How long can cystinuria live?

Update Date: 2023-09-27 Source: Network

summary

Cystinuria refers to a large amount of cystine in urine, which is a common genetic disease caused by the deficiency of renal tubular cystine transport. It can occur at any age, especially in children. How long can cystinuria live? Next, I'd like to share my views with you.

How long can cystinuria live?

The concentration of cystine in urine can be reduced by increasing the urine volume. The intake of liquid must be sufficient to ensure that the urine volume reaches 4L / D. the pH value of urine at night decreases,

Adequate water supply is more important. Alkalization of urine with sodium bicarbonate to pH > 7.5 and acetazolamide 5mg / kg (up to 250mg) before bedtime can significantly increase the solubility of cystine in urine,

Other drugs, such as penicillamine, can be considered (although toxicity limits its application). About half of the patients who use penicillamine have toxic reactions, including fever, rash and joint pain, nephrotic syndrome, various types of hemocytopenia, and SLE like reactions, but they are relatively rare

matters needing attention

Taking acetazolamide orally before going to bed can increase the solubility of cystine in urine, or penicillamine can be taken orally. Penicillamine can interact with cystine to form soluble cysteine and disulfide of penicillamine combined with cysteine. However, about half of the patients with long-term penicillamine treatment had toxic reactions, including fever, rash, joint pain, nephrotic syndrome, various kinds of hematocytopenia, and lupus erythematosus (SLE) like reactions, but they were relatively rare.