Early Vascular Aging in Children With Tuberous Sclerosis Complex

Objectives: Experimental data indicate that activating mutations in the mTOR (mammalian target of rapamycin) pathway may lead to abnormal arterial wall structure. Vascular anomalies like arterial stenoses are reported in pediatric patients with tuberous sclerosis complex (TSC). In addition, large re...

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Autores principales:	Piotr Skrzypczyk, Anna Maria Wabik, Michał Szyszka, Sergiusz Józwiak, Przemysław Bombiński, Aleksandra Jakimów-Kostrzewa, Michał Brzewski, Małgorzata Pańczyk-Tomaszewska
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	tuberous sclerosis complex early vascular aging central blood pressure arterial stiffness common carotid artery intima-media thickness arterial hypertension Pediatrics RJ1-570
Acceso en línea:	https://doaj.org/article/99fc50577e0e48aca91fd9e866ff1b17
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spelling	oai:doaj.org-article:99fc50577e0e48aca91fd9e866ff1b172021-12-01T13:57:07ZEarly Vascular Aging in Children With Tuberous Sclerosis Complex2296-236010.3389/fped.2021.767394https://doaj.org/article/99fc50577e0e48aca91fd9e866ff1b172021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fped.2021.767394/fullhttps://doaj.org/toc/2296-2360Objectives: Experimental data indicate that activating mutations in the mTOR (mammalian target of rapamycin) pathway may lead to abnormal arterial wall structure. Vascular anomalies like arterial stenoses are reported in pediatric patients with tuberous sclerosis complex (TSC). In addition, large renal lesions (angiomyolipoma—AML and cysts) are risk factors for arterial hypertension in adult patients with TSC. This study aimed to assess blood pressure, including central blood pressure and arterial damage (early vascular aging—EVA) in children with TSC.Materials and Methods: In a group of 33 pediatric patients with TSC (11.13 ± 4.03 years, 15 boys, 18 girls), we evaluated peripheral and central office blood pressure, 24-h ambulatory blood pressure, and arterial damage: aortic pulse wave velocity (aPWV) [m/s], [Z-score], augmentation index (AIx75HR [%]), common carotid artery intima-media thickness (cIMT) [mm], [Z-score], stiffness of common carotid artery (E-tracking), renal lesions in magnetic resonance and ultrasonography, and selected biochemical parameters. The control group consisted of 33 healthy children (11.23 ± 3.28 years, 15 boys, 18 girls).Results: In TSC group 7 (21.2%) children had arterial hypertension, 27 (81.8%) children had renal angiomyolipomas, 26 (78.8%)—renal cysts, and 4 (12.1%) patients were treated with mTOR inhibitors (2 patients with everolimus and 2 patients with sirolimus) at the moment of evaluation. Children with TSC had higher central systolic blood pressure (AoSBP) (98.63 ± 9.65 vs. 90.45 ± 6.87 [mm Hg], p < 0.001), cIMT (0.42 ± 0.05 vs. 0.39 ± 0.03 [mm], p = 0.011), cIMT Z-score (0.81 ± 1.21 vs. 0.16 ± 0.57, p = 0.007), aPWV (4.78 ± 0.81 vs. 4.25 ± 0.56 [m/s], p = 0.003) and aPWV Z-score (−0.14 ± 1.15 vs. −0.96 ± 0.87, p = 0.002) compared to healthy children, without differences in AIx75HR (8.71 ± 15.90 vs. 5.24 ± 11.12 [%], p = 0.319) and stiffness of common carotid artery. In children with TSC AoSBP correlated positively with serum cystatin C concentration (r = 0.377, p = 0.030) and with maximum diameter of renal cyst (R = 0.419, p = 0.033); mean arterial pressure (MAP) 24 h Z-score correlated with serum cystatin C concentration (R = 0.433, p = 0.013); and aPWV Z-score with daily urinary albumin loss [mg/24 h] (R = 0.412, p = 0.029).Conclusions: Children with tuberous sclerosis complex are at risk of elevated central blood pressure and early vascular aging. In children with TSC, blood pressure and arterial stiffness are related to renal involvement.Piotr SkrzypczykAnna Maria WabikMichał SzyszkaSergiusz JózwiakPrzemysław BombińskiAleksandra Jakimów-KostrzewaMichał BrzewskiMałgorzata Pańczyk-TomaszewskaFrontiers Media S.A.articletuberous sclerosis complexearly vascular agingcentral blood pressurearterial stiffnesscommon carotid artery intima-media thicknessarterial hypertensionPediatricsRJ1-570ENFrontiers in Pediatrics, Vol 9 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	tuberous sclerosis complex early vascular aging central blood pressure arterial stiffness common carotid artery intima-media thickness arterial hypertension Pediatrics RJ1-570
spellingShingle	tuberous sclerosis complex early vascular aging central blood pressure arterial stiffness common carotid artery intima-media thickness arterial hypertension Pediatrics RJ1-570 Piotr Skrzypczyk Anna Maria Wabik Michał Szyszka Sergiusz Józwiak Przemysław Bombiński Aleksandra Jakimów-Kostrzewa Michał Brzewski Małgorzata Pańczyk-Tomaszewska Early Vascular Aging in Children With Tuberous Sclerosis Complex
description	Objectives: Experimental data indicate that activating mutations in the mTOR (mammalian target of rapamycin) pathway may lead to abnormal arterial wall structure. Vascular anomalies like arterial stenoses are reported in pediatric patients with tuberous sclerosis complex (TSC). In addition, large renal lesions (angiomyolipoma—AML and cysts) are risk factors for arterial hypertension in adult patients with TSC. This study aimed to assess blood pressure, including central blood pressure and arterial damage (early vascular aging—EVA) in children with TSC.Materials and Methods: In a group of 33 pediatric patients with TSC (11.13 ± 4.03 years, 15 boys, 18 girls), we evaluated peripheral and central office blood pressure, 24-h ambulatory blood pressure, and arterial damage: aortic pulse wave velocity (aPWV) [m/s], [Z-score], augmentation index (AIx75HR [%]), common carotid artery intima-media thickness (cIMT) [mm], [Z-score], stiffness of common carotid artery (E-tracking), renal lesions in magnetic resonance and ultrasonography, and selected biochemical parameters. The control group consisted of 33 healthy children (11.23 ± 3.28 years, 15 boys, 18 girls).Results: In TSC group 7 (21.2%) children had arterial hypertension, 27 (81.8%) children had renal angiomyolipomas, 26 (78.8%)—renal cysts, and 4 (12.1%) patients were treated with mTOR inhibitors (2 patients with everolimus and 2 patients with sirolimus) at the moment of evaluation. Children with TSC had higher central systolic blood pressure (AoSBP) (98.63 ± 9.65 vs. 90.45 ± 6.87 [mm Hg], p < 0.001), cIMT (0.42 ± 0.05 vs. 0.39 ± 0.03 [mm], p = 0.011), cIMT Z-score (0.81 ± 1.21 vs. 0.16 ± 0.57, p = 0.007), aPWV (4.78 ± 0.81 vs. 4.25 ± 0.56 [m/s], p = 0.003) and aPWV Z-score (−0.14 ± 1.15 vs. −0.96 ± 0.87, p = 0.002) compared to healthy children, without differences in AIx75HR (8.71 ± 15.90 vs. 5.24 ± 11.12 [%], p = 0.319) and stiffness of common carotid artery. In children with TSC AoSBP correlated positively with serum cystatin C concentration (r = 0.377, p = 0.030) and with maximum diameter of renal cyst (R = 0.419, p = 0.033); mean arterial pressure (MAP) 24 h Z-score correlated with serum cystatin C concentration (R = 0.433, p = 0.013); and aPWV Z-score with daily urinary albumin loss [mg/24 h] (R = 0.412, p = 0.029).Conclusions: Children with tuberous sclerosis complex are at risk of elevated central blood pressure and early vascular aging. In children with TSC, blood pressure and arterial stiffness are related to renal involvement.
format	article
author	Piotr Skrzypczyk Anna Maria Wabik Michał Szyszka Sergiusz Józwiak Przemysław Bombiński Aleksandra Jakimów-Kostrzewa Michał Brzewski Małgorzata Pańczyk-Tomaszewska
author_facet	Piotr Skrzypczyk Anna Maria Wabik Michał Szyszka Sergiusz Józwiak Przemysław Bombiński Aleksandra Jakimów-Kostrzewa Michał Brzewski Małgorzata Pańczyk-Tomaszewska
author_sort	Piotr Skrzypczyk
title	Early Vascular Aging in Children With Tuberous Sclerosis Complex
title_short	Early Vascular Aging in Children With Tuberous Sclerosis Complex
title_full	Early Vascular Aging in Children With Tuberous Sclerosis Complex
title_fullStr	Early Vascular Aging in Children With Tuberous Sclerosis Complex
title_full_unstemmed	Early Vascular Aging in Children With Tuberous Sclerosis Complex
title_sort	early vascular aging in children with tuberous sclerosis complex
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/99fc50577e0e48aca91fd9e866ff1b17
work_keys_str_mv	AT piotrskrzypczyk earlyvascularaginginchildrenwithtuberoussclerosiscomplex AT annamariawabik earlyvascularaginginchildrenwithtuberoussclerosiscomplex AT michałszyszka earlyvascularaginginchildrenwithtuberoussclerosiscomplex AT sergiuszjozwiak earlyvascularaginginchildrenwithtuberoussclerosiscomplex AT przemysławbombinski earlyvascularaginginchildrenwithtuberoussclerosiscomplex AT aleksandrajakimowkostrzewa earlyvascularaginginchildrenwithtuberoussclerosiscomplex AT michałbrzewski earlyvascularaginginchildrenwithtuberoussclerosiscomplex AT małgorzatapanczyktomaszewska earlyvascularaginginchildrenwithtuberoussclerosiscomplex
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Early Vascular Aging in Children With Tuberous Sclerosis Complex

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