Electrocardiographic and clinical characteristics of idiopathic restrictive cardiomyopathy in children.

BACKGROUND: Idiopathic restrictive cardiomyopathy (RCM) is not a single disease and is rare. METHODS AND RESULTS: The clinical features and clinical course of 12 pediatric patients with RCM seen between 1978 and 2005 were retrospectively analyzed. The age at diagnosis ranged from 4 months to 12 years (median 4 years). The age of 7 patients diagnosed because of an abnormal electrocardiogram (ECG) ranged from 4 to 12 years. Three infants less than 2 years old presented with cardiomegaly. Obliquely elevated ST-T segments and the late peak T waves on 12-lead ECG were present in 8 patients (67%). Three patients with ST depression during exercise had no perfusion defects on radioisotope myocardial perfusion imaging. Two patients underwent orthotopic heart transplantation. Of the remaining 10 patients, 7 have died: 4 died suddenly and 3 died of right heart failure. Three patients with a hypertrophic left ventricular wall had a prolonged survival. The probability of survival at 1, 2 and 3 years was 78%, 52% and 26%, respectively. CONCLUSIONS: Obliquely elevated ST-T segments and the late peak T wave on ECG are characteristic, and reflect the restrictive physiology, which may indicate abnormalities of repolarization of ventricular muscle. The mode of death was either heart failure from pulmonary hypertension or sudden death from presumed ventricular arrhythmia.     

Cell infiltration caused deterioration in the prognosis of patients with clinical diagnosis of dilated cardiomyopathy (DCM): Application of biopsy criteria of myocarditis to 42 autopsy cases

Summary Many investigators consider viral myocarditis as an important cause of a dilated cardiomyopathy (DCM)-like state. In assessing myocarditis in endomyocardial biopsy samples, two sets of criteria, i.e., the Edwards and the Dallas criteria, are employed. However, no criteria have been established for cell infiltration in autopsy cases, nor have biopsy criteria for myocarditis been applied in autopsy cases. We analyzed the clinicopathologic features and small round cell infiltration in 42 autopsy cases whose clinical diagnoses were DCM, employing both the Edwards and the Dallas criteria. Of the 42 cases, 12 (29%) showed positive results for both sets of criteria. These 12 positive patients were proven to have myocarditis by autopsy and they showed more severe clinical features than 30 cases with negative cell infiltration.     

Suppression of adjuvant-induced arthritic bone destruction by cyclooxygenase-2 selective agents with and without inhibitory potency against carbonic anhydrase II.

In vitro assays revealed that COX-2 inhibitors with CA II inhibitory potency suppressed both differentiation and activity of osteoclasts, whereas that without the potency reduced only osteoclast differentiation. However, all COX-2 inhibitors similarly suppressed bone destruction in adjuvant-induced arthritic rats, indicating that suppression of osteoclast differentiation is more effective than that of osteoclast activity for the treatment. INTRODUCTION: Cyclooxygenase (COX)-2 and carbonic anhydrase II (CA II) are known to play important roles in the differentiation of osteoclasts and the activity of mature osteoclasts, respectively. Because several COX-2 selective agents were recently found to possess an inhibitory potency against CA II, this study compared the bone sparing effects of COX-2 selective agents with and without the CA II inhibitory potency. MATERIALS AND METHODS: Osteoclast differentiation was determined by the mouse co-culture system of osteoblasts and bone marrow cells, and mature osteoclast activity was measured by the pit area on a dentine slice resorbed by osteoclasts generated and isolated from bone marrow cells. In vivo effects on arthritic bone destruction were determined by radiological and histological analyses of hind-paws of adjuvant-induced arthritic (AIA) rats. RESULTS: CA II was expressed predominantly in mature osteoclasts, but not in the precursors. CA II activity was inhibited by sulfonamide-type COX-2 selective agents celecoxib and JTE-522 similarly to a CA II inhibitor acetazolamide, but not by a methylsulfone-type COX-2 inhibitor rofecoxib. In vitro assays clearly revealed that celecoxib and JTE-522 suppressed both differentiation and activity of osteoclasts, whereas rofecoxib and acetazolamide suppressed only osteoclast differentiation and activation, respectively. However, bone destruction in AIA rats was potently and similarly suppressed by all COX-2 selective agents whether with or without CA II inhibitory potency, although only moderately by acetazolamide. CONCLUSIONS: Suppression of osteoclast differentiation by COX-2 inhibition is more effective than suppression of mature osteoclast activity by CA II inhibition for the treatment of arthritic bone destruction.     

A CASE OF FOAMY MYOCARDIAL TRANSFORMATION OF INFANCY

A case of Infantile cardiomyopathy in a year and four months old girl, which was clinically characterized by tachycardia resistent to any drugs and marked hyperplasia of mitochondria in their cytoplasms on electron microscopic observation, was presented. In the present case light and electron microscopical proof of the diagnosis was obtained by right endomyocardial biopsy, while most of this condition has not been recognized prior to autopsy. In Japan this newly recognized infantile cardiomyopathy has not been reported, and we proposed that this condition could be designated as mitochondrial cardiomyopathy caused by unknown etiology. The literatures described previously in the world have been reviewed.     

Excretion kinetics of urinary metabolites in a patient addicted to trichloroethylene

Ikeda, M., Ohtsuji, H., Kawai, H., and Kuniyoshi, M. (1971).Brit. J. industr. Med.,28, 203-206. Excretion kinetics of urinary metabolites in a patient addicted to trichlorethylene. A male Japanese subject, single, aged 38, who worked at a workshop washing metal parts with trichloroethylene, was admitted to our clinic due to addiction to the solvent. Analyses of urine revealed the presence of up to 160 μg/ml of trichloro-compounds (mostly trichloroacetic acid) which gradually disappeared in three weeks as the psychotic symptoms cleared up. The excretion half-lives of trichloroethylene metabolites for the initial rapid phase (succeeding slow phase in parentheses) were 5·8 (49·7) hours for trichloroethanol, 22·5 (72·6) hours for trichloroacetic acid, and 7·5 (72·6) hours for total trichloro-compounds.     

Gel-sol transition of methylcellulose

The gelation mechanism of aqueous solutions of methylcellulose (MC) with different molecular weights was studied by small deformation oscillation rheological measurments and differential scanning calorimetry (DSC). The gelation is promoted by the increase in molecular weight of MC: the elastic modulus becomes larger, the gelation occurs at lower temperatures and the DSC peaks accompanying the gel-sol transition become sharper. Cooling DSC curves split into two peaks, indicating that the gel-to-sol transition occurred via two steps.     

Ascending aortic aneurysm associated with a quadricuspid aortic valve

We report here a rare case of ascending aortic aneurysm associated with a tricuspitalized quadricuspid aortic valve. A 45-year-old man had a fusiform ascending aortic aneurysm with aortic valve regurgitation. Transthoracic echocardiography revealed grade III aortic regurgitation. Chest computed tomography showed an ascending aortic aneurysm with a diameter of 48 mm. Surgery revealed that the aortic valve was a tricuspitalized quadricuspid aortic valve with an accessory cusp between the right coronary cusp and left coronary cusp.