| Literature DB >> 32302043 |
Eduardo Perrone1, Vânia D'Almeida2, Nara Lygia de Macena Sobreira3, Claudia Berlim de Mello4, Allan Chiaratti de Oliveira5, Stênio Burlin6, Maria de Fátima de Faria Soares6, Mirlene Cecília Soares Pinho Cernach7, Ana Beatriz Alvarez Perez1.
Abstract
Gomez-López-Hernández syndrome (GLHS) is characterized by rhombencephalosynapsis (RES), alopecia, trigeminal anesthesia and a distinctive phenotype, including brachyturricephaly. It has been suggested that GLHS should be considered as part of the spectrum of RES-associated conditions that include alopecia, trigeminal anesthesia, and craniofacial anomalies, rather than a distinct entity. To the best of our knowledge, 57 patients with GLHS have been described. Despite its first description in 1979, the etiology of this syndrome remains unknown. Here, we describe, to our knowledge, the first case of a patient with GLHS who was molecularly evaluated and had been prenatally exposed to misoprostol. We also reviewed the clinical and morphological features of the patients described to date to better delineate the phenotype and focus on any evidence for adverse pregnancy outcomes or exposure, including teratogens.Entities:
Keywords: GLH syndrome; disruption; misoprostol; rhombencephalosynapsis; trigeminal nerve
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Year: 2020 PMID: 32302043 PMCID: PMC8988015 DOI: 10.1002/ajmg.a.61594
Source DB: PubMed Journal: Am J Med Genet A ISSN: 1552-4825 Impact factor: 2.802