Association of multiple retinal nodular hamartomas and "confetti" skin lesions with end-stage renal disease in patients with tuberous sclerosis.

PURPOSE: The main purpose of this study is to explore characteristics of patients with chronic kidney disease in tuberous sclerosis (TSC) and to underline differences in clinical characteristics between end-stage renal disease (ESRD) patients and patients in earlier stages of chronic kidney disease. METHODS: This multicentric, retrospective study included data for 48 patients from seven South-Eastern European countries (Albania, Bosnia and Herzegovina, Croatia, Greece, Montenegro, Serbia, Slovenia) in the period from February to August 2020. Researchers collected data from local and national nephrological and neurological registries and offered clinical and laboratory results from medical histories in follow-up periods. RESULTS: This study enrolled 48 patients with a median age of 32.3 years (range, 18-46 years), and predominant female gender (60.45%). The percentage of patients with chronic kidney disease (CKD) diagnosis of the total number of patients was 66.90%, with end-stage renal disease development in 39.6%. The most prevalent renal lesions leading to chronic kidney disease were angiomyolipomas (AMLs) in 76.6%, while multiple renal cysts were present in 42.6% of patients. Nephrectomy was performed in 43% of patients, while the mTOR inhibitors were used in 18 patients (37.5%). The majority of patients had cutaneous manifestations of tuberous sclerosis-83.30% had hypomelanotic cutaneous lesions, and 68.80% had angiofibromas. Multiple retinal nodular hamartomas and "confetti" skin lesions were more frequent in end-stage renal disease (ESRD) than in patients with earlier stages of chronic kidney disease (p-0.033 and 0.03, respectively). CONCLUSION: Our study has also shown that retinal hamartomas and "confetti" skin lesions are more frequent in end-stage renal diseases (ESRD) patients than in other chronic kidney disease (CKD) patients. Usage of mTOR inhibitors can also reduce the number of complications and associated with tuberous sclerosis, such as dermatological manifestations and retinal hamartoma, which are more common in the terminal stage of chronic kidney disease.

Inflammatory Bowel Diseases in Renal Transplantat Recipients: A Case Series and Review of the Literature.

Inflammatory bowel diseases are autoimmune disorders affecting the gastrointestinal tract and producing a wide variety of extraintestinal manifestations. Kidneys are a rare target organ of their extraintestinal activity, but if affected, renal function could deteriorate to end-stage kidney disease, which is curable only by organ transplantation. Renal calculi are the most common pathological kidney manifestation in IBD patients, followed by tubulointerstitial nephritis, glomerulonephritis, and other kidney pathologies. The liver is the most commonly transplanted organ in IBD patients (primary sclerosing cholangitis and autoimmune hepatitis), and a scarcity of literature on kidney recipients is present to date regarding the incidence of renal insufficiency, kidney transplantations, post-transplant IBD course and further complications such as graft rejection or infections in this specific group of patients. De novo IBD is a paradoxical entity in the setting of rigorous post-transplant immunosuppression. In this case series, we present three patients who underwent kidney transplantation with a history of an IBD and one patient who developed de novo Crohn's disease after the deceased donor organ transplant was performed.

Malnutrition inflammation score (MIS) is stronger predictor of mortality in hemodialysis patients than waist-to-hip ratio (WHR)-4-year follow-up.

PURPOSE: The main purpose of this study is to correlate the significance of the malnutrition inflammation score (MIS) and the waist-to-hip ratio (WHR) in the prediction of mortality in hemodialysis patients. METHODS: The study was conducted from March 2016 to April 2020, covering 100 hemodialysis patients. Anthropometric measures (Waist circumference (WC), Hip circumference (HC), Waist-to-hip ratio (WHR), Caliper and BMI) and biomarkers of inflammation (IL6, hsCRP) and nutrition (Transfferin, Albumin) were determined and for the above-mentioned biomarkers, we monitored all-cause mortality for 4 years. The hemodialysis patients were divided in three groups based on their MIS score. RESULTS: In this prospective, longitudinal study, we enrolled 100 patients (54 males and 46 females) with a median age of 58 (51-65) years. All patients were divided into three groups according to MIS score values. We performed univariate Cox regression survival analysis for a period of 4 years, and then included for multivariate survival Cox regression analysis well-defined nutritional markers: BMI, mid-arm circumference, WHR and MIS score. The MIS score was demonstrated to be the best independent predictor of 4-year mortality in our study (p < 0.001). CONCLUSION: The results of our study have shown that there is no significance of WHR in the prediction of mortality in hemodialysis patients, but that the MIS score is a strong, independent predictor of all-cause 4-year mortality.

[Anesthesiological approach to patients with chronic kidney disease]. / Anestezioloski pristup bolesniku s kronicnom bubreznom bolesti.

Anesthesia in patients with chronic kidney disease (CKD) represents a challenge for anesthesiologists and other specialists in the perioperative team. There is a high incidence of CKD among patients preoperatively, especially in elderly population. For anesthesiologists, it is an imperative to understand the pathophysiology of CKD, prevention of further kidney damage and its complications. This requires experienced anesthesiologist, careful preoperative patient assessment, recognition and modification of the potential risk factors in order to improve patient outcome. Priorities for successful prevention of kidney damage are appropriate fluid distribution peri- and intraoperatively, maintenance of euvolemia, and avoiding hypotension.

Kidney transplantation from deceased donors with high terminal serum creatinine.

The increasing number of possible recipients for kidney transplantation and relatively unchanged number of organ donors has led to consideration of alternative strategies and expansion of deceased donor criteria in order to expand donor pool. Previously, kidneys from expanded criteria donors (ECD) were strongly underestimated because of the conventional opinion suggesting these kidneys to have a higher rate of preservation injury, delayed graft function, rejection and nonfunction. Reducing the difference between graft outcome in patients transplanted from ECD and standard criteria donor (SCD) is one of the goals of many respectable kidney transplantation centers. This assignment includes major concern about reduction of cold ischemia time, recipient selection, novel and adapted immunosuppressive regimens, increased nephron mass by dual kidney transplantation, and using histologic criteria for marginal donor graft selection. There are not many reports on the outcome of kidneys transplanted from donors with acute renal failure and high terminal creatinine. This review presents the exact definition of marginal donor, especially donor with acute renal failure. The management of such grafts during preimplantation and implantation period, outcomes and post-transplantation care are the main assignments for transplantation teams. Recipients of such grafts should be well informed about the possibilities and potential complications and sign their informed consent thereafter. Some respectable studies have shown that under certain, highly controlled conditions, these kidneys can be used safely, with excellent short- and long-term outcomes.

[End-stage renal disease in elderly]. / Zavrsni stadij zatajenja bubrega u starijih osoba.

The balance and quality of different renal replacement treatment modalities used in the elderly with end-stage renal disease vary between countries depending on economic resources, distribution of renal units, number of specialists, and patterns of reimbursement of both hospitals and physicians. Elderly patients with end-stage renal disease need detailed assessment of medical, psychological, motor, and social factors in order to choose an appropriate renal replacement treatment option. Presence or absence of significant comorbidity is much more important than the chronological age. The choice between hemodialysis and continuous ambulatory peritoneal dialysis is largely dependant on preferences of the local team and the patient. Patients with adequate cardiovascular systems are generally considered to be more suitable for hemodialysis. Hemoglobin should be optimized (Hb between 100 and 120 g/L) in all patients. Poor cardiac status and/or angina will require assessment, medical treatment, and, if necessary, surgical treatment or angioplasty. Transplantation should be considered in all reasonably fit and carefully selected patients older than 65 or even 70 years. Only the permanent shortage of suitable kidneys limits our ability to treat all those who could benefit from this type of treatment. Renal transplant recipients may benefit in terms of both survival and quality of life even if older than 70 years and beyond. Immunosuppressive therapy in elderly patients should be moderate.

[A role of WNT in kidney development and function]. / Uloga WNT u razvoju i funkciji bubrega.

WNT 4 is a secreted glycoprotein that is critical for nephrogenesis during mesenchymal to epithelial transformation. Lately there are some experimental modles witch confirm a role of Wnt 4 during regeneration process in acute renal failure. On the other hand there are some evidence that Wnt 4 plays important role in renal fibrosis during experimental renal injury in rats that provide tubuloinerstitial fibrosis. When will Wnt 4 have a protective role or when will induce fibrosis still is not known and therefore futher studies will be necessary to gain a more precise understanding.

[Renal transplantation in the elderly: characteristics of donor and recipient]. / Transplantacija bubrega kod starijih osoba--vaznost obiljezja darivatelja i primatelja.

Aging is a natural process that occurs in all tissues and organs resulting in a decreasing functional capacity. Aging of the population results in an increased number of elderly patients who require replacement of renal function. Renal transplantation is the method of choice for this group of patients if they have no contraindications for immunosuppressive therapy. The lack of donors is the main obstacle for renal transplantation. However, the use of organs from elderly donors for transplantation in elderly recipients is an appropriate method of renal replacement therapy in this group of patients.

[Expression of bone morphogenetic proteins in kidneys]. / Izrazaj kostanih morfogenetskih proteina u bubrezima.

Bone morphogenetic proteins (BMPs) are a large group of growth factors. More than 20 members of BMP family have been identified to date. Based on their structural and functional properties, BMPs can be divided into 4 subgroups: BMP 2/4, BMP 5/6/7/8a/8b, BMP 9/10 and BMP 12/13/14. Each BMP has a unique structural feature that differentiates it from all other family members. BMPs take part in different stages of kidney development. For example, BMP-2 inhibits branching morphogenesis in ureteric buds; BMP-4 is expressed in metanephritic mesenchyme along the Wolff canal before kidney development, its expression is also found in the mesenchyme surrounding the ureteric bud before the invasion of the mesenchymal cells in the ureteric stem and induction completion; BMP-7 is the only bone morphogenetic protein that is crucial in kidney development. BMP family members are also included in maintaining normal kidney structure and function. Experimental models have shown that BMP-7 prevents ischemia/reperfusion damage. Expression of BMP-7 is reduced in the samples of kidney tissue with diabetic nephropathy. Also, BMP-7 mRNA is reduced in clear cell kidney carcinomas, indicating its protective effects in maintaining normal kidney structure and function. Bone morphogenetic proteins are some of the key players in regulating normal kidney development, but their role also extends into maintaining normal kidney structure and function.