Recovery of hypothalamic‐pituitary‐adrenal axis in paediatric Cushing disease

Tatsi, Christina; Neely, Megan L.; Flippo, Chelsi; Bompou, Maria‐Eleni; Keil, Meg; Stratakis, Constantine A.

doi:10.1111/cen.14300

Cited by 14 publications

(17 citation statements)

References 30 publications

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“…Previous reports on factors predicting recovery of the HPA axis have been inconsistent [ 72 ]. The NIH study also reported, for the first time in paediatric CD, a statistically significant relationship ( p = 0.0342) between a shorter HPA axis recovery time, and the likelihood of recurrence of CD [ 82 ], as had been shown in adults [ 83 ]. All patients with recurrence of hypercortisolism had recovery of the axis by 15 months post-TSS.…”

Section: Treatment Of Paediatric Cushing’s Diseasementioning

confidence: 88%

“…The adrenal insufficiency (AI) which follows complete removal of an ACTH-secreting adenoma requires physiological glucocorticoid replacement with hydrocortisone 8–12 mg/m 2 /day, and may persist for many months. A recent study from the NIH looked at factors potentially influencing the duration of AI and recovery of the HPA axis [ 82 ]. In 102 paediatric CD patients who recovered adrenal function following post-TSS remission, the median time to recovery was 12.3 months with a range of 3–35 months.…”

Section: Treatment Of Paediatric Cushing’s Diseasementioning

confidence: 99%

“…The study also associated long-term remission with younger age, smaller adenoma, and morning serum cortisol of <1 μg/dL after surgery [ 36 ]. The current recurrence rate of paediatric patients treated at the NIH is 11.5% at a median of 44 months (range: 6.3–97 months) after TSS [ 82 ].…”

Section: Recurrence Of Cushing’s Disease After Remissionmentioning

confidence: 99%

See 2 more Smart Citations

Paediatric Cushing’s disease: Epidemiology, pathogenesis, clinical management and outcome

Ferrigno

Hasenmajer

Caiulo

et al. 2021

Rev Endocr Metab Disord

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Cushing’s disease (CD) is rare in paediatric practice but requires prompt investigation, diagnosis and therapy to prevent long-term complications. Key presenting features are a change in facial appearance, weight gain, growth failure, virilization, disturbed puberty and psychological disturbance. Close consultation with an adult endocrinology department is recommended regarding diagnosis and therapy. The incidence of CD, a form of ACTH-dependent Cushing’s syndrome (CS), is equal to approximately 5% of that seen in adults. The majority of ACTH-secreting adenomas are monoclonal and sporadic, although recent studies of pituitary tumours have shown links to several deubiquitination gene defects. Diagnosis requires confirmation of hypercortisolism followed by demonstration of ACTH-dependence. Identification of the corticotroph adenoma by pituitary MRI and/or bilateral inferior petrosal sampling for ACTH may contribute to localisation before pituitary surgery. Transsphenoidal surgery (TSS) with selective microadenomectomy is first-line therapy, followed by external pituitary irradiation if surgery is not curative. Medical therapy to suppress adrenal steroid synthesis is effective in the short-term and bilateral adrenalectomy should be considered in cases unfit for TSS or radiotherapy or when urgent remission is needed after unsuccessful surgery. TSS induces remission of hypercortisolism and improvement of symptoms in 70–100% of cases, particularly when performed by a surgeon with experience in children. Post-TSS complications include pituitary hormone deficiencies, sub-optimal catch-up growth, and persisting excess of BMI. Recurrence of hypercortisolism following remission is recognised but infrequent, being less common than in adult CD patients. With experienced specialist medical and surgical care, the overall prognosis is good. Early referral to an experienced endocrine centre is advised.

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Section: Treatment Of Paediatric Cushing’s Diseasementioning

confidence: 88%

Section: Treatment Of Paediatric Cushing’s Diseasementioning

confidence: 99%

See 1 more Smart Citation

Paediatric Cushing’s disease: Epidemiology, pathogenesis, clinical management and outcome

Ferrigno

Hasenmajer

Caiulo

et al. 2021

Rev Endocr Metab Disord

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“…Non-functional pituitary macroadenomas (more than one centimeter in diameter) comprise about one-third of all pituitary adenomas and are managed with trans sphenoidal surgery [ 1 , 2 ]. Restoration of normal pituitary function after surgery is important and has been reported in various studies from 20 to 50% [ 3 , 4 ]. Panhypopituitarism, deficiency in production of at least two pituitary hormones, is a most common complication after pituitary surgery [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Magnetic resonance imaging characteristics predict pituitary function in non-functional pituitary macro-adenoma undergoing trans-sphenoidal surgery

et al. 2022

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Introduction Maintaining the pituitary function after surgery is highly important. The aim of this study was to investigate the relationship between preoperative magnetic resonance imaging (MRI) characteristics and pituitary function after surgery of non-functional pituitary macroadenoma. Methods This retrospective study was performed between 2016 and 2018. Preoperative and postoperative MRI imaging data were retrieved from electronic registration system. The relationship between preoperative MRI characteristics and postoperative pituitary function as well as reconstruction of pituitary gland was investigated using regression models. Results Complete data were available for 44 patients. Before surgery, invisible normal tissue was observed in 23 patients (53.5%). Suprasellar extension and cavernous sinus invasion were seen in 36 patients (each one 49.1%). There was a significant reverse relationship between preoperative tumor size and postoperative thyroid stimulating hormone (TSH) (odds ratio (OR): − 0.99 (− 0.18, − 0.003), p = 0.04). In addition, we found a significant positive correlation between prolactin level after surgery and tumor size before surgery, (OR: 5.29 (1.65, 8.92), p = 0006). Moreover, postoperative panhypopituitarism was observed in 25% of patients with complete morphologic reconstitution of pituitary tissue. While the rate was 50% in patients with no or partial morphologic reconstruction of pituitary tissue. Conclusion Preoperative MRI characteristics predict TSH and prolactin level after operation. Furthermore, the adenoma size and volume prior to surgery are the main determinants of normal morphologic reconstruction of pituitary gland.

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“…Non-functional pituitary macroadenomas (more than one centimeter in diameter) comprise about onethird of all pituitary adenomas and heal with trans sphenoidal surgery (1,2). Restoration of normal pituitary function after surgery is important and has been reported in various studies from 20-50% (3,4). Panhypopituitarism, de ciency in production of at least two pituitary hormones, is a most common complication after pituitary surgery (5).…”

Section: Introductionmentioning

confidence: 99%

Magnetic Resonance Imaging Characteristics Predict Pituitary Function in Non-Functional Pituitary Macro-Adenoma Undergoing Trans-Sphenoidal Surgery

Hassani

Hashemi-Madani

Kachuee

et al. 2021

Preprint

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Introduction: Maintaining the pituitary function after surgery is highly important. The aim of this study was to investigate the relationship between preoperative magnetic resonance imaging (MRI) characteristics and pituitary function after surgery of non-functional pituitary macroadenoma Methods: This retrospective study was performed between from 2016 and to 2018 Preoperative and postoperative MRI imaging data were retrieved from electronic registration system. The relationship between MRI characteristics and pituitary function was investigated using regression models.Results: Complete data were available for 44 patients. Before surgery, invisible normal tissue was observed in 23 patients (53.5%). Suprasellar extension and cavernous sinus invasion were seen in 36 patients (each one 49.1%). There was a significant reverse relationship between preoperative tumor size and thyroid stimulating hormone (TSH) ( Odds Ratio (OR): -0.99 (-0.18, -0.003), p=0.04). in addition we found a significant positive correlation between prolactin level after surgery and tumor size before surgery, (OR: 5.29 (1.65, 8.92), p= 0006) . Moreover, postoperative panhypopituitarism was observed in 25% of patients with complete reconstruction of pituitary tissue. While the rate was 50% in patients with no or partial reconstruction of pituitary tissue. Conclusion: Preoperative MRI characteristics predict TSH and prolactin level after operation. Furthermore, the adenoma size and volume prior to surgery are the main determinants of normal reconstruction of pituitary gland (NRPG).

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Recovery of hypothalamic‐pituitary‐adrenal axis in paediatric Cushing disease

Cited by 14 publications

References 30 publications

Paediatric Cushing’s disease: Epidemiology, pathogenesis, clinical management and outcome

Paediatric Cushing’s disease: Epidemiology, pathogenesis, clinical management and outcome

Magnetic resonance imaging characteristics predict pituitary function in non-functional pituitary macro-adenoma undergoing trans-sphenoidal surgery

Magnetic Resonance Imaging Characteristics Predict Pituitary Function in Non-Functional Pituitary Macro-Adenoma Undergoing Trans-Sphenoidal Surgery

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