2011 - ISBTS 2011 Symposium


Oral Communications 3: Nutrition Outcomes

5.124 - Diagnosis of portal hypertension in children with intestinal failure associated liver disease. (IFALD) referred for intestinal transplantation (ITx)

Presenter: Girish, Gupte, Birmingham, United Kingdom
Authors: Girish Gupte1, Rohit Gowda1, Simon Olliff2, Kamarjit Mangat2, Chetan Mehta3, Patrick McKiernan1


124
Diagnosis of portal hypertension in children with intestinal failure associated liver disease. (IFALD) referred for intestinal transplantation (ITx)

Girish Gupte1, Rohit Gowda1, Simon Olliff2, Kamarjit Mangat2, Chetan Mehta3, Patrick McKiernan1

1Liver unit (including small bowel transplantation), Birmingham Children's Hospital, Birmingham, United Kingdom; 2Department of Radiology, Queen Elizabeth Hospital, Birmingham, United Kingdom; 3Department of Cardiology, Birmingham Children's Hospital, Birmingham, United Kingdom

A careful assessment of the severity of IFALD and portal hypertension (PHT) is important to guide therapy about inclusion of liver in intestinal transplant (ITx). Hepatic venous wedge pressure gradient (HVWPG) and splenic pulp pressure (SPP) are objective measurements  for  the severity of PHT. We audited the use of HVPG and SPP in assessment of children with IFALD assessed for ITx.

Methods: Retrospective audit of all patients who had HVPG/SPP measured between 1990- 2010 was undertaken. HVWPG or SPP (measured when HVWPG  was not possible) and laboratory parameters (bilirubin, liver biopsy, endoscopy for varices).were reviewed.

Results: HVPG/SPP measurements were performed in 18 patients (age 9months - 15 years) . Children were grouped into two categories- Group 1  (Significant Portal HT= HVWP>10and SPP>15) ad Group 2- (Non significant portal HT= HVWP<10,SPP <15).

 

Group1

N=8

Group 2

N=10

Median age (in months)

11

44

Bilirubin (μmol/l)

81

20

Spleen size on USS

12 cms

(8.9-14.8)

9.3 cms

(8.7-21.7)

Endoscopy

Varices absent -4,

present -2 (grade1)

.Not done-1

Varices – absent 10

Not done -1

Liver biopsy

Not done-1.

Mild – mod-1

Mod-severe-5

Absent fibrosis -1

Mild-mod-2

Mod-severe – 3

Severe -2

Not done-2

Not transplanted

1

2

Transplant and Type of Tx

Isolated liver-1

Isolated SB-1

Liver small bowel-2.

Listed for LSB-1

Not tx-1

ISB-1

LSB-2

Listed for LSB-1

MMV-3

Not Tx-2

Summary: The conventional methods of assessment of IFALD and portal hypertension (bilirubin, endoscopy and Liver biopsy) as shown in Table 1  can  misinterpret the severity of IFALD .

Conclusions:  HVWPG /SPP (objective meassurements) may be more reliable in establishing the presence of PHT and guide decision about liver inclusion graft in ITx recipients.


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