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0
Q

A 10 month old child was born on the 50th centile for weight and height and is now on the 9th centile. Parents report she has poor feeding and cries easily. She has also recently developed a rash across her abdomen. They also report some episodes of vomiting and diarrhoea. Give 3 differentials you would like to consider. (3)

A

Gastroenteritis
Cows milk intolerance/allergy
GORD?
Coeliac disease

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1
Q

What is the commonest cause of an acute abdomen in children? (1)
What radiological investigations could you perform and what would you be looking for? (4)

A

Appendicitis
Abdo xray - dilated loops of bowel and fluid level in RIF
Abdo uss - appendix mass/abscess

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2
Q

How is cows milk intolerance diagnosed? (1)
What alternative formulas are available? (2)
When should cows milk be reintroduce? (1)

A

Remove cows milk from diet and look for symptom resolution.

Soya milk- bad choice as up to 50% will also have same symptoms with soya milk.
Partially hydrolysed formula - whey based
Extensively hydrolysed formulas - caesin based
Amino acid based formula - used if symptoms not improved after use of hydrolysed formula.

Reintroduce after 1 year.

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3
Q

What is coeliac disease? (2)

What changes might be seen on biopsy? (2)

A

Enteropathy in which gliaden portion of gluten provokes a damaging immunological response in the proximal small intestine mucosa.

Mucosal changes - increased epithelial lymphocytes and variable degree of villious atrophy and crypt hypertrophy.

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4
Q

What symptoms may be seen in coeliac disease? (4)

A 
Malabsorption syndrome
    Failure to thrive after weaning
    Abdominal distension
    Buttock wasting
    Abnormal stools
    General irritability
    Anaemia
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5
Q

What other diseases may be associated with coeliac disease? (2)

A

Type 1 DM
Autoimmune thyroid disease
Down syndrome

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6
Q

How is coeliac disease confirmed? (3)

A

Serology - anti tissue transglutaminases (anti-TTG) or endomysial antibodies (both IgA)
Biopsy - jejunal biopsy for changes before diet changes
Diet change - remove gluten from diet
Symptom resolution and rebiopsy should show regrowth.

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7
Q

What nutritional advice should you give for Coeliac disease? (3)

A

Remove all wheat, barley and rye from diet.

Oats are ok.

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8
Q

What skin condition can be associated with coeliac disease? (1)

A

Dermatitis herpetiformis

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9
Q

Define failure to thrive. (2)

A

Demonstration of inadequate weight gain
Mild - fall across two centile lines
Severe - fall across three centile lines

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10
Q

Name 4 causes of failure to thrive. (4)

A 
Functional. ** most common
  Nutritional neglect
  Emotional neglect
  Abuse
  Psychiatric
Organic
  Feeding difficulties
  Poor retention of food
  Poor absorption of food
  Poor metabolism of food
  Increased metabolism
  Chronic disease
  Chromosomal disorders
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11
Q

What is Hirschsprung disease? (3)

What are the 2 types? (1)

A

Congenital condition where ganglionic cells do not complete their normal migration from proximal to distal bowel. Resulting in part of the large bowel without parasympathetic innervation. Having only sympathetic innervation leads to hypertonicity and failure to relax when faced with proximal dilation. This causes narrow and contracted region of bowel with proximal stasis of stool.

Short segment disease ** affects only rectum and sigmoid
Long segment disease affects entire colon

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12
Q

When does Hirschsprungs tend to present? (1)

How does it present? (2)

A

Neonatal- failure to pass meconium within 24 hours
Acute intestinal obstruction - distension, poor feeding, bilious vomiting
Severe life threatening enterocolitis secondary to c diff infection

Infant-
Chronic constipation- distention without soiling
Intermittent abdo pain and fever during episodes of retained faeces
Failure to thrive

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13
Q

What is treatment of Hirschsprung’s disease? (2)

A

Surgery
1- colostomy to decompress dilated colon
2- rectosigmoidectomy and anastomosis of normal bowel to anus and closure of colostomy. (At 3-6 months)

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14
Q

What investigations would help diagnosed Hirschsprung’s disease? (3)

A

AXR: dilated bowel loops and fluid levels
Anorectal manometry: measure of pressure at internal and external anal sphincters in response to rectal distension.
Rectal biopsy: rectal mucosa and submucosa demonstrate absence of ganglion cells with increased amount of Ach stained nerve endings.
Barium study: to estimate length of aganglionic segment.

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15
Q

What is the most common cause of jaundice in a neonate? (1)

What is the most common cause of jaundice in an older child? (1)

A

Physiological jaundice

Infective hepatitis

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16
Q

What causes physiological jaundice in a neonate? (2)

A

Combination of increased red cell breakdown (high levels of Hb in foetus) and immaturity of the hepatic enzymes causes unconjugated hyperbilirubinaemia.
This can also be exaggerated by dehydration if feeding is delayed.

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17
Q

Onset of jaundice in first 24 hours is always pathological. Give 2 examples of differentials you may want to consider. (2)

A

Excess haemolysis
1- immune mediated: rhesus or ABO incompatibility
2- intrinsic red blood cell defects: G6PD or hereditary spherocytosis
Congenital infections

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18
Q

In haemolytic disease of the newborn, which antibodies cross the placenta to cause haemolysis? (1)

A

IgG. Mother always O or rhesus - and creates antibodies against AB or rhesus +

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19
Q

How is rhesus haemolytic disease prevented? (1)

A

Administration of Anti-D immunoglobulin immediately after delivery or after any potentially sensitising events.

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20
Q

What is Coombs test? (1)

A

Blood test used to identify immune mediated haemolysis.

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21
Q

What is the main cause of jaundice between 2 days and 2 weeks of life? (1)

A

Physiological jaundice - peaks of day 3 of life.

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22
Q

What is the definition of prolonged jaundice? (2)

A

Persistent jaundice lasting more than 2 weeks old in a term infant or more than 3 weeks old in a preterm infant.

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23
Q

Name 2 causes of prolonged jaundice? (2)

A

Unconjugated ** - breastfeeding, infection (uti), congenital hypothyroidism, haemolytic anaemia eg SCD
Conjugated (dark urine and pale stools) - biliary atresia, neonatal hepatitis syndrome

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24
Name 3 investigations you would like to perform to determine cause in an infant with prolonged jaundice. (3)
FBC, Blood group (mother and child), fraction of conjugated bilirubin, urine culture, Coombs test, TFTs
25
What is the risk of untreated unconjugated hyperbilirubinaemia? (1)
Kernicterus - deposits of bilirubin the basal ganglia and cerebellum.
26
How does Kernicterus present? (3)
Lethargy, eye rolling, rigidity and seizures. | Long term : choreoathetoid cerebral palsy, sensorineural deafness and learning difficulties.
27
How is it determined when to treat jaundice? (1) | What are the two treatment options? (2)
Charts taking risk factors into account give treatment lines. Phototherapy with blue light converts bilirubin into harmless water soluble metabolites. Exchange transfusion - generally done using umbilical artery and vein catheters. Aliquots of blood are exchanged at a time until a total of twice baby's blood volume.
28
During phototherapy what supportive measure needs to be taken into account? (1)
Extra fluids.. phototherapy increases fluid loss.
29
What are the 4 factors that make up the definition of physiological jaundice? (4)
Onset after 24 hours Resolves within 2 weeks Unconjugated Bilirubin <350 micromols/L
30
Which infections can cause neonatal jaundice? (4)
``` "TORCH" Toxocoplasmosis Rubella CMV Herpes ```
31
A child presents at A+E with history of vomiting, lethargy, abdominal pain and polydipsia. What is the first test you want to perform? (1) What is the likely diagnosis? (1)
Blood glucose | DKA (DM type 1)
32
Name 3 initial blood tests you would perform for a known type 1 diabetic presenting in A+E with recent illness and abdominal pain. (3)
ABGs Blood glucose Capillary ketones U+E's
33
Name 4 physical signs you may see in DKA? (4)
Dehydration- dry mucous membranes, loss of skin turgor, tachycardia and hypotension Acidosis- ketones on breath, Kussmaul breathing Cerebral oedema- Headache, decreased GCS, seizures.
34
What are the 3 criteria for diagnosis of DKA? (3)
Blood glucose >11.1 mmol/L Blood ketones >3.0 mmol/L pH < 7.3 or bicarb < 15 mmol/L
35
Why is blood ketones a superior measure to urinary ketones? (1)
Urine dipsticks only detect the acetoacetic acid and not acetone or beta-hydroxybutyrate. This is the most accurate representation of the blood ketones levels at that moment.
36
What are the management steps for treating DKA?
A- check airway B- 100% oxygen C- ECG, IV cannula take bloods. ?Bolus of fluids 10mg/kg D- glucose levels, assess GCS ?NG tube Fluid replacement- Calculate fluid deficit and maintenance fluids and replace over 48 hours. Insulin- 0.1U/kg/hour IV Potassium replacement
37
What is the risk of quick fluid replacement in DKA? (1)
Cerebral oedema
38
A 3 week old is admitted with persistent vomiting and FTT. Blood tests show a hypochloraemic, hypokalaemic alkalosis. What is the most likely diagnosis? (1) What is the management? (1)
``` Pyloric stenosis (2nd to 4th week old ) Pylorotomy ```
39
A child less than 3 months old has a temperature of 38.1'C, but otherwise appears well. What is the next management? (1)
Admit
40
It is very important to recognise biliary atresia early. What is the main presenting symptom? (1) Is it conjugated or unconjugated? (1) Why is it so important? (2)
``` Destruction or absence of the extra hepatic biliary tree. Prolonged jaundice (>2 weeks) and pale stools (white) Conjugated Biliary atresia causes bile outflow obstruction leading to damage of intrahepatic bile ducts and ultimately liver damage requiring transplantation. ```
41
What is pyloric stenosis? (1) What is the presenting features? (1) What is the characteristic electrolyte imbalance seen? (2)
Hypertrophy of the smooth muscle of the pylorus Persistent projectile non bilious vomiting between 2-8 weeks old, infant still hungry, also weight loss, constipation, mild jaundice and dehydration develop. Hypochloraemic hypokalaemic metabolic alkalosis (loss of gastric acid in vomit and kidneys retaining H+ at expense of K+
42
How is pyloric stenosis diagnosed? (2) | What is the treatment? (1)
Clinically - during test feed peristaltic waves may be visible and pylorus should be palpable as an olive shaped mass in middle upper or right upper quadrant of infants abdomen. Ultrasound can confirm the diagnosis. Pyloromyotomy
43
What is intussusception? (1) What part of the bowel is most commonly affected? (1) What is the accepted normal pathophysiology? (1) What is the classical presenting triad? (3)
Invagination of a proximal part of bowel telescoping into a distal part. Usually occurs just proximal to ileo-caecal valve. Preceding viral infection enlarges Peyer's patches, the enlargement then causes them to get peristalsed into the distal part. Colicky abdo pain, abdo mass and vomiting (possibly bile stained)
44
What is a typical history of intussusception? (3) | What age group is most commonly affected? (1)
Episodes of screaming with legs drawn up to chest and is pale, vomiting (may be bilious), redcurrent jelly stool appears as blood supply becomes compromised (late sign). Age 6-9 months Ultrasound will show target sign. Air or contrast enema can be diagnostic. Reduction by air enema or if not possible surgery is required.
46
What is Meckel's diverticulum? (1) What is the rule of 2's? (4) Why does it often present with painless, severe rectal bleeding? (1)
Remnent of the foetal yolk sac. Occurs in 2% of population, 2 inches long, 2 feet proximal to the ileocaecal valve, usually presents before the age of two Meckels diverticulum often contains ectopic gastric mucosa and so bleeding occurs from peptic ulceration.
47
Name 3 features of "functional" recurrent abdominal pain. (i.e. no organic cause) (3)
Periumbilical pain No associated appetite loss or bowel disturbance Healthy, thriving child with normal physical exam FH of migraine, IBS or recurrent abdo pain.
48
Organic causes of recurrent abdominal pain in children are rare as the cause is usually functional. Name 3 organic causes that you would consider. (3)
``` Recurrent UTI Urinary calculus H pylori gastritis IBD Malrotation with intermittent volvulus Recurrent pancreatitis (others are for single episodes of abdo pain) ```
49
A 10 year old girl attends A+E with a single episode of lower abdominal pain lasting 5 hours. Name 3 surgical and 3 medical causes of acute abdo pain in a child. (6)
Surgical: acute appendicitis, malrotation & volvulus, intussusception, testicular torsion, strangulated inguinal hernia. Medical: constipation, gastroenteritis, mesenteric adenitis, pyelonephritis/UTI, pancreatitis, DKA, HSP, lower lobe pneumonia
50
What is malrotation? (2)
At week 4 of pregnancy, the intestines a a straight tube from stomach to the rectum, between weeks 4-10, the tube moves into the umbilical cord where it elongates and differentiates into large and small bowel. At week 10 the bowel moves back inside the abdomen and coils into cavity, by rotating 270' anti clockwise and fixing to posterior abdominal wall. Failure of this rotation results in the mesentery being short and unattached to the posterior wall. A small mesentery is more prone to twisting as opposed to the normal mesentery that lies obliquely from duodenal-jujenal junction across to caecum.
51
Name 2 complications of malrotation? (2)
Ladd's band may cause intestinal obstruction, and volvulus (twisting of bowel around short mesentery can cut off SMA causing ischaemia and gangrene)
52
What is a volvulus? (2)
Occurring as a complication of malrotation where the mesentery is now short and unattached to the posterior wall, the SMA has become a point of rotation. If a loop of bowel twists completely, the SMA may become occluded and ischaemia may ensue. Also causes small bowel obstruction.
53
A child is brought into your surgery wit his mother, who complains that he is vomiting green/yellow liquid. What do you expect the green/yellow fluid is? (1) What is the main cause of this presentation? (1) What surgical procedure is used to treat? (1)
Bile Bilious vomiting is small bowel obstruction until proven otherwise, e.g. volvulus, duodenal atresia Ladd's procedure
54
What is duodenal atresia? (1) What genetic condition is associated with 1/3 of cases? (1) What is the classical sign seen on x-ray or use? (1) In what situation would you expect non-bilious vomiting in a child with duodenal atresia? (1) How would you expect the amniotic fluid to be affected in the foetus? (1)
Congenital malformation of the duodenum due to failure to recanalise resulting in small bowel obstruction. 1/3 associated with Trisomy 21 Double bubble Usually obstruction occurs just distal to ampulla of Vater, if it occurs just proximal to the ampulla of Vater then there will be no bile in the vomit. Polyhydramnios - not able to swallow fluid due to obstruction.
55
Name 2 causes of double bubble sign on plain X-ray? (2) | What is the physiology behind the double bubble sign? (1
duodenal atresia, annular pancreas, midgut volvulus | Air in distended stomach and proximal duodenum
56
Name 3 causes of gastroenteritis in children. (3)
Rotavirus* (now vaccinated at 2m and 3m) | Norovirus, E.coli, Campylobacter, Shigella
57
A child presents with lower abdominal pain, bloody diarrhoea and weight loss. You suspect a diagnosis of IBD. What investigations would you perform? (3)
Bedside - stool sample, obs, Bloods - FBC (anaemia), CRP (inflamm) Imaging - Upper endoscopy, colonoscopy, some form of imaging the small intestine e.g. barium follow through, MR enterography or capsule endoscopy.
58
For Crohn's disease what is the first line management? (1)
Enteral feeding for 6-8 weeks - steroids are avoided in children due to side effects and enteral feeding has been shown to be as effective.
59
Can UC be treated with an elemental liquid diet? (1)
No, treatment is medical or possibly surgical. Aminosalicylates, corticosteroids, immunosuppressants and biologics (e.g. infliximab) are used. Try to avoid steroids if possible. May also need supplementary calories to maintain growth.
60
Name 1 HLA type that is associated with Coeliac disease. (1) What antibodies might help you make the diagnosis? (2) What might you seen duodenal biopsy? (2)
HLA DR3, DQ2/DQ8 Anti-endomysial antibodies, Anti tissue transglutaminase antibodies Villious atrophy, crypt hyperplasia
61
What event usually precedes lactose intolerance in infants? (1)
Gastroenteritis (lactase is in villi tips which are damaged by infection)
62
What is the difference between cows milk protein intolerance and allergy? (2)
Cows milk allergy is IgE mediated (rapid reaction, GI/anaphylactic reactions), intolerance is non-IgE mediated (delayed reaction, GI or systemic symptoms)
63
Constipation is frequently seen in children, and 95% is a functional cause. Define constipation in a child (1) Name 3 organic causes of constipation in a child (3)
Less than 3 motions a week and/or pain on defecation | Hirschprung's, CF, hypothyroidism, spinal cord lesions, anal fissure, Coeliac, cows milk protein allergy, hypercalcaemia
64
Descrive the management of constipation in a child? (3)
Assess for faecal impaction (e.g. overflow soiling, mass in rectum) First line - Movicol paediatric escalating dose (also give dietary advice) Second line - add in stimulant laxative if disimpaction not occurred within 2 weeks Third - if Movicol not tolerated or stools still hard add osmotic laxative such as lactulose
65
Name 3 red flags in a child presenting with constipation. (3)
``` Failure to pass meconium within 48 hours "Ribbon" stools Symptoms from birth Weakness in legs or locomotor delay Abdominal distension Faltering growth (amber flag) ```

Paediatrics (9 decks)

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