ABSTRACT
Two sets of monozygotic [MZ] twins with Kawasaki disease [KD] from two different families are reported. Twin set 1, previously healthy 71-day-old MZ twin girls were diagnosed with incomplete KD and pneumonia. The symptoms occurred at the same time in both girls. Both girls had ectasia of right coronary arteries. In twin set 2, the younger of 18-month-old MZ twin boys was diagnosed with KD and bronchitis. After 53 days, his elder brother was diagnosed with the same disease. The symptoms occurred at different time, but were almost identical. Neither boy displayed coronary artery changes. These findings support the hypothesis that genes susceptible to KD and coronary-artery lesions may exist in families. The different clinical characteristics among MZ twins from different families also suggest diverse and complex nature of KD
Subject(s)
Humans , Male , Female , Twins, Monozygotic , Environment , Pneumonia , Bronchitis , Genetic Predisposition to DiseaseABSTRACT
The clinical data of 76 children with EFE admitted from June, 1998 to December, 2009 was retrospectively analyzed. Tachypnea、 cough and cyanosis were common and all presented with heart failure. Electrocardiogram revealed sinus tachycardia 、left ventricular hyperthrophy and ST-T alterations. On chest radiograph 54 cases (54/76) had a cardiothoracic ratio ＞ 0. 65 and 33 with pneumonia.Echocardiopraphy revealed lowered left ventricular systolic function in all cases and decreased left ventricular diastolic function in 21 (21/76). 63 cases (63/76) presented with thickened and enhanced left ventricular endocardium and 11 (11/76) with thickened interventricular septals. All were treated with digitalis. 58children were improved and discharged, 10 gave up therapy and 8 died during hospitalization. 41 out of 58 improved cases were followed for 1 - 4 years, with 2. 6 years on average. Among them 28 were clinically cured, 7 deteriorated and 6 died of heart failure. Echocardiography is of diagnostic value for ERE. Systolic and diastolic function of the heart can be used to predict the outcome. Pulmonary infection is a major risk factor associated with deterioration. Long-term treatment is imperative to improve the prognosis.
ABSTRACT
Clinical data of 62 children with dilated cardiomyopathy (DCM)admitted to hospital from January 1998 to December 2008 were retrospectively analyzed. The age of patients was from 14 months to 14 year with the average of 8. 2 years. Respiratory infection was the main triggering factor. Cardiac dilatation was the basic conditions for diagnosis of DCM. Arrhythmia was characteristic for DCM, and color cardio uhrasonography was an important procedure of diagnosis and evaluation for cardiomyopathy. With the integrated treatment 38 cases were discharged with improvement ( 61% ), 11 cases died ( 18% ). Early diagnosis and integrated treatment are critical for management of cardiomyopathy in children.