title: Residual manifestations of hypercortisolemia following surgical treatment in a patient with Cushing syndrome.
abstract: BACKGROUND
  Cushing Syndrome is difficult to diagnose, and the comorbidities and persistent late effects of hypercortisolemia after treatment of the primary disease are challenging for the patient and the endocrinologist.
  
  
  OBJECTIVE
  To report the case of a girl with obesity and hypertension, ultimately diagnosed with Cushing syndrome due to primary pigmented nodular adrenocortical disease. In this case, the complications of hypercortisolism persisted short term despite surgical intervention.
  
  
  METHODS
  A 4 year old morbidly obese African-American girl with developmental delay presented with hypertensive emergency in the ER and 18-month history of progressive weight gain. Her previous history included premature adrenarche, hypertension, seizures and a random high cortisol with suppressed ACTH. She was subsequently stabilized, and a diagnostic work-up persistently demonstrated elevated cortisol and suppressed ACTH. An abdominal MRI showed bilateral adrenal multinodular disease, consistent with multinodular hyperplasia of the adrenal glands. Based on these findings the patient underwent a bilateral adrenalectomy, which confirmed primary pigmented nodular adrenocortical disease. The patient had a complicated, protracted post-operative course requiring adjustment of therapy for persistent hypertension. Two months after surgery, she was readmitted to the Emergency Department with hyperpyrexia and hypertension and succumbed to the complications of sepsis.
  This case highlights the significant diagnostic and therapeutic challenges in treating children with Cushing syndrome. Resolution of the source of hypercortisolemia does not imply regression of hypertension or recovery of the immune system. Although the child underwent bilateral adrenalectomy, persistent consequences of prolonged severe hypercortisolism contributed to her death two months later.
authors: Department of Pediatric Endocrinology and Metabolism, Texas Children's Hospital, Houston, TX USA.
authors: Sara K
authors: SK
authors: Bartz
authors: Department of Pediatric Endocrinology and Metabolism, Texas Children's Hospital, Houston, TX USA.
authors: Lefkothea P
authors: LP
authors: Karaviti
authors: Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Texas Children's Hospital, Houston, TX USA.
authors: Mary L
authors: ML
authors: Brandt
authors: Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Texas Children's Hospital, Houston, TX USA.
authors: Monica E
authors: ME
authors: Lopez
authors: Department of Radiology, Texas Children's Hospital, Houston, TX USA.
authors: Prakash
authors: P
authors: Masand
authors: Medical Director of Clinical Chemistry and POCT, Texas Children's Hospital and Baylor College of Medicine, Houston, TX USA.
authors: Sridevi
authors: S
authors: Devaraj
authors: Department of Pathology, Texas Children's Hospital and Baylor College of Medicine, Houston, TX USA.
authors: John
authors: J
authors: Hicks
authors: Baylor College of Medicine, Houston, TX USA.
authors: Lauren
authors: L
authors: Anderson
authors: The Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), NIH, Houston, TX USA.
authors: Maya
authors: M
authors: Lodish
authors: The Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), NIH, Houston, TX USA.
authors: Meg
authors: M
authors: Keil
authors: The Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), NIH, Houston, TX USA.
authors: Constantine A
authors: CA
authors: Stratakis
keywords: Cushing syndrome; Obesity; PPNAD; Primary pigmented nodular adrenocortical disease