Renal biopsy is essential in the management of renal parenchymal diseases. Thus far there is no publish report on the pattern of glomerulonephritis in Kelantan. We decided to establish the pattern of glomerulonephritis in Kelantan and use this information as our reference in future studies. Records of patients who had proven glomerulonephritis histologically were analysed. Their biological data, clinical presentation, etiology and clinicopathological pattern were studied. Where appropriate mean and standard deviation were calculated. A total of 74 biopsies were performed during the study period (between January 1991 and December 1993), out of which 72 biopsies (97.3%) were considered suitable for analysis. The male to female ratio was 1:1.1. Mean age at presentation was 27.6 +/- 12.2 years. Nephrotic syndrome was the commonest clinical presentation (65.3%). The main underlying cause was systemic lupus erythematosus (50%) followed by primary glomerulonephritis. Histologically, IgA nephropathy and minimal change disease were the main patterns among patients with primary glomerulonephritis while diffuse proliferative glomerulonephritis was the commonest pattern among patients with lupus nephritis. Hence the pattern of glomerulonephritis is similar to other reported series. The procedure is considered safe and has a high success rate.
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.
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
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.
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.
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.
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.
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.
Adequately biopsied renal tissue received in the Department of Pathology, University Hospital, Kuala Lumpur from 1,000 consecutive Malaysian patients during an eleven year period between 1970 and 1981 was reviewed. The youngest patient was 6 days old and the oldest 80 years. Both sexes were equally represented. The majority of the patients were Chinese (71%) with Malays and Indians comprising most of the remainder. Over half the patients (50.4%) presented with the nephrotic syndrome. Other modes of presentation included systemic lupus erythematosus, proteinuria and haematuria separately or in combination and hypertension. Minimal change (25.7%) and proliferative glomerulonephritis (24.8%) were present in about equal numbers and together accounted for over half of the cases (50.5%). Lupus nephritis was the third most common diagnosis (18.4%). In addition, there were patients with focal glomerulonephritis (5.4%), membranous glomerulonephritis (5.5%), Berger's disease (5.8%), amyloidosis (0.6%) and end stage renal disease (4.0%).
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.
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.
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.
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.
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.
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.