ABSTRACT
The heart and conduction system were studied in a 4 1/2-year-old boy with Kawasaki disease who died suddenly in the early phase of illness. Severe perivasculitis, neuritis, pancarditis, and involvement of all parts of the specialized conduction system were found. The boy's brother, born a year later, at the age of 12 months had relapsing Kawasaki disease. He received intravenous gamma globulin and recovered.
Subject(s)
Heart Conduction System/pathology , Mucocutaneous Lymph Node Syndrome/pathology , Myocardium/pathology , Aorta/pathology , Cardiovascular System/pathology , Child, Preschool , Coronary Vessels/pathology , Humans , Infant , Male , Mucocutaneous Lymph Node Syndrome/genetics , Mucocutaneous Lymph Node Syndrome/mortality , Myocarditis/complications , Myocarditis/pathology , Recurrence , gamma-Globulins/therapeutic useABSTRACT
Acute aortic dissection may present a clinical picture simulating myocardial infarction, including electrocardiographic changes. The mechanism underlying this mode of presentation has not heretofore been documented during life. We present here for the first time, a patient with acute aortic dissection and the clinical picture of acute myocardial infarction, where the mechanism of infarction has been demonstrated, by preoperative angiographic studies, probably to be due to compression of the extramural portion of the right coronary artery by the false channel of the dissecting hematoma.
Subject(s)
Aortic Aneurysm/complications , Aortic Dissection/complications , Myocardial Infarction/etiology , Aortic Dissection/diagnosis , Aortic Aneurysm/diagnosis , Aortography , Electroencephalography , Humans , Male , Middle AgedABSTRACT
We found that an abnormal hemoglobin with a very low oxygen affinity was responsible for overt cyanosis in an otherwise healthy adolescent. Hemoglobin Beth Israel, in which serine replaces the asparagine residue normally present at position 102 (G4) of the beta-polypeptide chain, was associated with normal blood counts and no apparent exercise intolerance in the heterozygous carrier. Cyanosis resulted from a drastically right-shifted oxygen dissociation curve, whose position and shape could account for the absence of "physiologic" anemia. The whole-blood oxygen tension at 50 per cent oxygen saturation was 88 mm Hg (normally 26 +/- 1 mm Hg), and the arterial blood was only 63 per cent saturated with oxygen despite a normal oxygen tension of 97 mm Hg. The hemolysate showed a low oxygen affinity but normal Bohr effect. Unexplained cyanosis, particularly in association with normal arterial oxygen tension should prompt a search for an abnormal hemoglobin, which may obviate the need for invasive diagnostic procedures.
