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Bowel continence is one of the most difficult challenges for patients with spina bifida. Incontinence acts as a social stigma for children and a barrier for adults seeking employment. We present an algorithm for stepwise decision-making in construction of personalized continence programs for greater likelihood of success. The protocol contains 13 assessment points including; stool consistency, frequency and amount; mobility; level of paraplegia: diet; medication; anal/rectal canal tone; prior programs attempted; family routines; age; accessibility; and learning issues. Based on outcomes of these assessments, an individualized bowel program is constructed. The algorithm helps the practitioner and patient decide on components and indicators of a successful continence program. The recommended program might include timed toileting, suppository, continence enema, and ACE procedure, or a combination. Evaluation and patient education address adequate fluid/fiber, appropriate toileting equipment, and use of stool softeners/laxatives. Descriptions are available. Key elements in monitoring a continuing plan for continence include: the degree of constipation and its etiology; changing age; family availability for assistance until interdependence is optimal; wheelchair accessibility of the toilet; and ability to transfer to and from the toilet. Use of the algorithm allows for careful decision-making based on information from the patient and family. This has led to greater success in bowel continence in children with spina bifida.
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PMID:Decision-making for a successful bowel continence program. 1121 28

Diabetic autonomic neuropathy (DAN) is a serious and common complication of diabetes. Despite its relationship to an increased risk of cardiovascular mortality and its association with multiple symptoms and impairments, the significance of DAN has not been fully appreciated. The reported prevalence of DAN varies widely depending on the cohort studied and the methods of assessment. In randomly selected cohorts of asymptomatic individuals with diabetes, approximately 20% had abnormal cardiovascular autonomic function. DAN frequently coexists with other peripheral neuropathies and other diabetic complications, but DAN may be isolated, frequently preceding the detection of other complications. Major clinical manifestations of DAN include resting tachycardia, exercise intolerance, orthostatic hypotension, constipation, gastroparesis, erectile dysfunction, sudomotor dysfunction, impaired neurovascular function, "brittle diabetes," and hypoglycemic autonomic failure. DAN may affect many organ systems throughout the body (e.g., gastrointestinal [GI], genitourinary, and cardiovascular). GI disturbances (e.g., esophageal enteropathy, gastroparesis, constipation, diarrhea, and fecal incontinence) are common, and any section of the GI tract may be affected. Gastroparesis should be suspected in individuals with erratic glucose control. Upper-GI symptoms should lead to consideration of all possible causes, including autonomic dysfunction. Whereas a radiographic gastric emptying study can definitively establish the diagnosis of gastroparesis, a reasonable approach is to exclude autonomic dysfunction and other known causes of these upper-GI symptoms. Constipation is the most common lower-GI symptom but can alternate with episodes of diarrhea. Diagnostic approaches should rule out autonomic dysfunction and the well-known causes such as neoplasia. Occasionally, anorectal manometry and other specialized tests typically performed by the gastroenterologist may be helpful. DAN is also associated with genitourinary tract disturbances including bladder and/or sexual dysfunction. Evaluation of bladder dysfunction should be performed for individuals with diabetes who have recurrent urinary tract infections, pyelonephritis, incontinence, or a palpable bladder. Specialized assessment of bladder dysfunction will typically be performed by a urologist. In men, DAN may cause loss of penile erection and/or retrograde ejaculation. A complete workup for erectile dysfunction in men should include history (medical and sexual); psychological evaluation; hormone levels; measurement of nocturnal penile tumescence; tests to assess penile, pelvic, and spinal nerve function; cardiovascular autonomic function tests; and measurement of penile and brachial blood pressure. Neurovascular dysfunction resulting from DAN contributes to a wide spectrum of clinical disorders including erectile dysfunction, loss of skin integrity, and abnormal vascular reflexes. Disruption of microvascular skin blood flow and sudomotor function may be among the earliest manifestations of DAN and lead to dry skin, loss of sweating, and the development of fissures and cracks that allow microorganisms to enter. These changes ultimately contribute to the development of ulcers, gangrene, and limb loss. Various aspects of neurovascular function can be evaluated with specialized tests, but generally these have not been well standardized and have limited clinical utility. Cardiovascular autonomic neuropathy (CAN) is the most studied and clinically important form of DAN. Meta-analyses of published data demonstrate that reduced cardiovascular autonomic function as measured by heart rate variability (HRV) is strongly (i.e., relative risk is doubled) associated with an increased risk of silent myocardial ischemia and mortality. The determination of the presence of CAN is usually based on a battery of autonomic function tests rather than just on one test. Proceedings from a consensus conference in 1992 recommended that three tests (R-R variation, Valsalva maneuver, and postural blood pressure testing)or longitudinal testing of the cardiovascular autonomic system. Other forms of autonomic neuropathy can be evaluated with specialized tests, but these are less standardized and less available than commonly used tests of cardiovascular autonomic function, which quantify loss of HRV. Interpretability of serial HRV testing requires accurate, precise, and reproducible procedures that use established physiological maneuvers. The battery of three recommended tests for assessing CAN is readily performed in the average clinic, hospital, or diagnostic center with the use of available technology. Measurement of HRV at the time of diagnosis of type 2 diabetes and within 5 years after diagnosis of type 1 diabetes (unless an individual has symptoms suggestive of autonomic dysfunction earlier) serves to establish a baseline, with which 1-year interval tests can be compared. Regular HRV testing provides early detection and thereby promotes timely diagnostic and therapeutic interventions. HRV testing may also facilitate differential diagnosis and the attribution of symptoms (e.g., erectile dysfunction, dyspepsia, and dizziness) to autonomic dysfunction. Finally, knowledge of early autonomic dysfunction can encourage patient and physician to improve metabolic control and to use therapies such as ACE inhibitors and beta-blockers, proven to be effective for patients with CAN.
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PMID:Diabetic autonomic neuropathy. 1271 21

Patients may present with anal incontinence (AI) following repair of a congenital anorectal anomaly years previously, or require total anorectal reconstruction (TAR) following radical rectal extirpation, most commonly for rectal cancer. Others may require removal of their colostomy following sphincter excision for Fournier's gangrene, or in cases of severe perineal trauma. Most of the data pertaining to antegrade continence enema (the ACE or Malone procedure) comes from the pediatric literature in the management of children with AI, but also with supervening chronic constipation, where the quality of life and compliance with this technique appears superior to retrograde colonic washouts. Total anorectal reconstruction requires an anatomical or physical supplement to the performance of a perineal colostomy, which may include an extrinsic muscle interposition (which may or may not be 'dynamized'), construction of a neorectal reservoir, implantation of an incremental artificial bowel sphincter or creation of a terminal, smooth-muscle neosphincter. The advantages and disadvantages of these techniques and their outcome are presented here.
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PMID:The antegrade continence enema procedure and total anorectal reconstruction. 2475 42

Purpose: To report the current status of Robotic approach to creation of Catheterisable channel (CC) with the author's personal experience compared to published literature on technical steps, follow up, and outcomes. Methods: CC data was extracted from the prospective database set up for all Robotic pediatric urology procedures performed by the author at his institution. A literature search was then performed to look at the evidence base. Results: Eighteen consecutive cases (8M:7F) of Robotic approach to creation of CC was identified and included. All attempted cases were successfully completed without any conversion to open approach. Median age at surgery was 10.75 years (IQR 6.9-16.5); Median OT 197 min (IQR 131-295) with concomitant procedures in 4 cases. Appendix was used in 14 cases as CC conduit and distal ureter in 4 cases. Median Length of stay (LOS) was 2.75 days (IQR 2-6) and Median FU 27.3 m. Whilst FU duration is comparable to published series, average OT and LOS was much lower in this series. The LOS in this robotic series is much lower than the author's experience with open approach (2.75 vs. 5.8 days). No major complications postoperatively except for one exit site wound infection managed conservatively. None of the CC have been revised in this series and all channels are patent with 12 F or 14 F admissible catheter size. There were no cases of incontinence related to technique of creation of CC and no incidence of exit site stomal stenosis with use of ACE stopper until channel matures and Clean intermittent catheterisation (CIC) is established. Conclusion: Robotic approach to CC is feasible, safe with excellent outcomes and minimum morbidity. Robotic complex bladder reconstructive surgery offers some advantages to children compared to open approach but is only currently performed in few tertiary centers with expertise.
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PMID:Robotic Approach to Creation of Continent Catheterisable Channels-Technical Steps, Current Status, and Review of Outcomes. 3071 32