Paeds fifth yr

Question 1

What is cerebral palsy?

Answer 1

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

Question 2

Causes of cerebral palsy?

Answer 2

Antenatal:
Maternal infections
Trauma during pregnancy
Perinatal:

Birth asphyxia
Pre-term birth
Postnatal:

Meningitis
Severe neonatal jaundice
Head injury

Question 3

Types of cerebral palsy?

Answer 3

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.

Question 4

Features of cerebral palsy?

Answer 4

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

Question 5

Management of cerebral palsy?

Answer 5

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

Question 6

What is achondroplasia?

Answer 6

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.

Question 7

Features of achondroplasia?

Answer 7

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

Question 8

Management of achondroplasia?

Answer 8

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.

Question 9

What is ADHD?

Answer 9

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

Question 10

Features of ADHD?

Answer 10

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

Question 11

Management of ADHD

Answer 11

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

Question 12

What is caput succedaneum?

Answer 12

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

Question 13

What is cephalohaematoma?

Answer 13

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.

Question 14

Summary of whooping cough?

Answer 14

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

Question 15

Summary of transient tachypnoea of newborn?

Answer 15

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

Question 16

Summary of scarlet fever?

Answer 16

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

Question 17

Summary of pneumonia in children?

Answer 17

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

Question 18

Summary of meconium aspiration syndrome?

Answer 18

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.

Question 19

Summary of measles?

Answer 19

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

Question 20

Summary of Kawasaki disease?

Answer 20

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

Question 21

Summary of hand, foot and mouth disease?

Answer 21

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

Question 22

Traffic light system for feverish illness?

Answer 22

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

Question 23

Summary of CF?

Answer 23

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

Question 24

Summary of croup?

Answer 24

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

Question 25

Summary of chickenpox?

Answer 25

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

Question 26

Summary of mumps?

Answer 26

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

Question 27

Summary of rubella?

Answer 27

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,

Question 28

Summary of erythema infecitosum?

Answer 28

Fifth disease/slapped cheek/parvovirus B19 Sx - lethargy, fever, headache Slapped cheek rash spreading to proximal arms and extensor surfaces

Question 29

Summary of bronchiolitis?

Answer 29

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

Question 30

Summary of acute epiglottitis?

Answer 30

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

Question 31

Summary of roseola infantum?

Answer 31

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

Question 32

School exclusion for paediatric illness?

Answer 32

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

Question 33

What is autism spectrum disorder?

Answer 33

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

Question 34

Features of autism?

Answer 34

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.

Question 35

Management of autism?

Answer 35

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

Question 36

Symptoms of acute asthma in a child?

Answer 36

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

Question 37

Management of acute asthma or viral induced wheeze in a child?

Answer 37

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)

Question 38

Discharging child with acute asthma?

Answer 38

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

Question 39

What is asthma?

Answer 39

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

Question 40

Features of asthma?

Answer 40

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

Question 41

Features indicating a diagnosis other than asthma?

Answer 41

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

Question 42

Diagnosis of asthma in a child?

Answer 42

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

Question 43

Medical therapy of asthma in under 5 years?

Answer 43

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.

Question 44

Medical therapy of asthma in child 5-12 years?

Answer 44

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.

Question 45

Medical therapy of asthma in children over 12 years?

Answer 45

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.

Question 46

What is coeliac disease?

Answer 46

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

Question 47

Features of coeliac disease?

Answer 47

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

Question 48

Diagnosis of coeliac disease?

Answer 48

Jejunal biopsy TTG (IgA) anti-endomysial and anti-gliadin antibodies are useful screening tests

Question 49

Management of coeliac disease?

Answer 49

Gluten free diet - rice, potatoes, corn Immunisation - functional hyposplenism

Question 50

Complications of coeliac disease?

Answer 50

Anaemia Vitamin deficiency Osteoporosis Ulcerative jejunitis Enteropathy-associated T-cell lymphoma Non-hodgkin lymphoma Small bowel adenocarcinoma

Question 51

What is cows milk protein allergy?

Answer 51

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.

Question 52

Features of cows milk protein allergy?

Answer 52

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

Question 53

Management of cows milk protein allergy?

Answer 53

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

Question 54

Management of cows milk protein allergy?

Answer 54

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

Question 55

Features of cows milk intolerance

Answer 55

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.

Question 56

Sx of constipation in children?

Answer 56

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

Question 57

Causes of constipation in children?

Answer 57

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

Question 58

Red flags for constipation in children?

Answer 58

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)

Question 59

Features of faecal impaction?

Answer 59

symptoms of severe constipation overflow soiling faecal mass palpable in the abdomen (digital rectal examination should only be carried out by a specialist)

Question 60

Management of constipation in paediatrics?

Answer 60

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

Question 61

Summary of encopresis?

Answer 61

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

Question 62

Causes of chronic diarrhoea in children?

Answer 62

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

Question 63

Most common cause of gastroenteritis in children?

Answer 63

Rotavirus

Question 64

Summary of febrile convulsions?

Answer 64

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

Question 65

Features of innocent murmurs (flow murmurs)?

Answer 65

Soft Short Systolic Symptomless Situation dependent

Question 66

When to refer murmur to paediatric cardiologist?

Answer 66

Murmur louder than 2/6 Diastolic murmurs Louder on standing Other symptoms such as failure to thrive, feeding difficulty, cyanosis or shortness of breath

Question 67

Summary of cyanotic heart disease?

Answer 67

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

Question 68

Summary of patent ductus arteriosus?

Answer 68

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

Question 69

Murmur in PDA?

Answer 69

Normal S1 with continuous crescendo-decrescendo 'machinery' murmur May not hear S2

Question 70

Summary of ASD?

Answer 70

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

Question 71

Murmur in ASD?

Answer 71

mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border with a fixed split second heart sound

Question 72

Summary of VSD?

Answer 72

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

Question 73

Murmur in VSD?

Answer 73

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.

Question 74

Summary of Eisenmenger syndrome?

Answer 74

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

Question 75

Summary of coarctation of aorta?

Answer 75

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

Question 76

Summary of congenital aortic valve stenosis?

Answer 76

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

Question 77

Summary of congenital pulmonary valve stenosis?

Answer 77

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

Question 78

Summary of Tetralogy of Fallot?

Answer 78

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

Question 79

What tet spells?

Answer 79

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

Question 80

What is Ebstein's anomaly?

Answer 80

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

Question 81

Summary of transposition of great arteries?

Answer 81

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