porozendoОстеопороз и остеопатииOsteoporosis and Bone Diseases2072-26802311-0716Endocrinology Research Centre10.14341/osteo13210porozendo-13210Research ArticleОригинальное исследованиеOriginal studyОценка эффективности терапии бисфосфонатами взрослых пациентов с несовершенным остеогенезомEvaluation of the efficacy of bisphosphonate therapy in adult patients with osteogenesis imperfectahttps://orcid.org/0000-0003-1643-3719ВалееваД. И.ValeevaD. I.

Валеева Диана Ильдаровна, ассистент кафедры внутренних болезней и клинической психологии

 ул. Ленина, 3, Уфа, Респ. Башкортостан, 450008

 

Diana I. Valeeva 

450008, Russia, Republic of Bashkortostan, Ufa, Lenin St, 3

Diana2537@yandex.ruhttps://orcid.org/0000-0002-8643-850XХусаиноваР. И.KhusainovaR. I.

Хусаинова Рита Игоревна, д.б.н., профессор 

Уфа

Rita I. Khusainova

Ufa

ritakh@mail.ruhttps://orcid.org/0000-0002-0841-3024ТюринA. B.TiurinA. V.

Тюрин Антон Викторович, д.м.н., доцент 

Уфа

Anton V. Tyurin

Ufa

anton.bgmu@gmail.comБашкирский государственный медицинский университетРоссияBashkir State Medical UniversityRussian Federation202511012026283411Copyright © Валеева Д.И., Хусаинова Р.И., Тюрин A.B., 20262026Валеева Д.И., Хусаинова Р.И., Тюрин A.B.Valeeva D.I., Khusainova R.I., Tiurin A.V.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.osteo-endojournals.ru/jour/article/view/13210Обоснование

Обоснование: Несовершенный остеогенез (НО) — это редкое наследственное заболевание соединительной ткани, характеризующееся снижением плотности костной ткани и рецидивирующими низкотравматическими переломами. На сегодняшний день бисфосфонаты (БФ) при НО используются с целью увеличения плотности кости и снижения количества новых переломов, однако имеющиеся данные касаются преимущественно использования БФ у детей. При этом, благодаря раннему началу антирезорбтивной терапии, все большее количество пациентов с НО достигают совершеннолетия, что требует определения показаний и оценки эффективности терапии БФ у взрослых пациентов с НО.

Цель исследования

Цель исследования: Оценка эффективности терапии бисфосфонатами у взрослых пациентов с несовершенным остеогенезом.

Материалы и методы

Материалы и методы: Проведено продольное сравнительное исследование 45 пациентов с НО на базе клиники Башкирского государственного медицинского университета. 24 пациента однократно получили золедроновую кислоту 5мг/100 мл внутривенно капельно. Минеральная плотность костной ткани (МПК) определялась при помощи рентгеновской денситометрии. Уровень болевого синдрома оценивался при помощи визуально-аналоговой шкалы. Оценка дисплазии соединительной ткани (ДСТ) проводилась по модифицированной таблице Т.И. Кадуриной.

Результаты

Результаты: Статистически значимые различия наблюдались при сравнении ИМТ у пациентов с I и III (23,6±4,6; 28,5±5,9; p=0,0183), I и V (23,6±4,6; 30,8±8,2; p=0,014) типами НО. Статистически значимых различий уровней балльно-количественной оценки ДСТ, болевого синдрома по ВАШ и z-критерия между разными типами заболевания не наблюдалось. У пациентов, получивших БФ в КБГМУ наблюдалось статистически значимое уменьшение болевого синдрома (p<0,0001) и увеличение уровня z-критерия (p=0,045) через год после введения препарата, независимо от наличия лечения бисфосфонатами ранее. При проведении корреляционного анализа у пациентов с НО обнаружена умеренная обратная связь между МПК и количеством переломов (r=-0,488, p=0,001), умеренная прямая связь между степенью выраженности ДСТ и уровнем болевого синдрома (r=0,408, p=0,005). Количество переломов также коррелировало с ИМТ (r=0,337, p=0,024).

Заключение

Заключение: У взрослых пациентов с НО сохраняются низкие уровни показателей МПК и z-критерия, и присутствует умеренный постоянный болевой синдром в костях, не связанный с переломами. Интенсивность данных показателей не зависит от наличия предыдущей терапии бисфосфонатами. Лечение БФ является эффективным, так как наблюдалось статистически значимое увеличение МПК и z-критерия по результатам денситометрии, и статистически значимое уменьшение уровня болевого синдрома в костях. Основными показаниями для введения БФ являются: наличие переломов в течение года, низкие показатели денситометрии (z-критерий<-2,0), наличие умеренного постоянного болевого синдрома в костях (ВАШ>5 баллов).

Background

Background: Osteogenesis imperfecta (OI) is a rare, inherited connective tissue disorder characterized by decreased bone density and recurrent low-trauma fractures. Bisphosphonates (BF) are currently used to increase bone density and reduce the incidence of new fractures, but the available data mainly concern the use of BF in children. At the same time, due to the early initiation of antiresorptive therapy, an increasing number of patients with OI are reaching adulthood, which requires determining the indications and evaluating the effectiveness of BF therapy in adult patients with OI.

Aims

Aims: Evaluation of the efficacy of bisphosphonate therapy in adult patients with osteogenesis imperfecta.

Materials and methods

Materials and methods: A longitudinal comparative study of 45 patients with osteogenesis imperfecta was conducted at the Clinic of the Bashkir State Medical University (BSMU). 24 patients received a single dose of zoledronic acid 5 mg / 100 ml intravenously by drip. Bone mineral density (BMD) was determined using X-ray densitometry. The level of pain syndrome was assessed using a visual analogue scale. Connective tissue dysplasia (CTD) was assessed using a modified table by T.I. ­Kadurina.

Results

Results: Statistically significant differences were observed when comparing BMI in patients with OI types I and III (23.6±4.6; 28.5±5.9; p=0.0183), I and V (23.6±4.6; 30.8±8.2; p=0.014). There were no statistically significant differences in the levels of CTD scoring, pain syndrome according to VAS and z-score between different types of the disease. Patients who received BF at BGMU showed a statistically significant decrease in pain syndrome (p<0.0001) and an increase in the z-criterion level (p=0.045) one year after the drug administration, regardless of the presence of previous bisphosphonate treatment. When conducting a correlation analysis in patients with OI, a moderate inverse correlation was found between BMD and the number of fractures (r=-0.488, p=0.001). The number of fractures also correlated with BMI (r=0.337, p=0.024). The severity of phenotypic signs of connective tissue dysplasia had a direct moderate correlation with the level of pain syndrome (r=0.408, p=0.005).

Conclusions

Conclusions: In adult patients with OI, low levels of BMD and z-score are maintained, and persistent bone pain syndrome not associated with fractures is present. The intensity of these parameters does not depend on the presence of previous therapy with bisphosphonates. Treatment with drugs from this group is effective, since a statistically significant increase in BMD and z-score according to the results of densitometry, and a statistically significant decrease in the level of pain syndrome in the bones were observed. The main indications for BF administration are: the presence of fractures within a year, low densitometry values (z-score <-2.0), and the presence of severe, moderate, persistent bone pain (VAS > 5 points).

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The authors declare that there are no conflicts of interest present.