msud maxamum

Action and Spectrum

Actions and Spectrum: 

msud maxamum is a valine, leucine, and isoleucine-free powdered medical food designed for the dietary management of maple syrup urine disease in individuals aged nine years and older. It provides a balanced mixture of essential and non-essential amino acids, carbohydrates, vitamins, and minerals while excluding the specific amino acids individuals with MSUD cannot metabolize.

This specialized formulation helps maintain metabolic balance and prevents the accumulation of harmful substances associated with MSUD, ensuring proper nutritional support for those affected. The spectrum of msud maxamum’s action encompasses providing vital nutrients and managing metabolic imbalances related to MSUD, contributing to improved clinical outcomes and the overall well-being of individuals with the disease. 

Drug Interaction

Adverse Reaction

Frequency not defined 

Drowsiness 

Vomiting 

Nausea  

Black Box Warning

Black Box Warning: 

None 

Contraindication / Caution

Contraindication/Caution: 

Contraindication 

None 

Caution 

For individuals with MSUD, following a carefully controlled diet restricting certain amino acids and working closely with healthcare professionals specializing in metabolic disorders is essential. This helps avert the buildup of harmful substances in the body and manage the condition effectively. 

Pregnancy / Lactation

Pregnancy consideration:  

US FDA pregnancy category: Not assigned. 

Lactation:   

Excreted into human milk: Not known. 

Pregnancy category: 

• Category A: well-controlled and Satisfactory studies show no risk to the fetus in the first or later trimester. 
• Category B: there was no evidence of risk to the fetus in animal studies, and there were not enough studies on pregnant women. 
• Category C: there was evidence of risk of adverse effects in animal reproduction studies, and no adequate evidence in human studies must take care of potential risks in pregnant women.    
• Category D: adequate data with sufficient evidence of human fetal risk from various platforms, but despite the potential risk, and used only in emergency cases for potential benefits.    
• Category X: Drugs listed in this category outweigh the risks over benefits. Hence these categories of drugs need to be avoided by pregnant women.    
• Category N: There is no data available for the drug under this category. 

Pharmacology

Pharmacology: 

msud maxamum is a valine, leucine, and isoleucine-free powdered formula designed for the dietary management of maple syrup urine disease (MSUD), a metabolic disorder. It provides a balanced mixture of essential and non-essential amino acids, carbohydrates, vitamins, and minerals while excluding the specific amino acids individuals with MSUD cannot metabolize.

By restricting the intake of these amino acids, msud maxamum aims to prevent the accumulation of harmful substances in the body and support proper nutrition in individuals with MSUD. This specialized formulation helps maintain metabolic balance and minimize the risk of adverse clinical outcomes associated with MSU. 

Pharmacodynamics: 

Mechanism of action: msud maxamum’s mechanism of action involves providing essential and non-essential amino acids, carbohydrates, vitamins, and minerals to individuals with MSUD while excluding the amino acids they cannot metabolize. This tailored nutritional composition helps maintain metabolic balance, prevents the accumulation of harmful substances associated with MSUD, and supports proper nutrition. 

Pharmacokinetics: 

Absorption 

Absorption refers to the uptake of nutrients from the gastrointestinal tract. 

Distribution 

Distribution involves the transport of these nutrients throughout the body 

Metabolism 

Metabolism pertains to the biochemical transformation of nutrients 

Elimination and Excretion 

Excretion is generally removal of waste products from the body 

Adminstartion

Administration: 

msud maxamum is administered as a valine, leucine, and isoleucine-free powdered formula for dietary management of maple syrup urine disease in individuals over nine years and older. It can be used as part of the treatment plan for individuals with MSUD, providing a balanced mixture of essential and also non-essential amino acids, carbohydrates, vitamins, and minerals. BCAA-free formula supplementation is used to reduce plasma BCAA levels within reference ranges for metabolic decompensations or emergencies.

In emergencies, administering emergency feeds, such as nasogastric or gastrostomy tube feeding, may be necessary to provide the necessary nutrition while avoiding the intake of specific amino acids that individuals with MSUD cannot metabolize. The administration of msud maxamum aims to support metabolic balance and prevent harmful substance accumulation associated with MSUD. 

Patient Information Leaflet

Patient information leaflet 

Generic Name: msud maxamum 

Why do we use msud maxamum? 

msud maxamum is a valine, leucine, and isoleucine-free powdered formula used for the dietary management of maple syrup urine disease (MSUD) in older children, teenagers, and adults. It’s designed for individuals with proven MSUD, including pregnant women. MSUD is a metabolic disorder that requires strict dietary restrictions on specific amino acids.

msud maxamum provides essential and non-essential amino acids, carbohydrates, vitamins, and minerals while omitting the specific amino acids that individuals with MSUD cannot metabolize. It is an essential medical food that helps prevent the accumulation of the harmful substances in the body and supports proper nutrition in those with MSUD. Please consult a healthcare professional before using msud maxamum, especially in cases of pregnancy or when considering interactions with other medications.