Kawasaki syndrome hospitalizations among children in Hawaii and Connecticut

OBJECTIVES: To estimate the incidence and describe recent trends of Kawasaki syndrome (KS) in 2 different areas of the United States. METHODS: Retrospective analysis of Hawaii and Connecticut State KS hospital discharge records for children younger than 5 years. RESULTS: In Hawaii, 175 KS hospitalizations for children younger than 5 years were reported during 1994 through 1997; the annual hospitalization rate per 100,000 children was 47.7. The rate for Hawaiian children younger than 1 year (83.2) was greater than that for 1- to 4-year-old children (39.0), and most hospitalizations occurred prior to age 2 years (median age, 17 months). In Connecticut, 171 KS hospitalizations for children younger than 5 years were reported during 1993 through 1996; the annual hospitalization rate per 100,000 children was 18.8, and the median age at hospitalization was 28 months. For both states, most hospitalizations were for boys. Although no clear seasonality was apparent, monthly peaks occurred in some of the years from December through March. CONCLUSIONS: Kawasaki syndrome seems to remain an endemic disease in the United States. A high KS annual hospitalization rate was seen in Hawaii, especially in children younger than 1 year, whereas in Connecticut, the KS rate was more consistent with those previously reported in the continental United States. Arch Pediatr Adolesc Med. 2000;154:804-808     

Kawasaki syndrome

Kawasaki syndrome is a fascinating worldwide illness of young children. This acute self-limited vasculitis has become the most common cause of acquired heart disease in children in the United States and Japan. KS causes significant coronary artery disease that may lead to myocardial infarction and sudden death. Clinical and epidemiologic features of KS support an infectious cause, but the etiology remains unknown. Clearly, additional research on the cause of KS and its pathogenesis is needed urgently to allow for improved diagnosis; more specific therapy; and, ultimately, prevention of the disorder.     

Kawasaki syndrome

Kawasaki's syndrome is an acute, largely self-limited multisystem vasculitis of childhood with prominent rheumatic complaints, involving both the heart and the joints. Although the etiologic agent has not been discovered, the evidence appears overwhelming that a microbial agent is the responsible trigger for this multisystem disease. Immunologically mediated phenomena appear to be important in the development of the significant complications of KS carditis, coronary vasculitis, and arthritis. Although truly effective therapy is not yet available, there is an exciting possibility that immunologic treatment may have beneficial effect. More effective preventive and therapeutic methods will become available when the elusive agent is discovered.     

Clinical manifestations associated with Kawasaki disease shock syndrome in Mexican children

Recently, there have been increasing reports of severe forms of Kawasaki disease (KD) associated with shock that have been managed in pediatric intensive care units. It has been suggested that KD is more severe in the Hispanic population. We conducted a study to determine the frequency of Kawasaki disease shock syndrome (KDSS) in our population and compared characteristics between patients with KD without shock and patients with KDSS. Data from 214 patients with KD treated in a tertiary pediatric hospital were collected during a 12-year period. We compared clinical and laboratory features of KD patients without shock and KDSS patients. Of 214 consecutive patients with KD, 11 (5 %) met the definition for KDSS. All of these patients received fluid resuscitation, seven (64 %) required inotropic treatment, and six (54 %), ventilatory support. On admission, seven of these patients (64 %) had an incomplete presentation of the disease, whereas in the group of patients without shock, the relative frequency of an incomplete presentation was 29 %. Twenty percent (3/11) of patients with KDSS presented giant coronary aneurysms versus none of 203 KD patients without shock (p?=?0.001); myocardial infarction, 27 % (3/11), versus 1 % (2/203) (p?=?0.001); and intravenous immunoglobulin (IVIG) resistance, 60 % (6/11), versus 12 % (24/203). Gastrointestinal manifestations in the acute phase occurred in 91 % of KDSS patients versus 30 % patients without shock (p?=?0.001). Conclusion. Patients with KD presenting in shock seem to have an increase in gastrointestinal manifestations, incomplete presentation, IVIG resistance, and worse cardiac outcomes. Larger, prospective, multicentre studies should be carried out to corroborate these findings.     

Kawasaki syndrome in Hawaii

OBJECTIVE: To describe the incidence and epidemiology of Kawasaki syndrome (KS) in Hawaii. METHODS: Retrospective analysis of the State Inpatient Database for Hawaii residents hospitalized with KS during 1996 through 2001. RESULTS: During 1996 through 2001, 267 persons younger than 18 years of age living in Hawaii were hospitalized with KS; 226 (84.6%) were younger than 5 years of age. The average annual incidence for KS was 45.2 per 100,000 children younger than 5 years of age. The incidence was higher for children younger than 1 year of age than for those 1-4 years of age (74.3 and 37.5 per 100,000). The KS incidence for Asian and Pacific Islander children and for White children was 70.9 and 35.3 per 100,000, respectively. Incidence was highest among Japanese American children living in Hawaii (197.7 per 100,000). Honolulu County had the most KS patients (85.0%) and the highest incidence (53.1 per 100,000) among Hawaii counties. For children younger than 5 years of age hospitalized with KS, the median length of stay was 2 days, and the median hospital charge was $9379. CONCLUSION: During 1996 through 2001, the annual incidence rate for KS among children younger than 5 years of age in Hawaii was the highest in the United States. The incidence among Japanese American children in Hawaii was higher than that among other racial groups in the state and when compared with children living in Japan.     

Kawasaki syndrome in Jamaica

OBJECTIVE: To determine the epidemiology, management and outcome of Kawasaki syndrome in patients presenting to the major referral centers in Jamaica (population, 2.5 million) from May, 1986, through June, 1998. DESIGN AND METHODS: Retrospective review of all cases of Kawasaki syndrome in major referral centers island wide. American Heart Association diagnostic criteria were used in case definition. Demographic, clinical diagnostic and laboratory data, management and outcome findings were analyzed. RESULTS: Of 98 probable cases 57 were diagnosed with definite Kawasaki syndrome. The average annual incidence was 2.7 per 100,000 children in the 0- to 5-year age group, in a predominantly black population. Eighty-one percent of cases were from the Kingston Metropolitan area (population, 800,000). Children in the first 3 years of life were represented by 67% of cases, with a mean age of presentation of 32 months (range, 5 to 120 months). There was a male preponderance (M:F ratio, 1.71:1). The mean time between onset of illness and diagnosis was 9.1 days (SD 3.9 days). The most common presenting clinical features included fever, anorexia, vomiting, conjunctivitis, exanthema and oropharyngeal and peripheral extremity changes (>45% of patients). Only 7 children received treatment with intravenous gamma-globulin. Overall 38.8% of patients had cardiovascular changes and 28% had coronary artery abnormalities. Girls (8 of 21, 38.1%) were significantly more likely to have coronary changes than boys (8 of 36, 28.5%) [P < 0.05]. Two (3.5%) patients experienced a recurrence. CONCLUSION: There is a tendency for late recognition and, hence, failure of treatment of Kawasaki syndrome in Jamaica. Greater awareness of the condition needs to be implemented. The finding of female children with an increased occurrence of coronary abnormalities warrants further investigation.     

Kawasaki syndrome in Denmark

OBJECTIVE: To describe the epidemiologic characteristics of Kawasaki syndrome (KS) and to estimate national KS incidence rates among children in Denmark. METHODS: A retrospective population-based study using hospital discharge records with a KS diagnosis for children younger than 15 years selected from the Danish National Hospital Register for 1981-2004. Incidence rates were calculated using the number of KS patients and corresponding census data. RESULTS: During 1981-2004, 360 children younger than 15 years were hospitalized with KS in Denmark, with 73% younger than 5 years. In this age group, the average annual incidence of KS gradually increased from 1981 to 1999 and thereafter stabilized at 4.5 to 5.0 per 100,000 person-years. The incidence was greater for boys than for girls (RR = 1.6, 95% CI = 1.2-2.0) and was highest among infants younger than 1 year (4.5), declining with increasing age (P = 0.03). However, the age-specific decline in incidence was only observed for boys, whereas the incidence for girls remained unchanged by age. The median length of hospital stay was 12 days, and the incidence peaked in the winter months. CONCLUSIONS: Major epidemiologic characteristics identified among Danish childhood KS are consistent with those described in previous studies, such as highest incidence among young children and winter-seasonality. The KS incidence rate among children younger than 5 years in Denmark increased steadily during the early study period (coinciding with global recognition of KS) and seems to have stabilized from 1998-1999 onwards. Although the incidence among Danish children was lower than that reported for several other European countries, differences in methodology challenge definite comparisons.     

Kawasaki syndrome.

Kawasaki syndrome, also known as mucocutaneous lymph node syndrome, is an acute vasculitis of infants and young children. We describe a four-year-old girl who presented with fever, a diffuse erythematous maculopapular rash, bilateral nonpurulent bulbar conjunctivitis, dry, red, fissured lips, a tongue with a strawberry "appearance", an erythematous pharynx, indurative erythema, and edema and desquamation of the face, hands and feet. She probably developed mitral valve prolapse during the course of the disease. The diagnosis of Kawasaki syndrome was arrived at by excluding other diseases and by the presence of all the clinical criteria for Kawasaki syndrome. Since this syndrome is rarely encountered in Turkey, this case is presented and the literature regarding the syndrome is reviewed.     

Nephrotic syndrome in Kawasaki disease

Abstract Renal manifestations in Kawasaki disease are rare, and nephrotic syndrome in this disorder has not been described previously. We report a 3 month old infant with Kawasaki disease, presenting with nephrotic syndrome which responded to steroid therapy. The patient, however, developed coronary aneurysms and died of acute myocardial infarction.     

Pustular rash in Kawasaki syndrome

The skin rash of Kawasaki syndrome is usually erythematous. A 23-month-old Costa Rican boy was admitted with a clinical picture compatible with Kawasaki syndrome, except for his skin lesions. He had diffuse, confluent, multiple sterile whitish pustular lesions on his chest, abdomen, neck, genitals, and thighs.     

Nephrotic syndrome in Kawasaki disease

Renal manifestations in Kawasaki disease are rare, and nephrotic syndrome in this disorder has not been described previously. We report a 3 month old infant with Kawasaki disease, presenting with nephrotic syndrome which responded to steroid therapy. The patient, however, developed coronary aneurysms and died of acute myocardial infarction.     

Alagille综合征五例临床和病理特点

Alagiue综合征是一种可累及肝脏、心脏、骨骼、眼睛和颜面等多系统或器官的显性遗传性疾病,常以婴儿期胆汁淤积为突出表现,临床上和胆道闭锁鉴别困难。国内以往未见该病报道。我院自2004年开始在婴儿期胆汁淤积患儿中明确诊断Alagille综合征5例,并进行了较系统的临床随访和肝脏病理研究,报告如下。