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Medicinteknisk utrustning (MT) har en stor och avgörande roll i dagens samhälle för att kunna utföra en patientsäker sjukvård. De senaste tekniska framstegen har försett sjukvården med otaliga skräddarsydda MT-produkter för olika behov som uppstår vid behandling av patienterna. Trots detta har vissa hälso- och sjukvårdssektorer särskilda behov som inte kan tillgodoses genom marknadens utbud. När en sådan situation uppstår, tillåter regelverket sjukvårdsverksamheter att utveckla egentillverkade produkter eller modifiera befintlig MT-utrustning för att lösa det specifika sjukvårdsproblemet. Egentillverkning av en MT-produkt är en reglerad process i enlighet med EU-förordning om MT-produkter MDR (EU 2017/745) artikel 5.5. Detta regelverk säkerställer en patientsäker och kvalitetssäkrad MT-utrustning för intern användning inom sjukvårdsverksamheten.  Karolinska Universitetssjukhuset (Karolinska) som är Nordens största sjukhus har, i enlighet med regelverket, tagit fram egna interna riktlinjer för egentillverkning av MT-produkter. Covid-19-pandemin har aktualiserat att tillämpa dessa rutiner i praktiken. Karolinska har vårdat ett stort antal Covid-19-patienter med svåra respiratoriska symptom som har krävt ett särskilt omhändertagande bland annat på intensivvårdsavdelningen (IVA).  Ett av de mest förekommande kirurgiska ingreppen på IVA-patienter under denna period har varit trakeotomi. På grund av de stora svårigheter som uppstår med att flytta patienten till vanlig operationssal har öron-näsa-halsenheten (ÖNH) på Karolinska, som ansvarar för alla kirurgiska trakeotomier på sjukhuset, behövt utföra operationen på patienten i IVA-sängen. För att utföra en mer patientsäker på-plats-operation och förbättra denna ohållbara arbetsmiljö har ÖNH-kirurger önskat och sökt ett mobilt operationsbord som kan dockas till de olika IVA-sängtyperna som finns på sjukhuset. Eftersom detta operationsbord inte fanns att köpa på marknaden har MT-mekaniska verkstaden på Karolinska utvecklat en prototyp av trakeotomibordet som uppfyller ÖNH-kirurgernas specifika behov.  I detta arbete undersöktes egentillverkning av MT-produkter inom Karolinska i samband med utveckling av trakeotomibordet. I detta avseende studerades hela processen som krävs av regelverket och Karolinskas interna föreskrifter, från behovsanalys och definition av MT-produkten till kvalitetssäkring och riskhanteringen som garanterar en godkänd MT-utrustning för intern användning inom Karolinska Universitetssjukhuset. / Medical devices have a crucial role in performing a high-quality healthcare with patient safety in focus. Recent technological advances have provided healthcare systems with countless customized medical products for the various needs that arise in the treatment of patients. Despite this, some health care sectors have special needs that cannot be met by the market supply. In such circumstances, the regulations allow healthcare providers to develop in-house production or modify existing medical equipment to solve the specific need of the medical staff. In-house production of a medical device is a detailed regulated process defined in accordance with MDR (EU 2017/745) Article 5 (5). This regulatory framework ensures a patient-safe and quality-assured medical equipment for internal use within the healthcare.  Karolinska University Hospital (Karolinska), which is the Nordic region's largest hospital, follows the regulatory praxis for in-house production of medical devices. These praxes were put into use during the Covid-19 pandemic. Karolinska had to take care of many Covid-19 patients with severe respiratory symptoms who required special care, including intensive care unit (ICU).  One of the most common surgical procedures on ICU patients during this period was tracheotomy. Due to the complications that occur in moving patients to regular operating theatre, the ear-nose-throat unit (ENT) at Karolinska, which is responsible for all surgical tracheotomies at the hospital, has had to perform operations directly on ICU beds.  To improve the quality of the operations and the surgeon's work condition, the Karolinska ENT have sought a mobile operating table with ability to be docked to various ICU beds, available at the hospital. Since this medical equipment was not available on the market, the medical engineering workshop at Karolinska has developed a prototype of the tracheotomy table that meets the specific needs of ENT surgeons. This project studies the in-house production of a medical device at Karolinska through examining the development of tracheostomy table’s prototype by medical engineering workshop. In this respect, the regulatory requirements that approve the tracheostomy table for internal use within Karolinska University Hospital, are studied.

Research on the Berard Auditory Integration Training method has shown improvement in the regulation of attention, activity and impulsivity of children whose auditory system have been re-trained. Anecdotal reports have found improvements in sleeping patterns, balance, allergies, eyesight, eating patterns, depression and other seemingly unrelated physiological states. During the Auditory Integration Training (AIT) procedure dynamic music, with a wide range of frequencies, is processed through a system of filters in the AIT device. The volume and tone of the music is constantly and randomly modulated. The researcher explored Berard AIT as a possible intervention for learners between the ages of six and twelve years, who experienced problems with sustained attention and who have difficulty in shifting their attention from task to task. The aim was to investigate if Berard AIT method would enable the learner to stay calm, relaxed yet alert, with focused attention and thus concentration with appropriate reflection before action. An experimental quantative study, with a pretest-posttest control group design, was conducted to investigate the effect that Berard Auditory Integration Training has on learners with attention problems. Checklists and different measurements were conducted before the training started and repeated three months after the training was completed, to monitor if any change in the way that the learners pay attention, occurred the case group of ten learners was compared with ten learners in a control group who did not initially receive the intervention. Although the results of the post-tests varied amongst the learners, most learners in the experimental group experienced a statistical significant increase in attention control and a reduction in behavioural problems by the third month following the listening sessions. / Thesis (M.Ed.), North-West University, Vaal Triangle Campus, 2010

Research on the Berard Auditory Integration Training method has shown improvement in the regulation of attention, activity and impulsivity of children whose auditory system have been re-trained. Anecdotal reports have found improvements in sleeping patterns, balance, allergies, eyesight, eating patterns, depression and other seemingly unrelated physiological states. During the Auditory Integration Training (AIT) procedure dynamic music, with a wide range of frequencies, is processed through a system of filters in the AIT device. The volume and tone of the music is constantly and randomly modulated. The researcher explored Berard AIT as a possible intervention for learners between the ages of six and twelve years, who experienced problems with sustained attention and who have difficulty in shifting their attention from task to task. The aim was to investigate if Berard AIT method would enable the learner to stay calm, relaxed yet alert, with focused attention and thus concentration with appropriate reflection before action. An experimental quantative study, with a pretest-posttest control group design, was conducted to investigate the effect that Berard Auditory Integration Training has on learners with attention problems. Checklists and different measurements were conducted before the training started and repeated three months after the training was completed, to monitor if any change in the way that the learners pay attention, occurred the case group of ten learners was compared with ten learners in a control group who did not initially receive the intervention. Although the results of the post-tests varied amongst the learners, most learners in the experimental group experienced a statistical significant increase in attention control and a reduction in behavioural problems by the third month following the listening sessions. / Thesis (M.Ed.), North-West University, Vaal Triangle Campus, 2010

When people hear the word "nurse," they often think of qualities such as compassion and patience. While these are essential characteristics, nurses must go even further when striving for professionalism. Nurses also need strong morals and ethics and the commitment to always act in the best interests of their patients. The term professionalism embraces a set of attitudes, skills and behaviours, attributes and values which are expected from those to whom society has extended the privilege of being considered a professional. The core values of professionalism include: honesty, integrity, altruism, respect, responsibility, accountability, compassion, empathy, dedication, self-improvement, competency of clinical skills and knowledge. Professional nursing practice is a commitment to compassion, caring and strong ethical values, continuous development of self and others, accountability and responsibility for insightful practice, demonstrating a spirit of collaboration and flexibility. Rapid changes in the nursing sector have recently occurred across all areas and settings, making for a chaotic and seldom unstable work environment. All of these changes have impacted the ability of ENA learners to maintain high levels of professionalism and collaboration. In particular, the researcher, as an educator within a private NEI responsible for teaching ENA learners, experienced a lack of professionalism amongst ENA learners during the course of their training, as well as after their enrolment with the South African Nursing Council (SANC). Currently, there seems to be a difference between the professionalism portrayed by ENA learners and that which is expected of them. The overall aim of this research is to enhance the professionalism of ENA learners in a private NEI in Potchefstroom, North West. The following objectives have been identified in order to reach this aim: * To explore and describe professionalism amongst ENA learners in a private NEI in Potchefstroom, North West, and * To formulate recommendations to advance professionalism amongst ENA learners in a private NEI in Potchefstroom, North West. This study was conducted by means of a case study approach as the selected topic focuses only on a private nursing education institution situated in Potchefstroom, North West. From the qualitative instrumental case study approach, a qualitative, explorative, contextual research design was followed. The population in the study was all the ENA learners of a private NEI in Potchefstroom, North West. A sample was selected through non-probable, purposive sampling according to inclusion criteria (n=25). Data collection was conducted by means of a ―World Café‖ method and a focus group. The data collected through the World Café method was recorded and transcribed and reconstructed by means of thematic analysis. The main theme that crystallised from data analysis was that ENA learners viewed professionalism as a set of behaviours that are displayed in their external environment. The behaviours are grouped into the following five subgroups, namely punctuality versus absenteeism, adhering to scheduling and duty hours, responsibility of observations and awareness, the role of dress code and the image of nursing and finally obedience to organisational rules and regulations and tolerance towards others. The World Café and focus group results were integrated with case records according to repetitive themes. From the results and conclusions, recommendations were formulated for nursing practice, nursing education and nursing research to enhance professionalism amongst ENA learners in general. / MCur, North-West University, Potchefstroom Campus, 2014

A Mucopolissacaridose tipo IVA (MPS IVA), é uma doença lisossômica de depósito ocasionada pela degradação deficiente dos glicosaminoglicanos (GAG) queratan sulfato e condroitin-6-sulfato devido à deficiência da enzima N-acetilgalactosamina 6-sulfatase. Como a fisiopatologia desta doença ainda não está totalmente elucidada e muitos estudos vêm demonstrando o envolvimento do estresse oxidativo e inflamação na patogênese de outros tipos de mucopolissacaridoses, o objetivo principal deste trabalho foi investigar os parâmetros de estresse oxidativo e mediadores inflamatórios em pacientes MPS IVA sob terapia de reposição enzimática (TRE). Foram analisadas amostras de urina e sangue de pacientes MPS IVA sob TRE (n=17) e controles saudáveis pareados por idade (n=10-14). Os pacientes apresentaram diminuição significativa nos níveis de defesas antioxidantes, medida através dos níveis de glutationa reduzida (GSH), e aumento da enzima superóxido dismutase (SOD) em eritrócitos. Em relação ao dano a biomoléculas, foi observado nos pacientes um aumento de lipoperoxidação (aumento dos níveis de isoprostanos urinários) e de dano oxidativo a proteínas (aumento dos níveis urinários de di-tirosina e diminuição dos grupamentos sulfidrila no plasma) comparativamente aos controles. Nossos resultados também mostraram que os pacientes MPS IVA sob TRE apresentaram maior dano ao DNA, sendo este dano de origem oxidativa e atingindo bases purínicas e pirimidínicas. Além disso, os pacientes apresentaram níveis significativamente aumentados de interleucina-6 e esta, por sua vez, apresentou correlação negativa com GSH, mostrando uma possível relação entre inflamação e estresse oxidativo nesta doença. Considerando que os níveis de GAG urinários ainda se encontravam elevados nos pacientes em comparação com o grupo controle, pode-se supor que os mesmos estejam, pelo menos em parte, correlacionados com os danos oxidativos encontrados nestes pacientes. Os dados encontrados no presente trabalho sugerem que pacientes MPS IVA sob TRE apresentam uma condição pró-inflamatória e oxidativa e que a suplementação com antioxidantes em combinação com a TRE deve ser investigada com o intuito de melhorar a qualidade de vida dos pacientes. Cabe ressaltar que este é o primeiro estudo em pacientes que relaciona MPS IVA com estresse oxidativo e inflamação. / The Mucopolysaccharidosis type IVA (MPS IVA) is a lysosomal storage disease caused by impaired degradation of keratan sulfate and chondroitin-6-sulfate glycosaminoglycans (GAG), due to a deficiency on the enzyme N-acetylgalactosamine-6-sulfatase. Since the pathophysiology of this disease is still not totally elucidated and many studies demonstrated the involvement of oxidative stress and inflammation in the pathogenesis of other mucopolysaccharidoses types, the principal objective of this study was to investigate oxidative stress parameters and inflammatory mediators in MPS IVA patients under enzyme replacement therapy (ERT). Urine anda blood samples of MPS IVA patients under ERT (n= 17) and healthy age-matched controls (n= 10-14) were evaluated. Patients presented a significant decrease in antioxidant defenses levels, assessed by reduced glutathione (GSH), and increased superoxide dismutase (SOD) activity in erythrocytes. With regard to the biomolecules damage, was observed that patients presented lipid peroxidation (increase of isoprostanes urinary levels) and protein damage (increase of di-tyrosine urinary levels and decrease of sulfhydryl groups in plasma), when compared to controls. Our results showed higher DNA damage levels in MPS IVA patients compared to control group, in both pyrimidines and purines bases. The pro-inflammatory cytokine interleukin 6 (IL-6) was significantly increased in patients and showed an inverse correlation with GSH levels, showing a possible link between inflammation and oxidative stress in MPS IVA disease. Considering that GAG urinary level were still high in ERT patients compared to the control group, we propose that GAG are, at least in part, related with oxidative damage found in MPS IVA patients. The data presented suggest that pro-inflammatory and pro-oxidant states occur in MPS IVA patients under ERT, and the supplementation of antioxidants in combination with ERT can be investigated with the purpose of improving the patient’s life quality. To the best of our knowledge, this is the first study in patients relating MPS IVA with oxidative stress and inflammation.

The Deepwater Horizon oil spill that started on April 20, 2010, in the Gulf of Mexico was the largest marine oil spill in the history of the petroleum industry. There was an unexpected and prolonged sedimentation event of oil-associated marine snow to the seafloor due to the oil spill. The sedimentation event occurred because of the coagulation process among oil associated marine particles. Marine scientists are developing models for the coagulation process of marine particles and oil, in order to estimate the amount of oil that may reach the seafloor along with marine particles. These models, used certain assumptions regarding the shape and the texture parameters of marine particles. Such assumptions may not be based on accurate information or may vary during and after the oil spill. The work performed here provided a quantitative analysis of the assumptions used in modeling the coagulation process of marine particles. It also investigated the changes in model parameters (shape and texture) during and after the Deepwater Horizon oil spill in different seasons (spring and summer). An Interactive Visualization Application was developed for data exploration and visual analysis of the trends in these parameters. An Interactive Statistical Analysis Application was developed to create a statistical summary of these parameter values.

When people hear the word "nurse," they often think of qualities such as compassion and patience. While these are essential characteristics, nurses must go even further when striving for professionalism. Nurses also need strong morals and ethics and the commitment to always act in the best interests of their patients. The term professionalism embraces a set of attitudes, skills and behaviours, attributes and values which are expected from those to whom society has extended the privilege of being considered a professional. The core values of professionalism include: honesty, integrity, altruism, respect, responsibility, accountability, compassion, empathy, dedication, self-improvement, competency of clinical skills and knowledge. Professional nursing practice is a commitment to compassion, caring and strong ethical values, continuous development of self and others, accountability and responsibility for insightful practice, demonstrating a spirit of collaboration and flexibility. Rapid changes in the nursing sector have recently occurred across all areas and settings, making for a chaotic and seldom unstable work environment. All of these changes have impacted the ability of ENA learners to maintain high levels of professionalism and collaboration. In particular, the researcher, as an educator within a private NEI responsible for teaching ENA learners, experienced a lack of professionalism amongst ENA learners during the course of their training, as well as after their enrolment with the South African Nursing Council (SANC). Currently, there seems to be a difference between the professionalism portrayed by ENA learners and that which is expected of them. The overall aim of this research is to enhance the professionalism of ENA learners in a private NEI in Potchefstroom, North West. The following objectives have been identified in order to reach this aim: * To explore and describe professionalism amongst ENA learners in a private NEI in Potchefstroom, North West, and * To formulate recommendations to advance professionalism amongst ENA learners in a private NEI in Potchefstroom, North West. This study was conducted by means of a case study approach as the selected topic focuses only on a private nursing education institution situated in Potchefstroom, North West. From the qualitative instrumental case study approach, a qualitative, explorative, contextual research design was followed. The population in the study was all the ENA learners of a private NEI in Potchefstroom, North West. A sample was selected through non-probable, purposive sampling according to inclusion criteria (n=25). Data collection was conducted by means of a ―World Café‖ method and a focus group. The data collected through the World Café method was recorded and transcribed and reconstructed by means of thematic analysis. The main theme that crystallised from data analysis was that ENA learners viewed professionalism as a set of behaviours that are displayed in their external environment. The behaviours are grouped into the following five subgroups, namely punctuality versus absenteeism, adhering to scheduling and duty hours, responsibility of observations and awareness, the role of dress code and the image of nursing and finally obedience to organisational rules and regulations and tolerance towards others. The World Café and focus group results were integrated with case records according to repetitive themes. From the results and conclusions, recommendations were formulated for nursing practice, nursing education and nursing research to enhance professionalism amongst ENA learners in general. / MCur, North-West University, Potchefstroom Campus, 2014

Mucopolissacaridoses (MPS) são erros inatos do metabolismo, fazem parte das doenças lisossômicas de depósito e ocorrem devido à deficiência na atividade de enzimas que catalisam a degradação de glicosaminoglicanos (GAGs). São doenças crônicas e progressivas causadas pelo acúmulo de um ou mais GAGs nos lisossomos das células. A MPS do tipo IV (síndrome de Morquio) ocorre pelo acúmulo de queratan sulfato e condoitin-6-sulfato, causado pela deficiência de duas diferentes enzimas, que distinguem essa MPS em dois subtipos, deficiência de N-acetilgalactosamina-6-sulfatase (MPS IV tipo A) ou de β-galactosidase (MPS IV tipo B). Na MPS IV A as características clínicas predominantes são relacionadas ao sistema esquelético e na maioria das vezes o paciente tem inteligência normal; não há uma terapia efetiva para a doença, deste modo o diagnóstico precoce direciona para o início de tratamentos paliativos. A investigação inicial dessa MPS se baseia em avaliação clínica, radiografias, quantificação e identificação de GAGs urinários e o diagnóstico definitivo pela medida da atividade enzimática da N-acetilgalactosamina-6-sulfatase (GALNS) em leucócitos ou fibroblastos. O objetivo desse trabalho foi estudar alguns parâmetros bioquímicos da enzima GALNS em leucócitos de indivíduos normais e adaptar a técnica de medida dessa atividade enzimática para sangue impregnado em papel filtro. O Km e a Vmáx da GALNS em leucócitos de indivíduos normais foi de 7,16mM e 77,3nmol/17h/mg de proteína, respectivamente. Também adaptamos a técnica da medida da atividade enzimática dessa enzima utilizando menor concentração de substrato (3mM), esta concentração teve correlação positiva com a concentração 10 mM, que é empregada na técnica original. A partir disto, foram estabelecidos os valores de referência em leucócitos para indivíduos normais e indivíduos afetados com MPS IV A. Os mesmos foram, respectivamente: 11,0 a 101,4 e 0,1 a 0,2 nmol/17h/mg de proteína. Foi também observado que, com o passar do tempo, a temperatura de 70ºC causa uma inativação gradual da atividade enzimática. Por último, apesar de várias alterações na técnica original em leucócitos, não foi possível adaptá-la para ser utilizada na medida da atividade da GALNS em sangue impregnado em papel filtro. A determinação dos parâmetros bioquímicos da GALNS é conveniente devido ao avanço nos estudos clínicos de desenvolvimento da terapia de reposição enzimática aos indivíduos afetados com MPS IV A e na distinção de indivíduos saudáveis, afetados e heterozigotos. Desenvolver técnicas fluorimétricas em amostras de sangue impregnado em papel filtro é de grande importância para serem utilizadas em triagem de recém-nascidos e populações de alto risco para MPS. Devendo ser confirmado por testes de atividade da enzima em leucócitos ou fibroblastos cultivados. A partir dos nossos protocolos, não foi possível desenvolver e padronizar uma técnica para medida da atividade da enzima N-acetilgalactosamina-6-sulfatase em sangue impregnado em papel filtro de modo a triarmos indivíduos com suspeita de MPS IV A. / Mucopolysaccharidosis (MPS) are inborn errors of metabolism, are part of the lysosomal storage diseases and occur due to the deficiency in the activity of enzymes that catalyze the degradation of glycosaminoglycans (GAGs). They are chronic and progressive diseases caused by the accumulation of one or more GAGs in the lysosomes of cells. The MPS type IV (Morquio syndrome) occurs by the accumulation of keratan sulfate and condoitin-6-sulfate, caused by two different enzyme deficiencies, which distinguish this MPS into two subtypes, deficiency of N-acetylgalactosamine-6-sulfatase (MPS IV type A) or the enzyme β-galactosidase (MPS type IV B). In MPS IVA the predominant clinical features are related to the skeletal system and in most cases the patient has normal intelligence. There is no effective therapy for the disease, so early diagnosis directs for the beginning of palliative treatments. The initial investigation of this MPS is based on clinical evaluation, radiographs, quantification and identification of urinary GAGs and the definitive diagnosis by measure of N-acetylgalactosamine-6-sulfatase (GALNS) activity in leukocytes or fibroblasts. The aim of this work was to study some biochemical parameters of the GALNS in leukocytes of normal individuals and adapt the technique of measuring this enzyme activity to blood impregnated on filter paper. The Km and Vmax of GALNS in leukocytes from normal subjects was 7.16 mM and 77.3 nmol/17h/mg of protein, respectively. We also adapted the technique of measuring the enzymatic activity of this enzyme using lower substrate concentration (3mM). This concentration had a positive correlation with 10 mM concentration, which is used in the original technique. From this, the reference values for normal and individuals affected with MPS IVA in leukocytes were established. They were 11.0 to 101.4 and 0.1 to 0.2 of nmol/17h/mg of protein, respectively. It was also observed that over time the temperature of 70°C causes a gradual inactivation of enzyme activity. Finally, despite several changes to the original technique in leukocytes, it has not been possible to adapt it to be used in measuring the activity of GALNS blood impregnated on filter paper. The determination of biochemical parameters of GALNS is convenient due to the advancement in clinical studies for the development of enzyme replacement therapy for individuals affected with MPS IVA and to distinguish between healthy and affected heterozygous individuals. The development of fluorometric techniques in blood impregnated on filter paper is of great importance for use in newborn screening and in high risk populations to MPS. It should be confirmed by tests of enzyme activity in leukocytes or cultured fibroblasts. Unfortunately, from our protocols, it was not possible to develop and standardize a technique for measuring the activity of the enzyme N- acetylgalactosamine-6-sulfatase in blood impregnated on filter paper for to screen individuals suspected of MPS IVA.

A Mucopolissacaridose tipo IVA (MPS IVA), é uma doença lisossômica de depósito ocasionada pela degradação deficiente dos glicosaminoglicanos (GAG) queratan sulfato e condroitin-6-sulfato devido à deficiência da enzima N-acetilgalactosamina 6-sulfatase. Como a fisiopatologia desta doença ainda não está totalmente elucidada e muitos estudos vêm demonstrando o envolvimento do estresse oxidativo e inflamação na patogênese de outros tipos de mucopolissacaridoses, o objetivo principal deste trabalho foi investigar os parâmetros de estresse oxidativo e mediadores inflamatórios em pacientes MPS IVA sob terapia de reposição enzimática (TRE). Foram analisadas amostras de urina e sangue de pacientes MPS IVA sob TRE (n=17) e controles saudáveis pareados por idade (n=10-14). Os pacientes apresentaram diminuição significativa nos níveis de defesas antioxidantes, medida através dos níveis de glutationa reduzida (GSH), e aumento da enzima superóxido dismutase (SOD) em eritrócitos. Em relação ao dano a biomoléculas, foi observado nos pacientes um aumento de lipoperoxidação (aumento dos níveis de isoprostanos urinários) e de dano oxidativo a proteínas (aumento dos níveis urinários de di-tirosina e diminuição dos grupamentos sulfidrila no plasma) comparativamente aos controles. Nossos resultados também mostraram que os pacientes MPS IVA sob TRE apresentaram maior dano ao DNA, sendo este dano de origem oxidativa e atingindo bases purínicas e pirimidínicas. Além disso, os pacientes apresentaram níveis significativamente aumentados de interleucina-6 e esta, por sua vez, apresentou correlação negativa com GSH, mostrando uma possível relação entre inflamação e estresse oxidativo nesta doença. Considerando que os níveis de GAG urinários ainda se encontravam elevados nos pacientes em comparação com o grupo controle, pode-se supor que os mesmos estejam, pelo menos em parte, correlacionados com os danos oxidativos encontrados nestes pacientes. Os dados encontrados no presente trabalho sugerem que pacientes MPS IVA sob TRE apresentam uma condição pró-inflamatória e oxidativa e que a suplementação com antioxidantes em combinação com a TRE deve ser investigada com o intuito de melhorar a qualidade de vida dos pacientes. Cabe ressaltar que este é o primeiro estudo em pacientes que relaciona MPS IVA com estresse oxidativo e inflamação. / The Mucopolysaccharidosis type IVA (MPS IVA) is a lysosomal storage disease caused by impaired degradation of keratan sulfate and chondroitin-6-sulfate glycosaminoglycans (GAG), due to a deficiency on the enzyme N-acetylgalactosamine-6-sulfatase. Since the pathophysiology of this disease is still not totally elucidated and many studies demonstrated the involvement of oxidative stress and inflammation in the pathogenesis of other mucopolysaccharidoses types, the principal objective of this study was to investigate oxidative stress parameters and inflammatory mediators in MPS IVA patients under enzyme replacement therapy (ERT). Urine anda blood samples of MPS IVA patients under ERT (n= 17) and healthy age-matched controls (n= 10-14) were evaluated. Patients presented a significant decrease in antioxidant defenses levels, assessed by reduced glutathione (GSH), and increased superoxide dismutase (SOD) activity in erythrocytes. With regard to the biomolecules damage, was observed that patients presented lipid peroxidation (increase of isoprostanes urinary levels) and protein damage (increase of di-tyrosine urinary levels and decrease of sulfhydryl groups in plasma), when compared to controls. Our results showed higher DNA damage levels in MPS IVA patients compared to control group, in both pyrimidines and purines bases. The pro-inflammatory cytokine interleukin 6 (IL-6) was significantly increased in patients and showed an inverse correlation with GSH levels, showing a possible link between inflammation and oxidative stress in MPS IVA disease. Considering that GAG urinary level were still high in ERT patients compared to the control group, we propose that GAG are, at least in part, related with oxidative damage found in MPS IVA patients. The data presented suggest that pro-inflammatory and pro-oxidant states occur in MPS IVA patients under ERT, and the supplementation of antioxidants in combination with ERT can be investigated with the purpose of improving the patient’s life quality. To the best of our knowledge, this is the first study in patients relating MPS IVA with oxidative stress and inflammation.