PAEDS - cardio, resp, GI Flashcards
(417 cards)
1
Q
define croup
A
acute infective URT disease affecting young children
2
Q
epidemiology of croup
a) time of year
b) age
A
a) spring/autumn
b) 6m-2y
3
Q
what causes croup?
A
MAINLY parainfluenza virus
others:
- influenza
- adenovirus
- respiratory syncytial virus (RSV)
4
Q
RFs for croup
A
- young
- family hx
- congenital/acquired airway narrowing
- male gender
5
Q
pathophysiology of croup
A
- upper resp tract infection causes oedema in the larynx
- swelling of larynx, vocal cords and trachea
- narrowing of subglottic region
6
Q
classic presentation of croup
A
classic:
- cold-like sx in days preceding (prodromal)
- acute stridor
- barking seal like cough
- worse at night
other:
- hoarse voice
- low-grade fever
- if severe»_space; resp distress
7
Q
signs of croup with respiratory distress
A
seal-like barking cough with:
- recession
- agitation
- lethargy
8
Q
investigations for croup
A
often clinical diagnosis
but consider:
- ABCDE
- ENT exam
- cervical lymph nodes exam
- lung auscultation
- CXR (to exclude other causes)
9
Q
what classic CXR sign will be present in croup?
A
steeple sign (narrowing of trachea)
10
Q
management of non-severe croup
A
- normally self-limiting (<48h)
- supportive with fluids and rest
- sit child up during attacks
- stay off school
- can give single dose of oral dexamethasone (150mcg/kg) and repeat after 12h if required
11
Q
what did croup used to be caused by before vaccinations? what did it lead to?
A
diptheria
led to epiglottitis > high mortality
12
Q
stepwise management options in severe croup
A
- oral dexamethasone
- oxygen
- nebulised budesonide
- nebulised adrenaline
- intubation and ventilation
13
Q
what dose of dexamethasone should be given in croup?
A
0.15mg/kg (single dose)
14
Q
define childhood pneumonia
A
- acute LRT infection
- infection and inflammation of the parenchyma with consolidation
15
Q
epidemiology of childhood pneumonia
A
relatively common - 14% of all deaths of children <5
16
Q
common bacterial causes of childhood pneumonia (inc most common) (6)
A
- MOST = streptococcus pnuemonia
- second = H.influenzae type B (Hib)
- group A strep
- group B strep
- S.aureus
- atypical = mycoplasma pneumonia/tuberculosis
17
Q
which children are likely to contract pneumonia caused by…
a) H.Influenzae type b
b) group B strep
A
a) pre or unvaccinated children
b) pre-vaccinated infants
18
Q
how is group B strep pneumonia contracted in children
A
often during birth as it colonises the vagina
19
Q
common viral causes of childhood pneumonia (inc most common) (4)
A
- most common = respiratory syncytial virus (RSV)
- parainfluenza virus
- influenza virus
- adenovirus
20
Q
risk factors for childhood pneumonia (5)
A
- young age
- unvaccinated for Hib
- immunocompromise
- pre exisiting illness e.g. CF, measles
- environment e.g. crowded home, parental smoking, indoor air pollution
21
Q
pathophysiology of childhood pneumonia
A
inflammation of lung parenchyma (lung tissue) and sputum filling the airways and alveoli
22
Q
signs and symptoms of childhood pneumonia
A
- cough (wet + productive)
- high fever (>38.5)
- lethargy
- delirium
- high RR
- tachypnoea
23
Q
characteristic chest signs of childhood pneumonia (3)
A
- bronchial breath sounds (harsh, equally loud on inspiration and expiration)
- focal coarse crackles
- dullness to percussion
24
Q
signs indicating sepsis secondary to childhood pneumonia (6)
A
- tachypnoea
- tachycardia
- hypoxia
- hypotension
- fever
- confusion
25
investigations for childhood pneumonia
1. sputum cultures and throat swabs - bacterial causes
2. viral PCR - viral causes
3. CXR
26
what will a CXR show in childhood pneumonia?
dense/fluffy opacity occupying a portion of/a whole lobe
27
investigations if child with pneumonia has suspected sepsis
1. blood cultures
2. capillary blood gas analysis (monitor resp, metabolic acidosis, lactate)
28
management of childhood pneumonia
a) typical
b) atypical
c) penicillin allergy
d) sepsis
a) 1st line = dispersible amoxicillin
can also add oxygen
b) add a macrolide e.g. erythromycin, clarithromycin
c) use macrolides as a monotherapy
d) IV abx e.g. co-amoxiclav
29
what immunisations can be used to help prevent childhood pneumonia? (4)
1. Hib
2. pneumococcus
3. measles
4. whooping cough
30
define asthma + what is it characterised by?
chronic inflammatory **obstructive** airway disease
characterised by **bronchoconstriction and
excessive secretion** production
31
cause of childhood asthma
normally ATOPY - tendency to develop IgE mediated reactions to common aeroallergens
32
pathophysiology of childhood asthma
airflow limitation caused by
1. hypersensitive bronchoconstriction
2. mucosal inflammation
3. increased mucosal production
33
presentation of childhood asthma
1. WHEEZE
- episodic, intermittent exacerbations
- diurnal variation (worse at night and early morn)
- bilateral, widespread, "polyphonic"
- improves w bronchodilators
2. chest tightness
3. dry cough w wheeze and SOB
4. typical triggers
34
typical triggers for childhood asthma
1. dust (house dust mites)
2. animals
3. cold air
4. exercise
5. smoke
6. food allergens e.g. peanuts, shellfish, egg
35
diagnosis of asthma
if probability is:
a) low
b) intermediate/high
- normally clinical
- children usually not diagnosed until 2-3y
a) consider referral to specialist
b) trial of tx can be implemented, if improves sx then diagnosis is made
36
what investigations for asthma can be used if there's intermediate possiblity/diagnostic doubt? (4)
1. spirometry with reversibility testing (only in children >5!)
2. direct bronchial challenge
3. fractional exhaled nitric oxide (FeNO)
4. peak flow variability with diary
37
medical therapy for asthma in children <5 (4 steps)
1. start SABA e.g. salbutamol
2. add low dose corticosteroid OR leukotrine antagonist (e.g. oral montelukast)
3. add other option from step 2
4. refer to specialist
38
medical therapy for asthma in children aged 5-12 (6 steps)
1. start SABA e.g. salbutamol
2. add regular low dose corticosteroid inhaler
3. add LABA e.g. salmeterol
4. titrate corticosteroid inhaler to medium dose. consider adding montelukast or oral theophylline
5. increase corticosteriod to high dose
6. refer to specialist
39
medical therapy for asthma in children aged 12+
same as adults!!
1. start SABA e.g. salbutamol
2. add regular low dose corticosteroid inhaler
3. add LABA e.g. salmeterol
4. titrate corticosteroid inhaler to medium dose. consider trial of montelukast, oral theophylline OR inhaled LAMA ie tiotropium
5. titrate corticosteroid dose to high. combine additional tx from step 4. option of adding oral beta-2-agonist ie oral salbutamol. refer to specialist
6. add oral steroids at lowest dose poss
40
presentation of acute asthma attack and auscultation findings
1. progressively worse SOB
2. signs of resp distress
3. tachypnoea
4. expiratory wheeze
5. "tight" on auscultation, reduced air entry
41
asthma severity: category 'moderate'
1. peak flow 50-75% predicted
2. normal speech
42
asthma severity: category 'severe'
1. peak flow 33-50% predicted
2. saturations <92%
3. unable to complete sentences in one breath
4. signs of resp distress
5. RR >40 in 1-5y, >30 in 5+
6. HR >140 in 1-5y, >125 in 5+
43
asthma severity: category 'life threatening'
1. peak flow <33% predicted
2. saturations <92%
3. exhaustion and poor resp effort
5. hypotension
6. silent chest
7. cyanosis
8. altered consciousness/confusion
9. normal paCO2 (suggests exhaustion as not getting rid of it)
44
stepwise approach to managing moderate to severe cases of acute asthma
1. salbutamol inhaler via spacer device
2. nebulisers with salbutamol/ipratropium bromide
3. oral prednisolone
4. IV hydrocortisone
5. IV mag sulphate
6. IV salbutamol
7. IV aminophylline
45
general staples of management for an acute asthma attack(4)
1. supplementary O2
2. bronchodilators e.g. salbutamol > ipratropium > magnesium sulphate
3. steroids e.g. oral prednisolone or IV hydrocortisone
4. abx if bacterial cause suspected
46
define viral-induced wheeze. what is it caused by?
an acute wheezy illness caused by viral infection with RSV (respiratory syncytial virus)
47
who is typically affected by a viral-induced wheeze? why?
typically under 3y
very small diameter airway > slight narrowing caused by inflammation and oedema leads to a proportionally larger restriction in airflow (Poiseuille's law)
48
risk factors for viral-induced wheeze
family history
49
how to differentiate between a viral induced wheeze and asthma
viral induced wheeze:
- presents <3
- no atopic hx
- only occurs during viral infections
50
signs and symptoms of a viral-induced wheeze
1. transient and episodic wheezing episodes
2. viral illness (fever, cough, coryzal sx) for 1-2 days preceding....
3. SOB
4. signs of resp distress
5. expiratory wheeze throughout the chest
51
management of viral-induced wheeze
a) moderate
b) severe/life-threatening
SAME AS ACUTE ASTHMA
a) - nebulised salbutamol
- nebulised ipratropium bromide
- steroids e.g. oral pred or IV hydrocortisone
- abx if evidence of infection
b) stepwise:
- O2
- salbutamol inhaler via spacer device
- nebulisers with salbutamol/ipratropium bromide
- oral prednisolone
- IV hydrocortisone
- IV mag sulphate
- IV salbutamol
- IV aminophylline
52
what type of wheeze in children is never heard in asthma or viral-induced wheeze? what should you suspect in this case?
asthma/viral-induced will never cause a FOCAL WHEEZE
be cautious of airway obstruction e.g. inhaled foreign body or tumour
53
what examination is contraindicated in croup? why?
throat examination
can cause child to get agitated > rapid airway constriction
54
management of a child with croup if they have an audible stridor at rest
admit to paediatric ward - stridor at rest = red flag for admission
55
what is bronchiolitis?
a lower respiratory viral infection of the bronchioles
56
which virus causes bronchiolitis?
respiratory syncytial virus (RSV)
57
epidemiology of bronchiolitis:
a) who?
b) when?
a) common in <2y (mostly <6m)
b) winter and spring months
58
risk factors for bronchiolitis
- breast fed for <2m
- smoke exposure e.g. parents
- siblings who attend nursery/school
- chronic lung disease from prematurity
- neuromusc disorder preventing clearance e.g. CF
59
physiological changes in bronchiolitis (4)
1. proliferation of goblet cells > excess mucus
2. IgE-mediated type 1 allergic reaction > inflammation
3. bronchiolar constriction
4. infiltration of lymphocytes, cytokines, chemokines
60
signs and symptoms of bronchiolitis
SX
- typically increasing over 2-5 days
- low-grade fever
- starts with coryzal sx e.g. nasal congestion, rhinorrhoea
- then chest sx e.g. cough, wheeze
- feeding difficulties
SIGNS
- inspiratory crackles
- expiratory wheeze
- hyperinflated chest
61
what lung sounds are heard in bronchiolitis?
inspiratory crackles
expiratory wheeze
62
investigations for bronchiolitis
1. nasopharyngeal aspirate or throat swab - RSV rapid testing and viral cultures
2. FBC
3. blood/urine cultures (IF fever)
63
signs of respiratory distress in an ill child (8)
1. raised RR
2. use of accessory muscles e.g. sternocleidomastoid, abdo, intercostal
3. recessions
4. nasal flaring
5. head bobbing
6. tracheal tugging
7. cyanosis
8. abnormal airway noises e.g. grunting, wheezing, stridor
64
indications for an infant with bronchiolitis to be admitted? (9) (age, MHx, milk intake, RR, O2, signs)
1. <3m
2. pre-existing condition e.g. prematurity, DS, CF
3. 50-75% or less of their normal milk intake
4. clinical dehydration
5. RR >70
6. O2 <92%
7. moderate to severe resp distress
8. apnoeas
9. parents not confident in ability to manage at home
65
typical management of bronchiolitis
supportive at home - adequate fluid/milk intake, nasal suctioning, rest
if in hosp - O2, may provide NG feeding
66
what preventional vaccine can be given to babies who are at high risk if they get bronchiolitis? when is it given and who is high risk?
a) palivizumab
b) given monthly in high risk season (oct-mar)
c) babies with CF, immunocompromise, CHD etc
67
what is cystic fibrosis?
an autosomal recessive disease caused by a mutation in the CFTR gene
68
what does the CFTR gene code for? where in the body is implicated?
encodes the CFTR protein - a chloride (Cl-) channel found in epithelial tissues e.g. lungs, pancreas, intestines, sweat glands and reproductive organs
69
how is CF passed on genetically?
it is autosomal recessive so both parents need to be carriers for the child to demonstrate the disease
70
epidemiology of CF
- 1/2 caucasian europeans are carriers
- most common lethal genetic disease in caucasian population
71
briefly outline the pathophysiology of CF in the
a) lungs
b) pancreas
c) GI tract
d) reproductive tract
OVERALL - cells can't transport Cl-
a) Cl- becomes trapped inside mucus-secreting cell rather than outside > less Cl- in mucus > water not attracted in > thickened mucus > impeded mucus clearance > chronic infection and inflammation
b) pancreatic duct is occluded in-utero > permanent damage to exocrine pancrease and insufficiency
c) small intestine secretes viscous mucus > bulky stools > bowel obstruction
d) 95% males infertile
72
presentation of CF in neonates (4)
1. failure to pass first meconium
2. bilious vomiting
(both signs of meconium ileus (small bowel obstruction)
3. failure to thrive
4. prolonged neonatal jaundice
73
presentation of CF in infancy > childhood (5)
1. failure to thrive
2. recurrent chest infections (wet-sounding cough), chronic sinusitis
3. pancreatic insufficiency - steatorrhea, diabetes
4. nasal polyps
5. chronic lung disease
74
when is CF normally identified? why?
in neonates - newborn screening (bloodspot test)
this is only a screening - still needs diagnostic testing
75
what is the gold std investigation for CF?
chloride sweat test - will be >60mmol/L
76
what happens when a sweat test is positive when investigating CF?
this is not sufficient to diagnose CF - need second test or identification of genetic mutation through genetic analysis
77
management for the respiratory sx of CF (4)
1. 1st line = chest physiotherapy twice daily
2. mucolytics e.g. DNase (reduces viscosity)
3. inhaled bronchodilator e.g. salbutamol
4. inhaled corticosteroid e.g. budesonide
78
management of pancreatic disease in CF
1. pancreatic enzyme supplementation (creon)
2. fat soluble vitamin (A, D and E) supplements
79
which bacteria are key colinisers in children with CF? which is particularly troublesome?
psuedomonas aeuruginosa (most troublesome, hard to treat and worsens prognosis)
staph aureus
80
how long should children with CF be on a course of antibiotics in the case of airway infections?
at least 2 weeks
81
what is the most likely infection a neonate will develop if the mother transmits group B strep?
neonatal sepsis
82
how is Pseudomonas aureginosa colinisation treated in children with CF?
aminoglycosides (e.g. gentamicin) or quinolones (e.g. ciprofloxacin)
think aureGinosa - Gent
Psuedo - cipro (sip)
83
what is acute epiglottitis?
a rare but serious infection of the supraglottis (upper part of larynx including epiglottis) that causes swelling and airway compromise
an airway emergency in children!
84
what age group is most commonly affected by acute epiglottitis?
2-6 years old
85
most common bacterial cause of acute epiglottitis
haemophilus influenzae type B (Hib)
86
RFs for acute epiglottitis (3)
1. non vaccination with Hib
2. immunocompromised
3. young age
87
signs and symptoms of acute epiglottitis
- high fever
- sore throat
- dribbling
- difficulty breathing
- dysphagia
- hoarse voice
- muffled voice
88
ddx for epiglottitis
1. croup
2. foreign body inhalation
3. inhalation of smoke
4. allergic laryngeal angioedema
5. infectious mono
88
features to help assess severity of epiglottitis
1. degree of stridor/recession
2. RR
3. HR
4. level of consciousness/exhaustion
5. pulse oximetry
89
which classic positioning do children display with acute epiglottitis?
the tripod position - sitting/standing while leaning forward with the hands on the knees/other surface
90
how is acute epiglottitis diagnosed?
if suspected do NOT try to examine the throat
diagnose by direct visualisation by a specialist - once in OP theatre and airway stabilised can perform laryngoscopy (will see swelling of supraglottic structures)
91
initial management of acute epiglottitis?
1. immediate senior involvement including airway specialists e.g. anaesthetics and ENT
2. endotracheal intubation may be needed
3. IV abx - cefotaxime or ceftriaxone
4. supplemental O2
5. consider corticosteroids e.g. dexamethasone
92
later management of a child with acute epiglottitis (once no longer intubated)
oral amoxicillin
93
what criteria is needed to be passed for a child to be discharged following an acute asthma attack? (3)
1. peak flow must be >75% expected
2. have had inhaler technique checked and recorded
3. be stable on discharge medications for at least 12-24h
94
what is the target oxygen saturation level in acutely asthmatic patients?
94-98%
95
define:
a) GOR
b) GORD
a) the passage of gastric contents into the oesophagus with/without regurgitation or vomiting
b) the presence of troublesome sx and/or complications of persistent GOR
96
what causes GOR/D in infants? (2)
1. short/narrow oesophagus
2. liquid diet and high caloric requirement puts strain on gastric capacity
97
risk factors for GOR/D in children (6)
1. premature
2. young infant (milk diet)
3. parental hx
4. obesity
5. hiatus hernia
6. neurodisability e.g. cerebral palsy
98
signs and symptoms of GOR/D in children
REGURGITATION and...
- distress e.g. excessive crying/crying while or after feeding
- milk coloured vomit
- hoarseness and/or chronic cough
- unexplained feeding difficulties e.g. refusing to feed, gagging, choking
- back arching
- faltering growth
99
what red flag features will NOT be present in GOR and suggest another pathology?
- frequent projectile vomiting (pyloric stenosis)
- bile stained vomit (obstruction)
- blood stained vomit (GI bleed)
- abdominal distension/tenderness (obstruction/acute surg condition)
- bulging fontanelle (meningitis, RIP)
- blood in stool (gastroenteritis, CMPA)
- chronic diarrhoea (CMPA)
- dysuria (UTI)
- fever (infection)
100
how does reflux and GORD differ?
it is normal for babies to reflux feeds - they have an immature LOS so contents easily reflux into oesophagus
it becomes more troublesome (GOR/GORD) when this causes the baby to become distressed
101
how is GOR usually investigated? what about in severe cases?
normally doesn't require investigations
in severe cases - can do barium meal and endoscopy
102
management for GOR/D in babies
a) mild GOR/D
b) problematic GOR/D
a) sometimes explanation, reassurance and practical advise is all that's needed
- small, frequent meals
- burping regularly to help milk settle
- not over-feeding
- keep baby upright after feeding
b) if problematic can give
- gaviscon mixed with feeds
- thickened milk/formula
- PPIs e.g. omeprazole (only if others inadequate!)
103
a) what is the name of the rare condition associated with GOR that causes brief episodes of abnormal movements in infants?
b) what are its key features?
c) what is its prognosis?
a) Sanlifer's Syndrome
b) key features are
- torticollis (forceful neck twisting)
- dystonia (twisting movements, arching of back of unusual posture)
c) normally resolves as reflux is treated
104
what is poor feeding? what age group is it most common in?
conditions in children <1 year that lead to impaired oral intake and are associated with medical/nutritional/feeding skill and/or psychosocial dysfunction
tends to be <1 but can be up to 3yo
105
common causes of poor feeding in children? (8)
1. GORD
2. malrotation
3. intussusception
4. Hirschsprung's
5. pyloric stenosis
6. gastroenteritis
7. infection
8. metabolic conditions
106
RFs for poor feeding (5)
1. prematurity
2. developmental delay
3. anatomical abnormalities of oropharynx/GI tract
4. GI surgery
5. trisomy 21
107
signs and symptoms of poor feeding
depends on cause! but general:
- taking >30 mins to feed
- inappropriate volume of feed
- vomiting
- abdominal pain/distension
- colic
- irritability/lethargy at mealtimes
- refusing to feed
- long term = faltering growth
108
how is poor feeding investigated? what should be considered?
process of ruling out possibilities!
consider...
- temporary exclusion of cow's milk (CMPA)
- oesophageal 24hr pH/impedence study (GORD)
- upper GI contrast study (structural probs)
- trial of lactose-free diet
- TTG antibodies (coeliac)
109
how is poor feeding managed?
depends on cause
general
- feeding assessment
- advice/nutritional support if anatomical abnormalities
- surgery if obstruction
- thickener/anti-reflux formula
- dietary modification
110
what is pyloric stenosis?
congenital hypertrophy of the pyloric sphincter (opening from the stomach to the small intestine) causing gastric outlet obstruction (stomach can't empty contents into duodenum)
111
epidemiology of pyloric stenosis (age, gender)
- 4-8 weeks
- more common in boys (4:1)
112
aetiology and RFs for pyloric stenosis
aetiology unknown!
RFs
- firstborn
- male
- family hx (especially on mum's side)
113
pathophysiology of pyloric stenosis
1. hypertrophy and narrowing of pyloric sphincter muscle
2. gastric outlet obstruction
3. excessive vomiting of stomach contents (can cause dehydration and electrolyte disturbance e.g. low Na2+ and K+)
4. bile is created by liver so vomiting in PS is nonbilious as hasn't reached liver yet!
114
signs and symptoms of pyloric stenosis
1. FORCEFUL VOMITING
- increased frequency over time
- increased forcefulness over time
- becomes projectile e.g. "hitting wall"
2. loss of interest in feeding
3. wave-like motion of abdomen shortly after feeding and just before vomiting
4. weight loss (if delayed presentation)
5. pale
115
what electrolyte complication can occur in pyloric stenosis? why?
hypochloric (low Cl-) hypokalaemic metabolic alkalosis
baby is vomiting up hydrochloric acid from stomach
116
investigations for pyloric stenosis, including diagnostic
1. test feed!!!
- examined after feeding
- peristalsis (rolling abdomen) seen
- palpable pyloric mass "feels like an olive" in the RUQ
2. blood gas analysis - hypochloric metabolic alkalosis
3. abdominal USS (diagnostic!)
117
management for pyloric stenosis
laparoscopic pyloromyotomy (known as "Ramstedt's operation")
incision made in pylorus to widen canal
118
what is gastroenteritis?
transient infection of the gut, characterised by sudden onset of diarrhoea
119
epidemiology of gastroenteritis
v common
cause of morbidity in younger children
in UK - 10% of children <5y annually
120
most common cause of paediatric gastroenteritis:
a) in developed countries
b) bacterial
c) parasitic
a) rotavirus
b) Campylobacter jejuni
c) Giardia and Cryptosporidium
121
RFs for paediatric gastroenteritis (2)
- travel
- close contact with other infected ppl
122
signs and symptoms of gastroenteritis
1. sudden change > loose, watery stools
2. often accompanied by vomiting
123
what is important to assess when a child comes in with gastroenteritis?
dehydration!
124
possible signs of dehydration in a child with gastroenteritis (6)
1. altered responsiveness e.g. irritable, lethargic
2. decreased urine output
3. sunken eyes
4. dry mucous membranes
5. reduced skin turgor
6. sunken fontanelles
125
investigations for gastroenteritis
normally none needed!
assess dehydration
126
what is the mainstay of management in children with gastroenteritis?
preventing and/or correcting dehydration
127
management of a child with gastroenteritis if there is:
a) no dehydration
b) clinical dehydration
c) shock
a) prevent dehydration - continue breastfeeding, encourage fluid intake to compensate
b) fluid deficit replacement (50ml/kg), maintenance fluid, continue breastfeeding, consider supplementing with oral rehydration solution (ORS)
c) IV therapy - bolus 0.9% sodium chloride then replace fluid deficit over 24hrs
128
what advice should be given to a parent who's child has gastroenteritis about returning to school?
do not return until 48 hours after last diarrhoea episode
129
which cardiac defects are associated with these genetic syndromes?
a) Down's syndrome
b) Edwards's syndrome
c) Patau's syndrome
d) Turner's
e) DiGeorge
f) Noonan syndrome
g) Marfan syndrome
a) AVSD
b) VSD
c) patent ductus arteriosus
d) bicuspid aortic valve, coarctation of the aorta
e) arch, truncal abnormalities
f) pulmonary valve stenosis
g) aortic root dilation
130
which septal defects are...
a) acyanotic
b) cyanotic?
a) VSD, ASD, AVSD, PDA
b) transposition of great arteries, tetralogy of fallot, severe Ebstein's anomaly
131
what is the role of the...
a) ductus venosus
b) foramen ovale
c) ductus arteriosus
in foetal circulation?
a) shunts oxygenated blood from the placenta in the umbilical vein towards the foetus' heart, bypassing the liver
b) shunts O2 blood from the RA to the LA (bypasses lungs)
c) connects pulmonary artery with the aorta (again bypassing the lungs)
132
briefly describe where O2 blood from the placenta and deoxygenated blood from the foetus' body travels in foetal circulation
1. blood from placenta > right heart > left heart > body
2. some to lungs BUT majority shunted through PDA > descending aorta (right heart)
133
what cardiac and respiratory changes occur in the foetus after birth?
1. baby's first breath
2. O2 is pulmonary dilator > pulmonary resistance falls allowing blood into lungs
3. foramen ovale closes (increased pul blood flow increases LA pressure and decreases RA pressure)
4. ductus arteriosus closes after a few days
5. aortic pressure rises
134
infusion of what helps to maintain ductal patency in neonates e.g. before surgery?
prostaglandin E1 (Alprostadil)
135
what is the most common cyanotic congenital heart disease?
tetralogy of fallot
136
4 cardiac features of tetralogy of fallot
PROV:
1. Pulmonary stenosis
2. Right ventricular hypertrophy
3. aorta Overriding the VSD
4. Ventricular septal defects
137
clinical features of tetralogy of fallot (4)
1. heart failure (before age 1)
2. tet spells
3. squatting
4. cynaotic - blue
138
investigations and key findings in tetralogy of fallot (3)
1. cardiac auscultation - HARSH SYSTOLIC EJECTION MURMUR over pulmonary and left sternal area
2. cxray - boot-shaped heart
3. echocardiogram (diagnostic)
139
how is tetralogy of fallot managed?
neonates: prostaglandin infusion (maintain ductus arteriosus allows blood to flow from aorta into pulmonary arteries)
surgery
beta-blockers
140
what is transposition of the great arteries (TGA)?
where the aorta is connected to the right ventricle and the pulmonary artery is connected to the left ventricle
O2 blood recirculates only in pulmonary circulation, deoxygenated systemic blood bypasses lungs
incompatible with life
141
what does survival depend on for infants born with TGA? why?
whether there is a PDA, VSD or ASD, as these will allow blood to mix
142
how does TGA present?
cyanosis in FIRST 24H
tachypnoea
tachycardia
failure to thrive
143
what is the main RF for TGA?
maternal diabetes
144
investigations and findings for TGA
bloods and gas - metabolic acidosis, decreases PaO2
CXR - 'egg on a string'
diagnostic = echo
145
management for TGA
emergency prostaglandin infusion and surgical correction
146
what is the most common septal defect?
ventricular septal defect (VSD)
147
what is VSD? what is it's pathophysiology? how does this explain why it's an acyanotic defect?
- hole in the septum between the R and L ventricle
- pressure is higher in L ventricle
- so blood shunts from L to right
- O2 blood then re-enters pulmonary circulation
= pulmonary OVERcirculation
- O2 blood still flowing around body but inefficient!
148
presentation of VSD and its classical sign on ausculation
- sweating
- fatigue
- poor weight gain
- tachypnoea/SOB
- on auscultation = pan systolic murmur at LLSE
149
investigations and findings for VSD
1. CXR - may have cardiomegaly, pulmonary oedema, plural effusion
2. echo (GOLD STD)
150
how is a VSD managed? when is surgical intervention indicated?
- MOST will close by themselves
- increase caloric intake
- diuretic (furosemide) to decrease fluid in circ
- ACE-Is
- digoxin
surgery if significant or causing complications later in life
151
what condition does VSD increase a child's risk of?
infective endocarditis
152
what is ASD?
atrial septal defect - hole between left and right atrium causing L>R shunt
153
why is ASD commonly asymptomatic?
because the L>R shunt is at at a lower pressure than in a VSD - less pul circulation overload
154
classical presentation of ASD on auscultation?
split double (split, fixed 2nd heart sound) + soft ejection systolic murmur
155
main RF for ASD
maternal rubella
156
if a child with ASD is symptomatic, how may they present?
chest pain, exercise intolerance
157
how is ASD diagnosed?
gold std = echo
158
how are ASDs <5mm managed?
- conservative: should close spontaneously within 12m of birth
- if heart probs, give diuretics e.g. furosemide
159
how are ASDs >1cm managed?
surgery
160
what is ASVD? what comorbidity is it strongly associated with?
- malformation of the atrioventricular septum connecting all 4 heart chambers
- essentially an ASD and a VSD
- strong association w Trisomy 21
161
signs and sx of AVSD
sx:
- tachypnoea
- poor feeding
- sweating
- failure to thrive
signs:
- hyperactive precordium
- thrill
- gallop rhythm
- HF > hepatomegaly and generalised oedema/crackles
162
definitive management of ASVD?
surgery!!!
lifelong FU needed
163
what is Eisenmenger's syndrome? what causes it to happen?
- if a septal defect e.g. VSD is left untreated
- constant L>R shunt
- increased strain on R side of heart > increased pressure on pulmonary artery > pulmonary HTN > increased pressure in RA > overcomes pressure in LA
- shunt REVERSES and becomes R>L
164
what will happen to a baby with Eisenmenger's syndrome
will become blue as septal defect has become cyanotic
165
what is coarctation of the aorta?
narrowing of the aorta distal to the left subclavian artery - at top of descending aorta
166
what pathophysiological changes occur as a result of coarctation of the aorta?
- severe obstruction of blood flow in descending aorta
- significant afterload on LV
- compensatory ventricular hypertrophy
167
how does coarctation of the aorta present?
relatively asymptomatic!
- headaches
- on exertion claudication and cool peripheries
168
investigations and findings for coarctation of the aorta (2)
1. chest xray- dilated aorta and rib knotching
2. ECG - left ventricular hypertrophy
169
how is coarctation of the aorta managed?
balloon angioplasty (opens aorta)
170
what is Ebstein's anomaly?
incorrect positioning (low insertion) of the tricuspid valve separating the R atrium and ventricle > blood leaks from V to A > LARGE A and SMALL V
171
most common cause of heart failure in children
congenital heart defects (CHD)
172
what can the causes of paediatric heart failure be split into?
overcirculation
pump failure
173
list some 'overcirculatory' causes of paediatric heart failure (4)
1. L>R shunt e.g. VSD, ASD, patent ductus arteriosus
2. parallel circulation e.g. transposition of the GAs
3. conditions causing increased cardiac output e.g. anaemia
4. mitral and aortic regurgitation
174
list some examples of 'pump failure' causes of paediatric heart failure (4)
1. aortic stenosis/pul stenosis
2. inflammatory e.g. myocarditis, HIV-related, Chaga's disease
3. dilated cardiomyopathy
4. rhythm disturbance e.g. complete heart block
175
signs and symptoms of HF in:
a) new-borns
b) infants
c) older/adolescents
a) nonspecific e.g. tachycardia, high RR
b) tachypnoea, feeding difficulties, irritability with feeding, sweating
c) fatigue, effort intolerance, dyspnoea, orthopnoea, abdominal pain, oedema, ascites
176
investigations and findings for paediatric HF (6)
1. bloods - BNP elevation
2. urine tests
3. CXR - enlarged heart
4. ECG
5. Echo - enlarged chambers
6. cardiac catheterisation
177
other than treating the underlying cause, how can paediatric HF be medically managed?
1. diuretics e.g. furosemide
2. inotropes e.g. epinephrin (increase contractility)
3. ACEis (reduce afterload)
4. digoxin
178
what is rheumatic fever?
an autoimmune systemic illness caused by an immunological reaction to a recent (2-4 weeks ago) Streptococcus pyogenes infection
179
who is most commonly affected by rheumatic fever?
children in developing countries
180
briefly describe the pathophysiology of rheumatic fever. what antibodies are involved?
1. strep A releases toxins including M proteins
2. B cells produce anti-M protein antibodies
3. these antibodies cross-react with other tissues e.g. heart, brain, joints and skin
181
what is the name of the granulomatous nodules found in rheumatic heart fever?
Aschoff bodies
182
what ALWAYS precedes the signs and symptoms seen in rheumatic fever?
a recent (2-4w) infection with strep A e.g. sore throat/scarlet fever
183
how is a diagnosis of rheumatic fever made?
evidence of recent strep infection accompanied by...
- 2 major criteria
- 1 major with 2 minor criteria
184
what are the minor and major criteria for rheumatic fever?
MINOR (RAPP)
- Raised ESR/CRP
- Arthralgia
- Prolonged PR interval
- Pyrexia
MAJOR (SPECS)
- Sydenham's chorea (often late)
- Polyarthritis
- Erythema marginatum (bright pink/red circular lesions with defined borders and central clearing)
- Carditis and valvulitis
- Subcutaneous nodules
185
what is sydenham's chorea?
a neurological complication of rheumatic fever characterised by rapid, uncoordinated jerking movements affecting the face, hands and feet
186
management of rheumatic fever (3)
1. oral abx 1st line = benzathine benzylpenicillin
2. anti-inflammatories - NSAIDs
3. tx of complications e.g. HF
187
how does the frequency of stool passage change from infants under 6m to children older than 3y?
3x per day for infants, decreases to 1x a day after 3y
188
what is encopresis?
involuntary faecal soiling/incontinence secondary to chronic constipation
189
pathogenesis 'cycle' of functional constipation in children (4)
1. painful defecation
2. voluntary withholding
3. prolonged fecal statis, re-absorption of fluids, increased size and consistency of stools
4. more pain
and then the cycle continues
190
NICE guidelines suggest a diagnosis of constipation can be made when 2 or more of the following are present in children <1 year...
1. stool pattern
- <3 stools a week
- hard large stool
- rabbit droppings
2. symptoms with defecation
- distress
- bleeding
- straining
3. history
- prev constipation
- prev/current anal fissure
191
NICE guidelines suggest a diagnosis of constipation can be made when 2 or more of the following are present in children >1 year...
1. stool pattern
- <3 stools a week
- overflow soiling
- rabbit droppings
- large infrequent stools that block toilet
2. symptoms with defecation
- poor appetite improving with passage
- abdo pain
- retentive posturing e.g. straight-legged, tiptoeing, back arching
- straining
- anal pain
3. history
- prev constipation
- prev/current fissure
- painful bowel movements + bleeding
192
causes of constipation in children (9)
1. dehydration
2. low-fibre
3. medications e.g. opiates
4. anal fissure
5. over-enthusiastic potty training
6. hypothyroid
7. Hirschsprung's
8. hypercalcemia
9. learning disabities
193
investigations are often unnecessary for constipation, but when may they be indicated and what would they be?
IF organic cause suspected/child remains constipated despite medical tx
- bloods (TFTs, serum calcium, coeliac panel)
- sweat test (CF)
- abdo xray
- anal mamometry
- rectal biopsy
- spinal imaging (if neuro cause suspected)
194
what constipation 'red flags' suggest an underlying disorder?
a) timing
b) passage of meconium
c) stool pattern
d) growth
e) neuro/locomotor
f) abdomen
g) other
a) reported from birth/first few weeks of life
b) >48h
c) 'ribbon' stools
d) faltering growth
e) previously unknown/undiagnosed weakness in legs, locomotor delay
f) distension
g) concern of child maltreatment
195
what presentation suggests idiopathic constipation as opposed to an underlying disorder?
a) timing
b) passage of meconium
c) stool pattern
d) growth
e) neuro/locomotor
f) abdomen
g) diet
a) starts AFTER first few weeks of life
b) <48h
c) not ribbon
d) generally well, weight and height ok
e) no neuro or locomotor probs
f) no distension
e) changes in infant formula, weaning, insufficient fluid or diet intake
196
what does a child need to be assessed for prior to starting constipation tx?
faecal impaction
197
factors suggesting faecal impaction
1. sx of severe constipation
2. overflow soiling
3. faecal mass palpable in abdomen
198
tx of constipation if faecal impaction is present
first line = polyethylene glycol + electrolytes (Movicol Paediatric Plain)
199
what laxatives can be used to treat paediatric constipation?
osmotic laxatives e.g. Movicol Paediatric Plain, stimulant laxatives e.g. Senna, other e.g. lactulose if not working
200
non-medical tx for constipation
- do NOT use dietary interventions alone
- regular toileting and behavioural interventions e.g. reward scheme
201
management options for constipation in very young children
a) not yet weaned (<6m)
b) weaned
a) give extra water in between feeds, abdominal massages, bicycling legs
b) offer extra water, diluted fruit juices and fruits (if not effective consider lactulose)
202
what is the most common cause of abdominal pain in children requiring surgery
appendicitis
203
uncomplicated vs complicated appendicitis?
complicated - inflammation of appendix without signs of necrosis/perforation
uncomplicated - inflammation progressed to focal or transmural necrosis and/or perforation
204
3 common causes of appendicitis in children
1. faecalith (hardened lump of poop)
2. lymphoid hyperplasia (dense collections of lymphocytes, reaches max size in adolescence)
3. pinworm infection
205
pathophysiology of appendicitis
1. obstruction to entrance of appendix
2. but appendix continues secreting mucus/fluids from intestinal lumen mucosa
3. fluid and mucus builds up
4. pushes on afferent nerve fibres
5. flora and bacteria in appendix trapped
6. WBCs invade > pus builds up
206
presentation of uncomplicated appendicitis
- loss of appetite
- nausea and vomiting
- low-grade fever
- abdominal pain: initially central and colicky, then localising at right iliac fossa (McBurney's)
- pain aggravated by movement
207
presentation of complicated appendicitis
- appendix mass
- generalised guarding (perforation)
- fever
- weakness
- rebound tenderness
208
investigations for appendicitis including 1st line imaging
1. bloods
- FBC (raised WCC)
- U&Es (may be deranged)
- CRP (raised)
2. urinalysis (rule out UTI)
3. first-line imaging = ultrasound
209
signs of appendicitis on USS (3)
1. non-compressible appendix (6mm diameter)
2. wall thickening
3. may see appendicolith (hard lump made of minerals and faeces)
210
ddx for paediatric appendicitis
1. mesenteric adenitis (usually preceded by sore throat)
2. meckel's diverticulitis (rectal bleeding)
3. gastroenteritis (more general pain)
4. UTI
211
management of uncomplicated and complicated appendicitis
uncomplicated - appendicectomy
complicated - IV fluids, appendicectomy, surgical drainage
for BOTH - laparoscopic approach, pre-operative abx therapy
212
complications of appendicitis (2)
1. perforation
2. abscess formation
213
which hernia types are the most common in children?
inguinal and umbilical
214
a) when do inguinal hernias present?
b) in who?
a) infancy, mostly appear in first 6m of life
b) mostly boys
215
what causes inguinal hernias in children?
failure of the processus vaginalis to close once the testes have moved through the inguinal canal into the scrotum
216
RFs for
a) inguinal hernia
b) umbilical hernia
a) family hx
b) down's syndrome, prematurity, familial tendency
217
presentation of an inguinal hernia
bulge or swelling in groin
- in boys, may be seen in scrotum
- may only be seen during crying/straining
218
presentation of an umbilical hernia
- bulge at umbilicus, size of pea-plum
- painless
- v obvious when crying/straining
219
investigations for paediatric hernias
normally clinical diagnoses
220
which children presenting with an inguinal hernia require emergency surgery? why?
children that present in first few months of life - due to risk of strangulation
ALSO those with an irreducible hernia
221
which children presenting with an inguinal hernia are at lower risk of strangulation? so what is the management?
children >1 year old
can have elective (rather than emergency) surgery
222
how are umbilical hernias usually managed?
- 95% close spontaneously
- surgery rarely required as strangulation rare
223
when is surgery indicated for umbilical hernias?
IF pain in hernia OR child is age >4
224
what is infective endocarditis?
infection of the endocardium (lining of the heart), often involving the heart valves
225
which children is IE commonly seen in?
those with hx of congenital/acquired heart disease e.g.
- ventricular septal defects
- patent ductus arteriosus
- TOF
226
give 2 common bacterial causes of IE
1. staphylococcus aureus
2. streptococcus viridans
227
which bacteria are associated with causing IE following:
a) dental procedures
b) GU/GI surgery
a) strep viridans
b) enterococci
228
presentation of IE
with a history of CHD/cardiac surgery/prosthetic material...
1. persistent low grade fever
2. heart murmur/change in murmur
3. splenomegaly
(cutaneous manifestations uncommon in paeds)
229
what are the 2 main investigations of choice for IE?
1. blood cultures - multiple samples from multiple sites
2. ECHO - identifying vegetations
230
which criteria is used to diagnose IE?
Modified Duke's Criteria
231
what combo of major/minor criteria must be met for IE to be diagnosed?
either:
2 major
1 major + 3 minor
5 minor
232
major Duke's criteria for IE diagnosis (2)
1. positive blood culture
2. positive ECHO findings of vegetation
233
minor Duke's criteria for IE diagnosis (6)
1. predisposition - heart condition
2. fever >38
3. vascular phenomena e.g. arterial emboli, conjunctival haemorrhage
4. immunologic factor e.g. glomerulonephritis, RF
5. positive blood culture not consistent with IE organisms
6. positive ECHO not meeting major criteria
234
examples of ECHO findings suggesting tx of IE needs to be surgical (3)
1. vegetation >15mm
2. valve perforation/rupture
3. heart failure
235
medical tx for IE if bacteria cause is...
a) streptococci
b) staph
c) penicillin resistant
a) IV benzylpenicillin for 4 weeks
b) IV fluclox
c) vancomycin + gentamicin
236
what is the infective endocarditis pathophysiological triad?
1. endothelial damage
2. platelet adhesion
3. microbial adherence
237
brief pathophysiology of coeliac disease
1. repeated exposure to protein gluten
2. inflammation and villous atrophy of small bowel mucosa
3. malabsorption
238
when do children normally present with coeliac disease? why?
normally before 3 years old, following intro of gluten into the diet
239
which genes are strongly associated with coeliac disease? (2)
1. HLA-DQ2
2. HLA-DQ8
240
presentation of coeliac disease in children ("classic" and "common")
"classical"
- faltering growth/failure to thrive
- abdominal distension
- diarrhoea
- foul-smelling, floating stools
- irritability
"common"
- mild, non specific GI sx
- anaemia: fatigue, pallor
- growth faltering
241
1st line and gold std investigations for coeliac disease
1st line = bloods
- serology IgA-TTG blood test (+ve)
- FBC and blood smear (low Hb, folate, ferritin, B12)
gold std = endoscopic jejunal/duodenal biopsy and histology
242
what will be seen on histology of the duodenum in a child with coeliac disease?
villous atrophy, crypt hyperplasia, intraepithelial lymphocytes
243
what is there a slight increased risk of in coeliac children who don't adhere to a gluten-free diet?
small bowel lymphoma
244
define failure to thrive
what are the NICE definitive guidelines in terms of falls in weight if birth weight was:
a) below the 9th centile
b) between the 9th and 91st centile
c) above the 91st centile
poor physical growth and development
a) fall in weight across one or more centile spaces
b) fall in weight across two or more centile spaces
c) fall in weight across three of more centile spaces
245
what are the 5 categories for causes of failure to thrive?
1. inadequate nutritional intake
2. difficulty feeding
3. malabsorption
4. increased energy requirements
5. inability to process nutritiom
246
inadequate nutritional intake causes of failure to thrive
1. maternal malabsorption if breastfed
2. Fe deficiency anaemia
3. family/parental problems
4. neglect
5. availability of food ie poverty
247
difficulty feeding causes of failure to thrive
1. poor suck e.g. due to cerebral palsy
2. cleft lip/palate
3. genetic e.g. abnormal facial structure
4. pyloric stenosis
248
malabsorption causes of failure to thrive
1. CF
2. coeliac
3. cow's milk intolerance
4. chronic diarrhoea
5. IBD
249
inability to process nutrients causes of failure to thrive
1. type 1 diabetes
2. metabolic errors
250
which key areas need to be assessed when investigating failure to thrive?
1. pregnancy, birth, development and social hx
2. feeding/eating history - breast/bottle, feeding times, volume, food choices, aversion, meal time routines, appetite
3. observe feeding
4. mums physical and mental health
5. parent-child interactions
6. growth chart plot and mid-parental height centile
251
initial investigations for a child failing to thrive
1. urine dipstick
2. coeliac screen
252
management of failure to thrive if caused by difficulty breastfeeding
- support from midwife, health visitors, peer groups
- supplement with formula
253
management of failure to thrive if inadequate nutrition is the cause
- encourage regular structured meals and snacks
- reduce milk consumption to increase appetite for other foods
- review by dietician
- additional energy dense foods
- nutritional supplement drinks
254
define...
a) Crohn's
b) UC
a) a transmural (across whole wall), focal, subacute or chronic inflammatory disease most commonly affecting the distal ileum and proximal colon
b) a recurrent, inflammatory and ulcerating disease involving the mucosa of the colon
255
risk factor for paediatric IBD
family history
256
pathophysiology of Crohn's:
a) location
b) type of inflammation
c) continuous or discontinuous?
d) granulomas or not?
e) rectal involvement
f) gut complications
g) classic associating condition
a) mouth to anus (most common is distal ileum and prox colon)
b) transmural inflammation
c) discontinous
d) granulomas
e) rectal sparing
f) fissures, fistulas, abscesses and strictures COMMON
g) perianal disease
257
pathophysiology of UC:
a) location
b) type of inflammation
c) continuous or discontinuous?
d) granulomas or not?
e) rectal involvement
f) gut complications
g) classic associating condition
a) colon only - in children, most common is pancolitis (whole colon)
b) mucosal inflammation
c) continuous
d) NO granulomas
e) NO rectal sparing
f) abscesses and strictures RARE
g) primary sclerosing cholangitis (PSC)
258
signs and symptoms of UC in children
(in children, sometimes persistent poor growth is only sign of disease)
1. rectal bleeding
2. diarrhoea
3. colicky pain
4. extra-intestinal - erythema nodosum, arthritis
259
signs and symptoms of Crohn's in children
(sometimes in children persistent poor growth is only sign of disease)
1. delayed puberty
2. classic presentation = abdominal pain, diarrhoea and weight loss
3. general ill health e.g. fever, lethargy
4. extra-intestinal - oral lesions, perianal skin tags, uveitis, arthralgia, erythema nodosum
260
investigations (including gold std) for IBD
1. bloods - FBC (may be anaemic), LFTs, ESR and CRP (raised)
2. stool microscopy and culture - rule out infection
3. faecal calprotectin (faecal inflamm marker, raised)
4. gold std = colonoscopy with biopsy and histology
261
what will be seen on histology of the colon in:
a) Crohn's
b) UC
a) granulomas, skip lesions, cobblestone appearance, fistula formation
b) mucosal inflammation, crypt abscesses and ulceration
262
ddx for paediatric IBD
- gastroenteritis
- coeliac disease
- IBS
- lactose intolerance
263
managing paediatric crohn's:
a) inducing remission
b) maintaining remission
a) 1st line = corticosteroid therapy, fluids
2nd line = immunosuppression e.g. mesalazine or bio agents e.g. infliximab
b ) first line = azathioprine (monotherapy)
alternative = methotrexate
264
what medication should be avoided in acute IBD attacks? why?
anti-motility drugs e.g. loperamide - can precipitate toxic megacolon
265
when is surgery indicated in paediatric crohns? give some surgical options
for those with failed medical management or severe complications e.g. strictures, perforation
options include:
- ileocaecal resection
- small bowel resection
- perianal disease surgery
266
GI and extraintestinal complications of crohn's
GI
- fistula e.g. rectovaginal
- strictures
- recurrent perianal fistula
- malignancy
extra
- malabsorption and osteoporosis secondary to this
- gallstones and renal stones
267
what investigation should not be perforemd during an acute chron's flare? why?
colonoscopy = risk of perforation
268
medical management of mild-moderate UC (induction and maintenance)
induction - mesalazine
maintenance - mesalazine
269
medical management for moderate-severe UC (induction and maintenance)
induction - corticosteroids
maintenance - anti-TNF e.g. infliximab OR azathioprine
270
hospital management of an acute severe UC flare (3)
1. IV corticosteroids
2. fluid resus
3. prophylactic heparin and stockings (due to prothrombotic state of IBD flares)
271
lifestyle/general management for paeds IBD
refer to IBD nurse specialists, consider enteral nutritional support if growth concerns, consider low residue diet
272
complications of UC (3)
1. toxic megacolon
2. colorectal adenocarcinoma
3. pouchitis (inflammation of ileal pouch following surgery)
273
define marasmus
how does it differ from Kwashikor
marasmus = acute malnutrition as a result of insufficient intake of protein AND calories
it is total calorie insufficiency, as opposed to Kwashikor which results from sufficient caloric intake but insufficient protein
274
aetiology and RFs of marasmus
aetiology - poverty, biological e.g. HIV, AIDs, infectious disease
RFs - low social status, no maternal education, war/natural disaster
275
general presentation of marasmus
in children
in infants
1. overt loss of adipose tissue and muscle
2. weight-for-height value LOW
3. failure to thrive
4. infants - irritability, apathy, sunken fontanelles
276
what signs of vitamin deficiency may a child with marasmus present with? (4)
1. vit A - dry eyes, Bitot spots (keratin deposits in eye)
2. Fe deficiency anaemia - koilonchyia, glossitis
3. hypocalcemia - Chvostek/Trousseau signs
4. calcium and vit D - rickets, deformities long term
277
3 steps for managing marasmus
1. resus and stabilisation - rehydrate, prevent infection, avoid refeeding syndrome
2. nutritional rehabilitation - gradual increase in intake, vaccines
3. FU and prevention of recurrence
278
what would be the visible differences seen in a child with marasmus vs kwashikor?
marasmus - loss of muscle mass, thin
kwashikor - ascites, moon face
279
what is kwashikor? what is it characterised by?
severe acute malnutrition characterised by severe protein deficiency, causing fluid retention (bilateral pitting oedema in absence of another medical cause)
280
RFs for kwashikor
1. monotonous corn based diet
2. recent weaning
3. recent infection e.g. measles
4. diarrhoea illness
5. rural communities, Africa
5. endemic food insecurity/famine
281
under what circumstances may kwashikor present in developed countries?
in association with TB, HIV or severely restricted diet e.g. in autism and behavioural disorders
282
classical presentation of kwashikor
1. weight loss
2. muscle wasting
3. OEDEMA of belly (ascites), feet and ankles
283
investigations for marasmus/kwashikor
primarily clinical diagnosis but can do it to look for underlying conditions/rule out dix
urine - rule out nephrotic syndrome and GN (will be no proteinuri/haemturia)
FBC - low Hb
U&Es - deranged
serum protein and albumin - low/normal
blood glucose — hypoglycemia
CXR - TB may co-exist
urine, blood, stool cultures and malaria screen
284
management options for kwashikor
if in community - ready-to-use therapeutic food (RUTF), broad spec abx e.g. amoxicillin
in hospital - regular milk-based liquid food, emperical abx, vitamin A supplementation
285
risk factor for IBS in children
parental hx
286
which age group is most commonly affected by IBS in the paediatric population?
teens
287
brief pathophysiology of IBS
altered gut reactivity (motility and secretion) due to environmental effects like stress/certain foods
288
signs and sx of IBS
ABC
A - abdo discomfort
B - bloating/distension
C - change in bowel habit
Other
- mucus in stool
- loss of appetite
- nausea
- altered stool passage e.g. straining, urgency, incomplete evacuation
289
signs and sx of IBS
ABC
A - abdo discomfort
B - bloating/distension
C - change in bowel habit
Other
- mucus in stool
- loss of appetite
- nausea
- altered stool passage e.g. straining, urgency, incomplete evacuation
290
investigations for IBS
diagnosis of EXCLUSION:
1st line = bloods
- FBC
- ESR and CRP
- coeliac serology
stool sample e.g. faecal cal protection
abdo imaging
upper endoscopy/colonoscopy if IBD suspected
291
management of non-severe IBS (2)
1. dietary modifications (limiting lactose, probiotics, high fibre NOT indicated in paeds)
2. stress management
292
management of severe IBS
medical tx, same as adults:
1. pain/bloating - antispasmodic e.g. buscopan
2. constipation - laxative e.g. senna
3. diarrhoea - antimotiltiy e.g. loperamide
293
what is Hirschprung’s disease?
a congenital condition of the colon, leading to functional obstruction
294
aetiology/pathophysiology of Hirschsprung’s disease
1. ganglion cells in the colon fail to develop (normally in rectosigmoid area)
2. lumen tonically contracted
3. peristalsis halts
4. obstruction
295
when is Hirchsprung’s commonly diagnosed?
within the first year of life, most commonly first few DAYS
296
risk factors and other conditions associated with Hirschsprung’s disease
associated with Down’s syndrome, multiple endocrine neoplasia
RF = male sex, genetics e/g/ Trisomy 21, sibling with HSD
297
how does Hirschsprung’s disease present?
1. failure to pass meconium in first 24 hours
2. abdo distension
3. bilious vomiting (bright green)
4. failure to thrive
other = constipation, explosive passage of liquid/foul stools, fever
298
1st line and gold standard investigations for Hirschsprung’s
1st line = abdominal x-ray (can also do contrast enema)
gold std = rectal biopsy
299
what findings would indicate Hirschsprung’s disease on:
a) x-ray
b) contrast enema
c) rectal biopsy
a) distended colon (automatically ruled out if no distension) - dilated loops of bowel with fluid levels
b) contracted distal bowel, dilated proximal bowel, transition zone
c) absence of ganglion cells, thickened unmyelinated nerves, increased Ach activity
300
differential diagnoses for Hirschsprung’s disease (5)
1. intussusception
2. constipation
3. pyloric stenosis
4. CF (meconium ileus)
5. small left colon syndrome
301
what is the classical triad of Hirschsprung’s disease?
1. failure to pass meconium
2. abdominal distension
3. bilious distension
302
initial and definitive management of Hirschsprung’s disease
initial = IV abx, NG tube insertion and bowel decompression
definitive = surgical
303
what is a serious complication of Hirschsprung’s disease surgery?
enterocolitis (infection)
304
what are the two surgical options for Hirschsprung's?
1. single-stage pull through procedure (single-stage RESECTION)
2. 3-stage procedure
305
what is colic defined as?
excessive crying in the first few months of life
306
epidemiology of colic
- how common?
- when is prevalence highest?
- V COMMON
- prevalence highest in <6 weeks, generally common <12m
307
aetiology of colic
unknown - possibly to do with gas production/abnormal motility and pain signalling/inflammation
308
RFs for colic (4)
1. family tension
2. parental anxiety/depression
3. inadequate parent-infant interaction
4. misinterpretation of crying
309
what are the Rome IV diagnostic criteria for colic? (2)
1. infant <5m when symptoms start AND stop
2. recurrent, prolonged periods of infant crying/fussing/irritability with non-obvious causes and cannot be settled by caregivers
310
other signs of colic
- crying in late afternoon/evening
- fist clenching
- drawing knees to abdomen
- arching back when crying
311
what types of crying are red flags when diagnosing colic? (2)
1. weak, continuous cry
2. abnormally high-pitched cry
312
how should colic be investigated?
full examination to rule out ddx - observations, weight plotting, physical exam, observe carer interactions, maternal breast exam
if no abnormalities besides inconsolable crying, further ix not indicated
313
ddx for colic
a) acute (6)
b) chronic (5)
a) - intussusception
- pyloric stenosis
- incarcerated/strangulated hernia
- trauma/NAI
- sepsis
- hydrocephalus
b) - hunger/dehydration
- inadequate winding
- constipation
- GORD
- non-IgE mediated food allergy
314
management for colic including strategies and advice for parents/carer
1. SELF-LIMITING - normally resolves by 5 months
2. reassurance
3. strategies - hold baby when crying, gentle motion, white noise, warm bath, optimise winding technique
4. advise - continue breastfeeding, rest when baby is asleep, reassure baby can be left in safe place if carer unable to cope
315
when is a referral re colic indicated?
1. parent/carer unable cope despite reassurance/advice
2. suspected faltering growth
3. symptoms persist >5m
4. suspected underlying cause
316
complications of colic
- child maltreatment
- early breastfeeding cessation
- family/relationship difficulties
- carer sleep deprivation/depression/fatigue/stress etc
317
what is cow's milk protein allergy (CMPA)? which proteins is cow's milk made up of?
multisystem disease caused by an adverse immune reaction to one or more cow milk proteins (caseins and whey)
318
before what age do most cases of CMPA present by?
1 year old
319
what are the two types of CMPA? give a brief pathophysiology
1. IgE-mediated
- production of specific IgE antibodies against the cow's milk proteins
- exposure to CM proteins > release of cell mediators e.g. histamines
2. Non-IgE mediated
- T-cell mediated response
320
causes of CMPA (3)
1. atopy
2. IgA deficiency
3. IgG subclass abnormalities
321
RFs for CMPA (3)
1. atopy (comorbid asthma, atopic eczema)
2. family hx of food allergy or atopy
3. male sex
322
symptoms of CMPA present up to X hours following cow's milk ingestion
2 hours
323
IgE mediated CMPA symptoms (GI, skin, resp)
GI
- oral pruritis
- nausea, vomiting and diarrhoea
- colicky abdo pain
skin
- pruritis
- erythema
- acute, localised urticaria
- acute angioedema (eyes, lips, face)
resp
- URT e.g. nasal itching, sneezing, congestion, conjunctivitis
- LRT e.g. cough, tight chest, wheeze, SOB
324
Non-IgE mediated CMPA symptoms (GI, skin, resp)
GI
- GORD
- loose/frequent stools
- blood/mucus in stools
- abdo pain
- colic
- food refusal/aversion
- perianal redness
- pallor and tiredness
- faltering growth in conjunction with one or more above
skin
- pruritis
- erythema
- atopic eczema
resp
- JUST LRT sx e.g. cough, tight chest, wheeze, SOB
325
investigations for CMPA - what is diagnostic?
1. allergy testing e.g. skin prick, serum-specific IgE test
2. diagnostic = ELIMINATION DIET
326
ddx for CMPA
GORD
327
management of CMPA in a formula-fed infant (first line and alternative)
first line replacement = extensive hydrolysed formula (EHF) milk
if no response/severe CMPA - give amino-acid based formula (AAF)
328
management of CMPA in a breast-fed infant
1. continue breastfeeding
2. eliminate cow's milk protein from maternal diet
3. consider prescribing calcium supplements to breastfeeding mums
4. use eHF milk when breastfeeding stops
329
at what age do children often become milk tolerant after having:
a) Ige mediated CMPA
b) non-IgE mediated CMPA
a) by 5 years old
b) by 3 years old
330
what is biliary atresia?
a rare liver disease of newborn babies where a section of the bile duct is either narrowed or absent
331
when can biliary atresia develop?
in utero OR in neonatal period
332
aetiology of biliary atresia
idiopathic
333
RFs for biliary atresia (3)
1. genetics
2. viral infection
3. immune dysregulation
334
pathophysiology of biliary atresia - which type of bilirubin is involved?
1. inflammation of biliary tree > discontinuous/obliterated bile ducts
2. ducts get blocked
3. results in cholestasis > bile cannot be transported from liver to bowel
4. bile builds in liver
5. prevents excretion of CONJUGATED bilirubin
335
signs of biliary atresia
SHORTLY AFTER BIRTH...
1. significant and persistent jaundice
2. pale stools +/- dark urine
3. failure to thrive
4. weight loss
5. ascites
6. hepatomegaly
7. bruising
336
investigations for biliary atresia - what is gold std?
1. bloods
- serum total bilirubin
- serum conjugated bilirubin (RAISED)
- FBC
- LFTs (deranged)
2. new-born screen - rule out CF
3. gold std - abdominal USS
337
what may be seen on abdo USS in biliary atresia?
- potential hepatosplenomegaly
- absent/multiple spleen
- ascites
- triangular cord sign
338
differential diagnoses for biliary atresia
- other biliary obstruction e.g. tumour
- hepatic viral infection e.g. CMV, HSV, hepatitis B
339
what defines persistent jaundice in...
a) term babies
b) premature babies
a) > 14 days
b) > 21 days
340
what is the first-line surgical management for biliary atresia?
Kasai procedure (replumbing bile duct into bowel)
341
definitive management for biliary atresia
liver transplant
342
what is toddler's diarrhoea?
- chronic (>14 days) NON SPECIFIC diarrhoea
- functional aka child is otherwise healthy
343
what is the most common cause of chronic diarrhoea in children aged 1-5?
toddler's diarrhoea
344
signs and symptoms of toddler's diarrhoea - what distinguishes it from pathological chronic diarrhoea?
- abnormal defecation frequency/consistency
- foul-smelling, watery, contains mucus with undigested food
- are NOT failing to thrive, have good nutritional state
- short mouth-anus transit time
- minority may have abdo pain
345
how is toddler's diarrhoea diagnosed?
clinically - thorough history and physical examination will be unremarkable
346
what is the difference between acute vs chronic diarrhoea?
acute = <14 days
chronic = >14 days
347
how is toddler's diarrhoea managed? what age can you reassure parents it will go away by?
reassurance - normally self-limiting, most cases go away by age 5
348
what is a choledochal cyst?
a rare congenital disorder where there are cystic dilatations at single/multiple segments of the biliary tree
349
what population is predominantly affected by choledocal cysts?
asian children
350
RFs for choledocal cysts (3)
1. female sex
2. young age
3. asian
351
brief pathophysiology of choledocal cysts
bile flow is blocked > builds up in liver
352
signs and symptoms of choledocal cysts
what does it present similarly to?
presents similarly to biliary atresia
triad of jaundice, abdominal pain and palpable abdominal mass
infants <12m may have vomiting and pale stools
353
3 imaging investigations for choledocal cysts - what is gold std?
1. abdominal USS
2. CT choliangiography
3. gold std = magnetic resonance cholangiopancreatography (MRCP)
354
management of choledochal cysts
surgery - cyst resection and restoration of bile flow
355
ddx for choledochal cysts
biliary atresia
356
what is neonatal hepatitis syndrome?
inflammation of the liver occurring in early infancy (1-2m) caused by a range of diseases
357
what are the categories of causes of neonatal hepatitis syndrome? (9)
1. congenital infection (MOST COMMON)
2. anatomical/structural
3. metabolic
4. genetic
5. neoplastic
6. vascular
7. toxic
8. immune
9. idiopathic
358
which congenital infections cause neoplastic hepatitis syndrome?
TORCH
toxoplasmosis
rubella
cytomegalovirus
herpes simplex
359
which is the most common TORCH infection causing neonatal hepatitis syndrome?
herpes simplex virus
360
anatomical/structural causes of neonatal hepatitis syndrome
1. biliary atresia
2. cholechodal cyst
3. neonatal sclerosing cholangitis
4. spontaneous biliary perforation
361
metabolic causes of neonatal hepatitis syndrome (2)
1. alpha 1-antitrypsin deficiency (A1AT)
2. CF
362
genetic causes of neonatal hepatitis syndrome (2)
trisomy 21 and 18
363
immune causes of neonatal hepatitis syndrome
lupus
364
presentation of neonatal hepatitis syndrome (3)
1. jaundice
2. failure to thrive
3. enlarged liver/spleen
365
what signs would indicate the cause of neonatal hepatitis syndrome being a TORCH infection?
jaundice in FIRST 24h of life
pneumonitis
petechial/purpuric rash
poor intrauterine growth
366
1st line investigation and finding for neonatal hepatitis syndrome
serum conjugated bilirubin - high
367
gold standard investigation for neonatal hepatitis syndrome - what is the hallmark finding?
liver biopsy - giant cell hepatitis (large multinucleated hepatocytes)
368
other than bloods and liver biopsy, what investigation can be done for neonatal hepatitis syndrome?
viral PCR - may identify congenital infection
369
management options for neonatal hepatitis syndrome if cause is
a) toxic
b) anatomical/structural
c) viral
a) dietary manipulation to remove toxins
b) surgery to relieve obstruction
c) self-limiting
370
what is the latin name for threadworms?
Enterobius vermicularis
371
what is alprostadil?
a prostaglandin (used to maintain ductus arteriosus before CHD surgery)
372
1st line tx for threadworms
single dose oral mebendazole for the entire family, hygiene advice
373
define intussusception - where do intestinal contents get stuck?
the prolapse of one part of the intestine into the lumen of the adjoining distal part
intestinal contents are retained in distended bowel above the intussusception
374
what is the most common cause of intestinal obstruction after the neonatal period?
intussusception
375
when is the peak presentation of intussusception? in which gender is it more common?
- 3 months-2 years
- boys
376
RFs for intussusception (2)
1. Meckel's diverticulum
2. polyps
377
pathophysiology of intussusception - where does it often happen?
1. often ileum (end of small intestine) passing into caecum (start of large intestine) through ileocaecal valve
2. retention of GI products above intussusception
3. fluid can pool in gut > hypovolaemia
4. blood supply to gut compromised
378
presentation of intussusception
1. paroxysmal (spasms) of severe colicky abdo pain with pallor
2. drawing up legs between episodes
3. bloodstained stool - becomes 'redcurrant jelly' later on
4. bile-stained vomit
5. refusing feeds
6. abdominal distension
379
what may be found on abdominal examination in intussusception?
palpable sausage-shaped mass
380
gold standard investigation for intussusception and what will be seen
abdominal ultrasound - target/bull's eye sign!
381
what will be seen on abdo x-ray in intussusception?
- distended small bowel
- absence of gas in distal colon/rectum
382
ddx for intussusception (6)
1. pyloric stenosis
2. appendicitis
3. gastroenteritis
4. testicular torsion
5. volvulus
6. colic
383
initial tx for intussusception
if shock/dehydration - IV fluid resus
384
management options for intussusception
a) if no peritonitis
b) if first line unsuccessful/signs of peritonitis
NG tube to decompress bowel THEN...
a) air/contrast enema (reverses it)
b) surgery required
385
complications of intussusception (4)
1. bowel perforation
2. bowel necrosis
3. peritonitis
4. sepsis
386
what is Meckel's diverticulum?
a congenital diverticulum (pouch) of the small bowel
387
what is a meckel's diverticulum a remnant of?
the omphalomesenteric/vitelline duct (supposed to obliterate during foetal development)
388
rule of 2s for Meckel's diverticulum...
- 2% population
- 2 feet from ileocaecal valve
- 2 inches long
389
at what age do children commonly present with Meckel's diverticulum?
< 2 years
390
how does Meckel's diverticulum normally present?
asymptomatic!
391
if Meckel's diverticulum is symptomatic, how does it present?
- abdominal pain (mimicking appendicitis)
- bright red, painless rectal bleeding (haematochezia)
- intestinal obstruction
- N&V
392
commonest cause of painless GI bleeding in children aged 1-2
Meckel's diverticulum
393
two abdominal complication related to Meckel's?
intussusception and volvulus
394
what may show in a FBC in meckel's diverticulum?
low Hb/haematocrit (due to bleeding)
395
diagnostic scan for meckel's diverticulum if pt is haemodynamically stable and has less severe bleeding
Meckel's scan - technetium-99m pertechnetate scan
396
what investigation should be done for Meckle's diverticulum if the child is more severely bleeding?
mesenteric arteriography
397
ddx for Meckle's
appendicitis
398
management of Meckel's diverticulum if...
a) asymptomatic
b) symptomatic
a) no tx required
b) SURGERY
if just bleeding - excision of diverticulum
if perforation/peritonitis - excision and/or bowel resection
399
how common are innocent heart murmurs? what are they caused by?
- common! about 30% of children
- due to febrile illness/anaemia, caused by increased cardiac output
400
what are the 5s's of innocent murmur presentation?
Soft, blowing/whooshing murmur
Systolic
Sitting/lying (varies with body position)
aSymptomatic
left Sternal edge (Still's murmur)
401
what will not be present in an innocent murmur?
any thrills or added sounds
402
what is mesenteric adenitis? what is primary and secondary?
inflammation of the lymph nodes found in the mesentery
primary - no obvious underlying inflamm cause
secondary - other underlying inflamm conditions
403
secondary causes of mesenteric adenitis (acute and chronic)
acute
- appendicitis
- other infections e.g. EBV
chronic
- IBD
- lupus
- sarcoidosis
- malignancy
- HIV
- TB
404
where is the lymphadenopathy located in mesenteric adenitis? when may it occur?
- terminal ileum
- may occur after bacterial infection e.g. gastroenteritis > bacteria travel into mesentery lymph nodes which causes localised inflammation
405
signs and symptoms of mesenteric adenitis (4)
v similar to others e.g. appendicitis
1. abdominal pain - severe, colic-like, typically right iliac fossa (terminal ileum) and/or periumbilical
2. fever
3. vomiting
4. change in bowel habit
406
1st line and gold std investigations for mesenteric adenitis
1st line = bloods (FBC, CRP), urinalysis
gold std = USS abdomen
407
ddx for mesenteric adenitis (5)
1. appendicitis
2. intussusception
3. meckel's diverticulum
4. ovarian pathology
5. constipation
408
management for mesenteric adenitis
SUPPORTIVE - self-limiting disease
hydration, analgesia e.g. paracetamol, NSAIDs
409
what is laryngomalacia? when should it be suspected?
- common, benign congenital abnormality of the larynx
- suspect in otherwise healthy infants (~4 weeks) with stridor/noisy breathing
410
what is a patent ductus arteriosus?
persistent opening between the aorta and pulmonary artery after birth
411
features of patent ductus arteriosus
1. continuous 'machinery' murmur
2. heaving apex beat
3.wide pulse pressure
4.left subclavicular thrill
412
surgical procedure for intestinal malrotation with vulvulus
Ladd's procedure
413
discharge medication following an acute asthma attack (2)
1. salbutamol inhaler
2. 3-5 days prednisolone PO
414
initial management of persistent ductus arteriosus
indomethacin (promotes duct closure)
415
venous hum murmur
a benign murmur in children - continuous blowing noise heard beneath the clavicles
416
what investigation can be done prenatally to diagnose foetal CHD?
foetal echocardiography
