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1.
Ann Cardiol Angeiol (Paris) ; 70(3): 171-176, 2021 Jun.
Artigo em Francês | MEDLINE | ID: mdl-33962784

RESUMO

Localized dissection of the descending thoracic aorta; class 3 of the classification proposed by the European Society of Cardiology; is a short and limited length dissection involving the descending thoracic aorta. It is a rare and unrecognized variant that poses a clinical and radiological diagnostic challenge. Indeed, it is manifested by an aspecific clinical profile represented by the acute aortic syndrome and characterized by a confused radiological aspect that entangled with the other differential diagnoses. We would like to emphasize the rarity of this uncommon pathology and the difficulty encountered in defining it based on a case treated in our department and a review of the literature.


Assuntos
Aneurisma da Aorta Torácica/diagnóstico por imagem , Dissecção Aórtica/diagnóstico por imagem , Doenças Raras/diagnóstico por imagem , Dor Aguda/etiologia , Idoso , Dissecção Aórtica/complicações , Dissecção Aórtica/cirurgia , Aneurisma da Aorta Torácica/complicações , Aneurisma da Aorta Torácica/cirurgia , Prótese Vascular , Dor no Peito/etiologia , Angiografia por Tomografia Computadorizada , Humanos , Masculino , Doenças Raras/complicações , Doenças Raras/cirurgia , Tomografia Computadorizada por Raios X
2.
Sci Total Environ ; 780: 146641, 2021 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-34030290

RESUMO

There are several ways for transmitting the SARS-CoV-2 virus to humans, and one of these ways is the contact with infected surfaces. Fuel stations have been a major concern for people and health experts due to the frequent use of common nozzles. This study was performed to identify the COVID-19 virus in the nozzles of fuel supply stations. In the current research, 25 fuel supply stations along the main street ways were investigated to recognize Coronavirus infection on the surfaces of the nozzles. For each fuel supply station, 4 nozzles were chosen (a total of 100 samples). The sampling was carried out at two periods of time, that is, before and after quarantine restrictions. Swapping was used for surface sampling, and Real-time PCR was used to determine the positive and negative results. The results showed that out of nine fuel supply stations (36 samples of nozzle surfaces), five were positive for the presence of SARS-CoV-2 on the nozzle surfaces before the corona restriction. The results showed that in the conditions after corona restriction, all samples were negative in terms of the presence of SARS-CoV-2 on the surfaces of the nozzles. In spite of the fact that gas stations can be one of the foremost inclined places for the transmission and spread of coronavirus due to the nearness and visit of individuals, but through the observance of health behaviors and implementing some procedures, cutting the transmission chain in gas stations can be facilitated.


Assuntos
COVID-19 , SARS-CoV-2 , Humanos , Irã (Geográfico) , Reação em Cadeia da Polimerase em Tempo Real
3.
Environ Res ; 196: 110948, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33684411

RESUMO

One of the simplest and most effective individual measures is to wear a mask to prevent the spread of respiratory droplets from carriers to healthy people and patients admitted to corona wards and their staff. This research aimed to investigate the contamination of internal and external surfaces of various masks used by patients and staff with SARS coronavirus, as well as the possibility of airborne transmission in Imam Khomeini Hospital, Ardabil. For this purpose, twenty-five staff members and ten patients participated voluntarily in this cross-sectional study. Sampling was performed using swaps on both sides (inside and outside) of various surgical masks, N-95, and filtering face piece FFP2 through standard methods in compliance with the relevant conditions and from a surface of at least 5 cm2. Next, the collected samples were immediately transferred to a laboratory and analyzed by real-time PCR method to detect the presence of SARS-CoV-2 virus after viral genome extraction. Based on the obtained results, from a total of 30 collected samples (25 of personnel masks plus 5 samples of hospitalized patients' masks). A total of 60 masks were sampled. For every collected sample, the researchers studied both inside and outside of the mask. Upon analyzing the data, it was showed that 6 mask samples were positive for the presence of coronavirus. Nonetheless, all samples taken from both inside and outside of the personnel masks (N-95 and FFP2 types of masks) were negative. Among the 6 positive samples, four cases were related to the internal part, one case to the outer part of the three-layer surgical masks, and one to the outer part of the N-95 masks in hospitalized patients. As masks reduce the concentration of virus particles, they can play an important role in creating immunity.


Assuntos
COVID-19 , SARS-CoV-2 , Estudos Transversais , Pessoal de Saúde , Hospitais , Humanos , Máscaras
4.
Environ Sci Pollut Res Int ; 28(30): 40771-40781, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33772474

RESUMO

Dust is an important factor to indoor pollution that contains suspended particles and heavy metals. Since today, use of computer has become an essential part of human life. Therefore, this study aimed to compare the amount of heavy metals deposited on CRTs and LCDs and their risk assessment. Ten dust samples for each type of monitor were collected from CRT and LCD screens in accordance with the standard OSHA ID-125 and ID-206, wet filter wipe method. Concentrations of Pb, Hg, Cd, Cr, and Cu were measured by inductively coupled plasma-optical emission spectrometer. Exposure and risk assessment of these elements were estimated using USEPA's exposure parameters. The average concentrations of heavy metals in the dust on CRTs (0.82 mg/kg) were higher than LCD screens (0.69 mg/kg). Cr with 62.824% and Hg with 0.849% had the highest and lowest average concentration in both types of monitors. The non-carcinogenic risk and carcinogenicity indexes of CRT monitors were 0.0009, and 3.94 × 10-11 and for LCD monitors were 0.0008 and 2.94 × 10-11. In both monitors, ingestion is the main route to exposure with heavy metals. The average concentration of heavy metals in CRT dust. HI values for studied metals in dust of CRTs and LCDs were less than safe limit of 1. Because RI < 10-4, cancer risk of studied elements in dust can be ignored.


Assuntos
Poeira , Metais Pesados , China , Cidades , Computadores , Poeira/análise , Monitoramento Ambiental , Humanos , Metais Pesados/análise , Medição de Risco
5.
Niger J Clin Pract ; 24(2): 193-198, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33605908

RESUMO

BACKGROUND: As enamel and finger buds have an ectodermal origin and grow at the same time, it might be supposed that factors and agents that affect dental caries might give rise to the diversity of dermatoglyphic dermal ridge patterns. AIM: The present study aimed to evaluate the relationship between dental caries and dermatoglyphics in 3 to 6-year-old Iranian children. METHODS: The present case-control study was performed on 3 to 6-year-old children. The decayed, missing, and filled teeth (dmft) scale was used to evaluate the oral and dental health status in the participants. Then, the patients were divided into two groups: 1) The case group: patients with a dmft of ≥3 (101 cases); 2) the control group: those with dmft value of zero (86 cases). Afterward, using a fingerprint recording device, the fingerprints were obtained from both hands of the participants. RESULTS: There was no significant relationship between the patterns of fingerprints and dental caries. The percentages of whorls, loops, and arches in the case group were higher than those in the control group; however, these differences were not statistically significant. CONCLUSION: Dermatoglyphic patterns could not be used as a variable for the detection or prediction of dental caries in Iranian children.


Assuntos
Cárie Dentária , Dermatoglifia , Estudos de Casos e Controles , Criança , Pré-Escolar , Índice CPO , Assistência Odontológica , Cárie Dentária/epidemiologia , Humanos , Irã (Geográfico)/epidemiologia
6.
Environ Res ; 195: 110765, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33497684

RESUMO

The prevalent respiratory viruses such as SARS-CoV-2 probably persist for a long time on fomites and environmental surfaces. Some recent studies have detected SARS-CoV-2 RNA on the surface of cell phones, door handles and other items in the inhabited sites of confirmed cases. For the aim of this study, a total of 50 environmental surface samples of SARS-CoV-2 was collected from Imam Khomeini Hospital in Ardabil. Forty-one environmental surface samples were proved negative for SARS-CoV-2 RNA while nine surface samples were positive. Our findings regarding surfaces contaminated with the virus are consistent with the results of recent similar researches as it was revealed that a number of different samples taken from hospital surfaces such as handles, cupboards, light switches, and door handles were positive for the presence of SARS-Cov-2.


Assuntos
COVID-19 , Coronavírus Relacionado à Síndrome Respiratória Aguda Grave , Fômites , Humanos , RNA Viral , SARS-CoV-2
7.
Water Sci Technol ; 82(11): 2415-2424, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33339795

RESUMO

The hematite (α-Fe2O3) nanostructures were synthesized by thermal oxidation of metal at 500 °C under atmospheric pressure. We studied the effect of the electrochemical pretreatment of the substrate before calcinations and its impact on the morphology, crystalline structure, lattice microstructural, and optical properties of α-Fe2O3. Uniform nanosheets were observed on the sample surface after calcination; their dimension and morphology were accentuated by the pretreatment, as confirmed by the SEM images. The characteristics of the nanostructures, analyzed by X-ray diffraction (XRD), revealed a rhombohedral symmetry with the space group R-3c and lattice constants: a = 0.5034 nm and c = 1.375 nm. The average crystallite size and strain, determined from the Williamson-Hall (W-H) plot, showed substantial variations after the substrate pretreatment. The Raman spectroscopy confirmed the changes in the crystal properties of the hematite submitted to pretreatment. The diffuse reflectance allowed to evaluate the optical gap which lies between 1.2 and 1.97 eV, induced by the electrochemical processing. The photocatalytic activity of α-Fe2O3 films was assessed by the degradation of methylene blue (MB) under LED light; 15% enhancement of the degradation for the pretreated specimens was noticed.


Assuntos
Luz , Azul de Metileno , Catálise , Oxirredução , Difração de Raios X
8.
Niger J Clin Pract ; 23(4): 545-549, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32246663

RESUMO

BACKGROUND: Handedness is one of the individual characteristics that causes different mental and practical capabilities including different abilities to perform oral hygiene instructions. It seems that the right-handed and left-handed individuals have different ability in brushing and removing plaque at different areas of the mouth. AIMS: To investigate the effect of handedness on decay pattern, gingival index, and plaque index in 6-10 years old children. SUBJECTS AND METHODS: In this cross-sectional study, children 6-10 years of age who fulfilled the inclusion criteria were selected. Dental caries and plaque and gingival indices were also measured and recorded for each child. Then the relationship between the handedness and the recorded variables was evaluated. Independent t-test and nonparametric Mann-Whitney test were used for the statistical comparison of quantitative variables between two right-handed and left-handed groups and Chi-square test was used for the statistical comparison of qualitative variables between these two groups. Data were analyzed using SPSS software version 20. RESULTS: The results showed that there was no significant relationship between any of the variables with being left- or right-handed. A significant relationship was observed only between the lowest plaque index and handedness (P < 0.05). CONCLUSION: A relationship cannot be considered between the handedness of children and their oral health status.


Assuntos
Cárie Dentária/epidemiologia , Lateralidade Funcional/fisiologia , Índice Periodontal , Criança , Estudos Transversais , Humanos
9.
Ann Cardiol Angeiol (Paris) ; 69(2): 93-95, 2020 Apr.
Artigo em Francês | MEDLINE | ID: mdl-32220415

RESUMO

The diversity of juvenile arterial disease aetiologies requires a systematic investigation when limb critical ischemia is diagnosed before the age of 50. We would like to share a rare case of a young woman who was diagnosed with Lupus disease revealed by distal ischemia of the foot.


Assuntos
Isquemia/etiologia , Extremidade Inferior/irrigação sanguínea , Lúpus Eritematoso Sistêmico/complicações , Adulto , Feminino , Humanos , Transtornos Relacionados ao Uso de Substâncias/complicações
10.
J Stomatol Oral Maxillofac Surg ; 121(2): 146-149, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31336213

RESUMO

Odontogenic lesions have been described in several syndromes. Despite multiple reports of ameloblastoma's association with various syndromes, it has not been added to the diagnostic criteria for any syndrome. Thus, the aim of this systematic review was gathering such cases' data. An electronic search in PubMed Central's database was performed. The search strategy was limited to human studies (case reports and case series), full-text English articles published from first of January 1975 until the end of 2018. Irrelevant articles or articles with inadequate information were omitted. Associated syndrome, patients' age and sex, lesions' location, radiographic features, and treatment modality were collected and analyzed. Ameloblastoma was reported in association with Gorlin syndrome (6 cases), epidermal nevus syndrome (2 cases), Gardner syndrome (2 cases), Simpson-Golabi-Behmel syndrome (1 case), and Williams syndrome (1 case). The commonest associated syndrome was Gorlin syndrome (50%) with maxillary and female predilections. Both cases of epidermal nevus syndrome (ENS) were female with mandibular involvement. In syndromes that odontogenic lesions are one of the diagnostic criteria, it is important to rule out ameloblastic changes. Although the relationship between ameloblastoma and these syndromes is not well known, any related information may be helpful in understanding the pathogenesis and the nature of this neoplasm.


Assuntos
Ameloblastoma , Síndrome do Nevo Basocelular , Síndrome de Gardner , Gigantismo , Feminino , Humanos , Mandíbula
11.
Niger J Clin Pract ; 22(10): 1328-1334, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31607720

RESUMO

OBJECTIVE: The aim of the this study was to evaluate the effect of honey on the healing of tooth extraction wounds in children 4‒9 years of age. SUBJECTS AND METHODS: In the present randomized clinical trial, 51 patients, 4‒9 years of age were selected randomly. All the subjects required extraction of one deciduous molar tooth. The subjects were randomly assigned to two groups. In group 1, after extraction of the tooth, the dentist used a cotton swab applicator to place a layer of honey on a piece of gauze moistened with normal saline solution (NSS) and placed it on the socket. In group 2, honey was not used; rather, NSS was applied. On days 3 and 7 after tooth extraction, the wound sizes were measured. RESULTS: In both groups, the wound sizes decreased significantly on the third day compared with baseline and on the seventh day compared with the third day (P < 0.05). On the third and seventh days after tooth extraction, wound sizes in the honey group were significantly lower than those in the NSS group (P < 0.05). CONCLUSION: Honey resulted in a decrease in wound sizes and faster healing after extraction of teeth in children. Therefore, use of honey can be recommended after minor surgeries in the oral cavity.


Assuntos
Apiterapia/métodos , Mel , Extração Dentária , Alvéolo Dental , Cicatrização/efeitos dos fármacos , Administração Tópica , Bandagens , Criança , Pré-Escolar , Assistência Odontológica , Feminino , Humanos , Masculino , Cloreto de Sódio/uso terapêutico , Alvéolo Dental/efeitos dos fármacos , Alvéolo Dental/patologia , Resultado do Tratamento
12.
Mater Sci Eng C Mater Biol Appl ; 103: 109843, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31349461

RESUMO

A series of germanium (Ge)-containing glasses were synthesized based on a starting glass composition of SiO2-ZnO-CaO-SrO-P2O5. Additions of GeO2 (6 and 12 mol%) were incorporated at the expense of SiO2, which retained the amorphous character, and each glass was processed to present similar particle size and surface area. Glass characterization using x-ray photoelectron spectroscopy (XPS) and magic angle spinning nuclear magnetic resonance (MAS-NMR) determined that the addition of GeO2 increased the fraction of lower Q-speciation and subsequently the concentration of non-bridging oxygens (NBO). Glass Polyalkenoate Cements (GPC) were formulated from each glass with 40, 50 and 60 wt% PAA, and presented time dependent solubility profiles (1, 10, 100, 1000 h) for the release of Si4+ (4-140 mg/l), Ca2+ (1-8 mg/l), Zn2+ (<6 mg/l), Sr2+ (2-37 mg/l), PO43- (2-43 mg/l) and Ge4+ (20-911 mg/l) and attained pH values close to 7.5 after 1000 h. Ge-GPCs containing 40 wt% polyacrylic acid (PAA) presented appropriate working time (Tw) and setting times (Ts), and the corresponding compressive strengths ranged from (14-30 MPa). The Ge-GPCs (40, 50 wt%) presented a linear increase (R2-0.99) with respect to time. Simulated Body Fluid (SBF) testing resulted in the Ge-GPCs encouraging the precipitation of crystalline hydroxyapatite on the GPC surface, more evidently after 100 and 1000 h incubation.


Assuntos
Líquidos Corporais/química , Germânio/química , Cimentos de Ionômeros de Vidro/química , Vidro/química , Humanos , Solubilidade
13.
Data Brief ; 21: 1008-1013, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30426059

RESUMO

For this data article the adsorption of Basic Blue 41 (BB 41) dye by activated carbon derived from filamentous algae (AAC) of available in agriculture waste as cheap adsorbents were examined. Activated carbon has been widely used as an adsorbent regard to its massive specific surface area, high porosity, reusability and thermal stability for the removal of pollutants from effluent. These filamentous algae grow widely in irrigation streams, causing decreasing speed of water flow and are not even eaten by livestock so are considered agricultural wastes. They can be used as precursors for activated carbon preparation and as adsorbent for the dye removal. The data of initial dye concentration (50-200 mg//L), pH of dye solution (3-9), adsorbent dosage (0.25-2 g/L), and contact time (5-200 min), were assessed. The structure of AAC was characterized by X-ray diffraction and Fourier transforms infrared spectroscopy. Activated carbon with a 94% removal of dye at concentration of 100 mg/L, pH 9, and adsorbent dose 1 g/L after 90 min. The data of isotherms and Kinetics indicated that the experimental data are fitted to Langmuir and second-pseudo-order models. Under the optimum conditions, maximum adsorption capacity of the AAC in Langmuir model enhanced to amount of 125 mg/g. According to the experimental data, filamentous algae are a suitable raw material for activated carbon production.

15.
Artigo em Inglês | MEDLINE | ID: mdl-27980792

RESUMO

BACKGROUND: Bisphenol A is a high production volume chemical widely used in manufacturing polycarbonate plastics and epoxy resins used in many industries. Due to its adverse effects on human health as an endocrine disruptor and many other effects on the various organs of the human body as well as aquatic organisms, it should be removed from the aquatic environments. This study aimed to mineralisation of BPA from aquatic environments by application of novel UV/SPS/H2O2/Cu system and optimization and modelling of its removal using central composite design (CCD) from response surface methodology (RSM). METHODS: CCD from RSM was used for modeling and optimization of operation parameters on the BPA degradation using UV/SPS/HP/Cu system. Effective operation parameters were initial persulfate, H2O2, Cu2+ and BPA concentration along with pH and reaction time, all in three levels were investigated. For analysis of obtained data ANOVA test was used. RESULTS: The results showed that a quadratic model is suitable to fit the experimental data (p < 0.0001). Analysis of response surface plots showed a considerable impact of all six selected variables which BPA and Cu2+ initial concentrations have been the highest and the least impact on the process, respectively. F-value of model was 54.74 that indicate significance of the model. The optimum values of the operation parameters were determined. The maximum removal of BPA was achieved 99.99 % in optimal conditions and in that condition TOC removal was about 70 %. Finally, validation and accuracy of the model were also evaluated by graphical residual analysis and the influential diagnostics plots. The higher relevance between actual and predicted values demonstrated the validation and applicability of the obtained equation as the model. CONCLUSIONS: According to the results, UV/SPS/HP/Cu system is an effective process in degradation and mineralisation of BPA and CCD methodology is a convenient and reliable statistical tool for optimizing BPA removal from aqueous solutions.

16.
J Med Life ; 8(Spec Iss 3): 178-183, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-28316687

RESUMO

Introduction: interpersonal communication skills are required for training and represent one of the most significant parts concerning the character of student learning. In another idea, learning is a constant method and learners favor a position of knowledge forms according to their character and individual practices. Evaluate the correlation between the learning methods and interpersonal conversation abilities of the nursing undergraduate in Medical Sciences Tehran University in 2012 was the purpose of this research. Methods: In this regular detailed cross-sectional analysis, 361 students from the School of Nursing and Midwifery were chosen during a census method. The information collection instruments were regulated, giving a questionnaire called Interpersonal Communication Skills Standards exam and VARK Learning Styles questionnaire. Data was examined by SPSS application (18th edition) by using Mann-Whitney and Kruskal-Wallis test. Results: 320 questionnaires were finished. 60.6% of the members were females. The average number of the students' conversation abilities level was 101.91 ± 10.35. More than half of the samples (58.8%) preferred multi-modal learning styles (Bi-Tri and Quad Modals) and 41.2% of the students preferred single modal learning styles. There were no significant differences between the Interpersonal Communication Skills and the learning styles (P= 0.46). Conclusion: According to no significant relationship between the communication skills of students with learning style and Demographic changeable and Lack of proper form of communication skills, we were ready to build different systems and courses related to improving the skills' level.

17.
J Med Life ; 8(Spec Iss 3): 90-95, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-28316672

RESUMO

Background: As the biggest collection among various collections of hospital employees, nurses perform a vital role in the progress of the hospital actions and the improvement of community health. Objectives: This research intended to find the relationship between the shortage of nursing workforce and hospitals performance of Tehran's Medical Sciences University. Materials and Methods: The current analysis was a cross-sectional definitive research, which was conducted in selected educational hospices associated to Tehran University of Medical Sciences during the year 2010. While using three researcher-made questionnaires, information was obtained from all clinical, paraclinical, commercial, managerial and support units of hospitals. Information was investigated according to the patterns of Iran's Healthcare Ministry and Medical Education by employing Excel software. Pabon Lasso model was used for performance measurement during the year 2010. The mixture of 3 indicators was applied to determine the period of stay, bed occupancy time and bed turnover. Results: The outcomes revealed that the personnel of nursing, in the 18 hospital wards (66.67%) were at levels below the standards, just one ward (3.7%) reached the standards requirements, and the remainder of the hospital wards (29.62%) were at greater levels than the standard requirements. Both hospitals were near the value 4 in the Pabon Lasso model. The correlation analysis among the nursing shortage and performance showed a meaningful relationship (P<0.05). Conclusion: Frequently, the examined hospices were challenged with a shortage of nursing workforce and the dissemination of workforce was not suitable. A decent supervision of workforce in conformity with the shortage of staff compensated and achieved the rules needed for the hospital's nursing workforce and this would guide to an improvement in the performance of the hospitals' actions and could present gratification for the personnel.

18.
Eur Arch Paediatr Dent ; 15(6): 401-5, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-24943132

RESUMO

AIM: One of the most important aspects of interceptive orthodontic diagnosis and treatment planning is space analysis. To date all methods use the size of permanent teeth to predict the dimensions of unerupted teeth. The aim of this study was to predict the permanent teeth size using maxillary primary first molar. METHODS: The size of primary maxillary first molars and permanent canines and premolars of 80 subjects was measured on their dental casts. Regression equations were determined between the size of primary maxillary first molars and permanent canines and premolars. RESULTS: The new regression equations for predicting permanent tooth size in the maxilla and mandible were, respectively, Y = 2.2X + 13 and Y = 2.4X + 9.5 among females and Y = 2.7X + 5.5 and Y = 2.4X + 9.5 among males. CONCLUSIONS: The results show that the primary maxillary first molar size can be used to predict the size of unerupted permanent teeth.


Assuntos
Dente Canino/anatomia & histologia , Dente Molar/anatomia & histologia , Odontometria/estatística & dados numéricos , Dente Decíduo/anatomia & histologia , Dente não Erupcionado/anatomia & histologia , Algoritmos , Dente Pré-Molar/anatomia & histologia , Calibragem , Estudos Transversais , Arco Dental/anatomia & histologia , Dentição Mista , Feminino , Previsões , Humanos , Masculino
20.
Rev Med Interne ; 31 Suppl 2: S279-91, 2010 Dec.
Artigo em Francês | MEDLINE | ID: mdl-21211680

RESUMO

Extraction and purification of an acid ß-glucosidase from human placenta (alglucerase) for the treatment of Gaucher disease, replaced a few years later by a recombinant enzyme (imiglucérase, Cerezyme(®)), has paved the way to the development of enzyme replacement therapies (ERT) for the treatment of lysosomal storage diseases (LSD) among which Fabry disease for which the long-term efficacy of the two currently available preparations (agalsidase alfa, Replagal(®) and Fabrazyme(®)) is still being investigated. Mucopolysaccharidosis (MPS) type I (Hurler and Scheie diseases), II (Hunter syndrome) and VI (Maroteaux-Lamy disease) also benefit from ERT using laronidase (Aldurazyme(®)), idursulfase (Elaprase(®)) and galsulfase (Naglazyme(®)), respectively. ERT reduces the hepatosplenomegaly and improves the physical and respiratory capacities of MPS patients with a globally acceptable safety profile although the possibility of infusion-associated should always be kept in mind. Alglucosidase alpha (Myozyme(®)) improves the cardiomyopathy and life expectancy of infants suffering from Pompe disease and is under evaluation for the treatment of the juvenile and adult forms of the disease. CNS involvement remains a major challenge for many LSD and innovative research and approaches are needed to address the fact that recombinant enzymes do not cross the blood-brain barrier and therefore are not expected to lead to any improvement in CNS damages, except if alternative routes such as intrathecal administration would be developed. Molecular chaperones (e.g. migalastat for Fabry disease) and inhibitors of glucosylceramide synthesis (e.g. eliglustat tartrate for Gaucher disease) are currently under investigation in various clinical trials.


Assuntos
Terapia de Reposição de Enzimas/métodos , Doença de Fabry/tratamento farmacológico , Hidrolases/uso terapêutico , Doenças por Armazenamento dos Lisossomos/tratamento farmacológico , alfa-Galactosidase/uso terapêutico , Humanos , Iduronato Sulfatase/uso terapêutico , Iduronidase/uso terapêutico , Mucopolissacaridose I/tratamento farmacológico , Mucopolissacaridose II/tratamento farmacológico , Mucopolissacaridose VI/tratamento farmacológico , N-Acetilgalactosamina-4-Sulfatase/uso terapêutico , Proteínas Recombinantes/uso terapêutico , Resultado do Tratamento , alfa-Glucosidases/uso terapêutico
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