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Diabetes Insípida

Diabetes Insípida

A diabetes Diabetes Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus insípida ( DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus)) é uma doença que se caracteriza pela incapacidade renal em concentrar a urina. Existem 2 subformas de DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus): DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus) central ( DIC DIC Disseminated intravascular coagulation (DIC) is a condition characterized by systemic bodywide activation of the coagulation cascade. This cascade results in both widespread microvascular thrombi contributing to multiple organ dysfunction and consumption of clotting factors and platelets, leading to hemorrhage. Disseminated Intravascular Coagulation) e DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus) nefrogénica (DIN). Na DIC DIC Disseminated intravascular coagulation (DIC) is a condition characterized by systemic bodywide activation of the coagulation cascade. This cascade results in both widespread microvascular thrombi contributing to multiple organ dysfunction and consumption of clotting factors and platelets, leading to hemorrhage. Disseminated Intravascular Coagulation, a quantidade de hormona antidiurética (ADH, pela sigla em inglês) produzida pelo hipotálamo ou libertada pela hipófise está diminuída. Na DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus) nefrogénica, os rins não respondem à ADH circulante. Ambas as condições resultam na incapacidade dos rins em concentrar a urina, causando poliúria, noctúria e polidipsia. DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus) central e nefrogénica são diferenciadas com base na medição dos níveis de osmolalidade sérica de sódio e urina e na resposta ao teste de privação de água. A DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus) central é tratada com desmopressina, enquanto a DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus) nefrogénica é tratada com diuréticos e restrição de sal na dieta.

Last updated: Dec 15, 2025

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Contents

Epidemiologia

  • Prevalência total: 1 em cada 25.000 pessoas
  • A diabetes Diabetes Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus insípida central ( DIC DIC Disseminated intravascular coagulation (DIC) is a condition characterized by systemic bodywide activation of the coagulation cascade. This cascade results in both widespread microvascular thrombi contributing to multiple organ dysfunction and consumption of clotting factors and platelets, leading to hemorrhage. Disseminated Intravascular Coagulation) é mais MAIS Androgen Insensitivity Syndrome comum do que a diabetes Diabetes Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus insípida nefrogénica (DIN).
  • Apenas 1 em cada 10 casos de DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus) é congénito.
  • Homens e mulheres são igualmente afetados.
  • 20% dos pacientes submetidos a neurocirurgia desenvolverão algum grau de DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus).

Fisiopatologia

Papel da hormona antidiurética (ADH)

A hormona antidiurética também é conhecida como vasopressina.

Função:

A hormona antidiurética regula a osmolalidade sérica e a pressão arterial.

  • Aumenta a absorção de água livre, resultando em:
    • ↓ Osmolalidade sérica
    • ↑ Osmolalidade urinária
    • ↑ Volume intravascular
  • Vasoconstrição

Produção:

  • Sintetizada no núcleo supraótico do hipotálamo
  • Armazenada e secretada pela hipófise posterior
  • Secretada em resposta a:
    • Aumento da osmolalidade plasmática (detetada pelos osmorrecetores (vasopressina tipo 2 (V₂)) no hipotálamo)
    • A hipovolemia (detetada por barorrecetores (vasopressina tipo 1 (V1)) no seio carotídeo e arco aórtico)
Regulação e produção de ADH

Regulação e via de produção da ADH

Imagem por Lecturio.

DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus) central

A diabetes Diabetes Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus insípida central é provocada pela produção insuficiente de ADH pelo hipotálamo ou pela libertação insuficiente da hipófise posterior.

  • Idiopática
  • Adquirida
    • Autoimune
    • Tumores hipofisários ou secundários
      • Craniofaringioma
      • Adenoma
    • Neurocirurgia ou traumatismo craniano
    • Doença infiltrativa
      • Sarcoidose
      • Histiocitose de células de Langerhans
    • Encefalopatia hipóxica
    • Meningite
    • Intoxicação por álcool
  • Congénita (raro)
    • Hipopituitarismo congénito
    • Síndrome de Wolfram

DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus) nefrogénica

A diabetes Diabetes Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus insípida nefrogénica é provocada por uma resposta insuficiente dos rins à ADH.

  • Adquirida
    • Tratamento a longo prazo com lítio
    • Hipercalcemia
    • Gravidez
    • Hipocalemia
    • Outros fármacos (antivíricos, antifúngicos, antibióticos, fármacos antineoplásicos)
    • Doença renal aguda ou crónica
      • Doença renal poliquística autossómica dominante (DRPAD)
      • Amiloidose renal
      • Síndrome de Bardet-Biedl
      • Síndrome de Bartter
      • Síndrome de Sjögren
    • A forma ligeira é frequentemente encontrada em idosos (declínio da função renal com a idade).
  • Congénita ( mais MAIS Androgen Insensitivity Syndrome comum)
    • Causa provável se a DIN se apresentar na infância
    • Mais MAIS Androgen Insensitivity Syndrome comum: mutações no gene Gene A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms. Basic Terms of Genetics que codifica V2
      • Transmissão hereditária ligada ao X
    • Menos comum: mutações no gene Gene A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms. Basic Terms of Genetics que codifica os canais da aquaporina (AQP)
      • Transmissão hereditária autossómica recessiva/autossómica dominante

Related videos

5:24
Actions of ADH
4:36
Antidiuretic Hormone (ADH) and Osmolality
9:43
Central Diabetes Insipidus
9:59
Diabetes Insipidus (DI): Etiology

Apresentação Clínica e Diagnóstico

Apresentação clínica

A DIC DIC Disseminated intravascular coagulation (DIC) is a condition characterized by systemic bodywide activation of the coagulation cascade. This cascade results in both widespread microvascular thrombi contributing to multiple organ dysfunction and consumption of clotting factors and platelets, leading to hemorrhage. Disseminated Intravascular Coagulation e DIN apresentam os mesmos sintomas:

  • Poliúria
    • Definida como > 3 L de débito urinário/dia
  • Noctúria (provocando sonolência diurna)
  • Polidipsia (secundária ao aumento do sódio sérico e da osmolalidade plasmática)
  • Os sintomas neurológicos podem ocorrer secundários à hipernatremia.
    • Irritabilidade
    • Coma Coma Coma is defined as a deep state of unarousable unresponsiveness, characterized by a score of 3 points on the GCS. A comatose state can be caused by a multitude of conditions, making the precise epidemiology and prognosis of coma difficult to determine. Coma, se grave

Diagnóstico

  • Teste de privação hídrica
    • A osmolalidade plasmática e urinária são medidas antes da restrição hídrica.
    • Sem ingestão de água por 2–3 horas.
    • Após este intervalo, são medidas a osmolalidade plasmática e urinária.
    • Medições a cada hora da osmolalidade plasmática e urinária
    • Se não existir aumento da osmolalidade urinária, é administrado um análogo da ADH (desmopressina).
      • Se a osmolalidade urinária aumentar → DIC DIC Disseminated intravascular coagulation (DIC) is a condition characterized by systemic bodywide activation of the coagulation cascade. This cascade results in both widespread microvascular thrombi contributing to multiple organ dysfunction and consumption of clotting factors and platelets, leading to hemorrhage. Disseminated Intravascular Coagulation (= a ausência de secreção central de ADH é compensada pela administração de desmopressina)
      • Se a osmolalidade urinária permanecer baixa → DIN (= defeito renal que impede a atuação da desmopressina com o seu efeito ADH)
  • Teste adicional:
    • Tomografia computorizada (TC) de crânio ou ressonância magnética (RM) (se suspeita de DIC DIC Disseminated intravascular coagulation (DIC) is a condition characterized by systemic bodywide activation of the coagulation cascade. This cascade results in both widespread microvascular thrombi contributing to multiple organ dysfunction and consumption of clotting factors and platelets, leading to hemorrhage. Disseminated Intravascular Coagulation)
    • Revisão de fármacos (sais de lítio, foscarnet Foscarnet An antiviral agent used in the treatment of cytomegalovirus retinitis. Foscarnet also shows activity against human herpesviruses and HIV. Antivirals for Herpes Virus, clozapina)
Craniofaringioma

TC de crânio de um craniofaringioma (massa quística calcificada): estima-se que a diabetes insípida ocorra em até 35% dos pacientes antes da cirurgia e 70 %–90% após a cirurgia.

Imagem: “Craniopharyngioma1” por Matthew R Garnett, Stéphanie Puget, Jacques Grill, Christian Sainte-Rose. Craniopharyngioma. Orphanet Journal of Rare Diseases. Licença: CC BY 2.0

Tratamento

DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus) central

  • Tratamento médico:
    • Desmopressina (1.ª linha)
    • Fármacos com efeito antidiurético (raramente usados)
      • Anti-inflamatórios não esteróides (AINEs), diuréticos tiazídicos, carbamazepina
      • Menos eficazes e com mais MAIS Androgen Insensitivity Syndrome efeitos adversos do que a desmopressina
  • Nutricional:
    • Dieta com baixo teor de sódio e redução proteica
    • Hidratação
    • Se as alterações eletrolíticas não normalizarem através da ingestão de água oral: dextrose Dextrose Intravenous Fluids EV com água ou fluidos hiposmolares EV
    • Considerações especiais em crianças:
      • O tratamento precoce é importante devido aos efeitos danosos da hipernatremia
      • Dar água a cada 2 horas (de dia e de noite)
      • Monitorizar a ingesta e o crescimento
      • Não está recomendado fazer uma dieta hipoproteica

DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus) nefrogénica

  • Tratamento médico:
    • Hidroclorotiazida
    • Indometacina
    • Amilorida
    • Evitar o agente agressor ( mais MAIS Androgen Insensitivity Syndrome frequentemente o lítio) → a função renal pode voltar ao normal após a descontinuação.
  • Nutricional: o mesmo que a DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus) central

Related videos

12:25
Diabetes Insipidus (DI): Management

Diagnóstico Diferencial

  • Diabetes Diabetes Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus mellitus: uma doença metabólica crónica caracterizada por resistência à insulina (tipo 2) ou produção insuficiente de insulina (tipo 1), resultando em hiperglicemia e poliúria pela diurese osmótica. Também pode apresentar-se com polidipsia pelo aumento da diurese. É expectável que os pacientes apresentem glicose sérica elevada e glicosúria.
  • Polidipsia psicogénica: ingestão excessiva de fluidos sem uma causa orgânica identificável, frequentemente encontrada em indivíduos com esquizofrenia, perturbações de ansiedade ou anorexia Anorexia The lack or loss of appetite accompanied by an aversion to food and the inability to eat. It is the defining characteristic of the disorder anorexia nervosa. Anorexia Nervosa nervosa. Este consumo excessivo de fluidos resulta em poliúria e, em casos graves, hiponatremia. O teste de privação hídrica é caracterizado por um aumento na osmolalidade urinária após a restrição de fluidos, o que o diferencia da DI DI Diabetes insipidus (DI) is a condition in which the kidneys are unable to concentrate urine. There are 2 subforms of di: central di (CDI) and nephrogenic di (NDI). Both conditions result in the kidneys being unable to concentrate urine, leading to polyuria, nocturia, and polydipsia. Arginine Vasopressin Disorders (Diabetes Insipidus).

Referências

  1. Bichet, D. G., & Verbalis, J. G. (2022). Arginine vasopressin deficiency and resistance. In J. L. Jameson, A. S. Fauci, D. L. Kasper, S. L. Hauser, D. L. Longo, & J. Loscalzo (Eds.), Harrison’s principles of internal medicine (21st ed., pp. 2895-2905). McGraw-Hill Education.
  2. Angelousi, A., Alexandraki, K. I., Mytareli, C., Lekka, A., Tziritis, E., & Grossman, A. (2023). New developments and concepts in the diagnosis and management of diabetes insipidus (AVP-deficiency and resistance). Journal of Neuroendocrinology, 35(4), e13233. https://doi.org/10.1111/jne.13233
  3. Bichet, D. G., & Verbalis, J. G. (2025, February 27). Arginine vasopressin deficiency (central diabetes insipidus): Etiology, clinical manifestations, and postdiagnostic evaluation. UpToDate. Retrieved June 8, 2025, from https://www.uptodate.com/contents/arginine-vasopressin-deficiency-central-diabetes-insipidus-etiology-clinical-manifestations-and-postdiagnostic-evaluation
  4. Bichet, D. G. (2025, February 27). Arginine vasopressin deficiency (central diabetes insipidus): Treatment. UpToDate. Retrieved June 8, 2025, from https://www.uptodate.com/contents/arginine-vasopressin-deficiency-central-diabetes-insipidus-treatment 
  5. Bichet, D. G., & Verbalis, J. G. (2024, May 8). Arginine vasopressin resistance (nephrogenic diabetes insipidus): Etiology, clinical manifestations, and postdiagnostic evaluation. UpToDate. Retrieved June 8, 2025, from https://www.uptodate.com/contents/arginine-vasopressin-resistance-nephrogenic-diabetes-insipidus-etiology-clinical-manifestations-and-postdiagnostic-evaluation 
  6. Bichet, D. G. (2025, February 27). Arginine vasopressin resistance (nephrogenic diabetes insipidus): Treatment. UpToDate. Retrieved June 8, 2025, from https://www.uptodate.com/contents/arginine-vasopressin-resistance-nephrogenic-diabetes-insipidus-treatment 
  7. Bockenhauer, D., van Hoeck, K., Ammenti, A., Ashton, E., Balling, R., Bayazit, A., … & Wühl, E. (2024). International expert consensus statement on the diagnosis and management of congenital nephrogenic diabetes insipidus. Nature Reviews Nephrology, 20(9), 615-629. https://doi.org/10.1038/s41581-024-00897-z 
  8. Flynn, K., Hatfield, J., Brown, K., Vietor, N., & Hoang, T. (2025). Central and nephrogenic diabetes insipidus: Updates on diagnosis and management. Frontiers in Endocrinology, 15, 1479764. https://doi.org/10.3389/fendo.2024.1479764 
  9. Tomkins, M., Lawless, S., Martin-Grace, J., Sherlock, M., & Thompson, C. J. (2022). Diagnosis and management of central diabetes insipidus in adults. Journal of Clinical Endocrinology & Metabolism, 107(10), 2701-2715. https://doi.org/10.1210/clinem/dgac381
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