Management of Children With Chronic Wet Cough and Protracted Bacterial Bronchitis: CHEST Guideline and Expert Panel Report.

BACKGROUND: Wet or productive cough is common in children with chronic cough. We formulated recommendations based on systematic reviews related to the management of chronic wet cough in children (aged ≤ 14 years) based on two key questions: (1) how effective are antibiotics in improving the resolution of cough? If so, what antibiotic should be used and for how long? and (2) when should children be referred for further investigations? METHODS: We used the CHEST expert cough panel's protocol for systematic reviews and the American College of Chest Physicians (CHEST) methodologic guidelines and GRADE framework (the Grading of Recommendations Assessment, Development and Evaluation). Data from the systematic reviews in conjunction with patients' values and preferences and the clinical context were used to form recommendations. Delphi methodology was used to obtain consensus for the recommendations/suggestions made. RESULTS: Combining data from the systematic reviews, we found high-quality evidence in children aged ≤ 14 years with chronic (> 4 weeks' duration) wet/productive cough that using appropriate antibiotics improves cough resolution, and further investigations (eg, flexible bronchoscopy, chest CT scans, immunity tests) should be undertaken when specific cough pointers (eg, digital clubbing) are present. When the wet cough does not improve following 4 weeks of antibiotic treatment, there is moderate-quality evidence that further investigations should be considered to look for an underlying disease. New recommendations include the recognition of the clinical diagnostic entity of protracted bacterial bronchitis. CONCLUSIONS: Compared with the 2006 Cough Guidelines, there is now high-quality evidence for some, but not all, aspects of the management of chronic wet cough in specialist settings. However, further studies (particularly in primary health) are required.

Use of Management Pathways or Algorithms in Children With Chronic Cough: CHEST Guideline and Expert Panel Report.

BACKGROUND: Using management algorithms or pathways potentially improves clinical outcomes. We undertook systematic reviews to examine various aspects in the generic approach (use of cough algorithms and tests) to the management of chronic cough in children (aged ≤ 14 years) based on key questions (KQs) using the Population, Intervention, Comparison, Outcome format. METHODS: We used the CHEST Expert Cough Panel's protocol for the systematic reviews and the American College of Chest Physicians (CHEST) methodological guidelines and Grading of Recommendations Assessment, Development and Evaluation framework. Data from the systematic reviews in conjunction with patients' values and preferences and the clinical context were used to form recommendations. Delphi methodology was used to obtain the final grading. RESULTS: Combining data from systematic reviews addressing five KQs, we found high-quality evidence that a systematic approach to the management of chronic cough improves clinical outcomes. Although there was evidence from several pathways, the highest evidence was from the use of the CHEST approach. However, there was no or little evidence to address some of the KQs posed. CONCLUSIONS: Compared with the 2006 Cough Guidelines, there is now high-quality evidence that in children aged ≤ 14 years with chronic cough (> 4 weeks' duration), the use of cough management protocols (or algorithms) improves clinical outcomes, and cough management or testing algorithms should differ depending on the associated characteristics of the cough and clinical history. A chest radiograph and, when age appropriate, spirometry (pre- and post-ß2 agonist) should be undertaken. Other tests should not be routinely performed and undertaken in accordance with the clinical setting and the child's clinical symptoms and signs (eg, tests for tuberculosis when the child has been exposed).

Tracheomalacia is associated with lower FEV1 and Pseudomonas acquisition in children with CF.

BACKGROUND: Tracheomalacia (TM) occurs in approximately 1 in 2,100 children. Because the trachea develops abnormally in animal models of cystic fibrosis (CF), we hypothesized this may also occur in children with CF, increasing their risk of TM. PURPOSE: To examine the prevalence and clinical consequences of TM in children with CF. METHODS: We studied children with CF born between 1995 and 2012. TM was defined as dynamic collapse of the trachea, and the severity was recorded as described in the chart. The effect of TM on patient outcomes, including FEV1 , CT changes, and acquisition of CF pathogens, was assessed using a longitudinal patient dataset. RESULTS: Eighty-nine percent of children with CF had at least one bronchoscopy (n = 97/109). Fifteen percent of these children had TM described in any bronchoscopy report (n = 15/97). Of the patients with TM, eight had meconium ileus (P = 0.003) and all were pancreatic insufficient. Pseudomonas aeruginosa infection occurred 1.3 years earlier among children with TM (P = 0.01). Starting FEV1 values by age 8 were diminished by over 18% of predicted for patients with TM. Life-threatening episodes of airway obstruction occurred in 3 of 15 patients with CF and TM, including one leading to death. Gender, prematurity, and hepatic disease were not associated with TM. No difference was observed in the frequency of bronchiectasis. CONCLUSIONS: TM is significantly more common in infants and children with CF than in the general population and is associated with airway obstruction and earlier Pseudomonas acquisition.

Growth of preschool age children receiving daily inhaled corticosteroids.

Data on the effects of inhaled corticosteroid (ICS) on linear growth in children <5 years old are limited with conflicting results from existing studies. This study was designed to investigate growth effects of inhaled corticosteroid use in children <5 years of age treated for asthma with ICS administered through a valved holding chamber (VHC). A retrospective cohort study was performed of 145 children in three treatment groups: (1) metered-dose inhaler (MDI) containing ultrafine beclomethasone dipropionate (n = 62), (2) MDI containing fluticasone propionate (n = 32), and (3) oral montelukast sodium (n = 51). Children <5 years of age between 2000 and 2009 treated for asthma with one of the three drugs were included in the study. Linear mixed model analysis was used to examine and compare growth during sustained treatment with each of the three medications. The three treatment groups did not differ significantly in their effect on growth rates (p = 0.64). However, female subjects had significantly slower growth than male subjects (p = 0.017), and the addition of intranasal corticosteroids (INS; p = 0.013) and the presence of atopy (p = 0.015) had a significant negative effect on growth. In children <5 years of age receiving maintenance therapy for chronic asthma, low-to-medium doses of ultrafine beclomethasone or fluticasone administered through a VHC were not associated with growth inhibition compared with children receiving oral montelukast. A small but statistically significant decrease in growth was seen in subjects with positive skin testing to inhalant allergens in female subjects and in subjects receiving INS.

Experience with cyclosporine in children with chronic idiopathic urticaria.

BACKGROUND: The identification of an autoimmune mechanism for many patients with chronic idiopathic urticaria (CIU) was used as a rational for a controlled clinical trial of cyclosporine for adults with CIU not responsive to usual measures. That randomized placebo controlled clinical trial demonstrated clinical efficacy, acceptable safety, and a suggestion of inducing remission in such patients. OBJECTIVE: To report our experience with cyclosporine in pediatric patients with CIU. METHODS: Fifty-four patients with CIU were referred to us during the period from 2000 through June of 2005. Seven of those, aged 9-16, failed therapy with high dose antihistamines even with the addition of alternate morning prednisone. Neoral brand of cyclosporine, 3 mg/kg/day divided b.i.d., was initiated in these patients. Cyclosporine serum concentrations, blood urea nitrogen (BUN), creatinine, and blood pressure were routinely monitored. RESULTS: All had cessation of hives. This occurred after 1-4 weeks for six of the seven and 8 weeks for one. While some experienced relapses, all were eventually off of all medications and free of hives. None of the seven experienced any adverse effects. CONCLUSIONS: Our experience in children is consistent with a previous controlled clinical trial in adults and supports the efficacy and safety of cyclosporine for CIU. However, we recommend that it be reserved for those whose CIU that is resistant to conventional measures and that patients be carefully monitored with cyclosporine serum concentrations and measures of renal function.

Chronic cough and tonsillar hypertrophy: a case series.

Two patients with intractable chronic cough were found to have tonsillar tissue impinging on their epiglottis. In both case, tonsillectomy was curative. The observations in these patients are consistent with a previous report indicating chronic cough from the uvula in contact with the epiglottis with cough cessation following uvulectomy.

Achalasia: unusual cause of chronic cough in children.

Achalasia is a rare motility disorder of the esophagus which results from lack of enervation of the lower esophageal sphincter muscles and leads to dilatation of proximal esophagus. Patients with achalasia presents typically with dysphagia, vomiting of undigested food and failure to thrive. Cough can be present in achalasia patients due to aspiration of food or due to airway compression by the dilated esophagus. We report two cases of achalasia presenting primarily with prolonged cough. Diagnosis of achalasia in both cases was delayed due to this atypical presentation. This highlights the importance of recognizing achalasia as a potential cause of chronic cough in order to avoid delayed diagnosis and mismanagement.

Long-term outcome of vocal cord dysfunction.

BACKGROUND: Vocal cord dysfunction (VCD) is an involuntary functional disorder commonly misdiagnosed as asthma. Previous reports describe the disorder and treatment but not the long-term outcome. OBJECTIVE: To determine the long-term outcome of VCD. METHODS: A retrospective medical record review identified 49 patients, ages 8 to 25 years, diagnosed as having VCD from 1989 to 2002. Telephone contact was attempted in all. RESULTS: Of the 49 patients, 41 had previously been treated for asthma; that diagnosis was confirmed by us as a comorbidity in only 12 patients. Two distinct phenotypes of VCD were observed. Symptoms were limited to exercise-induced VCD (EIVCD) in 29 and spontaneously occurring VCD (SVCD) in 20, only 4 of whom additionally had EIVCD. Twenty-eight of the 49 were successfully contacted by telephone. Eight of the 11 contacted patients with SVCD followed the recommendation to see our speech therapist, all of whom learned to control symptoms. However, 2 who also had EIVCD continued with that problem. Pretreatment with an anticholinergic inhaler prevented EIVCD in 6 patients in whom this was tried. Complete absence of symptoms, at times ranging from 1 week to 5 years (median, 5 months), was reported in 26 of the 28 contacted patients. CONCLUSIONS: VCD continues to be frequently misdiagnosed as asthma. Two phenotypes of VCD are apparent: EIVCD and SVCD. Speech therapy provides relief of symptoms for SVCD. Prevention of EIVCD with an anticholinergic inhaler in 6 patients suggests that a controlled clinical trial is warranted. Regardless of treatment, eventual spontaneous resolution was common.