Isoinmunización Rh en bajas respondedoras: Reporte de caso / Rh Isoimmunization in Low Responders: Case Report / Isoimunização Rh em pacientes com baixa resposta: relato de caso

Introducción. La isoinmunización Rh consiste en la producción de anticuerpos maternos en una gestante Rh negativa contra los antígenos de los eritrocitos Rh positivos fetales ocasionados por una hemorragia fetomaterna. En población gestante, el 15% son Rh negativo y la severidad de la afectación fetal está relacionada con una serie de procesos inmunológicos y la historia obstétrica. Si una gestante Rh negativa con riesgo de isoinmunización no recibe profilaxis con inmunoglobulina Anti-D se inmuniza el 16% en la primera gestación, el 30% en la segunda y el 50% después de la tercera. Con este reporte de caso queremos describir el subgrupo de pacientes gestantes con isoinmunización Rh bajas respondedoras. Presentación del caso. G9P5C1A2Gem1V7 de 43 años, remitida en semana 30 de gestación por isoinmunización Rh, no recibió inmunoglobulina Anti-D durante este embarazo, ni en los anteriores ni en el posparto, reporte de Coombs indirecto de 1/4 que se eleva a 1/16, seguimiento ecográfico normal. En semana 35.3 presenta anemia fetal leve y por tratarse de un embarazo alrededor del término se finaliza por cesárea. Recién nacido con adecuado peso para la edad gestacional, quien fue dado de alta a las 72 horas con evolución satisfactoria. Discusión. Las gestantes con isoinmunización Rh bajas respondedoras se sensibilizan con altos volúmenes sanguíneos sin repercusión hemodinámica in utero, produciendo una enfermedad hemolítica fetal leve. Esta respuesta inmune es poco frecuente y está asociada a factores protectores; sin embargo, son necesarios más estudios que sustenten esta condición. Conclusiones. El control prenatal y el Coombs indirecto cuantitativo seriado son las principales herramientas para la prevención de la isoinmunización. El conocimiento de la respuesta inmunológica permite identificar el subgrupo de las bajas respondedoras que tienen una evolución clínica más leve y menor morbilidad neonatal. Palabras clave: Embarazo; Isoinmunización Rh; Eritroblastosis Fetal; Globulina Inmune RHO(D); Hidropesía Fetal.

Introduction. Rh isoimmunization consists of a Rh-negative pregnant woman producing maternal antibodies against the antigens of fetal Rh-positive erythrocytes due to fetomaternal hemorrhage. 15% of the pregnant population is Rh negative, and the severity of fetal effects is related to a series of immunological processes and the obstetric history. If a Rh-negative pregnant woman at risk of isoimmunization does not receive a prophylaxis of Anti-D immunolobulin, 16% are immunized in the first pregnancy, 30% in the second and 50% after the third. In this case report we will describe the subgroup of low responder pregnant patients with Rh isoimmunization. Case Presentation. G9P5C1A2Gem1V7, 43 years old, referred on the 30th week of pregnancy due to Rh isoimmunization. She did not receive Anti-D immunolobulin during this pregnancy, nor in her previous pregnancies, nor during postpartum. Indirect Coombs report of 1/4, which increases to 1/16. Ultrasound monitoring is normal. At week 35.3 she presented mild fetal anemia, and because the pregnancy was near its term, it was ended by cesarean section. Newborn with adequate weight considering the gestational age, who was then discharged after 72 hours with satisfactory evolution. Discussion. Low responder pregnant women with Rh isoimmunization are sensitized with high blood volumes but without hemodynamic repercussions in utero, producing a mild fetal hemolytic disease. This immune response is infrequent and is associated with protective factors; however, further studies are required to support this condition. Conclusions. Prenatal control and serialized quantitative indirect Coombs testing are the main tools for the prevention of isoimmunization. Knowledge of the immunological response enables identifying the subgroup of low responders who present a milder clinical evolution and lower newborn morbidity. Keywords: Pregnancy; Rh Isoimmunization; Erythroblastosis, Fetal; RHO(D) Immune Globulin; Hydrops Fetalis.

Introdução. A isoimunização Rh consiste na produção de anticorpos maternos em uma gestante Rh negativa contra os antígenos dos eritrócitos fetais Rh positivos causados por hemorragia fetomaterna. Na população gestante, 15% são Rh negativos e a gravidade do envolvimento fetal está relacionada a uma série de processos imunológicos e ao histórico obstétrico. Se uma gestante Rh negativa com risco de isoimunização não receber profilaxia com imunoglobulina Anti-D, imuniza-se 16% na primeira gestação, 30% na segunda e 50% após a terceira. Com este relato de caso, queremos descrever o subgrupo de pacientes gestantes com isoimunização Rh de baixa resposta. Apresentação do caso. G9P5C1A2Gem1V7, 43 anos, encaminhada na 30ª semana de gestação para isoimunização Rh, não recebeu imunoglobulina Anti-D nesta gestação, nem nas anteriores nem no puerpério, laudo de Coombs indireto de 1/4 que sobe para 1/16, acompanhamento ultrassonográfico normal. Na semana 35,3, apresentou anemia fetal leve e por se tratar de uma gestação próxima ao termo, foi interrompida por cesariana. Recém-nascido com peso adequado para a idade gestacional, que recebeu alta às 72 horas com evolução satisfatória. Discussão. Gestantes com isoimunização Rh de baixa resposta são sensibilizadas com elevados volumes sanguíneos sem repercussões hemodinâmicas in utero, produzindo doença hemolítica fetal leve. Essa resposta imune é rara e está associada a fatores protetores; no entanto, mais estudos são necessários para fundamentar esta condição. Conclusões. O controle pré-natal e o Coombs indireto quantitativo seriado são as principais ferramentas para a prevenção da isoimunização. O conhecimento da resposta imunológica permite identificar o subgrupo de pacientes com baixa resposta que apresentam evolução clínica mais branda e menor morbidade neonatal. Palavras-chave: Gravidez; Isoimunização Rh; Eritroblastose Fetal; Inmunoglobulina RHO (D), Hidropisia Fetal.

Symptom cluster analysis of long COVID-19 in patients discharged from the Temporary COVID-19 Hospital in Mexico City.

INTRODUCTION: Several reports have emerged describing the long-term consequences of COVID-19 and its effects on multiple systems. METHODS: As further research is needed, we conducted a longitudinal observational study to report the prevalence and associated risk factors of the long-term health consequences of COVID-19 by symptom clusters in patients discharged from the Temporary COVID-19 Hospital (TCH) in Mexico City. Self-reported clinical symptom data were collected via telephone calls over 90 days post-discharge. Among 4670 patients, we identified 45 symptoms across eight symptom clusters (neurological; mood disorders; systemic; respiratory; musculoskeletal; ear, nose, and throat; dermatological; and gastrointestinal). RESULTS: We observed that the neurological, dermatological, and mood disorder symptom clusters persisted in >30% of patients at 90 days post-discharge. Although most symptoms decreased in frequency between day 30 and 90, alopecia and the dermatological symptom cluster significantly increased (p < 0.00001). Women were more prone than men to develop long-term symptoms, and invasive mechanical ventilation also increased the frequency of symptoms at 30 days post-discharge. CONCLUSION: Overall, we observed that symptoms often persisted regardless of disease severity. We hope these findings will help promote public health strategies that ensure equity in the access to solutions focused on the long-term consequences of COVID-19.

High-flow nasal cannula therapy for hypoxemic respiratory failure in patients with COVID-19.

INTRODUCTION: High-flow nasal cannula (HFNC) therapy in patients with hypoxemic respiratory failure due to COVID-19 is poorly understood and remains controversial. METHODS: We evaluated a large cohort of patients with COVID-19-related hypoxemic respiratory failure at the temporary COVID-19 hospital in Mexico City. The primary outcome was the success rate of HFNC to prevent the progression to invasive mechanical ventilation (IMV). We also evaluated the risk factors associated with HFNC success or failure. RESULTS: HFNC use effectively prevented IMV in 71.4% of patients [270 of 378 patients; 95% confidence interval (CI) 66.6-75.8%]. Factors that were significantly different at admission included age, the presence of hypertension, and the Charlson comorbidity index. Predictors of therapy failure (adjusted hazard ratio, 95% CI) included the comorbidity-age-lymphocyte count-lactate dehydrogenase (CALL) score at admission (1.27, 1.09-1.47; p < 0.01), Rox index at 1 hour (0.82, 0.7-0.96; p = 0.02), and no prior steroid treatment (0.34, 95% CI 0.19-0.62; p < 0.0001). Patients with HFNC success rarely required admission to the intensive care unit and had shorter lengths of hospital stay [19/270 (7.0%) and 15.0 (interquartile range, 11-20) days, respectively] than those who required IMV [104/108 (96.3%) and 26.5 (20-36) days, respectively]. CONCLUSION: Treating patients with HFNC at admission led to improvement in respiratory parameters in many patients with COVID-19.

Impact of preemptive hospitalization on health outcomes at the temporary COVID-19 hospital in Mexico City: a prospective observational study.

INTRODUCTION: In response to the evolution of the coronavirus disease 2019 (COVID-19) pandemic, the admission protocol for the temporary COVID-19 hospital in Mexico City has been updated to hospitalize patients preemptively with an oxygen saturation (SpO2) of >90%. METHODS: This prospective, observational, single-center study compared the progression and outcomes of patients who were preemptively hospitalized versus those who were hospitalized based on an SpO2 ⩽90%. We recorded patient demographics, clinical characteristics, COVID-19 symptoms, and oxygen requirement at admission. We calculated the risk of disease progression and the benefit of preemptive hospitalization, stratified by CALL Score: age, lymphocyte count, and lactate dehydrogenase (<8 and ⩾8) at admission. RESULTS: Preemptive hospitalization significantly reduced the requirement for oxygen therapy (odds ratio 0.45, 95% confidence interval 0.31-0.66), admission to the intensive care unit (ICU) (0.37, 0.23-0.60), requirement for invasive mechanical ventilation (IMV) (0.40, 0.25-0.64), and mortality (0.22, 0.10-0.50). Stratification by CALL score at admission showed that the benefit of preemptive hospitalization remained significant for patients requiring oxygen therapy (0.51, 0.31-0.83), admission to the ICU (0.48, 0.27-0.86), and IMV (0.51, 0.28-0.92). Mortality risk remained significantly reduced (0.19, 0.07-0.48). CONCLUSION: Preemptive hospitalization reduced the rate of disease progression and may be beneficial for improving COVID-19 patient outcomes.

Morbilidad neonatal extrema y sus desenlaces en un hospital de alta complejidad en el 2013 / Extreme neonatal morbidity and its outcomes in a high complexity hospital in 2013 / Morbidade neonatal extrema e seus resultados em um hospital de alta complexidade em 2013

Introducción. La Morbilidad neonatal extrema se refiere a cualquier evento ocurrido en los primeros veintiocho días de vida con riesgo vital inminente. Es multifactorial, son relevantes los retrasos en la atención. En 2010 se describió por primera vez como indicador de salud pública en Brasil. Metodología. Estudio transversal en un hospital de alta complejidad en el año 2013. Se revisaron las características sociodemográficas y asistenciales, los eventos de morbilidad y sus desenlaces, el indicador mismo, la mortalidad neonatal y los retrasos en la atención (tipos I - IV). Resultados. Ingresaron 1,190 neonatos, se presentaron 120 casos y se analizaron los primeros 60 por saturación de datos. La tasa de morbilidad neonatal extrema fue de 59 por cada 1,000 nacidos vivos y la de mortalidad de 13.9, similares a las brasileñas y superiores a las nacionales (33 por cada 1,000). Los eventos más importantes con respecto a mortalidad fueron peso menor de 1,500 gramos, ventilación mecánica, Apgar menor de 7 a los 5 minutos y malformaciones congénitas. Los retrasos en las madres fueron predominantemente de tipo I (45.4%) por pobre control prenatal. El retraso neonatal más frecuente fue el tipo IV (40%), por mala adherencia a protocolos institucionales. Discusión. El indicador local fue mayor que el nacional por la complejidad institucional y los retrasos en la atención, los cuales causaron saturación estadística. Se puede mejorar con mayor adherencia a estrategias existentes como el control prenatal y protocolos institucionales. Conclusión. La Morbilidad neonatal extrema visibiliza las falencias en atención materno­neonatal. Cómo citar. Delgado-Beltrán AM., Beltrán-Avendaño MA., Pérez-Vera LA. Morbilidad neonatal extrema y sus desenlaces en un hospital de alta complejidad en el 2013. MedUNAB. 2021;24(1): 61-71. Doi: https://doi.org/10.29375/01237047.3960

Introduction. Extreme neonatal morbidity refers to any event that occurs in the first twenty-eight days of life with imminent vital risk. It is multifactorial and delays in treatment are relevant. It was described for the first time as a public health indicator in Brazil in 2010. Methodology. This was a cross-sectional study in a high complexity hospital in 2013. Sociodemographic and assistance characteristics, morbidity events and their outcomes, the indicator itself, neonatal mortality, and delays in treatment (types I - IV) were reviewed. Results. 1,190 neonates were entered, 120 cases occurred and the first 60 were analyzed due to data saturation. The extreme neonatal morbidity rate was 59 for every 1,000 children born alive and the mortality rate was 13.9. This was similar to the figures in Brazil and greater than national statistics (33 for every 1,000). The most significant events with respect to mortality were weight lower than 1,500 grams, mechanical ventilation, an Apgar score lower than 7 at 5 minutes and congenital anomalies. Delays from mothers were predominantly type I (45.4%) due to poor prenatal control. The most frequent neonatal delay was type IV (40%) due to bad adherence to institutional protocols. Discussion. The local indicator was greater than the national indicator due to the institutional complexity and delays in treatment, which cause statistical saturation. This can be improved with better adherence to existing strategies, such as prenatal control and institutional protocols. Conclusion. Extreme neonatal morbidity shows the flaws in maternal - neonatal healthcare. Cómo citar. Delgado-Beltrán AM., Beltrán-Avendaño MA., Pérez-Vera LA. Morbilidad neonatal extrema y sus desenlaces en un hospital de alta complejidad en el 2013. MedUNAB. 2021;24(1): 61-71. Doi: https://doi.org/10.29375/01237047.3960

Introdução. A morbidade neonatal extrema refere-se a qualquer evento que ocorra nos primeiros vinte e oito dias de vida com risco de vida iminente. É multifatorial, e os atrasos no atendimento são relevantes. Em 2010 foi descrita pela primeira vez como um indicador de saúde pública no Brasil. Metodologia. Estudo transversal em um hospital de alta complexidade em 2013. Foram revisadas as características sociodemográficas e assistenciais, os eventos de morbidade e seus resultados, o próprio indicador, a mortalidade neonatal e os atrasos no atendimento (tipos I - IV). Resultados. Foram internados 1,190 recém-nascidos, foram identificados 120 casos e os primeiros 60 foram analisados devido à saturação dos dados. A taxa de morbidade neonatal extrema foi de 59 por 1,000 nascidos vivos e a taxa de mortalidade de 13,9, semelhante às taxas brasileiras e superior às taxas nacionais (33 por 1,000). Os eventos mais importantes em relação à mortalidade foram peso inferior a 1,500 gramas, ventilação mecânica, Apgar menor que 7 em 5 minutos e malformações congênitas. Os atrasos nas mães foram predominantemente do tipo I (45.4%) devido ao cuidado pré-natal precário. O atraso neonatal mais frequente foi do tipo IV (40%), devido à falta de aderência aos protocolos institucionais. Discussão. O indicador local foi superior ao nacional devido à complexidade institucional e aos atrasos no atendimento, o que causou saturação estatística. Pode ser melhorado com uma maior adesão às estratégias existentes, como atendimento pré-natal e protocolos institucionais. Conclusão. A morbidade neonatal extrema torna visíveis as deficiências no cuidado materno-neonatal. Cómo citar. Delgado-Beltrán AM., Beltrán-Avendaño MA., Pérez-Vera LA. Morbilidad neonatal extrema y sus desenlaces en un hospital de alta complejidad en el 2013. MedUNAB. 2021;24(1): 61-71. Doi: https://doi.org/10.29375/01237047.3960

Pentraxin-3 is a candidate biomarker on the spectrum of severity from pre-eclampsia to HELLP syndrome: GenPE study.

Pentraxin-3 has been reported as a promising biomarker of pre-eclampsia and its severity; however, available studies have small sample sizes, and analyses are not always adjusted for confounders. The aim of this study is to establish the strength of the association between maternal Pentraxin-3 level and pre-eclampsia or HELLP syndrome. It was a case-control study. Women with pre-eclampsia or HELLP syndrome were defined as cases, and women with healthy pregnancies at term (>37 weeks) were classified as controls. Plasma concentrations of Pentraxin-3 were determined at the time of delivery by quantitative enzyme immunoassay. Associations between Pentraxin-3 and pre-eclampsia and HELLP syndrome were assessed by multinomial logistic regression. Subsidiary analysis for the time of disease onset was also carried out. Odds ratios and 95% confidence intervals are reported. A total of 1024 pregnant women were included (461 controls, 368 pre-eclampsia, 195 HELLP). A positive log-linear relationship was found between the top pentraxin-3 quintile and HELLP syndrome. After adjustment for confounders (maternal age, ethnicity, socioeconomic position, date and place of recruitment, family history of pre-eclampsia, smoking, body mass index at beginning of pregnancy, gestational age and multiple pregnancy), the strength of the association was higher for HELLP syndrome [OR 1.13 (95% CI 1.08; 1.18)] than for pre-eclampsia [OR 1.03 (95% CI 1.03; 1.10)]. No difference according to time of onset or pentraxin-3 level was found. In summary, pentraxin-3 level was associated with pre-eclampsia, but it was more strongly associated with HELLP syndrome. Longitudinal studies with a lower probability of residual confounding are necessary to improve our knowledge about the role of pentraxin-3 in pre-eclampsia.

Chorea gravidarum: Case report and review of the literature / Corea Gravídica: reporte de caso y revisión de la literatura

OBJECTIVE: To present a case of chorea gravidarum and conduct a review of the published literature on the treatment for this condition, and on maternal and fetal prognosis. METHODS: Case presentation of a 16-year-old primiparous patient admitted to a Level III public hospital at 8 weeks of gestation complaining of involuntary head and limb movements and right lower limb hyperreflexia lasting three days. The patient had a history of Sydenham chorea. Treatment with antipsychotics and benzodiazepines was given to manage symptoms, and with benzathine penicillin to address the etiology, achieving control at 14 weeks. Treatment was discontinued at 35 weeks and the patient went on to have normal delivery at 39 weeks. A search was conducted in the Medline via PubMed, UptoDate, Medscape and Google Scholar databases using the terms "Pregnancy and Chorea Gravidarum". The search was limited to case reports and case series or review articles published between 2000 al 2019. RESULTS: Seven case reports and one review of the topic were found. In 4 of the 7 cases, treatment was based on haloperidol, benzodiazepines and chlorpromazine. Penicillin was used in one of two cases with a history of Sydenham chorea. Maternal and fetal prognosis was good in 6 of 7 cases, there was 1 case of intrauterine growth restriction. CONCLUSIONS: Treatment of gestation chorea is primarily expectant and the goal is to reduce symptoms. Maternal and fetal prognosis is good.

TITULO: COREA GRAVÍDICA: REPORTE DE CASO Y REVISIÓN DE LA LITERATURA. OBJETIVO: presentar un caso de corea gravídica y hacer una revisión sistemática de la literatura publicada sobre el tratamiento y pronóstico materno-fetal de la entidad. METODOS: se presenta el caso de una paciente de 16 años, primigesta, que ingresa a un hospital público de tercer nivel de complejidad, con 8 semanas de gestación, con clínica de 3 días de evolución caracterizada por movimientos involuntarios de la cabeza y las extremidades, con hiperreflexia en miembro inferior derecho. Antecedente de Corea de Sydenham. Se da tratamiento con antisicóticos y benzodiacepinas para la sintomatología y etiológico con penicilina benzatínica y control del cuadro a las 14 semanas. Suspendió el tratamiento a las 35 semanas. Parto normal a las 39 semanas. Se realizó una búsqueda en la base de datos de Medline vía PubMed, UptoDate, Medscape y Google Académico empleando los términos: "Pregnancy" and "Chorea Gravidarum". La búsqueda se limitó a reportes y series de casos o artículos de revisión, desde el año 2000 al 2019. RESULTADOS: se encontraron siete reportes de casos y una revisión de tema. El tratamiento se realizó con haloperidol benzodiacepinas y clorpromacina en cuatro de los siete casos. En uno de los dos casos con antecedentes de corea de Sydenham se utilizó penicilina. El pronóstico materno-fetal fue bueno en seis de siete casos, en un caso hubo restricción de crecimiento intrauterino. CONCLUSIONES: el tratamiento de la corea gestacional principalmente es expectante y su finalidad es disminuir la sintomatología, el pronóstico materno-fetal es bueno.

Chorea gravidarum: Case report and review of the literature / Corea gravídica: reporte de caso y revisión de la literatura

RESUMEN Objetivos: presentar un caso de corea gravídica y hacer una revisión sistemática de la literatura pu- blicada sobre el tratamiento y pronóstico materno- fetal de la entidad. Materiales y métodos: se presenta el caso de una paciente de 16 años, primigesta, que ingresa a un hospital público de tercer nivel de complejidad, con 8 semanas de gestación, con clínica de 3 días de evo- lución caracterizada por movimientos involuntarios de la cabeza y las extremidades, con hiperreflexia en miembro inferior derecho. Antecedente de Corea de Sydenham. Se da tratamiento con antisicóticos y benzodiacepinas para la sintomatología y etiológico con penicilina benzatínica y control del cuadro a las 14 semanas. Suspendió el tratamiento a las 35 semanas. Parto normal a las 39 semanas. Se realizó una búsqueda en la base de datos de Medline vía PubMed, UptoDate, Medscape y Google Académico empleando los términos: "Pregnancy" and "Chorea Gravidarum". La búsqueda se limitó a reportes y series de casos o artículos de revisión, desde el año 2000 al 2019. Resultados: se encontraron siete reportes de casos y una revisión de tema. El tratamiento se realizó con haloperidol benzodiacepinas y clorpromacina en cuatro de los siete casos. En uno de los dos casos con antecedentes de corea de Sydenham se utilizó penicilina. El pronóstico materno-fetal fue bueno en seis de siete casos, en un caso hubo restricción de crecimiento intrauterino. Conclusión: el tratamiento de la corea gestacional principalmente es expectante y su finalidad es disminuir la sintomatología, el pronóstico materno- fetal es bueno.

ABSTRACT Objectives: To present a case of chorea gravidarum and conduct a review of the published literature on the treatment for this condition, and on maternal and fetal prognosis. Materials and methods: Case presentation of a 16-year-old primiparous patient admitted to a Level III public hospital at 8 weeks of gestation complaining of involuntary head and limb movements and right lower limb hyperreflexia lasting three days. The patient had a history of Sydenham chorea. Treatment with antipsychotics and benzodiazepines was given to manage symptoms, and with benzathine penicillin to address the etiology, achieving control at 14 weeks. Treatment was discontinued at 35 weeks and the patient went on to have normal delivery at 39 weeks. A search was conducted in the Medline via PubMed, UptoDate, Medscape and Google Scholar databases using the terms "Pregnancy and Chorea Gravidarum". The search was limited to case reports and case series or review articles published between 2000 al 2019. Results: Seven case reports and one review of the topic were found. In 4 of the 7 cases, treatment was based on haloperidol, benzodiazepines and chlorpromazine. Penicillin was used in one of two cases with a history of Sydenham chorea. Maternal and fetal prognosis was good in 6 of 7 cases, there was 1 case of intrauterine growth restriction. Conclusion: Treatment of gestation chorea is primarily expectant and the goal is to reduce symptoms. Maternal and fetal prognosis is good.

Healthcare utilisation and costs of home mechanical ventilation.

BACKGROUND: Individuals using home mechanical ventilation (HMV) frequently choose to live at home for quality of life, despite financial burden. Previous studies of healthcare utilisation and costs do not consider public and private expenditures, including caregiver time. OBJECTIVES: To determine public and private healthcare utilisation and costs for HMV users living at home in two Canadian provinces, and examine factors associated with higher costs. METHODS: Longitudinal, prospective observational cost analysis study (April 2012 to August 2015) collecting data on public and private (out-of-pocket, third-party insurance, caregiving) costs every 2 weeks for 6 months using the Ambulatory and Home Care Record. Functional Independence Measure (FIM) was used at baseline and study completion. Regression models examined variables associated with total monthly costs selected a priori using Andersen and Newman's framework for healthcare utilisation, relevant literature, and clinical expertise. Data are reported in 2015 Canadian dollars ($C1=US$0.78=£0.51=€0.71). RESULTS: We enrolled 134 HMV users; 95 with family caregivers. Overall median (IQR) monthly healthcare cost was $5275 ($2291-$10 181) with $2410 (58%) publicly funded; $1609 (39%) family caregiving; and $141 (3%) out-of-pocket (<1% third-party insurance). Median healthcare costs were $8733 ($5868-$15 274) for those invasively ventilated and $3925 ($1212-$7390) for non-invasive ventilation. Variables associated with highest monthly costs were amyotrophic lateral sclerosis (1.88, 95% CI 1.09 to 3.26, P<0.03) and lower FIM quintiles (higher dependency) (up to 6.98, 95% CI 3.88 to 12.55, P<0.0001) adjusting for age, sex, tracheostomy and ventilation duration. CONCLUSIONS: For HMV users, most healthcare costs were publicly supported or associated with family caregiving. Highest costs were incurred by the most dependent users. Understanding healthcare costs for HMV users will inform policy decisions to optimise resource allocation, helping individuals live at home while minimising caregiver burden.

Síndrome de encefalopatía posterior reversible, eclampsia y síndrome de hellp / Reversible posterior encephalopathy syndrome, eclampsia and hellp syndrome

Resumen El síndrome de encefalopatía posterior reversible (PRES) es una entidad caracterizada por alteraciones clínicas y radiológicas debidas a un desbalance en la regulación de los vasos sanguíneos cerebrales. En la paciente obstétrica, el síndrome es más frecuente en la eclampsia y el síndrome de hemólisis, enzimas hepáticas elevadas y trombocitopenia (HELLP). Objetivo Caracterizar el PRES a nivel fisiopatológico, clínico, diagnóstico y terapéutico en la eclampsia y el síndrome de HELLP. Metodología Búsqueda bibliográfica con los términos MESH "Posterior Leukoencephalopathy Syndrome", "Pregnancy", "HELLP syndrome", "Eclampsia". Resultados La eclampsia es una de las condiciones más asociadas al PRES, las pacientes son primigestas, el curso clínico y los hallazgos imagenológicos son menos graves, el tratamiento debe enfocarse en la causa desencadenante, en este contexto debe optarse por desembarazar, administrar fármacos para manejar la hipertensión y las convulsiones. La literatura de PRES y HELLP se limita a series y reportes de caso. Las características propias de la fisiopatogenia del síndrome de HELLP pueden exagerar la respuesta vascular e incrementar el riesgo de sangrado lo que podría predisponer a un curso clínico más agresivo. El pronóstico del PRES en la paciente obstétrica es bueno, no se ha reportado riesgo de recurrencias en esta población. Conclusión Se necesitan estudios con un mayor seguimiento y número de pacientes que permitan aclarar las discordancias existentes. El conocimiento y manejo precoz del PRES contribuiría a la reducción de la morbimortalidad materna y las secuelas neurológicas a largo plazo.

ABSTRACT Posterior reversible encephalopathy syndrome (PRES) is an entity characterized by clinical and radiological manifestations due to an imbalance in cerebral blood vessels. In the obstetric patient, this syndrome is more frequent in eclampsia and hemolysis, elevated liver enzymes and thrombocytopenia (HELLP) syndrome. Aim To characterize PRES at physiopathological, clinical, diagnostic and therapeutic context in eclampsia and HELLP syndrome. Methodology Bibliographic search with the MESH terms "Posterior Leukoencephalopathy Syndrome", "Pregnancy", "HELLP Syndrome", "Eclampsia". Results Eclampsia is one of the most associated conditions with PRES, patients are primigravida, clinical course and imaging findings are less severe. The treatment should focus on the triggering cause, so emergent delivery and drugs to manage hypertension and seizures must be chosen. The literature on PRES and HELLP is limited to series and case reports. The characteristics of the pathophysiology of HELLP syndrome may exaggerate vascular response and increase the risk of bleeding, which may predispose to a more aggressive clinical course. Prognosis of PRES in the obstetric patient is good, there is not risk of recurrences reported in this population. Conclusion Studies with a greater follow-up and number of patients would clarify the existing discordances. Knowledge and early management of PRES would contribute to the reduction of maternal morbidity and mortality and long-term neurological sequelae.

Aproximación a la farmacología del sulfato de magnesio desde la perspectiva obstétrica / An approach to the pharmacology of magnesium sulfate from an obstetric perspective / Abordagem farmacológica do sulfato de magnésio apartir da perspectiva obstétrica

Introducción: Desde su primer uso en 1926 en el manejo de la eclampsia el sulfato de magnesio ha sido un medicamento utilizado y estudiado ampliamente por obstetras. Durante mucho tiempo se mantuvo escepticismo sobre sus potenciales beneficios, pero la aparición de estudios bien estructurados aportó evidencia a favor o en contra de algunos de estos. Objetivo: Realizar una revisión de la literatura acerca dela farmacología, fisiología, farmacocinética, mecanismos de acción, principales usos y regímenes de administración del sulfato de magnesio en obstetricia. Metodología: Búsqueda bibliográfica en Medline, a través de PubMed, utilizando los términos Magnesium Sulfate, Pharmacology, Obstetrics, Pre-eclampsia, Eclampsia, Neuroprotective Agents. Se adicionaron otros artículos con el fin de ampliar información en ciertos temas. Conclusiones: Las propiedades farmacológicas que expresa el sulfato de magnesio se relacionan directamente con su efecto antagónico con el calcio. Muestra efectos a nivel muscular, neuronal, cardiovascular, entre otros. Sus usos en obstetricia abarcan principalmente el manejo de la preeclampsia, prevención de la eclampsia, y prevención de la parálisis cerebral del recién nacido prematuro. El uso como agente tocolítico en el trabajo de parto prematuro aun es discutido ya que la evidencia es inconclusa.

Introduction: Since its first use in 1926 in eclampsia´s management magnesium sulphate has been a drug used and studied extensively by obstetricians. For a long time, practitioners remained sceptical about its potential benefits but the emergence of well-structured studies provided evidence in favor and against. Objective: A review of the literature on the pharmacology, physiology, pharmacokinetics, mechanisms of action, main applications and schemes of administration of the sulfate of magnesium in obstetrics. Methodology: Search in the database MEDLINE via PubMed, using the terms: Magnesium Sulfate, pharmacology, Obstetrics, Preeclampsia, Eclampsia, Neuroprotective Agents. Other papers were added in order to expand information on some topics. Conclusions: The pharmacological properties that express the magnesium sulfate is linked directly with its effect antagonistic with the calcium. Shows effects to level muscle, neuronal, cardiovascular, among others. Its uses in obstetrics include mainly the management of preeclampsia, prevention of eclampsia, and prevention of cerebral palsy in the premature neonate. The magnesium sulphate tocolytic effects even is discussed because the evidence is inconclusive.

Introdução: Desde que o sulfato de magnésio foi utilizado em 1926, no tratamento de eclampsia, tem sido usado e amplamente estudado por obstetras. Longo o ceticismo sobre seus potenciais benefícios permaneceu, mas tarde o aparecimento de estudos bem estruturados forneceram evidências a favor ou contra algum destes. Objetivo: Revisar a literatura sobre a farmacologia, fisiologia, fármaco cinética, mecanismos de ação, principais usos e administração de sulfato de magnésio em obstetrícia. Metodologia: Pesquisa bibliográfica Medline via PubMed utilizando os termos sulfato de magnésio, farmacologia, obstetrícia, pré-eclâmpsia, eclâmpsia, agentes neuroprotectores. Foram adicionados outros itens, a fim de obter maior informação sobre determinados temas. Conclusões: As propriedades farmacológicas que expressa o sulfato de magnésio estão diretamente relacionadas com o efeito antagônico do cálcio. Mostra os efeitos a nível muscular, neuronal, cardiovascular, entre outros. Em obstetrícia o uso abrange principalmente o tratamento da pré-eclampsia, a prevenção da eclampsia, e prevenção de paralisia cerebral no recém-nascido prematuro. Usa-lo como agente toco lítico no trabalho de parto prematuro é incerto porque a evidência é inconclusa.

Restricción del crecimiento intrauterino: una aproximación al diagnóstico, seguimiento y manejo / Intrauterine growth restriction: an approach to diagnosis, monitoring and management

ANTECEDENTES: La restricción del crecimiento intrauterino (RCIU) es una entidad de origen multifactorial que puede ser causada por una gran variedad de patologías a nivel materno, fetal o placentario, y que representa altas tasas de morbimortalidad materna y perinatal. Es importante realizar un diagnóstico certero de esta patología con el fin de llevar a cabo un enfoque de seguimiento y de manejo que pueda disminuir todas las complicaciones asociadas a la enfermedad. OBJETIVO: Realizar una revisión detallada y actualizada de la etiopatogenia, criterios diagnósticos, seguimiento con ecografía Doppler y manejo de la restricción del crecimiento intrauterino. MÉTODO: Se realizó una búsqueda de literatura publicada en inglés y español en bases de datos como PubMed/MEDLINE, Ovid, MDconsult utilizando las palabras clave: restricción del crecimiento intrauterino, feto pequeño para la edad gestacional, circulación fetal, ecografía Doppler, además de la búsqueda de estadísticas relacionadas con RCIU, de los resultados obtenidos se seleccionaron 61 artículos. La información obtenida fue clasificada y utilizada como soporte para la realización de esta revisión. RESULTADOS: Se presentan los estudios disponibles, las revisiones y artículos recomendados para la evaluación de pacientes con RCIU, adicionalmente aquellos que brindan nueva información con respecto al diagnóstico, evaluación de circulación fetal, seguimiento y manejo de esta entidad clínica. CONCLUSIÓN: La restricción del crecimiento intrauterino es una patología que presenta altas tasas de morbimortalidad perinatal. La identificación temprana de estos fetos y el seguimiento adecuado mediante la evalución Doppler permiten disminuir los resultados adversos y las secuelas a corto y largo plazo.

BACKGROUND: Intrauterine growth restriction has a multifactorial origin and can be caused by a variety of pathologies in the mother, fetus or placenta, representing high rates of maternal and perinatal morbidity and mortality. Therefore, it is important to accurately diagnose this condition in order to focus in the follow and management, which can reduce the complications associated with the disease. OBJECTIVE: To perform a detailed and up to date review of the etiology, diagnostic criteria, follow up with Doppler ultrasonography and management of intrauterine growth restriction. METHODS: A literature search was done in English and Spanish in databases such us PubMed/MEDLINE, Ovid, MDconsult using the following key words: intrauterine growth restriction, small for gestational age fetus, fetal circulation, Doppler ultrasonography. Also national statistics for intrauterine growth restriction were used, obtaining 61 documents. The information obtained was classified and used in the development of this review. RESULTS: The available studies are presented, and recommended revisions to the evaluation of patients with IUGR, additionally those that provide new information regarding the diagnosis, evaluation of fetal circulation, monitoring and management of this clinical entity. CONCLUSION: Intrauterine growth restriction is a pathology with a frequent diagnosis an represents high rates of perinatal morbidity and mortality. Early identification of these fetuses and adequate monitoring by Doppler evaluation allow reducing adverse outcomes and consequences in the short and long term.

Desenlaces materno-fetales de los embarazos atendidos en el Hospital Universitario de Santander, Bucaramanga (Colombia), 2007-2011. Estudio de cohorte / Maternal and foetal outcomes in twin pregnancies seen at Hospital Universitario de Santander, Bucaramanga (Colombia), 2007-2011. A cohort study

Los embarazos gemelares implican mayor riesgo de complicaciones materno-fetales que los embarazos únicos, particularmente en los monocoriales. El objetivo del trabajo fue describir las características clínicas y los desenlaces materno-fetales, por tipo de placenta (monocorial o bicorial), de los embarazos gemelares atendidos en el Hospital Universitario de Santander (HUS), institución de tercer nivel de complejidad localizada en Bucaramanga (Colombia). Materiales y métodos: estudio descriptivo de cohorte, se incluyeron las pacientes que terminaron un embarazo gemelar entre 2007 y 2011 en el HUS, hospital general de referencia de la región centro-oriental del país. Muestreo consecutivo. Se evaluó la edad gestacional en la primera consulta al hospital, los hallazgos clínicos en la evaluación inicial, la terminación del parto y los resultados perinatales. Se hace análisis descriptivo por tipo de corionicidad. Resultados: se incluyeron un total de 248 gestantes con embarazo gemelar en el periodo de estudio. La mediana de la edad gestacional en la primera atención en el hospital en fue de 34 semanas. Al ingreso, 127 (51,2 %) pacientes se diagnosticaron con embarazo monocorial, pero solo en dos terceras partes coincidió el diagnóstico prenatal de corionicidad con el del posparto. Se diagnosticó RCIU con más frecuencia en embarazos monocoriales que en bicoriales (22,3 vs. 7,5 %), y el doppler se encontró alterado con mayor frecuencia en fetos de embarazo monocorial (7,8 vs. 1,1 %). Los neonatos > 24 semanas de edad gestacional de embarazo monocorial pesaron, en promedio, 109 g (IC 95 %: 34-184) menos que los bicoriales. Conclusiones: los resultados de este estudio sugieren un problema de salud pública en este grupo de pacientes, con inicio tardío de control prenatal, de remisión tardía a centros especializados y capacidad insuficiente para definir corionicidad. Se requiere plantear estrategias de atención que incluyan considerar los embarazos gemelares como alto riesgo y garantizar la atención oportuna y adecuada, orientada por una guía de cuidado diferencial para este grupo de gestantes...

Twin pregnancies, especially monochorionic placentations, are associated with a higher rate of maternal and foetal complications when compared to singleton pregnancies. The objective of this work was to describe the clinical characteristics and the maternal and foetal outcomes according to the type of placentation (monochorial or dichorial) of twin pregnancies delivered at Hospital Universitario de Santander (HUS), a level III institution located in Bucaramanga, Colombia. Materials and methods: Descriptive cohort study. The cohort consisted of patients delivered of a twin pregnancy between 2007 and 2011 at the HUS, a general referral hospital for the central-eastern region of the country. The assessment included gestational age at the time of initial presentation to the hospital, clinical findings during the initial assessment, delivery completion, and perinatal results. Descriptive analysis by chorionicity type. Results: A total of 248 women with a twin pregnancy during the study period were included. The mean gestational age on the first visit to the hospital was 34 weeks. On admission, 127 patients (51.2%) were diagnosed with a monochorionic pregnancy, but only in two thirds of the cases was the prenatal chorionicity diagnosis consistent with the post-partum finding. IUGR was diagnosed more frequently in monochorionic than in dichorionic pregnancies (22.3% v. 7.5%) and abnormal Doppler findings were more frequent in monochorionic foetuses (7.8% v. 1.1%). Neonates > 24 of gestational age in monochorionic pregnancies weigh in average 109 gr (IC 95%: 34-184) less than dichorionic twins. Conclusions: The results of this study suggest a public health problem in this group of patients who come late for their prenatal visits and are late referrals to specialized centres, when there is already a limited ability to determine chorionicity. There is a need to develop care strategies in which twin pregnancies are included as a high-risk condition, and to ensure timely and adequate care provision in accordance with differential care guidelines for this group of pregnant women...

Multidrug-resistant tuberculosis: Treatment and outcomes of 93 patients.

BACKGROUND: Tuberculosis (TB) remains a leading cause of death worldwide and the emergence of multidrug-resistant TB (MDR TB) poses a threat to its control. There is scanty evidence regarding optimal management of MDR TB. The majority of Canadian cases of MDR TB are diagnosed in Ontario; most are managed by the Tuberculosis Service at West Park Healthcare Centre in Toronto. The authors reviewed 93 cases of MDR TB admitted from January 1, 2000 to December 31, 2011. RESULTS: Eighty-nine patients were foreign born. Fifty-six percent had a previous diagnosis of TB and most (70%) had only pulmonary involvement. Symptoms included productive cough, weight loss, fever and malaise. The average length of inpatient stay was 126 days. All patients had a peripherally inserted central catheter for the intensive treatment phase because medications were given intravenously. Treatment lasted for 24 months after bacteriologic conversion, and included a mean (± SD) of 5 ± 1 drugs. A successful outcome at the end of treatment was observed in 84% of patients. Bacteriological conversion was achieved in 98% of patients with initial positive sputum cultures; conversion occurred by four months in 91%. CONCLUSIONS: MDR TB can be controlled with the available anti-TB drugs.

Institutional care for long-term mechanical ventilation in Canada: A national survey.

INTRODUCTION: No national Canadian data define resource requirements and care delivery for ventilator-assisted individuals (VAIs) requiring long-term institutional care. Such data will assist in planning health care services to this population. OBJECTIVE: To describe institutional and patient characteristics, prevalence, equipment used, care elements and admission barriers for VAIs requiring long-term institutional care. METHODS: Centres were identified from a national inventory and snowball referrals. The survey weblink was provided from December 2012 to April 2013. Weekly reminders were sent for six weeks. RESULTS: The response rate was 84% (54 of 64), with 44 adult and 10 pediatric centres providing data for 428 VAIs (301 invasive ventilation; 127 noninvasive ventilation [NIV]), equivalent to 1.3 VAIs per 100,000 population. An additional 106 VAIs were on wait lists in 18 centres. More VAIs with progressive neuromuscular disease received invasive ventilation than NIV (P<0.001); more VAIs with chronic obstructive pulmonary disease (P<0.001), obesity hypoventilation syndrome (P<0.001) and central hypoventilation syndrome (P=0.02) required NIV. All centres used positive pressure ventilators, 21% diaphragmatic pacing, 15% negative pressure and 13% phrenic nerve stimulation. Most centres used lung volume recruitment (55%), manually (71%) and mechanically assisted cough (55%). Lack of beds and provincial funding were common admission barriers.CONCLUSIONS: Variable models and care practices exist for institutionalized care of Canadian VAIs. Patient prevalence was 1.3 per 100,000 Canadians.

Family caregiver perspectives on caring for ventilator-assisted individuals at home.

BACKGROUND: The trend of patients who are invasively ventilated to prefer home care is one that benefits both the patient and the health care system. However, this assumes a role for patients' family members to become informal caregivers. OBJECTIVE: To explore the impact of caring for a ventilator-assisted individual on informal caregivers. METHODS: A descriptive design with semistructured caregiver interviews and the Caregiver Burden Inventory were used. Participants were informal caregivers of a family member with a progressive neuromuscular disease on invasive ventilation for at least six months. Transcript coding was performed and regularly reviewed, and recruitment continued until data saturation. Qualitative analysis was based on 'thematic analysis'. RESULTS: A total of 21 caregivers were interviewed. Five themes developed: a sense of duty; restriction of day-to-day life; physical and emotional burden; training and education; and the need for more paid support. Caregivers described a sense of duty to take care of loved ones, but suffered a significant restriction of their own time with a negative impact on their physical and mental health. The initial transfer home was highlighted as the most stressful part of the process. The Caregiver Burden Inventory scores supported a high level of burden: median 49 (interquartile range 39.5 to 53.0) of a maximum 96. CONCLUSION: Homecare for ventilator-assisted individuals with progressive neuromuscular disease causes significant burden to informal caregivers. Approaches to lessen this burden, such as increased paid care, improved professional support and respite care, may enable home ventilation to be a more sustainable modality of care.

Preeclampsia y riesgo cardiovascular: estudio de seguimiento en la población de GenPE en Colombia / Pre-eclampsia and cardiovascular risk: a follow-up study of the GenPE population in Colombia

Introducción: las mujeres con antecedente de preeclampsia (PE) tienen mayor riesgo de desarrollar enfermedad cardiovascular. El estudio GenPE ha captado gestantes con y sin PE en Colombia durante diez años, haciendo posible hoy evaluar desenlaces cardiovasculares en este grupo. Objetivo: determinar la presencia de enfermedad cardiovascular en mujeres jóvenes expuestas o no a preeclampsia, captadas por el estudio GenPE en Bucaramanga, Colombia. Materiales y métodos: estudio de cohorte que incluyó 106 pacientes –a partir de la cohorte original, 666 pacientes (2005-2010)–, menores de 26 años, primigestantes, sin antecedentes de enfermedades crónicas. Se realizó examen físico y venopunción para glucemia, perfil lipídico, ácido úrico y apolipoproteínas A-I y B. Se evaluó la presencia de hipertensión arterial (HA), obesidad, diabetes mellitus tipo 2 y síndrome metabólico. Se establecieron diferencias entre las mujeres expuestas y no expuestas a preeclampsia y el cambio entre el ingreso a GenPE y el primer seguimiento mediante test Wilcoxon o chi cuadrado y riesgo relativo con IC 95%. Resultados: se encontró asociación entre la exposición a PE con cifras mayores de presión arterial diastólica (p = 0,003) e hipercolesterolemia (p = 0,040). En toda la población existen niveles subóptimos de colesterol HDL y Apo A-I. Al primer seguimiento no se evidencia asociación con desenlaces fuertes, tales como: hipertensión arterial, obesidad, diabetes mellitus tipo 2, síndrome metabólico. Conclusión: en una muestra de mujeres colombianas jóvenes expuestas y no expuestas a PE, en un primer seguimiento, en los primeros dos años posteriores al parto, se evidencian cambios en la presión arterial diastólica (PAD), y biomarcadores asociados a riesgo cardiovascular.

Introduction: Females having a background of pre-eclampsia (PE) are at greater risk of developing cardiovascular disease. The GenPE study has captured pregnant females with and without PE in Colombia during the last 10 years, making it possible today to evaluate cardiovascular outcomes in this group. Objective: Determining the presence of cardiovascular disease in young females who were and were not exposed to preclampsia and who were recruited by the GenPE study carried out in Bucaramanga, Colombia. Materials and methods: This was a cohort study which included 106 primiparous patients aged less than 26 years old having no background of chronic disease taken from an original cohort of 666 patients (2005-2010). They were physically examined and venopuncture was made for glycemia, lipid profile, uric acid and apolypoprotein A-I and B. The presence of hypertension, obesity, type 2 diabetes mellitus and metabolic syndrome were evaluated. Differences were established by Wilcoxon or Chisquared tests (relative risk and 95% CI) between females who had been exposed to PE and those who had not been so and the change between entering the GenPE study and the first follow-up. Results: An association was found between exposure to PE and having higher diastolic pressure figures (p = 0.003) and hypercholesterolemia (p = 0.040). Sub-optimal HDL cholesterol and apo A-I levels were found in the whole population. No association with strong outcomes was found during the first follow-up, such as hypertension, obesity, type 2 diabetes mellitus or metabolic syndrome. Conclusion: Changes in peripheral arterial disease (PAD) and cardiovascular risk-associated biomarkers became evident in a sample of young colombian females who developed PE during the first followup within 2 years after delivery.

Home mechanical ventilation: a Canadian Thoracic Society clinical practice guideline.

Increasing numbers of patients are surviving episodes of prolonged mechanical ventilation or benefitting from the recent availability of userfriendly noninvasive ventilators. Although many publications pertaining to specific aspects of home mechanical ventilation (HMV) exist, very few comprehensive guidelines that bring together all of the current literature on patients at risk for or using mechanical ventilatory support are available. The Canadian Thoracic Society HMV Guideline Committee has reviewed the available English literature on topics related to HMV in adults, and completed a detailed guideline that will help standardize and improve the assessment and management of individuals requiring noninvasive or invasive HMV. The guideline provides a disease-specific review of illnesses including amyotrophic lateral sclerosis, spinal cord injury, muscular dystrophies, myotonic dystrophy, kyphoscoliosis, post-polio syndrome, central hypoventilation syndrome, obesity hypoventilation syndrome, and chronic obstructive pulmonary disease as well as important common themes such as airway clearance and the process of transition to home. The guidelines have been extensively reviewed by international experts, allied health professionals and target audiences. They will be updated on a regular basis to incorporate any new information.

[Pulmonary and nonpulmonary alterations in Duchenne muscular dystrophy]. / Alteraciones pulmonares y no pulmonares en la distrofia muscular de Duchenne.

OBJECTIVE: To describe our experience in managing patients with Duchenne muscular dystrophy. PATIENTS AND METHODS: We analyzed the following variables in a group of 27 patients with Duchenne muscular dystrophy: arterial blood gases, lung function before and after mechanical ventilation, oxygen saturation (measured by pulse oximetry), nocturnal PaCO2 (measured transcutaneously by capnography), heart function, and dysphagia. RESULTS: The mean (SD) age was 26 (6) years and the mean age at which mechanical ventilation had initiated in the patients was 21 (5) years. Sixty-two percent had undergone tracheostomy and invasive mechanical ventilation. Arterial blood gas levels returned to normal once mechanical ventilation was administered and remained so for the entire treatment period (mean duration of follow-up, 56 [49] months). Thirteen patients had cardiac symptoms and they all presented abnormal electrocardiograms and echocardiograms indicating dilated cardiomyopathy, left ventricular dysfunction, and posterior hypokinesis. Only 9 patients were receiving enteral nutrition (7 through a gastrostomy tube and 2 through a nasogastric tube). The videofluoroscopic swallowing study confirmed that dysphagia was related to neuromuscular disease rather than the presence or not of a tracheostomy. Five patients (18%), 4 of whom were receiving invasive mechanical ventilation, died during the follow-up period. Three patients had serious heart disease. CONCLUSIONS: Mechanical ventilation confers clinical benefits and prolongs life expectancy in patients with Duchenne muscular dystrophy. Heart disease and feeding difficulties are determining factors in the prognosis of these patients.

Alteraciones pulmonares y no pulmonares en la distrofia muscular de Duchenne / Pulmonary and nonpulmonary alterations in Duchenne muscular dystrophy

Objetivo: Describir nuestra experiencia en el manejo de pacientes con distrofia muscular de Duchenne (DMD). Pacientes y métodos: En 27 pacientes con DMD analizamos los gases arteriales y la función pulmonar antes y después de la ventilación mecánica (VM); la pulsioximetría (saturación de oxihemoglobina) y la capnografía (presión arterial de anhídrido carbónico por determinación transcutánea) nocturna; la función cardíaca y la evaluación de la disfagia. Resultados: Se incluyó en el estudio a 27 pacientes con una edad media ± desviación estándar de 26 ± 6 años. Todos recibían VM, que se había iniciado cuando contaban 21 ± 5 años. El 62% eran portadores de traqueostomía y VM invasiva. Una vez iniciada la VM, se observó la normalización de los gases arteriales, que se mantuvo durante todo el tiempo de tratamiento (seguimiento medio: 56 ± 49 meses). Trece pacientes presentaban síntomas cardíacos y en todos ellos se observaban anormalidades en el electrocardiograma y ecocardiograma: miocardiopatía dilatada, disfunción ventricular izquierda o hipocinesia de la pared posterior. Sólo 7 pacientes llevaban una sonda de gastrostomía para alimentación y 2 una sonda nasogástrica. El estudio con videofluoroscopia permitió afirmar que los problemas de disfagia estaban relacionados con la enfermedad neuromuscular y no con la presencia o no de traqueostomía. Durante el período de seguimiento, 5 pacientes fallecieron (18%), 4 de ellos con VM invasiva; 3 pacientes presentaban una enfermedad cardíaca grave. Conclusiones: La VM proporciona beneficios clínicos y prolonga la vida de los pacientes con DMD. Las alteraciones cardíacas y nutricionales son factores determinantes en el pronóstico de estos pacientes

Objective: To describe our experience in managing patients with Duchenne muscular dystrophy. Patients and Methods: We analyzed the following variables in a group of 27 patients with Duchenne muscular dystrophy: arterial blood gases, lung function before and after mechanical ventilation, oxygen saturation (measured by pulse oximetry), nocturnal PaCO2 (measured transcutaneously by capnography), heart function, and dysphagia. Results: The mean (SD) age was 26 (6) years and the mean age at wich mechanical ventilation had initiatid in the patients was 21 (5) years. Sixty-two percent had undergone tracheostomy and invasive mechanical ventilation. Arterial blood gas levels returned to normal once mechanical ventilation was administered and remained so for the entire treatment period (mean duration of follow-up, 56 [49] months). Thirteen patients had cardiac symptoms and they all presented abnormal electrocardiograms and echocardiograms indicating dilated cardiomyopathy, left ventricular dysfunction, and posterior hypokinesis. Only 9 patients were receiving enteral nutrition (7 through a gastrostomy tube and 2 through a nasogastric tube). The videofluoroscopic swallowing study confirmed that dysphagia was related to neuromuscular disease rather than the presence or not of a tracheostomy. Five patients (18%), 4 of whom were receiving invasive mechanical ventilation, died during the follow-up period. Three patients had serious heart disease. Conclusions: Mechanical ventilation confers clinical benefits and prolongs life expectancy in patients with Duchenne muscular dystrophy. Heart disease and feeding difficulties are determining factors in the prognosis of these patients