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November 1966, Vol 112, No. 5 >
Article | November 1966

Galactitol in the Tissues of a Galactosemic Child FREE

RONALD QUAN-MA, PhD; HENRY J. WELLS, PhD; WILLIAM W. WELLS, PhD; FRANK E. SHERMAN, MD; THOMAS J. EGAN, MD
[+] Author Affiliations

Received for publication March 25, 1966.

Reprint requests to University of Pittsburgh School of Medicine, Biochemistry Department, Pittsburgh 15213 (Dr. H. J. Wells).

The proteolipid and phosphatide-peptide-bound myo-inositol were not included in the analyses.

Concentration expressed as uM/ml.

— None detectable.


Am J Dis Child. 1966;112(5):477-478. doi:10.1001/archpedi.1966.02090140149018.
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Comments

GALACTITOL has recently been isolated from the urine1 and brain2 of galactosemia patients. Subsequently the conversion of orally administered galactose- 1-C14 into galactitol-1-C14 by a 17-year-old male galactosemia patient was demonstrated.3 In order to account for the relatively slow excretion of administered radioactivity as galactitol-C14 it was suggested that there had either been deposition of galactitol in tissues of the body, or that the administered galactose-C14 had been incorporated into galactose-containing compounds. This hypothesis was further supported by the finding of galactitol in a variety of tissues in a rat fed large amounts of galactose.4 We now wish to report the galactitol, glucose, free, and phosphatide-bound myo-inositol content of body tissue from 17 areas of an infant presumed to be galactosemic.

Material and Methods  The patient studied was a 23-day-old male infant who was jaundiced on the third day of life.

REFERENCES

1 +
Wells, W.W.; Pittman, T.A.; and Egan, T.J.:  The Isolation and Identification of Galactitol From the Urine of Patients With Galactosemia , J Biol Chem 239:3192, 1964;.
2 +
Wells, W.W., et al:  The Isolation and Identification of Galactitol From the Brains of Galactosemia Patients , J Biol Chem 240:1002, 1965;.
3 +
Egan, T.J., and Wells, W.W.:  Alternate Metabolic Pathway in Galactosemia: Observations in a Patient With Galactosemia , Amer J Dis Child 111: 400, 1966;.
4 +
Quan-Ma, R., and Wells, W.W.:  The Distribution of Galactitol in Tissues of Rats Fed Galactose , Biochem Biophys Res Commun 20:486, 1965;.
5 +
Folch, J.; Lees, M.; and Sloane-Stanley, G.H.:  A Simple Method for the Isolation and Purification of Total Lipides From Animal Tissues , J Biol Chem 226:497, 1957;.
6 +
Wells, W.W.; Chin, T.; and Weber, B.:  Quantitative Analysis of Serum and Urine Sugars by Gas Chromatography , Clin Chim Acta 10:352, 1964;.
7 +
Wells, W.W.; Pittman, T.A.; and Wells, H.J.:  Quantitative Analysis of Myoinositol in Rat Tissue by Gas-Liquid Chromatography , Anal Biochem 10: 450, 1965;.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

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References

1 +
Wells, W.W.; Pittman, T.A.; and Egan, T.J.:  The Isolation and Identification of Galactitol From the Urine of Patients With Galactosemia , J Biol Chem 239:3192, 1964;.
2 +
Wells, W.W., et al:  The Isolation and Identification of Galactitol From the Brains of Galactosemia Patients , J Biol Chem 240:1002, 1965;.
3 +
Egan, T.J., and Wells, W.W.:  Alternate Metabolic Pathway in Galactosemia: Observations in a Patient With Galactosemia , Amer J Dis Child 111: 400, 1966;.
4 +
Quan-Ma, R., and Wells, W.W.:  The Distribution of Galactitol in Tissues of Rats Fed Galactose , Biochem Biophys Res Commun 20:486, 1965;.
5 +
Folch, J.; Lees, M.; and Sloane-Stanley, G.H.:  A Simple Method for the Isolation and Purification of Total Lipides From Animal Tissues , J Biol Chem 226:497, 1957;.
6 +
Wells, W.W.; Chin, T.; and Weber, B.:  Quantitative Analysis of Serum and Urine Sugars by Gas Chromatography , Clin Chim Acta 10:352, 1964;.
7 +
Wells, W.W.; Pittman, T.A.; and Wells, H.J.:  Quantitative Analysis of Myoinositol in Rat Tissue by Gas-Liquid Chromatography , Anal Biochem 10: 450, 1965;.

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