Cushing Disease in Children and Adolescents: Twenty Years’ Experience in A Tertiary Care Center in India

Shah, Nalini; George, Joe; Acharya, Shrikrishna V.; Lila, Anurag; Sarathi, Vijaya; Bandgar, Tushar; Jalali, Rakesh; Goel, Atul; Menon, P. S. N.

doi:10.4158/ep10143.or

Cited by 28 publications

(30 citation statements)

References 34 publications

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“…Since CD mainly affects adults, there were few studies about IPSS in children and adolescents, as summarized in Table 4. The sensitivity of IPSS at baseline in our study was lower than previous publications reporting sensitivities from 75.9 to 100% in children and adolescents [6–8, 16]. While the study by Batista et al [6] and by Magiakou et al [7] reported sensitivity of over 90%, the study by Storr et al [8] reported a much lower sensitivity of 75.9%.…”

Section: Discussioncontrasting

confidence: 86%

“…Therefore, the clinical usefulness of BIPSS for tumor lateralization in our cases was limited. As reported by previous studies in children and adolescents (Table 4), sampling lateralization rates by BIPSS ranged from 73.7 to 100%, and their accordant rates with actual tumor lateralization were 58.7–100% [5–8, 16, 18, 19], both of which were higher than our data. It should be noticed that the criteria of calculating the tumor lateralization accuracy in these studies were slightly different.…”

Section: Discussionsupporting

confidence: 53%

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The effects of sampling lateralization on bilateral inferior petrosal sinus sampling and desmopressin stimulation test for pediatric Cushing's disease

Chen

Lü

et al. 2018

Endocrine

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PurposeBilateral inferior petrosal sinus sampling (BIPSS) is useful for differential diagnosis of adult Cushing’s disease (CD) but may not be so reliable in pediatric cases. The purpose of this study was to evaluate the sensitivity of BIPSS before and after desmopressin stimulation in pediatric CD, and to explore related factors of false-negative results and meanings of sampling lateralization.MethodsWe retrospectively analyzed 16 pediatric CD patients who underwent 17 BIPSS procedures from 2006 to 2017. CD was diagnosed if inferior petrosal sinus (IPS) to peripheral adrenocorticotropic hormone (ACTH) ratio was >2 at baseline or >3 after desmopressin stimulation. Sampling lateralization was yielded if interpetrosal sinus gradient was >1.4. Magnetic resonance imaging (MRI) was conducted. All the patients underwent surgery and the diagnosis was confirmed.ResultsThe sensitivity was 64.7% (11/17) at baseline and 83.3% (10/12) after desmopressin stimulation. After stimulation, BIPSS reached its best sensitivity at 3 min. Sampling lateralization rate was 62.5% and 63.6% before and after stimulation, and the accordant rate with actual tumor lateralization was 50.0% and 42.9%, respectively. The accuracy of MRI in predicting the tumor lateralization was 80.0%. Sampling lateralization rate (81.8% in true-positive, 20.0% in false-negative, p = 0.036) and ACTH at dominant IPS (p = 0.001) was lower among false-negative patients.ConclusionsThe sensitivity of BIPSS in pediatric CD was low at baseline, but increased after desmopressin stimulation. Sampling lateralization cannot accurately indicate the tumor lateralization, but the absence of sampling lateralization with low ACTH at IPS is a hint of false-negative cases in BIPSS.

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Section: Discussioncontrasting

confidence: 86%

Section: Discussionsupporting

confidence: 53%

The effects of sampling lateralization on bilateral inferior petrosal sinus sampling and desmopressin stimulation test for pediatric Cushing's disease

Chen

Lü

et al. 2018

Endocrine

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“…Differences in management protocols and definitions of remission or ‘cure’ have led to rates of successful treatment reported from 60 to 98 % [1, 8, 10, 11]. Variation is also seen for rates of recurrence after remission, ranging from 6 to 27 % [10, 12, 13].…”

Section: Introductionmentioning

confidence: 99%

Long-term outcomes of children treated for Cushing’s disease: a single center experience

et al. 2016

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PurposePediatric Cushing’s disease (CD) is rare and there are limited data on the long-term outcomes. We assessed CD recurrence, body composition, pituitary function and psychiatric comorbidity in a cohort of pediatric CD patients.MethodsRetrospective review of 21 CD patients, mean age at diagnosis 12.1 years (5.7–17.8), managed in our center between 1986 and 2010. Mean follow-up from definitive treatment was 10.6 years (2.9–27.2).ResultsFifteen patients were in remission following transsphenoidal surgery (TSS) and 5 were in remission following TSS + external pituitary radiotherapy (RT). One patient underwent bilateral adrenalectomy (BA). CD recurrence occurred in 3 (14.3 %) patients: 2 at 2 and 6 years after TSS and 1 7.6 years post-RT. The BA patient developed Nelson’s syndrome requiring pituitary RT 0.6 years post-surgery. Short-term growth hormone deficiency (GHD) was present in 14 patients (81 % patients tested) (11 following TSS and 3 after RT) and 4 (44 % of tested) had long-term GHD. Gonadotropin deficiency caused impaired pubertal development in 9 patients (43 %), 4 requiring sex steroid replacement post-puberty. Four patients (19 %) had more than one pituitary hormone deficiency, 3 after TSS and 1 post-RT. Five patients (24 %) had long-term psychiatric co-morbidities (cognitive dysfunction or mood disturbance). There were significant long-term improvements in growth, weight and bone density but not complete reversal to normal in all patients.ConclusionsThe long-term consequences of the diagnosis and treatment of CD in children is broadly similar to that seen in adults, with recurrence of CD after successful treatment uncommon but still seen. Pituitary hormone deficiencies occurred in the majority of patients after remission, and assessment and appropriate treatment of GHD is essential. However, while many parameters improve, some children may still have mild but persistent defects.

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“…Diagnosing CS in children is particularly challenging as its presenting features are variable and can be subtle, especially in young patients [4]. As a result, there is often a significant delay in diagnosing CS in the paediatric age range [5], which can result in significant morbidity, mortality and a reduction in quality of life [6,7,8,9]. Therefore, it is important to have a readily available and robust investigation to accurately identify cases of CS amongst children with unexplained weight gain.…”

Section: Introductionmentioning

confidence: 99%

Investigation for Paediatric Cushing's Syndrome Using Twenty-Four-Hour Urinary Free Cortisol Determination

Shapiro

Elahi²,

Riddoch³

et al. 2016

Horm Res Paediatr

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Objective: Paediatric Cushing's syndrome (CS) remains a challenge to diagnose and exclude. We assessed the accuracy of 24-hour urinary free cortisol (UFC) determination in children referred for suspected CS. Design: We conducted a retrospective study of paediatric patients referred to our centre with suspected CS between 1982 and 2014. Patients: Of 66 subjects (mean age 12.9 years; range 4.4-16.9), there were 47 cases of CS (29 males), which included Cushing's disease (CD; 39 patients, 25 males), primary pigmented nodular adrenocortical disease (8 patients, 4 males) and 19 ‘controls' (6 males) in whom the diagnosis of CS was excluded. Measurements: The subjects had between one and five 24-hour UFC collections analysed by radioimmunoassay, chemiluminescent immunoassay or liquid chromatography-mass spectrometry. The data were normalised, corrected for body surface area (m²) and assessed using receiver operating characteristic analysis and an independent two-tailed t test. Results: The diagnostic accuracy of 24-hour UFC for CS was excellent (area under the curve 0.98, 95% CI 0.946-1.00, sensitivity 89%, specificity 100%). Conclusions: Twenty-four-hour UFC is a reliable and practical investigation with high diagnostic accuracy for paediatric CS. However, further investigations may be required if the UFC is normal but there is a high diagnostic suspicion of CS.

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Cushing Disease in Children and Adolescents: Twenty Years’ Experience in A Tertiary Care Center in India

Cited by 28 publications

References 34 publications

The effects of sampling lateralization on bilateral inferior petrosal sinus sampling and desmopressin stimulation test for pediatric Cushing's disease

The effects of sampling lateralization on bilateral inferior petrosal sinus sampling and desmopressin stimulation test for pediatric Cushing's disease

Long-term outcomes of children treated for Cushing’s disease: a single center experience

Investigation for Paediatric Cushing's Syndrome Using Twenty-Four-Hour Urinary Free Cortisol Determination

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