INTRODUCTION
Dietary phenylalanine (PA) restrictio n is the mo st effective fo rm fo r reducing its excess in the blo o d and is the o nly efficient metho d fo r treating phenylketo nuria.1 The diet is co mplex and sho uld b e a da pte d to c o mb ine the pa tie nts’ e a ting
habits, g ro wth and develo pment.2 It depends
basically o n the use o f industrializ ed pro ducts as substitutes free o f PA fo r pro teins that are no t fully supplied.
W hitehead3 o bserved that the main interest in the fo llo w-up o f patients with this disease is fo cused o n the pro teins, but that it is essential to co nsider pro tein requirements within the co ntext o f energ y. He stated that energ y co nsumptio n sho uld b e mo nito re d b y a nthro p o me tric measurements such as weig ht and heig ht. In a c o lla b o ra tive stud y o n 1 1 3 c hild re n in phenylketo nuria (PKU) treatment centers in the US, g ro wth standards were no rmal.4
In Brazil, Fisberg et al5 evaluated 6 0 children w ith PKU using c o ntinuo us a nthro p o me tric assessment metho ds, sho wing that as the time o f treatment became mo re delayed, there was a wo rsening o f weig ht and heig ht data in the late-diag no sed g ro up (late-diag no sed after 3 mo nths o f ag e). Thus, restricted diets may lead to vario us
Nutritional e valuation of childre n
with phe nylke tonuria
Associação de Pais e Amigos dos Excepcionais (APAE) de São Paulo, São Paulo, Brazil
Regina Mara Fisberg Maria Eugênia da Silva-Fernandes Benjamim José Schmidt Mauro Fisberg
ABSTRACT
Contex t: Dietary phenylalanine (PA) restrictio n is the mo st effective fo rm fo r reducing its excess in the blo o d and is the o nly efficient metho d fo r treating phenylketo nuria.The diet is co mplex and sho uld be adapted to co mbine the patients’ eating habits, g ro wth and develo pment. It depends basically o n the use o f industrializ ed pro ducts as substitutes free o f PA fo r pro teins that are no t fully supplied.
O bjective: To evaluate the nutritio nal status o f children with phenylketo nuria (PKU) by anthro po metric
measurements and fo o d intake. Design: Cro ss-sectio nal study.
Setting: Children with PKU attending the Asso ciatio n o f Parents and Friends o f Handicapped Children (Asso ciação de Pais e Amigo s do s Excepcio nais - APAE) and no rmal children attending at municipal day care centers in São Paulo . Pa rticipa nts: 4 2 children with PKU and 3 1 no rmal children ag ed 1 to 1 2 o f bo th sexes were assessed in two g ro ups, under and o ver 7 years o f ag e.
M a in M ea surements: W eig ht and heig ht measurements. Results: Children with PKU ingested calo ries, calcium, iro n, zinc, and co pper belo w the reco mmended values, whereas the pro tein intake was within the no rmal range. Fo o d intake in the gro up o f no rmal children was within no rmality rates. The height/ weight Z-sco re means fo r children with PKU were 0 .4 7 fo r tho se under 7 years and 1 .8 6 fo r 7 year-o lds and o ver; in no rmal children the means were 0 .9 7 <7 years and 1 .5 4 ≥7 years, with no statistically significant difference. The height/ age Z-sco re means were significantly lo wer in the PKU children <7 years (-1 .2 3 ) than in the no rmal co ntro ls (0 .9 1 ). Conclusions: The data presented demo nstrate the impo rtance o f nutritio nal surveillance in patients with PKU so as to suppo rt adequacy o f nutrient intake and to g uarantee g ro wth within the relevant standards. Key w ords: Phenylketo nuria. Artificial diets. Anthro po metric measurements.
diso rde rs o f the e c o no my o f huma n b e ing s, reflected in g ro wth and develo pment alteratio ns that depend o n the time o f appearance o f the diso rder, duratio n o f nutritio nal privatio n and degree o f severity o f the diso rder. Therefo re, based o n the se fa c ts, w e re so lve d to e va lua te the nutritio nal status o f PKU children.
METHODS
Participants
Fo rty-two PKU children ag ed 1 to 1 2 years o f bo th sexes to o k part in this study, divided into two g ro ups: under 7 years o ld (n = 2 4 ), and 7 and o ver (n = 1 8 ). They were fo llo wed up at the A sso c ia tio n o f Pa re nts a nd Frie nd s o f Handicapped Children (Asso ciação de Pais e Amig o s do s Excepcio nais - APAE). The co ntro l g ro up co mprised 3 1 no rmal children (< 7 years n = 9 ;
≥
7 years n = 2 2 ), who attended municipal day care centers in São Paulo and presented characteristics similar to tho se o f the PKU g ro up. The children were divided into two ag e g ro up s b e c a use the a ve ra g e a mo unt o f the spec ia l pro duc t to b e o ffered to the c hildren differed between the two ag e g ro ups, fo llo wing the p ro d uc t usa g e instruc tio ns g ive n b y the manufacturer.The pro c e dure s fo llo we d we re in c lo se ag reement with the ethical standards o f the Ethics Co mmittee o f the Federal University o f São Paulo , and were thus appro ved by its members.
Dietary assessment
Dietary assessments o f children with PKU were reco rded o n a special fo rm develo ped fo r a 3 -day study, 2 days being in the middle o f the week and 1 day during the weekend.6 The dietary fo rmula used fo r children with phenylketo nuria was PKU2 (Milupa, Friedrichsdo rf, G ermany). In the g ro up o f no rma l c hild re n, fo o d inta ke w a s e va lua te d b y the w e ig he d fo o d re c o rd .7 Standardizatio n o f measurements o f meal po rtio ns was achieved by cho o sing the mo st suitable utensil fo r each kind o f fo o d. The amo unt served was established acco rding to what children were used to ing esting . In additio n, the same amo unt o f fo o d
was served to all the children. Afterwards, fo o d served rando mly to three children at each meal was weighed to kno w the average amo unt o ffered. The average amo unt o f fo o d ingested at each meal was o btained by adding the to tal amo unt o f fo o d o ffered to the to tal amo unt o f repetitio ns, and then subtracting fro m this value the amo unt o f lefto vers. In o rder to o btain this data, the lefto vers were co llected in plastic bag s, identified with each child’s name, and weighed witho ut separating the fo o d, using do mestic scales with a capacity o f 5 kg and g raduatio ns o f 1 0 mg . Fo r fluid fo o d a pla stic g la ss with g ra dua tio ns o f 5 0 ml a nd capacity o f 5 0 0 ml was used. The metho d was used fo r 2 days fo r each child and the mean, o btained as described abo ve, was used as the reference fo r adequacy o f intake amo ng the gro up o f no rma l c hild re n, in re la tio n to the ir reco mmended diet.
Calculatio n o f the intake o f calo ries, pro tein, lipid, c a rb o hydra te , c a lc ium, iro n, z inc a nd c o ppe r wa s do ne using c o mpute r so ftwa re .8 W hen any fo o d that was no t listed in the so ftware d a ta b a se w a s ing e ste d , the Ta b le o f Fo o d Co mpo sitio n fro m the N atio nal Study o f Family Expenses was used, to g ether with So uci et al.9 .1 0 Fo r diet adequacy purpo ses, the Reco mmended Die ta ry A llo w a nc e s (RDA )1 1 w e re use d a s standard.
W e used the fo llo wing calculatio n to o btain the pe rc e nta g e a de q ua c y o f the ing e ste d in relatio n to the reco mmended: % adequacy = [(ing ested x 1 0 0 )/ reco mmended].
The metho do lo g y fo r the evaluatio n o f fo o d intake o f an individual o r a g ro up depends o n the o bjectives o f the pro ject and o n their feasibility and applicatio n. In o rder to use two different metho ds o f evaluating fo o d intake, the g ro ups were no t c o mpa red to ea c h o ther. The fo o d evaluatio n in the co ntro l g ro up had the aim o f d e mo nstra ting tha t the se c hild re n w e re no t restricted either reg arding quantity o r quality o f fo o d.
Anthropometric measurements
were weig hed using an anthro po metric scale while o nly wearing underwear, and after prio r qualificatio n by the examiner. Fo r children belo w 2 y e a rs, he ig ht w a s me a sure d w ith a n a nthro po metric ruler, a nd the pa tient wa s in do rsal decubitus. Fo r children o ver 2 years o ld, a mo bile stadio meter was used fo r measuring standing heig ht. The children sto o d with their backs to the stadio meter, heels alig ned and flat o n the flo o r, knees in a straig ht po sitio n, bo dy and head leaning o n the stadio meter; a wo o den, wide-based square with an ang le o f 9 0 ° was lo wered o ver the child’s head. Standardiz atio n a nd q ua lity c o ntro l o f a nthro p o me tric measurements were carried o ut acco rding to the re c o mme nd a tio ns o f Lo hma n e t a l.1 2 The calculatio ns o f the anthro po metric ratio s o f weight fo r ag e (W / A), heig ht fo r ag e (H/ A) and weig ht fo r height (W / H) were carried o ut using co mputer so ftwa re ,1 3 with distrib utio ns a na lyz e d a s Z-sco res based o n N CHS reference data.1 4
Statistical Metho ds. To analyz e the results, we applied the Mann-W hitney Test1 5 so as to co mpare the ag e g ro ups o f children in relatio n to the stud ie d va ria b le s. This w a s d o ne b y evaluating the fit to the no rmal distributio n curve (statistic Z) when the sample siz e required this.
RESULTS
Acco rding to the data sho wn in Table 1 and Fig ure 1 , referring to the two g ro ups studied, the pro tein intake was adequate when co mpared to the sta nd a rd re c o mme nd e d d ie t.1 1 N evertheless, when we analyz ed the averag e pro tein intake o f the g ro up o f no rmal children, this represented twic e (2 7 3 %) the sug g ested pro tein amo unt and this finding was similar to o the r stud ie s. The re c o mme nd e d inta ke o f
phenylalanine was o btained in 9 0 to 1 0 0 % o f the menus and this is reflected by the pro tein a d e q ua c y p re se nte d b y the stud ie d g ro up . Children with PKU ing ested averag e percentag es o f calo ries and nutrients with the exceptio n o f co pper in
≥
7 year o lds, which was lo wer than the reco mmended (Table 1 ).There was a substantial difference between the diet adequacy averag es fo und fo r calcium, iro n a nd c o p p e r inta ke in c hild re n w ith phenylketo nuria. The eating pattern fo llo wed by PKU children excluded fo o ds that were so urces o f a nima l pro tein. The fo llo wing fo o ds were included in their diet: cereals, breads, tubers, ro o ts, fruits and veg etables, in co ntro lled amo unts depending o n the characteristics o f the child. The patients were g iven examples o f menus with a list o f equivalents fo r substitutio n, and it sho uld be no ted that the menus were devised taking into acco unt the children’s preferences, ease o f preparatio n and amo unt o f phenylalanine, to the detriment o f o ther nutrients required fo r g ro wth. In the co ntro l g ro up the mea n percenta g e o f ing estio n adequacy reached at least two -thirds o f the reco mmended diet during their stay in the day-care institutio n, sho wing that this g ro up was no t at risk o f impairment o f its nutritio nal status. The children stayed in the institutio n until late afterno o n, receiving two snacks, lunch and so up in the afterno o n, with dinner at ho me expected to co mplement the day’s no urishment.
The weig ht fo r heig ht (W / H) and weig ht fo r a g e (W / A ) va lue s in c hild re n w ith phenylketo nuria did no t sho w any substantial differences when co mpared to no rmal children (Tables 2 and 3 ). The mean Z-sco re o f heig ht fo r a g e (H/ A ) sho w e d sta tistic a lly sig nific a nt differenc es b etween no rma l c hildren a nd the patients with phenylketo nuria < 7 years (Table
Ta ble 1 - M ea n da ily inta k e percenta ges of ca lories, proteins, ca lcium, iron, zinc a nd copper of children w ith phenylk etonuria , a ccording to a ge
Ag e Calo rie Pro tein Calcium Iro n Zinc Co pper
< 7 years 6 2 .6 (1 9 ) 1 0 5 .0 (3 2 ) 5 6 .1 (1 2 )* 7 2 .3 (2 0 )* 3 6 .6 (1 4 ) 8 5 .8 (2 6 )*
4 ), but within no rmality rates. In the evaluated g ro ups o f PKU children < 7 years and
≥
7 years, the lo w heig ht percentag es were 5 0 % and 2 2 %, respectively. These percentag es were hig her than tho se fo und in no rmal children (< 7 years - no children and≥
7 years - 1 8 %).DISCUSSION
The calo rie intake results sho wed that it was mo re difficult fo r children with PKU to o btain reco mmended calo ries than pro tein, since the sp e c ia l fo rmula sup p lie d a sma ll a mo unt o f calo ries. Ano ther facto r that needed to be taken into acco unt was that the amo unt o f ing ested phenyla la nine ha d to b e co ntro lled. Different fo o ds were included in the diet acco rding to preference, children’s acceptance, and ease o f p re p a ra tio n a nd a c q uisitio n, a nd w e re no t a lw a y s sub stitute d fo r a d e q ua te c a lo rie equivalents. W e no rmally used fats and sug ars
to increase the to tal calo rie intake. N evertheless, c hild re n d id no t a lw a ys a c c e p t the g re a te r a mo unts o f o il in fo o d p re p a ra tio n a s a g e increased. W e did no t have at o ur dispo sal lo w-pro tein fo o ds to increase the calo rie intake.
The results hig hlig ht the fact that it is no t always po ssible to fo llo w reco mmendatio ns fo r children with PKU who need lo w pro tein fo o ds. Ade q ua te c a lo rie inta ke pre ve nts the use o f pro te in a s a so urc e o f e ne rg y. The a ve ra g e calcium intake by children with PKU < 7 years was substantially lo wer than by children
≥
7 ye a rs, b e c a use a s a g e inc re a se d, a g re a te r a mo unt o f PA-fre e me d ic a l fo o d w a s ta ke n. Co nsequently, the amo unt o f calcium ing ested increased as a percentag e o f RDA.Ano ther facto r that sho uld be taken into acco unt is that with increasing ag e, PA intake increases and o ther fo o d calcium so urces can be used in g reater amo unts such as milk and cream in the fo rmulae, thus increasing the co ntent
Ta ble 2 - Distribution of w eight for height (W / H) Z-score of children w ith phenylk etonuria (PKU) a nd norma l (N ), a ccording to a ge
N o rmal < 7 years PKU < 7 years N o rmal ≥ 7 years PKU ≥ 7 years
ZW / H 0 .9 7 (1 .1 8 ) 0 .4 7 (1 .0 6 ) 1 .5 4 (3 .5 0 ) 1 .8 6 (3 .1 3 )
% -2 ZW / H - - -
-% +2 ZW / H 2 2 .2 1 2 .5 1 3 .6 2 2 .2
Standard deviatio n g iven in parenthesis
Ta ble 4 - Distribution of height for a ge (H/ A) Z-score of children w ith phenylk etonuria (PKU) a nd norma l (N ), a ccording to a ge
N o rmal < 7 years PKU < 7 years N o rmal ≥ 7 years PKU ≥ 7 years
ZH/ A 0 .9 1 (1 .2 6 )* -1 .2 3 (0 .8 4 )* -0 .0 5 (1 .0 3 ) -0 .3 2 (0 .9 1 )
% -2 ZH/ A - 1 6 .7 4 .5 5 .6
% +2 ZH/ A 2 2 .2 - -
-* p ≤ 0 .0 5 (PKU < 7 years is less than no rmal < 7 years); Standard deviatio n g iven in parenthesis
Ta ble 3 - Distribution of w eight for a ge (W / A) Z-score of children w ith phenylk etonuria (PKU) a nd norma l (N ), a ccording to a ge
N o rmal < 7 years PKU < 7 years N o rmal ≥ 7 years PKU ≥ 7 years
ZW / A 1 .1 7 (1 .4 7 ) -0 .4 4 (1 .0 8 ) 0 .1 1 (1 .2 0 ) 0 .3 0 (1 .1 2 )
% -2 ZW / A - 4 .2 -
-% +2 ZW / A 2 2 .2 - 9 .1 5 .6
o f this nutrient. The mea n c a lc ium inta kes o f children with PKU, as a percentage o f RDA, fo und by Aco sta et al1 6 were 8 4 to 1 3 6 %. The fo rmula used in that study was Phenyl-Free, which has a g reater amo unt o f calcium per g ram o f pro tein than that fo und in PKU2 . The 1 0 subjects assessed in that study were between 3 and 1 3 years o ld and o ther fo o d so urces that wo uld lead to a better intake o f this nutrient were no t mentio ned.
In the present study, the adequacy o f iro n intake by the children with PKU was better than fo r calcium. G ro pper et al1 7 fo und that the iro n intake by children with PKU (4 to 7 years o ld, 1 9 9 % and 7 to 1 1 years o ld, 2 9 6 %) was hig her than that o f the co ntro l g ro up (1 2 6 % and 1 7 4 % respectively), being g reater than RDA in bo th g ro ups. Data pro vided by Aco sta et al1 6 sho wed that iro n intake by children with PKU was abo ve the RDA. N evertheless, so me children had indices o f iro n nutritio n sta tus b e lo w no rma l va lue s. Scag lio ni et al 1 8 fo und co ncentratio ns o f serum ferritin substantially lo wer in the children with PKU than in no rmal children. Ho wever, o ther evaluated indices o f iro n nutritio n status did no t differ amo ng the g ro ups.
Lo ng hi et al 1 9 studied children with PKU a nd fo und iro n inta ke to b e 6 5 % o f the reco mmended. In the ag e g ro up fro m 1 2 to 2 6 mo nths, these rates were exactly the same as tho se fo und in o ur study (6 2 %). W hen the ag e g ro up fro m 5 years and 9 mo nths to 1 0 years and 1 0 mo nths (9 children) was evaluated, the a deq ua c y o f iro n inta ke wa s 1 6 4 % o f RDA. Ho wever, the hig h intake o f no n-heme iro n and its lo w a va ila b ility c o uld inte rfe re w ith the abso rptio n o f this element. Despite the fact that iro n intake in o ur study reached lo wer values than tho se referred to in the previo us literature, o ur g ro up with PKU had a lo w prevalence o f anemia (1 8 %), when co mpared with São Paulo State data.2 0 In o ur diet, children used mo dified po wdered milk in amo unts that supplied up to 1 5 % o f RDA fo r iro n. In children under 7 years, this p e rc e nta g e is a b o ve w ha t PKU2 w o uld supply (1 3 %).
Lo ng hi et al1 9 also sho wed that the mean amo unt o f z inc ing ested by children with PKU
was 6 4 % o f RDA. The same lo w values were fo und by Aco sta et al.2 1 These patients had an intake belo w 1 0 0 % o f RDA and 3 8 .5 % were belo w 6 7 % o f RDA.
W e hig hlig ht the fa c t tha t, fo r o the r evaluated nutrients (calcium, iro n, co pper), a g reater amo unt o f PKU2 was used as the g ro up ag e increased, which co nsequently increased the percentag e o f the attained RDA. This did no t happen in the case o f z inc. It may no t o nly have been the increase in the special fo rmula that led to increased adequacy, but also the inclusio n o f o ther fo o ds in g reater amo unts. Fo o ds which are so urces o f z inc, bo th o f animal o rig in (meats, fish a nd sea fo o d) a nd veg eta l o rig in (who le g rains, chestnut and leg umino us plants) were co mpletely absent fro m the diet.
The fo o d so urces o f z inc and co pper are similar, but their distributio ns were fo und to be different when we evaluated the diet. As the diet was co mpo sed basically o f veg etables (with the exclusio n o f leg umino us o nes) and fruits, meeting a reco mmendatio n fo r 1 mg co pper was easier than meeting o ne fo r 1 0 mg z inc. In balanced diets, co ntaining adequate amo unts o f all fo o ds, this do es no t o ccur, since 1 0 0 g beef co rrespo nds to 8 7 .5 % o f RDA fo r z inc.
W ith to ta l re stric tio n o f so urc e s ric h in animal pro teins and trace elements, children with PKU canno t maintain an adequate intake o f z inc a nd o the r me ta ls if the me d ic a l fo o d is
Figure 1 - Mean daily intake percentages of
inadequately supplemented.
Va lue s e q ua l to o r hig he r tha n tho se sug g ested fo r the intake o f co pper were also fo und by o ther autho rs.1 6 .1 7 .1 9 W e co nclude that the special fo rmulae meet the needs fo r mo st trace elements, but this do es no t happen with z inc and iro n.
Children with PKU are no rmal when they are bo rn, since phenylalanine hydro xylase in the mo ther’s liver will metabo lize phenylalanine. The weig ht at birth is no t chang ed because o f the illness.5 Ac c o rding ly, g ro wth de pe nds o n the amo unt o f PA ing ested and adequacy o f blo o d PA co ncentratio ns as well as the adequacy o f intake o f pro tein and energ y.
Sibing a et al,2 2 while studying 6 0 children with PKU receiving treatment, fo und a substantial g ro wth decrease (weig ht and heig ht), reg ardless o f the ag e at which the diet was initiated. W hen these results were co mpared to tho se fro m o ther centers, they co ncluded that phenylketo nuria, o r its tre a tme nt, w e re a sso c ia te d w ith he ig ht decrease. O ur results sho w a g reater relatio nship b e tw e e n p ro te in inta ke a nd he ig ht o f suc h patients. It is pro bable that fo r g ro wth, no t o nly is pro tein adequacy necessary, but also dietary balance as a who le.
W e ha ve a lrea dy desc rib ed tha t the diet ing ested b y c hildren with PKU in o ur study met a ll pro tein needs. Ho wever, the rec o mmended energ y inta kes were no t rea c hed, a nd pa rt o f the pro tein wa s pro b a b ly used b y the b o dy a s a ca lo rie so urce. Phenylketo nuria diets a re a b le to ma inta in weig ht fo r heig ht indic es. Either the c hild w ith PKU suffe rs a n a d a p ta tio n to the ing e ste d nutrie nts o r, a s the g ro up a g e inc re a se s, the re stric tio ns a re re d uc e d a nd g rea ter a mo unts o f fo o ds a re ing ested (spec ia l fo rmula a nd na tura l so urc e s) le a d ing to nutritio na l rec o very.
W e must po int o ut that during the treatment we used PKU1 , which meets the nutritio nal needs o f infants with PKU up to the co mpletio n o f their first year o f life, and PKU2 after this ag e. PKU2 has half the amo unt o f iro n and o ne third o f the amo unts o f z inc and co pper o f PKU1 . O n the o ther hand, as the g ro up ag e increases, o ther
natural fo o ds are added. Thus, the intake o f trace elements do es no t meet the reco mmended level,1 9 leading to lo wer g ro wth.
CONCLUSION
The se d a ta sug g e st tha t furthe r stud ie s sho uld b e c a rrie d o ut w ith lo ng itud ina l o bservatio n o f PKU children. The amo unts o f trace elements, especially z inc, in special fo rmulae sho uld be revised in o rder to adapt the diet better to RDA and to the evaluatio n o f g ro wth channels.
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Regina M a ra Fisberg - Department o f N utritio n, Public Health Scho o l, University o f São Paulo .
M a ria Eugênia da Silva -Ferna ndes – Department o f Bio chemistry, Federal University o f São Paulo .
Benja m im José Schm idt - Department o f Pediatrics, Federal University o f São Paulo .
M a uro Fisberg - MD, PhD. Department o f Pediatrics, Federal University o f São Paulo .
Sources of funding: CN Pq, FIN EP (Braz il), FAPESP Conflict of interest: N o t declared
La st received: 3 February 1 9 9 9 Accepted: 1 1 March 1 9 9 9 Address for correspondence: Reg ina Mara Fisberg
R. Silva Co rreia, 8 8 / 1 1 3
São Paulo / SP - Brasil - CEP 0 4 5 3 7 -0 4 0 E-mail: rfisberg @ usp.br
RESUMO
Contex to: A restrição dietética da fenilalanina (FAL) é a fo rma mais efetiva para reduzir o seu excesso no sang ue e o único méto do efetivo de tratar a fenilceto núria. A dieta é co mplexa e deve ser adaptada para co mbinar hábito s alimentares, crescimento e desenvo lvimento do s pacientes. Depende basicamente do uso de pro duto s industrializado s co mo substituto s pro téico s isento s de FAL, não to talmente suplementado s. O bjetivo: Avaliar o estado nutricio nal das crianças fenilceto núricas po r medidas antro po métricas e o co nsumo alimentar. Tipo de estudo: Estudo transversal Loca l: Asso ciação de Pais e Amig o s do s Excepcio nais (APAE) de São Paulo , Creche e Centro de Juventude da Prefeitura de São Paulo . Pa rticipa ntes: 4 2 crianças fenilceto núricas e 3 1 no rmais, na faixa etária de 1 a 1 2 ano s, divididas em do is g rupo s abaixo e acima de 7 ano s, de ambo s o s sexo s. Resulta dos: As fenilceto núricas apresentaram po rcentag em de adequação da ing estão de calo rias, cálcio , ferro , zinco e co bre abaixo do reco mendado . A ing estão pro téica estava dentro da no rmalidade. O co nsumo alimentar no g rupo de crianças no rmais apresento u-se dentro da faixa da no rmalidade. As médias de peso para estatura em esco re z das fenilceto núricas fo ram 0 .4 7 < 7 ano s e 1 .8 6 ≥ 7 ano s; nas no rmais 0 .9 7 < 7 ano s e 1 .5 4 ≥ 7 ano s, sem diferença estatisticamente sig nificante. As médias de E/ I apresentaram diferença estatisticamente sig nificante entre fenilceto núricas < 7 ano s (- 1 .2 3 ) e crianças no rmais de mesma faixa etária (0 ,9 1 ). Conclusã o: Co ncluiu-se que a vig ilância nutricio nal em crianças fenilceto núricas é impo rtante para manutenção do s padrõ es de crescimento e o adequado co nsumo de nutrientes.
