NATURE'S WAY OSTEOPRIME CAPSULES

Indications

• Homocystinuria
• Type I Gaucher's disease (specialist use only)

Dosage

Children: Refer to BNF for Children for specific dosing guidelines.

Adults: Refer to BNF for specific dosing guidelines.

Mechanism of action

Betaine acts as a methyl donor in the transsulfuration pathway, facilitating the conversion of homocysteine to methionine and thereby reducing homocysteine levels.

Pharmacodynamics

Betaine reduces plasma homocysteine concentrations, which is beneficial in conditions such as homocystinuria. It also has a role in osmoregulation and may influence cellular metabolism.

Pharmacokinetics

Betaine is well absorbed after oral administration, with a bioavailability of approximately 90%. It is primarily excreted in the urine as betaine and its metabolites.

Adverse effects

• abdominal discomfort
• agitation
• alopecia
• appetite decreased
• brain oedema
• diarrhoea
• glossitis
• irritability
• nausea
• skin reactions
• urinary incontinence
• vomiting
• arthralgia
• asthenia
• chest discomfort
• dizziness
• dyspnoea
• fever
• flushing
• gastrointestinal discomfort
• headache
• hypersensitivity
• hypertension
• hypotension
• infusion-related reactions
• nausea
• pain
• skin reactions
• tachycardia

Interactions

• may be given with supplements of Vitamin B12, pyridoxine, and folate under specialist advice

Precautions

• use in conjunction with dietary restrictions
• monitor plasma-methionine concentration before and during treatment
• interrupt treatment if symptoms of cerebral oedema occur
• use with caution in pregnancy due to limited information
• use with caution in breastfeeding due to lack of information

Pregnancy

Manufacturer advises avoid unless essential-limited information available.

Breast-feeding

Manufacturer advises caution-no information available.

Storage

Store in a cool, dry place; protect from light.

Formulations

• Cystadane oral powder 1 gram per 1 gram

Indications

• Osteoporosis
• Arthritis
• Inflammatory conditions
• Bone health enhancement

Dosage

Children: Refer to BNF for Children for specific dosing information.

Adults: Refer to BNF for specific dosing information.

Mechanism of action

Boron is thought to influence the metabolism of steroid hormones, particularly estrogen and testosterone. It may enhance the absorption and utilization of calcium and magnesium, contributing to improved bone density and health. Additionally, boron may help in reducing inflammation and modulating immune responses, potentially benefiting conditions characterized by joint pain and inflammation.

Pharmacodynamics

Boron exhibits a variety of effects on cellular processes, including the modulation of cell signaling pathways involved in bone metabolism and inflammation. It has been shown to influence the activity of osteoblasts and osteoclasts, cells responsible for bone formation and resorption, respectively. This modulation can lead to increased bone density and improved mineralization.

Pharmacokinetics

Boron is absorbed through the gastrointestinal tract, and its bioavailability can vary depending on the source and form of boron consumed. Once absorbed, boron is distributed throughout the body, with a higher concentration in bones and teeth. It is primarily excreted through urine, and its elimination half-life is not well defined but is thought to be relatively short. There is limited data on the pharmacokinetics of boron in humans.

Pregnancy

Boron is generally regarded as safe in dietary amounts during pregnancy, but high doses should be avoided as they may be harmful.

Breast-feeding

Boron is excreted in breast milk, and while typical dietary amounts are considered safe, high doses should be avoided.

Storage

Store in a cool, dry place away from direct sunlight.

Indications

• Molybdenum deficiency
• Support for enzymatic functions related to sulfur metabolism
• Potential use in metabolic disorders associated with sulfite toxicity

Dosage

Children: Refer to specific guidelines for dosing, as molybdenum is typically obtained from dietary sources and supplementation is uncommon.

Adults: Refer to specific guidelines for dosing, as molybdenum is typically obtained from dietary sources and supplementation is uncommon.

Mechanism of action

Molybdenum serves as a cofactor for several important enzymes, including sulfite oxidase, which converts sulfite to sulfate, and xanthine oxidase, which is involved in purine metabolism. These enzymatic reactions are crucial for the detoxification of sulfites and the metabolism of nitrogenous compounds.

Pharmacodynamics

Molybdenum is vital for the activity of enzymes that contribute to the metabolism of sulfur-containing amino acids, purine degradation, and the detoxification of harmful compounds. Its deficiency can lead to metabolic disorders, highlighting its importance in enzymatic processes within the body.

Pharmacokinetics

Molybdenum is absorbed mainly in the small intestine, and its absorption may be influenced by dietary factors. Once absorbed, it is distributed throughout the body and is predominantly found in the liver, kidneys, and bones. The half-life of molybdenum and its excretion primarily through the urine is not well characterized, but excess amounts are generally eliminated by the body.

Pregnancy

Molybdenum is an essential trace element required for normal human metabolism. Adequate intake during pregnancy is important, but excessive amounts may be harmful. Consultation with a healthcare provider is advised.

Breast-feeding

Molybdenum is secreted in breast milk, and adequate intake is important for the nursing infant. Mothers should ensure they meet recommended dietary allowances.

Storage

Store in a cool, dry place, away from direct sunlight and moisture. Keep out of reach of children.

Indications

• Potential role in bone health
• Support for connective tissue formation
• May aid in mineral metabolism

Dosage

Children: There is no established clinical dosage for silicon in paediatric populations, as it is not classified as an essential nutrient.

Adults: There is no established clinical dosage for silicon in adults, as it is not classified as an essential nutrient.

Mechanism of action

Silicon is believed to enhance the synthesis of glycosaminoglycans and collagen, which are important for the structural integrity of connective tissues. It may also influence the activity of certain enzymes involved in bone mineralization, thus playing a role in maintaining bone density and health.

Pharmacodynamics

The pharmacodynamics of silicon is not fully elucidated; however, it is thought to involve the modulation of bone metabolism and the promotion of connective tissue health. Silicon may have a synergistic effect with other minerals, such as calcium and magnesium, aiding in their utilization and metabolism in the body.

Pharmacokinetics

The pharmacokinetics of silicon is complex, as it is not absorbed through typical gastrointestinal pathways. Instead, silicon is thought to be taken up in the form of silicates and then distributed throughout the body, particularly in connective tissues. The elimination of silicon occurs primarily through renal excretion, with some variations depending on dietary intake and individual metabolism.

Pregnancy

Silicon is generally considered safe during pregnancy, as it is a naturally occurring element in the human body. However, specific recommendations regarding supplementation should be followed based on the advice of a healthcare provider.

Breast-feeding

Silicon is present in breast milk in small amounts. Its safety during breastfeeding is generally regarded as acceptable, although supplementation should be approached with caution and under medical advice.

Storage

Silicon should be stored in a cool, dry place, protected from light and moisture. Follow specific storage recommendations provided by the manufacturer if available.

Indications

• Osteoporosis in postmenopausal women
• Osteoporosis in men at increased risk of fracture
• Adjunctive treatment in certain bone-related disorders

Dosage

Children: Not established, refer to appropriate clinical guidelines for treatment of bone disorders in children.

Adults: Refer to prescribing information for specific dosing guidelines, as they may vary based on the formulation and condition being treated.

Mechanism of action

Strontium ranelate is thought to act through a dual mechanism of action. First, it stimulates the proliferation and activity of osteoblasts, which are cells responsible for bone formation. Second, it inhibits the formation and activity of osteoclasts, the cells that break down bone tissue. This leads to an overall increase in bone mass and a reduction in the risk of bone fractures. Strontium also influences calcium metabolism and may enhance the mineralization of bone.

Pharmacodynamics

Strontium has been shown to improve bone strength and density, reducing the incidence of vertebral and non-vertebral fractures in patients with osteoporosis. Its pharmacodynamic properties include an increase in bone formation and a decrease in bone resorption, which together help maintain a healthy balance in bone remodeling. Strontium's effects on the skeletal system are complemented by its ability to modulate other cellular processes related to bone health.

Pharmacokinetics

After oral administration, strontium is absorbed in the gastrointestinal tract, with its bioavailability influenced by dietary calcium. The peak plasma concentration occurs within a few hours of administration. Strontium is primarily distributed in bone and has a long biological half-life due to its incorporation into the mineral phase of bones. It is eliminated through renal clearance, with a significant fraction of the absorbed dose being retained in the body.

Adverse effects

• Nausea
• Vomiting
• Diarrhea
• Fatigue
• Headache
• Dizziness
• Skin rash
• Abdominal pain

Precautions

• Use with caution in patients with renal impairment
• Monitor for signs of gastrointestinal upset
• Assess bone health before initiating treatment

Pregnancy

Strontium should be used during pregnancy only if clearly needed. Animal studies have shown adverse effects, and there are no well-controlled studies in pregnant women.

Breast-feeding

It is unknown if strontium is excreted in human milk. Caution should be exercised when administering to breastfeeding women.

Storage

Store in a cool, dry place away from light. Keep out of reach of children.

Formulations

• Strontium ranelate oral powder
• Strontium ranelate tablets

Mechanism of action

Homocystinuria is a hereditary disorder characterized by high levels of the amino acid homocysteine. This condition can be caused by deficiencies or defects in cystathionine beta-synthase (CBS), 5,10-methylenetetrahydrofolate reductase (MTHFR), and cobalamin cofactor metabolism (cbl). CBS converts homocysteine to cystathionine, and a deficiency in this enzyme can lead to an accumulation of homocysteine. MTHFR is responsible for producing 5-methyltetrahydrofolate, a methyl donor that participates in the conversion of homocysteine back to methionine; therefore, an MTHFR deficiency can also lead to homocystinuria. Additionally, a defect in cobalamin (vitamin B12) cofactor metabolism can lead to homocystinuria, since a metabolite of cobalamin (methylcobalamin) promotes the conversion of homocysteine to methionine. Betaine transfers a methyl group via the enzyme betaine homocysteine methyl transferase (BHMT), converting homocysteine back into methionine and dimethylglycine (DMG). In patients with homocystinuria, betaine reduces homocysteine levels and improves health outcomes. Betaine acts as metabolic intermediate in transmethylating processes (creatine and methionine synthesis). Betaine acts as a methyl group donor in the remethylation of homocysteine to methionine in patients with homocystinuria. This reduces toxic concentrations of homocysteine, usually to 20 to 30% or less of pretreatment concentrations. Betaine or trimethylglycine is a quarternary ammonium compound that was first discovered in the juice of sugar beets (Beta vulgaris). Betaine is a metabolite of choline ... and is a substrate in one of the two recycling pathways that convert homocysteine to L-methionine. The other and principal recycling reaction is catalyzed by the enzyme methionine synthase and uses methylcobalamin as a cofactor and 5-methyltetrahydrofolate as a cosubstrate. Betaine-homocysteine methyltransferase (BHMT) is a zinc metalloenzyme which catalyzes the transfer of a methyl group from betaine to homocysteine in the formation of methionine. BHMT is found in the liver and kidneys and may also exist in brain tissue. Betaine acts to lower homocysteine levels in some with primary hyperhomocysteinemia/homocystinuria via this enzyme.

Pharmacodynamics

Betaine decreases plasma homocysteine concentrations in homocystinuria cases caused by deficiencies or defects in cystathionine beta-synthase (CBS), 5,10-methylenetetrahydrofolate reductase (MTHFR), and cobalamin cofactor metabolism (cbl). The decrease of homocysteine is estimated to be 20-30% of pre-treatment levels. Betaine supplementation in patients with homocystinuria also improves metabolic abnormalities in cerebrospinal fluid. Reports have shown that depending on the type of homocystinuria, the therapeutic effectiveness of betaine alone may be limited, insufficient to decrease total homocysteine levels and prevent clinical symptoms. In patients with homocystinuria due to cystathionine beta-synthase (CBS) deficiency, betaine should be used when serum total homocysteine levels remain high despite dietary therapy. Patients taking betaine for several years do not show evidence of tolerance. Also, betaine concentrations are not correlated with homocysteine concentrations. In patients with MTHFR deficiency and cbl defects, betaine may increase methionine and S-adenosyl methionine (SAM) plasma levels. Patients with CBS deficiency without a dietary restriction of methionine may accumulate excessive amounts of methionine. Clinical data shows that in patients with CBS deficiency, increased plasma methionine levels were associated with cerebral edema.