A boy with multicore myopathy associated with multiple pterygium syndrome and hypertrophic cardiomyopathy is described. Light microscopy of biopsy samples from the skeletal muscle and myocardium revealed multiple cores in the muscle fibers in the former but their absence in the latter. These results suggest that the pathogenesis of the histologic changes might differ between skeletal muscle and myocardium, and that further electron microscopic examination be done on both types of specimen. The prognosis of multicore myopathy is not usually good when cardiac involvement is present.

Abnormalities, Multiple↗

PubMedDate not supplied

Age dependency of stiffness of the abdominal aorta and the mechanical properties of the aorta in Kawasaki disease in children.

Measuring aortic distensibility has been shown to be useful in adults as a noninvasive method in the early detection of atherosclerosis. This study had two purposes: to assess the stiffness of the abdominal aorta by using two-dimensional echocardiography (2DE) in healthy neonates, children, and adults and to assess aortic distensibility in children with Kawasaki disease in acute and subacute phases. The study comprised 168 healthy subjects and 40 patients with Kawasaki disease. We recorded systolic (Ps) and diastolic (Pd) blood pressure and measured aortic diameter (Dd) at both minimum diastolic pressure and maximum systolic expansion (Ds) by 2DE. These measurements were used to determine (1) aortic strain (S) = (Ds - Dd)/Dd, (2) pressure strain elastic modulus (Ep) = (Ps - Pd)/S, and (3) normalized Ep (Ep*) = Ep/Pd. Significant correlations were found between S and age, Ep and age, and Ep* and age. In Kawasaki disease, Ep and Ep* showed negative correlations to day after onset. The aorta was less distensible in infants, became soft in 12- to 16-year-olds, and then stiffened with increasing age among normal subjects. In Kawasaki disease, aortic stiffness was high at the acute phase and normal at the subacute phase. These tendencies may be related to the biological characteristics of smooth muscle cells.

Acute Disease↗

PubMedDate not supplied

Clinicopathologic characteristics of hypertrophic cardiomyopathy detected during mass screening for heart disease.

Between 1981 and 1992 a total of 10 patients with hypertrophic cardiomyopathy (HCM) were detected by mass screening for heart disease in Tokyo's Adachi Ward. Four were first grade elementary school children and six were first grade junior high school adolescents. Two-dimensional echocardiography at the initial evaluation revealed asymmetric septal hypertrophy in four patients, diffuse hypertrophy of the left ventricle in five, and poor left ventricular contractility with wall thinning in one (dilated phase). Three of the five patients with diffuse hypertrophy progressed to asymmetric septal hypertrophy during the average 4-year follow-up period. The degree of septal thickness and the left ventricular wall thickness index were significantly less than in those of young adult controls (12 +/- 3 versus 21 +/- 9 mm, p < 0.05; and 22 +/- 4 versus 28 +/- 16 mm, p < 0.05, respectively). Right ventricular endomyocardial biopsy specimens obtained from 9 of the 10 patients showed features typical of HCM (e.g., myocyte hypertrophy with myofibril disarray) in five patients and atypical features (mainly interstitial fibrosis with perivascular cell infiltration) in another four. One patient with dilated phase disease died of congestive heart failure 6 months after the initial evaluation. These results indicate that HCM detected during mass screening is a mild form of the disease and may have atypical pathologic features, such as interstitial fibrosis and perivascular cell infiltration, mimicking the sequela of chronic myocarditis.

Adolescent↗

PubMedDate not supplied

Dilatation mechanism of balloon angioplasty in children: assessment by angiography and intravascular ultrasound.

PURPOSE: Little information is available about the dilatation mechanism in children. This prospective study aimed to (1) evaluate the dilatation mechanism of balloon angioplasty in children with arterial stenosis, and (2) compare the morphological changes seen by intravascular ultrasound (IVUS) and angiography. METHODS: Twenty consecutive patients, who had undergone a total of 23 procedures, were examined before and immediately after balloon angioplasty with a 4.3 Fr, 30 MHz rotational tip IVUS system. The lesions for IVUS study had resulted from coarctation of the aorta in six patients, pulmonary arterial stenosis in five, Blalock-Taussig shunt stenosis in three, subclavian artery stenosis in two, renal artery stenosis in two, coronary artery stenosis in one and ductus arteriosus in one. RESULTS: Four distinctive morphological types were identified: type I with arterial stretching, type IIa with superficial tearing, type IIb with deep intimal-medial tearing, type III with flap formation, and type IV with dissection. The diameter of the narrowest site before and after balloon angioplasty increased significantly from 2. 1 +/- 1.4 mm to 4.6 +/- 3.4 mm (p < 0.001). Eighteen of the 23 angioplasty procedures (78%) were considered to be successful, with a dilatation ratio of more than 50%. In most patients with successful dilatation, non-stretch mechanisms such as tearing, flap formation, or dissection were found. The positive percent (70%) of non-stretch mechanisms seen by IVUS was significantly higher than the positive findings (39%) by angiography (Chi2 = 6.47, p < 0.02). CONCLUSIONS: Non-stretch morphology of the arterial wall may be a common mechanism of dilatation after balloon angioplasty in children with arterial stenosis. IVUS is a useful modality for evaluating the effectiveness of balloon angioplasty and the mechanism of dilatation in individual cases.

Adolescent↗

PubMedDate not supplied

Intensive sequential post-induction therapy for adults with acute myelogenous leukemia in first remission: long-term follow-up and results.

We designed a post-induction therapy including intensive sequential therapy with non-cross-resistant drugs in an effort to prolong disease-free survival (DFS) for adults with acute myelogenous leukemia. Forty-five patients entered this study and 33 of 35 patients entering complete remission received the post-induction therapy. With a median follow-up for survivors of 3.5 years from complete remission, the actuarial 5-year DFS was 46% +/- 19% (95% confidence interval). The five-year DFS for patients over 45 years of age was comparable to that for patients under 45 years of age (50% +/- 26% vs 47% +/- 28%). Furthermore, the actuarial 5-year DFS for patients who required two courses of induction therapy was comparable to that for patients who required only one course of induction therapy (45% +/- 29% vs 50% +/- 25%). The toxicity of post-induction therapy was tolerable and no patients died during complete remission.

Aclarubicin↗

PubMedDate not supplied

Mutagenicity of N-acetyl and N,N'-diacetyl derivatives of 3 aromatic amines used as epoxy-resin hardeners.

3 epoxy-resin hardeners, 4,4'-diaminodiphenyl ether (DDE), 4,4'-diaminodiphenylmethane (DDM), and 4,4'-diaminodiphenylsulfone (DDS), and their N-acetyl and N,N'-diacetyl derivatives were examined for their mutagenicity using Salmonella typhimurium TA98 and TA100 as the tester stains and an S9 mix containing a rat-liver 9000 X g supernatant fraction as the metabolic activation system. DDE and DDM were mutagenic towards TA98 and TA100 in the presence of S9 mix while DDS exhibited no significant mutagenic activity towards these tester strains. These epoxy-resin hardeners were metabolized in vivo and their N-acetyl and N,N'-diacetyl metabolites were found in the urine. Among these acetyl metabolites, only N-acetyl-DDE was found to be mutagenic towards TA98 and TA100 in the presence of S9 mix. None of these acetyl metabolites exhibited significant mutagenic activity towards these tester strains in the absence of S9 mix.

Aniline Compounds↗

PubMedDate not supplied

Balloon valvuloplasty in pulmonary atresia and intact ventricular septum.

Explore the source record for details and available documents.

Catheterization↗

PubMedDate not supplied

7-Desmethyl-microcystin-RR, a hepatotoxin from a waterbloom of Microcystis aeruginosa.

A peptide toxin was isolated from a waterbloom of Microcystis aeruginosa from Lake Frøylandsvatn in Norway. The isolation procedure included liquid and solid phase extraction and reversed phase high performance liquid chromatography. Amino acid analysis yielded D-glutamic acid, D-erythro-beta-methylaspartic acid and D-alanine in equimolar and L-arginine in twofold molar ratios. The presence of dehydroalanine was confirmed by hydrogenation and subsequent amino acid analysis with combined gas liquid chromatography/mass spectrometry. Investigation of the toxin with fast atom bombardment mass spectrometry showed a nominal relative molecular mass of 1023. 3-Amino-9-methoxy-2,6,8-trimethyl-10-phenyl- 4,6-decadienoic acid (Adda) was identified by 1H NMR and 1H, 1H COSY spectroscopy. The structure of the toxin was elucidated as 7-desmethyl-microcystin-RR.

Amino Acid Sequence↗

PubMedDate not supplied

Heparin coated percutaneous cardiopulmonary support for the treatment of circulatory collapse after cardiac surgery.

To overcome the hemorrhagic complications that may occur during extracorporeal circulatory support for post cardiotomy shock patients, a heparinized circuit was introduced into the percutaneous cardiopulmonary support system and decreased systemically administered heparin during bypass. Heparin coated percutaneous cardiopulmonary support with low dose systemic heparinization was instituted in 13 patients (6 men and 7 women, mean age 62.2 +/- 8.5 years) who experienced circulatory collapse after cardiac surgery. Of the 13 patients, 9 could not be weaned from cardiopulmonary bypass and 4 had circulatory collapse in the operating room or in the intensive care unit. The duration of support ranged from 1 to 66 hr (mean 27.4 +/- 26.7), and the flow rate ranged from 1 to 3 L/min (2.2 +/- 0.5). An activated coagulation time of about 150 sec was maintained with or without minimal systematically administered heparin. Of the patients cannulated, 77% (10 of 13) were successfully weaned from percutaneous cardiopulmonary support and 39% (5 to 13) were long-term survivors. The causes of death were sepsis in three, progressive heart failure in three, lower leg ischemia in one, and vital infection in one. From the results of clinical or post mortem examinations, there was no massive bleeding or evidence of thromboembolism in the major organs. From observations made within 12 hr of initiation of percutaneous cardiopulmonary support, there was no significant decrease in the number of platelets, but platelet count had significantly decreased 24 hr after initiation.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

PubMedDate not supplied

Proinflammatory cytokine levels in patients undergoing cardiopulmonary bypass. Does lung reperfusion influence the release of cytokines?

Proinflammatory cytokines have been implicated in mediating tissue injury after cardiopulmonary bypass. Causative factors of inflammatory response after cardiopulmonary bypass include contact of the blood with the extracorporeal circuit and heart-lung reperfusion injury when discontinuing bypass. To evaluate proinflammatory cytokine release during cardiopulmonary bypass, plasma levels of interleukin-6, 8, and monocyte chemoattractant factor were measured in the radial artery (for systemic blood) and left atrium before and after cardiopulmonary bypass. A total of 13 patients were studied, with no deaths or complications. In both radial artery and left atrium, interleukin-6, 8, and monocyte chemoattractant factor rose significantly after cardiopulmonary bypass (p < 0.05 versus before cardiopulmonary bypass). These changes may have been caused by removal of the aortic cross clamp and recommencement of artificial ventilation, which result in reperfusion of the pulmonary capillary beds. There were no differences in cytokine levels after cardiopulmonary bypass in the radial artery and left atrium. This result suggested that lung reperfusion injury after cardiopulmonary bypass may not be the major causative factor of the release of proinflammatory cytokines.

Aged↗