Final Paeds III

Question 1

What is a key complication of systemic JIA? [1]

What is the typical presentation of this complication? [4]

What is a key investigational finding? [1]

Answer 1

A key complication is macrophage activation syndrome (MAS)
- Where there is severe activation of the immune system with a massive inflammatory response.

It presents with an acutely unwell child with disseminated intravascular coagulation (DIC), anaemia, thrombocytopenia, bleeding and a non-blanching rash.
- It is life threatening. A key investigation finding is a low ESR.

Question 2

Describe the general presentation of polyarticular JIA [2=

How do RF +ve and -ve polyarticular JIA present differently? [2]

Answer 2

Polyarticular JIA involves idiopathic inflammatory arthritis in 5 joints or more.
- mild fever, anaemia and reduced growth.

RF-positive polyarticular JIA
- is characterized by symmetric joint involvement, particularly in the small joints of the hands and feet.

RF-negative polyarticular JIA
- typically involves larger joints such as the knee, hip, and shoulder.

Question 3

What is another indication that a patient has oligoarthritis JIA? [1]

Answer 3

Patients tend not to have any systemic symptoms and inflammatory makers will be normal or mildly elevated.

Antinuclear antibodies are often positive, however rheumatoid factor is usually negative.

Question 4

[] is the most common gastrointestinal complication of HSP

Answer 4

Intussusception is the most common gastrointestinal complication.

Question 5

What Ix would you conduct for HSP?

Answer 5

Blood tests
- Coagulation studies: prothrombin time, partial thromboplastin time, and fibrinogen - Should be normal in HSP. Helps in ruling out other diagnoses such as thrombocytopenia
- Serum creatinine and electrolyte levels: ?renal failure
- Raised ESR

Urine test:
- May show hematuria, proteinuria or casts

Biopsy:
- confirmation of the diagnosis in patients with an unusual presentation (e.g., headaches, seizures, or pulmonary haemorrhage) is made via biopsy of an affected organ such as skin or kidney.
- renal biopsy: the immunofluorescence shows IgA deposition in the mesangium hence resembling IgA nephropathy.

Question 6

Describe the management of HSP [+]

Answer 6

Treatment of pain:
- Mild to moderate JOINT pain can typically be managed with either ibuprofen or paracetamol.
- Mild to moderate ABDOMINAL pain is managed with paracetamol and supportive care.
- Oral glucocorticoid therapy is used to manage severe abdominal pain in HSP patients. If abdominal pain is accompanied by nausea and vomiting, intravenous corticosteroids may be used.

Renal involvement:
- Specific treatment with intravenous corticosteroids (pulse dosing) is recommended only in patients with nephrotic-range proteinuria and/or those with declining renal function.
- A combination of corticosteroids, immunosuppressants, and plasmapheresis is used in the patient with rapidly progressive nephritis.
- Renal transplant is reserved in patients who develop end-stage renal disease.’

Question 7

When / why might a baby be suffering from IDA? [1]

Answer 7

If they are exclusively milk feeding (bottle / breastfeeding) at ~ 1 year age

Question 8

A baby has rhesus incompatibility. How would they present [4] due to which condition? [1]

How might you treat? [4]

Answer 8

Hydrops fetalis
- Skin oedema, pericardial effusions, ascites, secondary jaundice

Treatment:
- Phototherapy, IV IG, Blood transfusion, exchange transfusion

Question 9

How would you determine if a microcytic anaemia is due to IDA or thalassemia minor on initial investigation? [1]
How would you then determine if a patient has Beta thalassaemia minor / alpha thalassaemia minir? [2]

Answer 9

Look at ferritin level
- if low = IDA
- if normal = thal. minor

Then do Hb electrophoresis
- Elevated HbA2 = beta thal. minor
- Normal HbA2 = alpha thal. minor

Question 10

Normocytic MCV:

• How would you determine if a child is suffering from haemolytic cause / blood loss? [1]
• How would you determine if is from marrow hypoplasia / leukaemia

Answer 10

Reticulocytes increased

Question 11

Describe the treatment ladder for ITP [3]

Answer 11

Cutaneous symptoms only:
* Watchful waiting

Mucosal bleeding:
- Steroids/ IVIG/ topical or oral TXA

Chronic ITP:
- TPO agonists, MMF, rituximab, splenectomy

Question 12

Describe the typical presentation of a febrile seizure [1]

Answer 12

A typical presentation is a child around 18 months of age presenting with a 2 – 5 minute tonic clonic seizure during a high fever.

NB: The fever is usually caused by an underlying viral illness or bacterial infection such as tonsillitis. Once a diagnosis of a febrile convulsion has been made, look for the underlying source of infection.

Question 13

What is Dravet syndrome and how would you differentiate between a febrile covulsion?

Answer 13

Dravet syndrome
- previously known as severe myoclonic epilepsy of infancy, is a much more serious, lifelong condition, presenting with intractable seizures that are difficult to manage.

The first seizure often occurs with a fever, but it is distinct from febrile convulsions in that it commonly presents with additional issues including developmental delay.
- It is also unlikely to present after the first year of life.

Question 14

What are the two types of breath holding spells in children? [2]

Describe how each present [4]

Answer 14

They are often divided into two types: cyanotic breath holding spells and pallid breath holding spells (also known as reflex anoxic seizures)

Cyanotic breath holding spells:
- occur when the child is really upset, worked up and crying
- After letting out a long cry they stop breathing, become cyanotic and lose consciousness.
- Within a minute they regain consciousness and start breathing.
- They can be a bit tired and lethargic after an episode.

Reflex anoxic seizures
- occur when the child is startled
- the vagus nerve sends strong signals to the heart that causes it to stop beating
- The child will suddenly go pale, lose consciousness and may start to have some seizure-like muscle twitching.
- Within 30 seconds the heart restarts and the child becomes conscious again.
- PM: Typical features, child goes very pale
falls to floor, secondary anoxic seizures are common, rapid recovery

Question 15

Breath holding spells have been linked with []. Treating the child if they are [] can help minimise further episodes.

Answer 15

Breath holding spells have been linked with iron deficiency anaemia. Treating the child if they are iron deficiency anaemic can help minimise further episodes.

Question 16

Describe what is meant by hyaline membrane disease [1] and how it would present (clinically [3] and on imaging [4]

Answer 16

Lack of sufficient surfactant production

Clinical findings:
* Symptoms present in first 2 hours of life
(Symptoms that begin > 8 hrs are not due to HMD)
* May increase severity from 24 - 48 hours
* Then, gradual improvement > 48-72 hours

Imaging:
* Typically, diffuse “ground-glass” or finely granular appearance
* Bilateral and symmetrical distribution
* Air bronchograms are common
* Hypoaeration in non-ventilated lungs (Hypoaeration in non-ventilated lungs)

Question 17

Tx plan for hyaline membrane disease? [4]

Answer 17

• Positive end-expiratory pressure (PEEP
• Continuous positive airway pressure (CPAP)
• Surfactant administered via ETT
• Oxygen and diuretics

Question 18

Describe what is meant by Bronchopulmonary Dysplasia (AKA Chronic Respiratory Insufficiency of the Premature) [2]

Answer 18

BPD is consequence of early acute lung disease
- One definition involves an oxygen requirement at 28 days of life to maintain arterial oxygen tensions >50 mm Hg accompanied by abnormal chest radiographs

Question 19

Imaging findings for TTN? [4]

Answer 19

Imaging Findings
* Hyperinflation of the lungs
* Fluid in the fissures
* Laminar effusions
* Fuzzy vessels

Question 20

What is the most common cause of neonatal respiratory distress in full-term/postmature infants? [1]

Answer 20

Meconium Aspiration Syndrome

Question 21

Describe the basic pathophysiology of a reflex anoxic seizure [1]

Answer 21

The underlying pathophysiology is a vagal-induced brief cardiac asystole with resultant transient cerebral hypoperfusion caused by a sudden distressing stimulus

Question 22

What is the only definitive managment of a reflexic anoxic seizure? [1]

Answer 22

Pacemaker insertion is the only definitive treatment and is only used for frequent, severe cases.3 4

Question 23

NB: This is for adults

What is the mx of a tension type headache? [+]

Answer 23

ZtF:
- Management is with reassurance, analgesia, regular meals, avoiding dehydration and reducing stress.

PM (adults)
acute treatment:
- aspirin, paracetamol or an NSAID are first-line

prophylaxis:
- NICE recommend ‘up to 10 sessions of acupuncture over 5-8 weeks’
- low-dose amitriptyline is widely used in the UK for prophylaxis against tension-type headache.
- The 2012 NICE guidelines do not however support this approach ‘…there was not enough evidence to recommend pharmacological prophylactic treatment for tension-type headaches. The GDG considered that pure tension-type headache requiring prophylaxis is rare. Assessment is likely to uncover coexisting migraine symptomatology with a possible diagnosis of chronic migraine.’

Question 24

Describe the acute Mx of migraines in children [5]

Answer 24

Ztf: * **Rest**, fluids and low stimulus environment * **Paracetamol** * **Ibuprofen** * **Sumatriptan** * Antiemetics, such as **domperidone** (unless contraindicated) PM: first-line: offer combination therapy with * an **oral triptan and an NSAID**, or * an **oral triptan and paracetamol** * for young people aged 12-17 years consider a **nasal triptan in preference to an oral triptan** * if the above measures are not effective or not tolerated offer a **non-oral preparation of metoclopramide or prochlorperazine** and **consider adding a non-oral NSAID or triptan**

Question 25

Describe the management of mild, moderate and severe OCD [+]

Answer 25

**If functional impairment is mild low-intensity psychological treatments:** * **cognitive behavioural therapy (CBT) including exposure and response prevention (ERP)** * If this is insufficient or can't engage in psychological therapy, then offer choice of either a course of an **SSRI or more intensive CBT (including ERP)** **If moderate functional impairment** * offer a choice of either a course of an SSRI (**any SSRI for OCD but fluoxetine specifically for body dysmorphic disorder) or more intensive CBT (including ERP)** **If severe functional impairment** * **offer combined treatment with an SSRI and CBT (including ERP)** ## Footnote NB: These notes are for adults

Question 26

Describe the 5 step approach to the management of panic disorder? [5]

Answer 26

Again a stepwise approach: **step 1:** recognition and diagnosis **step 2:** treatment in primary care - see below - NICE recommend either **cognitive behavioural therapy or drug treatment SSRIs are first-line.** - If contraindicated or no response after 12 weeks then **imipramine or clomipramine should be offered** **step 3**: review and consideration of alternative treatments **step 4:** review and referral to specialist mental health services **step 5:** care in specialist mental health services

Question 27

The severity of Bulimia nervosa is also categorised based on the frequency of inappropriate compensatory behaviours (as per DSM-5): What determines mild, moderate, severe and extreme bulimia? [4]

Answer 27

**Mild**: An average of 1-3 episodes per week. **Moderate**: An average of 4-7 episodes per week. **Severe**: An average of 8-13 episodes per week. **Extreme**: An average of 14 or more episodes per week.

Question 28

Describe the dx of BN

Answer 28

The diagnostic criteria for Bulimia Nervosa, as per the ICD-10 and DSM-5, are outlined below. It's imperative to note that these criteria are not exhaustive and should be used in conjunction with clinical judgement. **ICD-10 Criteria:** * A **persistent preoccupation with eating, and an irresistible craving for food**; the patient succumbs to **episodes of overeating** in which **large amounts of food** are consumed in short periods of time. * The patient attempts to **counteract the 'fattening' effects of food by induced vomiting, purgative abuse, alternating periods of starvation, or use of drugs such as appetite suppressants**. * If the disorder occurs in diabetic patients they may choose to **neglect their insulin treatment.** **DSM-5 Criteria:** * **Recurrent episodes of binge eating** characterised by **both consuming an amount of food that is definitely larger than most people would eat during a similar period under similar circumstances and a sense of lack of control over eating during the episode**. * Recurrent inappropriate compensatory behaviours to** prevent weight gain such as self-induced vomiting; misuse of laxatives, diuretics or other medications; fasting; or excessive exercise.** * The binge eating and inappropriate compensatory behaviours both occur, on average, **at least once a week for three months.** * Self-evaluation is unduly influenced by body shape and weight.

Question 29

How does transient synovitis present on xray? [1] How does US of the hip present? [3]

Answer 29

**Xray**: normal **Ultrasound finding**s in transient synovitis can include **intracapsular fluid, joint effusion and synovial thickening**

Question 30

NICE recommend that a child aged between 3-9 years with a working diagnosis of transient synovitis can be managed in primary care if: [3]

Answer 30

They are **afebrile**, **mobile** but **limping** and symptoms have been **present for < 48 hours**

Question 31

Describe the management of Perthes disease (conservative [4] and surgical [3]

Answer 31

**PM**: * If less than **6 years: observation** * Older: **surgical management with moderate results** * **Operate on severe deformities** **Conservative management:** - This includes **activity modification** - **Pain** **management** with analgesics or anti-inflammatory medications - **physical therapy** to maintain range of motion and muscle strength. - **Orthotic device**s, such as casts or braces, may be used to provide containment of the femoral head within the acetabulum. **Surgical management:** * Procedures include **femoral or pelvic osteotomies to improve hip joint congruence** * **arthrodiastasis** to decompress the hip joint * in severe cases, **hip arthroplasty.**

Question 32

What is chondromalacia and how do you differentiate this from OSD? [2]

Answer 32

**Chondromalacia patella** **Similarities**: - knee pain presenting in sporty teenagers, managed conservatively **Differences**: - typically presents with pain on anterior or inner side of the knee ## Footnote Chondromalacia patellae is a condition where there is damaged cartilage behind the patella (kneecap). It is like a softening or wear and tear of the cartilage.

Question 33

What are the clinical signs of osteochondritis dissecans [5]

Answer 33

**Joint effusion** **Full range of movement** in the joint without signs of ligamentous instability **External tibial rotation when walking** - if medial femoral involvement **Tenderness on palpation of the articular cartilage of the medial femoral condyle, when the knee is flexed** **Wilson's sign** for detecting medial condyle lesion - with the knee at 90° flexion and tibia internally rotated, the gradual extension of the joint leads to pain at about 30°, external rotation of the tibia at this point relieves the pain

Question 34

Describe the investigations used for osteochondritis dissecans [4]

Answer 34

**X-ray** (anteroposterior, lateral and tunnel views) - may show the **subchondral crescent sign or loose bodies** **MRI** - used to evaluate cartilage, visualise loose bodies, stage and assess the stability of the lesion **CT** - may be used in preoperative planning and in cases where MRI is not available or contraindicated **Scintigraphy** - may be used to guide treatment as it may show increased uptake in the fragments - a sign of osteoblastic activity

Question 35

Describe the limited range of motion seen in hip movements of a child w SUFE [3]

Answer 35

There may be restriction in hip movements, especially **internal rotation, abduction and flexion**. Forced passive internal rotation often exacerbates the pain.

Question 36

Describe the management of SUFE

Answer 36

The management of slipped capital femoral epiphysis (SCFE) is primarily surgical, aiming to stabilise the epiphysis and prevent further slippage. The specific management steps are as follows: **In Situ Pinning:** - This is the most common treatment for stable SCFE. A single screw or pin is inserted percutaneously into the femoral head to secure it in place. In unstable SCFE, multiple screws may be used. **Open Reduction and Internal Fixation (ORIF):** - In severe cases or when closed methods fail, open reduction followed by internal fixation can be performed. This involves surgically exposing the hip joint, realigning the displaced femoral head under direct vision and then securing it with screws. **Osteotomy**: - If there is significant deformity or chronic SCFE, an osteotomy may be required. This procedure involves cutting and realigning the bone to correct its position and orientation.

Question 37

Describe which ages determine different treatment methods for DDH [3]

Answer 37

**Pavlik harness:** - Under 6 months **Closed reduction and spica casting:** - In infants aged six months to two years - or when Pavlik harness treatment fails **Open reduction**: - For children older than two years, or when closed reduction is unsuccessful

Question 38

What is talipes? [1] What is talipes equinovarus? [1] What is talipes calcaneovalgus? [1]

Answer 38

**Talipes** is a fixed abnormal ankle position that presents at birth. It is also known as **clubfoot**. It can occur spontaneously or be associated with other syndromes. It is usually identified at birth or during the newborn examination. **Talipes equinovarus** describes the **ankle in plantar flexion and supination.** **Talipes calcaneovalgus** describes the **ankle in dorsiflexion and pronation.**

Question 39

Desribe the Ponseti method used to treat talipes

Answer 39

The Ponseti method is a way of treating talipes without surgery. It is usually very successful. **Treatment is started almost immediately after birth**. It is performed by a properly trained therapist. The foot is **manipulated towards a normal position and a cast is applied to hold it in position**. This is **repeated over and over until the foot is in the correct position**. At some point an **achilles tenotomy** to **release tension** in the **achilles tendon is performed, often in clinic**. **After treatment with the cast is finished a brace is used to hold the feet in the correct position when not walking until the child is around 4 years old.** This brace is sometimes referred to as “boots and bars”.

Question 40

Describe what is meant by positional talipes [1] and how it is treated [1]

Answer 40

**Positional talipes** is a common condition where the **resting position of the ankle is in plantar flexion and supination**, however it is **not fixed in this position** and there is no structural boney issue in the ankle. - The muscles are **slightly tight around the ankle but the bones are unaffected**. This requires **referral** to a **physiotherapist** for some simple exercises to **help the foot return to a normal position**. Positional talipes will resolve with time.

Question 41

Wheat is mneumonic for osteogenesis imperfecta presentation? [4]

Answer 41

**BITE** **B**ones fracture **I**(eye) blue sclera **T**eeth imperfections **E**ar hearing loss

Question 42

A breastfed baby with GORD should have a trial [] first line

Answer 42

A breastfed baby with GORD should have a trial an **alginate (e.g. .Gaviscon) first line**

Question 43

How can you think of MODY and LADA? [1]

Answer 43

LADA to 'Adult variant-T1DM' and MODY to 'Young variant-T2DM'

Question 44

SCA - how can you tell from the blood film if the patient has aplastic crisis or sequestration crisis? [2]

Answer 44

Aplastic crisis has reduced reticulocytes, whereas sequestration crisis has increased reticulocytes