#NASPGHAN19 Liver Symposium Notes (Part 1)

Although I was unable to attend this year’s liver symposium at NASPGHAN19, I reviewed the lecture notes.  There is some terrific content.  Here are some of the slides (borrowed with permission from NASPGHAN).

Link to complete NASPGHAN Chronic Liver Disease Symposium 2019

Session I

How do I best evaluate a cholestatic infant? Sanjiv Harpavat MD Texas Children’s Hospital 

Related blog post: What is the evidence that biliary atresia starts in utero?

As for this algorithm, in my opinion, the 1st step needs to be to exclude emergencies associated with infantile cholestasis: coagulopathy, hypoglycemia, sepsis, and checking urine for reducing substances (cow’s milk formula can worsen liver disease if galactosemia is present). Subsequently, evaluation needs to proceed quickly to determine the etiology.

How do I interpret genetic results?  Saul J. Karpen MD, PhD, Emory University School of Medicine/Children’s Healthcare of Atlanta

What do abnormal liver enzyme levels mean in a tween?  William F. Balistreri MD, Cincinnati Children’s Hospital Medical Center

What do I do with this abnormal radiology finding? Jean Molleston MD, Riley Children’s Hospital

I have not selected slides from Dr. Molleston’s handout –the images are terrific.  For most of the problems that are presented, the lecture notes do not provide specific recommendations for management.

Disclaimer: NASPGHAN/gutsandgrowth assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. The discussion, views, and recommendations as to medical procedures, choice of drugs and drug dosages herein are the sole responsibility of the authors. Because of rapid advances in the medical sciences, the Society cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure. Some of the slides reproduced in this syllabus contain animation in the power point version. This cannot be seen in the printed version.

 

 

NASPGHAN/ESPGHAN Position Paper: Nutrition Support for Children with Chronic Liver Disease

M Mouzaki et al. JPGN 2019; 69: 498-511. Full text link: Nutrition Support of Children with Chronic Liver Diseases: A Joint Position Paper of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition 

Figure 2 (above) outlines an approach to assuring adequate intake of nutrients

Table 2 (above) provides an approach to laboratory monitoring. The authors recommend measuring every 3-6 months for most of the vitamins and micronutrients listed in this table

Table 3 (above) provides recommendations for specific elements of nutritional support.

Figure 1 (above) describes pathophysiology of malnutrition.

My take: This position paper provides useful advice for approaching nutritional support in children with chronic liver disease. Defining the specific patients in which these guidelines may be applicable requires individual assessment.  Thus, the authors note that the guidelines “should not be construed as establishing a legal standard of care or as encouraging, advocating, requiring, or discouraging any particular treatment.”

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

Distance from Transplant Center -Not a Good Thing for Chronic Liver Disease

It is said that “absence makes the heart grow fonder.”  This expression certainly cannot be extrapolated to the liver.  A recent study (DS Goldberg et al. Clin Gastroenterol Hepatol 2017; 15: 958-60) showed that increased distance to a liver transplant center was associated with higher mortality for patients with chronic liver failure (CLF).

This study examined 16,824 patients with CLF.  In the cohort (879, 5.2%) who lived  >150 miles from the closest LT center there was a 20% higher mortality rate (Hazard ratio of 1.20; P <.001).  According to the authors, mortality with distance “modeled as a continuous variable per unit increase in 50 miles.”

From the discussion:

  • “For patients with CLF, transplant remains the only option for long-term survival. Yet for the 11 out of 12 who are never transplanted, access to specialized care may still prolong life.”
  • Limitations: This study could not account for socioeconomic factors or control for geographical variation in care.  With regard to the later, death rates from liver disease are lowest in New York, where the entire population is within 150 miles of a transplant center.  In contrast, in New Mexico and Wyoming, which have the highest age-adjusted death rates, more than 95% of patients live >150 miles from a transplant center. However, there may be many other differences in care besides distance in these regions.

My take: This study, though with some limitations, bolsters the view that patients with chronic liver disease (and probably other chronic diseases) live longer if in proximity to specialized care.

Related blog posts:

Exquisite windows in St. Vitus Cathedral, Prague

 

Portopulmonary Hypertension -A Little More Data

A recent study (E Echocahrd-Dugelay et al. JPGN 2015; 61: 346-54, editorial 268-9) provides a little more data on the rare condition of portopulmonary hypertension (POPH). Untreated POPH can lead to right-sided heart failure and death; a prompt diagnosis improves the chance for responding to treatment.

In brief, the authors reviewed their experience with 14 patients that were diagnosed with POPH between 1983-2009. The authors also reviewed the literature for a total of 98 patients.

Findings:

  • 0.5% of children with portal hypertension had POPH
  • 0.9% of children with end-stage liver disease awaiting liver transplantation had POPH
  • Congenital portosystemic shunts (CPSS) appeared to be a risk factor for POPH and were noted in 3 of their 14 cases as well as 22 of 98 cases overall.
  • In treated patients (n=42), five-year survival was noted to be 80%. Treatment included vasodilator therapy, closure of CPSS, or liver transplantation.
  • Hepatopulmonary syndrome (with hypoxemia) may precede POPH; this was reported in 6 of the 98 patients in this report

Dr. Ronald Sokol’s commentary noted that guidelines for timing/frequency of pulse oximetry testing and formal echocardiographic screening are needed but “challenging given the present body of evidence.”  He recommends screening all pediatric liver transplantation candidates who have cirrhosis and portal hypertension with pulse oximetry and echocardiography as well as those with clinical features of POPH (eg. syncope, shortness of breath, dyspnea).  For other patient populations, it remains unclear.

Bottomline (from the authors): “Detection of POPH at an early stage requires systematic screening at regular intervals by echocardiography in children with all causes of portal hypertension.” Unanswered questions:

  • how much portal hypertension is needed to merit screening?
  • how often should screening take place?

Related blog posts:

 

 

Good News for Starbucks & Coffee Vendors

This blog has posted a number of favorable reports on coffee, even though I’m not a coffee enthusiast.  In general, coffee has favorable health effects when it is not paired with alcohol or tobacco.

A recent coffee study (Gastroenterol 2015; 148: 118-25) shows an association between coffee intake and reduced incidence of liver cancer and death from chronic liver disease in the U.S.

Here’s a link to a summary of the article: GastroHepNews Coffee and Liver Disease

  • During an 18-year follow-up period, there were 451 incident cases of hepatocellular carcinoma and 654 deaths from chronic liver disease.
  • Compared with non-coffee drinkers, the researchers noted that those who drank 2–3 cups per day had a 38% reduction in risk for hepatocellular carcinoma.
  • Those who drank ≥4 cups per day had a 41% reduction in hepatocellular carcinoma risk.
  • Compared with non-coffee drinkers, participants who consumed 2–3 cups coffee per day had a 46% reduction in risk of death from chronic liver disease, and those who drank ≥4 cups per day had a 71% reduction.
  • The inverse associations were similar regardless of the participants’ ethnicity, sex, body mass index, smoking status, alcohol intake, or diabetes status.

Related blog posts:

More liver-related news: Man with infected hepatitis C sentenced to 3 years for spitting in officer’s face (from The Republic/AP News)

Does Anyone Know Why This Toilet is in our Parking Garage?

Does Anyone Know Why This Toilet is in our Office Parking Garage?

Sarcopenia, fatigue, and nutrition in chronic liver disease

Several articles from a recent Clinical Gastroenterology and Hepatology have addressed nutritional aspects of chronic liver disease.

1. Sarcopenia?  This term refers to generalized loss of skeletal muscle.  It does not equate to malnutrition though there is significant overlap.  (Clinical Gastroenterology and Hepatology 2012; 10: 166-73 & editorial 100).  In this study, 112 adults with cirrhosis had CT scans which examined skeletal muscle at the L3 level; 40% had sarcopenia. Sarcopenia was independently associated with mortality and was not reflected in MELD score.  Patients had increased risk of death from sepsis and liver failure (HR 2.18).  Thus, sarcopenia joins hyponatremia, refractory ascites, hepatic encephalopathy as additional factors which add prognostic information to MELD score.

2. Fatigue in cirrhosis. (Clinical Gastroenterology and Hepatology 2012; 10: 174-81 & editorial 103).  Fatigue is common in cirrhosis and is multifactorial.  In this prospective study, 108 patients were evaluated with a fatigue impact scale. Fatigue improved after liver transplantation. Fatigue can be peripheral due to muscle weakness and dysfunction. And, fatigue can be central due to difficulty performing physical and mental activities.  Central fatigue is associated with an increased perceived effort for tasks and often related to depression; this type of fatigue is much more common with cirrhosis.  Although improved, fatigue often does not completely resolve with liver transplantation.

3. Nutrition recommendations. (Clinical Gastroenterology and Hepatology 2012; 10: 117-25).  A summary of nutrition recommendations in adults  with chronic liver disease is given in this article.  One common misconception is protein restriction.  This is not beneficial.  Protein recommendations are for adult patients with cirrhosis to receive 1-1.5 g/kg/day.  This amount is higher than for healthy individuals.  Protein restriction leads to protein catabolism, muscle breakdown and increases the likelihood of hepatic encephalopathy.

Additional references:

  • -Age Ageing 2010; 39: 412-23.  Sarcopenia consensus definitions in older people.
  • -Gastroenterology 2008; 134: 1741. Evaluation and management of end-stage liver disease in children. Recs vaccines due to functional asplenia/portal hypertension at age 2 for Neisseria (MCV4) or polysaccharide (MPSV4); at 6 weeks of age for pneumococcal conjugate vaccines. Reviews nutrition, varices, ascites, encephalopathy….
  • -Liver Transplant 2008; 14: 585-591. Poor growth often due to growth hormone resistance. Chronic malnutrition is a factor, but children with advanced liver dz may not grow despite adequate calories. Recs: for chronic liver dz: 130-150% of RDA based on ideal body wt; in infants 120-150 cal/kg/day. Increase MCT either thru formula or supplemental MCT.
  • -Liver Transplant 2006; 12: 1310. Review article on nutrition for OLTx patient.
  • -JPGN 2000; 30: 361. nutrition review and chronic liver disease.