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TGFß level in healthy and children with Marfan syndrome-effective reduction under sartan therapy.
Stark, Veronika C; Olfe, Jakob; Diaz-Gil, Daniel; von Kodolitsch, Yskert; Kozlik-Feldmann, Rainer; Reincke, Johannes; Stark, Maria; Wiegand, Peter; Zeller, Tanja; Mir, Thomas S.
Afiliación
  • Stark VC; Clinic for Pediatric Heart Medicine and Adults with Congenital Heart Disease, University Heart and Vascular Center, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
  • Olfe J; Clinic for Pediatric Heart Medicine and Adults with Congenital Heart Disease, University Heart and Vascular Center, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
  • Diaz-Gil D; Clinic for Pediatric Heart Medicine and Adults with Congenital Heart Disease, University Heart and Vascular Center, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
  • von Kodolitsch Y; Department of Cardiac Surgery & Department of Pediatric, Boston Children's Hospital/ Harvard Medical School, Boston, MA, United States.
  • Kozlik-Feldmann R; Department of Cardiology, University Heart and Vascular Center, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
  • Reincke J; Clinic for Pediatric Heart Medicine and Adults with Congenital Heart Disease, University Heart and Vascular Center, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
  • Stark M; Clinic for Pediatric Heart Medicine and Adults with Congenital Heart Disease, University Heart and Vascular Center, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
  • Wiegand P; Institute of Medical Biometry and Epidemiology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
  • Zeller T; Clinic for Pediatric Heart Medicine and Adults with Congenital Heart Disease, University Heart and Vascular Center, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
  • Mir TS; Department of General and Interventional Cardiology, University Center of Cardiovascular Science, University Heart and Vascular Center, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Front Pediatr ; 12: 1276215, 2024.
Article en En | MEDLINE | ID: mdl-38379908
ABSTRACT

Introduction:

Transforming growth factor ß (TGFß) metabolism plays an important role in the pathogenesis of Marfan syndrome (MFS). Accordingly, drug therapy uses TGFß receptor blockade to slow down the cardiovascular manifestations, above all aortic root dilatation. Angiotensin II type 1 receptor blockers (ARBs) have been shown to reduce TGFß levels in adults. Data on childhood are lacking and are now being investigated in the TiGer For Kids study presented here.

Methods:

We examined 125 children without chronic disease and 31 pediatric Marfan patients with a proven FBN1 variant with regard to TGFß levels. In addition, we measured TGFß levels during the initiation of ARB therapy in pediatric Marfan patients.

Results:

In children without chronic disease, TGFß levels were found to decrease from childhood to adolescence (p < 0.0125). We could not measure a relevantly increased TGFß level in pediatric Marfan patients. However, we showed a significant suppression of the TGFß level after treatment with ARBs (p < 0.0125) and a renewed increase shortly before the next dose.

Discussion:

The TGFß level in childhood changes in an age-dependent manner and decreases with age. The TGFß level drops significantly after taking ARBs. Based on our experience and data, a TGFß receptor blockade in childhood seems reasonable. So far, TGFß level cannot be used as an MFS screening biomarker.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Front Pediatr Año: 2024 Tipo del documento: Article País de afiliación: Alemania Pais de publicación: Suiza
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: Front Pediatr Año: 2024 Tipo del documento: Article País de afiliación: Alemania Pais de publicación: Suiza