Long-term anterior pituitary function in patients with paediatric Cushing’s disease treated with pituitary radiotherapy

Chan, LF; Storr, Helen L; Plowman, P. N.; Perry, Leslie; Besser, G. M.; Grossman, Ashley; Savage, Martin O.

doi:10.1530/eje-06-0588

Cited by 36 publications

(34 citation statements)

References 42 publications

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“…Post-therapeutic anterior pituitary function in children and adolescents following TSS alone and in combination with pituitary RT shows that isolated GHD is the most common anterior pituitary hormone defect seen, occurring in 56 and 83% respectively, although this can be transient (10,33). Our results are comparable to this.…”

Section: Discussionsupporting

confidence: 80%

Diagnosis, management and therapeutic outcome in prepubertal Cushing's disease

Dias¹,

Kumaran²,

Chan³

et al. 2010

European Journal of Endocrinology

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Objectives: Cushing's disease (CD) in prepubertal children is very rare and presents important diagnostic and therapeutic challenges. We report experience of the management of this subpopulation of CD patients. Study design/methods: Retrospective patient case note review. Results: Between 1985 and 2008, 17 prepubertal children (13M, 4F), aged 5.7-14.1 years presented to our centre for diagnosis and management of CD. All children had subnormal linear growth and excessive weight gain at presentation. A high proportion (85% of males, 75% of females) had evidence of excessive virilisation. Striae and hypertension were seen in 41% of patients. The investigation with highest sensitivity (100%) for CD was excessive increase of serum cortisol to i.v. CRH (mean increase 113%). Pituitary imaging performed in all the patients showed poor concordance with findings at surgery (31%). In contrast bilateral simultaneous inferior petrosal sinus sampling (BSIPSS), performed in 11/16 subjects showed a high correlation with surgical findings (91%). In 16 patients, transsphenoidal selective adenomectomy (TSS) achieved a cure rate of 44%. However, in the 11 patients who had pre-operative BSIPSS, the cure rate was 64%. Of the 16 patients, 9 patients who were not cured by TSS received external pituitary radiotherapy. Conclusions: Prepubertal CD had distinctive features with increased frequency in males, abnormal auxology and excessive virilisation. The cortisol response to i.v. CRH administration was particularly exuberant and contributed to diagnosis. BSIPSS was much more helpful than pituitary imaging in localisation of the microadenoma and was associated with improved cure rate by TSS.

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Section: Discussionsupporting

confidence: 80%

Diagnosis, management and therapeutic outcome in prepubertal Cushing's disease

Dias¹,

Kumaran²,

Chan³

et al. 2010

European Journal of Endocrinology

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“…Another long-term study of patients with CD who had received radiotherapy showed that GH deficiency was frequent but could recover, gonadotropin secretion was usually preserved with early or normal puberty and TSH and ACTH deficiencies were rare [6].…”

Section: Discussionmentioning

confidence: 99%

Management of Cushing syndrome in children and adolescents: experience of a single tertiary centre

et al. 2016

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What is Known:-Cushing syndrome is an extremely rare entity in the paediatric and adolescent age groups, so not many cohort studies have been published in this population.-Several tests can be employed to firstly diagnose hypercortisolaemia and secondly, identify the source of origin of it. The efficacy and safety of these tests in children is still uncertain. What is New:-This study includes cases due to the different aetiologies of endogenous hypercortisolaemia (pituitary, adrenal and ectopic hypercortisolaemia) allowing us to compare the differences in presentation, diagnosis, management and long-term outcome between the groups.-There is a difference in the prevalence of Cushing syndrome symptoms and in the performance of the tests in our cohort compared to previously published studies in the literature. ABSTRACT:The diagnosis and management of paediatric Cushing syndrome (CS) is highly challenging. This study aims to characterise its presentation, diagnosis, management and outcome by a retrospective case review of 30 patients (14 females) followed at a single tertiary paediatric endocrinology centre over a 30-year period.At presentation, median age was 8.9 years (0.2-15.5) and the commonest manifestations were weight gain (23/30), hirsutism (17/30), acne (15/30) and hypertension (15/30). Growth retardation was present in 11/30. Median BMI was +2.1SDS (-6.5 -+4.6). Urinary Free Cortisol (UFC) was abnormal in 17/18 (94%), midnight cortisol in 27/27 (100%) and Low Dose Dexamethasone Suppression (LDDS) test in 20/20 (100%). High Dose Dexamethasone Suppression (HDDS) test was abnormal in: 6/6 (100%) of adrenal tumours, 1/10 (10%) of Cushing Disease (CD) and 1/2 (50%) of ectopic tumours. Bilateral Inferior Petrosal Sinus Sampling (IPSS) identified 5 CD cases and 1 ectopic tumour.All patients underwent surgery and subsequently required cortisol replacement. Final diagnoses were 16 CD, 11 adrenal disease, 2 ectopic ACTH-secreting lesions and 1 case of unidentified aetiology. One year post-diagnosis, median BMI was 0.5SDS (-2.5 -+3.7), hypertension was present in 4/14 (28%) and 43% (12/30) of individuals were off hydrocortisone. Conclusion:The prevalence of the clinical manifestations differs from that reported in other series.Screening tests were highly sensitive, with UFC, midnight cortisol and LDDS performing well. One year post-treatment, BMI and BP normalized in the majority of patients, and almost half of them were able to discontinue replacement hydrocortisone.

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“…We have treated 12 patients during the past 25 years with a successful cure rate of 92%, which occurred at a mean interval of 0.83 years (range 0.13–2.86) following completion of therapy. We have recently analysed long-term pituitary function in 6 of these patients and have shown that although GH deficiency was frequent initially, some recovery may occur [41]. Gonadotrophin secretion was generally preserved with normal, or early [42] puberty, and TSH and ACTH deficiency was minimal.…”

Section: Treatment Of Cushing’s Diseasementioning

confidence: 99%

Advances in the Management of Paediatric Cushing’s Disease

Savage

Chan²,

Afshar³

et al. 2008

Horm Res Paediatr

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Cushing’s disease (CD) is rare in the paediatric age range, but may present a difficult therapeutic challenge. Most paediatric endocrinologists have limited experience managing children or adolescents with CD and thus benefit from close consultation with adult colleagues. Prior to definitive treatment, a diagnostic protocol for investigation is required which broadly follows the model for adult patients. Treatment strategies for CD are described and critically appraised. The management of paediatric CD patients after cure also presents challenges for optimizing growth, bone health, reproduction and body composition from childhood into and during adult life.

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Long-term anterior pituitary function in patients with paediatric Cushing’s disease treated with pituitary radiotherapy

Cited by 36 publications

References 42 publications

Diagnosis, management and therapeutic outcome in prepubertal Cushing's disease

Diagnosis, management and therapeutic outcome in prepubertal Cushing's disease

Management of Cushing syndrome in children and adolescents: experience of a single tertiary centre

Advances in the Management of Paediatric Cushing’s Disease

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