[Steroid-resistant focal segmental glomerulosclerosis treated with cascade plasmafiltration and rituximab].

A 39-year man with primary steroid resistant focal segmental glomerulosclerosis (FSGS) was treated with mycophenolate mofetil and ACE-inhibitors. After six months a different therapeutics approach was mandatory due to the worsening of renal function and the relapse of proteinuria at the nephrotic range. The combination of cascade plasmafiltration and single dose of rituximab (375 mg/m²) achieved clinical remission and improved renal function in six months follow up. Cascade plasmafiltration in association with rituximab can be considered as a salvage method for primary steroid-resistant FSGS. Clinical trials should be carried out for protocol approval.

Renal involvement in Waldenström's macroglobulinemia: case report and review of literature.

Waldenström's macroglobulinemia (WM) is a rare lymphoid neoplasia, accounting for 2% of all hematological malignancies. Renal complications occur rather rarely compared to multiple myeloma. The most common renal manifestations are mild proteinuria and microhematuria. We describe a case of MW presenting with acute renal failure and NS. A 67-year-old man was referred to our hospital for sudden onset nephrotic syndrome. Electrophoresis revealed a monoclonal component in the gamma region, which was classified as an IgM k. During hospitalization, acute kidney injury developed, with creatinine up to 5 mg/dL, despite adequate hydration and alkalinization. A kidney biopsy was performed, showing minimal change disease (MCD) with interstitial and capsular lymphoid infiltrates of B-Lymphocytes CD20+. B-lymphocytes infiltration suggested the possibility of renal localization of lymphoproliferative disorder. So, bone marrow histology was performed, revealing lymphoplasmacytic lymphoma (WM). The patient was treated with bortezomib, desamethasone, and rituximab, with partial recovery of renal function (creatinine 1.5 mg/dL) and complete remission of proteinuria after 8-month follow-up. The remission of NS in our patient with rituximab seems to emphasize the pathogenetic role of B cells in MCD, although a coincident effect of immunosuppression on both the underlying renal disease and the hematologic disease cannot be excluded.

Fludarabine in chronic lymphocytic leukemia with membranous nephropathy.

Chronic lymphocytic leukemia (CLL) is the most frequent form of leukemia in Western countries. Despite its relative frequency, the association of glomerular disease is extremely rare. We present a case of membranous nephropathy (MN) during CLL treated with fludarabine. A 74-year-old man was admitted to our hospital because of the onset of nephrotic syndrome (proteinuria was 7 g/24 h). Six years before, he had been diagnosed with CLL. Biochemical analysis showed the following results: creatinine was 1.7 mg/dL (creatinine clearance was 39 mL/min), urea was 64 mg/dL, hemoglobin was 8.6 g/dL, and white blood cells was 16,580/mm(3) (60% lymphocytes). The urine sediment revealed 7-8 red blood cells and many hyaline and granular casts. No monoclonal peak was demonstrated in either serum or urine electrophoresis. Bence-Jones proteinuria was negative. The patient underwent renal biopsy that showed MN with an extensive lymphocyte perivascular infiltration; immunohistochemistry on renal biopsy specimen showed that infiltrating lymphocytes were CD20+. Moreover, DNA from tissue fractions was analyzed by qualitative polymerase chain reaction-based detection of clonal gene rearrangements of the immunoglobulin heavy chain gene, confirming the monoclonality of the infiltrating lymphocytes. The patient was started on fludarabine as monotherapy, with complete remission of proteinuria and recovery of renal function (creatinine clearance was 75 mL/min) after 1 year of follow-up.

Prognostic value of extravascular lung water index in critically ill children with acute respiratory failure.

PURPOSE: In critically ill adults, a reduction in the extravascular lung water index (EVLWi) decreases time on mechanical ventilation and improves survival. The purpose of this study is to assess the prognostic value of EVLWi in critically ill children with acute respiratory failure and investigate its relationships with PaO(2), PaO(2)/FiO(2) ratio, A-aDO(2), oxygenation index (OI), mean airway pressure, cardiac index, pulmonary permeability, and percent fluid overload. METHODS: Twenty-seven children admitted to PICU with acute respiratory failure received volumetric hemodynamic and blood gas monitoring following initial stabilization and every 4 h thereafter, until discharge from PICU or death. All patients are grouped in two categories: nonsurvivors and survivors. RESULTS: Children with a fatal outcome had higher values of EVLWi on admission to PICU, as well as higher A-aDO(2) and OI, and lower PaO(2) and PaO(2)/FIO(2) ratio. After 24 h EVLWi decreased significantly only in survivors. As a survival indicator, EVLWi has good sensitivity and good specificity. Changes in EVLWi, OI, and mean airway pressure had a time-dependent influence on survival that proved significant according to the Cox test. Survivors spent fewer hours on mechanical ventilation. We detected a correlation of EVLWi with percent fluid overload and pulmonary permeability. CONCLUSIONS: Like OI and mean airway pressure, EVLWi on admission to PICU is predictive of survival and of time needed on mechanical ventilation.

Might there be an association between polycystic kidney disease and noncompaction of the ventricular myocardium?

We report on a paediatric case of autosomal dominant polycystic kidney disease, where myocardial hypertrophy proved a consequence of noncompaction of the ventricular myocardium. Deletion of PKD1 and PKD2, the genes responsible for polycystic renal disease, has been linked also to disorganized myocardial arrangement in experimental animals. Two adults with polycystic kidney disease and myocardial hypertrophy in whom a careful diagnostic workup led to a diagnosis of non-compaction of the ventricular myocardium have been reported in the literature. Nephrologists must be aware of the possible association between the two diseases because early recognition of the disease may help in preventing the onset of complications.

Blood pressure load, proteinuria and renal function in pre-hypertensive children.

It is as yet unclear whether blood pressure load (BPL) can affect renal function in pre-hypertensive children. We have studied 250 children, with a mean age of 9.12 +/- 3.28 years, with the aim of assessing if pre-hypertension in children can indeed affect renal function. The study cohort consisted of 146 children with pre-hypertension (group P) and a control group of 104 children with normal blood pressure (group C). All children were tested for orthostatic proteinuria, an exclusion criterion, glomerular filtration rate (GFR), and proteinuria, and ambulatory blood pressure monitoring was performed. Based on the BPL, group P was further subdivided into group P1 (BPL 40%, high BPL). We found that GFR was reduced in pre-hypertensive children (90.74 +/- 48.69 vs. 110.32 +/- 20.30 ml/min per 1.73 m(2), p < 0.0001) and that proteinuria was increased (145.36 +/- 110.91 vs. 66.84 +/- 42.94 mg/m(2) per 24 h; p < 0.0001). However, mean values were still within normal limits. A comparison of the group with high BPL and that with low BPL revealed that the former had relatively reduced GFR (79.15 +/- 42.04 vs. 96.78 +/- 51.20 ml/min per 1.73 m(2); p < 0.006) and increased proteinuria (198.29 +/- 142.17 vs. 118.31 +/- 80.07 mg/m(2) per 24 h; p < 0.036). In comparison to the reference values of the normal population, the GFR was reduced and proteinuria was increased in the group with high BPL. Based on our results, pre-hypertension in children with high BPL seems to be associated with reduced GFR and increased proteinuria. A reasonable doubt remains that the patients with higher proteinuria and larger reduction of GFR may harbor an as yet unknown subclinical renal condition responsible for the onset of pre-hypertension. Therefore, children with even mildly elevated BP are at risk of developing renal damage and should change their lifestyle to prevent further increases in BP.