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DOI 10.3892/mmr.2013.1788

Медицинская библиотека · Molecular medicine reports · 2013

Prenatal detection of TAR syndrome in a fetus with compound inheritance of an RBM8A SNP and a 334‑kb deletion: a case report.

Papoulidis Ioannis, Oikonomidou Eirini, Orru Sandro, Siomou Elisavet, Kontodiou Maria, Eleftheriades Makarios, Bacoulas Vasilios, Cigudosa Juan C, Suela Javier, Thomaidis Loretta, Manolakos Emmanouil

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Аннотация

Thrombocytopenia‑absent radius syndrome (TAR) is a rare genetic disorder that is characterized by the absence of the radius bone in each forearm and a markedly reduced platelet count that results in life‑threatening bleeding episodes (thrombocytopenia). Tar syndrome has been associated with a deletion of a segment of 1q21.1 cytoband. The 1q21.1 deletion syndrome phenotype includes Tar and other features such as mental retardation, autism and microcephaly. This study describes a case of a prenatally diagnosed fetus with compound inheritance of a small (334 kb) deletion, as detected by array‑comparative genomic hybridization, and a 5' untranslated region (UTR) low‑frequency allele (rs139428292) in gene RBM8A as detected by Sanger sequencing. The study describes the first case of prenatal analysis of TAR syndrome in a fetus with compound inheritance of a 334‑kb deletion in the 1q21.1 region and a low‑frequency 5' UTR single nucleotide polymorphism, and provides confirmation of the causal nature of the RBM8A gene in the diagnosis of TAR syndrome.

Для цитирования: Papoulidis Ioannis, Oikonomidou Eirini, Orru Sandro, Siomou Elisavet, Kontodiou Maria, Eleftheriades Makarios, Bacoulas Vasilios, Cigudosa Juan C, Suela Javier, Thomaidis Loretta, Manolakos Emmanouil. Prenatal detection of TAR syndrome in a fetus with compound inheritance of an RBM8A SNP and a 334‑kb deletion: a case report.. Molecular medicine reports. 2013. DOI: 10.3892/mmr.2013.1788.

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