Introduction: The Intestinal Rehabilitation and Transplant Center (CRITx) in our hospital was created in 2018 to support children with intestinal failure in the public health care system. Many of these patients come from families with low socioeconomic status. Despite those possible limitations, a training program was designed in order to allow parents to take care of their children after hospital discharge with home parenteral nutrition (HPN). This abstract shows the feasibility and the results of this HPN training program on this specific population. Methods: The training program was given to caregivers by two intestinal rehabilitation nurses and followed by a multidisciplinary team. The HPN curriculum covered the following aspects: aseptic technique, CVC dressing, line disconnection, parenteral nutrition connection and infusion pump handling; caregivers were trained to identify possible complications. The program was tailored according to the perception of the nurses on the learning progress of the caregivers. A rigid validation was applied to all caregivers at the end of each learning module. Results: From April 2018 to December 2020, 48 patients with intestinal failure were referred to CRITx, and 18 were discharged with HPN. Regarding patients in HPN, 61% were boys, median age at hospital admission was 9 months (range 2 to 95 months) and 15 out 18 patients had short bowel syndrome (11 patients with ultra-short bowel); median weight-for-age z-score was -3.32 (range -5.58 to 1.91). The mother was the main caregiver in 17/18 of the cases. Median monthly family income was USD 240 (range USD 185 to USD 1363), approximately 1 Brazilian minimum wage; unplanned 30-day readmission rate related to HPN was 22.2% (n=4). Median time of follow-up on HPN was 323 days (range 71 days to 850 days). One patient was submitted to a living donor intestinal transplant and the remaining 17 patients still on HPN, in good nutritional and developmental conditions. Conclusion: Despite the low-income scenario faced in our population, the provided training was able to assure safety for discharge the patients in HPN. The 30-day readmission rate in this population was similar to the current findings for patients with other complex chronic diseases.

Introduction: Intestinal transplantation remains the treatment of choice for patients with irreversible intestinal failure (IIF) and severe complications of parenteral nutrition. We describe the first case of a living donor intestinal transplantation (LDIT) performed in Brazil. This strategy was used after the clinical condition of the patient deteriorated while on the waiting list for a deceased donor. Methods: Case report, highlighting the planning and execution of the technique of LDIT. Result: A 3-year-old patient with IIF due to intestinal atresia was referred for intestinal transplantation due to progressive loss of venous access sites, with worsening of the venous thrombosis during the waiting time on the list for a deceased donor. After 8 months on the list, a LDIT was performed: the donor was her mother, and a 160 cm length small bowel graft was harvested. Total ischemia time was 62 minutes, and the graft was transplanted using the recipient’s aorta and inferior vena cava for vascular anastomosis. On postoperative day (POD) 15, the patient presented a severe acute cellular rejection (ACR) resistant to corticosteroids and thymoglobulin, successfully treated with alemtuzumab. She was discharged home on POD 80, totally free of parenteral nutrition or any intravenous fluid/electrolyte supplementation. The patient remained stable with a totally functioning graft during all the follow-up period; 11 months after LDIT, she was diagnosed with a central nervous system lymphoma, and died after treatment failure, 17 months after LDIT. Conclusion: LDIT is a viable and safe strategy for the treatment of children with IIF, especially in the scenario of the shortage of pediatric organ donors. Possible immunological advantages using a graft obtained from a living donor are uncertain.

Introduction: Optimal weight gain and nutritional recovery may be challenging in patients who have the association of severe malnutrition and intestinal failure associated liver disease (IFALD). In this situation, due to the necessity of lipid minimization, the level of energy requirement for optimal weight gain may not be achieved without higher levels of glucose infusion; in many centers, glucose infusion rate (GIR) is limited to 12.5 mg/Kg/min to 13 mg/Kg/min. Methods: Retrospective analysis including intestinal failure children with a weight-for-age z-score < -3 and a total bilirubin level > 6 mg/dL. Parenteral nutrition was switched to a high carbohydrate regimen when satisfactory weight gain was not obtained with conventional GIR associated with lipid limitation. Anthropometric measurements and liver function tests were routinely performed. Results: From April 2018 to December 2020, 48 patients were referred to our intestinal rehabilitation center. Five patients were included in this analysis: 3M/2F, median age at admission 8 months (range 6 months to 17 months); intestinal failure was caused by short bowel syndrome (SBS) in all but one patient. The 4 patients with SBS had a small bowel length < 15 cm. At the beginning of the treatment, median weight-for-age z-score was -4.72 (range -7.69 to -3.63) and median total bilirubin level was 11.76 mg/dL (range 10.78 to 16.7 mg/dL). Median duration of GIR > 13 mg/Kg/min was 56 days (range 28 to 84 days), including a period with a median of 35 days (range 14 to 56 days) when a GIR > 14 mg/Kg/min was used. Median time to resolve cholestasis (bilirubin level < 2 mg/dL) was 104 days (range 55 to 160 days). Median weight-for-age z-score at 2, 4, and 6 months after admission were -3.36 (-6.71 to -2.68), -3.15 (-5.09 to -2.47) and -2.36 (-4.18 to -1.73), respectively. No clinical signs of fatty acid deficiency were observed in any children. Conclusion: In extreme situations, involving the association of severe malnutrition and IFALD, when lipid minimization may impose a limitation in optimal gain weight, the use of a temporary high carbohydrate parenteral nutrition may be useful to achieve nutritional recovery while treating cholestasis.

Background. Data describing the technical aspects of living donor (LD) domino liver transplantation (DLT) in maple syrup urine disease (MSUD) are limited. The largest published series includes only 3 cases. One great challenge of this procedure is to ensure adequate vascular stumps for the LD, the MSUD patient, and the recipient of the domino graft. Here, we describe our experience in 11 cases of LD-DLT in MSUD, highlighting the technical aspects of LD-DLT. Methods. From September 2012 to September 2017, 11 patients with MSUD underwent LD liver transplantation at our institution, and MSUD livers were used as domino grafts in 11 children. Results. (1) MSUD patients: 10 patients received a left lateral segment. The donor's left hepatic vein (HV) was anastomosed to the confluence of the recipient's 3 HVs. No vascular grafts (VG) were required for portal vein (PV) anastomosis. Single arterial anastomosis was performed with microsurgery in 10 of 11 patients. (2) MSUD graft recipients: In 8 cases, HV reconstruction was performed between the graft's HV confluence and the recipient's HV confluence, and in 3 cases, a vena cava triangulation was necessary; 6 MSUD grafts required HV venoplasty. No VG were needed for HV reconstruction. VG were used for PV reconstruction in 3 cases due to sclerotic PV. In 2 cases, double arterial anastomoses were performed in the MSUD liver. All patients remain alive and well. Conclusions. Living donor liver transplantation followed by DLT for MSUD is a complex procedure and demands technical refinement. Special attention must be paid to vascular reconstruction.

NLL, 15 anos, masculino, pardo, brasileiro, solteiro, naturalde Itamogi (MG) e estudante. Paciente admitido no HospitalGeral do Grajau da Faculdade de Medicina da UNISA com queixade dor abdominal, em colica, localizada principalmente emepigastrio, havia cerca de tres meses, com aumento progressivoda intensidade, sem irradiacao e com piora apos alimentacao.Referia vomitos pos-prandiais de conteudo alimentar e emagre-cimento de 6 kg neste periodo.Acompanhante relatava a ingestao continua de corpos estra-nhos (cabelo e espuma do sofa) ha cerca de seis meses, desde aseparacao dos pais, tendo interrompido o habito com o inicio dador abdominal.Ao exame encontrava-se consciente, orientado, eupneico,afebril, acianotico, anicterico, corado e hidratado. Auscultacardiopulmonar sem alteracoes. Abdome normotenso, doloroso apalpacao em regiao de epigastrio, com massa palpavel emepigastrio e hipocondrio esquerdo, sem sinais de irritacaoperitoneal.Realizado RX de abdome, que demonstrou aumento do volumegastrico, com efeito de massa, deslocando colon transverso inferior-mente (Fig.1) . Submetido a endoscopia digestiva alta, que compro-vou a hipotese de tricobezoar, de grande volume, que se estendiadesde a cardia e insinuava-se pelo piloro (Fig.2), impossibilitando apassagem do aparelho e delimitacao de sua extensao, nao sendofactivel sua retirada endoscopica.Pela impossibilidade de tratamento conservador, optou-se porrealizacao de laparotomia exploradora, com gastrotomia anterior(Fig.3) e remocao de volumoso tricobezoar (Fig.4), que moldavatodo corpo, fundo antro- gastrico e duodeno, com odor fetido ecomposto principalmente de cabelos (Fig.5).Paciente evoluiu satisfatoriamente, recebendo alta no setimoPO, sendo encaminhado ao servico de Psiquiatria da Faculdadede Medicina da Universidade de Santo Amaro.