Partial monosomy 18p and 21q due to a paternal reciprocal translocation leading to holoprosencephaly

Abstract Here we report a patient with holoprosencephaly (HPE) associated with 45, XY,der(18)t(18;21)(p11.2;q21.3),-21 derived from a paternal balanced reciprocal translocation. Array comparative genomic hybridization analysis revealed 18p11.32-p11.21 and 21q11.2-q21.3 deletions. So far, nine cases...

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Main Authors: Hiroko Wakabayashi, Ayumi Matsumoto, Sakiko Komori, Masahide Goto, Toshihiro Tajima, Aiko Sasaki, Takayoshi Matsumura, Takanori Yamagata
Format: Article
Language:English
Published: Nature Publishing Group 2025-05-01
Series:Human Genome Variation
Online Access:https://doi.org/10.1038/s41439-025-00314-2
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author Hiroko Wakabayashi
Ayumi Matsumoto
Sakiko Komori
Masahide Goto
Toshihiro Tajima
Aiko Sasaki
Takayoshi Matsumura
Takanori Yamagata
author_facet Hiroko Wakabayashi
Ayumi Matsumoto
Sakiko Komori
Masahide Goto
Toshihiro Tajima
Aiko Sasaki
Takayoshi Matsumura
Takanori Yamagata
author_sort Hiroko Wakabayashi
collection DOAJ
description Abstract Here we report a patient with holoprosencephaly (HPE) associated with 45, XY,der(18)t(18;21)(p11.2;q21.3),-21 derived from a paternal balanced reciprocal translocation. Array comparative genomic hybridization analysis revealed 18p11.32-p11.21 and 21q11.2-q21.3 deletions. So far, nine cases of monosomy 18p with an unbalanced translocation (18;21) have been reported, four of which presented with HPE. Our case provides a detailed long-term clinical course and helps us to better understand these rare genetic events.
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institution DOAJ
issn 2054-345X
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publishDate 2025-05-01
publisher Nature Publishing Group
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series Human Genome Variation
spelling doaj-art-364033ffabe44a289d4d7407ae13930b2025-08-20T03:22:01ZengNature Publishing GroupHuman Genome Variation2054-345X2025-05-011211410.1038/s41439-025-00314-2Partial monosomy 18p and 21q due to a paternal reciprocal translocation leading to holoprosencephalyHiroko Wakabayashi0Ayumi Matsumoto1Sakiko Komori2Masahide Goto3Toshihiro Tajima4Aiko Sasaki5Takayoshi Matsumura6Takanori Yamagata7Division of Clinical Trial, Clinical Investigation Center, Jichi Medical University HospitalDivision of Cardiovascular and Genetics Research, Center for Molecular Medicine, Jichi Medical UniversityDepartment of Pediatrics, Jichi Medical UniversityDepartment of Pediatrics, Jichi Medical UniversityDepartment of Pediatrics, Jichi Medical UniversityDivision of Obstetrics, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, Center for Medical Genetics, National Center for Child Health and DevelopmentDivision of Cardiovascular and Genetics Research, Center for Molecular Medicine, Jichi Medical UniversityDepartment of Pediatrics, Jichi Medical UniversityAbstract Here we report a patient with holoprosencephaly (HPE) associated with 45, XY,der(18)t(18;21)(p11.2;q21.3),-21 derived from a paternal balanced reciprocal translocation. Array comparative genomic hybridization analysis revealed 18p11.32-p11.21 and 21q11.2-q21.3 deletions. So far, nine cases of monosomy 18p with an unbalanced translocation (18;21) have been reported, four of which presented with HPE. Our case provides a detailed long-term clinical course and helps us to better understand these rare genetic events.https://doi.org/10.1038/s41439-025-00314-2
spellingShingle Hiroko Wakabayashi
Ayumi Matsumoto
Sakiko Komori
Masahide Goto
Toshihiro Tajima
Aiko Sasaki
Takayoshi Matsumura
Takanori Yamagata
Partial monosomy 18p and 21q due to a paternal reciprocal translocation leading to holoprosencephaly
Human Genome Variation
title Partial monosomy 18p and 21q due to a paternal reciprocal translocation leading to holoprosencephaly
title_full Partial monosomy 18p and 21q due to a paternal reciprocal translocation leading to holoprosencephaly
title_fullStr Partial monosomy 18p and 21q due to a paternal reciprocal translocation leading to holoprosencephaly
title_full_unstemmed Partial monosomy 18p and 21q due to a paternal reciprocal translocation leading to holoprosencephaly
title_short Partial monosomy 18p and 21q due to a paternal reciprocal translocation leading to holoprosencephaly
title_sort partial monosomy 18p and 21q due to a paternal reciprocal translocation leading to holoprosencephaly
url https://doi.org/10.1038/s41439-025-00314-2
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