Evidence mapPaperPMID 42415556Full record

ArticleAnnals of pediatric endocrinology & metabolism2026

Long-term outcomes in 45,X/46,XY mosaicism: a 30-year retrospective study in Hong Kong.

Sarah Wing Yiu Poon, Sharon Wing Yan To, Lisa Lai Ping Siu, Shirley Sze Wing Cheng, Stephanie K L Ho, Gloria Shir Wey Pang, Pauline Po Lam So, Joanna Yuet Ling Tung, Ho-Ming Luk

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Article in Annals of pediatric endocrinology & metabolism, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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1citing papers in PubMed
field-weighted citation impact
1 · What the graph read from it

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3 · Its place in the literature

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1 citing paper in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

9 authors.

Sarah Wing Yiu Poon *Department of Paediatrics and Adolescent Medicine, Hong Kong Children's Hospital, Hong Kong.
Sharon Wing Yan To *Department of Clinical Genetics, Hong Kong Children's Hospital, Hong Kong.
Lisa Lai Ping SiuDepartment of Clinical Genetics, Hong Kong Children's Hospital, Hong Kong.
Shirley Sze Wing ChengDepartment of Clinical Genetics, Hong Kong Children's Hospital, Hong Kong.
Stephanie K L HoDepartment of Clinical Genetics, Hong Kong Children's Hospital, Hong Kong.
Gloria Shir Wey PangDepartment of Paediatrics and Adolescent Medicine, Hong Kong Children's Hospital, Hong Kong.
Pauline Po Lam SoDepartment of Obstetrics and Gynaecology, Tuen Mun Hospital, Hong Kong.
Joanna Yuet Ling TungDepartment of Paediatrics and Adolescent Medicine, Hong Kong Children's Hospital, Hong Kong.
Ho-Ming LukDepartment of Clinical Genetics, Hong Kong Children's Hospital, Hong Kong. lukhm@ha.org.hk.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

purpose45,X/46,XY mosaicism is a rare subset of sex chromosome abnormalities within the spectrum of differences of sex development. This study aimed to evaluate the long-term outcomes in a group of individuals with 45,X/46,XY mosaicism over a 30-year period.

methodsA retrospective review was performed including 68 patients diagnosed from January 1990 to December 2023 at a tertiary unit. Cytogenetic analysis, patient demographics and various health outcomes were examined.

resultsThirty-five patients were raised as females and 33 were raised as males. Fifteen patients were found to have 45,X/46,XY mosaicism prenatally. The prevalence of gonadal tumor was 17.6% among phenotypic females and 38.5% in males with abnormal genitalia, whereas no tumors were detected in males with normal genitalia. The mean external genitalia score was significantly lower in males with gonadal tumors compared to those without (6.6 vs 10.4, P=0.002). For females, while most gonadal tumors were identified in those who had gonadectomy at pubertal age (5 of 6, 83.3%), gonadal tumors were also detected in very young children (1.3 years old). There was no significant improvement in height in those treated with growth hormone therapy and final adult height remained suboptimal (overall final adult height z-score -2.6±1.2). Adherence to surveillance for associated systemic comorbidities was inconsistent.

conclusionGonadal tumor risk is higher in less masculinized males, while females may develop tumors across a wide age range. The long-term efficacy of growth hormone therapy in these patients remains unclear. Our findings emphasized the need for individualized surveillance in this population.

Indexed as

45Differences of sex developmentExternal genitalia scoreGonadal tumorGrowth hormone therapyX/46XY mosaicism

Identifiers

PMID42415556
PMCPMC13342892

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