Pyrrolidine and piperidinium symptoms?

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. Pyrrolidine and piperidinium symptoms?

Pyrrolidine and piperidinium symptoms?

Cystine crystal examination: cystine crystal can be seen in concentrated urine sediment in patients with large urinary cystine excretion, which is of great value in the diagnosis of this disease. The morning urine was centrifuged, and the hexagonal flat crystals similar to benzene ring were observed under light microscope. Crystallization often indicates that the concentration of urinary cystine is more than 200-250 mg / L.

Drug treatment: when urinary cystine excretion is too large (> 750 mg / D or 3 mmol / D), or the use of hydrated and alkalized urine can not effectively prevent urinary calculi, mercaptan derivatives, such as D-penicillamine, tiopronin, can be considered α- In recent years, captopril has been tried out. These drugs and cystine form disulfide with high solubility, which greatly reduces the concentration of free cystine in urine. But these drugs can cause serum disease-like reaction, transient neutropenia, proteinuria and reversible nephrotic syndrome.

Hydrated urine: drink more water to maintain polyuria (including at night). To ensure that the 24-hour urine volume is more than 4 L, especially before going to bed at night to drink water, so that the night urine volume is maintained at more than 1.5 L; Because the night long time does not drink water, the urine highly concentrates, is the cystinuria forms the urinary tract stone the key.

matters needing attention

The disease is a lifelong disease, some patients can not maintain treatment for a long time, so the probability of urinary calculi in patients with severe cystinuria is very high. Early diagnosis, timely prevention and treatment of stones, can reduce complications, so that long-term survival of children. Long term non treatment can cause renal failure due to complications. Hartup's disease can be relieved by itself with the increase of age.