Mock exam for AKP

CORRECT ANSWER:

The immediate priority is the child's safety. The parents are attempting to remove the child, who has a highly suspicious injury, from a place of safety, creating an emergency.

Police Protection under Section 46 of the Children Act 1989 is the most appropriate immediate power as it allows a police constable to prevent the removal of a child from hospital for up to 72 hours if they have reasonable cause to believe the child would otherwise suffer significant harm. This action does not require a court order and can be implemented immediately upon police arrival, directly addressing the imminent risk.

The paediatrician's duty is to recognise this immediate danger, alert security and call the police to use their statutory powers, as doctors cannot lawfully detain a child in these circumstances.

WRONG ANSWER ANALYSIS:

Option A (Section 47 Enquiry) is incorrect because this is a social care-led investigation to determine if a child is suffering, or likely to suffer, significant harm; it is a process, not an immediate power to prevent a child leaving.

Option B (Section 46) is not applicable as it is the correct answer.

Option C (Emergency Protection Order) is incorrect because, while it is an emergency measure, it requires an application to a court which causes a delay, making it unsuitable for a situation where parents are actively trying to leave.

Option D (Care Order) is incorrect as this is a long-term court order, granted after extensive court proceedings, which transfers parental responsibility to the local authority and is not used for immediate protection.

Option E (Section 20) is incorrect because this is a voluntary arrangement that requires parental consent to accommodate a child, which is clearly absent in this scenario of parental aggression and refusal.

CORRECT ANSWER:

The priority is to report the incident to the police. GMC guidance is explicit that when a patient presents with an injury from an attack with a knife, this information should be disclosed to the police without consent.

This duty is justified in the public interest to help prevent or prosecute serious crime and to protect others from harm. While the immediate clinical care of the patient is paramount, the nature of the injury strongly suggests a serious violent crime has occurred, creating a significant risk to public safety.

In paediatrics, any such injury also automatically triggers safeguarding concerns that must be considered alongside the police report. The decision to override confidentiality is based on the serious nature of the crime and the potential for further violence, not on the patient's age or capacity to consent.

WRONG ANSWER ANALYSIS:

Option A (No, confidentiality is absolute) is incorrect because confidentiality is not absolute and can be breached without consent if it is in the public interest, such as in the prevention or prosecution of serious crime.

Option C (Only if the boy consents) is incorrect because the public interest in reporting a serious crime like a stabbing overrides the need for patient consent, even if the patient is Gillick competent.

Option D (Only if the parents consent) is incorrect as the duty to report is based on public interest and is independent of parental consent, especially concerning a 14-year-old who may have capacity.

Option E (Only if the injury is life-threatening) is incorrect because the requirement to report is based on the mechanism of injury (a knife attack) indicating a serious crime, not the clinical severity of the wound itself.

CORRECT ANSWER:

The clinical presentation is highly suggestive of Prader-Willi Syndrome (PWS). This is characterised by a biphasic natural history: infantile hypotonia with feeding difficulties, followed by the onset of hyperphagia and rapid weight gain in early childhood.

The additional features of speech delay and small hands and feet further support this diagnosis. PWS is a genomic imprinting disorder affecting chromosome 15. Methylation analysis is the definitive first-line diagnostic test as it detects the abnormal parent-specific DNA methylation pattern present in over 99% of individuals, irrespective of the underlying genetic mechanism (paternal deletion, maternal uniparental disomy, or an imprinting centre defect). National guidelines recommend genetic testing to confirm a clinical suspicion of PWS.

WRONG ANSWER ANALYSIS:

Option A (Karyotype) is incorrect because it has insufficient resolution to detect the common microdeletions on chromosome 15 that cause most cases of Prader-Willi Syndrome.

Option B (Microarray) is incorrect as, while it can identify deletions, it will not detect cases caused by uniparental disomy or imprinting defects, which are also major causes.

Option D (CK levels) is incorrect because this test investigates primary muscle disease (myopathy), whereas the hypotonia in this context is central in origin and part of a wider genetic syndrome.

Option E (MRI Brain) is incorrect as it is a structural imaging modality and is not the primary method for diagnosing a specific genetic imprinting disorder like Prader-Willi Syndrome.

CORRECT ANSWER:

Active internal bleeding is an absolute contraindication to thrombolysis with Alteplase. The primary mechanism of Alteplase is to dissolve fibrin clots, but this action is not specific to the cerebral thrombus.

In the presence of active haemorrhage, administering a thrombolytic agent will impair haemostasis and can lead to catastrophic, uncontrollable bleeding, which poses a greater immediate threat to life than the ischaemic stroke itself. National guidelines from bodies like the RCPCH are clear that the risk of exacerbating an existing bleed far outweighs any potential benefit from reperfusion therapy. Therefore, the immediate priority is to identify and control any source of active bleeding before any consideration of thrombolysis.

WRONG ANSWER ANALYSIS:

Option A (Seizure at onset of stroke) is incorrect because, while a seizure at onset necessitates careful evaluation to exclude stroke mimics like Todd's paresis, it is considered a relative, not absolute, contraindication if stroke is confirmed.

Option B (Blood glucose > 22 mmol/L) is incorrect as significant hyperglycaemia is a relative contraindication that should be corrected, but it does not absolutely preclude the use of Alteplase.

Option C (Previous stroke 6 months ago) is incorrect because the standard exclusion criterion is typically for a stroke within the previous 3 months, making a stroke 6 months prior a less significant contraindication.

Option E (Age < 18 years) is incorrect because although Alteplase is not licensed for paediatric use, national protocols and guidelines support its off-label use in specialised centres for carefully selected paediatric patients.

CORRECT ANSWER:

The single most significant modifiable risk factor for SUDEP is the frequency of generalised tonic-clonic seizures (GTCS). NICE guidance explicitly states that uncontrolled seizures, particularly GTCS, are a key modifiable risk factor.

Poor adherence to anti-seizure medication is a primary driver for uncontrolled seizures. In clinical practice, the priority is therefore to optimise seizure control by addressing adherence. This involves exploring reasons for missed doses with the adolescent and family, simplifying medication regimens, and providing education on the critical link between consistent treatment and reducing mortality risk.

While other factors contribute, achieving the best possible seizure control through medication adherence offers the greatest potential to reduce the risk of SUDEP.

WRONG ANSWER ANALYSIS:

Option B (Sleeping prone) is a recognised risk factor, but it is secondary to the risk conferred by the seizure itself; optimising seizure control is the primary preventative strategy.

Option C (Use of Valproate) is incorrect as no specific anti-seizure medication is directly implicated as a primary risk factor for SUDEP; the focus is on the effectiveness of treatment, not the specific drug.

Option D (Age > 15) is a non-modifiable risk factor; while SUDEP risk increases in adolescence and young adulthood, age itself cannot be changed.

Option E (Male sex) is also a non-modifiable demographic risk factor and is considered less significant than the frequency of GTCS.

CORRECT ANSWER:

In the management of severe acute asthma in children, ipratropium bromide is an important adjunct to beta-agonists like salbutamol. National guidelines recommend its use in children with severe or life-threatening features.

The standard dosing frequency is every 4-6 hours. This is because ipratropium, an anticholinergic agent, has a longer duration of action than salbutamol. While initial doses may be given more frequently (e.g., every 20-30 minutes for the first two hours) in severe cases, this is quickly spaced out to 4-6 hourly.

This regimen provides sustained bronchodilation by acting on a different pathway to salbutamol, without the cumulative risk of significant anticholinergic side effects (like tachycardia, dry mouth, or ileus) that more frequent, prolonged dosing would cause. The clinical priority is to maximise bronchodilation safely, and a 4-6 hourly schedule achieves this balance effectively.

WRONG ANSWER ANALYSIS:

Option A (Every 20-30 minutes for first 2 hours) is incorrect as this frequency is typically reserved for salbutamol nebulisers in the initial phase, not for ongoing ipratropium bromide administration.

Option C (Every 12 hours) is incorrect because this interval is too infrequent to provide adequate bronchodilation and control during a severe, acute exacerbation.

Option D (Once daily) is incorrect as it is a maintenance dosing strategy for other conditions and is completely inappropriate for acute severe asthma.

Option E (Continuous infusion) is incorrect because ipratropium bromide is not administered as a continuous infusion; this route is reserved for agents like salbutamol in refractory life-threatening asthma.

CORRECT ANSWER:

A Driver Diagram is a core Quality Improvement tool used to translate a high-level aim into actionable steps. It provides a visual representation of the team's theory for change, linking the project's overall goal to the factors that will influence it.

This structure typically moves from left to right, starting with the specific, measurable Aim. This is then broken down into 'Primary Drivers', which are the high-level system components or factors that directly influence the aim. These are further broken down into 'Secondary Drivers', which are more specific elements. Finally, on the far right, are the specific 'Change Ideas' – the tangible interventions that the team will test using Plan-Do-Study-Act (PDSA) cycles. This logical flow helps teams maintain focus and communicate their strategy clearly to all stakeholders.

WRONG ANSWER ANALYSIS:

Option A (To map the patient journey) is incorrect because this is the function of a Process Map, which visually details every step in a patient's experience through a clinical pathway.

Option B (To identify the root cause of an error) is incorrect as this is achieved through Root Cause Analysis (RCA), often utilising tools like Fishbone (Ishikawa) diagrams or the '5 Whys' technique.

Option D (To display data over time) is incorrect; this describes the purpose of a Run Chart or a Statistical Process Control (SPC) chart, which are used to monitor the impact of changes.

Option E (To compare performance between consultants) is incorrect as this involves performance metric reporting or benchmarking, not the strategic planning function of a Driver Diagram.

CORRECT ANSWER:

The Medicines and Healthcare products Regulatory Agency (MHRA) mandates the 'Valproate Pregnancy Prevention Programme' for all female patients of childbearing potential. This programme is a legal requirement, not just a guideline.

Its central component is the annual completion of a Risk Acknowledgment Form by both the patient (or guardian) and the specialist prescriber. This ensures a formal, documented discussion has occurred regarding the significant risks of major congenital malformations and neurodevelopmental disorders in children exposed to valproate in utero. For a 16-year-old, this signed acknowledgment is the mandatory priority to confirm she understands these risks and the critical need for effective contraception, assuming valproate is deemed the only suitable treatment by her specialist. This process reinforces patient safety and informed consent in paediatric practice.

WRONG ANSWER ANALYSIS:

Option B (She must switch to Carbamazepine immediately) is incorrect because an immediate switch is not mandatory; valproate may be the only effective treatment, and the decision to change therapy requires careful specialist evaluation.

Option C (She must have a pregnancy test every month) is incorrect because while a pregnancy test is required before starting treatment, the core mandatory requirement of the programme is the annual risk acknowledgment, not monthly testing.

Option D (She must be sterilized) is incorrect because sterilisation is not a requirement of the programme and is an inappropriate suggestion for a 16-year-old.

Option E (No specific requirement if she uses condoms) is incorrect because condoms alone are not considered highly effective contraception, and the programme mandates the use of highly effective contraception alongside the annual risk review.

CORRECT ANSWER:

Fatigue is the most significant physiological contributor to error in this context. Working multiple consecutive night shifts disrupts circadian rhythms, leading to significant cognitive slowing, impaired decision-making, and reduced vigilance.

This state is a core concept in Human Factors training, which recognises that performance degradation from fatigue can be equivalent to that of alcohol intoxication. In a high-stakes resuscitation scenario, this cognitive impairment directly increases the risk of calculation errors. The "HALT" mnemonic (Hungry, Angry, Late/Lonely, Tired) is a practical tool used to prompt clinicians to recognise these personal physiological and psychological factors that can compromise patient safety. The key issue here is the doctor's physiological state after four nights, making fatigue the primary human factor.

WRONG ANSWER ANALYSIS:

Option A (Lack of knowledge) is less likely as calculation errors are often slips in execution rather than a fundamental gap in understanding.

Option B (Distraction) is a plausible environmental factor, but the question highlights the doctor's personal condition over external events.

Option D (Poor equipment) relates to system factors, whereas the error described is a cognitive mistake made by the individual.

Option E (Lack of supervision) is a system issue that might fail to catch an error, but it is not the direct physiological cause of the error itself.

CORRECT ANSWER:

Hepatitis B virus (HBV) carries the highest transmission risk following a percutaneous injury from a known positive source. The risk is approximately 30% if the source is Hepatitis B e-antigen (HBeAg) positive, which indicates high viral infectivity and active viral replication.

In contrast, if the source is HBeAg negative, the risk is lower. This is an order of magnitude higher than the risk from Hepatitis C (around 1-3%) and two orders of magnitude higher than from HIV (around 0.3%). Therefore, in an unvaccinated individual, immediate post-exposure prophylaxis protocols are critical following potential HBV exposure. This knowledge is fundamental for managing occupational health incidents and counselling staff and patients appropriately in any paediatric or adult setting.

WRONG ANSWER ANALYSIS:

Option A (HIV) is incorrect because its transmission risk via a needlestick injury is significantly lower, estimated at only 0.3%.

Option B (Hepatitis C) is incorrect as the risk of seroconversion is approximately 1-3%, which is substantially less than for HBeAg-positive Hepatitis B.

Option D (Cytomegalovirus) is incorrect because while it can be transmitted via blood, it is not considered a primary risk in this context compared to HBV, and its main transmission routes are saliva and urine.

Option E (Epstein-Barr Virus) is incorrect as it is primarily transmitted through saliva and is not a significant cause for concern in the context of a percutaneous blood exposure.

CORRECT ANSWER:

The clinical scenario describes nausea and vomiting secondary to two distinct causes: chemical (opioid-induced stimulation of the chemoreceptor trigger zone) and gastric stasis.

Metoclopramide is a dopamine (D2) receptor antagonist that acts both centrally on the chemoreceptor trigger zone and peripherally as a prokinetic agent, increasing gastric motility and accelerating gastric emptying. This dual action makes it the most physiologically appropriate choice to target both identified causes.

National guidelines recommend a stepwise approach to managing nausea and vomiting in paediatric palliative care, and for opioid-induced nausea, a dopamine antagonist like metoclopramide or haloperidol is often recommended. Given the explicit mention of gastric stasis, metoclopramide's prokinetic effect makes it superior to other anti-emetics in this context. It is also suitable for subcutaneous administration via a syringe driver, which is essential for a child at the end of life who is unable to tolerate oral medication.

WRONG ANSWER ANALYSIS:

Option A (Ondansetron) is incorrect because as a 5HT3 antagonist, it is most effective for chemotherapy-induced or post-operative nausea and vomiting, and it lacks the necessary prokinetic effect to address gastric stasis.

Option B (Cyclizine) is incorrect because this antihistamine is more effective for nausea caused by vestibular issues or raised intracranial pressure and can worsen gastric stasis due to its anticholinergic properties.

Option D (Dexamethasone) is incorrect because while it can be a useful broad-spectrum anti-emetic in palliative care, particularly for raised intracranial pressure or chemical causes, it has no prokinetic action.

Option E (Hyoscine Butylbromide) is incorrect because this antimuscarinic agent is primarily used to treat bowel colic and excess respiratory secretions; its anticholinergic effects reduce gut motility, which would exacerbate gastric stasis.

CORRECT ANSWER:

These are hyaline bodies, which are globular deposits of protein and calcium salts that accumulate in the optic nerve head. They are a common cause of pseudopapilloedema.

The key features in this presentation are the fundoscopic findings of elevated optic discs with "lumpy" or scalloped margins, but crucially, the preservation of sharp central vessels. In true papilloedema, the swelling of the nerve fibre layer would obscure these vessels. The headache is likely an incidental finding that prompted the examination.

The presence of calcification on ocular ultrasound is pathognomonic for buried optic disc drusen and is the definitive investigation to differentiate them from true papilloedema, which is a critical neurological sign. While often benign, they can be associated with slow, progressive visual field defects.

WRONG ANSWER ANALYSIS:

Option A (Papilloedema) is incorrect because true papilloedema involves swelling of the nerve fibre layer which obscures the optic disc vessels, and it would not show calcification on ultrasound.

Option C (Optic Neuritis) is incorrect as it typically presents with acute painful vision loss and a relative afferent pupillary defect, none of which are features in this case.

Option D (Papillitis) is incorrect because it is a form of optic neuritis and would present with visual impairment and pain, not calcified deposits.

Option E (Glaucoma) is incorrect as it is characterised by optic disc cupping and nerve fibre layer loss, not a lumpy, elevated disc with calcification.

CORRECT ANSWER:

Polysomnography is the definitive, gold-standard investigation for suspected Obstructive Sleep Apnoea. This overnight sleep study provides comprehensive physiological data by recording brain waves (EEG), eye movements, muscle activity, respiratory effort, airflow, and crucially, oxygen saturations.

It is the only investigation that can accurately quantify the Apnoea-Hypopnoea Index (AHI), which is the number of apnoea or hypopnoea events per hour of sleep. This detailed assessment is essential to confirm the diagnosis, determine severity, and differentiate between obstructive and central apnoea. The results directly inform management decisions, such as the necessity for Continuous Positive Airway Pressure (CPAP) therapy or referral for adenotonsillectomy, which is first-line treatment in many younger children.

While overnight oximetry is a useful screening tool, polysomnography is required for a definitive diagnosis as per national guidelines.

WRONG ANSWER ANALYSIS:

Option A (Capnography) is incorrect because while it measures end-tidal carbon dioxide, it does not quantify the key diagnostic metrics of apnoeas and hypopnoeas required to diagnose OSA.

Option C (MRI Pharynx) is incorrect as it provides static anatomical detail but cannot assess the dynamic airway collapse that occurs during sleep.

Option D (Microlaryngoscopy) is an invasive endoscopic procedure to examine the larynx under anaesthesia and is not a primary investigation for sleep-disordered breathing.

Option E (Epworth Sleepiness Scale) is incorrect because it is a subjective questionnaire to assess daytime sleepiness, which supports the clinical history but does not diagnose the underlying cause.

CORRECT ANSWER:

The clinical presentation strongly suggests Posterior Urethral Valves (PUV), causing severe bladder outlet obstruction. This leads to high pressure within the urinary tract, resulting in bilateral hydronephrosis and progressive renal impairment, indicated by the elevated creatinine.

The immediate, life-saving priority is to decompress the bladder to prevent further irreversible kidney damage. According to national guidance and standard paediatric practice, urethral catheterisation is the first-line and least invasive method to relieve the obstruction and allow the kidneys to recover. This initial step stabilises the patient, allowing for correction of electrolyte imbalances and planning for definitive surgical treatment.

WRONG ANSWER ANALYSIS:

Option A (Suprapubic aspiration) is incorrect because it provides only temporary, intermittent bladder emptying, not the continuous drainage required.

Option B (Nephrostomy insertion) is incorrect as it is a more invasive procedure reserved for cases where bladder drainage via a urethral or suprapubic catheter is unsuccessful or contraindicated.

Option D (Circumcision) is incorrect because it is completely unrelated to the internal urethral obstruction caused by the valves.

Option E (Cystoscopy and valve ablation) is incorrect as this is the definitive surgical treatment, which should only be performed once the infant is clinically stable, not as the immediate emergency intervention.

CORRECT ANSWER:

Xanthogranulomatous Pyelonephritis (XGP) is a severe, chronic inflammatory condition where renal parenchyma is destroyed and replaced by lipid-laden macrophages (foam cells). The underlying pathophysiology is almost invariably a combination of chronic urinary tract obstruction and recurrent bacterial infection.

Obstruction, often from staghorn calculi, leads to urinary stasis, which facilitates persistent infection, commonly with Proteus or E. coli. This triggers a chronic inflammatory response, and a presumed defect in macrophage function leads to the characteristic granulomatous reaction. In paediatrics, this destructive process requires prompt recognition, as management frequently involves nephrectomy to control the inflammatory process and prevent further complications. The 'bear paw' sign on CT is a classic radiological clue, representing dilated calyces filled with pus and debris surrounding a contracted renal pelvis.

WRONG ANSWER ANALYSIS:

Option B (Tuberculosis) is incorrect because renal tuberculosis results from haematogenous spread of Mycobacterium tuberculosis, forming caseating granulomas, a distinct pathological process not primarily driven by obstruction and stones.

Option C (Lymphoma infiltration) is incorrect as it is a malignant proliferation of lymphoid cells; the kidney lacks native lymphoid tissue, and this condition is not an inflammatory response to infection and obstruction.

Option D (IgG4 related disease) is incorrect as this is a systemic autoimmune, fibro-inflammatory condition characterised by lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells, not lipid-laden macrophages.

Option E (Malakoplakia) is incorrect because it is a rare granulomatous condition resulting from defective macrophage bactericidal function, often in immunocompromised patients, and is histologically defined by Michaelis-Gutmann bodies, not the widespread parenchymal destruction seen in XGP.

CORRECT ANSWER:

Neonatal Lupus Erythematosus (NLE) is a passively transferred autoimmune disease caused by maternal IgG autoantibodies crossing the placenta. The key antibodies are Anti-Ro (SSA) and Anti-La (SSB).

This baby presents with a classic triad of cutaneous, hepatic, and haematological signs: rash, jaundice (suggesting hepatitis), and a positive Direct Antiglobulin Test (DAT) indicating haemolysis. While the rash and haematological issues are often transient and resolve as maternal antibodies wane by 6-8 months, the most feared complication is congenital heart block, which is permanent.

Therefore, any infant born to a mother with SLE, especially if Anti-Ro/La positive, requires an urgent ECG to screen for conduction abnormalities, even if asymptomatic at birth. This proactive screening is a key recommendation to mitigate morbidity.

WRONG ANSWER ANALYSIS:

Option A (Anti-D) is incorrect because it causes haemolytic disease of the newborn in an RhD-negative mother with an RhD-positive baby, which is not the case here.

Option B (Anti-Kell) is incorrect as it is another cause of haemolytic disease of the newborn but is less common and not specifically associated with the rash seen in NLE.

Option D (Anti-c) is incorrect as it is another red cell antibody causing haemolytic disease of the newborn, unrelated to the systemic and cutaneous features of neonatal lupus.

Option E (Anti-Sm) is incorrect because although it is highly specific for SLE in the mother, it is not the antibody class responsible for causing the neonatal lupus syndrome.

CORRECT ANSWER:

When apnoea of prematurity persists despite optimal caffeine therapy and CPAP, the next logical step is to escalate non-invasive respiratory support. Nasal Intermittent Positive Pressure Ventilation (NIPPV) is more effective than CPAP for treating apnoea.

It provides a baseline positive pressure, similar to CPAP, but also delivers intermittent higher pressures, effectively providing a set number of breaths. This combination of a higher mean airway pressure and a backup respiratory rate helps to reduce the frequency and severity of apnoeic events and prevent the need for invasive mechanical ventilation. This approach aligns with the principle of using the least invasive yet effective modality to provide respiratory support, thereby minimising the risks associated with intubation.

WRONG ANSWER ANALYSIS:

Option A (Intubate and ventilate) is incorrect because it is an overly invasive step; escalating non-invasive support should be attempted first.

Option B (Switch to High Flow Nasal Cannula) is incorrect as it represents a de-escalation in respiratory support from CPAP and would not be appropriate for an infant with worsening apnoeas.

Option D (Perform a septic screen and lumbar puncture) is incorrect because while sepsis must be considered, the immediate priority is to stabilise the infant's respiratory status.

Option E (Stop caffeine and start Theophylline) is incorrect as Theophylline is a second-line agent with a narrower therapeutic index and is not the standard next step when optimised caffeine therapy fails.

CORRECT ANSWER:

This child has IVIG-resistant Kawasaki Disease, defined by persistent or recrudescent fever at least 36 hours after completion of the initial IVIG infusion.

The primary goal of treatment is to reduce systemic inflammation and prevent coronary artery aneurysms. National and international guidance recommends a second dose of IVIG (2g/kg) as the most appropriate next step for IVIG-resistant cases. This approach is proven to be effective in resolving fever and inflammation in a significant proportion of patients who did not respond to the first dose.

While corticosteroids are an alternative second-line therapy, and may be used first in high-risk patients, repeating the IVIG is a standard and validated escalation strategy.

WRONG ANSWER ANALYSIS:

Option A (Start broad-spectrum antibiotics) is incorrect because Kawasaki Disease is a vasculitis, not a bacterial infection, and the persistent fever is due to ongoing inflammation.

Option C (Start high-dose oral Aspirin only) is incorrect as high-dose aspirin is no longer standard UK practice, and low-dose aspirin alone is insufficient to control the severe inflammation of resistant disease.

Option D (Discharge home) is inappropriate as the child has persistent fever and active disease, placing him at high risk of developing coronary artery aneurysms.

Option E (Refer for coronary artery bypass) is incorrect as this is a surgical intervention for severe, established coronary artery disease, not a treatment for the acute inflammatory phase.

CORRECT ANSWER:

The infant's presentation of projectile vomiting and the blood gas and electrolyte results are classic for hypertrophic pyloric stenosis. This condition leads to a hypochloraemic, hypokalaemic metabolic alkalosis due to the loss of large volumes of gastric hydrochloric acid.

Following initial resuscitation with a 0.9% sodium chloride bolus to restore circulatory volume, the priority is to correct the underlying electrolyte and acid-base disturbance. The kidneys are unable to correct the alkalosis until the chloride and potassium deficits are replaced. Therefore, the appropriate ongoing fluid is 0.9% Sodium Chloride to correct the profound chloridaemia and volume deficit, with added Potassium Chloride to safely correct the significant hypokalaemia. This correction is essential pre-operatively to ensure the infant is safe for anaesthesia.

WRONG ANSWER ANALYSIS:

Option B (0.45% Sodium Chloride with Dextrose) is incorrect as this hypotonic solution would not provide sufficient chloride to correct the severe deficit and may contribute to hyponatraemia.

Option C (5% Dextrose with Sodium Bicarbonate) is incorrect because administering bicarbonate would dangerously worsen the already severe metabolic alkalosis.

Option D (Hartmann's Solution) is incorrect as it contains lactate, which is metabolised to bicarbonate in the liver, thereby exacerbating the alkalosis.

Option E (0.9% Sodium Chloride only) is incorrect because while it addresses the chloride deficit, it fails to correct the critical hypokalaemia, which carries a risk of cardiac arrhythmias.

CORRECT ANSWER:

The decision to administer live vaccines, such as the oral rotavirus vaccine, in infants with DiGeorge syndrome (22q11.2 deletion) depends on the degree of underlying T-cell immunodeficiency.

UK guidelines state that most patients can receive live vaccines safely, provided severe immunodeficiency has been excluded. This infant's CD4 count of 1200 cells/mm3 indicates only mild T-cell lymphopenia, which is not a contraindication.

The immune deficit in DiGeorge syndrome exists on a spectrum, and only those with severe T-cell defects (sometimes termed complete DiGeorge anomaly) are at risk from live vaccines. As this patient's cellular immunity is not severely compromised, the benefits of vaccination against rotavirus outweigh the risks.

Clinical practice guidelines often consider CD4 counts below 400 cells/mm3 to be a significant deficiency requiring avoidance of live vaccines. Therefore, proceeding with the standard immunisation schedule is the correct clinical decision.

WRONG ANSWER ANALYSIS:

Option B (No, it is contraindicated in all DiGeorge patients.) is incorrect because the contraindication is not absolute and is dependent on the severity of the individual's immunodeficiency.

Option C (Only if CD4 >1500.) is incorrect as there is no established national guideline with this specific high threshold, and a CD4 count of 1200 is considered sufficient for safe vaccination.

Option D (Only if the vaccine is inactivated.) is incorrect because the standard rotavirus vaccine is a live attenuated vaccine, and an inactivated formulation is not available in the routine UK schedule.

Option E (Only after 1 year of age.) is incorrect as the primary course of rotavirus vaccination must be completed by 24 weeks of age, and delaying offers no benefit in this context.

CORRECT ANSWER:

Adjunctive high-dose corticosteroids are a mandatory component of therapy for tuberculous meningitis. National guidelines recommend their use as they significantly reduce mortality and long-term neurological disability.

The inflammatory response to Mycobacterium tuberculosis within the central nervous system causes vasculitis, cerebral oedema, and raised intracranial pressure, leading to severe neurological sequelae. Dexamethasone works by suppressing this intense meningeal inflammation, thereby mitigating these pathological processes.

Evidence demonstrates that corticosteroids reduce the risk of death by approximately one quarter. While antituberculous chemotherapy is essential for eradicating the infection, the immediate priority to prevent irreversible neurological damage is to control the host inflammatory response. Therefore, starting dexamethasone concurrently with quadruple therapy is the standard of care in paediatric practice to improve overall outcomes.

WRONG ANSWER ANALYSIS:

Option A (Pyridoxine) is incorrect because it is routinely co-administered with isoniazid to prevent peripheral neuropathy, but it does not influence the primary neurological outcome of the meningitis itself.

Option C (Acyclovir) is incorrect as it is an antiviral agent used for herpes simplex encephalitis and has no role in the treatment of bacterial infections like tuberculosis.

Option D (Cotrimoxazole) is incorrect because it is an antibiotic used for prophylaxis against Pneumocystis jirovecii pneumonia, particularly in immunocompromised patients, not for treating tuberculous meningitis.

Option E (Phenytoin) is incorrect as it is an anti-epileptic medication that would only be indicated if the patient developed seizures, not as a prophylactic or primary treatment.

CORRECT ANSWER:

High-dose cytarabine is secreted into the tear film, causing a direct toxic effect on the rapidly dividing cells of the corneal and conjunctival epithelium. This results in a severe, painful chemical keratoconjunctivitis.

The clinical presentation of intensely painful, red, and watery eyes is characteristic of this well-documented toxicity. Standard UK chemotherapy protocols mandate the use of prophylactic topical steroid eye drops (e.g., dexamethasone) starting with cytarabine administration to mitigate this. Therefore, initiating or continuing steroid eye drops is the immediate and most appropriate management to control the profound inflammation and alleviate symptoms.

This intervention is a cornerstone of supportive care during high-dose cytarabine therapy and is typically managed by the paediatric oncology team.

WRONG ANSWER ANALYSIS:

Option A (Swab for viral PCR) is incorrect because the clinical picture is highly suggestive of a predictable chemotherapy side effect, not a primary viral infection.

Option B (Start IV Acyclovir) is incorrect as there are no features to suggest herpetic keratitis, and treating for a viral cause would delay appropriate management of the chemical injury.

Option D (Start chloramphenicol eye drops) is incorrect because this is an antibiotic for bacterial conjunctivitis and has no role in treating a sterile, chemical inflammation.

Option E (Urgent ophthalmology review) is less appropriate as the immediate first step, as this is an expected complication with established management that should be initiated without delay by the treating team.

CORRECT ANSWER:

This patient presents with the classic triad of fever, back pain, and haemoglobinuria, alongside haemodynamic instability (tachycardia and hypotension), pathognomonic for an Acute Haemolytic Transfusion Reaction (AHTR).

This is a medical emergency caused by ABO-incompatible blood leading to rapid intravascular haemolysis. UK guidelines, including those from the British Society for Haematology, state the absolute first step is to stop the transfusion immediately. Every additional millilitre of incompatible blood potentiates the catastrophic immune response, increasing the risk of disseminated intravascular coagulation (DIC), acute kidney injury, and death.

The priority is to halt the causative agent before initiating any other supportive measures. After stopping the transfusion, intravenous access must be maintained for fluid resuscitation and supportive care.

WRONG ANSWER ANALYSIS:

Option B (Administer IV Paracetamol) is incorrect because while it may address the fever, it does not treat the underlying life-threatening immunological reaction.

Option C (Administer IV Hydrocortisone) is incorrect as steroids may be used later to dampen the inflammatory response, but they are not the first-line, life-saving intervention.

Option D (Administer broad-spectrum IV antibiotics) is incorrect because although sepsis can present similarly, the classic triad makes AHTR the leading diagnosis, and stopping the transfusion is the immediate priority over treating a differential.

Option E (Slow the transfusion rate to 50%) is incorrect and dangerous because it allows the causative agent—the incompatible blood—to continue infusing, worsening the haemolysis.

CORRECT ANSWER:

This child meets the 2021 international consensus diagnostic criteria for Neurofibromatosis Type 1 (NF1) based on the presence of two key features: six or more cafe-au-lait macules and axillary freckling.

As there is no family history, this is likely a de novo mutation. The diagnosis is clinical, and the immediate priority shifts to screening for common, potentially serious complications. UK guidelines recommend annual surveillance by a paediatrician and an ophthalmologist for children with NF1.

Referral to ophthalmology is crucial to screen for Lisch nodules (iris hamartomas) and, more importantly, optic pathway gliomas, which occur in 15-20% of children with NF1 and can threaten vision. Early detection and monitoring are key components of managing the condition. Therefore, arranging an ophthalmology assessment is the most critical next step in this child's care pathway.

WRONG ANSWER ANALYSIS:

Option B (Reassure) is incorrect because the child's signs are highly indicative of a significant multi-system genetic disorder that requires lifelong monitoring and specialist input.

Option C (Request a full karyotype) is incorrect because NF1 is a single-gene disorder caused by a mutation in the NF1 gene on chromosome 17, not a chromosomal aneuploidy detectable by karyotyping.

Option D (Perform a whole-body MRI) is incorrect as it is not a first-line investigation for diagnosis; it is used later if there is clinical suspicion of internal neurofibromas or other complications.

Option E (Refer to dermatology for biopsy) is incorrect because cafe-au-lait macules are diagnosed clinically, and a biopsy is invasive and unnecessary to confirm an NF1 diagnosis.

CORRECT ANSWER:

This child is in septic shock with signs of peritonitis (rigid, tender abdomen with guarding), indicating probable bowel ischaemia or perforation. This is a life-threatening surgical emergency.

The immediate priority is aggressive resuscitation according to Advanced Paediatric Life Support (APLS) principles. This involves making the child nil by mouth (NBM), securing intravenous access for rapid fluid resuscitation to correct hypotension and tachycardia, and administering broad-spectrum intravenous antibiotics to manage sepsis.

Following initial stabilisation, an emergency laparotomy is mandatory to inspect the bowel, resect any non-viable tissue, and prevent further deterioration. Any delay for imaging or non-operative procedures in a haemodynamically unstable child with peritonitis would significantly increase morbidity and mortality.

WRONG ANSWER ANALYSIS:

Option A (Therapeutic air enema) is incorrect because it is absolutely contraindicated in the presence of shock or peritonitis due to the high risk of perforation.

Option B (Abdominal X-ray) is incorrect as it is not the definitive diagnostic investigation and would waste critical time in a patient requiring immediate resuscitation and surgery.

Option D (Abdominal ultrasound) is incorrect because although it is the primary imaging modality for diagnosis, performing it is inappropriate and unsafe in an unstable child with peritonitis who needs immediate surgical intervention.

Option E (IV morphine and observation) is incorrect because while analgesia is important, observation alone is wholly inadequate for a child in shock with a surgical abdomen.

CORRECT ANSWER:

According to NICE and other UK-based guidelines, a time-limited course of oral corticosteroids is the recommended first-line therapy to induce remission in children and young people with moderate-to-severe ulcerative colitis.

In this case of moderate pancolitis, the inflammation is extensive and active enough to warrant systemic steroid therapy to gain rapid control of the disease. The Paediatric Ulcerative Colitis Activity Index (PUCAI) is used to classify severity, with moderate disease scoring 35-64.

While aminosalicylates (5-ASA) are used for mild disease, moderate colitis requires a more potent anti-inflammatory agent for induction. The clinical priority is to achieve rapid symptom control and mucosal healing, which oral prednisolone effectively provides, before stepping down to maintenance therapy.

WRONG ANSWER ANALYSIS:

Option A (Exclusive Enteral Nutrition) is incorrect as it is a first-line induction therapy for Crohn's disease, not ulcerative colitis.

Option C (Oral 5-ASA monotherapy) is incorrect because it is indicated for inducing remission in mild disease or for maintaining remission, but lacks sufficient efficacy for moderate pancolitis.

Option D (Infliximab) is incorrect as this biologic therapy is reserved for severe colitis or as a second-line treatment if corticosteroids are ineffective or contraindicated.

Option E (Azathioprine) is incorrect because this immunomodulator is used for maintaining remission and is not recommended for induction due to its slow onset of action.

CORRECT ANSWER:

The child's welfare is paramount, a core principle of UK safeguarding legislation. The presence of domestic violence is a significant risk factor for both physical and emotional harm to a child, and this information is critical context for any child protection assessment.

As per GMC guidance and the 'Working Together to Safeguard Children' framework, while a clinician has a duty of confidentiality to the mother, this is overridden by the duty to protect a child from risk of significant harm. The most appropriate action is to be open and honest with the mother, explaining that this information must be shared with children's social care as part of the safeguarding referral. This maintains a level of trust while fulfilling your professional and legal obligation to the child. It is an act of informing, not seeking permission.

WRONG ANSWER ANALYSIS:

Option A (Document it but omit it from the safeguarding referral to protect the mother) is incorrect because withholding crucial risk information fundamentally undermines the purpose of a safeguarding referral and places the child at ongoing risk.

Option C (Advise the mother to report the abuse to the police herself) is incorrect because it inappropriately transfers the professional's safeguarding responsibility to the victim, delaying protective action.

Option D (Maintain the mother's confidentiality completely) is incorrect because the duty to protect a child from significant harm overrides the duty of confidentiality to an adult in this context.

Option E (Only share the information with the named safeguarding lead) is incorrect because while the safeguarding lead must be informed, the information must also be shared directly with social services to ensure a timely and comprehensive assessment.

CORRECT ANSWER:

General Medical Council (GMC) guidance is unequivocal in this situation. While a doctor's primary duty is to their patient, this is superseded by a wider duty to protect the public from a significant risk of death or serious harm.

The patient has been counselled but continues to drive, posing a direct danger. The correct and mandatory action, after attempting to persuade the patient to self-report, is to inform the Driver and Vehicle Licensing Agency (DVLA) directly. This is a justified breach of confidentiality in the public interest.

The clinician should inform the patient of their intention to disclose this information to the DVLA. This decision prioritises public safety over the individual's autonomy when a clear and present danger has been identified.

WRONG ANSWER ANALYSIS:

Option A (Maintain confidentiality as he has capacity) is incorrect because GMC guidance explicitly states that the duty to protect the public from serious harm can override the duty of confidentiality.

Option B (Tell his parents, so they can stop him) is incorrect as the patient is 17 and presumed to have capacity, making a disclosure to his parents an inappropriate breach of confidentiality.

Option D (Report him to the police for dangerous driving) is incorrect because the DVLA is the appropriate statutory body to deal with medical fitness to drive, not the police.

Option E (Document his refusal and take no further action) is incorrect as this would be a failure in the doctor's duty of care to the wider public, knowingly allowing a significant risk to persist.

CORRECT ANSWER:

The clinical history is pathognomonic for Prader-Willi Syndrome (PWS). This genetic disorder classically presents with a biphasic nutritional history.

The initial phase, from birth into infancy, is characterised by profound hypotonia, a poor suck, and feeding difficulties, often leading to failure to thrive and requiring nasogastric tube feeding. This is followed by a switch in early childhood (typically between 1-6 years) to the second phase, defined by hyperphagia—an insatiable, uncontrolled appetite—leading to progressive, severe obesity.

The co-existence of short stature is another key feature, as growth hormone deficiency is a common endocrinopathy in PWS. This distinct progression from infantile hypotonia and poor feeding to childhood hyperphagia and obesity is the cornerstone of a clinical diagnosis, prompting genetic testing for confirmation.

WRONG ANSWER ANALYSIS:

Option A (Growth Hormone Deficiency) is incorrect as isolated GHD does not explain the profound neonatal hypotonia, feeding issues, or the characteristic hyperphagia seen in this child.

Option B (Cushing's Syndrome) is incorrect because while it causes obesity, it is not associated with infantile hypotonia and typically presents with growth failure, not a preceding history of failure to thrive.

Option D (Simple obesity) is incorrect because it does not account for the significant hypotonia and feeding difficulties in infancy, which are hallmarks of a syndromic cause.

Option E (Beckwith-Wiedemann Syndrome) is incorrect as it is an overgrowth syndrome, meaning infants typically present with macrosomia (large size) at birth, not hypotonia and failure to thrive.

CORRECT ANSWER:

This patient presents with a hypercalcaemic crisis, evidenced by severe hypercalcaemia (adjusted Ca2+ 3.7 mmol/L) and signs of dehydration and end-organ effects including confusion.

The high calcium level impairs the kidney's ability to concentrate urine, causing a nephrogenic diabetes insipidus, which leads to significant polyuria, dehydration, and a reduced glomerular filtration rate (GFR). According to UK paediatric guidelines, the immediate priority is aggressive intravenous fluid resuscitation with isotonic saline (0.9% sodium chloride).

This rapidly expands intravascular volume, restores renal perfusion, improves GFR, and promotes calciuresis by increasing urinary calcium excretion. Addressing the severe dehydration is the critical first step before any other specific therapies are considered.

WRONG ANSWER ANALYSIS:

Option A (IV Furosemide) is incorrect because loop diuretics should only be considered after adequate rehydration, as their use in a dehydrated patient can worsen volume depletion and renal impairment, paradoxically exacerbating the hypercalcaemia.

Option C (IV Pamidronate) is incorrect as a first-line measure because bisphosphonates, while effective at lowering calcium by inhibiting bone resorption, have a slow onset of action (24-48 hours) and do not address the immediate life-threatening issue of dehydration.

Option D (IV Hydrocortisone) is incorrect as it is only effective for specific causes of hypercalcaemia, such as vitamin D toxicity or certain malignancies, and is not the priority for initial stabilisation.

Option E (Urgent haemodialysis) is incorrect because it is reserved for life-threatening, refractory hypercalcaemia with severe renal impairment and is not the initial management step.

CORRECT ANSWER:

This child presents with classic features of warm, distributive septic shock. The presence of fever and altered mental state (confusion) in a tachycardic child should immediately raise suspicion of sepsis.

The key haemodynamic findings are a wide pulse pressure (105/40 mmHg), bounding pulses, and a flash capillary refill time (<1 second). These signs indicate a high cardiac output state with profound peripheral vasodilation, leading to low systemic vascular resistance. The confusion signifies cerebral hypoperfusion, a critical sign of organ dysfunction. According to RCPCH and NICE guidelines, prompt recognition is vital, requiring immediate intervention with intravenous fluids and broad-spectrum antibiotics to prevent progression to cold shock and multi-organ failure. This clinical scenario demands urgent action based on the clear pattern of high-output septic shock. WRONG ANSWER ANALYSIS:

Option A (Cardiogenic shock) would present with signs of primary cardiac failure and low output, such as cool, clammy skin, weak pulses, and pulmonary oedema.

Option B (Compensated hypovolaemic shock) is incorrect because it is characterised by peripheral vasoconstriction, leading to cool peripheries and a prolonged capillary refill time.

Option C (Anaphylactic shock) is less likely as the history lacks a typical allergen trigger, urticaria, angio-oedema, or wheeze.

Option E (Neurogenic shock) is incorrect as it typically follows a significant spinal cord injury and presents with hypotension and paradoxical bradycardia, not tachycardia.

CORRECT ANSWER:

The bronchoscopy findings of mucosal ulceration and soot confirm a significant smoke inhalation injury, which is a form of chemical pneumonitis. This insult triggers a profound inflammatory response within the distal airways and alveoli, leading to increased capillary permeability, exudation of protein-rich fluid into the alveolar spaces, and surfactant inactivation.

This process is the hallmark of Acute Respiratory Distress Syndrome (ARDS). Importantly, the clinical and radiological signs of ARDS are often delayed, typically manifesting 12 to 48 hours after the initial injury. Therefore, a normal initial chest X-ray is common and should not provide false reassurance. Management priorities are guided by anticipation of this deterioration, necessitating early intubation for airway protection and supportive ventilation on a paediatric intensive care unit.

WRONG ANSWER ANALYSIS:

Option B (Pneumothorax) is incorrect as there is no evidence of barotrauma from ventilation or direct thoracic injury, and the admission chest X-ray was normal.

Option C (Pulmonary embolism) is incorrect because the patient has no immediate risk factors for thromboembolism, and the underlying pathophysiology is acute lung inflammation, not vascular obstruction.

Option D (Cardiogenic shock) is incorrect as the primary insult is to the respiratory system; while shock may occur later, it would likely be distributive or secondary to hypoxia, not primary cardiac failure.

Option E (Oesophageal perforation) is incorrect because the mechanism of injury is inhalation affecting the tracheobronchial tree, not the gastrointestinal tract.

CORRECT ANSWER:

This child has severe diabetic ketoacidosis (DKA) but is not in circulatory shock, as evidenced by the normal blood pressure and capillary refill time. National guidelines (NICE/BSPED) state that the priority is gradual correction of dehydration and hyperosmolality to minimise the risk of cerebral oedema.

The reduced GCS is due to the severe metabolic derangement, not necessarily established cerebral oedema. Therefore, the correct first step is to calculate the fluid deficit and maintenance requirements, and start a slow intravenous infusion of 0.9% sodium chloride over 48 hours. Insulin infusion should only be commenced at least one hour after starting fluid therapy to prevent rapid osmotic shifts that can precipitate cerebral oedema.

WRONG ANSWER ANALYSIS:

Option A (Administer IV Mannitol for cerebral oedema) is inappropriate as Mannitol is a treatment for clinically apparent cerebral oedema, which is not yet diagnosed, and it is not used prophylactically.

Option B (Administer a 10 ml/kg 0.9% saline bolus) is incorrect because fluid boluses are reserved for children with DKA who are in shock, and giving one unnecessarily increases the risk of cerebral oedema.

Option C (Start IV insulin infusion immediately) is incorrect as commencing insulin without prior fluid resuscitation can worsen dehydration and hypokalaemia, and increase the risk of cerebral oedema.

Option E (Administer IV Sodium Bicarbonate) is incorrect because its use is not recommended in DKA management as it can cause paradoxical CNS acidosis and is associated with an increased risk of cerebral oedema.

CORRECT ANSWER:

The crucial investigation in a non-diabetic child with profound hypoglycaemia is the 'critical sample', taken at the time of the low blood glucose.

The key to this diagnosis is the relationship between insulin and C-peptide. The body co-secretes pro-insulin, which is cleaved into insulin and C-peptide in a 1:1 molar ratio. Therefore, any cause of endogenous hyper-insulinaemia will result in high levels of both.

In this case, the serum insulin is very high (80 mU/L) while the C-peptide is suppressed (<0.1 nmol/L). This discordance proves the insulin is from an external, or exogenous, source. Given the history of a diabetic relative visiting, accidental administration of injectable insulin is the most likely diagnosis. This is a medical and safeguarding emergency. WRONG ANSWER ANALYSIS:

Option B (Sulfonylurea ingestion) is incorrect because sulfonylureas stimulate the pancreatic beta-cells to secrete more insulin, which would result in high levels of both insulin and C-peptide.

Option C (Congenital Hyperinsulinism) is incorrect as this is a cause of endogenous insulin secretion, which would also lead to elevated insulin and C-peptide levels.

Option D (An insulinoma) is incorrect because this rare insulin-secreting tumour would cause a concordant rise in both insulin and C-peptide.

Option E (Sepsis) is incorrect as hypoglycaemia in sepsis is typically associated with low or suppressed insulin levels, not profound hyper-insulinaemia.

CORRECT ANSWER:

Diazoxide is a first-line therapy for many forms of congenital hyperinsulinism (CHI), but a major side effect is fluid retention. This occurs due to its effect on renal tubular sodium and water handling, which can lead to peripheral oedema, pulmonary hypertension, and cardiac failure.

National consensus and standard paediatric endocrine practice recommend the co-administration of a thiazide diuretic, like chlorothiazide, to counteract this specific complication. Chlorothiazide is considered the first-line diuretic in this context, often started simultaneously with diazoxide to prevent fluid overload. It also has a mild synergistic hyperglycaemic effect. The development of respiratory distress alongside oedema indicates significant fluid overload, making the immediate addition of chlorothiazide the most critical next step in management.

WRONG ANSWER ANALYSIS:

Option A (Oral Furosemide) is incorrect because loop diuretics are considered second-line to thiazides for diazoxide-induced fluid retention and can cause significant electrolyte disturbance.

Option B (Oral Hydrochlorothiazide) is incorrect as it is not the preferred thiazide diuretic in this scenario.

Option C (Oral Spironolactone) is incorrect as it is a potassium-sparing diuretic that is less effective for this specific mechanism of fluid retention and is not the recommended first-line agent.

Option D (Oral Captopril) is incorrect because, as an ACE inhibitor, it is used for hypertension or heart failure, not for managing diuretic-resistant fluid overload caused by diazoxide.

Option E (Oral Sildenafil) is incorrect as it is a pulmonary vasodilator used to treat pulmonary hypertension itself, but it does not address the underlying cause, which is fluid retention.

CORRECT ANSWER:

In managing atopic eczema in children, once optimal topical therapy is established, persistent and severe nocturnal itch causing sleep disturbance warrants further intervention.

National Institute for Health and Care Excellence (NICE) guidelines suggest a short-term trial (typically 2 to 4 weeks) of a first-generation sedating antihistamine for children over 6 months of age with severe eczema and significant sleep disruption. The primary therapeutic benefit is derived from its sedative properties, which helps to break the nocturnal itch-scratch cycle, thereby improving sleep quality for the child and family.

It is crucial to understand that atopic eczema is not a primarily histamine-driven condition, so the antihistaminergic action is of little benefit; the goal is sedation. This is a pragmatic step to manage a distressing symptom while continuing comprehensive topical management.

WRONG ANSWER ANALYSIS:

Option B (A non-sedating antihistamine) is incorrect as these agents lack the sedative effect required to break the sleep-itch cycle and are not recommended for managing eczema.

Option C (Oral Montelukast) is incorrect because it is a leukotriene receptor antagonist used for asthma and has no established role in the management of atopic itch.

Option D (Long-term oral antibiotics) is inappropriate as antibiotics are reserved for treating active secondary bacterial infections, not for managing pruritus.

Option E (Oral Melatonin) is less appropriate because while it may induce sleep, it does not directly address the underlying sensation of itch, which is the primary cause of sleep disturbance in this case.

CORRECT ANSWER:

This infant presents with features highly suggestive of incomplete Kawasaki Disease (KD). According to RCPCH and NICE guidance, KD should be suspected in any child with a prolonged fever, particularly an infant under one year old.

The combination of fever for over five days, extreme inflammatory marker elevation (CRP >100), low albumin, and sterile pyuria strongly supports this diagnosis, even without the classic criteria. The most critical next step is an urgent echocardiogram to assess for coronary artery abnormalities. Early detection of aneurysms is paramount as it dictates immediate treatment with intravenous immunoglobulin (IVIG) and aspirin to prevent long-term cardiac sequelae such as thrombosis and myocardial infarction. Delaying the echocardiogram to pursue other investigations would be inappropriate given the high index of suspicion and the time-critical nature of treatment.

WRONG ANSWER ANALYSIS:

Option B (Start empirical antibiotics for UTI) is incorrect because the presence of sterile pyuria is a recognised feature of the systemic inflammation in Kawasaki Disease, not a bacterial urinary tract infection.

Option C (Reassure and give antipyretics) is inappropriate as the prolonged fever and significantly raised inflammatory markers clearly indicate a serious underlying pathology requiring urgent investigation, not simple observation.

Option D (Perform lumbar puncture) is less appropriate because while irritability can suggest meningitis, the overall clinical and laboratory picture is more specific for Kawasaki Disease, making the echocardiogram the higher priority investigation.

Option E (Start oral prednisolone) is incorrect as steroids are only considered as adjunctive therapy in specific circumstances for KD, and primary treatment with IVIG should be initiated first once the diagnosis is supported by echocardiography.

CORRECT ANSWER:

In a patient with Long QT Syndrome (LQTS), the immediate priority is to mitigate the risk of Torsades de Pointes (TdP), a life-threatening ventricular arrhythmia.

Both hypokalaemia and hypomagnesaemia are independent risk factors for QT interval prolongation and can precipitate TdP, especially in a patient with a baseline prolonged QTc. This patient's QTc of 510 ms is significantly prolonged, and the combination of low potassium and magnesium creates a high-risk electrical substrate in the myocardium.

Therefore, the most critical and urgent management step is to correct these electrolyte abnormalities. National guidelines emphasise that stabilising the cardiac membrane by normalising electrolytes is the cornerstone of immediate management to prevent arrhythmia. Repletion of magnesium is also crucial as it is required for the proper function of renal potassium channels, and correcting hypomagnesaemia is often necessary to successfully correct concurrent hypokalaemia.

WRONG ANSWER ANALYSIS:

Option B (Increase propranolol dose) is incorrect because while beta-blockers are the mainstay of chronic LQTS therapy, increasing the dose does not address the acute arrhythmogenic risk from severe electrolyte derangement.

Option C (Add IV magnesium sulfate) is incorrect because it is only one component of the necessary electrolyte correction; the significant hypokalaemia must also be addressed urgently, making overall electrolyte correction the most comprehensive answer.

Option D (Start oral Flecainide) is incorrect as flecainide is a sodium channel blocker that can further prolong the QT interval in some LQTS subtypes and is not a first-line therapy.

Option E (Arrange for ICD insertion) is incorrect because an Implantable Cardioverter-Defibrillator (ICD) is a consideration for long-term secondary prevention after stabilisation, not an immediate intervention for an acute metabolic trigger.

CORRECT ANSWER:

According to NICE guidance, drug treatment for ADHD should only be initiated by a specialist. For prescribing responsibility to be transferred to a GP, a formal Shared Care Agreement (SCA) must be in place.

Methylphenidate is typically classified as an "amber" drug, meaning it requires specialist initiation and ongoing monitoring under a shared care arrangement. The SCA is a crucial medicolegal document that explicitly outlines the responsibilities of the specialist, the GP, and the patient/family.

It ensures clear communication protocols and defines roles for prescribing, monitoring physical health parameters (like growth, heart rate, and blood pressure), and managing any adverse effects. This structured approach is essential for the safe and effective long-term management of this controlled medication in primary care.

WRONG ANSWER ANALYSIS:

Option A (A transfer of care letter to the GP) is incorrect because a simple letter lacks the formal, agreed-upon structure and specific responsibilities detailed in an SCA.

Option B (A 3-way conversation with the parents) is incorrect as, while good practice for communication, it is not a substitute for the formal, documented agreement required for safe governance.

Option D (A referral to the adult ADHD team) is incorrect because the patient is 10 years old and remains under the care of paediatric services until transition planning begins much later.

Option E (A copy of the boy's EHC plan) is incorrect because an Education, Health and Care plan addresses educational and social needs and is not the clinical governance document for medication prescribing.

CORRECT ANSWER:

The priority in managing a patient at high risk of refeeding syndrome is to initiate nutritional rehabilitation cautiously to prevent severe electrolyte and metabolic disturbances.

Junior MARSIPAN guidelines recommend a starting caloric intake of 20 kcal/kg/day for most medically unstable patients with anorexia nervosa. This approach provides approximately 800 kcal for this 40kg patient, balancing the need to start reversing the catabolic state without overwhelming the body's depleted metabolic capacity.

A sudden carbohydrate load can precipitate dangerous shifts in phosphate, potassium, and magnesium, leading to cardiac, respiratory, and neurological complications. This starting point allows for gradual metabolic adaptation, with slow increases of around 200-300 kcal every few days, guided by close biochemical monitoring.

WRONG ANSWER ANALYSIS:

Option A (5-10 kcal/kg/day) is incorrect as this is overly cautious and typically reserved for patients at extreme risk, which may lead to counterproductive underfeeding.

Option C (40-50 kcal/kg/day) is incorrect because it represents an aggressive starting intake that significantly increases the risk of precipitating severe refeeding syndrome.

Option D (60-70 kcal/kg/day) is incorrect as this level of intake is dangerously high for initial refeeding and would almost certainly cause severe metabolic derangement.

Option E (100% of her estimated target weight requirement) is incorrect because starting at full target requirements would overwhelm the patient's metabolic system, posing a very high risk of life-threatening refeeding complications.