renal concentrating dysfunction
Introduction
Introduction The kidney has a concentrated function, which causes disorders in the function of kidney enrichment due to various diseases, such as a decrease in renal concentrating function. Primary glomerular disease: When chronic glomerulonephritis affects the renal medulla, concentrated dysfunction may occur, which is manifested by increased urine output. Renal: such as glomerular disease, chronic tubulointerstitial disease, renal dysfunction, first increased nocturia, followed by renal polyuria, or even renal diabetes insipidus. Older people, especially those with high blood pressure and diabetes, are most prone to nocturia due to renal arteriosclerosis and decreased renal concentrating function. Fanconiyn syndrome (Fanconisyndrome) is also known as Fanconi-deToni syndrome, osteomalacia-renal glucosuria-amino aciduria-hyperphosphate syndrome, and multiple renal tubular dysfunctions are hereditary or acquired proximal kidneys. The clinical manifestations of a group of syndromes caused by abnormal function of tubules are all-acid urinary phosphate urine, glucoseuria, bicarbonate urine, and organic aciduria such as uric acid.
Cause
Cause
The etiology of this syndrome can be divided into two types: primary and secondary primary Fanconi syndrome, which are divided into three types: infant type, adult type and brush border loss type. Secondary Fanconi syndrome Including secondary to hereditary diseases and secondary acquired diseases.
The former includes: cystine storage disease tyrosinemia type I glycogen storage disease type I, galactosemia hereditary fructose intolerance cytochrome c oxidase deficiency, Wilson disease, Lowe syndrome hereditary Insufficient Alport syndrome, congenital nephrotic syndrome, vitamin D-dependent rickets.
The latter include: nephrotic syndrome, transplanted kidney, acute and chronic interstitial nephritis, multiple myeloma nephropathy, Sjogren's syndrome, renal amyloidosis, heavy metal poisoning, drugs (expired tetracycline amino sugar antibiotics, 6-mercaptopurine Kidney damage caused by platinum, etc., hypokalemia, hyperparathyroidism, and tumor-associated nephropathy. Children and children are mostly related to heredity, and adults are often secondary to immune diseases, metal poisoning or kidney disease. Various metabolic complications caused by excessive loss of electrolytes such as hypophosphatemia hypocalcemia, high chloride metabolic acidosis, vitamin D deficiency, osteoporosis dehydration, growth retardation, etc.; Thousands of proteins produce renal tubular proteinuria through renal ammonia uric acid clearance test showing renal tubular excretion dysfunction, glomerular function is basically normal or disproportionate compared with acidosis in recent years, some people will resist vasopressin resistance to polyuria Including it, but amino acid urine, diabetes, phosphate and urine as the basic diagnostic indicators of this disease is rare in China.
Examine
an examination
Related inspection
Glomerular filtration fraction glomerular filtration fraction (GFF)
According to the patient's cause of proximal tubular damage, there is laboratory evidence of proximal renal tubular damage, especially the diagnosis of the combination of amino aciduria, phosphate urine and glucose and urine.
Laboratory inspection:
1. Urine examination of urine is alkaline, low specific gravity urine protein, urine sugar positive, urinary calcium potassium, phosphorus uric acid increased renal all-amino acid urine.
2. Blood test blood calcium, phosphorus, potassium, uric acid carbon dioxide binding force decreased, blood chlorine increased blood alkaline phosphatase increased.
Other auxiliary inspections:
1. Conventional X-ray examination can find osteoporosis, skeletal malformation and urinary calculi.
2. Other examinations: Fanconi syndrome caused by cystine storage disease has cystine crystals examined in the cornea by crystallization analysis in the bone marrow slices, white blood cells, rectal mucosa or slit lamp.
Diagnosis
Differential diagnosis
1. Infant Fanconi syndrome differential diagnosis should pay attention to other causes of renal tubular acidosis, muscle weakness or gait instability similar to neurological disease or primary myopathy is also very similar to Fanconi syndrome infant type should Pay attention to the distinction.
2. Adults with other metabolic bone diseases caused by osteoporosis with myopathy are similar to Fanconi syndrome; uremia patients may have glucose urine or amino acid urine without hypophosphatemia; Wilson disease will also be confused with sports diseases Health search.
3. In general, the composite renal tubular discharge of solute must look for its primary disease, according to the cause of proximal tubular damage caused by the patient, with laboratory evidence of proximal renal tubular damage, especially with amino acid urine, Phosphateuria and glucoseuria combine to characterize each disease to establish a diagnosis.
