Effectiveness of rituximab in nephrotic syndrome treatment

Idiopathic nephrotic syndrome (INS) is a common chronic illness characterized by massive proteinuria and hypo-albuminemia in children. Baseline treatment is 6 month-corticotherapy. In cases of steroid resistant/dependent INS several types of treatment are used, including course of methyloprednisolone “pulses”, alkylating agents, cyclosporin A, levamisole and mycophenolate mofetil. It has been suggested that children with frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome had a significantly longer relapse-free period if rituximab (RTX) treatment was additionally applied. We present a case of a 4.5 boy who due to steroid-sensitive, steroid-dependent nephrotic syndrome has been successfully treated with RTX. Administration of the one dose of Rituximab in the patient caused immediate decrease of CD19/CD20 positive B lymphocyte population. The depletion of B cells has been observed for the next six months. With regard to the fact that RTX treatment may affect patient’s immune response, comprehensive immunodiagnostic has been conducted in a course of the Therapy

Diagnostic challenges associated with the first episode of idiopathic nephrotic syndrome in children

Aim: The study was aimed at assessing the difficulties in the diagnosis of the first episode of idiopathic nephrotic syndrome in children, based on the analysis of the initial presenting symptoms, laboratory findings and comorbidities, and evaluating the effect of the time to diagnosis on the course of the disease and its management. Material: The study included the clinical characteristics of 51 paediatric patients aged 4.89 ± 2.72 years. Factors such as the initial presenting symptoms/reason for performing urinalysis, time to diagnosis and presence of comorbid diseases were analysed. On hospital admission, clinical symptoms, laboratory findings and time to remission were assessed. Results: In 40 (78%) children urinalysis was prompted by the presence of oedema, in 11 (22%) by abdominal pain, recurring respiratory tract infections, or was performed as followup to infection. The time from onset of oedema to hospital admission was 1–60 days (median 5.5). Twenty-five children were admitted into hospital within 7 days from disease onset, 10 children – within 8–14 days, 5 children – after more than 14 days. Twelve patients had a recent history of allergy, 9 – frequent respiratory tract infections, 1 – urinary tract infections, and 2 had a family history of nephrotic syndrome. On hospital admission, 40 children presented with oedema (transudation of fluids in to body cavities was present in 27 patients), 25 – with infections (respiratory or gastrointestinal), 8 – with carious lesions, 3 – with insect stings. Laboratory testing showed nephrotic proteinuria – 303 ± 224 mg/kg/day, hypoalbuminemia – 2 ± 0.4 g/dL, mean total cholesterol level – 332.8 ± 104 mg/dL, triglycerides – 222.6 ± 95.3 mg/dL. Proteinuria resolved on average after 9 ± 5.75 days from treatment. A positive correlation has been demonstrated between the number of days that the symptoms persisted and total cholesterol level (r = 0.36, p = 0.012). Conclusions: In 37.5% of children, the diagnosis of idiopathic nephrotic syndrome is delayed, despite oedema. Seventy-one per cent of the children have been found to show signs of an acute or chronic inflammatory condition

Tuberculosis infection in children with proteinuria/nephrotic syndrome

Children with nephrotic syndrome (NS) are at greater risk of infections than the general population, due to immunodeficiency in the course of the disease and the treatment. In this study we present 4 children (2 girls, 2 boys), mean age 7.6 ±5.1 years, with NS/proteinuria and latent tuberculosis in 3 children and lymph node tuberculosis in 1 child. The reasons for testing these children for tuberculosis (TB) were the evaluation of the epidemiological status before treatment with corticosteroids (GCS), leukopenia and the relapse of NS, and non-nephrotic proteinuria. The diagnosis of TB infection was based on positive IGRA (Interferon-Gamma Release Assay). Chest X-ray was normal in all the children. Chest CT scan revealed an enlargement of lymph nodes in 1 child. The children were treated with isoniazid (3 children) and isoniazid, rifampicin and pyrazinamide (1 child). Three children with idiopathic nephrotic syndrome were treated with prednisone. The child with non-nephrotic proteinuria was treated with enalapril. Proteinuria disappeared in all children during anti-TB treatment