Tag Archives: ascites

Liver Shorts: PFIC/FIC1, Best Tenofovir, Ascites Practice Guidance

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DBE van Wessel et al. Hepatology 2021; 74: 892-906. Open Access: Impact of Genotype, Serum Bile Acids, and Surgical Biliary Diversion on Native Liver Survival in FIC1 Deficiency

This huge collaborative study with 130 patients provides a great deal of information about familial intrahepatic cholestasis type 1 (FIC1). Key findings:

  • Survival analysis showed an overall native liver survival (NLS) of 44% at age 18 years. NLS was comparable among FIC1-A, FIC1-B, and FIC1-C (% NLS at age 10 years: 67%, 41%, and 59%, respectively; P = 0.12)
  • The number of predicted protein truncating mutations did not correlate with natural history or prognosis

H Toyoda et al. Hepatology 2021; 74: 656-666. Treatment and Renal Outcomes Up to 96 Weeks After Tenofovir Alafenamide Switch From Tenofovir Disoproxil Fumarate in Routine Practice

In this study, the researchers 834 patients with CHB previously treated with TDF for ≥12 months who were switched to TAF in routine practice at 13 US and Asian center. Key findings:

  • “Overall, we observed continued improvement in virologic response, ALT normalization, and no significant changes in eGFR following switch to TAF from TDF.”
  • HBV DNA suppression increased from 88% to 92% at 48 weeks post-switch, and then 95% at 96 weeks postswitch
  • Improved renal function: “By week 96, 21% (55/267) of patients with CKD stage 2 at switch improved to stage 1 and 35% (30/85) of CKD stage 3-5 patients improved to stage 2 and 1.2% (1/85) to stage 1.”

SW Biggins et al. Hepatology 2021; 74: 1014-1048. Open Access. Diagnosis, Evaluation, and Management of Ascites, Spontaneous Bacterial Peritonitis and Hepatorenal Syndrome: 2021 Practice Guidance by the American Association for the Study of Liver Diseases

This practice guidance (with 276 references) is an update from similar guidelines published in 2012.

Key Points For Children:

  • Children with cirrhosis and ascites should be referred for evaluation for LT
  • Children undergoing LVP should receive 25% albumin infusion of 0.5-1.0 g/kg, or 6-8 g per liter of ascites removed.
  • Diagnostic paracentesis should be performed in children with ascites and fever, abdominal pain, or clinical deterioration. The risks and benefits of this procedure for use in all children with new ascites but without these symptoms have not been defined.
Atlanta Beltline Murals

#NASPGHAN19 Liver Symposium (Part 3)

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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 III – UPDATE ON PORTAL HTN: ASSESSMENT AND MANAGEMENT

What do I do now? The management of portal hypertensive complications: Varices, ascites, and encephalopathy Rene Romero, MD, Children’s Hospital of Atlanta

When there is good function, but the flow is all wrong: Approach to non-cirrhotic portal hypertension Evelyn Hsu, MD, Seattle Children’s Hospital

The role of the interventional radiologist in the treatment of portal HTN: How can I help you?  Jared R. Green, MD, Ann and Robert H. Lurie Children’s Hospital (SLIDES NOT AVAILABLE)

When to consider surgery in the treatment of portal HTN?  Riccardo Superina, MD, FRCS(C), FACS, Northwestern University  (SLIDES NOT AVAILABLE)

Outcome of “Successful” Biliary Atresia Patients

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A recent publication (J Pediatr 2014; 165: 539-546) from the Childhood Liver Disease Research and Education Network (CHiLDREN) provides a strong rationale for close followup of biliary atresia (BA) patients with their native livers.  The Biliary Atresia Study of Infants and Children (BASIC) is one of the ongoing longitudinal studies within CHiLDREN.

Among a cross-sectional study BASIC cohort of 219 children (median age 9.7 years) who survived with their native livers for at least 5 years, they had the following findings:

  • In preceding 12 months, cholangitis occurred in 17%, and 62% had experienced cholangitis at least once following hepatoportoenterostomy (HPE) (also called Kasai procedure.  The authors note wide discrepancy in usage of prophylactic antibiotics; some stop at 2 years following HPE and some never stop antibiotic prophylaxis.
  • In preceding 12 months, bone fractures occurred in 5.5%.  Overall, 15% had had at least one bone fracture at some point, which is higher than the general population. Only 14.6% of entire cohort were receiving vitamin D supplementation.
  • Portal hypertension: clinically detectable splenomegaly, thrombocytopenia, ascites, and variceal hemorrhage were seen in 56%, 43%, 17%, and 9% of patients in this cohort.
  • Health-related quality of life was reported as normal in 53%
  • Mean height and weight z-scores were normal in this cohort.
  • Over 98% had clinical or biochemical evidence of chronic liver disease.

Full-text Link

Bottomline: This BASIC study shows the need for careful followup of “successful” biliary atresia patients and provides more accurate data regarding risks of specific complications.

Briefly noted: J Pediatr 2014; 165: 547-55.  In this study with same first author (Vicky Ng), the investigators develop and validate a pediatric liver transplantation (LT) quality of live instrument for LT patients aged 8-18 years.

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Briefly Noted…Paracentesis, Hyperinsulinemic Hypoglcemia, and Hepatitis E

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Paracentesis is associated with reduced mortality in patients hospitalized with cirrhosis and ascites (Clin Gastroenterol Hepatol 2014: 496-503).  This study relied on a 2009 Nationwide Inpatient Sample database with about 8 million discharges per year.  Of the 17,771 eligible admissions, 61% underwent paracentesis, most (66%) within 1 day of admission.  “In-hospital mortality was reduced by 24% among patients who underwent paracentesis.”  The authors note that some providers may overestimate the risk of bleeding and that many have developed an increased reliance on interventional radiology. While the authors were not certain why this mortality benefit occurred, they note that patients who underwent paracentesis were probably at least as sick as those who did not.  “Patients who underwent paracentesis were actually more likely to have sepsis or acute renal failure, both markers of illness severity.”

Sirolimus helps with hyperinsulinemic hypoglycemia (NEJM 2014; 370: 1131-37).  This brief report showed that sirolimus can be an alternative to subtotal pancreatectomy.  It enabled the authors “to discontinue intravenous infusions of dextrose and glucagon in all four patients and to halt octreotide in three of four.  At 1 year of age, the four patients were continuing to receive sirolimus and were normoglycemic, without any apparent major adverse events.”  Of interest, these findings parallel the findings in adults with insulinoma who were treated with mTOR inhibitors.

Ribavirin treatment for chronic hepatitis E infection in transplant recipients (NEJM 2014; 370: 1111-20).  Retrospective uncontrolled study of 59 patients who had undergone solid-organ transplant had been treated with ribavirin at a mean dose of 600 mg for median of three months.  Median time for HEV infection prior to therapy was nine months. HEV RNA level was undetectable in 46 of 59 patients (78%) after cessation of ribavirin.

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Updated AASLD guidelines and Ascites

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The American Association for the Study of Liver Diseases (AASLD) updates all of its practice guidelines annually (AASLD: Practice Guidelines).  In adults with ascites due to cirrhosis, a recent publication highlights some of the more recent updates (Hepatology 2013; 57: 1651-53).

  • In adults, typically “cirrhosis cures hypertension.”  In addition, a prospective study has shown that propranolol shortens survival in patients with refractory ascites.  So, “consideration should be given to discontinuing beta-blockers or not initiating beta-blockers in those patients with refractory ascites.”
  • “Percutaneous endoscopic gastrostomy is advised against in patients with cirrhosis and ascites.”
  • “A meta-analysis of 17 trials involving 1225 patients..” demonstrates a reduction in mortality with albumin infusion after large-volume paracentesis.  Odds ratio of death with albumin infusion was 0.64.
  • There is an increasing prevalence of bacterial resistance due to widespread use of quinolines to prevent spontaneous bacterial peritonitis (SBP).  “It is prudent to limit prophylactic antibiotics to patients with well-defined criteria for SBP prophylaxis.”
  • A new biomarker (not available in U.S.) assists with the diagnosis of hepatorenal syndrome: urinary neutrophil gelatinase-associated lipocalin.
  • Vaptans are discussed in the update.  The largest trial with cirrhotic patients “demonstrated no clinical benefit in long-term management of ascites” and may increase mortality.