Cushing disease in pediatrics: an update
Cushing disease (CD) is the main cause of endogenous Cushing syndrome (CS) and is produced by an adrenocorticotropic hormone (ACTH)-producing pituitary adenoma. Its relevance in pediatrics is due to the retardation of both growth and developmental processes because of hypercortisolism. In childhood,...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
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Korean Society of Pediatric Endocrinology
2023-06-01
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| Series: | Annals of Pediatric Endocrinology & Metabolism |
| Subjects: | |
| Online Access: | http://e-apem.org/upload/pdf/apem-2346074-037.pdf |
| _version_ | 1797792671443451904 |
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| author | Marcio José Concepción-Zavaleta Cristian David Armas Juan Eduardo Quiroz-Aldave Eilhart Jorge García-Villasante Ana Cecilia Gariza-Solano María del Carmen Durand-Vásquez Luis Alberto Concepción-Urteaga Francisca Elena Zavaleta-Gutiérrez |
| author_facet | Marcio José Concepción-Zavaleta Cristian David Armas Juan Eduardo Quiroz-Aldave Eilhart Jorge García-Villasante Ana Cecilia Gariza-Solano María del Carmen Durand-Vásquez Luis Alberto Concepción-Urteaga Francisca Elena Zavaleta-Gutiérrez |
| author_sort | Marcio José Concepción-Zavaleta |
| collection | DOAJ |
| description | Cushing disease (CD) is the main cause of endogenous Cushing syndrome (CS) and is produced by an adrenocorticotropic hormone (ACTH)-producing pituitary adenoma. Its relevance in pediatrics is due to the retardation of both growth and developmental processes because of hypercortisolism. In childhood, the main features of CS are facial changes, rapid or exaggerated weight gain, hirsutism, virilization, and acne. Endogenous hypercortisolism should be established after exogenous CS has been ruled out based on 24-hour urinary free cortisol, midnight serum or salivary cortisol, and dexamethasone suppression test; after that, ACTH dependence should be established. The diagnosis should be confirmed by pathology. The goal of treatment is to normalize cortisol level and reverse the signs and symptoms. Treatment options include surgery, medication, radiotherapy, or combined therapy. CD represents a challenge for physicians owing to its multiple associated conditions involving growth and pubertal development; thus, it is important to achieve an early diagnosis and treatment in order to control hypercortisolism and improve the prognosis. Its rarity in pediatric patients has led physicians to have limited experience in its management. The objective of this narrative review is to summarize the current knowledge about the pathophysiology, diagnosis, and treatment of CD in the pediatric population. |
| first_indexed | 2024-03-13T02:37:23Z |
| format | Article |
| id | doaj.art-6eb6a53a7172431aa37c3bf351ff5485 |
| institution | Directory Open Access Journal |
| issn | 2287-1012 2287-1292 |
| language | English |
| last_indexed | 2024-03-13T02:37:23Z |
| publishDate | 2023-06-01 |
| publisher | Korean Society of Pediatric Endocrinology |
| record_format | Article |
| series | Annals of Pediatric Endocrinology & Metabolism |
| spelling | doaj.art-6eb6a53a7172431aa37c3bf351ff54852023-06-29T00:12:10ZengKorean Society of Pediatric EndocrinologyAnnals of Pediatric Endocrinology & Metabolism2287-10122287-12922023-06-01282879710.6065/apem.2346074.037988Cushing disease in pediatrics: an updateMarcio José Concepción-Zavaleta0Cristian David Armas1Juan Eduardo Quiroz-Aldave2Eilhart Jorge García-Villasante3Ana Cecilia Gariza-Solano4María del Carmen Durand-Vásquez5Luis Alberto Concepción-Urteaga6Francisca Elena Zavaleta-Gutiérrez7 Universidad Cientifica del Sur, Lima, Perú School of Medicine, National University of Trujillo, Trujillo, Perú Division of Medicine, Hospital de Apoyo Chepén, Chepén, Perú Division of Endocrinology, Clínica Javier Prado, Lima, Perú Division of Pediatric Endocrinology, Hospital Nacional Hipólito Unanue, Lima, Perú Division of Family Medicine, Hospital de Apoyo Chepén, Chepén, Perú School of Medicine, National University of Trujillo, Trujillo, Perú Division of Pediatrics, Hospital Belén de Trujillo, Trujillo, PerúCushing disease (CD) is the main cause of endogenous Cushing syndrome (CS) and is produced by an adrenocorticotropic hormone (ACTH)-producing pituitary adenoma. Its relevance in pediatrics is due to the retardation of both growth and developmental processes because of hypercortisolism. In childhood, the main features of CS are facial changes, rapid or exaggerated weight gain, hirsutism, virilization, and acne. Endogenous hypercortisolism should be established after exogenous CS has been ruled out based on 24-hour urinary free cortisol, midnight serum or salivary cortisol, and dexamethasone suppression test; after that, ACTH dependence should be established. The diagnosis should be confirmed by pathology. The goal of treatment is to normalize cortisol level and reverse the signs and symptoms. Treatment options include surgery, medication, radiotherapy, or combined therapy. CD represents a challenge for physicians owing to its multiple associated conditions involving growth and pubertal development; thus, it is important to achieve an early diagnosis and treatment in order to control hypercortisolism and improve the prognosis. Its rarity in pediatric patients has led physicians to have limited experience in its management. The objective of this narrative review is to summarize the current knowledge about the pathophysiology, diagnosis, and treatment of CD in the pediatric population.http://e-apem.org/upload/pdf/apem-2346074-037.pdfcushing diseasecushing syndromeneuroendocrinologydiagnosistreatment |
| spellingShingle | Marcio José Concepción-Zavaleta Cristian David Armas Juan Eduardo Quiroz-Aldave Eilhart Jorge García-Villasante Ana Cecilia Gariza-Solano María del Carmen Durand-Vásquez Luis Alberto Concepción-Urteaga Francisca Elena Zavaleta-Gutiérrez Cushing disease in pediatrics: an update Annals of Pediatric Endocrinology & Metabolism cushing disease cushing syndrome neuroendocrinology diagnosis treatment |
| title | Cushing disease in pediatrics: an update |
| title_full | Cushing disease in pediatrics: an update |
| title_fullStr | Cushing disease in pediatrics: an update |
| title_full_unstemmed | Cushing disease in pediatrics: an update |
| title_short | Cushing disease in pediatrics: an update |
| title_sort | cushing disease in pediatrics an update |
| topic | cushing disease cushing syndrome neuroendocrinology diagnosis treatment |
| url | http://e-apem.org/upload/pdf/apem-2346074-037.pdf |
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