Paediatrics Gastroenterology Flashcards

(171 cards)

1
Q

What are the causes of abdo pain in children?

A

Non-organic or functional (most common in children over 5)

Medical

Surgical

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2
Q

What are some medical causes of abdo pain?

A

Constipation is very common

UTI

Coeliac disease

IBD

IBS

Mesenteric adenitis

Adbominal migraine

Pyelonephritis

Henoch-Schonlein purpura

Tonsilitis

Diabetic ketoacidosis

Infantile colic

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3
Q

What are the additional causes of abdo pain in adolescent girls?

A

Dysmenorrhoea (period pain)

Mittelschmerz (ovulation pain)

Ectopic pregnancy

Pelvic inflammatory disease

Ovarian torsion

Pregnancy

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4
Q

What are some surgical causes of abdominal pain?

A

Appendicitis - central abso pain spreading to right iliac fossa

Intussusception - colicky non-specific abdo pain with redcurrant jelly stools

Bowel obstruction - pain, distention, absolute constipation and vomiting

Testicular torsion - sudden onset, unilateral testicular pain, nausea and vomiting

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5
Q

Name some red flags for serious abdominal pain?

A

Persistent or bilious vomiting

Severe chronic diarrhoea

Fever

Rectal bleeding

Weight loss or faltering growth

Dysphagia (difficulty swallowing)

Nighttime pain

Abdo tenderness

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6
Q

What initial investigations for abdo pain in children?

A

Anaemia for IBD or Coeliacs

Raised inflammatory markers (ESR and CRP) for IBD

Raised anti-TTG or anti-EMA antibodies for coeliac disease

Raised faecal calprotectin for IBD

Positive urine dipstick for UTI

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7
Q

How is recurrent abdo pain diagnosed in children?

A

Repeated episodes of abdo pain without an identifiable underlying cause (pain is non-organic or functional)

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8
Q

What is the result of recurrent abdo pain?

A

Missed days at school and parental anxiety

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9
Q

What abdo diagnoses overlap?

A

Recurrent abdo pain

Abdo migraine

IBS

Functional abdo pain

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10
Q

What often causes recurrent abdo pain?

A

Stressful life events (loss of relative / bullying)

Theory that its caused by signals from the visceral nerves (the nerves in the gut) with increased sensitivity and inappropriate pain signals

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11
Q

What is the management of recurrant abdo pain?

A

Explanation and reassurance:

  • Distracting the child from the pain with other activities
  • Encourage parents not to ask about the pain
  • Advice about sleep, regular meals, healthy balanced diet, staying hydrated, exercise and reducing stress
  • Probiotic supplements may help symptoms of IBS
  • Avoid NSAIDs e.g. ibuprofen
  • Address psychosocial triggers and exacerbating factors
  • Support from child psychologist
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12
Q

What is an abdominal migraine?

A

Episodic central abdo pain lasting more than 1 hour (examination will be normal) - may occur in young children before they develop traditional migraines as they get older

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13
Q

What is associated with an abdominal migraine?

A
  • Nausea and vomiting
  • Anorexia
  • Pallor
  • Headache
  • Photophobia
  • Aura
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14
Q

What is the general management of abdominal migraine?

A

Similar to adults - careful explanation and education is important

Treating acute attacks

Preventative measures

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15
Q

How to treat the acute attacks in abdominal migraine?

A

Low stimulus environment (quiet, dark room)

Paracetamol

Ibuprofen

Sumatriptan

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16
Q

What are some preventative medications against abdominal migraines?

A

Pizotifen, a serotonin agonist (main one - needs to be withdrawn slowly due to withdrawal - depression, anxiety, poor sleep, tremor)

Propanolol non selective beta blocker

Cyproheptadine, antihistamine

Fluarazine a CCB

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17
Q

What are most cases of constipation caused by?

A

Idiopathic or functional (not a significant cause other than lifestyle factors)

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18
Q

How often may breast fed babies open their bowels?

A

As little as once a week (this is normal)

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19
Q

What are the typical features which suggest constipation?

A
  • Less than 3 stools a week
  • Hard stools, difficult to pass
  • Rabbit dropping stool
  • Straining and painful passage of stools
  • Abdominal pain
  • Retentive posturing
  • Rectal bleeding associated with hard stools
  • Faecal impactation causing overflow soiling with incontinence of particularly loose smelly stools
  • Hard stools palpable in abdomen
  • Loss of sensation of the need to go for stools
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20
Q

What is encopresis?

A

Faecal incontinence (not pathological until 4 years of age) usually a sign of chronic constipation where rectum becomes stretched and looses sensation

Large hard stools remain in rectum whereas loose stools are able to bypass the blockage and leak out, causing soiling

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21
Q

What are some rarer causes of encopresis?

A
  • Spina bifida
  • Hirschprung’s disease
  • Cerebral palsy
  • Learning disability
  • Psychosocial stress
  • Abuse
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22
Q

What lifestyle factors can cause constipation?

A
  • Habitually not opening the bowels
  • Low fibre diet
  • Poor fluid intake and dehydration
  • Sedentary lifestyle
  • Psycosocial e.g. difficult home / school environment (always keep safeguarding in mind)
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23
Q

What causes desensitisation of the rectum?

A

Habit of not opening bowels = loose sensation of needing to open bowels - retain faeces in rectum causing faecal impactation where large hard stools block the rectum leading to desensitisation

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24
Q

What are some secondary causes of constipation?

A

Hirschsprung’s disease

Cystic fibrosis (particularly meconium ileus)

Hypothyroidism

Spinal cord lesions

Sexual abuse

Intestinal obstruction

Anal stenosis

Cows milk intolerance

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25
What are the **red flags** for **constipation**?
**Not passing meconium within 48 hours of birth** (cystic fibrosis or Hirschsprung’s disease) **Neurological signs** particularly in the lower limbs (cerebral palsy or spinal cord lesion) **Vomiting** (intestinal **obstruction or Hirschsprung’s** disease) **Ribbon stool** (anal **stenosis**) **Abnormal anus** (anal stenosis, inflammatory bowel disease or sexual abuse) **Abnormal lower back or buttocks** (spina bifida, spinal cord lesion or sacral agenesis) **Failure to thrive** (coeliac disease, hypothyroidism or safeguarding) **Acute severe abdominal pain and bloating** (obstruction or intussusception)
26
What are some complciations of constipation in children?
**Pain** **Reduced sensation** Anal **fissures** **Haemorrhoids** **Overflow** and soiling **Psychosocial morbidity**
27
When can a diagnosis of **idiopathic constipation** be made?
Without investigations, provided **red flags are considered**
28
How to manage **constipation in children**?
- Correct any reversible contributing factors, recommend a high fibre diet and good hydration - Start laxatives (**movicol** is first line) - **Disimpactation regimen** for faecal impactation with high doses of laxatives at first - Encourage and praise visiting the toilet (**schedule visits, bowel diary** and **start charts**) Laxatives continued long term and slowly weaned off as child develops normal, regular bowel habit
29
What is **gastro-oesophageal reflux**?
Contents from stomach **reflux** through the **lower oesophageal sphincter**
30
Why do babies have more reflux?
**Immaturity** of the **lower oesophageal sphincter** (90% of infants stop having reflux by 1 year)
31
What are some signs of problematic reflux?
- Chronic cough - Hoarse cry - Distress, crying or unsettled after feeding - Reluctance to feed - Pneumonia - Poor weight gain
32
What are the symptoms of GORD in children over 1 year?
Similar to adults with **heartburn**, **acid regurgitation**, **retrosternal** or **epigastric pain**, **bloating** and **nocturnal cough**
33
What are the causes of vomiting?
Overfeeding Gastro-oesophageal reflux Pyloric stenosis (projective vomiting) Gastritis or gastroenteritis Appendicitis Infections such as UTI, tonsillitis or meningitis Intestinal obstruction Bulimia
34
What are some **red flags** for **reflux**?
**Not keeping down any feed** (pyloric stenosis or intestinal obstruction) **Projectile or forceful vomiting** (pyloric stenosis or intestinal obstruction) **Bile stained vomit** (intestinal obstruction) **Haematemesis or melaena** (peptic ulcer, oesophagitis or varices) **Abdominal distention** (intestinal obstruction) **Reduced consciousness, bulging fontanelle or neurological signs** (meningitis or raised intracranial pressure) **Respiratory symptoms** (aspiration and infection) **Blood in the stools** (gastroenteritis or cows milk protein allergy) **Signs of infection** (pneumonia, UTI, tonsillitis, otitis or meningitis) **Rash, angioedema and other signs of allergy** (cows milk protein allergy) **Apnoeas** are a concerning feature and may indicate serious underlying pathology and need urgent assessment
35
What is the management for **simple cases of reflux**?
- Small, frequent meals - Burping regularly to help milk settle - Not over-feeding - Keeping baby upright after feeding (i.e. not lying flat)
36
What do more problematic cases of GORD get treated with?
**Gaviscon mixed with feeds** **Thickened milk or formula** (specific anti-reflux formulas are available) **Omeprazole** where ranitidine is inadequate (not ranitidine as its banned)
37
What further investigation is there for GORD?
**Barium meal** and **endoscopy**
38
What is the treatment for severe GORD?
**Surgical fundoplication**
39
What is **S****andifer's syndrome**?
Rare condition causing **brief episodes** of **abnormal movements** associated with GORD in infants - normally **neurologically normal** - **Torticollis** - **Dystonia** (abnormal muscle contractions causing twisting movements, arching of the back or unusual postures) Condition resolves as reflux is treated or improves
40
What are the differentials of **Sandifer's syndrome**?
**Infantile spasms** (West syndrome) and seizures
41
What is the **pyloric sphincter**?
Ring of smooth muscle between **stomach and duodenum**?
42
What is pyloric stenosis?
**Hypertrophy** (thickening) of the pylorus
43
What does pyloric stenosis typically cause?
**Projectile vomiting** (due to increasing power peristalsis of the stomach as it tries to push food into the duodenum)
44
What are the features of pyloric stenosis?
- First few weeks of life - Baby is **pale, thin** and **failing to thrive** - **Projectile vomiting** - Lump like **large olive** in upper abdomen caused by **hypertrophic muscle** of the **pylorus**
45
What will **blood gas analysis** show for pyloric stenosis?
**Hypochloric** (low cholride) **metabolic alkalosis** as the baby is vomiting the **hydrochloric acid** from the stomach
46
How is **pyloric stenosis diagnosed**?
**Abdominal ultrasound** to visualise the thickened pylorus
47
What is the treatment of **pyloric stenosis**?
**Laparoscopic pyloromyotomy** (aka Ramstedt's operation) - incision is made in the smooth muscle of the pylorus to widen the canal - **prognosis is excellent**
48
What is **acute gastritis**?
**Inflammation** of the **stomach** presenting with nausea and vomiting
49
What is **enteritis**?
Inflammation of the **intestines** and presents with diarrhoea
50
What is **gastroenteritis**?
Inflammation from the stomach to the intestines, presents with nausea, vomiting and diarrhoea
51
What is the most common cause of gastroenteritis in children?
Viral (easily spread)
52
Where to treat patients with viral gastroenteritis?
**Isolated** room
53
What is the main concern with gastroenteritis?
**Dehydration** - are they able to keep themselves hydrated / do they need admission for IV fluids Abx are generally not required
54
What is **steatorrhoea**?
Greasy stool with excessive fat content (suggest pancreatic insufficiency e.g. cystic fibrosis)
55
What key conditions should be thought about in loose stools?
**Infection** (gastroenteritis) IBD **Lactose** intolerance **Coeliac** disease **Cystic fibrosis** **Toddler’s diarrhoea** **IBS** **Medications** (e.g. antibiotics)
56
What are some common causes of **viral gastroenteritis**?
**Rotavirus** **Norovirus** Adenovirus (presents with more subacute diarrhoea)
57
What is **Escherichia Coli**?
Normal intestinal bacteria - certain strains cause gastroenteritis - spread through contact with **infected faeces, unwashed salads** or **contaminated water**
58
What does E.Coli produce?
**Shiga toxin** which causes abdo cramps, bloody diarrhoea and vomiting - destroys red blood cells and leads to **haemolytic uraemic syndrome**
59
What should be avoided if E.coli gastroenteritis is considered?
Antibiotics
60
What is a common cause of **travellers diarhorrea**?
Campylobacter jejuni
61
What does **campylobacter** mean?
"Curved bacteria"
62
What type of bacteria is campylobacter?
**Gram negative** with curved or spiral shape
63
How is campylobacter spread?
Raw / improperly cooked poultry Untreated water Unpasteurised milk
64
How long is the incubation of campylobacter?
2-5 days
65
How long for symptoms of campylobacter to resolve?
3 to 6 days
66
What are the symptoms of campylobacter?
- Abdo cramps - Diarrhoea often with blood - Vomiting - Fever
67
When are antibiotics considered for campylobacter? What are some typical choices?
Severe symptoms / other risks e.g. HIV or heart failure **azithromycin** or **ciprofloxacin**
68
What is **shigella** spread by?
Faeces contaminated drinking water, swimming pools and food
69
What is the incubation period for shigella? How long till symptoms resolve?
1 to 2 days Resolve in 1 week without treatment
70
What are the features of shigella?
Blood diarhoea, abdo cramps and fever
71
What can shigella lead to?
The **shiga toxin** causes **haemolytic uraemic syndrome**
72
What is the treatment of severe shigella cases?
**Azithromycin** or **ciprofloxacin**
73
How is **salmonella** spread?
Eating **raw eggs** or **poultry** or **food contaminated** with the **infected faeces** of small animals
74
What is the **incubation** period of **salmonella**? How long do symptoms take to resolve?
Incubation is 12 hours to 3 days Symptoms resolve within 1 week
75
What are the symptoms of salmonella?
**Watery diarrhoea** which can be associated with mucus / blood Abdo pain Vomiting
76
When are antibiotics used in salmonella?
Severe cases and **guided by stool culture** and **sensitivities**
77
What is **bacillus cereus**?
**Gram positive rod** spread through inadequately cooked foods
78
What is the typical food in bacillus cereus?
**Fried rice** left out at room temperature
79
What **toxin** does **bacillus cereus** produce on the food?
**Cereulide**
80
What does **cereulide** cause?
**Abdo cramping** and **vomiting** within 5 hours of ingestion
81
When does watery diarrhoea occur in bacillus cereus infection?
82
What types of bacteria is **yersinia enterocolitica**?
**Gram negative bacillus**
83
What are the carriers of **yersinia**?
**Pigs** (eating raw / undercooked pork can cause infection)
84
Who does **yersinia** normally affect?
**Children** with watery / bloody diarrhoea
85
What are the features of yersinia?
Lymphadenopathy Fever Abdo pain
86
How long is the incubation of yersinia? How long for symptoms of yersinia to resolve?
4 to 7 days, **illness can last longer than other casues of enteritis** with symptoms lasting 3 weeks or more
87
Why does **yersinia** sometimes get mistaken for **appendicitis**?
Older children or adults present with right sided abdominal pain due to **mesenteric lymphadenitis** (inflammation of the intestinal lymph nodes) and fever
88
When are antibiotics needed for yersinia?
**Severe cases** and guided by stool cultures and sensitivities
89
How can **staph aureus** cause diarrhoea?
Produces **enterotoxins** when growing on foods such as **eggs**, **dairy** and **meat** when eaten these cause small intestine inflammation
90
What are the symptoms of infection with **enterotoxin**?
Diarrhoea Perfuse vomiting Abdo cramps Fever
91
When does infection with enterotoxin show and then resolve?
Within hours of ingestion and settle within 12 to 24 hours
92
What is **giardia lamblia**?
Type of **microscopic parasite** living in the **small intestines of mammals** (pets, farmyard animals or humans)
93
How is giardia lamblia spread?
**Cysts** are released in the stools of infected mammals (these contminate food / water) and **are eaten**
94
How does **giardiasis** present?
No symptoms or **chronic diarrhoea** - diagnosis is made by **stool microscopy**
95
What is the treatment of **giardiasis**?
**Metronidazole**
96
How id gastroenteritis prevented?
Good hygiene
97
How to care for patients **in hospital** with **gastroenteritis**?
**Barrier nursing** and rigorous **infection control**
98
How long should children with **gastroenteritis** stay off of school?
**48 hours** after the symptoms have **completely resolved**
99
How can a causative organism for gastroenteritis be found?
**Microscopy**, **culture** and **sensitivities**
100
What is the general management of gastroenteritis?
Keep hydrated with **fluid challenge** (policy varies with hospital) Recording a **small volume** of fluid given orally every 5-10 minutes (if they can tolerate this then they can be managed at home) **Dioralyte** (rehydration solution) can be used if tolerated Dehydrated children / those which fail the fluid challenge may require **IV fluids** Give dry foods e.g. toast **Antidiarrhoeals/antiemetics are generally not recommended** (loperamide / metoclopramide) **Antidiarrhoeals are particularly not used** in E.Coli 0157 and shigella infections and where there is **bloody diarrhoea/ high fever** Abx only used in patients at risk of complications
101
Name some post gastroenteritis complications?
Lactose intolerance IBS Reactive arthritis Guillain-Barre syndrome
102
What is **coeliac's disease**?
**Autoimmune** condition where **explosure to gluten** causes an **immune reaction** which creates **inflammation** in the **small intestines**
103
When does coeliacs disease usually develop?
**Early childhood** (can start at any age)
104
Where do the autoantibodies target in coeliacs?
**Epithelial cells** of the intestine
105
Name the 2 antibodies in coeliacs?
**Anti-tissue transglutaminase** (anti-TTG) **Anti-endomysial** (anti EMA)
106
Do the antibody levels change in coeliacs?
**Correlate with disease activity** and rise with more active diease (may disappear with effective treatment)
107
Which part of the bowel is particularly affected in coeliacs?
**Jejunum** causing **atrophy** of the **intestinal villi**
108
What does coeliacs lead to?
**Malabsorption of nutrients** and disease related symptoms
109
How does **coeliacs present**?
Often asymptomatic (so have a low threshold for testing) **Failure to thrive** in young children Diarrhoea Fatigue Weight loss Mouth ulcers Anaemia seconds to **iron**, **B12** or **folate deficiency** **Dermatitis herpetiformis** which is an itchy blistering skin rash that typically appears on the abdomen
110
What neurological symptoms can coeliacs present with?
Peripheral neuropathy Cerebella ataxia Epilepsy
111
What disease is strongly linked with **coeliacs?**
Type 1 diabetes (all patients with a new diagnosis are tested for coeliacs)
112
What are the genetic associations with coeliacs?
HLA-DQ2 gene (90%) HLA-DQ8 gene
113
What auto-antibodies are associated with coeliacs?
Tissue transglutaminase antibodies (**anti-TTG**) Endomysial antibodies (**EMAs**) Deaminated gliadin peptides antibodies (**anti-DGPs**)
114
How to **diagnose** coeliacs?
Investigate whilst patient **remains on a diet containing gluten** **Check total immunoglobulin A levels** to exclude **IgA deficiency** before checking for **coeliac disease specific antibodies** * Raised **anti-TTG antibodies** (first choice) * Raised **anti-endomysial antibodies**
115
What do endoscopy and intestinal biopsy show for **coeliacs**?
**Crypt hypertrophy** **Villous atrophy**
116
What are the associations with coeliacs disease?
**Type 1 diabetes** **Thyroid disease** **Autoimmune hepatitis** **Primary biliary cirrhosis** **Primary sclerosing cholangitis** **Down’s syndrome**
117
What are the complications of untreated coeliacs disease?
**Vitamin deficiency** **Anaemia** **Osteoporosis** **Ulcerative jejunitis** Enteropathy-associated T-cell lymphoma (**EATL**) of the intestine Non-Hodgkin lymphoma (**NHL**) **Small bowel adenocarcinoma** (rare)
118
What is the treatment of coeliacs?
Lifelong **gluten free diet** is essentially curative (checking coeliac antibodies can be helpful in monitoring of the disease)
119
What is **inflammatory bowel disease**?
**Ulterative colitis** and **Crohn's disease** (causes inflammation of the GI tract)
120
What is the disease course of IBD?
Periods of **remission** and **exacerbation**
121
What features are **unique to Crohn's?**
**N – No blood or mucus** (these are less common in Crohns.) **E – Entire GI tract** **S – “Skip lesions”** on endoscopy **T – Terminal ileum most affected** and **Transmural** (full thickness) inflammation **S – Smoking is a risk factor** (don’t set the nest on fire)
122
What is associated with Crohn's?
Weight loss Strictures Fistulas
123
What are the features unique to UC?
**C** – Continuous inflammation **L** – Limited to colon and rectum **O** – Only superficial mucosa affected **S** – Smoking is protective **E** – Excrete blood and mucus **U** – Use aminosalicylates **P** – Primary sclerosing cholangitis
124
How does IBD present?
**IBD** should be suspected in children and teenagers presenting with **perfuse diarrhoea**, **abdo pain, bleeding, weight loss** or **anaemia** During flares = fevers, malaise and dehydration
125
What are some **extra-intestinal manifestations** of IBD?
Finger **clubbing** **Erythema nodosum** **Pyoderma gangrenosum** **Episcleritis and iritis** **Inflammatory arthritis** **Primary sclerosing cholangitis** (ulcerative colitis)
126
What is the testing for IBD?
**Faecal calprotectin** (released by intestines when inflammed - useful screening tool) **Endoscopy** (OGD and colonoscopy) with biopsy is the **gold standard investigation** for diagnosis of IBD **Imaging** with ultrasound, CT and MRI can be used to look for complications e.g. **fistulas, abscesses and strictures** (Blood tests for anaemia, thyroid, kidney and liver function - raised CRP indicates active inflammation)
127
What is the general management of IBD?
Referral to secondary care for assessment Managed by **multi-disciplinary team** (paediatricians, specialist nurses, pharmacists, dieticians and surgeons) Monitoring of **growth** and **pubertal development** (particularly when flaring or on steroids) Inducing remission during flares and then maintaining
128
How to **induce remission in Crohn's**?
**Steroids** (oral prednisolone or IV hydrocortisone) If steroids alone dont work then add immunosuppressants: **Azathioprine** **Mercaptopurine** **Methotrexate** **Infliximab** **Adalimumab**
129
How to maintain remission in **Crohn's**?
**First line:** * Azathioprine * Mercaptopurine **Alternatives:** * Methotrexate * Infliximab * Adalimumab
130
When is **surgery** considered in **Crohn's**?
Disease only affects the **distal ileum** (possible to resect this area to prevent further flares) Treat **strictures** and **fistulas**
131
How to induce remission in UC?
**_Mild to moderate disease_** First line: **aminosalicylate** (e.g. mesalazine oral or rectal) Second line: corticosteroids (e.g. prednisolone) **_Severe disease_** **First line: IV corticosteroids** (e.g. hydrocortisone) Second line: IV ciclosporin
132
How to maintain remission in UC?
**Aminosalicylate** (e.g. mesalazine oral or rectal) ## Footnote **Azathioprine** **Mercaptopurine**
133
When is **surgery** used for treatment of **ulcerative colitis**?
**Panproctocolectomy** (removing colon and rectum as UC only usually affects here) Patient is left with **ileostomy** or **ileo-anal anastomosis** (j-pouch) = ileum is folded back on itself and fashioned into a larger pouch **like a rectum** which is then attached to the anus
134
What is **bilary atresia**?
Congenital condition where a section of the bile duct is **narrowed** or **absent** resulting in **ch****olestasis**
135
What else is trapped due to **biliary atresia**?
Conjugated bilirubin
136
When does **biliary atresia** present?
Shortly after birth with significant **jaundice** due to **high conjugated bilirubin levels** (more than 14 days in term babies and 21 days in premature babies)
137
What is the initial investigation for possible **biliar atresia**?
**Conjugated** and **unconjugated bilirubin -** high proportion of **conjugated bilirubin** suggests the liver is processing the bilirubin for excretion by conjugating it - but not able to excrete
138
What are the majority of the causes of jaundice in the neonate?
**Benign** e.g. **breast milk jaundice**
139
What is the management of biliary atresia?
**Sugery** - Kasai portoenterostomy - a section of the small intesting is attached to the opening of the liver where bile duct normally attaches **Full liver transplant**
140
What is **absolute constipation**?
Patient is unable to pass stools or wind
141
What are some causes of **intestinal obstruction**?
**Meconium ileus** **Hirschsprung’s** disease **Oesophageal atresia** **Duodenal atresia** **Intussusception** **Imperforate anus** **Malrotation of the intestines with a volvulus** **Strangulated hernia**
142
How does **intestinal obstruction** present?
**Persistant vomiting** (bilious = bright green bile) **Abdo pain and distention** **Failure to pass stools** or wind **High pitched / tinking bowel sounds** early in the obstruction and **absent** later
143
What is the investigation for intestinal obstruction?
**Abdo x-ray** showing **dilated loops of bowel** proximal to the obstruction and **collapsed loops of bowel** distal to the obstruction ## Footnote **Absence of air in the rectum**
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How are patient with intestinal obstruction managed?
Reffered to **paediatric surgical unit** as an emergency with inital mangement of **nil by mouth** and inserting a **NG** tube to drain the stomach **IV fluids** to correct dehydration / electrolyte imbalances
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What is **Hirschsprung's disease**?
**Congenital condition** where nerve cells of the **myenteric plexus** are **absent in the distal bowel and rectum**
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What is the **myenteric plexus**?
Forms the **enteric nervous system** - brain of the gut aka **Auerbach's plexus**
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What is the **myenteric plexus** made up of?
Web of **neurons, ganglion cells, receptors, synapses** and **neurotransmitters** - responsible for stimulating **peristalsis** of the large bowel
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What is the **key pathophysiology** in **Hirschsprung's disease**?
**Absence of parasympathetic ganglion cells** (during development these cells start higher in the GI tract and gradually migrate down to the distal colon and rectum - here they dont travel all the way down)
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What is it called when the **entire colon** is affected by **Hirschsprungs disease**?
**Total colonic aganglionosis** the aganglionic section of the colon doesnt relax causing it to become constricted causing loss of movement of faeces
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What is associated with **Hirschisprungs?**
**Downs** syndrome **Neurofibromatosis** **Waardenburg syndrome** (genetic condition causing pale blue eyes, hearing loss and patches of white skin and hair) **Multiple endocrine neoplasia type II** **Family hx of Hirschsprung's**
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How does Hirschsprung's disease present?
**Delay in passing meconium** (more than 24 hours) **Chronic constipation since birth** **Abdo pain** and distention **Vomiting** **Poor weight gain** and failure to thrive Can be gradul / acute presentation
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What is **Hirschsprung associated enterocolitis**?
**Inflammation and obstruction of intestine** (occuring in 20% of neonates with Hirschsprung's disease) typically presents within 2-4 weeks of birth with fever, abdo distention, diarrhoea (often with blood) and features of sepsis
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Why is HAEC worrying and how is it treated?
Life threatening - leads to **toxic megacolon** and **perforation of bowel** Treated with **abx, fluid reuscitation** and **decompression** of the **obstructed bowel**
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What is the management of **Hirschsprung's**?
**Abdo x-ray** for diagnosing intestinal obstruction and demonstrating features of HAEC **Rectal biopsy** to confirm the diagnosis (histology will demonstate an **absence of ganglionic cells**) Fluid resuscitation and management of **intestinal obstruction** IV abx in HAEC
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What is the definitive management of Hirschsprung's?
**Surgical removal** of **aganglionic cells** - most have a normal life after surgery but may be some degree of incontinence
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What is **intussusception**?
Condition where bowel '**invaginates**' or '**telescopes**' into itself
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What conditions are associated with intussusception?
**Concurrent viral illness** **Henoch-Schonlein purpura** **Cystic fibrosis** Intestinal **polyps** **Meckel diverticulum**
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How does intusseception present?
- Severe colicy abdo pain - Pale, lethargic and unwell child - "**Redcurrant jelly stool**" - Right upper quadrant mass (sausage shaped) - Vomiting - Intestinal obstruction
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How is intussusception diagnosed?
**Ultrasound scan** or **contrast enema**
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How is intussuception managed?
**Therapeutic enemas** (contrast, water or air are pumped into the colon to force the folded bowel out of the bowel and into the normal position) **Surgical reduction** (if enemas don't work or if bowel becomes gangrenous/perforates)
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What are some complications due to intussuception?
Obstruction Gangrenous bowel Perforation Death
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What is **appendicitis**?
Inflammation of the appendix (a small thin tube sprouting from the caecum) - becomes inflammed due to infection being trapped there by obstruction at the point where the appendix meets the bowel
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What can appendicitis quickly lead to?
**Gangrene** and **rupture** causing **peritonitis**
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When is the peak incidence of appendicitis?
Patients aged 10-20
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What are the features of appendicitis?
**Abdo pain** which migrates from centre to **RIF** Tenderness at **Mc Burney's point** localised area one third from ASIS to umbilicus **Anorexia** (loss of appetite) N&V **Rovsing's sign** (palpation on LIF causes pain in RIF) **Guarding** on abdo palpation **Rebound tenderness** on RIF **Percussion tenderness** which is pain and tenderness when percussing the abdomen
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What do rebound tendernes and percussion tenderness suggest in appendicitis?
**Peritonitis** caused by **ruptured appendix**
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How is **appendicitis** diagnosed?
**Clinical presentation** and **raised inflammatory markers** CT scan can be used to **confirm the diagnosis** - particularly when another diagnosis is more likely **Ultrasound** in female to exclude ovarian and gynae pathology
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What to do if a patient has **clinical presentation** suggestive of appendicitis but investigations are negative, what is the next step?
**Diagnostic laparoscopy** to visualise appendix - can then proceed to an **appendicectomy** during same procedure
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What are the key differentials to appendicitis?
**Ectopic pregnancy** (take a serum / urine bHCG) **Ovarian cysts** (particularly with rupture / torsion) **Meckel's diverticulum** (malformation of distal ileum in 2 % of population, usually asymptomatic but can bleed, become inflammed, rupture or cause a **volvulus** / **intussusception**) - often removed prophylactically if itendified incidentally during other bado operations **Mesenteric adenitis** (inflammed abdo lymph nodes causing abdo pain in younger children associated with tonsillitis or URTI - not specific treatment required) **Appendix mass** - when **omentum** surrounds and sticks to the inflammed appendix, forming a mass in the RIF - managed conservatively with supportive treatment and abx followed by appendicectomy once condition has resolved
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What is the management of appendicitis?
**Emergency admission to hospital** (older children can be managed by adult general surgical teams \>10) **Appendicectomy** **Laparoscopic sugery** = fewer risks and faster recovery compared to open sugery (**laparotomy**)
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What are some complications of appendicectomy?
**Bleeding, infection, pain and scars** **Damage** to bowel, bladder or other organs **Removal of a normal appendix** **Anaesthetic risks** **Venous thromboembolism**