Cardiopulmonary Function Abnormalities in Cohort of Adults following Bronchopulmonary Dysplasia as Preterm Infants.

OBJECTIVE: This study was aimed to describe the cardiopulmonary profiles of adult patients with bronchopulmonary dysplasia (BPD), comparing them to normative adult values. STUDY DESIGN: This study presents a retrospective chart review of all BPD patients followed in the adult BPD clinic, identified from institutional and archive databases, born preterm at ≤33 weeks of estimated gestational age (EGA) between January 1980 and December 2000. RESULTS: Forty-four patients with BPD (26.4 ± 2.7 weeks of EGA) were included. Average age at follow-up was 19 years. Majority (61.4%) of the patients had a diagnosis of asthma. Mean spirometry values were: first second of forced expiration (FEV1) 74.1%, forced vital capacity (FVC) 80.7%, and FEV1/FVC 82.5%. Echocardiography (ECHO) images were reviewed, left ventricular (LV) structure and performance did not differ between obstructive and nonobstructive pulmonary function test (PFT) groups, but values of LV longitudinal strain were 4.8% lower than expected normal for adults. Patients with obstructive PFT had additional decreased right ventricular (RV) function by ECHO. CONCLUSION: BPD patients in this study were found to have a burden of cardiorespiratory alterations that persisted into adulthood, with RV performance abnormalities found among patients with obstructive PFT. KEY POINTS: · BPD patients born at extremes of prematurity have cardiorespiratory alterations in adulthood.. · Among patients with obstructive lung function, subtle cardiac performance abnormalities were found.. · Future directions should include systematic follow-up of premature newborns with BPD..

Recognizing clinical features of primary ciliary dyskinesia in the perinatal period.

Primary ciliary dyskinesia (PCD) is a rare, motile ciliopathy inherited through mostly autosomal recessive variants that results in chronic ear, sinus, and respiratory disease. Despite neonatal respiratory distress being a common presenting symptom in term infants with PCD, the diagnosis is often delayed due to non-familiarity of neonatal caregivers with phenotypic and diagnostic features of this disease. Organ laterality defects, prenatal cerebral ventriculomegaly, and a family history of suppurative respiratory disease may occur in PCD and should prompt neonatal testing for this condition. In this review of neonatal PCD diagnoses in a large PCD clinic, prevalence and details of neonatal PCD issues are presented, highlighting the typically delayed onset of neonatal respiratory distress and lobar atelectasis on chest radiography, specific presentations in premature neonates, and responses to perinatal therapies.

Treatment of severe bronchiolitis: A survey of Canadian pediatric intensivists.

OBJECTIVE: To describe management practices and the factors guiding admission and treatment decisions for viral bronchiolitis across Canadian pediatric intensive care units (PICUs). DESIGN: Cross-sectional survey. SETTING: Canadian PICUs. SUBJECTS: Pediatric intensivists. MEASUREMENTS AND MAIN RESULTS: A survey using two case scenarios (non-intubated vs intubated patients) was developed using focus groups and a literature review. We analyzed our results using descriptive statistics and multivariate logistic regression. Our response rate was 55% (57/103). Regarding bronchiolitis management, 75% (42/56) of respondents would use inhaled therapies, with nebulized epinephrine (33/56, 59%) and salbutamol (20/56, 36%) being the most common. Antibiotic use within the first hour of admission to PICU almost doubled in frequency (36% vs 71%) in patients who required mechanical ventilation (p 0.0004). High flow nasal cannula (HFNC; 32/56, 57%) and continuous positive airway pressure (CPAP; 16/56, 29%) were the preferred modes of non-invasive ventilation (NIV). CONCLUSION: The management of severe viral bronchiolitis is similar across Canadian PICUs. The use of NIV, inhaled treatments, and antibiotics is frequent, which differs from the recommendations made by published guidelines. Canadian pediatric intensivists use homogeneous PICU admission criteria based on patients' characteristics and severity of the clinical picture. Clinical practice guidelines for children with viral bronchiolitis should address the management of patients with severe clinical disease.

Do in-line respiratory filters protect patients? Comparing bacterial removal efficiency of six filters.

With all pulmonary function diagnostic and respiratory therapy equipment, cross-infection has always been a concern, especially in the cystic fibrosis population, in whom pulmonary function tests are done routinely. The aim of this study was to identify and compare the bacterial removal efficiency (BRE, ability of a filter to remove microorganisms) of six different filters used in hospital settings: Microgard (MG), Spirobac (SB), PALL (PL), and KOKO (KK), used in the pulmonary function laboratory; and Clear-Guard (CG) and Respigard (RG), used in ventilator circuits. Filters were tested in both saturated and nonsaturated conditions. A Pseudomonas aeruginosa suspension of 1 x 10(4) to 1 x 10(8) CFU/mL was nebulized onto each filter. A blood agar plate was held immediately downstream from the filter. Colony-forming units (CFU) were then counted after 24 hr of incubation. A peak flow was applied across the spirometry filters. Bacterial thresholds of the filters were also identified (concentration of bacteria at which a filter no longer has 100% BRE). There was a significant difference in BRE among the six filters in saturated states when challenged with 1 x 10(4) CFU/mL (MG, KK, CG, and RG, 100%; SB, 98.8%; PL, 42.7%; P = 0.003). There was no significant difference between saturated and nonsaturated states, or after application of a peak flow. Filter thresholds were significantly different (KK 1 x 10(8), MG 1 x 10(7), CG 1 x 10(6), RG 1 x 10(5), and SB and PL <1 x 10(4) CFU/mL). In conclusion, when all filters are exposed to the same extreme challenges, significant differences exist in their ability to remove bacteria.

Pulmonary interstitial glycogenosis: a new variant of neonatal interstitial lung disease.

We present the clinical, radiologic, and pathologic findings in lung biopsies from seven infants with atypical neonatal lung disease. All seven infants presented with tachypnea, hypoxemia, and diffuse interstitial infiltrates with overinflated lungs on chest radiographs in the first month of life. Lung biopsies from all cases showed similar pathology, with expansion of the interstitium by spindle-shaped cells containing periodic acid-Schiff positive diastase labile material consistent with glycogen. Immunohistochemical staining showed these cells to be vimentin positive but negative for leucocyte common antigen, lysozyme, and other macrophage markers. Electron microscopy revealed primitive interstitial mesenchymal cells with few cytoplasmic organelles and abundant monoparticulate glycogen. Minimal or no glycogen was seen in the alveolar lining cells. Five cases were treated with pulse corticosteroids; hydroxychloroquine was added in one case. Six of seven infants have shown a favorable clinical outcome. One infant died from complications of extreme prematurity and bronchopulmonary dysplasia. Three cases that have been followed for at least 6 years have shown clinical resolution and radiographic improvement. We propose the term "pulmonary interstitial glycogenosis" of the neonate for this new entity to be differentiated from other forms of interstitial lung disease. Because abundant glycogen is not normally found in pulmonary interstitial cells, we postulate an abnormality in lung cytodifferentiation involving interstitial mesenchymal cells.