[When a rear-end collision turns out to be a revelation: a case of IgG4 related kidney desease].

IgG4 related renal disease represents a frequent manifestation of the wider IgG4 related disease, a fibroinflammatory disorder with a not fully understood etiology that affects several organs. Through the clinical case presented, we will focus attention on this pathology and on the diagnostic difficulties that may arise, and on the investigations necessary for the diagnosis. Finally, the main therapeutic options will be discussed.

Increase of continuous treatments and regional citrate anticoagulation during renal replacement therapy in the ICUs of the North-West of Italy from 2007 to 2015.

BACKGROUND: Few reports have addressed the change in renal replacement therapy (RRT) management in the Intensive care Units (ICUs) over the years in western countries. This study aims to assess the trend of dialytic practice in a 4.5-million population-based study of the northwest of Italy. METHODS: A nine-year survey covering all the RRT provided in the ICUs. Consultant nephrologists of the 26 Nephrology and Dialysis centers reported their activities in the years 2007, 2009, 2012, and 2015. RESULTS: From 2007 to 2015 the patients treated increased from 1042 to 1139, and the incidence of RRT from 254 to 263 cases/10^6 inhabitants. The workload for dialysis center was higher in the larger hub hospitals. RRT for acute kidney injury (AKI), continuation of treatment in chronically dialyzed patients, or extrarenal indications accounted for about the stable rate of 70, 25 and 5% of all RRT sessions, respectively. Continuous modality days increased from 2731 days (39.5%) in 2007 to 5076 (70.6%) in 2015, when the continuous+prolonged treatment days were 6880/7196 (95.6% of total days). As to RRT timing, in 2015 only the classical clinical criteria, and no K-DIGO stage were adopted by most Centers. As to RRT interruption, in 2015 urine volume was the first criterion. Implementation of citrate anticoagulation (RCA) for RRT patients significantly increased from 2.8% in 2007 to 30.9% in 2015, when it was applied in all 26 Centers. CONCLUSIONS: From 2007 to 2015, current practice has changed towards shared protocols, with increasing continuous modality and RCA implementation.

Report on an Italian survey of 72 stuck hemodialysis catheters.

BACKGROUND: Reports of stuck hemodialysis catheters have been on the rise in recent years. Aim of this work is to report how this complication has been managed and the relative outcomes in a multicente Italian survey. METHODS: Since 2012, the Italian Society of Nephrology (SIN) Project Group of Vascular Access has collected data among nephrologists on this complication. Data regarding 72 cases of stuck tunnelled central venous catheter (tCVC) in 72 patients were retrieved thanks to this survey. RESULTS: In 11 patients (15%) the stuck catheter was directly buried or left in place. Sixty-one cases were managed through advanced removal techniques. Among these, 47 (77%) stuck tunnelled central venous catheters were successfully removed, while 14 (23%) failed to be withdrawn. Considering removed tCVCs, the use of endoluminal balloon dilatation alone or in combination with other tools showed a percentage of success of 88%. The removal procedure involved numerous specialists. Some complications occurred, such as breakage of the line or bleeding, and two cases of haemopericardium during an advanced procedure. CONCLUSION: The survey shows how the stuck catheter complication was managed in different ways, with conflicting results. When utilized, endoluminal balloon dilatation proved to be the most effective and most often utilized technique, while some cases had suboptimal management or failure. This underlines how delicate the procedure is, and the need for both precise knowledge of this complication and timely organization of removal attempts.

[Rapidly progressive Alport syndrome in a young woman: case report].

Alport syndrome is a hereditary clinical condition characterized by multisystemic changes (sensorineural and ocular deafness) associated with hematuria and proteinuria. Due to its genetic variability and multiple symptoms, it is often diagnosed by chance and too late. The present work focuses on this pathology through a clinical case report. It also mentions the new therapeutic possibilities relating to this disease.

Ultrasound in central venous access for hemodialysis.

Central venous cannulation is a frequent need for urgent or scheduled hemodialysis. Many studies confirmed the pivotal role of ultrasound in this procedure. Vascular access guidelines strongly recommend ultrasound guidance. Its usefulness has been demonstrated not only in reducing complications of venipuncture but more recently for the evaluation of central venous catheter (CVC) placement and real-time check for possible complications. The use of ultrasound requires a precise technique for the correct handling of the probe and the needle movement. Different approaches as the out-of-plane, in-plane, and oblique methods are available, enabling the most appropriate for the individual case. Although the learning curve for CVC ultrasound placement is usually fast, formal training and certification for beginners are strongly recommended.

Ultrasound evaluation of access complications: Thrombosis, aneurysms, pseudoaneurysms and infections.

Arteriovenous fistula (AVF) complications are classified based on fistula outcomes. This review aims to update colour Doppler (CD) and pulse wave Doppler (PWD) roles in managing early and late complications of the native and prosthetic AVF. Vascular access (VA) failure occurs because inflow or outflow stenosis activates Wirchow's triad inducing thrombosis. Therefore, the diagnosis of the tributary artery and outgoing vein stenosis will be the first topic considered. Post-implantation complications occur from the inability to achieve AVF maturation and dialysis suitability due to inflow/outflow stenosis. Late stenosis is usually a sequence of early defects repaired to maintain patency. Less frequently, in the mature AVF or graft, complications are acquired 'de novo'. They derive either from incorrect management of vascular access (haematoma, pseudoaneurysm, prosthesis infection) or wall pathologies (aneurysm, myxoid valve degeneration, kinking, coiling, abnormal dilation from defects of elastic structures). High-resolution transducers (10-20 MHz) allow the characterization of the wall damage, haemodynamic dysfunctions, early and late complications even if phlebography remains the gold standard for the diagnosis for its sensitivity and specificity.

[Multiple myeloma, plasma cell dyscrasias and kidneys: a few symptoms, extensive damage].

Multiple myeloma represents one of the main universal oncological diseases. Due to its clinical characteristics, it is often diagnosed only too late, when it has already determined systemic effects; this results in greater therapeutic difficulty and worse prognostic results. Through the clinical case discussed in this article, we want to focus on the often aspecific manifestations of this pathology and on the need for a correct clinical and diagnostic framework. The main renal manifestations secondary to the deposition of immunoglobulins in both the glomerular and tubular areas will also be examined.

[Assessing the quality of life in patients with a vascular access].

Patients undergoing haemodialytic treatment have a lower quality of life than the general population because of several factors. Their wellbeing can be assessed through a clinical evaluation or through the subjective point of view of the patients themselves: the perceived Health-Related Quality of Life (HRQoL) is an index calculated on the basis of the patients' own perspective. A well-functioning vascular access (VA) and the absence of complications are certainly associated with better health in patients on dialysis but unfortunately VA-related perceived HRQoL has so far been a subject of little interesting literature, even though the choice of the most appropriate access in the individual patient is today increasingly articulate and difficult. Information about subjective perception of health is typically collected through generic or specific questionnaires. The most used reproducible questionnaires available are SF-36, EuroQoL5D, SONG-HD, WHOQoL-BREF, VAQ, although not all of them have been used for a targeted assessment of the issues concerning HRQoL and VA function. This review confirms that the VAQ questionnaire is currently the simplest and most reliable tool to assess patient satisfaction with their VA.

[Efficacy of SUPRA HFR in the treatment of acute renal damage during multiple myeloma].

Acute Kidney Injury (AKI) is a frequent complication of multiple myeloma (MM) with unfavorable prognostic significance. Light chains removal, combined with hematological therapy (CT) seems to offer significant benefits to renal function recovery (RFR). The SUPRA HFR, through the combination of high cut-off membrane without albumin loss and adsorbent cartridge, represents one of the "emerging" light chain removal methods. We report our multicentric retrospective experience with SUPRA HFR in 7 MM patients. At the end of the treatment with SUPRA HFR a significant reduction in serum free light chains compared to baseline was observed (min 24%; max 90%; median 74%). Despite a not always early start of the treatment, all patients recovered renal function with withdrawal from dialysis in 6/7 cases. Our preliminary experience of a combination of SUPRA HFR treatment with CT in 7 MM patients with AKI showed a significative renale functional recovery, with favourable cost/benefit ratio and a simple treatment schedule. These encouraging data suggest to further extend such treatment option, waiting for larger studies in this field.

[Severe lactic acidosis requiring continuos haemodiafiltration in a young patient with unrecognized metabolic abnormality. Case report].

BACKGROUND: Lactic acidosis (LA) is the most common form of metabolic acidosis, defined by lactate values greater than 5 mmol/L and pH<7.34. The pathogenesis of LA involves hypoxic causes (type A) and non-hypoxic (type B), often coexisting. Identification and removal of the trigger are mandatory in the therapeutic management of LA. The case: A 38 years-old male patient entered the Emergency Ward for dyspnea, fever, vomiting and hyporexia. An important respiratory distress with hyperventilation due to severe LA was found, together with severe hypoglicemia, without renal impairment. Past medical history unremarkable, except for reported episodic hypoglicemia in the childhood, with fructose "intolerance", without any other data. No evidence of intoxications, septic shock or significant cytolysis. No drugs causing LA. The patient underwent orotracheal intubation, glucose infusion, and continuous haemodiafiltration for 36-hrs. A rapid general improvement was obtained with stabilization of acid-base balance. A diagnosis of fructose-1,6-diphosphatase deficiency was made. It is an autosomical recessive gluconeogenesis abnormality, with recurrent episodes of hypoglicemia and lactic acidosis after fasting, potentially lethal. The therapy is based on avoiding prolonged fasts, glucose infusion, and a specific diet, rich in glucose without fructose intake. CONCLUSIONS: The presence of not-otherwise-explained lactic acidosis in young patients has to place the suspect of an underlying and unknown metabolic derangement; in these cases, the involvement of the nephrologist appears to be pivotal for the differential diagnosis of the abnormalities of the acid-base balance, and for setting the best treatment.

Metformin-Associated Lactic Acidosis Undergoing Renal Replacement Therapy in Intensive Care Units: A Five-Million Population-Based Study in the North-West of Italy.

BACKGROUND: Metformin-associated lactic acidosis (MALA) is a severe complication of drug administration with significant morbidity and mortality. So far no study in large population areas have examined the incidence, clinical profile and outcome of acute kidney injury (AKI)-MALA patients admitted in intensive care units (ICUs) and treated by renal replacement therapy (MALA-RRT). METHODS: Retrospective analysis over a 6-year period (2010-2015) in Piedmont and Aosta Valley regions (5,305,940 inhabitants, 141,174 diabetics treated with metformin) of all MALA-RRT cases. RESULTS: One hundred and seventeen cases of AKI-MALA-RRT were observed (12.04/100,000 metformin treated diabetics, 1.45% of all RRT-ICU patients). Survival rate was 78.3%. The average duration of RRT was 4.0 days at mean dialysis effluent of 977 mL/kg/day. At admission most patients were dehydrated, and experienced shock and oliguria. CONCLUSION: Our data showed that MALA-RRT is a common complication, needing more prevention. Adopted policy of early, extended, continuous and high efficiency dialysis could contribute to an observed high survival rate. Video Journal Club "Cappuccino with Claudio Ronco" at http://www.karger.com/?doi=471917.