The reason of pyrrolidine and piperidine urine?

summary

Pyrrolidine and piperidinium are characteristic manifestations of renal aminoaciduria in children. High amino aciduria is a kind of metabolic disease characterized by excessive excretion of amino acids in urine, which can be divided into prerenal, renal and mixed. The therapeutic effects of various kinds of high amino acid urine are very different. Some patients can get good results by strictly controlling the intake of corresponding amino acids in diet or supplementing some vitamins; Some patients can not improve the clinical symptoms even if they take various measures to make the plasma amino acid level close to normal. Renal aminoaciduria is a disorder of reabsorption of amino acids in proximal renal tubules, which makes a large number of amino acids excreted from urine. Amino acids excreted from urine mainly include glycine, taurine, methylhistidine, etc. In addition, serine, threonine, leucine, phenylalanine can also be a small amount of discharge.

The reason of pyrrolidine and piperidine urine?

Pathological amino acid urine is caused by diseases, including "prerenal" amino acid urine: ① "overflow" amino acid urine: such as Phenylpyruvate urine and maple juice urine, which is caused by some amino acid metabolic defects. ② "Competitive" amino acid urine: such as hyperprolinemia, etc., is caused by competition with amino acids in the same transport system in renal tubules.

Renal "amino acid urine": it is caused by the defect of proximal convoluted tubule transport. ① Single group amino acid transport system defect: that is, the transport system defect of proximal renal tubule to a certain group of amino acids, which makes the group of amino acids excreted from urine, including lysine, arginine, ornithine, cystine, proline, hydroxyproline, glycine, aspartate, glutamate, etc. ② Multiple defects of amino acid transport system: due to multiple functional defects of proximal convoluted tubule, multiple amino acid urine, accompanied by diabetes, hyperphosphatemia, uric acid dysfunction, such as Fanconi syndrome, Lowe syndrome, etc.

Pathogenesis of urinary amino acid excretion increased or individual amino acid excretion significantly increased, known as amino acid urine. Amino acid is an important nutrient in human body, most of which can be used to synthesize protein. The amino acids in plasma can be filtered out to the original urine freely through the glomerulus, and most of them can be reabsorbed back to the blood through the proximal renal tubules. When the renal tubular function is decreased, the excretion of amino acids in urine increases. There are many causes of amino acid urine, most of which are genetic diseases, and some of which are caused by drugs or poisons.

matters needing attention

Renal amino acid urine is a hereditary disease. There are two basic points to prevent urinary calculi caused by cystinuria: one is to reduce the concentration of urinary cystine, the other is to increase the solubility of cystine. If the patient's urinary cystine excretion is less, it belongs to "acalculous cystinuria", no special treatment can be given, dynamic observation, regular review.