Paediatrics

Question 1

Mechanism of bilirubin production

Answer 1

Heme in Hb - biliverdin - bilirubin (and albumin) - liver - conjugated with glucuronide then excreted into SI, taken up into blood - urine - urobilirubinogen or metabolised by bacteria - stercobilin

Question 2

Bilirubin effect on brain

Answer 2

Kernicterus - acute bilirubin encephalopathy, crosses bbb and stains basal ganglia causing permanent damage

Question 3

Features of kernicterus

Answer 3

Lethargy, poor feeding, irritable, shrill cry
Hypertonic - opisthotonos
Seizures, coma

Question 4

When does bilirubin start having an effect

Answer 4

When unconjugated bilirubin level > albumin binding capacity
Only when young as bbb matures quickly so higher levels can be tolerated better

Question 5

Complications of kernicterus

Answer 5

Choreoathetoid cerebral palsy
Learning difficulties
Sensorineural deafness

Question 6

Bilirubin level which is jaundice

Answer 6

> 80umol/l

Question 7

3 categories of jaundice causes and examples of each

Answer 7

Increased haemolysis - rhesus incompatibility, ABO incompatibility, G6PD, spherocytosis, sepsis
Decreased secretion - liver dysfunction/post hepatic
Dehydration

Question 8

What is ABO incompatability caused by and features

Answer 8

some group O women have IgG anti-A-haemolysin which crosses placenta
Hb normal or slightly reduced, no splenomegaly

Question 9

G6PD - who and what to do

Answer 9

Avoid some drugs

Mediterranean, East, Africa

Question 10

Causes of sepsis in neonatal jaundice

Answer 10

GBS - ascending infection, PROM, prematurity

TORCH screen

Question 11

Features of liver dysfunction/post hepatic neonatal jaundice

Answer 11

Chalky pale stools

Conjugated bilirubin collects in liver and damages

Question 12

Check for dehydration in neonatal jaundice?

Answer 12

7-8 nappies a day
Mucous membranes
Weight loss

Question 13

Assessing neonatal jaundice

Answer 13

Preterm
Septic/unwell
Family history 
Maternal blood group 
Maternal infection 
Extent - how much of body, rate of rise (>10mmol/h = concerning), transcutaneous screening tool on sternum, sx (sleepy, poor feeding - phototherapy if sx)

Question 14

Inv neonatal jaundice

Answer 14

Transcutaneous bilirubin
FBC and blood film for reticulocytes and fragmented cells
Blood group and DCT
Bilirubin - conjugated accumulates in liver and damages
Sepsis inv

Question 15

Causes of baby jaundice >1d

Answer 15

Physiological
Infection
Haemolysis
Crigler-Najjar - decreased conjugation of bilirubin and glucuronic acid in liver

Question 16

Why physiological jaundice - 5

Answer 16

Immature enterohepatic circulation - reduced conjugations
High Hb conc as physiolgical Hb release
RBC life span shorter than for adults (70d vs 120)
Reduced gut flora - reduced bile pigment elimination
Breastfed - dehydration - reduced elimination

Question 17

5 causes of prolonged jaundice

Answer 17

Breast milk jaundice - unconjugated, fades by 4-5w
Hypothyroid - unconjugated
Infection  - unconjugated 
Metabolic liver disease - conjugated
Biliary atresia - conjugated

Question 18

Features of hypothyroid in baby

Answer 18

Reversible neuro problems/developmental delay 
Dry skin, coarse facies
Prolonged jaundice 
Constipation 
On Guthrie test

Question 19

When is bilirubin conjugated in prolonged jaundice

Answer 19

Metabolic liver disease

Biliary atresia

Question 20

What is biliary atresia

Answer 20

Angiopathy causes absence of hepatic bile ducts
Can cause toxic damage to liver cells and liver failure requiring transplant
Hepatosplenomegaly, dark urine, pale stools

Question 21

Inv prolonged jaundice

Answer 21

Split bilirubin - conjugated vs unconjugated
Unconjugated high = breast fed or physiological
Conj shouldn’t be >10-20% total
Radionucleotide scan to see liver drainage

Question 22

Manage biliary atresia causing prolonged jaundice

Answer 22

Kasai procedure creates bile duct from SI,, mostly successful if <60 days old

Question 23

Manage prolonged jaundice

Answer 23

Phototherapy - blue light converts bilirubin to soluble products
Exchange transfusion if not improving with phototherapy or increasing rapidly. Warm blood, umbilical artery and vein

Question 24

ADR of photherpay for jaundice - 4

Answer 24

Eye damage
Dehydration
diarrhoea
Difficult to control tempmerature

Question 25

Sepsis screen in neonates

Answer 25

Urine Blood cultures LP +- cxr

Question 26

RF for neonatal sepsis - 5

Answer 26

``` PROM (>18h) Maternal inf Preterm labour Foetal distress Central line/catheter ```

Question 27

Where does sepsis come from in neonate - 4

Answer 27

Environment Placenta Ascent from vagina During birth

Question 28

Early onset neonatal sepsis timing and bacteria

Answer 28

<48h of birth | E. coli and listeria

Question 29

Late on neonatal sepsis timing and bacteria

Answer 29

>48h Staph a E. coli GBS

Question 30

Presentation of neonatal sepsis

Answer 30

``` Labile temperature Lethargy Poor feeding Resp distress Collapse DIC ```

Question 31

Line infection in neonatal sepsis

Answer 31

Coag neg staph

Question 32

Manage sepsis in neonates

Answer 32

``` ABC Ventilation, inotropic, fluids FBC, CRP, glucose Cultures X-ray LP for culture, glucose, protein, WCC, gram stain Stool, throat, urine culture Abx: Early - benzylpenicillin and gentamycin - cefotaxime (meningitis). - amoxicillin (listeria - purulent conjunctivitis, maternal infection ) Late - fluclox and gentamycin, cefotaxime (meningitis) ```

Question 33

Sensitising events for rhesus haemolytic disease

Answer 33

Threatened miscarriage Amniocentesis Chorionic villus sampling Birth

Question 34

Sx of rhesus haemolytic disease

Answer 34

``` Jaundice, kernicterus Yellow vernix CCF - oedema, ascites Hepatosplenomgaly Progressive anaemia, bleeding Hydrops fetalis - severely affected oedematous foetus with stiff, oedematous lungs ```

Question 35

Manage rhesus disease

Answer 35

Inv: - Check for anti-D antibodies in all rh- mothers at 28 and 34w - May need foetal blood sampling and intraperitoneal transfusion if large number - Regular USS and amniocentesis Deliver baby before severe haemolysis Trt: - Exchange transfusion - remove maternal antibodies, if Hb <70 - Phototherapy - Immunisation with anti-D antibodies at 28w, reduces need for exchange transfusion - Maternal antibodies will persist for some time and continue to cause some haemolysis

Question 36

What is respiratory distress syndrome

Answer 36

Deficiency in alveolar surfactant - atelectasis - resp failure - reduced CO, hypotension, acidosis, renal failure

Question 37

Major factors in resp distress syndrome and why - 2

Answer 37

Premature - alveoli develop at 24w, surfactant at 24-36w with peak at 32w C section - no stress response to encourage fluid reabsorption

Question 38

Signs of RDS - 5

Answer 38

``` Increased work of breathing - nasal flaring, grunting, apnoea/dyspnoea Tachypnoea Chest wall recessions Cyanosis CXR - round glass, not hyperinflated ```

Question 39

Differentials of RDS - 7

Answer 39

``` Transient tachypnoea of newborn - due to fluid, resolves in 24h Meconium aspiration Pneumothorax Diaphragmatic hernia CHD Metabolic acidosis Hypoxic-ischaemic encephalopathy ```

Question 40

Treatment of RDS

Answer 40

Prevent with steroids if preterm birth risk O2 Support resp eg CPAP, ventilation Surfactant via NGT

Question 41

Complications of RDS - 4

Answer 41

Chronic lung disease of prematurity Retinopathy of prematurity Infection or trauma secondary to incubation Impaired head growth from steroids

Question 42

What is chronic lung disease of prematurity and 2 complications

Answer 42

Still requiring o2 at >=36w gestational age or still requiring o2 after 28d Bronchopulmonary dysplasia - injury to lungs from mechanical ventilation, or delayed maturation - wean slowly +- steroids CXR - hyperventilated, reticular markings 1. Necrotising bronchiolitis with alveolar fibrosis 2. Cardiomegaly indicates high pulmonary pressure 3. Pertussis and resp infections can cause resp failure - give RSV prophylaxis

Question 43

Cause of retinopathy of prematurity, what it causes and prevention/trt

Answer 43

O2 and prematurity derange VEGF, causing vascular proliferation at junction of retina. Causes retinal detachment, fibrosis, blindness, early glaucoma (in teens) Prevent by running at lower O2, and treat with laser and intravitreal anti-VEGF RF: premature, slow weight gain, low birth weight, anaemia, twin to twin transfusion (the donor), maternal smoking/drugs,

Question 44

RF for meconium aspiration - 4

Answer 44

Post term Chorioamnionitis Maternal hypertension or pre eclampsia Smoking or substance abuse

Question 45

4 broad causes of hypoxic injury and presentation

Answer 45

Hypoxic-ischaemic encephalopathy, need resuscitation at birth Mostly from problems in labour: - uterus - excessive contractions - cord - compression or prolapse - placenta - poor function, pre eclampsia, abruption - baby - cardioresp adaptation failure at birth

Question 46

Effects of HIE - 3

Answer 46

Brain damage Bradycardia Liver, kidney, GI damage

Question 47

Poor prognostic factors in HIE - 3

Answer 47

Slow rate of improvement Irregular breathing at 30 mins APGAR low at 20 mins

Question 48

Manage HIE - 5

Answer 48

Resuscitate, ventilate Hypothermia jacket (33.5) for 72h as long as: 1) >36w, 2) clinical evidence of deficiency (apgar), 3) cerebral function being monitored. = halves cerebral palsy rates, reduces delayed neuronal death in days after cytotoxic death Fluid restriction as transient renal impairment Inotropes and volume support for hypotension Monitor hypoglycaemia and electrolytes - early feeding, monitor until BM >2.5 3 times

Question 49

Mortality of prematurity

Answer 49

40% die on labour ward if <24w | 95% disability if 23w

Question 50

Causes of prematurity

Answer 50

Unknown cause - 40% Smoking Poverty Malnutrition

Question 51

Manage prematurity

Answer 51

Capable centre O2 and ventilation - nasal, CPAP, mechanical Temperature - plastic bag, incubator Cannula as fluids lost through skin NGT for small feeds - low birth weight formula if <2kg - encourage day 1 expression from mum - aim to remove by 36w - nutritional supplements - vit D and iron, as got from placenta in 3rd trimester, phosphate to prevent osteopoenia Monitor glucose

Question 52

Weights of low birth weight, very low and extremely low

Answer 52

Low <2500g Very low <1500g Extremely low <1000g

Question 53

Normal head and lengths at term

Answer 53

33cm head circ | 50cm length

Question 54

What is small for gestational age, 4 acute complications and long term risks

Answer 54

<10th percentile birth weight for gestational age Risk: hypoglycaemia, hypothermia, foetal death, congenital infection Long term - obesity and CHD

Question 55

Types of iugr

Answer 55

Symmetrical - early pregnancy, head/abdo circs/length proportional Asymmetrical - late pregnancy, head circ > abdo circ and length

Question 56

Causes of IUGR - 4

Answer 56

Poverty Twins Congenital infection Placental insufficiency

Question 57

Complications of prematurity

Answer 57

Immature resp centre - bradycardia, apnoea, desaturate <32w Hypothermia Hypoglycaemia (<2.6) - give TPN and monitor BM for 1st 2 days. Feed early - most of growth in 3rd trimester PDA Infection - IgG mostly crosses placenta in 1st 6m, but can get GBS or nosocomial - coag neg staph or fungal NEC Preterm brain injury Retinopathy of newborn Bronchopulmoanry dysplasia Cerebral palsy OSteopoenia

Question 58

What is NEC, sx, inv and treat, and long term outcome

Answer 58

Lack of bowel flora and immunoglobulins = bacterial invasion and ischaemia. - more likely if not breast fed - feed intolerance, vomiting, bile, distended abdo, blood in stool - perforate - shock - can cause sepsis and death - WCC raised, abn vital signs Broad spec abx, parenteral nutrition, mech vent and circulatory support Echo to check if reduced perfusion due to heart defect Long term - strictures, malabsorption, poor neurodevelomental outcome

Question 59

PDA? Sx, inv and treatment in newborn

Answer 59

Bradycardia, apnoea, difficult to wean off ventilation, bounding pulse, signs of HF Investiate with echo Close with ibuprofen or indomethacin (Pg synthetase inhibitor)

Question 60

Why prematurity hypothermia and consequences

Answer 60

Large surface area, minimal sc fat, naked, thin skin | Can cause hypoxia, hypoglycaemia and unable to gain weight due to increased energy expenditure

Question 61

What is preterm brain injury and what does it cause

Answer 61

IVH +- ischaemia of parenchyma Blood vessels supplying ventricles very thin and cerebral blood flow not yet regulated so changes in systemic circ pressure can cause bleed RF - RDS, pneumothorax Causes hemiplegia, hydrocephalus (head circ increasing rapidly and bulging fontanelle)

Question 62

What is cerebral palsy

Answer 62

Brain injury due to IVH - affects fine motor skills, conc, behaviour, abstract reasoning

Question 63

Inv of osteopoenia of newborn and prevent

Answer 63

Raised ALP | Phosphate supplements to prevent if <1.5kg

Question 64

Risk of GBS infection to baby

Answer 64

10% colonisation | 20-30% of women are colonised

Question 65

When to prevent GBS infection and how?

Answer 65

Proven in pregnancy, or high vaginal swab | - give IV penicillin durin labour

Question 66

Presentation of GBS and manage

Answer 66

First 12h, with pneumonia, PPHN or septic shock | Give resp support and abx

Question 67

What is in heel prick test and when

Answer 67

At 5d old 1. Hypothyroid 2. CF 3. Sickle cell 4. PKU 5. MCADD

Question 68

What to give on NNU discharge

Answer 68

Palivizumab - RSV prophylaxis

Question 69

What is apnoea and causes

Answer 69

No resp effort for >20s, or less time if cyanosis and bradycardia 1. Apnoea of prematurity - most common 2. Central - less CNS stimuli to resp muscles - causing no airflow or chest wall movement 3. Obstructive - pharyngeal instability/collapse, nasal obstruction. No airflow but have chest wall movement 4. Mixed - 50% of term babies

Question 70

Differentials of apnoea - 2

Answer 70

Normal neonatal periodic breathing - 3+ periods with no resp effort lasting 3+s in 20s periods, but no colour or hr change Subtle seizures

Question 71

Specific causes of apnoea

Answer 71

``` Apnoea of prematurity - from day 2-7 CVS - hypotension, hypertension, anaemia, PDA Resp - pneumonia, obstuction, RDS GI Infection CNS - IVH, seizure, hypoxic injury Metabolic Pain Meds - maternal, opiates, prostin Poorly positioned head and neck ```

Question 72

Manage apnoea - 3 monitor, 3 treat

Answer 72

If under 34w, O2 sats monitor until had 1w with no episodes. >34w, only monitor if unstable Apnoea monitor detects abdo wall movement Ensure patent airway Tactile stimulation - rub soles of feet Positive pressure ventilation, high flow nasal cannula Caffeine - relaxes SM and stimulates cardiac muscle and CNS

Question 73

Rf for asthm

Answer 73

``` Low birth weight Family history Bottle fed Atopy Male Pollution ```

Question 74

Features of asthma history

Answer 74

``` Wheeze more than once Night sx Sx between excerbations Triggers Atopy ```

Question 75

Manage asthma

Answer 75

``` Salbutamol spacer 3x a week = add inhaled ICS Review diagnosis and check technique then - LABA and low dose ICS - increase ICS/LABA Montelukast (LTRA), theophylline Increase ICS again Specialist PO prednisolone ``` All - individual asthma plan and inhaler leaflet

Question 76

Emergency asthma trt

Answer 76

``` O2 Salbutamol 5mg (2.5 if <5) nebs with 0.25mg ipratropium bromide 100mg hydrocortisone IV IV Mg Aminophylline Continuous nebs ITU ```

Question 77

Asthma red flags

Answer 77

``` Tiring Hr >200 - senior review Hr dropping - crash call Absent breath sounds Neuro deterioration ```

Question 78

When to discharge with asthma

Answer 78

Off o2 and inhalers >4h intervals

Question 79

Instructions for inhaler <5yo

Answer 79

Spacer and mask Aerosol so shake for 15s to mix with propellant Tilt to open mask for babies 1 spray metered dose Wait 10s or 10 breaths then repeat Wash with warm soapy water and leave to dry and change every 6m

Question 80

Inhaler for >5yo - 3 types

Answer 80

Turboinhaler: powder - suck Easi-breathe: breath activated, doesn't need coordination, can take top off and use as MDI in exacerbation >10oy accuhaler metered dose inhaler

Question 81

What is bronchiolitis

Answer 81

commonest lung inf in infants, exp prem and lung diseae 70% RSV Bronchioles become infected and inflammed with increased mucus secretion so airways blocked

Question 82

Presentation bronchiolitis

Answer 82

Mostly <6mo Low grade fever and coryza then dry cough, fever, wheeze (high pitched, exp>insp), recession, increased rr Can cause resp distress, low sats, nasal flaring End insp crackles Hyperinflation on cxr

Question 83

Inv bronchiolitis

Answer 83

PCR nasopharyngeal secretions - aspirated CXR to rule out pneumothorax Bloods ABG

Question 84

Treat bronchioliiss

Answer 84

O2 if sats <92% IV fluids or NGT May need CPAP Steroids and neb adrenaline but not routine Isolate according to virus No response to salbutamol as not bronchospasm Palivizumab RSV monoclonal antibody in SCID, CHD, NNU as prophylaxis

Question 85

What is CF

Answer 85

Aut recessive Defective gene causing defective protein - CF transmembrane conductance regulator - abnormal Cl transport - sticky mucus builds up outside cell

Question 86

Where does CF affect

Answer 86

Lungs- - mucus build up and impaired ciliary function Pancreas - thick secretions block ducts - pancreatic enzyme def and malabsorption Intestines - Thick meconium - meconium ileus Sweat glands - salty

Question 87

CF presentations - 4

Answer 87

Meconium illeus - bilious vomiting, abdo distension, delay in passing meconium Recurrent chest inf Intestinal malabsorption - pancreatic enzyme deficiency Newborn screening

Question 88

CF diagnosis

Answer 88

1 of: newborning screening OR sibling with CF AND 1 of: increased sweat chloride conc OR 2 identified CF mutations OR abn nasal epithelial ion transport (nasal potential difference)

Question 89

Manage CF

Answer 89

Monitor FEV1 for disease progression Daily physio x2 and exercise Neb DNAase or hypertonic saline to decrease viscosity of secretions for clearance Neb pseudomonal antibodies if colonised Prophylactic abx and rescue abx (PO then IV), central venous catheter if recurrent Pancreatic enzymes - creon Double lung transplant - last resort High calorie diet (150%) - may need gastrotomy to achieve this

Question 90

Lifestyle advice for CF

Answer 90

Avoid others with CF and infected people Avoid stables or compost - aspergillus fumigated Avoid jacuzzis - pseudomonas NaCl tablets in hot weather/exercise Annual flu vaccine Clean and dry nebulisers thoroughly

Question 91

Complications of CF - 5

Answer 91

``` Distal intestinal obstruction syndrome Recurrent resp infections, bronchiectasis, chronic lung disease Low body weight Infertility CF related diabetes ```

Question 92

Manage low body weight in CF

Answer 92

Pancreatic enzyme replacement, high calorie, supplements, NGT/PEG

Question 93

Diffference betweeen constipation and DIOS

Answer 93

DIOS - terminal ileum and prox colon obstuction instead of whole bowel - see faecal loading on axr, palpable mass in RIF = dehydrated thick faeces

Question 94

Cause of DIOS in CF and treatment

Answer 94

Lack of pancreatic enzymes or non compliance Hot weather/salt deficiency Cleared with oral gastrograffin

Question 95

Screening for CF

Answer 95

Guthrie - raised immunoreactive trypsinogen, then screened for common CF mutations. 2 = sweat test = pilocarpine electrical stimulation and sweat collection

Question 96

Viral wheeze timing

Answer 96

<2yo. Not by 5yo as immune to most viruses | Peaks at 5d then gets better by 7-10d, cough can last 4w

Question 97

RF and features of viral wheeze

Answer 97

Smoking in pregnancy Bronchiolitis as baby - makes airways hyperreaactive Associated with RTi

Question 98

Difference of bronchiolitis and wheeze

Answer 98

Fine insp crackles in bronchiolitis

Question 99

Manage viral wheeze

Answer 99

Salbutamol +- steroids trial for 8w Severe = neb salbutamol +- steroids and monitor CXR to check for other cause if not improving, eg pleural effusion (USS) Supportive - fluids, no resp distress, NGT to avoid exhaustion

Question 100

Most common causes of pneumonia in children

Answer 100

Virus most common Newborn - group b strep, gram neg enterococcus Infants - RSV, H infl, strep pn, pertussis, staph a, chlamydia trachomatis >5yo - strep pn, chlamydia trachomatis, mycoplasma pneumoniae All ages - mycobacterium TB

Question 101

Signs of pneumonia - 5

Answer 101

``` poor feeding malaise Resp distress Temp high (>39) End insp coarse crackles ```

Question 102

Inv pneumonia kids

Answer 102

CXR | Sputum/blood culture

Question 103

Treat pneumonia in kids

Answer 103

Mostly viral if <2yo - no abx | Abx - amoxicillin

Question 104

Culture TB

Answer 104

Slow growing mycobacterium Wait 6 weeks Positivity low, especially in children

Question 105

TB in body

Answer 105

Primary complex - heals or progresses Disseminated = lung apices, meninges, spine, nodes - active or dominant Extra-pulm, CNS and miliary more common in kids Normally primary, not latent reactivation

Question 106

Resistance in TB

Answer 106

``` MDR = to R and I XDR = to aminoglycosides and fluoroquinolones ```

Question 107

Signs of TB - 5

Answer 107

``` Malaise Anorexia Failure to thrive Cough Low grade fever ```

Question 108

Screen for TB

Answer 108

Screen for latent - Mantoux test - inject antigens intradermally and look for induration >5mm in 48-72h = type 4 sensitivity reaction - Can be false pos if recent vaccine or active TB - Can be false neg if <6mo Interferon gamma release assays - false negs <5yo CXR if either positive

Question 109

Diag TB

Answer 109

Culture and Ziehl-Neelson stain of sputum x3 - aspirate <5yo in morning as no sputum production CXR

Question 110

Treat TB and prevention

Answer 110

Rifampicin - 6m Isoniazid - 6m Pyrazinamide - 2m Ethambutol - 2m CNS - dexamethasone and 12m abx - check LFT, eyes and HIV first Latent - R and I for 3m as 10% chance of activation Prevention - BCG 65-70% effective, mostly against more severe miliary and CNS

Question 111

What is whooping cough and signs

Answer 111

Bordatella Pertussis, infants or >14yo, droplet spread with 10-14d incubation Week of coryza then spasmodic cough (can last 100), with inspiratory whoop (insp against closed glottis) Vomiting Worse at night or after feeds, better when crying

Question 112

Diagnose whooping cough

Answer 112

PCR nasal swab

Question 113

Complications of whooping cough

Answer 113

``` 100d cough Pneumonia Severe and fatal if <6mo Petechia on cheeks Conjunctival/retinal bleed, seizures, hypoxic encephalopathy from coughing ```

Question 114

Manage whooping cough

Answer 114

Macrolide - erythromycin, within 21d of onset Notifiable Admit if <6m and severe spasms, cyanosis attacks

Question 115

Upper airway obstuction acute managemet

Answer 115

Calm Don’t examine throat Organised Observe for hypoxia or deterioration Neb adrenaline and alert anaesthetist if severe Tracheal injubation if resp failure from secretions, obstruction or exhaustion

Question 116

Features of common cold and trt

Answer 116

Clear or purulent nasal discharge and blockage Persistent cough for 4w Viruses - rhinovirus, coronovirus, RSV Reassure parents, paracetamol, ibuprofen

Question 117

Most common cause of sore throat

Answer 117

Pharyngitis - inflamed pharynx and soft palate Local nodes enlarged and tender Viruses - adenoveritus, enterovirus Older - group a B-haemolytic strep

Question 118

What is croup, main danger, cause and age range

Answer 118

Laryngotracheobronchitis Mucosal inflammation and increased secretions Most dangerous = oedema in supraglottic area which can cause narrowing of trachea 95% viral - parainfluenza, RSV 6m-6yo in autumn

Question 119

Signs of croup

Answer 119

Fever and coryza, onset over days, then - Barking cough Harsh stridor and hoarseness. If at rest = bad Sx worse at night Resp distress

Question 120

Manage croup

Answer 120

Admit if <12m, upper airway obstuction or unsure about diagnosis Steroids - oral and neb reduce severity and duration Neb adrenaline if stridor at rest O2 if agitated

Question 121

Differentials for croup and key differences

Answer 121

Bacterial trachietis - staph a, pseudomembranous, high fever, toxic, rapidly progressive obstruction Acute epiglottitis - over hours, cannot drink, salivating, toxic and very ill, temp 38.5, muffled voice, soft whimpering stridor

Question 122

What is acute epiglottitis

Answer 122

Life threatening emergency | Intense swelling of epiglottis and surrounding tissues by H influenzae B

Question 123

Sx of epiglotitis

Answer 123

``` No cough Very painful throat - can’t swallow or speak Very ill and acute Temp >38.5 Soft inspiratory stridor Drooling Distress ```

Question 124

Manage epilottitis

Answer 124

Urgent admission and contact anaesthetist, paediatrician, ENT ICU Intubate under GA or tracheostomy Blood cultures and IV cefuroxime/ceftriaxone

Question 125

Causes of wet cough

Answer 125

<6yo Acute - viral, bacteria, pertussis Subacute - 3-8w, should be resolving - post-viral, or pneumonia Chronic >8w - bronchiectasis: CF, primary ciliary dyskinesia, untreated infection (ISS), persistent collapse - persistent bacterial bronchitis - ?primary ciliary dyskinesia - recurrent aspiration - immune problem - recurrent/unusual infections

Question 126

What is linked to neonatal rhinitis and bronchitis?

Answer 126

Kartagener syndrome - primary ciliary dyskinesia: 1) sinusitis - neonatal rhinitis, persistent nasal discharge 2) bronchitis 3) dextracardia Heterogenous genetic make up and manifestation Assoc with CHD, asplenia, hydrocephalus, renal disease

Question 127

Causes of pleural effusions - 3

Answer 127

Infectious - parapneumonic (pneumonia, lung abscess, bronchietasis), - non-TB bacterial pneumonia - staph a, strep pn, h infl (<5yo), group a strep (>5) - CXR if pneumonia and not improving after 48h of treatment Malignancy Congestive heart failure

Question 128

3 stages of parapneumonic effusion

Answer 128

1. Exudative - clear fluid from pneumonia, low WCC 2. Fibropurulent - complicated - deposition of fibrin in pleural space causing septations and loculations - increased WCC - thickened fluid = pus (empyema) 3. Organisational - fibroblasts infiltrate and intrapleural membranes are reorganised to become thick and nonelastic - prevent re-expansion - impair function - persistent pleural space with potential for infection - spontaneous healing or chronic empyema

Question 129

Investigate pleural effusion

Answer 129

``` CXR Confirm on chest USS Blood culture including anaerobes Sputum culture FBC - anaemia, WCC, platelets Electrolytes - SIADH? Antistreptolysin O titre Diagnostic tap if suspect malignancy, pleural fluid microbiology Chest CT if plan to operate ```

Question 130

Manage pleural effusion

Answer 130

IV abx then PO Chest drain Intrapleural fibrinolyic is complicated effusion

Question 131

Inhaled foreign body - features and inv

Answer 131

Average 3yo Quick onset, choke, cough CXR - shadowing, compare insp and exp film to see what’s not deflating (blocked) Bronchoscopy to identify object

Question 132

Positive cardiac screening

Answer 132

``` RR >60 Apnoea >20s at a time or with cyanosis Recession, nasal flaring Cyanosis Visible pulsation, heaves, thrills on precarious Murmur - absent if large defect Absent/weak femoral pulses Pulse ox at 6-12h - inv if difference between limbs >2% or o2<95% (esp in otherwise well child) ```

Question 133

RF for cardiac disease - 5

Answer 133

``` Antenatal scans or genetic abnormalities Family history Teratogens or substances Conditions - epilepsy, diabetes, SLE Viruses in 1st trimester ```

Question 134

Circulatory changes at birth

Answer 134

Before birth, LA pressure is low as minimal blood return from lungs, so blood shunts through foramen ovale from RA to LA At birth, resistance to pulmonary flow decreases so blood goes here from RA, then LA pressure increases, closing FO Ductus arteriosus closes in first hour so f lif - prevent with prostaglandins (prostin), close with NSAIDs (ibuprofen)

Question 135

Signs of innocent murmur

Answer 135

``` [4S] Systolic left Sternal edge aSymptomatic Soft Gone by 24hold, often heard in febrile illness or anaemia due to increased CO ```

Question 136

Signs of pathological murmur and inv

Answer 136

Loud, wide area, harsh, other findings | Echo, CXR, ECG

Question 137

Which heart abnormalities are critical and why

Answer 137

PDA dependent - baby will deteriorate when it closes around 2d old: - pulmonary atresia - hypoplastic left heart - tetralogy of fallot - transposition of great arteries - tota anomolous pulmonary venous drainage (into RA) - tricuspid atresia - truncus arteriosus

Question 138

What murmur is diastolic, left sternal border machinery

Answer 138

PDA

Question 139

Which murmur is ejection systolic, fixed splitting of 2nd heart sound

Answer 139

ASD

Question 140

Which murmur is pansstolic, lower left sternal border

Answer 140

VSD

Question 141

Which murmur is ejection systolic, upper left sternal border

Answer 141

Pulmonary stenosis

Question 142

Which murmur is upper left border, crescendo decrescendo

Answer 142

Coarctation of the aorta

Question 143

ASD murmur and sx

Answer 143

Ejection systolic upper left sternal edge, fixed splitting of 2nd heart sound Apical pancystolic murmur from AV regurg if partial AVSD Often asymptomatic, may have recurrent chest inf/wheeze or arrhythmias in adulthood

Question 144

VSD murmur

Answer 144

Pansystolic, left lower sternal border

Question 145

PDA murmur, pulse and complications

Answer 145

Machinery, diastolic, upper left sternal border beneath left clavicle Increased pulse pressure - bounding HF and pulmonary htn >1m after EDD = persistent PDA

Question 146

Pulmonary stenosis murmur

Answer 146

Ejection systolic | Upper left sternal border

Question 147

Coarctation of the aorta murmur

Answer 147

Crescendo decrescendo | Upper left sternal border

Question 148

Aortic or pulmonary stenosis presentation in child

Answer 148

Normally well

Question 149

2 CHD causes of sick neonate collapsed with shock

Answer 149

Coarctation | Hypoplastic left heart

Question 150

3 Causes of left to right shunt and presentation

Answer 150

Breathless or asymptomatic | ASD, VSD, PDA

Question 151

CHD causing breathless but not cyanosis child

Answer 151

Left to right shunt - ASD, PDA, VSD

Question 152

CHD causing blue child

Answer 152

Right to left shunt - Tetralogy of fallot, TGA Mixing: AVSD, complex congenital heart disease

Question 153

Symptoms of heart faiure in child

Answer 153

Breathless. Increasing on exertion or feeding Sweating Poor feeding - interrupted - sweat, become breathless and pale Vomiting Recurrent chest infections Palpitations or chest pain

Question 154

Signs of HF in child

Answer 154

``` Tachycardia, tachypnoea Peripheral cyanosis Poor weight gain Murmur or gallop rhythm Hepatosplenomegaly ```

Question 155

Causes of heart failure in infant - 4

Answer 155

Obstructed duct dependent circulation - hypoplastic left heart, - critical aortic valve stenosis, - severe coarctation, - interruption of aortic arch

Question 156

Causes of heart failure in child - 3

Answer 156

Left to right shunt - ASD, VSD, large PDA

Question 157

Causes of heart failure in adoelscent - 3

Answer 157

Eisenmenger Rheumatic fever Cardiomyopathy - unexplained deaths in family <30yo? Pacemakers <50yo?

Question 158

Presentation of CHD

Answer 158

``` Antenatal USS 18-20w then detailed echo Murmur HF Shock Cyanosis ```

Question 159

Causes of CHD

Answer 159

Maternal disorder - diabetes, SLE, rubella Maternal drugs - warfarin, foetal alcohol syndrome Chromosomal - Down’s, Edwards, Patau’s, Turner’s

Question 160

Inv PDA

Answer 160

CXR and ECG normal | Echo to visualise duct

Question 161

Close PDA and why

Answer 161

Decrease risk of bacterial endocarditis and pulmonary vascular disease With coil or occlusion device around 1y NSAIDs

Question 162

2 types of ASD

Answer 162

Secundum - centre of septum, involves foramen ovale | Primum - partial AVSD - between bottom of atrial septum and AV valves, abnormal valves

Question 163

Inv ASD

Answer 163

CXR - cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings ECG - secundum = partial RBBB and right axis deviation, from RV enlargement Echo - anatomy. Main diagnostic tool

Question 164

Manage ASD

Answer 164

Treat if causing RV diataion Secundum - cardiac catheterisation and occlusive device Primum - surgical correction Treat at 3-5yo to prevent RHF and arrhythmias

Question 165

VSD - what is small? Sx and inv and trt of small VSD

Answer 165

<3mm = small Asymptomatic Loud pansystolic murmur at lower left sternal edge, quiet P2 ECG and cxr normal, echo shoes abnormality Close spontaneously Prevent bacterial endocarditis by good dental hygiene

Question 166

Large VSD sx, inv, treat

Answer 166

Same size or bigger than aortic valve HF with SOB and failure to thrive, recurrent chest infections. Always cause pulmonary hypertension Signs - tachycardia, tachypnoea, active recording, hepatomegaly, soft pansystolic murmur, apical middiastoic murmur, loud p2 CXR shows cardiomegaly, pulm vessel enlarment and oedema ECG shows biventricular hypertrophy by 2mo (tall qrs) Echo shows defect Diuretics for HF and captopril (ACEi) Additional calorie input Surgery at 3-6m to treat pulmonary htn, HF and prevent long term damage

Question 167

Features of tetralogy of fallot and sx

Answer 167

VORP: VSD, Overriding aorta, Rv hypertrophy, subPulmonary stenosis causing RV outflow obstruction Sx: cyanosis, hypercyanotic spells (TIA, stroke, MI, death) followed by sleep, squat on exercise Signs - clubbing, loud harsh ejection systolic murmur at left sternal edge from day 1

Question 168

Diagnosis of ToF

Answer 168

Antenatally

Question 169

Inv ToF

Answer 169

CXR - small boot shaped heart from RV hypertrophy, increased pulmonary vascular markings ECG - normal at birth, then RV hypertrophy Echo - features

Question 170

Manage ToF

Answer 170

<6mo - medical, tube from subclavian to pulmonary artery or RV outflow balloon dilatation if cyanosis at birth 6m - definitive surgery to close VSD and relieve outflow obstruction Hypercyanotic spells - treat if >15m with - sedation, pain relief - IV propanolol - fluids, bicarbonate - artificial ventilation to reduce metaboic o2 demand

Question 171

Transposition of great arteries abnormalities

Answer 171

ASD, VSD and PDA to allow mixing

Question 172

Presentation and inv for TGA

Answer 172

Day 2 when PDA closes so reduced mixing - cyanosis, second heart sound loud, no murmur Inv - cxr = narrow mediastinum and egg shaped heart, increased pulmonary vascular markings. ECG normal. Echo shows vessels

Question 173

Manage TGA

Answer 173

Maintain mixing with prostin infusion or balloonatrial septoplasty to make FO flap incompetent Operate in first few days of life

Question 174

Complication of left to right shunt and treatment

Answer 174

Eisenmenger: sx from shunt decreases as grow, but pulm arteries become thickened and resistance increases. 10-15yo, shunt reverses and become cyanosis Life expectancy 40-50yo Treat high pulmonary blood flow, transplant

Question 175

What is AVSD and common associated condition

Answer 175

Common in Down’s syndrome Failure of endocarditis cushions - 5 leaflet valve between atria and ventricles Causes pulmonary hypertension

Question 176

Presentation of AVSD and treatment

Answer 176

``` Antenatal USS Cyanosis at birth HF att 2-3w of life No murmur Medical management for HF and surgical repair at 3-6m ```

Question 177

What is tricuspid atresia

Answer 177

Absence of tricuspid valve - small and nonfunctioning right heart Need ASD and VSD to live Mix blood through ASD = cyanosis

Question 178

Manage tricuspid atresia

Answer 178

Shunt from pulmonary to subclavian artery, reducing cyanosis Pulmonary artery banding reduces breathlessness Fontan proceedure - IVC and SVC go into pulmonary arteries

Question 179

Presentation of aortic stenosis

Answer 179

Aortic valve leaflets partly fused, restricting exit from LV May be critical stenosis - duct dependent (HF) Asymptomatic Chest pain/reduced exercise tolerance/syncope on exertion if severe Signs - small volume slow rising pulse, ejection systolic murmur, carotid thrill

Question 180

Inv aortic stenosis

Answer 180

CXR - post-stenotic dilatatation of ascending aorta, enlarged LV ECG - LV hypertrophy

Question 181

Manage aortic stenosis

Answer 181

Balloon valvotomy if sx on exercise or high resting pressure gradient May require valve replacement eventually HF - diuretics and digoxin, plus IV furosemide if still sx

Question 182

Findings of pulmonary stenosis on exam and inv

Answer 182

Asymptomatic Ejection systolic murmur, ejection click, RV heave if severe CXR - normal or post-stenosis dilatation of pulmonary artery ECG - RV hypertrophy

Question 183

Manage pulmonary stenosis

Answer 183

When pressure gradient very high, with transcatheter balloon dilatation

Question 184

What is coarctation of the aorta

Answer 184

Arterial duct tissue encircles aorta at point of duct insertion - aorta constricts when duct closes

Question 185

Features of CoA and associated condition

Answer 185

Present at d2 with collapse when duct closes VSD usually present Radiofemoral delay Assoc with Di George

Question 186

Manage CoA

Answer 186

Stent or surger by 5yo to avoid pulmonary hypertension

Question 187

Hypoplastic left heart syndrome?

Answer 187

Underdevelopment of left side of heart: - LV very small - mitral valve very small - aortic valve atresia - Ascending aorta small and coarctation

Question 188

Diagnosis of HLHS and presentation

Answer 188

Antenatal USS | Profound acidosis and CVS collapse at birth

Question 189

Manage hlhs

Answer 189

Neonatal - Norwood procedure | 6m or 3y - Fontan procedure

Question 190

Sx of inf endocarditis and who is at risk

Answer 190

``` Risk if CHD Fever, anaemia Clubbing, splinter haemorrhages Necrotic skin changes Splenomegaly ```

Question 191

Most common cause of inf endocarditis

Answer 191

Strep viridans

Question 192

Manage inf endo

Answer 192

Penicillin and aminoglycoside IV 6w

Question 193

Cause of cardiomyopathy

Answer 193

Dilated - inherited` secondary to metabolic disease or viral infection

Question 194

Features of dilated cardiomyoathy

Answer 194

Large poorly contracting heart | Suspect if HF and dilated heart

Question 195

Treatment dilated cardiomyopathy

Answer 195

Diuretics, ACEi, carvedilol (b blocker)

Question 196

Treat myocarditis

Answer 196

Most improve spontaneously | May need a transplant

Question 197

Causes of pulmonary hypertension - 4 classes

Answer 197

1) Pulmonary artery - pressure high from heart - VSD, AVSD, PDA idiopathic, HIV 2) Pulmonary vein - pressure must be higher to return to heart - left sided heart disease, pulmonary vein stenosis or compression 3) Pulmonary thromboembolism 4) Respiratory - chronic obstructive or premature lung disease, interstitialung disease, upper airway obtruction, sleep apnoea

Question 198

Manage pulmonary hypertension

Answer 198

Fix shunts if present - at 6 months Inhaled nitrous oxide IV magnesium sulphate Sildenafil - oral phosphodiesterase inhibitor Bosentan - endothelin receptor antagonist GMP Anticoagulation - heparin, aspirin or warfarin Heart and lung transplant only option if not treated and becomes pulmonary vascular disease

Question 199

Features of colic

Answer 199

First few weeks of life, resolves by 4mo Crying, pulling up legs, excessive flats for >3h on 3+ days a week Assoc with feeding difficulties

Question 200

Manage colic

Answer 200

Grandparent involvement, reduce stress, reassurance Movement Let baby finish first breast first Severe and persistent could be CMPI or GORD - 2w trial of protein hydrolysed formula and consider GOR treatment trial

Question 201

Crying - how to help parents - 5

Answer 201

``` Take concerns seriously Help them recognise hungry/tired Encourage vocal, vestibular or tactile stimulation Baby centred approach Involve health visitor ```

Question 202

Causes of excessive crying - 5

Answer 202

``` Infection - middle ear, URTI, meningitis Unrecognised fracture Oesophagitis Severe nappy rash and constipation Testicular torsion ```

Question 203

Constipation description

Answer 203

<3 stool a week Or excessive hardness with strain/pain With reluctance to feed Long standing - overdistended - reduced urge to defecate

Question 204

Causes of constipation

Answer 204

``` Function - lack of fibre, poor diet, reduced fluid intake, ignoring urge Hypothyroid Hypocalcaemia Painful fissure Anorectal abnormalities eg atresia Hirschprung’s ```

Question 205

Red flag sx for constipation

Answer 205

Failed to pass meconium in first 48h - Hirschprung’s Failure to thrive - hypothyroid, coeliac Neuro: Abnormal lowerlimb neuro/deformity - lumbosacral pathology Sacral dimple/central pit - spina bifida occulta Anus: Abnormal position, patency, appearance - Anatomy Bruising or multiple fissures - abuse

Question 206

Examination for constipation

Answer 206

Palpable mass/distinction - constipation, Hirschprung’s DRE by specialist if suspect pathology Spine - spina bifida occulta

Question 207

Manage constipation

Answer 207

``` Advice - - obey call to stool (toilet retraining) - gastrocolic reflex - dietary fibre and fluid 1st line laxative to evacuate overloaded rectum completely - macrogol softener eg polyethylene glycol and electrolytes (movicol paediatric plan). Escape over2w until resolved impaction 2nd - stimulant senna or sodium picosulphate Follow treatment with maintenance Enema if oral fails ```

Question 208

Dehydration 5 risks

Answer 208

``` Low birthweight and <6mo 6+ diarrhoea stools in 24h 3+ vomits in 24h Unable to tolerate fluids Malnutrition ```

Question 209

Why dehydration in kids?

Answer 209

Bigger surface area to weight ratio - higher insensible water losses Higher basal requirements Immature renal tubular reabsorption

Question 210

How much insensible losses and basal fluid requirements

Answer 210

Insensible losses - 300ml/kg2/d or 15-17ml/kg/d | Basal requirements - 100-120ml/kg/d

Question 211

Dehydration %s

Answer 211

``` 5-10% = clinical dehydration >10% = shock ```

Question 212

3 types of dehydration

Answer 212

Isonatraemic - proportional losses Hyponatraemic - diarrhoea/drinking hypotonic solution = sodium lost more than water - fall in plasma sodium - water shifts from ECF to ICF - increase in brain volume and shock Hypernatraemic - high insensible losses - fever/hot/dry envt or profuse low-sodium diarrhoea - water loss > sodium loss = hypertonic ECF compared to ICF so water shifts to ECF = reduced signs of dehydration - water drawn out of brain and cerebral shrinkage = jitttery, increased tone and reflexes, altered consciousness, multiple small cerebral haemorrhages

Question 213

Investigate dehydration

Answer 213

Stool culture if septic, blood/mucus, immunocompromised Plasma electrolytes, urea, creatinine, glucose if going to give IV fluids or suspect hypernatraemia (neuro signs) Bloodculture ifstarting abx

Question 214

MAnage dehydration

Answer 214

Oral rehydration - fluid deficit replacement (50ml/kg) over 4h as well as maintenance IV fluids - bolus if shock, and replace deficit over 24h Fluid replacement over 48h if hyperntaramia to avoid cerebral oedema and seizures and monitor sodium Abx if sepsis, extraintestinal bacterial spread, salmonella gastritis <6mo, malnourished or immunocompromised, specific bacteria or protazoa eg shigella, cholera, giardiasis, c dif with pseudomembranous colitis No antidiarrhoeals or anttiemetics as will prolong excretion of bacteria

Question 215

Fluid requirements

Answer 215

Maintenance: 100ml/kg/24h for first 10kg 50ml/kg/24h for next 10kg 20ml/kg/24h for the rest Emergency boluses: 20ml/kg/24h (1st 10kg)- 20ml/kg/24h (2nd 10kg) - 10ml/kg/24h (other) Correct fluid deficit: weight x % deficit x 10

Question 216

CAuses of failure to thrive and what is it

Answer 216

Head circ preserved relative to weight Drop 2 centile 1) inadequate intake - environmental, suck/swallow or anorexia from chronic illness 2) excessive loss - diarrhoea, vomitingg 3) malabsorption - coeliac, CF 4) inadequate use eg syndromes 5) excess requirements eg malignancy, CF, chronic infection, resp, renal failures

Question 217

Initial managemen of failrule to thrive - 3

Answer 217

Increase energy intake Diet and behavioural modification Monitoring growth

Question 218

Contributing factors to failure to thrive

Answer 218

Difficulty at home - neglect, abuse, deprivation Feeding - unskilled, inadequate breast milk Physiological - idiosyncratic growth pattern, normal child (look at family, preterm, alert and responsive)

Question 219

History in failure to thrive

Answer 219

``` Food diary Process of feeding Diarrhoea, vomiting, cough, lethargy Iugr, premature Normal development Family Psychosocial problems at home ```

Question 220

Examination for failure to thrive

Answer 220

Malabsorption - distended abdo, thin buttocks, miserable Chronic resp disease - chest deformity, clubbing Signs of Hf or nutritional deficiencies

Question 221

Inv failure to thrive

Answer 221

Bloods: FBC, ferritin, UE, glucose, LFT, calcium, TSH, crp Immunoglobulins MSU ``` Specific testS: IGF-2 - eg IBD Coeliac serology Sweat test Stools ``` Imaging: CXR Renal/CNS USS Skeletal survey - dwarfism, abuse

Question 222

Manage failure to thrive

Answer 222

GP, health visitor, paediatric dietician, clinical psychologist Admit if <6m and severe, require active feeding

Question 223

commonest causesss of gastroentteritis

Answer 223

Rotavirus (most) Norovirus (most in adults) Astrovirus, adenovirus Bacteria causes - less common in developed countries - blood in Stools - Campylobacter jejuni - severe abdo pain - shigella and salmonella - blood and pus, pain and tenesmus - cholera and E. coli - profuse, rapidly dehydrating diarrhoea

Question 224

Presentation of gastroenteritis

Answer 224

``` Loose,watery stools Vomiting High pitched cry and inconsolable - hypernatraemia No fever History of contact with DV/abroad Dehydration and shock ```

Question 225

Assess dehydration and shock

Answer 225

``` Weight loss CRT, BP, HR, weak pulse, rr UO Skin turgor, mucus membranes, fontanelle Pale/mottled, sunken eyes - assesses CSF ```

Question 226

Mimics of gastroenteritis

Answer 226

systemic - sepsis Local inf - resp, otitis media, hep a, uti Surgical - pyloric stenosis, intussusuceptiotn, appendicitis, NEC, Hirschprung’s Metabolic - dka Renal - hus

Question 227

Inv gatroentereitis

Answer 227

Clinical diagnois Stool culture if septic appearance or travel hx or no improvement in 7d- bacteria, ova, cysts, parasites Bloods and UEs if requiring fluids Blood culture if starting abx

Question 228

Manage gastroenteritis

Answer 228

Monitor weight continue breastfeeding ORT - diarolyte fluid challenge (5ml 5mins) then 50ml/kg over 4h NGT if won’t take ORT Fluids - dextrose, saline, potassium Ondansetron to reduce - vomit, need for fluids, need for admission Monitor UE, creatinine, glucose Hypernatraemia - half rehydration rate - so rate = (maintenance x2 + rehydration)/48 = hourly

Question 229

Complications of gastroenteritis

Answer 229

Dehydration Malnutrition Temporary sugar intolerance after DV with explosive water acid stools - give temporary lactose free diet Post-enteritis enteropoathy - resolves spontaneously

Question 230

Features of GORD

Answer 230

Recurrent regurg/vomiting but still gaining weight as normal and otherwise well Common in infancy Due to inappropriate relaxation of lower oesophageal sphincter due to functional immaturity

Question 231

4 risk factors for GORD

Answer 231

Mainly fluid Mainly horizontal posture Short intra-abdo length of oesophagus Malrotation/previous exomphalos

Question 232

Differentials for GORD

Answer 232

CMPI | Physiolgical, overfeeding

Question 233

Inv GORD

Answer 233

Clinical diagnosis, observe feeds If atypical hx, complications or failure of treatment response: - 24h oesophageal ph monitoring - endoscopy with biopsy to identify oesophagitis

Question 234

Complications of GORD

Answer 234

Failure to thrive - bleeding = iron deficiency Oesophagitis - heamatemeiis, heartburn, iron deficiency anaemia Pulmonary aspiration - pneumonia, cough, wheeze, apnoea Dystonic neck posturing - Sandifer syndrome

Question 235

Manage GORD

Answer 235

Mild - reassure, avoid overfeeding, feed upright, add thickeners Significant - H2 antagonist ranitidine or PPI omeprazole. Infant gaviscon If medical therapy fails or oesophageal stricture = Nissen fundoplication

Question 236

What is coeliac disease

Answer 236

Gluten sensitive AI diseae of SI T cell mediated Immunological response to environmental factor (gliadin) and genetics - HLADQ2/8

Question 237

Prestaiotan of coeliac diseae

Answer 237

``` Chronic Pale, loose stools 2-3x/d Faiure to thrive Vague abdo pain Pallor ```

Question 238

Rf for coeliac diseae

Answer 238

Family | AI - diabetes, thyroid

Question 239

Coeliac inv findings

Answer 239

Histology - crypt hyperplasia and villus atrophy, intraepithelial lymphocytes - on SI biopsy Serology - tTG antibodies, endomyseal antibodies Malabsorption Sx resolve and growth catches up with gluten withdrawal

Question 240

complications of coeiac

Answer 240

Anaemia - iron/folate def Osteoporosis Cancers - small bowel lymphoma

Question 241

Main causes of encropresis and soiling and manage

Answer 241

Constipation - give osmotic laxative macrogol | Behavioural - healthy toileting habits

Question 242

What is functional abdo pain and manage

Answer 242

All inv normal, no weight loss or bowl sx Periumbilical, doesn’t move or change Brief, no recognisted trigger Screen forcoeliac and constipation, reassure nd explain

Question 243

UC and crohn’s presentaitaon

Answer 243

UC - rectal bleeding, diarrhoea, colicky pain, weight loss, growth faiure CD - lethargy, ill health, GI sx

Question 244

Inv UC

Answer 244

Rule out infective colitis Endoscopy - confluent pancolitis Histology - mucosal infl, crypt damage, ulceration SI to rule out extracolic inflammation - CD

Question 245

Locations of CD and effects

Answer 245

Mouth - oral facial granulomatosis Small intestines - delayed puberty Distal proctitis - tenesmus and watery diarrhoea Lungs - granulomatous, transmural - strictures, fistulae Mostly distal ileum and prox colon

Question 246

Inv crohn’s

Answer 246

Inflammatory markers iron def anaemia serum low albumin Upper GI endoscope and ileocolonoscopy = non-caseating epithelioid granulomas SI imaging = narrowing, fistulae, thickening, mucosal irregularities

Question 247

Manage UC

Answer 247

Mild - aminosalicylates (mesalazine) for induction and maintenance, +- topical steroids if confined to rectum and sigmoid Acute exacerbation - systemic steroids and immunomodulators (azathioprine) Maintain remission - immunomodulators (azathioprine) +- low dose steroids Severe - biologics (infliximab, ciclosporin) for resistant disease Surgery if ineffective Severe fulminating = emergency - fluids, steroids, ciclosporin Colonoscopic screening regularly 10y after diagnosis as risk of adenocarcoma

Question 248

Manage crohn’s

Answer 248

Fully liquid diet for 6-8w Steroids Immunosuppresssion - azathioprine, mercaptopurine Anti-TNF - infliximab, adalimumab Enteral nutrition to correct growth failure surgery

Question 249

Differentials for malabsorption

Answer 249

Poor diet, iron deficiency | Protein losing enteropathy ie lymphangiectasia - secondary to heart disease, antitrypsin in stool

Question 250

When to suspect lactase deficiency

Answer 250

First feed = watery diarrhoea

Question 251

Causes of fat malabsorption - 2

Answer 251

Cholestatic liver disease or bilailry atresia - bile salts dont enter duodenum Lymphatic leak or obstuction prevents chylomicrons reaching thoracic duct and systemic circulation - lymphangiectasia

Question 252

Cause of nutrient, water, electrolyte malabropstiotn - 4

Answer 252

Short bowel syndrome from resection (congenital abn or NEC) Loss of terminal ileum from CD resection - lack of bile acid and B12 absorption Exocrine pancreatic dysfunction eg CF - lipase, protease, amylase Loss of absorptive area - coeliac

Question 253

MEsenteric adenitis - what is it and featuress

Answer 253

? Exist? Large mesenteric nodes on laparoscopy, normal appendix Non specific/RIF pain, assoc with cervical lymphadenopathy and URTI, resolves in 24-48h

Question 254

What is the most common cause of diarrhoea in toddlers and what is its cause

Answer 254

Toddler/chronic nonspec diarrhoea Persistent loose stools, varying consistency, often with undigested vegetables Children well and thriving Dysmotility of gut and fast transit diarrhoea, improves with age

Question 255

Differentials for toddler diarrhea - 3

Answer 255

coeliac Excessive fruit juice Temporary cows milk allergy after gastroenteritis

Question 256

What is typhoid and diagnosi

Answer 256

Salmonella typhi - gr neg bacilli Foecal oral transmission, <10d incubation Diagnose = culture of: bile, blood, bone marrow aspirate (gold standard)

Question 257

Treat typhoid

Answer 257

3rd gen cephalosporin ofloxacin

Question 258

Causes of soiling - 4

Answer 258

Developmental - haven’t learnt or physical/learning difficulty Biological - GI illness, fistula, previous pain, now constipation and overflow diarrhoea Behavoral - fear, ignore signs Contextual - stressful life events

Question 259

Manage soiling

Answer 259

Toilet training - star chart, foot support | Constipation - fluids, laxative, diet

Question 260

Tropical infections with short incubation periods

Answer 260

``` <10d Gastro Typhoid Plague Viral haemorrhagic fevers - lassa, dengue, Ebola ```

Question 261

Tropical inf with medium incubation period

Answer 261

21d Malaria Trypanosomiasis Leptospirosis

Question 262

Tropical inf with long incubation period

Answer 262

``` >21d Viral hepatitis Malaria - dormant in liver TB HIV Schistosomiasis Amoebic liver abscess ```

Question 263

Suggestive features in acute abdo pain

Answer 263

``` Hard faeces - constipation African/Mediterranean - sickle cell Recent URTI - renal disease Periodic and vomiting - abdo migraine Travel - TB PICA - do blood lead level and ferritin ```

Question 264

3 categories of acute abdo pain causes and examples of each

Answer 264

Surgical - intussuseption, appendicitis, inguinal hernia, peritonitis, inflamed meckel’s diverticulum Medical - dka, HSP, gastroenteritis, IBD, uti, pyelonephritis, sickle cell, mesenteric adenitis Extra-abdo - URTI, lower lobe pneumonia, testicular torsion, menstruation or PID

Question 265

Tests in acuteabdo pain

Answer 265

Urine dip AXR, USS, CTKUB Barium Bloods - FBC, CRP

Question 266

Symptoms of appendicitis

Answer 266

Anorexia Slight vomiting Central and colicky pain then RIF, worse with movement

Question 267

Signs of appendicitis

Answer 267

Flushed face with oral fetor Fever 37.2-38 Tenderness and guarding in RIF - McBurney’s point

Question 268

Diagnosis of appendicitis - 5

Answer 268

Clinical - Rosvig sign Axr - foecolith, perforation FBC Urine dip - leukocytes can be raised as appendix can be near bladder or ureter, nitrates shouldn’t be raised USS - thick and noncompressible appendix with increased blood flow

Question 269

Treatment appendicitis

Answer 269

Appendectomy if. Uncomplicated | Compicated - mass, abscess, perforaiotan, generalised guardian = fluid resusc and iv abx

Question 270

Primary damage causing head injury

Answer 270

Penetration injury BV injury - EDH, SDH, SAH Neural tissue - diffuse axonal injury, focal cerebral contusion/laceration

Question 271

Secondary damage causing head injury

Answer 271

``` Cerebral oedema Hypotension Hypoxia Seizures Hypoglycaemia Infection ```

Question 272

Urgent CT head for child indications

Answer 272

Poor breathing effort, unresponsive/only to pain Suspect NAI Post-traumatic seizure GCS<14 initially of <15 2h later Suspected open or depressed skull fractur, sign of basal skull fracture (haemotympanum, Battle’s sign, CSF leak) Focal neuro signs <1yo with bruising or swelling >5cm on head

Question 273

Head injury indications for admission and observation

Answer 273

``` Amnesia >5m Loc >5m witnessed ABnomal drowsiness 3+ vomiting Dangerous mechanism of injury ```

Question 274

Process of testes migration and when it goes wrong

Answer 274

Form on urogenital ridge of post abdo wall, migrate towards inguinal canal guided by gubernaculum (under influence of AMH). Then enveloped by processus vaginalis (tongue of peritoneum) which is obliterated after birth - failure = inguinal hernia or hydrocoele

Question 275

Cause of inguinal hernia and who/where

Answer 275

Patent processus vaginalis | More often in boys, premature, on right side

Question 276

Presentations of inguinal hernia - 4

Answer 276

Intermittent swelling in groin or scrotum on straining or crying Irreducible, firm and tender lump lateral to pubic tubercle Groin swelling may become more prominent on raising intra abdo pressures pressing on abdo May be unwell, irritable, vomit

Question 277

Manage inguinal herniae

Answer 277

Sustained gentle compression under opioid anaesthesia will reduce most Surgery delayed for 24-48h to allow oedema to reduce Emergency surgery if not reducing as risk of strangulation: - within 2d if <6w - within 2w if <6m (narrower canal = more likely to incarcerate) - within 2m if <6y

Question 278

umbilical hernia - management

Answer 278

Large or symptomatic - operate at 2-3yo incarcerated - reduce by compression and operate within 24h Most will resolve by 4-5yo, if not, refer to surgery

Question 279

Diaphragmatic hernia - inv and treatment and complication

Answer 279

X-ray at birth = opacification as no air entered bowel yet. At 6h = bowel loops in chest Causes pulmonary hypoplastic as impeded development and pulmonary htn Treat with iv suxamethonium to stop breathing at birth and prevent air entering lungs ECMO into jugular vein and carotid

Question 280

What Is Hirschprung’s disease and 4 complications

Answer 280

Congenital absence of ganglion cells from myenteric and submucosal plexus in a segment of colon. 75% rectosigmoid, 10% entire colon Causes narrow and contracted segment, functional GI obstuction, constipation and megacolon

Question 281

Presentaitaon of hirschprung’s diseae

Answer 281

Failure to pass meconium within 24h Abdo distension Bile stained vomit - late

Question 282

complications of Hirschprung’s - 5

Answer 282

``` GI perforation Bleed Ulcer Enterocolitis — life threatening, sometimes c dif Short gut syndrome after surgery ```

Question 283

Test/Inv for Hirschprung’s

Answer 283

Faeces on abdo exam DRE = tight anal sphincter, explosive discharge of stool and gas on withdrawal Diagnose with rectal suction biopsy of agangiolic section, with staining for ache +ve nerve excess Anorectal manometry or barium studies -gives idea of length of segment

Question 284

Treat Hirschprung’s

Answer 284

Excision of aganglionic segment and anastamosing normally innervated bowel to anus May need colostomy

Question 285

What is intussusception and where

Answer 285

Invagination of prox bowel into distal segment - telescope | Commonly ileum into caecum via ileocaecal valve

Question 286

Presentation of intussusception

Answer 286

Most common cause of intestinal obstruction in kids, peak at 3m-3yo - paroxysmal, severe colicky pain and pallor - pale around mouth and draws up legs during episode,s then lethargic afterwards - Refusal of feeds - vomitting , can be bile stained - dehydration - palpable sausage shaped mass - red current jelly stool with blood stained mucus (sloughing of mucus due to ischaemia or pressure from constipation) - abdo distension and shock >4yo: less likely to have rectal bleeding, longer hx over 3w+ and other pathology, eg CF, peutz-Jeghers, hsp

Question 287

6mo, intermittent colicky pain, vomiting and dehydration

Answer 287

Intussusception

Question 288

Cauuseso f intussusception

Answer 288

Anything in gut that encourages telescoping - meckels, polyps, Peyers patch hypertrophy, hsp, lymphoma, tumour

Question 289

Inv and management of intussusception

Answer 289

USS = doughnut or target sign Trt - NGT, NBM, fluids, call paeds surgeon Cross match blood Reduce with air enema (rectal sufflation - 75% success) If fails or perforates = reduction at laparoscopy or laparotomy

Question 290

Complications of intussusception - 5 and pathophysiology

Answer 290

Shock Sepsis Haemorrhage Peritonitis and gut necrosis - from stretching and constrictiotn of mesentery - venous obstruction - enorgement and bleeding from mucosa -ischaemia - fluid loss and perforation, peritonitis, gut necrosis

Question 291

features and inv of neonatal intestinal obsturction

Answer 291

Bilious vomiting X-ray and big stomach - duodenal atresia = 2 bubbles, no gas distal to stomach - diagnosed in utero, more common in downs, bile/milky vomit depending on location - midgut malrotation causing volvuus (should be 3x90 anticlockwise)

Question 292

Cause of pyloricstenosis and presentation - 7

Answer 292

Hypertrophy of pyloric muscle causes gastric outlet obstruction 3-8w: - projectile milky vomit after feed, increase in frequency and force overtime - then hungry after. No bile i nvomit - no diarrhoea, often have constipation - visible peristalsis - dehydrated - weight loss if late presentaitaon - hypochlorameic metabolic alkalosis with low Na and K - fhx

Question 293

Inv pyloric stenosis

Answer 293

Test feed - gastric peristalsis mayy be seen from leftto right across abdo Pyloric mass - olive shaped - in RUQ Can see visible peristalsis and distended stomach during feed USS if exam unremarkable VBG/UE = metabolic alkalosis, low na and cl

Question 294

Manage pyloric stenosis

Answer 294

Correct fluid and. Electrolyte disturbances ith IF fluids | Definitive = ramstedt’s pyloromyotomy - divide hypertrophic muscle down to mucosa

Question 295

When does testicular torsion normally happen

Answer 295

Adolescents or perinatal - hormone related? | More if undescended

Question 296

Presentation of testicular torsion - 5

Answer 296

Sudden onset pain in scrotum, groin or lower abdo, often referred to abdo or inuinal region with minimal pain in testis itself Red and swollen Hx of self limiting episodes Cremasteric reflex absent Worse on elevation - differentiates it from epididymitis (better on elevation)

Question 297

Manage testicular torsion

Answer 297

Within 6-12h or risk viability Surgical exploration mandatory, if confirmed: - fix contralateral testis as anatomical predisposition where not anchored properly Doppler USS to look at flow in testicular bv may differentiate torsion from epididymitis (NOT before surgery )

Question 298

Differential for testicular torsion

Answer 298

Epididymitis - better on elevation, can see on USS that not torsion, normally <5yo Idiopathic scrotal oedema - goes away in <5d, give anti inflamamtories Torsion of testicular appendage - over 1-2d, may be felt, small hydrocoele, just before puberty

Question 299

Testicular pain growing over 1-2d, just before puberty - what and treatment

Answer 299

Torsion of testicular appendage - hydatid of morgagni = embryological remnant on upper pole of testis Torsion due to rapid enlargement in response to gonadotropins Tortid hydatid may be felt Treat with surgical exploration and excision of appendage

Question 300

what is volvulus and 2 types

Answer 300

Twisting of structure around itself Sigmoid - most common Caecal

Question 301

Sigmoid volvulus sx and exam, inv and treatment

Answer 301

Constipation, abdo bloating , nausea, (vomiting - late sign) O/E - distended abdo, tympanic to percussion AXR - coffee bean sign Treat - sigmoidoscope decompression and leave flatus tube in situ

Question 302

Caecal volvulus sx and exam, inv and treatment

Answer 302

Colicky abdo pain, vomiting, abdo distension AXR - coffee bean, long axis from right lower quadrant to epigastrium or LUQ Treat - endoscopic decompression or surgical intervention via caecostomy

Question 303

Balanoposthitis sx and treat

Answer 303

Extensive redness, purulent discharge Peak 3yo, recurs in 1/3 Treat - abx (topical or systemic), topical steroids

Question 304

What is balanitis xerotica obliterans

Answer 304

Progressive scarring presenting as pathologically non-retractile (phimosis) with white thickening of foreskin, can extend onto glans, into meatus and ultimately urethra

Question 305

foreskin retractable?

Answer 305

Not normally in infancy, as prepuce is adherent to underlying glans to protect non-keratinised glandular and meatal squamous epithelium from urine - inflammation, ulceration, ammoniacal dermatitis/nappy rash

Question 306

Medical indications for circumciion

Answer 306

BXO Recurrent balanoposthitis causing refractory sx Prophylaxis of recurrent UTI, especially if congenital uropathy - posterior urethral valves or vesicoureteric reflex - or limited renal reserves

Question 307

What is hydrocoele

Answer 307

Patent processus vaginalis, narrow enough to prevent inguinal hernia, but may allow peritoneal fluid to leak into testis

Question 308

Presentation of hydrocoele

Answer 308

Asymptomatic scrotal swelling, often bilateral, may have blue colour Tense or lax Non tender and transilluminate

Question 309

Manage hydrocoole

Answer 309

Mostly resolve spontaneously | Surgery if persist beyond 24m

Question 310

Differentiate hydrocoele from inguinal hernia - 2

Answer 310

Can getabove hydrocoele, cannot get above hernia | Tender and non-reducible = strangulated hernia

Question 311

Signs of hernia obstruction - 3

Answer 311

Bilious vomiting Pain Absolute constipation

Question 312

Malrotation mechanism and presentation n

Answer 312

Mesentery not fixed at duodenojejunal flexuure or ileocaecal region, base is shorter than normal, predisposed to volvulus Present with: obstruction (Ladd bands obstructing duodenum, or volvulus) Compromised blood supply - signs = urgent laparotomy - superior mesenteric arterial blood supply to SI and proximal LI compromised Bilious vomiting = urgent upper GI contrast study Normally first 1-3d of life but can be up to 3w

Question 313

Management of malrotatiotn - 3

Answer 313

Scan with contrast or AXR Untwist volvulus, mobilise duodenum and place in non-rotated position (caecum and appendix in LIF) - mesentery broadened Remove appendix to avoid future confusion with appendicitis

Question 314

What is NEC

Answer 314

Air in abdo wall Tense, swollen, red abdo, unwell baby Manage - conservative - fluid, TPN, recovery in 7-10d Operate if fails, or perforate

Question 315

RF for gastroschiisis - 4

Answer 315

Economic disadvantage Young mother Smoking Cannabis in 1st trimester

Question 316

Manage gastrochisis - immediate andlong term

Answer 316

Decompress with NGT and wrap in cling film Put in silo tube and sqeeze every couple of days to push back into abdo - avoids anaesthetic, can check bowel for atresia etc first Long term - erythromycin prokinetic dose and lansoprazole (for 1y) to increase appetite and prevent reflux

Question 317

Complications of gastroschisis - 6 short term, 2 long term

Answer 317

``` Cardiac defects likely Dehydration, hypothermia, infection Short bowel can cause liver failure Abdo pressure increased during return = pressure on pelvic veins, kidneys and lungs = intraabdo compartment syndrome - check legs still pink and crt - avoid by returning slowly Long term - - malrotation causing vomiting/retching due to reduced gastroduodenal angle -reduced appetite ```

Question 318

What is exomphalos, prognosis and treatment

Answer 318

GI in bag with Wharton’s jelly and amnion LEss good outcome than gastrochisis as often with cardiac problems, trisomy 13 (midline defects) - many die in utero, short life expectancy Minor - close (<5cm) If big - keep clean and wait until big enough and cardioresp stable - >12mo

Question 319

Sx of tracheo-oesophageal fistula

Answer 319

Vomit all feeds | NGT won’t go down - seen as loop on X-ray

Question 320

Risks of trachoe oesophageal fistula

Answer 320

Prone to laryngomalacia and reflux - cough and aspiration - bronchiectasis if not treated Complication of surgery - damage to thoracic duct - chylothorax

Question 321

2 types of necklumps

Answer 321

Thyroglossal cyst - remnant of thyroid duct Branchial cyst or sinus (uncovered, fluid leaks) - cleft from anterior border of scm, can track back to tonsillar fossa

Question 322

cause of undescended testes and where

Answer 322

Arrested along normal pathway of descent | Can ascend to inguinal position during childhood due to shortening of cord structures

Question 323

How common is undescended testes and time line of descent

Answer 323

1% of neonates 30% of premature - 25% bilateral in these - as testicular descent via inguinal canal is in 3rd trimester -15-25w = transabdo phase, gubernaculum thickens and shortens to pull testes down -25-35w = inguinal scrotal phase -2-3m = testosterone surge can cause undescended testes to descend

Question 324

3 types of undescended testes

Answer 324

Retractable - can be manipulated into bottom of scrotum without tension but retract into inguinal region due to pull of cremaster muscle Palpable - palpated in groin but cannot be manipulated into scrotum - can be ectopic, outside normal line of descent (peritoneum, femoral triangle) Impalpable - no testis felt, may be in inguinal canal, intra abdo or absent

Question 325

exam for undescended testes

Answer 325

Massage contents of inguinal canal towards scrotum into a palpable position

Question 326

Inv for undescended testes

Answer 326

USS - not very reliable, only when bilateral impalpable to verify pelvic organs Hormonal - measure serum testosterone in response to IM injection of HCG - confirms presence of testicular tissue in children ith bilateral impalpable testes Laparoscopy - gold standard - under anaesthesia. - first do inguinal exam to ensure testis not in inguinal canal, ensure in abdo and viable

Question 327

Manage undescended testes

Answer 327

Watch and wait until 6m Orchiopexy - surgical placement of testis I scrotum - for: - fertility - optimise spermatogenesis as lower temp in testis - reduced risks of torsion and trauma

Question 328

Fertility in undescended tests after orchiopexy

Answer 328

Bilateral impalpable = usually sterile Bilateral palpable = 50% reduced Unilateral - fertility normal

Question 329

Complications of undescended testes and manage

Answer 329

Malignancy - higher dysplastic abnormalities and higher risk of malignancy - bilateral and intra abdo have greatest risk. Cannot self exam unless operated on Cosmetic and psychological - prosthesis can be used if absent, but wait until can have adult sized

Question 330

What is vesicoureteric reflex

Answer 330

Abnormal backflow of urine from bladder into ureters and kidney - uterers displaced laterally, meaning they’re more perpendicular and shorter intramural course, leading to vesicoureteric junction being less effective Predisposes to UTI

Question 331

Sx of vesicoureteric reflex

Answer 331

Mild - none | Severe - infection, pyelonephritis, renal scarring - reduced renal blood flow - htn

Question 332

Inv vesicoureteric reflex

Answer 332

Abdo USS Voiding cystourethrogram with contrast Radionuclide cystogram for renal scarring

Question 333

Treating vesicoureteric reflex

Answer 333

Grade 1-2 - tell parents how to recognise UTI, always send for MCS Grade 3-5 - prophylactic abx low dose daily Normally improves or disappear over time as ureters get longer and junction valve improves as grow - most by 1yo, almost all by 5yo Surgery to remove blockage or repair valve if no improvement/significant sx

Question 334

Features of UTI incl inv

Answer 334

Fever and vomiting Dipstick - polymorphs (>10^8). Mixed = contaminants USS for hydronephrosis Micturition cystourethrogram for vesiculoureteric reflex - no need if >1yo, not recurrent, don’t suspect pyelonephritis Technetium renography if recurrent inf, <1yo, fhx of abn - for scarring

Question 335

Treat UTI

Answer 335

<3mo - IV amoxicillin and gentamycin >3mo - PO trimethoprim (or nitrofurantoin, or coamox) 3d Consider trimethoprim as prophylaxis if. - recurrent, significant GU abn, renal damage

Question 336

Advice for parents if UTI - 3

Answer 336

Avoid constipation Ensure fluid intake and micturition Avoid nylon pants and bubble baths Cleanliness - wipe front to back

Question 337

Presentation, inv and manage posterior urethritis

Answer 337

Present with pain post-micturition +-blood Inv - scope under GA = visible inflammation Manage with IV steroids

Question 338

Features of nephrotic syndrome

Answer 338

Triad: 1) proteinuria (urine PCR high) 2) oedema - face, abdo, pedal 3) hypoalbuminaemia

Question 339

Causes of nephrotic syndrome and biggest RF

Answer 339

Mostly idiopathic minimal change - assoc with allergy and IgE - steroid dependent, steroid sensitive or steroid resistant Focal segmental glomerulosclerosis likely of steroid resistant 9x more common in SE Asia

Question 340

Complications of nephrotic syndrome

Answer 340

``` Infection - pneumococcal peritonitis Reduced clotting High cholesterol Increased fat uptake as more lipoprotein production due to increased liver stimulated to make more protein Relapse - 85% Renal failure if repeated ```

Question 341

Inv nephrotic syndrome

Answer 341

Dipstic for protein - 20% have microscopic haematuria, frothy, albuminous and casts UE normally normal FBC - cholesterol may be raised from increased liver stimulation, Hb raised from dehydration, albumin low so Ca low Biopsy if older, with haematuria, htn, raised urea, unselective protein loss or treatment failure

Question 342

Manage nephrotic syndrome

Answer 342

Daily steroids PO for 4w then every other day - no response = IV for 3d - 90% steroid sensitive, if not = focal segmental glomerulosclerosis Albumin to temporarily fill blood space and fluid if shock/instability/severe sx Extra pneumococcal vaccine if >2yo Prophylactic penicillin when acutely nephrotic Chicken pox prevention if not immunised (high risk of dissemination) - varicella IgG if not exposed, acyclovir IV if exposed Monitor urine daily for 2y - neg or trace for 3 consecutive samples = remission - 3+ or more for 3+ days = relapse - high dose until remission Immunosuppressants eg cyclophosphamide if >2 relapses in 6m or relapsing with steroid toxicity

Question 343

sx of post infective glomerulonephritis

Answer 343

Gross haematuria Oliguria causes oedema Hypertension from fluid retention Malaise, anorexia, fever, abdo pain

Question 344

Inv post infective glomerulonephritis

Answer 344

Dipstick for proteinuria - can be transient - and haematuria Creatinine and urea high Hb low due to dilute Complete C3 low as deposited (C4 normal) - if haven’t recovered in 8-12w, test for SLE, as both are raised in lupus glomerulonephritis ASO titre Microscopy - neutrophils

Question 345

Treat post inf glomerulonephritis

Answer 345

``` Diuretics Calcium ch blocker amlodipine Fluid and salt restriction, protein restriction when oliguric Oral penicillin Nitroprusside if encephalopathy ``` Oedema resolves in 5-10d, htn, haematuria and proteinuria take several weeks

Question 346

Common cause of post inf glomerulonephritis

Answer 346

Strep - pharyngitis, impetigo | 7-21d after

Question 347

Steroid SEs in children

Answer 347

``` Obesity, diabetes Poor growth Raised BP Osteoporosis Avascular necrosis of hip Adrenal suppression ```

Question 348

Causes of nephritis in children

Answer 348

``` B haemolytic strep from URTI, viruses, bacteria HSP Toxins Berger’s disease Malignancy Renal vein thrombus ```

Question 349

Acute glomerulonephritis features

Answer 349

``` 7yo Haematuria and oliguria (increased bp and uraemia) from immune cause Periorbital oedema Fever GI disturbance Loin pain ```

Question 350

Complicated presentations of glomerulonephritis - 3

Answer 350

Hypertensive encephalopathy - restless, drowsy, fits, severe headache, vomiting, reduced vision, coma Uraemia - acidosis, twitchy, stupor, coma Cardiac - gallop rhythm, cardiac failure/enlargement, pulmonary oedema

Question 351

Blood tests for glomerulonephritis

Answer 351

``` FBC, UE C3 low, C4 normal ASOT, Anti-dsDNA (SLE), ANA, ANCA (vasculitis) Blood cultures and virology Urine culture and specific gravity ```

Question 352

bladder capacity , ,post void capacity and rate of urine production

Answer 352

Age +1 x30 Empty to 10% capacity Make 1mg/kg/h or urine

Question 353

impact of dysfunctional bladder

Answer 353

social isolate, depression, anxiety | Incomplete emptying causing UTI - sphincter and pelvic floor muscles contract incorrectly - often also constipated

Question 354

Causes of dysfunctional bladder and management

Answer 354

Neuro - trauma, infection (transverse myelitis), spina bifida, sacral agenesis, tumour in spine or bladder Potty training Primary nocturnal eneuresis due to routine change Detrusor overactivity - on urodynamics assessment, treat with anticholinergics <8yo - eneuresis alarm, >8yo or needed immediately = terlipressin

Question 355

What is nocturnal eneuresis and 3 contributing factors

Answer 355

Intermittent involuntary voiding during sleep in absence of physical disease in child >5yo 1/month minimum for 3 months for diagnosis Primary = havne’t yet had prolonged period of being dry Second = has had prolonged dry period before 1. Defective sleep arousal 2. Nocturnal polyuria 3. Bladder factors - lack of inhibition of bladder emptying, reduced capacity, overactivity

Question 356

Manage nocturnal eneuresis

Answer 356

``` Motivational therapy No fluids 2h before bed Eneuresis alarm Desmopressin - reduces urine production during sleep Treat underlying condition ```

Question 357

Presentation of diabetes type 1

Answer 357

``` 4Ts Thirst Toilet Tired Thin Peak age of onset 5-7yo and around puberty ```

Question 358

Differentiate DM T1 and T2 on blood?

Answer 358

C peptide low in DM1, high until late in DM2

Question 359

Manage T1DM

Answer 359

``` Admit immediately if suspect Ketones = emergency Insulin total daily requirement: 0.5-1u/kg/24h for pre puberty 1.5u/kg/24h pubertal Daily dose - 1/3 rapid, 2/3 long - 2/3 before breakfast, 1/3 before dinner As basal-bolus or continuous (change /3d) or arm monitor (change /14d) ```

Question 360

Signs of too much insulin

Answer 360

Sweating, hunger, weakness, faint | Abdo pain, vomit, fit, coma

Question 361

Diet requirements in DM1

Answer 361

Kcal requirement = 1000 + 100-200 per year of age | 20% protein, 50% unrefined carbs, <30% fat

Question 362

follow up for DM1

Answer 362

Motivational education Check growth and fundi Carb counting and insulin dose adjustment - DAFNE Exercise Puberty - growth spurt, hormones can cause some insulin in resistance Nephropathy - urine dipstick for ACR - early morning sample Retinopathy - for detachment

Question 363

Manage hypoglycaemia

Answer 363

Mild/mod - oral glucose or gel | Severe - 5ml/kg of 10% IVI glucose or 0.5-1mg IV/IM glucagon

Question 364

Causes of hypothyroid

Answer 364

Congenital - dyshormonogenesis, iodine deficiency, maldescent of thyroid, athyrosis Acquired - Hashimoto’s, hypopituitary, trisomy 21, prematurity

Question 365

Signs of hypothyroid

Answer 365

Asymptomatic - pos Guthrie test Prolonged jaundice, widened posterior fontanelle Poor feeding, sleepy, constipation, dry skin Hypotonia, slow relaxing reflexes Flat nasal bridge, protruding tongue Low IQ, delayed puberty, short - if not treated

Question 366

tests of hypothyroid

Answer 366

Low T4, high TSH Low iodine uptake Low Hb Low bone age

Question 367

Manage hypothyroid

Answer 367

Levothyroxine

Question 368

Puberty trigger and hormones

Answer 368

By production of GnRH from hypothalamus, causing FSH and LH production

Question 369

Types of precocious puberty

Answer 369

True = premature activation of HPA - gonadotrophic dependent - small testes = CAH False = from excess sex steroids - gonadotropin independent

Question 370

Precocious puberty in girls - cause and inv and manage

Answer 370

<8yo Normally idiopathic and familial USS can establish cause can give GnRH analogues to halt early ‘normal’ puberty

Question 371

Precocious puberty in boys - cause and inv

Answer 371

<9yo Usually organic cause Exam testes: - unilateral enlargement = gonadal tumour - bilateral enlargement = intracranial lesion - MRI brain - small testes = adrenal cause

Question 372

Manage precocious puberty

Answer 372

GnRH analogues for girls to halt normal puberty Manage underlying pathology Manage behavioural issues associated with early puberty

Question 373

Premature thelarche?

Answer 373

Breast development, age 6m-2y Can be asymmetrical No pubic hair or growth spurt Non progressive and self limitng

Question 374

Premature adrenarche?

Answer 374

Pubic hair <8yo in girls and 9 in boys No other signs of sexual development Normally fromearly maturation of androgen production by adrenal gland, can be adrenal hyperplasia or adrenal tumour - differentiate with urinary steroid profile Usually self limiting USS ovaries and uterus and bone age to exclude precocious puberty

Question 375

Gonadotropin dependent causes of precocious puberty and test

Answer 375

Idiopathic/familial CNS abnormalities - congenital (hydrocephalus), acquired (infection, irradiation, surgery), tumour (microscopic hamartoma) Hypothyroid LH ++ FSH +

Question 376

Gonadotrophin independent causes of precocious puberty and test

Answer 376

Adrenal disorder - tumour, CAH Ovarian tumour - granulosa cell Testicular tumour - leydig cell Exogenous sex steriods FSH and LH low

Question 377

What is delayed puberty and cause - 3

Answer 377

By 14yo in girls or 15 in boys 1. Constitutional - just delayed 2. Hypogonadotrophic hypogonadism - hypothalamus or pituitary problem - systemic disease eg CF, crohn’s, anorexia - hypothalamo-pit disorder eg tumour, Kallmans syndrome 3. Hypergonadotrophic hypogonadism - ovaries/testes aren’t responding - kleinefelter’s, Turner’s

Question 378

Treat delayed puberty

Answer 378

Usually don’t need to | Testosterone (M) or oestradiol (F)

Question 379

Sx of growth hormone deficiency

Answer 379

Small height High cholesterol Decreased muscle mass, poor bone density Newborns = hypoglycaemia, small penis

Question 380

Causes of GH deficiency - 5 and treat

Answer 380

``` Genetics Trauma Infection Tumour Radiation ``` GH replacement

Question 381

Main 2 features of cerebral palsy and basic treatment

Answer 381

Dystonia - basal ganglia/thalamus pathology Spasticity - cerebellar pathology Rehab - physio, OT, orthopaedics, orthotics, muscle relaxants (baclofen targets GABA receptors), botulinum toxin (prevents nt Ach from axon endings at neuromuscular junction = flaccid paralysis)

Question 382

What is cerebral palsy

Answer 382

Non progressive disturbance to foetal or infant brain <2yo - single incident in time Abnormality of movement and posture, liming activity, can include learning disability and epilepsy

Question 383

Causes of cerebral palsy and premature cause

Answer 383

Prenatal = 80% - vascular occlusion, cortical migration disorder of neutrons, structural maldevelopment During delivery - hypoxic ischaemic injury Postnatal - hypoglycaemia, meningitis, encephalitis, head trauma Premature - periventricular leukomalacia = white matter necrosis near lateral ventricles

Question 384

Clinical presentaitaon of cerebral palsy

Answer 384

``` Early - abn tone - abn limb/trunk posture - abn walking - delayed motor milestones - asym hand function before 12mo - feeding difficulties Primitive reflexes persist ```

Question 385

Assess cerebral palsy functoin

Answer 385

Gross motor function classification system: Level 1 - walks independently 2 - walks with some limitaiotan 3 - walks with handheld device 4 - selfmobility with limitations - uses motor device 5 - transported in manual wheelchair

Question 386

Diagnosis of cerebral palsy

Answer 386

Clinical exam MRI may show damage Later = worse outcome due to deterioration in physical function (not neuro)

Question 387

3 subtypes of cerebral palsy

Answer 387

90% - spastic - UMN, uni or bilateral, hemi/quad/diplegia (legs >arms) Dykinetic - basal ganglia - kernicterus or HIE = chorea (irregular sudden brief), athetosis (slow writhing), dystonia (opposing muscle contraction) Ataxic - hypotonia in trunk and limb, balance then uncoordinated, ataxia, tremor

Question 388

Function of drugs in cerebral palsy

Answer 388

Botuinum toxin - prevents ACh release at nmj = flaccid paralysis Baclofen = intrathecal,oral or epidural = gaba agaonist to reduce spasticity

Question 389

epilepsy in children and why cause amnesia

Answer 389

Tendency to intermittent abnormal electrical activity in brain not related to fever - seizure = abn or excessive neuronal discharge Commonly in hippocampus - oedematous from prolonged generalised tonic clonic seizure - sclerosis - amnesia

Question 390

Features of different areas of focal seizures

Answer 390

Frontal - clonic movements Temporal - lip smacking, deja vu, aura, smell change Occipital - visual distortion Parietal - contralateral altered sensation

Question 391

Features of absence seizure

Answer 391

10s, can be in middle of sentence, eyes role up, can have some flickering of eyes Unaware Induced by hypoxia causing vasospasm - test by blowing out

Question 392

Infantile spasms? And inv

Answer 392

West syndrome Peak age 5yo Head bobbing and arm jerks every 3-30s Reduced IQ Epileptic encephalopathy with progressive psychomotor dysfunction - multiform and irretractable seizures, cognitive regression and behavioural deterioration EEG = hypsarrhthmia - high amplitude and irregular waves with spikes in background of chaotic and disorganised activity

Question 393

Treat infantile spasm

Answer 393

Prednisolone | 2nd - vigabatrin

Question 394

Myoclonic seizure andtrt

Answer 394

1-4yo ‘Thrown to ground’ and brief repetitive jerking Valproate

Question 395

Differentials for epilepsy - 5

Answer 395

``` Migraine Arrhythmia Night terror Faint Tic ```

Question 396

Causes of seeizures

Answer 396

Epilepsy - 70% idiopathic, presumed genetic - secondary = cerebral malformation or vascular occlusion or damage (inf, IVH) - cerebral tumour - neurodegenerative or neurocutaneous syndrome Non-epilepsy - febrile - metabolic - hypoglycaemia , hypocalc, hypomagnesium, hypo/ernatraemia - head trauma - meningitis, encephalitis - poisons, toxins

Question 397

Diagnose epielepsy

Answer 397

History and eyewitness EEG - may be normal, asym or slowing, try sleep deprivation or 24h tape/video-telemetry MRI or CT - structural - if neuro signs between seizures, or focal seizures - tumour, vascular lesions, sclerosis Functional - PET or SPECT for hypometabolism areas Genetic study Metabolic investigatiotns

Question 398

Manage epilepsy

Answer 398

Not after 1 seizur Only when causing significant inconvenience to life Aim for monotherapy low dose Give rescue therapy if prolonged - rectal diazepam or buccal midazolam Discontinue when 2y seizure free 1st line for all generalised = valproate, 2nd = lamotrigine. Can use carbamazepine for tonic clonic Focal - valproate or carbamazepine, lamotrigine but slow titration, 2nd - topiramate, levetiricitam

Question 399

Other treatment options for epilepssy

Answer 399

Ketogenic diet Vagal nerve stimulation Surgery if well localised focus points, temporal lobectomy for temporal sclerosis

Question 400

ADR of valproate, carbamazepine, lamotrigine, vigabitrin

Answer 400

Valproate - weight gain, hair loss, rare = liver failure Carbamazepine - neutropoenia, hyponatraemia, rash ataxia, liver enz induction Lamotrigine - rash Vigabitrin - sedation, restriction of visual fields

Question 401

febrile convulsions - epilepsy risk, what to do

Answer 401

1-2% risk - higher if neuro disorder, complex seizure or family history Look for source of fever Lorazepam IV, buccal midazolam or rectal diazepam after 5m for parents to give as rescue >10m = status epilepticus treatmetn

Question 402

Features of non-epileptic non-febrile seizures in kids

Answer 402

Breath holding - toddlers who are upset, crying, go blue and LOC with quick recover. No treatment needed, resolve spontaneous Reflex anoxic - from pain, fever, fear causing vagal inhibition = cardiac asystole. Pallor, falls to floor, can cause tonic clonic from hypoxia - rapid recovery Neurofibromatosis - cafe au lait spots - bones = pseudoarthrosis, bone cysts, scoliosis, sphenoid hypoplasia - eyes = lisch nodules, optic nerve glioma = blind Tubular sclerosis - cortical tubers with disproportionate growth = block CSF flow causing hydrocephalus and ventriculomegaly = epilepsy, retina hamartomas. Can remove but other tubers will grow Chiari syndrome - low lying cerebellar tonsils, often self correct with time. Crisis = sudden block of tonsils in foramen magnum = cardiorespiratory arrest and death Agenesis of corpus callosum = infantile spasms, developmental delay, microcephaly, retinal lesions, onset 3-5m, females only Dandy walker syndrome - aplasia/hypoplasia of cerebellar vermis = balance difficulties

Question 403

What else to ask about if present with allergy

Answer 403

``` Eczema Allergic rhinitis Conjunctivitis Asthma Urticaria Insect sting hypersensitivity Anaphylaxis ```

Question 404

What is hypersensitivity

Answer 404

Objectivity reproducible sx following exposure to a defined stimulus at a dose tolerated by normal people

Question 405

What is allergy

Answer 405

Hypersensitivity reaction initiated by specific immunological mechanisms - IgE or non IgE eg coeliac

Question 406

What is atopy

Answer 406

Personal or family tendency to produce IgE antibodies in response to ordinary exposure to potential allergens, usually proteins. Assoc with rhinitis, conjunctivitis, eczema, food allergies

Question 407

Mechanisms of allergic disease and timings

Answer 407

Early <20m = IgE; late = intolerance, not IgE Dendrite detects allergen, presents to T cells which release Th2 (IgE) and Il-5 which stimulate eosinophils IgE antibodies bind to allergen, then receptor on mast cells or basophils. Cross link = release of inflammatory cytokines and chemicals eg histamine

Question 408

Phases and features of allergy

Answer 408

Early - within minutes, from histamine release = urticaria, angioedema, sneezing ,bronchospasm Late - 4-6h = nasal congestion of upper airways, cough, bronchospasm Allergic rhinitis - sneeze, itchy runny blocked nose Conjunctivitis - itchy watery red eyes Hives - red raised itchy rash Wheeze, tight chest, SOB, cough Angioedema - swollen lips, tongue and eyes Tummy pain, sick, vomiting, diarrhoea

Question 409

Timing of different allergies

Answer 409

= allergic march Infancy = food and eczema Childhood = asthma, rhinitis, conjunctivitis

Question 410

Prevention of allergy

Answer 410

Breast feeding exclusively for 3-4m reduces cows milk allergy and eczema Probiotics Exposure to microbes - hygiene hypothesis

Question 411

Cause of secondary food allergy

Answer 411

Cross reactivity between protein

Question 412

diagnosis of food allergey - 3

Answer 412

Skin prick test >3mm weal with salt watery control Neg - very unlikely but Doesn’t rule out allergy if hx fits Measure IgE in serum - RAST test Ingest food under supervisor - test against placebo Non IgE - harder to diagnose as less characteristic - depends on hx - sx = diarrhoea, vomitin, colic, blood in stool (allergic proctitis), GORD, food refusal. Cut foood out and change milk then slowly reintroduce

Question 413

Severities f allergic rheinitsi and conjunctivitis

Answer 413

Intermittent - <4d a week or <4w at a time Persistent - >=4d a week and >=4w at a time Mild - normal sleep, daily activities and work/school Mod - severe = disrupted sleep and daily activities, school/work, 1 or more troublesome sx

Question 414

Manage sx of allergic rhintisi

Answer 414

Cough at night from postnasal drip - nasal spray (lateral and back, head down, 2 sprays in each nostril), sublingual - desensitised to decrease sx

Question 415

Manage urticaria and angioeema

Answer 415

Urticaria - antihistamines, steroids if severe | Angioedema - maintain airway, antihistamines, steroids and adrenaline

Question 416

Haematopoeisis sites and types of cell

Answer 416

Yolk sac <8w - liver - spleen - bone marrow from birth | Megaloblasts then macrocytes before birth, then normocytes after birth

Question 417

Types of globin chains and haemoglobin

Answer 417

A and y before birth Then a and B (increases after birth). By 18w = mostly a and b and hardly any y 4-8w gestation = Hb gower 1, Hb gower 2 and hb portland After 8w gestation = HbF (2a2y)

Question 418

What is HbF

Answer 418

2a2y Higher affinity for o2 - advantage in hypoxic envt of foetus Shorter life span - higher rate of haematopoeisis, predispose to neonatal jaundice

Question 419

Hb level at birth and 2m after

Answer 419

14-21.5g/dl, decrease to 10g/dl at 2m

Question 420

Causes of each type of anaemia and preterm

Answer 420

Iron, folic acid and vit B12 stores depleted in preterm babies so def in 2-4m Microcytic - iron def, thalassaemia Normocytic - chronic disease, acute blood loss, haemolytic anaemia, chronic renal failure Macrocytic - B12/folate def, alcohol, liver diseae, hypothyroid, myeloma/myelodysplasia

Question 421

Iron deficiency in kids - cause

Answer 421

Inadequate intake, malabsorapotin or blood loss From breast milk (low content but 50% absorbed) or cows milk (low content and only 10% absorbed), little absorbed in solids. Must mix feeding from 6mo Absorption increased with vit c, decreased with tannin in tea If little improvement with supplements and dietary advice,inv for malabsorpoaitn (coeiac) or chronic blood loss (meckel’’s)

Question 422

Tests for iron deficiency

Answer 422

Low MCV Low MCH Normal retic count Low serum ferritin

Question 423

Clinical features of iron def anaemia

Answer 423

Asymptomatic until <6-7g/dl tired, pallor Feed slowly, pica

Question 424

What is red cell aplasia, 3 causes and 5 tests

Answer 424

``` Depletion of erythrocyte precursor cells 3 main types - congenital, transient erythroblastopoenia or childhood or parvovirus B19 Tests; Low retic count despite low Hb Neg Coombs Normal bilirubin Absent red cell precursors on bone marrow aspirate Do parvovirus serology ```

Question 425

Causes of haemolytic anaemia - 4

Answer 425

Increased redcell destruction - intravascular or exxtravascula (liver, spleen) RBC life span reduces to a few days and BM production increases x8 RBC membrane defect - Hereditary spherocytosis, Enzyme disorder - G6PD Haemoglobinopathies - thalassaemia, sickle cell Immune - AI haemolytic anaemia, haemolytic disease of newborn

Question 426

Exam for haemolytic anaemia

Answer 426

Anaemia | Hepatosplenomealy

Question 427

Inv for haemolytic anaemia - 5

Answer 427

``` Increased unconjugated bilirubin Excess urinary urobilinogen Raised retic - polychromasia on blood film Abn blood film RBC shape Increased RBC precursors in BM ```

Question 428

CAuses of anaemia in children

Answer 428

Increased destruction - haemolysis Decreased production - RBC aplasia, ineffective erythropoeisis Blood loss - GI or bleeding disorder

Question 429

Bilirubin normal, retic normal or high in anaemia - causes

Answer 429

Blood loss or ineffective erythropoesis eg iron deficiency

Question 430

Cause f anaemia with low retic

Answer 430

Red cell aplasia - parvo, leukaemia

Question 431

What is hereditary spherocytosis and features | Diag and manage

Answer 431

Aut dom Spectrin, ankyrin abn proteins in RBC membrane Less deformable, block microvascularture in spleen Jaundice, anaemia, splenomegaly, gallstones Diag with blood film Treat - oral folic acid as higher requirements, splenectomy >7yo if poor growth

Question 432

requirements after splenectomy

Answer 432

HiB, men c and strep p vaccination | Lifelong penicillin prophylaxis

Question 433

complicatiotn of spherocytosis and treat

Answer 433

Aplastic crisis - BM failure - no RBC From additional parvo = 2 anaemia-inducing mechanisms Treat with blood transfusion

Question 434

What is G6PD and highest prevalenc

Answer 434

Africa, Mediterranean, Middle East G6P = rate limitng enzyme in pentose phosphate pathway, essential for prevention of oxidative damage to RBC Def = oxidant induced haemolysis X linked inheritance

Question 435

clinical manifestiaon of G6PD

Answer 435

Neonatal jaundice in first 3 days | Acute haemolysis precipitated by: inf, fava beans, drugs (antimalaraisl, antibiotics)

Question 436

Diag G6PD and manage

Answer 436

G6PD activity in Rbc May be misleadingly high during crisis As higher concentration of enzymes in reticulocytes Educate parents about signs - jaundice, pallor, dark urine, and give list of drugs to avoid

Question 437

What is sickle cell diseae and exacerbating factors

Answer 437

Mutation in beta-globin gene (HbS) - glutamate to valine - sticky hydrophobic pocket = sickle shaped RBC Decreased life span, get trapped in microvascular causing ischaemia EXacerbated by low oxygen, dehydration ad cold

Question 438

Features of sickles cell and prognosis

Answer 438

Anaemia Jaundice Infection from encapsulated organisms (pneumococci, h influenzae) - due to hypersplenism secondary to chronic sickling and microinfarction in spleen 50% die before 40yo

Question 439

Manage sickle cell

Answer 439

Prophylaxis: immunisations of pneumococcal, HiB and meningitis; oral penicillin, daily folic acid (higher RBC turnoveR) Avoid cold, dehydration, excess exercise, hypoxia - minimise vaso-occlusive events Hydroxyurea - increases HbF production, protective against sickle crisis BM transplant - 90% cure rate, only if HLA-identical sibling

Question 440

types of thalassaemia and prevalence where

Answer 440

B thalassaemia - reduced B globin, reduced Hb production, commonest in Indians - major (no HbA, most severe) or intermedia (milder, small amount of HbA) b-thalassaemia trait - usually asymptomatic, mild anaemia, confusion a-thalassammis - major (hydrops), HbH (3a-globin genes affected), trait (1 or 2 a globin genesaffected - anaemia or asymptomatic)

Question 441

Sx of b thalassameia

Answer 441

severe anaemia from 3-6mo, transfusion dependent Failure to thrive Extramedullary haematopoeisis - bone marrow expansion = facial features (bossing of skull and maxilla), hepatosplenomegaly - only if no transfusions

Question 442

Manage and diagnose b thalassamia

Answer 442

Monthly lifelong blood transfusions to maintain Hb >10 BM transplant - HLA identical siblings - only cure DNA analysis of Chorionic Villus Sampling if parents have traits trait - hypochromia, microcytic anaeima, increased RBC count, raised HbA2

Question 443

A thalassamia major -present and treat

Answer 443

Deletion of all 4 a gobingenes Oedema and ascites in uutero from foetal anaemia Requires intrauterine transfusions then monthly transfusions once born

Question 444

What is ITP

Answer 444

commonest cause of thrombocytopoenia in childhood Antiplatelet IgG autoantibodies destroy circulating platelets - may be increase in megakaryocytes in bone marrow as compensation

Question 445

Features of ITP

Answer 445

PResent at 2-10yo 1-2w after viral infection Petechia - small 1-2mm nonblanching red spots Purpura - larger nonblanching red spots/blotched Superficial bruising

Question 446

Diag ITP and 2 differentials

Answer 446

Diagnosis of exclusion | Differentials: ALL or SLE

Question 447

MAnage ITP

Answer 447

80% = Acute, benign and self limiting - remits in 6-8w Treat if major or persistent minor bleeding - with oral prednisolone or anti-D IV or IV Ig Platelet transfusion if life threatening haemorrhage

Question 448

Chronic ITP?

Answer 448

Platelets still low after 6m

Question 449

Complications of blood transfusion (5) and manage

Answer 449

``` Iron overload: Heart failure Liver cirrhosis DM Infertility Growth failure ``` Treat with iron chelation

Question 450

Inheritance and types of haemophilia

Answer 450

X linked recessive, but 30% sporadic Haemophilia A - factor 8 deficiency = commonest Haemophilia B - factor 9 def

Question 451

Sx of haemophilia and manage

Answer 451

(If <1% factor 8) - recurrent spontaneous bleedin into joints and muscles - can cause arthritis Prolonged bleeding Mostly present at 1yo recombinant factor 8/9 given IV when bleeding Avoid IM inj, aspirin and NSAIDs Prophylaxis if severe haemophilia to reduce chronic joint damage Desmopressin stimulates production of factor 8 and vWF

Question 452

Complications of haemophilia - 3

Answer 452

Transfusion transmitted inf- hep b/c, HIV Vascular access - may be hard, central lines = thrombosis or infected Antibodies to factor 8/9 develop in <20% - will need very high doses to treat bleeding

Question 453

What is Von willebrand factor and disease

Answer 453

Facilitates platelet adhesion Carries factor 8 Disease = quantitative or qualitative deficiency of vWF = can’t form platelet plug

Question 454

Inheritance of Von willebrand diseae

Answer 454

Aut dom

Question 455

Features of vonwillebrand and difference from haemophilia

Answer 455

Bruising, excessive prolonged bleeding, mucosal bleeding | Uncommon to get spotaneous soft tissue bleeds which you get in haemophilia

Question 456

manage Von willebrand disease and ADR

Answer 456

Depends on type and severity Mild - desmopressin - increases factor 8 and vWF in plasma - cautious <1yo as can lead to hyponatraemia and seizures Severe - plasma derived vWF as desmopressin is ineffective Avoid IM, aspirin and NSAIDs

Question 457

Organ infiltration from leukaemia

Answer 457

BM features and bone pai Reticuloendoteial - hepsplen and lymph Testicular CNS - headache, vomitin, nerve palsies

Question 458

image for leukaemia

Answer 458

T cell disease = mediastinal mass on cxr

Question 459

Most common leukaemia in kids and prognostic factors

Answer 459

ALL = 80% | Poor prognosis if <1 or >10yo, high WBC, slow response to chemo

Question 460

Manage ALL

Answer 460

Correct anaemia with transfusion Protect kidneys with allopurinol and fluids Combo chemo - eradicates leukaemic blasts and restores normal marrow function - for 2-3y - 95% success May need intrathecal chemo as cytotoxic drugs penetrate brain poorly Co-trimoxazole to prevent pneumocystis carinii pneumonia

Question 461

Brain tumour types

Answer 461

Most common solid tumour in children, leading cause of cancer death Almost always primary Astrocytoma (cortex and spinal cord), medulloblastoma (cerebella)

Question 462

Sx of brain tumour

Answer 462

From RICP - headache, vomiting, behavioural/personality change Visual disturbance Exam: papilloedema, tense fontanelle, increased head circumference Developmental delay

Question 463

Inv brain tumour

Answer 463

MRI for biological activity | LP - With neurosurg advice only

Question 464

Manage brain tumour and aim

Answer 464

Surgery - first line - to treat hydrocephalus, biopsy (not if brainstem tumour) and resection CRT - depending on tumour type and age of patient

Question 465

Diagnose juvenile idiopathic arthritis - 5

Answer 465

1. <16yo 2. >6w 3. 1+ joint 4. Rule out other causes eg septic, Perthes, post-trauma arthritis 5. Clinical or X-ray sx

Question 466

Variation in JIA

Answer 466

Oligo, polyarticular or systemic | Flares of a few weeks or more

Question 467

Inv JIA

Answer 467

RhF, ANA Anti-CCP - pos = more intensive treatment X-ray High risk for uveitis - screen regularly until 10yo Examine for other AI signs - nails, hand pain, joint movement pain, scoliosis, ulcers, uveitis

Question 468

Manage uveitis in JIA and complications

Answer 468

Methotrexate and steroid eye drops Causes RICP, glaucoma, cataracts Long term = sclerosis, fixed/different sized pupils

Question 469

Treat JIA

Answer 469

NSAIDs Steroid injections Physio Methotrexate - weekly, long term, start early to reduce joint damage Systemic corticosteroids - pulsed IV as induction if severe Biologics if severe/refractory to methotrexate

Question 470

Long term progression of JIA and complications

Answer 470

Synovial thickening from inflammation takes a long time to decrease but fluid will clear quickly Chronic inflam - vascularise - increased growth - leg length discrepancy 1/3 continues into adulthood Untreated = joint destruction, flexion contractures Growth failure from anorexia, chronic disease, systemic corticosteroids

Question 471

Genetic testing mechanisms - 3

Answer 471

Maternal blood sample Pre implantion genetic testing Amniocentesis

Question 472

Risk of genetic defect

Answer 472

Consanguinity increases risk from 2-3% to 5% of major birth defect 1/1400 eggs have de novo mutation 1/400 sperm have mutation , 75% due to paternal age

Question 473

Types of mutation and inheritance

Answer 473

``` Mendelian - aut or sex linked Non-Mendelian - imprinting (problem from not inheriting what’s necessary) = maternal 15 = angelman’s, paternal 15 = prader-willi - mitochondrial - maternal - multifacorial ``` Inheritance - structural = dominant, metabolic = recessive

Question 474

Counselling features for genetics

Answer 474

1. Diagnose 2. Estimate risk 3. Communicate to family

Question 475

Clinical features of downs

Answer 475

Round face, flat nasal bridge, upslanted palpebral fissures Small mouth and protruding tongue, small ears Flat occiput single palmar crease, inturned 5th digit, wide sandal gap Hypotonia, duodenal atresia, Hirschprung’s CHD - AVSD, ASD, VSD Hypothyroid, impaired vision and hearing

Question 476

Long term problems fro down’s

Answer 476

Delayed development Learning difficulties Small stature Increased infection risk and leukaemia Epilepsy Atlanto-occipital instability

Question 477

Inv down’s syndrome

Answer 477

FISH technique - tell parents

Question 478

Edward’s syndrome

Answer 478

Trisomy 18, USS 2nd trimester then amniocentesis and chromosome analysis Low birthweight Prominent occiput, small mouth and chin, short sternum Flexed overlapping fingers, rockerbottom feet Cardiac and renal malformation

Question 479

Patau’s syndrome

Answer 479

Trisomy 13, midline defects 2nd trim USS then amnio and chromosome analysis Structural brain defect, scalp defect, micropthalmia and other eye defects Cleft lip and palate Polydactyl Cardiac and renal malformation

Question 480

Prader willi, sx and manage

Answer 480

Random deletion of paternal chromosome 15, disrupting normal hypothalamus function Hypotonia at birth Distinctive facial features Reduced growth, lack of sexual development Learning difficulties and developmental delay, behavioural problems Excessive appetite leading to dangerous weight gain Manage: feed (initially poor), weight and diet, exercise, undescended testes, hormones, structure and routine

Question 481

Angelman syndrome, sx, diag and manage

Answer 481

Deletion or malfunction of maternal chr15, affecting nervous system = physical and intellectual disabity severe Delayed development at 6-12m, mobility problems Distinctive behaviours and features Feeding difficulties and reflux Diag - FISH, chromosome analysis, dNA methylation, gene mutation analysis ``` Manage: Communication and behavioural therapy Physio and back brace/spinal surgery for scoliosis Ankle/foot orthoses Anti-epileptics ```

Question 482

Turners syndrome features and when dianosed

Answer 482

45XO Antenatally - foetal oedema of neck, feet and hands Short, webbed neck, wide spaced nipples Lymphoedema of hands and feet in neonates Spoon shaped nails CHD - coarctation, aortic root dilatation, bicuspid aortic valve Puberty delayed, infertile as ovarian dysgenesis Normal intellect

Question 483

Manage turners

Answer 483

Growth hormone therapy | Oestrogen replacement

Question 484

Kleieefelter’s features

Answer 484

47XXY Infertility (first presentaiton) and hypogonadism and small testes Gynaecomastia, female-like pubic hair distribution Poor muscle tone Tall Normal intellect but some educational/psych problems

Question 485

Features of Noonan syndrome

Answer 485

``` Aut dom Characteristic facies Short webbed neck, pectus excavatum, short stature CHD - pulmonary stenosis and ASD Mild learning difficulties sometimes ```

Question 486

DiGeorge syndrome?

Answer 486

``` CATCH22 Cardiac abn - esp tetralogy of fallot Abnormal fascies Thymic aplasia Cleft palate Hypocalcaemia, hypoparathyroism 22q deletion ```

Question 487

What is fragile x and sx

Answer 487

``` Trinucleotide repeat disorder of X chromosome Sx worse in males: Learning difficulties, autism Long thin face with large low set ears High arched palate Macro orchidism Hypotonia Females = normal to mild sx ```

Question 488

Airway problem with genetic condition?

Answer 488

Pierre-robin syndrome - posterior displacement of tongue

Question 489

William’s sndrome sx

Answer 489

``` Short stature Transient neonatal hypercalcaemia Supraclavicular AS Friendly, extrovert personality Learning difficulties ```

Question 490

What is duschenne muscular dystrophy

Answer 490

X linked recessive Deletion of dystrophin gene which connects muscle fibre cytoskeleton to surrounding extracellular matrix through cell membrane Causes calcium influx, breakdown of calcium calmodulin complex and excess free radicals = myofibre necrosis Causing elevated plasma creatine kinase

Question 491

Sx of DMD - 4

Answer 491

Waddling gait Gower’s sign Language delay Pseudohypertrophy of calves due to replacement of muscle fibres by fat and fibrous tissue

Question 492

Prognosis of DMD

Answer 492

Not ambulance by 10-14yo | Death from resp failure and associated cardiomyopathy in late 20s

Question 493

Manage DMD - 4

Answer 493

Physio and splints to prevent contractures CPAP at night for weakness of intercostal muscles causing nocturnal hypoxia Glucocorticoids - preevent scoliosis and preserve mobility Gene altering drugs to skip nonsense mutation and produce small amount of dystrophin

Question 494

Achondroplasia sx and inheritance

Answer 494

``` Aut dom, 50% de novo Short stature due to limb shortening Large head with frontal bossing Depression of nasal bridge Short and broad hands Lumbar lordosis Hydrocephalus ```

Question 495

Marfans sx

Answer 495

Aut dom condition of CT Tall, long thin limbs, arachnodactyly Arm span greater than height Hyperextensible joints High arched palate Upwards dislocation of lenses, severe myopia Chest deformity and scoliosis

Question 496

Complication of marfans

Answer 496

``` Degeneration of media of vessel walls - dilated incompetent aortic root - valvular incompetence, mitral valve prolapse and regurg - aortic aneursym may dissect or rupture Monitor with echo ```

Question 497

PKU

Answer 497

Learning difficulties, seizures, microcephaly Testing in newborn screening Manage with phenylalanine restricted diet and anticonvulsants

Question 498

Birth defect associations

Answer 498

``` [VACTORL] Vertebral Anorectal Cardiac Transoeosophagela fisttula Oesophageal atresia Renal Limb ```

Question 499

ALL blasts

Answer 499

>20% of BM, should be 1-2%

Question 500

Cause of auer rods

Answer 500

AML, from myeloperoxidase, seen in blasts

Question 501

Sx of adenotonsilar hypertrophy

Answer 501

Excess pharyngeal lymphoid tissue = OSA, snoring, mouth breathing, recurrent inf Surrounding structures collapse when asleep = worse

Question 502

ALL B cell genetics and treatment

Answer 502

Philadelphia - adults - poor prognosis 12;21 - children - good prognosis Chemo

Question 503

RF for glue ear

Answer 503

``` Boys Down’s syndrome Winter Cleft palate Atopy ```

Question 504

Exam for glue ear

Answer 504

Retracted or bulging drum Grey/yellow Fluid level

Question 505

Cause of CML and progression

Answer 505

CML = BCR:ABL tyrosine kinase = increased cell division of myeloid cell (allows progressions through cycle without check points) Increased granulocytes and monocytes Build up = hepatospenometaly, more risk of mutations = progress to AML = blast crisis

Question 506

What is CLL and progression

Answer 506

B cell receptor interference - makes all the time, not just infection Smudge cells Build up in lymph system (come from blood) = lymphadenopathy Can form tumour = non-hodgkin’s lymphoma = richter’s transformation

Question 507

Sx of chronic leukaemias

Answer 507

Fatigue, infection, bleeding Lymphadenopathy in CLL Hepatosplenomegaly in CML Also hyperuricaemia (gout) and hyperviscosity sx

Question 508

Manage glue ear and ADRs

Answer 508

active observation for 3m Auto inflation through balloon in nose Surgery if persistent and bilateral, significant hearing difference, or getting worse - grommets for 3-12m and recheck hearing after. ADR = infection, tympanosclerosis, need ear plugs to swim

Question 509

Lymphoma? type of cell

Answer 509

Solid mass in lymph node B cells more common - mostly diffuse large B cell, very aggressive Burkit’s - EBV - starry sky appearance to microscope Hodgkin’s - alcohol assoc pain, reed-sternberg cells

Question 510

Lymphoma presentation

Answer 510

Bowel obstruction SC compression BM failure From lymphoma proliferation around body

Question 511

Pathogens in otiismedia

Answer 511

RSV, rhinovirus | Pneumococcus, H influenze, moraxella

Question 512

What is tonsiliis

Answer 512

Pharyngitis with intense tonsillar inflammation often with purulent exudate From group a b-haemolytic strep (systemic sx) or EBV

Question 513

EBV-caused blood cancers - 2

Answer 513

Burkitts or Hodgkin’s

Question 514

treat tonsilitis ad indications

Answer 514

Pen V PO or IV benzylpenicillin for 10d to avoid rheumatic fever and post strep glomerulonephrittis Tonsillectomy: - 7 in 1y - 5 in 1y for 2y - 3 in 1y for 3y - 2w of lost school in 1y - peritonsillary abscess - 4-6w after treated, or 2nd attack - febrile seizures - hypertrophy = airway obstruction, difficulty in delutination, interfering with speech - suspicion of malignancy - unilateral enlarged = ?lymphoma

Question 515

What is nappy rash and treatment

Answer 515

irriantcontact dermatitis if not changed regularly or diarrhoea W shaped, flexures/skin folds spared Erythematous and scalded appearance Trt - dry and clean, protective emollient, mild topical steroids

Question 516

Differnetial for nappy rash and treat

Answer 516

Candidiasis - includes flexures, anterior perineum and perianal Satellite lesions = pathognomic Widespread, vividly red, sharply bordered Triggered by systemic abx Treat - topical fungal (nystatin), oral nystatin to clear GI tract infection, cotrimoxazole cream

Question 517

Common scalp condition in babies

Answer 517

``` Seborrhhoeic dermatitiss - cradle cap <2mo, unknown cause Thick yellow scales on forehead, scalp - also neck, ears, nappy area and skin folds Not itchy, child not disturbed Most resolve alone +- emollient ```

Question 518

Small raised yellow bumps on face

Answer 518

Pearly-white, yellow Nose, cheeks, eyelids, forehead, chest Retention of keratin and sebaceous material from pilosebacious glands which are not fully formed - sebum = white, sweat = yellow Harmless, disappear in a few weeks, very common

Question 519

Large red tumour, complications and treatment

Answer 519

Cavernous haemangioma - strawberry naevus More common in females, low birth weight, premature, multiple gestations At birth or first few weeks, as flat red area, then develops raised dimple lesion, grow up to 4x size until 4y then regress - first sign is pale area in centre Cutaneous or internal - liver, heart, trachea, brain Very large can cause left to right shunt and HF Treat with b blockers if airway/brain/vision effected, as inhibits VEGF

Question 520

Port wine stain?

Answer 520

Vascular malformation of developmental origin, ectasia of superficial dermal capillaries Most often on face and unilateral with cut off distinct Flat at birth, darken and thicken with age Treat with laser if disfiguring Assoc with congenital glaucoma and sturge-Weber syndrome

Question 521

What is raised, pearly papules with central umbilication

Answer 521

Molluscum contagiosum, from DNA pox virus PAinless or itchy Disappear in a few months

Question 522

Red rash on day 2-3

Answer 522

Erythema toxicum neonatorum Harmless, disappears by day 5-7 Never in preterm babies Firm yellow/white small raised bumps filled with pus - eosinophils- on dark red area of skin Concentrated on trunk but begin on face - differentiate from lamp erythema which is only on exposed areas Never on palms and soles

Question 523

What is Mongolian blue spot

Answer 523

Dermal melanocytosis Blue/black pigmented lesion/birth mark present at birth or first couple of months 80% lumbosacral, usually afrocaribbean or Asian babies From trapping melanocytes in dermis during migration from neural crest to epidermis No colour/size change with time - differentiate from bruise

Question 524

What is naevus simplex

Answer 524

‘Stork bite mark’ 30-50% of children Forehead, eyelids, nose and back of head Distension of dermal capillaries Fade in 12m (except bac of head and neck)

Question 525

Acne in babies and manage? And assoc complicatiotns

Answer 525

Maternal oestrogen and progesterone Isotretinoin, plus steroid for first 5d to prevent initial flare Can also cause PV bleed or distended abdo from uterine dilatation in females for up to 6w

Question 526

What is neonatal pustular melanosis

Answer 526

Heals with time, doesn’t need abx Will leave brown marks Causes neutrophilia

Question 527

Eczema and managemet

Answer 527

``` Punched out erosions - risk of infection Can present at weaning - assoc with allergey Cream - water based (alcohol preservatives sting) Ointment - oil based Emollient to wash in Topical steroids: - mild = hydrocortisone - moderate = clobetasone butyrate - potent = betnovate - very potent = dermatovate Wet wraps and topical emolients ```

Question 528

ADR of steroids for eczema

Answer 528

Striae Petechia Atrophy Systemic if full body long term

Question 529

complication of eczeema

Answer 529

Eczema herpeticum | Can cause blindness if aoraund eyes

Question 530

What is impetigo and treatment

Answer 530

Localised highly contagious staph +- strep inf Lesions on face, neck and hands Red macules - rupture - yellow crusty lesions (spread rapidly) Manage - topical abx, oral flucllox if severe Avoid school until lesions are dry

Question 531

Causes of rash with fever

Answer 531

Viruses - roseola infantum, slapped cheek, measles, rubella Bacteria - scarlet fever, rheumatic fever, Lyme disease, salmonella typhi Others - Kawasaki, JIA

Question 532

Sudden high fever and generalised rash

Answer 532

Roseola infantum: Human herpes virus 6 40 degree for 3-5d +- sore throat, runny nose, cough, diarrhoea, swollen eyelids Generalised macular or maculopapular rash on trunk and neck +- limbs after temperature settles - for 1-2d Cervical lymphadenopathy Complication = febrile seizures

Question 533

red cheeks sparing nasal and periorbital regions

Answer 533

Slapped cheek syndrome - parvovirus B19 Common in spring 3 phases: 2-4d = bright red erythema over cheeks sparing nasal, periorbital, perioral regions Next 1-4d = erythematous macular to morbilliform eruption affecting extensors Days to weeks - fades and leaves reticulated lacy pattern

Question 534

Complications of slipped cheek syndrome

Answer 534

Adults = arthralgia and arthritis | Aplastic crisis, foetal hydrocephalus and death

Question 535

Rubella ?

Answer 535

Respiratory aerosols transmission, generally benign erythematous, 50% asymptomatic Younger = mild constitutional sx, erythematous rash, sub occipital adenopathy, eye pain and conjunctivitis Older = arthralgia, arthritis, thrombocytopoenia Purpura Incubate 14-21d Over 3d: 1. Exanthum on neck, spread to trunk and extremities within 24h 2. Begins to fade 3. Disappear

Question 536

Measles

Answer 536

Very contagious, 90% secondary Resp droplets or small particle aerosols, infectious before and after rash - exclude from school for 4d from rash onset Incubate 7-10d then 3Cs: Cough, Coryza, Conjunctivitis Maculopapular rash 2-4d after fever, starting on face with koplik spots in mouth, spread to trunk and extremities

Question 537

Complications of measles

Answer 537

Immunodeficiency - secondary bact and viral inf Resp - pneumonia, tracheeitiss Neuro - febrileseizures, encephalitis, subacute sclerosing pannencephalitis Other - corneal ulceration, diarrhoea, hepatitis, myocarditis

Question 538

Sore throat, fever, strawberry tongue

Answer 538

Scarlet fever - group a b haemolytic strep 5-15yo, aerosol or skin contact, 1-4d incubation Sore throat, headache, fever, flushed cheeks, rough red rash after 12-72h then fades and peels (desquamation) after 3-4d

Question 539

Complications of scarletfever

Answer 539

Rheumatic fever Glomerulonephrittis Erythema nodosum

Question 540

Long fever and conjunctivitis and rash

Answer 540

Kawasaki disease - CRASH Conjunctivitis bilateral Rash polymorphous Adenopathy - acute non purulent lymphadenopathy esp cervical Strawberry tongue or red cracked lips/mucosa Hands and feet changes - red soles and desquamation >5d fever

Question 541

Manage Kawasaki diseae

Answer 541

High dose aspirin for 2w or until afebrile IVIG within 10d of onset, once diagnosed and can repeat after 48h if no improvement Angiography for coronary artery aneurysm risk

Question 542

Shedding skin in infants and newborns

Answer 542

Scalded skin syndrome - staph a - toxin damages outer layer of skin Immature immune system Widespreadpatchy red rash with little blisters and progresses rapidly to cover up mos of skin surface = raw painful patches Fever Abx IV fo 2d then PO Clean skin with soap substitute, moisturise and analgesia

Question 543

What does herpes simplex virus cause

Answer 543

Through mucous membranes or skin - local damage - asymptomatic - gingivostomatitis - vesicles on lips, gums, anterior tongue, hard palate - cna’t eat or drink - with fever, can last 2w —- PO/IV aciclovir, analgesia - cold sores - eczema herpeticum - widespread vascular lesions on eczematous skin = emergency - herpetic whitlow - blepharitis or conjunctivitis - meningitis. Or encehalitis

Question 544

chickenpox

Answer 544

Varicella zoster - herpes virus 3 Airborne resp droplets and direct vesicle contact, 10-21d transmission Rash on face, torso and scalp then resto f body - vesicles with erythematous halo then central umbilicatiotn and crusting - see all stages a same time Resolves spontaneously i 4-10d, exclude from school for 5d from onset/ until no more vesicles

Question 545

Complications of chicken pox

Answer 545

Secondary bact inf - staph or strep - if persistent high temp - toxic shock or necrotising fasciitis Encephalitis - mostly cerebellitis,aseptic meningitis - a week after rash onset with cerebellar signs Immunocompromised = disseminate - haemorrhagic lesions, pneumonitis, DIC Give aciclovir if exposed and ISS

Question 546

Red spots on tongue and hands

Answer 546

Hand foot and mouth - cocksackie a16 <10yo High fever,cough,sore throat, stomach ache Red spots on tongue/inside mouth then yellow/grey ulcers Hands and soles - spots become blisters

Question 547

HSP sx

Answer 547

Acute IgA mediated disorder of generalised vasculitis - small vessels of skin, GI, kidneys, joints,lung, CNS Headache, anorexia, fever Rash on legs and buttocks: Erythematous andmacular/urticarial - blanching papules - nonblanching palpable Purpura Colicky abdo pain and vomiting, haematemesis, meleana - steroids if severe Joints - tender,, swollen and painful knee/ankle - no warmth, erythema and effusion SC oedema Testicular swelling (and can cause torsion ) Renal involvement -haematuria, nephrotic syndrome, can progress to CKD and hypertension - renal biopsy to determine treatment

Question 548

Monitor after HSP

Answer 548

BP and urine for 6m as renal involvement can prevent late

Question 549

Manage HSP

Answer 549

NSAIDs if renal function good and platelet production good Steroids if complications Follow up for renal complications

Question 550

Meningococcal septicaemia

Answer 550

Neisseria meningitidis in blood stream, droplet spread with prolonged exposure Rash - erythematous or maculopapular then petechia and purpura Fever, malaise, vomiting, headache, drowsy then cerebral oedema

Question 551

Meningococcaemia pathology - 4

Answer 551

Microvascular injury from endotoxins: Vascular permeability increases hypovolaemic shock Vasoconstrictor and vasodilation = blue and cold, or warm peripheries with bounding pulse and acidosis Loss of thromboresistance and intravascualr coagulation causes purpura, infarction, gangrene Myocardial dysfunction causes hypotension and electrolyte disturbance

Question 552

Causes of meningitis

Answer 552

``` N men, strep Pn, h infl GBS, E. coli in neonates Chemo Fungi, virus Invade meningitis from blood - infl and leak of proteins cause oedema, alter cerebral blood flow and metabolism Endotoxins causes endothelial damage ```

Question 553

What is glandular fever, sx and diagnosis and treatment

Answer 553

Infectious mononucleosis EBV Petechiae on palate, fever, malaise, tonsillitis/pharyngitis, lymphadenopathy, hepatosplenomegaly Diag - atypical t lymphocytes on blood film, positive monospot test Trt - symptomatic, corticosteroids if airway compromised. Penicillin if group a strep on tonsils, but NOT amoxicillin as can cause maculopapular rash with group a strep

Question 554

Features of ADHD

Answer 554

Inattention hyperactivity and impulsivity Age inappropriate <7yo onset and duration >6m IQ>50 Home and school/nursery, directly observed Doesn’t meet criteria for developmental abn, anxiety or mania

Question 555

Manage ADHD

Answer 555

PArental support and psych education CBT if school age Social skills training Methylphenidate (Ritalin - CNS stimulant to increase mood and concentration), then atomoxetine (NA reuptuake inhibitor - takes longer to work) - ADR headache, insomnia, loss of appetite

Question 556

Inv ADHD

Answer 556

Hyperthyroid | Hearing test

Question 557

What is conduct disorder

Answer 557

Persistent pattern of antisocial behaviour,, poor prognosis for antisocial PD treat with parental management,family therapy, and management

Question 558

Autism features

Answer 558

1. Social interaction 2. Communication 3. Behaviour and interests restricted, stereotyped and repetitive Signs of abn/impaired development <3yo Not attributable to other developmental disorder

Question 559

RF for autism

Answer 559

``` Maternal age Maternal drugs eg valproate Premature Hypoxia at birth Male Family history ```

Question 560

Autism differentials

Answer 560

Learning disability Asperers - but no language or intelligence abn Rett - girls, severe progressive Deafness

Question 561

Screen for autism and manage

Answer 561

``` CHAT- features in toddlers Key worker assigned Parental education CBT social skills training Modify environment Special schooling Support groups ```

Question 562

Rf for bronchiolitis

Answer 562

CLD, CHD <32w <3mo Immunodeficiency

Question 563

CF presentation in neonate, infant and child

Answer 563

Neonate - Guthrie, meconium, jaundice Infant - recurrent chest inf, steatthorea, failure to thrive Child - nasal polyp,rectal prolapse, bronchiectasis

Question 564

Hypoglycaemia in neonate - what level

Answer 564

<2mmol/l

Question 565

Stool in hyperbilirubinaemia

Answer 565

Chalky, white stools

Question 566

What is haemolytic disease of newbordn

Answer 566

Maternal IgG specific to abo blood group antigens pass through placenta and case haemolysis In first baby, no worse in subsequent pregnancies

Question 567

complication of meconium aspiration

Answer 567

Pneumothorax

Question 568

Commonest cause of RDS in newborn

Answer 568

transient tachypnoea of newborn

Question 569

Conditions assoc with autism

Answer 569

Fragile x Tourette’s Epilepsy

Question 570

Complicatiotns of gastroenteritis - 2

Answer 570

Haemorrhagic e coli - can cause haemolytic uraemic syndrome | Post-gastroenteretis syndrome - lactose intolerance

Question 571

What is antiTTG type of Ig?

Answer 571

IgA

Question 572

bed wettin differentials - 3

Answer 572

UTI Impaction Osmotic diuresis from diabetes - urine dip

Question 573

Causes of daytime enereiss

Answer 573

``` Lack of bladder sensation - development, psychogenic Neuropathic bladder UTI Constipation Ectopic ureter ```

Question 574

4 causes of nephrotic syndrome

Answer 574

Vasculatides - HSP Goodpasture’s IgA nephropathy after URTI Familial nephritis - alport’s

Question 575

Features to ask if suspecting familial nephritis

Answer 575

Sensorineural deafness Ocular defects End stage renal diseae in early adulthood X linked recessive

Question 576

UTI in kids - sx and RF (4)

Answer 576

50% underling structural abnormality Diarrhoa, vomit Increased risk of UTI if - infrequent, hurried, constipation, VUR

Question 577

Simple vs complex febrile convulsion

Answer 577

Simple: <15m, no other in 24h, no neuro problem, generalised Complex: >15m, more than 1 in 24h, focal neuroo

Question 578

When to start sc insulin after dka - 4

Answer 578

Clinically well Drinking pH normal Ketones <1

Question 579

Imagin for UTI

Answer 579

<6mo - always USS - during acute infection if recurrent or atypical, or within 6w if typical and recover within 48h >6m - USS during acute infection if atypical, or within 6w if recurrent follow all atypical/recurrent up with DMSA in 4-6m unless atypical >3yo (no need)

Question 580

Investigations in newly diagnosed diabetes

Answer 580

TFTs coeliac screen hbA1c Islet cell autoantibodies

Question 581

What is polycysic kidney disease and inheritance

Answer 581

Aut recessive Cystic dilatation of collecting ducts Assoc with congenital hepatic fibrosis - with biliary disgenesis and perioportal fibrosis Can lead to pulmonary hypoplasia = hyponatraemia, hypertension and renal failure UTIs, portal hypertension with haematemeis

Question 582

What is abdo tumour in kids and inv and treat

Answer 582

Wilm’s nephroblastoma, undifferentiated mesoderm Down regulates IGF-2 <4yo, painless palpable abdo mass Fever, flank pain, haematuria, hypertension USS renal pelvis distortion and hydronephrosis CT/MRI for surgery planning Nephrectomy and chemo

Question 583

What is rheumatic fever

Answer 583

Group a b-haemolytic step Causes heart valve disease, erythema nodosum, polyarthritis Major and minor criteria in Jones diagnostic criteria Treat: Aspirin, prednisolone