AVP-deficiency (central diabetes insipidus) registry in Russia: epidemiology and clinical characteristics

Alexander Lutsenko; Daria Sazonova; Zhukov Artem Yu.; Nino Katamadze; Ekaterina Pigarova; Larisa Dzeranova; Zhanna Belaya; Olga Vikulova; Natalia Mokrysheva

doi:10.1530/endoabs.99.EP324

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Endocrine Abstracts (2024) 99 EP324 | DOI: 10.1530/endoabs.99.EP324

ECE2024 Eposter Presentations Pituitary and Neuroendocrinology (214 abstracts)

AVP-deficiency (central diabetes insipidus) registry in Russia: epidemiology and clinical characteristics

Alexander Lutsenko ¹ , Daria Sazonova ¹ , Artem Yu. Zhukov ¹ , Nino Katamadze ¹ , Ekaterina Pigarova ¹ , Larisa Dzeranova ¹ , Zhanna Belaya ¹ , Olga Vikulova ¹ & Natalia Mokrysheva ¹

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¹Endocrinology Research Center, Moskva, Russian Federation

Background: Epidemiological data on AVP-deficiency is scarce, with non-univocal reported prevalence of 4 per 100.000 for all cases of central and nephrogenic DI without clear gender predominance. Disease registries are major tools for the assessment of epidemiological data, as well as real-life clinical practice, which could be quite valuable for AVP-d.

Aim.: To assess the epidemiological data on ADH-D in Russian Registry for Central Diabetes Insipidus (RCDI)

Materials and method: Database of the Russian Registry for Central Diabetes Insipidus, which includes data from 51 region. Date of analysis – 20-Nov-2023.

Results: Currently, our registry includes information on 3081 patients with AVP-d, from 51 regions of Russia: median age 32.4 years [18.0; 46.8], female-to-male ratio 1786(58%): 1295(42%), age at diagnosis: 32.4 years [18.0;46.8]. The most common causes of AVD-d were postoperative (16.4%), pathology of hypothalamic-pituitary development (9.7%), head trauma (6.6%) and hereditary forms of AVP-d (4.4%). Rare forms accounted for approximately 4% of the patients: neuroinfection, Langergans cell hystiocytosis, sarcoidosis, Sheehan syndrome, DIDMOAD syndrome and hypophysitis. Idiopathic ADH-D was registered in 41.5% of patients. Overall prevalence of AVP-d in Russia according to the National registry is 2.1 cases per 100 000 population. Among participating regions, the highest prevalence is seen in Moscow region (5.4 cases per 100 000 population), which could be attributed to a more thorough registration in tertiary care centers in Moscow. Data on current therapy was available in 2853 patients. Most of the patients receive oral desmopressin (n=1594, 55.9%), followed by sublingual (n=984, 34.5%) and intranasal (n=275, 9.6%) forms.

Conclusions: Russian Registry for AVP-deficiency provides valuable data on demography, etiology, treatment modalities. Continued improvment of the registry will provide more balanced epidemiological data, which is currently lacking worldwide.