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John

Great product, original Japanese Lactis EL027826792JP
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Mike

Good effect. I shall order again. Original made-in-japan package as promised EG401137451JP
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May

Real Japanese laennec. Thank you! I shall order again. EJ377189782JP
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Bob

Great effect! Got my hair back in 3 months. EG401137588JP
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Nike

Lost 3 kilos since started to drink it last month. Great for fitness EG299855913JP
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Any

Bought it for my son. Good effect and great service. EG299855961JP
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Nya

Original touchi, fast delivery and good price. Thank you. EG401137156JP
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Sam

Good medicine. Think it really helps me to fight cancer. EL025661542JP
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John

3 times cheaper than in our country! And great effect for knees EJ365886698JP
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Mike

Great thing to support diet. Lost 4 kilos for 6 weeks. Shall order more EJ365886675JP
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Home Japanese Medicines
Buphenyl tablets 500 mg for urea cycle disorders (sodium phenylbutyrate, Ammonaps, triButyrate)
Buphenyl tablets 500 mg for urea cycle disorders (sodium phenylbutyrate, Ammonaps, triButyrate)
Buphenyl tablets 500 mg for urea cycle disorders (sodium phenylbutyrate, Ammonaps, triButyrate)
Product Code Buphenyl
Availability 5
Price USD 2,290.00
Qty
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Buphenyl tablets 500 mg for urea cycle disorders (sodium phenylbutyrate, Ammonaps, triButyrate)

Product Code : Buphenyl

Availability : 5

USD 2,290.00

Buy now

General information on Japanese Buphenyl tablets 500 mg for urea cycle disorders (sodium phenylbutyrate, Ammonaps, triButyrate)

Package details: 250 tablets

Manufacturer: OrphanPacific Co. Ltd., Japan

Active ingredient: sodium phenylbutyrate (chemical formula C10H11NaO2)

Medical effect: Buphenyl tablets are effective for treatment of urea cycle disorders including following:

  • deficiencies of carbamylphosphate synthetase,
  • deficiencies of ornithine transcarbamylase,
  • deficiencies of argininosuccinic acid synthetase,
  • neonatal-onset deficiency and late-onset disease with a history of hyperammonemic encephalopathy.

Contraindications and precautions: do not use in pregnant or breastfeeding women.

If an allergic reaction occurs, stop using the medication and consult with your doctor. If you’re taking any other medication, please consult with your doctor before use.

This medicine requires dietary restrictions and the control of amino acid levels in blood. Plasma levels of ammonia, arginine, branched-chain amino acids, and serum proteins should be maintained within normal limits, and plasma glutamine should be maintained at levels less than 1,000 µmol/L. The treatment should be performed under the monitoring of the professionals.

Dosage and administration of Buphenyl tablets 500 mg from Japan for urea cycle disorders (sodium phenylbutyrate)

For adults and children weighting above 20 kg: 9.9–13.0 g of active ingredient/m2 of body per day. Divide the daily dosage into 3-6 times and take it with or right after each meal. The maximum dosage of 20 g per day should never be exceeded.

The dosage may be adjusted by the consulting doctor according to the blood ammonia level.

How effective are Japanese Buphenyl tablets 500 mg for urea cycle disorders (sodium phenylbutyrate)?

Buphenyl tablets contain sodium phenylbutyrate, a salt of an aromatic fatty acid. It compensates the lack of liver enzymes and helps the kidneys eliminate waste substances from the body. By this, Buphenyl tablets significantly reduce the blood ammonia level.

Who should take Buphenyl tablets 500 mg from Japan?

Buphenyl tablets are effective for treatment of urea cycle disorders involving deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase, including neonatal-onset deficiency and late-onset disease with a history of hyperammonemic encephalopathy. Neonatal-onset enzymatic deficiency used to be considered fatal. However, currently with hemodialysis, use of alternative waste nitrogen excretion pathways (sodium phenylbutyrate, sodium benzoate, and sodium phenylacetate), dietary protein restriction, and, in some cases, essential amino acid supplementation, the survival rate in newborns diagnosed after birth but within the first month of life is almost 80%. In patients diagnosed during gestation and treated prior to any episode of hyperammonemic encephalopathy, survival is 100%. Also, Buphenyl tablets are now studied as a potential treatment to various diseases, including cystic fibrosis, cancer, retinal ischemia and others (T. Iannitti, B. Palmieri. "Clinical and Experimental Applications of Sodium Phenylbutyrate". Drugs in R&D. 2011, September, 11 (3): 227–249).

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News

Shipping to USA, EU, Asia, Oceania and South America in August

Dear Customers! Japan Post imposed restrictions on shipping to USA, EU and most of other coutnries. Situation changes eevry day. Please check this link for details on each country by Japan Post. https://www.post.japanpost.jp/int/information/overview_en.html We can ship small orders of everything besides vials to Most Countries. Please ask us on detail.  WE STILL CAN SHIP TO MOST COUNTRIES - SEE DETAILS BELOW OR CONTACT US IF YOU HAVE QUESTIONS!   USA: - EXPRESS SERVICE. We ship tablets (ketas) and supplements, eye drops (glanatec) in total weight up to 0,5 kg per order. Delivery takes 6-10 days.  - SURFACE MAIL. We ship all other items (vials), takes 7-9 weeks or more, we offer tracking, Rate starts at 19 usd, free shipping for orders above 400 usd. If you can wait use this method. We can only ship vials and glass using surface mail.   EU - EUROPE We ship as usual (with possible delays) to the countries below (Alphabetic order):  Austria, Belgium, Bulgaria, Croatia, Czech, Denmark, Estonia, France (Monaco), Germany, Hungary, Italy, Latvia, Lithuania, Luxembourg, Netherlands (Holland), Norway, Poland, Portugal, Serbia, Slovenia, Spain, Sweden,     For other coutnries in Europe (Romania, Greece, Ukraine etc): We ship using express service to all other countries in EU at the moment. We can ship by express method for tablets and flat supplements in total weight not exceeding 0,5 kg. We ship in 7-12 business days. Delivery takes 6-10 days. USE EXPRESS METHOD. We can ship using surface mail. This takes 4-6 weeks or more, we offer tracking, Rate starts at 19 usd, free shipping for orders above 400 usd. If you can wait use this method. We can only ship vials and glass using surface mail.   ASIA - SINGAPORE, HONG KOG, KOREA, PHILIPPINES, TAIWAN, THAILAND, VIETNAM, MALAYSIA, PHILIPPINES. We ship as usual (with possible delays) to these countries   SHIPPING TO CHINA. We ship as usual by EMS or Surface mail.    SHIPPING TO INDIA. - WE STILL CAN SHIP TABLETS/CAPSULES. We can ship by express method for tablets and flat supplements in total weight not exceeding 0,5 kg. We ship in 7-12 business days. Delivery takes 8-14 days. USE EXPRESS METHOD. - WE SHIP BY SURFACE MAIL. This takes 4-6 weeks or more, we offer tracking, Rate starts at 19 usd, free shipping for orders above 400 usd. If you can wait use this method. We can only ship vials and glass using surface mail.   OCEANIA - AUSTRALIA. NEW ZEALAND To Australia: - WE STILL CAN SHIP TABLETS/CAPSULES. We can ship by express method for tablets and flat supplements in total weight not exceeding 0,5 kg. We ship in 7-12 business days. Delivery takes 8-14 days. USE EXPRESS METHOD. - WE SHIP BY SURFACE MAIL. This takes 4-6 weeks or more, we offer tracking, Rate starts at 19 usd, free shipping for orders above 400 usd. If you can wait use this method. We can only ship vials and glass using surface mail. TO NEW ZEALAND - WE SHIP AS USUAL    BRAZIL - WE STILL CAN SHIP TABLETS / CAPSULES / EYE DROPS. We can ship by express method for tablets and flat supplements in total weight not exceeding 0,5 kg. We ship in 7-12 business days. Delivery takes 8-14 days. USE EXPRESS METHOD. - WE SHIP BY SURFACE MAIL. This takes 4-6 weeks or more, we offer tracking, Rate starts at 19 usd, free shipping for orders above 400 usd. If you can wait use this method. We can only ship vials and glass using surface mail.   CANADA We ship as usual with delays due to reduced number of flights.   RUSSIA, UKRAINE, KAZAKHSTAN. - WE SHIP BY SURFACE MAIL. This takes 4-6 weeks or more, we offer tracking, Rate starts at 19 usd, free shipping for orders above 400 usd. If you can wait use this method. We can only ship vials and glass using surface mail. - WE CAN SHIP TO KAZAKHSTAN BY AIRMAIL WITH DELAYS. 
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Reviews

John

Great product, original Japanese Lactis EL027826792JP
more ▶

Reviews

Mike

Good effect. I shall order again. Original made-in-japan package as promised EG401137451JP
more ▶

Reviews

May

Real Japanese laennec. Thank you! I shall order again. EJ377189782JP
more ▶

Reviews

Bob

Great effect! Got my hair back in 3 months. EG401137588JP
more ▶

Reviews

Nike

Lost 3 kilos since started to drink it last month. Great for fitness EG299855913JP
more ▶

Reviews

Any

Bought it for my son. Good effect and great service. EG299855961JP
more ▶

Reviews

Nya

Original touchi, fast delivery and good price. Thank you. EG401137156JP
more ▶

Reviews

Sam

Good medicine. Think it really helps me to fight cancer. EL025661542JP
more ▶

Reviews

John

3 times cheaper than in our country! And great effect for knees EJ365886698JP
more ▶

Reviews

Mike

Great thing to support diet. Lost 4 kilos for 6 weeks. Shall order more EJ365886675JP
more ▶