Palabras clave
Insuficiencia suprarrenal
Alacrimia
Síndrome de Allgrove
Reporte de caso Esophageal Achalasia
Adrenal Insufficiency
Lacrimal Apparatus Diseases
Allgrove Syndrome
Case report
Cómo citar
Resumen
El síndrome de Allgrove es una enfermedad huérfana, con patrón de herencia autosómica recesiva caracterizada por insuficiencia suprarrenal, acalasia y alacrimia. Se describe el caso de un paciente masculino preescolar previamente sano, que ingresa a urgencias de una institución de 4to nivel de atención con deshidratación y mal estado general. El paciente completó 25 días hospitalizado en donde presentó múltiples paros cardiorrespiratorios, neumonía aspirativa, hipoglicemia sintomática persistente y pobre respuesta a corticoides, por lo que se sospechó una enfermedad metabólica. En consulta ambulatoria por endocrinología pediátrica llama la atención características adicionales como: hipercromía generalizada de la piel, alacrimia e inadecuada ganancia de peso y talla, sin reflujo gastroesofágico o acalasia. El síndrome de Allgrove es una patología con un amplio espectro de manifestaciones que se debe tener en cuenta a pesar de no presentarse con la triada usual.
Citas
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