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Anki 3a exports > paediatrics anki 1 > Flashcards

paediatrics anki 1 Flashcards

1
Q

Describe the anatomy of a patient with androgen insensitivity syndrome

A

Testes in abdomen/inguinal canal
Absence of uterus, vagina, cervix, fallopian tubes and ovaries

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2
Q

Describe the possibel presentation of a patient with partial androgen insensitivity syndrome

A

More ambiguous if partial
Micropenis/clitoromegaly
Bifida scrotum
Hypospadias
Diminished male characteristics

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3
Q

Descirbe the symptoms of androgen insensitivity syndrome

A

can present in infancy with inguinal hernias containing testes’primary amenorrhoea’-puberty
little or no axillary and pubic hair
undescended testes causing groin swellings
breast development may occur as a result of the conversion of testosterone to oestradiol
usually slightly taller than female average

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4
Q

Describe the key symptoms of Kawasaki disease

A

High grade fever and CREAM:
Conjunctivits (bilateral and non exudative)
Rash (non-bullous)
Edema/erythema of hands and feet
Adenopathy (cervical, commonly unilateral and non-tender)
Mucosal involvement (strawberry tongue, oral fissures etc)

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5
Q

Describe the management of patients with Kawasaki disease

A

High dose aspirin
IVIG
Echos and close follow up

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6
Q

Describe the rash typically seen in measles

A

Discrete maculopapular rash becoming blotchy and confluent
Desquamation that typically spares the palms and soles may occur after a week
Rash starts behind the ears then spreads to the whole body

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7
Q

Describe the mangement of measles

A

Mainly supportive-antipyretics
Admission for immunossuprressed or pregnant patients
Inform public health->notifiable disease
Vitamin A to children under 2 years
Ribavirin may reduce duration of symptoms but not routinely recommended

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8
Q

Describe the management of people who ocme into contact with patients with the measles

A

If no immunised: offer MMR-should be given within 72 hours

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9
Q

At what age does chicken pox usually occur?

A

1-9 years

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10
Q

Describe the rash associated with chicken pox?

A

Starts as raised red itchy spots on face/chest which then spreads to rest of body
Progresses into small, fluid filled blisters over a few days
Crusts over and heals, usually leaving no scars

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11
Q

Describe the management of chicken pox

A

Trim nails to prevent scratching and infection
Encourage loose clothing
Cooling measures like oatmeal baths and calamione lotion to reduce tiching
Analgesics and antipyretics for symptom relief
If immunocompromised: IV aciclovir and human varicella-zoster immunoglobulin (VZIG)

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12
Q

Describe the epidemiology of rubella

A

Less common now due to widespread vaccination

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13
Q

Describe the presentation of a patient with rubella

A

Fever: low grade
Coryza
Arthralgia
A rash that begins on the face and moves down to the trunk
Lymphadenopathy, especially post-auricular and suboccipital

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14
Q

Describe the rash associated with rubella

A

Maculopapular rash that starts on the face before spreading to the whole body, usually fades by day 3-5

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15
Q

Describe the pathophysiology of diphtheria

A

Releases an exotoxin encoded by a Beta-prophage
Exotoxin inhibits protein synthesis by catalyzing ADP-ribolysation of elongation factor EF

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16
Q

Describe the presentation of a patient with staphylococcal scalded skin syndrome

A

Superficial fluid-filled blisters, often leading to erythroderma
Desquamation and positive Nikolsky sign
Perioral crusting or fissuring with oral muscoa unaffected
Skin has a ‘scalded’ look due to loss of superficial layers of epidermis
Fever and irritability common due to underlying infection

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17
Q

Describe how a patient with meningitis might present?

A

Fever
Neck stiffness
Severe headache
Photophobia
Rash
Focal neurological deficits.signs of raised ICP

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18
Q

Describe the management of meningitis

A

<3 months: IV amoxicillin(or ampicillin) and IV cefotaxime
>3 months: IV cefotaxime (or ceftriaxone)
Dexamethasone
>3 months and bacterial
Fluids
Cerebral monitoring and supportive therapy
Public health notification and antibiotic prophylaxis

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19
Q

Describe the epidemiology of Fifth disease

A

Common in late winter and early spring

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20
Q

Describe the management of Fifth’s disease

A

Supportive: rest, hydration etc
Hospitalisation for severe complications

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21
Q

Describe the epidemiology of pneumonia in children

A

Highest incidence in infants
Young infants: usually viral
Older children: usually bacterial
Viral causes mroe common in the winter

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22
Q

Describe the symptoms of pneumonia in children

A

Usually preceded by an URTI
Fever
Difficulty breathing
Lethargy
Poor feeding

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23
Q

Describe the aetiology/risk factors of asthma

A

Genetics
Atopy(allergy, eczema)
Allergen exposure
Prematurity
Cold air
Low birth weight
Viral bronchiolitis early in life
Parental smoking

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24
Q

Describe the pathophysiology of asthma

A

Bronchial inflammation->oeadema and increased mucus production and infiltration with eosinophils, mast cells, neutrophils, lymphocytes->bronchial hyperresponsiveness->reversible aurflow obstruction

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25
Q

Describe the stepwise management of asthma in children over 5

A

SABA PRN(salbutamol)
ICS prophylaxis(beclomethasone)
LTRA(montelukaust)
Stop LTRA and add LABA(salmeterol)
Switch ICS/LABA for ICS +MART(formeterol and ICS)
Add separate LABA
High dose ICS(>400mcg) and referral

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26
Q

Descirbe the stepwise maangement of asthma in children<5 years

A

SABA PRN(salbutamol)
ICS prophylaxis(beclomethasone)-trial for 8 weeks then refer
LTRA(montelukaust)
Stop LTRA and add LABA(salmeterol)
Switch ICS/LABA for ICS
MART(formeterol and ICS)
Add separate LABA
High dose ICS(>400mcg) and referral

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27
Q

Describe the features of a SABA?

A

Short acting B2 agonists
Salbutamol/terbutaline
Few side effects, effective for 2-4 hours

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28
Q

Describe the features of a LABA

A

Long acting B2 agonists
Salmeterol/formoterol
12 hours
Can’t be used without ICS

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29
Q

Describe the features of ipratropium bromide

A

Anticholinergic bronchodilator
Young infants if other bronchodilators uneffective
Treatment for severe acute asthma

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30
Q

Describe the features of an ICS

A

Inhaled corticosteroids
Decrease airway inflammation->prophylaxis
Systemic side effects: impaired growth, adrenal suppression, altered bone metabolism

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31
Q

Describe the features of severe acute asthma

A

Too breathless to talk/feed
Use of accessory neck muscles
O2<92%

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32
Q

Describe the management of a severe acute asthma attack

A

O2 via facemask/nasal prongs
SABA: 10 puffs nebulised or through spacer
Oral prednisolone/Iv hydrocortisone
Nebulised ipratroprium bromide if poor response
Repeat bronchodilators every 20-30 minutes as needed

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33
Q

Describe the management of a life threatening asthma attack

A

O2 via face masks/nasal prongs
Nebulised B2 agonist and ipatropium bromide
IV hydrocortisone
Senior clinician involvement
If poor response:
Transfer to HDU->CXR and blood gases
IV salbutamol/aminophylline
Bolus of IV magenisum sulphate

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34
Q

Describe the epidemiology of croup

A

Children: 6 months-6 years
Peak incidence aged 3
Common in autumn
Highly prevalent-> affects 1/6 children at least once in their life

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35
Q

Describe the presentation of a child with mild croup

A

Occasional barking cough with no audible stridor
No recession
Child eating and drinking as normal

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36
Q

Describe the presentation of a patient with moderate croup

A

Frequent barking cough
Prominent stridor
Marked sternal recession
Agitated child
Tachycardia

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37
Q

Describe the management of mild croup

A

At home with simple analgesia, fluids, rest etc
Single dose dexamethasone in primary care
Minimise crying as this will worsen airway obstruction

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38
Q

Describe the management of moderate/severe croup

A

Admission to hospital
Monitoring: may need ENT intervention
Nebulised adrenaline for severe symptoms
Minimise crying

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39
Q

Describe the presentation of a patient with bronchiolitis

A

Sharp, dry cough
Laboured breathing/wheezing
Tachypnoea/tachychardia
Subcostal.intercostal recessions
Cyanosis/pallor
Fine end-inspiratory crackles and high pitched wheezes
Hyperinflation of the chest->prominent sternum, liver displacement downwards
Low grade fever, cough, rinhorrhoea, nasal congestion

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40
Q

Describe the at home management of bronchiolities

A

Supportive management-> fluids, simple analgesia etc

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41
Q

Describe the management of bronchiolitis in the hospital

A

Oxygen through nasal cannula/fluids
CPAP if respiratory failure
Suctioning of secretions
If severe: antiviral therapy(ribavarin)

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42
Q

Describe the pathophysiology of bronchiolitis obliterans

A

Bronchioles injured due to infection/inhalation of harmful substance
Leads to build up of scar tissue from an overactive cellular repair process
Scar tissue obstructs bronchioles-> impaired O2 absorption
Can lead to respiratory failure

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43
Q

Describe the aetiology and pathophysiology of cystic fibrosis

A

Mutations in CFTR protein on chromosome 7-> defects in chloride transport across cell membranes-> thick mucus secretions and impaired ciliary function
Secretions can block the pancreatic ducts-> enzyme deficiency and malabsorption

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44
Q

Describe the management of acute epiglottitis

A

Immediate senior involvement: ENT, anaesthetics
Endotracheal intubation
Culturing and examination of throat once airway secure
Oxygen
Nebulised adrenaline
IV antibiotics: 3rd gen cephalosporin: IV cefotaxime/ceftriaxone

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45
Q

Describe the pathophysiology of viral induced wheeze

A

Small airways->inflammation and oedema-> triggers smooth muscles of airway to constrict-> narrowing->wheeze
Restricted airway-> respiraotry distress

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46
Q

Describe the management of viral induced wheeze

A

Same as acute asthma treatment
SABA via spacer max 4 hourly up to 10 puffs
LTRA and ICS via spacer

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47
Q

Describe the epidemiology of otitis media

A

Common, especially in those <4 years
Often occurs post viral URTI

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48
Q

Describe the pathophysiology of otitis media

A

Secondary to oedema and narrowing of eustachian tube-> prevents middle ear from draining-> predisposing it to colonisation of bacteria

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49
Q

Describe the epidemiology of glue ear

A

Peaks at 2 years of age
Commonest cause of conductive hearing loss in children

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50
Q

Describe the management of glue ear

A

Audiometry to assess extent of hearing loss
Conservative-> wait and monitor, give it 3 months to resolve
If not resolved in >3 months: refer for grommets and adenoidectomy

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51
Q

Describe the pathophysiology of strabismus

A

In childhood before eyes have fully established connections with brain, brain copes with misalignment byy reducing signal from less dominant eye
One dominant eye and one ‘lazy’ eye
Lazy eye becomes progressively more disconnected resulting in ambylopia

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52
Q

Describe the presentation of a patient with impetigo

A

Erythematous macule that vesiculates or pustulates
Superficial erosion with a characteristic golden crust

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53
Q

Describe the management of localised non-bullous impetigo

A

Topical hydrogen peroxide
1%Fusidic acid or mupirocin

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54
Q

Describe the management of widespread non-bullous impetigo

A

Topical fusidic acis/mupirocin OR antibioics for 5 days(flucloxacillin)
Clarithromycin(allergic) or erythromycin(pregnancy) as alternative antibiotics

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55
Q

Describe the management of bullous impetigo

A

Oral antibiotics or up to 7 days
Flucloxacillin
Clarithromycin or erythromycin as alternatives

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56
Q

Describe the aetiology of toxic shock syndrome

A

TSS is caused by the exotoxin produced by certain strains of bacteria, acting as a superantigen.
This causes polyclonal T cell activation and massive cytokine release, notably IL-1 and TNF-alpha, leading to shock and multi-organ failure.

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57
Q

Describe the presentation of a patient with toxic shoxk syndrome

A

Early, non-specific flu like symptoms
Rapid progression to high fever, widespread rash
Multi-organ involvement -hypotension for cardiac depression and confusion for encephalopathy

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58
Q

Describe the management of toxic shock syndrome

A

DRABCDE
Aggressive fluid and electrolye resusciation
Immediate cessation to persisting infection sources
Antibiotics: clindamycin and cephalosporin
Corticosteroids in some cases

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59
Q

Describe the epidemiology of scarlet fever

A

Children aged 2-6 years
Peak incidence: 4 years

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60
Q

Describe the rash associated with scarlet fever

A

Red-pink blotchy macular rash with rough ‘SANDPAPER’ skin
Starts on trunk and spreads outwards

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61
Q

Describe the presentation of a patient with scarlet fever

A

Fever: 24-48 hours
Malaise, headache, sore throat, n+v
Strawberry tongue
Rash

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62
Q

Describe the pathophysiology of an ASD

A

Shunt from left to right
Blood continues to flow to lungs so no cyanosis
Increased blood flow to right side-> right side overload,right heart failure and pulmonary hypertension
Over time pulmonary pressure->systemic pressure->right to left shunt and cyanosis(Eisenmenger syndrome)

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63
Q

Describe the pathophysiology of coarctation of the aorta

A

Narrowing of aortic arch->reduced pressure of blood flowing to distal arteries and increases pressure in the heart and first 3 branches of the aorta(proximal)

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64
Q

Describe the signs of coarctation of the aorta in infancy

A

Tachypnoea and increased work of breathing
Poor feeding
Grey and floppy baby

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65
Q

Describe the signs of coarctation of the aorta in an older child

A

Left ventricular heave due to left ventricular hypertrophy
Underdeveloped left arm where there is reduced blood flow to the left subclavian artery
Underdevelopment of the legs
Adults: hypertension

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66
Q

A neonate is found to weak femoral pulses, how would you further investigate?

A

Suspect coarctation of aorta
Perform a 4 limb blood pressure: high blood pressure in limb supplied from arteries that come before the narrowing and lower blood pressure in lumbs that come after the narrowing

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67
Q

Describe the pahophysiology of a ventricular septal defect

A

Hole in ventricular septum
L-R shunt as pressure in left is greater: no cyanosis
Right sided overload, RHF, increased flow to pulmonary vessels and pulmonary hypertension
Over time, R pressure >L , R->L shunt->cyanosis(Eisenmenger syndrome)

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68
Q

Describe the medical management of tet spells

A

Oxygen
Beta blockers
IV fluids
Morphine
Sodium bicarbonate
Phenylehrine infusion

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69
Q

Define transposition of the great arterie

A

Attachments of the aorta and pulmonary trunk to the heart are transposed
RV pumps blood into the aorta
LV pumps blood into pulmonary vessels

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70
Q

Describe the epidemiology of transposition of the great arteries

A

M>F
Maternal diabetes

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71
Q

Describe the aetiology of transposition of the great arteries

A

Failure of the aorticopulmonary septum to spiral during septation
Aorta arises from RV and pulmonary vessels arise from LV
2 parallel circuits incompatible with life

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72
Q

Describe the symptoms of transposition of the great arteries

A

Cyanosis at/shortly after birth
Tachypnoea
Poor feeding/weight gain

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73
Q

Describe the symptoms of Ebstein’s anomaly

A

Cyanosis
SOB and tachypnoea
Poor feeding
Collapse
Heart failure symptoms like oedema

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74
Q

Describe the pathophysiology of congenital aortic valve stenosis

A

Aortic valve usually 3 leaflets, may have 1/2/3/4 leaflets isntead

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75
Q

Describe the symptoms of congenital aortic valve stenosis

A

Asymptomatic
Severe:Fatigue
SOB
Dizziness
Fainting
Symptoms worse one exertion
May present with heart failure a few months after birth

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76
Q

Describe the symptoms of congenital pulmonary valve stenosis

A

Asymptomatic-picked up accidentally
Fatigue on exertion
SOB
Dizziness
Fainting

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77
Q

Describe the management of congenital pulmonary valve stenosis

A

Mild-watch and wait-monitor
Ballon valvoplasty via venous catheter to dilate valve
Open heart surgery

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78
Q

Describe the epidemiology of noctunral enuresis

A

M>F
Roughly 2/3 will have a strong family history
Children generally healthy
Secondary type is associated with psychological stress

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79
Q

Describe the management of nocturnal enuresis

A

General advice:
Fluid intake
Toileting patterns-> encourage bladder emptying
Reward systems->’Star charts’ use for good behaviour(like using the toilet before bed), not for ‘dry’ night

1st line:
Enuresis alarm
Sensor pads that sense wetness
High success rates

2nd line:Desmopressin(Synthetic ADH)

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80
Q

Describe the aetiology of typical haemolytic uraemic syndrome

A

Toxin induces damage to the endothelium of glomerular capillary bed causing thrombotic microangiopathy

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81
Q

Describe the aetiology of atypical haemolytic uraemic syndrome

A

Familial-> dysregulation in complement cascade triggers atypical haemolytic uraemic syndrome

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82
Q

Describe the pathophysiology of haemolytic uraemic syndrome

A

Endothelial injury-> microvascular thrombosis->AKI+MAHA+thrombocytopenia

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83
Q

Describe the epidemiology of a UTI

A

Higher prevalence in males until 3 months, then higher prevalence in females

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84
Q

Describe the symptoms of a UTI in infants <3 months

A

Fever
Vomiting
Lethargy
Irritability
Poor feeding
Failure to thrive
Offensive urine

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85
Q

Describe the symptoms of a UTI in an infant aged between 3-12 months?

A

Fever
Poor feeding
Abdo pain
Vomiting

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86
Q

Describe the symptoms of a UTI in a child >1yr?

A

Frequency
Dysuria
Abdo pain
Haematuria

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87
Q

Describe some signs that would point towards an upper UTI

A

Fever->38 degrees
Loin pain and tenderness

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88
Q

Describe the management of a UTI in a patient <3 months

A

Immediate referral to a paediatrician
ABX

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89
Q

Describe the management of a UTI in a child >3 months

A

If upper: consider admission, oral cephalosporin/co-amoxiclav for 7-10 days
If lower: Oral nitrofurantoin/trimethoprim and safety net(bring back if no improvement in 24-48 hours)

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90
Q

Describe the epidemiology of vesicoureteric reflux

A

1-3% of children
Often familial predisposition

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91
Q

Describe the presentation of vesicoureteric reflux

A

Recurrent/atypical UTI’s
Persistent bacteriuria
Unexplained fevers, abdominal/flank pain
If severe: renal scarring-> hypertension and CKD

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92
Q

Describe the conservative management of vesicoureteric reflux

A

Prophylactic antibiotics to prevent UTIs
Monitor kidney function and growth
Treat constipation

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93
Q

Describe the surgial management of vesicoureteric reflux

A

Ureteral reimplantation

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94
Q

Describe the epidemiology of Wilms’ tumour

A

Children <5 years
Incidence peaks 3-4 years

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95
Q

Describe the management of Wilms’ tumour

A

Urgent review(within 48 hours)
Nephrectomy
Chemotherapy
Radiotherapy if advanced

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96
Q

Describe the pathophysiology of cryptorchidism

A

Incomplete migration of testis during embryogenesis from original retroperitoneal position near kidneys to final position in scrotum

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97
Q

Describe the management of bilateral undescended at birth testicles

A

Urgent referral within 24 hours
Genetics/endocrine-> rule out congenital adrenal hyperplasia
Review at 3 months
Refer to surgeons by 6 months
Orchidopexy at 6-18 months

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98
Q

Describe the management of unilateral cryptorchidism at birth

A

Review at 6-8 weeks
Then review at 3 months
Then review at 5 months
Refer by 6 months
Orchidopexy at 6-18 months

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99
Q

Describe the epidemiology of hypospadias

A

3/1000
Genetic element

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100
Q

Describe the features of hypospadias

A

Ventral urethral meatus
Hooded prepuce
Chrodee(ventral curve of penis) in severe cases
Urethral meatus may open more proximally in severe variants

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101
Q

Describe the amangement of hypospadias

A

Refer to specialist
If very distal, may not need treatment
Corrective surgery at around 12 months-DO NOT CIRCUMCISE

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102
Q

Describe the aetiology of phimosis

A

STI’s
Eczema
Psoriasis
Lichen planus/lichen sclerosis
Balanitis

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103
Q

Describe the presentation of a patient with phimosis/paraphimosis

A

Non-retractable foreskin-> may interfere with urination/sexual function
Paraphimosis->swollen and painful glanss, tight band of foreskin->ischaemia->discolouration and severe pain

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104
Q

Describe the management of phimosis

A

Wait and see
Topical corticosteroids
Stretching exercises
Personal hygiene

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105
Q

Describe the management of paraphimosis

A

Manual pressure
Osmotic agents
Puncture techniques
Surgical reduction and circumcision
Personal hygiene advice

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106
Q

Describe the pathophysiology of nephrotic syndrome

A

Damage to glomerular basement membrane and podocytes results in increaed permeability to protein
Lower plasma oncotic pressure->hypoalbuminaemia and oedema

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107
Q

Describe the management for nephrotic syndrome

A

High dose steroids, taper over time
Diuretics for oedema
Low salt diet

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108
Q

Describe a typical presentation of nephrotic syndrome in a child

A

Well child, insidious onset of pitting oedema, initially periorbital then generalised
History of recent URTI
Can progress to anorexia, GI changes, ascites, oliguria, SOB
Risk of infection/thrombosis

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109
Q

Descirbe the aetiology of minimal change disease

A

Idiopathic in most cases
Often seen post viral URTI
Drugs: NSAID’s, rifampicin
Hodgkin’s lymphoma
Infectious mononucleosis

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110
Q

Describe the management of minimal change disease

A

Oral corticosteroids->prednisolone, tapering regime
If poor response: immunosuppressives like ciclosporin/cyclophosphamide
Fluids restriction and lower salt intake
If high fluid overload: furosemide

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111
Q

Describe the prognosis of minimal change disease

A

1/3 resolve completely with no other episodes
1/3 have further relapses requiring further steroids
1/3 dependent on steroid therapy

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112
Q

Describe the pathophysiology of IgA nephropathy

A

IgA immune complexes become lodged in the mesangium of the glomerulus
Combination of IgA deposition, activation of the complement pathway and cytokine release lead to glomerular injury

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113
Q

Describe the management of IgA nephropathy

A

Isolated haematuria+no/minimal protenuria(<500-1000mg/day)+normal GFR: follow up to check renal function
Persistent protenuria(>500-1000mg/day)+normal/slightly reduced GFR: initial treatment with ACE inhibitors
Active disease: Falling GFR/no response to ACE inhibitors: immunosuppression with corticosteroids

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114
Q

Describe the prognosis of IgA nephropathy

A

30% progress to end stage renal failure

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115
Q

Describe the presentation of post strep glomerulonephritis

A

Haematuria(visible-ribena/coke), oliguria, hypertension +/-oedema 1-3 weeks post infection(s.pyogenes)
Some may be asymptomatic

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116
Q

Describe the maangement of post strep glomerulonephritis

A

Usually self resolving
Handle AKI

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117
Q

Describe some symptoms of hypogonadism

A

Lethargy
Weakness
Weight gain
Loss of libido
Erectile dysfunction
Gynaecomastia
Depression

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118
Q

Describe the management of hypogonadism

A

Hormone replacement therapy: usualy testosterone injections/oral
Monitoring therapy to check for polycythaemia, changes in bone mineral density, prostate status and LFTs

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119
Q

Describe the symptoms of Klinefelter’s syndrome

A

Taller height than average
Lack of secondary sexual characteristics
Small, firm testes
Infertile
Gynaecomastia-increased risk of breast cancer
Reduced libido
Wider hips
Weaker muscle
Subtle learning difficulties

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120
Q

Describe the prognosis of Klinefelter’s syndrome

A

Close to normal

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121
Q

Describe the epidemiology of Turner’s syndrome

A

Roughly 1/2500
Incidence DOES NOT increase with maternal age
Low risk of recurrence

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122
Q

Describe the clinical features of Turner’s syndrome

A

Short stature
Shield chest, widely spaced nipples
Webbed neck
High arching palate
Wide carrying angle/cubitus valgus
Delated/incomplete puberty
Primary amenorrhea
Bicuspid aortic valve, coarctation of the aorta
Infertilitiy

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123
Q

Describe the management of Turner’s syndrome

A

Human growth hormone: during childhood to increase height
Oestrogen replacement therapy: allow development of secondary sex characteristics, prevents osteoporosis
Medical care to manage associated problems, including fertility treatment

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124
Q

Describe the epidemiology of Down’s syndrome

A

Common
Incidence increases with increasing maternal age, especially if resulting from gamete non-disjunction

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125
Q

Describe the facial features of Down’s syndrome

A

Upslanting palepbral fissures
Prominent epicanthic folds
Brushfield spots in iris
Protruding tongue
Small, low-set ears
Round/flat face
Brachycephaly(small head with flat back)
Single transverse palmar crease

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126
Q

Describe the management of Down’s syndrome

A

MDT approach
OT, SALT, Physio, dietitiacn, paeds, GP
ENT and audiologist for ear problems
Cardiologists for congenital heart disease
Opticians for glasses

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127
Q

Describe the inheritance of Fragile X

A

X-linked dominant
Males always effects, females may or may not be(have spare copy of FMR1 gene on other X chromosome)

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128
Q

Descirbe the features of Noonan syndrome

A

Webbed neck
Pectus excavatum
Short stature
Pulmonary stenosis

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129
Q

Describe the features of Pierre-Robin syndrome?

A

Micrognathia
Posterior displacement of the tongue->upper airway obstruction
Cleft palate

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130
Q

Describe the features of Prader Willi syndrome

A

Hypotonia
Hypogonadism
Obesity
Short stature
Dysmorphic features
Typical history: feeding is a challenge initially due to hypotonia, then becomes hyperphagia

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131
Q

Describe the features of William’s syndrome

A

Very sociable
Starburst eyes(star like pattern on iris)
Wide mouth with big smile
Short stature
Learning difficulties
Friendly, extroverted personality
Transient neonatal hyperglycaemia
Supravalvular aortic stenosis

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132
Q

Describe the features of a patient with Duchenne muscular dystrophy

A

3-5 yrs present with progressive proximal muscle weakness
Calf pseudohypertrophy
Gower’s sign
30% also have intellectual impairment

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133
Q

Describe the prognosis of Duchenne muscular dystrophy?

A

Most can’t walk by age 12 years
Uusally survive until 25-30 years
Associated with dilated cardiomyopathy

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134
Q

Describe the features of myotonic dystrophy?

A

Progressive muscle weakness
Prolonged muscle contraction: patient can’t let go after shaking someones hand, or release grip on a doorknob
Cataracts
Cardiac arrhythmias

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135
Q

Describe the features of Angelman syndrome

A

Fascination with water
Happy demeanour
Widely spaced teeth
Also learnign difficulties, ataxia, hand flapping, ADHD, dysmorphic features, epilepsy etc

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136
Q

Describe the management of Angelman syndrome

A

No cure, MDT holistic care appproach
Physio and OT
CAMHS
Parental education
Educational and social services support
Anti-epileptic medication if needed

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137
Q

Describe the management of prader willi syndrome?

A

Growth hormone
Dietary management to prevent obesity
PT and exercise problems
Educational interventions to support cognitive development

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138
Q

Describe the features of Noonan syndrome

A

Turner’s(webbed neck, wide nipples, short, pectus carinatum/excavatum)
Pulmonary valve stenosis
Ptosis
Triangular shaped face
Low set ears
Coagulation problems: Factor 9 deficiency

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139
Q

Describe the management of William’s syndorme?

A

MDT approach
Echos and BP monitoring for cardiac complications: aortic stenosis and hypertension
Low calcium diet and avoid calcium and vitamin D supplements: hypercalcaemia

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140
Q

Describe the inheritance pattern of osteogenesis imperfecta?

A

Autosomal dominant

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141
Q

Describe the aetiology of osteogenesis imperfecta

A

Mutations in COL1A1 and COL1A2 which code for the alpha chains of type 1 collagen
Decreased synthesis of pro-alpha 1 or pro-alpha 2 collagen polypeptides

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142
Q

Describe the features of a patient with osteogenesis imperfecta

A

Presents in childhood
Fractures following minor trauma
Blue sclera
Deafness secondary to osteosclerosis
Dental imperfections
Bone deformities like bowed legs and scoliosis
Ligament laxity leading to joint hypermobility

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143
Q

Describe the management of osteogenesis imperfecta

A

Orthopaedic interventions: treat fractures and bone deformities
Medical management: bisphosphoonates to increase bone density
Physio, dental care, hearing aids, education and counselling

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144
Q

Describe the epidemiology of rickets

A

Most common cause: vitamin D deficiency for a long time
Can be caused by: poor nutrition, insufficiency sun exposure and malapbsorption syndromes

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145
Q

Describe the features of rickets

A

Aching bones and joints
Lower limb abnormalities(genu varum-bow legs, genu valgum-knock knees)
Rickety rosary: swelling at costochondral junction
Kyphoscoliosis
Craniobates(soft skull bones in early life)Harrison’s sulcus

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146
Q

Describe the mangement of rickets

A

Oral vitamin D:
400IU/day for children and young people,
6000IU for 8-12 weeks in children <12 years
Calcium and phosphorus supplements may be advised
Prevention:
breastfeeding babies have formula fortified with vitamin D, breastfeeding woman and children should all take vitamin D supplement

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147
Q

Describe the epidemiology of transient synovitis?

A

3-11 years
2x as common in males

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148
Q

Describe the aetiology of transient synovitis

A

After URTI
1-2 weeks prior

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149
Q

Describe the presentation of a patient with transient synovitis

A

Acute onset of limp, often with an avoidance of weight bearing
Pain in hip or referred knee pain
Mild to absent fever

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150
Q

Describe the management of transient synovitis

A

Self-limiting, requiring only rest and analgesia
Typically significant improvement within 24-48 hours
Fully resolve within 1-2 weeks
If fever/no improvement, immediate A&E
Should be followed up at 48 hours and 1 week to check for improvement

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151
Q

Describe the presentation of osteomyelitis

A

Fever
Pain at rest, worse when weight bearing
Swelling
Erythema of the affected site
If chronic: can have history of pain, soft tissue damage etc

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152
Q

Describe the management of osteomyelitis

A

6 weeks flucloxacillin
Clindamycin for pencillin allergy
Vancomycin if MRSA
Surgical debridement may be needed

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153
Q

Describe the symptoms of septic arthritis

A

Acute onset of tender swollen joint
Reduced range of movement
Systemic symptoms: fever, malaise, chills

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154
Q

Describe the management of septic arthritis

A

Epirical IV abx for 4-6 weeks total as IV first then oral
Flucloxacillin 1st line
Clindamycin if penicillin allergy
Vancomycin if MRSA

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155
Q

Describe the epidemiology of Perthes’ disease

A

Predominantly males
Aged 4-8 years

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156
Q

Describe the aetiology of Perthes’ disease

A

Multifactorial: genetics, trauma and other environemntal factors
Disruption in blood supply to the femoral head->avascular necrosis
Disruption can be due to clot formation, increased pressure within the bone or damage to the vessels

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157
Q

Describe the presentation of a patient with Perthes’ disease

A

Gradual onset of limp
Hip pain, which may be referred to the knee
No history of trauma(SUFE)
Persists for >4 weeks
Resitricted hip movements

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158
Q

Describe the diagnosis of Perthes’ disease

A

x-ray: -can be normal, may show sclerosis and fragmentation of epiphysis
Blood tests normal
MRI and technetium bone scan may be done

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159
Q

Describe the management of Perthes’ disease

A

Depends on extent of necrosis:
<50% of femoral head involved: conservative(bed rest, non-weight bearing and traction)
>50%: plaster cast to keep hip abducted or even osteotomy
If <6yrs: observation
Analgesia

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160
Q

Describe the prognosis of Perthes’ disease

A

Most resolve with conservative management

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161
Q

Describe the Catterall staging for Perthes’ disease

A

Clinical and histological features only
Sclerosis with/without cystic changes and preservation of the articular surface
Loss of structural integrity of the femoral head
Loss of acetabular integrity

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162
Q

Describe the epidemiology of Slipped Upper Femoral Epiphysis

A

Increasing with growing rates of childhood obesity

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163
Q

Describe the presentation of a patient with slipped upper femoral epiphysis

A

Typicallly adolescent, obese male going through a growth spurt
May be a history of minor trauma
Hip groin, thigh or knee pain
Restricted range of movement in hip: restricted internal rotation in flexion
Painful limp
Can be bilateral in 10-20% of cases
Trendelenburg gait

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164
Q

Describe the management of slipped upper femoral epiphysis

A

Surgical: internal fixation-cannulated screw
Prompt treatment important to prevent avascular necrosis of the femoral head

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165
Q

Describe the epidemiology of osgood schlatter disease

A

Adolescents ages 10-15
M>F
Higher prevalence in athletes and sports such as gymnastics and basketball

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166
Q

Describe the aetiology of osgood schlatter disease

A

Mechanical stress due to repetitive traciton on tibial tubercle from patellar tendon during rapid growth periods in adolescence
Other contributing factors: tight quadriceps muscle and poor flexibility

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167
Q

Describe the presentation of a patient with osgood schlatter disease

A

Anterior knee pain, often localised to tibial tubercle
Pain exacerbated by running, jumping, kneeling relieved by rest

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168
Q

Describe the prognosis of osgood schlatter

A

Resolves over time
Patient often left with a bony lump on their knee
Rarely avulsion fracture

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169
Q

Aside from positive Barlow and ortolani tests what should be checked on examination of a patient with developmental dysplasia of the hip?

A

Leg length symmetry
Level of knees when hips and knees are bilaterally flexed
Restricted abduction of the hip in flexion

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170
Q

Describe the management of developmental dysplasia of the hips

A

Can self-resolve in 3-6 weeks
Pavlik harness: keeps hips in flexed and abducted position
If severe: surgical intervention

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171
Q

Describe the epidemiology of juvenile idiopathic arthritis

A

Most common cause of chronic joint pain in children

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172
Q

Describe the features of Still’s diseasd (systemic juvenile idiopathic arthritis0

A

Slamon pink rash
Fevers
Lymphadenopathy
Weight loss
Joint pain and inflammation-swelling, stiffness, limited ROM
Splenomegaly
Muscle pain
Pleuritis/pericarditis

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173
Q

Describe the presentation of a patient with polyarticular JIA

A

Symmetrical inflammatory arthritis in >=5 joints
Can affect small joints of hands and feet as well as large joints like hips and knees
Minimal systemic symptoms: may have mild fever, anaemia and reduced growth

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174
Q

Describe the epidemiology of oligoarticular JIA

A

Girls <6 yrs most commonly

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175
Q

Describe the presentation of a patient with oligoarticular JIA

A

Monoarthritis-pain, stiffness, swelling etc
Anterior uveitis->refer to ophthalmology
Usually no systemic symptoms

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176
Q

Describe the presentation of enthesitis related JIA

A

Enthesitis
Anterior uveitis->refer to opthalmology
Check for symptoms of psoriatic arthritis
IBD symptoms

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177
Q

Describe the signs and symptoms of juvenile psoriatic arthritis

A

Psoriatic arthrtiis
Nail pitting
Onycholysis
Dactylitis
Enthesitis

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178
Q

Describe the management of juvenile idiopathic arthritis

A

Paediatric rheumatology with specialist MDT
NSAIDS: e.g. ibuprofen
Steroids: oral, IM or intra-articular in oligoarthritis
DMARDS: methotrexate, sulfasalazine, leflunomide
Biologics: TNF inhibitors: etenercept, infliximab, adalimumab

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179
Q

Describe the aetiology of torticollis

A

Unclear
Often thought to be related to posture or hevay carrying loads

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180
Q

Describe the management of torticollis

A

Reassurance: self resolve within 24-48 hours
Simple anaglesics
Physio
Intermittent heat or cold packs to reduce pain and spasms, sleep on firm pillow and maintain good posture
Advice against cervical collar

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181
Q

Describe the epidemiology of adolescent idiopathic scoliosis

A

10-18 years

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182
Q

Describe the signs and symptoms of a patient with scoliosis

A

Postural asymmetry
Absent or minimal pain
No neurological symptoms
Paraspinal prominences on forward bending
Shoulder asymmetry
Waist line asymmetry

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183
Q

Describe the management of scoliosis

A

Determined by Cobb angle
<10 degrees: regular exercise
11-20 degrees: observational monitoring and regular exercise
21-45 degrees: bracing and regular exercise
>45 degrees: surgical spine arthrodesis and regular exercise

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184
Q

Describe the aetiology of discoid meniscus

A

Developmental anomaly before birth

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185
Q

Describe the presentation of a patient with a discoid meniscus

A

Visible or audible palpable snap on terminal extension(10-20 degrees) along with pain or swelling and locking
Click during movement

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186
Q

Describe the management of discoid meniscus

A

Physio
If severe: arthroscopic partial meniscectomy

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187
Q

Describe the pathophysiology of leukaemia

A

Genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal white blood cell
Excessive production can suppress other cell lines->pancytopenia
Pancytopenia: Anemia, leukopenia, thrombocytopenia

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188
Q

Describe the presentation of a patient with leukaemia

A

Anaemia
Neutropenia: high WCC but low neutrophil levels
Frequent infections
Thrombocytopenia resulting in bleeding
Hepatosplenomegaly
Bone pain
May have DIC or thrombocytopenia-petechiae

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189
Q

Descire the management of tumour lysis syndrome

A

Good hydration and urine output before chemo
Allopurinol or rasburicase to suppress uric acid levels

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190
Q

Describe the management of CML

A

Tyrosine kinase inhibitors(associated with BCR-ABL defect): imatinib
Hydroxyurea
Interferon alpha
Allogenic bone marrow transplant

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191
Q

Describe the management of ALL

A

Combinatino chemo
CNS prophylactic agents
Maintainence therapy

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192
Q

Describe the ppresentation of a patient with CLL

A

Often asymptomatic-incidental lymphocytosis
Infections, bleeding, weight loss, anaemia-warm autoimmune haemolytic anaemia
Non tender symmetrical lymphadenopathy

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193
Q

Describe the prognosis of CLLL

A

Variable
1/3 don’t progress
1/3 progress slowly
1/3 progress actively

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194
Q

Describe the epidemiology of paediatric brain tumours

A

Leading cause of cancer related deaths in children
Most common solid organ malignancy in paediatric population

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195
Q

Describe the presentation of a child with a brain tumour

A

Persistent headaches which are worse in the morning
Signs of raised ICP: nausea, vomiting and reduced consciousness
Seizure in an older child with no fever and no history of seizures
Focal neurological deficits

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196
Q

Describe the presentation of a patient with a neurblastoma

A

Abdominal mass
Pallor, weight loss
Bone pain, limp
Hepatomegaly
Paraplegia
Proptosis

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197
Q

Describe the epidemiology of pyloric stenosis

A

1-3/1000 live births
6-8 weeks
M>F
First-borns most commonly

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198
Q

Describe the aetiology of pyloric stenosis

A

Genetics
Prematurity

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199
Q

Describe the presentation of a patient with pyloric stenosis

A

Postprandial vomiting: non bile stained, projectile, worsens after feeds
Palpable mass: hypertrophied pyloric sphincter palpable as smooth olive sized mass in RUQ/mid epigastric
May have constipation and dehydration

200
Q

Describe the management of pyloric stenosis

A

Supportive:nil by mouth and IV fluids
Surgical: Ramstedt pyloromyotomy(cuts hypertrophic sphincter and widens gastric outlet)

201
Q

Describe the epidemiology of mesenteric adenitis

A

More prevalent in children and adolescents
Link with viral pathogens-follows rep URTI

202
Q

Describe the aetiology of mesenteric adenitis

A

Viral: EBV, adeno, entero
Bacteria: Yersinia, camylobacter
Other: mycobacterium, salmonella, strep

203
Q

Describe the presentation of a patient with mesenteric adenitis

A

Diffuse abdominal pain: often mistaken for appendicitis
Low grade fever
Generalized abdominal tenderness
Pharyngitis.sore throat
Children: usually good overall health and unaltered appetitie

204
Q

Describe the presentation of a patient with intussusception

A

Paroxysmal, severe colicky pain, often causing the child to draw up his legs
Lethargy and decreased activity between pain episodes
Refusal of feeds
Vomiting: may be bile stained depending on location of intussusception ‘red current jelly’ stool-> blood stained mucus
Abdominal distention
Palpation: sausage shaped mass in RUQ

205
Q

Describe the management of intussusception

A

If stable: rectal air insufflation or contrast enema
Surgery

206
Q

Describe the epidemiology of intestinal malrotation

A

Considered rare-critical
Symptomatic malrotation most commonly presents in neonates

207
Q

Describe the aetiology of intestinal malrotation

A

Ariuses due to abnormal rotation and fixation of the mdigut during embryonic development
Usually happens during 4th-12th weeks gestation
Genetics may play a role

208
Q

Describe the features of GORD in children

A

Typically before 8 weeks
Vomiting regurgitation (milky vomits after feeds, can occur after being laid flat)
Distress, crying or unsettled after feeding
Poor weight gain
Vomiting
Reluctance to feed
Chronic cough

209
Q

Describe the management of GORD in children

A

Lifestyle:
Position during fees: head at 30 degrees
Sleep on backs to reduce risk of cot death
Ensure not overfed

Dietary:Thickened feed(containig rice starch, cornstarch etc)-if breastfeeding

Alginate therapy(Gavison mixed with feeds)-NOT for use at same time as thickened feed
PPI(e.g. omeprazole) only in certain situations

210
Q

Describe the epidemiology of appendicitis in children

A

Uncommon under 3 years
One of the most common acute surgical problems in children
Common in populations with a western diet

211
Q

Describe the aetiology of appendicitis

A

Obstruciton within appendix
Can be fibrous tissue, foreign body, hardened stool
Subsequent bacterial multiplication and infiltration can lead to tissue damage, pressure induced necrosis, perforation
Gangrene: thrombosis in appendix’s arterial supply, specifically ileocolic artery

212
Q

Describe the symptoms of a patient with appendicitis

A

Central abdominal pain radiating to the right iliac fossa
Low grade pyrexia
Minimal vomiting, nausea

213
Q

Describe the management of a patient with appendicitis

A

Prophylactic antibiotics: full sepsis 6 if appropriate
Laparoscopic appendicectomy
If evidence of perforation: open with lavage in theatre
If negative imaging: IV fluids and abx

214
Q

Describe the epidemiology of biliary atresia

A

F>M
Neonates only: perinatal(1-2 weeks of life) or postnatal(208 weeks)

215
Q

Describe the pathophysiology of biliary atresia

A

Either obliteration or discontinuity within the extrahepatic biliary system resulting in obstruction of bile flow
Results in neonatal presentation of cholestasis

216
Q

Describe the presentation of a patient with biliary atresia

A

First 2 weeks of life
Jaundice beyond physiological 2 weeks
Dark urine and pale stools
FTT

217
Q

Describe the management of biliary atresia

A

Surgical: Kasai procedure-hepatoportenterostomy
Post surgery: abx and bile acid enhancers

218
Q

Describe the prognosis of biliary atresia

A

Good if surgery
Liver transplant in 1st 2 years of life if failure

219
Q

Describe the epidemiology of febrile convulsions

A

Common: 3% of children
Children 6 months and 5 yrs

220
Q

Describe the aetiology of febrile convulsions

A

Abrupt rise in body temperature often related to an indection&
Can be triggered by bacterial and viral infections
Mc: URTI, ear infections and childhood exanthems

221
Q

Describe the symptoms of a febrile convulsion

A

High fever: >38 degrees
Tonic-clonic , LOC
Post ictal drowsiness/confusion

222
Q

Define constipation in children

A

<= 3 stools/week or significant difficulty in passing stools

223
Q

At what age is encopresis considered pathological?

224
Q

Describe the management of constipation in children

A

Correct reversible causes: increase fibre and hydration
Laxatives(movicol first line)
Faecal impaction with disimpaction regimen
Encourage and praise visiting toilet
Laxatives used short term then weaned off

225
Q

Describe the epidemiology of cerebral palsy

A

Mc cause of major motor impairment
Higher in areas with worse ante/perinatal care
Higher in premature infants and those of multiple pregnancies

226
Q

Describe the features of spastic cerebral palsy

A

Increased tone and reflexes(flexed hip and elbow,
‘clasp-knife’ spasticity
‘Scissor’ gait
Can be monoplegic, diplegic or hemiplegic

227
Q

Describe the features of dyskinetic cerebral palsy

A

Athetoid movements and oro-motor problems
Can exhibit signs of parkinsonism

228
Q

Describe the features of ataxic cerebral palsy

A

Typical cerebellar signs
Uncoordinated movements

229
Q

Describe the management of cerebral palsy

A

MDT approach-physio, OT, SALT, dieticians
Oral diazepam
Oral baclofen-muscle spasms
Botulinum toxin type A-contractures
Surgery
Orthopaedic surgery
General surgery e.g. for PEG fitting

230
Q

Describe the prognosis of cerebral palsy

A

Varibale impact on QOL-difficulties with mobility and communication
Associated with reduced life expectancy

231
Q

Describe the epidemiology of haemolytic disease of the newborn

A

Rare but serious
Most common in pregnancy where there is blood group incompatibility beetween the mother and fetus

232
Q

Describe the aetiology of haemolytic disease of the newborn

A

Immune response following rhesus or ABO blood group incompatibility between mtoehr and fetus
Sensitisation events include: antepartum haemorrhage, placental abruption, ECV, miscarriage/termination, ectopic pregnancy, delivery

233
Q

Describe the features of haemolytic disease of the newborn

A

Hydrops fetalis appearing as fetal oedema in at least 2 compartments, seen on antenatal USS
Yellow coloured amniotic fluid due to excess bilirubin
Neonatal jaundice and kernicterus
Fetal anaemia causing skin pallorr
Hepato/spleno-megaly
Severe oedema if hydrops fetalis whilst in utero

234
Q

Describe the features of a cephalohaematome

A

Commonly affects parietal region
Doesn’t cross suture lines
May take up to 3 months to resolve-managed conservatively

235
Q

Describe the features of caput seccedaneum

A

Soft, puffy swelling due to localised oedema
Crossess suture lines
Resolves within days-no tx needed

236
Q

Describe the interpretation of the APGAR score

A

0-3: very low
4-6: moderate low
7-10: good health

237
Q

Describe the key points of paediatric BLS

A

Unresponsive-> shout for help
Open airway
Look, listen, feel for breathing
5 rescue breaths
Check for signs of circulation
15 chest compressions: 2 rescue breaths

238
Q

Describe the pathophysiology of acute respiratory distress syndrome

A

Acute form of resp failure occuring within 1 week of trigger
Diffuse bilateral alveolar injury with endothelial disruption and leakage of fluid into alveoli from pulmonary capillaries
Decrease in surfactant production but different to neonatal respiratory distress syndrome

239
Q

Describe the clinical features of acute respiratory distress syndrome

A

Acute onset and severe, critically unwell within identifiable trigger like illness or trauma
Severe dyspnoea
Tachypnoea
Confusion and presyncope secondary to hypoxia
Diffuse bilateral crepitations on ausculation

240
Q

Describe the management of acute respiratory distress syndrome

A

Generally ITU
Intubation/ventilation to treat hypoxaemia
Hameodynamic support: aim for MAP>60mmHG, vasopressors, transfusions if Hb<70
Enteral nutrition support
DVT prophylaxis
Treat underlying cause
PPI to prevent gastric ulcers

241
Q

Describe the epidemiology of neonatal respiratory distress syndrome

A

Premature infants

242
Q

Describe the symptoms of neonatal respiratory distress syndrome

A

Within minutes of birth
Rapid, laboured breathing
Flaring nostrils
Gruntig sounds during exhalation
Indrawing of chest wall
Cyanosis
May progress to apnoea and hypoxia due ot fatigue

243
Q

Describe the management of neonatal respiratory distress syndrome

A

Intratracheal instillation of artificial surfactant
Supplemental oxygen/respiratory support: CPAP or mechanical ventilation
Caffeine
Supportive care: maintain body temperature, nutrition, manage other complications

244
Q

Describe the causes of late onset neonatal sepsis

A

S epidermis
S. aureus
P.aeruginosa
Klebsiella
Enterobacter
Pseudomonas
E.Coli

245
Q

Describe the management of neonatal sepsis

A

IV benzylpenicillin and gentamicin(monitor levels)
Re-measure CRP 18-24 hours after presentation in patients given abx
Maintain adequate oxygenation, fluid, glucose levels, metabolic acidosis

246
Q

Describe the aetiology of transient tachypnoea of the newborn

A

C-ssection
Passage through birth canal applies external pressure on thorax, aiding in expelling the birth. and increases fetal adrenaline which helps with breathing(don’t have in C-section)
Suboptimal epithelial clearance mechanisms

247
Q

Describe the presentaiton of transient tachypnoea of the newborn

A

Resp distress:
Tachypnoea(>60bpm)
Increased work of breathing
Potential desaturation/cyanosis

248
Q

Describe the management of transient tachypnoea of the newborn

A

usually self-resolving within 3 days of life
Oxygen to manage hypoxemia
Monitor for progression to penumonia or NRDS

249
Q

Describe the aetiology of meconium aspiration syndrome

A

Fetal distress/hypoxia-> intestinal relaxation+anal sphincter relaxation

250
Q

Describe the signs and symptoms of meconium aspiration syndrome

A

Presence of meconium-stained liquor during rupture of membranes or at birth(yellow/green apppearance of amniotic fluid)
Green staining of infant’s skin, nail beds or umbilical cord
Respiratory distress: tachypnoea, grunting, noisy breathing, cyanosis
Crackles on auscultation
Limp infant/low APGAR scores
Barrel shaped chest

251
Q

Describe the management of meconium aspiration syndrome

A

Gentle suctioning of mouth/nose to remove any visible residual meconium
Abx to reduce infection
Transfer baby to ICU if needed for careful monitoring and oxygen administration
If severe: artificial ventilation might be needed

252
Q

Describe the clinical features of neonatal hypoglyacaemia

A

Asx
Autonomic: jitteriness, irritable, tachypnoea, pallor
Neuroglycopenic: poor feeding/sucking, weak cry, drowsy, hypotonia, seizures
Others: apnoea, hypothermia

253
Q

Describe the management of asymptomatic neonatal hypoglycaemia

A

Encourage normal feeding(breast/bottle)
Monitor blood glucose

254
Q

Describe the management of symptomatic/severe neonatal hypoglycaemia

A

Admit to neonatal unit
IV infusion of 10% dextrose

255
Q

Describe the management of gastroshisis

A

Attempt vaginal delivery
Newborns to theatre as soon as possible after delivery(within 4 hours)-usually requires multiple surgeries to reposition organs back into abdominal cavity and close abdominal wall defect
Abx if infection,
IV fluids/nutrients

256
Q

Describe the pathophysiology of gastroschisis

A

Week 4 of gestation: lateral folds fail to fuse-> hole in abdominal wall-> organs protrude

257
Q

Describe the pathophysiology of exomphalos

A

Midgut herniates through umbilicus->pulls layer of peritoneum into umbilical cord to properly develop due to insufficient space in adbominal cavity->midgut doesn’t return

258
Q

Describe the management of exomphalos

A

C-section-> reduce risk of sac rupture
Staged surgical repair

259
Q

Describe the pathophysiology of duodenal atresia

A

Failure in duodenal vacuolization
During fetal development duodenal epithelium proliferates rapidly->complete duodenal obstruction->apoptosis of excess cells-> formation of small vacuoles which fuse-> re-establish duodenal passageway

260
Q

Describe the aetiologuy of oesophageal atresia and tracheo-oesophageal fistula

A

Associated with:
VACTER syndrome
CHARGE
Chromosomal abnormalities
DiGeorge syndrome
Neural tube defects

261
Q

Describe the management of oesophageal atresia

A

Surgical: connect parts of oesophagus and close off fistula
Post op: monitor for complications
Manage nutritional and respiratory support

262
Q

Describe the epidemiology of necrotising enterocolitis

A

First 3 weeks of life in premature neonates
Fatal in 1/5, significant morbidity

263
Q

Describe the signs and symptoms of necrotising enterocilitis

A

Vomiting(may be bile streaked)
Feed intolerance
Bloody, loose stools
Abdo distention
Absent bowel sounds
Systemic compromise-> acidodis on blood gas, resp distress

264
Q

Describe the management of necrotising enterocilitis

A

Nil by mouth
NG tube for gastric decompression
Broad spectrum abx
Supportive: IV fluids and ventilation
Surgical: resection of necrotic sections of bowel

265
Q

Describe the pathophysiology of congenital diaphragmatic hernia

A

Usually a failure of the pleuroperitoneal cavity to close completely

266
Q

Describe the presentation of congenital diaphragmatic hernia

A

Cyanosis soon after birth
Tachypnoea and tachycardia
Asymmetry of chest wall
Absent breath sounds on one side-usually left with heart shifted to right
Bowel sounds audible over chest wall

267
Q

Describe the management of congenital diaphragmatic hernia

A

Paeds emergency-> reduce pressure in chest
Resus in ‘head up’ position
Endotracheal intubation and careful fluid support
Avoid bag and mask-> stomach and intestines become distended with air and impair lung function
Oro-gastric tube
Surfactant
Open surgical repair of diaphragm when stable

268
Q

Describe the management of neonatal jaundice

A

Admit urgently if:
<24 hours, >7days and unwell, premature
Might not need any treatment if well and likely physiological
Increase fluid intake
Monitor bilirubin levels
Treat underlying cause
Phototherapy
Exchange transfusion

269
Q

Describe the signs ans symptoms of neonatal jaundice

A

Yellowing of skin and eyes
Poor feeding
Lethargy
Severe: kernicterus
If due to hepatitis/biliary atresia: dark stools and pale urine

270
Q

Describe the symptoms of toxoplasma gondii infeciton in pregnancy and neonates

A

Causes toxoplasmosis
Mother: Fever, fatigue
Fetus: chorioretinitis, hydrocephalus, rash, diffuse intracranial calcifications

271
Q

Describe the symptoms of rubella in pregnancy and neonates

A

Mother: lymohadenopathy, polyarthritis, rashes
Fetus: congenital rubella syndrome: deafness, cataracts, rash, heart defects

272
Q

Describe the symptoms of CMV infection in pregnancy and neonates

A

Mother: Mild sx
Infants: rashes, deafness, chorioretinitis, seizures, microcephaly, intracranial(periventricular) calcifications

273
Q

Describe the symptoms of a HSV infection in neonates

A

Blisters and inflammation of the brain: meningoencephalitis

274
Q

Describe the epidemiology of listeria

A

Found in many food products, especially unpasteurised dairy products and soft cheeses
Vertical transmission from mother to fetus through placenta or during delivery

275
Q

Describe the signs and symptoms of listeriosis in neonates

A

Neonatal sepsis
Meningitis
Respiratory distress due to aspiration of infected amniotic fluid
Chorioamnionitis
Premature labour
Stillbirth

276
Q

Describe the management of listeriosis

A

Abx: ampicillin + aminoglycoside(gentamicin)

277
Q

Describe the epidemiology of cleft lip/palate

A

Mc congenital deformity affecting orofacial structures

278
Q

Describe the pathophysiology of cleft lip/palate

A

Polygenic inheritance
Failure of frontal-nasal and maxillary processes to fuse: cleft lip
Failure of palatine processes and nasal septum to fuse: cleft palate

279
Q

Describe the management of cleft lip/palate

A

Cleft lip repaired earlier than cleft palate: 1st week-3 months
Cleft palate: 6-12 months

280
Q

Describe the local features of HSV in a neonate

A

Vesicular lesions on the skin
Eye involvement
Oral mucosa involvement without internal organ involvement

281
Q

Describe the management of HSV in neonates and pregnancy

A

Neonates: Parenteral acyclovir and intensive supportive therapy
Elective C-section/intrapartum IV acyclovir if active primary herpes lesions on mother at term or outbreak within 6 weeks of labour

282
Q

Describe the features of bronchopulmonary dysplasia

A

Low oxygen saturations
Increased work of breathing
Poor feading and weight gain
Crackles and wheeze in chest on auscultation
Increased susceptibility to infection

283
Q

Describe the management of bronchopulmonary dysplasia

A

Typically leave neonatal unit on low dose O2 at home
Followed up after 1 yr to wean off
RSV protection-> monthly injections of palivizumab for certain babies

284
Q

Describe the clinical features of typical(petit mal) absence seizures

A

Onset: 4-8 years, often doesn’t persist into adulthood
Duration few-30 secs, no warning, quick recovery, often lots in one day

285
Q

Describe the clinical features of West syndrome/infantile spasms

A

4-8 months
Flexion of head, trunks, limb-> extension of arms(Salaam attack), lasts 1-2 secs, repeat up to 50 times
clusters-‘jack-knife spasms)
Progressive mental handicap->associated with regression and high morbidity

286
Q

Describe the prognosis of West’s syndrome

A

Poor prognosis-> intellectual disability
Many develop Lennox-Gastaut syndrome later one

287
Q

Describe the clinical features of Dravet’s syndrome

A

Convulsive status epilepticus seizures during intercurrent illness or following vaccination
Refractory to antiepileptic treatment
Associated with loss of developmental milestones and ASD

288
Q

Describe the prognosis of Dravet’s syndrome

A

Estimated 15% mortality by age 20yrs

289
Q

Describe the features of Lennox Gastaut syndrome

A

May be an extension of infantile spasms
Onset 1-5 yrs
Tonic, atonic and atypical absence(last longer and have gradual onset) seizures
Idiopathic: normal psychomotor development
Symptomatic: associated neurological abnormalities

290
Q

Describe the management of Lennox Gastaut syndrome

A

Ketogenic diet
Often refractory to AED’s
Sodium valproate, lamotrigine, clobazam
Surgical: corpus callostomy and vagus nerve stimulation

291
Q

Describe the features of juvenile myoclonic epilepsy

A

Onset in adolescent and early adulthood, more common in girls
Sudden shock like myoclonic seizures that progress into generalised tonic-clonic seizures
Also absence seizures
Often in morning/after sleep deprivation

292
Q

Describe the management of juvenile myoclonic epilepsy

A

Usually good response to AED’s: sodium valproate and lamotrigine

293
Q

Describe the features of panayiotopoulos syndrome

A

Autonomic seizures with ictal vomiting, pupil dilation and syncope lasing up to 30 minutes
Vision changes: flashing lights, blurring, loss of vision3-6yrs onset
Typicaly stop after 2-3 years, otherwise normal development

294
Q

Describe the features of bening rolandic epilepsy

A

Mc in childhood, mc in males
3-10yrs
Paraesthesia(e.g.unilateral face) on waking up
Parents might notice tonic seizure overnight or find child on floor

295
Q

Describe the general management of childhood epilepsy

A

If reversible, treat causeAED’s
Ketogenic/low glycaemic diet
Surgery if refractory/caused by tumours
MDT
Emergency seizure plans for home and school, educate parents

296
Q

Describe the investigations/managment for developmental delay

A

Clinical and neuro exam
Genetics, metabolic screen, MRI/CT, hearing/vision assessments
Refer to specialist
Early intervention services: SALT, OT, Physio, educational support etc

297
Q

At what age is it concerning for a child to not be sitting without support?

298
Q

At what age is it concerning for a child to not be walking unsupported?

299
Q

Describe fine motor and vision milestones with regards to brick buillding

A

15 months: tower of2
18 months: 3
2 years: 6
3 years: 9
Adds a brick every 3 months

300
Q

Describe fine motor and vision milestones with regards to drawing

A

18 months: circular scribble
2 years: vertical line
3 yrs: circle
4 yrs: cross
5 yrs: square and triangle

301
Q

Describe the major soical behavious and play milestones

A

6 weeks: smile
3mths: laughs
6mths: not shy
9 mths: shy, takes everything to mouth

302
Q

Describe the major social behaviour and play milestones with regards to feeding

A

6 months: hand on bottle
12-15 months: uses spoon, drinks form cup
2 yrs: doesnt spill with cup/spoon
3: spoon and fork
5: knife and fork

303
Q

Describe the major social behaviour and play milestones with regards to dressing

A

12-15: helps getting dressed/undressed
18mths: takes off shoes
2 years: puts on hats and shoes
4 yrs: can dress and undress independently except for laces and buttons

304
Q

Describe the major social behaviour and play milestones with regards to play

A

9mths: ‘peek-a-boo’
12 mths: waves bye bye, plays pat a cake
18 mths: plays alone
2 yrs: plays near others
4 yrs: plays with others

305
Q

Describe the aetiology of retinoblastoma

A

Mutations in tumour suppressor gene RB1
Hereditary: germline mutations
Non-hereditary: somatic

306
Q

Describe the signs and symptoms of retinoblastoma

A

Leukoria(white pupil when you shine a light)
Strabismus
Ocular inflammation and redness
Deteriorating vision
Failure to thrive
Eye enlargement: developing countries

307
Q

Describe the management of retinoblastoma

A

Systemic chemo 1st line
Enucleation(remove eye-extensive disease with threat of extraocular spread)
Orbital exenteration
Radio
Genetic counselling

308
Q

Describe the epidemiology of neuroblastoma

A

Mc malignancy in children <5yrs

309
Q

Describe the pathophysiology of neuroblastoma

A

Arises from the neural crest tissue of adrenal medulla(mc) and sympathetic nervous system
Often starts in abdomen and spreads to bones, liver, skin
(haematogenous and lymphatic spread)
Catecholamine secreting tumour

310
Q

Describe the clinical features of neroblastoma

A

Mass effect of primary tumour: constipation, abdo distention
General: FTT, fatigue, malaise

Sx of metastasis:
Spine: numbness, weakness, loss of movement

Neck: breathlessness, Horner’s

Bone: pain and swelling

Bone marrow: leukopenia(infections), thrombocytopenia(bleeding/bruising), anaemia(SOB, pallor)

Skin: small raised, blue/black discoloured lumps

Liver: hepatomegaly and abdominal pain

311
Q

Describe the NICE referral pathway for suspected neuroblastoma

A

Very urgent referral(<48 hours) in children with palpable abdominal mass or unexplained enlarged abdominal organ OR
Unexplained haematuria

312
Q

Describe the management of neuroblastoma

A

Surgery followed by chemo
Radiation to primary site
Isotretinoin-> maintenance therapy-promotes differentiation of neuroblastoma cells into normal cells

313
Q

Describe the signs and sympotms of hepatoblastoma

A

Abdominal mass
Poor appetitie
Weight loss
Lethargy
Fever
Vomiting
Jaundice

314
Q

Describe the management of hepatoblastoma

A

Chemo
Surgery

315
Q

Describe the epidemiology of osteosarcoma

A

Mc primary malignant bone tumour in children and adolescents
Slight M:F predominance
Incidence peaks in adolescence-growth spurts

316
Q

Describe the signs and symptoms of osteosarcoma

A

Prolonged bone pain, often initially mistaken for growing pains/sports injuries
Bone swelling-usually in region of long bone metaphyses
Decreased ROM
Pathological fractures
Mc knee or proximal humerus
Systemic sx

317
Q

Describe the management of osteosarcoma

A

Surgical resection and limb salvage surgery
Radiotherapy
Chemo: multi-agent: methotrexate
Follow up imaging

318
Q

Describe the epidemiology of Ewing’s sarcoma

A

2nd most prevalent bone cancer in children and adolescents

319
Q

Describe the signs and symptoms of Ewing’s sarcoma

A

Nocturnal bone pain
Palpable mass/swelling
Restricted joint mobility
Systemic: fever, weight loss, fatigue

320
Q

Describe the management of Ewing’s sarcoma

A

Chemo 1st line
Surgery
Radiotherapy

321
Q

Describe the epidemiology of Hodgkin’s lymphoma

A

Bimodial distribution: mc in 3rd and 7th decades

322
Q

Describe the features of Hodgkin’s lymphoma

A

Non-tender lymphadenopathy, asymmetrical
May be painful after drinking-characteristic
B symptoms + pruritus
Hepato/splenomegaly

323
Q

Describe the lugano classification for Hodgkin’s lymphoma

A

Stage 1: single lymphatic site
Stage 2: >2 lymph nodes on same side as diaphragm
Stage 3: Involvement on both sides of diaphragm OR above diaphragm with splenic involvement
Stage 4: Disseminated with >;=1 extra lymphatic organs etc
A: asx
B: B symptoms
S: Splenic involvement
E: extranodal contiguous extension

324
Q

Describe the management of Hodgkin’s lymphoma

A

Chemoradiotherapy

325
Q

Describe the features of a medulloblastoma including sx

A

Mc malignant brain turmour in children
Typically arises in cerebellum
Sx: headaches, vomiting, ataxia and cranial nerve deficits

326
Q

Describe the features of a pilocytic astrocytoma including sx

A

Mc benign brain tumour in children
Often in cerebellum or optic pathway
Sx: headaches, nausea, visual disturbances and balance issues

327
Q

Describe the features of a ependymoma including sx

A

Arises from ependymal cells lining the ventricles or central canal of the spinal cord
Common locations: posterior fossa, spinal cord
Sx: hydrocephalus, headache, nausea, balance issues

328
Q

Describe the features of a craniopharyngioma including sx

A

Benign tumour near the pituitary gland and hypothalamus
Sx: endocrine dysfunction, vision problems, growth delays

329
Q

Describe the management of paediatric brain tumours

A

MDT
Steroids-> reduce intracranial swelling
Anticonvulsants
Chemo, radiotherapy
Surgical intervention-> including ventriculoperitoneal shunts or drains to treat hydrocephalus

330
Q

Describe the prognosis of paediatric brain tumours

A

Good for CNS tumours, pilocytic astrocytomas and craniopharyngiomas
Poorer prognosis with gliomas

331
Q

Describe the aetiology of von Willebrand’s disease

A

Usually genetic mutation that results in a deficiency/dysfunction of VWF-usually autosomal dominant inheritance

332
Q

Describe the signs and symptoms of von Willebrand’s disease

A

Excess/prolonged bleeding from minor wounds/post-op
Easy bruising
Menorrhagia
Epistaxis
GI bleeding

333
Q

Describe the management of von Willebrand’s disease

A

Desmopressin: temporarily increases F8 and VWF levels by releasing endoethlial stores
TXA for minor bleeding
VWF-F8 concentrates if above unsuccessful and bleeding persistent

334
Q

Describe the epidemiology of thalassaemia

A

Believed to give some protection against malaria-> found in Mediterranean Europe, central africe, Middle East, india and southeast asia

335
Q

Describe the inheritance of alpha thalassaemia

A

Autosomal recessive

336
Q

Describe the pathophysiology of alpha thalassaemia

A

non-functioning copies of the 4 alpha globin genes on chromosome 16
Symptomatic when >=2 copies of gene are lost

337
Q

Describe the features of alpha thalassaemia if 1 or 2 alpha globulin alleles are affected

A

Alpha thalassaemia trait-mild asx anaemia
Bloods: hypochromic + microcytic but Hb usually normal

338
Q

Describe the features of alpha thalassaemia if 3 alpha globulin alleles are affected

A

Symptomatic haemoglobin H disease
Hypochromic microcytic anemia with splenomegaly
Normal survival

339
Q

Describe the features of alpha thalassaemia if all 4 alpha globulin alleles are affected

A

Incompatible with life
Lack of alpha globin-> excess gamma chains-> Hb Barts
Hydrops fetalis

340
Q

Describe the signs and symptoms of alpha thalassaemia

A

Jaundice
Fatigue
Facial bone deformities

341
Q

Describe the management of alpha thalassaemia

A

Blood transfusions
Stem cell transplant
Splenectomy may be used especially in Hb H

342
Q

Describe the pathophysiology of beta thalassaemia

A

Non-funcitoning copies of the 2 beta globin genes-chromosome 11
Beta thalassaemia minor(trait)-> one functional and one dysfunctional
Beta thalassaemia major: complete absence of beta globin synthesis

343
Q

Describe the signs and symptoms of beta thalassaemia major

A

Presents in 1st yr of life with FTT and hepatosplenomegaly
Severe symptomatic anaemia
Maxillary overgrowth and prominent parieta/frontal bones-> chipmunk face
Frontal bossing-> ‘hair on end’ appearance on skull x ray

344
Q

Describe the management of beta thalassaemia major

A

Regular blood transfusions-> iron overload
Bone marrow transplant-> potentially curative but risks
Iron chelation for iron overload(desferrioxamine, deferiprone)

345
Q

Describe the symptoms of beta thalassaemia minor

A

Usually mild asymptomatic anaemia

346
Q

Describe the epidemiology of sickle cell disease

A

Mc in people of Afrian descent-portection against malaria

347
Q

Describe the pathophysiology of sickle cell disease

A

HbAS instead of normal HbAA
Abnormal beta globin chain polymerises when deoxygenated-> erythrocyte forms a sickle shape.
Makes them susceptible to aggregation and haemolysis-> obstructed blood flow->
vaso-occlusive crisis-> damage to major organs and susceptibility to infections

348
Q

Describe the aetiology of sickle cell

A

Autosomal recessive inheritance
Homozygous: mc and most severe: HbSS
Can have one normal and one abnormal
Can inherit one copy of HbS and other gene for normal HbA-> sickle cell trait

349
Q

Describe the signs and symptoms of sickle cell

A

Vaso-occlusive crisis: severe pain due to tissue ischaemia
Dactylitis common presentation in infants <6 months
Anaemia: increased haemolysis of sickle cellsJaundice-> consequence of haemolysis
Acute chest syndrome: lung infarction or infection

350
Q

Describe the management of an acute sickle cell crisis

A

Pain relief: IV opiates for vaso-occlusive crisis
O2 supplementation as required
IV fluids: improve blood flow
Top-up transfusions: severe crisis/aplastic crisis
Abx if sign of infection

351
Q

Descirbe the long-term management of sickle cell anaemia

A

Hydroxycarbamidee-reduce frequency of crises
Regular transfusions, folic acid supplements, iron chelation therapy
Prophylactic abx (oral penicillin)
Immunisations: flu and pneumococcal
Genetic counselling
Stem cell transplants
Regular transcranial doppler ultrasonography: children 2-16 years
Crizanlizumab: monoclonal antibody: >16yrs

352
Q

Describe the signs and symptoms of fanconi anaemia

A

Cytopenias-> increased bruising/bleeding/infections
Symptomatic anaemia-> impaired oxygen-carrying capacity, aplastic anaemia
Physical abnormalities: short stature, VACTERL-H malformations
Cafe-au lait spots
Increased risk of acute myeloid leukemia

353
Q

Describe the signs and symptoms of haemophilia

A

Usually early in life with spontaenous deep+severe bleeding into soft tissues, joints and muscles-previously joint damage resulting in deforming arthropathy(haemoarthroses and hematomas)
Excessive bleeding post surgery/trauma
Cerebral haemorrhage-> not as common anymore

354
Q

Describe the management of haemophilia

A

Desmopressin if minor
Recombinant factor 8/9 if major bleed If severe: regular prophylactic recombinant clotting factor tx, physio and patient education-> prevention of joint arthropathy
Gene therapy
Vaccination for Hep B, dental advice etc
Antifibrinolytics: TXA for but avoid in muscle haematomas/haemarthrosis

355
Q

Describe the pathophysiology of ITP?

A

Spleen produces antibodies directed against the glycoprotein 2b/3a or Ib-5-9 complex

356
Q

Describe the aetiology of ITP

A

Often triggered by a viral infection or immunisation
Can be secondary due to:
AI conditions(E.g SLE)
Infections(H.pylori, CMV)
Medications
Lymphoproliferative disorders

357
Q

Describe the signs and sx of ITP

A

Bruising
Petechial/purpuric rash
Bleeding(lc)-epistaxis or gingival bleeding
Unusually heavy menstrual flow in women/blood in urine/stools

358
Q

Describe the management of ITP

A

Usually self-resolving(80% within 6 mths)-conservative watch and wait
Avoid team sports etc
TXA may be used especially if menorrhagia
Persistent/very low platelet count:
Oral /IV corticosteroids
IVIG
Platelet transfusions but ONLY in emergency as a temporary measure

359
Q

Describe the prognosis of ITP in children

A

Generally self-resolving1/5: chronic

If not resolved in 6 months: consider differentials including bone marrow aspirate

360
Q

Describe the aetiology of TTP

A

Hereditary: congenital mutatin of ADAMST13
AI: AI inhibition of ADAMST13

361
Q

Describe the clinial features of TTP

A

Pentad of:
Fever
Microangiopathic haemolytic anaemia
Thrombocytopenic purpura

CNS involvement: headache, confusion, seizures
AKI
Rare, typically adult females

362
Q

Describe the management of TTP

A

Fresh frozen plasma-contains vWF
Plasma exchange: removes antibodies and toxins associated with pathogenesis of disease
High dose steroids, low dose aspirin and rituximab

363
Q

Describe the epidemiology of testicular torsion

A

Mc in neonates and males between 13-16 years

364
Q

Describe the signs and symptoms of testicular torsion

A

Sudden onset, severe pain in one testicle
Pain can be referred to the lower abdomen
N+V
Unilateral loss of cremaster reflex
Negative Prehn’s sign: persistent pain despite elevation of testicle
Swollen tender testis retracted upwards
May be hx of previous similar pain episodes whcih self-resolved

365
Q

Describe the management of testicular torsion

A

Urgent surgical exploration
Bilateral orchidopexy-fixation of both testicles to prevent future torsion

366
Q

Describe the prognosis of testicular torsion

A

Worse in neonates-> testis rarely viable

367
Q

Describe the aetiology of testicular torsion in neonates?

A

Torsion is extravaginal-> spermatic cord and tunica vaginalis twist together in or just below inguingal canal

368
Q

Describe the management of testicular torsion in neonates

A

Torted testis is removed and contralateral testis is fixed in place

369
Q

Descirbe the epidemiology of precocious puberty

A

Mc in females than males
Average onset of puberty has been decreasing over the past few decades-thought to be due to obesity

370
Q

Describe the pathophysiology of gonadotrophin dependent precocious puberty

A

‘central/true’
Due to premature activation of hypothalamic-pituitary-gonadal axis
FSH and LH raised

371
Q

Descirbe the pathophysiology of gonadotrophin independent precocious puberty

A

‘pseudo/false’
Due to excess sex hormones
FSH and LH low

372
Q

Describe the management of precocious puberty

A

GnRH analogues to suspend progression of puberty
Surgery to resect tumours
Glucocorticoids for CAH
Depends on underlying cause

373
Q

Describe the inheriitance of Kallmann’s syndrome

A

Usually X-linked recessive

374
Q

Describe the pathophysiology of Kallmann’s syndrome

A

Failure of GnRH -secreting neurons to migrate to the hypothalamus

375
Q

Describe the management of Kallmann’s syndrome

A

Testosterone supplementation
Gonadotrophin supplementation may result in sperm production if fertility is desired later in life

376
Q

Describe the epidemiology of congenital adrenal hyperplasia

A

Boy-more severe
75%: salt-losing
25%: non-salt losing

377
Q

Describe the pathophysiology of congenital adrenal hyperplasia

A

Impaired adrenal steroid biosynthesis
Deficiency in cortisol production->
compensatory overproduction of ACTH by anterior pituitary
Elevated ACTH-> increased production of adrenal androgens-> virilization of female infants and affect genital development

378
Q

Describe the aetiology of congenital adrenal hyperplasia

A

21 hydroxylase deficiency-mc-90%
->cortisol deficiency and excess androgen production
11-beta-hydroxylase17-hydroxylae deficiency: very rare

379
Q

Describe the signs and symptoms of congenital adrenal hyperplasia

A

Ambiguous genitalia(exposure to excessive androgen exposure in utero)-male infants appear normal so delays diagnosis
Salt wasting crisis
Precocious puberty
Virilisation
Infertility
Heigth and growth abnormalities-grow fast initially but end up short

380
Q

Describe the sx of a salt-wasting crisis

A

Dehydration and vomiting
Hyponatraemia
Hyperkalaemia
Circulatory shock and metabolic acidosis
Life-threatening

381
Q

Describe the management of congenital adrenal hyperplasia

A

Glucocorticoid(hydrocortisone) and mineralocorcticoid(fludrocortisone) replacement
Patient education: if unwell: increase hydrocortisone and may need IV fluids
Surgical intervention: virilised females-correct external genital abnormalities

382
Q

Describe the management of obesity in children

A

Encourage healthy lifestyle
>12yrs with severe physical/psychological comorbidities: drug treatment such as orlistat under MDT

383
Q

Describe the epidemiology of congenital hypothyroidism

384
Q

Describe the aetiology of congenital hypothyroidism

A

Primary congenital hypothyroidism
Thyroid dysgenesis-mc cause
Dyshormonogenesis
Secondary or central congenital hypothyroidism
Defects in hypothalamus or pituitary gland leading to low TSH secretion
May be secondary to maternal carbimazole use or maternal antibodies

385
Q

Describe the clinical features of congenital hypothyroidism

A

Prolonged neonatal jaundice
Delayed mental and physical milestones
Short stature
Puffy face, macroglossia
Hypotonia
Myxoedema
Bradycardia
If not dx early: altered neurodevelopment and cognitive disability

386
Q

Describe the management of congenital hypothyroidism

A

Immediate thyroid hromone replacement therapy with levothyroxine
Regular monitoring of TSH and T4 for dose adjustments according to growth
Long term follow up to monitor growth and development and to ensure treatment adherence

387
Q

Describe the epidemiology of pica

A

Mc in young children<5yrs and pregnancy
Higher prevalence in low and middle income countries
Common in individuals with developmental disabilities:
ASD etc and psych disorders(OCD, schizophrenia)

388
Q

Describe the aetiology of pica

A

Nutritional deficiencies: iron and zinc
Developmental and behavioral
Psychiatric disorders: OCD, schizophrenia, ASD,, intellectual disorders

389
Q

Describe the symptoms of pica

A

Persistent craving and ingestion of non-food for at least a month
Geophagia: dirt/clay
Cornstarch
Ice
Paper
Chalk
Soap
Hair

390
Q

Describe the management of pica

A

Iron/zinc supplements
Dietary counselling
Behavioural therapy including CBT
SSRIs: underlying OCD or severe psychiatric disorders
Environmental modification: remove objects in environment, supervision and restricted access to pica substances

391
Q

Describe the epidemiology of eczema

A

Very common
Childhood onset common
Prevalence decreases with age
Urbanisation and industrialisation associated with higher prevalence

392
Q

Describe the pathophysiology of eczema

A

Vast lymphcoytic infiltration into dermis
Often IgE-mediated allergic response to environmental allergens

393
Q

Describe the general features of eczema

A

Itchy, erythematous rash
Repeated scratching can exacerbate affecteed areas
In infants: face and trunk
Younger children: extensor surfaces
Older children: more typical-flexor surfaces and creases of face and neck

394
Q

Describe the management of eczema

A

Conservative: avoid triggers
Simple emollients: use lots
Topical steroids(emollient first then steroid 30 minutes later)
Wet wrapping
Light therapy
Systemic: oral steroids, oral ciclosporin, DMARDS like methotrexate, biologics

395
Q

Describe the symptoms of eczema herpeticum

A

Vesicles and punched out erosions where the vesicles have deroofed will appear
May affect large areas of skin including sites that are not currently eczematous
May be multi–organ involvement

396
Q

Describe the cllinical features of Stevens Johnson syndrome

A

Within a week of medication intake, initially resembling a URTI with cough, cold, fever and sore throat
Rash is maculopapular with characteristic target lesions-> may develop into vesicles or bullae)
Nikolsky sign positive: blisters and erosions appear when skin is rubbed gently
Mucosal involvement<10% of body surface-TEN: >30% if skin

397
Q

Describe the management of Steven-Johnson syndrome

A

Hospital admission
Supportive care-> fluid and electrolyte management, pain control, treat secondary infections

398
Q

Describe the prognosis of Steven-Johnson syndrome

A

10% mortality rate-usually due to dehydration, infection or DIC
TEN: 30% mortality rate

399
Q

Describe the aetiology of allergic rhinitis

A

IgE mediated response to allergens within the environment
Seasonal: hayfever-pollens
Perennial: throughout the year
Occupational: exposure to specific allergens

400
Q

Describe the signs and sx of allergic rhinitis

A

Nasal pruritus
Sneezing
Clear nasal discharge
Post-nasal drip
Nasal pruritus
Eye redness
Eye puffiness
Watery eye discharge

401
Q

Describe the management of allergic rhinitis

A

Avoid triggers
Nasal irrigation with saline
Oral/intranasal antihistamines
Intranasal steroids
Oral steroids
Short course or topical nasal decongestant-not for prolonged periods
ENT referral

402
Q

Describe the features of erythema toxicum

A

Benign rash in newborns
Erythematous macules, papules and pustule
Waxes and wanes over several days-usually no more than one day

403
Q

Describe the rash assocaited with scepticaemia?

A

Non-blanching purpuric rash
Lethargy, headache, fevers, vomiting

404
Q

Describe the rash assocaited with slapped cheek syndrome

A

Rash on both cheeks
Fever, URTI sx

405
Q

Describe the rash associated with hand foot and mouth disease

A

Blisters on hands and feet
Grey ulcerations in buccal cavity, fever, lethargy

406
Q

Describe the rash associated with scarlet fever

A

Coarse red rash on cheeks
Sore throat, headache, fever, bright red tongue
Sandpaper texture rash

407
Q

Describe the rash associated with measles?

A

Erythematous, blanching maculoppapular rash
Fever, cough, runny nose, conjuncitivitis, Koplik spots

408
Q

Describe the rash associated with urticaria

A

Raised, itchy red rashes
Usually not accompanied by fever

409
Q

Describe the rash associated with chickenpox

A

Maculopapular vesicular rash that crusts over and forms blisters

410
Q

Describe the rash associated with roseola

A

Lace-like rash across whole body
High fever

411
Q

Describe the rash associated with rubella

A

Starts on head and spreads to trunk
Post-auricular lymphadenopathy

412
Q

Describe the epidemiology of urticaria

A

Women>men
Peak incidence: 20-40yrs

413
Q

Describe the pathophysiology of urticaria

A

Release of histamine and other mediators form mast cells and basophils-> increased vascular permeability and formation of wheels
Both immune-mediated and non-immune mechanisms contribute to development of urticaria

414
Q

Describe the signs and sx of urticaria

A

Pruritus
Erythematous wheals with well defined borders
Wheals that vary in shape and size
Rapid onset and resolution
Occasionally angiodema-> can involve lips, eyelids or extremities

415
Q

Describe the management of urticaria

A

ID and remove triggers
Pharmacological:Non sedating antihistamine: cetirizine, loratadine
Other antihistamines or LRTA
Short course oral corticosteroids
Symptomatic management: antipruritic creams like calamine lotion

416
Q

Describe the features of stork marks

A

Red/pink patches, often on eyelids/head/neck
Very common
Easier to see when baby cries
Usually fade by age 2 on forehead/eyelids, longer if back of head or neck

417
Q

Describe the features of haemangiomas

A

Blood vessels that form from raised lump on skin
Appear soon after birth, bigger in first 6-12 mths, then shrink and disappear by age 7
More common in girls, premature babies and multiple births
May need tx if affect vision, breathing or feeding

418
Q

Describe the features of port wine stains

A

Red, purple or dark marks usually on face or neck
Present from birth
Usually on 1 sideof body
Sometimes can become lumpier if not treated
Can be made lighter using laser tx
Sign of Sturg-Weber syndrome/Klippel-trenaunay syndrome but this is rare

419
Q

Describe the features of cafe-au-lait spots

A

Light/brown pathces anywhere on the body
Common, lots of children have 1/2
Darker on black/brown skin
Sign of NF1 if >=6 spots

420
Q

Describe the features of blue-grey spots

A

Loook like bruises
There from birth
Mc on babies with black/brown skin
No tx, usually go away by age 4
Should be recorded on medical records-avoid thinking its abuse

421
Q

Describe the epidemiology of anaphylaxis

A

Relatively uncommon
Higher risk in patients with asthma/atopy
Incidence rising especially in Western countries

422
Q

Describe the aetiology of anaphylaxis

A

Type 1 hypersensitivity reaction.
Allergen reacts with specific IgE antibodies causing a rapid release of histamine and other vasoactive substances-> increases capillary permeability causing oedea and shock

423
Q

Describe the clinical features of anaphylaxis

A

Sudden onset and rapid progression of sx
Airway: hoarse voice, lip swelling, stridor indicative of upper airway obstruction and laryngeal oedema
Breathing: wheezing, SOB, fatigue, SpO2<94%
Circulation: tachycardia, hypotension/shock, angioedema, confusion
Others: Generalised pruritusWidespread erythematous or urticarial rash
GI: abdominal pain, diarrhoea, vomiting

424
Q

Describe the acute management of anaphylaxis

A

Immediate IM adrenaline
Remove trigger
Manage ariway and high flow oxygen
IV fluids
If no response, repeat adrenaline

425
Q

Describe the long term management of anaphylaxis

A

Counselling on use of adrenaline auto-injectors
Supply of 2 auto-injectors
Written advice
Referral to local allergy service for follow up

426
Q

 What is rheumatic fever?

A

AI systemic complication of lancefield Group A beta-haemolytic strep infection (scaarlet fever) that occurs 2-4 weeks post infection

427
Q

Describe the epidemiology of rheumatic fever

A

Mc in developing countries

428
Q

Describe the pathophysiology of rheumatic fever

A

2-4 weeks post beta haemolytic strep infection(scarlet fever) autoantibodies generated that target the strep and cross-react with the endocardium leading to valvular disease

429
Q

Describe the split of different vavle diseases occuringin rheumatic heart disease

A

Mitral valve disease: 70% MC
Aortic valve: 40%
Tricuspid: 10%
Pulmonary valves: 2%

430
Q

Describe the minor criteria for rheumatic fever

A

Raised ESR/CRP
Pyrexia
Athralgia(if arthritis not a major criteria)
Prolonged PR interval

431
Q

Describe the management of rheumatic fever

A

Oral/IV bbenzylpenicillin
Analgesia for arhritic sx: NSAIDs, aspirin(not in young children)
Treatment of heart failure: diuretics, ACE inhibitor, surgery for valve defects if severe
Sydenham’s chorea: self-limiting, acutely haloperidol/diazepam

432
Q

Describe the prognosis of rheumatic fever

A

No cure for rheumatic heart diseaseIf severe valvular disease: requires surgery-40% with severe rheumatic heart disease

433
Q

Describe the aetiology of paediatric heart disease

A

Usually congenital heart defects
Acquired: myocarditis, arrhythmias, htn
Different underlying mechanisms compared to adults

434
Q

Describe the signs and symptoms of paediatric heart failure

A

Infants:
Difficulty feeding
Faltering growth

Young children:
Abdo pain and vomiting especially on exertion
Poor appetite

Adolescents:
Exercise intolerance
Fatigue

All ages: cyanosis and hepatomegaly

435
Q

Describe the management of paediatric heart failure

A

Conservative: fluid restriction and dietitian guided feeding plans
Medical: diuretics with inotropic support: ACE inhibitors mc used
Surgical: ventricular assist device to improve circulation
Correction of anatomical defect if present
Heart transplant in end stage cases

436
Q

Describe the prognosis of paediatric heart failure

A

Progressive
Leading cause of mortality in children with heart disease

437
Q

Describe the pathophysiology of infective endocarditis

A

Damage to endocardium-> heart valve forms local blood clot-> platelets and fibrin deposits allow bacterium to stick to endocardium-> formation of vegetation
Valves have no dedicated blood supply-> body can’t launch immune response to vegitations

438
Q

Describe the major criteria for infective endocarditis

A

Blood culture positive for IE
(2 times separate positive blood cultures for 2 sites showing typical microorganisms)
Evidence of endocardial involvement: echo showing vegetation, abscess etc

439
Q

Describe the minor criteria for infective endocarditis

A

Fever: >38 degrees
Immunological: Roth spots, splinter haemorrhages, Osler’s nodes
Vascular: septic emboli, Janeway lesions
Echo
minor criteria
Predisposing features: knwon valve disease, IVDU, prosthetic valve disease
Microbiological evidence that doesn’t meet major criteria

440
Q

Describe the management of infective endocarditis

A

6 week courseof IV abx-usually midline insertion
When organism and sensitivities not known: amoxicillin (vancomycin)

441
Q

Describe the prognosis of infective endocarditis

A

Without tx, can rapidly lead to heart failure and death

442
Q

Describe the aetiology of congenital heart block

A

Maternal systemic AI diseases: SLE and Sjogren: anti-Ro and/or anti La
Structural heart defects
Some cases idiopathic-can run in families

443
Q

Describe the signs and symptoms of congenital heart block

A

Asx
Neonates: bradycardia and/or circulatory shock
Older children: pre-syncope, syncope, usually in first few years of life

444
Q

Describe the management of congenital heart block

A

Asx: close monitoring
Sx: neonatal ICU admission->isoprenalineImplant pacemaker

445
Q

Describe the prognosis of congenital heart block

A

Up to 20% chance of intrauterine fetal death
Good prognosis after pacemaker insertion
20% ris of recurrence in future pregnancies->pre-conception counselling

446
Q

Describe the signs and sx of IBS

A

> =6 monthsAbdo painBloatingChange in bowel habit
Sx made worse by eating
Passage of mucus
Lethargy, nausea, backache, bladder sx also common

447
Q

Describe the management of IBS

A

Dietary and lifestyle: stress maanagement, low FODMAP diet
Antispasmodics: mebeverine, laxatives, anti-diarrhoeal
TCA’sRefractory: psychotherapy including CBT

448
Q

Describe the epidemiology of gastroenteritis

A

Common worldwise and affects all ages
Leadig cause of death in children under 5 worldwide

449
Q

Describe the bacterial causes of gastroenteritis

A

S.aureus-cooked meats and cream products
Bacillus cereus-reheated rice
Clostridium perfringens
Campylobacter
E.coli
Salmonella
Shigella

450
Q

Describe the presentation of a patient with gastroenteritis

A

Diarrhoea and vomitng
Blood in stool-bacterial pathogen
Systemic: malaise and fever
Signs of dehydration/shock if severe

451
Q

Describe the management of gastroenteritis

A

Notifiable if outbreaks
Supportive: fluid replacement orally and using sachets
Clinical dehydration/shock: IV fluids or NG rehydration
Severe and immunocompromised: abx
Ondansetron in severe cases
Gradual reintroduction of food-avoid fruit juice and carbonated drinks

452
Q

Describe the prognosis of gastroenteritis

A

Vomiting 1-2 days, diarrhoea 5-7 days
Most children: complete resolution within 2 weeks
Secondary lactose intolerance: diarrhoea for up to 6 weeks

453
Q

Describe the epidemiology of Crohn’s disease

A

Mc in northern climates and develoepd countries
Bimodal age of onset: 15–40yrs, then 60-80yrs
Mc in caucasians

454
Q

Describe the clinical features of Crohn’s disease

A

GI: crampy abdo pain and non-bloody diarrhoeaa
Systemic: weight los and fever
Aphthous ulcers in mouth
Cachectic and pale(anaemia), clubbing
Erythema nodosum
Pyoderma gangrenosum
Anterior uveitis
Axial spondyloarthropathy
Gallstones
AA amyloidosis

455
Q

Describe the management of Crohn’s disease

A

Smoking cessation
In flare: monotherapy with glucocorticoids(oral/IV prednisolone or hydrocortisone), biologics
Remission: azathioprine, mercaptopurine, methotrexate, biologics(infliximab or adalimumab)
Surgical: treat complications

456
Q

Describe the management of peri-anal fistulae in Crohn’s disease

A

Drainage seton
Fistulotomy

457
Q

Describe the management of peri-anal abscess in Crohn’s disease

A

IV abx: ceftriaxone+metronidazole
Exam under anaesthetic and incision and drainage

458
Q

Describe the clinical features of Ulcerative colitis

A

Diarrhoea containing blood and/or mucus
Tenesmus/urgency, cramps

Systemic: wt loss, fever, malaise, anprexia

Exam: pallor, clubbing

Derm: erythema nodosum, pyoderma gangrenosum

Ocular: anterior uveitis

MSk: sacroilitis

Hepatobiliary: PSCAA amyloidosis

459
Q

Describe the management of mild-moderate Ulcerative colitis

A

Topical amiosalicylate
Oral if no improvement in 4 weeks
Consdiier adding oral prednisolone
Etrasimod
Oral tacrolimus

460
Q

Describe the management of acute severe Ulcerative colitis

A

IV corticosteroids
Add IV ciclosporin of no improvements in 72 hours
Trial etrasimod(velsipity)
Emergency surgery

461
Q

Describe the epidemiology of coeliac disease

A

F>M
Bimodal: infancy or 50-60yrs
Increased incidence in Irish

462
Q

Describe the pathophysiology of coeliac disease

A

Sensitivity to gluten and realted prolamines results in villous atrophy of the lining of the small intestine and results in malabsorption

463
Q

Describe the symptoms of coeliac disease in children

A

Abdo pain
Distention
N+V
Diarrhoea
Steatorrhoea
Fatigue
Weight loss

464
Q

Describe the management of coeliac disease

A

Lifelong gluten free diet
Patient education
Supplements for deficiencies: iron/vitamins etc
Regular monitoring to ensure diet adherence and screen for complications

465
Q

Describe the presentation of a child with malnutrition

A

Poor growth
Plateaued weight gain or weight loss
Difficulties concentrating
Intellectual disability
Specific vitamin deficiencies

466
Q

Describe the management of malnutrition in the uk

A

Vitamin supplements
Increase caloric intake with high-energy food and drinks(smoothies, milkshakes, cheese)
Specific supplements: iron

467
Q

Describe some signs that might point to non-organic FTT

A

Nutritional indicators
Social indicators-signs of neglect(poor hygiene, unattetended medical needs)
Poor parent-child interactions, parental mh issues

468
Q

Describe the management of FTT

A

Stabilisation if severe dehydration/electrolyte imbalances
Nutritional supprot-dietitian
Mineral and vitamin supplementation
Address underlying cause
Follow up to plot growth chart

469
Q

Describe the aetiology of Hirschsprung’s disease

A

Aganglionic segment of bowel due to a developmental failure of the parasympathetic Auerbach and Meissner plexuses

470
Q

Describe the pathophysiology of Hirschsprung’s disease

A

Parasympathetic neuroblasts fail to migrate form the neural crest to the distal colon-> developmental failure of parasympathetic Auerbach and Meissner plexuses->uncoordinated peristalsis-> funcitonal obstruciton

471
Q

Describe the epidemiology of Hirschsprung’s disease

A

Rare
3 x more common in males
Down’s syndrome

472
Q

Describe the management of Hirschsprung’s disease

A

Rectal washouts/bowel irrigation
Definitive: surgery to affected segment of colon

473
Q

Describe the pathophysiology of Meckel’s diverticulum

A

Remnant of vitellointestinal duct which usually disappears around 6th week gestation
Contains ectopic ileal , gastric or pancreatic mucose

474
Q

Describe the presentation of a patient with Meckel’s diverticulum

A

Usually asx
Painless rectal bleeding-ulceration of adjacent tissue
Intestinal obstruction
Intussusception
Abdominal pain mimicking appendicitis

475
Q

Describe the management of Meckel’s diverticulum

A

Laparoscopic surgical resection of diverticulum l if narrow neck/symptomatic using wedge excision or small bowel resection and anastamosis

476
Q

Describe the management of toddler’s diarrhoea?

A

Reassurance will go by 6yrs
4F’s:Fat-higher fat diets improve: whole milk, cheese, ice cream
Fruit juice: limit
Fluid: consider limiting to meal and snack times if drinking too much
Fibre: not high fibre or low fibre

477
Q

Describe the symptoms of infantile colic

A

Bouts of excessive crying and pulling up of legs
Often worse in evening

478
Q

Describe the management of infantile colic

A

Reassurance and supprot for parents
Advice about feeding positions and environments etc: continue feeding normally
If concerns about health then further evaluation: poor weight gain, vomiting, fever etc

479
Q

Describe the epidemiology of cow’s milk protein intolerance

A

First 3 months
Formula fed infants

480
Q

Describe the presentation of infants with cow’s milk protein intolerance

A

Persistent diarrhoea, vomiting, FTT, abdo pain in first few months of life
Usually present after introduction of cow’s milk to the diet
Abdo pain: may draw up legs if younger
Diarrhoea: may be bloody/mucus
Eczema/urticaria
Immediate IgE mediated: urticaria, angioedema, vomiting wheezing within 2 hours of presentation

481
Q

Describe the management of cow’s milk protein intolerance in a formula fed infant

A

Extensive hydrolysed formula milk replacement
Amino acid-based formula as second line

482
Q

Describe the management of cow’s milk protein intolerance in a breast fed infant

A

Continue breasteeding
Eliminate cow’s milk protein from maternal diet
Use extensively hydrolysed formula milk when done breastfeeding until 12 months of age

483
Q

Describe the epidemiology of choledochal cyst

A

Rare
More common in Asian children
3 x as common in girls

484
Q

Describe the signs and symptoms of choledochal cyst

A

Asx-found before birth on antenatal scan
Triad of abdo pain, jaundice and abdominal mass
Jaundice-blocking of bile drainage
Abdo pain
Cholangitis
Peritonitis if cyst bursts/leaks
Pancreatitis

485
Q

Describe the aetiology of neonatal hepatitis

A

Viruses: rubella, CMV, hepatitis A/B/C
Idiopathic-mc
Genetic: A1AT deficiency

486
Q

Describe the presentation of neonatal hepatitis

A

Jaundice
Pruritus
Rashes
Dark urine
Hepatomegaly
FTT

487
Q

Describe the management of neonatal hepatitis

A

Medical: Ursodeoxycholic acid-increase bile formation
Surgery: cirrhotic liver disease/liver transplant if severe
Optimise nutrition and vitamin supplementation

488
Q

Describe the features of a reducible hernia

A

Bowel can be reduced back into abdo cavity
Painless and often asymptomatic

489
Q

Describe the features of a strangulated hernia

A

Serious acute medical condition where a hernia compromises blood supply to intestines or abdominal tissues-> ischaemia and necrosis of affected bowel tissue
Risk of sepsis and bowel perforation

490
Q

Describe the features of an incarcerated hernia

A

Blood supply not necessarily compromised
Presents with abdominal pain and irreducible mass

491
Q

Describe the features of umbilical hernias

A

Often due to failure of umbilical ring to close after umbilical cord falls off
Very common in children
Often close without intervention

492
Q

Describe the features of epigastric hernias

A

Herniation between sternum and umbilicus
Don’t close spontaneously
Not common in children

493
Q

Describe the features of inguinal hernias

A

Protrusion through inguinal canal, entering either the deep inguinal ring(direct) or through weakness in abdominal wall(indirect hernia)
Indirect due to paten processus vaginalis-common in infants
Direct rare in infants

494
Q

Describe the epidemiology of hernias

A

Common in children
M>F
Strangluated more common in adults due to age-related weakening of abdominal wall

495
Q

Describe the presentaiton of a patient with a strangulated hernia

A

Severe abdominal pain
Vomiting
Hx of intermittent pain, especially when hernia still reducible
Signs of bowel obstructions: abdo distention, constipation, inability to pass stool/gas

496
Q

Describe the management of a strangulated hernia

A

Surgical:
Release herniated bowel to restore blood flow
Remove necrotic tissue to prevent sepsis
Reinforce weakened area of abdo wall with mesh etc

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