Hypokalemic rhabdomyolysis: a rare manifestation of primary aldosteronism

Rhabdomyolysis is a rare presentation of hypokalemia, although muscle weakness is a well-known manifestation of hypokalemia. Primary aldosteronism is characterized by hypertension, suppressed plasma renin activity, increased aldosterone excretion and hypokalemia with metabolic alkalosis. Rhabdomyolysis is not common in primary aldosteronism. We present here a 40-year-old woman presenting with rhabdomyolysis accompanied by severe hypokalemia as heralding symptom of primary aldosteronism

Fluctuations of estimated glomerular filtration rate outside kidney disease improving global outcomes diagnostic criteria for acute kidney injury in end-stage liver disease outpatients and outcome postliver transplantation

Background. Renal dysfunction in end-stage liver disease (ESLD) results fromsystemic conditions that affect both liver and kidney with activation of vasoconstrictor systems. In this setting, estimated glomerular filtration rate (eGFR) may undergo variations often outside Kidney Disease Improving Global Outcomes criteria for acute kidney injury (AKI) diagnosis, whose meaning is not clear. The aim of this study was to evaluate eGFR variations in ESLD outpatients listed for liver transplant (liver Tx) and the association with post-Tx outcome. Methods. Fifty-one patients with ESLD were retrospectively evaluated from listing to transplant (L-Tx time), intraoperatively (Tx time), and up to 5 years post-Tx time. Variations between the highest and the lowest eGFR occurring in more than 48 hours, not satisfying Kidney Disease Improving Global Outcomes guideline, were considered as fluctuations (eGFR-F). Fluctuations of eGFR greater than 50%were defined as eGFR drops (DeGFR). Early graft dysfunction, AKI within 7 days, chronic kidney disease, and short- and long-term patient survivals were considered as outcomes. Results. All patients presented eGFR-F, whereas DeGFR were observed in 18 (35.3%) of 51 (DeGFR+ group). These patients presented higher levels of Model for End-stage Liver Disease score, pre-Tx bilirubin and significantly greater incidence of post-Tx AKI stages 2 to 3 compared with patients without drops (DeGFR−). DeGFR was the only independent predictive factor of the occurrence of post-Tx AKI. The occurrence of AKI post-Tx was associated with the development of chronic kidney disease at 3 months and 5 years post-Tx. Conclusions. Drops of eGFR are more frequently observed in patients with a worse degree of ESLD and are associated with a worse post-Tx kidney outcome

Rapidly Progressive Renal and Hepatic Failure in AL-Amyloidosis: Bortezomib and Steroid Support in a Young Woman Two Months After Delivery

INTRODUCTION: The epidemiology of amyloidosis is not well known and its diagnosis is difficult, due to unspecific early clinical manifestations. Amyloidosis is considered when organ failure occurs. Renal, cardiac and hepatic involvement usually occurred despite therapy. Presenting this case report, we aimed to underline the need of new treatments for amyloidosis. MATERIALS AND METHODS: We describe a 41 years old woman admitted to the hospital with proteinuria (1280 mg/24h) and rapid deterioration of renal function (serum creatinine from 0.8 to 1.6 mg/dL). Autoantibodies, immunoglobulin and C3/C4 were negative. A renal biopsy showed the presence of AL-Amyloidosis with k-light chains deposition at immunofluorence. Subsequently, the patient showed nephrotic syndrome onset (proteinuria 4000 mg/24h with albuminuria 3400 mg/24h) and increased rates of cholestasis with hepatomegaly and hepatic failure. RESULTS: Treatment with bortezomib and dexamethasone gave a complete hematological response but renal function was not improved. DISCUSSION: This case is very interesting because renal involvement was the initial presentation of amyloidosis and rapid progressive renal and hepatic failure was subsequentely observed; its management was challenging from the clinical approach to the final diagnosis and treatment. CONCLUSIONS: In terms of organ response, it is necessary to develop new strategies to counteract the progressive organ failure due to amyloid deposition

ANCA-Negative Paucimmune Glomerulonephritis and Glomerular Recovery: Possible Role of Mesenchymal Stem Cells in a 69 Year-Old Patient

BACKGROUND: Pauci-immune crescentic glomerulonephritis is one of the most common causes of rapidly progressive glomerulonephritis, usually associated with anti-neutrophil cytoplasmic antibody (ANCA) but a minority of patients lack ANCAs. To date no effective treatments have been addressed for ANCA-negative glomerulonephritis despite several studies describe the possibility to adopt pharmacologic therapies. Cellular, molecular and preclinical studies demonstrate that mesenchymal stem cells (MSCs) contribute to glomerular cell turnover and repair and might represent a new therapeutic approach for ANCA-negative glomerulonephritis. AIM: In our study we presented a case report of a patient with ANCA-negative pauci-immune glomerulonephritis in order to underlie the growing need of an alternative therapy for glomerular diseases. MATERIALS AND METHODS: We report the case of a 69 year-old woman who presented to our department with diarrhea, vomiting and increased serum creatinine levels. At the First Aid Department, renal ultrasonography excluded dilatations of urinary tract and since the occurrence of progressive dyspnea, anuria and severe hypertension renal replacement therapy was started. RESULTS: A second kidney Doppler ultrasonography showed hyperechoic parenchyma, reduced thickness and increased resistive index. Urinalysis revealed persistent active sediment and proteinuria. Autoimmunity profile showed C3, IgE and IgM increased levels. Renal biopsy was performed showing diffuse extracapillary proliferative glomerulonephritis with negative Immunofluorescence (IF). ANCA test was negative for a second time by both IF analysis and antigen-specific ELISA. The diagnosis was ANCA-negative glomerulonephritis and steroid bolus was administered, improving urinary sediment and proteinuria but not glomerular filtration rate. DISCUSSION: Our data demonstrated that pharmacological treatment of ANCA-negative glomerulonephritis correlated with poor response and is not effective, pointing out the importance of new therapeutic options for this disease. In this regard MCSs may represent a valid alternative in the treatment of ANCA-negative glomerulonephritis. CONCLUSIONS: MSCs may provide an effective therapeutic option in glomerular diseases and future studies should be performed in order to pave the way for this cell-based therapy

Possible Consequences of Non-Adherence to Immunosuppression: a Case of Acute T-Cell Mediated Kidney Rejection and IgA Nephropathy

Patients with primary immunoglobulin A nephropathy (IgAN) usually represent ideal candidates for a renal transplantation. IgA nephropathy represents the most frequent form of recurrent glomerulonephritis post kidney transplant. The therapeutic effects of post transplant immunosuppressive therapy seem to be related to the ability to regulate T-cell immunity and the Th1/Th2 balance. T-cell dysregulation plays an important role in IgAN pathogenesis and recurrence post kidney transplantation. We describe the case of a 52 years old Asian woman with IgAN who received an unrelated living donor kidney transplant. She had independently withdrawn all immunosuppressive maintenance therapy seven years after transplantation followed by acute kidney dysfunction. Acute T-cell mediated rejection was demonstrated in the first kidney biopsy. High steroid pulses were administered with partial response. Recurrence of native IgAN associated with partial resolution of T-cell mediated rejection was observed, as showed in the second kidney biopsy. We hypothesize that recurrence of primary nephropathy could be a manifestation of T-cells activation in non-adherent patients partially responsive to T-cell anti-rejection therapy