Outcome of “Successful” Biliary Atresia Patients

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.

Related blog posts:

Briefly Noted…Paracentesis, Hyperinsulinemic Hypoglcemia, and Hepatitis E

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.

Related blog posts:

Updated AASLD guidelines and Ascites

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.