• Gertsen Reece posted an update 6 months ago

    The association between amyloidosis and collagen vascular diseases, such as rheumatoid arthritis (RA) is well-documented. Amyloid goiter is an extremely rare pathologic condition caused by a massive amyloid infiltration of the thyroid tissue. Our patient had been diagnosed with RA 20 years ago and was on hemodialysis for 7 years. He was assessed for decreased appetite, dysphagia, and nausea during the hemodialysis. On physical examination, the thyroid was diffusely enlarged with multiple nodules. He was biochemically euthyroid. Ultrasound of the thyroid gland showed multinodular goiter. A total thyroidectomy was performed. Histopathological examination showed dilated follicles surrounded by abundant homogeneous substance that stained positive with Congo red. The patient was reported as amyloid goiter. Complaints of the patient improved after the surgery. In the literature, amyloid goiter with RA in a hemodialysis patient is very rare. Amyloid goiter should be considered if there is a rapid thyromegaly causing pressure symptoms in the background of any disease with chronic inflammation. Copyright © 2020 Indian Journal of Nephrology.A 28-year-old male, 3 years post renal transplant with stable graft function, presented with vomiting for 2 days. He had graft dysfunction and graft biopsy done revealed acute cell – mediated rejection BANFF-IA. After receiving glucocorticoids for rejection, he developed severe enterocolitis and impending respiratory failure. Chest X-ray and computed tomography of the chest revealed miliary mottling. Evaluation showed presence of filariform larvae of Strongyloides stercoralis in the stool and sputum. A diagnosis of Strongyloides Hyperinfection Syndrome (SHS) was made. After a prolonged course of treatment with noninvasive ventilation, broad-spectrum antimicrobials, parenteral ivermectin and oral albendazole therapy, he eventually recovered. This case report is to highlight that Strongyloides Hyperinfection Syndrome should also be considered in the differential in any immunocompromised patient presenting with miliary mottling in imaging. Copyright © 2020 Indian Journal of Nephrology.Membranous nephropathy is known to be associated with number of autoimmune diseases. Occurrence of PLA2R positive membranous nephropathy with sjogren’s syndrome and chronic inflammatory demyelinating neuropathy (CIDP) is quite rare. Role of PLA2R antigen in autoimmune diseases like sjogren’s syndrome and CIDP is largely unknown. Choice and initiation of immunosuppression if required may also be governed by the presence of other autoimmune diseases along with PLA2R positive membranous nephropathy. Copyright © 2020 Indian Journal of Nephrology.Acquired pure red cell aplasia (PRCA) following use of recombinant erythropoietin (rEPO) is distinctly rare and sporadically reported in the literature. We discuss a case of PRCA following the usage of rEPO (darbepoetin-α) during the management of anemia of chronic kidney disease in an elderly male subject with review of literature and a brief insight into proposed pathophysiologic mechanism, diagnosis, and management. Copyright © 2020 Indian Journal of Nephrology.Deranged coagulogram is a common problem, which a nephrologist faces before doing a renal biopsy. We describe a rare cause of coagulopathy in a patient with rapidly progressive renal failure due to acquired factor X deficiency caused by systemic light chain amyloidosis (AL). The patient had prolonged prothrombin and activated partial thromboplastin time, which got corrected on mixing with normal plasma, and factor X activity was markedly reduced at 5%. Rectal biopsy and immunofixation electrophoresis established the diagnosis of AL and the patient was started on bortezomib-based chemotherapy. Hence, appropriate coagulation work-up should be conducted in patients with renal dysfunction with prolonged coagulation times, as it can sometimes reveal the underlying diagnosis in situations where renal biopsy could not be done due to high risk of bleeding. Copyright © 2020 Indian Journal of Nephrology.Introduction Renal biopsy is primarily indicated in patients with diabetes mellitus (DM) with proteinuria, to diagnose non-diabetic renal disease (NDRD). However, Renal Pathology Society classification (RPSc) – 2010 has classified diabetic nephropathy (DN) into four classes of glomerular lesions with a separate scoring for tubulointerstitial and vascular lesions. selleck inhibitor Paucity of data from Indian subcontinent prompted us to plan this study to classify DN on biopsy as per the RPSc and correlate the clinical profile with histology. Materials and Methods Patients with DM who underwent renal biopsy for various indications (between Aug 2013 and Nov 2015) were included in the study. DN on histology was classified according to RPSc. Histopathology lesions of DN were correlated with clinical and biochemical profiles. Results Of the 267 patients studied, 252 (94.3%) were type 2 DM. NDRD alone was seen in 65 (24.34%), DN in 161 (60.3%), and NDRD with DN in 41 (15.3%). The most common indications for biopsy were rapidly progressive renal failure (76.7%) and nephrotic syndrome (16.4%). The most common glomerular class was class IV (43.5%), followed class III (41%), class II (13.3%), and class I (1.9%). The most common NDRD seen was acute interstitial nephritis (AIN) in 20.2% and is frequently associated with class III. Tubulointerstitial chronicity and not the arteriolar chronicity, was correlated with low estimated glomerular filtration rate (eGFR). Conclusions Most patients with DN subjected to renal biopsy were in class IV, and AIN was the most common NDRD. Only tubulointerstitial chronicity correlated with low eGFR. Copyright © 2020 Indian Journal of Nephrology.Introduction The Brescia-Cimino radiocephalic fistula, originally devised as a side-to-side anastomosis, is simple to perform and provides a smooth vein-artery interface but requires greater mobilization for vein-artery approximation than does the end-to-side approach. Distal cephalic vein ligation is performed to prevent venous hypertension. The study aims to assess primary patency at one month following distal side-to-side radiocephalic fistula creation with and without distal cephalic vein ligation, to assess the effect of distal cephalic vein ligation on flow rates at one month, and to document the occurrence of venous hypertension over one year follow up. Method A total of 100 patients requiring vascular access for hemodialysis were assigned to side-to-side anastomosis with and without distal cephalic vein ligation. Follow up Doppler exam was performed at one month for assessment of patency and flow rates and after one year clinical exam to document venous hypertension and the need for revision surgery. The quantitative and qualitative variables were evaluated using student t test and chi square test, respectively.

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