MyMedR

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

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*

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

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

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 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 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

Renal diseases in pregnancy: a clinical review and appraisal

Sinnathuray TA

Med J Malaya, 1971 Jun;25(4):253-6.

PMID: 4261295

Matched MeSH terms: Nephrotic Syndrome/complications

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

Renal biopsies in children in HUSM, Kelantan: indications, results and complications

Van Rostenberghe H, Nik Abidin NZ, Samarendra S

Malaysian Journal of Child Health, 1997;9(2):166-169.

MyJurnal

During a period of three years (February 1995 --January 1998), 30 biopsies were performed for patients within the paediatric age group in Hospital Universiti Sains Malaysia (HUSM). The majority of these patients (19 cases) had steroid-resistant Nephrotic Syndrome. Other indications were lupus erythematosus (5 cases), acute or chronic glomerulonephritis (5 cases) and infantile nephrotic syndrome (1 case). The biopsy of the 19 cases of steroid-resistant nephrotic syndrome gave the following findings: 10 showed minimal- change nephrotic syndrome, 4 focal segmental glomerulosclerosis, 3 mesangial proliferative glomerulonephritis and one diffuse sclerosing glomerulonephritis while there was insufficient glomeruli for a conclusive diagnosis in one case. The 5 patients with acute/chronic glomerulonephritis showed diffused sclerosing glomerulonephritis. The other 5 patients with lupus nephritis showed mesangial proliferative glomerulonephritis (2) and severe proliferative glomerulonephritis (3). The 5-month-old child with infantile nephrotic syndrome showed mesangial proliferative glomerulonephritis. There were no severe complications noted during or immediately after the procedure. There were 3 cases of gross haematuria, one lasting less than 24 hours and the other two less than

Matched MeSH terms: Nephrotic Syndrome

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

Prophylactic antibiotics in children

Sinniah D

Med J Malaya, 1971 Dec;26(2):84-9.

PMID: 4260865

Matched MeSH terms: Nephrotic Syndrome/prevention & control

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

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

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

Nephrotic syndrome in adults

Cheong IKS

Family Practitioner, 1983;6:37-40.

Matched MeSH terms: Nephrotic Syndrome

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*

Nephrotic syndrome - an approach to management

Cheong IKS

Family Practitioner, 1981;4:28-33.

Matched MeSH terms: Nephrotic Syndrome

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 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

Displaying publications 1 - 20 of 35 in total