scholarly journals Encapsulating Peritoneal Sclerosis: Pathophysiology and Current Treatment Options

2019 ◽  
Vol 20 (22) ◽  
pp. 5765 ◽  
Author(s):  
Rajesh M. Jagirdar ◽  
Andreas Bozikas ◽  
Sotirios G. Zarogiannis ◽  
Maria Bartosova ◽  
Claus Peter Schmitt ◽  
...  

Encapsulating peritoneal sclerosis (EPS) is a life-threatening complication of long-term peritoneal dialysis (PD), which may even occur after patients have switched to hemodialysis (HD) or undergone kidney transplantation. The incidence of EPS varies across the globe and increases with PD vintage. Causative factors are the chronic exposure to bioincompatible PD solutions, which cause long-term modifications of the peritoneum, a high peritoneal transporter status involving high glucose concentrations, peritonitis episodes, and smoldering peritoneal inflammation. Additional potential causes are predisposing genetic factors and some medications. Clinical symptoms comprise signs of intestinal obstruction and a high peritoneal transporter status with incipient ultrafiltration failure. In radiological, macro-, and microscopic studies, a massively fibrotic and calcified peritoneum enclosed the intestine and parietal wall in such cases. Empirical treatments commonly used are corticosteroids and tamoxifen, which has fibrinolytic properties. Immunosuppressants like azathioprine, mycophenolate mofetil, or mTOR inhibitors may also help with reducing inflammation, fibrin deposition, and collagen synthesis and maturation. In animal studies, N-acetylcysteine, colchicine, rosiglitazone, thalidomide, and renin-angiotensin system (RAS) inhibitors yielded promising results. Surgical treatment has mainly been performed in severe cases of intestinal obstruction, with varying results. Mortality rates are still 25–55% in adults and about 14% in children. To reduce the incidence of EPS and improve the outcome of this devastating complication of chronic PD, vigorous consideration of the risk factors, early diagnosis, and timely discontinuation of PD and therapeutic interventions are mandatory, even though these are merely based on empirical evidence.

Author(s):  
Sabrina Giglio ◽  
Giovanni Montini ◽  
Francesco Trepiccione ◽  
Giovanni Gambaro ◽  
Francesco Emma

AbstractRenal tubular acidosis (RTA) comprises a group of disorders in which excretion of hydrogen ions or reabsorption of filtered HCO3is impaired, leading to chronic metabolic acidosis with normal anion gap. In the current review, the focus is placed on the most common type of RTA, Type 1 RTA or Distal RTA (dRTA), which is a rare chronic genetic disorder characterized by an inability of the distal nephron to secrete hydrogen ions in the presence of metabolic acidosis. Over the years, knowledge of the molecular mechanisms behind acid secretion has improved, thereby greatly helping the diagnosis of dRTA. The primary or inherited form of dRTA is mostly diagnosed in infancy, childhood, or young adulthood, while the acquired secondary form, as a consequence of other disorders or medications, can happen at any age, although it is more commonly seen in adults. dRTA is not as “benign” as previously assumed, and can have several, highly variable long-term consequences. The present review indeed reports and summarizes both clinical symptoms and diagnosis, long-term outcomes, genetic inheritance, epidemiology and current treatment options, with the aim of shedding more light onto this rare disorder. Being a chronic condition, dRTA also deserves attention in the transition between pediatric and adult nephrology care, and as a rare disease it has a place in the European and Italian rare nephrological diseases network.


2019 ◽  
Vol 1 (1) ◽  
pp. 16-38
Author(s):  
Mosaad A. Abdel-Wahhab ◽  
Aziza A. El-Nekeety ◽  
Soher E. Aly

Mycotoxins are ubiquitous compounds found in the natural life cycle of food- producing plants. They have a range of diverse chemical and physical properties and toxicological effects on man and animal. Mycotoxins are considered the most important contaminants of the food chain due to their chronic adverse effects on health and the economy. Mycotoxins are known as the 21th century “Great Masquerader” due to its complex natural history involving different tissues and resembling different diseases at each stage in its evolution. Mycotoxins can induce a variety of clinical symptoms including epistaxis, conjunctivitis, coughing, apnea, wheezing, vomiting and nausea. Some mycotoxins induce acute pulmonary hemorrhage, bone marrow failure and pneumonia. Knowledge about these symptoms enables the clinician to ask questions for possible exposure to the main classes of mycotoxins to protect children from sources of such exposure. These sources may include food, clothes, furniture and indoor air at home. Early childhood exposure to mycotoxins may be critical determinants of later health effects. Exposure in utero and through early infancy may additionally be important. Several well-known diseases such as neural tube defects, liver and esophageal cancers are associated with the consumption of mycotoxin-contaminated food. Knowledge of previous short or long term exposure to mycotoxins may help paediatricians to more accurately diagnose and provide treatment options to children and their families. The current review discusses the problems associated with the occurrence of different common mycotoxins in children’s food and the possible halal strategies to counteract these problems.  


Neurosurgery ◽  
2007 ◽  
Vol 60 (6) ◽  
pp. 1059-1066 ◽  
Author(s):  
Dongsheng Guo ◽  
Kai Shu ◽  
Rudong Chen ◽  
Changshu Ke ◽  
Yanchang Zhu ◽  
...  

Abstract OBJECTIVE The aim of this study was to investigate the microsurgical results of symptomatic sacral perineurial cysts of 11 patients and to discuss the treatment options of the past 10 years. METHODS We retrospectively reviewed the records of 11 patients with symptomatic sacral perineurial cysts who underwent microsurgical treatment at Tongji Hospital, Huazhong University of Science and Technology from 1993 through 2006. The philosophy was to perform total or partial cyst wall removal, to imbricate the remaining nerve sheath if possible, and to repair local defect with muscle, Gelfoam (Pharmacia & Upjohn, Kalamazoo, MI), and fibrin glue. Patient outcomes were assessed by comparing the preoperative and postoperative examination results. The average follow-up time obtained from return visits to the neurosurgery clinic or by telephone questionnaires ranged from 2 months to 13 years. A literature search and analysis of current treatment options were performed. RESULTS Nine of the 11 patients (82%) experienced complete or substantial relief of their preoperative symptoms. One patient (Patient 4) experienced worsening of bladder dysfunction after surgery and recovered slowly to subnormal function during the subsequent 2 months. The symptoms of Patient 9 did not resolve, and magnetic resonance imaging showed that the cyst had reoccurred. The patient underwent reoperation 3 months later without any improvement. One patient (Patient 11) experience a cerebrospinal fluid leakage complication. Neither new postoperative neurological defects nor infection were observed in our series. In the literature, there are six different treatment options under debate and controversially discussed. CONCLUSION Microsurgical treatment yielded the best long-term resolution of patient symptoms to date and should be recommended to appropriately selected patients.


2022 ◽  
Vol 18 ◽  
Author(s):  
Bart. W. Driesen ◽  
Michiel Voskuil ◽  
Heynric B. Grotenhuis

Abstract: The Fontan operation was introduced in 1968. For congenital malformations where biventricular repair is not suitable, the Fontan procedure has provided a long-term palliation strategy with improved outcome compared to the initially developed procedures. Despite these improvements, several complications merely as a result of a failing Fontan circulation (including myocardial dysfunction, arrhythmias, increased pulmonary vascular resistance, protein losing enteropathy, hepatic dysfunction, plastic bronchitis and thrombo-embolism) will limit life-expectancy in this patient cohort. This review provides an overview of the most common complications of the Fontan circulation and the currently available treatment options.


2019 ◽  
Vol 11 (3) ◽  
Author(s):  
Tristan Juhan ◽  
Michael Stone ◽  
Omid Jalali ◽  
Will Curtis ◽  
John Prodromo ◽  
...  

Rotator cuff disease is one of the most common causes of shoulder pain, yet controversy still exists regarding treatment of “irreparable” tears. Nonoperative management, including physical therapy and steroid injections, should be reserved for those without significant pain or functional impairment. Debridement may be used for low-demand patients, and should be performed with partial cuff repair, subacromial decompression, and/or acromioplasty to maximize outcomes. Biceps tenotomy and/or tenodesis have been shown to reduce postoperative pain and improve satisfaction when performed in conjunction with rotator cuff repairs, with no difference in functional outcome comparatively. Tendon transfers have been advocated with the potential benefit to improve function and decrease pain. More recently, extracellular matrix and human-derived dermal allografts have been used off-label as patch grafts in irreparable tears. Superior capsular reconstructive techniques and subacromial balloon spacers serve a similar function by acting to depress the humeral head in a cuff-deficient shoulder, however long-term data is needed before widespread adoption of these procedures. Finally, reverse shoulder arthroplasty serves as a salvage option for low demand elderly patients.


Toxins ◽  
2020 ◽  
Vol 12 (3) ◽  
pp. 161 ◽  
Author(s):  
Nadine Kaesler ◽  
Anne Babler ◽  
Jürgen Floege ◽  
Rafael Kramann

Cardiac remodeling occurs frequently in chronic kidney disease patients and affects quality of life and survival. Current treatment options are highly inadequate. As kidney function declines, numerous metabolic pathways are disturbed. Kidney and heart functions are highly connected by organ crosstalk. Among others, altered volume and pressure status, ischemia, accelerated atherosclerosis and arteriosclerosis, disturbed mineral metabolism, renal anemia, activation of the renin-angiotensin system, uremic toxins, oxidative stress and upregulation of cytokines stress the sensitive interplay between different cardiac cell types. The fatal consequences are left-ventricular hypertrophy, fibrosis and capillary rarefaction, which lead to systolic and/or diastolic left-ventricular failure. Furthermore, fibrosis triggers electric instability and sudden cardiac death. This review focuses on established and potential pathophysiological cardiorenal crosstalk mechanisms that drive uremia-induced senescence and disease progression, including potential known targets and animal models that might help us to better understand the disease and to identify novel therapeutics.


2001 ◽  
Vol 86 (07) ◽  
pp. 356-365 ◽  
Author(s):  
Lina Badimon

SummaryAtherothrombosis defines the occurrence of thrombosis on athero-sclerotic lesions. Atherosclerosis is the most prevalent disease of our time and its thrombotic complications are responsible for an exceedingly high number of deaths and disabilities. Over the past few years, experimental investigation and clinical and pathologic observations have led to a better understanding of how a thrombus forms and also of its incidence in acute ischemic syndromes. A thrombus is usually found secondary to atherosclerotic plaque disruption. Mural thrombosis, also at the site of plaque rupture, is an important mechanism in the progression of atherosclerosis even when symptoms are absent. Because atherosclerosis is a silent and asymptomatic disease until complications arise with thrombosis producing clinical symptoms, it is necessary to have models that reproduce the human disease in its early stages. Unfortunately, not all the experimental models of vascular disease have human resemblance and validity. Knowledge of the disease process and of what an experimental animal model can offer is a milestone for a successful investigation. Experimental models of vascular disease have enhanced our understanding of the pathophysiological processes leading to vascular obstruction in both spontaneous and accelerated atherosclerosis and thrombosis. Animal models have provided insight into the role of platelets, lipids, renin-angiotensin system (RAS), cytokines and growth factors in the evolution and progression of atherosclerosis and have suggested potential therapeutic interventions. Significant advances in our understanding of the vascular biology and pathology of atherosclerosis and thrombosis, and of the interactions of blood cells, lipids and proteins with the vascular wall, have allowed us to formulate new experimental hypotheses and to test therapeutic strategies, either pharmacological or surgical.


2019 ◽  
Vol 8 (3) ◽  
pp. 75-79
Author(s):  
Natasha Povey ◽  
Dr Fiona Curtis

Obesity is a major public health concern: in the United Kingdom (UK) over two thirds of the population are obese or overweight, the prevalence of obesity is growing exponentially, and current treatment options have limited success – making the need for novel therapies vital. Brown adipose tissue (BAT) has the ability to safely dissipate chemical energy as heat and in 2009 was found to be active in human adults, leading to hope that its therapeutic manipulation could contribute to weight loss. This review discusses methods proposed for BAT activation and potential pitfalls in our current understanding to evaluate if BAT can be used as a future treatment for obesity. To date, ơ3-adrenergic receptor (ơ3-AR) agonists and cold activation have been shown to be the most promising options to activate BAT. However, cold activation requires a high degree of patient cooperation and ơ3-AR agonists appear non-effective long-term. Nonetheless, ơ3-AR agonists are likely to be a more realistic treatment than cold activation, making our next challenge to understandand mitigate the mechanisms that inhibit BAT activation in long-term ơ3-AR agonist administration. Our assumptions about BAT activation are predominately from rodent studies and based on measurements from [18F]-fl uorodeoxyglucose (18F-FDG)-positron-emission tomography and computed tomography (PET/CT) imaging, both with their respective limitations. BAT has offered huge insight into weight homeostasis, with the potential of offering prospective therapeutics for obesity and beyond. Nevertheless, before we can truly understand the real possibilities of BAT we need to further our current understanding of the physiological controls of BAT, potentially leading to more suitable therapies. The focus for future research should be to improve and standardise the methodology used to measure BAT activation, enabling larger clinical trials and better comparisons.


2021 ◽  
Author(s):  
Kantida Koysombat ◽  
Atikah Haneef

Short bowel syndrome (SDS) is a malabsorption disorder caused by loss of function of the small intestine, either by trauma or innately. Current treatment options include parenteral nutrition (PN) or allograft transplants. Long term PN dependence can lead to complications due to line infections and toxicity from the formula itself. A lack of healthy donors results in long waiting lists and high mortality rates. With allograft transplants, long-term graft and patient survival rates are poor (48% and 39% respectively at 5 years); graft loss occurs due to rejection (48%), thrombosis (28%), sepsis (12%); main causes of death are due to bacterial infection (94%) and rejection. Costs associated with PN annually per patient approximate to GBP40,000, whereas one allograft procedure costs approximately GBP80,000; not including intervention due to complications. Interest in developing an off-the-shelf bioengineered alternative have been expressed. Autologous transplants could be a more beneficial route to improving survival rates, enabling the transplant of patients healthy cells back to them. We describe here the development of a synthetic poly(ethylene terephthalate) scaffold using electrospinning, which showed excellent physical and chemical characteristics; high surface area:volume ratio, high mechanical strength, high porosity, and the ability to be chemically/physically functionalised without losing integrity in structure and physical properties. The cost of electrospinning is far lower in comparison to the current available treatment options, potentially providing a stable, off-the-shelf, ready-to-culture product as the need arises for applications in tissue engineered small intestine (TESI), or 3D models for small bowel research.


Author(s):  
Ulrich Mehnert

This chapter provides an overview of the pathophysiological basis of bladder, bowel, and sexual dysfunction, relevant therapy principles, and treatment options. These dysfunctions are frequent sequelae of neurotrauma and neurodegenerative diseases that require adequate management and treatment. Knowledge of basic neurophysiological and pathophysiological processes involved is important for understanding and interpretation of clinical symptoms and findings and for understanding current treatment concepts. Treatment is mandatory and therapy options range from simple conservative measures to major surgery. The main therapeutic principles include protection of kidney function, reduction of urinary and/or faecal incontinence, independent management of bladder and bowel function, ability to sustain a satisfactory sexual relationship, fertility support, and improvement of quality of life. To comply with such principles and to select, initiate, maintain, and eventually adapt the ‘optimal’ treatment regimen for each patient requires a specialized multidisciplinary team.


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