<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>2182-7230</journal-id>
<journal-title><![CDATA[Revista Nutrícias]]></journal-title>
<abbrev-journal-title><![CDATA[Nutrícias]]></abbrev-journal-title>
<issn>2182-7230</issn>
<publisher>
<publisher-name><![CDATA[Associação Portuguesa dos Nutricionistas]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2182-72302014000300004</article-id>
<title-group>
<article-title xml:lang="pt"><![CDATA[Dieta Cetogénica - Abordagem Nutricional]]></article-title>
<article-title xml:lang="en"><![CDATA[Ketogenic Diet - Nutritional Approach]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Vasconcelos]]></surname>
<given-names><![CDATA[Carla]]></given-names>
</name>
<xref ref-type="aff" rid="A01"/>
</contrib>
</contrib-group>
<aff id="A01">
<institution><![CDATA[,Centro Hospitalar de São João Unidade de Nutrição e Dietética ]]></institution>
<addr-line><![CDATA[Porto ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>09</month>
<year>2014</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>09</month>
<year>2014</year>
</pub-date>
<numero>22</numero>
<fpage>16</fpage>
<lpage>19</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S2182-72302014000300004&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S2182-72302014000300004&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S2182-72302014000300004&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="pt"><p><![CDATA[A dieta cetogénica, proposta por Wilder em 1921, é um tratamento não farmacológico utilizado na epilepsia refratária à terapêutica anti-epiléptica em indivíduos com esta patologia, essencialmente crianças. Caracteriza-se por uma dieta rica em gordura e pobre em hidratos de carbono, com o fim de simular o estado de jejum prolongado. O seu mecanismo de acção não está completamente desvendado, porém são evidentes os efeitos anti-epilépticos observados. Cerca de um terço a metade dos doentes tratados com esta dieta apresentam marcada redução das crises epilépticas ou mesmo a sua cessação. Actualmente, a dieta cetogénica é indicada para o tratamento de vários síndromes epilépticos e outras patologias, sendo considerada como terapêutica de primeira linha na deficiência do transportador de glicose tipo 1 e na deficiência de desidrogénase do piruvato. Tal como noutras terapias, a dieta cetogénica não é isenta de efeitos laterais. Desidratação, hipoglicemia, obstipação, recusa alimentar, litíase renal, hipercolesterolemia, hipertrigliceridemia, atraso de crescimento, entre outros, são alguns dos efeitos laterais observados. Contudo, na maioria dos casos não é necessário interromper o tratamento. Desde o surgimento da dieta cetogénica clássica, têm surgido outras alternativas à mesma, com o objectivo de a tornar menos restritiva, mais fácil de implementar e cumprir. O aparecimento da dieta cetogénica suplementada com triglicerídeos de cadeia média, da dieta modificada de Atkins e da dieta com baixo índice glicémico são exemplos dessas alternativas. Apesar de haver ainda algumas lacunas sobre a sua forma de actuação, esta pode ser, em alguns casos, eficaz, devendo ser considerada como hipótese no tratamento da epilepsia refratária a outras terapêuticas.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[The ketogenic diet is a nonpharmacologic treatment for intractable epilepsy, proposed by Wilder in 1921. It is a diet based on high fat and low carbohydrate, which mimic the metabolism of fasting. The mechanism of action of the ketogenic diet has not been fully elucidated. However, the antiepileptic effects are evident. About one third to half of the patients treated with the ketogenic diet appeared to have had a marked reduction or cessation of their epileptic crisis. Currently, the ketogenic diet is indicated for the treatment of several epilepsy syndromes and other childhood disorders. This diet is an appropriate first line therapy for patients with glucose transporter deficiency syndrome type 1 and pyruvate dehydrogenase deficiency. There are adverse effects present in this treatment, as well as in others. Dehydration, hypoglycemia, constipation, refusal to eat, kidney stones, hypercholesterolemia, hypertriglyceridemia, growth retardation are some of the side effects reported. Nevertheless, the interruption of the treatment is not necessary in most of the cases. There are new alternatives to the classic ketogenic diet that are less restrictive and easier to implement and maintain. Some of the examples of these alternatives are the medium-chain triglyceride ketogenic therapy, the modified Atkins diet and the low glycemic index treatment. Although there are some gaps on its form of actuation, in some cases this treatment can be efficient and must be considered as an hypothesis in the treatment of intractable childhood epilepsy, as well as in other disorders.]]></p></abstract>
<kwd-group>
<kwd lng="pt"><![CDATA[Corpos cetónicos]]></kwd>
<kwd lng="pt"><![CDATA[Dieta cetogénica]]></kwd>
<kwd lng="pt"><![CDATA[Epilepsia]]></kwd>
<kwd lng="en"><![CDATA[Ketone bodies]]></kwd>
<kwd lng="en"><![CDATA[Ketogenic diet]]></kwd>
<kwd lng="en"><![CDATA[Epilepsy]]></kwd>
</kwd-group>
</article-meta>
</front><body><![CDATA[ <p align="right"><b>CIENTIFICIDADES - ARTIGO DE REVIS&#195;O</p></b> <br/>     <p><b >Dieta Cetog&#233;nica - Abordagem Nutricional</b>     <p><b >Ketogenic Diet - Nutritional Approach</b>     <p>&nbsp;</p>     <p> <b >Marta Rola<sup>1</sup>; Carla Vasconcelos<sup>2</sup></b>     <p ><sup>1</sup>Nutricionista <br/><sup>2</sup>Nutricionista da Unidade de Nutri&#231;&#227;o e Diet&#233;tica do Centro Hospitalar de S&#227;o Jo&#227;o, Alameda Professor Hern&#226;ni Monteiro, 4200-319 Porto, Portugal </p>     <p><a href="#c0">Endere&#231;o para correspond&#234;ncia</a><a name="topc0"></a></b></p>     <p>&nbsp;</p>     <p><b >RESUMO</b></p>     <p>  <br/>A dieta cetog&#233;nica, proposta por Wilder em 1921, &#233; um tratamento n&#227;o farmacol&#243;gico utilizado na epilepsia refrat&#225;ria &#224; terap&#234;utica anti-epil&#233;ptica em indiv&#237;duos com esta patologia, essencialmente crian&#231;as. Caracteriza-se por uma dieta rica em gordura e pobre em hidratos de carbono, com o fim de simular o estado de jejum prolongado. O seu mecanismo de ac&#231;&#227;o n&#227;o est&#225; completamente desvendado, por&#233;m s&#227;o evidentes os efeitos anti-epil&#233;pticos observados. Cerca de um ter&#231;o a metade dos doentes tratados com esta dieta apresentam marcada redu&#231;&#227;o das crises epil&#233;pticas ou mesmo a sua cessa&#231;&#227;o. <br/>Actualmente, a dieta cetog&#233;nica &#233; indicada para o tratamento de v&#225;rios s&#237;ndromes epil&#233;pticos e outras patologias, sendo considerada como terap&#234;utica de primeira linha na defici&#234;ncia do transportador de glicose tipo 1 e na defici&#234;ncia de desidrog&#233;nase do piruvato.  <br/>Tal como noutras terapias, a dieta cetog&#233;nica n&#227;o &#233; isenta de efeitos laterais. Desidrata&#231;&#227;o, hipoglicemia, obstipa&#231;&#227;o, recusa alimentar, lit&#237;ase renal, hipercolesterolemia, hipertrigliceridemia, atraso de crescimento, entre outros, s&#227;o alguns dos efeitos laterais observados. Contudo, na maioria dos casos n&#227;o &#233; necess&#225;rio interromper o tratamento. <br/>Desde o surgimento da dieta cetog&#233;nica cl&#225;ssica, t&#234;m surgido outras alternativas &#224; mesma, com o objectivo de a tornar menos restritiva, mais f&#225;cil de implementar e cumprir. O aparecimento da dieta cetog&#233;nica suplementada com triglicer&#237;deos de cadeia m&#233;dia, da dieta modificada de Atkins e da dieta com baixo &#237;ndice glic&#233;mico s&#227;o exemplos dessas alternativas.  <br/>Apesar de haver ainda algumas lacunas sobre a sua forma de actua&#231;&#227;o, esta pode ser, em alguns casos, eficaz, devendo ser considerada como hip&#243;tese no tratamento da epilepsia refrat&#225;ria a outras terap&#234;uticas. </p>     ]]></body>
<body><![CDATA[<p><b >Palavras-Chave</b>:Corpos cet&#243;nicos, Dieta cetog&#233;nica, Epilepsia </p>     <p>&nbsp;</p>  <hr>     <p>&nbsp;</p>     <p><b >ABSTRACT</b> </p>      <p>The ketogenic diet is a nonpharmacologic treatment for intractable epilepsy, proposed by Wilder in 1921. It is a diet based on high fat and low carbohydrate, which mimic the metabolism of fasting. The mechanism of action of the ketogenic diet has not been fully elucidated. However, the antiepileptic effects are evident. About one third to half of the patients treated with the ketogenic diet appeared to have had a marked reduction or cessation of their epileptic crisis. <br/>Currently, the ketogenic diet is indicated for the treatment of several epilepsy syndromes and other childhood disorders. This diet is an appropriate first line therapy for patients with glucose transporter deficiency syndrome type 1 and pyruvate dehydrogenase deficiency. <br/>There are adverse effects present in this treatment, as well as in others. Dehydration, hypoglycemia, constipation, refusal to eat, kidney stones, hypercholesterolemia, hypertriglyceridemia, growth retardation are some of the side effects reported. Nevertheless, the interruption of the treatment is not necessary in most of the cases. <br/>There are new alternatives to the classic ketogenic diet that are less restrictive and easier to implement and maintain. Some of the examples of these alternatives are the medium-chain triglyceride ketogenic therapy, the modified Atkins diet and the low glycemic index treatment. <br/>Although there are some gaps on its form of actuation, in some cases this treatment can be efficient and must be considered as an hypothesis in the treatment of intractable childhood epilepsy, as well as in other disorders. </p>     <p><b >keywords</b>: Ketone bodies, Ketogenic diet, Epilepsy </p>      <p>&nbsp;</p> <hr>     <p>&nbsp;</p>     <p><b >INTRODU&#199;&#195;O</b>     <br/>A     ]]></body>
<body><![CDATA[dieta cetog&#233;nica (DC) &#233; um tratamento n&#227;o farmacol&#243;gico para a epilepsia     refrat&#225;ria, caracterizado por uma dieta rica em gordura e pobre em hidratos de     carbono (1).     <br/>A     epilepsia afecta um grande n&#250;mero de indiv&#237;duos, sendo um dos dist&#250;rbios     neurol&#243;gicos mais frequentes e graves em idade pedi&#225;trica (2). Caracteriza-se     por dist&#250;rbios parox&#237;sticos da fun&#231;&#227;o cerebral, decorrentes de descargas     neuronais excessivas, s&#250;bitas e temporais, com manifesta&#231;&#227;o cl&#237;nica heterog&#233;nea     e com v&#225;rias etiologias (3). Cerca de 20 a 30% dos indiv&#237;duos com epilepsia     desenvolvem crises convulsivas refrat&#225;rias &#224; administra&#231;&#227;o de f&#225;rmacos (1),     ]]></body>
<body><![CDATA[crises que n&#227;o s&#227;o controladas por doses m&#225;ximas toleradas de 2 ou 3 f&#225;rmacos     anti-epil&#233;pticos (2). As crises epil&#233;pticas refrat&#225;rias representam uma amea&#231;a     para a crian&#231;a, devido ao aumento do risco de acidentes e mortalidade,     malnutri&#231;&#227;o secund&#225;ria, com consequente atraso no desenvolvimento f&#237;sico e     cognitivo, decorrente da doen&#231;a e da terap&#234;utica, cuja efic&#225;cia n&#227;o &#233; a     pretendida (4).     <br/>Uma     das op&#231;&#245;es de tratamento n&#227;o farmacol&#243;gico da epilepsia refrat&#225;ria &#233; a DC (2),     que se tem revelado eficaz na redu&#231;&#227;o da frequ&#234;ncia de crises epil&#233;pticas em     crian&#231;as com esta patologia (4).     ]]></body>
<body><![CDATA[<br/><u>Indica&#231;&#245;es     e Contra-indica&#231;&#245;es da Dieta Cetog&#233;nica</u><u></u>     <br/>Na     &#250;ltima d&#233;cada, a DC tem vindo a ser cada vez mais utilizada no tratamento da     epilepsia refrat&#225;ria (1). Est&#227;o descritos v&#225;rios s&#237;ndromes epil&#233;pticos e outras     patologias em que a DC pode ser particularmente ben&#233;fica. De destacar a     import&#226;ncia desta dieta na terap&#234;utica de primeira linha no tratamento de duas     doen&#231;as metab&#243;licas: defici&#234;ncia do transportador da glicose tipo 1 e     defici&#234;ncia de desidrog&#233;nase do piruvato (5).      <br/>A     ]]></body>
<body><![CDATA[DC tamb&#233;m demonstrou ser &#250;til na epilepsia miocl&#243;nica, nomeadamente no S&#237;ndrome     de Dravet e no S&#237;ndrome de Doose, na esclerose tuberosa complexa, no S&#237;ndrome     de Rett e nos espasmos infantis. Estudos realizados tamb&#233;m referiram que a DC     poder&#225; ter efeitos ben&#233;ficos na doen&#231;a de Lafora, na panencefalite subaguda     esclerosante, no S&#237;ndrome de Landau-Kleffner, na glicogenose tipo V e em     doen&#231;as da cadeia respirat&#243;ria mitocondrial (1).     <br/>Antes     de iniciar a DC &#233; de extrema import&#226;ncia a avalia&#231;&#227;o do doente para excluir a     presen&#231;a de qualquer patologia em que seja contra-indicado este tratamento. Na     <a href ="/img/revistas/nut/n22/n22a04t1.jpg">Tabela 1</a> est&#227;o descritas as contra-indica&#231;&#245;es para o uso desta dieta. Na DC a     
]]></body>
<body><![CDATA[principal fonte de energia prov&#233;m dos l&#237;pidos e, por essa raz&#227;o, o doente n&#227;o     poder&#225; ter qualquer patologia relacionada com o transporte ou com a oxida&#231;&#227;o     dos &#225;cidos gordos. Este tratamento tamb&#233;m est&#225; contra-indicado na defici&#234;ncia     de carbox&#237;lase do piruvato e em porfirias (1).      <br/><u>Prescri&#231;&#227;o     da Dieta Cetog&#233;nica</u><u></u>     <br/>Antes     de iniciar a DC &#233; importante identificar o tipo de crises, efectuar uma     avalia&#231;&#227;o laboratorial e nutricional, excluir a exist&#234;ncia de     contra-indica&#231;&#245;es, e avaliar poss&#237;veis factores que possam dificultar o sucesso     ]]></body>
<body><![CDATA[da dieta (lit&#237;ase renal, dislipidemia, doen&#231;a hep&#225;tica, acidose metab&#243;lica     cr&#243;nica, entre outros)(1, 6). De igual modo, a discuss&#227;o de quest&#245;es     psicossociais e a avalia&#231;&#227;o do ambiente familiar s&#227;o de extrema import&#226;ncia     para o sucesso da DC (1, 7). Os pais e/ ou cuidadores da crian&#231;a devem compreender     perfeitamente a aplica&#231;&#227;o da dieta, a necessidade do seu cumprimento rigoroso,     os cuidados a ter e os efeitos secund&#225;rios que podem ocorrer (1, 7).      <br/>O     c&#225;lculo da dieta &#233; realizado de forma personalizada. Tradicionalmente, o valor     energ&#233;tico total di&#225;rio dever&#225; ser de 80 a 90% das necessidades energ&#233;ticas     di&#225;rias recomendadas para a idade, de forma a garantir que a obten&#231;&#227;o de     ]]></body>
<body><![CDATA[energia seja feita a partir do metabolismo dos l&#237;pidos (1). A DC cl&#225;ssica &#233;     calculada atrav&#233;s de uma raz&#227;o entre a quantidade de gordura, em gramas, e o     somat&#243;rio das quantidades de prote&#237;nas e hidratos de carbono, tamb&#233;m em gramas     (1). As raz&#245;es normalmente utilizadas s&#227;o de 3:1 e de 4:1, ou seja 3 ou 4     gramas de gordura, para 1 grama de prote&#237;nas e hidratos de carbono, respectivamente.     Habitualmente, em crian&#231;as dos 2 aos 11 anos inicia-se a DC com uma raz&#227;o de     4:1, e em crian&#231;as com menos de 2 anos ou clinicamente fr&#225;geis inicia-se com     uma raz&#227;o de 3:1 a 3,5:1 (permitindo uma maior quantidade de prote&#237;nas), bem     como em crian&#231;as com mais de 11 anos ou que apresentem valores de &#237;ndice de     massa corporal (IMC) para a idade iguais ou superiores ao percentil 95 (7).     ]]></body>
<body><![CDATA[<br/>A     quantidade de prote&#237;na &#233; calculada de acordo com as necessidades do indiv&#237;duo,     de forma a permitir o seu crescimento adequado. A gordura e os hidratos de     carbono s&#227;o calculados tendo em conta a raz&#227;o estipulada (8), sendo que a     quantidade de hidratos de carbono dever&#225; ser no m&#237;nimo de 10g/dia (9). Na     <a href ="/img/revistas/nut/n22/n22a04t2.jpg">Tabela 2</a> encontra-se a distribui&#231;&#227;o do valor energ&#233;tico total pelos diferentes     
macronutrientes numa DC cl&#225;ssica com uma raz&#227;o de 4:1, bem como noutras dietas     cetog&#233;nicas (10). Normalmente, na DC s&#227;o realizadas 3 a 4 refei&#231;&#245;es por dia, em     que cada uma dever&#225; conter a raz&#227;o estipulada correcta. As refei&#231;&#245;es realizadas     na DC cl&#225;ssica dever&#227;o incluir alimentos ricos em prote&#237;na de alto valor     ]]></body>
<body><![CDATA[biol&#243;gico, baixa quantidade de frutas e vegetais e grande quantidade de     gordura. Um dos alimentos base da dieta s&#227;o as natas, que podem fornecer,     aproximadamente, cerca de 50% do total de l&#237;pidos calculado (7). Tamb&#233;m s&#227;o     utilizados alimentos como manteiga, &#243;leos, maionese, carne e peixe gordos (11).     Assim, a DC cl&#225;ssica &#233; constitu&#237;da maioritariamente por triglicer&#237;deos de     cadeia longa (12). Todos os alimentos dever&#227;o ser pesados e as refei&#231;&#245;es s&#227;o     tendencialmente pequenas (1).      <br/>O     m&#233;todo tradicional de inicia&#231;&#227;o da DC envolve um per&#237;odo de jejum, no qual o     doente apenas poder&#225; ingerir &#225;gua ou bebidas n&#227;o a&#231;ucaradas, ainda que com     ]]></body>
<body><![CDATA[restri&#231;&#227;o (1, 13, 14). Este jejum tem como finalidade promover um estado de     cetose no doente. O jejum dever&#225; ter uma dura&#231;&#227;o entre 12h a 72h no m&#225;ximo,     durante o qual a glicemia deve ser monitorizada (1, 14). O estado de cetose &#233;     confirmado atrav&#233;s do valor de corpos cet&#243;nicos urin&#225;rios, que dever&#225; estar     entre 60-80mg/dL (4).      <br/>A     necessidade de jejum inicial para a efic&#225;cia da DC tem sido posta em causa.     Tem-se verificado que o tempo necess&#225;rio para o estabelecimento do estado de     cetose &#233; semelhante ao observado nos protocolos em que &#233; feito jejum inicial ou     n&#227;o (15). Por outro lado, a aus&#234;ncia de jejum tem menos efeitos adversos,     ]]></body>
<body><![CDATA[nomeadamente, hipoglicemia, acidose, perda de peso, e a DC &#233; melhor tolerada     (1, 16). Alguns centros continuam a iniciar a DC com jejum, pois parece que o     tempo necess&#225;rio para a diminui&#231;&#227;o do n&#250;mero de crises epil&#233;ticas &#233; mais curto     (17). A restri&#231;&#227;o de fluidos inicial tamb&#233;m parece n&#227;o ser obrigat&#243;ria para o     sucesso da DC (18).     <br/>Ap&#243;s     o per&#237;odo de jejum, a DC &#233; introduzida gradualmente, em intervalos de 1/3 at&#233; o     valor cal&#243;rico total das refei&#231;&#245;es ser tolerado. Outra abordagem realizada     consiste em iniciar a DC com o valor cal&#243;rico total, mas aumentar gradualmente     a raz&#227;o de 1:1; 2:1; 3:1 a 4:1, permitindo que o doente se adapte &#224; quantidade     ]]></body>
<body><![CDATA[de gordura (1).     <br/>Todo     o processo inicial de administra&#231;&#227;o da DC &#233; realizado habitualmente em     internamento. O internamento, com uma dura&#231;&#227;o de cerca de 5 dias, constitui um     per&#237;odo importante na aprendizagem por parte dos pais e/ou cuidadores sobre a     DC, o c&#225;lculo das refei&#231;&#245;es, a pesagem dos alimentos, a leitura de r&#243;tulos dos     alimentos e os cuidados a ter no caso de surgimento de infec&#231;&#245;es (14). Por&#233;m, a     necessidade de internamento inicial tem sido questionada. J&#225; foi demonstrado     ser poss&#237;vel iniciar a DC sem internamento obtendo igualmente melhoria das     crises epil&#233;pticas (15, 19). O in&#237;cio do tratamento em ambulat&#243;rio permite     ]]></body>
<body><![CDATA[garantir um ambiente mais calmo para o doente e a redu&#231;&#227;o dos custos com a     hospitaliza&#231;&#227;o (1). Contudo, mais estudos s&#227;o necess&#225;rios, de prefer&#234;ncia     prospectivos, para confirmar estes dados (19).      <br/>A     DC n&#227;o &#233; uma dieta equilibrada por implicar limita&#231;&#245;es na alimenta&#231;&#227;o. Os     grandes fornecedores de vitaminas e minerais, como a fruta, os hort&#237;colas e os     cereais, bem como os alimentos ricos em c&#225;lcio, s&#227;o exemplos de alimentos com     menor representa&#231;&#227;o nesta dieta. Por essa raz&#227;o, &#233; essencial a suplementa&#231;&#227;o de     vitaminas do complexo B e c&#225;lcio (1), bem como de vitamina D, n&#227;o s&#243; pela     alimenta&#231;&#227;o, mas tamb&#233;m pela tend&#234;ncia a valores baixos de vitamina D que     ]]></body>
<body><![CDATA[crian&#231;as com epilepsia apresentam (20). Todos os suplementos vitam&#237;nicos e     minerais t&#234;m de ser isentos de hidratos de carbono, com o objectivo de manter o     estado de cetose (1). Na pr&#225;tica, recorre-se geralmente a um suplemento     polivitam&#237;nico com minerais, de forma a cobrir todas as necessidades em micronutrientes     (11, 21).     <br/><u>Mecanismo     da Dieta Cetog&#233;nica</u><u></u>     <br/>Apesar     da DC ser utilizada no tratamento da epilepsia h&#225; mais de 80 anos, o mecanismo     subjacente &#224; sua efic&#225;cia ainda n&#227;o &#233; totalmente conhecido (22). V&#225;rios estudos     ]]></body>
<body><![CDATA[t&#234;m sido realizados no sentido de descobrir qual o mecanismo subjacente ao     efeito anti-epil&#233;ptico da dieta. Algumas hip&#243;teses t&#234;m sido colocadas,     nomeadamente: mudan&#231;as no pH cerebral; altera&#231;&#245;es no balan&#231;o     hidroelectrol&#237;tico; efeito inibit&#243;rio directo dos &#225;cidos gordos, nomeadamente     dos &#225;cidos gordos polinsaturados; altera&#231;&#245;es de neurotransmissores; mudan&#231;as no     metabolismo energ&#233;tico, relacionadas, em parte, com a produ&#231;&#227;o e metabolismo de     corpos cet&#243;nicos (6). O mecanismo de actua&#231;&#227;o dos corpos cet&#243;nicos ainda n&#227;o     est&#225; esclarecido (12, 23). Estes poder&#227;o ter uma actividade antiepil&#233;ptica     directa ou actuarem de forma a estabilizar as membranas neuronais, atenuando a     actividade el&#233;ctrica anormal (12). Por outro lado, os &#225;cidos gordos     ]]></body>
<body><![CDATA[polinsaturados parecem regular a excitabilidade das membranas neuronais atrav&#233;s     do bloqueio dos canais de c&#225;lcio ou dos canais de s&#243;dio dependentes de     voltagem. Contudo, alguns estudos contrariam esta hip&#243;tese (12).     <br/>Dadas     as altera&#231;&#245;es metab&#243;licas, fisiol&#243;gicas e hormonais que ocorrem nesta dieta,     parece que n&#227;o &#233; um &#250;nico mecanismo que explicar&#225; todos os efeitos cl&#237;nicos da     DC, mas sim um conjunto de mecanismos que actuam paralelamente e possivelmente     de forma sin&#233;rgica (6).     <br/><u>Efeitos     Laterais da Dieta Cetog&#233;nica</u><u></u>     ]]></body>
<body><![CDATA[<br/>A     ideia de que a DC &#233; um tratamento &#8220;natural&#8221;, e como tal n&#227;o tem efeitos     adversos, comparando com os f&#225;rmacos anti-epil&#233;pticos, deve ser desmistificada     (24).      <br/>As     complica&#231;&#245;es iniciais, mais frequentes, ainda durante o tempo de hospitaliza&#231;&#227;o     s&#227;o: desidrata&#231;&#227;o, hipoglicemia, v&#243;mitos, diarreia, obstipa&#231;&#227;o e recusa     alimentar (1, 25, 26). De entre as complica&#231;&#245;es a longo prazo destacam-se a     lit&#237;ase renal (27), infec&#231;&#245;es recorrentes, acidose, hiperuricemia,     hipocalcemia, hipoproteinemia, hipercolesterolemia, hipertrigliceridemia,     ]]></body>
<body><![CDATA[irritabilidade, letargia, recusa alimentar/ anorexia e tamb&#233;m j&#225; foram     descritos casos de cardiomiopatia (1, 25, 26). O atraso de crescimento &#233; outra     potencial complica&#231;&#227;o. Aparentemente a DC fornece os nutrientes necess&#225;rios &#224;     manuten&#231;&#227;o do crescimento dentro dos par&#226;metros normais, especialmente em     crian&#231;as mais velhas (28), por&#233;m, &#233; poss&#237;vel ocorrer atraso de crescimento em     algumas crian&#231;as (29). O conte&#250;do mineral &#243;sseo tamb&#233;m parece estar diminu&#237;do,     sobretudo em crian&#231;as mais novas e com um IMC mais baixo (30). Desta forma, a     monitoriza&#231;&#227;o do crescimento em crian&#231;as com DC reveste-se de extrema     import&#226;ncia, bem como o ajuste das necessidades energ&#233;ticas e proteicas, de     forma a permitir o seu crescimento e ao mesmo tempo manter o controlo das     ]]></body>
<body><![CDATA[crises epil&#233;pticas (28, 29, 31).     <br/>Relativamente     a complica&#231;&#245;es graves, estas parecem ser raras (24). Ballaban-Gil et al     reportaram que num conjunto de 52 crian&#231;as a fazer DC, cinco desenvolveram     complica&#231;&#245;es graves, nomeadamente hipoproteinemia, S&#237;ndrome de Fanconi e     altera&#231;&#245;es hep&#225;ticas, sendo que apenas uma crian&#231;a teve de interromper a dieta     (25).     <br/>A     DC pode causar diversas complica&#231;&#245;es, mas na maioria das situa&#231;&#245;es estas podem     ser resolvidas sem necessidade de a interromper. Contudo, &#233; importante evitar     ]]></body>
<body><![CDATA[ao m&#225;ximo as complica&#231;&#245;es graves que possam levar &#224; sua interrup&#231;&#227;o, como     algumas infec&#231;&#245;es, pneumonia lip&#243;ide devido a aspira&#231;&#227;o, cardiomiopatias, entre     outras que p&#245;em a vida em risco. Assim, &#233; importante a detec&#231;&#227;o pr&#233;via de     doen&#231;as graves e uma interven&#231;&#227;o activa, bem como um suporte nutricional     adequado (26).     <br/><u>Monitoriza&#231;&#227;o</u>     <br/>Ap&#243;s     a alta hospitalar ou o in&#237;cio da DC em ambulat&#243;rio &#233; de extrema import&#226;ncia o     seguimento do doente para garantir o poss&#237;vel controlo das crises epil&#233;pticas,     um bom estado nutricional e de sa&#250;de. Para al&#233;m da equipa multidisciplinar,     ]]></body>
<body><![CDATA[constitu&#237;da por M&#233;dico, Nutricionista, Enfermeiro e outros profissionais de     sa&#250;de, a fam&#237;lia &#233; um elemento chave para o sucesso do tratamento (7).      <br/>Inicialmente,     o doente dever&#225; ser visto a cada 3 meses. Ap&#243;s um ano a cumprir esta dieta, o     seguimento dever&#225; ser realizado a cada 6 meses (1). Em cada visita dever&#225; ser     efectuada a avalia&#231;&#227;o do crescimento, da frequ&#234;ncia das crises epil&#233;pticas, de     par&#226;metros laboratoriais e de altera&#231;&#245;es do tr&#226;nsito intestinal (nomeadamente,     obstipa&#231;&#227;o e esteatorreia) (7). Entre as visitas, os pais e/ou cuidadores     dever&#227;o vigiar os corpos cet&#243;nicos urin&#225;rios v&#225;rias vezes por semana (1),     medidos atrav&#233;s da imers&#227;o de tiras teste na urina (32). O resultado de cetose     ]]></body>
<body><![CDATA[urin&#225;ria necess&#225;ria, mas n&#227;o for&#231;osamente suficiente, para atingir um controlo     &#243;ptimo das crises, ser&#225; de 3+ a 4+ (80-160mmol/L)(33). Tamb&#233;m dever&#227;o registar     o n&#250;mero de crises epil&#233;pticas.      <br/><u>Dura&#231;&#227;o     e Efic&#225;cia da Dieta Cetog&#233;nica</u><u></u>     <br/>A     dura&#231;&#227;o da dieta n&#227;o &#233; igual em todos os casos, nem o m&#233;todo de descontinua&#231;&#227;o.     &#201; aconselhado que a crian&#231;a permane&#231;a na DC pelo menos durante 3 meses (1). Se     n&#227;o se observar diminui&#231;&#227;o do n&#250;mero crises epil&#233;pticas at&#233; este momento, a     dieta dever&#225; ser descontinuada. Se se obtiver uma redu&#231;&#227;o da frequ&#234;ncia das     ]]></body>
<body><![CDATA[crises epil&#233;pticas superior a 50%, a dieta poder&#225; ser interrompida ap&#243;s 2 anos     (1), por&#233;m, j&#225; foram relatados casos em que a DC foi realizada durante 6 a 12     anos (34). Martinez et al observaram que em crian&#231;as sem crises epil&#233;pticas     durante a DC, cerca de 80% permaneceram sem crises ap&#243;s suspenderem o     tratamento (35), demonstrando que poder&#225; haver continua&#231;&#227;o da aus&#234;ncia das     crises. A interrup&#231;&#227;o da DC dever&#225; ser gradual, durante 2 a 3 meses, diminuindo     a raz&#227;o de 4:1, para 3:1 e para 2:1; mantendo-se de seguida a ingest&#227;o de alimentos     cetog&#233;nicos, aumentando-se as calorias e os fluidos sem restri&#231;&#245;es. Na aus&#234;ncia     de cetose urin&#225;ria, o doente reinicia uma alimenta&#231;&#227;o sem limita&#231;&#245;es (1).      <br/>Diversos     ]]></body>
<body><![CDATA[estudos t&#234;m sido realizados com o intuito de avaliar a efic&#225;cia da DC no     tratamento da epilepsia refrat&#225;ria. A maioria destes s&#227;o retrospectivos e     come&#231;aram a surgir na d&#233;cada de 20 e 30 do s&#233;culo anterior. De uma forma geral,     cerca de um ter&#231;o a metade dos doentes parecem ter uma boa resposta &#224; DC,     ocorrendo cessa&#231;&#227;o ou marcada redu&#231;&#227;o da frequ&#234;ncia das crises epil&#233;pticas (4).     Vining et al, num estudo prospectivo n&#227;o randomizado, relataram a efic&#225;cia da     DC na redu&#231;&#227;o da frequ&#234;ncia das crises, e tendo em conta que cerca de 40% das     crian&#231;as tiveram uma redu&#231;&#227;o de mais de 50% do n&#250;mero de crises ap&#243;s 1 ano de     tratamento, exclu&#237;ram o efeito placebo, evidenciando assim o poss&#237;vel efeito     terap&#234;utico da DC (14). Noutro estudo prospectivo, Freeman et al, relataram uma     ]]></body>
<body><![CDATA[diminui&#231;&#227;o do n&#250;mero de crises superior a 50% em 59% dos casos ao fim de 3 meses     com DC, sendo esta mais eficaz do que v&#225;rios f&#225;rmacos anti-epil&#233;pticos recentes     (36). Apesar dos v&#225;rios estudos j&#225; realizados, n&#227;o existe muita evid&#234;ncia     baseada em estudos randomizados. Por&#233;m, um estudo prospectivo randomizado     recente, concluiu a efic&#225;cia da DC no tratamento da epilepsia refrat&#225;ria em     crian&#231;as (37).      <br/>Uma     revis&#227;o sistem&#225;tica relatou que apesar de faltarem estudos controlados, existe     evid&#234;ncia suficiente para determinar que a DC &#233; eficaz na redu&#231;&#227;o da frequ&#234;ncia     das crises em crian&#231;as com epilepsia refrat&#225;ria (38). Mais recentemente, outra     ]]></body>
<body><![CDATA[revis&#227;o sistem&#225;tica relatou que cerca de 15,6% dos doentes tratados com DC     demonstraram aus&#234;ncia de crises epil&#233;pticas e que cerca de 33% teve uma redu&#231;&#227;o     superior a 50% do n&#250;mero de crises (39).      <br/>Em     Portugal apenas um estudo foi ainda publicado relatando a experi&#234;ncia de um     servi&#231;o de Pediatria no tratamento de crian&#231;as com epilepsia refrat&#225;ria com DC,     tendo os resultados apoiado a utiliza&#231;&#227;o da mesma (40).     <br/><u>Alternativas     &#224; Dieta Cetog&#233;nica Cl&#225;ssica</u><u></u>     <br/>A     ]]></body>
<body><![CDATA[DC por si s&#243; exige uma altera&#231;&#227;o dr&#225;stica da alimenta&#231;&#227;o e, normalmente, pode     n&#227;o ser muito bem tolerada no in&#237;cio. Por outro lado, &#233; um regime alimentar     muito exigente, uma vez que &#233; necess&#225;ria a quantifica&#231;&#227;o dos v&#225;rios alimentos     com vista a controlar as crises epil&#233;pticas. Ao longo do tempo t&#234;m surgido     outras alternativas &#224; DC tradicional, com vista a aumentar a qualidade de vida     do doente e da fam&#237;lia. Estas s&#227;o mais liberais, mas com efeito semelhante no     controlo das crises. Uma das primeiras alternativas &#224; DC tradicional foi a DC     suplementada com triglicer&#237;deos de cadeia m&#233;dia, que t&#234;m um efeito mais     cetog&#233;nico que os de cadeia longa, presentes na DC cl&#225;ssica (1, 41). A dieta     modificada de Atkins foi criada com o intuito de ser menos restritiva e     ]]></body>
<body><![CDATA[aumentar a palatibilidade, especialmente para crian&#231;as com problemas     comportamentais e adolescentes em que os pais e neurologistas est&#227;o relutantes     em iniciar a DC (42). Esta pode ser iniciada em casa sem jejum, nem restri&#231;&#227;o     cal&#243;rica e h&#237;drica (11). Neste caso, os hidratos de carbono s&#227;o restritos a     10g/dia em crian&#231;as e 20g/dia em adultos, sendo que o consumo de gordura &#233;     encorajado, de forma a manter o estado de cetose (11). Por &#250;ltimo, a dieta de     baixo &#237;ndice glic&#233;mico &#233; outra alternativa apresentada, que permite uma maior     liberaliza&#231;&#227;o da quantidade total de hidratos de carbono, mas restrita a     alimentos com baixo &#237;ndice glic&#233;mico (inferior a 50), de forma a manter n&#237;veis     glic&#233;micos est&#225;veis (11, 43). Esta alternativa tamb&#233;m poder&#225; ser iniciada em     ]]></body>
<body><![CDATA[casa, sem o jejum inicial (11). Na <a href ="/img/revistas/nut/n22/n22a04t2.jpg">Tabela 2</a> encontra-se a distribui&#231;&#227;o     
energ&#233;tica pelos diferentes macronutrientes nas tr&#234;s alternativas &#224; DC cl&#225;ssica     anteriormente referidas.     </p>     <p><b >AN&#193;LISE CR&#205;TICA</b> <br/>A DC &#233; a prova de que a alimenta&#231;&#227;o pode ser utilizada como forma terap&#234;utica, por vezes com melhores resultados que os f&#225;rmacos convencionais.  <br/>Os efeitos laterais que podem advir da DC poder&#227;o ser muito menores, quando comparados com os efeitos provocados por crises m&#250;ltiplas di&#225;rias e pela multiterapia farmacol&#243;gica. <br/>A DC surge como uma op&#231;&#227;o n&#227;o farmacol&#243;gica no tratamento da epilepsia refrat&#225;ria. A aplica&#231;&#227;o desta dieta &#233; extremamente exigente tanto para o doente, como para a fam&#237;lia, pois implica uma mudan&#231;a radical nos h&#225;bitos alimentares.  <br/>Embora a DC exista h&#225; quase um s&#233;culo e apesar dos muitos estudos realizados, muito ainda est&#225; por decifrar relativamente aos mecanismos, efic&#225;cia, indica&#231;&#245;es e contra-indica&#231;&#245;es, efeitos laterais, entre outros. Mesmo assim, a DC deve surgir como uma boa op&#231;&#227;o terap&#234;utica no tratamento da epilepsia refrat&#225;ria, sendo necess&#225;rias equipas multidisciplinares preparadas para a implementa&#231;&#227;o deste tratamento. </p>     <p>&nbsp;</p>     <!-- ref --><p><b >REFER&#202;NCIAS BIBLIOGR&#193;FICAS</b> <br/>1. Kossoff EH, Zupec-Kania BA, Amark PE, Ballaban-Gil KR, Christina Bergqvist AG, Blackford R, et al. Optimal clinical management of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study Group. Epilepsia. 2009; 50(2):304-17.    &nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;[&#160;<a href="javascript:void(0);" onclick="javascript: window.open('/scielo.php?script=sci_nlinks&ref=1727117&pid=S2182-7230201400030000400001&lng=','','width=640,height=500,resizable=yes,scrollbars=1,menubar=yes,');">Links</a>&#160;]<!-- end-ref --><!-- ref --> <br/>2. Vasconcelos MM, Azevedo PM, Esteves L, Brito AR, Olivaes MC, Herdy GV. [Ketogenic diet for intractable epilepsy in children and adolescents: report of six cases]. Rev Assoc Med Bras. 2004; 50(4):380-5.    &nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;[&#160;<a href="javascript:void(0);" onclick="javascript: window.open('/scielo.php?script=sci_nlinks&ref=1727118&pid=S2182-7230201400030000400002&lng=','','width=640,height=500,resizable=yes,scrollbars=1,menubar=yes,');">Links</a>&#160;]<!-- end-ref --><!-- ref --> <br/>3. Reming VM. Medical Nutrition Therapy for Neurologic Disorders. In: Mahan LK, Escott-Stump S, editores. 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St. Louis: Mosby; 1994. p. 561-74.    &nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;[&#160;<a href="javascript:void(0);" onclick="javascript: window.open('/scielo.php?script=sci_nlinks&ref=1727125&pid=S2182-7230201400030000400009&lng=','','width=640,height=500,resizable=yes,scrollbars=1,menubar=yes,');">Links</a>&#160;]<!-- end-ref --><!-- ref --> <br/>10. Zupec-Kania B, Neal E, Schultz R, Roan ME, Turner Z, Welborn M. An update on diets in clinical practice. J Child Neurol. 2013; 28(8):1015-26.    &nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;[&#160;<a href="javascript:void(0);" onclick="javascript: window.open('/scielo.php?script=sci_nlinks&ref=1727126&pid=S2182-7230201400030000400010&lng=','','width=640,height=500,resizable=yes,scrollbars=1,menubar=yes,');">Links</a>&#160;]<!-- end-ref --><!-- ref --> <br/>11. Kossoff EH, Wang HS. Dietary therapies for epilepsy. Biomed J. 2013; 36(1):2-8.    &nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;[&#160;<a href="javascript:void(0);" onclick="javascript: window.open('/scielo.php?script=sci_nlinks&ref=1727127&pid=S2182-7230201400030000400011&lng=','','width=640,height=500,resizable=yes,scrollbars=1,menubar=yes,');">Links</a>&#160;]<!-- end-ref --><!-- ref --> <br/>12. Dhamija R, Eckert S, Wirrell E. Ketogenic diet. Can J Neurol Sci. 2013; 40(2):158-67.    &nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;[&#160;<a href="javascript:void(0);" onclick="javascript: window.open('/scielo.php?script=sci_nlinks&ref=1727128&pid=S2182-7230201400030000400012&lng=','','width=640,height=500,resizable=yes,scrollbars=1,menubar=yes,');">Links</a>&#160;]<!-- end-ref --><!-- ref --> <br/>13. Carroll J, Koenigsberger D. The ketogenic diet: a practical guide for caregivers. J Am Diet Assoc. 1998; 98(3):316-21.    &nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;[&#160;<a href="javascript:void(0);" onclick="javascript: window.open('/scielo.php?script=sci_nlinks&ref=1727129&pid=S2182-7230201400030000400013&lng=','','width=640,height=500,resizable=yes,scrollbars=1,menubar=yes,');">Links</a>&#160;]<!-- end-ref --><!-- ref --> <br/>14. Vining EP, Freeman JM, Ballaban-Gil K, Camfield CS, Camfield PR, Holmes GL, et al. A multicenter study of the efficacy of the ketogenic diet. Arch Neurol. 1998; 55(11):1433-7.    &nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;[&#160;<a href="javascript:void(0);" onclick="javascript: window.open('/scielo.php?script=sci_nlinks&ref=1727130&pid=S2182-7230201400030000400014&lng=','','width=640,height=500,resizable=yes,scrollbars=1,menubar=yes,');">Links</a>&#160;]<!-- end-ref --><!-- ref --> <br/>15. Wirrell EC, Darwish HZ, Williams-Dyjur C, Blackman M, Lange V. 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<body><![CDATA[<p>&nbsp;</p>     <p>  <b ><a href="#topc0">Endere&#231;o para correspond&#234;ncia</a><a name="c0"></a></b> <br/>Marta Rola  <br/>Rua Louren&#231;o Marques, n&#186;50 &#8211; 2&#186;esq, 4445-498 Ermesinde, Portugal <br/><a href="mailto:pocao.rola@hotmail.com">pocao.rola@hotmail.com</a></p> </p>  <br/>Recebido a 21 de Setembro de 2014 <br/>Aceite a 7 de Outubro de 2014 <br/>       ]]></body><back>
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