Cyclophosphamide for the Treatment of Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposition.

About 3% of the population aged more than 50 years, is affected by monoclonal gammopathy of undetermined significance (MGUS), a premalignant condition that may progress to lymphoproliferative disorders. Since MGUS does not represent the diseases associated with end organ damage, a new term, monoclonal gammopathy of renal significance (MGRS) is coined for the monoclonal gammopathies that are associated with renal disorders. MGRS is classified into various types, including monoclonal immunoglobulin deposition disease (MIDD) and proliferative glomerulonephritis with monoclonal immunoglobulin deposition (PGNMID). PGNMID presents with membranoproliferative glomerulonephritis-type lesions associated with immunoglobulin deposition. This disease entity has a poor prognosis and its optimum treatment is yet to be established. We present the case of an elderly male, a known patient of light chain deposition disease, a form of MIDD, who initially lost to follow-up but later presented with PGNMID, wherein he was treated with steroid and cyclophosphamide, to which he responded.

An Index Case of Diffuse Alveolar Hemorrhage Secondary to Chikungunya.

Chikungunya virus (CHIKV) is an alphavirus transmitted by mosquitoes, mostly by Aedes aegypti and Aedes albopictus. It is starting to become a very common entity in Pakistan, with a wide range of clinical manifestations. Here we report a case of a young male with CHIKV who presented with a clinical manifestation of diffuse alveolar hemorrhage which has not been observed so far in a patient suffering from this illness.

Frequency of maintenance hemodialysis patients meeting K/DOQI criteria for serum calcium, phosphorus, calcium phosphorus product and PTH levels; a single institutional experience from Pakistan: a cross sectional study.

INTRODUCTION: There is a great scarcity of literature in Pakistan investigating the proportion of end stage renal disease (ESRD) patients undergoing hemodialysis (HD) who meet the recommended kidney diseases outcome quality initiative (K/DOQI) guidelines for serum calcium (Ca), phosphorus (P), calcium phosphorus product (Ca x P) and parathyroid hormone (PTH) levels. Our study aimed to determine frequencies of patients who met the K/DOQI targets for these minerals at a tertiary care hospital's dialysis unit. METHODS: 111 ESRD patients on maintenance HD were selected from a tertiary care hospital. Serum Ca and P were assayed on chemistry analyser. PTH was measured through electrochemiluminescence sandwich method. Data were compared with K/DOQI targets and analysed using SPSS-21. RESULTS: The mean age of patients was 55.85 years (SD ± 13.95). Gender distribution was almost equal with 49.5% males and 50.5% females. The patients had mean corrected serum Calcium 9.12 ± 0.64 mg/dL, Phosphorus 4.57 ± 1.54 mg/dL and Parathyroid hormone 333.8 ± 278.4 pg/mL. The patients had achieved K/DOQI target ranges of Ca, P, PTH, Ca x P product and all 4 criteria in 63.1%, 47.6%, 38.7%, 84.7% and 10.8% respectively. CONCLUSION: Majority of patients on maintenance HD at our institution did not achieve the recommended K/DOQI target ranges. Further studies pertaining to the Asian subcontinent will prove resourceful for comparison of mineral metabolism and dialysis outcome of ESRD patients.

Renal Biopsy: A much needed tool in patients with Systemic Lupus Erythematosis (SLE).

BACKGROUND AND OBJECTIVE: Systemic lupus erythematosis (SLE) is an inflammatory disorder associated with significant morbidity and mortality. Kidneys are frequently affected in SLE and various stages of lupus nephritis have been identified based on severity of the disease. Treatment varies with the staging and correct diagnosis is essential for timely intervention as it can have significant impact on morbidity and mortality. The objective of the study was to determine whether laboratory parameters of lupus nephritis (LN); including urinalysis, serum creatinine (S. Cr) and 24 hours urine protein can accurately predict histologic staging of the disease. METHODS: This retrospective study was conducted in department of Nephrology, Liaquat National Hospital Karachi from January 2012 to December 2014. Fifty one patients of SLE who underwent renal biopsy were selected. Patients, urinalysis at the time of renal biopsy, serum creatinine and 24 hours urine collection for protein were noted. All patients renal biopsy was read by the same pathologist. Patients were clinically staged based on these parameters and their histologic staging based on biopsy findings were compared, to see their correlation. Data was analyzed using SPSS version 17. Chi-square test was used to analyze categorical data and p<0.05 was considered significant. Cohen's kappa (κ) analysis was used to examine the agreement by comparing lupus nephritis staging done by laboratory and histological ground. P value <0.05 indicates that agreement was unlikely due to chance alone. RESULTS: Among 51 patients analyzed, 37 patients were females (72.5%) and 14 patients were males (27.5%) with mean age of 32.51 + 16.91 years. In stage II, kappa (κ) of 0.304 represented fair strength of agreement and a p value of 0.012 (p<0.05)which was statistically significant. In stage III, kappa was 0.209 indicating none to slight agreement and a p value of 0.131 (p>0.05). In stage IV, kappa (κ) was 0.141 (slight agreement)and p value 0.301 (p>0.05) in stage V; kappa (κ) of 0.030 represented poor agreement and a p value of 0.828 (p>0.05). CONCLUSION: Staging of lupus nephritis done on basis of laboratory findings did not correlate well with underlying histological staging. Therefore, renal biopsy is an essential tool in approach to lupus nephritis in order to provide timely and appropriate treatment to patients.

Frequency of pulmonary hypertension in hemodialysis patients.

OBJECTIVE: Pulmonary hypertension (PH) has been described in hemodialysis (HD) patients and has been associated with increased morbidity and mortality. Our objective was to determine the prevalence of pulmonary hypertension in patients on regular hemodialysis. METHODS: This cross sectional study was conducted in Department of Nephrology, Liaquat National Hospital Karachi from April 2013 to March 2014. Eighty patients of end stage renal disease (ESRD), on maintenance hemodialysis (HD); underwent Trans thoracic Echocardiography were selected. Systolic pulmonary arterial pressure (SPAP) was recorded. Pulmonary hypertension was defined as, pulmonary artery pressure (PAP) greater than 30 mm Hg at rest. Pulmonary hypertension was further divided into mild (PAP b/w 30-45mmHg), moderate (PAP b/w 45-65mmHg) and severe pulmonary hypertension (PAP > 65mmHg). The effect of different vascular accesses, age, gender, dialysis vintage on the development of pulmonary hypertension was observed. RESULTS: Out of 80 patients, 45 patients (56%) had pulmonary hypertension (PH); 25(55.5%) had moderate, 13(29%) had mild, and 7 (15.5%) patients had severe pulmonary hypertension (PH). Pulmonary hypertension was present in 41(60%) patients with AVF, 3(27%) patients with tunnel cuffed catheter and 1 patient had AV bridge graft. Pulmonary hypertension was more common in females; present in 28 females (67%) and 17 males (45%), that was statistically significant (p<0.05). Mean duration of hemodialysis in (months) of patients with PH was 20.93 ± 12 vs. 10.29 ±10 in patients without PH (p<0.05). Age had no relation to development of PH. CONCLUSION: ESRD patients on HD have strong tendency to develop PH. Our study demonstrated that PH is more common among females. Duration of hemodialysis and AV access has strong relation to the development of PH.

CT guided percutaneous renal biopsy versus ultrasound guided for obtaining adequate tissue.

OBJECTIVE: To study the diagnostic yield of specimen obtained by percutaneous renal biopsy (PRB) under CT guidance and Ultrasound (US) guidance. METHODS: This study was conducted at the department of Nephrology at Liaquat National Hospital and Dr. Ziauddin Hospital, Karachi. Renal biopsy specimens obtained between January 2007 and September 2009 were studied for number of glomeruli obtained. In addition data was collected of how many patients had to undergo renal biopsy again because of nonavailability of renal cortex (the area of the kidney that contains glomeruli necessary for diagnosing renal disease) by both methods. RESULTS: We analyzed 205 renal biopsy specimens. Fifty were obtained via CT and 155 under US guidance. All 50 specimens obtained by CT guidance had renal cortex, compared to 147/155 (94.8%) specimen obtained by US guidance. Mean number of glomeruli in US guided specimens was 10.28 +/- 6.85, compared to CT guided specimen which was 23.34 +/- 13.42. Definitive diagnosis was made in 100% of CT guided biopsy compared to 94.8% (p<0.001) in US guided specimens. None of the patients undergoing CT guided biopsy required re-biopsy. CONCLUSION: PRB of native kidney under CT guidance is a more effective tool compared to ultrasound guidance in obtaining renal cortex that prevents patients from undergoing biopsy twice and provides sufficient number of glomeruli for definitive diagnosis of renal diseases especially when focal disease is suspected.