Palliative care in pulmonary medicine / Cuidados paliativos em pneumologia

ABSTRACT Palliative care was initially developed for patients with advanced cancer. The concept has evolved and now encompasses any life-threatening chronic disease. Studies carried out to compare end-of-life symptoms have shown that although symptoms such as pain and dyspnea are as prevalent in patients with lung disease as in patients with cancer, the former receive less palliative treatment than do the latter. There is a need to refute the idea that palliative care should be adopted only when curative treatment is no longer possible. Palliative care should be provided in conjunction with curative treatment at the time of diagnosis, by means of a joint decision-making process; that is, the patient and the physician should work together to plan the therapy, seeking to improve quality of life while reducing physical, psychological, and spiritual suffering.

RESUMO Inicialmente os cuidados paliativos foram desenvolvidos para pacientes com câncer avançado. Hoje este conceito evoluiu e engloba qualquer doença crônica que ameace a vida. Estudos realizados para comparar os sintomas de fim de vida mostraram que os pacientes pneumológicos apresentavam a mesma prevalência de sintomas, como dor e dispneia, porém recebiam menos tratamento paliativo que os pacientes oncológicos. É preciso desmitificar a ideia de que cuidados paliativos só devem ser adotados quando não há mais possibilidade de tratamento curativo. O cuidado paliativo deve ser associado ao tratamento curativo no momento do diagnóstico, por meio de decisão compartilhada, isto é, paciente e médico planejam as decisões sobre a terapêutica a ser tomada, buscam a melhora na qualidade de vida e a redução do sofrimento, tanto espiritual como físico e psicológico.

Influence of pulmonary rehabilitation on clinical characteristics in patients with chronic heart failure and chronic obstructive pulmonary disease / Influência da reabilitação pulmonar nas características clínicas em pacientes com insuficiência cardíaca crônica e doença pulmonar obstrutiva crônica

Background: The improvement of treatment strategies in patients with chronic obstructive pulmonary disease (COPD) and especially with comorbid pathology should provide rational conversion of standard schemes of therapy and rehabilitation in accordance with their clinical, pathogenic, functional and economic feasibility. Objective: To assess the influence of pulmonary rehabilitation on clinical characteristics in patients with chronic heart failure (CHF) and concomitant COPD. Methods: The study included 102 patients with CHF and concomitant COPD (males, 62%; mean age, 68.2 ± 4.5 years). All patients were divided into two groups: control group (CG) (n = 54), received only standard therapy of CHF and COPD; and intervention group (IG) (n = 48) were additionally taught the full yogic breathing as a program of pulmonary rehabilitation. Calculation of points by clinical evaluation scale (CES), assessment of CHF functional class (FC) (NYHA) and 6-minute walk test (6MWT - with the evaluation of dyspnea by the Borg scale) were performed in all patients on admission to the department and at discharge. Significant association was defined by p value < 0.05. Results: At baseline, there were no significant differences in clinical characteristics of the patients and studied parameters between the groups. At discharge both groups showed significant reduction of dyspnea by the Borg scale (in CG: from 7.2 ± 0.8 points to 5.2 ± 0.3; in IG: from 7.4 ± 0.6 points to 3.2 ± 0.4), the number of points by CES (in CG: from 10.8 ± 0.3 points to 7.2 ± 0.4; in IG: from 10.7 ± 0.6 points to 5.9 ± 0.6). Increase in exercise tolerance (by the distance of 6MWT) was observed in both groups (in CG: from 215 ± 24 m to 275 ± 22 m; in IG: from 219 ± 21 m to 308 ± 24 m). The changes were more significant in IG compared to CG. We observed the prominent decrease in CHF FC and length of hospital stay in IG. Conclusions: Application of full yogic breathing as the program of pulmonary rehabilitation in addition to standard therapy of the patients with CHF and COPD is associated with a significant decrease in CHF FC, an increase in exercise tolerance and a reduced length of hospital stay

Evaluación de la disnea asociada al ejercicio / Exercise-induced dyspnea evaluation

Exercise-induced dyspnea is a common pediatric question but difficult to address since usually symptoms are described ambiguously by the child or parents. Most of times dyspnea is secondary to poor training but sometimes may be due to an underlying condition like exercise-induced bronchoconstriction or vocal cord dysfunction. To provide clinicians with a practical approach about exercise-induced dyspnea we have review pathogenesis and clinical characteristics of respiratory diseases and proposed an algorithm for study.

Frecuentemente nos vemos enfrentados a evaluar un niño con síntomas vagos asociados al ejercicio que el mismo paciente o sus padres describen como ahogo o sensación de pecho apretado. La mayoría de las veces se trata de cansancio atribuible al ejercicio normal que solo refleja pobre condicionamiento físico del individuo; sin embargo, este cansancio puede ser desproporcionado al esfuerzo, lo que obliga a considerar enfermedades comunes como asma o poco comunes como disfunción de cuerdas vocales. El objetivo de esta revisión es actualizar el conocimiento aquellas enfermedades respiratorias que forman parte del diagnóstico diferencial de la disnea asociada al ejercicio y proponer un algoritmo de estudio que permita un acercamiento práctico según causas de origen.

Dyspnées en milieu hospitalier ORL du CHU Souro Sanou : aspects épidémiologiques, étiologiques, thérapeutiques et évolutifs

Objectif : Décrire les aspects épidémiologiques, étiologiques, thérapeutiques et évolutifs des cas de dyspnées dans le service d'ORL/CCF du CHU Souro Sanou. Méthodologie : Il s'agit d'une étude rétrospective et descriptive qui a colligé les dossiers cliniques de 151 patients hospitalisés pour une dyspnée dans le service au cours de la période de janvier 2012 à décembre 2016. Résultats : Les patients de 0 à 5 ans et de 5 ans à 10 ans représentaient 53,64% et 12,58%. Les corps étrangers des voies respiratoires étaient les plus fréquents avec 28,48% des étiologies. Les amygdalites hypertrophiques et obstructives associées aux végétations adénoïdes et les végétations adénoïdes isolées représentaient chacune 12,88%. Les taux de guérison et de décès étaient de 84,11% et de 9,27%. Le cancer de l'hypopharynx était la principale cause de décès avec 21,43% des cas. Conclusion : Les dyspnées en ORL sont des urgences diagnostiques et thérapeutiques. Les causes variées nécessitent un plateau technique adapté et du personnel qualifié

Efeitos da ventilação não-invasiva sobre a hiperinsuflação dinâmica de pacientes com DPOC durante atividade da vida diária com os membros superiores / Effects of noninvasive ventilation on dynamic hiperinflation of patients with COPD during activities of daily living with upper limbs

CONTEXTUALIZAÇÃO: Pacientes com doença pulmonar obstrutiva crônica (DPOC) queixam-se de dispneia nas atividades de vida diária (AVD) com os membros superiores (MMSS). A hiperinsuflação dinâmica (HD) é um dos mecanismos ventilatórios que contribuem para a dispneia. Para minimizar a HD, propõe-se a utilização de sistemas de ventilação não-invasiva (VNI). OBJETIVOS: Verificar se existe HD e dispneia durante a realização de uma AVD com os MMSS com e sem o uso da VNI. MÉTODOS: Participaram 32 pacientes com DPOC de moderada a muito grave, com idades entre 54 a 87 anos (69,4±7,4). Os pacientes elevaram potes com pesos de 0,5 a 5 kg durante 5 minutos, iniciando a elevação a partir da cintura pélvica em direção a uma prateleira localizada acima da cabeça, com e sem o uso da VNI (BiPAP®; IPAP 10 cmH2O; EPAP 4 cmH2O). Foram avaliadas a capacidade inspiratória (CI) e a dispneia (Escala de Borg). A CI foi mensurada antes e após a simulação da AVD. Na análise dos dados foram utilizados o teste t de Student para amostras dependentes e o teste de Wilcoxon. RESULTADOS: Houve redução significativa da CI após a AVD com e sem VNI (p=0,01). A dispneia aumentou após a AVD com e sem a VNI, mas entre ambos os protocolos não houve diferença. CONCLUSÕES: A simulação da AVD com os MMSS resultou em aumento da HD e dispneia. A VNI ofertada com pressões preestabelecidas não foi suficiente para evitar a HD e a dispneia.

BACKGROUND: Patients with chronic obstructive pulmonary disease (COPD) complain of dyspnea on activities of daily living (ADLs) with the upper limbs. Dynamic hyperinflation (DH) is one of the ventilatory mechanisms that may contribute towards dyspnea. To minimize the DH it is indicated the use of noninvasive ventilation (NIV). OBJECTIVES: To verify whether there is DH and dyspnea during the performance of ADL with the upper limbs with and without the use of NIV. METHODS: 32 patients with moderate-to-severe COPD, aged 54 to 87 years (mean 69.4, SD 7.4) were evaluated. The subjects lift up containers weighing between 0.5 and 5.0 kg over a five-minute period, starting from the waist level and putting them onto a shelf located above head height, with and without the use of NIV (BiPAP®; IPAP 10cmH2O; EPAP 4 cmH2O). The inspiratory capacity (IC) and dyspnea (Borg scale) were evaluated on all the patients. The IC was measured before and after simulation of the ADL. In order to analyze the data, Student's t test for dependent samples and the Wilcoxon test were used. RESULTS: There were statistically significant reductions in IC after the ADL with and without NIV (p=0.01). The dyspnea increased after the ADL with and without the NIV, however between both interventional procedures protocols no between-group difference was observed. CONCLUSIONS: The simulation of an ADL with the upper limbs resulted in an increase in DH and dyspnea. The NIV supplied with pre-established pressure was not enough to prevent the DH and dyspnea.

Entrenamiento muscular de las extremidades inferiores en el paciente con enfermedad pulmonar obstructiva crónica / Lower extremity exercise training in the rehabilitation of patients with chronic obstructive pulmonary disease

Several studies have shown that poor exercise tolerance in Chronic Obstructive Pulmonary Disease (COPD) patients is multifactorial in origin. However, a major exercise-limiting factor in COPD is peripheral muscle dysfunction, particularly the muscles of the lower extremities, characterized by atrophic muscles and reduced fatigue resistance due to morphological and metabolic alterations of peripheral muscles. This chapter therefore evaluated the scientific evidence regarding the beneficial effect of lower extremities exercise in the pulmonary rehabilitation in COPD patients. The technical characteristics of this exercise training were also reviewed. Exercise training of lower limbs was recommended in respiratory rehabilitation of COPD patients. The lower extremities muscle exercise training provides significant benefits to patients with COPD in terms of reduction of dyspnea and improvemet in exercise capacity and in quality of life (quality level of evidence = A, strong recommendation). Higher-intensity exercise training and with interval exercise of the lower extrmities produces greater physiological benefits.

Diversos estudios han demostrado que la pobre tolerancia al ejercicio de los pacientes con Enfermedad Pulmonar Obstructiva Crónica (EPOC) es de origen multifactorial. Sin embargo, un importante factor limitante del ejercicio en los pacientes con EPOC es la disfunción muscular periférica, sobre todo de los músculos de las extremidades inferiores, que se caracteriza por atrofia muscular y reducida resistencia a la fatiga dado por alteraciones morfológicas y metabólicas de los músculos periféricos. En este capitulo se evaluó la evidencia científica que existe en cuanto a los beneficios del entrenamiento muscular de extremidades inferiores (EEII) en la rehabilitación respiratoria en pacientes con EPOC. También se revisan las características técnicas de dicho entrenamiento. Se recomienda la realización de entrenamiento muscular de EEII en rehabilitación respiratoria de pacientes con EPOC. El entrenamiento muscular de extremidades inferiores otorga significativos beneficios a los pacientes con EPOC en cuanto a disminuir la disnea, mejorar la capacidad de ejercicio y la calidad de vida (calidad de la evidencia A, recomendación fuerte). El entrenamiento de EEII de alta intensidad y con intervalos produce significativos beneficios fisiológicos.

Disnea y derrame pleural izquierdo masivo como forma de presentación de la disección de la aorta descendente / Dyspnea and left pleural effusion as a dissection of the thoracic aorta

Se presenta el caso de un paciente que acudió a urgencias por disnea intensa. La historia clínica, la situación de bajo gasto cardíaco y el derrame pleural masivo serohemático llevaron a la sospecha de disección aórtica.

Evolución funcional respiratoria en dos pacientes con enfisema y fibrosis pulmonar / Functional respiratory evolution in two patients with emphysema and pulmonary fibrosis

La combinación de enfisema y fibrosis pulmonar es una condición frecuentemente subdiagnosticada. Los estudios funcionales aislados pueden generar interpretaciones inadecuadas. No hemos hallado comunicaciones de casos que documenten la evolución espirométrica de estos pacientes. Se presentan dos casos de fibrosis y enfisema combinados que permiten observar la evolución funcional a largo plazo y comprender los valores espirométricos actuales en forma más precisa. Los hallazgos más relevantes son: 1) espirometría con discretas alteraciones funcionales en presencia de disnea marcada y, en un paciente, necesidad de oxigenoterapia crónica, 2) evolución funcional con seudonormalización del patrón espirométrico obstructivo inicial, posiblemente como consecuencia del desarrollo de fibrosis. Un defecto obstructivo leve en un paciente con diagnóstico de obstrucción crónica al flujo aéreo y marcado deterioro de su condición clínica y clase funcional debería alertar sobre la posibilidad de fibrosis pulmonar asociada. La tomografía y la disponibilidad de estudios funcionales previos permiten comprender esta condición.

Combined pulmonary fibrosis and emphysema (CPFE) is a frequently under-diagnosed condition. Isolated pulmonary function tests (PFT) can give rise to misinterpretations. We have found no reports on these patients’ spirometric progression. We describe two cases of CPFE, showing long-term functional evolution to have a more accurate understanding of current spirometric values. The most relevant findings are: 1) spirometry with discrete functional alterations in the presence of a marked dyspnea and the need, in one patient, for chronic oxygen therapy; and 2) functional evolution reflecting "pseudonormalisation" of the initial obstructive spirometric pattern, possibly as a result of fibrosis development. A mild obstructive defect in a patient with chronic airflow limitation and marked impairment of his/her clinical status and functional class should alert on the possibility of associated pulmonary fibrosis. A computed tomography (CT) and previous PFTs will allow a better understanding of this condition.

Adaptation of the Medical Research Council Dyspnea Scale and the Oxygen Cost Diagram for its use in Puerto Rico

INTRODUCTION: Dyspnea is a common and disabling symptom for patients with chronic lung diseases. The Medical Research Council Dyspnea Scale (MRC) and the Oxygen Cost Diagram (OCD) are widely used instruments validated in English to measure breathlessness. OBJECTIVE: To translate and culturally adapt the MRC and the OCD for its use in Puerto Rico. METHOD: The scales were translated to Spanish and back translated. They were tested in patients attending a pulmonary clinic to assess its relevance and comprehension. Subjects answered the instruments, had a structured clinical interview and provided feedback. A multidisciplinary committee analyzed the source of misunderstanding using the input of the subjects, the clinical physicians, and the evaluators; made adjustments, and retested the instruments until inconsistencies were not observed. RESULTS: Placing emphasis on time spent walking instead of distance traveled improved the discrimination between grade two and three in the MRC. In the OCD, placing the activities to the right side eased the interpretation of the scale. Numerical symbols were eliminated to minimize discomfort in the severely impaired subjects reluctant to mark the line near zero. Reversing the order of both scales encouraged a thorough reading of the activities from minimal to high energy demanding eliciting a more fitting response compared to structured clinical interview especially in the severely ill patient. CONCLUSION: Using cross-cultural research methodologies to translate the MRC and OCD allowed the identification of differences in conceptualization when assessing the severity of dyspnea in Puerto Rico. Further testing is needed to confirm psychometric properties.

Iron therapy for breath holding attacks in non-anemic infants and children

Breath holding attacks are common paroxysmal disorders in infants and children. Research trials showed good response to iron therapy. Most of these trials however, included anemic patients. To study the effect of iron therapy in the treatment of breath holding attacks in non anemic infants and children. This study has been carried out in the pediatric department, Sohag faculty of medicine in the period from 20 March 2005 to 20 February 2006. During this period all patients with breath holding spells were identified. Patients with anemia manifested clinically or with low Hb level or with low ferritin level were excluded from the study. We also excluded patients with abnormal neurological examination or abnormal electroencephalography [EEG]. The remaining patients [non-anemic neurologically normal patients] received oral iron in a dose of 6 mg/kg/day for 3 months. The response was determined either by disappearance [complete response] or decrease in the frequency and duration of attacks [partial response]. We identified 56 patients with breath holding spells. 27 patients were excluded from the study [20 were anemic, 4 had abnormal EEG and 3 had abnormal neurological examination]. The remaining 29 patients had been included in this study and received oral iron therapy. 3 patients received iron therapy but losses follow up. Concerning the 26 patients who had completed the study: 13 patients showed complete response, 4 patients showed partial response and 9 patients did not show any response. Iron therapy should be tried in all infants and children with breath holding attacks

Effects of an Inpatient Pulmonary Rehabilitation Program on Dyspnea, Exercise Capacity, and Health Related Quality of Life for Patients with Chronic Lung Disease / 간호학회지

PURPOSE: The purpose of this study was to examine the effects of an inpatient pulmonary rehabilitation program on dyspnea, exercise capacity, and health related quality of life in inpatients with chronic lung disease. METHOD: This quasi experimental study was designed with a nonequivalent control group pre-post test time series. Twenty three patients were assigned to the experimental group and nineteen to the control group. The inpatient pulmonary rehabilitation program was composed of upper and lower extremity exercise, breathing retraining, inspiratory muscle training, education, relaxation and telephone contacts. This program consisted of 4 sessions with inpatients and 4 weeks at home after discharge. The control group was given a home based pulmonary rehabilitation program at the time of discharge. The outcomes were measured by the Borg score, 6MWD and the Chronic Respiratory Disease Questionnaire(CRQ). RESULTS: There was a statistically significant difference in dyspnea between the experimental group and control group, but not among time sequence, or interaction between groups and time sequence. Also significant improvements in exercise capacity and health related quality of life were found only in the experimental group. CONCLUSIONS: An Inpatient pulmonary rehabilitation program may be a useful intervention to reduce dyspnea, and increase exercise capacity and health related quality of life for chronic lung disease patients.

Efeitos da cinesioterapia respiratória na mobilidade da caixa torácica, capacidade de exercício e qualidade de vida dos pacientes portadores de DPOC / The effects of respiratory exercising in the chest wall mobility, exercising capacity and quality of life in COPD patients

O paciente portador de Doença Pulmonar Obstrutiva Crônica (DPOC) apresenta perda progressiva da função pulmonar, que desencadeia diminuição em sua atividade física global, prejudicando sua qualidade de vida. Com o objetivo de otimizar a função desses pacientes, foram desenvolvidos os programas de reabilitação pulmonar. Os principais componentes desses programas são: treinamento de membros inferiores, treinamento de membros superiores, treinamento ventilatório e intervenção psicossocial. A cinesioterapia respiratória é uma técnica muito aplicada na prática clínica, contudo pouco estudada. Diante disso, o objetivo do presente estudo foi verifi car a contribuição da cinesioterapia respiratória na mobilidade da caixa torácica, capacidade de exercício e qualidade de vida no paciente com DPOC. Foram selecionados 03 pacientes com diagnóstico de DPOC grau moderado, que foram submetidos a um programa de cinesioterapia respiratória. Os efeitos do programa foram avaliados pelos seguintes parâmetros: espirometria, cirtometria torácica, distância percorrida em seis minutos (DP6min) e questionário de qualidade de vida. Após dez sessões de cinesioterapia respiratória os pacientes apresentaram um aumento na mobilidade da caixa torácica e na qualidade de vida, mantendo a capacidade de exercício. Portanto, a cinesioterapia respiratória pode ser uma técnica que benefi cie funcionalmente o paciente portador de DPOC.

The Chronic Obstructive Pulmonary Disease (COPD) patients present gradual loss of the pulmonary function, which implies in the reduction of their global physical activity, harming their quality of life. With the objective of optimizing these patients' function, the rehabilitation program was developed. The main components of these programs are: training of limb members, training of upper members, ventilatory training, and psychosocial intervention. Moreover, respiratory exercising is a largely applied technique within the clinical practice; however, there are not many studies. The objective ofthe present study was to verify the contribution of the respiratory exercising in the chest wall mobility, as well as the COPD patients' capacity of exercise and quality of life. Three moderate degree COPD patients were selected and submitted to a respiratory exercising program. The effects of this program were evaluated according to the following parameters: spirometry, thoracic cirtometry, a six-minute-walk test, and a quality- of-life questionnaire. After ten sessions of respiratory exercising, the patients presented a chest wall mobility increase and better quality of life while keeping their exercising capacity. Thus, respiratory exercising is one technique that functionally benefi ts the COPD patient.

Características clínicas a nivel domiciliario de los pacientes con EPOC atendidos en una institución de primer nivel de Risaralda / Clinical Characteristics on a Domestic Level of Patients with Chronic Obstructive Pulmonary Disease Attended to in a Primary Care Institution of Risaralda

La Enfermedad Pulmonar Obstructiva Crónica EPOC, es un proceso patológico que se caracteriza por obstrucción progresiva al flujo de aire; no es completamente reversible y se acompaña de alteración inflamatoria producida por la inhalación prolongada de gases nocivos. La obstrucción al flujo de aire se debe a una combinación variable de inflamación y remodelación de las vías aéreas periféricas, hipersecreción de moco y destrucción del parénquima pulmonar (enfisema), con disminución del retroceso elástico, hiperinsuflación, con cierre prematuro de las vías aéreas periféricas. Mediante este estudio se evaluaron las características clínicas a nivel domiciliario de los pacientes con enfermedad pulmonar obstructiva crónica, atendidos en una institución de primer nivel en un municipio de Risaralda, durante el 1er semestre de 2004. De 170 pacientes con EPOC de un hospital de 1er. nivel de Risaralda, se evaluaron 32 pacientes mediante encuesta aplicada en su vivienda, para analizar las características clínicas. Género mayoritario masculino 62.5 por cien, con mayor frecuencia se hospitalización; 81.2 por cien de los pacientes entre los 70 y 91 años de edad; exposición al tabaco (actual o previa) 60 por cien, y 46.7 por cien con animales domésticos en la vivienda. En cuanto la causa de la enfermedad el 61 por cien de la población refería el tabaquismo; el cambio de clima fue la razón más expuesta por los pacientes como causa de exacerbación en un 62.5 por cien; el uso de los inhaladores se calificó como incorrecto por parte de las investigadoras en un 76.6 por cien el número de hospitalizaciones fue de 80 por cien entre 1 a 3 veces en el último año; el 40.6 por cien tenía depresión al momento de la visita domiciliaria relacionada con el número de hospitalizaciones. (Valor de p: 0,1).

Evaluación del conocimiento sobre efecto del cigarrillo en internas fumadoras. Pereira 2004 / Evaluation of Inpatient Smokers’ Knowledge of the Effect of Cigarettes. Pereira 2004

El objetivo general de este estudio fue evaluar el nivel de conocimiento sobre los daños causados por el cigarrillo, en las internas que fuman en un Establecimiento Penitenciario y Carcelario de Mujeres de Pereira, durante el segundo semestre de 2004. Inicialmente se identificaron y cuantificaron las condenadas fumadoras, obteniendo como población estudio un total de 34 internas entre las 149 presentes en la institución. Luego se aplicó una encuesta semiestructurada, la cual permitió recolectar las variables: continuas (nombre, edad etc.) y discontinuas, divididas en variables dicotómicas (procedencia, ¿tiene hijos?) y discretas (escolaridad, cambios en el organismo desde que comenzó a fumar). La información recolectada se condensó en una base de datos en epiinfo, la cual se analizó en forma univariada; posteriormente se realizó análisis bivariado, entre las variables más representativas. Las principales limitaciones en el desarrollo del trabajo fueron la falta de cooperación de las internas, algunas de las cuales presentaron una actitud apática y desconfiada alterando los datos de la encuesta y ocasionando de esta manera sesgos de información, además del horario para la recolección de información, ya que se cruzaba con otras actividades realizadas por ellas o por los estudiantes. El 91 por cien de las internas conoce que el hábito del cigarrillo trae efectos adversos para la salud; de ese 91 por cien, 52 por cien cree que causa cáncer de pulmón; el 15 por cien que causa ‘enfermedad en los pulmones’. El análisis bivariado mostró que a mayor edad, mayor es el conocimiento acerca de los efectos que el cigarrillo ejerce sobre la salud y la relación con el número de cigarrillos consumidos diariamente. Las internas tienen algún conocimiento de los efectos adversos del cigarrillo, porque la mayoría aceptó la relación de éste con enfermedades; pero la concordancia es básicamente para problemas respiratorios, más no para alteraciones sistémicas; además creen que to...

Síndrome de morquio (mucopolisacaridosis tipo IV) y embarazo / Morquio syndrome (Mucopolysaccharidosis type IV) and pregnancy

Se presenta el caso de una paciente embarazada, portadora de Síndrome de Morquio (Mucopolisacaridosis tipo IV), que evoluciona con polihidroamnios y disnea. Se comenta el manejo y resolución del caso.