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Dear Editor: Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common inherited kidney disorder and affects up to 12 million individuals worldwide. Radiologic imaging is critical for successful management. Until recently, the treatments were only symptomatic but EMA in 2017 and FDA in 2018 approved Tolvaptan for ADPKD therapy. Renal imaging provide important diagnostic and management guidance in the monitoring of disease progression. In the past, standard radiographic imaging has not provided the accuracy necessary to reliably measure renal [...]

Purpose: Calciphylaxis or Calcific Uraemic Arteriolopathy (CUA) is a rare systemic disorder associated with endstage renal disease and carrying a very poor prognosis and high mortality. It is characterized by skin necrosis and gangrene secondary to thrombosis and calcification of dermo-epidermal arterioles. Pathogenetic mechanisms inducing calciphylaxis are for the most part unknown. Methods: We report a case of calciphylaxis in a patient on long-term haemodialysis with severe secondary hyperparathyroidism. Despite therapies for hyperparathyroidism, the injury healed only after parathyroidectomy. Results: Calciphylaxis is an obliterative vasculopathy. The role of severe hyperparathyroidism in the pathogenesis of calciphylaxis is not well understood. A unique feature is the phenotypic differentiation of smooth muscle cells into osteoid-like cells. The vascular smooth muscle cells, exposed to high inorganic phosphate levels, could be transformed into osteoblast-like cells in patients undergoing haemodialysis with a poor control of phosphate intake. Conclusions: The uraemic condition contributes to vascular smooth muscle cell apoptosis and differentiation into bone forming osteoblast-like cells resulting in medial calcification. Nowadays no effective treatments are available for the successful management of calciphylaxis.

To the Editor: Today in developed countries, infection-related glomerulonephritides are incommon and often associated with debilitating diseases. Legionnaires’ disease (LD) is one of the most common causes of severe community-acquired acute pneumonia in Europe and in up to 40% of cases of hospital-acquired pneumonia. The acute kidney injury (AKI) in LD is a well described complication but the renal morphology has only been reported in a few cases. The Tubulointerstitial Nephritis (TIN) is the most diagnosed histological change; instead, there [...]

SummaryHepatitis C virus (HCV) infection is a never-ending public health problem. Many studies have investigated the incidence of HCV infection among dialysis patients, but there have only been a few epidemiological studies in renal conservative therapy. We studied 320 subjects with pre-dialysis chronic kidney disease living in Sicily, Italy. The incidence of HCV infection was 6.25%. In Europe, incidence ranges from 0.2% to 3.5%. It appears that the incidence of HCV infection is higher in the studied patient population than in the population as a whole.

Strongyloidiasis is caused by a small intestinal nematode with a complex life cycle. In Italy the infection is endemic in rural areas of the Po Valley. The clinical syndrome of S. stercoralis encompasses a broad spectrum of symptoms and signs and, in the immunocompromised host, larvae can migrate to different organs and tissues. Also immune response seems to play a role in the pathogenesis of the disease. We report a case of strongyloidiasis complicated by Gram-negative sepsis and nephrotic syndrome in an immigrant from South America with a normal immune response. Whereas sepsis cleared up quickly, parasitic clearance was obtained only after treatment with ivermectin and nephrotic syndrome was still present three months after the end of treatment.

Sir, The importance of renal function in the prognosis of patients with multiple myeloma (MM) has been well established and rapid reversal of renal failure may offer the best long-term outcome. The use of extracorporeal means of removing immunoglobulin free light chains (FLCs) responsible for cast nephropathy in patients with MM is controversial. Nephrologists are interested in a treatment strategy combining chemotherapy and extracorporeal treatments in patients with biopsy-proven cast nephropathy or a high probability of cast nephropathy (defined as >200 mg/dL on the FLC assay). Some previous studies have shown that the plasma exchange is unable to remove sufficient FLCs for clinical benefits. The issue of whether an extended duration of dialysis with high cut-off (HCO) dialysers is more effective than plasma exchange at removing FLC or reversing renal failure is not settled [1]. Hutchison and Leung reported that a 60% reduction in FLCs by Day 21 is associated with recovery of renal function for 80% of the studied patients [2]. Our experience: a 43-year-old man, who was not complaining of any previous disease and with a previously normal renal function, suffering from biopsy proven lambda myeloma kidney, without any histological sign of chronic renal damage, and renal failure dialysis dependant, in 2 weeks received 10 haemodialysis (HD) treatments with HCO-dialyser (Theralite Gambro). We quantified the concentration of FLCs by nephelometry (Biocite, N latex test; Siemens). Initially, λ FLC concentration was 5500 mg/L. At the end of HCO dialyser HD cycle, the concentration was 94.80 mg/L (Table 1). Table 1. Concentrations and ratios of FLC before and after HCO-dialyser HDa We did not observe any adverse effects. We observed by Day 7 a sustained and >50% reduction of FLCs with dialysis alone before the chemotherapy was initiated. After the third HCO-dialyser HD, the patient started PAD Orlowsky chemotherapy (Bortezomib–Doxorubicin–Dexamethasone) with successful haematological result but with partial renal function recovery. Currently (5 months after HCO-dialyser treatment), the patient is on maintenance HD two times a week. Some reports show a recovery of renal function after several months. If the patient does not recover normal renal function, we think that a new kidney biopsy can help his management.

Wegener Granulomatosis is a systemic Anti-Neutrophil Cytoplasmic Autoantibody-associated Vasculitis, affecting small-to-medium vessels. Clinical presentation with simultaneous involvement of kidney and upper and lower respiratory tract is unusual. We report an instructive case of WG, analyzing clinical course, laboratory, and radiological features, kidney, lung, and larynx histological pictures. Besides renal biopsy, nephrology team performed larynx and lung biopsies because of unusual clinical presentation, computed tomography chest examination, and relevant malignancy risk regarding following immunosuppressant therapy.

■■ Non-traumatic spontaneous rupture of the kidney in subjects on chronic hemodialysis is a rare event. In most cases it is associated with acquired cystic kidney disease (ACKD) [1] or the presence of kidney cancer. [2] There are few reports on this topic. Until 2009 Goto identified in the Japanese literature only five reports of renal cell carcinoma in patients with end-stage renal disease (ESRD) manifesting with spontaneous rupture. [3] In the general population primary neoplasms of the renal pelvis are uncommon and most are malignant. The majority of these cases consist of transitional cell carcinoma. Squamous cell carcinoma (SCC) and adenocarcinoma (AC) of the renal pelvis are highly uncommon tumors and constitute less than 1% of upper urinary tract neoplasms. [4] SCC and AC are known to be associated with chronic renal calculi and infection. The incidence of coexisting urinary stone disease varies from 18% in the United States to 100% in Hong Kong. [5,6] The

To the EditorWe read with interest the recent work: ‘‘Minimal changenephrotic syndrome in a patient with strongyloidiasis’’ [1]whereDr.Miyzakiandcolleaguesquote15reportedcasesofnephropathy associated with Strongyloides stercoralis (Ss).We would like to add to this list another case that wereported in 2007 regarding this topic [2]. A 25-year-oldmale,borninEcuadorandlivinginItalyfrom3 yearsofage,developed fever, vomiting, malnutrition, abdominal pain,watery diarrhea, dehydration with arterial hypertension andedema in both lower extremities. On admission, laboratorytests showed proteinuria (4 g/day), hypoalbuminemia(1.9 g/dl), hypercholesterolemia, eosinophilia and lowplatelets. Renal and liver function tests, serum immuno-globulin and complement, antinuclear antibodies, ANCAwere unremarkable; HAV, HBV, HCV, HIV, VDRL, BKdetection and fecal and urine cultures were negative.Screening stool for rhabditiform Ss larvae was positive.Hemoculture was positive for Escherichia coli. The renalbiopsy specimen contained 32 glomeruli under lightmicroscopy examination and all had a normal appearance.No vascular or tubularinterstitial lesions were seen. Immu-nofluorescent studies were negative for IgA, IgG, IgM, lightchains, C1q, C3, C4 and fibrinogen. The electron micro-scopic examination showed disappearance of slight dia-phragms and moderate fusion of foot processes ofglomerular epithelial cells, associated with microvillousdegeneration and sometimes with a tortuous course of thebasement membrane. We made a diagnosis of minimalchange disease. The patient was treated with prednisone(1 mg/kg/day), sulfamethoxazole-trimethoprim (800–160mg twice a day) and albendazole (400 mg twice a day for3 days); sepsis cleared up quickly and the patient was dis-charged; however, 3 months later he was admitted againbecause of acute renal failure, diarrhea and nephrotic syn-drome. We detected rhabditiform Ss larvae and IgG anti-Ss(283 UI/ml). The patient was successfully treated withivermectin; screening for rhabditiform Ss larvae and IgGanti-Ss became negative with recovery of normal renalfunction. Six months later the patient did not show any signof parasitic infection but there was proteinuria (1 g/day)without any other sign of nephrotic syndrome. Afterwardsthe patient was lost to follow-up.

Sir, We read with interest the recent case report ‘Minimal change disease with interferon-β therapy for relapsing remitting multiple sclerosis’ [1]. In this paper, the authors include renal thrombotic microangiopathy (TMA) among rare side effects of interferon (IFN) therapy, more frequently described with IFN-α [2]. We report here our experience about this topic. A 36-year-old white female with a 3-year history of multiple sclerosis and normal blood pressure and renal function was admitted for acute renal failure and pulmonary oedema. Three months previously, she started subcutaneous IFN-β-1a treatment of 22 μg thrice weekly. On admission, physical examination showed high blood pressure and severe pleuropericarditis without neurological or dermatological findings. Laboratory tests revealed microangiopathic haemolytic anaemia. Other immunological and microbiological laboratory tests were unremarkable. A renal biopsy disclosed signs of TMA; among 43 glomeruli, light microscopy revealed focal ischaemic signs and mild mesangial cell proliferation; vessel narrowing with thrombi and thickening of arteriolar walls and intimal onion skin-like swelling; light interstitial lymphomonocytic infiltration and focal tubular atrophy. Immunofluorescence showed mesangial IgM, C1q and fibrinogen staining. A diagnosis of haemolytic–uraemic syndrome was made. She was treated with transfusions, haemodialysis, plasma exchange and methylprednisolone i.v. followed by oral prednisone. Her cardiac function improved, and haematological signs progressively disappeared, but renal function did not recover. IFN-β treatment was discontinued. She is now receiving peritoneal dialysis treatment. IFN-α is known to cause a variety of renal lesions, including TMA [3,4], but to our knowledge, our observation is the first report of TMA induced by INF-β. Editorial note: This letter had been sent to Aravindan A. et al., but we did not receive a response. Conflict of interest statement. None declared.