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  • 机译 生酮饮食诱导的哺乳期妇女严重酮症酸中毒:病例报告和文献复习。
    摘要:The ketogenic diet (KD) is a high-fat, adequate-protein, and low-carbohydrate diet that leads to nutritional ketosis and weight loss. It is known to induce ketosis but is not an established cause of clinically significant ketoacidosis. Lactation ketoacidosis is well established in bovine literature but remains a rare phenomenon in humans. Here we present a life-threatening case of severe ketoacidosis in a nondiabetic lactating mother on a strict ketogenic diet. We review the available case reports of lactation ketoacidosis in humans and the mechanisms thereof. Although ketogenic diet has been shown to be safe in nonpregnant individuals, the safety of this diet in lactating mothers is not known. Health professionals and mothers should be made aware of the potential risk associated with a strict ketogenic diet when combined with lactation. Prompt diagnosis and immediate treatment cannot be overemphasized. To our knowledge, this is the first reported case of life-threatening lactation ketoacidosis associated with ketogenic diet while consuming an adequate number of calories per day.
  • 机译 镧诱导的不透射线的肠道沉淀物:肠道异物的潜在原因
    摘要:Lanthanum carbonate is a commonly prescribed oral phosphate binder for use in patients with acute or chronic kidney disease. The elemental form of lanthanum is a soft metal, which will appear radiopaque on a standard X-ray radiograph. This case report illustrates the potential for Lanthanum to masquerade as multiple radiopaque intestinal foreign bodies, leading to the extensive mobilization of medical resources and consultations including serial X-ray monitoring, multiple consultants including acute care and colorectal surgery. Given the few published reports describing this finding in the literature, it is essential to consider Lanthanum precipitates in the differential diagnosis of radiopaque intestinal foreign bodies in patients with chronic kidney disease to avoid unnecessary utilization of medical resources for this predominantly benign condition.
  • 机译 预防性治疗:成功治疗两名患者的慢性肾脏病罕见并发症
    摘要:Calciphylaxis, or calcification uremic arteriolopathy, is a rare disease thought to occur due to arteriolar calcifications of the dermis and is responsible for ischemia with cutaneous necrosis and painful panniculitis. Its mechanism remains poorly understood which makes its management challenging and difficult to standardize. We report our management of two patients diagnosed with calciphylaxis. In one patient, calciphylaxis was mentioned upon admission given the context of preexisting secondary hyperparathyroidism and the existence of multiple risk factors. In both patients, the diagnosis was confirmed histologically. Our two observations highlight the difficulty of the diagnosis and the complexity of the therapeutic management that has been personalized according to patient characteristics and clinical evolution. Several therapeutic means can be implemented once the diagnosis is made; nevertheless, its prognosis remains pejorative despite the therapeutic advances. Broad debridement, good phosphocalcic balance control, and the correction of the risk factors top the list of any therapeutic strategy. One of the major challenges of the therapy is normalizing the calcium-phosphate balance. Thus, Cinacalcet and sodium thiosulfate appear to be promising treatments.
  • 机译 严重低钠血症过度矫正后渗透性脱髓鞘综合征的临床解决
    摘要:Osmotic Demyelination Syndrome (ODS) occurs after rapid overcorrection of severe chronic hyponatremia usually in those with a predisposition such as chronic alcoholism, malnutrition, or liver disease. Rarely, do patients make a full recovery. We report a case of ODS secondary to overcorrection of severe hyponatremia with pathognomonic clinical and radiologic signs making a complete neurological recovery. A detailed course of events, review of literature, and optimal and aggressive management strategies are discussed. There is some controversy in the literature regarding the prognosis of these patients. Our aim here is to show that, with aggressive therapy and long-term care, recovery is possible in these patients.
  • 机译 一名年轻的非洲裔美国男性的急性肾脏衰竭
    摘要:Retroperitoneal fibrosis (RPF) is a condition characterized by chronic inflammatory and fibrotic changes in the retroperitoneum that can lead to serious complications including kidney failure, mesenteric and limb ischemia, and deep venous thrombosis among others. Affected individuals may present with nonspecific symptomology that would require a high clinical index of suspicion for prompt diagnosis. We herein discuss a case of a young African-American man with recurrent deep venous thrombosis who presents with a 4-week history of constant aching pain of abdomen and back and kidney failure. Initial noncontrast computed tomogram (CT) only revealed mild bilateral hydroureteronephrosis with inflammatory changes but without obvious mass or lymphadenopathy. At the insistence of the renal consulting team to rule out RPF, a CT-urogram was performed which revealed an infiltrative mass encasing the aorta, inferior vena cava, and common iliac vessels. Laparoscopic biopsy revealed dense fibroadipose tissue, lymphocytic aggregates, focal scattered IgG4-positive plasma cells, and fibrin deposition. Patient underwent bilateral nephrostomy placement and empirical corticosteroid therapy with resolution of kidney failure. Our case illustrates a classic presentation of RPF with relatively benign findings on noncontrast CT that could have been missed if clinicians did not keep a high index of suspicion for the condition.
  • 机译 在肾-胰腺移植受者中,晚期发作的浆细胞瘤样PTLD表现为急性高血糖症
    摘要:Allograft infiltration has been described in up to 20% of all patients with posttransplant lymphoproliferative disorder (PTLD), most representing EBV-positive B-cell lymphomas. Plasma cells are often observed in humoral rejection biopsies, but graft infiltration by plasmacytoma-like PTLD is rare. We report the case of a 54-year-old simultaneous pancreas-kidney transplant recipient (immunosuppression: OKT3, methylprednisolone, cyclosporine, and azathioprine), diagnosed with an IgG-kappa monoclonal gammopathy of undetermined significance eighteen years after transplant. Nine months later, pancreas allograft biopsy performed due to new-onset hyperglycemia (HgA1C 8.6%, C-peptide 6.15ng/mL and anti-GAD 0.9UI/mL) revealed a monotypic plasma cell infiltrate, CD19, CD79a, CD138 positive, with IgG-kappa light chain restriction, and EBV negative. PET-scan FDG uptake was limited to pancreas allograft. Tumor origin could not be established (using DNA microsatellite analysis). Despite treatment with bortezomib and dexamethasone, patient eventually died one month later. This is the first report of a late onset extramedullary plasmacytoma involving a pancreas allograft.
  • 机译 透析患者严重地低血钙症和地诺单抗后甲状旁腺激素的剧增:一例病例报告并文献复习
    摘要:Chronic kidney disease-mineral and bone disorder (CKD-MBD) is frequently present in advanced stages of chronic kidney disease (CKD) patients with high risk of fracture and elevated socioeconomic burden. Denosumab, an injectable human monoclonal antibody with affinity for nuclear factor-kappa ligand (RANKL), is an effective treatment for osteoporosis in postmenopausal women and men. Unlike the bisphosphonates, the pharmacokinetics and pharmacodynamics of denosumab are not influenced by the renal function and are being increasingly used for patients having CKD-MBD with low bone mineral density (BMD) to reduce the risk of fragility fractures. Hypocalcemia is a known side effect of this drug along with compensatory increase in parathyroid hormone (PTH). However, limited information is available in the literature regarding this potentially life-threatening side effect with denosumab in end-stage renal disease (ESRD) patients on dialysis. We present a patient with ESRD on peritoneal dialysis who developed severe symptomatic hypocalcemia and dramatic increase in PTH following denosumab therapy. She was conservatively managed with calcium supplementation and appropriate adjustment in calcium dialysate. We have also reviewed the literature on the use of denosumab in dialysis patients and looked at additional factors that may precipitate severe hypocalcemia in these patients. We believe that denosumab should be used with caution in dialysis patients since it may lead to profound hypocalcemia. Clinicians should ensure special attention in recognizing patients at risk of developing this serious adverse effect, so that prompt treatment and preventive strategies can be implemented.
  • 机译 21岁的人患有膜增生性肾小球肾炎和肾病综合征的门静脉血栓形成。
    摘要:Membranoproliferative glomerulonephritis, one of the main causes of nephrotic syndrome, is associated with a state of hypercoagulability that leads to increased risk of thrombotic events. Portosystemic collaterals may reopen due to reversal of the flow within the existing veins and be a presenting feature of thrombosis. We describe a patient who presented with large portosystemic collaterals and signs of portal hypertension and was subsequently found to be affected by membranous proliferative glomerulonephritis. Proteinuria and microscopic haematuria in a patient with signs of portal hypertension and no pre-existing liver disease should raise the suspicion of an underlying kidney disease.
  • 机译 醋酸ir酯引起的高钙血症:未报告的不良反应
    摘要:Hyperkalemia, a potential life threating condition, is a commonly encountered problem in chronic kidney disease (CKD) patients. Patiromer acetate, a nonabsorbable cation exchange polymer, is a gastrointestinal agent for chronic therapy in patients with persistent hyperkalemia. Patiromer is generally well tolerated in patients; common side effects are gastrointestinal, such as diarrhea, constipation, flatulence, and vomiting. Hypercalcemia, although a theoretical possibility, has not been reported in any major clinical trials. We present a case of hypercalcemia associated with patiromer acetate used for treatment of hyperkalemia in a stage IV CKD patient. Clinicians should be aware of the possibility of hypercalcemia while taking patiromer.
  • 机译 利妥昔单抗治疗成人肾病综合征后的迟发性中性粒细胞减少症
    摘要:A 41-year-old woman developed nephrotic syndrome at the age of 32 and was diagnosed with minimal change nephrotic syndrome based on a renal biopsy. Although remission was achieved with administration of prednisolone (PSL) and cyclosporine, the nephrotic syndrome recurred. She was also started on rituximab (RTX). She developed late-onset neutropenia after RTX treatment (R-LON) and improved 17 days later. Although the majority of R-LON cases undergo spontaneous remission, cases of death have been reported. This report is intended to warn about R-LON, since the use of RTX for adult-onset nephrotic syndrome is expected to increase in the future.
  • 机译 接受Nivolumab免疫治疗的非小细胞肺癌患者的急性间质性肾炎
    摘要:Immune-checkpoint-inhibitors (ICPIs) represent a novel class of immunotherapy against several malignancies. These agents are associated with several “immune-mediated” adverse effects, but the reported renal toxicity of ICPIs is less well defined. We present the case of a 60-year-old man with a history of non-small cell lung cancer, who developed acute kidney injury (AKI) approximately 3.5 months after initiation of immunotherapy with nivolumab. Urinalysis revealed sterile pyuria, without microscopic hematuria or proteinuria. Immunological examination was negative. A renal biopsy showed severe interstitial inflammatory infiltration of T-cells, monocytes, and eosinophils without interstitial granulomas and normal appearance of glomeruli, indicating acute interstitial nephritis (AIN) as the cause of AKI. After a short-term course of corticosteroids and permanent nivolumab discontinuation, partial recovery of renal function was noted. AIN is a rare adverse effect of ICPIs that mandates the close monitoring of renal function in patients under immunotherapy with these agents.
  • 机译 类风湿关节炎合并肾性血栓性微血管病,可能是由于慢性他克莫司动脉病和严重高血压的合并症
    摘要:A 51-year-old woman with rheumatoid arthritis presented with mild hypertension 20 months after tacrolimus treatment and developing proteinuria 24 months after the treatment. Tacrolimus was discontinued 27 months after the treatment, followed by heavy proteinuria, accelerated hypertension, and deteriorating renal function without ocular fundus lesions as a clinical sign of malignant hypertension. Renal biopsy revealed malignant nephrosclerosis characterized by subacute and chronic thrombotic microangiopathy (TMA), involving small arteries, arterioles, and glomeruli. Focal segmental glomerulosclerosis, probably secondary to chronic TMA, was identified as a cause of heavy proteinuria. The zonal tubulointerstitial injury caused by subacute TMA may have mainly contributed to deteriorating renal function. The presence of nodular hyalinosis in arteriolar walls was indicative of tacrolimus-associated nephrotoxicity. Together with other antihypertensive drugs, administration of aliskiren stabilized renal function with reducing proteinuria. Owing to the preexisting proteinuria prior to severe hypertension and the complex renal histopathology, we postulated that chronic TMA, which was initially triggered by tacrolimus, was aggravated by severe hypertension, resulting in overt renal TMA.
  • 机译 癫痫发作后的急性尿酸肾病:病例报告和审查
    摘要:Acute hyperuricemia most commonly occurs in patients who experience tumor lysis syndrome. Hyperuricemia along with other electrolyte abnormalities like hyperkalemia, hypocalcemia, and hyperphosphatemia leads to acute kidney injury (AKI) due to acute uric acid nephropathy which is associated with significant morbidity. High risk patients are thus closely monitored for signs of these laboratory abnormalities. Extreme exercise, rhabdomyolysis, and seizures are rare causes of acute hyperuricemia. Serum uric acid level is not routinely monitored as a part of postictal labs. We report an unusual case of AKI in a young male with recurrent seizures and no associated rhabdomyolysis who was found to have acute uric acid nephropathy. Timely administration of Rasburicase prevented the need for dialysis in this patient and led to complete renal recovery. This case illustrates the importance of doing a urine microscopy and checking uric acid level in patients with recurrent seizures who develop unexplainable AKI, as timely management helps improve outcome. We also briefly review the pathophysiology of seizure related hyperuricemia and acute uric acid nephropathy.
  • 机译 尿路梗阻的非典型原因
    摘要:Acute kidney injury due to urinary tract obstruction invariably suggests lower urinary tract obstruction or bilateral ureteric obstruction since obstruction of a single kidney while the contralateral kidney is normal and not obstructed would not cause a perceptible rise in creatinine. Assuming a total body volume of 42 L, 70 kg male that generates approximately 1400 mg of creatinine daily (20 mg/kg/day) who has complete urinary tract obstruction would experience a 3.33 mg/dL per day increase in serum creatinine. Thus, for an individual who had prior normal renal function and who presents with a creatinine of 30 mg/dL, one could surmise that the obstructive pathology had lasted at least 10 days. However, the rise in serum creatinine is a poor marker of renal injury and subsequent prognosis. Urinary tract obstruction leading to AKI can be due to a variety of causes, and its management is tailored to the underlying etiology. This case series describes the varied clinical course of four patients at our center who experienced AKI from atypical causes of obstructive uropathy. Current and future diagnostic modalities and caveats in the treatment of this disease entity are also discussed.
  • 机译 高的Lyso-Gb3血浆水平与年轻的非白蛋白尿男性经典法布里病患者的miR-29和miR-200尿排泄减少相关
    摘要:Renal involvement is associated with a greater morbidity and mortality in Fabry disease. Pathological albuminuria, the first Fabry nephropathy clinical manifestation, can occur from early childhood, although histological lesions such as tubulo-interstitial fibrosis and glomerulosclerosis are present or may precede the onset of pathological albuminuria. In renal cells, exposure to Lyso-Gb3 is correlated with increased expression of Transforming Growth Factor-βeta (TGF-β). miR-21, miR-192, and miR-433 that promote fibrosis are activated by TGF-β, and miR-29 and miR-200 that suppress fibrosis are inhibited by TGF-β. A 23-year-old male was diagnosed with FD. αGalA decreased enzyme activity: 0.1 nmol/hour/liter; genotype: [c.317T>G (p.L106R)]; GFR: 104.4 mL/min/m2; urinary albumin excretion: 6.00 mg/day; plasma Lyso-Gb3: 124.5 nmol/L. A decrease urinary excretion of miR-29 and miR-200 was found (p <0.005) compared to controls. In addition to its usefulness as a phenotype marker, Lyso-Gb3 has been proposed as an indicator of therapeutic response. We detect an association of high Lyso-Gb3 plasma values with decreased urinary excretion of miRNAs with known antifibrotic effect (miR-29 and miR-200). Although the present work is a case report, it could be hypothesized that one of the harmful Lyso-Gb3 effects could be the miRNAs regulation through changes in TGF-β expression.
  • 机译 心包填塞:在cANCA相关的血管炎和肾小球肾炎与非常高的ANA滴度相关的罕见临床表现
    摘要:ANCA (anti-neutrophil cytoplasmic antibody) vasculitides are systemic autoimmune diseases in which anti-neutrophilic cytoplasmic antibodies activate primed neutrophils, thereby generating an inflammatory cascade resulting in the damage of small sized blood vessels in various organs of the body, including the heart. Pleuropericardial involvement is underrecognized as a complication of ANCA vasculitis and is highlighted in this case report of a 51-year-old male who presented with an initial symptomatic presentation of pleuropericardial effusion progressing to pericardial tamponade in the setting of a later renal biopsy proven pauci-immune crescentic glomerulonephritis with high ANA titres along with positive cANCA (cytoplasmic ANCA) and PR3 (proteinase 3) antibodies. He was found to have acute renal failure which progressively got better with cyclophosphamide.
  • 机译 肾移植患者的黑色食管
    摘要:Acute esophageal necrosis (AEN) is an uncommon disease characterized by gastrointestinal bleeding and endoscopic findings of circumferential black-colored necrosis of the distal esophagus. Patients at risk include elderly males over the age of 65, who typically have multiple chronic medical issues including vascular disease and diabetes. Mortality is reported to be 32%. Here, we present a case of AEN in a renal transplant patient and describe potential inciting factors such as immunosuppression and opportunistic diseases.
  • 机译 慢性淋巴细胞性白血病的多面孔:肾,心脏和骨骼并发症的患者。
    摘要:We describe a patient who had chronic lymphocytic leukaemia (CLL) Binet stage A at presentation with further evidence of disease at multiple sites but who initially required no treatment. However, several years later, her peripheral blood lymphocyte count started to increase, and soon after that she suffered an acute myocardial infarct (in the absence of coronary atheroma) together with proteinuric renal failure due to membranoproliferative glomerulonephritis. Her renal function improved markedly following anti-CLL chemotherapy. We postulate that her cardiac and renal disease were both complications of her CLL. In patients with CLL who develop new clinical signs or symptoms (even if apparently unrelated), consideration should be given as to whether these may be disease complications as this may serve as an indication to commence anti-CLL therapy; close liaison between different specialties is vital.
  • 机译 放射引导性肾动脉栓塞术与Amplatzer血管栓塞作为AL淀粉样变性病难治性肾病综合征的抢救治疗
    摘要:Nephrotic syndrome is common in immunoglobulin light-chain (AL) amyloidosis and successful therapy may pose a challenge. We report the case of a 63-year-old patient with severe nephrotic syndrome due to primary renal AL-amyloidosis with well-preserved renal function at first presentation. Therapy with high dose steroids, loop diuretics, and ACE-inhibitors did not affect his proteinuria and he was seriously disabled because of symptomatic orthostatic hypotension and anasarca. With the patient's informed consent, medical nephrectomy was tried with nonsteroidal-anti-inflammatory drugs (NSAIDs), cyclosporine, and aminoglycosides, with significant deterioration of his renal function, but without relevant effect on his proteinuria. Despite adequate anticoagulation life threatening thrombotic and bleeding complications occurred. Total renal ablation was finally achieved using an Amplatzer vascular plug Typ IV (AVP 4) with a self-expanding Nitinol mesh design, which was placed in both main renal arteries in the same intervention. The patient became completely anuric, protein loss stopped, and serum albumin slowly rose to normal levels. The patient's clinical condition dramatically improved and he regained his full mobility at the price of a lifelong renal replacement therapy. To our knowledge, this is the first reported usage of such a vascular occluder in the setting of refractory nephrotic syndrome with normal kidney function at the time of first presentation.
  • 机译 使用20岁埋藏式腹膜透析导管成功开始透析:病例报告和文献复习
    摘要:Buried peritoneal dialysis (PD) catheters are typically inserted several weeks or months before the anticipated need for dialysis. Occasionally, renal function unexpectedly stabilizes after the surgery, and a patient may go years before the catheter is needed. We report a case of successful initiation of PD with a twenty-year-old buried catheter. We outline the steps needed to optimize the catheter function and review the benefits of the buried PD catheter.

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