Hep B-related Hepatocellular Carcinoma in Kids: 8 Needles in 4 Haystacks

Over a 25-year period, investigators (DB Mogul et al. JPGN 2018; 67: 437-440) from 4 medical centers identified 8 patients (8-17 years) with hepatocellular carcinoma (HCC) associated with hepatitis B virus (HBV).

The authors indicate that all of the cases were thought to have acquired HBV via vertical transmission.

Key features:

  • 3 were asymptomatic; 50% reported abdominal pain
  • Only 1 case presented to a hepatologist
  • 4 patients had ALT values <1.5 times the upper limit of normal
  • Among those with documented HBeAg (n=3), all were negative and all were positive for anti-HBeAb
  • Alphafetoprotein was elevated in 3 patients, normal in 2 patients and not documented in 3 patients.

My take: HCC rarely occurs in children with HBV.  The most effective way to reduce HCC is through prevention, particularly vaccination.  The role of regular imaging which could detect tumors earlier remains unclear (in the absence of a risk factor like cirrhosis); in this series, only one patient presented to a hepatologist.

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Lake Agnes, Banff

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#NASPGHAN18 Highlights (Part 2)

I did not make it to this year’s meeting but did get a chance to catch up on a lot information via the PG 2018 Syllabus and based on information posted online.

Here are a couple of highlights for me:

My favorite slide from postgraduate course -Dr. Robert Kramer

Slides regarding the topic of Treat-toTarget Dr. Eric Benchimol:

Slides regarding GI symptoms and autism from Dr. Kara Margolis:

Slide regarding the frequency of bariatric surgery: Dr. Rohit Kohli:

Slides regarding intestinal failure population from Dr. Conrad Cole:

From Dr. Miranda van Tilburg regarding psychological therapies for functional GI disorders:

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications/diets (along with potential adverse effects) should be confirmed by prescribing physician/nutritionist.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.

Briefly Noted: Progression of Fatty Liver Disease on MRI

M Mouzaki  et al. J Pediatr 2018; 201: 86-92. This study with 65 patients evaluated nonalcoholic fatty liver disease (NAFLD) progression between two MRI studies, with a median time span of 27 months.

Key findings:

  • There was no correlation between change in liver stiffness and change in ALT; there was a weak correlation between ALT change and fat fraction.
  • MRI fat fraction and stiffness decreased in 29% and 20% of patients respectively and increase in 25% and 22% respectively.

My take: When we find effective therapies, we will need better non-invasive markers to follow NAFLD progression.

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Tea House Trail, near Lake Louise, Banff

Pediatric Fatty Liver Disease Study: New York City

A recent autopsy study (DM Fernandes et al. J Pediatr 2018; 200: 174-80) examined nonalcoholic fatty liver disease (NAFLD) in a pediatric cohort of 582 (2-19).  Approximately 75% were in 14-19 years of age and 50% were black; black pediatric patients (n=290) were over-represented in this sample only 25% of the New York population is black or African American based on the 2010 census.

Key findings:

  • Causes of death: 49% homicides, 31% accidents, 10% acute illness, 9% suicide, 1% other
  • Overall, NAFLD was present in 4.5%; this low overall prevalence was due in part to the low rate of NAFLD in black children; only 3 of 290 (1%) had NAFLD and none had nonalcoholic steatohepatitis (NASH)
  • The rate of NAFLD was 7.9% in hispanics and 8.3% in white patients.
  • In this cohort, 36% were overweight or obese.  In this subgroup, 14.1% of hispanics and 14.8% of whites had NAFLD.
  • Overall, NASH was present in 1.7% of the entire cohort.  NASH and fibrosis have been shown in prior studies as the best predictors of disease progression

My take: If black children are not killed by homicide or accidents, it is unlikely that they will die from NAFLD due to its low prevalence.

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Effects of Surgical Shunts on Liver Atrophy in Patients with Noncirrhotic Portal Vein Thrombosis

A recent study (AS Elnaggar et al. Liver Transplantation 2018; 24: 881-87; editorial 868-9) examine the effect of different types of surgical shunts in the setting of portal vein thrombosis (PVT).  The authors examined surgical shunts from 1998-2011 in their institution (senior author: Jean Emond).

  • 40 patients received “portal flow-preserving shunts”: 32 mesoportal and 8 selective splenorenal
  • 24 received portal flow-diverting shunts (16 nonselective splenorenal and 8 mesocaval)
  • Of these 64 patients, only 39 had preoperative and postoperative cross-sectional imaging.   In addition, only 11 patients who had mesoportal shunt (7 children and 4 adults) had preoperative and postoperative cross-sectional imaging allowing for volume comparison.

Key finding:

  • In patients receiving portal flow-preserving shunts (mainly mesoportal), this was associated with liver volume expansion (886 versus 1131 cm to the third), whereas diverting shunts were not.

The authors note that liver atrophy, especially in children, can have a “significant effect on cognitive function and somatic growth.”  Thus, restoring portal flow may improve adverse effects that PVT has caused.

Limitations:

  • Lack of validation of their formula to calculate liver volumes in the pediatric age group
  • Relatively short follow-up:5.7 months in the portal-preserving group and 11 months in the other group
  • Small numbers of patients..

Long-term followup of patients who have needed surgical shunts is needed.  For mesoportal shunts, strictures have been noted in the hepatic end in 15% of patients.

My take: This study shows that portal blood flow, which was interrupted by PVT and restored by mesoportal (Rex) shunt, is important in maintaining liver mass. So, while all shunts may stop upper GI bleeding, mesoportal shunt is likely to improve other adverse effects of PVT.

Virginia Museum of Fine Arts (Richmond) has acquired Chihuly’s Red Reeds

Elastography –Accuracy in Children

Background: Transient elastography may help identify patients at greater risk for advanced fibrosis but there is a significant overlap between fibrosis stages and values with transient elastography. In some conditions, like fatty liver, elastography may be less helpful than in others (eg. chronic viral hepatitis).

A recent study (J Pediatr 2018; 198: 84-9) examined the results of transient elastography in 267 children (97 for calibration, 170 for validation) between 2006-2016.  The median age was 13 years.  Liver diseases included autoimmune (21%), viral (19%), fatty liver (11%), cholestatic (9%), primary sclerosing cholangitis (9%) and post-transplantation (12%).

Key findings:

  • Cut points to discriminate F3-F4 and F4 were >8.6 kPa and >11.5 with 81% and 84% accuracy, respectively in calibration cohort
  • To discriminate F3-F4 and F4 in validation cohort, these cut points, >8.6 kPa and >11.5, had accuracy of 67% and 75% respectively

Figure 2 provides much greater detail in the typical values for each Metavir stage. For example, in the calibration cohort, the median values and range were the following:

  • F0   6.0 (3.2-12.4)
  • F1   6.2 (3.2-75)
  • F2   5.8 (2.5-52.7)
  • F3  10.3 (4.9-32.6)
  • F4  20.5 (5.9-68.1)

In the validation cohort, values were similar:

  • F0   6.2 (3.0-75.0)
  • F1   7.1 (3.3-31.6)
  • F2   7.1 (2.5-52.7)
  • F3  9.6 (4.4-75.0)
  • F4  20.8 (6.8-75.0)

My take: Elastography in measuring liver stiffness is a lot like checking a CRP for inflammatory bowel disease.  That is, it can be helpful but cannot be relied on the same way as many radiographic tests (eg. CT scans for appendicitis).

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