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DOI 10.1016/j.jfma.2021.12.022

Медицинская библиотека · Journal of the Formosan Medical Association = Taiwan yi zhi · 2022

Short stature leads to a diagnosis of Jansen-de Vries syndrome in two unrelated Taiwanese girls: A case report and literature review.

Tsai Meng-Ju Melody, Lee Ni-Chung, Chien Yin-Hsiu, Hwu Wuh-Liang, Tung Yi-Ching

Клинический случайОбзорСтатьяПеревод в очереди

Аннотация

Short stature and intellectual disability are two of the major components of many dysmorphic syndromes. Jansen-de Vries syndrome (JDVS) is a rare syndromic disorder that was discovered recently using next-generation sequencing. It is characterized by hypotonia, developmental delay, a dysmorphic face, short stature, and high pain threshold and is caused by the variants of the protein phosphatase magnesium-dependent 1D (PPM1D) gene. Here, we report the first two cases of PPM1D mutations in Taiwan; both had de novo variants in exon 6. Both presented with short stature, developmental delay, and dysmorphic faces. In addition to the characteristics listed above, syndactyly was noted in one. Genetic studies should be considered when approaching a patient with growth retardation, intellectual disability, and other major or minor dysmorphisms.

Для цитирования: Tsai Meng-Ju Melody, Lee Ni-Chung, Chien Yin-Hsiu, Hwu Wuh-Liang, Tung Yi-Ching. Short stature leads to a diagnosis of Jansen-de Vries syndrome in two unrelated Taiwanese girls: A case report and literature review.. Journal of the Formosan Medical Association = Taiwan yi zhi. 2022. DOI: 10.1016/j.jfma.2021.12.022.

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