A 10-year-old girl with type 1 diabetes for 3 years attends for annual screening. She is asymptomatic and examination is unremarkable. Screening blood tests show: HbA1c 56 mmol/mol (7.3%), serum creatinine 48 µmol/L, total cholesterol 4.2 mmol/L. Two consecutive early morning urine samples show albumin:creatinine ratios of 3.2 mg/mmol and 3.5 mg/mmol (normal <3.0 mg/mmol). Blood pressure is 108/68 mmHg (75th centile for age, sex and height). What is the most appropriate management?
Q72
A 7-year-old girl with epilepsy has been taking lamotrigine monotherapy for focal seizures with good control for 2 years. She now presents with a 3-day history of widespread erythematous rash involving her trunk and limbs, fever of 38.5°C, and oral mucosal ulceration. She was started on co-trimoxazole for a urinary tract infection 5 days ago. Examination reveals tender bilateral cervical lymphadenopathy and facial oedema. Blood tests show: WBC 12.5 × 10⁹/L with 15% eosinophils, ALT 180 IU/L, AST 165 IU/L. What is the most appropriate immediate management?
Q73
A 13-year-old boy with type 1 diabetes for 5 years presents to clinic for routine review. His recent HbA1c is 68 mmol/mol (8.4%), which is higher than his previous values of 55-58 mmol/mol. His blood glucose diary shows unexplained hyperglycaemia in the late afternoon and evening despite apparently good adherence to his insulin regimen. He is Tanner stage 4 puberty. His weight is on the 50th centile and has remained stable. What is the most likely explanation for the deteriorating glycaemic control?
Q74
An 8-year-old boy with focal epilepsy has been taking carbamazepine for 18 months with complete seizure control. His mother brings him to clinic concerned about behavioral changes. Over the past 3 months, his teacher has reported poor concentration, irritability, and deteriorating academic performance. He has also had two unexplained falls without loss of consciousness. His carbamazepine level is within the therapeutic range. What is the most likely explanation for these symptoms?
Q75
A 12-year-old girl with type 1 diabetes for 4 years is being reviewed in clinic. She uses an insulin pump (continuous subcutaneous insulin infusion) with good diabetes control. Her HbA1c is 52 mmol/mol (6.9%). She is going on a school sports trip involving 3 hours of moderate-intensity hiking daily for 5 days. She asks for advice about managing her diabetes during this activity. What is the most appropriate strategy to prevent hypoglycaemia during these activities?
Q76
A 5-year-old girl is brought to the paediatric neurology clinic following three witnessed episodes over 2 months. During these episodes, she suddenly stops playing, stares blankly for approximately 8-10 seconds with subtle eye fluttering, then immediately resumes her previous activity with no post-ictal confusion. Her development is normal and there are no abnormal findings on neurological examination. Her EEG shows 3 Hz spike-and-wave discharges during hyperventilation. What is the most important prognostic factor regarding seizure remission in this condition?
Q77
Which biochemical feature is the defining diagnostic criterion that distinguishes diabetic ketoacidosis from hyperosmolar hyperglycaemic state in children with type 1 diabetes mellitus?
Q78
A 14-year-old boy with type 1 diabetes mellitus for 6 years attends the emergency department with a 2-day history of vomiting and abdominal pain. He has continued his insulin but has been unable to eat. Observations: temperature 37.8°C, heart rate 115 bpm, blood pressure 105/65 mmHg, respiratory rate 28/min with deep sighing respiration, capillary refill time 3 seconds. Capillary blood glucose is 18.2 mmol/L. Urinalysis shows 3+ ketones and 2+ glucose. What is the single most important immediate investigation to guide management?
Q79
A 9-year-old boy with epilepsy has been taking sodium valproate 600 mg twice daily for juvenile myoclonic epilepsy for the past 3 years with good seizure control. At his routine clinic appointment, his weight has increased from the 75th to the 98th centile over the past year, and examination reveals mild tremor of his hands. Blood tests show: serum sodium 139 mmol/L, potassium 4.2 mmol/L, ALT 58 IU/L (normal 10-40), AST 52 IU/L (normal 10-40), ammonia 65 µmol/L (normal 11-32). What is the most appropriate next step in management?
Q80
A 6-year-old girl with type 1 diabetes mellitus attends the paediatric diabetes clinic for routine follow-up. She was diagnosed 18 months ago and uses a basal-bolus insulin regimen. Her mother reports that she has been experiencing episodes of sweating, tremor, and irritability before breakfast on several occasions over the past 2 weeks. Blood glucose monitoring shows readings of 3.4 mmol/L and 3.6 mmol/L at these times. What is the most appropriate initial management step?
Chronic Paediatric Conditions UK Medical PG Practice Questions and MCQs
Question 71: A 10-year-old girl with type 1 diabetes for 3 years attends for annual screening. She is asymptomatic and examination is unremarkable. Screening blood tests show: HbA1c 56 mmol/mol (7.3%), serum creatinine 48 µmol/L, total cholesterol 4.2 mmol/L. Two consecutive early morning urine samples show albumin:creatinine ratios of 3.2 mg/mmol and 3.5 mg/mmol (normal <3.0 mg/mmol). Blood pressure is 108/68 mmHg (75th centile for age, sex and height). What is the most appropriate management?
A. Commence ACE inhibitor therapy to prevent progression of nephropathy
B. Repeat albumin:creatinine ratio in 3-6 months and review glycaemic control (Correct Answer)
C. Arrange urgent paediatric nephrology referral for renal biopsy
D. Commence statin therapy to reduce cardiovascular risk
E. Increase frequency of HbA1c monitoring to every 2 months
Explanation: ***Repeat albumin:creatinine ratio in 3-6 months and review glycaemic control***
- The presence of two elevated **albumin:creatinine ratio (ACR)** samples suggests **microalbuminuria**, an early sign of **diabetic nephropathy**. However, in children, microalbuminuria can be **transient** due to various factors like illness or exercise, and requires confirmation with further testing.
- Her **HbA1c** of 7.3% indicates suboptimal **glycaemic control** (target typically <6.5% for children with type 1 diabetes). Optimizing glucose control is the cornerstone of preventing progression of diabetic complications, including nephropathy, and should be pursued before considering pharmacotherapy.
*Commence ACE inhibitor therapy to prevent progression of nephropathy*
- **ACE inhibitors** are typically considered for **persistent microalbuminuria** (defined as three out of three positive samples over 3-6 months) or established hypertension, which is not the case here given her normal blood pressure and only two positive ACRs.
- Initiating an ACE inhibitor in a 10-year-old with only two samples suggesting microalbuminuria, without optimizing **glycaemic control** first, is premature.
*Arrange urgent paediatric nephrology referral for renal biopsy*
- **Renal biopsy** is an invasive procedure and is generally reserved for cases of **atypical kidney disease** presentation, such as rapidly declining **GFR**, significant **haematuria**, or proteinuria that is severe or unresponsive to standard management, which are not present here.
- In a patient with established **type 1 diabetes** and early microalbuminuria, the findings are consistent with the natural progression of **diabetic kidney disease**, making a biopsy unnecessary at this stage.
*Commence statin therapy to reduce cardiovascular risk*
- This patient's **total cholesterol** level of 4.2 mmol/L is within an acceptable range for a child and does not warrant **statin therapy** at this age. Statin use in children is generally reserved for severe dyslipidaemia or specific genetic conditions.
- While **cardiovascular risk** is a concern in type 1 diabetes, the primary intervention at this stage is **glycaemic control** and blood pressure management, not lipid-lowering medication.
*Increase frequency of HbA1c monitoring to every 2 months*
- Standard monitoring for **HbA1c** in type 1 diabetes is typically every 3 months, which provides sufficient data to assess **glycaemic control** and adjust treatment plans effectively.
- While the HbA1c is elevated, simply increasing the **frequency of monitoring** without implementing changes to the **diabetes management plan** (e.g., insulin regimen, diet, exercise) will not improve her glycaemic control or address the emerging microalbuminuria.
Question 72: A 7-year-old girl with epilepsy has been taking lamotrigine monotherapy for focal seizures with good control for 2 years. She now presents with a 3-day history of widespread erythematous rash involving her trunk and limbs, fever of 38.5°C, and oral mucosal ulceration. She was started on co-trimoxazole for a urinary tract infection 5 days ago. Examination reveals tender bilateral cervical lymphadenopathy and facial oedema. Blood tests show: WBC 12.5 × 10⁹/L with 15% eosinophils, ALT 180 IU/L, AST 165 IU/L. What is the most appropriate immediate management?
A. Discontinue lamotrigine immediately and admit for monitoring and supportive care (Correct Answer)
B. Continue lamotrigine but discontinue co-trimoxazole and prescribe oral prednisolone
C. Reduce lamotrigine dose by 50% and start oral antihistamines for the rash
D. Discontinue both medications and commence alternative antiepileptic drug immediately
E. Continue both medications and start intravenous immunoglobulin therapy
Explanation: ***Discontinue lamotrigine immediately and admit for monitoring and supportive care***- The patient's presentation with **widespread erythematous rash**, **fever**, **oral mucosal ulceration**, **lymphadenopathy**, **facial oedema**, **eosinophilia** (15%), and **elevated liver enzymes** is highly suggestive of **DRESS syndrome** (Drug Reaction with Eosinophilia and Systemic Symptoms).
- **Lamotrigine** is a well-known cause of DRESS syndrome, and immediate cessation of the suspected offending drug is the most critical initial step to prevent progression to multi-organ failure and reduce mortality. Admission is essential for comprehensive monitoring and supportive care.
*Continue lamotrigine but discontinue co-trimoxazole and prescribe oral prednisolone*
- Continuing **lamotrigine** is highly dangerous as it is a potent trigger for **severe cutaneous adverse reactions** (SCARs) like DRESS syndrome; its continued administration could worsen the life-threatening systemic reaction.
- While co-trimoxazole can also cause DRESS, the long-term use of lamotrigine and its known interaction with co-trimoxazole (which can increase lamotrigine levels) makes lamotrigine a strong suspect. Discontinuing only co-trimoxazole while keeping the primary culprit would be insufficient.
*Reduce lamotrigine dose by 50% and start oral antihistamines for the rash*
- A **dose reduction** of the offending drug is inadequate for managing a severe hypersensitivity reaction like DRESS; complete and immediate withdrawal is necessary to halt the immune-mediated damage.
- **Oral antihistamines** only provide symptomatic relief for pruritus and do not address the underlying systemic inflammation, organ involvement, or the progression of the DRESS syndrome.
*Discontinue both medications and commence alternative antiepileptic drug immediately*
- While discontinuing both suspected drugs is appropriate, immediately initiating a new **antiepileptic drug** during an acute DRESS episode carries a significant risk of inducing a cross-reactive hypersensitivity reaction or complicating the clinical picture.
- The immediate priority is the stabilization of the patient and management of the DRESS syndrome; alternative seizure control strategies should be considered cautiously and tailored after the acute reaction is under control.
*Continue both medications and start intravenous immunoglobulin therapy*
- Continuing **lamotrigine** and **co-trimoxazole** in the presence of DRESS syndrome is contraindicated as it perpetuates the immune response and organ damage.
- While **intravenous immunoglobulin (IVIG)** can be used in some severe SCARs like toxic epidermal necrolysis, it is not established as a primary or sole treatment for DRESS syndrome, and it does not replace the crucial need to withdraw the offending medication.
Question 73: A 13-year-old boy with type 1 diabetes for 5 years presents to clinic for routine review. His recent HbA1c is 68 mmol/mol (8.4%), which is higher than his previous values of 55-58 mmol/mol. His blood glucose diary shows unexplained hyperglycaemia in the late afternoon and evening despite apparently good adherence to his insulin regimen. He is Tanner stage 4 puberty. His weight is on the 50th centile and has remained stable. What is the most likely explanation for the deteriorating glycaemic control?
A. Insulin omission or reduction to avoid weight gain
B. Development of insulin antibodies reducing insulin efficacy
C. Physiological insulin resistance associated with puberty (Correct Answer)
D. Lipohypertrophy at injection sites reducing insulin absorption
E. Undiagnosed coeliac disease affecting carbohydrate absorption
Explanation: ***Physiological insulin resistance associated with puberty***
- During **mid-to-late puberty (Tanner stages 3-4)**, increased secretion of **growth hormone** and **sex steroids** naturally induces **insulin resistance**.
- This physiological change explains the patient's **deteriorating glycemic control** (higher HbA1c, unexplained late afternoon/evening hyperglycemia) despite good adherence, often necessitating a **20-50% increase in insulin dosage**.
*Insulin omission or reduction to avoid weight gain*
- While **insulin omission (diabulimia)** is a concern in adolescents with type 1 diabetes, the patient's **stable weight on the 50th centile** makes this explanation less likely.
- Deliberate insulin reduction often leads to more significant and variable **hyperglycemia** or recurrent **diabetic ketoacidosis**, not just a gradual rise in HbA1c.
*Development of insulin antibodies reducing insulin efficacy*
- Clinically significant **insulin antibodies** are exceedingly rare with modern **recombinant human insulin analogues** and typically manifest as very severe and erratic insulin resistance.
- The presented scenario of a gradual, explainable deterioration during a specific physiological stage (puberty) does not align with the characteristics of insulin antibody-mediated resistance.
*Lipohypertrophy at injection sites reducing insulin absorption*
- **Lipohypertrophy** can indeed cause erratic and unpredictable insulin absorption, leading to **hyperglycemia** or **hypoglycemia**.
- However, the patient's specific age and **Tanner stage 4** point more strongly towards a systemic physiological cause rather than a localized injection site issue.
*Undiagnosed coeliac disease affecting carbohydrate absorption*
- **Coeliac disease** is a common comorbidity with Type 1 Diabetes, but it typically causes **malabsorption**, leading to **unexplained hypoglycemia** or *improved* glycemic control if carbohydrate intake is poorly absorbed.
- Furthermore, **weight loss** or **failure to thrive** would be expected, which contradicts this patient's **stable weight on the 50th centile** and *worsening hyperglycemia*.
Question 74: An 8-year-old boy with focal epilepsy has been taking carbamazepine for 18 months with complete seizure control. His mother brings him to clinic concerned about behavioral changes. Over the past 3 months, his teacher has reported poor concentration, irritability, and deteriorating academic performance. He has also had two unexplained falls without loss of consciousness. His carbamazepine level is within the therapeutic range. What is the most likely explanation for these symptoms?
A. Subclinical seizure activity despite therapeutic drug levels
B. Carbamazepine-induced hyponatraemia affecting cognitive function (Correct Answer)
C. Development of attention deficit hyperactivity disorder
D. Behavioral side effects of carbamazepine
E. Progression of underlying neurological condition
Explanation: ***Carbamazepine-induced hyponatraemia affecting cognitive function***
- **Carbamazepine** can cause **SIADH**, leading to **hyponatraemia** which presents insidiously with **irritability**, poor concentration, and **deteriorating academic performance**.
- The unexplained falls may indicate **ataxia** or gait instability resulting from low sodium levels rather than seizure activity.
*Subclinical seizure activity despite therapeutic drug levels*
- While possible, the history of **complete seizure control** for 18 months makes new subclinical activity less likely than a metabolic side effect.
- **Subclinical seizures** typically wouldn't explain the acute onset of **unexplained falls** as the primary motor symptom in this context.
*Development of attention deficit hyperactivity disorder*
- **ADHD** usually presents with long-standing patterns of inattention and does not explain the **physical falls** described.
- The sudden decline in academic performance and behavior after 18 months of stability suggests an **acquired medical cause** rather than a primary psychiatric disorder.
*Behavioral side effects of carbamazepine*
- While **carbamazepine** can cause irritability, these effects usually appear near the **initiation of therapy** rather than after 18 months of good tolerance.
- Behavioral side effects alone do not account for the **neurological signs** such as unexplained falls.
*Progression of underlying neurological condition*
- Progression typically manifests as **breakthrough seizures** or specific **focal neurological deficits** rather than generalized cognitive decline.
- The clinical stability prior to these symptoms makes a **drug-induced metabolic complication** a more probable and reversible explanation.
Question 75: A 12-year-old girl with type 1 diabetes for 4 years is being reviewed in clinic. She uses an insulin pump (continuous subcutaneous insulin infusion) with good diabetes control. Her HbA1c is 52 mmol/mol (6.9%). She is going on a school sports trip involving 3 hours of moderate-intensity hiking daily for 5 days. She asks for advice about managing her diabetes during this activity. What is the most appropriate strategy to prevent hypoglycaemia during these activities?
A. Set a temporary basal rate reduction of 20-30% starting 1-2 hours before exercise (Correct Answer)
B. Discontinue the insulin pump during all physical activities and give subcutaneous rapid-acting insulin before meals
C. Increase carbohydrate intake to 30g per hour during exercise without adjusting basal insulin
D. Switch from pump therapy to multiple daily injections for the duration of the trip
E. Give 50% of usual bolus doses and reduce basal rate by 50% throughout the 5 days
Explanation: ***Set a temporary basal rate reduction of 20-30% starting 1-2 hours before exercise***- **Moderate-intensity exercise** increases insulin sensitivity, raising the risk of **hypoglycaemia**. Reducing the **basal rate** 1-2 hours prior allows existing insulin to wane and the lower dose to take effect, preventing a drop in blood glucose.- This is a well-established and flexible strategy for **insulin pump users** during **prolonged physical activity**, allowing for proactive management of glucose levels.*Discontinue the insulin pump during all physical activities and give subcutaneous rapid-acting insulin before meals*- Disconnecting the pump for 3 hours removes all **basal insulin**, which is essential for preventing **hyperglycaemia** and **ketosis**, even during exercise.- Relying solely on mealtime boluses would lead to uncontrolled glucose levels, as the body still requires continuous background insulin.*Increase carbohydrate intake to 30g per hour during exercise without adjusting basal insulin*- While additional **carbohydrates** are often needed, consuming them without reducing **insulin** can lead to **hyperglycaemia** and excessive caloric intake.- The safest approach for managing **prolonged activity** combines appropriate carbohydrate supplementation with a **reduction in insulin dosage**.*Switch from pump therapy to multiple daily injections for the duration of the trip*- Switching to **multiple daily injections (MDI)** from a pump removes the **precision** and **flexibility** of continuous insulin delivery.- This change is unnecessary and could lead to **unstable glycaemic control** due to different insulin kinetics and a lack of familiarity with the new regimen during a trip.*Give 50% of usual bolus doses and reduce basal rate by 50% throughout the 5 days*- A drastic **50% reduction** in both basal and bolus insulin across *all 5 days* is likely excessive for periods of rest or lower activity.- Such a broad reduction risks **hyperglycaemia** and potentially **diabetic ketoacidosis (DKA)** during non-exercise periods, making it an unsafe and imprecise strategy.
Question 76: A 5-year-old girl is brought to the paediatric neurology clinic following three witnessed episodes over 2 months. During these episodes, she suddenly stops playing, stares blankly for approximately 8-10 seconds with subtle eye fluttering, then immediately resumes her previous activity with no post-ictal confusion. Her development is normal and there are no abnormal findings on neurological examination. Her EEG shows 3 Hz spike-and-wave discharges during hyperventilation. What is the most important prognostic factor regarding seizure remission in this condition?
A. Age at onset of seizures (Correct Answer)
B. Frequency of seizures before treatment initiation
C. Duration of each individual seizure episode
D. Presence of photoparoxysmal response on EEG
E. Family history of epilepsy
Explanation: ***Age at onset of seizures***
- For **Childhood Absence Epilepsy (CAE)**, an earlier age of onset (typically between 4 and 8 years) is the most significant **positive prognostic factor** for achieving **seizure remission**.
- Most children who present within this age range are likely to outgrow the condition by **adolescence**.
*Frequency of seizures before treatment initiation*
- While a high frequency of absence seizures can impact a child's daily life and learning, it does not reliably predict the likelihood of **long-term remission** in CAE.
- Many children with frequent daily episodes achieve excellent control and eventual remission with appropriate **antiepileptic medication**.
*Duration of each individual seizure episode*
- Typical absence seizures are brief (5-10 seconds); slight variations in duration do not significantly alter the **prognostic outcome** for remission.
- Significantly longer or **atypical absence seizures** (which have different clinical and EEG characteristics) carry a different, generally less favorable prognosis.
*Presence of photoparoxysmal response on EEG*
- A **photoparoxysmal response** is an EEG finding typically associated with certain **idiopathic generalized epilepsies**, such as Juvenile Myoclonic Epilepsy, rather than being a primary prognostic factor for typical CAE remission.
- Its presence is not considered the most important factor in predicting the likelihood of outgrowing **Childhood Absence Epilepsy**.
*Family history of epilepsy*
- While CAE often has a **genetic predisposition**, the presence of a family history of epilepsy does not stand as the most important prognostic factor for **seizure remission** in an individual child.
- The specific clinical presentation and characteristic **EEG findings (3 Hz spike-and-wave)** are more critical for predicting remission than family history alone.
Question 77: Which biochemical feature is the defining diagnostic criterion that distinguishes diabetic ketoacidosis from hyperosmolar hyperglycaemic state in children with type 1 diabetes mellitus?
A. Blood glucose concentration greater than 30 mmol/L
B. Serum osmolality greater than 320 mOsmol/kg
C. Presence of significant ketonaemia or ketonuria (Correct Answer)
D. Serum bicarbonate less than 15 mmol/L
E. pH less than 7.3
Explanation: ***Presence of significant ketonaemia or ketonuria***
- The hallmark of **Diabetic Ketoacidosis (DKA)** is the accumulation of ketone bodies resulting from **absolute insulin deficiency**, which leads to unrestrained **lipolysis**.
- In contrast, **Hyperosmolar Hyperglycaemic State (HHS)** is characterized by a relative insulin deficiency sufficient to prevent **ketogenesis**, making the presence of high ketones the primary differentiator.
*Blood glucose concentration greater than 30 mmol/L*
- While extreme **hyperglycaemia** (>33.3 mmol/L) is a core feature of **HHS**, glucose levels in **DKA** can also be significantly elevated.
- High blood glucose alone cannot distinguish between the two states as both involve **osmotic diuresis** and varying degrees of glucose elevation.
*Serum osmolality greater than 320 mOsmol/kg*
- Severe **hyperosmolality** is the defining trait of **HHS**, but mild elevations in osmolality can still occur in patients with **DKA**.
- This parameter measures solute concentration and does not address the underlying **pathophysiological process** of ketone production unique to DKA.
*Serum bicarbonate less than 15 mmol/L*
- Low **bicarbonate** levels indicate **metabolic acidosis**, which is typical of **DKA** but is a consequence of ketone production rather than the specific diagnostic threshold.
- In some cases of **HHS**, bicarbonate can be mildly reduced due to dehydration or lactic acidosis, making it less specific than direct ketone measurement.
*pH less than 7.3*
- A **pH less than 7.3** confirms **acidemia**, which is required for a diagnosis of **DKA**, but it is secondary to the accumulation of organic acids (ketones).
- Like bicarbonate, **pH** is a measure of the severity of acidosis rather than the unique biochemical marker that identifies the metabolic pathway of **ketogenesis**.
Question 78: A 14-year-old boy with type 1 diabetes mellitus for 6 years attends the emergency department with a 2-day history of vomiting and abdominal pain. He has continued his insulin but has been unable to eat. Observations: temperature 37.8°C, heart rate 115 bpm, blood pressure 105/65 mmHg, respiratory rate 28/min with deep sighing respiration, capillary refill time 3 seconds. Capillary blood glucose is 18.2 mmol/L. Urinalysis shows 3+ ketones and 2+ glucose. What is the single most important immediate investigation to guide management?
A. Serum electrolytes, urea, creatinine and bicarbonate (Correct Answer)
B. Blood cultures and full blood count
C. Serum amylase and lipase
D. Chest radiograph
E. Abdominal ultrasound
Explanation: ***Serum electrolytes, urea, creatinine and bicarbonate***
- Confirmation of **Diabetic Ketoacidosis (DKA)** severity relies on assessing **bicarbonate** levels and pH, with a low bicarbonate being a key diagnostic criterion for metabolic acidosis.
- Monitoring **electrolytes**, especially **potassium**, is crucial to guide fluid and insulin therapy, as DKA patients often have a total body potassium deficit, and managing it prevents life-threatening arrhythmias.
*Blood cultures and full blood count*
- While **infection** can precipitate DKA, these tests do not directly confirm the metabolic diagnosis or dictate the immediate fluid and insulin management strategy.
- An elevated **white cell count** can be a stress response in DKA and is not always indicative of an underlying bacterial infection requiring immediate antimicrobial therapy.
*Serum amylase and lipase*
- **Abdominal pain** is a common symptom in DKA, often related to metabolic acidosis and gastric stasis, and typically resolves with DKA treatment.
- Though DKA can cause mild elevations in **amylase**, these tests are not immediately essential unless there is a strong clinical suspicion of co-existing **acute pancreatitis** after initial stabilization.
*Chest radiograph*
- The **deep sighing respirations** (Kussmaul breathing) are a compensatory mechanism for the metabolic acidosis, not an indication of primary lung pathology requiring immediate imaging.
- A chest X-ray would only be considered if there were specific clinical signs or symptoms suggesting a respiratory infection, such as **pneumonia**, as a potential trigger for DKA.
*Abdominal ultrasound*
- Abdominal pain in DKA is usually **functional** and non-surgical, meaning it's rarely due to an anatomical issue requiring immediate imaging.
- This investigation is generally reserved for cases where abdominal pain persists despite correction of **metabolic derangements** and adequate rehydration.
Question 79: A 9-year-old boy with epilepsy has been taking sodium valproate 600 mg twice daily for juvenile myoclonic epilepsy for the past 3 years with good seizure control. At his routine clinic appointment, his weight has increased from the 75th to the 98th centile over the past year, and examination reveals mild tremor of his hands. Blood tests show: serum sodium 139 mmol/L, potassium 4.2 mmol/L, ALT 58 IU/L (normal 10-40), AST 52 IU/L (normal 10-40), ammonia 65 µmol/L (normal 11-32). What is the most appropriate next step in management?
A. Continue current dose and repeat liver function tests in 1 month
B. Discontinue valproate immediately and commence levetiracetam
C. Reduce valproate dose to 400 mg twice daily and monitor symptoms
D. Check serum valproate level and consider dose reduction if therapeutic (Correct Answer)
E. Add topiramate as adjunctive therapy to facilitate valproate withdrawal
Explanation: ***Check serum valproate level and consider dose reduction if therapeutic*** - The patient exhibits several **dose-related side effects of sodium valproate**, including **weight gain**, **fine tremor**, and **hyperammonemia** (elevated ammonia, 65 µmol/L). - Checking **serum valproate levels** is crucial to guide a safe dose adjustment, allowing mitigation of side effects while aiming to maintain **seizure control**, especially given his 3 years of seizure-freedom. *Continue current dose and repeat liver function tests in 1 month* - Continuing the current dose ignores significant clinical signs of **drug toxicity**, such as **tremor** and rapid **weight gain**, which require active management. - While transaminases are mildly elevated, the symptomatic presentation combined with **hyperammonemia** necessitates a more immediate and proactive intervention than just observation. *Discontinue valproate immediately and commence levetiracetam* - **Abrupt withdrawal** of an effective anti-epileptic drug like valproate carries a high risk of **seizure exacerbation** or **status epilepticus**. - Switching to a new drug should be a gradual process if necessary, and valproate remains a first-line treatment for **juvenile myoclonic epilepsy (JME)**. *Reduce valproate dose to 400 mg twice daily and monitor symptoms* - An **empirical dose reduction** without knowing the current **serum valproate level** risks reducing the drug below its therapeutic range, potentially leading to **seizure recurrence**. - **Therapeutic drug monitoring** provides objective data to guide dose adjustments, ensuring efficacy is maintained while managing side effects. *Add topiramate as adjunctive therapy to facilitate valproate withdrawal* - **Topiramate** can actually exacerbate **valproate-induced hyperammonemia**, making it an unsuitable choice, especially when the primary goal is to manage existing toxicity. - Introducing **polypharmacy** is generally avoided if monotherapy can be optimized, and direct dose adjustment of valproate is the initial appropriate step for managing its side effects.
Question 80: A 6-year-old girl with type 1 diabetes mellitus attends the paediatric diabetes clinic for routine follow-up. She was diagnosed 18 months ago and uses a basal-bolus insulin regimen. Her mother reports that she has been experiencing episodes of sweating, tremor, and irritability before breakfast on several occasions over the past 2 weeks. Blood glucose monitoring shows readings of 3.4 mmol/L and 3.6 mmol/L at these times. What is the most appropriate initial management step?
A. Reduce the evening long-acting insulin dose (Correct Answer)
B. Increase the evening long-acting insulin dose
C. Add a bedtime snack containing complex carbohydrates
D. Switch from basal-bolus to mixed insulin twice daily
E. Arrange urgent continuous glucose monitoring
Explanation: ***Reduce the evening long-acting insulin dose***
- The patient is experiencing recurrent **pre-breakfast hypoglycemia**, evidenced by readings <4 mmol/L and **autonomic symptoms** (sweating, tremors), which indicates an excess of **basal insulin** overnight.
- Reducing the **evening long-acting insulin** is the standard adjustment to ensure glucose levels remain within the target range during the night and early morning hours.
*Increase the evening long-acting insulin dose*
- This action would dangerously lower overnight blood glucose levels even further, increasing the risk of **severe hypoglycemia** or seizures.
- It is contraindicated when the patient is already showing **low glucose readings** and symptoms upon waking.
*Add a bedtime snack containing complex carbohydrates*
- While a bedtime snack might provide a temporary glucose buffer, it serves as a compensatory mechanism rather than addressing the **underlying cause** of insulin excess.
- Relying on snacks can lead to **unnecessary weight gain** and may result in suboptimal glycemic control compared to adjusting the insulin dose.
*Switch from basal-bolus to mixed insulin twice daily*
- **Basal-bolus regimens** generally offer superior glycemic control and flexibility compared to **mixed insulin**, which is more prone to causing hypoglycemic peaks.
- Switching the entire regimen is unnecessary when a simple **dose titration** of the current regimen is likely to resolve the issue.
*Arrange urgent continuous glucose monitoring*
- While **CGM** is a valuable tool for tracking trends, the diagnosis of fasting hypoglycemia is already clinically clear from the **SMBG (Self-Monitoring of Blood Glucose)** readings and symptoms.
- Clinical management should be prioritized by adjusting the insulin dose first rather than delaying action for **urgent monitoring** setup.
