Sleep-related painful erection in a 50-year-old man successfully treated with cinitapride.

INTRODUCTION: The sleep-related painful erection (SRPE) is a well-established parasomnia characterized by episodes of penile pain during an erection and typically appears during REM sleep. It is associated with nocturnal awakenings, anxiety, and irritability. AIM: To report a case study that highlights the successful treatment of SRPE with cinitapride. METHODS: We present a case report of a 50-year-old man suffering from SRPE that was studied by polysomnography. RESULTS: Severe fragmentation of rapid eye movement (REM) sleep was observed, and nine episodes of sleep-related erections were observed through the night; they were associated with REM sleep, and five of them were classified as SRPE. Cinitapride before the onset of sleep was started. Both the frequency and intensity of SRPE gradually decreased during a period of 6 months with the maintenance of normal sexual function. CONCLUSION: Cinitapride can play a role in reducing SRPE at night probably due to central modulation of neurotransmitters mediating erection.

[Treatment of central sleep apnea syndrome of multifactorial origin by home ventilatory support]. / Síndrome de apnea central de origen multifactorial tratado mediante soporte ventilatorio domiciliario.

We report the case of a patient with chronic renal failure and primary hyperparathyroidism who developed nonhypercapnic central sleep apnea syndrome (CSAS), which was multifactorial in origin and attributed to metabolic factors. Given an inadequate response to oxygen therapy and continuous positive airway pressure (CPAP) revealed by several polygraph studies, the patient was treated with bilevel positive airway pressure ventilatory support. Three months after treatment commenced, a parathyroidectomy was performed and hemodialysis was initiated. At this point it was observed that the patient no longer experienced somnolence; moreover, polysomnography revealed partial improvement in the CSAS and normalization of ventilatory patterns on application of nasal CPAP at 7 cm H2O. We discuss the pathogenesis of CSAS associated with chronic kidney failure along with the treatment options and conclude that treatment should be customized due to the lack of predictability of patient response.

[Sleep apnea-hypopnea syndrome in a pediatric population: differences between children with tonsillar hypertrophy and those with concomitant disease]. / Síndrome de apneas-hipopneas durante el sueño en población infantil: diferencias en su expresión entre niños con hipertrofia amigdalar y con enfermedad concomitante.

OBJECTIVE: Our aim was to compare clinical and polysomnographic variables in pediatric patients with sleep apnea-hypopnea syndrome (SAHS) secondary to tonsillar hypertrophy with those in patients with concomitant disease. PATIENTS AND METHODS: We studied 42 children with SAHS (mean [SD] age, 8 [4] years; body mass index [BMI], 19.6 [5.2] kg/m2; neck circumference, 29 [4] cm; and BMI percentile, 67 [36]), 26 of whom were otherwise healthy (group A) and 16 of whom had concomitant disease (group B). RESULTS: A comparison of groups A and B showed no significant differences in age (7.7 [3.9] years vs 8.4 [3.9] years; P=not significant [NS]); sex, BMI (17.6 [4] kg/m2 vs 20.4 [6] kg/m2; P=NS), neck circumference (29.3 [4.7] cm vs 30.7 [3.5] cm; P=NS), or BMI percentile (61 [37] vs 76 [34]; P=NS). Tonsillar hypertrophy was more frequent in group A (P=.02) and craniofacial abnormalities (P=.008), macroglossia (P=.04), and dolichocephalia (P=.04) were more frequent in group B. No significant differences were observed in neurophysiologic variables or in the respiratory disturbance index, although group A presented higher oxygen saturation levels (97 [1.7] vs 95 [2]; P< .007), lower oxygen desaturation index scores (7 [7] vs 15 [10]; P=.007), and a lower cumulative percentage of time with oxygen saturation lower than 90% (2.2 [4] vs 16.4 [4]; P=.01). Twenty-three patients (88.5%) in group A underwent tonsillectomies compared to 7 (44%) patients in group B (P=.003). Seven patients (44%) in group B were treated with continuous positive airway pressure (CPAP) and 2 patients were treated with bi-level positive airway pressure (BiPAP), compared to 1 patient (3.8%) treated with CPAP in group A (P=.003). Three children in group B underwent maxillary surgery. The evolution of clinical and polygraphic variables was more favorable in group A (P=.04). CONCLUSIONS: Children with SAHS suffer from repeated infections, delayed weight gain, hyperactivity, and neuropsychiatric manifestations. Obesity (associated with concomitant disease) and sleepiness are uncommon. Although most patients require surgery, as many as a third require treatment with CPAP or BiPAP. Furthermore, children with SAHS and concomitant disease show no specific clinical characteristics, although they tend to be more obese, have more craniofacial abnormalities, and greater nocturnal hypoventilation.

Complementary home mechanical ventilation techniques. SEPAR Year 2014.

This is a review of the different complementary techniques that are useful for optimizing home mechanical ventilation (HMV). Airway clearance is very important in patients with HMV and many patients, particularly those with reduced peak cough flow, require airway clearance (manual or assisted) or assisted cough techniques (manual or mechanical) and suctioning procedures, in addition to ventilation. In the case of invasive HMV, good tracheostomy cannula management is essential for success. HMV patients may have sleep disturbances that must be taken into account. Sleep studies including complete polysomnography or respiratory polygraphy are helpful for identifying patient-ventilator asynchrony. Other techniques, such as bronchoscopy or nutritional support, may be required in patients on HMV, particularly if percutaneous gastrostomy is required. Information on treatment efficacy can be obtained from HMV monitoring, using methods such as pulse oximetry, capnography or the internal programs of the ventilators themselves. Finally, the importance of the patient's subjective perception is reviewed, as this may potentially affect the success of the HMV.

Noninvasive respiratory muscle aids during PEG placement in ALS patients with severe ventilatory impairment.

UNLABELLED: Although no clear recommendations are given about when percutaneous endoscopic gastrostomy (PEG) should be placed in amyotrophic lateral sclerosis (ALS) patients, some experts underline the risk of respiratory complications when patients had severe ventilatory muscle impairment (SVMI). AIM: To evaluate the efficacy of noninvasive ventilation (NIV) and mechanically assisted cough (MAC) to avoid respiratory complications related to PEG placement in ALS patients with SVMI. MATERIAL AND METHODS: Prospective study including ALS patients who had chosen to have PEG placement timed by swallowing dysfunction with the aid of NIV and MAC if needed. PEG was carried out under volume-cycled NIV through a nasal mask. MAC was applied prior to and at the end of the procedure. RESULTS: Thirty ALS patients (60.43±12.03years) were included. Prior to PEG placement: BMI 25.0±4.6kg/m(2), ALSRFS-R 19.5±5.0, Norris bulbar sub-score 15.1±6.6, %FVC 35.9±18.1%, PCF 2.3±1.2L/s, PImax -35.6±24.6cmH(2)O, and PEmax 40.5±23.9cmH(2)O. Three patients had PEG placement under tracheotomy ventilation because NIV SpO(2) was below 88%. No patient died during the procedure nor did any have respiratory complications. Survival at 1month was 100%. CONCLUSION: Respiratory support provided by volume-cycled NIV and MAC permits successful PEG placement in most ALS patients with SVMI.

Técnicas complementarias a la ventilación mecánica domiciliaria. Año SEPAR 2014 / Complementary Home Mechanical Ventilation Techniques. SEPAR Year 2014

La ventilación mecánica domiciliaria (VMD) precisa técnicas complementarias para optimizar el tratamiento, que son revisadas en el presente trabajo. El manejo de las secreciones respiratorias es muy importante, ya que muchos pacientes, particularmente cuando descienden los flujos de tos, precisan la aplicación de técnicas manuales o asistidas para el manejo de secreciones, técnicas de tos asistida (manual o mecánica) y aspiración mecánica de secreciones. Si se aplica la VMD de forma invasiva, el buen manejo de la cánula de traqueostomía es esencial para su éxito. Las alteraciones durante el sueño pueden estar presentes en estos pacientes, siendo un aspecto a valorar, estudiadas mediante polisomnografía o poligrafía respiratoria, que además ayudan a identificar las asincronías entre el paciente y el ventilador. Otras técnicas que pueden ser requeridas en pacientes con VMD son la fibrobroncoscopia o el soporte nutricional (especialmente si se requiere la colocación de una sonda de gastrostomía). La monitorización de la VMD aporta información sobre la efectividad del tratamiento aplicado. Existen varios métodos para ello, como pueden ser la pulsioximetría, la capnografía o los programas internos de los ventiladores. Finalmente, se revisa la importancia de la percepción subjetiva del paciente, ya que puede tener potencial influencia en el éxito de la VMD

This is a review of the different complementary techniques that are useful for optimizing home mechanical ventilation (HMV). Airway clearance is very important in patients with HMV and many patients, particularly those with reduced peak cough flow, require airway clearance (manual or assisted) or assisted cough techniques (manual or mechanical) and suctioning procedures, in addition to ventilation. In the case of invasive HMV, good tracheostomy cannula management is essential for success. HMV patients may have sleep disturbances that must be taken into account. Sleep studies including complete polysomnography or respiratory polygraphy are helpful for identifying patient-ventilator asynchrony. Other techniques, such as bronchoscopy or nutritional support, may be required in patients on HMV, particularly if percutaneous gastrostomy is required Information on treatment efficacy can be obtained from HMV monitoring, using methods such as pulse oximetry, capnography or the internal programs of the ventilators themselves. Finally, the importance of the patient’s subjective perception is reviewed, as this may potentially affect the success of the HMV

A controlled trial of noninvasive ventilation for chronic obstructive pulmonary disease exacerbations.

PURPOSE: This prospective, multicenter, double-blind, placebo-controlled study tested the hypothesis that noninvasive positive pressure ventilation reduces the need for endotracheal intubation in patients hospitalized in a pulmonary ward because of acute exacerbation of chronic obstructive pulmonary disease. MATERIALS AND METHODS: Seventy-five consecutive patients with exacerbation (pH, 7.31 +/- 0.02; Pao(2), 45 +/- 9 mm Hg; Paco(2), 69 +/- 13 mm Hg) were randomly assigned to receive noninvasive ventilation or sham noninvasive ventilation during the first 3 days of hospitalization on top of standard medical treatment. RESULTS: The need for intubation (according to predefined criteria) was lower in the noninvasive ventilation group (13.5% vs 34%, P < .01); in 31 patients with pH not exceeding 7.30, these percentages were 22% and 77%, respectively (P < .001). Arterial pH and Paco(2) improved in both groups, but changes were enhanced by noninvasive ventilation. Length of stay was lower in the noninvasive ventilation group (10 +/- 5 vs 12 +/- 6 days, P = .06). In-hospital mortality was similar in both groups. CONCLUSIONS: These results demonstrate that noninvasive positive pressure ventilation, in a pulmonary ward, reduces the need for endotracheal intubation, particularly in the more severe patients, and leads to a faster recovery in patients with acute exacerbation of chronic obstructive pulmonary disease.

Duchenne muscular dystrophy: prolongation of life by noninvasive ventilation and mechanically assisted coughing.

OBJECTIVE: To quantitate prolongation of survival for patients with Duchenne muscular dystrophy with the use of noninvasive intermittent positive-pressure ventilation (IPPV) with and without access to a protocol involving mechanically assisted coughing. DESIGN: In this retrospective review of all patients with Duchenne muscular dystrophy visiting a neuromuscular disease clinic, patients were trained to use mouth piece and nasal IPPV and mechanically assisted coughing to maintain oxyhemoglobin saturation >94% (protocol). Survival was considered prolonged when noninvasive IPPV was required full time. RESULTS: Ninety-one of 125 patients used noninvasive IPPV part time for 1.9 +/- 1.3 yr, and 51 went on to require it full time for 6.3 +/- 4.6 yr. Of the 31 noninvasive IPPV users who died without access to the protocol, 20 died from respiratory causes and seven died from cardiac causes. None of the 34 full-time noninvasive IPPV users with access to the protocol underwent tracheotomy or died from respiratory complications during a period of 5.4 +/- 4.0 yr, whereas three died from heart failure. Five patients with no breathing tolerance were extubated or decannulated to continuous noninvasive IPPV. CONCLUSIONS: Noninvasive respiratory aids can prolong survival and permit extubation or decannulation of patients with Duchenne muscular dystrophy with no breathing tolerance.

Obstructive sleep apnea syndrome as first manifestation of pharyngeal non-Hodgkin's lymphoma.

Obstructive sleep apnea syndrome (OSAS) is characterized by repetitive episodes of upper airway obstruction during sleep that provoke an abnormal number of apneas and hypopneas, leading to arousals and, as a result, to an altered sleep architecture. Here we present a patient with clinical symptoms characteristic of OSAS. During follow-up, pharyngeal non-Hodgkin's lymphoma was diagnosed and treated, with a nearly complete normalization of polysomnography.

Mechanical insufflation-exsufflation: pressure, volume, and flow relationships and the adequacy of the manufacturer's guidelines.

OBJECTIVE: Pulmonary complications of neuromuscular disease can be averted by increasing peak cough flows with the use of a forced exsufflation device. The purpose of this study was to examine the pressure, volume, and flow relationships for a range of settings generated by this device, and compare them with clinically efficacious values and the manufacturer's guidelines. METHODS: The In-exsufflator was connected to a standard lung model. The resulting forced deflation volumes, flows, and pressures were averaged over 10 cycles at each setting. RESULTS: The set insufflation pressures significantly correlated with the generated insufflation pressures and volumes and the exsufflation volumes and flows. Increasing set insufflation time significantly increased generated insufflation pressures, flows, and volumes and exsufflation volumes. Increasing set exsufflation time did not significantly increase generated exsufflation flows. At set pressures of 40 to -40 cm H2O, insufflation time of 3 sec, and exsufflation time of 2 sec, the exsufflation flow was 4.09 l/sec. A plateau insufflation volume of 3.8 l was reached after 4.9 sec of insufflation. CONCLUSIONS: In-exsufflator performance was very consistent. Its clinical effectiveness can be explained by its generation of exsufflation flows >2.7 l/sec. Increasing insufflation times more than exsufflation times is more important for optimal function. Current manufacturer use guidelines may not yield optimal exsufflation flows.

Síndrome de apneas-hipopneas durante el sueño en población infantil: diferencias en su expresión entre niños con hipertrofia amigdalar y con enfermedad concomitante / Sleep apnea-hypopnea syndrome in a pediatric population: differences between children with tonsillar hypertrophy and those with concomitant disease

Objetivo: Comparar la expresión clínica y polisomnográfica del síndrome de apneas-hipopneas durante el sueño (SAHS) en niños con hipertrofia amigdalar y enfermedad concomitante. Pacientes y métodos: Se estudió a 42 niños con SAHS ­con una edad media (± desviación estándar) de 8 ± 4 años, índice de masa corporal (IMC) de 19,6 ± 5,2 kg/m2, cuello de 29 ± 4 cm y percentil de IMC de 67 ± 36­, 26 sanos (grupo A) y 16 con enfermedad concomitante (grupo B). Resultados: Al comparar los grupos A y B no se observaron diferencias en la edad (7,7 ± 3,9 frente a 8,4 ± 3,9 años; p = no significativa [NS]), el sexo, el IMC (17,6 ± 4 frente a 20,4 ± 6 kg/m2; p = NS), el perímetro del cuello (29,3 ± 4,7 frente a 30,7 ± 3,5 cm; p = NS) ni el percentil de IMC (61 ± 37 frente a 76 ± 34; p = NS). En el grupo A fue más frecuente la hipertrofia amigdalar (p = 0,02), y en B, las alteraciones del macizo facial (p = 0,008), macroglosia (p = 0,04) y dolicocefalia (p = 0,04). No se observaron diferencias en las variables neurofisiológicas ni en el índice de alteración respiratoria, aunque el grupo A presentó mayor saturación de oxígeno basal (97 ± 1,7 frente a 95 ± 2%; p < 0,007), menor índice de desaturaciones/h (7 ± 7 frente a 15 ± 10; p = 0,007) y menor porcentaje de tiempo de sueño con saturación de oxihemoglobina inferior al 90% (2,2 ± 4 frente a 16,4 ± 4; p = 0,01). Fueron tratados con amigdalectomía 23 pacientes del grupo A (88,5%) frente a 7 (44%) del B (p = 0,003). En el grupo B, 7 pacientes recibieron tratamiento con presión positiva continua de la vía aérea (44%) y 2 con BiPAP®, frente a uno (3,8%) en el grupo A (p = 0,003). Se realizó cirugía maxilar a 3 niños del grupo B. La evolución clínica y poligráfica fue más favorable en el grupo A (p = 0,04). Conclusiones: Los niños con SAHS cursan con infecciones de repetición, retraso ponderal, hiperactividad y manifestaciones neuropsíquicas, mientras que son poco frecuentes la somnolencia y la obesidad, la cual se asocia a enfermedad concomitante. Aunque la mayoría necesitará cirugía, hasta un tercio precisará tratamiento con presión positiva continua de la vía aérea/BiPAP®. Además, los niños con SAHS y enfermedad concomitante no muestran características especiales en su expresión clínica, aunque tienden a ser más obesos, con mayores alteraciones del macizo facial y mayor hipoventilación nocturna

Objective: Our aim was to compare clinical and polysomnographic variables in pediatric patients with sleep apnea­hypopnea syndrome (SAHS) secondary to tonsillar hypertrophy with those in patients with concomitant disease. Patients and methods: We studied 42 children with SAHS (mean [SD] age, 8 [4] years; body mass index [BMI], 19.6 [5.2] kg/m2; neck circumference, 29 [4] cm; and BMI percentile, 67 [36]), 26 of whom were otherwise healthy (group A) and 16 of whom had concomitant disease (group B). Results: A comparison of groups A and B showed no significant differences in age (7.7 [3.9] years vs 8.4 [3.9] years; P=not significant [NS]); sex, BMI (17.6 [4] kg/m2 vs 20.4 [6] kg/m2; P=NS), neck circumference (29.3 [4.7] cm vs 30.7 [3.5] cm; P=NS), or BMI percentile (61 [37] vs 76 [34]; P=NS). Tonsillar hypertrophy was more frequent in group A (P=.02) and craniofacial abnormalities (P=.008), macroglossia (P=.04), and dolichocephalia (P=.04) were more frequent in group B. No significant differences were observed in neurophysiologic variables or in the respiratory disturbance index, although group A presented higher oxygen saturation levels (97 [1.7] vs 95 [2]; P<.007), lower oxygen desaturation index scores (7 [7] vs 15 [10]; P=.007), and a lower cumulative percentage of time with oxygen saturation lower than 90% (2.2 [4] vs 16.4 [4]; P=.01). Twenty-three patients (88.5%) in group A underwent tonsillectomies compared to 7 (44%) patients in group B (P=.003). Seven patients (44%) in group B were treated with continuous positive airway pressure (CPAP) and 2 patients were treated with bilevel positive airway pressure (BiPAP), compared to 1 patient (3.8%) treated with CPAP in group A (P=.003). Three children in group B underwent maxillary surgery. The evolution of clinical and polygraphic variables was more favorable in group A (P=.04). Conclusions: Children with SAHS suffer from repeated infections, delayed weight gain, hyperactivity, and neuropsychiatric manifestations. Obesity (associated with concomitant disease) and sleepiness are uncommon. Although most patients require surgery, as many as a third require treatment with CPAP or BiPAP. Furthermore, children with SAHS and concomitant disease show no specific clinical characteristics, although they tend to be more obese, have more craniofacial abnormalities, and greater nocturnal hypoventilation

Síndrome de apnea central de origen multifactorial tratado mediante soporte ventilatorio domiciliario / Treatment of central sleep apnea syndrome of multifactorial origin by home ventilatory support

Se describe el caso de una paciente afectada de insuficiencia renal crónica e hiperparatiroidismo primario, que presentó síndrome de apnea central (SAC) no hipercápnico, de origen multifactorial, que se atribuyó a factores metabólicos. Fue tratada inicialmente con soporte ventilatorio bipresión tras comprobar, mediante diversos estudios poligráficos, la respuesta inadecuada a oxigenoterapia y presión positiva continua de la vía aérea (CPAP). Tres meses más tarde, tras paratiroidectomía e inicio de hemodiálisis, se observaron la desaparición de la somnolencia, mejoría parcial del SAC en la polisomnografía y normalización del patrón ventilatorio al aplicar CPAP nasal a 7 cmH2O. Se discute la patogenia del SAC asociado a insuficiencia renal crónica, así como las opciones de tratamiento, que en cualquier caso debe ser individualizado, debido a su respuesta imprevisible

We report the case of a patient with chronic renal failure and primary hyperparathyroidism who developed nonhypercapnic central sleep apnea syndrome (CSAS), which was multifactorial in origin and attributed to metabolic factors. Given an inadequate response to oxygen therapy and continuous positive airway pressure (CPAP) revealed by several polygraph studies, the patient was treated with bilevel positive airway pressure ventilatory support. Three months after treatment commenced, a parathyroidectomy was performed and hemodialysis was initiated. At this point it was observed that the patient no longer experienced somnolence; moreover, polysomnography revealed partial improvement in the CSAS and normalization of ventilatory patterns on application of nasal CPAP at 7 cm H2O. We discuss the pathogenesis of CSAS associated with chronic kidney failure along with the treatment options and conclude that treatment should be customized due to the lack of predictability of patient response