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Long-term follow-up of Cushing's disease:a case report.

Baş VN, Ağladıoğlu SY, Onder A, Ozışık P, Peltek Kendirci HN, Cetinkaya S, Aycan Z - J Clin Res Pediatr Endocrinol (2013)

Bottom Line: It is rare in childhood.While adrenal axis suppression continued for approximately 1 year, clinical improvement was clearly observed after the third month following surgery.The findings in this patient demonstrate that decreased growth rate despite rapid weight gain in children can be early sign of Cushing's disease and emphasize the importance of monitoring of growth in obese children.

View Article: PubMed Central - PubMed

Affiliation: Sami Ulus Women Health, Children's Education and Research Hospital, Clinics of Pediatric Endocrinology, Ankara, Turkey. E-mail: veyselbas@yahoo.com.

ABSTRACT
Cushing's disease is a condition in which hypercortisolism develops due to excessive hypophyseal adrenocorticotropic hormone production. It is rare in childhood. In this paper, we report the case of a 10-year-old male patient with hypophyseal microadenoma-related Cushing's disease who presented with obesity and was found to show poor height growth at follow-up. The diagnosis was confirmed with inferior petrosal sinus sampling, and the adenoma was successfully removed by transsphenoidal surgery. While adrenal axis suppression continued for approximately 1 year, clinical improvement was clearly observed after the third month following surgery. The findings in this patient demonstrate that decreased growth rate despite rapid weight gain in children can be early sign of Cushing's disease and emphasize the importance of monitoring of growth in obese children.

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General appearance of the case before the operation
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f1: General appearance of the case before the operation

Mentions: The 10-year-old male patient presented to the clinic with a complaint of rapid weight gain over the past year. There was a history of type 2 diabetes in the family. The patient was prepubertal. His blood pressure was 100/60 mm/Hg. Height was 134.6 cm [-0.4 standard deviation (SD)] and body weight was 48.1 kg (+1.9 SD). Body mass index (BMI) was 26.3 (+2.3 SD). Physical examination revealed acanthosis nigricans on the neck (Figure 1a). The patient’s biochemical parameters were within normal range, and his morning and evening cortisol levels were 16.9 mcg/dL and 3.2 mcg/dL, respectively. An oral glucose tolerance test was performed, and the results were consistent with hyperinsulinism and insulin resistance. The patient was started on metformin treatment. On the 6th month of follow-up, the patient had a height of 135.6 cm (-0.7 SD). Body weight was 52.6 kg, and body mass index was 28.6 (+2.6 SD). Noting the inadequacy of a 1-cm increase in height despite the 4-kg increase in body weight, serum cortisol levels (day-night) were repeated and the results revealed a disrupted cortisol rhythm with a morning serum cortisol level of 23 mcg/dL and an evening cortisol level of 10 mcg/dL, along with elevated 24-hour urinary free cortisol levels assessed over three days (652 mcg/day, 884 mcg/day, and 674 mcg/day, respectively). Cushing’s syndrome was suspected due to lack of suppression in the low-dose dexamethasone suppression test (ACTH: 16.2 pg/mL, cortisol: 16 mcg/dL), and the diagnosis of Cushing’s disease was confirmed by the demonstration of suppression in the high-dose dexamethasone suppression test (ACTH: 5.9 pg/mL, cortisol: 1.6 mcg/dL). The evaluation of the case in terms of the systemic effects of hypercortisolism showed normal bone mineral density and no hypertensive eye-ground changes. The 24-hour blood pressure readings were consistent with systolic and diastolic hypertension. The hypophyseal magnetic resonance imaging results were reported to be normal, and the results of inferior petrosal sinus sampling were consistent with rightward lateralized microadenoma (Table 1). The pathology results of the microadenoma that was removed by transsphenoidal surgery were evaluated to be consistent with ACTH-secreting adenoma. Hydrocortisone treatment was continued(7 mg/m2/day) due to the ongoing suppression of the adrenal axis following surgery. The growth rate of the patient increased up to 6 cm/year over the duration of the one-year follow-up, and his BMI decreased from +2.6 to +0.6 (Figure 1b). On the 12th month of treatment, the peak serum cortisol response of 28 mcg/dL in a low-dose ACTH stimulation test was determined to be sufficient, and the hydrocortisone treatment that was initially started at physiological dosage was gradually stopped. The physical examination findings and basal cortisol measurement values in the follow-up period are presented in Table 2.


Long-term follow-up of Cushing's disease:a case report.

Baş VN, Ağladıoğlu SY, Onder A, Ozışık P, Peltek Kendirci HN, Cetinkaya S, Aycan Z - J Clin Res Pediatr Endocrinol (2013)

General appearance of the case before the operation
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3814537&req=5

f1: General appearance of the case before the operation
Mentions: The 10-year-old male patient presented to the clinic with a complaint of rapid weight gain over the past year. There was a history of type 2 diabetes in the family. The patient was prepubertal. His blood pressure was 100/60 mm/Hg. Height was 134.6 cm [-0.4 standard deviation (SD)] and body weight was 48.1 kg (+1.9 SD). Body mass index (BMI) was 26.3 (+2.3 SD). Physical examination revealed acanthosis nigricans on the neck (Figure 1a). The patient’s biochemical parameters were within normal range, and his morning and evening cortisol levels were 16.9 mcg/dL and 3.2 mcg/dL, respectively. An oral glucose tolerance test was performed, and the results were consistent with hyperinsulinism and insulin resistance. The patient was started on metformin treatment. On the 6th month of follow-up, the patient had a height of 135.6 cm (-0.7 SD). Body weight was 52.6 kg, and body mass index was 28.6 (+2.6 SD). Noting the inadequacy of a 1-cm increase in height despite the 4-kg increase in body weight, serum cortisol levels (day-night) were repeated and the results revealed a disrupted cortisol rhythm with a morning serum cortisol level of 23 mcg/dL and an evening cortisol level of 10 mcg/dL, along with elevated 24-hour urinary free cortisol levels assessed over three days (652 mcg/day, 884 mcg/day, and 674 mcg/day, respectively). Cushing’s syndrome was suspected due to lack of suppression in the low-dose dexamethasone suppression test (ACTH: 16.2 pg/mL, cortisol: 16 mcg/dL), and the diagnosis of Cushing’s disease was confirmed by the demonstration of suppression in the high-dose dexamethasone suppression test (ACTH: 5.9 pg/mL, cortisol: 1.6 mcg/dL). The evaluation of the case in terms of the systemic effects of hypercortisolism showed normal bone mineral density and no hypertensive eye-ground changes. The 24-hour blood pressure readings were consistent with systolic and diastolic hypertension. The hypophyseal magnetic resonance imaging results were reported to be normal, and the results of inferior petrosal sinus sampling were consistent with rightward lateralized microadenoma (Table 1). The pathology results of the microadenoma that was removed by transsphenoidal surgery were evaluated to be consistent with ACTH-secreting adenoma. Hydrocortisone treatment was continued(7 mg/m2/day) due to the ongoing suppression of the adrenal axis following surgery. The growth rate of the patient increased up to 6 cm/year over the duration of the one-year follow-up, and his BMI decreased from +2.6 to +0.6 (Figure 1b). On the 12th month of treatment, the peak serum cortisol response of 28 mcg/dL in a low-dose ACTH stimulation test was determined to be sufficient, and the hydrocortisone treatment that was initially started at physiological dosage was gradually stopped. The physical examination findings and basal cortisol measurement values in the follow-up period are presented in Table 2.

Bottom Line: It is rare in childhood.While adrenal axis suppression continued for approximately 1 year, clinical improvement was clearly observed after the third month following surgery.The findings in this patient demonstrate that decreased growth rate despite rapid weight gain in children can be early sign of Cushing's disease and emphasize the importance of monitoring of growth in obese children.

View Article: PubMed Central - PubMed

Affiliation: Sami Ulus Women Health, Children's Education and Research Hospital, Clinics of Pediatric Endocrinology, Ankara, Turkey. E-mail: veyselbas@yahoo.com.

ABSTRACT
Cushing's disease is a condition in which hypercortisolism develops due to excessive hypophyseal adrenocorticotropic hormone production. It is rare in childhood. In this paper, we report the case of a 10-year-old male patient with hypophyseal microadenoma-related Cushing's disease who presented with obesity and was found to show poor height growth at follow-up. The diagnosis was confirmed with inferior petrosal sinus sampling, and the adenoma was successfully removed by transsphenoidal surgery. While adrenal axis suppression continued for approximately 1 year, clinical improvement was clearly observed after the third month following surgery. The findings in this patient demonstrate that decreased growth rate despite rapid weight gain in children can be early sign of Cushing's disease and emphasize the importance of monitoring of growth in obese children.

Show MeSH
Related in: MedlinePlus