Paediatrics Flashcards

Question

Signs of pneumonia in children

Answer 1

Derangement in basic obs can indicate sepsis 2nd to pneumonia - Tachypnoea (raised respiratory rate) - Tachycardia (raised heart rate) - Hypoxia (low oxygen) - Hypotension (shock) - Fever - Confusion

Answer 2

- CXR is gold standard (not routinely required but helpful when doubt or severe) To find causative agent: - Sputum culture - Throat swabs for bacterial cultures - Viral PCR If sepsis suspected: - Blood cultures - Capillary blood gas to monitor lactate, and resp or metabolic acidosis

Answer 3

- Abx according to local guidelines - 1st line: usually amoxicillin +/- macrolide e.g. clarithromycin to cover atypical pneumonia or if penicilin allergy - IV abx if sepsis - O2 used to keep sats > 92% (15L 100%)

Answer 4

- An autosomal recessive condition that is caused by a defective protein called CF transmembrane conductance regulator (CFTR), a cyclic AMP-dependent chloride channel in cell membranes. - Gene on chromosome 7. - Most common mutation in the UK (78%) is Δ (delta) F508

Answer 5

- 1 in 2500 live births - 1 in 25 carriers

Answer 6

The mutation means that there is abnormal transport of ions across the cell membranes of epithelial cells. **Key consequences** Thick pancreatic and biliary secretions = blockage of the ducts = lack of digestive enzymes such as pancreatic lipase in GI tract - Thick airway secretions that reduce airway clearance, resulting in bacterial colonisation and susceptibility to airway infections - Congenital bilateral absence of the vas deferens in males. Patient has healthy sperm but cannot travel from testes to ejaculate = infertility

Answer 7

Newborn - Diagnosed through newborn screening (heel-prick) - Meconium ileus (meconium blocks ileum) Infancy/children - Prolonged neonatal jaundice - Failure to thrive - Recurrent chest infections - Malabsorption, steatorrhoea - Abdominal pain + bloating

Answer 8

- Low weight or height on growth charts - Nasal polyps - Finger clubbing - Crackles and wheezes on auscultation - Abdominal distension

Answer 9

Need to know for exams: - Newborn blood spot testing picks up most cases - The sweat test = gold standard for diagnosis Genetic testing for CFTR gene during pregnancy by amniocentesis, or newborn blood test

Answer 10

Remember this test! Gold standard for confirming diagnosis Pilocarpine is applied on patch of skin Electrodes placed either side of the skin, small currents run through and cause skin to sweat Lab-issued gauze/filter paper absorbs sweat and sent to lab for chloride conc testing. Diagnostic chloride concentration > 60mmol/l.

Answer 11

Patients struggle to clear the airway secretions = perfect moist, warm environment for bacteria Key colonisers for exams - Staphylococus aureus - Pseudomonas Treatment = long-term prophylactic flucloxacillin to prevent SA infection Pseudomonas (BAD GUY!) = hard to treat and worsens prognosis, treated with long term nebulised antibiotics

Answer 12

- Specialist MDT - Daily chest physio = clear mucus and reduces infection risk - Exercise improves respiratory function and reserve, and helps clear sputum - A high-calorie diet is required for malabsorption - CREON tablets for pancreatic insufficiency (replaces missing lipase enzymes) - Prophylactic flucloxacillin tablets - Treat chest infections - Bronchodilators such as salbutamol - Nebulised DNase (dornase alfa) = enzyme to make secretions less viscous and easier to clear - Nebulised hypertonic saline Vaccinations for pneumococcal, influenza and varicella

Answer 13

Managed and followed-up in specialist CF clinics, about every 6m Monitoring of sputum and bacterial colonisation e.g. pseudomona Also monitoring and screening for diabetes, osteoporosis, vitamin D deficiency and liver failure.

Answer 14

Prognosis depends on multiple factors, including severity of symptoms, type of genetic mutation, adherence to treatment, frequency of infection and lifestyle. Life expectancy improved to around 47yo - 90% develop pancreatic insufficiency - 50% of adults develop cystic fibrosis-related diabetes and need insulin - 30% develop liver disease

Answer 15

Inflammation and infection in the bronchioles, the small airways of the lungs. RSV - respiratory syncytial virus Common in < 1 year olds Most common < 6m Sometimes up to 2 years old, particularly ex-premature babies with chronic lung diseases

Answer 16

- Coryzal symptoms. = running or snotty nose, sneezing, mucus in throat and watery eyes. - Signs of respiratory distress - Dyspnoea - Tachypnoea - Poor feeding - Mild fever (under 39ºC) - Apnoeas - Wheeze and crackles on auscultation

Answer 17

Remember this very well, foundations of paeds to spot respiratory distress! - Raised respiratory rate - Use of accessory muscles of breathing, such as the sternocleidomastoid, abdominal and intercostal muscles - Intercostal and subcostal recessions - Nasal flaring - Head bobbing - Tracheal tugging - Cyanosis - Abnormal airway noises

Answer 18

- URTI with coryzal symptoms, 50% recover - 50% develop chest symptoms within 1 - 2 days - Symptoms worst on day 3 or 4. - Symptoms usually last 7 to 10 days - Full recovery within 2 - 3 weeks

Answer 19

Most infants can be managed at home with safety netting advice Reasons to admit: < 3 months or pre-existing condition such as prematurity or CF - ≤ 50 – 75% of normal milk intake - Clinical dehydration - Respiratory rate > 70 - O2 sats < 92% - Severe respiratory distress (deep recession, grunting) - Apnoeas - Parents cannot manage or access medical help from home

Answer 20

- Ensuring adequate intake, oral, NG tube or IV fluids - Saline nasal drops and nasal suctioning - Supplementary oxygen if O2 < 90% (92 if <6m or high risk) - Ventilatory support if required - e.g. high-flow humidified O2 ## Footnote Guidelines advise against neubulised hypertonic saline, bronchodilatory, steroids and abx

Answer 21

Capillary blood gas (taken from big toe) Signs of poor ventilation - Rising pCO2 - indicates airway collapse - Falling pH - CO2 build-up and failing to buffer the resulting respiratory acidosis

Answer 22

Monoclonal antibody that targets RSV. Monthly injection for high-risk babies e.g. ex-premature and those with congenital heart disease Passive protection by circulating antibodies, and does not activate the baby's immune system until virus is encountered.

Answer 23

- Wheezing- whistling sound caused by narrowed airways, typically heard during expiration - Grunting is caused by exhaling with the glottis partially closed to increase positive end-expiratory pressure - Stridor is a high pitched inspiratory noise caused by obstruction of the upper airway, for example in croup

Answer 24

Intense swelling of the epiglottis (tissue that covers the trachea when swallowing) and surrounding tissues associated with septicaemia. Life-threatening emergency due to the high risk of respiratory obstruction.

Answer 25

H.influenza type B (Hib), introduction of vaccine in infants reduced incidence by 99%

Answer 26

Very acute onset with: - High fever in a very ill, toxic-looking (septic) child - intense painful throat that prevents the child from speaking or swallowing = drooling saliva - Soft inspiratory stridor and increasing respiratory difficulty over hours - Child sitting upright and immobile with mouth open to optimise airflow

Answer 27

Onset: croup days, epiglottis hours Preceding coryza: croup: yes, epiglottis: no Able to drink: croup: yes, epiglottis: no Drooling saliva: croup: no, epiglottis: yes Appearance: croup: unwell, epiglottis: toxic, very ill Fever: croup: <38.5°C epiglottis: >38.5°C Stridor: croup: harsh, rasping, Epiglottis: soft, whispering Voice, cry: croup: hoarse, epiglottis: muffled, reluctant to speak

Answer 28

If acute epiglottis suspected, then urgent hospital admission and treatment need to be commenced. Do not do investigations, and do not lie child down or exam throat with spatula as it can contribute to resp obstruction and death. Lateral neck x-ray = characteristic “thumb sign” or “thumbprint sign”. s a soft tissue shadow that looks like a thumb pressed into the trachea. Caused by oedematous and swollen epiglottis.

Answer 29

- Remain calm and organised to reduce anxiety - Call most senior paediatrician and anaesthetist available - Management focuses on ensuring that the airway is secure, intubation or tracheostomy - not required but should be prepared to do so if upper airways close - When intubated, transfer to ICU Additional treatment once the airway is secure: - IV antibiotics (e.g. cefotaxime or ceftriaxone) - Steroids (i.e. dexamethasone)

Answer 30

With right treatment, recovery in 2-3 days Complication: epiglottic abscess = a collection of pus around the epiglottis. Life-threatening and treatment is similar to acute epiglottis

Answer 31

Croup is an acute infective respiratory disease, typically in children aged 6m to 2y but can be older. It is a URTI that causes oedema in the larynx. Classic cause for exams = parainfluenza virus Usually improves < 48 hours and responds well to steroids particularly dexamethasone

Answer 32

- Increased work of breathing - “Barking” (like a sea lion) cough,in clusters of coughing episodes - Hoarse voice - Stridor - Low-grade fever - Start and worse at night

Answer 33

Most cases = supportive treatment at home (fluids and rest). Admission if - Stridor and/or sternal recession at rest - High fever - Respiratory rate > 60 - Cyanosis - Lethargy or agitation - Fluid intake < 75% of normal or no wet nappies for 12 hours - Aged under 3 months - Chronic conditions 1st line - oral dexamethasone very effective, single dose of 150 mcg/kg, and repeated after 12hrs if needed. Predinisolone as alternative Neubilised adrenaline for temporary symptom relief

Answer 34

Congenital, or acquired Congenital - Maternal rubella or cytomegalovirus infection during pregnancy - Genetic deafness can be autosomal recessive or autosomal dominant Perinatal - Prematurity - Hypoxia during or after birth After birth - Jaundice - Meningitis and encephalitis - Otitis media or glue ear - Chemotherapy

Answer 35

The UK newborn hearing screening programme (NHSP) - ideally at 4 - 5 weeks, but up to 3 months. Special equipment delivers sound to each eardrum and identifies response - detects congenital hearing problems

Answer 36

Parental concern over hearing and behavioural changes associated with not being able to hear - Ignoring calls or sounds - Frustration or poor behaviour - Poor speech and language development - Poor school performance

Answer 37

- < 3 years old = basic response to sound (e.g. turning head towards sound) - Audiometry for older children - test with headphones and specific tones and volumes - Results are recorded on an audiogram - identify and differentiate between sensorineural or conductive hearing loss

Answer 38

- Frequency (Hz) on x-axis - Volume (dB) on y-axis - ascending loud to quiet - Hearing tested to establish minimum volume (dB) required for patient to hear different frequencies (Hz) - Air and bone conduction tested separately in each ear (normal = 0 - 20dB) Sensorineural hearing loss - both air and bone conduction > 20dB Conductive hearing loss = bone conduction reading normal but air conduction readings > 20dB Mixed = both air and bone conduction > 20dB, but bone conduction > air conduction by 15dB or more

Answer 39

MDT approach - Speech and language therapy - Educational psychology - ENT specialist - Hearing aids for children who retain some hearing - Sign language

Answer 40

Infection in the middle ear - space between tympanic membrane and inner ear - contains cochlea, vestibular apparatus and nerves

Answer 41

Preceded by viral URTI, bacteria enter the ear from back of throat through the eustachian tube Most common bacteria = streptococcus pneumoniae Others include Haemophilus influenzae and staphylococcus aureus

Answer 42

- Ear pain - Reduced hearing - URTI symptoms e.g. fever, cough, coryzal symptoms, sore throat, generally unwell - Vertigo and balance issues if vestibular system is affected - Discharge if tympanic membrane perforated Symptoms can be non-specific, especially in young children and infants - fever, vomiting, irritability, lethargy or poor feeding - worth examining ears!

Answer 43

Examination of both ears and throat of unwell children as they are common sites of infection that produce non-specific symptoms. Use a otoscope Normal tympanic membrane = pearly grey, translucent and slightly shiny + malleus visible through membrane Otitis media = bulging, red, inflamed membrane

Answer 44

If symptoms severe or diagnostic doubt - consider referral to paeds Always specialist referral and consider admission in < 3m olds with temp > 38 or 3-6m with temp > 39 Most cases resolve without abx within 3 days and NICE 2018 recommends not prescribing abx for OM

Answer 45

Three options: - Immediate abx - patients with significant comorbidities, systemically unwell or immunocompromised - Delayed abx - prescription of abx for use and collection after 3 days if no improvement or worsened - Perforated eardrum 1st line = amoxicillin for 5 days Safety net, eduction and advice to patients and parents on when to seek further medical attention

Answer 46

- Otitis media with effusion - Hearing loss (usually temporary) - Perforated eardrum - Recurrent infection - Mastoiditis (rare) - Abscess (rare)

Answer 47

Glue ear is otitis media with effusion. The middle ear becomes full of fluid, causing a loss of hearing in that ear. The Eustachian tube connects the middle ear to the back of the throat, helping drain secretions. If blocked then secretions will build up in middle ear.

Answer 48

Checking what blood type the patient has and checking the transfusion blood is a match, not prepared blood Cross match - same as above but requesting a specific number of blood from the lab to be available immediately

Answer 49

Reduction of hearing in affected ear

Answer 50

Otoscopy - a dull tympanic membrane with air bubbles or a visible fluid level, although it can look normal.

Answer 51

Referral for audiometry to establish diagnosis and extent of hearing loss. Conservative treatment and usually resolves in 3 month If co-morbidities affecting ear structure e.g. Down's syndrome or cleft palate might need hearing aids or grommets

Answer 52

Tiny tubes inserted into the tympanic membrane by the ENT surgeon This allows fluid from the middle ear to drain through the tympanic membrane to the ear canal. Inserted under GA as a day case Grommets usually fall out within a year, 1 in 3 will require further grommet insertion

Answer 53

Squint refers to misalignment of the eyes, aka strabismus. This results in the images on the retina not matching and the person will experience double vision.

Answer 54

- Strabismus: the eyes are misaligned - Amblyopia: the affected eye becomes passive and has reduced function compared to the other dominant eye, as the brain has learnt to ignore signals from the affected eye - Esotropia: inward-positioned squint (affected eye towards the nose) - Exotropia: outward positioned squint (affected eye towards the ear) - Hypertropia: upward moving affected eye - Hypotropia: downward moving affected eye

Answer 55

Usually idiopathic in healthy children - Hydrocephalus - Cerebral palsy - Space-occupying lesions e.g. retinoblastoma - Trauma

Answer 56

- General inspection - Eye movements - Fundoscopy (or red reflex) to rule out retinoblastoma, cataracts etc. - Visual acuity **Hirschberg’s test** - Pen-torch shone in patient's eye from 1m away, observe reflection on their cornea, normally central and symmetrical. - Deviation indicates a squint **Cover test** Cover one eye and ask patient to focus on object in front of them, move cover across to opposite eye. If previously covered eye moves inwards = drifted outwards when covered = exotropia if it moves outwards = drifted inwards when covered (esotropia).

Answer 57

- Treatment needs to start before 8yo as visual fields develop until then., otherwise squint becomes permanent - Managed by an ophthalmologist - Treat underlying pathology - Occlusive patch - covers good eye to allow weaker eye to develop - Atropine drops - blurs vision in good eye

Answer 58

Infection of tissues in the eyelid or skin around the eyes - unilateral. Main clinical features: fever, erythema, tenderness and oedema around the affected eyelid.

Answer 59

Prompt IV antibiotics such as high-dose ceftriaxone to prevent spread of infection causing orbital cellulitis.

Answer 60

Usually, the pressure in LV > RV, so blood flows from LV to RV via the VSD = acyanotic because blood is oxygenated. However, left to right shunt leads to right-sided overload, RHF and pulmonary HTN Over time, this increases RV pressure and causes right to left shunt, bypassing lungs = deoxygenated blood in systemic circulation = patient cyanotic, known as Eisenmenger syndrome

Answer 61

A congenital defect (hole) in the septum between the two ventricles. Varies from small (≤3mm) to entire septum. Usually associated with genetic conditions such as Down’s Syndrome and Turner’s Syndrome.

Answer 62

- Picked up on antenatal scans - Newborn baby check - murmur However, often symptomless and can present in adulthood. If symptoms, typically: - Poor feeding - Dyspnoea - Tachypnoea - Failure to thrive Examinations: - Pan-systolic murmur - prominently at left lower sternal border in 3rd + 4th intercostal space

Answer 63

- Ventricular septal defect - Mitral regurgitation - Tricuspid regurgitation.

Answer 64

Paediatric cardiologist Small VSD with no symptoms = monitoring as they can close spontaneously Surgery: Transvenous catheter closure via the femoral vein or open heart surgery. Increased risk of infective endocarditis, so prophylactic abx should be considered during surgery

Answer 65

- Secundum ASD (80%) - deficiency of the foramen ovale and surrounding atrial septum - Partial atrioventricular septal defect (AVSD or primum ASD) - deficiency of atrioventricular septum

Answer 66

Acyanotic heart disease as left to right shunt Symptoms - None (commonly) - Poor feeding, failure to thrive - Recurrent chest infections/wheeze - Arrhythmias (40+) Signs - Hepatomegaly - Oedema - Ejection systolic murmur particularly at the upper left sternal edge, due to increased flow across the pulmonary valve due to L to R shunt - Partial ASD - apical pan systolic murmur from AV valve regurgitation.

Answer 67

- CXR - cardiomegaly, enlarged pulmonary arteries and increased pulmonary vascular markings - Echocardiogram to determine anatomy - gold standard

Answer 68

- Treatment is needed if ASD is large enough to cause right-heart dilatation. - Secundum ASD - cardiac catheterisation to insert occlusion device to close the hole - Partial AVSD = surgical correction - 3-5 years old to prevent right heart failure and arrhythmias in later life.

Answer 69

A congenital condition where there is narrowing of the aortic arch, usually around the ductus arteriosus Mild to severe Usually associated with TUrner's syndrome This narrowing reduces blood pressure flowing to distal arteries and increases BP in areas proximal to the narrowing, such as the heart and the first three branches of the aorta.

Answer 70

- Very sick baby - grey and floppy - Poor feeding Signs - Normal in 1 day after birth, then present with acute resp collapse once duct closes (if severe) - Neonates: weak/absent femoral pulses - Severe heat failure - Four limb BP will reveal HTN in limbs supplied by arteries before the narrowing, low BP in limbs supplied by arteries after the narrowing

Answer 71

CXR - cardiomegaly from heart failure and shock if severe ECG - normal

Answer 72

Mild - watch and wait as often symptom-free until adulthood Severe - emergency surgery shortly after birth - Prostaglandin E (Alprostadil) given to keep ductus arteriosis patent while waiting for surgery to allow for blood flow to distal circulation Surgery is then performed to correct the coarctation and to ligate the ductus arteriosus.

Answer 73

A blood vessel present in the fetus in the womb that connects the pulmonary artery to the aorta - shunting oxygenated blood straight into the aorta, bypassing the fetal lungs.

Answer 74

The ductus arteriosus normally stops functioning shortly after birth and closes within 2-3 weeks. PDA is the failure of it to close.

Answer 75

- Prematurity - Maternal rubella infection - Female sex - <3m

Answer 76

Aorta pressue > pulmonary vessels pressure Bloods from aorta flows into pulmonary artery = left to right shunt, leading to pulmonary hypertension and RH strain as the RV struggles to contract against increased resistance. This leads to right ventricular hypertrophy, which increases blood flow to the pulmonary vessels and returning the LH leading to LVH.

Answer 77

- Shortness of breath/ tachypnoea - Difficulty feeding - Poor weight gain - Lower respiratory tract infections Sometimes patients with PDA can be asymptomatic in childhood and present with heart failure as an adult Signs: Continuous crescendo-decrescendo "machinery" murmur that can continue during the second heart sound, making the S2 hard to hear

Answer 78

Echocardiogram = investigation of choice Doppler flow studies during the echo can determine the size of the LtoR shunt and check for RVH + LVH.

Answer 79

- Symptomatic preterm infants: Ibruprofen as NSAIDs effective in promoting ductal closure. - Term infants and children with small/moderate PDA: trans-catheter closure to prevent heart failure - Large ducts - surgical ligation

Answer 80

- Coarctation of the aorta - ASD - Pulmonary hypertension

Answer 81

Congenital aortic valve stenosis are born with a narrow aortic valve that restricts blood flow from the LV into the aorta The aortic valve is usually made up of 3 leaflets, which prevents backflow of blood from the aorta into the LV. Patients with aortic stenosis may have one, two, three or four leaflets.

Answer 82

- Most common is an asymptomatic murmur - Severe stenosis = reduced exercise tolerance, chest pain on extortion or syncope - Neonates with critical aortic stenosis and duct-dependent circulation may present with severe heart failure leading to shock Signs - Ejection systolic murmur loudest at aortic area (2nd ICS, right sternal border) - crescendo-decrescendo - Carotid thrill (always)

Answer 83

Echocardiogram - normal or prominent LV with post-stenotic dilatation of ascending aorta

Answer 84

Regular monitoring with echocardiograms to know when to intervene, and treatment options: Symptomatic on exercise: balloon valvuloplasty, using a balloon to open the narrowed valves. Significant aortic valve stenosis - aortic valve eventually. Early palliative treatment to delay this

Answer 85

- Left ventricular outflow tract obstruction - Heart failure - Ventricular arrhythmia - Infective endocarditis - Sudden death, often on exertion

Answer 86

The pulmonary valve usually has leaflets. If they develop abnormally, they can become thickened or fused, which causes a narrowing between the pulmonary artery and RV - this is known as congenital pulmonary valve stenosis.

Answer 87

- Tetralogy of Fallot - William syndrome - Noonan syndrome - Congenital rubella syndrome

Answer 88

- Often asymptomatic murmur - More significant stenosis can present with fatigue on exertion, shortness of breath, dizziness and fainting. Signs - Ejection systolic murmur heard loudest at the pulmonary area (2nd ICS, left sternal border) - Palpable thrill in the pulmonary area - RV heave due to RVH - Raised JVP with giant A waves

Answer 89

- Echocardiogram = gold standard - Mild = watch and wait and followed up by cardiologist Symptomatic or moderate - balloon valvuloplasty with venous catheter. Catheter inserted under x-ray guidance via femoral vein to IVC to RH and valve is dilated by inflating a balloon Open-heart surgery if above is ineffective

Answer 90

A congenital heart condition that combines 4 pathologies: - Ventricular septal defect (VSD) - Overriding aorta - Pulmonary valve stenosis - Right ventricular hypertrophy

Answer 91

1. Pulmonary valve stenosis - restrictss the flow of deoxygenated blood from RV to the pulmonary artery 2. Right ventricular hypertrophy - RV compensates for increased resistance by undergoing hypertrophy (boot-shaped heart) 3. Ventricular septal defect (VSD) - gap between the RV and LV, which allows shunting of blood between them. Due to RV hypertrophy, pressure in the RV > LV and deoxygenated blood is shunted from RV to LV 4. Overriding aorta - aorta entrance sits above the septal defect, meaning that a greater proportion of deoxygenated blood enters the aorta and systemic circulation from RV

Answer 92

- Rubella infection - Maternal age > 40 - Alcohol consumption in pregnancy - Diabetic mother

Answer 93

- Most picked up on antenatal scans - Cyanosis - Clubbing - Poor feeding - Poor weight gain Signs - Ejection systolic murmur heard loudest in the pulmonary area (second intercostal space, left sternal border) - “Tet spells”

Answer 94

This is when the right to left shunt in TOF temporarily worsens, e.g. during exertion. This will precipitate a cyanotic episode.

Answer 95

Older children may squat, younger children = position their knees to their chest - this increases systemic vascular resistance = more blood into the pulmonary vessels Medical management by experienced paediatrician if lasts > 15mins - O2 in hypoxic children - Beta-blockers to relax RV - IV fluids to increase pre-load - Morphine to reduce respiratory drive and improve breathing - Sodium bicarbonate for metabolic acidosis - Phenylephrine infusion to increase systemic vascular resistance

Answer 96

CXR - boot-shaped heart, normal shape does not rule out TOF Echocardiogram is gold standard and will show the four cardinal features ECG - normal at birth then RVH when older

Answer 97

- In neonates, a prostaglandin infusion to maintain the ductus arteriosus, allows blood flow from aorta back to the pulmonary arteries - Definitive treatment at 6 months with total surgical repair through open heart surgery is the definitive treatment. 5% mortality. 90% will live into adulthood with corrective surgery

Answer 98

This is a condition where the attachments of the aorta and the pulmonary artery to the heart are swapped (“transposed”). The aorta is connected to the right ventricle and the pulmonary artery is connected to the left ventricle. The deoxygenated oxygen is returned to the body and the oxygenated blood is returned to the lungs. Parallel circulation with no mixing of blood and is incompatible with life, unless there is mixing through VSD, ASD or PDA which allow a shunt between the systemic circulation and pulmonary circulation.

Answer 99

- Usually picked up on antenatal scans - Cyanosis will always be present - A shunt created by VSD, ASD or PDA usually compensates by allowing the mixing of pulmonary and systemic circulation - However, the child will present with poor feeding, poor weight gain respiratory distress, and tachycardia within a few weeks of birth

Answer 100

Gold standard is echocardiogram and will show abnormal arterial connections and associated abnormalities.

Answer 101

VSD will buy some time before the definitive treatment In PDA, a prostaglandin infusion will ensure patency - Balloon septostomy will create a large ASD to allow blood returning from lungs to left side to flow into right side and then systemic circulation - Definitive management is open heart surgery. A cardiopulmonary bypass machine used to do an “arterial switch” soon after birth. VSD or ASD also corrected if present

Answer 102

A complex clinical syndrome in which the heart, due to structural or functional defects, cannot pump enough blood to meet the metabolic needs of the child's body.

Answer 103

Symptoms - Breathlessness (particularly on feeding or exertion) - Sweating - Poor feeding - Recurrent chest infections Signs - Poor weight gain or faltering growth - Tachypnoea - Tachycardia - Heart murmur, gallop rhythm - Enlarged heart - Hepatomegaly - Cool peripheries

Answer 104

Neonates - usually due to left-heart obstruction e.g. critical aortic valve stenosis, severe coarctation of the aorta Infants - VSD, ASD, persistent PDA Older children - Eisenmenger syndrome, rheumatic heart disease

Answer 105

Blood tests: FBC, U+Es, LFTs, CRP, TFTs, bone profile, Magnesium, B-type natriuretic peptide (BNP) Imaginig: CXR, echocardiogram, ECG

Answer 106

Depends on underlying cause - Conservative: Fluid restriction and dietitian-guided feeding plans - Medical: diuretics with inotropes if required Surgical: correction of the anatomical defect if present; heart transplant in end-stage cases

Answer 107

A short-lived, multisystem autoimmune response to a preceding infection with group A β-haemolytic streptococcus. Mainly affects children aged 5 - 15 Progresses to rheumatic heart disease in up to 80% of cases

Answer 108

If rheumatic fever remains untreated, it can progress into chronic rheumatic heart disease The most common is mitral stenosis from long-term scarring and fibrosis. However, aortic, tricuspid and rarely pulmonary valves can be affected.

Answer 109

- Pharyngeal or skin infection - Latent interval of 2-6 weeks - Then polyarthritis, mild fever and malaise Rare in the developed world because of improvements in living conditions, antibiotics for streptococcal pharyngitis

Answer 110

Jonas criteria - two major or one major and one minor and evidence of preceding group A streptococcal infection 5 major manifestations - Carditis (50%) - Migratory arthritis (80%) - Sydenham chorea (10%) - Erythema marginatum (5%) - rash on trunk and limbs 4 minor manifestations - Fever (≥38.5°C) - Polyarthralgia - Elevated ESR or CRP - Prolonged PR interval on electrocardiogram.

Answer 111

- Bed rest - High-dose aspirin to reduce the inflammatory response of the heart and joints - Heart failure: diuretics and ACE inhibitor - Anti-streptococcal abx if persistent infection

Answer 112

Prophylaxis: benzathine penicillin, or oral penicillin - Oral erythromycin if penicillin allergy - 10 years after last acute episode or until 21, or lifelong if severe valvular disease - Surgical valve repair or replacement

Answer 113

Infection of the endothelium (inner surface) of the heart. Most commonly affects the heart valves.

Answer 114

All children with congenital heart disease (except secundum ASD). Highest risks: - VSD, coarctation of aorta and PDA due to the turbulent jet of blood. - Prosthetic material/valves

Answer 115

Most common: Staphylococcus aureus.

Answer 116

Suspect IE in any child or adult with sustained fever, malaise, raised ESR, unexplained anaemia or haematuria (microscopic) Other features - Changing or new heart murmur - Splinter haemorrhages - Clubbing (late) - Necrotic skin lesions on palms and soles (Janeway lesions) - Osler's nodes - Splenomegaly - Neurological signs from cerebral infarction - Retinal infarcts (Roth spots) - Arthritis/arthralgia DO NOT rely on peripheral stigmata

Answer 117

Blood cultures before antibiotics. 30 min intervals, 3 different sites. If sepsis, do not delay empirical antibiotics Imaging Echocardiography- transoesophageal echocardiography (TOE), vegetations (an abnormal mass containing fibrin, platelets and the infective organism) on valves

Answer 118

Modified dukes criteria One major & three minor criteria OR five minor criteria Major criteria are: - Persistently positive blood cultures (typical bacteria) - Specific imaging findings (e.g. vegetation on echocardiogram) Minor criteria are: - Predisposition (e.g., IV drug use or heart valve pathology) - Fever > 38°C - Vascular phenomena (e.g., splenic infarction, intracranial haemorrhage and Janeway lesions) - Immunological phenomena (e.g., Osler’s nodes, Roth spots and glomerulonephritis) - Microbiological phenomena (e.g., positive cultures not qualifying as a major criterion)

Answer 119

- 6 weeks of IV high-dose penicillin + aminoglycoside (e.g. gentamicin) - Surgical removal might be needed if VSD or prosthetic valves

Answer 120

Good dental hygiene, strongly encouraged for children with congenital heart disease Avoid tattoos and piercings Antibiotics prophylaxis no longer recommended in the UK, but dental procedures and surgery can be associated with bacteremia

Answer 121

Innocent murmurs or flow murmurs are common in children, they occur because of fast blood flow through the heart during systole 6S to remember the features of innocent murmurs - Soft - Short - Systolic - S1 and S2 normal - Symptomless - Situation dependent - quieter on standing or only present when child is ill or feverish

Answer 122

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

Answer 123

ECG Chest Xray Echocardiography

Answer 124

- Mitral regurgitation heard at the mitral area (fifth intercostal space, mid-clavicular line) - Tricuspid regurgitation heard at the tricuspid area (fifth intercostal space, left sternal border) - Ventricular septal defect heard at the left lower sternal border

Answer 125

- Aortic stenosis heard at the aortic area (second intercostal space, right sternal border) - Pulmonary stenosis heard at the pulmonary area (second intercostal space, left sternal border)

Answer 126

Supraventricular tachycardia (SVT) HR is about 200 - 300 beats/min

Answer 127

Heart failure in neonates or young infants Its known as re-entry tachycardia because there is premature activation of the atrium via an accessory pathway Cause of fetal hydrops (severe oedema) and intrauterine death

Answer 128

ECG - narrow complex tachycardia (250 to 300 beats/min)

Answer 129

- Restoration of sinus rhythm is key - IV adenosine – the treatment of choice. It works by breaking the re-entry circuit between the AV node and the accessory pathway - Electrical cardioversion if adenosine fails - Maintenance therapy e.g. flecainide, in most cases, attacks stop after 1yo - Wolff-white syndrome - atrial pacing (pacemaker) or percutaneous radiofrequency ablation or cryoablation of the accessory pathway.

Answer 130

A condition where there is hypertrophy and narrowing of the pyloric sphincter - smooth muscle between the stomach and duodenum. This leads to gastric outlet obstruction.

Answer 131

As it is increasingly difficult for food to pass from the stomach to the duodenum, the stomach compensates by increasing the peristalsis force to push food through. Eventually becomes strong enough to eject food into the oesophagus, causing projectile vomiting - a classic symptom of pyloric stenosis to look out for in exams

Answer 132

- 2 - 8 weeks of age - Most common in boys and those with FHx

Answer 133

- Typically a thin, pale, hungry baby with failure to thrive and weight loss - Projectile vomiting Signs on examination - Visible peristalsis - Firm, round mass present in upper abdomen "feels like a large olive" due to hypertrophic muscle of the pylorus.

Answer 134

- Test feed to visualise peristalsis - Blood gas - Abdominal ultrasound to visualise the thickened pylorus

Answer 135

- Hypochloraemic (low chloride) metabolic alkalosis as a result of vomiting stomach contents

Answer 136

IV fluids to correct fluid and electrolyte balance Definitive treatment: laparoscopic pyloromyotomy - incision made in pylorus smooth muscle to widen canal, allowing food to pass from stomach to duodenum. Prognosis = excellent after opreation.

Answer 137

This is where contents from the stomach reflux through the lower oesophageal sphincter into the oesophagus, throat and mouth. In babies, the lower oesophageal sphincter is still developing so stomach contents can easily reflux Normal for babies to reflux and as long as there is normal growth, it is not a problem. 90% infants stop having reflux by 1 year

Answer 138

If reflux is accompanied by the following features: - Chronic cough - Hoarse cry - Distress after feeding - Reluctance to feed - Pneumonia - Poor weight gain Children > 1 year = similar to adults with heartburn, acid regurgitation, retrosternal or epigastric pain, bloating and nocturnal cough.

Answer 139

- Not keeping down any feed (pyloric stenosis or intestinal obstruction) - Projectile or forceful vomiting (pyloric stenosis or intestinal obstruction) - Reduced consciousness, bulging fontanelle or neurological signs (meningitis or raised intracranial pressure) - Blood in the stools (gastroenteritis or cows milk protein allergy) - Rash, angioedema and other signs of allergy (cow milk protein allergy)

Answer 140

Simple, advice and reassurance: - Small, frequent meals - Burping regularly - Not over-feeding Keep the baby upright after feeding If more problematic: - Gaviscon mixed with feeds - Thickened milk or formula (specific anti-reflux formulas) - Proton pump inhibitors (e.g., omeprazole)

Answer 141

- The invagination of a proximal segment of the bowel into a distal one - Often the ileum folding into the caecum

Answer 142

- Severe, colicky abdominal pain - Pale, lethargic and unwell child - “Redcurrant jelly stool” - A "sausage-shaped" mass palpated inRUQ - Vomiting - Intestinal obstruction presenting as absolute constipation and abdominal distention TOM TIP: Look out for the “redcurrant jelly stool” in your exams The other classic feature is the sausage-shaped mass in the abdomen. The typical child in the exam will have had a viral upper respiratory tract infection preceding the illness and will have features of intestinal obstruction (vomiting, absolute constipation and abdominal distention). Ultrasound is the initial investigation of choice.

Answer 143

Abdominal ultrasound is the gold standard Classic "target" sign

Answer 144

A congenital condition where nerve cells of the myenteric plexus are absent in the distal bowel and rectum. The myenteric plexus, or Auerbach’s plexus, forms the enteric nervous system. It is the brain of the gut.

Answer 145

- Therapeutic enemas - contrast, water or air pumped into colon to force the folded bowel back into normal position - Surgical reduction if above ineffective - Gangrenous or perforated bowel = surgical resection

Answer 146

- Overfeeding - Gastro-oesophageal reflux - Pyloric stenosis (projective vomiting) - Gastritis or gastroenteritis - Appendicitis

Answer 147

- Delay in passing meconium (more than 24 hours) - Chronic constipation since birth - Abdominal pain and distention - Vomiting - Poor weight gain and failure to thrive

Answer 148

During normal fetal development, the parasympathetic ganglion cells migrate down to the distal colon and rectum from the higher GI tract. In Hirschprung's disease, this process does not occur, leading to an absence of parasympathetic ganglion cells in the distal colon and rectum.

Answer 149

Inflammation and obstruction of the intestine occurring in around 20% of neonates with Hirschsprung’s disease. Presents within 2 -4 weeks after birth Fever, abdominal distention, diarrhoea (often with blood) and features of sepsis. Life-threatening, it can lead to toxic megacolon and perforation of the bowel. Mx = urgent IV abx, fluid resuscitation and decompression of the obstructed bowel.

Answer 150

Abdominal x-rays can help diagnose intestinal obstruction and demonstrate features of HAEC. Rectal biopsy to confirm the diagnosis. Histology demonstrates the absence of ganglionic cells. Systemically unwell and enterocolitis = fluid resuscitation and mx of intestinal obstruction. IV antibiotics are required in HAEC. Definitive mx = surgical removal of the aganglionic section of bowel.

Answer 151

Wide range of causes, but below are common: Organic - Appendicitis - most common - Constipation - Urinary tract infection - Coeliac disease - IBD Functional - IBS - Abdominal migraine Additional causes in adolescent girls: - Dysmenorrhea (period pain) - Ectopic pregnancy - Pelvic inflammatory disease - Ovarian torsion - Pregnancy

Answer 152

- Persistent or bilious vomiting - Severe chronic diarrhoea - Fever - Rectal bleeding - Weight loss or faltering growth - Dysphagia (difficulty swallowing) - Nighttime pain - Abdominal tenderness

Answer 153

- Anaemia - IBD or coeliac's disease - Raised anti-TTG or anti-EMA - coeliac's - Raised CRP - IBD - Raised faecal calprotectin - IBD - Positive urine dipstick - UTI

Answer 154

Stressful life events such as bullying

Answer 155

Reassurance and explanation Measures to help with the pain: - Distraction with other activities or interests - Advice about sleep, regular meals, balanced diet, staying hydrated, exercise and reducing stress - Probiotic supplements for IBS - Avoid NSAIDs such as ibuprofen Address psychosocial triggers - Support from a school counsellor or child psychologist

Answer 156

The infrequent passage of dry, hardened faeces often accompanied by straining or pain and bleeding associated with hard stools. Very common problem in children, usually due to lifestyle factors and not more serious pathology.

Answer 157

The frequency of bowel opening varies hugely, breast-fed babies might only have one stool a week. Important to establish what is normal for the child and how it has changed. - Less than 3 stools a week - Hard, rabbit-dropping stools that are difficult to pass - Straining and pain - Abdominal pain - Rectal bleeding associated with hard stools - Faecal impaction causing overflow soiling - Loss of the sensation of the need to open the bowels - Papable stools in the abdomen

Answer 158

- Hirschsprung's disease - Cystic fibrosis (particularly if meconium ileus) - Hypothyroidism

Answer 159

- Not passing meconium within 48 hours of birth (cystic fibrosis or Hirschsprung’s disease) - Vomiting (intestinal obstruction or Hirschsprung’s disease) - Abnormal anal anatomy/patency (inflammatory bowel disease or sexual abuse) - Failure to thrive (coeliac disease, hypothyroidism or safeguarding) - Acute severe abdominal pain and bloating (obstruction or intussusception)

Answer 160

- Habitually not opening the bowels, can cause faecal impaction - Low fibre diet - Poor fluid intake and dehydration - Sedentary lifestyle Psychosocial problems such as a difficult home or school environment (think safeguarding)

Answer 161

Explain diagnosis and provide reassurance - treatment can take months - HIgh fibre, hydration - Laxatives -macrogol is 1st line - Faecal impaction - disimpaction with high dose laxatives and continue long-term until child develops normal, regular bowel habit

Answer 162

Appendicitis is inflammation of the appendix. Caused by trapped infection and can quickly proceed to gangrene and rupture. This releases faecal and infective matter into the abdomen, causing peritonitis Peak incidence between 10 to 20, uncommon <3 years old

Answer 163

Symptoms - Key presenting feature = abdominal pain - It starts central then localises to right iliac fossa - Anorexia - Nausea and vomiting Signs - Abdominal tenderness at McBurney's point (1/3 distance from ASIS to umbilicus) - Rovsing’s sign (palpation of the left iliac fossa causes pain in the RIF) - Guarding - Rebound tenderness suggests peritonitis

Answer 164

- Clinical diagnosis supported by raised inflammatory markers - CT abdomen to confirm the diagnosis - In females, USS to rule out ovarian and gynae pathology - If symptoms point to appendicitis but investigations are negative then diagnostic laparoscopy to visualise the appendix. - Then appendicectomy if indicated.

Answer 165

- Ectopic pregnancy - consider this in females of childbearing age and do a pregnancy test - Ovarian cysts - pelvic and iliac fossa pain, particularly with rupture or torsion.

Answer 166

Appendicectomy - laparoscopic preferred to laparotomy (open)

Answer 167

- Bleeding, infection, pain and scars - Damage to bowel or bladder - Venous thromboembolism (DVT or PE)

Answer 168

IBD is an umbrella term for ulcerative colitis and Crohn's disease, they both cause inflammation of the GI tract with periods of exacerbation and remission.

Answer 169

Crow's nest - N – No blood or mucus (less common in Crohns.) - E – Entire GI tract (mouth to anus) - S – “Skip lesions” on endoscopy - T – Terminal ileum most affected and Transmural (full thickness) inflammation - S – Smoking is a risk factor (don’t set the nest on fire)

Answer 170

U = CLOSE UP - C – Continuous inflammation - L – Limited to colon and rectum - O – Only superficial mucosa affected - S – Smoking is protective - E – Excrete blood and mucus - U – Use aminosalicylates - P – Primary sclerosing cholangitis

Answer 171

- Abdominal pain - Diarrhoea - Weight loss However, children can present with no GI symptoms and lethargy, general ill health, weight loss and failure to thrive

Answer 172

- Rectal bleeding, diarrhoea and colicky pain. Weight loss and failure to thrive sometimes, but less common than in Crohn's

Answer 173

Common exam question - Finger clubbing - Erythema nodosum (red lesion on shins) - Pyoderma gangrenosum (skin ulcers) - Episcleritis and iritis - Inflammatory arthritis - Primary sclerosing cholangitis (ulcerative colitis)

Answer 174

Bloods - FBC (anaemia), thyroid, kidney and liver function. Raised CRP = raised inflammation Faecal calprotectin screening - released by the intestines when inflamed. 90% sensitive and specific for IBD in adults. Endoscopy (OGD and colonoscopy) + biopsy is gold standard

Answer 175

Blood tests for anaemia, infection, thyroid, kidney and liver function. Raised CRP = active inflammation Faecal calprotectin - released by the intestines when inflamed. Endoscopy (OGD and colonoscopy) with biopsy is gold standard

Answer 176

NICE guidelines 2019, talk to seniors before treating Medical: Inducing Remission: - 1st line: steroids (e.g. oral prednisolone or IV hydrocortisone). Maintaining Remission: - Tailored to patient, patients can choose not to take meds when well - 1st line: azathioprine, mercaptopurine Surgery - Resection of distal ileum if only this area is affected - Treatment of secondary strictures and fistulas

Answer 177

**Inducing Remission** Mild to moderate disease 1st line: aminosalicylate (e.g. mesalazine oral or rectal) Severe disease 1st line: IV corticosteroids (e.g. hydrocortisone) **Maintaining Remission** Aminosalicylate (e.g. mesalazine oral or rectal) or immunopressants **Surgery** - Removal of colon and rectum (panprotocolectomy) - Permanent ileostomy (stoma) or J-pouch, the small intestine is connected to the anal canal after removal of the colon and rectum

Answer 178

Autoimmune disease where autoantibodies are created when exposed to gluten. Remember anti-tissue transglutaminase (anti-TTG) and anti-endomysial (anti-EMA) - correlates to disease activity The antibodies target epithelial cells in the intestine, causing inflammation that particularly affects the jejunum Cause atrophy of intestinal villi and malabsorption

Answer 179

Genetic associations - HLA-DQ2 gene (90%) - Type 1 diabetes

Answer 180

Often asymptomatic, so low threshold for testing - Failure to thrive in young children - Abdominal pain - Diarrhoea - Steatorrhoea (fatty, pale, hard to flush stools) - Fatigue - Weight loss - Mouth ulcers - Anaemia secondary to iron, B12 or folate deficiency - Dermatitis herpetiformis abdominal itchy, blistering rash) Rarer neurological symptoms - Peripheral neuropathy - Cerebellar ataxia - Epilepsy Test all patients with a new diagnosis of T1DM for coeliac

Answer 181

- Patients remain on gluten-containing diet - Check total immunoglobulin A levels to exclude IgA deficiency as coeliac disease-specific antibodies are IgA (false negative) - 1st line: raised anti-TTG antibodies - 2nd line: raised anti-endomysial antibodies Endoscopy and intestinal biopsy - crypt hypertrophy & villous atrophy

Answer 182

All products containing wheat, rye and barley are removed from the diet and this leads to the resolution of the symptoms. Supervised by dietician. Monitor coeliac antibodies

Answer 183

- Vitamin deficiency - Anaemia - Osteoporosis - Ulcerative jejunitis - Non-Hodgkin lymphoma (NHL)

Answer 184

Pathological immune response mounted against a specific food protein. IgE mediated or non-IgE mediated. Most common causes: - Infants - cow's milk, egg and peanuts - Older children – peanut, tree nut, fish and shellfish

Answer 185

A non-immunological hypersensitivity reaction to a specific food. Usually occurs few hours after ingestion and involves the GI tract.

Answer 186

- Formula-fed baby - Family history of atopy

Answer 187

- Usually occurs after 10 - 15 mins (up to 2hrs) after ingestion **Mild reaction** - Urticaria and itchy skin - Facial swelling **Severe reaction** - Breathing difficulties - Wheeze - Stridor - Abdominal pain, vomiting, diarrhoea - Shock, collapse

Answer 188

- Diarrohea - Vomiting - Abdominal pain - Sometimes failure to thrive

Answer 189

- Clinical history is key - timing after exposure to allergen, previous and subsquent exposure and reaction to allergen, symprtoms, personal or family Hx of atopy or allergy - Skin prick test - a drop of food is added to skin and site is marked, weals of ≥ 4mm are positive - Measure blood IgE - Food challenge test (gold stardard)

Answer 190

- Avoidance of relevant food(s) - Food labelling in the EU mandates listing common allergens - Child and family learning to manage allergic attack with written self-management plans and training. - Mild (no cardiorespiratory symptoms): non-sedating antihistamines - Severe - IM adrenaline by autoinjector (e.g. Epipen) - Cow's milk and egg allergy usually resolves in early childhood, but nuts and seafood usually persists through to adulthood.

Answer 191

- Breast-feeding - mothers should avoid dairy - Special hydrolysed formulas designed for cow's milk allergy. The milk protein is broken down so they don't trigger an immune response - Severe - elemental formulas of basic amino acids (e.g. neocate). - Slowly introduce milk products, they might eventually be able to tolerate it. Should outgrow by 3. - Same management for cow's milk intolerance -

Answer 192

- Life-threatening medical emergency - Severe type 1 hypersensitivity reaction, where IgE stimulates mast cells to rapidly release histamine and other pro-inflammatory chemicals - mast cell degranulation. - Rapid airway, breathing and/or circulation compromise.

Answer 193

- Often accompanied by history of exposure to allergen - Urticaria - Itching - Angio-oedema, with swelling around lips and eyes - Abdominal pain Additional symptoms - Shortness of breath - Wheeze - Swelling of the larynx, causing stridor - Tachycardia - Collapse

Answer 194

Medical emergency - managed by an experienced paediatrician ABCDE A – Airway: Secure the airway B – Breathing: O2 (15L 100%) - salbutamol for wheezing. C – Circulation: IV bolus of fluids D – Disability: Lie the patient flat to improve cerebral perfusion E – Exposure: Look for flushing, urticaria and angioedema 1. Intramuscular adrenaline, repeated after 5 minutes if required (150mcg for < 6, 300mcg for 6 to 12, 500mcg > 12 2. Antihistamines, e.g. oral chlorphenamine or cetirizine 3. Steroids, usually intravenous hydrocortisone

Answer 195

- Observation on paediatric unit as biphasic reactions are possible - Measure serum mast cell tryptase within 6 hours. It is released during mast cell degranulation and remains in blood for 6 hours after (common exam q!) - Education and follow-up of family and child, ensure family is trained in basic life support

Answer 196

- All children and adolescents with anaphylactic reactions - Generalised allergic reactions (without anaphylaxis) with risk factors: - Asthma requiring inhaled steroids - Poor access to medical treatment (e.g. rural)

Answer 197

Common condition in children Combination of acute gastritis - inflammation of the stomach and enteritis - inflammation of intestines.

Answer 198

- Rotavirus - Norovirus

Answer 199

Bacillus cereus - gram-positive rod, produces a toxin called cereulide when growing on food Course: - Abdominal pain + vomiting 5 hours of ingestion - Watery diarrhoea 8 hours after ingestion - Resolution within 24hrs Favourite one in exams because of its unique course

Answer 200

- E.coli - infected faeces, unwashed salads or contaminated water. - Campylobacter jejuni - traveller's diaherra: - Under cooked poultry - Untreated water

Answer 201

Incubation 2 to 5 days. Resolve after 3 to 6 days. Symptoms are: - Abdominal cramps - Diarrhoea often with blood - Vomiting - Fever Abx if severe or risk factors e.g. HIV

Answer 202

- Good hygiene - Isolation - barrier nursing and infection control - School absence until 48 hours after resolution - Microscopy, culture and sensentivies to idenify causative organism - Ensure hydration - oral or IV - Antidiarrhoeal medication such as loperamide and antiemetics not recommended

Answer 203

- Infection (gastroenteritis) - Inflammatory bowel disease - Lactose intolerance - Coeliac disease - Cystic fibrosis - Toddler’s diarrhoea - Irritable bowel syndrome - Medications (e.g. antibiotics)

Answer 204

A chronic non-specific diarrhoea seen in small children between 1 - 4. Common cause of chronic diarrhoea but should be a diagnosis of exclusion

Answer 205

- Frequent, poorly formed brown and smelly stools - Hallmark feature = food material in stools Important to note that the child is constitutionally well, normally active and has unimpaired growth. - Normal apetite and fluid intake - Examination and labs negative

Answer 206

Hisotry might reveal very high intake of fruit juices - hyperosmolar and contain sorbitol, a non-absorbable sugar alcohol - for example, pear juice, 2%; apple juice, 0.5%.

Answer 207

- Parental reassurance - Avoid full-strength fruit juices - Reduce intake of peas, corn and carrots which are not chewed properly and appear in stools - Faeces should become firm by age 3 years or when the child is toilet trained.

Answer 208

Protution of abdominal content through an opening in the diaphragm - usually left-sided through the posteriolateral foreman of the diaphragm

Answer 209

- Usually picked up on the antenatal scan - If not, presents with respirstory distress - Apex beat and heart dound displaced to right chest with poor air entry on left chest

Answer 210

XR of chest + abdomen

Answer 211

- Resusitation might cause pneumothrox of normal lung - If diagnosis suspected, then a large nasogastric tube used to apply suction to prevent distension of the intrathoracic bowel. Then surgrica; repair However, the worry with most infants is pulmonary hyplasia - whee the lung is underdeveloped due to compressinon from hernia, mortality is high.

Answer 212

Insulin is a hormone that regulates blood sugar levels and it is produced by the pancreas, specfically the beta cells in the islets of Langerhans. Type 1 diabetes occurs when the beta cells are unable to produce insulin, due to destruction through an autoimmune process. Insulin stimulates cells to take up glucose so they can use it as fuel, however if insulin is absent and cells cannot use glucose, the body will believe that it is starving and the glucose levels will keep rising.

Answer 213

1. Stimulate cells to absorb glucose from blood and use it as fuel 2. Stimulates muscle and liver cells to glucose from blood and store it as glycogen

Answer 214

Classic triad: - Polyuria - Polydipsia - Weight loss Less common: - Secondary enuresis (bedwetting in previously dry child) - Recurrent infections Symptoms starts 1 - 6 weeks before diabetic ketoacidosis

Answer 215

- Raised random blood glucose (>11.1 mmol/L), glycosuria, and ketosis. - If in doubt, fasting blood glucose > 7mmol/L) or raised HbA1c (> 48mmol/mol) - FBC, renal profile (U +Es) - TFT and thyroid peroxide antibodies for associated autoimmune thyroid disease - Tissue transglutaminase (anti-TTG) antibodies for associated coeliac's)

Answer 216

- Patient and family education - Patients need to become "expert patients" as they become independent from their family Management: - Long-acting (basal) and short-acting (bolus) insulin i.e. basal bolus regime Basal in the evening - provides constant insulin e.g. Lantus Bolus - before meal times, calculated according to the number of carbohydrates consumed, e.g. Actrapid - Insulin pumps - continuous infusion of insulin at different rates to control blood sugar levels

Answer 217

- Hunger - Tremor - Sweating - Irritability - Dizziness - Pallor - Severe: reduced consciousness, coma and death

Answer 218

- Rapid acting glucose - lucozade - SLower acting carbohydrates - biscuits or toast - Severe - IV dextrose and IM glucagon

Answer 219

Chronic hyperglycemia = damage to vascular endothelial cells, immunosuppression and optimal environment for infectious organisms MAcrovascular complications - Coronary artery disease - major cause of death - Peripheral ischaemia = poor healing, ulcers and diabetic foot - Stroke - HTN Microvascular complications - Peripheral neuropathy - Retinopathy - Renal disease particularly glomerulosclerosis (scarring of glomerulus)

Answer 220

- HbA1c - glycated haemoglobin - every 3 - 6 months - Capillary blood glucose - immediate result via glucose meter - Flash glucose monitoring (e.g. FreeStyle Libre) - measures glucose level in interstitial fluid, 5 minute lag behind blood glucose

Answer 221

DKA occurs in type 1 diabetes when the person is producing enough insulin and not injecting enough insulin. The body cannot use and process glucose, therefore the cells are starvinig and undergo ketogenesis to produce an alternate fuel. Eventually ketone and glucose levels keep increasing Initially the kidneys compensate by producing bicarbonate to buffer the ketone acids. Over time, the ketone acid use up the bicarbonate and the blood becomes acidic - this is ketoacidosis

Answer 222

- Dehydration due to hyperglycemia, the high levels of glucose in the body filters out through the kidney which draws water with it through osmotic diuresis - causes polyuria and polydipsia - Potassium imbalance - insulin drives potassium into cells. Blood K is normal or high in DKA but total body K is low as not stored in cells

Answer 223

Symptoms of the underlying hyperglycaemia, dehydration and acidosis: - Polyuria - Polydipsia - Nausea and vomiting - Weight loss - Dehydration and subsequent hypotension - Altered consciousness - Symptoms of an underlying trigger (i.e. sepsis)

Answer 224

- Check hospital criteria - Hyperglycaemia (i.e. blood glucose > 11 mmol/l) - Ketosis (i.e. blood ketones > 3 mmol/l) - Acidosis (i.e. pH < 7.3)

Answer 225

- Dehydration, potassium imbalance and acidosis kills the paitent! - Priority is fluid resus to correct the above - BUT rapid IV fluids = cerebral oedema! SO correct dehydration evenly over 48 hours - GIve fixed rate insulin infusion - allows cells to glucose and prevents ketone production - IV dextrose once BM < 14mmol/L - Add potassium to IV fluids and monitor levels - Treat underlying cause

Answer 226

A syndrome that occurs when an infection causes a child to become systemically unwell. It results from severe systemic inflammatory response - life-threatening! Low-threshold for treating suspected sepsis!

Answer 227

1. Pathogens enter bloodstream 2. Recognition by WBCs that release cytokines e.g. interleukins, TNF 3. Vasodilation, leaky capillary and blood clotting 4. Organs are not adequately perfused as blood is drawn into the extracellular space and used in clots, consumption of platelets and clotting factors also lead to disseminated intravascular coagulopathy 5. Blood lactate rises as hypo-perfused organs undergo anaerobic respiration which produces lactate 6. Septic shock - when sepsis leads to cardiovascular dysfunction, fall in systolic BP

Answer 228

- Aggressive IV fluids to improve BP, reduce lactate and tissue perfusion - If not effective, step up to high dependency or ICU

Answer 229

- Deranged physical observations - Prolonged capillary refill time (CRT) - Fever or hypothermia - Deranged behaviour - Poor feeding - Inconsolable or high pitched crying - High pitched or weak cry - Reduced consciousness - Reduced body tone (floppy) - Skin colour changes (cyanosis, mottled pale or ashen)

Answer 230

If under 3 months with temperature 38C or above

Answer 231

NICE guidelines: https://cks.nice.org.uk/topics/sepsis/diagnosis/risk-stratification/ Traffic system: green (low), amber, red (high) Patients categorised based on examination in various systems - Colour - normal vs cyanosis, pale, ashen - Activity - active happy responsive vs abnormal, drowsy or abnormal cry - Respiratory - normal vs respiratory distress, tachypnoea or grunting - Circulation and hydration - normal skin and moist membranes vs tachy, dry membranes

Answer 232

Emergency! Call for senior help early for experienced support! 1) GIve high-flow oxygen (15L 100% as standard) 2) Obtain IV/IO access and take bloods (FBC, U+E, CRP, clotting, blood gas for lactate and acidosis, cultures) 3) GIve abx within 1 hr of presentation 4) IVCfluids - 20ml/kg IV bolus saline if shock or lactate above 2mmol/L

Answer 233

- CXR - if pneumonia suspected - LP if meningitis suspected - Meningococcal PCR if meningococcal disease suspected - Once bacterial cause idenitified, culture and sensitivities can guide abx use, seek microbiologist advice - Continue abx for 5 - 7 days after

Answer 234

Inflammation of the meninges, the membrane lining the brain and spinal cord

Answer 235

Neonates: group B streptococcus (GBS) Older children: - Neisseria meningitidis (meningococcus) - Streptococcus pneumoniae (pneumococcus)

Answer 236

Meningococcal infection in the blood - causing "non-blanching rash"

Answer 237

- Fever - Neck stiffness - Photophobia - Meningococcal septicaemia - non-blanching rash Non-specific features: - Bulging fontanelle - Hypotonia - Poor feeding - Drowsiness - Hypothermia

Answer 238

- Kernig's test - lie flat on back, flex hip and knee at 90, straighten one knee at a time, pain/resistance = positive - Brudzinski's test - lie flat on back, lift patient's neck and head off bed flexing chin to chest, if hips and knees flex = positive

Answer 239

- Lumbar puncture for CSF analysis - Blood culture - Meningococcal PCR if meningococcal disease suspected

Answer 240

- <1 month with fever - 1 to 3 months with fever and are unwell - < 1 year with unexplained fever and other features of serious illness

Answer 241

- Urgent stat injection (IM or IV) of benzylpenicillin then transfer urgently to hospital

Answer 242

- Ideally LP and CSF analysis prior to abx but DO NOT delay treatment if patient is acutely unwell Follow local antibiotics guidelines, typically: - Under 3 months – cefotaxime + amoxicillin ( to cover listeria contracted during pregnancy) - Above 3 months – ceftriaxone - Steroids (dexamethasone) to prevent hearing loss and neurological damage - Notify public health

Answer 243

- Herpes simplex virus (HSV) - Enterovirus - Varicella zoster virus (VZV)

Answer 244

- Appearance: cloudy - Protein: high - Glucose: low - White Cell Count: high (neutrophils) - Culture: bacteria

Answer 245

- Appearance: clear - Protein: normal/mildly raised - Glucose: high - WCC: high (lymphocytes) - Culture: negative

Answer 246

A single dose of ciprofloxacin asap

Answer 247

- Upper respiratory tract infection caused by Bordetella pertussis (a gram-negative bacteria). - The whooping is caused by the forced inhalation of air after severe cough fits making a loud "whooping" noise - Children and pregnant women are vaccinated against pertussis.

Answer 248

- Starts with mild coryzal symptoms, low-grade fever and mild dry cough - After a week, severe cough fits start with cough-free periods in between - paroxysmal cough. - Cough fits keep building under patient is out of breath and the forced sucking of air causes loud whooping sound. - Coughing fits so severe can cause the patient to faint, vomit or even develop a pneumothorax Not everyone will whoop, and some infants might develop aponea instead of cough

Answer 249

- Nasopharyngeal or nasal swab with PCR testing or bacterial culture - Cough > 2 weeks = anti-pertussis toxin immunoglobin G test - oral fluids of 5 - 16, and bloods in 17 and older

Answer 250

Pertussis is a notifiable disease to UK Health Security Agency (UKHSA) - Usually supportive management, stay off school/work until 48hr after abx, and 14 days (21 days for high-risk work) from onset of cough - Acutely unwell with apnoea, cyanosis, severe coughing fits or < 6m = admission - Macrolide antibiotics such as clarithromycin if onset within 21 days or vulnerable patients. - Prophylactic antibodies for vulnerable close contacts - e.g. pregnant women or unvaccinated infants - Key complication = bronchiectasis.

Answer 251

Poor physical growth and development in a child

Answer 252

Fall in weight across: - One or more centile spaces if birth weight below 9th centile - Two or more centile spaces if their birth weight 9th - 91 st centile Three or more centile spaces if birth weight above 91st centile

Answer 253

- Inadequate nutritional intake (e.g. parental neglect, poverty) - Difficulty feeding (poor suck, pyloric stenosis) - Malabsorption (e.g. coeliac) - Increased energy requirements (e.g. hyperthyroidism) - Inability to process nutrition (T1DM) - Undiagnosed UTI

Answer 254

A full history, examining the child and completing relevant investigations. Key areas: - Pregnancy, birth, developmental and social history - Feeding history - breast/bottle feeding times, volume and frequency and any difficulties with feeding. - Eating history - food choices, food aversion, mealtime routines and appetite - Observe feeding - Mum's physical and mental health - Parent-child interactions - Height, weight and BMI (if > 2 years) and plotting these on a growth chart - Mid (mean)-parental height centile

Answer 255

- Height > 2 centile spaces below mid-parental height centile - BMI below the 2nd centile

Answer 256

NICE recommends the following initial investigations: - Urine dipstick - UTI - Coeliac screen (anti-TTG or anti-EMA antibodies)

Answer 257

- MDT - Faltering growth: regular reviews to monitor weight gain - Difficulties with breastfeeding: advice from midwives, lactation consultants, supplement with formula milk Inadequate nutrition: - Structured mealtimes - Reduced milk consumption - Energy-dense foods - Nutritional supplement drinks

Answer 258

Safeguarding involves all aspects of ensuring the welfare of a child. Legal framework = Children Act 1989

Answer 259

The process of protecting a child that is at risk of or suffering harm.

Answer 260

- Physical - Emotional - Sexual - Neglect - Financial - Identity

Answer 261

Not limited to the list below, talk to senior or safeguarding lead if in doubt: - Change in behaviour or extreme emotional states - Dissociative disorders - Bullying, self-harm or suicidal behaviours - Unusually sexualised behaviours - Poor hygiene - Poor physical or emotional development - Missing appointments or not complying with treatments

Answer 262

- Report concerns to the safeguarding lead, each NHS organisation should have one - Safeguarding lead will escalate further - Generally escalated to the children's services (social services) who can investigate further and decide further action - Usually social services will put in extra support for the family - If child is in immediate danger = police - Acutely unwell or need a place of safety = hospital admission Maintaining a professional, open, honest and trusting relationship with parents or carers is important, even when they are responsible for the abuse. This will make all aspects of any investigation and management easier, and lead to better outcomes. Extra support for parents - Home visit programmes to support parents - Parenting programmes - Attachment-based interventions to help bonding and nurturing - Child–parent psychotherapy - Multi-systemic therapy for child abuse and neglect (MST-CAN) - CBT for children who suffered abuse

Answer 263

- Peripheral cyanosis (blueness of the hands and feet) may occur when a child is cold or unwell - Central cyanosis - slate blue tongue blue colour, fall in arterial blood oxygen tension. - Persistent cyanosis in an otherwise well infant is sign of structural heart disease.

Answer 264

Cyanosis in a newborn infant with respiratory distress (respiratory rate >60 breaths/min) may be due to: - Cyanotic congenital heart disease - Respiratory distress syndrome (surfactant deficiency) - Infection–GBS septicaemia

Answer 265

Congenital heart disease - 1st line: CXR + ECG - Diagnostic: echocardiography + doppler USS

Answer 266

Chronic neurological disorder, characterised by recurrent unprovoked seizures, consisting of transient signs and/or symptoms associated with abnormal, excessive or synchronous neuronal activity in the brain Focal: Discharge arises from one or part of one hemisphere Generalised: Discharge arises from both hemispheres

Answer 267

- Generalised tonic-clonic - tonic (muscle tensing) and clonic (muscle jerking) - Focal - starts in temporal lobe and causes focal neurological deficits - Absence seizures - patient blanks and stares into space for 10 -20 secs - Atonic - brief lapses of muscle tone, lasts around 3 mins - Myoclonic - sudden brief muscle contraction, causing sudden jerks

Answer 268

Depends which area of the brain is affected (covered in more detail in neurology) They start in the temporal lobes - Hallucinations - Memory flashbacks - Déjà vu - Doing strange things on autopilot

Answer 269

- Detailed history - Electroencephalogram (EEG) - after second simple tonic-clonic seizure - MRI in first seizure is in children under 2 years, focal seizures, 1st line meds ineffective Consider: - ECG - Electrolytes - sodium, potassium, calcium and magasium - Blood glucose - Blood cultures, urine cultures and LP if sepsis or meningitis suspected

Answer 270

Important to avoid situations where a seizure might endanger the child - Showers rather than baths - Be very cautious with swimming unless seizures are well controlled and they are closely supervised - Be cautious with heights - Be cautious with traffic - Be cautious with any heavy, hot or electrical equipment - Older teens need to avoid unless specific criteria are met e.g. must be seizure-free for ≥12m before bring allowed to drive in an unaware seizure

Answer 271

- Teratogenic! Avoided in women of child-bearing age unless no alternative and strict criteria to ensure they do not get pregnant - Liver damage and hepatitis - Hair loss - Tremor

Answer 272

1st line: sodium valproate 2nd line: lamotrigine

Answer 273

First line: carbamazepine or lamotrigine Second line: sodium valproate or levetiracetam

Answer 274

Seizure associated with fever in the absence of another cause or intracranial infection Age group: Between 6 months and 5 years Occur early in an infection when temperature rises rapidly Brief generalized tonic-clonic (<5 min)

Answer 275

- Generalised, tonic clonic seizures that last < 15 mins and occur only once during a febrile illness

Answer 276

- Febrile convulsions are complex when they consist of focal seizures, last 15 - 30 mins, or occur multiple times during a febrile illness - Increased risk of epilepsy (4 - 12%, 1 - 2 % in gen pop)

Answer 277

- Epilepsy - Meningitis, encephalitis - Intracranial space occupying lesions, for example brain tumours or intracranial haemorrhage - Trauma (always think about non accidental injury

Answer 278

- Diagnosis of exclusion - Infection screen: blood and urine cultures and LP - If child is unconcious or haemodynamically unstable = DO NOT DELAY ABX - Typical presentation = child around 18m with tonic-clonic seizure during high fever - Usually caused by viral or bacterial infection

Answer 279

Admit to paediatrics if: - First seizure - Features of a complex seizure All cases - Identify and manage the underlying source of infection - Control the fever with simple analgesia (Eg. Calpol) Education on what to do in a future episode: - Stay with the child - Put the child in a safe place e.g. carpeted floor - Recovery position - - Don’t put anything in their mouth - - Rescue therapy with rectal diazepam or buccal midazolam If seizure > 5 mins = ambulance

Answer 280

- Usually no lasting damage, but 1 in 3 will have another febrile convulsion

Answer 281

- Gross motor - Vision and fine motor (vision needed for fine motor) - Hearing, speech and language - Social, emotional and behavioural.

Answer 282

Limit ages are the age by which the developmental milestones should have been achieved. Limit ages are usually 2SDs from the mean.

Answer 283

From the head to toe development - 4 months: able to support own head and - 6 months: They can keep their trunk supported on their pelvis, but often don’t have the balance to sit unsupported - 9 months: sit unsupported, crawl, maintain standing position and bounce when supported - 12 months: They should stand and begin cruising (walking whilst holding onto furniture). - 15 months: Walk unaided. - 18 months: Squat and pick things up from the floor. - 2 years: Run. Kick a ball. - 3 years: Climb stairs one foot at a time. Stand on one leg for a few seconds. Ride a tricycle. 4 years: Hop. Climb and descend stairs like an adult.

Answer 284

Expressive language milestones: - 3 months: Cooing noises - 6 months: Makes noises with consonants (starting with g, b and p) - 9 months: Babbles, sounding more like talking but not saying any recognisable words - 12 months: Says single words in context, e.g. “Dad-da” or “Hi” - 18 months: Has around 5 – 10 words - 2 years: Combines 2 words. Around 50+ words total. - 2.5 years: Combines 3 – 4 words - 3 years: Using basic sentences - 4 years: Tells stories Receptive language milestones: - 3 months: Recognises parents and familiar voices and gets comfort from these - 6 months: Responds to tone of voice - 9 months: Listens to speech - 12 months: Follows very simple instructions 18 months: Understands nouns, for example “show me the spoon” - 2 years: Understands verbs, for example “show me what you eat with” - 2.5 years: Understands propositions (plan of action), for example “put the spoon on/under the step” - 3 years: Understands adjectives, for example “show me the red brick” and “which one of these is bigger?” - 4 years: Follows complex instructions, for example “pick the spoon up, put it under the carpet and go to mummy”

Answer 285

Early Milestones: 8 weeks: Fixes their eyes on an object 30 centimetres in front of them and makes an attempt to follow it. They show a preference for a face rather than an inanimate object. 6 months: Palmar grasp of objects (wraps thumb and fingers around the object). 9 months: Scissor grasp of objects (squashes it between thumb and forefinger). 12 months: Pincer grasp 14-18 months: They can clumsily use a spoon to bring food from a bowl to their mouth.

Answer 286

When a child displays slow development in all developmental domains - gross motor, vision and fine motor, hearing and speech, language and cognition, social/emotional and behaviour.

Answer 287

- Down’s syndrome - Fetal alcohol syndrome - Fragile X

Answer 288

Delay in acquiring skills in the gross motor domain such as: sitting, crawling walking unaided, running, climbing etc.

Answer 289

- Cerebral palsy - Ataxia - Spina bifida - Visual impairment

Answer 290

Delay in acquiring skills in the fine motor domain such as grasping objects, drawing, writing

Answer 291

Expressive language: cooing, babbling, words, sentences Receptive language: understanding, responding

Answer 292

- Social circumstance - e.g. multiple languages - Hearing impairment - Learning disability - Neglect - Autism - Cerebral palsy

Answer 293

- Referral to speech and language, audiology and the health visitor. - Safeguarding if neglect is a concern

Answer 294

Personal and social refers to the child’s development of skills in interacting, communicating, playing and building relationships

Answer 295

- Lost/ regression of developmental milestones - Not able to hold an object at 5 months - Not sitting unsupported at 12 months - Not walking independently at 18 months - Not running at 2.5 years - No words at 18 months - No interest in others at 18 months - Strong parental concerns

Answer 296

Emotional and social neglect Parenting issues Autism

Answer 297

A neurodevelopmental condition characterised by extreme “hyperactivity” and inability to concentrate. It affects the person’s ability to carry out everyday tasks and perform well in school.

Answer 298

- Difficulties in sustaining attention and persisting tasks - Lack of impulsive control - Manifests as disorganised and poorly regulated - DIfficulties taking turns or waiting their turn - Interrupts others' play and conversation - often loses possessions - Short tempers and poor relationships as peers find them annoying - Low self-esteem

Answer 299

- Detailed assessment by a specialist - Management by a specialist - Parental and child education - strategies to manage child - Healthy diet and exercise can improve symptoms e.g. some food colourings can exacerbate symptoms Medications (CNS stimulants) , need to be managed by a specialist: - Methylphenidate (“Ritalin“) - Dexamfetamine - Atomoxetine

Answer 300

It is a neurodevelopmental condition characterised by a deficit in social interaction, communication and flexible behaviour. It encompasses a huge range of behaviours. Ranges from normal intelligence with difficulties with reading emotions and responding to severe learning disabilities and deficits in self-care.

Answer 301

Wide range of features which vary between individuals, categorised as followed: Social interaction - Lack of eye contact - Avoiding physical contact - Unable to read non-verbal cues Difficulties making and keeping friends - Not displaying desire to share (e.g. not playing with others) Behaviour - Intensive and deep interests that are persistent and rigid - Self-stimulating behaviours e.g. hand-flapping, rocking - Repetitive behaviour and fixed routines - Anxiety and distress with experiences outside their normal routine - Extremely restricted food preferences

Answer 302

- Made by a specialist e.g. paediatric psychiatrist - Detailed history and assessment of child's behaviour and social communication - A diagnosis can be made before yer of 3

Answer 303

- Depends on the severity of the condition MDT: - Child psychology and child and adolescent psychiatry (CAMHS) - Speech and language specialists - Dietician - Paediatrician - Social workers - SEN support - Charities e.g. national autistic society

Answer 304

AN is a psychriastic conditon where the person feel they are overweight despite evidence of normal or low body weight. It involves obsessively restricting calorie intake or excessive exercise.

Answer 305

- Excessive weight loss - Amenorrhoea - Lanugo hair - Hypokalaemia - Hypotension - Hypothermia - Changes in mood, anxiety and depression - Solitude Cardiac complications: arrhythmia, atrophy and sudden cardiac death.

Answer 306

Unlike with anorexia, people with bulimia often have a normal body weight. Their body weight tends to fluctuate. Involves cycles of binge eating and then purging by vomiting or taking laxatives.

Answer 307

- Alkalosis, due to vomiting hydrochloric acid - Hypokalaemia - Erosion of teeth - Swollen salivary glands - Mouth ulcers - Gastro-oesophageal reflux and irritation - Russell's sign - calluses on knuckles from being scraped across teeth

Answer 308

Characterised by episodes of excessive eating, often as a result of psychological distress. Not a restrictive disorder and patients are usually overweight Binges may involve: - A planned binge involving “binge foods” - Eating very quickly - Unrelated to hunger - Becoming uncomfortably full - Eating in a “dazed state”

Answer 309

- FBC - Serum chemistry - metabolic alkalosis and hypokalaemia (if vomiting is present) - TFT - LFT

Answer 310

- Patient and carer education Management focuses on behaviour and environmental changes - Self help resource - Counselling - Cognitive behavioural therapy (CBT) - Addressing other areas of life, such as relationships and past experiences - Severe cases might need admission, and close monitoring for refeeding syndrome

Answer 311

Refeeding syndrome occurs in people that have been in a severe nutritional deficit when they start to eat again. When cells receive glucose, protein and fat again, they use up electrolytes leading to: - Hypomagnesaemia - Hypokalaemia - Hypophosphatemia Risks of cardiac arrhythmias, heart failure and fluid overload.

Answer 312

Depends on local protocol under specialist supervision: - Slowly reintroducing food with restricted calories - Magnesium, potassium, phosphate and glucose monitoring along with other routine bloods - Fluid balance monitoring - Supplements with B vitamins and thiamine

Answer 313

Cancer of a particular line of stem cells in the bone marrow. Haroon says only Acute lymphoblastic leukaemia (ALL) - accounts for 80% of leukaemia in children

Answer 314

Abnormal rapid proliferation of lymphoblasts - the precursor cells to lymphocytes Peak is 2 - 5 years old

Answer 315

Genetic mutation in one of the precursor cells in bone marrow leads to excessive production of a single type of abnormal white blood cell. Suppression of other cell lines, underproduction of other cell types Result = pancytopenia, low: - Red blood cells (anaemia), - White blood cells (leukopenia) - Platelets (thrombocytopenia)

Answer 316

- Radiation exposure - abdominal x-ray during pregnancy is main environmental risk factor - Down’s syndrome

Answer 317

Non-specific - Persistent fatigue - Unexplained fever - Failure to thrive - Weight loss - Night sweats - Pallor (anaemia) - Petechiae (non-blanching spots < 2mm) - Abnormal bruising (thrombocytopenia) - Unexplained bleeding (thrombocytopenia) - Generalised lymphadenopathy - Unexplained or persistent bone or joint pain - Hepatosplenomegaly

Answer 318

- NICE recommend to refer any child with unexplained petechiae or hepatomegaly for specialist assessment - FBC within 48 hours - pancytopenia - Blood film - blast cells - BM and lymph node biopsy

Answer 319

Main treatment: chemotherapy Other treatments - Radiotherapy - BM transplant - Surgery

Answer 320

ALL cure rate = 80%

Answer 321

- Astrocytoma (~40%) - varies from benign to highly malignant (glioblastoma multiforme) Mostly primary unlike in adults

Answer 322

- Medulloblastoma - arises in the midline of the posterior fossa - gives rise to spinal metastases - Ependymoma - mostly in the posterior fossa where it behaves like medulloblastoma - Brainstem glioma - Craniopharyngioma - developmental tumour arising from an embryological remnant.

Answer 323

Malignancy arising from astrocytes, cells that support the nerve cells in the brain - Raised ICP = headache and behavioural changes - Seizures - Persistent or recurrent vomiting - Problems with balance, coordination or walking - Abnormal eye movements

Answer 324

- MRI - Lumbar puncture for staging - seek neurosurgical advice if raised ICP

Answer 325

1st line: surgery - treats hydrocephalus, providing a tissue diagnosis and resection. Radiotherapy after surgery for malignant CNS tumours

Answer 326

Most common malignant renal tumour in children, typically affecting children under 5 years of age

Answer 327

- Peak: 2 - 5 years old - Family history Congenital syndromes: e.g. WAGR

Answer 328

Incidental finding of abdominal mass most common - Abdominal pain - Haematuria - Lethargy - Fever - Hypertension - Weight loss

Answer 329

Abdo ultrasound to visualise kidneys CT or MRI scan to stage the tumour. Biopsy to identify histology for definitive diagnosis

Answer 330

Referral for urgent paediatric review within 48 hours if enlarged unexplained abdominal mass Surgical excision of the tumour along with the affected kidney (nephrectomy). Adjuvant chemotherapy Adjuvant radiotherapy Renal transplantation if bilateral and renal failure

Answer 331

Malignancies of immune system cells - Hodgkin's (common in adolescence) - Non-Hodgkin's (common in childhood)

Answer 332

Classically painless lymphadenopathy in the neck, clinical history usually several months B-symptoms - pruritus, weight loss and fever are uncommon

Answer 333

- Lymph node biopsy - Reed-Sternberg cells (owls eye nuclei) - Radiology of nodal sites and bone marrow biopsy to stage disease

Answer 334

Combination chemo (multiple drugs) +/- radiotherapy (same for NHL) Positron emission tomography (PET) scan to monitor treatment response

Answer 335

- Enlarging node without clear infective cause - Persistently enlarged node - Unusual site e.g. supraclavicular - Associated fever, weight loss, enlarged liver/spleen - If CXR abnormal (From lecture)

Answer 336

Cancer of the lymphatic system that histologically do not have Reed-Sternberg cells present. Can either be T-cell or B-cell malignancy

Answer 337

- T-cell and B-cell malignancies - mediastinal mass (between the lungs) with varying degrees of bone marrow infiltration. Can lead to SVC obstruction - dyspnoea, facial swelling and flushing, and venous distention in the neck - B-cell malignancies - painless lymphadenopathy in neck, throat, armpit or groin - Abdominal pain if abdominal disease from obstruction

Answer 338

Biopsy, CT or MRI of nodal sites and examination of bone marrow and CSF analysis

Answer 339

Multiagent chemotherapy, survival 80% now!

Answer 340

Diagnostic testing - testing fetus or person for genetic condition e.g. amniocentesis for Down's syndrome Predictive testing - specific gene mutation e.g. BRCA1 breast cancer Carrier testing - testing parents for gene for a specific autosomal recessive condition Make sure the patient gives their informed consent as test results can have life-changing implications

Answer 341

Karyotyping - looks at number of chromosomes, size and structure - helps to diagnose Down's (trisomy 21) or Turner's syndrome (45 XO) Microarray Testing - lysing genetic material using enzymes, then tested for by plating it and comparing it to a known gene of certain size e.g. for cystic fibrosis Specific Gene Testing - Double stranded DNA is split - Gene probe made of complementary DNA for the specific gene you want to test for. - If the specific gene is present, they will bind to the gene probe - Used to confirm whether a patient has a particular gene

Answer 342

A condition caused by an extra copy of chromosome 21 - trisomy 21. Associated with dysmorphic features and a number of other conditions e.g. VSD and increased risk of ALL

Answer 343

- Hypotonia (reduced muscle tone) - Brachycephaly (small head with a flat back) - Short neck - Short stature - Flattened face and nose - Prominent epicanthic folds - Upward sloping palpebral fissures (upslanting eyes) - Single palmar crease

Answer 344

- Learning disability - Recurrent otitis media - Deafness. - Eustachian tube abnormalities = glue ear and conductive hearing loss. - Visual problems such as myopia (nearsightedness), and cataracts - Cardiac defects affect 1 in 3, particularly ASD, VSD, patent ductus arteriosus and tetralogy of Fallot Increased risk of: - Leukaemia - Dementia

Answer 345

All women offered screening to see.if more invasive produces needed to confirm diagnosis 1st line: combined test: 11 - 14 weeks gestation - USS and maternal blood test Ultrasound measures nuchal translucency - the thickness of the back of the neck of the fetus, > 6mm = Down's syndrome Maternal blood tests: - Beta‑human chorionic gonadotrophin (beta-HCG), higher result = greater risk. - Pregnancy‑associated plasma protein‑A (PAPPA), lower = greater risk. Triple Test: 14 - 20 weeks gestation, maternal blood test results - Beta-HCG - Alpha-fetoprotein (AFP), lower = greater risk - Serum oestriol (female sex hormone), lower = greater risk Quadruple Test: 14 and 20 weeks gestation. Same as triple test but includes inhibin-A, higher = greater risk

Answer 346

If risk score is > 1 in 150 from screening, then offered amniocentesis or chorionic villus sampling. Chorionic villus sampling (CVS) - ultrasound-guided biopsy of placental tissue (< 15w) Amniocentesis involves ultrasound-guided aspiration of some amniotic fluid Non-Invasive Prenatal Testing (NIPT) = relatively new abnormalities test. Analyses maternal blood sample which contains fetal DNA to check for conditions such as Down's Syndrome

Answer 347

Supportive care from MDT - Occupational therapy - Speech and language therapy - Physiotherapy - Dietician - Paediatrician - GP - Health visitors - Cardiologist - ENT specialist - Audiologist for hearing aids - Optician for glasses - Social services for social care and benefits - Educational needs support - Charities such as the Down’s Syndrome Association

Answer 348

When asked by the examiner about management of a complex multi-system disorder such as Down's syndrome, start answer with "management would involve members of the multidisciplinary" - picks up most of the marks

Answer 349

- Regular thyroid checks (2 yearly) - Echocardiogram - Regular audiometry - Regular eye checks

Answer 350

Klinefelter syndrome occurs when a male has an additional X chromosome, making them 47 XXY. Rarely 48 XXXY or 49 XXXXY - more severe features

Answer 351

Normal male until puberty, then develop indictive features - Taller height - Wider hips - Gynaecomastia - Weaker muscles - Hypogonadism with small testicles - Reduced libido - Shyness - Infertility - Subtle learning difficulties (particularly affecting speech and language)

Answer 352

- Testosterone injections - Advanced IVF techniques - Breast reduction surgery Multidisciplinary team input: - Speech and language therapy - Occupational therapy - Physiotherapy - Educational support if dyslexia or learning disabilities

Answer 353

Life expectancy is close to normal. There is a slight increased risk of: - Breast cancer compared with other males (but still less than females) - Osteoporosis - Diabetes - Anxiety and depression

Answer 354

When a female only has one X chromosome instead of the normal 2 - so 45XO Classic triad to remember for exams: - Short stature - Wedded or thick neck - Widely spaced nipples Others - - Cubitus valgus - abnormality of the elbow, arms extended with palms facing forward - Underdeveloped ovaries - Late or incomplete puberty - Most women are infertile

Answer 355

- Recurrent otitis media - Recurrent UTI - Coarctation of the aorta - Hypothyroidism - Hypertension - Obesity - Diabetes - Osteoporosis - Specific learning disabilities

Answer 356

- Growth hormone therapy for short stature - Oestrogen and progesterone replacement for female secondary sex characteristics, regulate the menstrual cycle and prevent osteoporosis - Fertility treatment - Regular monitoring - Control associated conditions such as hypertension and hypothyroidism

Answer 357

Trisomy 18

Answer 358

Trisomy 13

Answer 359

Affected fetuses are detected during antenatal scans, and the diagnosis is confirmed by amniocentesis and chromosomal analysis Or non-invasive prenatal testing (NIPT) can be used

Answer 360

- Low birthweight - Prominent occiput - Small-mouth and chin - Short sternum - Flexed, overlapping fingers - ‘Rocker-bottom’ feet - Cardiac and renal malformations

Answer 361

- Rocker bottom feet - Structural defect of the brain - Scalp defects -Small eyes (microphthalmia) - Cleft lip and palate - Polydactyly - Cardiac and renal malformations.

Answer 362

- Genes carried on the X chromosome - Females are carriers, 50% of sons are affected, 50% of daughters are carriers. - All daughters of affected males are carriers. - None of the offspring of affected males have the disorder. - Possibility of new mutations.

Answer 363

Caused by mutation in the FMR1 (fragile X mental retardation 1) gene on the X chromosome. Codes for fragile X mental retardation protein, which plays a role in cognitive development in the brain

Answer 364

- It is X-linked, but whether it is dominant or recessive is unclear. - Males are always affected, but females vary in how severely they are affected. - Females have a spare normal copy of the FMR1 gene on their other X chromosome. - De novo (random) mutation are possible

Answer 365

- Speech and language delay - Intellectual disability (average IQ 50) - Long, narrow face - Large ears - Large testicles after puberty - Hypermobile joints (particularly in the hands) - ADHD - Autism - Seizures

Answer 366

MDT for supportive treatment - Learning disability - Seizures - ADHD - Autism

Answer 367

A genetic condition caused by the loss of functional genes on the proximal arm of chromosome 15 inherited from the father. Deletion of that portion of the chromosome or both copies of chromosome 15 are inherited from the mother.

Answer 368

- Constant insatiable hunger that leads to obesity - Poor muscle tone as an infant (hypotonia) - Mild-moderate learning disability - Hypogonadism - Fairer, soft skin that is prone to bruising - Mental health problems, particularly anxiety - Narrow forehead - Almond-shaped eyes - Strabismus (squint) - Thin upper lip

Answer 369

Under dietician guidance - Carefully limiting access to food with locks on cupboards and fridges - Lower than normal calorie intake - Education to teachers, carers and relatives. Growth hormone = treatment recommended by NICE, improves muscle development, body composition and obesity MDT: - Dietician - Education support - Social workers - Psychologists or psychiatrists - Physiotherapists - Occupational therapists

Answer 370

A genetic syndrome caused by loss of function of the UBE3A gene, specifically the copy inherited from the mother - only that copy works. Caused either by a deletion on chromosome 15, a specific mutation, or where two copies of chromosome 15 is from the father, with none from mum.

Answer 371

Main features: - Unusual fascination with water - Happy demeanour - Widely spaced teeth Other features: - Delayed development and learning disability - Severe delay or absence of speech development - Ataxia

Answer 372

MDT to support patient and family - Parental education - Social services and support - Educational support - Physiotherapy - Occupational therapy - Psychology - CAMHS - Anti-epileptic medication where required

Answer 373

Genetic condition caused by deletion on one copy of chromosome 7. Results in only a single copy of the gene on deleted region Usually occurs spontaneous deletion around conception rather than inherited

Answer 374

Characteristic features - Very sociable personality - Starbust eyes (star-like pattern on the iris) - Wide mouth with big smile

Answer 375

- Supravalvular aortic stenosis (narrowing just above the aortic valve) - Hypercalcaemia Easy to test in exams

Answer 376

MDT approach to support patient and family - Echocardiograms and blood pressure monitoring are important to assess for aortic stenosis and hypertension. Low-calcium diet and avoid it D and calcium supplement to control hypercalcaemia

Answer 377

The urinary tract includes the urethra, bladder, ureters and kidneys. Urinary tract infections are infections anywhere along this pathway.

Answer 378

K - Klebsiella E - E.coli (most common) P - Proteus (most in boys as found under foreskin) P - Pseudomonas S- Streptococcus faecalis

Answer 379

Fever - sometimes the only symptom, especially in young children. Babies might present with non-specific symptoms: - Fever - Lethargy - Irritability - Vomiting - Poor feeding - Urinary frequency More specific in older children: - Fever - Abdominal pain - suprapubic - Vomiting - Dysuria - Urinary frequency - Incontinence

Answer 380

1st line: urine dipstick Ideally - clean-catch sample to avoid contamination and unreliable results Nitrites = gram-negative bacteria (such as E. coli) break down nitrates in urine into nitrites. Leukocytes = significant rise due to infection + inflammation If both present = UTI If only nitrite = treat as UTI as better indicator If only leukocytes = don't treat as UTI unless clinical evidence If nitrite or leukocytes present = send MSU to lab for culture and sensitivity testing

Answer 381

< 3 month with fever = immediate admission with IV abx (e.g. ceftriaxone), full septic screen with bloods, cultures and lactate > 3 months = oral abx if otherwise well for 3 days If sepsis or pyelonephritis = in-patient IV abx for 2-4 days then oral abx for 7 - 10 days Follow local guidelines, typical UTI abx in children: - Trimethoprim - Nitrofurantoin - Amoxicillin

Answer 382

Investigate for underlying cause and kidney damage - Abdominal ultrasound scans if reccurent or atypical - DMSA (Dimercaptosuccinic Acid) Scan 4 - 6 months after illness to assess the damage, DMSA (radioactive material) is injected and gamma camera assesses how kidneys take up dye. - Areas which have no dye = damage

Answer 383

- Seriously ill/Septicaemia - Poor urine flow - Abdominal mas - Raised creatinine - Failure to respond to Abx within 48 hours - Infection with non E coli organism

Answer 384

- Where urine has a tendency to flow from the bladder back into the ureters. This predisposes patients to develop upper urinary tract infection

Answer 385

Micturating cystourethrogram (MCUG). When there is atypical or recurrent UTIs in < 6m or FHx of vesico-ureteric reflux: 1. Catheterisation 2. Contrast injected into bladder 3. Imaging to determine whether contrast is refluxing into ureters Prophylactic abx 3 days up to Ix

Answer 386

Depends on severity - Avoid constipation - Avoid an excessively full bladder - Prophylactic antibiotics - Surgical input from paediatric urology

Answer 387

Acute pyelonephritis is when the infection affects the tissue of the kidney. It can lead to scarring in the tissue and consequently a reduction in kidney function.

Answer 388

Along with the clinical features seen in UTI, the diagnosis of acute pyelonephritis is made if either there is: - A temperature greater than 38°C - Loin pain or tenderness

Answer 389

Involuntary urination at night - i.e. bed-wetting Most children can remain dry at night by 3-4

Answer 390

Inability to control bladder during the day, most children gain control of daytime urination by 2 years old

Answer 391

This is where the child has never managed to remain consistently dry ar night

Answer 392

Most common - Varation in normal development, particularly if younger than 5 - Family history Other causes - Overactive bladder - Fluid intake before bed Psychological distress - e.g. pressures at school - Secondary - chronic constipation, UTI, learning disability

Answer 393

- Initially - 2-week diary of toileting, fluid intake and bedwetting episodes to see patterns and areas for change - Reassurance for parents if child is < 5 that it will likely resolve without treatment - Reduce evening fluid intake, easy access to loo - Encouragement and positive reinforcement e.g. star/sticker charts for agreed behaviours e.g. help change sheets, not for dry nights - Avoid punishment as it can be counterproductive - Enuresis alarms (prolonged use > 3m) - Desmopressin, a synthetic analogue of antidiuretic hormone

Answer 394

Where a child begins wetting the bed when they have previously been dry for at least 6 months. Indicator of underlying illness/maltreatment

Answer 395

- UTI - Constipation - Type 1 diabetes - New psychological problems e.g. stress at school - Abuse at home- consider safeguarding - especially if it is deliberate, parental punishment or unexplained Treat underlying causes or refer to secondary care for specialist management

Answer 396

Daytime incontinence, the child is dry at night but has episodes of urinary incontinence during the day.

Answer 397

More common in girls - Urge - overactive bladder with little warning before emptyinh - Stress - leakage caused by increased pressure on bladder e.g. coughing, laughing

Answer 398

A conditon that occurs when the glomerulus BM becomes highly permeable to protein, allowing proteins to leak from the blood into the urine. Most common 2 and 5 years.

Answer 399

- Low serum albumin <25g/l - High urine protein content (>3+ protein on urine dipstick) - Oedema - Presents with frothy urine, generalised oedema (particularly periorbital on waking) and pallor.

Answer 400

- Deranged lipid profile, with high levels of cholesterol, triglycerides and LDLs - HTN - Hyper-coagulability

Answer 401

Minimal change disease (90%) - can occurs in otherwise healthy children with no risk factors.

Answer 402

- Renal biopsy and standard microscopy = no abnormalities - Urinalysis = small molecular weight proteins and hyaline casts.

Answer 403

- Corticosteroids (i.e. prednisolone). - Prognosis = good, but might reoccur.

Answer 404

Managed by experienced pediatrician and renal specialist - High dose steroids (i.e. prednisolone) - 4w then wean over 8w - Low salt diet - Diuretics - Albumin infusions if severe hypoalbuminaemia - Antibiotic prophylaxis if severe - 80% children respond to steroids = steroid sensitive - relapse likely - 20% steroid resistant - ACE inhibitors and immunosuppressants such as cyclosporine, rituximab

Answer 405

- Hypovolaemia - Thrombosis - anti-clotting proteins lost in urine and liver responds to low albumin by producing prothrombotic proteins - Infection - immunoglobulins leaked in urine - Acute or chronic renal failure - Relapse

Answer 406

Injury or damage to the kidney that can lead to acute renal failure - a sudden, reversible reduction in kidney function. Oliguria (<0.5 ml/kg/hr)

Answer 407

**Prerenal - mot common in children** Hypovolaemia: - gastroenteritis - sepsis - haemorrhage **Renal** Most common: haemolytic uraemic syndrome **Postrenal** Obstruction e.g. blocked catheter

Answer 408

Prerenal - Hypovolaemia so urgently corrected with fluid replacement and circulatory support to avoid acute tubular injury and necrosis Renal - Circulatory overload treated with fluid restriction and diuretic to increase urine output to balance sodium and water - HIgh-calorie, normal protein feed - Urgently treat metabolic acidosis, hyperkalaemia, and hyperphosphataemia Post-renal - Identify site of obstruction and relief by nephrostomy (bag + tube to drain urine from kidney) or bladder catheterization. Dialysis if above ineffective or severe metabolic acidosis

Answer 409

Haemolytic uraemic syndrome (HUS) involves thrombosis in small blood vessels throughout the body, usually triggered by Shiga toxins from either E. coli O157 (raw/undercooked ground meat) and Shigella (contaminated food/drink, infected person)

Answer 410

Mostly affects children after gastroenteritis episode. Antibiotics and anti-motility medication (e.g., loperamide) used to treat gastroenteritis increases risk

Answer 411

- Microangiopathic haemolytic anaemia (destruction of RBC in small vessels) - Acute kidney injury (damage by thrombi) - Thrombocytopenia (low platelets) as they are used up in clots

Answer 412

Gastroenteritis caysed by E.coli O157 or shigella DIarrhoea which turns bloody after 3 days Then HUS symptoms a week after diarrhoea onset: - Fever - Abdominal pain - Lethargy - Pallor - Reduced urine output (oliguria) - Haematuria - Hypertension - Bruising - Jaundice (due to haemolysis) - Confusion

Answer 413

Stool culture for causative organism. Medical emergency = hospital admission and treatment of: - Hypovolaemia (e.g., IV fluids) - Hypertension - Severe anaemia (e.g., blood transfusions) - Severe renal failure (e.g., haemodialysis) Self-limiting and most recover with good supportive care

Answer 414

Progressive loss of renal function due to numerous conditions. Familial and congenital conditions are more common than acquired in children.

Answer 415

Stage 1: eGFR >90 ml/min Stage 2: eGFR 60–89 ml/min Stsge 3: eGFR: 30–59 ml/min Stage 4: 15–29 ml/min Stage 5: <15 ml/min

Answer 416

- Renal dysplasia ± reflux - Obstructive uropathy - Glomerular disease - Congenital nephrotic syndrome

Answer 417

Typical asymptomatic until renal function < 1/3 of normal or stage 4. Often picked up on antenatal ultrasound: - Anorexia and lethargy - Polydipsia and polyuria - Faltering growth - HTN - Acute-on-chronic renal failure precipitated by infection/dehydration - Incidental finding of proteinuria

Answer 418

- Good protein intake and calorie supplements to maintain growth, nasogastric/gastrostomy feeding if required - Phosphate (milk products) restriction and activated vitamin D to prevent renal osteodystrophy - Bicarbonate supplements to prevent acidosis - Erythropoietin to prevent anaemia - Growth hormone - Dialysis and transplantation

Answer 419

Tumour arising from the neural crest tissue in the adrenal medulla and sympathetic nervous system. - Usually < 5 years of age - Good prognosis, 80% cure rate

Answer 420

- Abdominal mass usually adrenal in origin - but can anywhere along sympathetic chain from the neck to the pelvis - Pallor - Weight loss - Hepatomegaly - Bone pain - Limp

Answer 421

- Urinary catecholamine (e.g. adrenaline) - raised - Biopsy - Bone marrow sampling

Answer 422

- Surgery - often curative in local disease - Chemo, radiotherapy and surgery - metastatic disease - Prognosis for metastatic disease is poor

Answer 423

- Aka mucocutaneous lymph node syndrome. - Systemic, medium-sized vessel vasculitis. Typically children < 5 years. - No clear cause or trigger - More common in boys, particularly Japanese and Korean

Answer 424

- Persistent high fever >39 for more than 5 days - Unhappy and unwell - Widespread erythematous maculopapular rash (flat discoloured colour and raised bumps) and desquamation (skin peeling) on the palms and soles. Other features include: - Strawberry tongue (red tongue with large papillae) - Cracked lips - Cervical lymphadenopathy - Bilateral conjunctivitis

Answer 425

TOM TIP: If you come across a child with a fever persisting for more than 5 days, think of Kawasaki disease! A rash, strawberry tongue, lymphadenopathy and conjunctivitis will seal the diagnosis in your exams.

Answer 426

- FBC - anaemia, leukocytosis, thrombocytosis and CRP - LFT - hypoalbuminemia and raised enzymes - Urinalysis - leukocytosis without infection - Gold standard: Echocardiogram - due to increased risk of coronary artery aneurysm

Answer 427

1st line - High dose aspirin to reduce the risk of thrombosis - IV immunoglobulins to reduce the risk of coronary artery aneurysms - Follow-up: echo to monitor for coronary artery aneurysms - Kawasaki disease - aspirin used in children, usually avoided in children due to risk of Reye’s syndrome. EXAM FODDER!

Answer 428

An infection that spreads very easily, best prevention is the MMR vaccine. On the rise in the UK due to public anxiety about the MMR vaccine. Initial exposure occurs through droplet spread and it is highly infectious during viral shedding.

Answer 429

- Fever - Koplik spots (blue, white spots on the inside of the cheek) - Conjunctivitis - Coryzal symtoms - Cough - Maculopapular rash which spreads downwards from behind the ears to the whole of the body

Answer 430

Oral fluid (OF) sample: tested for measles IgM (first) /IgG (later) and measles RNA

Answer 431

- Supportive treatment - oral fluids, rest, paracetamol/ibuprofen - Stay off school until 5 days after rash onset - Notify public health

Answer 432

- Otitis media (most common) - Pneumonia (most common of death worldwide)

Answer 433

- Caused by varicella zoster virus (VZV), and it results in a highly contagious, generalised vesicular rash (fluid-filled blisters). - Immunity after one episode and will not be affected again

Answer 434

Characterised by widespread, erythematous, raised vesicular (fluid-filled), blistering lesions Starts on trunk or face and spreads outwards to whole body over 2- 5 days When lesions scab over, they stop being contagious Other symptoms: - Fever is often the first symptom - Itch - General fatigue and malaise

Answer 435

Highly contagious Direct contact with lesions or infected droplets from cough or sneeze Incubation period = 10 days to 3 weeks

Answer 436

- Bacterial superinfection - Dehydration - Conjunctival lesions - Pneumonia - Encephalitis (presenting as ataxia) - After the infection, the virus can lie dormant in sensory dorsal root ganglion cells and cranial nerves reactivate later in life as shingles or Ramsay Hunt syndrome.

Answer 437

- If a pregnant woman is not immune and is exposed to chickenpox, varicella zoster immunoglobulins given - Chickenpox in pregnancy < 28 weeks can lead to developmental problems i.e. congenital varicella syndrome - Chickenpox in mother around time of delivery can lead to life-threatening neonatal infection - treated with immunoglobulins and aciclovir.

Answer 438

- Supportive treatment - Complications such as encephalitis require admission - Itching - calamine lotion and chlorphenamine (antihistamine). - Absence from school and avoid pregnant women and immunocompromised patients until lesions are dry and crusted over

Answer 439

A generally mild disease in children that occurs in winter and spring Rare in UK now due to the MMR vaccine Incubation period is 15 to 20 days, spread through infected droplets

Answer 440

- Low grade fever - Maculopapular rash starting on face then spreads across body - Cough, running nose, sore throat

Answer 441

- Supportive - Diagnosis should be confirmed serologically if any risk of exposure to non-immune pregnant women

Answer 442

Congenital rubella syndrome

Answer 443

Caused by an exfoliative staphylococcal toxin which causes separation of epidermal skin from the granular cell layer. Mainly affects infants and young children <5

Answer 444

Caused by an exfoliative staphylococcal toxin which causes separation of epidermal skin from the granular cell layer. Mainly affects infants and young children < 5

Answer 445

- Fever - Malaise - Purulent, crusting, localised infection around eyes/nose/mouth with widespread erythema and tenderness of the skin - Epidermis separates on gentle pressure (Nikosky sign)

Answer 446

- IV anti-staphylococcal antibiotics (e.g. flucloxacillin) - Fluids - Analgesia

Answer 447

TB an infectious disease caused by Mycobacterium tuberculosis, a small rod-shaped bacteria (a bacillus). They are acid-fast due to a waxy coating, stained using Zeihl-Neelsen stain, and bright red against a blue background. On the rise again due to children with HIV and multidrug-resistant TB

Answer 448

- Most children are infected by an infected adult in the household - Immigrants from areas with high tuberculosis prevalence - People with relatives or close contacts from countries with a high rate of TB - Immunocompromised (e.g., HIV or immunosuppressant medications)

Answer 449

- Nearly 50% infants and 90% older children have no or minimal symptoms. - However, they will have latent disease - positive tuberculin skin test (aka Mantoux tests) Symptomatic - Fever - Anorexia and weight loss - Cough - CXR changes - hilar lymphadenopathy, lung lesions (Ghon focus) with surrounding lymph node invovlement = Ghon complexes - Then comes dormancy and dissemination - Reactivation - Post-primary TB

Answer 450

Localized disease or widely disseminated (called miliary TB), to sites such as bones, joints, kidneys, pericardium, and CNS. Infants and young children prone to tuberculous meningitis.

Answer 451

- Gastric washing x3 consecutive mornings using NG tube then Ziehl–Neelsen stain and mycobacterial culture to identify sensitivities - Mantoux tests - but could be positive due to past vaccination. - UK guidelines state that ≥ 5mm induration = positive - interferon-gamma release assays (IGRA) is a new blood test that assesses T cells' response to antigens found in TB but not the BCG vaccine.

Answer 452

RIPE Rifampicin, isoniazid, pyrazinamide, ethambutol Then just rifampicin and isoniazid after 2 months Unless MDR-TB then depending on sensitivities Side effects Rifampicin “red-I’m-pissin’” can cause red/orange discolouration of secretions Isoniazid (“I’m-so-numb-azid”) can cause peripheral neuropathy. Pyridoxine (vitamin B6) is co-prescribed to reduce the risk. Pyrazinamide can cause hyperuricaemia (high uric acid levels), resulting in gout and kidney stones. Ethambutol (“eye-thambutol”) can cause colour blindness and reduced visual acuity. Rifampicin, isoniazid and pyrazinamide are all associated with hepatotoxicity.

Answer 453

Mother to baby - during pregnancy (intrauterine), at delivery (intrapartum) or breast-feeding Affects over 3 million children worldwide

Answer 454

>18 months - detection of antibodies against the virus < 18 months = HIV DNA PCR as antibodies (transplacental maternal IgG) from the mother can be present in infants under 18m Two negative HIV DNA PCRs within the first 3 months of life, at least 2 weeks after completion of postnatal antiretroviral therapy (ART), indicate the infant is not infected, although this is confirmed by the loss of maternal HIV antibodies from the infant’s circulation after 18 months of age.

Answer 455

- Some infants progress rapidly to symptomatic disease and AIDs within 1 year - However, most are asymptomatic for a few years - Mild immunoexpression = lymphadenopathy - Moderate = recurrent bacterial infections, chronic diarrhoea and lymphocytic interstitial pneumonitis - Severe AIDS = opportunistic infections such as pneumocystis jirovecii pneumonia (PCP), severe faltering growth and encephalopathy.

Answer 456

Antiretroviral therapy should be started in all infants and some older children depending on clinical status, HIV load and CD4 count - aim to achieve a normal CD4 count and undetectable viral load. - Immunisations - routine immunisation schedule - MDT approach including the family - Regular follow-ups should include weight, development, and clinical signs of disease. - Effective ART means HIV is more of a chronic disease - Paediatric HIV clinics in the UK increasingly manage adolescents and ART adherence, safe sex practices, fertility, and pregnancy.

Answer 457

Encephalitis is Inflammation of the brain which can be either infectious or non-infections (usually autoimmune) - Infection by neurotoxic virus e.g. enteroviruses, respiratory viruses (influenza), HSV-1 - Delayed swelling (postinfectious encephalopathy), e.g. following chickenpox - Slow virus infection e.g. HIV

Answer 458

- Fever, altered consciousness, and often seizures. - Behavioural changes - Initially indistinguishable from meningitis

Answer 459

- FBC - Blood glucose and blood gas (for acidosis) - Coagulation screen, C-reactive protein Urea and electrolytes, liver function tests - Culture of blood, throat swab, urine, stool for bacteria - Rapid antigen test for meningitis organisms (can be done on blood, CSF, or urine) - LP to send CSF fluid (see meningitis for CSF results) - Throat swab for viral PCR testing - LP is contraindicated if active seizures, hemodynamically unstable - Brain MRI after the LP - HIV testing is also recommended in all patients with encephalitis

Answer 460

- High-dose aciclovir - Proven or high-suspicion of HSV encephalitis = IV aciclovir for 3 weeks

Answer 461

Toxin-producing S. aureus and group A streptococci can cause this rare syndrome, the toxin can be released from infection at any site (e.g. abrasions, burns). Toxin acts as a superantigen. - Fever over39°C - Hypotension - Dffuse erythematous,macular rash.

Answer 462

- Characterised by - Fever > 39 - Hypotension - Diffuse erythematous, macular rash Causes organ dysfunction - Mucositis: Conjunctivae, oral mucosa, genital mucosa - Gastrointestinal: Vomiting/Diarrhoea - Renal impairment - Liver impairment - Clotting abnormalities and thrombocytopenia

Answer 463

- Intensive care support for shock - Debridement of infected areas (removal of infected tissue) - Antibiotics such as ceftriaxone with clindamycin to stop toxin production - After 1-2 weeks, desquamation of the palms, soles, fingers and toes - Risk of recurrent skin and soft tissue infections

Answer 464

- Cause: group A streptococcal infection - Most common in children 5–12 years. - Fever usually precedes headache and tonsillitis by 2–3 days. - Classic ‘sandpaper-like’ maculopapular rash that starts on chest or abdomen and spreads, with flushed cheeks and perioral sparing. - White-coated, sore or swollen tongue - Only bacterial cause of childhood exanthema - Mx Antibiotics (penicillin V or erythromycin) to prevent complications including acute glomerulonephritis or, very rarely in high-income countries, rheumatic fever.

Answer 465

A rare, severe subcutaneous infection, often involving the skin down to fascia and muscle. The affected area may enlarge rapidly, leaving poorly perfused necrotic tissue, usually at the centre. Accompanied by severe pain and systemic illness, which usually requires intensive care.

Answer 466

A strain of S.aureus or group A streptococcus that produces the Panton-Valentine leukocidin (PVL) toxin

Answer 467

Urgent surgical debridement of necrotic tissue to stop infection from spreading, then IV antibiotics and IV immunoglobulin might be given

Answer 468

8 weeks: - 6 in 1 vaccine (diphtheria, tetanus, pertussis, polio, haemophilus influenzae type B (Hib) and hepatitis B) - Meningococcal type B - Rotavirus (oral vaccine) 12 weeks: - 6 in 1 vaccine booster - Pneumococcal (13 different serotypes) - Rotavirus booster 16 weeks: - 6 in 1 vaccine booster - Meningococcal type B booster 1 year: - 2 in 1 HiB/MenC (no longer given 2024) - MMR vaccine (measles, mumps and rubella) first dose - Pneumococcal booster - Meningococcal type B booster Yearly from age 2 – 15 - Influenza vaccine (nasal vaccine) - if eligible 3 years 4 months: - Pre-school booster of 4 in 1 (diphtheria, tetanus, pertussis and polio) - MMR vaccine booster 12 – 13 years: - Human papillomavirus (HPV) vaccine (2 doses given 6 to 24 months apart) 14 years: Teen booster of 3 in 1 (tetanus, diphtheria and polio) - Meningococcal groups A, C, W and Y

Answer 469

Human Parvovirus B19 - Asymptomatic - Erythema infectiosum – most common with fever, malaise, headache, and myalgia followed by a characteristic rash on the face (slapped-cheek) a week later, progressing to a maculopapular, reticular (lace-like) rash on the trunk and limbs - Aplastic crisis – the most serious consequence of HPV-B19 infection, in children with sickle cell disease or thalassaemia); and in immunocompromised children - Fetal disease – transmission of maternal HPV-B19 infection may lead to fetal hydrops and death due to severe anaemia, but most affected fetuses recover Supportive mangement

Answer 470

Allergy is an umbrella term for hypersensitivity of the immune system to allergens. Allergens are proteins (antigens) the immune system recognises as foreign and potentially harmful.

Answer 471

A predisposition to having hypersensitivity reactions to allergens. Atopic conditions: eczema, asthma, hayfever, allergic rhinitis and food allergies. Runs in families

Answer 472

This leading theory suggests there are two main contributors to a child developing an allergy to a food: - Break in skin barrier (infection or eczema) that allows allergens e.g. peanut protein to enter and react with the immune system - The child's GI tract has not previously been exposed to the allergen, and the immune system recognises the allergen as foreign and harmful. - The immune system becomes sensitised to that allergen, and the next time it is exposed to the allergen, a full immune response is mounted If the baby, after weaning after 6m, is regularly exposed to the antigen via the GI tract, then the immune system will not recognise it as harmful So the theory is that regular exposure to an allergen through food and preventing exposure to that allergen through the skin barrier can help prevent food allergies from developing.

Answer 473

Detailed history is the most important aspect of diagnosis - can diagnose with history alone sometimes: Most important areas: - Timing after exposure to the allergen - Previous and subsequent exposure and reaction to the allergen - Symptoms of rash, swelling, breathing difficulty, wheeze and cough - Previous personal and family history of atopic conditions and allergies

Answer 474

The most reliable information about whether a patient has an allergy is a clear and detailed history. There are three main ways to test for allergy: - Skin prick testing - RAST testing - which involves blood tests for total and allergen-specific immunoglobulin E (IgE) - Both test sensitisation, not allergy so unreliable - Gold standard: food challenge testing - the child is given increasing amounts of the allergen under close monitoring - Patch Testing - useful for allergic contact dermatitis e.g. latex

Answer 475

- Avoidance of allergen - Avoiding triggering foods - House dust mites - regular hoovering and changing sheets and pillows in patients that are allergic to house dust mites - Staying indoors when the pollen count is high - Prophylactic antihistamines - Anaphylactic reactions - adrenalin auto-injector - Sometimes, specialist centres will gradually expose patients to allergens over months, called immunotherapy to try reduce their reaction Following Exposure Treatment of allergic reactions are with: Antihistamines (e.g. cetirizine) Steroids (e.g. oral prednisolone, topical hydrocortisone or IV hydrocortisone) Intramuscular adrenalin in anaphylaxis Antihistamines and steroids work by dampening the immune response to allergens. Close monitoring is essential after an allergic reaction to ensure it does not progress to anaphylaxis.

Answer 476

A chronic atopic condition caused by defects in the normal continuity of the skin barrier, leading to inflammation in the skin. Genetic component, runs in families, an atopic condition Mild (occasional mild patches responding well to emollients) to severe (large patches of skin affected - strong topical steroids or systemic treatments)

Answer 477

Infants - dry, red, itchy and sore patches of skin over the flexor surfaces (the inside of elbows and knees) and on the face and neck. Patients with eczema experience periods where the condition is well controlled and flares.

Answer 478

Caused by defects in the skin barrier. Tiny gaps in the skin barrier provide an entrance for irritants, microbes and allergens that create an immune response, resulting in inflammation and the associated symptoms.

Answer 479

- Emollients - maintenance therapy to create an artificial barrier over the skin, can be used instead of soaps and washes that disrupt skin barrier Avoid environmental triggers: e.g. some washing powders, cleaning products, emotional events or stress - Flares - treated with thicker emollients, topical steroids, “wet wraps” (covering affected areas in emollients and sealing with a wrap O/N) The steroid ladder from weakest to most potent: - Mild: Hydrocortisone 0.5%, 1% and 2.5% - Moderate: Eumovate (clobetasone butyrate 0.05%) - Potent: Betnovate (betamethasone 0.1%) - Very potent: Dermovate (clobetasol propionate 0.05%)

Answer 480

- Thinning of the skin - Telangiectasia - enlarged capillaries under the skin - Systemic absorption of steroid - Extra caution with thin skins e.g. eyes and face, best to avoid these areas in children

Answer 481

Bacterial infection by staphylococcus aureus, treated with flucloxacillin Severe = admission and IV antibiotics. Eczema Herpeticum - herpes simplex virus (HSV) or varicella zoster virus (VZV) infection - treat with oral or IV aciclovir

Answer 482

A condition caused by an IgE-mediated type 1 hypersensitivity reaction. Environmental allergens cause an allergic inflammatory response in the nasal mucosa. - Seasonal - e.g. hay fever Perennial (year-round), house dust mite allergy - Occupational

Answer 483

- Runny, blocked and itchy nose - Sneezing - Itchy, red and swollen eyes - Part of atopy Diagnosis is usually made based on the history. Skin prick testing can be useful, particularly testing for pollen, animals and house dust mite a

Answer 484

- Detailed history - Skin prick testing particularly for pollen, animals, house dust mites allergy

Answer 485

- Avoid the trigger. - Hoovering, good home ventilation and changing pillows - Staying indoors during high pollen count Oral antihistamines are taken prior to exposure to reduce allergic symptoms: - Non-sedating antihistamines include cetirizine, loratadine and fexofenadine - Sedating antihistamines include chlorphenamine (Piriton) and promethazine - Nasal antihistamines - good for rapid response - Referral to an immunologist if ineffective

Answer 486

- Puberty starts age 8 – 14 in girls - Lasts about 4 years - In girls, breast buds mark the start of puberty, then pubic hair and growth spurt, and then menstrual periods about 2 years from the start of puberty.

Answer 487

- Begins around 9 – 15 - In boys, puberty starts with enlargement of the testicles, then of the penis, gradual darkening of the scrotum, development of pubic hair, growth spurt (18m after testicle enlargement) and deepening of the voice. - In girls and boys, there is development of acne, axillary hair, body odour, and mood changes

Answer 488

- Hypogonadism - Hypogonadotropic hypogonadism - Hypogonadotropic hypogonadism

Answer 489

A lack of oestrogen and testosterone, that normally rise before and during puberty. This causes: - Hypogonadotropic hypogonadism: a deficiency of LH and FSH so the gonads do not produce oestrogen and testosterone - Hypergonadotropic hypogonadism: a lack of response to LH and FSH by the gonads

Answer 490

- Previous damage to the hypothalamus or pituitary, e.g. radiotherapy or surgery - Growth hormone deficiency - Hypothyroidism - CF or IBD can temporarily delay puberty - Consistutential delay - without underlying cause

Answer 491

Result of abnormal functioning of the gonads. This could be due to: - Previous damage to the gonads (e.g. testicular torsion, cancer) - Congenital absence of the testes or ovaries - Klinefelter's Syndrome (XXY) - Turner’s Syndrome (XO)

Answer 492

Investigations if no evidence of puberty in a girl aged 13 or a boy aged 14. - Detailed history - general health, development, family history, diet and lifestyle. - Examination - height, weight, stage of pubertal development and features of underlying conditions. - FBC for anaemia - U&E for CKD - Anti-TTG or anti-EMA antibodies Hormonal blood tests: - Early morning serum FSH and LH (the gonadotropins). - TFT - Genetic testing with microarray

Answer 493

- Treat underlying condition - Patients with constitutional delay - reassurance and observation - Replacement sex hormones under expert guidance

Answer 494

- Most common - physiologic anaemia of infancy - natural dip in Hb between 6 - 9 weeks of age - Anaemia of prematurity - Blood loss - Haemolysis

Answer 495

Premature neonates are much more likely to become significantly anaemic during the first few weeks of life compared with term infants. Causes - Less time in utero receiving maternal iron - Rapid growth outpaces RBC production in first few weeks - Reduced erythropoietin levels

Answer 496

Haemolytic disease of the newborn occurs when a rhesus D antigen-negative mother's immune system mounts a response against a rhesus D-positive fetus This occurs through sensitisation events where the maternal and fetal blood mixes Usually no problems during first pregnancy The mother's anti-D antibodies can cross the placenta into the fetus during subsequent pregnancies, and cause a rhesus-positive fetus' own immune system to attack its own RBC - causing haemolysis, anaemia and high bilirubin levels.

Answer 497

- Microcytic anaemia (small RBCs) - Normocytic anaemia (normal-sized RBCs) - Macrocytic anaemia (large RBCs)

Answer 498

TAILS. T – Thalassaemia A – Anaemia of chronic disease I – Iron deficiency anaemia L – Lead poisoning S – Sideroblastic anaemia

Answer 499

AAAHH A – Acute blood loss A – Anaemia of Chronic Disease A – Aplastic Anaemia H – Haemolytic Anaemia H – Hypothyroidism

Answer 500

Megaloblastic - impaired DNA synthesis = abnormally large cell, due to a vitamin deficiency: - B12 deficiency - Folate deficiency Normoblastic macrocytic anaemia: - Alcohol - Reticulocytosis (usually from haemolytic anaemia or blood loss) - Hypothyroidism - Liver disease

Answer 501

General - Tiredness - Shortness of breath - Headaches - Dizziness - Palpitations Iron deficiency anaemia: - Pica - Hair loss

Answer 502

Generic signs of anaemia: Pale skin Conjunctival pallor Tachycardia Raised respiratory rate Signs of specific causes of anaemia: Iron deficiency: - Koilonychia - Atrophic glossitis - Brittle hair and nails Haemolytic anaemia - jaundice Thalassamia - bone deformities

Answer 503

- Full blood count for haemoglobin and MCV - Blood film - Reticulocyte count - Ferritin (low iron deficiency) - B12 and folate - Bilirubin (raised in haemolysis) - Haemoglobin electrophoresis (haemoglobinopathies)

Answer 504

The bone marrow need iron to produce haemoglobin - Dietary insufficiency - Loss of iron, e.g. heavy menstruation - Inadequate iron absorption, e.g. IBD

Answer 505

Iron travels bound to transferrin in blood. Total iron binding capacity (TIBC) - level of transferrin - Both TIBC and transferrin levels increase in iron deficiency and decrease in iron overload. Transferrin Saturation (%) = Serum Iron / Total Iron Binding Capacity Ferritin = form of iron in cells - Low ferritin = IDA - Normal ferritin can still mean IDA as extra ferritin released during inflammation, infection or cancer

Answer 506

Normal ranges below - Serum Ferritin: 12 – 200 ug/L - Serum Iron: 14 – 31 μmol/L (not useful) - Total Iron Binding Capacity: 54 – 75 μmol/L

Answer 507

Dietary supplement with dietician guidance - Ferrous sulphate or ferrous fumarate - Blood transfusions - rarely needed

Answer 508

Nonglomerular - Infection (bacterial, viral, tuberculosis, schistosomiasis) - Kidneys stones - Sickle cell disease Glomerular - Acute glomerulonephritis (usually with proteinuria) - Chronic glomerulonephritis (usually with proteinuria) - IgA nephropathy

Answer 509

A height > 2 standard deviations below the average for their age and sex. This is the same as being below the 2nd centile.

Answer 510

Boys: (mother's height + father's height + 14cm) / 2 Girls: (mother's height + father's height – 14cm) / 2

Answer 511

- Familial short stature - Constitutional delay in growth and development - Malnutrition - Chronic diseases, such as coeliac disease, inflammatory bowel disease or congenital heart disease - Endocrine disorders, such as hypothyroidism - Genetic conditions, such as Down syndrome

Answer 512

Variation of normal development. Short stature in childhood but normal height in adulthood Key feature - delayed bone age, assessed by x-ray of wrist and hand Diagnosis = history and examination, supported by x-ray findings Management = exclude other causes, reassurance and monitoring growth over time

Answer 513

The development of secondary sexual characteristics before 8 y/o in females and 9 y/o in males in the UK.

Answer 514

- Precocious puberty - Premature breast development (thelarche) - Premature pubic hair development (pubarche) - Isolated premature menarche.

Answer 515

Gonadotropin-dependent - more common in females due to the ovaries being very sensitive to gonadotropins. Familial or idiopathic Gonadotropin-independent e.g. adrenal tumours - more seen in males as the testes are relatively insensitive to gonadotropin e.g.

Answer 516

- Treat underlying cause e.g. adrenal tumour - Reducing the rate of skeletal maturation, which is assessed by bone age. - Addressing psychological/behavioural difficulties associated with early progression through puberty. - Delayof menarche - gonadotrophin-releasing hormone analogue (stimulates LH and FSH) - Gonadotropin-independent - source of sex steroid identified and treated with inhibitors (e.g. medroxyprogesterone acetate, testolactone)

Answer 517

The testes develop in the abdomen and then gradually migrate down, through the inguinal canal and into the scrotum. Testes develop in the abdomen > inguinal canal > scrotum prior to birth However, 5% still in abdomen - undescended testes (cryptorchidism). Might be palpable in the inguinal canal Risks in older children or after puberty - higher risk of testicular torsion, infertility and testicular cancer.

Answer 518

- Family history - Low birth weight - Small for gestational age - Prematurity - Maternal smoking during pregnancy

Answer 519

Watch and wait in newborns, testes will descend in the first 3 – 6 months most of the time If not, then refer to paediatric urologist. Orchidopexy (surgical correction of undescended testes) between 6 and 12 months of age.

Answer 520

This is a normal response where prepubescent boys' testes to move out of the scrotum into the inguinal canal when it is cold or cremasteric reflex is activated. It should resolve by puberty, if not then correction with orchidopexy.

Answer 521

- Height - Weight - Head circumference - BMI Plot on growth chat - common exam question! Age on x-axis and weight, height and head circumference on the y-axis (separate charts).

Answer 522

Centiles (cent– meaning hundred) on the growth chart indicate where a child’s growth compares to the normal distribution for their age and sex. If a child is not maintaining their centile, that is concerning e.g. a girl on the 91st centile dropping to 9th centile

Answer 523

- Fetal - most rapid - First 2 years (infantile): rapid growth driven by nutritional factors - From 2 years to puberty (childhood phase): steady slow growth During puberty (growth spurt): rapid growth spurt driven by sex hormones

Answer 524

When a newborn takes its first breath, this expands the alveoli, decreasing the pulmonary vascular resistance > pressure drop in right atrium. Left atrial pressure now exceeds right atrial pressure, which compresses the atrial septum and causes functional closure of the foramen ovale (fossa ovalis) Increased blood oxygenation causes a drop in circulating prostaglandins > closure of the ductus arteriosus (ligamentum arteriosum)

Answer 525

- Natural food for babies during first 6 months - Promotes bonding between mother and baby - Mother: reduces risk of breast and ovarian cancer and type 2 diabetes. - Colostrum - even higher in protein and antibodies - Lowers risk of GI, ear and low resp infections - Lowers risk of diabetes, hypertension and obesity later in life

Answer 526

By day 5 Breastfed = 10% Formula-fed = 5% However, if continued weight loss or failure to regain weight by two weeks = admission and assessment for possible causes Most common cause is dehydration due to under-feeding

Answer 527

The gradual transition from milk to normal food. - Starts around 6 months of age. - Pureed fruit, root vegetables and “baby rice” - No honey until 1yo due to infantile botulism - Over 6 months, this will gradually resemble the diet of an older child, supplemented with snacks and milk

Answer 528

- 6 weeks: Smiles - 3 months: Communicates pleasure - 6 months: Curious and engaged with people - 9 months: They become cautious and apprehensive with strangers - 12 months: Engages with others by pointing and handing objects. Waves bye bye. Claps hands. - 18 months: Imitates activities such as using a phone - 2 years: Extends interest to others beyond parents, such as waving to strangers. Plays next to but not necessarily with other children (parallel play). Usually dry by day. - 3 years: They will seek out other children and plays with them. Bowel control. - 4 years: Has best friend. Dry by night. Dresses self. Imaginative play.

Answer 529

Birth - Aware of light - May fix and follow horizontally a face or large coloured toy 6–8 weeks - Face fixation and following large coloured toy 6 months - Fixates 2.5-cm brick - Visually directed reach - Responds to preferential looking tests of acuity (e.g. Keeler or Teller cards) 12 months - Fixates 1mm crumb 1–2 years - Preferential looking tests of acuity (e.g. Cardiff cards) 2–3 years - Names or matches pictures in linear array (e.g. Kay pictures or Lea symbols). Distant and near 3years+ - Names or matches letters (e.g. Sonksen logMAR, or logMAR crowded). Distant and near

Answer 530

Visual acuity is low at birth - light sources, faces and areas of high contrast, and large targets from 30cm away. But gradually increases to normal adult levels by about 5 years of age. Vision screening is performed at school entry or in preschool children

Answer 531

- Health professionals (paediatrician, physiotherapist, occupational therapist, speech and language therapist, clinical psychologist, specialist health visitor, dietician) and often a social worker

Answer 532

- Diabetes - Hyperthyroidism - Systemic lupus erythematosus

Answer 533

- Genetic malformations - IUGR - Macrosomia Neonatal: - Hypoglycemia - Respiratory distress - HCM (Lily disease)

Answer 534

Transient hypoglycaemia is common during the 1st day of life Easily prevented by early feeding Monitor the infant's blood glucose for the first 24 hours and treat hypoglycaemia

Answer 535

Maternal Graves' disease = 1-2% chance that baby will be hyperthyroid as well because TSH-receptor antibody crosses the placenta and stimulates fetal thyroid hormone production. Fetal tachycardia on CTG Neonatal weight loss, irritability, heart failure, diarrhoea in first 2 weeks Anti-thyroid drugs until condition resolves

Answer 536

SLE with Ro (SS-A) or La (SS-B) - neonatal lupus syndrome with self-limiting rash, and rarely heart block SLE with antiphospholipid syndrome (causes hypercoagulable state) is associated with recurrent miscarriage, IUGR, pre-eclampsia, placental abruption and preterm delivery.

Answer 537

The APGAR score measures a newborn's vitals to see if extra medical care is needed There are 5 components to the APGAR score: Appearance, Pulse, Grimace, Activity, Respiration. **Appearance** 2 is for a pink baby 1 if the baby is blue peripherally 0 if the baby is blue all over **Pulse:** 2 for >100 beats per minute 1 for <100 beats per minute 0 for a non-detectable heart rate **Grimace:** relates to the response to stimulation 2 for crying on stimulation 1 for a grimace 0 for no response **Activity:** 2 for flexed limbs that resists extension 1 for some flexion 0 for a floppy baby **Respiration:** 2 for a strong cry 1 for a weak cry 0 for no respiratory effort NICE advise that newborns are assessed at 1 and 5 minutes.

Answer 538

- Birth - delay cord clamping if possible - Start clock/note time , dry/wrap, stimulate, keep warm (heat lamp + plastic wrap if preterm) - Assess colour , tone, breathing, HR - Ensure open airway - If gasping / not breathing 1. Give 5 inflations (30 cm H2O) – start in air 2. Apply PEEP (positive-end expiratory pressure) 5–6 cm H2O, if possible 3. Apply SpO2 +/- ECG - Reassess: if no increase in HR, look for chest movement If the chest is not moving - Check mask, head and jaw position - 2 person support - Consider suction, laryngeal mask/tracheal tube - Repeat inflation breaths - Consider increasing the inflation pressure - Reassess: if no increase in HR, look for chest movement - Once chest is moving, continue ventilation breaths - If heart rate is not detectable or < 60 min-1 after 30 seconds of ventilation: 1. Synchronise 3 chest compressions to 1 ventilation 2. Increase oxygen to 100% 3. Consider intubation if not already done or laryngeal mask if not possible - Reassess heart rate and chest movement every 30 seconds - If the heart rate remains not detectable or < 60/min: - Vascular access and drugs - Consider other factors e.g. pneumothorax, hypovolaemia, congenital abnormality - Update parents and debrief team and complete records

Answer 539

- Birth: Dry the baby, remove any wet towels and cover and start the clock or note the time. - Within 30 seconds: Assess tone, breathing and heart rate. - Within 60 seconds: If gasping or not breathing - open the airway and give 5 inflation breaths - Re-assess: If no increase in heart rate look for chest movement - If chest not moving: Recheck head position, consider 2-person airway control and other airway manoeuvres, repeat inflation breaths and look for a response. - If no increase in heart rate look for chest movement - When the chest is moving: If heart rate is not detectable or slow (< 60 min-1) - start chest compressions with 3 compressions to each breath. - Reassess heart rate every 30 seconds. If heart rate is not detectable or slow (<60 beats per minute) consider venous access and drugs

Answer 540

Prolonged hypoxia leads to hypoxic-ischaemic encephalopathy (HIE) and potentially long-term consequences of cerebral palsy

Answer 541

- Screening tool to pick up abnormalities in newborns - Performed 72 hours after birth and repeated at 6 - 8 weeks at the GP

Answer 542

- Oxygen sats - pre-ductal (right-hand) and post-ductal (feet) O2 sats (≤2% difference) - General appearance: colour, tone, cry - Head - **Eyes** - red reflex absent in congenital cataracts and retinoblastoma - Shoulder and arm - **Chest/heart** - murmurs, side of heart - Abdomen - **Testes** - palpate to check if descended - **Legs/hips** - Developmental dysplasia of the hip, Barlow's and Ortolani's manoeuvre - Back - Reflexes - moro, suckling, rooting, grasp, stepping

Answer 543

1. Airway, breathing: If respiratory distress then: - Clear the airway - Oxygen - CPAP - Mechanical ventilation 2. Monitoring: - O2 sats (91 - 95%) - HR - RR - Temperature - BP - BM - Blood gases - Weight 3. Temperature control: - Plastic wrap if extremely preterm - Radiant warmer 4. Lines: - Peripheral intravenous line: for IV fluids, antibiotics, and other drugs. - Arterial line: if frequent ABG, blood pressure - PIC line for parenteral nutrition 5. Investigations: - haemoglobin, neutrophil, platelet count - blood urea, creatinine, electrolytes, and lactate - Blood culture - Blood glucose - CRP 6. Antibiotics - Broad-spectrum 7. Minimal handling 8. Keep parents informed and allow them to see and touch their baby

Answer 544

1st day: 60–90 ml/kg - Increase by 20–30 ml/kg per day to 150–180 ml/kg per day by about day 5 of life

Answer 545

To provide support and education for the patient, couples and families affected by a genetic condition - Primary goal - provide info to allow for greater autonomy and choice in reproductive decisions and other areas of personal life. Includes: - Listening to questions and concerns of the patient and family - Establishing correct diagnosis - Risk estimation - by providing pedigree info, drawing a pedigree of three generations = essential part of a clinical genetic assessment. - Communication - info presented in an understandable and unbiased way, written info and diagrams helpful to refer back to later - Discussion of management and prevention e.g. reproductive options if risk to offspring e.g. not having any more children, sperm donor or IVF - Signposting to support e.g. charity Unique - Counselling should be non-directive but aid in decision-making

Answer 546

It depends on gestational age 23 - 25 weeks of gestation might encounter many problems and require many weeks of intensive hospital care, and have a high overall mortality. Prognodid after 30 weeks = excellent with modern neonatal care

Answer 547

1st day - about 60–90 ml/kg is usually required, which increases by 20–30 ml/kg per day to 150–180 ml/kg per day by about day 5 of life

Answer 548

- Need for resuscitation and stabilisation at birth Respiratory: – Respiratory distress syndrome – Pneumothorax – Apnoea and bradycardia Metabolic: – hypoglycaemia – hypocalcaemia – electrolyte imbalance – osteopenia of prematurity Others - Hypotension - Patent ductus arteriosus - Temperature control - Nutrition - Infection - Jaundice - Necrotizing enterocolitis - Retinopathy of prematurity - Anaemia of prematurity

Answer 549

This is caused by the lack of surfactant, a phospholipid and protein mixture that lowers surface tension. This causes widespread alveolar collapse and inadequate gaseous exchange Common in preterm infants ≤ 28 weeks

Answer 550

- Tachypnoea over 60 breaths/minute - Laboured breathing with chest wall recession (particularly sternal and subcostal indrawing) and nasal flaring - Expiratory grunting - Cyanosis if severe

Answer 551

"ground-glass" appearance with an indistinct heart border

Answer 552

- Ambient oxygen - Surfactant therapy by providing surfactant into lungs via tracheal tube or catheter - CPAP, high-flow nasal cannula therapy or mechanical ventilation via a tracheal tube.

Answer 553

Common in very low birthweight under 32 weeks Bradycardia caused by the infant not breathing for 20 - 30s, usually caused by underdeveloped central respiratory control Gentle physical stimulation to start breathing again. Treatment: respiratory stimulant caffeine, CPAP or mechanical ventilation might be needed

Answer 554

- Large surface area to volume ratio - Their skin is thin and heat permeable so transepidermal water loss in 1st week of life - Little subcutaneous fat for insulation

Answer 555

Convection: - Raise temperature of ambient air in incubator - Warm clothes Radiation: - Cover baby - Double walls for incubators Evaporation: Dry and wrap at birth; if extremely preterm, place baby’s body directly into plastic bag at birth without drying Conduction: - Nurse on heated mattress

Answer 556

Ductus arteriosus is common in preterm infants It shunts blood from left to right side of circulation Asymptomatic or apnoea and bradycardia Bounding pulse + systolic murmur

Answer 557

If symptomatic, pharmacological closure with prostaglandin synthetase inhibitor or ibuprofen

Answer 558

High nutritional requirements due to rapid growth Orogastric or nasogastric tube if <35-36 weeks as start sucking and swallowing around then Breast milk +/- phosphate (prevent osteopenia), protein or calories Iron supplements

Answer 559

Most common surgical emergency in neonates Inflammatory disease that can lead to bowel necrosis, multi-organ failure

Answer 560

- Prematurity (especially < 32 weeks) - Feeding cow's milk formula (breastfeeding is protective)

Answer 561

- Feed intolerance - Vomiting (might be bile stained) - Abdomen distention - Fresh blood in stool - Later shock

Answer 562

- Distended bowel loops - Thickening of bowel walls with intramural gas - Pneumatosis intestinalis is gas in the bowel wall - Gas in the portal veins - Late stage: bowel perforation (indicated by pneumoperitoneum - free gas in the peritoneal cavity)

Answer 563

- Stop oral feeding - Broad-spectrum antibiotics to cover aerobic and anaerobic - Parenteral nutrition - Ventilatory support - Once stablised, immediate transfer to neonatal surgerical team for surgery to remove dead bowel tissue

Answer 564

- Cerebral palsy (5 - 10%) - Learning difficulties Difficulties with: - fine motor skills, e.g. threading beads - concentration - behaviour problems, especially attention deficit disorders

Answer 565

- Physiological release of haemoglobin from haemolysis due to high haemoglobin concentration at birth - Newborn RBC lifespan = 70 days (adults 120 days) - Hepatic bilirubin metabolism = less efficient

Answer 566

- Haemolytic anaemia - Infection - Inborn errors of metabolism - Liver disease

Answer 567

≥ 80 µmol/l

Answer 568

Urgent! Neonatal sepsis most common cause

Answer 569

- Physiological jaundice - Breast milk jaundice (unconjugated hyperbilirubinemia) - Infection e.g. UTI

Answer 570

Unconjugated - Breast milk jaundice (can last up to 12 weeks) - Infection (particularly UTI) - Gastrointestinal obstruction (e.g. pyloric stenosis Conjugated - Bile duct obstruction - Neonatal jaundice

Answer 571

- FBC and blood film for polycythaemia or anaemia - Conjugated bilirubin: elevated levels indicate a hepatobiliary cause - Blood type testing of mother and baby for ABO or rhesus incompatibility - Direct Coombs Test (direct antiglobulin test) for haemolysis - Thyroid function, particularly for hypothyroid - Blood and urine cultures if infection is suspected. Suspected sepsis needs treatment with antibiotics.

Answer 572

Total bilirubin levels monitored and plotted on treatment threshold charts. Specific to gestational age of the baby at birth. Age = x-axis Total bilirubin level = y-axis If bilirubin reaches threshold, then treatment to lower levels TOM TIP: potential exam question to plot or interpret Treatment 1st line: phototherapy Extremely high levels = exchange transfusions, replacing neonatal blood with donor blood

Answer 573

Early onset (< 48hrs after birth) sepsis Late onset (> 48 hrs after birth) sepsis

Answer 574

Bacteria have ascended from the birth canal and invaded the amniotic fluid, which secondarily infect the fetus.

Answer 575

- Prolonged/premature rupture of the amniotic membranes - When evidence of chorioamnionitis e.g. maternal fever during labour

Answer 576

- Fever - Reduced tone and activity - Poor feeding - Respiratory distress or apnoea - Vomiting - Tachycardia or bradycardia - Hypoxia - Jaundice within 24 hours - Seizures - Hypoglycaemia Red flags - Confirmed or suspected sepsis in the mother - Signs of shock - Seizures - Term baby needing mechanical ventilation - Respiratory distress starting more than 4 hours after birth

Answer 577

- Septic screen which includes FBC for neutropenia, Blood cultures, CRP and urine sample - - Consider if indicated: lumbar pucture and CXR

Answer 578

- Empirical antibiotics without waiting for results - IV abx (usually benzylpenicillin or amoxicillin) to cover group B streptococci, L. monocytogenes and other gram +ve bacteria - Combined with cover for gram -ve bacteria (aminoglycoside e.g. gentamicin) - If CRP and cultures negative and no clinical features of infection then abx stopped after 36 - 48hours - If positive, then CSF analysis

Answer 579

- Neonate's environment - Hospital acquired infections are a risk in neonatal units - Staff must adhere to effective hand hygiene - NICU = indwelling venous catheters for parenteral nutrition

Answer 580

Staphylococcus epidermidis but there are others

Answer 581

Initial - flucloxacillin and gentamicin covers most gram -ve bacilli and staphylococci

Answer 582

Conditions where there is: - Structural abnormality - Extra abnormal portion - Abnormal number of chromosomes

Answer 583

- Where a portion of the chromosome is missing - Rare, unlike to come across them in med school - E.g. cri du chat, missing portion of chromosome 5

Answer 584

Portion of the chromosome is duplicated The chromosome contain twice the number of copies of that gene E.g. Charcot-Marie-Tooth, caused by duplication of the short arm of chromosome 17

Answer 585

Where one chromosome is directly swapped with the portion of another chromosome Banalaced (reciporcal translocation Unbalanced (nonreciprocal translocation - one portion of the first chromosome attaches to the other without exchange Usually does not lead to specific genetic syndromes, but predisposes the person to conditions e.g. cancer and infertility E.g. "Philadelphia chromosome", reciprocal translocation between 9 and 12 in AML

Answer 586

An extra chromosome - total of 47 instead of 46, three conditions for exams! Patau syndrome - trisomy 13 Edwards syndrome - trisomy 18 Down's syndrome - trisomy 21

Answer 587

When chromosomal abnormality occurs after conception The abnormality occurs in a portion of the cells in the body and not others Leading to different genetic material in different cells in the person's body Each case is unique and effects unpredictable

Answer 588

The most common chronic inflammatory joint disease in children and adolescents in the UK. Defined as persistent joint swelling (> 6 weeks) presenting before 16 years of age in absence of infection or any other defined cause

Answer 589

Systemic JIA Polyarticular JIA Oligoarticular JIA Enthesitis related arthritis Juvenile psoriatic arthritis

Answer 590

AKA Still's disease - a systemic illness that can occur throughout childhood in boys and girls: Typical features: - Subtle salmon-pink rash - High swinging fevers - Joint inflammation and pain - Enlarged lymph nodes - Weight loss - Joint inflammation and pain - Splenomegaly - Muscle pain - Pleuritis and pericarditis Think systemic JIA if a patient presents with a salmon-pink rash, fevers > 5 days and joint pain Key complication: macrophage activation syndrome, acutely unwell child with disseminated intravascular coagulopathy, anaemia, thrombocytopenia, bleeding and non-blanching rash - low ESR

Answer 591

Non-infective - Kawasaki's disease - Systemic JIA (Still's disease) - Leukaemia

Answer 592

Negative antinuclear antibodies and rheumatoid factors Raised inflammatory markers = raised CRP, ESR, platelets an serum ferritin

Answer 593

Idiopathic inflammatory arthritis in 5 joints or more Symmetrical and can affect small joints of the hands and feet and large joints like the hips and knees Minimal systemic symptoms but sometimes mild fever, anaemia and reduced growth Equivalent of rheumatoid arthritis in adults

Answer 594

Most children are negative for rheumatoid factor and are known as "seronegative" If rheumatoid factor positive then "seropositive" - tend to older children and adolescents and disease pattern is similar to RA in adults

Answer 595

Inflammatory arthritis that involves 4 joints or less Usually only affects a single, larger joint e.g. knee or ankle More common in girls < 6 years Usually no systemic symptoms

Answer 596

Anterior uveitis, referral to ophthalmologist for management and follow up

Answer 597

- Inflammatory markers normal or mildly elevated - Antinuclear antibodies often positive - Rheumatoid factor negative

Answer 598

AN enthesis (entheses) is the point where the tendon of a muscle inserts into a bone Enthesitis = inflammation of this insertion Inflammatory arthritis

Answer 599

HLA B27 gene Associated with psoriasis and psoriatic arthritis and IBD

Answer 600

The patient will be tender to localised palpation of the entheses Key areas to elicit tenderness of the entheses: - Interphalangeal joints in the hand - Wrist - Over the greater trochanter on the lateral aspect of the hip - Quadriceps insertion at the anterior superior iliac spine - Quadriceps and patella tendon insertion around the patella - Base of achilles, at the calcaneus - Metatarsal heads on the base of the foot

Answer 601

- Seronegative inflammatory arthritis associated with psoriasis - Symmetrical polyarthritis affecting small joints similar to RA OR - Asymmetrical arthritis affecting the large joints in the lower limbs

Answer 602

- Plaques of psoriasis on the skin - Pitting of the nails (nail pitting) - Onycholysis, separation of the nail from the nail bed - Dactylitis, inflammation of the full finger - Enthesitis, inflammation of the entheses, which are the points of insertion of tendons into bone

Answer 603

- Paediatric rheumatology with specialist MDT - Aim to reduce inflammation within the joints, minimise symptoms and maximise function Medical treatment depends on severity and response: - NSAIDs, such as ibuprofen - Steroids, either oral, intramuscular or intra-articular in oligoarthritis - Disease modifying anti-rheumatic drugs (DMARDs), such as methotrexate, and sulfasalazine - Biologic therapy, such as the tumour necrosis factor inhibitors e.g. infliximab

Answer 604

Intussuspection is when the bowel telescopes in on itself. Presentation: 6 to 18 months, colicky pain and a sausage-shaped mass in RUQ. Blood and mucus in stools (red currant jelly stool" = late feature and might not be present if child brought in early. 1st line treatment= air insufflation. This is the use of per rectal air under radiological guidance to reduce the telescoping of the bowel.

Answer 605

Age; Respiratory rate (breaths per minute) <1 = 30-40 1-2 = 25-35 2-5 = 25-30 5-12 = 20-25 >12 = 15-20

Answer 606

<1 year 110–160 2–5 years 95–150 5–12 years 80–120 >12 years 60–100

Answer 607

Investigations - Induced by hyperventilation (blow on a piece of paper or windmill for 2–3 minutes) - EEG: 3Hz generalized, symmetrical and waves and spike discharges Management - Ethosuximide - Sodium valproate Good prognosis: 90-95% become seizure free in adolescence

Answer 608

- Typically presents in 4 - 12 years old - More common in males Clinical features - Seizures typically occur at night or on waking up - Partial seizures (e.g. paraesthesia affecting the face - unilateral OR abnormal feelings in tongue) - Secondary generalisation may occur (i.e. parents may only report tonic-clonic movements) - Child is otherwise normal Investigations: - EEG: Centrotemporal spikes (sharp waves from Rolandic area of brain) Management - May not need medications - The prognosis is excellent, seizures usually stop by adolescence

Answer 609

- Presents within 4 - 8 months - More common in males - Often associated with a serious underlying condition (birth asphyxia, encephalitis) - Can be idiopathic Clinical features - Sudden muscle stiffening: "head bobbing" or " body crunching" - flexion of the head, trunk and arms followed by extension of the arms - It lasts only 1-2 seconds but may be repeated up to 50 times - Progressive mental handicap Investigations: EEG shows hypsarrhythmia in two-thirds of infants Management: Vigabatrin is now considered first-line therapy (+/- steroids) Poor prognosis: loss of skills, learning disability

Answer 610

- Typical onset is in the teenage years - More common in girls Clinical features - Sudden, shock-like movements of both arms - E.g. throwing drinks or cereal in morning (due to myoclonus - involuntary, uncontrolled muscle contraction) Also can have generalized tonic-clonic seizures Triggers of above seizures: sleep deprivation or drinking too much alcohol (most powerful) Learning not affected Management: Usually good response to Sodium valproate, Levetiracetam or Lamotrigine

Answer 611

It is a highly aggressive, rare type of bone cancer predominantly affecting teenagers and young adults (textbook says more common in younger children) Common sites: pelvis, femur, and tibia. Clinical features: nocturnal bone pain, presence of a mass or swelling, restricted joint mobility, and systemic symptoms Ix: X-ray to visualise bone, bone biopsy Mx: MDT, surgery, chemo and radiotherapy if impossible to resect e.f. pelvis or skeleton

Answer 612

Type of bone cancer, most common in adolescents and younger adults, 10 to 20 years old Common site: femur, tibia, humerus Presentation: persistent bone pain, worse at night Diagnosis: - Urgent direct access x-ray within 48 hours. - Poorly defined bone lesion "fluffy" appearance, with a "sunburst appearance" from a periosteal reaction (bone lining irritation) - CT/MRI for staging Mx: - urgent specialist assessment within 48 hours - Treatment = surgical resection, often with limb amputation - Adjuvant chemo to improve outcome - MDT - paeds oncologists and surgeons, physios, dietitians, prosthetics and orthotics and social services

Answer 613

AKA Chronic Lung Disease of Prematurity When infants have oxygen requirement after postmenstrual age (Gestational age + postnatal age) of 36 weeks.

Answer 614

- Low oxygen saturation - Increased work of breathing - Poor feeding and weight gain - Crackles and wheezes on chest auscultation - Increased susceptibility to infection

Answer 615

CXR - fibrosis and lung collapse, cystic changes and distension of the lungs

Answer 616

Give corticosteroids to mothers showing signs of premature labour at < 36 weeks gestation After birth: - Using CPAP instead of intubation and ventilation when possible - Caffeine to stimulate the respiratory effort - Avoid over-oxygenating with supplementary oxygen

Answer 617

- Formal sleep study to assess baby's O2 sats - Some babies are discharged home on low dose oxygen then weaned off over first year - Respiratory synitical virus protection with monthly palivizumab injections

Answer 618

Meconium is the 1st stool passed by a newborn Meconium aspiration occurs when neonates inhale thick meconium. The asphyxiated neonate might start gasping and aspirate meconium before delivery. Most common in post-term deliveries

Answer 619

Meconium is a lung irritant and causes mechanical obstruction and chemical pneumonitis - Meconium-stained liquor - Respiratory distress at or shortly after birth - If significant = persistent pulmonary hypertension of the newborn (PPHN) = R to L heart shunt = cyanotic

Answer 620

Perinatal Hx and examination CXR - consolidations, patchy infiltrates and hyperinflation ABG - hypoxemia, hypercapnia, respiratory acidosis

Answer 621

Supportive O2 therapy Artificial ventilation is often required COmplication: Persistent pulmonary hypertension of the newborn Mx: - Mechanical ventilation and circulatory support - Inhaled nitric oxide (potent vasodilator) or sildenafil (Viagra) - High-frequency oscillatory ventilation

Answer 622

Maintenance fluid over 24 hours (Holliday-Segar formula) 100ml/kg/day for the first 10kg 50ml/kg/day for the next 10kg of weight 20ml/kg/day for weight over 20kg There is an online calculator

Answer 623

Percentage dehydration = [(well weight (kg) - current weight (kg)/well weight] x 100 Fluid deficient (ml) = percentage dehydration x weight (kg) x 10 Total fluid requirement (ml) = maintenance fluid (ml) + fluid (ml)h

Answer 624

Congenital abnormality where the ball of femur (femoral head) and the socket of the pelvis (acetabulum) do not articulate properly

Answer 625

Barlow's test - checks for posterior dislocation Ortolani's test - checks for relocation on hip abduction

Answer 626

- Female - Firstborn - Family history of DDH - Frank breech presentation

Answer 627

Ultrasound to confirm diagnosis

Answer 628

If < 6 months at presentation = Pavlik harness, it is kept on permanently and keeps the baby's hips flexed and abducted, adjusted as baby grows. Holds femoral head in the correct position to allow the hip socket (acetabulum) to develop a normal shape. Regular reviews and removed when baby's hips are more stable, usually after 6 to 8 weeks. Surgery if harness ineffective or diagnosis after 6 months of age After surgery, a hip spica cast used to immobilise hips