Immunoglobulin M Nephropathy in a Patient with Wilson's Disease.

Immunoglobulin M nephropathy (IgMN) is characterized by the deposition of immunoglobulin M in a dominant distribution in the renal glomeruli. Primary immunoglobulin M nephropathy is diagnosed after consistent light microscopy (LM), immunofluorescence (IF), electron microscopy (EM) results, and exclusion of known systemic disorders causing immunoglobulin M deposition in the glomeruli. The secondary disease has been reported with a few conditions though it has never been reported with any primary disease of the liver. We report the case of an adolescent male patient who presented with nausea, vomiting, diarrhea, and worsening anasarca. He was found to have nephrotic-range proteinuria that did not respond to conventional corticosteroid treatment. He was subjected to a renal biopsy which revealed a diagnosis of immunoglobulin M nephropathy. His liver function tests were deranged and an ultrasound scan of the abdomen revealed a coarse irregular liver. Workup revealed elevated urine copper excretion and a low ceruloplasmin level. He was diagnosed as a case of Wilson's disease and started on penicillamine and pyridoxine. He was also started on intravenous cyclophosphamide for the corticosteroid-resistant nephrotic syndrome to which he responded remarkably well. His edema settled, proteinuria resolved, and liver functions normalized. Currently, he is in remission and enjoying good health. To the best of our knowledge, we report the first known association between IgM nephropathy and Wilson's disease. It is presently not clear if causation can necessarily be established. This may be the result of defective IgM clearance by the liver or an altered metabolism of the antibody or immune complexes, as with hepatic-associated immunoglobulin M (IgM) nephropathy. Further studies are needed to elucidate the exact mechanism of this disease.

Exploring the process of final year objective structured clinical examination for improving the quality of assessment.

OBJECTIVE: To explore the process of final year Objective Structured Clinical Examination (OSCE) for improving the quality of assessment. METHODS: The analytical cross-sectional study was conducted with purposive sampling on one-year Medicine Objective Structured Clinical Examination (OSCE) scores of Final Year batch of 2009 at the Shifa College of Medicine, Islamabad. The scores from December 2008 to December 2009 of 77 Final Year students were analysed. The process of examination and the interpretation of the scores was evaluated using the Standards for Educational and Psychological Testing as the conceptual framework for validity testing which identifies five sources of test validity evidence. Internal consistency reliability of the examination was determined by Cronbach's alpha. Comparison and Correlation between students' end-of-clerkship (EOC) and end-of-year (EOY) examination scores were analysed by paired sample t-test and Pearson's Correlation Coefficient respectively. RESULTS: There was no significant positive correlation between the scores of end-of-clerkship and end-of-year Medicine Objective Structured Clinical Examination. Overall, the students' performance in the former segment was better. Evaluation of exam stations showed that mean scores significantly decreased in almost all end-of-year stations. Reliability decreased from 0.53 in the former to 0.48 in the latter assessment. Validity evidence showed that content validity was established by blueprinting of the objective exam. Response process evidence revealed that checklists, response key and rating scale were discussed with the faculty observing the stations. However, lack of other important sources of validity like standard setting for pass/fail criteria and poor reliability are serious threats to the validity of such exam scores. CONCLUSIONS: Multiple sources of validity evidence are needed to make defensible assumptions from performance scores. Consideration of all sources and threats to validity evidence is important to improve the quality of assessment.

The structure and function of a new clinical skills and medical informatics laboratory (SCIL) in a developing country--a two year institutional experience.

OBJECTIVE: To combine clinical skills and medical informatics learning by offering a combined 'SCIL' rotation to third year medical students and to determine its long-term impact. METHODS: A combined clinical skills and medical informatics laboratory (SCIL) was set up at our institution with international collaboration. Nine months to one year after formal third year SCIL rotations were conducted, a questionnaire rated on (1-5) Likert Scale was administered to the inaugural class undergoing this rotation. RESULTS: The rotation was rated positively in terms of both acquisition of clinical skills as well as medical informatics skills. (overall rotation rating: 3.32 +/- 0.53) CONCLUSION: Our results have shown the positive long-term impact on undergraduate medical students of a combined clinical skills and medical informatics rotation.

Preliminary experience with a new medicine morning report format incorporating multimedia and up-to-date.

To determine the impact of multimedia and up-to-date on internal medicine resident learning in morning report (MR), we converted our traditional medicine morning report to 'Up-to-date' programme incorporated and multimedia supported format which includes computer, multimedia projector and up-to-date CD ROM. A questionnaire was administered three months after the change and rated on a Likert scale. Preliminary experience demonstrated of a favourable overall resident and faculty perception and acceptance of the change.

Is there a need for establishing a formal nephrologist-directed primary care program in dialysis units?

We have introduced an annual timetable format for addressing the "primary care" needs of the hemodialysis population. For 102 patients enrolled, fourteen interventions adapted for the dialysis population from the US Preventive Services Task Force recommendations were implemented successfully in 65% areas in our pilot year, which include important features like annual history and physical examination, breast examination, mammography, pap smear, lipid profile, adult specific immunization and stool occult blood. Flexible sigmoidoscopy program was unsuccessful in our pilot year.

Mycophenolate Mofetil (MMF) Efficacy in Glomerulonephritis (GN), a Retrospective Analysis.

Mycophenolate Mofetil MMF has been widely used in post-transplant immunosuppression. Its role is emerging in GN. MMF demonstrated promising results compared with cyclosphosphamide in stage IV lupus nephritis, in a recently published trial. It has been found to have a wide safety profile, with mostly gastroinetestinal side effects, which can be avoided through titration. Its action is through inhibition of the enzyme IMDPH (ionosine monophosphate dehydrogenase), leading to purine antagonism and inhibition of lymphocytes. We were aiming to demonstrate the efficacy of MMF in our GN population. In this study, we reviewed 17 patients who received MMF (dose - 1 gm po bid) for the past year. They were only included if it was given for the management of resistant primary glomerulonephritis. Complete remission has been defined as proteinuria of less than 0.5 g/day and partial remission as a reduction of proteinuria 50% of starting MMF therapy; all 17 MMF therapy patients uniformly achieved good BP ((29%) achieved complete remission and this group consisted of 1 membranous GN, 2 lupus GN (type IV and membranous), one FSGS and one with MPGN. Four of 17 (23%) were non-responders to therapy. This group articles.aspx? id=41 to side effects. We conclude that the MMF appears to be an effective alternate treatment modality in resistant membranous GN, lupus nephritis (type IV and V) and possibly MPGN, and to a lesser extent in resistant FSGS. Further prospective data may demonstrate the efficacy of MMF in GN.

The effect of active nutritional counseling in improving biochemical nutritional parameters and fluid overload problems in maintenance hemodialysis patients.

Management of nutritional status is an integral part of the management of patients with renal disease on maintenance hemodialysis (HD). We investigated whether active nutritional counseling can improve biochemical nutritional parameters and fluid overload problems in patients on HD. A total of 110 patients, on three times per week HD, were enrolled in the study. Information regarding patient characteristics was collected with the help of a questionnaire. Patients with hypoalbuminemia, hyperkalemia, hyperphosphatemia, and more than three kilograms inter-dialytic weight gains were identified and were given active nutritional counseling. The above parameters were followed over a seven-month period. Active nutritional counseling resulted in significant decrease in the prevalence of hyperkalemia as well as high inter-dialytic weight gains (p < 0.001). However, the prevalence of hypoalbuminemia and hyperphosphatemia remained unchanged over the study period. Our study suggests that active nutritional counseling can improve certain important biochemical parameters and fluid overload problems in patients on maintenance HD.

Renal artery aneurysm: a case report and review of literature.

Renal artery aneurysm is an uncommon clinical occurrence. We report a 32-year-old lady with refractory hypertension who was found to have renal artery aneurysm. After a long clinical course, the aneurysm was successfully treated with coil embolization.