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Causes of chronic cough in children


Authors: Julie M Marchant, MBBS, FRACP, PhD, Anne B Chang, MBBS, FRACP, PhD, FAPSR, FAHMS
Section Editor: George B Mallory, MD
Deputy Editor: Alison G Hoppin, MD

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Dec 2019. | This topic last updated: Nov 11, 2019.

INTRODUCTION

There are many causes of cough; the majority originate in the lungs, but there are also nonpulmonary etiologies, some proven
and others controversial. The causes are presented in the table (table 1).

The causes of chronic cough in children will be reviewed here. The evaluation of a child with chronic cough should include a
detailed history, physical examination, chest radiograph, and spirometry and follow a child-specific algorithm [1-3]; the clinical
approach is summarized in the algorithm (algorithm 1) and discussed in more detail in a separate topic review. (See "Approach
to chronic cough in children".)

DEFINITIONS

Chronic cough — Chronic cough in children aged 14 years and younger is typically defined as a cough lasting more than four
weeks [1,2,4]. This definition is used by most published international guidelines (American, European, and Australian) because
most acute respiratory infections in children resolve within this interval and evaluation at four weeks permits relatively early
diagnosis of serious underlying illnesses. (See "Approach to chronic cough in children", section on 'Chronic cough'.)

Specific cough — Specific cough refers to a chronic cough that is ultimately attributable to an underlying abnormality or
disease, which is usually, but not always, of pulmonary origin (table 1). In most cases of specific cough the initial evaluation will
identify signs or symptoms suggesting the cause of the cough (known as "specific cough pointers"). These include symptoms
such as wet cough or wheeze; timing, such as onset in neonatal period; associated conditions, such as failure to thrive or
digital clubbing; abnormalities on chest radiograph or spirometry (table 2); or classically recognizable cough sounds (eg, brassy
or croup-like) (table 3). Many of these pointers are easily recognizable and are strong predictors of a specific cause. A specific
cause of cough is very unlikely if no pointers are found on the initial evaluation [5]. One of the most important and
discriminating pointers is presence of wet or productive cough [5]. A chronic wet cough signifies the presence of airway
secretions [6], and in most cases airway infection, and should not be ignored in children [7,8].

The main causes of specific cough are described in the remainder of this topic review.

Nonspecific cough — Conversely, "nonspecific" cough is defined as a chronic cough that does not have an identifiable cause,
after a reasonable evaluation including history, physical examination, radiography, and spirometry. A chronic cough is likely to
be nonspecific if it is dry and there are no abnormalities identified on initial evaluation (ie, no "specific cough pointers" (table
1)). This type of cough usually resolves gradually, but children should be reevaluated periodically for the emergence of signs or
symptoms of specific cough. In some cases, nonspecific cough may represent a postviral syndrome.
OVERVIEW OF CAUSES IN CHILDREN

The three most common causes of chronic cough in children are asthma, protracted bacterial bronchitis (PBB), and nonspecific
cough that resolves spontaneously, as described in hospital-based multicenter studies and a systematic review [9-11] and
supported by a multicenter study in which children were enrolled from primary care or emergency departments [3]. Other
causes are outlined in the table (table 1). For some other diagnoses such as tuberculosis, the frequency varies by region [9,12].

Of note, the causes of chronic cough in children are different from that of adults, so evaluation and management of children
should be based on child-specific protocols and not those designed for adults [9,13]. As an example, gastroesophageal reflux
disease (GERD) and upper airway cough syndrome (formerly known as postnasal drip syndrome) are thought to be common
causes of chronic cough in adults but are probably not common causes in children [9,14]. Adult cough guidelines have
suggested empirical treatment of asthma, GERD, and upper airway cough syndrome (UACS) because these are the most
common causes in this population. By contrast, pediatric guidelines do not recommend empiric therapy [2,15,16], although
adolescents 15 years and older may be managed using guidelines for adults [17]. (See "Evaluation of subacute and chronic
cough in adults".)

PULMONARY CAUSES OF CHRONIC COUGH IN CHILDREN

Aspiration — Chronic or recurrent aspiration is an uncommon but important cause of chronic cough. It may be due to a primary
swallowing dysfunction or secondary to disorders such as gastroesophageal reflux or achalasia. Other risk factors for
aspiration include neurologic and developmental abnormalities, neuromuscular disease, and anatomic airway abnormalities
(eg, laryngeal cleft or tracheoesophageal fistula). Some individuals with these disorders have a history of feeding difficulties, or
coughing during feeding. However, the absence of cough during swallowing does not exclude aspiration, because aspiration
can be "silent," especially if it is chronic [18]. (See "Aspiration due to swallowing dysfunction in infants and children" and
"Congenital anomalies of the larynx".)

Asthma — In some children, chronic cough can be the most prominent presenting symptom of asthma. However, isolated
cough (cough in absence of other symptoms) is rarely asthma in children [19].

● Respiratory symptoms – The cough associated with asthma is typically dry. A wet cough does not exclude asthma but
should also raise the possibility of protracted bacterial bronchitis (PBB) or aspirated foreign body (see 'Protracted bacterial
bronchitis' below and 'Inhaled retained airway foreign body' below). Typically in asthma there are associated symptoms of
wheezing, exertional dyspnea, or atopy, although these features may not be recognized or initially reported by the parents.

Asthma in the absence of any of these symptoms, sometimes termed "cough-dominant asthma," or "cough-variant asthma"
may present as an apparently nonspecific cough. Most studies have suggested that this is an uncommon cause of cough
in children [13,15,20,21]. As an example, a randomized trial in children with nonspecific cough showed no benefit from
treatment with beta agonists and steroids [22]. Moreover, observational studies show that nonspecific cough has different
risk factors and triggers than classic asthma and that nonspecific cough generally resolves spontaneously [23].

Although cough-dominant asthma is probably uncommon in children, it is still appropriate to consider the possibility of
asthma in a child presenting with a chronic dry cough and no other apparent symptoms, primarily because the presence or
absence of wheezing is not reliably recognized by the parents or reported on history [24]. Therefore, evaluation for chronic
cough in children should include careful questioning about clinical symptoms of wheezing or atopy, with spirometry and
response to bronchodilators, and if warranted a trial of anti-asthma medications, as discussed below.

● History – Clues to the presence of asthma include a history of eczema, rhinitis, or bronchiolitis [25]. A family history of
atopy or asthma is common. In addition a history of wheeze and paroxysmal cough (that responds to bronchodilators)
may be reported [25], although parental reporting of wheeze is often inaccurate [24]. Episodes of asthma are often
triggered by acute upper respiratory viral illnesses, exercise, cold, or known allergens.

● Evaluation – Initial evaluation of any child with chronic cough includes a chest radiograph and spirometry (if the child is
able). The presence of bilateral hyperinflation on chest radiograph is often, although not always, indicative of asthma. In
addition, changes within the right middle lobe are seen in children with asthma (image 1). Spirometry (generally performed
in children aged >6 years) that shows an obstructive pattern on the flow volume loop, which reverses with bronchodilators,
indicates obstructive airway disease, most commonly asthma.

If the initial evaluation suggests a provisional diagnosis of asthma, the next step is a trial of asthma medications (empiric
trial of bronchodilators [short-acting beta-2 agonists] and low-dose inhaled corticosteroids) for two to four weeks, followed
by reevaluation (algorithm 1). A clear response to this therapy strongly supports the diagnosis of asthma, but does not
fully establish the diagnosis, because nonspecific cough unrelated to asthma frequently resolves spontaneously.
Therefore, asthma medication should not be continued unless the diagnosis of asthma can be made with confidence [1].
Lack of response to asthma medications generally is sufficient to exclude asthma. (See "Asthma in children younger than
12 years: Initial evaluation and diagnosis" and "Asthma in children younger than 12 years: Initiating therapy and monitoring
control".)

In cases where it is particularly difficult to establish or exclude the possibility of asthma, an airway hyperresponsiveness
challenge test in the pulmonary laboratory may be indicated if the child is old enough for testing (>6 years). (See "Asthma
in children younger than 12 years: Initial evaluation and diagnosis", section on 'Bronchoprovocation testing'.)

Chronic endobronchial suppurative disease — Chronic endobronchial suppurative disease represents a spectrum ranging from
PBB, to chronic suppurative lung disease (CSLD), to bronchiectasis.

The relationship among these disorders has not been fully established. A pathobiologic model that has been proposed
suggests PBB, CSLD, and bronchiectasis represent a spectrum of disease, with PBB at one end and irreversible bronchiectasis
at the other, and that some children with PBB will progress to CSLD, then to bronchiectasis (figure 1) [26-28]. All three diseases
have similar pathobiology with airway neutrophilia, neutrophilic inflammation (elevated matrix metallopeptidase 9 [MMP-9],
interleukin 1 beta [IL1-beta], interleukin 8 [IL-8]) endobronchial infection with typical organisms and impaired mucociliary
clearance and efferocytosis (removal of dying cells by macrophages) [29,30].

Support for this model comes from a prospective study of 106 children with PBB, which found that a small percentage of
children with PBB (12 percent) developed bronchiectasis within two years [31]. Risk factors for development of bronchiectasis
were recurrent episodes of PBB and nontypeable Haemophilus influenzae infection. A subsequent study found alveolar
macrophages from children with PBB or bronchiectasis demonstrated reduced ability to phagocytose apoptotic airway
epithelial cells as well as nontypeable H. influenzae, suggesting a possible etiology linking these two conditions and disease
pathogenesis [32]. Further work is necessary to confirm whether PBB does indeed progress to bronchiectasis.

Protracted bacterial bronchitis — PBB is one of the most common causes of chronic wet cough, particularly in young
children (<5 years of age), accounting for approximately 40 percent of referrals to pediatric pulmonary specialist clinics in
resource-rich countries [11]. This disorder is now incorporated into American, Australian, and European cough guidelines
[2,15,16,33,34]. Identification and treatment of PBB is important because, if untreated, it may be a precursor to bronchiectasis
[28,31,35]. (See 'Bronchiectasis' below.)

Diagnosis — PBB is usually diagnosed based on clinical criteria [29]:

● Chronic wet cough (duration at least four weeks)


● No other symptoms or signs of other causes
● No evidence of an alternative diagnosis after a standard evaluation (including normal spirometry and normal radiograph,
other than bilateral peribronchial accentuation) (table 2)
● Resolution of the cough after a two-week course of appropriate antibiotics
PBB is caused by typical respiratory pathogens, such as H. influenzae, Streptococcus pneumoniae, and Moraxella catarrhalis
[13,29]. Bronchoscopy is not required for the diagnosis but, if performed, reveals mucopurulent discharge in the bronchi
(picture 1).

In some children the diagnostic criteria listed above are met but the disease is more severe. In these cases the term "PBB-
extended" is used if the cough only resolves after an extended course (four weeks) of antibiotics; the term "recurrent PBB" is
used when a child has more than three episodes per year [29]. (See 'Recurrent protracted bacterial bronchitis' below.)

Differential diagnosis

● Asthma – The clinical symptoms of PBB can be similar to asthma. Unlike asthma, PBB does not respond to
bronchodilators and the cough is wet. However, asthma and PBB can coexist. If a child has a chronic wet cough and
apparent asthma, but fails to respond to asthma medications, empiric treatment for PBB should be considered [26].

● Foreign body – The symptoms of PBB may also be indistinguishable from foreign body aspiration. Young children are at
risk for unrecognized foreign body aspiration, and should be evaluated if there are suspicious features, such as a history of
sudden onset of cough after choking, or while eating or playing. (See 'Inhaled retained airway foreign body' below.)

● Bronchiectasis – Bronchiectasis may be associated with a previous or current respiratory infection or by other conditions
that predispose to chronic lung infection, including immunodeficiency, anatomic abnormalities, cystic fibrosis, or primary
ciliary dyskinesia (table 4). Evaluation for these disorders is warranted in a child who fails to respond to treatment, has
recurrent episodes of PBB, or has failure to thrive or other pointers of specific cough [29,35,36]. (See 'Recurrent protracted
bacterial bronchitis' below and "Causes of bronchiectasis in children".)

Treatment — Children with a clinical diagnosis of PBB should be treated with a prolonged course of antibiotics, with a
minimum course of two weeks [37-39]. In a meta-analysis of three randomized controlled trials including 190 children with
chronic wet cough, treatment with antibiotics improved cough resolution compared with placebo (odds ratio [OR] for persistent
cough 0.15, 95% CI 0.07-0.31). The studies included in the meta-analysis were heterogeneous in the definition of chronic cough,
the specific antibiotic used, and duration of therapy [38]. In one of the trials included in this meta-analysis, treatment with a two-
week course of amoxicillin-clavulanate was significantly more likely to achieve resolution of cough compared with placebo (48
percent versus 16 percent) [37].

For children who continue to cough after two weeks of antibiotics, our clinical experience suggests that some will respond to a
more prolonged course (up to four weeks). No high-level evidence to support this approach exists, although a retrospective
review of children with PBB in the United Kingdom suggested that there may be an association between longer initial courses
of antibiotics and a decrease in the rate of recurrent PBB [40]. Further, it has been shown in another retrospective review that
for each month of prior wet cough, it took an extra one day to achieve cough resolution while on antibiotic treatment [41].
Better evidence is needed to determine whether a prolonged course of antibiotics is beneficial, in view of the inherent risks of
antibiotic therapy.

For patients with a first clinical presentation and typical features of PBB, the antibiotics are usually selected empirically,
targeted to the most likely causative organisms, or can be based upon the sensitivities of organisms if cultures are available
from sputum or bronchoalveolar lavage (BAL). The most widely used antibiotic is oral amoxicillin-clavulanate, as it is active
against beta-lactamase-producing strains of H. influenzae. Alternatives include most oral second- or third-generation
cephalosporins, trimethoprim-sulfamethoxazole, or a macrolide [34]. Azithromycin is not recommended for this purpose,
because of a lack of studies demonstrating efficacy for chronic wet cough, and concerns about increasing resistance of S.
pneumoniae and H. influenzae to macrolide antibiotics [34].

Bronchoscopy is appropriate prior to antibiotic treatment for patients with atypical features or suspicion of an inhaled foreign
body; and also for patients who fail to respond completely to a four-week course of empiric treatment. Some practitioners elect
to perform bronchoscopy with BAL and bacterial culture to guide antibiotic selection, especially in children with a very long
duration of cough (eg, ≥12 months) [42].
Recurrent protracted bacterial bronchitis — Children with recurrent PBB (>3 episodes in one year) should undergo an
evaluation to evaluate for bronchiectasis or an underlying disorder that predisposes to chronic lung infection (table 4) [31].
Important considerations are a retained foreign body, congenital abnormalities, and disorders that can lead to bronchiectasis
such as cystic fibrosis, primary ciliary dyskinesia, and immune deficiencies such as selective antibody deficiency [43]. These
further investigations usually involve referral to a specialist for bronchoscopy and/or high-resolution computed tomography
(HRCT) chest scan, sweat test, and an immune evaluation [38]. (See 'Differential diagnosis' above and "Causes of
bronchiectasis in children".)

Chronic suppurative lung disease — CSLD refers to a condition with the clinical characteristics of bronchiectasis, such as a
chronic wet cough unresponsive to oral antibiotics, but without the radiologic changes on HRCT chest diagnostic of
bronchiectasis [26,27,30]. In the past, the term was used more broadly, to describe any condition with chronic purulent
secretions on the lungs, such as empyema, bronchiectasis, or lung abscess [27].

CSLD should be suspected in children with recurrent PBB or chronic wet cough that persists despite four weeks of oral
antibiotics. Such children should be evaluated with chest HRCT, to help identify any underlying cause, and determine if
bronchiectasis has developed [42]. Management of CSLD is not well established, but is similar to that for bronchiectasis,
including repeated courses of antibiotics and airway clearance therapies. (See 'Bronchiectasis' below.)

Bronchiectasis — Bronchiectasis is defined by abnormal radiographic signs on chest HRCT. The major sign on chest HRCT
is an increase in the bronchial-arterial ratio, and dilatation of peripheral airways with bronchial wall thickening and lack of
peripheral airway tapering [44].

Bronchiectasis is likely in a child with a chronic wet cough that persists despite four weeks of antibiotics [42]. In older children,
the cough may be productive of mucopurulent sputum. Other symptoms may include recurrent episodes of PBB or other chest
infections, exertional dyspnea, hemoptysis, or digital clubbing [27,44]. Children with any of these features should be evaluated
with chest HRCT, which will determine if bronchiectasis is present and in some cases may help identify an underlying cause.

Patients with CSLD or bronchiectasis should be evaluated for underlying causes such as trachea-bronchomalacia, cystic
fibrosis, immunodeficiency (primary or secondary), ciliary dyskinesia, or recurrent aspiration. Referral to a specialist for further
investigation is suggested as investigations include bronchoscopy, magnetic resonance imaging (MRI) chest (if vascular ring
suggested), sweat chloride test, and genetics to exclude cystic fibrosis, extended immune function, cilial biopsy, and genetics.
(See "Clinical manifestations and evaluation of bronchiectasis in children".)

The management of bronchiectasis involves a multidisciplinary approach, including antibiotics based on lower airway cultures
(oral, or parenteral if oral therapy is inadequate), personalized self-management plans, and airway clearance techniques
including respiratory physiotherapy advice, as well as regular physical activity and nutritional advice [45]. Multicenter
randomized controlled trials in children have demonstrated that oral amoxicillin-clavulanate is superior to placebo for
nonsevere exacerbations, and this remains first choice for empirical therapy in the absence of lower airway cultures [46,47].
Children should receive vaccinations as per national schedule, including pneumococcal vaccination and annual influenza
vaccination where available [48]. (See "Management of bronchiectasis in children without cystic fibrosis".)

Chronic pneumonia — In areas in which tuberculosis (TB) is endemic, it is a common cause of chronic cough in children and
adults. Eight countries account for two-thirds of new cases: India (27 percent), China (9 percent), Indonesia (8 percent), the
Philippines (6 percent), Pakistan (5 percent), Nigeria (4 percent), Bangladesh (4 percent), and South Africa (3 percent) [49].
Hence, it is important to consider TB in any child with chronic cough in these or other countries where the disease is prevalent,
or in those who are at risk of exposure due to travel or infected contacts. On examination, children with TB may have a
monophonic wheeze due to hilar lymphadenopathy or tuberculoma. Investigations include standard tests such as tuberculin
skin test, interferon-gamma release assay, or other available microbiologic investigation such GeneXpert test (detects
deoxyribonucleic acid [DNA] to TB bacteria in sputum sample). Imaging with a chest radiograph and chest computed
tomography (CT) may reveal mediastinal widening, lung collapse, or findings of secondary bronchiectasis. (See "Tuberculosis
disease in children".)
Other chronic infections that have a significant role in certain geographical areas or in immunocompromised individuals
include nontuberculous mycobacteria, fungi (histoplasmosis or Coccidioides), Legionella, and Chlamydia pneumoniae. As an
example, Toxocara canis infection is common in Hungarian children with chronic cough [50]. Investigations such as
bronchoscopy, BAL, and chest CT are warranted if atypical chronic infection is suspected. Further investigations for one of
these infections are guided by the clinical suspicion, based on the geographical area or exposures (eg, serum T. canis
immunoglobulin G [IgG]), and age or immune status of the child. These and any other type of chronic pneumonia may cause a
chronic cough. (See "Pneumonia in children: Epidemiology, pathogenesis, and etiology" and "Nontuberculous mycobacterial
pulmonary infections in children" and "Zoonoses from dogs", section on 'Toxocara canis'.)

In young infants, infections with Chlamydia trachomatis, cytomegalovirus, or other agents may cause a chronic pneumonia,
sometimes known as "afebrile pneumonia of infancy." (See "Pneumonia in children: Epidemiology, pathogenesis, and etiology",
section on 'In neonates' and "Chlamydia trachomatis infections in the newborn", section on 'Pneumonia'.)

Eosinophilic lung disease — The possibility of eosinophilic lung disease should be considered in children with elevated
eosinophils in peripheral blood and chronic cough [51]. Pediatric eosinophilic pneumonia is characterized by infiltration of
alveolar spaces, resulting in local or diffuse pulmonary infiltrates on radiography [51]. The diagnosis relies on the finding of
elevated eosinophils in fluid from BAL. Elevation is often defined as eosinophils >20 percent of total cell count, although the
cut-off is controversial, as a disorder known as "nonasthmatic eosinophilic bronchitis" in adults in defined as airway
eosinophilia of >3 percent [52]. (See "Evaluation of subacute and chronic cough in adults", section on 'Nonasthmatic
eosinophilic bronchitis'.)

Eosinophilic lung diseases can be classified as primary or secondary. Primary conditions are those in which no other cause is
determined. Secondary conditions can be due to infectious causes such as parasites (eg, Ascaris lumbricoides, Strongyloides
stercoralis) or fungus (eg, allergic bronchopulmonary aspergillosis, pulmonary coccidioidomycosis, pulmonary histoplasmosis),
or rarely drugs [53]. Investigations include peripheral bloods for eosinophil count, bronchoscopy with BAL, and serologic
assessment for secondary causes where warranted. (See "Overview of pulmonary eosinophilia".)

Inhaled retained airway foreign body — The possibility of a retained airway foreign body should always be considered in young
children (aged <5 years) with a chronic cough. The cough is typically wet-sounding, but may be dry; and there may be
associated wheezing or halitosis. The first step is a focused history, specifically asking about an episode of choking, even if
weeks before, or sudden onset of the cough in a young child while eating or playing. Even if there is no such history, a foreign
body remains a possibility and should be considered in any young child with a chronic cough. (See "Airway foreign bodies in
children", section on 'Presentation'.)

The evaluation should also include a careful and sometimes prolonged auscultatory assessment. Findings suspicious for
bronchial obstruction include asymmetry in aeration, and focal adventitious sounds, most commonly unilateral low-pitched
monophonic wheeze. Only approximately 10 percent of objects aspirated by children are radio-opaque. Therefore, it is
important to review the radiograph closely for findings suspicious for bronchial obstruction, particularly unilateral lung
hyperinflation (lucency of the lung field distal to the obstruction) (image 2). If possible, chest radiographs should be taken in
both inspiration and expiration when foreign body aspiration is suspected because the unilateral hyperinflation may be more
apparent on the expiratory film (image 3). (See "Airway foreign bodies in children", section on 'Imaging'.)

If the history, examination, or radiograph is suspicious for a foreign body, the child should always be evaluated with
bronchoscopy, which also permits removal of the foreign body. (See "Airway foreign bodies in children", section on
'Bronchoscopy'.)

Uncommonly, an aspirated foreign body may go undiagnosed for months or even years, and can cause focal bronchiectasis.
This possibility should be considered in otherwise healthy individuals with focal bronchiectasis. The foreign body may not be
evident on chest radiograph or even chest CT; bronchoscopic evaluation may be necessary to make the diagnosis. (See
'Bronchiectasis' above and "Causes of bronchiectasis in children", section on 'Acquired bronchial obstruction'.)
Interstitial lung disease — Interstitial lung disease (ILD), also known as "diffuse lung disease," is a group of disorders that
involve the pulmonary parenchyma and interfere with gas exchange. These disorders are classified together because of similar
clinical, radiographic, physiologic, or pathologic manifestations, but the underlying causes are diverse and include genetic,
autoimmune, and acquired/infectious disorders. They tend to present with tachypnea, cough, fine crackles on auscultation, or
hypoxemia; failure to thrive or clubbing of the digits may be present. A plain chest radiograph is usually abnormal, but rarely
specific. Spirometry (in children >6 years) typically shows a restrictive pattern. The presence of ILD is confirmed by HRCT.
Further investigation to determine the type of ILD may include genetic testing or lung biopsy. (See "Classification of diffuse lung
disease (interstitial lung disease) in infants and children" and "Approach to the infant and child with diffuse lung disease
(interstitial lung disease)".)

Noninfective bronchitis — Exposure to environmental pollutants can trigger noninfective bronchitis and chronic cough in
children. Worldwide, indoor air pollution is a major contributor to respiratory health, with the largest burden falling in developing
countries; children are more susceptible than adults due to developing lungs and immune systems [54]. The most important
indoor air pollutants include environmental tobacco smoke, biologic pollutants (eg, dust mite, mold, pets), nitrogen dioxide
from unvented gas heating, and particulate matter from wood stoves and cooking [55]. The importance of environmental
tobacco smoke in respiratory health of children is well established and should always be addressed in children with chronic
cough [55]. (See "Secondhand smoke exposure: Effects in children".)

Similarly, hazardous outdoor air pollutants can be a contributing factor to chronic cough and bronchitis in children. As an
example, a study in Tennessee found an association between toxicity-weighted emissions and the prevalence of chronic
bronchitis in children [56]. Another found that exposure to volcanic acid air pollution in Hawaii was associated with chronic
cough in children [57].

Identification and mitigation of environmental tobacco smoke and other environmental pollutants is an important step in
management of chronic cough. In most cases, these exposures contribute to the development or persistence of chronic cough,
but are unlikely to be the sole cause [9].

Postinfection (self-resolving)

Viral infections — Respiratory viral infections are the most common cause of acute cough, and also likely the most common
cause of "nonspecific" chronic cough. These children have dry cough and do not have any specific cough "pointers" (table 2) [5].
These cases resolve without any therapies, and can be managed with reassurance and observation. (See 'Nonspecific cough'
above and "Approach to chronic cough in children", section on 'Nonspecific cough'.)

Bordetella pertussis infection — In young children, Bordetella pertussis or parapertussis infection can present with a typical
paroxysmal whooping cough. In infants and older children, the classic features may not be seen. A cough post-pertussis
infection may persist for weeks after the infection has cleared. As an example, in a prospective cohort study, 37 percent of
children with chronic cough had serologic evidence of a recent pertussis infection, although most of the subjects had been fully
immunized [58]. Thus, post-pertussis cough should be considered in children with chronic cough regardless of immunization
status. (See "Pertussis infection in adolescents and adults: Clinical manifestations and diagnosis" and "Pertussis infection in
infants and children: Clinical features and diagnosis", section on 'Clinical features'.)

If B. pertussis infection is suspected, evaluation depends on the patient's age and duration of cough:

● For children one year and older with chronic cough, the primary purpose of laboratory testing is to confirm the diagnosis
and void further invasive work-up since antimicrobial therapy usually is not indicated three weeks or more after symptom
onset, and the chronic cough will usually resolve gradually without treatment.

● For infants, different strategies are used for laboratory confirmation, and antimicrobial therapy may be indicated up to six
weeks after symptom onset. Details of laboratory testing and indications for treatment are discussed separately.

(See "Pertussis infection in infants and children: Clinical features and diagnosis", section on 'Laboratory confirmation' and
"Pertussis infection in infants and children: Treatment and prevention", section on 'Indications'.)
Other infections — Infections such as Mycoplasma pneumoniae and C. pneumoniae also may cause chronic cough, with or
without evidence of an antecedent pneumonia. This chronic cough tends to resolve with time, antimicrobials specifically for the
cough are not usually unnecessary in otherwise healthy children. Laboratory confirmation of these organisms is difficult and
patients with pneumonia are often treated empirically; the diagnosis sometimes can be made by serology, polymerase chain
reaction (PCR), or antigen detection. (See "Mycoplasma pneumoniae infection in children" and "Pneumonia caused by
Chlamydia pneumoniae in children".)

Space-occupying lesions — A rare cause of chronic cough in children is a mediastinal mass (eg, due to cancer), which may be
evident on initial chest radiograph. Children with findings suspicious for cancer should be further evaluated with HRCT of the
chest or MRI and referred to an oncology specialist.

EXTRAPULMONARY CAUSES OF CHRONIC COUGH IN CHILDREN

Cardiac — Rarely, chronic cough in children is caused by underlying cardiac disease, particularly pulmonary hypertension or
cardiogenic pulmonary edema. Pulmonary arterial hypertension may be idiopathic, or secondary to congenital heart disease,
connective tissue disease, or HIV infection (see "Pulmonary hypertension in children: Classification, evaluation, and diagnosis").
Cardiogenic pulmonary edema is most often caused by left-sided heart disease. (See "Approach to cyanosis in children",
section on 'Pulmonary edema'.)

The possibility of underlying cardiac disease may be raised by focused history and examination, as well as chest radiograph,
during evaluation of a child with chronic cough. When evaluation raises suspicion for cardiac disease, consultation with a
pediatric cardiologist (where available) and investigation with an echocardiogram and electrocardiogram are warranted.

Ear disease (otogenic cough) — A rare cause of chronic cough in children is the otogenic reflex (also known as the ear-cough
reflex) [15]. In a minority of individuals, the external ear is innervated by a branch of the vagus nerve. In this case, irritants (eg,
cerumen) can cause chronic cough and removal of the irritants may improve the symptom [59,60]. Although this reflex is
common among adults with chronic cough, its prevalence in children with chronic cough is similar to that in healthy children
[61].

Esophageal disorders/gastroesophageal reflux — Whether gastroesophageal reflux disease (GERD) is an important cause of


isolated chronic cough in children is controversial. Most experts suggest that GERD is not a common cause except in children
with neurologic abnormalities predisposing to aspiration [9,14]. A systematic review of the etiologies of chronic cough in
children included 14 prospective studies, and of these, only two reported GERD as a common cause in children [9,62,63].

Data from ambulatory monitoring of esophageal pH also suggest that acid reflux is not a common cause of chronic cough. In
one such study, more than 80 percent of coughs were independent of a reflux event, and when the events coincided, reflux was
no more likely to precede than to follow cough [64]. There is somewhat better evidence to suggest a role for non-acid reflux in
triggering chronic cough. In a few studies that used impedance manometry (a technique that can detect both acid and non-acid
reflux), a temporal association between reflux events and some of the episodes of cough was noted in approximately 50
percent of children with chronic unexplained cough, suggesting a possible association in some individuals [65-68].

An expert-panel systematic review of childhood cough and GERD has endorsed that GERD treatments should not be used in
children <14 years old with chronic cough when no clinical features of GERD are present [69] (see "Clinical manifestations and
diagnosis of gastroesophageal reflux disease in children and adolescents", section on 'Clinical manifestations'). For children
with chronic cough and without underlying lung disease who do have gastrointestinal GERD symptoms, it is suggested that
they be investigated and treated following current GERD guidelines for children [70,71]. Importantly, for these children with
chronic cough and gastrointestinal GERD symptoms, it is recommended that a treatment trial of four to eight weeks of
appropriate therapy be used, with reevaluation for response and cessation of therapy if there is no therapeutic benefit.
Medications — A small number of medications are known to cause a chronic cough. In particular, cough is a well-recognized
side effect of angiotensin-converting enzyme (ACE) inhibitors. Other medications that may occasionally cause cough include
any inhaled medications and PPI, and rarely others as an idiosyncratic side effect. If suspected, the medication should be
withdrawn and the cough usually resolves.

Other medications can cause lung disease that results in chronic cough, such as interstitial lung disease (ILD) with cytotoxic
drugs.

Habit cough (tic cough) and somatic cough disorder (psychogenic cough)

● Habit cough (also known tic cough) describes a cough with repetitive and habitual features similar to a vocal tic, especially
suppressibility, distractibility, and suggestibility [72].

● Somatic cough disorder (previously known as psychogenic cough) is a diagnosis of exclusion after evaluation for other
causes of cough and tic cough are excluded and the child fulfills criteria for somatic disorder as outlined in the Diagnostic
and Statistical Manual of Mental Disorders, 5th edition (DSM-5) [72].

A habit or somatic cough has a distinctive nature; it may consist of short, single, dry coughs (tics) or may be loud and repetitive
(barking/honking in nature). The cough is typically more prominent during office visits and absent during sleep; a history of an
antecedent initial upper respiratory tract illness is common and may be a triggering event [72,73]. The physical examination is
normal apart from the cough. Although typical clinical characteristics are often evident on first consultation, this remains a
diagnosis of exclusion after other possible causes have been evaluated [72]. The typical age range for children with these
disorders is 4 to 18 years, with a median of 10 years [74]. The prevalence varies substantially among different populations and
also depends on awareness of this diagnosis among clinicians. In a study of patients referred for chronic cough in Australia,
the prevalence of habit cough was 4.3 percent overall and 8 percent in a pulmonology clinic in Sydney [11].

The most successful therapy for these types of cough in children has been suggestion therapy, which employs a distractor,
such as sipping warm water, as an alternative behavior to the cough (table 5) [73,74]. In a systematic review, suggestion
therapy was successful in resolving cough in 96 percent of patients [75]. An explanation and reassurance (directed primarily to
the child) also may be effective [76]. Children with somatic cough disorder may require referral to a psychologist and/or
psychiatrist if unresponsive to suggestion therapy.

Cases of Tourette syndrome presenting with chronic cough have been reported; children with other vocal or motor tics should
be further evaluated for this or another tic disorder [77,78]. (See "Hyperkinetic movement disorders in children", section on 'Tic
disorders' and "Tourette syndrome: Pathogenesis, clinical features, and diagnosis".)

Upper airway pathology — Upper airway disorders are probably not a cause of chronic cough in children; the main
considerations are:

● Upper airway cough syndrome – Upper airway cough syndrome (UACS), also known as "postnasal drip," is probably an
uncommon cause of chronic cough in children, although there is some controversy on this question [9,14]. In a systematic
review, only two of the ten included studies reported that UACS was a common cause of cough in children; both of these
studies were from Turkey [9]. By contrast, UACS is thought to be a common cause of chronic cough in adults. (See
"Evaluation of subacute and chronic cough in adults", section on 'Upper airway cough syndrome'.)

● Chronic rhinosinusitis – Similarly, chronic noninfectious rhinitis is probably not a common cause of cough in children,
although it is a recognized cause of UACS in adults. Nonetheless, if a child has chronic cough and symptoms of a chronic
noninfectious rhinitis (eg, because of chronic clear nasal drainage and boggy or edematous nasal mucosa), a trial of
intranasal glucocorticoids is appropriate. (See "An overview of rhinitis".)

● Chronic sinusitis – Chronic sinusitis is not a common cause of chronic cough in children except in association with an
immune defect predisposing to chronic infection [9,15,79]. Typical symptoms of chronic sinusitis are a thick and colored
chronic nasal discharge. It is common practice to treat patients empirically with antibiotics, and in some cases both the
discharge and cough improve with treatment. However, this response does not prove an association between sinusitis and
cough. This is because patients with protracted bacterial bronchitis (PBB) may present with identical symptoms, and
would respond to antibiotics. Moreover, many other cases of chronic cough will improve or resolve over time regardless of
the intervention. (See "Acute bacterial rhinosinusitis in children: Clinical features and diagnosis", section on 'Clinical
features'.)

● Obstructive sleep apnea – No evidence exists to suggest a relationship between obstructive sleep apnea (OSA) and
chronic cough in children [9]. Hence children with suspected OSA should be treated separately for both conditions
according to sleep and cough guidelines, respectively.

MANAGEMENT

In the management of chronic cough, the key principles are:

● Specific cough – Targeted treatment for the suspected or confirmed cause, as determined by a systematic evaluation. The
approach to treatment for several of these disorders is included in the discussion above:

• Asthma (see 'Asthma' above and "Asthma in children younger than 12 years: Initiating therapy and monitoring control")

• Protracted bacterial bronchitis (PBB) (see 'Protracted bacterial bronchitis' above)

• Habit cough (tic cough) (see 'Habit cough (tic cough) and somatic cough disorder (psychogenic cough)' above)

● Nonspecific cough – For patients with nonspecific cough (chronic dry cough with no specific cough pointers (table 1)),
management focuses on watchful waiting. Drug trials are occasionally warranted. Details of management and monitoring
are discussed separately. (See "Approach to chronic cough in children", section on 'Nonspecific cough'.)

● Counseling – In addition, management of any type of chronic cough should include counseling to:

• Identify and mitigate exacerbating factors, such as exposure to environmental tobacco smoke
• Avoid over-the-counter cough suppressants
• Define the burden of the cough on the child and parent(s), and manage their expectations

These steps are discussed in detail in the linked topic. (See "Approach to chronic cough in children", section on 'Other
management considerations'.)

SOCIETY GUIDELINE LINKS

Links to society and government-sponsored guidelines from selected countries and regions around the world are provided
separately. (See "Society guideline links: Chronic cough in children".)

INFORMATION FOR PATIENTS

UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education
pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient
might have about a given condition. These articles are best for patients who want a general overview and who prefer short,
easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These
articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are
comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your
patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the
keyword(s) of interest.)

● Basics topic (see "Patient education: Cough in children (The Basics)")

SUMMARY AND RECOMMENDATIONS

● Chronic cough in children is defined as a cough lasting more than four weeks. Specific cough refers to a chronic cough
that is ultimately attributable to an underlying abnormality or disease, which is usually, but not always, of pulmonary origin
(table 1). Conversely, "nonspecific cough" is defined as a chronic cough that does not have an identifiable cause after a
reasonable evaluation. (See 'Definitions' above.)

● The evaluation of a child with chronic cough should include a detailed history, physical examination, chest radiograph, and
spirometry (when possible) using algorithmic approach (algorithm 1). If the initial evaluation provides clues suggesting a
specific cause of cough, further evaluation is focused on that diagnostic possibility. (See "Approach to chronic cough in
children".)

● Protracted bacterial bronchitis (PBB) is a common cause of chronic cough in young children (<5 years of age) in Australia,
Europe, and similar populations.

• A provisional diagnosis of PBB can be made in a child presenting with chronic wet cough, no other symptoms, and no
evidence of an alternative diagnosis (eg, retained airway foreign body or pneumonia) after a standard clinical
evaluation (table 2). (See 'Protracted bacterial bronchitis' above.)

• For patients with a provisional diagnosis of PBB, we suggest antibiotic therapy (Grade 2B). Small randomized trials
have shown that, compared with placebo, a one- to two-week course of antibiotics improves cough resolution in
children with chronic wet cough. We typically treat with oral amoxicillin-clavulanate for two weeks. Alternatives include
oral second- or third-generation cephalosporins, trimethoprim-sulfamethoxazole, or a macrolide. Azithromycin should
not be used in this setting, due to lack of demonstrated efficacy and concerns of inducing antibiotic-resistant
organisms. (See 'Treatment' above.)

• For children who continue to cough after two weeks of antibiotics, we suggest prolonging the course of antibiotics (up
to four weeks) (Grade 2C). This is based largely on our clinical experience and two retrospective case series; clinical
trial data are lacking. (See 'Treatment' above.)

• If a wet cough fails to improve after a four-week course of antibiotics, or if PBB recurs frequently, the patient should be
further evaluated for other causes, including a retained foreign body, congenital abnormalities, and bronchiectasis and
its causative disorders such as cystic fibrosis, primary ciliary dyskinesia, and immune deficiencies. (See 'Recurrent
protracted bacterial bronchitis' above and 'Bronchiectasis' above.)

● Asthma is a common cause of chronic cough in children. It usually is associated with symptoms of wheezing, although
some children may have cough as their primary or only symptom of asthma. Therefore, evaluation for chronic cough in
children should include careful questioning about clinical symptoms of exertional dyspnea, recurrent wheezing, or atopy,
with spirometry and evaluation of response to bronchodilators. If the initial evaluation suggests a provisional diagnosis of
asthma, the next step is a trial of asthma medications (empiric trial of bronchodilators [short-acting beta-2 agonists]
and/or low-dose inhaled corticosteroids) for two to four weeks, followed by reevaluation (algorithm 1). Asthma medication
should not be continued unless the diagnosis of asthma can be made with confidence. (See 'Asthma' above.)

● Young children with a chronic cough that have a history of sudden onset after a choking episode or began after eating or
playing should always be evaluated to exclude the possibility of an inhaled foreign body. These children should be
evaluated with a chest radiograph as a first step but should also be referred to a specialist for evaluation and urgent
bronchoscopy, regardless of radiographic findings. (See 'Inhaled retained airway foreign body' above and "Airway foreign
bodies in children".)

ACKNOWLEDGMENTS — The editorial staff at UpToDate would like to acknowledge Roni Grad, MD, who contributed to an
earlier version of this topic review.

Use of UpToDate is subject to the Subscription and License Agreement.

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Topic 6356 Version 34.0


GRAPHICS

Causes of chronic cough in children

Major evaluation method (in addition to


Primary cause Risk factors or mechanisms
clinical findings)

Pulmonary causes

Aspiration* (recurrent small volume) Primary swallowing dysfunction or laryngeal Swallowing assessment (eg, videofluoroscopic) and
disorders (eg, laryngeal cleft, trachea-esophageal other evaluation as indicated ¶
fistula), gastroesophageal reflux, achalasia

Asthma, cough-dominant asthma* Genetics, environment, atopy, post-acute respiratory Lung function, airway hyperresponsiveness
infections

Chronic endobronchial suppurative disease* Cystic fibrosis Sweat test, genetic screening
(protracted bacterial bronchitis, chronic suppurative Immunodeficiency (primary or secondary) Evaluation of immune function
lung disease, bronchiectasis)  Primary ciliary dyskinesia Cilia biopsy, genetic testing
Aspiration Refer to "Aspiration" above

Chronic pneumonia* Chronic atelectasis, mucous plugging, plastic Chest CT, bronchoscopy
bronchitis Relevant microbial assessment (eg, QuantiFERON
Pathogens include tuberculosis, nontuberculosis gold and Gene Xpert for tuberculosis)
mycobacteria, mycoplasma, fungi, and chlamydia

Eosinophilic lung disease* Primary or secondary (ie, related to parasitic Bloods and bronchoalveolar lavage
disease)

Inhaled retained foreign body* Young child, history of choking (even if days or Bronchoscopy
weeks before cough onset)

Interstitial lung disease* Primary genetic abnormality, post-severe infection Relevant genetic or autoimmune test, ± lung biopsy
bronchiolitis obliterans, autoimmune disease,
radiation, drugs

Mechanical inefficiency   Tracheobronchomalacia and other airway anomalies Dynamic bronchoscopy


Vascular rings or other anomalies that cause Chest CT with contrast
tracheal narrowing Chest MRI (if vascular cause suspected)   

Noninfective bronchitis* Exposure to environmental pollutants (eg, tobacco History and removal of trigger
smoke, fungi, traffic)

Postinfection (self-resolving) Viral infections, pertussis, parapertussis PCR and/or serology

Space-occupying lesions* Cysts and tumors Chest CT or MRI scan

Extrapulmonary causes

Cardiac*  May cause cough due to airway compression, ECG and other evaluation as indicated
pulmonary edema, or arrhythmia 

Ear disease*  Oto-respiratory reflex (Arnold reflex), in which Examination of the ear canal, and removal of the
stimulation of the auricular branch of the vagus object or treatment of disease that is triggering the
nerve triggers cough cough

Esophageal disorders  Gastroesophageal reflux (acid and non-acid) Esophageal pH monitoring or impedance monitoring,
± endoscopy

Medications* ACE inhibitors (common); any inhaled medication, Discontinuation of medication


proton pump inhibitors, other drugs (uncommon) Evaluation for interstitial lung disease (eg, HRCT)
Certain other medications (eg, cytotoxic drugs) may
be associated with interstitial lung disease

Habit cough (tic cough)* May be isolated, but more likely if other tics are Suppressibility, distractibility, suggestibility,
present variability, and the presence of a premonitory
sensation; cough absent during sleep. 
Response to behavioral therapy (eg, suggestion
therapy)

Somatic cough disorder (psychogenic cough)* More likely in individuals with generalized anxiety or Disproportionate thoughts and anxiety about the
other somatic symptom disorders seriousness of symptoms
Response to reassurance, counseling, or
psychotherapy

Upper airway pathology Chronic sinusitis, obstructive sleep disorders Δ Evaluation guided by suspected disorder (CT,
polysomnography)

CT: computed tomography; MRI: magnetic resonance imaging; PCR: polymerase chain reaction; ECG: echocardiogram; ACE: angiotensin-converting enzyme; HRCT: high-resolution
computerized tomography.
* Children with these disorders typically have specific signs and symptoms that are clues to the underlying disease, sometimes known as specific cough "pointers."
¶ Evaluation for suspected aspiration may include bronchoscopy, esophageal pH monitoring or impedance monitoring, endoscopy, nuclear medicine scans. Refer to UpToDate topic
on evaluation of children with suspected swallowing dysfunction.
Δ Possibly related to aspiration of secretions rather than primary pathology.
Graphic 113206 Version 7.0
Algorithm for the evaluation of chronic cough in children
PBB: protracted bacterial bronchitis; TB: tuberculosis; CF: cystic fibrosis; PCD: primary ciliary dyskinesia.
* Specific cough pointers include: [1]
Symptoms – Chronic wet/productive cough, chest pain, history suggestive of inhaled foreign body, dyspnea, exertional dyspnea, hemoptysis, failure to thrive, feeding difficulties (includ
epidemiologic risk factors for exposure to TB
Signs – Respiratory distress, digital clubbing, chest wall deformity, or auscultatory crackles
Tests – Chest radiographic changes (other than perihilar changes) or lung function abnormalities
Refer to UpToDate content on chronic cough in children.
¶ Habit cough (also known as tic cough) is typically absent at night or when distracted and may be honking or short/dry. [2]
Δ For diagnostic evaluation, refer to UpToDate content on pertussis and tracheomalacia. Tic (habit) cough is diagnosed based on characteristic symptoms.
◊ A child with a dry cough and no specific cough pointers can be safely watched without treatment (the watch and wait approach). The child should be followed and reevaluated in 2 to 4 wee
suppressing medications may be done; refer to UpToDate content for patient selection and precautions.

References:
1. Kantar A, Chang AB, Shields MD, et al. ERS statement on protracted bacterial bronchitis in children. Eur Respir J 2017; 50: 1602139.
2. Weinberger M, Hoegger M. The cough without a cause: Habit cough syndrome. J Allergy Clin Immunol 2016; 137:930.

Graphic 113691 Version 3.0


Signs and symptoms suggesting a specific cause of cough in children (specific cough "pointers")

Specific chronic cough "pointer" Possible major underlying etiology

History
Pulmonary symptoms

Chronic wet or productive cough* Suppurative lung diseases (protracted bacterial bronchitis, chronic suppurative lung
disease, bronchiectasis), aspiration, abscess, cavitations

Hemoptysis Infection (eg, tuberculosis), interstitial lung disease, bronchiectasis, autoimmune lung
disease

Wheeze (at rest or on exertion) Asthma (if no other specific cough pointer present other than spirometry, and
dyspnea that responds to bronchodilators); bronchiectasis, eosinophilic disorders (if
other specific cough pointer[s] present)

Dyspnea (at rest or on exertion) Asthma or any severe lung disease



Classically recognizable cough sounds These cough characteristics (eg, barking, honking, whooping) often suggest a specific
cause of cough ¶

Recurrent pneumonia Immunodeficiency, obstructed airways or any conditions causing bronchiectasis

Timing and triggers

Symptoms from neonatal period Congenital abnormality related to airways, immune function, or causes with
predisposition to bronchiectasis (eg, primary ciliary dyskinesia)

Onset after an episode of choking Inhaled retained foreign body

Cough worsens when child is anxious or attention is focused and is absent Habit cough (tic cough)
during sleep. Cough improves with distraction or suggestion and can be
voluntarily suppressed

Child has disproportionate thoughts and anxiety about the seriousness of Somatic cough disorder (psychogenic cough)
symptoms

Associated symptoms or conditions

Cardiac disease Primary cardiac disease causing cough, tracheomalacia or primary ciliary dyskinesia

Neurologic and developmental abnormalities Aspiration

Feeding difficulties Laryngeal or trachea disorders, aspiration

Failure to thrive Any severe lung disease, cystic fibrosis, immunodeficiency, indolent infections (eg,
tuberculosis)

Exposure to tuberculosis, pertussis, and/or sick animals and travel history Tuberculosis and other mycobacterium, pertussis, parasites (eg, Toxocara), and/or
zoonoses (eg, trematodes, Strongyloides, Q fever)

History of deep infections ± immunodeficiency (primary or secondary to Opportunistic infections (eg, fungal)
cancer treatment or medications)

Autoimmune disease Interstitial lung disease

Angiotensin-converting enzyme inhibitor use Known adverse effect of angiotensin-converting enzyme inhibitor

Chronic fever Indolent infection with or without immunodeficiency

Examination
Digital clubbing Bronchiectasis or interstitial lung disease
Chest wall abnormality Any lung disease, neuromuscular disease
Wheezing or crepitations Any lung disease; in particular, asthma, bronchiolitis obliterans, bronchiectasis (from
any cause), bronchopulmonary dysplasia, heart failure, immunodeficiency and
aspiration
Hypoxia Any lung disease

Routine investigations/tests
Abnormal chest radiography Any lung disease
Abnormal spirometry Obstructive or restrictive lung/chest wall diseases

* In young children, wet cough is substituted for productive cough.


¶ Classically recognized characteristics are a cough that sounds barking/brassy, honking, paroxysmal/whooping or staccato, or that produces casts. Refer to separate table on
classically recognizable cough sounds for details.

Adapted from: Chang AB, Oppenheimer JJ, Weinberger MM, Weir KA, Rubin BK, Irwin RS. Use of management pathways or algorithms in children with chronic cough: CHEST Guideline and
Expert Panel Report. Chest 2017;151:884-890.

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Classically recognizable cough sounds in children

Cough characteristic Suggested underlying pathology


Barking or brassy cough Tracheomalacia, habit cough (habit cough).  If acute: Croup
Honking or 'goose-like' cough Tracheomalacia, habit cough (tic cough), somatic cough disorder (psychogenic cough)
Paroxysmal cough (with or without inspiratory whoop) Pertussis and parapertussis
Staccato cough Chlamydia in infants
Cough productive of casts Plastic bronchitis and conditions associated with mucous plugs such as ABPA (refer to UpToDate topic)
Chronic wet or productive cough only in the mornings Suppurative lung diseases (eg, bronchiectasis, cystic fibrosis)
Wet or productive cough Presence of endobronchial secretions

ABPA: allergic bronchopulmonary aspergillosis.

Adapted from:
1. Chang AB, Glomb WB. Guidelines for evaluating chronic cough in pediatrics: ACCP evidence-based clinical practice guidelines. Chest 2006; 129:260S.
2. Chang AB, Landau LI, Van Asperen PP, et al. Cough in children: Definitions and clinical evaluation. Position statement of the Thoracic Society of Australia and New Zealand. Med J
Australia 2006; 184:398.

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Right middle lobe atelectasis in a child with chronic cough

Chest radiograph showing right middle lobe changes reflecting atelectasis. This child presented with chronic cough and exertional dyspnea. The child was treated with asthma m
and the radiographic changes resolved.

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Spectrum of chronic endobronchial suppurative disease in children

In this pathobiologic model, protracted bacterial bronchitis (PBB), chronic suppurative lung disease (CSLD), and radiographic-confirmed bronchiectasis
likely represent a spectrum of disease, with similar underlying mechanisms of airway neutrophilia, endobronchial bacterial infection, and impaired
mucociliary clearance. If PBB is untreated, it is likely that some (but not all) children will progress to develop CSLD. Some will ultimately develop
bronchiectasis, which is initially reversible but may become irreversible. There is some overlap between these entities.

Modified from: Chang AB, Upham JW, Masters IB, et al. Protracted bacterial bronchitis: The last decade and the road ahead. Pediatr Pulmonol 2016; 51:225.
http://onlinelibrary.wiley.com/wol1/doi/10.1002/ppul.23351/abstract. Copyright © 2016 Wiley Periodicals. Reproduced with permission of John Wiley & Sons Inc. This
image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact
Wiley's permissions department either via email: permissions@wiley.com or use the RightsLink service by clicking on the 'Request Permission' link accompanying this
article on Wiley Online Library (http://onlinelibrary.wiley.com).

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Bronchoscopy in a child with protracted bacterial bronchitis
Bronchoscopy in a child with protracted bacterial bronchitis (PBB) reveals mucopurulent secretions in the bronchi.

Courtesy of Drs. Julie Marchant and Anne Chang.

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Causes and evaluation of bronchiectasis in children

Category Specific examples Diagnostic tools

Congenital bronchiectasis
  Cartilage deficiency (Williams-Campbell Syndrome) Chest imaging
Tracheobronchomegaly (Mounier-Kuhn Syndrome)
Congenital hyperlucent lung (Swyer-James-MacLeod syndrome)

Bronchial narrowing/obstruction
Congenital abnormalities Tracheomalacia, bronchomalacia, tracheal or bronchial stenosis, Chest imaging (CT scan with angiogram) – To evaluate for
bronchogenic cyst, tracheal bronchus, vascular ring, pulmonary sequestration, CPAM, vascular rings, or pulmonary artery
artery aneurysm, or dilation abnormalities
Bronchopulmonary sequestration (intralobar) Flexible bronchoscopy – To evaluate for bronchomalacia, ectopic
CPAM, formerly known as CCAM bronchus, bronchial stenosis, or vascular compression

Foreign body aspiration Peanuts, candy, small objects History (eg, of choking episode, even if remote, and/or recurrent
pneumonia), physical examination, chest imaging, and rigid
bronchoscopy
Mucoid impaction Mucus plug (postoperative, asthma, middle lobe syndrome) Chest imaging, serology for Aspergillus, IgE, flexible bronchoscopy
ABPA with BAL, and biopsy in case of BG

BG
Hilar adenopathy Tuberculosis Chest imaging, flexible bronchoscopy, serology, cultures, and biopsy
Histoplasmosis if needed

Sarcoidosis
Tumors Airway adenoma, endobronchial teratoma Chest imaging, flexible bronchoscopy

Immunodeficiency
Immunoglobulin deficiencies Congenital agammaglobulinemia (Bruton disease) Quantitative immunoglobulin levels, immunoglobulin subclasses,
Selective IgG subclass deficiency (IgG2, IgG4) humoral panel with specific antibody titers to childhood vaccines
and pneumococcal vaccine
Common variable immunodeficiency
IgA deficiency with ataxia-telangiectasia
Selective IgA deficiency (possible association)
Leukocyte dysfunction Chronic granulomatous disease (NADPH oxidase dysfunction) DHR test, nitroblue tetrazolium test, genetic testing
Combined Severe combined immunodeficiency Chest radiograph, immunoglobulin levels, specific antibody titers,
immunodeficiencies DiGeorge syndrome evaluation of lymphocyte subsets (T, B, and NK), and assessment of
T cell function
Bare lymphocyte syndrome (T cell receptor defect caused by MHC
deficiency)
CXCR4 mutation (WHIM syndrome)
CD40 deficiency or CD40 ligand deficiency
(hypergammaglobulinemia M syndrome)
Others
Complement deficiency Eg, C3 deficiency, ficolin-3 deficiency Complement assays (C3, C4, and THC)
Acquired Pharmacologic immunosuppression, HIV infection, malnutrition, History, blood counts, quantitative immunoglobulins
immunodeficiencies malignancy, etc

Abnormal secretion clearance (leading to chronic airway infection)


Abnormal secretions Cystic fibrosis Sweat test, genetic testing
Ciliary dysfunction Primary ciliary dyskinesia (known as Kartagener syndrome if situs Nasal or bronchial ciliary biopsy with motility studies, electron
inversus is present) microscopy, genetic testing
Situs inversus in Kartagener syndrome
Unknown mechanism Young syndrome (obstructive azoospermia and sinopulmonary Sperm count
disease)
Other causes CNS abnormalities, muscle weakness, and weak cough History and examination

Infection
Persistent bacterial Varied organisms History of chronic wet cough, sputum culture
bronchitis
Bacterial pneumonia Staphylococcus aureus, Streptococcus pneumonia, Klebsiella, History and cultures
Pseudomonas aeruginosa
Childhood infection Eg, pertussis, measles History of infection
Viral infections Adenovirus (particularly types 7 and 21), influenza, herpes simplex History, viral studies (titers, PCR, culture)
Other infections Fungal (histoplasmosis) History, serology, cultures
Mycobacterium tuberculosis, atypical mycobacterium
Possibly mycoplasma
Recurrent aspiration Neurologic disorders History, chest imaging
Vocal cord paralysis Swallowing studies and contrast esophagram
Swallowing dysfunction Direct laryngoscopy with probing for laryngeal cleft
Laryngeal clefts
Tracheoesophageal fistula
Gastroesophageal reflux disease
Miscellaneous disorders
Bronchiolitis obliterans Postinfectious History, PFTs, chest CT scan
Post-transplant with chronic rejection (bone marrow, lung
transplant)
Interstitial lung disease
Other causes
Genetic disorders Yellow nail syndrome (MIM %153300) – Yellow nails, lymphedema, Physical examination, family history, possible genetic testing
and respiratory tract disease with pleural effusions
Autoimmune and connective Rheumatoid arthritis History, physical examination
tissue disease Scleroderma Rheumatologic work-up
Relapsing polychondritis Cartilage biopsy for relapsing polychondritis
Tracheobronchial amyloidosis Biopsy for amyloid
Marfan syndrome
Inhalation of toxic fumes and Ammonia Exposure history
dusts Nitrogen dioxide Chest imaging
Other irritant gases
Smoke
Talc
Silicate

CPAM: congenital pulmonary airway malformation; CCAM: congenital cystic adenomatoid malformation (CCAM); CT: computed tomography; ABPA: allergic bronchopulmonary
aspergillosis; BG: bronchocentric granulomatosis; IgE: immunoglobulin E; BAL: bronchoalveolar lavage; IgG: immunoglobulin G; IgA: immunoglobulin A; NADPH: nicotinamide adenine
dinucleotide phosphate; DHR: dihydrorhodamine 123; MHC: major histocompatibility complex; WHIM: warts, hypogammaglobulinemia, infections, and myelokathexis; THC: total
hemolytic complement (CH50); CNS: central nervous system; PCR: polymerase chain reaction; PFTs: pulmonary function tests.

Courtesy of Khoulood Fakhoury, MD, and Adaobi Kanu, MD.

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Airway foreign body

An expiratory chest radiograph showing unilateral lung hyperinflation, suggestive of the presence of foreign body aspiration. This child's bronchoscopy revealed a nut in the right
bronchus.

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Obstructive emphysema in a child following foreign body aspiration

Inspiratory and expiratory chest radiographs in a child following right-sided foreign body aspiration.
Top panel: The inspiratory film appears normal.
Bottom panel: The expiratory film demonstrates hyperlucency of the right lung, indicating obstructive emphysema.

Courtesy of Charles Marquette, MD.

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Major elements of a 15-minute suggestion-therapy session for habit cough*
• Open the session expressing confidence that the coughing will be stopped.

• Explain thecough as a vicious cycle that started with an initial irritant that is now gone, and that the cough itself is causing more
irritation more cough.
• Instruct
the patient to concentrate solely on holding back the urge to cough, for an initially brief timed period (eg, 1 minute).
Progressively increase this time period and utilize an alternative behavior, such as sipping lukewarm water or inhaling a soothing
cool mist from a vaporizer, to “ease the irritation.”
• Tell the patient that each second the cough is delayed makes it easier to suppress further coughing.
• Repeatexpressions of confidence that the patient is developing the ability to resist the urge to cough; “it’s becoming easier to
hold back the cough, isn’t it” (nodding affirmatively generally results in a similar affirmation movement by the patient).
• When the patient shows that he or she is able to suppress the cough (usually for about 10 minutes), ask in a rhetorical manner,
“you’re beginning to feel that you can resist the urge to cough, aren’t you?” (said with an affirmative head nod). 
• Closethe session when the patient can repeatedly respond positively to the question, “do you feel that you can now resist the
urge to cough on your own?” This question is only asked after the patient has gone 5 minutes without coughing.
• Express confidence that if the urge to cough recurs, the patient can do the same thing at home (autosuggestion) ¶ 

* Habit cough is also known as tic cough.


¶ The autosuggestion is for 15-minute sessions at home, in which the patient concentrates on holding back the cough using sips of lukewarm water to “ease the irritation causing
cough”.

Reproduced with permission from the ©ERS 2017. Breathe Mar 2017, 13 (1) 22-30; DOI: 10.1183/20734735.015216.

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Contributor Disclosures

Julie M Marchant, MBBS, FRACP, PhD Nothing to disclose Anne B Chang, MBBS, FRACP, PhD, FAPSR, FAHMS Grant/Research/Clinical
Trial Support: Grants from the Australia National Health Medical Research Council. George B Mallory, MD Grant/Research/Clinical Trial
Support: TOPP-2 [pulmonary hypertension (patient registry)]. Alison G Hoppin, MD Nothing to disclose

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a
multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is
required of all authors and must conform to UpToDate standards of evidence.

Conflict of interest policy

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