Paraneoplastic Cast Nephropathy Associated With Pancreatic Mixed Acinar-Neuroendocrine Carcinoma: A Case Report.

Acute kidney injury is common in patients with cancer and may result from sepsis, obstruction, radiotherapy, chemotherapeutic agents, and nonsteroidal anti-inflammatory drugs. Rare reports of acute kidney injury due to cast nephropathy in patients with pancreatic acinar cell carcinoma have been described, but a pathogenetic link between cast nephropathy and carcinoma was not established. We report a patient with pancreatic mixed acinar-neuroendocrine carcinoma who developed severe acute kidney injury. Kidney biopsy showed cast nephropathy characterized by fractured periodic acid-Schiff-negative casts, associated with mononuclear and giant cell reaction. The patient did not have multiple myeloma and casts did not show immunoglobulin light chain restriction on immunofluorescence. Analysis using liquid chromatography-tandem mass spectrometry and immunohistochemistry identified 2 acinar cell-specific proteins, regenerating islet-derived 1α and carboxypeptidase A1, in both tubular casts and tumor cells. Thus, this case demonstrates that solid tumor-specific proteins can be nephropathic by obstructing renal tubules, resulting in acute kidney injury, a previously proposed but not characterized pathophysiologic mechanism for paraneoplastic nephropathy associated with carcinoma.

Renal failure requiring dialysis complicating slow continuous ultrafiltration in acute heart failure: importance of systolic perfusion pressure.

BACKGROUND: Recent reports have raised concerns regarding renal outcomes in patients with decompensated acute heart failure (HF) treated with slow continuous ultrafiltration (SCUF). The purpose of this study was to identify risk factors for renal failure (RF) requiring dialysis in patients with acute HF initiated on SCUF. METHODS AND RESULTS: We studied 63 consecutive patients with acute HF who required SCUF because of congestion refractory to hemodynamically guided intensive medical therapy. Median serum creatinine at SCUF initiation was higher in patients who developed RF requiring dialysis [2.5 (interquartile range 1.8-3.3) vs 1.6 (1.2-2.3) mg/dL; P < .001]. Weight loss within 48 hours of SCUF initiation was larger in patients who did not progress to RF [-6 (-10 to -2) vs -4 (-6 to -2) kg; P = .03]. Systolic perfusion pressure had a nonlinear association with RF requiring dialysis, with a threshold effect noted at 90 mm Hg. Twelve-month mortality in patients who were moved to dialysis versus those who were not was 95% versus 35%, respectively (P < .001). CONCLUSIONS: In patients with acute HF initiated on SCUF, onset of RF requiring dialysis is associated with high mortality. Systolic perfusion pressure which incorporates both perfusion and venous congestion parameters may present a modifiable risk factor for worsening RF during SCUF in acute HF patients.

Presence and outcomes of kidney disease in patients with pulmonary hypertension.

BACKGROUND AND OBJECTIVES: Pulmonary hypertension is associated with higher mortality rates. The associations of nondialysis-dependent CKD and all-cause mortality in patients with pulmonary hypertension were studied. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: The study population included those patients who underwent right heart catheterization for confirmation of pulmonary hypertension between 1996 and January 2011. Pulmonary hypertension was defined as the presence of mean pulmonary artery pressure ≥ 25 mmHg at rest measured by right heart catheterization. CKD was defined as the presence of two measurements of eGFR<60 ml/min per 1.73 m(2) 90 days apart. The risk factors associated with CKD as well as the association between CKD and death in those patients with pulmonary hypertension using logistic regression and Cox proportional hazard models were examined. RESULTS: Of 1088 patients with pulmonary hypertension, 388 (36%) patients had CKD: 340 patients had stage 3 CKD, and 48 (4%) patients had stage 4 CKD. In the multivariable analysis, older age, higher hemoglobin, and higher mean right atrial pressures were independently associated with CKD. During a median follow-up of 3.2 years (interquartile range=1.5-5.6 years), 559 patients died. After adjusting for relevant covariates, presence of stage 3 CKD (hazard ratio, 1.37; 95% confidence interval, 1.14 to 1.66) and stage 4 CKD (hazard ratio, 2.69; 95% confidence interval, 1.88 to 3.86) was associated with all-cause mortality in those patients with pulmonary hypertension. When eGFR was examined as a continuous measure, a 5 ml/min per 1.73 m(2) lower eGFR was associated with a 5% (95% confidence interval, 1.03 to 1.07) higher hazard for death. This higher risk with CKD was similar irrespective of demographics, left ventricular function, and pulmonary capillary wedge pressure. CONCLUSION: In a clinical population referred for right heart catheterization, presence of CKD was associated with higher all-cause mortality in those patients with pulmonary hypertension. Mechanisms that may underlie these associations warrant additional studies.

Recovery from AKI and short- and long-term outcomes after lung transplantation.

BACKGROUND AND OBJECTIVES: Patients with AKI after lung transplantation are at increased risk for CKD and death. Whether patients who completely recover from AKI have improved long-term outcome compared with patients who do not completely recover remains unknown. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This study retrospectively evaluated data on 657 patients who underwent lung transplantation from 1997 to 2009. Outcomes analyzed were the incidence of renal recovery after AKI and the association of this recovery with short- and long-term mortality. AKI was defined by an absolute increase in serum creatinine of ≥0.3 mg/dl or a percent increase in serum creatinine of ≥50% from baseline at any time during the first 2 weeks after transplantation. RESULTS: Four hundred twenty-four (65%) patients experienced AKI in the first 2 weeks after transplantation. Of these patients, complete renal recovery occurred in 142 (33%) patients. The incidence of in-hospital complications was similar between patients who recovered renal function and patients without recovery. At 1 year, the cumulative incidence of CKD was 14% and 22% (P=0.10) and patient survival rate was 81% and 76% (P=0.20) in patients with complete recovery from AKI and patients without recovery, respectively. Patients with completely recovered AKI had similar risk-adjusted long-term mortality compared with patients who did not recover (hazard ratio [95% confidence interval]=1.42 [1.15-2.05] versus 1.53 [1.01-2.00]). CONCLUSIONS: Patients who recover completely from early AKI after lung transplantation have a similar risk for CKD and long-term mortality compared with patients who do not recover.

Cardiorenal outcomes after slow continuous ultrafiltration therapy in refractory patients with advanced decompensated heart failure.

OBJECTIVES: The purpose of this study was to examine the clinical outcomes of using slow continuous ultrafiltration (SCUF) in patients with acute decompensated heart failure (HF) refractory to intensive medical therapy. BACKGROUND: Several studies have demonstrated the clinical usefulness of early SCUF in patients with acute decompensated HF to improve fluid overload and hemodynamics. METHODS: We reviewed clinical data from 63 consecutive adult patients with acute decompensated HF admitted to the Heart Failure Intensive Care Unit from 2004 through 2009 who required SCUF because of congestion refractory to hemodynamically guided intensive medical therapy. RESULTS: The mean creatinine level was 1.9 ± 0.8 mg/dl on admission and 2.2 ± 0.9 mg/dl at SCUF initiation. After 48 hours of SCUF, there were significant improvements in hemodynamic variables (mean pulmonary arterial pressure: 40 ± 12 mm Hg vs. 33 ± 8 mm Hg, p = 0.002, central venous pressure: 20 ± 6 mm Hg vs. 16 ± 8 mm Hg, p = 0.007, mean pulmonary wedge pressure: 27 ± 8 mm Hg vs. 20 ± 7 mm Hg, p = 0.02, Fick cardiac index: 2.2 l/min/m(2) [interquartile range: 1.87 to 2.77 l/min/m(2)] vs. 2.6 l/min/m(2) [interquartile range: 2.2 to 2.9 l/min/m(2)], p = 0.0008), and weight loss (102 ± 25 kg vs. 99 ± 23 kg, p < 0.0001). However, there were no significant improvements in serum creatinine levels (2.2 ± 0.9 mg/dl vs. 2.4 ± 1 mg/dl, p = 0.12) and blood urea nitrogen (60 ± 30 mg/dl vs. 60 ± 28 mg/dl, p = 0.97). Fifty-nine percent required conversion to continuous hemodialysis during their hospital course, and 14% were dependent on dialysis at hospital discharge. Thirty percent died during hospitalization, and 6 patients were discharged to hospice care. CONCLUSIONS: In our single-center experience, SCUF after admission for acute decompensated HF refractory to standard medical therapy was associated with high incidence of subsequent transition to renal replacement therapy and high in-hospital mortality, despite significant improvement in hemodynamics.

Short-term and long-term outcomes of acute kidney injury after lung transplantation.

BACKGROUND: The effect of acute kidney injury (AKI) after lung transplantation has been described infrequently and with inconsistent results. Using a newly adopted and validated definition of AKI proposed by the Acute Kidney Injury Network (AKIN), we examined the incidence of AKI and associated renal morbidity and short-term and long-term mortality. METHODS: We retrospectively evaluated data of 657 patients who underwent lung transplantation from 1997 to 2009. Outcomes analyzed were the incidence of AKI, as defined and categorized into 3 stages according to creatinine criteria from the AKIN classification (AKIN 1, AKIN 2, and AKIN 3), cumulative incidence of chronic kidney disease (CKD), as defined by an estimated glomerular filtration rate ≤ 29 ml/min/1.73 m(2), and/or the onset of end-stage renal disease, as defined by the need for renal replacement therapy for 8 weeks with no recovery on follow-up or need for kidney transplant, and long-term mortality. RESULTS: We identified 424 patients (65%) who had at least 1 AKI (309 [47%] AKIN 1 and 115 [17%] AKIN 2-3) event in the first 2 weeks after transplantation. At 1 year, the cumulative incidence of CKD was 5.8%, 12.8%, 24.5 % in the no-AKI, AKIN 1, and AKIN 2-3 patients, respectively. After a median follow-up of 2.2 years, 277 (42%) died. One-year patient survival was 91%, 82%, 66% in the no-AKI, AKIN 1, and AKIN 2-3 patients, respectively. Adjusting for age, sex, race, type and cause of lung transplant, diabetes, and hypertension, the hazard ratio for death was 1.7 (95% confidence interval, 1.2-2.2; p = 0.0002) for AKIN 1 and 2.9 (95% confidence interval, 1.7-3.7; p < 0.001) for AKIN 2-3. CONCLUSIONS: AKI is a common complication after lung transplantation and is associated with increased risk of CKD and all cause-mortality on long-term follow-up.

Serum bicarbonate and mortality in stage 3 and stage 4 chronic kidney disease.

BACKGROUND AND OBJECTIVES: The incidence and prevalence of metabolic acidosis increase with declining kidney function. We studied the associations of both low and high serum bicarbonate levels with all-cause mortality among stage 3 and 4 chronic kidney disease (CKD) patients. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We examined factors associated with low (<23 mmol/L) and high (>32 mmol/L) serum bicarbonate levels using logistic regression models and associations between bicarbonate and all-cause mortality using Cox-proportional hazard models, Kaplan-Meier survival curves, and time-dependent analysis. RESULTS: Out of 41,749 patients, 13.9% (n = 5796) had low and 1.6% (n = 652) had high serum bicarbonate levels. After adjusting for relevant covariates, there was a significant association between low serum bicarbonate and all-cause mortality (hazard ratio [HR] 1.23, 95% CI 1.16, 1.31). This association was not statistically significant among patients with stage 4 CKD and diabetes. The time-dependent analysis demonstrated a significant mortality risk associated with a decline from normal to low bicarbonate level (HR 1.59, 95% CI 1.49, 1.69). High serum bicarbonate levels were associated with death irrespective of the level of kidney function (HR 1.74, 95% CI 1.52, 2.00). When serum bicarbonate was examined as a continuous variable, a J-shaped relationship was noted between serum bicarbonate and mortality. CONCLUSIONS: Low serum bicarbonate levels are associated with increased mortality among stage 3 CKD patients and patients without diabetes. High serum bicarbonate levels are associated with mortality in both stage 3 and stage 4 CKD patients.

IgA-dominant Staphylococcus infection-associated glomerulonephritis: case reports and review of the literature.

Background and objectives. The mesangial deposition of IgA is rarely described with proliferative glomerulonephritis associated with Staphylococcus infection. Recently, this association has been increasingly recognized possibly due to the increased rate of Staphylococcus infection. Design setting, participants and measurements. We report two cases of methicillin-sensitive Staphylococcus aureus bacteremia associated with acute proliferative glomerulonephritis with dominant mesangial deposit of IgA. We searched MEDLINE (1960-2009) for similar reports. We pooled individual patient data and reported descriptive statistics of all published cases. Results. Forty-six cases were included in the final analysis. The mean age of presentation was 59, with a male predominance (84%). Clinical presentation was notable for rapidly progressive glomerulonephritis with nephrotic-range proteinuria and normal complement levels in 52 and 72%, respectively. Methicillin-resistant S. aureus (68%) was the most common pathogen isolated with a latent period ranging from 1 to 16 weeks. Diffuse mesangial proliferation was commonly found with crescentic lesions noted in 35% of the cases. Antimicrobial treatment was associated with renal recovery in 58% of the cases. Need for renal replacement therapy was significantly associated with pre-existing diabetes, hypertension and interstitial fibrosis seen on kidney biopsy. Conclusions. IgA-dominant post-Staphylococcus glomerulonephritis is a rare clinical entity with certain unique clinical and morphologic features. It is difficult to differentiate from primary IgA nephropathy in cases where the infection is not apparent. An acute onset of rapidly progressive glomerulonephritis, with normal complement levels and deposition of mesangial IgA in an elderly patient should raise suspicion for this rare form of glomerulonephritis.

Reversible hepatic decerebration: a case report and review of the literature.

Hepatic encephalopathy is a syndrome of neuropsychiatric dysfunction caused by portosystemic venous shunting with or without the presence of intrinsic liver disease. Clinical presentations are variable ranging from an abnormal sleep pattern to somnolence and deep coma. Decerebrate and decorticate posturing, have been rarely reported with hepatic encephalopathy. We report a case of a 59-year-old-man with a history of Child-Pugh B liver disease secondary to chronic alcoholism who was admitted because of coma. He had a transjugular intrahepatic portosystemic shunt 3 months prior to his presentation. He was found to have decerebrating posture. He was treated for hepatic encephalopathy with complete recovery and resolution of the neurologic findings. The physician should be aware that decerebration and decortication posture can occur with hepatic encephalopathy and can be reversible.

Spontaneous splenic rupture precipitated by cough: a case report and a review of the literature.

Spontaneous rupture of a normal spleen without a history of trauma is a rare clinical entity. We report on a case of atraumatic splenic rupture in a 61-year-old man who presented to the emergency department for abdominal pain and hypotension. There was no evidence of hematologic or infectious disease involving the spleen. The chronic cough described by the patient was the main trigger for the rupture. Although, spontaneous splenic rupture is rare, it is vital that physicians consider this diagnosis when evaluating patients with abdominal pain and hypotension. Failure to consider splenic rupture could be catastrophic and early diagnosis is essential for a better outcome.