Renal Glucosuria

Last Updated: November 2, 2003

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Synonyms and related keywords: glucose in urine, renal glycosuria, Fanconi syndrome, cystinosis, Wilson disease, hereditary tyrosinemia, oculocerebrorenal syndrome, Lo e syndrome

AUTHOR INFOR ATION

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Author* Leonard G Feld$ %$ &'%, Chairman, #e%artment o" +ediatrics, Atlantic ,ealth -ystem, +ro"essor, #e%artment o" +ediatrics, .niversity o" 'edicine and #entistry o" Ne /ersey Leonard 0 Feld, '#, +h#, is a member o" the "ollo ing medical societies* American Academy o" +ediatrics, American College o" +hysician E1ecutives, American #iabetes Association, American ,eart Association, American -ociety o" Ne%hrology, American -ociety o" +ediatric Ne%hrology, American -ociety o" &rans%lant -urgeons, Eastern -ociety "or +ediatric Research, !nternational -ociety o" Ne%hrology, /uvenile #iabetes Foundation !nternational, National 2idney Foundation, -ociety "or E1%erimental )iology and 'edicine, and -ociety o" +ediatric Research Editor3s4* Laurence Fin(er)$ %, Clinical +ro"essor, #e%artment o" +ediatrics, .niversity o" Cali"ornia at -an Francisco and -tan"ord .niversity5 Ro(ert *onop$ &'arm%, Clinical Assistant +ro"essor, #e%artment o" +harmacy, -ection o" Clinical +harmacology, .niversity o" 'innesota5 Lut'er Tra+is$ %, William W 0lauser +ro"essor o" +ediatrics and +ediatric Ne%hrology, #e%artment o" +ediatrics, #ivisions o" Ne%hrology and #iabetes, .niversity o" &e1as 'edical )ranch and Children6s ,os%ital5 Howard Trac'tman$ %, +rogram #irector, +ediatrics Research, -chneider Children6s ,os%ital, +ro"essor, #e%artment o" +ediatrics, #ivision o" Ne%hrology, Albert Einstein College o" 'edicine5 and ,rai) - Lan)man$ %, ,ead, #ivision o" +ediatric Ne%hrology, Children6s 'emorial ,os%ital o" Chicago5 +ro"essor, #e%artment o" +ediatrics, North estern .niversity -chool o" 'edicine #isclosure

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Cystinosis #iabetes 'ellitus Fanconi -yndrome 8culocerebrorenal #ystro%hy 3Lo e -yndrome4 Wilson #isease

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INTRO%U,TION

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-ack)round: Renal glycosuria is the e1cretion o" glucose in the urine in detectable amounts at normal blood glucose concentrations or in the absence o" hy%erglycemia7 !n general, it is a benign condition and does not re9uire any s%eci"ic thera%y7 0lycosuria may be associated ith tubular disorders such as Fanconi syndrome, cystinosis, Wilson disease, hereditary tyrosinemia, or oculocerebrorenal syndrome 3Lo e syndrome47 &at'op'ysiolo)y: 0lucose is "reely "iltered by the glomerulus ith a "ractional e1cretion o" less than 07:;7 Adults e1crete about <= mg o" glucose %er day7 Reabsor%tion o" glucose occurs %redominantly on the brush border membrane o" the convoluted segment o" the %ro1imal tubule7 0lucose enters the tubular cells by an active carrier(mediated trans%ort %rocess, hich is sodium de%endent, and e1its via the basolateral membrane by "acilitated di""usion by a glucose trans%orter, hich is sodium inde%endent7 &he sodium>glucose cotrans%orter is %art o" the -0L&: grou% o" sodium cotrans%ort %roteins7 &he human intestinal -0L&: has been locali?ed to chromosome 227 -0L&: is "ound in the straight segment o" the %ro1imal tubule7 &he other cotrans%orter is -0L&2, hich is e1%ressed in the -: segment o" the %ro1imal tubule and is locali?ed to chromosome <7 &he "acilitative glucose trans%orters have iso"orms 0L.& :(=7 0L.&2 mainly is associated ith glucose trans%ort in the convoluted %ortion o" the %ro1imal tubule7 !n segments ith high reabsor%tive rates 3-: and -2 segments4, the carrier is high ca%acity, lo a""inity7 At birth, a high(a""inity lo (ca%acity %ath ay also e1ists to com%ensate "or the reduced activity o" the high(ca%acity lo (a""inity %ath ay7 0lucose reabsor%tion is age de%endent7 !n %remature in"ants o" less than 30 ee$s6 gestation, glycosuria is 9uite common because the "iltered load o" glucose delivered to the $idney o"ten is too high "or the immature ne%hron to handle7 0lycosuria normally occurs hen the %lasma glucose content is above 300 mg>dL, but some glucose may be seen in the urine at %lasma glucose levels as lo as :=0 because there

is a great deal o" variability in the glucose(handling ca%acity o" individual ne%hrons7 &his variability arises "rom variation in the length o" the %ro1imal tubule and di""erences in glomerular si?e and location7 &ubular ma1imum "or glucose 3&m glucose, mg>min>:7@3 m 24 corrected "or the glomerular "iltration rate 30FR4 does not vary as a "unction o" age7 &m glucose>0FR 3mg>mL4 %resents as "ollo s*

!n"ants ( 07A(27AB Children ( :7C2(27AB Adults ( 273:(27@0

&he &m glucose "or children e1%ressed in mg>min>:7@3 m 2 is as "ollo s*

+remature in"ants ( 2=(:A0 &erm in"ants ( 3<(2CC Children ( 2=B(B0:

Fre1uency:

In t'e US: !ncidence is estimated at 07:<(<73;7

ortality2 or(idity: Renal glycosuria is a benign condition7 ,o ever, morbidity is signi"icant in Fanconi syndrome, Lo e syndrome, and cystinosis 3see #i""erentials47 ,LINI,AL
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History: 'edical history o""ers no clues "or either %rimary or benign renal glycosuria7 !n cases associated ith combined tubular de"ects or hy%erglycemia 3ie, diabetes mellitus4, history is s%eci"ic to the disease or syndrome7 Renal glycosuria is "irst noted on routine urinalysis7 !n cases o" glycosuria associated ith tubular disorders, a history o" gro th "ailure, %olyuria, %olydi%sia, or dehydration may e1ist7 &'ysical: No %hysical e1amination "indings are relevant to renal glycosuria, unless associated ith a secondary cause

3eg, Fanconi syndrome, diabetes mellitus47 !n cases associated ith tubular disorders, signs or sym%toms may include hy%o%hos%hatemic ric$ets, dehydration, short stature, muscle hy%otonia, or ocular changes o" cataracts or glaucoma 3Lo e syndrome4 or 2ayser(Fleischer ring 3Wilson disease47 ,auses: &he renal abnormality is s%eci"ic to glucose and not other monosaccharides7 &he inheritance %attern is autosomal recessive, although autosomal dominance has been re%orted7 0lycosuria can be divided into 3 clinical scenarios, as "ollo s*

)enign glycosuria* &his condition has 3 variations and generally is discovered on routine urinalysis7
o

&y%e A is so(called classic glycosuria, ith reduction in both glucose threshold and ma1imal glucose reabsor%tion rate7 !n ty%e ), there is a reduction in the glucose threshold, normal reabsor%tive rate, and an increased s%lay7 &y%e 8 is de"ined by the com%lete absence o" glucose reabsor%tion7 +lasma glucose concentration, glucose tolerance testing, serum insulin concentrations, and glycosylated hemoglobin concentrations are normal7 8ther renal tubular abnormalities are absent7 ,o ever, there have been "amilies ith glycosuria and uricosuria in absence o" other as%ects o" renal tubular dys"unction7

0lycosuria ith diabetes mellitus and %regnancy( induced diabetes mellitus* 8bviously, %atients have elevated %lasma glucose concentration, abnormal glucose tolerance testing, and increased glycosylated hemoglobin concentrations7 &ubular dys"unction 3Fanconi syndrome4* &his includes a large number o" disorders characteri?ed by %resence o" %hos%haturia, bicarbonaturia, aminoaciduria, %olyuria, renal tubular acidosis, gro th

"ailure, and ric$ets7 !dio%athic, inherited, or ac9uired "orms e1ist7 &hera%y is directed to the tubular abnormality and disease state7 %IFF/R/NTIALS
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Cystinosis #iabetes 'ellitus Fanconi -yndrome 8culocerebrorenal #ystro%hy 3Lo e -yndrome4 Wilson #isease Ot'er &ro(lems to (e ,onsidered: !ntestinal glucose(galactose malabsor%tion !nterstitial ne%hritis ,ereditary tyrosinemia ,eavy metal into1ication 5OR*U&
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La( Studies:

.rinalysis ith microsco%ic analysis Fasting blood glucose concentration -erum electrolytes, bicarbonate, %hos%horus, and uric acid 0lycosylated hemoglobin Consider 2B(hour urine collection "or amino acids hen other tubular abnormalities e1ist7 Fractional e1cretion o" %hos%horus 3D:=;4, uric acid 3D:=; or uric acid %er dL 0FR D07==4, sodium 3normal limit ENLF :(3;4, %otassium 3D2=;4, bicarbonate 3NL D:=;4
Section 7 o" !#

TR/AT /NT

Author !n"ormation !ntroduction Clinical #i""erentials Wor$u% &reatment 'edication Follo (u% 'iscellaneous )ibliogra%hy

edical ,are: )enign renal glycosuria is a sel"(limiting %rocess and re9uires no s%ecial medical care7 !" other associated "indings suggest tubular disorders, then other interventions are re9uired7 ,onsultations: Consultation ith a %ediatric ne%hrologist may be a%%ro%riate7 %iet: No s%ecial dietary instructions are re9uired7 !n a very rare case o" e1tremely large amounts o" urinary glucose, glucose or another carbohydrate may be re9uired during e%isodes o" great %hysical activity to %revent hy%oglycemia7

/%I,ATION

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No treatment is re9uired "or benign renal glycosuria7 FOLLO59U&

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Furt'er Outpatient ,are:

Routine medical "ollo (u% care ith the %rimary care %rovider is re9uired7

&ro)nosis:

+rognosis "or benign renal glycosuria is e1cellent7

&atient /ducation:

)enign renal glycosuria has no relationshi% to diabetes mellitus7 Ensure that the %atient and "amily understand this and the e1cellent %rognosis7 IS,/LLAN/OUS
Section ; o" !#

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edical2Le)al &it"alls:

No medical>legal concerns e1ist "or benign renal glycosuria, but be sure to rule out other causes o" high urinary glucose concentrations, as indicated in

the di""erential diagnosis7 -I-LIOGRA&H<

Section !# o" !#

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)rodehl /, Fran$en A, 0ellissen 2* 'a1imal tubular reabsor%tion o" glucose in in"ants and children7 Acta +aediatr -cand :A@2 /ul5 <:3B4* B:3( 20E'edlineF7 )rodehl /, Edelmann C' /r, eds* Renal glycosuria7 !n* +ediatric 2idney #isease7 2nd ed7 Little )ro n G Co5 :AA2*:C0:(:07 Crombie #L* !ncidence o" glycosuria and diabetes7 +roc R -oc 'ed :A<:5 ==* 20=7 #esHeu? /F, &ur$ E, Wright E* Congenital selective NaI #(glucose co( trans%ort de"ects leading to renal glycosuria and congenital selective intestinal malabsor%tion o" glucose and galactose7 !n* &he 'etabolic and 'olecular )asis o" !nherited #iseases7 :AA=*3=<3(3=C07 Elias L/, Longo N* 0lucose trans%orters7 Ann Rev 'ed :AA25 B3* 3@@( 3A3E'edlineF7 Elsas L/, Rosenberg LE* Familial renal glycosuria* a genetic rea%%raisal o" he1ose trans%ort by $idney and intestine7 / Clin !nvest :A<A 8ct5 BC3:04* :CB=(=BE'edlineF7 ,ar$ness /* +revalence o" glycosuria and diabetes mellitus* A com%rehensive survey in an urban community7 )r 'ed / :A<25 :* :=037 /ac$son W+, 'arine N, Jini$ A!* &he signi"icance o" glycosuria7 Lancet :A<C 'ay B5 :3@=BA4* A33(<E'edlineF7 /ones #+, Chesney RW* #evelo%ment o" tubular "unction7 +ediatric Clinics o" North America :AA25 :A* 33(=@E'edlineF7 2eller #'* 0lucose e1cretion in man and dog7 Ne%hron :A<C5 =3:4* B3( <<E'edlineF7 'arble A* Nondiabetic melituria7 !n* 'arble A, White +, )radley RF, et al eds7 /oslin6s #iabetes 'ellitus 7 ::th ed7 +hiladel%hia, +a* )C #ec$er !nc5 :A@:7 -anter R, 2inner ', Lassen CL, et al* 'olecular analysis o" the -0L&2 gene in %atients ith renal glucosuria7 / Am -oc Ne%hrol 2003 Nov5 :B3::4* 2C@3( C2E'edlineF7 -!mmons RA* Cell glucose trans%ort and glucose handling during "etal and neonatal develo%ment7 !n* Rolin RA, Fo1 WW, eds7 Fetal and Neonatal +hysiology7 2nd ed7 +hiladel%hia* W) -aunders Co5 :AAC*=C=(A07 -%it?er A* &he develo%ing $idney and the %rocess o" gro th7 !n* &he -eldin #W, 0iebisch 0, eds7 2idney* +hysiology and +atho%hysiology7 :AC=7 Wool" L!, 0ood in )L, +hel%s CE* &(m(limited renal tubular reabsor%tion and the genetics o" renal glucosuria7 / &heor )iol :A<< 'ay5 ::3:4* :0( 2:E'edlineF7 Keli$ovic !* Aminoaciduria and glycosuria7 !n* )arratt &', Avner E#, ,armon WE, eds7 +ediatric Ne%hrology7 Bth ed7 Li%%incott Williams G Wil$ins5

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