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PedAM

Pediatric Disease Annotations & Medicines




Disease dilated cardiomyopathy
Symptom C0264886|conduction defects
Sentences 9
PubMedID- 25886484 These disorders are called laminopathies, and they include autosomal dominant emery-dreifuss muscular dystrophy (emdm), autosomal repressive emdm, limb-girdle muscular dystrophy 1b (lgmd1b), dilated cardiomyopathy with conduction defects (cmd1a), and cmt type 2b1 (cmt2b1).
PubMedID- 23360689 dilated cardiomyopathy with conduction defects and arrhythmias requiring pacemaker placement affect both groups of patients.
PubMedID- 23853505 He presented one of the families showing familial dilated cardiomyopathy with conduction defects due to mutation in lamin a/c gene (28).
PubMedID- 22006699 dilated cardiomyopathy with conduction defects in a patient with partial merosin deficiency due to mutations in the laminin-alpha2-chain gene: a chance association or a novel phenotype.
PubMedID- 22106718 The meeting focused on the following main areas: 1) the biology of the nucleus and the nuclear envelope and the biological pathways affected in laminopathies; 2) the epidemiology of muscle laminopathies, lipodystrophies and progeroid syndromes; 3) the emery- dreifuss muscular dystrophy (edmd) from a clinical point of view; 4) dilated cardiomyopathy with conduction defects (cmd-cd) from diagnosis to therapy; 5) familial partial lipodystrophy of the dunningan type (fpld): diagnosis and therapy; 6) progeroid laminopathies, including mandibuloacral displasia (mada) and hutchinson-gilford progeria (hgps): diagnosis and therapeutic trials.
PubMedID- 23815988 Emery-dreifuss muscular dystrophy (edmd) is classically characterized clinically by a triad of: (1) slowly progressive muscle weakness and wasting in a scapulo-humeroperoneal distribution; (2) early contractures of the elbows, ankles, and posterior neck; and (3) dilated cardiomyopathy with conduction defects .
PubMedID- 22905185 Lmna(-/-) mice display multiple tissue defects and die by 6-8 weeks of age reportedly from dilated cardiomyopathy with associated conduction defects.
PubMedID- 22691392 They include emery-dreifuss muscular dystrophy, limb-girdle muscular dystrophy type 1b, dilated cardiomyopathy with conduction defects, dunnigan type familial partial lipodystrophy, mandibuloacral dysplasia .
PubMedID- 23853504 Mutations on the lamin a/c gene cause several defined clinical conditions, commonly termed as laminopathies, consisting in a heterogeneous group of diseases which include: the autosomal dominant and recessive forms of emery dreifuss muscular dystrophy (edmd2 and edmd3); the limb girdle muscular dystrophy 1b (lgmd1b); the congenital muscular dystrophy-l (cmdl); the dilated cardiomyopathy with conduction defects (dcm1a); the heart hand syndrome of slovenian type (hhs); a recessive form of sensory-motor peripheral neuropathy (cmt2b); the familial partial lipodystrophy of the dunnigan type (fpld2); the hutchinson- gilford progeria syndrome (hgps); the atypical form of werner syndrome (ws); the restrictive dermopathy (rd) and the mandibuloacral dysplasia (mada) (13).

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