Maladie de Cushing de l'enfant et de l'adolescent. Résultats thérapeutiques

Mathivon, L.; Jc, Carel; Coutant, Régis; Derome, P; Adamsbaum, C.; Pf, Bougnères; Chaussain, J L

doi:10.1016/s0929-693x(97)87570-7

Cited by 5 publications

(4 citation statements)

References 34 publications

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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: 62%

“…Nowadays, it still has a role, but should be reserved for patients in whom surgery and radiotherapy fail to stop the secretion of ACTH from the pituitary adenoma (25). Although adrenalectomy is the only treatment that offers an immediate control of hypercortisolism with 100% certainty, it is necessary to consider its side effects, including potential adrenal insufficiency crisis.…”

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: 62%

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

View full text Add to dashboard Cite

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“…Ceci peut être expliqué par l’âge un peu avancé de nos patients, les patients inclus dans l’étude sont majoritairement de jeunes adultes, avec un âge moyen de 19 ans, ayant déjà terminé leur croissance. La déminéralisation osseuse est fréquente: elle est notée lors du diagnostic chez 75% des patients [ 2 – 5 ]. La démarche diagnostique dans le syndrome de Cushing chez l'adolescent est comparable à l'adulte [ 6 ].…”

Section: Discussionunclassified

“…L'incidence annuelle de la maladie de Cushing, qui est l’étiologie la plus fréquente chez l'adolescent, est de l'ordre de 1,2 à 1,7 nouveaux cas pour 1000 000 [ 1 ]. Le syndrome de Cushing chez l'enfant et l'adolescent diffère de la pathologie adulte par le mode de présentation et la prise en charge thérapeutique [ 2 , 3 ]. Les moyens diagnostiques eux, sont relativement proches.…”

Section: Introductionunclassified

Le syndrome de cushing chez l’adolescent: à propos de 18 patients

Haraj¹,

Aziz²,

Chadli³

2015

Pan Afr Med J

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Le syndrome de Cushing est une pathologie rare mais grave chez l'enfant et l'adolescent. Elle diffère de la pathologie adulte par le mode de présentation et la prise en charge. Il s'agit d'une étude rétrospective des dossiers de patients suivis pour syndrome de Cushing au service d'endocrinologie de Casablanca entre 2002 - 2015, incluant les patients âgés au moment du diagnostic de moins de 22 ans, et ayant un suivi d'au moins 1 an. Au total 18 dossiers ont été inclus. L’âge moyen est de 19,55 ans, avec une prédominance féminine. La durée d’évolution moyenne est de 4,05 ans. Le tableau clinique est fait souvent d'une cassure de poids, une obésité ou une séborrhée et acné. La démarche diagnostique est comparable à celle de l'adulte. Sur le plan étiologique on retrouve une prédominance de la maladie de Cushing (15 patients). Sur le plan thérapeutique, 14 patients ont bénéficié d'une chirurgie hypophysaire, avec complément par radiothérapie chez 3 patients devant l’échec de la chirurgie, Une ablation d'une tumeur surrénalienne chez une patiente et une surrénalectomie bilatérale chez trois patients. L’évolution a été marquée par une guérison chez 9 patients et le décès chez 4 (suite à un syndrome de Nelson, infection sévère, choc hémorragique, corticosurrénalome). Les résultats de cette étude soulignent la gravité de cette maladie, ce qui nécessite d'organiser le suivi, en élaborant des programmes spécifiques de suivi médical et de prise en charge psychologique.

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Cushing’s Syndrome in Children and Adolescents

Mastorakos¹,

Kanaka‐Gantenbein²

2005

Adrenal Glands

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Maladie de Cushing de l'enfant et de l'adolescent. Résultats thérapeutiques

Cited by 5 publications

References 34 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

Le syndrome de cushing chez l’adolescent: à propos de 18 patients

Cushing’s Syndrome in Children and Adolescents

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