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

Li F Chan1, Helen L Storr, Ashley B Grossman

  • 1Department of Endocrinology, William Harvey Research Institute, St Bartholomew's and The Royal London School of Medicine and Dentistry. l.chan@qmul.ac.uk

Insights

Pediatric Cushing's syndrome (CS) is rare and challenging to diagnose and treat. This review highlights key differences between pediatric and adult CS, focusing on long-term outcomes.

Area of Science:

  • Pediatric endocrinology
  • Internal medicine

Background:

  • Cushing's syndrome (CS) involves prolonged exposure to excess glucocorticoids.
  • Pediatric CS is rare but presents diagnostic and therapeutic challenges.

Purpose of the Study:

  • To provide an overview of pediatric Cushing's syndrome.
  • To highlight differences between pediatric and adult CS.
  • To discuss challenges in managing pediatric CS long-term outcomes.

Main Methods:

  • Review of existing literature on pediatric Cushing's syndrome.
  • Comparison of pediatric and adult CS data.
  • Analysis of long-term outcome parameters in childhood CS.

Main Results:

  • Pediatric CS protocols are adapted from adult data, but significant differences exist.
  • Long-term outcomes (height, bone density, reproduction, body composition, psychological health) are key challenges.
  • Multidisciplinary care and collaboration are essential for managing pediatric CS.

Conclusions:

  • Pediatric CS requires specialized management distinct from adult cases.
  • Addressing long-term outcomes is crucial for improving quality of life in childhood CS survivors.
  • Further research comparing pediatric and adult CS is warranted.

Related Concept Videos

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...
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...
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...
COPD: Pathogenesis and Clinical Features01:20

COPD: Pathogenesis and Clinical Features

Chronic obstructive pulmonary disease (COPD) is a group of lung conditions that progressively worsen over time, including chronic bronchitis and emphysema. This cluster of diseases collectively leads to a gradual and irreversible decline in lung function over time.
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...
Attention-Deficit/Hyperactivity Disorder01:30

Attention-Deficit/Hyperactivity Disorder

Attention-deficit/hyperactivity disorder (ADHD) is a neurodevelopmental disorder characterized by persistent inattention, hyperactivity, and impulsivity. It affects approximately 5-8% of children globally, with around 60-70% of cases persisting into adulthood. ADHD has significant implications for educational attainment, social interactions, and occupational success.
Diagnostic Criteria and Symptoms
To diagnose ADHD, symptoms must manifest before age 12 and be evident across multiple settings.