Displaying publications 1 - 20 of 35 in total

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  1. Fulltext CD80 Insights as Therapeutic Target in the Current and Future Treatment Options of Frequent-Relapse Minimal Change Disease
    Teh YM, Lim SK, Jusoh N, Osman K, Mualif SA
    Biomed Res Int, 2021;2021:6671552.
    PMID: 33506028 DOI: 10.1155/2021/6671552
    Minimal change disease (MCD) is the most common cause of idiopathic nephrotic syndrome in children, and it is well known for its multifactorial causes which are the manifestation of the disease. Proteinuria is an early consequence of podocyte injury and a typical sign of kidney disease. Steroid-sensitive patients react well with glucocorticoids, but there is a high chance of multiple relapses. CD80, also known as B7-1, is generally expressed on antigen-presenting cells (APCs) in steroid-sensitive MCD patients. Various glomerular disease models associated with proteinuria demonstrated that the detection of CD80 with the increase of urinary CD80 was strongly associated closely with frequent-relapse MCD patients. The role of CD80 in MCD became controversial because one contradicts finding. This review covers the treatment alternatives for MCD with the insight of CD80 as a potential therapeutic target. The promising effectiveness of CD20 (rituximab) antibody and CD80 inhibitor (abatacept) encourages further investigation of CD80 as a therapeutic target in frequent-relapse MCD patients. Therapeutic-based antibody towards CD80 (galiximab) had never been investigated in MCD or any kidney-related disease; hence, the role of CD80 is still undetermined. A new therapeutic approach towards MCD is essential to provide broader effective treatment options besides the general immunosuppressive agents with gruesome adverse effects.
    Matched MeSH terms: Nephrotic Syndrome
  2. Fulltext IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome
    Trautmann A, Vivarelli M, Samuel S, Gipson D, Sinha A, Schaefer F, et al.
    Pediatr Nephrol, 2020 Aug;35(8):1529-1561.
    PMID: 32382828 DOI: 10.1007/s00467-020-04519-1
    Idiopathic nephrotic syndrome newly affects 1-3 per 100,000 children per year. Approximately 85% of cases show complete remission of proteinuria following glucocorticoid treatment. Patients who do not achieve complete remission within 4-6 weeks of glucocorticoid treatment have steroid-resistant nephrotic syndrome (SRNS). In 10-30% of steroid-resistant patients, mutations in podocyte-associated genes can be detected, whereas an undefined circulating factor of immune origin is assumed in the remaining ones. Diagnosis and management of SRNS is a great challenge due to its heterogeneous etiology, frequent lack of remission by further immunosuppressive treatment, and severe complications including the development of end-stage kidney disease and recurrence after renal transplantation. A team of experts including pediatric nephrologists and renal geneticists from the International Pediatric Nephrology Association (IPNA), a renal pathologist, and an adult nephrologist have now developed comprehensive clinical practice recommendations on the diagnosis and management of SRNS in children. The team performed a systematic literature review on 9 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, formulated recommendations and formally graded them at a consensus meeting, with input from patient representatives and a dietician acting as external advisors and a voting panel of pediatric nephrologists. Research recommendations are also given.
    Matched MeSH terms: Nephrotic Syndrome/diagnosis; Nephrotic Syndrome/drug therapy*
  3. Fulltext Ischemic Stroke in a Young Patient with Nephrotic Syndrome and Antiphospholipid Syndrome
    Neoh KK, Tang ASN, Looi I, Anita BM
    Case Rep Nephrol, 2020;2020:8828864.
    PMID: 33294240 DOI: 10.1155/2020/8828864
    We report a case of a 21-year-old man with underlying nephrotic syndrome (NS) secondary to minimal change disease, who developed an ischemic stroke with left hemiparesis. He received intravenous thrombolysis followed by a mechanical thrombectomy. After mechanical thrombectomy, he developed acute kidney injury which subsequently required haemodialysis. Further workup revealed that he had concomitant antiphospholipid syndrome (APS) and NS. He was started on vitamin K antagonist anticoagulant. This case report illustrates the importance of workup in identifying causes of ischemic stroke in a young patient.
    Matched MeSH terms: Nephrotic Syndrome
  4. 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
  5. 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
  6. 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
  7. Intravenous Rituximab in Severe Refractory Primary Focal Segmental Glomerulosclerosis
    Wee Leng G, Mustafar R, Kamaruzaman L, Mohd R, Cader RA, Wei Yen K, et al.
    Acta Med Indones, 2018 Jul;50(3):237-243.
    PMID: 30333274
    Managing primary or even secondary glomerulonephritis remains a challenge to many nephrologists. In primary focal segmental glomerulosclerosis (FSGS) with heavy proteinuria, renin aldosterone system blockade and high dose of oral prednisolone is the mainstay of treatment. Other immunosuppressive medications like Cyclophosphamide, Cyclosporine A and Mycophenolate Mofetil (MMF) are warranted if a complete remission is not achieved.  We illustrate a case of 21 year old gentleman with primary FSGS that was difficult to achieve remission despite on high dose steroid and oral Cyclophosphamide. He was also not responsive to a combination of MMF and Cyclosporine A (CSA) and even throughout the therapy he developed significant steroid and CSA toxicity. He presented to our center with severe nephrotic syndrome and acute kidney injury requiring acute haemodialysis. Despite re-challenged him again on high dose prednisolone, total of 2.4g of intravenous Cyclophosphamide, and MMF, he failed to achieve remission. He was subsequently given intravenous Rituximab 500mg/weekly for 4 doses and able to attained remission for 1 year. He relapsed again and a second course of Rituximab 500mg/weekly for 6 doses were given to attain remission. This case demonstrates the difficulty in managing refractory steroid dependent FSGS and we found that Rituximab is proven beneficial in this case to induce remission.
    Matched MeSH terms: Nephrotic Syndrome/complications*
  8. 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
  9. 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
  10. 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
  11. Fulltext Rapunzel syndrome in a paediatric patient
    Ngo, C.W., Syauki, H., Kumar, M.V.
    Journal of Surgical Academia, 2017;7(1):66-68.
    MyJurnal
    Rapunzel syndrome, or generically known as trichobezoar, is a rare condition. It usually happens among teenage population. We are presenting a case report of Rapunzel syndrome that happened in a 4-year-old child. She was initially investigated for nephrotic syndrome, as she had high blood pressure and hypoalbuminaemia. However, it was later found out to be a trichobezoar, indirectly causing both hypertension and malnutrition. This condition demanded a combination of surgical and psychiatric discipline for diagnosis and its treatment.
    Matched MeSH terms: Nephrotic Syndrome
  12. Fulltext Myeloma kidney – a treatable yet often forgotten disease
    Lim, Christopher Thiam Seong, Fuah, Kar Wah, Khoo, Yoong Khean
    Malaysian Journal of Medicine and Health Sciences, 2017;13(2):63-65.
    MyJurnal
    Multiple myeloma is a blood dyscrasias that accounts of almost 10% of all hematological malignancy. The presentation of myeloma kidney is highly variable and it often presents as renal insufficiency, renal tubular dysfunction and proteinuria of various types. In Malaysia the true incidence of myeloma kidney is unknown. Often the diagnosis of myeloma kidney was missed out despite the patient has sought medical treatment early. A high index of suspicion is required when the middle to elderly age patients present with unexplained renal impairment and enlarged kidneys. We present here the presentation of a rare subtype of myeloma in a relatively young patient whereby the patient presented with nephrotic syndrome and aoztemia.
    Matched MeSH terms: Nephrotic Syndrome
  13. 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
  14. Fulltext Kimura's Disease: A Rare Cause of Nephrotic Syndrome with Lymphadenopathy
    Othman SK, Daud KM, Othman NH
    Malays J Med Sci, 2011 Oct;18(4):88-90.
    PMID: 22589678
    Kimura's disease is a rare condition and typically presents as non-tender subcutaneous swellings in the head and neck region, usually in the pre-auricular and submandibular areas. It is associated with lymphadenopathy (both local and distal), marked peripheral eosinophilia, and an elevated IgE level. It can easily be mistaken for a malignant disorder. Fine needle aspiration can be misleading, and a diagnosis is established only by histopathological examination. Renal involvement, which may affect up to 60% of patients, is the only systemic manifestation. We report a case of Kimura's disease in a Malay patient who was associated with steroid-responsive nephrotic syndrome.
    Matched MeSH terms: Nephrotic Syndrome
  15. 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
  16. Arterial thrombosis and critical limb ischaemia in a case of nephrotic syndrome
    Koh KH, Tan C, Tan S, Ngu L
    Nephrology (Carlton), 2009 Sep;14(6):622.
    PMID: 19712262 DOI: 10.1111/j.1440-1797.2008.01001.x
    Matched MeSH terms: Nephrotic Syndrome/complications*
  17. 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*
  18. Fulltext Subclinical acquired syphilis masquerading as membranous glomerulonephritis
    Soehardy Z, Hayati SN, Rozita M, Rohana AG, Halim AG, Norella K, et al.
    Med J Malaysia, 2006 Oct;61(4):484-6.
    PMID: 17243528 MyJurnal
    Membranous glomerulonephritis (MGN) is one of the common forms of nephrotic syndrome in the adult population. The majority of MGN are idiopathic, but the secondary forms can be seen in the setting of autoimmune disease, neoplasia, infection and following exposure to certain therapeutic agents. Histologically, MGN is an immunologically mediated disease in which immune complexes deposit in the subepithelial space. Syphilis is a venereal disease that can also be acquired by exposure to infected blood. Untreated syphilis may progress and develop renal complications such as membranous glomerulonephritis (MGN) or diffuse endocapillary glomerulonephritis with or without crescent formation. Today, with increasing awareness of sexually transmitted diseases especially HIV infection coupled by the practice of protected sexual intercourse and advancement of medicine, we have seen fewer and fewer cases of acquired syphilis. Furthermore, majority will present with typical syphilitic symptoms of such as chancre, rash, fever and lymph node enlargement in which case the diagnosis is easily obtained. We are reporting a case of acquired syphilis masquerading as membranous glomerulonephritis without typical syphilitic symptoms.
    Matched MeSH terms: Nephrotic Syndrome/diagnosis
  19. Fulltext Interferon induced glomerular disease in a patient with chronic hepatitis C
    Suresh RL, Suryati Y, Merican I
    Med J Malaysia, 2003 Oct;58(4):594-6.
    PMID: 15190636
    Chronic hepatitis C manifests with many extrahepatic features including renal involvement. However, less commonly, interferon therapy for chronic hepatitis C can also result in renal involvement and we describe a case when interferon therapy resulted in minimal change glomerulopathy, a form of involvement which, carries a good prognosis. Our patient developed nephrotic syndrome while on interferon therapy and HCV RNA levels were undetectable at that time. The disease showed excellent response to steroid therapy.
    Matched MeSH terms: Nephrotic Syndrome/chemically induced*
  20. 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
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