Revisión del tratamiento del síndrome obesidad hipoventilación / Review of the treatment of obesity hypoventilation syndrome

El síndrome de obesidad hipoventilación (SOH) se define como la coexistencia de obesidad (índice de masa corporal≥ 30 kg/mg²), alteraciones respiratorias durante el sueño e hipoventilación alveolar crónica que acarrea hipercapnia diurna(pCO2 ≥ 45 mmHg), cuando otras causas de hipoventilación han sido excluidas. Los pilares de tratamiento para el SOH sonla pérdida de peso y la presión positiva en la vía aérea (PAP) durante el sueño. El manejo de estos pacientes ha de sermultidisciplinario, con modificación de hábitos de vida, intensificación de la actividad física y estrategias de intervenciónfarmacológica o quirúrgica para la pérdida de peso, individualizando las terapias según cada paciente y sus comorbilidades.El modo de inicio de la PAP dependerá de la coexistencia de apnea obstructiva del sueño (AOS), considerando que apro-ximadamente el 90% de los pacientes con SOH presentan concomitantemente AOS, aunque de estos el 73% se consideraAOS grave. En aquellos pacientes con SOH y AOS grave en situación de estabilidad clínica se inclinará por inicio conpresión positiva continua en la vía aérea. Por otra parte, en aquellos pacientes sin AOS grave o durante una hospitalización,se recomienda iniciar directamente ventilación mecánica no invasiva. El seguimiento del paciente debe contemplar loscambios de estrategia necesarios para intentar un control óptimo del peso y el cumplimiento de la PAP.(AU)

Obesity hypoventilation syndrome (OHS) is defined as the coexistence of obesity (body mass index ≥ 30 kg/mg²), respiratorydisturbances during sleep and chronic alveolar hypoventilation leading to daytime hypercapnia (pCO2 ≥ 45 mmHg), whenother causes of hypoventilation have been excluded. The mainstays of treatment for OHS are weight loss and positive airwaypressure (PAP) during sleep. The management of these patients must be multidisciplinary, with modification of lifestyle habits,intensification of physical activity and pharmacological or surgical intervention strategies for weight loss, individualizing thera-pies according to each patient and their comorbidities. The mode of onset of PAP will depend on the coexistence of obstruc-tive sleep apnea (OSA), considering that approximately 90% of patients with OHS present concomitant OSA, although 73%of these are considered severe OSA. In those patients with OHS and severe OSA in a situation of clinical stability, it is recom-mended to start with continuous positive airway pressure. On the other hand, it is recommended to start non invasive mecha-nical ventilation directly for those patients without severe OSA or during hospitalization. The follow-up of the patient mustconsider the necessary changes in strategy to try to achieve optimal weight control and compliance with the PAP.(AU)

The Overlap of Obesity-Hypoventilation Syndrome and Obstructive Sleep Apnea: How to Treat?

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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.

Narcotic antagonist therapy of the obesity hypoventilation syndrome.

A child with respiratory failure and cor pulmonale secondary to the obesity hypoventilation syndrome (OHS) was found to have abnormal beta-endorphin levels in cerebrospinal fluid (CSF) and serum. A single iv dose of 10 microgram/kg of naloxone early in the course of respiratory failure resulted in dramatic improvement which lasted approximately 3 to 4 h. The patient failed to response to progesterone, and because of deteriorating respiratory status a low-dose continuous infusion of naloxone, 2 microgram/kg.h, was begun and gradually increased to 10 microgram/kg.h, during which time there was a dramatic improvement in respiratory status and clinical condition. After 5 days, naloxone infusion was discontinued and progressive respiratory deterioration recurred. The child died of over-whelming sepsis and disseminated intravascular coagulation.

Progesterone for outpatient treatment of Pickwickian syndrome.

Ten patients with the Pickwickian syndrome, characterized by obesity, hypoxemia, hypercapnia, polycythemia, and cor pulmonale, underwent long-term treatment as outpatients with medroxyprogesterone acetate. Although there was no significant weight change in the group, PaO2 rose 12.6 +/- 2.7 mm Hg (SEM) from 49 +/- 2.6 mm Hg to 62 +/- 2.3 mm Hg (P less than 0.001), while PaCO2 fell 13 +/- 2.6 mm Hg from 51 +/- 1.9 mm Hg to 38 +/- 1.2 mm Hg (P less than 0.001). Hematocrit fell from 56 +/- 2.5% to 50 +/- 1.2%, a mean fall of 6% (P less than 0.01), during medroxyprogesterone acetate therapy. In the 2 patients who had cardiac catheterization before and during medroxyprogesterone acetate therapy, mean pulmonary arterial pressure fell 13 and 19 mm Hg. There were no recurrences of cor pulmonale during treatment. These effects on arterial blood gas values and clinical state were sustained during therapy. On withdrawal of medroxyprogesterone acetate during 1-month period, arterial oxygen and carbon dioxide tensions deteriorated to their previous pretreatment values. Reinstitution of medroxyprogesterone acetate caused improvement in both the oxygen and carbon dioxide tensions. We conclude that sublingual medroxyprogesterone acetate therapy is useful in the management of the Pickwickian syndrome.