{"title":"Giant Renal Angiomyolipomas and Pulmonary Lymphangioleiomyomatosis: Follow-up Report after More than a Decade.","authors":"Kiran Nasir, Aasim Ahmad","doi":"10.4103/sjkdt.sjkdt_324_21","DOIUrl":"10.4103/sjkdt.sjkdt_324_21","url":null,"abstract":"<p><p>Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are two major presentations of tuberous sclerosis (TS), an autosomal dominant multisystem disorder. Renal AMLs can lead to life-threatening complications like hemorrhage and cause progressive renal failure requiring dialysis and kidney transplant. mTOR inhibitors showed promising results in TS patients with renal AMLs, LAM, and subependymal giant cell astrocytomas. This case report is a follow-up of a patient we reported in 2010 with giant AMLs and LAM who required an emergency nephrectomy for massive hemorrhage from giant left-sided AMLs.</p>","PeriodicalId":21356,"journal":{"name":"Saudi Journal of Kidney Diseases and Transplantation","volume":"34 6","pages":"671-675"},"PeriodicalIF":0.5,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140898974","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M K Krishnapriya, S Karthika, Anisha Babu, Antriya Annie Tom
{"title":"Individualization of the Dosage Regimen of Erythropoietin is Crucial in End-stage Renal Disease Patients.","authors":"M K Krishnapriya, S Karthika, Anisha Babu, Antriya Annie Tom","doi":"10.4103/1319-2442.397212","DOIUrl":"https://doi.org/10.4103/1319-2442.397212","url":null,"abstract":"<p><p>The objective of this study was to understand the utilization pattern of erythropoietin in end-stage renal disease patients, along with the effect of body weight and sex on the patients' responses. In this retrospective single-center study, 120 patients were included who were on a once weekly (n = 79), twice weekly (n = 37), or thrice weekly (n = 4) regimen. The doses of erythropoiesis-stimulating agents (ESA) were collected, and the erythropoietin resistance index (ERI) was determined. The Kruskal-Wallis test was used to evaluate the dose schedules, and the once-weekly regimen produced a greater response (P = 0.001). The asymptotic significance of Pearson's Chi-square-test equating the mean ERI and body mass index (BMI) was 0.034. No statistically significant correlation was estimated between sex and mean ERI (P = 0.201). Our study demonstrated that the once-weekly regimen dominated over the others in terms of efficacy, and individuals with a higher BMI were found to respond better to the ESA therapy.</p>","PeriodicalId":21356,"journal":{"name":"Saudi Journal of Kidney Diseases and Transplantation","volume":"34 5","pages":"474-481"},"PeriodicalIF":0.5,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141591256","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Relationship between Serum TWEAK Levels and Carotid Intima-media Thickness in Patients with Fabry Disease.","authors":"Kultigin Turkmen, Ismail Baloglu, Talat Aykut, Salih Demir, Ebru Altın, Zeynep Aybike Akguzel, Muhammet Kocabas, Fatma Humeyra Yerlikaya","doi":"10.4103/1319-2442.397202","DOIUrl":"10.4103/1319-2442.397202","url":null,"abstract":"<p><p>Fabry disease (FD) is associated with inflammation, proteinuria, and chronic kidney disease. Tumor necrosis factor-like weak inducer of apoptosis (TWEAK) plays an important role in inflammation in diabetic nephropathy and lupus nephritis. Since there is a close relationship linking serum TWEAK (sTWEAK), inflammation, and carotid intima-media thickness (CIMT) in various kidney diseases, we aimed to determine the relationship between sTWEAK levels and CIMT in subjects with and without proteinuria in a cross-sectional study involving 15 FD patients (seven females, eight males) and seven healthy controls (four females, three males). There were no differences in age, sex, estimated glomerular filtration rate, and biochemical parameters (serum glucose, albumin, creatinine, uric acid, C-reactive protein (CRP), low-density lipoprotein, and high-density lipoprotein) between FD patients and healthy controls. The spot urine protein-creatinine ratios of healthy controls and FD patients were 90 mg/g and 185 mg/g, respectively (P = 0.022). STWEAK levels were higher in FD patients than in healthy controls (P = 0.007). The CIMT of FD patients and healthy controls was 0.55 ± 0.14 mm and 0.42 ± 0.04 mm, respectively (P = 0.007). STWEAK was positively correlated with CRP and CIMT, and negatively with proteinuria (P = 0.005, P = 0.013, and P = 0.018, respectively). In the multivariate analysis, only sTWEAK was an independent variable of increased CIMT. We demonstrated that sTWEAK and CIMT were increased in FD patients. STWEAK might have a role in the pathogenesis of subclinical atherosclerosis in FD.</p>","PeriodicalId":21356,"journal":{"name":"Saudi Journal of Kidney Diseases and Transplantation","volume":"34 5","pages":"406-415"},"PeriodicalIF":0.5,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141591260","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Amina Bekara, Mohammed El Amine Bekara, Lallia Amara, Nassima Amamra
{"title":"Risk Factors of Anxiety among Hemodialyzed Patients in Ténès, Algeria: A Cross-Sectional Study.","authors":"Amina Bekara, Mohammed El Amine Bekara, Lallia Amara, Nassima Amamra","doi":"10.4103/1319-2442.397205","DOIUrl":"https://doi.org/10.4103/1319-2442.397205","url":null,"abstract":"<p><p>This study aimed to evaluate the level of anxiety and its risk factors among patients undergoing hemodialysis in the Ténès area, Chlef Province, Algeria. The survey was conducted on 45 hemodialyzed patients at the public hospital of Ténès (Ahmed Bourass). Sociodemographic data were collected using a questionnaire, and anxiety was diagnosed by the hospital anxiety and depression scale. Among 45 patients, 44.44% were anxious. Anxiety was significantly associated with profession and antidepressant treatment (P <0.05). The other factors were insignificantly related to anxiety, such as age, sex, education level, and comorbidities. Anxiety is prevalent among end-stage renal disease patients. Unemployment and medications were identified as the main risk factors. Multidisciplinary strategies need to be established to prevent the occurrence of anxiety and improve patients' quality of life.</p>","PeriodicalId":21356,"journal":{"name":"Saudi Journal of Kidney Diseases and Transplantation","volume":"34 5","pages":"437-442"},"PeriodicalIF":0.5,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141591258","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"IgG4-Related Diseases Involving Bilateral Renal Pelvises Successfully Treated with Steroid Therapy.","authors":"Illyoung Seo, Youri Kim","doi":"10.4103/1319-2442.397207","DOIUrl":"10.4103/1319-2442.397207","url":null,"abstract":"<p><p>Immunoglobulin (Ig) G4-related diseases are very rare diseases and are difficult to diagnose and treat. Here, we report a rare condition, an IgG4-related disease involving the bilateral renal pelvises, which was successfully treated with steroid therapy. A 64-year-old female was admitted with left flank pain and hematuria. Computed tomographic images showed irregular enhancing masses around the bilateral renal pelvises and lymph nodes in the left para-aortic area. A histopathological examination of a renal needle biopsy revealed numerous cells that were positive for IgG and more than 10 IgG4-positive cells per high-powered field. The IgG4/IgG ratio was more than 40%. Her serum IgG4 level was elevated to 1440 mg/dL, and her IgG4/IgG ratio was 82.1%. After a diagnosis of IgG4 sclerosing disease, a double-J ureteral stent was inserted and steroid therapy was performed for 20 weeks. Her symptoms dramatically improved, and the follow-up images showed a resolved state. There was no relapse of the disease for more than 6 months after the removal of the stent.</p>","PeriodicalId":21356,"journal":{"name":"Saudi Journal of Kidney Diseases and Transplantation","volume":"34 5","pages":"449-454"},"PeriodicalIF":0.5,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141591182","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Improved Arterial Stiffness Indices 3 and 6 Months after Living-donor Renal Transplantation.","authors":"Bhanu Pratap Singh, Vijoy Kumar Jha, Harshit Khurana, Debasish Mahapatra, Shashibhusan","doi":"10.4103/1319-2442.397199","DOIUrl":"https://doi.org/10.4103/1319-2442.397199","url":null,"abstract":"<p><p>Arterial stiffness is a non-traditional risk factor of cardiovascular disease and may explain part of the excess cardiovascular risk in chronic kidney disease patients. Successful renal transplantation (RT) may restore renal function and improve several metabolic abnormalities involved in arterial stiffness. This prospective study conducted non-invasive assessments of arterial stiffness indices [the augmentation index (AI) and pulse wave velocity (PWV)] in end-stage kidney disease (ESKD) patients before RT and 3 and 6 months after living-donor RT, alongside the effects of age and calcineurin inhibitors on arterial stiffness. The study included 26 ESKD patients (22 males and 4 females; mean age, 34.07 years; median duration of dialysis, 10 months) scheduled for RT and followed up for three visits (within 1 week before transplantation, and 3 and 6 months after transplantation). Six months after successful RT, the patients had nearly normal serum creatinine and significantly improved serum phosphate and intact parathyroid hormone levels. The pretransplant AI was 21.53% ± 13.61% which reduced significantly 6 months after RT to 16.19% ± 10.74% (P <0.05). Although there was a reduction in PWV 6 months after RT from the pre-transplant PWV, it was not significant. A significant correlation between age and the augmentation index was noted 3 and 6 months after RT. Patients on tacrolimus-based immunosuppression after RT showed significant improvements in the AI compared with patients on a cyclosporine-based regimen. RT helped to improve arterial stiffness indices, resulting in reduced cardiovascular risk.</p>","PeriodicalId":21356,"journal":{"name":"Saudi Journal of Kidney Diseases and Transplantation","volume":"34 5","pages":"378-388"},"PeriodicalIF":0.5,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141591183","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nimisha Dange, Kiran P Sathe, Alpana Kondekar, Vishal Sawant
{"title":"Effectiveness of Supramaximal Angiotensin-converting Enzyme Inhibition in Controlling Proteinuria in Congenital Nephrotic Syndrome with Cytomegalovirus Infection and an NPHS1 Mutation.","authors":"Nimisha Dange, Kiran P Sathe, Alpana Kondekar, Vishal Sawant","doi":"10.4103/1319-2442.397210","DOIUrl":"https://doi.org/10.4103/1319-2442.397210","url":null,"abstract":"<p><p>Congenital nephrotic syndrome (NS) is characterized by early-onset heavy proteinuria. Most cases of congenital NS are associated with genetic mutations in the podocyte proteins. The causal relationship of perinatal infections with congenital NS has not yet been proven. Inadequate response to the treatment of such infections should prompt us to conduct genetic testing for congenital NS. The heavy proteinuria associated with congenital NS is usually difficult to control with conventional treatment. It often results in progressive kidney disease with a high risk of mortality in early life. Here, we describe an infant who developed congenital NS and was found to have a coexisting Cytomegalovirus infection and an underlying NPSH1 mutation. Proteinuria did not respond to a standard dose of enalapril. A supramaximal dose of enalapril was tried and was effective and safe in controlling the proteinuria. It was associated with improved growth, complete resolution of edema, normal serum albumin, and normal renal function beyond 2 years of age.</p>","PeriodicalId":21356,"journal":{"name":"Saudi Journal of Kidney Diseases and Transplantation","volume":"34 5","pages":"462-467"},"PeriodicalIF":0.5,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141591180","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Assessment of Depression and Its Correlation with Serum Levels of Interleukin-6 in Patients on Maintenance Hemodialysis.","authors":"Shabana Nazneen, Manjusha Yadla, Prabhakar Reddy","doi":"10.4103/1319-2442.397201","DOIUrl":"10.4103/1319-2442.397201","url":null,"abstract":"<p><p>Depression is the most common psychiatric disorder in patients on hemodialysis. An imbalance between proinflammatory and anti-inflammatory cytokines is thought to play a role in the pathogenesis of depression in patients on dialysis. We undertook this study to assess the correlations of the proinflammatory cytokine interleukin 6 (IL-6) and the anti-inflammatory cytokine IL-10 with depression in patients on maintenance hemodialysis. This cross-sectional observational study was carried out at our hospital, a tertiary care referral government teaching hospital, over 2 years. Depression was assessed using the Becks Depression Inventory score. A higher cutoff of >16 was taken for a diagnosis of depression. Clinical, demographic, and laboratory parameters were analyzed. Cytokines were assessed using enzyme-linked immunosorbent assays. Eighty patients satisfied the inclusion criteria. The prevalence of depression in our study was 41.5%. Depression was more common in those who were unemployed and/or illiterate. Mild depression was found in patients from the upper-lower and lower-middle classes, but severe depression was seen in lower-economic classes. Serum levels of IL-6 showed a positive correlation with the severity of depression. Depression was common in patients on maintenance hemodialysis. High levels of serum IL-6 were observed in those with depression. Depression in patients on maintenance hemodialysis is associated with a considerable risk of mortality.</p>","PeriodicalId":21356,"journal":{"name":"Saudi Journal of Kidney Diseases and Transplantation","volume":"34 5","pages":"397-405"},"PeriodicalIF":0.5,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141591178","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Hypernatremia Masking a Case of Primary Sjögren Syndrome-induced Distal Renal Tubular Acidosis.","authors":"Chinmaye Sapre, Abhishek Prajapati, Rohan Parikh","doi":"10.4103/1319-2442.397208","DOIUrl":"10.4103/1319-2442.397208","url":null,"abstract":"<p><p>Distal renal tubular acidosis (dRTA), also known as Type 1 renal tubular acidosis, is a rare disorder. It primarily occurs through the inability to secrete H+ ions. The causes of dRTA can be divided into primary and secondary. The most common secondary cause of dRTA is Sjögren syndrome. dRTA typically presents as hypokalemia with non-anion gap metabolic acidosis. Here, we present a patient where Sjögren's syndrome causing dRTA was masked by the presence of hypernatremia causing metabolic acidosis with a high anion gap.</p>","PeriodicalId":21356,"journal":{"name":"Saudi Journal of Kidney Diseases and Transplantation","volume":"34 5","pages":"455-457"},"PeriodicalIF":0.5,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141591181","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Case Report of Fibrillary Glomerulonephritis with Mild Albuminuria: A Viewpoint on Proteomics.","authors":"Xavier Enrique Guerra-Torres","doi":"10.4103/1319-2442.397209","DOIUrl":"10.4103/1319-2442.397209","url":null,"abstract":"<p><p>Fibrillary glomerulonephritis (FGN) is a rare glomerular disorder characterized by the deposition of randomly arranged fibrils in the mesangium and the glomerular basement membrane. Clinical features include massive albuminuria, hematuria, high blood pressure, and kidney failure. Usually, the renal prognosis is not favorable, with evolution to end-stage renal disease in approximately 50% of cases. Recent studies in proteomics have identified a member of the heat shock protein family, also called DNAJB9, which is deposited in the glomerulus of patients with FGN and is not present in other diseases, such as amyloidosis or immunotactoid glomerulopathy. These findings are the first step to clarify the pathogenesis of this disease and could facilitate its diagnosis. Hence, we present a case of FGN with mild albuminuria at baseline and discuss the usefulness of this novel biomarker for diagnosing this group of patients.</p>","PeriodicalId":21356,"journal":{"name":"Saudi Journal of Kidney Diseases and Transplantation","volume":"34 5","pages":"458-461"},"PeriodicalIF":0.5,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141591177","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}