Dosing & Uses
Dosage Forms & Strengths
powder for oral administration
- 5g/packet (as L-glutamine)
Sickle Cell Disease
Endari: Indicated to reduce acute complications of sickle cell disease
<30 kg (<66 lb): 5 g PO BID
30-65 kg (66-143 lb): 10 g PO BID
≥65 kg (≥143 lb): 15 g PO BID
Also see Administration regarding mixing oral powder with beverage or food
Short Bowel Syndrome
NutreStore: Indicated for short bowel syndrome in patients receiving specialized nutritional support when used in conjunction with recombinant human growth hormone
30 g/day PO in divided doses (ie, 5 g taken 6 times each day) for up to 16 weeks
Also see Administration regarding mixing oral powder with water
Dosage Forms & Strengths
powder for oral administration
- 5g/packet (as L-glutamine)
Sickle Cell Disease
Endari: Indicated to reduce acute complications of sickle cell disease in adults and children aged ≥5 yr
<5 years: Safety and efficacy not established
≥5 years
- <30 kg (<66 lb): 5 g PO BID
- 30-65 kg (66-143 lb): 10 g PO BID
- ≥65 kg (≥143 lb): 15 g PO BID
- Also see Administration regarding mixing oral powder with beverage or food
Short Bowel Syndrome
NutreStore: Safety and efficacy not established
Adverse Effects
>10%
Endari
- Constipation (21%)
- Nausea (19%)
- Headache (18%)
- Abdominal pain (17%)
- Cough (16%)
- Pain in extremity (13%)
- Back pain (12%)
- Chest pain (12%)
NutreStore
- Adverse effects listed below are those reported that exceed growth hormone without glutamine supplementation
- Peripheral edema (81%)
- Nausea (31%)
- Tenesmus (19%)
- Rhinitis (19%)
- Dizziness (13%)
- Rash (13%)
- Ear or hearing symptoms (13%)
1-10%
NutreStore
- Pruritus (6%)
Warnings
Contraindications
None
Cautions
Metabolized to glutamate and ammonia; levels of these metabolites may increase with hepatic impairment
Monitor renal and hepatic function
Pregnancy & Lactation
Pregnancy
No available data regarding use in pregnant women to inform a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes
Lactation
Endogenous glutamine is present in human milk; there is no information on effects of supplement on the breastfed infant or effect on milk production; developmental and health benefits from breastfeeding should be considered along with mother’s clinical need for drug and any potential adverse effects on breastfed infant from drug or from the underlying maternal condition
Pregnancy Categories
A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.
B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk. C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done. D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk. X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist. NA: Information not available.Pharmacology
Mechanism of Action
Sickle cell disease
- The mechanism of action of the amino acid L-glutamine in treating sickle cell disease (SCD) is not fully understood
- Oxidative stress phenomena are involved in the pathophysiology of SCD; sickle RBCs are more susceptible to oxidative damage than normal RBCs, which may contribute to the chronic hemolysis and vaso-occlusive events associated with SCD
- Pyridine nucleotides, NAD+ and its reduced form NADH, play roles in regulating and preventing oxidative damage in RBCs L-glutamine may improve the NAD redox potential in sickle RBCs through increasing the availability of reduced glutathione
Short bowel syndrome
- L-glutamine has important functions in regulation of gastrointestinal cell growth, function, and regeneration
- When glutamine was administered in combination with growth hormone to rats, villous height, bowel growth, plasma insulin-like growth factor I, and body weight were significantly higher than in rats treated with either glutamine or rhGH alone
Absorption
Peak plasma time: 30 min
Peak plasma concentration: 150 mcg/mL
Distribution
Vd: ~200 mL/kg
Metabolism
Glutamine participates in various metabolic activities, including the formation of glutamate, and synthesis of proteins, nucleotides, and amino sugars
Elimination
Half-life: ~1 hr
Metabolism is the major route of elimination for glutamine
Although glutamine is eliminated by glomerular filtration, it is almost completely reabsorbed by the renal tubules
Administration
Oral Administration
Endari
- Mix immediately before ingestion with 8 oz of cold or room temperature beverage (eg, water, milk, apple juice) or 4-6 oz of soft food (eg, applesauce, yogurt)
- Complete dissolution is not required prior to administration
NutreStore
- Dissolve each 5 g packet in 8 oz water prior to drinking; volume of water may be varied according to the patient’s preference
- Take with meals or snacks at 2-3 hr intervals while awake
- If transiently intolerant to oral intake, a dose may be delayed for up to 2 hr
Storage
Store at 20-25°C (68-77°F) away from direct sunlight
Images
Formulary
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Adding plans allows you to:
- View the formulary and any restrictions for each plan.
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- Compare formulary status to other drugs in the same class.
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