Investigation, management and therapeutic outcome in 12 cases of childhood and adolescent Cushing's syndrome

Weber, A.; Trainer, Peter; Grossman, Ashley; Afshar, F.; Medbak, S.; Perry, Leslie; Plowman, P. N.; Rees, Lesley; Besser, G. M.; Savage, Martin O.

doi:10.1111/j.1365-2265.1995.tb01888.x

Cited by 92 publications

(67 citation statements)

References 39 publications

(27 reference statements)

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“…However, there was a remarkable improvement of surgical cure (85.7%) in the latter ten years. In the literature, surgical cure rates range from 40% to 100% (mean: 70.5%), according to table 2 (4)(5)(6)12,13,(20)(21)(22)(23)(24)(25)(26)(27)(28). In the present study, plasma cortisol levels considered were < 1.8 μg/dL during the first days after surgery for cure definition -this is a very strict criterion.…”

Section: Discussionmentioning

confidence: 99%

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Surgical management of pediatric Cushing's disease: an analysis of 15 consecutive cases at a specialized neurosurgical center

Oliveira

Castro²,

Antonini³

et al. 2010

Arq Bras Endocrinol Metab

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Objective: The aim of this study was to review the results of surgery for pediatric patients with Cushing's disease who were less than 18 years old and underwent transsphenoidal surgery in a specialized center during a 25-year period. Subjects and methods: Retrospective study, in which the medical records, histology and pituitary imaging of 15 consecutive pediatric patients with Cushing's disease (mean age: 13 years) were evaluated by the same team of endocrinologists and a neurosurgeon from 1982 to 2006. Patients were considered cured when there was clinical adrenal insufficiency and serum cortisol levels were below 1.8 μg/dL or 50 nmol/L after one, two, three, or seven days following surgery; they therefore required cortisone replacement therapy. Follow-up was for a median time of 11.5 years (range: 2 to 25 years). Results: Clinical and biochemical cure was achieved in 9/15 patients (60%) exclusively after transsphenoidal surgery. Hypopituitarism was observed in four patients; growth hormone deficiency, in two; permanent diabetes insipidus, in one case. Conclusions: Cushing's disease is rare in children and adolescents. Transsphenoidal surgery is an effective and safe treatment in most of these patients. Plasma cortisol level < 1.8 μg/dL following surgery is the treatment goal and is a good predictive factor for long-term cure of Cushing's disease. Arq Bras Endocrinol Metab. 2010;54(1):17-23 Keywords Cushing's disease; pituitary tumor; transsphenoidal surgery; pediatric neurosurgery resumo Objetivo: O objetivo deste estudo foi avaliar os resultados cirúrgicos em pacientes pediátricos com doença de Cushing com idade inferior a 18 anos, submetidos à cirurgia transfenoidal num centro especializado, durante um período de acompanhamento de 25 anos. Sujeitos e métodos: Estudo retrospectivo dos prontuários médicos de 15 pacientes pediátricos com doença de Cushing (idade média de 13 anos), sendo avaliados aspectos clínicos, laboratoriais, histológicos e radiológicos. Todos os pacientes foram avaliados pela mesma equipe de endocrinologistas e operados por um mesmo neurocirurgião, entre 1982 e 2006. O tempo médio de seguimento foi 11,5 anos (2 a 25 anos). Os pacientes foram considerados curados quando houve insuficiência adrenal e níveis de cortisol plasmático inferiores a 1,8 μg/dL ou 50 nmol/L no pós-operatório um, dois, três ou sete dias após a cirurgia; estes pacientes necessitaram de reposição de corticosteroide. Resultados: Cura clínica e bioquímica foi alcançada em 9/15 pacientes (60%) após a cirurgia transfenoidal. Hipopituitarismo foi observado em quatro pacientes; déficit de hormônio de crescimento, em dois; diabetes insípido permanente, em um. Conclusões: A doença de Cushing é rara na infância e na adolescência. A cirurgia transfenoidal é um tratamento efetivo e seguro para a maioria dos pacientes. Uma concentração de cortisol plasmático < 1,8 μg/dL nos primeiros dias pós-cirurgia transfenoidal é o objetivo do tratamento e um fator preditivo tardio para a cura da doença de Cushing. Arq Bras Endocrinol...

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Section: Discussionmentioning

confidence: 99%

“…CD is rare in the pediatric age group (6,11,12). The disease remains a complex diagnostic challenge in childhood, because many of the diagnosis criteria and definition of cure after transsphenoidal surgery are based on results obtained in adults that have been extrapolated to the pediatric population (13).…”

Section: Discussionmentioning

confidence: 99%

Surgical management of pediatric Cushing's disease: an analysis of 15 consecutive cases at a specialized neurosurgical center

Oliveira

Castro²,

Antonini³

et al. 2010

Arq Bras Endocrinol Metab

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“…In childhood and adolescence, CD accounts for w75-80% of paediatric Cushing's syndrome cases, but is still rare (1). In children, the hypercortisolaemia of CD can cause severe morbidity, so early diagnosis, based on a series of standardised biochemical and radiological investigations, is essential (1)(2)(3)(4)(5).…”

Section: Introductionmentioning

confidence: 99%

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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“…Defined as hypercortisolaemia caused by an ACTH-secreting corticotroph adenoma, pediatric CD accounts for approximately 75-80% of all pediatric CS cases (7,11,12). This compares to 49-71% in adult CS (11,12). The median age of presentation is 14.1 yrs (141 cases taken from the literature), with the youngest reported case being 6.2 years (14).…”

Section: Acth-dependent Csmentioning

confidence: 99%

“…Careful auxological evaluation is the key to differentiating CS from simple obesity (10). Close collaboration with adult endocrinologists is recognised as an essential part of management of pediatric CS and adopted by many large centres (11,12).…”

mentioning

confidence: 99%

Pediatric Cushing's syndrome: clinical features, diagnosis, and treatment

Chan

Storr

Grossman

et al. 2007

Arq Bras Endocrinol Metab

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Cushing's syndrome (CS) results from prolonged exposure to supraphysiological levels of circulating glucocorticoids, endogenously or exogenously derived. Although rare in childhood, CS remains a difficult condition to diagnose and treat. A multidisciplinary approach and close collaboration with adult colleagues is adopted at most large centres that manage pediatric CS patients. Although pediatric protocols are derived from adult data, significant differences exist between adult and childhood CS. Furthermore, long term outcome parameters including final height, bone mineral density, reproductive function, body composition and psychological health pose challenges for pediatric care. This article will aim to provide an overall view of pediatric CS highlighting some of the differences between adult and pediatric CS. A síndrome de Cushing (SC) resulta da exposição prolongada a níveis suprafisiológicos de glicocorticóides circulantes, tanto endógenos como de seus derivados exógenos. Embora rara na infância, a SC permanece uma condição difícil de ser diagnosticada e tratada. Uma avaliação multidisciplinar e a colaboração próxima com colegas da área não-pediátrica são adotadas na maioria dos grandes centros que cuidam de pacientes pediátricos com SC. Embora os protocolos pediátricos sejam derivados de dados em adultos, existem diferenças significativas entre a SC no adulto e na infância. Além disso, parâmetros evolutivos finais, incluindo altura final, densidade mineral óssea, função reprodutiva, composição corporal e saúde psicológica trazem desafios no cuidado pediátrico. Este artigo procura oferecer uma visão geral da SC pediátrica, focalizando algumas das diferenças entre a SC adulta e a pediátrica.

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Investigation, management and therapeutic outcome in 12 cases of childhood and adolescent Cushing's syndrome

Cited by 92 publications

References 39 publications

Surgical management of pediatric Cushing's disease: an analysis of 15 consecutive cases at a specialized neurosurgical center

Surgical management of pediatric Cushing's disease: an analysis of 15 consecutive cases at a specialized neurosurgical center

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

Pediatric Cushing's syndrome: clinical features, diagnosis, and treatment

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