Life-Threatening Retroperitoneal Hemorrhage Following Cyst Rupture in Autosomal Dominant Polycystic Kidney Disease (ADPKD): A Case Report.

BACKGROUND Autosomal dominant polycystic kidney disease (ADPKD) is the leading genetic cause of kidney failure worldwide. It is characterized by cyst formation and growth, kidney parenchymal destruction, and complications including cyst infection, nephrolithiasis, cyst rupture, and cyst hemorrhage. Cyst bleeding is typically a self-limited event. This case report describes a 60-year-old man with ADPKD admitted with retroperitoneal hemorrhage following renal cyst rupture requiring embolization of a bleeding left lumbar artery and use of tranexamic acid. CASE REPORT A 60-year-old man with ADPKD presented with altered mental status. Labs noted hemoglobin of 4.7 g/dL. Abdominal imaging revealed polycystic kidneys and large left retroperitoneal hematoma. Angiogram demonstrated active bleeding from left L3 lumbar artery which was embolized. He was admitted to intensive care unit for hemorrhagic shock requiring multiple blood transfusions. Hemoglobin continued to downtrend despite blood products with repeat imaging demonstrating expanding retroperitoneal bleed. He underwent repeat angiogram and though there was no active bleeding, prophylactic embolization of left L1, L3, L4 lumbar and left renal capsular arteries were performed. Hemoglobin stabilized for next 3 days but continued to downtrend subsequently. Oral tranexamic acid was trialed with stabilization of the hemoglobin. CONCLUSIONS Life-threatening retroperitoneal hemorrhage following cyst rupture in the absence of major trauma or use of anti-coagulants, is a rare complication in ADPKD. Treatment involves resuscitation with blood products, management of shock, and interventional radiology-guided embolization. Tranexamic acid may be considered when the above measures fail. Nephrectomy may be indicated for refractory bleeding. This report highlights the diagnosis and management of massive cyst bleeding in ADPKD.

Cardiovascular Outcomes in Kidney Transplant Recipients With ADPKD.

Introduction: Cardiovascular disease leads to high morbidity and mortality in patients with kidney failure. Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a systemic disease with various cardiac abnormalities. Details on the cardiovascular profile of patients with ADPKD who are undergoing kidney transplantation (KT) and its progression are limited. Methods: Echocardiographic data within 2 years before KT (1993-2020), and major adverse cardiovascular events (MACEs) after transplantation were retrieved. The primary outcome is to assess cardiovascular abnormalities on echocardiography at the time of transplantation in ADPKD as compared with patients without ADPKD matched by sex (male, 59.4%) and age at transplantation (57.2 ± 8.8 years). Results: Compared with diabetic nephropathy (DN, n = 271) and nondiabetic, patients without ADPKD (NDNA) (n = 271) at the time of KT, patients with ADPKD (n = 271) had lower rates of left ventricular hypertrophy (LVH) (39.4% vs. 66.4% vs. 48.6%), mitral (2.7% vs. 6.3% vs. 7.45) and tricuspid regurgitations (1.8% vs. 6.6% vs. 7.2%). Patients with ADPKD had less diastolic (25.3%) and systolic (5.6%) dysfunction at time of transplantation. Patients with ADPKD had the most favorable post-transplantation survival (median 18.7 years vs. 12.0 for diabetic nephropathy [DN] and 13.8 years for nondiabetic non-ADPKD [NDNA]; P < 0.01) and the most favorable MACE-free survival rate (hazard ratio = 0.51, P < 0.001). Patients with ADPKD had worsening of their valvular function and an increase in the sinus of Valsalva diameter post-transplantation (38.2 vs. 39.9 mm, P < 0.01). Conclusion: ADPKD transplant recipients have the most favorable cardiac profile pretransplantation with better patient survival and MACE-free survival rates but worsening valvular function and increasing sinus of Valsalva diameter, as compared with patients with other kidney diseases.

Management of autosomal dominant polycystic kidney disease in the era of disease-modifying treatment options.

Autosomal dominant polycystic kidney disease (ADPKD) is the reported etiology in 10% of end-stage kidney disease (ESKD) patients and has an estimated prevalence of 12.5 million cases worldwide across all ethnicities. There have been major advancements over the last two decades in understanding the pathogenesis and development of disease-modifying treatment options for ADPKD, culminating in regulatory approval of tolvaptan for ADPKD patients at risk of rapid progression to kidney failure. This review highlights the genetic mutations associated with ADPKD, defines patients at risk of rapid progression to ESKD, and focuses on the management of ADPKD in the era of disease-modifying agents.

Impact of serum magnesium levels at hospital discharge and one-year mortality.

BACKGROUND: We aimed to determine the optimal range of discharge serum magnesium in hospitalized patients by evaluating one-year mortality risk according to discharge serum magnesium. METHODS: This was a single-center cohort study of hospitalized adult patients who survived until hospital discharge. We classified discharge serum magnesium, defined as the last serum magnesium within 48 hours of hospital discharge, into ≤1.6, 1.7-1.8, 1.9-2.0, 2.1-2.2, and ≥2.3 mg/dL. We assessed one-year mortality risk after hospital discharge based on discharge serum magnesium, using discharge magnesium of 2.1-2.2 mg/dL as the reference group. RESULTS: Of 39,193 eligible patients, 8%, 23%, 34%, 23%, and 12% had a serum magnesium of ≤1.6, 1.7-1.8, 1.9-2.0, 2.1-2.2, and ≥2.3 mg/dL, respectively, at hospital discharge. After the adjustment for several confounders, discharge serum magnesium of ≤1.6, 1.7-1.8, and ≥2.3 mg/dL were associated with higher one-year mortality with hazard ratio of 1.35 (95% CI 1.21-1.50), 1.14 (95% CI 1.06-1.24), and 1.17 (95% CI 1.07-1.28), respectively, compared to discharge serum magnesium of 2.1-2.2 mg/dL. There was no significant difference in one-year mortality between patients with discharge serum magnesium of 1.9-2.0 and 2.1-2.2 mg/dL. CONCLUSION: The optimal range of serum magnesium at discharge was 1.9-2.2 mg/dL. Both hypomagnesemia and hypermagnesemia at discharge were associated with higher one-year mortality.

Alkalemia and Hepatic Encephalopathy in a Chronic Dialysis Patient.

Hepatic encephalopathy (HE) includes cognitive, psychiatric and neuromotor abnormalities observed from brain dysfunction secondary to liver disease and/or porto-systemic shunting. HE can have a wide range of clinical manifestations ranging from trivial lack of awareness, decreased attention span, personality changes to confusion, seizures, coma, and death. The onset of HE in cirrhosis is a poor prognostic factor. While HE has a complex pathogenesis which is not completely understood, hyperammonemia plays an important role in neurotoxicity and brain dysfunction. Alkalemia facilitates the conversion of NH4+ to NH3, which is free to cross the blood-brain barrier exacerbating HE. Prompt recognition and correction of underlying risk factors is central to the management of HE.

Malignancy-induced lactic acidosis in adult lymphoma.

Malignancy-induced lactic acidosis (MILA), a rare paraneoplastic phenomenon, is mostly described with hematologic malignancies (lymphomas and leukemias) but has also been reported with solid tumors. It is a subset of type B lactic acidosis being mediated without evidence of tissue hypoperfusion. Lymphoma-induced lactic acidosis is often considered an oncologic emergency and is associated with an increased risk of mortality and poor prognosis. It has a complex pathophysiology centered in the "Warburg effect," i.e., the programming of cancer cells to depend on aerobic glycolysis for promotion of their proliferation and anabolic growth. The treatment of lymphoma-induced lactic acidosis is focused on prompt administration of chemotherapy. The role of alkali therapy in this setting is controversial and has limited proven benefit with a potential for worsening the lactic acidosis. If alkali therapy is used in the presence of severe acidemia to optimize cardiovascular status, it should be administered judiciously.

Cost analysis of two modalities of continuous renal replacement therapy.

BACKGROUND: Continuous veno-venous hemofiltration (CVVH) and continuous veno-venous hemodialysis (CVVHD) are costly therapies reserved for use in critically ill patients with kidney failure. DESIGN: Quality improvement study at St. Elizabeth's Medical Center, Boston, MA, USA. SETTING AND PARTICIPANTS: Members of nephrology and pharmacy department, working alongside intensive care unit nursing staff and hospital administration, undertook an initiative to transition from CVVH to CVVHD, to simplify therapy administration, lessen need for electrolyte repletion, and reduce total treatment-related costs. QUALITY IMPROVEMENT PLAN: We postulated that conversion from CVVH to CVVHD would result in fewer filter clotting events, longer filter use (up to 72 hours), lower resource utilization, and confer overall cost benefit. Over 12 months, patients initiated on CVVH were identified. Following conversion to CVVHD, patients initiated on CVVHD over 9 months were identified. Patient characteristics, comorbidities, and hospital-related outcomes, as well as CRRT-related information including treatment modality, treatment duration, and treatment-related costs were obtained. MEASURES: Daily treatment-related costs, intensive care unit and hospital length of stay (LOS), and in-hospital mortality. RESULTS: During the baseline period, 77 patients were initiated on CVVH, and during the intervention period, 60 patients were initiated on CVVHD. Following conversion from CVVH to CVVHD, mean (±SD) daily total treatment cost decreased from $1813 ± $2143 to $775 ± $766 (P < 0.001). Conversion from CVVH to CVVHD had no impact on intensive care unit LOS (11.8 ± 9.7 vs. 12.4 ± 9.5 days; P = 0.53), hospital LOS (15.0 ± 11.1 vs. 16.4 ± 14.0 days; P = 0.89), or in-hospital mortality (58% vs. 60%; P = 0.85). LIMITATIONS: Nursing costs, costs of dialysis machine utilization, phosphate repletion, and systemic anti-coagulation were not studied. CONCLUSION: Transition from CVVH to CVVHD resulted in a significant cost benefit and reduced resource utilization. There was no difference in LOS and in-hospital mortality between the two treatment modalities.

Streptococcus pluranimalium infective endocarditis and brain abscess.

Infective endocarditis is an infection of the endocardium of the heart typically seen in individuals with underlying risk factors. We report a rare case of Streptococcus pluranimalium bacteremia causing infective endocarditis and brain abscess. Only 3 cases were reported in the literature on infective endocarditis in patients secondary to S. pluranimalium.