributing to coagulation abnormalities in patients with Kawasaki disease.     

Two Young Adults Who Had Acute Coronary Syndrome after Regression of Coronary Aneurysms Caused by Kawasaki Disease in Infancy

We report two young adult patients who had acute coronary syndrome after regression of coronary aneurysms caused by Kawasaki disease (KD). A 26 year-old man had acute anterior myocardial infarction at midnight after drinking alcohol. He had had bilateral coronary aneurysms caused by KD at the age of 8 months. Selective coronary angiograms (CAGs) at the age of 7 years revealed regression of both coronary aneurysms. He had no symptoms until the onset of acute myocardial infarction. The other patient was a 24 year-old man diagnosed as having a subendocardial infarction. He had had bilateral coronary aneurysms caused by KD at the age of 1 year. CAGs at the age of 9 years showed that both had regressed. It should be recognized that young adults with apparently normal coronary arteries angiographically after regression of large coronary aneurysms caused by KD may occasionally have acute coronary syndromes. We suspect intimal involvement of the coronary arterial wall after regression of the large aneurysms underlies the acute coronary syndrome in adults. Risk factors for atherosclerosis must be avoided in this population.     

Kawasaki disease in parents and children

Aim: To estimate the probability that the parents of patients with Kawasaki disease also had a history of the same disease. Methods: Self-reported parents' histories of Kawasaki disease were collected from data of the 16th nationwide survey of the disease conducted in Japan from January 1999 to December 2000. The incidence of Kawasaki disease was calculated by using data reported in all 16 nationwide surveys and live births in the Japanese vital statistics. The expected number of parents with a history of Kawasaki disease in the general population, which was calculated by using the assumed number of parents in the vital statistics and the incidence of this disease, was compared with the observed number. Results: Among 14 163 parent pairs of patients with Kawasaki disease, 33 parents (25 mothers and 8 fathers) had a history of the disease. The number of parents expected to have a history of Kawasaki disease was 16.1 (8.4 mothers and 7.7 fathers). From a Poisson distribution, the probability of the observed number was less than 0.001 among parents or mothers. The prevalence of a recurrence of Kawasaki disease and incidences involving siblings of patients whose parents had a history of the disease were five or six times higher than those of all patients who were reported in the 16th survey.

Conclusion: When compared with parents in the general population, the probability of a history of Kawasaki disease was significantly higher in those parents whose children suffered from the same disease. This suggests that, epidemiologically, a genetic predisposition to Kawasaki disease may be implicated in its occurrence.     

Hepatomegaly and Splenomegaly in Kawasaki Disease

Pathologic studies of the liver were performed on 30 autopsied cases of Kawasaki disease. The cases were classified into four groups (stages I-IV), and stage IV was further divided into two subgroups according to the duration of the illness at the time of death. Liver weights were markedly increased in stage II (12-25 days) and in stage III (28-36 days) but returned to normal in stage IVb (7 months to 6 years). Likewise, spleen weights were also markedly increased in stages II and III. Stage I (0-9 days) and stage II were characterized by acute inflammation in portal area, and degree of inflammatory changes decreased gradually. There was significant correlation between hepatomegaly and the degree of inflammation in portal areas, but not with definite heart failure or the use of drugs. These data suggest that the pathogenesis of hepatomegaly in acutee-stage Kawasaki disease involves the inflammation in portal areas and/or latent heart failure.     

Pyuria in patients with Kawasaki disease

Kawasaki disease(KD) is an acute, febrile vasculitis that predominantly develops in children ≤ 5 years of age and can lead to multiple organ injuries including the kidneys. Of these injuries, pyuria is a common feature of patients with KD, occurring in 30%-80% of patients. Sterile pyuria is most common in KD patients ≤ 1 year of age. KD patients with sterile pyuria exhibit more severe inflammatory reactions and may have subclinical renal injuries. Sterile pyuria in KD is associated with mononuclear cells(not neutrophils) in the urine. Although sterile pyuria in KD was at one time thought to be due to urethritis caused by a non-specific vasculitis of the urethra, recent studies suggest that sterile pyuria in KD originates from the urethra, the kidney as a resultof mild and sub-clinical renal injuries, and/or the bladder due to cystitis. Pyuria is not always sterile in KD, but can result from a urinary tract infection(UTI). As causative pathogens, Escherichia coli and Klebsiella oxytoca have been reported. The clinical phenotypes do not differ between those with or without UTI. Because some KD patients with UTIs have urinary tract abnormalities such as vesicoureteral reflux, a complete UTI workup including renal ultrasound, voiding cystourethrogram and/or dimercaptosuccinic acid renal scan recommended in KD patients with UTIs.     

华法林在川崎病合并冠状动脉瘤中的治疗进展

川崎病是儿童急性多系统血管炎性疾病,已成为儿童后天获得性心脏病常见原因之一,其冠状动脉病变是多见并发症.临床上川崎病冠状动脉病变严重程度分5级,其中Ⅳ级(巨大冠状动脉瘤;或1支冠状动脉内多个动脉瘤,但无狭窄者)和Ⅴ级(冠状动脉造影显示有狭窄或闭塞,伴或不伴心肌缺血)患儿推荐长期口服小剂量阿司匹林联合华法林或注射低分子肝素.该文旨在结合国内外研究,从华法林适应证、初始剂量、国际标准化比值、维持剂量范围、华法林在川崎病治疗中的影响因素(年龄、性别、身高、体重、目标INR)、药物与食物相互作用、不良反应以及近年来因华法林在不同个体用药差异性较大而被学者逐渐重视起来的基因研究(如VKORC1、CYP2C9)等来阐述华法林在川崎病合并冠状动脉瘤中的治疗进展.未来需更多高质量大样本多中心随机研究进一步明确影响因素,为其合理有效而安全的应用提供建议,实现精准医疗.     

Chronic Granulomatous Disease Associated with Atypical Kawasaki Disease

Chronic granulomatous disease (CGD) is an infrequent inherited disorder characterized by recurrent infections and abnormal granuloma formation. Patients with CGD have an exuberant inflammatory response and an increased risk of developing autoimmunity. We present the case of a 1-year-old boy with CGD who developed several of the characteristic clinical features of Kawasaki Disease. His illness responded to intravenous immunoglobulin, aspirin, and corticosteroids.     

The Relationship between Coronary Artery Aneurysm and QT Interval Dispersion in Acute Phase of Kawasaki Disease

Objective

QT dispersion (QTd) has been proposed as a marker of ventricular repolarization inhomogeneity and several investigations have proved the relationship between it and cardiac ischemia, ventricular arrhythmia and sudden cardiac death. The aim of this study was to assess the relation between coronary artery involvement and QTd, and QTc dispersion (QTcd) in the acute phase of Kawasaki disease (KD).

Methods

We studied 65 patients with acute KD. Patients were divided into 3 groups. Group one consisted of 48 patients without coronary artery involvement. Group two comprised 13 patients with small to medium size aneurysm. In Group three there were 4 patients with giant aneurysm or multiple small to medium size aneurysms or thrombosis in coronary arteries. For each patient 12 lead electrocardiography was obtained, and QT, QTc, QTd, QTcd, and RR interval were calculated.

Findings

There were 40 males and 25 females with a mean age of 41.4±31.1 months. There was no significant difference in QT, QTc, RR measurements between 3 groups. QTd was greater in group 3 versus group 1 and 2, but the difference was not statistically significant (P=0.06). QTcd was significantly greater in group 3 than in groups 1 and 2 (75.02±11.53 ms versus 46.82±15.39 ms and 48.88±10. 55 ms respectively (P = 0.04). The sensitivity of QTcd ≥60 ms to detect the patients with severe coronary arteries involvement was 100%, the specificity was 93.4%, positive predictive value was 50%, negative predictive value was 100%, and accuracy was 93.8%.

Conclusion

QTcd can be used as a predictive factor for diagnosis of severe coronary arteries involvement in the acute phase of KD.