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Primer caso de displasia tanatofórica tipo 1 en la región central del Perú con variante patogénica en el gen FGFR3

Translated title of the contribution: A case of coexistence of the FGFR3 and KIAA0753 gene mutation in thanatophoric dysplasia type 1

Research output: Contribution to journalOriginal Articlepeer-review

Abstract

Introduction: Thanatophoric dysplasia type 1 is a form of lethal skeletal dysplasia, it is characterized by disproportions of the axial-appendicular skeleton in addition to short stature, macrocephaly, frontal prominence, narrow thorax, femoral bowing and micromelia. Case report: 38-week-old male neonate, born by elective cesarean section due to skeletal malformations and polyhydramnios. The clinical evolution showed progressive deterioration over time and despite the different oxygenatory and ventilatory support devices. Conclusions: For its study, obstetric ultrasound, physical examination and radiographic findings are important. However, the diagnosis must be confirmed through a genetic study in order to discover new variants or associations, as well as to make known their real case mix in a certain region.

Translated title of the contributionA case of coexistence of the FGFR3 and KIAA0753 gene mutation in thanatophoric dysplasia type 1
Original languageSpanish
JournalRevista del Cuerpo Medico Hospital Nacional Almanzor Aguinaga Asenjo
Volume16
Issue number2
DOIs
StateIndexed - 2023

Bibliographical note

Publisher Copyright:
© 2023 Medical Body of the Almanzor Aguinaga Asenjo National Hospital. All rights reserved.

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