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An introduction to genetics and application to Crohn's disease.

Gene-based diagnostics and therapeutics are being explored for application in many areas of healthcare. An understanding of the principles of genomics has become fundamental to patient care within all specialties of nursing and is expected to be included in certification exams in the near future. This introductory article is designed to provide practicing Wound, Ostomy, and Continence nurses with basic information to enhance their abilities to comprehend genetics information, apply it to practice, and translate it to colleagues and patients. Specifics about the genetic basis of Crohn's disease are used to illustrate the application of the concepts described in the article.

Alternative Splicing↗

Renal transplantation in children with severe bladder dysfunction.

PURPOSE: Renal transplantation in children with bladder dysfunction carries a risk for the renal graft. We report our experience with transplantation in 15 patients 6 to 18 years old with severe abnormalities of the lower urinary tract. MATERIALS AND METHODS: A total of 18 renal transplants were performed in 15 children with bladder dysfunction secondary to myelomeningocele (3), occult spina bifida (1), malformation/agenesis of the sacrum (5), posterior urethral valves (4), female hypospadias (1) and bladder exstrophy (1) between 1979 and 2003. Urological surgery was performed before transplantation in 14 cases-7 bladder augmentations, 5 incontinent urinary conduits/reservoirs and 2 vesicostomies. Voiding was maintained by intermittent catheterization in 9 cases and incontinent ostomies in 6. Graft implantation was performed by extraperitoneal route with ureteral anastomosis to the native bladder in cases of bladder augmentation. Immunosuppression consisted of triple therapy with polyclonal/monoclonal antibodies. RESULTS: Urological complications consisted of urethral obstruction due to mucus hypersecretion (1), urinary fistula (1), ureterovesical obstruction (1), stone formation (3), urinary tract incrustation by Corynebacterium urealyticum (1) and pyelonephritis (2). Graft survival rates at 1 and 5 years were 77% and 62%, respectively, with a median of 79 months (95% CI 51 to 107). Three graft losses were related to urological disease. CONCLUSIONS: Renal transplantation in children with severe bladder dysfunction can achieve similar results to those obtained in the general population. Meticulous selection of patients and surgical reparative techniques ensuring voiding and adequate control of urinary infections are mandatory. Augmentation cystoplasty and intermittent catheterization are appropriate techniques currently used for achieving this outcome.

Adolescent↗

Restoration of fecal continence after functional gluteoplasty: long-term results, technical refinements, and donor-site morbidity.

PURPOSE: For patients with severe fecal incontinence, reconstruction of the anal sphincter, via gluteoplasty, may improve quality of life, but little is known about long-term functional results. We present our comprehensive experience with gluteoplasty, highlighting technical refinements, donor-site morbidity, and functional outcomes. METHODS: We performed a retrospective analysis of 25 consecutive patients (22 female, 3 male; mean age 42 years, range 23-65) undergoing gluteoplasty for fecal incontinence at a university teaching hospital from 1996-2004. Etiology of incontinence was as follows: obstetrical injury (n = 13), irritable bowel syndrome (n = 3), previous rectal surgery (n = 3), Crohn disease (n = 3), impalement (n = 1), rectocele (n = 1), and idiopathic (n = 1). RESULTS: Gluteoplasty was successful in restoring fecal continence in 18 patients (72%) and was partially successful in 4 patients (16%). Two patients required permanent ostomy because of refractory incontinence. Donor-site morbidity and perirectal complications were observed in 16 patients (64%) and included dysthesias (n = 7), cellulitis (n = 5), irregular contour (n = 3), abscess (n = 2), seroma (n = 2), fistula (n = 1), but no hip dysfunction or altered gait. Mean length of follow-up was 20.6 months (range: 3-68 months). CONCLUSIONS: Despite a high incidence of donor-site and perirectal complications, unilateral functional gluteoplasty was successful in restoring long-term fecal continence in most patients.

Adult↗

A custom-made distraction device for experimental mandibular distraction osteogenesis.

Distraction osteogenesis has recently become popular in craniofacial reconstruction, after it was first described for long bones. Despite the widespread clinical use of this technique, currently, lack of an ideal experimental model for small animals hampers investigations focused on its molecular basis, which warrants further elucidation. The authors developed a new device for distraction of the rat mandible and, to asses its effectiveness, practiced on 30 animals assigned to either an acute 3-mm distraction group (n = 7) or a gradual distraction group (0.25 mm twice a day for 6 days; n = 23). The authors applied the devices to a localization that allowed them to perform the ostomies posterior to the molar teeth. Because of a U-shaped plate that the authors used for posterior pin fixation, their device maintained its stability until the end of the study, despite the delicate anatomy of the bone at the posterior part of the mandible. Additionally, the authors described a practical and easy method of transient mandibular stabilization method that facilitates the manipulations while the mouth is open for safer airway control during surgery. This new nutrition technique with enteral feeding solution solved the problem of progressive weight loss after surgery. Application of the device was easy and practical, without demanding complex manipulations. The authors did not observe any device dislodgement or a high rate of accidental fractures during the manipulations. They demonstrated that the device works properly and is able to create ossified regenerate bones that fill the entire distraction gap, which can be used for various investigations during distraction osteogenesis.

Animals↗

Ileal pouch-anal anastomosis for ulcerative colitis and familial adenomatous polyposis: historical development and current status.

In summary, the history and development of the proctocolectomy and ileal pouch-anal anastomosis has involved innovative animal and clinical research by several surgical investigators. This evolution followed the classic process of academic surgical progress: a clinical problem is identified; solutions are studied in the laboratory; and these solutions are applied back to the clinical situation with success. Dr. Sabiston's disappointment with clinical results in ulcerative colitis and familial polyposis patients led to laboratory experiments in which a new technique was shown safe in dogs. The further work of his collaborator Dr. Ravitch as well as that of Sir Alan Parks and Dr. Utsunoimya proved small-scale clinical application of the new technique. Finally, large-scale outcomes work by Dr. Fazio at the Cleveland Clinic Foundation and others has allowed further refinements to occur and has highlighted other areas to study. The work of these investigators and other has allowed lack of a permanent ostomy with satisfactory functional results in more than 95% of patients. Continued experience with these procedures has and will lead to further improvements in operative times, morbidity rates, and functional results. Although research in this area will continue, the evolution of this operation has allowed it to become the gold standard for the treatment of ulcerative colitis and familial adenomatous polyposis.

Adenomatous Polyposis Coli↗

The "Fistula VAC," a technique for management of enterocutaneous fistulae arising within the open abdomen: report of 5 cases.

BACKGROUND: Management of intestinal fistulae in open abdominal wounds remains a significant clinical challenge for those caring for patients surviving damage control abdominal operations. Breaking the cycle of tissue inflammation, infection, and sepsis, resulting from leakage of enteric contents, should be a major goal in the approach to these complex patients. We describe a technique utilizing vacuum assisted closure (VAC) which achieves control of enteric flow from fistulae in open abdominal wounds. METHODS: The fistula-VAC is fashioned from standard sponge supplies, negative pressure pumps, and ostomy appliances. The fistula-VAC was changed every three days prior to split thickness skin grafting, and every five days following grafting. RESULTS: Five patients underwent application of the fistula-VAC. All patients had complete diversion of enteric contents. This enteric diversion allowed for successful skin grafting in all patients. CONCLUSION: Application of the fistula-VAC should be considered a useful option in treating patients with intestinal fistulae in open abdominal wounds.

Colostomy↗

Long-term prognosis in Crohn's disease: factors that affect quality of life.

BACKGROUND: There are many studies investigating quality of life in recently diagnosed patients and following surgery for Crohn's disease, but there are none investigating quality of life changes with disease duration. The response shift model suggests quality of life improves with time following diagnosis. AIM: To assess how well the model applies to patients with Crohn's disease. METHODS: The Cardiff Crohn's disease database contains data on all patients diagnosed there since 1934. Three hundred and ninety four patients diagnosed before 1 January 1985 were traced and the mortality status on 31 December 2004 established. Two hundred and eleven still living were sent quality of life questionnaires. Two hundred and eighty five questionnaires were sent to patients with varying disease duration attending out-patient clinics in Leicester. RESULTS: Eighty-nine valid replies were received from Cardiff, 63 from Leicester patients diagnosed over 20 years, 69 from Leicester patients diagnosed <10 years. There was no difference in quality of life between newly diagnosed and established patients. Of greatest concern was possible need for ostomy, uncertain nature of disease, and lack of energy. Stepwise regression showed that increased disease severity, older age and smoking adversely affect quality of life. DISCUSSION: Quality of life is equally poor in patients with established disease as in those newly diagnosed, and directly correlates with disease severity. The response shift model may not be applicable in Crohn's disease.

Adult↗

What are the differences in body image between patients with a conventional stoma compared with those who have had a conventional stoma followed by a continent pouch?

This paper describes a qualitative study of seven patients which explored the perceptions of body image in patients with a conventional stoma, compared with those who have had a conventional stoma followed by a continent pouch. The operation of choice for patients with ulcerative colitis (the group of patients in the study) who wish to avoid permanent ileostomy is restorative proctocolectomy with ileal reservoir, more commonly known as the 'pouch' procedure. The outcome of this study, using a phenomonenological approach, reflects the problems faced by patients undergoing stoma surgery; that is, patients with a stoma, on the whole, expressed difficulties in coming to terms with an ostomy, with perceived negative feelings of body image. In contrast, patients with a continent pouch, provided they were not experiencing difficulties with the pouch, were of the opinion that such a procedure enhanced their lives over that of an ileostomy. However, in examining these patients' feelings, it was found that the majority still considered their experience traumatic. This implies that the benefits of a continent pouch were not so obvious, as body image changes of stoma surgery still had a perceived negative effect on the patients. This affected the outcome of the study so that the differences of the two groups of patients were not as significant as perceived by the writer.

Adaptation, Psychological↗

An external urine collection device for incontinent women. Evaluation of long-term use.

Urinary incontinence is common in aged women, may precipitate nursing home admission, and may prompt use of a urine collection device, usually an indwelling urethral catheter. The safety and efficacy of a new external urine collection device for women that is affixed to the perineum by an adhesive developed for ostomy bags was evaluated. Applied to 26 aged women, 78% of 2,264 devices were leak-free for 24 hours and 49% for 48 hours. The incidence of new bacteriuria was less than half that found in our earlier studies of long-term urethral catheters in the same institution. Perineal erythema was infrequent and preexisting decubitus ulcers improved or did not change. Four patients were withdrawn, one each because of periurethral itching, diminished urine output, recurrent wetness, and fracture of the proximal femur associated with severe osteoporosis. This device may offer an alternative to urethral catheters for management of urinary incontinence but should not be used on women with urine retention and should be used with care on women with severe osteoporosis. Controlled trials must determine effects upon bacteriologic complications and health-care costs.

Aged↗

Health-related quality of life in Swedish patients with ulcerative colitis.

OBJECTIVE: The aim of this study was to characterize the health-related quality of life (HRQOL) in a Swedish population of patients with ulcerative colitis. METHODS: A total of 211 patients with ulcerative colitis were studied. Demographic and disease-related factors were noted. HRQOL was measured by one disease specific questionnaire, the Rating Form of IBD Patient Concerns (RFIPC) and one generic, The Sickness Impact Profile (SIP). Additional questions regarding information needs, medication, and well-being were asked. Disease activity was measured by symptom cards, laboratory samples, endoscopy, and two indices of disease activity. The influence of additional concomitant disease was also evaluated. RESULTS: Functional impairment as measured by the SIP was primarily in psychological and social areas and to a lesser extent in the physical areas. The highest scores for individual items of the RFIPC were those related to potential complications, e.g., needing an ostomy appliance, needing surgery, developing cancer, losing bowel control, and uncertainty about the disease and effects of medication. Patients with active disease scored higher on both SIP and RFIPC when compared to patients in remission. Presence of coexisting disease weighted heavily on HRQOL. CONCLUSION: Ulcerative colitis has a negative influence on the subjective functional status and seems to cause many worries and concerns. Patients in relapse had greater concerns, more impairment of functional status, and a reduced subjective sense of well-being than patients in clinical remission. Nevertheless, the patients in this Swedish study scored a much better HRQOL than has previously been reported using these questionnaires in patients with ulcerative colitis from the US, France, and Austria.

Adult↗

Medical therapy to reduce postoperative Crohn's disease recurrence.

Clinical recurrence of Crohn's disease after surgical resection is a significant problem, with reported rates as high as 55% at 5 yr and 76% at 15 yr. Specific factors that predispose to postoperative recurrence of Crohn's disease have not been well defined. In addition, the underlying pathophysiology of recurrent disease and the reason for its localization to the neoterminal ileum are not well understood. Various operative techniques have been evaluated but none, aside from formation of an ostomy, has been shown to reduce the risk of recurrence. In contrast, there is increasing evidence that postoperative medical therapy has the potential to decrease the risk of postoperative recurrence. Historically, sulfasalazine may have a modest effect on reducing postoperative recurrence of ileal or ileocolonic disease. However, 5-ASA preparations that can selectively deliver mesalamine to the small bowel or anastomotic margin should be more effective. Indeed, in several studies and as confirmed by a meta-analysis, mesalamine has been demonstrated to reduce significantly postoperative recurrence of Crohn's disease. Metronidazole and 6-mercaptopurine or azathioprine also seem to be of benefit in postoperative prophylaxis of disease recurrence, but additional controlled studies are required to define better the efficacy and dose-response of these agents. Corticosteroids are ineffective at reducing postoperative recurrence.

Anti-Infective Agents↗

Cross-cultural variation in disease-related concerns among patients with inflammatory bowel disease.

OBJECTIVE: The aim of this work was to study cross-cultural variations in the impact of inflammatory bowel disease (IBD) on health-related quality of life by an international comparison of disease-related concerns. METHODS: Item and factor scores on the Rating Form of Inflammatory Bowel Disease Patient Concerns and overall mean concern levels were compared by analysis of variance among 2002 IBD patients in eight countries. RESULTS: The overall level of concern varied from 51 out of 100 in Portugal to 19 in Sweden, with intermediate scores for Italy (43), Canada (40), United States (39), France (39), Austria (33), and Israel (25). Having surgery, an ostomy, the uncertain nature of the disease, and medication side effects were each rated among the first five in importance in six countries. Other items varied considerably. For example, concern regarding pain and suffering was high in Israel and low in Portugal, whereas concern over developing cancer was low in Italy. Concern over financial issues and access to high-quality health care were inversely associated with measures of national economic prosperity. CONCLUSIONS: 1) Cross-cultural comparisons of patient concerns related to IBD are feasible using translated scales. 2) Reporting tendencies vary greatly; within Europe, patients from southern countries report greater overall concern. 3) The complications and the variable evolution of disease elicit general concern, but the importance of specific issues varies among countries. 4) The reasons for national differences may have social, cultural, and/or economic determinants with relevance to the patient-physician relationship, patient education, and therapeutic decision making.

Canada↗

Health-related quality of life in Crohn's disease: a prospective longitudinal study in 231 patients.

OBJECTIVE: The aim of this work was to assess the quality of life (QoL) of patients with Crohn's disease (CD) prospectively over 1 yr and to determine factors of influence. METHODS: A total of 231 CD patients were included. At month 0 (M0), M3, M6, M9, and M12, patients were given a validated QoL questionnaire (self-administered) to fill in and a clinical form referring to the period of 3 months before the visit. The QoL questionnaire was made up of the Short-Form-36 and the Rating Form of Inflammatory Bowel Disease Patients Concerns. The impact on QoL of the following factors was analyzed: age, gender, CD duration and localization, presence of extradigestive manifestations or concomitant disease, disease course, medical treatments, and surgery. We studied the correlations between QoL and disease activity assessed by both patients and investigators by a visual analog scale. RESULTS: At M0, all the scores of the Short-Form-36 were significantly lower than those of a standard population, nevertheless improving between M0 and M12. Patients' main worries were first "having an ostomy bag" followed by "uncertain nature of the disease," "energy level." and finally "having surgery." QoL was better correlated with assessment of disease course by the patient than by the investigator. Significant factors of impairment in QoL were female gender, tobacco, active CD, involvement of the colon, hospitalization, corticoid treatment, and surgery in the past 3 months. Conversely, intake of immunosuppressors improved QoL. CONCLUSION: Patients' QoL is impaired by CD and is underestimated by doctors. Tobacco, hospitalization, and use of corticoids have a negative impact on QoL. Conversely, the use of immunosuppressors is associated with a better QoL.

Adolescent↗

Skin changes induced by a zinc oxide dressing compared with a hydrocolloid dressing in healthy individuals.

BACKGROUND/PURPOSE: Incidence of skin complications in ostomy patients constitutes a well-known and well-described problem. The reasons are, however, very difficult to describe because of the many factors contributing to the problem. This article describes the skin changes derived exclusively from the adhesives used in a carefully controlled, long-term study using two fundamentally different types of adhesives: a hydrocolloid adhesive and a zinc oxide adhesive. METHODS: The adhesives were changed daily on the volar forearm of 11 volunteers for a 4-week period. Once a week, transepidermal water-loss (TEWL), water content of the skin, erythema and the peel force applied for removal of the adhesives were measured. On the last day of the study, a replica of the skin surface was obtained to determine changes in the skin topography, and a biopsy was taken to study changes at the cellular level. RESULTS AND CONCLUSION: We found increased TEWL and decreased water content in skin treated with the zinc oxide adhesive, but increased water-loss and water content when the hydrocolloid adhesive was used. In addition, the area treated with zinc oxide adhesive showed significant increase of epidermal thickness, scaly appearance and parakeratosis with similarities to pathological dry skin diseases such as psoriasis and atopic dermatitis, changes that were not found when using the hydrocolloid adhesive. The skin response seems to be the result of the content of zinc oxide and the mechanical interaction of the zinc oxide adhesive. We conclude that the nature of the adhesive plays an important role in the skin response to repeated application of adhesives, as seen in peristomal skin.

Adhesiveness↗

Longitudinal mechanical tension induces growth in the small bowel of juvenile rats.

INTRODUCTION: The aim of our study was to apply longitudinal force to the small bowel to increase the length of intestine in juvenile rats. METHODS: Fifty juvenile rats had double barrelled, blind loop ostomies created using an isolated segment of bowel. Our intestinal lengthening device was inserted into one of the loops and the second loop served as a control. Once the device was deployed, the experimental, control, and in situ segments of bowel were evaluated for length, weight, histology, and disaccharidase enzyme activity. RESULTS: Mechanical tension increased intestinal length by 149%. The lengthened bowel also exhibited a greater total weight (218%), greater mucosal weight (122%), and increased protein mass (164%) compared with the control limb of bowel. Histologically, there was a markedly increased thickness of the muscularis propria in the lengthened bowel (200% increase compared with the control limb). Functionally, we found increased total disaccharidase activity in the lengthened bowel (between 47% and 350%, depending on the particular enzyme tested; p<0.01). CONCLUSION: Mechanical tension induces intestinal growth by increasing length, weight of the bowel and mucosa, and protein mass. Histological changes, such as increases in Paneth cells, suggest that increased proliferation and reorganisation of the mucosa and muscularis propria are a response to mechanical tension. Functionally, increased intestinal length corresponds with increased disaccharidase activity, thus implying potential increased absorptive capacity of the lengthened bowel.

Animals↗

Alternatives to ileostomy after colectomy for inflammatory bowel disease.

Proctocolectomy and Brooke ileostomy have long been the standard surgical procedure for patients with refractory ulcerative colitis or colonic Crohn's disease. Ileorectal anastomosis, ileoanal anastomosis, the continent ileostomy, and the continent ostomy valve are now being used in selected patients as options to the Brooke ileostomy. The indications for and advantages of these procedures and their potential disadvantages and contraindications are discussed in this chapter.

Anal Canal↗

[Percutaneous endoscopic gastrostomy. Reality in the intra- and extra-community clinical nutritional practice].

BACKGROUND: This study aimed to review our experience during 8 years in the use of percutaneous endoscopic gastrostomy (PEG) and its application in home enteral nutrition (HEN). MATERIAL: We studied 207 patients (56 women and 151 men) who had undergone a PEG from the beginning of 1994 to the end of 2002 as they needed prolonged enteral nutrition (> 4 weeks). In those cases in which home enteral nutrition was programmed, the patients/relatives were trained in the techniques and care of the PEG and EN, and the control was done through the nutrition out-patient clinic. RESULTS: Mean duration time of the PEG was 640 days and 175 patients (84.6%) needed PEG for more than 60 days and 135 for more than 6 months. Mean calorie supply was 1,730 +/- 288 Kcal/day. Administration mode was by intermittent infusion due to seriousness in 162 cases and continuous infusion through volemetric pump in 45 patients. In 2 patients with hyperemesis gravidarum, percutaneous endoscopic gastrojejunostomy (PEGJ) was done in the 3rd and 4th month of pregnancy, the pregnancy finishing successfully by vaginal delivery. Performance of PEG facilitated hospital discharge and programming of home enteral nutrition in 195 patients (94%). The most frequent complications were gastrostomy infection that occurred in 41 patients and the appearance of granuloma in the ostomy in 34 cases. Only one patient died in direct relationship due to peritonitis after PEG. CONCLUSION: Our study includes the advantages of PEG as an enteral nutrition technique, permitting the establishment of a home enteral nutrition program with limited incidence of complications and very low mortality.

Enteral Nutrition↗

Extraesophageal varices.

Esophageal varices are the most common site of variceal bleeding. However, bleeding from varices that are not located in the esophagus may account for up to 30% of all variceal bleeding. Significant varices can occur in the stomach, duodenum, jejunum, ileum, colon, rectum, and biliary tree. They can also occur at the site of a surgical ostomy. These types of varices bleed less commonly than esophageal varices, but they can also be far more difficult to diagnose and treat. The absence of stigmata of recent esophageal variceal bleeding and certain clues in the patient's history and clinical presentation should raise the clinician's suspicion of an extraesophageal site of variceal bleeding. Particularly, patients with extrahepatic causes of portal hypertension, cirrhotic patients with a prior history of gastrointestinal surgery, and patients who present with profound bleeding but without hematemesis need to be evaluated further if an obvious site of esophageal variceal bleeding is not seen on initial endoscopy. In this article, we review the features particular to extraesophageal varices as well as the diagnosis and management of bleeding from these varices. We use the term extraesophageal rather than ectopic because the term ectopic varices implies exclusion of both esophageal and gastric varices.

Digestive System↗