{"id":15543,"date":"2022-12-16T16:17:05","date_gmt":"2022-12-16T19:17:05","guid":{"rendered":"https:\/\/www.ufrgs.br\/levi\/?page_id=15543"},"modified":"2022-12-16T22:19:02","modified_gmt":"2022-12-17T01:19:02","slug":"sindrome-de-prune-belly","status":"publish","type":"page","link":"https:\/\/www.ufrgs.br\/levi\/sindrome-de-prune-belly\/","title":{"rendered":"S\u00edndrome de Prune Belly"},"content":{"rendered":"\t\t
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Publicado em 16 de dezembro de 2022.<\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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Nos \u00faltimos anos o cuidado a sa\u00fade do rec\u00e9m-nascido e da crian\u00e7a tem evolu\u00eddo muito em rela\u00e7\u00e3o a preven\u00e7\u00e3o e diagnostico precoce de algumas s\u00edndromes gen\u00e9ticas, ficando evidente a necessidade de aprofundar conhecimentos cient\u00edficos sobre as possibilidades de identificar e orientar a fam\u00edlia sobre os cuidados.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t

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SPB<\/h1>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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A S\u00edndrome de Prune Belly (SPB) \u00e9 uma forma de uropatia fetal gen\u00e9tica, sem etiologia definida, por\u00e9m v\u00e1rias teorias t\u00eam sido propostas para esclarecer a etiopatogenia\u00b9.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t

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A s\u00edndrome consiste na triade das seguintes caracter\u00edsticas<\/h1>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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Defici\u00eancia ou aus\u00eancia de musculatura da parede abdominal.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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Criptorquidismo bilateral: ou test\u00edculos n\u00e3o descidos, ou a aus\u00eancia de um ou dos dois test\u00edculos na bolsa testicular.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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Malforma\u00e7\u00e3o do trato urin\u00e1rio\u00b9.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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Rec\u00e9m-nascido com abdome caracter\u00edstico da s\u00edndrome de Prune Belly\u00b2<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t

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Etiologia<\/h1>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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Consultando a bibliografia espec\u00edfica constata-se que ainda existe dicotomia entre a etiologia da s\u00edndrome. Uma das linhas de investiga\u00e7\u00e3o sugere a obstru\u00e7\u00e3o uretral e\/ou ureteral, que levaria o feto a desenvolver uma dilata\u00e7\u00e3o grosseira das vias urin\u00e1rias, que por sua vez distenderia a parede abdominal, comprometendo sua forma\u00e7\u00e3o adequada e impedindo a migra\u00e7\u00e3o dos test\u00edculos\u00b2.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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Outros pesquisadores atribuem o defeito mesod\u00e9rmico como a origem da s\u00edndrome a um dist\u00farbio na instaura\u00e7\u00e3o do mesoderma durante a terceira semana de gesta\u00e7\u00e3o, pois esse folheto embrion\u00e1rio \u00e9 respons\u00e1vel pela forma\u00e7\u00e3o do trato urin\u00e1rio, rins e musculatura abdominal\u00b2.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t

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Incid\u00eancia e caracter\u00edsticas<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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Sabe-se que a incid\u00eancia estimada da SPB \u00e9 de 3,6 a 3,8 por 100.000 nascidos vivos e 95% das crian\u00e7as afetadas s\u00e3o do sexo masculino, somente 3-5% dos casos em meninas, podendo ser denominada nesses casos, como pseudoss\u00edndrome de Prune Belly, por n\u00e3o apresentar a criptorquidia\u00b3.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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Em geral, os rec\u00e9m-nascidos apresentam pele da parede abdominal frouxa e enrugada, se assemelhando a uma ameixa seca, origem do nome em ingl\u00eas da s\u00edndrome, mas em raro dos casos a musculatura abdominal anterior pode estar ausente\u00b3.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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A <\/span>criptorquidia \u00e9 a caracter\u00edstica principal da s\u00edndrome<\/span><\/strong> e os test\u00edculos s\u00e3o intra-abdominais, localizados junto aos segmentos ureterais dilatados, na altura dos vasos il\u00edacos. No trato urin\u00e1rio, as altera\u00e7\u00f5es cl\u00ednicas se manifestam em grau variado, embora os rins possam ser normais\u00b9.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t

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Progn\u00f3stico<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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Quanto ao progn\u00f3stico, at\u00e9 <\/span>30%<\/span><\/strong> dos que <\/span>sobrevivem por longos per\u00edodos desenvolvem insufici\u00eancia renal cr\u00f4nica<\/span><\/strong> e, eventualmente, necessitam de transplante renal\u00b3. A <\/span>mortalidade perinatal<\/span><\/strong> em consequ\u00eancia da s\u00edndrome<\/span> ocorre entre 10 a 25%<\/span><\/strong> devido \u00e0 baixa produ\u00e7\u00e3o de urina durante o per\u00edodo fetal por displasia renal e anormalidades do trato urin\u00e1rio\u00b9.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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A vesicostomia e a sonda vesical de demora s\u00e3o procedimentos que ajudam na preserva\u00e7\u00e3o da fun\u00e7\u00e3o renal, auxiliando na drenagem tempor\u00e1ria das vias urin\u00e1rias at\u00e9 que a crian\u00e7a se desenvolva para a realiza\u00e7\u00e3o de cirurgias. A enfermagem possui atribui\u00e7\u00f5es fundamentais para executar as t\u00e9cnicas adequadas na inser\u00e7\u00e3o e manuten\u00e7\u00e3o do cateter para reduzir os riscos de infec\u00e7\u00e3o e promover a perman\u00eancia da sua fixa\u00e7\u00e3o\u00b9.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t

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As cirurgias encontradas na literatura s\u00e3o:<\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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Orquidopexia<\/span> – corre\u00e7\u00e3o da criptorquidia bilateral, recomenda-se que seja realizada antes de um ano de vida;<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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Procedimentos no trato urin\u00e1rio;<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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Abdominoplastia <\/span>– reconstru\u00e7\u00e3o da parede abdominal anterior\u00b3.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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A. Ao nascimento<\/span> \u00a0B. Aos nove anos de idade<\/span><\/strong> apresentando cicatrizes cir\u00fargicas por reconstru\u00e7\u00e3o da parede abdominal\u00b3.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t

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O tratamento se baseia em:<\/h1>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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\n\t\t\t\n\t\t\t\t\t\t<\/span>\n\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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terapia medicamentosa;<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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\n\t\t\t<\/path><\/clipPath><\/path><\/clipPath><\/defs><\/path><\/g><\/path><\/g><\/svg>\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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controle da disfun\u00e7\u00e3o da elimina\u00e7\u00e3o de urina<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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nutri\u00e7\u00e3o adequada<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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terapia especifica (fisioterapia, fonoaudiologia, terapia ocupacional e psicologia)<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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\n\t\t\t<\/path><\/clipPath><\/path><\/clipPath><\/defs><\/path><\/g><\/path><\/g><\/svg>\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t
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Cuidados<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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Em rela\u00e7\u00e3o aos <\/span>Cuidado a crian\u00e7a com S\u00edndrome de Prune Belly <\/span><\/strong>deve ser centrado na disfun\u00e7\u00e3o anat\u00f4mica e no dano sens\u00f3rio-motor. A fam\u00edlia dever ser orientada para os cuidados b\u00e1sicos das infec\u00e7\u00f5es urinarias de repeti\u00e7\u00e3o, de modo a evitar hospitaliza\u00e7\u00f5es desnecess\u00e1rias.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t

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Considera\u00e7\u00f5es Finais<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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A <\/span>S\u00edndrome de Prune Belly<\/span><\/strong> \u00e9 uma doen\u00e7a gen\u00e9tica rara de causas gen\u00e9ticas de malforma\u00e7\u00f5es da parede do abd\u00f4men em crian\u00e7as. O diagn\u00f3stico precoce pode prevenir complica\u00e7\u00f5es e melhorar a qualidade dos cuidados ao longo da vida dessas crian\u00e7as.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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O enfermeiro pediatra deve se familiarizar cada vez mais com as s\u00edndromes gen\u00e9ticas em pediatria. Aumenta a probabilidade de um diagn\u00f3stico precoce e, portanto, o tratamento e o cuidado centrado nas necessidades da crian\u00e7a e sua fam\u00edlia come\u00e7ar\u00e1 mais cedo. Isso pode melhorar a qualidade de vida dessas crian\u00e7as e dos cuidados a eles dirigidos.<\/span><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t

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\n\t\t\t\n\t\t\t\t\t\t<\/span>\n\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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Refer\u00eancias<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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1. Arlen AM, Nawaf C, Kirsch AJ. Prune belly syndrome: current perspectives. Pediatric Health Med Ther [Internet]. 2019 Aug 06 [cited 2022 Nov 21]; 10:75-81. DOI 10.2147\/PHMT.S188014. Available from: https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC6689549\/.
2. Carvalho NDS, Cavachini C, Dudus MM. S\u00edndrome de Prune Belly. Resid\u00eancia pedi\u00e1trica [Internet]. 2017 Aug 01 [cited 2022 Nov 21]; 8(1):45-57. DOI 10.25060\/residpediatr-2018.v8n1-07. Available from: https:\/\/cdn.publisher.gn1.link\/residenciapediatrica.com.br\/pdf\/v8n1a07.pdf
3. Pomajzl AJ, Sankararaman S. Prune Belly Syndrome. StatPearls [Internet]. 2022 Oct 08 [cited 2022 Nov 21]; Available from: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK544248\/<\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t

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Como citar:<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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SLODKOWSKI, H.C.; VALES, E.N.; KARL, I.D.S.; S\u00edndrome de Prune Belly. Blog LEVi@enf2022(viewed December 2022) Avaliable from: https:\/\/www.ufrgs.br\/levi\/<\/span>sindrome-de-prune-belly<\/span><\/a><\/p>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t","protected":false},"excerpt":{"rendered":"

Publicado em 16 de dezembro de 2022. Nos \u00faltimos anos o cuidado a sa\u00fade do rec\u00e9m-nascido e da crian\u00e7a tem evolu\u00eddo muito em rela\u00e7\u00e3o a preven\u00e7\u00e3o e diagnostico precoce de algumas s\u00edndromes gen\u00e9ticas, ficando evidente a necessidade de aprofundar conhecimentos cient\u00edficos sobre as possibilidades de identificar e orientar a fam\u00edlia sobre os cuidados. SPB A…
Leia mais<\/a><\/p>\n","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"open","ping_status":"closed","template":"elementor_header_footer","meta":{"spay_email":""},"jetpack-related-posts":[{"id":13964,"url":"https:\/\/www.ufrgs.br\/levi\/publicacoes-2022\/","url_meta":{"origin":15543,"position":0},"title":"Publica\u00e7\u00f5es 2022","date":"18 de outubro de 2022","format":false,"excerpt":"A import\u00e2ncia do Letramento em Sa\u00fade para o cuidado da CRIANESAutoras: Enf\u00aa Esp. Su\u00e9len Heningues Leiman, Prof\u00aa Dr\u00aa Alessandra Vaccari. Data da publica\u00e7\u00e3o 17 de dezembro de 2022 A brincadeira como estrat\u00e9gia de promo\u00e7\u00e3o do desenvolvimento infantilAutoras: Acad. Enf. Agatha Caroline Valanera Teixeira, Acad. Enf. Alessandra Porto D'\u00e1vila, Acad. 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