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We studied 33 patients affected by juvenile and adult myotonic dystrophy type 1 (DM1). The aim of the study was to assess clinical and laboratory parameters that could predict the requirement of noninvasive ventilation (NIV) in DM1. Secondary outcome was to assess the interplay between genetic profile, muscle impairment severity and presence of cardiac comorbidities.Patients with genetic diagnosis of DM1 were recruited. An abnormal trinucleotide repeat (CTG) expansion of dystrophy protein kinase gene (DMPK) on chromosome 19q13.3 was the prerequisite for inclusion. The number of triplet repeats was measured in genomic DNA to classify subjects. A multidisciplinary team evaluated the patients every 6-8 months up to 18 years with serial cardiological and respiratory function assessments. Neurological progression was monitored using a validated DM1-specific rating scale (MIRS). Independent variables considered for the study outcomes were gender, genetic status, age of presentation, MIRS scores, and results of pulmonary function tests (PFTs).Patients were 17 males (51.5%) and 16 females (48.5%). 16 cases were younger than mean age of 31.4 years, the remaining 17 were up to 65. 12 subjects (36.4%) underwent NIV during follow up. Cardiac comorbidities were detected in 63.6% of cases and in 91% of patients in NIV. Among PFTs, forced vital capacity (FVC) was a reliable indicator of respiratory decline. FVC values were significantly associated with clinical muscle severity assessed by MIRS.Severity of muscular impairment, CTG expansion size, age and presence of cardiac comorbidities predict respiratory impairment in DM1.

Respiratory depression, presenting as desaturation and bradypnea, is common during the early postoperative period. However, it has not been evaluated by appropriate monitoring. The purpose of the present study was to identify the incidence and predictors of desaturation and bradypnea following general anesthesia, using a continuous and centralized monitoring system, in non-ICU patients who did not have serious complications and did not undergo major surgery. Patients were connected to a continuous and centralized monitoring system via a pulse oximeter and respiratory rate sensor for at least 8 h after extubation. We assessed the incidence and risk factors for desaturation (SpO2 < 90% for > 10 s) and bradypnea (respiratory rate < 8 breaths/min for > 2 min) events. We retrospectively collected the clinical data of 1064 adult patients in the study. The incidences of desaturation and bradypnea were 12.1% and 5.1%, respectively. Most desaturation events occurred after the termination of oxygen administration. The greatest incidence of bradypnea was within the first hour after surgery, reducing over time. Analysis revealed that age (odds ratio [OR] 1.04, 95% confidence interval [CI] 1.03-1.06; p < 0.001), BMI (OR 1.12, 95% CI 1.06-1.18; p < 0.001) and current smoking (OR 1.91, 95% CI 1.12-3.42; p = 0.023) were significant risk factors for desaturation. Sleep apnea syndrome (OR 4.23, 95% CI 1.09-13.5; p = 0.021) and postoperative opioid administration (OR 2.76, 95% CI 1.44-5.20; p = 0.002) were significantly associated with bradypnea. Age (OR 1.04, 95% CI 1.01-1.07; p = 0.010) and postoperative opioid administration (OR 3.16, 95% CI 1.22-7.87; p = 0.019) showed a significant association with the occurrence of both desaturation and bradypnea. This study demonstrated the incidence and predictors of postoperative desaturation and bradypnea, and suggests the need for monitoring oxygen saturation and respiratory rate for at least 8 h after surgery in non-ICU patients. Use of monitoring systems might provide a safety net for postoperative patients.

The Stewart-Figge acid-base model has been criticized for being mathematically complex. We aimed to develop simpler formalisms, which can be used at the bedside. The following simplifications were used: (1) [Ca 2+ ] and [Mg 2+ ] are replaced by their mid-reference concentrations (2) pH is set to 7.4. In the new model [SIDa] is replaced by its adjusted form, [SIDa, adj] = [Na + ] + [K + ] - [Cl - ] + 6.5 and [SIG] is replaced by "bicarbonate gap", [BICgap] = [SIDa, adj] - (0.28⋅[Albumin]) - (1.82⋅[Phosphate i ])- [HCO 3 ̄]. The diagnostic performance of the model was tested in 210 patients with acute respiratory diseases and 17 healthy volunteers. [BICgap] was also compared to albumin-corrected anion gap ([AGc]). The concordant correlation coefficient between [SIDa, adj] and [SIDa] and between [BICgap] and [SIG] was 0.98 in both comparisons. The mean bias (limits of agreement) of [SIDa, adj] - [SIDa] and of [BICgap] - [SIG] were 0.53 meq/l (- 0.46 to 1.53) and 0.50 meq/l (- 0.70 to 1.70), respectively. A [SIDa, adj] < 50.4 meq/l had an accuracy of 0.995 (p < 0.001) for the diagnosis of strong ion (SI) acidosis, while a [SIDa, adj] > 52.5 meq/l had an accuracy of 0.997 (p < 0.001) for the diagnosis of SI alkalosis. A [BICgap] > 11.6 meq/l predicted unmeasured ion (UI) acidosis with an accuracy of 0.997 (p < 0.001), while an [AGc] > 19.88 meq/l predicted UI acidosis with an accuracy of 0.994 (p < 0.001). The "[BICgap] model" is a reliable tool for the assessment of acid-base disorders in patients with acute respiratory diseases. [BICgap] is not inferior to [AGc] in the diagnosis of UI acidosis.

Background: The impact of cardiorespiratory comorbidity on operative outcomes after esophagectomy remains controversial. This study investigated the effect of cardiorespiratory comorbidity on postoperative complications for patients treated for esophageal or gastroesophageal junction cancer., Patients and Methods: A European multicenter cohort study from five high-volume esophageal cancer centers including patients treated between 2010 and 2017 was conducted. The effect of cardiorespiratory comorbidity and respiratory function upon postoperative outcomes was assessed., Results: In total 1590 patients from five centers were included; 274 (17.2%) had respiratory comorbidity, and 468 (29.4%) had cardiac comorbidity. Respiratory comorbidity was associated with increased risk of overall postoperative complications, anastomotic leak, pulmonary complications, pneumonia, increased Clavien-Dindo score, and critical care and hospital length of stay. After neoadjuvant chemoradiotherapy, respiratory comorbidity was associated with increased risk of anastomotic leak [odds ratio (OR) 1.83, 95% confidence interval (CI) 1.11-3.04], pneumonia (OR 1.65, 95% CI 1.10-2.47), and any pulmonary complication (OR 1.52, 95% CI 1.04-2.22), an effect which was not observed following neoadjuvant chemotherapy or surgery alone. Cardiac comorbidity was associated with increased risk of cardiovascular and pulmonary complications, respiratory failure, and Clavien-Dindo score ≥ IIIa. Among all patients, forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC) ratio > 70% was associated with reduced risk of overall postoperative complications, cardiovascular complications, atrial fibrillation, pulmonary complications, and pneumonia., Conclusions: The results of this study suggest that cardiorespiratory comorbidity and impaired pulmonary function are associated with increased risk of postoperative complications after esophagectomy performed in high-volume European centers. Given the observed interaction with neoadjuvant approach, these data indicate a potentially modifiable index of perioperative risk.

Introduction: There are limited data on modern intermittent hemodialysis (IHD) efficacy on salicylate elimination from topical poisoning., Case Report: A 54-year-old male sought treatment for dyspnea but was then diagnosed with salicylate toxicity from topical application of Dencorub Extra Strength Heat Gel® for 1 week. Each tube contained 100 g with 26 % methylsalicylate (26 g). Laboratory workup was remarkable for an elevated anion gap of 30 and salicylate concentration of 78.7 mg/dL [5.7 mmol/L (N < 0.4 mmol/L)]. Treatment with urinary alkalinization was followed by hemodialysis for 5 h. Extraction ratios were 0.44 with clearance rates of 78.5 mL/min. Salicylate concentrations fell rapidly following initiation of hemodialysis with no rebound observed., Discussion: Modern high flux IHD is an effective method of removing salicylates in the treatment of chronic topical poisoning.

Dysfunctional breathing is characterised by an abnormal breathing pattern leading to respiratory symptoms. The 25-item Self Evaluation of Breathing Questionnaire (SEBQ) has been developed to measure breathing-related symptoms and their severity but lacks thorough evaluation. To determine reproducibility, internal consistency and predictors of SEBQ score, 180 participants completed an online SEBQ with additional demographic and lifestyle questions. Two weeks later, 155 of those repeated SEBQ. Test-retest correlation of the SEBQ was high [intraclass correlation coefficient (3, 1) = 0.89; 95 % CI 0.85-0.92]. There was no difference in SEBQ score between test and retest (15.1 (11.6) [mean (SD)] versus 14.7 (12.4); P = 0.4) and the score showed a typical error (standard error of measurement) of 4.0. Internal consistency was high (Cronbach's α = 0.93), and a single factor structure for items was shown. Smoking status, reported respiratory disease, recent respiratory illness and female gender were positively-associated predictors of SEBQ score, and together explained 25.6 % of score variance (P ≤ 0.001). The SEBQ has high test-retest reproducibility and its score may be predicted by current smoking, chronic respiratory disease, recent respiratory illness and female gender, thus may be a useful clinical screening tool for dysfunctional breathing.

This paper reviews 17 papers or commentaries published in Journal of Clinical Monitoring and Computing in 2015, within the field of respiration. Papers were published covering monitoring and training of breathing, monitoring of gas exchange, hypoxemia and acid-base, and CO2 monitoring.

Objective: The main aim of the study was to investigate the effects of age in pulmonary consultation and to evaluate differences between geriatric and adult patients., Material and Methods: Consultation referrals to the clinic of chest diseases for patients from emergency, inpatient and outpatient clinics of a tertiary care general hospital between December 2010 and April 2011 were examined retrospectively. The patients were divided into two groups, namely those aged 65 years and over (geriatric patients) and those under the age of 65 years (adults)., Results: Out of 272 patients 135 (49.6%) were geriatric patients and 137 (50.4%) were adult patients. The mean age of the geriatric patients and adults was 75.6±7.1 years and 49.7±11.8 years, respectively. While the geriatric patients showed a significantly higher presence of pathologies in chest radiography, respiratory complaints and concomitant diseases compared to the adult group, the respiratory function test results were lower. The complaint of shortness of breath was higher in the geriatric group compared to the adult group (71% in geriatric patients and 59.1% in adults). The most common diagnosis was chronic obstructive pulmonary disease (COPD, 37%) in the geriatric patients and asthma (27.7%) in the adult patients. As to the results of consultations, treatment-oriented recommendations were given for 63 (46.6%) geriatric patients and approval for operation was given for 75 (54.7%) adult patients., Conclusion: Consultants should take the age of patients into consideration during evaluation in order to achieve the most appropriate treatment plan for these patients and reduce the potential postoperative complications to a minimum.

Capnography is a standard monitoring tool during general anaesthesia. Diaphragmatic movement with the weaning of muscle relaxant effect produces the characteristic "curare cleft" on capnography. Various artefacts can mimick this trace intraoperatively. Cautious interpretation and identification of these is essential to avoid any undue overdosing of the patients with muscle relaxants. We report "curare cleft" like artefact during ventilation with a single lumen tube in a patient with unilateral capnothorax undergoing minimally invasive esophagectomy.

Mechanical ventilation is a current support therapy for newborns affected by respiratory diseases. However, several side effects have been observed after treatment, making it mandatory for physicians to determine more suitable approaches. High fidelity simulation is an efficient educational technique that recreates clinical experience. The aim of the present study is the design of an innovative and versatile neonatal respiratory simulator which could be useful in training courses for physicians and nurses as for mechanical ventilation. A single chamber prototype, reproducing a pulmonary lobe both in size and function, was designed and assembled. Volume and pressure within the chamber can be tuned by the operator through the device control system, in order to simulate both spontaneous and assisted breathing. An innovative software-based simulator for training neonatologists and nurses within the continuing medical education program on respiratory disease management was validated. Following the clinical needs, three friendly graphic user interfaces were implemented for simulating three different clinical scenarios (spontaneous breathing, controlled breathing and triggered/assisted ventilation modalities) thus providing physicians with an active experience. The proposed pulmonary simulator has the potential to be included in the range of computer-driven technologies used in medical training, adding novel functions and improving simulation results.

Objective: Biologics are highly effective in ankylosing spondylitis (AS). In this self-controlled study, we assessed the additive value of complex physiotherapy in decreasing chest pain and tenderness and improving respiratory function in AS patients treated with tumor necrosis factor α (TNF-α) inhibitors., Patients and Methods: The trial consisted of 2 parts. In study I, clinical data of AS patients with (n=55) or without biological therapy (n=20) were retrospectively analyzed and compared. Anthropometrical data, duration since diagnosis and patient assessment of disease activity, pain intensity, tender points, sacroiliac joint involvement determined by X-ray, functional condition, and physical activity level were recorded. Subjective, functional, and physical tests were performed. In study II, 10 voluntary patients (6 men and 4 women, age 52.4 ± 13.6 years) with definite AS and receiving anti-TNF therapy were recruited. It was a prospective, non-randomized physiotherapeutic trial. BASFI (Bath Ankylosing Spondylitis Functional Index), BASDAI (Bath Ankylosing Spondylitis Disease Activity Index), modified Schober Index, occiput-to-wall distance, and fingertip-to-floor distance were evaluated. Forced vital capacity, forced 1-s expiratory volume, peak expiratory flow, and maximum voluntary ventilation were recorded. Furthermore, typical tender points were recorded. A targeted physiotherapy program was conducted twice a week for 12 weeks and all above parameters were recorded at baseline and after 12 weeks., Results: Differences in patient assessment of disease activity (p=0.019) and pain intensity (p=0.017) were found in study I. Pain and tenderness of the thoracic spine were observed in both groups. Back pain without biologic therapy was slightly higher than other group. In study II, we found that patient assessment of disease activity and pain intensity significantly improved after the physical therapy program (p=0.002 and p<0.001). BASFI and BASDAI increased after treatment (p=0.004 and p<0.001). The finger-to-floor distance, chest expansion, and modified Schober index increased (p=0.008, p<0.001, and p=0.031, respectively). The respiratory functional parameters showed a tendency towards improvement., Conclusion: AS patients already receiving biological therapy may benefit from additional targeted physiotherapy. Physical therapy may be of important additive value in AS patients being treated with biological. The exercise program presented here showed an improvement in functional parameters as well as spine and chest mobility, thereby enhancing the favorable effects of biological therapy.

Background: Laparoscopic gastrectomy (LAG) is recognized as a less invasive surgery, but no advantage in terms of respiratory function recovery has been demonstrated. We investigated respiratory function recovery in the early period after LAG compared with open gastrectomy (OG) for measuring the recovery of oxygen saturation level (SaO(2))., Methods: The study population comprised 454 patients who underwent distal gastrectomy or total gastrectomy for preoperatively diagnosed T1N0 gastric cancer: 192 underwent laparoscopy-assisted distal gastrectomy (LADG), 190 underwent open distal gastrectomy (ODG), 42 underwent laparoscopy-assisted total gastrectomy (LATG), and 30 underwent open total gastrectomy (OTG)., Results: The number of days until SaO(2) reached 95% or higher in room air was significantly smaller in the LADG group (1.54 days) than in the ODG group (1.81 days; p = 0.010) and also significantly smaller in the LATG group (1.48 days) than in the OTG group (2.03 days; p = 0.043)., Conclusions: LAG patients recovered their oxygenation earlier than OG patients. The laparoscopic procedure might confer a respiratory benefit for gastrectomy patients.

Background: Patients with reactive airways are at risk for adenosine-induced bronchoconstriction, mediated via A(2B) and/or A(3) adenosine receptors., Methods and Results: In this randomized, double-blind, placebo-controlled crossover trial, we examined the safety of regadenoson, a selective adenosine A(2A) receptor agonist, in patients with moderate chronic obstructive pulmonary disease (COPD) (n = 38) and patients with severe COPD (n = 11) with a baseline mean forced expiratory volume in 1 second (FEV(1)) of 1.74 +/- 0.50 L and 1.0 +/- 0.35 L, respectively, 37% of whom had dyspnea during activities of daily living. Patients receiving glucocorticoids or oxygen and those with pretreatment wheezing were included. Short-acting bronchodilators were withheld for at least 8 hours before treatment. No differences emerged between regadenoson and placebo on multiple lung function parameters, including repeated FEV(1) and forced vital capacity, respiratory rate, pulmonary examinations, and oxygen saturation. The mean maximum decline in FEV(1) was 0.11 +/- 0.02 L and 0.12 +/- 0.02 L (P = .55) in patients after regadenoson and placebo, respectively, and new-onset wheezing was observed in 6% and 12%, respectively (P = .33). No patient required acute treatment with bronchodilators or oxygen., Conclusions: This pilot study showed the overall safety of regadenoson in 49 compromised outpatients with clinically stable moderate and severe chronic obstructive pulmonary disease.

Background: Patients with reactive airways are at risk for adenosine-induced bronchoconstriction, mediated via A(2B) and/or A(3) adenosine receptors., Methods and Results: To examine the effects of regadenoson, a selective adenosine A(2A) receptor agonist, on airway resistance, we conducted a randomized, double-blind, placebo-controlled crossover trial in asthmatic patients with a positive adenosine monophosphate challenge test. The mean ratio of the forced expiratory volume in 1 second (FEV(1)) at each tested time point relative to the baseline FEV(1) was significantly higher after treatment with regadenoson compared with placebo from 10 to 60 minutes after treatment. One patient had a substantial but asymptomatic FEV(1) reduction (-36.2%) after regadenoson that reversed spontaneously. The most common adverse events with regadenoson were tachycardia (66%), dizziness (53%), headache (45%), and dyspnea (34%). The mean heart rate significantly increased with regadenoson (maximum of +10.4 beats/min) versus placebo., Conclusions: In this pilot safety study of 48 patients with mild or moderate asthma who had bronchial reactivity to adenosine monophosphate, regadenoson was safe and well tolerated.

Purpose: Virtual endoscopy (VE) is a new diagnostic tool that generates 3-dimensional (3D) views of a lumen by exploiting cross-sectional images. The purpose of this study was to evaluate the usefulness of VE as a diagnostic tool in the diseases of the larynx and pharynx., Materials and Methods: Twenty-two patients with a mean age of 57 years were included in the study. The patients underwent larynx examination, optical endoscopy (OE), and computed tomography (CT) of the larynx. Later, VE was produced from the CT images., Results: Eight patients had larynx carcinoma, a 5-year-old patient had a laryngeal web, a 43-year-old man had fish bone stuck in his submucosal layer, 10 patients were normal, and the remaining two patients were under follow-up for treated nasopharynx carcinoma and had no evidence for recurrence. VE showed the laryngeal tumor in seven patients and the laryngeal web in one patient, but failed to show a plaquelike tumor and the fishbone within the submucosa., Conclusions: Our findings suggest that VE is a useful and complimentary method of 3D imaging in the diseases compromising the laryngeal lumen. Furthermore, it may be superior to OE in severe stenosis or obstructions where the endoscope cannot be passed through.

Background: Patients with congestive heart failure (CHF) have increased ventilatory equivalent for carbon dioxide (Ve/VCO(2)), which may contribute to the symptom of exercise-induced hyperpnea. We have developed a technique in which simultaneous blood volume single photon emission computed tomography imaging and transmission tomography are used to measure extravascular lung density (ELD). We investigated the correlation between Ve/VCO(2) and ELD in patients with CHF., Methods and Results: Thirteen patients with stable CHF and eleven control subjects were studied. Attenuation-corrected blood volume emission tomography was acquired with simultaneous transmission tomography to measure pulmonary blood volume and total lung density, respectively. Seven CHF patients underwent maximal exercise treadmill testing with online respiratory gas analysis. ELD was calculated as total lung density minus pulmonary blood volume. SPECT and transmission tomography were repeated immediately after exercise. CHF patients had significantly higher total lung density and ELD compared with normal subjects. No differences in pulmonary blood volume were observed. There was a significant inverse correlation between ELD and left ventricular ejection fraction at rest in CHF patients (r = -0.77, P <.001). A strong correlation was also found between post-exercise ELD and Ve/VCO(2) at peak exercise (r = 0.74, P =.008) and at anaerobic threshold (r = 0.67, P =.024)., Conclusion: Patients with chronic CHF have increased ELD. The correlation between ELD and Ve/VCO(2) suggests that increased lung water may contribute to the ventilatory abnormalities seen in patients with CHF.

Although pulmonary function tests have been used to evaluate horses with clinically normal lungs and those with recurrent airway obstruction (RAO), comprehensive studies of equine respiratory function, considering factors such as sex, age and usage are not available. Studies on the influence of these factors on pulmonary function in healthy horses are required for interpretation of measurements made in those with respiratory disease. The study was performed with 63 warmblood horses and the status of their pulmonary health was evaluated by clinical examination. The functional aspect of pulmonary health was assessed by indices derived from the single-breath diagram for CO2 (SBD-CO2). Differences in pulmonary functional indices between the sexes, age groups and usage groups adjusted for pulmonary health were analysed with hierarchical linear models. It was shown that the effects on pulmonary function of age, sex and usage of a horse were significant and have to be considered when establishing the true pulmonary functional status of a horse suffering from RAO. The multivariate statistical model used included the covariates age, sex and sporting discipline of the horses and accounted for the complex, non-linear interactions between the different covariates.

The paper reviews how forced oscillation techniques (FOT) for measuring respiratory input impedance Zrs,in have recently been used in clinical applications. Zrs,in is clinically relevant, as it provides data on both the resistive, Rrs, and nonresistive, Xrs, components of the respiratory system. Additionally, when excitatory test signals extending into low- (<4 Hz) or high-frequency (>100 Hz) ranges are used, reliable partitioning of lung tissue from airway components is feasible. Adult and paediatric studies examining the use of Zrs,in for routine lung-function assessment, sleep and mechanical ventilation are reviewed. For clinicians, Zrs,in repeatable and sensitive to airway resistance. It is helpful for assessing unco-operative and severely obstructed patients, for monitoring mechanics during artificial ventilation and for tracking airway closure during sleep studies. For paediatricians, longitudinal studies of the growth and development of the respiratory system can also be made using Zrs,in. Forced oscillation techniques, however, require further standardisation, and Zrs,in is limited by upper-airway shunt artifacts. In conclusion, measurement of Zrs,in using FOT is an important and sophisticated non-invasive lung-function test, showing good potential for future clinical applications.

Examination of the lung function in school children is complicated and necessitates a considerable amount of technical equipment. The general practitioner carrying out the examination should not forget that a detailed clinical examination and a good stethoscope can provide important information for the diagnosis, differential diagnosis and further therapy of the child. The range of so-called simple electronic spirometers with a print-out of data tempts practitioner to use them in the diagnostics of the functioning of the lung. There is, however, considerable danger that these instruments lead more often in wrong results. A simple bell spirograph with a recording unit together with a helium analysis instrument allows the doctor to make a complete spirogram which gives exact information. In addition, the doctor requires a special lung laboratory to which to transfer children for a period of supervision and larger. The doctor should be aware that an absence of clinical symptoms, proved in practice, does not necessarily mean that the lung is working normally. Indication of an extended examination of the lungs can be deduced from a blood gas analysis, which can, with some practice be determined in the office. A reduced pO2 or an increased pCO2 can indeed indicate a disorder even when the child appears on examination to be clinically healthy. Instruments new commonly in use for analyzing the blood gas with capillary blood can also be introduced into the office.

The review of the literature on Cheyne-Stokes respiratory periods reveals enormous variation of the opinions expressed. The original description concerned periodicity characterized by rhythmic changes of respiratory phases and respiratory pauses in a relation of 60 : 15 seconds. In the respiratory phase there were 30 respirations of increasing depths and frequency at the beginning of the phase and decreasing depths and frequency at the end of the phase. Literature data about Cheyne-Stokes respiration comprise a multiplicity of all rhythymic forms. The duration of respiratory cycles varies between 12 and 130 seconds. The relation of the respiratory phase and respiratory pause between 6 : 4 or 75 : 70 seconds, and the number of breaths between 3 and 30 during one respiratory phase. Cheyne-Stokes periods were observed in health subjects as well as in patients with neurological, neurosurgical, cardiac, pulmonary and paediatric diseases. Cheyne-Stokes periods were explained as sequel of prolongation of circulation time between pulmonary alveoli and respiratory centre, through increased sensitivity of the respiratory centre to CO2, diminished sensitivity of the respiratory centre to CO2 and O2-deficit, local blood flow disturbances, section of pathways in the brain stem with disinhibition of basic rhythms, brain immaturity, alterations of consciousness, and respiratory obstructions. Rhythmic changes of the heart beat, of excitability of the heart muscle, of blood pressure, of EEG and of neurological and mental signs were observed. In spite of numerous observations detailed analysis of the respiratory cycle was performed in only a few cases. Major studies are lacking.