Glucagonoma

Definição e Epidemiologia

Definição

Um glucagonoma Glucagonoma A glucagonoma is a glucagon-secreting neuroendocrine tumor that originates from the β-cells in the pancreatic islets. Most glucagonomas are malignant, and many of them are part of the autosomal dominant condition known as multiple endocrine neoplasia syndrome type 1 (MEN 1). Elevated levels of glucagon lead to increased gluconeogenesis and glycogenolysis. Glucagonoma é um tumor Tumor Inflammation secretor de glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions com origem nas células α das ilhotas pancreáticas que surge, por vezes, no contexto de MEN 1.

Epidemiologia

• Incidência:
  • 0,1 a 1 caso por 10 milhões
  • Razão homens : mulheres = 1 : 1
• Idade máxima ao diagnóstico: 45-70 anos
• 50% – 80% metastático ao diagnóstico
• Tamanho e localização:
  • 50% – 80% surgem na cauda do pâncreas (↑ número de células α).
  • Normalmente > 3 cm
• Associação com MEN 1:
  • 10% – 20% dos glucagonomas estão associados a MEN 1
  • Os glucagonomas aparecem em apenas 3% dos doentes com MEN 1.
• Casos esporádicos: 80% – 90%

Fisiopatologia

Fisiologia normal do glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions

Secretado pelas células α nas ilhotas pancreáticas, o papel do glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions é tornar a energia prontamente disponível através da produção de glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance e da interrupção do seu armazenamento.

• Estimula:
  • Glicogenólise: metabolização do glicogénio → glicose
  • Gluconeogénese: produção de glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance a partir de aminoácidos e de lípidos
  • Lipólise: metabolização dos lípidos → ácidos gordos + glicerol → produção de energia
    • Ácidos gordos → cetogénese
    • Glicerol → ácido pirúvico → ciclo do ácido cítrico
  • Efeito final: ↑ glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance
• Inibe:
  • Glicogénese: produção de glicogénio
  • Síntese de ácidos gordos
  • Síntese de colesterol
• Outros efeitos do glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions:
  • Relaxamento do músculo liso GI → usado em algumas técnicas de imagiologia radiológica GI
  • Efeitos inotrópicos positivos → usado para tratar a toxicidade dos beta-bloqueadores e dos bloqueadores dos canais de cálcio
• Normalmente é secretado em resposta a:
  • Hipoglicemia e jejum
  • Péptido inibitório gástrico
  • Grelina
• A secreção é inibida por:
  • Hiperglicemia
  • Insulina
  • Somatostatina

Fisiopatologia dos glucagonomas

• ↑ desregulado do glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions:
  • Hiperglicemia
  • ↓ Aminoácidos (usados para produzir glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance)
  • ↓ Reservas lipídicas (usadas para produzir glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance)
• Função das células β pancreáticas preservada:
  • A produção de insulina permanece sob controlo normal.
  • ↑ Glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance → ↑ insulina → 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
• Expressam grandes quantidades de recetores de somatostatina (inibidores)
• Locais comuns de metastização:
  • Fígado (1º)
  • Gânglios linfáticos regionais
  • Osso
  • Glândulas suprarrenais
  • Rins
  • Pulmões

Fisiopatologia da MEN 1

Os glucagonomas são incomuns, mas 10% – 20% dos que são encontrados estão associados a MEN 1.

• Defeito autossómico dominante 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 MEN 1 (supressor tumoral)
• Predispõe os doentes a tumores nas seguintes localizações:
  • Glândulas paratiroides
  • Células das ilhotas pancreáticas
  • Hipófise
• Tumores da paratiroide:
  • Hiperparatiroidismo primário → ↑ cálcio
  • Quase universais na MEN 1
  • Sintomas de hipercalcémia:
    • ↓ Densidade mineral óssea
    • Litíase renal
    • Poliúria e polidipsia
    • Obstipação
• Tumores pancreáticos:
  • Gastrinoma Gastrinoma A gastrinoma is a tumor that secretes excessive levels of the hormone gastrin and is responsible for Zollinger-Ellison syndrome (ZES). Gastrinomas are frequently associated with multiple endocrine neoplasia 1 (MEN 1) and can arise from the pancreas, stomach, duodenum, jejunum, and/or even from the lymph nodes. Gastrinoma / síndrome de Zollinger-Ellison ( mais MAIS Androgen Insensitivity Syndrome comum): ↑ gastrina → úlceras pépticas múltiplas
  • Insulinoma Insulinoma A benign tumor of the pancreatic beta cells. Insulinoma secretes excess insulin resulting in hypoglycemia. Pancreatic Neuroendocrine Tumors (PanNETs) (comum): ↑ insulina → ↓ glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance
  • Glucagonoma Glucagonoma A glucagonoma is a glucagon-secreting neuroendocrine tumor that originates from the β-cells in the pancreatic islets. Most glucagonomas are malignant, and many of them are part of the autosomal dominant condition known as multiple endocrine neoplasia syndrome type 1 (MEN 1). Elevated levels of glucagon lead to increased gluconeogenesis and glycogenolysis. Glucagonoma: ↑ glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions → ↑ glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance
  • Somatostatinoma Somatostatinoma A somatostatin-secreting tumor derived from the pancreatic delta cells (somatostatin-secreting cells). It is also found in the intestine. Somatostatinomas are associated with diabetes mellitus; cholelithiasis; steatorrhea; and hypochlorhydria. The majority of somatostatinomas have the potential for metastasis. Pancreatic Neuroendocrine Tumors (PanNETs): inibe uma variedade de processos GI
  • VIPoma VIPoma A VIPoma is a rare neuroendocrine tumor arising primarily in the pancreas that releases large amounts of vasoactive intestinal polypeptide (VIP). This process leads to chronic watery diarrhea with concomitant hypokalemia and dehydration, as well as wheezing and flushing (known as Verner-Morrison or WDHA syndrome). VIPoma: ↑ péptido intestinal vasoativo ( VIP VIP A highly basic, 28 amino acid neuropeptide released from intestinal mucosa. It has a wide range of biological actions affecting the cardiovascular, gastrointestinal, and respiratory systems and is neuroprotective. It binds special receptors. Gastrointestinal Neural and Hormonal Signaling) → regula a atividade do músculo liso e o fluxo sanguíneo no trato gastrointestinal
  • Tumores não funcionantes
• Adenomas hipofisários (hipófise anterior):
  • Prolactinoma Prolactinoma A pituitary adenoma which secretes prolactin, leading to hyperprolactinemia. Clinical manifestations include amenorrhea; galactorrhea; impotence; headache; visual disturbances; and cerebrospinal fluid rhinorrhea. Hyperprolactinemia ( mais MAIS Androgen Insensitivity Syndrome comum) → ↑ prolactina
  • Outros podem secretar:
    • Hormona do crescimento (GH)
    • Hormona adrenocorticotrópica (ACTH)
    • Hormona estimulante da tiroide (TSH)

Apresentação Clínica

A apresentação dos glucagonomas pode ser confundida com a de muitas outras doenças, e o diagnóstico final da maioria dos casos só é confirmado quando há disseminação metastática da doença.

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

• 75% – 95% dos casos
• Normalmente sem cetoacidose diabética (função das células β e secreção de insulina preservada)
• Sintomas:
  • Poliúria
  • Polidipsia
  • Visão turva
  • Perda de peso

Eritema necrolítico migratório (ENM)

• 70% dos casos
• Não é específico dos glucagonomas
• Erupção cutânea dolorosa e pruriginosa
• As lesões aumentam e diminuem
• Localização:
  • Começa nas zonas intertriginosas (áreas onde a pele se toca) e periorificiais
  • Numa fase tardia, generalizado: virilha, períneo, nádegas, abdómen inferior, extremidades e rosto
• Progressão da aparência das lesões:
  • Placas e pápulas eritematosas
  • As lesões aumentam e coalescem
  • Clareira central e erosão
  • Endurecimento central com bolhas, crostas e bordas descamativas (pode assemelhar-se a impétigo)
• Manifestações nas mucosas:
  • Estomatite (inflamação da boca)
  • Queilite (inflamação dos lábios)
  • Glossite (inflamação da língua)
• Acredita-se que seja causado por:
  • ↓ Aminoácidos
  • Défices nutricionais

Outros achados frequentes

• Perda de peso:
  • Característica mais MAIS Androgen Insensitivity Syndrome comum à apresentação (80% dos casos)
  • Devido ao ↑ da lipólise e da gluconeogénese a partir dos lípidos
• Diarreia crónica
• Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types
• Trombose venosa profunda (TVP) / eventos tromboembólicos
• Manifestações neuropsiquiátricas:
  • Depressão
  • Demência
  • Psicose

Mnemónica

Para recordar as manifestações clínicas de um glucagonoma Glucagonoma A glucagonoma is a glucagon-secreting neuroendocrine tumor that originates from the β-cells in the pancreatic islets. Most glucagonomas are malignant, and many of them are part of the autosomal dominant condition known as multiple endocrine neoplasia syndrome type 1 (MEN 1). Elevated levels of glucagon lead to increased gluconeogenesis and glycogenolysis. Glucagonoma, lembrar os 6 Ds:

• Diabetes (ou resistência à insulina devido à hiperglicemia)
• Dermatite (ENM)
• Diminuição do peso
• Diarreia
• Trombose venosa profunda (deep vein thrombosis Thrombosis Formation and development of a thrombus or blood clot in the blood vessel. Epidemic Typhus, em inglês)
• Depressão

Diagnóstico

O diagnóstico de glucagonoma Glucagonoma A glucagonoma is a glucagon-secreting neuroendocrine tumor that originates from the β-cells in the pancreatic islets. Most glucagonomas are malignant, and many of them are part of the autosomal dominant condition known as multiple endocrine neoplasia syndrome type 1 (MEN 1). Elevated levels of glucagon lead to increased gluconeogenesis and glycogenolysis. Glucagonoma é feito com base numa combinação entre a história clínica, exame objetivo, níveis elevados de glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions e imagem que mostra uma massa pancreática. O estadiamento da doença maligna é também uma parte importante do processo de diagnóstico, uma vez que as metástases estão frequentemente presentes ao diagnóstico.

Análises Laboratoriais

• ↑ Níveis de glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions: geralmente > 500 pg/ml
• ↑ Glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance
• Hemograma: anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types normocítica
• Painel metabólico:
  • Diarreia crónica → alterações eletrolíticas
  • Hiperparatiroidismo na MEN 1 → ↑ cálcio
• Biópsia de pele:
  • Células epidérmicas acantolíticas
  • Infiltrado linfocítico e neutrofílico

Imagiologia

• Objetivo:
  • Localizar o tumor Tumor Inflammation com precisão
  • Determinar a extensão da doença
• Tomografia computadorizada com contraste IV:
  • Melhor método inicial de diagnóstico
  • Alta sensibilidade e especificidade
  • Achados:
    • Massa pancreática, geralmente na cauda
    • Tipicamente > 3 cm
• Ressonância magnética:
  • Pode ter maior sensibilidade para metástases hepáticas que a TC
  • Achados:
    • ↓ Intensidade em imagens ponderadas em T1
    • ↑ Intensidade em imagens ponderadas em T2
• Cintigrafia do recetor de somatostatina:
  • Exame para deteção do análogo da somatostatina radiomarcado (octreótido)
  • Os glucagonomas têm ↑ recetores de somatostatina e ligam-se rapidamente ao octreótido.
  • Permite a localização do tumor Tumor Inflammation e a deteção de metástases
  • Está a ser substituído por PETs funcionais
• PET PET An imaging technique that combines a positron-emission tomography (PET) scanner and a ct X ray scanner. This establishes a precise anatomic localization in the same session. Nuclear Imaging funcional:
  • Utilizado para a localização do tumor Tumor Inflammation e deteção de metástases
  • Melhor resolução, quantificação, especificidade e sensibilidade que a cintigrafia do recetor de somatostatina

Estadiamento e prognóstico

• O estadiamento é feito utilizando o sistema TNM:
  • Tamanho do tumor Tumor Inflammation
  • Extensão da invasão local
  • Envolvimento dos gânglios linfáticos
  • Presença e localização das metástases
• O prognóstico é determinado de acordo com o estadio.
• A cura raramente é possível a partir do momento em que há metastização.
• Taxas de sobrevivência a 5 anos:
  • Estadio I: 61%
  • Estadio II: 52%
  • Estadio III: 41%
  • Estadio IV: 15%

Tratamento

Tratamento inicial

• Controlar 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.
• Suporte nutricional (pode exigir nutrição parentérica total)
• Análogos da somatostatina:
  • Octreótido
  • Inibe a secreção de glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions.
  • Melhora o ENM, 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, a diarreia e os sintomas neurológicos
• Profilaxia de TVP:
  • Enoxaparina
  • Heparina

Tratamento avançado

• Resseção pancreática (se localizado no pâncreas)
• Resseção hepática (se envolvimento limitado do fígado)
• Embolização da artéria hepática (metástases mais MAIS Androgen Insensitivity Syndrome extensas):
  • Técnica paliativa
  • Permite infundir fármacos de quimioterapia na artéria hepática durante o procedimento
• Ablação por radiofrequência das lesões hepáticas pequenas
• Quimioterapia
• Terapêutica-alvo molecular para a doença avançada:
  • Sunitinibe (inibidor da tirosina cinase)
  • Everolimus Everolimus A derivative of sirolimus and an inhibitor of tor serine-threonine kinases. It is used to prevent graft rejection in heart and kidney transplant patients by blocking cell proliferation signals. It is also an antineoplastic agent. Immunosuppressants (inibidor do alvo mamífero da rapamicina ( mTOR mTOR Peutz-Jeghers Syndrome))

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: doença causada por um comprometimento da secreção e / ou função da insulina que leva à hiperglicemia: os doentes geralmente apresentam sintomas clássicos de hiperglicemia, incluindo poliúria, polidipsia, visão turva e perda de peso. Tanto 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 mellitus como os glucagonomas apresentam hiperglicemia persistente, mas deve-se suspeitar de glucagonoma Glucagonoma A glucagonoma is a glucagon-secreting neuroendocrine tumor that originates from the β-cells in the pancreatic islets. Most glucagonomas are malignant, and many of them are part of the autosomal dominant condition known as multiple endocrine neoplasia syndrome type 1 (MEN 1). Elevated levels of glucagon lead to increased gluconeogenesis and glycogenolysis. Glucagonoma se houver achados dermatológicos, diarreia crónica ou TVP. O glucagonoma Glucagonoma A glucagonoma is a glucagon-secreting neuroendocrine tumor that originates from the β-cells in the pancreatic islets. Most glucagonomas are malignant, and many of them are part of the autosomal dominant condition known as multiple endocrine neoplasia syndrome type 1 (MEN 1). Elevated levels of glucagon lead to increased gluconeogenesis and glycogenolysis. Glucagonoma pode ser confirmado através do doseamento dos níveis de glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions e da imagiologia.
• Cirrose: fibrose hepática progressiva crónica que leva à insuficiência hepática: a cirrose é frequentemente causada por hepatite vírica crónica, doença hepática alcoólica e esteatose hepática não-alcoólica. Esta doença apresenta-se com icterícia, prurido, hemorragia digestiva alta, ascite e/ou perda de peso. Embora raro, o ENM pode ocorrer na cirrose, mas as telangiectasias Telangiectasias Ataxia-telangiectasia aracneiformes são manifestações cutâneas muito mais MAIS Androgen Insensitivity Syndrome comuns. A cirrose também pode causar níveis elevados de glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions, embora na cirrose estes níveis sejam geralmente< 500 pg/ml, em comparação com glucagonomas, em que os níveis são frequentemente> 500 pg/ml. A cirrose é diagnosticada por ecografia.
• Pancreatite aguda: inflamação aguda do pâncreas, que se apresenta tipicamente com dor epigástrica grave e elevação dos níveis séricos de lipase Lipase An enzyme of the hydrolase class that catalyzes the reaction of triacylglycerol and water to yield diacylglycerol and a fatty acid anion. It is produced by glands on the tongue and by the pancreas and initiates the digestion of dietary fats. Malabsorption and Maldigestion ou amilase (2–3 vezes o limite superior do normal): o glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions pode estar aumentado na pancreatite aguda e a imagem por TC ou RM mostra um aumento focal ou difuso do pâncreas. Ao contrário da pancreatite aguda, os glucagonomas tipicamente não são dolorosos.
• Desnutrição ou deficiências nutricionais: deficiência de zinco, pelagra e kwashiorkor Kwashiorkor A syndrome produced by severe protein deficiency, characterized by retarded growth, changes in skin and hair pigment, edema, and pathologic changes in the liver, including fatty infiltration, necrosis, and fibrosis. The word is a local name in gold coast, africa, meaning ‘displaced child’. Although first reported from africa, kwashiorkor is now known throughout the world, but mainly in the tropics and subtropics. It is considered to be related to marasmus. Malnutrition in children in resource-limited countries podem-se apresentar com erupção cutânea semelhante ao ENM. O tratamento inicial envolve a correção das deficiências subjacentes. No entanto, nenhuma das condições listadas está geralmente associada a hiperglicemia e a níveis elevados de glucagon Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal glucagon-like peptides. Glucagon is secreted by pancreatic alpha cells and plays an important role in regulation of blood glucose concentration, ketone metabolism, and several other biochemical and physiological processes. Gastrointestinal Secretions, o que permite a sua distinção dos glucagonomas (além das diferenças na história clínica).
  • A deficiência de zinco está associada a doenças de má absorção (por exemplo, doença inflamatória intestinal) e pode causar hipogonadismo, disfunção imunológica, dificuldade de cicatrização de feridas, alopécia, perda do paladar e diversas lesões cutâneas, incluindo uma erupção cutânea semelhante ao ENM.
  • A pelagra é causada pela deficiência de niacina e pode manifestar-se por uma erupção cutânea semelhante ao ENM, diarreia e demência.
  • Kwashiorkor Kwashiorkor A syndrome produced by severe protein deficiency, characterized by retarded growth, changes in skin and hair pigment, edema, and pathologic changes in the liver, including fatty infiltration, necrosis, and fibrosis. The word is a local name in gold coast, africa, meaning ‘displaced child’. Although first reported from africa, kwashiorkor is now known throughout the world, but mainly in the tropics and subtropics. It is considered to be related to marasmus. Malnutrition in children in resource-limited countries é uma doença de desnutrição aguda. Esta doença é observada tipicamente em crianças e resulta em edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema periférico simétrico, com início nas regiões mais MAIS Androgen Insensitivity Syndrome dependentes.