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  • CLASSES

    Amino Acids and Derivatives Supplements
    Amino Acids for Short Bowel Syndrome
    Supplemental Dietary Agents

    DEA CLASS

    Rx

    DESCRIPTION

    Oral amino acid
    Used for short bowel syndrome and sickle cell disease
    Routine monitoring of hepatic and renal function recommended in patients receiving parenteral nutrition and L-glutamine

    COMMON BRAND NAMES

    Endari

    HOW SUPPLIED

    Endari/NutreStore Oral Pwd F/Recon: 5g

    DOSAGE & INDICATIONS

    For the treatment of short bowel syndrome in patients receiving specialized nutritional support in conjunction with recombinant human growth hormone.
    NOTE: Glutamine and rh-GH therapy should be used in conjunction with optimal management of short bowel syndrome which may include a specialized oral diet, enteral feedings, parenteral nutrition, fluid, and micronutrient supplements.
    Oral dosage
    Adults

    5 g (1 packet) PO 6 times daily for up to 16 weeks given in conjunction with a specialized diet adjusted for individual patient requirements and preferences. Recombinant human growth hormone (rh-GH) should be given during the first 4 weeks of therapy. Safety and efficacy have not been studied beyond 16 weeks of treatment.

    To reduce the acute complications of sickle cell disease.
    NOTE: L-glutamine is designated as an orphan drug by the FDA for this indication.
    Oral dosage
    Adults weighing more than 65 kg

    15 g (3 packets) PO twice daily.

    Adults weighing 30 to 65 kg

    10 g (2 packets) PO twice daily.

    Children and Adolescents 5 to 17 years weighing more than 65 kg

    15 g (3 packets) PO twice daily.

    Children and Adolescents 5 to 17 years weighing 30 to 65 kg

    10 g (2 packets) PO twice daily.

    Children and Adolescents 5 to 17 years weighing less than 30 kg

    5 g (1 packet) PO twice daily.

    MAXIMUM DOSAGE

    Adults

    Maximum dosage information is not available.

    Geriatric

    Maximum dosage information is not available.

    Adolescents

    Maximum dosage information is not available; safety and efficacy have not been established for the treatment of short bowel syndrome.

    Children

    5 to 12 years: Maximum dosage information is not available; safety and efficacy have not been established for the treatment of short bowel syndrome.
    1 to 4 years: Safety and efficacy have not been established.

    DOSING CONSIDERATIONS

    Hepatic Impairment

    Specific guidelines for dosage adjustments in hepatic impairment are not available; it appears that no dosage adjustments are needed.

    Renal Impairment

    Specific guidelines for dosage adjustments in renal impairment are not available; it appears that no dosage adjustments are needed.

    ADMINISTRATION

    Oral Administration

    NutreStore
    Reconstitute each dose in 8 ounces (240 mL) of water prior to consumption. However, the volume of water may be varied according to the patient's preference.
    Administer with meals or snacks at 2 to 3 hour intervals while awake. Doses may be delayed up to 2 hours if the patient experiences a transient intolerance to oral intake.
     
    Endari
    Reconstitute each dose with 8 ounces (240 mL) of cold or room temperature beverage (e.g., water, milk, apple juice) or 4 to 6 ounces of soft food (e.g. applesauce, yogurt) immediately prior to consumption. Complete dissolution is not required prior to administration.

    STORAGE

    Endari:
    - Protect from direct sunlight
    - Store at controlled room temperature (between 68 and 77 degrees F)
    NutreStore:
    - Store at 77 degrees F; excursions permitted to 59-86 degrees F
    Sympt-X:
    - Storage information not provided in labeling

    CONTRAINDICATIONS / PRECAUTIONS

    Hepatic disease, hepatic encephalopathy, renal impairment

    L-glutamine is metabolized to glutamate and ammonia. Routine monitoring of patients with hepatic disease/hepatic impairment or renal impairment who are receiving parenteral nutrition and L-glutamine is recommended. Ammonia liberated from the use of the product could exacerbate hepatic encephalopathy.

    Geriatric

    Clinical trials of L-glutamine did not contain sufficient numbers of patients 65 years or older to determine whether they respond differently from younger patients. Reported clinical experience has not identified differences. In general, doses in the geriatric population should be cautious and individualized taking into account the greater frequency of decreased renal, hepatic, or cardiac function, and of concomitant disease in this population.

    Pregnancy

    There are no data available on L-glutamine use in human pregnancy to inform a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes. Animal reproduction studies have not been conducted with L-glutamine.

    Breast-feeding

    Endogenous glutamine is present in human milk. There are no data available on the effect of L-glutamine on the breastfed infant or the effect on milk production. Consider the developmental and health benefits of breast-feeding along with the mother's clinical need for L-glutamine and any potential adverse effects on the breastfed child from L-glutamine or the underlying maternal condition.[61644] [62082]

    ADVERSE REACTIONS

    Severe

    gastrointestinal fistula / Delayed / 10.0
    pancreatitis / Delayed / 10.0

    Moderate

    chest pain (unspecified) / Early / 12.0-12.0
    hot flashes / Early / 0-1.0
    constipation / Delayed / 10.0
    hemorrhoids / Delayed / 10.0
    peripheral edema / Delayed / Incidence not known

    Mild

    cough / Delayed / 16.0-16.0
    dyspepsia / Early / 0-1.0
    back pain / Delayed / 10.0
    headache / Early / 10.0
    nausea / Early / 10.0
    abdominal pain / Early / 10.0
    tenesmus / Delayed / 10.0
    vomiting / Early / 10.0
    flatulence / Early / 10.0
    xerostomia / Early / 10.0
    infection / Delayed / Incidence not known
    arthralgia / Delayed / Incidence not known
    fever / Early / Incidence not known
    rash / Early / Incidence not known
    influenza / Delayed / Incidence not known
    hypoesthesia / Delayed / Incidence not known
    rhinitis / Early / Incidence not known
    pruritus / Rapid / Incidence not known
    dizziness / Early / Incidence not known
    musculoskeletal pain / Early / Incidence not known

    DRUG INTERACTIONS

    There are no drug interactions associated with L-Glutamine products.

    PREGNANCY AND LACTATION

    Pregnancy

    There are no data available on L-glutamine use in human pregnancy to inform a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes. Animal reproduction studies have not been conducted with L-glutamine.

    Endogenous glutamine is present in human milk. There are no data available on the effect of L-glutamine on the breastfed infant or the effect on milk production. Consider the developmental and health benefits of breast-feeding along with the mother's clinical need for L-glutamine and any potential adverse effects on the breastfed child from L-glutamine or the underlying maternal condition.[61644] [62082]

    MECHANISM OF ACTION

    Glutamine is an amino acid used in the biosynthesis of proteins.
     
    Short bowel syndrome
    The role of L-glutamine in intestinal adaptation in patients with short bowel syndrome (SBS) remains largely unclear. Research has alluded to its role in gastrointestinal cell growth, function, and regeneration. Patients who have undergone bowel resection develop intestinal failure from the short-bowel resulting in malabsorption of fluids, electrolytes, and other nutrients; dependence on long-term parenteral nutrition may result. Over a period of time a resected bowel undergoes intestinal adaptation where the bowel dilates and lengthens and there is an increase in villus height, crypt depth, cell proliferation, and enzyme activity. This adaptation results in enhanced fluid, electrolyte, and nutrient absorption and an increase in transit time. Intestinal mucosa has the capability of extracting glutamine from arterial circulation where it takes part in the replication of cells located in the intestinal mucosa. Furthermore, glutamine has been shown to be involved in the maintenance of proliferative and secretory functions, mucosal structure, and the passive barrier to bacterial infiltration of intestinal cells.
     
    Sickle cell disease
    The mechanism of L-glutamine in treating sickle cell disease is not fully understood. Sickle red blood cells (RBC) are more vulnerable to oxidative damage compared to normal RBC, which may contribute to the chronic hemolysis and vaso-occlusive events associated with sickle cell disease. The pyridine nucleotides, NAD+ and its reduced form NADH, partially regulate and prevent oxidative damage in RBC. L-glutamine may improve the NAD redox potential in sickle RBC by increasing the availability of reduced glutathione.

    PHARMACOKINETICS

    L-glutamine is administered orally. After an IV bolus dose, Vd was estimated to be approximately 200 mL/kg with a terminal half-life of approximately 1 hour. Endogenous L-glutamine takes part in various metabolic activities, including formation of glutamate and synthesis of proteins, nucleotides, and amino sugars; exogenous L-glutamine is expected to undergo similar metabolism. L-glutamine is eliminated by glomerular filtration but is almost completely reabsorbed by the renal tubules.

    Oral Route

    Mean peak L-glutamine concentrations of 150 mcg/mL occurred approximately 30 minutes after oral administration of L-glutamine 0.1 g/kg in healthy subjects. Multiple dose pharmacokinetics have not been adequately described. Plasma concentrations of L-glutamine after oral administration may be highly variable in patients with short bowel syndrome. Extent of absorption is dependent largely on the length, segment, and the presence or absence of ileal-cecal valve in the bowel remnant of these patients.