Dermatan sulfate and heparan sulfate and keratan sulfate panel - Serum or Plasma   93726-8

LOINC Code


LOINC code93726-8
nameDermatan sulfate and heparan sulfate and keratan sulfate panel - Serum or Plasma
descriptionMucopolysaccharidoses (MPSs) are rare genetic disorders of glycosaminoglycan (GAG) catabolism caused by a deficiency in the activity of a single, specific lysosomal enzyme required for GAG degradation. GAGs include dermatan sulfate, heparan sulfate, keratan sulfate, and chondroitin sulfate. MPS patients have elevated GAG fragments in blood, urine and cerebral spinal fluid (CSF) as well as a buildup of incompletely degraded GAG in lysomes. The GAG accumulation interferes with normal cell, tissue and organ function, which may result in cardiac abnormalities, organ failure, cognitive impairment, and skeletal or joint abnormalities. There are nine primary MPS disorders, MPS I - MPS IX. Each type is associated with a specific enzyme deficiency and gene locus. Measuring GAG levels in urine can screen for MPS disorders, though false-negatives are common. To distinguish specific MPS disorders, enzyme activity assays based on cultured fibroblasts, leucocytes, plasma or serum are utilized. Predicting MPS disease severity is difficult for most types, however, patients who are homozygous for a nonsense allele or have two different nonsense alleles have the severe form of MPS I. [PMID: 22210669]
statusACTIVE

Fully-Specified Name

componentDermatan sulfate & heparan sulfate & keratan sulfate panel
property-
timePt  =  Point in time:  To identify measures at a point in time. This is a synonym for “spot” or “random” as applied to urine measurements.
systemSer/Plas
    Ser  =  Serum
    Plas  =  Plasma
scale-
method

Additional Names

short nameMPS panel SerPl

Basic Attributes

classPANEL.CHEM
type1  Laboratory
order vs. observationOrder

Panel Components

2203-8Dermatan sulfate [Mass/volume] in Serum or Plasma
93725-0Heparan sulfate [Mass/volume] in Serum or Plasma
93724-3Keratan sulfate [Mass/volume] in Serum or Plasma

Associated Observations

LOINC codes that represent optional associated observation(s) for a clinical observation or laboratory test. A LOINC term may represent a single associated observation or panel containing several associated observations.

History/Usage

first released
last updated2.67
last change typeADD  - added

Related Names

Chemistry
Chondroitin B sulfate
Chondroitin sulfate B
Dermatin
HS
keratosulfate
MPS panel
Mucopolysaccharides
Mucopolysaccharidosis
Pan
PANEL.CHEMISTRY
Panl
Pl
Plasma
Plsm
Pnl
Point in time
Random
SerP
SerPl
SerPlas
Serum
Serum or plasma
SO4
SO4-2
SR
Sulf

Copyright © 2024 Regenstrief Institute, Inc. All Rights Reserved. To the extent included herein, the LOINC table and LOINC codes are copyright © 1995-2024, Regenstrief Institute, Inc. and the Logical Observation Identifiers Names and Codes (LOINC) Committee. See https://loinc.org/license for the full LOINC copyright and license.

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