Case Reports in Nephrology最新文献

PD-1/PD-L1 inhibitors such as pembrolizumab have radically improved the prognosis for many patients with advanced malignancies. Although revolutionary, its use can be complicated and limited by various immune-related adverse effects. Effective management depends on early recognition and prompt intervention. Herein, we describe a unique syndrome of hypercalcemia, with associated acute renal injury and hypoxic respiratory failure that was responsive to corticosteroids suggestive of immunotoxicity from pembrolizumab.

Ogilvie's syndrome, also known as acute colonic pseudo-obstruction (ACPO), is a rare, nonobstructive dilation of the colon of unclear etiology. We present the case of a patient who presented with Ogilvie's syndrome and significant hypokalemia due to colonic loss despite repletion. This case report demonstrates the difficulty in diagnosis, treatment, and outcome.

Mycobacterium tuberculosis (MTB) is an under-recognised cause of genitourinary disease. IgA nephropathy (IgAN), a leading cause of glomerulonephritis worldwide, has been described as a rare consequence of disseminated MTB infection. In this case report, we present the first case of MTB associated IgAN in Africa. Finding IgAN on kidney biopsy in an MTB endemic area should prompt a thorough investigation for MTB to increase the chance of remission of IgAN and prevent inappropriate use of immunosuppression.

We present the case of an 86-year-old Caucasian male with an 11-year history of low-grade chronic lymphocytic leukemia (CLL) presenting with nephrotic syndrome (NS). Renal biopsy findings showed a diffuse mesangial and endocapillary proliferative glomerulonephritis (GN) lesion with fine granular deposits, consistent with a rare morphologic variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID)-lambda light chain (LC) only. Monthly combination therapy of rituximab (500 mg/m² on day 1), fludarabine (30 mg/m² on days 1-3), and cyclophosphamide (750 mg/m² on days 1-3) was administered. Five courses of this regimen resulted in hematological remission, as well as a partial renal response with a reduction in the spot urine protein-to-creatinine ratio (UPCR) of 815.3 mg/g (reduction > 50% proteinuria without improvement in kidney function). This condition is a rare morphological variant of PGNMID, poorly described in CLL patients. We review the literature and suggest that this case provides sheds light on the unknown pathophysiological mechanisms of monoclonal immunoglobulins (MIg)-mediated glomerular damage in CLL patients, and may be helpful for the investigation of a more effective treatment.

Central diabetes insipidus (DI) is characterized by a deficiency in arginine vasopressin (AVP), an antidiuretic hormone leading to excessive free water loss in the urine and hypernatremia. Central DI can be the first presentation of several occult diseases. However, patients with central DI who have functioning thirst mechanisms and access to water may initially exhibit normal sodium levels. We report a 57-year-old woman who was admitted to the hospital due to cholangitis. Her initial serum sodium was normal and she rapidly developed severe hypernatremia after fluid restriction. The results of the laboratory workup indicated DI, which dramatically responded to desmopressin. MRI showed an ill-defined faint hyper signal intensity in T1, T2/FLAIR lesions involving the bilateral hypothalamus. The histopathological findings confirmed the diagnosis of Langerhans cell histiocytosis (LCH) with multiorgan involvement. Serum sodium returned to normal after receiving desmopressin and water replacement therapy.

Malignant pleural mesothelioma is rarely associated with nephrotic syndrome. Cryoglobulinemia is found in various pathological statuses, such as hepatitis C virus infection but rarely in malignant neoplasms. We recently encountered a patient with malignant mesothelioma coincident with nephrotic syndrome and cryoglobulinemia in the course of chemotherapy. A 60-year-old man employed as a building painter was diagnosed with malignant mesothelioma by lung biopsy two years earlier and was started on chemotherapy. Nivolumab seemed effective in controlling mesothelioma, but skin immune-related adverse events occurred during the course of treatment. After discontinuation of nivolumab and administration of gemcitabine as an alternative therapy, the patient was referred to a nephrologist because of the subsequent development of edema, renal injury, and proteinuria. Following the investigation, he was diagnosed with nephrotic syndrome and cryoglobulinemia with C4-dominant cold activation. However, a percutaneous renal biopsy could not be performed due to persistent severe cough induced by pleural involvement. The patient died a little over three years after the pathological diagnosis of pleural mesothelioma. Our case had three key features nephrotic syndrome was possibly associated with malignant mesothelioma; cryoglobulinemia occurred in malignant mesothelioma; and concomitant nephrotic syndrome and cryoglobulinemia occurred after chemotherapy. Unfortunately, our rare case lacks a basis in renal pathology or evidence of links between the pathogenesis of malignant mesothelioma, cryoglobulinemia, and nephrotic syndrome. This case does not provide a causal mechanism, but may be worth adding to the case list as one of the rare renal involvement in a patient with malignant mesothelioma.

Encapsulating peritoneal sclerosis (EPS) is a rare but rather serious complication of long-term peritoneal dialysis. The etiology of EPS is multifactorial, with long-term peritoneal dialysis, multiple peritonitis episodes, and uncontrolled hyperparathyroidism considered to be major risk factors for this often life-threatening condition. We report a case of a 55-year-old female patient with Down syndrome and end-stage renal disease (ESRD) on long-term peritoneal dialysis (PD) with extensive intestinal peritoneal calcifications and a rather uncomplicated long follow-up.

Spontaneous retroperitoneal bleeding is a rare occurrence that might have catastrophic implications. We present a 58-year-old male with a 4-day history of progressively worsening left-sided flank pain due to retroperitoneal hemorrhage from a nonfunctional renal paraganglioma. Abdominal contrast CT scan was helpful in locating the tumor, estimating tumor size and extent of bleeding, visualizing the interaction between the tumor and the surroundings, and ruling out any potential metastasis; however, it lacked specificity in identifying the origin of the mass, needing histologic investigation for a conclusive diagnosis. MRI was not available at our center. We report a rare case of spontaneous retroperitoneal bleeding as a complication of an unusual nonfunctional renal paraganglioma, which was initially misdiagnosed as renal cell carcinoma but later confirmed by postoperative histopathology.