Paediatrics: Neonatal and Newborn Flashcards
What is jaundice?
A state of hyper-bilirubinaemia > 80 umol/L caused by pre-hepatic, hepatic or post-hepatic factors
When is neonatal jaundice normal and when is it worrying?
Jaundice presents > 24hrs post birth = physiological
Jaundice presents < 24hrs post birth = pathological (often haemolytic)
What is classed as prolonged jaundice and who is this most common in?
What are the other causes?
- Prolonged jaundice is > 14 d post birth or >21 d if premature
- Typically caused by breast feeding
Other causes:
- Hypothyroidism
- Biliary atresia (conjugated)
- Congenital infection (CMV, rubella)
- Galactosaemia
- UTI
What is the normal physiological mechanism of jaundice
RBC in neonates have much shorter life span than adults (70 vs 120 d) - RBC broken down to release Hb
Typically presents at 2 days and lasts 2 weeks
Explain how breast feeding causes jaundice
Breast milk contains enzymes that inhibit conjugation enzymes in the liver –> unconjugated jaundice
Peaks at 3-4 d resolves in 3 wks - 3 months
It is a primary cause of prolonged jaundice (i.e. jaundice lasting > 14 days or 21 days in prematurity)
What are the likely causes of jaundice < 24hrs birth, explain? (4)
(explain the overall process and the specific conditions that cause this)
Pathological - typically due to haemolytic process (pre-hepatic)
Mothers IgG Ab crosses placenta and reacts with foetal Ags on RBCs –> haemolysis –> rapidly increased levels of unconjugated bilirubin
- Rh haemolytic disease - mother is Rh-ve, baby is Rh+ve –> haemolysis
- ABO incompat - mother is O type, child is not –> mothers anti-A/B haemolysin IgG cross placenta and haemolyse RBC in infant
- G6PD deficiency - intermittent acute haemolytic episodes (X-linked recessive, heterozygous males, homozygous females)
- Spherocytosis - spherical shaped RBCs, haemolysis triggered in spleen, this is worsened following viral infection (autosomal dominant)
What is the possible danger of unconjugated bilirubin to the neonate?
Unconjugated bilirubin is not water soluble and cannot be wee’d out, therefore builds up causing yellowing of skin –> can cross BBB –> Kernicteris
What are the investigative findings of a haemolytic cause of jaundice?
Heinz bodies
+ve Coombs test (DAT)
What is the presentation of biliary atresia?
jaundice, pale chalky stools, yellow urine, hepato-splenomegaly
- conjugated bilirubin due to absence of intra/extrahepatic bile ducts
What is the main cause of neonatal hepatitis and what is the presentation?
a1-antitrypsin deficiency - hepatitis and prolonged neonatal jaundice, bleed (due to vitamin k def), portal HTN
What are the general symptoms of jaundice?
Yellow skin starts at head (eyes) –> trunk –> limbs
Sleepy, fatigue, poor feeding, vomiting
What are the key investigations for jaundice?
- Split bilirubin - measures conjugated _ unconjugated (>80 umol/l; >150 umol/l requires treatment)
- Coombs test
- Direct (detects Abs on RBCs) - +ve = haemolytic process
- Indirect (detects free Abs in serum) - done during pre-natal - Blanching test for skin
What is the primary treatment options for jaundice?
List any side effects and monitoring required that you can think of.
What is the treatment of biliary atresia?
What is the treatment of RhHD?
- Phototherapy (450nm blue light converts unconjugated bilirubin to conjugated to wee out)
- AE: fluctuation in temp, diarrhoea, dry skin, rash, eye damage, low mother bonding, poor appetite
- Monitor SBR every 4-6hrs when initiated; 6-10hrs when stabilised or falling; reduce intensity or stop when 50 below treatment line, if bilirubin rises after halted treatment most likely haemolytic process - Blood transfusion - blood removed from baby via central line, donor blood injected - must be twice childs blood volume (2x80ml/kg)
- Surgery if biliary atresia - Kasai procedure
- IV immunoglobulins 500mg/kg over 4hrs if haemolytic disease
What is the worrying complication of jaundice caused by haemolytic disease, breast feeding etc?
Uncojugated bilirubin is insoluble –> cannot be excreted via urine –> cross BBB and stains basal ganglia and brain stem –> kerniecterus (encephalitis)
Early - Jaundice, lethargy, poor feeding
Late - ^muscle tone, opisthotonos, seizures, coma
What is birth asphyxia?
What are the causes?
Lack of oxygen supply to baby in utero –> Oxygen deprived baby attempts to breathe –> primary apnoea (HR+BP maintained) –> gasping for breaths –> secondary apnoea (HR+BP fall) –> terminal
Causes respiratory depression (hypoxic, hypercapnia, resp acidosis) and CV depression (low CO + perfusion + met acidosis )
- Transiet (common) - transient blockage of oxygen supply due to uterine contractions
- Continuous (rare + dangerous) - complete cord prolapse (occludes blood supply), abruption (reduced gas exchange)
What are the symptoms of birth asphyxia and how can it be screened for?
Mild - hyperventilation, very sensitive to touch, starring eyes
Moderate - abnormal tone/posture, does not feed ± seizures
Severe - fluctuation between hypo/hypertonia, prolonged seizures, unresponsive to pain
Screen using APGAR score
7-10 is good
< 7 is worrying and needs medical intervention
What is the management of birth asphyxia?
Resuscitation - ABCDE
- Dry baby and maintain temperature
- Assess HR, RR, tone (APGAR)
- If gasping or not breathing - 5 ventilation breaths
- Reassess for chest movements
- If HR not improving or < 60bpm - compressions (3:1)
- Airway positioning and lung expansion (+ve pressure ventilation mask)
- Intubate
- if HR still not improving, consider DOPE
- Fluids to correct hypo - saline 0.9% with dextrose
- +ve inotrope or chrono trope agent
What are the complications of birth asphyxia?
Hypoxic ischaemic encephalopathy
Athetoid cerebral palsy
What is the clinical presentation of RhHD?
- Antenatal:
- Foetalis hydrops - severe oedema in 2 or more compartments (e.g. ascites, pericardial effusion, pleural effusion) - typically oedematous stiff legs
- Foetal anaemia - Post-natal:
- Foetalis hydrops
- Blueberry muffin rash -
- Jaundice (unconjugated) –> kernicterus
- Hepatosplenomegaly
What is the comments cause of AKI in children?
HUS
What are the key investigations for mothers ante-natally to determine risk of RhHD?
- INDCT - at booking visit (8wks) and 28 weeks
- +ve test indicates requirement for serial Ab titres - Serial Ab titres
- <4umol = unlikely
- 10-100umol = 10% risk
- >100umol = 70% risk of RhHD - Maternal blood group test
- +ve Rh-ve
- Raised anti-Rh titres
What can be given prophylactically for these mothers?
Anti-D IgG Ab to all mothers who are Rh-ve
What tests are done on the foetal blood postpartum?
- Hb + Serum bilirubin (monitor every 4 hrs, to determine rate of SBR increase + Hb decrease)
- Glucose
- DCT = +ve
- Blood group = Rh+ve
What is the management of RhHD baby?
- Monitor Hb + SBR
- Phototherapy
- Haemodialysis + IV IG if SBR > 100umol
- folic acid for 6 months
- Monitor Hb for 2 weeks
What is the typical and atypical causes of HUS?
typical (epidemic) - bacterial infection with E.Coli 0157 or Shigella (D+HUS)
atypical (sporadic) - usually familial but can be due to pregnancy, COCP, malignancy
What is HUS?
Clinical triad of:
- Microangiopathic haemolytic anaemia
- Thrombocytopoenia
- AKI
What are the key features of typical HUS?
- Diarrhoea becomes bloody at 1-3 days (prodrome)
- Abdominal pain, fever, bloating
- Gut: Rectal prolapse, blood diarrhoea, haemorrhage colitis, bowel wall necrosis
- Pancreas: Jaundice, DM type 1, neuro irritability, encephalopathy
What are the key investigative findings for HUS?
- DCT = -ve
- Film - fragmented RBC, reticulocytosis, haemolytic anaemia, thrombocytopenia
- FBC - ^WCC, low platelets (Early sign) deranged U+Es
- Stool sample - detect E.coli 0157
What is involved in a prolonged jaundice screen and when do you conduct one?
SBR (split bilirubin - total and conjugated) Urine MSC DCT FBC, Blood film LFTs, TFTs, U+Es
What are the symptoms of kernicteris?
Actue = poor feeding, lethargy Severe = irritability, opisthotonos, hypertonia, seizures, coma
What is the major complication of kernicterus? (rare)
Choreoathetoid cerebral palsy
What is the treatment for HUS?
- Supportive - Fluid to rehydrate
- Eculizumab
Abx are CI
What is the definition of prematurity
Any birth < 37 weeks
Typically between 24-37 weeks
At what age of gestation are complications exceedingly higher
< 32 weeks
What is the prophylactic management of a baby who is suspected to be premature?
- Tocolysis and Corticosteroids
- corticosteroids promote pulmonary maturity
- takes 24hrs to work
- birth must be delayed using tocolytics (TAN)
- Nifidepine (CCB), Atosiban (oxytocin-r antagonist), Terbutaline
- Alternatively use salbutamol, NSAIDs, ritodrine
What is the (a) preferred and (b) common delivery method for premature babies?
Vaginal is preferred - lower risk of RDS and apnoea of prematurity
CS is common - as majority of premature babies are breech birth
What is the post-natal process of a premature baby?
- Delay cord clamping by 1 minute
- Place baby in plastic back under humidified ventilated chamber
- PEEP
- PIP
- CPAP
- Surfactant if req (requires intubation, oxygen, ventilation) - Fluid resuscitation and NGT with parenteral feeds
- Prophylactic Abx - gentamicin and benzylpenicillin
- Measure weight and temperature
- Monitor oxygen sats
- Monitor glucose
- Start specific treatment and transfer to neonatal ward
What is the presentation of a patient with RDS?
- Dyspnoea
- Tachypnoea
- Signs of respiratory distress: indrawn chest, SC/IC recession, tracheal tug, nasal flaring, increased WoB, accessory muscles
- Cyanosis
What is the treatment for RDS?
- ABCDE
- Surfactant administration -
- intubate, oxygenate and ventilate any patient
- administer surfactant bolus down ETT
- give 2nd dose if high O2 requirement
- consider for all patient < 27 weeks with oxygen requirement > 30-40% - Prophylactic abx - gentamicin and benzylpenicillin
- IV fluids, NGT, parenteral feeds
What is apnoea of prematurity?
transient episodes of apnoea in premature babies often < 34 weeks
What is the treatment options for apnoea of prematurity?
Should resolve by 34 weeks
- Tactile stimulation
- Blood transfusion
- Gastric feeds
- Respiratory ventilatory support
What is SIDS/SUDI?
Sudden infantile death syndrome - nay death of infant < 12 months, where the cause is not immediately obvious
Aka SUDI (sudden unexpected death in an infant)
Explain the aftermath process of SIDS/SUDI?
Parents
- Detailed history
- Referral to coroner - inform parents
- Warn of home visit by police
Child
- After resuscitation failure
- remove ETT, IO needle;
- retain vascular access, clothing, bedding, nappies for police - Samples
- Blood - cultures, toxicology, metabolic and coagulation screen
- Lumbar function
- Nasopharyngeal swab - virology and toxicology
- Urine - biochemistry and freeze immediately - Inform
- SUDI team
- Community midwife/health visitor
- Family GP
- Neonatologist (if neonate) - Home visit by paediatrician and police within 24 hrs
What is chronic lung disease in the newborn defined as?
What is the treatment?
Who is it common in?
Any baby with O2 requirements beyond 28 days
Tx:
- Wean O2 down slowly
- Steroids - to promote pulmonary maturity
Prematurity
Long term ventilator use
What are the symptoms of necrotising enterocolitis?
Abdominal distension/bloating
Poor feeding
Blood diarrhoea
Bilious vomiting
What are the symptoms of intraventicular haemorrhage in the newborn?
Apnoea Visual disturbances Seizures and lethargy Abnormal eye movements Decreased muscle tone Absent MORO reflex
What are the complications of prematurity? (7)
Respiratory distress syndrome (RDS) Chronic lung disease (CLD) Apnoea of prematurity Necrotising enterocolitis Intraventricular haemorrhage Retinopathy of prematurity SIDS/SUDI
What are the complications of prematurity? (11)
Hypothermia Feeding problems Jaundice Respiratory distress syndrome (RDS) Chronic lung disease (CLD) Apnoea of prematurity Necrotising enterocolitis Intraventricular haemorrhage Retinopathy of prematurity Hearing problems SIDS/SUDI
What are the similarities and differences between (a) talipes equinovarus and (b) calcaneovalgus?
Both are positional club foot and have association with hip dysplasia
(a) Talipes equinovarus
usually B/L
whole foot is inverted and supinated
forefoot is adducted
heel is internally rotated and plantar flexed
calf of affected leg appears smaller and thinner
a/w Edward syndrome (tris.18), oligohydramnios, CP, spina bifida
- treated with plaster and bracing
(b) talipes calcaneovalgus
usually U/L
foot is everted and dorsiflexed
- treated with passive exercises and manipulation
A newborn baby presents with a swelling over his parietal bone. He does not seem to be in any distress and all other newborn checks are normal except for some slight jaundice. All investigative texts including DCT is -ve. What is the swelling likely to be?
Cephalohaematoma
Bleeding within the periosteum causing a swelling over the parietal bone confined to the sutures
Symptoms are usually of blood loss
- anaemia
- jaundice (due to blood broken down to produce bilirubin)
A newborn baby presents with a swelling over his parietal bone. He does not seem to be in any distress and all other newborn checks are normal except for some slight jaundice. All investigative texts including DCT is -ve. What is the swelling likely to be?
Cephalohaematoma
Bleeding within the periosteum causing a swelling over the parietal bone confined to the sutures. The centre of the haematoma is soft.
Symptoms are usually of blood loss
- anaemia
- jaundice (due to blood broken down to produce bilirubin)
Must exclude subgaleal haematoma (between periosteum and scalp)
What is a possible explanation for absent moro reflex?
intraventicular haemorrhage
What investigation would you use to diagnose suspected necrotising enterocolitis?
What would be shown?
How would you treat this?
Abdominal X-Ray - pneumatosis intestinal (gas within the wall of the bowel)
Stop feeds, NGT, TPN, fluids, Abx
Surgery - but be aware of short gut syndrome
What might a port wine stain indicate?
Surge-Weber syndrome - seizures, developmental delay and glaucoma
Port wine stain - is a neurocutaneous facial haemangioma than often follow distribution of the trigeminal nerve
Tx - A/E drug, Lazer treatment for stain
What is the possible treatment for strawberry naevi?
Grows between 12-18 months
Usually self-resolves after
Can use BB to shrink
