Long-term outcomes of living-related small intestinal transplantation in children: A single-center experience.

Pediatric patients with irreversible intestinal failure present a significant challenge to meet the nutritional needs that promote growth. From 2002 to 2013, 13 living-related small intestinal transplantations were performed in 10 children, with a median age of 18 months. Grafts included isolated living-related intestinal transplantation (n=7), and living-related liver and small intestine (n=6). The immunosuppression protocol consisted of induction with thymoglobulin and maintenance therapy with tacrolimus and steroids. Seven of 10 children are currently alive with a functioning graft and good quality of life. Six of the seven children who are alive have a follow-up longer than 10 years. The average time to initiation of oral diet was 32 days (range, 13-202 days). The median day for ileostomy takedown was 77 (range, 18-224 days). Seven children are on an oral diet, and one of them is on supplements at night through a g-tube. We observed an improvement in growth during the first 3 years post-transplant and progressive weight gain throughout the first year post-transplantation. Growth catch-up and weight gain plateaued after these time periods. We concluded that living donor intestinal transplantation potentially offers a feasible, alternative strategy for long-term treatment of irreversible intestinal failure in children.

Chronic intestinal failure in children.

BACKGROUND: Chronic intestinal failure (CIF) in childhood is caused by congenital malformations and inflammatory diseases of the gut. Its reported prevalence is 13.7 per million population. Long-term home parenteral nutrition has dramatically improved the life expectancy and quality of life of children with CIF. The affected children are now treated with parenteral nutrition at home as soon as their medical state and family circumstances allow. METHODS: The authors present data from a patient registry and review publications retrieved by a selective literature search. RESULTS AND CONCLUSION: Children with CIF can now be expected to survive beyond adolescence, at the very least, and enjoy good quality of life. This goal can only be achieved if nutritional therapy is carried out safely and the affected children's development is closely monitored by an interdisciplinary team that consists of primary care physicians/family doctors, neonatologists, pediatric gastroenterologists, and pediatric surgeons. Moreover, the prevention, early detection, and appropriate treatment of complications such as infection, liver disease, renal dysfunction, and disturbances of bone metabolism is of vital importance. The patients' families must be supported by specially qualified ambulatory nurses and social workers. Treatment with parenteral, enteral, and oral nutrition and surgery enables most infants with CIF to meet all their nutritional needs orally by the time they start going to school. For children who suffer from intractable complications, intestinal transplantation provides a real and increasing chance of survival.

Survival outcomes of pediatric intestinal failure patients: analysis of factors contributing to improved survival over the past two decades.

BACKGROUND: Intestinal failure (IF) is associated with significant and life-threatening complications. Recent studies suggest that treatments for IF in the pediatric population are improving over time. Based on this, we examined whether pediatric IF survival rates have improved in our patient population over the past two decades, and secondarily examined which aspects of patient care contributed to changes in survival. METHODS: We conducted a retrospective chart review of all pediatric patients with IF at our children's hospital from 1990 through 2009. Cox regression analyses were used to determine change in survival rates over time (5-y cohorts), and we examined multiple covariates to determine their potential influence on survival rates over time. RESULTS: A significant improvement in survival of 171 children with IF was noted over the past two decades. Children with an onset of IF in the 1990-1994 cohort had significantly decreased survival compared with children in all subsequent cohorts (P = 0.011). The only intervention that was identified between this time period and future periods was the establishment of a comprehensive intestinal failure clinical care team. While the latter three cohorts were not significantly different, progressively increased survival was noted. Ability to wean off parenteral nutrition (PN) and small bowel length greater than 10% of the expected length were significantly associated with improved survival over this time period (P < 0.01). Other tested covariates, including the more recent use of ethanol lock therapy (to prevent catheter sepsis) and lipid reduction strategies (to treat PN-associated cholestasis) failed to show a significant impact on improved survival. CONCLUSIONS: Despite a striking improvement in survival of children with IF over the past two decades, the only identified intervention that significantly impacted survival was establishment of a comprehensive care team. These findings emphasize the need for multi-disciplinary efforts to care for such complex and challenging children.

Is intestinal transplantation now an alternative to home parenteral nutrition?

Patients with irreversible intestinal failure and complications of parenteral nutrition should now be routinely considered for small intestine transplantation. Despite attempts for >40 years immunological graft intolerance presented an impenetrable barrier to successful engraftment until the development in the late 1970s of the powerful calcineurin-inhibitor immunosuppressive agents. Their use over the last 17 years has led to small intestinal transplantation being generally considered as a routine option for patients with irreversible intestinal failure and failing parenteral nutrition. The 1-year patient survival rates (%) are now excellent for renal (95), liver (78), heart (82) and lung (75) transplantation. In contrast, survival rates for small intestinal transplantation have been slow to improve, although they are now approaching those for lung and liver transplantation (intestine 78%, intestine and liver 60%, multivisceral 66%), and well-performing centres report recent 1-year graft survival rates as high as 92%. Patient 5-year survival (%) has also improved (intestine alone 50, intestine and liver 50 and multivisceral 62) and compares increasingly favourably with renal (85), liver (67), heart (67) and lung (46). Currently, small intestinal transplantation is reserved for patients with irreversible small intestinal failure who have a poor prognosis on parenteral nutrition. However, as 5-year patient survival following intestinal transplantation approaches that for parenteral nutrition there will be increasing pressure to offer this modality of treatment as an alternative to parenteral nutrition, especially for those patients who have a poor quality of life as a result of parenteral nutrition.

[The modifying effect of novocaine on the clinical manifestations of the intestinal form of radiation sickness]. / Modifitsiruiushchee vliianie novokaina na klinicheskie proiavleniia kishechnoi formy luchevoi bolezni.

On rats and dogs exposed to absolute lethal doses of X-irradiation (rats--10, 12 Gy; dogs--15 Gy sublethally) it has been shown that novocaini injection to animals immediately after radiation exposure reduces the primary radiation reaction and at the same time shifts the changes in morphofunctional state of peripheric blood on 24 hours, reduces the symptoms of acute radiation sickness, increases the average life length of the animals on 1,5 days. A conclusion concerning the expressed therapeutic influence of intraperitoneal novocaini injection immediately after radiation has been drawn.

[The mechanisms of the action of radioprotectors of different chemical classes in the intestinal syndrome]. / O nekotorykh mekhanizmakh deistviia radioprotektorov raznykh khimicheskikh klassov pri kishechnom sindrome.

Some thiazole, triazole, thiadiazine, and heterylalkane derivatives increased the lifespan of gamma-irradiated (9-14 Gy) mice up to 6-22 days (as opposed to 4.1 days in the irradiated control). The efficacy of chemical agents within the dose range mentioned above was mainly associated with protecting intestinal epithelium stem cells the number of which was 4-9 times as large as that of nontreated animals. There was a moderate (40-50%) decrease in oxygen consumption under the effect of the most active radioprotectors.

Gas gangrene infections of the small intestine, colon and rectum.

Thirty-three cases of gas gangrene infections originating from the small bowel, colon, and rectum are reviewed. The distinction between localized and diffuse, spreading, types of infection is made. The overall mortality rate was 42.4% and mortality was highest for infections following elective bowel resections. Treatment consisted of antibiotics and surgical debridement, with hyperbaric oxygen used as adjunctive therapy for the more serious cases. The importance of early recognition of clostridial infection is stressed as the key to improved survival.

Ischemic bowel disease.

The clinical, radiologic and pathologic features of 25 cases of ischemic bowel disease are presented. The majority of patients presented with the triad of abdominal pain, diarrhea and vomiting. In 13 patients the diarrhea was associated with the passage of bright red blood per rectum. There were 10 cases of infarction, 11 of enterocolitis and 4 had resulted in stricture formation. In five cases of enterocolitis the lesion was transient; symptoms improved with conservative medical management and the radiologic findings returned to normal. Barium enema examination yielded abnormal findings in the majority of the cases in which it was performed. Plain films of the abdomen, however, were not helpful. The actual mortality in this group of patients was 44%, 80% in those with infarction of the bowel and 20% in the other two groups.