[Bilirubin removal with Coupled Plasma Filtration and Adsorption in patients affected by hilar cholangiocarcinoma]. / Rimozione della bilirubina con Coupled Plasma Filtration and Adsorption in pazienti con colangiocarcinoma ilare.

BACKGROUND: Patients affected by hilar cholangiocarcinoma are eligible for surgery only in the 20-30% of the cases and postoperative mortality is 40-50%. Many specialists are involved in the treatment of this disease, like surgeons, gastroenterologists, oncologists and radiotherapists. Recent studies have shown that preoperative bilirubinaemia is a predictor of morbidity and mortality after surgery. Coupled Plasma Filtration and Adsorption (CPFA) is a blood purification extracorporeal therapy recommended for sepsis and able to reduce bilirubinaemia. METHODS: We treated 10 patients referred to our centre affected by hilar cholangiocarcinoma complicated by obstructive jaundice with 34 CPFA sessions to test its ability to reduce preoperative bilirubin levels and we checked for mortality at 90 days. RESULTS: CPFA reduced preoperative bilirubin of 30% for session; it also improved others inflammation and coagulation tests. Mortality at 90 days was 40%. CONCLUSIONS: CPFA is an effective therapy for hyperbilirubinaemia. Lowering preoperative bilirubinaemia and improvement of coagulation tests subsidized the management of the patients but in our study did not affect postoperative mortality. Further studies to evaluate the indications for treatments that remove bilirubin in this setting are needed.

[Gross Hematuria after kidney biopsy. A case report]. / Ematuria macroscopica dopo una biopsia renale. Caso clinico.

We describe the clinical case of a patient experiencing severe gross hematuria causing clotting in the renal pelvis, after undergoing a kidney biopsy.The ecocolordopper and CT angiography performed did not reveal the cause of hematuria.The kidney arteriography allowed the diagnosis, revealing an arteriovenous fistula responsible for bleeding together with a small false aneurysm in the lower pole of the biopsied kidney. Both lesions were successfully treated with superselective embolization with microcoils. We discuss about the diagnostic and therapeutic approach of these rare post-biopsy complications briefly focusing on the technical aspects and on possible risks that the transcatheter embolotherapy may result.

[The Ormond's disease: an intriguing obstructive nephrouropathy]. / La malattia di Ormond: una intrigante nefrouropatia ostruttiva.

Idiopathic retroperitoneal fibrosis also known as Ormonds disease is a rare disorder characterized by the development of fibrotic tissue in the retroperitoneum involving the abdominal aorta and iliac arteries, ureters and the inferior vena cava. The aberrant tissue may compress ureters leading to obstructive nephrouropathy and renal failure, which are the most common clinical manifestations of this condition. The nephrologist is often consulted to make differential diagnosis for acute renal failure and obstructive uropathy. Ultrasounds may suggest the disease and the diagnosis will be confirmed by computed tomography or magnetic resonance, but biopsy is still the diagnostic gold standard. The aim of therapy is to remove the ureteral obstruction and prevent the progression and recurrence of the disease. After urine drainage by ureteral stents, medical long-term therapy is usually started whereas the open surgery is reserved as a last resort in selected patients. The pathophysiology of Ormond's disease is uncertain. For years the disease was considered reactive to local and /or systemic triggers with primarily involvement of abdominal aorta but at present is classified in the more broad spectrum of IgG4- Related- Disease, clinical pathological entity on autoimmune basis that can affect almost all of the body districts. This last concept has shed light on the understanding of the pathogenesis and opened new therapeutic perspectives with the use of biological agents. In this paper, on the basis of our paradigmatic clinical case of bilateral obstructive nephrouropathy associated with acute renal failure and examining the recent literature, we describe the clinical and therapeutic approach to Ormonds disease.

[Management of antirheumatic drugs in kidney failure]. / Gestione dei farmaci antireumatici nell'insufficienza renale.

The nephrologist deals with the management of patients with rheumatic disease, both diagnostically and therapeutically. He must determine whether the renal pathology is related to the rheumatologic disease, mostly through the use of the renal biopsy. In the second case, he must know the nephrotoxic potential of the drugs prescribed and adjust their use to the degree of renal impairment. This task is made difficult by the absence of controlled clinical trials regarding their use on patients with renal insufficiency or on chronic dialysis. For this reason, the prescription will have to take into account the pharmacokinetics of the drugs. Kidney failure can affect the metabolism of antirheumatic drugs determining their accumulation, which can lead to increased toxicity, either renal or systemic. On the other hand, dialysis can cause excessive drug removal, leading to sub-therapeutic pharmacological effects and to the need for additional doses. In this brief review, we will consider the nephrotoxic effects of some important drugs used in rheumatology and examined individually, with specific reference to rheumatoid arthritis: methotrexate, leflunamide, hydroxychloroquine, cyclosporine, biological DMARDs. In the past, therapeutic success in rheumatic diseases associated with kidney impairment was severely limited by the well- known nephrotoxicity of drugs such as gold salts, D-penicillamine, NSAIDs, COX-2 inhibitors. Although generally effective, they are contraindicated in case of kidney failure. Biologic therapies have recently opened new therapeutic perspectives. Nevertheless, it is worth stressing how our knowledge of their action is still incomplete and this may result in exposure to immune-mediated renal disease.

[Role of the ultrasounds in the kidney biopsy: a case of postbiopsy hematoma linked to renal arteriovenous fistula]. / Ruolo degli ultrasuoni nella biopsia renale: un caso di ematoma post-bioptico associato a fistola artero-venosa renale.

In the planning of a kidney biopsy procedure, ultrasound examination has a crucial role before percutaneous renal biopsy (PRB) in detecting renal abnormalities that could contraindicate the biopsy; during PRB as a method to locate the kidney; and after PRB to diagnose and monitor postbiopsy complications. The case of a 40-year-old woman who underwent ultrasound-guided PRB for urinary abnormalities is described. Careful renal assessment by ultrasonography before the kidney biopsy was not performed. The post-PRB ultrasound examination revealed a perinephric hematoma along with an arteriovenous fistula (AVF) at the lower pole of the biopsied kidney. Surprisingly, a later renal angiography showed AVFs in both kidneys and therefore a diagnosis of non-iatrogenic, idiopathic AVFs was made. Based on our unusual case report, we discuss the diagnostic approach and therapeutic strategies for renal AVFs and we emphasize the usefulness of ultrasound for initial pre-PRB evaluation. In addition, with respect to the post-biopsy hematoma, the advantages offered by ultrasound during and after the implementation of PRB are debated. Finally, the role of post-biopsy hematoma as a possible indicator of post-PRB complications is reported.

[Diagnostic pathway of an unusual case of nephrotic syndrome: immunotactoid glomerulopathy]. / Una insolita sindrome nefrosica (Immunotactoid Glomerulopathy): percorso diagnostico.

Immunotactoid glomerulopathy is a clinicopathological entity characterized by extracellular deposition of microtubular substructures, which are negative for the usual staining that identifies amyloid within the mesangium and capillary walls of renal glomeruli. Despite ongoing debate in the nephrological community, it is kept distinct from fibrillary glomerulonephritis on the basis of the size and arrangement of the microtubules and microfibrils. It is clinically characterized by the presence of glomerular proteinuria in the nephrotic range, microscopic hematuria and hypertension, and is often associated with hypocomplementemia, monoclonal gammopathy, and lymphoprolipherative disorders. A 47-year-old woman was referred to our unit for evaluation of proteinuria associated with nephrotic syndrome. Laboratory findings revealed a serum M component and hypocomplementemia. Renal biopsy yielded three fragments for optical microscopy, immunofluorescence, and electron microscopy; Congo red staining was used. Renal histology showed a morphological pattern of membranoproliferative glomerulonephritis. Immunofluorescence showed IgG deposits with monoclonal kappa light chain restriction as well as C3 and C1q deposits. Electron microscopy revealed the presence within the mesangium of microtubules measuring >35 nm that were focally parallel oriented. The final diagnosis was nephrotic syndrome caused by immunotactoid glomerulopathy. The clinical diagnosis of immunotactoid glomerulopathy is based on pathological, clinical and hematological features and requires the exclusion of other diseases that are associated with organized glomerular deposits. We discuss the diagnostic options offered by the clinical and morphological elements of this case; the use of electron microscopy is emphasized, especially when a renal syndrome is associated with paraproteinemia.

Effects of efficiency and length of acetate-free biofiltration session on postdialysis solute rebound.

BACKGROUND: Postdialytic rebound (PDR) of plasma solutes is a relevant drawback of intermittent hemodialysis, but its pathophysiological process remains undefined. We assessed the independent effects of efficiency and length of dialytic session on PDR of urea, phosphate, and potassium. METHODS: Uremic patients were evaluated at the beginning and end of dialysis and after 180 minutes in 2 randomized crossover studies. In study 1, we compared the effect of standard versus higher efficiency acetate-free biofiltration (AFB) while maintaining the same duration of 4 hours. In study 2, we compared the effect of 3- versus 5-hour AFB sessions while maintaining similar efficiency. RESULTS: In study 1, greater Kt/V (1.49 +/- 0.20 versus 1.22 +/- 0.15; P < 0.0001) was coupled with significant increases in both absolute removal and PDR of urea and phosphate (PDR of urea, +45% versus +29%; PDR of phosphate, +79% versus +52%), but not of potassium. Similarly, in study 2, shortening the AFB session while maintaining similar absolute removal and Kt/V (1.28 +/- 0.09 versus 1.31 +/- 0.09) significantly increased PDR of urea and phosphate (PDR of urea, +32% versus +19%; PDR of phosphate, +63% versus +36%), but not of potassium. In both studies, greater PDRs of urea and phosphate were associated with estimated greater removal of these solutes per hour. CONCLUSION: The rate of removal of phosphate and urea is a critical determinant of their PDR; conversely, potassium is not influenced by removal rate, likely because of its marked cell compartmentalization.