Disease Description (Causes and Symptoms)
- Definition: A form of nephrotic syndrome in which adhesion (effective loss, effacement) of the foot processes is observed on electron microscopy of the glomeruli, while the findings are almost normal on light microscopy and immunofluorescence testing. English name: Minimal change disease (MCD).
- Causes/Pathophysiology: Cytokines or other blood-borne factors, presumed to result from an immune abnormality (mainly involving T cells), act on podocytes, damaging the foot process structure and increasing the permeability of the filtration barrier → massive proteinuria occurs. As a result, decreased blood albumin, accumulation of tissue fluid due to reduced intravascular oncotic pressure (edema), and increased synthesis of lipids and coagulation factors by the liver (hyperlipidemia and hypercoagulability) may occur.
- Main symptoms: Generalized edema (especially of the face, limbs, and genitals), acute or gradually worsening edema, heavy proteinuria (foamy urine), fatigue, shortness of breath (when pleural effusion or ascites is present), and increased risk of infections and thrombosis.
- Key points in diagnosis: Urine tests (proteinuria), low blood albumin and hyperlipidemia findings, and, when necessary, kidney biopsy (foot process effacement on electron microscopy). In children, a biopsy may be omitted if there is a good response to steroids.
Treatment Methods
- First-line treatment (standard): High-dose corticosteroids (oral prednisone/prednisolone) — the treatment response rate is high in children (most are steroid-sensitive).
- The specific dose and duration are determined according to the patient’s age, weight, and response, so always follow the specialist’s instructions.
- Steroid resistance, relapse, or steroid dependence:
- Immunosuppressants: Options include cyclophosphamide, calcineurin inhibitors (cyclosporine, tacrolimus), mycophenolate mofetil (MMF), and rituximab (anti-CD20), among others.
- The choice varies according to patient characteristics (age, risk of adverse effects, pregnancy plans, etc.).
- Conservative and symptomatic treatment:
- Low-salt diet (to relieve edema) and adequate calorie and protein intake (follow the doctor’s recommendations).
- Diuretics (for severe edema), and, when necessary, intravenous albumin infusion (use alone is temporary; combination with a diuretic may be considered).
- ACE inhibitors/ARBs: To reduce proteinuria (with concurrent blood pressure management).
- Management of hyperlipidemia: Statins and other treatments (for persistent hyperlipidemia).
- Prevention of thrombosis: Consider anticoagulation in cases of severe hypoalbuminemia (e.g., albumin <2–2.5 g/dL) or clinical risk factors, as determined by a doctor.
- Infection prevention and management: Prevent infections during immunosuppressive treatment (evaluate vaccinations and provide early treatment if an infection occurs).
- Treatment precautions:
- Monitoring is necessary for adverse effects of steroids and immunosuppressants (osteoporosis, growth delay in children, hyperglycemia, increased risk of infection, etc.).
- Herbal medicines, folk remedies, and other nephrotoxic or unproven medications are prohibited.
Checklist Before an Outpatient Visit (Including Example Questions)
- Items/records to prepare:
- A list of all medications and supplements currently being taken (including doses and dosing times).
- Copies, if available, of recent urine test results (proteinuria, urine protein/creatinine ratio) and blood test results (blood albumin, creatinine, electrolytes, and lipid levels).
- Records of changes in weight and blood pressure, and photos or a diary of edema (location and time of onset).
- Notes on changes in symptoms (shortness of breath, abdominal pain, pain, fever, changes in urine output, etc.).
- Vaccination records (especially those related to infection risk).
- Examples of things to ask in advance at the outpatient visit:
- Have there been any recent changes in the proteinuria (or urine test) values?
- Have any new symptoms (fever, cough, decreased urine output, etc.) occurred while taking the medication?
- Is there a history of response to previous steroid treatment (number of improvements and relapses)?
General Example Questions to Ask the Doctor
- What are the expected benefits and major adverse effects of this medication (steroid/immunosuppressant)?
- How long does it usually take to achieve treatment success (complete remission)? How likely is relapse?
- Is the risk high enough to require anticoagulation (thrombosis prevention), and if so, which medication should be started and when?
- Are there any medications, foods, or activities that should be specifically avoided in daily life?
- What are the recommendations regarding vaccinations (especially live attenuated vaccines)?
Examples of Disease-Specific Questions
- (For children/guardians) When starting steroids for the first time, in how many weeks does proteinuria usually disappear? What is the next plan if there is no response?
- (For adults) Please explain whether a kidney biopsy is recommended at the time of initial diagnosis in adults and when a biopsy would be recommended.
- (For patients with relapse) In cases of frequent relapse or steroid dependence, what are the advantages, disadvantages, and long-term adverse effects of available steroid-sparing medications?
If you need additional information, please provide test results (such as the recent urine protein/creatinine ratio and blood albumin and creatinine levels), and we can help you prepare more specific items and questions.