NutreStore

Amino Acids and Derivatives Supplements
Amino Acids for Short Bowel Syndrome

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.

gastrointestinal fistula / Delayed / 10.0
pancreatitis / Delayed / 10.0

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

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

Endari

Rx

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

5 g 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.

15 g PO twice daily.

10 g PO twice daily.

15 g PO twice daily.

10 g PO twice daily.

5 g PO twice daily.

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

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

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

Endari/NutreStore Oral Pwd F/Recon: 5g

Maximum dosage information is not available.

Maximum dosage information is not available.

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

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.

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.

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.

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.

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]