MO1028EFFECTIVENESS AND KIDNEY PROGNOSIS IN THE TREATMENT OF CORTICORRESISTANT NEPHROTIC SYNDROME IN PEDIATRICS

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Ana Vinuesa Jaca ◽  
Lina Catherine Hernández Zúñiga ◽  
Pedro Arango Sancho ◽  
Elena Codina Sampera ◽  
Yolanda Calzada Baños ◽  
...  
Keyword(s):  
Renal Function ◽  
Nephrotic Syndrome ◽  
Kidney Transplantation ◽  
Complete Remission ◽  
Immunosuppressive Treatment ◽  
Focal And Segmental Glomerulosclerosis ◽  
Mesangial Proliferation ◽  
Segmental Glomerulosclerosis ◽  
Diffuse Mesangial Proliferation ◽  
The Mean

Abstract Background and Aims To report the response to different treatments and the renal prognosis in a cohort of patients with corticosteroid-resistant nephrotic syndrome (CRNS). Method Retrospective observational study in patients with CRNS. For this, the results of the histology, the different treatment guidelines used in each case and the evolution of renal function were collected, determining the results in terms of remission and renal survival in the different groups. Results Of the initial cohort of 37 patients, 33 were included, excluding 4 patients with Finnish-type genetic CRNS. The mean age at diagnosis was 6.1 years. 54.5% were women. Regarding the initial biopsy, 45.5% corresponded to minimal changes (15 patients), 27.3% (9) focal and segmental glomerulosclerosis, 15.1% (5) diffuse mesangial proliferation and 12.1% (4) others. The mean follow-up was 53 months (3-115 months). 27 patients (84.4%) received cyclosporine (CyA), 66.7% (18) of them presented complete remission and 22.2% (6) partial response. Of the patients in complete remission, 33% had at least one relapse after 17 months of treatment (7–27 months). Rituximab was administered in 12 patients (37.5%), of which 7 had not previously responded to immunosuppressants. 100% of frequent relapsers presented complete remission after administration of Rituximab, although 3 had subsequent relapses (60%) after 21 months of treatment (12-34 months). 57% of the patients who did not respond to immunosuppressants did not respond to Rituximab either, with Ofatumumab allowing complete remission in one of them. When relating the results with the histology, we saw how the remission in minimal changes and diffuse mesangial proliferation was 100% and 80%, respectively, although it was 33.3% in focal and segmental glomerulosclerosis. Similarly, renal failure was more frequent in patients with focal segmental glomerulosclerosis (77.7%). Of the remissions (24; 72.7%), 3 were partial (9.1%) and 6 (18.2%) did not respond to any immunosuppressive treatment, with the need for kidney transplantation in 2 of them (6%) and with 1 deceased due to an infectious cause (3%). Conclusion Histology and, especially, focal and segmental glomerulosclerosis, play a prognostic role in the CRNS with a lower remission rate and a greater deterioration in renal function and the need for associated kidney transplantation.

scholarly journals Focal and Segmental Glomerulosclerosis and Membranous Nephropathy overlapping in a patient with Nephrotic Syndrome: a case report

2020 ◽  
Vol 42 (1) ◽  
pp. 113-117
Author(s):  
Crislaine Aparecida da Silva ◽  
Fabiano Bichuette Custódio ◽  
Maria Luíza Gonçalves dos Reis Monteiro ◽  
Stanley de Almeida Araújo ◽  
Liliane Silvano Araújo ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Membranous Nephropathy ◽  
Morphological Characteristics ◽  
Stage Ii ◽  
Primary Case ◽  
Focal And Segmental Glomerulosclerosis ◽  
Morphological Findings ◽  
Segmental Glomerulosclerosis ◽  
Podocyte Detachment ◽  
Foot Process

Abstract Introduction: Some cases of membranous nephropathy (MGN) present focal segmental glomerulosclerosis (FSGS) typically associated with disease progression. However, we report a case of a patient who seemed to have MGN and FSGS, both primary. Case presentation: A 17-year-old female, Caucasian, presenting lower extremity edema associated with episodes of foamy urine and high blood pressure, had physical and laboratorial exams indicating nephrotic syndrome. A renal biopsy was performed and focal and segmental glomerulosclerosis were observed under light microscopy in some glomeruli presented as tip lesion, and in others it was accompanied by podocyte hypertrophy and podocyte detachment in urinary space, compatible with podocytopathy FSGS. Besides, there were thickened capillary loops with basement membrane irregularities due to "spikes" compatible with MGN stage II. Immunofluorescence showed finely granular IgG, IgG4, and PLA2R deposits in capillary loops and, in electron microscopy, subepithelial deposits and foot process effacement. These morphological findings are compatible with FSGS and MGN stage II. Conclusions: In the present case, clinical and morphological characteristics showed a possible overlap of primary FSGS and MGN as focal and segmental glomerulosclerosis does not seem to be related with MGN progression but with the podocytopathy FSGS.

scholarly journals Effect of Protein A Immunoadsorption in Nephrotic Syndrome of Various Etiologies

1999 ◽  
Vol 10 (9) ◽  
pp. 2014-2017
Author(s):  
VINCENT L. M. ESNAULT ◽  
DOMINIQUE BESNIER ◽  
ANGELO TESTA ◽  
PIERRE COVILLE ◽  
PIERRE SIMON ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Nephrotic Syndrome ◽  
Serum Albumin ◽  
Antihypertensive Drugs ◽  
Immunosuppressive Treatment ◽  
Protein A ◽  
Short Term ◽  
Focal And Segmental Glomerulosclerosis ◽  
Albumin Infusion ◽  
Baseline Levels

Abstract. Protein A immunoadsorption (IA) has proved effective in reducing proteinuria in patients with nephrotic syndrome after recurrence of focal and segmental glomerulosclerosis (FSGS) in kidney transplants. The effect of IA in nephrotic syndrome of other etiologies remains unknown. Nine patients with nephrotic syndrome secondary to membranous nephropathy (four cases), diabetes mellitus (one case), IgA nephropathy (two cases), and amyloidosis (two cases) had three to five IA of 2.5 plasma volumes over 4 to 8 d. Patients received no concomitant immunosuppressive treatment, and antihypertensive drugs were left unchanged. Proteinuria decreased from 12.64 ± 5.49 to 3.35 ± 2.2 g/24 h (mean ± SD) in all patients after three to five IA. Hematocrit decreased from 37.32 to 32.64% (12.5% hemodilution) and serum albumin from 25.43 to 18.6 g/L (26.4% decrease). Proteinuria returned to baseline levels within 1 mo, as described in recurrent FSGS following transplantation. When serum albumin balance was controlled by albumin infusion after IA in two patients, comparable decreases in proteinuria were observed. Therefore, IA is effective in producing short-term reduction of proteinuria in nephrotic syndromes related not only to FSGS but also to membranous and IgA nephropathies, diabetes mellitus, and amyloidosis, which suggests that IA removes a nonspecific circulating hemodynamic-altering or permeability-increasing factor.

scholarly journals Collapsing focal segmental glomerulosclerosis probably triggered by dengue virus infection - two case reports

2020 ◽  
Author(s):  
Patrícia Cruz Queiroz ◽  
Ana Elisa Souza Jorge ◽  
Plínio Henrique Vaz Mourão ◽  
Maria Goretti Moreira Guimarães Penido
Keyword(s):  
Renal Function ◽  
Nephrotic Syndrome ◽  
Virus Infection ◽  
Dengue Virus ◽  
Focal Segmental Glomerulosclerosis ◽  
Case Reports ◽  
Kidney Injury ◽  
Dengue Virus Infection ◽  
Segmental Glomerulosclerosis ◽  
Collapsing Fsgs

Abstract The reported cases describe the association between collapsing focal segmental glomerulosclerosis (FSGS) and acute dengue virus infection. In both cases, patients were diagnosed with dengue virus infection and had a severe kidney disease, with nephrotic syndrome and acute kidney injury. Kidney biopsy was performed and showed collapsing FSGS. The first patient, a 27-year-old man, was diagnosed with dengue virus infection and developed nephrotic syndrome after two weeks of illness. He was treated with methylprednisolone for three days and intravenous furosemide. This patient evolved well, although his renal function did not fully recover. The second patient, a 32-year-old man, was diagnosed with a milder clinical presentation of dengue virus infection. He had a past medical history of nephrotic syndrome in childhood, which might have caused its relapse. This patient was treated with intravenous furosemide and also did not fully recover renal function. These cases highlight the possible implication of dengue virus infection in the etiology of collapsing variant of FSGS. Healthcare professionals should be prepared to identify similar cases.

P0389THE PREVALENCE, CLINICAL AND PATHOLOGICAL CHARACTERISTICS OF PRIMARY FOCAL SEGMENTAL GLOMERULOSCLEROSIS IN TURKISH ADULTS: THE DATA FROM TSN-GOLD ( TURKISH SOCIETY OF NEPHROLOGY GLOMERULAR DISEASES) WORKING GROUP

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Ilhan Kurultak ◽  
Ozkan Gungor ◽  
Savas Ozturk ◽  
Ahmet Burak Dirim ◽  
Necmi Eren ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Nephrotic Syndrome ◽  
Focal Segmental Glomerulosclerosis ◽  
Pathological Examination ◽  
Glomerular Diseases ◽  
Segmental Glomerulosclerosis ◽  
Hospitalization Period ◽  
The Mean ◽  
Primary Glomerular Diseases ◽  
Turkish Society

Abstract Background and Aims Focal segmental glomerulosclerosis (FSGS) is one of the primary glomerulopathies which is the most common cause of end-stage renal disease among all primary glomerular diseases. In adults, nephrotic syndrome develops 10-35% due to FSGS. However, its frequency has been increasing in recent years. This study was aimed to present the data and the prevalence of primary FSGS patients in Turkey. Method These data were obtained from the National Multicenter (47 centers) Primary Glomerular Diseases registry system, which was entered into the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) database between May 2009 and June 2019. The presented data in this study is cross-sectional and includes the recorded data of the patients in the hospitalization period for the kidney biopsy. These demographic, clinic and pathological data of patients were evaluated with using statistically. Results Of the 3875 patients enrolled in the Primary Glomerular Diseases database, 850 (21.9%) had FSGS. The mean age of the patients was 47.1 ± 13.7 and 435 (52%) of patients were male. Two hundred and eighty one (33.1%) had hypertension and 93 (11%) had diabetes. Five hundred and four patients (59.3%) were diagnosed as nephrotic syndrome. At the time of diagnosis, the mean systolic blood pressure was 13.8 ± 18.6 mm Hg and the mean diastolic blood pressure was 81.4± 11.4 mm Hg. The laboratory findings at the time of diagnosis on following; serum creatinine 1.2 ± 0.04 mg / dl, albumin 3.3 ± 0.03 g / dl, and 24 hours urinary proteinuria amounts to 4743 ± 181 mg / day. Demographical and clinical data of the patients at the time of diagnosis were presented on Table 1. In pathological examination; the mean number of glomeruli was 16.8 ± 0.3, global sclerotic glomeruli were 3.1 ± 0.1, and segmental sclerotic glomeruli were 2.4 ± 0.1. Summary of Pathological Findings were presented on Table 2 Conclusion It is very difficult to determine the absolute incidence and prevalence of primary FSGS. Incidence rates have been reported to range from 0.2 to 1.8 / 100,000 per year in the literature. In a United States study, the most common diagnosis in 2501 kidney biopsies was FSGS (35.9%). According to this database, the prevalence of FSGS in primary glomerular diseases in our country is 21.9%.

P1824RETROSPECTIVE STUDY FOR THE ANALYSIS AND EVOLUTION OF RENAL FUNCTION IN A COHORT OF PATIENTS AFFECTED BY THE NEPHRONOPHTHISIS COMPLEX

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Pedro Arango Sancho ◽  
Víctor López Baez ◽  
Elena Codina Sampera ◽  
Yolanda Calzada Baños ◽  
Ana Vinuesa Jaca ◽  
...  
Keyword(s):  
Renal Function ◽  
Kidney Transplantation ◽  
Filtration Rate ◽  
Clinical Presentation ◽  
Renal Involvement ◽  
Medium Term ◽  
Transplant Patients ◽  
Therapy Program ◽  
Frequent Form ◽  
The Mean

Abstract Background and Aims Determine evolution of renal function in nephronophthisis patient cohort, prognosis and variability of clinical presentation. Method Retrospective observational study to determine evolution of renal function in our cohort(n=13) between 4.5-20 years affected by different syndromes associated with nephronophthisis complex in last 20 years. We compared clinical-analytical parameters to diagnosis with current ones, including needs for kidney transplantation and results of genetics. Results Chronic-Kidney-Disease (CKD) prevalence at diagnosis was between 0.9-8.5 years (mean 5.3 years), staging of CKD was: 30.7% stage 2, 15.38% stage 3a and 1 patient stage 3b. 23.07% in stages 4 and 5. 69.23% needs first kidney transplantation and even a second one 15.38%. The average age at first transplantation was 6.04 years(2-11.67 years). The mean age to enter in renal replacement therapy program was 1.3 years(0.1-3.67 years). Among non-transplant, from diagnosis to present its being 0.5-7.25 years(median, 4.24 years). Most frequent symptom at the onset of the disease was delay in weight gain and height(84.61%) and also in cognitive development(53.84%), followed by polyuria-polydipsia(46.15%) and other signs/symptoms such as ataxia, pigmentary retinitis, hepatic insufficiency or skeletal alterations. In fact, in up to 54% of cases, renal involvement was a casual finding. Even with this, the most frequent form of presentation was purely renal(38.46%), followed by Joubert's Syndrome(30.77%), RHYNS(15.38%), Senior-Loken(7.7%) and Maizner-Saldino(7.7%). Currently, calculated glomerular filtration rate ranges between30.2-90.21 ml/min/1.73 m2 among recipient, with majority in the CKD stages: 3a(56%) and in between 30-128 ml/min/1.73 m2 among non-transplant patients, with predominance CKD 2 (50%). Conclusion The prognosis of renal function in the short-medium term in nephronophthisis is unfortunate. The possible initial symptomatic renal silence, the need (>50%) of multidisciplinary management and the frequency of CKD advanced to the diagnosis make necessary the knowledge of the disease and its possible associations, as well as its active search.

Altered Signaling and Regulatory Mechanisms of Apoptosis in Focal and Segmental Glomerulosclerosis

2001 ◽  
Vol 12 (7) ◽  
pp. 1422-1433 ◽  
Author(s):  
WANSHENG WANG ◽  
ALEX TZANIDIS ◽  
MAJA DIVJAK ◽  
NAPIER MAURICE THOMSON ◽  
ALICIA NOEMI STEIN-OAKLEY
Keyword(s):  
Renal Function ◽  
Reverse Transcription ◽  
Fas Ligand ◽  
Regulatory Mechanisms ◽  
Terminal Deoxynucleotidyl Transferase ◽  
Tubulointerstitial Injury ◽  
Focal And Segmental Glomerulosclerosis ◽  
Reverse Transcription Pcr ◽  
Segmental Glomerulosclerosis

Abstract. The purpose of this study was to investigate signaling and regulatory mechanisms of apoptosis in a model of focal and segmental glomerulosclerosis. Sprague-Dawley rats received two doses of puromycin aminonucleoside (PAN) (day 0 and week 3) and a uninephrectomy (PAN model). Apoptosis was detected with the use of the terminal deoxynucleotidyl transferase mediated dUTP nick end labeling technique. Bax, Bcl-2, Fas, and Fas ligand expression was analyzed by competitive reverse transcription-PCR. Bax, Bcl-2, and Fas mRNA were localized by in situ hybridization. Renal function was transiently impaired after the first PAN dose. After the second PAN dose, further progressive renal impairment, tubular atrophy, interstitial fibrosis, and glomerulosclerosis were evident. Eighteen percent of PAN samples demonstrated up to 4 apoptotic cells/50 glomeruli, compared with 7% of sham controls (not significant). No consistent significant change in glomerular Bax, Bcl-2, Fas, and Fas ligand mRNA was evident by reverse transcription-PCR, although focal increases in glomerular Bcl-2 mRNA were demonstrated by in situ hybridization. In the tubulointerstitium, apoptosis was increased from weeks 1 to 12 (P < 0.01 PAN versus sham), correlated to renal function and tubulointerstitial injury (P < 0.01). Total renal Bax, Fas, and Fas ligand mRNA were upregulated in the PAN model, peaking at week 17 (P < 0.01 versus sham), whereas Bcl-2 mRNA was not significantly different in PAN versus sham controls. In situ hybridization in the PAN model demonstrated prominent Bax mRNA in dilated tubules and infiltrating leukocytes. Fas mRNA signal was localized to tubular epithelial cells and leukocytes. The results suggest that altered apoptotic signaling and regulatory mechanisms contribute to the tubulointerstitial injury in this model.

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