Paediatrics CBLS Flashcards
Patient presenting with dermatomyositis will have what characteristic features:
- Heliotrope eyelid rash
- Malar Rash
- GOTTRON’S papules
- Interstitial lung disease
- Proximal weakness
- trouble raising arms above head
- trouble rising from a chair
When suspecting dermatomyositis it is important to check?
CK
LDH
What scoring system may be used to distinguish septic arthritis from transient synovitis?
KOCHER CRITERIA
- non weight bearing
- fever > 38.5
- ESR > 40
- WBC > 12000
Recap NICE guidelines for suspected leukaemia in children and young people:
REFER of IMMEDIATE specialist attention is:
- UNEXPLAINED PETECHIAE
- OR HEPATOSPLENOMEGALY
Scoring system used for measuring joint hypermobility in children?
the BEIGHTON SCORING SYSTEM
- Looks at fingers
- Thumbs
- Elbows
- Knees
- Spine
What is the definition of JIA?
- autoimmune
- inflammation of synovial membrane
- 6 weeks to 16 years
Typical features of Systemic JIA (Still’s disease)
1. Subtle salmon pink rash
2. High swinging fever (Quotidian fever)
3. Lymphadenopathy
4. Weight loss
5. Splenomegaly
6. Muscle pain
7. Pleuritis, pericarditis
DYSREGULATED INNATE IMMUNE RESPONSE
- increased production of inflammatory cytokines
- major role of increased IL-6 and IL-1
Important investigations when suspecting sJIA
- Blood tests - FBC, ferritin, CRP, ESR, U&Es, LFTs
- CXR –> pleural effusion?
- Cardiac echo –> pericardial effusion / myocarditis
- Urine
- dipstick
- +/- microscopy
- +/- albumin/creatinine ratio - Imaging
- USS abdo
- whole body STIR MRI - Investigations to exclude infection
- blood cultures
- ASOT / throat sab
- Viral PCR / serology - EBV, CMV
- PETS, TRAVEL ? - Investigations to exclude malignancy
- bone marrow
- lymph node
- urine catecholamines
Treatment of JIA
- Anti-inflammatory –> NSAIDS, glucocorticoids
- DMARD –> methotrexate (3 month trial)
- BIOLOGICS
- anti-TNF –> etanercept / adalimumab
- IL-6 blocker –> tocilizumab
- IL-1 blocker –> anakinra
For resistant disease
- TOCILIZUMAB / ABATACEPT
- RITUXIMAB (anti-CD20) (IF RF +ve)
- TOFACITINIB (JAK inhibitor) (oral)
Important complications of sJIA
- Anterior Uveitis
- Growth failure (from steroids and chronic disease)
- flexion contractures
- Macrophage activation syndrome (high ferritin)
What signs may you see in Marfan’s syndrome?
The STEINBERG sign
- folding thumb in palm
The WALKER-MURDOCH sign
- patient puts their hand around other wrist
- if thumb and fifth finger overlap +ve
What is the name of the test used to assess functionality and stability of arch of foot
JACK TEST
Conditions which may predispose a child to easy bruising?
PLATELET ABNORMALITIES
- thrombocytopenia
- Von Willebrand disease:
- decreased vWF –> less clots - Vitamin K deficiency
- Leukaemia / malignancy
What is haemophilia?
Inherited severe bleeding disorders
Haemophilia A
- caused by deficiency in factor VIII
Haemophilia B
- deficiency in factor IX
X-LINKED RECESSIVE
What investigations should be considered for child with easy bruising?
- FULL BLOOD COUNT
- COAGULATION SCREEN
- prothrombin time (EXTRINSIC PATHWAY), coagulation
- Factor (SEVEN) –> activates 10
- activated partial thromboplastin time (INTRINSIC PATHWAY) –> platelet
- Intrinsic is 1,2,5 –> activates 10
Idiopathic thrombocytopenic purpura
Most often acute, following VIRAL infection
Clinical
- petechiae
- gingival bleeding
- epistaxis
- menorrhagia
- GI bleeding
- intracranial haemorrhage
Management
- supportive in children
- PLATELETS –> if severe bleeding or v. low. Platelet count
- corticosteroids
- IVIG, or anti D immunoglobulin
- splenectomy
Thrombotic Thrombocytopenic Purpura
Rare, many small blood clots throughout the body
Deficiency of ADAMTST13
- would normally break up large multimers of vWF
- adhesion and aggregation of platelets to wVF bits
Disseminated intravascular coagulation
accelerated clotting within blood vessels
increased consumption of platelet and clotting factors
uncontrollable bleeding
Haemolytic Uraemic syndrome
Commonly post E COLI infection 0157.
Shiga toxin
- ABDO PAIN
- VOMITTING
- BLOODY DIARRHOEA
then
- haemolytic anaemia
- thrombocytopenia
- AKI
Tx
- mainly supportive
- no ABx
- plasma infusion and plasma exchange
- ECULIZUMAB (if severe CNS involvement)
Viruses which may trigger T1DM?
- coxsackie B
- enterovirus
Different types of insulin available
-
SHORT ACTING SOLUBLE
- 30-60 mins onset
- take 15-30 mins before meal
- try to mirror normal physiology -
Rapid acting human analogues
- even quicker than soluble
- just before or after a meal -
Intermediate and long acting
- take several hours for onset of effect
- last 16-24hrs
- once or twice daily -
Biphasic insulins
- quick onset
- 1 injection
What are the different types of insulin regimes available?
-
Fixed dose once daily
- Long acting or intermediate acting insulin
- BEDTIME
- Only suitable T2DM
-
Fixed dose twice daily
- on assumption patient eats 3 regular meals
- biphasic insulin injected twice daily
- worries about nocturnal hypoglycaemia due to peak trough pattern of evening dose
-
Multiple daily injection basal bolus
- Intermediate or long acting given at night.
- Rapid acting or short acting given at meal times.
- Good flexibility
- used with carbohydrate counting to calculate short/rapid acting dose
-
Insulin pump
- continuous infusion of short acting
- able to give bolus
- adjustable rate
- flexible
Recap the process of carb counting
- carb counting” is the process of calculating the carbohydrate load
- then calculating how many units of insulin you will need to inject to account for this.
NOTES
To do this, use the “500 rule.”
This is 500 divided by the total daily dose of insulin.
So, as an example, let’s say the patient’s total daily dose of insulin is 25 units.
500/25 = 20
Therefore 1 unit of insulin will be sufficient to cover 20 g of carbohydrate in the meal*
What is a correction dose?
- dose of insulin given in addition to usual insulin dose in order to resolve an episode of hyperglycaemia
NOTES
- Initially, this can be calculated based on the “100 rule”
- 100 divided by your total daily dose of insulin = insulin sensitivity factor.
- So, if you are on a total daily dose of 25 units (all forms of insulin, however long-acting they are), then your initial estimated insulin sensitivity will be 4. 1 unit of insulin is estimated to lower blood glucose by 4 mmol/litre.