Selected Notes paeds 1 Flashcards
(495 cards)
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
2
Q
Describe the possibel presentation of a patient with partial androgen insensitivity syndrome
A
<ul><li>More ambiguous if partial</li><li>Micropenis/clitoromegaly</li><li>Bifid scrotum</li><li>Hypospadias</li><li>Diminished male characteristics</li></ul>
3
Q
Descirbe the symptoms of androgen insensitivity syndrome
A
<ul><li>can present in infancy with inguinal hernias containing testes</li><li>'primary amenorrhoea'-puberty</li><li>little or no axillary and pubic hair</li><li>undescended testes causing groin swellings</li><li>breast development may occur as a result of the conversion of testosterone to oestradiol</li><li>usually slightly taller than female average</li></ul>
4
Q
Describe the key symptoms of Kawasaki disease
A
High grade fever and CREAM:<br></br><ul><li>Conjunctivits (bilateral and non exudative)</li><li>Rash (non-bullous)</li><li>Edema/erythema of hands and feet</li><li>Adenopathy (cervical, commonly unilateral and non-tender)</li><li>Mucosal involvement (strawberry tongue, oral fissures etc)</li></ul><br></br>
5
Q
Describe the management of patients with Kawasaki disease
A
<ul><li>High dose aspiring</li><li>IVIG</li><li>Echos and close follow up</li></ul>
6
Q
Describe the rash typically seen in measles
A
<div><ul><li>Discrete maculopapular rash becoming blotchy and confluent</li><li>Desquamation that typically spares the palms and soles may occur after a week</li><li>Rash starts behind the ears then spreads to the whole body</li></ul></div>
7
Q
Describe the mangement of measles
A
<ul><li>Mainly supportive-antipyretics</li><li>Admission for immunossuprressed or pregnant patients</li><li>Inform public health->notifiable disease</li><li>Vitamin A to children under 2 years</li><li>Ribavirin may reduce duration of symptoms but not routinely recommended</li></ul>
8
Q
Describe the management of people who ocme into contact with patients with the measles
A
<ul><li>If no immunised: offer MMR-should be given within 72 hours</li></ul>
9
Q
At what age does chicken pox usually occur?
A
1-9 years
10
Q
Describe the rash associated with chicken pox?
A
<ul><li>Starts as raised red itchy spots on face/chest which then spreads to rest of body</li><li>Progresses into small, fluid filled blisters over a few days</li><li>Crusts over and heals, usually leaving no scars</li></ul>
11
Q
Describe the management of chicken pox
A
<ul><li>Trim nails to prevent scratching and infection</li><li>Enocurage loos clothing</li><li>Cooling measures like oatmeal baths and calamione lotion to reduce tiching</li><li>Analgesics and antipyretics for symptom relief</li><li>If immunocompromised: IV aciclovir and human varicella-zoster immunoglobulin (VZIG)</li></ul>
12
Q
Describe the epidemiology of rubella
A
<ul><li>Less common now due to widespread vaccination</li><li><br></br></li></ul>
13
Q
Describe the presentation of a patient with rubella
A
<ul><li>Fever: low grade</li><li>Coryza</li><li>Arthralgia</li><li>A rash that begins on the face and moves down to the trunk</li><li>Lymphadenopathy, especially post-auricular and suboccipital</li></ul>
14
Q
Describe the rash associated with rubella
A
<ul><li>Maculopapular rash that startso n the face before spreading to the whole body, usually fades by day 3-5</li></ul>
15
Q
<b>Describe the pathophysiology of diphtheria:<br></br></b><br></br><ul><li>Releases an {{c1::exotoxin}} encoded by a {{c2::Beta-prophage}}</li><li>Exotoxin inhibits {{c3::protein synthesis}} by catalyzing {{c4::ADP-ribolysation}} of {{c5::elongation factor EF-2}}</li></ul>
A
<ul><li>Releases an exotoxin encoded by a Beta-prophage</li><li>Exotoxin inhibits protein synthesis by catalyzing ADP-ribolysation</li></ul>
16
Q
Describe the presentation of a patient with staphylococcal scalded skin syndrome
A
<ul><li>Superficial fluid-filled blisters, often leading to erythroderma</li><li>Desquamation and positive Nikolsky sign</li><li>Perioral crusting or fissuring with oral muscoa unaffected</li><li>Skin has a 'scalded' look due to loss of superficial layers of epidermis</li><li>Fever and irritability common due to underlying infection</li></ul>
17
Q
Describe how a patient with meningitis might present?
A
<ul><li>Fever</li><li>Neck stiffness</li><li>Severe headache</li><li>Photophobia</li><li>Rash</li><li>Focal neurological deficits.signs of raised ICP</li></ul>
18
Q
Describe the management of meningitis
A
<ul><li><3 months: IV amoxicillin(or ampicillin) and IV cefotaxime</li><li>>3 months: IV cefotaxime (or ceftriaxone)</li><li>Dexamethasone if >3 months and bacterial</li><li>Fluids</li><li>Cerebral monitoring and supportive therapy</li><li>Public health notification and antibiotic prophylaxis</li></ul>
19
Q
Describe the epidemiology of Fifth disease
A
<ul><li>Common in late winter and early spring</li></ul>
20
Q
Describe the management of Fifth’s disease
A
<ul><li>Supportive: rest, hydration etc</li><li>Hsopitlisation for severe complications</li></ul>
21
Q
Describe the epidemiology of pneumonia in children
A
<ul><li>Highest incidence in infants</li><li>Young infants: usually viral</li><li>Older children: usually bacterial</li><li>Viral causes mroe common in the winter</li></ul>
22
Q
Describe the symptoms of pneumonia in children
A
<ul><li>Usually preceded by an URTI</li><li>Fever</li><li>Difficulty breathing</li><li>Lethargy</li><li>Poor feeding</li></ul>
23
Q
Describe the aetiology/risk factors of asthma
A
<ul><li>Genetics</li><li>Atopy(allergy, eczema)</li><li>Allergen exposure</li><li>Prematurity</li><li>Cold air</li><li>Low birth weight</li><li>Viral bronchiolitis early in life</li><li>Parental smoking</li></ul>
24
Q
Describe the pathophysiology of asthma
A
<ul><li>Bronchial inflammation-> oeadema and increased mucus production and infiltration with esoniphils, mast cells, neutrophils, lymphocytes->bronchial hyperresponsiveness->reversible aurflow obstruction</li></ul>
25
Describe the stepwise management of asthma in children over 5
- SABA PRN(salbutamol)
- ICS prophylaxis(beclomethasone)
- LTRA(montelukaust)
- Stop LTRA and add LABA(salmeterol)
- Switch ICS/LABA for ICSMART(formeterol and ICS)
- Add separate LABA
- High dose ICS(>400mcg) and referral
26
Descirbe the stepwise maangement of asthma in children<5 years
- SABA PRN(salbutamol)
- ICS prophylaxis(beclomethasone)-trial for 8 weeks then refer
- LTRA(montelukaust)
- Stop LTRA and add LABA(salmeterol)
- Switch ICS/LABA for ICSMART(formeterol and ICS)
- Add separate LABA
- High dose ICS(>400mcg) and referral
27
Describe the features of a SABA?
- Short acting B2 agonists
- Salbutamol/terbutaline
- Few side effects, effective for 2-4 hours
28
Describe the features of a LABA
- Long acting B2 agonists
- Salmeterol/formoterol
- 12 hours
- Can't be used without ICS
29
Describe the features of ipratropium bromide
- Anticholinergic bronchodilator
- Young infants if other bornchodilators uneffective
- Treatment for severe acute asthma
30
Describe the features of an ICS
- Inhaled corticosteroids
- Decrease airway inflammation->prophylaxis
- Systemic side effects: impaired growth, adrenal suppression, altered bone metabolism
31
Describe the features of severe acute asthma
- Too breathless to talk/feed
- Use of accessory neck muscles
- O2<92%
32
Describe the management of a severe acute asthma attack
- 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
33
Describe the management of a life threatening asthma attack
- 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
34
Describe the epidemiology of croup
- Children: 6 months-6 years
- Peak incidence aged 3
- Common in autumn
- Highly prevalent-> affects 1/6 children at least once in their life
35
Describe the presentation of a child with mild croup
- Occasional barking cough with no audible stridor
- No recession
- Child eating and drinking as normal
36
Describe the presentation of a patient with moderate croup
- Frequent barking cough
- Prominent stridor
- Marked sternal recession
- Agitated child
- Tachycardia
37
Describe the management of mild croup
- At home with simple analgesia, fluids, rest etc
- Single dose dexamethasone in primary care
- Minimise crying as this will worsen airway obstruction
38
Describe the management of moderate/severe croup
- Admission to hospital
- Monitoring: may ned ENT intervention
- Nebulised adrenaline for severe symptoms
- Minimise crying
39
Describe the presentation of a patient with bronchiolitis
- Sharp, dry cough
- Laboured breathing/wheezing
- Tachypnoea/tachychardia
- Subcostla.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
40
Describe the at home management of bronchiolities
- Supportive management-> fluids, simple analgesia etc
41
Describe the management of bronchiolitis in the hospital
- Oxygen through nasal cannula/fluids
- CPAP if respiratory failure
- Suctioning of secretions
- If severe: antiviral therapy(ribavarin)
42
Describe the pathophysiology of bronchiolitis obliterans
- 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
43
Aetiology of cystic fibrosis:
Mutations in {{c1::CFTR protein}} on Chromosome {{c1::7}}-> defects of {{c1::chloride transport}} across cell membranes-> {{c1::thick mucus secretions}} and {{c1::impaired ciliray functions.}}
Secretions block {{c1::pancreatic ducts}}-> enzyme deficiency and malabsorption
Mutations in {{c1::CFTR protein}} on Chromosome {{c1::7}}-> defects of {{c1::chloride transport}} across cell membranes-> {{c1::thick mucus secretions}} and {{c1::impaired ciliray functions.}}
Secretions block {{c1::pancreatic ducts}}-> enzyme deficiency and malabsorption
44
Describe the management of acute epiglottitis
- Immediate senrio involvement: ENT, anaesthetics
- Endotracheal intubation
- Culturing and examination of throat once airway secure
- Oxygen
- Nebulised adrenaline
- IV antibiotics: 3rd gen cephalosporin: IV cefotaxime/ceftriaxone
45
Describe the pathophysiology of viral induced wheeze
- Small airways->inflammation and oedema-> triggers smooth muscles of airway to constrict-> narrowing-> wheeze
- Restricted airway-> respiraotry distress
46
Describe the management of viral induced wheeze
- Same as acute asthma treatment
- SABA via spacer max 4 hourly up to 10 puffs
- LTRA and ICS via spacer
47
Describe the epidemiology of otitis media
- Common, espically in those <4 years
- Often occurs post viral URTI
48
Describe the pathophysiology of otitis media
- Secondary to oedema and narrowing of eustachian tube-> prevents middle ear from draining-> predisposing it to colonisation of bacteria
49
Describe the epidemiology of glue ear
- Peaks at 2 years of age
- Commonest cause of conductive hearing loss in children
50
Describe the management of glue ear
- 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
51
Describe the pathophysiology of strabismus
- 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
52
Describe the presentation of a patient with impetigo
- Erythematous macule that vesiculates or pustulates
- Superficial erosion with a characteristic golden crust
53
Describe the management of localised non-bullous impetigo
- Topical hydrogen peroxide 1%
- Fusidic acid or mupirocin
54
Describe the management of widespread non-bullous impetigo
- Topical fusidic acis/mupirocin OR antibioics for 5 days(flucloxacillin)
- Clarithromycin(allergic) or erythromycin(pregnancy) as alternative antibiotics
55
Describe the management of bullous impetigo
- Oral antibiotics or up to 7 days
- Flucloxacillin
- Clarithromycin or erythromycin as alternatives
56
Describe the aetiology of toxic shock syndrome
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.
57
Describe the presentation of a patient with toxic shoxk syndrome
- Early, non-specific flu like symptoms
- Rapid progression to high fever, widespread rash
- Multi-organ involvement -hypotension for cardiac depressiona nd ocnfusion for encephalopathy
58
Describe the management of toxic shock syndrome
- DRABCDE0aggressive fluid and electrolye resusciation
- Immediate cessation to persisting infection sources
- Antibiotics: clindamycin and cephalosporin
- Corticosteroids in some cases
59
Describe the epidemiology of scarlet fever
- Children aged 2-6 years
- Peak incidence: 4 years
60
Describe the rash associated with scarlet fever
- Red-pink blotchy macular rash with rough 'SANDPAPER' skin
- Starts on trunk and spreads outwards
61
Describe the presentation of a patient with scarlet fever
- Fever: 24-48 hours
- Malaise, headache, sore throat, n+v
- Strawberry tongue
- Rash
62
Describe the pathophysiology of an ASD
- Shunt from left to right
- Blood continues to flow to lungs so no cyanosis
- Increased blood flow to right side-> right side overload,r ight heart failure and pulmonary hypertension
- Over time pulmonary pressure>systemic pressure-> right to left shunt and cyanosis(Eisenmenger syndrome)
63
Describe the pathophysiology of coarctation of the aorta
- 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)
64
Describe the signs of coarctation of the aorta in infancy
- Tachypnoea and increased work of breathing
- Poor feeding
- Grey and floppy baby
65
Describe the signs of coarctation of the aorta in an older child
- Left ventricular heave due to left ventricular hypertrophy
- Underdeveloped left arm where there is reduced blood flow to the left subclavian arter
- Underdevelopment of the legs
- Adults: hypertension
66
A neonate is found to weak femoral pulses, how would you further investigate?
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
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
67
Describe the pahophysiology of a ventricular septal defect
- 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)
68
Describe the medical management of tet spells
- Oxygen
- Beta blockers
- IOV fluids
- Morphine
- Sodium bicarbonate
- Phenylehrine infusion
69
Define transposition of the great arterie
- Attachments of the aorta and pulmonary trunk to the heart are transposed
- RV pumps blood into the aorta
- LV pumps blood into pulmonary vessels
70
Describe the epidemiology of transposition of the great arteries
- M>F
- Maternal diabetes
71
Describe the pathophysiology of transposition of the great arteries
- 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
72
Describe the symptoms of Ebstein's anomaly
- Cyanosis
- SOB and tachypnoea
- Poor feeding
- Collapse
- Heart failure symptoms like oedema
73
Describe the pathophysiology of congenital aortic valve stenosis
- Aortic valve usually 3 leaflets, may have 1/2/3/4 leaflets isntead
74
Describe the symptoms of congenital aortic valve stenosis
Asymptomatic
Severe:
Severe:
- Fatigue
- SOB
- Dizziness
- Fainting
Symptoms worse one exertion
- May present with heart failure a few months after birth
75
Describe the symptoms of congenital pulmonary valve stenosis
Asymptomatic-picked up accidentally
- Fatigue on exertion
- SOB
- Dizziness
- Fainting
76
Describe the management of congenital pulmonary valve stenosis
- Mild-watch and wait-monitor
- Ballon valvoplasty via venous catheter to dilate valve
- Open heart surgery
77
Describe the epidemiology of noctunral enuresis
- M>F
- Roughly 2/3 will have a sstrong family history
- Children generally healthy
- Secondary type is associated with psychological stress
78
Describe the management of nocturnal enuresis
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)
79
Describe the aetiology of typical haemolytic uraemic syndrome
- Toxin induces damage to the endothelium of glomerular capillary bed causing thrombotic microangiopathy
80
Describe the aetiology of atypical haemolytic uraemic syndrome
- Familial-> dysregulation in complement cascade triggers atypical haemolytic uraemic syndrome
81
Describe the pathophysiology of haemolytic uraemic syndrome
- Endothelial injury-> microvascular thrombosis-> AKI+MAHA+thrombocytopenia
82
Describe the epidemiology of a UTI
- Higher prevalence in males until 3 months, then higher prevalence in females
83
Describe the symptoms of a UTI in infants <3 months
- Fever
- Vomiting
- Lethargy
- Irritability
- Poor feeding
- Failure to thrive
- Offensive urine
84
Describe the symptoms of a UTI in an infant aged between 3-12 months?
- Fever
- Poor feeding
- Abdo pain
- Vomiting
85
Describe the symptoms of a UTI in a child >1yr?
- Frequency
- Dysuria
- Abdo pain
- Haematuria
86
Describe some signs that would point towards an upper UTI
Fever>38 degrees
Loin pain and tenderness
Loin pain and tenderness
87
Describe the management of a UTI in a patient <3 months
- Immediate referral to a paediatrician
- ABX
88
Describe the management of a UTI in a child >3 months
- 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)
89
Describe the epidemiology of vesicoureteric reflux
- 1-3% of children
- Often familial predisposition
90
Describe the presentation of vesicoureteric reflux
- Recurrent/atypical UTI's
- Persistent bacteriuria
- Unexplained fevers, abdominal/flank pain
- If severe: renal scarring-> hypertension and CKD
91
Describe the conservative management of vesicoureteric reflux
- Prophylactic antibiotics to prevent UTIs
- Monitor kidney function and growth
- Treat constipation
92
Describe the surgial management of vesicoureteric reflux
- Ureteral reimplantation
93
Describe the epidemiology of Wilms' tumour
- Children <5 years
- Incidence peaks 3-4 years
94
Describe the management of Wilms' tumour
- Urgent review(within 48 hours)
- Nephrectomy
- Chemotherapy
- Radiotherapy if advanced
95
Describe the pathophysiology of cryptorchidism
- Incomplete migration of testis during embryogenesis from original retroperitoneal position near kidneys to final position in scrotum
96
Describe the management of bilateral undescended at birth testicles
- 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
97
Describe the management of unilateral cryptorchidism at birth
- Review at 6-8 weeks
- Then review at 3 months
- Then review at 5 months
- Refer by 6 months
- Orchidopexy at 6-18 months
98
Describe the epidemiology of hypospadias
- 3/1000
- Genetic element
99
Describe the features of hypospadias
- Ventral urethral meatus
- Hooded prepuce
- Chrodee(ventral curve of penis) in severe cases
- Urethral meatusmay open more proximally in severe variants
100
Describe the amangement of hypospadias
- Refer to specialist
- If very distal, may not need treatment
- Corrective surgery at around 12 months-DO NOT CIRCUMCISE
101
Describe the aetiology of phimosis
- STI's
- Eczema
- Psoriasis
- Lichen planus/lichen sclerosis
- Balanitis
102
Describe the presentation of a patient with phimosis/paraphimosis
- Non-retractable foreskin-> may interfere with urination/sexual function
- Paraphimosis-> swollen and painful glanss, tight band of foreskin-> ischaemia-> discolouration and severe pain
103
Describe the management of phimosis
- Wait and see
- Topical corticosteroids
- Stretching exercises
- Personal hygiene
104
Describe the management of paraphimosis
- Manual pressure
- Osmotic agents
- Puncture techniques
- Surgical reduction and circumcision
- Personal hygiene advice
105
Describe the pathophysiology of nephrotic syndrome
- Damage to glomerular basement membrane and podocytes results in increaed permeability to protein
- Lower plasma oncotic pressure-> hypoalbuminaemia and oedema
106
Describe the management for nephrotic syndrome
- High dose steroids, taper over time
- Diuretics for oedema
- Low salt diet
107
Describe a typical presentation of nephrotic syndrome in a child
- Well child, insidious onset of pitting oedema, initially periorbital then generalised
- History of recent URTI
- Cna progress to anorexia, GI changes, ascites, oliguria, SOB
- Risk of infection/thrombosis
108
Descirbe the aetiology of minimal change disease
- Idiopathic in most cases
- Often seen post viral URTI
- Drugs: NSAID's, rifampicin
- Hodgkin's lymphoma
- Infectious mononucleosis
109
Describe the management of minimal change disease
- Oral corticosteroids-> prednisolone, tapering regime
- If poor response: immunosuprpesives like ciclosporin/cyclophosphamide
- Fluids restriction and lower salt intake
- If high fluid overload: furosemide
110
Describe the prognosis of minimal change disease
- 1/3 resolve completely with no other episodes
- 1/3 have further relapses requiring further steroids
- 1/3 dependent on steroid therapy
111
Describe the pathophysiology of IgA nephropathy
- 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
112
Describe the management of IgA nephropathy
- 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
- IAcitve disease: Falling GFR/no reponse to ACE inhibitors: immunosuppression with corticosteroids
113
Describe the prognosis of IgA nephropathy
- 30% progress to end stage renal failure
114
Describe the presentation of post strep glomerulonephritis
- Haematuria(visible-ribena/coke), oliguria, hypertension +/-oedema 1-3 weeks post infection(s.pyogenes)
- Some may be asymptomatic
115
Describe the maangement of post strep glomerulonephritis
- Usually self resolving
- Handle AKI
116
Describe some symptoms of hypogonadism
- Lethargy
- Weakness
- Weight gain
- Loss of libido
- Erectile dysfunction
- Gynaecomastia
- Depression
117
Describe the management of hypogonadism
- Hormone replacement therapy: usualy testosterone injections/oral
- Monitoring therapy to check for polycythaemia, changes in bine ineral density, prostate status and LFTs
118
Describe the symptoms of Klinefelter syndrome
- 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
119
Describe the prognosis of Klinefelter syndrome
- Close to normal
120
Describe the epidemiology of Turner's syndrome
- Roughly 1/2500
- Incidence DOES NOT increase with maternal age
- Low risk of recurrence
121
Describe the clinical features of Turner's syndrome
- Short stature
- Shield chest, widely spaced nipples
- Webbed neck
- High arching palate
- Wide carrying angle/cubitus valgus
- Delated/incomplete puberty
- Primary amenorrhoea
- Bicuspid aortic valve, coarctation of the aorta
- Infertilitiy
122
Describe the management of Turner's syndrome
- Human growth hormone: during childhood to increase height
- Oestrogen replacement therapy: allow development of secondary sex characteristics, prevent osteoporosis
- Medical care to manage associated problems, including fertility treatment
123
Describe the epidemiology of Down's syndrome
- Common
- Incidence increases with increasing maternal age, especially if resulting from gamete non-disjunction
124
Describe the facial features of Down's syndrome
- 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
125
Describe the management of Down's syndrome
- MDT approach
- OT, SALT, Physio, dietitiacn, paeds, GP
- ENT and audiologist for ear problems
- Cardiologists for congenital heart disease
- Opticians for glasses
126
Describe the inheritance of Fragile X
- X-linked
- Males always effects, females may or may not be(have spare copy of FMR1 gene on other X chromosome)
127
Descirbe the features of Noonan syndrome
- Webbed neck
- Pectus excavatum
- Short stature
- Pulmonary stenosis
128
Describe the features of Pierre-Robin syndrome?
- Micrognathia
- Posterior displacement of the tongue->upper airway obstruction
- Cleft palate
129
Describe the features of Prader Willi syndrome
- Hypotonia
- Hypogonadism
- Obesity
- Short stature
- Dysmorphic features
Typical history: feeding is a challenge initially due to hypotonia, then becomes hyperphagia
130
Describe the features of William's syndrome
- 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
131
Describe the features of a patient with Duchenne muscular dystrophy
- 3-5 yrs present with progressive proximal muscle weakness
- Calf pseudohypertrophy
- Gower's sign
- 30% also have intellectual impariment
132
Describe the prognosis of Duchenne muscular dystrophy?
- Most can't walk by age 12 years
- Uusally survive until 25-30 years
- Associated with dilated cardiomyopathy
133
Describe the features of myotonic dystrophy?
- Progressive muscle weakness
- Proloonged muscle contraction: patient can't let go after shaking someones hand, or release grip on a doorknob
- Cataracts
- Cardiac arrhythmias
134
Describe the features of Angelman syndrome
- Fascination with water
- Happy demeanour
- Widely spaced teeth
- Also learnign difficulties, ataxia, hand flapping, ADHD, dysmorphic features, epilepsy etc
135
Describe the management of Angelman syndrome
- No cure, MDT holistic care appproach
- Physio and OT
- CAMHS
- Parental education
- Educational and social services support
- Anti-epileptic medication if needed
136
Describe the management of prader willi syndrome?
- Growth hormone
- Dietary management to prevent obesity
- PT and exercise problems
- Educational interventions to support cognitive development
137
Describe the features of Noonan syndrome
- 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
138
Describe the management of William's syndorme?
- MDT approach
- Echos and BP monitoring for cardiac complications: aortic stenosis and hypertension
- Low calcium diet and avoid calcium and vitamin D supplements: hypercalcaemia
139
Describe the inheritance pattern of osteogenesis imperfecta?
- Autosomal dominant
140
Describe the aetiology of osteogenesis imperfecta
- 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
141
Describe the features of a patient with osteogenesis imperfecta
- Presents in childhood
- Fractures following minor trauma
- Blue clera
- Deafness secondary to oseoosclerosis
- Denatal imperfections
- Bone deformities like bowed legs and scoliosis
- Ligament laxity leading to joint hypermobility
142
Describe the management of osteogenesis imperfecta
- Orthopaedic interventions: treat fractures and bone deformities
- Medical management: bisphosphoonates to increase bone density
- Physio, dental care, hearing aids, education and counselling
143
Describe the epidemiology of rickets
- Most common cause: vitamin D deficiency for a long time
- Can be caused by: poor nutrition, insufficiency sun exposure and malapbsorption syndormes
144
Describe the features of rickets
- 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
145
Describe the mangement of rickets
- Oral vitamin D: 400IU/dayfor chidlren and young people, 6000IU for 8-12 weeks in children <12 years
- Calcium and phosphorus supplements may be adivsed
- Prevention: breastfeeding babies have formula fortified with vitamin D, breastfeeding woman and children should all take vitamin D supplement
146
Describe the epidemiology of transient synovitis?
- 3-11 years
- 2x as common in males
147
Describe the aetiology of transient synovitis
- After URTI 1-2 weeks prior
148
Describe the presentation of a patient with transient synovitis
- Acute onset of limp, often with an avoidance of weight bearing
- Pain in hip or referred knee pain
- Mild to absent fever
149
Describe the management of transient synovitis
- 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
150
Describe the presentation of osteomyelitis
- Fever
- Pain at rest, worse when weight bearing
- Swelling
- Erythema of the affected site
- If chronic: can have hisotry of pain, soft tissue damage etc
151
Describe the management of osteomyelitis
- 6 weeks flucloxacillin
- Clindnamycin for pencillin allergy
- Vancomycin if MRSA
- Surgical debriedement may be needed
152
Describe the symptoms of septic arthritis
- Acute onset of tender swollen joint
- Reduced range of movement
- Systemic symptoms: fever, malaise, chills
153
Describe the management of septic arthritis
- Epirical IV abx for 4-6 weeks total as IV first then oral
- Flucloxacillin 1st line
- Clindamycin if penicillin allergy
- Vancomycin if MRSA
154
Describe the epidemiology of Perthes' disease
- Predominantly males
- Aged 4-8 years
155
Describe the aetiology of Perthes' disease
- 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 ot the vessels
156
Describe the presentation of a patient with Perthes' disease
- 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
157
Describe the diagnosis of Perthes' disease
- x-ray: -can be normal, may show sclerosis and fragmentation of epiphysis
- Blood tests normal
- MRI and tehnetium bone scan may be done
158
Describe the management of Perthes' disease
- 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
159
Describe the prognosis of Perthes' disease
- Most resolve with conservative management
160
Describe the Catterall staging for Perthes' disease
- Clinical and histological features only
- Sclerosis with/without cystic changes and preservatoin of the articular surface
- Loss of structura integrity of the femoral head
- Loss of acetabular integrity
161
Describe the epidemiology of Slipped Upper Femoral Epiphysis
- Increasing with growing rates of childhood obesity
162
Describe the presentation of a patient with slipped upper femoral epiphysis
- 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
163
Describe the management of slipped upper femoral epiphysis
- Surgical: internal fixation-cannulated screw
- Prompt treatment important to prevent avascular necrosis of the femoral head
164
Describe the epidemiology of osgood schlatter disease
- Adolescents ages 10-15
- M>F
- Hihger prevalence in athletes and sports such as gymnastics and basketball
165
Describe the aetiology of osgood schlatter disease
- 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
166
Describe the presentation of a patient with osgood schlatter disease
- Anterior knee pain, often localised to tibial tubercle
- Pain exacerbated by running, jumping, kneeling relieved by rest
167
Describe the prognosis of osgood schlatter
- Resolves over time
- Patient often left with a bony lump on their knee
- Rarely avulsion fracture
168
Aside from positive Barlow and ortolani tests what should be checked on examination of a patient with developmental dysplasia of the hip?
- Leg length symmetry
- Level of knees when hips and knees are bilaterally felxed
- Restricted abduction of the hip in flexion
169
Describe the management of developmental dysplasia of the hips
- Can self-resolve in 3-6 weeks
- Pavlik harness: keeps hips in flexed and abducted position
- If severe: surgical intervention
170
Describe the epidemiology of juvenile idiopathic arthritis
- Most common cause of chronic joint pain in children
171
Describe the features of Still's diseasd (systemic juvenile idiopathic arthritis0
- Slamon pink rash
- Fevers
- Lymphadenopathy
- Weight loss
- Joint pain and inflammation-swelling, stiffness, llimited ROM
- Splenomegaly
- Muscle pain
- Pleuritis/pericarditis
172
Describe the presentation of a patient with polyarticular JIA
- 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
173
Describe the epidemiology of oligoarticular JIA
- Girls <6 yrs most commonly
174
Describe the presentation of a patient with oligoarticular JIA
- Monoarthritis-pain, stiffness, swelling etc
- Anterior uveitis-> refer to ophthalmology
- Usually no systemic symptoms
175
Describe the presentation of enthesitis related JIA
- Enthesitis
- Anterior uveitis-> refer to opthalmology
- Check for symptoms of psoriatic arthritis
- IBD symptoms
176
Describe the signs and symptoms of juvenile psoriatic arthritis
- Psoriatic arthrtiis
- Nail pitting
- Onycholysis
- Dactylitis
- Enthesitis
177
Describe the management of juvenile idiopathic arthritis
- 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
178
Describe the aetiology of torticollis
- Unclear
- Often thought to be related to posture or hevay carrying loads
179
Describe the management of torticollis
- 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
180
Describe the epidemiology of adolescent idiopathic scoliosis
- 10-18 years
181
Describe the signs and symptoms of a patient with scoliosis
- Postural asymmetry
- Absent or minimal pain
- No neurological symptoms
- Paraspinal prominences on forward bending
- Shoulder asymmetry
- Waist line asymmetry
182
Describe the management of scoliosis
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
183
Describe the aetiology of discoid meniscus
- Developmental anomaly before birth
184
Describe the presentation of a patient with a discoid meniscus
- Visible or audible palpable snap on terminal extension(10-20 degrees) along with pain or swelling and locking
- Click during movement
185
Describe the management of discoid meniscus
- Physio
- If severe: arthroscopic partial meniscectomy
186
Describe the pathophysiology of leukaemia
- 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 rpoduction can suppress other cell lines-> pancytopenia
- Pancytopenia: Anemia, leukopenia, thrombocytopenia
187
Describe the presentation of a patient with leukaemia
- Anaemia
- Neutropenia: high WCC but low neutrophil levels
- Frequent infections
- Thrombocytopenia resulting in bleeding
- Hepatosplenomegaly
- Bone pain
- May have DIC or thrombocytopenia-petechiae
188
Descire the management of tumour lysis syndrome
Good hydration and urine output before chemo
Allopurinol or rasburicase to suppress uric acid levels
Allopurinol or rasburicase to suppress uric acid levels
189
Describe the management of CML
- Tyrosine kinase inhibitors(associated with BCR-ABL defect): imatinib
- Hydroxyurea
- Interferon alpha
- Allogenic bone marrow transplant
190
Describe the management of ALL
- Combinatino chemo
- CNS prophylactic agens
- Maintainence therapy
191
Describe the ppresentation of a patient with CLL
- Often asymptomatic-incidental lymphocytosis
- Infections, bleeding, weight loss, anaemia-warm autoimmune haemolytic anaemia
- Non tender symmetrical lymphadenopathy
192
Describe the prognosis of CLLL
Variable
- 1/3 don't progress
- 1/3 progress slowly
- 1/3 progress actively
193
Describe the epidemiology of paediatric brain tumours
- Leading cause of cancer related deaths in children
- Most common solid organ malignancy in paediatric population
194
Describe the presentation of a child with a brain tumour
- Persisten headaches which are worse in the morning
- Signs of raised ICP: nausea, vomiting and reduced consciousness
- Aeizure in an older child with no fever and no hisotry of seizures
- Focal neurological deficits
195
Describe the presentation of a patient with a neurblastoma
- Abdominal mass
- Pallor, weight loss
- Bone pain, limp
- Hepatomegaly
- Paraplegia
- Proptosis
196
Describe the epidemiology of pyloric stenosis
- 1-3/1000 live births
- 6-8 weeks
- M>F
- First-borns most commonly
197
Describe the aetiology of pyloric stenosis
- Genetics
- Prematurity
198
Describe the presentation of a patient with pyloric stenosis
- 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
199
Describe the management of pyloric stenosis
- Supportive:nil by mouth and IV fluids
- Surgical: Ramstedt pyloromyotomy(cuts hypertrophic sphincter and widens gastric outlet)
200
Describe the epidemiology of mesenteric adenitis
- More prevalent in children and adolescents
- Linkw ith viral pathogens-follows rep URTI
201
Describe the aetiology of mesenteric adenitis
- Viral: EBV, adeno, entero
- Bacteria: Yersinia, camylobacter
- Other: mycobacterium, salmonella, strep
202
Describe the presentation of a patient with mesenteric adenitis
- Diffuse abdominal pain: often mistaken for appendicitis
- Low grade fever
- Generalized abdominal tenderness
- Pharyngitis.sore throat
- Children: usually good overall health and unaltered appetitie
203
Describe the presentation of a patient with intussusception
- 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
204
Describe the management of intussusception
- If stable: rectal air insufflation or contrast enema
- Surgery
205
Describe the epidemiology of intestinal malrotation
- Consdered rare-critical
- Symptomatic malrotation most commonly presents in neonates
206
Describe the aetiology of intestinal malrotation
- 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
207
Describe the features of GORD in children
- 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
208
Describe the management of GORD in children
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)
- Alginate therapy(Gavison mixed with feeds)-NOT for use at same time as thickened feed
- PPI(e.g. omeprazole) only in certain situations
209
Describe the epidemiology of appendicitis in children
- Uncommon under 3 years
- One of the most common acute surgical problems in children
- Commonin populations with a western diet
210
Describe the aetiology of appendicitis
- 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 ni appendix's arterial supply, specifically ileocolic artery
211
Describe the symptoms of a patient with appendicitis
- Central abdominal pain radiating to the right iliac fossa
- LLow grade pyrexia
- Minimal vomiting, nausea
212
Describe the management of a patient with appendicitis
- 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
213
Describe the epidemiology of biliary atresia
- F>M
- Neonates only: perinatal(1-2 weeks of life) or postnatal(208 weeks)
214
Describe the pathophysiology of biliary atresia
- Either obliteration or discontinuity within the extrahepatic biliary system resulting in obstruction of bile flow
- Results in neonatal presentation of cholestasis
215
Describe the presentation of a patient with biliary atresia
- First 2 weeks of life
- Jaundice beyond physiological 2 weeks
- Dark urine and pale stools
- FTT
216
Describe the management of biliary atresia
- SSurgical: Kasai procedure-hepatoportenterostomy
- Post surgery: abx and bile acid enhancers
217
Describe the prognosis of biliary atresia
- Good if surgery
- Liver transplant in 1st 2 years of life if failure
218
Describe the epidemiology of febrile convulsions
- Common: 3% of children
- Children 6 months and 5 yrs
219
Describe the aetiology of febrile convulsions
- Abrupt rise in body temperature often related oto an indection
- Can be triggered by bacterial and viral infections
- Mc: URTI, ear infections and childhood exanthems
220
Describe the symptoms of a febrile convulsion
- High fever: >38 degrees
- Tonic-clonic , LOC
- Post ictal drowsiness/confusion
221
Define constipation in children
- <= 3 stools/week or significant difficulty in passing stools
222
At what age is encopresis considered pathological?
- >4 years
223
Describe the management of constipation in children
- 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
224
Describe the epidemiology of cerebral palsy
- Mc cause of major motor impairment
- Higher in areas with worse ante/perinatal care
- Higher in premature infants and those of multiple pregnancies
225
Describe the features of spastic cerebral palsy
- Increased tone and reflexes(flexed hip and elbow, 'clasp-knife' spasticity
- 'Scissor' gait
- Can be monoplegic, diplegic or hemiplegic
226
Describe the features of dyskinetic cerebral palsy
- Athetoid movements and oro-motor problems
- Can exhibit signs of parkinsonism
227
Describe the features of ataxic cerebral palsy
- Typical cerebellar signs
- Uncoordinated movements
228
Describe the management of cerebral palsy
- 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
229
Describe the prognosis of cerebral palsy
- Varibale impact on QOL-difficulties with mobility and communication
- Associated with reduced life expectancy
230
Describe the epidemiology of haemolytic disease of the newborn
- Rare but serious
- Most common in pregnancy where there is blood group incompatibility beetween the mother and fetus
231
Describe the aetiology of haemolytic disease of the newborn
- 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
232
Describe the features of haemolytic disease of the newborn
- 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
233
Describe the features of a cephalohaematome
- Commonly affects parietal region
- Doesn't cross suture lines
- May take up to 3 months to resolve-managed conservatively
234
Describe the features of caput seccedaneum
- Soft, puffy swelling due to localised oedema
- Crossess suture lines
- Resolves within days-no tx needed
235
Describe the interpretation of the APGAR score
- 0-3: very low
- 4-6: moderate low
- 7-10: good health
236
Describe the key points of paediatric BLS
- Unresponsie-> shout for help
- Open airway
- Look, listen, feel for breathing
- 5 rescue breaths
- Check for signs of circulation
- 15 chest compressions: 2 rescue breaths
237
Describe the pathophysiology of acute respiratory distress syndrome
- 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
238
Describe the clinical features of acute respiratory distress syndrome
- 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
239
Describe the management of acute respiratory distress syndrome
- Generally ITU
- Intubation/ventilation to treat hypoxaemia
- Hameodynamic support: aim for MAP>60mmHG, tvasopressors, transfusions if Hb<70
- Enteral nutrition support
- DVT prophylaxis
- Treat underlying cause
- PPI to prevent gastric ulcers
240
Describe the epidemiology of neonatal respiratory distress syndrome
- Premature infants
241
Describe the symptoms of neonatal respiratory distress syndrome
- 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
242
Describe the management of neonatal respiratory distress syndrome
- Intratracheal instillation of artificial surfactant
- Supplemental oxygen/respiratory support: CPAP or mechanical ventilation
- Caffeine
- Supportive care: maintain body temperature, nutrition, amange other complications
243
Describe the causes of late onset neonatal sepsis
- S epidermis
- S. aureus
- P.aeruginosa
- Klebsiella
- Enterobacter
- Pseudomonas
- E.Coli
244
Describe the management of neonatal sepsis
- 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
245
Describe the aetiology of transient tachypnoea of the newborn
- C-ssection-> passage through birth canal applies external pressure on thorax, aiding in expelling the birth.
- Suboptimal epithelial clearance mechanisms
246
Describe the presentaiton of transient tachypnoea of the newborn
Resp distress:
- Tachypnoea(>60bpm)
- Increased work of breathing
- Potential desaturation/cyanosis
247
Describe the management of transient tachypnoea of the newborn
- usually self-resolving within 3 days of life
- Oxygen to manage hypoxaemia
- Monitor for progression to penumonia or NRDS
248
Describe the aetiology of meconium aspiration syndrome
- Fetal distress/hypoxia-> intestinal relaxation+anal sphincter relaxation
249
Describe the signs and symptoms of meconium aspiration syndrome
- 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
250
Describe the management of meconium aspiration syndrome
- Gentle suctioning of mouth/nose to remove any visible residual meconium
- Abx to reduce infection
- Transfer baby to ICU if eeded for careful monitoring and oxygen administration
- If severe: artificial ventilation might be needed
251
Describe the clinical features of neonatal hypoglyacaemia
- Asx
- Autonomic: jitteriness, irritable, tachypnoea, pallor
- Neuroglycopenic: poor feeding/sucking, weak cry, drowsy, hypotonia, seizures
- Others: apnoea, hypothermia
252
Describe the management of asymptomatic neonatal hypoglycaemia
- Encourage normal feeding(breast/bottle)
- Monitor blood glucose
253
Describe the management of symptomatic/severe neonatal hypoglycaemia
- Admit to neonatal uit
- IV infusion of 10% dextrose
254
Describe the management of gastroshisis
- 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 infefction, IV fluids/nutrients
255
Describe the pathophysiology of gastroschisis
- Week 4 of gestation: lateral folds fail t fuse-> hole in abdominal wall-> organs protrude
256
Describe the pathophysiology of exomphalos
- 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
257
Describe the management of exomphalos
- C-section-> reduce risk of sac rupture
- Staged surgical repair
258
Describe the pathophysiology of duodenal atresia
- Failure in duodenal vacuolization
- During fetal development duodenal epithelium proliferates rapidly-> complete duodenal obstruction-> apoptosis of excess cells-> formation of small vacuoles whcih fuse-> re-establish duodenal passageway
259
Describe the aetiologuy of oesophageal atresia and tracheo-oesophageal fistula
Associated with:
- VACTER syndrome
- CHARGE
- Chromosomal abnormalities
- DiGeorge syndorme
- Neural tube defects
260
Describe the management of oesophageal atresia
- Surgical: connect parts of oesophagus and close off fistula
- Post op: monitor for complicaitons
- Manage nutritional and respiratory support
261
Describe the epidemiology of necrotising enterocolitis
- First 3 weeks of life in premature neonates
- Fatal in 1/5, significant morbidity
262
Describe the signs and symptoms of necrotising enterocilitis
- Vomiting(may be bile streaked)
- Feed intolerance
- Bloody, loose stools
- Abdo distention
- Absent bowel sounds
- Systemic compromise-> acidodis on blood gas, resp distress
263
Describe the management of necrotising enterocilitis
- Nil by mouth
- NG tube for gastric decompression
- Broad spectrum abs
- Supprotive: IV fluids and ventilation
- Surgical: resecitn of necrotic sections of bowell
264
Describe the pathophysiology of congenital diaphragmatic hernia
- Usually a failure of the pleuroperitoneal cavity to close completely
265
Describe the presentation of congenital diaphragmatic hernia
- Cyanosis soon after birth
- Tachypnoea and tachycardia
- Asymmetry of chest wall
- Absent breath sounds on onse side-usually left with heart shifted to right
- Bowel sounds audible over chest wall
266
Describe the management of congenital diaphragmatic hernia
- 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 nd impari lunmg funciton
- Oro-gastric tube
- Surfactant
- Open surgical repair of diaphragm when stable
267
Describe the management of neonatal jaundice
- Admit urgently if: <24 hours, >7days and unwell, premature
- Might not need any treatment if well and liekly physiological
- Increase fluid intake
- Monitor bilirubin levels
- Treat underlying cause
- Phototherapy
- Exchange transfusion
268
Describe the signs ans symptoms of neonatal jaundice
- Yellowing of skin and eyes
- Poor feeding
- Lethargy
- Severe: kernicterus
- If due to hepatitis/biliary atresia: dark stools and pale urine
269
Describe the symptoms of toxoplasma gondii infeciton in pregnancy and neonates
- Causes toxoplasmosis
- Mother: Fever, fatigue
- Fetus: chorioretinitis, hydrocephalus, rash, intracranial calcifications
270
Describe the symptoms of rubella in pregnancy and neonates
- Mother: lymohadenopathy, polyarthritis, rashes
- Fetus: congenital rubella syndrome: deafness, cataracts, rash, heart defects
271
Describe the symptoms of CMV infection in pregnancy and neonates
- Mother: Mild sx
- INfants: rashes, deafness, chorioretinitis, seizures, microcephaly, intracranial calcifications
272
Describe the symptoms of a HSV infection in neonates
- Blisters and inflammation of the brain: meningoencephalitis
273
Describe the epidemiology of listeria
- Found in many food products, especially unpasteurised dairy products and soft cheeses
- Vertical transmission from mother to fetus through placenta or during delivery
274
Describe the signs and symptoms of listeriosis in neonates
- Neonatal sepsis
- Meningitis
- Respiratory distress due to aspiration of infected amniotic fluid
- Chorioamnionitis
- Premature labour
- Stillbirth
275
Describe the management of listeriosis
- Abx: ampicillin + aminoglycoside(gentamicin)
276
Describe the epidemiology of cleft lip/palate
- Mc congenital deformity affecting orofacial structures
277
Describe the pathophysiology of cleft lip/palate
- Polygenic inheritance
- Failure of frontal-nasal and maxillary processes to fuse: cleft lip
- Failure of palatine processes and nasal septum to fuse: cleft palate
278
Describe the management of cleft lip/palate
- Cleft lip repaired earlier than cleft palate: 1st week-3 months
- Cleft palate: 6-12 months
279
Describe the local features of HSV in a neonate
- Vesicular lesions on the skin
- Eye involvement
- Oral mucose involvement without internal organ involvement
280
Describe the management of HSV in neonates and pregnancy
- 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
281
Describe the features of bronchopulmonary dysplasia
- Low oxygen saturations
- Increased work of breathing
- Poor feading and weight gain
- Crackles and wheeze in chest on auscultation
- Increased susceptibility to infection
282
Describe the management of bronchopulmonary dysplasia
- Typically leave neonatal unit on low dose O2 at home
- Followed up after 1 yr to wean off
- RSV protection-> monthyl injecitons of palivizumab for certain babies
283
Describe the clinical features of typical(petit mal) absence seizures
- Onset: 4-8 years, often doesn't persist into adulthood
- Duration few-30 secs, no warning, quick recovery, often lots in one day
284
Describe the clinical features of West syndrome/infantile spasms
- 4-8 months
- Flexion of head, trunks, limb-> extension of arms(Salaam atack), lasts 1-2 secs, repeat up to 50 times
- clusters-'jack-knife'spasms)
- Progressive mental handicap-> associated with regression and high morbidity
285
Describe the prognosis of West's syndrome
- Poor prognosis-> intellectual disability
- Many develop Lennox-Gastaut syndrome later one
286
Describe the clinical features of Dravet's syndrome
- Convulsive status epilepticus seizures during intercurrent illness or following vaccination
- Refractory to antiepileptic treatment
- Associated with loss of developmental milestones and ASD
287
Describe the prognosis of Dravet's syndrome
- Estimated 15% mortality by age 20yrs
288
Describe the features of Lennox Gastaut syndrome
- May be an extension of infantile spasms
- Onset 1-5 yrs
- Tonic, atonic and atypical absence(last longer and have gradual onset) seizures
- Idopathic: normal psychomotor developmnt
- Symptomatic: associatede nuerological abnormalities
289
Describe the management of Lennox Gastaut syndrome
- Ketogenic diet
- Often refractory to AED's
- Sodium valproate, lamotrigine, clobazam
- Surgical: corpus callostomy and vagus nerve stimulation
290
Describe the features of juvenile myoclonic epilepsy
- 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
291
Describe the management of juvenile myoclonic epilepsy
- Usually good response to AED's: sodium valproate and lamotrigine
292
Describe the features of panayiotopoulos syndrome
- Autonomic seizures with ictal vomiting, pupil dilation and syncope lasing up to 30 minutes
- Vision changes: flasing lights, blurring, loss of vision
- 3-6yrs onset
- Typicaly stop after 2-3 years, otherwise normal development
293
Describe the features of bening rolandic epilepsy
- 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
294
Describe the general management of childhood epilepsy
- If reversible, treat cause
- AED's
- Ketogenic/low glycaemic diet
- Surgery if refractory/caused by tumours
- MDT
- Emergency seizure plans for home and school, educate parents
295
Describe the investigations/managment for developmental delay
- Clinical and neuro exam
- Genetics, metabolic screen, MRI/CT, hearing/vision assessments
- Refer to specialist
- Early intervention services: SALT, OT, Physio, educational support etc
296
At what age is it concerning for a child to not be sitting without support?
12 months
297
At what age is it concerning for a child to not be walking unsupported?
- 18 months
298
Describe fine motor and vision milestones with regards to brick buillding
- 15 months: tower of2
- 18 months: 3
- 2 years: 6
- 3 years:9
Adds a brick every 3 months
299
Describe fine motor and vision milestones with regards to drawing
- 18 months: circular scribble
- 2 years: vertical line
- 3 yrs: circle
- 4 yrs: cross
- 5 yrs: square and triangle
300
Describe the major soical behavious and play milestones
- 6 weeks: smile
- 3mths: laughs
- 6mths: not shy
- 9 mths: shy, takes everything to mouth
301
Describe the major social behaviour and play milestones with regards to feeding
- 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
302
Describe the major social behaviour and play milestones with regards to dressing
- 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
303
Describe the major social behaviour and play milestones with regards to play
- 9mths: 'peek-a-boo'
- 12 mths: waves bye bye, plays pat a cake
- 18 mthd: plays alone
- 2 yrs: plays near others
- 4 yrs: plays with others
304
Describe the aetiology of retinoblastoma
- Mutations in tumour suppressor gene RB1
- Hereditary: germline mutations
- Non-hereditary: somatic
305
Describe the signs and symptoms of retinoblastoma
- Leukoria(white pupil when you shine a light)
- Strabismus
- Ocular inflammation and redness
- Deteriorating vision
- Failure to thrive
- Eye enlargement: developing countries
306
Describe the management of retinoblastoma
- Systemic chemo 1st line
- Enucleation(remove eye-extensive disease with threat of extraocular spread)
- Orbital exenteration
- Radio
- Genetic counselling
307
Describe the epidemiology of neuroblastoma
- Mc malignancy in children
- <5yrs
308
Describe the pathophysiology of neuroblastoma
- Comprises neural crest cells-> differentiate to form the sympathetic chain and adrenal glands in lumbar areas.
- Often starts in abdomen and spreads to bones, liver, skin(haematogenous and lymphatic spread)
- Catecholamine secreting tumour
309
Describe the clinical features of neroblastoma
- 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
310
Describe the NICE referral pathway for suspected neuroblastoma
- Very urgent referrla(<48 hours) in children with palpable abdominal mass or unexplained enlarged abdominal organ OR
- Unexplained haematuria
311
Describe the management of neuroblastoma
- Surgery followed by chemo
- Radiation to primary site
- Isotretinoin-> mainatinence therapy-promotes differentiation of neuroblastoma cells into normal cells
312
Describe the signs and sympotms of hepatoblastoma
- Abdominal mass
- Poor appetitie
- Weight loss
- Lethargy
- Fever
- Vomiting
- Jaundice
313
Describe the management of hepatoblastoma
- Chemo
- Surgery
314
Describe the epidemiology of osteosarcoma
- Mc primary malignany bone tumour in children and adolescents
- Slight M:F predominance
- Incidence peaks in adolesnces-growth spurts
315
Describe the signs and symptoms of osteosarcoma
- 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
316
Describe the management of osteosarcoma
- Surgical resection and limb salvage surgery
- Radiotherapy
- Chemo: multi-agent: methotrexate
- Follow up imagina
317
Describe the epidemiology of Ewing's sarcoma
- 2nd most prevalent bone cancer in children and adolescents
318
Describe the signs and symptoms of Ewing's sarcoma
- Nocturnal bone pain
- Palpable mass/swelling
- Restricted joint mobility
- Systemic: fever, weigh tloss, fatigue
319
Describe the management of Ewing's sarcoma
- Chemo 1st line
- Surgery
- Radiotherpay
320
Describe the epidemiology of Hodgkin's lymphoma
- Bimodial distribution: mc in 3rd and 7th decades
321
Describe the features of Hodgkin's lymphoma
- Non-tender lymphadenopathy, asymmetrical
- May be paingul after drinking-characteristic
- B symptoms + pruritus
- Hepato/splenomegaly
322
Describe the lugano classification for Hodgkin's lymphoma
- Stage 1: single lymohatic site
- Stage 2: >=2 lymph nodes on same side as diaphragm
- Satge 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 sx
S: Splenic involvement
E: extranodal contiguous extension
323
Describe the management of Hodgkin's lymphoma
- Chemoradiotherapy
324
Describe the features of a medulloblastoma including sx
- Mc malignant brain turmour in children
- Typically arises in cerebellum
- Sx: headaches, vomiting, ataxia and cranial nerve deficits
325
Describe the features of a pilocytic astrocytoma including sx
- Mc benign brain tumour in children
- Often in cerebellum or optic pathway
- Sx: ehadaches, nausea, visual disturbances and balance issues
326
Describe the features of a ependymoma including sx
- 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
327
Describe the features of a craniopharyngioma including sx
- Benign tumour near the pituitary gland and hypothalamus
- Sx: endocrine dysfunction, vision problems, growth delays
328
Describe the management of paediatric brain tumours
- MDT
- Steroids-> reduce intracranial swelling
- Anticonvulsants
- Chemo, radiotherapy
- Surgical intervention-> including ventriculoperitoneal shunts or drains to treat hydrocephalus
329
Describe the prognosis of paediatric brain tumours
- Good for CNS tumours, pilocytic astrocytomas and craniopharyngiomas
- Poorer prognosis with gliomas
330
Describe the aetiology of von Willebrand's disease
- Usually genetic mutation that results in a deficiency/dysfunction of VWF-usually autosomal dominant inheritance
331
Describe the signs and symptoms of von Willebrand's disease
- Excess/prolonged bleeding from minor wounds/post-op
- Easy bruising
- Menorrhagia
- Epistaxis
- GI bleeding
332
Describe the management of von Willebrand's disease
- Desmopressin: temporarily increases F8 and VWF levels by releasing endoethlial stores
- TXA for minor bleeding
- VWF-F8 concentrates if above unsuccessful and bleeding persistent
333
Describe the epidemiology of thalassaemia
- Believed to give some protection against malaria-> found in mediterranean europe, central africe, middle ease, india and southeast asia
334
Describe the inheritance of alpha thalassaemia
- Autosomal recessive
335
Describe the pathophysiology of alpha thalassaemia
- non functioning copies of the 4 alpha globin genes on chromosome 16
- Symptomatic when >=2 copies of gene are lost
336
Describe the features of alpha thalassaemia if 1 or 2 alpha globulin alleles are affected
- Alpha thalassaemia trait-mild asx anaemia
- Bloods: hypochromic + microcytic but Hb usually normal
337
Describe the features of alpha thalassaemia if 3 alpha globulin alleles are affected
- Symptomatic haemoglobin H disease
- Hypochromic microcytic anaemia with splenomegaly
- Normal survival
338
Describe the features of alpha thalassaemia if all 4 alpha globulin alleles are affected
- Incompatible with life
- Lack of alpha globin-> excess gamma chains-> Hb Barts
- Hydrops fetalis
339
Describe the signs and symptoms of alpha thalassaemia
- Jaundice
- Fatigue
- Facial bone deformities
340
Describe the management of alpha thalassaemia
- Blood transfusions
- Stem cell transplant
- Splenectomy may be used especially in Hb H
341
Describe the pathophysiology of beta thalassaemia
- 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
342
Describe the signs and symptoms of beta thalassaemia major
- 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
343
Describe the management of beta thalassaemia major
- Regular blood transfusions-> iron overload
- Hydroxycrbamide-> boost HbF levels
- Bone marrow transplant-> potentially curative but risks
- Iron chelation for iron overload(desferrioxamine, deferiprone)
344
Describe the symptoms of beta thalassaemia minor
- Usually mild asymptomatic anaemia
345
Describe the epidemiology of sickle cell disease
- Mc in people of Afrian descent-portection against malaria
346
Describe the pathophysiology of sickle cell disease
- 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
347
Describe the aetiology of sickle cell
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
348
Describe the signs and symptoms of sickle cell
- Vaso-occlusive crisis: severe pain due to tissue ischaemia
- Dactylitis common presentatino in infants <6 months
- Anaemia: increased haemolysis of sickle cells
- Jaundice-> consequence of haemolysis
- Acute chest syndrome: lung infarction or infection
349
Describe the management of an acute sickle cell crisis
- 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
350
Descirbe the long-term management of sickle cell anaemia
- 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
351
Describe the signs and symptoms of fanconi anaemia
- Cytopenias-> increased bruising/bleeding/infections
- Symptomatic anaemia-> mpaired oxygen-carrying capacity, aplastic anaemia
- Physical abnormalitis: short stature, VACTERL-H malformations
- Cafe-au lait spots
- Increased risk of acute myeloid leukaeemia
352
Describe the signs and symptoms of haemophilia
- Usually early in life with spontaenous deep+severe bleeding into soft tissues, joints and muscles-previously joint damage resulting in deforming arthropathy(haemoarthroses and haematomas)
- Excessive bleeding post surgery/trauma
- Cerebral haemorrhage-> not as common anymore
353
Describe the management of haemophilia
- 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 forbut avoid in muscle haematomas/haemarthrosis
354
Describe the pathophysiology of ITP?
- Spleen produces antibodies directed against the glycoprotein 2b/3a or Ib-5-9 complex
355
Describe the aetiology of ITP
- 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
356
Describe the signs and sx of ITP
- Bruising
- Petechial/purpuric rash
- Blleeding(lc)-epistaxis or gingival bleeding
- Unusually heavy menstrual flow in women/blood in urine/stools
357
Describe the management of ITP
- 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
358
Describe the prognosis of ITP in children
- Generally self-resolving
- 1/5: chronic
- If not resolved in 6 months: consider differentials including bone marrow aspirate
359
Describe the aetiology of TTP
- Hereditary: congenital mutatin of ADAMST13
- AI: AI inhibition of ADAMST13
360
Describe the clinial features of TTP
Pentad of:
- Fever
- Microangiopathic haemolytic anaemia
- Thrombocytopenic purpura
- CNS involvement: headache, confusion, seizures
- AKI
Rare, typically adult females
361
Describe the management of TTP
- Fresh frozen plasma-contains vWF
- Plasma exchange: removes antibodies and toxins associated with pathogenesis of disease
- High dose steroids, low dose aspirin and rituximab
362
Describe the epidemiology of testicular torsion
- Mc in neonates and males between 13-16 years
363
Describe the signs and symptoms of testicular torsion
- 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
364
Describe the management of testicular torsion
- Urgent surgical exploration
- Bilateral orchidopexy-fixation of both testicles to prevent future torsion
365
Describe the prognosis of testicular torsion
- Worse in neonates-> testis rarely viable
366
Describe the aetiology of testicular torsion in neonates?
- Torsion is extravaginal-> spermatic cord and tunica vaginalis twist together in or just below inguingal canal
367
Describe the management of testicular torsion in neonates
- Torted testis is removed and contralateral testis is fixed in place
368
Descirbe the epidemiology of precocious puberty
- Mc in females than males
- Average onset of puberty has been decreasing over the past few decades-thought to be due to obesity
369
Describe the pathophysiology of gonadotrophin dependent precocious puberty
- 'central/true'
- Due to premature activation of hypothalamic-pituitary-gonadal axis
- FSH and LH raised
370
Descirbe the pathophysiology of gonadotrophin independent precocious puberty
- 'pseudo/false'
- Due to excess sex hromones
- FSH nad LH low
371
Describe the management of precocious puberty
- GnRH analogues to suspend progression of puberty
- urgery to resect tumours
- Glucocorticoids for CAH
- Depends on underlying cause
372
Describe the inheriitance of Kallmann's syndrome
- Usually X-linked recessive
373
Describe the pathophysiology of Kallmann's syndrome
- Failure of GnRH -secreting neurons to migrate to the hypothalamus
374
Describe the management of Kallmann's syndrome
- Testosterone supplementation
- Gonadotrophin supplementation may result in sperm production if fertility is desired laater in life
375
Describe the epidemiology of congenital adrenal hyperplasia
- Boy-more severe
- 75%: salt-losing
- 25%: non-salt losing
376
Describe the pathophysiology of congenital adrenal hyperplasia
- 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
377
Describe the aetiology of congenital adrenal hyperplasia
- 21 hydroxylase deficiency-mc-90%-> cortisol deficiency and excess androgen production
- 11-beta-hydroxylase
- 17-hydroxylae deficiency: very rare
378
Describe the signs and symptoms of congenital adrenal hyperplasia
- 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
379
Describe the sx of a salt-wasting crisis
- Dehydration and vomiting
- Hyponatraemia
- Hyperkalaemia
- Circulatory shock and metabolic acidosis
- Life-threatening
380
Describe the management of congenital adrenal hyperplasia
- Glucocorticoid(hydrocortisone) and mineralocorcticoid(fludrocortisone) replacement
- Patient education: if unwell: increase hydrocortisone and ma need IV fluids
- Surgical intervention: virilised females-correct external genital abnormalities
381
Describe the management of obesity in children
- Encourage healthy lifestyle
- >12yrs with severe physical/psychological comorbidities: drug treatment such as orlistat under MDT
382
Describe the epidemiology of congenital hypothyroidism
- F>M
383
Describe the aetiology of congenital hypothyroidism
- 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 too maternal carbimazole use or maternal antibodies
384
Describe the clinical features of congenital hypothyroidism
- Prolonged neonatal jaundice
- Delayed mental and physical milestones
- Short stature
- Puffy face, macroglossia
- Hypotonia
- Myxoedema
- Bradycardia
- If not dx early: altered nerudevelopment and cognitive disability
385
Describe the management of congenital hypothyroidism
- 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
386
Describe the epidemiology of pica
- 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)
387
Describe the aetiology of pica
- Nutritional deficiencies: iron and zinc
- Developmental and behavioral
- Psychiatric disorders: OCD, schizophrenia, ASD,, intellectual disorders
388
Describe the symptoms of pica
- Persistent craving and ingestion of non-food for at least a month
- Geophagia: dirt/clay
- Cornstarch
- Ice
- Paper
- Chalk
- Soap
- Hair
389
Describe the management of pica
- Iron/zinc supplements
- Dietary counselling
- Behavioural therapy including CBT
- SSRIs: underlying OCD or severe psychiatric disorders
- Environmental modification: remove objects in environment, supervision aand restricted access to pica substances
390
Describe the epidemiology of eczema
- Very common
- Childhood onset common
- Prevalence decreases with age
- Urbanisation and industrialisation associated with higher prevalence
391
Describe the pathophysiology of eczema
- Vast lymphcoytic infiltration into dermis
- Often IgE-mediated allergic response to environmental allergens
392
Describe the general features of eczema
- 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
393
Describe the management of eczema
- 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
394
Describe the symptoms of eczema herpeticum
- 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
395
Describe the cllinical features of Stevens Johnson syndrome
- 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
396
Describe the management of Steven-Johnson syndrome
- Hospital admission
- Supportive care-> flluid and electrolyte management, pain control, treat secondary infections
397
Describe the prognosis of Steven-Johnson syndrome
- 10% mortality rate-usually due to dehydration, infection or DIC
- TEN: 30% mortality rate
398
Describe the aetiology of allergic rhinitis
- IgE mediated response to allerganes within the environemnt
- Seasonal: hayfever-pollens
- Pereennial: throughout the year
- Occupational: exposure to specific allergens
399
Describe the signs and sx of allergic rhinitis
- Nasal pruritus
- Sneezing
- Clear nasal discharge
- Post-nasal drip
- Nasal pruritus
- Eye redness
- Eye puffiness
- Watery eye discharge
400
Describe the management of allergic rhinitis
- Avoid triggers
- Nasal irrigation with saline
- Oral/intranasal antihistamines
- Intranasal teroids
- Oral steroids
- SHort course or topical nasal decongestant-not for prolonged periods
- ENT referral
401
Describe the features of erythema toxicum
- Benign rash in newborns
- Erythematous macules, papules and pustule
- Waxes and wanes over severeal days-usually no more than one day
402
Describe the rash assocaited with scepticaemia?
- Non-blanching purpuric rash
- Lethargy, headache, fevers, vomiting
403
Describe the rash assocaited with slapped cheek syndrome
- Rash on both cheeks
- Fever, URTI sx
404
Describe the rash associated with hand foot and mouth disease
- Blisters on hands and feet
- Grey ulcerations in buccal cavity, fever, lethargy
405
Describe the rash associated with scarlet fever
- Coarse red rash on cheeks
- Sore throat, headache, fever, bright red tongue
- Sandpaper texture rash
406
Describe the rash associated with measles?
- Erythematous, blanching maculoppapular rash
- Fever, cough, runny nose, conjuncitivitis, Koplik spots
407
Describe the rash associated with urticaria
- Raised, itchy red rashes
- Usually not accompanied by fever
408
Describe the rash associated with chickenpox
- Maculopapular vesicular rash that crusts over and forms blisters
409
Describe the rash associated with roseola
- Lace-like rash across whole body
- High fever
410
Describe the rash associated with rubella
- Starts on head and spreads to trunk
- Post-auricular lymphadenopathy
411
Describe the epidemiology of urticaria
- Women>men
- Peak incidence: 20-40yrs
412
Describe the pathophysiology of urticaria
- Release of histamine and other mediators form mast cells and basophils-> increased vascular permeability and formation of wheals
- Both immune-mediated and non-immune mechanisms contribute to development of urticaria
413
Describe the signs and sx of urticaria
- Pruritus
- Erythematous wheals with well defined borders
- Whelas that vary in shape and size
- Rapid onset and resolution
- Occasionally angiodema-> can involve lips, eyelids or extremities
414
Describe the management of urticaria
- 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
415
Describe the features of stork marks
- Red/pink patches, often on eyelids/head/neck
- Very commmon
- Easier to see when baby cries
- Usually fade by age 2 on forehead/eyelids, longer if back of head or neck
416
Describe the features of haemangiomas
- 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
417
Describe the features of port wine stains
- 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 lgihter using laser tx
- Sign of Sturg-Weber syndrome/Klippel-trenaunay syndrome but this is rare
418
Describe the features of cafe-au-lait spots
- 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
419
Describe the features of blue-grey spots
- Loook like bruises
- There from birth
- Mc on babies with black/brown skin
- No tx, usulaly go away by age 4
- Should be recorded on medical records-avoid thinking its abuse
420
Describe the epidemiology of anaphylaxis
- Relatively uncommon
- Higher risk in patients with asthma/atopy
- Incidence rising especially in Western countries
421
Describe the aetiology of anaphylaxis
- 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
422
Describe the clinical features of anaphylaxis
- 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 pruritus
- Widespread erythematous or urticarial rash
- GI: abdominal pain, diarrhoea, vomiting
423
Describe the acute management of anaphylaxis
- Immediate IM adrenaline
- Remove trigger
- Manage ariway and high flow oxygen
- IV fluids
- If no response, repeat adrenaline
424
Describe the long term management of anaphylaxis
- Counselling on use of adrenalinie auto-injectors
- Supply of 2 auto-injectors
- Written advice
- Referral to local allergy service for follow up
425
What is rheumatic fever?
- AI systemic complication of lancefield Group A beta-haemolytic strep infection (scaarlet fever) that occurs 2-4 weeks post infection
426
Describe the epidemiology of rheumatic fever
- Mc in developing countries
427
Describe the pathophysiology of rheumatic fever
- 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
428
Describe the split of different vavle diseases occuringin rheumatic heart disease
- Mitral valve disease: 70% MC
- Aortic valve: 40%
- Tricuspid: 10%
- Pulmonary valves: 2%
429
Describe the minor criteria for rheumatic fever
- Raised ESR/CRP
- Pyrexia
- Athralgia(if arthritis not a major criteria)
- Prolonged PR interval
430
Describe the management of rheumatic fever
- 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
431
Describe the prognosis of rheumatic fever
- No cure for rheumatic heart disease
- If severe valvular disease: requires surgery-40% with severe rheumatic heart disease
432
Describe the aetiology of paediatric heart disease
- Usually congenital heart defects
- Acquired: myocarditis, arrhythmias, htn
- Different underlying mechanisms compared to adultss
433
Describe the signs and symptoms of paediatric heart failure
Infants:
- Difficulty feeding
- Faltering growth
Young children:
- Abdo pain and vomiting especially on exertion
- Poor appetite
Adolescents:
- Exercise intolernace
- Fatigue
All ages: cyanosis and hepatomegly
434
Describe the management of paediatric heart failure
- 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
435
Describe the prognosis of paediatric heart failure
- Progressive
- Leading cause of mortality in children with heart disease
436
Describe the pathophysiology of infective endocarditis
- Damage to endocardium-> heart valve forms local blood clot-> platelets and fibrin deposits allow bacterium to stick to endocardium-> formation of vegetations
- Valves have no dedicated blood supply-> body can't launch immune response to vegitations
437
Describe the major criteria for infective endocarditis
- 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
438
Describe the minor criteria for infective endocarditis
- 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
439
Describe the management of infective endocarditis
- 6 week courseof IV abx-usually midline insertion
- When organism and sensitivities not known: amoxicillin (vancomycin)
440
Describe the prognosis of infective endocarditis
- Without tx, can rapidly lead to heart failure and death
441
Describe the aetiology of congenital heart block
- Maternal systemic AI diseases: SLE and Sjogren: anti-Ro and/or anti La
- Structural heart defects
- Some cases idiopathic-can run in families
442
Describe the signs and symptoms of congenital heart block
- Asx
- Neonates: bradycardia and/or circulatory shock
- Older children: pre-syncope, syncope, usually in first few years of life
443
Describe the management of congenital heart block
- Asx: close monitoring
- Sx: neonatal ICU admission-> isoprenaline
- Implant pacemaker
444
Describe the prognosis of congenital heart block
- Up to 20% chance of intrauterine fetal death
- Good prognosis after pacemaker insertion
- 20% ris of recurrence in future pregnancies-> pre-conception counselling
445
Describe the signs and sx of IBS
- >=6 months
- Abdo pain
- Bloating
- Change in bowel habit
- Sx made worse by eating
- Passage of mucus
- Lethargy, nausea, backache, bladder sx also common
446
Describe the management of IBS
- Dietary and lifestyle: stress maanagement, low FODMAP diet
- Antispasmodics: mebeverine, laxatives, anti-diarrhoeal
- TCA's
Refractory: psychotherapy including CBT
447
Describe the epidemiology of gastroenteritis
- Common worldwise and affects all ages
- Leadig cause of death in children under 5 worldwide
448
Describe the bacterial causes of gastroenteritis
- S.aureus-cooked meats and cream products
- Bacillus cereus-reheated rice
- Clostridium perfringens
- Campylobacter
- E.coli
- Salmonella
- Shigella
449
Describe the presentation of a patient with gastroenteritis
- Diarrhoea and vomitng
- Blood in stool-bacterial pathogen
- Systemic: malaise and fever
- Signs of dehydration/shock if severe
450
Describe the management of gastroenteritis
- 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
451
Describe the prognosis of gastroenteritis
- Vomiting 1-2 days, diarrhoea 5-7 days
- Most children: complete resolution within 2 weeks
- Secondary lactose intolerance: diarrhoea for up to 6 weeks
452
Describe the epidemiology of Crohn's disease
- Mc in northern climates and develoepd countries
- Bimodal age of onset: 15--40yrs, then 60-80yrs
- Mc in caucasians
453
Describe the clinical features of Crohn's disease
- 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
454
Describe the management of Crohn's disease
- Smoking cessation
- In flare: monotherapy with glucocorticoids(oral/IV prednisolone or hydrocortisone), biologics
- Remission: azathioprine, mercaptopurine, methotrexate, biologics(infliximab or adalimumab)
- Surgical: treat complications
455
Describe the management of peri-anal fistulae in Crohn's disease
- Drainage seton
- Fistulotomy
456
Describe the management of peri-anal abscess in Crohn's disease
- IV abx: ceftriaxone+metronidazole
- Exam under anaesthetic and incision and drainage
457
Describe the clinical features of Ulcerative colitis
- 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: PSC
- AA amyloidosis
458
Describe the management of mild-moderate Ulcerative colitis
- Topical amiosalicylate
- Oral if no improvement in 4 weeks
- Consdiier adding oral prednisolone
- Etrasimod
- Oral tacrolimus
459
Describe the management of acute severe Ulcerative colitis
- IV corticosteroids
- Add IV ciclosporin of no improvements in 72 hours
- Trial etrasimod(velsipity)
- Emergency surgery
460
Describe the epidemiology of coeliac disease
- F>M
- Bimodal: infancy or 50-60yrs
- Increased incidence in Irish
461
Describe the pathophysiology of coeliac disease
- Sensitivity to gluten and realted prolamines results in villous atrophy of the lining of the small intestine and results in malabsorption
462
Describe the symptoms of coeliac disease in children
- Abdo pain
- Distention
- N+V
- Diarrhoea
- Steatorrhoea
- Fatigue
- Weight loss
463
Describe the management of coeliac disease
- Lifelong gluten free diet
- Patient education
- Supplements for deficiencies: iron/vitamins etc
- Regular monitoring to ensure diet adherence and screen for complications
464
Describe the presentation of a child with malnutrition
- Poor growth
- Plateaued weight gain or weight loss
- Difficulties concentrating
- Intellectual disability
- Specific vitamin deficiencies
465
Describe the management of malnutrition in the uk
- Vitamin supplements
- Increase caloric intake with high-energy food and drinks(smoothies, milkshakes, cheese)
- Specific supplements: iron
466
Describe some signs that might point to non-organic FTT
- Nutritional indicators
- Social indicators-signs of neglect(poor hygiene, unattetended medical needs)
- Poor parent-child interactions, parental mh issues
467
Describe the management of FTT
- Stabilisation if severe dehydration/electrolyte imbalances
- Nutritional supprot-dietitian
- Mineral and vitamin supplementation
- Address underlying cause
- Follow up to plot growth chart
468
Describe the aetiology of Hirschsprung's disease
- Aganglionic segment of bowel due to a developmental failure of the parasympathetic Auerbach and Meissner plexuses
469
Describe the pathophysiology of Hirschsprung's disease
- 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
470
Describe the epidemiology of Hirschsprung's disease
- Rare
- 3 x more common in males
- Down's syndrome
471
Describe the management of Hirschsprung's disease
- Rectal washouts/bowel irrigation
- Definitive: surgery to affected segment of colon
472
Describe the pathophysiology of Meckel's diverticulum
- Remnant of vitellointestinal duct which usually disappears around 6th week gestation
- Contains ectopic ileal , gastric or pancreatic mucose
473
Describe the presentation of a patient with Meckel's diverticulum
- Usually asx
- Painless rectal bleeding-ulceration of adjacent tissue
- Intestinal obstruction
- Intussusception
- Abdominal pain mimicking appendicitis
474
Describe the management of Meckel's diverticulum
- Laparoscopic surgical resection of diverticulum l if narrow neck/symptomatic using wedge excision or small bowel resection and anastamosis
475
Describe the management of toddler's diarrhoea?
- 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
476
Describe the symptoms of infantile colic
- Bouts of excessive crying and pulling up of legs
- Often worse in evening
477
Describe the management of infantile colic
- 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
478
Describe the epidemiology of cow's milk protein intolerance
- First 3 months
- Formula fed infants
479
Describe the presentation of infants with cow's milk protein intolerance
- Persistent diarrhoea, vomiting, FTT, abdo pain in first few months of life
- Usually present after introduction of cow's milk to diet
- Abdo pain: may draw up legs if younger
- Diarrhoea: may be bloody/mucus
- Eczema/urticaria
- Immediate IgE mediated: urticaria, angioedema, vomitinfg wheezing within 2 hours of presentation
480
Describe the management of cow's milk protein intolerance in a formula fed infant
- Extensive hydrolysed formula milk replacement
- Amino acid-based formula as second line
481
Describe the management of cow's milk protein intolerance in a breast fed infant
- Continue breasteeding
- Eliminate cow's milk protein from maternal diet
- Use extensively hydrolysed formula milk when done breastfeeding until 12 months of age
482
Describe the epidemiology of choledochal cyst
- Rare
- More common in Asian children
- 3 x as common in girls
483
Describe the signs and symptoms of choledochal cyst
- 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
484
Describe the aetiology of neonatal hepatitis
- Viruses: rubella, CMV, hepatitis A/B/C
- Idiopathic-mc
- Genetic: A1AT deficiency
485
Describe the presentation of neonatal hepatitis
- Jaundice
- Pruritus
- Rashes
- Dark urine
- Hepatomeglay
- FTT
486
Describe the management of neonatal hepatitis
- Medical: Ursodeoxycholic acid-increase bile formation
- Surgery: cirrhotic liver disease/liver transplant if severe
- Optimise nutrition and vitamin supplementation
487
Describe the features of a reducible hernia
- Bowel can be reduced back into abdo cavity
- Painless and often asymptomatic
488
Describe the features of a strangulated hernia
- 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
489
Describe the features of an incarcerated hernia
- Blood supply not necessarily compromised
- Presents with abdominal pain and irreducible mass
490
Describe the features of umbilical hernias
- Often due to failure of umbilical ring to close after umbilical cord falls off
- Very common in children
- Often close without intervention
491
Describe the features of epigastric hernias
- Herniation between sternum and umbilicus
- Don't close spontaneously
- Not common in children
492
Describe the features of inguinal hernias
- 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
493
Describe the epidemiology of hernias
- Common in children
- M>F
- Strangluated more common in adults due to age-related weakening of abdominal wall
494
Describe the presentaiton of a patient with a strangulated hernia
- 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
495
Describe the management of a strangulated hernia
- Surgical:
- Release herniated bowel to restore blood flow
- Remove necrotic tissue to prevent sepsis
- Reinforce weakened area of abdo wall with mesh etc
