Case Reports in Nephrology最新文献

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.

Gadolinium-induced acute pancreatitis is a rare phenomenon associated with the administration of gadolinium-based contrast agents. Only five cases of gadolinium-induced acute pancreatitis have been reported worldwide in patients with native pancreas and none with a pancreatic graft. We present a 32-year-old woman with prior history of simultaneous pancreas-kidney transplant who presented with generalized abdominal pain associated with systemic inflammatory response syndrome requiring admission to the intensive care unit. This occurred within 48 hours after having a magnetic resonance imaging (MRI) with gadolinium for investigation of subacute left optic atrophy. She was noted to have a marked rise in serum lipase, and the computed tomography findings were consistent with acute graft pancreatitis. Other causes of pancreatitis were ruled out, and she was managed conservatively with aggressive hydration, bowel rest, and analgesia with good recovery. This is the first reported case of gadolinium-induced acute graft pancreatitis occurring in a simultaneous pancreas-kidney transplant recipient. Clinicians should consider this rare differential diagnosis as a cause of graft pancreatitis in patients who have received gadolinium-based contrast agents.

Acute tubulointerstitial nephritis (ATIN) can be caused by any number of factors, and it accounts for several percent of renal biopsy cases. In Japan, case reports exist, but there are few single-center series of ATIN cases. Case 1. A teenage male patient developed fever and cough on day X-61 and was found to have normal renal function and positive C-reactive protein (CRP) by his primary care physician. On day X-20, he presented with cough and nasal discharge in addition to low-grade fever, and his doctor noted renal dysfunction with serum creatinine of 2.12 mg/dL, negative urine occult blood, and positive urine glucose. Renal biopsy results showed diffuse interstitial nephritis with scarce glomerular involvement. There was no concurrent uveitis. Renal function normalized after 4 months of treatment with moderate-dose prednisolone. Cases 2-10. Of the 422 cases for which renal biopsies were performed at our institution from 2008 to 2021, acute tubulointerstitial nephritis was confirmed clinically and pathologically in 9 cases in addition to case 1, accounting for 2.4% of all biopsy cases. In the analysis of the 10 patients, the median age was 40 years old, eGFR at diagnosis was 19.4 (3.2-49.1) mL/min/1.73 m², and 2 of them underwent hemodialysis, but both were weaned from dialysis, and the eGFR after treatment was 53.6 (20.8-110.0) mL/min/1.73 m²; all patients showed improvement (P < 0.001). Treatment consisted of steroids in 8 patients and no steroids in 2 patients, the latter being treated by discontinuation of the suspect drugs and treatment of infection; 7 of the 10 patients were examined for ocular uveitis, and uveitis was diagnosed in 5 patients. The causes and clinical course of ATIN are diverse, but it is treated according to individual judgment in addition to standard treatment, and it generally has a good renal prognosis.

Mucormycosis is a rare invasive fungal infection with a high mortality rate caused by members of the family Mucoraceae. It mainly affects immunocompromised hosts such as poorly controlled diabetes mellitus, previous solid organ transplant, high-dose steroids, and hematologic malignancy. The most common sites of the disease are rhinocerebral, the skin, the lungs, and the gastrointestinal tract. In this era of COVID-19 infection, there has been a significant rise in invasive mucormycosis predominantly reported from southeast. We present a case of isolated renal mucormycosis in an apparently healthy individual with post-COVID-19 infection presenting as unilateral hydronephrosis. Timely identifying at-risk populations and having a high degree of suspicion with involvement of multidisciplinary teams are of utmost importance to diagnose and treat a rare and fatal infection. Even if there is a long history, antifungal drugs and removal of the source can result in a good outcome.

This was a case of a 39-year-old gentleman known to have diabetes mellitus since February 2021 on insulin glargine (Lantus) 16 units nocte and sitagliptin/metformin 50/500 mg once a day who presented to a tertiary teaching hospital in Kenya in May 2021 with a three-week history of vomiting and diarrhea. He had been previously admitted to a different facility with acute alcoholic pancreatitis. His examination was nonremarkable except for mild dehydration and pallor. He had moderate metabolic acidosis and deranged renal function. Prior to this, his creatinine was normal. As part of the evaluation for the rapid deterioration of renal function, a kidney biopsy performed revealed oxalate nephropathy. He was started on renal replacement therapy with hemodialysis.