Paeds - GI + liver Flashcards
Cow’s milk protein allergy (CMPA) pathophysiology
Immune-mediated allergic response to CASEIN and WHEY.
Can be IgE-mediated; non-IgE-mediated, and mixed
One of most common childhood food allergies (7% of formula/mixed-fed infants have)
CMPA Pathophys
IgE-mediated = type-I hypersensitivity reaction
- CD4+ TH2 cells stimulate B cells to produce IgE antibodies against cow’s milk protein which trigger the release of of histamine and other cytokines from mast cells and basophils
Non-IgE-mediated = T cell activation against cow’s milk protein.
CMPA RFx
Personal or family Hx of ATOPY
Exclusive breaastfeeding is possibly protective
Presentation of CMPA
IgE-mediated:
- Acute/rapid onset:
- Pruritis, Erythema, Urticaria, Acute angio-oedema on face
- Oral pruritis + angio-oedema; Nausea/vomiting; Diarrhoea; colicky abdo pain
- Lower and upper resp tract Sx
Non-IgE mediated:
- Non-acute (48hrs - 1 week after ingestion)
- Pruritis, erythema, ECZEMA
- GORD, Loose or frequent stools, Blood and/or mucus in stools, Abdominal pain, Infantile colic, Food refusal or aversion, Constipation, Perianal redness, Pallor and tiredness, Faltering growth
- Lower resp Sx only
Dx of CMPA
Allergy-focussed Hx:
- Personal and family history of atopy
- Diet and feeding history of infant
- Mother’s diet if breastfed
- Any previous management used for symptoms
- Which milk/foods
- Age of onset
- Speed of onset following exposure
- Duration
- Severity and frequency of occurrence
- Setting of reaction
- Reproducibility of symptoms
GI exam; review GROWTH CHART; signs of atopic co-morb
Bloods: RAST (specific IgE antibodies)
- sensitive but non-specific (false +ves)
- fbc + iron study if concerned about anaemia
CMPA DDx
- Food intolerance
- Allergy to other things
- Chronic GI disease (GORD, coeliac, IBD, gastroenteritis, constipation)
- Pancreatic insufficiency (cystic fibrosis)
- UTI
When to refer for RAST test
- Faltering growth with at least of the above symptoms
- One or more acute systemic or severe delayed reactions
- Confirmed IgE-mediated food allergy with asthma
- Persistent parental suspicion of a food allergy despite lack of clear history
- Clinical suspicion of multiple food allergies, especially with concomitant significant eczema
CMPA Mx
Eliminate cow’s milk from diet
If formula fed -> hypoallergenic:
- Extensively hydrolysed formula
- Amino acid formula (more expensive, for kids who keep having Sx from hydrolysed, or v severe Sx)
Soya-based formulas not recommended under 6 months
Why are soya based formulas not recommended in < 6 months
Isoflavones have weak oestrogenic effect, and absorption of minerals and trace elements may be inhibited by phytate found in this milk
Complications of CMPA
- MALABSORPTION or reduced intake
- Rarely anaphylaxis
Kwashiorkor vs Marasmus
- Marasmus is a deficiency of all macronutrients
- Emaciated, no oedema - Kwashiorkor is a deficiency in protein specifically (typically in people who have access to carbs but less protein).
- Develop oedema which counterbalances fat + muscle loss
- Higher risk of death
- Develop oedema which counterbalances fat + muscle loss
Both are life threatening
Biliary atresia
Bile duct narrowed or absent (prevents excretion of conjugated bilirubin)
Biliary atresia Px
Neonates with significant, persistant jaundice (14 days if term; 21 terms if prem)
Bilary atresia Ix
Conjucgated + uncong bilirubin in blood
- high conj = liver processing bilirubin but unable to excrete
Biliary atresia Mx
‘Kasai portoenterostomy’ surgery
- section of small intestine is attached to liver opening
Oft full liver transplant needed to resolve condition
Choledochal cyst
Congenital swelling of bile duct which can cause blockage and lead to significant, prolonged neonatal jaundice (conjugated bilirubin)
Hirschspurng’s disease
Congenital:
- ABSENCE of PARASYMPATHETIC ganglionic cells of the MYENTERIC (Auerbach’s) PLEXUS in large bowel + rectum
Subtypes of Hirschsprung’s
- Short-segment (recto-sigmoid only - most common)
- long-segment (till splenic flexure)
- total colonic agangliogenesis
Most common site is actually at ileocolonic junction
Which gene has strongest association with Hirschsprung’s + other associations
Receptor Tyrosine Kinase (RET) gene
Also associated with:
- Down’s
- Neurofibromatosis
- Waardenburg syndrome (genetic: pale blue eyes, hearing loss, patches of white skin/hair)
- Multiple endocrine neoplasia type 2
+ more common in MALES
Hirschsprung’s pathophys
The neuroblast which would develop into the enteric nervous system, is arrested in its migration from the vagal segment of neural crest
(or it gets there but doesn’t develop
- apoptosis, bad differentiation, failure to proliferate)
Aganglionic segment = TONIC STATE -> FAILURE of PERISTALSIS + bowel movements
- the internal anal sphinter is not relaxed by presance of faeces
-> FUNCTIONAL OBSTRUCTION
-> Proximal bowel DILATION -> distension
What is a complication of Hirschsprung’s
Increased intraluminal pressure -> reduced mural blood flow -> stasis + BACTERIAL PROLIFERATION
-> At risk for HIRCHSPRUNG’S ASSOCIATED ENTEROCOLITIS
- fluids, bowel decompression, broad-spectrum IV Abx
Esp likely in year after surgery
Also rupture
RFx for Hirschsprung’s
- Males
- Chromosomal abnormalities (esp Down’s)
- FHx - tho usually genetically sporadic
Hirschsprung’s Px
Classical triad:
- Failure to pass MECONIUM within 48 hrs of birth
- Abdo DISTENSION
- BILIOUS VOMITING
Also:
- palpable faecal mass in left lower abdo
- sometimes tympanic
Rectal examination -> EMPTY RECTAL VAULT
- oft results in forceful discharge of gas + fecal material
DDx of Hirchsprung’s
- Meconium plug syndrome
- Meconium ileus
- Intestinal atresia
- DUODENAL ATRESIA PARTICULARLY ASSOCIATED WITH DOWNS
- Double bubble sign
- NG decompression + nil by mouth
- DUODENAL ATRESIA PARTICULARLY ASSOCIATED WITH DOWNS
- Intestinal malrotation (midgut volvulus)
- Anorectal malformation (found on exam)
- Constipation (if it’s nothing else)
Hirschsprung’s Ix
- Plain abdo radiograph
- Contrast/barium enema (CI if there’s perforation)
- short transition zone +/- rectal diameter similar to sigmoid colon
Gold: rectal suction biopsy (bedside, high acurracy. Need antibiotic cover + good decompression as can’t washout for 48hrs after.)
-> stained for acetylcholinesterase
- risk of perforation, bleeding + inadequate sample - NICE says try to avoid
When should a rectal suction biopsy be considered?
- Delayed passage of meconium (more than 48 hours after birth in term babies)
- Constipation since first few weeks of life
- Chronic abdominal distension plus vomiting
- Family history of Hirschsprung’s disease
- Faltering growth in addition to any of the previous features
Management of Hirschsprungers
Symptomatic relief:
- IV Abx
- NG tube
- Bowel decompression
Liase with paeds surgeon
Surgery is only definitive Tx (in first few months)
- resecting the aganglionic section of bowel and connecting the unaffected bowel to the dentate line (pull through)
Complications of surgery for Hirschsprunger’s
constipation,
enterocolitis,
perianal abscess,
faecal soiling
adhesions
Difference in appearance between vomit after repeated vomiting and bilious vomiting
Bile only turns green after mixing with stomach acid (hence green = obstruction)
If repeated vomiting -> bile from gall bladder coming up = more golden
Big complication of bowel obstruction
Necrosing bowel
Malrotation
Umbrella term but most common:
- Lack of fixation to retro-peritoneum (-> volvulus + obstruction)
- Narrow midgut mesenteric base
- Can get abnormal attachment of caecum to right UPPER peritoneal cavity (Ladd bands) -> partial obstruction
Malrotation Sx
- Bilious vomiting in neonate/infant < 1 y/o
- Abdo pain
- Distension + tenderness
Present earlier if more proximal ie in duodenum
When upper mesenteric artery gets compressed by Ladd bands -> tend to present at older age with malneutrition
Malformation Dx
- Upper gastrosintestinal CONTRAST series
- CT abdomen (with oral + IV contrast)
- Abdo X-ray
- FBC
Consider:
- USS
- Lower GI contrast
- ABG
Malformation Tx
Urgent or timely surgery depending on if it has gone ischaemic or not
- Open Laprotomy + Ladd band procedure
Pre op:
- NG tube
- Abx for gram -ve (cefotaxime)
- IV fluid resuscitation
Post op: Bowel rest until activity resumes
Malformation complication
Volvulus -> Acute ischaemia -> Necrosis within a few hours
Pathophys of why bicarb increases in excess vomiting
Bicarb secreted from blood into intestine after acid leaves stomach
When acid can’t leave stomach - bicarb not secreted so get build up of bicarb in blood while acid is projectile vomited
Intussusception
Invagination (telescoping) of proximal bowel into diatal bowel (usually ileum into caecum)
Intussusception Epid
- Primarily in 3 months - 2 YRS
Intussusception aet
- can be triggered by recent/recurrent viral infection as bowel becomes hypermobile + enlargement of Payer’s patches
- Lymphoid hyperplasia (acts as lead point for bowel to telescope)
- Meckel’s diverticulum
- Polyps (esp larger ones)
- CF (increased viscosity -> blockage -> lead point)
- Henoch-Schonlein Purpura (inflam)
- Rotavirus vaccination
Can be idiopathic
Intussusception Sx
- Severe, paroxysmal, COLICKY PAIN (child draws up leg to improve)
- Lethargy + decreased activity inbetween
- Refusing feeds
- Vomiting - may be bilious
- REDCURRENT JELLY (almost black) stool
- DISTENSION
- Susage shaped mass in RIF on palpation (not always)
Intussusception Ix
-
Abdo USS - Target sign (concentric echogenic + nonechogenic bands)
- can also show free air or gangrene if present
Intussusception Mx
- Rectal air inflation +/- contrast enema (can sometimes resolve in this way)
- only if child is stable as if there is ischaemia - air inflation can cause perforation - Operative reduction if:
- non-op failed
- Child has PERITONITIS or PERFORATION
- HAEMODYNAMIC INSTABILITY
DDx for RIF pain
GI:
- Appendicitis
- Intussusception
- Inflamed Meckel’s diverticulum
- Diverticulitis (more common in LIF)
- IBD (esp CROHN’S - terminal ileitis)
- Mesenteric adenitis
Gynae:
- Ectopic preg (if gone through puberty)
- Ovarian torsian
- Ovarian cyst (torsion or rupture)
- Pelvic Inflam Disease (lower abdo pain, fever, abnormal vaginal discharge - less common in kids)
Urology:
- Pyelonepritis
- Uretric colic (kidney stones related usually; can also get blood clots in PKD)