Cancer

THE

AMERICAN

JOURNAL

OF

CLINICAL

NUTRITION

Vol.

21,

No.

6,

June,

1968,

pp.

693-698

Printed

in

U.S.A.

Comments

in

Biochemistry

Iv.

Fructose
Enzyme Intolerance,
H.

Metabolism
Essential Glycogen-Storage Fructosuria, Disease’ Fructose

Deficiencies: and

ROBERT

HERMAN,

M.D.,

AND

DAVID

ZAKIM,

M.D.

E
liverSSENTIAI.

FRUCTOSURIA

is of

due

to

a

de- D-giyceraldeimyde

ant! (10-17). in is (12). It one present Dietary 20) inhibitor 21, 22) and

dihydroxyacetone has of been the in fructose te, is of Time sweating, various and confusion, cyanosis, and, fructose Repeated amino cirrhoretardation wimicim tlmougimt various in hypogiycesymptoms vonmi wlmiclm, imypotenin ting two suggestedhepatic inis acto enact con-

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ficielmcy
tablisimeti to by time and is Time sugar test 100 ml 10-20% in only values by take time urine time

of
the up

imepatic
failure

fructokinase,
lmomogenates (1, 2).

as
Die-

esi)im0splmtte
timat of
altiolases

a

defect

U-14C-fructose

( 1 8)
to (19, competitive (13,(14-16,

fructose

tar)
lized
ilm Imign

fructose

is
liver (3,

absorbed
and 4). Time

but
consequently condition witim despite

not
appears

metabo- tolerance
verteti is becumulates a zymes nmia for toler- of of to are 25 if noris i nfa trenm time profotllmd, conma, ncy, prottuced test may atmd dea fruc- as fructest

fructose-1-pimospha

individual urine (3, or 4). bloodImigimer gives An as

essential time positive fructose values

tosuria tosuria. reducnmg ance ing/ mal About excreted wlmeieas indivitiuals fructose pyruvate jects fructosuria to fructose, fructose Fructose to tosuria Fructose deficieimcy lase
1

asymptomatic

resulting

a oral fructose

23-26). result can of

ulousness,

gives

time cause

hypoglycenmia

compared

of15-25
of the 1-2% time urine is After

mg/100
administered in excreted oral blood or

ml
tiose time by lactate

(4-7).sion,
patients be normal aimce sodes aciduria, and

convulsions, tim Severe . with (14-16, result hepa physical 23, in tomegaly, and an 26,

imypoglvcemia
oral 27). albuminuria, jaundice, mental

may
tolerepi-

(3). administration

intravelmous

become
but not(2, but is infusion in 6,

elevated
individuals 8). a in but D-Sorbitol larger in nornmal not is fructoseDivisioim,
Colorado.

in

normal
with is fraction time urine subjects essential result

sub- sis,
essential (i-l-l(;, converted of sent

23,
iim tiiverse

28,

29).
fasimion:

Time with

disease
neonatal

may
jaundice,

pre-

timis persistelmt imepatolmmegaly,(5). leads(30).

vomiting
and Synmptoins weaning nmay be

failure
for and to

to
scimool in some

timrive,
meals

excreted

tiislike

in
(14,

infants
15), difficult

typically
accomplish intravenous (14, time symptoms 26) diet glu-

start
cases be-

imyperuricemia

in time

frucof

after wealming a cause Time

(9).
intolerance of converts the
Hospital,

ofin of 28,

time
time 30, in

aversion
consists acute stage from 31). young The

to
of

sweetened

food.
anti (14, tend ex16, to

Imepatic

plmospimofructoaido1-pimosplmate U. S. Army

tleatmelmt fructose

timat From
Research

into cose Mcdi- clusiolm 26, Fitzsimons 23, be 693

Metabolic
and Nutrition Denver,

cal

laboratory,

Geimeral

severe

infantswimerea.s

older

694 cimildren
Members different i)atieflts tose-containing terest been caries that reported (14, 26).

Herman often
of degrees. imave time

and 24, 25).whicim
time to of to of of

Zakiin
does liver (14). not seem Time to decreased be assinmilated utilization by of

are
time

symptonmless
family Many may if be not

(13,
all is free

affected

fiuctose...