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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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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
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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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Unknown Deposits

Dear Customers!   IF YOU PAID BY WISE COM before MAY 3rd   AND STILL DID NOT RECEIVE PAYMENT CONFIRMATION FROM US -   Please contact us by email and advise Exact payout amount in EURO.   Our bank had a disruption during holidays, we can not see comments and references, we only see “WISE” and Amount in EURO for all payment received during holidays.   THE LIST OF SIX TRANSACTIONS IS BELOW:   237.90 EUR - APRIL 27th 924.20 EUR - APRIL 27th   236.17 EUR - APRIL 28th 189.20 EUR - APRIL 28th   272.34 EUR - MAY 5th   224.15 EUR - MAY 10th   Please kindly assist if you paid and help to identify payments.   Sincerely yours Japan Health Centre
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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 ▶