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PEDIATRIC RESPIRATORY DISORDERS
see also my usmle 2 med 6 for acute epiglotitis
Aspiration
Definition: the inhalation of fluid (gastric contents, amniotic fluids or liquids) or solid objects into the tracheobronchial system. Mostly right mainstem because of the straight pathway.
Etiology
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Impaired gag reflex or swallowing, tubes in the epiglottis, communication between esophagus and trachea tracheoesophageal fistula
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High risk: young children who can=t adequately chew and unconscious patients
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Pathology
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Normally the epiglottis protects the trachea from foreign objects. Depending on the content aspirated, there may be only trauma, irritation or inflammation.
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Chemicals can erode and destroy the trachea or lung tissue.
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Manifestations
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Wheezing, coughing, retractions, stridor
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Hypoxemia/Hypoxia
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Respiratory distress; Respiratory arrest
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Management
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Infants: Prevention is best management
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Suction baby=s airway when the head is delivered
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Control use of baby powder
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Avoid overfeeding
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Cut toddlers food in small pieces
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Avoid toys with small pieces
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Remove object by suction bronchoscopy, or obstructed airway maneuver (Heimlich)
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Sudden Infant Death Syndrome (SIDS)
This is the greatest cause of death between 1 week and 1 year.
7000 thousand infants die yearly. However this has decreased since parents are now encouraged to lay their infant on his/her back.
Etiology: Exact cause is unknown. Sudden death theories.
1. Brain stem abnormality preventing effective cardiorespiratory control.
2. Abnormal T lymphocyte mediated inflammatory response in the lungs which causes edema, obstruction and hypoxia.
Risk factors:
 Mothers under 20 years with poor economic status
 Low birth weight babies
 Prematurity
 Multiple birth babies
 Siblings of children who have died from SIDS
 Prenatal influences
 Smoking
 Anemia
 Lack of prenatal care
Treatment:
No treatment since this is sudden death.
Home monitoring of high risk infants for respiratory and cardiovascular functioning has been successful for prevention of the near misses.
Respiratory Distress Syndrome (Hyaline Membrane Disease)
Incidence:
Usually a complication of prematurity.
Etiology:
1. Decreased surfactant production
2. Immature lung tissue
 At 24 weeks gestation there are very small amounts of surfactant and few developed terminal air sacs, plus under-developed vascularity causes little chance for survival.
 At 26-28 weeks, there is usually sufficient surfactant and lung development to permit survival.
Pathophysiology:
 1. Alveoli unable to expand due to decreased surfactant (this reduces surface tension and allows the alveoli to expand).
 2. Unable to exchange oxygen and carbon dioxide ( atelectasis) which leads to respiratory acidosis.
 3. Decreased pulmonary blood flow (may be due to ductus arrteriosus remaining open and decreased vascular development).
 4. Alveoli are small and have a difficult time expanding.
 5. Can develop metabolic acidosis related to increased lactic acid and anaerobic metabolism.
Manifestations:
 Increased respirations, intercostal retractions, labored breathing -- expiratory grunting, nasal flaring
 Decreased pH, increased pCO2, deceased pO2 (resp. acidosis)
 Chest X-ray shows atelectasis
 Central cyanosis
Management:
 Dexamethasone to mothers in premature labor and having an imminent delivery (helps to mature lungs)
 Artificial surfactant to infant (via endotracheal tube) after delivery
 Positive pressure ventilation
 Correct the acid/base balance
 Provide calories
Bronchopulmonary Dysplasia
Incidence:
 Most common in newborn infants who remain O2 dependent for more than 28 days; severity and mortality rate of BPD decreased since advent of artificial surfactant for RDS.
 Associated with chronic lung disease in infants and children.
Etiology:
 Premature birth, RDS, prolonged mechanical ventilation, O2 toxicity.
Pathology:
 Mechanical ventilation with oxygen acts as an irritant to lung tissue.
 Endotracheal tube causes irritation.
 Scarring and stiffening of the lung tissue.
 Alveoli fail to multiply.
 Chronic inflammation results in increased mucus and plugging of the alveoli and shunt unit.
Manifestations:
 Tachycardia
 Retractions
 Barrel chest
 Shallow breathing
 Hypoxemia
 Compensated respiratory acidosis (decreased pH, increased CO2)
Management:
 Mechanical ventilation, oxygenation (with gradual weaning of both). Infant may go home on the ventilator.
 Adequate nutrition and calories.
Prognosis:
 Rapid lung development occurs during the first year of life and lung function usually improves.
 These children usually have some degree of respiratory dysfunction as they age as a result of BPD (increased respiratory infections, asthma).
UPPER RESPIRATORY INFECTIONS
Croup
Syndrome of clinical manifestations in the upper airway resulting in narrowing of the airway.
Incidence:
 Viral origin: 85% of cases (3 months to 5 years usually).
 Bacterial origin: rare (usually in children 3-7 years).
Pathophysiology:
 Inflammation of trachea, epiglottis, larynx, or pharynx causes narrowing of airway.
 Stridor caused by turbulent airflow over inflamed vocal cords on inspiration. (Wheezing is from obstructed lower airways on expiration)
 Spasms.
 Increased mucous production.
Manifestations:
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Acute Laryngotracheobronchitis
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Epiglotitis
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Spasmodic Croup
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Bronchiolitis - RSV
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Agent
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Virus
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Hemophilus Influenza Bacteria
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Virus
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Respiratory Syncytial Virus
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Age
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6 months to 3 years
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2-7 years
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3 months to 5 years
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Less than 2 years
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S/S
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Croupy cough,
stridor,
low grade fever,
pale retractions,
worse at night
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Respiratory distress,
stridor, agitation,
retractions,
drooling,
muffled voice,
a frog-sitting position
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Stridor,
wet barky cough,
usually at night,
relieved by cold moist air
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Breathlessness,
rapid shallow breaths, cough,
wheezing retractions
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Onset
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Gradual
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Sudden (life threat)
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Sudden at night
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Gradual
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Managed
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Hydration, humidity, oxygen
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Hospitalized, intubation or tracheotomy, antibiotics
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Mist tent, vaporizer, oxygen
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Supportive, oxygen, hydration
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CYSTIC FIBROSIS
An autosomal recessive (chromosome 7) exocrine gland disorder resulting in excessive, thick mucous production which results in obstruction of the respiratory and pancreatic ducts.
Incidence:
Affects about 1 out of 2,500 white children -- most common chronic lung disease in children; 2-5% of whites are carriers. Rare in African Blacks and Asians.
Pathology:
 Production and stasis of thick tenacious mucous.
 Pancreatic exocrine deficiency of trypsin, amylase and lipase.
 Chronic respiratory disease.
 Elevated sweat sodium chloride due to ineffective chloride out of the cell and high reabsorption of sodium into the cells.
Respiratory system:
Accumulations of thick mucous in bronchi results in obstruction, inflammatory response, air trapping and atelectasis.
Respiratory manifestations:
 Recurrent infections, chronic rhinitis
 Rales, rhonchi
 Clubbing of fingers
 Hypoxia
 Cyanosis
Secondary respiratory manifestations:
 Barrel chest
 Kyphosis
Management for respiratory problems:
 Antibiotics
 Steroids
 Bronchodilators
 Mucolytic agents
 Chest percussion
 Lung transplants
GI Manifestations:
 Pancreatic deficiency in 80-90% of the cases
 Newborn infants with a meconium ileus are at high risk
 Malabsorption problems
 Failure to thrive
 Gall bladder problems
 Steatorrhea
Management for GI problems:
 Pancreatic enzyme replacement
Diagnosis:
 Sweat chloride elevated sweat test
 Infants with a meconium ileus
 Blood testing in newborns for elevated immunoreactive trypsin
RESPIRATORY DISTRESS
Predisposing Factors:
 Preterm delivery (especially if born by Caesarean section or if mother is diabetic)
 Underweight for gestational age or wasting (especially if covered in meconium)
 Fetal distress or asphyxia at birth
 Complicated labour e.g. prolonged rupture of membranes or vacuum extraction
 clinical chorioamnionitis in the mother
 Cardinal Signs:
 tachypnoea (> 60 per minute)
 recession
 central cyanosis (tongue) in room air
 expiratory grunting
 Respiratory distress is diagnosed if 2 or more cardinal signs are present.
Causes:
 Respiratory
 hyaline membrane disease
 wet lung syndrome
 meconium aspiration
 pneumonia
 pneumothorax
 diaphragmatic hernia
 Metabolic
 hypothermia
 acidosis
 Rarer causes
 patent ductus arteriosus
 congenital heart disease
 lung hypoplasia
A chest x-ray and examination of the gastric aspirate for lung maturity (shake test), pus cells and bacteria will aid in the diagnosis.
Shake Test
 Place 0.5 ml gastric aspirate in a clean, dry test tube. Add 0.5 ml normal saline and replace cap.
 Shake well for 15 seconds.
 Add 1 ml 95% alcohol to the 1 ml mixture of gastric aspirate and saline.
 Again replace cap, shake well for 15 seconds and read at 15 minutes.
 If no bubbles are present the test is negative.
 If an incomplete ring of bubbles is seen the test is intermediate.
 A complete ring of bubbles with or without extension of bubbles over the surface indicates a positive test.
 Infants with a positive test are very unlikely to develop hyaline membrane disease.
HYALINE MEMBRANE DISEASE (HMD):
 This is the most important cause of neonatal respiratory distress (RD).
 It is virtually confined to preterm infants in whom it is the major cause of death. It is due to a deficiency of surfactant in the infant's lungs.
 Sufactant lowers the surface tension in the alveoli in mature infants and allows respiration with minimal physical effort.
 Lack of adequate amounts of surfactant leads to progressive alveolar collapse.
 Synthesis of surfactant is inhibited by hypoxia, acidosis and cold.
 Thus the more asphyxiated or shocked the infant, the more severe the hyaline membrane disease is likely to be.
 With preterm infants every effort must be made to avoid these factors.
Clinical Signs:
 The features of respiratory distress (tachypnoea, recession, grunting, cyanosis) are usually apparent within hours of birth.
 On auscultation the air entry is reduced and there may be crepitations.
 The baby is inactive and tends to lie in the frog position. Moderate generalized oedema is usually present.
 The chest X-ray typically shows an under-expanded chest with a fine reticulo-granular appearance over both lung fields, "air-bronchograms" extend beyond the borders of the heart and thymus and the outline to the cardiothymic shadow is indistinct.
Management:
 Preterm delivery should be prevented if possible. Antenatal steroids should be given to all women at high risk of preterm delivery to accelerate fetal lung maturity.
 Whenever possible, delivery should be delayed for 48 hours while two doses of betamethasone 12 mg are given intramuscularly 24 hours apart.
 The use of antenatal steroids does not always prevent hyaline membrane disease but decreases the severity.
 The basic aim of treatment in the newborn infant is to maintain oxygenation of the blood at a safe level, keep body temperature and biochemical status within normal limits and provide adequate nutrition.
 The sick infant should be handled as little as possible while regular and frequent observations (skin temperature, colour, heart rate, and respiration) are recorded.
 Most infants starts to replenish lung surfactant and recover after 72 hours
 . Usually the respiratory distress has resolved by a week. Infants with hyaline membrane disease should be transferred as soon as possible to an intensive care unit.
Relief of hypoxia
 is the most important aspect of treatment.
 Adequate oxygenation may be achieved by increasing the concentration of oxygen in the inspired air using a perspex head-box.
 Too much oxygen is as dangerous as too little and can damage the eyes (retinopathy of prematurity) and lungs.
 Thus when administering oxygen one must have adequate facilities for measuring the inspired oxygen concentration (FiO 2) and the arterial oxygen tension (PaO 2) or transcutaneous oxygen saturation (Sa O 2).
 Blood samples are obtained by radial artery puncture or from an indwelling umbilical artery catheter.
 The aim is to maintain PaO 2 between 7 and 10 k Pa (50-80 mmHg).
 The Sa O 2 must be kept between 85 and 90%.
 Severe cases of HMD may require other means of achieving sufficient oxygenation such as continuous positive airway pressure (CPAP) via nasal prongs or intermittent positive pressure ventilation (IPPV) via an endotracheal tube.
Correction of acidosis:
 Respiratory acidosis due to a high PaCO2 can be corrected by improving ventilation.
 Metabolic acidosis will improve with adequate oxygenation but may need correction with 4% sodium bicarbonate given intravenously very slowly according to the formula:
 mmol bicarbonate = base deficit x wt in kg x 0.6
 It is wise to give only half the calculated amount initially and then to reassess the situation.
 Do not use 8% sodium bicarbonate. It has a very high osmolality.
Temperature control:
 Prevent hypothermia by nursing baby under a radiant heat source or in an incubator with the temperature controlled (between 31°C and 34°C) to maintain the neutral thermal environment.
Nutrition:
 Fluid, electrolyte and energy requirements should be supplied by intravenous infusion at first. Milk feeds by nasogastric tube should be started as soon as possible provided the infant does not vomit.
Additional treatment may include:
 blood transfusion for anaemia
 phototherapy for jaundice
 antibiotics for infection
Surfactant replacement therapy:
 Both natural and artificial surfactant can placed down an endotracheal tube to either prevent or treat hyaline membrane disease. As the price is extremely high it is usually only given to infants requiring intubation and ventilation. Hopefully early or prophylactic treatment will become affordable in future. Surfactant therapy is most effective if antenatal steroids have been given.
Complications:
 The most important are:
 Early
pneumothorax
periventricular haemorrhage
heart failure due to a persistent patent ductus arteriosus
pneumonia
 Late
chronic lung disease (bronchopulmonary dysplasia)
WET LUNG SYNDROME [see usmle med 6 on usmle 2 web page for details and graphic]
 This is the commonest cause if respiratory distress. It is a condition which may affect both preterm and term infants, particularly if delivered by caesarian section.
 It is attributed to delayed clearing of the fetal lung fluid into the vessels and lymphatics after birth.
 The condition is not due to surfactant deficiency and the shake test is usually positive.
 Within an hour or two of birth there are features of respiratory distress and the chest is hyperinflated. X-ray chest shows increased vascular markings with prominent hilar streaking.
 Additional oxygen is required but usually does not exceed 40%.
 Most infants improve within twelve to twenty four hours though tachypnoea may persist for several days.
 It is important to differentiate wet lung syndrome from hyaline membrane disease as the former do not require refereral to an intensive care unit.
MECONIUM ASPIRATION
 This is due to the inhalation of meconium during or immediately after delivery. It usually follows on fetal distress during labour. It is limited to mature or wasted infants since preterm infants rarely pass meconium in utero.
 The inhaled meconium produces areas of emphysema and atelectasis throughout the lungs.
 There is considerable risk of pneumothorax and pneumomediastinum.
 A pneumonitis may be caused by chemical irritation or secondary bacterial infection.
 Many infants with severe meconium aspiraton die or suffer severe lung damage.
 At birth the meconium is present in the mouth and pharynx and may stain the skin, nails, cord and placenta.
 Birth asphyxia is common and signs of severe respiratory distress are often noted after resuscitation.
 Air trapping causes over-distension of the chest and there are often wide-spread rhonchi and crepitations.
 On x-ray the lungs look over-expanded and contain multiple areas of atelectasis.
 The heart size may be increased and pneumothorax or pneumomediastinum may be seen.
Treatment:
 Prevention: eliminate fetal hypoxia at all times
 Suck meconium from mouth, pharynx as soon as the head is delivered and before breathing starts. Use a large, end hole catheter.
 Manage in the same way as severe respiratory distress
 Gastric lavage using 2% sodium bicarbonate solution
 Antibiotic (Penicillin and Gentamicin) if secondary bacterial infection develops. Steroids are not indicated.
 Every effort must be made to prevent meconium aspiration by a strict policy of suctioning the upper airways before delivery of the head whenever the liquor is meconium stained. If the infant fails to breathe well after birth, direct suctioning down an endotracheal tube must be done before starting ventilation.
PNEUMONIA
 Infection acquired before or during passage through the birth canal may cause pneumonia which is often difficult to distinguish clinically from other causes of respiratory distress . E.coli and the group B haemolytic Streptococcus are responsible for the majority of infections.
 The latter may cause a picture indistinguishable from that of hyaline membrane disease. Staph aureus, Klebsiella, Pseudomonas, etc may also give rise to serious infection.
 Pneumonia on day 1 results from chorioamnionitis caused by an ascending spread of bacterial from the cervix with or without prolonged rupture of the membranes.
 The mother usually has no clinical signs of infection.
 The newborn with pneumonia may present with birth asphyxia, apnoeic spells or features of respiratory distress.
 Fine crackles may be heard.
 The diagnosis is confirmed by chest x-ray.
 The causative organism may be cultured from the trachea or blood. In congenital pneumonia due to chorioamnionitis the gastric aspirate at birth contains bacteria and pus cells on Gram stain.
Treatment:
 general measures as for respiratory distress
 specific therapy with appropriate antibiotics
PNEUMOTHORAX
Common causes:
 meconium aspiration
 vigorous resuscitation after birth
 hyaline membrane disease, especially if on positive pressure ventilation or CPAP
 A small pneumothorax may have few clinical signs. In more severe cases, especially those with tension pneumothorax, there is a rapid deterioration in condition.
 The infant becomes increasingly dyspnoeic or apnoeic and is often pale or cyanosed.
 The affected side will tend to be hyper-resonant to percussion and breath sounds are diminished.
 The affected side will also transilluminate with a bright light source. A chest x-ray will confirm the diagnosis.
Treatment:
Mild degrees of pneumothorax may resolve spontaneously. In some cases the rate of resorption of the pneumothorax in term infants can be accelerated by letting the baby breathe a high oxygen concentration. More severe cases will need an under water chest drain.
DIAPHRAGMATIC HERNIA
 Herniation of abdominal viscera through the diaphragm (usually on the left side) usually presents at birth with severe respiratory distress, cyanosis and a shift of the mediastinum and interference with lung function.
 The main diagnostic clues are maternal polyhydramnios, shift of the maximal heart sounds to the right and a scaphoid appearance of the abdomen. Bowel sounds may be heard in the chest.
 Many cases are detected by routine antenatal ultrasonography.
 X-ray immediately confirms the diagnosis - the condition must be regarded as a surgical emergency for early surgery may be life-saving.
CHRONIC LUNG DISEASE(Bronchopulmonary dysplasia)
 Preterm infants who require intubation and ventilation for respiratory distress may develop chronic lung disease (bronchopulmonary dysplasia).
 They have respiratory distress and need additional oxygen for weeks to months.
 Some may also need prolonged ventilation.
 Chest X-ray shows hyperinflation, streaky fibrosis and a bubbly appearance
 . Steroids may help wean these infants off a ventilator and hasten recovery.
 Severe cases die of respiratory failure or secondary pneumonia.
 Antenatal steroids and possibly surfactant therapy reduces the risk of chronic lung disease
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