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PURPOSE: Initially introduced as a safer alternative to smoking, electronic cigarettes (e-cigarettes) and vaping have since been associated with lung injury. Nevertheless, there is limited perioperative data on their potential contribution to the harmful effects of mechanical ventilation on the lungs. We hypothesized that, in adults undergoing noncardiothoracic surgeries, preoperative vaping/e-cigarette use is associated with hypoxemia during the first postoperative hour, and with an increased incidence of intraoperative and postoperative pulmonary complications. METHODS: We conducted a retrospective cohort study in which we included patients reporting as vapers/e-cigarette users within one year before surgery as the exposure group, and nonvapers as the control group. The primary outcome was the time-weighted average (TWA) SpO2/FIO2 ratio in the postanesthesia care unit during the first postoperative hour. The secondary outcome was a composite of intraoperative and postoperative pulmonary complications until discharge. We used entropy balancing to adjust for confounding, and fit weighted linear regression and logistic regression models to estimate treatment effects. RESULTS: A total of 110,940 patients met the inclusion criteria, and 1,941 of these were vapers/e-cigarette users. The average treatment effect on the treated for TWA SpO2/FIO2 ratio (N = 109,217) was estimated to be a mean difference of 4 (95% confidence interval [CI], 1 to 8; P = 0.007). This is equivalent to a 4% change in SpO2 at a 30% FIO2 (or at a fixed FIO2). The difference was statistically significant. The average treatment effect on the treated for experiencing intraoperative and postoperative pulmonary complications (N = 110,940) was an odds ratio of 1.04 (95% CI, 0.71 to 1.54; P = 0.84). CONCLUSION: Vaping/e-cigarette use was neither associated with clinically significant hypoxemia during the first hour in the postanesthesia care unit nor with an increase in pulmonary complications. Nevertheless, our findings cannot definitively exclude the deleterious effects of vaping and e-cigarette use on the lungs, and anesthesiologists should consider potential perioperative complications.
RéSUMé: OBJECTIF: Initialement introduites comme une alternative plus sécuritaire au tabagisme, les cigarettes électroniques et le vapotage ont depuis été associés à des lésions pulmonaires. Néanmoins, il existe peu de données périopératoires concernant leur contribution potentielle aux effets nocifs de la ventilation mécanique sur les poumons. Nous avons émis l'hypothèse que, chez les adultes bénéficiant de chirurgies non cardiothoraciques, l'utilisation préopératoire de vapotage/de cigarette électronique était associée à une hypoxémie au cours de la première heure postopératoire et à une incidence accrue de complications pulmonaires peropératoires et postopératoires. MéTHODE: Nous avons mené une étude de cohorte rétrospective dans laquelle nous avons inclus les patient·es déclarant avoir vapoté ou utilisé des cigarettes électroniques dans l'année précédant la chirurgie comme groupe d'exposition, et les personnes ne vapotant pas comme groupe témoin. Le critère d'évaluation principal était le rapport de SpO2/FIO2 moyen pondéré dans le temps en salle de réveil pendant la première heure postopératoire. Le critère d'évaluation secondaire était un mélange de complications pulmonaires peropératoires et postopératoires jusqu'au congé de l'hôpital. Nous avons utilisé l'équilibrage d'entropie pour ajuster les facteurs de confusion et ajusté les modèles de régression linéaire pondérée et de régression logistique pour estimer les effets du traitement. RéSULTATS: Au total, 110 940 patient·es répondaient aux critères d'inclusion, dont 1941 étaient des vapoteurs ou vapoteuses/utilisaient des cigarettes électroniques. L'effet moyen du traitement sur le rapport de SpO2/FIO2 moyen pondéré dans le temps des personnes traitées (N = 109 217) a été estimé à une différence moyenne de 4 (intervalle de confiance [IC] à 95 %, 1 à 8; P = 0,007). Cela équivaut à une variation de 4 % de la SpO2 à 30% FIO2 (ou à une FIO2 fixe). La différence était statistiquement significative. L'effet moyen du traitement sur les personnes traitées pour des complications pulmonaires peropératoires et postopératoires (N = 110 940) était un rapport de cotes de 1,04 (IC 95 %, 0,71 à 1,54; P = 0,84). CONCLUSION: L'utilisation du vapotage et de la cigarette électronique n'était associée ni à une hypoxémie cliniquement significative au cours de la première heure en salle de réveil, ni à une augmentation des complications pulmonaires. Néanmoins, nos résultats ne peuvent exclure de manière définitive les effets délétères du vapotage et de l'utilisation de la cigarette électronique sur les poumons, et les anesthésiologistes devraient tenir compte des complications périopératoires potentielles.
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How to cite this article: Gangireddy S, Jindal A. Beyond the Nasal Prongs: A Joust of Oxygen Delivery Methods in Post-op Hypoxemia. Indian J Crit Care Med 2024;28(6):625.
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How to cite this article: Mishra S, Kothari N, Sharma A, Goyal S, Rathod DK, Meshram T, et al. Author Response: Beyond the Nasal Prongs: A Joust of Oxygen Delivery Methods in Post-op Hypoxemia. Indian J Crit Care Med 2024;28(6):626-627.
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Down syndrome (DS), the genetic condition caused by trisomy 21 (T21), is characterized by delayed neurodevelopment, accelerated aging, and increased risk of many co-occurring conditions. Hypoxemia and dysregulated hematopoiesis have been documented in DS, but the underlying mechanisms and clinical consequences remain ill defined. We report an integrative multi-omic analysis of â¼400 research participants showing that people with DS display transcriptomic signatures indicative of elevated heme metabolism and increased hypoxic signaling across the lifespan, along with chronic overproduction of erythropoietin, elevated biomarkers of tissue-specific hypoxia, and hallmarks of stress erythropoiesis. Elevated heme metabolism, transcriptional signatures of hypoxia, and stress erythropoiesis are conserved in a mouse model of DS and associated with overexpression of select triplicated genes. These alterations are independent of the hyperactive interferon signaling characteristic of DS. These results reveal lifelong dysregulation of key oxygen-related processes that could contribute to the developmental and clinical hallmarks of DS.
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OBJECTIVE: Obstructive sleep apnea (OSA) has been associated with an elevated cardiovascular risk, increased daytime sleepiness, cognitive decline, and slower electroencephalographic activity (EEG). This study assesses EEG patterns during wakefulness in OSA patients compared to those without sleep-disordered breathing. MATERIALS AND METHODS: This retrospective study analyzed 30 OSA patients with an Apnea/Hypopnea Index (AHI) of 15 or higher, as well as 29 individuals without sleep-disordered breathing (AHI of 5 or lower) who underwent hospital polysomnography and met all inclusion criteria. Sociodemographic and EEG data were obtained from the sleep laboratory database. Blinded EEG analysis was conducted by two observers, assessing activity in the frontal, central, and occipital regions. RESULTS: No significant differences were observed in EEG activity between OSA and non-OSA patients. However, a weak correlation was found between decreased C3 EEG frequency and higher AHI (p = 0.033), as well as increased total sleep time and higher O2 EEG frequency (p = 0.038). Lower amplitudes in C3 (p = 0.043) and O1 (p = 0.031) were correlated with reduced average oxygen saturation. CONCLUSION: Our findings suggest that OSA-related hypoxemia may impact neuronal activity, highlighting the need to address this sleep-disordered breathing in order to potentially prevent the cognitive decline observed in OSA patients.
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INTRODUCTION: Home oxygen therapy is one of the few interventions that can improve survival in patients with chronic obstructive pulmonary disease (COPD) when administered appropriately, although it may cause side effects and be an unnecessary burden for some patients. AREAS COVERED: This narrative review summarizes the current literature on the assessment of hypoxemia, different types of home oxygen therapy, potential beneficial and adverse effects, and emerging research on home oxygen therapy in COPD. A literature search was performed using MEDLINE and EMBASE up to January 2024, with additional articles being identified through clinical guidelines. EXPERT OPINION: Hypoxemia is common in patients with more severe COPD. Long-term oxygen therapy is established to prolong survival in patients with chronic severe resting hypoxemia. Conversely, in the absence of chronic severe resting hypoxemia, home oxygen therapy has an unclear or conflicting evidence base, including for palliation of breathlessness, and is generally not recommended. However, beneficial effects in some patients cannot be precluded. Evidence is emerging on the optimal daily duration of oxygen use, the role of high-flow and auto-titrated oxygen therapy, improved informed decision-making, and telemonitoring. Further research is needed to validate novel oxygen delivery systems and monitoring tools and establish long-term effects of ambulatory oxygen therapy in COPD.
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In patients with pulmonary emphysema and mild to moderate airflow limitation, one does not expect the features marked exertional dyspnea and hypoxemia as well as a profound decrease in diffusing capacity of the lung for carbon monoxide (DLCO). Here we describe this phenotype and its prognosis. From our database, we retrospectively selected cases associating emphysema, exertional breathlessness, O2 requirement at least upon exercise, forced expiratory volume in 1 sec (FEV1) ≥ 50% predicted, and DLCO ≤ 50% predicted, without associated combined pulmonary fibrosis and emphysema, right-to-left shunt, or severe pulmonary hypertension. Over a 12-year period, we identified 16 patients with emphysema and the above presentation. At the initial evaluation, the median age was 62 years (interquartile range 53.8-68.9). The median FEV1 and DLCO% predicted and mean pulmonary artery pressure were 86 (65-95)%, 38 (31-41)%, and 20 (17-25) mm Hg, respectively. On room air, the median arterial partial pressure of oxygen and partial pressure of carbon dioxide in arterial blood were 63.5 (55.8-69) mm Hg and 34.5 (31-36) mm Hg with increased median alveolar-arterial oxygen difference (46 [39-51] mm Hg). After the initial evaluation, the respiratory condition worsened in 13 of 14 (92.8%) patients with one or more re-evaluations (median follow-up 2.6 [0.9-5.8] years). In 12, lung transplantation was considered. Four patients died after 5.8, 5.7, 7.1, and 0.8 years of follow-up, respectively. We describe an underrecognized phenotype of pulmonary emphysema featuring a particular profile characterized by marked exertional dyspnea, impaired pulmonary gas exchange with low DLCO and marked oxygen desaturation at least on exercise but with mild or moderate airway obstruction.
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BACKGROUND: Nasal cannulas and face masks are common oxygenation tools used in conventional oxygen therapy for patients undergoing endoscopic surgery with sedation. However, as a novel supraglottic ventilation technique, the application of supraglottic jet oxygenation and ventilation (SJOV) in endoscopic surgery has not been well established. METHOD: We searched six electronic databases from inception to January 16, 2024, to assess the oxygenation/ventilation efficacy and side effects of the of SJOV in endoscopic surgery. The primary outcome was the incidence of hypoxemia. The secondary outcomes were the incidence of respiratory depression and adverse effects (nasal bleeding, sore throat, and dry mouth). RESULTS: Nine trials involving 2017 patients were included. The results demonstrated that the incidence of hypoxemia was lower in the SJOV group compared with the conventional oxygen therapy (COT) group [9 trails; 2017 patients; risk ratio (RR) = 0.18; 95% confidence interval (CI), (0.11-0.28)]. Subgroup analyses showed that SJOV reduced the incidence of hypoxemia in the high-risk group but had no effect on the low-risk group. The incidence of respiratory depression is lower in SJOV than in COT, but has increased side effects such as dry mouth. There was no statistically significant difference in nose bleeding or sore throat between the two groups. CONCLUSION: Compared with the COT, the SJOV decreased the incidence of hypoxemia in high-risk patients during endoscopic surgery with sedation. There was an increased risk of dry mouth, but not of nose bleeding or sore throat, during endoscopic surgery under sedation.
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Background: The objective of this study was to conduct a systematic review and meta-analysis of the clinical application effects of transnasal high flow nasal cannula compared to other conventional modalities for oxygen therapy devices in patients undergoing bronchoscopy. Methods: A comprehensive literature search was conducted in multiple English databases, including PubMed, Web of Science, and Cochrane Library, to collect relevant studies on the application of high flow nasal cannula in patients undergoing bronchoscopy, and conducted a meta-analysis utilizing RevMan 5.4 software, following the predetermined inclusion and exclusion criteria. Results: A total of 12 studies meeting the inclusion criteria were included, involving 1,631 patients (HFNC group: n = 811, other oxygen therapy group: n = 820). The meta-analysis results demonstrated that HFNC significantly reduced the incidence of hypoxemia and improved the minimum oxygen saturation compared to conventional oxygen therapy (RR = 0.27, 95% CI: 0.18-0.41, p < 0.00001; MD = 6.09, 95% CI: 3.73-8.45, p < 0.00001). Furthermore, HFNC showed statistically significant differences when compared to non-invasive ventilation in terms of hypoxemia incidence (RR = 3.52, 95% CI: 1.13-10.97, p = 0.03) and minimum oxygen saturation (MD = -1.97, 95% CI: -2.97--0.98, p < 0.0001). In addition, HFNC resulted in significantly shorter surgical time and higher PaO2 at the end of the procedure compared to conventional oxygen therapy (MD = 1.53, 95% CI: 0.66-2.40, p = 0.0006; MD = 15.52, 95% CI: 10.12-20.92, p < 0.00001). However, there were no statistically significant differences observed in PaCO2, EtCO2, and MAP at the end of the procedure (MD = 1.23, 95% CI: -0.74-3.20, p = 0.22; MD = -0.35, 95% CI: -3.77-3.06, p = 0.84; MD = -0.54, 95% CI: -2.44-1.36, p = 0.58). Conclusion: When HFNC or NIV is utilized during the examination and treatment of bronchoscopy patients, both oxygenation modalities enhance oxygenation function and reduce the incidence of hypoxemia compared to conventional oxygen therapy. HFNC can be regarded as a viable alternative to NIV for specific high-risk patients undergoing bronchoscopy. It decreases the duration of bronchoscopy and improves the PaO2 levels at the end of the procedure, but does not significantly impact the PaCO2, EtCO2, and mean arterial pressure. Systematic review registration: https://www.crd.york.ac.uk/PROSPERO/, identifier 1414374462@qq.com.
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Background and Aims: Colonoscopy is a critical diagnostic and therapeutic procedure in gastroenterology. However, it carries risks, including hypoxemia, which can impact patient safety. Understanding the factors that contribute to the incidence of severe hypoxemia, specifically the role of procedure duration, is essential for improving patient outcomes. This study aims to elucidate the relationship between the length of colonoscopy procedures and the occurrence of severe hypoxemia. Methods: We conducted a retrospective cohort study at Sheba Medical Center, Israel, including 21,524 adult patients who underwent colonoscopy from January 2020 to January 2024. The study focused on the incidence of severe hypoxemia, defined as a drop in oxygen saturation below 90%. Sedation protocols, involving a combination of Fentanyl, Midazolam, and Propofol were personalized based on the endoscopist's discretion. Data were collected from electronic health records, covering patient demographics, clinical scores, sedation and procedure details, and outcomes. Statistical analyses, including logistic regression, were used to examine the association between procedure duration and hypoxemia, adjusting for various patient and procedural factors. Results: We initially collected records of 26,569 patients who underwent colonoscopy, excluding 5045 due to incomplete data, resulting in a final cohort of 21,524 patients. Procedures under 20 min comprised 48.9% of the total, while those lasting 20-40 min made up 50.7%. Only 8.5% lasted 40-60 min, and 2.9% exceeded 60 min. Longer procedures correlated with higher hypoxemia risk: 17.3% for <20 min, 24.2% for 20-40 min, 32.4% for 40-60 min, and 36.1% for ≥60 min. Patients aged 60-80 and ≥80 had increased hypoxemia odds (aOR 1.1, 95% CI 1.0-1.2 and aOR 1.2, 95% CI 1.0-1.4, respectively). Procedure durations of 20-40 min, 40-60 min, and over 60 min had aORs of 1.5 (95% CI 1.4-1.6), 2.1 (95% CI 1.9-2.4), and 2.4 (95% CI 2.0-3.0), respectively. Conclusions: The duration of colonoscopy procedures significantly impacts the risk of severe hypoxemia, with longer durations associated with higher risks. This study underscores the importance of optimizing procedural efficiency and tailoring sedation protocols to individual patient risk profiles to enhance the safety of colonoscopy. Further research is needed to develop strategies that minimize procedure duration without compromising the quality of care, thereby reducing the risk of hypoxemia and improving patient safety.
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BACKGROUND: There is growing concern that pulse oximeters are routinely less accurate in hospitalized patients with darker skin pigmentation, in turn increasing risk of undetected (occult) hypoxemia and adverse clinical outcomes. The aim of this systematic review and meta-analysis was to synthesize evidence on racial and ethnic disparities in occult hypoxemia prevalence and clinical impacts of undetected hypoxemia. METHODS: Ovid MEDLINE, Embase, and CINAHL databases were searched for relevant articles published through January 2024. Eligible studies must have been conducted among adults in inpatient or outpatient settings and report occult hypoxemia prevalence stratified by patient race or ethnicity, or clinical outcomes stratified by patient race or ethnicity and occult hypoxemia status. Screening for inclusion was conducted independently by two investigators. Data extraction and risk of bias assessment were conducted by one investigator then checked by a second. Outcome data were synthesized using random-effects meta-analyses. RESULTS: Fifteen primary studies met eligibility criteria and reported occult hypoxemia prevalence in 732,505 paired oximetry measurements from 207,464 hospitalized patients. Compared with White patients, occult hypoxemia is likely more common among Black patients (pooled prevalence ratio = 1.67, 95% CI 1.47 to 1.90) and among patients identifying as Asian, Latinx, Indigenous, multiracial, or other race or ethnicity (pooled prevalence ratio = 1.39, 95% CI 1.19 to 1.64). Findings from studies reporting clinical outcomes suggest that Black patients with undetected hypoxemia may experience poorer treatment delivery outcomes than White patients with undetected hypoxemia. No evidence was found from outpatient settings. DISCUSSION: This review and included primary studies rely on self-identified race or ethnicity, which may obscure variability in occult hypoxemia risk. Findings underscore that clinicians should be aware of the risk of occult hypoxemia in hospitalized patients with darker skin pigmentation. Moreover, oximetry data from included studies suggests that the accuracy of pulse oximeters could vary substantially from patient to patient and even within individual patients. TRIAL REGISTRATION: PROSPERO ( CRD42023402152 ).
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BACKGROUND: The dominant feature of COVID-19-associated ARDS is gas exchange impairment. Extravascular lung water index is a surrogate for lung edema and reflects the level of alveolocapillary disruption. The primary aim was the prediction of extravascular lung water index by the alveolar-arterial oxygen difference. The secondary aims were in determining the relationship between the extravascular lung water index and other oxygenation parameters, the FIO2 , end-tidal oxygen concentration, pulmonary oxygen gradient (FIO2 minus end-tidal oxygen concentration), and PaO2 . METHODS: This observational prospective single-center study was performed at the Department of Anaesthesiology and Intensive Care, The University Hospital in Ostrava, The Czech Republic, during the COVID-19 pandemic, from March 20, 2020, until May 24, 2021. RESULTS: The relationship between the extravascular lung water index and alveolar-arterial oxygen difference showed only a mild-to-moderate correlation (r = 0.33, P < .001). Other extravascular lung water index correlations were as follows: FIO2 (r = 0.33, P < .001), end-tidal oxygen concentration (r = 0.26, P = .0032), FIO2 minus end-tidal oxygen concentration (r = 0.15, P = .0624), and PaO2 (r = -0.15, P = .01). CONCLUSIONS: The alveolar-arterial oxygen difference does not reliably correlate with the extravascular lung water index and the degree of lung edema in COVID-19-associated ARDS.
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Purpose: This study aimed to systematically evaluate the clinical effects of using transnasal high-flow nasal cannula (HFNC) and conventional oxygen therapy (COT) in patients undergoing gastrointestinal endoscopy. Methods: A comprehensive literature search was conducted from 2004 to April 2024 to collect relevant studies on the application of HFNC in patients undergoing gastrointestinal endoscopy. Multiple Chinese and English databases, including China National Knowledge Infrastructure (CNKI), Wanfang Data, Web of Science, PubMed, and Cochrane Library, were searched systematically for randomized controlled trials (RCTs). Two researchers independently screened the literature, extracted data, and assessed the risk of bias in the included studies. RevMan 5.4 software was utilized for conducting the network meta-analysis. Results: A total of 12 RCTs involving 3,726 patients were included. Meta-analysis results showed that HFNC reduced the incidence of hypoxemia and improved the minimum oxygen saturation (SpO2) compared with COT [odds ratio (OR) = 0.39, 95% confidence interval (CI): 0.29-0.53], [mean difference (MD) = 4.07, 95% CI: 3.14-5.01], and the difference was statistically significant. However, the baseline SpO2 levels and incidence of hypercapnia were not statistically significantly different between the HFNC and COT groups [MD = -0.21, 95% CI: -0.49-0.07]; [OR = 1.43, 95% CI: 0.95-2.15]. In terms of procedure time, the difference between HFNC and COT was not statistically significant, and subgroup analyses were performed for the different types of studies, with standard deviation in the gastroscopy group (MD = 0.09, 95% CI: -0.07-0.24) and the endoscopic retrograde cholangiopancreatography group (MD = 0.36, 95% CI: -0.50-1.23). The results demonstrated a significant reduction in the adoption of airway interventions in the HFNC group compared to the COT group (OR = 0.16, 95% CI: 0.05-0.53), with a statistically significant difference; this result was consistent with those of the included studies. Conclusion: The application of HFNC improves the incidence of hypoxemia, enhances oxygenation, and reduces airway interventions during gastrointestinal endoscopy. However, HFNC does not significantly affect baseline SpO2, hypercapnia, or procedure time. The limitations of this study must be acknowledged, and further high-quality studies should be conducted to validate these findings.
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BACKGROUND: Beneficial effects of breathing at FIO2 < 0.21 on disease outcomes have been reported in previous preclinical and clinical studies. However, the safety and intra-hospital feasibility of breathing hypoxic gas for 5 d have not been established. In this study, we examined the physiologic effects of breathing a gas mixture with FIO2 as low as 0.11 in 5 healthy volunteers. METHODS: All 5 subjects completed the study, spending 5 consecutive days in a hypoxic tent, where the ambient oxygen level was lowered in a stepwise manner over 5 d, from FIO2 of 0.16 on the first day to FIO2 of 0.11 on the fifth day of the study. All the subjects returned to an environment at room air on the sixth day. The subjects' SpO2 , heart rate, and breathing frequency were continuously recorded, along with daily blood sampling, neurologic evaluations, transthoracic echocardiography, and mental status assessments. RESULTS: Breathing hypoxia concentration dependently caused profound physiologic changes, including decreased SpO2 and increased heart rate. At FIO2 of 0.14, the mean SpO2 was 92%; at FIO2 of 0.13, the mean SpO2 was 93%; at FIO2 of 0.12, the mean SpO2 was 88%; at FIO2 of 0.11, the mean SpO2 was 85%; and, finally, at an FIO2 of 0.21, the mean SpO2 was 98%. These changes were accompanied by increased erythropoietin levels and reticulocyte counts in blood. All 5 subjects concluded the study with no adverse events. No subjects exhibited signs of mental status changes or pulmonary hypertension. CONCLUSIONS: Results of the current physiologic study suggests that, within a hospital setting, delivering FIO2 as low as 0.11 is feasible and safe in healthy subjects, and provides the foundation for future studies in which therapeutic effects of hypoxia breathing are tested.
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Methemoglobinemia is a condition characterized by the presence of abnormal hemoglobin, known as methemoglobin, in the blood, which impairs the ability of red blood cells to carry oxygen effectively. Symptoms include cyanosis, shortness of breath, fatigue, and in severe cases, organ damage or death. We presented a case of a 49-year-old female with multiple myeloma who developed drug-induced methemoglobinemia while on dapsone prophylaxis for Pneumocystis carinii pneumonia (PCP). Despite normal glucose-6-phosphate dehydrogenase (G6PD) levels, the patient exhibited cyanosis and shortness of breath. The case underscores the importance of considering methemoglobinemia in patients with unexplained hypoxemia, especially when associated with medication use. Diagnosis relies on clinical assessment, arterial or venous blood gas analysis with co-oximetry, and a thorough medication history. Methemoglobinemia poses a diagnostic challenge due to its varied presentations and requires a high index of suspicion, particularly in patients with multiple myeloma receiving potentially causative medications such as dapsone. Thorough evaluation, interdisciplinary collaboration, and prompt treatment are essential for favorable outcomes in these complex cases.
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We report a case of a 42-year-old woman diagnosed with pulmonary benign metastasizing leiomyomatosis with a random nodular pattern on image and with a rare clinical condition progressing with respiratory failure and severe hypoxemia. This study is relevant due to the rarity of the tomographic pattern and the patient's clinical presentation. There is no treatment guideline for this comorbidity, which further increases the importance of publishing case reports in the literature.
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Abernethy syndrome is a rare congenital malformation stemming from a portosystemic shunt. Diagnosis proves challenging due to nonspecific clinical symptoms, with presentation varying based on age and disease severity. Consequences include hepatic, cardiovascular, renal, gastrointestinal, and neurological complications, and growth retardation. We report the case of a child presenting with perioral and digital cyanosis, observed in early childhood. Clinical examination revealed low saturation, telangiectasias, digital clubbing, and collateral venous circulation in the thorax. Imaging confirmed the diagnosis of Abernethy syndrome.
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INTRODUCTION: Sleep-disordered breathing (SDB) is a major comorbidity in idiopathic pulmonary fibrosis (IPF) and is associated with a poor outcome. There is a lack of knowledge regarding the impact of SDB treatment on IPF. We assessed at one year: (1) the effect of CPAP and/or nocturnal oxygen therapy on IPF regarding lung function, blood mediators, and quality of life; (2) adherence to SDB treatment and SDB changes. METHODOLOGY: This is a prospective study of consecutive newly diagnosed IPF patients initiating anti-fibrotic treatment. Lung function, polysomnography, blood tests and quality of life questionnaires were performed at inclusion and after one year. Patients were classified as obstructive sleep apnoea (OSA), central sleep apnoea (CSA), and sleep-sustained hypoxemia (SSH). SDB therapy (CPAP and/or nocturnal oxygen therapy) was initiated if needed. RESULTS: Fifty patients were enrolled (36% had OSA, 22% CSA, and 12% SSH). CPAP was started in 54% of patients and nocturnal oxygen therapy in 16%. At one-year, polysomnography found improved parameters, though 17% of patients had to add nocturnal oxygen therapy or CPAP, while 33% presented SDB onset at this second polysomnography. CPAP compliance at one year was 6.74 h/night (SD 0.74). After one year, matrix metalloproteinase-1 decreased in OSA and CSA (p = 0.029; p = 0.027), C-reactive protein in OSA (p = 0.045), and surfactant protein D in CSA group (p = 0.074). There was no significant change in lung function. CONCLUSIONS: Treatment of SBD with CPAP and NOT can be well tolerated with a high compliance. IPF patients may exhibit SDB progression and require periodic re-assessment. Further studies to evaluate the impact of SDB treatment on lung function and serological mediators are needed.