Betaine

Oral
Adjunct in homocystinuria treatment

Patient w/ CBS deficiency.

Significant: Hypermethioninaemia and severe cerebral oedema (CBS deficiency).
Nervous: Agitation, irritability, brain oedema, depression, personality disorder, sleep disturbances.
GI: Anorexia, diarrhoea, glossitis, nausea, stomach discomfort, vomiting, dental disorders, dysgeusia.
Genitourinary: Urinary incontinence.
Dermatologic: Hair loss, urticaria, abnormal skin odour.

PO: C

Monitor total plasma homocysteine level to determine response. Monitor methionine serum level prior to therapy and periodically thereafter in patient w/ CBS and maintain at <1,000 mmol/L.

Description: Betaine is a methyl group donor that functions in the normal metabolic cycle of methionine. It is used to remethylate homocysteine to methionine, thereby decreasing homocysteine plasma concentration in patient w/ homocystinuria.
Pharmacokinetics:
Absorption: Rapidly absorbed. Time to peak plasma concentration: 0.9 ± 0.3 hr.
Distribution: Rapidly distributed. Volume of distribution: 1.3 L/kg.
Excretion: Via urine (4%). Elimination half-life: 14 hr.

Store between 15-30°C. Protect from moisture.

Other Agents Affecting Metabolism

A16AA06 - betaine ; Belongs to the class of amino acids and derivatives products. Used in treatment of alimentary tract and metabolism problems.

Schwahn B, Hafner D, Hohlfeld T et al. Pharmacokinetics of oral betaine in healthy subjects and patients with homocystinuria. Aug 22, 2002. British Journal of Clinical Pharmacology. 2003:6-13. doi: 10.1046/j.1365-2125.2003.01717.x. Accessed 03/08/2017

Anon. Betaine. Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. https://online.lexi.com. Accessed 02/08/2017.

Buckingham R (ed). Betaine. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 02/08/2017.

Crystadane Powder For Soln (Orphan Europe SARL). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed/. Accessed 02/08/2017.

Joint Formulary Committee. Betaine. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 02/08/2017.

McEvoy GK, Snow EK, Miller J et al (eds). Betaine. AHFS Drug Information (AHFS DI) [online]. American Society of Health-System Pharmacists (ASHP). https://www.medicinescomplete.com. Accessed 02/08/2017.