scholarly journals Blood Pressure Differences in Nephrotic Syndrome Patients with Steroid Resistant Nephrotic Syndrome Patients and Steroid Sensitive Nephrotic Syndrome

2021 ◽  
Vol 9 (T3) ◽  
pp. 349-352
Author(s):  
Muhammad Akbar ◽  
Oke Rina Ramayani ◽  
Gema Nazri Yanni
Keyword(s):  
Blood Pressure ◽  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Systolic Blood Pressure ◽  
Diastolic Blood Pressure ◽  
Pediatric Nephrology ◽  
Disease Classification ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Steroid Sensitive

BACKGROUND: Syndrome nephrotic is the most common kidney disease found in pediatric kidney disease, classification based on clinical response to steroids or histopathological characteristics. Increased blood pressure in steroid-resistant nephrotic syndrome (NS) is still a complication to be aware of in cases of NS. AIM: The aim of the study was to determine the differences in systolic and diastolic blood pressure in patients with steroid-sensitive NS and steroid-resistant NS. METHODS: Analytical correctional study in 50 children with NS divided into 25 Steroid Resistant NS (SRNS) groups and 25 steroid sensitive NS (SSNS) people who met inclusion and exclusion criteria to assess systolic and diastolic blood pressure in each group in pediatric nephrology division of the general hospital of Haji Adam Malik Medan. RESULTS: There is a difference in systolic blood pressure in the SRNS and SSNS groups which mean p = 0.024 and there is no difference in diastolic blood pressure in the SRNS group with SSNS p = 0.358. If linked levels of proteinuria to blood pressure, systolic and diastolic in both groups found no significant link p>0.05 high blood pressure with the degree of proteinuria in both group. CONCLUSION: There are differences in systolic blood pressure in the SRNS and SSNS groups.

scholarly journals Selenium level in steroid-resistant and steroid-sensitive nephrotic syndrome

2020 ◽  
Vol 60 (6) ◽  
pp. 316-20
Author(s):  
Sudung Oloan Pardede ◽  
Andini Striratnaputri ◽  
Muzal Kadim
Keyword(s):  
Nephrotic Syndrome ◽  
Venous Blood ◽  
Pediatric Nephrology ◽  
Cross Sectional Study ◽  
Cross Sectional ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Peroxidase Enzyme ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive

Background The mechanisms of pathogenesis of steroid-resistant nephrotic syndrome (SRNS) and steroid-sensitive nephrotic syndrome (SSNS) are not well understood. Antioxidants, such as glutathione peroxidase enzyme (GPx) and its cofactor, selenium, are thought to slow the progress of nephrotic syndrome (NS). Objective To compare selenium levels in SRNS and SSNS pediatric patients. Methods This cross-sectional study was conducted in 51 SRNS and 30 SSNS patients, aged 2 to 18 years, who visited the Pediatric Nephrology Outpatient Clinic at Cipto Mangunkusumo Hospital, Jakarta. Subjects were included by consecutive sampling. Selenium was measured on venous blood using GC tools MS ™ (Agilent technologies, inc.). Results Median selenium levels in SRNS patients were 92 (range 42-154) μg/L and in SSNS patients were 93 (range 69-193) μg/L. Conclusion Selenium levels in SRNS and SSNS patients were not significantly different.

Whole-Exome Sequencing Solved over 2-Decade Kidney Disease Enigma

Nephron ◽  
2021 ◽  
pp. 1-6
Author(s):  
Suramath Isaranuwatchai ◽  
Ankanee Chanakul ◽  
Chupong Ittiwut ◽  
Chalurmpon Srichomthong ◽  
Vorasuk Shotelersuk ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Exome Sequencing ◽  
Whole Exome Sequencing ◽  
Treatment Plan ◽  
Unknown Etiology ◽  
Pediatric Case ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Whole Exome

Chronic kidney disease of unknown etiology (CKDu) has been a problem in renal practice as indefinite diagnosis may lead to inappropriate management. Here, we report a 54-year-old father diagnosed with CKDu at 33 years old and his 8-year-old son with steroid-resistant nephrotic syndrome. Using whole-exome sequencing, both were found to be heterozygous for c.737G>A (p.Arg246Gln) in LMX1B. The diagnosis of LMX1B-associated nephropathy has led to changes in the treatment plan with appropriate genetic counseling. The previously reported cases with this particular mutation were also reviewed. Most children with LMX1B-associated nephropathy had nonnephrotic proteinuria with normal renal function. Interestingly, our pediatric case presented with steroid-resistant nephrotic syndrome at 8 years old and progressed to ESRD requiring peritoneal dialysis at the age of 15 years. Our report emphasized the need of genetic testing in CKDu for definite diagnosis leading to precise management.

scholarly journals Reverse Phenotyping after Whole-Exome Sequencing in Steroid-Resistant Nephrotic Syndrome

2019 ◽  
Vol 15 (1) ◽  
pp. 89-100 ◽  
Author(s):  
Samuela Landini ◽  
Benedetta Mazzinghi ◽  
Francesca Becherucci ◽  
Marco Allinovi ◽  
Aldesia Provenzano ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Exome Sequencing ◽  
Whole Exome Sequencing ◽  
Clinical Signs ◽  
Long Term Outcome ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Pathogenic Variants ◽  
Whole Exome ◽  
Steroid Sensitive

Background and objectivesNephrotic syndrome is a typical presentation of genetic podocytopathies but occasionally other genetic nephropathies can present as clinically indistinguishable phenocopies. We hypothesized that extended genetic testing followed by reverse phenotyping would increase the diagnostic rate for these patients.Design, setting, participants, & measurementsAll patients diagnosed with nephrotic syndrome and referred to our center between 2000 and 2018 were assessed in this retrospective study. When indicated, whole-exome sequencing and in silico filtering of 298 genes related to CKD were combined with subsequent reverse phenotyping in patients and families. Pathogenic variants were defined according to current guidelines of the American College of Medical Genetics.ResultsA total of 111 patients (64 steroid-resistant and 47 steroid-sensitive) were included in the study. Not a single pathogenic variant was detected in the steroid-sensitive group. Overall, 30% (19 out of 64) of steroid-resistant patients had pathogenic variants in podocytopathy genes, whereas a substantial number of variants were identified in other genes, not commonly associated with isolated nephrotic syndrome. Reverse phenotyping, on the basis of a personalized diagnostic workflow, permitted to identify previously unrecognized clinical signs of an unexpected underlying genetic nephropathy in a further 28% (18 out of 64) of patients. These patients showed similar multidrug resistance, but different long-term outcome, when compared with genetic podocytopathies.ConclusionsReverse phenotyping increased the diagnostic accuracy in patients referred with the diagnosis of steroid-resistant nephrotic syndrome.

Levamisole treatment in steroid-sensitive and steroid-resistant nephrotic syndrome

1998 ◽  
Vol 12 (6) ◽  
pp. 459-462 ◽  
Author(s):  
K. Tenbrock ◽  
J. Müller-Berghaus ◽  
A. Fuchshuber ◽  
D. Michalk ◽  
U. Querfeld
Keyword(s):  
Nephrotic Syndrome ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Steroid Sensitive

scholarly journals The role of cell signaling molecules in the pathogenesis of glomerulonephritis in children

2021 ◽  
Vol 64 (2) ◽  
pp. 37-41
Author(s):  
Angela Ciuntu ◽  
Keyword(s):  
Nephrotic Syndrome ◽  
Cell Signaling ◽  
Clinical Manifestations ◽  
Signaling Molecules ◽  
Control Group ◽  
Healthy Children ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive

Background: Cytokines are functional class of tiny proteins and glycoprotein and fundamentally they are monomers that function as soluble mediators in an autocrine or paracrine manner. Cytokines are produced by a number of cell types, predominantly leukocytes, and their targets implicate both immune and non-immune cells. Material and methods: This study was performed on 75 children with glomerulonephritis (GN), aged from 2 up to 17 years. There were 20 children with steroid-sensitive nephrotic syndrome (SSNS), 15 children with steroid-resistant nephrotic syndrome (SRNS), 20 children with chronic glomerulonephritis (CGN) nephrotic form and 20 children with CGN mixed form. This study was performed on patients experiencing disease relapse and clinical remission. The control group consisted of 20 healthy children. Results: The results of this study demonstrated increased levels of cell signaling molecules (IL-8, TNF-α, MCP-1, MIP-1α) in the urine during clinical manifestations, valuable result due to their major role in the immunopathogenic mechanism of proteinuria in nephrotic syndrome. Conclusions: Determination of urinary concentrations of cellular signaling molecules may be useful as a predictive non-invasive method for estimating disease activity, monitoring disease progression, differentiating steroid-sensitive nephrotic syndrome from steroid-resistant nephrotic syndrome, and assessing the effectiveness of treatment in children with different variants of GN

scholarly journals Use of Cyclosporine Therapy in Steroid Resistant Nephrotic Syndrome (SRNS): A Review

2015 ◽  
Vol 8 (4) ◽  
pp. 136 ◽  
Author(s):  
Syed Raza Shah ◽  
Areeba Altaf ◽  
Mohammad Hussham Arshad ◽  
Anum Mari ◽  
Sahir Noorani ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Side Effects ◽  
Immunosuppressive Treatment ◽  
Pediatric Nephrology ◽  
Calcineurin Inhibitors ◽  
Symptomatic Treatment ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Cyclosporine Therapy ◽  
Difficult Challenge

<p>A chronic, progressive disorder Steroid Resistant Nephrotic Syndrome (SRNS) accounts for 10-20% of all children with Nephrotic Syndrome. It is a heterogeneous disorder comprised of persistent edema, proteinuria, hypoalbuminemia and hyperlipidemia. Treatment for steroid-resistant nephrotic syndrome (SRNS) is challenging and children who suffer from SRNS require aggressive treatment to achieve remission. Calcineurin inhibitors have been used more in an empirical manner than on the basis of clear rationale. It was in 1984 when cyclosporine was first considered for the treatment of steroid resistant nephrotic syndrome. Cyclosporin is a calcineurin inhibitor that suppresses immune response by downregulating the transcription of various cytokine genes. Till now many studies have been conducted to determine dosages, duration of therapy, side effects and advantages of cyclosporine. Treatment of SRNS remains a difficult challenge in pediatric nephrology.  Treatment should be individualized according to the underlying histopathology, and clinical and environmental conditions of the children. There is an urgent need to distinguish as soon as possible those patients who may benefit from prolonged immunosuppressive treatment from those who will not benefit from such treatment and who will just suffer from its major side effects. The emerging evidence that the majority of genetic forms of SRNS should receive symptomatic treatment only, should also be clinically tested and studies baring its significance should be evaluated in the future.</p>

A genetic study of steroid-resistant nephrotic syndrome: relationship between polymorphism -173 G to C in the MIF gene and serum level MIF in children

2015 ◽  
Vol 7 (1) ◽  
pp. 102-107 ◽  
Author(s):  
O. R. Ramayani ◽  
N. Sekarwana ◽  
P. P. Trihono ◽  
A. H. Sadewa ◽  
A. Lelo
Keyword(s):  
Nephrotic Syndrome ◽  
Steroid Resistance ◽  
Enzyme Linked Immunosorbent Assay ◽  
Healthy Children ◽  
Single Nucleotide ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Increased Risk ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive

There is no satisfactory explanation as to why some nephrotic syndrome (NS) patients respond to glucocorticoids and others do not. The aim of this study was to investigate an association between single nucleotide polymorphism of the MIF gene -rs755622 and serum MIF concentrations in NS patients. During a period between November 2011 and September 2012, 120 consecutive children divided into three groups [healthy children, steroid-resistant nephrotic syndrome (SRNS) and steroid-sensitive nephrotic syndrome (SSNS)] were examined. Children were defined as healthy when they had a normal estimated glomerular filtration rate and spot urinary albumin creatinine ratio <150 μg/mg creatinine. SRNS was diagnosed in children who did not respond to the usual doses of steroids within 4 weeks of initiating treatment. SSNS patients were defined as those who had remission after usual doses of steroids. The genotype of -173 G to C polymorphism of the MIF gene was determined using polymerase chain reaction restriction fragment length polymorphism methods. Serum MIF concentration was measured using sandwich enzyme-linked immunosorbent assay. The allele frequency of the C allele was higher in SRNS compared with that of SSNS patients (P=0.025). There was a trend toward an association between genotypes and serum MIF disturbances. In conclusion, this study noted elevated circulating serum MIF levels and higher frequency of the C allele of the MIF gene in SRNS patients. The presence of the C allele implies an increased risk for steroid resistance.

Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium

2013 ◽  
Vol 28 (8) ◽  
pp. 1235-1241 ◽  
Author(s):  
Caroline Straatmann ◽  
Rose Ayoob ◽  
Rasheed Gbadegesin ◽  
Keisha Gibson ◽  
Michelle N. Rheault ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Treatment Outcome ◽  
Pediatric Nephrology ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome

scholarly journals Comparison of clinical and lab profile between steroid sensitive and steroid resistant nephrotic syndrome at onset of disease and evaluating predictors for developing steroid resistance in nephrotic syndrome

2020 ◽  
Vol 7 (6) ◽  
pp. 1304
Author(s):  
Anitha Palaniyandi ◽  
Subramani Palaniyandi
Keyword(s):  
Nephrotic Syndrome ◽  
Age At Onset ◽  
Steroid Resistance ◽  
First Episode ◽  
Observation Study ◽  
Serum Triglycerides ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive

Background: Nephrotic syndrome is a notable chronic disease in children. The objective of this study was to compare the clinical and lab profile between steroid sensitive nephrotic syndrome and steroid resistant nephrotic syndrome at the onset of disease. Certain parameters were tested if they could be significate predictors of developing steroid resistance at the onset of first episode of nephrotic syndrome.Methods: Retrospective observation study done children 1-12 years diagnosed with nephrotic syndrome in Sri Ramachandra Medical College and Hospital, Department of Paediatrics, Chennai. Sample size 150. Period of study Jan 2013- Dec 2015. Variables considered were age at onset, sex, parental consanguinity with essential lab parameters done at the onset of nephrotic syndrome proteinuria, pyuria, microscopic hematuria, urine protein creatinine ratio, serum creatinine, serum triglycerides and serum albumin. Children less than 1 year of age, cases with secondary causes of nephrotic syndrome and steroid dependant nephrotic syndrome, children with incomplete records were not included in this study. 150 cases who fulfilled the study criteria were included in this study.Results: 75 cases of steroid sensitive nephrotic syndrome (SSNS) were compared with an equal number of steroid resistant nephrotic syndrome (SRNS). 85 children had onset of disease before 3 years of age and majority had 3+ proteinuria and males predominated in both the groups. The overall consanguinity rates were higher among SRNS group. Triglyceride level >300 mg/dl predominated in SRNS group along with a higher severity of hypoalbuminemia when compared to SSNS group. None of the parameters tested were significant predictors of developing SRNS subsequently.Conclusions: Comparing steroid sensitive with steroid resistance nephrotic syndrome, no lab parameter could identify the risk of a child developing steroid resistance subsequently. This could be a field of interest in future studies that could predict the development of steroid resistance at the onset of first episode of nephrotic syndrome itself. 

Sign in / Sign up

Export Citation Format

Share Document