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Biotinidase Deficiency
(Multiple Carboxylase Deficiency, Late Onset)
Summary
Description:
The late onset form of MULTIPLE CARBOXYLASE DEFICIENCY (deficiency of the activities of biotin-dependent enzymes propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, and PYRUVATE CARBOXYLASE) due to a defect or deficiency in biotinidase which is essential for recycling BIOTIN.
Also Known As:
Multiple Carboxylase Deficiency, Late Onset; Carboxylase Deficiency, Multiple, Late-Onset; Deficiency, Biotinidase; Deficiency, Multiple Carboxylase, Late-Onset; Biotinidase Deficiencies Show All >>
Networked: 173
relevant articles (7 outcomes,
6 trials/studies)
for this Disease
Key Drugs and Agents for Biotinidase Deficiency
Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
-
Biotin (Vitamin H)
:
7 outcomes 2 studies in 103 results
:
FDA 13
-
Vitamins
:
1 outcome in 11 results
: IBA
-
Biotinidase
:
3 studies in 45 results
: IBA
-
biocytin
:
1 study in 11 results
: IBA
-
Valproic Acid (Valproate, Semisodium)
:
1 study in 3 results
:
FDA 51
Generic
-
Transaminases
:
1 study in 1 result
: IBA
-
Adenosine Monophosphate (AMP)
:
1 study in 1 result
: IBA
-
Late-onset multiple carboxylase deficiency
:
14 results
: IBA
-
Ligases
:
9 results
: IBA
-
DNA (Deoxyribonucleic Acid)
:
6 results
: IBA
Show All >>
Diseases Related to Biotinidase Deficiency
-
Exanthema (Rash)
-
Ataxia (Dyssynergia)
-
Muscle Hypotonia (Hypotonia)
-
Seizures (Seizure)
-
Alopecia (Baldness)
Show All >>
Key Therapies for Biotinidase Deficiency
Efficacy Chart >>
-
Erythrocyte Transfusion
:
1 result
-
Anesthesia
:
1 result
-
Aftercare (After-Treatment)
:
1 result
-
Oral Administration
:
1 result
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