1/100. Kawasaki syndrome-like illness associated with infection caused by enterotoxin B-secreting staphylococcus aureus.Two children had symptoms and clinical signs that were characteristic of the diagnostic criteria for Kawasaki syndrome, temporally associated with staphylococcus aureus bacteremia. One child initially had focal osteomyelitis that was evident clinically and radiographically, and radiographic evidence of multifocal osteomyelitis was noted at follow-up. The blood-borne S. aureus isolates from these two patients secreted staphylococcal enterotoxin B and were negative for toxic shock syndrome toxin. Staphylococcal and streptococcal superantigens may play a role in the pathogenesis of some cases of Kawasaki syndrome or Kawasaki syndrome-like illness.- - - - - - - - - - ranking = 1keywords = illness (Clic here for more details about this article) |
2/100. Kawasaki disease: A brief history.Tomisaku Kawasaki published the first English-language report of 50 patients with Kawasaki disease (KD) in 1974. Since that time, KD has become the leading cause of acquired heart disease among children in north america and japan. Although an infectious agent is suspected, the cause remains unknown. However, significant progress has been made toward understanding the natural history of the disease and therapeutic interventions have been developed that halt the immune-mediated destruction of the arterial wall. We present a brief history of KD, review progress in research on the disease, and suggest avenues for future study. Kawasaki saw his first case of KD in January 1961 and published his first report in Japanese in 1967. Whether cases existed in japan before that time is currently under study. The most significant controversy in the 1960s in japan was whether the rash and fever sign/symptom complex described by Kawasaki was connected to subsequent cardiac complications in a number of cases. Pathologist Noboru Tanaka and pediatrician Takajiro Yamamoto disputed the early assertion of Kawasaki that KD was a self-limited illness with no sequelae. This controversy was resolved in 1970 when the first Japanese nationwide survey of KD documented 10 autopsy cases of sudden cardiac death after KD. By the time of the first English-language publication by Kawasaki in 1974, the link between KD and coronary artery vasculitis was well-established. KD was independently recognized as a new and distinct condition in the early 1970s by pediatricians Marian Melish and Raquel Hicks at the University of hawaii. In 1973, at the same Hawaiian hospital, pathologist Eunice Larson, in consultation with Benjamin Landing at los angeles Children's Hospital, retrospectively diagnosed a 1971 autopsy case as KD. The similarity between KD and infantile periarteritis nodosa (IPN) was apparent to these pathologists, as it had been to Tanaka earlier. What remains unknown is the reason for the simultaneous recognition of this disease around the world in the 1960s and 1970s. There are several possible explanations. KD may have been a new disease that emerged in japan and emanated to the western world through hawaii, where the disease is prevalent among Asian children. Alternatively, KD and IPN may be part of the spectrum of the same disease and clinically mild KD masqueraded as other diseases, such as scarlet fever in the preantibiotic era. case reports of IPN from Western europe extend back to at least the 19th century, but, thus far, cases of IPN have not been discovered in japan before world war ii. Perhaps the factors responsible for KD were introduced into japan after the world war ii and then reemerged in a more virulent form that subsequently spread through the industrialized western world. It is also possible that improvements in health care and, in particular, the use of antibiotics to treat infections caused by organisms including toxin-producing bacteria reduced the burden of rash/fever illness and allowed KD to be recognized as a distinct clinical entity. Itsuzo Shigematsu, Hiroshi Yanagawa, and colleagues have conducted 14 nationwide surveys in japan. These have indicated that: 1) KD occurred initially in nationwide epidemics but now occurs in regional outbreaks; 2) there are approximately 5,000 to 6,000 new cases each year; 3) current estimates of incidence rates are 120 to 150 cases per 100,000 children <5 years old; 4) KD is 1.5 times more common in males and 85% of cases occur in children <5 years old; and 5) the recurrence rate is low (4%). In 1978, David Morens at the Centers for disease Control and Prevention published a case definition based on Kawasaki's original criteria. The Centers for disease Control and Prevention developed a computerized database in 1984, and a passive reporting system currently exists in 22 states. Regional investigations and national surveys suggest an annual incidence of 4 to 15 cases per 100 000 children <5 years o- - - - - - - - - - ranking = 0.40344262955723keywords = illness, state (Clic here for more details about this article) |
3/100. Kawasaki-like syndromes associated with human immunodeficiency virus infection.Kawasaki disease is an acute febrile vasculitic syndrome of early childhood. It is very rarely seen in adults. Among the adult patients with Kawasaki disease who have been described, a disproportionate number are infected with human immunodeficiency virus (hiv). This suggests that the immunocompromised state may predispose individuals to this syndrome. We report our experience with 2 hiv-positive patients who presented with Kawasaki-like syndromes and review the published literature on hiv-positive patients with similar syndromes.- - - - - - - - - - ranking = 0.0034426295572343keywords = state (Clic here for more details about this article) |
4/100. Classical Kawasaki disease in a neonate.A case is reported of classical Kawasaki disease in an infant younger than 2 weeks of age. echocardiography detected a coronary artery aneurysm on the fifth day of the illness. Administration of intravenous gamma globulin resulted in rapid improvement. Kawasaki disease is rare in neonates, but it may follow a rapid and severe course.- - - - - - - - - - ranking = 0.2keywords = illness (Clic here for more details about this article) |
5/100. Assessment of coronary function in children with a history of Kawasaki disease using (15)O-water positron emission tomography.BACKGROUND: Coronary abnormalities after Kawasaki disease (KD) may be associated with endothelial dysfunction due to intimal hypertrophy. The purpose of this study was to evaluate myocardial flow reserve (MFR) and endothelial function in regressed aneurysmal regions after KD. methods and Results- Subjects were 12 patients aged 16.0 /-2.6 years who suffered from KD at 1.7 /-1.5 years and 12 normal subjects aged 26.5 /-3.4 years. MFR and endothelial function were estimated, respectively, by changes in myocardial blood flow (MBF) during ATP infusion and by that during cold pressor test using (15)O-water positron emission tomography. Data from 24 regressed aneurysmal regions were compared with those from the corresponding regions (n=36) in the control group. Although the MBF at rest in the regressed aneurysmal regions was similar to that in controls, the MBF at a hyperemic state induced by ATP infusion in the regressed aneurysmal regions was significantly lower than that in the control regions. Therefore, the MFR in regressed aneurysmal regions was significantly lower than that in controls (3.53 /-0.95 versus 4.60 /-1.14; P<0.05). MBF at rest and during the cold pressor test did not change in the control regions, but it was significantly reduced in regressed aneurysmal regions. The ratio of MBF during the cold pressor test to MBF at rest was significantly lower in regressed aneurysmal regions than in control regions (0.67 /-0.15 versus 1.00 /-0.15; P<0.05). CONCLUSIONS: MFR and endothelial function are often impaired in regressed aneurysmal regions after KD, and tomography enables the noninvasive evaluation of coronary function.- - - - - - - - - - ranking = 0.0034426295572343keywords = state (Clic here for more details about this article) |
6/100. A case of intravenous immunoglobulin-resistant Kawasaki disease treated with methotrexate.Kawasaki disease, an acute febrile vasculitis of unknown etiology, is usually treated with high doses of immunoglobulin (IVIG) and aspirin. However, 20% of children show persistent or recurrent fever despite IVIG, and coronary artery aneurysm progression. In such cases of resistance to IVIG treatment, repeated IVIG administration or the initiation of steroid therapy, and the use of cyclophosphamide have been reported. We aimed to show in this study that methotrexate (MTX) may be used as a treatment for Kawasaki disease resistant to IVIG treatment. We report the case of a 6-year old boy who was admitted at another hospital with an initial complaint of a fever for 5 days and skin rashes for 3 days. The patients fever persisted despite three courses of IVIG (2 gm/kg, 1 gm/kg, 1 gm/kg, respectively) over a 14-day period. On day 14 of his illness he showed a dilated right coronary artery, and on day 19 dexamethasone, at a daily dose of 0.3 mg/kg, was given but this resulted in defervescence. However, upon stopping the dexamethasone treatment, his fever recurred and he was transferred to our hospital. On days 31 and 38 of his illness, IVIG (400 mg/kg for 5 days, twice) was administered and from day 38 onwards the patient was given dexamethasone (0.6 mg/kg, daily) and MTX (10 mg/BSA, once weekly) whereupon his fever subsided and did not recur. On day 48 dexamethasone was replaced with prednisolone, which was subsequently tapered. The patient is now taking MTX and being observed on an outpatient basis. We report the case of a boy with IV-globulin resistant Kawasaki disease, who after repeated infusions of IVIG and steroid therapy showed fever recurrence, which that subsided after MTX treatment.- - - - - - - - - - ranking = 0.4keywords = illness (Clic here for more details about this article) |
7/100. Large vessel arteritis associated with human herpesvirus 6 infections.A 9-month-old boy presented with chronic arteritis of the aorta and its major branches. The clinical manifestations at onset of his illness were compatible with those of Kawasaki syndrome. However, the febrile period lasted for 2 months despite various immunosuppressive therapies, and the levels of c-reactive protein remain high 18 months after onset. Elevated circulating immune complexes, decreased serum complement levels, hypergammaglobulinaemia and monoclonal gammopathy were observed. Active HHV-6 infection was shown by increased serum levels of antihuman herpesvirus-6 (HHV-6) IgG and IgM antibodies, and positive HHV-6 dna in sera, peripheral blood mononuclear cells (PBMNC) and lymph nodes. HHV-6 was actively replicating in PBMNC and lymph nodes, as shown by the detection of transcripts for the virus structural antigen. These results suggest that large vessel arteritis can be associated with HHV-6 infection.- - - - - - - - - - ranking = 0.2keywords = illness (Clic here for more details about this article) |
8/100. Sudden death in a 21-year-old man caused by thrombosed coronary aneurysm: late sequelae or a very late onset of Kawasaki disease?Kawasaki disease (KD; mucocutaneous lymph node syndrome) is an infantile febrile illness of unknown origin that occurs in worldwide epidemics. The main clinical features of the disease, such as fever, mucositis, cervical lymphoadenopathy and skin rash, associated with coronary aneurysms or arteritis are diagnostic. Late sequelae of coronary arteritis are very rare in the second decade of life; we describe the case of a 21-year-old male who died suddenly. The autopsy revealed a large thrombotic coronary aneurysm, coronary arteritis and myocarditis, supporting the diagnosis of a late fatal sequela of KD. The hypothesis of a very late onset of the disease is also considered and discussed.- - - - - - - - - - ranking = 0.2keywords = illness (Clic here for more details about this article) |
9/100. Fatal late onset necrotising enterocolitis in a term infant: Atypical Kawasaki disease or polyarteritis nodosa of infancy?A 3-month-old infant died following a 3-week illness that commenced with diarrhoea and vomiting, and produced progressive infarction of his small bowel. Post-mortem identified multiorgan arteritis of uncertain aetiology, giving rise to coronary artery occlusion and myocardial infarction as well as necrosis of the entire small bowel and other organs. Kawasaki disease and polyarteritis nodosa of infancy are proposed as possible aetiologies in this case.- - - - - - - - - - ranking = 0.2keywords = illness (Clic here for more details about this article) |
10/100. Kawasaki disease resistant to immunoglobulin g therapy.We describe a 3-year-old boy with Kawasaki disease who, despite therapy with two doses of intravenous immunoglobulin g and high-dose aspirin, developed myocarditis in the 1st week of illness and coronary artery aneurysms in the 2nd week. He required a short course of systemic corticosteroids for remission of the illness. The myocarditis subsided and coronary aneurysms remained static and uncomplicated during 6 weeks of follow-up. The diagnosis and recent trends in management of this not uncommon vasculitic disorder are outlined.- - - - - - - - - - ranking = 0.4keywords = illness (Clic here for more details about this article) |
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