Advances in the management of paediatric Cushing's disease

Martin O Savage1, Li F Chan, Farhad Afshar

  • 1Department of Endocrinology, Barts and the London School of Medicine and Dentistry, London, UK. m.o.savage@qmul.ac.uk

Hormone Research
|May 28, 2008
PubMed

Insights

Cushing's disease (CD) in children is rare and challenging to manage. Effective treatment requires specialized diagnostic protocols and long-term care focusing on growth and overall health.

Area of Science:

  • Pediatric Endocrinology
  • Endocrinology
  • Rare Diseases

Background:

  • Cushing's disease (CD) is uncommon in pediatric patients.
  • Management of pediatric CD poses therapeutic challenges due to limited specialist experience.
  • A structured diagnostic approach, similar to adults, is necessary before treatment.

Purpose of the Study:

  • To describe and critically appraise treatment strategies for pediatric Cushing's disease.
  • To highlight the diagnostic protocol for pediatric CD.
  • To discuss the long-term management challenges post-cure for pediatric CD patients.

Main Methods:

  • Review of diagnostic protocols for pediatric Cushing's disease.
  • Critical appraisal of current treatment strategies for pediatric CD.
  • Discussion of long-term follow-up considerations for growth, bone health, reproduction, and body composition.

Main Results:

  • Pediatric CD diagnosis follows adult models but requires specialized endocrine consultation.
  • Treatment strategies for pediatric CD are varied and require careful consideration.
  • Post-cure management is crucial for optimizing long-term health outcomes in pediatric patients.

Conclusions:

  • Pediatric Cushing's disease necessitates a tailored diagnostic and therapeutic approach.
  • Collaboration between pediatric and adult endocrinologists is beneficial.
  • Long-term multidisciplinary care is essential for addressing growth, bone health, reproduction, and body composition issues post-cure.

Related Concept Videos

Cushing Syndrome II: Pathophysiology01:19

Cushing Syndrome II: Pathophysiology

Cortisol production is normally governed by the hypothalamic–pituitary–adrenal (HPA) axis, which maintains hormonal balance through tightly regulated feedback mechanisms. Disruption of this regulatory system is central to the development of Cushing syndrome, whether the excess cortisol originates from external medications or internal pathology. Persistent cortisol elevation alters metabolism, immune function, and endocrine signaling, producing the characteristic clinical features of the...
Cushing Syndrome I: Introduction01:26

Cushing Syndrome I: Introduction

Cushing syndrome refers to the collection of clinical manifestations that arise when tissues are exposed to excessive amounts of cortisol or cortisol-like medications over an extended period. Cortisol, a glucocorticoid produced by the adrenal cortex, regulates metabolism, immune responses, and the body’s adaptation to stress. When its concentration remains chronically elevated, these physiological pathways become dysregulated, resulting in the characteristic features of the syndrome.Exogenous...
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Adrenal Gland Disorders01:27

Adrenal Gland Disorders

Adrenal gland disorders manifest when the production of adrenal hormones deviates from the norm, resulting in either excessive or insufficient concentrations.
Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...
Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses a challenge in...
Chronic Pancreatitis II: Collaborative Care01:29

Chronic Pancreatitis II: Collaborative Care

The management of chronic pancreatitis is multifaceted, involving a comprehensive approach that includes thorough assessment, diagnostic testing, and a variety of management strategies.
Assessment: