phenylephrine-hydrochloride and Bronchiectasis

Primary ciliary dyskinesia (PCD) is a multi-organ disorder associated with chronic oto-sino-pulmonary disease, neonatal respiratory distress, situs abnormalities and reduced fertility. Repeated respiratory tract infections leads to the almost universal development of bronchiectasis. These clinical manifestations are a consequence of poorly functioning motile cilia. However, confirming the diagnosis is quite difficult and is often delayed, so the true incidence of PCD may be significantly higher than current estimates. Nasal nitric oxide has been earmarked as a useful screening tool for identifying patients, but its use is limited in pre-school-aged children. Due to the rarity of PCD, the evidence base for management is somewhat limited, and treatment regimens are extrapolated from other suppurative lung disorders, like cystic fibrosis.

Topics: Bronchiectasis; Child; Diagnosis, Differential; Early Diagnosis; Humans; Kartagener Syndrome; Nitric Oxide; Nose; Rare Diseases; Respiratory Tract Infections

The use of positive-pressure nasal ventilation in combination with LTOT in stable COPD patients with hypercapnic respiratory failure controls hypoventilation and improves daytime ABGs, sleep, and quality of life. Nasal ventilation in COPD is unlikely to produce benefit unless used with supplemental oxygen therapy at night. The patients who show the greatest reduction in overnight PaCO2 with ventilation are the patients most likely to benefit from long-term ventilatory support. Although there is now evidence for short-term benefit from NPPV in hypercapnic COPD, large multicenter studies with survival, exacerbations, and hospital admissions as the primary end points are required to evaluate longer-term effects of this potentially important intervention.

Topics: Bronchiectasis; Cystic Fibrosis; Home Care Services; Humans; Hypercapnia; Long-Term Care; Lung Diseases, Obstructive; Nose; Oxygen Inhalation Therapy; Positive-Pressure Respiration; Respiratory Mechanics; Ventilators, Negative-Pressure

Indigenous children in high-income countries have a heavy burden of bronchiectasis unrelated to cystic fibrosis. We aimed to establish whether long-term azithromycin reduced pulmonary exacerbations in Indigenous children with non-cystic-fibrosis bronchiectasis or chronic suppurative lung disease.. Between Nov 12, 2008, and Dec 23, 2010, we enrolled Indigenous Australian, Maori, and Pacific Island children aged 1-8 years with either bronchiectasis or chronic suppurative lung disease into a multicentre, double-blind, randomised, parallel-group, placebo-controlled trial. Eligible children had had at least one pulmonary exacerbation in the previous 12 months. Children were randomised (1:1 ratio, by computer-generated sequence with permuted block design, stratified by study site and exacerbation frequency [1-2 vs ≥3 episodes in the preceding 12 months]) to receive either azithromycin (30 mg/kg) or placebo once a week for up to 24 months. Allocation concealment was achieved by double-sealed, opaque envelopes; participants, caregivers, and study personnel were masked to assignment until after data analysis. The primary outcome was exacerbation (respiratory episodes treated with antibiotics) rate. Analysis of the primary endpoint was by intention to treat. At enrolment and at their final clinic visits, children had deep nasal swabs collected, which we analysed for antibiotic-resistant bacteria. This study is registered with the Australian New Zealand Clinical Trials Registry; ACTRN12610000383066.. 45 children were assigned to azithromycin and 44 to placebo. The study was stopped early for feasibility reasons on Dec 31, 2011, thus children received the intervention for 12-24 months. The mean treatment duration was 20·7 months (SD 5·7), with a total of 902 child-months in the azithromycin group and 875 child-months in the placebo group. Compared with the placebo group, children receiving azithromycin had significantly lower exacerbation rates (incidence rate ratio 0·50; 95% CI 0·35-0·71; p<0·0001). However, children in the azithromycin group developed significantly higher carriage of azithromycin-resistant bacteria (19 of 41, 46%) than those receiving placebo (four of 37, 11%; p=0·002). The most common adverse events were non-pulmonary infections (71 of 112 events in the azithromycin group vs 132 of 209 events in the placebo group) and bronchiectasis-related events (episodes or investigations; 22 of 112 events in the azithromycin group vs 48 of 209 events in the placebo group); however, study drugs were well tolerated with no serious adverse events being attributed to the intervention.. Once-weekly azithromycin for up to 24 months decreased pulmonary exacerbations in Indigenous children with non-cystic-fibrosis bronchiectasis or chronic suppurative lung disease. However, this strategy was also accompanied by increased carriage of azithromycin-resistant bacteria, the clinical consequences of which are uncertain, and will need careful monitoring and further study.. National Health and Medical Research Council (Australia) and Health Research Council (New Zealand).

Topics: Anti-Bacterial Agents; Australia; Azithromycin; Bronchiectasis; Carrier State; Child; Child, Preschool; Chronic Disease; Disease Progression; Double-Blind Method; Drug Resistance, Bacterial; Early Termination of Clinical Trials; Episode of Care; Female; Haemophilus influenzae; Humans; Infant; Intention to Treat Analysis; Length of Stay; Lung Diseases; Male; Microbial Sensitivity Tests; Moraxella catarrhalis; Native Hawaiian or Other Pacific Islander; Nose; Severity of Illness Index; Staphylococcus aureus; Streptococcus pneumoniae; Suppuration; Time Factors

The levels of exhaled and nasal nitric oxide (eNO and nNO) in groups of patients with inflammatory lung diseases are well documented but the diagnostic use of these measurements in an individual is unknown.. The levels of nNO and eNO were compared in 31 children with primary ciliary dyskinesia (PCD), 21 with non-CF bronchiectasis (Bx), 17 with cystic fibrosis (CF), 35 with asthma (A), and 53 healthy controls (C) using a chemiluminescence NO analyser. A diagnostic receiver-operator characteristic (ROC) curve for PCD using NO was constructed.. The median (range) levels of nNO in parts per billion (ppb) in PCD, Bx, CF, and C were 60.3 (3.3-920), 533.6 (80-2053), 491.3 (31-1140), and 716 (398-1437), respectively; nNO levels were significantly lower in PCD than in all other groups (p<0.05). The median (range) levels of eNO in ppb in PCD, Bx, CF, A, and C were 2.0 (0.2-5.2), 5.4 (1.0-22.1), 2.6 (0.8-12.9), 10.7 (1.6-46.7), and 4.85 (2.5-18.3), respectively. The difference in eNO levels in PCD reached significance (p<0.05) when compared with those in Bx, A and C but not when compared with CF. Using the ROC curve, nNO of 250 ppb showed a sensitivity of 97% and a specificity of 90% for the diagnosis of PCD.. eNO and nNO cannot be used diagnostically to distinguish between most respiratory diseases. However, nNO in particular is a quick and useful diagnostic marker which may be used to screen patients with a clinical suspicion of PCD.

Topics: Adolescent; Asthma; Biomarkers; Breath Tests; Bronchiectasis; Child; Cystic Fibrosis; Forced Expiratory Volume; Humans; Kartagener Syndrome; Nitric Oxide; Nose; ROC Curve

Hydrogen cyanide (HCN) in exhaled breath has been proposed as a biomarker for airway inflammation, and also a marker of the presence in the airways of specific organisms, especially Pseudomonas aeruginosa. However the production of HCN by salivary peroxidase in the oral cavity increases orally exhaled concentrations, and may not reflect the condition of the lower airways. Using SIFT-MS we aimed to determine an appropriate single-exhalation breathing maneuver which avoids the interference of HCN produced in the oral cavity. We have established that the SIFT-MS Voice200™ is suitable for the online measurement of HCN in exhaled breath. In healthy volunteers a significantly higher end exhaled HCN concentration was measured in oral exhalations compared to nasal exhalations (mean ± SD) 4.5 ± 0.6 ppb versus 2.4 ± 0.3 ppb, p < 0.01. For the accurate and reproducible quantification of end exhaled HCN in breath a nasal inhalation to full vital capacity and nasal exhalation at controlled flow is recommended. This technique was subsequently used to measure exhaled HCN in a group of patients with chronic suppurative lung disease (CSLD) and known microbiological colonization status to determine utility of HCN measurement to detect P. aeruginosa. Median nasal end exhaled HCN concentrations were higher in patients with CSLD (3.7 ppb) than normal subjects (2.0 ppb). However no differences between exhaled HCN concentrations of subjects colonized with P. aeruginosa and other organisms were identified, indicating that breath HCN is not a suitable biomarker of P. aeruginosa colonization.

Topics: Adult; Biomarkers; Breath Tests; Bronchiectasis; Case-Control Studies; Cystic Fibrosis; Exhalation; Female; Humans; Hydrogen Cyanide; Inhalation; Male; Mass Spectrometry; Middle Aged; Mouth; Nose; Pseudomonas aeruginosa; Pseudomonas Infections; Reproducibility of Results

Primary ciliary dyskinesia (PCD) is associated with chronic airway inflammation resulting in bronchiectasis.. The levels of exhaled nitric oxide (eNO), carbon monoxide (eCO) and nasal NO (nNO) from bronchiectatic patients with PCD (n=14) were compared with those from patients with non-PCD bronchiectasis without (n=31) and with cystic fibrosis (CF) (n=20) and from normal subjects (n=37) to assess the clinical usefulness of these measurements in discriminating between PCD and other causes of bronchiectasis.. Exhaled NO levels were lower in patients with PCD than in patients with non-PCD non-CF bronchiectasis or healthy subjects (median (range) 2.1 (1.3-3.5) ppb v 8.7 (4.5-26.0) ppb, p<0.001; 6.7 (2.6-11.9) ppb, p<0.001, respectively) but not lower than bronchiectatic patients with CF (3.0 (1.5-7.5) ppb, p>0.05). Nasal levels of nNO were significantly lower in PCD patients than in any other subjects (PCD: 54.5 (5.0-269) ppb, non-PCD bronchiectasis without CF: 680 (310-1000) ppb, non-PCD bronchiectasis with CF: 343 (30-997) ppb, control: 663 (322-1343) ppb). In contrast, eCO levels were higher in all patient groups than in control subjects (PCD: 4.5 (3.0-24.0) ppm, p<0.01, other bronchiectasis without CF: 5.0 (3.0-15.0) ppm, p<0.001; CF: 5.3 (2.0-23.0) ppm, p<0.001 v 3.0 (0.5-5.0) ppm). Low values in both eNO and nNO readings (<2.4 ppb and <187 ppb, respectively) identified PCD patients from other bronchiectatic patients with a specificity of 98% and a positive predictive value of 92%.. The simultaneous measurement of eNO and nNO is a useful screening tool for PCD.

Topics: Adult; Breath Tests; Bronchiectasis; Carbon Monoxide; Ciliary Motility Disorders; Female; Forced Expiratory Volume; Humans; Male; Mouth Breathing; Nitric Oxide; Nose; Sensitivity and Specificity; Vital Capacity

Pulse delivery (PD) of oxygen was compared with continuous flow (CF) utilizing transtracheal oxygen catheter (TTO) and nasal cannula (NC) in 20 stable patients with chronic hypoxemia. Oxygen saturation, respiratory rate, and accuracy of pulsed oxygen delivery were measured during sleep studies and these parameters, as well as arterial blood gases, were evaluated during rest and exercise. Additionally, bulk liquid oxygen use was measured under each condition, for a period of 1 month. Pulse delivery NC was evaluated in six subjects, CF NC in 14 subjects, and PD and CF TTO in 20 subjects over the 1-month period. Results showed that, as a group, patients were adequately oxygenated when utilizing the PD with both NC and TTO as assessed by arterial blood gases, oximetry, and hematocrit. However, four subjects could not be adequately oxygenated on PD NC during exercise even at the maximum liter per minute setting and could not be studied with this mode of therapy. The PD settings in the remaining subjects were equivalent to continuous flow settings for TTO and NC as assessed by PaO2 for rest and SaO2 for exercise and sleep. Compared with standard CF NC, the daily bulk oxygen use was decreased by 29.4 percent with CF TTO, by 48.2 percent with PD NC, and by 49.9 percent with PD TTO. We conclude that, compared with CF NC, PD of oxygen via TTO or NC by this method appears to be a safe, reliable, effective, and cost-effective method of oxygen delivery in the majority of subjects when used with proper screening.

Topics: Aged; Analysis of Variance; Bronchiectasis; Catheterization; Chronic Disease; Evaluation Studies as Topic; Exercise Test; Female; Humans; Hypoxia; Lung Diseases, Obstructive; Male; Middle Aged; Nose; Oxygen Inhalation Therapy; Polysomnography; Trachea

Previous work confirmed the abnormal potential difference between the undersurface of the inferior nasal turbinate and a reference electrode in cystic fibrosis, but the technique is difficult and the results show overlap between the cystic fibrosis and the control populations. In the present study the potential difference from the floor of the nose has therefore been assessed in normal subjects, as well as in adult patients with cystic fibrosis, bronchiectasis and Young's syndrome. Voltages existing along the floor of the nasal cavity were recorded. The mean potential difference was similar in controls (-18 (SD 5) mv) and in patients with bronchiectasis (-17 (6) mv) and Young's syndrome (-20 (6) mv). The potential difference in cystic fibrosis (-45 (8) mv) was significantly different from controls (p less than 0.002) and there was no overlap between the cystic fibrosis values and values obtained in normal and diseased controls. This simple technique therefore discriminates well between patients with cystic fibrosis and other populations, raising the possibility of its use to assist in diagnosis.

Topics: Adolescent; Adult; Amyotrophic Lateral Sclerosis; Bronchiectasis; Cystic Fibrosis; Female; Humans; Male; Membrane Potentials; Nose; Platybasia; Syndrome

Nasal mucociliary clearance was measured using a saccharin technique in 172 patients with perennial rhinitis (76 also had asthma) and in 121 patients with chronic infected rhinosinusitis (40 had asthma, 35 had bronchiectasis). All patient groups had significantly longer mean nasal mucociliary clearance times than that of a group of healthy subjects. Grossly prolonged clearance (greater than 60 minutes) occurred in significantly more patients with the clinical syndrome of chronic infected rhinosinusitis and bronchiectasis than in the syndromes of chronic infected rhinosinusitis with or without asthma, and perennial rhinitis with or without asthma. The abnormal clearance was shown not to be due to an intrinsic ciliary defect by in vitro examination of nasal cilia but probably to be due to a combination of mucus and ciliary factors in vivo.

Topics: Adolescent; Adult; Asthma; Bronchiectasis; Cilia; Female; Humans; Male; Middle Aged; Mucus; Nose; Rhinitis; Rhinitis, Allergic, Perennial; Rhinitis, Allergic, Seasonal; Saccharin; Sinusitis

Four girls born to second cousin parents developed chronic chest infection and bronchiectasis in infancy. Three were studied in detail: they all had the same HLA haplotype, all showed random orientation of cilia or compound cilia in the respiratory tract, and all had low levels of the C1 and C2 components of the complement system. Although the cause of the respiratory disease in this family remains unclear, it is suggested that the low C1 levels may have contributed to the disease in two of the children while the low C2 levels were artefacts and the ciliary abnormalities were secondary to chronic chest infection.

Topics: Bronchiectasis; Child; Child, Preschool; Cilia; Complement C1; Complement C2; Female; Humans; Male; Nose; Recurrence; Respiratory Tract Infections

Usually, nasal polyposis in early childhood (children aged less than 5 years) is caused by cystic fibrosis of Kartagener's syndrome. In later age groups, recurrent sinus infections, allergy and ASA disease (asthma, aspirin intolerance and nasal polyps) have to be taken into consideration. Four cases of early childhood polyposis are reported which fit into none of these etiological groups. This newly defined Woakes' syndrome comprises recurrent nasal polyposis with broadening of the nose, frontal sinus aplasia, bronchiectasis, and dyscrinia (production of highly viscous mucus). The disease seems to be hereditary. The possible origins of the disease are discussed.

Topics: Bronchiectasis; Child; Child, Preschool; Humans; Infant; Infant, Newborn; Nasal Polyps; Nose; Nose Neoplasms; Syndrome

Topics: Adenoviridae Infections; Ampicillin; Anal Canal; Bronchiectasis; Child; Child, Preschool; Cloxacillin; Complement Fixation Tests; Female; Finland; Follow-Up Studies; Gastroenteritis; Heart Diseases; Hemorrhagic Disorders; Hepatomegaly; Humans; Infant; Kidney Diseases; Male; Meningism; Meningoencephalitis; Nose; Parasympatholytics; Penicillins; Pneumonia, Viral; Pulmonary Fibrosis; Radiography

Topics: Bronchiectasis; Humans; Hypersensitivity; Immune System Diseases; Nose

Topics: Bronchi; Bronchial Diseases; Bronchiectasis; Disease; Humans; Nose; Respiration Disorders

Topics: Bronchiectasis; Humans; Kartagener Syndrome; Larynx; Nose; Trachea; Ventilators, Mechanical; Viscera

Topics: Bronchiectasis; Constitution and Bylaws; Humans; Nasal Polyps; Neoplasms; Nose; Nose Neoplasms; Viscera