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laughing angel

ED find: cirrhosis in a 15yo female

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Odd find last night... not quite sure what to make of it.

 

Parents bring in a 15yo female with abd pain for 5h. Last week she had a similar pain but not as severe as tonight. Had diarrhea at the time. Went to UC where she was given rx for Lomotil and Amoxil and the diagnosis "abd pain." No labs on paperwork they bring with them. Pain and diarrhea resolved over 3 days.

 

On exam - tachy in the 120s, temp 100.4o, otherwise wnl.

skin was an odd shade of green on the face, but nowhere else - I thought it might be a poor choice of makeup.

abd rigid. pt localizes pn to rlq, but tender everywhere. unable to appreciate organs.

 

CT abd/pelvis with IV and PO contrast (hospital protocol), CBC, CMP, serum hcg ordered.

 

While waiting for pt to finish po contrast, labs come back. CO2 18, liver enzymes generally elevated 1.5-2x nl across the board (from what I remember... didn't print out, but can get actual numbers).

WBC 12.1. Hct up slightly, hgb nl.

 

I was somewhat focused on ruptured appy, so I didn't chas the elevated liver enzymes. Reviewed with attending who didn't suggest anything either.

 

CT comes back with small, severely cirrhotic liver with portal htn. splenomegaly (it took up nearly 1/4 of abd). ascending and transverse colon wall thickening c/w infectious colitis. Significant free fluid, no air. Appendix nl; rest of ct nl.

 

Of course, any time anything interesting comes up an attending takes over, so all I know about her from this point on is what is in our chart. She had a pelvic US done that was neg, then she was transferred to the local children's hospital.

 

Any thoughts? This pt had no prior hx of GI complaints, and family adamantly deny fmh of anything similar.

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Hepatitis,Cholestasis or malignancy(leukemia)?

It doesn't sound good whatever the cuprit. If you get follow-up would be interested.

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Odd find last night... not quite sure what to make of it.

 

Parents bring in a 15yo female with abd pain for 5h. Last week she had a similar pain but not as severe as tonight. Had diarrhea at the time. Went to UC where she was given rx for Lomotil and Amoxil and the diagnosis "abd pain." No labs on paperwork they bring with them. Pain and diarrhea resolved over 3 days.

 

On exam - tachy in the 120s, temp 100.4o, otherwise wnl.

skin was an odd shade of green on the face, but nowhere else - I thought it might be a poor choice of makeup.

abd rigid. pt localizes pn to rlq, but tender everywhere. unable to appreciate organs.

 

CT abd/pelvis with IV and PO contrast (hospital protocol), CBC, CMP, serum hcg ordered.

 

While waiting for pt to finish po contrast, labs come back. CO2 18, liver enzymes generally elevated 1.5-2x nl across the board (from what I remember... didn't print out, but can get actual numbers).

WBC 12.1. Hct up slightly, hgb nl.

 

I was somewhat focused on ruptured appy, so I didn't chas the elevated liver enzymes. Reviewed with attending who didn't suggest anything either.

 

CT comes back with small, severely cirrhotic liver with portal htn. splenomegaly (it took up nearly 1/4 of abd). ascending and transverse colon wall thickening c/w infectious colitis. Significant free fluid, no air. Appendix nl; rest of ct nl.

 

Of course, any time anything interesting comes up an attending takes over, so all I know about her from this point on is what is in our chart. She had a pelvic US done that was neg, then she was transferred to the local children's hospital.

 

Any thoughts? This pt had no prior hx of GI complaints, and family adamantly deny fmh of anything similar.

 

This is probably cryptogenic cirrhosis (most of which I am rapidly starting to believe are autoimmune). Other possibilities are Wilson's or Alpha-1 antitrypsin. I have seen one or two kids with Cirrhosis secondary to HCV and your patient would have been a newborn right about the time we started testing for HCV. No mention of HBV? If the parents are Asian think carrier. The colitis brings up the possibility of PSC with undiagnosed UC. Malignancy is a rare cause of liver failure in peds.

 

What was the Bili? You didn't mention jaundice so that would go against the cholganitis type stuff.

 

With the fluid which I would assume is ascites you probably have SBP.

My DDx would be:

Autoimmune

Alpha-1

Wilson's

Viral (both Hep and CMV/EBV)

 

Either way she needs to be evaluated for a transplant. This has been going on for some time and the presentation is just a manifestation of the cirrhosis not the underlying disease.

 

David Carpenter, PA-C

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Guest lisnek
Could be Sclerosing Cholangitis if the patient has IBD.

 

could be, esp w that CT!

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http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1432410

 

heroin use linked to cirrhosis in adolescents...

 

I would point out when that was published (ie before the HCV ab test was present). Also they used young as below 35. The average age was around 20. This probably represents a cohort of heroin users with HCV infection who are rapid progressors (about 5-10% of HCV patients).

 

David Carpenter, PA-C

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could be, esp w that CT!

 

Could be but it would be unusual to see cirrhosis in this age group. The pattern would be atypical for UC. There is some debate whether Crohn's is associated with PSC. There is another school that labels all non AMA positive cholangitis with autoimmune features as Autoimmune cholangitis. Kind of moving away from PSC.

 

I think this does resemble some type of autoimmune cholangitis but again the presenting complaint is usually puritis or jaundice neither of which seems to be present here. The other question that the OP did not mention is the patients body habitus. We have seen occasional cases of pediatric NASH in 15 year olds. Sad but it happens.

 

Given the overall presentation I would be this is going to end up cryptogenic. The other thing to think about is long term Tylenol use. Is the patient an athlete? We have seen a couple of overdose cases with short term (3 weeks of Tylenol at the top end of the dose). You could postulate a long term (2-3 years) at the top end of the dose which could end in cirrhosis.

 

As you are discovering the diagnosis of liver disease is complex. I usually start with labs and biopsy. This will usually get the answer.

 

Laughing Angel - if you get a chance can you post the labs. LFTs, bili, and CR, INR would be interesting.

 

David Carpenter, PA-C

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CBC Without Diff Sep 10 2007 00:27

WBC7.1K/MM34.5-13.0

RBC4.52M/MM34.20-5.60

Hgb15.6g/dL12.0-15.0

Hct44.2%35.0-45.0

MCV98fL78-95

MCH34.4pg26.0-32.0

MCHC35.2g/dL31.0-37.0

RDW14.8%12.1-18.2

Platelet70K/MM3130-450

 

Comprehensive Metabolic Panel Sep 10 2007 00:27

Sodium137mmol/L135-145

Potassium3.1mmol/L3.5-5.2

Chloride113mmol/L96-110

CO218mmol/L21-31

Anion Gap64-16

Glucose Random97mg/dL65-99

BUN5mg/dL8-25

Creatinine0.6mg/dL0.6-1.1

AST117IU/L10-41

ALT64IU/L2-35

Alkaline Phos349IU/L50-162

Bilirubin Total4.6mg/dL0.2-1.1

Calcium7.6mg/dL8.7-10.4

Protein, Total6.2g/dL5.4-7.6

Albumin2.1g/dL2.9-4.1

BUN/Creat Ratio810-28

Alb/Glob Ratio0.51.0-2.0

 

UA/I Sep 10 2007 00:13

Color, URYellowYellow

Appearance, URClearClear

Glucose, URNegativeNegative

Protein, URNegativeNegative

Bilirubin, URNegativeNegative

Urobil, UR2.0Normal

pH, UR6.05.0-9.0

Blood, URNegativeNegative

Ketones, URNegativeNegative

Nitrite, URNegativeNegative

Leuk Esterase, URNegativeNegative

Spec Gravity, UR1.0051.005-1.03

 

HCG, QualNegative

 

If I get a chance, I'll call over to the accepting hospital and see if they can give an update...

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NASH? Guess 15 is a little young though.

 

Possible but unusual. With the RLQ pain could this be PSC - possible but unlikely. My differential would be:

1. Autoimmune

2. Wilson's

3. Viral (pretty unlikely).

Pretty short. Other weird things like storgage disorders are too rare to show up but would be considered. The MELD is 12 (assuming normal INR) so she should be listed and managed for symptoms. She has evidence of splenic sequestration but she is maintaining her sodium. I would call her a compensated cirrhotic unless the RLQ pain turns out to be SBP.

 

Interesting case.

 

David Carpenter, PA-C

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Wilson's was the first thing that popped into my head and its sticking.

Yeah but this is cirrhosis. If she was 40 then maybe but I have never even heard of a cirrhotic at this age.

You would have to consider something like Budd-Chirari but that would be rare also. Viral also. Laughing Angel - any update.

 

David Carpenter, PA-C

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Not that it matters much now, 5 months out, but my knee-jerk reaction was Type II autoimmune hepatitis. In addition to the typical "liver spew" as we call it, an anti-Liver-Kidney-Muscle antibody can help pick that up.

 

AST:ALT ratio & TCP is sure c/w cirrhosis, if there wasn't ample evidence already...

Edited by Tom Scoufalos
Typo

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