Paeds fifth yr Flashcards
What is cerebral palsy?
permanent neurological problems resulting from damage to the brain around the time of birth.
not a progressive condition, however the nature of the symptoms and problems may change over time during growth and development
huge variation in the severity and type of symptoms
Causes of cerebral palsy?
Antenatal:
Maternal infections
Trauma during pregnancy
Perinatal:
Birth asphyxia
Pre-term birth
Postnatal:
Meningitis
Severe neonatal jaundice
Head injury
Types of cerebral palsy?
Spastic: hypertonia (increased tone) and reduced function resulting from damage to upper motor neurones
Dyskinetic: problems controlling muscle tone, with hypertonia and hypotonia, causing athetoid movements and oro-motor problems. This is the result of damage to the basal ganglia.
Ataxic: problems with coordinated movement resulting from damage to the cerebellum
Mixed: a mix of spastic, dyskinetic and/or ataxic features
Spastic CP is also known as pyramidal CP. Dyskinetic CP is also known as athetoid CP and extrapyramidal CP.
Features of cerebral palsy?
Infants at risk need follow up for S+S that may develop
Failure to meet milestones
Increased or decreased tone, generally or in specific limbs
Hand preference below 18 months is a key sign to remember for exams
Problems with coordination, speech or walking
Feeding or swallowing problems
Learning difficulties
Hemiplegic / diplegic gait: indicates an upper motor neurone lesion
Broad based gait / ataxic gait: indicates a cerebellar lesion
High stepping gait: indicates foot drop or a lower motor neurone lesion
Waddling gait: indicates pelvic muscle weakness due to myopathy
Antalgic gait (limp): indicates localised pain
Complications:
Learning disability
Epilepsy
Kyphoscoliosis
Muscle contractures
Hearing and visual impairment
Gastro-oesophageal reflux
Management of cerebral palsy?
MDT approach - manage symptoms and maximise function
PT - maximise function, strengthen muscles
OT - everyday activities, adaptations
SALT - NG, PEG
Dieticians - PEG
Orthopaedic surgeons - release contractures or lengthen tendons
Paediatricians - optimise medication - Muscle relaxants (e.g. baclofen) for muscle spasticity and contractures
Anti-epileptic drugs for seizures
Glycopyrronium bromide for excessive drooling
Social workers
Charity and support groups
What is achondroplasia?
most common cause of disproportionate short stature (dwarfism). It is a type of skeletal dysplasia.
achondroplasia gene, fibroblast growth factor receptor 3 (FGFR3), is on chromosome 4.
Achondroplasia results from either a sporadic mutation or inheritance of an abnormal copy of this gene.
autosomal dominant pattern
Homozygous gene mutation is fatal in the neonatal period. So patients with achondroplasia are heterozygous
causes abnormal function of the epiphyseal plates (growth plates). This restricts the bone growth in length, leading to short bones and short stature.
Features of achondroplasia?
disproportionate short stature. The average height is around 4 feet - limbs more affected - femur and humerus. Spine not affected. Trunk normal
Intelligence and life expectancy are not affected by the condition
Short digits
Bow legs (genu varum)
Disproportionate skull
Foramen magnum stenosis
Skull base grows and fuses via endochondral ossification - causing flattened mid-face and nasal bridge. Cranial vault grows and fuses via membranous ossification (so isn’t affected by achondroplasia) and leads to normal sized vault and frontal bossing
Associations:
Recurrent otitis media, due to cranial abnormalities
Kyphoscoliosis
Spinal stenosis
Obstructive sleep apnoea
Obesity
Foramen magnum stenosis can lead to cervical cord compression and hydrocephalus
Management of achondroplasia?
MDT approach
Paediatricians
Specialist nurses
Physiotherapists
Occupational therapists
Dieticians
Orthopaedic surgeons
ENT surgeons
Geneticists
Pt’s tend to have normal life expectancy if they are not affected by complications. Can become overweight. Psychosocial implications.
What is ADHD?
ADHD ss a condition incorporating features relating to inattention and/or hyperactivity/impulsivity that are persistent. Most people have some spectrum of hyperactivity/impulsivity - ADHD is extremes
Features should be consistent across various settings. I.e., not hyperactive at school and calm at home - would be environmental problem
Features of ADHD?
All the features of ADHD can be part of a normal spectrum of childhood behaviour. When many of these features are present and it is adversely affecting the child, ADHD can be considered:
Very short attention span
Quickly moving from one activity to another
Quickly losing interest in a task and not being able to persist with challenging tasks
Constantly moving or fidgeting
Impulsive behaviour
Disruptive or rule breaking
Management of ADHD
detailed assessment should be carried out by a specialist in childhood behavioural problems before a diagnosis is made - referral to CAHMS
Parental and child education
Establishing a healthy diet and exercise - and eliminating any triggers
Medication if conservative hasn’t worked or if severe - CNS stimulants - Methylphenidate (“Ritalin“) - height and weight monitored every 6m
Dexamfetamine
Atomoxetine
and baseline ECG as drugs are cardiotoxic
What is caput succedaneum?
describes oedema of the scalp at the presenting part of the head, typically the vertex. This may be due to mechanical trauma of the initial portion of the scalp pushing through the cervix in a prolonged delivery or secondary to the use of ventouse (vacuum) delivery.
soft, puffy swelling due to localised oedema
crosses suture lines
no Tx needed
What is cephalohaematoma?
seen as a swelling on the newborns head. It typically develops several hours after delivery and is due to bleeding between the periosteum and skull. The most common site affected is the parietal region
Jaundice may develop as a complication.
A cephalohaematoma up to 3 months to resolve.
Summary of whooping cough?
Gram-negative Bordetella pertussis
Routinely immunised at 2, 3, and 4 m and 3-5 yrs
Sx - catarrhal please (URTI Sx, lasts 1-2wks)
paroxysmal phase (cough increases in severity, cough worse at night and after feeding, end with vomiting and central cyanosis, inspiraotiry whoop, spells of apnoea, persistent coughing (subconjunctival haemorrhages) - lasts 2-8 weeks)
convalescent phase (cough subsides over weeks to months)
Dx - per nasal swab for Bordtella, may take several days-wks to come back, PCR and serology
Tx - under 6m - admit, notifiable disease, oral macrolide if cough within previous 21 days, household contacts offered ABx prophylaxis, school exclusion for 48 hours commencing ABx
Complications - subconjunctival haemorrhage, pneumonia, bronchiectasis, seizures
Women 16-32 weeks pregnant will be offered vaccine
Summary of transient tachypnoea of newborn?
Commonest cause of respiratory distress in the newborn period
Caused by delayed resorption of fluid in the lungs
Common following C-sections - possibly due to lung fluid not being squeezed out during passage through birth canal
CXR - hyperinflation of lungs and fluid in horizontal fissure
Tx - observation, supportive care, supplementary O2
Usually settles within 1-2 days
Summary of scarlet fever?
reaction to erythrogenic toxins produced by Group A haemolytic streptococci (usually Streptococcus pyogenes)
Common children 2-6yrs, peak incidence at 4yrs
Spread via respiratory route
Sx - fever (24-48hours), malaise, headahce, N/V, sore throat, strawberry tongue, rash (fine punctate erythema on torso, flushed appearance, rough sandpaper texture, desquamation later in course of illness)
Dx - throat swab, but ABX commented immediately
Tx - oral penicillin V for 10 days, if not, azithromycin, can return to school 24 hours after ABx, notifiable disease
Complications - otitis media, rheumatic fever, acute glomerulonephritis, invasive complications (e.g. bacteraemia, meningitis, necrotizing fasciitis) are rare but may present acutely with life-threatening illness
Summary of pneumonia in children?
S. pneumonia is most likely causative agent
Tx:
Amoxicillin is first-line for all children with pneumonia
Macrolides may be added if there is no response to first line therapy
Macrolides should be used if mycoplasma or chlamydia is suspected
In pneumonia associated with influenza, co-amoxiclav is recommended
Summary of meconium aspiration syndrome?
refers to respiratory distress in the newborn as a result of meconium in the trachea
more common in post-term deliveries
RF - history of maternal hypertension, pre-eclampsia, chorioamnionitis, smoking or substance abuse.
Summary of measles?
Rarely seen in developed word due to immunisation programmes
RNA paramyxovirus, one of the most infectious known viruses, spread by aerosol transmission, infective from prodrome until 4 days after rash starts, incubation period = 10-14 days
Prodrome: irritable, conjunctivitis, fever
Koplik spots: white spots (‘grain of salt’) on buccal mucosa
Rash: starts behind ears then to whole body, discrete maculopapular rash becoming blotchy & confluent
Diarrhoea in 10% of pt’s
Ix - IgM antibodies can be detected within a few days of rash onset
Tx - supportive, admission in immunosuppressed or pregnancy pt’s, notifiable disease
Complications - otitis media: the most common complication, pneumonia: the most common cause of death, encephalitis: typically occurs 1-2 weeks following the onset of the illness)
subacute sclerosing panencephalitis: very rare, may present 5-10 years following the illness, febrile convulsions, keratoconjunctivitis, corneal ulceration, diarrhoea, increased incidence of appendicitis, myocarditis
Contacts - MMR is child not immunised comes in contact with measles - given within 72 hours
Summary of Kawasaki disease?
Type of vasculitis predominately seen in children
Sx - high grade fever lasts for >5 days, resistant to antipyretics, conjunctival injection, bright red, cracked lips, strawberry tongue, cervical lymphadenopathy, red palms of hands and soles of feet which later peel
Clinical diagnosis
Tx - high dose aspirin, IV immunoglobulin, ECHO - screening for coronary artery aneurysms
Complications - coronary artery aneurysm
Summary of hand, foot and mouth disease?
Coxsackie A16, also enterovirus 71
Very contagious - outbreaks at nursery
Mild systemic upset: sore throat, fever
Vesicles in the mouth, followed by vesicles on the palms and soles of the feet
Tx - symptomatic Tx (hydration, analgesia, reassurance), no need for exclusion from school
Traffic light system for feverish illness?
Under 5
Assesses: colour, activity, respiratory, circulation and hydration and other
Green (low risk) - normal colour, responds normally to social cues, content/smiles, stays awake or awakens quickly, strong normal cry/not crying, normal skin and eyes, moist mucous membranes, no amber or red signs
Amber (intermediate risk) - pallor, not responding normally to social cues, no smile, wakes only with prolonged stimulation, decreased activity, nasal flaring, tachypnoea, O2 <95%, crackles in chest, CRT >3, dry mucous membranes, poor feeding, reduced urine output, age 3-6m >39, fever >5 days, rigours, swelling of limb/joint, non-weight bearing limb
Red - pale/mottled/ashen/blue, no response to social cues, appears ill to HCP, does not wake or if roused does not stay awake, weak, high-pitched or continuous cry, grunting, tachypnoea, moderate or severe chest indrawing, reduced skin turgor, age <3m temp >38, non-blanching rash, bulging fontanelle, neck stiffness, status epilepticus, focal neurological signs, focal seizures
Tx:
Green - managed at home with appropriate advice
Amber - provide parents with a safety net or refer to a paediatric specialist for further assessment
Red - refer child urgently to a paediatric specialist
Summary of CF?
AR - causing increased viscosity of secretions (e.g. lungs and pancreas)
defect in the cystic fibrosis transmembrane conductance regulator gene (CFTR) - which codes cAMP regulated chloride channel
Features - meconium ileum, recurrent chest infections, malabsorption (steatorrhoea, FTT), liver disease, short stature, DM, delayed puberty, rectal prolapse, nasal polyps, male infertility, female sub fertility
Organisms that colonise CF pt’s - Staph aureus, Pseudomonas aeruginosa, Burkholderia cepacia, Aspegillus
Dx - sweat test (>60)
Tx - MDT approach, chest PT and postural drainage, high calorie diet, minimise contact with each other, vitamins, pancreatic enzyme supplements, lung transplantation
Lumacaftor/Ivacaftor (Orkambi) - if homozygous for delta mutation
Summary of croup?
URTI seen in infants and toddlers. Para influenza virus
Peak incidence - 6m-3y, more common in autumn
Sx - stridor (due to laryngeal oedema and secretions), barking cough worse at night, fever, coryza Sx
Mild, moderate and severe
CKS suggest admitting any child with moderate or severe croup, or <6m, known upper airway abnormalities, uncertainty about diagnosis
Ix - clinic diagnosis, CXR - subglottic narrowing (steeple sign)
Tx - single dose of oral dexamethasone (0.15mg/kg) to all children regardless of severity
emergency Tx - high-flow O2 and nebuliser adrenaline
Summary of chickenpox?
Varicella zoster virus
Fever initially
Itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular
Systemic upset is usually mild
Tx - keep cool, trim nails, calamine lotion, school exclusion until lesions crusted over, immunocompromised patients and newborns with peripartum exposure should receive varicella zoster immunoglobulin (VZIG). If chickenpox develops then IV aciclovir should be considered
Complication - secondary infection (NSAIDs increase risk, necrotising fasciitis), pneumonia, encephalitis, disseminated haemorrhage chickenpox, arthritis, nephritis, pancreatitis
Summary of mumps?
RNA paramyxovirus
tends to occur in winter and spring
Fever, malaise, muscular pain
Parotitis (‘earache’, ‘pain on eating’): unilateral initially then becomes bilateral in 70%
Tx - rest, paracetamol, notifiable disease
Complications - orchitis, hearing loss, meningoencephalitis, pancreatitis
Summary of rubella?
caused by togavirus
Rash: pink maculopapular, initially on face before spreading to whole body, usually fades by the 3-5 day
Lymphadenopathy: suboccipital and postauricular
risk of congenital rubella syndrome - sensorineural deafness, congenital cataracts, congenital heart disease, growth retardation,
Summary of erythema infecitosum?
Fifth disease/slapped cheek/parvovirus B19
Sx - lethargy, fever, headache
Slapped cheek rash spreading to proximal arms and extensor surfaces
Summary of bronchiolitis?
Commonly respiratory syncytial virus. Other causes - mycoplasma, adenoviruses. More serious if bronchopulmonary dysplasia, congenital disease or CF
Most common cause of LRTI in <1 yr, peak incidence at 3-6 months as material IgG provides protection
Higher incidence in winter
Features - coryza Sx precede, dry cough, breathlessness, wheezing, feeding difficulties
Immediate referral if: apnoea, looks seriously unwell, severe respiratory distress, central cyanosis, O2 <92% on air
Consider referral - RR >60, difficulty breastfeeding or inadequate oral fluid intake, clinical dehydration
Ix - immunofluorescence of nasopharyngeal secretions
Tx - supportive, humidified O2 if <92%, NG feedings, suction of upper airway secretions
Summary of acute epiglottitis?
Haemophilus influenzae type B
Features - rapid onset, high temp, generally unwell, stridor, saliva drooling, tripod position
Dx - direct visualisation (only by senior/airway trained staff), X-rays (lateral - thumb sign)
Tx - immediate senior involvement (emergency airway support), endotracheal intubation may be necessary to protect airways, DO NOT examine chrome, O2, IV ABx
Summary of roseola infantum?
Sixth disease
Human herpes virus 6
incubation 5-15 days
children 6m-2y
Sx - high fever lasting a few days, followed by: maculopapular rash, Nagayama spots: papular enanthem on the uvula and soft palate, febrile convulsions occur in around 10-15%, diarrhoea and cough are also commonly seen
School exclusion not needed
School exclusion for paediatric illness?
No exclusion - conjunctivitis, slapped cheek, roseola, mononucleosis, head lice, threadworms, hand foot and mouth
24hrs after commencing antibiotics - scarlet fever
2 days after ABx (or 21 days from onset if no ABx) - whooping cough
4 days from onset of rash - measles
5 days from onset of rash - rubella
all lesions crusted over - chickenpox
5 days from onset of swollen glands - mumps
until Sx have settled for 48 hours - D&V
Until lesions are crusted and healed, or 48 hours
after commencing antibiotic treatment - impetigo
until treated - scabies
until recovered - influenza
