Tag Archives: biliary atresia

Guideline Links: Infant Cholestasis and Esophageal Atresia-Tracheoesophageal fistula

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One way that I use this blog is to use the search function for previous posts with useful links.  For example, I know if I search “foreign” that I will come across a post that has a summary as well as a link to the NASPGHAN recommendations on Foreign Bodies (Foreign Bodies in Children -Expert Guidance).

This post has two links :

Related blog posts:

oneboatcostamaya

 

Newborn Bilirubin Measurements To Identify Biliary Atresia

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It is remarkable how tricky making a diagnosis of biliary atresia can be, even when one has seen the presentation many times.  For parents and many providers, one of the pitfalls includes the inability of recognizing acholic stools. To identify biliary atresia, promotion of stool color cards, over the last two decades, has not been very effective. To address this problem, a recent study (S Harpavat et al. JPGN 2016; 62: 799-803) describes the use of direct or conjugated bilirubin measurements in the newborn period. This study was conducted between 2009-2011.

As with previous studies, 35 infants with biliary atresia all had elevated direct bilirubin. In the non-biliary atresia group, 8936 of 9102 infants had direct bilirubin measurements within the laboratory’s reference range.  Thus, this study suggests that newborn direct bilirubin has a sensitivity 100% and specificity of 98.2%.

In a related publication, the same group published a letter to the editor (NEJM 2016; 2016; 375: 605-6) that describes a prospective two-stage screening of newborns for biliary atresia.  Of 11,636 infants included over a 15-month period, 121 were identified who had direct-bilirubin >95% reference interval in their laboratory.  At a median of 14 days, 11 remained abnormal: 2 had biliary atresia.  Overall, they state the net sensitivity was 100%, then net specificity of 99.9%, and the positive predictive value was 18.2%.

Related blog posts:

Sullivan's Island, SC

Sullivan’s Island, SC

Early Preview of Basic Science Review: Biliatresone

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Generally, one of the best lectures at our national meeting is the “Basic Science Year in Review.”  I would be surprised if a recent study (O Waisbourd-Zinman et al. Hepatology 2016; 64: 880-93) does not get some attention during this review.

Even though biliary atresia (BA) remains the leading cause of pediatric liver transplantation, the exact reasons for its development have not been elucidated.  There are data “implicating both immune dysregulation and genetic factors in human BA.  Toxin-induced BA is not inconsistent with these findings and may represent a primary injury, with immune dysregulation representing a secondary insult.”

One of the reasons for suspecting a toxin dates back to outbreaks of a BA-like disease in newborn lambs that occurred in New South Wales in 1964 and 1988. During both these periods, severe droughts led to pregnant livestock grazing on atypical flora.  Ultimately, a plant toxin termed “biliatresone” was isolated from Australian plants (Dysphania species).

Here’s how this study advanced the science on biliatresone:

  • The authors treated mouse cholangiocytes in 3D spheroid culture and neonatal extrahepatic duct exlplants with biliatresone and compounds that regluate glutathione (GSH)
  • The authors determined the effects of biliatresone on SOX17 levels and the effects of Sox17 knockdown on cholangiocytes in 3D culture

Key findings:

  • “Biliatresoe caused disrupton of cholangiocyte apical polarity and loss of monolayer integrity.”
  • “Neonatal bile duct explants treated with the toxin showed lumen obstruction with increased subepithelial staining for α-smooth muscle actin and collagen, consistent with fibrosis”
  • “Biliatresone caused a rapid and transient decrease in GSH…and caused a significant decrease in cholangiocyte SOX17, and Sox17 knockdown in cholangiocyte spheroids mimicked the effects of biliatresone.”
  • These findings are easiest to appreciate in their figures, particularly Figure 4 and 5.

While pregnant women are not likely exposed to biliatresone, the authors showed that the effects of the toxin on lowering GSH was sufficient for cholangiocyte injury.; in addition, they showed that “SOX17 is required to maintain the epithelial architecture of the gallbladder and the cystic duct.”  Thus, there are likely other exposures that could lead to similar outcomes

My take: I will let Dr. Barnard explain the elegant experiments.  This study strongly supports maternal dietary factors as a contributing role in the pathophysiology of BA.  Now identifying these teratogens is crucial.

Also noted: X Zhao et al. Hepatology 2016; 64: 894-907. This study “strongly support redox stress as a critical contributing factor in biliatresone-induced cholangioctye injury” in Zebrafish.  Specifically, the authors identified that gene transcripts involved in redox stress, particularly regarding glutathione were upregulated after exposure to biliatresone.

Related blog posts:

Glacier Natl Park

Glacier Natl Park

Bad News Bili

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A study (BL Schneider et al. J Pediatr 2016; 170: 211-7) from ChiLDReN (Childhood Liver Disease Research Network) shows that infants with biliary atresia whose total bilirubin (TB) does not drop below 2 mg/dL (34.2 microM) at anytime during the first 3 months after hepatoportoenterostomy (HPE) (Kasai) are at high risk for disease progression.

Key findings:

  • 68/137 (50%) had TB <2.0 at some point following HPE.
  • In the cohort with TB ≥ 2.0, the odds ratio for liver transplantation or death was 16.8.  Higher TB was associated with diminished weight gain, coagulopathy, and hypoalbuminemia
  • In the cohort with TB ≥ 2.0, transplant-free survival at 2 year occurred in only 20% compared with 86% in the TB <2.0 group
  • Interestingly, only 6.6% had variceal bleeding among the entire cohort by age 2 years.

The TB was associated with multiple other parameters of worsening liver function, indicating that TB is not the only measure to affect the decision of liver transplantation.

My take: About half of all patients following a Kasai were at high risk for early progressive liver disease.  TB ≥ 2.0 is a useful indicator of high risk.

Related blog posts:

Walnut Street Bridge, Chattanooga

Walnut Street Bridge, Chattanooga

Helpful Review on Biliary Atresia

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Biliary atresia (BA) remains the leading cause of pediatric liver transplantation and a frequent cause of cholestasis in newborns.  A recent review (AG Feldman, CL Mack. JPGN 2015; 61: 167-75) provides a helpful update. The article begins with a review on pathogenesis, though this remains unknown and continues to be an area of speculation.

The section on evaluation includes a suggested diagnostic algorithm for neonatal cholestasis.  In short, for a 2 week old with jaundice , the authors recommend (STEP 1) fractionating the bilirubin.  The infant is considered cholestasis if the direct bilirubin is ≥1 mg/dL (if total bilirubin is <5 mg/dL) or if direct bilirubin ≥20% of total bilirubin (if total bilirubin is >5 mg/dL).

Among cholestatic infants, the authors recommend (STEP 2) next checking ultrasound and alpha-one antitrypsin (A1AT) (level & phenotype).  The text implies that the authors would check a GGTP.  While this is not in their algorithm, many would suggest checking urine reducing substances, coags, serum glucose, and consideration of sepsis evaluation; these tests can identify issues that are more urgent than identifying biliary atresia.

STEP 3: If U/S and A1AT, are not diagnostic, consider urine culture, urine reducing substances, urine succinylacetone, and additional infectious studies.

STEP 4: Proceed with liver biopsy. If findings of biliary atresia (eg. bile plugs, bile duct proliferation, portal fibrosis), proceed with intraoperative cholangiogram.

Other points:

  • “It is rare for an infant with BA to have a GGTP level <200 U/L.” If low GGTP, consider PFIC, inborn error of bile acid metabolism, and panhypopituitarism.
  • Extensive differential diagnosis table given ((Table 1)
  • “Late diagnosis of BA remains a problem in the United States. The average age of HPE [hepatoportoenterostomy] is 61 days and 44% of patients still undergo HPE after 60 days of life.”  The authors indicate a goal for HPE of taking place  at <45 days of life.
  • Successful HPE can occur even with late diagnosis. 10% to 20% of children who undergo HPE after 100 to 120 days of life still have success in restoring bile flow.”
  • Early/successful HPE is helpful in increasing 10-year transplant-free rate.  Early on, 3 months after HPE, those with a total bilirubin <2 mg/dL compared with those with a total bilirubin of >6 mg/dL have a much lower likelihood of liver transplantation by 2 years of age: 84% vs. 16%.
  • Recommends checking a pulse oximetry at routine followup visits following HPE to look for the possibility of hepatopulmonary syndrome.
  • The article reviews complications including ascites, portal hypertension/GI bleeding, cholangitis, malignancy, and hepatopulmonary syndrome/portopulmonary hypertension.
  • Outcomes: With HPE, “up to two-thirds of patients with BA have short-term clearance of jaundice.” Yet, “80% of patients with biliary atresia will require liver transplantation during childhood.”

Also noted:

“Biliary Atresia is Associated with Hypertension” JPGN 2015; 61: 182-86.

“Pathogenesis of biliary atresia: defining biology to understand clinical phenotypes” A Asai, A Miethke, JA Bezerra. Nat Rev Gastroenterol Hepatol 2015; 12: 343-52.  This review provides in-depth review examines more precise phenotyping, influencing factors (eg. cytomegalovirus), and potential mechanisms.

Related blog posts:

From Mt Washburn, Yellowstone

From Mt Washburn, Yellowstone

Impact of Kasai Portoenterostomy on Liver Transplantation

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Briefly noted: JS Neto, et al. Liver Transpl 2015; 21: 922-7.

This retrospective cohort study of 347 biliary atresia patients who underwent liver transplantation )LT) (1995-2013) divided patients into eraly Kasai failures (K-EF) (27%), late Kasai failures (K-LF) (33%), and no Kasai portenterostomy (No-K) (40%). K-EF was defined by patients who underwent LT before 12 months of age.

Key findings:

  • After adjustment of confounding factors, the K-LF group had an 84% less probability of dying and 55% less chance to undergo retransplantation.
  • Having a K-EF did not have an effect on patient or graft survival compared to No-K.
  • Both the K-LF and K-EF had more post-LT biliary complications.

Bottomline: This retrospective study suggests that if a Kasai portoenterostomy helps postpone LT then this results in improved outcomes; whereas if it is ineffective, it does not impact survival compared to those who did not undergo a Kasai.

Related blog posts:

From Cascade Canyon, Grand Tetons

From Cascade Canyon, Grand Tetons

“What Causes Biliary Atresia?”

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Deceptive Tweet or Great ‘Hook?’

Deceptive Tweet or Great 'Hook?'

The title was tweeted by the AGA and definitely piqued my interest.  The link that was provided connects to a full-text article (Mack C, CMGH 2015; 1: 267-74) on the potential immunopathogeneis of biliary atresia in the neonatal period.  While the article provides a lot of information, it really does not come close to answering the title question.  In my view, this article reminds me of many other articles on other enigmatic liver diseases in which the clues on pathogenesis led in the wrong direction (eg. antioxidants for gestational alloimmune liver disease)

Here’s from the abstract and the summary:

From abstract:

The neonatal presentation of BA prompts the question of what potential modifications of unique aspects of the neonatal immune system set the stage for the progressive biliary disease. This review also discusses the characteristics of neonatal immune response and the theories on how alterations of this response could contribute to the pathogenesis of BA. These include aberrant type 1 helper T-cell (TH1) and TH17 responses, deficiencies in regulatory T cells, activation of humoral immunity, and autoimmunity. To advance our understanding of the etiology of BA, future studies should focus on the unique aspects of the neonatal immune system that have gone awry.

From summary:

Biliary atresia is a devastating disease wherein the vast majority of patients require liver transplantation for survival. It is critical to grasp the immunopathogenesis of BA in order to provide future therapies that control the intrahepatic biliary inflammation and prevent subsequent fibrosis. Evidence exists for a key role of both arms of the adaptive immune response in bile duct injury. The neonatal presentation of BA provides a clue to disease pathogenesis. Early events that impact the neonatal immune system (ie, perinatal virus infection) may alter the immune response and promote a progressive inflammatory or biliary autoimmune disease. To advance our understanding of the etiology of BA, future studies should focus on those unique aspects of the neonatal immune system that have gone awry, as detailed throughout this review.

Bottomline: Clues are important but do not answer the question of what causes biliary atresia.

Related blog posts:

N2U Part 5 -Biliary Atresia and Kwashiokor

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2015 N2U Syllabus & Presentations

Growth Failure, Macro- and Micronutrients, and Biliary Atresia  James Heubi   (Syllabus pg 62 –68)

Case in Point:  AK is a 5-month-old Hispanic male with biliary atresia s/p hepatoportoenterostomy at age 6 weeks who was seen in clinic with a 2 month history of poor nutritional intake (full details on syllabus pg 62).

Initial Focus/Management:

  • Increasing caloric density 24 cal with MCT-containing formula, then up to 30 cal (avoid monotherapy with portagen due to EFA deficiency); if not effective, then nocturnal tube feedings (NG/NJ) are likely to be needed. Parenteral supplementation –not being used at Cincinnati in pretransplant patients.
  • Ascites, when present, limits fluid volume intakes. Aldactone (often at about 3 mg/kg/day) can be helpful; check to make sure urine sodium indicates some natriuresis.
  • Fat soluble vitamin supplementation/micronutrient supplementation (when needed). Check levels at about 3 months of age. If Vitamin D (25-OH) is greater than 20 (in pediatrics), this is probably reasonable in this population. With Alagille/hyperlipidemic patients, need to correct vitamin E for cholesterol (or total lipid) (Sokol RJ, Heubi JE, et al. NEJM 1984; 310: 1209-12).
  • For biliary atresia, direct bilirubin >2 indicates need for fat soluble monitoring; in other cholestatic conditions (eg. Alagille, PFIC), don’t rely on direct bilirubin as fat soluble deficiency can develop with lower direct bilirubins.
  • Vitamin D supplementation: 1000 units/kg/day Drisdol D3 (expensive). D3 preferred but D2 usually OK. Monitor levels and increase dosing if needed. Check monthly until adequate level. Alternatives: Bio-D-Mulsion Forte (D3) http://www.bioticsresearch.com/node/1570, Nature’s Blend Ultra Strength (D3). http://www.nationalvitamin.com
  • Vitamin E supplementation: Liqui-E (w TPGS) or Nutr-E-sol 15-25 IU/kg/day. Alternative: Aqua-E
  • Vitamin A supplementation/monitoring: AquaADEKs is reasonable supplement. Harder to monitor vitamin A levels.
  • If failed Kasai, likely headed to transplantation fairly quickly.

Kwashiokor –Rob Shulman (Syllabus pgs 21-33)

Case in point: 15 mo –Fed a diet of coconut and rice milks managed by pediatrician and chiropractor.  (This can occur with BRAT diets as well.)

Key points:

  • Terminology: from language spoken in a region of Ghana. Term developed to describe the sickness a baby gets when the new baby comes. This is a result of child who gets displaced from breastfeeding as the result of a sibling being born. Willaims CD. Lancet 1935; 226: 1151-52. Original description
  • Etiology: protein deficiency, protein quality, infection (‘pushes them over the edge’)
  • Microbiome in Kwashiokor References: Tilg et al. Nature Rev. 2013;10:261-262.  Smith et al. Science. 2013;339:548-554. Initial study showed discrepant microbiome in identical twins with and without Kwashiokor. Followup study by placing stool (from Kwashiokor and from healthy children) in mice. Stool from twin with Kwashiokor resulted in mice malnutrition (Garrett W. NEJM 2013; 368: 1746-47). (Related blogs:Gut microbiomes of Malawian twin pairs discordant for … and Microbiome and the risk of Kwashiokor | gutsandgrowth )

Feeding plan/ Prevention of Refeeding Syndrome:

  • Check labs –including protime, zinc, phosphorus, potassium, magnesium
  • Oral feedings with standard formulas/diet (usually). Limit feeding volumes initially for 1st week Kwashiokor (page 37 in syllabus) –about ½ full caloric intake (consider ½ strength formula).
  • ‘Advancing diet slowly is not needed with other forms of malnutrition.’
  • Give multivitamin. In 3rd world, it is recommended to add additional vitamin A (200,000 units once).
  • Hold off on iron (even in multivitamin) until improved for a few weeks.
  • Albumin infusions are not recommended àassociated with worse outcomes
  • Refeeding syndrome is an iatrogenic disease! This is associated with Kwashiokor and not with other malnutrition diseases.
  • Add Kphos to feedings (eg Neutraphos, NeutraphosK). Usually drop in phosphorus drop most likely in first 48 hours –monitor carefully in first few days and again during increments in feeding.
  • In 3rd World countries, addition of antibiotics (amoxicillin or cefdinir for 7 d) to therapeutic regimens for uncomplicated severe acute malnutrition associated with a significant improvement in recovery and mortality rates. In U.S. this translates to low threshold for using antibiotics but not required in every case.

Disclaimer: This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

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.

Subway Art (Chicago)

Subway Mosaic Art (Chicago)

NASPGHAN Postgraduate Course 2014 -Liver Module

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This blog entry has abbreviated/summarized the presentations. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.  I’ve attached the course syllabus as well:

PG Course Syllabus – FINAL

Primary Sclerosing Cholangitis –Dennis Black (Le Bonheur Children’s Hospital)

  • Up-to-date review provided
  • GWAS (genome-wide association study) identified 16 significant risk loci which account for only 7.3% of overall risk; environmental influences need to be worked out
  • Pediatric studies –total of 328 patients reported to date

Is pediatric disease the same disease as in adults?

  • Incidence in pediatrics: 0.23/100,000 incidence vs 1.1/100,000 in adults
  • Mean age at diagnosis 13 years in pediatrics.
  • 30% of pediatric patients have overlap with autoimmune hepatitis which is higher than in adult patients.

Other pointers:

  •  Discussed “Autoimmune cholangitis.” Imaging needed in autoimmune hepatitis to look for primary sclerosing cholangitis.
  • IBD Association with PSC: IBD occurs in about 55% of PSC patients. If PSC diagnosed first, usually with right-sided colitis.  If IBD diagnosed first, than pancolitis is more commonly noted.
  • Add IgG4 as part of workup to rule out IgG4 cholangiopathy (sensitive to immunosuppression).

Treatment:

  • Supportive care for cholestasis (vitamins, pruritus management, etc
  • Monitoring for complications (rare cases of cholangiocarcinoma in pediatric population).  14 drugs tested to date –mainly in adults.  “All without proven positive impact on long-term outcome.”
  • Ursodeoxycholic acid –widely used but controversial because higher doses associated with worsened outcomes in adult study (Lindor et al).  Ongoing study in pediatric population with ursodeoxycholic acid.
  • Vancomycin (Aliment Pharm 37: 2013; 604.  Adults n=35). Both Flagyl and Vanc seemed to be helpful. Uncontrolled pediatric studies with vancomycin reviewed. Vancomycin study in the works for pediatric/adults.
  • No prospective randomized controlled trials in children and very little data in adults. Hard endpoints –very difficult in children/not practical in children (eg. portal hypertension, transplant, death).

PSC and Transplantation: PSC 2.6% of total transplants –long-term outcome is similar.

Related Blog Posts:

The Jaundiced Infant –Saul Karpen (Emory)

  •  “We don’t estimate jaundice very well… Our eyes do an awful job.”
  • Breastmilk Jaundice: Archives of Disease in Childhood 1978; 53: 506-16.  Only 12 of 853 had jaundice beyond 3 weeks of life.
  • Cholestasis. One of the best studies looking at etiology was recently published:  Hoerning A, et al Front Pediatr. 2014; 2: 65. N=82.  Only 1 patient had CMV.  41% had biliary atresia.

Biliary atresia (BA):

  • Reviewed study indicating that liver biopsy was most accurate means of making diagnosis of biliary atresia (blog comment: this study result may not be accurate in all settings as the interpretation relies on the ability/reliability of pathologist).  High utility of stool pigment & ultrasound (including flow).
  • In retrospective study (Pediatrics 2011; 128 e1428-33), all the BA patients had elevated direct bilirubin by 24-48 hrs of life.
  • Genetic panels and whole exome sequencing (~$4-7K) are happening now. Cost-effective.

Take-home message: Molecular understanding possible for conjugated/unconjugated hyperbilirubinemias. Direct bilirubin >1 is abnormal

Related blog posts:

Acute Liver Failure –Estella Alonso (Children’s Hospital of Chicago) (pg 43)

Points:

  • Few patients receive a full diagnostic workup (J Pediatr 2009;155:801‐6)–especially with regard to metabolic and autoimmune disorders.
  • Reviewed etiologies –most frequently “indeterminant” especially in younger patients.  Acetaminophen is most frequent etiology in teenagers and adults.
  • Systemic inflammation is common in acute liver failure (Bucuvalas, J JPGN 2013;56: 311–315). Soluble IL2 receptor alpha –significantly higher in patients that died.  Immune regulation important aspect regarding survival. Should steroids be used in cases with high inflammation?

Prognosis: Squires et al. J Pediatr 2006;148:652-8, Lee et al. JPGN 2005;40:575-81, Baliga et al. Liver Transpl 2004;10:1364-71

  • 33% ‐53% survival with native liver
  • 61% survival including LT
  • 70%‐80% after LT
  • Multiorgan failure is most common etiology of death. Bleeding is “a rare cause of mortality.”

Management:

  • Reviewed including coagulopathy/bleeding, cardiovascular collapse, hepatic encephalopathy/cerebral edema
  • Pediatric N-acetylcysteine Trial Squires, et al Hepatology 2013;57:1542‐9 N=182.  Patients with NAC seemed to do worse, but not statistically proven.  This study has stopped the widespread use of NAC in acute liver failure.
  • Discussed approach to neurological complications in ALF. Hussain et al, JPGN 2014;58:449‐56. Retrospective study (n=18). Early EEGs obtained. Hypertonic saline may be more effective than mannitol.  Hypothermia may be helpful adjunct.
  • Timing of Transplantation discussed (pg 54 in syllabus). Difficult to predict spontaneous survival.

Related blog posts:

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.

This Year's Pumpkin

This Year’s Pumpkin

Clinical Science Year in Review in Pediatric GI – NASPGHAN 2014

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For many participants at NASPGHAN, the “year in review” presentations are a highlight.  This year was no exception.

This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

William Balistreri –Clinical Science Year in Review 

Lay press remains excellent source of information.

Benefit of microbiome. (from NPR) Now there is elephant poop coffee -$645/lb ($70/cup).  Link: No. 1 Most Expensive Coffee Comes From Elephant’s No. 2 : The ... Collecting elephant poop is probably a less ideal job than what most of us have.  As for coffee, “make mine de-crap.”

Elephant Microbiome Collector

Topic of the year: Hepatitis C

  • 25 years since identification of Hepatitis C in 1989
  • Now approaching cure (Related blog post: Wiping out Hepatitis C | gutsandgrowth). All-oral highly effective regimen –newest regimen as easy as one pill per day for 8-12 weeks. Direct-acting antivirals (DAAs). Moving past 1st generation of DAAs: telaprevir/boceprevir with interferon/ribavirin.(refs = Pawlotsky, Gastroenterology 146:1176, 2014 and Schmidt, Clinical Gastroent Hepatol 12:728, 2014)
  • New drugs for HCV –just in time –increasing risk of HCV complications. Ann Intern Med 2014; 160: 293.
  • Goal –SVR –sustained virological response
  • Reviewed large number of articles: Sofosbuvir, Simeprevir, Sofosbuvir/Ledipasvir (Harvoni).  3-D regimen: ABT-450, ABT-267, ABT-333 –will be approved in coming weeks (Related blog post:Have You Heard of Harvoni? | gutsandgrowth)
    • Gane, NEJM 368:34, 2013
    • Zeuzem, NEJM 370:1993,2014
    • Kowdley, N Engl J Med 370:1879, 2014
    • Lawitz, Lancet 383:515, 2014
    • Feld, New England Journal of Medicine, 370:1594, 2014
  • Mild side effects with newer drug therapies
  • Awaiting pediatric studies.
  • Costly $1000/pill –“if dog swallows it,” may have to look for it in the stool
  • Stay updated with recommendations: www.hcvguidelines.org  (AASLD/IDSA)

Hepatitis B –success of vaccination.

  • Preventing perinatal transmission with HBIG/vaccine. JAMA 2013; 310: 974. Those born after 1984, with much lower HCC. Ann Intern Med 2014; 160: 828; Hepatology 2014; 60: 448
  • Give antivirals (eg. telbivudine) for HBeAg-positive mothers prior to delivery. (Related blog post: Hepatology Update -Summer 2014 | gutsandgrowth) Greenup, Journ of Hepatology 61:502, 2014 AND Zhang, Hepatology 60:468, 2014
  • Antiviral therapy lowers the risk of HCC. Hepatology 2014; 147: 143 (Wu et al).
  • Make sure children with IBD are being screened for hepatitis B. ~13% may not be immune. Moses, Am J Gastro 107:133, 2012

Trend of the Year: Social Media

  • Genome sequencing –tremendous advance. Families may push for this option on their own.
  • Magnets –banned. Social media allowed this problem to be quickly identified. (Related blog post: Buckyball Recall –It’s Official | gutsandgrowth)
  • Social media allows family to share information and get answers. Internet blogging allows families to reach out to scientists.
    • Schumacher, Pediatrics 133:e1345, 2014
    • Enns, Genetics in Medicine, March 2014
  • BiliCam –can take picture with mobile phones.

Biliary Atresia

Threat of the Year: Obesity along with NAFLD

  •  NAFLD can have significant liver histologic abnormalities even with normal ALT levels. J Pediatr 2014; 164: 707.
  • Clinical burden of NAFLD is not restricted to liver-related morbidity or mortality Armstrong, HEPATOLOGY 59:1174, 2014. Also, concern for obstructive sleep apnea and cardiovascular disease.  Sundaram, J Pediatr 164:699, 2014. Pacifico, HEPATOLOGY 59:461, 2014
  • Elastography is promising tool. Xanthakos, J Peds 164:186, 2014
  • Current treatment –lifestyle changes. Snacking contributes to fatty liver. Sleep curtailment is associated with obesity. Spaeth. SLEEP 36:981, 2013, Taveras, Pediatrics 133:1013, 2014, Mitchell, Pediat 131:e1428, 2013
  • Increased antibiotics in early life associated with obesity due to alteration of microbiome. Bailey, JAMA Pediatrics, Sept 29, 2014
  • Suggestion for future: “Diet Water.”

Diet Water.jpg

For those who want to learn more from Dr. Balistreri directly, I would recommend the Aspen Conference:

Aspen Meeting

Related link: Dr. Balistreri’s Review of the Growth and Development of the Pediatric Gastroenterology Specialty.