Gastro Flashcards

1
Q

What are the features of conjugated hyperbilirubinaemia?

A
prolonged/persistent neonatal jaundice (at >2wks)
pale stools
dark urine
bleeding tendency
failure to 
*liver pathology*
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2
Q

What is biliary atresia?

A

1/14000
progressive destruction / absence of extrahepatic biliary tree and intrahepatic biliary ducts
-> chronic liver failure + death without surgery

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3
Q

How might biliary atresia present?

A

mild conjugated jaundice
failure to thrive
hepatomegaly +- splenomegaly

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4
Q

How would you diagnose biliary atresia?

A

LFTs no help
fasting abdo US: may sho contracted/absent gallbladder
radioisotope scan TIBIDA: good liver uptake no excretion into bowel
liver biopsy: extrahepatic biliary obstruction
confirmation on operative cholangiography at laparotomy: fails to outline a normal biliary tree

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5
Q

How do you manage biliary atresia?

A

surgical bypass of fibrotic ducts
hepatoportoenterostomy: Kasai procedure - loop of jejenum anastamosed to cut surface of porta hepatis allowing drainage of any remaining patent ductules
surgery before 60 days = 80% success - bile drainage
if unsuccessful: liver transplant

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6
Q

What are complications of the Kasai procedure?

A

cholangitis
malabsorption of fats and fat soluble vitamins
progression to cirrhosis and portal htn -> liver transplant

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7
Q

What is a choledochal cyst?

A

cystic dilatations of the extrahepatic biliary system
present in infancy with cholestasis
present in older children with abdo pain, palpable mass, jaundice or cholangitis

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8
Q

How would you diagnose and treat choledochal cysts?

A

diagnose on US or radionuclide scanning
surgical excision of cyst with a Roux-en-Y anastamosis ot the biliary duct
complications incl: cholangitis
2% risk malignancy in any part of biliary tree

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9
Q

What are the causes of neonatal hepatitis syndrome?

A
congenital infection
alpha1-antitrypsin deficiency
galactosaemia
tyrosinaemia -T1
CF
inborn errors of metabolism
errors of bile acid synth
PFIC
intestinal failure assoc liver disease - assoc with TPN
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10
Q

How do infants with neonatal hepatitis syndrome present?

A
prolonged neonatal jaundice
hepatic inflammation
IUGR
hepatosplenomegaly at birth
giant cell hepatitis on biopsy
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11
Q

What is alpha 1 antitrypsin deficiency?

A
AR 1/2-4000
Chr 14 protease inhibitor
liver disease assoc with PiZZ phenotype
estimate level in plasma
50% -> liver disease req transplant
emphysema may develop in adult life
avoid smoking!!!
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12
Q

What is galactosaemia?

A

1/40000
poor feeding, vomiting, jaundice + hepatomegaly when fed milk
untreated -> liver failure, cataracts + developmental delay
can be rapidly fatal due to G-ve sepsis -> shock + DIC
screening: galactose in urine
diagnosis: measure galactose-1-phosphate-uridyltransferase in RBCs
galactose free diet prevents progression
ovarian failure and learning difficulties may occur later

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13
Q

What are inborn errors of bile acid synthesis?

A

neonatal cholestasis + normal GGT
screen for elevated cholenoic bile acids in urine
diagnosed on mass spectrometry of urine for bile acids
treatment = ursodeoxycholic acid

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14
Q

What is Progressive Familial Intrahepatic Cholestasis?

A

disorders of bile transporter defects caused by recessive mutations in different genes
presents with jaundice, pruritus, diarrhoea, failure to thrive, rickets, liver disease
low GGT#some req transplant

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15
Q

What is Alagille syndrome?

A

AD
intrahepatic biliary hypoplasia with severe pruritus + failure to thrive
triangular facies, skeletal abnormalities, congenital heart disease, renal tubular disorders, defects in the eye
variable prognosis
50% survive without liver transplant

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16
Q

How does viral hepatitis present?

A
nausea, vomiting, abdo pain, lethargy, jaundice
large tender liver
splenomegaly in some
elevated liver transaminases
coagulation normal
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17
Q

Hep A in children

A

RNA virus
usually a mild illness
recover clinically and biochemically in 2-4wks
some may develop prolonged cholestatic hepatitis or fulminant hepatitis
chronic liver disease doesn’t occur
diagnosis confirmed by detecting IgM antibidy
no treatment
HNIG prophylaxis or vaccination for close contacts

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18
Q

Heb B in children

A
DNA virus
causes acute and chronic liver disease
majority resolve spontaneously
perinatal HepB:
mostly asymptomatic
90% become chronic carriers
older children:
1-2% ->fulminant hepatic failure
5-10% become chronic carriers
anti-HBc= acute infection
HBsAg= ongoing infection
no treatment for acute infection
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19
Q

chronic Hep B in children

A

30-50% will develop chronic Hep B liver disease
of whom 10% progress to cirrhosis
long term risk = hepatocellular carcinoma

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20
Q

Hep B treatment

A

poor efficacy
Interferon:
effective for 50% infected horizontally, 30% perinatally
oral antiviral therapy effective in 23%

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21
Q

preventing HepB

A

antenatal screening of pregnant women: HbsAg
babies with +ve mothers to receive course of hep B vaccination
if mother has HBeAg also give hepBimmunoglobulin

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22
Q

Hep C in children

A

RNA virus
vertical transmission
rarely causes acute infection
2025% lifetime risk cirrhosis/hepatocellular carcinoma

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23
Q

EBV in children

A

usually asymptomatic

40% have hepatitis that may become fulminant

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24
Q

Acute liver failure (fulminant hepatitis) in children

A
development of massive hepatic necrosis
loss of liver function
\+- hepatic encephalopathy
high mortality
mostly due to paracetamol OD and viral hepatitis
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25
How does acute liver failure present?
``` jaundice encephalopathy (irritability, confusion, drowsiness) coagulopahy hypoglycaemia electrolyte disturbance complications incl: cerebral oedema, haemorrhage, sepsis, pancreatitis raised transaminases abnormal coag ```
26
What is posseting?
non forceful return small amounts milk with return of swallowed air
27
What is regurgitation?
frequent non forceful return large amounts of milk may indicate GORD
28
What is gastro-oesophageal reflux?
involuntary passage of gastric contents into the oesophagus | due to inappropriate relaxation of oesophageal sphincter due to functional immaturity
29
What increases risk of gastro-oesophageal reflux in infants?
mainly fluid diet horizontal posture short intra-abdo length of oesophagus
30
what are common symptoms of gastro-oesophageal reflux in infants?
recurrent regurgitation during 1st year normal weight gain otherwise well spontaneous resolution by 12 months
31
What are the complications of gastro-oesophageal reflux?
``` FTT from severe vomiting oesophagitis recurrent pulm aspiration dystonic neck posturing apparent life threatening event ```
32
When is severe reflux from gastro-oesophageal reflux seen?
children with cerebral palsy preterm infants following surgery for oesophageal atresia/diaphragmatic hernia
33
With an atypical history of reflux what investigations might be performed?
24hr oesophageal pH monitoring to quantify degree of acid reflux 24hr impedence monitoring endoscopy + oesophageal biopsy
34
How would you manage gastro-oesophageal reflux?
feed thickeners (nestargel, carobel) 30 degree head up prone position after feed omeprazole or ranitidine
35
What is a Nissen fundoplication?
reserved for children suffering from complications of gastro-oesophageal reflux refractive fundus wrapped around intra-abd oesophagus
36
What is pyloric stenosis?
hypertrophy of the pyloric muscle -> gastric outlet obstruction 2-7 wks boys 4:1 girls
37
How does pyloric stenosis present?
``` vomiting, incr freq and force->projectile ~ 1 month age hunger after vomiting weight loss hypochloraemic metabolic acidosis hyponatraemia, hypokalaemia ```
38
How would you go about confirming diagnosis of pyloric stenosis?
test feed look for visible gastric peristalsis over the abdomen pyloric mass in RUQ? US if clinical examination is not conclusive
39
How would you manage pyloric stenosis?
correct fluid/electrolyte disturbance IVF: 0.45%saline + 5%dextrose with K supplements Ramstedt's pyloromyotomy: division of hypertrophied muscle down to mucosa feed within 6hrs, discharge in 2 days
40
What are the signs + symptoms of acute appendicitis?
central colicky abdo pain -> RIF vomiting anorexia low grade fever abdo pain aggravated by movement guarding at Mc Burney's point
41
What investigations might confirm diagnosis?
generally none very helpful US may support clinical findings other imaging and bloods no use
42
what are the complications of appendicitis?
appendix mass abscess perforation
43
How would you manage appendicitis?
conservative: if no signs of peritonitits, IV abx, appendicetomy after a few wks laparotomy: if symptoms progress if signs of generalised guarding consistent with perforation: fluid resus and IV abx prior to laparotomy
44
What is non-specific abdominal pain?
pain which resolves in 24-48hrs less severe than appendicitis often accompanied by URTI + cervical lymphadenopathy
45
what is intussusception?
Invagination of proximal bowel into a distal segment Mostly ileocaecal junction Blood supply to telescoped bowel is compromised -> ischaemia -> necrosis
46
How does intussusception present?
``` 3 months - 2 years paroxysmal colicky pain - screaming + drawing up legs increasing lethargy reduced feeding vomiting - poss bile stained sausage shaped abdo mass redcurrent jelly stool abdo distension shock ```
47
What are complications of intusussception?
hypovolaemic shock bowel perforation -> peritonitis + gut necrosis
48
How would you manage intussusception?
``` immed fluid resus urgent reduction: attempt rectal air insufflation in theatre 75% success rate if unsuccessful -> surgical reduction ```
49
What is Meckel diverticulum?
2% have one: an ileal remnant of vitello-intestinal duct containing ectopic gastric mucosa or pancreatic tissue most asymptomatic
50
How would you expect a symptomatic Meckel diverticulum to present?
``` severe rectal bleeding neither bright red nor true melaena volvulus around a band diverticulitis technetium scan = incr ectopic gastric mucosa uptake surgical resection req ```
51
What is malrotation?
obstruction or obstruction + compromised blood supply bilious vomiting in first few days of life -> urgent upper G contrast study abdo pain + tenderness urgent surgical correction untwist volvulus and place in non-rotated position
52
What is recurrent abdo pain?
pain sufficient to interrupt normal activities, lasts at least 3 months oft peri-umbilical likely due to either IBS, abdominal migraine, functional dyspepsia
53
How would you manage recurrent abdo pain?
urine M, C + S | abdo US
54
What imaging might confirm diagnosis of intussusception?
abdo XR | abdo US`
55
What is IBS?
assoc with altered GI motility | abnormal sensation of intra-abdominal events
56
How might IBS present?
``` abdo pain relieved by defecation explosive, loose, mucousy stool bloating feeling of incomplete defecation constipation ```
57
When might you suspect GI ulcer in a child?
recurrent abdo pain wakes them at night hx of ulceration in 1st degree rel
58
What does H.pylori do and how is it identified?
``` G-ve bacteria causes nodular antral gastritis produces urease Clo test: Clabelled urea admin PO or gastric/antral biopsy ```
59
What does H.pylori eradication therapy comprise?
PPI: omeprazole/lansoprazole amoxicillin clarithromycin/metronidazolep
60
What do you do if symptoms persist following eradication therapy?
upper GI endoscopy to look for pathology | if normal = functional dyspepsia
61
What symptoms are associated with functional dyspepsia?
recurrent epigastric pain early satiety bloating post prandial vomiting
62
What is the most common cause of gastroenteritis in children living in developed countries?
rotavirus
63
What symptoms might you see with Campylobacter gastroenteritis?
severe abdo pain | blood in stool
64
What symptoms might you expect with Shigella gastroenteritis?
high fever watery stools blood + pus in stool tenesmus
65
Salmonella
?
66
cholera
?
67
What factors increase risk of hypovolaemic shock due to dehydration?
infant 6 diarrhoeal stools in 24 hrs >3 vomits in 24 hrs unable to tolerate fluids malnutrition
68
What is the likely cause of profuse diarrhoea in a child recently treated with broad spec abx?
pseudomembranous colitis C-diff overgrowth cdiff toxin identification in stool
69
fever bloody diarrhoea high urea haemolytic anaemia
``` E.coli 0157 haemolytic uraemic syndrome thrombocytopenia treatment supportive abx contraindicated ```
70
What is transient lactose intolerance?
common sequelae of viral gastroenteritis +ve clinitest: non-absorbed sugar in stool 24hrs oral rehydration solution then normal diet ?cut out lactose, then gradually reintroduce after a few months
71
cows milk protein allergy
?
72
What is coeliac disease?
gluten sensitive enteropathy gliadin -> immunological response in proximal small intestine mucosa villi-> shorter-> absent = flat mucosa
73
What presentation would you classically expect with coeliac disease?
malabsorptive syndrome: FTT, abdo distension, buttock wasting, abnormal stools, irritability 8-24 months
74
How would you test for coeliac disease?
Ttg test: IgA tissue transglutaminase antibodies endomysial antibodies confirmation: jejunal biopsy: mucosal changes and villous atrophy gluten withdrawal: resolution of symptoms + catch up growth gluten free for life!
75
What is Toddler diarrhoea?
AKA chronic non-specific diarrhoea persistent loos stools in otherwise well preschool children undigested vegetables common intestinal hurry due to maturational delay spontaneous resolution by 5yrs for most
76
What is short bowel syndrome?
nutrient, water + electrolyte malabsorption due to congenital abnormalities / resection following NEC
77
What is Hirschsprung disease?
absence of myenteric plexus of rectum and variable distance of colon = narrow contracted segment
78
How does Hirschsprung usually present?
failure to pass meconium in first 24hrs + abdo distension + bilious vomiting in later childhood: profound chronic constipation, abdo distension + growth failure
79
How is Hirschsprung disease diagnosed?
suction rectal biopsy: absence of ganglion cells | anorectal manometry may indicate the extent of the constricted segment and aid surgery but not diagnose
80
How is Hirschsprung disease managed?
colostomy | anastamosing normally innervated bowel to anus
81
What is congenital diaphragmatic herniation?
1/3000, oft diagnosed on antenatal USS, 90% left sided Loops of bowel in left hemithorax, mediastinal shift to right, no gastric bubble Defect occurs during fusion of pleuro-peritoneal membranes to create diaphragm Likely to cause hypoplastic left lung and malrotation of the bowel
82
How might congenital diaphragmatic herniation present?
Tachycardia, tachypnoea, cyanosis Asymmetry of chest wall Absent breath sounds of left Bowel sound audible on chest wall
83
How is congenital diaphragmatic herniation treated?
``` Stabilise Correct and electrolyte disturbance of acidosis Supportive treatment, may req vent Delayed surgery 7-10 days ```
84
How does duodenal atresia present?
``` Hx polyhydramnios Bile stained vomiting in first 24 hrs No abdo distension Normal meconium passage Double bubble on abdo XR 1/3 with DA have Down syndrome Surgical repair req: duodeno-duodenostomy ```
85
Typical signs of appendicitis
``` Poorly defined umbilical pain -> RIF Nausea Anorexia Vomiting Low grade fever Tenderness + guarding over Mc Burney's point ```
86
Atypical signs of appendicitis
Loose stools: 10% due to pelvic/retrocaecal appendix Urinary sx: pelvic appendix V young children abdo pain may not be obvious
87
Inguinal hernia
Commonly indirect Due to patent processus vaginalis More common in premature babies + males Usually R sided due to delayed descent of R testes
88
Volvulus
``` Complete twisting of a loop of bowel around its mesenteric stalk Assoc with malrotation of bowel Commonly presents in neonatal period Can present up to 12 months Bilious vomiting + abdo pain ```
89
What are characteristic histological findings in coeliac disease?
Partial -> total villous atrophy Crypt hyperplasia + lengthening Intraepithelial lymphocytes
90
How is coeliac disease diagnosed?
tTGA IgA EMA IgA Gold standard: duodenal + jejunal biopsy at endoscopy
91
Conditions associated with coeliac disease
Incr risk of enteropathy assoc T cell + non Hodgkin's lymphoma Vitiligo Thyroid disease Dermatitis herpetisformis
92
What causes appendicitis?
``` Obstruction of appendiceal opening into the caecum -> inflammation Obstruction can be caused by: Faecolith Adhesions Lymphoid hyperplasia ```
93
What is mesenteric adenitis?
Inflammation of the mesenteric lymph nodes after a viral URTI Vague central colicky abdo pain Tachycardia, high fever, flushed appearance Evidence of viral infection: lymphadenopathy/pharyngitis Self limiting Conservative management, avoid laparotomy
94
Management of meconium plug in cystic fibrosis
Presentation: bile stained vomiting, abdo distension, no passage of meconium Gastrograffin enema - unless peritonitic! CI Seen in CF Start prophylactic fluclox Sweat test at 6 wks