MyMedR

Fulltext Effect of mycophenolate mofetil in steroid dependent and steroid resistant nephrotic syndrome patients in Pakistan

Iftikhar, E., Khan, Humayun I., Rabia, T., Sheikh, Shabbir A., Malik, Aaqil, Nor Iza A. Rahman

Malaysian Journal of Paediatrics and Child Health, 2011;17(1):0-0.

MyJurnal

Objective: To describe the effect of mycophenolate mofetil in Pakistani children with steroid dependent and steroid resistant nephrotic syndrome. Methods: This is cross sectional retrospective review of 16 patients; 9 boys and 7 girls (11 SD/FRNS and 5 SRNS) for a period of 4.8 years. This study was conducted in Mayo hospital and Fatima Memorial hospital specialist care centre, Lahore involving urban and suburban population. Results: The median age of the group was 4 years (1.6 to 12.6 years). Seven patients had histological diagnosis of MCN, 3 had diffuse mesangial proliferation, one of membranoproliferative glomerulonephritis and 4 had FSGS. Out of 5 SRNS 4 were found to have FSGS and 1 had membranoproliferative glomerulonephritis (MPGN). A total of three patients were completely off steroids and in two patients MMF was also successfully stopped. Number of relapses /patient /year calculated by applying Wilcoxan signed rank test was found to be 4.31 + 0.87(3.00-6.00 /patient/year) before starting MMF, which dropped to 1.12 + 0.718 (0.00- 2.000 /patient/year) after starting MMF, p=0.0001. Reduction in steroid dose from mean of 0.85 + 0.18 mg/kg/day to 0.3mg/kg/day + 1.56 was achieved in 12 months, p

Matched MeSH terms: Nephrotic Syndrome

Thymoma-associated myasthenia gravis and LGI1-encephalitis, with nephrotic syndrome post-thymectomy

Hor JY, Lim TT, Cheng MC, Chia YK, Wong CK, Lim SM, et al.

J Neuroimmunol, 2018 04 15;317:100-102.

PMID: 29395322 DOI: 10.1016/j.jneuroim.2018.01.011

Thymoma is associated with a wide spectrum of autoimmune paraneoplastic syndromes, though it is uncommon for multiple paraneoplastic syndromes to be present in a single individual. We report a rare case of an elderly gentleman who was found to have thymoma-associated myasthenia gravis and LGI1-encephalitis with myokymia, who presented with nephrotic syndrome (minimal change glomerulopathy) after thymectomy. The latter two paraneoplastic syndromes had manifested when prednisolone was tapered down to low dose. This case serves to remind neurologists that apart from paraneoplastic neurological manifestations, thymoma may also be associated with renal disease. Nephropathy in myasthenia patients with thymoma should be properly evaluated, as it is treatable with immunotherapy, and it may even occur post-thymectomy.

Matched MeSH terms: Nephrotic Syndrome

Fulltext Safety and Efficacy of Pneumococcal Vaccination in Pediatric Nephrotic Syndrome

Goonewardene ST, Tang C, Tan LT, Chan KG, Lingham P, Lee LH, et al.

Front Pediatr, 2019;7:339.

PMID: 31456997 DOI: 10.3389/fped.2019.00339

Nephrotic syndrome affects both children and adults. Idiopathic nephrotic syndrome is reported to be one of the most frequent renal pathologies in childhood. Nephrotic children are at high risk for severe pneumococcal infections as one of the life-threatening complications of nephrotic syndrome due to involvement of the immunosuppressive regimen and the acquired immune deficiency induced by nephrotic syndrome including decreased plasma IgG and low complement system components. Aiming to prevent pneumococcal infection is of paramount importance especially in this era of ever-increasing pneumococcal resistance to penicillins and cephalosporins. The pneumococcal vaccines currently available are inactivated vaccines-the two main forms in use are polysaccharide vaccines and conjugated vaccines. However, the data supporting the use of these vaccines and to guide the timing and dosage recommendations is still limited for nephrotic children. Thus, this review discusses the evidences of immunogenicity and safety profile of both vaccinations on nephrotic patients as well as the effect of nephrotic syndrome treatment on vaccine seroresponses.

Matched MeSH terms: Nephrotic Syndrome

Fulltext FAT1 mutations cause a glomerulotubular nephropathy

Gee HY, Sadowski CE, Aggarwal PK, Porath JD, Yakulov TA, Schueler M, et al.

Nat Commun, 2016 Feb 24;7:10822.

PMID: 26905694 DOI: 10.1038/ncomms10822

Steroid-resistant nephrotic syndrome (SRNS) causes 15% of chronic kidney disease (CKD). Here we show that recessive mutations in FAT1 cause a distinct renal disease entity in four families with a combination of SRNS, tubular ectasia, haematuria and facultative neurological involvement. Loss of FAT1 results in decreased cell adhesion and migration in fibroblasts and podocytes and the decreased migration is partially reversed by a RAC1/CDC42 activator. Podocyte-specific deletion of Fat1 in mice induces abnormal glomerular filtration barrier development, leading to podocyte foot process effacement. Knockdown of Fat1 in renal tubular cells reduces migration, decreases active RAC1 and CDC42, and induces defects in lumen formation. Knockdown of fat1 in zebrafish causes pronephric cysts, which is partially rescued by RAC1/CDC42 activators, confirming a role of the two small GTPases in the pathogenesis. These findings provide new insights into the pathogenesis of SRNS and tubulopathy, linking FAT1 and RAC1/CDC42 to podocyte and tubular cell function.

Matched MeSH terms: Nephrotic Syndrome/congenital*; Nephrotic Syndrome/genetics

Fulltext Fatal haemorrhagic chickenpox complicating nephrotic syndrome

Gangaram HB, Cheong IK

Med J Malaysia, 1993 Dec;48(4):446-8.

PMID: 8183171

We report a 14 year old Indian-Muslim girl who developed a fulminant, disseminated and fatal varicella infection while receiving steroids for nephrotic syndrome. The terminal phase of her illness was complicated by a bleeding dyscrasia and circulatory collapse. Varicella infection in healthy children is a benign disease. However in neonates and immunosuppressed patients it may be severe and often fatal. There are many reports of fatalities occurring in cancer patients receiving chemotherapy, patients on immunosuppressives for asthma, haemolytic anaemia, rheumatic fever, and renal and bone marrow transplantation. Patients with nephrotic syndrome receiving cyclophosphamide treatment are at particular risk of developing severe chickenpox infection. To our knowledge, there has been only one report of fatal chickenpox infection in a child who received steroids for nephrotic syndrome. We report here a case of fatal haemorrhagic chickenpox complicating nephrotic syndrome.

Matched MeSH terms: Nephrotic Syndrome/complications*; Nephrotic Syndrome/drug therapy

Fulltext Renal-limited AL amyloidosis - a diagnostic and management dilemma

Fuah KW, Lim CTS

BMC Nephrol, 2018 11 06;19(1):307.

PMID: 30400895 DOI: 10.1186/s12882-018-1118-8

BACKGROUND: Amyloidosis is a disorder caused by extracellular tissue deposition of insoluble fibrils which may result in a wide spectrum of symptoms depending upon their types, sites and amount of deposition. Amyloidosis can be divided into either systemic or localized disease.

CASE PRESENTATION: We present a case of a middle-aged gentleman who presented with persistent nephrotic syndrome with worsening renal function. Repeated renal biopsies showed the presence of renal-limited AL amyloidosis. Systemic amyloidosis workup was unremarkable apart from a slightly raised band of IgG lambda level with no associated immunoparesis. The nephrotic syndrome and renal histology did not improve over a 3-year period despite being given two courses of chemotherapies.

CONCLUSION: We hope that early recognition of this unusual localised presentation of renal- limited AL Amyloidosis and its poor response to conventional treatment can alert the nephrologist to the potential existence of this rare condition.

Matched MeSH terms: Nephrotic Syndrome/blood*; Nephrotic Syndrome/diagnosis*; Nephrotic Syndrome/therapy

Fulltext Primary focal segmental GlomerulosSclerosis and minimal change disease as one spectrum of disease

Fah, Then Ru, Jun, Tan Yi, Lim, Christopher Thiam Seong

Malaysian Journal of Medicine and Health Sciences, 2018;14(3):64-66.

MyJurnal

Minimal Change Disease (MCD) and Focal Segmental Glomerulosclerosis (FSGS) are common causes of nephrotic syndrome. These two conditions are similar in their presentations but differentiated via their histopathological features and responsiveness to corticosteroids. There are ongoing debates whether MCD and FSGS are at the same spectrum of disease rather than separate entities. FSGS has been postulated to be the severe end of the spectrum of MCD. We have reported a case that has primary FSGS after years of poorly controlled MCD, which supports both conditions are the same spectrum of disease.

Matched MeSH terms: Nephrotic Syndrome

Parental Knowledge on Nephrotic Syndrome and Disease Relapse in children

Diong SC, Syed Zakaria SZ, Rasat R, Wan Ismail WJ

Med J Malaysia, 2019 08;74(4):288-295.

PMID: 31424035

INTRODUCTION: Parental knowledge on nephrotic syndrome and disease relapse is important for early recognition and treatment of relapse to prevent the complications. Parental knowledge on nephrotic syndrome was reported to be inadequate from published studies. To date, there is no study on parental knowledge on childhood nephrotic syndrome in Malaysia. This study is thus aimed at to determine the level of knowledge on NS and disease relapse among parents of children with nephrotic syndrome and determine factors that influence knowledge on nephrotic syndrome and disease relapse.

STUDY DESIGN AND METHODS: This was a cross-sectional study conducted in Paediatric Nephrology Clinic, Hospital Selayang from November 2016 to November 2017. Seventy-eight parents were recruited based on universal sampling. Selfadministered questionnaire in Bahasa Malaysia and English was designed through focus group discussion with five subject matter experts and validated through content validity. Data was analysed using IBM SPSS Statistics 23.0.

RESULTS: Majority of parents or guardians (91%) were able to answer more than 50% of the questions correctly. Of these, 56% were able to answer more than 75% of the questions correctly. A 'cut-off' of 75% was defined as good knowledge. Parents of children with frequent relapses had higher parental knowledge and this was statistically significant (p=0.025).

CONCLUSION: Parental knowledge on nephrotic syndrome and disease relapse was still inadequate as only 56% parents had good knowledge. The main areas of deficit in parental knowledge were related to medications, infections, home urine dipstick monitoring, and recognition of warning signs during relapse.

Matched MeSH terms: Nephrotic Syndrome/diagnosis*; Nephrotic Syndrome/therapy

Five-year review of nephrotic syndrome in Singapore children

Chuan PL, Leng SC, Sinniah R

J Singapore Paediatr Soc, 1975 Oct;17(2):113-23.

PMID: 1207079

Matched MeSH terms: Nephrotic Syndrome/drug therapy*; Nephrotic Syndrome/genetics; Nephrotic Syndrome/epidemiology

Nephrotic syndrome - an approach to management

Cheong IKS

Family Practitioner, 1981;4:28-33.

Matched MeSH terms: Nephrotic Syndrome

Nephrotic syndrome in adults

Cheong IKS

Family Practitioner, 1983;6:37-40.

Matched MeSH terms: Nephrotic Syndrome

Immunotactoid glomerulopathy--an unusual deposition disease: report of the first Malaysian case

Cheah PL, Looi LM, Ghazalli R, Chua CT

Malays J Pathol, 1999 Jun;21(1):59-62.

PMID: 10879280

A 31-year-old Malay female presented with nephrotic syndrome without renal impairment. Renal biopsy features were in keeping with immunotactoid glomerulopathy (ITG). Non-Congophilic deposits were seen causing thickening of the glomerular capillary basement membrane with segmental accentuation, and widening of the mesangium. Immunofluorescence examination showed moderate amounts of IgG and C3 in the glomerular capillary walls with some in the mesangium. Ultrastructurally, 20-nm thick fibrils with microtubular organisation were present predominantly in the subendothelial region with similar fibrils in the mesangium. Although immunotactoid glomerulopathy and fibrillary glomerulonephritis (FG) have been recognised as entities with extracellular fibrillary material in the kidney, to date much remains to be clarified regarding these 2 conditions. While the renal biopsy findings in this patient are consistent with ITG, her clinical presentation is unlike that of usual ITG in that she is of a much younger age and has no associated haemopoietic disorder. Response to initial treatment of 8 weeks of prednisolone therapy was poor.

Matched MeSH terms: Nephrotic Syndrome/complications; Nephrotic Syndrome/pathology

Fulltext Mutations in KEOPS-complex genes cause nephrotic syndrome with primary microcephaly

Braun DA, Rao J, Mollet G, Schapiro D, Daugeron MC, Tan W, et al.

Nat Genet, 2017 Oct;49(10):1529-1538.

PMID: 28805828 DOI: 10.1038/ng.3933

Galloway-Mowat syndrome (GAMOS) is an autosomal-recessive disease characterized by the combination of early-onset nephrotic syndrome (SRNS) and microcephaly with brain anomalies. Here we identified recessive mutations in OSGEP, TP53RK, TPRKB, and LAGE3, genes encoding the four subunits of the KEOPS complex, in 37 individuals from 32 families with GAMOS. CRISPR-Cas9 knockout in zebrafish and mice recapitulated the human phenotype of primary microcephaly and resulted in early lethality. Knockdown of OSGEP, TP53RK, or TPRKB inhibited cell proliferation, which human mutations did not rescue. Furthermore, knockdown of these genes impaired protein translation, caused endoplasmic reticulum stress, activated DNA-damage-response signaling, and ultimately induced apoptosis. Knockdown of OSGEP or TP53RK induced defects in the actin cytoskeleton and decreased the migration rate of human podocytes, an established intermediate phenotype of SRNS. We thus identified four new monogenic causes of GAMOS, describe a link between KEOPS function and human disease, and delineate potential pathogenic mechanisms.

Matched MeSH terms: Nephrotic Syndrome/genetics; Nephrotic Syndrome/pathology

Fulltext Nephrotic syndrome in a patient with relapsed of chronic myeloid leukemia after peripheral blood stem cell transplantation

Bee PC, Gan GG, Sangkar VJ, Haris AR

Med J Malaysia, 2008 Mar;63(1):71-2.

PMID: 18935742 MyJurnal

Nephrotic syndrome (NS) is a well documented complication after allogeneic peripheral blood stem cell transplantation. It is usually due to autoimmune glomerulonephritis and thought to be a clinical manifestation of graft versus host disease. NS has also been reported to be associated with other hematological malignancies. We report a case of nephrotic syndrome in a patient who relapsed after allogeneic peripheral blood stem cell transplantation (PBSCT) for chronic myeloid leukemia (CML). The renal biopsy was suggestive of minimal change disease. There was no other evidence of graft versus host disease. He was treated with high dose prednisolone, with no response and finally succumbed to the underlying disease.

Matched MeSH terms: Nephrotic Syndrome/etiology*

Hemostatic abnormalities in nephrotic syndrome

Abdullah R

Vet. Clin. North Am. Small Anim. Pract., 1988 Jan;18(1):105-13.

PMID: 3282374

Nephrotic syndrome is often associated with a hypercoagulable state and thrombotic complications. Thrombosis may be due to a number of abnormalities in blood, including AT III deficiency, increased concentrations of fibrinogen, factors V and VIII, and platelet hyperaggregability. The therapeutic approach to thrombosis in nephrotic syndrome is the use of anticoagulants as a preventive measure or an attempt at thrombolysis with streptokinase, urokinase, or stanozolol.

Matched MeSH terms: Nephrotic Syndrome/blood; Nephrotic Syndrome/complications; Nephrotic Syndrome/veterinary*

Displaying publications 21 - 35 of 35 in total