scholarly journals Causes of The Nephrotic Syndrome In Sweden 2014-2019 Depending On Clinical Presentation And Demographics

Author(s):  
Anneli Jönsson ◽  
Thomas Hellmark ◽  
Mårten Segelmark ◽  
Anna Forsberg ◽  
Karl Dreja

Abstract Background: Many different pathological processes can affect the integrity of the glomerular capillary wall and cause massive leakage of protein resulting in the Nephrotic Syndrome (NS). The prognosis and response to therapy differs depending on diagnosis, but renal biopsy cannot always be performed promptly. These is insufficient knowledge to which extent clinical parameters can predict the diagnosis. Methods: Age, gender, haematuria, proteinuria, plasma creatinine, plasma albumin and final diagnosis were retrieved for all adult patients with NS as indication for biopsy or massive albuminuria in conjunction with a low plasma albumin from the biopsy module of the Swedish Renal Registry (SRR) between 2014 and 2019. A basic calculator was developed to demonstrate the importance of clinical presentations in relation to the likelihood of having a specific diagnosis.Results: 913 unique patients were included in the study. Overall membranous nephropathy (17%) was the most common diagnoses, but when studying those <50 years old or women minimal change nephropathy (21 and 17 %) was the most frequent diagnosis. When examining those between 50 and 70 years-old, those with chronic kidney disease (CKD) 4 and those with negative dipstick tests for hematuria diabetic nephropathy (23, 30 and 21 %) was the most common underlying disease. Among those with high grade hematuria (grade 3-4 on dipsticks) Membranoproliferative glomerulonephritis was most common (14%), closely followed by IgA nephropathy (13%). Focal segmental glomerulosclerosis (9.7%) was less common than in many comparable studies. Conclusions: Clinical parameters have a profound impact on the likelihood of different diagnoses in adult patients with NS. Differences in clinical practice, inclusion criteria in studies and probably genetic background are important to account for when comparing data from different parts of the world.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4320-4320
Author(s):  
Edward Agura ◽  
R. Brian Berryman ◽  
Luis Pineiro ◽  
Mirjana Tadic-Ovcina ◽  
Ryan Woelfel ◽  
...  

Abstract BACKGROUND: Allogeneic transplantation, a treatment previously reserved only for healthy younger patients, can be modified by reducing the intensity and toxicity of the chemotherapy regimens, to allow older patients to receive this treatment safely. Such reduced intensity transplant (RIT) regimens are now in common use in patients through age 70, but relapse of the underlying disease (such as leukemia or lymphoma) remains a frequent cause of post-transplant mortality. To address this issue, we proposed and tested a “mid-intensity” conditioning regimen using the nucleoside analogue clofarabine (CLO) with busulfan (BU). We hypothesized that this combination would be well tolerated and offer greater anti-leukemic efficacy than existing RIT regimens. METHODS: To date, thirteen patients have been enrolled on this single institution, phase II, IRB-approved trial. The diagnoses at the time of transplant were AML(n=6), ALL (n=1), MDS (n=1) and MDS transforming to AML (n=5). 9 of 13 patients had prior therapies, 1 with 3 lines, 4 with 2 lines, and 4 with 1line. The conditioning regimen consisted of CLO 40mg/m2/day iv daily d-8 through d-4, BU 3.2 mg/kg/day iv daily d-3 and -2, followed by a day of rest. Donor cells were infused on day 0. Donors were matched at HLA A, B, C, DR and DQ using DNA sequence-based typing or mid-resolution DNA-based typing. Mismatch at 1 allele was permitted and occurred for 1 donor. GVHD prophylaxis consisted of oral Tacrolimus and MTX 5mg/m2 iv d+1, +3 and +6. BU and CLO pharmacokinetic samples were collected on all patients for subsequent analysis. RESULTS: Study endpoints included toxicity, incidence and severity of acute graft vs. host disease (AGVHD), and response to therapy. Grade 4 hematologic toxicity was observed in all patients. All patients had hematologic nadirs lasting 9–15 days. GCSF was used in 11 of 13 subjects. The median time to recovery of ANC (defined as 3 days &gt;500/ul) was 13 days. Donor engraftment (defined as &gt;80% donor chimerism at d30) occurred in all patients based on FISH and/or chromosomal analysis (STR-short tandem repeats). There were no cases of hepatic, cardiac, or renal toxicity attributed to the conditioning regimen by d60. Hand/foot syndrome occurred in two patients; one of these was the only incidence of grade 3 toxicity considered to be definitely related to clofarabine. One subject suffered respiratory failure (grade 3) of unknown etiology that resolved completely and was deemed to be possibly related to the treatment. Grade 4 toxicities attributed to the conditioning regimen were not seen in this study. There were no transplant related mortalities. Milder acute toxicities considered definitely or probably related to the regimen included: fluid retention/edema, n=3 (23%) (2 grade 1 and 1 grade 2); nausea/vomiting n=6(46%) (5 − grade 1 and 1 − grade 2); skin rash, n=3(23%) (all grade 1); erythroderma, n=1(8%) (grade 1); elevated transaminases, n= 4(31%) (3 − grade 1 and 1 − grade 2); elevated ALP (alkaline phosphatase), n=1(8%) (grade 1); elevated total bilirubin n=1(8%) (grade 1); diarrhea, n=1(8%) (grade 1). Acute GVHD (AGVHD) occurred in 10 of 12 (83%) evauable patients within the first 100 days. Of these, 90% were grade 1–2 and 10% grade 3–4. No patients died as a result of AGVHD. The disease responses are as follows: 9 (75%) patients who were refractory, in relapse, or with active disease (n= 4, 1, 4) prior to initiation of CLO/BU, achieved a complete remission by d30. 3 (25%) patients were in remission at initiation of clofarabine, and all were in continued complete remission at d30. One patient was not evaluable for disease response at d30 due to illness. Relapse occurred in 4 (33%) patients at d62, d60, d120, and d335. Of these patients, 2 were refractory, 1 was in relapse and 1 was in CR at baseline. Currently, 8 (67%) patients remain in remission with a median follow up of 208 days. CONCLUSIONS: CLO/BU is a mid-intensity allogeneic transplant conditioning regimen with promising antileukemic efficacy that seems to be well tolerated without significant cardiac, renal or pulmonary toxicities. Furthermore, CLO/BU resulted in full allogeneic engraftment by day 30 in all patients. The study continues to accrue patients and will be updated at the meeting.


2021 ◽  
Vol 2021 ◽  
pp. 1-12
Author(s):  
Hanchang He ◽  
Minwa Lin ◽  
Lu You ◽  
Tongqing Chen ◽  
Zijie Liang ◽  
...  

Background. Increasing evidences have reported gut microbiota dysbiosis in many diseases, including chronic kidney disease and pediatric idiopathic nephrotic syndrome (INS). There is lack evidence of intestinal microbiota dysbiosis in adults with INS, however. Here, we to address the association between the gut microbiome and INS. Methods. Stool samples of 35 adult INS patients and 35 healthy volunteers were collected. Total bacterial DNA was extracted, and the V4 regions of the bacterial 16S ribosomal RNA gene were sequenced. The fecal microbiome was analyzed using bioinformatics. The correlation analysis between altered taxa and clinical parameters was also included. Results. We found that microbial diversity in the gut was reduced in adult patients with INS. Acidobacteria, Negativicutes, Selenomonadales, Veillonellaceae, Clostridiaceae, Dialister, Rombousia, Ruminiclostridium, Lachnospira, Alloprevotella, Clostridium sensu stricto, Megamonas, and Phascolarctobacterium were significantly reduced, while Pasteurellales, Parabacteroides, Bilophila, Enterococcus, Eubacterium ventriosum, and Lachnoclostridium were markedly increased in patients with INS. In addition, Burkholderiales, Alcaligenaceae, and Barnesiella were negatively correlated with serum creatinine. Blood urea nitrogen levels were positively correlated with Christensenellaceae, Bacteroidales_S24.7, Ruminococcaceae, Ruminococcus, and Lachnospiraceae_NK4A136, but were negatively correlated with Flavonifractor_plautii and Erysipelatoclostridium_ramosum. Enterobacteriales, Enterobacteriaceae, Porphyromonadaceae, Escherichia/Shigella, Parabacteroides, and Escherichia_coli were positively correlated with albumin. Proteinuria was positively correlated with Verrucomicrobia, Coriobacteriia, Thermoleophilia, Ignavibacteria, Coriobacteriales, Nitrosomonadales, Coriobacteriaceae, and Blautia, but was negatively correlated with Betaproteobacteria, Burkholderiales, and Alcaligenaceae. Conclusion. Our findings show compositional alterations of intestinal microbiota in adult patients with INS and correlations between significantly altered taxa and clinical parameters, which points out the direction for the development of new diagnostics and therapeutic approaches targeted intestinal microbiota.


2020 ◽  
Vol 103 (10) ◽  
pp. 1048-1056

Background: Candidemia is the most common nosocomial invasive fungal infection that causes high mortality. Emergence of drug-resistant Candida is reported worldwide but there are few studies in Thailand. Objective: To determine the epidemiology, antifungal susceptibility of Candida, and outcomes among adult patients with candidemia. Materials and Methods: A prospective, observational study in adult patients with candidemia was conducted in 2015 at a university hospital. Demographic, microbiological, and outcome data were recorded. Results: Fifty-two patients with candidemia were identified, of whom 76.9% had an underlying disease and 69.2% had risks for candidemia. Sixty-four percent of candidemia patients contracted non-albicans Candida and 36% had Candida albicans. C. tropicalis was the most common non-albicans Candida species isolated (35%), followed by C. parapsilosis (19%), and C. glabrata (10%). Fluconazole resistance was found in 12.5% of C. albicans and in 11.1% of C. parapsilosis isolates. Reduced fluconazole susceptibility or high-level fluconazole resistance was found in 68.7% of C. tropicalis isolates. All except C. parapsilosis had excellent susceptibility to echinocandins. Seventy-three percent (38/52) of patients received antifungal treatment, of whom, 78.9% received empiric fluconazole therapy, and 89.7% were started on antifungal treatment 24 hours after the isolation of Candida. The overall mortality rate was 51.9%. Conclusion: Fluconazole-resistant Candida became more prevalent particularly in C. tropicalis, which was the predominant species among non-albicans Candida causing candidemia. Empiric treatment with either amphotericin B or echinocandins would be appropriate in high-risk patients with suspected candidemia. Trial registration: Thai Clinical Trials Registry, TCTR20150605001 Keywords: Candida, Fluconazole, Resistant, Thailand


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1031.1-1032
Author(s):  
G. Figueroa-Parra ◽  
A. Moreno-Salinas ◽  
C. M. Gamboa-Alonso ◽  
M. A. Villarreal-Alarcón ◽  
D. Á. Galarza-Delgado

Background:Dermatological manifestations are not rare in patients with rheumatic diseases (RD). Multidisciplinary management and direct interaction between these disciplines are essential. According to Dermatology-Rheumatology clinics, most diagnoses evaluated are systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA), with dermatitis being the most common manifestation. It is important to be aware that skin problems in RD patients are not always related to the underlying condition(1). Nowadays, there is significant evidence to support the manifold advantages of the joint dermatology-rheumatology clinics, including improved quality of care for patients and multidisciplinary training for new physicians(2). This ongoing trend is intended to highlight the important interaction between specialties that treat overlapping conditions, and it has been incorporated in academic health centers to give a comprehensive approach to patients.Objectives:Our purpose was to describe the collaboration between the Rheumatology and Dermatology services during the evaluation of RD patients.Methods:An observational, retrospective study was performed in the Rheumatology Service of the University Hospital “Dr. Jose Eleuterio Gonzalez” in Monterrey, Mexico, between March 2019 and February 2020. All the patients with a Rheumatology or Dermatology consultation requested were included (hospitalized and outpatients). Demographic (age, gender, baseline diagnosis), the reason for consultation, specialty requested, type of treatment, final diagnoses, and agreement in final diagnosis were registered. Results are shown in descriptive statistics.Results:One hundred and seventy-four patients were included, 142 (81.6%) patients from the outpatient clinic and 32 (18.4%) patients hospitalized. The mean age was 45.1 (SD±15.8) years, 135 (77.6%) were females, 54 (31%) patients were under initial diagnosis evaluation, 30 (17.2%) had RA, 25 (14.4%) patients had SLE, 15 (8.6%) patients had psoriatic arthritis, 12 (6.9%) patients had systemic sclerosis, 6 (3.4%) patients had dermatomyositis. The main reasons for consultation in hospitalized patients were acute lupus (15.6%), subacute lupus (12.5%), purpura (12.5%), cutaneous vasculitis (9.4%), urticarial dermatitis (9.4%), dermatomyositis (6.3%) and others (34.3%). The consultation requested was: 156 (89.7%) to Dermatology and 18 (10.3%) to Rheumatology. The type of treatment prescribed was topic/local in 37 (21.3%) patients, systemic in 25 (14.4%) and both in 92 (52.9%) patients. The final diagnoses were related to the underlying disease in 102 (77%) patients and unrelated in 40 (23%) patients. The agreement between initial clinical suspicion and final diagnoses reached 75.9% between Rheumatology and Dermatology services. Figure 1.Conclusion:The collaboration between Rheumatology and Dermatology services are very important. Most of the patients were under initial evaluation. All the rheumatologists and dermatologists should be aware of the interdependence from both specialties to give the best quality of care to the patients.References:[1]Samycia M, McCourt C, Shojania K, Au S. Experiences From a Combined Dermatology and Rheumatology Clinic: A Retrospective Review. J Cutan Med Surg. 2016;20(5):486-489. doi:10.1177/1203475416649138.[2]Theodorakopoulou E, Dalamaga M, Katsimbri P, Boumpas DT, Papadavid E. How does the joint dermatology-rheumatology clinic benefit both patients and dermatologists?. Dermatol Ther. 2020;33(3):e13283. doi:10.1111/dth.13283Figure 1.Disclosure of Interests:None declared


Medicina ◽  
2022 ◽  
Vol 58 (1) ◽  
pp. 94
Author(s):  
Ioana Cretu ◽  
Bogdan Cretu ◽  
Catalin Cirstoiu ◽  
Adrian Cursaru ◽  
Mihaela Milicescu ◽  
...  

Background and Objectives: The occurrence of rheumatological side effects in a patient after receiving immunotherapy for cancer is becoming increasingly common. Oncologists often fail to diagnose and refer affected patients to rheumatologists. This paper presents the various rheumatological adverse events that occur after immunotherapy in patients as well as their treatment and evolution. Materials and Methods: A total of 36 patients were monitored between November 2018 and March 2020. The oncologist monitoring the immunotherapy-treated patients identified the occurrence of musculoskeletal side effects. The grading of toxicities was performed by both the oncologist and the rheumatologist using common terminology criteria for adverse events (CTCAE). Rheumatological treatment was administered, and for some patients, immunotherapy was discontinued. Results: The clinical presentations of the patients varied. Mild side effects (grade 1–2) were reported in a higher proportion than severe side effects (grade 3–5). Therefore, thirty-one patients had mild-to-moderate side effects, and five patients had severe side effects. Adverse reactions occurred, on average, 10 weeks after the initiation of immunotherapy; this indicated that the severity of the toxicity was dose dependent. Patients were treated with NSAIDs or prednisone, depending on the severity of the side effects, and for patients with severe manifestations, immunotherapy was discontinued. The remission of rheumatic manifestations varied depending on the grade of the manifestations. Conclusions: The clinical, biological, and ultrasound presentations of the patients with adverse events followed by cancer treatments differed from classic rheumatological manifestations. Thorough examinations of these patients by both oncologists and rheumatologists are needed in order to correctly diagnose and treat rheumatological adverse events. Multiple studies that include a larger number of participants are needed in order to better understand the pathogenesis and clinical evolution of these patients under different treatment conditions.


2020 ◽  
Vol 6 (1) ◽  
pp. 32-37
Author(s):  
Chhimi Wangmo ◽  
Nor Tshering Lepcha

Introduction: The aim of this hospital-based study was to assess the prevalence and associated factors of pterygium among adult patients visiting the Ophthalmology Outpatient Department (OPD) in the Jigme Dorji Wangchuck National Referral Hospital (JDWNRH), Thimphu. Methods: A cross sectional study was carried out in the ophthalmology OPD in the national referral hospital, Thimphu from 1st January, 2018 to 31st December, 2018, during which 1599 adult patients were selected through systematic random sampling. Results: The prevalence of pterygium was 12.8% (95% CI: 11.2 -14.5). Among 271 eyes with pterygium, the distribution of grade 1, grade 2 and grade 3 pterygium was 34.7%, 56.1% and 9.2% respectively. Pterygium wasmost common in the age group of 36-55 years. The significant factors associated with pterygium were age group, occupation and usage of sunglasses. Individuals who were 36-55 years (adjusted OR 2.70, 95% CI 1.82-4.0) and >55 years (adjusted OR 2.17, 95% CI 1.34-3.50) had significantly higher risk than 18-35 years (p< 0.002), and not using sunglasses (adjusted OR 1.97, 95% CI1.17-3.33, p = 0.007) significantly increased the risk of pterygium. Indoor occupation, particularly being a student was protective against pterygium (OR 0.08, 95% CI 10.02-0.33, p< 0.001). Only 14.1% used sunglasses and among them, 27.6% were aware that sunglasses can protect from ultraviolet radiation. Conclusion: This study found a high prevalence of pterygium among adults aged 18 years and above. Those aged 36-55 years and an occupation involving outdoor activities were affected more. Encouraging usage of sunglasses may reduce pterygium


2021 ◽  
Vol 15 (6) ◽  
pp. 2102-2106
Author(s):  
Alireza Ghanadan ◽  
Zahra Naraghi ◽  
Kambiz Kamyab- Hesari ◽  
Azita Nikoo ◽  
Zahra Halaji ◽  
...  

Background: Pathologic examination of Acute Generalized Exanthematous Pustulosis (AGEP) and Pustular Psoriasis (PP) are similar. We encountered many patients with PP or AGEP who cannot be distinguished clinically, pathologically and based on disease course from each so we designed a comprehensive interpersonal histopathologic overview of these patients' samples. Method: Histopathological data of 16 patients over 3.5 years were analyzed. Four pathologists separately reviewed specimens based on eighteen criteria (9 Epidermal and 9 Dermal). Severity score for each criterion was considered as to be (0 to 3+). We compared the final pathologic diagnosis with primary one. Results: Neutrophilic and lymphocytic infiltration in dermis were seen in all cases of AGEP while intraepithelial pustules. Subcorneal and intraepithelial pustules, spongiosis, neutrophilic exocytosis, neutrophilic and lymphocytic infiltration in dermis were observed in all cases of PP. The most severe neutrophilic inflammation; acanthosis and neutrophilic or lymphocytic infiltration were seen in PP. The authors of this study have been reported generalized pustular clinical presentations of patients have been taken HCQ, and in the recent pandemic it is also one of the concerns that many studies have been focused (....). Conclusion: When primary histopathologic report is AGEP/PP overlap, clinical judgment is the best way to manage and it is more probable that the final diagnosis being PP. When only AGEP or PP is histopathologic diagnostic report, it is usually enough to make final diagnosis and appropriate management. Key words: hydroxychloroquine, HCQ, generalized pustular cutaneous eruption, Pustular Psoriasis (PP), Acute Generalized Exanthematous Pustulosism, AGEP, Histopathological overview, pathology, review


2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Gemma Patella ◽  
Alessandro Comi ◽  
Giuseppe Coppolino ◽  
Nicolino Comi ◽  
Giorgio Fuiano ◽  
...  

Abstract Background and Aims Steroid-dependent nephrotic syndrome (SDNS) may require a prolonged multi-drug therapy with risk of drug toxicity and renal failure. Rituximab (RTX) treatment has been found to be helpful in reducing the steroid dosage and the need for immunosuppressants (ISs), but little data are currently available regarding very long-term outcomes in adults. We herein describe a long-term, single-center experience of RTX use in a large series of adults with SDNS. Method We studied 23 adult patients with SDNS (mean age 54.2±17.1 y; 65% male; BMI 28.5±4.7), mostly consequent to membranous (47.8%) or focal glomerulonephritis (30.2 %) who were eligible to start a RTX regimen. Before entering the RTX protocol, proteinuria and eGFR were 7.06±3.87 g/24h and 65.9±28.2 ml/min/1.73 m2, respectively; albumin and CD19/CD20 ratio were 2.9±0.9 g/L and 0.99±0.01 respectively; the mean number of ISs was 2.39±0.89 and the mean annual rate of relapses was 2.2±0.9. Results Patients were followed over a mean follow-up of 64 months (range: 12-144). After RTX (mean dose: 1202.1±372.4 mg) the rate of relapses was virtually nullified (p&lt;0.001). eGFR remained roughly stable (62.1±19.8 ml/min/1.73 m2, p=NS), while proteinuria, albumin, CD19/CD20 and BMI all significantly improved (p ranging from 0.01 to 0.001). The mean number of additional ISs was also reduced (0.44±0.12; p&lt;0.001) and RTX enabled discontinuation of steroids in 13/23 (56.5%) patients. No major adverse events related to therapy were recorded. Conclusion Findings from this large case-series with a remarkable very long follow-up reinforce the role of RTX as an efficient and safe weapon to improve outcomes in adult patients suffering from SDNS.


Sign in / Sign up

Export Citation Format

Share Document