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
What is autism spectrum disorder?
a neurodevelopmental condition characterized by qualitative impairment in social interaction and communication as well as repetitive stereotyped behaviour, interests, and activities
Symptoms are usually present during early childhood, but may be manifested later.
no cure for ASD, early diagnosis and intensive educational and behavioural management may improve outcomes.
B>G (3:1), prevalence increased over time, 50% of children with ASD have intellectual disability
Features of autism?
broad range of clinical manifestations.
Social communication impairments and repetitive behaviours are present during early childhood (typically evident before 2–3 years of age)
Playing alone or uninterested in other children
Fail to regulate social interaction with nonverbal cues - eye gaze, facial expression, gestures
Failure to form and maintain relationships - become socially isolated
Stereotyped and repetitive motor mannerisms, inflexible adherence to nonfunctional routines or rituals are often seen.
Particular ways about everyday activities
Intellectual impairment or language impairment
ADHD and epilepsy commonly seen in ASD
Also associated with a higher head circumference to the brain volume ratio.
Management of autism?
early diagnosis and early intensive treatment have the potential to affect outcomes
educational and behavioural management, medical therapy, and family counselling.
Early education and behavioural interventions - applied behavioural analysis, ASD preschool program, Treatment and Education of Autistic and Communication related handicapped CHildren (TEACHH), oint Attention Symbolic Play Engagement and Regulation (JASPER)
SSRIs - reduce symptoms like repetitive stereotyped behaviour, anxiety, and aggression
Antipsychotic drugs: useful to reduce symptoms like aggression, self-injury.
Methylphenidate - ADHD
Family support and counselling - Parental education on interaction with the child and acceptance of his/her behaviour
Symptoms of acute asthma in a child?
Worsening SOB
Signs of respiratory distress
Fast RR
Expiratory wheeze
Chest sounds ‘tight’ on auscultation
Reduced air entry
Silent chest
Moderate - peak flow >50%, normal speech, no other features
Severe - peak flow <50%, saturations <92%, unable to complete full sentences, signs of respiratory distress, RR >40 in 1-5yrs, >30 in >5yrs, HR >140 in 1-5yrs, >125 in >5yrs
Life-threatening - peak flow <33%, sats <92%, exhaustion and poor respiratory effort, hypotension, silent chest, cyanosis, altered consciousness/confusion
Management of acute asthma or viral induced wheeze in a child?
Supplementary oxygen if required (i.e. oxygen saturations less than 94% or working hard)
Bronchodilators (e.g. salbutamol, ipratropium and magnesium sulphate)
1 - inhaled/nebulised salbutamol
2 - inhaled or nebuliser ipratropoim bromide
3 - IV Mg sulphate
4 - IV aminophylline
Mild cases - outpatient - with regular salbutamol inhalers via a spacer (e.g. 4-6 puffs every 4 hours).
Moderate to severe cases require a stepwise approach working upwards until control is achieved:
1 - Salbutamol inhalers via a spacer device: starting with 10 puffs every 2 hours
2 - Nebulisers with salbutamol / ipratropium bromide
3 - Oral prednisone (e.g. 1mg per kg of body weight once a day for 3 days)
4 - IV hydrocortisone
5 - IV magnesium sulphate
6 - IV salbutamol
7 - IV aminophylline
8 - anaesthetist and ICU
Steroids to reduce airway inflammation: prednisone (orally) or hydrocortisone (intravenous)
Antibiotics only if a bacterial cause is suspected (e.g. amoxicillin or erythromycin)
Discharging child with acute asthma?
Can be considered when child is on 6 puffs 4 hourly of salbutamol
Reducing regime of salbutamol
Finish course of steroids - typically 3 days total
Safety-net info
Individualised written asthma plan
What is asthma?
chronic inflammatory airway disease leading to variable airway obstruction.
Smooth muscle hypersensitive and responds to stimuli by constricting and causing airflow obstruction - reversible with bronchodilators
atopic condition - FHx
Features of asthma?
Episodic symptoms with intermittent exacerbations
Diurnal variability, typically worse at night and early morning
Dry cough with wheeze and shortness of breath
Typical triggers
A history of other atopic conditions such as eczema, hayfever and food allergies
Family history of asthma or atopy
Bilateral widespread “polyphonic” wheeze heard by a healthcare professional
Symptoms improve with bronchodilators
Features indicating a diagnosis other than asthma?
Wheeze only related to coughs and colds, more suggestive of viral induced wheeze
Isolated or productive cough
Normal investigations
No response to treatment
Unilateral wheeze suggesting a focal lesion, inhaled foreign body or infection
Diagnosis of asthma in a child?
no gold standard test or diagnostic criteria for asthma. Based on Hx and examination
Children are usually not diagnosed with asthma until they are at least 2 to 3 years old. When there is a low probability of asthma and the child is symptomatic, consider referral to a specialist for diagnosis.
intermediate or high probability of asthma, a trial of treatment can be implemented and if the treatment improves symptoms a diagnosis can be made.
Ix:
Spirometry with reversibility testing (in children aged over 5 years)
Direct bronchial challenge test with histamine or methacholine
Fractional exhaled nitric oxide (FeNO)
Peak flow variability measured by keeping a diary of peak flow measurements several times a day for 2 to 4 weeks
Medical therapy of asthma in under 5 years?
Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required
Add a low dose corticosteroid inhaler or a leukotriene antagonist (i.e. oral montelukast)
Add the other option from step 2.
Refer to a specialist.
Medical therapy of asthma in child 5-12 years?
Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required
Add a regular low dose corticosteroid inhaler
Add a long-acting beta-2 agonist inhaler (e.g. salmeterol). Continue salmeterol only if the patient has a good response.
Titrate up the corticosteroid inhaler to a medium dose.
Consider adding:
Oral leukotriene receptor antagonist (e.g. montelukast)
Oral theophylline
Increase the dose of the inhaled cortico
steroid to a high dose.
Referral to a specialist. They may require daily oral steroids.
Medical therapy of asthma in children over 12 years?
Same as adults
Start a short-acting beta 2 agonist inhaler (e.g. salbutamol) as required
Add a regular low dose corticosteroid inhaler
Add a long-acting beta-2 agonist inhaler (e.g. salmeterol). Continue salmeterol only if the patient has a good response.
Titrate up the corticosteroid inhaler to a medium dose.
Consider a trial of an oral leukotriene receptor antagonist (i.e. montelukast), oral theophylline or an inhaled LAMA (i.e. tiotropium).
Titrate the inhaled corticosteroid up to a high dose. Combine additional treatments from step 4, including the option of an oral beta 2 agonist (i.e. oral salbutamol). Refer to specialist.
Add oral steroids at the lowest dose possible to achieve good control under specialist guidance.
What is coeliac disease?
caused by sensitivity to the protein gluten. Repeated exposure leads to villous atrophy which in turn causes malabsorption.
Children normally present before the age of 3 years, following the introduction of cereals into the diet
Associated with - HLA-DQ2, HLA-DQ8
Features of coeliac disease?
May coincide with introduction of cereals (e.g. gluten)
FTT
Diarrhoea
Abdominal distension
Older children - anaemia
Dermatitis herpetiformis
Neuro Sx - peripheral neuropathy, cerebellar ataxia, epilepsy
Some cases not diagnosed until adulthood
Diagnosis of coeliac disease?
Jejunal biopsy
TTG (IgA)
anti-endomysial and anti-gliadin antibodies are useful screening tests
Management of coeliac disease?
Gluten free diet - rice, potatoes, corn
Immunisation - functional hyposplenism
Complications of coeliac disease?
Anaemia
Vitamin deficiency
Osteoporosis
Ulcerative jejunitis
Enteropathy-associated T-cell lymphoma
Non-hodgkin lymphoma
Small bowel adenocarcinoma
What is cows milk protein allergy?
hypersensitivity to the protein in cow’s milk.
This may be IgE mediated, in which case there is a rapid reaction to cow’s milk, occurring within 2 hours of ingestion.
It can also be non-IgE medicated, with reactions occurring slowly over several days.
different to lactose intolerance and cow’s milk intolerance. People with cow’s milk protein allergy do not have an allergy to lactose. Lactose is a sugar, not a protein.
Cow’s milk intolerance is not an allergic process and does not involve the immune system.
Features of cows milk protein allergy?
More common in formula fed children, and in those with personal or FHx of other atopic conditions
Apparent when weaning from breast to formula milk, or food containing milk
GI Sx - bloating, wind, diarrhoea, vomiting
Allergy Sx - urticarial rash, angio-oedema, cough, wheeze, sneezing, watery eyes, eczema
Rarely - anaphylaxis
Management of cows milk protein allergy?
Dx - based on history and examination
Skin prick testing
Avoiding cow’s milk should fully resolve symptoms:
Breast feeding mothers should avoid dairy products
Replace formula with special hydrolysed formulas designed for cow’s milk allergy
Hydrolysed formulas - proteins broken down so no longer trigger immune response. In severe cases infants may require elemental formulas made of basic amino acids (e.g. neocate).
Most outgrow by age 3
Every 6 months - trued on first step of milk ladder
Management of cows milk protein allergy?
Dx - based on history and examination
Skin prick testing
Avoiding cow’s milk should fully resolve symptoms:
Breast feeding mothers should avoid dairy products
Replace formula with special hydrolysed formulas designed for cow’s milk allergy
Hydrolysed formulas - proteins broken down so no longer trigger immune response. In severe cases infants may require elemental formulas made of basic amino acids (e.g. neocate).
Most outgrow by age 3
Every 6 months - trued on first step of milk ladder
Features of cows milk intolerance
GI Sx - bloating, wind, diarrhoea and vomiting
NO allergic Sx
Infants with cow’s milk intolerance will grow out of it by 2 – 3 years. They can be fed with breast milk, hydrolysed formulas and weaned to foods not containing cow’s milk. After one year of age they can be started on the milk ladder.
Sx of constipation in children?
fewer than 3 complete stools a week
hard large stool, rabbit droppings
>1 yr - overflow soiling, large, infrequent that block toilet
distress on passing stool
bleeding associated hard stool
straining
poor appetite improves with passage of large stool
retentive posturing - typical straight-legged, tiptoed, back arching posture
Hx - previous episode of constipation, previous/current anal tissue, painful bowel movements and bleeding ass/ w/ hard stools
Causes of constipation in children?
idiopathic
dehydration
low-fibre diet
medications - opiates
anal fissure
over-enthusiastic potty training
hypothyroidism
Hirschsprungs disease
hypercalcaemia
learning disabilities
CF - meconium ileus
spinal cord lesions
sexual abuse
intestinal obstruction
anal stenosis
cows milk intolerance
psychosocial problems - difficult at home/school
sedentary lifestyle
habitually not opening the bowels - desensitisation of rectum
Red flags for constipation in children?
From birth or first few weeks of life
Meconium passing >48 hours
Ribbon stools
Faltering growth (amber)
Previously unknown or undiagnosed weakness in legs, locomotor delay
Abdominal distension
Disclosure or evidence that raises concerns over possibility of child maltreatment (Amber)
Features of faecal impaction?
symptoms of severe constipation
overflow soiling
faecal mass palpable in the abdomen (digital rectal examination should only be carried out by a specialist)
Management of constipation in paediatrics?
Faecal impaction - Movicol Paediatric Plain, (escalating dose) add stimulant laxative if need to, then osmotic laxative (lactulose)
Maintenance - Movicol, then stimulant, then lactulose
Ensure adequate fluid and fibre intake alongside laxatives
Consider regular toileting and non-punitive behavioural interventions
For all children consider asking the Health Visitor or Paediatric Continence Advisor to help support the parents.
Infants not yet weaned (usually < 6 months)
bottle-fed infants: give extra water in between feeds. Try gentle abdominal massage and bicycling the infant’s legs
breast-fed infants: constipation is unusual and organic causes should be considered
Infants who have or are being weaned
offer extra water, diluted fruit juice and fruits
if not effective consider adding lactulose
Summary of encopresis?
Faecal incontinence
Considered pathological at age 4
Sign of chronic constipation - rectum becomes stretched and looses sensation
Other causes = spina bifida, Hirschsprung, cerebral palsy, learning disability, psychosocial stress, abuse
Causes of chronic diarrhoea in children?
most common cause in the developed world is cows’ milk intolerance
toddler diarrhoea: stools vary in consistency, often contain undigested food
coeliac disease
post-gastroenteritis lactose intolerance
Most common cause of gastroenteritis in children?
Rotavirus
Summary of febrile convulsions?
type of seizure that occurs in children with a high fever
not caused by epilepsy or underlying neuro pathology - e.g., meningitis/tumours
definition only children between 6m and 5 years
simple - generalised, tonic clonic, less than 15 mins, occur once during single febrile illness
complex - partial or focal seizures, lasting more than 15 minutes or occur multiple times during same febrile illness
Dx - exclude other neuro pathology
Tx - manage underlying infection, control fever with simple analgesia, simple don’t need further Ix, reassure/educate parents
Prognosis - 1/3 will have another, increased risk of developing epilepsy
Features of innocent murmurs (flow murmurs)?
Soft
Short
Systolic
Symptomless
Situation dependent
When to refer murmur to paediatric cardiologist?
Murmur louder than 2/6
Diastolic murmurs
Louder on standing
Other symptoms such as failure to thrive, feeding difficulty, cyanosis or shortness of breath
Summary of cyanotic heart disease?
Occurs when deoxygenated blood enters systemic circulation. Blood bypasses pulmonary circulation and the lungs.
RIGHT TO LEFT SHUNT - blood doesn’t get oxygenated at lungs
Causes of R to L shunt - VSD, ASD, PDA, TGA
Usually VSD, ASD, PDA are not cyanotic as pressure in L of heart is greater, and prevents shunt
If pulmonary pressure increases beyond systemic pressure, blood flows from R to L > causing cyanosis. This is Eisenmenger syndrome
TGA is always cyanotic
Summary of patent ductus arteriosus?
RF - genetic, prematurity, rubella
Stops functioning within few days, closes within few weeks
Causes L to R shunt > causing pulmonary hypertension > R sided heart strain > RV hypertrophy > increased blood through pulmonary vessels > LV hypertrophy
Sx - newborn murmur, SOB, difficulty feeding, poor WG, LRTI
Dx - ECHO
Tx - monitored until age 1 using ECHO, trans-catheter or surgical closure can be performed
Murmur in PDA?
Normal S1 with continuous crescendo-decrescendo ‘machinery’ murmur
May not hear S2
Summary of ASD?
Two walls - septum primum (has osmium primum) and septum secondum (has foramen ovale, osmium secondum) - failure to close
Leads to shunt - L to R due to pressure. Increased flow to right side of heart > R overload and R heart strain > R HF and pulmonary HTN > Eisenmenger syndrome (pulm pressure > systemic pressure) > R to L shunt > pt becomes cyanotic
Types - osmium secondum, patent foramen ovale, osmium primum
Complications - stroke, AF/atrial flutter, pulmonary HTN, RHF, Eisenmenger syndrome
ASD cause of stroke in pt with DVT!!!
Sx in adulthood - SOB, HF, stroke
Sx in childhood - SOB, difficulty feeding, poor WG, LRTI, found on NIPE
Tx - watching and waiting if small and asymptomatic, surgery (TV catheter closure or open heart surgery), anticoagulants
Murmur in ASD?
mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border with a fixed split second heart sound
Summary of VSD?
Associations - Downs, Turners
Can cause Eisenmenger syndrome > due to increase blood flow > RV HTN > pressure in RA>LA > shunt RtoL > pt becomes cyanotic
Sx - symptomless, newborn check, antenatal scans, poor feeding, dyspnoea, tachypnoea, FTT
Tx - Smal VSD w/ no Sx (no PHTN or HF) - can be watched. Surgery - TV catheter closure or open heart surgery. ^ risk of IE with VSD
Murmur in VSD?
pan-systolic murmur more prominently heard at the left lower sternal border in the third and fourth intercostal spaces.
There may be a systolic thrill on palpation.
Summary of Eisenmenger syndrome?
R to L shunt > due to ASD, VSD, or PDA. As increased pressure in pulmonary vessels, RV strain and HF, eventually pressure in RA>LA.
Findings - RV heave, loud P2, raised JVP, peripheral oedema
Murmur - ASD (mid-systolic, crescendo-decrescendo), VSD (pan-systolic), PDA (continuous crescendo-decrescendo machinery), arrhythmias
cyanosis, clubbing, SOB, plethoric complexion
reduced life expectancy by around 20 years > HF, infection, thromboembolism, haemorrhage
Tx - ideally defect managed to prevent Eisenmenger syndrome. Cannot reverse condition > heart-lung transplant. Medical follow up:
O2, Tx of PHTN (sildenafil), Tx of arrhythmias, Tx of polycythaemia, Tx and prevention of thrombosis, Prevention of IE
Summary of coarctation of aorta?
Narrowing of aortic arch - usually around ductus arteriosus. Severity can vary.
Association - Turners
Reduces pressure of blood flowing to arteries distal to narrowing. Increased pressure in areas proximal to narrowing.
Sx - weak femoral pulses. Systolic murmur in left infraclavicular area and below left scapula. Tachypnoea, poor feeding, grey/floppy baby
Signs developing over time - left ventricular heave (due to LV hypertrophy), underdeveloped L arm as reduced flow to L subclavian artery, underdevelopment of legs
Tx - depends on severity. if critical shortly after birth - prostaglandin used to keep ductus arteriosus open > allows blood to flow to systemic circulation > then surgery to correct coarctation and ligate ductus arteriosus
Summary of congenital aortic valve stenosis?
Narrow aortic valve - prevents blood flow from LV into aorta
Sx - incidental murmur on routine exam, or SOB, dizziness, fainting, worse on exertion, HF within months of birth if severe
Ejection systolic murmur over aortic area, crescendo-decrescendo and radiates to carotids
ECHO, ECH, exercise testing
Tx - percutaneous balloon arch valvoplasty, surgical aortic valvotomy, valve replacement
Complications - LV outflow tract obstruction, HF, ventricular arrhythmia, endocarditis, sudden death
Summary of congenital pulmonary valve stenosis?
Associations - TOF, William syndrome, Noonan syndrome, congenital rubella syndrome
Sx - may be asymptomatic, fatigue on exertion, SOB, dizziness, fainting
Ejection systolic murmur heard over pulmonary area, palpable thrill, RV heave, raised JVP
Tx - ECHO, mild - watching and waiting, if symptomatic - balloon valvuloplasty via venous catheter, or open heart surgery
Summary of Tetralogy of Fallot?
Four co-existing pathologies > VSD, overriding aorta, pulmonary valve stenosis, RV hypertrophy
Overriding aorta - entrance to aorta is placed further to the right than normal - above VSD - means that when RV contracts and sends blood upwards, the aorta is in the direction of travel of that blood - so greater proportion of deoxygenated blood enters aorta from right side
Stenosis of pulmonary valve - greater resistance against the flow of blood from the RV. This encourages blood to flow through VSD and into aorta, rather than pulmonary vessels. Therefore overriding aorta and pulmonary stenosis > blood shunted from right to left heart. Causes cyanosis.
Right ventricular hypertrophy as increased strain from RV pumping against resistance of LV
RF - rubella, ^ age of mother, alcohol consumption in pregnancy, DM mother
Ix - ECHO, CXR (boot shaped due to RV thickening)
Presentation - antenatal scans, ejection systolic murmur (pulmonary stenosis) on NIPE, HF before one year of age, cyanosis, clubbing, poor feeding, poor WG, Tet spells
Tx - neonates - prostaglandin infusion to maintain ductus arterioles - allows blood to flow from aorta back to pulmonary arteries
Total surgical repair by open heart surgery
What tet spells?
Intermittent symptomatic periods where R to L shunt becomes temporarily worse - precipitating cyanotic episode. Happens when pulmonary vascular resistance increases or the systemic resistance decreases.
E.g., - child exerting themselves and generating CO2 - causes systemic vasodilation and hence reduces systemic vascular resistance. Causes blood to pump from RV to aorta.
Precipitated by waking, physical exertion or crying. Child - irritable, cyanotic and SOB. Severe spells - reduced consciousness, seizures, and potentially death.
Tx - older children may squat, younger children with knees to chest > encourages systemic vascular resistance and encourages blood to enter pulmonary vessels.
Supplementary O2, BB, IV fluids, morhpine, sodium bicarbonate, phenylephrine
What is Ebstein’s anomaly?
Tricuspid valve is set lower in the R side of the heart > causes bigger RA and smaller RV > causes poor flow to pulmonary vessels
Associated with R to L shunt via ASD > cyanosis
Associated with Wolff-Parkinson-White syndrome
Sx - HF Sx, gallop rhythm on auscultation (S3+4), cyanosis, SOB, tachypnoea, poor feeding, collapse or cardiac arrest
Usually present few days after birth - when ductus arteriosus closes
Dx - ECHO
Tx - Treat arrhythmias and HF, prophylactic ABx, surgical correction
Summary of transposition of great arteries?
Attachments of aorta and pulmonary trunk have swapped
RV > aorta. LV > pulmonary artery
2 separate circulations that don’t mix > systemic circulation and right side of heart. Pulmonary system and L side of heart.
Associated with VSD, coarctation of aorta, pulmonary stenosis
Sx - antenatal scans, cyanosis, initially compensated with PDA or VSD, but within few weeks - respiratory distress, tachycardia, poor feeding, poor WG, sweating
Survival requires shunt between the 2 circulations - e.g., PDA, ASD, VSD
Tx - prostaglandin infusion to maintain ductus arteriosus, balloon septostomy (create ASD), open heart surgery, cardiopulmonary bypass
