Indian Journal of Nephrology最新文献

Podocyte infolding glomerulopathy (PIG) is a rare pathological entity, diagnosed by electron microscopic demonstration of diffuse infolding of the podocytes into the glomerular basement membranes. We report the first case from United Kingdom exhibiting typical ultrastructural features of PIG in a male with Type II diabetes mellitus, hypertension and common variable immune deficiency. Renal biopsy revealed phospholipase A2 receptor (PLA2R) immunostain positive membranous nephropathy (MN) but no serum PLA2R antibodies. Diffuse infolding of the podocytes into the glomerular basement membranes along with pathognomonic microspherular and microtubular intra basement membrane clusters distributed diffusely and globally were noted on electron microscopy, diagnostic of PIG. We postulate a shared pathomechanistic link between PIG and MN, highlighting the overlapping features of both conditions.

Adenine phosphoribosyltransferase (APRT) enzyme deficiency is a rare inborn metabolic error causing an accumulation of 2,8 dihydroxyadenine (DHA), leading to kidney stones and crystal nephropathy. If untreated, it progresses to end stage renal disease (ESRD) with a subsequent risk of crystal nephropathy recurrence post-renal transplantation. Recurrence post-transplant can be prevented, and allograft outcomes can be improved if treatment with an xanthine oxidoreductase (XOR) inhibitor is initiated before or at the time of kidney transplantation. We describe a case involving a 24-year-old male with ESRD, found to have APRT enzyme deficiency during transplant evaluation, successfully managed with pre- and post-transplant XOR inhibitors to prevent recurrence, resulting in a positive allograft outcome.

Urine sediment analysis is a highly valuable yet underutilized test in today's advanced medical landscape. The analysis of urine sediment is a simple, cost-effective, and powerful diagnostic tool in the hands of a skilled nephrologist, generally in all kidney diseases and particularly more so in the setting of acute kidney injury (AKI). The impact of AKI is far-reaching and encompasses elevated mortality rates, increased morbidity, longer hospital stays, and higher overall healthcare expenses. Timely and compartmental diagnosis of AKI with the use of a simple urine sediment analysis leads to targeted therapeutic strategies and also serves as a prognostic guide. The widespread use of automated analysis in recent times has its own set of limitations, as it fails to identify pathological casts, crystals, and dysmorphic red blood cells (RBCs). Hence, it is the need of the hour to learn this time-honored art of urine sediment analysis, to provide comprehensive patient care.

Chronic kidney disease in immune thrombocytopenia (ITP) is uncommon, and renal transplant in this setting is rare. We discuss the successful renal transplant of a 29-year-old male with chronic ITP. During transplant, he was managed with thrombopoietin receptor agonist eltrombopag, intravenous methylprednisone, and intravenous immunoglobulin to maintain adequate platelet level. He recovered well with no major complications and good graft function and has been stable during the follow-up period. The case report highlights that renal transplantation is a feasible option in patients with ITP, even in the presence of low platelet counts.

Systemic lupus erythematosus (SLE) is an autoimmune disease that can involve multiple organ systems. The most common form of vasculitis seen in SLE is small vessel vasculitis. Aortitis in SLE or antiphospholipid syndrome is an extremely rare complication. Here, we present a 32-year-old female who presented with a history of prolonged abdominal pain, who was evaluated and diagnosed to have aortitis as an unusual involvement in SLE with secondary antiphospholipid antibody syndrome.