RESUMO
BACKGROUND: A 31-year-old woman with tuberous sclerosis complex presented with a 1 week history of subjective fever, chills, rigors, poor appetite and dizziness. INVESTIGATIONS: Physical examination, urine and blood analysis, CT of the abdomen, chest and brain, and chest X-ray. DIAGNOSIS: End-stage renal disease, septic shock and urinary tract infection secondary to huge bilateral angiomyolipomas of the kidney associated with tuberous sclerosis complex. MANAGEMENT: Antibiotic therapy, vasopressor treatment and bilateral nephrectomy, followed by hemodialysis while awaiting renal transplantation.
Assuntos
Falência Renal Crônica/etiologia , Esclerose Tuberosa/complicações , Adulto , Angiomiolipoma/complicações , Angiomiolipoma/etiologia , Angiomiolipoma/cirurgia , Antibacterianos/uso terapêutico , Progressão da Doença , Feminino , Humanos , Falência Renal Crônica/terapia , Neoplasias Renais/complicações , Neoplasias Renais/etiologia , Neoplasias Renais/cirurgia , Nefrectomia , Diálise Renal , Vasoconstritores/uso terapêuticoRESUMO
A 14-year-old male was referred for dyslipidemia. His findings were consistent with metabolic syndrome. Although he lacked the typical physical appearance, his accelerated weight gain combined with a decreased linear growth velocity suggested Cushing syndrome. He was subsequently found to have adrenocorticotropic hormone-independent Cushing syndrome secondary to primary pigmented nodular adrenal disease without Carney Complex. After bilateral adrenalectomy, his lipid profile returned to normal. In this article, we discuss the role of glucocorticoids on lipid and lipoprotein metabolism.