Mayo Test Catalog, (Sorted By Test Name) - Mayo Medical ...

ALLOI
88888
14-15 years: 2.2-32.1 IU/L
16-18 years: 1.4-19.7 IU/L
> or =19 years: 0.0-5.8 IU/L
Bone %
0-6 years: 41.5-82.7%
7-9 years: 39.9-85.8%
10-13 years: 31.8-91.1%
14-15 years: 30.6-85.4%
16-18 years: 38.9-72.6%
> or =19 years: 19.1-67.7%
Bone
0-6 years: 43.5-208.1 IU/L
7-9 years: 41.0-258.3 IU/L
10-13 years: 39.4-346.1 IU/L
14-15 years: 36.4-320.5 IU/L
16-18 years: 32.7-214.6 IU/L
> or =19 years: 12.1-42.7 IU/L
Intestine %
0-6 years: 0.0-18.4%
7-9 years: 0.0-18.3%
10-13 years: 0.0-11.8%
14-15 years: 0.0-8.2%
16-18 years: 0.0-8.7%
> or =19 years: 0.0-20.6%
Intestine
0-6 years: 0.0-37.7 IU/L
7-9 years: 0.0-45.6 IU/L
10-13 years: 0.0-40.0 IU/L
14-15 years: 0.0-26.4 IU/L
16-18 years: 0.0-12.7 IU/L
> or =19 years: 0.0-11.0 IU/L
Placental
Not present
Clinical References: 1. Tietz Textbook of Clinical Chemistry, Edited by CA Burtis, ER Ashwood.
Philadelphia, WB Saunders Company, 1999 2. Moss DW: Alkaline phosphatase isoenzymes. Clin
Chem 1982;28:2007-2016
Allo-isoleucine, Blood Spot
Clinical Information: Maple-syrup urine disease (MSUD) is an autosomal recessive deficiency of
the branched-chain-ketoacid dehydrogenase (BCKDH) complex. The BCKDH complex is involved in
the metabolism of the branched-chain amino acids (BCAA): isoleucine (Ile), leucine (Leu), and valine
(Val). Classic MSUD presents in the neonate with feeding intolerance, failure to thrive, vomiting,
lethargy, and maple-syrup odor to urine and cerumen. If untreated, it progresses to irreversible mental
retardation, hyperactivity, failure to thrive, seizures, coma, cerebral edema, and possibly death. MSUD
is a panethnic condition, but is most prevalent in the Old Order Mennonite community in Lancaster,
Pennsylvania. The incidence of MSUD is approximately 1:200,000 live births in the general population
and 1:760 live births among the Old Order Mennonites. Newborn screening includes the measurement
of BCAA (Leu, Ile, and Val), which are elevated in MSUD. However, unaffected infants receiving total
parenteral nutrition frequently have increased levels of BCAA, a situation that often triggers
unnecessary follow-up investigations. Abnormal concentrations of allo-isoleucine (Allo-Ile) are
pathognomonic for MSUD. The determination of Allo-Ile (second-tier testing) in the same newborn
screening specimens that reveals elevated BCAA allows for positive identification of patients with
MSUD and differentiation from BCAA elevations due to dietary artefacts, reducing the occurrence of
false-positive newborn screening results. Treatment of MSUD aims to normalize the concentration of
BCAA by dietary restriction of these amino acids. Because BCAA belong to the essential amino acids,
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