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Langerhans Cell Histiocytosis: A 24-Year Retrospective Review in the Sultanate of Oman.

作者信息

Al Jadeedi Sondus, Al Alawi Khalifa, Al Hajri Abdallah, Al Azri Ahmed, Al Shaqsi Sultan

机构信息

Department of Plastic, Reconstructive and Craniofacial Surgery, Khoula Hospital, Muscat, Sultanate of Oman.

Department of Plastic Surgery and Reconstructive Surgery, Hamad Medical Corporation (HMC), Doha, Qatar.

出版信息

J Craniofac Surg. 2025 Aug 12. doi: 10.1097/SCS.0000000000011755.

DOI:10.1097/SCS.0000000000011755
PMID:40795127
Abstract

BACKGROUND

Langerhans cell histiocytosis (LCH) is a relatively rare and ill-defined multisystem disorder. The epidemiology of this disease in the Sultanate of Oman has never been described before. Directorate General of Khoula Hospital (DGXH) is constituted of Khoula Hospital, Al-Nahdha Hospital, and Wattaya Obstetrics and Gynecology Complex, which is one of the main hospitals to manage cases of LCH in Sultanate. This study presents an analysis of the LCH data collected over 24 years in Khoula and Al-Nahdha Hospitals.

METHOD

This is a retrospective review of patients diagnosed with any form of LCH between 2000 and 2024 in Khoula and Al-Nahdha Hospitals. Data were extracted through a comprehensive review of patient medical records. Variables include basic demographics and clinical data points.

RESULTS

A total of 43 patients diagnosed with Langerhans cell histiocytosis were included in this study. An upward trend in reported cases was observed over the 5-year period, with a markedly lower incidence among individuals over 18 years of age. The cohort was predominantly male (53.5%), and females tended to present at a younger age. Single-system LCH was the most common disease classification, accounting for 83.7% of cases. Skeletal involvement was the predominant manifestation (88.8%), followed by cutaneous involvement. Within the craniomaxillofacial region, the parietal bone was the most frequently affected site. The 5-year overall survival rate in this cohort was 100%.

CONCLUSION

This study presents the first investigation of the epidemiological characteristics of LCH in 2 main referral hospitals in Sultanate of Oman. The establishment of a registry system for rare diseases is crucial for improving diagnostic accuracy and supporting future epidemiological research and informed policymaking.

摘要

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