nephrotic syndrome

nephrotic syndrome

Nephrotic syndrome is a symptom complex associated with an increase in glomerular permeability, accompanied by proteinuria over 2 g / m2 / day, hypoalbuminemia (less than 30 g / l), edema and hyperlipidemia. The predominant age is 1.5-4 years. The predominant gender is male. Etiology.

• • Primary kidney lesions
  • Minimal change disease
  • Focal Glomerulosclerosis
  • Membranous glomerulonephritis
  • Membranoproliferative glomerulonephritis
  • Mesangioproliferative glomerulonephritis
  • IgA nephropathy
  • Focal segmental glomerulosclerosis.
  • Secondary kidney damage
  • metabolic
  • Diabetes
  • Amyloidosis
  • Systemic diseases
  • SLE
  • Purpura of Shenlai-na-Genoha
  • Polyarteritis nodosa
  • Sjögren’s syndrome
  • Sarcoidosis
  • Serum sickness
  • Erythema multiforme
  • neoplastic
  • Leukemia
  • Lymphomas 4 Multiple myeloma
  • Carcinomas (bronchi, breast, colon, stomach, kidney)
  • Melanoma
  • nephrotoxic, medicinal
  • Salts of gold
  • Penicillamine
  • NSAIDs
  • Lithium salts
  • Heroin
  • allergic
  • Insect bites
  • snake venoms
  • Antitoxins
  • infectious
  • Bacterial
  • Viral
  • Protozoan
  • Helminthiases
  • Hereditary family
  • Allport syndrome
  • Fabry disease
  • mixed
  • Nephropathy of pregnancy
  • Malignant arterial hypertension.

Pathomorphology

• • Minimal change disease. Electron microscopy reveals edema and diffuse swelling of the processes of podocytes, the content of vacuoles, lysosomes and other organelles is increased in podocytes
  • Focal glomerulosclerosis – segmental hyalinization, nodular and coarse-grained deposits of IgM and C3, disappearance of processes in podocytes. Global sclerosis leads to glomerular atrophy
  • Membranous glomerulonephritis – electron microscopy reveals immune complexes in the form of dense deposits, immunofluorescence microscopy – deposits of IgG, IgA, IgM, complement components
  • Idiopathic membranous proliferative glomerulonephritis has three forms
  • Type I – mesangio-capillary glomerulonephritis – deposits (C3, IgG) are located subendothelially, the basement membrane is intact
  • Type II – disease with intramembranous dense deposits – discrete deposits of C3 on the capillary wall, the basement membrane of the tubules and Bdumen’s capsule
  • Type III – subendothelial and subepithelial deposits, on the plane of which a fenestrated basement membrane is formed
  • Mesangioproliferative glomerulonephritis – mesangial cells and matrix contain deposits of IgA, IgM. SZ.

Clinical picture

• • Complaints: loss of appetite, malaise, puffiness of the eyelids, abdominal pain, muscle atrophy
  • Foamy urine is an early objective symptom
  • Edema up to anasarca
  • Difficulty breathing with pleural effusion or swelling of the larynx
  • Chest pain with pleural effusion
  • swelling of the scrotum
  • Volume increase

abdomen with ascites

• • Increase in the volume of the knee joints with hydroarthrosis
  • Pain in the abdomen with swelling of the mesentery
  • Babies often have orthostatic hypotension and shock
  • Oliguriya, OPN
  • High susceptibility to infections due to loss of immunoglobulins, transferrin, zinc (pneumonia, pyelonephritis)
  • Venous thrombosis (renal veins – in 4-8% of cases)
  • Nephrotic crisis as a consequence of activation of the kinin system against the background of hypovolemic shock, massive diuretic therapy
  • Peritoneal signs
  • Erysipelas-like skin erythema
  • Increase in body temperature
  • Violation of calcium-phosphorus metabolism
  • Hypocalcemia
  • convulsive syndrome
  • Bone demineralization
  • tubular disorders
  • Glucosuria
  • Aminoaciduria
  • Hypokalemia.

Laboratory data

• • Urine
  • Proteinuria over 2 g/m2/day, protein/creatinine ratio over 2
  • The urine sediment contains hyaline, fatty, waxy and epithelial casts.
  • Microhematuria, erythrocyte casts
  • Hypoalbuminemia less than 25 g/l, decreased levels of a- and y-globulins, ACTH, TSH, ceruloplasmin, transferrin, antistreptolysin O, complement, immunoglobulins. With SLE, the concentration of IgG is increased, with membranous glomerulonephritis, the level of SZ is not changed
  • Hyponatremia less than 1 mmol/l, hypokalemia
  • Hyperlipidemia: increased total cholesterol, triglycerides, free and esterified cholesterol, phosphatides
  • Lipiduria
  • microcytic anemia
  • Blood clotting disorders due to the loss of coagulation factors IX, XII, thrombolytic factors (urokinase, antithrombin III) in the urine, an increase in the content of factor VIII and platelets in the blood serum.

Treatment is prescribed according to the results of a pathomorphological study obtained as a result of a kidney biopsy.

• • Diet number 7c
  • Treatment of kidney pathology, against which the nephrotic syndrome developed.
  • Parenteral administration of protein solutions, plasma.
  • Plasmapheresis is not indicated (hypoproteinemia).
  • Diuretics
  • Furosemide 40 mg, in the absence of effect, the dose is increased (up to 240 mg / day)
  • Thiazide diuretics (hydrochlorothiazide) are ineffective when glomerular filtration is less than 25-30 ml / min
  • Potassium-sparing – spironolactone 150-200 mg / day.
  • Anticoagulants
  • Heparin 10,000 IU 2 r / day IV under the control of blood clotting time.
  • Antibiotics – for secondary infection.
  • NSAIDs have a temporary antiproteinuric effect, but generally adversely affect the prognosis.
  • ACE inhibitors – captopril 50-100 mg / day.
  • Lipid-lowering drugs – statins (lovastatin 20-60 mg / day).
  • With a nephrotic crisis
  • Replenishment of BCC with plasma substitutes
  • Antikinin agents (parmidine 0.5 g 4 r / day)
  • Antihistamines (suprastin, diprazine, etc.)
  • Antibiotics.
  • Plasma ultrafiltration – with edematous syndrome torpid to diuretics.
  • Nephrectomy followed by chronic hemodialysis and kidney transplantation.

Complications

• • Protein deficiency
  • Arterial hypertension.

The prognosis depends on the etiology of nephrotic syndrome

• • Complete remission begins with glomerulonephritis caused by infections, drugs, with the effectiveness of glucocorticoid therapy
  • With glomerulonephritis with minimal changes, 90% of patients are amenable to therapy
  • Membranous glomerulonephritis
  • In 50% of cases, kidney failure begins within 15 years.
  • In 50% of cases, remission begins or proteinuria persists against the background of intact kidneys.
  • Focal glomerulosclerosis and membranous proliferative glomerulonephritis respond poorly to therapy, renal failure develops within 10 years, in 20% of cases renal failure occurs within 2 years
  • Treatment with glucocorticoids is ineffective in mesangioproliferative glomerulonephritis
  • After kidney transplantation, the recurrence of the disease begins in patients with focal glomerulosclerosis, SLE, IgA nephropathy, type II membranous proliferative glomerulonephritis.

See also Chronic renal failure, Membranoproliferative glomerulonephritis, Membrano glomerulonephritis, Minimal change disease, Mesangioproliferative glomerulonephritis, Lupus nephritis, Nephritic idiopathic steroid resistant syndrome (n1) ICD N04 Nephrotic syndrome