Long-Term Intestinal Failure and Home Parenteral Support: A Single Center Experience.

Introduction: Home parenteral nutrition (HPN) and/or home parenteral hydration (HPH) are the gold-standard treatment for patients with long-term intestinal failure (IF). The authors aimed to assess the impact of HPN/HPH on nutritional status and survival of long-term IF patients, as well as HPN/HPH-related complications. Methods: This was a retrospective study including IF patients under HPN/HPH followed in a single large tertiary Portuguese hospital. The data collected included demographics, underlying conditions, anatomical characteristics, type and duration of parenteral support, IF functional, pathophysiological, and clinical classifications, body mass index (BMI) at the beginning and end of follow-up, complications/hospitalizations, current patient status (deceased, alive with HPN/HPH, and alive without HPN/HPH), and cause of death. Survival after HPN/HPH beginning, until death or August 2021, was recorded in months. Results: Overall 13 patients were included (53.9% female, mean age 63.46 years), and 84.6% of patients presented type III IF and 15.4% type II. Short bowel syndrome caused 76.9% of IF. Nine patients received HPN and 4 HPH. Eight patients (61.5%) were underweight at the beginning of HPN/HPH. At the end of follow-up, 4 patients were alive without HPN/HPH, 4 maintained HPN/HPH, and 5 died. All patients improved their BMI (mean initial BMI 18.9 vs. 23.5 at the end, p < 0.001). Eight patients (61.5%) were hospitalized due to catheter-related complications, mainly infectious (mean hospitalization episodes 2.25, mean hospital stay of 24.5 days). No deaths were related to HPN/HPH. Conclusion: HPN/HPH significantly improved IF patients' BMI. HPN/HPH-related hospitalizations were common, however causing no deaths, reinforcing that HPN/HPH is an adequate and safe therapy for long-term IF patients.

Introdução: A nutrição parentérica domiciliária (NPD) e/ou a hidratação parentérica domiciliária (HPD) constituem o tratamento gold-standard para doentes com falência intestinal (FI) crónica. O objetivo do presente estudo foi avaliar o impacto da NPD/HPD no status nutricional e sobrevivência dos doentes com FI crónica, bem como as complicações relacionadas à NPD/HPD. Métodos: Estudo retrospetivo, incluindo doentes com FI sob NPD/HPD, seguidos num hospital terciário em Portugal. Informação recolhida para cada doente: dados demográficos, patologia de base, características anatómicas, tipo e duração do suporte parentérico, classificação funcional, fisiopatológica e clínica da FI, índice de massa corporal (IMC) no início e final do seguimento, complicações/hospitalizações, estado atual do doente (falecido, vivo sob NPD/HPD e vivo sem NPD/HPD) e causa de morte. A sobrevida após início da NPD/HPD foi calculada em meses, até à data da morte ou agosto de 2021. Resultados: Incluídos 13 doentes (53.9% do sexo feminino, idade média 63.46 anos), 84.6% com FI tipo III e 15.4% com FI tipo II. A síndrome do intestino curto foi causa de 76.9% das FI. Nove doentes foram tratados com NPD e 4 com HPD. Oito doentes (61.5%) apresentavam IMC baixo no início da NPD/HPD. No final do seguimento, 4 doentes estavam vivos sem NPD/HPD, 4 mantinham NPD/HPD e 5 faleceram. Todos os doentes melhoraram significativamente o seu IMC (IMC médio no início do seguimento 18.9kg/m2 vs 23.5kg/m2 no final, p<0.001). Oito doentes (61.5%) tiveram de ser hospitalizados devido a complicações relacionadas com o cateter, sobretudo de causa infeciosa (número médio de hospitalizações por doente 2.25, duração média de internamento 24.5 dias). Não houve mortes relacionadas com a NPD/ HPD. Conclusão: A NPD/HPD melhorou significativamente o IMC dos doentes com FI. As hospitalizações relacionadas com a NPD/HPD foram comuns, contudo não causaram mortes, reforçando o facto que a NPD/HPD é uma terapêutica segura e adequada para doentes com FI crónica.

Recurrent midgut volvulus in an adult patient - The case for pexy? A case report and review of the literature.

INTRODUCTION: Intestinal malrotation is a rare condition, with an incidence estimated between 0,2 to 1%. Most cases are diagnosed and treated during childhood. Adult presentations are rare and most adults present with chronic nonspecific complaints. Midgut volvulus is the most feared complication of intestinal malrotation, far more common among the pediatric than the adult population. Presentation in adulthood with a midgut volvulus accounts for a minority of these patients (15%). The Ladd procedure is the standard surgical management of midgut volvulus and intestinal malrotation. Most evidence on the outcomes of the Ladd procedure originates from studies on pediatric population and the recurrence among children who have had a Ladd procedure is low (2-7%). PRESENTATION OF CASE: We report an exceedingly rare case of a patient who presented in adulthood with a midgut volvulus and less than two years after undergoing Ladd procedure presented with a recurrence of the midgut volvulus. The recurrent midgut volvulus was successfully treated by a fixation procedure (cecopexy and duodenopexy). CONCLUSION: Reports of midgut volvulus in adult patients are scarce and reports of recurrence even scarcer hence the rate of recurrence among adult patients has yet to be determined. The recurrence rate in some of the available adult series is much higher than the rate reported among children. Should the rate of recurrence among adult patients prove higher, it poses the question of whether the Ladd procedure should be modified to include bowel fixation when performed in adults.

Molecular Staging of Patients with Colon Cancer. The C-Closer-II Study: A Multicentre Study in Portugal.

INTRODUCTION: Approximately 20% - 30% of histological lymph node-negative patients with colorectal cancer relapse at five years after surgical treatment. This recurrence is likely due to occult nodal disease undetected by standard histopathological practice which has implications in terms of the clinical management of patients. MATERIAL AND METHODS: Lymph nodes were collected from colectomy specimens. A central section from each lymph node was histologically examined following haematoxylin-eosin staining and the remaining tissue was subjected to OSNA - one step nucleic acid amplification analysis. RESULTS: A total of 1046 lymph nodes from 59 pN0 patients were assessed. Of these, 753 lymph nodes were examined by both methods. The median number of lymph nodes assessed with OSNA - one step nucleic acid amplification was 12 (IQR: 7;16). Among pN0 patients, 17 had OSNA - one step nucleic acid amplification-positive lymph nodes, resulting in a positive molecular staging rate of 28.8% (95% CI: 17.8 - 42.1). Among these patients, 12 (70.59%) were molecular-staged as pN1 and 5 (29.41%) were molecular staged as pN2. The tumour burden of lymph nodes assessed with OSNA - one step nucleic acid amplification ranged from 270 to 17 000 cytokeratin 19 mRNA copies/µL. Most of these patients (88.2%) were found to have lymph nodes with micrometastases only (250 - 4999 copies/µL). DISCUSSION: We provide the results from the first study of the use of the OSNA - one step nucleic acid amplification assay in colorectal cancer patients in Portugal. Our results are in-line with other international studies, showing the improvement on patients' staging by molecular examination of lymph nodes. CONCLUSION: In our study, 28.8% of patients with histologically negative lymph nodes were found to have metastatic lymph nodes using OSNA - one step nucleic acid molecular assessment. OSNA - one step nucleic acid assay allows a more accurate staging of patients with colorectal cancer and standardizes lymph node assessment.

Introdução: Cerca de 20% - 30% dos doentes com cancro colo-rectal, com gânglios linfáticos regionais negativos por histologia têm recidiva do carcinoma colo-rectal, após cinco anos do tratamento cirúrgico. Esta recorrência é provavelmente devida à presença de metástases ganglionares ocultas, não detetadas no exame anatomo-patológico standard. Material e Métodos: Os gânglios linfáticos foram obtidos a partir de espécimes de colectomia. Uma secção central de cada gânglio linfático foi analisada histologicamente com a coloração de hematoxilina-eosina e o tecido restante foi sujeito a análise de OSNA - one step nucleic acid amplification. Resultados: Um total de 1046 gânglios linfáticos de 59 doentes pN0 foram avaliados. Foram examinados 753 gânglios linfáticos por ambos os métodos. A mediana de gânglios linfáticos avaliados com OSNA - one step nucleic acid amplification foi de 12 (IQR:7; 16). Entre os doentes pN0, 17 tinham gânglios linfáticos positivos para OSNA - one step nucleic acid amplification, resultando numa taxa de estadiamento molecular positiva de 28,8% (95% CI: 17,8 - 42,1). De entre esses doentes, 12 (70,59%) apresentaram-se molecularmente pN1 e cinco (29,41%) pN2. A carga tumoral dos gânglios linfáticos avaliada com OSNA - one step nucleic acid amplification variou de 270 a 17 000 cópias/µL de ARNm de citoqueratina 19. A maioria desses doentes (88,2%) apresentou gânglios linfáticos com micrometástases (250 - 4999 cópias/µL). Discussão: Apresentamos os resultados do primeiro estudo do ensaio OSNA - one step nucleic acid amplification levado a cabo, em pacientes com cancro colo-rectal, em Portugal. Os nossos resultados estão em linha com outros estudos internacionais, demostrando uma melhoria no estadiamento dos doentes, pelo exame molecular dos gânglios linfáticos. Conclusão: Verificou-se que 28,8% dos doentes com gânglios linfáticos histologicamente negativos apresentavam doença ganglionar metastática, usando a avaliação molecular de OSNA - one step nucleic acid amplification. O ensaio OSNA - one step nucleic acid amplification permite um estadiamento mais preciso de doentes com carcinoma do colon e padroniza a avaliação dos gânglios linfáticos.