Katsunori Tatara

Long-term prognosis of giant coronary aneurysm in Kawasaki disease: an angiographic study.

The incidence of coronary obstruction subsequent to giant coronary aneurysm in Kawasaki disease was studied. In 20 cases, aneurysms with a maximal diameter greater than 8 mm were identified by coronary angiography 2 to 120 months (mean 16.9 months) after onset. There were 25 giant aneurysms among these 20 patients, all of whom underwent coronary angiography between 12 and 134 months (mean 31.7 months) after initial examination. Coronary obstruction occurred in six cases (30.0%), all within 4 years of onset of disease. There were five obstructive aneurysms in the right coronary artery (5/12; 41.7%) and two in the left coronary artery (2/13; 15.4%). One of these patients developed symptomatic myocardial infarction. Two had abnormal electrocardiographic findings suggesting myocardial infarction. In five cases, persistent perfusion defects were found by myocardial imaging. On the other hand, in two patients giant aneurysms persisted without obstructive changes for greater than 10 years. In both cases the aneurysm was in the left coronary artery and obstruction of the right coronary artery was found at initial angiography. These results seem to indicate the limited efficacy of antiplatelet therapy for giant coronary aneurysms in Kawasaki disease and that giant aneurysms are likely to progress to become obstructive within a few years even if antiplatelet therapy is given. Other forms of treatment, including surgery, should therefore be considered in such patients.

Decreased resting energy expenditure in patients with Duchenne muscular dystrophy.

BACKGROUND Skeletal muscle metabolism is a major determinant of resting energy expenditure (REE). Although the severe muscle loss that characterizes Duchenne muscular dystrophy (DMD) may alter REE, this has not been extensively investigated. METHODS We studied REE in 77 patients with DMD ranging in age from 10 to 37 years using a portable indirect calorimeter, together with several clinical parameters (age, height, body weight (BW), body mass index (BMI), vital capacity (VC), creatine kinase, creatinine, albumin, cholinesterase, prealbumin), and assessed their influence on REE. In addition, in 12 patients maintaining a stable body weight, the ratio of energy intake to REE was calculated and defined as an alternative index for the physical activity level (aPAL). RESULTS REE (kcal/day, mean±SD) in DMD patients was 1123 (10-11 years), 1186±188 (12-14 years), 1146±214 (15-17 years), 1006±136 (18-29 years) and 1023±97 (≥30 years), each of these values being significantly lower than the corresponding control (p<0.0001). VC (p<0.001) was the parameter most strongly associated with REE, followed by BMI (p<0.01) and BW (p<0.05). The calculated aPAL values were 1.61 (10-11 years), 1.19 (12-14 years), 1.16 (15-17 years), and 1.57 (18-29 years). CONCLUSION The REE in DMD patients was significantly lower than the normal value in every age group, and strongly associated with VC. Both the low REE and PAL values during the early teens, resulting in a low energy requirement, might be related to the obesity that frequently occurs in this age group. In contrast, the high PAL value in the late stage of the disease, possibly due to the presence of respiratory failure, may lead to a high energy requirement, and thus become one of the risk factors for development of malnutrition.

Collateral circulation in Kawasaki disease with coronary occlusion or severe stenosis

Forty patients with Kawasaki disease with severe coronary sequelae were investigated. All had at least a 90% reduction in the diameter of the major coronary artery. Collateral vessels were seen in 32 of 33 (97%) patients with total occlusion. All patients with severe stenosis but not total occlusion had no or poorly developed collateral vessels. Analysis according to the presence or absence of collateral vessels showed no significant differences in the results of treadmill stress testing and myocardial imaging between these two groups. In patients treated surgically, the abnormalities recognized by these tests were normalized or improved when the bypass was patent. These data indicate that collateral circulation in patients with Kawasaki disease cannot be seen angiographically unless there is total occlusion and the presence of collateral circulation cannot provide protection against stress-induced myocardial ischemia.

Efficacy and tolerance of gastrostomy feeding in Japanese muscular dystrophy patients.

Although muscular dystrophy patients often have feeding difficulty and need long-term enteral nutrition, only a few reports have described gastrostomy feeding in these patients. This study was designed to evaluate the efficacy and tolerance of gastrostomy feeding in patients with muscular dystrophy. We performed a retrospective, multicenter study on 144 patients with muscular dystrophy who received gastrostomy feeding between 2007 and 2009 in 25 neuromuscular centers in Japan. There were 77 Duchenne muscular dystrophy (median age at gastrostomy placement 26 years, range 13-47 years), 40 myotonic dystrophy (median age 54.5 years, range 13-70 years), 11 Fukuyama congenital muscular dystrophy (median age 22 years, range 13-29 years), 5 limb girdle muscular dystrophy (median age 62 years, range 43-78 years), and 5 facioscapulohumeral muscular dystrophy (median age 52 years, range 28-67 years) patients. Many benefits including amelioration of malnutrition, swallowing difficulty and respiratory status were observed after the introduction of gastrostomy feeding. Especially in patients with Duchenne muscular dystrophy, mean body weight significantly increased after gastrostomy placement. Although most complications, which are commonly observed in other populations, were tolerable, respiratory failure and peritonitis were important concerns. These findings suggest that gastrostomy placement at an appropriate time is advisable in patients with muscular dystrophy.

Autonomic function in patients with Duchenne muscular dystrophy

Background:  Assessing autonomic function is important for patients with chronic heart failure, but the way that autonomic function changes in patients with Duchenne muscular dystrophy (DMD) and correlates with other clinical parameters during their young age is not clearly known.

Long-term prognosis of Kawasaki disease patients with coronary artery obstruction

SummaryThe prognosis of coronary artery obstruction was studied in patients with Kawasaki disease. Between May 1973 and December 1987, coronary artery obstruction was diagnosed by coronary angiography in 30 patients (21 males, 9 females), of whom, only 8 (26.7%) had clinical symptoms. One patient died after 9 years of illness. Two complained of frequent chest pain, which disappeared after bypass surgery in one case and spontaneously in the other. Five had symptomatic myocardial infarction. Myocardial ischemia was diagnosed in 31.8% by treadmill stress testing, but was well demonstrated in 85.7% by thallium-201 myocardial tomography. Frequent ventricular premature beats, Wenckebachtype atrioventricular block, and ST-segment depression accompanied by chest pain were recognized by 24-h Holter monitoring. In the past, the methods used to determine the prognosis of Kawasaki disease patients with coronary artery obstruction were not adequate. However, the examinations used in this study revealed an improved ability to determine the prognosis in this disease. Myocardial tomography, in particular, provided a more accurate evaluation of myocardial damage. Ventricular arrhythmias seem to be a serious problem in these patients. Therefore, careful observation using these tests, especially myocardial tomography and Holter monitoring, should be done even if the patients are free of symptoms.

Management of severe coronary sequelae of Kawasaki disease.

Abstract Thirty-four patients with severe coronary sequelae of Kawasaki disease were prospectively investigated. Complications included giant coronary aneurysm (15 patients), coronary obstruction (11), stenosis (2) and surgical patients (6). The patients had been managed for four years according to our own follow-up schedule in which we carried out for example treadmill stress tests two or three times a year and myocardial imaging once a year. One patient died after surgery during the observation period while jogging at school. Although his bypass grafts were patent, treadmill tests and myocardial imaging revealed that he had ischemia. Five patients were newly diagnosed with ischemia by treadmill tests and myocardial imaging, and all underwent bypass surgery. Even after successful surgery, it was necessary to wait for normal ischemic findings in some patients. We conclude that serial treadmill stress tests and myocardial imaging are mandatory in managing severe coronary sequelae of Kawasaki disease. Restriction of daily activity and exercise is also necessary after diagnosis of ischemia by treadmill tests.

Development of atopic asthma in infants with pulmonary congestion caused by congenital heart disease.

We studied the effects of pulmonary congestion on the development of atopic asthma in 31 infants with congenital heart disease. Respiratory distress did not resolve after surgery in seven patients, six of whom had a family history of allergy. Pulmonary congestion in infancy may increase the risk of atopic asthma in genetically predisposed children.

Contribution of pulmonary hemodynamics on manifestation of allergic asthma in patients with congenital heart disease

The purpose of this study was to determine whether pulmonary hemodynamic abnormalities relate to manifestations of allergic asthma.

国内筋ジストロフィー専門入院施設におけるDuchenne型筋ジストロフィーの病状と死因の経年変化（1999年～2012年）

To elucidate changes in medical treatment for Duchenne muscular dystrophy (DMD) in Japan, we analyzed the clinical courses and causes of death of inpatients with DMD registered in the muscular dystrophy ward database of 27 hospitals in Japan specializing in muscular dystrophy treatment since 1999. The total number of hospitalized cases in 1999 was 873, which gradually reduced to 733 in 2012. The mean age of DMD patients in 1999 was 23.6 years old, while that was 30.1 years old in 2012, with patients 40 years and older accounting for 94 cases in the latest year. The respirator dependent rate gradually increased from 58.6% in 1999 to 86.1% in 2012. Artificial respiration therapy was introduced earlier in more recent years and the mean age in recent years was shown to be 17.2 years old. The oral nutritional supply rate in 1999 was 95.1%, which fell to 66.8% in 2012, while gastrostomy feeding gradually increased to 129 cases in 2012. The rate of clinical diagnosis of DMD was 52.3% in 1999 and decreased to 43.7% in 2012, which showed progress towards more accurate diagnosis of DMD. From 2000 to 2012, 521 deaths were reported, with approximately half of the causes heart related, followed by respiratory related. The mean age of death gradually increased to 32.4 years old in 2012 from 26.7 years old in 2000. The mean age of survival of all DMD patients was 37.5 years old. Progress in multidisciplinary medical care for respiratory failure, cardiomyopathy, nutritional problems, and other related factors has extended the lifespan of DMD patients.