We have demonstrated growth retardation in a child with hereditary fructose intolerance (HFI) at levels of fructose ingestion that did not produce acute symptoms. Highly significant growth was achieved by fructose restriction. A protocol is presented to evaluate the possible role of disturbed adenine nucleotide metabolism and energy dissipation in abnormal growth in children with HFI. Evidence is presented suggesting that, in patients with HFI, fructose exposure induces dysfunction detectable by increased rates of lysosomal residual body excretion in the urine. If found, this dysfunction offers the possibility of heterozygote identification and the possibility of elucidation of pathogenetic mechanisms relevant to a broad range of metabolic diseases.

Agency
National Institute of Health (NIH)
Institute
National Center for Research Resources (NCRR)
Type
General Clinical Research Centers Program (M01)
Project #
2M01RR000079-25
Application #
3089650
Study Section
General Clinical Research Centers Committee (CLR)
Project Start
1974-12-01
Project End
1991-11-30
Budget Start
1986-12-01
Budget End
1987-11-30
Support Year
25
Fiscal Year
1987
Total Cost
Indirect Cost
Name
University of California San Francisco
Department
Type
Schools of Medicine
DUNS #
073133571
City
San Francisco
State
CA
Country
United States
Zip Code
94143
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