The effect of galsulfase enzyme replacement therapy on the growth of patients with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome).
Identifieur interne : 000218 ( Main/Exploration ); précédent : 000217; suivant : 000219The effect of galsulfase enzyme replacement therapy on the growth of patients with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome).
Auteurs : P. Harmatz [États-Unis] ; C J Hendriksz [Royaume-Uni] ; C. Lampe [Allemagne] ; J J Mcgill [Australie] ; R. Parini [Italie] ; E. Leão-Teles [Portugal] ; V. Valayannopoulos [France] ; T J Cole [Royaume-Uni] ; R. Matousek [États-Unis] ; S. Graham [États-Unis] ; N. Guffon [France] ; A. Quartel [États-Unis]Source :
- Molecular genetics and metabolism [ 1096-7206 ] ; 2017.
Abstract
Mucopolysaccharidosis (MPS) VI is an autosomal recessive lysosomal storage disorder arising from deficient activity of N-acetylgalactosamine-4-sulfatase (arylsulfatase B) and subsequent intracellular accumulation of the glycosaminoglycans (GAGs) dermatan sulfate and chondroitin-4-sulfate. Manifestations are multi-systemic and include skeletal abnormalities such as dysostosis multiplex and short stature. Reference height-for-age growth charts for treatment-naïve MPS VI patients have been published for both the slowly and rapidly progressing populations. Categorization of disease progression for these charts was based on urinary GAG (uGAG) level; high (>200μg/mg creatinine) levels identified subjects as rapidly progressing. Height data for 141 patients who began galsulfase treatment by the age of 18years were collected and stratified by baseline uGAG level and age at ERT initiation in 3-year increments. The reference MPS VI growth charts were used to calculate change in Z-score from pre-treatment baseline to last follow-up. Among patients with high baseline uGAG levels, galsulfase ERT was associated with an increase in Z-score for those beginning treatment at 0-3, >3-6, >6-9, >9-12, and >12-15years of age (p<0.05). Increases in Z-score were not detected for patients who began treatment between 15 and 18years of age, nor for patients with low (≤200μg/mg creatinine) baseline uGAG levels, regardless of age at treatment initiation. The largest positive deviation from untreated reference populations was seen in the high uGAG excretion groups who began treatment by 6years of age, suggesting an age- and severity-dependent impact of galsulfase ERT on growth.
DOI: 10.1016/j.ymgme.2017.03.008
PubMed: 28457718
Affiliations:
- Allemagne, Australie, France, Italie, Portugal, Royaume-Uni, États-Unis
- Angleterre, Auvergne-Rhône-Alpes, Californie, Grand Londres, Rhône-Alpes, Île-de-France
- Londres, Lyon, Paris
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Harmatz</name><affiliation wicri:level="2"><nlm:affiliation>UCSF Benioff Children's Hospital Oakland, Oakland, CA, USA. Electronic address: pharmatz@mail.cho.org.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>UCSF Benioff Children's Hospital Oakland, Oakland, CA</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Hendriksz, C J" sort="Hendriksz, C J" uniqKey="Hendriksz C" first="C J" last="Hendriksz">C J Hendriksz</name><affiliation wicri:level="1"><nlm:affiliation>University of Pretoria, The Mark Holland Metabolic Unit, Salford Royal Foundation NHS Trust, Salford, UK.</nlm:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University of Pretoria, The Mark Holland Metabolic Unit, Salford Royal Foundation NHS Trust, Salford</wicri:regionArea><wicri:noRegion>Salford</wicri:noRegion></affiliation></author><author><name sortKey="Lampe, C" sort="Lampe, C" uniqKey="Lampe C" first="C" last="Lampe">C. Lampe</name><affiliation wicri:level="1"><nlm:affiliation>Department of Pediatric and Adolescent Medicine, Villa Metabolica, University Medical Center of the University of Mainz, Germany.</nlm:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Pediatric and Adolescent Medicine, Villa Metabolica, University Medical Center of the University of Mainz</wicri:regionArea><wicri:noRegion>University Medical Center of the University of Mainz</wicri:noRegion><wicri:noRegion>University Medical Center of the University of Mainz</wicri:noRegion><wicri:noRegion>University Medical Center of the University of Mainz</wicri:noRegion></affiliation></author><author><name sortKey="Mcgill, J J" sort="Mcgill, J J" uniqKey="Mcgill J" first="J J" last="Mcgill">J J Mcgill</name><affiliation wicri:level="1"><nlm:affiliation>Department of Metabolic Medicine, Lady Cilento Children's Hospital, South Brisbane, QLD, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Department of Metabolic Medicine, Lady Cilento Children's Hospital, South Brisbane, QLD</wicri:regionArea><wicri:noRegion>QLD</wicri:noRegion></affiliation></author><author><name sortKey="Parini, R" sort="Parini, R" uniqKey="Parini R" first="R" last="Parini">R. Parini</name><affiliation wicri:level="1"><nlm:affiliation>UOS Malattie Metaboliche Rare, Clinica Pediatrica Ospedale San Gerardo, Monza, Italy.</nlm:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>UOS Malattie Metaboliche Rare, Clinica Pediatrica Ospedale San Gerardo, Monza</wicri:regionArea><wicri:noRegion>Monza</wicri:noRegion></affiliation></author><author><name sortKey="Leao Teles, E" sort="Leao Teles, E" uniqKey="Leao Teles E" first="E" last="Leão-Teles">E. Leão-Teles</name><affiliation wicri:level="1"><nlm:affiliation>Unidade Doenças Metabólicas, Hospital Pediátrico Integrado, Centro Hospitalar de S. João, Porto, Portugal.</nlm:affiliation><country xml:lang="fr">Portugal</country><wicri:regionArea>Unidade Doenças Metabólicas, Hospital Pediátrico Integrado, Centro Hospitalar de S. João, Porto</wicri:regionArea><wicri:noRegion>Porto</wicri:noRegion></affiliation></author><author><name sortKey="Valayannopoulos, V" sort="Valayannopoulos, V" uniqKey="Valayannopoulos V" first="V" last="Valayannopoulos">V. Valayannopoulos</name><affiliation wicri:level="3"><nlm:affiliation>Reference Center for Inherited Metabolic Diseases and IMAGINE Institute, Necker-Enfants Malades Hospital, Paris, France.</nlm:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Reference Center for Inherited Metabolic Diseases and IMAGINE Institute, Necker-Enfants Malades Hospital, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Cole, T J" sort="Cole, T J" uniqKey="Cole T" first="T J" last="Cole">T J Cole</name><affiliation wicri:level="3"><nlm:affiliation>Population, Policy and Practice Programme, UCL Institute of Child Health, London, UK.</nlm:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Population, Policy and Practice Programme, UCL Institute of Child Health, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Matousek, R" sort="Matousek, R" uniqKey="Matousek R" first="R" last="Matousek">R. Matousek</name><affiliation wicri:level="2"><nlm:affiliation>BioMarin Pharmaceutical Inc., Novato, CA, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>BioMarin Pharmaceutical Inc., Novato, CA</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Graham, S" sort="Graham, S" uniqKey="Graham S" first="S" last="Graham">S. Graham</name><affiliation wicri:level="2"><nlm:affiliation>BioMarin Pharmaceutical Inc., Novato, CA, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>BioMarin Pharmaceutical Inc., Novato, CA</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Guffon, N" sort="Guffon, N" uniqKey="Guffon N" first="N" last="Guffon">N. Guffon</name><affiliation wicri:level="3"><nlm:affiliation>Reference Center for Inherited Metabolic Diseases, Hôpital Femme Mère Enfant, Lyon, France.</nlm:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Reference Center for Inherited Metabolic Diseases, Hôpital Femme Mère Enfant, Lyon</wicri:regionArea><placeName><region type="region">Auvergne-Rhône-Alpes</region><region type="old region">Rhône-Alpes</region><settlement type="city">Lyon</settlement></placeName></affiliation></author><author><name sortKey="Quartel, A" sort="Quartel, A" uniqKey="Quartel A" first="A" last="Quartel">A. Quartel</name><affiliation wicri:level="2"><nlm:affiliation>BioMarin Pharmaceutical Inc., Novato, CA, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>BioMarin Pharmaceutical Inc., Novato, CA</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author></analytic><series><title level="j">Molecular genetics and metabolism</title><idno type="eISSN">1096-7206</idno><imprint><date when="2017" type="published">2017</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Mucopolysaccharidosis (MPS) VI is an autosomal recessive lysosomal storage disorder arising from deficient activity of N-acetylgalactosamine-4-sulfatase (arylsulfatase B) and subsequent intracellular accumulation of the glycosaminoglycans (GAGs) dermatan sulfate and chondroitin-4-sulfate. Manifestations are multi-systemic and include skeletal abnormalities such as dysostosis multiplex and short stature. Reference height-for-age growth charts for treatment-naïve MPS VI patients have been published for both the slowly and rapidly progressing populations. Categorization of disease progression for these charts was based on urinary GAG (uGAG) level; high (>200μg/mg creatinine) levels identified subjects as rapidly progressing. Height data for 141 patients who began galsulfase treatment by the age of 18years were collected and stratified by baseline uGAG level and age at ERT initiation in 3-year increments. The reference MPS VI growth charts were used to calculate change in Z-score from pre-treatment baseline to last follow-up. Among patients with high baseline uGAG levels, galsulfase ERT was associated with an increase in Z-score for those beginning treatment at 0-3, >3-6, >6-9, >9-12, and >12-15years of age (p<0.05). Increases in Z-score were not detected for patients who began treatment between 15 and 18years of age, nor for patients with low (≤200μg/mg creatinine) baseline uGAG levels, regardless of age at treatment initiation. The largest positive deviation from untreated reference populations was seen in the high uGAG excretion groups who began treatment by 6years of age, suggesting an age- and severity-dependent impact of galsulfase ERT on growth.</div></front></TEI><affiliations><list><country><li>Allemagne</li><li>Australie</li><li>France</li><li>Italie</li><li>Portugal</li><li>Royaume-Uni</li><li>États-Unis</li></country><region><li>Angleterre</li><li>Auvergne-Rhône-Alpes</li><li>Californie</li><li>Grand Londres</li><li>Rhône-Alpes</li><li>Île-de-France</li></region><settlement><li>Londres</li><li>Lyon</li><li>Paris</li></settlement></list><tree><country name="États-Unis"><region name="Californie"><name sortKey="Harmatz, P" sort="Harmatz, P" uniqKey="Harmatz P" first="P" last="Harmatz">P. Harmatz</name></region><name sortKey="Graham, S" sort="Graham, S" uniqKey="Graham S" first="S" last="Graham">S. Graham</name><name sortKey="Matousek, R" sort="Matousek, R" uniqKey="Matousek R" first="R" last="Matousek">R. Matousek</name><name sortKey="Quartel, A" sort="Quartel, A" uniqKey="Quartel A" first="A" last="Quartel">A. Quartel</name></country><country name="Royaume-Uni"><noRegion><name sortKey="Hendriksz, C J" sort="Hendriksz, C J" uniqKey="Hendriksz C" first="C J" last="Hendriksz">C J Hendriksz</name></noRegion><name sortKey="Cole, T J" sort="Cole, T J" uniqKey="Cole T" first="T J" last="Cole">T J Cole</name></country><country name="Allemagne"><noRegion><name sortKey="Lampe, C" sort="Lampe, C" uniqKey="Lampe C" first="C" last="Lampe">C. 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