MyMedR

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

Fulltext Renal vein thrombosis and the nephrotic syndrome

Segasothy M, Kamal A, Pang KS

Med J Malaysia, 1983 Jun;38(2):114-7.

PMID: 6621439

A 31 year old Chinese man developed the nephrotic syndrome, and wasfound to have some of the clinical features of renal vein thrombosis such as a rapid deterioration in renal function and great variability in proteinuria. Radiological studies confirmed the diagnosis of bilateral renal vein thrombosis. The clinical features and pathogenesis of renal vein thrombosis are discussed.

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

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 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

The pathology of the nephrotic syndrome in Malaysians

Prathap K, Looi LM, Lam KL, Wang F, Chua CT

Malays J Pathol, 1983 Aug;6:39-49.

PMID: 6599867

Matched MeSH terms: Nephrotic Syndrome/pathology*

Schistosoma japonicum-type infection in Malaysia--report of the first living case

Murugasu R, Wang F, Dissanaike AS

Trans R Soc Trop Med Hyg, 1978;72(4):389-91.

PMID: 705845

The first living patient with a Schistosoma japonicum-type infection who presented with the nephrotic syndrome is reported in detail. It is not clear whether the nephrotic syndrome was due to the schistosome infection or to the deposition of hepatitis B antigen and antibody complexes. This is the tenth case of schistosomiasis reported from aborigines in Malaysia and a sylvatic source of infection is suggested.

Matched MeSH terms: Nephrotic Syndrome/etiology

Renal disease in Kuala Lumpur. A study of 100 patients with renal biopsy

Mukherjee AP, Yuen TK

Med J Aust, 1971 Jan 30;1(5):257-60.

PMID: 5545973

Matched MeSH terms: Nephrotic Syndrome/epidemiology

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*

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

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

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 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

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

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*

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

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

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*

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*

Fulltext Pattern of lupus nephritis in Malaysia

Kong NCT, Cheong IKS, Chong SM, Suleiman AB, Morad Z, Lajin I, et al.

Med J Malaysia, 1988 Sep;43(3):200-5.

PMID: 3241577

Between 1980-1986, 219 renal biopsies were performed on patients with lupus nephritis (LN) presenting at the General Hospital, Kuala Lumpur. There were 172 (78.5%) females and 47 (21.5%) males. The ethnic distribution of 48.4% Malays, 46.1% Chinese and 5.5% Indians reflected their proportional composition in the general population. Peak incidence (40.6%) of cases occurred in the third decade of life (20-29 group) followed by 26.5% and 20.1% in the second and fourth decades respectively. The median age was 24 for females and 27 for males. In both sexes, nephrotic syndrome was the commonest mode of presentation (62.2%) followed by proteinuria (20.5%). Acute oliguric renal failure occurred in 11 patients (5%) and 8 of these showed crescentic glomerulonephritis with more than 50% crescents. The commonest histological picture was diffuse proliferative LN (WHO Stage IV-44.7%) which included 70% (19/27) of those with crescentic disease. This was followed by membranous LN (28.8%) of which 6 (all males) had crescentic disease. 7 (12.3%) of our patients had crescentic nephritis with a female to male distribution of 14: 13, suggesting either more aggressive disease or delayed diagnosis in males.
Key words - Renal biopsies, lupus nephritis, nephrotic syndrome, proteinuria.

Matched MeSH terms: Nephrotic Syndrome/etiology

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