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At least 973 records · Page 54Linked to original sources
PubMed1994

Antibodies against beta adrenoceptors in mothers of children with congenital heart block.

To determine whether antibodies against beta adrenergic activity interact with neonatal cardiac cell receptors and alter their physiological behaviour. An "in vitro" experimental model measuring the frequency of contraction, the production of cAMP and binding assay on neonatal and adult rat atria was employed. Sera and IgG fraction from mothers of infants with congenital heart block (CHB) interact with neonatal rat atria increasing the frequency of contraction and cAMP production. These effects were abolished by propranolol, pointing to a beta adrenergic participation. IgG also competed with 3H-CGP to beta adrenergic receptors on neonatal cardiac membranes. Neither the contractile nor the cAMP effects or binding assay were obtained using adult instead of neonatal rat atria. Reactivity against cardiac neurotransmitter receptors may be another serum factor(s) to be considered in the pathophysiology of the development of CHB.

Animals↗
PubMed1999

Bacillus popilliae endocarditis with prolonged complete heart block.

Bacillus popilliae, a fastidious, aerobic, gram-positive, spore-forming bacillus, has never been reported as a pathogen in human infectious diseases. We report the first case of a human infected by the pathogen B. popilliae, which presented as endocarditis involving the bicuspid aortic valve and complicated with prolonged (> 30 days; to our knowledge, the longest in the literature) complete heart block. Although surgery may be warranted by previous reports, the patient was successfully managed by medical treatment instead, because of the absence of evidence from various approaches that support the existence of perivalvular extension of infection.

Bacillus↗
PubMed2005

A gene locus for progressive familial heart block type II (PFHBII) maps to chromosome 1q32.2-q32.3.

Cardiac conduction defects that are associated with dilated cardiomyopathy (DCM) are generally considered to be sporadic clinical entities, although familial forms of disorders with these clinical features have been identified in a number of families in different countries. An autosomal dominant cardiac disorder characterised by conduction abnormalities and DCM, termed progressive familial heart block type II (PFHBII) (OMIM 140400), has been described in a South African Caucasian family of Northern European descent. Known candidate loci for isolated conduction disorders, isolated DCM and conduction disorders complicated by DCM were excluded from disease causation in this family by linkage analysis, with the exception of the DCM-associated (CMD1D) locus on chromosome 1q32, where a maximum multipoint lod score of 3.7 in the interval between D1S3753 and D1S414, was generated. This region encompassed the troponin T gene (TNNT2), however, genetic fine mapping and haplotype analysis excluded TNNT2 as cause of PFHBII and placed the disease-causative gene within a 3.9 cM (2.85 Mb) interval, flanked by D1S70 and D1S505. Analysis of KCNH1, KIAA0205, LAMB3 and PPP2R5A, which map within the critical interval, indicated that the PFHBII-causative mutation does not lie within the coding regions or splice junctions of these plausible candidate genes. The data indicate the existence of a novel locus involved in the pathogenesis of cardiac conduction abnormalities and DCM.

Chromosome Mapping↗
PubMed1995

Mobitz type I heart block after pokeweed ingestion.

The leaves of Phytolacca americana (pokeweed), when ingested, typically produce a self-limited but severe gastroenteritis characterized by intense vomiting and frothy diarrhea. Although cardiac effects have been reported in previous cases of pokeweed ingestion, no cardiac toxin has ever been identified in pokeweed. We report the case of a family who ingested raw and/or cooked pokeweed leaves, and 1 member who developed a Mobitz type I heart block associated with vomiting which resolved after i.v. promethazine. This suggests the possibility that some cardiac effects of pokeweed are secondary to the increased vagal tone seen with severe gastrointestinal colic.

Adult↗
PubMedDate not supplied

Complete heart block in a patient with Wegener's granulomatosis in remission--a case report.

Wegener's granulomatosis is a systemic inflammatory disorder of unknown cause that usually affects the upper and lower respiratory tracts as well as the kidney. Cardiac involvement is rare, although electrocardiographic abnormalities, coronary artery vasculitis, cardiac arrhythmias, and myocardial infarction have been reported in the literature. A 27-year-old female patient with Wegener's granulomatosis in remission is described in whom complete heart block developed in the 13th month of treatment with cyclophosphamide. A temporary pacing was applied and pulse methylprednisolone and cyclophosphamide were commenced. On the ninth day of treatment, normal sinus rhythm was achieved. In conclusion, cardiac rhythm abnormalities should always be kept in mind both in the diagnosis and follow-up of Wegener's granulomatosis.

Adult↗
PubMed2000

Sox6 is a candidate gene for p100H myopathy, heart block, and sudden neonatal death.

The mouse p locus encodes a gene that functions in normal pigmentation. We have characterized a radiation-induced mutant allele of the mouse p locus that is associated with a failure-to-thrive syndrome, in addition to diminished pigmentation. Mice homozygous for this mutant allele, p(100H), show delayed growth and die within 2 wk after birth. We have discovered that the mutant mice develop progressive atrioventricular heart block and significant ultrastructural changes in both cardiac and skeletal muscle cells. These observations are common characteristics described in human myopathies. The karyotype of p(100H) chromosomes indicated that the mutation is associated with a chromosome 7 inversion. We demonstrate here that the p(100H) chromosomal inversion disrupts both the p gene and the Sox6 gene. Normal Sox6 gene expression has been examined by Northern blot analysis and was found most abundantly expressed in skeletal muscle in adult mouse tissues, suggesting an involvement of Sox6 in muscle maintenance. The p(100H) mutant is thus a useful animal model in the elucidation of myopathies at the molecular level.

Amino Acid Sequence↗