Version May 2024
Component of parenteral nutrition (Elcys): Note: Correct severe fluid, electrolyte, and acid-base disorders prior to administration. Cysteine dose is based on recommended daily protein (as amino acid) requirement.
Stable or critically ill patients: IV: 7 mg cysteine hydrochloride per gram of amino acids (equivalent to 4.8 mg cysteine per gram of amino acids).
There are no dosage adjustments provided in the manufacturer's labeling; use with caution. Contains aluminum, which may accumulate in renal impairment.
There are no dosage adjustments provided in the manufacturer's labeling; may require more frequent monitoring.
(For additional information see "Cysteine: Pediatric drug information")
Parenteral nutrition additive: Added as a fixed ratio to crystalline amino acid solution; dosage will vary with the daily amino acid dosage. Note: Correct severe fluid, electrolyte, and acid-base disorders prior to administration.
ASPEN Recommendations: Infants: IV: 30 to 40 mg of cysteine hydrochloride per gram of amino acids; a dose as low as 20 mg of cysteine hydrochloride per gram of amino acids may be adequate and should be used in times of cysteine shortage (Ref); current practice suggests supplementation should be continued through the first year of life; practice varies widely (Ref); consult institution-specific policies.
Product-specific dosing:
L-cysteine: Infants: IV: 40 mg cysteine hydrochloride per gram of amino acids.
Elcys:
Infants and Children ≤11 years: IV: 22 mg cysteine hydrochloride per gram of amino acids.
Children ≥12 years and Adolescents: IV: 7 mg cysteine per gram of amino acids.
There are no dosage adjustments provided in manufacturer's labeling; may require more frequent monitoring. Contains aluminum, which may accumulate in renal impairment.
There are no dosage adjustments provided in the manufacturer's labeling; may require more frequent monitoring.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified. Frequency not defined.
Cardiovascular: Flushing
Central nervous system: Localized warm feeling
Dermatologic: Localized erythema
Endocrine & metabolic: Hyperammonemia, metabolic acidosis
Gastrointestinal: Nausea
Local: Infusion site reaction, injection site phlebitis, venous thrombosis at injection site
Miscellaneous: Fever
Known hypersensitivity to one or more amino acids or any component of the formulation
L-cysteine (generic): Additional contraindications: Hepatic coma; metabolic disorders involving impaired nitrogen utilization
Elcys: Additional contraindications: Inborn errors of amino acid metabolism; pulmonary edema or acidosis due to low cardiac output
Concerns related to adverse effects:
• Hepatobiliary effects: Hepatobiliary disorders (eg, cholecystitis, cholelithiasis, cholestasis, cirrhosis, hepatic steatosis, fibrosis) may occur in patients without liver disease and may lead to hepatic failure. Increase in blood ammonia levels and hyperammonemia may also occur. Monitor liver function.
• Hyperammonemia: Asymptomatic hyperammonemia without overt liver dysfunction has been reported. Infants (birth to 2 years of age) are especially at risk. Monitor blood ammonia levels frequently in infants.
Disease-related concerns:
• Cardiac disease: Use with caution in patients with cardiac insufficiency.
• Diabetes: Hypertonic glucose solutions should be used with caution in patients with diabetes or prediabetic patients.
• Hepatic impairment: Use with caution in patients with hepatic impairment. Use in this patient population may result in hyperammonemia, metabolic alkalosis, prerenal azotemia, serum amino acid imbalances, stupor, and coma. In addition, IV parenteral nutrition in patients with impaired hepatic function may result in an increase in BUN (a modest rise is expected during increased protein intake).
• Renal impairment: Use with caution in patients with renal impairment; may require more frequent monitoring. IV parenteral nutrition in patients with impaired renal function will induce or augment an increase in BUN (a modest rise in BUN is to be expected during increased protein intake). Discontinue infusion, when clinically indicated, if BUN levels exceed normal postprandial limits and continue to rise.
• Pulmonary disease: Use with caution in patients with pulmonary disease.
Special populations:
• Infants: Metabolic acidosis has occurred in infants related to the additional acid provided by the "hydrochloride" component of cysteine; each 160 mg of L-cysteine delivers 1 mmol of hydrochloride; patients receiving the typical dose of L-cysteine of 40 mg for every gram of protein receive an additional 0.5 to 1 mEq/kg/day of chloride. To balance the extra hydrochloride ions and prevent acidosis, the use of acetate salts of electrolytes may be necessary (Cochran 1988; Heird 1988; Laine 1991; Plogsted 2015).
Dosage form specific issues:
• Aluminum: Contains aluminum; toxic aluminum concentrations may be seen with high doses, prolonged use, or renal impairment. Premature infants are at higher risk due to immature renal function and aluminum intake from other parenteral sources. Parenteral aluminum exposure of >4 to 5 mcg/kg/day is associated with CNS and bone toxicity; tissue loading may occur at lower doses. See manufacturer's labeling.
• Precipitates: Periodically inspect solution, infusion set and catheter for precipitates. Pulmonary vascular precipitates causing pulmonary vascular emboli and pulmonary distress has been reported (some fatal). If signs of pulmonary distress occur, stop the infusion.
Other warnings/precautions:
• Acetate: Use acetate-containing solutions with extreme caution in patients with respiratory or metabolic alkalosis and in conditions in which there is an increased level or an impaired utilization of acetate such as severe hepatic impairment.
• Administration: For central or peripheral IV administration; peripheral administration of nutrition is dependent upon osmolality of solution.
• Potassium: Use potassium-containing solutions with extreme caution, if at all, in patients with severe renal failure, hyperkalemia, and in conditions where potassium retention is present (eg, metabolic acidosis).
• Sodium: Use sodium-containing solutions with extreme caution, if at all, in patients with severe renal impairment, heart failure, and conditions in which edema exists with sodium retention.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution, Intravenous, as hydrochloride [preservative free]:
Elcys: 50 mg/mL (10 mL)
No
Solution (Elcys Intravenous)
50 mg/mL (per mL): $11.36
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
IV: Not for direct IV infusion; must be diluted and mixed in parenteral nutrition solutions. Administered as a component of peripheral or central parenteral nutrition. Central or peripheral administration of nutrition is dependent upon osmolality of the final solution; solutions with osmolarity ≥900 mOsm/L must be infused via central venous catheter. Ensure proper catheter/needle placement prior to and during infusion; avoid extravasation. May require use of inline filter (consult specific product labeling and/or individual institutional policies and procedures). Cysteine may be added to amino acid solution prior to further admixing with dextrose for parenteral nutrition. Refer to manufacturer's labeling for additional information.
Parenteral: IV infusion: Not for direct IV infusion; administer as a component of parenteral nutrition solution: Infuse parenteral nutrition solution at rate as directed. Ensure proper catheter/needle placement prior to and during infusion; avoid extravasation.
Component of parenteral nutrition: Additive to amino acid solutions to meet the nutritional requirements of the following patients requiring parenteral nutrition: Infants (L-cysteine [generic] and Elcys) or adult and pediatric patients with severe liver disease who may have impaired enzymatic processes (Elcys).
Cysteine is generally considered to be a nonessential amino acid in adults because it can be synthesized from methionine (an essential amino acid). The RDA for methionine + cysteine is increased in during pregnancy (IOM 2005).
Appropriate use as an additive to amino acid solutions in pregnant patients requiring parenteral nutrition is not expected to cause adverse maternal or fetal outcomes.
Cysteine is present in breast milk.
The recommended dietary allowance for methionine + cysteine is increased in lactating patients (IOM 2005). Appropriate use as an additive to amino acid solutions in pregnant patients requiring parenteral nutrition would not be expected to cause adverse effects to a breastfed infant. According to the manufacturer, the decision breastfeed during therapy should consider the risk of infant exposure, the benefits of breastfeeding to the infant, and benefits of treatment to the mother.
Fluid and electrolyte status, blood glucose, kidney and liver function, CBC and coagulation parameters throughout treatment, blood ammonia levels (if hepatic impairment present), triglycerides (if adding lipid emulsion), acid-base balance, serum proteins, blood culture, urine osmolarity and glucose, nitrogen intake; Monitor solution, infusion set, inline filter, and catheter for precipitates.
Endogenous cysteine is an amino acid synthesized from methionine via the transulfuration pathway, and serves as a precursor substrate for both glutathione and taurine. Exogenous cysteine provides cysteine to the systemic circulation of patients requiring parenteral nutrition who cannot synthesize adequate quantities due to deficient or insufficient cystathionase activity.
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