2019 RC Specialties > GI > Flashcards

GI Flashcards

(148 cards)

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1
Q

Gastroschisis is associated with what anomaly?

A

Intestinal Atresia

1 in 10

BUT other malformations rare (versus omphalocele has many)

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2
Q

List 3 ways to tell gastroschisis from omphalocele:

A
  1. No membranous sac
  2. Periumbilical (R)
  3. No liver eviscerated
  4. Abdo developed well
  5. 1/10= intestinal atresia
    BUT other malformations rare
  6. Need more hydration due to lack of sac
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3
Q

XR bubble in stomach and left of this. None distal. XR sign? Dx? Associated syn? Heart lesion?

A
  • Double bubble
  • Congenital duodenal atresia
  • Trisomy 21
  • AV canal defect

Tx: NG decompression, fluid resus, look of other anomalies, Sx repair

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4
Q

List three things associated with chronic anal fissures in older kids.

A
  • Constipation
  • Crohn’s Dx
  • Chronic Diarrea
  • Prior Rectal Sx
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5
Q

What is the CC triad for Crohn’s Disease?

A

Diarrhea
Wt loss
Abdo Pain

Other: FTT, dermatitis herpteformis

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6
Q

Best test to tell diaphragmatic eventration versus hernia:

  • diaphragm fluoro
  • US
  • exploratory laparotomy
  • MRI
A

US

Diaphragmatic eventration= abnormal elevation= paradoxical motion of hemidisphragm
- DX usually XR but if uncertain US to confirm
Tx: Sx plication of muscle

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7
Q

13 y.o. early morning throat pain. Bad breath in am.

  • Upper GI
  • AUS
  • CXR
  • pH probe
A

pH probe

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8
Q

List three RF for GERD:

A
  • neuro impairment
  • obesity
  • lung dx
  • esophageal atresia
  • prematurity
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9
Q

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
A

Air enema
(less complication than saline)

R/O INTUSSUSCEPTION

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10
Q

T or F: intussusception is most common abdo emergency in kids < 2

A

True

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11
Q

T or F: most common site of intussusception is ileocolic

A

True

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12
Q

Abdo pain+
palpable sausage shaped abdo mass
+ bloody currant jelly stool

A

Intussusception

more common pain, vomit, mass

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13
Q

What test can you order to see intussusception? Sign on image

A

US.

Bull’s eye.

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14
Q

Swallowed nickel. In stomach on XR. Next?

  • observation
  • upper endoscopy and removal
  • cathartics
A

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.

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15
Q

If FB in esophagus:

A

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.

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16
Q

Child swallowed coin and in stomach on XR. What do you do? (1 pt)

A

Observe.

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17
Q

List three indications for FB removal in esophagus:

A
  1. Battery
  2. Magnet
  3. Sharp or pointed
  4. Meat (can wait up to 12 h)
  5. size dimension (won’t get through GI; vary per age)
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18
Q

List tests for hepatic synthetic function:

A

“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

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19
Q

Stool reducing substance NOT (+) in:

  • glucose
  • sucrose
  • fructose
  • lactose
  • galactose
A

Sucrose/starch NOT reducing substances.

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20
Q

Watery diarrhea. Flatulence. Abdo distended. Abdo Pain. (+) Unabsorbed reducing sugar. Dx? Tests?

A

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

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21
Q

List 4 test for celiac Dx?

A
  1. Anti- tissue transglutaminase IgA antibodies (anti-TTG)
  2. IgA level
  3. Anti-endomesial antibody testing (anti-EMA)
  4. IgG anti-deaminated gliadin peptide antibodies
  5. HLA DQ2 and HLA DQ8 (for unclear cases as min. one must be (+))
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22
Q

Kid with CP. Tolerate GT. Got botox in leg. P/E oral secretion and less hypertonic. Next:

  • pH probe
  • swallow study
  • observe
A

Observe.

Botox can have systemic spread past local and cause dysphagia.

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23
Q

Progressive dysphagia with solid. List two most common cause:

A
  1. EoE
  2. Achalasia
  3. GERD with stricture
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24
Q

List two things on ddx for dysphagia for solid and two for solid + lq.

A

Solid only

  1. EoE
  2. Peptic stricture
  3. Extrinsic (vascular ring, mass)

Solid + Liquid

  1. Neuromuscular (CP, Muscular dystrophy, MS)
  2. Achalasia
  3. Systemic AI (myasthenia gravis, scleroderma)
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25
List 3 AE for botox in CP:
1. Transient fever or pain, bruising 2. Weakness in injected area 3. Spread (i.e. dysphagia, resp distress)
26
Post TEF with resp distress. 3 Dx:
- Tracheomalacia - Re-fistulization - GERD w/ laryngospasm - tracheal stricture - vocal cord paralysis - Cardiac anomaly (VACTERL)
27
Bubbling at mouth and nose with cyanosis with feeds. Can't pass NG. Likely Dx? Most common type?
TEF Most common type: H type
28
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.
29
T or F: 75% of intussusception cases have lead point.
False. 90%= idiopathic.
30
What is intussusception?
"Telescoping" of segment of proximal bowel downstream.
31
T or F: intussusception most common abdo obstruction in kids 6 mo-3 y.o.
True
32
List three lead point example for intussusception:
1. Meckel's 2. Polyp 3. HSP 4. Appendix 5. Non Hodgkin's lymphoma 6. CF intussusception
33
What are 3 contraindications to air/ barium enema in intussusception?
1. Unstable (refractory shock) 2. Perf 3. Peritonitis 4. Prolonged symp
34
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
35
Two indications for fundoplication:
Refractory Esophagitis Chronic pul dx due to aspiration Strictures
36
Four recommendations for 2 mo. BB with GERD that eats 6-8 oz every 6-8 hours.
1. Reduce vol 2. Increase freq 3. Feed upright 4. Break to burp 5. Keep upright 20-30 min. after feed
37
Three RF for pyloric stenosis?
- male - first born - prem - maternal smoking - bottle feed - erythro or azithro within 2 wk of life
38
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
39
What are indications for G-Tube:
- FTT - severe GERD - neurologic impairment versus GJ more for delayed gastric emptying or dysmotility
40
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
41
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.
42
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
43
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)
44
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
45
Teen black male. Intermittent abdo pain + diarrhea. Likely dx? Test?
Secondary lactase deficiency (adult type) Do breath hydrogen test.
46
T or F: if stool reducing substances are negative than disaccharide deficiency ruled out.
False. SUCROSE not stool reducing so not all = (+).
47
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)
48
What are the two indication you treat salmonella gastroenteritis?
1. < 3 mon. (risk of bacteraemia) | 2. Immunocompromised (HIV, CA, sickle cell)
49
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)
50
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 ```
51
Shwachman-Diamond Syndrome BB. Vitamin Level Normal - A - E - B12 - D
B12
52
What is the triad for Shwachman-Diamond Syndrome?
1. Exocrine Pancreatic Insuff 2. Cytopenia (e.g. neutropenia or any line) 3. 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
53
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
54
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
55
List 3 atypical ppt of Celiac dx?
**Iron deficiency anemia **Dermatitis herpetiformis Aphthous Stomatitis Dental enamel hypoplasia **Osteoporosis Pubertal Delay Arthritis Hepatitis
56
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
57
Delayed meconium. Distended above. Calcification on XR: - Hirschsprung - CF - Duodenal Atresia
CF | seen in 10-15% of pt
58
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
59
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
60
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
61
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
62
T or F: meckel's diverticulum account for 1/2 of all lower GI bleeds in kids < 2
True
63
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
64
What is the most common cause of rectal bleeding in kids < 1?
Anal fissure
65
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)
66
What is Meckel's diverticulum
Embryonic out pouching of bowel
67
Painless rectal bleed in 2 y.o. Dx? Test? Tx?
Meckel's Diverticulum Meckel Radionuclide (Tc99m) scan Tx: Sx resection
68
2 y.o. pass bright red blood in stool. Pale. Rectal exam has blood + stool on gloves. Hob 94. Likely Dx?
Meckel's diverticulum
69
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.
70
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
71
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
72
List 2 surgical complications of HSP:
1. Intussusception | 2. Intestinal Perf
73
What is the HSP triad:
HSP= small vessel vasculitis. Common kindergarten. Follows URTI (usually GAS). 1. Palpable purpura (leg or buttock) 2. Abdo pain (+/- N/V, bloody stool) 3. Arthritis and Arthralgia Risk: GI bleed, bowel wall edema cause intussusception, or ischemia w/ perf, Hematuria, Proteinuria
74
Most common cause of rectal prolapse?
Most: idiopathic R/O CF, sacral nerve root lesion
75
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
76
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
77
6 y.o. w/ severe abdo pain + vomit. Tense abdo. 3 things on management:
1. NPO 2. NG to decompress gut/stomach 3. IVF 4. Analgesia 5. Sx consult
78
List two periodic syndrome associated with migraine develop as adult:
1. Cyclical vomiting 2. Abdominal migraine 3. Benign Paroxysmal vertigo
79
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 ```
80
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
81
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
82
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
83
Most specific test for pancreatitis: - amylase - lipase - AUS - abdo CT - AXR
Lipase
84
Pancreatitis Etiologies:
GOTCHA IBD and CF - Gallstones, biliary sludge - Other: Meds (steroids, VPA, Imran), Idiopathic - Trauma - Calcium High - HSP, Hyperlipid - IBD - CF
85
Peptic ulcer. Treatment? - amox + clarithro + PPI - clinda + clarithro - flagyl + others
Amox + Clarithro + PPI
86
What are two situations where air enema is unlikely to work for intussusception?
1. Multiple recurrences 2. Known lead point 4. Ileoileal intussusception
87
Patients w/ IBD have wt loss due to: - inadequate nutrient intake - ++ metabolic demands - malabsorption - drug-nutrient interaction
Inadequate nutrient intake.
88
List two skin complications of IBD:
- erythema nodosum (more CD) - pyoderma gangrenosum (more UC) - psoriasis - perianal skin tags - epidermolysis bullosa
89
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
90
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
91
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
92
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
93
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)
94
List 2 life threatening complications of UC:
- Htypercoaguloable state resulting in stroke or PE - Toxic megacolon - Perforation - Primary sclerosing cholangitis - Adenocarcinoma
95
Which is true of encopresis: - am usually - usually preschool - colon dilated - soft softeners cure the problem
Colon Dilated
96
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
97
Red flags for encopresis:
- delayed passage of meconium - FTT - bloody stools - severe abdo distension - perianal fistula - absent anal wink - sacral dimple
98
T or F: true constipation as neonate likely due to: Hirschsprung disease, intestinal pseudo obstruction, hypothyroidism.
True
99
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
100
T or F: low albumin results in false (-) for sweat chloride.
True.
101
Girl with rectal prolapse x 2 reduced easily in ER. What 1 test to do?
Sweat chloride.
102
3 mo. BB with constipation and FTT. what test to do: - TSH - Anal manometry - Sweat Chloride
Anal Manometry Even better= Barium enema or BX
103
Most useful intervention in encopresis? - pharm - behav - pharm + behav - biofeedback
Pharm + Behavioural Steps: 1. Relief impaction 2. Soften stool 3. Behav (regular postprandial toilet sitting, no punishment, avoid power struggle) 4. Balanced diet + activity 5. Psychiatric/Behav Therapy
104
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.
105
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 ```
106
what % of conjugated bill is considered too high?
20
107
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
108
The earliest and most common ppt of alpha-1 antitrypsin in children?
Persistent Jaundice as newborn
109
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)
110
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
111
What does the liver bx in Alagille show?
Paucity of bile duct "Disappearing bile ducts"
112
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
113
3 reasons for liver failure encephalopathy?
- elevated serum ammonia - glutamine accumulation in astrocyte causing cerebral edema - increased GABA receptor activity causing hepatic encephalopathy
114
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
115
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
116
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
117
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
118
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.
119
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
120
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. ```
121
Which is NOT associated with increased risk of Celiac Dx? - T21 - Turner - Russell-Silver - William's - Type 1 DM
Russell-Silver
122
What has gluten:
Whey Rye Barley This turns into gliadin which binds with our endogenous made TTG that activate antigen presenting cel.
123
What is the gold standard to Dx Celiac
Bx Bx= gold standard - villus atrophy - crypt hyperplasia - increased intraepithelial lymphocytes
124
Which IBD has transmural inflammation
Crohn's Versus UC= superficial submucosa
125
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
126
List organic causes of constipation:
- HIgh Ca - LO K - LO Thyroid - Celiac Dx - Lead poisoning - Mercury poisoning - UC - Medications - CNS (CP, Neurotube dx, Hirschsprung)
127
Most common cause of diarrhea in 3 y.o. - lactose into - IBD - IBS - Toddler's diarrhea - parasitic infection
Toddler's diarrhea = fructose intolerance
128
List symptoms of reflux in older child:
- Halitosis - Reswallowing - Retrosternal chest pain - Nocturnal cough
129
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
130
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
131
Which is Gilbert's Syndrome?
AD | Unconjugated
132
Most common indication for liver transplant in kids:
Biliary Atresia
133
Top three causes of fulminant hepatic failure in kids?
Infection: hepatitis Toxin: acetaminophen IEM: Neonatal hemochromatosis, Wilson's, Tyrosinemia type 1
134
List liver failure complications:
- encephalopathy - cereal edema - coag. - bone marrow failure - low BG - lyte imbalance - renal dysfunction - ascites - pancreatitis
135
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
136
If low Factor 8 only. Is it liver failure?
No- made in blood vessels. * low only= DIC * 8 normal but 5+7 low= liver
137
Can we treat hepatitis B?
Yes. If > 2, BW abnormal. = IFN-alpha OR = Lamivudine
138
What is the risk of hepatitis B?
Hepatocellular CA risk up (30X) Cirrhosis (25%)
139
How is Hep C dx?
Hep C Antibody | Confirm w/ PCR
140
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
141
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.
142
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
143
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.
144
Post Kasai Nutrition:
ADEK | Progestrimil (MCT oil) or BF with MCT
145
Way to remember Alagille?
Intrahepatic biliary atresia! ``` Triangle face Smooth broad forehead posterior embryotoxon long nose pancreatic insuff butterfly vertebrae ```
146
Triad of Eosinophilia Esophagitis?
GERD Hx of atopy FHx of EoE or atopy
147
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.
148
NG now dumping. What's up?
- feed given too fast - formula too hyperosmolar - tube displaced (put Jejunum)
2019 RC Specialties
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