dott.ssa maria cappello · 2019. 6. 10. · la sindrome da intestino corto in italia: una survey...

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