Author: Leo and Lucy
Keywords: Fanconi syndrome
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Fanconi syndrome (FS) is a tubal dysfunction disorder, proximal tubule complex transport dysfunction, glucose, bicarbonate, phosphate, uric acid, potassium, sodium and some amino acids from the urine Excessive excretion.
definition
Fanconi syndrome (FS) is a tubal dysfunction disorder, proximal tubule complex transport dysfunction, glucose, bicarbonate, phosphate, uric acid, potassium, sodium and some amino acids from the urine Excessive excretion.
Clinical manifestations
Renal glucuria, renal organic acid urine, renal tubular proteinuria, proximal renal tubular acidosis, urinary phosphate and electrolyte loss, which caused hypophosphatemia, hypocalcemia, dehydration, osteoporosis Wait.
Etiology
Divided into primary and secondary categories.
Primary FS is divided into infant type, adult type and brush-shaped missing type of three types;
Secondary FS also includes secondary to inherited disease and secondary to acquired acquired disease.
- Most children are related to inheritance. Acquired Fanconi syndrome is mainly seen in adults, refers to acquired acquired factors caused by FS, can be seen in a variety of serious damage to the proximal tubular diseases such as drug nephrotoxicity, tubulointerstitial nephritis, nephrotic syndrome, Renal vein thrombosis, multiple myeloma, light chain deposition disease, amyloidosis, Sjogren's syndrome, hypokalemic nephropathy, hyperparathyroidism and renal medullary cystic disease, heavy metal poisoning, Balkan kidney disease.
- The use of antibiotics and antiviral drugs after infection is a common cause of acquired vancomere syndrome.
diagnosis
According to the typical clinical manifestations of Fanconi syndrome:
Amino acid urine, renal diabetes, phosphate urinary, uric acid urinary, bicarbonate urine, high chlorine metabolic acid, the syndrome is easy to diagnose.
But important is the early diagnosis, the majority of patients were diagnosed with bone and kidney damage was often misdiagnosed as other diseases, but also pay attention to the diagnosis of incomplete vancome syndrome.
Cause of diagnosis: because FS is only a syndrome, need to make the cause of diagnosis.
In order to obtain the cause of diagnosis, blood removal, urine biochemical tests, if necessary, need to do renal biopsy pathological examination.
For a variety of tubulointerstitial neonatal acquired vancome syndrome, renal biopsy is often necessary, it is not only conducive to the diagnosis and differential diagnosis of vancomini syndrome, but also conducive to the diagnosis and identification of tubulointerstitial nephropathy Diagnosis, which clearly caused Fan Kooni syndrome of the primary disease, is conducive to the removal of the etiology of vancomycin syndrome. Acquired Fanconi syndrome can often be diagnosed from the original disease clues.
Clinically, doctors often found that patients with acidosis and consider the diagnosis of this syndrome, further examination found that urinary glucose, bicarbonate, phosphate, uric acid, potassium and sodium excretion increased.
treatment
Etiology of treatment is the most effective treatment of acquired vancomini syndrome, and can cure FS. On a variety of tubulointerstitial disease caused by Fan Coney syndrome, to the primary disease for effective treatment. Drugs caused by the removal of the disease after the disease can be reversed. Symptomatic treatment is mainly to supplement the proximal tubule complex dysfunction caused by bicarbonate, phosphate, potassium, sodium loss from the urine. And hypuricemia is due to excessive uric acid from the kidneys caused by hyperuricemia and urinary tract stones, urinary urinary tract should do a good job of prevention and treatment, amino acid and renal tubular proteinuria without special treatment.
Adefovir dipivoxil and vancomycin syndrome
Antiviral drugs adefovir dipivoxil (ADV) can cause FS. The drug can cause impaired renal tubular function, hypophosphatoid osteomalacia, overbased phosphatase, muscle weakness and creatine kinase elevation. ADV nephrotoxicity is time-dependent and dose-dependent.
ADV renal damage may be associated with strong affinity for ADV in the proximal tubule of ankylated transporter tube (HOAT-1), leading to the accumulation of drugs in the proximal tubule and the toxicity of ADV to mitochondria. Etiology of treatment is Fan Kangi syndrome the most effective treatment, disable ADV, the clinical manifestations have been improved or disappeared, renal diabetes, proteinuria also improved.
Long-term use of ADV patients, especially in older patients, in addition to routine monitoring of renal function, the need to pay attention to changes in blood phosphorus and urine, etc., help early detection of kidney damage, such as the body is not fixed pain with renal function Abnormalities, urinary protein, renal diabetes, etc., to be further examination of the possibility of exclusion of vancomycin syndrome, early diagnosis, once the clinical diagnosis of FS, must immediately disable the ADV, kidney damage can be reversed.
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