PAEDS 3: Haem, Neuro, CAMHS and Safeguarding Flashcards
What is Acute Lymphoblastic Leukaemia (ALL)?
Uncontrolled proliferation of undifferentiated cells in the bone marrow
What is the most common childhood cancer?
ALL
How does ALL present?
Bone marrow failure - anaemia (SOB, pallor), thrombocytopenia (petechial rash, bleeding), neutropenia (recurrent infections)
Local infiltration - lymphadenopathy (+/- thymic enlargement), splenomegaly, hepatomegaly, testes, CNS, bone sx
What investigations would you do for ALL?
Bedside = examination + obs
Bloods = FBC and clotting, peripheral blood film (lymphoblasts, “Auer rods”)
Imaging = CXR showing enlarged thymus
Special tests = bone marrow biopsy
How is ALL managed?
Chemotherapy + supportive care w/blood products and abx etc.
Other options = CNS-targeted therapy (intrathecal i.e. into CSF) intrathecal, molecular treatment
Definitive = transplant (siblings usually most ideal)
What is the process of chemotherapy for ALL?
- Remission induction
- Consolidation and CNS therapy
- Intensification
- Maintenance
What is Immune Thrombocytopenic Purpura?
IgG mediated destruction of platelets
Most common cause of thrombocytopenia in children
Most common in 2-6yo
How does ITP present?
Petechiae, superficial bruising, epistaxis etc.
Recent infections, bleeding (cuts, nose), autoimmune conditions (PMHx, FHx)
What investigations should be done for ITP?
Diagnosis of exclusion
Bedside = examination
Bloods = FBC, blood smear
How is ITP managed?
Self-limiting
Major bleeding give IVIG and corticosteroids if >3 months
Chronic ITP (i.e. >6m) give immunosuppressants and consider splenectomy 2nd line
What is the aetiology of Sickle Cell Disease?
Defective beta globin chain (point mutation at codon 6 on Chr 11 = glutamine to valine)
Autosomal recessive condition
What are RFs for Sickle Cell Disease?
Afro-Carribean; higher prevalence in areas w/malaria
How can sickle cell disease present?
Hand and foot syndrome (swollen hands and feet; dactylitis - one of earliest signs)
Acute chest syndrome
Splenic sequestration (anaemia, shock, death)
Painful crises/vaso-occlusive (+/- priapism)
Infection (pneumococcus and parvovirus)
Splenomegaly
What investigations should do you do Sickle Cell Disease?
Bedside = family origins questionnaire (FOQ) - Indian, Mediterranean, Middle East, Guthrie testing after antenatal screening
Bloods = haemoglobin electrophoresis (gold-standard), FBC, blood smear (sickle cells, howell-jowell bodies assoc. w/hyposplenism, nucleated RBCs)
How are acute sickle cell crises managed?
Analgesia
Oxygen
Hydration
Exchange transfusion (ACS, priapism, stroke)
Abx (in infection i.e. parvovirus)
What does long term management of sickle cell disease include?
Education = minimise trigger exposure for crises (cold, dehydration, excessive exercise, hypoxia)
Vaccination = encapsulated organisms (NHS)
Prophylaxis = OD oral penicillin, OD oral folic acid
Medical management of chronic problems = hydroxycarbamide (for recurrent hospital admission for ACS or vaso-occlusive crises)
Severe cases = HSCT
What is beta-thalassaemia?
Reduced synthesis of beta globin chain
Autosomal recessive
Manifests after first 3-6 months of life bc of HbF
How does beta thalassaemia present?
MAJOR = extramedullary haemotopoeisis (bone expansion, hepatosplenomegaly, frontal bossing), anaemia (HF, growth retardatio), Fe overload (HR, gonadal failure)
TRAIT = asx, microcytosis/normal-low-normal Hb
How is beta thalassaemia investigated?
BEDSIDE = FOQ questionnaire
Guthrie testing after antenatal screening
BLOODS = Hb electrophoresis (gold standard), blood smear (microcytic red cells, tear drop cells, microspherocytes, target cells, schistocytes, nucleated RBCs)
How is beta thalassaemia managed?
MAJOR = blood transfusion +/- iron chelation w/desferrioxamine, deferiprone (NOTE: HSCT reserved for children w/HLA identical sibling)
MINOR = no treatment needed
What are the main causes of iron deficiency anaemia in children?
Inadequate iron intake - breastmilk, cow’s milk, solids once weaned
Malabsorption
Blood loss
How does IDA present in children?
No sx until Fe <60-70g/L
Feed slowly/tire quickly
Pica = eating non-food items like soil or dirt etc.
How is IDA in children investigated?
FBC and iron studies - low MCV, low ferritin
How is IDA in children managed?
Dietary advice
Oral ferrous sulphate (200mg TDS) until normal Hb - continue for at least 3/12 after and monitor iron levels
What is Caput Succedaneum?
Neonatal swelling that crosses suture lines, resolves over few days, start at birth
Assoc. w/vaginal delivery
What is Cephalohaematoma?
Neonatal swelling that is benign, in margins of skull sutures, resolve over few months, start day 2-3 of life (delayed onset)
Assoc. w/assisted delivery
How do extracranial haemorrhages in neonates present and how are they investigated and managed?
Measure head circumference
Usually self-resolves
What is a subgaleal haemorrhage?
More severe neonatal swelling assoc. w/hypovolaemia and shock and may be assoc. w/coagulopathy
May be assoc. w/NAI (if not at birth)
What is hydrocephalus?
Increased volume of CSF occupying ventricles
What are the two aetiologies of hydrocephalus?
Communicating
Non-communicating
What is communicating hydrocephalus?
Flow of CSF is obstructed after it exits the ventricles due to failure to resorb CSF
Caused by: meningitis, SAH
What is non-communicating hydrocephalus?
Flow of CSF obstructed within the ventricles.
Caused by:
- congenital aqueduct stenosis e.g. Dandy-Walker malformation (4th ventricle enlarged w/no outlets), Chiari malformation (cerebellar tonsils displaced down through foramen magnum)
- acquired aqueduct stenosis through IVH (preterm infants, grade 3 or 4)
How does hydrocephalus present?
ACUTE = vomiting, irritable, impaired consciousness
CHRONIC = failure to thrive, developmental delays
OTHER = increased head circumference, tense fontanelle, increased tone, papilloedema, ataxic gait
Sunset sign = eyes appear to be drive down bilaterally
How is hydrocephalus investigated?
BEDSIDE = measure head circumference
IMAGING = cranial USS
How is hydrocephalus managed?
NOTE: make sure to treat any identifiable underlying causes
1st line = ventriculoperitoneal shunt (risk of blockage of infection - if happens need replacement)
Medical mx = furosemide (inhibits CSF production, removes excess fluid)
What is Intraventricular Haemorrhage?
Bleeding into ventricles, occurs more often in premature babies due to VLBW and LBW
What can cause IVH?
Changes in perfusion in brain due to:
- ECMO in preterm babies
- congenital CMV infection
Roughly what are the different grades of IVH?
I = subependymal region/germinal matrix
II = extension into normal sized ventricles, filling <50% volume of ventricle
III = extension into dilated ventricles
IV = IVH w/parenchymal extension
How does IVH present?
Sleepiness, lethargy, apnoea, reduced/absent moro reflex, hypotonia, tense fontanelle
How is IVH investigated?
Trans-fontanelle USS
How is IVH managed?
Fluid replacement
Anticonvulsant
Acetazolamide (reduce CSF) +/- LP
VPS if hydrocephalus
What risk factors do you only need one of to do a CT head scan within 1 hour for <16yo?
- suspicion of NAI
- post-traumatic seizure
- GCS <14 (or if under 1yo <15)
- at 2hrs post-injury GCS <15
- suspected open or depressed skull fracture, tense fontanelle
- signs of basal skull fracture (‘panda eyes’, haemotympanum, CSF leakage, Battle’s sign)
- focal neurological deficit
- under 1yo w/ bruise, swelling or laceration >5cm on head
What risk factors do you need 2 or more of to do a CT head scan within 1 hour for <16yo OR one of which warrants 4 hour observation period?
- LOC >5mins (witnessed)
- abnormal drowsiness
- 3 or more discrete episodes of vomiting
- dangerous mechanism of injury e.g. RTA, fall from >3m, high-speed injury
- amnesia >5mins
- any current bleeding or clotting disorder
What is a Breath Holding Spell?
Temporary apnoea, occurs during vigorous crying due to pain, frustration, anger, fear
Usually stop by age 4-5
How does a breath holding spell present?
During spell, child may:
- cry and be silent while holding breath
- open mouth as if crying but no cry
- turn pale/blue/grey
- become floppy or stiff
- LOC for 1-2mins
How is breath holding spell investigated?
clinical diagnosis
How are breath holding spells managed?
Resolves spontaneously
Can use behaviour modification (distraction)
What is Microcephaly?
A birth defect where a newborn’s head is smaller than expected compared to others of the same sex and age.
May also have under-developed brain.
Very rare.
What are causes of microcephaly?
Infections during pregnancy: rubella, toxoplasmosis, CMV
Severe malnutrition
Exposure to alcohol, drugs or toxins in-utero
Disruption to blood supply in-utero
What may children with microcephaly experience?
Seizures, developmental delay, intellectual disability, hearing loss, vision loss
What investigations should be done for Microcephaly?
During pregnancy: USS
After birth: measure head circumference
Test for underlying causes
How is microcephaly managed?
Will depend on symptoms
MDT approach: paediatrician, SALT, OT/PT
What professionals may be involved in an MDT for a child w/microcephaly?
Paediatrician
Psychiatrist
ADHD SNs
Mental health and Learning Disability trusts
CAMHS
Parent groups
Social care workers
School/college and school nurses
What is Cerebral Palsy?
A non-progressive abnormality of movement and posture, causing activity limitation attributed to non-progressive disturbances that occured in the developing foetal or infant brain.
Must occur before age 2 (otherwise called “acquired brain injury”)
Most common cause of motor impairment in children
What are RFs for cerebral palsy?
Antenatal: preterm birth, chorioamnionitis, maternal infection
Perinatal: LBW, HIE, neonatal sepsis
Postnatal: meningitis
How might cerebral palsy present?
- delayed milestones
- abnormal limb/trunk posture and tone e.g. stiff legs and scissoring, unable to weight bear, hypotonia, spasticity, fisted hands
- feeding difficulties
- abnormal gait
- hand preference before 1yo
How is cerebral palsy investigated?
Full developmental assessment
MRI head
How is cerebral palsy managed?
MDT required
Paediatrician - medical problems
- stiffness = diazepam, baclofen
- sleeping = melatonin
- constipation = movicol
- drooling = anticholinergic
- keeping eye out for epilepsy (33% affected)
Other MDT members needed inc. nurses, PT, SALT, OT
What is the prognosis of cerebral palsy?
WALKING = children who sit by age 2 likely to walk unaided by 6yo
SPEECH = 50% have communication difficulties
SUPPORT = SCOPE disability charity
More severe CP = lower life expectancy
What are the different types of cerebral palsy?
SPASTIC (most common) = muscles are stiff and tight, motor cortex damage
DYSKINETIC = involuntary movements, basal ganglia damage
ATAXIC = shaky movements, affecting balance and proprioception, cerebellar damage
MIXED types = combination damage
What is ADHD?
Dysfunction of brain neurone circuitry that rely on dopamine in controlling self-monitoring/regulation
Affects boys more than girls (3:1)
What are some RFs for ADHD?
FHx, prematurity, LBW, FAS (foetal alcohol syndrome), conduct/mood disorder, ASD, LD
What is the triad of symptoms of ADHD?
- Impaired attention
- Overactivity
- Impulsivity
Assess impact at school and at home
Assess presence since childhood
How is ADHD investigated?
Thorough history to assess impact on education and relationships
Rating scales:
- Conner’s comprehensive behaviour rating scale
- Strengths and difficulties questionnaire
How is ADHD managed?
MDT approach
- Watchful waiting (10w)
- ADHD-focused group parent-training programme
- Specialist referral for medication (methylphenidate 6w trial, others inc. lisdexamphetamine, dexamphetamine, atomoxetine, guanfacine)
Monitoring - height, weight, HR/BP, ECG
What is the aetiology of ASD?
> 1% prevalence, presents age 2-4, more common in boys (4:1)
How does ASD present?
- Deficits in verbal/non-verbal communication
- Deficits in social interaction
- Restrictive or repetitive behaviours/interests
What symptoms may be in the history for ASD?
- delays in language development
- social skills domain of developmental hx e.g. not playing w/others
- challenges understanding jokes/sarcasm
- throwing tantrums
- repetitive behaviours
- picky eating, fascination w/water
How is ASD investigated?
Hearing, speech and language assessment
Cognitive assessment e.g. WISC, WPPSI
Autism diagnosis and assessment:
- ADI-R (autism diagnostic inventory) = clinical diagnostic instrument, suitable for mental ages >18m
- ADOS (autism diagnostic observatory schedule) = semi-structured standardised assessment, suitable for all developmental levels and ages, focus on current data
- DISCO (diagnostic interview for social and communication disorders) = detailed, semi-structured interview, suitable for all ages, assessing development from birth to current day
How is ASD managed?
PSYCHOSOCIAL = aim to increase attention, engagement and reciprocal communication, adjust to developmental level, increase carer/teachers’ understandings, techniques to expand communication
SALT = social skills training
DIFFICULT BEHAVIOUR = anticipate and reduce factors that increase these
FAMILIES AND CARERS = attend to their needs, offer personal, social and emotional support, offer practical support in caring role (respite breaks and emergency plans), plan for the future (e.g. transition to adult care), offer carer’s needs assessment
EDUCATIONAL = assess for LD, if needing extra support, discuss EHC plan (legal doc describing child’s special education, health and care needs)
PHARMACOLOGICAL = antipsychotics/melatonin/methylphenidate
*EHC = education, health and care plan
What are RFs for eating disorders?
Female, co-morbid depression, OCD
How does anorexia nervosa present?
- BMI <17.5 (or weight >15% less than expected)
- Deliberate weight loss (e.g. laxatives, vomiting, excessive exercise, appetite suppressants etc.)
- “Fear of the fat” / distorted body image (i.e. overvalued ideas of being fat despite being thin)
How does bulimia nervosa present?
- Binging or persistent preoccupation w/eating and/or irresistible craving for food
- Purging behaviours (attempts to counteract “fattening” effects of body) e.g. diuretics, vomiting, laxatives
- Psychopathology (feeling of a loss of control, morbid dread of fatness, patient sets sharply defined weight threshold)
How are eating disorders investigated?
BEDSIDE: SCOFF questionnaire, Physical exam (weight, height, lanugo hair, BP, squat test), ECG (bradycardia, arrhythmia, long-QT)
BLOODS AND URINE DRUG SCREEN: exclude medical causes i.e. hyperthyroidism (LOW - ESR, Hb, platelets, WCC, Na, K, Phos, T4, glucose) (HIGH - cortisol, cholesterol, carotenaemia, GH, glands, LFTs)
IMAGING: DEXA to screen for osteoporosis (if >2yr history)
How are eating disorders managed?
MILD = advice/support for 8 weeks + BEAT charity
MODERATE = routine CEDS referral
SEVERE = urgent CEDS referral; consider A&E if prolonged QT, signs of end-organ failure, MEED (medical emergencies in eating disorders) guidelines
THERAPY = family therapy, CBT, consider fluoxetine
What are personality disorders?
A set of consistent thoughts, feelings and behaviours shown across time in a variety of settings which may lead to suffering of the individual or others.
ICD-11: a marked disturbance in personality functioning, which is nearly always associated with considerable personal and social disruption.
What are the key signs to diagnose a personality disorder?
- Pervasive: occurs in all/most areas of life
- Persistent: evident (starts) in adolescence and continues through adulthood
- Pathological: causes distress to self or others, impairs function
Cannot be diagnosed in <18s
What are the types of personality disorders?
Paranoid
Schizoid
Schizotypal
Histrionic
EUPD/Borderline
Dissocial/Antisocial
Anankastic
Anxious/avoidant
Dependent
How are personality disorders managed?
BIO - antipsychotics/antidepressants
PSYCHO - DBT (and others)
SOCIAL - therapeutic community
What are the core features of depression?
Low mood, anhedonia, anergia
What investigations should be done for childhood depression?
Clinical diagnosis
BEDSIDE: PHQ-9, CDI (children’s depression inventory)
BLOODS: FBC, TFTs, HbA1c
How is depression or GAD in children managed?
- Watch and wait
- PSYCHO - family therapy, CBT (12-18s)
- BIO - fluoxetine (first line SSRI)
- SOCIAL - charities e.g. Mind, school support
What are key features of generalised anxiety disorder?
At least 6m of excessive, hard to control worrying
How is GAD investigated in children?
Clinical diagnosis
BEDSIDE: GAD-7
BLOODS: TFTs
What is OCD and how does it present?
Recurrent obsessional thoughts or compulsive acts
OBSESSIONS = involuntary thoughts, images, impulses, self-recognised as product of own mind
COMPULSIONS = repetitive mental operations or physical acts, compelled to perform in response to obsession
What questions are important to ask in a OCD history?
- Do you wash or clean a lot?
- Do you check the time a lot?
- Is there any thought that keeps bothering you?
- Do your daily activities take a long time to finish?
- Are you concerned about putting things in a special order or are you very upset by a mess?
- Do these problems trouble you?
How is OCD investigated?
BEDSIDE: Yale-Brown OCD scale
BLOODS: FBC, TSH
How is OCD in children managed?
PSYCHO: family therapy, CBT
BIO: high dose SSRI (sertraline)
SOCIAL: charities like Mind, contact w/schools
What is Tourette’s?
Type of tic disorder involving both motor and vocal tics for over 1 year (before age of 18).
Tic = fast, repetitive muscle movements that result in sudden and difficult to control body jolts or sounds.
What conditions is Tourette’s associated with?
ADHD, OCD
How is Tourette’s managed?
Self-help e.g. sleep, stress mx, family support & avoidance of triggers
If very debilitating = Comprehensive Behavioural Intervention for Tics (CBIT), namely Habit Reversal Therapy
If CBIT ineffective = medication
- alpha-2-agonist: Clonidine
- antipsychotic or tetrabenazine or topiramate
- botulinum toxin
What are the four types of child abuse?
Physical
Sexual
Emotional
Neglect
What are RFs for child abuse?
CHILD: crying persistently, children <4, disability, LBW, chronic ill health, prematurity, being unwanted, unable to fulfill parental expectations
ADULT: mental illness, PND, lack of support network, drugs, own child abuse, alcohol misuse, LD, criminal activity, financial difficulties
How should a case of suspected child abuse be managed?
Seek HELP: senior + safeguarding
Clear documentation
Keep child safe: admit
When should NAI be suspected?
- mechanism of injury not compatible w/injury sustained
- developmental stage inconsistent w/injury history
- little or no explanation for sig. injury
- inconsistent histories
- delayed presentation
- recurrent injuries
- parents reaction inappropriate to situation
What are some ways NAI may present?
bruising, bites, fractures (spiral fracture, long bones), drowsiness (subdural haematoma), eye trauma, neglect, failure to thrive, STIs, recurrent UTIs, history not consistent, torn frenulum labii superioris, glove and stocking burn, anal fissures, encopresis
How should NAI be investigated?
Thorough examination and documentation
Check Child Protection Register
Skeletal survey, CT head scan, Bloods and bone profile (rule out leukaemia, ITP, haemophilia/coagulopathies), Fundoscopy (retinal haemorrhages)
How should NAI be managed?
Senior help and safeguarding team
ADMIT
Child in need plan = a plan made to give children extra support for health, safety +/- developmental issues
Child protection plan = plan made to protect children thought to be at risk of significant harm
What is Shaken Baby Syndrome?
Form of NAI, more common in children <2yo, caused by shaking of infant e.g. due to frustration because lots of crying
What is the triad assoc. with shaken baby syndrome?
- Retinal haemorrhages
- Encephalopathy (cerebral swelling)
- Subdural haematoma
At what age is a person assumed to have capacity to consent to treatment by default?
Children 16 or over
What should be done in a legal sense if a child is seeking treatment aged 12-16?
Check if Gillick competent
Can consent, but cannot refuse, treatment
How many persons with parental responsibility are needed to give consent to treatment?
Only ONE person w/parental responsibility needed
Who is responsible to make decisions in best interests of the child?
Responsibility of the clinician
What should a clinician do if there is strong disagreement with parents about what to do for a child?
Apply for appeal from the Court of Protection
How can decision making for families be facilitated in practice?
- always involve the child in decision-making
- speak clearly, explain things understandably
- Play specialists are useful esp. for younger children
- elicit ICE
- offer discussion w/ and w/o parents
