Renal Glucosuria

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    Renal GlucosuriaLast 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, Loe syndrome

    AUTHOR INFORATION Section ! o" !#

    Author !n"ormation!ntroductionClinical#i""erentialsWor$u%&reatment'edicationFollo(u%'iscellaneous)ibliogra%hy

    Author* Leonard G Feld$ %$ &'%, Chairman, #e%artmento" +ediatrics, Atlantic ealth -ystem, +ro"essor,#e%artment o" +ediatrics, .niversity o" 'edicine and#entistry o" Ne /ersey

    Leonard Feld, '#, +h#, is a member o" the "olloing

    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 #iabetesFoundation !nternational,National idney Foundation,-ociety "or E1%erimental )iology and 'edicine, and-ociety o" +ediatric Research

    Editors4* Laurence Fin(er)$ %, Clinical +ro"essor,

    #e%artment o" +ediatrics, .niversity o" Cali"ornia at -anFrancisco 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 lauser+ro"essor o" +ediatrics and +ediatric Ne%hrology,#e%artment o" +ediatrics, #ivisions o" Ne%hrology and#iabetes, .niversity o" &e1as 'edical )ranch andChildren6s os%ital5 Howard Trac'tman$ %, +rogram#irector, +ediatrics Research, -chneider Children6sos%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, Northestern .niversity -choolo" 'edicine

    #isclosure

    .uick Find

    Author !n"ormation!ntroductionClinical#i""erentials

    Wor$u%&reatment'edicationFollo(u%'iscellaneous)ibliogra%hy

    Clic$ "or relatedimages7

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    Cystinosis

    #iabetes 'ellitus

    Fanconi -yndrome

    8culocerebrorenal#ystro%hy Loe-yndrome4

    Wilson #isease

    ,ontinuin)/ducation

    C'E available "orthis to%ic7 Clic$hereto ta$e thisC'E7

    &atient /ducation

    Clic$here "or%atient education7

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    INTRO%U,TION Section 0 o" !#

    Author !n"ormation!ntroductionClinical#i""erentialsWor$u%&reatment'edicationFollo(u%'iscellaneous)ibliogra%hy

    -ack)round: Renalglycosuria isthe e1cretion o" glucoseinthe urine in detectable amounts at normal blood glucoseconcentrations or in the absence o" hy%erglycemia7 !ngeneral, it is a benign condition and does not re9uire anys%eci"ic thera%y7 lycosuria may be associated ith tubulardisorders such as Fanconi syndrome, cystinosis, Wilsondisease, hereditary tyrosinemia, or oculocerebrorenalsyndrome Loe syndrome47

    &at'op'ysiolo)y: lucose is "reely "iltered by theglomerulus ith a "ractional e1cretion o" less than 07:;7

    Adults e1crete about

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    is a great deal o" variability in the glucose(handling ca%acityo" individual ne%hrons7 &his variability arises "rom variation inthe length o" the %ro1imal tubule and di""erences inglomerular si?e and location7

    &ubular ma1imum "or glucose &m glucose, mg>min>:7@3 m24corrected "or the glomerular "iltration rate FR4 does notvary as a "unction o" age7 &m glucose>FR mg>mL4 %resentsas "ollos*

    !n"ants ( 07(27B

    Children ( :72(27B

    Adults ( 273:(27@0

    &he &m glucose "or children e1%ressed in mg>min>:7@3 m2is

    as "ollos*

    +remature in"ants ( 2=(:0

    &erm in"ants ( 3

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    eg, Fanconi syndrome, diabetes mellitus47

    !n cases associated ith tubular disorders, signs orsym%toms may include hy%o%hos%hatemic ric$ets,

    dehydration, short stature, muscle hy%otonia, or ocularchanges o" cataracts or glaucoma Loe syndrome4 orayser(Fleischer ring Wilson disease47

    ,auses: &he renal abnormality is s%eci"ic to glucose and notother monosaccharides7 &he inheritance %attern is autosomalrecessive, although autosomal dominance has beenre%orted7 lycosuria can be divided into 3 clinical scenarios,as "ollos*

    )enign glycosuria* &his condition has 3 variations and

    generally is discovered on routine urinalysis7

    o &y%e A is so(called classic glycosuria, ithreduction in both glucose threshold andma1imal glucose reabsor%tion rate7

    o !n ty%e ), there is a reduction in the glucose

    threshold, normal reabsor%tive rate, and anincreased s%lay7

    o &y%e 8 is de"ined by the com%lete absence o"

    glucose reabsor%tion7 +lasma glucoseconcentration, glucose tolerance testing, seruminsulin concentrations, and glycosylatedhemoglobin concentrations are normal7 8therrenal tubular abnormalities are absent7oever, there have been "amilies ithglycosuria and uricosuria in absence o" otheras%ects o" renal tubular dys"unction7

    lycosuria ith diabetes mellitus and %regnancy(

    induced diabetes mellitus* 8bviously, %atients have

    elevated %lasma glucose concentration, abnormalglucose tolerance testing, and increased glycosylatedhemoglobin concentrations7

    &ubular dys"unction Fanconi syndrome4* &his

    includes a large number o" disorders characteri?ed by%resence o" %hos%haturia, bicarbonaturia,aminoaciduria, %olyuria, renal tubular acidosis, groth

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    "ailure, and ric$ets7 !dio%athic, inherited, or ac9uired"orms e1ist7 &hera%y is directed to the tubularabnormality and disease state7

    %IFF/R/NTIALS Section 4 o" !#

    Author !n"ormation!ntroductionClinical#i""erentialsWor$u%&reatment'edicationFollo(

    u%'iscellaneous)ibliogra%hy

    Cystinosis#iabetes 'ellitusFanconi -yndrome8culocerebrorenal #ystro%hy Loe -yndrome4Wilson #isease

    Ot'er &ro(lems to (e ,onsidered:

    !ntestinal glucose(galactose malabsor%tion!nterstitial ne%hritisereditary tyrosinemiaeavy metal into1ication

    5OR*U& Section 6 o" !#

    Author !n"ormation!ntroductionClinical#i""erentialsWor$u%&reatment'edicationFollo(u%'iscellaneous)ibliogra%hy

    La( Studies:

    .rinalysis ith microsco%ic analysis

    Fasting blood glucose concentration

    -erum electrolytes, bicarbonate, %hos%horus, and uric acid

    lycosylated hemoglobin

    Consider 2B(hour urine collection "or amino acids hen other tubular

    abnormalities e1ist7

    Fractional e1cretion o" %hos%horus D:=;4, uric acid D:=; or uric acid %erdL FR D07==4, sodium normal limit NL :(3;4, %otassium D2=;4,bicarbonate NL D:=;4

    TR/AT/NT Section 7 o" !#

    http://www.emedicine.com/ped/topic1991.htm#section~author_information%23section~author_informationhttp://www.emedicine.com/ped/topic1991.htm#section~introduction%23section~introductionhttp://www.emedicine.com/PED/topic538.htmhttp://www.emedicine.com/PED/topic1329.htmhttp://www.emedicine.com/ped/topic1991.htm#section~treatment%23section~treatmenthttp://www.emedicine.com/ped/topic1991.htm#top%23tophttp://www.emedicine.com/ped/topic1991.htm#section~differentials%23section~differentialshttp://www.emedicine.com/ped/topic1991.htm#section~workup%23section~workuphttp://www.emedicine.com/ped/topic1991.htm#top%23tophttp://www.emedicine.com/ped/topic1991.htm#section~clinical%23section~clinicalhttp://www.emedicine.com/ped/topic1991.htm#section~author_information%23section~author_informationhttp://www.emedicine.com/ped/topic1991.htm#section~introduction%23section~introductionhttp://www.emedicine.com/ped/topic1991.htm#section~clinical%23section~clinicalhttp://www.emedicine.com/ped/topic1991.htm#section~differentials%23section~differentialshttp://www.emedicine.com/ped/topic1991.htm#section~workup%23section~workuphttp://www.emedicine.com/ped/topic1991.htm#section~treatment%23section~treatmenthttp://www.emedicine.com/ped/topic1991.htm#section~medication%23section~medicationhttp://www.emedicine.com/ped/topic1991.htm#section~follow-up%23section~follow-uphttp://www.emedicine.com/ped/topic1991.htm#section~follow-up%23section~follow-uphttp://www.emedicine.com/ped/topic1991.htm#section~miscellaneous%23section~miscellaneoushttp://www.emedicine.com/ped/topic1991.htm#section~bibliography%23section~bibliographyhttp://www.emedicine.com/PED/topic538.htmhttp://www.emedicine.com/PED/topic581.htmhttp://www.emedicine.com/PED/topic756.htmhttp://www.emedicine.com/PED/topic1329.htmhttp://www.emedicine.com/PED/topic2441.htmhttp://www.emedicine.com/ped/topic1991.htm#section~author_information%23section~author_informationhttp://www.emedicine.com/ped/topic1991.htm#section~introduction%23section~introductionhttp://www.emedicine.com/ped/topic1991.htm#section~clinical%23section~clinicalhttp://www.emedicine.com/ped/topic1991.htm#section~differentials%23section~differentialshttp://www.emedicine.com/ped/topic1991.htm#section~workup%23section~workuphttp://www.emedicine.com/ped/topic1991.htm#section~treatment%23section~treatmenthttp://www.emedicine.com/ped/topic1991.htm#section~medication%23section~medicationhttp://www.emedicine.com/ped/topic1991.htm#section~follow-up%23section~follow-uphttp://www.emedicine.com/ped/topic1991.htm#section~miscellaneous%23section~miscellaneoushttp://www.emedicine.com/ped/topic1991.htm#section~bibliography%23section~bibliography
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    Author !n"ormation!ntroductionClinical#i""erentialsWor$u%&reatment'edicationFollo(u%'iscellaneous)ibliogra%hy

    edical ,are: )enign renal glycosuria is a sel"(limiting %rocess and re9uires nos%ecial medical care7 !" other associated "indings suggest tubular disorders, thenother 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" e1tremelylarge amounts o" urinary glucose, glucose or another carbohydrate may be re9uiredduring e%isodes o" great %hysical activity to %revent hy%oglycemia7

    /%I,ATION Section 8 o" !#

    Author !n"ormation!ntroductionClinical#i""erentialsWor$u%&reatment'edicationFollo(u%'iscellaneous)ibliogra%hy

    No treatment is re9uired "or benign renal glycosuria7

    FOLLO59U& Section o" !#

    Author !n"ormation!ntroductionClinical#i""erentialsWor$u%&reatment'edicationFollo(u%'iscellaneous)ibliogra%hy

    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" !#

    Author !n"ormation!ntroductionClinical#i""erentialsWor$u%&reatment'edicationFollo(u%'iscellaneous)ibliogra%hy

    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

    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