Paediatrics

Question 1

Who can complete then routine examination of the newborn

Answer 1

Hospital paediatrician
Advanced neonatal nurse Practitioner
GP
Specially trained nurse/midwife

Question 2

Routine examination of newborn

Answer 2

Head to toe
Completely undressed

• Skull shape, maximum circumference, fontanelles
• Face: any dysmorphisms, examine for cleft palate
• Ears: position, size, shape, patency
• Neck
• Chest: shape, symmetry, nipple position. RR 40-60, palpate precordium
• Abdomen: look at umbilical stump, inguinal hernias, palpate organs
• Genital: female inspect. male - urinary meatus, descended testes
• Femoral pulses
• Anus: patency
• Spine: deformity, dimple, naevi, hair
• Limbs: tone. Digit number. Clubbed foot, palmar creases
• DDH
• CNS: tone (should be flexed at hip and knee), pull baby to sitting by holding wrists. Ventral suspension
  Moro reflex

Question 3

Moro reflex

Answer 3

Act as if to drop the baby
Arms should extend and then flex

If bilateral absence then CNS damage
If unilateral then birth trauma e.g. Erb’s palsy

Question 4

Define vernix

Answer 4

Normal cheesy white substance on the skin of baby at birth

Question 5

Skull molding

Answer 5

Over riding skull bones
Palpable ridges
Normal
Resolves in 2-3 days

Question 6

Caput succedaneum

Answer 6

Swelling or oedema on new born scalp
From prolonged pressure during delivery
Resolves in a few days

Question 7

Chignon

Answer 7

Temporary swelling of infants head after ventouse suction cap
Increases rate of jaundice

Question 8

Cephalhaematoma

Answer 8

Bruise on neonates head from prolonged stage 2 of labour or instrumental delivery
Subperisoteal
Boundaries are limited by bone margins

Question 9

Subconjuctival haemorrhage in newborn

Answer 9

Can happen in precipitate delivery (<2 hours) or if cord around neck
Harmless
Will resolve in a few weeks

Be careful if later than newborn as can indicate abuse

Question 10

Epstein’s peals

Answer 10

Self resolving white inclusion cysts on palate

Question 11

Tongue tie

Answer 11

Shortened tongue frenulum

Question 12

Ranula

Answer 12

Self resolving bluish mouth floor swelling

mucus retention cyst

Question 13

Retracted testes

Answer 13

Due to exaggerated cremastus muscle

Palpate along to determine location of testes

Question 14

Mongolian blue spot

Answer 14

Normal birth mark

Increased in Asian and Africans

Question 15

Erythema toxicum

Answer 15

Normal neonatal rash
Erythematous base with small pustules
Unknown cause

Question 16

Primitive reflexes

Answer 16

Rooting
Grasping
Moro
Walking
Sucking
Asymmetrical tonic neck reflex - fencing posture
Galant reflex
Swimming reflex

Question 17

Describe grasping reflex

Answer 17

Baby will grasp anything in palms

Disappears at 5-6 months

Question 18

Describe asymmetrical tonic neck reflex

Answer 18

Fencing posture
Present from 1-4 months
When head is turned to the side, the arm on that side will straighten , opposite will bend
If >6months then UMN disorder

Question 19

Characteristics of pathological heart murmur

Answer 19

All Diastolic
All Pansystolic
Late systolic murmurs
Loud murmurs > 3/6
Continuous murmur
Associated cardiac abnormalities
Heard over upper L sternal border

Symptomatic:
SOB, easy fatigue, cyanosis, FTT, clubbing, hepatomegaly

Question 20

Characteristic of innocent heart murmur

Answer 20

Always systolic
Short duration
Low intensity
Increases with increased cardiac output
Intensity can change with posture
no thrill or heave
No radiation
No symptoms

Question 21

Predisposing conditions for heart murmurs

Answer 21

Down's
Noonan's
Turner's
Marfan's
Foetal alcohol syndrome
Foetal rubella infection

Question 22

Still’s murmur

Answer 22

Innocent murmur
Mid L sternal border
Mid-systolic
Grad 2-3
Twanging, musical, vibrating sound

Question 23

Pulmonary flow murmur

Answer 23

Innocent murmur
Upper L sternal border
Mid systolic
Grade 1-3
Grating

Question 24

Acyanotic causes of congenital heart disease

Answer 24

Ventricular septal defect
Atrial septal defect
Patent Ductus Arteriosus
Pulmonary valve stenosis
Coarctation of the aorta
Hypoplastic left heart syndrome
Hypertrophic obstructive cardiomyopathy 
Dextrocardia

Question 25

Cyanotic causes of congenital heart disease

Answer 25

``` Tetralogy of fallot Transposition of great arteries Tricuspid atresia Total anomalous pulmonary drainage Persistent truncus arteriosus Ebstein's abnormality ```

Question 26

Epidemiology of congenital heart disease

Answer 26

``` 8 per 1000 live births 1/3 of congenital abnormalities Increased if FHx Increased if parents are relatives Intrauterine - drugs, alcohol, rubella infection Down's syndrome DiGeorge's syndrome Williams syndrome Noonan's syndrome Turner's syndrome Maternal diabetes ```

Question 27

Atrial septal defect

Answer 27

Left to Right shunt Can be ostium secundum defect (foramen ovale) with normal valves or partial AVSD affecting valves Usually asymptomatic Tx - close ASD in early childhood

Question 28

Ventricular septal defect

Answer 28

``` 25% of all congenital heart disease Typically asymptomatic Pansystolic murmur at left lower sternal edge Most close spontaneously Can have heart failure ```

Question 29

Patent ductus arteriosus

Answer 29

Common Defined as patent ductus arteriosus if patent at 1 month Connects pulmonary artery to aorta Low diastolic pressure due to back flow through pulmonary artery Wide pulse pressure Bounding peripheral pulses Continuous or machinery murmur in left infraclavicular area Most close spontaneously

Question 30

Tetralogy of Fallot

Answer 30

4 cardinal anatomical abnormalities 1. VSD 2. Over-riding aorta 3. R ventricular outflow obstruction 4. R ventricular hypertrophy Cyanosis Paroxysmal hypercyanotic spells - Tachypnoea, restlessness. Become white or floppy for minute-hours Treated with prostaglandin E infusion R to L shunt Increased risk of cerebral ischaemia, endocarditis, HF, brain abscess, polycythaemia

Question 31

Transposition of the great arteries

Answer 31

2 separate parallel circuits Systemic venous blood returns to R heart and back to systemic flow Not compatible with life unless mixing ASD or PDA Severe hypoxia No heart failure

Question 32

Heart defect associated with Noonan's syndrome

Answer 32

Pulmonary stenosis

Question 33

Heart defect associated with Turner's syndrome

Answer 33

Coarctation of aorta

Question 34

Neonatal history

Answer 34

PREGNANCY - parity - any extra scans - maternal problems in pregnancy - medications BIRTH - gestation - induced or spontaneous labour - PROM? - RF for infection - Vaginal or C-section SINCE BIRTH - Feeeding, meconium, urine, vomiting - Receive Vit K - Any problems or concerns Fhx and social - genetic conditions - RF for DDH - SIDS - Social set up

Question 35

Causes of sick newborn

Answer 35

``` Sepsis Hypoxic Ischaemia Encephalopathy Transient Tachypnoea of newborn RDS Hypoglycaemia Jaundice Necrotising enterocolitis Cardiac murmur Neonatal abstinence syndrome Seizures ```

Question 36

Changes in foetal circulation at birth

Answer 36

In utero - blood vessels that supply and drain lungs are constricted Increased pulmonary vascular resistance Blood bypasses lungs through ductus arteriosus Pulmonary vessels dilate due to increased O2 Encourages blood flow to lungs Closure of ductus arteriosus ``` Pulmonary expansion Increased O2 tension Decreased pulmonary vascular resistance Increased pulmonary blood flow Increased LA filling Closure of foramen ovale ```

Question 37

Apgar score

Answer 37

HR - 0-absent, 1-<100, 2>100 Respiration - 0-absent, 1-gasping, 2-regular Muscle tone - 0-flaccid, 1=some flexion, 2=well flexed Reflex irritability 0-none, 1-grimace, 2-cry/cough Colour - 0-pale/blue, 1-body pink, blue limbs, 2-pink

Question 38

Cleft palate/lip

Answer 38

1 in 1000 children Tends to run in families Problems - Difficulty feeding - Milk aspiration - Speech difficulties - Conductive hearing loss due to Eustacian tube dysfunction - Dental problems Surgically corrected at 9 months Give specialist advice on managing baby

Question 39

Neural tube defects

Answer 39

Failure of neural tube to close in early pregnancy - incidence decreased with folic acid supplements Screening with US and alpha fetoprotein Anencephaly - most severe. complete failure of development of cranial nerve tube Myelomeningocoele - open lesion with malformed and exposed spinal cord covered by meninges. Severe neurological abnormality of lower limbs Meningocoele - spinal cord intact, exposed meninges. High risk of meningitis and hydrocephalus Spina bifida occulta - hidden abnormality. Develop bladder dysfunction and pyramidal tract signs in legs as child grows - Deep pit, tuft of hair, naevus, lipoma in midline US offers diagnosis

Question 40

Hypospadias

Answer 40

Urethra abnormally placed 1 in 500 boys Most commonly ventrally on glans up to perineum Increased severity with increased penile curve Severe cases require repair before 2 years DO NOT CIRCUMCISE as foreskin used in reconstruction

Question 41

Bowel obstruction

Answer 41

1 in 1000 babies Can be anatomical or functional Clinical features: - Bile stained vomiting - Failure to pass meconium - Abdominal distension - Visible peristalsis Oesophageal atresia: mucus, chokes on first feed Duodenal atresia - bile stained vomit, double bubble on AXR Jejunal atresia = most common. distension. bile stained vomit

Question 42

Hirschsprung's disease

Answer 42

Absence of myenteric nerve plexus in the rectum may extend along the colon abdomen distends 15% present with acute enterocolitis

Question 43

Exomphalos

Answer 43

Often diagnosed Antenatally Also called omphalocele Abdominal contents protrude through umbilical ring, covered by transparent sac.

Question 44

Gastroschsis

Answer 44

Bowel protrudes through defect in anterior abdominal wall adjacent to umbilicus no sac covering Cover with cling film Increased risk of dehydration

Question 45

Hypoxic ischaemic encephalopathy

Answer 45

Hypoxia. Hypercapnia. Metabolic acidosis. Decrease tissue perfusion. Brain damage, disability or death Causes: - prolonged uterine contractions - placental abruption - ruptured uterus - cord compression - shoulder dystocia - cord prolapse - maternal hypotension - anaemia - IUGR MILD - infant irritable, responds excessively to stimulation MODERATE - marked abnormalities in tone and movement SEVERE - no normal spontaneous movements or response to pain. prolonged seizures. 30-40% mortality

Question 46

Brachial nerve palsies

Answer 46

Occurs if breech birth or shoulder dystocia Upper nerve root C5-6 = ERB's palsy Lower nerve root = KLUMPKEYS palsy

Question 47

Facial nerve palsy

Answer 47

``` Via compression of facial nerve on ischial spine Unilateral Facial weakness on crying Eye remains open Usually transient ```

Question 48

Define pre-term infant

Answer 48

Under 34 weeks

Question 49

Medical problems in pre-term infants

Answer 49

``` Resuscitation at birth RDS Hypotension PDA Temperature control issues Hypoglycaemia, hypocalcaemia, electrolyte imbalance Nutrition Infection Jaundice Necrotising entercolitis Retinopathy of prematurity Anaemia of prematurity Brocnhopulmonary dysplasia Inguinal hernias ```

Question 50

Respiratory distress syndrome

Answer 50

Deficiency of surfactant from type II pneumocytes Widespread alveolar collapse and inadequate gas exchange Steroids given Antenatally stimulate surfactant production Tachypnoea >60 Laboured breathing, chest wall recession, nasal flaring Expiratory grunting Cyanosis if severe CXR - diffuse granular or ground glass appearance Treat with CPAP and O2

Question 51

Necrotising enterocolitis

Answer 51

Affects pre-term babies in first few weeks Bacterial invasion of ischaemia bowel wall increased if cows milk fed Infant stops tolerating feeds, milk aspirated from stomach Vomiting +/- bile staining Distended abdomen Fresh blood in stools Distended loops of bowel Thickening of bowel wall Can cause perforation Stop feeding Start broad spec Abx

Question 52

Retinopathy of prematurity

Answer 52

Affects developing blood vessels at the junction of vascular and non-vascularised retina Vascular proliferation Retinal detachment Fibrosis Blindness

Question 53

Bronchopulmonary dysplasia

Answer 53

Infants who still require O2 at 36 weeks Lung damage from pressure and volume trauma from ventilation CXR - widespread opacification and cystic changes

Question 54

Jaundice in neonates

Answer 54

Over 50% of newborns are visibly jaundiced - Marked physiological release of Hb from breakdown of Hb at birth - RBC lifespan is only 70 days - Hepatic bilirubin metabolism if less efficient in first few days May be a sign of: - Haemolytic anaemia - Infection - metabolic or liver disease

Question 55

Kernicterus

Answer 55

Encephalopathy resulting from deposition of unconjugated bilirubin in basal ganglia and brainstem nuclei - lethargy and poor feeding - irritability - Increased muscle tone - Seizures, coma - Survivors can develop choreoathetoid CP - learning disability and sensorineural deafness

Question 56

Causes of jaundice in neonate

Answer 56

Haemolytic disorders: Rhesus/ABO incompatability. G6PD deficiency. Spherocytosis. ``` Physiological. Breast milk jaundice Infection Haemolysis Bruising Polycythaemia Hypothyroid High GI obstruction ```

Question 57

Management of jaundice

Answer 57

Phototherapy - blue/green band converts unconjugated bilirubin to water soluble pigment Exchange transfusion - if dangerously high levels. Remove baby blood and replace with donor blood

Question 58

Causes of infection in neonate

Answer 58

Group B Strep - Pneumonia or meningitis - Give prophylactic Abc intrapartum Listeria monocytogenes - from unpasteurised milk, soft cheese, undercooked poultry - Bacteraemia with mild flu like illness - Meconium staining of licquor - Widespread rash, septicaemia Conjunctivitis - sticky eye on day 3 or 4 - Chlamydia, gonorrhoea, - Can be staph or strep

Question 59

Epidemiology of sudden infant death syndrome

Answer 59

Most common aged 2-4 months Incidence is decreasing with Back to Sleep campaign RFs - Low birth weight and pre-term - Increased in males - Multiple births - Increasing maternal parity - Parental low income - Poor or overcrowded housing - Maternal age under 20 - Single unsupported mother - Maternal smoking during pregnancy or in the same room post birth - Sleeping prone

Question 60

Prevention of SIDS

Answer 60

Back to Sleep Campaign Lie infant on back Feet to foot position Do not smoke in same room Avoid overheating Baby should sleep in same room for first 6 months Avoid sleeping with the infant e.g. sofa, chair

Question 61

Investigations and management for SIDS

Answer 61

Detailed clinical exam Remove any ET tubes and needles but retain venous lines Retain child clothes, bedding and nappy for police ``` Nasopharyngeal aspirate Blood for toxicology, metabolic screen Chromosomal anomalies Blood cultures Urine LP for virology and culture ``` ``` PM and coroners review Give parents opportunity to see and hold child Social review Case discussion Bereavement counselling ```

Question 62

Causes of shock in child

Answer 62

Hypovolaemia - Dehydration from gastroenteritis - DKA - Blood loss from trauma Maldistribution of fluid - anaphylaxis - Septicaemia Cardiogenic - Arrhythmia - Heart failure

Question 63

Causes of respiratory distress in children

Answer 63

``` Croup Epiglottitis Foreign body Congenital malformations Trauma Asthma Bronchiolitis Pneumonia pneumothorax ```

Question 64

Causes of drowsiness in children

Answer 64

``` Post-ictal Status epilepticus Meningitis Encephalitis DKA Hypoglycaemia Electrolyte disturbance Inborn metabolism error Head injury Drug/ poison ingestion Intracranial haemorrhage ```

Question 65

Paediatric BLS

Answer 65

Open airway - Infants: neutral position - Children: sniffing position 5 rescue breaths Check pulses - < 1 year = brachial and femoral - > 1 year = carotid and femoral If no pulse or pulse <60 then chest compressions 15:1 Adrenaline every 3-5 minutes 10 micrograms per kg IV

Question 66

Apparent life-threatening events

Answer 66

Combination of apnoea, colour change, alteration in muscle tone, choking Most common in under 10 weeks Most are brief with rapid recovery Causes - Infections (RSV, pertussis) - Seizures - Upper airway obstruction - GORD

Question 67

Clinical manifestations of Down's syndrome

Answer 67

``` Round face, flat nasal bridge Upslanted palpebral fissures Epicanthic folds Brushfield spots in iris Small mouth, protruding tongue Small low set ears Short neck Flat occiput Single palmar crease Incurved 5th finger Hypotonia Congenital heart defects Duodenal atresia Hirschsprung's disease ```

Question 68

Later medical problems in Down's syndromes

Answer 68

Delayed motor milestones Moderate-severe LD Small stature Increased susceptibility to infection hearing impairment from secretory otitis media Visual impairment - cataracts, squint, myopia Increased risk of leukaemia and solid tumour Hypothyroidism Coeliac disease Epilepsy Alzheimer's disease

Question 69

Genetics of Down's

Answer 69

TRISOMY 21 | 94% meiotic non-disjunction (increases with maternal edge)

Question 70

Edward's syndrome

Answer 70

``` TRISOMY 18 1 in 8000 Multiple severe abnormality Low birth weight Prominent occiput Small mouth and chin Short sternum Fixed overlapping fingers Rocker bottom feet Cardiac and renal malformations ```

Question 71

Patau's syndrome

Answer 71

``` TRIMSOMY 13 1 in 14,000 Structural defect in the brain Scalp defects Small eyes (microphthalmia) Cleft palate and lip Polydactyly Cardiac and renal malformations ```

Question 72

Turner's syndrome

Answer 72

``` 45 XO 95% are spontaneously miscarried Often diagnosed on US - cystic hygroma and foetal neck oedema Lymphoedema of hands and feet Spoon shaped nails Short stature Neck webbing Wide carrying angle Widely spaced nipples Congenital heart defects Delayed puberty Infertile Hypothyroidism Renal anomalies Pigmented moles Recurrent otitis media ```

Question 73

Klienfelter's syndrome

Answer 73

``` 47 XXY 1-2 per 1000 males Infertility Hypogonadism Small testes Pubertal development, may appear normal Gynaecomastia ```

Question 74

Autosomal dominant conditions

Answer 74

``` Achondroplasia Ehler's Danlos syndrome Familial hypercholesterolaemia Huntington's disease Noonan's syndrome Otosclerosis Tuberous Sclerosis Marfan's syndrome Myotonic dystrophy Neurofibromatosis Osteogenesis imperfect Polyposis coli ```

Question 75

Describe autosomal dominant inheritance

Answer 75

Chromosomes 1-22 Children of someone affected 50% affected 50% normal

Question 76

Autosomal recessive conditions

Answer 76

``` Cystic fibrosis Congenital adrenal hyperplasia Galactosaemia Freidreich ataxia Glycogen storage disease Phenylketonuria Hurler syndrome Tay-Sachs disease Sickle cell disease Thalassaemia ```

Question 77

X linked inheritance & diseases

Answer 77

Can be dominant or recessive males affected, females usually healthy but carriers - Colour blindness - Duchenne's and Becker's muscular dystrophy - Fragile X syndrome - G6PD - Haemophilia A and B - Hunter's syndrome

Question 78

Fragile X syndrome

Answer 78

``` Moderate-severe LD Macrocephaly Macro-orchidism Long face Large everted ears Prominent mandible Broad forehead mitral valve prolapse Joint laxity and scoliosis Autism Hyperactivity ```

Question 79

Imprinting

Answer 79

Normally in a gene - mothers and fathers acts equally In imprinting, one is more functional that the other Chromosome 15 - If mothers unfunctional: Angelman's syndrome - If fathers unfunctional: Prader-Willi syndrome

Question 80

Prader-Willi Syndrome

Answer 80

Chromosome 15 - parental gene unfunctional ``` Hypotonia Neonatal feeding difficulties Failure to thrive Obesity in later childhood with hyperphagia Hypogonadism Developmental delay and LD ```

Question 81

Angelman's syndrome

Answer 81

Chromosome 15 - maternal gene non-functional Severe intellectual and developmental disability Sleep disturbance Seizures and jerky movements (hand flapping) Frequent laughter and smiling

Question 82

William's syndrome

Answer 82

Short stature Transient neonatal hypercalcaemia Congenital heart disease - aortic stenosis Mild-moderate LD

Question 83

Newborn screening

Answer 83

Hearing | Blood spot test

Question 84

Newborn hearing screening

Answer 84

Within 4-5 weeks usually prior to discharge 1st test: Automated Oto-Acoustic Emission (AOAE) - play sound in ear, try to detect echo response from baby's cochlea 2nd test: Automated Auditory Brainstem Response (AABR) - play sound, detect response from brain stem via electrodes. assesses integrity from inner ear AABR if no clear response on AOAE or >48 hours in SCBU or NICU

Question 85

Newborn Blood Spot Test

Answer 85

Blood spot/heel prick/Guthrie test Ideally on day 5 (day 5-8) Tests for: - Phenylketonuria - Cystic fibrosis - Congenital hypothyroidism - Maple syrup urine disease - Sickle cell disease - MCAD deficiency - Homocysteinuria - Glutaric aciduria type 1 - Isoraleric acidaemia

Question 86

Phenylketonuria

Answer 86

Testing looks for total biopterin and dihydropteridine reducates allows for early implementation of phenylalanine restricted diet Early diagnosis and treatment decreases handicap Inborn error of amino acid metabolism Absent phenylalanine hydroxylase - important in formation of catecholamine's, neurotransmitters and melanine Musty or mousey odour Developmental delay Recurrent vomiting

Question 87

Homocysteinuria

Answer 87

``` Rare inherited metabolic disorder Raised blood and urine concentration of homocysteine Autosomal recessive Normal at birth Complications in childhood Tall stature (Marfanoid habitus) Fine brittle hair High arched palate Pectus excavatum Limited joint mobility Dislocation of lens Increased risk of VTE,MI, osteoporosis, mitral valve prolapse ```

Question 88

Maple syrup urine disease

Answer 88

``` 1 in 185,000 Autosomal recessive Maple syrup odour in cerumen Poor feeding Vomiting Poor weight gain Lethargy Dystonia Seizures ```

Question 89

Clinical features of foetal alcohol syndrome

Answer 89

Failure of growth - Never catch up Facial abnormalities - Microcephaly - Flat philtrum - Thin upper lip - Retrognathia, micrognathia - Microphalamia - Cleft palate/lip - posterior rotation of ears Neurodevelopement - Low IQ, memory problems - Hyperactivity, attention deficits - Poor judgement - Immature behaviour - Speech and language delay - Sucking and feeding problems Also - Congenital heart disease (ASD and VSD) - Partial deafness - Significant visual disability

Question 90

Stages of growth

Answer 90

FOETAL - fastest period of growth, accounting for 30% of eventual height - Size at birth depends on size of mother and placental nutrient supply - IGF-2, insulin and human placental lactogen control growth INFANTILE - growth dependent on adequate nutrition - good health and normal thyroid function necessary - rapid but decelerating growth rate - inadequate weight gain = FTT CHILDHOOD - slow and steady - role of pituitary growth hormone. GH is secreted to produce insulin life growth factor 1 - IGF-1 - profound unhappiness can decrease GH and causes psychological short stature PUBERTY - sex hormones (testosterone and estradiol) causes back lengthening and increased GH secretion - Adds 15% to final height

Question 91

Define short stature

Answer 91

Height below the 2nd centile (2SD below the mean) Many will be normal, just short with short parents The further under the centile, the high risk of pathological cause Height will fall through centile lines Can be plotted on height velocity chart

Question 92

Calculating genetic height

Answer 92

Mothers height + fathers height /2 If female -7cm If male +7cm

Question 93

Aetiology of short stature

Answer 93

- Familial - IUGR and extreme prematurity - Constitutional delay of growth and puberty - Hypothyroidism - Growth hormone deficiency - Corticosteroid excess, Cushing's - Nutritional / chronic illness - Psychosocial deprivation - Chromosomal disorders/syndromes - Achondroplasia Extreme short stature - Laron syndrome - Primordial dwarfism - idiopathic - SHOX gene abnormality

Question 94

Constitutional delay of growth and puberty

Answer 94

Increased in males Variation in normal puberty timings Not pathological Can be induced by dieting or excessive physical training Long legs in comparison to back Onset of puberty can be induced with androgens/oestrogens

Question 95

Hypothyroidism causing short stature

Answer 95

Usually caused by autoimmune thyroiditis in childhood Growth failure Excessive weight gain When treated = catch up, can limit final height Congenital hypothyroidism is often diagnosed at birth and therefore does not cause short stature

Question 96

Causes of growth hormone deficiency

Answer 96

Craniopharyngioma - bitemporal hemianopia, papilloedema, optic atrophy - hypothalamic tumour - head injury - meningitis - cranial irradiation - Laron syndrome

Question 97

Chromosomal syndromes causing short stature

Answer 97

``` Laron's syndrome Down's syndrome Noonan's syndrome Russell-Silver syndrome Turner's syndrome ```

Question 98

Investigations for short stature

Answer 98

``` FBC U&Es LFTs TFTs ESR and CRP Urinalysis Karyotyping Bone age - radiograph of L hand and wrist If pathological short stature then delay in bone age ``` Growth hormone provocation tests MRI scan if neurological symptoms

Question 99

Management of short stature

Answer 99

Somatotropin used in growth hormone deficiency Stop when final height attained or adherence poor

Question 100

Noonan's syndrome

Answer 100

``` Autosomal dominant Congenital heart defects - pulmonary vascular stenosis Short stature Learning problems Pectus excavatum Impaired blood clotting Webbed neck Flat nose ```

Question 101

Silver Russell Syndrome

Answer 101

``` Primordial dwarfism SGA Feeding problems Hypoglycaemia Excessive sweating Blue tinge to sclera Triangular face, small jaw, pointed chin Wide and late closing fontanelle Decreased subcut fat ```

Question 102

Laron syndrome

Answer 102

``` Autosomal recessive Insensitivity to growth hormone Abnormally short stature Prominent forehead, depressed nasal bride, underdeveloped mandible Truncal obesity Micropenis in males ```

Question 103

William's syndrome

Answer 103

``` Deletion of sections of chromosome 7 Developmental disorder Broad forehead, short nose, full cheeks Elfin face Intellectual disability Short stature Aortic stenosis Hypercalcaemia ```

Question 104

Advantages of breast feeding

Answer 104

Ideal nutrition for infants during first 6 months Life saving in developing countries Decreases GI infection risk Decreased necrotising enterocolitis Increased mother child bond Decreased diabetes, HTN and obesity in later life

Question 105

Disadvantages of breast feeding

Answer 105

Unknown volume intake Transmission of infection Transmission of drugs Vit K deficiency as not in breast milk (but in formula) Less flexibility - other family can't help

Question 106

Colostrum

Answer 106

Produced for first few days Increased protein and immunoglobulin component Decreased volume First breast feed should take place as soon as possible after birth

Question 107

Physiology of breast feeding

Answer 107

Baby roots and sucks Tactile receptors in nipple activated Hypothalamus sends efferent impulses to pituitary Anterior pituitary produces PROLACTIN which stimulates milk production by cuboidal cells in acini of breast Posterior pituitary produces OXYTOCIN to contract myoepithelial cells in alveoli into large ducts (let down reflex) ``` Prolactin = increased milk production Oxytoxin = contraction of breast ```

Question 108

Describe nutritional requirements of the baby

Answer 108

Breast of formula feeding for first 12 months Cow's milk may be given from 1 year Introduce solid foods after 6 months Done gradually If starting food before 6 months - avoid wheat, eggs and fish No honey in under 1s due to infantile botulinism

Question 109

Define failure to thrive

Answer 109

Suboptimal weight gain in children (infants and toddlers) Below 5th percentile or fall across centiles - Mild: fall across 2 centile lines - Severe: fall across 3 centile lines Hard to distinguish TT from a normal but small baby They will be asymptomatic

Question 110

Aetiology of failure to thrive

Answer 110

70% inorganic (under feeding/psychosocial) 30% organic (secondary to disease) - Decreased intake (social/economic/education) - Decreased ability CNS: CP, tumour, suck/swallow reflex GI; GORD, pyloric stenosis Cleft palate Micrognathia - Decreased absorption: Lactose allergy, CF, IBD, Coeliac Biliary atresia, cirrhosis Obstruction: hernia, malrotation, intussusception Neonatal: NEC, short bowel syndrome - Increased requirement Storage disease, inborn error CHD CF, bronchopulmonary dysplasia Hypo/hyperthyroid disease Malignancy

Question 111

Investigations in failure to thrive

Answer 111

``` FBC, ESR, CRP, U&Es Glucose TFTs Sweat chloride Coeliac - tissue transglutaminase ``` 2nd line - Lead levels - Abdominal US - CXR - Head US - ECG/ echo

Question 112

Management of failure to thrive

Answer 112

``` Health visitor involvement 3 meals a day + snacks Increased energy density foods Regular meal times Praise when food eaten Never force feed a child ``` MDT: health visitor, paediatrician, dietician, social worker

Question 113

Marasmus

Answer 113

Weight for height > 3SD Wasted, wizened appearance No oedema Withdrawn and apathetic

Question 114

Kwashorkor

Answer 114

``` Generalised oedema and severe wasting Flaky pain skin Hyperkeratosis Distended abdomen Hepatomegaly angular stomitis Diarrhoea, hypothermia, bradycardia, hypotension ```

Question 115

Types of abuse

Answer 115

``` Physical Emotional Neglect Sexual Fabricated or induced illness ```

Question 116

Risk factors for abuse

Answer 116

Child - Failure to meet parental expectation e.g. wrong gender or disabled - Resulted from forced, coercive or commercial sex Parent - Mental health problems - Parental indifference, intolerance or over anxious - Alcohol or drug use Family - Step parents - Domestic violence - Social isolation - Young parental age Environment - Poverty, poor housing

Question 117

Injuries more likely to indicate physical abuse

Answer 117

Fractures: in non-mobile child, rib fractures, multiple fractures, fractures of different ages, skull fractures Bruises: shape of hand or object, on the neck (e.g. strangulation), Wrists and ankles, buttocks in under 2s Burns: in non-mobile child, in shape of implement e.g. iron or cigarette, glove and stocking from forced immersion

Question 118

Emotional abuse

Answer 118

Persistent emotional maltreatment resulting in severe adverse effects on child’s emotional development Inappropriate expectations / over protection / made to feel worthless, unloved or inadequate

Question 119

Presentation of emotional abuse

Answer 119

Hardest to identify Babies – apathetic, delayed development Toddlers: violent, apathetic, fearful School age: wetting, soiling, non-attendance Adolescents: self-harm, depression, aggressive

Question 120

Neglect

Answer 120

Persistent failure to meet a child’s physical or psychological needs. Failure of: Adequate food and clothing Shelter Protection from physical and emotional harm or danger Inadequate supervision

Question 121

Presentation of neglect

Answer 121

``` Consistently misses important medical appointment Lacks needed medical and dental care Dirty Inadequate clothing in cold weather Using alcohol or drugs Child mentions no one at home ```

Question 122

Sexual abuse

Answer 122

Forcing or enticing a child to take part in sexual activity Includes: physical contact (rape, oral sex), non-contact (watching sexual activities, looking at pornographic material, encouraging them to behave inappropriately

Question 123

Presentation of sexual abuse

Answer 123

``` Child may tell someone STI with no cause Vaginal bleeding, discharge, itching Rectal bleeding Self harm, aggressive or sexualised behaviours ```

Question 124

Fabricated or induced illness

Answer 124

Behaviour of parents (usually mother >80%) which causes harm to children Verbal fabrication: parents invent signs and symptoms. Abuse is done through tests and treatments Induction of illness: - Suffocation of child presenting as acute life threatening event - Administration of noxious substance or poison - Excessive administration of normal substance e.g. salt - Excessive or unnecessary use of medication **Only happens when a parent/guardian around**

Question 125

Effects of alcohol abuse on the child

Answer 125

Severe congential abnormalities Imapired growth and leaning and behavioural impairment Foetal alcohol syndrome

Question 126

Effects of heroin abuse on the child

Answer 126

Withdrawal syndrome Advise methadone Decreased birth weight

Question 127

Effects of cocaine abuse in the child

Answer 127

``` Powerful vasoconstrictor Increased miscarriage Increased placental abruption Premature labour Foetal death in utero ```

Question 128

Effects of alcohol/drug misuse on child

Answer 128

Physical harm - access to drugs or paraphernalia - increased violence due to decreased inhibition - Increased strangers in home - Chemical dependence ``` Not meeting child's physical needs Inadequate supervision Disruption to education or poor school attendance Child assuming parental role Decreased boundaries and routine Frightening Emotional stresses - fear of abandonment/parents dying/not loving child Feeling responsible for parents drug use ```

Question 129

Describe meconium

Answer 129

``` Black colour First stool GI cells Filled with bilirubin If not expelled quickly, increased risk of jaundice Should be expelled in <24 hours ```

Question 130

Palpable posterior fontanelle indicates what?

Answer 130

hypothyroidism or Down's syndrome

Question 131

Describe presentation of metabolic conditions

Answer 131

If problem with fat/protein/carbohydrate metabolism Presents after first 1-2 days Have a good first feed Then start to get unwell, minimal further feeds Ill due to build up of toxic metabolites from failed metabolism

Question 132

Fields of development

Answer 132

Gross motor Vision and fine motor Hearing, speech and language Social, emotional and behavioural

Question 133

How are developmental milestones judged?

Answer 133

Median age - when half of the population at that level Limit age - 2SD above mean. Age at which it should have been achieved If a baby is pre-term should be calculated from expected date of delivery to allow to catch up. Only up to 2 year.

Question 134

Gross Motor Developmental milestones

Answer 134

6-8 weeks: raises head to 45 degrees when prone 6-8 months: sits without support. At 6m = round back, 8m = straight back (limit 9 months) 8-9 months: crawling 10 months: cruising around furniture 12 months: walking unsteady (limit 18m) 15 months: walks steadily

Question 135

Vision and fine motor milestones

Answer 135

6 weeks: follows object by moving head (limit 3 months) 4 months: reaches for toys 4-6 months: palmar grasp 7 months: Transfers objects between hands 10 months: pincer grip (limit 12 months) 16-18 months: makes marks with crayons ``` 18 months: 3 blocks 2 years: 6 blocks 2.5 years: 8 blocks 3 years: 3 block bridge 4 years: steps ``` ``` 2 years - line 3 years - circle 3.5 years - cross 4 years - square 5 years - triangle ```

Question 136

Hearing, speech and language milestones

Answer 136

Newborn - startles to loud noise 3-4 months: vocalises alone or when spoken too. Coos and laughs 7 months: turns to soft sounds out of sight. Uses should indiscriminately 10 months: sounds used discriminately to parents 12 months: 2-3 words (other than mama and dada) 18 months: 6-10 words. Shows 2 parts of body. 20-24 months: uses 2+ words to make simple phrases 2.5-3 years: talks constantly in 3-4 word sentences

Question 137

Social, emotional and behavioural normal milestones

Answer 137

6 weeks: smile 6-8 months: puts food in mouth 10-12 months: waves bye. peek a boo. 12 months: drinks from a cup 2 handed 18 months: self feeds with spoon 18-24 months: symbolic play 2 years: dry by day. pulls off some clothing 2.5-3 years: parallel play, starting interactive play. taking turns

Question 138

Standardised testing of milestones

Answer 138

Screening - schedule of growing skills - Denver development screening test Assessment - Griffith's/ Bailey Infant Development scales Formal IQ testing

Question 139

Define developmental delay

Answer 139

Slow acquisition of all skills (global delay) or one of specific delay particularly in relation to developmental problems in 0-5 years

Question 140

Define learning difficulty

Answer 140

Used in relation to children of school age and may be cognitive, physical or both

Question 141

Define impairment

Answer 141

Loss or abnormality of physiological function or anatomical structure

Question 142

Define disability

Answer 142

Restriction or lack of ability due to impairment

Question 143

Features suggestive of neurodevelopmental concerns

Answer 143

``` Positive FHx Antenatal screening positive Neonatal encephalopathy Birth asphyxia Dysmorphic features Pre-term infants Abnormal neurological behaviour Global delay ```

Question 144

Conditions that cause abnormal development and learning difficulty PRE NATAL

Answer 144

PRE-NATAL - Genetic: Down's, Fragile X - Cerebral dysgenesis: microcephaly, absent corpus collosum, hydrocephalus - Vascular: occlusions, haemorrhage - Metabolic: hypothyroid, phenylketonuria - Teratogens: alcohol, drugs - Infection: rubella, CMV, toxoplasmosis, HIV - Tuberosclerosis, neurofibromatosis

Question 145

Conditions that cause abnormal development and learning difficulty PERINATAL

Answer 145

Extreme prematurity Birth asphyxia - hypoxic ischaemic encephalopathy Metabolic - hypoglycaemia, hyperbilirubinaemia

Question 146

Conditions that cause abnormal development and learning difficulty POSTNATAL

Answer 146

Infection: meningitis, encephalitis Anoxia: suffocation, near drowning, seizures Trauma: head injury Vascular: stroke

Question 147

Investigations for developmental delay

Answer 147

Cytogenic - chromosome karyotyping - Fragile X analysis Metabolic: TFTs, LFTs, U&Es, plasma amino acids CK, blood lactate, ammonia ABG Urate, ferritin ``` Congenital infection screen Cranial US in newborn CT/MRI Skeletal survey - bone age EEG Nerve conduction studies hearing. vision. ```

Question 148

Global developmental delay

Answer 148

All fields Usually apparent in the first 2 years Always investigate for cause Gets clearer to define as child gets older

Question 149

Abnormal motor development symptoms

Answer 149

``` Delay in acquisition of motor skills Balance problems Abnormal gait Asymmetry of hand use - hand dominance not acquired until 1-2 years Involuntary movements Loss of motor skills (rare) ``` usually presents between 3months-2years

Question 150

Causes of abnormal motor development

Answer 150

``` Central motor deficit - cerebral palsy Congenital myopathy Primary muscle disease Spinal cord lesions - spina bifida Global developmental delay ```

Question 151

Define cerebral palsy

Answer 151

Abnormality of movement and posture causing activity limitation attributed to non-progressive disturbances that occurred in developing foetal or infant brain Often accompanied by distrubances in cognition, communication, perception, sensation or behavioural problems.

Question 152

Causes of cerebral palsy

Answer 152

80% antenatal - Vascular occlusion - cortical migration disorders - structural maldevelopment - genetic syndromes - congenital infections 10% hypoxic ischaemic injury during delivery 10% post natal - meningitis/encephalitis - encephalopathy - head trauma - hypoglycaemia - hydrocephalus - hyperbilirubinaemia

Question 153

Presentation of cerebral plasy

Answer 153

``` Abnormal limb and/or trunk posture Abnormal tone in infancy Delayed motor milestones Feeding difficulties - slow feeding, gagging, vomiting Abnormal gait once walking Asymmetric hand function <12 months Primitive reflexes may persist ```

Question 154

Types of cerebral palsy

Answer 154

Spastic - can be split into hemiplegia, quadriplegia or diplegia Dyskinetic Ataxic

Question 155

Spastic CP

Answer 155

``` Damage to upper motor neurone limb tone increases (spasticity) Brisk reflexes, extensor plantars Tone is velocity dependent (faster movement = increased tone) Presents early ```

Question 156

Spastic CP - hemiplegia

Answer 156

Arms > Legs Unilateral Face spared presents at 4-12 months Fisting of hand, flexed arm, pronated forearm Then tiptoe walking If large brain lesion may have hemianopia

Question 157

Spastic CP - quadriplegia

Answer 157

All 4 limbs Severe Extensor posturing Poro head control Low central tone Seizures, microcephaly, moderate-severe LD May have history of hypoxic ischaemic injury

Question 158

Spastic CP - diplegia

Answer 158

``` Legs >> arms All 4 limbs Walking abnormal Can appear normal arm movements Assocaited with pre-term births due to periventricular haemorrhage ```

Question 159

Dyskinetic CP

Answer 159

``` Movements which are involuntary, uncontrolled, occasionally stereotypes Often more evident on movement - Muscle tone variable - primitive reflexes predominate - Can have: chorea, athetosis, dystonia - Intellect usually intact - Usually presents with floppiness and poor trunk control Movements appear ~ 1 year ``` Most commonly caused by hypoxic ischaemic brain injury

Question 160

Ataxic CP

Answer 160

``` Hypotonic Most are genetic Early trunk and limb hypotonia Poor balance Delayed motor development Inco-ordinate movements Intention tremor Ataxic gait ```

Question 161

Causes of speech and language delay

Answer 161

Hearing loss Global developmental delay Difficulty in speech production - cleft palate, CP Environmental deprivation - lack of opportunity for social interaction Normal variant / familial pattern

Question 162

Speech and language disorders

Answer 162

Disorders in - Language comprehension - Language expression - Phonation and speech production e.g. stammer (dysfluency), dysarthria, dyspraxia - Pragmatics - Social and communication disorders

Question 163

Testing language development

Answer 163

Symbolic Toy Test - for early language development | Reynell test - for expressive/receptive language in pre-school children

Question 164

Dyspraxia

Answer 164

Disorder of motor planning or execution No findings in neurological exam Disorder of higher cortical function ``` Problems with: - Hand writing - Dressing - Cutting up food - Poorly established laterality - Copying and drawing - Difficulty co-ordinating biting and chewing. Drooling common. Achieves normal gross motor milestones ```

Question 165

Dyslexia

Answer 165

Disorder of reading skills disproportionate to IQ | Reading age > 2 years behind chronological age

Question 166

Causes of sensorineural hearing impairment

Answer 166

Antenatal: congenital infection, pre-term, hyperbilirubinaemia, hypoxic ischaemic brain injury Post-natal: meningitis, encephalitis, trauma, drugs, neurodegenerative disease

Question 167

Sensorineural hearing loss in child

Answer 167

``` 1 in 1000 May be profound Does not improve May progress Manage with amplification and cochlear implant if required ```

Question 168

Conductive hearing loss

Answer 168

Causes: otitis media + effusion, Eustachian tube dysfunction (Down's, cleft palate, mid facial hypoplasia), wax Intermittent or resolves Max 60dB hearing loss

Question 169

Stabismus

Answer 169

Squint - misalignment of visual axes - Refer after 3m to ophthalmology - Usually caused by failure to develop binocular vision due to refractive errors - Concomitant: common, refractive error. generally convergent squint eye - paralytic: varies with gaze direction due to paralysis of motor nerves: SINISTER ? space occ lesion

Question 170

Aetiology of visual impairment in child

Answer 170

Genetic - cataracts, albinism, retinal dystrophy, retinoblastoma Antenatal: congenital infection, retinopathy of immaturity, hypoxic ischaemic encephalopathy, trauma, optic nerve hypoplasia Postnatal - trauma, infection, juvenile idiopathic arthritis

Question 171

Gross motor function classification

Answer 171

Level 1 - walks without limitations Level 2 - walks with limitations Level 3 - walks with handheld mobility device Level 4 - self-mobility without limitations (can be powered mobility) Level 5 - transported in manual wheelchair

Question 172

Common medical problems in those with developmental delay

Answer 172

Hearing Vision Orthopaedic (fixed contractures, spasms, spinal deformity, osteoporosis, fractures) GI - GORD, constipation, aspiration GU - UTI, delay in continence, neuropathic bladder Resp - infections, aspiration, chronic lung disease, sleep apnoea Neuro - epilepsy, CP, microcephaly, hydrocephalus Failure to thrive Poor weight gain

Question 173

Causes of vomiting in infants

Answer 173

``` GORD Feeding problems Infection (gastroenteritis, meningitis, UTI, pertussis, Otitis media) Dietary protein intolerances Obstruction - Pyloric stenosis - Atresia - Intussusception - Malrotation - Volvulus - Strangulated inguinal hernia - Hirschsprung's disease Inborn errors of metabolism Congenital adrenal hyperplasia Renal failure ```

Question 174

Causes of vomiting in Pre-school children

Answer 174

``` Gastroenteritis infection Appendicitis Obstruction - Intussusception - Malrotation - Volvulus - Adhesions Raised ICP Coeliac disease Renal failure Testicular torsion Metabolism errors ```

Question 175

Causes of vomiting in school age+ adolescents

Answer 175

``` Gastroenteritis Infection (sepsis, meningitis, pyelonephritis) Peptic ulcers H. pylori infection Appendicitis Migraine Raised ICP DKA Coeliac Alcohol/drugs Cyclical vomiting syndrome Bulimia, anorexia Pregnancy Testicular torsion ```

Question 176

Red flags for vomiting

Answer 176

``` Bile stained Haematemesis Projectile vomiting in first few weeks Abdominal tenderness Abdominal distension hepatosplenomegaly Blood in stool Severe dehydration Bulging fontanelle FTT ```

Question 177

What does bile stained vomit indicate?

Answer 177

Intestinal obstruction | Surgical emergency until proven otherwise

Question 178

What does bulging fontanelle indicate?

Answer 178

Raised ICP

Question 179

Define posseting

Answer 179

Small amounts of milk which accompany the return of swallowed air

Question 180

GORD

Answer 180

Involuntary passage of gastric contents into oesophagus Extremely common in infancy Due to inappropriate relaxtion of LOS due to functional immaturity Usually resolves by 1 year More common in - CP - Pre-term infnats - Post-op oesophageal atresia

Question 181

Investigations for GORD

Answer 181

Usually just a clinical diagnosis - 24 hour oesophageal pH monitoring to quantify degree of reflux - 24 hour impedance monitoring - Endoscopy and biopsy

Question 182

Management of GORD

Answer 182

``` Parental reassurance Adding inert thickening agents to feed Position in 30 degrees prone after feeds Ranitidine/Omeprazole Consider cow's milk allergy Surgery only if unresponsive (Nissen fundoplication) ```

Question 183

Pyloric stenosis - Define - Presentation - Epidemiology

Answer 183

Hypertrophy of pyloric muscle causing gastric outlet obstruction. Presents at 2-7 weeks Increase in boys (4:1), first borns, maternal FHx Vomiting increases in frequency and forcefulness, becomes projectile Weight loss if delayed presentation Hypochloraemic metabolic alkalosis

Question 184

Diagnosis of pyloric stenosis

Answer 184

Give feed to calm the baby and then examine - Gastric peristalsis may be seen as wave from L to R - Palpable pyloric mass in RUQ - US useful if diagnosis in doubt

Question 185

Management of pyloric stenosis

Answer 185

Correct fluids and electrolytes Pyloromyotomy - division of hypertrophied muscle Can feed within 6 hours of Op Discharged after 2 days

Question 186

Infant colic

Answer 186

Paroxysmal, inconsolable crying or screaming Drawing up of knees and passage of excessive flatus 40% of all babies in first few weeks, resolves by 4 months Benign but frustrating Give support and reassurance May be due to milk protein allergy or GORD

Question 187

Appendicitis

Answer 187

Most common cause of abdo pain requiring surgery Uncommon in under 3s Symptoms: anorexia, vomiting, central colicky abdominal pain Signs: flushed face, low grade fever, abdo pain, increased on movement, tenderness at McBurneys point Perforation is rapid as omentum less well developed Appendectomy or IV ABx

Question 188

Non-specific abdo pain and mesenteric adenitis

Answer 188

Abdo pain resolves in 24-48 hours Pain less severe than appendicitis Often accompanied by URTI and cervical lymphadenopathy Can only be diagnosed if large mesenteric nodes notes on laparatomy

Question 189

Intussusception

Answer 189

Invagination of proximal bowel into a distal segment Most commonly ileum into caecum Most common cause of obstruction after the neonatal period Peaks at 3 months-2 years

Question 190

Presentation of intussusception

Answer 190

Paroxysmal severe colicky pain and pallor Draws up legs Initially recovers between painful episodes then lethargic May refuse feeds May vomit, becomes bile stained Sausage shaped mass in abdomen Redcurrant jelly stool (blood stained mucus) Abdominal` distension or shock

Question 191

Investigations and management of intussusception

Answer 191

X-ray: distended small bowel, absence of gas pattern in distal colon US can confirm diagnosis Management - Rectal air insufflation unless peritonitis present - Surgery if that fails or peritonitis

Question 192

Meckel diverticulum

Answer 192

Ileal remnant of vitello-intestinal duct which contains ectopic gastric mucosa or pancreatic tissue Present in 2% Asymptomatic in most Severe rectal bleeding, neither fresh or melena Can present as intussusception, volvulus or appendicitis. Treat with surgical resection

Question 193

Malrotation

Answer 193

During rotation of the small bowel in foetal life the mesentery is not fixed at the duodenojejunal flexure or ileocecal region. Predisposed to volvulus Ladd bands may cross the duodenum, contributing to bowel obstruction Presents - Obstruction with bilious vomiting - obstruction with compromised blood supply Surgery untwists the volvulus. Does not correct the Malrotation. Remove appendix to avoid later confusion.

Question 194

Define recurrent abdominal pain

Answer 194

Pain sufficient to interrupt normal activities Lasts >3 months 10% of school age children

Question 195

Causes of recurrent abdominal pain

Answer 195

Cause only found in 10% - IBS. Constipation. Dyspepsia. Abdominal migraine. Gastritis. Peptic ulcer. IBD. Malrotation - Dysmenorrhoea. Ovvairan cysts. PID - Hepatitis, gallstones, pancreatitis - UTI - Bullying, abuse, stress

Question 196

Abdominal migraine

Answer 196

Associated with abdominal pain and headaches. In some abdominal symptoms predominate Midline pain with vomiting

Question 197

IBS

Answer 197

Altered GI motility and abnormal sensation of intra-abdominal events Symptoms can occur post GI infection Abdominal pain, often worse before or relieved by defaecation Loose stools/constipation Bloating Tenesmus

Question 198

Causes of gastroenteritis

Answer 198

``` Most commonly ROTAVIRUS (60% of <2s) Increased in early spring and winter - Adenovirus - Norovirus - Calcivirus - Coronavirus - Campylobacter - E.coli ```

Question 199

Degrees of dehydration

Answer 199

No clinically detectable dehydration (<5% body weight) Clinical dehydration (5-10%) Shock (>10%)

Question 200

Signs and symptoms of dehydration

Answer 200

``` Appears unwell or deteriorating Altered responsiveness, irritable, lethargic Decreased consciousness (shock) Pale or mottled skin (shock) Cold peripheries Sunken eyes Dry mucus membranes Tachycardia Tachypnoea Weak pulse Increased cap refill (shock) Decreased skin turgor Hypotension (shock) ```

Question 201

Management of dehydration

Answer 201

``` CLINICAL DEHYDRATION Oral Rehydration Solution Often and small volumes Consider NG - If deteriorating step 2 ``` SHOCK - IV therapy - Rapid 0.9% NaCl - Consider ICU - Then step 2 2. IV therapy for rehydration. Replace deficit + maintenance Monitor U&Es, creatinine, glucose After: full strength milk. Reintroduce solid food. Diligent hand washing. No school for 48 hours.

Question 202

Post-gastroenteritis syndrome

Answer 202

Introduction of normal diet can cause watery diarrhoea | Normally ORS for further 24 hours then reintroduce solids again.

Question 203

Presentation of malabsorption

Answer 203

Abnormal stools Failure to thrive Specific nutrient deficiencies

Question 204

Coeliac disease

Answer 204

Gliadin provokes immunological response in proximal small bowel. 1 in 3000 Presents as profound malabsorption aged 8 months - 2 years after introduction of gluten. FTT and abdominal distension Buttock wasting Abnormal stools Positive IgA tissue transglutaminase Diagnosis on small bowel biopsy

Question 205

Causes of nutrient malabsorption

Answer 205

Cholestatic liver disease or biliary atresia Lymphatic leakage or obstruction Short bowel syndrome Loss of terminal ileum function (Crohn's, resection) Exocrine pancreatic dysfunction - CF. Absent lipase, protease, amylase Small intestine mucosal disease (Coeliac, lactase deficiency)

Question 206

Acrodermatitis enteropathica

Answer 206

Due to zinc malabsorption | Erythematous rash and mouth and anus.

Question 207

Red flags of constipation

Answer 207

Failure to pass meconium in first 24 hours (HIRSCHSPRUNG'S) FTT/growth failure Gross abdominal distension (Hirschprung's) Abnormal lower limb neurology Spina bifida signs Abnormal anorectal anatomy Perianal bruising, fissures = sexual abuse

Question 208

Long standing constipation

Answer 208

Rectum becomes over distended Loss of sensation to poo Involuntary soiling Requiring long term laxative use

Question 209

Hirschsprung's disease

Answer 209

Absence of ganglion cells from myenteric and submucosal plexus of large bowel results in narrow contracted segment. Abnormal bowel extends from rectum ending in normally innervated dilated colon 75% only affects rectum/sigmoid 10% whole colon **failure to pass meconium** in first 24 hours Later abdominal distension and bile stained vomiting On DRE narrow segment and gush of fluid/stool/flatus on removal Diagnosed by absence of ganglion cells and presence of ACHE + nerve trunks on suction rectal biopsy Management: colostomy with later anastomoses.

Question 210

Management of constipation

Answer 210

If faeces not palpable - encouragement and close supervision If palpable - Macrogol e.g. Movicol for 2 weeks - If stools passed done - If not then add stimulant laxative e.g. senna with or without lactulose - If no success then consider enema or manual evacuation under GA After - balanced diet, sufficient fluids, maintenance laxatives

Question 211

Epidemiology of UTI

Answer 211

3-7% of girls <6 1-2 % of boys <6 12-30% have recurrence within 1 year Up to 50% have structural abnormality

Question 212

Presentation of UTI in infant

Answer 212

``` Fever Vomiting Lethargy FTT/ poor feeding Jaundice Septicaemia Offensive urine Febrile convulsion ```

Question 213

Presentation of UTI in children

Answer 213

``` Dysuria Frequency Abdo/loin pain Fever/rigors Lethargy Anorexia D&V Haematuria Offensive cloudy urine Febrile convulsions Recurrence of enuresis ```

Question 214

Collecting urine in children

Answer 214

``` Clean catch - waiting clean pot when nappy removed *recommended* Adhesive plastic bag on cleaned perineum Urethral catheter if urgent Suprapubic aspiration - rarely done In older children MSU ```

Question 215

In UTI - dipstick findings and when to treat

Answer 215

Positive leukocytes and nitrites = regard as UTI and treat Negative leukocytes, positive nitrites = start Abx, diagnosis depends on urine culture Positive leukocyte, negative nitrites = only Abx if clinical UTI If both negative - UTI unlikely.

Question 216

Aetiology of UTI

Answer 216

1. E.coli 2. Klebsiella 3. Proteus (increased in boys) 4. Pseudomonas 5. Strep faecalis

Question 217

Factors contributing to UTI

Answer 217

Incomplete bladder emptying - Infrequent voiding - Vulvitis - Obstruction from constipation - Neuropathic bladder - Vesicoureteral reflex

Question 218

Vesicoureteric reflux

Answer 218

Developmental abnormality of vesicoureteric junctions. | Ureters are displaced laterally and enter the bladder directly rather than at an angle

Question 219

Investigations for UTI

Answer 219

Some debate over benefit of investigating Investigate atypical or recurrent UTI Atypical: - Seriously ill/ sepsis - Poor urine flow - Abdominal or bladder mass - Raised CK - Failure to respond to Abx in 48 hours US to detect structural abnormalities Further investigations depends on US findings

Question 220

Management of UTI

Answer 220

If under 3 months - refer to hospital for IV Abx If over 3 months - oral Abx for 7-10 days If only LUTS then 3 days oral ``` High fluid intake Regular voiding Ensure complete emptying Prevent or treat constipation Good perineal hygiene Consider circumcision ```

Question 221

Define enuresis

Answer 221

Lack of bladder control Primary - lack of control to be continent Secondary - loss of previously achieved enuresis

Question 222

Aetiology of primary daytime enuresis

Answer 222

``` Lack of attention to bladder sensation - developmental or psychogenic Too preoccupied to respond Detrusor instability Bladder neck weakness Neuropathic bladder UTI Constipation Ectopic ureter ```

Question 223

Examination/investigation in enuresis

Answer 223

``` Test perineal sensation - S4/5 Urine sample - dip, microscopy and culture US may show bladder pathology Urodynamic studies may be required Spine X-ray or MRI for defects ```

Question 224

Management of enuresis

Answer 224

Once neurological cause excluded - star charts, bladder training, pelvic floor exercises Treat constipation Last resort - anticholinergic drugs (oxybutynin)

Question 225

Causes of secondary enuresis

Answer 225

Emotional upset most common UTI Polyuria from osmotic diuresis (diabetes, CKD) Test osmolality of urine

Question 226

Management of nocturnal enuresis

Answer 226

Straightforward but pain staking Treatment is rare in under 6s Explain to parent and child than problem is beyond conscious control Stop punishment Star chart for each dry night Enuresis alarm - ideally child should get up when alarm indicates wet pants Desmopressin - short term relief (sleepovers, holidays) - synthetic analogue of ADH

Question 227

Epidemiology of nocturnal enuresis

Answer 227

6% of 5 years olds 3% of 10year olds Increased in boys (2:1) Most are psychologically normal - child needs to be free from stress

Question 228

Define encopresis

Answer 228

Voluntary or involuntary passage of faeces, usually into clothing, after the age at which bowel control is usually achieved (any defaecation not in toilet) No known cause Bowel control normally achived by age of 3 Soiling at 4 or older is abnormal

Question 229

Epidemiology of encopresis

Answer 229

1% of 5 year olds | Increased in boys

Question 230

Causes of encopresis

Answer 230

Bowel control never established - disorganised family, understaffed institution, mental retardation Emotion causes - - Regressive soiling due to upsetting event - Aggressive soiling - poor relationships between sibling. Rebellious act. Physical causes - Faecal impaction - Hirschsprung's disease

Question 231

management of encopresis

Answer 231

Assess possible cause Treat faecal retention Give adequate toilet training Emotional support Regressive usually settles with emotional problems. Aggressive more troublesome.

Question 232

Causes of proteinuria

Answer 232

``` Orthostatic proteinuria (only proteinuria when child upright) Glomerular abnormalities - Minimal change disease - Glomerulonephritis - Abnormal GBM Raised glomerular filtration pressure Decreased renal mass Hypertension Tubular proteinuria ```

Question 233

Nephrotic syndrome

Answer 233

Heavy proteinuria Hypoalbuminaemia Oedema

Question 234

Investigations in nephrotic syndrome

Answer 234

``` Urine protein on dipstick FBC and ESR U&Es and creatinine Complement leves C3 and C4 Antistreptolysin O or anti-DNAase B titres Urine microscopy and culture Urine sodium concentration Hep B and C screen ```

Question 235

Steroid sensitive nephrotic syndrome

Answer 235

``` 85-90% of nephrotic syndrome Do not progress to renal failure Increased in boys Increased in Asians Often precipitated by respiratory infections ``` No macroscopic haematuria, normal BP, complement levels and renal function, Most commonly minimal change disease

Question 236

Management of nephrotic syndrome

Answer 236

Oral corticosteroids 60mg/m2 After 4 weeks decrease dose to 40mg/m2 on alternate days for 4 weeks If not responding - renal biopsy

Question 237

Biopsy of minimal change disease

Answer 237

Normal light microscopy | Fusion of podocytes on electron microscopy

Question 238

Complications of nephrotic syndrome

Answer 238

Hypovolaemia - treat with IV albumin Thrombosis - hypercoagulable state Infection 1/3 resolve. 1/3 frequent relapses (steroid dependent). 1/3 infrequent relapses

Question 239

Steroids resistant nephrotic syndrome | CAUSES

Answer 239

Refer to paeds nephrologist - Focal segmental glomerulosclerosis - Membranoproliferative GN (mesangiocapillary GN) - Membranous nephropathy

Question 240

Focal segmental glomerulosclerosis

Answer 240

Most common steroid resisntat nephrotic syndrome Familial or idiopathic 30% cause end stage renal failure in 5 years 20% respond to cyclophosphamide/ciclolsporin/tacrolimus Recurrence post transplant is common

Question 241

Membranoproliferative glomerulonephritis

Answer 241

Also called mesangiocapillary GN Increased in older children Haematuria and low complement present Decreasing renal function over many years

Question 242

Membranous nephropathy

Answer 242

Associated with Hep B May precede SLE Most remit spontaneously in 5 years

Question 243

Congenital nephrotic syndrome

Answer 243

``` Presents in first 3 months Rare Autosomal recessive Increased in Finnish High mortality Albuminuria is so severe that unilateral nephrectomy may be required to control it. ```

Question 244

Appearances of haematuria

Answer 244

Glomerular haematuria - brown urine, deformed red cells and casts +/- proteinuria LUT haematuria = red urine, beginning or end of urinary stream. No proteinuria

Question 245

Causes of Haematuria

Answer 245

Non glomerular - Infection - Trauma - Stones - Tumours - Sickle cell disease - Bleeding disorders - Renal vein thrombosis - Hypercalciuria Glomerular - Acute glomeruloneprhtisi (usually with proteinuria) - Chronic glomerulonephritis (usually with proteinuria) - IgA nephropathy - Familial nephritis (Alport's) - Thin basement membrane

Question 246

Investigations in haematuria

Answer 246

Urine microscopy and culture Protein and calcium excretion Kidney and urinary tract US U&Es, creatinine, calcium, phosphate, albumin FBC, platelets, clotting screen, sickle cell screen If suggestive of glomerular haematuria - ESR. Complement levels. Anti-DNA antibodies - Throat swab. - Abtistreptolysin O / anti-DNAase B titres - Hep B and C screen - Renal biopsy if indicated

Question 247

Acute nephritis

Answer 247

Hypertension Haematuria Oedema Decreased urine output and volume overload

Question 248

Causes of nephritic syndrome

Answer 248

``` Post infections (streptococcal) Vasculitis - Henoch-Schonlein putpura - SLE - Wegner's granulomatosis - Polyarteritis nodosa IgA nephropathy Mesangiocapillary GN Goodpasture's syndrome ```

Question 249

Post-streptococcal glomerulonephritis

Answer 249

``` Following strep sore throat or skin infection Raised anti-DNAase B titres Low C3 complement Long term prognosis good ASO positive ```

Question 250

Features of Henoch-Schonlein purpura

Answer 250

Characteristic skin rash - Symmetrical, over buttocks and extensor limb surfaces - Trunk spared - Urticarial, rapidly becoming maculopapular and purpuric - palpable Arthralgia Periarticular oedema Adnominal pain - colicky. Haematemesis or malena Glomerulonephritis Increased in boys aged 3-10 years Often preceded by URTI

Question 251

Alport's disease

Answer 251

X linked recessive Haematuria Progresses to end stage renal failure by early adult life Associated with deafness and ocular defects

Question 252

Pathophysiology of meningitis

Answer 252

Infection of meninges usually follows bacteraemia Much of the damage is caused by the host response Release of inflammatory mediators and leukocytes causes endothelial damage Causes cerebral oedema. Raised ICP, decreased cerebral blood flow Inflammation causes vasculopathy and cerebral cortical infarction Fibrin deposits can block resorption of CSF causing hydrocephalus

Question 253

Organisms causing meningitis in newborns

Answer 253

Group B streptococcus E. Coli and other coliforms Listeria monocytogenes

Question 254

Organisms causing meningitis in 1 months - 6 years

Answer 254

Neisseria meningitides Streptococcus pneumonia Haemophilus influenza (not common beyond 6 years)

Question 255

Presentation of meningitis (symptoms)

Answer 255

``` Fever Headache Photophobia Neck stiffness Irritable Reduced consciousness Lethartgy Poor feeding Seizures Vomiting Hypotonia ```

Question 256

Signs of meningitis

Answer 256

``` Fever Purpuric rash neck stiffness (not always in infants) Bulging fontanelle Opistghotonus - arching back + Brudzinski's and Kernig's sign Signs of shock Focal neurology Papilloedema (rare) ```

Question 257

Investigations for meningitis

Answer 257

FBC (raised WCC and platelets) U&Es LFTs Raised CRP Blood cultures LP for red cells, white cells, glucose, culture and microscopy Blood/urine/CSF for rapid antigen testing and PCR MRI

Question 258

Brudzinski's sign

Answer 258

Lay supine | Flexion of neck causes flexion of the legs

Question 259

Kernig's sign

Answer 259

Lay supine with hips and knees flexed | On straightening of legs - pain

Question 260

CSF changes in bacterial meningitis

Answer 260

Purulent CSF Raised protein Low glucose Raised WCC

Question 261

CSF changes in viral meningitis

Answer 261

Clear CSF Normal or raised protein Normal or low glucose Raised lymphocytes

Question 262

CSF changes in TB meningitis

Answer 262

Turbid/Clear or viscous CSF Mildly raised lymphocytes Very raised protein Very low glucose

Question 263

CSF changes in encephalitis

Answer 263

Clear CSF Normal or raised protein Normal or low glucose Raised lymphocytes

Question 264

Management of meningitis

Answer 264

No delay in IV ABx and supportive therapy ABx depend on pathogen found Start with 3rd gen cephalosporin - cefotaxime or ceftriaxone Dexamethasone to decrease complications

Question 265

Complications of meningitis

Answer 265

Hearing loss Local vasculitis - CN palsies or other focal lesions Local cerebral infection - epilepsy Subdural effusion - particularly in H. influenzae or pneumococcal. Most resolve spontaneously Hydrocephalus Cerebral abscess

Question 266

Causes of viral meningitis

Answer 266

``` 2/3 are viral Enteroviruses EBV Adenovirus Mumps ``` Less severe, makes full recovery If fail to respond to treatment or supportive therapy consider atypical pathogens - Mycoplasma or Borelia burgdorferi (lyme disease)

Question 267

Encephalitis

Answer 267

Inflammation of brain substance Meninges can also be affected Can be caused by - Direct invasion of cerebrum by neurotoxic virus e.g. herpes simplex - Delayed brain swelling following disordered immunological response (post-infectious encephalopathy from chicken pox) - Slow virus infection (HIV)

Question 268

Causes of encephalitis

Answer 268

``` Enteroviruses Respiratory viruses Herpes viruses (HSV, varicella, HHV6) Mycoplasma Boreglia burgdorferi Rocky mountain spotted fver ``` Herpes simplex virus is rare but has devastating consequences

Question 269

Presentation of encephalitis

Answer 269

As meningitis - manage as meningitis until proven otherwise ``` Fever Headache Photophobia Neck stiffness Irritable Reduced consciousness Lethartgy Poor feeding Seizures Vomiting Hypotonia ```

Question 270

Management of encephalitis

Answer 270

High dose IV aciclovir

Question 271

HSV encephalitis

Answer 271

Destructive infection EEG and CT/MRI may show focal changes particularly in temporal lobes 3 weeks of acyclovir required Untreated mortality rate = 70% Survivors often have severe neurological problems

Question 272

Define fever

Answer 272

Temperature over 37.5 degrees

Question 273

How to measure fever

Answer 273

In under 4 weeks - electronic thermometer in axilla Over 4 weeks as above or tympanic infrared thermometer

Question 274

Fever & age of child

Answer 274

Under 3 months - often bacterial | Over 3 months - more likely to be viral

Question 275

RFs for infection

Answer 275

``` Illness of other family members Prevalent illnesses in community Unimmunised recent travel abroad Contact with animals - brucellosis Immunodeficient ```

Question 276

Red flags for child with fever

Answer 276

``` Maculopapular non blanching rash Irritable/reduced consciousness Over 38 degrees if <3m, over 39 if >3m Bulging fontanelle, neck stiffness, seizures, focal neurology Bile stained vomiting Significant respiratory distress Severe dehydration or shock ```

Question 277

Septic screen

Answer 277

Blood cultures FBC CRP Urine sample Consider - CXR - LP - Rapid antigen screen on blood/urine/CSF - Meneningococcal and pneumococcal PCR on blood/CSF

Question 278

Causes of fever & presentation

Answer 278

``` Meningitis Seizures - meningitis, encephalitis Otitis media Tonsillitis Epiglottitis, viral croup - stridor URTI Appendicitis, pyelonephritis, hepatitis - abdo pain Gastroenteritis - diarrhoea Septicaemia - shock Septic arthritis ```

Question 279

Causes of prolonged fever

Answer 279

``` Bacterial infection - endocarditis Kawasaki disease Drug reaction Malignant disease Connective tissue disorder ```

Question 280

Toxic shock syndrome

Answer 280

``` Caused by toxin producing Staph aureus and Group A strep Characterised by: - Fever > 39 - Hypotension - Diffuse erythematous macular rash ``` 1-2 weeks later - desquamation of fingers and toes. Toxin causes organ dysfunction: mucositis, D&V, renal and liver impairment, altered consciousness need to surgical debride areas of infection + 3rd cephalosporin + clindamycin

Question 281

Necrotising fascitis

Answer 281

Severe subcut infection involving skin, fascia and muscle Enlarges rapidly, leaving poorly perfused areas of necrotic tissue Severe pain and systemic illness requiring ITU Surgical debridement necessary + IV abx` Caused by Staph Aureus or group A strep

Question 282

Impetigo

Answer 282

High contagious skin infection with staph or strep No school until resolved Weepy red honey coloured crush Increased if pre-existing skin disease Start as erythematous macules then vesicular/pustular then secretes the honey coloured exudate. Lesions common on hands, face and neck Topical antibiotics

Question 283

Cuases of maculopapular rash + fever

Answer 283

``` Human herpes virus 6&7 Enteroviral rash Parovirus (slapped cheek) measles rubella scarlet fever (group A strep) erythema marginatum (rheumatic fever) Salmonella typhi (rose coloured spots_ Lyme disease Kawasaki disease Juvenile idiopathic arthritis ```

Question 284

Causes of vescicular/pustular rash + fever

Answer 284

``` Varicella zoster Herpes simplex Coxsackie (hand foot and mouth) Impetigo Boils Toxic epidermal necrolysis Erythema multiforme Steven-Johnson's syndrome ```

Question 285

Causes of petechial purpuric rash + fever

Answer 285

``` Meningitis Infective endocarditis Enterovirus Henoch-Schonlein purpura Vasculitis Malaria Thrombocytopaenia ```

Question 286

Herpes viruses

Answer 286

``` HSV 1 HSV 2 - Herpes simplex 1 and 2. Responsible for cold sores and genital Varicella Zoster CMV EBV Human herpes virus 6, 7 and 8 HHV8 is associated with Kaposi's sarcoma ``` Hallmark 1. Primary infection 2, Latency and long term persistence 3. Reactivation

Question 287

Herpes simplex 1 & 2

Answer 287

Wide variety of presentations Treat with acyclovir Can by asymptomatic Can cause: - Gingivostomatitis - most common in children, 10mont-3years. vesicular lesions on lips, gums, tongue and soft palate. Painful ulceration - Eczema herpeticum - herpes infection + eczema - Herpetic whitlow

Question 288

Chicken pox

Answer 288

Primary infection with varicella zoster 200-500 lesions on head and trunk then progresses to limbs Appears as crops of papules, vesicles with surrounding erythema and pustules for up to 1 week lesions may occur on palate. Can causes permanent depigmentation from scratching If new lesions after 10 days = defective immunity Complications: - Staph/strep infection - Encephalitis - Purpura fulminans - large areas of skin necrosis Supportive treatment unless immunocompromised then IV acyclovir

Question 289

EBV infection

Answer 289

``` Causes infectious mononucleosis, Burkitt's lymphoma or associated with nasopharyngeal carcinoma - Fever - Malaise - Tonsillopharyngitis - Lymphadenopathy - Splenomegaly (50%) hepatomegaly (10%) - Maculopapular rash Symptoms persist for 1-3 months ` Diagnosed with atypical lymphocytes, Positive monospot and IgM and IgG to EBV ```

Question 290

CMV infection

Answer 290

``` Transmitted in saliva, genital secretions or breast milk Can cause infectious mononucleosis symptoms can cause - retinitis - pneumonitis - bone marrow failure - encephalitis - hepatitis - colitis - oesophagitis ```

Question 291

Human Herepes virus 6 and 7

Answer 291

Most children are infected by age of 2 from family members Can cause exanthem subitum - roseola infantum High fever and malaise for few days followed by macular rash as fever wains

Question 292

Parvovirus B19

Answer 292

Slapped cheek Causes erythema infectiosum or 5th disease - most common - fever, malaise, headache, myalgia - followed by slapped cheek rash on face - then maculopapular rash "lace like rash" on trunk and limbs outbreaks in spring Infects erythroblastoid red cell precursors in bone marrow Can cause aplastic crisis - in children with chronic haemolytic anaemias Maternal transmission can cause foetal hydrops

Question 293

Enteroviruses

Answer 293

Includes coxsackie, echovirus, poliovirus Transmission if faecal oral 90% are asymptomatic Hand, foot and mouth disease - painful vesicular lesions on hands, feet or mouth. Self resolving Herpangia - vesicular and ulcerated lesions on soft palate and uvula. Meningitis/encephalitis Bornholme disease/ Pleurodynia = fever, pleuritic chest pain and muscle tenderness

Question 294

Measles

Answer 294

Rash - Spreads downwards from behind ears to whole body - From discrete maculopapular to confluent and blotchy - May desquamate in 2nd week Cough Conjunctivitis and cold symptoms Koplik's pots - white spots on buccal mucosa Complications - Encephalitis (mortality 15%0 - Subacute sclerosing panencephalitis - 7 years post infection Treatment symptomatic

Question 295

Mumps

Answer 295

Spread via droplet infection. Infection from parotid gland before dissemination Fever, malaise, parotitis (earache and pain on eating) Fever settles in 3-4 days Raised amylase Mild and self-limiting Hearing loss can occur (transient)

Question 296

Rubella

Answer 296

German measles - Causes damage to foetus - Prodrome with mild low grade fever - Maculopapular rash from face down - Fades in 3-5 days - Not itchy (in children) - Prominent lymphadenopathy (particular post-auricular) No treatment

Question 297

Kawasaki disease

Answer 297

Systemic vasculitis 6 months - 4 years Increased in Japanese and Blacks High fever, difficult to control for > 5 days Need 4 of 5 other features - Conjunctival infection - Mucus membrane changes - cervical lymphadenopathy - polymorphous rash - peeling of skin on fingers and toes Can cause coronary aneurysms Mortality 1-2%

Question 298

Pathogens causing respiratory infections

Answer 298

80-90% are VIRAL - Respiratory syncytial virus - Rhinovirus - Parainfluenza - Influenza - Metapneumovirus - Adenovirus Bacterial - Streptococcus pneumoniae - H. influenzae - morexella catarrhalis - Bordetella pertussis - mycoplasma pneumoniae

Question 299

RF for respiratory infection

Answer 299

``` Parental smoking Low socioeconomic status Decreased nutrition Underlying lung disease Congenital heart disease Boys Immunodeficiency ```

Question 300

Classification of respiraotyr infections

Answer 300

``` URTI laryngeal/tracheal infection Bronchitis Bronchiliolitis Pneumonia ```

Question 301

URTI

Answer 301

Includes - Common cold (coryza) - Sore throat (pharyngitis, tonsillitis) - Acute otitis media - Sinusitis

Question 302

presentation of URTI

Answer 302

``` Nasal discharge and blockage Fever Painful throat Earache Cough Difficulty feeding febrile convulsions Acute exacerbation of asthma ```

Question 303

Causes of coryza

Answer 303

Most common infection * rhinovirus Coronavirus Respiratory syncytial virus

Question 304

Causes of pharyngitis

Answer 304

``` Sore throat Adenovirus Enterovirus Rhinovirus In older child Group A beta haemolytic strep ```

Question 305

Causes of tonsillitis

Answer 305

Group A haemolytic strep | EBV

Question 306

Symptoms and management of tonsillitis

Answer 306

``` Sore throat Headache Apathy Abdo pain Cervical lymphadenopathy White exudate ``` Treat with penicillin (erythromycin) Avoid amoxicillin as maculopapular rash if EBV infection

Question 307

Otitis media

Answer 307

Increased at 6-12 months Tympanic membrane = red and bulging and loss of light reflection Caused by RSV and rhinovirus OR pneumococcus, H.influenzae or M. catharalis Regualr analgesia If ABx = amoxicillin

Question 308

When is tonsillectomy indicated

Answer 308

Recurrent severe tonsillitis Peritonsilar abscess Obstructive sleep apnoea

Question 309

Causes of upper airways obstruction / STRIDOR

Answer 309

``` * viral laryngotracheobronchitis * - "Croup" Epiglottiits Bacterial tracheitis Inhalation of smoke and hot air Trauma to throat Foreign body Hypocalcaemia Measles Infectious mononucleosis Diphtheria ```

Question 310

Characteristic features of laryngeal and tracheal infections

Answer 310

Stridor Hoarseness Barking cough Dyspnoea Can rapidly be life threatening in young chgildren

Question 311

Croup

Answer 311

Laryngotracheobronchitis Mucosal infections & increased secretion Can cause critical narrowing if subglottic 95% viral - parainfluenza, RSV, influenza 6 months - 6 years Barking cough, hoarseness, stridor, fever Symptoms worse at night Low threshold for admission in < 1 year due to narrow airway If severe mange with nebulised adrenaline and ITU

Question 312

Acute epiglottitis

Answer 312

``` Life threatening emergency Caused by H. influenza B Intense swelling of epiglottis 1-6 years Acute onset Toxic looking child Saliva drooling Soft inspiratory stridoe Child sitting immobile, upright with mouth open Admit to ICU ASAP, RSI under GA IV abx ```

Question 313

Whooping cough

Answer 313

Bordetella pertussis Bronchitis highly contagious Catarrhal phase - week of coryza (cold symptoms) Characteristic - INSPIRATORY WHOOP Paroxysmal cough Spasms of cough - worse a night Can cause vomiting, conjunctival haemorrhages, epistaxis 3-6 weeks Symptoms decrease slowly Complications - pneumonia, bronchiectasis

Question 314

Bronchiolitis

Answer 314

Rare after age of 1 90% aged 1-9 months 80% due to RSV (respiratory syncytial virus) Others: metapneumovirus, parainfluenza, rhinovirus, adenovirus, influenza Coryzal symptoms precede a dry cough and SOB Feeding difficulty due to SOB

Question 315

Signs and symptoms of bronchiolitis

Answer 315

``` Sharp dry cough Tachypnoea Subcoastal and intercostal recession Hyperinflation of chest - prominent sternum, downwards displaced liver high pitched wheeze exp>ins Tachycardia Fine end expiratory crackles Cyanosis Pallor ```

Question 316

Features of croup

Answer 316

``` Onset over days Preceding coryza Cough severe and barking Able to drink No drooling of saliva Appears unwell Fever <38.5 Harsh rasping stridor Hoarse voice ```

Question 317

Features of epiglottiits

Answer 317

``` Onset over hours No preceding coryza Absent or slight cough Not able to drink Drooling saliva Toxic looking child Fever > 38.5 Soft whispering stridor Reluctant to speak ```

Question 318

Investigations in URTI

Answer 318

PCR of nasopharyngeal secretions CXR unnecessary if straightforward CXR - hyperinflation of lungs and focal atelectasis

Question 319

Causes of pneumonia in childhood

Answer 319

Newborn - group B strep. gram negative enterococci Infants - RSV, Step pneumoniae, H. influenza, Bordella pertussis, chlamydia trachomatis Over 5s - mycoplasma pneumoniae, strep pneumoniae, chlamydia pneumoniae

Question 320

Symptoms and signs of pneumonia

Answer 320

``` Fever SOB Usually preceded by URTI Cough Lethargy Unwell Tachypnoea Nasal flaring Chest in drawing End expiratory crackles ```

Question 321

Management of pneumonia

Answer 321

``` Most managed at home Admit if sats < 93%, severe raised RR, SOB, grunting, apnoea, not feeding Oxygen for hypoxia Analgesia if required ABx IV fluids for dehydration ```

Question 322

Malaris

Answer 322

From female anopheles mosquito - plasmodium falciparum 700,000 deaths per year Fever, diarrhoea, vomiting, flu like symptoms, jaundice, anaemia, thrombocytopaenia Typical onset 7-10 days after inoculation Children are susceptible to severe anaemia and cerebral malaria Quinine required in most cases

Question 323

Typhoid

Answer 323

Worsening fever, headaches, cough, abdominal pain, anorexia, malaise and myalgia Salmonella typhi GI symptoms may not appear until 2nd week Rose colour spots on trunk Complications: GI perforation, myocarditis, hepatitis, nephritis

Question 324

Dengue fever

Answer 324

Viral infection transmitted by mosquitos Fine erythematous rash, myalgia, arthralgia, high fever 2y rash with desquamation after Dengue haemorrhagic fever when previously infected child reinfected with different strain: severe capillary leak, hypotension most recover

Question 325

Common allergies in children

Answer 325

``` Food allergy Eczema Allergic rhinitis and conjunctivitis Asthma Urticaria Insect sting hypersensitivity Anaphylaxis ```

Question 326

Define hypersensitivity

Answer 326

Objectively reproducible symptoms or signs following exposure to defined stimulus at a dose which is tolerated in normal people

Question 327

Define allergy

Answer 327

Hypersensitivity reaction initiated by specific immunological mechanism. Can be IgE mediated (peanut allergy) or non IgE mediated (Coeliac)

Question 328

Define atopy

Answer 328

Personal or familial tendency to produce IgE antibodies to potential allergens, usually proteins. Strongly associated with asthma, allergic rhinitis and conjunctivitis, eczema and food allergy

Question 329

Pathophysiology of atopy

Answer 329

Allergens bind to IgE sensitised mast cells and basophils Histamine is released from intracellular granules mast cells are concentrated in skin, lungs and GI Histamine causes: - Local vasodilation (erythema) - Increased capillary permeability (wheal) - Vasodilation of surrounding arterioles (flare) - Stimulation of sensory nerves (itching) - Smooth muscle contraction (bronchoconstriction and increased GI motility) - Increased nasal, salivary and gland secretions As histamine is a potent vasodilator, can cause peripheral blood pooling, hypotension and circulatory shock

Question 330

Allergic march

Answer 330

Children develop allergic disorders at different ages - Eczema and food allergy in infancy - Allergic rhinitis and conjunctivitis in preschool/primary school - hayfever often precedes asthma

Question 331

Epidemiology of food allergies

Answer 331

5% of children 3% of adults Increasing incidence ? processed diet ? hygiene hypothesis 2% of infants have cow's milk protein allergy

Question 332

Symptoms of IgE mediated food allergy

Answer 332

``` Pruritus Erythema Diarrhoea Abdominal pain Acute urticaria Acute angio-oedema (usually facial) Oral itching Nausea and vomiting Nasal itching, rhinorrhoea, sneeze Allergic conjunctivitis Cough, SOB, wheeze, asthma Feeling of impending doom CV collapse ```

Question 333

Symptoms of non-IgE mediated food allergy

Answer 333

``` Pruritus Erythema Diarrhoea Abdominal pain Atopic eczema GORD Infantile colic Stools loose/frequent/blood/mucus Constipation Perianal redness Pallor and tiredness FTT Food aversion or avoidance ```

Question 334

Investigations for food allergy

Answer 334

Food diary | Physician supervised oral food challenge, skin prick, food specific IgE tests

Question 335

Describe skin prick testing

Answer 335

Drop of food placed on forearm Needle pricks skin through food Saline for comparison Reaction read after 15-20 minutes

Question 336

Common food allergen

Answer 336

``` Milk Eggs Fish and seafood peanuts Sesame Tree nuts Soybeans Wheat Kiwi ```

Question 337

Investigations for non-IgE mediated food allergy

Answer 337

2-6 weeks trial elimination and reintroduce after to determine symptoms

Question 338

Food protein induced enterocolitis

Answer 338

Presents with projectile vomiting, diarrhoea and FTT for first few months. Usually due to Cow's milk and soy protein formulas non-IgE mediated

Question 339

Eosinophilic oesophagitis and gastroenteritis

Answer 339

Nausea, abdominal pain, reflux, FTT Does not respond to antacids. Non-IgE mediated food allergy

Question 340

Management of food allergies

Answer 340

Food avoidance - particularly if anaphylactic Dietician referral - reading food labels, preventing deficiencies Antihistamines - for mild symptoms e.g. urticaria, itching Adrenaline - if resp symptoms or anaphylaxis Medical emergency bracelets Injection immunotherapy (desensitisation) only used for pollen and venom

Question 341

Non-immunologically mediated food intolerance causes

Answer 341

Metabolic: lactase deficiency Pharmacological: preservatives, artificial food colours, sweeteners, alcohol, caffeine Toxic - contaminated foods Psychological: food aversion Avoid trigger foods

Question 342

Non-immunologically mediated food intolerance symptoms

Answer 342

``` Headache Fatigue GI upset Urticaria Behavioural problems ```

Question 343

Define anaphylaxis

Answer 343

Severe life threatening generalised or systemic hypersensitivity reaction - Sudden onset and rapid progression of symptoms AND - life threatening A/B/C problem

Question 344

Aetiology of anaphylaxis

Answer 344

Occurs when allergen reacts with IgE antibodies on mast cells in type 1 hypersensitivity reaction Rapid release of histamine - Peanuts, pulses, tree nuts (brazil, almond, hazelnut), fish, shellfish, eggs, sesame, milk - bee/wasp sting - antibiotics, NSAIDs, contrast media, anaesthetic drugs

Question 345

presentation of anaphylaxis

Answer 345

``` Initially skin symptoms: generalised itching, urticaria, erythema, rhinitis, conjunctivitis, angio-oedema Dyspnoea Stridor Wheeze Palpitations Tachycardia nausea and vomiting Abdominal pain LOC ```

Question 346

Management of anaphylaxis

Answer 346

ABCDE High flow O2 through non-rebreathe Lie flat Raise legs Adrenaline IM 0.5ml of 1:1000 (500 micrograms) Repeat after 5 minutes if no improvement May benefit from IV adrenaline (anaesthetist only) Establish airway (often intubation) IV fluid challenge - 1L in 10 minutes if hypotensive If further deterioration: salbutamol, ipratropium, aminophylline, mag sulphate) After initial resus - Chlorphenamine 10mg - Hydrocortisone 200mg Lower doses for children - age appropriate

Question 347

Investigations for anaphylaxis

Answer 347

Serum mast cell tryptase can be measured for diagnosis if required. Peak at 1 hour, Do at emergency and then 1-2 hours later

Question 348

Follow up management of anaphylaxis

Answer 348

``` Observe for 6-12 hours If under 16 admit to paediatric ward - Organise allergen testing - Organise epi pen and training - Written management plan - Encourage emergency medical bracelet ```

Question 349

Types of allergic rhinitis

Answer 349

Seasonal allergic rhinitis (hayfever): occurs at certain times of year. Tress grass or pollen Perennial rhinitis - persistent, occurs year round. dust mites, domestic pets Occupational: flour, wood dust, latex gloves

Question 350

Pathogenesis of allergic rhinitis

Answer 350

IgE mediated inflammation of nasal mucosa due to histamine release - Acute phase (mins) - sneezing, increased nasal secretion - Late phase (6-12hours) - nasal obstruction

Question 351

Epidemiology of allergic rhinitis

Answer 351

20% of UK population Children and adolescents tend to have seasonal, adults = perennial Peak onset is in children, 80% diagnosed under 20 10% of 6 year olds, 15% adolescents Often persists into adult hood RFs - History or FH of atopy - Exposure to common allergens - Air pollution - Decreased exposure to infection - Exposure to cigarette smoke

Question 352

Conditions associated with allergic rhinitis

Answer 352

Asthma Eczema Conjunctivitis

Question 353

Presentation of allergic rhinitis

Answer 353

If seasonal. Spring = tree pollen, summer = grass pollen If house or dust mites = worse on waking and worse in winter ``` Sneezing Rhinorrhoea and nasal congestion - usually bilateral - Normal = clear - Infection: yellow/green - If blood and unilateral: tumour, foreign body, nose picking - Blood and bilateral: nose picking, bleeding points, granulomatous disorder Itchy nose and/or palate Eyes: watering, itchy, red, swollen ``` Controlled by antihistamines or topical steroids Nasal mucosa appears swollen and greyish

Question 354

Investigations for allergic rhinitis

Answer 354

usually a clinical diagnosis RAST testing Skin prick testing

Question 355

Management of allergic rhinitis

Answer 355

Education, allergy avoidance, antihistamines, topical steroids - Avoid open grassy spaces, keep windows closed, no animals in the house - Topical nasal antihistamines AS REQUIRED - Oral antihistamines REGULAR THERAPY (cetirizine) - Topical nasal steroids - in obstruction. Slower onset than antihistamines Other - nasal saline washouts - sodium cromoglicate (less effective) - intranasal ipratropium Surgery - reduction of inferior turbinates or to correct a deviated septum

Question 356

Define wheeze

Answer 356

``` High pitched whistling sounds that occurs when smaller airways are narrowed Occurs in - bronchospasm - swelling of mucosal lining - excessive secretions - foreign body ```

Question 357

Causes of wheeze in the child

Answer 357

``` Asthma Bronchiolitis URTI Transient wheezing in infancy Croup Pneumonia Cardiac failure Recurrent feed aspiration GORD Foreign body inhalation CF Congenital abnormality of lung, airway or heart Idiopathic ```

Question 358

Key features of bronchiolitis

Answer 358

Aged 1-9 months Poor feeding, apnoea, dry cough Laboured breathing Apnoea, cyanosis, resp failure

Question 359

Transient wheezing in infancy

Answer 359

Often after viral infection (RSV) Disappears by 3 years Increased in pre term babies or maternal smokers

Question 360

How long do maternal antibodies circulate in the neonate

Answer 360

First 3 months

Question 361

Causes of prolonged fever

Answer 361

``` Atypical infections Connective tissue disease Kawasaki's disease Systemic JIA (juvenile idiopathic arthritis) SLE Malignancy (lymphoma or leukaemia) ```

Question 362

Kawaski's disease

Answer 362

``` Fever > 5 days Rash Conjunctivitis Cervical lymphadenopathy Peeling of hands and feet Strawberry tongue ``` Untreated causes coronary aneurysms Treat with aspirin and steroids (serial echoes)

Question 363

Systemic juvenile idiopathic arthritis

Answer 363

Extraarticular manifestations apart from arthritis Splenomegaly Fever (high grade) Anaemia Salmon pink rash - seen on trunk or pressure areas Fever and rash can come and go - often at the same time every day or twice a day Anaemia of chronic disease Neutrophilia Thrombocytosis Raised ESR and CRP

Question 364

Investigations for fever in under 1 month old

Answer 364

``` FBC CRP Blood culture Urine culture LP ALWAYS Treat ABx empirically ```

Question 365

Measles

Answer 365

Caused by measles virus ``` Fever > 40 Cough Runny nose Coryza Inflamed eyes Kolpik's spots (white spots in the mouth) / Enanthem Red flat rash starts on face and spreads - Cephalocaudal progression ``` No treatment!

Question 366

Complications of measles

Answer 366

Occur in 30% Diarrhoea Blindness Inflammation of the brain Pneumonia Subactue sclerosing panencephalitis

Question 367

Subacute sclerosing panencephalitis

Answer 367

Inflammation of the entire brain Can occur 7-10 years after measles virus ``` Symptoms initially subtle - Mood changes Then severe - Seizures - Coma - Death ```

Question 368

Mumps

Answer 368

Also known as epidemic parotitis Caused by the mumps virus ``` Fever Muscle pain Headache Fatigue Trismus - spasm of muscles of mastication ``` Followed by painful swelling of one or both parotid salivary glands Can be associated with ear ache No treatment

Question 369

Complications of mumps

Answer 369

``` Meningitis Encephalitis Orchiditis (usually unilateral) Epididymitis (usually unilateral) Rarely causes infertility ``` Glomerulonephritis Arthritis of large joints Myocarditis Pancreatitis No increased risk of congenital malformation in pregnant women

Question 370

Rubella

Answer 370

Also known as German measles or 3 day measles Caused by rubella virus Rash starts 2 weeks after exposure and lasts 3 days - pink or light red Starts on face and spreads Rash is not as bright as measles ``` Lymphadenopathy Flu like symptoms Exanthem (rash on face) Joint pains Headache Conjunctivitis ```

Question 371

Which of the MMR can cause congenital malformations?

Answer 371

Rubella