Showing papers on "Hypoventilation published in 2007"

Rapid-Onset Obesity With Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation Presenting in Childhood

Abstract: OBJECTIVE.The goal was to characterize the phenotype and potential candidate genes responsible for the syndrome of late-onset central hypoventilation with hypothalamic dysfunction. METHODS.Individuals with late-onset central hypoventilation with hypothalamic dysfunction who were referred to Rush University Medical Center for clinical or genetic assessment in the past 3 years were identified, and medical charts were reviewed to determine shared characteristics of the affected subjects. Blood was collected for genetic testing of candidate genes (PHOX2B, TRKB, and BDNF) and for high-resolution conventional G-banding, subtelomeric fluorescent in situ hybridization, and comparative genomic hybridization analysis. A subset of these children were studied in the Pediatric Respiratory Physiology Laboratory at Rush University Medical Center. RESULTS.Twenty-three children with what we are now naming rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation were identified. Comprehensive medical charts and blood for genetic testing were available for 15 children; respiratory physiology studies were performed at Rush University Medical Center on 9 children. The most characteristic manifestations were the presentation of rapid-onset obesity in the first 10 years of life (median age at onset: 3 years), followed by hypothalamic dysfunction and then onset of symptoms of autonomic dysregulation (median age at onset: 3.6 years) with later onset of alveolar hypoventilation (median age at onset: 6.2 years). Testing of candidate genes (PHOX2B, TRKB, and BDNF) revealed no mutations or rare variants. High-resolution chromosome analysis, comparative genomic hybridization, and subtelomeric fluorescent in situ hybridization results were negative for the 2 patients selected for those analyses. CONCLUSIONS.We provide a comprehensive description of the clinical spectrum of rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and auto

Respiratory function during parenteral opioid titration for cancer pain.

Abstract: Background: Respiratory depression is the most feared opioid-related side-effect yet research on the topic is sparse. We evaluated changes in respiratory parameters during parenteral opioid titration for cancer pain to determine if opioid titration was associated with evidence of hypoventilation. The primary outcome measure was to measure changes in end-tidal CO2 (ET-CO2) during opioid titration to pain control.Methods: Subjects with severe cancer pain admitted for parenteral opioid titration for poorly controlled pain were eligible. Those who were oxygen dependent were excluded. ET-CO2, O2 saturation, respiratory rate (RR), and vital signs were monitored daily until pain control was achieved.Results: 30 patients completed the study of which 29 are reported. The mean ET-CO2 at initial evaluation was 33.39 ∓ 5.0 and 34.79 ∓ 5.7 mmHg at pain control (P =0.14, 95% CI -0.5 to 3.3). None had an ET-CO2 ≥50 mmHg. All maintained O2 saturation ≥92%. RR dropped transiently below 10/minute in two subjects.Conclusion...

Previously undiagnosed obesity hypoventilation syndrome

Abstract: There are approximately 300 million obese individuals (body mass index (BMI) 30 kg/m2 or higher) worldwide,1 and in the UK nearly one quarter of all adults are classified as clinically obese.2 Obesity hypoventilation syndrome (OHS) describes a subgroup of obese individuals who develop chronic daytime hypercapnia (arterial carbon dioxide tension (Paco2) >6 kPa) and hypoxia (arterial oxygen tension (Pao2) <8 kPa) in the absence of chronic obstructive pulmonary disease (COPD).3,4 Presentation is usually indolent, with symptoms arising due to hypercapnia and sustained hypoventilation (hypersomnolence, alterations in cognitive function, headache, peripheral oedema, hypertension, congestive cardiac failure).5 At Southend Hospital we have noticed an increase in acute admissions in obese individuals with type II respiratory failure of initially unknown cause in whom a diagnosis of OHS was eventually made. We collected data on 11 patients (seven men) diagnosed with OHS from 1996 to 2005 from the respiratory disease register. Patients with possible overlap syndrome were excluded (smokers with forced expiratory volume in 1 s/forced vital capacity (FEV1/FVC) ratio <70%). Patient demographics, lung function and Epworth sleep score (ESS) were documented. The results of initial sleep studies on air were analysed. Initial management was recorded and follow-up data were reviewed regarding ESS, blood gases, long-term use of continuous positive airway pressure (CPAP) or non-invasive ventilation (NIV, using bi-level pressure support ventilation). The mean (SD) age of the 11 patients was 59 (12) years and the mean (SD) BMI was 52.7 (16.6) kg/m2 (range 37–102). Two patients were current smokers, one an ex-smoker and …

New modes of mechanical ventilation in the preterm newborn: evidence of benefit.

Abstract: The introduction of modern mechanical ventilation in neonatal medicine in the 1960s was followed shortly thereafter by its use in premature infants with hyaline membrane disease. Most premature infants born before 30 weeks’ gestation receive some form of respiratory support, particularly those with fewer weeks of gestation.1 Although mechanical ventilation is frequently a life-saving therapy, its use increases the risk of lung injury, particularly in preterm infants in whom the incidence of bronchopulmonary dysplasia (BPD) remains high.2 Before the current generation of neonatal ventilators, conventional mechanical ventilation (CMV) was provided mainly with time-cycled pressure limited (TCPL) ventilators developed from adaptation of Ayre’s T piece.3 This method, also known as intermittent mandatory ventilation (IMV), was and probably still is in many centres, the most common mode of ventilation. During IMV mechanical breaths of fixed duration are delivered at predetermined time intervals. This frequently leads to asynchrony depending on the phase of the spontaneous breath when these IMV breaths are delivered. Inspiratory asynchrony occurring when a mechanical breath is delivered at the end of and extends beyond spontaneous inspiration can produce an inspiratory hold that limits the spontaneous respiratory rate or results in excessive lung inflation. Expiratory asynchrony occurring when a mechanical breath is delivered during exhalation can delay lung deflation and elicit active expiratory efforts against positive pressure producing large fluctuations in intrathoracic pressure. Asynchrony can affect gas exchange, and has been linked to increased risk of air leaks4 5 and intraventricular haemorrhage (IVH).6 As volume monitoring was lacking in most IMV devices, it was difficult to detect excessive lung inflation, gas trapping or hypoventilation. Advances in ventilator technology allowed mechanical breaths to be synchronised with the onset of spontaneous inspiration. This was achieved by using signals derived from spontaneous respiratory activity. Synchronisation was also extended …

Clinical review: long-term noninvasive ventilation.

Abstract: Noninvasive positive ventilation has undergone a remarkable evolution over the past decades and is assuming an important role in the management of both acute and chronic respiratory failure. Long-term ventilatory support should be considered a standard of care to treat selected patients following an intensive care unit (ICU) stay. In this setting, appropriate use of noninvasive ventilation can be expected to improve patient outcomes, reduce ICU admission, enhance patient comfort, and increase the efficiency of health care resource utilization. Current literature indicates that noninvasive ventilation improves and stabilizes the clinical course of many patients with chronic ventilatory failure. Noninvasive ventilation also permits long-term mechanical ventilation to be an acceptable option for patients who otherwise would not have been treated if tracheostomy were the only alternative. Nevertheless, these results appear to be better in patients with neuromuscular/-parietal disorders than in chronic obstructive pulmonary disease. This clinical review will address the use of noninvasive ventilation (not including continuous positive airway pressure) mainly in diseases responsible for chronic hypoventilation (that is, restrictive disorders, including neuromuscular disease and lung disease) and incidentally in others such as obstructive sleep apnea or problems of central drive.

Anesthetic implications of undiagnosed late onset central hypoventilation syndrome in a child: from elective tonsillectomy to tracheostomy.

Abstract: Late onset central hypoventilation syndrome is a neurological disorder that can present with postoperative respiratory complications and delayed emergence in children after anesthesia. We present a child who had unanticipated respiratory complications following an elective tonsillectomy who eventually required a tracheostomy and long-term ventilatory support.

Central hypoventilation with PHOX2B expansion mutation presenting in adulthood

Abstract: Congenital central hypoventilation syndrome most commonly presents in neonates with sleep related hypoventilation; late onset cases have occurred up to the age of 10 years. It is associated with mutations in the PHOX2B gene, encoding a transcription factor involved in autonomic nervous system development. The case history is described of an adult who presented with chronic respiratory failure due to PHOX2B mutation-associated central hypoventilation and an impaired response to hypercapnia.

Long-term respiratory support in children with giant omphalocele.

Abstract: Omphalocele is one of the most common fetal abdominal wall defects. When this defect is of giant size, significant respiratory compromise may occur and impact on prognosis. We present three infants with giant omphalocele, highlighting the potential need for ongoing ventilatory support after the neonatal period in children born with this condition. The three cases had very different outcomes but all had significant ventilatory insufficiency and required substantial respiratory support at least into the second year of life. The possibility of a requirement for long-term ventilatory support should be discussed with families at antenatal diagnosis. A conservative surgical approach, together with early monitoring for hypoventilation and screening for the development of pulmonary hypertension is indicated for these children to limit morbidity. We suggest early tertiary respiratory input and advocate for a specific case manager to oversee the regional care of these children.

Respiratory disturbance during sleep in COPD patients without daytime hypoxemia.

Abstract: Chronic obstructive pulmonary disease (COPD) is associated with significant morbidity and mortality. Its possible association with obstructive sleep apnea is a major cause of concern for clinicians. As the prevalence of both COPD and sleep apnea continues to rise, further investigation of this interaction is needed. In addition, COPD patients are at risk for hypoventilation during sleep due to the underlying respiratory dysfunction. In this study, 13 COPD subjects and 13 non-COPD control subjects were compared for the presence and severity of obstructive sleep apnea and nocturnal hypoventilation. All 26 subjects had presented to a sleep clinic and showed no signs of daytime hypoxemia. After matching for BMI and age, COPD subjects had a similar prevalence of sleep apnea with a lower degree of severity compared to the control subjects. However, less severe events, such as RERA, occurred at similar rates between the two groups. There was no significant difference between groups in the magnitude of oxyhemoglobin desaturation during sleep. Interestingly, severity and presence of nocturnal hypoxemia correlated with that of sleep apnea in the control group, but not in the COPD subjects. In conclusion, COPD without daytime hypoxemia was not a risk factor for sleep apnea or nocturnal hypoventilation in this study.

Histamine provocation in young, awake children with bronchial asthma, using a fall in oxygenation as the only indicator of a bronchial reaction.

Abstract: Bronchial provocation with histamine was performed in 11 boys and 6 girls, age range 27-74 y, with unspecific respiratory symptoms or bronchial asthma, using a fall in oxygenation as the only indicator of a bronchial reaction In addition to transcutaneous oxygen tension (tcPO2), transcutaneous carbon dioxide tension (tcPCO2) was continuously monitored during the provocation procedure in order to identify possible changes in ventilation A fall of 20% or more in the tcPO2 below a “floating” baseline value, defined as the highest tcPO2 value between the inhalations of histamine up to that point, was regarded as indicating a significant bronchial reaction One child was excluded from the study because of an “early, false-positive” reaction due to hyperventilation during the inhalation, verified by a decrease in the tcPCO2 followed by a compensatory period of hypoventilation, resulting in a fall of more than 15% in the tcPO2 after the inhalation of saline In the vast majority of the children, however, the tcPO2 values remained stable during the first dose stages of saline and histamine, with either a gradual fall immediately before or a distinct fall in conjunction with the reaction The mean reaction concentration was significantly lower in the group of children with clinical asthma, 074 mg/ml, compared with the group of children with unspecific respiratory symptoms, 200 mg/ml (p = 003) In conclusion, a 20% fall in the tcPO2 can be used as the only indicator of a bronchial reaction during bronchial provocation tests in young, awake children Changes in ventilation evaluated by monitoring tcPCO2, makes it possible to distinguish between a fall in oxygen tension due to an early, “false” reaction as a result of hypoventilation and a “true” bronchial reaction □Asthma, bronchial provocation, oxygenation, young children

Broken O-ring causing hypoventilation.

Abstract: The following equipment failure resulted in underventilation and CO, retention in an obstetric patient undergoing Caesarean section. A 19-year-old, 135 kg smoker was scheduled for an elective Caesarean section. After premedication with ranitidine a routine rapid sequence induction was performed using thiopentone and suxamethonium. The trachea was intubated with an 8 mm Oxford tracheal tube and anaesthesia was maintained with enflurane and nitrous oxide (50%) in oxygen. The anaesthetic gases were delivered by an Ohmeda Excel 410 anaesthetic machine via a Bain breathing system. After confirmation of correct placement of the tracheal tube by manual ventilation, inspection of the chest and of the capnograph trace, the patient’s lungs were ventilated by a Penlon Nuffield 20 ventilator. Neuromuscular block was maintained with vecuronium. Monitoring included pulse oximetry, inspired oxygen concentration, capnography, ECG and automated noninvasive blood pressure. Shortly after surgery had commenced, the end-tidal carbon dioxide level was noted to be increasing (up to 10 kPa) with an associated rise in the inspired C02. All other measurements, including oxygen saturation remained unremarkable The patient’s chest was moving adequately and symmetrically, with bilateral air entry and no abnormal sounds on ausculation. The lung inflation pressure had not changed; the tracheal tube was checked for position and leaks by direct laryngoscopy. Meanwhile, the Bain system was changed for a new one, the ventilator disconnected, and an attempt was made to ventilate the lungs manually using the reservoir bag. It was then immediately apparent that there was an almost complete failure of fresh gas flow at the common gas outlet despite an unchanged Rotameter reading and the oxygen analyser still registering 50% concentration. A replacement anaesthetic machine was urgently requested. Oxygenation was maintained by repeatedly using the oxygen bypass button to fill the reservoir bag between manual ventilations. Anaesthesia was maintained with intermittent boluses of propofol. The anaesthetic machine was checked again and eventually a soft hiss was detected from the enflurane vaporizer manifold. The vaporizer, an Ohmeda ‘Tec 4, was removed, carefully re-sited, and turned on again. This appeared to resolve the problem: the leak vanished, fresh gas flow returned, and it was possible to maintain normocapnia with appropriate Rotameter settings. However, some minutes later the same fault recurred. The replacement anaesthetic machine was connected immediately and the remainder of the anaesthetic proceeded uneventfully. The baby was delivered in good condition and the mother made an uneventful recovery with no recall of any intra-operative events. Detailed subsequent inspection of the faulty anaesthetic machine revealed that the cause of the problem was a ruptured O-ring seal on the vaporizer manifold which was causing a large but apparently intermittent leak of fresh gas (Fig. 1). An absent O-ring has been previously reported as the cause of a leak from the machine back-bar [l]. It is salutary that the O-ring in the incident which we report failed in such a way that allowed the machine to pass the preanaesthetic occlusion test recommended by the Association Fig. 1.

A full term infant with cyanotic episodes. Congenital central hypoventilation syndrome.

Abstract: Disclosure Statement This was not an industry supported study. Dr. Berry has received research from Itamar Medicine. Dr. Wagner has indicated no financial conflicts of interest.

[Ventilatory dysfunction in motor neuron disease: when and how to act?].

Abstract: Amyotrophic lateral sclerosis is a devastating progressive neurodegenerative disorder, involving motor neurons in the cerebral cortex, brainstem and spinal cord. Mean duration of survival from the time of diagnosis is around 15 months, being pulmonary complications and respiratory failure responsible for more than 85% of deaths. Albeit the inevitability of respiratory failure and short-term death, standardized intervention protocols have been shown to significantly delay the need for invasive ventilatory support, thus prolonging survival and enhancing quality of life. The authors present an intervention protocol based on clinical progression and respiratory parameters. Decisions regarding initiation of non-invasive positive pressure ventilation (NIPPV) and mechanically assisted coughing, depend on development of symptoms of hypoventilation and on objective deterioration of respiratory parameters especially in what concerns bulbar muscle function. These include maximum inspiratory capacity (MIC), difference between MIC and vital capacity (MIC-VC), and assisted peak cough flow (PCF). These standardized protocols along with patient and caregivers education, allow for improved quality of life, prolonged survival and delay or eventually prevent the need for tracheotomy and invasive ventilatory support. Supplemental oxygen should be avoided in these patients, since it precludes use of oxymetry as feedback for titrating NIPPV and MAC, and is associated with decreased ventilatory drive and aggravated hypercapnia.

Mechanical ventilation for severe asthma.

Abstract: Acute exacerbations of asthma can lead to respiratory failure requiring ventilatory assistance. Noninvasive ventilation may prevent the need for endotracheal intubation in selected patients. For patients who are intubated and undergo mechanical ventilation, a strategy that prioritizes avoidance of ventilator-related complications over correction of hypercapnia was first proposed 30 years ago and has become the preferred approach. Excessive pulmonary hyperinflation is a major cause of hypotension and barotrauma. An appreciation of the key determinants of hyperinflation is essential to rational ventilator management. Standard therapy for patients with asthma undergoing mechanical ventilation consists of inhaled bronchodilators, corticosteroids, and drugs used to facilitate controlled hypoventilation. Nonconventional interventions such as heliox, general anesthesia, bronchoscopy, and extracorporeal life support have also been advocated for patients with fulminant asthma but are rarely necessary. Immediate mortality for patients who are mechanically ventilated for acute severe asthma is very low and is often associated with out-of-hospital cardiorespiratory arrest before intubation. However, patients who have been intubated for severe asthma are at increased risk for death from subsequent exacerbations and must be managed accordingly in the outpatient setting.

Ventilatory support and pharmacological treatment of patients with central apnoea or hypoventilation during sleep

Abstract: The concept of central sleep apnoea or hypoventilation encompasses hypercapnic central hypoventilation, such as obesity hypoventilation syndrome and eucapnic or hypocapnic central sleep apnoea. Among subjects with eucapnic or hypocapnic central sleep apnoea, several therapeutic options are available for those with Cheyne-Stokes respiration (CSR). CSR is frequent in patients with New York Heart Association stage III and IV chronic heart failure, and in various neurological disorders. In these patients, treatment modalities include optimising cardiac condition and drugs, such as theophylline, acetazolamide and/or oxygen. Ventilatory support, such as nasal continuous positive airway pressure (CPAP), bi-level pressure support, or adaptive servo- ventilation (ASV), has been shown to improve CSR in patients with cardiac failure; however, convincing evidence that nasal CPAP improves life expectancy in these patients is lacking. Nevertheless, the treatment of associated obstructive sleep-disordered breathing is indicated per se, as it may improve cardiac function. There is currently no proof that bi-level ventilation is superior to nasal CPAP. The few available studies that have focused on ASV have shown satisfactory control of CSR in cardiac failure patients. While ASV is not a first-line treatment choice, it appears to be superior to oxygen, CPAP and bi-level pressure ventilation in controlling the apnoea/hypopnea index and probably sleep fragmentation. As yet there are no data on mortality and, as such, firm conclusions cannot be drawn as to the role of ASV in the management of cardiac failure patients suffering from CSR. Obesity-related hypoventilation has increased dramatically over recent decades due to the epidemic increase in obesity in the developed countries. Obesity hypoventilation syndrome predisposes to the development of pulmonary hypertension and cor pulmonale. Noninvasive home ventilation is increasingly applied in obese patients with hypercapnic respiratory failure, however, initial mechanical ventilatory support can be reduced to nasal continuous positive airway pressure in only a subset of these individuals.

Mechanical ventilation in chronic ventilatory insufficiency

Abstract: Mechanical ventilation has become an important treatment option in chronic ventilatory failure. There are different diseases which lead to ventilatory failure and to home mechanical ventilation (HMV). A primary loss of in- and expiratory muscle strength is the reason for respiratory deterioration in neuromuscular disease. In most of these diseases ventilatory failure develops because of the progressive character of muscular damage. Initially, ventilatory failure can be found during night-time. In the case of hypercapnia at daytime, life expectancy is strongly reduced, especially in amyotrophic lateral sclerosis and Duchenne muscular dystrophy. HMV leads to a prolongation of life and to an increase in quality of life, if bulbar involvement is not severe. Impressive clinical improvements under HMV have been found in restrictive disorders of the rib cage like kyphoscoliosis or posttuberculosis sequelae, with an increase of quality of life, walking distance and a decrease in pulmonary hypertension. Only few data are published about long-term results of HMV in Obesity Hypoventilation. In terms of retrospective analyses of clinical data HMV seems to improve survival in this population. Some patients only need CPAP treatment, but most patients have to be treated with ventilatory support. The application of HMV in patients with chronic ventilatory failure due to chronic obstructive pulmonary disease (COPD) is growing, but there are controversial results in randomised clinical trials. Analysis of these data suggest better results of HMV in patients with severe hypercapnia, with the application of higher effective ventilatory pressure and a ventilator mode with a significant reduction in the work of breathing. Under such conditions HMV leads to a reduction of hypercapnia, an improvement in sleep quality, walking distance and quality of life, but until now there is no evidence in reduction of mortality in COPD.

A Full Term Infant with Cyanotic Episodes

Abstract: Asleep evaluation was requested for a full-term female infant 4 weeks of age Although there were no perinatal problems, the infant remained in the Neonatal Intensive Care Unit (NICU) because of episodes of cyanosis occurring only during sleep, in which the arterial oxygen saturation by pulse oximetry dropped as low as 60% During these episodes the infant was noted to breathe shallowly without an increase in respiratory rate until aroused by the caregiver These episodes were noted daily during the entire stay in the NICU During wakefulness the infant had a normal oxygen saturation of 96%–98% No abnormalities in gastrointestinal function or muscle tone were noted by the caregivers Physical examination: Normal for age while awake Laboratory evaluation: a chest radiograph, electrocardiogram, echocardiogram, and fluoroscopy of the diaphragm were all normal A MRI showed no evidence of brainstem abnormalities Screening studies for inborn errors of metabolism were negative Sleep study: Tracings from polysomnography during wakefulness and NREM sleep are shown in Figure 1 while the patient breathed room air Figure 1 Thirty-second tracings during wakefulness (left) and NREM sleep (right) are shown Right central and occipital electroencephalographic and right and left oculographic derivations as well as a chin electromyographic tracing are shown The PETCO2 is a tracing What is going on with this infant during sleep? Answer: Congenital Central Hypoventilation Syndrome Congenital Central Hypoventilation Syndrome (CCHS) is a rare disorder affecting approximately 1 per 200,000 live births1 CCHS is usually present from birth and is characterized by alveolar hypoventilation without evidence of lung, neuromuscular, or structural brainstem abnormalities During wakefulness, many patients have normal ventilation although ventilatory responses to hypercapnia or hypoxemia by the rebreathing method are absent or blunted and a perception of dyspnea is absent The most severely affected CCHS patients also have hypoventilation during wakefulness Those patients with normal awake ventilation have peripheral chemoreceptor responses to hypoxemia or hypercapnia2 It has been hypothesized that the abnormality in CCHS patients is in the central integration of chemoreceptor information rather than defective chemoreceptors During sleep, all CCHS patients have worsening of ventilation with profound hypoventilation, exhibited by normal respiratory rates and diminished tidal volumes associated with severe falls in arterial oxygen saturation The abnormalities are often worse during NREM than REM sleep, as the control of breathing is entirely metabolic during NREM sleep Of note, one study found that CCHS patients did have intact arousal responses to hypercapnia3 However, the arousal response to hypercapnic hypoxemia is impaired as protracted periods of severe arterial oxygen desaturation can occur before arousal from sleep Other forms of autonomic dysregulation may be seen in these patients These abnormalities can include the following: Hirschprung disease (20% of cases), esophageal dysmotility, tumors of neural crest origin (6% of cases), decreased heart rate variability, decreased heart rate response to exercise, decreased pupillary light response, intermittent profuse sweating, and dysregulation of body temperature with decreased baseline body temperature1,4,5 Hirschprung disease is often suspected when an infant fails to pass meconium by 24–48 hours after birth Esophageal dysmotility can cause difficulty with feeding The neural crest tumors include neuroblastoma and ganglioneuroma The diagnosis of CCHS should be considered in infants with apneic or cyanotic spells especially during sleep The infants with the most severe cases do not breathe after birth and require immediate ventilatory support In others, the abnormalities are noted when the infants sleep Milder cases may present later with signs of cor pulmonale or hypoxic damage to central nervous system structures The diagnosis of CCHS depends on exclusion of other causes of hypoventilation, such as brainstem malformation, inborn errors of metabolism, myopathy, diaphragmatic paralysis, and lung or respiratory pump abnormalities A suspected diagnosis is confirmed by genetic testing for mutations in the PHOX2b gene Most persons with CCHS are heterozygous for polyalanine repeat expansion mutations in exon 3 of PHOX2b The expansion results in lengthening the normal 20-repeat polyalanine tract to 25–33 repeats Longer expansions are associated with more severe phenotypes Most mutations occur de novo, but in families with CCHS it is inherited as an autosomal dominant trait5 Treatment includes life-long ventilatory support for all patients during sleep Some patients will require ventilatory support awake as well Ventilatory support is usually provided by a volume cycled ventilator via a tracheosotomy In older and milder patients noninvasive mask ventilation may suffice Diaphragmatic pacing has also been used Infants with CCHS must be closely monitored as they are at risk for hypoventilation or apnea at sleep onset Children with CCHS are also at increased risk during chest infections due to their abnormal temperature control, lack of perception of dyspnea, and lack of appearance of respiratory distress1 In the present case, the awake tracing (Figure 1) shows a normal SpO2 and end-tidal PCO2 During NREM sleep, a pattern of reduced tidal volume without an increase in respiratory rate is seen even though the end-tidal PCO2 is very increased and the arterial oxygen saturation is severely decreased A blood sample was sent to a referral laboratory at Rush University Genetic analysis demonstrated a mutation in the PHOX2b gene consistent with a diagnosis of CCHS The patient underwent tracheostomy and was started on nocturnal volume cycled ventilation

A case of Kartagener's syndrome with acute respiratory failure successfully treated by NPPV

Abstract: Kartagener’s syndrome is characterized by the triad of situs inversus, bronchiectasis, and chronic pansinusitis. Pulmonary infections, such as pneumonia or other conditions, aggravate the pulmonary symptoms of Kartagener’s syndrome and lead to the exacerbation of hypoventilation and hypercapnia. These conditions may induce acute respiratory failure (ARF). Noninvasive positive pressure ventilation (NPPV) is known to be safe and well tolerated in patients with Kartagener’s syndrome, and it has assumed a prominent role in the management of ARF. The attractiveness of NPPV is primarily related to its advantages over invasive mechanical ventilation. It is used in patients with ARF due to the exacerbation of chronic obstructive pulmonary disease or cardiogenic pulmonary edema. We report a case of ARF in a patient with Kartagener's syndrome that was successfully treated with NPPV.(Korean J Med 73:S976-S980, 2007)

Treatment of chronic respiratory diseases in obese people

Abstract: Magali Poulain and colleagues do not appear to have considered the action of the renin–angiotensin system in their review of the effect of obesity on chronic respiratory diseases.[1][1] A recent study showed increased activation of the renin–angiotensin system in obese people.[2][2] However, a

[Valvular bronchoblocation in the treatment of patients with disseminated drug-resistant pulmonary tuberculosis].

Abstract: The results of treatment are analyzed in 89 patients with disseminated drug-resistant pulmonary tuberculosis. The application of an endobronchial back valve to patients with drug-resistant pulmonary tuberculosis gives rise to hypoventilation and atelectasis in the affected areas of the lung, promotes the stabilization and regression of a tuberculous process, and it is not attended by the development of endobronchial complications.