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The therapeutic effect of liver transplantation in 14 children with homozygous familial hypercholesterolemia: a prospective cohort: Liver transplant for familial hypercholesterolemia.
Lin, Dongni; Lu, Yefeng; Qiu, Bijun; Feng, Mingxuan; Luo, Yi; Xue, Feng; Zhou, Tao; Zhu, Jianjun; Zhang, Jianjun; Wang, Lvya; Xia, Qiang; Wan, Ping.
Afiliación
  • Lin D; Department of Liver Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, 1630 Dongfang Road, Shanghai, 200127, China.
  • Lu Y; Department of Liver Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, 1630 Dongfang Road, Shanghai, 200127, China.
  • Qiu B; Department of Liver Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, 1630 Dongfang Road, Shanghai, 200127, China.
  • Feng M; Department of Liver Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, 1630 Dongfang Road, Shanghai, 200127, China.
  • Luo Y; Department of Liver Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, 1630 Dongfang Road, Shanghai, 200127, China.
  • Xue F; Department of Liver Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, 1630 Dongfang Road, Shanghai, 200127, China.
  • Zhou T; Department of Liver Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, 1630 Dongfang Road, Shanghai, 200127, China.
  • Zhu J; Department of Liver Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, 1630 Dongfang Road, Shanghai, 200127, China.
  • Zhang J; Department of Liver Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, 1630 Dongfang Road, Shanghai, 200127, China.
  • Wang L; Beijing Institute of Heart Lung and Blood Vessel Diseases, 2 Anzhen Road, Chaoyang District, Beijing 100029, China; Department of Cardiology, Beijing Anzhen Hospital Affiliated with Capital Medical University, 2 Anzhen Road, Chaoyang District, Beijing 100029, China. Electronic address: wangluya@126.
  • Xia Q; Department of Liver Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, 1630 Dongfang Road, Shanghai, 200127, China; Shanghai Institute of Organ Transplantation, 1630 Dongfang Road, Shanghai, 200127, China; Shanghai Research Center of Organ Transplantation & Immune Engine
  • Wan P; Department of Liver Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, 1630 Dongfang Road, Shanghai, 200127, China. Electronic address: gufeng182@126.com.
J Clin Lipidol ; 2024 Sep 05.
Article en En | MEDLINE | ID: mdl-39294020
ABSTRACT

OBJECTIVES:

Homozygous familial hypercholesterolemia (HoFH) is characterized by elevated low-density lipoprotein cholesterol (LDL-C) and early-onset cardiovascular disease. To assess the therapeutic effects of liver transplantation (LT) on HoFH patients, we observed and analyzed the outcomes of HoFH children after LT. STUDY

DESIGN:

This prospective cohort study included all LT candidates under 18 years old diagnosed with HoFH at Ren Ji Hospital between November 2017 and July 2021. The patients were followed until October 2023. They were treated according to the standard protocol at our center. We collected data on changes in lipid profiles, clinical manifestations, and cardiovascular complications at different time points, and recorded postoperative recipient and graft survival.

RESULTS:

Fourteen HoFH patients with a median age of 7 (2-12) years were included. Preoperatively, xanthomas and arcus corneas occurred in 14 and 3 patients, respectively, with 10 patients showing mild cardiovascular disease. All patients underwent LT. Recipient and graft survival rates were 100 % over a median follow-up duration of 35 (27-71) months. Median LDL-C levels dropped from 11.83 (7.99-26.14) mmol/L preoperatively to 2.3 (1.49-3.39) mmol/L postoperative at the last measurement. Thirteen patients discontinued lipid-lowering treatment after LT, while only one patient resumed statins 6 months post-operation. Xanthomas and arcus corneas significantly improved. Cardiovascular complications regressed in five patients, with no progression observed in the others.

CONCLUSIONS:

LT is a safe and effective treatment for severe HoFH patients beyond lipid-lowering control. Early LT improves prognosis and quality of life while minimizing the risk of cardiovascular complications.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: J Clin Lipidol Asunto de la revista: BIOQUIMICA / METABOLISMO Año: 2024 Tipo del documento: Article País de afiliación: China Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: J Clin Lipidol Asunto de la revista: BIOQUIMICA / METABOLISMO Año: 2024 Tipo del documento: Article País de afiliación: China Pais de publicación: Estados Unidos