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Is Liver Transplantation Alone an Effective Treatment for Homozygotic Familial Hypercholesterolemia in Children?
Yilmaz Dagli, Hatice; Artan, Reha; Civan, Dudu Duygu; Inan Aydemir, Nurel; Yilmaz, Aygen.
Afiliación
  • Yilmaz Dagli H; Department of Pediatric Gastroenterology, Akdeniz University, Antalya, Turkey.
  • Artan R; Department of Pediatric Gastroenterology, Akdeniz University, Antalya, Turkey.
  • Civan DD; Department of Pediatric Gastroenterology, Akdeniz University, Antalya, Turkey.
  • Inan Aydemir N; Department of Pediatric Gastroenterology, Akdeniz University, Antalya, Turkey.
  • Yilmaz A; Department of Pediatric Gastroenterology, Akdeniz University, Antalya, Turkey.
Pediatr Transplant ; 28(7): e14853, 2024 Nov.
Article en En | MEDLINE | ID: mdl-39282976
ABSTRACT

AIM:

To examine the long-term results and treatment effectiveness of liver transplantation (LT) in the treatment of homozygous familial hypercholesterolemia (HoFH) in children and adolescents.

METHOD:

Patients who underwent LT due to HoFH between 2007 and 2023 were included in the study. The patients' demographic data, clinical findings, preoperative and postoperative laboratory examinations, transplantation complications, and postoperative disease courses were evaluated.

RESULTS:

There were five boys with an average age of 6.2 (median 6, range 4-10) years in the study. The average total cholesterol level of the patients before transplantation was 923 (median 950, range 780-1002) mg/dL and the average LDL-cholesterol level was 864 (median 852, range 770-957) mg/dL. No patients died of transplant-related complications. After an average follow-up of 9.2 (median 9, range 1.5-16) years, the average total cholesterol level of the patients was 197 (median 164, range 137-359) mg/dL, and the average LDL-cholesterol level was 138 (median 92, range 85-313) mg/dL. Four (80%) patients developed atherosclerotic cardiovascular disease during follow-up, and two (40%) died of this cause.

CONCLUSION:

LT in the treatment of HoFH did not help our patients reach the target LDL-cholesterol level after transplantation and did not prevent the development of cardiovascular disease. Therefore, LT alone is not curative in the treatment of HoFH.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Trasplante de Hígado / Hiperlipoproteinemia Tipo II / LDL-Colesterol Límite: Adolescent / Child / Child, preschool / Female / Humans / Male Idioma: En Revista: Pediatr Transplant Asunto de la revista: PEDIATRIA / TRANSPLANTE Año: 2024 Tipo del documento: Article País de afiliación: Turquía Pais de publicación: Dinamarca
Texto completo
Añadir a Mi BVS
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XML
PubMed Links
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Trasplante de Hígado / Hiperlipoproteinemia Tipo II / LDL-Colesterol Límite: Adolescent / Child / Child, preschool / Female / Humans / Male Idioma: En Revista: Pediatr Transplant Asunto de la revista: PEDIATRIA / TRANSPLANTE Año: 2024 Tipo del documento: Article País de afiliación: Turquía Pais de publicación: Dinamarca