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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 Out Of Stock
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 : Out Of Stock

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).

Frank Yelian | 1 reviews
It has been well known that the buphenyl are effective treating patients with carbamylphosphate synthetase deficiency. Your products have good reputation in the health care industry.

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News

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

Notification from 1st April 2021 - Regarding shipping in Pandemic and Japan Post Stop Act. Dear Customers!   WE CAN SHIP to USA, EU, UAE, Australia, New Zealand, Canada, Hong-Kong, China, Korea, Vietnam, Mexico and all other countries!   Japan Post imposed restrictions on shipping to most of other coutnries. Situation changes eevry day, you can find latest news here - https://www.post.japanpost.jp/int/information/index_en.html Please use search on page to find your country.   IF JAPAN POST DOES NOT SHIP OR SHIPS BY SURFACE MAIL ONLY WE CAN SHIP BY COURIER.   For shiping by Japan Post check this link with full list of countries https://www.post.japanpost.jp/int/information/overview_en.html For shipping by Сourier check table below or contact us as situation changes every day.   SEE TABLE BELOW. SEE COMMENTS BELOW TABLE IF YOU CAN NOT FIND YOUR COUNTRY -- CONTACT US! W CAN SHIP. Shipping type Economy Mail ** Express Service Methods: * SURFACE AIRMAIL Courier05 *** EMS  Argentina NO YES YES YES Armenia YES  YES YES NO Australia YES  YES YES NO Austria YES  YES YES YES Azerbaijan YES  YES YES NO Belarus YES  YES YES NO Belgium YES  YES YES YES Brazil YES NO YES NO Bulgaria YES  YES YES YES Canada YES  YES YES NO Chile NO YES YES YES China YES  YES YES YES Colombia NO YES YES YES Congo YES NO YES NO Costa Rica YES NO YES NO Croatia YES  YES YES YES Cyprus NO YES YES YES Czech YES  YES YES YES Denmark YES  YES YES YES Ecuador NO NO YES NO Egypt YES YES YES NO Estonia YES  YES YES YES Finland YES  YES YES YES France YES  YES YES YES Georgia YES NO YES NO Germany YES  YES YES YES Great Britain YES  YES YES YES Greece YES  NO YES NO Hungary YES  YES YES YES India YES  NO YES NO Indonesia YES  YES YES YES Iran / Iraq NO YES  NO YES Ireland YES YES YES YES Israel YES NO YES NO Italy YES  YES YES YES Kazakhstan YES  YES NO NO Kenya NO YES YES YES Laos YES NO YES NO Latvia YES  YES YES YES Liechtenstein YES  YES YES YES Lithuania YES  YES YES YES Luxemurbg YES  YES YES YES Malaysia YES  NO YES NO Mexico YES  YES YES YES Moldova YES NO YES NO Mongolia NO NO YES NO New Zealand YES  YES YES YES Norway YES  YES YES YES Pakistan NO YES YES YES Panama NO NO YES NO Peru YES NO YES NO Poland YES  YES YES YES Portugal YES  YES YES YES Romania YES  NO YES NO Russia YES NO NO (ASK US) NO Saudi Arabia YES NO YES NO Serbia YES  YES YES NO Slovakia YES  YES YES NO Spain YES  YES YES YES Sri Lanka YES  YES YES YES Sweden YES  YES YES YES Switzerland YES  YES YES YES Tajikistan YES NO YES NO Turkey YES  YES YES YES UAE (Dubai) YES  NO YES NO Ukraine YES YES NO YES USA YES  YES* YES NO Uzbekistan YES NO YES NO Vietnam YES  YES YES YES           * We select appropriate available method. If you need specific method contact us. ** Economy mail is shipped by Air if airmail is available, if not we ship by Surface mail. There are restrictions applied by Japan Post. *** Courier-05, shipping for parcels with tablets up to 5 boxes (most cases), vials 10-20 pcs drops up to 0,5 kg in weight. Can ship to specific regions/cities, please check before order. IF YOU DO NOT SEE COUNTRY ON LIST, CONTACT US!   USA: - EXPRESS SERVICE. We ship by express, Delivery takes 8-12 days.  - AIRMAIL MAIL. If you prefer economy mail, we ship by surface mail, takes 2-4 weeks, no tracking, only local number in Japan, Rate starts at 29 usd, free shipping for orders above 400 usd and 2 kg. If you can wait use this method.   EU - EUROPE We ship as usual to all countries besides Greece, Romania, Moldova and Russia.  For Romania, Greece and Moldova: We ship using express service to all other countries in EU at the moment. Or by surface mail.   ASIA - SINGAPORE, HONG KOG, KOREA, 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 Airmail.    SHIPPING TO INDIA. - WE CAN SHIP BY COURIER. We can ship by express method almost everything. Delivery takes 8-14 days. USE EXPRESS METHOD. - WE SHIP BY SURFACE MAIL. This takes 6-8 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: - EXPRESS SERVICE. We ship by express, Delivery takes 8-12 days.  - AIRMAIL MAIL. If you prefer economy mail, we ship by surface mail, takes 2-4 weeks, no tracking, only local number in Japan, Rate starts at 29 usd, free shipping for orders above 400 usd and 2 kg. If you can wait use this method. TO NEW ZEALAND - WE SHIP AS USUAL    BRAZIL - WE CAN SHIP BY COURIER. We can ship by express method almost everything. Delivery takes 8-14 days. USE EXPRESS METHOD. - WE SHIP BY SURFACE MAIL. This takes 10-15 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 - EXPRESS SERVICE. We ship by express, Delivery takes 8-12 days.  - AIRMAIL MAIL. If you prefer economy mail, we ship by surface mail, takes 2-4 weeks, no tracking, only local number in Japan, Rate starts at 29 usd, free shipping for orders above 400 usd and 2 kg. If you can wait use this method.   UKRAINE, KAZAKHSTAN. RUSSIA. - WE CAN SHIP TO KAZAKHSTAN AND UKRANE as usual by  AIRMAIL WITH POSSIBLE DELAYS. EMS and courier is available for Ukraine.  - Only surface mail is allowed for Russia due to strict customs control for all other methods.
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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 ▶