ํฐ์คํ๋ก๋ (Tiopronin)
**ํฐ์คํ๋ก๋(Tiopronin)**์ ์ฃผ๋ก ๋ฐ๋ ค๊ฒฌ์ **์์คํด ์๋ก๊ฒฐ์**(๋ฐฉ๊ด ๊ฒฐ์)์ ๊ด๋ฆฌํ๊ณ ์๋ฐฉํ๋ ๋ฐ ์ฌ์ฉ๋๋ ํน์ **ํญ์๋ก๊ฒฐ์์ **์ ๋๋ค. ์์คํด๋จ์ฆ์ ํน์ ์๋ฏธ๋ ธ์ฐ(ํนํ ์์คํด)์ ์ ์ธ๋จ๊ด ์ฌํก์์ ๋ฐ์ํ๋ ์ ์ ์ ๋์ฌ ๊ฒฐํจ์ผ๋ก, ์์คํด์ ์ฐ์ฑ ์๋ณ์์ ๋งค์ฐ ์ฉํด๋๊ธฐ ์ด๋ ค์ ๊ฒฐ์์ ํ์ฑํ๊ธฐ ์ฝ์ต๋๋ค. ์์ด ์กฐ์ (์ด์ ๋จ๋ฐฑ, ์์นผ๋ฆฌํ ์๋จ) ๋ฐ ์๋ณ ์์นผ๋ฆฌํ๊ฐ ๊ฒฐ์์ ์ฉํดํ๊ฑฐ๋ ์ฌ๋ฐ์ ๋ฐฉ์งํ๋ ๋ฐ ์์ ํ ํจ๊ณผ์ ์ด์ง ์์ ๋ ์ฃผ๋ก ํฐ์คํ๋ก๋์ด ์ฌ์ฉ๋ฉ๋๋ค. * **์ฃผ์ ํน์ง:** * ์๋ณ ๋ด ์์คํด ์ฉํด๋๋ฅผ ๋์ด๋ **ํฌ๋ ์ดํธ์ **๋ก ์์ฉํฉ๋๋ค. * ํ๋์ค๋ผ๋ฏผ์ ๋นํด ๋ถ์์ฉ ๋ฐ์๋ฅ ์ด ๋ฎ์ ์ ์์ด ์ข ์ข ์ ํธ๋์ง๋ง, ๋น๊ต์ ๊ณ ๊ฐ์ ๋๋ค. * ์๋ฐฉ์ ์ฑ๊ณต์ ์ํด์๋ ํ์ ์น๋ฃ์ ๋ณดํธ์์ ์๊ฒฉํ ์์๋๊ฐ ํ์ํฉ๋๋ค.
์์ฉ ๊ธฐ์ : Tiopronin is an active **sulfhydryl (-SH) compound**. In the urine, it undergoes a **thiol-disulfide exchange** with cystine (which is a cysteine-cysteine disulfide). * **Mechanism Pathway:** Tiopronin + Cystine โ **Tiopronin-Cysteine Disulfide Complex** + Cysteine. * This newly formed complex is up to 50 times more water-soluble than cystine alone. * By increasing the solubility of cystine in the urine, tiopronin prevents the precipitation of cystine crystals and facilitates the gradual dissolution of existing **cystine calculi**.
๋๋ฌผ ์ข ๋ณ ์ฉ๋
- Prophylactic treatment of cystine urinary calculi ยท 30 mg/kg PO q12h ยท PO ยท q12h ยท Increase water intake and urine diuresis. Alkalinize urine (pH 6.5-7.0) using potassium citrate. In cases with low cystine excretion and low urolith recurrence rate, tiopronin dose may be individually decreased (<30 mg/kg) or stopped.
- Dissolution of uroliths ยท Approximately 40 mg/kg PO q12h ยท PO ยท q12h ยท Reevaluation of uroliths with ultrasound or radiography every 4th week. After urolith dissolution, give prophylactic dose. If urolith dissolution is not achieved after 2-3 months, surgery is recommended.
- In conjunction with an alkalinizing, protein and sodium restricted diet (e.g., u/d) ยท 30-40 mg/kg PO divided into two daily doses ยท PO ยท divided into two daily doses
- Treatment of cystine urinary calculi ยท 20 mg/kg PO twice daily ยท PO ยท twice daily ยท 1-3 months ยท Relatively high incidence of adverse effects
- Prevention of cystine urinary calculi ยท 15 mg/kg PO twice daily ยท PO ยท twice daily
์ฉ๋์ ๋ฉดํ ์์ ์ ๋ฌธ๊ฐ๋ฅผ ์ํ ์์ ์ฐธ๊ณ ์๋ฃ์ ๋๋ค. ํญ์ ์ต์ ๋ผ๋ฒจ๊ณผ ๊ฐ๋ณ ํ์์ ๋ํด ํ์ธํ์ญ์์ค.
ํฌ์ฌ ๊ฒฝ๋ก
๊ธ๊ธฐ
- Agranulocytosis
- Aplastic anemia
- Thrombocytopenia or other significant hematologic abnormality
- Impaired renal or hepatic function
- Sensitivity to either tiopronin or penicillamine
์ด์๋ฐ์
- Coombs'-positive regenerative spherocyte anemia
- Aggressiveness
- Proteinuria
- Thrombocytopenia
- Elevations in liver enzymes and bile acids
- Dermatologic effects (small pustules, dry crusty nose, ecchymosis, itching, rashes, mouth ulcers, jaundice)
- Myopathy (staggering, difficulty chewing)
- Lethargy
- Sulfur odor of the urine
- GI distress
- Allergic reactions (adenopathy)
- Arthralgias
- Dyspnea
- Fever
- Edema
- Nephrotic syndrome
์ฝ๋ฌผ ์ํธ์์ฉ
- Nephrotoxic drugs ยท Potentially additive toxic effects
- Hepatotoxic drugs ยท Potentially additive toxic effects
- Bone marrow depressing drugs ยท Potentially additive toxic effects
๋ชจ๋ํฐ๋ง
- Physical examination (1, 3, 6, & 12 months after start, then twice a year)
- Ultrasonography/radiography of the urinary tract
- Urinalyses (specific gravity, protein, pH, sediment, and cyanide nitroprusside reaction) using AM samples
- CBC (with platelets)
- Liver enzymes (alkaline phosphatase, ALT)
- Quantitative measurements of urinary cystine excretion related to urinary creatinine excretion (AM samples, before start and once a year during treatment)
๊ณผ์ฉ๋
There is little information available regarding acute toxicity or overdosage of tiopronin in veterinary species. It is suggested to contact an animal poison control center for further information and guidance in the event of an overdose situation. Treatment would likely be supportive and symptomatic.
VetSheet ์ฝ๋ฌผ ๋ ํผ๋ฐ์ค๋ ๋ฉดํ ์์ ์ ๋ฌธ๊ฐ๋ฅผ ์ํ ์์ ์์ฌ๊ฒฐ์ ๋ณด์กฐ ๋๊ตฌ์ด๋ฉฐ, ์ ๋ฌธ์ ํ๋จ์ด๋ ์ ์กฐ์ฌ์ ์ต์ ๋ผ๋ฒจ์ ๋์ ํ์ง ์์ต๋๋ค.