Encapsulating Peritoneal Sclerosis (EPS) is a rare but serious complication of long-term peritone... more Encapsulating Peritoneal Sclerosis (EPS) is a rare but serious complication of long-term peritoneal dialysis (PD) that is associated with a high morbidity and mortality. EPS has been previously referred to as sclerosing peritonitis, sclerosing encapsulating peritonitis or the ‘abdominal cocoon’. EPS is characterised by diffuse thickening and sclerosis of the peritoneal membrane which leads to a decrease in ultrafiltration, ascites and ultimately partial or complete small bowel obstruction. The aetiology of EPS is unknown but it has been reported to be closely associated with the duration on PD, recurrent peritonitis, high transporter status and use of high concentration of glucose dialysis solutions. Stopping or switching from PD seems to be another trigger factor. Several treatment options including oestrogen and immunosuppressive therapy have been tried, but the mortality remains high at approximately 50%. In some cases, surgery has been attempted but this is associated with high ...
Indian Journal of Dermatology, Venereology and Leprology, 2019
References 1. Bernard P, Antonicelli F. Bullous pemphigoid: A review of its diagnosis, associatio... more References 1. Bernard P, Antonicelli F. Bullous pemphigoid: A review of its diagnosis, associations and treatment. Am J Clin Dermatol 2017;18:513‐28. 2. Walmsley N, Hampton P. Bullous pemphigoid triggered by swine flu vaccination: Case report and review of vaccine triggered pemphigoid. J Dermatol Case Rep 2011;5:74‐6. 3. Schmidt E, Zillikens D. The diagnosis and treatment of autoimmune blistering skin diseases. Dtsch Arztebl Int 2011;108:399‐405, I‐III. 4. Iwata H, Kitajima Y. Bullous pemphigoid: Role of complement and mechanisms for blister formation within the lamina lucida. Exp Dermatol 2013;22:381‐5. 5. Lear JT, Tan BB, English JS. Bullous pemphigoid following influenza vaccination. Clin Exp Dermatol 1996;21:392. 6. Fournier B, Descamps V, Bouscarat F, Crickx B, Belaich S. Bullous pemphigoid induced by vaccination. Br J Dermatol 1996;135:153‐4. 7. Hafiji J, Bhogal B, Rytina E, Burrows NP. Bullous pemphigoid in infancy developing after the first vaccination. Clin Exp Dermatol 201...
Correspondence: Dr Rozita Mohd Department of Medicine, PPUKM, Jalan Yaacob Latiff, Cheras, 56000 ... more Correspondence: Dr Rozita Mohd Department of Medicine, PPUKM, Jalan Yaacob Latiff, Cheras, 56000 Kuala Lumpur, Malaysia. Contact no: +60122022794, Fax no : +6091456934, Email : rozi8286@gmail.com INTRODUCTION Intravenous immunoglobulin (IVIG) is a valuable fractionated blood product that contains > 90% of IgG and 10% of Ig M and IgA. It is widely used in various spectrums of autoimmune diseases including systemic lupus erythematosus (SLE) with or without renal involvement. In Lupus Nephritis (LN), IVIG has been demonstrated to be beneficial at various stages of treatment. At present there is no consensus on the IVIG dosing intervals and treatment duration. The most commonly used is 400mg/kg/day in 4-5 divided doses (high dose). Even though there are centres reported beneficial effects with a low dose regimen (85mg/kg/day in 4-5 divided doses), it was commonly associated with poor response on thrombocytopenia, alopecia, vasculitic and proteinuria. Generally, IVIG is well tolerated...
Introduction: Hypovitaminosis D is reported to be associated with several medical complications. ... more Introduction: Hypovitaminosis D is reported to be associated with several medical complications. Recent studies have reported a high worldwide prevalence of Vitamin D deficiency in the general population (up to 80 %). This is even higher in patients with chronic kidney disease (CKD) and increases with advancing stages of CKD. Objectives: To determine the difference in serum Vitamin D [25-hydroxyvitamin D, 25(OH) D] levels between CKD patients and normal healthy population. Materials and Methods: A prospective cross-sectional study involving 50 normal volunteers (control) and 50 patients with CKD stages 2-4. Their demographic profiles were recorded and blood samples taken for serum 25(OH) D, intact parathyroid hormone (iPTH) and other routine blood tests. Results: All subjects regardless of renal status had hypovitaminosis D (< 30ng/mL). The mean serum 25(OH) D were comparable in the control and CKD groups (15.3 ± 4.2 ng/mL vs 16.1 ± 6.2 ng/mL, p = NS). However, within the Vitamin...
Introduction: Hyperuricemia is associated with chronic kidney disease (CKD) progression and poor ... more Introduction: Hyperuricemia is associated with chronic kidney disease (CKD) progression and poor cardiovascular outcomes. We studied the effect of febuxostat on estimated glomerular filtration rate (eGFR), proteinuria and monitored the safety profile of the medication. Material and Methods: This is a prospective open-label, randomized study in CKD stage 3 and 4 patients with diabetic nephropathy and asymptomatic hyperuricemia. Patients were randomized into febuxostat 40 mg daily and no treatment group using block randomization method and were followed up for 6 months. Their usual care for diabetes mellitus, hypertension and dyslipidemia were continued in the study. Blood and urine investigations were monitored at baseline, 3 months and 6 months. Results: The eGFR in febuxostat group was stabilized at 6 months with no significant reduction [26.2 (IQR 14.30) at baseline to 26.3 (IQR 15.2) ml/min/1.73 m2]. Whereas, there was a significant reduction of the eGFR in no treatment group fro...
Introduction: Post-transplant lymphoproliferative disorder (PTLD) is a recognized complication ex... more Introduction: Post-transplant lymphoproliferative disorder (PTLD) is a recognized complication exclusive to solid organ transplant recipients and carries a high mortality. Methods: We retrospectively reviewed records of all renal transplant recipients under follow up at our institution over the last seven years (2005-2011). We reviewed the patient characteristics, immunosuppression regimen and risk factors for the development of PTLD and its outcomes in our transplant cohort. Results: Four out of 63 patients were diagnosed with PTLD. PTLD was incidentally diagnosed on a transplant biopsy that was performed for an unexplained rise in serum creatinine in three patients. The fourth patient presented with left submandibular lymphadenopathy. Majority presented within 18 months of renal transplantation. After the diagnosis of PTLD on graft biopsy, all patients were fully investigated and two patients had systemic involvement. In the patients with systemic involvement, reduction of immunos...
Focal segmental glomerulosclerosis (FSGS) is one of the commonest causes of glomerulonephritis re... more Focal segmental glomerulosclerosis (FSGS) is one of the commonest causes of glomerulonephritis related end stage renal disease. FSGS can be primary or secondary and its management varies. Patients with primary FSGS who present with nephrotic syndrome need treatment with glucocorticoids alone or in combination with other immunosuppressive drugs such as calcineurin inhibitors, cyclophosphamide or mycophenolatemofetil. Alternative therapies that have been tried in refractory FSGS include rituximab and plasmapheresis. Most of the literature regarding plasmapheresis is recurrent FSGS in renal transplant patients. To the best of our knowledge, there is very limited data on plasmapheresis in refractory FSGS in the native kidneys. Herein we report a case of refractory FSGS in native kidneys that failed to respond to all immunosuppressive therapy and finally responded to regular plasmapheresis.
Introduction The alteration of the gut microbiome in the gut-kidney axis has been associated with... more Introduction The alteration of the gut microbiome in the gut-kidney axis has been associated with a pro-inflammatory state and chronic kidney disease (CKD). A small-scaled Italian study has shown an association between the gut microbiome and Immunoglobulin A Nephropathy (IgAN). However, there is no data on gut microbiota in IgAN in the Asian population. This study compares the gut microbial abundance and diversity between healthy volunteers and Malaysian IgAN cohort. Methods A comparative cross-sectional study was conducted involving biopsy-proven IgAN patients in clinical remission with matched controls in a Malaysian tertiary centre. Demographic data, routine blood and urine results were recorded. Stool samples were collected and their DNA was extracted by 16S rRNA gene sequencing to profile their gut microbiota. Results Thirty-six IgAN patients (13 male; 23 female) with the mean age of 45.5 ± 13.4 years and median estimated glomerular filtration rate (eGFR) of 79.0 (62.1–92.2) ml...
We reported a case of primary renal lymphoma (PRL) presented with non-oliguric acute kidney injur... more We reported a case of primary renal lymphoma (PRL) presented with non-oliguric acute kidney injury and bilateral kidney infiltrates in an individual with human immunodeficiency virus (HIV) disease. Acute kidney injury secondary to lymphoma infiltrates is very rare (less than 1% of hematological malignancy). A 37-year-old gentleman with underlying human immunodeficiency virus (HIV) disease was on combined antiretroviral therapy since diagnosis. He presented to our center with uremic symptoms and gross hematuria. Clinically, bilateral kidneys massively enlarged and were ballotable. Blood investigations showed hemoglobin of 3.7 g/L, urea of 65.6 mmol/L, and serum creatinine of 1630 µmol/L with hyperkalemia and metabolic acidosis. An urgent hemodialysis was initiated, and he was dependent on regular hemodialysis subsequently. Computed tomography renal scan showed diffuse nonenhancing hypodense lesion in both renal parenchyma. Diagnosis of diffuse large B cell lymphoma with germinal cent...
Hormone Molecular Biology and Clinical Investigation
Background Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory condition with... more Background Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory condition with multi-organ involvement predominantly affecting young women. There are very limited studies in pregnancy in Asian SLE patients and therefore we embarked on this study to identify pregnancy outcomes of Malaysian women with SLE. Materials and methods We performed a retrospective study of pregnancy outcomes in SLE patients in our institution from January 2007 to December 2014. A total of 71 pregnancies from 44 women were analysed. Results The mean age of our cohort was 30.5 ± 3.9 years. The rate of active disease at conception, antiphospholipid syndrome and lupus nephritis were 22.5%, 32.4% and 57.7% respectively. SLE flare occurred in 33 out of 71 pregnancies whereas 19 pregnancies were complicated with preeclampsia. The livebirth rate for our cohort was 78.9%, whilst preterm delivery was 42.9%. On univariate analysis, active disease and flare in pregnancy were both strongly associated wi...
Background: Neurological manifestations in Systemic Lupus Erythematous (SLE) varies and commonly ... more Background: Neurological manifestations in Systemic Lupus Erythematous (SLE) varies and commonly affects the Central Nervous System (CNS) rather than the peripheral nervous system. Neuropsychiatric or CNS manifestation can be as high as 24-54%, whereas the peripheral nervous system involvement is lower around 5-27%. Chronic Inflammatory Demyelinating Polyradiculopathy (CIDP) is one of the three commonest peripheral nervous system involvements in SLE patients and results with severe debilitating effects. However, it is rarely reported. Methods: A retrospective review of all SLE patients that were diagnosed with CIDP between 2000 and 2015 was done under follow up at our center that were diagnosed with CIDP between 2000 and 2015. We reviewed their medical records and analyzed their clinical presentation, investigations, treatment instituted, response to therapy and any neurological sequealae. Results: A total of 512 case notes were reviewed. Of these 4 patients presented with CIDP (3 f...
International Journal of Research in Medical Sciences
Background: Systemic lupus erythromatosus (SLE) is an autoimmune disease with 20–65% of patients ... more Background: Systemic lupus erythromatosus (SLE) is an autoimmune disease with 20–65% of patients developing lupus nephritis (LN). Studies have reported 10% of LN patients will end up with end stage renal disease and their mortality rate is higher compared to patients without LN. Abnormality of regulatory T cells (Tregs) level is thought to be a potential factor for this LN development. The aim of study was to evaluate the percentage of Tregs in LN patients.Methods: This was a comparative cross sectional study involving LN patients and age and gender matched controls with a 2:1 ratio. The patients were grouped into active and inactive LN based on their lupus activity index; complement levels, ANA, dsDNA antibodies, ESR, SLE Disease Activity Index (SLEDAI2K) score and also urine PCI (uPCI>0.05 for active group). Disease history, demographic data, routine blood test, peripheral blood for differentials count were taken and recorded. Peripheral blood mononuclear cells were stained wit...
Encapsulating Peritoneal Sclerosis (EPS) is a rare but serious complication of long-term peritone... more Encapsulating Peritoneal Sclerosis (EPS) is a rare but serious complication of long-term peritoneal dialysis (PD) that is associated with a high morbidity and mortality. EPS has been previously referred to as sclerosing peritonitis, sclerosing encapsulating peritonitis or the ‘abdominal cocoon’. EPS is characterised by diffuse thickening and sclerosis of the peritoneal membrane which leads to a decrease in ultrafiltration, ascites and ultimately partial or complete small bowel obstruction. The aetiology of EPS is unknown but it has been reported to be closely associated with the duration on PD, recurrent peritonitis, high transporter status and use of high concentration of glucose dialysis solutions. Stopping or switching from PD seems to be another trigger factor. Several treatment options including oestrogen and immunosuppressive therapy have been tried, but the mortality remains high at approximately 50%. In some cases, surgery has been attempted but this is associated with high ...
Indian Journal of Dermatology, Venereology and Leprology, 2019
References 1. Bernard P, Antonicelli F. Bullous pemphigoid: A review of its diagnosis, associatio... more References 1. Bernard P, Antonicelli F. Bullous pemphigoid: A review of its diagnosis, associations and treatment. Am J Clin Dermatol 2017;18:513‐28. 2. Walmsley N, Hampton P. Bullous pemphigoid triggered by swine flu vaccination: Case report and review of vaccine triggered pemphigoid. J Dermatol Case Rep 2011;5:74‐6. 3. Schmidt E, Zillikens D. The diagnosis and treatment of autoimmune blistering skin diseases. Dtsch Arztebl Int 2011;108:399‐405, I‐III. 4. Iwata H, Kitajima Y. Bullous pemphigoid: Role of complement and mechanisms for blister formation within the lamina lucida. Exp Dermatol 2013;22:381‐5. 5. Lear JT, Tan BB, English JS. Bullous pemphigoid following influenza vaccination. Clin Exp Dermatol 1996;21:392. 6. Fournier B, Descamps V, Bouscarat F, Crickx B, Belaich S. Bullous pemphigoid induced by vaccination. Br J Dermatol 1996;135:153‐4. 7. Hafiji J, Bhogal B, Rytina E, Burrows NP. Bullous pemphigoid in infancy developing after the first vaccination. Clin Exp Dermatol 201...
Correspondence: Dr Rozita Mohd Department of Medicine, PPUKM, Jalan Yaacob Latiff, Cheras, 56000 ... more Correspondence: Dr Rozita Mohd Department of Medicine, PPUKM, Jalan Yaacob Latiff, Cheras, 56000 Kuala Lumpur, Malaysia. Contact no: +60122022794, Fax no : +6091456934, Email : rozi8286@gmail.com INTRODUCTION Intravenous immunoglobulin (IVIG) is a valuable fractionated blood product that contains > 90% of IgG and 10% of Ig M and IgA. It is widely used in various spectrums of autoimmune diseases including systemic lupus erythematosus (SLE) with or without renal involvement. In Lupus Nephritis (LN), IVIG has been demonstrated to be beneficial at various stages of treatment. At present there is no consensus on the IVIG dosing intervals and treatment duration. The most commonly used is 400mg/kg/day in 4-5 divided doses (high dose). Even though there are centres reported beneficial effects with a low dose regimen (85mg/kg/day in 4-5 divided doses), it was commonly associated with poor response on thrombocytopenia, alopecia, vasculitic and proteinuria. Generally, IVIG is well tolerated...
Introduction: Hypovitaminosis D is reported to be associated with several medical complications. ... more Introduction: Hypovitaminosis D is reported to be associated with several medical complications. Recent studies have reported a high worldwide prevalence of Vitamin D deficiency in the general population (up to 80 %). This is even higher in patients with chronic kidney disease (CKD) and increases with advancing stages of CKD. Objectives: To determine the difference in serum Vitamin D [25-hydroxyvitamin D, 25(OH) D] levels between CKD patients and normal healthy population. Materials and Methods: A prospective cross-sectional study involving 50 normal volunteers (control) and 50 patients with CKD stages 2-4. Their demographic profiles were recorded and blood samples taken for serum 25(OH) D, intact parathyroid hormone (iPTH) and other routine blood tests. Results: All subjects regardless of renal status had hypovitaminosis D (< 30ng/mL). The mean serum 25(OH) D were comparable in the control and CKD groups (15.3 ± 4.2 ng/mL vs 16.1 ± 6.2 ng/mL, p = NS). However, within the Vitamin...
Introduction: Hyperuricemia is associated with chronic kidney disease (CKD) progression and poor ... more Introduction: Hyperuricemia is associated with chronic kidney disease (CKD) progression and poor cardiovascular outcomes. We studied the effect of febuxostat on estimated glomerular filtration rate (eGFR), proteinuria and monitored the safety profile of the medication. Material and Methods: This is a prospective open-label, randomized study in CKD stage 3 and 4 patients with diabetic nephropathy and asymptomatic hyperuricemia. Patients were randomized into febuxostat 40 mg daily and no treatment group using block randomization method and were followed up for 6 months. Their usual care for diabetes mellitus, hypertension and dyslipidemia were continued in the study. Blood and urine investigations were monitored at baseline, 3 months and 6 months. Results: The eGFR in febuxostat group was stabilized at 6 months with no significant reduction [26.2 (IQR 14.30) at baseline to 26.3 (IQR 15.2) ml/min/1.73 m2]. Whereas, there was a significant reduction of the eGFR in no treatment group fro...
Introduction: Post-transplant lymphoproliferative disorder (PTLD) is a recognized complication ex... more Introduction: Post-transplant lymphoproliferative disorder (PTLD) is a recognized complication exclusive to solid organ transplant recipients and carries a high mortality. Methods: We retrospectively reviewed records of all renal transplant recipients under follow up at our institution over the last seven years (2005-2011). We reviewed the patient characteristics, immunosuppression regimen and risk factors for the development of PTLD and its outcomes in our transplant cohort. Results: Four out of 63 patients were diagnosed with PTLD. PTLD was incidentally diagnosed on a transplant biopsy that was performed for an unexplained rise in serum creatinine in three patients. The fourth patient presented with left submandibular lymphadenopathy. Majority presented within 18 months of renal transplantation. After the diagnosis of PTLD on graft biopsy, all patients were fully investigated and two patients had systemic involvement. In the patients with systemic involvement, reduction of immunos...
Focal segmental glomerulosclerosis (FSGS) is one of the commonest causes of glomerulonephritis re... more Focal segmental glomerulosclerosis (FSGS) is one of the commonest causes of glomerulonephritis related end stage renal disease. FSGS can be primary or secondary and its management varies. Patients with primary FSGS who present with nephrotic syndrome need treatment with glucocorticoids alone or in combination with other immunosuppressive drugs such as calcineurin inhibitors, cyclophosphamide or mycophenolatemofetil. Alternative therapies that have been tried in refractory FSGS include rituximab and plasmapheresis. Most of the literature regarding plasmapheresis is recurrent FSGS in renal transplant patients. To the best of our knowledge, there is very limited data on plasmapheresis in refractory FSGS in the native kidneys. Herein we report a case of refractory FSGS in native kidneys that failed to respond to all immunosuppressive therapy and finally responded to regular plasmapheresis.
Introduction The alteration of the gut microbiome in the gut-kidney axis has been associated with... more Introduction The alteration of the gut microbiome in the gut-kidney axis has been associated with a pro-inflammatory state and chronic kidney disease (CKD). A small-scaled Italian study has shown an association between the gut microbiome and Immunoglobulin A Nephropathy (IgAN). However, there is no data on gut microbiota in IgAN in the Asian population. This study compares the gut microbial abundance and diversity between healthy volunteers and Malaysian IgAN cohort. Methods A comparative cross-sectional study was conducted involving biopsy-proven IgAN patients in clinical remission with matched controls in a Malaysian tertiary centre. Demographic data, routine blood and urine results were recorded. Stool samples were collected and their DNA was extracted by 16S rRNA gene sequencing to profile their gut microbiota. Results Thirty-six IgAN patients (13 male; 23 female) with the mean age of 45.5 ± 13.4 years and median estimated glomerular filtration rate (eGFR) of 79.0 (62.1–92.2) ml...
We reported a case of primary renal lymphoma (PRL) presented with non-oliguric acute kidney injur... more We reported a case of primary renal lymphoma (PRL) presented with non-oliguric acute kidney injury and bilateral kidney infiltrates in an individual with human immunodeficiency virus (HIV) disease. Acute kidney injury secondary to lymphoma infiltrates is very rare (less than 1% of hematological malignancy). A 37-year-old gentleman with underlying human immunodeficiency virus (HIV) disease was on combined antiretroviral therapy since diagnosis. He presented to our center with uremic symptoms and gross hematuria. Clinically, bilateral kidneys massively enlarged and were ballotable. Blood investigations showed hemoglobin of 3.7 g/L, urea of 65.6 mmol/L, and serum creatinine of 1630 µmol/L with hyperkalemia and metabolic acidosis. An urgent hemodialysis was initiated, and he was dependent on regular hemodialysis subsequently. Computed tomography renal scan showed diffuse nonenhancing hypodense lesion in both renal parenchyma. Diagnosis of diffuse large B cell lymphoma with germinal cent...
Hormone Molecular Biology and Clinical Investigation
Background Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory condition with... more Background Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory condition with multi-organ involvement predominantly affecting young women. There are very limited studies in pregnancy in Asian SLE patients and therefore we embarked on this study to identify pregnancy outcomes of Malaysian women with SLE. Materials and methods We performed a retrospective study of pregnancy outcomes in SLE patients in our institution from January 2007 to December 2014. A total of 71 pregnancies from 44 women were analysed. Results The mean age of our cohort was 30.5 ± 3.9 years. The rate of active disease at conception, antiphospholipid syndrome and lupus nephritis were 22.5%, 32.4% and 57.7% respectively. SLE flare occurred in 33 out of 71 pregnancies whereas 19 pregnancies were complicated with preeclampsia. The livebirth rate for our cohort was 78.9%, whilst preterm delivery was 42.9%. On univariate analysis, active disease and flare in pregnancy were both strongly associated wi...
Background: Neurological manifestations in Systemic Lupus Erythematous (SLE) varies and commonly ... more Background: Neurological manifestations in Systemic Lupus Erythematous (SLE) varies and commonly affects the Central Nervous System (CNS) rather than the peripheral nervous system. Neuropsychiatric or CNS manifestation can be as high as 24-54%, whereas the peripheral nervous system involvement is lower around 5-27%. Chronic Inflammatory Demyelinating Polyradiculopathy (CIDP) is one of the three commonest peripheral nervous system involvements in SLE patients and results with severe debilitating effects. However, it is rarely reported. Methods: A retrospective review of all SLE patients that were diagnosed with CIDP between 2000 and 2015 was done under follow up at our center that were diagnosed with CIDP between 2000 and 2015. We reviewed their medical records and analyzed their clinical presentation, investigations, treatment instituted, response to therapy and any neurological sequealae. Results: A total of 512 case notes were reviewed. Of these 4 patients presented with CIDP (3 f...
International Journal of Research in Medical Sciences
Background: Systemic lupus erythromatosus (SLE) is an autoimmune disease with 20–65% of patients ... more Background: Systemic lupus erythromatosus (SLE) is an autoimmune disease with 20–65% of patients developing lupus nephritis (LN). Studies have reported 10% of LN patients will end up with end stage renal disease and their mortality rate is higher compared to patients without LN. Abnormality of regulatory T cells (Tregs) level is thought to be a potential factor for this LN development. The aim of study was to evaluate the percentage of Tregs in LN patients.Methods: This was a comparative cross sectional study involving LN patients and age and gender matched controls with a 2:1 ratio. The patients were grouped into active and inactive LN based on their lupus activity index; complement levels, ANA, dsDNA antibodies, ESR, SLE Disease Activity Index (SLEDAI2K) score and also urine PCI (uPCI>0.05 for active group). Disease history, demographic data, routine blood test, peripheral blood for differentials count were taken and recorded. Peripheral blood mononuclear cells were stained wit...
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