Wednesday, December 25, 2013

Minimal Change Nephrosis

  
  Minimal Change Nephrosis (MCN) is also called Lipoid Nephrosis. It is characterized with the basically normal glomeruli under light microscope; and with glomerular epithelial podocytes enation, fusion and vanish, with no visible mesangial cell proliferation, the matrix broadening and immunoglobulin deposition under the electron microscope. The clinical manifestation of Minimal Change Nephrosis are often presenting as Nephritic Syndrome, such as: large volume of proteinuria, hypoalbuminemia, edema, hyperlipidemia and so on. Minimal Change Nephrosis is one of the most common glomerular disease in Children. MCN is sensitive to the glucocorticoids treatment with tendency of easy relieving and easy relapsing. For this disease the general prognosis is good, less of the patients (<5%) may progress to End Stage Kidney Failure.
  The cause of Minimal Change Nephrosis.
  Minimal Change Nephrosis can be divided into primary and secondary MCN. The cause of primary MCN is unknown. The cause of secondary MCN includes:
  Drugs: non-steroidal anti-inflammatory drugs (NASIDs), antibiotic (rifampicin, ampicillin, cefixime and so no), Interferon, gold and lithium compound, Methimazole, and Enalapril and so on.
  Infection: HIV, Guillain-Barre Syndrome, Syphilis, Parasite (such as blood fluke) and so on.
  Tumor: Hodgkin’s Disease, Non-Hodgkin’s Disease, solid tumor, Acidophilic Cell- Lymphoma (Kumara Disease).
  Allergy: food, pollen, dust, insect biting and so on.
  Familial Minimal Change Nephrosis: at present, only reported that less families have the Familial Minimal Change Nephrosis, and the pathological gene is unclear.
  The Clinical Manifestation.
  1. Edema.
  The obvious edema is often the first manifestation of MCN. The most children patients present the facial edema, and the adult patients present obvious edema in lower limb.
  2. Proteinuria
  Patients present large volume of proteinuria, and they are often high-selectivity proteinuria, mainly are albumin. The urine protein will be more than 10g/d.
  3. Hypoalbuminemia
  The plasma albumin is often significantly declined. Some individuals patients (commonly to see in children) will be less than 10g/d. Hypoalbuminemia is closely related with amounts of protein lost.
  4. Hyperlipidemia
  Patients with MCN may appear lipid metabolic disorder, plasma cholesterol and triacylglycerol obvious increasing, serum showing chyle color. The patients with hyperlipidemia usually have lipid urine.
  5. Blood Pressure Change
  The patients with obvious hypoalbuminemia and effective circulating blood volume decrease may appear orthostatic hypotension, pulselessness and so on. Meanwhile, some patients may appear temporary hypertension.
  6. Hematuria (blood in the urine)
  About 20% of the patients may appear microscopic hematuria.
  7. Kidney Function Change.
  Most of the patients have a normal kidney function. Less of them may accompany with Prerenal Azotemia due to obvious edema. Some individual patients may appear serious Acute Kidney Failure without obvious inducements. Such disease is called Idiopathic Acute Kidney Failure.
  Diagnosis and Differentiation
  Minimal Change Nephrosis (MCN) is the pathological diagnosis. MCN often has the typical clinical manifestation of Nephrotic Syndrome, that is: large volume of proteinuria (≥3.5g/d), Hypoproteinemia (≤30g/L), severe edema and hyperlipidemia. The pathological feature is: under the light microscope, the glomeruli are basically normal; fatty degeneration can be seen in the epithelial cells of proximal convoluted tubule; the immune pathological test is negative; under electronic microscope, there are broad masses glomerular epithelial foot processes disappeared, and there is no electron dense deposition in the glomeruli are the main diagnosis basis .
  For MCN patients, especially for adult patients, they must carefully detect and exclude the factors for secondary MCN, such as drugs, tumor, infection and allergy, which can only diagnose primary MCN.
  To clarify the diagnosis of MCN must rely on the pathological diagnosis of renal biopsy. It is not difficult to make the diagnosis according to the clinical symptoms and laboratory examination (including renal pathology) result. For children Nephrotic Syndrome patients, they may not consider to do renal biopsy at first. They may receive the glucocorticoid treatment regarding as MCN. If they are steroid-resistant and steroid-dependent, they need to do renal biopsy. For adult Nephrotic Syndrome patients, MCN is not their main pathological type, when they can’t make the diagnosis of MCN according to the clinical symptoms , it is necessary for them to do the renal biopsy.
  Treatments
  1. General Treatment
  The patient at the period of large volume of proteinuria, they should mostly keep stay on the bed or do some proper activities around the bed so as to to prevent the deep venous thrombosis. Patients with obvious edema should have a low salt diet.
  2. Diuretic Treatment
  For patients with low response of glucocorticoid and refractory edema, they can use Thiazide for diuretics, the common drug is hydrochlorothiazide. If the effect is not so good, they can use together with Potassium- sparing diuretics, such as Spirolactone, Triamterene and so on, If it is still not good, it can change to Loop Diuretics.
  3. Anticoagulant Therapy
  Thrombus and embolism are the common complications of Nephrotic Syndrome. If the patient plasma albumin is < 20g/L, they need to give conventional anticoagulant (such as low molecular heparin subcutaneous injection or taking oral warfarin) and platelet aggregation drugs(dipyridamole or taking oral aspirin). If patients are conformed to have the complications of thrombus and embolism, they need to give anticoagulant treatment as soon as possible.
  4 Acute Kidney Failure Treatment.
  For less of MCN patients, their pathogeny of Acute Kidney Failure cannot be found, it can be called MCN complications of idiopathic Acute Kidney Failure. If patients with such disease can be treated correctly and in time, most of them can get complete or better recovery.
  The treatment measures are as the followings:
  ① Loop diuretic: For patients with good response to loop diuretic should use a large dosage of loop diuretic so that it can break the blockage of tubular casts;
  ② Hemodialysis: If patients have no response for diuretic, and need dialysis, they should do dialysis to maintain the life, and to do some proper dehydration after complementing the plasma products in order to reduce the renal interstitial edema;
  ③ The primary disease treatment: For most patients their pathological type is MCN so they should be treated positively;
  ④ Alkalization of urine: Patients can take sodium bicarbonate alkaline by oral to alkalize the urine and reduce the formation of casts.
  5 Special Treatment
  (1) Glucocorticoid: Using the Glucocorticoid to treat MCN, most patients have a good effective and a quick respond. But with the increasing of the patient ages, the effect of Glucocorticoid has will be decreased. So the Glucocorticoid has effect in treating MCN but it is very easy to relapse. In order to reduce the relapsing rate, in China, usually we will use large dosage at the beginning and then gradually reduce the dosage and maintain the dosage for long term.
  (2) Cyclophosphamide (CTX): The cyclophosphamide treatment may use oral taken or intravenous injection method. Such kind of medicine may induce the relapsing kidney disease to get a long time or complete relieving and reduce its relapse. The doctor should pay more attention to the side-effects of CTX and give the treatment these symptoms, such as total number white blood cells reducing, hair loss, gastrointestinal reaction, hemorrhagic cystitis, gonad damage and so on; And try to avoid the large dosage of CTX cause gonad toxicity, which may lead to the serious side-effects of male infertility and tumor.
  (3) Cyclosporin A (CsA): The CsA treatment is suitable for patients of Hormone-dependence and Hormone-resistance.
  (4) Other Immunosuppressors: There is the report of effective treatment of Mycophenloate Mofetil and Tacrolimus (FK506) for MCN.
  6 The characteristic treatment in our hospital
  In our hospital, for the treatment of MCN, besides using some conventional treatments, in order to improve the patient’s kidney function, adjust their immunity and blood circulation, the doctors will ask the patients receive some characteristic Traditional Chinese Medicine (TCM) Treatments, such as: TCM Physiotherapy and Osmotherapy. This is a series of internal and external treatments of Natural Immune Balance Therapy. It is a kind of natural treatment with no gastrointestinal absorption and metabolism of liver and kidneys, no pains and no trauma. Together with the clinical practice it has composed an integrative treatment, which has achieved the effects of curing kidney disease both internal and external, coordinating and co-compensating each other, consolidating the treatment effect.
  For each patient, after the physical examination, the expert doctors will make up a treatment schedule for them according to their examination results and their disease condition, 10 days is one treatment course, commonly, the patient will receive 2 courses continuously treatment, for patient in special condition, their treatment may prolong or stop according to their illness condition.
  Prognosis

  Compared Minimal Change Nephrosis with other pathological types of Nephrotic Syndrome, it may have a good prognosis. More than 70% of children patients have no kidney damage and abnormal urine test after they entered adulthood; And more than 90% of adults patients may keep a normal kidney function for more than 10 years.

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