PAEDS GI & LIVER Flashcards

(172 cards)

1
Q

MALABSORPTION
What is malabsorption?

A
  • Disorders affecting digestion or absorption of nutrients
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2
Q

MALABSORPTION
What are some causes of malabsorption?

A
  • Small intestine disease = coeliac
  • Exocrine pancreas dysfunction = CF
  • Cholestatic liver disease, biliary atresia
  • Short bowel syndrome (NEC, bowel removal)
  • Loss of terminal ileum function (resection, Crohn’s, absent bile acid)
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3
Q

HIRSCHSPRUNG’S DISEASE
What is Hirschsprung’s disease?

A
  • Absence of ganglionic cells from myenteric (Auerbach’s) plexus of large bowel resulting in narrow, contracted section of bowel > large bowel obstruction
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4
Q

HIRSCHSPRUNG’S DISEASE
Where is most affected by Hirschsprung’s disease?
What is it associated with

A
  • 75% confined to rectosigmoid
  • Commonly ileum moves into the caecum via the ileocaecal valve
  • M»F, Down’s syndrome
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5
Q

HIRSCHSPRUNG’S DISEASE
What is the clinical presentation of Hirschsprung’s disease?

A

SYMPTOMS
- bilious vomiting
- delay passing meconium (>24-48hrs)
- poor weight gain (failure to thrive)
- constipation

SIGNS
- abdominal distention
- DRE = empty rectal vault or explosive stools on insertion of gloved finger

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6
Q

HIRSCHSPRUNG’S DISEASE
What are some investigations for Hirschsprung’s disease?

A
  • AXR = dilated colon
  • contrast enema = dilated colon followed by non-dilated (transition zone)
  • rectal suction biopsy (GOLD STANDARD) = lack of ganglion cells in submucosa
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7
Q

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

A
  • Hirschsprung-associated enterocolitis (HAEC) = inflammation + obstruction of intestine, sometimes due to C. difficile
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8
Q

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

A
  • 2-4w after birth = fever, abdo distension, diarrhoea (bloody) + signs of sepsis
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9
Q

HIRSCHSPRUNG’S DISEASE
What is the management of Hirschsprung’s disease?

A
  • Bowel irrigation as initial management so meconium can pass
  • Surgical resection of aganglionic section of bowel = anorectal pullthrough (anastomosing innervated bowel>anus)
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10
Q

PYLORIC STENOSIS
What is pyloric stenosis?

A
  • Hypertrophy of the pyloric (circular) muscle causing gastric outlet obstruction
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11
Q

PYLORIC STENOSIS
what are the risk factors?

A
  • neonates (typically presents 2-6 weeks of age)
  • male
  • first-born
  • family history
  • caucasian
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12
Q

PYLORIC STENOSIS
What is the clinical presentation of pyloric stenosis?

A

SYMPTOMS
- projectile, non-bilious vomiting (usually 30 mins after feed)
- poor feeding
- dehydration (reduced wet nappies)
- poor weight gain
- constipation

SIGNS
- visible peristalsis
- olive shaped mass in upper abdomen
- evidence of dehydration (tachycardic, hypotensive, sunken fontanelle, dry mucous membranes, mottled skin)

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13
Q

PYLORIC STENOSIS
What are some investigations for pyloric stenosis?

A
  • Test feed = visible gastric peristalsis
  • Capillary blood gas = Hyponatraemic, hypokalaemic + hypochloraemic metabolic acidosis
  • USS = Dx, visualises thickened pylorus with target sign, and antral nipple sign
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14
Q

PYLORIC STENOSIS
What is the management of pyloric stenosis?

A

1st line
- nil-by-mouth and NG tube insertion (to decompress stomach)
- IV fluids (rehydration + correct electrolyte imbalances)
- ramstedt pyloromyotomy (laparoscopic)

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15
Q

PYLORIC STENOSIS
What is Ramstedt’s pyloromyotomy?
What is the after care?

A
  • Incision into smooth muscle of pylorus to widen canal
  • Can feed 6h after
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16
Q

ABDOMINAL PAIN
What are some causes of acute abdominal pain?

A
  • Surgical = appendicitis, intussusception, Meckel’s, malrotation, mesenteric adenitis
  • Boys = exclude testicular torsion + strangulated inguinal hernia
  • Medical = UTI, DKA, HSP, lower lobe pneumonia
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17
Q

ABDOMINAL PAIN
What is recurrent abdominal pain?

A
  • Recurrent pain sufficient to interrupt normal activities + lasting ≥3m
  • Often functional abnormalities of gut motility or enteral neurones = IBS, abdominal migraine or functional dyspepsia
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18
Q

ABDOMINAL PAIN
What are some causes of recurrent abdominal pain?

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

ABDOMINAL PAIN
What are some red flags in recurrent abdominal pain for organic disease?

A
  • Epigastric pain at night, haematemesis = duodenal ulcer
  • Vomiting = pancreatitis
  • Jaundice = liver disease
  • Dysuria, secondary enuresis = UTI
  • Bilious vomiting + abdo distension = malrotation
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20
Q

ABDOMINAL PAIN
What are some investigations for abdominal pain?

A
  • Guided by clinical features, urine MC&S essential
  • Endoscopy if dyspeptic
  • Colonoscopy if any PR bleeding
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21
Q

ABDOMINAL PAIN
How can abdominal pain be managed?

A
  • Encourage parents to not ask about or focus on pain
  • Distract child with other interests + activities
  • Advice about sleep, regular balanced meals, exercise etc
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22
Q

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

A
  • Bowel telescopes (invaginates) into itself (proximal bowel into distal segment)
  • Commonly ileocaecal valve (ileum>caecum)
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23
Q

INTUSSUSSEPTION
what are the risk factors?

A
  • young children
  • male gender
  • preceding viral illness
  • henoch-schonlein purpura (HSP)
  • meckel’s diverticulum
  • lymphoma
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24
Q

INTUSSUSCEPTION
What are the clinical features of intussusception?

A

SYMPTOMS
- severe colicky abdominal pain
- drawing knees up to chest (child turns pale)
- bilious vomiting
- bloodstained stool (redcurrent jelly)
- irritability

SIGNS
- sausage shaped mass in RUQ
- abdominal distention
- hypovolaemic shock

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25
INTUSSUSCEPTION What are the investigations for intussusception?
- abdominal USS (1st line) = shows 'target sign' - AXR = distended small bowel + no gas distally in large bowel - contrast enema = meniscus sign - FBC + CRP - U&Es capillary/venous blood gas
26
INTUSSUSCEPTION What is the management of intussusception?
1st line - resuscitation - radiological reduction (air insufflation) - IV antibiotics 2nd line - surgery (laparotomy)
27
MECKEL'S DIVERTICULUM What is Meckel's diverticulum?
- Ileal remnant of the vitello-intestinal duct which contains ectopic gastric mucosa or pancreatic tissue
28
MECKEL'S DIVERTICULUM What is the rule of 2s with meckel's diverticulum?
Rule of 2s – - 2% population - 2 feet from ileocaecal valve - 2 inches - 2 types of tissue - 2y/o
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MECKEL'S DIVERTICULUM What is the clinical presentation of Meckel's diverticulum?
- Severe, painless, dark red PR bleeding - May present with intussusception, volvulus or diverticulitis (mimics appendicitis)
30
MECKEL'S DIVERTICULUM What are the investigations for Meckel's diverticulum?
- Technetium scan will demonstrate increased uptake by ectopic gastric mucosa
31
CONSTIPATION What is constipation?
- Infrequent passage of dry, hardened faeces often accompanied by straining or pain, by definition <3 complete stools per week
32
CONSTIPATION What are some features of constipation?
- less than 3 stools per week - hard stools that are difficult to pass - rabbit dropping stools - straining + painful passage of stools - abdominal pain - holding an abnormal posture (retentive posturing) - rectal bleeding - faecal impaction causing overflow soiling - hard stools may be palpable in abdomen - loss of sensation of need to open bowels
33
CONSTIPATION What are some causes of constipation?
- Usually idiopathic - Meds (opiates) - LDs - Hypothyroidism - Hypercalcaemia - Poor diet (dehydration, low fibre) - Occasionally forceful potty training
34
CONSTIPATION What are some red flags in constipation?
- Delayed passage of meconium = Hirschsprung's, CF - Failure to thrive = hypothyroid, coeliac - Abnormal lower limb neurology = lumbosacral pathology - Perianal bruising or multiple fissures = ?abuse
35
CONSTIPATION What investigations might you do in constipation?
- Abdo exam may reveal palpable faecal mass - PR examination only by an expert
36
CONSTIPATION What are some complications of constipation?
- Acquired megacolon - Anal fissures - Soiling + behavioural problems - Child may avoid defecating due to pain > constipation + overflow diarrhoea
37
CONSTIPATION What is the process of constipation and overflow diarrhoea?
- Prolonged faecal status = resorption of fluids = increase in size + consistency - This leads to rectal stretching + reduced sensation > overflow + soiling (very smelly)
38
CONSTIPATION What is the medical management of constipation?
- 1st = MACROGOL (osmotic) laxative like polyethylene glycol + electrolytes (Movicol) - 2nd = stimulant laxative if no effect like Senna, bisocodyl ± osmotic laxative (lactulose) or stool softener (docusate) if hard stools - 3rd = consider enema ± sedation or specialist manual evacuation - Continue for several weeks after regular bowel habit then gradual dose reduction
39
GOR What is gastro-oesophageal reflux (GOR)? What are some risk factors?
- Involuntary passage of gastric contents into the oesophagus due to inappropriate relaxation of the lower oesophageal sphincter, often due to functional immaturity - Preterm delivery, neuro disorders (cerebral palsy)
40
GORD What is the clinical presentation of GORD?
- Recurrent regurgitation or vomiting but normal weight gain - chronic cough - hoarse cry - distress, crying or unsettled after feeding - reluctance to feed - pneumonia - poor weight gain
41
GORD What are the investigations for GORD?
- Usually clinical but if atypical Hx, complications or failed Tx... – 24h oesophageal pH monitoring – Endoscopy + biopsy to identify oesophagitis – Contrast studies like barium meal
42
GORD What are some complications of GORD?
- Failure to thrive from severe vomiting - Oesophagitis = haematemesis, discomfort on feeding or heartburn, Fe anaemia - Aspiration > recurrent pneumonia, cough/wheeze - Sandifer syndrome = dystonic neck posturing (torticollis)
43
GORD What is the management of uncomplicated GORD?
ADVICE - Small + frequent meals, do not over feed - Regular burping to help milk settle - Keep baby upright after feeds MEDICAL - gaviscon mixed with feeds (if formula fed) - thickened milk or formula - PPI (OMEPRAZOLE) SURGERY (very rare) - fundoplication
44
GORD What is the management of more significant GORD?
- Acid suppression = H2 receptor antagonists (ranitidine) or PPI (omeprazole) - Surgical Mx (fundoplication) if complications, unresponsive to intensive medical treatment or oesophageal strictures
45
GASTROENTERITIS What is gastroenteritis?
- Inflammation of the stomach and intestines with diarrhoea, nausea + vomiting
46
GASTROENTERITIS What is the difference in gastroenteritis in developing and developed countries?
- Developing = causes thousands of deaths, mostly bacteria from contaminated food - Developed = mostly viral, infants susceptible to dehydration
47
GASTROENTERITIS What is the clinical presentation of gastroenteritis?
- DIARRHOEA = change in consistency of stools to loose/liquid ± increase in frequency of passing stools (acute if <2w, often lasts 5–7d) - VOMITING (1–3d), abdominal cramps - Bloody diarrhoea associated with bacterial infection
48
GASTROENTERITIS what is the general management?
- barrier nursing + infection control - children need to stay off school until 48hrs after symptoms have resolved - stool microscopy, culture + sensitivities - fluid challenge - oral rehydration solutions - avoid anti-diarrhoeal + anti-emetic medications - antibiotics only given in severe disease or if pt is at high risk of complications
49
GASTROENTERITIS What are 5 bacteria that can cause gastroenteritis?
- Campylobacter jejuni - E. coli - Shigella - Salmonella - Bacillus cereus
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GASTROENTERITIS What is campylobacter jejuni? How is it spread? How does it present?
- #1 bacterial cause worldwide, gram negative curved/spiral bacteria - Raw/poorly cooked poultry, untreated water, unpasteurised milk - Abdominal cramps, bloody diarrhoea, vomiting + fever
51
GASTROENTERITIS What is the management of campylobacter jejuni?
- Abx considered after isolating organism where pts have severe symptoms or other risk factors - Azithromycin or ciprofloxacin
52
GASTROENTERITIS What E. coli strain is important to be aware of in terms of gastroenteritis? How is it spread? How does it present?
- E. coli 0157 as produces the Shiga toxin - Contact with infected faeces, unwashed salads or contaminated water - Abdominal cramps, bloody diarrhoea + vomiting
53
GASTROENTERITIS What is a complication of E. coli 0157?
- Destroys blood cells + can lead to haemolytic uraemic syndrome - Abx increase this risk so avoid
54
GASTROENTERITIS How is Shigella spread? How does it present? Complication?
- Faeces contaminating drinking water, swimming pools + food - Bloody diarrhoea, abdominal cramps + fever - Shiga toxin > HUS
55
GASTROENTERITIS How is Salmonella spread? How does it present?
- Raw eggs, poultry - Watery diarrhoea ± mucus or blood
56
GASTROENTERITIS What is Bacillus Cereus? How does it present?
- Gram +ve rod spread through inadequately cooked food, grows well on food, classically undercooked or reheated rice - Produces toxin (cereulide) > abdominal cramping + vomiting soon after ingestion, reaches intestines + different toxin causes watery diarrhoea, resolves within 24h
57
GASTROENTERITIS What are the main investigations for gastroenteritis?
- Assess for dehydration as main concern - FBC, CRP/ESR, U+Es - ?Stool MC&S (electron microscopy if viral) if blood in stool, immunocompromised, travel Hx, not improved in a week
58
GASTROENTERITIS What are signs of clinical dehydration?
- Sunken eyes - Reduced skin turgor - Lethargic - Tachycardia, tachypnoea - Dry mucous membranes - Appears unwell - Oliguria
59
GASTROENTERITIS What are signs of clinical shock?
- Pale/mottled - Hypotension - Prolonged CRT - Cold - Decreased GCS - Sunken fontanelle - Weak pulses - Anuria
60
GASTROENTERITIS What are some complications of gastroenteritis?
- Isonatraemic + hyponatraemic dehydration - Hypernatraemic dehydration - Post-infective lactose intolerance (remove lactose + slowly reintroduce) - Guillain-Barré - Dehydration #1 cause of death
61
GASTROENTERITIS What is isonatraemic dehydration?
- Water loss + Na+ loss are proportional
62
GASTROENTERITIS What is hypernatraemic dehydration?
- Water loss exceeds Na+ loss + fluid shifts from ICF>ECF (rare)
63
GASTROENTERITIS What are the general measures of managing gastroenteritis?
- Isolation if in hospital with barrier nursing as spreads easily - School isolation until Sx settled for 48h - Continue feeds (not solids) - Encourage PO fluids - Discourage fruit juices + carbonated drinks - Mx at home if can keep fluid down
64
GASTROENTERITIS What is the management of gastroenteritis with clinical dehydration?
- 50ml/kg low osmolarity oral rehydration solution (Dioralyte) in addition to maintenance fluid, - may need NG tube if unable to drink or vomiting
65
TODDLER'S DIARRHOEA What is Toddler's diarrhoea? How common is it?
- Chronic non-specific diarrhoea - Commonest cause of persistent loose stools in pre-school children
66
TODDLER'S DIARRHOEA What is the clinical presentation of Toddler's diarrhoea?
- Stools vary in consistency (well-formed>explosive + loose) - Presence of undigested vegetables common = 'peas + carrots diarrhoea'
67
TODDLER'S DIARRHOEA What is the management of Toddler's diarrhoea?
- Most outgrow by age 5 but may be delay in reaching faecal continence - Ensure adequate fat to slow gut transit + fibre, avoid fresh fruit juice
68
BILIARY ATRESIA What is biliary atresia?
- Congenital condition where section of bile duct either narrowed or absent - Results in cholestasis as bile cannot be transported from liver>bowel so increase in conjugated bilirubin - this results in fibrosis and cirrhosis of the liver
69
BILIARY ATRESIA What is the clinical presentation of biliary atresia?
SYMPTOMS - prolonged jaundice >2 weeks - pale stools - dark urine - irritability SIGNS - hepatomegaly - scleral icterus - failure to thrive - abdominal distention - signs of portal hypertension (if severe)
70
BILIARY ATRESIA What are the investigations for biliary atresia?
- LFTs = high conjugated bilirubin, raised GGT and ALP - abdominal USS = absence or abnormality of gallbladder and bile ducts - intraoperative cholangiogram to consider - liver biopsy - HIDA scan
71
BILIARY ATRESIA What is the management of biliary atresia?
1st line - Kasai portoenterostomy - ursodeoxycholic acid 2nd line - liver transplant
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CHOLEDOCHAL CYST What is a choledochal cyst?
Cystic dilatations of extrahepatic or intrahepatic biliary system It is a congenital anomaly
73
NEONATAL HEPATITIS What is neonatal hepatitis syndrome?
- Prolonged neonatal jaundice + hepatic inflammation
74
NEONATAL HEPATITIS What are some investigations for neonatal hepatitis syndrome?
- Deranged LFTs with raised unconjugated + conjugated bilirubin - Liver biopsy = multinucleated giant cells + Rosette formation
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NEONATAL HEPATITIS What are 4 main causes of neonatal hepatitis?
- Congenital infection - Alpha-1-antitrypsin (A1AT) deficiency - Galactosaemia - Wilson's disease
76
NEONATAL HEPATITIS What is A1AT deficiency?
- Deficiency of protease A1AT which inhibits neutrophil elastase + protects tissues
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NEONATAL HEPATITIS How do you diagnose A1AT deficiency?
- Serum A1AT concentration
78
NEONATAL HEPATITIS What is galactosaemia?
- Deficiency of galactose-1-phosphate uridyltransferase (GALT) involved in galactose metabolism (lactose breaks down into galactose)
79
NEONATAL HEPATITIS What are the complications of galactosaemia?
- Rapidly fatal course with shock, DIC + haemorrhage due to gram -ve sepsis - Liver failure, cataracts + Developmental delay if untreated
80
NEONATAL HEPATITIS What is the management of galactosaemia?
- Stop cow's milk, breastfeeding C/I - Dairy-free diet - IV fluids
81
NEONATAL HEPATITIS What is Wilson's disease?
- Reduced synthesis of caeruloplasmin (normally binds to copper + allows it to be excreted with bile)
82
NEONATAL HEPATITIS How does Wilson's disease present?
Sx of copper accumulation - Eyes (Kayser-Fleischer rings) - Brain (Parkinsonism + psychosis) - Kidneys (vit D resistant rickets) - Liver (jaundice)
83
NEONATAL HEPATITIS What are the investigations for Wilson's disease?
- 24h urine copper assay (high), - serum caeruloplasmin (low)
84
FAILURE TO THRIVE What is failure to thrive?
- Failure to gain adequate weight or achieve adequate growth at a normal rate during infancy or childhood - Descriptive term (aka faltering growth)
85
FAILURE TO THRIVE What are the different categories of causes for failure to thrive?
- Inadequate calorie intake (most common) - Malabsorption - Inadequate retention - Increased calorie requirements
86
FAILURE TO THRIVE What are some causes of inadequate calorie intake?
- Impaired suck/swallow (cleft palate, neuro-motor dysfunction, CP) - Inadequate availability of food (socioeconomic deprivation) - Neglect - Maternal depression
87
FAILURE TO THRIVE What are some causes of malabsorption?
- Cystic fibrosis - Cow's milk protein intolerance - Coeliac disease - IBD - Short gut syndrome
88
FAILURE TO THRIVE What are some causes of inadequate retention?
- Vomiting (GORD, pyloric stenosis), - gastroenteritis
89
FAILURE TO THRIVE What is marasmus?
- Severe protein malnutrition - Weight for height >3 standard deviations below the median - Wasted, wrinkly appearance due to severe protein-energy malnutrition
90
FAILURE TO THRIVE What is kwashiorkor?
- Severe protein malnutrition - Generalised oedema - Sparse + depigmented hair - Skin rash - Angular stomatitis - Distended abdomen - Hepatomegaly + diarrhoea
91
FAILURE TO THRIVE How is failure to thrive defined by height?
- Mild = fall across 2 centile lines on growth chart - Severe = fall across 3 centile lines on growth chart
92
FAILURE TO THRIVE How does NICE define faltering growth in children by weight?
- ≥1 centile spaces if birth weight was <9th centile - ≥2 centile spaces if birth weight was 9th–91st centile - ≥3 centile spaces if birth weight was >91st centile - Current weight is below 2nd centile for age, regardless of birth weight
93
FAILURE TO THRIVE What are some investigations for failure to thrive?
- Serial measurements on growth charts for Dx - Full Hx + examination - Measure height + weight > BMI (if >2y) - Calculate mid-parental height - Food diary - Urine dipstick for UTI + coeliac screen (anti-TTG) for 1st line investigations
94
FAILURE TO THRIVE What would you ask about in a failure to thrive history + examination?
- Pregnancy, birth, developmental + social Hx - Feeding or eating Hx (breast/bottle, times, volume, frequency) - Observe feeding - Mum's physical + mental health - Parent-child interactions
95
FAILURE TO THRIVE What might BMI tell you? Any other investigations to perform?
- <2nd centile = ?undernutrition or small build, <0.4th centile = likely undernutrition > assessment + intervention - Specific underlying cause if suspected (CRP/ESR, U+Es, LFTs, TFTs)
96
FAILURE TO THRIVE What is the MDT management approach for failure to thrive?
- Health visitor (parental support if inorganic) - Dietician may suggest nutritional supplement drinks or add energy dense foods, encourage regular structured mealtimes + Snacks - Community paediatrician - SALT if impaired suck or swallow
97
FAILURE TO THRIVE When would hospital admission be required?
- Severe failure to thrive + require active refeeding - Can use this time to observe + improve method of feeding if needed
98
FAILURE TO THRIVE What is the last line consideration in failure to thrive?
- Enteral tube feeding - Must have clear goals + defined end point - Only used if serious concerns about weight gain + other interventions tried
99
CMPA What is cow's milk protein allergy (CMPA)?
- Affects children <3y where there's hypersensitivity to protein in cow's milk, - most outgrow by age 3, - IgE and non-IgE types
100
CMPA How does cows milk protein allergy (CMPA) differ from lactose intolerance?
- Lactose is a sugar - Explosive watery stools, abdo distension, flatulence + audible bowel sounds
101
CMPA What is the clinical presentation of cows milk protein allergy (CMPA)?
- Apparent when weaned from breast > formula milk or food with milk - GI = bloating + wind, abdo pain, D+V, failure to thrive - Allergic = urticaria, cough, wheeze, sneezing, itching - Anaphylaxis + angioedema is rare
102
CMPA How does cows milk protein allergy (CMPA) differ to cow's milk intolerance?
- cows milk intolerance is not an allergic reaction (mild-mod delayed reactions) so does not involve immune system - Same GI Sx but not allergic features - Infants may be able to tolerate some cow's milk just with Sx, same Mx
103
CMPA What are the investigations for cows milk protein allergy (CMPA)?
- IgE mediated = skin prick tests + RAST for cow's milk protein - Gold standard if doubt = elimination diet under dietician supervision
104
CMPA What is the management for cows milk protein allergy (CMPA)?
FORMULA FED - 1st line = extensive hydrolysed formula milk - 2nd line = amino acid-based formula BREASTFED - continue breastfeeding - eliminate cows milk protein from maternal diet. (consider prescribing calcium supplements to mother) - use extensively hydrolysed milk when breastfeeding stops, until 12 months of age and at least for 6 months
105
CMPA How do hydrolysed and amino acid-based formulas work?
- Proteins broken down so they no longer trigger an immune response
106
CMPA How should allergic attacks be managed in cows milk protein allergy (CMPA)?
- Antihistamines or IM adrenaline (EpiPen) if severe reactions
107
FOOD ALLERGIES What food allergies present in children?
- Infants = milk, egg, peanut - Older children = peanut, fish + shellfish
108
KWASHIOKOR what is it?
it is a type of protein-energy malnutrition caused by a severe deficinecy pf protein/amino acids
109
KWASHIOKOR what are the clinical features?
- growth retardation - diarrhoea - anorexia - oedema - defining characteristic - skin/hair depigmentation - abdominal distension with fatty liver
110
KWASHIOKOR what are the investigations?
bloods - FBC, U+E, serum protein, urine dipstick - hypoalbuminaemia - normo/microcytic anaemia - low calcium, magnesium, phosphate and glucose
111
MARASMUS what is it?
a type of protein-energy malnutrition caused by a severe energy (calories) deficiency
112
MARASMUS what are the clinical features?
- height is relatively preserved compared to weight - wasted appearance - muscle atrophy - listless - diarrhoea - constipation
113
MARASMUS what are the investigations?
- bloods - FBC, U+Es - stool MC+S for intestinal ova, cysts and parasites
114
KWASHIOKOR what is the management?
ready-to-use therapeutic food (RUTF) antibiotics milk-based liquid food followed by RUTF if severe (complicated)
115
MARASMUS what is the management?
correct dehydration and electrolyte imbalance treat underlying infection/parasitic infections treat causative disease orally refeed slowly - watch for refeeding syndrome
116
HERNIA what are the most common types of hernia that affect children?
indirect inguinal hernia - caused by an opening in the abdominal wall that is present at birth umbilical hernia
117
HERNIA what are the risk factors for developing a hernia?
- premature, underweight babies - male gender - family history - medical conditions - undescended testes, CF - African descent
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HERNIA what is the clinical presentation?
- lump or swelling near the groin/belly button - pain or tenderness around the groin/lower belly - unexplained crying or fussiness - visible bulge that gets bigger during straining, crying or coughing
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HERNIA what is the treatment for umbilical hernias?
small = 85% will close without surgery large/stays open past 2yrs = surgery
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HERNIA what is the treatment for indirect inguinal hernias?
require surgery to reduce the hernia
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HERNIA what are the complications of hernias?
strangulation/incarceration - there is 30% chance of testicular infarction due to pressure on gonadal vessels
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CHOLEDOCHAL CYST How may it present?
- Cholestatic jaundice - abdominal mass - pain in RUQ - nausea and vomiting - fever
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CHOLEDOCHAL CYST what are the investigations?
can be detected on ultrasound before the child is born after the baby is born, the parent's may notice lump in RUQ, the following tests are then done: - CT scan - cholangiography
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CHOLEDOCHAL CYST What are the complications?
- Cholangitis - small risk of malignancy
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CHOLEDOCHAL CYST What is the management?
Surgical cyst excision
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CHOLEDOCHAL CYST what are the different types?
Type 1 - cyst of extrahepatic bile duct (most common) Type 2 - abnormal pouch/sac opening from duct Type 3 - cyst inside the wall of the duodenum Type 4 - cysts on both intrahepatic and extrahepatic bile ducts
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LIVER FAILURE what are the causes?
- chronic hepatitis - biliary tree disease - toxin induced - A1AT deficiency - autoimmune hepatitis - wilson's disease - CF - budd-chiari syndrome - primary sclerosing cholangitis
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LIVER FAILURE what is the clinical presentation?
- jaundice (not always present) - GI haemorrhage - pruritis - FTT - anaemia - enlarged hard liver - non-tender splenomegaly - hepatic stigmata e.g. spider naevi - peripheral oedema and/or ascites - nutritional disorders - developmental delay - chronic encephalopathy
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LIVER FAILURE what are the investigations?
- BLOODS - FBC = low WCC, low Hb (if GI bleed), low platelets - LFT = normal/low bilirubin, raised AST/ALT - coagulation = increased prothrombin time (PTT) - glucose = normal/low - U+Es, viral serology, IgG, complement, autoimmune antibodies - METABOLIC STUDIES - sweat test = CF - serum/urinary copper = Wilson's disease - ABDOMINAL ULTRASOUND - LIVER BIOPSY - UPPER GI ENDOSCOPY
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LIVER FAILURE what is the management?
- treat the underlying cause + give nutritional support - lower protein, increased energy, higher carb diet - vitamin supplementation (ADEK) - fluid restriction for ascites (alternatively use spironolactone) - liver transplantation
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LIVER FAILURE what is the prognosis?
there is up to 50% mortality without liver transplant
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MALABSORPTION How does it present?
It manifests as: – Abnormal stools (difficult to flush, offensive odour) – Failure to thrive or poor growth – Nutrient deficiencies (Fe anaemia, B12 deficiency)
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HIRSCHSPRUNG'S DISEASE What is a complication of Hirschsprung associated enterocolitis (HAEC)?
Toxic megacolon + perforation = life-threatening
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HIRSCHSPRUNG'S DISEASE How is Hirschsprung associated enterocolitis (HAEC) managed?
Urgent Abx, fluid resus + decompression of obstructed bowel
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INTUSSUSCEPTION What is the epidemiology?
- Most common cause of intestinal obstruction in infants 2m–2y, M>F
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MECKEL'S DIVERTICULUM What is the management of Meckel's diverticulum?
Surgical resection, may need transfusion if severe haemorrhage
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CONSTIPATION What is encopresis?
Involuntary soiling it is not considered pathological until 4yrs old
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GORD When can it become problematic?
Problematic = chronic cough, hoarse cry, distress after feeding + reluctance to feed, failure to thrive
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GASTROENTERITIS What is the most common cause?
- Viral rotavirus in paeds, - norovirus in adults
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GASTROENTERITIS What are some risk factors?
- Poor hygiene, - immunocompromised, - poorly cooked foods
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GASTROENTERITIS what is the management for shigella infection?
severe = azithromycin or ciprofloxacin
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GASTROENTERITIS what is the management for shigella infection?
severe = azithromycin or ciprofloxacin
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GASTROENTERITIS What is hyponatraemic dehydration?
- Child with diarrhoea drinks large quantities of water, Na+ loss greater than water so fall in plasma Na+ – Fluid shifts from ECF>ICF + can result in convulsions
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GASTROENTERITIS How is shock managed in gastroenteritis?
Rapid IVI (0.9% NaCl 20ml/kg), repeat if necessary, Abx if septicaemia
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TODDLER'S DIARRHOEA What causes it?
Likely maturational delay in intestinal motility
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BILIARY ATRESIA What genetic mutation is biliary atresia associated with?
Associated with CFC1 gene mutations
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NEONATAL HEPATITIS How does it present?
- Intruterine growth restriction (IUGR), - hepatosplenomegaly at birth, - failure to thrive - dark urine
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NEONATAL HEPATITIS What is the cause of A1AT deficiency?
AR on chromosome 14
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NEONATAL HEPATITIS What is the presentation of A1AT deficiency?
- Prolonged neonatal jaundice (cholestasis), worse on breast feeding, - can have (prolonged) bleeding due to vitamin K deficiency, - COPD
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NEONATAL HEPATITIS What is the management for A1AT deficiency?
- ?Transplantation - Never smoke
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NEONATAL HEPATITIS How does galactosaemia present?
- Poor feeding, - vomiting, - jaundice + hepatomegaly when fed milk
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NEONATAL HEPATITIS What are the genetics for Wilson's disease?
AR on chromosome 13
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FAILURE TO THRIVE What are some causes of increased calorie requirements?
- Chronic illness (CHD, CKD, CF, HIV), - hyperthyroidism, - cancer
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FAILURE TO THRIVE What are some causes of inability to process nutrients properly?
- T1DM, - inborn errors of metabolism
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CMPA What is cow's milk protein allergy (CMPA) associated with?
- More common in formula fed babies - those with personal or FHx of atopy
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FOOD ALLERGIES What are the different types of food allergy?
- IgE mediated = urticaria, angioedema, wheeze - Non-IgE = D+V, failure to thrive, abdo pain
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FOOD ALLERGIES What is a food intolerance?
Non-immunological hypersensitivity reaction to food
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GASTROENTERITIS How does hypernatraemic dehydration present?
Jittery movements, increased muscle tone, hyperreflexia, convulsions, drowsiness/coma
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GASTROENTERITIS How is hypernatraemic dehydration managed?
Slow rehydration over 48h
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NEONATAL HEPATITIS What is the management of Wilson's disease?
Penicillamine for copper chelation
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INFANT COLIC what is it?
infant colic = excessive infant crying with no obvious trigger RULE OF 3s should be suspected if a baby cries more than 3 hours per day, 3 days per week for more than 3 weeks but are otherwise healthy
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INFANT COLIC What is the epidemiology?
It occurs in up to 40% of babies It typically occurs in few few weeks of life and resolves by 4 months
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INFANT COLIC What is the clinical presentation?
SYMPTOMS - excessive crying (>3hrs for >3days for >3weeks) - difficult to sooth - arched back with knees up to their tummy - face and clenched fists - fussiness (more in evening) - flatulence SIGNS - healthy appearance despite distress - tense abdomen - normal growth and development
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INFANT COLIC what is the risk of this condition?
It is very frustrating for parents It may precipitate non-accidental injury in infants already at risk
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INFANT COLIC what is the cause?
Unknown but thought to be gastrointestinal If severe and persistent it may be due to cow's milk protein allergy or GORD
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INFANT COLIC what are the investigations?
no investigations are necessary if baby is feeding well, growing normally and has normal physical examination to consider - growth chart assessment - parental interview - physical examination
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INFANT COLIC What is the management?
- reassurance (not harmful and should resolve at 3-4 months) - if formula fed = trial of hypoallergenic feed - if breastfed = mother to avoid cows milk, caffeine + spicy foods - simethicone - probiotics - alternative therapies (spinal manipulation or cranial osteopathy)
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MESENTERIC ADENTITIS what is it?
self-limiting inflammatory process affecting mesenteric lymph nodes, usually in RLQ it is classically mistaken for appendicitis
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MESENTERIC ADENTITIS what are the causes?
BACTERIA - yersinia enterocolitica - e.coli - strep VIRUSES - EBV OTHER - inflammatory bowel disease - lymphoma
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MESENTERIC ADENTITIS what are the clinical features?
SYMPTOMS - abdominal pain (usually RLQ or RIF) - nausea and vomiting - diarrhoea - anorexia SIGNS - abdominal tenderness (RIF) - rebound tenderness - fever
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MESENTERIC ADENTITIS what are the investigations?
- FBC + CRP - urine dip - abdominal USS = enlarged LN to consider - abdominal CT - stool culture
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MESENTERIC ADENTITIS what is the management?
1st line - supportive care (rest, hydration, analgesia) - antibiotics (only if bacterial cause is confirmed) 2nd line - surgical intervention (rare, if complications arise or other pathologies are suspected)