Transition of severe diffuse mesangial hypercellularity to minimal change disease in a child with steroid-resistant nephrotic syndrome

2011 ◽  
Vol 76 (12) ◽  
pp. 504-506
Author(s):  
S. Fujinaga ◽  
T. Watanabe ◽  
A. Endo ◽  
D. Hirano ◽  
Y. Ohtomo ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Mesangial Hypercellularity

scholarly journals Intravenous Cyclophosphamide Induces Remission in Children with Difficult to Treat Steroid Resistant Nephrotic Syndrome from Minimal Change Disease

2021 ◽  
Author(s):  
Maha Haddad ◽  
Arun Kale ◽  
Lavjay Butani
Keyword(s):  
Nephrotic Syndrome ◽  
North American ◽  
Minimal Change Disease ◽  
Immunosuppressive Agents ◽  
Laboratory Data ◽  
Minimal Change ◽  
Indian Children ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
American Children

Abstract Background: Steroid resistant nephrotic syndrome (SRNS), while uncommon in children, is associated with significant morbidity. Calcineurin inhibitors (CNIs) remain the first line recommended therapy for children with non-genetic forms of SRNS, but some children fail to respond to them. Intravenous (IV) cyclophosphamide (CTX) has been shown to be effective in Asian-Indian children with difficult to treat SRNS (SRNS-DTT). Our study evaluated the outcome of IV CTX treatment in North American children with SRNS-DTT.Methods: Retrospective review of the medical records of children with SRNS-DTT treated with IV CTX from January 2000 to July 2019 at our center. Data abstracted included demographics, histopathology on renal biopsy, prior and concomitant use of other immunosuppressive agents and serial clinical/laboratory data. Primary outcome measure was attainment of complete remission (CR). Results: Eight children with SRNS-DTT received monthly doses (median 6; range 4-6) of IV CTX. Four (50%) went into CR, 1 achieved partial remission and 3 did not respond. Three of the 4 responders had minimal change disease (MCD). Excluding the 1 child who responded after the 4th infusion, the median time to CR was 6.5 (range 0.5-8) months after completion of IV CTX infusions. Three remain in CR at a median of 8.5 years (range: 3.7-10.5 years) after completion of CTX; one child relapsed and became steroid-dependent. No infections or life-threatening complications related to IV CTX were observed.Conclusions: IV CXT can induce long term remission in North-American children with MCD who have SRNS-DTT.

scholarly journals Intravenous cyclophosphamide induces remission in children with difficult to treat steroid resistant nephrotic syndrome from minimal change disease

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Maha Haddad ◽  
Arundhati Kale ◽  
Lavjay Butani
Keyword(s):  
Nephrotic Syndrome ◽  
North American ◽  
Minimal Change Disease ◽  
Immunosuppressive Agents ◽  
Laboratory Data ◽  
Minimal Change ◽  
Indian Children ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
American Children

Abstract Background Steroid resistant nephrotic syndrome (SRNS), while uncommon in children, is associated with significant morbidity. Calcineurin inhibitors (CNIs) remain the first line recommended therapy for children with non-genetic forms of SRNS, but some children fail to respond to them. Intravenous (IV) cyclophosphamide (CTX) has been shown to be effective in Asian-Indian children with difficult to treat SRNS (SRNS-DTT). Our study evaluated the outcome of IV CTX treatment in North American children with SRNS-DTT. Methods Retrospective review of the medical records of children with SRNS-DTT treated with IV CTX from January 2000 to July 2019 at our center. Data abstracted included demographics, histopathology on renal biopsy, prior and concomitant use of other immunosuppressive agents and serial clinical/laboratory data. Primary outcome measure was attainment of complete remission (CR). Results Eight children with SRNS-DTT received monthly doses (median 6; range 4–6) of IV CTX. Four (50%) went into CR, 1 achieved partial remission and 3 did not respond. Three of the 4 responders had minimal change disease (MCD). Excluding the 1 child who responded after the 4th infusion, the median time to CR was 6.5 (range 0.5–8) months after completion of IV CTX infusions. Three remain in CR at a median of 8.5 years (range: 3.7–10.5 years) after completion of CTX; one child relapsed and became steroid-dependent. No infections or life-threatening complications related to IV CTX were observed. Conclusions IV CXT can induce long term remission in North-American children with MCD who have SRNS-DTT.

scholarly journals Complications and renal biopsy profile in childhood steroid resistant nephrotic syndrome

2020 ◽  
Vol 7 (6) ◽  
pp. 1420
Author(s):  
Subramani Palaniyandi ◽  
Anitha Palaniyandi
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Minimal Change Disease ◽  
Disease Incidence ◽  
Early Morning ◽  
Minimal Change ◽  
Observation Study ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Urine Albumin

Background: Nephrotic syndrome is a notable chronic disease in children. The objective of this study was to study the complications and renal biopsy profile in childhood steroid resistant nephrotic syndrome.Methods: Retrospective observation study done in Sri Ramachandra Medical College and Hospital, Department of Paediatrics, Chennai. Inclusion criteria was children aged 1-12 years diagnosed with steroid resistant nephrotic syndrome defined as absence of remission despite therapy with daily prednisolone at a dose of 2mg/kg/day for 4 weeks. Remission defined as urine albumin nil/trace in 3 consecutive early morning samples. Children less than 1 year of age, children with renal transplant and incomplete records were excluded. Period of study January 2013- December 2015. Informed consent was obtained and 75 cases who fulfilled the study criteria were included in this study. Variables assessed were incidence of hypertension (both at onset of disease and later during the course of disease), incidence of urinary tract infection and its microbiology, associated co-morbidities, complications of nephrotic syndrome and renal biopsy profile.Results: Incidence of hypertension at onset of disease was 13.3% and later during the course of the disease was 48%. Most common infection was UTI (28%) and the most common organism isolated in urine culture was E-coli. Incidence of other co-morbidities like asthma, atopy was 17.3%. No case had evidence of end stage renal disease. 60% of cases had undergone renal biopsy and minimal change disease was the most common biopsy finding.Conclusions: Hypertension and UTI remain important complications in nephrotic syndrome and hence all such children should be continued to be monitored for these complications. Minimal change disease (MCD) was the most common renal biopsy finding.

scholarly journals Rituximab Use in Nephrotic Syndrome

2018 ◽  
Vol 3 (1) ◽  
Keyword(s):  
Nephrotic Syndrome ◽  
Mycophenolate Mofetil ◽  
Side Effects ◽  
Calcineurin Inhibitor ◽  
Minimal Change ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Steroid Dependent ◽  
Minimal Change Glomerulonephritis ◽  
Steroid Dependent Nephrotic Syndrome

Background: Nephrotic syndrome may be caused by primary (idiopathic) renal disease or by a variety of secondary causes. Patients present with marked edema, proteinuria, hypoalbuminemia, and often hyperlipidemia. Treatment of most patients should include fluid and sodium restriction, oral or intravenous diuretics, and angiotensin-converting enzyme inhibitors. Adults with nephrotic syndrome may benefit from corticosteroid treatment. The treatment of patients with the steroid-resistant nephrotic syndrome (SRNS) and steroid-dependent nephrotic syndrome (SDNS) is challenging. On the basis of suggestions that B lymphocytes are crucial in the pathogenesis of the nephrotic syndrome, rituximab (a monoclonal antibody against CD20 antigen) is used in treatment of these patients. Aim of study: To evaluate the role of rituximaband mycophenolic acid in treatment of patientswith steroid-resistant (SRNS) and steroid-dependent nephrotic syndrome (SDNS), whom not respond or relapse after calcineurin inhibitor (CNI) (tacrolimus or cyclosporine) had been used. Patients and methods: Case series study was done between 2012 - 2015 in AL-Sadder Teaching Hospital Nephrology Center and record 40 patients with different age groups, males and females with different histopathological types (Minimal Change Glomerulonephritis, Focal Segmental Glomerulosclerosis, Mesengeo Prolifrative Glomerulonephritis). These patients were taking prednisilone and\or calcineurin inhibitor (tacrolimus “prograf”) or (cyclosporine “sandimmune”), and they get either Steroid Dependent Nephrotic Syndrome or Steroid Resistant Nephrotic Syndrome with frequent admission more than four time per year. To these patients we start rituximab intravenous infusion monthly for at least six months with the use of steroid and mycophenolate mofetil during these six months. The patients followed up for 3-12 months after initiation of rituximab by different investigations and the patients were classified according to their response into complete, partial and no response. After one year stop rituximab treatment, follow the patients clinically and by investigations for (1-2) years to determine which patients get relapse. Results: Majority (80%) of patients with nephrotic syndrome who had good response to rituximab were younger age group < 15 years. Better response to rituximab associated with Minimal Change Glomerulonephritis. There was significant reduction in blood urea, serum creatinine, urine (protein/creatinine) ratio and serum cholesterol. Serum albumin was significant elevated. Response to rituximab was not significantly associated with gender or steroid response. Majority of patients with good response not relapse and need more time for follow up. Relapsing after stopping rituximab not significantly associated with age, gender, histopathological type and steroid response. Conclusion: Rituximab and mycophenolate mofetil used in steroid-resistant nephrotic syndrome to get ride from side effects of calcineurine inhibitor (tacrolimus or cyclosporine). Rituximab and mycophenolate mofetil used in steroid-dependent nephrotic syndrome after calcineurine inhibitorto get ride from side effects of steroid. Improvement in renal function is result from stopping of calcineurine inhibitor (nephrotoxic drugs) and/or from rituximab and mycophenolate mofetil. Cost of rituximab is less than the cost that needed if the patients had frequent admissions to the hospital or developed renal failure and ended with dialysis.

scholarly journals Case Report: Novel Dietary Supplementation Associated With Kidney Recovery and Reduction in Proteinuria in a Dialysis Dependent Patient Secondary to Steroid Resistant Minimal Change Disease

2021 ◽  
Vol 9 ◽  
Author(s):  
Rasheed A. Gbadegesin ◽  
Loren P. Herrera Hernandez ◽  
Patrick D. Brophy
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Steroid Resistance ◽  
Small Subset ◽  
Disease Course ◽  
End Stage Kidney Disease ◽  
Steroid Resistant ◽  
End Stage

Minimal change disease (MCD) is the most common cause of nephrotic syndrome worldwide. For decades, the foundation of the treatment has been corticosteroids. However, relapse rate is high and up to 40% of patients develop frequent relapsing/steroid dependent course and one third become steroid resistant. This requires treatment with repeated courses of corticosteroids, and second and third line immunomodulators increasing the incidence of drug related adverse effects. More recently, there have been reports of a very small subset of Nephrotic Syndrome (NS) patients who are initially steroid sensitive and later become secondarily steroid resistant. The disease course in this small subset is often protracted leading ultimately to end stage kidney disease requiring dialysis or kidney transplantation. Unfortunately, patients with this disease course do not do well post transplantation because 80% of them will develop disease recurrence that will ultimately lead to graft failure. Few approaches have been tried over many years to reduce the frequency of relapses, and steroid dependence and there is absolutely no therapeutic intervention for patients who develop secondary steroid resistance. Nonetheless, their therapeutic index is low, evidencing the need of a safer complementary treatment. Several hypotheses, including an oxidative stress-mediated mechanism, and immune dysregulation have been proposed to date to explain the underlying mechanism of Minimal Change Disease (MCD) but its specific etiology remains elusive. Here, we report a case of a 54-year-old man with steroid and cyclosporine resistant MCD. The patient rapidly progressed to end stage kidney disease requiring initiation of chronic dialysis. Intradialytic parenteral nutrition (IDPN), albumin infusion along with a proprietary dietary supplement, as part of the supportive therapy, led to kidney function recovery and complete remission of MCD without relapses.

A rare cause of steroid-resistant nephrotic syndrome in a child: Answers

2019 ◽  
Vol 35 (4) ◽  
pp. 621-623
Author(s):  
Lale Guliyeva ◽  
Yılmaz Tabel ◽  
Ali Düzova ◽  
Nusret Akpolat ◽  
Seza Özen ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome

A case of minimal change disease during pregnancy – Benefits of early diagnosis and use of corticosteroids

2021 ◽  
pp. 1753495X2199021
Author(s):  
Priyanka S Sagar ◽  
Eddy Fischer ◽  
Muralikrishna Gangadharan Komala ◽  
Bhadran Bose
Keyword(s):  
Nephrotic Syndrome ◽  
Early Diagnosis ◽  
Renal Biopsy ◽  
Early Pregnancy ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Risk Of Bleeding ◽  
Increased Risk ◽  
Prompt Diagnosis ◽  
In Pregnancy

Nephrotic syndrome presenting in pregnancy is rare and poses a diagnostic and therapeutic challenge. Timing of renal biopsy is important given the increased risk of bleeding and miscarriage, and the choice of immunosuppression is limited due to the teratogenicity profiles of standard drugs. We report and discuss a case of minimal change disease diagnosed by renal biopsy during early pregnancy and treated with corticosteroids throughout the pregnancy. Prompt diagnosis and treatment of glomerular disease in pregnancy are vital to prevent poor maternal and fetal outcomes.

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.

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