Hypercorticism in children

Hypercorticism in children is a syndrome caused by a constantly high level of glucocorticoids in the blood as a result of hyperfunction of the adrenal cortex.

ICD-10 code

• E24 Itsenko-Cushing syndrome.
• E24.0 Itsenko-Cushing's disease of pituitary origin.
• E24.1 Nelson syndrome.
• E24.2 Drug-induced Itsenko-Cushing syndrome.
• E24.3 Ectopic ACTH syndrome.
• E24.8 Other conditions characterized by Cushingoid syndrome.
• E24.9 Itsenko-Cushing syndrome, unspecified.

Causes of hypercorticism in a child

The causes of hypercorticism are very diverse.

• Endogenous hypercorticism can be caused by:
  • Itsenko-Cushing's disease is a neuroendocrine disease of the hypothalamus and/or pituitary gland;
  • Itsenko-Cushing syndrome - a disease of the adrenal cortex (benign or malignant corticosteroma, nodular hyperplasia of the adrenal cortex);
  • ACTH-ectopic syndrome (tumors of the bronchi, pancreas, thymus, liver, ovaries, secreting ACTH or corticotropin-releasing hormone);
  • hyperaldosteronism (Conn's syndrome).
• Exogenous hypercorticism is caused by long-term administration of synthetic glucocorticosteroids (drug-induced Itsenko-Cushing syndrome).
• Functional hypercorticism is also distinguished in pubertal-juvenile dyspituitarism, hypothalamic syndrome, obesity, diabetes mellitus, and liver diseases.

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Symptoms of hypercorticism in a child

Dysplastic obesity is typical: a "moon-shaped" face, excess fat on the chest and abdomen with relatively thin limbs. Trophic changes in the skin develop (pink and purple striae on the thighs, abdomen, chest, dryness, thinning). Myopathy, hypertension, systemic osteoporosis, encephalopathy, steroid diabetes, secondary immunodeficiency, delayed sexual development progress. In girls, amenorrhea sometimes occurs after the onset of menstruation. Patients complain of weakness and headaches.

In addition to obesity, growth retardation is often the first manifestation of the disease. Gradually developing obesity and slowing or stopping growth may not initially be accompanied by any other symptoms.

During examination, attention is drawn to a large face, purple cheeks, double chin, fat deposits above the 7th cervical vertebra. Due to excessive production of androgens by the tumor, signs of pathological masculinization often appear in the form of hypertrichosis, acne, and coarsening of the voice. Arterial hypertension is characteristic. Increased susceptibility to infections in some cases leads to sepsis.

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Diagnostics of hypercorticism in a child

Blood cortisol levels are usually elevated but are subject to wide fluctuations from day to day. Repeated blood cortisol tests are necessary to confirm the diagnosis. Most patients have a disrupted circadian rhythm of cortisol production; blood should be taken at 8 a.m. and 8 p.m., while the hormone levels may be the same (in healthy children over 3 years of age, morning cortisol concentrations are several times higher than evening ones). Polycythemia (increased hemoglobin concentration and red blood cell count), lymphopenia, and eosinopenia are often observed. Glucose tolerance may be impaired in the diabetic type. Hypokalemia sometimes occurs. Osteoporosis is expressed in the vertebral bodies (on X-rays of the spine).

Visualization methods (CT, MRI, ultrasound) are used to establish a nosological diagnosis. Topical diagnostics (micro- or macroadenoma of the pituitary gland, tumor of the adrenal gland and other organs) are performed in patients with previously clinically and laboratory proven hypercorticism syndrome.

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Treatment of hypercorticism in a child

Includes surgical, radiation and drug (steroidogenesis blockers - mitotane, dopamine agonists) methods, determined by the severity of the disease and the size of the lesion.