Paediatric Gastroenterology Flashcards

1
Q

Medical Causes of Abdominal Pain

A

Other causes need to be excluded before diagnosing non-organic pain:

Constipation is also very common
Urinary tract infection
Coeliac disease
Inflammatory bowel disease
Irritable bowel syndrome
Mesenteric adenitis
Abdominal migraine
Pyelonephritis
Henoch-Schonlein purpura
Tonsilitis
Diabetic ketoacidosis
Infantile colic
There are addition causes in adolescent girls:

Dysmenorrhea (period pain)
Mittelschmerz (ovulation pain)
Ectopic pregnancy
Pelvic inflammatory disease
Ovarian torsion
Pregnancy

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2
Q

Surgical Causes of Abdominal Pain

A

Appendicitis causes central abdominal pain spreading to the right iliac fossa
Intussusception causes colicky non-specific abdominal pain with redcurrant jelly stools
Bowel obstruction causes pain, distention, absolute constipation and vomiting
Testicular torsion causes sudden onset, unilateral testicular pain, nausea and vomiting

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3
Q

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
Abdominal tenderness
Initial investigations that may indicate the pathology:

Anaemia can indicate inflammatory bowel disease or coeliac disease
Raised inflammatory markers (ESR and CRP) can indicate inflammatory bowel disease
Raised anti-TTG or anti-EMA antibodies indicates coeliac disease
Raised faecal calprotectin indicates inflammatory bowel disease
Positive urine dipstick indicates a urinary tract infection

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4
Q

Recurrent Abdominal Pain

A

A diagnosis of recurrent abdominal pain is made when a child presents with repeated episodes of abdominal pain without an identifiable underlying cause. The pain is described as non-organic or functional. This is common and can lead to psychosocial problems, such as missed days at school and parental anxiety. There is overlap between the diagnoses of recurrent abdominal pain, abdominal migraine, irritable bowel syndrome and functional abdominal pain.

Recurrent abdominal pain often corresponds to stressful life events, such as loss of a relative or bullying. The leading theory for the cause is increased sensitivity and inappropriate pain signals from the visceral nerves (the nerves in the gut) in response to normal stimuli.

Management involves careful explanation and reassurance. Measures that can help manage the pain are:

Distracting the child from the pain with other activities or interests
Encourage parents not to ask about or focus on the pain
Advice about sleep, regular meals, healthy balanced diet, staying hydrated, exercise and reducing stress
Probiotic supplements may help symptoms of irritable bowel syndrome
Avoid NSAIDs such as ibuprofen
Address psychosocial triggers and exacerbating factors
Support from a school counsellor or child psychologist

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5
Q

Abdominal migraine

A

Children are more likely than adults to suffer with a condition called abdominal migraine. This may occur in young children before they develop traditional migraines as they get older. Abdominal migraine presents with episodes of central abdominal pain lasting more than 1 hour. Examination will be normal.

There may be associated:

Nausea and vomiting
Anorexia
Pallor
Headache
Photophobia
Aura

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6
Q

Managing abdominal migraine

A

Management of abdominal migraine is similar to migraine in adults. Careful explanation and education is important. It involves treating acute attacks and preventative measures. Preventative medications are initiated by a specialist.

Treating the acute attack:

Low stimulus environment (quiet, dark room)
Paracetamol
Ibuprofen
Sumatriptan
Preventative medications:

Pizotifen, a serotonin agonist
Propranolol, a non-selective beta blocker
Cyproheptadine, an antihistamine
Flunarazine, a calcium channel blocker
Pizotifen is the main preventative medication to remember for abdominal migraine. It needs to be withdrawn slowly when stopping as it is associated with withdrawal symptoms such as depression, anxiety, poor sleep and tremor.

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7
Q

Constipation

A

Constipation in children is a very common problem in paediatrics. Most cases of constipation can be described as idiopathic constipation or functional constipation, meaning there is not a significant underlying cause other than simple lifestyle factors. It is important to think about possible secondary causes of constipation, such as Hirschsprung’s disease, cystic fibrosis or hypothyroidism.

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8
Q

Presentation of constipation

A

How often someone opens their bowels varies between individuals. This is even more variable in breast-fed babies, which can have as little as one stool a week. Someone opening their bowels daily may be constipated, whereas someone opening their bowels twice a week may not, if that is normal for them.

Typical features in the history and examination that suggest constipation are:

Less than 3 stools a week
Hard stools that are difficult to pass
Rabbit dropping stools
Straining and painful passages of stools
Abdominal pain
Holding an abnormal posture, referred to as retentive posturing
Rectal bleeding associated with hard stools
Faecal impaction causing overflow soiling, with incontinence of particularly loose smelly stools
Hard stools may be palpable in abdomen
Loss of the sensation of the need to open the bowels

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9
Q

Encopresis

A

Encopresis is the term for faecal incontinence. This is not considered pathological until 4 years of age. It is usually a sign of chronic constipation where the rectum becomes stretched and looses sensation. Large hard stools remain in the rectum and only loose stools are able to bypass the blockage and leak out, causing soiling.

Other rarer causes of encopresis include:

Spina bifida
Hirschprung’s disease
Cerebral palsy
Learning disability
Psychosocial stress
Abuse

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10
Q

Lifestyle factors and constipation

A

There are a number of lifestyle factors that can contribute to the development and continuation of constipation:

Habitually not opening the bowels
Low fibre diet
Poor fluid intake and dehydration
Sedentary lifestyle
Psychosocial problems such as a difficult home or school environment (always keep safeguarding in mind)

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11
Q

Desensitisation of the Rectum

A

Often patients develop a habit of not opening their bowels when they need to and ignoring the sensation of a full rectum. Over time they loose the sensation of needing to open their bowels, and they open their bowels even less frequently. They start to retain faeces in their rectum. This leads to faecal impaction, which is where a large, hard stool blocks the rectum. Over time the rectum stretches as it fills with more and more faeces. This leads to further desensitisation of the rectum. The longer this goes on, the more difficult it is to treat the constipation and reverse the problem.

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12
Q

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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13
Q

Red flags for constipation

A

Red flags are things in the history or examination that should make you think about serious underlying conditions that may be causing the constipation. These should prompt further investigations and referral to a specialist:

Not passing meconium within 48 hours of birth (cystic fibrosis or Hirschsprung’s disease)
Neurological signs or symptoms, 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)

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14
Q

Complications of constipation

A

Pain
Reduced sensation
Anal fissures
Haemorrhoids
Overflow and soiling
Psychosocial morbidity

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15
Q

Managing constipation

A

A diagnosis of idiopathic constipation can be made without investigations, provided red flags are considered. It is important to provide adequate explanation of the diagnosis and management as well as reassure parents about the absence of concerning underlying causes. Explain that treating constipation can be a prolonged process, potentially lasting months.

NICE clinical knowledge summaries recommend:

Correct any reversible contributing factors, recommend a high fibre diet and good hydration
Start laxatives (movicol is first line)
Faecal impaction may require a disimpaction regimen with high doses of laxatives at first
Encourage and praise visiting the toilet. This could involve scheduling visits, a bowel diary and star charts.
Laxatives should be continued long term and slowly weaned off as the child develops a normal, regular bowel habit.

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16
Q

Gastro-oesophageal reflux

A

Gastro-oesophageal reflux is where contents from the stomach reflux through the lower oesophageal sphincter into the oesophagus, throat and mouth.

In babies there is immaturity of the lower oesophageal sphincter, allowing stomach contents to easily reflux into the oesophagus. It is normal for a baby to reflux feeds, and provided there is normal growth and the baby is otherwise well this is not a problem, however it can be upsetting for parents. This usually improves as they grow and 90% of infants stop having reflux by 1 year.

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17
Q

Presentation of gastro-oesophageal reflux

A

It is normal for babies to have some reflux after larger feeds. It becomes more troublesome when this causes them to become distressed. Signs of problematic reflux include:

Chronic cough
Hoarse cry
Distress, crying or unsettled after feeding
Reluctance to feed
Pneumonia
Poor weight gain
Children over one year may experience similar symptoms to adults, with heartburn, acid regurgitation, retrosternal or epigastric pain, bloating and nocturnal cough.

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18
Q

Causes of vomiting

A

Vomiting is very non-specific and is often not indicative of underlying pathology. Some of the possible causes of vomiting include:

Overfeeding
Gastro-oesophageal reflux
Pyloric stenosis (projective vomiting)
Gastritis or gastroenteritis
Appendicitis
Infections such as UTI, tonsillitis or meningitis
Intestinal obstruction
Bulimia

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19
Q

Red flags of vomiting

A

Certain features in the history should make you think about serious underlying problems:

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

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20
Q

Managing gastro-oesophageal reflux

A

In simple cases some explanation, reassurance and practical advice is all that is needed. Advise:

Small, frequent meals
Burping regularly to help milk settle
Not over-feeding
Keep the baby upright after feeding (i.e. not lying flat)

More problematic cases can justify treatment with

Gaviscon mixed with feeds
Thickened milk or formula (specific anti-reflux formulas are available)
Proton pump inhibitors (e.g., omeprazole) where other methods are inadequate

Rarely in severe cases they may need further investigation with a barium meal and endoscopy. Surgical fundoplication can be considered in very severe cases, however this is very rarely required or performed.

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21
Q

Sandifer’s Syndrome

A

This is a rare condition causing brief episodes of abnormal movements associated with gastro-oesophageal reflux in infants. The infants are usually neurologically normal. The key features are:

Torticollis: forceful contraction of the neck muscles causing twisting of the neck
Dystonia: abnormal muscle contractions causing twisting movements, arching of the back or unusual postures
The condition tends to resolve as the reflux is treated or improves. Generally the outcome is good. It is worth referring patients with these symptoms to a specialist for assessment, as the differential diagnosis includes more serious conditions such as infantile spasms (West syndrome) and seizures.

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22
Q

Features of pyloric stenosis

A

The pyloric sphincter is a ring of smooth muscle that forms the canal between the stomach and the duodenum. Hypertrophy (thickening) and therefore narrowing of the pylorus is called pyloric stenosis. This prevents food traveling from the stomach to the duodenum as normal.

After feeding, there is increasingly powerful peristalsis in the stomach as it tries to push food into the duodenum. Eventually it becomes so powerful that it ejects the food into the oesophagus, out of the mouth and across the room. This is called “projectile vomiting”.

Features

Pyloric stenosis typically presents in the first few weeks of life, with a hungry baby that is thin, pale and generally failing to thrive. The classic description of vomiting you should remember for your exams is “projectile vomiting”.

If examined after feeding, often the peristalsis can be seen by observing the abdomen. A firm, round mass can be felt in the upper abdomen that “feels like a large olive”. This is caused by the hypertrophic muscle of the pylorus.

Blood gas analysis will show a hypochloric (low chloride) metabolic alkalosis as the baby is vomiting the hydrochloric acid from the stomach. This is a common data interpretation question in exams, so worth remembering.

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23
Q

Managing pyloric stenosis

A

Diagnosis is made using an abdominal ultrasound to visualise the thickened pylorus.

Treatment involves a laparoscopic pyloromyotomy (known as “Ramstedt’s operation“). An incision is made in the smooth muscle of the pylorus to widen the canal allowing that food to pass from the stomach to the duodenum as normal. Prognosis is excellent following the operation.

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24
Q

Gastroenteritis

A

Acute gastritis is inflammation of the stomach and presents with nausea and vomiting. Enteritis is inflammation of the intestines and presents with diarrhoea. Gastroenteritis is inflammation all the way from the stomach to the intestines and presents with nausea, vomiting and diarrhoea.

Gastroenteritis is a very common condition in children. The most common cause of gastroenteritis is viral. It is very easily spread and patients presenting with gastroenteritis often have an affected family member or contact.

It is essential to isolate the patient in any healthcare environment, such as a paediatric assessment unit or hospital ward, as they can easily spread it to other patients.

Dehydration is the main concern. The key to management is establishing whether they are able to keep themselves hydrated or whether they need admission for IV fluids. Antibiotics are generally not recommended or required. Most children make a full recovery with simple supportive management, but beware gastroenteritis can potentially be fatal, especially in very young or vulnerable children with other health conditions.

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25
Q

Differential Diagnosis of Diarrhoea

A

Loose stools are a common complaint and not all cases are caused by gastroenteritis. Stools from normal babies can vary from loose stools several times a day to one stool per week. Steatorrhoea means greasy stools with excessive fat content. This suggests a problem with digesting fats, such as pancreatic insufficiency (think about cystic fibrosis).

Key conditions to think about in patients with loose stools are:

Infection (gastroenteritis)
Inflammatory bowel disease
Lactose intolerance
Coeliac disease
Cystic fibrosis
Toddler’s diarrhoea
Irritable bowel syndrome
Medications (e.g. antibiotics)

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26
Q

Viral gastroenteritis

A

Viral gastroenteritis is common. It is highly contagious. Common causes are:

Rotavirus
Norovirus
Adenovirus is a less common cause and presents with a more subacute diarrhoea.

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27
Q

E. coli

A

Escherichia coli (E. coli) is a normal intestinal bacteria. Only certain strains cause gastroenteritis. It is spread through contact with infected faeces, unwashed salads or contaminated water.

E. coli 0157 produces the Shiga toxin. This causes abdominal cramps, bloody diarrhoea and vomiting. The Shiga toxin destroys blood cells and leads to haemolytic uraemic syndrome (HUS).

The use of antibiotics increases the risk of haemolytic uraemic syndrome, therefore antibiotics should be avoided if E. coli gastroenteritis is considered.

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28
Q

Campylobacter Jejuni

A

Campylobacter is a common cause of travellers diarrhoea. It is the most common bacterial cause of gastroenteritis worldwide. Campylobacter means “curved bacteria”. It is a gram negative bacteria that has a curved or spiral shape. It is spread by:

Raw or improperly cooked poultry
Untreated water
Unpasteurised milk
Incubation is usually 2 to 5 days. Symptoms resolve after 3 to 6 days. Symptoms are:

Abdominal cramps
Diarrhoea often with blood
Vomiting
Fever
Antibiotics can be considered after isolating the organism where patients have severe symptoms or other risk factors such as HIV or heart failure. Popular antibiotic choices are azithromycin or ciprofloxacin.

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29
Q

Shigella

A

Shigella is spread by faeces contaminating drinking water, swimming pools and food. The incubation period is 1 to 2 days and symptoms usually resolve within 1 week without treatment. It causes bloody diarrhoea, abdominal cramps and fever. Shigella can produce the Shiga toxin and cause haemolytic uraemic syndrome. Treatment of severe cases is with azithromycin or ciprofloxacin.

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30
Q

Salmonella

A

Salmonella is spread by eating raw eggs or poultry, or food contaminated with the infected faeces of small animals. Incubation is 12 hours to 3 days and symptoms usually resolve within 1 week. Symptoms are watery diarrhoea that can be associated with mucus or blood, abdominal pain and vomiting. Antibiotics are only necessary in severe cases and should be guided by stool culture and sensitivities.

31
Q

Bacillus Cereus

A

Bacillus cereus is a gram positive rod spread through inadequately cooked food. It grows well on food not immediately refrigerated after cooking. The typical food is fried rice left out at room temperature.

Whilst growing on food it produces a toxin called cereulide. This toxin causes abdominal cramping and vomiting within 5 hours of ingestion. When it arrives in the intestines it produces different toxins that cause a watery diarrhoea. This occurs more than 8 hours after ingestion. All of the symptoms usually resolves within 24 hours.

The typical course is vomiting within 5 hours, then diarrhoea after 8 hours, then resolution within 24 hours.

TOM TIP: The typical exam patient with bacillus cereus develops symptoms soon after eating leftover fried rice that has been left at room temperature. It has a short incubation period after eating the rice before symptoms occur, and they recover within 24 hours. Examiners like this question because the course of bacillus cereus is easy to distinguish from the other causes of gastroenteritis, so if you remember one cause of gastroenteritis this is probably the most likely to come up in exams.

32
Q

Yersinia Enterocolitica

A

Yersinia is a gram negative bacillus. Pigs are key carriers of Yersinia, and eating raw or undercooked pork can cause infection. It is also spread through contamination with the urine or faeces of other mammal such as rats and rabbits.

Yersinia most frequently affects children, causing watery or bloody diarrhoea, abdominal pain, fever and lymphadenopathy. Incubation is 4 to 7 days and the illness can last longer than other causes of enteritis with symptoms lasting 3 weeks or more. Older children or adults can present with right sided abdominal pain due mesenteric lymphadenitis (inflammation in the intestinal lymph nodes) and fever. This can give the impression of appendicitis.

Antibiotics are only necessary in severe cases and should be guided by stool culture and sensitivities.

33
Q

Staphylococcus Aureus Toxin

A

Staphylococcus aureus can produce enterotoxins when growing on food such as eggs, dairy and meat. When eaten these toxins cause small intestine inflammation. This causes symptoms of diarrhoea, perfuse vomiting, abdominal cramps and fever. These symptoms start within hours of ingestion and settle within 12 to 24 hours. It is not actually the bacteria causing the enteritis but the staphylococcus enterotoxin.

34
Q

Giardiasis

A

Giardia lamblia is a type of microscopic parasite. It lives in the small intestines of mammals. These mammals may be pets, farmyard animals or humans. It releases cysts in the stools of infected mammals. The cysts contaminate food or water and are eaten, infecting a new host. This is called faecal-oral transmission.

Infection may not cause any symptoms, or it may cause chronic diarrhoea. Diagnosis is made by stool microscopy. Treatment is with metronidazole.

35
Q

Principles of Gastroenteritis Management

A

Good hygiene helps prevent gastroenteritis. When patients develop symptoms they should immediately be isolated to prevent spread. Barrier nursing and rigorous infection control is important for patients in hospital to prevent spread to other patients. Children need to stay off school until 48 hours after the symptoms have completely resolved.

A sample of the faeces can be tested with microscopy, culture and sensitivities to establish the causative organism and antibiotic sensitivities.

The key to managing gastroenteritis is to ensure they remain hydrated whilst waiting for the diarrhoea and vomiting to settle. Attempt a fluid challenge. Each hospital will have a policy for this. It involves recording a small volume of fluid given orally every 5-10 minutes to ensure they can tolerate it. If they are able to tolerate oral fluid and are adequately hydrated they can usually be managed at home. Rehydration solutions (e.g. dioralyte) can be used if tolerated. Dehydrated children or those that fail the fluid challenge may require IV fluids.

Once oral intake is tolerated a light diet can be slowly reintroduced. Dry foods such as toast may be better tolerated.

Antidiarrhoeal medication such as loperamide and antiemetic medication such as metoclopramide are generally not recommended. Antidiarrhoeal medications are particularly avoided in e. coli 0157 and shigella infections, and where there is bloody diarrhoea or high fever.

Antibiotics should only be given in patients that are at risk of complications once the causative organism is confirmed.

36
Q

Post Gastroenteritis Complications

A

These are possible post-gastroenteritis complications:

Lactose intolerance
Irritable bowel syndrome
Reactive arthritis
Guillain–Barré syndrome

37
Q

Coeliac disease

A

Coeliac disease is an autoimmune condition where exposure to gluten causes an immune reaction that creates inflammation in the small intestine. It usually develops in early childhood but can start at any age.

In coeliac disease autoantibodies are created in response to exposure to gluten. These autoantibodies target the epithelial cells of the intestine and lead to inflammation. There are two antibodies to remember: anti-tissue transglutaminase (anti-TTG) and anti-endomysial (anti-EMA). These antibodies correlate with disease activity and will rise with more active disease and may disappear with effective treatment.

Inflammation affects the small bowel, particularly the jejunum. It causes atrophy of the intestinal villi. The intestinal cells have villi on them that help with absorbing nutrients from the food passing through the intestine. The inflammation causes malabsorption of nutrients and disease related symptoms.

38
Q

Presentation of coeliac disease

A

Coeliac disease is often asymptomatic, so have a low threshold for testing for coeliac disease in patients where it is suspected. Symptoms can include:

Failure to thrive in young children
Diarrhoea
Fatigue
Weight loss
Mouth ulcers
Anaemia secondary to iron, B12 or folate deficiency
Dermatitis herpetiformis is an itchy blistering skin rash that typically appears on the abdomen
Rarely coeliac disease can present with neurological symptoms:

Peripheral neuropathy
Cerebellar ataxia
Epilepsy
TOM TIP: Remember that we test all patients with a new diagnosis of type 1 diabetes for coeliac disease, even if they don’t have symptom, because the conditions are often linked.

39
Q

Genetic associations of coeliac disease

A

HLA-DQ2 gene (90%)
HLA-DQ8 gene

40
Q

Auto-antibodies of coeliac disease

A

Tissue transglutaminase antibodies (anti-TTG)
Endomysial antibodies (EMAs)
Deaminated gliadin peptides antibodies (anti-DGPs)
TOM TIP: Anti-TTG and anti-EMA antibodies are IgA. Some patients have an IgA deficiency. When you test for these antibodies, it is important to test for total Immunoglobulin A levels because if total IgA is low the coeliac test will be negative even when they have the condition. In this circumstance you can test for the IgG version of the anti-TTG or anti-EMA antibodies or do an endoscopy with biopsies.

41
Q

Diagnosing coeliac disease

A

Investigations must be carried out whilst the patient remains on a diet containing gluten otherwise it may not be possible to detect the antibodies or inflammation in the bowel.

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
Endoscopy and intestinal biopsy show:

“Crypt hypertrophy”
“Villous atrophy”

42
Q

Coeliac disease is associated with many other conditions

A

Type 1 diabetes
Thyroid disease
Autoimmune hepatitis
Primary biliary cirrhosis
Primary sclerosing cholangitis
Down’s syndrome

43
Q

Complications of Untreated Coeliac Disease

A

Vitamin deficiency
Anaemia
Osteoporosis
Ulcerative jejunitis
Enteropathy-associated T-cell lymphoma (EATL) of the intestine
Non-Hodgkin lymphoma (NHL)
Small bowel adenocarcinoma (rare)

44
Q

Treating coeliac disease

A

A lifelong gluten free diet is essentially curative. Relapse will occur on consuming gluten again. Checking coeliac antibodies can be helpful in monitoring the disease.

45
Q

Crohn’s versus Ulcerative Colitis

A

Crohn’s and ulcerative colitis have features that are distinct from each other. These features are often tested in exams.

Crohn’s (crows NESTS)

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)
Crohn’s is also associated with weight loss, strictures and fistulas.

Ulcerative Colitis (remember U – C – CLOSEUP)

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

46
Q

Presentation of inflammatory bowel disease

A

Suspect inflammatory bowel disease in children and teenagers presenting with perfuse diarrhoea, abdominal pain, bleeding, weight loss or anaemia. They may be systemically unwell during flares, with fevers, malaise and dehydration.

47
Q

Extra-Intestinal Manifestations of IBD

A

Patients with inflammatory bowel disease can develop signs outside the gastrointestinal system that examiners like to test. It is worth remembering these extra-intestinal manifestations:

Finger clubbing
Erythema nodosum
Pyoderma gangrenosum
Episcleritis and iritis
Inflammatory arthritis
Primary sclerosing cholangitis (ulcerative colitis)

48
Q

Testing for inflammatory bowel disease

A

Blood tests for anaemia, infection, thyroid, kidney and liver function. A raised CRP indicates active inflammation.

Faecal calprotectin is released by the intestines when inflamed. It is a useful screening test and is more than 90% sensitive and specific for IBD in adults.

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 such as fistulas, abscesses and strictures.

49
Q

General management of IBD

A

Any child presenting with symptoms suggestive of inflammatory bowel disease should be referred to secondary care for specialist assessment and management. They will be closely followed up by the multi-disciplinary team. This team includes paediatricians, specialist nurses, pharmacists, dieticians and surgeons if necessary.

It is essential to monitor the growth and pubertal development in children with inflammatory bowel disease, particularly when they are having exacerbations or being treated with steroids.

Management involves a combination of inducing remission during flares and maintaining remission when well. It is essential to monitor and supporting growth and development, and patients are likely to need input from a dietician.

50
Q

Managing Crohn’s

A

This section is based on NICE guidelines last updated May 2019. Please see the full guidelines and talk to seniors before treating patients.

Inducing Remission

First line are steroids (e.g. oral prednisolone or IV hydrocortisone).

If steroids alone don’t work, consider adding immunosuppressant medication under specialist guidance:

Azathioprine
Mercaptopurine
Methotrexate
Infliximab
Adalimumab

Maintaining Remission

Treatment is tailored to individual patients based on risks, side effects, nature of the disease and patient preference. It is reasonable not to take any medications whilst well.

First line:

Azathioprine
Mercaptopurine
Alternatives:

Methotrexate
Infliximab
Adalimumab

Surgery

When the disease only affects the distal ileum it is possible to surgically resect this area to prevent further flares. Crohn’s typically involves the entire GI tract. Surgery can also be used to treat strictures and fistulas secondary to Crohn’s disease.

51
Q

Managing Ulcerative Colitis

A

This section is based on NICE guidelines last updated May 2019. Please see the full guidelines and talk to seniors before treating patients.

Inducing Remission

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

Maintaining Remission

Aminosalicylate (e.g. mesalazine oral or rectal)
Azathioprine
Mercaptopurine

Surgery

Ulcerative colitis usually only affects the colon and rectum. Therefore, removing the colon and rectum (panproctocolectomy) will remove the disease. The patient is then left with either a permanent ileostomy or something called an ileo-anal anastomosis (J-pouch). This is where the ileum is folded back on itself and fashioned into a larger pouch that functions like a rectum. This “J-pouch” is then attached to the anus and collects stools prior to the person passing a motion.

52
Q

Biliary Atresia

A

Biliary atresia is a congenital condition where a section of the bile duct is either narrowed or absent. This results in cholestasis, where the bile cannot be transported from the liver to the bowel. Conjugated bilirubin is excreted in the bile, therefore biliary atresia prevents the excretion of conjugated bilirubin.

Biliary atresia presents shortly after birth with significant jaundice due to high conjugated bilirubin levels. Suspect biliary atresia in babies with a persistent jaundice, lasting more than 14 days in term babies and 21 days in premature babies. The initial investigation for possible biliary atresia is conjugated and unconjugated bilirubin. A high proportion of conjugated bilirubin suggests the liver is processing the bilirubin for excretion (by conjugating it), but it is not able to excrete the conjugated bilirubin because it cannot flow through the biliary duct into the bowel.

There are many causes of jaundice in the neonate. The majority of cases are benign (e.g. breast milk jaundice), however more serious causes such as biliary atresia need to be excluded by measuring the conjugated bilirubin level.

53
Q

Managing biliary atresia

A

Management of biliary atresia is with surgery. The “Kasai portoenterostomy” involves attaching a section of the small intestine to the opening of the liver, where the bile duct normally attaches. This is somewhat successful and can clear the jaundice and prolong survival. Often patients require a full liver transplant to resolve the condition.

54
Q

Causes of intestinal obstruction

A

Intestinal obstruction is where a physical obstruction prevents the flow of faeces through the intestines. This blockage will lead to a back-pressure through the gastrointestinal system, causing vomiting. It also causes absolute constipation, where the patient is unable to pass stools or wind.

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

55
Q

Presentation of intestinal obstruction

A

Persistent vomiting. This may be bilious, containing bright green bile.
Abdominal pain and distention
Failure to pass stools or wind
Abnormal bowel sounds. These can be high pitched and “tinkling” early in the obstruction and absent later.

56
Q

Diagnosing intestinal obstruction

A

The initial investigation of choice is an abdominal xray. This may show dilated loops of bowel proximal to the obstruction and collapsed loops of bowel distal to the obstruction. There will also be absence of air in the rectum.

57
Q

Managing intestinal obstruction

A

Patients presenting with intestinal obstruction need to be referred to a paediatric surgical unit as an emergency. Initial management involves making them nil by mouth and inserting a nasogastric tube to help drain the stomach and stop the vomiting. They will also require IV fluids to correct any dehydration and electrolyte imbalances, and keep them hydrated while waiting for definitive management of the underlying cause.

58
Q

Hirschsprung’s disease

A

Hirschsprung’s disease is a congenital condition where nerve cells of the myenteric plexus are absent in the distal bowel and rectum. The myenteric plexus, also known as Auerbach’s plexus, forms the enteric nervous system. It is the brain of the gut.

This nerve plexus runs all the way along the bowel in the bowel wall, and is a complex web of neurones, ganglion cells, receptors, synapses and neurotransmitters. It is responsible for stimulating peristalsis of the large bowel. Without this stimulation the bowel looses it’s motility and stops being able to pass food along its length.

The key pathophysiology in Hirschsprung’s disease is the absence of parasympathetic ganglion cells. During fetal development these cells start higher in the GI tract and gradually migrate down to the distal colon and rectum. Hirschsprung’s occurs when the parasympathetic ganglion cells do not travel all the way down the colon, and a section of colon at the end is left without these parasympathetic ganglion cells.

The length of colon without innervation varies between patients from a small area to the entire colon. When the entire colon is affected this is called total colonic aganglionosis. The aganglionic section of colon does not relax, causing it to becomes constricted. This leads to loss of movement of faeces and obstruction in the bowel. Proximal to the obstruction the bowel becomes distended and full.

59
Q

Genetics and associations of Hirschsprung’s

A

There are a number of genes on various chromosomes that may have a modifying effect to increase the risk of Hirschsprung’s. A family history of Hirschsprung’s greatly increases the risk. Therefore where you are considering a diagnosis of Hirschsprung’s always take a careful family history.

Hirschsprung’s disease usually occurs in isolation, however it is associated with a number of other syndromes, including:

Downs syndrome
Neurofibromatosis
Waardenburg syndrome (a genetic condition causing pale blue eyes, hearing loss and patches of white skin and hair)
Multiple endocrine neoplasia type II

60
Q

Presentation of Hirschsprung’s

A

The severity of the presentation and the age at diagnosis varies significantly depending on the individual and the extent of the bowel that is affected. It can present with acute intestinal obstruction shortly after birth or more gradually developing symptoms:

Delay in passing meconium (more than 24 hours)
Chronic constipation since birth
Abdominal pain and distention
Vomiting
Poor weight gain and failure to thrive

61
Q

Hirschsprung-Associated Enterocolitis

A

Hirschsprung-associated enterocolitis (HAEC) is inflammation and obstruction of the intestine occurring in around 20% of neonates with Hirschsprung’s disease. It typically presents within 2-4 weeks of birth with fever, abdominal distention, diarrhoea (often with blood) and features of sepsis. It is life threatening and can lead to toxic megacolon and perforation of the bowel. It requires urgent antibiotics, fluid resuscitation and decompression of the obstructed bowel.

62
Q

Managing Hirschsprung’s

A

Abdominal xray can be helpful in diagnosing intestinal obstruction and demonstrating features of HAEC.

Rectal biopsy is used to confirm the diagnosis. The bowel histology will demonstrates an absence of ganglionic cells.

Unwell children and those with enterocolitis will require initial fluid resuscitation and management of the intestinal obstruction. IV antibiotics are required in HAEC.

Definitive management is by surgical removal of the aganglionic section of bowel. Most patients will live a normal life after corrective surgery, although they can have long term disturbances in bowel function and may be left with some degree of incontinence.

63
Q

Intussusception

A

Intussusception is a condition where the bowel “invaginates” or “telescopes” into itself. Picture the bowel folding inwards. This thickens the overall size of the bowel and narrows the lumen at the folded area, leading to a palpable mass in the abdomen and obstruction to the passage of faeces through the bowel. It typically occurs in infants 6 months to 2 years and is more common in boys.

64
Q

Conditions associated with intussusception

A

It is associated with various conditions:

Concurrent viral illness
Henoch-Schonlein purpura
Cystic fibrosis
Intestinal polyps
Meckel diverticulum

65
Q

Presentation of intussusception

A

Severe, colicky abdominal pain
Pale, lethargic and unwell child
“Redcurrant jelly stool”
Right upper quadrant mass on palpation. This is described as “sausage-shaped”
Vomiting
Intestinal obstruction
TOM TIP: Look out for the “redcurrant jelly stool” in your exams as this indicates intussusception as a diagnosis. The other classic feature is the sausage-shaped mass in the abdomen. The typical child in the exam will have had a viral upper respiratory tract infection preceding the illness and will have features of intestinal obstruction (vomiting, absolute constipation and abdominal distention). Ultrasound is the initial investigation of choice.

66
Q

Managing intussusception

A

Diagnosis is made mainly by ultrasound scan or contrast enema.

Therapeutic enemas can be used to try to reduce the intussusception. 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 may be necessary if enemas do not work.

If the bowel becomes gangrenous (due to a disruption of the blood supply) or the bowel is perforated, then surgical resection is required.

67
Q

Complications of intussusception

A

Obstruction
Gangrenous bowel
Perforation
Death

68
Q

Appendicitis

A

Appendicitis is inflammation of the appendix. The appendix is a small, thin tube sprouting from the caecum. The appendix becomes inflamed due to infection trapped in the appendix by obstruction at the point where the appendix meets the bowel. The inflammation can quickly proceed to gangrene and rupture. The appendix can rupture and release faecal content and infective material into the abdomen. This leads to peritonitis, which is inflammation of the peritoneal contents.

The peak incidence of appendicitis is in patients aged 10 to 20 years.

69
Q

Signs and symptoms of appendicitis

A

The key presenting feature of appendicitis is abdominal pain. This typically starts as central abdominal pain, that moves down to the right iliac fossa (RIF) over time and eventually becomes localised in the RIF. On palpation of the abdomen there is tenderness in McBurney’s point. This is a localised area one third the distance from the anterior superior iliac spine (ASIS) to the umbilicus.

Other classic features are:

Loss of appetite (anorexia)
Nausea and vomiting
Rovsing’s sign (palpation of the left iliac fossa causes pain in the RIF)
Guarding on abdominal palpation
Rebound tenderness is increased pain when quickly releasing pressure on the right iliac fossa
Percussion tenderness is pain and tenderness when percussing the abdomen
Rebound tenderness and percussion tenderness suggest peritonitis, caused by a ruptured appendix.

70
Q

Diagnosing appendicitis

A

Diagnosis is based on the clinical presentation and raised inflammatory markers. Performing a CT scan can be useful in confirming the diagnosis, particularly where another diagnosis is more likely. An ultrasound scan is often used in female patients to exclude ovarian and gynaecological pathology.

When a patient has a clinical presentation suggestive of appendicitis but investigations are negative, the next step is to perform a diagnostic laparoscopy to visualise the appendix directly. The surgeon can then proceed to an appendicectomy during the same procedure if indicated.

71
Q

Differentials of appendicitis

A

Ectopic Pregnancy

Consider ectopic pregnancy in girls of childbearing age. This is a gynaecological emergency with a relatively high mortality if mismanaged. A serum or urine bHCG (pregnancy test) to exclude pregnancy is essential in adolescent girls.

Ovarian Cysts

Ovarian cysts can cause pelvic and iliac fossa pain, particularly with rupture or torsion.

Meckel’s Diverticulum

Meckel’s diverticulum is a malformation of the distal ileum that occurs in around 2% of the population. It is usually asymptomatic, however it can bleed, become inflamed, rupture or cause a volvulus or intussusception. They are often removed prophylactically if identified incidentally during other abdominal operations.

Mesenteric Adenitis

Mesenteric adenitis describes inflamed abdominal lymph nodes. This presents with abdominal pain, usually in younger children. This is often associated with tonsillitis or an upper respiratory tract infection. No specific treatment is required.

Appendix Mass

An appendix mass occurs when the omentum surrounds and sticks to the inflamed appendix, forming a mass in the right iliac fossa. This is typically managed conservatively with supportive treatment and antibiotics, followed by appendicectomy once the acute condition has resolved.

72
Q

Managing appendicitis

A

Patients with suspected appendicitis need emergency admission to hospital under the surgical team. Older children, for example those aged above 10 years, can often be managed by adult general surgical teams at local hospitals, provided there is a paediatric department in the hospital. Younger children will need to be admitted under paediatric surgeons.

Removal of the inflamed appendix (appendicectomy) is the definitive management for acute appendicitis. Laparoscopic surgery is associated with fewer risks and faster recovery compared to open surgery (laparotomy).

73
Q

Complications of Appendicectomy

A

Bleeding, infection, pain and scars
Damage to bowel, bladder or other organs
Removal of a normal appendix
Anaesthetic risks
Venous thromboembolism (deep vein thrombosis or pulmonary embolism)