Images in cardiovascular medicine. Giant coronary aneurysm of Kawasaki disease developing during postacute phase.
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Biomedical subjects
Publications and source records attributed to T Kosuda.
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This is a report of two-year-old female monozygotic twins, both having a ventricular septal defect (VSD, Kirklin type II) and accompanying moderate pulmonary hypertension (PH). The two patients underwent a patch closure procedure to repair the VSD at the same time, and their postoperative courses were uneventful. Rubenstein and Weaver have reported the only case of surgical treatment for monozygotic twins having both VSD and PH. Our patients are the second such case of surgical treatment of VSD and PH in monozygotic twins.
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A 61-year-old man was admitted to our hospital with pain and weakness in both lower legs. Eosinophil count was abnormally high (6800/mm3). Chest X-ray film revealed multiple infiltrates in both lung fields. A specimen obtained by transbronchial lung biopsy demonstrated pulmonary hemorrhage. Vasculitis syndrome was suspected and 1 g per day of methylprednisolone was administered for 3 days. Pulmonary infiltrates quickly disappeared, but 2 weeks later massive intestinal hemorrhage occurred. Emergency ilectomy was done, but the patient died of bleeding from other intestinal lesions. Microscopic examination of the resected ileum revealed mucosal ulceration and necrotizing vasculitis in small arteries. This case is important in that vascular occlusion and ischemia progressed soon after vascular inflammation was relieved by administration of corticosteroid hormone.
A 45-year-old man with diffuse infiltrates on the chest X-ray film was admitted to the hospital. At first, sarcoidosis was suspected due to the elevated serum angiotensin-converting enzyme (ACE) activity. Transbronchial lung biopsy and bronchoalveolar lavage, however, did not lead to that diagnosis. Pulmonary alveolar proteinosis (PAP) was diagnosed after an open lung biopsy. The diffuse infiltrates on his chest X-ray film diminished spontaneously. Two years later, diffuse infiltrates increased again with an elevated serum ACE activity. In this case, it seemed that the severity of PAP was associated with serum ACE activity. We review the literature on the relationship between serum ACE activity and dysfunction of alveolar macrophages in PAP.
Case 1. A 40-year-old Japanese female with a 11 years history of sarcoidosis. Biopsy of a 0.5 cm-sized right breast nodule showed sarcoid granuloma. About two years later she had had bloody galactorrhoea and biopsy of an apparently normal site of the breast revealed periductal sarcoid granuloma and duct papillomatosis. Case 2. A 63-year-old Japanese female in whom biopsy of a 1 cm-sized right breast nodule revealed sarcoid granuloma. BHL, lung, eyes, muscle, lymph nodes and skin lesions were subsequently detected and the last two were histologically confirmed as sarcoidosis. After nine months all lesions disappeared. Some problems presented in the literature such as periductal sarcoid granuloma formation, latent sarcoidosis in the grossly normal breast and coexistent breast cancer are discussed briefly.
Thyroid disorders were detected in 3.7% of our 269 cases of sarcoidosis histologically confirmed. This is close to the rate in the literature. As a probability of this complication that some of closely allied autoimmune disorder may relate both disease, except by chance. In 2 of our cases, these diseases appeared alternately as follows. A 49 years old woman suffered from granular-hard struma with hypothyroid from 4 years ago when sarcoidosis had completely healed. On the other side, a 55 years old woman had the apparent struma for about 16 years from her 6 years of age and gradually disappeared after the signs of sarcoidosis became apparent. She also had a Basedow's patient in her family.
Dipyridamole-loading cineventriculography was performed to evaluate left ventricular (LV) function in 76 patients with Kawasaki disease. Forty-five of 76 patients underwent the procedure within 6 months from the onset of illness (group 1A, normal findings on coronary angiogram; group 1B, maximal diameter of coronary lesions less than 4 mm; group 1C, maximal diameter of coronary lesions greater than 4 mm). Thirty-one of 76 patients underwent the procedure greater than 6 months after onset (group 2A, coronary lesions improved to normal or less than 4 mm; group 2B, remaining coronary lesions greater than 4 mm; group 2C, stenosis or obstruction of the coronary artery). Global LV ejection fraction increased in groups 1A, 1B and 2A after infusion of dipyridamole, but there was no significant change in groups 1C, 2B and 2C. In analyzing LV excursion, the pattern of LV wall motion with dipyridamole in groups 1B and 2A were similar to that in group 1A, but in 38% (5 of 13) of group 2A patients, abnormal regional LV wall motion after administration of dipyridamole was detected. Abnormal LV wall motion after dipyridamole infusion was detected in 42% (5 of 12) of group 1C patients, 38% (3 of 8 cases) of group 2B patients and 70% (7 of 10 cases) of group 2C patients. In conclusion, analysis of LV wall motion with dipyridamole is useful in evaluating cardiac involvement in Kawasaki disease.
A 21-year-old man developed hypopituitarism, with symptomatic hypogonadism and diabetes insipidus (DI), as well as uveitis, retinal vasculitis, and papilledema in association with systemic sarcoidosis. A suprasellar tumor was demonstrated by computed tomography (CT). Although ophthalmic symptoms disappeared with prednisone and the DI was controlled with Desmopressin (DDAVP), the hypogonadism did not improve with human menopausal gonadotropin (HMG) and human chorionic gonadotropin (HCG). In long-term follow-up, the hypogonadism unexpectedly resolved.
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The clinical course of 775 sarcoidosis cases in Japan collected by the Japan Sarcoidosis Committee was followed up in the 6th month, 1st, 2nd, 3rd, 4th-6th, 7th-9th and 10th year and over after the first discovery. The means follow-up period was 5.5 years. The accumulated rates of disappearance of BHL, lung, eye, and whole sarcoidosis lesions calculated by life-table method were 63%, 56%, 59% and 48% respectively at the 1st year, 75% 67%, 71% and 59% respectively at the 2nd year, 79%, 71%, 76% and 64% respectively at the 3rd year, 82%, 74%, 81% and 67% respectively at the 4th-6th year. Judging from the accumulated rates mentioned above, the speakers defined the course of the disease as "good" when the whole sarcoidosis lesions cleared within 2 years. It was revealed that out of 25 factors examined, factors such as "age under 29", "no eye lesions" and "without complaints" at the time of first discovery, were significantly useful for pre-dieting the "good" results. The disappearance of BHL within 6 months was one of the most important indices predicting the "good" outcome of sarcoidosis.