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A patient with Cushing disease lateralizing a pituitary adenoma by inferior petrosal sinus sampling using desmopressin: a case report.

Lim JH, Kim SJ, Jung MK, Kim KE, Kwon AR, Chae HW, Kim DH, Kim HS - Ann Pediatr Endocrinol Metab (2016)

Bottom Line: During the previous 2 years, she had experienced weight gain, secondary amenorrhea, growth retardation, and back pain.Pituitary magnetic resonance imaging was suspicious for microadenoma.To eliminate ectopic ACTH syndrome, and lateralize the pituitary tumor, inferior petrosal sinus sampling (IPSS) was performed by desmopressin use to stimulate ACTH.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Severance Children's Hospital, Endocrine Research Institute, Yonsei University College of Medicine, Seoul, Korea.

ABSTRACT
A 14-year-old girl was referred for evaluation of the etiology of Cushing syndrome. During the previous 2 years, she had experienced weight gain, secondary amenorrhea, growth retardation, and back pain. Random serum cortisol level, 24-hour urinary free cortisol excretion, and overnight and low-dose dexamethasone suppression tests suggested Cushing syndrome. Midnight adrenocorticotropic hormone (ACTH) level and high-dose dexamethasone suppression test confirmed Cushing disease. Pituitary magnetic resonance imaging was suspicious for microadenoma. To eliminate ectopic ACTH syndrome, and lateralize the pituitary tumor, inferior petrosal sinus sampling (IPSS) was performed by desmopressin use to stimulate ACTH. Finally, the patient was diagnosed with Cushing disease due to ACTH-secreting pituitary microadenoma, lateralized to the left side; subsequently underwent transsphenoidal surgery. Here we report a case of a 14-year-old girl diagnosed with Cushing disease with a pituitary tumor lateralized by IPSS using desmopressin, which is very rare in pediatric Cushing disease.

No MeSH data available.


Related in: MedlinePlus

Initial morphology suggesting Cushing syndrome, including moon-shaped face (A), a buffalo hump (B), truncal obesity, and abdominal striae (C).
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Figure 1: Initial morphology suggesting Cushing syndrome, including moon-shaped face (A), a buffalo hump (B), truncal obesity, and abdominal striae (C).

Mentions: On admission, the patient's height and weight were 142 cm (<3rd percentile) and 53.5 kg (50th–75th percentile), and her BMI was 26.58 kg/m2. Hypertension (149/109 mmHg) was observed with a normal heart rate (96 beats/min), body temperature (36.9℃), and respiratory rate (16 breaths/min). She appeared chronically ill, with diffusely thin, wrinkled skin with plethora. She displayed a moon-shaped face (Fig. 1A), a buffalo hump (Fig. 1B), truncal obesity, acanthosis nigricans, and abdominal striae (Fig. 1C).


A patient with Cushing disease lateralizing a pituitary adenoma by inferior petrosal sinus sampling using desmopressin: a case report.

Lim JH, Kim SJ, Jung MK, Kim KE, Kwon AR, Chae HW, Kim DH, Kim HS - Ann Pediatr Endocrinol Metab (2016)

Initial morphology suggesting Cushing syndrome, including moon-shaped face (A), a buffalo hump (B), truncal obesity, and abdominal striae (C).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4835561&req=5

Figure 1: Initial morphology suggesting Cushing syndrome, including moon-shaped face (A), a buffalo hump (B), truncal obesity, and abdominal striae (C).
Mentions: On admission, the patient's height and weight were 142 cm (<3rd percentile) and 53.5 kg (50th–75th percentile), and her BMI was 26.58 kg/m2. Hypertension (149/109 mmHg) was observed with a normal heart rate (96 beats/min), body temperature (36.9℃), and respiratory rate (16 breaths/min). She appeared chronically ill, with diffusely thin, wrinkled skin with plethora. She displayed a moon-shaped face (Fig. 1A), a buffalo hump (Fig. 1B), truncal obesity, acanthosis nigricans, and abdominal striae (Fig. 1C).

Bottom Line: During the previous 2 years, she had experienced weight gain, secondary amenorrhea, growth retardation, and back pain.Pituitary magnetic resonance imaging was suspicious for microadenoma.To eliminate ectopic ACTH syndrome, and lateralize the pituitary tumor, inferior petrosal sinus sampling (IPSS) was performed by desmopressin use to stimulate ACTH.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Severance Children's Hospital, Endocrine Research Institute, Yonsei University College of Medicine, Seoul, Korea.

ABSTRACT
A 14-year-old girl was referred for evaluation of the etiology of Cushing syndrome. During the previous 2 years, she had experienced weight gain, secondary amenorrhea, growth retardation, and back pain. Random serum cortisol level, 24-hour urinary free cortisol excretion, and overnight and low-dose dexamethasone suppression tests suggested Cushing syndrome. Midnight adrenocorticotropic hormone (ACTH) level and high-dose dexamethasone suppression test confirmed Cushing disease. Pituitary magnetic resonance imaging was suspicious for microadenoma. To eliminate ectopic ACTH syndrome, and lateralize the pituitary tumor, inferior petrosal sinus sampling (IPSS) was performed by desmopressin use to stimulate ACTH. Finally, the patient was diagnosed with Cushing disease due to ACTH-secreting pituitary microadenoma, lateralized to the left side; subsequently underwent transsphenoidal surgery. Here we report a case of a 14-year-old girl diagnosed with Cushing disease with a pituitary tumor lateralized by IPSS using desmopressin, which is very rare in pediatric Cushing disease.

No MeSH data available.


Related in: MedlinePlus