Gastroenterology Flashcards
Guidelines on infant feeding?
- WHO recommend exclusive breastfeeding for 1st 6m
- NICE guidelines state 1st feed ideally within 1h of birth
Advantages of breast feeding for infant? (5)
- Ideal nutrition for 1st 4-6m
- Life-saving in developing countries
- Decrease in GI infection/preterm NEC
- Enhances mother-child relationship
- Decreased risk of IDDM, HTN and obesity in later life
Advantages of breast feeding for mother? (3)
- Promotes close attachment
- Increased time interval b/w children – important at reducing birth rate in developing countries
- Possible decrease in pre-menopausal breast cancer
Potential complications/disadvantages of breast feeding? (8)
- Unknown intake
- Breast milk jaundice
- Infection transmission (CMV, HBV, HIV)
- Drug transmission - inc recreational
- Nutrient inadequacies (if beyond 6m)
- Vit K deficiency
- Less flexible
- Emotional upset
How is colostrum different from normal breast milk? (2)
Higher protein
Higher immunoglobulin
What maternal hormones are important in breast feeding? (2)
Prolactin - increased milk production
- Ant pituitary
Oxytocin - let down reflex
- Post pituitary
Why is unmodified cow’s milk unsuitable for babies?
- Too much protein/electrolytes
- Inadequate iron/vitamins
From what age can pasteurised cow’s milk be given?
1y (formula before this)
- Still many vitamin deficiencies, so must have supplements unless on good mixed solid diet
- Should have full-fat up to 5y
When may specialised infant formula be needed? (5)
- Cow’s milk protein allergy/intolerance
- Lactose intolerance
- CF
- Neonatal cholestatic liver disease
- Following neonatal intestinal resection
Differences b/w cow’s milk formula and specialised formula?
Cow’s milk:
- Protein derived from cow’s milk protein
- CHO = lactose
- Fat mainly long chain triglycerides
Specialised:
- Protein either hydrolyzed cow’s milk protein, a-a’s or from soya
- CHO = glucose polymer
- Fat = combination of medium and long chain triglycerides
How is FTT categorised?
Mild - fall across 2 centile lines
Severe - fall across 3 centile lines
Causes of FTT? (broad categories) (5)
- Inadequate intake
- Inadequate retention - D/V, GORD
- Malabsorption - coeliac, CF, NEC etc
- Failure to utilise nutrients - Down’s, IUGR, metabolic disorders, storage disorders etc
- Increased requirements - thyrotoxicosis, CF, malignancy, HIV, CKD etc
What is the MUST tool?
Malnutrition universal screening tool
What are the 2 types of protein/energy malnutrition?
Marasmus (no oedema)
Kwashiorkor (oedema)
Features of Kwashiorkor?
Generalised oedema
Severe wasting
Plus
- A ‘flaky-paint’ skin rash with hyperkeratosis (thickened skin) and desquamation
- Distended abdo and enlarged liver (fatty infiltration)
- Angular stomatitis
- Hair which is sparse and depigmented
- Diarrhoea, hypothermia, bradycardia and hypotension
- Low plasma albumin, potassium, glucose and magnesium
Recommended intake for different age groups?
0-3m = 18-32 oz (530-950ml) 4-6m = 28-40 oz (830-1,180ml) 7-9m = 24-36 oz (710 – 1,060ml) + 1-2.5 cups solids 10-12m = 18-30 oz (530- 890ml) + 3-4.5 cups solids
Normal frequency of bowels opening in infants?
4/day in first few days
2/day by 1y
By 4y, have pattern of adults (3/d-3/w)
- -> YET highly variable
- Breast fed may not pass stools for days
4 things to consider if constipation in baby?
- Hirschprung’s
- Anorectal malformations
- Hypothyroidism
- Hypercalcaemia
Red flags in constipation? (8)
- Failure to pass stools in 1st 24h
(?Hirschprungs) - FTT (coeliac, hypothyroid)
- Gross abdo distension (Hirschprung/other GI motility disorder)
- Abnormal lower limb pathology/urinary incontinence - ?Lumbosacral
- Sacral dimple - spina biffida etc?
- Abnormal appearance of anus
- Bruising around anus (?abuse)
- Perianal fistulae/fissures/abscesses (Crohns?)
What is encoparesis?
Toilet-trained child soiling clothes
What is soiling due to overflow and aim of management?
- Rectum becomes overdistended with loss of feeling the need to defecate
- → Involuntary soiling may occur as contractions of full rectum inhibit internal sphincter
- Initial aim is to evacuate rectum completely
What is functional encoparesis?
Repeated involuntary faecal soiling not caused by organic defect or illness
How do you differentiate b/w functional encoparesis and soiling due to overflow?
- Check for s+s of constipation
- If yes –> overflow
What is Hirschprung’s disease and how much bowel is usually affected?
- Absence of ganglion cells from myenteric and submucosal plexuses of part of large bowel
- → Narrow, contracted segment
- Abnormal bowel extends from rectum for variable distance, ending in normally innervated, dilated colon
- 75% - confined to rectosigmoid
- 10% entire colon is involved
Presenting features of Hirschprung’s disease?
- Usually in neonatal period
- Failure to pass meconium in 1st 24h
- Abdo distension
- Bile-stained vomit
- PR may reveal narrowed segment
- Withdrawal of examining finger often releases gush of liquid stool and flatus
- Temporary improvement in obstruction following PR can delay diagnosis
- Occasionally present with life-threatening Hirschprung enterocolitis during 1st few wks, sometimes due to C. Diff
- Later childhood presentation = chronic constipation, usually profound + abdo distension (no soiling)
- Growth failure may present
How is Hirschprung’s diagnosed?
Suction rectal biopsy
- Absence of ganglion cells
- Presence of large Ach-esterase +ve nerve trunks
Anorectal manometry or barium studies
- May be useful
- Give surgeon idea of length of a ganglionic segment
- Unreliable for diagnosis
Management of Hirschprung’s?
- Surgical
- Initial colostomy
- Followed by anastomosing normally innervated bowel to anus
Most common cause of gastroenteritis in children?
Rotavirus
- Esp winter/spring
What bacteria is most common cause of gastroenteritis?
Campylobacter jejuni
- Associated w/ severe abdo pain
- Yet bacterial causes much less common
Ddx of gastroenteritis? (6)
- Systemic infection – septicaemia, meningitis
- Local infection – resp tract, otitis media, Hep A, UTI
- Surgical disorders – pyloric stenosis, intussusception, acute appendicitis, necrotizing enterocolitis, Hirschprung disease
- Metabolic disorder – diabetic ketoacidosis
- Renal disorder – haemolytic uraemic syndrome
- Other – coeliac disease, cow’s milk protein intolerance, adrenal insufficiency
→ If in doubt, hospital referral essential
Indicators that diagnosis is not gastroenteritis?
- Temp >38 (<3m) or <39 (<3m)
- SOB or tachypnoeic
- Altered conscious state
- Neck stiffness
- Bulging fontanelle
- Non-blanching rash
- Blood +/- mucus in stool
- Bilious (green) vomit
- Severe or localized abdo pain
- Abdo distension or rebound tenderness
When is stool microscopy indicated in suspected gastroenteritis?
- Suspect septicaemia
- Blood or mucus in stool
- Child is immunocompromised
- Consider if recently abroad, diarrhea not improved in 7d, uncertain about diagnosis
Main complication of gastroenteritis?
Dehydration
Pos –> shock
What factors in gastroenteritis put the child at increased risk of dehydration? (5)
- Infants, esp <6m or those with low birth weight (greater SA:weight → more water losses)
- If passed >6 diarrhoeal stools in 24h
- Vomitted ≥3x in 24h
- Unable to tolerate (or not offered) extra fluids
- If malnourished
3 categories of dehydration?
- No clinically detectable dehydration (usually <5% loss of body wt)
- Clinical dehydration (5-10%)
- Shock (>10%)
What do you look at when assessing hydration status? (13)
- General appearance
- Consciousness level
- Urine output
- Skin colour
- Extremeties temperature
- Eyes (sunken or not)
- Mucous membranes
- Heart rate
- Resp rate
- Peripheral pulses (strong/weak)
- Cap refill
- Skin turgor
- BP
Red flags when assessing hydration? (6)
- Appears unwell or deteriorating
- Altered responsiveness eg irritable, lethargic
- Sunken eyes
- Tachycardia
- Tachypnoea
- Reduced skin turgor
–> Helps to identify those who may progress to shock
Signs of hypernatraemic dehydration? (5)
- Jittery movements
- Increased muscle tone with hyperreflexia
- Altered consciousness
- Seizures
- Multiple, small cerebral haemorrhages
Treatment of
a) Clinical dehydration
b) Shock?
a) Oral rehydration solution
- Fluid deficit replacement fluids (50ml/kg)
- Over 4h
- + maintenance requirement
- Continue breast feeding
- IV therapy if deteriorate/continual vomiting
b) IV therapy
- Rapid infusion of 0.9%NaCl
- Repeat if nec
- Fluid deficit = 100ml/kg
- Maintenance fluids
When are abx indicated in gastroenteritis? (6)
- Suspected or confirmed sepsis
- Extraintestinal spread
- Salmonella if <6m
- Malnourished
- Immunocompromised
- Specific bacterial or protozoal infections (e.g. C. Diff associated with pseudomembranous colitis, cholera, shigellosis)
Ddx for vomiting in infants? (8)
- GORD
- Feeding problems
- Infection
- Dietary protein intolerances
- Intestinal obstruction
- Inborn errors of metabolism
- Congenital adrenal hyperplasia
- Renal failure
Types of intestinal obstruction in infants? (8)
- Pyloric stenosis
- Atresia (duodenal, other)
- Intussusception
- Malrotation
- Volvulus
- Duplication cysts
- Strangulated inguinal hernia
- Hirschprung disease
Types of intestinal obstruction in preschool children? (5)
- Intussusception
- Malrotation
- Volvulus
- Adhesions
- Foreign body
Causes of vomiting in preschool children? (8)
Infection (gastro most common) Appendicitis Intestinal obstruction Raised ICP Coeliac Renal failure Inborn errors of metabolism Torsion of testes
Causes of vomiting in school age children and adolescents? (13)
Infection Peptic ulceration (h pylori) Appendicitis Migraine Raised ICP Coeliac Renal failure DKA Alcohol/drug Cyclical vomiting syndrome Bulimia/anorexia Pregnancy Torsion of testes
Sx of GORD?
- Frequent regurgitation/vomiting
- Putting on weight and otherwise well
Severe reflux more common if? (3)
Cerebral palsy
Preterm
After surgery for oesophageal atresia
Management of GORD?
If uncomplicated:
- → Excellent prognosis
- Thickening agents (e.g. Nestargel, Carobel) and positioning in a 30° headup prone position after feeds
More significant GORD:
- H2 receptor antagonists (e.g. ranitidine) or PPIs
- Drugs which enhance gastric emptying (e.g. domperidone) may be tried
- If no response, other diagnoses such as cow’s milk protein allergy should be considered and further Ix performed
Surgical management:
- Reserved for children with complications unresponsive to intensive medical treatment or oesophageal stricture
Complications of GORD? (7)
- FTT from severe vomiting
- Oesophagitis – haematemesis, discomfort on feeding or heartburn, iron deficiency anaemia
- Recurrent pulmonary aspiration – recurrent pneumonia, cough or wheeze, apnoea in preterm infants
- Dystonic neck posturing – Sandifer syndrome
- ALTE
- SIDS
- Barrett’s – v rare, managed with PPIs
How does malabsorption manifest? (3)
- Abnormal stools
- FTT or poor growth in most but not all
- Specific nutrient deficiencies, either singly or in combination
Ddx with malabsorption? (9)
- Coeliac
- Food allergy/intolerance
- Cholestatic liver disease/biliary atresia
- Lymphatic leakage/obstruction
- Short bowel syndrome
- Loss of terminal ileal function
- Exocrine pancreatic dysfunction
- SI mucosal disease
- Parasitic infection
Classical presentation of coeliac?
- Profound malabsorptive syndrome at 8–24m after introduction of wheatcontaining weaning foods
- →FTT, abdo distension, buttock wasting abnormal stools and general irritability
- However, this is no longer the most common presentation and children are now more likely to present less acutely in later childhood
Clinical features of coeliac?
- Highly variable
- Mild, non-specific GI sx eg diarrhea or vomiting
- Anaemia (iron +/or folate deficiency)
- Growth failure
- Alternatively, identified on screening of children at increased risk (T1DM, autoimmune thyroid disease, Down syndrome) and 1st degree relatives of individuals with known coeliac disease
Diagnosis of coeliac?
- Serology (must eat gluten 6w prior) - IgA tissue transglutaminase antibodies
- Confirmed by SI biopsy
Difference b/w food allergy and food intolerance?
Food allergy = immune mediated (usually IgE)
–> Allergy sx = urticaria to anaphylaxis 10-15m after ingestion
Food intolerance = non-immunological hypersensitivity reaction
–> Sx= usually GI - D+V, hours after ingestion
How is diagnosis of food allergy usually made? (3)
Skin prick test
Measurement of IgE in blood
Gold standard test = double blinded placebo controlled food challenge
- Rarely done
What is a type 1 and type 2 reaction in food allergies?
Type 1 reaction:
- Usually instant
- Where body suffers notable itching, swelling, hives and breathing difficulties
- Whilst most of these reactions are mild, some can be more serious and result in an anaphylactic shock
Type 2 reaction:
- Not immediate → difficult to detect
- Different foods breakdown at different rates so a reaction can be anywhere from 24-72 hours after ingestion
How is food allergy managed?
- Avoid food
- Give parents written advice and training about how to manage allergic attack
- Oral antihistamines if mild (no cardio-resp sx)
- IM epinephrine if severe
What is cow’s milk protein allergy and intolerance?
Allergy: Immune response to cow's milk protein Most likely IgE mediated --> hives, rashes, wheezing - Within 2h
Intolerance:
Not immune mediated
–> GI reactions
- Usually much later
How is cow’s milk protein allergy/intolerance managed?
If breast feeding eliminate from mother’s diet and take calcium supplements instead
If bottle fed, use hydrolysed (a-a) formula
Resolved in 90% by 6y
What is toddler diarrhoea?
= Chronic non-specific diarrhoea
- Commonest cause of persistent loose stools in preschool children
- Stools are of varying consistency, sometimes well formed, sometimes explosive and loose
- Presence of undigested vegetables common
- Affected children are well and thriving
- No precipitating dietary factors
Aetiology:
- Probably results from underlying maturational delay in intestinal motility which leads to intestinal hurry
- Loose stools not due to malabsorption
- Most grown out of their sx by 5y
- Achieving faecal continence may be significantly delayed
Management of toddler diarrhoea?
- Some relief of symptoms can be achieved by ensuring that child’s diet contains adequate fat (which slows gut transit) and fibre
- XS consumption of fresh fruit juice, esp those high in non-absorbable sorbitol, can exacerbate sx
Causes of diarrhoea in children? (6)
- Toddler diarrhoea
- Cow’s milk protein allergy
- Coeliac
- Gastroenteritis
- Lactose intolerance
- Following bowel surgery with malabsorption
Where does Crohn’s most commonly affect?
Distal ileum
Proximal colon
Presentation of Crohns in children/adolescents? (4)
Growth failure/ puberty delay
Classical presentation (25%) - Abdo pain, diarrhoea, wt loss
Gen ill health
- Fever, lethargy, wt loss
Extra-intestinal
- Oral lesions, perianal skin tags, uveitis, arthralgia, erythema nodosum
What do blood tests show in Crohn’s?
- Raised inflammatory markers (platelet count, ESR and CRP)
- Iron deficiency anaemia
- Low serum albumin
→ Helpful in both making a diagnosis and confirming a relapse
How is Crohn’s diagnosed?
- Based on endoscopic and histological findings on biopsy
- Upper GI endoscopy, ileocolonoscopy and small bowel imaging required
- Histological hallmark = presence of non caseating epithelioid cell granulomata
- → Although this is not identified in up to 30% at presentation
- Small bowel imaging may reveal narrowing, fissuring, mucosal irregularities and bowel wall thickening
How is Crohn’s remission induced?
- Nutritional therapy
- –> Normal diet replaced by whole protein modular feeds (polymeric diet) for 6–8w
- Effective in 75% of cases
- Systemic steroids required if ineffective
How is relapses/ maintenance of Crohn’s managed?
- Relapse common → immunosuppressant medication (azathioprine, mercaptopurine or methotrexate) may be required to maintain remission
- Anti-tumour necrosis factor agents (infliximab or adalimumab) when conventional treatments failed
- Long-term supplemental enteral nutrition (overnight NG or gastrostomy feeds) helpful in correcting growth failure
- Surgery necessary for complications of Crohn disease – obstruction, fistulae, abscess formation or severe localised disease unresponsive to medical treatment, often manifesting as growth failure
- Long-term prognosis for Crohn’s beginning in childhood is good - most lead normal lives, despite occasional relapsing disease
Clinical presentation of UC?
Characteristically:
- Rectal bleeding
- Diarrhoea
- Colicky pain
- Wt loss and growth failure may occur, although less frequent than Crohn’s
- Extraintestinal complications include erythema nodosum and arthritis
Extra-intestinal manifestations of
a) Crohns? (5)
b) UC? (2)
a) Oral lesions Perianal skin tags Uveitis Arthralgia Erythema nodosum
b) Erythema nodosum
Arthritis
How is UC diagnosed?
- Endoscopy (upper and ileocolonoscopy) + histological features, after exclusion of infective causes of colitis
- → Confluent colitis extending from rectum proximally for variable length
- In contrast to adults, in whom colitis usually confined to distal colon, 90% of children have pancolitis
- Histology reveals mucosal inflammation, crypt damage (cryptitis, architectural distortion, abscesses and crypt loss) and ulceration
- Small bowel imaging required to check extra colonic inflammation suggestive of Crohn’s not present
How is mild UC managed?
- Aminosalicylates (balsalazide and mesalazine) used for induction and maintenance therapy
- Disease confined to rectum and sigmoid colon may be managed with topical steroids
- More aggressive or extensive disease requires systemic steroids for acute exacerbations and immunomodulatory therapy, e.g. azathioprine to maintain remission alone or in combination with low dose corticosteroid therapy
How is severe fulminating UC managed?
= Medical emergency
- IV fluids and steroids
- If this fails to induce remission, may use ciclosporin
- Colectomy with ileostomy or ileorectal pouch undertaken for severe fulminating disease which may be complicated by toxic megacolon, or for chronic poorly controlled disease
What screening must be undertaken in those with UC?
- Increased incidence of adenocarcinoma of colon in adults (1 /200 risk for each year of disease b/w 10-20y from diagnosis)
- Regular colonoscopic screening performed after 10y from diagnosis
What is colic?
- Common sx complex that occurs during 1st few months of life
- Paroxysmal, inconsolable crying or screaming often accompanied by drawing up of knees and passage of XS flatus takes place several times a day, esp in evening
- No firm ev that cause is GI, but often suspected
- Occurs in up to 40% of babies
Features of colic?
- Typically occurs in 1st few wks of life, resolves by 4m
- Benign but v frustrating and worrying for parents → may precipitate NAI in infants already at risk
- Gripe water often recommended but unproven benefit
- If severe and persistent → may be due to cow’s milk protein allergy or GORD
- → Empirical 2w trial of a whey hydrolysate formula followed by trial of antireflux treatment may be considered
What is functional/recurrent abdo pain?
Pain sufficient to interrupt normal activities and lasts ≥3m
- 10% of school aged children
- Cause only identified in <10%
- Pain characteristically periumbilical
- Children otherwise well
- May be manifestation of stress
Prognosis of functional abdo pain?
- ½ rapidly recover
- ¼ take months to recover
- ¼ remain into adulthood with migraine, IBS or functional dyspepsia
Causes of recurrent abdo pain?
> 90% no cause found
GI
- IBS, constipation, dyspepsia, abdo migraine, gastritis, peptic ulcer, IBD, malrotation
Gynae
- Dysmenorrhoea, ovarian cysts, PID
Hepato-biliary/pancreatic
- Hepatitis, gall stones, pancreatitis
Urinary tract
- UTI, PUJ obstruction
Psychosocial
-Bullying, abuse, stress
In abdo pain what do these sx suggest?
a) - Epigastric pain at night, haematemesis
b) Diarrhoea, wt loss, growth failure, blood in stools
c) Vomiting
a) Duodenal ulcer
b) IBD
c) Pancreatitis
In abdo pain what do these sx suggest?
a) Jaundice
b) Dysuria, secondary eneuresis
c) Bilious vomiting and abdo distension
a) Liver disease
b) UTI
c) Malrotation
Sx of gastritis? (7)
- Epigastric pain
- Abdo bloating
- Diarrhoea
- Nausea
- Vomiting – coffee ground appearance = bleeding in stomach
- Melaena
- Loss of appetite
Causes of gastritis? (5)
- H Pylori – not as strong an association as in adults
- NSAIDs
- Chemotherapy
- Pernicious anaemia
- Infections
How is H pylori detected? (3)
- Urease breath test (13C)
- Stool antigens
- Serological testing is unreliable in children
Lifestyle advice to be given in gastritis?
- Eat smaller and more frequent meals
- Avoid irritant foods (acidic, spicy, fried)
- Drink alcohol in moderation
- Avoid NSAIDs
- Manage stress
- Reduce smoking
What is mesenteric adenitis?
Inflamed lymph glands in abdomen which cause abdo pain
- Not usually serious and gets better without treatment
Sx of mesenteric adenitis? (4)
- Pain in abdomen usually centrally or in RIF
- Fever and generally unwell
- N +/- D
- Sore throat or sx of cold before pain started
Ddx of mesenteric adenitis? (2)
- Ectopic pregnancy
- Appendicitis
Is it conjugated or unconjugated bilirubin that gives urine/stools colour?
Conjugated (water soluble)
Is it conjugated or unconjugated bilirubin that can cause kernicterus?
Unconjugated
- Normally bound to albumin
- If albumin is saturated, it is free to cross BBB
Causes of prolonged neonatal jaundice (unconjugated)? (6)
- Breast milk jaundice
- Infection (particularly urinary tract)
- Haemolytic anaemia, e.g. G6PD deficiency
- Hypothyroidism
- High GI obstruction
- Crigler–Najjar syndrome
Causes of prolonged neonatal jaundice (conjugated)? (3 categories)
Due to liver disease, accompanied by pale stools, dark urine, FTT, bleeding tendency
Bile duct obstruction
- Biliary atresia
- Choledochal cyst
Neonatal hepatitis syndrome
- Congenital infection
- Inborn errors of metabolism
- Alpha1-antitrypsin deficiency
- Galactosaemia
- Tyrosinaemia (type 1)
- Errors of bile acid synthesis
- Progressive familial intrahepatic cholestasis (PFIC)
- CF
- Intestinal failure-associated liver disease – associated with long term parental nutrition
Intrahepatic biliary hypoplasia
- Alagille syndrome
What level of conjugated bilirubin is classed as jaundice?
> 20micromol/L
What is kernicterus?
Manifestations? Prognosis?
- Encephalopathy from deposition of unconjugated bilirubin in basal ganglia and brainstem nuclei
- Usually occurs when level of bilirubin exceeds albumin binding capacity in blood
- → Neurotoxic effect can vary in severity from transient disturbance to severe damage and death
Manifestations inc:
- Poor feeding
- Lethargy
- Irritability
- Increased muscle tone and an arched back
–> Infants who survive may develop choreoathetoid cerebral palsy, learning difficulties and sensorineural deafness
What % newborns become visibly jaundiced and why? (3 reasons)
> 50%
- Marked physiological release of Hb from breakdown of RBCs because of high Hb conc at birth
- RBC life span of newborn infants (70 days) is shorter than in adults (120 days)
- Hepatic bilirubin metabolism less efficient in 1st few days of life
Likely causes of jaundice at <24h old? (2)
Haemolytic disorders:
- Rhesus haemolytic disease, ABO
incompatibility, spherocytosis and G6PD deficiency can all cause this
Congenital infection:
- Should inc other abnormal signs such
as growth restriction, hepatosplenomegaly and thrombocytopenic purpura
Likely causes of jaundice 2d-2w old? (4)
Physiological jaundice: normal for most infants
Breast milk jaundice: most common cause affecting up to 15% of healthy breast fed infants and gradually disappears by 4-5w
Dehydration: exacerbated if milk intake poor from delay in establishing breastfeeding - sometimes IV fluids needed
Infection: unconjugated hyperbilirubinaemia from poor fluid intake, haemolysis and reduced hepatic function
Likely causes of jaundice >2w old?
= Persistent neonatal jaundice (5-10% newborns)
Biliary atresia (conjugated) - Important to diagnose promptly as surgical treatment adversely affected by delay
Breast milk jaundice (unconjugated)
Infection (uncongugated)
Congenital hypothyroidism (unconjugated)
How does assessing stool colour help in assessing jaundice?
Pale stool –> lack of conjugated bilirubin
- Most likely biliary tree or post-hepatic obstruction
Management of jaundice?
Monitored
- Check transcutaneous bilirubin, serum bilirubin
- Chart to see if need phototherapy
Phototherapy
- Single if not too high with breaks + feeding etc
- Continuous if v high (IV/enteral feeding/hydration)
Exchange transfusion
- If bilirubin dangerously high (rarely done)
- Continuous phototherapy continued
What is biliary atresia?
- Progressive disease – destruction or absence of extrahepatic biliary tree and intrahepatic biliary ducts
→ Chronic liver failure + death unless surgical intervention
Clinical features of biliary atresia?
- Normal birth weight but FTT as disease progresses
- Mildly jaundiced
- Stools pale + urine dark (after meconium)
- Pale stool warrants investigation always (even if no jaundice)
- Hepatomegaly often present
- Splenomegaly will develop (due to portal HTN)
How is biliary atresia diagnosis confirmed?
Diagnosis confirmed at laparotomy by operative cholangiography → fails to outline normal biliary tree
Treatment of biliary atresia?
Surgery
- Success rate diminishes with age (80% <60d)
- Even if successful often progression to cirrhosis and portal HTN
Liver transplant if fails
- Commonest reason for paediatric liver transplant
