Lifestyle Untreated individuals with DβH<unk>  should avoid hot environments, strenuous exercise, standing still for prolonged time and dehydration. Care should be taken when changing position from horizontal to upright. Medications Since the conversion of dihydroxyphenylserine (Droxidopa; trade name: Northera; also known as L-DOPS, L-threo-dihydroxyphenylserine, L-threo-DOPS and SM-5688), to norepinephrine bypasses the<unk>  beta-hydroxylation step of catecholamine synthesis, L-Threo-DOPS is the ideal therapeutic agent. In humans with DβH<unk> , L-Threo-DOPS, a synthetic precursor of noradrenaline, administration has proven effective in dramatic increase of blood pressure and subsequent relief of postural symptoms. L-DOPS continues to be studied pharmacologically and pharmacokinetically and shows an ability to increase the levels of central nervous system norepinephrine by a significant amount. This is despite the fact that L-DOPS has a relative difficulty crossing the blood-brain barrier when compared to other medications such as L-DOPA. When used concurrently, there is evidence to show that there is increased efficacy as they are both intimately involved and connected to the pathway in becoming norepinephrine. There is hope and evidence that L-DOPS can be used much more widely to help other conditions or symptoms such as pain, chronic stroke symptoms, and progressive supranuclear palsy, amongst others. Clinically, L-DOPS has been already shown to be helpful in treating a variety of other conditions related to hypotension including the following: * Diabetes induced orthostatic hypotension * Dialysis-induced hypotension * Orthostatic intolerance * Familial amyloidotic polyneuropathy * Spinal Cord Injury related hypotension Empirical evidence of mild effectiveness has been reported using mineralocorticoids or adrenergic receptor agonists as therapies. Other medications that can bring relief to symptoms include: * phenylpropanolamine- due to pressor response to vascular α-adrenoceptors * indomethacin Vitamin C (ascorbic acid) is also a required cofactor for the<unk>  beta<unk>  enzyme. Recent research has shown that vitamin C rapidly catalyzes the conversion of<unk>  to norepinephrine through stimulation of the<unk>  beta<unk>  enzyme.
Dopamine beta hydroxylase deficiency
Aromatic L-amino acid decarboxylase deficiency
Dopamine transporter deficiency syndrome
Cerebral creatine deficiency
N-Acetylglutamate synthase deficiency
Prolidase deficiency
Beta-ketothiolase deficiency
Pantothenate kinase-associated neurodegeneration