Nobuyuki Kiyosawa

Revision of diagnostic guidelines for Kawasaki disease (the 5th revised edition)

Abstract  Diagnostic guidelines for Kawasaki Disease was revised to meet the present situation in 2002. This issue intends to explain new guidelines and their backgrounds. Major alterations are interpretation of cases with 4 or fewer febrile days shortened by early intravenous immunoglobulin treatment, and the clinical importance of atypical (incomplete, or suspected) cases.

Aneurysms of the coronary arteries in Kawasaki disease. An angiographic study of 30 cases.

Thirty patients with coronary aneurysms associated with Kawasaki disease underwent coronary arteriography. Of 53 aneurysms, five were saccular, 24 sacculofusiform, 19 fusiform and five tubular. When tubular aneurysms were included in the fusiform type, the incidence of each configuration in the right coronary artery was almost the same as that in the left coronary artery. The left anterior descending coronary artery had the most aneurysms, followed by the right coronary, left main and circumflex arteries. Right coronary aneurysms always involved the bifurcation or the region from which a branch vessel arose; 13 of 31 left coronary aneurysms did not involve the bifurcation.

Cerebral thrombotic complications in adolescent leukemia/lymphoma patients treated with L-asparaginase-containing chemotherapy

We described the cerebral thrombotic complications developed in 2 adolescent patients treated with L-asparaginase-containing regimens. For determining risk factors, we retrospectively analysed hemostatic markers in 19 pediatric patients with leukemia or lymphoma who were treated with either 1 of the 2 L-asparaginase-containing regimens; 11 were treated with VLP1 and the remaining 8 were treated with the VLAD protocol. The data indicated that low coagulation factors in association with increased plasma D-dimer levels during or post-L-asparaginase administration combined with fresh frozen plasma infusion might have activated coagulation processes in these patients. Careful management is required to prevent such episodes in patients with markedly decreased coagulation factors and increased D-dimer levels following L-asparaginase administration.

Asymptomatic myocardial infarction in Kawasaki disease: Long-term prognosis

SummaryEight patients with Kawasaki disease who had sustained asymptomatic myocardial infarction 8–15 years ago (mean, 13.1 years) were reexamined by various noninvasive cardiac function tests to assess long-term prognosis.At present, electrocardiograms (ECGs) are normal in six patients. However, all eight patients had a prolonged preejection period (PEP) to left ventricular ejection time (LVET) ratio 30 s after amylnitrate (AN) inhalation. Six patients had perfusion defects by exercise thallium-201 myocardial scintigraphy, and two patients developed ST segment depression in treadmill exercise testing.These patients are symptom-free even though their physical activity has not been restricted. Yet they proved to have serious abnormalities suggesting sequelae of myocardial infarction or existing myocardial ischemia. Judging from the results of noninvasive cardiac function tests and recently performed coronary angiography, five of the eight patients require coronary bypass surgery.

Optimal dosage and differences in therapeutic efficacy of IGIV in Kawasaki disease

In an initial study, three groups of patients with Kawasaki disease received either aspirin alone or alkylated immunoglobulin G intravenous preparation (IGIV) 200 mg/kg daily x3 days + aspirin, or 400 mg/kg alkylated IGIV daily x3 days + aspirin. In a second study, three groups of patients were treated with either 100, 200 or 400 mg/kg of native IGIV in combination with aspirin daily for 5 days.

Neutropenia in the acute phase of Kawasaki disease and prevention of coronary artery aneurysm

Background:  The significance of neutropenia in Kawasaki disease (KD) has not been fully elucidated as yet.

Anthracycline—Induced Congestive Heart Failure in two Pediatric Leukemia Cases and Long Term Follow—Up

Endomyocardial biopsy was performed on two leukemia patients who had recovered from severe congestive heart failure (CHF) due to anthracycline cardiomyopathy at 41 months and 47 months after CHF. Microscopic myocardial findings in both patients revealed that myocytes were hypertrophic, but interstitial fibrosis was not observed, suggesting a compensatory mechanism for the damaged heart muscle during the acute episode of CHF. The improvement of clinical symptoms and the normalization of cardiac function, including fractional shortening and ECG changes, is thought to have been associated with this myocardial repairing process.

Survey of severe respiratory syncytial virus infection in Kyoto Prefecture from 2003 to 2007

Background:  Respiratory syncytial virus (RSV) infection in infants can develop into a severe condition.

Exsanguinating bleeding following tooth extraction in a 12-year-old girl: a rare case of acquired haemophilia A.

Acquired haemophilia A (AHA) is a life-threatening haemorrhagic disorder that occurs with various underlying conditions such as autoimmune disease, drug reactions, lymphoproliferative diseases, solid tumours and pregnancy/postpartum status. However, in half of all reported cases, the underlying disease is unknown. Most AHA cases develop in adults; paediatric/adolescent cases are extremely rare. The main clinical symptom is bleeding into the skin, muscles, soft tissues and/or mucous membranes. Here, we report the case of an otherwise healthy 12-year-old girl who presented with prolonged bleeding postexodontia. After being diagnosed with AHA, she was successfully treated with recombinant activated factor VII infusion and oral prednisolone. To avoid such unanticipated bleeding when performing dental extraction, preoperative haemostatic screening tests are recommended.