Autosomal dominant polycystic kidney disease: New insights into treatment

Autosomal dominant polycystic kidney disease (ADPKD) is the world′s most common inherited kidney disease. An increasing number of animal and human studies have enhanced our understanding of the molecular and cellular pathology of ADPKD. New treatment options are being tested in clinical trials in spite of the failure of mammalian target of rapamycin inhibitor therapy. The main and most effective therapy remains control of hypertension by renin-angiotensin-aldosterone system (RAAS) blockade. This review focuses only on promising therapies, including dual inhibition of RAAS, vasopressin receptor antagonists, increased fluid intake, and blockade of certain receptors of cyclic adenosine monophosphate. Also, the paper reviews what these advances mean to patients and clinicians and elaborates on how these changes can be immediately applied to clinical practice. There is an urgent need for discovery of new therapies targeted toward ADPKD in comparison with therapeutic progress of all other renal diseases

Update on pathogenesis, management, and treatment of hypertension in autosomal dominant polycystic kidney disease

Hypertension is a common early finding in autosomal dominant polycystic kidney disease (ADPKD). Improvements in screening and diagnosis of ADPKD have allowed earlier diagnosis, later onset of end-stage renal disease, and better survival. However, the main and most effective therapy remains control of hypertension. Hypertension is the most important modifiable risk factor in ADPKD. Therefore, early management of hypertension reduces the incidence of cardiovascular events in ADPKD patients. Stimulation of the renin–angiotensin–aldosterone system (RAAS) plays a central role in the pathogenesis of hypertension in ADPKD. Therapies that block the RAAS have improved patient management, blood pressure control, and ADPKD patient survival. This review highlights the current understanding of the epidemiology, potential pathogenetic mechanisms and proposes a strategy for the treatment and management of hypertension in ADPKD

Erectile dysfunction in hemodialysis patients

Erectile dysfunction (ED) is a common problem seen among patients on hemodialysis (HD), but it is still a taboo subject in our country. The attention given to this sexual problem remained low, and the prevalence of ED among these patients has not been well characterized. We carried out this study in order to determine the prevalence and severity of ED in HD patients. We conducted a descriptive cross-sectional study in our HD unit in March 2013. ED was evaluated using the International Index Erection Function. Thirty patients with a mean age of 49.1 years were eligible for this study. The main causes of chronic kidney disease were hypertension (62.5%) and diabetes (41.6%). The prevalence of ED was 80%, including 33.3% severe ED. Plasma levels of gonadotropins: luteinizing hormone (LH), follicule-stimulating hormone were in the standards except for one patient who had an elevated level of LH. Prolactin was elevated in four cases. ED was present in 8.4% of patients before the discovery of renal failure and in 91.6% of patients at the beginning of dialysis. For 19 patients (79.1%), the ED had increased during the dialysis sessions. A significant number of our HD patients presented with ED of varying degrees. Nephrologists should pay attention to the problem of ED in order to improve the quality of their life

Efficacy and safety of calcium acetate-magnesium carbonate in the treatment of hyperphosphatemia in dialysis patients

A phosphate binder combining calcium and magnesium offers an interesting therapeutic option to control hyperphosphatemia in dialysis patients. We investigated the effectiveness and tolerance of calcium acetate-magnesium carbonate (Ca-Mg). This is a 16-week prospective study including 16 dialysis patients. After an initial two-week washout period, serum phosphorus (sPho) ≥1.8 mmol/L, serum calcium (sCa) ≤2.6 mmol/L, and serum magnesium ≤1.5 mmol/L were the main inclusion criteria. The initial dose of Ca-Mg depended on sPho level and was titrated for every two weeks to have a sPho ≤ 1.8 mmol/L. A second two-week washout period followed the 12 weeks of treatment. Ca-Mg significantly reduced the mean sPho levels from 2.14 to 1.75 mmol/L by the end of the 12-week treatment period (P <0.006). Two weeks after the completion of the Ca-Mg study, the mean sPho levels increased to 2.2 mmol/L. The mean sCa levels did not significantly change during the Ca-Mg trial. The mean serum intact parathyroid hormone declined significantly from 446 pg/mL at the beginning of the study to 367 pg/mL at the end of the 12-week treatment period (P = 0.0002). Digestive tolerance was good in all patients which allowed good compliance. There were no episodes of hypercalcemia. However, six patients had a moderate hypermagnesemia (21 episodes) requiring adjustment of treatment dose. The Ca-Mg proved to be effective in the control of hyperphosphatemia in dialysis patients with good clinical and biological tolerance. Thus, in patients with hypercalcemia or poor tolerance to calcium carbonate, Ca-Mg might be a good alternative

Renal AA amyloidosis in a patient with hereditary complete complement C4 deficiency

Hereditary complete C4 deficiency has until now been reported in 30 cases only. A disturbed clearance of immune- complexes probably predisposes these individuals to systemic lupus erythematosus, other immune- complex diseases and recurrent microbial infections. We present here a 20- year- old female with hereditary complete C4 deficiency. Renal biopsy demonstrated renal AA amyloidosis. This unique case further substantiates that deficiency of classical pathway components predisposes to the development of recurrent microbial infections and that the patients may develop AA amyloidosis. Furthermore, in clinical practice, the nephrotic syndrome occurring in a patient with hereditary complete complement C4 deficiency should lead to the suspicion of renal AA amyloidosis

Nodular glomerulosclerosis in patients' without history of diabetes mellitus: a case report

INTRODUCTION: Diabetic nephropathy can occur during the course of both type1 and type 2 diabetes mellitus. The characteristic lesions are diffuse or nodular (Kimmelsteil-Wilson) diabetic glomerulosclerosis. The reported cases represent unusual presentations of diabetes mellitus. CASE PRESENTATION: We report the case of a 49-year-old man without prior history of diabetes mellitus who presented with rapidly progressive renal failure and whose renal biopsy revealed nodular (Kimmelsteil-Wilson) glomerulosclerosis lesions characteristic of diabetes. CONCLUSION: Renal manifestations of diabetes mellitus may antedate other more common presenting symptoms of this disease and we critically review the literature on this subject

Acute rejection episodes after kidney transplantation

Acute rejection episodes (AREs) are a major determinant of renal allograft survival. The incorporation of new immunosuppressive agents explains, at least partially, the improvement seen in the results of transplantation in recent years. The objectives of this study are to analyze the incidence and severity of AREs, their risk factors and their influence on graft and patient survival. We retrospectively studied 280 kidney transplants performed in adults at the Charles Nicolle Hospital, Tunis, between 1986 and 2004. The diagnosis of ARE was based on clinical data and response to treatment. Allograft biopsies were performed in ten cases. The treatment of AREs consisted of pulse methylprednisolone and anti-thymocyte globulin. There were 186 males (66.4&#x0025;) and 94 females (33.6&#x0025;), and their mean age was 31 &#x00B1; 8.9 years. Overall, the 280 study patients experienced a total of 113 AREs. Of them, 85 had only one ARE, 28 had two to three and none had more than three AREs. A total of 68 AREs were completely re-versible, 42 were partially reversible while three could not be reversed with treatment. The mean inci-dence of AREs was 40.4&#x0025;. The incidence was &gt; 45&#x0025; between 1986 and 1997, decreased to 20.5&#x0025; between 1998 and 2000 and to 9&#x0025; between 2001 and 2004. Graft survival rates in patients with and without AREs were respectively 91&#x0025; and 93&#x0025; at three years, 82&#x0025; and 90&#x0025; at five years and 73&#x0025; and 83&#x0025; at 10 years. We found a decrease in the incidence of AREs in recent years in our study patients, and this was related to the introduction of sensitized cross-match and the newer immunosuppressive agents, particularly MMF. Additionally, AREs had a deleterious impact on late graft survival in our study population