Hypercortisism in children

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

ICD-10 code

• E24 Syndrome Itenko-Cushing.
• E24.0 Disease of Itenko-Cushing's pituitary origin.
• E24.1 Nelson's Syndrome.
• E24.2 Medication syndrome of Itenko-Cushing.
• E24.3 Ectopic ACTH syndrome.
• E24.8 Other conditions characterized by Cushing's syndrome.
• E24.9 Itenko-Cushing syndrome, unspecified.

Causes of the hypercorticism in a child

The causes of hypercorticism are very diverse.

• Endogenous hypercorticosis can cause:
  • Itzenko-Cushing's disease is a neuroendocrinal disease of the hypothalamus and (or) the pituitary;
  • Isenko-Cushing syndrome - adrenal cortex disease (corticosteroid benign or malignant, nodular hyperplasia of the adrenal cortex);
  • ACTH-ectopic syndrome (tumors of the bronchi, pancreas, thymus, liver, ovary, secreting ACTH or corticotropin-releasing hormone);
  • hyperaldosteronism (Connes syndrome).
• Exogenous hypercortisy is due to the long-term administration of synthetic glucocorticosteroids (Isenko-Cushing's drug syndrome).
• There is also functional hypercorticism in pubertal juvenile dyspituitarism, hypothalamic syndrome, obesity, diabetes, liver diseases.

[1], [2], [3],

Symptoms of the hypercorticism in a child

Characterized by dysplastic obesity: "lunar" face, excess fat on the chest and abdomen with relatively thin limbs. Develop trophic skin changes (pink and purple striae on the hips, abdomen, chest, dryness, thinning). Progressing myopathy, hypertension, systemic osteoporosis, encephalopathy, steroid diabetes, secondary immunodeficiency, delayed sexual development. After the onset of menstruation, amenorrhea sometimes occurs in girls. Patients complain of weakness, headaches.

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

On examination, attention is drawn to a large face, purple cheeks, double chin, fat deposition over VII cervical vertebra. Because of the excessive production of androgen by the tumor, there are often signs of pathological masculinization in the form of hypertrichosis, acne, coarsening of the voice. Arterial hypertension is typical. Increased susceptibility to infections in some cases leads to sepsis.

[4], [5], [6], [7]

Diagnostics of the hypercorticism in a child

Cortisol levels in the blood are usually elevated, but are subject to wide fluctuations on different days. To confirm the diagnosis, repeated studies of cortisol in the blood are necessary. The daily rhythm of cortisol production is disturbed in most patients, the blood should be taken at 8 and at 20 hours, while the hormone levels may be the same (in healthy children over 3 years morning cortisol concentrations are several times higher than nighttime). Often noted polycythemia (increased concentration of hemoglobin and erythrocyte content), lymphopenia, eosinopenia. Diabetic type glucose tolerance may be impaired. Sometimes there is hypokalemia. Osteoporosis is expressed in the bodies of the vertebrae (on the roentgenograms of the spine).

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

[8], [9]

Treatment of the hypercorticism in a child

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