PAEDS - GI/LIVER TO DO Flashcards

1
Q

IBD
What is the clinical presentation of Crohn’s disease?

A
  • Abdominal pain (RLQ), diarrhoea (often non-bloody) + weight loss
  • Failure to thrive
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2
Q

IBD
What is the clinical presentation of Ulcerative colitis?

A
  • PR bleeding (+ mucus), diarrhoea + colicky pain (LLQ)
  • Tenesmus and urgency too
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3
Q

IBD
What extra-intestinal features are seen in…

i) Crohn’s disease?
ii) Ulcerative colitis?
iii) Both?

A

i) Perianal disease = skin tags, anal fissures, abscesses + fistulas, strictures, obstruction
ii) primary sclerosing cholangitis
iii) Arthritis, erythema nodosum, pyoderma gangrenosum, uveitis + episcleritis, finger clubbing

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4
Q

IBD
What are some initial investigations for IBD?

A
  • FBC (microcytic anaemia, raised WCC + platelets)
  • U+Es
  • Low albumin (malabsorb)
  • Raised ESR/CRP
  • Stool MC&S
  • Faecal calprotectin released by intestines when inflamed (useful screening)
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5
Q

IBD
What is the medical management of Crohn’s disease?

A
  • Whole protein modular enteral feed for 6–8w can induce remission
  • Flares = PO prednisolone or IV hydrocortisone
  • Remission = azathioprine, methotrexate or mercaptopurine
  • Biologics (remission) = infliximab, adalimumab
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6
Q

IBD
How do you induce remission in Ulcerative colitis?

A
  • Mild-mod = 1st line topical (rectal) aminosalicylate (5-ASA, mesalazine), add PO if remission not achieved or extensive disease, 2nd line = PO prednisolone
  • Severe = 1st line IV hydrocortisone, 2nd line IV ciclosporin
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7
Q

IBD
How do you maintain remission in Ulcerative colitis?
What should be cautioned?

A
  • PO/PR mesalazine, azathioprine or mercaptopurine
  • Mesalazine can cause acute pancreatitis
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8
Q

IBD
What is the surgical management of Ulcerative colitis?

A
  • Panproctocolectomy = curative as removes disease
  • Pt left with permanent ileostomy or ileo-anal anastomosis (J-pouch) where ileum folded back on itself + fashioned into large pouch that functions as a rectum as it attaches to anus
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9
Q

COELIAC DISEASE
What is the aetiology of coeliac disease?

A
  • Genetics = HLA-DQ2 + HLA-DQ8
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10
Q

COELIAC DISEASE
What is the clinical presentation of coeliac disease?

A
  • Abnormal stools (smelly, diarrhoea, floating)
  • Abdo pain, distension + buttock wasting
  • Failure to thrive, weight loss, fatigue
  • Dermatitis herpetiformis = itchy blistering skin rash, often on abdo
  • Nutrient deficiencies (B12, folate, Fe)
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11
Q

COELIAC DISEASE
What are the characteristic features seen on small intestinal biopsy?

A
  • Villous atrophy
  • Crypt hyperplasia
  • Increased intraepithelial lymphocytes
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12
Q

COELIAC DISEASE
What are some complications of coeliac disease?

A
  • Anaemias
  • Osteoporosis
  • Lymphoma (EATL)
  • Hyposplenism
  • Lactose intolerance
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13
Q

HIRSCHSPRUNG’S DISEASE
What is a complication of Hirschsprung’s disease?

A
  • Hirschsprung-associated enterocolitis (HAEC) = inflammation + obstruction of intestine, sometimes due to C. difficile
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14
Q

HIRSCHSPRUNG’S DISEASE
How does hirschsprung associated enterocolitis (HAEC) present?

A
  • 2-4w after birth = fever, abdo distension, diarrhoea (bloody) + signs of sepsis
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15
Q

PYLORIC STENOSIS
What are some investigations for pyloric stenosis?

A
  • Test feed = visible gastric peristalsis
  • Hyponatraemic, hypokalaemic + hypochloraemic metabolic alkalosis
  • USS = Dx, visualises thickened pylorus
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16
Q

PYLORIC STENOSIS
What is the management of pyloric stenosis?

A
  • Correct fluid + electrolyte disturbances (0.45% saline, 5% dextrose + K+ supplements) before any surgery
  • Laparoscopic Ramstedt’s pyloromyotomy
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17
Q

ABDOMINAL PAIN
What are some causes of acute abdominal pain?

A
  • Surgical = appendicitis, intussusception, Meckel’s, malrotation, mesenteric adenitis
  • Boys = exclude testicular torsion + strangulated inguinal hernia
  • Medical = UTI, DKA, HSP, lower lobe pneumonia
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18
Q

ABDOMINAL PAIN
What are some causes of recurrent abdominal pain?

A
  • No structural cause in >90%
  • GI = IBS, abdominal migraine, coeliac
  • Gynae = ovarian cysts, PID, Mittelschmerz (ovulation pain)
  • Hepatobiliary = hepatitis, gallstones, UTI
  • Psychosocial = bullying, abuse, stress
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19
Q

APPENDICITIS
What are the signs of appendicitis?

A
  • Low grade fever
  • Abdominal pain aggravated by movement
  • RIF tenderness + guarding (McBurney’s point)
  • Rebound + percussion tenderness (precipitated by cough, jump)
  • Rovsing’s sign = LIF pressure causes RIF pain
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20
Q

INTUSSUSCEPTION
What is intussusception and where does it most commonly affect?

A
  • Bowel telescopes (invaginates) into itself (proximal bowel into distal segment)
  • Commonly ileocaecal valve (ileum>caecum)
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21
Q

INTUSSUSCEPTION
What are some signs of intussusception?

A
  • RUQ ‘sausaged-shaped’ mass
  • Redcurrant jelly stool as blood + mucus in stool
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22
Q

MECKEL’S DIVERTICULUM
What is Meckel’s diverticulum?

A
  • Ileal remnant of the vitello-intestinal duct which contains ectopic gastric mucosa or pancreatic tissue
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23
Q

CONSTIPATION
What are some features of constipation?

A
  • Hard or like rabbit droppings (type 1)
  • May have PR bleed if hard
  • Waxing + waning of pain with stool passage
  • Retentive posturing
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24
Q

CONSTIPATION
What are some causes of constipation?

A
  • Usually idiopathic
  • Meds (opiates)
  • LDs
  • Hypothyroidism
  • Hypercalcaemia
  • Poor diet (dehydration, low fibre)
  • Occasionally forceful potty training
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25
CONSTIPATION What are some red flags in constipation?
- Delayed passage of meconium = Hirschsprung's, CF - Failure to thrive = hypothyroid, coeliac - Abnormal lower limb neurology = lumbosacral pathology - Perianal bruising or multiple fissures = ?abuse
26
CONSTIPATION What investigations might you do in constipation?
- Abdo exam may reveal palpable faecal mass - PR examination only by an expert
27
CONSTIPATION What are some complications of constipation?
- Acquired megacolon - Anal fissures - Soiling + behavioural problems - Child may avoid defecating due to pain > constipation + overflow diarrhoea
28
CONSTIPATION What is the medical management of constipation?
- 1st = macrogol (osmotic) laxative like polyethylene glycol + electrolytes (Movicol) - 2nd = lactulose (osmotic) if movicol is not tolerated +/- stimulant e.g. Senna - 3rd = consider enema ± sedation or specialist manual evacuation - Continue for several weeks after regular bowel habit then gradual dose reduction
29
GORD What are the investigations for GORD?
- Usually clinical but if atypical Hx, complications or failed Tx... – 24h oesophageal pH monitoring – Endoscopy + biopsy to identify oesophagitis – Contrast studies like barium meal
30
GORD What are some complications of GORD?
- Failure to thrive from severe vomiting - Oesophagitis = haematemesis, discomfort on feeding or heartburn, Fe anaemia - Aspiration > recurrent pneumonia, cough/wheeze - Sandifer syndrome = dystonic neck posturing (torticollis)
31
GORD What is the management of uncomplicated GORD?
- Small + frequent meals, do not over feed - Regular burping to help milk settle - Keep baby upright after feeds - Trial thickening agents like Nestargel or add Gaviscon to feeds (not at same time)
32
GORD What is the management of more significant GORD?
- Acid suppression = H2 receptor antagonists (ranitidine) or PPI (omeprazole) - Surgical Mx (fundoplication) if complications, unresponsive to intensive medical treatment or oesophageal strictures
33
GASTROENTERITIS What is the difference in gastroenteritis in developing and developed countries?
- Developing = causes thousands of deaths, mostly bacteria from contaminated food - Developed = mostly viral, infants susceptible to dehydration
34
GASTROENTERITIS What are 5 bacteria that can cause gastroenteritis?
- Campylobacter jejuni - E. coli - Shigella - Salmonella - Bacillus cereus
35
GASTROENTERITIS What is the management of campylobacter jejuni?
- Abx considered after isolating organism where pts have severe symptoms or other risk factors - Azithromycin or ciprofloxacin
36
GASTROENTERITIS What E. coli strain is important to be aware of in terms of gastroenteritis? How is it spread? How does it present?
- E. coli 0157 as produces the Shiga toxin - Contact with infected faeces, unwashed salads or contaminated water - Abdominal cramps, bloody diarrhoea + vomiting
37
GASTROENTERITIS What is a complication of E. coli 0157?
- Destroys blood cells + can lead to haemolytic uraemic syndrome - Abx increase this risk so avoid
38
GASTROENTERITIS How is Shigella spread? How does it present? Complication?
- Faeces contaminating drinking water, swimming pools + food - Bloody diarrhoea, abdominal cramps + fever - Shiga toxin > HUS
39
GASTROENTERITIS How is Salmonella spread? How does it present?
- Raw eggs, poultry - Watery diarrhoea ± mucus or blood
40
GASTROENTERITIS What is Bacillus Cereus? How does it present?
- Gram +ve rod spread through inadequately cooked food, grows well on food, classically undercooked or reheated rice - Produces toxin (cereulide) > abdominal cramping + vomiting soon after ingestion, reaches intestines + different toxin causes watery diarrhoea, resolves within 24h
41
GASTROENTERITIS What are signs of clinical shock?
- Pale/mottled - Hypotension - Prolonged CRT - Cold - Decreased GCS - Sunken fontanelle - Weak pulses - Anuria
42
GASTROENTERITIS What is the management of gastroenteritis with clinical dehydration?
- 50ml/kg low osmolarity oral rehydration solution (Dioralyte) in addition to maintenance fluid, - may need NG tube if unable to drink or vomiting
43
BILIARY ATRESIA What is the clinical presentation of biliary atresia?
- Prolonged jaundice >2w - Pale stools + dark urine (obstructive pattern) - Failure to thrive - Hepatosplenomegaly
44
BILIARY ATRESIA What are the investigations for biliary atresia?
- Serum split bilirubin = conjugated elevated - USS abdo gold standard for Dx, laparotomy confirms
45
BILIARY ATRESIA What is the management of biliary atresia?
- Kasai portoenterostomy (attach section of small intestine to opening of liver where bile duct attaches) - Some will need full liver transplant - Success decreases with age so early Dx crucial
46
NEONATAL HEPATITIS What are some investigations for neonatal hepatitis syndrome?
- Deranged LFTs with raised unconjugated + conjugated bilirubin - Liver biopsy = multinucleated giant cells + Rosette formation
47
NEONATAL HEPATITIS What are 4 main causes of neonatal hepatitis?
- Congenital infection - Alpha-1-antitrypsin (A1AT) deficiency - Galactosaemia - Wilson's disease
48
NEONATAL HEPATITIS What is galactosaemia?
- Deficiency of galactose-1-phosphate uridyltransferase (GALT) involved in galactose metabolism (lactose breaks down into galactose)
49
NEONATAL HEPATITIS What are the complications of galactosaemia?
- Rapidly fatal course with shock, DIC + haemorrhage due to gram -ve sepsis - Liver failure, cataracts + Developmental delay if untreated
50
NEONATAL HEPATITIS What is the management of galactosaemia?
- Stop cow's milk, breastfeeding C/I - Dairy-free diet - IV fluids
51
NEONATAL HEPATITIS How does Wilson's disease present?
Sx of copper accumulation - Eyes (Kayser-Fleischer rings) - Brain (Parkinsonism + psychosis) - Kidneys (vit D resistant rickets) - Liver (jaundice)
52
FAILURE TO THRIVE What are the different categories of causes for failure to thrive?
- Inadequate calorie intake (most common) - Malabsorption - Inadequate retention - Increased calorie requirements
53
FAILURE TO THRIVE What is marasmus?
- Severe protein malnutrition - Weight for height >3 standard deviations below the median - Wasted, wrinkly appearance due to severe protein-energy malnutrition
54
FAILURE TO THRIVE How is failure to thrive defined by height?
- Mild = fall across 2 centile lines on growth chart - Severe = fall across 3 centile lines on growth chart
55
FAILURE TO THRIVE How does NICE define faltering growth in children by weight?
- ≥1 centile spaces if birth weight was <9th centile - ≥2 centile spaces if birth weight was 9th–91st centile - ≥3 centile spaces if birth weight was >91st centile - Current weight is below 2nd centile for age, regardless of birth weight
56
FAILURE TO THRIVE What are some investigations for failure to thrive?
- Serial measurements on growth charts for Dx - Full Hx + examination - Measure height + weight > BMI (if >2y) - Calculate mid-parental height - Food diary - Urine dipstick for UTI + coeliac screen (anti-TTG) for 1st line investigations
57
CMPA What is the clinical presentation of cows milk protein allergy (CMPA)?
- Apparent when weaned from breast > formula milk or food with milk - GI = bloating + wind, abdo pain, D+V, failure to thrive - Allergic = urticaria, cough, wheeze, sneezing, itching - Anaphylaxis + angioedema is rare
58
KWASHIOKOR what are the clinical features?
- growth retardation - diarrhoea - anorexia - oedema - defining characteristic - skin/hair depigmentation - abdominal distension with fatty liver
59
KWASHIOKOR what are the investigations?
bloods - FBC, U+E, serum protein, urine dipstick - hypoalbuminaemia - normo/microcytic anaemia - low calcium, magnesium, phosphate and glucose
60
MARASMUS what is it?
a type of protein-energy malnutrition caused by a severe energy (calories) deficiency
61
MARASMUS what are the clinical features?
- height is relatively preserved compared to weight - wasted appearance - muscle atrophy - listless - diarrhoea - constipation
62
HERNIA what are the most common types of hernia that affect children?
indirect inguinal hernia - caused by an opening in the abdominal wall that is present at birth umbilical hernia
63
HERNIA what are the risk factors for developing a hernia?
- premature, underweight babies - male gender - family history - medical conditions - undescended testes, CF - African descent
64
CHOLEDOCHAL CYST How may it present?
- Cholestatic jaundice - abdominal mass - pain in RUQ - nausea and vomiting - fever
65
CHOLEDOCHAL CYST what are the investigations?
can be detected on ultrasound before the child is born after the baby is born, the parent's may notice lump in RUQ, the following tests are then done: - CT scan - cholangiography
66
CHOLEDOCHAL CYST What are the complications?
- Cholangitis - small risk of malignancy
67
CHOLEDOCHAL CYST what are the different types?
Type 1 - cyst of extrahepatic bile duct (most common) Type 2 - abnormal pouch/sac opening from duct Type 3 - cyst inside the wall of the duodenum Type 4 - cysts on both intrahepatic and extrahepatic bile ducts
68
LIVER FAILURE what are the causes?
- chronic hepatitis - biliary tree disease - toxin induced - A1AT deficiency - autoimmune hepatitis - wilson's disease - CF - budd-chiari syndrome - primary sclerosing cholangitis
69
IBD Are granulomas found in Crohn's disease?
Yes - it is granulomatous
70
IBD Are goblet cells present in Crohn's disease?
Yes
71
IBD What is the histology of Crohn's disease?
Non-caseating epithelioid cell granulomata
72
IBD Which layer of the GI tract is affected by ulcerative colitis?
Only the mucosa
73
IBD what is the histology of ulcerative colitis?
- Increased crypt abscesses, - pseudopolyps, - ulcers
74
COELIAC DISEASE What is the pathophysiology?
Autoimmune response to alpha-gliadin portion of protein gluten causes inflammation in small intestine (particularly jejunum)
75
CONSTIPATION What is encopresis?
Involuntary soiling
76
GORD When can it become problematic?
Problematic = chronic cough, hoarse cry, distress after feeding + reluctance to feed, failure to thrive
77
GASTROENTERITIS What is the most common cause?
- Viral rotavirus in paeds, - norovirus in adults
78
GASTROENTERITIS What are some risk factors?
- Poor hygiene, - immunocompromised, - poorly cooked foods
79
GASTROENTERITIS what is the management for shigella infection?
severe = azithromycin or ciprofloxacin
80
GASTROENTERITIS what is the management for shigella infection?
severe = azithromycin or ciprofloxacin
81
GASTROENTERITIS How is shock managed in gastroenteritis?
Rapid IVI (0.9% NaCl 20ml/kg), repeat if necessary, Abx if septicaemia
82
BILIARY ATRESIA What genetic mutation is biliary atresia associated with?
Associated with CFC1 gene mutations
83
NEONATAL HEPATITIS How does it present?
- Intruterine growth restriction (IUGR), - hepatosplenomegaly at birth, - failure to thrive - dark urine
84
NEONATAL HEPATITIS What is the cause of A1AT deficiency?
AR on chromosome 14
85
NEONATAL HEPATITIS What is the presentation of A1AT deficiency?
- Prolonged neonatal jaundice (cholestasis), worse on breast feeding, - can have (prolonged) bleeding due to vitamin K deficiency, - COPD
86
NEONATAL HEPATITIS What is the management for A1AT deficiency?
- ?Transplantation - Never smoke
87
NEONATAL HEPATITIS How does galactosaemia present?
- Poor feeding, - vomiting, - jaundice + hepatomegaly when fed milk
88
NEONATAL HEPATITIS What are the genetics for Wilson's disease?
AR on chromosome 13
89
FAILURE TO THRIVE What are some causes of inability to process nutrients properly?
- T1DM, - inborn errors of metabolism
90
CMPA What is cow's milk protein allergy (CMPA) associated with?
- More common in formula fed babies - those with personal or FHx of atopy
91
NEONATAL HEPATITIS What is the management of Wilson's disease?
Penicillamine for copper chelation