Nutrition support considerations in pediatric small bowel transplantation.

Enteral autonomy is the primary goal of intestinal failure therapy. Intestinal transplantation (ITx) is an option when enteral autonomy cannot be achieved and management complications become life-threatening. The purpose of this review is to summarize existing medical literature related to nutrition requirements, nutrition status, and nutrition support after pediatric ITx. Achieving or maintaining adequate growth after intestinal transplant in children can be challenging because of episodes of rejection that require the use of corticosteroids, occurrences of infection that require a reduction or discontinuation of enteral or parenteral support, and fat malabsorption caused by impaired lymphatic circulation. Nutrient requirements should be assessed and modified regularly based on nutrition status, growth, ventilatory status, wound healing, and the presence of complications. Parenteral nutrition (PN) should be initiated as a continuous infusion early postoperatively. Enteral support should be initiated after evidence of graft bowel function and in the absence of clinical complications. Foods high in simple carbohydrates should be limited, as consumption may result in osmotic diarrhea. Short-term use of a fat-free diet followed by a low-fat diet may reduce the risk of the development of chylous ascites. Micronutrient deficiencies and food allergies are common occurrences after pediatric ITx. Enteral/oral vitamin and mineral supplementation may be required after PN is weaned. Nutrition management of children after ITx can be challenging for all members of the healthcare team. Anthropometric parameters and micronutrient status should be monitored regularly so that interventions to promote growth and prevent or reverse nutrient deficiencies can be implemented promptly.

Outcomes of Adult Intestinal Transplant Recipients Requiring Dialysis and Renal Transplantation.

BACKGROUND: Data on dialysis and renal transplantation (RT) after intestinal transplantation (IT) are sparse. Whether changes in immunosuppression and surgical techniques have modified these outcomes is unknown. METHODS: Two hundred eighty-eight adult intestinal transplants performed between 1990 and 2014 at the University of Pittsburgh were analyzed for incidence, risk factors and outcomes after dialysis and RT. Cohort was divided into 3 eras based on immunosuppression and surgical technique (1990-1994, 1995-2001, and 2001-2014). Receiving RT, or dialysis for 90 days or longer was considered as end-stage renal disease (ESRD). RESULTS: During a median follow-up of 5.7 years, 71 (24.7%) patients required dialysis, 38 (13.2%) required long-term dialysis and 17 (6%) received RT after IT. One-, 3-, and 5-year ESRD risk was 2%, 7%, and 14%, respectively. No significant era-based differences were noted. Higher baseline creatinine (hazard ratio [HR], 3.40 per unit increase, P < 0.01) and use of liver containing grafts (HR, 2.01; P = 0.04) had an increased ESRD risk. Median patient survival after dialysis initiation was 6 months, with a 3-year survival of 21%. Any dialysis (HR, 12.74; 95% CI 8.46-19.20; P < 0.01) and ESRD (HR, 9.53; 95% CI, 5.87-15.49; P < 0.01) had higher mortality after adjusting for covariates. For renal after IT, 1- and 3-year kidney and patient survivals were 70% and 49%, respectively. All graft losses were from death with a functioning graft, primarily related to infectious complications (55%). CONCLUSIONS: In intestinal transplant recipients, renal failure requiring dialysis or RT is high and is associated with increased mortality. Additionally, the outcomes for kidney after IT are suboptimal due to death with a functioning graft.

Intestinal Transplantation: International Outcomes.

Intestinal transplantation has continued to evolve over the past decade. Fewer patients have received intestine transplants in the past 5 years, perhaps due to efforts in intestine rehabilitation. Despite improvement in earlier outcomes, long-term survival has remained steady over the past decade. This is potentially due to the complications of immunosuppression, as well as inherent poor graft half-life due to chronic rejection. Improvements in outcome will require multidisciplinary efforts to understand the long-term mechanisms of intestine graft acceptance and to properly optimize and individualize immunosuppression for the transplant recipient.

Giant insulinoma: a report of 3 cases and review of the literature.

Insulinoma is a rare pancreatic neuroendocrine tumor that is usually described as benign, sporadic, and very small (<2 cm). However, there have been rare case reports of insulinoma presenting as a giant tumor. We describe 3 cases of giant insulinomas, all of which developed liver metastases. The patients were aged 38, 63, and 67 years. Clinically, all patients presented with Whipple's triad associated with a large mass located in the pancreatic tail. The tumors ranged in size from 10 to 15 cm. On microscopic examination, the tumors were well differentiated with amyloid deposition ranging between 20% and 30%. Immunohistochemically, all 3 tumors showed strong diffuse expression of chromogranin and synaptophysin, whereas they were only focally positive for insulin. One patient developed liver recurrence 3 years after resection of the primary tumor yet remained asymptomatic without treatment. Another patient with liver recurrence underwent right hepatectomy and has been free of disease for 2 years. The third patient died of metastatic disease 13 years after initial surgery. Giant insulinomas are characterized by focal expression of insulin and high rates of liver metastases. Long-term follow-up is mandatory in these patients, as recurrence is expected after primary surgery.