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Showing 1 to 12 of 172 entries
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The use of port-a-caths in adult patients with Lysosomal Storage Disorders receiving Enzyme Replacement Therapy-one centre experience.

Molecular genetics and metabolism reports

McLoughlin M, Stepien KM, McNelly B, Thompson L, Gorton J, Hendriksz CJ.
PMID: 29071212
Mol Genet Metab Rep. 2017 Oct 21;13:111-114. doi: 10.1016/j.ymgmr.2017.10.003. eCollection 2017 Dec.

Port-a-cath is a widely used device in patients with long-term venous access demand such as frequent or continuous administration of medications such as Enzyme Replacement Therapy (ERT), chemotherapy delivery, blood transfusions, blood products, and fluids. Patients with Lysosomal Storage...

Exercise-based cardiac rehabilitation in patients with chronic heart failure: a Dutch practice guideline.

Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation

Achttien RJ, Staal JB, van der Voort S, Kemps HM, Koers H, Jongert MW, Hendriks EJ.
PMID: 25492106
Neth Heart J. 2015 Jan;23(1):6-17. doi: 10.1007/s12471-014-0612-2.

RATIONALE: To improve the quality of exercise-based cardiac rehabilitation (CR) in patients with chronic heart failure (CHF) a practice guideline from the Dutch Royal Society for Physiotherapy (KNGF) has been developed.GUIDELINE DEVELOPMENT: A systematic literature search was performed to...

The oral health needs of children, adolescents and young adults affected by a mucopolysaccharide disorder.

JIMD reports

James A, Hendriksz CJ, Addison O.
PMID: 23430854
JIMD Rep. 2012;2:51-8. doi: 10.1007/8904_2011_46. Epub 2011 Sep 06.

BACKGROUND: As one of the 4 United Kingdom national centres for Lysosomal Storage Disorders, Birmingham Children's Hospital (BCH) cares for 49 patients with a mucopolysaccharide (MPS) disorder. Presently, their oral health needs have never been fully investigated. Enzyme therapies...

Mortality in patients with morquio syndrome a.

JIMD reports

Lavery C, Hendriksz C.
PMID: 24718838
JIMD Rep. 2015;15:59-66. doi: 10.1007/8904_2014_298. Epub 2014 Apr 10.

BACKGROUND: Morquio syndrome A (mucopolysaccharidosis type IVA) is an autosomal recessive, life-limiting lysosomal storage disease characterized by deficient activity of the enzyme galactosamine-6-sulfatase. The disease affects multiple body systems, and patients require multidisciplinary care from an early age.METHODS: To...

Exercise-based cardiac rehabilitation in patients with coronary heart disease: a practice guideline.

Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation

Achttien RJ, Staal JB, van der Voort S, Kemps HM, Koers H, Jongert MW, Hendriks EJ.
PMID: 23975619
Neth Heart J. 2013 Oct;21(10):429-38. doi: 10.1007/s12471-013-0467-y.

BACKGROUND: To improve the quality of exercise-based cardiac rehabilitation (CR) in patients with coronary heart disease (CHD) the CR guideline from the Dutch Royal Society for Physiotherapists (KNGF) has been updated. This guideline can be considered an addition to...

Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders.

Molecular genetics and metabolism reports

Hendriksz CJ, Harmatz P, Giugliani R, Roberts J, Arul GS.
PMID: 29845015
Mol Genet Metab Rep. 2018 Mar 07;15:71-73. doi: 10.1016/j.ymgmr.2018.02.007. eCollection 2018 Jun.

Totally implantable vascular access devices (TIVADs) are commonly used in conjunction with enzyme replacement therapy (ERT) for lysosomal storage disorders (LSDs). This case series describes potential complications associated with long-term TIVAD use, such as compromise of skin integrity, infection,...

Lipid profile in adult patients with Fabry disease - Ten-year follow up.

Molecular genetics and metabolism reports

Stepien KM, Hendriksz CJ.
PMID: 28736719
Mol Genet Metab Rep. 2017 Jul 13;13:3-6. doi: 10.1016/j.ymgmr.2017.06.010. eCollection 2017 Dec.

BACKGROUND: Fabry disease, an X-linked genetic condition, results from alpha-galactosidase deficiency and increased accumulation of glycosphingolipids in cardiovascular tissues. Clinical manifestation includes vasculature associated complications. Hyperlipidaemia is one of the cardiovascular risk factors however it has never been well...

Bichat guidelines for the clinical management of tularaemia and bioterrorism-related tularaemia.

Euro surveillance : bulletin Europeen sur les maladies transmissibles = European communicable disease bulletin

Bossi P, Tegnell A, Baka A, van Loock F, Werner A, Hendriks J, Maidhof H, Gouvras G.
PMID: 29183485
Euro Surveill. 2004 Dec;9(12):27-28. doi: 10.2807/esm.09.12.00503-en.

Francisella tularensis is one of the most infectious pathogenic bacteria known, requiring inoculation or inhalation of as few as 10 organisms to initiate human infection. Inhalational tularaemia following intentional release of a virulent strain of F. tularensis would have...

Bichat guidelines for the clinical management of botulism and bioterrorism-related botulism.

Euro surveillance : bulletin Europeen sur les maladies transmissibles = European communicable disease bulletin

Bossi P, Tegnell A, Baka A, Werner A, van Loock F, Hendriks J, Maidhof H, Gouvras G.
PMID: 29183487
Euro Surveill. 2004 Dec;9(12):31-32. doi: 10.2807/esm.09.12.00505-en.

Botulism is a rare but serious paralytic illness caused by botulinum toxin, which is produced by the Clostridium botulinum. This toxin is the most poisonous substance known. It 100 000 times more toxic than sarin gas. Eating or breathing...

Bichat guidelines for the clinical management of anthrax and bioterrorism-related anthrax.

Euro surveillance : bulletin Europeen sur les maladies transmissibles = European communicable disease bulletin

Bossi P, Tegnell A, Baka A, van Loock F, Hendriks J, Werner A, Maidhof H, Gouvras G.
PMID: 29183499
Euro Surveill. 2004 Dec;9(12):21-22. doi: 10.2807/esm.09.12.00500-en.

The spore-forming Bacillus anthracis must be considered as one of the most serious potential biological weapons. The recent cases of anthrax caused by a deliberate release reported in 2001 in the United States point to the necessity of early...

Oxygen drives hepatocyte differentiation and phenotype stability in liver cell lines.

Journal of cell communication and signaling

van Wenum M, Adam AAA, van der Mark VA, Chang JC, Wildenberg ME, Hendriks EJ, Jongejan A, Moerland PD, van Gulik TM, Oude Elferink RP, Chamuleau RAFM, Hoekstra R.
PMID: 29399736
J Cell Commun Signal. 2018 Sep;12(3):575-588. doi: 10.1007/s12079-018-0456-4. Epub 2018 Feb 04.

The in vitro generation of terminally differentiated hepatocytes is an unmet need. We investigated the contribution of oxygen concentration to differentiation in human liver cell lines HepaRG and C3A. HepaRG cells were cultured under hypoxia (5%O

Nightly sleep apnea severity in patients with atrial fibrillation: Potential applications of long-term sleep apnea monitoring.

International journal of cardiology. Heart & vasculature

Linz D, Baumert M, Desteghe L, Kadhim K, Vernooy K, Kalman JM, Dobrev D, Arzt M, Sastry M, Crijns HJGM, Schotten U, Cowie MR, McEvoy RD, Heidbuchel H, Hendriks J, Sanders P, Lau DH.
PMID: 31763438
Int J Cardiol Heart Vasc. 2019 Oct 18;24:100424. doi: 10.1016/j.ijcha.2019.100424. eCollection 2019 Sep.

In patients with atrial fibrillation (AF), the prevalence of moderate-to-severe sleep-disordered breathing (SDB) ranges between 21% and 72% and observational studies have demonstrated that SDB reduces the efficacy of rhythm control strategies, while treatment with continuous positive airway pressure...

Showing 1 to 12 of 172 entries