thegoodfellas/blogset-br huggingface dataset:
tais como fisioterapeutas, psicologos e terapeutas ocupacionais - que poderao ajuda-lo a entender melhor a doenca e o impacto para voce e para sua familia.O diagnostico da sindrome de Hunter (MPS II)Em geral, os primeiros sinais que levam ao diagnostico da sindrome de Hunter (MPS II) em criancas e jovens sao os sinais e sintomas visiveis. De forma geral, o diagnostico e habitualmente estabelecido entre os dois e quatro anos de idade. Antes de fazer um diagnostico definitivo, os medicos podem realizar testes laboratoriais para medir a atividade da enzima iduronato-2-sulfatase (I2S). A triagem laboratorial mais usada para MPS e um teste de urina para detectar a presenca de GAGs. E importante observar que o teste de urina para GAGs pode ser normal, mesmo quando a crianca e portadora de MPS.O medico pode tambem solicitar um conjunto de raios-X (geralmente das maos, ossos longos e coluna vertebral), a fim de localizar um padrao especifico de desenvolvimento osseo que possa ser sugestivo de um diagnostico da sindrome de Hunter.O diagnostico definitivo da sindrome de Hunter e realizado ao se medir a atividade da I2S no soro, em leucocitos ou em fibroblastos obtidos por biopsia da pele.Em algumas pessoas com sindrome de Hunter, a analise do gene I2S pode determinar a gravidade clinica da doenca. O diagnostico pre-natal esta disponivel como rotina e pode ser realizado atraves da medida da atividade enzimatica da I2S no liquido amniotico ou no tecido das vilosidades corionicas.A genetica da sindrome de Hunter (MPS II)(A sindrome de Hunter (MPS II) tem um padrao de heranca ligado ao cromossomo X. A mae portadora transmitira o gene com a mutacao, causadora da sindrome, com uma probabilidade de 50% a cada gestacao. O pai com a sindrome de Hunter transmitira o gene com a mutacao codificador da I2S para todas as suas filhas e nao transmitira para nenhum dos seus filhos.)Estima-se que a sindrome de Hunter (MPS II) afeta em media 1 em cada 155 mil nascimentos vivos. Como a sindrome e um disturbio hereditario recessivo, ligado ao cromossomo X, afeta principalmente pessoas do sexo masculino. E transmitida de uma geracao para outra de uma forma especifica. Quase todas as celulas no corpo humano tem 46 cromossomos, sendo 23 herdados do pai e 23 herdados da mae. O gene que codifica a producao do I2S esta localizado no cromossomo X. As mulheres tem dois cromossomos X, um herdado do pai e o outro da mae; ja os homens tem um cromossomo X herdado da mae e um cromossomo Y herdado do pai.Se o homem receber uma copia anormal do gene I2S, ele desenvolvera a sindrome de Hunter. Um homem pode herdar uma copia anormal do gene I2S de duas formas diferentes. A mais frequente e quando sua mae e uma portadora, ou seja, ela tem um gene I2S anormal e outro normal, transmitindo o gene anormal para ele. A outra forma e quando ocorre uma mutacao espontanea no gene I2S durante a formacao da celula primordial que gerara o embriao.. No primeiro caso, a mae e uma portadora e o risco de
passar o gene com a mutacao e de 50%; no segundo caso, a chance de ocorrer novamente uma mutacao espontanea em outra gestacao, embora seja muito baixa, nao chega ser nula.As mulheres que carregam uma copia anormal do gene I2S geralmente nao sao afetadas. Embora incomuns, ja foram relatados casos de Hunter em mulheres e, normalmente, a doenca se apresenta de forma mais branda.Aspectos bioquimicos da sindrome de Hunter (MPS II)O corpo humano depende de uma ampla gama de reacoes bioquimicas para manter suas funcoes vitais, inclusive para produzir energia, crescer e se desenvolver, para a comunicacao dentro do corpo e para a protecao contra infeccoes. Uma outra funcao essencial e a quebra de grandes biomoleculas, e e ai que reside a base da sindrome de Hunter (MPS II) e das outras doencas de deposito lisossomico.A fisiopatologia da sindrome de Hunter esta relacionada a um problema em uma parte do tecido conjuntivo do corpo conhecida como matriz extracelular. A matriz e constituida por uma variedade de acucares e proteinas e ajuda a formar a estrutura arquitetonica de suporte do organismo. A matriz envolve as celulas como um sistema de malha organizado e age como uma cola que mantem as celulas unidas. Uma das partes da matriz extracelular e uma molecula complexa denominada proteoglicano. Como muitos componentes do organismo, os proteoglicanos precisam ser quebrados e substituidos. Quando o organismo quebra os proteoglicanos, um dos produtos resultantes e o mucopolissacarideos ou GAG. Ha varios tipos de GAGs, cada um encontrado em locais distintos do corpo.Na sindrome de Hunter, o problema ocorre na quebra ou na decomposicao de dois GAGs: dermatan-sulfato e heparan-sulfato. A primeira etapa na quebra do dermatan-sulfato e heparan-sulfato requer a participacao da enzima lisossomica I2S. Nas pessoas acometidas pela sindrome de Hunter, esta enzima e parcial ou totalmente inativa. Como resultado, os GAGs se acumulam nas celulas de todo o organismo, principalmente nos tecidos que contem grandes quantidades de dermatan-sulfato e heparan-sulfato. A medida que este deposito aumenta, passa a interferir na maneira como as celulas e orgaos funcionam no corpo, causando varios graves sintomas.O acumulo de GAGs nao e o mesmo para todas as pessoas com sindrome de Hunter, resultando em uma amplo e diverso espectro de problemas medicos.
like I'm in flightI offer loveDrunk from my hateIt's like I'm huffin' paintAnd I love it the more I suffer,I suffocateRight before I'm about to drown,She resuscitates me,She fuckin' hates me,And I love itWait!Where you going?I'm leaving youNo you ain'tCome backWe're running right backHere we go again,It's so insaneCuz when it's going good,It's going greatI'm Superman with the wind in his backShe's Lois LaneBut when it's bad,It's awfulI feel so ashamed,I snap "who's that dude?"I don't even know his nameI laid hands on him,I never stood so low againI guess I don't know my own strength[Rihanna]Just gonna stand there and watch me burnWell that's alright because I like the way it hurtsJust gonna stand there and hear me cryWell that's alright because I love the way you lieI love the way you lieI love the way you lie[Eminem]You ever love somebody so much,You can barely breathe when you with 'em?You meetAnd neither one of you even know it hit 'emGot that warm fuzzy feeling,Yeah them chills you still get 'emNow you gettin' fuckin' sick of lookin' at 'emYou swore you'd never hit 'em,Never do nothing to hurt 'emNow you're in each other's face spewing venom in your words when you spit 'emYou push,Pull each other's hair,Scratch, claw, bit 'emThrow 'em down,Pin 'em,So lost in the moments when you're in 'emIt's the craze that the corporate controls you bothSo they say it's bestTo go your separate waysGuess that they don't know yaCause today,That was yesterday,Yesterday is over and it's a different daySound like broken records playing overBut you promised her,Next time you'd show restraintYou don't get another chanceLife is no Nintendo GameBut you lied againNow you get to watch her leave out the windowGuess that's why they call it "window pane"[Rihanna]Just gonna stand there and watch me burnWell that's alright because I like the way it hurtsJust gonna stand there and hear my cryWell that's alright because I love the way you lieI love the way you lieI love the way you lie[Eminem]Now I know we said things,Did thingsThat we didn't meanAnd we fall back into the same patterns,Same teamBut your temper's just as bad as mine isYou're the same as meWhen it comes to love you're just as blindedBaby please come backIt wasn't youBaby it was meMaybe our relationship isn't as crazy as it seemsMaybe that's what happens when a tornado meets a volcanoAll I know is I love you too much,To walk away nowCome inside,Pick up your bags off the sidewalkDon't you hear sincerity in my voice when I talk?Told you this is my fault,Look me in the eyeballNext time I'm pissed,I'll aim my fist at the dry wallNext time?There won't be no next timeI apologizeEven though I know it's liesI'm tired of the games,I just want her backI know I'm a liarIf she ever tries to fucking leave again,Ima tie her to the bedAnd set this house on fireJust gonna[Rihanna]Just gonna stand there and watch me burnWell that's alright because I like the way it hurtsJust gonna stand there and hear my cryWell that's alright because I love the way
Lately I've been hard to reachI ve been too long on my ownEverybody has a private worldwhere they can be aloneAre you calling me?Are you trying to get through?Are you reaching out for me?And I'm reaching out for you?I'm just so fucking depressed I just cant seem to get out this slumpIf I could just get over this hump butI need something to pull me out this dumpI took my bruises took my lumps fell down and I got right back upBut i need that spark to get psyched back up and in order for me to pick the mic back upI dont know how or why or when I ended up this position i'm inI'm started to feel dissin' again so I decided just to pick this penUp and try to make an attempt to vent but i just cant admitOr come to grips the fact that I may be done with rap I need a new outletAnd I know some shits so hard to swallow but I cant just sit back and wallowIn my own sorrow but I know one fact i'll be one tough act to followOne tough act to follow I'll be one tough act to followHere today gone tomorrow but you'd have to walk a thousand milesIn my shoes, just to seeWhat its like, to be meIll be you, lets trade shoesJust to see what id be likefeel your pain, you feel mineGo inside eachothers mindsJust to see what we'd findLook at shit through eachothers eyesBut don't let them say you ain't beautifulThey can all get fucked just stay true to youSo don't let them say you ain't beautifulThey can all get fucked just stay true to youI think im starting to lose my sense of humor everythings so tense and gloomI almost fee like I gotta check the temperature of the room just as soon asI walk in I'ts like all eyes on me so I try to avoid any contactCuz if I do that then it opens the door for conversation like I want thatIm not looking for extra attention I just want to be just like youBlend in with the rest of the room maybe just point me to the closest restroomI dont need no fucking man servant tryin ta follow me around and try to wipe my assLaugh at every single joke i crack and half of them aint even funny like hahhhhh"marshall your so funny man you should be a comedian god damn"Unfortunately i am i just hide behind the tears of a clownSo why dont you all sit downListen to the tale that im about to tellHell we dont gotta trade our shoesAnd you dont gotta walk no thousand milesIn my shoes, just to seeWhat its like, to be meIll be you, lets trade shoesJust to see what id be likefeel your pain, you feel mineGo inside eachothers mindsJust to see what we'd findLook at shit through eachothers eyesBut don't let them say you aint beautifulThey can all get fucked just stay true to youSo don't let them say you aint
beautifulThey can all get fucked just stay true to youNobody asked for life to deal us with these bullshit hands we're dealtWe gotta take these cards ourselves and flip em dont expect no helpNow I could have either just stayed at home sit on my ass and pissed and moanedOr take this situation with which im placed in and get up and kick my ownI was never the type of kid to wait by the door and pack his bagsAnd sat on the porch and hope and prayed for a dad to show up who never didI just wanted to fit in in every single place every school i wentI dreamed of being that cool kid even if it meant acting stupidaunt edna always told me keep making that face and it'll get stuck like thatMeanwhile im just standing there holding my tongue trynwa twalk like dwisThen i stuck my tongue on that frozen stop sign pole at 8 years oldI learned my lesson then cuz i wasn't trying to impress my friends no moreBut i already told you my whole life story not just based on my descriptionCuz where you see it from where your sittin its probably 110% differentI guess we would have to walk a mile in eachothers shoes at leastWhat size you wear? I wear tens lets see if you can fit your feetIn my shoes, just to seeWhat its like, to be meI'll be you, lets trade shoesJust to see what it'd be likeTo feel your pain, you feel mineGo inside eachothers mindsJust to see what we'd findLook at shit through eachothers eyesBut don't let them say you aint beautifulThey can all get fucked just stay true to youSo don't let them say you aint beautifulThey can all get fucked just stay true to youSoYeahTo my babiesStay strongDaddy will be home soonAnd to the rest of the worldGod gave you shoes to fit youSo put em on and wear themBe yourself manBe proud of who you areAnd even if it sounds cornyDont ever let anyone tell you you ain't beautiful
Quando me sinto soTe faco mais presenteEu fecho os meus olhosE enxergo a genteEm questao de segundosVoo pra outro mundoOutra constelacaoNao da para explicarAo ver voce chegandoQual a sensacaoA gente nao precisa ta colado pra ta juntoOs nossos corpos se conversam por horas e horasSem palavras tao dizendo a todo instante um pro outroO quanto se adoramEu nao preciso te olharPra te ter em meu mundoPorque aonde quer que eu vaVoce esta em tudoTudo, tudo que eu precisoTe vivo If you ever leave me, baby,Leave some morphine at my door'Cause it would take a whole lot of medicationTo realize what we used to have,We don't have it anymore.There's no religion that could save meNo matter how long my knees are on the floorOh so keep in mind all the sacrifices I'm makin'To keep you by my sideAnd keep you from walkin' out the door.'Cause there'll be no sunlightIf I lose you, babyThere'll be no clear skiesIf I lose you, babyJust like the clouds, my eyes will do the sameIf you walk awayEveryday, it will rain, rain, rainI'll never be your mother's favoriteYour daddy can't even look me in the eyeOooh if I was in their shoes, I'd be doing the same thingSayin' there goes my little girlWalkin' with that troublesome guyBut they're just afraid of something they can't understandOooh but little darlin' watch me change their mindsYea for you I'll try I'll try I'll try I'll tryI'll pick up these broken pieces 'til I'm bleedingIf that'll make you mine'Cause there'll be no sunlightIf I lose you, babyThere'll be no clear skiesIf I lose you, babyJust like the clouds, my eyes will do the sameIf you walk awayEveryday it will rain, rain, rainOh don't just say goodbyeDon't just say goodbyeI'll pick up these broken pieces 'til I'm bleedingIf that'll make it right'Cause there'll be no sunlightIf I lose you, babyThere'll be no clear skiesIf I lose you, babyAnd just like the clouds, my eyes will do the sameIf you walk awayEveryday it will rain, rain, rain Let's go to the beach, eachLet's go get awayThey say, what they gonna say?Have a drink, clink, found the bud lightBad bitches like me, is hard to come byThe patron own, let's go get it onThe zone own, yes I'm in the zoneIs it two, three? Leave a good tipI'mma blow off my money and don't give two shitsI'm on the floor, floorI love to danceSo give me more, more, 'till I can't standGet on the floor, floorLike it's your last danceIf you want more, moreThen here I amStarships were meant to flyHands up and touch the skyCan't stop 'cause we're so highLet's do this one more timeStarships were meant to flyHands up and touch the skyLet's do this one last timeHands up...(We're higher than a motherfucker)(We're higher than a motherfucker)(We're higher than a motherfucker)Jump in my hoopty hoopty hoopI own thatAnd I ain't paying my rent this monthI owe thatBut fuck who you want, and fuck who you likeDance all ya life there's no end in sightTwinkle, twinkle little starNow everybody let me hear you say
ray-ray-rayNow spend all your money 'cause they pay-pay-payAnd if you're a G, you a G-G-GMy name is Onika, you can call me NickiGet on the floor, floorLike it's your last chanceIf you want more, moreThen here I amStarships were meant to flyHands up and touch the skyCan't stop 'cause we're so highLet's do this one more timeStarships were meant to flyHands up and touch the skyLet's do this one last timeHands up...(We're higher than a motherfucker)(We're higher than a motherfucker)(We're higher than a motherfuck-e)Starships were meant to flyHands up and touch the skyCan't stop 'cause we're so highLet's do this one more timeStarships were meant to flyHands up and touch the skyLet's do this one last timeHands up...(We're higher than a motherfucker)(We're higher than a motherfucker)(We're higher than a motherfucker)
To all these rappers I apologizeI know it ain't fair,The only ball I dropNew Years', Times Square,The world is mineSixth SenseI see the seven signsNow baby,Let's get started for lifeEverytime I look into your eyes(Mr Worldwide)I feel like I could stare in them for a lifetimeWe can get started for lifeTonightFor lifeTonightFor lifeWe can get started for lifeTonightFor lifetonightFor lifeWe can get startedYou know it feels rightBig news, PitbullTom Cruise, MumbaiAll lit up like a December night,Like the 4th of July, Vanilla SkyThrilla in ManillaKnockin' 'em out like PacquiaoNew Ali, new FrazierBut for now it's off to MalaysiaTwo passports, three cities, two countries, one dayNow that's worldwide,If you think it's a game let's playDaleEverytime I look into your eyesI feel like I could stare in them for a lifetimeWe can get started for lifeTonightFor lifeTonightFor lifeWe can get started for lifeTonightFor lifetonightFor lifeWe can get startedYou know it feels rightI am what they thought I'd never become,I believed and became it,Now I'm here to claim itI hustle anything, you name itI went from evictions to food stamps,To back in work, what damn (?)To a passport, flooded with stampsNow slowly, everywhere I landTwo passports, three cities, two countries, one dayNow that's worldwide,If you think it's a game let's playDaleLet's get it startedCause if it feels right, we shouldn't waste any more time,Let's get it started, don't think about itYou know I'm gonna make it alright, alrightCause if it feels right,You know I've made up my mindLet's get it started, don't think about itI know that we can make it alright alrightEverytime I look into your eyesI feel like I could stare in them for a lifetimeWe can get started for lifeTonightFor lifeTonightFor lifeWe can get started for lifeTonightFor lifetonightFor lifeWe can get startedDon't start what you can't finishYou know it feels rightLet's goYou know it feels right When I look into your eyesIt's like watching the night skyOr a beautiful sunriseTheres so much they holdAnd just like them old starsI see that you've come so farTo be right where you areHow old is your soul?I won't give up on usEven if the skies get roughI'm giving you all my loveI'm still looking upAnd when you're needing your spaceTo do some navigatingI'll be here patiently waitingTo see what you findCause even the stars they burnSome even fall to the earthWe've got a lot to learnGod knows we're worth itNo I won't give upI don't wanna be someone who walks away so easilyI'm here to stay and make the difference that I can makeOur differences they do a lot to teach us how to useThe tools and gifts we got yeah, we got a lot at stakeAnd in the end, you're still my friend at least we did intendFor us to work we didn't break, we didn't burnWe had to learn how to bend without the world caving inI had to learn what I've got, and what I'm notAnd who I amI won't give up on usEven if the skies get roughI'm giving you all my loveI'm still looking upI'm still looking upI won't give
up on usGod knows I'm tough, he knowsWe got a lot to learnGod knows we're worth itI won't give up on usEven if the skies get roughI'm giving you all my loveI'm still looking up
Deixa eu entrarDentro do seu coracaoDescobrir pouco a pouco a paixaoTenta...Me sentir em quanto eu sinto voceDessa vez e amorE pra valerAi ai ai ai aiNao tem jeito de esquecerVoce de mim nao saiMinha vida sem voceNao tem sentidoEu nao aguento maisAi ai ai ai aiNao tem jeito de esquecerVoce de mim nao saiMinha vida sem voceNao tem sentidoEu nao aguento maisVoce de mim nao saiDeixa eu entrarDentro do seu coracaoDescobrir pouco a pouco a paixaoTenta...Me sentir em quanto eu sinto voceDessa vez e amorE pra valerAi ai ai ai aiNao tem jeito de esquecerVoce de mim nao saiMinha vida sem voceNao tem sentidoEu nao aguento maisAi ai ai ai aiNao tem jeito de esquecerVoce de mim nao saiMinha vida sem voceNao tem sentidoEu nao aguento maisAi ai ai ai aiNao tem jeito de esquecerVoce de mim nao saiMinha vida sem voceNao tem sentidoEu nao aguento maisAi ai ai ai ai ... As long as you love meAs long as you love meAs long as you love meWe're under pressureSeven billion people in the world trying to fit inKeep it togetherSmile on your face even though your heart is frowningBut, hey, now, you know, girlWe both know it's a cruel worldBut I will take my chancesAs long as you love meWe could be starvingWe could be homelessWe could be brokeAs long as you love meI'll be your platinumI'll be your silverI'll be your goldAs long as you love me (love me)As long as you love me (love me)I'll be your soldier (ha, ha)Fighting every second of the day for your dreams, girlI'll be your H.O.V.A. (yey, yey)You can be my Destiny's Child on scene, girlSo don't stress and don't cryOh, we don't need no wings to flyJust take my handAs long as you love meWe could be starvingWe could be homelessWe could be brokeAs long as you love meI'll be your platinumI'll be your silverI'll be your goldAs long as you love me (love me)As long as you love me (wow) (wow) (love me)I don't know if this makes senseBut you're my hallelujahGive me a time and placeI'll rendezvous itI'll fly you to itI'll beat you thereGirl, you know I got youUs, trustA couple things I can't spell without uNow we on top of the world (world)'Cause that's just how we doUsed to tell me sky's the limitNow the sky's our point of viewMan, we stepping out like woa (oh, God)Cameras point and shoot (shoot)Ask me "what's my best side"I stand back and point at you (you)You the one that I argue withFeel like I need a new girl to be bothered withBut the grass ain't always greener on the other sideIt's green where you water it, so, I knowWe got issues, baby, true, true, trueBut I'd rather work on this with youThan to go ahead and start with someone newAs long as you love meAs long as you love me (love me)We could be starving (yeah)We could be homeless (hey)We could be brokeAs long as you love meI'll be your platinum (platinum)I'll be your silverI'll be your gold(As long as you
love me)(As long as you love me)(As long as you love me) I'll be your silver, I'll be your gold(As long as you love me) as long as you love me, you love me, you love me (there's only one thing, yeah)As long as you love me, you love me (be your gold)Long as you love me, long as you love me, long as you love me, yeahAs long as you love me