Gastro Flashcards
Crohn’s acronym for features
Crow’s NEST
No blood or mucus in stools (less common)
Entire GI tract
Skip lesions (on endoscopy)
Terminal ileitis (most affected, can affect GIT anywhere from mouth to anus)
Transmural (full thickness) inflammation
Smoking - is a Risk factor! (don’t set the nest on fire)
Strictures and fistulae
UC acronym for features
UC Closeup
Continuous inflammation
Limited to colon and rectum
Only superficial mucosa affected
Smoking is protective
Excrete blood and mucus
Use ASA
PSC
Extra INTESTINAL manifestations of IBD (CD vs UC)
Clubbing
Aphthous ulcers
Arthritis
Uveitis, episcleritis
CD
- Erythema nodosum
- sacroilietis and ank spend and peripheral arthtitis
UC
- Pyoderma gangrenous
- PSC
Management of CD vs UC (basics)
CD Induction
- EEN
- Steroids
- Biologics (infliximab, ADA) - anti TNFalpha Ab
- Surgery is conservative - only to manage emergencies, fistulae, strictures and localised disease
CD maintenance
- Immunomodulators: 6MP and AZA (need to know TPMT genotype)
- Steroids: MTX
- Biologics: TNF alpha inhibitors (infliximab)
UC
- ASAs - topical (suppositories) and/or PO
- Steroid - enemas (topical) and/or PO
UC remission
- Thiopurines: 6MP and AZA or infliximab
- Colectomy (total colectomy and rectum): emergency settings - cures intestinal disease but not EI manifestations
Complications of IBD
- Delayed puberty
- relative indication for escalation in tx or surgery. May need steroid therapy in the meantime - consult endocrine specialists - Stunted growth (reduction in final adult height)
- treat disease
- optimise nutrition
- refer endo for consideration of GH - Metabolic bone disease (from malnutrition, vitamin D deficiency)
- DEXA to monitor
- Ca, Ph, calcitriol supps
- Endo to consider bisphosphonates - Malignant potential (colon cancer and lymphoma)
- after 8 yrs of disease (2 if PSC) need yearly screening colonoscopies
Mx of biliary atresia
- Kasai procedure - palliative, needs to be done before day 60 of life
- Prophylactic abx (prevent cholangitis)
- Nutritional support
- Liver transplant
Cx of biliary atresia
Recurrent cholangitis
Pruritis, jaundice
Cirrhosis
Portal HTN
Liver transplant
Features of alagille syndrome
- Intrahepatic biliary hypoplasia
- Dysmorphic features
- Deep set eyes
- Triangular facies (pointed chin and prominent forehead)
- Straight long nose
- Small low set ears - Cardiac defects (peripheral pulmonary artery stenosis, septal defects, right sided defects)
- Renal (structural and parenchymal abnormalities)
- Skeletal (butterfly vertebrae)
- Ocular (posterior embryuotoxon -> peripheral corneal opacification)
- Delayed gross motor milestones
Complications of cirrhosis
HEPATIC
Hypersplenism
Encephalopathy, oesophageal varicies
Portal HTN (signs include varicies, caput medusae, peripheral edema, jaundice, ascites encephalopathy)
Pruritus (treat with Urso first line, other options available)
Ascities
Thrombosis of portal vein
Infection (SBP and ascending cholangitis)
Coagulopathy (need to be on vitamin K supps)
Carcinoma (hepatocellular)
If decompensated, synthetic function is lost and you will get
- Low albumin
- High INR (coagulopathy as clotting cascade proteins are synthesised in the liver)
Clinical signs of Wilsons disease
AD
Most common cause of fulminant liver failure in kids >3yo
Disorder of copper metabolism -> deposition in liver, heart, kidneys, brainstem, bones, eyes
Liver: signs of portal HTN, decompensated cirrhosis
Eyes: Keiser Fleischer rings
Kidneys: proteinuria, haematuria
Joints: arthritis
HEart: cardiomyopathy, arrhythmias
BRain: movement disorders, dystonia
Ix: low serum Cooper and caeroloplasmin, raised urinary copper
Mx: copper chelation (D penicillamine)
metabolic causes of chronic liver disease
WATCH
Wilsons disease
Alpha 1 antitrypsin deficiency
Tyrosinaemia type 1
Cystic fibrosis
Hereditary fructose intolerance
Side effects of immunosuppressive medications
Malignancy (lymphoma)
Pancyopaenia, neutropenia
Liver transaminase elevation (AST, ALT)
Eczema, psoriasis
Heart failure
Infections!
Immunise BEFORE starting treatment (can’t have live attenuated during treatment)
Neuro - GBS and optic neuritis
Delayed wound healing
Principles of mx of CLD
General monitoring
- Weight
- Albumin
- Bili
- PT (coats)
- Psych
Nutritional support
- high calorie and protein diet (NG, PEG)
- Fat soluble vitamin supplements (Vit AEDK) + zinc, iron, Ca
- May need TPN
Mx of ascites, fluid retention
- Na and water restriction
- Diuretics: spiro +/- furosemide
- +/- IV albumin infusions
- diagnostic ascitic tap only if needed for diagnosis of infections
Hemodialysis if hepatorenal syndrome
Presentation of hepatic encephalopathy
Asterixis
Slow mentation -> drowsy or confusion -> delirium
Could be vague like lack of energy and decline in school performance
+/- EEG changes
Caused by high levels of ammonia (nitrogenous load) as unable to be metabolised by liver
What scoring is used to waitlist patients for liver transplant?
PELD (paediatric end stage liver disease score)
Based on
1. INR
2. total bilirubin
3. serum albumin
4. age under 1 hr
5. height less than 2 SD from mean for age and gender
Complications of liver transplant
Rejection
- Acute (within 3 months of transplant)
- Chronic
Infections
- <3mo: fungal and bacterial
- >3mo: viral and fungal
Biliary strictures
Hepatic and portal vein thrombosis
Post transplant lymphoproliferative disorder (often EBV driven)
AI hepatitis
Recurrence of primary disease (AIH, PSC and vital hepatitis)
Chronic hepatitis
Bowel perforation
Side effects of calcineurin inhibitors (CSA and tacro)
-nephrotoxicity -> HTN
-neurotoxicity
-hyperlipidaemia
GI cx of T21
Duodenal atresia
Hirschsprung disease
Slow transit constipation
Conditions associated with coeliac disease
CF
T1DM
Thyroid disorders
Juvenile arthritis
AI liver disease
crohns disease
IgA deficiency, CVID
What are the main signs/sx of the following vitamin deficiencies
Vitamin A
Vitamin E
Vitamin K
Vitamin D
Zinc
Vit A - night blindness
Vit E - ataxia
Vit K - bruising, bleeding
Vit D - rickets, knock knees, #s
zinc - rashes
Fat globules vs fatty acids (ix)
Fat globules: Maldigestion (luminal)
- ex: CF
Fatty acids: malalbsorption (mucosal)
- ex: Coeliac disease
What does reducing substances test for?
Carbohydrate malabsorption or maldigestion
What cardiac defect is assoc w Alagille syndrome
peripheral pulmonary artery stenosis, septal defects, right sided defects
What ix check synthetic function of liver?
- Low albumin
- High INR (coagulopathy as clotting cascade proteins are synthesised in the liver)
Causes of chronic liver disease
- Chronic hepatitis
- Idiopathic
- Autoimmune
- NAFLD
- Infective/viral: CMV, EBV, HSV, Hep B and hep C - Metabolic
- Glycogen storage disorders
- Galactosaemia
- Tyrosinaemia
- Hereditary frustose intolerance
- Niemann PIck
- Gauchers
- WIlsons - Genetic
- Alpha 1 antitrypsin deficiency
- Haemochromatosis
- CF related liver disease - Biliary
- ALagilles
- Biliary atresia
- PSC - Malignant - HCC
- Congestive cardiac failure
