STEROID-RESISTANT NEPHROTIC SYNDROME (SRNS)

Transcription

1 MARIO NEGRI INSTITUTE FOR PHARMACOLOGICAL RESEARCH CLINICAL RESEARCH CENTRE FOR RARE DISEASES ALDO E CELE DACCO' Villa Camozzi Ranica (Bergamo) Italy Telephone fax STEROID-RESISTANT NEPHROTIC SYNDROME (SRNS) Referring Physician Surname Registration Form (revised January 2010) Name Hospital Address Telephone Number Fax Number Patient Data Country Surname Name Sex Birth Date Birth Place Is patient living? If NO, date of death: Address telephone mobile phone Codice fiscale (for italian resident only) Tessera sanitaria (for italian resident only) Family ISS code Diagnosis (Biopsy) Biopsy Date Referring Hospital for the Biopsy Patient Code (please leave blank) Main Laboratory Data at the time of Biopsy: Serum Creatinine (mg/dl) Serum Albumin (g/dl) Urine Sediment Serum Cholesterol (mg/dl) Proteinuria (g/24h) s

2 Renal Biopsy Number of glomeruli studied in the light microscopy Light microscopy and Immunofluorescence Electron microscopy At least 1 glomerulus with segmental or global collapse and overlying podocyte hypertrophy and hyperplasia. At least 1 segmental lesion involving the tip domain (presence of adhesion or confluence of podocytes with parietal or tubular cells at the tubular lumen or neck). At least 1 glomerulus with segmental endocapillary hypercellularity occluding lumina. More than 50% of glomeruli with segmental lesions have perihilar sclerosis or hyalinosis, of which at least 1 glomerulus with perihilar hyalinosis (with or without sclerosis) At least 1 glomerulus with segmental increase in matrix obliterating the capillary lumina. (please, record any other biopsies)

3 Clinical Data Recording Please, specify : Age at onset Age at onset First sign/symptom Microhematuria Gross Hematuria Proteinuria Edema Serum Albumin <3 g/dl Hypercholesterolemia Hypertension Chronic Renal Failure End Stage Renal Disease (ESRD) Associated features : Diabetes Obesity (Body Mass Index) Infectious diseases (HIV, HBV, HCV etc.) Autoimmune diseases (LES, thyroiditis, celiac etc.) Cancer Deafness Ocular abnormalities Peritoneal Dialysis Hemodialysis Renal Transplantation Transplantation Failure Reasons for Transplantation Failure Start at (Date): Start at (Date): Date Genital abnormalities (cryptorhidism etc.) Date of Return to Chronic Dialysis Please specify the type of the feature or other features: Other Renal Transplantation Date

4 Primitive Nephropaty Treatment: Agent/ Dosage Date of beginning Date of suspension Remission (CR/PR/NR) Relapse Corticosteroid Therapy Cyclophosphamide Cyclosporine Mycophenolate Mofetil Angiotensin Converting Enzyme (ACE) inhibitors Angiotensin Receptor Blockers (ARBs) Statins Rituximab More information on the above treatments, other treatments and notes Clinical Data Recording (at the time of samples collection) Date of Collection Serum Creatinine (mg/dl) Serum Albumin (g/dl) Urine Sediment Serum Cholesterol (mg/dl) Proteinuria (g/24h)

5 Family History Consanguinity of parents: Family history for Renal Diseases: Family history for other diseases: If YES specify if there are family members with: Renal biopsy demonstrating FSGS, without evidence of other systemic diseases causing FSGS ESRD/Dialysis Renal Transplantation Proteinuria/Nephrotic Syndrome Autoimmune diseases Diabetes Cancer Deafness Other (please, specify) Chronic Renal Failure Please, specify if there are family members with: (Please, list the relation and the type of the disease) Please if there is a family history draw the pedigree I I I III IV Please point out the proband with an arrow Please affix a progressive number to each component of each generation

6 Family History Identification of the Family Members: Name and Surname I1 I2 I3 If one of the family members is affected, please compile a new registration form!