Necrotizing Enterocolitis following Gastroschisis Repair: An Update

Purpose: Necrotizing enterocolitis (NEC) is a known complication of gastroschisis with an incidence above the expected rate in the neonatal population. While many physicians today are aware of this association, the last publication to explore this association in detail and identify possible risk factors of NEC in gastroschisis patients was published over twenty years ago. From our large database of patients with gastroschisis managed by a single group of pediatric surgeons, we reviewed our experience and the recent literature to update what is known about gastroschisis and NEC. Methods: From 2001 to 2017, a gastroschisis registry was maintained. Data from 218 gastroschisis patients were reviewed. Patient demographics and hospital course were reviewed. Patients with confirmed NEC were compared to those without NEC. Results: Two hundred eighteen patients were born with gastroschisis during the time frame of this study. We observed a 5% rate (11 of 218) of NEC. Five patients (45%) developed recurring NEC and 4 patients (36%) were readmitted for NEC development following initial discharge. Variables associated with NEC included low gestational age (P=0.016) and low birth weight (P=0.003). Patients born prior to 37 weeks gestation had a 4.8 times greater risk of developing NEC than those born at term. Rates of IUGR were not statistically different between NEC and non-NEC patients. The method of delivery (cesarean vs vaginal), use of a silo, and form of nutrition were not significantly associated with NEC development. Conclusions: The overall incidence of NEC has decreased compared to earlier reports. NEC does complicate the hospital course for patients, significantly increasing duration of in-hospital treatment. NEC in gastroschisis differs in comparison to traditional NEC, presenting later in life. Risk factors identified include low gestational age and low birth weight. Avoiding elective preterm deliveries may decrease the rate of NEC in gastroschisis

Gastric outlet obstruction due to adenocarcinoma in a patient with Ataxia-Telangiectasia syndrome: a case report and review of the literature

Background: Ataxia-Telangiectasia syndrome is characterized by progressive cerebellar dysfunction, conjuctival and cutaneous telangiectasias, severe immune deficiencies, premature aging and predisposition to cancer. Clinical and radiographic evaluation for malignancy in ataxia-telangiectasia patients is usually atypical, leading to delays in diagnosis. Case presentation: We report the case of a 20 year old ataxia-telangiectasia patient with gastric adenocarcinoma that presented as complete gastric outlet obstruction. Conclusion: A literature search of adenocarcinoma associated with ataxia-telangiectasia revealed 6 cases. All patients presented with non-specific gastrointestinal complaints suggestive of ulcer disease. Although there was no correlation between immunoglobulin levels and development of gastric adenocarcinoma, the presence of chronic gastritis and intestinal metaplasia seem to lead to the development of gastric adenocarcinoma. One should consider adenocarcinoma in any patient with ataxia-telangiectasia who presents with non-specific gastrointestinal complaints, since this can lead to earlier diagnosis

Immune competence of cancer-reactive T cells generated de novo in adult tumor-bearing mice

The impact of timing of antigen introduction into fetus and neonates leads to the suggestion that pre-existing antigens are tolerogenic to immunocompetent cells generated thereafter. This hypothesis predicts that in patients with cancer who are undergoing bone marrow transplantation, newly produced T cells with specificity for pre-existing tumor cells will be inactivated by the tumor antigens in the host. Because the effect of tumor cells on developing cancer-reactive T cells has not been investigated, we set out to systematically analyze the impact of tumor cells in the periphery on the development of tumor-reactive T cells in the thymus and their immunocompetence in the periphery. Our data demonstrate that in the host in which a tumor is established in the periphery, the cancer-reactive T cells develop normally, remain fully immunocompetent, become activated in the periphery, and cause regression of large established tumors. The immunocompetence of T cells generated in an antigen-bearing host is also confirmed in a skin graft transplantation model