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Questions and Answers
What imaging sign is associated with intussusception in a transverse image?
What imaging sign is associated with intussusception in a transverse image?
- String sign
- Claw sign
- Ring sign
- Doughnut sign (correct)
Only surgical reduction is the treatment required for children with intussusception.
Only surgical reduction is the treatment required for children with intussusception.
False (B)
What is the most common site affected by Hirschsprung disease?
What is the most common site affected by Hirschsprung disease?
Rectum
The primary pathology of Hirschsprung disease involves failure of migration of ______ from the neural crest.
The primary pathology of Hirschsprung disease involves failure of migration of ______ from the neural crest.
Match the following investigations with their descriptions:
Match the following investigations with their descriptions:
Which of the following is a characteristic of asthma?
Which of the following is a characteristic of asthma?
Exercise-induced asthma is primarily associated with allergic clinical phenotypes.
Exercise-induced asthma is primarily associated with allergic clinical phenotypes.
What is the primary treatment for the early phase of allergic asthma?
What is the primary treatment for the early phase of allergic asthma?
The early phase of allergic asthma typically starts within ______ minutes after exposure to an allergen.
The early phase of allergic asthma typically starts within ______ minutes after exposure to an allergen.
Match the components with their corresponding phases of allergic asthma:
Match the components with their corresponding phases of allergic asthma:
What is the most common age group for foreign bodies in the airway?
What is the most common age group for foreign bodies in the airway?
Pulmonary sequestration is characterized by the part of the lung having its own bronchial communication.
Pulmonary sequestration is characterized by the part of the lung having its own bronchial communication.
What is the most common type of foreign body found in the airway of young children?
What is the most common type of foreign body found in the airway of young children?
In pulmonary sequestration, the blood supply comes from the ______.
In pulmonary sequestration, the blood supply comes from the ______.
Match the following features with their corresponding types of pulmonary sequestration:
Match the following features with their corresponding types of pulmonary sequestration:
Which of the following symptoms may indicate a retained foreign body in the respiratory system?
Which of the following symptoms may indicate a retained foreign body in the respiratory system?
A chest X-ray is a reliable method to identify foreign bodies in the respiratory system.
A chest X-ray is a reliable method to identify foreign bodies in the respiratory system.
What is the primary management approach upon suspicion of a foreign body in the respiratory system?
What is the primary management approach upon suspicion of a foreign body in the respiratory system?
The _____ effect occurs during partial obstruction of the airway, leading to hyperinflation.
The _____ effect occurs during partial obstruction of the airway, leading to hyperinflation.
Match the obstruction type to its consequence:
Match the obstruction type to its consequence:
What is the first step in the management of duodenal atresia?
What is the first step in the management of duodenal atresia?
Duodenal atresia is primarily caused by the failure of recanalization of the duodenal lumen.
Duodenal atresia is primarily caused by the failure of recanalization of the duodenal lumen.
What is the preferred surgical method for connecting atretic ends of the duodenum?
What is the preferred surgical method for connecting atretic ends of the duodenum?
The double bubble sign is visible in an _______________ X-ray for duodenal atresia.
The double bubble sign is visible in an _______________ X-ray for duodenal atresia.
Match the types of duodenal atresia with their descriptions:
Match the types of duodenal atresia with their descriptions:
What is the primary issue in intussusception?
What is the primary issue in intussusception?
Intussusception mainly affects adults rather than children.
Intussusception mainly affects adults rather than children.
What is the initial step often leading to intussusception?
What is the initial step often leading to intussusception?
In intussusception, one part of the intestine telescopes into ______.
In intussusception, one part of the intestine telescopes into ______.
Match the terms related to intussusception with their definitions:
Match the terms related to intussusception with their definitions:
Which of the following is NOT a clinical feature of aganglionosis?
Which of the following is NOT a clinical feature of aganglionosis?
The absence of air in the pelvis on a plain X-ray indicates a possible case of aganglionosis.
The absence of air in the pelvis on a plain X-ray indicates a possible case of aganglionosis.
What is the primary surgical treatment for early presentation of aganglionosis?
What is the primary surgical treatment for early presentation of aganglionosis?
The __________ is the absent reflex that indicates a lack of inhibitory control in rectal manometry.
The __________ is the absent reflex that indicates a lack of inhibitory control in rectal manometry.
Match the investigation with its corresponding finding:
Match the investigation with its corresponding finding:
What is the most common site affected by congenital lobar emphysema?
What is the most common site affected by congenital lobar emphysema?
Congenital pulmonary adenomatoid malformation primarily presents with functional cysts.
Congenital pulmonary adenomatoid malformation primarily presents with functional cysts.
What is the primary treatment for congenital lobar emphysema?
What is the primary treatment for congenital lobar emphysema?
In congenital pulmonary adenomatoid malformation, the risk of lung tumors necessitates surgical resection within ______ of age.
In congenital pulmonary adenomatoid malformation, the risk of lung tumors necessitates surgical resection within ______ of age.
What FEV/FVC ratio indicates airflow limitation?
What FEV/FVC ratio indicates airflow limitation?
Match the conditions with their characteristics:
Match the conditions with their characteristics:
A biological agent used for asthma management includes Omalizumab, which is an Anti-IL-5.
A biological agent used for asthma management includes Omalizumab, which is an Anti-IL-5.
What is the controller therapy step for a patient experiencing daily symptoms and night symptoms at least once a week?
What is the controller therapy step for a patient experiencing daily symptoms and night symptoms at least once a week?
The short-acting beta-agonist used for sudden exacerbation of asthma symptoms is known as ______.
The short-acting beta-agonist used for sudden exacerbation of asthma symptoms is known as ______.
Match the following asthma management conditions with their appropriate controller therapy:
Match the following asthma management conditions with their appropriate controller therapy:
What is the most common congenital malformation of the larynx?
What is the most common congenital malformation of the larynx?
Stridor in laryngomalacia is characterized as having a normal sound profile.
Stridor in laryngomalacia is characterized as having a normal sound profile.
At what age does laryngomalacia typically resolve?
At what age does laryngomalacia typically resolve?
In laryngomalacia, the shape of the epiglottis is typically described as _____ shaped.
In laryngomalacia, the shape of the epiglottis is typically described as _____ shaped.
Match the airway conditions with their characteristics:
Match the airway conditions with their characteristics:
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Study Notes
Intussusception
- Intussusception is an intestinal obstruction where one part of the intestine telescopes into another.
- A lead point mass formation is often the initial step.
- Young children are more susceptible.
- Ultrasound (USG) is a common investigation technique, showing a 'doughnut' sign in transverse images.
- Barium contrast enema is rarely used, but can demonstrate a 'claw sign'.
- Treatment aims to reduce the intussusception, which can be achieved through USG-guided hydrostatic reduction using saline, contrast medium, or air.
- Surgical reduction is employed if other methods fail.
Hirschsprung Disease
- Caused by a failure of migration of neurons from the neural crest.
- Most commonly affects the rectum, followed by the sigmoid colon.
- Aganglionosis (absence of nerve cells) occurs both in the submucosal and myenteric plexuses.
- Associated with Down's syndrome.
Asthma
- Characterized by reversible airway obstruction, airway hyperresponsiveness, and chronic airway inflammation.
- Variable intensity and frequency of episodes.
- Allergic asthma is the most common type in children, associated with allergic conditions and responding well to treatment.
- Non-allergic asthma is more prevalent in adults, lacking a family history and often difficult to treat.
- Exercise-induced asthma is another form.
- Two main phases:
- Early phase: triggered by allergens, leading to mast cell degranulation and the release of inflammatory mediators like histamine, prostaglandins, and leukotrienes. Results in bronchoconstriction, mucosal edema, and increased secretions.
- Late phase: occurs 3-4 hours after the early phase, characterized by recruitment of inflammatory cells like eosinophils, basophils, and lymphocytes. Triggered by repeated allergen exposure.
- Treatment typically involves β2-agonists (bronchodilators) and, for more severe cases, steroids.
Pulmonary Sequestration
- Segregation or isolation of a non-functioning part of the lung with no communication with the bronchus.
- Blood supply originates from the systemic circulation.
- Increased risk of recurrent infections.
- Commonly located in the lower lobes of the lung fields.
- Classified as intrapulmonary (shared pleural covering) or extrapulmonary (separate pleural covering).
- Investigation includes chest X-ray and CT scan.
- Treatment involves surgical resection.
Foreign Bodies in the Airway
- A paediatric emergency, typically affecting children under 3 years of age.
- Common objects include nuts, coins, and small toys.
- May cause immediate choking or delayed presentation with recurrent wheeze, breathing difficulty, suppurative complications, or pneumonia.
- Consequences of obstruction include complete collapse (atelectasis) or hyperinflation due to a ball valve effect.
- Important to initiate management if there is suspicion, suggestive history, focal lung findings, or abnormal X-rays.
- Removal is performed using a rigid bronchoscope for lower airway objects and Heimlich's maneuver for upper airway objects in children older than 1 year.
Duodenal Atresia
- Failure of the duodenal lumen's recanalization.
- Associated with Down's syndrome.
- Presents with polyhydramnios and bilious vomiting soon after birth.
- Types include Type 1 (connected by a fibrous band), Type 2 (connected together), and Type 3 (disconnected with a gap).
- Investigations include X-ray (revealing a "double bubble" sign ), USG, and CT scan.
- Treatment involves surgical correction, either duodeno-duodenostomy or duodeno-jejunostomy.
Gastrointestinal System
- Consequences of aganglionosis include:
- Impaired intestinal motility (peristalsis).
- Sustained contraction without relaxation due to missing nerve fibers.
- Narrowing of the intestine, particularly in the rectum.
- A common cause of congenital motility disorder and lower intestinal obstruction in infancy.
Clinical Features of Hirschsprung Disease
- Delayed passage of meconium (more than 48 hours after birth).
- Abdominal distension.
- Normal anal sphincter with a narrowed lumen on rectal examination.
Investigations for Hirschsprung's Disease
- Radiological investigation includes a X-ray (showing absence of air in the pelvis) and a barium contrast enema (diagnostic, showing segments that may not be visualized).
- Rectal manometry reveals the absence of the recto-anal inhibitory reflex (RAIR).
- Rectal biopsy shows absent ganglia and hypertrophied nerve fibers.
Treatment for Hirschsprung's Disease
- Early presentation:
- Pull-through procedure involves removing the affected segment and pulling the unaffected proximal segment through and attaching it to the anus.
- Delayed presentation:
- Colostomy is performed to relieve obstruction.
- Pull-through procedure is then carried out.
Airway Malformations and Foreign Bodies
Laryngomalacia
- The most common congenital malformation of the larynx.
- Refers to softness, floppiness, or laxity of supraglottic structures (epiglottis, arytenoids, aryepiglottic folds).
- Presents with stridor, an indication of upper airway obstruction.
- Onset is early, either right after birth or within a few weeks.
- Stridor is usually intermittent, occurring during crying, agitation, or after feeding, and is often worse in the supine position.
- Laryngoscopy is used to diagnose laryngomalacia.
- Treatment mainly involves reassurance as the condition typically resolves by 6-12 months of age.
Laryngomalacia vs Congenital Subglottic Stenosis
| Feature | Laryngomalacia | Congenital subglottic stenosis |
|---|---|---|
| Positional variation of stridor | + (cry) | - (Normal) |
| Stridor | Muffled | Normal |
| Breathing pattern | Inspiratory | Biphasic (Inspiration and Expiration) |
Congenital Lobar Emphysema
- Characterized by cartilaginous bronchial dysplasia leading to abnormal compliance of bronchi, causing near complete collapse during expiration.
- Results in air trapping, hyperinflation, and emphysema.
- Most common sites are the left upper lobe and right middle lobe.
- Emphysema compresses other lobes, causing atelectasis, impaired oxygenation, and respiratory distress, especially in newborns.
- Mediastinal shift occurs towards the unaffected side.
- Investigation includes chest X-ray and CT scan.
- Treatment involves lobectomy of the affected lobe.
Congenital Pulmonary Adenomatoid Malformation (CPAM)
- Hamartomatous or dysplastic lung tissue with multiple cysts.
- The most common cause of cystic lung disease in newborns.
- Cysts are non-functional, resulting in respiratory distress and increased infection risk.
- Diagnosed via chest X-ray and CT scan.
- Treatment involves surgical resection within the first year of life to minimize the risk of lung tumors like sarcoma or carcinoma.
Lung Function Abnormalities
| Condition | Parameters |
|---|---|
| Airflow limitation | FEV ↓ |
| Bronchodilator response | FEV ↑ by > 12% |
| Exercise challenge | Airflow obstruction worsened: FEV ↓ by > 15% |
| Diurnal variation/Am to Pm variation | FEV, or PEF (Peak Expiratory Flow) ↑ by > 20% |
| FEV/FVC ratio | < 0.8 |
Management of Asthma (GINA Guidelines)
- Focus on identifying and eliminating exacerbating factors.
- Employ long-term management strategies.
- Address acute exacerbations effectively.
Long-Term Management of Asthma (Age 6-11 years)
- Stepwise management based on symptom frequency:
- Step I: < 2 exacerbations per month, use short-acting beta-agonist (SABA) as needed and low-dose inhaled corticosteroid (ICS) if SABA is used frequently.
- Step II: > 2 exacerbations per month but not daily, use daily low-dose ICS.
- Step III: Daily symptoms plus night symptoms ≥ 1/week, use daily low-dose ICS + long-acting beta-agonist (LABA) OR daily medium-dose ICS.
- Step IV: Daily symptoms plus night symptoms ≥ 1/week plus low lung function, use daily medium-dose ICS + LABA OR daily high-dose ICS.
- Step V: Uncontrolled on Step IV, use daily high-dose ICS + LABA (+/- other treatments) and consider biological agents as last resort.
Biological Agents (Last Resort)
- Omalizumab (anti-IgE)
- Mepolizumab (anti-IL-5)
- Dupilumab (anti-IL-4 receptor)
- Anti-TSLP (Thymic stromal lymphopoietin): New drug
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