Paediatrics Flashcards

Question

specific symptoms of iron deficiency anaemia

Answer 1

Pica = describes dietary cravings for abnormal things such as dirt Hair loss

Answer 2

Pale skin Conjunctival pallor Tachycardia Raised respiratory rate

Answer 3

Koilonychia refers to spoon shaped nails Angular chelitis Atrophic glossitis is a smooth tongue due to atrophy of the papillae Brittle hair and nails can indicate

Answer 4

bone deformities

Answer 5

Full blood count for haemoglobin and MCV Blood film Reticulocyte count - immature RBCs. High when there's active production of RBCs to replace what's lost e.g. in Haemolysis or blood loss Ferritin (low iron deficiency) B12 and folate Bilirubin (raised in haemolysis) Direct Coombs test (autoimmune haemolytic anaemia) Haemoglobin electrophoresis (haemoglobinopathies)

Answer 6

Anaphylaxis is a life-threatening medical emergency. It is caused by a severe type 1 hypersensitivity reaction. Immunoglobulin E (IgE) stimulates mast cells to rapidly release histamine and other pro-inflammatory chemicals. This is called mast cell degranulation. This causes a rapid onset of symptoms, with airway, breathing and/or circulation compromise.

Answer 7

``` Urticaria Itching Angio-oedema, with swelling around lips and eyes Abdominal pain Shortness of breath Wheeze Swelling of the larynx, causing stridor Tachycardia Lightheadedness Collapse ```

Answer 8

``` call for help - need paediatrician ABCDE approach a - secure airway b - oxygen, salbutamol if wheezing c - IV bolus of fluids d - lie flat for cerebral perfusion e - look for flushing, urticaria and angio-oedema ``` Definitive mx: - IM adrenaline (repeat after 5 mins if needed) - Antihistamines - chlorphenamine or cetirizine - Steroids - IV hydrocortisone Keep in for observation - think biphasic reaction Do serum mast cell tryptase within 6 hours Education and follow up for family BLS for parents Adrenalin auto-injector to take home

Answer 9

a second anaphylactic reaction after successful treatment of the first

Answer 10

Anaphylaxis can be confirmed by measuring the serum mast cell tryptase within 6 hours of the event. Tryptase is released during mast cell degranulation and stays in the blood for 6 hours before gradually disappearing.

Answer 11

all children and adolescents with anaphylactic reactions. considered in children with generalised allergic reactions (without anaphylaxis) with certain risk factors: - Asthma requiring inhaled steroids - Poor access to medical treatment (e.g. rural locations) - Adolescents, who are at higher risk - Nut or insect sting allergies are higher risk - Significant co-morbidities, such as cardiovascular disease

Answer 12

a rapid deterioration in the symptoms of asthma. This could be triggered by any of the typical asthma triggers, such as infection, exercise or cold weather.

Answer 13

Progressively worsening SOB Signs of respiratory distress Fast respiratory rate (tachypnoea) Expiratory wheeze on auscultation heard throughout the chest The chest can sound “tight” on auscultation, with reduced air entry A silent chest is an ominous sign.

Answer 14

Moderate Severe Life threatening

Answer 15

Peak flow >50% predicted | Normal speech

Answer 16

Peak flow 33-50% predicted Saturations <92% Unable to complete sentences in one breath Signs of respiratory distress Respiratory rate >40 in 1-5yo or >30 in over 5 yo HR >140 in 1-5 yo or >125 in over 5 yo

Answer 17

``` Peak flow <33% predicted Saturations <92% Exhaustion and poor respiratory effort Hypotension Silent chest Cyanosis Altered consciousness/confusion ```

Answer 18

O2 Bronchodilators - salbutamol inhaled/neb, ipratropium inhaled/neb, magnesium sulphate IV, aminophylline IV Steroids - oral prednisolone or IV hydrocortisone ABx Intubation and ICU Monitor K+ when using high doses of salbutamol

Answer 19

when they are having 6 puffs of salbutamol every 4 hours - prescribe a reducing regime of salbutamol for home

Answer 20

Reducing regime of salbutamol inhaler Finish steroid course Safety net Asthma action plan

Answer 21

a chronic inflammatory airway disease leading to variable airway obstruction. 1) The smooth muscle in the airways is hypersensitive. 2) The smooth muscle responds to stimuli by constricting and causing airflow obstruction. 3) This bronchoconstriction is reversible with bronchodilators such as inhaled salbutamol.

Answer 22

Episodic symptoms with intermittent exacerbations Diurnal variability, typically worse at night and early morning Dry cough with wheeze and shortness of breath Typical triggers A history of other atopic conditions such as eczema, hayfever and food allergies Family history of asthma or atopy Bilateral widespread “polyphonic” wheeze heard by a healthcare professional Symptoms improve with bronchodilators

Answer 23

``` Dust (house dust mites) Animals Cold air Exercise Smoke Food allergens (e.g. peanuts, shellfish or eggs) ```

Answer 24

Not diagnosed until at least 2-3 years old

Answer 25

Spirometry with reversibility testing (in children aged over 5 years) Direct bronchial challenge test with histamine or methacholine Fractional exhaled nitric oxide (FeNO) Peak flow variability measured by keeping a diary of peak flow measurements several times a day for 2 to 4 weeks

Answer 26

1. SABA - salbutamol 2. + low dose ICS / montelukast 3. + other option from 2 4. refer to specialist

Answer 27

1. SABA - salbutamol 2. + low dose ICS 3. + LABA - salmeterol 4. increase ICS to medium dose. consider adding oral montelukast or oral theophylline 5. increase ICS to high dose 6. refer to specialist

Answer 28

same as adults 1. SABA - salbutamol 2. + low dose ICS 3. + LABA 4. Increased ICS to medium dose. consider Adding oral montelukast, oral theophylline or LAMA (tiotropium) 5. Increased ICS to high dose and combine additional treatments from step 4 6. Oral steroids at lowest possible dose and refer to specialist

Answer 29

inhaled steroids can slightly reduce growth velocity and can cause a small reduction in final adult height of up to 1cm when used long term (for more than 12 months) Is dose dependent Poorly controlled asthma can lead to a more significant impact on growth and development.

Answer 30

chronic atopic condition caused by defects in the normal continuity of the skin barrier, leading to inflammation in the skin

Answer 31

family hx | atopy

Answer 32

Presents in infancy Dry red itchy sore patches of skin over flexor surfaces (inside elbows and knees) and one face/neck Flares Can range from mild to severe

Answer 33

Defects in the skin barrier Gaps in skin barrier allow for entrance of irritants, microbes and allergens that create an immune response = inflammation

Answer 34

Maintenance and flare management Maintenance = 1. Emollients 2. Soap substitutes 3. lifestyle - use emollients often, after washing and before bed. Avoid things that break down skin barrier e.g. hot baths, scratching or scrubbing skin. Don't use soaps and body washes. 4. avoid environmental triggers - changes in temp, dietary products, washing powders, cleaning products and emotional stress Flares 1. thicker emollients 2. topical steroids 3. wet wraps 4. treat bacterial/viral infections

Answer 35

- zinc impregnanted bandages - topical tacrolimus - phototherapy - systemic immunosuppressants - oral corticosteroids - methotrexate - azathioprine

Answer 36

``` E45 Dirpobase cream Oilatum cream Aveeno cream Cetraben cream Epaderm cream ```

Answer 37

``` 50:50 ointment (50% liquid paraffin) Hydromol ointment Diprobase ointment Cetraben ointment Epaderm ointment ```

Answer 38

(Hug Every Budding Dermatologist) 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 39

Thinning of the skin | Skin more prone to flares, bruising, tearing, stretch marks and telangiectasia

Answer 40

staphylococcus aureus (mx = flucloxacillin)

Answer 41

viral skin infection in patients with eczema caused by the herpes simplex virus (HSV) or varicella zoster virus (VZV). Patients can be very unwell.

Answer 42

``` Eczema + Widespread painful vesicular rash Vesicles contain pus Fever Lethargy Irritability Reduced oral intake Lymphadenopathy ```

Answer 43

Viral swabs/clinical diagnosis

Answer 44

Acyclovir (may need to be IV)

Answer 45

Attention deficit hyperactivity disorder (ADHD) is at the extreme end of “hyperactivity” and inability to concentrate (“attention deficit“). It affects the person’s ability to carry out everyday tasks, develop normal skills and perform well in school.

Answer 46

symptoms must be persistent, across various settings and negatively affecting the child Very short attention span Quickly moving from one activity to another Quickly losing interest in a task and not being able to persist with challenging tasks Constantly moving or fidgeting Impulsive behaviour Disruptive or rule breaking

Answer 47

- parent and child education - Healthy diet and exercise - 10 week watch and wait period - medication (last resort and must be >5yo): central nervous system stimulants e.g. methylphenidate (Ritalin), lisdexamfetamine, dexamfetamine, atomoxetine

Answer 48

Persistent features - 6+ in children under 16yo and 5+ in children over 17 yo Must be an element of developmental delay INATTENTION features: - Doesn't follow through on instructions - Reluctance to engage in mentally intense tasks - Easily distracted - finds it difficult to sustain tasks - finds it difficult to organise tasks or activities - often forgetful in daily activities - often loses things necessary for tasks or activities - often doesn't seem to listen when spoken to directly HYPERACTIVITY/IMPULSIVITY features: - Unable to play quietly - Talks excessively - Doesn't wait their turn easily - Spontaneously leaves their seat - Is often 'on the go' - Often interrupts or intrusive - Will answer prematurely - before a question has been finished - Will run and climb when its not appropriate

Answer 49

Abdominal pain Nausea Dyspepsia

Answer 50

``` Weight and height every 6 months Baseline ECG (its cardio toxic) ```

Answer 51

Neuro-developmental disorder the full range of people affected by a deficit in social interaction, communication and flexible behaviour. The autistic spectrum has a significant range. On one end patients have normal intelligence and ability to function in everyday life but displaying difficulties with reading emotions and responding to others. This was previously known as Asperger syndrome. On the other end, patients can be severely affected and unable to function in normal environments.

Answer 52

Deficits in social interaction, communication and behaviour: ``` SOCIAL INTERACTION - Lack of eye contact Delay in smiling Avoids physical contact Unable to read non-verbal cues Difficulty establishing friendships Not displaying a desire to share attention (i.e. not playing with others) ``` COMMUNICATION - Delay, absence or regression in language development Lack of appropriate non-verbal communication such as smiling, eye contact, responding to others and sharing interest Difficulty with imaginative or imitative behaviour Repetitive use of words or phrases BEHAVIOUR - Greater interest in objects, numbers or patterns than people Stereotypical repetitive movements. There may be self-stimulating movements that are used to comfort themselves, such as hand-flapping or rocking. Intensive and deep interests that are persistent and rigid Repetitive behaviour and fixed routines Anxiety and distress with experiences outside their normal routine Extremely restricted food preferences

Answer 53

Should be made by a specialist Diagnosis can be made before the age of 3 Detailed history of the child's behaviour and communication Assessment in school

Answer 54

MDT: Child psychology and child and adolescent psychiatry (CAMHS) Speech and language specialists Dietician Paediatrician Social workers Specially trained educators and special school environments Charities such as the national autistic society Early educational and behavioural interventions SSRIs - for repetitive stereotyped behaviour, anxiety and aggression Antipsychotic drugs - for aggression and self injury Methylphenidate - for ADHD family support and counselling

Answer 55

ADHD Epilepsy Higher head circumference to brain volume ratio

Answer 56

a congenital condition where a section of the bile duct is either narrowed or absent. This results in cholestasis (bile isn't transported from the liver to bowel). Conjugated bilirubin is usually excreted in the bile therefore biliary atresia prevents the excretion of conjugated bilirubin

Answer 57

Significant jaundice shortly after birth (high conjugated bilirubin levels) Persistent jaundice (>14 days in term babies and >21 days in premature babies) Dark urine and pale stools Apetite and growth disturbance Hepatomegaly & splenomegaly

Answer 58

Conjugated and unconjugated bilirubin - high proportion of conjugated bilirubin US of biliary tree and liver

Answer 59

Surgery = kasai portoenterostomy | attach section of small intestine to opening of liver where bile duct normally attaches

Answer 60

``` pathogenesis is unclear, is a congenital illness contributing factors = - infection - congenital malformations - retained toxins within bile ```

Answer 61

More common in females | Ssen in neonates

Answer 62

Unsuccessful anastomosis Progressive liver disease Cirrhosis with eventual hepatocellular carcinoma

Answer 63

- Prognosis good if surgery successful | - Unsuccessful surgery = liver transplant in first 2 years of life

Answer 64

``` Unresponsive child? Call for help Open airway If not breathing: - 5 rescue breaths - Check for signs of circulation - in infants use brachial or femoral pulse and in children use femoral pulse - Chest compressions - 15 compressions: 2 breaths - Attach ECG monitor/defibrillator ```

Answer 65

Chest compressions should be 100-120 /min Depth of compression = 4cm in infant and 5cm in child (depresses the lower half of the sternum by at least 1/3rd) In children compress the lower half of the sternum In infants use a two thumb encircling technique for chest compression

Answer 66

Measured out of 10 Used to assess the health of a newborn baby 0-3 low score, 4-6 moderate low, 7-10 normal baby 1) Appearance - 0 blue/pale, 1 blue extremities and 2 pink 2) Pulse - 0 absent, 1 <100 and 2 >100 3) Grimace (response to stimulation) - 0 no response, 1 little response, 2 good response 4) Activity (muscle tone) - 0 floppy, 1 flexed arms/legs, 2 active 5) Respiration - 0 absent, 1 slow/irregular, 2 strong/crying

Answer 67

1) Resuscitation fluids - if the child is shocked/haemodynamically compromised 2) Replacement fluids - if the child is dehydrated and is in a fluid deficit e.g. from vomiting/diarrhoea/DKA/burns 3) Maintenance fluids - if there's no dehydration but the child can't meet their fluid requirements enterally

Answer 68

Monitor U&Es and plasma glucose every 24 hours at least

Answer 69

- Appears unwell - Altered responsiveness - Sunken eyes - Tachycardia - Tachypnoea - Reduced skin turgor - Dry mucous membranes - Decreased urine output

Answer 70

100ml/kg/day for first 10kg of weight 50ml/kg/day for next 10kg of weight 20ml/kg/day for weight over 20kg

Answer 71

Holliday-Segar formula

Answer 72

Isotonic crystalloids (0.9% NaCl) + 5% glucose

Answer 73

Birth to day 1 = 50-60ml/kg/day Day 2 = 70-80ml/kg/day Day 3 = 80-100 ml/kg/day Day 4 = 100-120 ml/kg/day Day 5-28 = 120-150 ml/kg/day

Answer 74

Calculate the percentage dehydration: - 5% dehydrated if signs of dehydration but no red flags - 10% dehydrated if signs of shock - Can use formula to calculate it accurately: (well weight - current weight) / well weight x100 Fluid deficit = % dehydration x weight (kg) x 10

Answer 75

Maintenance fluids + fluid deficit

Answer 76

0.9% NaCl 10ml/kg bolus over <10 mins Use smaller bolus if: - Neonatal period - DKA - Septic chock - Trauma - Cardiac pathology

Answer 77

Used to assess if a patient who is younger than 16 is competent to consent to treatment, for example contraception Need to fulfil following: - Understand the professionals advice - Cannot be persuaded to inform their parents or allow the professional to contact the parents on their behalf - They are likely to start or continue having sexual intercourse with or without contraceptives - Unless they receive contraceptives, their physical or mental health is likely to suffer - The young persons best interests requires them to receive contraceptive advice or tx without parental consent

Answer 78

Idiopathic/functional constipation = no underlying cause Secondary constipation

Answer 79

``` Hirschsprungs Cystic fibrosis Hypothyroidism Spinal cord lesions Sexual abuse Intestinal obstruction Anal stenosis Cows milk intolerance ```

Answer 80

Breast fed babies can open their bowels as little as once per week Less than 3 stools a week Hard stools that are difficult to pass Rabbit dropping stools Straining and painful passages of stools Abdominal pain Holding an abnormal posture, referred to as retentive posturing Rectal bleeding associated with hard stools Faecal impaction causing overflow soiling, with incontinence of particularly loose smelly stools Hard stools may be palpable in abdomen Loss of the sensation of the need to open the bowels

Answer 81

Faecal incontinence Not pathological until 4 yo Sign of chronic constipation - rectum becomes stretched and loses sensation

Answer 82

``` Chronic constipation Spina bifida Hirschprung’s disease Cerebral palsy Learning disability Psychosocial stress Abuse ```

Answer 83

``` Habitually not opening the bowels Low fibre diet Poor fluid intake and dehydration Sedentary lifestyle Psychosocial problems such as a difficult home or school environment (always keep safeguarding in mind) ```

Answer 84

Not passing meconium within 48 hours of birth (cystic fibrosis or Hirschsprung’s disease) Neurological signs or symptoms, particularly in the lower limbs (cerebral palsy or spinal cord lesion) Vomiting (intestinal obstruction or Hirschsprung’s disease) Ribbon stool (anal stenosis) Abnormal anus (anal stenosis, inflammatory bowel disease or sexual abuse) Abnormal lower back or buttocks (spina bifida, spinal cord lesion or sacral agenesis) Failure to thrive (coeliac disease, hypothyroidism or safeguarding) Acute severe abdominal pain and bloating (obstruction or intussusception)

Answer 85

correct reversible factors high fibre diet good hydration start laxatives - movicol faecal impaction = disimpaction regime with high dose of laxatives at first praise after visiting toilet, schedule toilet visits, bowel diary and star charts

Answer 86

a congenital condition where nerve cells of the myenteric plexus (Auerbach's plexus) are absent in the distant bowel and rectum. Auerbach's plexus is responsible for stimulating peristalsis of the large bowel -without it the bowel loses its motility and stops being able to pass food along its length

Answer 87

absence of parasympathetic ganglion cells in the Auerbach's plexus at the distal colon and rectum

Answer 88

Total colonic aganglionosis The aganglionic section of the colon doesn't relax = it becomes constricted - loss of movement of faeces and obstruction of bowel

Answer 89

``` Family hx downs syndrome Neurofibromatosis Waardenburg syndrome - pale blue eyes, hearing loss, and patches of white skin and hair Multiple endocrine neoplasia type II ```

Answer 90

``` acute intestinal obstruction after birth delay in passing meconium (>24 hrs) chronic constipation since birth abdominal pain and distention vomiting poor weight gain and failure to thrive ```

Answer 91

Hirschsprung associated enterocolitis

Answer 92

Inflammation and obstruction of the intestine occurring in around 20% of neonates with hirschsprungs disease

Answer 93

``` 2-4 weeks old fever abdominal distention diarrhoea (often bloody) features of sepsis can lead to toxic megacolon and perforation of the bowel ```

Answer 94

abx fluid resuscitation decompression of the obstructed bowel

Answer 95

abdominal xray - look for intestinal obstruction and HAEC Rectal biopsy - for diagnosis. Absence of ganglionic cells on histology

Answer 96

surgical removal of ganglionic section of bowel

Answer 97

acute infective respiratory disease affecting young children. URTI causing oedema in the larynx = stridor

Answer 98

6 months to 2 years (can be in older children)

Answer 99

PARAINFLUENZA VIRUS influenza adenovirus respiratory syncytial virus (RSV) diphtheria - leads to epiglottitis

Answer 100

``` Increased work of breathing “Barking” cough, occurring in clusters of coughing episodes - worse at night Hoarse voice Stridor Low grade fever ```

Answer 101

``` Oral dexamethasone - single dose for all children regardless of severity (can give prednisolone alternatively) Oxygen Nebulised budesonide Nebulised adrenalin Intubation and ventilation ``` Very responsive to steroids children can be cared for at home

Answer 102

more common in autumn

Answer 103

Mild Moderate Severe

Answer 104

Occasional barking cough No audible stridor at rest No or mild suprasternal +/- intercostal recessions Child is happy and eating/playing

Answer 105

``` Frequent barking cough Easily audible stridor at rest suprasternal and sternal wall retraction at rest no or little distress/agitation child can be placated ```

Answer 106

``` frequent barking cough prominent inspiratory stridor at rest marked sternal wall retractions significant distress and agitation or lethargy tachycardia ```

Answer 107

``` if its moderate or severe or: - <6 months old - known upper airway abnormalities - uncertain about diagnosis ```

Answer 108

clinical diagnosis CXR - PA view shows steeple sign (subglottilc narrowing) and lateral view shows thumb sign (epiglottis swelling)

Answer 109

a posterior-anterior view will show subglottic narrowing, commonly called the 'steeple sign' a lateral view in acute epiglottis will show swelling of the epiglottis - the 'thumb sign'

Answer 110

Diabetic ketoacidosis Medical emergency Common way that children with new diagnosis of T1DM present There is extreme hyperglycaemic ketosis, resulting in metabolic acidosis

Answer 111

1) ketoacidosis - ketogenesis occurs (fatty acids converted to ketones when there's insufficient glucose & glycogen stores). Kidneys initially produce bicarb to buffer ketone acids, then can't compensate = ketoacidosis 2) Dehydration - hyperglycaemia overwhelms the kidneys & glucose is filtered into the urine. Glucose draws water out with it = osmotic diuresis = polyuria = dehydration = polydipsia 3) Potassium imbalance = insulin drives K into cells = hyperkalaemia. Total body K is low as no K is stored in cells. When tx with insulin starts = severe hypokalaemia as K is drawn into cells = arrhythmias

Answer 112

cerebral oedema. Dehydration & hyperglycaemia = water moves from intracellular to extracellular space in the brain so cells are dehydrated and shrink. When dehydration & hyperglycaemia is corrected rapidly = rapid shift of water intracellularly in the brain = brain cells swell & brain becomes oedematous Monitor GCS in children with DKA

Answer 113

headaches altered behaviour bradycardia changes to consciousness

Answer 114

slow IV fluids IV mannitol IV hypertonic saline

Answer 115

``` Polyuria Polydipsia Nausea and vomiting Weight loss Acetone smell to their breath Dehydration and subsequent hypotension Altered consciousness Symptoms of an underlying trigger (i.e. sepsis) ```

Answer 116

``` Hyperglycaemia = BM >11 Ketosis = blood ketones >3 Acidosis = ph <7.3 ```

Answer 117

1. Correct dehydration over 48 hrs 2. Fixed rate insulin infusion 3. Avoid fluid boluses 4. Treat underlying triggers 5. Prevent hypoglycaemia with IV dextrose once BM <14 6. + K to IV fluids and monitor serum K 7. Monitor for signs of cerebral oedema 8. Monitor glucose, ketones and pH

Answer 118

a disease where the pancreas stops being able to produce insulin. What causes the pancreas to stop producing insulin is unclear. When the pancreas is not producing insulin, the cells of the body cannot take glucose from the blood and use it for fuel. The cells cannot use glucose, so the level of glucose in the blood keeps rising, causing hyperglycaemia.

Answer 119

4.4 to 6.1

Answer 120

25-50% of new T1DM children present in DKA Polyuria Polydipsia Weight loss Secondary enuresis Recurrent infections

Answer 121

``` FBC U&E Lab glucose Blood cultures HbA1C TFTs Thyroid peroxidase antibodies (TPO for autoimmune thyroid disease) anti-TTG (coeliacs) Insulin antibodies, anti-GAD antibodies and islet cell antibodies (antibodies associated with T1DM) ```

Answer 122

1. Patient and family education 2. Subcut insulin regime 3. Monitor dietary carbohydrate intake 4. Monitor BMs - waking, at each meal and before bed 5. Monitoring and mx for complications

Answer 123

Hypoglycaemia Hyperglycaemia and DKA

Answer 124

``` Too much insulin Not enough carbs Malabsorption Diarrhoea vomiting sepsis ```

Answer 125

``` Hunger tremor sweating irritability dizziness pallor reduced consciousness coma death ```

Answer 126

rapid acting glucose - lucozade slower acting carb - biscuit or toast IV 10% dextrose IM glucagon

Answer 127

Macrovascular complications: - CAD - Stroke - HTN Microvascular complications - Peripheral neuropathy - Retinopathy - Glomerulosclerosis Infection related complications - UTI - Pneumonia - Skin and soft tissue infections particularly in feet - Cadidiasis

Answer 128

HbA1C Capillary blood glucose Flash glucose monitoring (5 minute lag behind BM)

Answer 129

an acquired syndrome characterised by activation of coagulation pathways, resulting in formation of intravascular thrombi and depletion of platelets and coagulation factors Tendency for both bleeding and thrombosis simultaneously

Answer 130

- Infection (sepsis) - Malignancy - Severe burns - Trauma - Shock - Obstetric emergencies - Acute haemolytic transfusion reaction

Answer 131

Activation of the coagulation cascade intravascularly Causes microvascular thrombosis Small thrombi can lead to multi-organ failure At the same time, widespread activation of coagulation leads to reduction in the concentration of circulating coagulation factors = consumptive coagulopathy = risk of bleeding increases = thrombocytopenia Simultaneous bleeding and thrombosis

Answer 132

- Evidence of precipitating factor - bleeding from unusual sites: ears, nose, GI, GU, Respiratory, venipuncture site (bleeding from 3 unrelated sites = highly suggestive) - Widespread unexpected bruising - new confusion or disorientation - petechiae or purpura - Lived reticularis - mottled lace like patterning of the skin - Purpura fulminans: widespread skin necrosis - Localised infarction and gangrene (e.g. of fingers) - Oliguria, hypotension or tachycardia

Answer 133

ISTH scoring system - platelet count low - d dimer. raised - PTT prolonged - fibrinogen levels decreased

Answer 134

treat underlying cause platelet transfusion if bleeding concentrated solutions of clotting factors cryoprecipitate or fibrinogen concentrate for low fibrinogen If thrombosis is prominent factor - LMWH - unfractionated heparin has shorter 1/2 life so good for this

Answer 135

``` multi organ failure life threatening haemorrhage cardiac tamponade (becks triad) Haemothorax Intracranial haemorrhage Gangrene and loss of digits ```

Answer 136

3 copies of chromosome 21 = trisomy 21

Answer 137

``` 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 (gap between upper and lower eyelid) Single palmar crease ```

Answer 138

Learning disability Recurrent otitis media Deafness. Eustachian tube abnormalities lead to glue ear and conductive hearing loss. Visual problems such myopia, strabismus and cataracts Hypothyroidism occurs in 10 – 20% Cardiac defects affect 1 in 3, particularly ASD, VSD, patent ductus arteriosus and tetralogy of Fallot Atlantoaxial instability Leukaemia is more common in children with Down’s Dementia is more common in adults with Down’s Duodenal atresia sub fertility

Answer 139

offered to all women - they can refuse combined test is done between 11 and 14 weeks triple test/quadruple test (just maternal blood tests) done between 14 and 20 weeks

Answer 140

USS - measure nuchal translucency (>6mm = downs) Maternal blood tests - beta-HCG increased and pregnancy associated plasma protein A (PAPPA) is low + mothers age Done at 11-14 weeks

Answer 141

b-HCG (increased) Alpha-fetoprotein (AFP) (low) Serum estriol (low) Done at 14-20 weeks

Answer 142

b-HCG (increased) Alpha-fetoprotein (AFP) (low) Serum estriol (low) inhibin A (high) Done at 14-20 weeks

Answer 143

If the risk of Downs syndrome is greater than 1 in 150, calculated by screening tests

Answer 144

Chorionic villus sampling - US guided biopsy of placental tissue (before 15 weeks) Amniocentesis - US guided aspiration of amniotic fluid using a needle (done later in pregnancy) non-invasive prenatal testing (NIPT) - maternal blood test to detect fetal DNA

Answer 145

miscarriage - 1 in 200 women infection in uterus cramping, spotting or leaking amniotic fluid rh problems

Answer 146

MDT management Regular thyroid checks (2 yearly) Echocardiogram to diagnose cardiac defects Regular audiometry for hearing impairment Regular eye checks

Answer 147

Prognosis varies depending on the severity of the associate complications. The average life expectancy is 60 years.

Answer 148

cramping, bleeding or leaking of amniotic fluid infection miscarriage preterm labour limb defects in infants - if done before 9 weeks

Answer 149

PAPP-A is lower in Edwards and patau PAPP-A = pregnancy associated plasma protein A

Answer 150

Associated with - personality disorders, OCD and anxiety Female Genetic component

Answer 151

the person feel they are overweight despite evidence of normal or low body weight. It involves obsessively restricting calorie intake with the intention of losing weight. Often the person exercises excessively and may use diet pills or laxatives to restrict absorption of food.

Answer 152

``` Excessive weight loss Amenorrhoea Lanugo hair is fine, soft hair across most of the body Hypokalaemia Hypotension Hypothermia Changes in mood, anxiety and depression Solitude ```

Answer 153

Arrhythmias Cardiac atrophy Sudden cardiac death

Answer 154

Unlike with anorexia, people with bulimia often have a normal body weight. Their body weight tends to fluctuate. The condition involves binge eating, followed by “purging” by inducing vomiting or taking laxatives to prevent the calories being absorbed.

Answer 155

Alkalosis, due to vomiting hydrochloric acid from the stomach Hypokalaemia Erosion of teeth Swollen salivary glands Mouth ulcers Gastro-oesophageal reflux and irritation Calluses on the knuckles where they have been scraped across the teeth = Russell’s sign.

Answer 156

Binge eating disorder is characterised by episodes where the person excessively overeats, often as an expression of underlying psychological distress. This is not a restrictive condition like anorexia or bulimia, and patients are likely to be overweight.

Answer 157

``` A planned binge involving “binge foods” Eating very quickly Unrelated to whether they are hungry or not Becoming uncomfortably full Eating in a “dazed state” ```

Answer 158

``` Self help resources Counselling CBT Admission in severe cases for observed refeeding and monitoring of refeeding syndrome SSRI ```

Answer 159

Hypomagnesaemia Hypokalaemia Hypophosphataemia

Answer 160

occurs in people that have been in a severe nutritional deficit for an extended period, when they start to eat again.

Answer 161

BMI <20 Little to eat for past 5 days Long period of malnutrition

Answer 162

Cardiac arrhythmias HF Fluid overload

Answer 163

Metabolism in cells/organs slows dramatically while malnourished When food is introduced again, cells start to process glucose, protein and fats = uses up magnesium, potassium and phosphorus

Answer 164

Slowly reintroduce food with restricted calories Monitor magnesium, potassium and phosphate Monitor glucose Fluid balance monitoring ECG monitoring Supplementation with electrolytes, vitamins (especially B12 and thiamine)

Answer 165

Epiglottitis is inflammation and swelling of the epiglottis haemophilus influenza type B Can completely block airway in hours = life threatening emergency

Answer 166

children who haven't had their vaccines

Answer 167

``` Patient presenting with a sore throat and stridor Drooling Tripod position, sat forward with a hand on each knee High fever Difficulty or painful swallowing Muffled voice Scared and quiet child Septic and unwell appearance ```

Answer 168

lateral xray neck = thumb print sign | don't do if patient is acutely unwell

Answer 169

``` don't distress the child get senior paediatrician and anaesthetist secure airway if necessary IV ceftriaxone Dexamethasone ```

Answer 170

epiglottic abscess

Answer 171

``` LOC tonic (muscle tensing) movements clonic (muscle jerking) movements Typically the tonic phase comes before the clonic phase. tongue biting incontinence groaning irregular breathing Post ictal period ```

Answer 172

``` 1st = sodium valproate 2nd = lamotrigine or carbamazepine ```

Answer 173

start in temporal lobe affects hearing speech memory and emotions present with: - hallucinations - memory flashbacks - deja vu - strange behaviours

Answer 174

(reverse of mx for tonic clonic seizures) 1st = carbamazepine or lamotrigine 2nd = sodium valproate or levetiracetam

Answer 175

typical in children blank, stare into space then abruptly returns to normal unaware during seizure 10-20 seconds most patient grow out of absence seizures

Answer 176

1st = sodium valproate or ethosuximide

Answer 177

drop attacks brief lapse in muscle tone 3 minutes in length begin in childhood

Answer 178

Lennox-Gastaut syndrome.

Answer 179

``` 1st = sodium valproate 2nd = lamotrigine ```

Answer 180

sudden brief muscle contractions sudden jump/shock like seizures remains awake

Answer 181

juvenile myoclonic epilepsy (Janz syndrome)

Answer 182

``` 1st = sodium valproate 2nd = lamotrigine, levetiracetam or topiramate ```

Answer 183

West syndrome

Answer 184

starts at 6 months old clusters of full body spasms 1. Flexion of head, trunk, limbs → extension of arms (Salaam attack); last 1-2 secs, repeat up to 50 times 2. Progressive mental handicap 3. EEG: hypsarrhythmia

Answer 185

1/3 die by 25 yo | 1/3 seizure free

Answer 186

prednisolone | vigabatrin

Answer 187

Febrile convulsions are seizures that occur in children whilst they have a fever. They are not caused by epilepsy or other underlying neurological pathology (such as meningitis or tumours). Occur between 6 months - 5 yo

Answer 188

EEG after 2nd tonic clonic seizure MRI brain if under 2 yo, focal seizures or no response to 1st line anti epileptic ECG Blood electrolytes Blood glucose Blood cultures, urine cultures or lumbar puncture

Answer 189

showers instead of baths cautious when swimming cautious with heights cautious with traffic older teenagers - avoid alcohol

Answer 190

1st line for most forms of epilepsy apart from focal seizures Teratogenic, so patients need careful advice about contraception Liver damage and hepatitis Hair loss Tremor

Answer 191

1st line for focal seizures Agranulocytosis Aplastic anaemia Induces the P450 system so there are many drug interactions

Answer 192

``` Folate and vitamin D deficiency Megaloblastic anaemia (folate deficiency) Osteomalacia (vitamin D deficiency) ```

Answer 193

Night terrors | Rashes

Answer 194

Stevens-Johnson syndrome or DRESS syndrome. These are life threatening skin rashes. Leukopenia

Answer 195

a seizure lasting more than 5 minutes or 2 or more seizures without regaining consciousness in the interim.

Answer 196

ABCDE Secure the airway Give high-concentration oxygen Assess cardiac and respiratory function Check blood glucose levels Gain intravenous access (insert a cannula) IV lorazepam, repeated after 10 minutes if the seizure continues IV phenobarbital/phenytoin infusion

Answer 197

Buccal midazolam | Rectal diazepam

Answer 198

normal for a baby to reflux feeds, and provided there is normal growth and the baby is otherwise well 90% of infants stop having reflux by one year old

Answer 199

``` Chronic cough Hoarse cry Distress, crying or unsettled after feeding Reluctance to feed Pneumonia Poor weight gain ```

Answer 200

``` Overfeeding Gastro-oesophageal reflux Pyloric stenosis (projective vomiting) Gastritis or gastroenteritis Appendicitis Infections such as UTI, tonsillitis or meningitis Intestinal obstruction Bulimia ```

Answer 201

Can't keep down any feed = pyloric stenosis/intestinal obstruction Projectile vomiting = pyloric stenosis/intestinal obstruction Bile stained vomit = intestinal obstruction Haematemesis / melaena = peptic ulcer / oesophagitis / varices Abdominal distention = intestinal obstruction Reduced LOC / bulging fontanelle / near signs = meningitis / raised ICP Respiratory symptoms = aspiration / infection Blood in stools = gastroenteritis / cows milk protein allergy Signs of infection Rash / angioedema / allergy = cows milk protein allergy Apnoeas

Answer 202

Small, frequent meals Burping regularly to help milk settle Not over-feeding Keep the baby upright after feeding (i.e. not lying flat)

Answer 203

Gaviscon mixed with feeds Thickened milk or formula (specific anti-reflux formulas are available) Ranitidine Omeprazole where ranitidine is inadequate barium meal and endoscopy to ix surgical fundoplication

Answer 204

rare condition brief episodes of abnormal movements associated with GORD in infants. The infants are usually neurologically normal. The key features are: 1) Torticollis: forceful contraction of the neck muscles causing twisting of the neck 2) Dystonia: abnormal muscle contractions causing twisting movements, arching of the back or unusual postures Resolves as reflux is treated

Answer 205

to rule out infantile spasms (west syndrome) and seizures

Answer 206

an IgA vasculitis that presents with a purpuric rash affecting the lower limbs and buttocks in children.

Answer 207

Triggered by an URTI or gastroenteritis leads to IgA deposits in blood vessels in affected organs organs involved = skin, kidneys, GI tract

Answer 208

children under 10 years old

Answer 209

Purpura (red purple rash, palpable under the skin, start on legs and spreads to buttocks) Joint pain (knees and ankles) Abdominal pain Renal involvement

Answer 210

purpura can lead to skin ulceration and necrosis abdominal pain can lead lead GI haemorrhage, intussusception & bowel infarction IgA nephritis

Answer 211

IgA nephritis Macroscopic/microscopic haematuria & proteinuria Nephrotic syndrome = haematuria, proteinuria and oedema

Answer 212

Need to exclude meningococcal septicaemia and leukaemia ``` FBC & blood film Renal profile Serum albumin (nephrotic syndrome) CRP Blood cultures urine dipstick Urine protein:creatinine ratio BP ```

Answer 213

meningococcal septicaemia leukaemia Idiopathic thrombocytopenic purpura haemolytic uraemic syndrome

Answer 214

Palpable purpura + one of: - diffuse abdo pain - arthritis / arthralgia - IgA deposits on histology (biopsy) - proteinuria / haematuria

Answer 215

``` simple analgesia rest hydration steroids monitor with urine dipstick and BP ```

Answer 216

abdo pain settles in days no kidney involvement = recover in 4-6 weeks 1/3 have disease recurrence in 6 months small chance of developing end stage renal failure

Answer 217

the child is born with an underactive thyroid gland

Answer 218

dysgenesis = underdeveloped thyroid gland dyshormonogenesis = a fully developed thyroid gland that doesn't produce enough hormone

Answer 219

newborn blood spot screening test

Answer 220

``` Prolonged neonatal jaundice Poor feeding Constipation Increased sleeping Reduced activity Slow growth and development ```

Answer 221

autoimmune thyroiditis (Hashimoto's thyroiditis)

Answer 222

``` antithyroid peroxidase (anti-TPO) antibodies antithyroglobulin antibodies. ```

Answer 223

T1DM | Coeliac disease

Answer 224

``` Fatigue and low energy Poor growth Weight gain Poor school performance Constipation Dry skin and hair loss ```

Answer 225

TFTs - TSH, T3 and T4 thyroid US Thyroid antibodies - anti-TPO and antithyroglobulin antibodies

Answer 226

Levothyroxine

Answer 227

autoimmune inflammation occurs in the joints. diagnosed where there is arthritis without any other cause, lasting more than 6 weeks in a patient under the age of 16.

Answer 228

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

Answer 229

Still's disease

Answer 230

``` Subtle salmon-pink rash High swinging fevers Enlarged lymph nodes Weight loss Joint inflammation and pain Splenomegaly Muscle pain Pleuritis and pericarditis ```

Answer 231

Negative ANA and RF | raised CRP, ESR, platelets and ferritin

Answer 232

systemic JIA (still's disease) Kawasaki disease rheumatic fever Leukaemia

Answer 233

Macrophage activation syndrome (MAS) = severe activation of immune system with a massive inflammatory response

Answer 234

``` Caused by systemic JIA Unwell child DIC Anaemia Thrombocytopenia. Bleeding Non blanching rash ``` LOW ESR

Answer 235

inflammatory arthritis of 5+ joints, symmetrical, small joints of hands & feet + large joints Mild fever Anaemia Reduced growth

Answer 236

4 joints or less | Larger joint involvement - knee/ankle

Answer 237

Girl <6 yo

Answer 238

Anterior uveitis = refer to opthalmologist

Answer 239

Normal/mildly elevated inflammatory markers ANA often positive RF usually negative

Answer 240

paediatric version of seronegative spondyloarthropathy inflammatory arthritis of joints and enthesitis

Answer 241

males >6 yo

Answer 242

MRI scan - enthesitis HLA-B27 gene

Answer 243

Psoriasis IBD Anterior uveitis - refer to ophthalmologist for screening even if asymptomatic

Answer 244

seronegative inflammatory arthritis associated with psoriasis symmetrical polyarthritis affecting the small joints similar to rheumatoid, or an asymmetrical arthritis affecting the large joints in the lower limb.

Answer 245

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 246

NSAIDs, such as ibuprofen Steroids, either oral, intramuscular or intra-artricular in oligoarthritis Disease modifying anti-rheumatic drugs (DMARDs), such as methotrexate, sulfasalazine and leflunomide Biologic therapy, such as the tumour necrosis factor inhibitors etanercept, infliximab and adalimumab

Answer 247

superficial bacterial skin infection, usually caused by the staphylococcus aureus bacteria. A “golden crust” is characteristic of a staphylococcus skin infection

Answer 248

staph aureus | streptococcus pyogenes

Answer 249

contagious = keep off school until lesions have healed or they've been on abx for 48 hrs don't scratch lesion hand hygiene and don't share towels

Answer 250

non-bullous | bullous

Answer 251

typically occurs around the nose or mouth. The exudate from the lesions dries to form a “golden crust”. They are unsightly but do not usually cause systemic symptoms or make the person unwell.

Answer 252

1st line = antiseptic cream - hydrogen peroxide 1% cream 2nd line = topical fusidic acid 3rd line = oral flucloxacillin

Answer 253

is always caused by the staphylococcus aureus bacteria bacteria can produce epidermolytic toxins that break down the proteins that hold skin cells together. This causes 1 – 2 cm fluid filled vesicles to form on the skin. These vesicles grow in size and then burst, forming a “golden crust”. Eventually they heal without scarring. These lesions can be painful and itchy

Answer 254

Staphylococcus scalded skin syndrome

Answer 255

flucloxacillin - oral or IV

Answer 256

``` Cellulitis if the infection gets deeper in the skin Sepsis Scarring Post streptococcal glomerulonephritis Staphylococcus scalded skin syndrome Scarlet fever ```

Answer 257

``` Meconium ileus Hirschsprung’s disease Oesophageal atresia Duodenal atresia Intussusception Imperforate anus Malrotation of the intestines with a volvulus Strangulated hernia ```

Answer 258

``` persistent bilious vomiting abdo pain and distention failure to pass stools/wind high pitched tinkling bowel sounds in early obstruction absent bowel sounds in late obstruction ```

Answer 259

abdo xray = dilated loops of bowel proximal to lesion and collapsed bowel distal to obstruction

Answer 260

NBM - NG tube to drain stomach IV fluids Refer to paediatric surgical unit for emergency surgery

Answer 261

a condition affecting the small bowel, where the normal peristalsis that pushes the contents along the length of the intestines, temporarily stops.

Answer 262

condition where the bowel “invaginates” or “telescopes” into itself.

Answer 263

more common in boys | 6 months - 2 years old

Answer 264

``` Concurrent viral illness Henoch-Schonlein purpura Cystic fibrosis Intestinal polyps Meckel diverticulum ```

Answer 265

Redcurrant jelly stool Severe, colicky abdominal pain Pale, lethargic and unwell child Right upper quadrant mass = sausage shaped mass Vomiting Intestinal obstruction - vomiting, constipation and abdo distention

Answer 266

US abdomen | contrast enema

Answer 267

therapeutic enema - contrast, water or air enemas are pumped into the colon to force the folded bowel out of the bowel Surgical resection - if enemas don't work or the bowel becomes gangrenous/perforation

Answer 268

Obstruction Gangrenous bowel Perforation Death

Answer 269

also known as mucocutaneous lymph node syndrome It is a systemic, medium-sized vessel vasculitis

Answer 270

<5 yo Males Asian - Japanese/Korean children

Answer 271

Persistent high fever >39 for more than 5 days Widespread erythematous maculopapular rash desquamation (skin peeling) on palms and soles strawberry tongue with large papillae Cracked lips Cervical lymphadenopathy Bilateral conjunctivitis

Answer 272

FBC - anaemia, leukocytosis and thrombocytosis LFTs - hypoalbuminaemia and elevated liver enzymes Raised ESR Urinalysis - raised white cells Echo - coronary artery pathology

Answer 273

Acute phase: The child is most unwell with the fever, rash and lymphadenopathy. This lasts 1 – 2 weeks. Subacute phase: The acute symptoms settle, the desquamation and arthralgia occur and there is a risk of coronary artery aneurysms forming. This lasts 2 – 4 weeks. Convalescent stage: The remaining symptoms settle, the blood tests slowly return to normal and the coronary aneurysms may regress. This last 2 – 4 weeks.

Answer 274

``` High dose aspirin (reduces the risk of thrombosis) IV immunoglobulins (reduces the risk of coronary artery aneurysms) ```

Answer 275

Risk of Reyes syndrome (swelling of liver and brain)

Answer 276

coronary artery aneurysm - so follow up with echocardiograms

Answer 277

cancer of a particular line of the stem cells in the bone marrow. This causes unregulated production of certain types of blood cells.

Answer 278

can be classified depending on how rapidly they progress (chronic is slow and acute is fast) and the cell line that is affected (myeloid or lymphoid).

Answer 279

Acute lymphoblastic leukaemia (ALL) is the most common in children Acute myeloid leukaemia (AML) is the next most common Chronic myeloid leukaemia (CML) is rare

Answer 280

ALL peaks aged 2 – 3 years AML peaks aged under 2 years

Answer 281

A genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal white blood cell. Excessive production of a single type of cell = suppression of other cell lines = pancytopenia Anaemia Leukopenia Thrombocytopenia

Answer 282

``` Radiation exposure Downs Kleinfelter syndrome Noonan syndrome Fanconi's syndrome ```

Answer 283

``` Persistent fatigue Unexplained fever Failure to thrive Weight loss Night sweats Pallor (anaemia) Petechiae and abnormal bruising (thrombocytopenia) Unexplained bleeding (thrombocytopenia) Abdominal pain Generalised lymphadenopathy Unexplained or persistent bone or joint pain Hepatosplenomegaly ```

Answer 284

FBC - anaemia. leukopenia, thrombocytopenia and high numbers of the abnormal WBCs Blood film = BLAST CELLS bone marrow biopsy lymph node biopsy For staging = - CXR - CT scan - LP - genetic analysis and immunophenotyping of the abnormal cells

Answer 285

Unexplained petechiae or hepatomegaly in any child Do urgent FBC and refer

Answer 286

``` MDT Chemotherapy Radiotherapy Bone marrow transplant Surgery ```

Answer 287

``` Failure to treat the leukaemia Stunted growth and development Immunodeficiency and infections Neurotoxicity Infertility Secondary malignancy Cardiotoxicity ```

Answer 288

Cure rate for ALL is 80% | Less positive outcomes for AML

Answer 289

inflammation of the meninges. The meninges are the lining of the brain and spinal cord. This inflammation is usually due to a bacterial or viral infection.

Answer 290

children = - neisseria meningitidis - streptococcus pneumonia Neonates = - group b strep

Answer 291

the meningococcus* bacterial infection in the bloodstream *neisseria meningitidis

Answer 292

gram negative diplococcus

Answer 293

non blanching rash | the neisseria meningitidis bacteria causes DIC and subcutaneous haemorrhages

Answer 294

``` fever neck stiffness vomiting headache photophobia altered consciousness seizures ```

Answer 295

``` hypotonia poor feeding lethargy hypothermia bulging fontanelle ```

Answer 296

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

Answer 297

Kernigs test - lie on back, flex hip to 90 d then slowly straighten the knee to stretch the meninges and cause spinal pain in meningitis Brudzinski's test - lie on back, gently lift head and neck off the bed and flex their chin to their chest. Positive if they involuntarily flex their hips and knees

Answer 298

If they have suspected meningitis + non blanching rash = IM benzylpenicillin STAT & transfer to hospital

Answer 299

Blood culture and LP before abx - Do meningococcal PCR on blood cultures Under 3 months old = cefotaxime + amoxicillin Older than 3 months = ceftriaxone Steroids (dexamethasone) - to reduce risk of hearing loss and neurological damage

Answer 300

Significant exposure to person with meningococcal meningitis/septicaemia within the 7 days prior to the onset of illness = need prophylaxis

Answer 301

``` single dose of ciprofloxacin Give ASAP (ideally within 24 hrs of initial diagnosis) ```

Answer 302

acyclovir | supportive treatment

Answer 303

herpes simplex virus enterovirus varicella zoster virus

Answer 304

Viral PCR testing on CSF from LP

Answer 305

Needle inserted into lower back between L3 and L4 (spinal cord ends at L1-2). A sample of CSF is collected.

Answer 306

``` Bacterial culture Viral PCR Cell count Protein glucose ```

Answer 307

``` Cloudy appearance High protein Low glucose High WCC (neutrophils) Culture = bacteria ```

Answer 308

``` Clear appearance] Mildly raised or normal protein Normal glucose High WCC (lymphocytes) Culture = negative ```

Answer 309

Hearing loss is a key complication Seizures and epilepsy Cognitive impairment and learning disability Memory loss Cerebral palsy, with focal neurological deficits such as limb weakness or spasticity

Answer 310

a viral infection spread by respiratory droplets

Answer 311

14-25 days

Answer 312

MMR 80% protective against mumps

Answer 313

Flu like prodrome: - fever - muscle aches - lethargy - reduced appetite - headache - dry mouth Parotid gland swelling: - unilateral or bilateral - associated pain Other symptoms: - Abdo pain (pancreatitis) - Testicular pain and swelling (orchitis) - Confusion, neck stiffness and headache (meningitis/encephalitis)

Answer 314

is usually a self limiting condition that lasts around 1 week.

Answer 315

Viral saliva swab for PCR testing | Can test the swab or blood for antibodies to mumps virus

Answer 316

Notifiable disease - talk to public health | Supportive mx - fluids, rest, analgesia

Answer 317

Pancreatitis Orchitis Meningitis Sensorineural hearing loss

Answer 318

0-4 yo - septic arthritis - developmental dysplasia of hip - transient synovitis 5-10 yo - septic arthritis - transient synovitis - perches disease 10-16 yo - septic arthritis - slipped upper femoral epiphysis - JIA

Answer 319

infection with Epstein Barr virus found in the saliva of infected individuals. Infection may be spread by kissing or by sharing cups, toothbrushes and other equipment that transmits saliva. Infected as child = few symptoms, infections as teenager/young adult = severe symptoms

Answer 320

intensely itchy maculopapular rash

Answer 321

``` Fever Sore throat Fatigue Lymphadenopathy (swollen lymph nodes) Tonsillar enlargement Splenomegaly and in rare cases splenic rupture ```

Answer 322

Test for heterophile antibodies - general antibodies made that are not specific to EBV antigens but the test is almost 100% specific for EBV. Can take 6 weeks to produce antibodies and not everyone produces them so is 70-80% sensitive. Test for them with: - Monospot test - RBCs from patients introduced to RBCs from horses. Heterophile antibodies present if the RBCs react to the horses - Paul-Bunnell test - same but blood from sheep

Answer 323

Heterophile antibodies (monospot or paul-bunnell test) EBV antibodies that target viral capsid antigen (IgM in early infection & IgG after infection)

Answer 324

self limiting supportive mx Avoid alcohol avoid contact sports - splenic rupture risk

Answer 325

The acute illness lasts around 2 – 3 weeks, however it can leave the patient with fatigue for several months once the infection is cleared.

Answer 326

``` Splenic rupture Glomerulonephritis Haemolytic anaemia Thrombocytopenia Chronic fatigue ``` Is associated with Bursitis lymphoma

Answer 327

Polio Flu vaccine Hepatitis A Rabies

Answer 328

``` Pneumococcus Meningococcus Hepatitis B Pertussis (whooping cough) Haemophilus influenza type B Human papillomavirus (HPV) Shingles (herpes-zoster virus) ```

Answer 329

Measles, mumps and rubella vaccine: contains all three weakened viruses BCG: contains a weakened version of tuberculosis Chickenpox: contains a weakened varicella-zoster virus Nasal influenza vaccine (not the injection) Rotavirus vaccine

Answer 330

diphtheria | tetanus

Answer 331

live attenuated vaccines

Answer 332

6 in 1 vaccine (diphtheria, tetanus, pertussis, polio, haemophilus influenzae type B (Hib) and hepatitis B) Meningococcal type B Rotavirus (oral vaccine)

Answer 333

6 in 1 vaccine (again) Pneumococcal (13 different serotypes) Rotavirus (again)

Answer 334

6 in 1 vaccine (again) | Meningococcal type B (again)

Answer 335

``` 2 in 1 (haemophilus influenza type B and meningococcal type C) Pneumococcal (again) MMR vaccine (measles, mumps and rubella) Meningococcal type B (again) ```

Answer 336

Influenza vaccine (nasal vaccine)

Answer 337

``` 4 in 1 (diphtheria, tetanus, pertussis and polio) MMR vaccine (again) ```

Answer 338

HPV (2 doses given 6 to 24 months apart)

Answer 339

3 in 1 (tetanus, diphtheria and polio) | Meningococcal groups A, C, W and Y

Answer 340

Gardasil (protects from strains 6, 11, 16 and 18)

Answer 341

Children at risk of TB infection - relatives from countries with high TB prevalence Offered from birth

Answer 342

a syndrome that occurs when an infection causes the child to become systemically unwell. It is the result of a severe systemic inflammatory response. It is a life threatening condition

Answer 343

``` Deranged physical observations Prolonged CRT Fever / 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) Shock involves circulatory collapse and hypoperfusion of organs. ```

Answer 344

macrophages, lymphocytes and mast cells release cytokines, interleukins and TNF Causes the release of nitrous oxide = vasodilation cytokines cause endothelial lining of blood vessels to become more permeable = oedema & reduction in intravasc vol Get DIC as the coagulation system is activated = consumption of platelets and clotting factors to create blood clots = thrombocytopenia and haemorrhages Blood lactate rises due to anaerobic respiration

Answer 345

when sepsis has lead to cardiovascular dysfunction. Arterial BP falls = organ hypo perfusion Rise in blood lactate due to anaerobic respiration by organs

Answer 346

IV fluids | If that fails - ICU for inotropes e.g. noradrenalin

Answer 347

``` Give O2 Get IV access Take bloods - FBC, U&E, CRP, INR, blood gas (lactate & acidosis) Take blood cultures Urine dipstick Give Abx within 1 hour of presentation IV fluids - 20ml/kg IV bolus of saline ```

Answer 348

``` CXR Abdo and pelvic USS LP for meningitis Meningococcal PCR blood test Serum cortisol (if adrenal crisis suspected) ``` Continue abx for 5-7 days if bacterial infection suspected

Answer 349

a rare autosomal recessive condition that causes a progressive loss of motor neurones, leading to progressive muscular weakness.

Answer 350

the lower motor neurones in the spinal cord.

Answer 351

SMA type 1 has an onset in the first few months of life, usually progressing to death within 2 years. SMA type 2 (most common) has an onset within the first 18 months. Most never walk, but survive into adulthood. SMA type 3 has an onset after the first year of life. Most walk without support, but subsequently loose that ability. Respiratory muscles are less affected and life expectancy is close to normal. SMA type 4 has an onset in the 20s. Most will retain the ability to walk short distances but require a wheelchair for mobility. Everyday tasks can lead to significant fatigue. Respiratory muscles and life expectancy are not affected.

Answer 352

``` Fasiculations Reduced muscle bulk Reduced tone Reduced power Reduced/absent reflexes ```

Answer 353

``` MDT physio NIV SMA 1 - tracheostomy and ventilation may be required PEG feeding ```

Answer 354

``` VSD ASD PDA Coarctation of the aorta Aortic valve stenosis ```

Answer 355

tetralogy of Fallot transposition of the great arteries (TGA) tricuspid atresia ebsteins anomaly

Answer 356

lithium use in pregnancy

Answer 357

a congenital heart condition where the tricuspid valve is set lower in the right side of the heart (towards the apex), causing a bigger right atrium and a smaller right ventricle poor flow to r ventricle

Answer 358

right to left via an atrial septal defect = cyanosis

Answer 359

Wolff-Parkinson-White syndrome.

Answer 360

tricuspid regurgitation (pan-systolic murmur) and tricuspid stenosis (mid-diastolic murmur) ``` Evidence of heart failure (e.g. oedema) Gallop rhythm heard on auscultation characterised by the addition of the third and fourth heart sounds Cyanosis Shortness of breath and tachypnoea Poor feeding Collapse or cardiac arrest ```

Answer 361

echo | cxr - box shaped heart due to R atrium hypertrophy

Answer 362

tx arrhythmias and HF prophylactic abx (IE) surgical correction

Answer 363

IV amoxicillin + cefotaxime (to cover for listeria)

Answer 364

an X-linked recessive inherited disorder in the dystrophin genes required for normal muscular function.

Answer 365

progressive proximal muscle weakness from 5 years calf pseudohypertrophy Gower's sign: child uses arms to stand up from a squatted position 30% of patients have intellectual impairment

Answer 366

raised creatinine kinase | genetic testing has now replaced muscle biopsy as the way to obtain a definitive diagnosis

Answer 367

supportive | MDT

Answer 368

most children cannot walk by the age of 12 years | patients typically survive to around the age of 25-30 years

Answer 369

dilated cardiomyopathy

Answer 370

neonatal sepsis Werdnig-Hoffman disease (spinal muscular atrophy type 1) hypothyroidism Prader-Willi maternal drugs e.g. benzodiazepines maternal myasthenia gravis

Answer 371

single dose mebendazole for whole household and hygiene advice

Answer 372

hypertrophy and narrowing of the pylorus (exit of the stomach)

Answer 373

``` Present in the first few weeks of life hungry, thin pale baby failure to thrive PROJECTILE VOMITING Mass in upper abdomen - LARGE OLIVE after feeding, can observe peristalsis in the abdomen ```

Answer 374

hypochloric metabolic alkalosis | low chloride & alkalotic from vomiting hydrochloric acid

Answer 375

Abdo USS - visualise thickened pylorus

Answer 376

Laparoscopic pyloromyotomy = ramstedt's operation Incision is made in the smooth muscle of the pylorus to widen the canal

Answer 377

prodrome, e.g. low-grade fever rash: maculopapular, initially on the face before spreading to the whole body, usually fades by the 3-5 day lymphadenopathy: suboccipital and postauricular

Answer 378

arthritis thrombocytopaenia encephalitis myocarditis

Answer 379

autosomal recessive condition abnormal gene for beta-globing on chromosome 11 one copy of the gene = sickle cell trait (asymptomatic) two copies of the gene = sickle cell disease

Answer 380

a genetic condition that causes sickle (crescent) shaped red blood cells. This makes the red blood cells fragile and more easily destroyed, leading to a haemolytic anaemia. Patients with sickle cell anaemia are prone to various types of sickle cell crises.

Answer 381

normal haemoglobin: HbAA sickle cell trait: HbAS homozygous sickle cell disease: HbSS. Some patients inherit one HbS and another abnormal haemoglobin (HbC) resulting in a milder form of sickle cell disease (HbSC) sickle cells are fragile and haemolyse; they block small blood vessels and cause infarction

Answer 382

women at risk of being carrier are offered testing during pregnancy tested on newborn screening heel prick on day 5 diagnosis = haemoglobin electrophesis

Answer 383

sickle cell disease cystic fibrosis congenital hypothyroidism ``` 6 inherited metabolic disease - phenylketonuria medium chain acyl-coA dehydrogenase deficiency maple syrup urine disease isovaleric academia glutamic acuduria type 1 homocytinuria ```

Answer 384

``` anaemia increased infection risk stroke avascular necrosis of large joints e.g. hip pulmonary HTN painful and persistent penile erectile (priapism) CKD Sickle cell crises acute chest syndrome ```

Answer 385

Avoid dehydration and other triggers of crises Ensure vaccines are up to date Antibiotic prophylaxis to protect against infection, usually with penicillin V (phenoxymethypenicillin) Hydroxycarbamide can be used to stimulate production of fetal haemoglobin (HbF). Fetal haemoglobin does not lead to sickling of red blood cells. This has a protective effect against sickle cell crises and acute chest syndrome. Blood transfusion for severe anaemia Bone marrow transplant can be curative

Answer 386

an umbrella term for a spectrum of acute crises related to the condition. These range from mild to life threatening.

Answer 387

``` occur spontaneously infection dehydration cold significant life events ```

Answer 388

Have a low threshold for admission to hospital Treat any infection Keep warm Keep well hydrated (IV fluids may be required) Simple analgesia such as paracetamol and ibuprofen (NSAIDs should be avoided where there is renal impairment) Penile aspiration is used to treat priapism

Answer 389

caused by the sickle shaped blood cells clogging capillaries and causing distal ischaemia

Answer 390

dehydration | raised haematocrit

Answer 391

pain fever priapism

Answer 392

Splenic sequestration crisis is caused by red blood cells blocking blood flow within the spleen. = acutely enlarged and painful spleen. The pooling of blood in the spleen can lead to severe anaemia and circulatory collapse (hypovolaemic shock).

Answer 393

emergency - supportive - blood transfusions - fluid resuscitation need splenectomy if recurrent crises

Answer 394

a situation where there is temporary loss of the creation of new blood cells. This is most commonly triggered by infection with parvovirus B19. = significant anaemia

Answer 395

fever or respiratory symptoms with new infiltrates seen on CXR

Answer 396

infection - pneumonia or bronchiolitis non infective - pulmonary vaso occlusion or fat emboli

Answer 397

medical emergency Antibiotics or antivirals for infections Blood transfusions for anaemia Incentive spirometry using a machine that encourages effective and deep breathing Artificial ventilation with NIV or intubation may be required

Answer 398

FEVER ``` Babies: Fever Lethargy Irritability Vomiting Poor feeding Urinary frequency ``` ``` Children: Fever Abdominal pain, particularly suprapubic pain Vomiting Dysuria (painful urination) Urinary frequency Incontinence ```

Answer 399

A temperature greater than 38°C | Loin pain or tenderness

Answer 400

clean catch urine sample for: - urine dip - nitrates, leukocytes - MC&S

Answer 401

All children under 3 months with a fever should start immediate IV antibiotics (e.g. ceftriaxone) and have a full septic screen = blood cultures, bloods, lactate + consider LP Oral abx if >3 months if otherwise well: - trimethoprim - nitrofurantoin - cefalexin - amoxicillin

Answer 402

abdo USS for: - children <6 months with first UTI have USS within 6 weeks - Children with recurrent UTIs have USS within 6 weeks - Children with atypical UTI have USS during illness DMSA scan : - used to ix 4-6 months after illness to assess for damage from recurrent/atypical UTIs Micturating cystourethrogram: Used to assess for vesico-ureteric reflux

Answer 403

an eruptive widespread rash There are 6 underlying causes

Answer 404

``` Measles (measles virus) Scarlet fever (strep pyogenes) Rubella (rubella virus) Duke's disease (non specific) Parvovirus B19 - slapped cheek disease Roseola infantum ```

Answer 405

Start with fever, coryza and conjunctivitis Koplik spots on buccal mucosa rash that starts on face and behind ears, then spreads to the rest of the body - red macular rash with flat lesions

Answer 406

Report to public health - notifiable disease | isolate for 4 days after symptoms resolve

Answer 407

``` Pneumonia Diarrhoea Dehydration Encephalitis Meningitis Hearing loss Vision loss Death ```

Answer 408

Group A streptococcus - strep pyogenes | associated with tonsillitis

Answer 409

``` red-pink blotchy macular rash with rough 'sandpaper' skin that starts on the trunk and spreads outwards red flushed cheeks fever lethargy sore throat strawberry tongue cervical lymphadenopathy ```

Answer 410

penicillin V for 10 days

Answer 411

notifiable disease | stay off school until 24 hours after starting abx

Answer 412

erythematous macular rash on face and spreads to rest of body (not as bad as measles) mild fever joint pain sore throat lymphadenopathy - behind ears and back of neck)

Answer 413

notifiable disease stay off school for 5 days after rash appears avoid pregnant women

Answer 414

thrombocytopenia encephalitis dangerous in pregnancy - congenital rubella syndrome: deafness, blindness, congenital heart disease

Answer 415

non-specific 'viral rash'

Answer 416

slapped cheek disease / erythema infectiosum

Answer 417

mild fever coryza muscle aches lethargy bright red rash on both cheeks - slapped cheeks spreads to give reticular erythematous rash on trunk and limbs that's itchy

Answer 418

human herpes virus 6 / 7

Answer 419

torsion of the colon around it's mesenteric axis resulting in compromised blood flow and closed loop obstruction

Answer 420

a genetic condition that results in brittle bones that are prone to fractures genetic mutations that affect collagen formation

Answer 421

``` blue sclera hyper mobility unusual/recurrent fractures triangular face short stature deafness from early adulthood dental problems - formation of teeth bone deformities - bowed legs and scoliosis joint and bone pain ```

Answer 422

bisphosphonates vitamin D supplements MDT

Answer 423

inflammation at the tibial tuberosity where the patella ligament inserts anterior knee pain in teenagers more common in males unilateral usually

Answer 424

a gradual onset of symptoms: Visible or palpable hard and tender lump at the tibial tuberosity Pain in the anterior aspect of the knee The pain is exacerbated by physical activity, kneeling and on extension of the knee

Answer 425

stress from running/jumping at the time of growth in the epiphyseal plate = inflammation on the tibial epiphyseal plate get small multiple avulsion fractures as the patella ligament pulls away tiny pieces of bone leads to growth of the tibial tuberosity = visible lump below the knee

Answer 426

reduction in physical activity Ice NSAIDs

Answer 427

fully resolves over time rare complication = avulsion fracture

Answer 428

most common cause of disproportionate short stature (dwarfism). It is a type of skeletal dysplasia.

Answer 429

autosomal dominant inheritance mutation in FGFR3 gene on chromosome 4 homozygous gene mutations = fatal in the neonatal period therefore patients have one normal and one abnormal gene

Answer 430

``` disproportionate short stature average height = 4 feet limbs mostly affected by reduced bone length, spine length less affected intelligence and life expectancy not affected short digits genu varum disproportionate skull foramen magnum stenosis ```

Answer 431

Recurrent otitis media, due to cranial abnormalities Kyphoscoliosis Spinal stenosis Obstructive sleep apnoea Obesity Foramen magnum stenosis can lead to cervical cord compression and hydrocephalus

Answer 432

MDT | leg lengthening surgery - not done very much

Answer 433

A condition where there is a structural abnormality in the hips due to abnormal development of bones during pregnancy. Instability in the hips - tendency for subluxation or dislocation

Answer 434

First degree family history Breech presentation from 36 weeks onwards Breech presentation at birth if 28 weeks onwards Multiple pregnancy

Answer 435

Done during the neonatal examination (at birth then 6-8 weeks old) Ortolani test Barlow test

Answer 436

Different leg lengths Restricted hip abduction on one side Significant bilateral restriction in abduction Difference in the knee level when the hips are flexed Clunking of the hips on special tests

Answer 437

done to look for developmental dysplasia of the hips lie baby on back, flex hips and knees, abduct the hips and apply pressure behind the legs - see if the hips will dislocate anteriorly

Answer 438

done to look for developmental dysplasia of the hips lie baby on back, with hips and knees flexed and knees together, put gentle downward pressure on the knee to see if the femoral head will dislocate posteriorly

Answer 439

If its suspected do an USS of the hips | x-rays helpful in older infants

Answer 440

Pavlik harness (<6 months old) - keeps the hips flexed and abducted to hold femoral head in the correct position while the acetabulum develops to a normal shape surgery if the harness fails / >6 months - then need hip spica cast

Answer 441

a fixed abnormal ankle position that presents at birth. clubfoot

Answer 442

Talipes equinovarus describes the ankle in plantar flexion and supination. Talipes calcaneovalgus describes the ankle in dorsiflexion and pronation.

Answer 443

Ponseti Method - foot is manipulated into a normal position and cast appleid - this is repeated until the foot is in the correct position - need achilles tenotomy - brace to hold feet in correct position afterwards

Answer 444

the head of the femur is displaced (“slips”) along the growth plate.

Answer 445

Boys 8-15 yo | more common in obese children

Answer 446

``` growth spurt minor trauma hip, groin, thigh or knee pain restricted range of hip movement painful limp restricted movement in hip ``` prefer their hip in external rotation

Answer 447

xray inflammatory markers to exclude other causes technetium bone scan CT/MRI scan

Answer 448

surgery to return femoral head to correct position

Answer 449

disruption of blood flow to the femoral head, causing avascular necrosis of the bone.

Answer 450

4-12 yo | more common in body

Answer 451

idiopathic

Answer 452

a slow onset of: ``` Pain in the hip or groin Limp Restricted hip movements There may be referred pain to the knee There will be no history of trauma ```

Answer 453

xray can be normal technetium bone scan MRI scan

Answer 454

``` mild = bed rest traction crutches analgesia physio regular xrays to assess healing ``` more severe/older children = surgery to improve the alignment of the femoral head and hip