{"id":41898,"date":"2026-02-18T18:00:00","date_gmt":"2026-02-18T21:00:00","guid":{"rendered":"https:\/\/www.grupomedcof.com.br\/blog\/?p=41898"},"modified":"2026-02-18T15:57:03","modified_gmt":"2026-02-18T18:57:03","slug":"hiperplasia-adrenal-congenita","status":"publish","type":"post","link":"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/","title":{"rendered":"Hiperplasia Adrenal Cong\u00eanita: fisiopatologia, espectro cl\u00ednico e mais!"},"content":{"rendered":"<div id=\"ez-toc-container\" class=\"ez-toc-v2_0_74 counter-hierarchy ez-toc-counter ez-toc-transparent ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Sum\u00e1rio<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Alternar tabela de conte\u00fado\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Toggle<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewBox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" fill=\"currentColor\"><\/path><\/svg><svg style=\"fill: #999;color:#999\" class=\"arrow-unsorted-368013\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"10px\" height=\"10px\" viewBox=\"0 0 24 24\" version=\"1.2\" baseProfile=\"tiny\"><path d=\"M18.2 9.3l-6.2-6.3-6.2 6.3c-.2.2-.3.4-.3.7s.1.5.3.7c.2.2.4.3.7.3h11c.3 0 .5-.1.7-.3.2-.2.3-.5.3-.7s-.1-.5-.3-.7zM5.8 14.7l6.2 6.3 6.2-6.3c.2-.2.3-.5.3-.7s-.1-.5-.3-.7c-.2-.2-.4-.3-.7-.3h-11c-.3 0-.5.1-.7.3-.2.2-.3.5-.3.7s.1.5.3.7z\"\/><\/svg><\/span><\/span><\/span><\/a><\/span><\/div>\n<nav><ul class='ez-toc-list ez-toc-list-level-1 ' ><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#Fisiopatologia_O_%E2%80%9CEfeito_Gargalo%E2%80%9D\" >Fisiopatologia: O &#8220;Efeito Gargalo&#8221;<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#O_Bloqueio_da_21-Hidroxilase\" >O Bloqueio da 21-Hidroxilase<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#Espectro_Clinico\" >Espectro Cl\u00ednico<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#Forma_Classica_Perdedora_de_Sal_Grave\" >Forma Cl\u00e1ssica Perdedora de Sal (Grave)<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#Forma_Classica_Virilizante_Simples\" >Forma Cl\u00e1ssica Virilizante Simples<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#Forma_Nao_Classica_Tardia\" >Forma N\u00e3o Cl\u00e1ssica (Tardia)<\/a><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#Diagnostico_e_Triagem_Neonatal\" >Diagn\u00f3stico e Triagem Neonatal<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#Teste_do_Pezinho_Triagem\" >Teste do Pezinho (Triagem)<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#Confirmacao_Diagnostica\" >Confirma\u00e7\u00e3o Diagn\u00f3stica<\/a><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-10\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#Tratamento_Cronico\" >Tratamento Cr\u00f4nico<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-11\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#Ajuste_de_dose_em_situacoes_de_estresse\" >Ajuste de dose em situa\u00e7\u00f5es de estresse<\/a><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-12\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#Manejo_da_Crise_Adrenal_Emergencia\" >Manejo da Crise Adrenal (Emerg\u00eancia)<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-13\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#_Seguimento_Ambulatorial\" >&nbsp;Seguimento Ambulatorial<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-14\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/#Como_conquistar_a_aprovacao_na_residencia\" >Como conquistar a aprova\u00e7\u00e3o na resid\u00eancia?<\/a><\/li><\/ul><\/nav><\/div>\n\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"1024\" height=\"576\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/Blog-MedCof-2026-02-18T155526.579-1024x576.jpg\" alt=\"\" class=\"wp-image-41906\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/Blog-MedCof-2026-02-18T155526.579-1024x576.jpg 1024w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/Blog-MedCof-2026-02-18T155526.579-300x169.jpg 300w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/Blog-MedCof-2026-02-18T155526.579-768x432.jpg 768w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/Blog-MedCof-2026-02-18T155526.579-1536x864.jpg 1536w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/Blog-MedCof-2026-02-18T155526.579-747x420.jpg 747w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/Blog-MedCof-2026-02-18T155526.579-150x84.jpg 150w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/Blog-MedCof-2026-02-18T155526.579-696x392.jpg 696w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/Blog-MedCof-2026-02-18T155526.579-1068x601.jpg 1068w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/Blog-MedCof-2026-02-18T155526.579.jpg 1920w\" sizes=\"auto, (max-width: 1024px) 100vw, 1024px\" \/><\/figure>\n\n\n\n<p>A<strong> Hiperplasia Adrenal Cong\u00eanita<\/strong> \u00e9 uma doen\u00e7a autoss\u00f4mica recessiva muito presente nas provas de resid\u00eancia para Pediatria. Ela se caracteriza por um defeito na esteroidog\u00eanese adrenal. A forma mais comum (>90% dos casos) \u00e9 a defici\u00eancia da enzima <strong>21-hidroxilase (21-OHase)<\/strong>.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\" id=\"h-fisiopatologia-o-efeito-gargalo\"><span class=\"ez-toc-section\" id=\"Fisiopatologia_O_%E2%80%9CEfeito_Gargalo%E2%80%9D\"><\/span><strong>Fisiopatologia: O &#8220;Efeito Gargalo&#8221;<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p>Para entender a cl\u00ednica, \u00e9 preciso visualizar o c\u00f3rtex adrenal dividido em tr\u00eas zonas funcionais, onde o colesterol \u00e9 o substrato inicial:<\/p>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Zona Glomerulosa:<\/strong> Produz <strong>Aldosterona<\/strong> (regula\u00e7\u00e3o hidroeletrol\u00edtica).<\/li>\n\n\n\n<li><strong>Zona Fasciculada:<\/strong> Produz <strong>Cortisol<\/strong> (metabolismo e resposta ao estresse).<\/li>\n\n\n\n<li><strong>Zona Reticulada:<\/strong> Produz <strong>Androg\u00eanios<\/strong> (DHEA, Androstenediona).<\/li>\n<\/ol>\n\n\n\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"1024\" height=\"496\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-82-1024x496.png\" alt=\"\" class=\"wp-image-41899\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-82-1024x496.png 1024w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-82-300x145.png 300w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-82-768x372.png 768w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-82-867x420.png 867w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-82-150x73.png 150w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-82-696x337.png 696w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-82-1068x517.png 1068w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-82.png 1179w\" sizes=\"auto, (max-width: 1024px) 100vw, 1024px\" \/><figcaption class=\"wp-element-caption\">Zonas da gl\u00e2ndula adrenal e os horm\u00f4nios produzidos por cada uma. Fonte: <a href=\"https:\/\/www.lecturio.com\/pt\/concepts\/glandulas-suprarrenais-anatomia\/\">https:\/\/www.lecturio.com\/pt\/concepts\/glandulas-suprarrenais-anatomia\/<\/a>\u00a0<\/figcaption><\/figure>\n\n\n\n<h2 class=\"wp-block-heading\" id=\"h-o-bloqueio-da-21-hidroxilase\"><span class=\"ez-toc-section\" id=\"O_Bloqueio_da_21-Hidroxilase\"><\/span><strong>O Bloqueio da 21-Hidroxilase<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p>A enzima 21-OHase \u00e9 fundamental para a s\u00edntese de Aldosterona e Cortisol. Sua defici\u00eancia cria um bloqueio:<\/p>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>D\u00e9fice:<\/strong> N\u00e3o h\u00e1 produ\u00e7\u00e3o adequada de Cortisol e Aldosterona.<\/li>\n\n\n\n<li><strong>Ac\u00famulo:<\/strong> Os precursores anteriores ao bloqueio acumulam-se, sendo o principal marcador a <strong>17-Hidroxiprogesterona (17-OHP)<\/strong>.<\/li>\n\n\n\n<li><strong>Desvio (Shunt):<\/strong> O excesso de 17-OHP \u00e9 desviado para a \u00fanica via livre: a produ\u00e7\u00e3o de <strong>androg\u00eanios<\/strong> na zona reticulada.<\/li>\n\n\n\n<li><strong>Hiperplasia:<\/strong> A falta de Cortisol elimina o <em>feedback<\/em> negativo no hipot\u00e1lamo\/hip\u00f3fise \u2192 aumento do CRH e ACTH\u2192\u00a0 est\u00edmulo cont\u00ednuo e crescimento (hiperplasia) da gl\u00e2ndula adrenal.<\/li>\n<\/ol>\n\n\n\n<figure class=\"wp-block-image size-full\"><img loading=\"lazy\" decoding=\"async\" width=\"611\" height=\"364\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-85.png\" alt=\"\" class=\"wp-image-41902\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-85.png 611w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-85-300x179.png 300w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-85-150x89.png 150w\" sizes=\"auto, (max-width: 611px) 100vw, 611px\" \/><figcaption class=\"wp-element-caption\">\u00a0Esteroidog\u00eanese no c\u00f3rtex adrenal. Fonte: Acervo de aulas do Grupo MedCof.\u00a0<\/figcaption><\/figure>\n\n\n\n<figure class=\"wp-block-image size-full\"><img loading=\"lazy\" decoding=\"async\" width=\"724\" height=\"403\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-86.png\" alt=\"\" class=\"wp-image-41903\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-86.png 724w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-86-300x167.png 300w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-86-150x83.png 150w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-86-696x387.png 696w\" sizes=\"auto, (max-width: 724px) 100vw, 724px\" \/><figcaption class=\"wp-element-caption\">Esteroidog\u00eanese interrompida a n\u00edvel da enzima 21-hidroxilase no c\u00f3rtex adrenal. Fonte: Acervo de aulas do Grupo MedCof.<\/figcaption><\/figure>\n\n\n\n<h2 class=\"wp-block-heading\" id=\"h-espectro-clinico\"><span class=\"ez-toc-section\" id=\"Espectro_Clinico\"><\/span><strong>Espectro Cl\u00ednico<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p>A gravidade depende da atividade enzim\u00e1tica residual.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-forma-classica-perdedora-de-sal-grave\"><span class=\"ez-toc-section\" id=\"Forma_Classica_Perdedora_de_Sal_Grave\"><\/span><strong>Forma Cl\u00e1ssica Perdedora de Sal (Grave)<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>Representa 75% dos casos cl\u00e1ssicos. Atividade enzim\u00e1tica <strong>&lt; 2%<\/strong>.<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Fisiopatologia:<\/strong> Aus\u00eancia quase total de Cortisol e Aldosterona.<\/li>\n\n\n\n<li><strong>Quadro Cl\u00ednico:<\/strong> Viriliza\u00e7\u00e3o pr\u00e9-natal. Ocorre a &#8220;Crise Adrenal&#8221; cl\u00e1ssica entre a <strong>2\u00aa e 3\u00aa semana de vida<\/strong> (10-14 dias).<\/li>\n\n\n\n<li><strong>Sinais de Alarme:<\/strong> V\u00f4mitos (confundidos com refluxo ou estenose de piloro), desidrata\u00e7\u00e3o grave, hipotens\u00e3o e choque.<\/li>\n\n\n\n<li><strong>Laborat\u00f3rio Cr\u00edtico:<\/strong>\n<ul class=\"wp-block-list\">\n<li>Hiponatremia (Perda de Na+).<\/li>\n\n\n\n<li>Hipercalemia (Reten\u00e7\u00e3o de K+).<\/li>\n\n\n\n<li>Acidose Metab\u00f3lica (Reten\u00e7\u00e3o de H+).<\/li>\n\n\n\n<li>Hipoglicemia (Falta de Cortisol).<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-forma-classica-virilizante-simples\"><span class=\"ez-toc-section\" id=\"Forma_Classica_Virilizante_Simples\"><\/span><strong>Forma Cl\u00e1ssica Virilizante Simples<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>Atividade enzim\u00e1tica residual de <strong>3-7%<\/strong>.<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Fisiopatologia:<\/strong> Produ\u00e7\u00e3o de Aldosterona suficiente para evitar a perda de sal, mas Cortisol insuficiente.<\/li>\n\n\n\n<li><strong>Quadro Cl\u00ednico:<\/strong> Viriliza\u00e7\u00e3o pr\u00e9 e p\u00f3s-natal devido ao excesso de androg\u00eanios.\n<ul class=\"wp-block-list\">\n<li><em>Meninas:<\/em> Ambiguidade genital ao nascer (clitoromegalia, fus\u00e3o de l\u00e1bios &#8211; Escala de Prader).<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"1024\" height=\"355\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-87-1024x355.png\" alt=\"\" class=\"wp-image-41905\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-87-1024x355.png 1024w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-87-300x104.png 300w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-87-768x267.png 768w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-87-1210x420.png 1210w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-87-150x52.png 150w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-87-696x242.png 696w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-87-1068x371.png 1068w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-87.png 1288w\" sizes=\"auto, (max-width: 1024px) 100vw, 1024px\" \/><figcaption class=\"wp-element-caption\">Escala de Prader para avalia\u00e7\u00e3o de viriliza\u00e7\u00e3o genital. Fonte: Acervo de aulas do Grupo MedCof.\u00a0<\/figcaption><\/figure>\n\n\n\n<ul class=\"wp-block-list\">\n<li><em>Meninos:<\/em> Genit\u00e1lia normal ao nascer, mas com viriliza\u00e7\u00e3o p\u00f3s-natal (aumento do p\u00eanis, pubarca precoce, acne, voz grave).<\/li>\n\n\n\n<li><strong>Crescimento:<\/strong> Estir\u00e3o inicial r\u00e1pido, mas fechamento precoce das ep\u00edfises \u2192 <strong>Baixa estatura final<\/strong>.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-forma-nao-classica-tardia\"><span class=\"ez-toc-section\" id=\"Forma_Nao_Classica_Tardia\"><\/span><strong>Forma N\u00e3o Cl\u00e1ssica (Tardia)<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>\u00c9 a forma mais leve. Aldosterona e Cortisol normais \u00e0s custas de hiperest\u00edmulo.<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Quadro Cl\u00ednico:<\/strong> Assintom\u00e1tica ao nascer. Na inf\u00e2ncia\/adolesc\u00eancia surge pubarca precoce, hirsutismo, acne severa e irregularidade menstrual (mimetiza S\u00edndrome dos Ov\u00e1rios Polic\u00edsticos).<\/li>\n<\/ul>\n\n\n\n<figure class=\"wp-block-image size-full is-resized\"><img loading=\"lazy\" decoding=\"async\" width=\"400\" height=\"316\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-26.jpeg\" alt=\"\" class=\"wp-image-41904\" style=\"width:659px;height:auto\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-26.jpeg 400w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-26-300x237.jpeg 300w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-26-150x119.jpeg 150w\" sizes=\"auto, (max-width: 400px) 100vw, 400px\" \/><figcaption class=\"wp-element-caption\">Classifica\u00e7\u00e3o de Ferriman e Gallwey para diagn\u00f3stico de hirsutismo. Fonte: <a href=\"https:\/\/doi.org\/10.1590\/S0104-42302010000100005\">https:\/\/doi.org\/10.1590\/S0104-42302010000100005<\/a>\u00a0<\/figcaption><\/figure>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Diagnostico_e_Triagem_Neonatal\"><\/span><strong>Diagn\u00f3stico e Triagem Neonatal<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Teste_do_Pezinho_Triagem\"><\/span><strong>Teste do Pezinho (Triagem)<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>Dosagem de <strong>17-OH-Progesterona (17-OHP)<\/strong> em sangue seco, coletado idealmente entre o <strong>3\u00ba e 5\u00ba dia de vida<\/strong>.<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Interpreta\u00e7\u00e3o e Armadilhas:<\/strong>\n<ul class=\"wp-block-list\">\n<li><strong>Falso Positivo:<\/strong> Prematuridade, baixo peso, estresse perinatal (a adrenal do prematuro \u00e9 imatura e produz mais andr\u00f3genos naturalmente).<\/li>\n\n\n\n<li><strong>Falso Negativo:<\/strong> Uso de corticoides pela m\u00e3e na gesta\u00e7\u00e3o ou coleta muito precoce (&lt; 48h).<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Confirmacao_Diagnostica\"><\/span><strong>Confirma\u00e7\u00e3o Diagn\u00f3stica<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>Se a triagem for alterada, solicitar dosagem s\u00e9rica de: <strong>17-OHP, Cortisol, Androstenediona, Testosterona e Eletr\u00f3litos (Na, K)<\/strong>.<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><em>Genit\u00e1lia At\u00edpica:<\/em> Solicitar cari\u00f3tipo (para diferenciar menina virilizada de menino criptorqu\u00eddico) e USG p\u00e9lvica.<\/li>\n<\/ul>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Tratamento_Cronico\"><\/span><strong>Tratamento Cr\u00f4nico<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p>O objetivo \u00e9 repor os horm\u00f4nios deficientes e suprimir o ACTH para frear a produ\u00e7\u00e3o de androg\u00eanios.<\/p>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Glicocorticoide (Hidrocortisona):<\/strong> Droga de escolha na inf\u00e2ncia.\n<ul class=\"wp-block-list\">\n<li><em>Dose:<\/em> 10-15~mg\/m2\/dia (dividido em 3 tomadas).<\/li>\n\n\n\n<li><em>Por que Hidrocortisona?<\/em> Tem meia-vida curta e menor impacto negativo no crescimento \u00f3sseo comparada \u00e0 prednisona ou dexametasona.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Mineralocorticoide (Fludrocortisona):<\/strong> Para formas perdedoras de sal.\n<ul class=\"wp-block-list\">\n<li><em>Dose:<\/em> 100-200~mcg\/dia<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Suplementa\u00e7\u00e3o de Sal (NaCl):<\/strong> 1 a 2g\/dia at\u00e9 o in\u00edcio da introdu\u00e7\u00e3o alimentar.<\/li>\n<\/ol>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-ajuste-de-dose-em-situacoes-de-estresse\"><span class=\"ez-toc-section\" id=\"Ajuste_de_dose_em_situacoes_de_estresse\"><\/span><strong>Ajuste de dose em situa\u00e7\u00f5es de estresse<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>Em casos de febre, trauma ou cirurgia, a demanda de cortisol aumenta. O paciente n\u00e3o consegue responder a esse estresse.<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Conduta:<\/strong> <strong>Dobrar ou triplicar<\/strong> a dose de Hidrocortisona. Se houver v\u00f4mitos, administrar Hidrocortisona endovenosa (100mg\/m2).<\/li>\n<\/ul>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Manejo_da_Crise_Adrenal_Emergencia\"><\/span><strong>Manejo da Crise Adrenal (Emerg\u00eancia)<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p>A crise perdedora de sal \u00e9 uma emerg\u00eancia com risco de vida. Memorize os <strong>&#8220;5 S&#8221;<\/strong> do tratamento:<\/p>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>S<\/strong>upport (Suporte): Monitoriza\u00e7\u00e3o card\u00edaca, acesso venoso, estabiliza\u00e7\u00e3o (MOVE).<\/li>\n\n\n\n<li><strong>S<\/strong>alt (Sal\/Volume): Expans\u00e3o vol\u00eamica vigorosa com <strong>Soro Fisiol\u00f3gico 0,9%<\/strong> (para corrigir a hipovolemia e hiponatremia).<\/li>\n\n\n\n<li><strong>S<\/strong>ugar (A\u00e7\u00facar): Corre\u00e7\u00e3o da hipoglicemia.<\/li>\n\n\n\n<li><strong>S<\/strong>teroids (Esteroides): <strong>Hidrocortisona<\/strong> em dose de ataque (100mg\/m2 EV). N\u00e3o usar outros corticoides de in\u00edcio pois a hidrocortisona tem efeito mineralocorticoide tamb\u00e9m.<\/li>\n\n\n\n<li><strong>S<\/strong>earch (Busca): Investigar o fator desencadeante (infec\u00e7\u00e3o, m\u00e1 ades\u00e3o, etc.).<\/li>\n<\/ol>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"_Seguimento_Ambulatorial\"><\/span><strong>&nbsp;Seguimento Ambulatorial<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p>O monitoramento visa evitar o <strong>avan\u00e7o da idade \u00f3ssea<\/strong> (sinal de tratamento insuficiente e excesso de androg\u00eanios) e a S\u00edndrome de Cushing iatrog\u00eanica (excesso de tratamento).<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Crescimento:<\/strong> Monitorar velocidade de crescimento e RX de idade \u00f3ssea.<\/li>\n\n\n\n<li><strong>Cirurgia:<\/strong> Em meninas virilizadas, pode-se indicar clitoroplastia ou corre\u00e7\u00e3o do seio urogenital.<\/li>\n<\/ul>\n\n\n\n<h2 class=\"wp-block-heading\" id=\"como-conquistar-a-aprova\u00e7\u00e3o-na-resid\u00eancia\"><span class=\"ez-toc-section\" id=\"Como_conquistar_a_aprovacao_na_residencia\"><\/span><strong>Como conquistar a aprova\u00e7\u00e3o na resid\u00eancia?<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p>Quer alcan\u00e7ar a aprova\u00e7\u00e3o nas provas de resid\u00eancia m\u00e9dica? Ent\u00e3o seja um&nbsp;<a href=\"https:\/\/www.grupomedcof.com.br\/home\/\">MedCofer<\/a>! Aqui te ajudaremos na busca da aprova\u00e7\u00e3o com conte\u00fados de qualidade e uma&nbsp;<a href=\"https:\/\/www.grupomedcof.com.br\/blog\/presenca-da-medcof-nas-aprovacoes-em-residencia-medica-no-brasil\/\">metodologia que j\u00e1 aprovou mais de 35 mil residentes no pa\u00eds!&nbsp;<\/a>Por fim, acesse o nosso<a href=\"https:\/\/www.youtube.com\/@GrupoMedcof\/featured\">&nbsp;<strong>canal do youtube&nbsp;<\/strong><\/a>para ver o nosso material.<\/p>\n\n\n\n<figure class=\"wp-block-embed is-type-video is-provider-youtube wp-block-embed-youtube wp-embed-aspect-16-9 wp-has-aspect-ratio\"><div class=\"wp-block-embed__wrapper\">\n<div class=\"youtube-embed\" data-video_id=\"OKXm-giAISE\"><iframe loading=\"lazy\" title=\"Reprodu\u00e7\u00e3o Humana: at\u00e9 onde o ginecologista geral pode ir? | GoCast | EP.02\" width=\"696\" height=\"392\" src=\"https:\/\/www.youtube.com\/embed\/OKXm-giAISE?feature=oembed&#038;enablejsapi=1\" frameborder=\"0\" allow=\"accelerometer; autoplay; clipboard-write; encrypted-media; gyroscope; picture-in-picture; web-share\" referrerpolicy=\"strict-origin-when-cross-origin\" allowfullscreen><\/iframe><\/div>\n<\/div><\/figure>\n","protected":false},"excerpt":{"rendered":"<p>Entenda a fisiopatologia da 21-hidroxilase, formas cl\u00ednicas, teste do pezinho, crise adrenal e tratamento com hidrocortisona da Hiperplasia Adrenal Cong\u00eanita.<\/p>\n","protected":false},"author":21,"featured_media":41906,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[120],"tags":[3581],"ppma_author":[3617],"class_list":{"0":"post-41898","1":"post","2":"type-post","3":"status-publish","4":"format-standard","5":"has-post-thumbnail","7":"category-pediatria","8":"tag-efeito-gargalo"},"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v24.3 (Yoast SEO v27.4) - https:\/\/yoast.com\/product\/yoast-seo-premium-wordpress\/ -->\n<title>Hiperplasia Adrenal Cong\u00eanita: fisiopatologia, espectro cl\u00ednico e mais! - Blog de Medcof: conte\u00fados, editais, revalida e mais!<\/title>\n<meta name=\"description\" content=\"Entenda a fisiopatologia da 21-hidroxilase, formas cl\u00ednicas, teste do pezinho, crise adrenal e tratamento com hidrocortisona da Hiperplasia Adrenal Cong\u00eanita.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/\" \/>\n<meta property=\"og:locale\" content=\"pt_BR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Hiperplasia Adrenal Cong\u00eanita: fisiopatologia, espectro cl\u00ednico e mais!\" \/>\n<meta property=\"og:description\" content=\"Entenda a fisiopatologia da 21-hidroxilase, formas cl\u00ednicas, teste do pezinho, crise adrenal e tratamento com hidrocortisona da Hiperplasia Adrenal Cong\u00eanita.\" \/>\n<meta property=\"og:url\" content=\"https:\/\/www.grupomedcof.com.br\/blog\/hiperplasia-adrenal-congenita\/\" \/>\n<meta property=\"og:site_name\" content=\"Blog de Medcof: conte\u00fados, editais, revalida e mais!\" \/>\n<meta property=\"article:publisher\" content=\"https:\/\/www.facebook.com\/MedCofQbank\/\" \/>\n<meta property=\"article:published_time\" content=\"2026-02-18T21:00:00+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/Blog-MedCof-2026-02-18T155526.579.jpg\" \/>\n\t<meta property=\"og:image:width\" content=\"1920\" \/>\n\t<meta property=\"og:image:height\" content=\"1080\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/jpeg\" \/>\n<meta name=\"author\" content=\"Kiara Adelino\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:label1\" content=\"Escrito por\" \/>\n\t<meta name=\"twitter:data1\" content=\"Kiara Adelino\" \/>\n\t<meta name=\"twitter:label2\" content=\"Est. tempo de leitura\" \/>\n\t<meta name=\"twitter:data2\" content=\"7 minutos\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"Article\",\"@id\":\"https:\\\/\\\/www.grupomedcof.com.br\\\/blog\\\/hiperplasia-adrenal-congenita\\\/#article\",\"isPartOf\":{\"@id\":\"https:\\\/\\\/www.grupomedcof.com.br\\\/blog\\\/hiperplasia-adrenal-congenita\\\/\"},\"author\":{\"name\":\"Kiara Adelino\",\"@id\":\"https:\\\/\\\/www.grupomedcof.com.br\\\/blog\\\/#\\\/schema\\\/person\\\/db19a651f86c5fc202d6eee744fd3292\"},\"headline\":\"Hiperplasia Adrenal Cong\u00eanita: fisiopatologia, espectro cl\u00ednico e mais!\",\"datePublished\":\"2026-02-18T21:00:00+00:00\",\"mainEntityOfPage\":{\"@id\":\"https:\\\/\\\/www.grupomedcof.com.br\\\/blog\\\/hiperplasia-adrenal-congenita\\\/\"},\"wordCount\":995,\"publisher\":{\"@id\":\"https:\\\/\\\/www.grupomedcof.com.br\\\/blog\\\/#organization\"},\"image\":{\"@id\":\"https:\\\/\\\/www.grupomedcof.com.br\\\/blog\\\/hiperplasia-adrenal-congenita\\\/#primaryimage\"},\"thumbnailUrl\":\"https:\\\/\\\/www.grupomedcof.com.br\\\/blog\\\/wp-content\\\/uploads\\\/2026\\\/02\\\/Blog-MedCof-2026-02-18T155526.579.jpg\",\"keywords\":[\"Efeito Gargalo\"],\"articleSection\":[\"Pediatria\"],\"inLanguage\":\"pt-BR\"},{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/www.grupomedcof.com.br\\\/blog\\\/hiperplasia-adrenal-congenita\\\/\",\"url\":\"https:\\\/\\\/www.grupomedcof.com.br\\\/blog\\\/hiperplasia-adrenal-congenita\\\/\",\"name\":\"Hiperplasia Adrenal Cong\u00eanita: fisiopatologia, espectro cl\u00ednico e mais! 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