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 |
|