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dc.contributor.author | Poterico, Julio A. | |
dc.contributor.author | Vásquez, Flor | |
dc.contributor.author | Chávez-Pastor, Miguel | |
dc.contributor.author | Trubnykova, Milana | |
dc.contributor.author | Chavesta, Félix | |
dc.contributor.author | Chirinos, Jenny | |
dc.contributor.author | Salcedo, Nancy | |
dc.contributor.author | Mena, Rosmery | |
dc.contributor.author | Cubas, Sulema | |
dc.contributor.author | González, Rocío | |
dc.contributor.author | Alvariño, Rossana | |
dc.contributor.author | Abarca-Barriga, Hugo | |
dc.date.accessioned | 2019-01-25T16:59:36Z | |
dc.date.available | 2019-01-25T16:59:36Z | |
dc.date.issued | 2016 | |
dc.identifier.uri | https://hdl.handle.net/20.500.12866/4866 | |
dc.description.abstract | Chromosome 18 pericentric inversion carriers could have offspring with recombinant chromosomes, leading to patients with clinical variable manifestations. Patients with 18p-/18q+ rearrangements share some clinical characteristics, while other characteristics differ. Factors for such divergence include the length of the inverted segment, among others. Here, we describe a Peruvian child with dysmorphic features, intellectual disability persistent microscopic hematuria, aortic pseudocoarctation, and descending aorta arteritis, among others. Karyotype analysis of family members determined the mother as the carrier of a pericentric inversion: 18[inv(18)(p11.2q21.3)]. This child carries a recombinant chromosome 18, with chromosomal microarray analysis detecting two genomic imbalances in patient's chromosome 18: one duplicated region and one deleted segment in the large and the short arms, respectively. Persistent microscopic hematuria has not been reported among 18p-/18q+ phenotypes. Our patient elucidates that other factors play significant and yet unknown roles for not fulfilling the proposed genotype-phenotype correlation associated with hemizygosity in this type of recombinant chromosome 18 or presenting these features as the patient ages. | en_US |
dc.language.iso | eng | |
dc.publisher | Thieme Gruppe | |
dc.relation.ispartofseries | Journal of Pediatric Genetics | |
dc.rights | info:eu-repo/semantics/restrictedAccess | |
dc.rights.uri | https://creativecommons.org/licenses/by-nc-nd/4.0/deed.es | |
dc.subject | chromosome 18 pericentric inversion | en_US |
dc.subject | genotype-phenotype correlation | en_US |
dc.subject | hematuria | en_US |
dc.subject | microarray analysis | en_US |
dc.subject | recombinant chromosome 18 | en_US |
dc.title | A Peruvian Child with 18p-/18q+ Syndrome and Persistent Microscopic Hematuria | en_US |
dc.type | info:eu-repo/semantics/article | |
dc.identifier.doi | https://doi.org/10.1055/s-0037-1604099 | |
dc.subject.ocde | https://purl.org/pe-repo/ocde/ford#3.02.03 | |
dc.subject.ocde | https://purl.org/pe-repo/ocde/ford#1.06.07 | |
dc.relation.issn | 2146-460X |
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