92757-4
Cholestane-3-beta, 5-alpha, 6-beta triol [Moles/volume] in DBS
Active
Part Description
LP310337-3 Cholestane-3-beta, 5-alpha, 6-beta triol
The oxidation products of cholesterol, particularly cholestane-3beta,5alpha,6beta-triol and 7-ketocholesterol, have proven to be sensitive and specific biomarkers for early detection of Niemann Pick type C (NPC) disease. NPC disease is a rare genetic disorder that results in the accumulation of cholesterol and other lipids internally. Patients often present in childhoold with hepatospenomegaly and central nervous system degeneration, although the presentation is broad and adult-onset psychiatric or visceral disease can occur. Mutations in the NPC1 or NPC2 gene are responsible, with the vast majority ocurring in NPC1. Prior to cholestane-3beta,5alpha,6beta-triol and 7-ketocholesterol, filipin staining of skin biopsy material was the assay of choice, although it was cumbersome, insensitive, and not widely avaialble PMID: 21518695.
Source: Regenstrief LOINC
Fully-Specified Name
- Component
- Cholestane-3-beta, 5-alpha, 6-beta triol
- Property
- SCnc
- Time
- Pt
- System
- Bld.dot
- Scale
- Qn
- Method
Additional Names
- Short Name
- Chol-3b,5a,6b-triol DBS-sCnc
- Display Name
- Cholestane-3-beta, 5-alpha, 6-beta triol (DBS) [Moles/Vol]
- Consumer Name Alpha Get Info
- Cholestane-3-beta, 5-alpha, 6-beta triol, Dried blood spot
Basic Attributes
- Class
- CHEM
- Type
- Laboratory
- First Released
- Version 2.66
- Last Updated
- Version 2.66
- Order vs. Observation
- Observation
Member of these Panels
LOINC | Long Common Name |
---|---|
92741-8 | Cholestane-3-beta, 5-alpha, 6-beta triol and Lyso-sphingomyelin panel - DBS |
92745-9 | Hepatosplenomegaly due to lysosomal storage disorders screening panel - DBS |
Language Variants Get Info
Tag | Language | Translation |
---|---|---|
es-ES | Spanish (Spain) | Colestano-3-beta, 5-alfa, 6-beta triol: Synonyms: Cuantitativo |
es-MX | Spanish (Mexico) | Colestano-3-beta, 5-alfa, 6-beta triol: |
fr-FR | French (France) | Choléstane-3-bêta, 5-alpha, 6-bêta triol: |
it-IT | Italian (Italy) | Colestano-3-beta, 5-alfa, 6-beta triol: Synonyms: Chimica Concentrazione di Sostanza Punto nel tempo (episodio) Sangue Spot sangue secco |
zh-CN | Chinese (China) | 胆甾烷-3-beta, 5-alpha, 6-beta 三醇: Synonyms: Ⅲ B III α β 三 克分子浓度; |
Example Units
Unit | Source |
---|---|
nmol/mL | Example UCUM Units |
LOINC Terminology Service (API) using HL7® FHIR® Get Info
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- CodeSystem lookup
- https:
//fhir.loinc.org/CodeSystem/$lookup?system=http: //loinc.org&code=92757-4
LOINC Copyright
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