Dott.ssa Maria Cappello

UOC Gastroenterologia ed Epatologia Azienda Ospedaliera

Universitaria Policlinico Palermo

Commissione Nutrizione ed Alcologia

Update sul management della sindrome da intestino corto

AcaseofSBS•  A.G., female, 54 years •  Short bowel syndrome type 2 (extensive digiunoileal

resection in July 2016 because of intestinal necrosis complication of volvulus occurred after Hartman procedure for diverticular disease; ileostomy closed on 11/2017); residual short bowel less than 150 cm

•  Colostomy •  Home parenteral nutrition (daily until february 2018;

then every other day) plus liquid diet •  Recurrent septic complication of CVCs (in 2 years

10 PICC, 2 Groshong catheters removed because of sepsis)

AcaseofSBS•  A.G., female, 54 years •  On admission in our gastroenterology nutrition clinic (jan 2018) : body

weight 36.7 kg; height 154 cm; BMI 15,4 •  Diarrhoea (10 colostomy bags every day; liquid stools). Sarcopenic.

On wheelchair because of oligonutrients and vitamines deficiencies. •  Adds ONS and liquid diet; MCT oil, multivitamin preparations •  Supportive therapy: loperamide, PPI •  A new PICC inserted and monitored by hospital PICC team. •  Screening colonoscopy did not show polyps or any proliferative

lesions. •  Starts teduglutide 0,05 mg /kg sc daily on July 2018 (compassionate

programme)

AcaseofSBS•  A.G., female, 54 years •  Home assistance by company PSP programme: monitoring of water

balance, body weight, CVC functioning •  On October 2018: BMI 18,97; body weight 45,2. Good performance

status, autonomous in daily activities •  On March 2019: still on teduglutide; body weight 47 Kg; BMI 19,82;

HPN three times a week. Stool consistency increased, stool frequency decreased (3 colostomy bags /day)

•  Side-effects: occasional bloating, treated with probiotics. An episode of cholecystitis in August (temporary withdrawal of teduglutide, ursodesossycolic acid added)

•  End-point: weaning from HPN •  This is the first report of a patient with short bowel syndrome treated

with teduglutide in Sicily

The rarest and less known organ failure: estimated prevalence 5-20 per million CIF due to benign disease has been included in the 2013 Orphanet list of rare diseases

Short bowel syndrome : classification

«A small bowel lenght less than 200 cm»

End-jejunostomy Jejuno-colicanastomosis

Jejuno-ilealanastomosisIleo-caecalvalveandcolonincontinuity

Short bowel syndrome: etiology

•  Mesenteric ischemia •  Crohn’s disease •  Radiation enteritis •  Post-surgical intraabdominal

adhesions •  Post-operative complications

Multidisciplinary Expert Team

Chirurgo

Radiologo Patologo

Gastroenterologo Nephrologist

Nutrizionista Psicologo

InfermiereADI

Assistentesociale

Caregiver

Short bowel Syndrome: Not only the gut!

Short bowel syndrome: clinical course •  Phase I: acute stage •  Phase II: adaptation stage •  Phase III: maintenance

•  3-4 weeks (hospital) •  1-2 years (HPN) •  Lifelong: special diet or •  HPN

Reversible condition in 50% of adults and 70% of children. In adults the probability of CIF reversibility is higher when: 1)  there is more than 35 cm SB with a jejuno-ileal anastomosis, the ileo-cecal valve and an intact colon or 2) more than 60 cm SB with a jejuno-colic anastomosis or 3) more than 115 cm SB with an end-jejunostomy, provided that the remaining bowel is healthy. Plasmatic citrullin > 20 μmol/l is a predictor of reversibility.

Therapy of short bowel syndrome

•  Antidiarrhoics:loperamide,PPI,octreotide,cholestyramine

•  Dietpoorinlipidsandoxalates•  Homeparenteralnutrition(HPN)•  Growthhormone•  GL-P2

Prevalence of intestinal failure and short bowel syndrome (based on HPN data)

In Europe: •  Pediatric cases: 2-6,8/million

inhabitants

•  Adults: 5 – 20/million inhabitants

In Italy prevalence* is increasing: 1,7/million 1994 33/million 2017 estimated on HPN data

Short bowel: need of increased awareness

•  5-year outcomes are improving:

•  36% are weaned from TPN

•  39% still on TPN

Overall survival 65% Crohn’s disease patients have better prognosis

•  Cost of illness high •  High rate of

hospitalizations (40% for underlying disease, 30% for HPN complications)

•  Strict monitoring •  Hematochemistry every 1-3

months •  Vitamin and trace elements

every 6 months •  US every year •  DEXA every year •  Liver biopsy

Catheter related complications •  Catheter-related infections (0,14 – 0,83 episodes/patient –year) •  Catheter occlusion (0,07 episodes/year) •  Central vein thrombosis (0,01-0,03 episodes/year) •  Catheter malfunction Metabolic complications •  Nephrolitiasis •  Liver disease (IFALD) 19 – 75% •  Osteopathy Low quality of life

GLP-2 is a peptide produced by enterocytes after food ingestion. Teduglutide is a dipeptidyil-peptidase degradation resistant GLP-2 analogue

TEDUGLUTIDE as a novel therapeutic agent for SBS

TEDUGLUTIDE:mechanismofaction

Responders were 63% in the teduglutide group and 30% in the placebo group, p=0.002

STEPS-2study

STEPS-2study

A retrospective real life analysis from a tertiary referral center All patients exposed to teduglutide From 2009 to 2015 11 patients were totally weaned From parenteral nutrition at a Median times of 10 months (range 3 – 36 months) >50% achieved enteral autonomy after months. 10/11 who achieved enteral Independence had the colon

……in contrast to randomized, controlled studies reduction of parenteral support took longer

early clinical markers of response: increase in stool consistency and reduction of stool frequency as well as sensation of thirst. Top responders patients with colon in continuity

Morethan50%onimmunosuppressants

La sindrome da intestino corto in Sicilia: una survey AIGO

DOMANDESULCENTROESULMEDICO

1)Leioperainunrepartodi:

a)Gastroenterologiab)Medicinac)Serviziodinutrizioneclinicad)Territorio

2)Ilsuorepartoèall’internodiun:

a)OspedaleUniversitario

b)Ospedale

c)Presidioterritoriale

3)Qualèlasuasferadiinteresse:

a)IBD

b)GastroenterologiaGenerale

c)NutrizioneClinica

d)Altro:specificare

DOMANDESUSINDROMEDAINTESTINOCORTO

1)Hainfollow-uppazienticonsindromedaintestinocorto? SI NO

2)Numero:

3)EtiologiaSBSnelsuocampione:

PatologiacausadiSBS NumeropazientiMalattiadiCrohn Ischemiamesenterica Enteritedaraggi Complicanzechirurgiche Poliposifamiliare Volvolo Malformazioniintestinali Enteritenecrotizzante Altro

Cognome Nome

Professione:

Specialità:

Affiliazione(Reparto/Dipartimento):

Ospedale

Indirizzo

Città CAP

Tel./cell.

Fax:

e-mail:

La sindrome da intestino corto in Italia: una survey AIGO

4)Neipazienticonprecedentiresezioniintestinalisitrattadi:

TipologiaSBSpost-chirurgico NumeropazientiDigiunostomiaterminale,nocolonincontinuità Anastomosidigiuno-colica,novalvolaileociecale,colonincontinuità

Anastomosidigiuno-ileale,valvolaileociecalepresenteecolonincontinuità

5)Hainfollow-uppazienticoninsufficienzaintestinalecronicadaaltrecause? SI No

6)Numero

7)Etiologiainsufficienzaintestinalecronicanelsuocampione:

Condizionecausale NumeropazientiSindromepseudoostruttiva Sclerodermia Hirschsprung Fistoleintestinali Celiachiarefrattaria Altro

La sindrome da intestino corto in Italia: una survey AIGO

8)Ilsuopazienteèinnutrizioneparenteraledomiciliare? SI No

9)Sesiqualeaccessovenoso?

a)PICC

b)Port-a-cath

c)Altro

10)Isuoipazientisonoassistitidaunprogrammadiassistenzadomiciliareintegrata? SI NO

11)Sesiquale:

a)ErogatodaASPdiappartenenza

b)ErogatodaAziendafarmaceutica

12)Isuoipazientiassumonointegratorinutrizionalioraliopersonda? SI NO

13)Vuoleaggiungereuncommento?

Conclusions•  SBS is a rare but disabling condition with high direct and

indirect costs both for patients and caregivers and the health system

•  The improved management by adopting supportive measures and the increasing diffusion of home-care facilities for parenteral nutrition and novel technical devices has increased life expexctancy

•  Teduglutide represents a novel opportunity to a further reduction of morbidity and possible weaning from HPN

•  Active case finding is warranted to select patients •  A multidisciplinar approach is the key for better results