Light chain proximal tubulopathy with lambda restriction presenting as acute kidney injury

Monoclonal gammopathies can produce a variety of glomerular, tubular, vascular and interstitial lesions. The spectrums of renal lesions produced by these monoclonal gammopathies include AL/AH amyloidosis, light chain cast nephropathy or myeloma kidney and various proximal tubulopathies. Out of these, proximal tubule centered lesions are much less identified and diagnosed and light chain proximal tubulopathy (LCPT) is one among them. In LCPT the excess free light chains (mostly kappa type by immunofluorescence microscopy) in serum are filtered by the glomeruli and are reabsorbed by proximal tubules causing its damage. These monoclonal light chains when sequestered in the proximal tubules can give rise to crystalline and noncrystalline histological variants. Here we present a rare case of noncrystalline variant of LCPT with lambda light chain restriction who presented with renal insufficiency which on later investigations revealed to be multiple myeloma

Acute Kidney Injury Secondary to Necrotizing Sarcoid Granulomatosis

Background. Sarcoidosis is a chronic disease characterized by noncaseating lesions involving any organ and tissue in the body. Hypercalcemia and acute kidney injury is a common renal presentation of sarcoidosis. Necrotizing sarcoid granulomatosis (NSG) is a granulomatous disease entity which presents with nodular masses of sarcoid like granuloma which primarily effects the lungs. It is a rare necrotizing variant of sarcoidosis. Extra pulmonary presentation of NSG is very rare. Case presentation. We present a 36-year-old female with hypercalcemia and acute kidney injury refractory to treatment. Whole body Flourine-18-fluorodeoxyglucose positron emission tomography computed tomography (18F-FDG PET/CT) showed increased metabolic uptake with ill-defined lesions in the liver, spleen, and pelvic lymph nodes. Biopsy of the ill-defined lesions in the liver showed necrotizing granulomatous lesions without angiitis. All the markers of tuberculosis were negative and angiotensin converting enzyme levels were elevated. Patient improved with 1 mg/kg/day oral steroid therapy and is on regular follow-up with minimal dose of steroids. Conclusion. Necrotizing sarcoid granulomatosis (NSG) is a rare systemic granulomatous disease. Due to its rarity and diagnostic difficulty, treatment is challenging for clinicians, pathologists and radiologists. Treatment of choice for symptomatic patients is steroid therapy. Prognosis is good with complete recovery

Deep vein thrombosis occurring early postrenal transplant

In kidney transplant recipients (KTRs), there can be an increased risk of thrombotic diseases. Here, we report a case of KTR, who developed ipsilateral acute iliofemoral deep vein thrombosis (DVT). A 38-year-old male with end-stage renal disease underwent deceased donor renal transplantation. He received antithymocyte globulin induction (2 mg/kg) and was maintained on tacrolimus, mycophenolate mofetil, and steroids. He had delayed graft function. One month posttransplant, the patient presented with right lower limb edema, with Doppler showing DVT of the right external iliac, common femoral, and proximal superficial femoral veins. He was treated with unfractionated heparin and underwent placement of retrieval filter in the infrarenal inferior vena cava (IVC) through the right internal jugular vein approach. Heparin was overlapped and switched to oral acenocoumarol. At 3 months postoperative, the patient is stable with no limb edema and serum creatinine of 0.8 mg/dl. A follow-up venous Doppler demonstrated the resolution of the thrombus. In conclusion, our patient developed DVT within 30-day postoperative period with no apparent risk factor and was successfully treated with anticoagulation and placement of IVC filter. Venography, thrombolysis, and thrombectomy pose challenges in the KTRs because of increased risk of adverse effects such as bleeding, contrast-induced nephropathy, and pulmonary embolism

A rare case of pancake kidney with glomerulonephritis

Developmental anomalies of the kidney and the urinary tract occur in 3-6 per 1000 live births. Pancake kidney is one of the rarest types of renal ectopia. Urinary system anomalies often coexist with malformations of other organs and systems. We report a case of pancake kidney which was detected incidentally while treating a patient with renal failure and nephrotic syndrome