Paediatrics Flashcards

Question

Give 3 causes of conductive hearing loss.

Answer 1

1. Glue ear 2. Ear wax 3. Otitis media 4. Perforated ear drum

Answer 2

1. Speech and language delay 2. Social problems e.g. behavioural issues 3. Academic underachievement

Answer 3

Inflammation of the paranasal sinuses

Answer 4

Viruses e.g. RSV and rhinovirus

Answer 5

Short symptoms suggest viral (<10 days) Purulent nasal discharge Nasal obstruction Facial pain/pressure and swelling

Answer 6

Paracetomol and ibuprofen: symptomatic relief Amoxicillin/ doxycycline: if bacterial Avoid antihistamines: may thicken secretions

Answer 7

Inspiration + upper airways + narrowing

Answer 8

- Stridor - Hoarseness - Barking cough (sea lion) - Dyspnoea

Answer 9

Degree of chest retraction and degree of stridor

Answer 10

Avoid examination using a spatula as this may precipitate total obstruction

Answer 11

Inflammation of the vocal cords

Answer 12

Laryngotracheobronchitis

Answer 13

URTI → mucosal inflammation and increased secretions → larynx oedema

Answer 14

Subglottic area → critical narrowing of the trachea

Answer 15

Parainfluenza 1, 2, 3 (most common) Metapneumovirus RSV Influenza

Answer 16

Autumn time 6 months- 6 years old; peak incidence ≈2 years old

Answer 17

- Barking cough (worse at night) - Harsh stridor - Hoarse voice - Preceding non specific viral URTI - Increased work of breathing - Coryza: low grade fever

Answer 18

Conservative Oral dexamethasone 1.8mg (0.15mg/kg)

Answer 19

Cyanosis Altered consciousness Rising HR/RR Restlessness

Answer 20

Pseudomembranous croup is an uncommon but dangerous disease very similar to severe croup

Answer 21

- High fever - Appears toxic - Tracheal tenderness - Rapidly progressive airways obstruction (thick exudate cant be cleared by coughing)

Answer 22

S. aureus Strep Group A H. influenzae

Answer 23

IV Vancomycin

Answer 24

Inflammation and localised oedema of the epiglottis Life-threatening airway obstruction

Answer 25

Haemophilus influenza type B

Answer 26

>99% reduction in cases Most common in ages 2-7 years

Answer 27

- Intense swelling of epiglottis - Onset very acute - High fever - Dysphagia and speech difficulty due to pain - Drooling - Stridor - Minimal cough - Tripod position

Answer 28

Immobile Upright Open mouth to optimise the airway

Answer 29

Laryngoscopy

Answer 30

Do not examine throat with spatula or lie the child down Do not upset Do not cannulate

Answer 31

IV Abx: Cefotaxime Intubate Supplemental O2 Corticosteriods (dexamethasone) Tracheostomy: if complete obstruction

Answer 32

Croup: 1) Days 2) Severe barking cough 3) Preceding coryza 4) Can drink Acute Epiglottitis: 1) Hours 2) Absent/minor cough 3) No preceding coryza 4) Can't drink

Answer 33

Croup: 1) None 2) Unwell appearance 3) Fever <38.5 4) Harsh stridor with hoarse cry Acute Epiglottitis: 1) Drooling 2) Toxic, very unwell 3) Fever >38.5 4) Soft whispering stridor, muffled cry/reluctant to speak

Answer 34

Croup = stridor + Parainfluenza virus B Bronchiolitis = wheeze + RSV

Answer 35

Bordetella pertussis: Gram- coccobacillus

Answer 36

Inhaled droplets

Answer 37

- Malaise - Conjunctivitis - Nasal discharge - Sore throat - Dry cough - Mild fever

Answer 38

After two weeks of the catarrhal phase →Paroxysmal coughing stage (barking cough)

Answer 39

Dry hacking cough that is worse at night and after feeds Coughing episodes followed by characteristic 'whoop' → loss of consciousness

Answer 40

Inspiration against a closed glottis Child chokes, gasps and face reddens

Answer 41

Vomiting Apnoea Cyanosis Coughing fits → Subconjunctival haemorrhage/anoxia → seizures/syncope

Answer 42

- Notifiable disease - Nasopharyngeal swabs: PCR - Bacterial culture: MCS - Oral anti-pertussis IgG - Marked lymphacytosis= COMMON

Answer 43

Hospitalised: if <6 months (risk of apnoea) 10-14 days incubation Off school for 48 hours after Abx commencement: - Macrolides: Azithromycin or Erythromycin - Erythromycin: pregnant women

Answer 44

LRTI following a URTI Inflammation and infection in the bronchioles

Answer 45

Commonest lung infection in infants (peaks at 3-6 months) Rare in those aged >1 years

Answer 46

Congenital heart defects Cystic Fibrosis Down's syndrome

Answer 47

Respiratory syncytial virus (RSV): 80%of cases Metapneumovirus Parainfluenza Rhinovirus Adenovirus

Answer 48

RSV + Metapneumovirus dual infection

Answer 49

- Premature/low birth weight - Cystic Fibrosis - Congenital heart disease - Down's syndrome - Immunocompromised

Answer 50

Coryza precedes dry cough Tachycardia Signs of respiratory distress Dyspnoea Tachypnoea Poor feeding Mild fever (under 39ºC) Apnoeas episodes Wheeze and crackles Cyanosis Sub/ intercostal recession Hyperinflation of chest: -Prominent sternum -Liver displaced downwards

Answer 51

- Infrequent feeding - Respiratory distress - Hypoxia

Answer 52

Pulse oximetry Viral throat swab ABG FBC CXR: excludes pneumothorax

Answer 53

Oxygen therapy Supportive (NG nutrition, fluids, temp control) Ventilation (CPAP) Palivizumab prophylaxis Ribavarin: if high risk

Answer 54

Antiviral against RSV Used against Hep C (+ interferons/ simeprevir, sofosbuvir) and haemorrhagic fevers

Answer 55

High flow humidified oxygen via nasal cannulae

Answer 56

Inflammation of the lungs

Answer 57

Pneumonia that develops within 48 hours of admission

Answer 58

CURB-65:** **C**onfusion **U**rea >7 **R**espiratory rate ≥ 30 **B**lood pressure < 90 systolic or ≤ 60 diastolic. Age ≥ **65** **Score 0/1:** consider treatment at home **Score ≥ 2:** consider hospital admission and dual antibiotics **Score ≥ 3:** consider intensive care assessment

Answer 59

Group B strep: maternal Gram negative enterococci

Answer 60

S. pneumoniae Haemophilus influenza B S. Aureus (infrequent but serious)

Answer 61

RSV Influenza A/B Parainfluenza Adenovirus

Answer 62

Mycoplasma pneumoniae Chlamydia pneumoniae

Answer 63

Productive cough +/- haemoptysis - Non-productive cough = atypical infection High fever (> 38.5ºC) Tachypnoea Tachycardia Hypoxia Hypotension Increased work of breathing Lethargy Delirium (acute confusion associated with infection) Pleuritic chest pain Poor feeding Respiratory distress

Answer 64

**Bronchial breathing (**loud breathing due to turbulent blood flow) **Focal coarse crepitations (**crackles from purulence) **Dullness to percussion**

Answer 65

Pulse oximetry Sputum and blood MCS FBC: CRP CXR: consolidations

Answer 66

If resp distress/O2 <92%: admit to hospital Oxygen IV fluids If acutely unwell: IV benzylpenicillin ORAL Abx for newborns: amoxicillin

Answer 67

- Raised respiratory rate - Use of accessory muscles to breath - Intercostal and subcostal recessions - Nasal flaring - Head bobbing - Tracheal tugging - Cyanosis (due to low oxygen saturation) - Abnormal airway noises

Answer 68

Inspiration + nonmusical + heard when air is forced through passages blocked by exudates

Answer 69

Expiration + musical + narrowing of smaller airways (e.g bronchioles)

Answer 70

Expiration + coarse sounds + narrowing of tracheobronchial passages

Answer 71

Chronic lung condition where airways inflame in response to hypersensitivity (IgE)

Answer 72

Transient early wheeze Persistent and recurrent wheeze

Answer 73

Viral associated wheeze: e.g. RSV Small airways obstructed due to inflammation More common in males Resolves by 5 years old

Answer 74

Presence of IgE to common inhalant allergens (Atopic Asthma) FHx Associated with eczema, hayfever and food allergies

Answer 75

Bronchial inflammation leads to: 1) Oedema 2) Excessive mucous production 3) Infiltration of cells

Answer 76

Airway narrowing and reversible airflow obstruction

Answer 77

Eosinophils Neutrophils Mast cells Lymphocytes

Answer 78

Low BW FHx Bottle fed Atopy Male Pollution ADAM33 Gene Samter’s triad: - Asthma - Nasal polyps - Aspirin insensitivity

Answer 79

Pollen Dust Feathers Exercise Pollution Cold air Illness (viruses)

Answer 80

Dyspnea: dinural (worse at night and in the morning) Dry cough Expiratory polyphonic wheeze Chest tightness Hx of atopic conditions and triggers **Symptoms improve with bronchodilators** SILENT CHEST RED FLAG = exacerbation

Answer 81

**Spirometry:** -FEV1/FEC: <70% of predicted -FEV1: reduced -Bronchodilator reversibility (BDR): increases FEV1 by ≥12% + ≥200ml volume CXR FBC: raised eosinophil count = inflammation from allergic reaction Direct bronchial challenge test: histamine Allergy testing PEFR diary: diurnal variation - >20% predicted 3/7 days Fractional exhaled nitric oxide (FeNO): >40 ppb (measures lung inflammation)

Answer 82

FEV1/FVC: >70%: Mild 60-69%: Moderate 50-59%: Moderately Severe 35-49%: Severe <34%: Very Severe

Answer 83

1. **SABA** PRN (short acting beta 2 agonist) (**blue** reliever): e.g. **salbutamol** 2. + low dose **ICS** daily if uncontrolled (**brown** preventer): e.g. **beclomethasone** 3. + **LTRA** (leukotriene receptor antagonist): e.g. **montelukast** 4. + **LABA** if uncontrolled (**green-purple** long acting beta 2 agonist): **salmetrol**

Answer 84

1) SABA 2) 8 week moderate dose ICS trial 3) Add LTRA

Answer 85

O SHIT ME: Oxygen Salbutamol (nebulised) Hydrocortisone IV/ oral prednisolone Ipratropium bromide (nebulised) Theophylline IV Magnesium sulphate IV Escalate

Answer 86

Rare, autosomal recessive genetic ciliary disorder Triad of situs inversus, chronic sinusitis and bronchiectasis This leads to recurrent chest infections

Answer 87

Autosomal recessive multi-system disease of water and salt transport affecting the mucosal glands

Answer 88

Autosomal recessive mutations with cystic fibrosis transmembrane conductance regulator gene on chromosome 7 (CFTR)

Answer 89

CFTR gene is a critical chloride channel: 1) Decreased excretion of chloride into the airway lumen and an increased reabsorption of sodium into the epithelial cells 2) Less excretion of salt → less excretion of water and increased viscosity/tenacity of airway secretion

Answer 90

**Meconium ileus** **Recurrent respiratory tract infections** **Pancreatitis** **Steatorrhoea** **Salty skin** Poor weight and height: malnutrition due to malabsorption leads to **failure to thrive **and slow growth Chronic thick productive cough Clubbing Dysponea Crackles and wheeze Abdominal pain and bloating Nasal polyps Male almost always infertile Secondary amenorrhoea in females Arthropathy DM

Answer 91

- Newborn blood spot testing: immunoreactive trypsinogen test (mucus prevents trypsinogen from reaching the intestines and causes build up in blood) - SWEAT TEST: sodium >60mmol/L - Genetic testing

Answer 92

Younger: S. aureus, S. pneumoniae EVENTUALLY >90% will have pseudomonas aeruginosa

Answer 93

Salbutamol + ICS Lung transplant if FEV1 <30% expected Pseudomonas Aeruginosa abx: nebulised tobramycin Inhalation of recombinant human deoxyribonuclease (rhDNAse) Mucolytics (dornase alfa or Acetylcysteine) Hypertonic saline by inhalation (hydrators) Amiloride (inhibits sodium transport) Airway clearance techniques: chest physiotherapy, exercise and postural drainage. PERT (pancreatic enzyme replacement therapy)

Answer 94

1) Holes/connections 2) Narrowing/stenosis 3) Complex (right→left shunt)

Answer 95

Bicuspid aortic valve VSD

Answer 96

ASD PDA VSD AVSD

Answer 97

VSD PAD ASD AVSD BREATHLESS (acyanotic)

Answer 98

Stabilise the patient Increase calorie intake NG tube Diuretics and ACEi to prevent HF symptoms Surgical repair

Answer 99

Coarctation of the aorta (collapse with shock) AS PS

Answer 100

Tetralogy of Fallot Transposition of the great arteries BLUE (cyanotic)

Answer 101

Pulmonary pressure exceeds systemic pressure Reverses shunt**→** cyanosis (left→ right becomes right**→left shunt)**

Answer 102

Soft Systolic aSymptomatic left Sternal edge normal heart Sounds

Answer 103

FBC CXR PaO2 Echo +/- cardiac catheterisation

Answer 104

Foetal alcohol syndrome Trisomy 21

Answer 105

- Ostium Secondum 90% (high hole) - Ostium Primum (10% low hole) - AVSD

Answer 106

All other CHDs

Answer 107

Asymptomatic Fixed and widely split S2 Ejection systolic murmur in pulmonary area Palpitations

Answer 108

Tube between the aorta and pulmonary artery that fails to close 1 month after birth

Answer 109

- Poor feeding (failure to thrive) - Tachypnoea - Active precordium - Thrill - Hepatomegaly - Oedema - Pneumonias - CCF + pulmonary hypertension - LOUD S2 - Large, bounding collapsing pulse (Gallop rhythm) - Heaving apex beat - Left subclavicular thrill - Continuous machinery murmur pulmonary area

Answer 110

ECG and CXR: normal Echo: ensures no duct dependant circulation (e.g in pulmonary atresia)

Answer 111

Ibuprofen/Indomethacin (prostaglandin inhibitor): closes duct SURGERY after 1 year: bacterial endocarditis risk

Answer 112

High risk of bacterial endocarditis

Answer 113

Poor feeding and FTT Tachypnoea Thrill Hepatomegaly Oedema Harsh loud pansystolic mumur best heard in LLSE +/- thrill Gallop rhythm Active pre-cordium

Answer 114

Echo: precise defect ECG: hypertrophy CXR: - Cardiomegaly - Pulmonary oedema - Enlarged pulmonary arteries

Answer 115

Muscular VSDs: 30% close spontaneously Heart failure: Diuretics Additional calorie input Surgery: prevents pulmonary capillary bed damage due to pulmonary HTN and high blood flow

Answer 116

Trisomy 21

Answer 117

Poor feeding Tachypnoea Hepatomegaly Oedema Failure to thrive No murmur Thrill Gallop rhythm Present at antenatal US scan Cyanosis at birth/ heart failure at 2-3 weeks Often detected by echo check up in Down's

Answer 118

Treat heart failure Surgical repair: at 3-6 months

Answer 119

Arterial duct tissue encirculing the aorta at the point it inserts into the duct: 1) Constricts aorta when duct closes 2) Obstruction to LV outflow

Answer 120

When ductus arteriosus closes; acute circulatory collapse: Weak femoral pulses Radio-femoral delay Ejection systolic murmur Difference in pre and post-ductal saturations Heart failure Metabolic acidosis

Answer 121

4 limb BP CXR: cardiomegaly

Answer 122

Surgical repair: by 5 years old

Answer 123

Aortic valve leaflets partly fused together- restricted exit from LV

Answer 124

- Ejection systolic murmur: URSE! - Palpable carotid thrill in suprasternal region - LVH - Critical AS: severe heart failure

Answer 125

Radiograph: prominent LV ECG

Answer 126

- Regular echos - Balloon valvotomy/dilation - Valve replacement: surgery

Answer 127

Pulmonary valve leaflets partly fused together which restricts exit from the RV

Answer 128

Systolic ejection murmur in ULSE that can radiate to the back Right ventricular heave

Answer 129

ECG: upright T wave/ RV hypertrophy CXR

Answer 130

Transcathether balloon dilation

Answer 131

1) Aorta connected to RV 2) Pulmonary artery connected to LV (deoxygenated blood to body) 3) Incompatible with life (often have naturally occurring ASD,VSD, PDA)

Answer 132

- Cyanosis - S2: loud and singular - Reduced sats

Answer 133

CXR: narrow upper mediastinum 'egg on side' appearance of cardiac shadow Echo: abnormal arterial connections Check for 22q deletion

Answer 134

Maintain patency of ductus arteriosus: prostaglandin infusion Balloon atrial septostomy Surgery: arterial switch procedure in first few days of life

Answer 135

Down's syndrome 22q deletions

Answer 136

1) VSD 2) Overriding aorta 3) Pulmonary stenosis 4) RVH

Answer 137

- Cyanotic - Breathlessness - Acidosis - Collapse

Answer 138

Echo CXR: small, up tilted, boot shaped apex Harsh systolic murmur: LLSE

Answer 139

Close VSD and relieve right ventricular outflow tract obstruction: shunt to increase pulmonary blood flow

Answer 140

Posterior leaflets of tricuspid valve displaced anteriorly

Answer 141

Lithium in pregnancy (e.g bipolar mum)

Answer 142

Tricuspid regurgitation Tricuspid Stenosis RA enlargement

Answer 143

Pan-systolic

Answer 144

Mid-diastolic

Answer 145

Coarctation of the aorta Aortic stenosis Aortic dissection

Answer 146

AVSD Tetralogy of Fallot VSD

Answer 147

AVSD Tetralogy of Fallot VSD

Answer 148

Idiopathic systemic vasculitis that most commonly effects children aged 6 months-5 years

Answer 149

Coronary artery aneurysm formation

Answer 150

MyHEART: Mucosal Involvement:-inflamed dry lips/ strawberry tongue Hand and feet swelling Eye: bilateral conjunctivitis lymphAdenopathy (cervical) Rash Temperature: >5 days of high° fever

Answer 151

1) Acute febrile (1-2 weeks) Fever + 4 of criteria (MyHEART) 2) Subacute: remission of fever (4-6 weeks) development of coronary artery aneurysms 3) Convalescent (6-12 weeks) Resolution of clinical signs + normalisation of inflammatory markers

Answer 152

Echo is essential: coronary artery pathology FBC: - High ESR, CRP WCC - High Platelets LFT: high AST and hypoalbuminemia High a1-antitrypsin Bilirubin High Urinalysis: raised WCC without infection

Answer 153

Aspirin (reduce the risk of aneurysms and thrombosis) IV Immunoglobulins Echo: 6 weeks later to check for aneurysms Infliximab (anti-TNF): if permanent inflammation

Answer 154

HSP is an IgA mediated, autoimmune hypersensitivity vasculitis of childhood (Skin, joint, gut, kidneys)

Answer 155

Infections (group A strep, mycoplasma and EBV) Vaccinations Exposure to allergens, cold and pesticides Insect bite

Answer 156

- Fever - Rash - Abdominal pain/symptoms - Renal involvement - Arthritis

Answer 157

Urinanalysis: protein/haematuria Raised ESR Raised serum IgA Raised WCC Increased anti-streptolysin O titrates: detects Group A Strep

Answer 158

1) Purpura (non-blanching) 2) Arthritis/arthralgia 3) Abdominal pain

Answer 159

NSAIDs: joint pain Corticosteroids: arthralgia Most recover in 2 months

Answer 160

Infection of valves/endocardium → destruction due to infective organisms forming vegetation

Answer 161

S. viridans

Answer 162

IV drug users Prosthetics Structural heart defects

Answer 163

Any kid with fever and significant cardiac murmur Weight loss Night sweats Anaemia: unexplained Petechiae Clubbing Splinter haemorrhages (due to small septic emboli) Osler’s nodes (nodules from inflammation) Janeway lesion (flat from small septic emboli)

Answer 164

Blood cultures: take 3+ samples within first 24h in hospital Echo: confirms presence of vegetations High ESR: (monitors treatment)

Answer 165

Abx: **Empirical**: Amoxicillin (rifampicin if prosthetic valve) + gentamycin + vancomycin **Once known** Streps: IV penicillin and gentamycin for 6 weeks Valve replacement/debridement

Answer 166

Meningococcal disease: septicemia

Answer 167

Systemic febrile illness + Pharyngitis (occurs 2-4 weeks after infection) Autoimmune condition triggered group A B-haemolytic strep

Answer 168

1/2 major + 2 minor plus evidence of preceding strep infection (scarlet fever/ throat swab/ high serum ASO antibody titre, ECHO, ECG or CXR) JONES FEAR: mnemonic of criteria

Answer 169

JONES FEARP: JONES: Major - Joint arthritis: polyarthritis - Organ inflamation: Carditis(+ve echo, changed murmur, CCF, cardiomegaly) - Nodules subcutaneous - Erythema marginatum - Sydenham's chorea- neurological manifestation FEARP: Minor - Fever - ECG PR interval <0.2s - Arthralgia pain - Raised ESR/ CRP - Previous rheumatic fever/ rheumatic heat disease

Answer 170

Aspirin + Prednisolone: carditis (be careful of Reye's syndrome) Benzylpenicillin → Penicillinn V 10 days: Pharyngitis NSAIDS: joint pain Prednisolone: synderham's Chorea

Answer 171

Highly infectious disease caused by varicella zoster virus (VZV)

Answer 172

Reactivation of dormant virus after a bout of chicken pox leads to herpes zoster (shingles) in posterior root ganglia

Answer 173

- Immunocompromised - Older age - Steroid use - Malignancy - Dangerous in neonates and to the foetus if contracted in pregnancy

Answer 174

Infective from 4 days prior to rash until all lesions have scabbed (day 5)

Answer 175

- T 38-39° - Headache - Malaise - Coryzal symptoms - Crops of vesicles (itchy)

Answer 176

Mostly on the head, neck and trunk Very sparse on the limbs

Answer 177

1) Macule 2) Papule 3) Vesicle (red surround) 4) Ulcers 5) Crust

Answer 178

Bacterial superinfection

Answer 179

- Shingles: only one dermatome distribution - Generalised herpes zoster/ simplex - Dermatitis herpetiformis - Impetigo

Answer 180

- Clinical - Fluorescent antibody tests: IgM and IgG

Answer 181

Calamine lotion Antivaricella: zoister immunoglobulin Acyclovir: if high risk Flucloxacillin: in bacterial superinfection 5 days off school for kids

Answer 182

Acute viral infection caused by single stranded RNA morbillivirus from the paramyxovirus family

Answer 183

Respiratory droplets

Answer 184

From prodrome until 4 days after the rash of measles appear

Answer 185

RNA Morbillivirus from the paramyxovirus

Answer 186

1) Incubaction period: usually asymptomatic 2) Prodrome: pyrexia, malaise, anorexia, conjunctivitis, cough, coryza and koplik spots (blue-white spots on inside of mouth opposite to molars) 3) Exanthem: Erythematous maculopapular non-itchy rash which becomes blotchy and confluent (lasts 3 days and leaves behinds a brown discolouration) + High fever

Answer 187

IgM & IgG positive Salivary swab/serum sample: for measles specific immunoglobulin (within 6 weeks of onset) Salivary swab: RNA detection

Answer 188

Paracetamol/ Ibuprofen + fluids

Answer 189

More common if <5 years or >20 years: - Otitis media - Croup/ tracheitis - Pneumonia - Encephalitis

Answer 190

7-13 years post measles: chronic complication Progressive changes in behaviour, myoclonus, dystonia, dementia → death

Answer 191

Miscarriage, prematurity and low birthweight

Answer 192

Disease of streptococcus pyogenes (group A strep) exotoxins usually following tonsillitis

Answer 193

<10 yr olds Unusual in <2 yr olds

Answer 194

- Acute tonsillitis and fever → rash 24-48 hours after - Strawberry tongue - Flushed face - Cervical lymphadenopathy - Scarletiniform rash: Red, 'pin-prick' blanching sandpaper rash on chest outwards

Answer 195

Clinical Throat swab Antigen detection kits: - Strep antibody tests

Answer 196

Penicillin/ Azithromycin: 10 days Rest Fluids Paracetamol/ibuprofen

Answer 197

- Syndenhams Chorea - Otitis media - Rheumatic fever - Glomerulonephritis

Answer 198

RNA virus: Rubivirus Togaviridae

Answer 199

Inhaled droplets

Answer 200

14-21 days

Answer 201

5 days before and 5 days after start of rash

Answer 202

- Lethargy - Low grade fever - Headache - Mild conjunctivitis - Anorexia - Pink-red maculopapular rash - Suboccipital lymphadenopathy

Answer 203

PCR testing Saliva: rubella IgM in saliva FBC: low WBC, high lymphocytes and thrombocytopenia

Answer 204

MMR vaccine Paracetamol: for fever

Answer 205

When a mother is infected with rubella during pregnancy

Answer 206

Weeks 1-4 = blindness (congenital cataracts + glaucoma) Weeks 4-8 = congenital heart disease (PDA) Weeks 8-12 = deafness

Answer 207

Parvovirus B19 transmitted through droplets

Answer 208

≈1 week: - Mild - Headache, rhinitis, sore throat, fever, malaise THEN l ≈1 week asymptomatic

Answer 209

Classic 'slapped cheek' rash: erythematous macular morbilliform rash develops on the limbs 1-4 days after the facial rash Arthralgia

Answer 210

B19 specific IgM: current or recent infection B19 specific IgG: immunity PCR

Answer 211

Conservative

Answer 212

Acute superficial bacterial skin infection

Answer 213

Well defined lesions Honey/golden coloured crusts on erythematous base Systemic symptoms: Lymphadenopathy, mild fever and malaise

Answer 214

Topical fusidic acid Oral flucloxacillin: if severe

Answer 215

Inflammation of the leptomininges

Answer 216

Neonates: GBS, E.coli, lysteria monocytogenes. 1m-6 years: Neisseria meningitidis, S.pneumoniae, H.influenzae. > 6 years: Neisseria meningitidis.

Answer 217

- Immunocompromised - Crowding - Endocarditis - DM - Alcoholism and cirrhosis - IV drug abuse - Renal/adrenal insufficiency - Malignancy - Sickle cell disease

Answer 218

BACTERIAL: 1) The pia-arachnoid is congested with polymorphs causing a layer of pus to form VIRAL: 1) Lymphocytic inflammatory CSF without pus formation 2) No polymorphs/ adhesions 3) Little or no cerebral oedema unless encephalitis develops

Answer 219

Common in infants, children or elderly

Answer 220

Viral > Bacterial

Answer 221

Septic signs occur before meningeal signs: -Malaise, fever, headache, temperature, rigor, vomiting - Tachycardia - Tachyponea - Hypotension - Poor feeding - Abnormal cry Meningeal signs are late and less common in young children: Classic= headache + fever + neck stiffness + photophobia + Malaise, fever, headache, temperature, rigor, vomiting +ve Kernigs (resistance to extending knee when hip is flexed) +ve Brudzinki's (neck flexion = hip flexion)

Answer 222

DO NOT DELAY TREATMENT OVER INVESTGATIONS Blood Cultures EDTA blood for PCR Throat swabs - Lactate - FBC - Glucose - U&Es **LP immediately:** **Appearance:** Bacteria: cloudy Virus: normal **Protein:** Bacteria: high Virus: mildly raised or normal **Glucose:** Bacteria: low Virus: normal **WCC:** Bacteria: high and PMN Virus: high and lymphocytes **Culture and sensitivity:** Bacteria: positive Virus: positive

Answer 223

High flow O2 Abx: <3m= Cefotaxime + Amoxicillin + Aciclovir >3m= Ceftriaxone + Dexamethasone (reduces inflammation) In GP = Benzylpenicillin (Cefotaxime if allergic)

Answer 224

Viral illness commonly causing lesions involving the hands, foot and mouth

Answer 225

Feacal-oral route

Answer 226

Coxsackievirus A16 Enterovirus 71

Answer 227

<10 yrs old Outbreaks common in nurseries, schools and childcare centres

Answer 228

- Fever - Malaise - Loss of appetite - Sore mouth/throat - Cough - Abdo pain

Answer 229

On buccal mucosa, tongue or hard palate Begin as macular lesions that progress to vesicles which then erode Yellow ulcers surrounded by red haloes

Answer 230

Palms, soles and between fingers/ toes Erythematous macules but rapidly progress to grey vesicles with an erythematous base

Answer 231

Clinical diagnosis Swab lesions PCR

Answer 232

Fluid intake Soft diet Paracetamol/Ibuprofen If mouth is very painful, use topical agents e,g, lidocaine oral gel Stay off school until better

Answer 233

Inflammation of brain parenchyma Usually caused by infection (normally viral)

Answer 234

- Flu like prodrome: often self-limiting - Reduced GCS - Odd behaviour - Vomiting - Focal neurological symptoms - Fits/ focal seizures - Fever - Meningism symptoms - Headache

Answer 235

- CSF, MC&S and PCR - Bloods - Stool (enteroviruses) - Urine - LP

Answer 236

Anciclovir: if HSV suspected/confirmed Dexamethasone: treats high ICP

Answer 237

A granulomatous disease caused by Mycobacterium tuberculosis

Answer 238

Inhaled droplets

Answer 239

Overseas contact HIV +ve Odd CXR

Answer 240

Anorexia Low fever Failure to thrive Malaise **Cough +/- haemoptysis** **Erythema nodosum** **Lymph nodes**: a cold abscess (firm, painless and no red inflammation) **Constitutional symptoms** Spinal TB: pain Dyspnea: gradually as lungs become damaged Clubbing Lupus vulgaris: apple jelly nodules Auscultation: possible crackles Haematuria, increased frequency and nocturia Pyuria in the absence of a positive culture = sterile pyuria

Answer 241

3x sputum culture + Ziehl-Neelsen stain smear on Lowenstein Jensen medium: will turn red if acid-fast bacilli Chest X-ray: millet seeds, consolidation, hilar lymphadenopathy and pleural effusions Tuberculin tests Mantoux Test: if latent - < 6mm or >15mm =give BCG Interferon-Gamma Release Assays (IGRAs): if latent

Answer 242

Rifampicin (6 months) Isoniazid (6 months) Pyrazinamide (2 months) Ethambutol (2 months) BCG vaccine: if high risk

Answer 243

Limited protection against TB and leprosy in high risk groups

Answer 244

- Previous BCG vaccination - Hx TB - HIV - Pregnancy - + tuberculin test - NO EVIDENCE FOR EFFICACY >35

Answer 245

1) Pleural effusion 2) Pericardial effusion 3) Lung collapse 4) Lung consolidation

Answer 246

Hepatitis “red-an-orange-pissin and tears” Flu like symptoms Impaired COCP function

Answer 247

Hepatitis I’m-so-numb-azid: paraesthesia Agranulocytosis

Answer 248

Hyperuricaemia – GOUT Arthralgia Myalgia Hepatitis

Answer 249

“Eye-thambutol”: colour blindness (Optic Neuritis)

Answer 250

Normal treatment + Isoniazid for 6 months

Answer 251

A retrovirus (RNA virus) that infects cells of the immune system

Answer 252

Two strains: HIV-1 (more common) HIV-2

Answer 253

Infected bodly fluids: 1. Sexual**:** most cases. MSM have a higher risk 2. Parenteral: via needlestick or needle sharing 3. Vertical**:** via breastfeeding or vaginal delivery

Answer 254

**Acute infection:** Asymptomatic or flu-like illness **Clinical latency:** May be asymptomatic or present with non-AIDS defining illnesses: Fever and night sweats Persistent lymphadenopathy Parotid enlargement Hepatosplenomegaly Persistent diarrhoea Reduced platelets Weight lost Opportunistic infections: e.g. thrush **AIDS:** AIDS-defining illness

Answer 255

AIM: Before 1st birthday Tests at 3&6 months: - HIV viral PCR - ELISA: HIV antibody, p24 antigen test and IgA Monitor CD4 T-cell count and viral load

Answer 256

**Treatment aim:** normal CD4 count and undetectable viral load Highly Active Anti-Retrovirus therapy (HAART) Prophylactic co-trimoxazole Pre+post exposure prophylaxis: zidovudine Vaccinations Condoms and regular HIV tests Pre-test and post-test counselling

Answer 257

Those with AIDs defining conditions/CD4 <15%

Answer 258

S. aureus + Group A strep Toxin acts is a super antigen that can cause organ dysfunction

Answer 259

- Fever >39° - Hypotension - Diffuse erythematous, macular rash

Answer 260

Intensive care Infected areas: surgically debrided Abx: Ceftriaxone + Clindamycin

Answer 261

Cavernous haemangioma Self limiting Concerning if over eye and in airway

Answer 262

Permanent capillary haemangioma Present in Sturge-Weber Syndrome

Answer 263

Too many capillaries in the meninges Area of face innervated by trigeminal nerve affected by Port Wine Stain

Answer 264

Non- caucasian ancestry Congential dermal melanocytosis Lower back/ buttocks

Answer 265

Flat light brown patches on the skin >5 = Neurofibromatosis

Answer 266

Sebaceous plugs from sweat glands

Answer 267

'Nettle-like' rashes, raised and itchy patches Allergen exposure linked to child abuse if late presentation, symmetry and odd history

Answer 268

Erythema toxicum neonatorum: benign red blotches that resolve spontaneously following birth Histamine reaction

Answer 269

Dangerous in immunocompromised

Answer 270

- Cough - Coryza - Conjunctivitis - Koplik spots (inside mouth)

Answer 271

Slapped cheek

Answer 272

Hand, foot and mouth

Answer 273

Prodromal malaise Parotids Orchitis Infertility

Answer 274

Pox like nodules that last for months Harmless

Answer 275

Acanthosis nigricans

Answer 276

Cat faeces infected with toxoplasma gondii

Answer 277

- Intracranial calcification - Microcephaly/hydrocephalus - Chorioretinitis - Cerebral palsy

Answer 278

Serology: >95% asymptomatic

Answer 279

Pyrimethamine + Sulphadiazine + Spiramycin

Answer 280

Infection associated with eating dog faeces Leads to blindness

Answer 281

Cat: Toxoplasmosis (fits, deafness) Dog: Toxocara (acquired blindness)

Answer 282

Infection to the developing foetus/ newborn by any of: Toxoplasmosis Other agents ( Syphillis, Varicella- zoster and Parvovirus B19 Rubella (german measles) Cytomegalovirus Herpes simplex

Answer 283

Cytomegalovirus (CMV)

Answer 284

- Growth retardation - Microcephaly - Hepatosplenomegaly - Hepatitis - Anaemia/jaundice/ thrombocytopenia - IUGR - Chorioretinitis - Motor and cognitive impairment

Answer 285

Benzylpenicillin

Answer 286

Blindness Low IQ Epilepsy Jaundice Respiratory distress 30% die even when treated

Answer 287

Chronic atopy leading to epidermis inflammation: includes atopic dermatitis and contact dermatitis

Answer 288

15-20% of children Increasing incidence

Answer 289

Emollients Topical corticosteroids Antihistamines Phototherapy Systemic immunosuppression

Answer 290

Severe infection of skin (HSV 1+2) Potentially life threatening More common in kids with eczema

Answer 291

Epidermal necrosis due to hypersensitivity

Answer 292

- Sulfonamides - Anti-epileptics - Penicillin - NSAIDs

Answer 293

Painful erythematous macules Severe mucosal ulceration

Answer 294

Steroids Immunoglobulins Immunosuppressant

Answer 295

Type 1 hypersensitive reaction: IgE simulates mast cell degranulation Life threatening

Answer 296

Foods Insect sting Drugs Latex Exercise Inhaled allergens

Answer 297

Mainly nuts

Answer 298

Occurs within minutes of drug exposure and lasts 1-2 hours **Flushing:** vasodilation **Angio-oedema**: increased vascular permeability- fluid centrally shifted peripherally **Central cyanosis** **Wheeze, dyspnea:** bronchoconstriction **Urticaria** **Anaphylactic shock** **Cardiac arrest**

Answer 299

ABCDE ECG Mast cell tryptase ABG

Answer 300

ABCDE IM Adrenaline: second dose after five minutes if no response - <6 months: 0.01mg - 6m-6 years: 0.15mg - 6-12 years: 0.3mg - >12 years: 0.5mg If anaphylactic shock: IV adrenaline IV Antihistamine (chlorphenamine 10mg) IV HydrocortisoneSalbutamol if wheeze IV fluids High flow O2 Monitor pulse oximetry, ECG, BP

Answer 301

Poor growth rate or weight falling 2 centile lineS

Answer 302

Inadequate calorie intake: - Poor breastfeed technique - Poverty - Unsuitable food offered - Neglect

Answer 303

1) Gross motor 2) Fine motor and vision 3) Speech, language and hearing 4) Social

Answer 304

Following child over time: incorporated into well-child checks, general physical examination and routine immunisation visits

Answer 305

Along normal route, but takes longer to reach milestones

Answer 306

Does not follow normal pattern Impairment

Answer 307

Biological: - Prematurity - Low birth weight - Birth asphyxia - Hearing/vision impairment Environmental: - Poverty - Poor parental education - Maternal alcohol/drugs

Answer 308

A M L PPPP R S T: Atonic neck reflex Moro reflex Landau reflex Plantar grasp reflex Palmar grasp reflex Parachute reflex Positive support reflex Rooting reflex Stepping reflex Trunk incurvation

Answer 309

Touch hand/soles and baby will grasp/curl toes

Answer 310

Palmar = until 6 months Plantar = until 9-12 months

Answer 311

1) Hold baby upright with one sole on table top 2) Hip and knee will flex and other foot step forward

Answer 312

1) Hold baby supine and abruptly lower the body about 2 feet 2) Arms will abduct/extend and legs flex

Answer 313

Until 2 months

Answer 314

1) baby supine, turn head to one side and hold jaw on shoulder 2) Arms/legs the heads turned to will extend and opposite will flex

Answer 315

Until 6 months

Answer 316

1) Stroke perioral skin at corner of the mouth 2) Mouth will open and baby will turn head toward stimulus and suck

Answer 317

Until 4 months

Answer 318

1) Support baby prone and stroke one side of the back 2) Spine will curve towards stimulated side

Answer 319

0-2 months

Answer 320

1) Suspend baby prone 2) Head will lift up and the spine will straighten

Answer 321

0-6 months

Answer 322

1) Suspend baby prone and slowly lower the head towards a surface 2) Arms and legs will extend in a protective fashion

Answer 323

8 months onwards

Answer 324

1) Hold baby upright until feet touch a surface 2) Hips, knees and ankles will extend and partially bear weight for 20/30 seconds

Answer 325

0-6 months

Answer 326

- Parachute - Positive support - Landau - Neck/head righting reflexes - Lateral propping

Answer 327

Motor neuron abnormalities in infants

Answer 328

Physical or mental impairment preventing children from going about daily life

Answer 329

1) Down's syndrome 2) Cerebral Palsy

Answer 330

- A permanent neurological problem resulting from damage to the brain around the time of birth - Lesions are non-progressive, but manifest clinically overtime

Answer 331

- Cognition - Communication - Perception - Sensation - Behaviour - Seizures

Answer 332

Antenatal e.g vascular occlusion/congenital infection (CMV, toxoplasmosis or rubella) (majority) Birth asphyxia/trauma

Answer 333

- Meningitis/encephalitis/encephalopathy - Head trauma - Intraventricular haemorrhage

Answer 334

- Abnormal limb/trunk posture and tone - Delayed motor milestones - Abnormal gait

Answer 335

- Feeding difficulties - Learning difficulties - Epilepsy - Language/speech difficulties - Persistent primitive reflexes

Answer 336

Gross Motor Function Classification System (GMFCS)

Answer 337

Gross Motor Function Classification System I-III: - Measures child's functional ability following diagnosis of cerebral palsy

Answer 338

Level 1: walks without limitation Level 2: walks with limitations Level 3: walks using handheld mobility device Level 4: self mobility with limitations (may use powered mobility) Level 5: manual wheelchair transportation

Answer 339

Clinicaly MRI: identifies cause

Answer 340

- Spastic (majority) - Dyskinetic - Ataxic - Mixed

Answer 341

- Damage to UMN pathway - Limb tone persistently increased (spastic) - Brisk deep tendon reflexes - Clasp knife reflexes

Answer 342

1) Hemiplegia 2) Diplegia 3) Quadriplegia

Answer 343

- Seizures - Low IQ - Swallowing difficulties

Answer 344

Movements of involuntary control Muscle tone variable Chorea Athetosis Dystonia

Answer 345

Jerky movements

Answer 346

Slow writhing movements distally: e.g. fanning fingers

Answer 347

Twisting appearance from simultaneous contraction of agonist and antagonist muscle groups

Answer 348

Cerebellum damage

Answer 349

- Poor balance - Delayed motor development - Hypo/hypertonia - Uncoordinated, intention tremor and ataxic gait (evident later on)

Answer 350

Lack of voluntary co-ordination of muscle movements that can include ataxic gait, speech and abnormal eye movements

Answer 351

Basal ganglia damage

Answer 352

Oral Diazepam Baclofen Botulinum toxin (botox): relaxes muscles

Answer 353

- Physiotherapist - Speech Therapist - Occupational Therapist - Orthoptist/ Audiologist - Dietitian - Social worker

Answer 354

Trisomy 21

Answer 355

FHx Old maternal age: >35

Answer 356

Deafness Blindness Hypothyroidism Dementia Learning disability Hypotonia Short stature CHD Atlantoaxial instability ALL

Answer 357

- Epicanthic folds (inner corner of eye) - Protruding tongue - Small low set ears - High arched palate - Palpebral fissures (eyes at angle) - Single palmar crease - Brachycephaly (flat head) - Brushfield spots (white spots in eyes)

Answer 358

Trisomy 18

Answer 359

Severe psychomotor + growth retardation in those that survive 1st year of life: - Micrognathia - Low set ears - Rocker bottom feet - Overlapping of fingers - Learning disabilities

Answer 360

Severe physical and mental congenital abnormalities due to Trisomy 13 Can cause Holoprosencephaly

Answer 361

Microcephalic Microphthalmia (one small eye) Hypotelorism (decreased distance between eyes) Cleft lip/palate Scalp lesions Rocker bottom feet CHD IUGR and low BW Severe learning difficulties

Answer 362

Brain doesn't divide into two halves

Answer 363

Death within 7-10 days

Answer 364

Only one copy of a gene is expressed

Answer 365

Angelmans syndrome Prader Willi syndrome

Answer 366

Absence of one member of a pair of chromosome

Answer 367

Turner's syndrome

Answer 368

Downs Edwards Pataus XXX syndrome Klinefelter's

Answer 369

Huntingtons Neurofibromatosis Hereditary spherocytosis Marfans

Answer 370

CF Albinism PKU (phenylketouria)

Answer 371

Duchenne's muscular dystrophy Red-green colour blindness G6PD deficiency Haemophilia A/B

Answer 372

Loss of functional genes on the proximal arm of the chromosome 15 inherited from the father First human disorder attributed to genomic imprinting

Answer 373

Deletion in the paternally inherited chromosome 15 or maternal uniparental disomy 15 OPPOSITE TO ANGELMAN'S

Answer 374

Infant: Genital hypotonia, development delay and failure to thrive/ poor feeding, and blue eyes blonde hair Adolescence: Obesity, learning disabilities, behavioural difficulties and hyperphagia (always hungry)

Answer 375

Raised ghrelin

Answer 376

Growth Hormonea Anti-psychotics: Olanzapine SSRI's

Answer 377

Genetic imprinting disorder Due to maternal deletion of chromosome 15 OPPOSITE OF PRADER WILLI

Answer 378

**Fascination with water Happy demeanour Dysmorphic features: widely spaced teeth** Learning disability Speech impairment Ataxia (broad based gait) Strabismus Drooling Epilepsy Microcephaly V similar to autism

Answer 379

Chromosomal analysis Fluorescence in situ hybridisation (FISH): deletions

Answer 380

Behavioural modification programmes Speech therapy Physiotherapy Parental education Anti-convulsants for Epilepsy: valproate/ clonazepam

Answer 381

Loss/abnormality of the second X chromosome (45 XO)

Answer 382

Increased risk of CHD Renal malformations Hearing loss Osteoporosis Obesity Diabetes Atherogenic lipid profile

Answer 383

**Short stature Webbed neck Cubitus valgus (increased carrying angle) Widely spaced nipples Almost all infertile Delayed puberty Ovarian failure** Low posterior hairline High arching palate Recurrent otitis media CV and renal malformations

Answer 384

Autoimmune conditions: Thyroid Diabetes Coeliac Crohn's

Answer 385

- Amniocentesis or chorionic villous sampling - Chromosomal analysis

Answer 386

Short stature: recombinant human growth hormone Oestrogen + progesterone (12 years): to initiate puberty, prevent osteoporosis and regulate the menstrual cycle Fertility treatment

Answer 387

Autosomal dominant mutations in the RAS/ MAPK pathway 60% are new spontaneous mutations

Answer 388

Learning disability Cryptorchidism Pectus excavatum Pulmonary stenosis Short stature Broad forehead Downward sloping eyes with ptosis Hypertelorism (wide space between the eyes) Prominent nasolabial folds Low set ears Webbed neck Widely spaced nipples

Answer 389

Autosomal dominant disorder of benign neuromas that encompasses NF1, NF2 and Schwannomatosis

Answer 390

NF1 gene defect on chr 17: Skin lesions More common then NF2

Answer 391

NF2 gene defect on chr 22: bilateral acoustic neuromas (schwannomas, meningiomas and ependymonas) Less common

Answer 392

2/7 = diagnosis ** CRABBING** **C**afé-au-lait spots (6+) (>5mm kids, >15mm adults) **R**elative with NF1 **A**xillary or inguinal freckles (skin folds) **B**ony dysplasia e.g. **B**owing of a long bone or sphenoid wing dysplasia (absence of bone around eyes) **I**ris hamartomas (Lisch nodules yellow-brown spots in eyes) (2 or more) are yellow brown spots on the iris **N**eurofibromas (2 or more) or 1 plexiform neurofibroma **G**lioma of the optic nerve

Answer 393

Hearing loss Tinnitus Balance problems Bilateral schwannomas

Answer 394

Rarely >6 cafe-au-lait spots in NF2

Answer 395

Intervene when tumours produce pressure symptoms or suggestive of malignant change Surgery

Answer 396

Mutation in the FMR1 (fragile X mental retardation 1) gene on the X chromosome

Answer 397

FMR1 gene includes a CCG repeat As its passed from each generation it lengthens Once it reaches >200 no fragile X protein is made TRINUCLEOTIDE REPEAT DISORDER

Answer 398

Fragile X syndrome

Answer 399

Delayed speech + language Macro-orchidism Macrocephaly Large ears Long narrow face Hypermobile joints ADHD Seizures Autism

Answer 400

Molecular genetic testing of FMR1 gene

Answer 401

Minocycline: improves behaviour

Answer 402

Additional X chromosome (47XXY) Chief genetic cause of hypogonadism

Answer 403

Gyaecomastia Azoospermia Tall Macro-orchidism Reduced pubic hair

Answer 404

Testosterone injections Advanced IVF techniques: fertility Mastectomy (gynaecomastia)

Answer 405

Neurodevelopmental disorder of social interaction, repetitive behaviour and communication impairments

Answer 406

Abnormal development manifesting before the age of 3

Answer 407

1) Reciprocal social interaction 2) Impairment of language and communication 3) Restricted, repetitive behaviour

Answer 408

- Communication difficulties - Social interaction - Difficulties with imagination/ rigidity of thought

Answer 409

- No desire to interact with others - Oblivious to others feelings - No understanding of unspoken social rules - Lack of empathy - Poor eye contact

Answer 410

Restrictive/ repetitive movements: self-stimulating e.g. hand-Flapping or rocking Obsessive fixations Inability to play/write imaginatively Same questions

Answer 411

Repeats speech Disordered language Poor non-verbal communication No social awareness, unable to start up or keep a conversation

Answer 412

Epilepsy Visual and hearing Mental health (ADHD, depression and anxiety)

Answer 413

Early behavioural intervention Education for parents Support in schools Risperidone: aggression/irritability Melatonin: sleep difficulties SSRI's: repetitive behaviours

Answer 414

Risperidone

Answer 415

Pervasive development disorder which lies within the autistic spectrum.

Answer 416

Boys:Girls 8:1

Answer 417

- Lack of delayed cognition and language - Aspergers are above average intelligent - Aspergers are more likely to seek social interaction and share activities/ friendships

Answer 418

- Obsessed with complex subjects - Concrete thinking - Pedantic - Normal speech - Clumsiness - Solitary but socially aware - Poor sleep patterns

Answer 419

Persistant and extreme hyperactivity and attention deficit

Answer 420

Prematurity Fetal alcohol syndrome

Answer 421

- Hyperactivity - Inattention - Impulsivity

Answer 422

1) Present before 12 2) Developmentally inappropriate 3) Severel symptoms in multiple settings 4) Clear effect on social/academic/ occupational functioning

Answer 423

6/9: inattentive 6/9: hyperactive/impulsive symptoms

Answer 424

1) Easily distracted 2) Does not appear to be listening when spoken to 3) Has difficulty sustaining attention 4) Avoids/ dislikes sustained mental effort 5) Forgetful in daily activities 6) Difficulty following instructions/ fails to complete tasks 7) Difficulty organising tasks 8) Careless mistakes/ lack of attention to detail 9) Loses important items

Answer 425

1) Blurts out answers 2) Has difficulty awaiting turn 3) Interrupts / intrudes others

Answer 426

1) Squirms/ fidgets 2) Cannot remain seated 3) Runs/ climbs in inappropriate situations 4) Often 'on the go' 5) Talks excessively 6) Cannot perform leisure activities quietly

Answer 427

Diet and exercise Methylphenidate (ritalin); 6 week trial + monitor growth

Answer 428

Sudden reduction in cerebral perfusion with oxygenated blood

Answer 429

Transient occurence of signs/symptoms due to abnormal excessive neuronal activity in the brain

Answer 430

Diazepam or Lorazepam if >5 min

Answer 431

Seizure: Flashing lights/ hyperventilation Syncope: Upright, exertion, blood, needles

Answer 432

Seizure: Typical aura Syncope: Nausea, sweating or palpitations

Answer 433

Seizure: Variable Syncope: 1-30s

Answer 434

Seizure: Common + prolonged Syncope: Less common + brief

Answer 435

Seizure: Common Syncope: Uncommon

Answer 436

Seizure: Common Syncope: rare

Answer 437

Seizure: Partial- pale Tonic clonic- red,blue Syncope: Very pale

Answer 438

Seizure: Yes + slow + confused Syncope: No + rapid orientation

Answer 439

Seizures occurring in children aged 6m-6 years with fever and raised temperature (>37.8°)

Answer 440

- Generalised tonic-clonic - <15 mins - Occurs once during illness - Should be recovered/drowsy within 1 hr

Answer 441

Focal or partial seizures May reoccur during same febrile illness >15 mins

Answer 442

- Metabolic (electrolytes/glucose) - Viral (meningo) - CNS lesion - Epilepsy - Trauma

Answer 443

1) First febrile seizure 2) >5 min duration 3) Drowsy >1 hour after seizure 4) Previous history of: - <18 months - Complex seizure - On Abx

Answer 444

- Reduced consciousness - Septicaemic shock - Likely invasie meningococcal - Signs of raised ICP - Focal neurology - Bleeding tendency

Answer 445

To make sure you don’t have a bloody tap

Answer 446

Paroxysmal, self limiting brief asystole (<15s) that occurs in infants (6m- 2yrs) due to common triggers such as: - Pain - Cold foods - Fright - Fever

Answer 447

When a child is startled, strong signals from the vagus nerve cause the heart to stop beating

Answer 448

Stops breathing Stiff/rigidity Incontinence Pale/blue NO tongue bite

Answer 449

Check ferritin + treat No drugs needed Pacemaker Child usually grows out of it

Answer 450

Tendency to have seizures: transient episodes of abnormal electrical activity in the brain. Symptom: not a true condition Diagnosed after a minimum of 2 seizures

Answer 451

Usually none found Infection Hypo (glucose, Na, Ca, Mg) Trauma Metabolic defects CNS tumour Lights Exercise

Answer 452

Generalised (both hemispheres) Focal/Partial (one hemisphere + start in the temporal lobe)

Answer 453

1) Transient LoC <30s 2) Abrupt onset and no motor phenomena apart from eyelid flickering 3) Precipitated by hyperventilation 4) Stare momentarily and stop moving 5) No recall but knows they've missed something 6) 2/3rds female 7) 4-12 yrs old

Answer 454

Absent Myoclonic Tonic Tonic-clonic Atonic

Answer 455

Frontal Temporal Parietal Occipital

Answer 456

Brief, repetitive jerking movements of limbs, neck or trunk

Answer 457

Non-epileptic myoclonic seizure of diaphragm

Answer 458

Tone increase (muscle tensing)

Answer 459

- Rigid - Fall to ground - Stop breathing/cyanosis

Answer 460

- Rhythmic contractions - Limb jerking - Irregular breathing - Saliva/ cyanosis - Tongue bite/incontinence

Answer 461

Deep sleep/unconsciousness for up to several hours

Answer 462

Transient loss of muscle tone causing drop to the floor or drop of head

Answer 463

Motor phenomena (clonic) Asymmetrical

Answer 464

Contralateral altered sensation

Answer 465

- Auditory/ smell/ taste phenomena - Lip smacking/ plucking clothes - Longer seizures - Deja vu

Answer 466

Vision distortion

Answer 467

EEG: within 24 hours MRI head: focal lesions Bloods: Glucose + Electrolytes CK: raised in true epileptics after clonus and tonic seizures vs normal in pseudoseizures

Answer 468

Trigger education 1st line: sodium valproate TERATOGENIC Increases GABA activity Used by most other types as well

Answer 469

Carbamazepine (only epilepsy to use this)

Answer 470

Rectal diazepam

Answer 471

Seizure >30 minutes **OR** Multiple seizures, without regaining consciousness, lasting >30 minutes Brian swells and can cause herniation due to electrolyte imbalance from the firing neurons metabolic demand not being met

Answer 472

ABCDE: - Secure airway - IV access - Check BP - Check temp - Check glucose Drugs: - Lorazepam IV (5 min (Benzodiazepine increases GABA activity)) - Lorazepam IV (15 min) - Phenytoin IV (>20 min) - GA e.g. Propofol (>30 min) + Refer to PICU

Answer 473

1) Infantile spasms 2) Hypsarrhythmia (chaotic EEG) 3) Learning disability

Answer 474

Learning disability Hypopigmented skin lesions Growth restriction Spasms: 5-10 seconds + just before/ after sleep - Clusters of head nodding/trunk jerks

Answer 475

EEG: hypsarrhythmia

Answer 476

Vigabatrin ACTH (daily IM) Prednisolone PROGNOSIS IS POOR

Answer 477

Multi-system hamartomas

Answer 478

TSC1 (chr 9 hamartin) or TSC2 (chr 16 tuberin) Autosomal dominant mutations regulating cell growth

Answer 479

Epilepsy Learning disability Poliosis: white patches of hair Angiofibromas: skin coloured papules over the nose and cheeks Ash leaf spots Cafe-au-lait spots Shagreen patches: thick, dimpled pigemented skin Subungual fibromata

Answer 480

Focal seizures and infantile spasms occur in infancy 2° to tuber formation in the brain

Answer 481

Clinical EEG: epilepsy MRI head: cortical tubers

Answer 482

Treat complications Vigabatrin

Answer 483

X-linked recessive Mutation to gene encoding dystrophin

Answer 484

Strengthens muscle fibres

Answer 485

Duchenne: loss of Dystrophin (more severe) Becker: misshapen dystrophin

Answer 486

- 1-6 yrs old: Duchenne - 10-20yrs old: Becker - Waddling clumsy gait - Calf pseudohypertrophy - Classic gower manoeuvre (on fours getting up) - Respiratory impairment - Wheelchair: 9-12 yrs old - Scoliosis - Osteoporosis

Answer 487

Using hands to crawl up from a sitting position to a standing position

Answer 488

Raised: creatinine kinase Muscle biopsy: - Abnormal fibres surrounded fat/fibrous tissue

Answer 489

Exercise: maintains muscle power and mobility, and delays scoliosis Prednisolone: slows muscle strength and function decline Creatine supplementation

Answer 490

Inflammation from the stomach to the intestines: causes nausea, vomiting and diarrhoea that lasts <14 days

Answer 491

Children= Rotavirus Adults = Noravirus

Answer 492

600k Vaccine is part of the schedule

Answer 493

Poor hygiene Immunocompromised Poorly cooked food

Answer 494

Diarrhoea Blood: likely bacterial Fever, fatigue, headache and myalgia N&V Abdo cramping

Answer 495

Dehydration → shock

Answer 496

**FBC**: **Low MCV:** +/- Fe deficiency **High MCV:** if alcohol abuse/decreased B12 absorption **Raised WCC**: if parasitic cause **Raised ESR + CRP** **U&E:** indicates dehydration **Stool Feacal occult blood + MCS:** Bacteria/parasites/C. Diff **Abdominal x-ray:** toxic megacolon indicates C. difficile

Answer 497

Isolate 48 hrs Oral rehydration (Dioralyte) Avoid sugary drinks Anti-emetics (metoclopramide, 5HT3 Receptor antagonist and 5HT4 agonist) Anti-motility agents (loperamide hydrochloride) Abx Stop C antibiotics: if pseudomembranous colitis

Answer 498

Transient lactose intolerance

Answer 499

1) Lactose-free diet 2) Slowly reintroduce lactose after a few months

Answer 500

- Weight loss - Stools green and frothy - Increased frequency

Answer 501

1) Primary: lactase deficiency 2) Secondary: due to small intestine injury from e.g. infection, coeliac or IBD 3) Post-infective lactose intolerance.

Answer 502

Stool: non-absorbed sugars Hydrogen breath test

Answer 503

Infant who isn't sick or hungry cries for: >3 hours a day >3 days/week >3 weeks

Answer 504

A IgE mediated hypersentivity reaction to milk

Answer 505

Vomiting Abdominal pain Diarrhoea Malabsorption Intestinal bleeding Bloating Allergy symptoms: urticaria and lip swelling Anaphylaxis

Answer 506

RAST test: skin prick blood test to measure IgE specific antibodies in the blood

Answer 507

Breast feeding mothers: avoid dairy products Use special hydrolysed formulas Adrenaline in severe reactions Antihistamines Normally resolved by 5 years old

Answer 508

Most common cause of loose stools in preschool children

Answer 509

Intestinal motility delay Often undigested vegetables + mucus

Answer 510

Improve diet: High fat (whole milk) Normal fibre Reduce fruit juice

Answer 511

Gluten sensitive enteropathy: damaging immunological response to proximal small intestine mucosa

Answer 512

Anti-endomysial IgA (Anti-EMA IgA) Anti-tissue transglutaminase (Anti-TTG IgA)

Answer 513

HLA DQ2 DQ8

Answer 514

Downs T1DM Autoimmune thyroid disease

Answer 515

**GLIAD:** **GI** Malabsorption **Failure to thrive in children** Fatigue and weakness Diarrhoea Abdominal pain **L**ymphoma and Carcinoma Enteropathy-associated T-cell lymphoma Small bowel adenocarcinoma **I**mmunulogical IgA deficiency T1DM Primary biliary cholangitis **A**phthous ulcers **A**naemia Hyposplenism Iron deficiency **D**ermatological **D**ermatitis herpetiformis (itchy rash in response to gluten) **D**ental enamel defects

Answer 516

Oesophago-gastro-duodenoscopy (OGD) and duodenal biopsy: -Crypt hypertrophy - Intraepithelial lymphocytes - Villous atrophy - Lamina propria infiltration **Antibodies**: - Anti-EMA IgA - Anti-TTG IgA - Blood smear and FBC

Answer 517

IBD of transmural inflammation of the GIT (from mouth → anus) Terminal ileum + colon most affected

Answer 518

**NESTS:** **N**o blood or mucus (less common) **E**ntire GI tract **S**kip lesions on endoscopy **T**erminal ileum most affected and **T**ransmural inflammation **S**moking is a risk factor

Answer 519

Relapsing and remitting course Abdominal pain and tenderness: RLQ (ileum and may mimic appendicitis) Systemic symptoms: -Diarrhoea (not bloody) -Anaemia -Pyrexia -Malaise -Weight loss Aphthous ulcer Erythema nodosum + pyoderma gangrenosum Arthritis and spondylitis - Episcleritis - Osteoporosis Finger clubbing Uveitis Glossitis Failure to thrive Perianal abscesses, fistulae, tags or strictures

Answer 520

Inflammation of the fatty layer of skin Reddish, painful, tender lumps

Answer 521

**Faecal calprotectin:**: raised (indicates intestinal inflammation) **Colonscopy and biopsy**: - Transmural inflammation - Deep ulcers - Skip lesions - Cobblestone mucosa FBC **ASCA** Serum B12, folate and iron: low

Answer 522

Macroscopic: -Affects any part of GIT -Oral and perianal disease -Discontinuous involvement (skip lesions) -Deep ulcers and fissures in mucosa (cobblestone appearance) Microscopic: -Transmural inflammation -Non-ceasing Granulomas present 50%

Answer 523

Smoking cessation Inducing remission: - Corticosteroids manage attacks: budesonide (mild), prednisolone (moderate) or hydrocortisone (severe) - Refractory: Anti-TNF (INFLIXIMAB) - Peri-anal disease – abx (METRONIDAZOLE) Maintaining remission: - Immunosuppressants: azathioprine - Mercaptopurine (chemotherapy) Anaemia: iron, B12 or folate deficiency should be treated Surgery

Answer 524

Chronic IBD of submucosal ulcers from the colon **→**rectum (mainly distal colon)

Answer 525

**CLOSE UP:** **C**ontinuous inflammation **L**imited to colon and rectum **O**nly superficial mucosa affected **S**moking is protective **E**xcrete blood and mucus **U**se **aminosalicylates** **P**rimary Sclerosing Cholangitis: in the majority of patients

Answer 526

Abdominal discomfort, tenderness and distention Tenesmus and faecal urgency Erythema nodosum + pyoderma gangrenosum Arthritis and spondylitis Finger clubbing Uveitis Systemic symptoms: - Diarrhoea (Blood +/- mucus PR) - Anaemia - Pyrexia - Malaise - Weight loss

Answer 527

**Faecal calprotectin**: raised **Colonoscopy and biopsy** Bloods: -FBC: low Hb and high WCC -LFTs: low albumin -CRP/ES: high during flares -Serum B12, folate and iron: low -**pANCA** Stool: - MCS - C. Diff Test (CDT) CT (perforation or megacolon)

Answer 528

Macroscopic: - Affects only colon - Begins in rectum and extends proximally - Continuous involvement - Red mucosa (bleeds easily) - Ulcers and pseudopolyps (regenerating mucosa) Microscopic: - Mucosal and submucosal inflammation - No granulomata - Goblet cell depletion - Crypt abscesses

Answer 529

5-aminosalicylic acid (5-ASA): e.g. olsalazine reduces inflammation and maintains remission Glucocorticoid: e.g prednisolone if not responsive to 5-ASA Immunosuppressants: azathioprine/mercaptopurine (chemotherapy) +/- ciclosporin (DMARDs) Admit if acute and severe with fluids Surgery

Answer 530

Low protein + essential amino acids intake

Answer 531

- Oedema - Hair changes - Mental changes

Answer 532

Increase protein + vitamins diet

Answer 533

Malnutrition due to lack of calories from all energy sources

Answer 534

Most common cause of intestinal obstruction (atresia) One segment of the bowel invaginates the other

Answer 535

Ileocaecal

Answer 536

Male aged 6-18m

Answer 537

Sudden Paroxysms of colicky abdominal pain Early bile stained vomit RUQ mass (sausage shape) RED CURRENT JELLY stools

Answer 538

USS: ‘target sign’. X ray + barium contrast: - Dilated loops of bowel close to the obstruction - Collapsed loops of bowel far from the obstruction - Absence of air in the rectum

Answer 539

Immediate IV fluid resus Analgesia: e.g. morphine Interventional radiology: rectal air insufflation Surgery

Answer 540

Narrowing of the pyloric sphincter prevents food from travelling from the stomach → duodenum

Answer 541

- Projectile vomiting after feeding - No bile - Constipation - Weight loss - Visible gastric peristalsis (LUQ) - Firm mass in upper abdomen that feels like a large olive on test feed

Answer 542

- Metabolic alkalosis - Hyponatraemia - Hypochloraemia - Hypokalaemia

Answer 543

Abdominal US: thickened pylorus sphincter U+E Blood gas

Answer 544

IV fluids: rehydrate + correct electrolyte disturbance Stop feeding to stop vomiting Ramstedt's Pyloromyotomy

Answer 545

Inflammatory bowel necrosis Most common GI emergency in neonates (Tends to affect premature)

Answer 546

- Prematurity - IUGR - PDA

Answer 547

- Feeding problems - Abdominal distension - N&V - Bloody mucoid stool and bilious vomiting

Answer 548

Abdo X-ray: - Portal venous gas - Dilated loops of bowel - Bowel wall oedema (thickened bowel walls) - Pneumatosis intestinalis (gas in the bowel) - Pneumoperitoneum (free gas in the peritoneal cavity indicates perforation) Blood tests: -FBC: thrombocytopenia and neutropenia -CRP -CBG: metabolic acidosis -Blood culture: sepsis

Answer 549

Nil by mouth IC fluids Total parenteral nutrition (TPN) IV Cefotaxime/ Vancomycin for 10-14 days Surgical emergency

Answer 550

Death of the bowel tissue → bowel perforation. Bowel perforation → peritonitis and shock

Answer 551

Constipation lasting >6 months

Answer 552

Absent bowel movements due to missing nerve cells (ganglia) in myenteric plexus segment of the colon

Answer 553

- No bowel movements in 1st 48hrs = Meconium Ileus intestinal - Vomiting bilious green - Absolute constipation - Bowel sounds tinkling → absent

Answer 554

Meconium Ileus

Answer 555

GI perforation (Short gut syndrome after surgery)

Answer 556

X ray + barium contrast: - Dilated loops of bowel close to the obstruction - Collapsed loops of bowel far from the obstruction - Absence of air in the rectum Rectal biopsy: absence of ganglionic cells

Answer 557

Bowel washouts/irrigation Bypass surgery/ ileostomy//colostomy surgical of the aganglionic section of bowel

Answer 558

1) Higher concentration of RBCs with short lifespan 2) RBCs breakdown → unconjugated bilirubin 3) Unconjugated bilirubin becomes conjugated in liver → liver immaturity → slower conjugation

Answer 559

Conjugated jaundice in the first 24 hours of life is always pathological: <24hrs = Rhesus haemolytic disease (+ve Coombs test) >14 days = Biliary atresia (pale stools)

Answer 560

- Low BW - Prematurity - Breast fed babies - Maternal diabetes - FHx

Answer 561

First seen normally on forehead and face Neuro signs: - Change in muscle tone, altered crying - Pale stools and dark urine

Answer 562

Kernicterus

Answer 563

Phototherapy Exchange transfusion

Answer 564

High levels of unconjugated bilirubin Acute bilirubin encephalopathy that can cause: - Athetoid movements - Deafness - Low IQ

Answer 565

Jaundice treatments

Answer 566

Reflux of stomach contents through the lower oesophageal sphincter and into the oesophagus to irritate the lining

Answer 567

Distress after feeds/failure to feed & thrive Apnoea Heartburn Worse lying down Related to meals Relieved by antacids Burning retrosternal pain Dyspepsia Acid regurgitation + Water brash Bloating Nocturnal cough Hoarse voice Odonophagia and dysphagia

Answer 568

PPI trial: symptoms relieved Endoscopy

Answer 569

Avoid over-feeding Thicken feeds e.g. carobel Raise head of bed Hold head whilst feeding Small regular meals (not soon before bed) Smoking cessation/alcohol reduction Avoid hot drinks and spicy foods Stop drugs: NSAIDs, steroids, CCBs or nitrates Antacid + Na/Mg alginate (Gaviscon) PPI: lansoprazole - 11 month PPI: undiagnosed dyspepsia - 2 months PPI: endoscopically confirmed GORD Nissen fundoplication

Answer 570

Fundoplication if still failure to thrive / severe oesophagitis

Answer 571

Malformation of GI tract (bulge in distal ileum)

Answer 572

2% of population 2 feet from ileocaecal valve 2 different mucosa Affects <2 year old Males 2x than females ≤2 inches 2% are symptomatic 2 types of the mucosal lining

Answer 573

Always considered when patients have haemorrhage or obstruction Technetium scan: a spot CT scan: if volvulus/intusseception

Answer 574

>2cm Narrow neck Fibrous band to abdominal wall Inflamed/ thickened

Answer 575

Impaired lung development: - Pulmonary hypoplasia - Pulmonary hypertension

Answer 576

Evisceration of abdominal contents > 5cm opening

Answer 577

Congenital condition where the bile duct is narrowed/absent (extra/intra-hepatic a biliary tree scaring) Results in cholestasis

Answer 578

Elevated unconjugated/conjugated bilrubin = jaundice after birth Splenomegaly Pale stools/ dark urine

Answer 579

- Fasting abdominal US: contracted/absent gallbladder - Laparotomy - Transcutaneous bilirubin: raised conjugated bilirubin - LFT’s: abnormal - ERCP imaging: fails to outline a normal biliary tree

Answer 580

Kasai procedure

Answer 581

Lymph drainage: lymphoedema (eg congenital, blockade) Venous pressure and drainage: venous obstruction e.g thrombosis Lowered oncotic pressure (low albumin / protein): - Malnutrition - Decreased liver production - Increased loss e.g. gut or Kidney (nephrotic syndrome) Salt and water retention: - Heart failure - Kidney: impaired GFR

Answer 582

A group of symptoms where the glomerulus basement membrane becomes highly permeable to protein: - **Proteinuria:** filtration barrier becomes disrupted and more permeable - **Hypoalbuminaria:** albumin is lost in urine **→**peripheral and periorbital oedema - **Hypertension** - **Peripheral oedema**

Answer 583

Minimal change disease

Answer 584

Heavy proteinuria: ▪ First morning urine (protein:creatinine) >1g/m2/24 hrs Hypoalbuminaemia: ▪ Fluid retention and oedema <25-30 Oedema: ▪ Pitting oedema and gravitational Can also have Hyperlipidaemia

Answer 585

Urinalysis (dipstick = UTI, MC&S = protein) 24 hour urine collection Protein levels >3.5mg Renal biopsy BP Low serum albumin Low Anti-TIII = prone to renal vein thrombosis/ DVT/ PE Antistreptolysin O and anti-DNAse B titres Throat swab

Answer 586

Congenital (<1 year) Steroid sensitive Steroid resistant

Answer 587

1) Normal BP: renal function 2) No haematuria 3) No nephritis 4) Histology: minimal change disease

Answer 588

1) Elevated BP: impaired renal function 2) Haematuria 3) Nephritis 4) Histology: underlying glomerulopathy/ basement membrane abnormality

Answer 589

1st: Prednisolone 2nd: Renal biopsy if unresponsive

Answer 590

Diuretics/ACE-I = oedema NSAIDs: reduce proteinuria

Answer 591

A collection of signs and symptoms that occur as a result **of kidney inflammation** It is NOT a diagnosis, but a clinical picture. Leads to glomerular basement membrane damage: - **Haematuria** - **Hypertension** - **Oedema**

Answer 592

Categorised into 3 groups: Type III hypersensitivity: - Post-streptococcal glomerulonephritis - IgA nephropathy - Diffuse proliferative glomerulonephritis Multiple causes: - Membranoproliferative glomerulonephritis - Rapidly progressive glomerulonephritis - Defect in collagen synthesis: Alport syndrome

Answer 593

Haematuria Proteinuria Decreased urine output (salt and water retention = HTN) Impaired GFR (Increased Creatinine) Oedema Hypertension Abdominal pain Rash

Answer 594

FBC: mild normochromic +normocytic anaemia U&Es: increased urea + creatinine, hyperkalaemia + acidosis Low C3 + normal C4 complement Renal biopsy Urinalysis: - Haematuria - Red blood cell casts - Proteinuria (<3.5g per day) Microscopy: RBC casts

Answer 595

Fluid balance (salt restriction + diuretics) Blood pressure control (ACEi/ARB) Correct electrolyte imbalance (potassium/ acidosis) Penicillin: if infection

Answer 596

X linked recessive disorder associated with hearing loss, ocular defects and progressive kidney disease Almost always has haematuria

Answer 597

Upper = Pyelonephritis Lower = Cystitis

Answer 598

E.coli Klebsiella

Answer 599

>6 weeks of: -Suprapubic pain: worse with a full bladder and relieved by emptying the bladder -Changes in storage and voiding e.g. bedwetting - Dysuria - Suprapubic burning - Worse during menstruation - Changes in urine appearance/consistency e.g. cloudy, foul and - haematuria - Confusion

Answer 600

Similar **presentation to lower UTIs** + **triad of**: 1) High fever and rigors 2) Loin-groin/back pain 3) N&V

Answer 601

Vulvitis in girls Balanitis in boys

Answer 602

Urinalysis dipstick: only if >3m olds - Leukocyte esterases - Nitrites - Haemoglobin MSU MCS: mid stream (<3m olds can only use this) - RBCs - WBCs - Bacteria - Inflammatory markers Vaginal swab and NAAT: STI LP Blood cultures USS: KUB DMSA: to asses scarring if pyelonephritis MCUG: reflux

Answer 603

IV cefuroxime for 7 days: **THEN** < 3m old: IV Amoxicillin and Gentamycin > 3m old: Trimethoprim THEN Nitrofurantoin if no response

Answer 604

Anomaly of vesicoureteric junction where the ureters are displaced laterally and enter bladder directly = not at an angle → back flow

Answer 605

Incomplete emptying = increase risk of UTI (cystitis) Renal damage: if high pressure/infrarenal reflux

Answer 606

Micturating cystourethrogram

Answer 607

Acute decline in kidney function → rise in serum creatinine and/or fall in urine output

Answer 608

- Hypovolaemia (nephrotic syndrome, gastroenteritis and haemorrhage) - Circulatory failure

Answer 609

Vascular: haemolytic uraemia syndrome/vasculitis Tubular: acute tubular necrosis Glomerular: glomerulonephritis Interstital: pyelonephritis

Answer 610

Obstruction Congenital: posterior urethral valves Acquired: blocked urinary catheter

Answer 611

Cardiac surgery Bone marrow transplant Toxicity: DRUGS (NSAIDs, vancomycin, acyclovir or aminoglycosides)

Answer 612

Urinary output < 0.5ml/kg/hr for >6 hours Serum creatinine increased by ≥50% in the past week Serum creatinine increase by ≥25 µmol/l in 48 hours Hyperkalaemia

Answer 613

Fluids Furosemide: If urine osmolality low Renal replacement therapy: if pulmonary oedema/HTN/ not responding to tx

Answer 614

Calcium resonium (mild) IV calcium gluconate (if ECG changes) Insulin + dextrose (removes K from body) Diuretics/Dialysis (removes K from body)

Answer 615

Deterioration of renal function for >3 months Defined as: eGFR <60, proteinuria or ≥3mg/mmol albumin:creatine ratio

Answer 616

- Congenital dysplastic kidney - Chronic pyelonephritis - Glomerulonephritis - HTN - Adult PKD - Diabetic nephropathy

Answer 617

- Anorexia/lethargy - Polydipsia/polyuria - Hypertension - Retinopathy - Anaemia - Failure to thrive - Seizures - Renal Ricketts - Pruritus (itching) (unfiltered phosphorus)

Answer 618

**eGFR:** - <60 mL/min/1.73m^2 Renal chemistry: - Creatinine: high - Urea: high - Electrolyte abnormalities Urinalysis: - Haematuria +/- proteinuria +/- albuminuria

Answer 619

Oral sodium bicarbonate: treats metabolic acidosis Iron supplementation and EPO: treats anaemia Vitamin D Dialysis Renal transplantation

Answer 620

Autoimmune disorder: T-cell mediated B-cell destruction in the Islets of Langerhan leading to absolute Insulin Deficiency

Answer 621

Decreased insulin secretion +/- increased resistance

Answer 622

1) Stimulates glycogen formation from glucose in the liver 2) Stimulates glucose uptake from blood → cells 3) Lowers blood sugar

Answer 623

**Diabetes:** Random blood glucose: ≥11 mmol/L - Finger-prick HbA1C: ≥48 mmol/L - Average BM over 2-3 months Random plasma glucose: ≥11 mmol/L OGTT: ≥11.1 mmol/L Fasting plasma glucose: ≥7.0. mmol/L 1. Fasting BM 2. 75g glucose 3. Second BM 2 hours later **Pre-diabetes:** HbA1C: ≥42-48 mmol/L Impaired glucose tolerance: ≥7.8 - 11.1 mmol/L Impaired fasting glucose: ≥6.1 - 6.9 mmol/L **Asymptomatic:** Same as above on TWO SEPARATE OCCASIONS

Answer 624

Gluconeogenesis = produce glucose from amino acids/muscle breakdown Ketogenesis = fatty acids converted to ketones

Answer 625

Weight loss /lean/low BMI (Type 1) Weight gain (Type 2) Polyuria Polydipsia Lethargy Impaired growth Reduced visual acuity Recurrent infections: e.g. balantitis/pruititus vulvae from repeat candida infections Acanthosis nigricans Diabetic foot: - Pulseless peripherals - Calluses - Ulceration - Charcot join

Answer 626

**TYPE 1** 1st line: Insulin **TYPE 2** 1st line: lifestyle and diet advice (increase exercise, stop smoking, vegetables and oily fish, low carbohydrate and high fibre) 2nd line: metformin 3rd line: metformin + another anti-diabetic (eg. sulfonylureas) 4th line >58 mmol/mol despite treatment: triple therapy (i.e +DPP-4i) 5th line: intermediate-acting insulin + metformin

Answer 627

- Vomiting - Reduced consciousness - Acetone/fruity smell to their breath - **Kussmaul respiration:** deep laboured breathing in an attempt to reverse metabolic acidosis

Answer 628

Hyperglycaemia = >7mmol/L Acidosis pH <7.3 Ketones in urine

Answer 629

**FIG-PICK** **F**luids (over 48 hours) 0.9% NaCl. **I**nsulin 0.1units/kg/h (fixed rate infusion 1hr after giving fluids ) **G**lucose **P**otassium **I**nfection **C**hart: hourly blood glucose and ECG **K**etones Avoid bicarbonate as increases risk of cerebral oedema

Answer 630

- Irritable - Sweaty - Headache - Pallor - Drowsy - Slurred speech - Convulsions

Answer 631

1) Hypothalamus secretes thyrotropin-releasing hormone (TRH) 2) Anterior pituitary secretes thyroid-stimulating hormone (TSH) 3) Thyroid releases T3 and T4

Answer 632

Congenital: - Dysgenesis - Dyshormonogenesim Acquired: - Prematurity - Iodine deficiency - Hashimoto's Thyroiditis (autoimmune) - De Quervains thyroiditis - Drugs

Answer 633

Thyroid peroxidase antibodies (Anti-TPO) Anti-thyrogobulin (Anti-TgAb)

Answer 634

Lithium Amiodarone

Answer 635

**Menorrhagia** **Myxoedema**: autoimmune hypothyroidism **METABOLIC** **M**arcocytosis O**e**dema (incl orbital) **T**hyroid goiter **A**naemia **B**radycardia **O**besity **L**ateral eyebrow loss **I**rregular menstruation **C**onstipation Learning difficulties (Congenital normally picked up on screening and asymptomatic)

Answer 636

**Thyroid function test:** Primary hypothyroidism: ↑ TSH, ↓ T3 & T4 Secondary hypothyroidism: ↓ TSH, ↓ T3 & T4 **ESR and CRP**: de Quervain's thyroiditis **Anti-TPO and Anti-TgAb:** hashimotos thyroiditis Guthrie test (heel-prick): IDENTIFIES CONGENITAL COAUSES

Answer 637

Levothyroxine (replacement T4)

Answer 638

Grave’s disease (autoimmune) Toxic multi-nodular goitre De Quervains thyroiditis Drugs

Answer 639

Anti-TSH receptor antibodies (Anti-TSH)

Answer 640

Amiodarone

Answer 641

Graves Disease Thyroid Eye Disease Amiodarone

Answer 642

**General**: THYROIDISMG **T**remor **H**eart rate increase **Y**awning **R**estless **O**ligomenorrhoea **I**rritability **D**iarrhoea **I**ntolerance to heat **S**weating **M**uscle wasting (weight loss) **Goitre** **Grave’s** **disease**: - Diffus Goitre - Grave’s eye disease - Pretibial myoexoedema (musin deposits in skin) - Bilateral exophthalmos **Toxic multi-nodule goitre:** - Goitre with firm nodules

Answer 643

**Thyroid function test:** ↓ TSH, ↑ T3 & T4 **ESR and CRP**: de Quervain's thyroiditis **Anti-TSH** Anti-TPO Anti-TgAb

Answer 644

**Grave’s disease:** - Carbimazole (anti-thyroid drug): titration-block or block and replace **General**: - Radioactive iodine - Beta blockers: e.g. propranolol for symptomatic relief - Surgical resection

Answer 645

- IV hydrocortisone - IV Fluids - Propanolol

Answer 646

1) Autosomal recessive mutation in chr 6 1) Defective 21-hydroxylase enzyme 1) Less gluco+ mineralocorticoids produced 1) More testosterone made

Answer 647

Hyponatraemia Hypoglycaemia **Hyper**kalaemia

Answer 648

SALT-LOSERS: (no aldosterone production) - Hyponatraemia - Hypoglycaemia - **Hyper**kalaemia - Metabolic acidosis - Hypoglycaemia - Elevated 17alpha-hydroxyprogesterone

Answer 649

IV fluids: restores electrolyte levels Glucocorticoids: fludrocortisone Cortisol: IV hydrocortisone

Answer 650

Hypogonadotrophic hypogonadism causing: - Delayed puberty - Anosmia

Answer 651

Development of secondary sexual characteristics before: - 8 years in females - 9 years in males

Answer 652

First stage of breast development

Answer 653

Maturation of the adrenal gland → androgen production → body odour and mild acne

Answer 654

Growth of pubic hair

Answer 655

First ejaculation and testicular size >3ml

Answer 656

The absence of secondary sexual characteristics by age 14 or 16

Answer 657

Turner syndrome (45X0)

Answer 658

Constitutional delay (runs in the family)

Answer 659

Brain tumour

Answer 660

GnRH super-agonists can be given to suppress pulsatility of GnRH secretion

Answer 661

1) GnRH (from hypothalamus) → increased FSH+LH 2) Testes/ovaries → increased testosterone & oestrogen/progesterone → inhibited FSH/LH secretion

Answer 662

Joint swelling/stiffness >6 weeks Aged <16 No other cause is identified

Answer 663

Oligoarticular: 1-4 joints in first 6 months Polyarticular RF -ve: 5+ joints in first 6 months Polyarticular RF +ve: 5+ joints in first 6 months /+ve RF seen on two occasions Systemic onset JIA: Arthritis + 2 weeks fever Psoriatic: arthritis + psoriasis

Answer 664

Fluctuating fever Salmon-pink rash Uveitis Pain Morning stiffness Swelling Psoriasis Dactylitis Nail pitting Rash Uveitis

Answer 665

Chronic anterior uveitis Growth failure Osteoporosis

Answer 666

MCV: normocytic anaemia FBC: - Raised: WCC, CRP, ESR, platelets and serum ferritin. RF / HLA-B27 MSK examination X-rays

Answer 667

NSAIDs Analgesics Corticosteroid tablets/injections DMARDs (e.g. methotrexate) TNFi (infliximab) IL 6 inhibitor (tocilizumab)

Answer 668

Chronic autoimmune disease affecting every organ of the body Relapsing and remitting

Answer 669

SOAP BRAIN MD Serositis (pleuritis/pericarditis) Oral ulcers Arthritis Photosensitivity Blood (pancytopenia) Renal (proteinuria) ANA +ve (anti-nuclear antibody) Immunological Neurological (psych/seizures) Macular rash (salmon-pink) Discoid rash

Answer 670

No cure Avoid sun and use sunscreen Corticosteroid (prednisolone) DMARD (hydroxychloroquine) NSAIDs

Answer 671

Inflammation of the bone/bone marrow Normally limited to one bone, but can be at multiple sites Can be in periphery or axial skeleton

Answer 672

Distal femur Proximal tibia

Answer 673

S. Aureus H. influenza Group A beta haemolytic strep

Answer 674

Severe pain Immobile limb (pseudo paresis) Swollen over area Acute febrile illness (lethargy/high temp)

Answer 675

X-ray MRI Blood cultures FBC: Raised WCC/CRP

Answer 676

1) >3m old = 7 days IV Cefuroxime 2) THEN + 5 weeks oral (if tolerated and CRP <10): - Flucloxacillin: S.aureus - Co-amoxiclav: if unsure

Answer 677

Infection of the joint space: can lead to bone destruction

Answer 678

Septic arthritis until proven otherwise

Answer 679

1) S. aureus 2) H. influenza

Answer 680

**Suspected in all monoarthritic cases:** especially the knee Hot, swollen, painful and restricted joint Acute Systemic symptoms

Answer 681

**Hot joint policy:** presume patient has septic arthritis until its excluded Aspirate synovial joint: ****MCS, FBC & crystal examination - WCC, CRP/ESR

Answer 682

Abx Therapeutic joint aspiration Analgesia

Answer 683

Neonatal hip joint abnormality

Answer 684

- Female 6x more likely - Breech presentation - FHx - Firstborn child - Oligohydramnios

Answer 685

Asymmetry Abnormal gait: - Affected hip externally rotated - Toe-Walking on affected side - Waddling gait

Answer 686

Barlow Test = attempt to dislocate femoral head posteriorly Ortolani Test = attempt to relocate a dislocated femoral head Dynamic US: early diagnosis important as if appropriately aligned in first few months = resolve spontaneously USS: - Femoral head coverage <50% - Alpha angle <60 degrees CT MRI

Answer 687

Most spontaneously stabilise in 3-6 weeks Pavlik harness (flexion-abduction orthosis) if <4-5m old

Answer 688

Temporarily disrupted blood flow to femoral head causing avascular necrosis in children aged 4-8yrs

Answer 689

Hip/groin pain Limp Restricted hip movements Referred pain to the knee

Answer 690

Limited abduction and internal rotation X-ray: abnormal ossification Blood tests: excludes inflammation Technetium bone scan MRI

Answer 691

Bed rest Traction Crutches Analgesia

Answer 692

Slipped capital femoral epiphysis Femoral head slips through a growth plate fracture

Answer 693

Hip, groin, thigh or knee pain Restricted internal rotation Painful limp Restricted range of hip movement Hip kept in external rotation

Answer 694

Surgical pinning of the hip

Answer 695

Autosomal dominant Most common form of short-limb dwarfism due to REDUCED growth of cartilaginous bone

Answer 696

- Large skull - Normal trunk - Short arms and legs - Short stature - Frontal bossing - Marked lumbar lordosis - Bow legs (genu varum) - Disproportionate skull - Foramen magnum stenosis

Answer 697

Low bone density and micro-architectural defects in bone tissue with normal mineralisation Increased bone fragility and susceptibility to fracture

Answer 698

1) > 1 vertebral crush fracture OR 2) Bone density 2 long bone fractures (before age 10) or >3 fractures (age 19).

Answer 699

Inherited: osteogenesis imperfecta Acquired: drug induced, malabsorption

Answer 700

Stage before osteoporosis: less severe

Answer 701

**Asymptomatic:** with the exception of fractures **Common fragility fractures:** vertebral crush, radial wrist (Colles' fracture) and proximal femur

Answer 702

**Dual-energy X-ray absorptiometry (DEXA) scan:** measures bone loss - T score <2.5 = osteoporosis

Answer 703

Vitamin D and calcium supplements (AdCal-D3) Stop smoking HRT: to prevent fragility fracture in high risk women Anti-resorptive drugs: - **Biphosphonates:** alendronate (inhibits osteoclast activity) - Oestrogen (promotes osteoblast formation) - Denosumab Anabolic drugs - Parathyroid hormone receptor agonists

Answer 704

Autosomal dominant condition Increased fragility of bone (collagen type 1 = bone/teeth)

Answer 705

- Hypermobility - Blue / grey sclera (the “whites” of the eyes) - Triangular face - Short stature - Deafness - Dental problems - Bone deformities: bowed legs and scoliosis - Joint and bone pain - Fractures - Bruises

Answer 706

Sillence classification

Answer 707

Clinical diagnosis X-rays: diagnoses fractures and bone deformities Genetic testing Bone densitometry

Answer 708

IV pamidronate: reduces fracture incidence, increases bone mineral density, reduces pain and increases energy levels Oramorph or IV morphine Bisphosphates: alendronate to increase bone density Vitamin D: prevents deficiency

Answer 709

A disorder of bone mineralisation leading to bone weakness Caused by vitamin D deficiency

Answer 710

Bowed legs Knock knees Rachitic rosary (expanded ribs) Craniotabes (soft skull) Delayed closure of the sutures Frontal bossing Delayed teeth bowing of legs Growth failure Bone pain

Answer 711

Serum 25-hydroxyvitamin D: < 25 nmol/L X-ray

Answer 712

Correct deficiency: i.e colecalciferol + calcium

Answer 713

Neonate: <140g/L 1-12months: <100g/L 1-12years: <110g/L

Answer 714

Impaired red cell production (Iron deficiency) Increased red cell destruction (haemolytic disease of newborn, thalassaemias, sickle cell and G6PD deficiency) Blood loss (meckel's diverticulum and von willebrand disease)

Answer 715

Jaundice → kernicterus Oedema Hepatosplenomegaly Fatigue Headaches Dizziness Pale skin Conjunctival pallor Angular cheilitis Glossitis Aphthous ulcers Tachycardia

Answer 716

- Excessive bleeding (menorrhagia or GI bleed) - Poor dietary intake (poor feeding) - Malabsorption (CMPI) - Increased requirements (growth spurts or pregnancy)

Answer 717

Fatigue Pica Pale skin Angular chelitis Atrophic glossitis Post-cricoid webs Koilonychia Brittle hair and nails Hair loss

Answer 718

Hb FBC MCV: microcytic hypo chromic reticulocytes Transferrin saturation: Increased in iron deficiency Low ferritin and serum Fe

Answer 719

Treat cause: Ferrous sulphate (oral iron therapy) - May cause N/D/V, abdo pain and constipation Iron rich diet - Meat and dark green vegetables Blood transfusion

Answer 720

Enzyme essential for preventing oxidative damage to red cells RBCs lacking G6PD = exposed to oxidant induced haemolysis

Answer 721

Intermittant neonatal jaundice Anaemia Gallstones Splenomegaly Acute intravascular haemolysis (triad signs below): - Dark urine - Fever - Flank pain

Answer 722

Blood film: Heinz bodies G6PD enzyme assay

Answer 723

Avoid triggers

Answer 724

Autosomal recessive Misshapen β-globin= HbS RBC sickled, fragile and prone to haemolysis Can get stuck in capillaries**→** vaso-occlusive crisis

Answer 725

**Newborn screening with Guthrie heel prick:** - **Screening is offered during pregnancy.** **FBC:** normocytic anaemia with reticulocytosis **Blood film:** sickled RBCs, target cells and Howell-Jolly bodies **Hb electrophoresis and solubility: diagnostic** (increased HbS and reduced/absent HbA)

Answer 726

Hydroxycarbamide (chemotherapy increases HbF) Blood transfusions

Answer 727

Mutation on codon 6 of B-globulin gene Change in AA sequence from: Glutamine → Valine

Answer 728

Excess of alpha chains Autosomal recessive HBB gene on chr 11 HbA2 RAISED = ANAEMIA

Answer 729

Long term folic acid Bone marrow transplant Regular transfusions

Answer 730

Autosomal dominant mutations changes RBC shaped from concave to sphere Shape sphere RBC get destroyed as they pass through the spleen Causes haemolytic anaemia

Answer 731

Parvovirus infection

Answer 732

Clinically FHx Blood film: spherocytes MCHC: raised FBC: raised reticulocytes

Answer 733

Oral folic acid Splenectomy: if severe

Answer 734

Destruction of platelets by IgG autoantibodies

Answer 735

Asymptomatic Prolonged bleeding Petachie Purpura/bruising

Answer 736

Prednisolone & IV IgG Rituximab (monoclonal antibody used in cancer)

Answer 737

Dx of exclusion Low platelets!

Answer 738

Abnormality to vWF→ no adhesion between platelets and damaged endothelium→ excessive bleeding

Answer 739

Tranexamic acid (stop excessive bleeding post surgery) In response to bleeds: IV infusion of vWF, desmopressin or IV factor VII

Answer 740

Lymphoblast proliferation in the bone marrow: B cell lineage Associated with down syndrome

Answer 741

Acute Lymphoblastic Leukaemia (ALL)

Answer 742

- Increased infections (leukopenia) - **Bone and joint pain:** due to marrow expansion **Petechiae and epistaxis:** due to thrombocytopenia - Hepatosplenomegaly (blast cells enlarge →abdominal distension) - Lymphodenopathy (settle in lymph nodes) **Abdominal fullness and pain:** (due to hepatosplenomegaly) B-cell symptoms (night sweats, fever and weight loss) Pallor and Fatigue: anaemia Failure to thrive

Answer 743

[t(12;21] or [t(9;22)] mutations

Answer 744

FBC: pancytopenia Blood film: blast cells Bone marrow biopsy (CONFIRMS)

Answer 745

Chemotherapy **Allopurinol:** tumour lysis syndrome from chemotherapy can release uric acid → Gout Radiotherapy Stem cell transplantation for: - Girls: 2 years - Boys: 3 years Surgery

Answer 746

1) Induction 2) Consolidation 3) Interim maintenance 4) Delayed intensification 5) Maintenance

Answer 747

Endocrine related e.g. growth and development problems. Intellectual. Fertility problems. Psychological issues. Cardiac and renal toxicity

Answer 748

Tumours arising from neural crest tissue in the adrenal medulla and sympathetic nervous system

Answer 749

1) Abdo mass (can be anywhere on sympathetic chain) 2) METASTASES SYMPTOMS

Answer 750

Raised urinary catecholamines Biopsy MIBG scan (radiolabelled tumour-specific agent)

Answer 751

Chemotherapy Radiotherapy Surgery

Answer 752

Most common renal tumour in childhood that is: - Unilateral - Presents as asymptomatic abdominal mass

Answer 753

Abdominal mass Haematuria Abdominal pain Anorexia Anaemia

Answer 754

US CT/MRI: staging Biopsy: staging

Answer 755

Chemotherapy Nephrectomy Radiotherapy

Answer 756

Headache Change in behaviour Nausea/vomiting Over-sleepy Papilloedema (may decrease visual acuity)

Answer 757

Malignant tumour of retinal cells (eye) Mutated RB1on chr 13

Answer 758

- Loss of red-reflex: replaced by white pupil (leukocoria) - Pain around the eye - Poor vision

Answer 759

1st line: Laser therapy Chemotherapy + radiotherapy Phototherapy Surgical repair/removal

Answer 760

CRACO 1) Colour (skin, lips, tongue) 2) Activity 3) Respiratory 4) Circulation/Hydration 5) Other

Answer 761

Colour : - Pallor - Blue Activity : - Not responding to social cues - Does not stay awake - High pitched cry Respiratory : - Grunting - Tachypnoea - Chest in-drawing - RR >60 Circulation/Hydration : - Reduced skin turgor - Tachycardia Other : - Temperature - Non-blanching rash - Bulging fontanelle - Neck stiffness - Status epilepticus - Focal signs/seizures

Answer 762

<12m: >160 bpm 12-24m: >150bpm 2-5yrs: >140bpm

Answer 763

<3m: >38° 3-6m: >39°

Answer 764

6-12m: >50 >12m: >40

Answer 765

100ml/Kg/day: for first 10Kg 50ml/Kg/day: for the next 10Kg 20ml/Kg/day: for every Kg after that

Answer 766

0.9% Sodium Chloride + 5% glucose +/- KCl

Answer 767

Cerebral oedema

Answer 768

150ml/Kg/day

Answer 769

1. Size 2. Growth plates 3. Skill sutures 4. Ossification 5. Congenital problems e.g. dextrocardia and osteogenesis imperfecta

Answer 770

1) Insufficient surfactant production in immature lungs 2) Lung cannot provide body with enough oxygen due to surfactant disruption → to lung collapse

Answer 771

- **Sepsis, pneumonia**, trauma or aspiration - **Premature birth:** not enough surfactant produced

Answer 772

Prematurity C-section

Answer 773

Tachypnoea: RR 40-60/ >60 Subcostal and intercostal recession Stridor Cyanosis

Answer 774

Nasopharyngeal aspirate: PCR Bloods: amylase, FBC, U+E, CRP CXR: bilateral diffuse infiltrates, hyperinflation, air trapping and focal atelectasias PCWP: pulmonary capillary wedge pressure (<19mmHg) Pulse oximetry ABG: Refractory hypoxaemia

Answer 775

Antenatal steroids (i.e. dexamethasone to induce foetal lung maturation) O2 Intubate CO2 monitoring Endotracheal surfactant Nasal CPAP Mechanical ventilation + Intubation Supplementary oxygen Fluids

Paediatrics Flashcards

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