Atypical Kawasaki Disease with Aortic Aneurysm

Kawasaki disease (mucocutaneous lymph node syndrome), initially described in Japan, is now being seen with increasing frequency in the United States.1 The diagnosis is based on the typical constellation of signs and symptoms.1,2 Mortality is 1% to 2%, and significant morbidity results from aneurysmal formation in midsize arteries, especially the coronary arteries, which may result in rupture or myocardial infarction. We report an unusual case of Kawasaki disease. The initial febrile illness was an atypical presentation. A large abdominal aortic aneurysm developed, which subsequently was resected. Seven months after the febrile illness an asymptomatic myocardial infarction secondary to bilateral coronary arterial aneurysms was documented.

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Therapy of acute Kawasaki disease

Cardiology in the Young ◽

10.1017/s1047951100000457 ◽

1991 ◽

Vol 1 (3) ◽

pp. 254-255

Author(s):

Jane W. Newburger

Keyword(s):

Myocardial Infarction ◽

Early Childhood ◽

Kawasaki Disease ◽

Sudden Death ◽

Cervical Lymphadenopathy ◽

Unknown Etiology ◽

Acute Kawasaki Disease ◽

Arterial Insufficiency ◽

Infancy And Early Childhood ◽

Arterial Aneurysms

Kawasaki disease is an acute vasculitis of unknown etiology that occurs predominantly in infancy and early childhood. It is characterize by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy.1,2 Coronary arterial aneurysms, or ectasia, develop in approximately 15 to 25% of children with the disease, and may lead to myocardial infarction, sudden death, or chronic coronary arterial insufficiency.2–4

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Lymphadenitis as the Dominant Manifestation of Kawasaki Disease

PEDIATRICS ◽

10.1542/peds.93.3.525 ◽

1994 ◽

Vol 93 (3) ◽

pp. 525-528

Author(s):

Julie Kim Stamos ◽

Kathleen Corydon ◽

James Donaldson ◽

Stanford T. Shulman

Keyword(s):

Kawasaki Disease ◽

Cervical Lymphadenopathy ◽

Febrile Illness ◽

The United States ◽

Acute Febrile Illness ◽

Conjunctival Injection ◽

Coronary Abnormalities ◽

Acquired Heart Disease ◽

Mucosal Changes ◽

Infants And Young Children

Kawasaki disease (KD) is an acute febrile illness primarily affecting infants and young children. Its importance relates to the fact that 20% to 25% of untreated patients develop coronary abnormalities that can lead to myocardial infarction or even to death.1 KD is a leading cause of acquired heart disease in children in many regions, including the United States.2 Because there are no specific diagnostic tests for KD, the diagnosis is established by the presence of fever and four of five criteria without other explanation for the illness: (1) nonexudative conjunctival injection; (2) oral mucosal changes; (3) changes of the peripheral extremities; (4) rash, primarily truncal; and (5) cervical lymphadenopathy.

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Abstract 286: Loss of Smad3 Exacerbates Abdominal Aortic Aneurysm Formation With Enhanced Inflammation in an Experimental Mouse Model

Arteriosclerosis Thrombosis and Vascular Biology ◽

10.1161/atvb.33.suppl_1.a286 ◽

2013 ◽

Vol 33 (suppl_1) ◽

Author(s):

Xiaohua Dai ◽

Anandita Arora ◽

Jianbin Shen ◽

Hong Jiang ◽

Li Li

Keyword(s):

Abdominal Aortic Aneurysm ◽

Aortic Aneurysm ◽

Calcium Chloride ◽

The United States ◽

Protective Role ◽

Elastic Fibers ◽

Aneurysm Formation ◽

Abdominal Aortic ◽

The Media ◽

Chloride Treatment

Introduction Abdominal aortic aneurysm (AAA) is a complex vascular disease that causes more than 10,000 deaths each year in the United States. Extensive studies have been performed in search of pharmaceutical treatment but surgical repair still remains the most effective treatment. TGF-β signaling is an important mechanism in the pathogenesis of aneurysms; however, there is debate as to whether its role is protective or destructive. Smad3 is a major intracellular mediator of the canonical pathway of TGF-β signaling. Hypothesis We hypothesize that Smad3-mediated TGF-β signal pathway plays important roles in the pathogenesis of AAA. Methods To test this hypothesis, we analyze the effects of loss of Smad3 on aneurysm formation in the calcium chloride induced AAA model using Smad3 knockout mice. Results Three weeks after calcium chloride treatment, the abdominal aorta displayed increased dilation, forming aneurysms. Histology and immunohistochemistry analyses show increased cell proliferation and enhanced inflammatory cell infiltration in the media and adventitia of the vessel wall. This was accompanied by elastic fibers degradation, increased MMPs expression and reduced expression of smooth muscle markers. Further analysis showed that the expression and nuclear localization of Smad2 and Smad4 was significantly increased. Conclusions These results demonstrate that Smad3-mediated TGF-β signaling plays a protective role in the pathogenesis of AAA and Smad2/Smad4 upregulation is not sufficient to compensate for the loss of Smad3 in this experimental model.

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Fatal Myocardial Infarction Following Abdominal Aortic Aneurysm Resection

Annals of Surgery ◽

10.1097/00000658-198019250-00013 ◽

1980 ◽

Vol 192 (5) ◽

pp. 667-673 ◽

Cited By ~ 101

Author(s):

NORMAN R. HERTZER

Keyword(s):

Myocardial Infarction ◽

Abdominal Aortic Aneurysm ◽

Aortic Aneurysm ◽

Abdominal Aortic ◽

Aneurysm Resection ◽

Fatal Myocardial Infarction

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Typical and atypical clinical signs and symptoms of myocardial infarction and delayed seeking of professional care among blacks

American Journal of Critical Care ◽

10.4037/ajcc1997.6.1.7 ◽

1997 ◽

Vol 6 (1) ◽

pp. 7-13 ◽

Cited By ~ 28

Author(s):

HO Lee

Keyword(s):

Myocardial Infarction ◽

Chest Pain ◽

Clinical Signs ◽

Signs And Symptoms ◽

The United States ◽

United States Department ◽

Manual Search ◽

Black Patients ◽

Definitive Therapy ◽

Clinical Signs And Symptoms

BACKGROUND: Despite the fact that the effectiveness of thrombolytic therapy for acute myocardial infarction is inversely related to the time between the onset of signs and symptoms and definitive therapy, long delays in seeking treatment have been reported consistently. A variety of reasons for the delays have been suggested. Because such delays are associated with longer hospital stays and higher mortality and morbidity, interventions that reduce delays are especially important. PURPOSE: To examine research on patients with myocardial infarction who delay seeking professional treatment and the factors related to the delay, and to review studies indicating that black patients have premonitory clinical signs and symptoms of myocardial infarction and changes in the structure and function of the cardiovascular system that are different from those in whites. METHODS: Studies were reviewed by using MEDLINE and by doing a manual search of relevant research journals in cardiovascular, nursing, and behavioral medicine published since 1970. Data published by the United States Department of Health and Human Services and the Agency for Health Care Policy and Research were also reviewed. RESULTS: Although the lengths of the delays have varied considerably, blacks have generally experienced longer delays than whites between acute onset of signs and symptoms of myocardial infarction and arrival at the emergency department. Studies show that black patients have a lower incidence of classic chest pain or discomfort but an increased incidence of dyspnea, whereas white patients are much more likely to complain of chest pain. CONCLUSION: Culturally sensitive public education about typical and atypical premonitory clinical signs and symptoms of myocardial infarction and the significance of early treatment of myocardial infarction in blacks is needed.

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