Cargando…
Aortic Root Dilatation in Mucopolysaccharidosis I–VII
The prevalence of aortic root dilatation (ARD) in mucopolysaccharidosis (MPS) is not well documented. We investigated aortic root measurements in 34 MPS patients at the Children’s Hospital of Orange County (CHOC). The diagnosis, treatment status, age, gender, height, weight and aortic root parameter...
Autores principales: | , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2016
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5187804/ https://www.ncbi.nlm.nih.gov/pubmed/27916847 http://dx.doi.org/10.3390/ijms17122004 |
_version_ | 1782486901694922752 |
---|---|
author | Bolourchi, Meena Renella, Pierangelo Wang, Raymond Y. |
author_facet | Bolourchi, Meena Renella, Pierangelo Wang, Raymond Y. |
author_sort | Bolourchi, Meena |
collection | PubMed |
description | The prevalence of aortic root dilatation (ARD) in mucopolysaccharidosis (MPS) is not well documented. We investigated aortic root measurements in 34 MPS patients at the Children’s Hospital of Orange County (CHOC). The diagnosis, treatment status, age, gender, height, weight and aortic root parameters (aortic valve annulus (AVA), sinuses of Valsalva (SoV), and sinotubular junction (STJ)) were extracted by retrospective chart review and echocardiographic measurements. Descriptive statistics, ANOVA, and paired post-hoc t-tests were used to summarize the aortic dimensions. Exact binomial 95% confidence intervals (CIs) were constructed for ARD, defined as a z-score greater than 2 at the SoV. The patient age ranged from 3.4–25.9 years (mean 13.3 ± 6.1), the height from 0.87–1.62 meters (mean 1.24 ± 0.21), and the weight from 14.1–84.5 kg (mean 34.4 ± 18.0). The prevalence of dilation at the AVA was 41% (14/34; 95% CI: 25%–59%); at the SoV was 35% (12/34; 95% CI: 20%–54%); and at the STJ was 30% (9/30; 95% CI: 15%–49%). The highest prevalence of ARD was in MPS IVa (87.5%). There was no significant difference between mean z-scores of MPS patients who received treatment with hematopoietic stem cell transplantation (HSCT) or enzyme replacement therapy (ERT) vs. untreated MPS patients at the AVA (z = 1.9 ± 2.5 vs. z = 1.5 ± 2.4; p = 0.62), SoV (z = 1.2 ± 1.6 vs. z = 1.3 ± 2.2; p = 0.79), or STJ (z = 1.0 ± 1.8 vs. z = 1.2 ± 1.6; p = 0.83). The prevalence of ARD was 35% in our cohort of MPS I–VII patients. Thus, we recommend screening for ARD on a routine basis in this patient population. |
format | Online Article Text |
id | pubmed-5187804 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-51878042016-12-30 Aortic Root Dilatation in Mucopolysaccharidosis I–VII Bolourchi, Meena Renella, Pierangelo Wang, Raymond Y. Int J Mol Sci Article The prevalence of aortic root dilatation (ARD) in mucopolysaccharidosis (MPS) is not well documented. We investigated aortic root measurements in 34 MPS patients at the Children’s Hospital of Orange County (CHOC). The diagnosis, treatment status, age, gender, height, weight and aortic root parameters (aortic valve annulus (AVA), sinuses of Valsalva (SoV), and sinotubular junction (STJ)) were extracted by retrospective chart review and echocardiographic measurements. Descriptive statistics, ANOVA, and paired post-hoc t-tests were used to summarize the aortic dimensions. Exact binomial 95% confidence intervals (CIs) were constructed for ARD, defined as a z-score greater than 2 at the SoV. The patient age ranged from 3.4–25.9 years (mean 13.3 ± 6.1), the height from 0.87–1.62 meters (mean 1.24 ± 0.21), and the weight from 14.1–84.5 kg (mean 34.4 ± 18.0). The prevalence of dilation at the AVA was 41% (14/34; 95% CI: 25%–59%); at the SoV was 35% (12/34; 95% CI: 20%–54%); and at the STJ was 30% (9/30; 95% CI: 15%–49%). The highest prevalence of ARD was in MPS IVa (87.5%). There was no significant difference between mean z-scores of MPS patients who received treatment with hematopoietic stem cell transplantation (HSCT) or enzyme replacement therapy (ERT) vs. untreated MPS patients at the AVA (z = 1.9 ± 2.5 vs. z = 1.5 ± 2.4; p = 0.62), SoV (z = 1.2 ± 1.6 vs. z = 1.3 ± 2.2; p = 0.79), or STJ (z = 1.0 ± 1.8 vs. z = 1.2 ± 1.6; p = 0.83). The prevalence of ARD was 35% in our cohort of MPS I–VII patients. Thus, we recommend screening for ARD on a routine basis in this patient population. MDPI 2016-11-29 /pmc/articles/PMC5187804/ /pubmed/27916847 http://dx.doi.org/10.3390/ijms17122004 Text en © 2016 by the authors; licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC-BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Bolourchi, Meena Renella, Pierangelo Wang, Raymond Y. Aortic Root Dilatation in Mucopolysaccharidosis I–VII |
title | Aortic Root Dilatation in Mucopolysaccharidosis I–VII |
title_full | Aortic Root Dilatation in Mucopolysaccharidosis I–VII |
title_fullStr | Aortic Root Dilatation in Mucopolysaccharidosis I–VII |
title_full_unstemmed | Aortic Root Dilatation in Mucopolysaccharidosis I–VII |
title_short | Aortic Root Dilatation in Mucopolysaccharidosis I–VII |
title_sort | aortic root dilatation in mucopolysaccharidosis i–vii |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5187804/ https://www.ncbi.nlm.nih.gov/pubmed/27916847 http://dx.doi.org/10.3390/ijms17122004 |
work_keys_str_mv | AT bolourchimeena aorticrootdilatationinmucopolysaccharidosisivii AT renellapierangelo aorticrootdilatationinmucopolysaccharidosisivii AT wangraymondy aorticrootdilatationinmucopolysaccharidosisivii |