Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042961 (volvulus)
 4,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Transplant-transmitted malignances are rare but devastating events. Primary brain tumors are the least common among reported donor-derived malignancies. We report a case of donor-transmitted pineoblastoma, a PNET, in a two-yr-old male recipient, who presented with a rapidly growing mass in the right mandible, four months after multiple visceral organ transplantation. The recipient had liver, pancreas, and small bowel transplants because of end-stage liver failure and short gut syndrome, which was secondary to large bowel resection for management of gastroschisis complicated by intestinal volvulus. The donor autopsy results became available seven wk after transplantation, which found a pineoblastoma with meningeal spread. Evaluation of eyes, adrenal glands, bone marrow, and other organs did not identify metastasis outside the CNS. A biopsy of the recipient's right mandibular mass revealed a malignant small round blue cell tumor with the immunohistochemistry profile of a PNET. Staging evaluation revealed the tumor in the right mandible with bone marrow involvement. Further investigation showed that recipient's tumor and donor's pineoblastoma shared the same immunophenotype and HLA type, suggesting the recipient's tumor is a donor-transmitted pineoblastoma. This is the first case report of donor-transmitted pineoblastoma post-organ transplant.
 ...
PMID:Donor transmission of pineoblastoma in a two-yr-old male recipient of a multivisceral transplant: a case report. 2128 15

There are several reasons for the possible development of a short bowel syndrome, which, however, occurs only rarely. The main causes consist of extended intestinal resections in cases of congenital anomalies (e.g., gastroschisis, intestinal atresia or dysplasia) or ischaemic lesions due to a volvulus. In addition, an intestinal stoma at a more upper segment of the GI tract can result in the functional manifestation of a short bowel syndrome. The differentiation between temporary and persisting types is essential for initiation of an adequate treatment. Loss or exclusion of organic resorption area at the inner surface of the (small) intestine can be associated with numerous pathological consequences requiring treatment. As a principle consideration from the paediatric point of view, the potential of intestinal adaptation needs to be assessed. Basic conservative treatment options are parenteral and enteral nutrition regimens, in particular, to prevent complications (such as D-lactate acidosis). The main surgical approaches are the procedures called LILT (longitudinal intestinal lengthening and tailoring) according to Bianchi and STEP (serial transverse enteroplasty). The technique to create intestinal segments of antiperistalsis has been abandoned. Because of the encouraging results of intestinal transplantation, this novel treatment option has gained greater attention over the past few years and is now also an option for paediatric patients. The limiting factor and thus major complication is the central venous catheter for long-term treatment. Catheter-related complications are still the main reason for a considerable mortality in these children.
...
PMID:[Age-dependent visceral medicine: paediatric visceral medicine - visceral medical paediatrics - considerations on the short bowel syndrome in childhood]. 2323 40

Short bowel syndrome (SBS) is the most common cause of intestinal failure in infants. In neonates and young infants, necrotizing enterocolitis, gastroschisis, intestinal atresia, and intestinal malrotation/volvulus are the leading causes of SBS. Following an acute postsurgical phase, the residual gastrointestinal tract adapts with reorganization of the crypt-villus histoarchitecture and functional changes in nutrient absorption and motility. A cohesive, multidisciplinary approach can allow most neonates with SBS to transition to full enteral feeds and achieve normal growth and development. In this article, the clinical features, management, complications, and prognostic factors in SBS are reviewed.
 ...
PMID:Short bowel syndrome in the NICU. 2341 63

The goal of any treatment programme for short bowel syndrome SBS is to achieve nutritional enteral autonomy. This must begin with conservation of as much bowel as possible from the time of first presentation. Frequent causes of the short bowel syndrome are intestinal atresia, necrotizing enterocolitis, midgut volvulus, extended intestinal aganglionosis, 'vanished gut' often associated with gastroschisis and occasionally catastrophic trauma. Atresia is more amenable to successful surgery than other causes, except when associated with gastroschisis. Intrinsic dysmotility has a poor prognosis. Intestinal lengthening procedures are only indicated if there is sufficient bowel dilatation. Extended intestinal aganglionosis is rarely amenable to any form of non-transplant surgery. Options available are to conserve bowel, close stomas early (use all available bowel to the maximum or even re-feed stoma effluent into the distal unused bowel), release adhesions causing obstruction, resect strictures, taper or excise localized dilatations and finally address dilated bowel with lengthening and tailoring operations. These procedures aim to improve effective peristalsis, thereby reducing bacterial overgrowth and improving nutrient contact with enteral mucosa to maximize absorption and intestinal adaptation. The Bianchi longitudinal splicing operation and the serial transverse enteroplasty operations have stood the test of time in providing considerable improvement in enteral nutritional autonomy in around 60% of cases. In SBS without dilatation attempts at 'mechanically' delaying transit (nipple valves, reversed bowel segments, colon interposition) have had inconsistent outcomes. Growing neomucosa and lengthening bowel by longitudinal stretch are still experimental.
 ...
PMID:Non-transplant surgery for short bowel syndrome. 2398 89

Short bowel syndrome (SBS) is the most common cause of intestinal failure in children. It is defined as the inability to maintain adequate nutrition enterally as a result of a major loss of the small intestine. SBS is a life-threatening entity associated with potential significant morbidity and mortality. The etiology in the pediatric age group includes necrotizing enterocolitis (32%), atresia (20%), volvulus (18%), gastroschisis (17%), and aganglionosis (6%). It is characterized by substrate malabsorption, electrolyte imbalance, intestinal bacterial overgrowth, steatorrhea, and weight loss. Current medical management includes parenteral nutrition, progressive feeds as tolerated, various medications, and surgical manipulations. However, frequently this management is not successful in achieving the goal of attaining normal growth and development without parenteral nutrition. It has been known for decades that there is a normal physiologic response of the residual intestine to massive bowel resection referred to as intestinal adaptation. The mechanisms that control this process are unknown. Unfortunately, intestinal adaptation and the current management are not always successful. As a result of new knowledge regarding the pathophysiology of SBS over the past two decades, several novel strategies have been developed in experimental animal models as well as limited clinical trials in infants and children. They can be divided into several categories that potentially influence intestinal (1) absorption, (2) secretion, (3) motility, and (4) adaptation. More recently, newer modalities have been studied including small intestine transplantation, and the use of specific intestinal growth factors. Ultimately, tissue and organ engineering will become the treatment for infants and children with SBS.
 ...
PMID:Novel therapies for the management of short bowel syndrome in children. 2398 26

Intestinal failure (IF), due to short bowel syndrome (SBS), results from surgical resection of a major portion of the intestine, leading to reduced nutrient absorption and need for parenteral nutrition (PN). The incidence is highest in infants and relates to preterm birth, necrotizing enterocolitis, atresia, gastroschisis, volvulus, and aganglionosis. Patient outcomes have improved, but there is a need to develop new therapies for SBS and to understand intestinal adaptation after different diseases, resection types, and nutritional and pharmacological interventions. Animal studies are needed to carefully evaluate the cellular mechanisms, safety, and translational relevance of new procedures. Distal intestinal resection, without a functioning colon, results in the most severe complications and adaptation may depend on the age at resection (preterm, term, young, adult). Clinically relevant therapies have recently been suggested from studies in preterm and term PN-dependent SBS piglets, with or without a functional colon. Studies in rats and mice have specifically addressed the fundamental physiological processes underlying adaptation at the cellular level, such as regulation of mucosal proliferation, apoptosis, transport, and digestive enzyme expression, and easily allow exogenous or genetic manipulation of growth factors and their receptors (e.g., glucagon-like peptide 2, growth hormone, insulin-like growth factor 1, epidermal growth factor, keratinocyte growth factor). The greater size of rats, and especially young pigs, is an advantage for testing surgical procedures and nutritional interventions (e.g., PN, milk diets, long-/short-chain lipids, pre- and probiotics). Conversely, newborn pigs (preterm or term) and weanling rats provide better insights into the developmental aspects of treatment for SBS in infants owing to their immature intestines. The review shows that a balance among practical, economical, experimental, and ethical constraints will determine the choice of SBS model for each clinical or basic research question.
...
PMID:Animal models of gastrointestinal and liver diseases. Animal models of infant short bowel syndrome: translational relevance and challenges. 2534 47

Aim: To evaluate the results of the use of the T-tube ileostomy in neonatal intestinal surgery cases. Materials and Methods: A retrospective review of sixty two neonates underwent intestinal obstruction surgery by using T-tube ileostomy was conducted between January 1990 and January 2013.The pathologies of the intestinal obstruction were; thirty four of jejunoileal atresia cases, thirteen case meconium ileus, eight cases perforated necrotizing enterocolitis (NEC), three cases meconium peritonitis, three cases with bowel resection due to intestinal volvulus, and one case of gastroschisis. Results: Mean duration of T-tube placement was 13 days (range9-20days) and the sites of T-tube insertion closed spontaneously in 2 days (range 1-4 days). The mean duration for starting oral intake postoperatively in these patients was 9 days (6-16 days). All patients well tolerated the procedure and there were no serious complications related to the T-tube insertion. However, four patients died due to other reasons like sepsis, respiratory failure and prematurity. Conclusion: T-tube enterostomy is an effective and safe technique for treatment of selected cases of neonatal intestinal surgery. It showed less morbidity and mortality rates than the conventional stoma. Therefore, it is considered a helpful approach in cases where there is danger of hypoperistaltic dilated bowel proximal to the anastomosis.
 ...
PMID:Use of T-Tube Enterostomy in Neonatal Gastro-intestinal Surgery. 2789 54

Ultrashort bowel syndrome (USBS) is a group of heterogeneous disorders where the length of small bowel is less than 10 cm or 10% of expected for the age. It is caused by massive loss of the gut which in the neonatal period can be a result of vanishing gastroschisis or surgical resection following mid-gut volvulus, jejunoileal atresia and/or extensive necrotising enterocolitis. The exact prevalence of USBS is not known although there is a clear trend towards increasing numbers because of increased incidence and improved survival. Long-term parenteral nutrition (PN) is the mainstay of treatment and is best delivered by a multidisciplinary intestinal rehabilitation team. Promoting adaptation is vital to improving long-term survival and can be achieved by optimising feeds, reducing intestinal failure liver disease and catheter-related bloodstream infections. Surgical techniques that can promote enteral tolerance and hence improve outcome include establishing intestinal continuity and bowel lengthening procedures. The outcome for USBS is similar to patients with intestinal failure due to other causes and only a small proportion of children who develop irreversible complications of PN and will need intestinal transplantation. In this review, we will summarise the available evidence focusing particularly on the epidemiology, management strategies and outcome.
 ...
PMID:Epidemiology, management and outcome of ultrashort bowel syndrome in infancy. 2886 23

Currently, the most important determinant of gastroschisis outcomes in high resource settings is whether the condition is associated with intestinal complications, such as atresia, necrosis, perforation, or volvulus. This form of the anomaly, known as complex gastroschisis, accounts for most of the mortality and a disproportionate burden of the morbidity from gastroschisis. There is some disagreement about what constitutes complex gastroschisis, and little consensus on the type and timing of surgical interventions. This article establishes a clear definition of complex gastroschisis. Surgical approaches to treatment of the diverse presentations of complex gastroschisis will be described and the timing of such interventions will be discussed. Contemporary outcomes of complex gastroschisis will be reviewed. Finally, a non-congenital intestinal complications that may arise in gastroschisis patients will be discussed.
 ...
PMID:Surgical strategies in complex gastroschisis. 3041 62

As survival of gastroschisis patients has improved significantly, it has become apparent that longitudinal follow up strategies need to be developed. Problems concerning patients with gastroschisis are usually associated with gastrointestinal morbidity, but there is mounting evidence that also neurodevelopmental, cognitive, behavioral and late-onset auditory sequelae exist. The presence of associated anomalies, as well as complex features (bowel atresia, necrosis, volvulus, perforation) increase morbidity and impact long-term outcomes. Multidisciplinary follow-up is required, and the key elements of such follow-up are presented here.
 ...
PMID:Medium and long-term outcomes of gastroschisis. 3041 65


<< Previous 1 2 3 4 5 Next >>