History - sections Flashcards
(103 cards)
1
Q
How do you define Cerebral Palsy?
A
A group of non-progressive movement disorders affecting movement and posture, resulting from cortical disturbance in the developing fetal or infant brain. These cortical insults may be due to a variety of causes
Important – the lesion is non-progressive. But the motor problems can be progressive, because problems can exacerbate as they grow older, even though the lesion isn’t growing.
2
Q
What are possible causes of Cerebral Palsy?
A
Fetal:
- Hypoxic ischaemic injury
- Intraventricular / Germinal matrix haemorrhage (especially in preterm infants)
- Congenital infections
- Prematurity
Neonatal:
- Hyperbilirubinemia / Kernicterus
- Sepsis / Fever / Infections (meningitis / encephalitis)
- Head trauma
3
Q
How would you classify the different types of CP?
A
(1) By type of motor impairment
- Spastic: Most common (70%), rigidity, UMN picture.
- Ataxic: Least frequent, due to cerebellar lesions, usually presents with intention tremor
- Dyskinetic (atheroid or dystonic): Damage to basal ganglia, usually due to bilirubin encephalopathy/HIE.
- Dystonic: slow, strong contractions with abnormal posturing. Involuntary movements
(2) By distribution
- Hemiplegic
- Diplegic (both legs > arms)
- Quadraplegic
4
Q
How would you support a patient and family with CP?
A
Speech and language therapy - helping with swallowing.
Feeding - might need a gastrostomy tube.
Education - they can have cognitive difficulties
PT and OT - splints and walking aids.
Orthopaedics - they get scoliosis and hip dislocation
Neuro - ↑ risk of epilepsy
Urology - ↑ risk of UTI and incontinence
Resp - ↑ respiratory infections so they get lots of rescue packs
5
Q
What is DDH?
A
Congenital instability of the hip joint resulting in frequent dislocations. You can pick it up with screening tests in newborns (Barlow and Ortolani), or in walking kids as a painless limp.
6
Q
DDH - treatment?
A
Treatment - you give them a Pavlik Harness. Or alternatively you can surgery them within 18 months of birth.
7
Q
How does DMD typically present?
A
- Usually diagnosed 4-5 years of age.
- Gross motor delay with ‘clumsiness’, waddling gait, proximal myopathy
- Reduced or absent reflexes, although ankle jerk often preserved
- Gower’s sign = walking up legs (proximal myopathy)
- Calf pseudohypertrophy - muscle replaced by fat and fibrous tissue
8
Q
What investigations would you do in suspected DMD?
A
- ↑ Creatinine Kinase
- Genetic studies
- EMG (myopathy pattern)
- Muscle biopsy (absent dystrophin on immunohistochemistry). But it’s distressing, so avoid it if there’s a positive genetic result.
9
Q
What’s the genetic/pathophysiology stuff in Duchenne Muscular Dystrophy?
A
- X-linked recessive, 1/4000 male infants.
- 1/3 of cases are new mutations
- Xp21 = dystrophin. Dystrophin maintains integrity of muscle cell wall.
- Absence of dystrophin leads to progressive muscle cell damage.
10
Q
What is Becker’s Muscular Dystrophy?
A
Similar to DMD but milder.
The signs and symptoms of Becker muscular dystrophy are usually milder and more varied. In most cases, muscle weakness becomes apparent later in childhood or in adolescence and worsens at a much slower rate
DMD kills you in your 20s, Becker kills you in your 40s.
11
Q
What brain tumours can cause gait problems in kids?
A
11
Q
What brain tumours can cause gait problems in kids?
A
12
Q
When would bruising in a child be a cause for concern from a safeguarding POV?
A
Dodgy patterns, child too young to be mobile, incidental presentation of bruising (kid comes in for something else and you happen to notice it), kid presenting late for another condition esp in A and E (avoid regular contact with the same doctor), other stigmata of NAI, child too friendly to you/weird around parents.
13
Q
What is the most common cause of acute hip pain in children?
A
Transient synovitis – child afebrile, decreased RoM, particularly internal rotation, pain possibly referred to knee, onset post some other mild infection eg. sore throat
14
Q
What is the most common cause of acute hip pain in children?
A
Transient synovitis – child afebrile, decreased RoM, particularly internal rotation, pain possibly referred to knee, onset post some other mild infection eg. sore throat
15
Q
What condition may develop post transient synovitis in a small proportion of children?
A
Perthes disease
16
Q
What condition may develop post transient synovitis in a small proportion of children?
A
Perthes disease
17
Q
What is Perthes disease
A
avascular necrosis of capital femoral epiphysis of femoral head. Mainly affects 5-10yo males. Can be bilateral in some. Acute presentation has a painful limp- in chronic Perthes is often a painless limp.
18
Q
What investigations may be requested in suspected Perthes disease?
A
- Frog view X-rays should be requested. Increased density in femoral head, subsequent fragmentation and irregularity.
- MRI or bone scan
19
Q
What is the treatment of Perthes disease?
A
Rest, physio, some may require traction.
Prognosis good, particularly for those 6yo and under with less
than half of the epiphysis involved.
Worst outcome is hip arthroplasty.
20
Q
In obese 15 year old males with only a minor trigger, what limp cause is most likely?
A
SUFE – slipped capital femoral epiphysis
21
Q
What is SUFE – slipped capital femoral epiphysis
A
Association with metabolic endocrine abnormalities
eg. hypothyroidism and hypogonadism
Hip pain may be referred to the knee
Restricted abduction and internal hip rotation
22
Q
How best is SUFE managed?
A
Surgical Mx – pin fixation in situ, prevent femoral head displacement postero-inferiorly
Fix other side as well as high chance of bilateral slip
Prompt Tx required to prevent avascular necrosis
23
Q
What top differential diagnoses are possible in a child presenting with a painful, swollen, hot and erythematous joint?
A
- Reactive arthritis
- Juvenile idiopathic arthritis
- Septic arthritis
24
What is Reactive arthritis
Most common form of childhood arthritis, transient joint swelling often of ankles/knees, usually follows extra-articular infection eg. Psoriasis, Viral, Salmonella, gonococcus, Lyme disease. Low grade fever. No Tx or NSAIDs. Good prognosis.
25
What is Juvenile Idiopathic Arthritis
Most common chronic inflammatory joint disease in children/adolescents. Many subtypes, can be further classified according to Rheum factor and HLA B27 tissue type.
Morning joint pain, intermittent limp, reports avoidance of previous activity rather than lots of pain.
26
What is Juvenile Idiopathic Arthritis
Most common chronic inflammatory joint disease in children/adolescents. Many subtypes, can be further classified according to Rheum factor and HLA B27 tissue type.
Morning joint pain, intermittent limp, reports avoidance of previous activity rather than lots of pain.
27
What is Septic arthritis
Most common in children under 2yo. Often from haematogenous spread or from osteomyelitis. Single joint affected, hip of particular concern. Post neonatal period most commonly Staph A or H infuenzae. May have underlying immunodeficiency or Sickle Cell. Joint effusion may be detectable. Difficult to diagnose in toddlers as joint well covered by subcut fat.
FBC, cultures, joint aspiration, X-ray to exclude bony injury
28
What underlying disease must be considered in a patient with Juvenile Idiopathic Arthritis in many joints with systemic involvement?
Connective Tissue Disease – E-D or Marfan’s
29
If JIA is not treated adequately, what long term complications could develop?
- Uncontrolled disease activity, bone expansion from overgrowth, may cause leg lengthening or valgus deformity due to knee effects, wrist deformity, advancement of bone age
- Chronic anterior uveitis, flexion contracture of joints, growth failure
30
Which cause of limp/joint pain is particularly important to diagnose asap in children?
Septic Arthritis.
S Aureus (the most likely causative organism) - rapidly and irreparably destroy joint cartilage.
31
How is septic arthritis managed?
Aspirate/swab and culture
Blood cultures
IV antibiotics
Joint washout
32
What could be used to differentiate between Transient Synovitis and Septic Arthritis?
Aspirate joint + culture
Kocher’s criteria - point given for each of the four following criteria:
Non-weight-bearing on affected side
Erythrocyte sedimentation rate > 40
Fever > 38.5 °C
White blood cell count > 12,000
4 is most severe → 99% likelihood of septic A.
33
What investigations are appropriate in a child with limp/joint pain?
FBC / ESR / CRP
Blood Cultures
X-rays
MRI/bone scan may be necessary
Aspiration and culture of joint effusions
34
What subtle sign may be seen on tibial x-ray indicative of Toddler’s Fracture?
Toddler fractures =minimally/undisplaced spiral fractures
Usually of the tibia
Potentially difficult diagnosis to establish on account of both the symptoms and imaging findings being subtle eg. periosteal reaction
35
How is DDH managed?
May self resolve
Pavlik harness used for fixation
Surgical measures appropriate if harnessing is unsuccessful
36
How would you manage transient synovitis?
Ibuprofen
Massaging affected leg and applying heat packs
2 weeks to spontaneously recover
Don't exercise aggressively during these 2 weeks
37
Naevus simplex/stork mark
Common - 40% newborns
Flat, pink macules often in midline face or posterior neck, prominent with crying
Usually fade within 2 years
Caused by abnormal capillary dilation
38
Naevus flammeus/port wine stain
Macular lesion with midline cut-off
Grows during childhood and doesn’t regress
If in v1 distribution- ?Sturge Weber
39
Cavernous haemangioma (strawberry mark)
Appears within weeks of birth- rapid proliferation in first 6-12 months before stabilisation and involution in next 10 years (involution = shrinkage of an organ due to old age)
Treated if ulcerated, periocular, airway obstruction etc- oral propranolol – vasoconstriction reduces the mark.
40
Erythema toxicum
Common rash appearing day 2-3 after birth
White vesicles at centre of erythematous base
Baby otherwise well
Reassurance
41
Milia
White pimples on nose and cheeks
Benign
Self-resolving
42
Mongolian blue spot
Dark blue pigmentation- often on buttocks
Document- can be mistaken for NAI
43
Nappy Rash
Convex surfaces of buttocks, lower abdomen and thighs- flexures spared
Frequent changing of nappies and emollients
Candida may complicate a nappy rash – flexural involvement (groin) and satellite lesions (random lesions outside the nappy area)
44
Seborrheic dermatitis = cradle cap
First 2 months of life
Begins on scalp as yellow crusting.
Spreads - red scaly rash on trunk and nappy area
Increased risk of subsequent atopic eczema
Cause = some kind of fungus.
Symptoms = NOT ITCHY (contrast with eczema). It looks horrendous on the head, but the baby isn’t bothered.
Rx = olive oil or antifungal shampoo.
45
Atopic eczema
Itchy erythematous patches on flexure surfaces. But eczema in toddlers/pre-schoolers can often affect extensor surfaces of joints.
On infants – face (cheeks usually)
Excoriations from scratching
Most subsides by age 7
Treated with frequent emollient use, bath/soap substitutes and topical steroids
Complications - infection (bacterial, HSV - eczema herpeticum – potentially life-threatening, admit for IV acyclovir)
Immunomodulators in >2
Adjuncts: anti-histamines
46
Impetigo
Highly contagious
Staph or group A strep
Begins as red sores - become vesicles that break- honey-coloured scab
Bullous impetigo - staph exfoliating toxin A
Spread by contact
Topical fusidic acid or oral fluclox / erythromycin if pen allergic.
Classically “golden crusting” rash.
47
Staphylococcal Scalded Skin Syndrome (SSSS)
Caused by staph toxin B
Nikolsky positive – gentle pressure peels off areas of epidermis
May have fever
Mx = usually admit, IV flucloxacillin.
48
Scarlet fever
Group A strep pharyngitis complication
Red and feels like sandpaper, blanching 12-72hr after onset of fever
Affects chest, armpits and spreads outwards
Tongue - red and bumpy (strawberry)
Long-term complications = kidney disease, rheumatic heart disease, and arthritis
49
Meningococcal rash
Non-blanching, purpuric lesions
Meningitis!!
50
Hand, Foot and mouth
Cosackie normally
Vesicles on face, palate that evolve into ulcers
Vesicles and maculopapular rash on palms of hands and soles of feet
Self-limiting – subsides in a few days
You can still go to school. They might go off school because they feel bad and they can’t eat properly, but they shouldn’t be excluded from school on infectious grounds.
51
HSV
Primary infection often asymptomatic. But if not - painful vesicles on lips, gums and anterior tongue and hard palate (gingivostomatitis) with fever, anorexia for up to 2 weeks
Usually seen in those 10 months to 3 years
Remains latent – reactivation- cold sore
May cause erythema multiforme or eczema herpeticum in those with eczema
More serious in neonates vertically infected during delivery- encephalitis
52
VZV = Chicken Pox
Small, itchy blisters which scab over
Fever, lethargy
Infectious until scabs form
Reactivation - shingles
53
EBV
Often asymptomatic
Can cause infectious mononucleosis - some get maculopapular rash
Don’t give amoxicillin. If you do, they get a huge rash
54
HHV6/7
Roseola infantum
High fever followed by generalised macular rash
Common cause of febrile convulsions
55
Measles
Fever
Conjunctivitis and coryza
Cough
Koplik spots - starting day 2- white spots on buccal mucosa
Rash day 3-4- begins behind ears and spreads- maculopapular
Complications: encephalitis, SSPE (subacute sclerosing panencephalitis)
Lymphadenopathy – submandibular (this is how you differentiate measles from rubella)
56
Rubella
Malaise and fever
Small petechial lesions on soft palate - forchleimer spots
Rash from day 7 - pink-red macular and discrete lesions. Fades by 2nd day
Complications - congenital rubella (fetal abnormalities), thrombocytopenia, arthritis)
Lymphadenopathy – post-auricular & suboccipital
57
Mumps
Painful parotid swelling
Fever, muscle pain, headache, tiredness
58
Parvovirus B19
Slapped cheek/fifth disease/erythema infectiosum
Fever, malaise, rash on cheeks
Aplastic crisis
Fetal hydrops (fluid overload) if vertical transmission in utero
59
Molluscum contagiosum
Translucent papules with punctum
Last 6-12 months
Transmitted by contact
Resolves on its own
60
Ringworm
Dermatophyte infection – ring with central clearing
E.g. tinea corporis (body), tinea capialis (head)
Scaly patch with central clearing
Treated with topical antifungal
61
Candida
May complicate nappy rash - involves flexer surfaces
62
Scabies
Mite saroples scabei
Intensely itchy red papules
Burrow marches in web spaces
Treated with insecticide lotions - applied jawline to feet and left on overnight. All family members treated.
63
Erythema nodosum
Red/blue lumps on shins
Lasts 2-3weeks
Seen in:
Strep infection
Idiopathic
TB
IBD
Causes = NODOSUM
NO cause in 60%, Drugs, Oral contraceptives, Sarcoidosis, UC (Crohns, Behcet’s), Microbiology (bacterial, viral, TB, deep fungal, any chronic infection)
64
Erythema multiforme
Caused by
HSV and other virsues
Target lesions
Lasts ~72hours
65
Kawasaki
Vasculitis
Dry blistering lips and oral cavity
Diffuse red rash on palms and soles
Strawberry tongue
66
Henoch-Schonlein Purpura
IgA mediated
Erythematous, macular or urticarial rash that progresses to blanchable purpura, and then palpable purpura
Often on extensors and buttocks
67
NF1 – mnemonic = BLANCO1
Bone lesions
Leisch nodules (iris)
Axillary freckling
Neurofibromas
Café au lait
Optic glioma
1
68
Tuberous sclerosis
Baby with a fit with a hypopigmented patch.,
ASH LEAF mnemonic
Ash-leaf patches (hypopigmented areas)
Shagreen spots (localise leathery thickenings)
Heart rhabdomyosarcoma
Lung hamartoma
Epilepsy from cortical tuber
Angiomyolipoma in kidney
Facial angiofibroma
69
FSGS- what is it?
focal-segmental glomerulosclerosis
can evolve from other kidney disease, vasculitis, or infection
70
Steroid-resistant nephrotic syndrome
- FSGS
- MPGN
- Membranous nephropathy
- Congenital nephrotic syndrome
71
How would you treat a kid with a suspected anaphylaxis reaction?
IM adrenaline 1:1000 (higher concentrations than used for cardiac arrest)
can give repeat doses or infusion
High-flow oxygen
Assess airway – consider intubation for laryngospasm/oedema, reduced GCS
chlorphenamine (antihistamine)
hydrocortisone
consider bronchodilators (salbutamol nebs) for bronchospasm
72
What are the Coombs tests and Kleihauer test and how do you use them to prevent haemolytic disease of the newborn?
Direct Antiglobulin Test – take washed red blood cells and look for attached antibodies (with a second anti-Fc antibody)
Indirect Antiglobulin Test – take serum and see if it contains antibodies that bind to red blood cells and haemolyse them
IAT is used to check maternal serum for anti-D antibodies so you don't need to sample the fetal blood for a DAT
DAT is to see if a haemolytic anaemias has auto-immune cause
Kleihauer test can quantify the fetal RBCs in the maternal circulation from “sensitising events” (bleeds, procedures), to calculate anti-D doses
73
What are the indications for kidney biopsy in Nephrotic disease?
Steroid resistant
Age <1
Family history
Signs, Symptoms or tests indicate secondary cause (ie ANA, facial rash > lupus)
74
What are the risk factors for early onset neonatal sepsis?
PROM (>18h, especially if also prem)
Signs of maternal infection (fever, chorioamnionitis, UTI)
Vaginal GBS or previous infant with GBS
Preterm labour
Fetal distress
Skin and mucosal breaks
75
What are the risk factors for Late onset (>48h) neonatal sepsis?
Central lines and catheters
Congenital malformation (eg spina bifida)
Severe illness, malnutrition, immunodeficiency
76
What is the differential of early onset jaundice?
Haemolysis (unconjugated) - Rhesus haemolytic disease, ABO incompatibility, G6PD deficiency, Spherocytosis
Sepsis (conjugated)
77
DDX of persistent jaundice?
Biliary atresia (conjugated)
Breast milk jaundice (unconjugated) (disappears by 4-5w of age)
Infection (esp. UTI)
Congenital hypothyroid (should be discovered on Guthrie)
78
What is the consequence of jaundice?
Depends if conjugated or unconjugated. Unconjugated can cross BBB and cause kernicterus (irreversible brain damage). Conjugated cannot cross BBB so does not cause harm by itself, but is usually the result of a pathological process that requires sorting (i.e. biliary atresia).
79
What are Downs babies at increased risk of?
Duodenal atresia
Squint
Hypothyroidism
Leukemia
Hirschsprungs
Alzheimers
80
Non-infectious causes of high temperature:
- Heatstroke
- Heat exhaustion
81
General advice for enuresis?
Manage expectations: it’s common, it’s not their fault, don’t use punishments
Behavioural: star charts (for toileting behaviours, not for dry at night)
82
What are your differentials for secondary enuresis?
Psychological/emotional problems
Sexual abuse
UTIs
Diabetes mellitus
Threadworm infection
83
Management of enuresis in <5y?
Manage expectations: it’s common, it’s not their fault, don’t use punishments, have they toilet trained their child? If not, why not?
Behavioural: star charts (for toileting behaviours, not for dry at night)
Take child to the toilet if child wakes at night
Trial: 2 nights in a row w/t nappies if toilet trained in the day for >6m. (+alternative bed protection)
84
When to investigate enuresis in <5y?
Children >2y despite awareness of toileting needs and showing appropriate toileting behaviour are struggling not to wet themselves in day and night.
85
How would you diagnose UTI in children of various ages?
All infants under 3 months with UTI should be referred to paeds, send urine sample for urgent microscopy & culture.
In children 3 months to 3 years, urgent microscopy & culture is the preferred method for diagnosing UTI.
If the child is over 3 years old, dipstick testing for leukocytes & nitrites is as good as microscopy/culture, and so dipstick should be used.
86
Treatment of UTIs
<3m refer to paeds (as for any infection under 3m)
>3m and upper tract: consider referral and treat for 7-10d (e.g. Cephalosporin or co-amoxiclav)
>3m and lower tract: treat 3d with nitrofurantoin or trimethoprim or amoxicillin
87
What is a seizure?
Disruption of normal neurological electrical activity that usually is associated with either a change in behaviour, abnormal movements or other changes in neurological function.
88
What is a febrile convulsion?
Occur in children between 6 months – 6 years of age
Associated with a temperature of 38 degrees or above
In the absence of CNS infection, metabolic imbalance or neurological condition
Children with simple febrile seizures have rapid and full neurological recovery
Diagnosis is clinical and as such no specific investigations are needed
Initial investigations to rule out hypoglycaemia or UTI in infants may be taken as a general precaution
89
What’s the long term prognosis for someone with a febrile convulsion?
Children tend to grow out of them and develop normally
For simple febrile convulsions, there’s no increased risk of epilepsy (assuming they’re developmentally normal and there’s no family history of epilepsy)
90
Convulsions should be managed as per any seizure, with consideration of benzodiazepines to halt a prolonged seizure
However, febrile convulsions usually stop on their own fairly quickly
Treat any source of fever
Consider antipyretics if they have a subsequent temperature. There’s little evidence that paracetamol will ↓ risk of future febrile convulsion. But paracetamol is nice for the kid.
Educate and reassure the family: – Tell parents about risks of recurrence (about 1 in 6) and how to manage future febrile seizures. If it lasts more than 5 minutes, an ambulance should be called. Return to hospital if they have another febrile convulsion during the same illness. Risk of febrile convulsions in another illness = 30%.
After 1 minute, there’s a very low chance of status epilepticus.
After 5 minutes, there’s quite a high chance of status epilepticus. So 5 minutes is the threshold for calling the ambulance.
Let them book in with the epilepsy nurse specialist to discuss things properly if they want.
91
What is a breath-holding attack?
Involuntary episodes where kids are angry → cry → take a deep breath → forget to breathe out → turns blue & extends limbs → goes limp → loses consciousness ± a few convulsive jerks, but no post-ictal phase.
Common in 6 month – 2 year age group
No association with bad parenting. It is not the parent’s fault at all! The kid will grow out of it.
92
What would make you worry that the seizure was due to a space occupying lesion?
Symptoms of headache, raised ICP, behavioural changes, localising neurological signs
Gradual onset (rapid onset of these symptoms would suggest a cerebrovascular lesion)
93
Childhood absence epilepsy (CAE)
Tx
Ethosuximide
Lamotrigine
Sodium valproate
94
Juvenile absence epilepsy (JAE) Tx
Sodium valproate
Lamotrigine
Ethosuximide (if no tonic-clonic seizures)
95
Juvenile myoclonic epilepsy (JME/Janz syndrome) Tx
Sodium valproate
Lamotrigine
96
Benign rolandic epilepsy tx?
Not always necessary as seizures infrequent.
Carbamazepine
Lamotrigine
Levetiracetam
Sodium valproate
97
West syndrome (infantile spasms) tx?
Corticosteroids
Vigabatrin
98
Childhood absence epilepsy (CAE)
Px?
Absence seizures (10-100 per day). 5-15 seconds long. Provoked by hyperventilation.
99
Juvenile myoclonic epilepsy (JME/Janz synd px?
Myoclonic seizures, tonic-clonic seizures, absence seizures
100
Juvenile absence epilepsy (JAE) px
Absence seizures (may have atypical i.e. longer absences). 8/10 have tonic-clonic seizures too.
101
Benign rolandic epilepsy px
Partial seizures whilst asleep – tingling on one side of mouth. May have tonic/clonic movements of mouth which spread to arm/leg. Sometimes progress to tonic-clonic.
102
West syndrome (infantile spasms) px
Sudden flexion forward in a tonic fashion, 1-2 seconds long. Can occur in clusters.
