The problem of acromegalia in Ukraine. Creation of the All-Ukrainian National Register of Acromegaly Patients and analysis of previous data

Abstract

Acromegaly is a rare disease with a latent origin, caused by hypersecretion of somatotropic hormone, usually due to pituitary adenoma. The aim is to study the initial data of the All-Ukrainian National Register of Acromegaly Patients (hereinafter — the Register), accumulated during the first year of its operation, and to
create recommendations for the management of patients with uncontrolled disease. Material and methods. The analysis of epidemiological, biochemical and clinical characteristics of patients with acromegaly, assessment of patients’ coverage by different methods of treatment (neurosurgical, medical and/or radiation), detection of comorbid diseases, assessment of quality of care and compliance of Ukrainian medical realities with international standards of patient management. The diagnosis was made by performing a glucose tolerance test and determining insulin-like growth factor. Results. From 2020 to the present, data is being collected to create the Register, which is located on a single local server and computer facilities of the public organization «Ukrainian Association of Clinical Endocrinologists». As of February 1, 2022, the Register contains data for 452 patients with acromegaly, although the estimated number of patients with acromegaly for Ukraine according to the probable population prevalence should be 1158-5661 patients. The ratio of women to men was 3.1:1. The average age of diagnosis is 43 years (for men — 40 years, for women — 44 years). According to the results of magnetic resonance imaging, 357 people (79%) had macroadenomas, 77 people (17%) had microadenomas and in 18 people (4%) the size of the tumor was uncertain. The main symptoms of acromegaly were acromegaloid facial features (96.9%), arthralgia (73.9%), headache (71.5%), fatigue (50.9%), hyperhidrosis (48.5%), hypertension 44.0%), muscle weakness (42.0%), menstrual disorders (27.0%), sleep apnea syndrome (23.9%), decreased visual acuity (23.5%), depression (23.0%), bone pain (19.0%), dizziness (15.0%), obesity (12.0%), decreased libido (10.6%), edema (6.0%), galactorrhea in women (4.9%) and infertility (3.0%). 92.9% of patients in the Register had complications, including pathology of the thyroid gland (65.9%), hypertension (59.1%), acromegaly arthropathy (55.1%), reproductive disorders (49.6%), night apnea (33.0%), diabetes mellitus (31.0%), carpal tunnel syndrome (10.4%) and intestinal polyps (3.1%). Among the disorders of carbohydrate metabolism, diabetes mellitus was most often diagnosed (31.0%), less often — impaired fasting blood glucose disorders (17.5%) and impaired glucose tolerance (5.1%). The overall incidence of cardiovascular pathology was 80.3%, of which hypertension (59.1%), cardiomegaly (19.7%), heart valve disease (14.4%), cardiac arrhythmia (5.3%) and heart failure (2.4%) were registered. The diagnosis of malignancies was established in 17 patients, of which 6 patients had breast cancer, 6 had thyroid cancer, 2 had cervical cancer, 1 had skin cancer, 1 had meningioma and 1 had thymoma. In 67.9% of patients, the method of primary treatment was transnasal transsphenoidal surgery. The frequency of prescribing radiation therapy to patients was 12.0%, of which in 79.6% of cases radiation therapy was combined with surgical treatment and in 20.4% was performed as monotherapy. 265 patients (58.6%) received medical treatment, of which 127 were operated (47.9%). 81.2% of patients required medical treatment. 191 patients (72.1% of the total number receiving drug therapy) received dopamine agonists, 55 patients (20.7%) — somatostatin analogues and 19 people (7.2%) — pegvisomant. The proportion of patients who did not receive treatment for acromegaly is 17.3%. Among all patients, 6.2% had complete clinical and laboratory remission of the disease, 9.1% had incomplete remission; lack of biochemical control
of acromegaly was diagnosed in 84.7%. 13.5% of patients, despite inadequate biochemical control, reported an improvement in qual-ity of life and improved course of comorbid diseases. Conclusions. Data from the Register allow to state an extremely low percentage of clinical and laboratory remission in patients with acromegaly and, accordingly, to predict a significant trend towards further development of comorbid conditions, increased mortality, steady increase in the number of patients included in the Register, changes in the frequency of detected comorbid pathology and complications, as well as treatment indicators.