Paediatrics Flashcards
Mechanism of bilirubin production
Heme in Hb - biliverdin - bilirubin (and albumin) - liver - conjugated with glucuronide then excreted into SI, taken up into blood - urine - urobilirubinogen or metabolised by bacteria - stercobilin
Bilirubin effect on brain
Kernicterus - acute bilirubin encephalopathy, crosses bbb and stains basal ganglia causing permanent damage
Features of kernicterus
Lethargy, poor feeding, irritable, shrill cry
Hypertonic - opisthotonos
Seizures, coma
When does bilirubin start having an effect
When unconjugated bilirubin level > albumin binding capacity
Only when young as bbb matures quickly so higher levels can be tolerated better
Complications of kernicterus
Choreoathetoid cerebral palsy
Learning difficulties
Sensorineural deafness
Bilirubin level which is jaundice
> 80umol/l
3 categories of jaundice causes and examples of each
Increased haemolysis - rhesus incompatibility, ABO incompatibility, G6PD, spherocytosis, sepsis
Decreased secretion - liver dysfunction/post hepatic
Dehydration
What is ABO incompatability caused by and features
some group O women have IgG anti-A-haemolysin which crosses placenta
Hb normal or slightly reduced, no splenomegaly
G6PD - who and what to do
Avoid some drugs
Mediterranean, East, Africa
Causes of sepsis in neonatal jaundice
GBS - ascending infection, PROM, prematurity
TORCH screen
Features of liver dysfunction/post hepatic neonatal jaundice
Chalky pale stools
Conjugated bilirubin collects in liver and damages
Check for dehydration in neonatal jaundice?
7-8 nappies a day
Mucous membranes
Weight loss
Assessing neonatal jaundice
Preterm Septic/unwell Family history Maternal blood group Maternal infection Extent - how much of body, rate of rise (>10mmol/h = concerning), transcutaneous screening tool on sternum, sx (sleepy, poor feeding - phototherapy if sx)
Inv neonatal jaundice
Transcutaneous bilirubin
FBC and blood film for reticulocytes and fragmented cells
Blood group and DCT
Bilirubin - conjugated accumulates in liver and damages
Sepsis inv
Causes of baby jaundice >1d
Physiological
Infection
Haemolysis
Crigler-Najjar - decreased conjugation of bilirubin and glucuronic acid in liver
Why physiological jaundice - 5
Immature enterohepatic circulation - reduced conjugations
High Hb conc as physiolgical Hb release
RBC life span shorter than for adults (70d vs 120)
Reduced gut flora - reduced bile pigment elimination
Breastfed - dehydration - reduced elimination
5 causes of prolonged jaundice
Breast milk jaundice - unconjugated, fades by 4-5w Hypothyroid - unconjugated Infection - unconjugated Metabolic liver disease - conjugated Biliary atresia - conjugated
Features of hypothyroid in baby
Reversible neuro problems/developmental delay Dry skin, coarse facies Prolonged jaundice Constipation On Guthrie test
When is bilirubin conjugated in prolonged jaundice
Metabolic liver disease
Biliary atresia
What is biliary atresia
Angiopathy causes absence of hepatic bile ducts
Can cause toxic damage to liver cells and liver failure requiring transplant
Hepatosplenomegaly, dark urine, pale stools
Inv prolonged jaundice
Split bilirubin - conjugated vs unconjugated
Unconjugated high = breast fed or physiological
Conj shouldn’t be >10-20% total
Radionucleotide scan to see liver drainage
Manage biliary atresia causing prolonged jaundice
Kasai procedure creates bile duct from SI,, mostly successful if <60 days old
Manage prolonged jaundice
Phototherapy - blue light converts bilirubin to soluble products
Exchange transfusion if not improving with phototherapy or increasing rapidly. Warm blood, umbilical artery and vein
ADR of photherpay for jaundice - 4
Eye damage
Dehydration
diarrhoea
Difficult to control tempmerature
Sepsis screen in neonates
Urine
Blood cultures
LP
+- cxr
RF for neonatal sepsis - 5
PROM (>18h) Maternal inf Preterm labour Foetal distress Central line/catheter
Where does sepsis come from in neonate - 4
Environment
Placenta
Ascent from vagina
During birth
Early onset neonatal sepsis timing and bacteria
<48h of birth
E. coli and listeria
Late on neonatal sepsis timing and bacteria
> 48h
Staph a
E. coli
GBS
Presentation of neonatal sepsis
Labile temperature Lethargy Poor feeding Resp distress Collapse DIC
Line infection in neonatal sepsis
Coag neg staph
Manage sepsis in neonates
ABC Ventilation, inotropic, fluids FBC, CRP, glucose Cultures X-ray LP for culture, glucose, protein, WCC, gram stain Stool, throat, urine culture Abx: Early - benzylpenicillin and gentamycin - cefotaxime (meningitis). - amoxicillin (listeria - purulent conjunctivitis, maternal infection ) Late - fluclox and gentamycin, cefotaxime (meningitis)
Sensitising events for rhesus haemolytic disease
Threatened miscarriage
Amniocentesis
Chorionic villus sampling
Birth
Sx of rhesus haemolytic disease
Jaundice, kernicterus Yellow vernix CCF - oedema, ascites Hepatosplenomgaly Progressive anaemia, bleeding Hydrops fetalis - severely affected oedematous foetus with stiff, oedematous lungs
Manage rhesus disease
Inv:
- Check for anti-D antibodies in all rh- mothers at 28 and 34w
- May need foetal blood sampling and intraperitoneal transfusion if large number
- Regular USS and amniocentesis
Deliver baby before severe haemolysis
Trt:
- Exchange transfusion - remove maternal antibodies, if Hb <70
- Phototherapy
- Immunisation with anti-D antibodies at 28w, reduces need for exchange transfusion
- Maternal antibodies will persist for some time and continue to cause some haemolysis
What is respiratory distress syndrome
Deficiency in alveolar surfactant - atelectasis - resp failure - reduced CO, hypotension, acidosis, renal failure
Major factors in resp distress syndrome and why - 2
Premature - alveoli develop at 24w, surfactant at 24-36w with peak at 32w
C section - no stress response to encourage fluid reabsorption
Signs of RDS - 5
Increased work of breathing - nasal flaring, grunting, apnoea/dyspnoea Tachypnoea Chest wall recessions Cyanosis CXR - round glass, not hyperinflated
Differentials of RDS - 7
Transient tachypnoea of newborn - due to fluid, resolves in 24h Meconium aspiration Pneumothorax Diaphragmatic hernia CHD Metabolic acidosis Hypoxic-ischaemic encephalopathy
Treatment of RDS
Prevent with steroids if preterm birth risk
O2
Support resp eg CPAP, ventilation
Surfactant via NGT
Complications of RDS - 4
Chronic lung disease of prematurity
Retinopathy of prematurity
Infection or trauma secondary to incubation
Impaired head growth from steroids
What is chronic lung disease of prematurity and 2 complications
Still requiring o2 at >=36w gestational age or still requiring o2 after 28d
Bronchopulmonary dysplasia - injury to lungs from mechanical ventilation, or delayed maturation - wean slowly +- steroids
CXR - hyperventilated, reticular markings
- Necrotising bronchiolitis with alveolar fibrosis
- Cardiomegaly indicates high pulmonary pressure
- Pertussis and resp infections can cause resp failure - give RSV prophylaxis
Cause of retinopathy of prematurity, what it causes and prevention/trt
O2 and prematurity derange VEGF, causing vascular proliferation at junction of retina.
Causes retinal detachment, fibrosis, blindness, early glaucoma (in teens)
Prevent by running at lower O2, and treat with laser and intravitreal anti-VEGF
RF: premature, slow weight gain, low birth weight, anaemia, twin to twin transfusion (the donor), maternal smoking/drugs,
RF for meconium aspiration - 4
Post term
Chorioamnionitis
Maternal hypertension or pre eclampsia
Smoking or substance abuse
4 broad causes of hypoxic injury and presentation
Hypoxic-ischaemic encephalopathy, need resuscitation at birth
Mostly from problems in labour:
- uterus - excessive contractions
- cord - compression or prolapse
- placenta - poor function, pre eclampsia, abruption
- baby - cardioresp adaptation failure at birth
Effects of HIE - 3
Brain damage
Bradycardia
Liver, kidney, GI damage
Poor prognostic factors in HIE - 3
Slow rate of improvement
Irregular breathing at 30 mins
APGAR low at 20 mins
Manage HIE - 5
Resuscitate, ventilate
Hypothermia jacket (33.5) for 72h as long as:
1) >36w, 2) clinical evidence of deficiency (apgar), 3) cerebral function being monitored. = halves cerebral palsy rates, reduces delayed neuronal death in days after cytotoxic death
Fluid restriction as transient renal impairment
Inotropes and volume support for hypotension
Monitor hypoglycaemia and electrolytes - early feeding, monitor until BM >2.5 3 times
Mortality of prematurity
40% die on labour ward if <24w
95% disability if 23w
Causes of prematurity
Unknown cause - 40%
Smoking
Poverty
Malnutrition
Manage prematurity
Capable centre
O2 and ventilation - nasal, CPAP, mechanical
Temperature - plastic bag, incubator
Cannula as fluids lost through skin
NGT for small feeds - low birth weight formula if <2kg
- encourage day 1 expression from mum
- aim to remove by 36w
- nutritional supplements - vit D and iron, as got from placenta in 3rd trimester, phosphate to prevent osteopoenia
Monitor glucose
Weights of low birth weight, very low and extremely low
Low <2500g
Very low <1500g
Extremely low <1000g
Normal head and lengths at term
33cm head circ
50cm length
What is small for gestational age, 4 acute complications and long term risks
<10th percentile birth weight for gestational age
Risk: hypoglycaemia, hypothermia, foetal death, congenital infection
Long term - obesity and CHD
Types of iugr
Symmetrical - early pregnancy, head/abdo circs/length proportional
Asymmetrical - late pregnancy, head circ > abdo circ and length
Causes of IUGR - 4
Poverty
Twins
Congenital infection
Placental insufficiency
Complications of prematurity
Immature resp centre - bradycardia, apnoea, desaturate <32w
Hypothermia
Hypoglycaemia (<2.6) - give TPN and monitor BM for 1st 2 days. Feed early - most of growth in 3rd trimester
PDA
Infection - IgG mostly crosses placenta in 1st 6m, but can get GBS or nosocomial - coag neg staph or fungal
NEC
Preterm brain injury
Retinopathy of newborn
Bronchopulmoanry dysplasia
Cerebral palsy
OSteopoenia
What is NEC, sx, inv and treat, and long term outcome
Lack of bowel flora and immunoglobulins = bacterial invasion and ischaemia.
- more likely if not breast fed
- feed intolerance, vomiting, bile, distended abdo, blood in stool - perforate - shock
- can cause sepsis and death
- WCC raised, abn vital signs
Broad spec abx, parenteral nutrition, mech vent and circulatory support
Echo to check if reduced perfusion due to heart defect
Long term - strictures, malabsorption, poor neurodevelomental outcome
PDA? Sx, inv and treatment in newborn
Bradycardia, apnoea, difficult to wean off ventilation, bounding pulse, signs of HF
Investiate with echo
Close with ibuprofen or indomethacin (Pg synthetase inhibitor)
Why prematurity hypothermia and consequences
Large surface area, minimal sc fat, naked, thin skin
Can cause hypoxia, hypoglycaemia and unable to gain weight due to increased energy expenditure
What is preterm brain injury and what does it cause
IVH +- ischaemia of parenchyma
Blood vessels supplying ventricles very thin and cerebral blood flow not yet regulated so changes in systemic circ pressure can cause bleed
RF - RDS, pneumothorax
Causes hemiplegia, hydrocephalus (head circ increasing rapidly and bulging fontanelle)
What is cerebral palsy
Brain injury due to IVH - affects fine motor skills, conc, behaviour, abstract reasoning
Inv of osteopoenia of newborn and prevent
Raised ALP
Phosphate supplements to prevent if <1.5kg
Risk of GBS infection to baby
10% colonisation
20-30% of women are colonised
When to prevent GBS infection and how?
Proven in pregnancy, or high vaginal swab
- give IV penicillin durin labour
Presentation of GBS and manage
First 12h, with pneumonia, PPHN or septic shock
Give resp support and abx
What is in heel prick test and when
At 5d old
- Hypothyroid
- CF
- Sickle cell
- PKU
- MCADD
What to give on NNU discharge
Palivizumab - RSV prophylaxis
What is apnoea and causes
No resp effort for >20s, or less time if cyanosis and bradycardia
- Apnoea of prematurity - most common
- Central - less CNS stimuli to resp muscles - causing no airflow or chest wall movement
- Obstructive - pharyngeal instability/collapse, nasal obstruction. No airflow but have chest wall movement
- Mixed - 50% of term babies
Differentials of apnoea - 2
Normal neonatal periodic breathing - 3+ periods with no resp effort lasting 3+s in 20s periods, but no colour or hr change
Subtle seizures
Specific causes of apnoea
Apnoea of prematurity - from day 2-7 CVS - hypotension, hypertension, anaemia, PDA Resp - pneumonia, obstuction, RDS GI Infection CNS - IVH, seizure, hypoxic injury Metabolic Pain Meds - maternal, opiates, prostin Poorly positioned head and neck
Manage apnoea - 3 monitor, 3 treat
If under 34w, O2 sats monitor until had 1w with no episodes. >34w, only monitor if unstable
Apnoea monitor detects abdo wall movement
Ensure patent airway
Tactile stimulation - rub soles of feet
Positive pressure ventilation, high flow nasal cannula
Caffeine - relaxes SM and stimulates cardiac muscle and CNS
Rf for asthm
Low birth weight Family history Bottle fed Atopy Male Pollution
Features of asthma history
Wheeze more than once Night sx Sx between excerbations Triggers Atopy
Manage asthma
Salbutamol spacer 3x a week = add inhaled ICS Review diagnosis and check technique then - LABA and low dose ICS - increase ICS/LABA Montelukast (LTRA), theophylline Increase ICS again Specialist PO prednisolone
All - individual asthma plan and inhaler leaflet
Emergency asthma trt
O2 Salbutamol 5mg (2.5 if <5) nebs with 0.25mg ipratropium bromide 100mg hydrocortisone IV IV Mg Aminophylline Continuous nebs ITU
Asthma red flags
Tiring Hr >200 - senior review Hr dropping - crash call Absent breath sounds Neuro deterioration
When to discharge with asthma
Off o2 and inhalers >4h intervals
Instructions for inhaler <5yo
Spacer and mask
Aerosol so shake for 15s to mix with propellant
Tilt to open mask for babies
1 spray metered dose
Wait 10s or 10 breaths then repeat
Wash with warm soapy water and leave to dry and change every 6m
Inhaler for >5yo - 3 types
Turboinhaler: powder - suck
Easi-breathe: breath activated, doesn’t need coordination, can take top off and use as MDI in exacerbation
>10oy accuhaler metered dose inhaler
What is bronchiolitis
commonest lung inf in infants, exp prem and lung diseae
70% RSV
Bronchioles become infected and inflammed with increased mucus secretion so airways blocked
Presentation bronchiolitis
Mostly <6mo
Low grade fever and coryza then dry cough, fever, wheeze (high pitched, exp>insp), recession, increased rr
Can cause resp distress, low sats, nasal flaring
End insp crackles
Hyperinflation on cxr
Inv bronchiolitis
PCR nasopharyngeal secretions - aspirated
CXR to rule out pneumothorax
Bloods
ABG
Treat bronchioliiss
O2 if sats <92%
IV fluids or NGT
May need CPAP
Steroids and neb adrenaline but not routine
Isolate according to virus
No response to salbutamol as not bronchospasm
Palivizumab RSV monoclonal antibody in SCID, CHD, NNU as prophylaxis
What is CF
Aut recessive
Defective gene causing defective protein - CF transmembrane conductance regulator - abnormal Cl transport - sticky mucus builds up outside cell
Where does CF affect
Lungs- - mucus build up and impaired ciliary function
Pancreas - thick secretions block ducts - pancreatic enzyme def and malabsorption
Intestines - Thick meconium - meconium ileus
Sweat glands - salty
CF presentations - 4
Meconium illeus - bilious vomiting, abdo distension, delay in passing meconium
Recurrent chest inf
Intestinal malabsorption - pancreatic enzyme deficiency
Newborn screening
CF diagnosis
1 of: newborning screening OR sibling with CF
AND
1 of: increased sweat chloride conc OR 2 identified CF mutations OR abn nasal epithelial ion transport (nasal potential difference)
Manage CF
Monitor FEV1 for disease progression
Daily physio x2 and exercise
Neb DNAase or hypertonic saline to decrease viscosity of secretions for clearance
Neb pseudomonal antibodies if colonised
Prophylactic abx and rescue abx (PO then IV), central venous catheter if recurrent
Pancreatic enzymes - creon
Double lung transplant - last resort
High calorie diet (150%) - may need gastrotomy to achieve this
Lifestyle advice for CF
Avoid others with CF and infected people
Avoid stables or compost - aspergillus fumigated
Avoid jacuzzis - pseudomonas
NaCl tablets in hot weather/exercise
Annual flu vaccine
Clean and dry nebulisers thoroughly
Complications of CF - 5
Distal intestinal obstruction syndrome Recurrent resp infections, bronchiectasis, chronic lung disease Low body weight Infertility CF related diabetes
Manage low body weight in CF
Pancreatic enzyme replacement, high calorie, supplements, NGT/PEG
Diffference betweeen constipation and DIOS
DIOS - terminal ileum and prox colon obstuction instead of whole bowel - see faecal loading on axr, palpable mass in RIF = dehydrated thick faeces
Cause of DIOS in CF and treatment
Lack of pancreatic enzymes or non compliance
Hot weather/salt deficiency
Cleared with oral gastrograffin
Screening for CF
Guthrie - raised immunoreactive trypsinogen, then screened for common CF mutations. 2 = sweat test
= pilocarpine electrical stimulation and sweat collection
Viral wheeze timing
<2yo. Not by 5yo as immune to most viruses
Peaks at 5d then gets better by 7-10d, cough can last 4w
RF and features of viral wheeze
Smoking in pregnancy
Bronchiolitis as baby - makes airways hyperreaactive
Associated with RTi
Difference of bronchiolitis and wheeze
Fine insp crackles in bronchiolitis
Manage viral wheeze
Salbutamol +- steroids trial for 8w
Severe = neb salbutamol +- steroids and monitor
CXR to check for other cause if not improving, eg pleural effusion (USS)
Supportive - fluids, no resp distress, NGT to avoid exhaustion
Most common causes of pneumonia in children
Virus most common
Newborn - group b strep, gram neg enterococcus
Infants - RSV, H infl, strep pn, pertussis, staph a, chlamydia trachomatis
>5yo - strep pn, chlamydia trachomatis, mycoplasma pneumoniae
All ages - mycobacterium TB
Signs of pneumonia - 5
poor feeding malaise Resp distress Temp high (>39) End insp coarse crackles
Inv pneumonia kids
CXR
Sputum/blood culture
Treat pneumonia in kids
Mostly viral if <2yo - no abx
Abx - amoxicillin
Culture TB
Slow growing mycobacterium
Wait 6 weeks
Positivity low, especially in children
TB in body
Primary complex - heals or progresses
Disseminated = lung apices, meninges, spine, nodes - active or dominant
Extra-pulm, CNS and miliary more common in kids
Normally primary, not latent reactivation
Resistance in TB
MDR = to R and I XDR = to aminoglycosides and fluoroquinolones
Signs of TB - 5
Malaise Anorexia Failure to thrive Cough Low grade fever
Screen for TB
Screen for latent - Mantoux test - inject antigens intradermally and look for induration >5mm in 48-72h = type 4 sensitivity reaction
- Can be false pos if recent vaccine or active TB
- Can be false neg if <6mo
Interferon gamma release assays - false negs <5yo
CXR if either positive
Diag TB
Culture and Ziehl-Neelson stain of sputum x3 - aspirate <5yo in morning as no sputum production
CXR
Treat TB and prevention
Rifampicin - 6m
Isoniazid - 6m
Pyrazinamide - 2m
Ethambutol - 2m
CNS - dexamethasone and 12m abx - check LFT, eyes and HIV first
Latent - R and I for 3m as 10% chance of activation
Prevention - BCG 65-70% effective, mostly against more severe miliary and CNS
What is whooping cough and signs
Bordatella Pertussis, infants or >14yo, droplet spread with 10-14d incubation
Week of coryza then spasmodic cough (can last 100), with inspiratory whoop (insp against closed glottis)
Vomiting
Worse at night or after feeds, better when crying
Diagnose whooping cough
PCR nasal swab
Complications of whooping cough
100d cough Pneumonia Severe and fatal if <6mo Petechia on cheeks Conjunctival/retinal bleed, seizures, hypoxic encephalopathy from coughing
Manage whooping cough
Macrolide - erythromycin, within 21d of onset
Notifiable
Admit if <6m and severe spasms, cyanosis attacks
Upper airway obstuction acute managemet
Calm
Don’t examine throat
Organised
Observe for hypoxia or deterioration
Neb adrenaline and alert anaesthetist if severe
Tracheal injubation if resp failure from secretions, obstruction or exhaustion
Features of common cold and trt
Clear or purulent nasal discharge and blockage
Persistent cough for 4w
Viruses - rhinovirus, coronovirus, RSV
Reassure parents, paracetamol, ibuprofen
Most common cause of sore throat
Pharyngitis - inflamed pharynx and soft palate
Local nodes enlarged and tender
Viruses - adenoveritus, enterovirus
Older - group a B-haemolytic strep
What is croup, main danger, cause and age range
Laryngotracheobronchitis
Mucosal inflammation and increased secretions
Most dangerous = oedema in supraglottic area which can cause narrowing of trachea
95% viral - parainfluenza, RSV
6m-6yo in autumn
Signs of croup
Fever and coryza, onset over days, then -
Barking cough
Harsh stridor and hoarseness. If at rest = bad
Sx worse at night
Resp distress
Manage croup
Admit if <12m, upper airway obstuction or unsure about diagnosis
Steroids - oral and neb reduce severity and duration
Neb adrenaline if stridor at rest
O2 if agitated
Differentials for croup and key differences
Bacterial trachietis - staph a, pseudomembranous, high fever, toxic, rapidly progressive obstruction
Acute epiglottitis - over hours, cannot drink, salivating, toxic and very ill, temp 38.5, muffled voice, soft whimpering stridor
What is acute epiglottitis
Life threatening emergency
Intense swelling of epiglottis and surrounding tissues by H influenzae B
Sx of epiglotitis
No cough Very painful throat - can’t swallow or speak Very ill and acute Temp >38.5 Soft inspiratory stridor Drooling Distress
Manage epilottitis
Urgent admission and contact anaesthetist, paediatrician, ENT
ICU
Intubate under GA or tracheostomy
Blood cultures and IV cefuroxime/ceftriaxone
Causes of wet cough
<6yo
Acute - viral, bacteria, pertussis
Subacute - 3-8w, should be resolving
- post-viral, or pneumonia
Chronic >8w
- bronchiectasis: CF, primary ciliary dyskinesia, untreated infection (ISS), persistent collapse
- persistent bacterial bronchitis - ?primary ciliary dyskinesia
- recurrent aspiration
- immune problem - recurrent/unusual infections
What is linked to neonatal rhinitis and bronchitis?
Kartagener syndrome - primary ciliary dyskinesia:
1) sinusitis - neonatal rhinitis, persistent nasal discharge
2) bronchitis
3) dextracardia
Heterogenous genetic make up and manifestation
Assoc with CHD, asplenia, hydrocephalus, renal disease
Causes of pleural effusions - 3
Infectious - parapneumonic (pneumonia, lung abscess, bronchietasis),
- non-TB bacterial pneumonia - staph a, strep pn, h infl (<5yo), group a strep (>5)
- CXR if pneumonia and not improving after 48h of treatment
Malignancy
Congestive heart failure
3 stages of parapneumonic effusion
- Exudative - clear fluid from pneumonia, low WCC
- Fibropurulent - complicated - deposition of fibrin in pleural space causing septations and loculations
- increased WCC
- thickened fluid = pus (empyema) - Organisational - fibroblasts infiltrate and intrapleural membranes are reorganised to become thick and nonelastic
- prevent re-expansion
- impair function
- persistent pleural space with potential for infection
- spontaneous healing or chronic empyema
Investigate pleural effusion
CXR Confirm on chest USS Blood culture including anaerobes Sputum culture FBC - anaemia, WCC, platelets Electrolytes - SIADH? Antistreptolysin O titre Diagnostic tap if suspect malignancy, pleural fluid microbiology Chest CT if plan to operate
Manage pleural effusion
IV abx then PO
Chest drain
Intrapleural fibrinolyic is complicated effusion
Inhaled foreign body - features and inv
Average 3yo
Quick onset, choke, cough
CXR - shadowing, compare insp and exp film to see what’s not deflating (blocked)
Bronchoscopy to identify object
Positive cardiac screening
RR >60 Apnoea >20s at a time or with cyanosis Recession, nasal flaring Cyanosis Visible pulsation, heaves, thrills on precarious Murmur - absent if large defect Absent/weak femoral pulses Pulse ox at 6-12h - inv if difference between limbs >2% or o2<95% (esp in otherwise well child)
RF for cardiac disease - 5
Antenatal scans or genetic abnormalities Family history Teratogens or substances Conditions - epilepsy, diabetes, SLE Viruses in 1st trimester
Circulatory changes at birth
Before birth, LA pressure is low as minimal blood return from lungs, so blood shunts through foramen ovale from RA to LA
At birth, resistance to pulmonary flow decreases so blood goes here from RA, then LA pressure increases, closing FO
Ductus arteriosus closes in first hour so f lif - prevent with prostaglandins (prostin), close with NSAIDs (ibuprofen)
Signs of innocent murmur
[4S] Systolic left Sternal edge aSymptomatic Soft Gone by 24hold, often heard in febrile illness or anaemia due to increased CO
Signs of pathological murmur and inv
Loud, wide area, harsh, other findings
Echo, CXR, ECG
Which heart abnormalities are critical and why
PDA dependent - baby will deteriorate when it closes around 2d old:
- pulmonary atresia
- hypoplastic left heart
- tetralogy of fallot
- transposition of great arteries
- tota anomolous pulmonary venous drainage (into RA)
- tricuspid atresia
- truncus arteriosus
What murmur is diastolic, left sternal border machinery
PDA
Which murmur is ejection systolic, fixed splitting of 2nd heart sound
ASD
Which murmur is pansstolic, lower left sternal border
VSD
Which murmur is ejection systolic, upper left sternal border
Pulmonary stenosis
Which murmur is upper left border, crescendo decrescendo
Coarctation of the aorta
ASD murmur and sx
Ejection systolic upper left sternal edge, fixed splitting of 2nd heart sound
Apical pancystolic murmur from AV regurg if partial AVSD
Often asymptomatic, may have recurrent chest inf/wheeze or arrhythmias in adulthood
VSD murmur
Pansystolic, left lower sternal border
PDA murmur, pulse and complications
Machinery, diastolic, upper left sternal border beneath left clavicle
Increased pulse pressure - bounding
HF and pulmonary htn
>1m after EDD = persistent PDA
Pulmonary stenosis murmur
Ejection systolic
Upper left sternal border
Coarctation of the aorta murmur
Crescendo decrescendo
Upper left sternal border
Aortic or pulmonary stenosis presentation in child
Normally well
2 CHD causes of sick neonate collapsed with shock
Coarctation
Hypoplastic left heart
3 Causes of left to right shunt and presentation
Breathless or asymptomatic
ASD, VSD, PDA
CHD causing breathless but not cyanosis child
Left to right shunt - ASD, PDA, VSD
CHD causing blue child
Right to left shunt
- Tetralogy of fallot, TGA
Mixing: AVSD, complex congenital heart disease
Symptoms of heart faiure in child
Breathless. Increasing on exertion or feeding
Sweating
Poor feeding - interrupted - sweat, become breathless and pale
Vomiting
Recurrent chest infections
Palpitations or chest pain
Signs of HF in child
Tachycardia, tachypnoea Peripheral cyanosis Poor weight gain Murmur or gallop rhythm Hepatosplenomegaly
Causes of heart failure in infant - 4
Obstructed duct dependent circulation
- hypoplastic left heart,
- critical aortic valve stenosis,
- severe coarctation,
- interruption of aortic arch
Causes of heart failure in child - 3
Left to right shunt - ASD, VSD, large PDA
Causes of heart failure in adoelscent - 3
Eisenmenger
Rheumatic fever
Cardiomyopathy - unexplained deaths in family <30yo? Pacemakers <50yo?
Presentation of CHD
Antenatal USS 18-20w then detailed echo Murmur HF Shock Cyanosis
Causes of CHD
Maternal disorder - diabetes, SLE, rubella
Maternal drugs - warfarin, foetal alcohol syndrome
Chromosomal - Down’s, Edwards, Patau’s, Turner’s
Inv PDA
CXR and ECG normal
Echo to visualise duct
Close PDA and why
Decrease risk of bacterial endocarditis and pulmonary vascular disease
With coil or occlusion device around 1y
NSAIDs
2 types of ASD
Secundum - centre of septum, involves foramen ovale
Primum - partial AVSD - between bottom of atrial septum and AV valves, abnormal valves
Inv ASD
CXR - cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings
ECG - secundum = partial RBBB and right axis deviation, from RV enlargement
Echo - anatomy. Main diagnostic tool
Manage ASD
Treat if causing RV diataion
Secundum - cardiac catheterisation and occlusive device
Primum - surgical correction
Treat at 3-5yo to prevent RHF and arrhythmias
VSD - what is small? Sx and inv and trt of small VSD
<3mm = small
Asymptomatic
Loud pansystolic murmur at lower left sternal edge, quiet P2
ECG and cxr normal, echo shoes abnormality
Close spontaneously
Prevent bacterial endocarditis by good dental hygiene
Large VSD sx, inv, treat
Same size or bigger than aortic valve
HF with SOB and failure to thrive, recurrent chest infections. Always cause pulmonary hypertension
Signs - tachycardia, tachypnoea, active recording, hepatomegaly, soft pansystolic murmur, apical middiastoic murmur, loud p2
CXR shows cardiomegaly, pulm vessel enlarment and oedema
ECG shows biventricular hypertrophy by 2mo (tall qrs)
Echo shows defect
Diuretics for HF and captopril (ACEi)
Additional calorie input
Surgery at 3-6m to treat pulmonary htn, HF and prevent long term damage
Features of tetralogy of fallot and sx
VORP:
VSD, Overriding aorta, Rv hypertrophy, subPulmonary stenosis causing RV outflow obstruction
Sx: cyanosis, hypercyanotic spells (TIA, stroke, MI, death) followed by sleep, squat on exercise
Signs - clubbing, loud harsh ejection systolic murmur at left sternal edge from day 1
Diagnosis of ToF
Antenatally
Inv ToF
CXR - small boot shaped heart from RV hypertrophy, increased pulmonary vascular markings
ECG - normal at birth, then RV hypertrophy
Echo - features
Manage ToF
<6mo - medical, tube from subclavian to pulmonary artery or RV outflow balloon dilatation if cyanosis at birth
6m - definitive surgery to close VSD and relieve outflow obstruction
Hypercyanotic spells - treat if >15m with
- sedation, pain relief
- IV propanolol
- fluids, bicarbonate
- artificial ventilation to reduce metaboic o2 demand
Transposition of great arteries abnormalities
ASD, VSD and PDA to allow mixing
Presentation and inv for TGA
Day 2 when PDA closes so reduced mixing - cyanosis, second heart sound loud, no murmur
Inv - cxr = narrow mediastinum and egg shaped heart, increased pulmonary vascular markings. ECG normal. Echo shows vessels
Manage TGA
Maintain mixing with prostin infusion or balloonatrial septoplasty to make FO flap incompetent
Operate in first few days of life
Complication of left to right shunt and treatment
Eisenmenger: sx from shunt decreases as grow, but pulm arteries become thickened and resistance increases. 10-15yo, shunt reverses and become cyanosis
Life expectancy 40-50yo
Treat high pulmonary blood flow, transplant
What is AVSD and common associated condition
Common in Down’s syndrome
Failure of endocarditis cushions - 5 leaflet valve between atria and ventricles
Causes pulmonary hypertension
Presentation of AVSD and treatment
Antenatal USS Cyanosis at birth HF att 2-3w of life No murmur Medical management for HF and surgical repair at 3-6m
What is tricuspid atresia
Absence of tricuspid valve - small and nonfunctioning right heart
Need ASD and VSD to live
Mix blood through ASD = cyanosis
Manage tricuspid atresia
Shunt from pulmonary to subclavian artery, reducing cyanosis
Pulmonary artery banding reduces breathlessness
Fontan proceedure - IVC and SVC go into pulmonary arteries
Presentation of aortic stenosis
Aortic valve leaflets partly fused, restricting exit from LV
May be critical stenosis - duct dependent (HF)
Asymptomatic
Chest pain/reduced exercise tolerance/syncope on exertion if severe
Signs - small volume slow rising pulse, ejection systolic murmur, carotid thrill
Inv aortic stenosis
CXR - post-stenotic dilatatation of ascending aorta, enlarged LV
ECG - LV hypertrophy
Manage aortic stenosis
Balloon valvotomy if sx on exercise or high resting pressure gradient
May require valve replacement eventually
HF - diuretics and digoxin, plus IV furosemide if still sx
Findings of pulmonary stenosis on exam and inv
Asymptomatic
Ejection systolic murmur, ejection click, RV heave if severe
CXR - normal or post-stenosis dilatation of pulmonary artery
ECG - RV hypertrophy
Manage pulmonary stenosis
When pressure gradient very high, with transcatheter balloon dilatation
What is coarctation of the aorta
Arterial duct tissue encircles aorta at point of duct insertion - aorta constricts when duct closes
Features of CoA and associated condition
Present at d2 with collapse when duct closes
VSD usually present
Radiofemoral delay
Assoc with Di George
Manage CoA
Stent or surger by 5yo to avoid pulmonary hypertension
Hypoplastic left heart syndrome?
Underdevelopment of left side of heart:
- LV very small
- mitral valve very small
- aortic valve atresia
- Ascending aorta small and coarctation
Diagnosis of HLHS and presentation
Antenatal USS
Profound acidosis and CVS collapse at birth
Manage hlhs
Neonatal - Norwood procedure
6m or 3y - Fontan procedure
Sx of inf endocarditis and who is at risk
Risk if CHD Fever, anaemia Clubbing, splinter haemorrhages Necrotic skin changes Splenomegaly
Most common cause of inf endocarditis
Strep viridans
Manage inf endo
Penicillin and aminoglycoside IV 6w
Cause of cardiomyopathy
Dilated - inherited` secondary to metabolic disease or viral infection
Features of dilated cardiomyoathy
Large poorly contracting heart
Suspect if HF and dilated heart
Treatment dilated cardiomyopathy
Diuretics, ACEi, carvedilol (b blocker)
Treat myocarditis
Most improve spontaneously
May need a transplant
Causes of pulmonary hypertension - 4 classes
1) Pulmonary artery - pressure high from heart - VSD, AVSD, PDA idiopathic, HIV
2) Pulmonary vein - pressure must be higher to return to heart - left sided heart disease, pulmonary vein stenosis or compression
3) Pulmonary thromboembolism
4) Respiratory - chronic obstructive or premature lung disease, interstitialung disease, upper airway obtruction, sleep apnoea
Manage pulmonary hypertension
Fix shunts if present - at 6 months
Inhaled nitrous oxide
IV magnesium sulphate
Sildenafil - oral phosphodiesterase inhibitor
Bosentan - endothelin receptor antagonist
GMP
Anticoagulation - heparin, aspirin or warfarin
Heart and lung transplant only option if not treated and becomes pulmonary vascular disease
Features of colic
First few weeks of life, resolves by 4mo
Crying, pulling up legs, excessive flats for >3h on 3+ days a week
Assoc with feeding difficulties
Manage colic
Grandparent involvement, reduce stress, reassurance
Movement
Let baby finish first breast first
Severe and persistent could be CMPI or GORD - 2w trial of protein hydrolysed formula and consider GOR treatment trial
Crying - how to help parents - 5
Take concerns seriously Help them recognise hungry/tired Encourage vocal, vestibular or tactile stimulation Baby centred approach Involve health visitor
Causes of excessive crying - 5
Infection - middle ear, URTI, meningitis Unrecognised fracture Oesophagitis Severe nappy rash and constipation Testicular torsion
Constipation description
<3 stool a week
Or excessive hardness with strain/pain
With reluctance to feed
Long standing - overdistended - reduced urge to defecate
Causes of constipation
Function - lack of fibre, poor diet, reduced fluid intake, ignoring urge Hypothyroid Hypocalcaemia Painful fissure Anorectal abnormalities eg atresia Hirschprung’s
Red flag sx for constipation
Failed to pass meconium in first 48h - Hirschprung’s
Failure to thrive - hypothyroid, coeliac
Neuro:
Abnormal lowerlimb neuro/deformity - lumbosacral pathology
Sacral dimple/central pit - spina bifida occulta
Anus:
Abnormal position, patency, appearance - Anatomy
Bruising or multiple fissures - abuse
Examination for constipation
Palpable mass/distinction - constipation, Hirschprung’s
DRE by specialist if suspect pathology
Spine - spina bifida occulta
Manage constipation
Advice - - obey call to stool (toilet retraining) - gastrocolic reflex - dietary fibre and fluid 1st line laxative to evacuate overloaded rectum completely - macrogol softener eg polyethylene glycol and electrolytes (movicol paediatric plan). Escape over2w until resolved impaction 2nd - stimulant senna or sodium picosulphate Follow treatment with maintenance Enema if oral fails
Dehydration 5 risks
Low birthweight and <6mo 6+ diarrhoea stools in 24h 3+ vomits in 24h Unable to tolerate fluids Malnutrition
Why dehydration in kids?
Bigger surface area to weight ratio - higher insensible water losses
Higher basal requirements
Immature renal tubular reabsorption
How much insensible losses and basal fluid requirements
Insensible losses - 300ml/kg2/d or 15-17ml/kg/d
Basal requirements - 100-120ml/kg/d
Dehydration %s
5-10% = clinical dehydration >10% = shock
3 types of dehydration
Isonatraemic - proportional losses
Hyponatraemic - diarrhoea/drinking hypotonic solution = sodium lost more than water - fall in plasma sodium - water shifts from ECF to ICF - increase in brain volume and shock
Hypernatraemic - high insensible losses - fever/hot/dry envt or profuse low-sodium diarrhoea - water loss > sodium loss = hypertonic ECF compared to ICF so water shifts to ECF = reduced signs of dehydration
- water drawn out of brain and cerebral shrinkage = jitttery, increased tone and reflexes, altered consciousness, multiple small cerebral haemorrhages
Investigate dehydration
Stool culture if septic, blood/mucus, immunocompromised
Plasma electrolytes, urea, creatinine, glucose if going to give IV fluids or suspect hypernatraemia (neuro signs)
Bloodculture ifstarting abx
MAnage dehydration
Oral rehydration - fluid deficit replacement (50ml/kg) over 4h as well as maintenance
IV fluids - bolus if shock, and replace deficit over 24h
Fluid replacement over 48h if hyperntaramia to avoid cerebral oedema and seizures and monitor sodium
Abx if sepsis, extraintestinal bacterial spread, salmonella gastritis <6mo, malnourished or immunocompromised, specific bacteria or protazoa eg shigella, cholera, giardiasis, c dif with pseudomembranous colitis
No antidiarrhoeals or anttiemetics as will prolong excretion of bacteria
Fluid requirements
Maintenance: 100ml/kg/24h for first 10kg
50ml/kg/24h for next 10kg
20ml/kg/24h for the rest
Emergency boluses: 20ml/kg/24h (1st 10kg)- 20ml/kg/24h (2nd 10kg) - 10ml/kg/24h (other)
Correct fluid deficit: weight x % deficit x 10
CAuses of failure to thrive and what is it
Head circ preserved relative to weight
Drop 2 centile
1) inadequate intake - environmental, suck/swallow or anorexia from chronic illness
2) excessive loss - diarrhoea, vomitingg
3) malabsorption - coeliac, CF
4) inadequate use eg syndromes
5) excess requirements eg malignancy, CF, chronic infection, resp, renal failures
Initial managemen of failrule to thrive - 3
Increase energy intake
Diet and behavioural modification
Monitoring growth
Contributing factors to failure to thrive
Difficulty at home - neglect, abuse, deprivation
Feeding - unskilled, inadequate breast milk
Physiological - idiosyncratic growth pattern, normal child (look at family, preterm, alert and responsive)
History in failure to thrive
Food diary Process of feeding Diarrhoea, vomiting, cough, lethargy Iugr, premature Normal development Family Psychosocial problems at home
Examination for failure to thrive
Malabsorption - distended abdo, thin buttocks, miserable
Chronic resp disease - chest deformity, clubbing
Signs of Hf or nutritional deficiencies
Inv failure to thrive
Bloods:
FBC, ferritin, UE, glucose, LFT, calcium, TSH, crp
Immunoglobulins
MSU
Specific testS: IGF-2 - eg IBD Coeliac serology Sweat test Stools
Imaging:
CXR
Renal/CNS USS
Skeletal survey - dwarfism, abuse
Manage failure to thrive
GP, health visitor, paediatric dietician, clinical psychologist
Admit if <6m and severe, require active feeding
commonest causesss of gastroentteritis
Rotavirus (most)
Norovirus (most in adults)
Astrovirus, adenovirus
Bacteria causes - less common in developed countries - blood in Stools
- Campylobacter jejuni - severe abdo pain
- shigella and salmonella - blood and pus, pain and tenesmus
- cholera and E. coli - profuse, rapidly dehydrating diarrhoea
Presentation of gastroenteritis
Loose,watery stools Vomiting High pitched cry and inconsolable - hypernatraemia No fever History of contact with DV/abroad Dehydration and shock
Assess dehydration and shock
Weight loss CRT, BP, HR, weak pulse, rr UO Skin turgor, mucus membranes, fontanelle Pale/mottled, sunken eyes - assesses CSF
Mimics of gastroenteritis
systemic - sepsis
Local inf - resp, otitis media, hep a, uti
Surgical - pyloric stenosis, intussusuceptiotn, appendicitis, NEC, Hirschprung’s
Metabolic - dka
Renal - hus
Inv gatroentereitis
Clinical diagnois
Stool culture if septic appearance or travel hx or no improvement in 7d- bacteria, ova, cysts, parasites
Bloods and UEs if requiring fluids
Blood culture if starting abx
Manage gastroenteritis
Monitor weight
continue breastfeeding
ORT - diarolyte fluid challenge (5ml 5mins) then 50ml/kg over 4h
NGT if won’t take ORT
Fluids - dextrose, saline, potassium
Ondansetron to reduce - vomit, need for fluids, need for admission
Monitor UE, creatinine, glucose
Hypernatraemia - half rehydration rate - so rate = (maintenance x2 + rehydration)/48 = hourly
Complications of gastroenteritis
Dehydration
Malnutrition
Temporary sugar intolerance after DV with explosive water acid stools - give temporary lactose free diet
Post-enteritis enteropoathy - resolves spontaneously
Features of GORD
Recurrent regurg/vomiting but still gaining weight as normal and otherwise well
Common in infancy
Due to inappropriate relaxation of lower oesophageal sphincter due to functional immaturity
4 risk factors for GORD
Mainly fluid
Mainly horizontal posture
Short intra-abdo length of oesophagus
Malrotation/previous exomphalos
Differentials for GORD
CMPI
Physiolgical, overfeeding
Inv GORD
Clinical diagnosis, observe feeds
If atypical hx, complications or failure of treatment response:
- 24h oesophageal ph monitoring
- endoscopy with biopsy to identify oesophagitis
