Paeds II Flashcards
Describe the investigations of epiglottitis [2]
If the patient is acutely unwell and epiglottitis is suspected then investigations should not be performed.
- lateral xray of the neck shows a characteristic “thumb sign” or “thumbprint sign” from oedematous and swollen epiglottis.
- Neck xrays are also useful for excluding a foreign body.
Describe the management of epiglottitis [+]
A key point that is often talked about with epiglottitis is the importance of not distressing the patient, as this could prompt closure of the airway. If you see a child with suspected epiglottitis, leave them well alone and in their comfort zone
- Alert senior paediatrician and anaesthetist
Management of epiglottis centres around ensuring the airway is secure.
- Most patients do not require intubation
- However there is an ongoing risk of sudden upper airway closure, so preparations need to be made to perform intubation at any time.
- Intubation is often difficult and needs to be performed in a controlled environment with facilities available to do a tracheostomy (intubating through the neck) if the airway completely closes.
- When patients are intubated they are transferred to an intensive care unit.
Additional treatment once the airway is secure:
* IV antibiotics (e.g. ceftriaxone)
* Steroids (i.e. dexamethasone)
* IV fluids
Vaccination:
- Ensuring that children
receive the Haemophilus influenzae type B (Hib) vaccine is crucial for preventing pediatric cases of acute epiglottitis.
Describe a complication of epiglottitis [1]
A common complication to be aware of is the development of an epiglottic abscess, which is a collection of pus around the epiglottis. This also threatens the airway, making it a life threatening emergency. Treatment is similar to epiglottitis.
Describe the clinical features of whooping cough [+]
The typical clinical findings include 2-3 days of coryza precede onset of:
coughing bouts
* usually worse at night and after feeding, may be ended by vomiting
* central cyanosis may occasionally be seen
* more severe coughing normally starts after a week or more where get sudden and reoccuring coughing attacks coupled with gaps inbetween (paroxysmal cough)
inspiratory whoop
- not always present
infants may have spells of apnoea
persistent coughing may cause subconjunctival haemorrhages or even anoxia leading to syncope & seizures
Describe how you dx whooping cough [2]
A nasopharyngeal or nasal swab with PCR testing or bacterial culture can confirm the diagnosis within 2 to 3 weeks of the onset of symptoms.
Where the cough has been present for more than 2 weeks patients can be tested for the anti-pertussis toxin immunoglobulin G this is tested for in the oral fluid of children aged 5 to 16 and in the blood of those aged over 17.
Mx for whooping cough? [3]
Supportive care
Macrolide Abx:
- azithromycin, erythromycin and clarithromycin can be beneficial in the early stages (within the first 21 days) or vulnerable patients.
- Co-trimoxazole is an alternative to macrolides.
Infants under 6 months with suspect pertussis should be admitted
Name 4 complications of whooping cough infection [4]
Complications
* subconjunctival haemorrhage
* pneumonia
* bronchiectasis
* seizures
NB: The symptoms typically resolve within 8 weeks, however they can last several months. It is also known as the “100-day cough” due to the potential long duration of the cough
When does routine immunisation of infants for whooping cough occur? [4]
infants are routinely immunised at 2, 3, 4 months and 3-5 years
NB: neither infection nor immunisation results in lifelong protection - hence adolescents and adults may develop whooping cough despite having had their routine immunisations
When in a pregnancy are women offered a whooping cough vaccine? [1]
Women who are between 20-32 weeks pregnant will be offered the vaccine.
Describe the common presentating symptoms of CF [+]
- Chronic cough
- Thick sputum production
- Recurrent respiratory tract infections
- Loose, greasy stools (steatorrhoea) due to a lack of fat digesting lipase enzymes
- Abdominal pain and bloating
- Parents may report the child tastes particularly salty when they kiss them, due to the concentrated salt in the sweat
- Poor weight and height gain (failure to thrive)
Describe the common presentating signs of CF [5]
- Low weight or height on growth charts
- Nasal polyps
- Finger clubbing
- Crackles and wheezes on auscultation
- Abdominal distention
Describe the pathological manifestations of CF in the respiratory system [4]
Persistent cough:
- Productive or non-productive, often exacerbated by mucus accumulation and infections.
Wheezing and dyspnea:
- Resulting from airway obstruction and bronchospasm.
Recurrent respiratory infections:
- Commonly caused by pathogens such as
Staphylococcus aureus, Haemophilus influenzae, and Pseudomonas aeruginosa.
Nasal polyps and chronic sinusitis:
- Due to mucus buildup and chronic inflammation in the sinuses.
Describe the pathological manifestations of CF in the GI system [5]
- Meconium ileus: Neonates with CF may present with intestinal obstruction due to thickened meconium.
- Pancreatic insufficiency: Steatorrhea, weight loss, and malabsorption of fat-soluble vitamins due to pancreatic duct obstruction.
- Distal intestinal obstruction syndrome (DIOS): Partial or complete intestinal obstruction due to inspissated faecal material.
- Biliary cirrhosis: Impaired bile flow leading to liver damage, portal hypertension, and potential liver failure.
- Gastroesophageal reflux disease (GERD): Increased prevalence in CF patients may exacerbate pulmonary complications.
TOM TIP: The key colonisers to remember for your exams are [2]. Patients with cystic fibrosis take long term prophylactic [] to prevent [1] infection.
TOM TIP: The key colonisers to remember for your exams are staph aureus and pseudomonas. Patients with cystic fibrosis take long term prophylactic flucloxacillin to prevent staph aureus infection.
Pseudomonas should be remembered as a particularly troublesome coloniser that is hard to treat and worsens the prognosis of patients with cystic fibrosis.
How can you treat long term pseudomonas infection? [2]
Pseudomonas colonisation can be treated with long term nebulised antibiotics such as tobramycin.
Oral ciprofloxacin is also used.
Describe the investigations used for CF
Newborn screening:
- Most cases of cystic fibrosis (CF) are identified through newborn screening programs, which typically involve measuring immunoreactive trypsinogen (IRT) in blood samples.
- An elevated IRT level warrants further testing, such as sweat chloride testing or genetic analysis
.
Sweat chloride test:
- The gold standard diagnostic test for CF, this measures the concentration of chloride in sweat
- Elevated chloride levels (>60 mmol/L) are diagnostic of CF, while intermediate levels (30-59 mmol/L) require further investigation.
Genetic testing:
- Identifying mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene can confirm the diagnosis, provide prognostic information, and guide treatment decisions. However, not all CFTR mutations are detectable through current testing methods.
Describe the management of CF [+]
Airway clearance:
- Chest physiotherapy: active cycle of breathing, autogenic drainage, and positive expiratory pressure device
- High-frequency chest wall oscillation: rapid oscillations to loosen and clear mucus from the airways.
- Exercise: Regular physical activity promotes airway clearance, enhances lung function, and improves overall health.
Pharmacologic interventions:
- Mucolytics: Agents like dornase alfa and hypertonic saline reduce mucus viscosity, improving airway clearance and lung function.
- Bronchodilators: Inhaled β2-agonists and anticholinergics help relax airway smooth muscle, improving airflow and lung function.
- Anti-inflammatories: inhaled corticosteroids and oral nonsteroidal anti-inflammatory drugs (e.g., ibuprofen) help reduce airway inflammation and improve lung function.
- Antibiotics: Regular use of inhaled antibiotics (e.g., tobramycin, aztreonam) helps manage chronic infections, particularly with Pseudomonas aeruginosa. Systemic antibiotics may be required for acute exacerbations. Prophylactic flucloxacillin tablets to reduce the risk of bacterial infections (particularly staph aureus)
- CFTR modulators: Small molecule drugs like ivacaftor, lumacaftor, and elexacaftor target specific CFTR mutations, improving protein function and clinical outcomes. Selection depends on the patient’s specific CFTR genotype.
Describe the nutritional management for patients with CF [3]
Pancreatic enzyme replacement therapy (PERT):
- Oral administration of pancreatic enzymes with meals assists digestion and absorption of nutrients.
Fat-soluble vitamin supplementation:
- Ensuring adequate intake of vitamins A, D, E, and K helps prevent deficiencies due to malabsorption.
High-energy diet:
- A high-calorie, high-fat diet addresses increased energy requirements and compensates for malabsorption, promoting growth and weight maintenance.
Patients with CF need monitoring for which conditions? [5]
Patients with cystic fibrosis are managed and followed up in specialist clinics, typically every 6 months. They require regular monitoring of their sputum for colonisation of bacteria like pseudomonas.
They also need monitoring and screening for diabetes, osteoporosis, vitamin D deficiency and liver failure.
rectal prolapse (due to bulky stools)
Describe the pathophysiology of sepsis [+]
The causative pathogens are recognised by macrophages, lymphocytes and mast cells
These cells release vast amounts of cytokines, such as interleukins and tumor necrosis factor, to alert the immune system to the invader.
These cytokines activate other parts of the immune system. This immune activation leads to further release of chemicals such as nitrous oxide that causes vasodilation. The immune response causes inflammation throughout the body.
Many of these cytokines cause the endothelial lining of blood vessels to become more permeable.
This causes fluid to leak out of the blood into the extracellular space, leading to oedema and a reduction in intravascular volume.
The oedema around blood vessels creates a space between the blood and the tissues, reducing the amount of oxygen that reaches the tissues.
Activation of the coagulation system leads to deposition of fibrin throughout the circulation, further compromising organ and tissue perfusion. It also leads to consumption of platelets and clotting factors, as they are being used up to form the blood clots. This leads to thrombocytopenia, haemorrhages and an inability to form clots and stop bleeding. This is called disseminated intravascular coagulopathy (DIC).
Blood lactate rises as a result of anaerobic respiration in the hypo-perfused tissues with an inadequate oxygen. A waste product of anaerobic respiration is lactate.
What are the signs of sepsis? [+]
Typically presents as SHOCK:
- Tachycardia
- Tachypnoea
- Prolonged CRT
- Low BP - late sign!
- Deranged physical observations
- Prolonged capillary refill time (CRT)
- Fever or hypothermia
- Deranged behaviour
- Poor feeding
- Inconsolable or high pitched crying
- High pitched or weak cry
- Reduced consciousness
- Reduced body tone (floppy)
- Skin colour changes (cyanosis, mottled pale or ashen)
- Shock involves circulatory collapse and hypoperfusion of organs.
Describe the tx of sepsis [+]
A-E
Blood tests, including a FBC, U&E, CRP, clotting screen (INR), blood gas for lactate and acidosis
Blood cultures, ideally before giving antibiotics
Intubate and ventilate if required
Fluid resus
- 20ml/kg IV bolus of normal saline if the lactate is above 2 mmol/L or there is shock. This may be repeated.
Inotropes
IV Ceftriaxone (within 1hr)
Glucose if required
Electrolyte correction
Correction of coagulopathy
How do you give fluids if lactacte is above 2 mmol/L in paed. sepsis? [1]
IV fluids: 20ml/kg IV bolus of normal saline if the lactate is above 2 mmol/L or there is shock. This may be repeated.
