PAEDIATRIC RESPIRATORY REVIEWS (2006) 7, 84–88

MINI-SYMPOSIUM: LUNG PHYSIOLOGY

Physiological basis of respiratory signs and

symptoms
Peter D. Sly* and Rachel A. Collins

Division of Clinical Sciences, Telethon Institute for Child Health Research, Centre for Child Health Research,
The University of Western Australia, PO Box 855, West Perth WA 6872, Australia

KEYWORDS Summary An understanding of the physiological processes underlying respiratory

Respiratory physiology; function in the healthy individual is essential in recognising and understanding disease
Cough; processes. Departure from the normal pattern of tidal breathing is an early and reliable
Wheeze; sign of respiratory disease. Understanding the normal distributions of pressures within the
Stridor; thoracic cage, airways and lungs allows one to predict the site of airway obstruction.
Respiratory disease; Respiratory signs and symptoms are based on alterations of normal physiology by the
Infants; disease process. Unfortunately, these basic principals are rarely emphasised in current
Children teaching programs.
ß 2006 Elsevier Ltd. All rights reserved.

INTRODUCTION is referred to as an abdominal breathing pattern; this is,

during inspiration, descent of the diaphragm is accompa-
An understanding of the physiological processes underlying nied by an outward displacement of the abdominal wall.
respiratory function in the healthy individual is essential in This may be accompanied by sternal retractions and inter-
recognising and understanding disease processes. Accurate costal recessions. This pattern of breathing is normal owing
interpretation of the physical signs of respiratory disease to the very compliant chest wall and the fact that the liver is
relies on knowledge not only of the alterations in respira- a very large organ (in relative terms) in the neonate. As the
tory system physiology that occur in disease, but also of the child grows, the chest wall stiffens and the liver is reduced in
processes occurring in order to cause deviation from the (relative) size. Outward displacement of the stiffer chest
normal healthy state. wall gradually becomes the primary mechanism of increas-
The aim of this review is to outline the physiological basis ing intrathoracic volume during inspiration, and abdominal
of respiratory signs in health, and to contrast these with breathing is uncommon in healthy children after the first
respiratory signs and symptoms seen in disease. year of life.

BASIC RESPIRATORY SIGNS IN Maintenance of lung volume

HEALTH
Maintenance of an adequate lung volume at end-expiration
Tidal breathing patterns is important to ensure that the small airways remain open.
By definition, the lung volume at the end of expiration is
The tidal breathing pattern changes with age and with
known as the functional residual capacity (FRC). Far more
development of the chest wall. Newborn infants have what
energy is required to open an airway that has closed than to
* Corresponding author. Tel.: +61 8 9489 7814;
dilate an open airway. This is because a closed airway will
Fax: +61 8 9489 7706. not open until sufficient force is supplied to overcome
E-mail address: [email protected] (P.D. Sly). surface tension forces, which are much larger when the
1526-0542/$ – see front matter ß 2006 Elsevier Ltd. All rights reserved.
doi:10.1016/j.prrv.2006.03.007
RESPIRATORY SIGNS AND SYMPTOMS 85

airway walls are apposed. Continually having to open  the balance between the time allowed for expiration and
closed airways at the onset of each inspiration would be the time-constant of emptying of the respiratory system;
very ‘energy inefficient’ and would be associated with a  ‘braking’ or slowing of the expiratory flow using either
much larger work of breathing. The lung volume at which the glottis or ‘post-inspiratory’ activation of the inspira-
the airways begin to close spontaneously during expiration tory muscles.
is known as the closing volume.
Several physiological mechanisms are involved in main- In healthy adults and older children, the primary mechan-
taining FRC above the closing volume in healthy children. ism determining FRC is the balance between the static elastic
The primary mechanism(s) vary with age and physiological recoil pressures of the lung and chest wall (Fig. 1). The
development. A full description of the physiology involved volume at which these forces are equal and opposite is
is beyond the scope of this article, and the interested reader known as the elastic equilibrium volume (EEV). In children
is referred to textbooks of respiratory physiology. Briefly, with healthy lungs, the EEV is well above the closing volume.
the mechanisms include: During relaxed tidal breathing, inspiration is an active process
and expiration is largely passive, driven by the elastic recoil
 the balance of static elastic recoil forces of the lungs and pressure of the respiratory system (lungs and chest wall). If
chest wall; no active expiration occurs, FRC coincides with EEV. In the
presence of active expiration, for example during exercise,
FRC may be pushed below EEV.

Figure 2 Schematic representation of the balance of static

elastic recoil forces in the respiratory system. The static volume–
pressure curves of the chest wall (CW), lungs (L) and respiratory
system (RS) are shown for an adult (panel A) and newborn (panel
Figure 1 Schematic representation of the pressures within the B) respiratory system. The elastic equilibrium volume (EEV) is the
airways, alveoli and pleural space during normal inspiration (A) and volume at which the outward recoil of the lungs is equal and
expiration (B). (Adapted from Sly PD. Stridor and croup. In: opposite to the inward recoil of the chest wall. The closing volume
Robinson MJ, Robertson DM (eds) Practical Paediatrics, 5th (CV) is the volume at which the airways begin to close sponta-
edn. Churchill Livingstone, 2003; 449–453. neously during a relaxed expiration. TLC, total lung capacity.
86 P. D. SLY AND R. A. COLLINS

In newborn infants, especially those born prematurely, Wheeze is primarily heard during expiration. This is
the elastic recoil of the chest wall is very small and because of the normal decrease in calibre of the intrathor-
inadequate to balance that of the lungs. In these circum- acic airways that occurs during expiration (Fig. 2). If,
stances, EEV occurs at a lower absolute lung volume and however, the airway obstruction is severe enough, the
may even be below the closing volume (Fig. 2). Infants normal increase in calibre of the intrathoracic airways that
maintain a FRC above closing volume and EEV by reducing occurs during inspiration may be insufficient to overcome
the time allowed for expiration, by breathing more rapidly the flow limitation, and wheeze during inspiration may be
and/or by slowing expiratory flow with glottic breaking or heard. The volume of the wheeze is not an indication of
post-inspiratory inspiratory muscle activity. the degree of airway obstruction. In fact, severe obstruc-
tion may limit flow to such a degree that insufficient flow is
RESPIRATORY SIGNS AND passing through the limited segment, meaning that insuffi-
SYMPTOMS IN DISEASE cient energy is dissipated to induce airway wall fluttering.
Thus, although wheeze indicates flow limitation, the
Cough absence of wheeze does not mean the absence of flow
Cough is a common feature of many respiratory illnesses limitation.
and is important in pulmonary host defence. Chronic
cough is very common in young children, possibly owing Stridor
to increased cough receptor sensitivity. Cough receptors As is the case with wheeze, stridor is a respiratory sound
exist at various locations within the respiratory tract, reflecting flow limitation within the airway tree and occurs
including irritant receptors in the larynx and large airways more commonly in children owing to their relatively small
and stretch receptors in the lung parenchyma that stimu- airways. Stridor is a harsh, high-pitched respiratory noise
late cough in response to mechanical irritation, respiratory that occurs primarily during inspiration and reflects
infections, the aspiration of foreign particles and over- obstruction of the extrathoracic airways. Stridor can also
distention of the lungs. Cough may also be initiated occur with an expiratory component, indicating obstruc-
voluntarily. tion of the intrathoracic airways or severe extrathoracic
Whether voluntary or due to receptor stimulation, obstruction.
cough requires a complex sequence of events and involves
both afferent pathways in the vagus and efferent pathways Crackles
in the somatic nervous system. The initial event in cough
involves opening of the glottis and a short inspiration of a Crackles are discontinuous, non-musical adventitious
variable volume of air in order to increase lung volume. respiratory sounds attributable to sudden opening of the
Next, the glottis closes, the chest wall, abdominal and airways. Crackles are generally associated with pulmonary
perineal muscles contract, and high intrathoracic and disorders and heart failure but can also be heard in normal
abdominal pressures are generated. Shortly after closure, individuals during inspiration from residual volume (but not
the glottis re-opens, causing rapid decompression of the FRC) and during the first few breaths on awakening. Both
airways and a high-velocity expulsion of gas; this in turn the number and character (timing, duration, pitch) of
leads to a proximal movement of the airway contents. In crackles occurring depend on the size of the airways from
addition to movement of material in the larger airways by which they originate and the pathophysiology occurring in
high-velocity airflow, compression and collapse of the the surrounding tissue.
intrathoracic airways squeezes secretions into more central
airways, where gas velocity is sufficient to clear the material. Tachypnoea
Mucus can also be dislodged from the airway wall by
shearing forces and vibration of the airway walls with Elevations in respiratory rate during sleep or at rest are a
high-velocity gas flow. sensitive and early indicator of disturbances in pulmonary
function, although they do not suggest the aetiology.
Respiratory rate must be expressed relative to age,
Wheeze decreasing from 40–60 breaths per minute in newborns
Wheeze is an indicator of expiratory flow limitation in the to 30–50 in infants aged between 1 week and 3 months,
intrathoracic airways. Smaller, more compliant airways in 20–40 between the ages of 3 and 24 months, and 14–24
children and infants make them more susceptible to from 2 to 10 years, and to the adult rate of 12–20 from
wheeze than adults, as less airway narrowing is required approximately 10 years of age.
in order to achieve expiratory flow limitation and wheeze An increased respiratory rate is frequently one of the
during tidal breathing. Wheeze represents ‘fluttering’ of the earliest signs of respiratory disease in children. Several
airway walls at the site of airflow limitation, occurring as an pathological processes can underlie the need for an
energy conservation mechanism when the driving pressure increased respiratory rate; including: increased PACO2
exceeds the pressure required to produce maximal flow. necessitating and increased alveolar ventilation; and
RESPIRATORY SIGNS AND SYMPTOMS 87

increased lung stiffness or small airway narrowing necessi- hyperinflation is the ‘barrel’ chest deformity, which
tating an increased FRC or an increased closing volume, describes an increase in both the anteroposterior and
again necessitating an increased FRC. Thus, tachypnoea is lateral thoracic dimensions. Hyperinflation is more easily
a non-specific sign of the onset of lung disease. appreciated on a chest X-ray, on which flattening of the
diaphragms, a more horizontal orientation of the ribs and
Grunting an increased air shadow in front of the heart (on lateral
X-ray) can be seen.
Grunting is an extreme form of glottic braking that occurs
in neonates and infants owing to partial closure of the
Sternal retractions
vocal cords during expiration in order to maintain high
intra-alveolar pressure and limit alveolar collapse in the Sternal retractions are visible indrawings of the skin of the
absence of sufficient surfactant. Grunting is classically chest or neck and can be a sign of increased work of
heard in hyaline membrane disease, although it can be breathing. Sternal retractions can occur during tidal breath-
present in other diseases with alveolar pathology, such as ing in normal infants during the first weeks of life owing to
extensive pneumonia. the higher compliance of the chest wall than the lungs,
and become less common with age as the chest wall
Hyperinflation stiffens. Diseases causing upper airway obstruction (e.g.
croup) or increased lung stiffness (e.g. lower respiratory
Hyperinflation refers to an increase in lung volume above
tract infection) are common causes.
that usually seen at rest. As previously discussed, the end-
expiratory lung volume coincides with the EEV of the
respiratory system in adults and older children with
Intercostal retractions
normal lungs. Hyperinflation occurs naturally in two pri- Also a sign of increased work of breathing, intercostal
mary settings: (1) in the presence of a significant increase retractions are more common in older children with stiffer
in resistance, and (2) in the presence of a significant chest walls. Indrawing of the compliant intercostal regions
decrease in elastic recoil. Both of these conditions result of the chest occurs when increasingly negative pressures
in an increase in the time-constant of emptying of the are generated during inspiration, and are often seen in
respiratory system. If the respiratory rate required to conjunction with hyperinflation.
satisfy ventilatory demands does not allow sufficient
expiratory time, hyperinflation occurs. Hyperinflation Use of accessory muscles of respiration
may also develop during mechanical ventilation. On the-
oretical grounds, an expiratory time equal to three times The use of accessory muscles during respiration represents
the expiratory time constant allows emptying of 95% of increased work of breathing. This is a non-specific sign of
the end-inspiratory volume, whereas an expiratory time disease.
equal to five times the expiratory time constant allows
emptying of 99% of the volume. The expiratory time Flaring of the alae nasae
constant (t) is the product of resistance (R) and com- A manifestation of increased work of breathing, flaring of
pliance (C), i.e. t = R  C. In practice, if the time allowed the alae nasae represents attempts to reduce nasal resis-
for expiration is less than three times the expiratory time tance to airflow.
constant, hyperinflation develops.
Hyperinflation can be useful. The increase in lung
Harrison’s sulci
volume is associated with an increase in airway calibre
secondary to mechanical interdependence. A patient with Harrison’s sulcus is a horizontal depression along the
severe airflow obstruction may have expiratory flow limita- lower border of the thorax at the site of diaphragmatic
tion during tidal breathing at rest. The only way in which the attachment occurring in conjunction with flaring of the
expiratory flow can be increased at times of increased lower rib cage. The importance of the diaphragm as a
ventilatory demand, such as during exercise or febrile respiratory muscle in early childhood, together with the
illnesses, is to increase lung volume, thus moving tidal incompletely calcified chest wall, makes children more
breathing to a more advantageous part of the expiratory susceptible to this deformity. Chronic hyperinflation with
flow–volume curve. However, increasing lung volume also diaphragm-flattening causes the muscular pull of the
increases lung stiffness (i.e. decreases compliance) and diaphragm on the lower ribs to act horizontally rather
increases the work of breathing. Breathing at increased than vertically, pushing the lower ribs outward and form-
lung volumes may also put the respiratory muscles, includ- ing a groove in the ribs where the resultant bending
ing the diaphragm, at a mechanical disadvantage and occurs. Harrison’s sulci are typically seen in diseases
increase the risk of respiratory muscle fatigue. associated with chronically increased work of breathing
Hyperinflation may be difficult to detect clinically, espe- such as cystic fibrosis, pulmonary fibrosis and poorly
cially in infants. The classical physical sign associated with controlled asthma.
88 P. D. SLY AND R. A. COLLINS

Pectus carinatum
Pectus carinatum is an anterior displacement of the upper PRACTICE POINTS
sternum and results in a typical ‘pigeon chest’ appearance.  Understanding the distribution of pressures
This deformity is most common before the chest wall fully within the airways, lung and thorax and how this
stiffens and is associated with long-standing hyperinflation changes during normal breathing allows the loca-
and Harrison’s sulci. lisation of airway obstruction with a fair degree of
accuracy.
Pectus excavatum  Stridor with an expiratory component indicates
either more severe extra-thoracic obstruction or
Pectus excavatum is a posterior displacement of the sternum,
an intra-thoracic component and warrants inves-
resulting in the ‘sunken chest’ deformity. This deformity is
tigation.
most commonly the result of a congenital chest wall defor-
 The presence of wheeze implies expiratory flow
mity that becomes increasingly obvious as the child grows.
limitation.
Milder cases may not appear until adolescence. Although
 A loud wheeze does not imply severe obstruction.
long-standing upper airway obstruction may produce a
Very severe obstruction may result in a ‘silent’
pectus excavatum, this form is much less common than
chest as insufficient volume of air is moving
the congenital one. The cosmetic deformity may be quite
through the airways to generate wheeze.
severe but has no functional consequences. The rib cage is
 The stage of physiological development needs to
able to perform its primary function, namely expanding the
be taken into account when interpreting physical
chest wall, lowering pleural pressure and allowing inspiration
signs; for example, sternal retractions are normal in
to occur, with normal efficiency. Exercise capacity is not
neonates, especially those born prematurely, but
limited by pectus excavatum, but significant psychological
not in older children.
problems may be associated with the deformity, including an
avoidance of sport owing to the child’s embarrassment
associated with changing clothes in front of his or her peers.