#NASPGHAN19 Liver Symposium (Part 3)

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)

Guidelines: Microscopic Colitis & Vascular Diseases of the Liver

In brief:

AGA Microscopic Colitis Guideline: GC Nguyen et al. Gastroenterol 2016; 150: 242-6. Technical review DS Pardi et al. Gastroenterol 2016; 150: 247-74. Patient guide: pg 275.

EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatology 2016; 64: 179-202.  Topics covered include Budd-Chiari, Portal vein obstruction, Heriditary hemorrhagic telangiectasia, veno-occlusive disease of the liver, management of anticoagulation in liver disease

Two Shorts -Minimal Hepatic Encephalopathy and Fish Oil Protection

Briefly noted: JPGN 2014; 59: 689-94.  In this study, the authors took a consecutive sample of 13  children (ages 4-18 years) with non cirrhotic extrahepatic portal vein portal vein obstruction (EHPVO).  Three tests (fasting ammonia, quantified EEG, a psychometric battery) identified that minimal hepatic encephalopathy (MHE) affects approximately 50% of children with EHPVO.

Also: JPGN 2014; 59: 708-13.  In this study in 7-day old rabbits, those who receive TPN with fish oil-based lipid emulsion had protection against biochemical and liver histologic damage in comparison to rabbits who received TPN with soybean oil. Given the lack of head-to-head randomized studies in infants, this study provides some important evidence of fish oil benefit compared with standardized soybean lipid emulsion.

Related blog posts:

Thrombophilia and Portal Vein Thrombosis

Most cases of portal vein thrombosis (PVT) remain without apparent explanation (JPGN 2012; 55: 599-604).

In this study from Brazil, 32 children with portal vein clots (1990-2011) were reviewed in this cross-sectional study.  The median age at diagnosis was 2.4 years.  Hereditary or acquired thrombophilia was detected in 34% and 9 of these patients had identified risk factors.

The most common thrombophilia factor was the presence of heterozygous state for methylenetetrahydrofolate reductase (MTHFR) C677T which was present in 11 patients.   2 of these patients had a cofactor, including G20210A prothrombin gene, and factor V Leiden.

Risk factors included umbilical catheterization in 13 patients (40.6%).

18 (56%) had no risk factors. 

Related blog entry:

VTE with IBD | gutsandgrowth

Additional references:

  • -Hepatology 2009; 39: 1729.  AASLD practice guidelines on vascular disorders. Recs mesoRex shunt for PVT.
  • -JPGN 2008; 47: 630. n=108. Infrequent thrombophilia d/o with PVT. Banding/sclero -effective.
  • -J Pediatr 2006; 148: 735. PVT more common than previously thought; umbilical vein catheter, esp if misplaced, is risk factor.
  • -NEJM 2011; 365: 147. Review. In cirrhosis pts, platelet count as low as 60K usually sufficient to preserve thrombin generation equivalent to lower limit of normal range in healthy subjects. PVT occurs in 8-25% of pts listed for OLTs. Thus, many procoagulant factors as well as potential propensity for bleeding.
  • -J Pediatr 2003; 142: 197. Rex procedure corrects clotting abnormalities.
  • -J Pediatr 2005; 146: 568. Portal vein -cavernous transformation –associated with cholestasis.
  • -J Pediatr 2000; 136: 805. 49% of children with PV clot had bleeding by 16 yrs & 76% by 24 yrs. (n=44).
  • -Gastroenterol 2001; 120: A-49, #256. Attention span deficits can be corrected c REX.
  • -Hepatology 2000; 31: 345-348 & 587-591. Hyperhomocysteine mutation frequent when PVT assoc c cirrhosis. Etiologic factors. 87% c thrombophilic d/o.
  • -Gastroenterol 2001; 120: 490-497 & 579. Anticoagulation helpful.
  • -J Peds 2006; 149: 275. Reference values.
  • -NEJM 2005; 352: 1791. Thrombosis of cerebral veins & sinuses (review); includes assoc c IBD.  Specific thrombophilia disorders: Antithrombin deficiency, protein S and C deficiencies,Factor V Leiden mutation, (the A for G 20210) prothrombin gene mutation, lupus anticoagulant, antiphospholipid antibodies, factor XI, and MTHFR-hyperhomocysteinemia (thermolabile variant of methylenetetrahydrofolate reductase- C677T MTHFR-). The later was paricularly interesting in view of the recent report of hyperhomocysteinemia in patients with inflammatory bowel disease (Thromb Haemost 1998,80;542-5)