Atypical Hemolytic Uremic Syndrome: experience of a pediatric center

Abstract

In the last two years we admitted in our Hospital  38 children with acute renal failure (ARF). Six of them were affected by hemolytic uremic syndrome (HUS) atypical. The aHUS is diagnosed in the presence of thrombotic microangiopathy (MAT), renal insufficiency (GFR 5%).

The clinical presentation of our children has been varied and so also its evolution. Patients observed were all male, aged 2 to 12 years, and no one had a family history of kidney disease. In four patients we documented alterations of complement factors (MCP deficiency and factor H and presence of anti factor H). Repeated blood transfusions were required in 4 patients and in 3 patients the platelet count was slightly reduced. In 5 patients we did plasmapheresis and in 3 patients dialysis (hemodialysis and peritoneal dialysis). In three patients in whom the diagnosis was not clear, renal biopsy was performed to confirm the diagnosis. Eculizumab was administered in 3 patients resistant to plasma exchange. We obtain a rapid response on MAT with normalization of platelet count. The effect on renal function was variable (complete remission in a patient, partial improvement in another, and unresponsiveness in the last). The last had on Kidney biopsy signs of severe impairment and we documented the presence of antibodies to eculizumab. HUS is a rare condition, but probably much more common than reported. In children with ARF and microangiopathic anemia is necessary evaluated  complement factors as early to obtain an improved clinical response to treatment with eculizumab.

Keywords: atypical hemolytic uremic syndrome, acute renal failure, pediatric, eculizumab.

Sorry, this entry is only available in Italian.

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ATYPICAL HEMOLITIC UREMIC SYNDROME AFTER ADMINISTRATION OF DOCETAXEL

Abstract

The purpose of this study is to describe the clinical case of a patient suffering from a gland carcinoma with bilateral inguinal and pelvic lymph node metastases in treatment with weekly administrations of Docetaxel.
After two therapy cycles, the patient developed an atypical uremic hemolytic Syndrome (SEUa), treated with infusions of frozen fresh plasma, hemodialysis, and antibiotics.
Because of a severe septic secondary complication on an extensive lymph node abscess, the administration of Eculizumab was not possible.
The patient survived the mentioned Syndrome and is currently in periodic dialysis treatment.

Keywords: aHUS, Docetaxel, Eculizumab

Sorry, this entry is only available in Italian.

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Membranous glomerulonephritis (MGN), ongoing studies

Abstract

The membranous nephropathy (MN) is the major cause of nephrotic syndrome in in the adult, account for 20% of cases with annual incidence is 1 in 100.000.
In the past 10 years, the role of podocytes has been identified; environmental triggers in genetically predisposed patients can activate podocytes to exhibit antigenic epitopes (receptor of phospholipase A2, thrombospondin type 1) that become targets of specific autoantibodies with subsequent complement activation. The discovery of this mechanisms has opened new horizons in the therapy of MN and novel drugs are available with more specific mechanism of action.
Rituximab, a monoclonal antibody directed against CD20 expressed by lymphocytes B, has been used in several trials and appears able to induce remission of nephrotic syndrome in 60% of patients (GEMRITUX trial) with similar risk profile. Nowadays it remains to define the most effective therapeutic pattern.
In MN, the concept of targeting disease control, has permit novel therapies with specific blocking mechanisms (belimumab) and non-specific (ACTH) and new therapeutic options, such as ofatumumab, bortezomib and eculizumab, that have allowed to recognize pathological processes involved in the glomerular diseases.

Key Words: Membranous Glomerulonephritis, membranous nephropathy, Rituximab, ECULIZUMAB

Sorry, this entry is only available in Italian.

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