GI Flashcards
Gastroschisis is associated with what anomaly?
Intestinal Atresia
1 in 10
BUT other malformations rare (versus omphalocele has many)
List 3 ways to tell gastroschisis from omphalocele:
- No membranous sac
- Periumbilical (R)
- No liver eviscerated
- Abdo developed well
- 1/10= intestinal atresia
BUT other malformations rare - Need more hydration due to lack of sac
XR bubble in stomach and left of this. None distal. XR sign? Dx? Associated syn? Heart lesion?
- Double bubble
- Congenital duodenal atresia
- Trisomy 21
- AV canal defect
Tx: NG decompression, fluid resus, look of other anomalies, Sx repair
List three things associated with chronic anal fissures in older kids.
- Constipation
- Crohn’s Dx
- Chronic Diarrea
- Prior Rectal Sx
What is the CC triad for Crohn’s Disease?
Diarrhea
Wt loss
Abdo Pain
Other: FTT, dermatitis herpteformis
Best test to tell diaphragmatic eventration versus hernia:
- diaphragm fluoro
- US
- exploratory laparotomy
- MRI
US
Diaphragmatic eventration= abnormal elevation= paradoxical motion of hemidisphragm
- DX usually XR but if uncertain US to confirm
Tx: Sx plication of muscle
13 y.o. early morning throat pain. Bad breath in am.
- Upper GI
- AUS
- CXR
- pH probe
pH probe
List three RF for GERD:
- neuro impairment
- obesity
- lung dx
- esophageal atresia
- prematurity
Admitted for viral gastro. Intermittent scream and vomit in 8 month old. Pale, lethargic. Which is most helpful ind x:
- AXR
- serum lactate
- air enema
Air enema
(less complication than saline)
R/O INTUSSUSCEPTION
T or F: intussusception is most common abdo emergency in kids < 2
True
T or F: most common site of intussusception is ileocolic
True
Abdo pain+
palpable sausage shaped abdo mass
+ bloody currant jelly stool
Intussusception
more common pain, vomit, mass
What test can you order to see intussusception? Sign on image
US.
Bull’s eye.
Swallowed nickel. In stomach on XR. Next?
- observation
- upper endoscopy and removal
- cathartics
Observation
** once in stomach, 95% ingested without difficulty.
XR monitoring till pass for long or sharp, straight pin.
Watch out for multiple magnets - can cause pressure necrosis and perf
Note: failure to progress out within 3-4 wk= impending perf.
If FB in esophagus:
remove ASAP (battery, sharp, meat can wait 12 hr)
asymptomatic blunt and coin can be watched up to 24h to see if pass into stomach.
Child swallowed coin and in stomach on XR. What do you do? (1 pt)
Observe.
List three indications for FB removal in esophagus:
- Battery
- Magnet
- Sharp or pointed
- Meat (can wait up to 12 h)
- size dimension (won’t get through GI; vary per age)
List tests for hepatic synthetic function:
“Plt down -> INR up -> Alb down -> Bili up -> Glucose down”
Synthetic F’n:
- INR, PTT up (does not improve with Vit K IM)
- albumin low
Metabolic F’n: BG
Storage Capacity F’n:
- low cholesterol, TG, lipoprotein
Excretory F’n:
- Bilirubin
Stool reducing substance NOT (+) in:
- glucose
- sucrose
- fructose
- lactose
- galactose
Sucrose/starch NOT reducing substances.
Watery diarrhea. Flatulence. Abdo distended. Abdo Pain. (+) Unabsorbed reducing sugar. Dx? Tests?
Carb malabsorption
- Primary (congenital sucrose deficiency, lactase deficiency etc.)
- Secondary (temp lactose intolerance, intractable diarrhea of infancy, toddler’s diarrhea)
Hydrogen Breath Test (correlate to degree of malabsorption)
Stool pH < 5.5
(+) reducing substance
List 4 test for celiac Dx?
- Anti- tissue transglutaminase IgA antibodies (anti-TTG)
- IgA level
- Anti-endomesial antibody testing (anti-EMA)
- IgG anti-deaminated gliadin peptide antibodies
- HLA DQ2 and HLA DQ8 (for unclear cases as min. one must be (+))
Kid with CP. Tolerate GT. Got botox in leg. P/E oral secretion and less hypertonic. Next:
- pH probe
- swallow study
- observe
Observe.
Botox can have systemic spread past local and cause dysphagia.
Progressive dysphagia with solid. List two most common cause:
- EoE
- Achalasia
- GERD with stricture
List two things on ddx for dysphagia for solid and two for solid + lq.
Solid only
- EoE
- Peptic stricture
- Extrinsic (vascular ring, mass)
Solid + Liquid
- Neuromuscular (CP, Muscular dystrophy, MS)
- Achalasia
- Systemic AI (myasthenia gravis, scleroderma)
List 3 AE for botox in CP:
- Transient fever or pain, bruising
- Weakness in injected area
- Spread (i.e. dysphagia, resp distress)
Post TEF with resp distress. 3 Dx:
- Tracheomalacia
- Re-fistulization
- GERD w/ laryngospasm
- tracheal stricture
- vocal cord paralysis
- Cardiac anomaly (VACTERL)
Bubbling at mouth and nose with cyanosis with feeds. Can’t pass NG. Likely Dx? Most common type?
TEF
Most common type: H type
Progressive non bilious vomit. Small palpable olive on RUQ. Likely lab issue:
- met acid
- resp acidosis
- low K
- alkalotic urine
- high Na
Low K
Classic=
Hypo-Chloremia
Hypo-Kalemia
Metabolic Alkalosis
With Later Paradoxical Urinary Acidosis.
T or F: 75% of intussusception cases have lead point.
False.
90%= idiopathic.
What is intussusception?
“Telescoping” of segment of proximal bowel downstream.
T or F: intussusception most common abdo obstruction in kids 6 mo-3 y.o.
True
List three lead point example for intussusception:
- Meckel’s
- Polyp
- HSP
- Appendix
- Non Hodgkin’s lymphoma
- CF intussusception
What are 3 contraindications to air/ barium enema in intussusception?
- Unstable (refractory shock)
- Perf
- Peritonitis
- Prolonged symp
Infant vomit with chronic wheeze. Upper GI show dent in upper esophagus. Two dx?
- Vascular Ring
- Pulmonary Artery Sling
Other: Mediastinal mass, mediastinal lymphadenopathy, esophageal FB
Two indications for fundoplication:
Refractory Esophagitis
Chronic pul dx due to aspiration
Strictures
Four recommendations for 2 mo. BB with GERD that eats 6-8 oz every 6-8 hours.
- Reduce vol
- Increase freq
- Feed upright
- Break to burp
- Keep upright 20-30 min. after feed
Three RF for pyloric stenosis?
- male
- first born
- prem
- maternal smoking
- bottle feed
- erythro or azithro within 2 wk of life
T or F: Perianal dx should always make you think of Crohn’s dx?
True
Can be from constipation but typically then < 1 y.o.
Older pt= chronic fissure= constipation, Crohn’s, chronic diarrhea, prior rectal Sx
Tx: no constipation, sitz bath +/- topical tx
What are indications for G-Tube:
- FTT
- severe GERD
- neurologic impairment
versus GJ more for delayed gastric emptying or dysmotility
Oral rehydration in kid w/ mod dehydration:
- 100cc/kg over 4h
- 50 cc/kg over 4h
- NS bolus 20cc/kg
100 cc/kg over 4h
Remember % x 10
over 4 hour
moderate= up to 10%
If severe= bolus
Post scoliosis Sx. Bilious Emesis. Likely etiology?
Superior mesenteric artery syndrome.
= compress duodenum by artery against aorta
RF: thin, bed rest, abdo Sx, lumbar lordosis
Classic: thin teen with vomiting 1-2 wk after scoliosis Sx. Lengthening of vertebrate stretch of aorta and extrinsic compress duodenum.
Name two test to confirm GERD dx.
Clinical dx!
Options:
- PH monitoring (document acid reflux episodes but doesn’t Dx GERD, best for acid suppression during tx)
- Intraluminal impedance: differentiate between antegarde vs. retrograde material; good for resp symp
Non-Options:
- Upper GI- for vomi and dysphagia; does not tell GERD
- Manometry- good for motility, achalasia; no GERD
- Endoscopy- tell complication like erosive esophagitis
Name 4 complication of severe GERD
** FTT
Esophagus:
- *- esophagitis
- *- barrett esophagus (intestinal metaplasia of distal esophagus)
- *- stricture
- adenocarcionma
Resp:
- *- Asthma (Aspiration, airway hyper responsive)
- *- recurrent pneumonia
- laryngeal edema, granulomas
- chronic cough
** Dental Enamel Erosion
BRUE (brief resolve unexplained event)
Name 5 non pharma tx for GERD:
Life style:
- low vol high cal feed
- thicken feed
- hydrolyzed if CMPA
- low fat meal at din
- avoid acidic food (chocolate, mint, tomato) and carbonated or caffeinated fluid
- upright positioning after eating
- no tobacco, alcohol
- wt loss if obese
- NJ or GJ feed if aspirated
Teen black male. Intermittent abdo pain + diarrhea. Likely dx? Test?
Secondary lactase deficiency (adult type)
Do breath hydrogen test.
T or F: if stool reducing substances are negative than disaccharide deficiency ruled out.
False.
SUCROSE not stool reducing so not all = (+).
Why do you tx Sallmonella bacteremia in 2 month old:
- eliminate carrier state
- decrease duration of symp
- prevent meningitis
- eliminate shedding from GI tract
Prevent meningitis
ABX NOT recommend for uncomplicated gastroenteritis as:
- suppress normal flora
- prolong excretion
- risk creating carrier state
- does NOT shorten dx
BUT give ABX if:
- < 3 mo. = risk of blood infection
- immunocompromised (HIV, CA, sickle cell)
What are the two indication you treat salmonella gastroenteritis?
- < 3 mon. (risk of bacteraemia)
2. Immunocompromised (HIV, CA, sickle cell)
12 y.o. Fever. Hepatomegaly. HR 85 despite temp 39.5. CBC and urine normal. BCX show gram (-) bacilli (rode). Cause?
Typhoid fever.
- Salmonella tyhpi
- relative bradycardia
- fever, HSM, dactylics, rose spot
- complication: hepatic failure, intestinal perf
- IV 2 wk (ceftriax)
13 mo. General puffy + lethargy. Urine (-) for protein or blood. Alb 13. Tx:
- steroids
- IV albumin
- protein hydrolysate formula
- lactose free formula
Protein hydrolysate formula
Low albumin: R/O renal R/O liver R/O leaky gut R/O post-fontan R side heart issue R/O intake
Shwachman-Diamond Syndrome BB. Vitamin Level Normal
- A
- E
- B12
- D
B12
What is the triad for Shwachman-Diamond Syndrome?
- Exocrine Pancreatic Insuff
- Cytopenia (e.g. neutropenia or any line)
- Bone (metaphyseal dysostosis, thoracic dystrophy, short stature)
Most common CC: infancy with poor growth and steatorrhea
Definitive Dx: molecular analysis
Tx: pancreatic enzyme replacement, ADEK vit, risk of CA
2 y.o. with Celiac. most definitive test:
- jejunal bx
- anti-gliadin antibody
- anti tissue transglutaminase antibody
- gluten free diet
Jejunal bx
1st= anti-TTG IgA + IgA levels
= if (+) -> BX
Bx= gold standard
- villus atrophy
- crypt hyperplasia
- increased intraepithelial lymphocytes
List 4 other cases of flat intestinal villi beside Celiac Dx?
- *- autoimmune enteropathy
- tropical sprue
- giardiasis
- HIV enteropathy
- bacterial overgrowth
- *- crohn’s dx
- eosinophilic gastroenteritis
- cow’s milk enteropathy
- graft vs. host dx
- TB
- lymphoma
List 3 atypical ppt of Celiac dx?
**Iron deficiency anemia
**Dermatitis herpetiformis
Aphthous Stomatitis
Dental enamel hypoplasia
**Osteoporosis
Pubertal Delay
Arthritis
Hepatitis
List 2 conditions associated with Celiac Dx?
- *Genetic
- *T21, *Williams, *Turners
DX
- 1DM
- AI thyroiditis
- Sjogren
- Addison
- Primary Sclerosing Cholangitis
- Autoimmune Hep
- Primary Biliary Cirrhosis
Delayed meconium. Distended above. Calcification on XR:
- Hirschsprung
- CF
- Duodenal Atresia
CF
seen in 10-15% of pt
6 y.o. with hx of diarrhea. Albumin 24. Name 4 dx on DDX:
> Gastric inflam
- eosinophilia gastroenteropahy
> Intestinal Inlam
- *- Celiac
- *- Crohn’s Disease
- *- Food protein enteropathy
- Tropical Sprue (S. india, philippines, carribean)
> Mucosal Inflammation
- *- post-infectious diarrhea
- bacterial overgrowth
- giardiasis
> Radiation Enteritis
> **Lymphangiectasia
- Primary: Turner, Noonan
- Secondary: constrictive pericarditis, CHF, Post-Fontan, Abdo TB, lymphoma, sarcoidosis
> Colonic Inflam
- IBD
- NEC
- Congenital dx of glycosylation
13 mon. Diaper rash + diarrhea with new foods.
- RAST + skin testing
- carb intolerance common
- cow’s milk protein
- citrus fruit + tomato cause via immune mediated mech
- if no mucus not immune problem
Carb intolerance common
- loose watery diarrhea w/ onset of new food
- sucrase-isomaltase deficiency- common in Aboriginal. Okay with milk + lactose but sucrose and starch (juice, fruit, starches) develop diarrhea, diaper rash
- stool pH, reducing substance, breath test
What is FPIES versus Proctitis vs. Enteropathy? Is allergy testing positive for these?
NO HELP TO TEST.
Food protein induced enterocolitis syndrome (FPIES)
- whole tract
- 1 wk-3 mo.
- hours after exposure
- resolve by 3
Food protein induce proctitis:
- large bowel
- BF 2-8 wk old
- bloody stool
- okay by 9 mo.
Food protein induced enteropathy
- any; cow’s milk up to 2 y.o.
- small intestine
i. e. Celiac
List 3 reasons for GI bleed in infant, kid, teen:
Infant:
- anal fissure
- swallowed maternal blood
- bacterial enteritis
- milk protein allergy
- intussusception
- NEC
- coagulation dx (hemorrhagic dx of newborn)
Child:
- UGI: stress ulcer, gastritis, mallory weiss tear, esophagitis
- LGI: anal fissure, colonic polyp, colitis, intussusception, Meckel’s diverticulum
- HSP, HUS
- sexual abuse
- coagulopathy
Teen:
- peptic ulcer, gastritis, mallory-weiss
- esophageal varices
- bacterial enteritis
- IBD
- colonic polyp
T or F: meckel’s diverticulum account for 1/2 of all lower GI bleeds in kids < 2
True
1 y.o. 3 soft red stool. Asymptomatic. Same happened at 8 mo. old. Hbg 70. Next:
- Upper scope
- UGI
- Nuclear med scan for ectopic gastric tissue
Nuclear med scan
R/O Meckel’s
What is the most common cause of rectal bleeding in kids < 1?
Anal fissure
What is the Meckel Diverticulum “Rules”
Rule of Two’s
- likely ppt at 2 y.o.
- 2% of pop’n
- 2X M>F
- 2 feet proximal to ileoceccal valve
- 2 inches long
- 2 types mucosa (gastric, pancreatic)
What is Meckel’s diverticulum
Embryonic out pouching of bowel
Painless rectal bleed in 2 y.o. Dx? Test? Tx?
Meckel’s Diverticulum
Meckel Radionuclide (Tc99m) scan
Tx: Sx resection
2 y.o. pass bright red blood in stool. Pale. Rectal exam has blood + stool on gloves. Hob 94. Likely Dx?
Meckel’s diverticulum
2 wk old. 1 wk hx of stool mixed with blood. BF well. Passed stool within first 24h. Most likely dx?
- anal fissure?
- CMPA
- Meckel’s diverticulum
Anal fissure
Most common cause in infant.
Could be proctocolitis, FPIES with vomiting usually, and both not most common.
2 wk old. Tachy. Low BP. Abdomen tense. Next dx approach?
- Gen Sx
- AUS
- IV Abx
- AXR
- Barium small with small bowel through
Likely NEC
= AXR
- pneumotosis intestinalis
- portal venous gas
- pneumoperitoneum
1 mo baby with blood in stool. Cause?
- anal fissure
- cow’s milk
- colitis
- mom’s blood
Anal fissure
Most common
Scary: NEC, Volvulus, Hirschsprung Enterocolitis, Vascular malformation or coagulopathy
List 2 surgical complications of HSP:
- Intussusception
2. Intestinal Perf
What is the HSP triad:
HSP= small vessel vasculitis. Common kindergarten. Follows URTI (usually GAS).
- Palpable purpura (leg or buttock)
- Abdo pain (+/- N/V, bloody stool)
- Arthritis and Arthralgia
Risk: GI bleed, bowel wall edema cause intussusception, or ischemia w/ perf, Hematuria, Proteinuria
Most common cause of rectal prolapse?
Most: idiopathic
R/O CF, sacral nerve root lesion
2 wk old BB. Abdo mass palpable. Hx of umbilical cath. Cause of hepatosplenomegaly?
Portal vein thrombosis.
- associated w/ prolonged, traumatic or septic umbilical vein cath
- associated w/ neonatal dehydration, systemic infection, or IBD in older kids
- block portal vein and back up to spleen, portal HTN
List 3 red flags for abdo pain:
- Fever
- Wt loss (Constitutional)
- bile or bloody emesis
- Jaundice
- HSM
- Pain outside of umbilicus
- Wakes from sleep
- High CRP, anemia
- FHX IBD, Celiac
6 y.o. w/ severe abdo pain + vomit. Tense abdo. 3 things on management:
- NPO
- NG to decompress gut/stomach
- IVF
- Analgesia
- Sx consult
List two periodic syndrome associated with migraine develop as adult:
- Cyclical vomiting
- Abdominal migraine
- Benign Paroxysmal vertigo
What is fitz hugh curtis?
Sexually active Chlamydial and Gonococci ascend Disseminate from fallopian tube through peritoneum to liver= Perihepatitis RUQ Acute Pain \+/- Vomiting Guarding, tender AST normal or mild elevated Biliary dx uncommon unless sickle cell US normal
Irritable 1 month old. Emesis x 1. Distended abdo. mild tachy. mass in R scrotal. Firm. Non reducible. No transillumination. Next:
- US
- Urgent Sx consult
- Nuclear Scan
- Testicular doppler
Urgent Sx consult
Concern: Incarcerated Hernia
6 y.o. severe abdo pain 8/10. Febrile. Tachy 160. Abdo rigid. As call Sx what do you do?
- Cx and Abx
- Bolus NS + Morphine
- Bolus NS + CT
Bolus NS + Morphine for Analgesia
Tx:
- ABC
- Fluid resus
- ABX + Sx
What is primary versus secondary causes of peritonitis?
Primary:
- spontaneous
- seed into cavity
- pneumococci
- Cefotaxime
Secondary
- Rupture appendix, hernia, Meckel’s, midgut volvulus, HUS, IBD, NEC etc.
- polymicrobial
- Amp, Gent, Flagyl
Most specific test for pancreatitis:
- amylase
- lipase
- AUS
- abdo CT
- AXR
Lipase
Pancreatitis Etiologies:
GOTCHA IBD and CF
- Gallstones, biliary sludge
- Other: Meds (steroids, VPA, Imran), Idiopathic
- Trauma
- Calcium High
- HSP, Hyperlipid
- IBD
- CF
Peptic ulcer. Treatment?
- amox + clarithro + PPI
- clinda + clarithro
- flagyl + others
Amox + Clarithro + PPI
What are two situations where air enema is unlikely to work for intussusception?
- Multiple recurrences
- Known lead point
- Ileoileal intussusception
Patients w/ IBD have wt loss due to:
- inadequate nutrient intake
- ++ metabolic demands
- malabsorption
- drug-nutrient interaction
Inadequate nutrient intake.
List two skin complications of IBD:
- erythema nodosum (more CD)
- pyoderma gangrenosum (more UC)
- psoriasis
- perianal skin tags
- epidermolysis bullosa
How do you manage perianal abscess in IBD patient.
Infant w/ no predisposing Dx: self-limited; no Tx (even if fistula)
Older kids/predisposing Dx:
- good perianal hygiene
- antibiotics
- tx underlying dx
- topical tacrolimus may help IBD pt
- Sx last resort
Post total colectomy for UC patient. “J-pouch”. Now 5d bloody stool 8X per day. Stool culture negative. Dx? Tx?
Dx: Pouchitis
CC: bloody stool, abdo pain, +/- low grade fever
Tx: PO metronidazole or ciprofloxacin
Note: probiotics decrease rate of pouchitis
Crohn’s pt on azathioprine (Imuran). 2 wk later abdo pain, vomiting. Two causes:
AE for azathioprine:
- *- N/V, abdo pain
- pancreatitis
- hepatitis
Crohn’s complication: stricture, fistula, abscess
5 y.o. bloody stool. Hyper pigmented lesion on lip + mucosa. Intussusception Dx. Underlying Cause:
- HSP
- Peutz-Jehgers
- Langerhans Histiocytosis
Peutz-Jegher Syn
- inherited colorectal CA syndrome
- mucocutaneous pigmentation + extensive GI hamargtoma polyposis
Name 2 RF for adenocarcinoma in setting of dx UC IBD.
- Pancolitis
- Primary Sclerosing Cholangitis
Note:
- risk increase after 8-10 yr of dx
- proctitis alone has no increased risk
- once > 10 yr dx, screen colonoscopy q 1-2 yr (if you get PSC then annual regardless of UC onset)
List 2 life threatening complications of UC:
- Htypercoaguloable state resulting in stroke or PE
- Toxic megacolon
- Perforation
- Primary sclerosing cholangitis
- Adenocarcinoma
Which is true of encopresis:
- am usually
- usually preschool
- colon dilated
- soft softeners cure the problem
Colon Dilated
6 y.o. 2-3 loose BM a day. Leaks into underwear. No red flags. P/E normal except fecaloma on DRE.
- TSH
- Stool Cx
- Rectal Bx
- Psych consult
- No investigations
No investigations
Tx: PEG3350, balanced diet with fibre (0.5 g/kg/d), regular activity
Red flags for encopresis:
- delayed passage of meconium
- FTT
- bloody stools
- severe abdo distension
- perianal fistula
- absent anal wink
- sacral dimple
T or F: true constipation as neonate likely due to: Hirschsprung disease, intestinal pseudo obstruction, hypothyroidism.
True
3 month with constipation since birth, FTT, no stool in rectal exam. Next:
- rectal bx
- TSH
- sweat chloride
- F/u in 6 mo.
Rectal Bx
R/O hirshsprung
T or F: low albumin results in false (-) for sweat chloride.
True.
Girl with rectal prolapse x 2 reduced easily in ER. What 1 test to do?
Sweat chloride.
3 mo. BB with constipation and FTT. what test to do:
- TSH
- Anal manometry
- Sweat Chloride
Anal Manometry
Even better= Barium enema or BX
Most useful intervention in encopresis?
- pharm
- behav
- pharm + behav
- biofeedback
Pharm + Behavioural
Steps:
- Relief impaction
- Soften stool
- Behav (regular postprandial toilet sitting, no punishment, avoid power struggle)
- Balanced diet + activity
- Psychiatric/Behav Therapy
What is threshold for term baby with no RF for photo at 5 d?
360
If don’t know should do Coombs and Hgb as this would change your risk line and photo.
4 wk old with conjugated hyperbili. Next imaging test:
- US w/ doppler
- MRI
- CT abdo
- HIDA Scan
US w/ Doppler
** Key R/O sepsis, Biliary atresia, galactosemia, A1AT
W/U = AST, ALT, GGT - INR, Alb, Glucose, Ammonia - Urine (R/O UTI), urine reducing substance (galactosemia) - Newborn screen or sweat chloride - AUS - HIDA Scan
what % of conjugated bill is considered too high?
20
List 7 things on your ddx for conjugated hyperbili
Syndrome
> **Alagille
> PFIC (progressive familial intrahepatic cholestasis)
Obstructive > ** Biliary atresia > ** Choledochal cyst > Inspissated Bile Syndrome > Perf of bile duct
Infection
> **TORCH
> **UTI
> Sepsis
Endo
> **Hypothyroidism
> Hypopituitarism
Metabolic > **CF > **Galactosemia > Alpha-1-antitrypsin > Tyrosinemia > IEM
Drugs/Toxin
> **TPN, Meds
Systemic: CHD, Shock
Non-cholestatic: **Dubin-Johnson, Rotor
The earliest and most common ppt of alpha-1 antitrypsin in children?
Persistent Jaundice as newborn
Butterfly vertebrae. Heart murmur. Cholestatic jaundice at 2 wk of life. Eyes shows:
- chorioretinitis
- posterior embryotoxon
- cataract
- glaucoma
Posterior embryotoxon (film or memb around eye)
List 5 features of Alagille Syndrome:
Facies: broad forehead, deep wide set eyes, long straight nose
Eyes: posterior embryotoxon, micro cornea, shallow anterior chamber
Cholestasis- prolonged conjugated bili
Peripheral pul stenosis
Butterfly vertebrae
Interstitial nephro
Pancreatic insuff
Short
What does the liver bx in Alagille show?
Paucity of bile duct
“Disappearing bile ducts”
Brown ring around eye. Kayser Fleischer Rings. Dx? Complication?
Wilson Disease
- cooper accumulate
- Complication: hepatic dx, neuro dx (tremor, parkinsonism)
- N/V, AST/ALT, portal HTN, hemolytic anemia
- high ceruloplasmin
- 24h quantitive urine copper test of choice
- restrict dietary copper
- oral copper chelation tx
- liver transplant
3 reasons for liver failure encephalopathy?
- elevated serum ammonia
- glutamine accumulation in astrocyte causing cerebral edema
- increased GABA receptor activity causing hepatic encephalopathy
What is true re: extra-hepatic portal HTN:
- associated w/ hepatomegaly
- associated w/ splenomegaly
- LFT abnormal
- most common cause splenic vein thrombosis
Associated w/ splenomegaly
List 1 cause of extra hepatic versus intrahepatic causes of portal HTN.
Extra-hepatic:
- portal vein thrombosis
- splenic vein thrombosis
- AV fistula
Intra-hepatic:
- hepatocellular dx
- hepatitis
- cirrhosis
- hepatic fibrosis
T or F: you MUST R/O portal HTN in child w/ asymptomatic splenomegaly without hepatocellular dx?
TRUE
Bleeding may not occur till > 6 y.o.
Dx: US with Doppler (portal vein, esophagus)
Long term complication:
- bleed
- hypersplenism
- biliary obstruction
- growth slow
- DD
- neuropsych
- hepatopulmonary syn
Name 2 features on P/E that suggest Portal HTN
Hepatic etiology signs:
- jaundice
- palmar erythema
- vascular telangiectasia
- ascites
- hepatomegaly
Extra-hepatic etiology signs:
- caput medusa
- splenomegaly
- anorectal varices
13 y.o. mild jaundice + fatigue. HIGH AST, ALT. Bili mild high. Negative a-1-a, hep B, hep C. Dx:
- Autoimm Hep
- Hep C
- Wilson’s
- Sclerosing Cholangitis
- A-1-A
Autoimmune Hepatitis
* link with anti-smooth muscle antibody*
R/O viral, a-1-a, wilsson, IBD, celiac, IBD, PSC.
Tx: steroids
Re: Question
- ALP, GGT up if biliary tree issue.
- Wilson’s rare.
Viral hepatitis. What to monitor serially?
- INR
- GGT
- Albumin
- AUS
- Alk-phos
INR
Hepatocyte= AST, ALT but elevation does not correlate with necrosis.
F’n: low albumin, PTT, INR, low BG, low lipid
Cholestasis= Bili, GGT, alk-phos
H pylori dx. Treatment:
- H2 blocker
- amox, claritho, PPI
- claritho + H2
- amox + PPI
Amox + Clarithro + PPI
CC: asymp. or pain, vomit, Fe deficiency, growth issue Dx: Bx must tx all pt (including asymp.) Treatment: amox, clarithro, PPI - Abx x 2 wk - PPI x 1 mo.
Which is NOT associated with increased risk of Celiac Dx?
- T21
- Turner
- Russell-Silver
- William’s
- Type 1 DM
Russell-Silver
What has gluten:
Whey
Rye
Barley
This turns into gliadin which binds with our endogenous made TTG that activate antigen presenting cel.
What is the gold standard to Dx Celiac
Bx
Bx= gold standard
- villus atrophy
- crypt hyperplasia
- increased intraepithelial lymphocytes
Which IBD has transmural inflammation
Crohn’s
Versus UC= superficial submucosa
NOT on Terminal Ileitis DDX:
- Crohn’s
- Lymphoma
- TB
- Yersinia
- Celiac
Celiac Dx= NOT
DDX:
- crohn’s
- lymphoma
- yersinia
- TB
- chronic granulomatous dx
- servere eosinophilia gastroenteropathy
- lymphonodular hyperplasia
List organic causes of constipation:
- HIgh Ca
- LO K
- LO Thyroid
- Celiac Dx
- Lead poisoning
- Mercury poisoning
- UC
- Medications
- CNS (CP, Neurotube dx, Hirschsprung)
Most common cause of diarrhea in 3 y.o.
- lactose into
- IBD
- IBS
- Toddler’s diarrhea
- parasitic infection
Toddler’s diarrhea
= fructose intolerance
List symptoms of reflux in older child:
- Halitosis
- Reswallowing
- Retrosternal chest pain
- Nocturnal cough
Fun Fact: What are extensively hydrolyzed formula versus a.a. based?
Extensively hydrolyzed = 9/10
- Nutramigen
- Alimentum
- Pregestimil
A.A based= 99/100
- Neonate
- Puramino
What are bugs that cause bloody stool? Which treated?
E coli (no tx)
Shigella (no tx)
Salmonella
- Tx if: <3 month OR immunocompromised
- Amox or TMP-Sx
Campylobacter (consider)
- Abx if bloody stool, fever, severe course, immunosuppressed
- Azithro/erythro x 5d (shorten duration, and prevent relapse)
- Cipro can be used (high resistance)
Yersinia
Stenotrophomonas
Which is Gilbert’s Syndrome?
AD
Unconjugated
Most common indication for liver transplant in kids:
Biliary Atresia
Top three causes of fulminant hepatic failure in kids?
Infection: hepatitis
Toxin: acetaminophen
IEM: Neonatal hemochromatosis, Wilson’s, Tyrosinemia type 1
List liver failure complications:
- encephalopathy
- cereal edema
- coag.
- bone marrow failure
- low BG
- lyte imbalance
- renal dysfunction
- ascites
- pancreatitis
EXTRA: Liver BW tips:
- AST> ALT
- ALP LOWW
- ALP HIII
- ALP Norma but all other high
AST> ALT:
Myopathy, renal, hemolysis, adeno
Alk-P
> Low=Zinc
> HI but other BW Normal= familial hyperphos
> Normal but all rest high= Wilson’s
If low Factor 8 only. Is it liver failure?
No- made in blood vessels.
- low only= DIC
- 8 normal but 5+7 low= liver
Can we treat hepatitis B?
Yes.
If > 2, BW abnormal.
= IFN-alpha
OR
= Lamivudine
What is the risk of hepatitis B?
Hepatocellular CA risk up (30X)
Cirrhosis (25%)
How is Hep C dx?
Hep C Antibody
Confirm w/ PCR
12 y.o. chronic LFT elevation. Hyperechogenic liver. Likely dx?
- AI hepatitis
- NASH
- Wilson’s
- Hep B
- Recurrent viral myositis
Non-alcoholic fatty liver dx
** esp if high BMI
= Do US
= R/O AI hep, AIAT, Wilson’s, Hep C
Initial W/U for IBD
CBC + diff ESR CRP Albumin \+/- US with doppler (terminal ileum, flow, abscess, liver, pancreatitis) Upper endoscopy and colonoscopy MRE
**No small bowel follow through.
Treatment of IBD: UC vs. Crohn’s
** Rescue Steroids anytime!
CD
- rare: 5-ASA (mesalamine)
- Immune modulators: MTX (M), Azathioprine (Imuran)
- Biologics: Infliximab (Remicade)
- Sx complications
UC
- 5-ASA (PO, PR)(mesalamine)
- Immuno modulators: Azathioprine (Imuran)
- Biologic: Infliximab (Remicade)
- Sx
What is gold standard to dx Biliary atresia?
HIDA Scan + Bx
Scan can be negative. BX key to ensure intrahepatic fine (not Alagille)
Need to do Kasai within 1-2 mo.
Post Kasai Nutrition:
ADEK
Progestrimil (MCT oil) or BF with MCT
Way to remember Alagille?
Intrahepatic biliary atresia!
Triangle face Smooth broad forehead posterior embryotoxon long nose pancreatic insuff butterfly vertebrae
Triad of Eosinophilia Esophagitis?
GERD
Hx of atopy
FHx of EoE or atopy
How do you manage EoE?
ALL PPI
Severe= Food impact=
= PPI
+ Inhaled steroid (no aerochamber and turn upside down thens swallow)
+/- food protein elimination diet
Mild-Mod= PPI to see if responsive or not
If no change= Bx and then start same treatment as severe.
NG now dumping. What’s up?
- feed given too fast
- formula too hyperosmolar
- tube displaced (put Jejunum)
