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1.
Public Health ; 230: 163-171, 2024 May.
Article in English | MEDLINE | ID: mdl-38555685

ABSTRACT

OBJECTIVES: Immunisation against preventable diseases as meningitis is crucial from a public health perspective to face challenges posed by these infections. Nurses hold a great responsibility for these programs, which highlights the importance of understanding their preferences and needs to improve the success of campaigns. This study aimed to investigate nurses' preferences regarding Meningococcus A, C, W, and Y (MenACWY) conjugate vaccines commercialised in Spain. STUDY DESIGN: A national-level discrete choice experiment (DCE) was conducted. METHODS: A literature review and a focus group informed the DCE design. Six attributes were included: pharmaceutical form, coadministration evidence, shelf-life, package contents, single-doses per package, and package volume. Conditional logit models quantified preferences and relative importance (RI). RESULTS: Thirty experienced primary care nurses participated in this study. Evidence of coadministration with other vaccines was the most important attribute (RI = 43.78%), followed by package size (RI = 22.17%), pharmaceutical form (RI = 19.07%), and package content (RI = 11.80%). There was a preference for evidence of coadministration with routine vaccines (odds ratio [OR] = 2.579, 95% confidence interval [95%CI] = 2.210-3.002), smaller volumes (OR = 1.494, 95%CI = 1.264-1.767), liquid formulations (OR = 1.283, 95%CI = 1.108-1.486) and package contents including only vial/s (OR = 1.283, 95%CI = 1.108-1.486). No statistical evidence was found for the remaining attributes. CONCLUSIONS: Evidence of coadministration with routine vaccines, easy-to-store packages, and fully liquid formulations were drivers of nurses' preferences regarding MenACWY conjugate vaccines. These findings provide valuable insights for decision-makers to optimize current campaigns.


Subject(s)
Meningococcal Vaccines , Neisseria meningitidis , Nurses , Humans , Spain , Vaccines, Conjugate , Choice Behavior , Pharmaceutical Preparations
3.
BMC Infect Dis ; 23(1): 385, 2023 Jun 08.
Article in English | MEDLINE | ID: mdl-37291530

ABSTRACT

Respiratory syncytial virus (RSV) is a leading cause of acute lower respiratory infection (ALRI) in children, causing frequent outpatient visits and hospitalizations. Our study aimed to describe the clinical and direct economic burden of ALRI hospitalizations related to RSV in children in Spain and the characteristics of patients and their episodes. In this retrospective study, ALRI hospitalizations in children aged < 5 years for 2015-2018 were reviewed using anonymized administrative public hospital discharge data from Spain. Three case definitions were considered: (a) RSV-specific; (b) RSV-specific and unspecified acute bronchiolitis (RSV-specific and bronchiolitis); and (c) RSV-specific and unspecified ALRI (RSV-specific and ALRI). The study reported a mean of 36,743 yearly admissions potentially due to RSV, resulting in a mean annual cost of €87.1 million. RSV-specific codes accounted for 39.2% of cases, unspecified acute bronchiolitis for 20.1%, and other unspecified ALRI codes for the remaining 40.6%. The mean hospitalization rate per 1,000 children was 55.5 in the first year of life, 16.0 in the second, and 5.4 between 24 and 59 months. A considerable proportion of cases occurred in children under two years old (> 80.4%) and even during the first year of life (> 61.7%). Otherwise healthy children accounted for 92.9% of hospitalizations and 83.3% of costs during the period. Children born preterm accounted for 1.3% of hospitalizations and 5.7% of costs. The findings revealed that RSV still contributes to a high burden on the Spanish health care system. Children under one year of age and otherwise healthy term infants accounted for most of the substantial clinical and economic burden of RSV. Current evidence potentially underestimates the true epidemiology and burden of severe RSV infection; thus, further studies focusing on the outpatient setting are needed.


Subject(s)
Bronchiolitis , Respiratory Syncytial Virus Infections , Respiratory Syncytial Virus, Human , Respiratory Tract Infections , Infant, Newborn , Infant , Humans , Child , Retrospective Studies , Financial Stress , Hospitalization , Bronchiolitis/epidemiology , Hospitals, Public
4.
BMC Infect Dis ; 23(1): 86, 2023 Feb 07.
Article in English | MEDLINE | ID: mdl-36750925

ABSTRACT

BACKGROUND: Influenza may trigger complications, particularly in at-risk groups, potentially leading to hospitalization or death. However, due to lack of routine testing, influenza cases are infrequently coded with influenza-specific diagnosis. Statistical models using influenza activity as an explanatory variable can be used to estimate annual hospitalizations and deaths associated with influenza. Our study aimed to estimate the clinical and economic burden of severe influenza in Spain, considering such models. METHODS: The study comprised ten epidemic seasons (2008/2009-2017/2018) and used two approaches: (i) a direct method of estimating the seasonal influenza hospitalization, based on the number of National Health Service hospitalizations with influenza-specific International Classification of Diseases (ICD) codes (ICD-9: 487-488; ICD-10: J09-J11), as primary or secondary diagnosis; (ii) an indirect method of estimating excess hospitalizations and deaths using broader groups of ICD codes in time-series models, computed for six age groups and four groups of diagnoses: pneumonia or influenza (ICD-9: 480-488, 517.1; ICD-10: J09-J18), respiratory (ICD-9: 460-519; ICD-10: J00-J99), respiratory or cardiovascular (C&R, ICD-9: 390-459, 460-519; ICD-10: I00-I99, J00-J99), and all-cause. Means, excluding the H1N1pdm09 pandemic (2009/2010), are reported in this study. RESULTS: The mean number of hospitalizations with a diagnosis of influenza per season was 13,063, corresponding to 28.1 cases per 100,000 people. The mean direct annual cost of these hospitalizations was €45.7 million, of which 65.7% was generated by patients with comorbidities. Mean annual influenza-associated C&R hospitalizations were estimated at 34,894 (min: 16,546; max: 52,861), corresponding to 75.0 cases per 100,000 (95% confidence interval [CI]: 63.3-86.3) for all ages and 335.3 (95% CI: 293.2-377.5) in patients aged ≥ 65 years. We estimate 3.8 influenza-associated excess C&R hospitalizations for each hospitalization coded with an influenza-specific diagnosis in patients aged ≥ 65 years. The mean direct annual cost of the estimated excess C&R hospitalizations was €142.9 million for all ages and €115.9 million for patients aged ≥ 65 years. Mean annual influenza-associated all-cause mortality per 100,000 people was estimated at 27.7 for all ages. CONCLUSIONS: Results suggest a relevant under-detected burden of influenza mostly in the elderly population, but not neglectable in younger people.


Subject(s)
Influenza, Human , Aged , Humans , Seasons , Influenza, Human/epidemiology , Spain , State Medicine , Hospitalization , Pandemics
5.
Rehabilitación (Madr., Ed. impr.) ; 57(1): 100732-100732, Ene-Mar. 2023. tab, graf
Article in Spanish | IBECS | ID: ibc-214200

ABSTRACT

Introducción: La actividad física es esencial en la población con artrosis, sin embargo, el confinamiento durante la pandemia por COVID obligó al cambio en los estilos de vida. Se llevó a cabo un estudio observacional y descriptivo con el objetivo de evaluar el ejercicio físico realizado en personas con patología osteoarticular degenerativa (POD) durante la pandemia por COVID-19. Material y métodos: Se realizó una encuesta telefónica a personas mayores de 60 años con POD atendidos previamente en el Hospital Central de la Cruz Roja, valorando el ejercicio físico durante el confinamiento por pandemia COVID-19. Se analizaron las variables (tiempo de ejercicio físico, causas si el ejercicio fue reducido, y la intensidad de dolor) y se compararon con la situación previa al confinamiento domiciliario. Resultados: Se incluyeron un total de 33 pacientes (ocho hombres, 25 mujeres), con una edad media de 75,6 años. La poliartrosis fue el diagnóstico más frecuente. El 51,5% realizaban la misma cantidad de ejercicio físico, el 21,21% realizaban más y 24,2% menos que antes. Solo el 6,1% realizaba más de una hora al día de actividad. El 36,4% tenían más dolor que previamente. Conclusiones: Aunque existe una alta adherencia a los ejercicios aprendidos en el servicio de rehabilitación, el confinamiento por COVID-19 ha tenido un efecto negativo en el nivel de ejercicio realizado por los pacientes con POD. Es recomendable incentivar el ejercicio físico durante períodos de confinamiento.(AU)


Introduction: Physical activity is essential in the osteoarthritis population, however, confinement during the COVID pandemic forced lifestyle changes. An observational and descriptive study was conducted to assess physical exercise in people with degenerative osteoarticular disease (DOD) during the COVID-19 pandemic. Material and methods: A telephone survey was conducted among people over 60 years of age with DOD previously treated at the Hospital Central de la Cruz Roja, assessing physical exercise during the COVID-19 pandemic confinement. The variables (time of physical exercise, causes if exercise was reduced, and pain intensity) were analysed and compared with the situation prior to home confinement. Results: A total of 33 patients (8 men, 25 women) were included, with a mean age of 75.6 years. Polyarthrosis was the most frequent diagnosis. 51.5% performed the same amount of physical exercise, 21.21% performed more and 24.2% less than before. Only 6.1% performed more than 1 h a day of activity. 36.4% had more pain than previously. Conclusions: Although there is a high adherence to the exercises learned in the rehabilitation service, COVID-19 confinement has had a negative effect on the level of exercise performed by POD patients. It is advisable to encourage physical exercise during periods of lockdown.(AU)


Subject(s)
Humans , Male , Female , Middle Aged , Aged , Aged, 80 and over , Controlled Confinement , Exercise , Joint Diseases , Pandemics , Coronavirus Infections , Severe acute respiratory syndrome-related coronavirus , Pain Measurement , Rehabilitation , Spain , Surveys and Questionnaires , Epidemiology, Descriptive , Cross-Sectional Studies
6.
Rehabilitacion (Madr) ; 57(1): 100732, 2023.
Article in Spanish | MEDLINE | ID: mdl-35545482

ABSTRACT

INTRODUCTION: Physical activity is essential in the osteoarthritis population, however, confinement during the COVID pandemic forced lifestyle changes. An observational and descriptive study was conducted to assess physical exercise in people with degenerative osteoarticular disease (DOD) during the COVID-19 pandemic. MATERIAL AND METHODS: A telephone survey was conducted among people over 60 years of age with DOD previously treated at the Hospital Central de la Cruz Roja, assessing physical exercise during the COVID-19 pandemic confinement. The variables (time of physical exercise, causes if exercise was reduced, and pain intensity) were analysed and compared with the situation prior to home confinement. RESULTS: A total of 33 patients (8 men, 25 women) were included, with a mean age of 75.6 years. Polyarthrosis was the most frequent diagnosis. 51.5% performed the same amount of physical exercise, 21.21% performed more and 24.2% less than before. Only 6.1% performed more than 1 h a day of activity. 36.4% had more pain than previously. CONCLUSIONS: Although there is a high adherence to the exercises learned in the rehabilitation service, COVID-19 confinement has had a negative effect on the level of exercise performed by POD patients. It is advisable to encourage physical exercise during periods of lockdown.


Subject(s)
COVID-19 , Osteoarthritis , Aged , Female , Humans , Male , Middle Aged , Communicable Disease Control , Exercise , Pandemics , SARS-CoV-2
7.
BMC Infect Dis ; 22(1): 759, 2022 Sep 29.
Article in English | MEDLINE | ID: mdl-36175846

ABSTRACT

Respiratory syncytial virus (RSV) infection is a major cause of morbidity in children. However, its disease burden remains poorly understood, particularly outside of the hospital setting. Our study aimed to estimate the burden of medically attended acute lower respiratory infection (ALRI) cases potentially related to RSV in Spanish children. Longitudinal data from September 2017 to June 2018 of 51,292 children aged < 5 years old from the National Healthcare System (NHS) of two Spanish regions were used. Three case definitions were considered: (a) RSV-specific; (b) RSV-specific and unspecified acute bronchiolitis (RSV-specific and Bronchiolitis), and; (c) RSV-specific and unspecified ALRI (RSV-specific and ALRI). A total of 3460 medically attended ALRI cases potentially due to RSV were identified, of which 257 (7.4%), 164 (4.7%), and 3039 (87.8%) coded with RSV-specific, unspecific bronchiolitis, and unspecific ALRI codes, respectively. Medically attended RSV-specific and ALRI cases per 1000 children was 134.4 in the first year of life, 119.4 in the second, and 35.3 between 2 and 5 years old. Most cases were observed in otherwise healthy children (93.1%). Mean direct healthcare cost per medically attended RSV-specific and ALRI case was €1753 in the first year of life, €896 in the second, and €683 between 2 and 5 years old. Hospitalization was the main driver of these costs, accounting for 55.6%, 38.0% and 33.4%, in each respective age group. In RSV-specific cases, mean direct healthcare cost per medically attended case was higher, mostly due to hospitalization: €3362 in the first year of life (72.9% from hospitalizations), €3252 in the second (72.1%), and €3514 between 2 and 5 years old (74.2%). These findings suggest that hospitalization data alone will underestimate the RSV infections requiring medical care, as will relying only on RSV-specific codes. RSV testing and codification must be improved and preventive solutions adopted, to protect all infants, particularly during the first year of life.


Subject(s)
Respiratory Syncytial Virus Infections , Respiratory Syncytial Virus, Human , Respiratory Tract Infections , Child , Child, Preschool , Humans , Infant , Financial Stress , Hospitalization , Respiratory Syncytial Virus Infections/epidemiology , Respiratory Tract Infections/epidemiology , Spain
8.
Rev. esp. anestesiol. reanim ; 69(7): 433-436, Ago.- Sep. 2022.
Article in Spanish | IBECS | ID: ibc-207289

ABSTRACT

El estudio de la técnica anestésica libre de opioides aporta evidencias de su efectividad y seguridad. Sin embargo, aún no están bien definidos todos sus riesgos y beneficios, ni en qué pacientes o intervenciones puede ser superior a la técnica anestésica convencional basada en opioides. Las cirugías intensivas y/o duraderas plantean dudas para la utilización de esta técnica por la respuesta a cambios hemodinámicos bruscos, al no producir la simpaticolisis a través de la actuación sobre el receptor μ y haber poca experiencia de uso. Una paciente con obesidad mórbida fue sometida a cistectomía radical con derivación urinaria tipo Bricker mediante laparotomía infraumbilical, consiguiéndose una adecuada estabilidad hemodinámica y una analgesia óptima en el postoperatorio sin emplear opioides intraoperatorios. La anestesia libre de opioides está en expansión con una evidencia creciente. No obstante, es necesario seguir investigando sobre sus posibilidades de utilización, las distintas combinaciones de fármacos que se puedan emplear y la resolución de complicaciones que puedan ocurrir.(AU)


Opioid-free anaesthesia shows evidence about its efectivity and security, even though its risks and benefits are not well defined. Neither are the patient profile or sort of surgery where it could be superior to the conventional opioid-based anaesthetic technique. Aggressive and/or long-lasting surgeries set out several queries on this technique regarding sudden hemodynamic changes, as it does not produce sympatholysis through μ receptor and there is modest experience in this technique. A morbidly obese patient received open radical cystectomy with Bricker-type urinary diversion using infraumbilical incision under OFA protocol, maintaining an adequate hemodynamic stability and excellent analgesia in postoperatory care without using any intraoperative opioids. Opioid-free anaesthesia technique is developing its evidence. However, it is necessary to keep on researching its clinical applications, different drug combinations and solutions to its expected complications.(AU)


Subject(s)
Humans , Female , Middle Aged , Anesthesia/methods , Analgesics, Non-Narcotic/administration & dosage , Analgesics, Non-Narcotic/pharmacology , Analgesics, Non-Narcotic/therapeutic use , Cystectomy , Obesity, Morbid/complications , Laparotomy , Pain Management , Anesthetics , Anesthesiology , General Surgery , Neoplasms , Drug Combinations
9.
Article in English | MEDLINE | ID: mdl-35869004

ABSTRACT

Opioid-free anaesthesia shows evidence about its efectivity and security, even though its risks and benefits are not well defined. Neither are the patient profile or sort of surgery where it could be superior to the conventional opioid-based anaesthetic technique. Aggressive and/or long-lasting surgeries set out several queries on this technique regarding sudden hemodynamic changes, as it does not produce sympatholysis through µ receptor and there is modest experience in this technique. A morbidly obese patient received open radical cystectomy with Bricker-type urinary diversion using infraumbilical incision under OFA protocol, maintaining an adequate hemodynamic stability and excellent analgesia in postoperatory care without using any intraoperative opioids. Opioid-free anaesthesia technique is developing its evidence. However, it is necessary to keep on researching its clinical applications, different drug combinations and solutions to its expected complications.


Subject(s)
Anesthesia , Obesity, Morbid , Urinary Diversion , Analgesics, Opioid , Cystectomy/methods , Humans , Obesity, Morbid/surgery , Urinary Diversion/methods
10.
Int J Biometeorol ; 66(6): 1173-1187, 2022 Jun.
Article in English | MEDLINE | ID: mdl-35275236

ABSTRACT

Precipitation is one of the meteorological variables usually involved in the aerobiological studies, which presents a complex relationship with atmospheric levels of pollen and fungal spores and the temporal characteristics of their seasons. This complexity is due in a large part to rainfall's twofold impact of having, prior to pollination, a positive influence on subsequent pollen production and of contributing, during pollination, to pollen removal from the air through a wash-out effect. To better explore this impact, we place particular emphasis on extreme rainfall by calculating the correlation between airborne pollen and fungal spore parameters and the precipitation indices that the Expert Team on Climate Change Detection and Indices (ETCCDI) proposed for characterising climate extremes. Parameters for twenty-seven pollen and fungal spore taxa measured in six aerobiological stations in the NE Iberian Peninsula have been considered. We have distinguished between annual and winter ETCCDI in order to compare the correlations between extreme rainfall and airborne pollen concentrations and to avoid the wash-out effect as far as possible. Results show a positive influence from an increase in moderately extreme winter rainfall, specifically on subsequent pollen/fungal spore production: the percentage of all possible significant correlations is higher for winter than for annual rainfall. Furthermore, while annual rainfall in this region has nearly the same number of positive as negative correlations, the positive correlations for winter rainfall are more than twice that of the negative ones. The seasonal consideration on rainfall ETCCDI made with the aim to avoid the confounding overlapping of different rainfall impacts has led to more sharpened observations of its positive and negative effects on airborne pollen and fungal spore concentrations.


Subject(s)
Air Pollutants , Allergens , Air Pollutants/analysis , Allergens/analysis , Environmental Monitoring , Meteorology , Pollen , Seasons , Spores, Fungal
13.
An. sist. sanit. Navar ; 44(3): 457-462, Dic 27, 2021. ilus
Article in Spanish | IBECS | ID: ibc-217318

ABSTRACT

Los pacientes con lesión medular crónica tienenunas alteraciones fisiopatológicas que determinan unaimportante morbilidad y mortalidad en el periodo perioperatorio. El marcapasos diafragmático es un dispositivo que permite la ventilación pulmonar en pacientescon lesiones cervicales altas y mejora la calidad de vidafrente al uso de ventilación mecánica. Presentamos el caso de una paciente con lesiónmedular crónica y portadora de un marcapasos diafragmático que fue programada para realización de nefrolitotomía percutánea y colocación de catéter doble Jderecho. El anestesista debe conocer la situación fisiopatológica de estos pacientes para asegurar la seguridad en el proceso perioperatorio.(AU)


Patients with chronic spinal cord injury suffer froma number of pathophysiological alterations that canlead to important morbidity and mortality in the perioperative period. The diaphragmatic pacemaker is adevice that enables pulmonary ventilation in patientswith high cervical cord injuries and provides them witha better quality of life when compared to mechanicalventilation. We present here the clinical case of a patient withchronic spinal cord injury who used a diaphragmaticpacemaker, and who was scheduled for percutaneousnephrolithotomy and double-J stent implantation. Theanesthesiologist should know the pathophysiologicalsituation of these patients in order to provide a safeperioperatory care.(AU)


Subject(s)
Humans , Female , Adult , Perioperative Period , Spinal Cord Injuries , Pacemaker, Artificial , Inpatients , Physical Examination , Phrenic Nerve , Respiration, Artificial , Neck Injuries
14.
Article in English, Spanish | MEDLINE | ID: mdl-34565571

ABSTRACT

Opioid-free anaesthesia shows evidence about its efectivity and security, even though its risks and benefits are not well defined. Neither are the patient profile or sort of surgery where it could be superior to the conventional opioid-based anaesthetic technique. Aggressive and/or long-lasting surgeries set out several queries on this technique regarding sudden hemodynamic changes, as it does not produce sympatholysis through µ receptor and there is modest experience in this technique. A morbidly obese patient received open radical cystectomy with Bricker-type urinary diversion using infraumbilical incision under OFA protocol, maintaining an adequate hemodynamic stability and excellent analgesia in postoperatory care without using any intraoperative opioids. Opioid-free anaesthesia technique is developing its evidence. However, it is necessary to keep on researching its clinical applications, different drug combinations and solutions to its expected complications.

15.
An Sist Sanit Navar ; 44(3): 457-462, 2021 Dec 27.
Article in Spanish | MEDLINE | ID: mdl-34132246

ABSTRACT

Patients with chronic spinal cord injury suffer from a number of pathophysiological alterations that can lead to important morbidity and mortality in the perioperative period. The diaphragmatic pacemaker is a device that enables pulmonary ventilation in patients with high cervical cord injuries and provides them with a better quality of life when compared to mechanical ventilation. We present here the clinical case of a patient with chronic spinal cord injury who used a diaphragmatic pacemaker, and who was scheduled for percutaneous nephrolithotomy and double-J stent implantation. The anesthesiologist should know the pathophysiological situation of these patients in order to provide a safe perioperatory care.


Subject(s)
Pacemaker, Artificial , Spinal Cord Injuries , Diaphragm , Humans , Quality of Life , Respiration, Artificial , Spinal Cord Injuries/complications , Spinal Cord Injuries/therapy
17.
Rev. esp. anestesiol. reanim ; 68(3): 165-170, Mar. 2021.
Article in Spanish | IBECS | ID: ibc-231013

ABSTRACT

Introducción: La narcolepsia es el segundo trastorno del sueño en frecuencia y se caracteriza por somnolencia excesiva durante el día junto con otros síntomas como cataplejía, parálisis del sueño y alucinaciones. Su fisiopatología y tratamiento, así como los síndromes que asocia, pueden interferir de forma severa con el acto anestésico. Metodología: Debido al déficit de evidencia de calidad que aporte un grado de recomendación alto en la anestesia de estos pacientes, se realizó una revisión narrativa de la literatura no sistemática en Pubmed. Como descriptores se usaron narcolepsy, anesthesia, surgery, perioperative, opioid, obstructive, apnea y sleep y se emplearon de forma individual y cruzándolos con conectores AND y OR. Conclusión: La recomendación de evitar opioides y la estabilidad de la anestesia libre de opioides (OFA), hace de esta última una opción para estos pacientes. Se describe un caso en el que se empleó de forma segura.(AU)


Introduction: Narcolepsy is the second most common sleep disorder. It is characterised by excessive daytime sleepiness together with other symptoms such as cataplexy, sleep paralysis, and hallucinations. The pathophysiology and treatment of this disease, together with its associated syndromes, can severely interfere with anaesthesia. Methodology: Due to the lack of quality evidence on which to base a high grade of recommendation for anaesthesia in these patients, we performed a non-systematic, narrative review of the literature in Pubmed. We used the descriptors narcolepsy, anesthesia, surgery, perioperative, opioid, obstructive, apnea and sleep both individually and with AND and OR connectors. Conclusion: The recommendation to avoid opioids and the stability of opioid-free anaesthesia (OFA) make this approach an option to consider in these patients. We describe a case in which it was used safely.(AU)


Subject(s)
Humans , Male , Female , Anesthesia , Narcolepsy/diagnosis , Analgesics, Opioid/adverse effects , Sleep Paralysis , Cataplexy/diagnosis
18.
Rev Esp Anestesiol Reanim (Engl Ed) ; 68(3): 165-170, 2021 Mar.
Article in English, Spanish | MEDLINE | ID: mdl-33160690

ABSTRACT

INTRODUCTION: Narcolepsy is the second most common sleep disorder. It is characterised by excessive daytime sleepiness together with other symptoms such as cataplexy, sleep paralysis, and hallucinations. The pathophysiology and treatment of this disease, together with its associated syndromes, can severely interfere with anaesthesia. METHODOLOGY: Due to the lack of quality evidence on which to base a high grade of recommendation for anaesthesia in these patients, we performed a non-systematic, narrative review of the literature in Pubmed. We used the descriptors narcolepsy, anesthesia, surgery, perioperative, opioid, obstructive, apnea and sleep both individually and with AND and OR connectors. CONCLUSION: The recommendation to avoid opioids and the stability of opioid-free anaesthesia (OFA) make this approach an option to consider in these patients. We describe a case in which it was used safely.


Subject(s)
Anesthesia , Cataplexy , Narcolepsy , Sleep Paralysis , Analgesics, Opioid/adverse effects , Cataplexy/diagnosis , Humans , Narcolepsy/diagnosis
19.
Stem Cell Res Ther ; 11(1): 453, 2020 10 27.
Article in English | MEDLINE | ID: mdl-33109263

ABSTRACT

Primary immunodeficiency diseases (PIDs) are rare diseases that are characterized by genetic mutations that damage immunological function, defense, or both. Some of these rare diseases are caused by aberrations in the normal development of natural killer cells (NKs) or affect their lytic synapse. The pathogenesis of these types of diseases as well as the processes underlying target recognition by human NK cells is not well understood. Utilizing induced pluripotent stem cells (iPSCs) will aid in the study of human disorders, especially in the PIDs with defects in NK cells for PID disease modeling. This, together with genome editing technology, makes it possible for us to facilitate the discovery of future therapeutics and/or cell therapy treatments for these patients, because, to date, the only curative treatment available in the most severe cases is hematopoietic stem cell transplantation (HSCT). Recent progress in gene editing technology using CRISPR/Cas9 has significantly increased our capability to precisely modify target sites in the human genome. Among the many tools available for us to study human PIDs, disease- and patient-specific iPSCs together with gene editing offer unique and exceptional methodologies to gain deeper and more thorough understanding of these diseases as well as develop possible alternative treatment strategies. In this review, we will discuss some immunodeficiency disorders affecting NK cell function, such as classical NK deficiencies (CNKD), functional NK deficiencies (FNKD), and PIDs with involving NK cells as well as strategies to model and correct these diseases for further study and possible avenues for future therapies.


Subject(s)
Induced Pluripotent Stem Cells , Primary Immunodeficiency Diseases , Gene Editing , Humans , Killer Cells, Natural , Stem Cell Transplantation
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