Effectiveness of different treatments in obesity hypoventilation syndrome.

Obesity hypoventilation syndrome (OHS) is an undesirable consequence of obesity, defined as daytime hypoventilation, sleep disorder breathing and obesity; during the past few years the prevalence of extreme obesity has markedly increased worldwide consequently increasing the prevalence of OHS. Patients with OHS have a lower quality of life and a higher risk of unfavourable cardiometabolic consequences. Early diagnosis and effective treatment can lead to significant improvement in patient outcomes; therefore, such data has noticeably raised interest in the management and treatment of this sleep disorder. This paper will discuss the findings on the main current treatment modalities OHS will be discussed.

Obesity hypoventilation syndrome: a current review / Síndrome de obesidade-hipoventilação: uma revisão atual

ABSTRACT Obesity hypoventilation syndrome (OHS) is defined as the presence of obesity (body mass index ≥ 30 kg/m²) and daytime arterial hypercapnia (PaCO2 ≥ 45 mmHg) in the absence of other causes of hypoventilation. OHS is often overlooked and confused with other conditions associated with hypoventilation, particularly COPD. The recognition of OHS is important because of its high prevalence and the fact that, if left untreated, it is associated with high morbidity and mortality. In the present review, we address recent advances in the pathophysiology and management of OHS, the usefulness of determination of venous bicarbonate in screening for OHS, and diagnostic criteria for OHS that eliminate the need for polysomnography. In addition, we review advances in the treatment of OHS, including behavioral measures, and recent studies comparing the efficacy of continuous positive airway pressure with that of noninvasive ventilation.

RESUMO A síndrome de obesidade-hipoventilação (SOH) é definida pela presença de obesidade (índice de massa corpórea ≥ 30 kg/m2) e hipercapnia arterial diurna (PaCO2 ≥ 45 mmHg), na ausência de outras causas. A SOH é frequentemente negligenciada e confundida com outras patologias associadas à hipoventilação, em particular à DPOC. A importância do reconhecimento da SOH se dá por sua elevada prevalência, assim como alta morbidade e mortalidade se não tratada. Na presente revisão, abordamos os recentes avanços na fisiopatologia e no manejo da SOH. Revisamos a utilidade da medição do bicarbonato venoso como rastreamento e os critérios diagnósticos que descartam a necessidade de polissonografia. Destacamos ainda os avanços no tratamento da SOH, incluindo medidas comportamentais, e estudos recentes que comparam a eficácia do uso de pressão positiva contínua nas vias aéreas e de ventilação não invasiva.

[Obesity hypoventilation syndrome and pulmonary hypertension. An association little-known in Mexico]. / El síndrome de hipoventilación del obeso y la hipertensión pulmonar. Una asociación poco conocida en México.

The obesity hypoventilation syndrome (OHS) refers to the combination of obesity, daytime hypercapnia and sleep-disordered breathing. Obesity has risen to epidemic proportions in the last three decades in the United States, Mexico and Europe. The OHS is associated with obstructive sleep apnea syndrome in 30%. Without treatment, mortality is 46% at 50 months. So in this paper we analyze the OHS, obesity and pulmonary hypertension, the pathophysiology, clinical presentation and diagnosis as well as the treatment, which is aimed at the correction of sleep-disordered breathing and hypoxemia; although there is little experience with the use of specific pulmonary vasodilator drugs.

El síndrome de hipoventilación del obeso (SHO) se refiere a la combinación de obesidad, hipercapnia diurna y trastornos respiratorios del sueño. La obesidad ha aumentado con características epidémicas en las últimas tres décadas en Estados Unidos, México y Europa. El SHO se asocia al síndrome de apnea obstructiva del sueño hasta en el 30%. Sin tratamiento, la mortalidad es de 46% a 50 meses. Por lo que en este trabajo se analizan el SHO, la obesidad y la hipertensión pulmonar, la fisiopatología, la presentación clínica y el diagnóstico, además del tratamiento, el cual está orientado a la corrección de los trastornos respiratorios del sueño y la hipoxemia; aún hay poca experiencia con el uso de medicamentos vasodilatadores pulmonares específicos.

Obesity hypoventilation syndrome: a current review.

Obesity hypoventilation syndrome (OHS) is defined as the presence of obesity (body mass index ≥ 30 kg/m²) and daytime arterial hypercapnia (PaCO2 ≥ 45 mmHg) in the absence of other causes of hypoventilation. OHS is often overlooked and confused with other conditions associated with hypoventilation, particularly COPD. The recognition of OHS is important because of its high prevalence and the fact that, if left untreated, it is associated with high morbidity and mortality. In the present review, we address recent advances in the pathophysiology and management of OHS, the usefulness of determination of venous bicarbonate in screening for OHS, and diagnostic criteria for OHS that eliminate the need for polysomnography. In addition, we review advances in the treatment of OHS, including behavioral measures, and recent studies comparing the efficacy of continuous positive airway pressure with that of noninvasive ventilation.

Cyclophosphamide for rapid-onset obesity, hypothalamic dysfunction, hypoventilation, and autonomic dysregulation syndrome.

Patients with rapid-onset obesity, hypothalamic dysfunction, hypoventilation, autonomic dysregulation, and neural crest tumor syndrome have poor long-term outcomes. We report a patient who was treated successfully with high-dose cyclophosphamide immunoablation. This experience offers a novel therapeutic approach and an indirect insight into the underlying pathogenesis of this syndrome.

[Sleep hypoventilation]. / Hipoventilação relacionada ao sono.

Sleep hypoventilation is seen in patients with neuromuscular disease, as well as in those with obesity hypoventilation syndrome (OHS), which is defined as the combination of obesity, chronic hypercapnia, and hypoxemia during wakefulness that is aggravated during sleep. In 90% of cases, OHS is accompanied by obstructive sleep apnea. The diagnosis of OHS is based on hypoventilation and pulmonary hypertension that cannot be explained by alterations in pulmonary function. The mortality of patients with OHS is greater than is that of obese patients without hypoventilation. The principal neuromuscular diseases associated with OHS are the muscular dystrophies. The progression to chronic respiratory failure results from respiratory muscle weakness and impaired airway secretion clearance, causing atelectasis and pneumonia. With a decrease of greater than 50% in respiratory muscle strength, there is a reduction in VC. Cough peak flow < 160 L/min is associated with impaired airway secretion clearance, and values near 270 L/min indicate the need for assisted cough techniques. Obstructive sleep apnea usually worsens sleep hypoventilation. Noninvasive ventilation during sleep can improve survival, symptoms, and hypoventilation during wakefulness, as well as being able to improve pulmonary function in patients with neuromuscular disease. Patients with OHS can require oxygen therapy.

Hipoventilação relacionada ao sono / Sleep hypoventilation

Tanto SHO como as doenças neuromusculares estão relacionadas à hipoventilação durante o sono. Define-se SHO como a combinação de obesidade, hipercapnia e hipoxemia crônica durante a vigília que se agrava durante o sono. Em 90 por cento dos casos, SHO está associada à apneia obstrutiva do sono. O diagnóstico baseia-se na presença de hipoventilação diurna e hipertensão pulmonar que não são justificadas por alterações da função pulmonar. A mortalidade dos pacientes com SHO é maior que aquela de pacientes sem hipoventilação e controlados para obesidade. As doenças neuromusculares são representadas principalmente pelas distrofias musculares. A progressão para insuficiência respiratória crônica surge como consequência da fraqueza dos músculos respiratórios e da limpeza inadequada das vias aéreas, causando atelectasias e pneumonias. Quando há uma redução maior que 50 por cento da forca muscular respiratória, ocorre uma diminuição na CV. A medida do pico de fluxo da tosse < 160 L/min está associada à limpeza inadequada das vias aéreas, e, com valores em torno de 270 L/min, há indicação de uso de técnicas de tosse assistida. A apneia obstrutiva do sono geralmente agrava a hipoventilação durante o sono. O suporte pressórico não invasivo durante a noite pode aumentar a sobrevida, melhorar os sintomas e a hipoventilação diurna. Além disso, no caso de doenças neuromusculares, pode diminuir o declínio da função pulmonar. A oxigenoterapia pode ser necessária nos casos de SHO.

Sleep hypoventilation is seen in patients with neuromuscular disease, as well as in those with obesity hypoventilation syndrome (OHS), which is defined as the combination of obesity, chronic hypercapnia, and hypoxemia during wakefulness that is aggravated during sleep. In 90 percent of cases, OHS is accompanied by obstructive sleep apnea. The diagnosis of OHS is based on hypoventilation and pulmonary hypertension that cannot be explained by alterations in pulmonary function. The mortality of patients with OHS is greater than is that of obese patients without hypoventilation. The principal neuromuscular diseases associated with OHS are the muscular dystrophies. The progression to chronic respiratory failure results from respiratory muscle weakness and impaired airway secretion clearance, causing atelectasis and pneumonia. With a decrease of greater than 50 percent in respiratory muscle strength, there is a reduction in VC. Cough peak flow < 160 L/min is associated with impaired airway secretion clearance, and values near 270 L/min indicate the need for assisted cough techniques. Obstructive sleep apnea usually worsens sleep hypoventilation. Noninvasive ventilation during sleep can improve survival, symptoms, and hypoventilation during wakefulness, as well as being able to improve pulmonary function in patients with neuromuscular disease. Patients with OHS can require oxygen therapy.

Obesidad: una epidemia del siglo XXI / Obesity: an epidemic of the 21st century

La OMS considera a la obesidad como la epidemia del siglo XXI, con alta prevalencia en la población pediátrica. La obesidad, además del sustrato biológico está ligada a factores culturales, acceso a alimentos y cambios en el modo de comer de ciertas sociedades. Salvo en un pequeño porcentaje, la obesidad no responde a una etiología única; es de origen multifactorial. No es una enfermedad en sí, pero es un factor de riesgo para numerosas patologías, tanto de la niñez como del adulto. Se describe la obsidad extrema o Síndrome de Picwick, así como algunas enfermedades que causan obesidad. Se mencionan algunos conocimientos actuales sobre fisiopatología, diagnóstico y trartamiento, así como las dificultades terapéuticas y sus frecuentes fallos en el largo plazo. Se enfatiza finalmente la importancia de la prevención y la responsabilidad del equipo de salud en la misma, especialmente en la edad pediátrica (AU)