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Supplementary figure 1: The yearly number and type of gut transplantation in patients

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Supplementary figure 1: The yearly number and type of gut transplantation in patients

with CIPO. There has been variable increase in the number of total and modified

multivisceral transplants over the last 10 years of the study period.

(2)

Supplementary figure 2: Cumulative chronic intestinal pseudo-obstruction (CIPO)

patient (A) and overall graft (B) survival according to age

(3)

Supplementary figure 3: Primary allograft survival after gut transplantation for chronic

intestinal pseudo-obstruction (CIPO). A) Liver-contained multivisceral versus liver-free

visceral allografts. B) Modified multivisceral (stomach-pancreas -intestine) versus

intestine-only allograft.

(4)

Supplementary table 1. Intestinal and Multivisceral Retransplantation in patients with Chronic Intestinal Pseudo-Obstruction (CIPO)

Primary Transplant Graft Failure Retransplantation No. Age

(year) Date Allograft Time(month) Cause Date Allograft Outcome 1 23 8/1993 Intestine 5 Acute rejection 11/1993 Multivisceral Dead 2 7 11/1998 Intestine 83 Acute Rejection 4/2005 Multivisceral* Alive 3 7 3/2002 Intestine 15 Chronic rejection 8/2009 Intestine** Alive 4 21 8/2004 Intestine 74 Acute rejection 1/2006 Multivisceral Alive 5 15 10/2004 MMVTx 38 Chronic rejection 12/2008 Multivisceral** Alive 6 19 1/2005 MMVTx 41 Acute rejection 9/2008 Intestine*** Alive 7 24 8/2005 MMVTX 59 Chronic rejection 3/2007 Multivisceral Alive MMVTx :Modified Multivisceral Transplantation * With Kidney ** With colon

*** Partial re-transplant with intestine only allograft

(5)

Supplementary table 2: The response of Clinically Pertinent Questions Representing Most of the QOL Domains in the 9 Adult Chronic Intestinal Pseudo-Obstruction (CIPO) Recipients with Complete Data Before and After Gut Transplantation (GT)

Domain Before GT After GT P value

Constipation/diarrhea 7.5 + 1.5 3.2+ 1.6 <0.0001

Nausea/ vomiting 7.6 + 0.9 2.1 + 0.9 <0.0001

Swallowing food 2.1 + 1.0 2.3 + 1.4 0.87

Weight gain/loss 4.4 + 1.6 3.1 + 1.7 0.01

Wake up from sleep at night 5.1 + 2.5 4.5 + 2.3 0.12

Use medicine to sleep 3.6 + 1.4 4.5 + 2.3 0.042

Mood swings 4.6 + 2.0 3.9 + 1.7 0.01

Nervousness 4.6 + 2.1 3.9 + 1.6 0.03

Headache 3.3 + 2.0 3.6 + 2.4 0.35

Tension/stress 5.0. + 1.9 3.8 + 1.5 0.001

Interest in future 2.8 + 0.9 2.4 + 1.0 0.55

Think about suicide 1.4 + 0.7 1.3 + 0.3 0.87

Forgetfulness 3.2 + 1.8 4.1 + 1.3 0.01

Make daily decision 2.0 + 1.5 2.2 + 1.7 0.34

Ability to solve problems 2.6 + 1.2 3.0 + 1.4 0.06

Fatigue 4.7 + 2.7 4.3 + 2.3 0.62

energy 3.9 + 1.5 3.3 + 1.7 0.04

Joint pain 4.0 + 2.0 5.2 + 1.8 0.001

Involuntary body movements 3.1 + 1.8 3.8 + 1.9 0.01

Loss of balance 3.4 + 1.3 4.1 + 1.5 0.01

Sexual interest 4.6 + 2.4 4.6 + 2.4 0.95

Social interaction 3.1 + 1.3 3.0 + 1.2 0.87

Family/friend support 4.1 + 1.2 3.3 + 1.1 0.005

Leisure activities 5.9 + 2.8 4.1 + 2.0 0.001

Take medicine on time 3.2 + 1.3 3.4 + 1.4 0.79

Technical skills of your doctor 1.7 + 1.5 1.2 + 0.6 0.03

(6)

Supplementary table 3. The Worldwide Published Series of Intestinal and Multivisceral Transplantation in Patients with Chronic Intestinal Pseudo-Obstruction (CIPO)

Number of Patients Patient Surviva

Author Center Year

Child/Adult Total Age (year)

Follow-Up

(year) 1-year 3-yr 1 Sigurdsson et al14 Pittsburgh, PA 1999 8/0 8 1-19 0.2 – 5 75%* NA 2 Masetti et al15 Miami, FL 1999 3/0 3 0.6 - 2.8 0.1 – 1.4 66%* NA 3 Iyer et al16 Omaha, NE 2001 8/0 8 0.7-12.8 1.0 – 6.0 88% NA 4 Bond et al17 Pittsburgh, PA 2004 17/7 24 1 - 47 NA 82%-85% 75%

5 Masetti et al18 Modena, Italy 2004 0/6 6 21 – 37 1.2 – 3.0 83% NA 6 Loinaz et al19 Miami, FL 2005 12/0 12 2.0 – 8 0.7 – 1.5 67% 50%

7 Sauvat et al20 Paris, France 2006 6/0 6 0.5 – 14 0.5 – 10.5 NA 71%

8 Millar et al21 Birmingham, UK 2009 12/0 12 NA NA 61% 50%

9 Lauro et al22 Bologna, Italy 2013 0/11 11 NA 0.2 – 10.6 77% 70%

*Crude Survival, NA, not available

The study patients reported in references 6, 7, and 10 were part of late series reported from the same center in references 9, 11, and 14, respectively.

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