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Residual manifestations of hypercortisolemia following surgical treatment in a patient with Cushing syndrome.

Bartz SK, Karaviti LP, Brandt ML, Lopez ME, Masand P, Devaraj S, Hicks J, Anderson L, Lodish M, Keil M, Stratakis CA - Int J Pediatr Endocrinol (2015)

Bottom Line: 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.Resolution of the source of hypercortisolemia does not imply regression of hypertension or recovery of the immune system.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Endocrinology and Metabolism, Texas Children's Hospital, Houston, TX USA.

ABSTRACT

Context: 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.

Patient: 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.

Conclusions and outcome: 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.

No MeSH data available.


Related in: MedlinePlus

a CT angiogram of the abdomen demonstrating thickened limbs of the adrenal glands with nodular contour. Arrows point to adrenal glands. b Magnetic resonance imaging (MRI) of the abdomen confirmed the presence of thickened nodular adrenal glands with no evidence of a dominant nodule to suggest adenoma or adrenal cortical carcinoma. Arrows point to the two adrenal glands
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Fig3: a CT angiogram of the abdomen demonstrating thickened limbs of the adrenal glands with nodular contour. Arrows point to adrenal glands. b Magnetic resonance imaging (MRI) of the abdomen confirmed the presence of thickened nodular adrenal glands with no evidence of a dominant nodule to suggest adenoma or adrenal cortical carcinoma. Arrows point to the two adrenal glands

Mentions: Imaging studies obtained included an MRI of the brain showing no increase in the adenohypophyseal size, and no evidence of a pituitary adenoma. CT scan of the abdomen revealed diffusely thickened limbs of both adrenal glands, with a nodular contour. MRI was performed in an attempt to get a more discriminating view of the adrenal glands. (Fig. 3). MRI again demonstrated uniformly thickened adrenal glands with no evidence of an adenoma or adreno-cortical carcinoma. A skeletal survey was negative for fibrous dysplasia. Bilateral breast ultrasound and echocardiogram were negative for myxomas and fibromas.Fig. 3


Residual manifestations of hypercortisolemia following surgical treatment in a patient with Cushing syndrome.

Bartz SK, Karaviti LP, Brandt ML, Lopez ME, Masand P, Devaraj S, Hicks J, Anderson L, Lodish M, Keil M, Stratakis CA - Int J Pediatr Endocrinol (2015)

a CT angiogram of the abdomen demonstrating thickened limbs of the adrenal glands with nodular contour. Arrows point to adrenal glands. b Magnetic resonance imaging (MRI) of the abdomen confirmed the presence of thickened nodular adrenal glands with no evidence of a dominant nodule to suggest adenoma or adrenal cortical carcinoma. Arrows point to the two adrenal glands
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4551381&req=5

Fig3: a CT angiogram of the abdomen demonstrating thickened limbs of the adrenal glands with nodular contour. Arrows point to adrenal glands. b Magnetic resonance imaging (MRI) of the abdomen confirmed the presence of thickened nodular adrenal glands with no evidence of a dominant nodule to suggest adenoma or adrenal cortical carcinoma. Arrows point to the two adrenal glands
Mentions: Imaging studies obtained included an MRI of the brain showing no increase in the adenohypophyseal size, and no evidence of a pituitary adenoma. CT scan of the abdomen revealed diffusely thickened limbs of both adrenal glands, with a nodular contour. MRI was performed in an attempt to get a more discriminating view of the adrenal glands. (Fig. 3). MRI again demonstrated uniformly thickened adrenal glands with no evidence of an adenoma or adreno-cortical carcinoma. A skeletal survey was negative for fibrous dysplasia. Bilateral breast ultrasound and echocardiogram were negative for myxomas and fibromas.Fig. 3

Bottom Line: 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.Resolution of the source of hypercortisolemia does not imply regression of hypertension or recovery of the immune system.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Endocrinology and Metabolism, Texas Children's Hospital, Houston, TX USA.

ABSTRACT

Context: 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.

Patient: 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.

Conclusions and outcome: 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.

No MeSH data available.


Related in: MedlinePlus