Fabio Fusaro

Neonatal outcome of gastroschisis is mainly influenced by nutritional management

Objective: The aim of the study was to evaluate early minimal enteral feeding (MEF) and gradual enteral nutrition increment on neonatal outcome of gastroschisis. Patients and Methods: An intervention group was prospectively assessed and compared with an observational historical control group. The prospective study relied on a new protocol of enteral nutrition. According to the new protocol, MEF was initiated 5 days after bowel reintegration and milk amounts were increased 12 mL/kg/day. In the control group, enteral nutrition was delayed until resolution of postoperative ileus, and increment of feeding was not systematized. Results: Twenty-two patients were included in the MEF group and compared with 51 control patients. Infants in the control group had lower gestational age (36 vs 35 gestational weeks [GW], P = 0.03) and birth weight (2465 vs 2200 g, P = 0.05). Time to first enteral nutrition (5 vs 11.5 days, P = 0.0005) was significantly shorter in the MEF group. All patients in this group were fully enteral fed at day 60, though 30.4% of patients in the control group still needed parenteral nutrition at day 60 (P = 0.004). Incidence of nosocomial infection was reduced (9% of patients vs 40%, P = 0.016) and hospital stay tended to be shorter in the MEF group (40 vs 54.5 days, P = 0.08). In the univariate analysis, factors influencing the length of parenteral nutrition during the 2 periods were the severity of perivisceritis and new nutritional protocol. In the multivariate analysis, only nutritional protocol was significantly associated with the length of parenteral nutrition (P = 0.038). Conclusions: Early MEF and controlled increase of nutritional elements after bowel reintegration significantly improved outcome of gastroschisis in newborns.

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.

Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children

BACKGROUND Management of patients with total intestinal aganglionosis (TIA) is a medical challenge because of their dependency on parenteral nutrition (PN). Intestinal transplantation (ITx) represents the only alternative treatment for patients with irreversible intestinal failure for achieving intestinal autonomy. METHODS Among 66 patients who underwent ITx in our center, 12 had TIA. They received either isolated ITx (n = 4) or liver-ITx (LITx, n = 8) after 10 to 144 months of total PN. All grafts included the right colon. RESULTS After a median follow-up of 57 months, the survival rate was 62.5% in the LITx group and 100% in the ITx patients. The graft survival rate was 62.5% in the LITx group and 75% in the ITx group. All the surviving patients were fully weaned from total PN, after a median of 57 days. Pull through of the colon allograft was carried out in all patients. Fecal continence is normal in all but one of the surviving children. CONCLUSION These results suggest that ITx with colon grafting should be the preferred therapeutic option in TIA. Early referral to a transplantation center after diagnosis of TIA is critical to prevent PN-related cirrhosis and thereby to permit ITx, which is associated with a good survival rate.

Recovery of graft steatosis and protein-losing enteropathy after biliary diversion in a PFIC 1 liver transplanted child.

Nicastro E, Stephenne X, Smets F, Fusaro F, de Magnée C, Reding R, Sokal EM. Recovery of graft steatosis and protein‐losing enteropathy after biliary diversion in a PFIC 1 liver transplanted child.

Donor age and ABCB1 1199G>A genetic polymorphism are independent factors affecting long-term renal function after kidney transplantation.

BACKGROUND In renal tubular cells, cytochrome P4503A enzyme and adenosine triphosphate-binding cassette transporter activities result in intracellular drug or metabolite exposure variability, depending on genetic polymorphisms. Our aim was to establish whether long-term renal function is affected by genetic polymorphisms in biotransformation enzymes and drug transporters of the donor after kidney transplantation. MATERIALS AND METHODS The study was conducted in a selected cohort of 97 kidney recipients. Genotyping of donors was performed on renal biopsy samples obtained before transplantation. Serum creatinine levels and Cockcroft-Gault estimated glomerular filtration rate were considered 1 y after transplantation and at the last follow-up. RESULTS Long-term function was significantly better in recipients of an organ from donors carrying the ABCB1 1199A mutated allele (median and range creatinine values were 1.1 mg/dL [0.8-1.5mg/dL] in case of at least one ABCB1 1199A allele versus 1.5 mg/dL [0.7-3.7 mg/dL] for homozygous carriers of wild-type allele, P < 0.01). ABCB1 1199G>A polymorphism and donor age had an independent impact on both serum creatinine and estimated glomerular filtration rate. Unlike donor age, the mutated ABCB1 1199A allele was found to have a protective effect on renal function. CONCLUSIONS Donor age and ABCB1 1199G>A polymorphism affect long-term renal function after transplantation. Analysis of genetic factors offers a promising approach to calcineurin inhibitor toxicity risk assessment.

Pleurodesis with povidone-iodine for refractory chylothorax in newborns: Personal experience and literature review

INTRODUCTION Refractory chylothorax is a severe clinical issue, particularly in neonates. Conventional primary approach is based on diet with medium-chain fatty acids and/or total parenteral nutrition. In nonresponders, proposed second line treatments include chemical or surgical pleurodesis, thoracic duct ligation, pleuroperitoneal shunting and pleurectomy but none of these have been shown to be superior to other in terms of resolution rate and safety. Our aim is to report our experience on povidone-iodine use for chemical pleurodesis in newborn infants with chylothorax unresponsive to conservative treatment. Our aim is to report our experience on povidone-iodine use for chemical pleurodesis in newborn infants with chylothorax unresponsive to conservative treatment. METHODS Since 2013, povidone-iodine pleurodesis was attempted in all patients with persistent chylothorax who failed conservative treatment (no response to at least 10 days of total parenteral nutrition and maximum dosage of intravenous octreotide). Pleurodesis consisted in the injection of 2 ml/kg of a 4% povidone-iodine solution inside the pleural space, leaving the pleural tube clamped for the subsequent 4 hours. RESULTS Five patients were treated with chemical pleurodesis of persistent chylothorax. Four of 5 patients had their pleural effusion treated by one single povidone-iodine infusion. Median time for resolution was 4 days. A patient with massive superior vena cava thrombosis did not benefit from pleurodesis. None of the patients experienced long term side effects of the treatment. CONCLUSION Our data suggest that povidone-iodine pleurodesis may be considered a safe and effective option to treat refractory chylothorax in newborns.

Autologous gastrointestinal reconstructive surgery: complement or alternative to intestinal transplantation

Purpose of reviewAutologous intestinal reconstructive surgery has evolved over the past 3 decades from rescue to main surgical procedure in the multidisciplinary approach to short bowel syndrome (SBS) patients with intestinal failure. The purpose of this review is to clarify the actual place of intestinal reconstructive surgical techniques in the management of intestinal failure related to SBS and their relationship with intestinal transplantation. Recent findingsRecent reports from centers of excellence in intestinal rehabilitation underline the efficacy and safety of autologous intestinal reconstructive surgery in patients with SBS. Outcome parameters as survival, parenteral nutrition weaning, and clinical conditions were improved in SBS patients treated by gastrointestinal reconstructive surgery. SummaryAutologous intestinal reconstructive procedures are pivotal to achieve enteral autonomy in patients with intestinal failure related to SBS. They should be considered mutually supportive and not antagonistic to intestinal transplantation.

Pediatric Chronic Intestinal Failure in Italy: Report from the 2016 Survey on Behalf of Italian Society for Gastroenterology, Hepatology and Nutrition (SIGENP)

Background: Intestinal failure (IF) is the reduction in functioning gut mass below the minimal level necessary for adequate digestion and absorption of nutrients and fluids for weight maintenance in adults or for growth in children. There is a paucity of epidemiologic data on pediatric IF. The purpose of this study was to determine the prevalence, incidence, regional distribution and underlying diagnosis of pediatric chronic IF (CIF) requiring home parenteral nutrition (HPN) in Italy. Methods: Local investigators were selected in 19 Italian centers either of reference for pediatric HPN or having pediatric gastroenterologists or surgeons on staff and already collaborating with the Italian Society for Pediatric Gastroenterology, Hepatology and Nutrition with regard to IF. Data requested in this survey for children at home on Parenteral Nutrition (PN) on 1 December 2016 included patient initials, year of birth, gender, family’s place of residence and underlying diagnosis determining IF. Results: We recorded 145 CIF patients on HPN aged ≤19 years. The overall prevalence was 14.12/million inhabitants (95% CI: 9.20–18.93); the overall incidence was 1.41/million inhabitant years (95% CI: 0.53–2.20). Conclusion: Our survey provides new epidemiological data on pediatric CIF in Italy; these data may be quantitatively useful in developing IF care strategy plans in all developed countries.

Pediatric Short Bowel Syndrome: Predicting Four-Year Outcome after Massive Neonatal Resection

Objectives The aim of this study was to ascertain predictors of survival, liver disease (LD), and enteral autonomy 48 months after resection in neonatal short bowel syndrome (SBS) patients with residual small bowel length (SBL) ≤40 cm. Patients and Methods Medical records of all SBS patients followed up between 1996 and 2016 were retrospectively reviewed. Survival rate, prevalence of LD, and of enteral autonomy were evaluated. Results Forty‐seven patients were included, and 43 were still alive at the end of the study period, with cumulative 48‐month survival of 91.5%. Twenty‐one (45%) patients developed LD, all within the first 6 months. On the final follow‐up visit, three (6%) patients were still jaundiced and progressed toward end‐stage LD. LD prevalence was higher in patients with recurrent bloodstream infections (odds ratio [OR] 5.4, 95% confidence interval [CI] 1.5‐19.3). Of the 43 surviving patients, 22 (51%) had enteral autonomy 48 months after resection. The probability of weaning off parenteral nutrition (PN) was strongly correlated with the remaining SBL. Conclusion Survival of patients who have undergone neonatal massive small bowel resection has improved in recent years. Multidisciplinary strategies can improve the course of LD, but not the probability of weaning off PN, which seems to be strongly dependent on the anatomical profile of residual bowel. Therefore, the primary surgical approach should be as conservative as possible to gain even small amounts of intestinal length, which may be crucial in promoting intestinal adaptation.

Kidney transplantation in infantile myofibromatosis and fibromuscular dysplasia: a case report

IntroductionWe report what we believe to be the first case of a child affected by two rare vascular diseases complicated by kidney failure and successfully treated by kidney transplantation.Case presentationA 3-year-old Caucasian girl with fibromuscular dysplasia and infantile myofibromatosis presented with arterial hypertension and renal failure. She received a deceased donor kidney transplantation distal to an iliac graft. The technical peculiarities of this transplantation are described, as well as her favorable long-term outcome.ConclusionKidney transplantation may be considered in a patient with vascular diseases and a history of iliac surgery.