Congenital abdominal wall defects
Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven in Succeeding in Paediatric Surgery Examinations, 2017
The long-term outcome for patients with gastroschisis is excellent, with an overall survival of more than 90%. The presence of intestinal atresia, which is found in 10% of cases, is the most important prognostic determinant for a poor outcome. Mortality is mainly related to intestinal failure, associated liver disease, and factors associated with small bowel and/or liver transplantation. In full-term infants with gastroschisis necrotising enterocolitis has been encountered at higher than expected frequencies (up to 18.5%). Cryptorchidism is associated with gastroschisis with an incidence of 15%–30%, and gastro-oesophageal reflux in 16% of cases. Most cases of cryptorchidism, even after replacement of herniated testes into the abdominal cavity, will result in normal testicular descent. Most long-term survivors of gastroschisis will lead normal lives. Scenario 3, is suggestive of ‘vanishing gastroschisis’. This condition is associated with a very small abdominal wall defect and is characterised by necrosis and disappearance of some or all of the intestine, resulting in short bowel syndrome. These patients are likely to be on long-term total parenteral nutrition. In scenario 4, one should suspect intestinal atresia in cases that fail to establish on feeds by 4–6 weeks. This can be ruled out with a lower GI contrast study.
Necrotizing enterocolitis
Prem Puri in Newborn Surgery, 2017
The “clip and drop back technique,” which consists of resection of all necrotic bowel leaving the remaining clipped segments within the abdominal cavity without creating ostomies or anastomoses, has been advocated for patients with extensive multifocal disease. The viable segments are then re-anastomosed at a second operation 48–72 hours later. Delayed re-exploration has been proposed in a yet more controversial technique, the “patch, drain, and wait.” This approach involves irrigation of the abdominal cavity, primary approximation of intestinal perforations, placement of a Stamm gastrostomy, and insertion of two Penrose drains beneath the diaphragm that course along the lateral aspect of the peritoneal cavity and exit from the lower quadrants for continued peritoneal drainage.28 Postoperatively, patients are kept on long-term parenteral nutrition. The drains are left in place until the drainage ceases and patients are tolerating enteral feeds. The authors advocate postponing a second operation during the 2-week period immediately postoperatively, and in cases where return of bowel function does not occur, reoperation may occur as late as 2 months.
Development of palliative medicine in the United Kingdom and Ireland
Eduardo Bruera, Irene Higginson, Charles F von Gunten, Tatsuya Morita in Textbook of Palliative Medicine and Supportive Care, 2015
Is there ever a situation among patients with incurable, metastatic cancer where nutritional support, such as total parenteral nutrition, appears to benefit patients? As emphasized earlier, we believe that this approach is outside the standard of care for most patients with cancer. However, case reports and small series suggest that occasionally, these patients may benefit from total parenteral nutrition. Certainly, selection bias may result in the publication of the most favorable outcomes. The Mayo Clinic published a report on a 20-year retrospective experience with total parenteral nutrition in this group of patients. Â 48 Overall, the use of total parenteral nutrition at this institution was quite conservative; only 52 patients with incurable cancer, or 15% of all patients treated at home, received this intervention. Although most patients did poorly, 16 did live for 1 year or longer, presumably as a result of the total parenteral nutrition. In an attempt to tease out predictive factors in order to provide guidance on who might benefit from total parenteral nutrition from a practice perspective, no such predictive factor emerged. Thus, the conclusion of this study was that clinical discretion-in the setting of a multidisciplinary approach and after in-depth discussions with the patient and family members-should guide management when this nonstandard approach is considered.
Gastrointestinal manifestations of primary immune deficiencies in children
Published in International Reviews of Immunology, 2018
This is a rare X-linked syndrome due to a mutation in transcription factor forkhead box protein (FOXp3) affecting T-regulatory cells which leads to autoimmunity.42 IPEX is characterized by a triad of immune dysregulation, polyendocrinopathy (usually Type 1 diabetes mellitus) and enteropathy. This is an autoimmune disorder that affects males and presents in the first year of life.43 Children are affected by watery diarrhea, eczema, autoimmune hemolytic anemia, thrombocytopenia and neutropenia. Many patients require total parenteral nutrition. Patients usually die in childhood and the only cure is bone marrow transplantation. Histopathology of the small bowel in patients with IPEX syndrome has shown severe villous atrophy and mucosal erosion with lymphocytic infiltrates of the submucosa or lamina propria.44
Management of COPD patients during COVID: difficulties and experiences
Published in Expert Review of Respiratory Medicine, 2021
Mario Cazzola, Josuel Ora, Andrea Bianco, Paola Rogliani, Maria Gabriella Matera
Hospitalized patients with COVID-19 need prompt and appropriate nutritional intervention because sarcopenia induced by a decrease in weight and muscle mass may appear due to prolonged hospitalization that often causes protracted immobilization with consequent catabolism. This risk is even higher in COPD patients who often have a relatively high prevalence of malnutrition and sarcopenia [77]. All patients admitted with COVID-19 should have their nutritional status assessed. According to the European Society for Clinical Nutrition and Metabolism expert statement, nutritional intervention must be considered as an integral part of the approach to patients with SARS-CoV-2 infection [78]. This statement has proposed 10 recommendations for managing nutritional care in COVID-19 patients highlighting that all elderly, frail and comorbid subjects, including those with COPD, should receive nutritional therapy. If the patient cannot be fed satisfactorily with enteral nutrition, it is necessary to begin parenteral nutrition.
Nutritional status as a predictor of adverse events and survival in pediatric autologous stem cell transplant
Published in Pediatric Hematology and Oncology, 2020
Izabela Kranjčec, Nuša Matijašić, Mario Mašić, Alen Švigir, Gordana Jakovljević, Ante Bolanča
Seventy-seven children and adolescents were treated with single autologous stem cell transplant. The majority were males (54.5%), with a mean age of 7.9 years (0–4 y 36.4%, 5–9 y 27.3%, 10–14 y 15.6%, 15–19 y 20.8%, median 6 years). The most common diagnoses were extracranial solid tumor (81.8%), predominately neuroblastoma (40.3%), and Ewing sarcoma (22.1%). Medulloblastoma (9.1%) was the leading brain tumor and third most frequent indication. In 67 patients (87%) transplant was used as frontline therapy. Complete remission before the procedure was achieved in 41.4% patients. The prevailing conditioning regimen was carboplatin/etoposide/melphalan (46.8%), followed by busulfan/melphalan (27.3%). The average CD34+ cell dose totaled 9.38 × 106/kg (range 2.16–39.08 × 106/kg). Average neutrophil time to engraftment was 11.56 days (maximum 32 days) and platelet time to engraftment was 16.61 days (maximum 37 days). The mean number of erythrocyte transfusions was 1.53 (range 0–10) and of platelet transfusions 5.64 (range 1–30). Parenteral nutrition was used in 81.8% of our patients, and applied during a mean of 11 days. Hospitalization duration ranged from 14 to 37 days, with a mean of 23.63 days.
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