IBD Case of the Month: Pediatric Diagnosis of IBD - PowerPoint Presentation

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IBD Case of the Month:Pediatric Diagnosis of IBD

Developed by the CCFA Nursing Initiatives Committee

Author: Kristin Madden, NP

University

of Nebraska Medical Center

Children’s Hospital & Medical Center

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Instructions

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Objectives

Identify ‘red flag’ symptoms and how to order labs/diagnostics to lead you to diagnosis.

Identify radiologic, laboratory, and more invasive methods of testing for diagnosis of IBD.

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Introduction/Background

November 2011: a 21 month old female presents to clinic with chief complaint of hematochezia.

History includes 6-8 weeks of loose stools (4-5x/day) with visible mucus and bright red blood. No recent laboratory tests have been conducted.

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What additional information will be helpful?

What is the family history?

Is there important birth history?

Is the review of systems revealing of additional factors?

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Review of Systems (ROS)What is important & why

General

: pertinent negatives – no recent travel, no recent antibiotics. This is important to evaluate as we need to consider infectious etiologies to the presenting symptoms.

Skin

: no eczema, no erythema

nodosum

or

pyoderma

gangrenosum

. These findings are supportive evidence for allergic vs inflammatory or autoimmune diseases.

ENT

: Determine if there are any additional chronic disease processes or mouth sores that could support

Crohn

Disease.

Respiratory

: Any chronic cough, asthma or pneumonias that would indicate aspiration or compromised immune system?

Cardiovascular

: Rule out chronic disease of heart, hypertension, etc.

GU

: Rule out anatomical issues or urinary reflux.

Muscular/Skeletal

: Is there

hypotonia

, developmental delay or

syndromic

appearances?

Hematologic/Lymphatic

: Easy bruising/bleeding present (liver disease)? Any enlarged lymph nodes?

Neurologic

: Headaches or irritability present?

Endocrine

: Are there current Autoimmune diseases present increasing risk for GI Autoimmune Disease

?

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Physical Exam

Vitals

: Temp – 36.5, Pulse – 109,

Resp

– 30, BP – 94/69

Growth

: Head Cir – 45.7, Height – 77.6, Weight – 9.75, Weight for Length – 35.75%

General

: alert, no distress

Head

:

normocephalic

Eyes/Ears/Nose/Throat

: sclera clear, conjunctiva pink, nose clear, throat clear, without oral lesions

Neck

: supple, no masses

Lungs

: clear to auscultation bilaterally

CV

: regular rate and rhythm, no murmur, equal pulse and cap refill<3 sec

Abdomen

: soft,

nondistended

,

nontender

, no

organomegaly

, normal bowel sounds, no masses/hernia/guarding. Liver edge palpable 2-3 cm below right costal margin

Skin

: No eczema and no skin rash noted.

Musculoskeletal

: No reported joint pain or stiffness

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Previous Workup

Radioallergosorbent

test (RAST) positive for cows milk allergy

Complete blood count (CBC) – normal/no anemia

Liver enzymes (AST/ALT) – 614/832; elevated

Erythrocyte Sedimentation Rate (ESR) – 64; elevated inflammatory marker

C-reactive Protein (CRP) – 11.1; elevated inflammatory marker

Fecal occult blood – positive

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Do you have red flags/cause for concern based on physical exam & previous workup?

No concern

Only minimal concern

Significant concern

Major concern indicating need for admission

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Do you have a Differential Diagnosis?

Autoimmune hepatitis or Primary

Sclerosing

Cholangitis

Celiac Disease

Constipation

Crohn's Disease

Functional Abdominal

Pain

Immune Deficiency

Infection

Irritable Bowel Syndrome

Metabolic Disease

Milk +/- soy protein allergy

Ulcerative

Colitis

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What would be ordered for workup?

Allergy testing

Blood work

Capsule endoscopy

CT enterography or MR enterography

Liver Biopsy

pH probe

Upper endoscopy and colonoscopy

Stool

studies

Upper GI

Nothing

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Laboratory Results

CBC

– unremarkable

ESR

53 (h), CRP 0.7

AST

- 345/ALT – 925 (h)

GGT

– 446 (h)

ANA

– negative

SMA

– 48 (+)

IgG

– 1405 (h)

ANCA

– 1:80 (+)

Fecal

Calprotectin

1207 (h)

Acute Hepatitis Panel

(-)

Stool Culture, C-diff and Adeno

(-)

AFP

– 4

CPK

– 116

Urine organic/serum amino

(-)

Scopes (pathology) – Cecum, transverse, descending and ascending colon with focal acute colitis.

Liver biopsy (pathology) – Dense portal inflammation with cholangiolar proliferation. Cholangitis. Portal fibrosis with bridging fibrosis Stage III/IV.

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What is your Diagnosis?

Autoimmune liver disease based on liver biopsy results:

Dense portal inflammation with

cholangiolar

proliferation. Cholangitis. Portal fibrosis with bridging fibrosis Stage III/IV

.

Cholangitis can be seen in Autoimmune or Primary

Sclerosing

Cholangitis.

Inflammatory Bowel Disease – likely ulcerative colitis based on pathology:

Cecum, transverse, descending and ascending colon with focal acute colitis

. Prefer to see both chronic and acute inflammation. But will start treatment based on these results.

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What is your plan of care?

Treat colitis with Sulfasalazine 10mg/kg TID

(maintenance dose for >2 years is 30-50 mg/kg/day)

Prednisolone 1 mg/kg BID

Azathioprine 1 mg/kg (after obtaining TPMT enzyme activity level +/

- genetics)

Follow clinical response to treatment and laboratory response to treatment

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SummaryIn this case study it is important to complete a workup and not be distracted by the young age of the patient or the history of milk protein allergy as an explanation for blood in the stool.

If workup had not been completed it could have been easy to miss colitis and liver disease.

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Thank you!

We hope you enjoyed this case. Check back next month for a new case!

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INCORRECT

The symptoms are concerning and intervention/further workup is necessary for the ongoing health and safety of the child.

Anemia and

hypoalbuminemia

may develop.

Ongoing elevation of liver enzymes may progress to damage the liver permanently.

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question

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INCORRECTThe symptoms are concerning and intervention/further workup is necessary for the ongoing health and safety of the child

Minimal concern would indicate you are not making appropriate decisions for intervention.

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question

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CORRECT

The symptoms are concerning and intervention/further workup is necessary for the ongoing health and safety of the child

Significant concern indicates you will order workup based on symptoms you feel are urgent but not emergent.

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question

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INCORRECT

The symptoms are concerning and intervention/further workup is necessary for the ongoing health and safety of the child

Symptoms are not emergent. There is not a clinical indication affecting safety of the child.

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question

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CORRECTCrohn's Disease is a chronic inflammatory process that can affect the GI tract from the mouth to the anus usually in a skip lesion or discontinuous process.

Most common presenting symptoms include diarrhea, abdominal pain, fever and weight loss.

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question

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UNLIKELYCeliac disease is a lifelong sensitivity to gluten caused by an immune response. It results in damage to the small intestine and can present in a variety of ways including: chronic or recurrent diarrhea, abdominal distention, anorexia, weight loss/failure to thrive, abdominal pain, vomiting and/or constipation.

However WILL NOT present with bloody diarrhea.

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question

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CORRECTUlcerative Colitis is a chronic relapsing disease affecting the colon and rectum. Generally inflammation is continuous. Presentation is similar to that of Crohn's Disease with higher likelihood of rectal bleeding in addition to diarrhea, abdominal pain, fever and weight loss.

Abdominal pain/cramping before bowel movement, fever and weight loss can also be seen

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question

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CORRECTImmune deficiency is a state in which the immune system's ability to fight infectious disease is compromised or entirely absent.

A person who has an immunodeficiency of any kind is said to be immunocompromised. An immunocompromised person may be particularly vulnerable to opportunistic infections, in addition to normal infections that could affect everyone.

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question

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CORRECTInfection needs to be on the differential as presenting symptoms many times include bloody diarrhea. If overlapped by viral illness may see increase in liver enzymes as well.

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question

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CORRECTAutoimmune

l

iver disease is a progressive inflammatory disorder characterized by high levels of transaminases and immunoglobulin G (IgG). Sometimes autoimmune

l

iver

d

isease may overlap with primary

sclerosing

c

holangitis.

These liver diseases may be seen in conjunction with ulcerative

c

olitis.

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question

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CONSIDERED

Metabolic disease is more likely explanatory for the elevated liver enzymes but not for the hematochezia.

Metabolic diseases are the second most common indication for liver transplantation. These include: Wilson’s Disease, alpha1 antitrypsin deficiency, Crigler-Najjar syndrome, inborn error of bile acid metabolism,

tyrosinemia

, disorders of the urea cycle, organic acidemia, acid lipase defect,

oxaluria

type I and disorders of carbohydrate metabolism.

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question

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UNLIKLEYMilk and soy

p

rotein

a

llergy or intolerance may be characterized by either constipation or diarrhea and there may be blood present. However, new onset at 21 months of age is unlikely. Additionally this would not explain elevated liver enzymes.

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question

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INCORRECT Irritable bowel

s

yndrome is a diagnosis of exclusion. Diagnosis made when we are not able to explain symptoms with an organic disease state. We would not see elevated liver enzymes or

hematochezia

with this diagnosis.

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question

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INCORRECTFunctional abdominal

p

ain is abdominal discomfort or pain at least once per week for at least 2 months.

There is also no evidence of an inflammatory, anatomic, metabolic or neoplastic process that explains the symptoms.

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INCORRECTConstipation is the decrease in frequency of bowel movements or difficulty defecating associated with distress. There may be associated blood, but it is related to the hard stool.

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CORRECTCBC, CMP, ESR, CRP, PT/INR, GGT, ammonia, TSH/Free T4, acute

h

epatitis

p

anel, ANA, IgG, ANCA, smooth

m

uscle

a

ntibody, anti

l

iver

k

idney

m

icrosomal Ab, AFP, CPK, urine

o

rganic

a

cid, serum

a

mino

a

cid, complement

s

tudies,

acylcarnitine

profile, HIV

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question

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CORRECTFecal calprotectin, stool

c

ulture, C-

difficile

,

a

denovirus, fecal

o

ccult

b

lood

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INCORRECTClinical symptomatology warrants investigation

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Slide35

INCORRECT Allergy testing would not be helpful for diagnosing hematochezia and elevated liver enzymes

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CORRECTBoth esophagogastroduodenoscopy and colonoscopy will be useful to discover etiology of clinical presentation, although would not be first line. Will want to know lab results and stool study findings prior to scopes.

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CORRECTLiver biopsy is indicated to work up the elevated liver enzymes. Ideally coordinated with scopes for single anesthesia exposure.

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INCORRECTUpper GI is not useful for this presentation. UGI is classically used to look for malrotation or causes of gastric outlet obstruction.

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INCORRECTCapsule endoscopy can be useful in determining GI bleed without known cause after upper and lower endoscopies have been completed.

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question

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Correct CT or MR enterography can better define at both an intraluminal and extraluminal level. If Crohn's Disease is diagnosed and there is question of further small bowel involvement +/- abscess or fistula formation this test can be helpful.

It is important to recognize that this IS NOT first line for diagnostic purposes. But can be helpful in determining extent of disease.

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question

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INCORRECTpH probe is useful in measuring the exposure of acid reflux from the stomach to the esophagus. It is not indicated in this clinical presentation.

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question

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Family History

Mom – possible

Crohn

Disease

Maternal Aunt – Ulcerative Colitis

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question

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Birth HistoryBorn full term, vaginal delivery, uncomplicated pregnancy, normal birth weight & appropriate weight gain during first year of life.

Milk Protein Allergy – required elemental formula.

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question

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Review of Systems (ROS)

General: denies fatigue, no fever, good appetite, sleeps well, no weight loss, no recent travel and no recent antibiotic exposures

Skin: no skin rash (e.g. eczema)

Ear/Nose/Throat: no mouth sores, denies frequent sinus or ear infections

Respiratory: no asthma, chronic cough and no episodes of pneumonia

Cardiovascular: negative

GU: negative

Muscular/Skeletal: negative

Hematologic/Lymphatic: negative

Neurologic: negative

Endocrine: negative

Psychosocial: negative

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question

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Complete Blood Count (CBC)

The CBC and differential are a series of tests of the peripheral blood that is inexpensive and easily/rapidly performed as a screening test.

CBC allows us to understand if anemia is present, platelet count is high or low (liver disease) or if infection may be present.

It can help us determine if urgent vs. emergent response is needed

It establishes a baseline to understand response to treatment.

Back to

labs

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Inflammatory markers (ESR/CRP)

ESR – non-specific method for detecting illnesses associated with

chronic

or acute infection or inflammation. Remember it is non-specific and therefore not diagnostic for any particular organ disease or injury.

CRP –

acute

phase reactant protein used to indicate an inflammatory illness. A positive result indicates the presence but not the cause of an

acute

inflammatory reaction. Again non-specific.

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labs

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Liver Enzymes (AST/ALT)

AST – used in evaluation of patients with heart disease or suspected hepatocellular disease. If cellular injury is chronic, levels will be persistently elevated. Found in heart muscle, liver cells, skeletal muscle cells, kidneys, pancreas and red blood cells.

ALT – used to identify hepatocellular disease of the liver. Accurate monitor of improvement or worsening of liver diseases. Found predominately in the liver, lesser in the kidneys, heart and skeletal muscle.

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labs

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Gamma-Glutamyl Transferase (GGT)

A sensitive indicator of

hepatobiliary

disease. The highest concentrations of this enzyme are found in the liver and biliary tract.

This test is used to detect liver cell dysfunction and it is highly accurate in indicating even the slightest degree of cholestasis.

This is the most sensitive liver enzyme for detecting biliary obstruction, cholangitis or

cholecystitis

.

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labs

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Antinuclear antibody (ANA)

Used to detect autoimmune diseases.

Back to

labs

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Smooth Muscle Antibody (SMA)

Used primarily to aid in the diagnosis of autoimmune hepatitis.

Is positive in 70-80% of patients with autoimmune hepatitis.

Back to

labs

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Immunoglobulin G (IgG)

Used to detect and monitor the course of diseases including immune deficiencies, autoimmune diseases, chronic infections as well as several others.

Back to

labs

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Antineutrophil cytoplasmic antibody (ANCA)

ANCAs are antibodies directed against cytoplasmic

c

omponents of neutrophils. There are two types: C-ANCA and P-ANCA

P-ANCA is found in as many as 75% of patients with ulcerative colitis or

sclerosing

cholangitis. As well as 50% of patients with autoimmune hepatitis.

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labs

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Fecal Calprotectin

Neutrophil protein found in the stool. Elevated in the presence of inflammation or infection.

A helpful screening test for patients presenting with abdominal pain and stool changes.

Can be useful to monitor IBD and response to treatment.

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labs

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Acute Hepatitis Panel

Screens for Hepatitis A, B & C.

Used to screen for causes of elevated liver enzymes.

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labs

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Infectious Stool Studies

Adenovirus – virus that can cause diarrhea.

Stool culture – used to screen for enteric bacteria including salmonella,

shigella

and campylobacter. These can cause diarrhea +/- blood.

Clostridium

difficile

– an infectious toxin that can cause bloody diarrhea.

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labs

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Alpha-Fetoprotein (AFP)

Used as a tumor marker to identify cancers such as

hepatomas

.

Will be elevated in newborn babies.

Increased levels are found in as many as 90% of adult patients with

hepatomas

.

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labs

Slide57

Creatine phosphokinase (CPK)

Used to identify neurologic or skeletal muscle diseases.

Helpful when working up elevated liver enzymes to determine if coming from liver of muscle disease.

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labs

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Metabolic Studies

Urine organic acids – urine test to screen for metabolic disease that could explain elevated liver enzymes.

Serum amino acids – blood test used to screen for metabolic disease that could explain elevated liver enzymes.

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labs