paeds4 Flashcards
(41 cards)
What is kernicterus? BIND?
A rare complication of unconjugated hyperbilirubinaemia.
unconj bili crosses the BBB
can initially cause BIND (billirubin induced neurological dysfunction -starts with changes in tone/suck –> seizures, coma.
Causes long term neurological sequelae of elevated billirubin are given the term kernicterus and include: choreo-athetoid cerebral palsy and hearing loss
Is visual assessment of neonatal jaundice reliable?
Nope
When does physiological jaundice usually present?
Day 2 - 5
Why do infants develop neonatal jaundice?
Increased levels of conjugated or unconjugated billirubin.
CONJUGATED is always pathological - eg biliary atresia (Stools are white).
UNCONJUGATED - unconjugated rises with increased RBC destruction.
Normal infants have higher haematocrit 0.6 and short RBC life span so will naturally have higher levels.
An enzyme in the enterohepatic circulation called B glucoronidase also increases unconj haem.
Conditions like polycythaemia, G6PD def, hered sphero - all increase RBC breakdown
Extravasation of blood - eg cephalohaematoma also increases breakdown
Breast feeding - poor latch - underfeeding measn understooling and less excretion of billirubin
Breast milk also contains B glucoronidase which increases unconj billirubin through enterohepatic circ.
Baby presents with jaundice -
History questions?
Feature
Further information
Age of onset
<24 hours is pathological
>2 weeks is prolonged
Antenatal course
Maternal blood group and antibodies
Maternal infectious serology
Birth
Birth trauma
Instrumental delivery
Feeding
Breast vs formula feeds
Poor weight gain
Output (urine/stools)
Hydration status
Dark urine and pale stools (biliary obstruction)
Family history
ABO/Rhesus
Spherocytosis/ G6PD deficiency
Prolonged jaundice
Thyroid dysfunction
Neonatal jaundice -
What examination findings?
General tone
Neurological exam (Looking for poor suck/hypotonia - BIND billirubin induced neurological dysfunction)
Hydration status: capillary refill time, heart rate, mucous membranes
Vital signs - fever - Looking for sepsis
Plethora - Looking for polycythaemia
Bruising/ cephalohaematoma - Extravasation of blood - increased damage
Hepatosplenomegaly
Pattern and degree of jaundice
Key clinical features (red flags) in neonatal jaundice?
RED FLAGS -
Jaundice within the first 24 hours
Unwell/febrile child
Dark urine and pale stools (biliary obstruction)
Significant weight loss >10% within the first week of life
Cephalohaematoma or significant bruising
Investigations in neonatal jaundice?
Depends on age:
- Within 24 hours - ALL Must have SBR (Conjugated and unconjugated, FBE and Coombs). (main causes here are sepsis and ABO incompatability)
- 24 hrs to 2weeks (usually no ix necessary unless red flags). Causes here - sepsis, ABOI, Insufficient feeding, physiologic, breastmilk
- After 2 weeks
SBR (Conjugated/unconjugated)
FBE, film and reticulocytes
TFTs
Group and DAT
LFT if conj bili is over 10%
Treatment of neonatal jaundice
Depends on cause and on severity of hyperbilirubinaemia
PHOTOTHERAPY - greater than 285micromol/L
TRANSFUSION - greater than 360micromol/L
Specific treatments for different causes of neonatal jaundice
Sepsis
Immediate treatment as per SEPSIS – assessment and management with IV antibiotics
Haemolysis
Discuss with local paediatric services
Dehydration/ feeding concerns
Hydration, feeding plan and support
Consider maternal and child health nurse & lactation consultant involvement
Physiological jaundice
Exaggerated physiological response
Should resolve by 2–3 weeks
Breast Milk Jaundice
Diagnosis of exclusion
Do NOT stop breastfeeding
May last up to 6 weeks, no further bilirubin levels necessary, unless jaundice is deemed to be worsening
Hypothyroidism
Discuss with local paediatric services
Extra-hepatic obstruction
Uncommon but early diagnosis improves outcome
May present with dark urine, pale stools & conjugated hyperbilirubinaemia
NOT excluded by negative abdominal US
If suspected discuss with tertiary paediatric services within 24 hours
What’s the pathophysiology of hirschsprungs disease?
Congenital Absence of myenteric plexus (Auerbachs) in DISTAL colon
10% full colon involved (Total colonic aganglionosis)
70% - only Recto-sigmoid
(myenteric plexus - responsible for bowel motility/peristalsis)
ABSENCE OF PARASYMPATHETIC GANGLION CELLS in distal colon
Presentation of Hirschsprungs disease in the neonate? Whats a complication they can get in the first few weeks?
Can get
FAILURE to pass meconium (constip) in first 24 hours of life
Later - Constipation (not passing stools)
With PR exam - a gush of fluid passes
IN the first few weeks - HIrschprungs associated Enterocolitis (due to clostridium difficile)
- fever, abdo distension, diarrhoea, PR bleed
- life threatening
- can lead to Toxic megacolon
Clinical features of HIrschprungs disease in later life?
- SWELLING/Distension in area around belly
- Constipation
3. Failure to thrive
- Flatulence
- VOmiting - green/brownish
- Diarrhoea
- Severe fatiguie
Genetics and associations with hirschsprungs?
FAMILY HISTORY??
- Downs syndrome
- neurofibromatosis
MEN TYPE 2
Investigations in Hirschsprungs? Definitive management?
Abdominal Xray - obstruction? enterocolitis?(needs IVABs)
RECTAL BIOPSY - diagnosis
Bowel histology - absence of ganglionic cells
Definitive managment - surgical removal of aganglionic section of bowel - can have ongoing bowel dysfunction +/- incontinence
Clinical features of intussusception?
- Severe, colicky abdominal pain in a child between 6 months to two years (several times an hour - Drawing up legs)
- Pallor and lethargy
- VOMITING (prominent) but billious vomiting is late sign
- Blood or mucous in stools
- Red currant jelly stool is a late sign
- Diarrhoea is common
- Often preceded by Viral URTI
- Mother doesnt want to leave mothers arms between episodes
- Right upper quadrant sausage shaped mass
- Distended abdo (late)
- Hypovolaemic shock (late)
- Acute intestinal obstruction can occur
Pathophys and demographic affected by INTUSSUSCEPTION?
Typically infants between 6months and 2 years of age and is more common in boys.
ASSOCIATIONS - HSP, CF, intestinal polyps, Meckels, Recurrent viral infections
Xray findings suggestive of Intussusception
Can also get signs of obstruction
U/S findings suggestive of intussusception
Investigations in intussusception?
Abdominal XR (to exclude bowel obstruction) - specific findings - absent liver edge, target sign (round opacity in RUQ), Crescent sign - in LUQ).
Abdo u/s - not needed if high clinical suspciion
- donut sign
- useful if suspicion but no mass or signs on plain xray
AIR ENEMA - both diagnostic and therapeutic - investigation of choice if high suspicion (Eg bowel obstruction and palpable mass)
Treatment of intusussecption
AIR Enema
small risk of bowel perforation and bacteraemia
WIll be admitted afterwards for observation
(Can also use contrast, or water enemas)
Surg reduction if enemas dont work
If gangrenous bowel due to ischaemia or if perforation of bowel occurs - then needs surg resection
What are the complications of intussusception?
- Obstruction
- Gangrenous bowel (Due to ischaemia)
- Perforation of bowel
- Death
