Long-term outcome of home parenteral nutrition in patients with ultra-short bowel syndrome.
OBJECTIVE
The patients with ultra-short bowel syndrome (U-SBS) have been considered potential candidates for a preemptive/rehabilitative intestinal transplantation owing to the high risk of death from the underlying disease. We hypothesized that children with U-SBS, in the absence of intestinal failure-associated liver disease (IFALD), could also have a good rate of survival on home parenteral nutrition (HPN).


METHODS
A prospective database from the "Bambino Gesù" Artificial Nutrition and Intestinal Failure Program was used to evaluate outcomes and morbidities of consecutive patients with ≤ 10 cm of small bowel enrolled since 2000.


RESULTS
Eleven patients were identified with a median bowel length of 7.5 (3-9) cm. Eight patients developed IFALD, which reversed in 7 of them; the IFALD progressively worsened in 1 patient until death. One patient underwent isolated intestinal transplantation and 1 patient is no longer receiving parenteral nutrition (PN) and both are fully enterally fed. The other patients remained at least partially dependent on HPN. The number of days of inpatient care decreased in all of the patients except for the 1 who had repeated episodes of central line infections.


CONCLUSIONS
The survival of patients with U-SBS receiving HPN was good. Although IFALD was frequent, it had been manageable in most of the patients, but in a single complex case, it led to death. The multidisciplinary management warranted to these patients to approach the school age, to grow, and to maintain the oral intake. Patients with U-SBS are rare, and to better understand their long-term survival, further studies, including more large patient populations, are required.
*Hepatology, Gastroenterology and Nutrition Unit †Department of Medical and Surgical Neonatology ‡Department of Paediatric Surgery and Transplantation, Bambino Gesù Children's Hospital, Rome, Italy.
Antonella
A
Diamanti
Andrea
A
Conforti
Fabio
F
Panetta
Giuliano
G
Torre
Manila
M
Candusso
Pietro
P
Bagolan
Raffaele Edo
RE
Papa
Chiara
C
Grimaldi
Fabio
F
Fusaro
Teresa
T
Capriati
Domenica
D
Elia
Jean
J
De Ville de Goyet
D002648:Child; D002657:Child Development; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007421:Intestine, Small / Q000503:physiopathology; D008107:Liver Diseases / Q000209:etiology*; D008297:Male; D016331:Parenteral Nutrition, Home; D012189:Retrospective Studies; D012778:Short Bowel Syndrome / Q000150:complications* / Q000503:physiopathology / Q000628:therapy*; D015996:Survival Rate; D013997:Time Factors