Gastroenterology Flashcards

1
Q

What can GOR be caused by in children?

A

Inappropriate relaxation of LOS (functional immaturity) - most resolve by 12m of age but if persistent, can be due to GORD

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2
Q

How would babies with GOR present?

A

Regurgitation/’Vomiting’, difficulty feeding, persistent cough

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3
Q

What Ix would you do in a child with GOR?

A
  • Clinical Dx
  • 24h LOS pH monitoring (primarily >4)
  • OGD
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4
Q

What is the Mx of a child with GOR?

A
  • Referral if complications such as recurrent AP and OM, unexplained seizure-like events, apnoea etc (same day referral if haematemesis, melaena or dysphagia present)
  • Assessment by paediatrician if red flag Sx, no improvement 1y+, faltering growth
  • Reassure (v common, resolves by 12m) but review if projectile regurgitation/new concerns/1y+
  • Must sleep on back
  • If breast fed, do BF assessment then –> consider trial of alginate/Gaviscon for 1-2 weeks –> if not then pharmacological (GORD - gaviscon/alginate, omeprazole, ranitidine, dunno/metaclop)
  • if formula-fed, review feeding Hx –> smaller-more frequent feeds (150-180ml/kg/day) –> trial of thickened formula –> alginate trial–>pharmacological
    PACES: reassure that this is common and gets better with time, due to food pipe not being completely formed/mature and so food comes back wrong way. Mx depends on breast or formula-feeding and safety net: keep an eye for blood/green vomit - seek medical attention
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5
Q

What is the cause of pyloric stenosis in children? Who does this usually affect?

A

Hypertrophy of the circular muscles of the pylorus causing gastric outlet obstruction. Affects babies aged 2-8 weeks, boys&raquo_space; girls (4:1)

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6
Q

How may a child with pyloric stenosis present?

A
  • PROJECTILE vomiting occurring ~30 mins after a feed (non-billious). Increases in frequency and forcefulness over time, ultimately –> projectile
  • Olive mass in the RUQ
  • Visible peristalsis on the abdomen
  • Weight loss and depressed fontanelle, either hungry or too tired to feed
  • Hypochloraemic hypokalaemia metabolic alkalosis (low Cl-, K+, Na+ and H+) –> can progress to dehydrated lactic acidosis
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7
Q

What Ix would you do in a child with potential pyloric stenosis?

A
  • Test feed (observe for gastric peristalsis)

- USS confirmation (target lesion >3mm thickness)

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8
Q

How would you manage a child with pyloric stenosis?

A

Initial:
- IV slow fluid resuscitation to correct ion disturbances if present; 1.5x maintenance rate + 5% dextrose + 0.45% saline

Surgery:
- Laparoscopic Ramstedt pyloromyotomy

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9
Q

What is infant colic and how would you manage it?

A
  • > Common symptom in young babies (40% in 1st few months of life) - babies will have inconsolable crying and drawing up of the hands and feet
  • > Usually resolves by 3-6 months (~peaks at 6w)

Mx:

  • > REASSURE common and stops by 6m
  • > Soothe the infant (gentle motions, optimal winding technique, warm baths, white noise)
  • -> if persistent, consider GOR or cows milk protein allergy (therefore consider 2-week trial of whey hydrosalate formula followed by 2-week trial of anti-reflux treatment)
  • > Parents to look after themselves!! Support from friends/family as exhausting
  • > support groups such as www.cry-sis.org.uk/health visitor
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10
Q

What is the most common cause of acute abdominal pain in childhood? How may they present? How would you Ix and Mx this patient?

A

Appendicitis! (Rare <3y) - [Feacolith more common in pre-school children and perforation]

Sx:
- RLQ pain, vomiting and nausea, fever, tenderness, anorexia

Ix:

  • FBC, CRP, Pregnancy test if indicated
  • Clinical (observation)
  • USS/AXR +/- CTAP

Mx:

  • GAME - group+save, abx IV, MRSA screen and NBM (no eating or drinking)
  • Appendectomy
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11
Q

What is Meckel’s Diverticulum? (Rule of 2s?) Who does it affect?

A

Very common condition affecting those aged 1-2y (most common congenital condition of SB)
Ileal remnant of the vitello-intestinal duct on the anti-mesenteric border containing ectopic gastric mucosa or pancreatic tissue.

Rule of 2s

  • (1 to) 2yo
  • 2% population
  • 2x more common in boys
  • 2 feet from ileocaecal valve
  • 2 inches long
  • 2 different mucosae
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12
Q

How may a child with Meckel’s diverticulum present?

A
  • Painless MASSIVE PR bleeding (dark red blood)
  • may present in addition to intussusception, volvulus or diverticulitis
  • may have billious vomiting, dehydration, intractable constipation
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13
Q

How would you manage (Ix and Mx) a child with Meckel’s Diverticulum?

A

Ix: Technetium scan indicates increased uptake by gastric mucosa in 70%, also AXR or USS (+laprascopy)

Mx:

  • Asymptomatic i.e. incidental finding requires no Tx
  • Symptomatic - if bleeding, excise diverticulum + blood transfusion if haemodynamically unstable, if obstruction then excise + lyse adhesions, if perforated/peritonitis then excise + SB resection with Abx post-operatively (cefotaxime, clindamycin/metronidazole)
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14
Q

What is intussusception and who does it affect? What can cause it/other associations?

A

Invagination of the proximal bowel into the distal component - 95% involves ileum through to the caecum through IC valve.
Affects those aged 3m - 2 years old

Causes: idiopathic, post-gastroenteritis due to enlarged Peyer’s patches, lead points, hypertrophy e.g. in CF. Associated with lymphoma, gastroenteritis, CF and HSP.

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15
Q

What are the signs of intussusception?

A
  • colic pain (draws up into ball)
  • vomiting (may be bile-stained depending on site)
  • abdominal distension (+ sausage shaped mass in RUQ and/or emptiness in RLQ O/E)
  • late sign: redcurrant jelly stool (bloody mucus)
  • shock
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16
Q

How would you manage (Ix + Mx) a patient with intussusception? What is the recurrence rate?

A

Ix:
- 1st line: Abdo USS (target mass) –> 2nd line would be AXR –> barium/gastrograffin enema

Mx: (Emergency)
- Drip and Suck
- Rectal air insufflation/ barium/GG enema + BS ABx :clindamycin and gentamicin or Tazocin
(pt under light GA and barium is trickled in and the area is assessed and treated). 75% success
- If not/perforation, then surgical reduction + BS Abx

5% recurrence rate - if recurrent maybe investigate for lead point e.g. Meckel’s diverticulum/polyps/appendix

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17
Q

What does intestinal malrotation/volvulus describe? Whats the most significant concern [BMJ]?

A

Malrotation describes the range of rotational and fixation disorders occurring from abnormal embryonic development. This can pre-dispose to volvulus if the mesentery is not fixed.

Most significant concerns are where there is midgut volvulus due to a lack of retroperitoneal fixation of the intestine and a narrow midgut base. This causes duodenal obstruction -> billious vomiting

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18
Q

How may someone with malrotation/volvulus present?

A

Malroation can be symptomatic and can present at any age with volvulus
OR
Present in first few days of life with obstruction +/- compromised blood supply

Sx: abdominal pain, billious vomiting, peritonitis, scaphoid abdomen (concave)

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19
Q

How would you manage a patient with potential malrotation/volvulus?

A

Ix:

  • URGENT upper GI contrast study to assess potency [if billious vomiting]
  • USS

Mx:
- Urgent laparotomy (Ladd’s procedure). Untwist and replace bowel in correct position and remove necrotic segments.

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20
Q

How would you Dx IBS in children? How may they present?

A

IBS concerns the altered GI motility and abnormal sensation (with/without psychosocial stress + anxiety effect)
Exclusion Dx - ensure not coeliacs
Sx - bloating, diarrhoea which may have mucus/constipation, abdominal pain which is often better after defecation, tenesmus
- otherwise seems well (no systemic signs)

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21
Q

How may gastroenteritis present and what are the most common causative organisms?

A

~10% yearly prevalence in those <5y

Sx:

  • sudden change to loose stools
  • may have vomiting
  • fever
  • abdominal pain
  • travel Hx?
  • [increased risk if <6mo, >2 vomits in 24h, >5 stools in <24h and cannot tolerate extra fluids) –> dehydration –> shock
  • blood in stool (CHESS organisms)

Causes:

  1. ROTAVIRUS (60%)
  2. Campyloacter j
  3. Shigella/salmonella
  4. E.Coli/Cholera
  5. Protozoa (giardia)
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22
Q

How would you investigate potential gastroenteritis?

A

Ix:

  • clinical examination (dehydration)
  • other: stool sample analysis*, bloods, AXR to rule out other causes [if bloody diarrhoea/risk of shock/unknown Dx)

*can do stool sample analysis (young/viral - stool electron microscopy; older/bacterial - stool culture)

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23
Q

How would you manage a child with acute gastroenteritis?

A

Mx:

  • give rehydration advice + reducing spread (ORS 200ml after each loose stool in >5s, <5y can have 50ml/kg IV fluids over 4h + ORS as maintenance)
  • notify HPU if caused by campylobacter, listeria, e.coli, salmonella or shigella
  • SAFETY NET - diarrhoea usually stops within 5-7d (most by 2w) and vomiting in 1-2d (by 3d)
24
Q

What is Crohn’s disease and how may a patient present?

A

Crohn’s - chronic transmural inflammation of the GI tract from mouth-anus, commonly distal ileum and proximal colon

Sx:

  • abdominal pain
  • diarrhoea (may be bloody, mucus)
  • fever
  • growth failure, weight loss
  • loss of appetite, lethargy
  • peri-anal skin tags, apthous mouth ulcers
  • Extra GI: uveitis, erythema nodusum, arthralgia
25
Q

How would you investigate a patient with suspected Crohn’s?

A

Ix:

  • Bloods (FBC inc WBC, iron, B12/folate, CRP, ESR)
  • Stool culture - faecal calprotectin
  • colonoscopy (+OGD) and biopsy -> cobblestone, non-caseating epthelioid cell granulomas
  • upper GI + small bowel contrast scan
26
Q

How would you manage a patient with Crohn’s?

A

Mx:
MDT based - can include gastroenterologists, dieticians, specialist nurses, endocrinologists (growth), school

Induce remission:

  • nutritional management via polymeric diet (liquid nutritional shakes for 6-8 weeks), may require NG
  • steroids i.e. prednisolone

Maintain:

  • Aminosalicylates (Mesalazine)
  • Immunosuppressive drugs (Azathioprine, Mercaptopurine, Methotrexate - but check sufficient TPMT levels first + receive flu vaccines + NO live vaccines)
  • Biologics (Infliximab, Adilumubab)
  • Surgery if complications - obstruction, abscess, unresponsive to treatment

+ Support (C&C UK) + education of features of a flare up

PACES:
o Explain the diagnosis (inflammation of the digestive system leading to malabsorption and bloody diarrhoea due to abnormal immune system response)
o Explain that it is a life-long condition and there is always a risk of relapse
o Reassure that there are many medications that can be used to settle down the inflammation any time it flares up + they will be seen by a gastroenterologist
o Explain complications (malabsorption and bowel cancer)
o Support: Crohn’s and Colitis UK

27
Q

What is Ulcerative Colitis and how may it present?

A

UC = form of IBD where there is inflammation of the lining of the large bowel/gut, usually occurring distally then moving proximally

presentation:

  • rectal bleeding
  • diarrhoea
  • abdominal pain
  • arthritis, erythema nodusum/pyoderma gangrenosum
  • associated with PSC, toxic megacolon, enteric arthritis
28
Q

How would you investigate a child with suspected UC?

A

Ix:

  • same as Crohn’s (bloods, faecal calprotectin, colonoscopy+biopsy*, contrast scans to exclude Crohn’s)
  • in children, 90% have pancolitis
  • severity graded using PUCAI score (paediatric UC activity index where severe>65 and mild-moderate = 10-64) OR also Truelove+Witts score

*colonsocopy + biopsy –> colitis moving from distal to proximal, histology showing crypt damage, mucosal inflammation/ulceration)

29
Q

How would you manage a child with UC?

A

Mx: (MDT based)
1st line –> topical or if not working after 4w, oral aminosalycilates (often used to maintain remission, can use azathioprine etc if insufficient)

2nd line –> oral corticosteroid e.g. prednisolone or beclomethasone

3rd line –> oral tacrolimus

4th line –> biological agents

5th line/resistant disease –> surgery (colectomy with ileostomy/ileojejunal pouch)

+ Education + Support (C&C UK)

SEVERE fulminating disease:

  • MDT approach
  • IV CS/cyclosporin + assess need for surgery (shock/tachycardia, AXR dilation of bowel, stools>8/day, high CRP, platelets/low albumin)

PACES:
o Explain the diagnosis + it is a condition that tends to have flare-ups every so often and remission periods
o Reassure that there are medications that can help reduce likelihood + treat flare-ups
o Explain the complications (growth issues, bowel cancer)
o Explain that they will be seen by a gastroenterologist
o Support: Crohn’s and Colitis UK

30
Q

Explain how a child with toddlers diarrhoea may present? How would you manage them?

A

chronic and non-specific diarrhoea

  • commonest cause of loose stools in preschool children
  • due to duly in maturation of intestinal motility and so there is a fast transit of contents in gut (NOT malabsorption)

Sx:

  • varying consistency in stools (well-formed to explosive, loose stools)
  • often parents say they can see undigested vegetables e.g. corn, carrots in stool
  • otherwise child is well and thriving

Mx:

  • rule out other causes if uncertain
  • increase fibre + fat in diet (+++ yogurts, whole milk, cheese) to relieve symptoms
  • avoid fruit juice and squash
31
Q

What is childhood constipation and what red flags is it important to exclude?

A

Very common condition where there is infrequent passage of stools –> (1/+ of painful defecation, faecal impaction, overflow incontinence or active defecation avoidance behaviour).

Red flags to exclude:

  • failure to pass meconium in <24h of life, gross abdominal distension (Hirschsprungs)
  • failure of growth (Coeliac, hypothyroid)
  • abnormal lower limb neurology/deformity (lumbosacral pathology) or sacral dimple of spina bifida
  • abnormal appearance/patency/position of anus (abnormality), perianal bruising/fissures (SA or Crohn’s - fistulas etc)
32
Q

How would you manage a child with constipation? What are the different types of laxatives?

A

Ix: rule out other Dx if there is uncertainty (AXR for impaction?)

Mx:

  • advice on behaviour (scheduled toiling after mealtimes, bowel habit diary, reward system)
  • advice on diet and lifestyle (adequate fluids and fibre)
  • medications:
  • –> Impaction [Disimpaction Tx] = 2w of ESCALATING dose of Movicol paediatric plain (osmotic laxative of polythene glycol + electrolyte) +/- other agents if needed*
  • –> No impaction/treated impaction [Maintenance] = Movicol continued until regular habits established (can be for months)
  • if insufficient then may add a stimulant laxative such as Senna, sodium citrate enema, phosphate enema, MEUA
  • emphasise usage of laxatives are safe even in long term

Types:

  • Bulk-forming: fybogel, methylcellulose
  • Osmotic: lactulose, Movicol
  • Stimulant: Bisacodyl, Senna, sodium picosulphate - Stool-softener: arachisoil, docusatesodium

PACES:

  • explain this is simple constipation and v common
  • explain treatment (stop hard and difficult-passing stool) and that it takes time to work (can be used for a long time for maintenance until bowel habits are reestablished)
  • also advise on behaviour (rewards, regular toilet times) and fluids
33
Q

What is Hirschprung’s disease?

A

Hirschprung’s is a congenital condition where there is an absence of ganglion cells in the myenteric (Auerbach’s) and submucosal (Meissner’s) plexus. Begins in the rectum and spreads proximally to variable distances (75% rectosigmoid), ending at a normally innervated dilated colon

34
Q

How may children with Hirschprung’s present? What are some RF for it?

A

Affects v young infants (<1)

  • Failure to pass meconium <24h (abdominal distension, billions vomiting
  • explosive passage of liquid/foul stools
  • may present in first few weeks of life with severe Hirschprung enterocolitis (C.diff)

RF:

  • Downs syndrome
  • MEN2a
35
Q

How would you manage (Ix+Mx) a child with Hirschprungs?

A

Ix:

  • AXR (if obstruction)
  • contrast barium enema (showing dilated distal segrment and narrowed proximal segment)
  • Diagnosis = suction-assisted full thickness rectal biopsy (absence of ganglion cells)

Mx:

  • 1st line = initially bowel irrigation and then surgery (anorectal pull through - colostomy followed by anastomosing the normally innervated bowel (total colonic agangliosis would require an initial ileostomy, no irrigation, with later corrective surgery)
  • ++ Metronidazole if enterocolitis
36
Q

How would a child with an anal fissure present and be managed?

A

Anal fissure - tear around the skin of the anus, usually seen in co-existing constipation

Sx:

  • painful passing of stool
  • bright red blood on wiping

Mx:

  • conservative = ensuring softer stools (increase oral fluid intake, fibre intake), analgesia for pain (topical also like lidocaine/GTN ointment, advise against holding in stool and good anal hygiene
  • if constipated, consider them for treatment of this also
  • SAFETY NET: if not healed within 2w, seek further help
37
Q

How would a child with threadworm present and be managed?

A

Sx:
- anal itching

Mx:

  • single dose of mebendazole (Ovex) for the ENTIRE household - can be repeated in 2w if infection persists
  • advise rigorous hygiene for at least 2w/6w if not on mebendazole (hand washing, cutting nails and no biting of them, showering every morning, daily changing of bedsheets and nightwear)
  • children <6mo not given mebendazole, just hygiene measures
38
Q

What are the types of lactose intolerance and how may a child with LI present + SE’s?

A

LI = Deficiency in lactase which breaks down lactose –> glucose + galactose which means the lactose ferments in the gut, increasing gas causing pain and bloating. Affects up to 75% of the world!

RFs - FHx, ethnicity (» Asian/African/Hispanic than caucasian)

Types:
> Primary (70%) - deficient lactase
> Secondary (30%) - damage to gut, temporary lactase deficiency (post-gastroenteritis, Crohn’s, coeliac, alcoholism)

Sx:

  • wind/gas
  • bloating after lactose ingestion
  • diarrhoea
  • abdominal rumbling/pain
39
Q

How would you investigate and manage LI in children?

A

Ix:
- clinical diagnosis (trial of lactose-free diet for 2w and see if symptoms resolve)
- lactase hydrogen breath test (>20 parts/million - increased due to overgrowth of bacteria
in SI)
- FBC to rule out secondary disease (WCC, anaemia)

note: previously used to do a lactose intolerance test where ingestion of lactose after fasting caused a LACK of a rise in blood sugar

Mx:

  • dietician referral
  • avoid dairy and milk products - (in primary you can experiment with diet and see thresholds by trying high-fat dairy, hard cheeses and milk substitutes; in secondary LI, cut out dairy and give time for gut to heal - may need vita/Ca/digestive enzyme supplements during this time)
40
Q

What is Coeliac’s disease and who does it affect?

What are 2 associations?

A

Autoimmune disease where there is an intolerance to gliadin (a gluten peptide found in gluten, wheat, barley and rye) causing shorter villi and flat mucosa in the proximal small intestine

Can affect any ages - seen in 1% of population (common)

Associated with HLA-DQ2 (95%) and DQ8 (80%)

41
Q

How may a child with Coeliac disease present?

A

Sx:

  • malabsorption (failure to thrive, abdominal distension, bloating, irritability, wasted buttocks)
  • diarrhoea
  • presents 8-24m after introduction of wheat foods
  • pathognominic sign = dermatitis herpetiformis (pruritic papulovesicular elbow/knee rash)
42
Q

How would you manage (Ix+Mx) a child with suspected Coeliac?

A

Ix:

  • serology (most sensitive = IgA anti-tTG, but also can do anti-EMA; if IgA deficient do IgG DGP = deiminated gliadin peptide
  • FBC + blood smear, vitamin D (anaemia, iron and B12/folate deficiency)
  • OGD + jejunal biopsy to confirm diagnosis in older children/adults (shows villous atrophy and crypt hyperplasia, increased intraepithelial lymphocytes)
  • in very young children, no biopsy/histology - just do EMA and HLA testing

Mx:

  • AVOID gluten - wheat, barley, rye FOR LIFE
  • MDT management (gastroenterologist, nurse, GP, dietician, school)
  • Dietician referral
  • annual (6-12m review) checking growth, symptoms, adherence to diet and blood tests if needed
  • support (Coeliac UK)
  • advise importance of keeping to strict gluten free diet otherwise risk of deficiencies (iron, VitD), osteoporosis, EATL, hyposplenism.
43
Q

What is mesenteric adenitis and how does it present?

A

Cause of abdominal pain, mainly in children <15y. Commonly preceded by a recent viral/bacterial infection e.g. UTI

Sx:

  • abdominal pain (central, diffuse but may be RIF)
  • nausea +/- diarrhoea, reduced appetite
  • INFECTIOUS picture (fever, lymphadenopathy, raised WCC)
44
Q

How would you Ix and Mx a patient with potential mesenteric adenitis?

A

Ix:

  • exclude other causes such as appendicitis (bloods, urine)
  • abdominal USS and CT would show enlarged mesenteric lymph nodes

Mx:

  • Self limiting
  • Analgesia + Hydration (symptoms resolve within 2 weeks but usually after a few days)
  • Abx sometimes given
  • Safety net (increased pain, deterioration)
45
Q

What is a hernia and what are the different types seen in children?
What are some types associated with?

A

Protrusion of contents through a weakened/defective membrane.

Types include:

  • Indirect inguinal
  • Umbilical
  • Umbilical granuloma (wet, moist and leaks fluid –> treated with salt)
  • Femoral hernia (difficult to differentiate from IIH; often done during surgery)
  • Gastroschisis - paraumbilical abdominal wall defect without peritoneal covering
  • Omphalocele/Exomphalos - bowel protruding through the body WITH peritoneal covering and within the umbilicus. Associated with chromosomal abnormalities (15% - inc Trisomy 13, 18 and 21 and Turners).
46
Q

How would you manage Gastroschisis and Omphalocele?

A

Gastroschisis - paraumbilical abdominal wall defect without peritoneal covering
–> Mx = immediate surgery and covered with cling film

Omphalocele/Exomphalos - bowel protruding through the body WITH peritoneal covering and within the umbilicus.
–> Mx with staged closure starting immediately + taking 6-12 months in total.

47
Q

What is the aetiology of and how would you manage an:

  • -> Indirect inguinal hernia (also same for femoral hernia)
  • -> Umbilical hernia
A

IIH:
- Pathophysiology = bowel protruding through processes vaginalis, risk of necrosis/infection (RF = male, prematurity, CTD)
- Differentiate from hydrocele (this will transilluminate)
- Sx - enlarged scrotal sac, palpable loops of bowel/fluid/may have pain, may appear on crying/straining
- vomiting/obstruction/poor feeding/red skin may indicate incarceration
- Clinical Dx, see if reproducible and examine supine vs standing (more commonly on RIGHT side due to delayed descent of right testicle)
- Mx = Surgical correction
–> If it is reducible, then manual reduction is preferred, with elective surgery being scheduled
for repair
–> If it is incarcerated, emergency manual reduction of hernia contents is attempted under
sedation (IV morphine), with repair after 48 hours to allow oedema to settle

Umbilical:

  • common in newborns and often resolve by 12m so watch and wait
  • RF = afro-caribbean ethnicity, Down’s, mucopolysaccharide diseases
  • <1y = W&W; >1y and large and symptomatic then surgical repair at 2-3yo, if small and asymptomatic then repair at 4-5yo
  • If hernia incarcerates then urgent surgery within 24h and manual reduction till then
48
Q

What is encopresis and how is it treated?

A

Soiling of underwear with stool in children past the age of toilet training (>4y)

  • Usually due to overflow incontinence secondary to constipation
  • Mx = enquire about psych stressors, treat constipation
49
Q

What is acute liver failure (ALF) and what are the main causes of liver disease/ALF in those i) <2y, ii) >2y and iii) older children?

A

ALF - massive hepatic necrosis with loss of liver function (+/- hepatic encephalopathy). Uncommon but has a high mortality - main causes are paracetamol OD, infection and metabolic disease.

Main causes:

i) <2y:
- Infection (HSV), Metabolic disease

ii) >2y
- Paracetamol OD

iii) Older children
- Viral infection (HIV, HCV)
- Autoimmune (hepatitis, PSC/PBC)
- NAFLD
- Metabolic (Wilsons)
- A1AT
(+ Paracetamol)

50
Q

What are the symptoms of liver disease/ALF?

A

Sx:

  • Jaundice
  • Coagulopathy
  • Encephalopathy (irritable –> confusion/drowsiness)
  • Hypoglycaemia, Electrolyte disturbance
51
Q

How would you investigate a child with suspected liver disease?

A

Ix:

  • Bloods - liver function (INR, PT, alb) and inflammation (AST, ALT +/- ALP) [early BR normal especially in metabolic disease]
  • Abnormal clotting
  • Plasma ammonia raised
  • EEG + CT show acute hepatic encephalopathy + cerebral oedema
52
Q

How would you manage acute liver failure? What are some poor prognosis indicators and complications of ALF?

A

Mx:

  • Refer to national paediatric liver centre
  • Stabilise child:
  • -> Maintain blood glucose >4mmol/L using IV Dextrose
  • –> Prevent sepsis with BS Abx and antifungals
  • –> Prevent haemorrhage with IV VitK and PPIs/H2 antagonists
  • -> Prevent cerebral oedema by fluid restriction and mannitol diuresis
  • Mx depends on cause

Poor prognosis features include rising Br, falling transaminases, shrinking liver, worsening coagulopathy and coma

Complications = hepatic encephalopathy, cirrhosis, portal HTN

53
Q

How would you manage AI hepatitis?

A

note:
PBC = anti-mitochondrial Abs PSC = pANCA, anti-SM Abs

Mx:

  • Prednisolone and Azathioprine
  • PSC = ursodeoxycholic acid which aids bile flow
  • Liver transplant in severe cases
54
Q

How would you manage Wilson’s disease?

A

Mx:

  • Zinc (blocks intestinal copper absorption)
  • Trientine/Pencillamine (increased urinary coper excretion)
  • Pyridoxine (vitamin B6 given to prevent peripheral neuropathy but can take up to 1y to work)
  • Symptomatic treatment for tremor, dystonia, speech impediment; transplant in end-stage cases
55
Q

How would you manage NAFLD?

A

Mx:

  • Weight loss (/bariatric surgery)
  • Statins
  • Ursodeoxycholic acid
  • Treat insulin resistance and diabetes
  • Vitamin E + C
56
Q

How would you manage a child with paracetamol OD?

A

Mx:
- if <1h –> activated charcoal and check paracetamol level at least 4/+ hours after ingestion and then give NAC if indicated
if >1h –> check paracetamol level at least 4/+ hours after ingestion and give NAC if indicated (don’t need to wait for bloods if ingested >150mg/kg)