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Article ; Online: Clinical outcomes and patient satisfaction following total hip and knee arthroplasty in patients with inherited bleeding disorders: A 20-year single-surgeon cohort.

Anderson, J A / Mason, J A / Halliday, B

Haemophilia : the official journal of the World Federation of Hemophilia

2018 Volume 24, Issue 5, Page(s) 786–791

Abstract: Introduction: Persons with haemophilia and other inherited bleeding disorders are prone to disabling joint arthropathy frequently requiring arthroplasty for end-stage joint disease. Higher complication rates and more modest post-operative functional ... ...

Abstract	Introduction: Persons with haemophilia and other inherited bleeding disorders are prone to disabling joint arthropathy frequently requiring arthroplasty for end-stage joint disease. Higher complication rates and more modest post-operative functional outcomes have previously been described. Aim: To evaluate the clinical outcomes and patient satisfaction of persons with inherited bleeding disorders (predominantly haemophilia) undergoing total hip and knee replacement. Methods: Retrospective, single-centre cohort study with longitudinal assessment of patients with inherited bleeding disorders who underwent total hip and knee replacement over a 20-year period. Eligible patients were clinically assessed with Harris Hip Score (HHS), Knee Society Score (KSS), pain visual analogue scale (VAS) and a patient satisfaction questionnaire. Results: Thirty-one patients (48 joints) met the inclusion criteria. Mean age at surgery was 49.3 years (SD: 13.1, range 21-75 years) with a mean follow-up of 9.33 years (1.7-19.3). The majority (26/31) of patients had haemophilia A, predominantly severe haemophilia A (22/26). Reported pain levels were low, and patient satisfaction was high. Joint-specific outcome scores were "good" to "excellent" in 67% of total hip replacement patients and 92% of total knee replacement patients. A low complication rate was observed, with 2 patients requiring revision surgery and 4 patients requiring re-operation without implant revision. Conclusion: Arthroplasty is a reliable procedure in patients with inherited bleeding disorders with end-stage hip or knee arthropathy. The overall complexity of this group is highlighted, and the need for multidisciplinary care is emphasised.
MeSH term(s)	Adult ; Aged ; Arthroplasty, Replacement, Hip/methods ; Arthroplasty, Replacement, Knee/methods ; Female ; Hemorrhage/complications ; Hemorrhage/pathology ; Humans ; Male ; Middle Aged ; Patient Satisfaction ; Quality of Life/psychology ; Treatment Outcome ; Young Adult
Language	English
Publishing date	2018-07-19
Publishing country	England
Document type	Journal Article
ZDB-ID	1229713-6
ISSN	1365-2516 ; 1351-8216 ; 1355-0691
ISSN (online)	1365-2516
ISSN	1351-8216 ; 1355-0691
DOI	10.1111/hae.13559
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Article ; Online: Australian multicentre study of current real-world prophylaxis practice in severe and moderate haemophilia A and B.

Mason, J A / Parikh, S / Tran, H / Rowell, J / McRae, S

Haemophilia : the official journal of the World Federation of Hemophilia

2018 Volume 24, Issue 2, Page(s) 253–260

Abstract: Introduction: With the emergence of novel treatment products for haemophilia and an increasing focus on the benefits of pharmacokinetic driven individualized prophylaxis, robust national data with regard to current patterns of factor consumption and ... ...

Abstract	Introduction: With the emergence of novel treatment products for haemophilia and an increasing focus on the benefits of pharmacokinetic driven individualized prophylaxis, robust national data with regard to current patterns of factor consumption and adherence are required. Aim: To characterize current Australian practice with regard to use of prophylactic clotting factor infusions in patients with moderate or severe haemophilia A (HA) and haemophilia B (HB). Methods: This was a retrospective, non-interventional study utilizing Australian Bleeding Disorder Registry (ABDR) data collected over a 12 month period. Registered and consented patients with moderate or severe HA or HB without inhibitors were included. Results: A total of 718 HA (551 severe, 167 moderate) and 166 HB (87 severe, 79 moderate) patients were included. Regular prophylaxis was prescribed in 453 patients (82%) with severe HA, 42 patients (25%) with moderate HA, 66 patients (75%) with severe HB and 11 patients (14%) with moderate HB. Near universal prophylaxis was achieved in the paediatric subgroup. The mean weekly dose of factor VIII in severe HA was 84 international units/kg/wk (IU/kg/wk) vs 71 IU/kg/wk of factor IX in severe HB. Most patients on prophylaxis were treated ≥3 times/wk (HA) or 2 times/wk (HB). Non-adherence peaked in the 20-29 year age group. Older individuals on regular prophylaxis used more factor than was expected for their prescribed regimen. Conclusion: Prophylaxis rates in severe haemophilia are comparable with other developed nations. The benefit of a national registry is demonstrable. Furthermore research into the underlying reasons for non-compliance in young adults with haemophilia is required.
MeSH term(s)	Australia ; Female ; Hemophilia A/drug therapy ; Hemophilia A/pathology ; Hemophilia B/drug therapy ; Hemophilia B/pathology ; Humans ; Male
Language	English
Publishing date	2018-01-03
Publishing country	England
Document type	Journal Article ; Multicenter Study
ZDB-ID	1229713-6
ISSN	1365-2516 ; 1351-8216 ; 1355-0691
ISSN (online)	1365-2516
ISSN	1351-8216 ; 1355-0691
DOI	10.1111/hae.13375
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Article: OSL-dating of the Pleistocene-Holocene climatic transition in loess from China, Europe and North America, and evidence for accretionary pedogenesis

Constantin, D. / Mason, J.A. / Veres, D. / Hambach, U. / Panaiotu, C. / Zeeden, C. / Zhou, L. / Marković, S.B. / Gerasimenko, N. / Avram, A. / Tecsa, V. / Groza-Sacaciu, S.M. / del Valle Villalonga, L. / Begy, R. / Timar-Gabor, A.

Earth-science reviews. 2021 Oct., v. 221

2021

Abstract: Loess deposits intercalated by paleosols are detailed terrestrial archives of Quaternary climate variability providing information on the global dust cycle and landscape dynamics. Their paleoclimatic significance is often explored by quantifying their ... ...

Abstract	Loess deposits intercalated by paleosols are detailed terrestrial archives of Quaternary climate variability providing information on the global dust cycle and landscape dynamics. Their paleoclimatic significance is often explored by quantifying their mineral magnetic properties due to their sensitivity to local/regional hydroclimate variability. Detailed chronological assessment of such regional proxy records around the climatic transitions allow a better understanding of how regional records react to major global climatic transitions such as the Pleistocene-Holocene climatic transition.Logs of high-resolution magnetic susceptibility and its frequency dependence were used as paleoclimatic proxies to define the environmental transition from the last glacial loess to the current interglacial soil as reflected in nine loess-paleosol sequences across the northern hemisphere, from the Chinese Loess Plateau, the southeastern European loess belt and the central Great Plains, USA. The onset of increase in magnetic susceptibility above typical loess values was used to assess the onset of, and developments during, the Pleistocene-Holocene climatic transition.High-resolution luminescence dating was applied on multiple grain-sizes (4–11 μm, 63–90 μm, 90–125 μm) of quartz extracts from the same sample in order to investigate the timing of Pleistocene-Holocene climatic transition in the investigated sites.The magnetic susceptibility signal shows a smooth and gradual increase for the majority of the sites from the typical low loess values to the interglacial ones. The initiation of this increase, interpreted as recording the initiation of the Pleistocene-Holocene climatic transition at each site, was dated to 14–17.5 ka or even earlier. Our chronological results highlight the need of combining paleoclimatic proxies (magnetic susceptibility) with absolute dating when investigating the Pleistocene-Holocene climatic transition as reflected by the evolution of this proxy in order to avoid chronostratigraphic misinterpretations in loess-paleosol records caused by simple pattern correlation.The detailed luminescence chronologies evidence the continuity of eolian mineral dust accumulation regardless of glacial or interglacial global climatic regimes. Coupled with magnetic susceptibility records this indicates that dust sedimentation and pedogenesis act simultaneously and result in a non-negligible accretional component in the formation of Holocene soils in loess regions across the Northern Hemisphere. The luminescence ages allowed the modeling of accumulation rates for the Holocene soil which are similar for European, Chinese and U.S.A. loess sites investigated and vary from 2 cm ka⁻¹ to 9 cm ka⁻¹. While accretional pedogenesis has often been implicitly or explicitly assumed in paleoclimatic interpretation of loess-paleosol sequences, especially in the Chinese Loess Plateau, our luminescence data add direct evidence for ongoing sedimentation as interglacial soils formed.
Keywords	Holocene epoch ; climate ; dust ; evolution ; holocene soils ; landscapes ; loess ; luminescence ; magnetic susceptibility ; magnetism ; paleoclimatology ; paleosolic soil types ; quartz ; soil formation ; China ; Europe ; Great Plains region
Language	English
Dates of publication	2021-10
Publishing place	Elsevier B.V.
Document type	Article
ZDB-ID	1792-9
ISSN	0012-8252
ISSN	0012-8252
DOI	10.1016/j.earscirev.2021.103769
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Article: The clinical characterisation of systemic lupus erythematosus in a Far North Queensland Indigenous kindred.

Mason, J A / Bossingham, D

Lupus

2009 Volume 18, Issue 2, Page(s) 144–148

Abstract: The aetiology of systemic lupus erythematosus (SLE) is thought to involve both genetic and environmental factors. In other complex diseases, analysis of large multi-case families has resulted in insights into biological mechanisms. We have sought to ... ...

Abstract	The aetiology of systemic lupus erythematosus (SLE) is thought to involve both genetic and environmental factors. In other complex diseases, analysis of large multi-case families has resulted in insights into biological mechanisms. We have sought to characterise the members of an extended Indigenous family, five of whom have been diagnosed with SLE. Pedigree members were evaluated using the Lupus Screening Questionnaire, clinical interviews and medical records. Participants contributed blood and urine samples for laboratory analysis. A Mendelian pattern of inheritance was not observed. The five affected members (all female) shared two American College of Rheumatology criteria (positive ANA and arthritis) but showed a wide variety of other SLE manifestations. Disease onset, severity and progression were discordant. Including the five individuals with SLE, 15 blood relatives and two non-consanguineous spouses had autoimmune manifestations. Autoimmune haemolytic anaemia (one case), idiopathic thrombocytopenic purpura (ITP) (one case) and hypothyroidism (two cases) were observed in non-SLE affected individuals. Anti-nuclear antibodies were present in 12 blood relatives and one non-consanguineous spouse. Infections (especially of the skin) were observed to be common in the kindred. The lack of clear Mendelian inheritance or phenotypic concordance makes a rare monogenic explanation for SLE unlikely in this family. The finding of familial autoimmunity associated with SLE further supports the hypothesis that a common genetic pathway can precipitate autoimmunity, with further genes and possible environmental factors interacting to produce the eventual phenotype. Future genetic linkage studies may reveal a rare 'autoimmune gene' variant in this kindred.
MeSH term(s)	Adolescent ; Adult ; Female ; Genetic Predisposition to Disease ; Humans ; Lupus Erythematosus, Systemic/epidemiology ; Lupus Erythematosus, Systemic/genetics ; Male ; Middle Aged ; Oceanic Ancestry Group/genetics ; Pedigree ; Queensland ; Severity of Illness Index ; Young Adult
Language	English
Publishing date	2009-02
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1154407-7
ISSN	0961-2033
ISSN	0961-2033
DOI	10.1177/0961203308094997
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Article ; Online: Assessment and validation of a defined fluid restriction protocol in the use of subcutaneous desmopressin for children with inherited bleeding disorders.

Mason, J A / Robertson, J D / McCosker, J / Williams, B A / Brown, S A

Haemophilia : the official journal of the World Federation of Hemophilia

2016 Volume 22, Issue 5, Page(s) 700–705

Abstract: Introduction: Despite the availability of subcutaneous desmopressin (1-deamino-8-d-arginine vasopressin, SC-DDAVP) as a haemostatic agent for children with mild bleeding disorders, few publications specifically address the safety or efficacy of this ... ...

Abstract	Introduction: Despite the availability of subcutaneous desmopressin (1-deamino-8-d-arginine vasopressin, SC-DDAVP) as a haemostatic agent for children with mild bleeding disorders, few publications specifically address the safety or efficacy of this mode of administration. Aim: Our aim was to assess whether a defined fluid restriction protocol was effective in preventing hyponatremia in children receiving perioperative SC-DDAVP, and to document adequate biological and clinical response in this setting. Methods: We retrospectively analysed a cohort of children with mild bleeding disorders prescribed SC-DDAVP over a 5-year period following institution of a 'two-thirds maintenance' fluid restriction protocol. Results: Sixty-nine patients received SC-DDAVP following this protocol, including 15 with mild haemophilia A, 49 with von Willebrand disease (VWD) and five with platelet storage pool disorder. In patients who underwent formal preoperative assessment a complete or partial response was observed in 28/29 with type 1 VWD and 14/15 with mild haemophilia A. Perioperative SC-DDAVP provided excellent haemostasis in all patients, with no requirement for factor concentrate or blood products. Mild asymptomatic hyponatremia was detected in seven children who received multiple doses of DDAVP (lowest sodium 129 mmol L(-1) ); however, adherence to the prescribed fluid restriction protocol was questionable in six of these cases. Symptomatic hyponatremia was not observed. Conclusion: Subcutaneous desmopressin was well-tolerated, with no serious side-effects observed, and good biological responses in preoperative trials. A two-thirds maintenance fluid regimen was effective at preventing symptomatic hyponatremia in our cohort, and is now the standard protocol for fluid restriction post-DDAVP administration in our centre.
MeSH term(s)	Adolescent ; Blood Coagulation Disorders, Inherited/drug therapy ; Blood Coagulation Disorders, Inherited/pathology ; Child ; Child, Preschool ; Deamino Arginine Vasopressin/adverse effects ; Deamino Arginine Vasopressin/therapeutic use ; Hemophilia A/drug therapy ; Hemophilia A/pathology ; Hemostatics/adverse effects ; Hemostatics/therapeutic use ; Humans ; Hyponatremia/etiology ; Injections, Subcutaneous ; Platelet Storage Pool Deficiency/drug therapy ; Platelet Storage Pool Deficiency/pathology ; Retrospective Studies ; Severity of Illness Index ; von Willebrand Diseases/drug therapy ; von Willebrand Diseases/pathology
Chemical Substances	Hemostatics ; Deamino Arginine Vasopressin (ENR1LLB0FP)
Language	English
Publishing date	2016-09
Publishing country	England
Document type	Journal Article
ZDB-ID	1229713-6
ISSN	1365-2516 ; 1351-8216 ; 1355-0691
ISSN (online)	1365-2516
ISSN	1351-8216 ; 1355-0691
DOI	10.1111/hae.12949
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Article: Parent material influence on soil response to vegetation change, Southeastern Minnesota, U.S.A.

Mason, J. A. / Jacobs, P. M. / Gruley, K. E. / Reyerson, P. / Hanson, P. R.

Geoderma

2016 Volume -, Issue 275, Page(s) 1

Language	English
Document type	Article
ZDB-ID	281080-3
ISSN	0016-7061
Database	Current Contents Nutrition, Environment, Agriculture

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Article: Laser diffraction analysis of the disintegration of aeolian sedimentary aggregates in water

Mason, J. A. / Greene, R. S. B. / Joeckel, R. M.

Catena

2011 Volume 87, Issue 1, Page(s) 107

Language	English
Document type	Article
ZDB-ID	519608-5
ISSN	0341-8162
Database	Current Contents Nutrition, Environment, Agriculture

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Article: A new method to constrain soil development time using both OSL and radiocarbon dating

Miao, X. / Wang, H. / Hanson, P. R. / Mason, J. A. / Liu, X.

Geoderma

2015 Volume -, Issue 261, Page(s) 93

Language	English
Document type	Article
ZDB-ID	281080-3
ISSN	0016-7061
Database	Current Contents Nutrition, Environment, Agriculture

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Article ; Online: Universal infant hearing screening by automated auditory brainstem response measurement.

Mason, J A / Herrmann, K R

Pediatrics

1998 Volume 101, Issue 2, Page(s) 221–228

Abstract: Background: Our purpose was to identify infants with a bilateral, permanent, handicapping hearing loss and to provide them with amplification before age 6 months.: Methodology: The study population consisted of 10,372 infants born during a 5-year ... ...

Abstract	Background: Our purpose was to identify infants with a bilateral, permanent, handicapping hearing loss and to provide them with amplification before age 6 months. Methodology: The study population consisted of 10,372 infants born during a 5-year period. Universal hearing screening by automated auditory brainstem response was done in the nursery. Infants who failed the screening test were followed up diagnostically. Infants who were not tested in the nursery were followed up as outpatients. Hearing aids were recommended for those infants who had bilateral hearing loss. Results: Successful screening in the nursery was achieved for 96% of infants. The failure rate was 4%. The incidence of bilateral loss requiring amplification was 1.4/1000. The false-positive rate was 3.5% after the initial screening and .2% when a two-stage screening procedure was used. The incidence of congenital bilateral hearing loss in the well population was 1/1000, and in the neonatal intensive care unit population, 5/1000. The cost of screening was $17 per infant, and the cost to identify each true bilateral hearing loss was $17,750. Amplification was recommended for 15 infants; well infants who used hearing aids before age 6 months achieved age-appropriate speech and language development. Conclusions: Mild, moderate, and severe bilateral, persistent hearing loss can be identified in the nursery by automated auditory brainstem response measurement to provide amplification before age 6 months and thus optimize speech and language development.
MeSH term(s)	Audiometry/economics ; Audiometry/methods ; Costs and Cost Analysis ; Evoked Potentials, Auditory, Brain Stem ; False Positive Reactions ; Follow-Up Studies ; Hearing Loss, Bilateral/congenital ; Hearing Loss, Bilateral/diagnosis ; Hearing Loss, Bilateral/epidemiology ; Humans ; Incidence ; Infant, Newborn ; Neonatal Screening/economics
Language	English
Publishing date	1998-02
Publishing country	United States
Document type	Journal Article
ZDB-ID	207677-9
ISSN	1098-4275 ; 0031-4005
ISSN (online)	1098-4275
ISSN	0031-4005
DOI	10.1542/peds.101.2.221
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Article ; Online: Selective thermal emission from patterned steel.

Mason, J A / Adams, D C / Johnson, Z / Smith, S / Davis, A W / Wasserman, D

Optics express

2010 Volume 18, Issue 24, Page(s) 25192–25198

Abstract: Patterned metal films have been shown to possess unique optical properties resulting from the excitation of surface plasmon polaritons at the patterned metal surface. Here we demonstrate spectrally selective thermal emission from patterned steel ... ...

Abstract	Patterned metal films have been shown to possess unique optical properties resulting from the excitation of surface plasmon polaritons at the patterned metal surface. Here we demonstrate spectrally selective thermal emission from patterned steel substrates. The materials and processes used in this work were chosen for their potential scalability to large-area and low cost production of metal films with distinct and designable thermal signatures. The samples studied were characterized by reflection and emission spectroscopy, and a factor of 2.6 emission enhancement is demonstrated for the design wavelength. These results are compared to numerical simulations.
Language	English
Publishing date	2010-11-22
Publishing country	United States
Document type	Journal Article
ZDB-ID	1491859-6
ISSN	1094-4087 ; 1094-4087
ISSN (online)	1094-4087
ISSN	1094-4087
DOI	10.1364/OE.18.025192
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