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Heart Abnormalities (heart + abnormality)
Selected AbstractsMan1, an inner nuclear membrane protein, regulates left,right axis formation by controlling nodal signaling in a node-independent mannerDEVELOPMENTAL DYNAMICS, Issue 12 2008Akihiko Ishimura Abstract Man1, an inner nuclear membrane protein, regulates transforming growth factor , signaling by interacting with receptor-associated Smads. In Man1 -deficient (Man1,/,) embryos, vascular remodeling is perturbed by misregulation of Smad activity. Here, we show that Man1,/, embryos exhibit abnormal heart morphogenesis including the looping abnormality. We searched for the molecular basis underlying the heart abnormalities and found that the left side-specific genes responsible for left,right (LR) asymmetry, Nodal, Lefty2, and Pitx2, were expressed bilaterally in the lateral plate mesoderm and that their expression was enhanced significantly in mutants. Notably, Lefty1, a marker for the midline barrier, was maintained in Man1,/, mutants. Crossing Man1,/+ with Nodal hypomorphs (Nodalneo/+), in which Nodal signaling in the node is disrupted, to generate double homozygous embryos (Man1,/,; Nodalneo/neo) revealed that the bilateral Nodal was retained in Man1,/,; Nodalneo/neo embryos. These results suggest that Man1 regulates LR asymmetry by controlling Nodal signaling in a node-independent manner. Developmental Dynamics 237:3565,3576, 2008. © 2008 Wiley-Liss, Inc. [source] Use of morphine and 6-monoacetylmorphine in blood for the evaluation of possible risk factors for sudden death in 192 heroin usersADDICTION, Issue 4 2003Anna Fugelstad Abstract Aims, To detect risk factors for sudden death from heroin injection. Design, Evaluation of data from forensic investigations of all fatal cases of suspected heroin death in a metropolitan area. Only cases with detectable morphine and 6-monoacetylmorphine (6-MAM) in blood were included in order to select heroin intoxication cases. Setting, Stockholm, Sweden. Measurements, Autopsy investigation and toxicological analysis of blood and urine; and police reports. Findings, In two-thirds of the 192 cases, death occurred in public places, and mostly without any time delay. Blood concentrations of morphine ranged from 50 to 1200 ng/g, and of 6-MAM from 1 to 80 ng/g. Codeine was detected in 96% of the subjects. In the majority of cases the forensic investigation indicated polydrug use, the most common additional findings being alcohol and benzodiazepines. However, in one-quarter of the cases other drug combinations were found. Previous abstinence from heroin and use of alcohol were identified as risk factors. For 6-MAM there was also a correlation with the presence of THC and benzodiazepines. Despite a high frequency of heart abnormalities (e.g. myocarditis and focal myocardial fibrosis), these conditions did not correlate with morphine or 6-MAM blood concentrations. Conclusions, We confirm that alcohol intake and loss of tolerance are risk factors for death from heroin use, whereas no connection to heart pathology was observed. Further, prospective, studies should focus on other possible risk factors. [source] Normal embryonic development and cardiac morphogenesis in mice with Wnt1-Cre-mediated deletion of connexin43GENESIS: THE JOURNAL OF GENETICS AND DEVELOPMENT, Issue 6 2006M. Kretz Abstract Mice harboring a null mutation in the gap junction protein connexin43 (Cx43) die shortly after birth due to an obstruction of the right ventricular outflow tract of the heart. These hearts exhibit prominent pouches at the base of the pulmonary outlet, i.e., morphological abnormalities that were ascribed to Cx43-deficiency in neural crest cells. In order to examine the Cx43 expression pattern in neural crest cells and derived tissues and to test whether neural crest-specific deletion of Cx43 leads to the conotruncal defects seen in Cx43null mice, we ablated Cx43 using a Wnt1-Cre transgene. Deletion of Cx43 was complete and occurred in neural crest cells as well as in neural crest-derived tissues. Nevertheless, hearts of mice lacking Cx43 specifically in neural crest cells were indistinguishable from controls. Thus, the morphological heart abnormalities of Cx43 null mice are most likely not caused by lack of Cx43 in neural crest cells. genesis 44:269,276, 2006. © 2006 Wiley-Liss, Inc. [source] Results of living donor liver transplantation in five children with congenital cardiac malformations requiring cardiac surgeryPEDIATRIC TRANSPLANTATION, Issue 8 2006Jose Pablo Garbanzo Abstract:, In the pediatric population, the concomitant presentation of end-stage liver disease and congenital cardiac malformation occurs rarely. Determining the surgical priority in these cases is a challenge due to the presence of hemodynamic alterations that increase surgical risks. We examined five cases that received living-donor liver transplantation. In four patients that had congenital heart disease with a left to right shunt, two had cardiac surgery first, one had both heart and liver surgery simultaneously, and one underwent liver transplantation first. Both of the patients that received heart surgery before liver transplantation needed emergency liver transplantation because of post-operative liver failure. All five patients had a good outcome. Meticulous surgery, close monitoring, and adequate volume management, in addition to tailoring management decisions to the patient's specific condition, make it possible to correct both the liver and the heart abnormalities with satisfactory results. [source] Echocardiographic abnormalities in sickle cell diseaseAMERICAN JOURNAL OF HEMATOLOGY, Issue 3 2004Shahid Ahmed Abstract Echocardiographic abnormalities in patients with sickle cell disease (SCD) were determined, and pulmonary arterial systolic pressure (PASP) was estimated. Clinical data and echocardiograms of 38 adult hospitalized patients with SCD at two tertiary care hospitals were reviewed. Fisher's exact test was performed to determine correlation between pulmonary hypertension and various clinical variables. Pulmonary hypertension was the most common abnormality identified in 22 (58%) patients. The estimated mean PASP was 37.5 ± 10.9 mmHg. Older age and prior history of acute chest syndrome were significantly correlated with an increased prevalence of pulmonary hypertension (P < 0.05). Patients with hemoglobin levels <8 g/dL had PASP 43.2 ± 0.5 compared to a mean PASP of 33.3 ± 6.0 in patients with hemoglobin ,8 g/dL (P = 0.01). Eight (21%) patients had evidence of a hyperdynamic left ventricle. Left heart abnormalities included dilated atrium in 14 (37%), dilated ventricle in 5 (13%), ventricle hypertrophy in 5 (13%), and ventricle dysfunction in 3 (9%) patients. Right heart abnormalities included dilated atrium in 9 (24%), dilated ventricle in 6 (16%), and ventricle dysfunction in 3 (9%) patients. Despite an increased incidence of abnormal flow across the valves on Doppler analysis, no patient had structurally abnormal valves. A majority of patients with SCD had evidence of pulmonary hypertension, which correlated with older age and history of acute chest syndrome. Other structural and functional echocardiographic abnormalities were less common. Am. J. Hematol. 76:195,198, 2004. © 2004 Wiley-Liss, Inc. [source] | |||||||||||||