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1
Q

What are the diagnostic criteria for childhood functional abdominal pain ?

A

> /= 1 week for >/= 2 months

  1. Episodic or continuous abdominal pain
  2. Insufficient criteria for other FRID’s
  3. No evidence of an inflammatory, anatomic, metabolic, neoplastic process

Becomes CFAP syndrome if:
CFAP + 25% of the time have either daily loss of function OR an additional somatic complaint

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2
Q

What are red flags for abdominal pain?

A
  • Weight loss -Unexplained fevers
  • Pain radiating to the back
  • Bilious emesis
  • Hematemesis
  • Hematochezia/melena
  • Chronic diarrhea -Gastrointestinal blood loss
  • Oral ulcers -Dysphagia
  • Unexplained rashes -Nocturnal symptoms
  • Arthritis -Anemia/pallor
  • Delayed puberty –Deceleration of linear growth velocity
  • Family history of IBD, celiac, PUD
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3
Q

Name 8 conditions in the organic differential diagnosis of constipation?

A 
Obstruction***
Hirschsprung disease
Electrolyte abnormalities (Hypothyroidism, hypercalcemia***, hypokalemia)
Celiac disease***
TSH***
Meds (Opiates, anticholinergics
ADHD medications)
Spinal cord anomalies

More rare:
Lead toxicity
Botulism
Cystic fibrosis
Anal achalasia
Imperforate anus/Anal stenosis
Pelvic mass (sacral teratoma)
Abnormal abdominal musculature (prune belly, gastroschisis, Down
syndrome)
Pseudoobstruction (visceral neuropathies, myopathies,
mesenchymopathies)
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4
Q 
What is the average number of BMs/day for:
0-3 months breastfed
0-3 months formula fed
6-12 months
1-3 years
>3 years
A 
0-3 months breastfed: 2.9 (5-40 BM/week)
0-3 months formula fed: 2.0(5-28 BM/week)
6-12 months: 1.8 (5-18 BM/week)
1-3 years: 1.4 (4-21 BM/week)
>3 years: 1.0 (3-14 BM/week)
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5
Q

Name 5 complications of chronic constipation

A
  1. Pain: Anal or abdominal
  2. Rectal fissure
  3. Encopresis (overflow diarrhea)
  4. Enuresis
  5. Urinary tract infection
  6. Rectal prolapse/solitary ulcer
  7. Stasis syndrome
    (Bacterial overgrowth–>Carbohydrate fermentation–>Maldigestion)
  8. Social exclusion/depression/anxiety
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6
Q

What are the Rome III criteria

for functional constipation?

A

In the absence of organic pathology, 2 of the following must occur

In children >=4 years-need 2 months of symptoms, insufficient criteria for IBS

In children <4 years-need 1 month of symptoms

  1. <=2 BM/week d
  2. o ≥ 1 episode incontinence/wk (after toilet trained)
  3. History of excessive stool retention
  4. History of painful or hard bowel movements
  5. Presence of a large fecal mass in the rectum
  6. History of large-diameter stools that may obstruct the toilet
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7
Q

Name 10 Alarm Signs and
Symptoms That Suggest the Presence of an
Underlying Disease Causing the Constipation?

A 
Constipation starting extremely early in life (<1 mo)
Passage of meconium >48 h
Family history of HD
Ribbon stools
Blood in the stools in the absence of anal fissures
Failure to thrive
Fever
Bilious vomiting
Abnormal thyroid gland
Severe abdominal distension
Perianal fistula
Abnormal position of anus
Absent anal or cremasteric reflex
Decreased lower extremity strength/tone/reflex
Tuft of hair on spine
Sacral dimple
Gluteal cleft deviation
Extreme fear during anal inspection
Anal scars
Weight loss
Urinary retention
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8
Q

What are the components of constipation management?

A

Education

Behavioural modification

Disimpaction

Daily maintenance stool softeners (should continue until after toilet trained to eliminate fear of defecation)

Dietary modification

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9
Q

What are 2 time periods where children are prone to functional constipation?

A

At the time of toilet learning (age 2-4 years)

During the start of school

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10
Q

What is fecal impaction?

A

Presence of a large and hard mass in the abdomen
Dilated vault filled with stool on rectal examination
History of overflow incontinence

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11
Q

Name 3 methods of fecal disimpaction

A

PEG 3350 1-1.5g/kg/day x 3 days

Enema x 6 days

High dose mineral oil

PEG via NG tube until clear effluent

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12
Q

What dose of PEG 3350 do you use for maintenance therapy?

A

Starting dose at 0.4 g/kg/day – 1 g/kg/day

For infants-dose up to 0.8 g/kg/day

Increasing the dose every two days until the child has1-2 soft stools per day

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13
Q

What are behavioural modifications that can be recommended in constipation?

A
  • Routine scheduled toilet sitting for 3 min to 10 min 1-2x a day
  • Ensure that the child has a footstool on which they can support their legs to effectively increase intra-abdominal pressure (valsalva)
  • There should be no punishment for not stooling during the toileting time
  • Praise and reward for stooling and the behaviour of toilet sitting can be offered.
  • Regular physical activity
  • Stool diaries
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14
Q

What are recommendations for dietary modifications in constipation?

A
  • A balanced diet that includes whole grains, fruits and vegetables
  • Carbohydrates (especially sorbitol) found in prune, pear and apple juices
  • Fibre intake of 0.5 g/kg/day (to a maximum of 35 g/day) for all children
  • For infants: reduce cow’s milk intake, increase fluid intake
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15
Q

How long should patients with constipation be treated for?

A

6 months
Until stools soft
Until after toilet training done

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16
Q

What stool softener is contraindicated in infancy?

A

Mineral oil is contraindicated in infants because of uncoordinated swallowing and the risk of aspiration and subsequent pneumonitis.

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17
Q

What are the Rome III criteria for functional dyspepsia?

A
  1. Persistent or recurrent pain or discomfort centered in the upper abdomen
    (above the umbilicus)
  2. Not relieved by defecation or associated with the onset of a change in stool
    frequency or stool form (i.e., not irritable bowel syndrome)
  3. No evidence of an inflammatory, anatomic, metabolic or neoplastic process
    that explains the subject’s symptoms
  • Criteria fulfilled at least once per week for at least 2 months prior to diagnosis
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18
Q

What are the Rome III criteria for infant dyschezia?

A

Must include both of the following in an infant less than 6 months of age

1) . At least 10 minutes of straining and crying before successful passage of soft stools
2) No other health problems

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19
Q

What is the goal of managing constipation?

A

Evacuation of stools without pain

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20
Q

What are the Rome III criteria for infant colic?

A

Must include all of the following in infants from birth to
4 months of age:

  1. Paroxysms of irritability, fussing or crying that starts and stops without
    obvious cause
  2. Episodes lasting 3 or more hours/day and occurring at least3 days/wk for
    at least 1 week
  3. No failure to thrive
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21
Q

What are the Rome III criteria for IBS?

A

Must include both of the following:

  1. Abdominal discomfort** or pain associated with two or more of the following
    at least 25% of the time:
    a. Improvement with defecation
    b. Onset associated with a change in frequency of stool
    c. Onset associated with a change in form (appearance) of stool
  2. No evidence of an inflammatory, anatomic, metabolic, or neoplastic process
    that explains the subject’s symptoms
  • Criteria fulfilled at least once per week for at least 2 months prior to diagnosis

** “Discomfort” means an uncomfortable sensation not described as pain.

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22
Q

What are the Rome III criteria for abdominal migraine?

A

Must include all of the following:

  1. Paroxysmal episodes of intense, acute periumbilical pain that lasts for 1 hour or more
  2. Intervening periods of usual health lasting weeks to months
  3. The pain interferes with normal activities
4. The pain is associated with 2 of the following: 
Anorexia
Nausea
Vomiting
Headache
Photophobia
Pallor
  1. No evidence of an inflammatory, anatomic, metabolic, or neoplastic process considered that explains the subject’s symptoms
    * Criteria fulfilled two or more times in the preceding 12 months
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23
Q

What is the definition of celiac disease?

A

Immune-mediated enteropathy caused by a permanent sensitivity to gluten in genetically susceptible individuals

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24
Q

What conditions have an increased risk of celiac disease?

A

Type I diabetes

Selective IgA deficiency

Down syndrome

Turner syndrome

Williams syndrome

First degree relatives of individuals with celiac disease

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25
What is the most common age of presentation of celiac disease?
6-24 months (after introduction of solids)
26
How does celiac disease present?
``` Chronic or recurrent diarrhea Abdominal distension Anorexia Failure to thrive or weight loss Short stature Abdominal pain Vomiting Constipation Irritability Rarely: Celiac crisis (explosive watery diarrhea, marked ado distension, severe hypoK) ```
27
Name 9 non-GI manifestations of celiac disease
``` Dermatitis herpetiformis Dental enamel erosions Osteopenia/osteoporosis Delayed puberty Short stature Iron-deficiency anemia (resistant to oral Fe) Hepatitis Arthritis Ataxia Epilepsy with occipital calcifications Alopecia areata Erythema nodosum ```
28
How do you diagnose celiac disease?
Screening: anti-TTG IgA (+IgA and IgG) -Best serological test -May be falsely -ve in < 2years old (because can be IgA deficient) -ant-TTG IgG if IgA deficient (less sensitive) Deaminated gliadins - New - May be useful if IgA deficient or < 2 years old Definitive diagnosis: Endoscopy and small bowel biopsy
29
In what age group are anti-TTG antibodies unreliable?
<2 years of age
30
What 4 screening tests would you order in a child with suspected celiac disease?
CBC Albumin Anti-TTG IgA
31
What is the most definitive diagnostic test for celiac disease?
Endoscopic small bowel biopsy Finding=villous atrophy with hyperplasia of the crypts and abnormal surface epithelium
32
Name 4 causes of small intestinal flat villi other than celiac disease
``` Acute infectious enteritis (Rotavirus) Tropical sprue Cow’s milk protein intolerance Eosinophillic gastroenteritis Microvillus inclusion disease Autoimmune enteropathy Immunodeficiencies (including CVID or IPEX syndrome) ```
33
How do you treat celiac disease?
Only treatment for celiac disease is a gluten-free diet (GFD) –Strict, lifelong diet ``` Avoid: Wheat Rye Barley +/ - oats ```
34
What is the definition of UC?
Generalized bowel inflammation confined to the mucosa, starting at the rectum and involving a variable extent of colon proximally
35
What is the definition of CD?
Generalized inflammation of any portion of alimentary tract, from mouth to anus
36
What are the clinical features of CD?
Abdominal pain (72% at diagnosis) Weight loss (58%) Diarrhea (56%) Blood in stool (22%) – late feature; more common with colonic involvement Growth failure (20-30%) Perirectal inflammation w/ fissures/fistulas (7%) Other: N+V Delayed puberty Arthritis Chronic hepatitis
37
What are the clinical features of UC?
``` Bloody diarrhea (In almost all patients, except those with isolated proctitis) ``` Abdominal pain with BM Fever (in fulminant disease) ``` Growth failure (10%) (Almost always due to corticosteroids in UC) ``` Arthritis Sclerosing Cholangitis (3%) Chronic active hepatitis (<1%)
38
What groups are at increased risk of IBD?
``` Ashkenazy Jews Autoimmune disorders (UC especially) 1st degree relatives PSC Turner syndrome Hermansky-Pudlak syndrome Glycogen storage disorder type 1B Inborn errors of leukocyte adhesion ```
39
What type of IBD is most common in children?
65% CD 33% UC 2% IBD-U
40
Which disorders have decreased frequency of IBD?
Disorders of coagulation: Von Willebrand disease Hemophilia
41
What macroscopic features distinguish UC and CD? - Rectum - Distribution - Terminal ileum - Bowel wall - Mucosa - Stricture - Fistula
- Rectum: UC-yes, CD-variable - Distribution: UC-diffuse/continuous inflammation from rectum proximally, CD-patchy - Terminal ileum: UC-not involved, CD-thick, stenosed - Bowel wall: UC-normal, CD-thickened (transmural inflammation) - Mucosa: UC-hemorrhagic, CD-cobblestone/linear - Stricture: UC-rare, CD-common - Fistula: UC-rare, CD-common - Perianal disease: UC-rare, CD, common
42
Name 10 diseases in the differential diagnosis of IBD
Infection Salmonella, Shigella, Campylobacter, E. coli O157:H7, Aeromonas ***Yersinia – great mimicker of Crohn’s C. diff Parasites: Giardia, Entamoeba histolytica Tuberculosis (with isolated T.I. involvement) HUS, HSP Chronic granulomatous disease Ischemic colitis Dietary protein intolerance Vasculitis (e.g. HSP) Behcet’s Disease (CD) Small bowel lymphoma
43
What initial laboratory investigations should you order in IBD?
CBC: Microcytic anemia, can have iron deficiency Thrombocytosis ESR (elevated in 80% with CD, 40% with UC) CRP (sensitive for bowel inflammation) Albumin (low) – protein loss, poor nutrition (LFTs (esp GGT) – look for hepatic involvement) Fecal calprotectin (measures inflammation in gut, helps differentiate from IBS)
44
How do you ANCA and ASCA antibodies help differentiative between UC and CD?
UC: pANCA positive CD: ASCA positive
45
What finding on biopsy distinguishes CD from UC?
Noncaseating granulomas (pathaneumonic for CD), Transmural inflammation Both CD/UC have crypt architecture distortion, cryptitis, crypt abscess
46
Last 2 diagnostic tests that can confirm the diagnosis of Crohn's disease.
Upper and lower endoscopy with biopsies | MRI-enterography or UGI-SBFT
47
Name 3 acute complications of Crohn's disease
``` Perforation Stricture with SBO Abdo abscess Peri-anal abscess/fistula Renal stones Gallstones ```
48
What are the complications of steroids?
``` Growth failure Osteoporosis Cushingoid Hirsutism Acne Bruising Hair loss Mood swings Insomnia Hyperglycemia Diabetes AVN of the hip ```
49
What are the complications of UC?
``` Increased risk of adenocarcinoma Sclerosing cholangitis (PSC) ```
50
List 2 factors increase the risk of adenocarcinoma in UC (past SAQ)
Increased duration of colitis Increased extent of colitis (pancolitis > left sided) Association with primary sclerosing cholangitis
51
What is a prognostic factor in CD?
Ileocolitis (respond more poorly to medical therapy and greater need for surgery)
52
What medications can be used for induction IBD therapy?
``` Steroids 5-ASA (mild) Biologics (remicade) Enteral nutrition Methotrexate ```
53
What medications can be used for maintenance IBD therapy?
``` Not steroids 5-ASA 6MP-Immuran Enteral nutrition Methotrexate 6-MP/Immurane ```
54
What medications can be used for induction CD therapy?
Steroids | EEN
55
What medications can be used for induction CD therapy?
5-ASA | Steroids
56
Name 10 extraintestinal manifestations of IBD
``` Arthritis Ankylosing spondylitis Clubbing Osteoporosis Erythema nodosum Pyoderma gangrenosum Metastatic Crohns disease (skin) Aphthous ulcers Cheilitis*** Uveitis Primary sclerosing cholangitis Cholelithiasis Growth failure Pubertal delay Autoimmune hemolytic anemia Nephrolithiasis Peripheral neuropathy Acute pancreatitis (Crohn's) ```
57
What type of renal stones are classically associated with Crohn's disease?
Oxalate
58
List 10 causes of erythema nudism
``` IBD Mycoplasma TB Group A strep*** Coccioidomycosis EBV Cat Scratch disease Sarcoidosis OCP Celiac Penicillin Leukemia Lymphoma Behcet's disease ```
59
List 4 conditions that are associated with pyoderma gangrenosum?
UC RA Multiple myeloma PAPA syndrome - acronym for pyogenic arthritis, pyoderma gangrenosum and acne (genetic)
60
What are the clinical criteria for Toxic Megacolon?
1) Radiographic evidence (TC>=56mm) 2) Systemic toxicity Fever >101.5 Tachycardia Dehydration Electrolyte disturbance ALC or coma Hypotension or shock (suspect in UC patient with pain all the time)
61
What radiographic finding is suggestive of toxic megacolon?
Transverse colon diameter >=56mm (or>40 mm in <10 years)
62
How do you diagnose lactose intolerance?
Do a breath test, but milk elimination trial best practically
63
How do you diagnose PLE
Stool alpha-1 antitrypsin
64
What is the first investigation you should order for conjugated hyperbilirubinemia in an infant?
AUS
65
List 6 causes of unconjugated hyperbilirubinemia (pre-hepatic) in infants?
Hemolysis - Sepsis - Galactosemia - Hemoglobinopathies - Membranopathies Non-hemolytic processes - Hypothyroidism - Breast feeding jaundice (volume depletion) - Breast milk jaundice
66
List 6 causes of conjugated hyperbilirubinemia and a definitive test for each (past SAQ)
1. Biliary atresia-intra-operative cholangiogram 2. Choledochal cyst-AUS 3. Alagille-genetic testing for JAG1 and NOTCH2 4. TORCH 5. Sepsis-blood culture 6. Galactosemia-GALT activity Comprehensive ddx: 1. Extrahepatic Biliary Obstruction: • Biliary Atresia • Choledochal cyst • Biliary sludge (TPN) ``` 2. Genetic: • Alagille • CF • PFIC • Bile acid transport defects – e.g. Dubin Johnson and Rotor ``` 3. Infections: • TORCH • Sepsis/UTI 4. Metabolic: • Alpha-1-antitrypsin deficiency • IEM → FAOD, tyrosinemia, galactosemia • Bile acid synthesis defects 5. Endo: • Congenital Hypothyroidism • Panhypopit 6. Medication: • TPN • Antibiotics → Ceftriaxone, Fluconazole, Micafungin 7. Systemic Disorders • Congenital heart disease/CHF • Shock
67
After how many weeks of life should a jaundiced infant have a fractionated bilirubin assessment ?
2 weeks
68
What 3 criteria are required for acute liver failure?
INR >1.5 +encephalopathy OR INR>2 even without encephalopathy AFTER attempted correction with ONE dose of IV vitamin K
69
Name 7 causes of pediatric acute liver failure
In order of most common: 1. Indeterminate 2. Drugs and toxins (acetaminophen, AEDs, INH) 3. Metabolic (HFI, galactosemia, tyrosinemia, Wilson's, NAFLD) 4. Immune (GALD, hemochromatosis, autoimmune hepatitis, HLH) 5. Infectious (Adeno, enterovirus, HBV, herpesvirus) 6. Ischemia/shock (CHD, asyphyxia, Budd Chiari) 7. Irradiation damage 8. Infiltrative disease
70
What drugs can cause fulminant liver failure?
``` Acetaminophen AED (VPA, Carbamazepine) Anti-TB drugs (INH) Inhaled anaesthetics Mushrooms ```
71
What investigations would you order in acute liver failure?
-CBC, lytes, BUN, creatinine -ALT, AST, ALP, GGT, CRP Bilirubin (conjugated, unconjugated, delta) -INR/PTT, albumin, glucose, ammonia Causes: - Acetaminophen level, tox screen - Viral serology: Hepatitis A, B, C, E, EBV, CMV, Adeno and Enterovirus - AIH: Immunoglobulins, anti-LKM, anti-SMA, ANA, - Wilson: Serum copper, ceruloplasmin, 24-h urinary Cu - A-1-AT: Serum alpha-1-AT - Ferritin, LDH - Liver imaging - Liver biopsy (correct INR, check Plt, CBC 4-6post)
72
List 4 steps in the initial management of acute liver failure (past SAQ)
For the exam: 1. Transfer to a transplant centre (if not already at one) and treat the underlying cause if known 2. Manage coagulopathy – Vitamin K, and if needed FFP, Cryo, and Platelets 3. NPO on maintenance IV D5/0.9NS with close monitoring of lytes, end-organ function, fluid balance, and urine output 4. Enemas and lactulose Detailed management: - Transfer to a transplant centre - Admit to ICU - Treat the underlying cause (e.g. acetaminophen OD, steroids for AIH) - Intubate if necessary, minimize/avoid sedatives - PPI for GI bleed prophylaxis - Close monitoring of lytes/glucose, urine output, fluid balance with replacements as needed - Coagulopathy →VitK, FFP, Cryoprecipitate, Platelets - Consider plasmapharesis, dialysis - Monitor for infection → 50% of patients experience serious infection, more so with gram + - Protein restriction in diet - Encephalopathy →enemas, lactulose q2-4h, antibiotics (rifaximin or neomycin), flumazenil may temporarily reverse early encephalopathy - Monitor for cerebral edema - Transplant if advanced ALF
73
Name 4 interventions for coagulopathy in acute liver failure
IV PPI Vitamin K FFP +/- Cryo for bleeding/prior to invasive procedure
74
What is the only factor made outside the liver?
8
75
Name 2 ways patients with acute liver failure have impaired immune systems
Impaired neutrophil and Kupffer cells phagocytic function, Decreased complement levels
76
Name 2 signs of portal hypertension on physical exam in child with liver disease
Ascites Caput medusae Splenomegaly Recanalization of umbilical vein
77
What is the significance of HBc Antibody?
Immune response to naturally acquired HBV
78
What is the significance of HBe antigen?
``` Positive = active infection Negative = seroconversion ```
79
What is the significance of HBe antibody?
If present with –ve Hbe Ag, supports seroconversion
80
How do you tell if there is active mutant HepB infection on serology?
HBeAg negative
81
What is the CPS recommendation on duration of breastfeeding?
Exclusive x 6 months | Continued with complementary foods >2 years
82
What is the ratio of whey: casein of breastmilk?
Whey:casein 70:30 (bovine 18:82)
83
What is the benefit of high whey ratio in breastmilk?
Whey fraction promotes gastric emptying, more easily absorbed
84
Preterm milk has more total nitrogen. True of false?
True
85
What vitamins and minerals are low in breastmilk?
Vitamin D and K Calcium and phosphorus (more bioavailable) Iron, zinc, copper decline over time, but adequate to meet needs until 6 months
86
List 10 advantages of breastfeeding
For mom: - Improves weight loss - Delayed onset of menses - Decreased breast and ovarian cancer, heart disease and risk factors - Cheaper For infant: Decreases - Bacterial meningitis - Bacteremia - Gastroenteritis - Respiratory tract infections - OM - UTIs - Negative effects of 2nd hand smoke - SIDS - Obesity later in life - Childhood malignancies including lymphoma and leukemia - Type I and II DM - Allergic disease including atopic dermatitis and asthma - IBD Increases - Neurocognitive testing - Faster gastric emptying - Stimulates intestinal growth, differentiation
87
List all the maternal and infant contraindications to breastfeeding
Infant: -Galactosemia and congenital lactase deficiency ``` Maternal indications: HIV HTLV Untreated active TB HSV on breast Chemotherapy/cytotoxic drugs Radioactive isoptopes/radiation therapy ```
88
What is expected frequency of breastfeeding in first weeks of life?
Feed 8-12x/day, avoid >4 hour break
89
What is the expected number of wet diapers and stools in first week of life?
Day 3: 3-4 wet diapers/day, 1-2 stools (transitional) Day 7: 6 wet diapers/day, yellow stool with each feeding
90
When should vitamin K be given after birth?
Within 6 hours
91
What is the dose of vitamin K that should be given for neonates?
<1500g-0.5 mg IM | >1500g-1.0 mg IM
92
For how long should you supplement with vitamin D in breastfed babies?
Until 6 months
93
Name 3 indications for increase vitamin D supplementation to 800IU per day in breastfed babies
Living north of latitude 55 degrees, October-April Living in community with high prevalence of vitamin D deficiency Children with dark skin
94
Until what age should you avoid fluoride supplementation?
6 months of age
95
When should you supplement fluoride in children >6 months of age?
Don't brush teeth 2x/day High risk of dental caries (family history, geographic area) Living without fluoridated water (<0.3 ppm)
96
How much fluoride should you supplement for children: 6 mos-3 years 3 years-6 years >6 years
6 mos-3 years 0.25 mg/day 3 years-6 years 0.5 mg/day >6 years 1 mg/day
97
Phyoestrogens in soy are contraindicated in which condition?
Congenital hypothyroidism (phytoestrogens can inhibit thyroid peroxidase, potentially lowering free thyroxine concentrations)
98
What is Acrodermatitis enteropathica?
AR disorder caused by inability to absorb sufficient zinc
99
What abnormality on bloodworm can hint at Zn deficiency?
Low ALP
100
When does acrodermatitis enteropathica typically present?
Often after weaning from breast to cow’s milk (because breastmilk increasees zinc bioavailability)
101
Name 10 clinical features of acrodermatitis enteropathica?
``` Chronic diarrhea*** Irritability Rash (perioral, perineal)*** Eye problems (photophobia, conuncivitis) Hair – reddish tint, alopecia*** Stomatitis Glossitis*** Paronychia, nail dystrophy Growth retardation Delayed wound healing Superinfection with Candida Cognitive dysfunciton ```
102
Name 5 tests to establish the diagnosis of acrodermatitis enteropathica
1. Serum zinc 2. Hair, urine, parotid zinc levels 3. ALP (may be low in late disease) 4. Maternal breast milk level (Differentiate between genetic and acquired deficiency) 5. Derm biopsy: parakeratosis, pallor of the upper epidermis
103
For how long do you need to give zinc supplementation for acrodermatitis enteropathica?
Zinc 1 mg/kg/day for life
104
Name 8 physical exam features of kwashiorkor (inadequate protein intake in presence of reasonable caloric intake)
``` 80-90% of IBW Edema*** Sparse hair - dull brown/red*** Pitting edema Hyperpigmented hyperkeratosis or flaky rash Enlarged parotid glands Apathy, disinterest Enlarged/soft liver Abdo distended ```
105
Name 5 physical exam features of marasmus (severe protein energy malnutrition)
``` <70% of IBW Depleted body fat stores Edema usually absent Skin is dry and thin Hair thin, sparse, easily pulled Apathetic and weak Bradycardia/hypothermia ```
106
Spot diagnosis: http://www.medicalook.com/diseases_images/pellagra.jpg
Pellagra, niacin deficiency | http://www.medicalook.com/diseases_images/pellagra.jpg
107
Name 2 micronutrient deficiencies associated with cheilosis and glossitis?
``` Iron deficiency Riboflavin deficiency (vitamin B2) ```
108
Name 3 signs and symptoms of vitamin A deficiency
``` Night blindness Eye dryness Xeropthalmia Bitot spots Corneal ulceration and scarring Anemia Follicular hyperkeratosis Growth retardation ```
109
Spot diagnosis: http://1.bp.blogspot.com/-5gxcnbXX3xM/TVTNfFDhxqI/AAAAAAAACN4/336dEINIxII/s1600/37065.imgcache.jpg
Rachitic rosary (rickets)
110
Spot diagnosis: https://pedclerk.bsd.uchicago.edu/sites/pedclerk.uchicago.edu/files/uploads/wt.png
Metaphyseal cupping (rickets)
111
What deficiency is associated with goat's milk?
Folate
112
What deficiency is associated with strict vegan diet?
B12
113
What are the only 2 appropriate indications for soy milk formula?
Galactosemia | Cultural/religious reasons
114
What vitamin deficiency is associated with gum bleeding/petechiae?
Vitamin C
115
What condition is associated with rusty hair?
Kwashikorkor
116
What is the diagnosis in a black baby with the following findings: bow-legged, metaphyseal cupping/flaring, rachitic rosary, Harrison groove, delayed walking
Rickets
117
What is the first investigation you should order in a swallowed foreign body?
AP+ lateral Neck xray CXR AXR
118
If a foreign body is located in the esophagus or stomach, what intervention is the next step?
Esophageal FB: Endoscopic removal if lodged >24 hours TO PREVENT PERF; Glucagon (0.05 mg/kg IV) facilitates passage of distal food Stomach FB: >6 cm remove within 24 hours, otherwise repeat XR in 4 weeks
119
In what time frame should button battery be removed?
Within 6 hours if in esophagus Within 24 hours if in stomach The charges on the battery can cause liquefaction necrosis and perforation can occur rapidly
120
Why must 2 or more magnets in the stomach be removed immediately?
Magnets will try to attach to each other and can tramp the bowel leading to a perforation
121
What conditions can predispose to esophageal foreign bodies?
EoE Motility disorder Esophageal atresia
122
Differential diagnosis of rectal prolapse
``` Idiopathic (most) CF Repaired HD IBD Ehler's Danlos Celiac disease Chronic dysentery Bladder extrophy Chronic constipation Malnutrition ```
123
What education should you give parents about constipation?
- Not a disease - Not dangerous - Very common - Symptoms may be due to failed toilet training - Symptoms improve when child learns the correct way to have BM - PEG-non absorbable sugar, doesn't change how bowel works, won't become addicted
124
What is the best way to treat encopresis?
Behavioural | Pharmacologic-bowel washouts
125
What investigation should you order in a child with recurrent rectal prolapse?
Sweat chloride
126
What is prevalence of celiac disease?
1%
127
Name 3 side effects of 5-ASA
Headache Bloody stools Pancreatitis
128
Name a side effect of azathioprine
Pancreatitis
129
Why do patients with IBD lose weight?
Inadequate nutrient intake
130
How many days do you have to get biliary atresia to transplant?
60 days
131
What questions should you ask on history for a patient in acute liver failure? (previous OSCE)
Symptoms: fatigue, malaise, anorexia, nausea, abdominal pain, fever and jaundice, encephalopathy Tylenol use (ALWAYS ask), medications Substances (EtOH, street drugs, Ecstasy, mushrooms, herbals) Travel (HepA), recent illness School performance, personality Δ (Wilson's) Family hx (autoimmune)
132
What 3 factors can you order to help differentiate between Vit K deficiency, DIC and liver failure?
``` Factor 8-not made in liver Factor 5-vit K independent Factor 7-vit K dependent Vit K deficiency-Factor 7 low DIC-all factors low Liver failure-Factor 5 and 7 low ```
133
In a patient with hepatitis, which test is the most sensitive indicator of liver synthetic function?
INR ``` Other markers of liver synthetic function: Albumin Glucose Lactate NH3 ```
134
What are the most common causes liver failure in >5 years?
``` Autoimmune hepatitis (raised IgG) Wilson's (cognitive issues) Acetaminophen Budd Chiari NAFLD ```
135
What is the usual initial manifestation of alpha 1 antitrypsin?
Neonatal cholestasis or later-onset childhood cirrhosis
136
How long does breastfeeding jaundice last ?
4-12 weeks
137
What initial test should you order in a baby with conjugated hyperbilirubinemia?
AUS-to differentiate between BA and other structural anomalies of biliary tree (e.g. choledochal cyst) In BA, usually do not see gall bladder
138
What is the definitive test for biliary atresia?
Percutaneous cholangiogram HIDA scan be done, but takes time (phenobarbital x 5 days) and not specific
139
What condition looks like biliary atresia on clinical presentation and biopsy, but HIDA and percutaneous cholangiogram are normal?
Idiopathic neonatal hepatitis (or intrahepatic cholestasis) Other differentiating factors: - INH recurs in families whereas BA does not - INH is more common if premature or SGA
140
How do you rehydrate mild dehydration (<5%) (CPS)?
ORS 50 ml/kg over 4 hours | Replace ongoing losses
141
How do you rehydrate moderate dehydration (5-10%) (CPS)?
ORS 100 ml/kg over 4 hours | Replace ongoing losses
142
How do you rehydrate severe dehydration (>10%) (CPS)?
Bolus 20-40 ml/kg for one hour ORT when stable Replace ongoing losses
143
You have a child with malabsorption, what tests do you do to figure out whether it's a fat, carbohydrate or protein malabsorption issue?
Fat-fecal elastase (pancreatic exocrine function, serum trypsinogen (pancreatic exocrine function), fecal fat, vitamin ADEK Carbohydrate-stool pH low, stool for reducing substances, hydrogen breath test Protein-stool alpha-1 antitrypsin
144
Name 2 signs of vitamin E deficiency
``` Hemolytic anemia Neurologic: Areflexia Ataxia Opthalmoplegia Nystagmus Impaired sensation (vibratory, proprioception) ```
145
Name 4 signs of vitamin C deficiency
Petechiae Bleeding gums Muscle pain, especially lower legs Fatigue
146
Name 3 signs of vitamin B12 deficiency
``` Macrocytic anemia Atrophic glossitis Hyperpigmentation of knuckles Sensory deficits Developmental regression ```
147
Name 3 signs of niacin (B3) deficiency
``` 4Ds Dementia Dermatitis-photosensitive Diarrhea Death ```
148
What supplementation do you recommend for a breastfed baby who's mother is vegan?
B12
149
How do you manage EoE?
Oral Fluticasone | Allergy testing and allergen avoidance diet OR hydrolyzed formulas
150
What diagnosis results in throat pain in the morning and bad breath?
GERD
151
What is Peutz Jehgers?
``` Autosomal dominant Mucocutaneous lentigines (hyperpigmented lesions) Intestinal polyposis (increased risk of intussusception) ```
152
What bacteria is associated with Guillain Barre?
Campylobacter
153
What are the NASPHAGN criteria for cyclic vomiting syndrome? (Past SAQ)
- At least 5 attacks in any interval, or a minimum of 3 attacks during a 6-month period - Episodic attacks of intense nausea and vomiting lasting 1 hr to 10 days and occurring at least one week apart - Stereotypical pattern and symptoms in the individual patient - Vomiting during attacks occurs at least 4x/hr for at least 1 hr - Return to baseline health between episodes - Not attributed to another disorder
154
What is the typical age of onset of cyclic vomiting syndrome?
2-5 years of age
155
When does physiologic GERD resolve in most infants?
Peaks at 4 months | Resolves by 15 mo in most
156
Indications for combined Multiple Intraluminal Impedance and esophageal pH Monitoring (NASPGHAN)
- Evaluating the efficacy of anti-reflux therapy - Evaluation of reflux-related symptom association (e.g. correlating cough, wheeze, chest pain with acid reflux episodes)
157
When should UGI be used in a patient with reflux?
R/O GI obstruction or complication of GERD (e.g. in patients with dysphagia, need to r/oesophageal stricture)
158
List 4 non-pharmacologic treatments for reflux in infants (past SAQ)
- Feeding technique, appropriate volume, frequency - Thickened feeds - Prone or upright position while aware - Trial of extensively hydrolyzed protein formula to r/o CMPA x 2-4 weeks
159
List 4 non-pharmacologic treatments for reflux in children
- Avoid acidic, reflux inducing foods (tomato, mint, chocolate), pop, caffeine, alcohol - Avoid night time eating - Left side position, head elevation during sleep - Weight reduction for obese - Elimination of smoke exposure
160
What is the recommended ant-reflux medication in children? (NASPGHAN)
PPI trial x 4 weeks, if successful, continue x 3 months Superior to H2RAs in relieving symptoms and healing esophagitis Prokinetics not recommended (side effects > benefits)
161
List 5 complications of GERD
``` Dental erosions Strictures Erosive esophagitis Barretts Esophagus FTT Worsening asthma Reflux laryngitis ```
162
Is there evidence for nutritional interventions in colic ? (CPS)
Only in a very small group of infants | Can trial extensively hydrolyzed formula/elimination diet x 2 weeks
163
List 5 signs to suggest an alternative diagnosis to GERD in an infant with vomiting (past SAQ)
-Failure to thrive, weight loss -Signs of dehydration -Infectious symptoms (fever) -Abdominal distention -Bilious Vomiting -Hepatosplenomegaly -Peristent, forceful vomiting -Diarrhea -Constipation -Bulging fontanelle -Macro/microcephaly -Seizures -Abdominal tenderness or distension -Documented/suspected genetic/metabolic syndrome -Abdominal tenderness or distension - New onset of vomiting after age 6 months -GI bleeding
164
What condition is often in the family history for patients with cyclic vomiting?
Migraines (80%)
165
List 5 causes of acute pancreatitis in children
- Gallstones - Triglyceridemia - Hypercalcemia - Mutations in genes for pancreatic or biliary ductal system (PRSSI, SPINK1, CFTR genes)-->progresses to chronic pancreatitis - Drug-induced - Infection (e.g. Mumps, coxsackie) - Trauma
166
List 3 serious complications of severe pancreatitis
``` Jaundice Hypocalcemia SIRS ARDS DIC ```
167
List 3 counseling points for functional abdominal pain (past SAQ)
Give diagnosis of functional abdominal pain Follow up regularly with consistency Reassure parents and child Educate parents to avoid reinforcing the symptoms with secondary gain Should return to regular activities CBT for management of pain and functional disability
168
What foods are common sources of transfats?
Fast food | Processed/pre-packaged foods
169
When should patients with UC and colonic CD be screened for colon cancer?
Colonoscopy with biopsies q1-2 years after had UC/colonic CD >10 years
170
What is pouchitis and how do you treat it?
Inflammation of J pouch after ileostomy for UC Presents with fever, bloody diarrhea, abdominal pain Stool cultures negative Treat with flagyl
171
List 4 reasons for growth failure in Crohns disease
1. Inadequate caloric intake 2. Suboptimal absorption or excessive loss of nutrients 3. Effects of chronic inflammation on bone metabolism 4. Use of corticosteroids during treatment
172
List 3 causes of lower GI bleed in a neonate <1 mo
``` Swallowed maternal blood NEC Malrotation Fissures Coagulopathy HD ```
173
List 3 causes of lower GI bleed in an infant (1 mo-2 years)
``` Allergic colitis Anal fissure Intussuseption Meckel's divericulum Infectious colitis Lymphonodular hyperplasia GI duplication Coagulopathy ```
174
List 3 causes of lower GI bleed in an older child
``` Infectious colitis IBD Juvenile polyps Adenomas/hyperplastic polyps HSP Meckel's Intussuseption Hemorrhoids ```
175
List 10 causes of esophageal dysphagia
Mechanical: i.e. Solid>liquid 1. Vascular ring 2. Mediastinal lesion (e.g. adenopathy) 3. Vertebral anomalies 4. Esophageal web, rings 5. Esophagitis: GERD, EoE chronic infections) 6. Stricture: corrosive injury, pill induced, peptic 7. Esophageal diverticula 8. Esophgeal FB 9. Schatzki ring (thin ring of mucosal tissue near the lower esophageal sphincter) Motility problems: i.e. Solid=Liquid 1. GERD 2. Achalasia cardia 3. Diffuse esophageal spasm 4. Scleroderma
176
What is the characteristic finding on barium swallow in achalasia?
Bird's beak
177
What is the best diagnostic test for achalasia?
Esophgeal manometry
178
Workup of for FTT if history and physical do not yield a diagnosis (CPS)
Step 1: * CBC * ESR or CRP * Serum lytes, venous blood gas, blood glucose * BUN, creatinine * Serum protein and albumin * Serum iron, TIBC, saturation, ferritin * Calcium, phosphate and ALP * Liver enzymes (AST, ALT, GGT) * Serum Ig * anti-TTG (accompanied by total IgA level) * TSH * Urinalysis ``` Step 2 • Sweat chloride • Vitamin levels • Fecal elastase • Bone age ```
179
``` What is the expected daily weight gain (g/day) in the following age groups? 0-3 mo 3-6 mo 6-9 mo 9-12 mo 1-3 yr 4-6 yr ```
``` 0-3 mo: 30 g/day 3-6 mo: 20 g/day 6-9 mo: 15 g/day 9-12 mo: 12 g/day 1-3 yr: 8 g/day 4-6 yr: 6 g/day ```
180
List three anthropometric measures of nutrition that can be used other than weight
1. Mid-arm Circumference 2. Triceps skin fold 3. Scapular skin fold 4. Head circumference velocity 5. Height velocity
181
Explain the difference between secretory and osmotic diarrhea
Secretory - Occurs when the intestinal epithelial cell solute transport system is in an active state of secretion - Large volume watery stools - Normal stool ion gap (<100 mOsm/kg) - Ex: cholera, toxigenic E. Coli, carinoid, C. difficile Osmotic - Occurs after ingestion of a poorly absorbed solute - Less volume - Stops with fasting - Elevated stool ion gap (>100 mOsm/kg) - Ex: Lactase deficiency, glucose-galactose malabsorption, lactulose, laxative abuse
182
List 5 causes of chronic diarrhea (>2 weeks) in an infant
- Postinfectious secondary lactase deficiency - Cow's milk or soy protein intolerance - Toddler’s diarrhea - Excessive fruit juice (sorbitol) ingestion - Celiac disease - Cystic fibrosis - Primary immune deficiency - Shwachman-Diamond - Cholestatic liver disease
183
List 5 causes of chronic diarrhea (>2 weeks) in an older child/adolescent
- Postinfectious secondary lactase deficiency - Irritable bowel syndrome - Inflammatory bowel disease - Lactose intolerance - Giardiasis - Laxative abuse - Constipation with encopresis - Thyrotoxicosis - Malignancy (e.g. VIP from neuroblastoma) - Chronic pancreatitis - Cholestatic liver disease
184
What is the most common viral gastroenteritis?
Rotavirus
185
List 4 infectious causes of bloody diarrhea
Camplyobacter Enterohemorrhagic Escherichia coli Shigella Entameoba histolytica
186
What 3 conditions can Yersinia mimic?
TB (terminal ileitis) Crohns (terminal ileitis, prolonged course of diarrhea 1-4 weeks and weight loss) Appendicitis (can present with fever, RLQ tenderness and leukocytosis)
187
List 3 clinical presentations of amebiasis
1) Intestinal amebiasis - 1-3 weeks of increasingly severe diarrhea progressing to bloody diarrhea - Abdominal pain and tenesmus - Weight loss common 2) Ameboma - Annular lesion of cecum 3) Extra-intestinal infection - Liver abscess most common most common site - Also lungs, pleura, pericardium
188
How do you treat amebiasis
- Asymptomatic cyst excreters: iodquinol, paromomycin or diloxanide (amebicide) - Mild to moderate intestinal or extra-intestinal symptoms: flagyl than amebicide (above)
189
List 2 causes of gastroenteritis that have very short incubation periods (1-6 hours)
1. S. Aureus - Source: Prepared foods (eg salads, dairy, meat) 2. B. Cereus Source: Rice, meat Incubation time is 1-6 hours for both!
190
Which is the only sugar that is does not result in a positive for stool reducing substances?
Sucrose
191
What clues on history are suggestive of toddler's diarrhea as a cause of diarrhea in infants?
- Normal growth - Well appearing - More severe in AM
192
What is the most common cause of congenital diarrhea?
Microvillus inclusion disease
193
List 4 causes of protein losing enteropathy (past SAQ)
- Milk protein allergy - Celiac disease - Inflammatory bowel disease - Infections (ie. Giardiasis) - Intestinal lymphangiectasia - Right sided heart dysfunction (post-Fontan procedure) - Hypertrophic gastritis (Ménétrier's disease)
194
How do you treat protein losing enteropathy?
Low-fat, high-protein, medium-chain triglyceride (MCT) diet.
195
Which has higher fat content, hind milk or fore milk?
Hind milk has 50 % higher fat content than fore milk
196
How does the composition of breastmilk for preterms differ from term infants?
Milk for a preterm infant is high in protein, lower in carbohydrates and fat but higher in calories (similar to colostrum)
197
What are the only indications for soy based formula?
Galactosemia Congenital lactase deficiency Vegan family
198
Introduction of lumpy foods should not be delayed beyond __ months
9 months
199
What is the maximum amount of cow's milk recommended per day for toddlers?
Max of 750 mls per day = 25 ounces
200
Avoid honey under __ months because of the risk of botulism
12 months
201
When should complementary foods be introduced?
6 months | Should iron rich-meat, meat alternatives, and iron fortified cereals
202
What intervention can be tried in infants with severe colic? (CPS)
Time limited (2 week) trial of hydrolyzed formula to r/o CMPA
203
Soy formula should not be used in what two groups of infants?
Congenital hypothyroidism | Non-IgE mediated CMPA
204
What kind of water should be used to prepare powdered formula?
Boiled water because powdered formula is NOT sterile | Concentrate is sterile-can use tap water
205
List 4 ways milk can cause iron deficiency
Low in Fe Can displace Fe-rich foods Can inhibit Fe absorption Excessive intake can cause occult blood loss in stool
206
What dietary recommendations would you make for a child 12-24 months (CPS)?
- Begin regular schedule of meals and snacks - Little or no added salt or sugar - Need nutritious, higher-fat foods - Encourage continued BF, or 500 mL per day homo (3.25%) milk after 9-12 months - Limit juice and sweetened bev to max 1-2 x/day; encourage water for thirst - Transition from bottle to open cup should be complete by 12 mo
207
How long should vitamin D BE continued in breastfed child?
Continue vitamin D 400 IU daily if BF up to 24 mo
208
List 5 causes of PUD
H. pylori NSAIDs Zollinger ellisons Stress-sepsis, shock, severe burns
209
Treatment for H. pylori gastritis
2/3 of Amox/Clarithro/Flagyl x2 weeks + PPI x 1 month
210
List 5 causes of malnutrition in neurologically impaired children (CPS)
1. Inadequate Intake 2. Increased losses (GERD &frequent emesis and regurgitation may be a source of caloric loss) 3. Altered metabolism -May require more calories because of increased tone 4. Oromotor dysfunction 5. Non-nutritional factors affecting growth (specific syndromes, endocrine dysfunction, ethnicity, genetic potential, puberty)
211
List 5 nutritional interventions for neurologically impaired child who is malnourished (past SAQ)
1. Determine Energy Needs - Can use indirect calorimetry - Other methods: Krick method, height-based method, REE-based method 2. Improve oral intake - Adequate positioning - Adjust food consistency - Increase caloric density 3. Enteral nutrition - Trial of NG feeds - If required >3 months, G-tube - Consider fundo if reflux refractory to medical management - GJ if not candidate for fundo 4. Manage reflux 5. Enteral formula - If need high-calorie or not tolerating increased formula volume, may concentrate and/or add modular nutrients, such as glucose polymer or lipids
212
What weight for height %iles should be targeted for neurologically impaired children?
In children <3 y.o.and in children with normal activity level: -50th percentile In children who are wheelchair bound but able to accomplish transfers: -25th percentile For the bedridden patient: -10th percentile
213
Above 12 months of age, what formula should be used in neurologically impaired children? (CPS)
A paediatric 1 kcal/mL formula
214
What is the best form of nutrition for a patient with short gut syndrome?
Protein hydrolysate and medium-chain triglyceride–enriched formula
215
How much vitamin D should be given to preterms?
200 IU (up to 400 IU)
216
How much vitamin D is in formula?
400 IU per 1 L of formula
217
List 4 effects of excess vitamin D (past SAQ)
Related to hypercalcemia: 1. Abdominal pain 2. Polyuria 3. Nephrolithiasis 4. HTN 5. Constipation 6. Pancreatitis 7. Lethargy
218
How much iron should be given to preterm infants during the first year of life? (CPS)
2 mg/kg/day of elemental iron, introduced between four and six weeks of age
219
What is the rationale for iron supplementation in prems (CPS)?
Physiologic nadir is more significant in prems Supplementation results in improved hemoglobin levels and iron stores (after 6 mos of age)
220
What two pieces of advice about vitamins and fluoride would you give to a mother with a newborn who lives in a community with only well water?
1. Vitamin D 400 IU if exclusive breastfeeding or 800 IU if risk factors 2. Supplemental fluoride after 6 months - Dose= 0.25mg/day from 6mos-3yrs if not brushing teeth twice a day - Supplemental fluoride should be given in preparations that maximize the topical effect, such as mouthwashes or lozenges
221
What supplement should you give a newborn of a breastfeeding vegan mother?
Vitamin B12
222
List 3 things you would do to refeed a child with Kwashiorkor safely
1. Initiate feeds with a specialized high calorie formula (F 75), small amounts frequently to aim for 80-100kcal/kg/day 2. Do not start iron until rehabilitation phase (week 2) 3. Monitor electrolytes, including phosphate for refeeding syndrome
223
List 4 clinical features of Wilson's disease
1. Hepatitis to hepatic failure 2. Neurologic: tremor, chorea, parkisonism, personality change, deterioration in school performance 3. Kayser fleischer rings 4. Coombs-negative hemolytic anemia 5. Renal fanconi syndrome
224
List 4 risk factors for TPN cholestasis (past SAQ)
1. Prematurity 2. Low birthweight 3. Prolonged duration of TPN 4. Sepsis 5. NEC 6. Short gut syndrome
225
List 5 hepatotropic viruses other than the hepatitis viruses
``` HSV EBV/CMV VZV Adeno Enterovirus Parvo Arboviruses Coxsackie Dengue Yellow fever HIV Rubella ```
226
List 3 laboratory clues that indicate a diagnosis of autoimmune hepatitis
↑AST/ALT from 100-300, but can be >1000 Conjugated hyperbilirubinemia Autoantibodies-anti-smooth muscle, ANA, LKM Hypergammaglobulinemia Cytopenias
227
List 5 signs of poor prognosis in acute liver failure
- Stage 4 encephalopathy - Age <1 y.o. - INR >4 - Need for dialysis before transplant - Acute liver volume loss***(on past exams)
228
List 3 complications of liver transplant (past SAQ)
``` Acute and chronic rejection Thrombosis (portal vein or hepatic artery) Infection Metabolic syndrome PTLD Osteopenia ```
229
List 5 indications for liver transplant in children
Biliary atresia Metabolic disorders: α1-Antitrypsin deficiency, tyrosinemia type I, GSD type IV, Wilsons, neonatal hemochromatosis, Crigler-Najjar type I, familial hypercholesterolemia, primary oxalosis, organic academia, UCDs Acute hepatitis: fulminant hepatic failure, viral, toxin, or drug induced Chronic hepatitis with cirrhosis: hepatitis B or C, autoimmune Intrahepatic cholestasis: idiopathic neonatal hepatitis, Alagille, familial intrahepatic cholestasis Miscellaneous: cryptogenic cirrhosis, congenital hepatic fibrosis, Caroli disease, CF, polycystic liver disease, TPN induced cirrhosis
230
List 4 causes of portal HTN in children
1) Extrahepatic: - Portal vein thrombosis - Portein Vein agenesis/atresia/stenosis - Splenic Vein Thrombosis 2) Intrahepatic Causes: Hepatocellular Disease - Hepatitis (acute, chronic) - Wilson’s - α1-Antitrypsin deficiency - Hepatotoxicity (from drugs, toxins, TPN) Biliary Tract Disease - Biliary Atresia (extrahepatic) - CF - Choledochal cyst - Sclerosing Cholangitis Post-sinusoidal Obstruction - Budd-Chiari Syndrome: - Veno-occlusive Disease
231
How does Gilbert syndrome typically present?
Neonatal jaundice Recurrent episodes of unconjugated hyperbilirubinemia
232
What is Dub-John and Rotor?
AR cause of mild conjugated hyperbilirubinemia Usually detected during adolescence Recurrent episodes of jaundice exacerbated by infection, pregnancy, OCPs, alcohol, and surgery
233
List 6 clinical features of Alagille syndrome
- Dysmorphic Features: broad forehead, deep-set + widely spaced eyes, long/straight nose,pointed chin - Eyes: posterior embryotoxon, microcornea, optic disk drusen, shallow anterior chamber - CVS: peripheral pulmonic stenosis, and other CHD - Vertebral: butterfly vertebrae, fused vertebrae, spinabifidaocculta, rib anomalies - Nephro: Tubulointerstitial nephropathy - Short stature - Pancreatic insufficiency - Defective spermatogenesis
234
List 3 long term complications of chronic cholestasis
``` Fat malabsorption Metabolic bone disease Pruritus Xanthomas Cirrhosis Portal HTN ```

FRCPC (27 decks)

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