Pulmonary outcomes of congenital diaphragmatic hernia patients based on defect size (CDH Study Group Stage).

Background: Congenital diaphragmatic hernia (CDH) is associated with significant pulmonary morbidity. Previous investigation has shown that postnatal inpatient morbidity is linked to diaphragmatic defect size. The objective of this study was to evaluate long-term pulmonary outcomes by CDH study group defect size. Methods: A retrospective analysis was conducted for CDH patients (n=133) managed in a neonatal intensive care unit (NICU) at a single children's hospital within an adult hospital system and subsequently followed up at a comprehensive multidisciplinary CDH clinic (n=102) from January 2012 to April 2022. CDH patients were stratified according to Congenital Diaphragmatic Hernia Study Group (CDHSG) Stage, and then categorized as low-risk (LR), defect size A and B, or high-risk (HR), defect size C and D. Inpatient data, including the presence of pulmonary hypertension, extracorporeal life support (ECLS) utilization, and mechanical ventilation days, were collected. Post-discharge data including the prevalence of asthma, pulmonary hypertension, emergency department visits, the total number of hospitalizations, and average rehospitalization days were collected. Frequentist analysis was used. Results: The outcomes for 133 NICU patients were analyzed (HR: n=54, LR: n=79). During NICU stay, the prevalence of pulmonary hypertension [HR: 16/54 (30%) vs. LR: 9/79 (12%), P=0.009], ECLS utilization [HR: 19/54 (35%) vs. LR: 4/79 (5%), P<0.001], and the average number of mechanical ventilation days [HR: 17 days (IQR: 12-27) vs. LR: 5 days (IQR: 2-9), P<0.001] were significantly higher in the HR CDH group. Post NICU discharge, the prevalence of asthma [HR: 20/54 (37%), vs. LR: 17/79 (22%), P=0.050)] and the total days of rehospitalization [HR: 9 (IQR: 2-27) vs. LR: 4 (IQR: 1-8), P=0.035] were significantly higher in HR group. Of the patients seen in the comprehensive multidisciplinary CDH clinic, obstructive lung disease measured by impulse oscillometry was increased in the HR CDH population compared to the reference group [median R5Hz was 12.95 kPa/(L/s) in CDH vs. 9.8 kPa/(L/s) (P=0.010)]. Conclusions: HR CDHSG Stage is associated with worse inpatient and long-term pulmonary outcomes.

Pulmonary hypertension screening in children with sickle cell disease.

BACKGROUND: Screening for pulmonary hypertension (PHT) is recommended in children with sickle cell disease (SCD). However, best approaches are poorly described. We examined the utility of PHT symptoms, echocardiogram (ECHO), N-terminal-pro hormone brain natriuretic peptide (NT-proBNP), and BNP to screen for PHT in the SCD pediatric population. METHODS: Children (8-18 years old) with SCD-HbSS and HbSthal° were prospectively included and underwent PHT screening. The screening consisted of a comprehensive PHT symptoms evaluation, ECHO measurement, and NT-proBNP and BNP levels. RESULTS: A total of 73 patients were included (mean age 12 ± 5.7 years; >80% on hydroxyurea), of which 37% had a symptom consistent with PHT, including exertional dyspnea (26.5%), fatigue (17.6%), palpitation (14.7%), and chest pain (10.3%). ECHO was obtained in 53 (72.6%) patients, with only ECHO of 48 patients included in the final analysis. Elevated ECHO peak tricuspid regurgitant jet velocity (TRV) >2.5 m/s or indirect findings to suggest PHT were seen in only two of 48 (4.2%). No significant differences were seen between those with and without PHT symptoms when compared for NT-proBNP, BNP, hemoglobin, pulmonary function testing, fractional exhaled nitric oxide, asthma, oxygen saturation, and sleep apnea. CONCLUSION: PHT symptoms are not consistent with ECHO, NT-proBNP nor BNP findings in children with SCD. PHT prevalence based on TRV was low in children on hydroxyurea, therefore screening may not be warranted for this group.

Sleep Apnea Screening in Children With Sickle Cell Anemia.

BACKGROUND: Screening for obstructive sleep apnea (OSA) is recommended by current guidelines in children with sickle cell anemia (SCA), but no specific approach is described. The Pediatric Sleep Questionnaire (PSQ) is a validated detection tool for OSA in children. We assessed the utility of PSQ to screen for OSA in children with concomitant SCA and snoring. MATERIALS AND METHODS: A prospective study, in children 4 to 18 years old with SCA. Subjects were assessed for snoring and PSQ administered at the same visit. All children with snoring were then referred for polysomnography. RESULTS: A total of 106 subjects were screened. Habitual snoring prevalence was 51/106 (48.1%). In the snoring group, OSA was detected in 83.9% (apnea-hypopnea index [AHI] ≥1.0/h) and 22.6% (AHI ≥5.0/h), respectively. Sensitivity and specificity of PSQ in children with snoring was 46.2% and 20.0% (AHI ≥1.0/h), and 57.1% and 50.0% (AHI ≥5.0/h), respectively. Physician assessment for snoring had a high sensitivity of 70.3% but low specificity of 58.4% (AHI ≥1.0/h), and 87.5% and 41.5% (AHI ≥5.0/h), respectively. CONCLUSION: PSQ is a poor screening tool for detection of OSA in those children with SCA who snore. Physician assessment for snoring could however be an initial approach before polysomnography.

Intravenous immunoglobulin as adjuvant therapy for COVID-19: A case report and literature review.

Severe acute respiratory syndrome coronavirus 2 has infected and caused the death of an alarming number of individuals worldwide. No specific treatment has been internationally standardized for coronavirus disease 2019 (COVID-19); however, in some cases, intravenous immunoglobulin (IVIG) has been used as adjuvant treatment in critically ill patients with COVID-19 pneumonia. We report a case of a 50-year-old man with severe COVID-19 pneumonia who received 5 days course of IVIG as adjuvant therapy. Invasive respiratory support was avoided. The patient had a successful recovery and was discharged without supplemental oxygen. A high dose of IVIG may improve survival in patients with severe COVID-19 pneumonia. In the current report, we reviewed literature on how IVIG use may improve the early stages of the disease.

Validation of the breathmobile case identification survey for asthma screening in children with sickle cell disease.

Background: Asthma is a chronic airway disorder with variable/recurring symptoms, airflow obstruction, bronchial hyperresponsiveness, and an inflammation. The expert panel report of the National Heart Lung and Blood Institute recommends asthma screening in sickle cell disease (SCD); however, specific approach is not mentioned. We hypothesize that the breathmobile case identification survey (BCIS) is a valid asthma screening tool in children with SCD.Methods: This prospective, single-center study enrolled 129 SCD patients aged 5 to 18 years from March 2016 to March 2018. All patients completed BCIS, spirometry, and fractional exhaled nitric oxide (FeNO). A single pulmonologist blinded to the BCIS results evaluated patients for asthma.Results: Asthma prevalence was 41%. Male gender (60.4%; p = 0.041), allergic rhinitis (86.8%; p < 0.01), hydroxyurea usage (73.6%; p < 0.01), and family history of asthma (34%; p < 0.01) were higher but not self-reported parental asthma history, eczema, and tobacco smoke exposure in the asthma group compared to the nonasthma group. FEV1 (p = 0.003), FVC (p = 0.02), FEV1/FVC (p = 0.053), and FEF25-75% (p = 0.02) were lower in asthma. FeNO levels were comparable in both groups. The sensitivity, specificity, positive predictive value, and negative predictive value of the abbreviated BCIS were 67.3%, 90.8%, 83.3%, and 80.2% for asthma; and 82.1%, 90.8%, 76.7%, and 93.2% for persistent asthma, respectively. Persistent asthma patients had a trend of higher hydroxyurea use (82.8% vs. 58.3%; p = 0.049) and tobacco smoke exposure (55.2% vs. 29.2%; p = 0.057) compared to intermittent asthma.Conclusion: We have validated the BCIS to screen for asthma in SCD. Spirometry but not FeNO may support an asthma diagnosis.

Cardiac arrhythmias associated with volume-assured pressure support mode in a patient with autonomic dysfunction and mitochondrial disease.

NONE: A 15-year-old boy with autonomic dysfunction and mitochondrial disease was diagnosed with sleep-related hypoventilation at 6 years of age and treated with bilevel positive airway pressure therapy. At 12 years of age, treatment was transitioned to volume-assured pressure support (VAPS) due to clinical evidence of respiratory muscle weakness. Subsequent titration polysomnogram revealed the emergence of cardiac arrhythmia (isolated premature ventricular contractions, bigeminy, and trigeminy) while on VAPS mode that improved after transition to bilevel positive airway pressure therapy. During the titration study, higher tidal volumes correlated with increased pressures and the presence of arrhythmia. Prior to initiation of VAPS therapy, the patient had normal electrocardiogram evaluations. This case highlights the potential relationship between VAPS therapy and cardiac arrhythmias, especially in patients with underlying conditions with associated cardiac abnormalities, such as autonomic dysfunction and mitochondrial disease. While using VAPS mode, patients should be closely monitored for cardiac rhythm abnormalities.

The Effect of Comprehensive Medical Care on the Long-Term Outcomes of Children Discharged from the NICU with Tracheostomy.

BACKGROUND: Survival of infants with complex care has led to a growing population of technology-dependent children. Medical technology introduces additional complexity to patient care. Outcomes after NICU discharge comparing Usual Care (UC) with Comprehensive Care (CC) remain elusive. OBJECTIVE: To compare the outcomes of technology-dependent infants discharged from NICU with tracheostomy following UC versus CC. METHODS: A single site retrospective study evaluated forty-three (N=43) technology-dependent infants discharged from NICU with tracheostomy over 5½ years (2011-2017). CC provided 24-hour accessible healthcare-providers using an enhanced medical home. Mortality, total hospital admissions, 30-days readmission rate, time-to-mechanical ventilation liberation, and time-to-decannulation were compared between groups. RESULTS: CC group showed significantly lower mortality (3.4%) versus UC (35.7%), RR, 0.09 [95%CI, 0.12-0.75], P=0.025. CC reduced total hospital admissions to 78 per 100 child-years versus 162 for UC; RR, 0.48 [95% CI, 0.25-0.93], P=0.03. The 30-day readmission rate was 21% compared to 36% in UC; RR, 0.58 [95% CI, 0.21-1.58], P=0.29). In competing-risk regression analysis (treating death as a competing-risk), hazard of having mechanical ventilation removal in CC was two times higher than UC; SHR, 2.19 [95% CI, 0.70-6.84]. There was no difference in time-to-decannulation between groups; SHR, 1.09 [95% CI, 0.37-3.15]. CONCLUSION: CC significantly decreased mortality, total number of hospital admissions and length of time-to-mechanical ventilation liberation.

Characteristics and Outcomes of Children with Clinical History of Atopic Versus Non-atopic Asthma Admitted to a Tertiary Pediatric Intensive Care Unit.

BACKGROUND: Children admitted to the Pediatric Intensive Care Unit (PICU) with status asthmaticus have variable clinical courses, and predicting their outcomes is challenging. Identifying characteristics in these patients that may require more intense intervention is important for clinical decision-making. OBJECTIVE: This study sought to determine the characteristics and outcomes, specifically length of stay and mortality, of atopic versus non-atopic asthmatics admitted to a PICU with status asthmaticus. METHODS: A retrospective study was conducted at a children's hospital from November 1, 2008 to October 31, 2013. A total of 90 children admitted to the PICU were included in the analysis. Patients were divided into two groups based on the presence of specific historical data indicative of a clinical history of atopy. Children were considered to be atopic if they had a parental history of asthma, a personal history of eczema, or a combined history of wheezing (apart from colds) and allergic rhinitis (diagnosed by a medical provider). The median hospital Length Of Stay (LOS), PICU LOS, cardiopulmonary arrest, and mortality were compared between atopic and non-atopic asthma groups. Regression models were used to estimate the LOS stratified by atopic or non-atopic and by history of intubation in present hospitalization. RESULTS: Median hospital LOS for atopic children was 5.9 days (IQR of 3.8-8.7) and 3.5 days (IQR of 2.2-5.5) for non-atopic asthmatics (z = 2.9, p = 0.0042). The median PICU LOS was 2.5 days (IQR 1.4-6.1) for atopic asthmatics and 1.6 days (IQR 1.1-2.4) for non-atopic asthmatics (z = 2.5, p = 0.0141). The median LOS was significantly higher for atopic intubated patients compared to non-atopic intubated patients (p=0.021). Although there was an increased tendency towards intubation in the atopic group, the difference was not significant. There was no significant difference in cardiopulmonary arrest or mortality. CONCLUSION: A clinical history of atopic asthma in children admitted to the PICU with status asthmaticus was associated with longer length of stays The longest LOS was observed when atopic patients required intubation.

Role of prophylactic azithromycin to reduce airway inflammation and mortality in a RSV mouse infection model.

BACKGROUND: Respiratory syncytial virus (RSV) infection is an important cause of morbidity and mortality in vulnerable populations. Macrolides have received considerable attention for their anti-inflammatory actions beyond their antibacterial effect. We hypothesize that prophylactic azithromycin will be effective in reducing the severity of RSV infection in a mouse model. METHODS: Four groups of BALB/c mice were studied for 8 days: Control (C), RSV-infected (R), early prophylaxis with daily azithromycin from days 1 to 8, (E), and late prophylaxis with daily azithromycin from days 4 to 8 (L). Mice were infected with RSV on day 4, except for the control group. All groups were followed for a total of 8 days when bronchoalveolar lavage cell count and cytokines levels were measured. Mouse weight, histopathology, and mortality data were obtained. RESULTS: Prophylactic azithromycin significantly attenuated post-viral weight loss between group R and both groups E and L (P = 0.0236, 0.0179, respectively). IL-6, IL-5, and Interferon-Gamma were significantly lower in group L (P = 0.0294, 0.0131, and 0.0056, respectively) compared with group R. The total cell count was significantly lower for group L as compared with group R (P < 0.05). Mortality was only observed in group R (8%). Lung histology in the prophylactic groups showed diminished inflammatory infiltrates and cellularity when compared with group R. CONCLUSION: Prophylactic azithromycin effectively reduced weight loss, airway inflammation, cytokine levels and mortality in RSV-infected mice. These results support the rationale for future clinical trials to evaluate the effects of prophylactic azithromycin for RSV infection.

Use of Nasal Non-Invasive Ventilation with a RAM Cannula in the Outpatient Home Setting.

BACKGROUND: Nasal non-invasive-ventilation (Nasal NIV) is a mode of ventilatory support providing positive pressure to patients via a nasal interface. The RAM Cannula is an oxygen delivery device that can be used as an alternative approach to deliver positive pressure. Together they have been successfully used to provide respiratory support in neonatal in-patient settings. OBJECTIVE: To describe the outpatient use of Nasal NIV/RAM Cannula as a feasible alternative for home respiratory support in children with chronic respiratory failure. METHODS: We performed a retrospective case series of 18 children (4 months to 19 years old) using the Nasal NIV/RAM Cannula in the Pediatric Pulmonary Clinic at the McGovern Medical School, UTHealth (2014-16). Consideration for Nasal NIV/RAM Cannula utilization included: inability to wean-off in-patient respiratory support, comfort for dyspnea, intolerability of conventional mask interfaces and tracheostomy avoidance. RESULTS: Average age was 7 years. 50% were Caucasian, 38% African-American and 11% Hispanics. Pulmonary disorders included: chest wall weakness (38%), central control abnormalities (33%), obstructive lung disease (16%) and restrictive lung disease (11%). Indications for Nasal NIV/RAM Cannula initiation included: CPAP/BPAP masks intolerability (11%), dyspnea secondary to chest wall weakness (38%) and tracheostomy avoidance (50%). Average length of use of Nasal NIV/RAM Cannula was 8.4 months. Successful implementation of Nasal NIV/Ram Cannula was 94%. One patient required a tracheostomy following the use of Nasal NIV/RAM Cannula. Significant decrease in arterial PaCO2 pre and post Nasal NIV/RAM cannula initiation was notable (p=0.001). CONCLUSION: Outpatient use of Nasal NIV/RAM Cannula may prove to be a feasible and save treatment alternative for children with chronic respiratory failure, chest wall weakness, dyspnea and traditional nasal/face mask intolerance to avoid tracheostomy.

Clinical outcomes of an inpatient pediatric obesity treatment program in the USA.

Background When treating recalcitrant and severe childhood obesity, pharmaceutical options are limited and few patients qualify for bariatric surgery. A prolonged inpatient program serves as an alternative treatment. The purpose of this project was to describe the development of a medically supervised inpatient weight management program and evaluate its effectiveness. Methods This is a retrospective chart review of 18 patients [4-18 years, mean body mass index (BMI) 50.2 kg/m2] admitted to an inpatient pediatric weight management program from October 2011 through December 31, 2012 to evaluate the biometric, laboratory, sleep and behavioral changes that occurred from admission to discharge from the program. Results Average weight loss was 15% (6.9%-21.5%, p = 0.0001), the decrease in BMI was 15.1% (1.61-21.57, p = 0.0001), systolic blood pressure and diastolic blood pressure decreased by 7.2% (p = 0.003) and 10.3% (p = 0.040), respectively. The reduction in heart rate was 15% (p = 0.013). Upon admission, nine patients had obstructive sleep apnea syndrome (OSAS), of which one was treated with tonsillectomy and six were not compliant with home positive airway pressure (PAP) therapy. At discharge, three patients no longer required PAP and five required decreased PAP settings. Upon admission, seven patients met the criteria for an internalizing disorder. At discharge, symptom reduction was noted. Conclusion An intensive pediatric inpatient weight management program leads to successful weight loss, improvement in hemodynamic parameters, reduction in OSA treatment requirements and symptom improvement in anxiety and depressive disorders in obese children.

Anti-inflammatory effect of prophylactic macrolides on children with chronic lung disease: a protocol for a double-blinded randomised controlled trial.

INTRODUCTION: Recent studies suggest that the high mortality rate of respiratory viral infections is a result of an overactive neutrophilic inflammatory response. Macrolides have anti-inflammatory properties, including the ability to downregulate the inflammatory cascade, attenuate excessive cytokine production in viral infections, and may reduce virus-related exacerbations. In this study, we will test the hypothesis that prophylactic macrolides will reduce the severity of respiratory viral illness in children with chronic lung disease by preventing the full activation of the inflammatory cascade. METHODS AND ANALYSIS: A randomised double-blind placebo-controlled trial that will enrol 92 children to receive either azithromycin or placebo for a period of 3-6 months during two respiratory syncytial virus (RSV) seasons (2015-2016 and 2016-2017). We expect a reduction of at least 20% in the total number of days of unscheduled face-to-face encounters in the treatment group as compared with placebo group. Standard frequentist and Bayesian analyses will be performed using an intent-to-treat approach. DISCUSSION: We predict that the prophylactic use of azithromycin will reduce the morbidity associated with respiratory viral infections during the winter season in patients with chronic lung disease as evidenced by a reduction in the total number of days with unscheduled face-to-face provider encounters. ETHICS AND DISSEMINATION: This research study was approved by the Institutional Review Board of the University of Texas Health Science Center in Houston on 9 October 2014. On completion, the results will be published. TRIAL REGISTRATION NUMBER: NCT02544984.

Management of obstructive sleep apnea in children: A practical approach.

The management of sleep disordered breathing (SDB) in children differs between institutions, and there is a need for an updated review of current practice. Literature was reviewed using the PubMed database from 1995 to 2015 by four tertiary care providers experienced in the management of children with SDB. Articles were selected for clinical applicability, strength of evidence, and practicality for practicing clinicians. Fifty-five articles were identified by tertiary care providers in pediatric anesthesiology, pediatric pulmonology, sleep medicine, and pediatric otolaryngology. Each reviewed and analyzed literature independently based on their specialties, and a consensus document was created. The consensus was that the majority of children with SDB do not undergo polysomnography (PSG) before adenotonsillectomy (T&A). Indications for PSG are presented, with a practical approach recommended for the otolaryngologist. Clinical practice guidelines are available from leading national societies, but their recommendations differ. T&A is the first-line treatment and is highly effective in normal-weight but not in obese children. The perioperative management of children is challenging and needs to be individualized. Young children, those with severe obstructive sleep apnea, and those with significant comorbidities need to be observed overnight.

Application of an Asthma Screening Questionnaire in Children with Sickle Cell Disease.

Objective: Asthma in sickle cell disease (SCD) patients is associated with elevated morbidity and mortality. Early detection and initiation of treatment may therefore lead to improved outcome. Utility of an asthma screening questionnaire to identify obstructive airway disease and physician diagnosed asthma in children with SCD at an outpatient setting as an effective, easy-to-administer screening tool has not previously been evaluated in this population. Methods: A previously validated asthma screening questionnaire and spirometry were prospectively administered to 41 SCD children at a routine clinic visit. Results: Prevalence of obstructive airway was 51.2% (n = 21) and physician diagnosis of asthma 33.3% (n = 13). Sensitivity (40%) and specificity (75%) of the questionnaire was poor in detecting obstructive airway disease, but sensitivity (77%), specificity (100%), positive predictive value (100%), and negative predictive value (90%) were high in detecting physician diagnosis of asthma. Conclusion: An asthma screening questionnaire could be a useful tool in identifying at-risk SCD children who may benefit from further management.

Resolution of airflow obstruction on polysomnography after laryngotracheal reconstruction with anterior tracheal wall suspension in a patient with DiGeorge Syndrome.

DiGeorge Syndrome (DGS) may be associated with airway abnormalities including laryngomalacia and suprastomal collapse of the trachea (SCT), which may lead to sleep disordered breathing (SDB). We present a 4-year-old boy with DGS, SCT, and SDB by polysomnography (PSG) while the tracheostomy tube was capped. The patient underwent anterior tracheal wall suspension (ATWS) with concurrent tracheostomy decannulation. Following the repair, the patient experienced improved airway patency visually and by PSG with resolution of obstructive sleep apnea and hypoventilation. ATWS is an effective method to repair SCT in selected patients and may lead to early decannulation and improvement of SDB.

Obstructive sleep apnoea and arthrogryposis.

Arthrogryposis is a rare condition characterised by multiple congenital joint contractures. We present a case of a 10-year-old child with arthrogryposis and snoring. Polysomnography revealed significant obstructive sleep apnoea and hypoventilation that improved but did not completely resolve with adenotonsillectomy. With continuous positive airway pressure (CPAP) therapy, there was full resolution of all sleep disordered breathing. Initially, the patient admitted to difficulty tolerating nasal CPAP at home. However, she steadily improved adherence to therapy and admitted that with nasal CPAP use for the whole night, she felt more energised during the daytime.

Airway obstruction in congenital central hypoventilation syndrome.

Congenital central hypoventilation syndrome (CCHS) is the failure of the autonomic system to control adequate ventilation while asleep with preserved ventilatory response while awake. We report a case of a patient with CCHS who presented with intrathoracic and extrathoracic airway obstruction after tracheostomy tube decannulation and phrenic nerve pacer placement. Nocturnal polysomnography (NPSG) revealed hypoxia, hypercapnia and obstructive sleep apnoea, which required bilevel positive airway pressure titration. Airway endoscopy demonstrated tracheomalacia and paretic true vocal cords in the paramedian position during diaphragmatic pacing. Laryngeal electromyography demonstrated muscular electrical impulses that correlated with diaphragmatic pacer settings. Thus, we surmise that the patient's upper and lower airway obstruction was secondary to diaphragmatic pacer activity. Thorough airway evaluation, including NPSG and endoscopy, may help identify the side effects of diaphragmatic pacing, such as airway obstruction, in patients with CCHS.

SFTPC gene mutation p.R167Q in a premature infant.

We present an infant who was born premature at 23 weeks gestation with bronchopulmonary dysplasia and a SFTPC gene mutation, p.R167Q, who had a complicated neonatal course requiring 4 months of mechanical ventilation. Over time, his clinical course has improved, and he only requires oxygen by nasal cannula and low dose hydroxychloroquine, suggesting that p.R167Q mutation contributed to his clinical course and may manifest with a variable disease pattern making long-term prognostication difficult in the immediate newborn period.

Sleep disordered breathing in children with mitochondrial disease.

A retrospective chart review study was performed to determine the presence of sleep disordered breathing (SDB) in children with primary mitochondrial disease (MD). The symptoms, sleep-related breathing, and movement abnormalities are described for 18 subjects (ages 1.5 to 18 years, 61% male) with MD who underwent polysomnography in our pediatric sleep center from 2007 to 2012. Of the 18 subjects with MD, the common indications for polysomnography were excessive somnolence or fatigue (61%, N = 11), snoring (44%, N = 8), and sleep movement complaints (17%, N = 3). Polysomnographic measurements showed SDB in 56% (N = 10) (obstructive sleep apnea in 60% (N = 6), hypoxemia in 40% (N = 4), and sleep hypoventilation in 20% (N = 2)). There was a significant association between decreased muscle tone and SDB (P: 0.043) as well as obese and overweight status with SDB (P = 0.036). SDB is common in subjects with MD. Early detection of SDB, utilizing polysomnography, should be considered to assist in identification of MD patients who may benefit from sleep-related interventions.

Early diagnosis and treatment of invasive pulmonary aspergillosis in a patient with cystic fibrosis.

Invasive pulmonary aspergillosis is a rare and fatal complication in patients with cystic fibrosis (CF) who lack concomitant risk factors. The few documented cases in children have all resulted in deaths during hospitalisation. We present the case of a 12-year-old boy with CF who was admitted for an exacerbation which was unresponsive to antibiotic therapy. The findings on imaging raised concerns about a possible fungal infection. As a result, voriconazole therapy was started prior to his respiratory deterioration. He was later found to be ß-D glucan and Aspergillus Ag galactomannan positive confirming the suspicion for invasive pulmonary aspergillosis. Three months after diagnosis, he was discharged home under stable condition. Voriconazole was continued beyond discharge and resulted in improvement of respiratory symptoms. This underscores the importance of early treatment of pulmonary aspergillosis in patients with CF. Unfortunately, the patient died 6 months after diagnosis from a CF exacerbation.