Клинический случай синдрома Блоха — Сульцбергера

Purpose.To present a clinical case of Bloch Sulzberger syndrome. Material and methods. The examinations were performed to diagnose the disease: а visual examination of the skin, cytological analysis of the gallbladder fluid, general and biochemical blood tests, genetic research. Results.During a visual examination of the skin, a differential diagnosis was made with infectious dermatitis, toxic-allergic dermatitis, epidermolysis bullosa and linear IgA-dependent dermatosis in children. Crucial in the diagnosis belonged to a genetic study, after which a deletion of exons 410 of the IKBKG gene was detected, which confirmed Bloch Sulzberger syndrome. Conclusion.Newborns with vesicle-bullous rashes entering the neonatal pathology department and observed by neonatologists require a thorough examination, a mandatory consultation of a dermatologist inorder to determine further management tactics.Цель.Представить клинический случай синдрома БлохаСульцбергера. Материал и методы.Для диагностики заболевания были проведены осмотр кожи, цитологический анализ пузырной жидкости, общий и биохимический анализы крови, генетическое исследование. Результаты.При осмотре кожи был проведен дифференциальный диагноз с дерматитами, буллезным эпидермолизом и линеарным IgA-зависимым дерматозом у детей. Решающее значение в постановке диагноза принадлежало генетическому исследованию, после проведения которого была выявлена делеция экзонов 410 гена IKBKG, что подтвердило синдром БлохаСульцбергера. Заключение.Новорожденные с везикуло-буллезными высыпаниями, поступающие в отделение патологии новорожденных и наблюдаемые неонатологами, требуют тщательного обследования, обязательной консультации дерматолога с целью определения дальнейшей тактики ведения

Pedagogical opportunities of student communicative culture development

The paper relevance is determined by the need to search for forms and means for a communication culture development among modern students. In the paper the concept "communicative culture" is clarified, the possibilities of the socio-cultural and educational environment are shown; the main characteristics and properties of communicative culture are presented (universality, standard in the social norms transfer, the "feedback" effect) in the paper. The specifics of the communication culture are considered, the content of the various levels (elementary, ideal, etc.) is disclosed; criteria for assessing the dynamics of communicative culture development (stability, creativity, etc.) are defined. © 2017

Pedagogical opportunities of student communicative culture development

The paper relevance is determined by the need to search for forms and means for a communication culture development among modern students. In the paper the concept "communicative culture" is clarified, the possibilities of the socio-cultural and educational environment are shown; the main characteristics and properties of communicative culture are presented (universality, standard in the social norms transfer, the "feedback" effect) in the paper. The specifics of the communication culture are considered, the content of the various levels (elementary, ideal, etc.) is disclosed; criteria for assessing the dynamics of communicative culture development (stability, creativity, etc.) are defined. © 2017

Russian federal clinical guidelines on the diagnostics, treatment, and prevention of osteoporosis

Screening using the Fracture Risk Assessment Tool (FRAX) is recommended in all postmenopausal woman and men over 50 (A1) in order to identify individuals with high probability of fractures. It is recommended to diagnose osteoporosis and start treatment in patients with fragility fracture of large bones of the skeleton and/or high individual probability of major fragility fractures (FRAX) and/or detected decrease in bone mineral density (BMD) up to -2.5 T-score as assessed by DXA in the femoral neck and/or lumbar vertebrae (A1). Patients with back pain, lifetime height loss of 4 cm or height loss of 2 cm since a previous medical examination, those who receive glucocorticoids, patients with long lasting decompensated type 2 diabetes mellitus, or those receiving insulin therapy, as well as patients who were previously diagnosed with fragility fractures at the other sites are advised to underwent standard lateral X-ray imaging of the spine (Th4-L5) in order to verify the presence of compression vertebral fractures (B1). Dual-energy X-ray absorptiometry (DXA) is recommended for individuals whose 10-year probability of major osteoporotic fracture (FRAX) falls within the medium risk group (B1). It is recommended to include the trabecular bone score (TBS) the FRAX algorithm in order to improve the sensitivity of this method (B1). Laboratory testing is recommended for the differential diagnosis with other causes of increased skeletal fragility in all patients with newly diagnosed osteoporosis and when previously prescribed antiosteoporostic treatment was ineffective (B1). Bisphosphonates (BPs), antibodies to receptor activator of nuclear factor kappa-beta ligand (RANKL) (denosumab), or parathyroid hormone analogue (teriparatide) are equally recommended to prevent fragility fractures and increase BMD in patients with osteoporosis (A1). Denosumab is also recommended to prevent BMD loss and fractures in females receiving aromatase inhibitors therapy for breast cancer and males with prostate cancer receiving hormone-deprivation therapy and having no bone metastases (A1). Since teriparatide has the anabolic effect, it is recommended as the first line treatment in patients with severe osteoporosis having history of vertebral fractures, in the individuals with very high risk of fragility fractures, or in the cases when antiresorptive treatment was ineffective (B1). All medications for treatment of osteoporosis are recommended in combination with calcium and vitamin D supplements (A1). Copyright © 2017 by the MediaSphere

Russian federal clinical guidelines on the diagnostics, treatment, and prevention of osteoporosis [Федеральные клинические рекомендации по диагностике, лечению и профилактике остеопороза]

Screening using the Fracture Risk Assessment Tool (FRAX) is recommended in all postmenopausal woman and mеn over 50 (A1) in order to identify individuals with high probability of fractures. It is recommended to diagnose osteoporosis and start treatment in patients with fragility fracture of large bones of the skeleton and/or high individual probability of major fragility fractures (FRAX) and/or detected decrease in bone mineral density (BMD) up to -2.5 T-score as assessed by DXA in the femoral neck and/or lumbar vertebrae (A1). Patients with back pain, lifetime height loss of 4 cm or height loss of 2 cm since a previous medical examination, those who receive glucocorticoids, patients with long lasting decompensated type 2 diabetes mellitus, or those receiving insulin therapy, as well as patients who were previously diagnosed with fragility fractures at the other sites are advised to underwent standard lateral X-ray imaging of the spine (Th4-L5) in order to verify the presence of compression vertebral fractures (B1). Dual-energy X-ray absorptiometry (DXA) is recommended for individuals whose 10-year probability of major osteoporotic fracture (FRAX) falls within the medium risk group (B1). It is recommended to include the trabecular bone score (TBS) the FRAX algorithm in order to improve the sensitivity of this method (B1). Laboratory testing is recommended for the differential diagnosis with other causes of increased skeletal fragility in all patients with newly diagnosed osteoporosis and when previously prescribed antiosteoporostic treatment was ineffective (B1). Bisphosphonates (BPs), antibodies to receptor activator of nuclear factor kappa-beta ligand (RANKL) (denosumab), or parathyroid hormone analogue (teriparatide) are equally recommended to prevent fragility fractures and increase BMD in patients with osteoporosis (A1). Denosumab is also recommended to prevent BMD loss and fractures in females receiving aromatase inhibitors therapy for breast cancer and males with prostate cancer receiving hormone-deprivation therapy and having no bone metastases (A1). Since teriparatide has the anabolic effect, it is recommended as the first line treatment in patients with severe osteoporosis having history of vertebral fractures, in the individuals with very high risk of fragility fractures, or in the cases when antiresorptive treatment was ineffective (B1). All medications for treatment of osteoporosis are recommended in combination with calcium and vitamin D supplements (A1). Copyright © 2017 by the MediaSpher

Федеральные клинические рекомендации по диагностике, лечению и профилактике остеопороза

Screening using the Fracture Risk Assessment Tool (FRAX) is recommended in all postmenopausal woman and men over 50 (A1) in order to identify individuals with high probability of fractures. It is recommended to diagnose osteoporosis and start treatment in patients with fragility fracture of large bones of the skeleton and/or high individual probability of major fragility fractures (FRAX) and/or detected decrease in bone mineral density (BMD) up to -2.5 T-score as assessed by DXA in the femoral neck and/or lumbar vertebrae (A1). Patients with back pain, lifetime height loss of 4 cm or height loss of 2 cm since a previous medical examination, those who receive glucocorticoids, patients with long lasting decompensated type 2 diabetes mellitus, or those receiving insulin therapy, as well as patients who were previously diagnosed with fragility fractures at the other sites are advised to underwent standard lateral X-ray imaging of the spine (Th4-L5) in order to verify the presence of compression vertebral fractures (B1). Dual-energy X-ray absorptiometry (DXA) is recommended for individuals whose 10-year probability of major osteoporotic fracture (FRAX) falls within the medium risk group (B1). It is recommended to include the trabecular bone score (TBS) the FRAX algorithm in order to improve the sensitivity of this method (B1). Laboratory testing is recommended for the differential diagnosis with other causes of increased skeletal fragility in all patients with newly diagnosed osteoporosis and when previously prescribed antiosteoporostic treatment was ineffective (B1). Bisphosphonates (BPs), antibodies to receptor activator of nuclear factor kappa-beta ligand (RANKL) (denosumab), or parathyroid hormone analogue (teriparatide) are equally recommended to prevent fragility fractures and increase BMD in patients with osteoporosis (A1). Denosumab is also recommended to prevent BMD loss and fractures in females receiving aromatase inhibitors therapy for breast cancer and males with prostate cancer receiving hormone-deprivation therapy and having no bone metastases (A1). Since teriparatide has the anabolic effect, it is recommended as the first line treatment in patients with severe osteoporosis having history of vertebral fractures, in the individuals with very high risk of fragility fractures, or in the cases when antiresorptive treatment was ineffective (B1). All medications for treatment of osteoporosis are recommended in combination with calcium and vitamin D supplements (A1). Copyright © 2017 by the MediaSphere.Скрининг для выявления групп с высокой вероятностью переломов рекомендован с использованием алгоритма FRAX среди всех женщин в постменопаузе и мужчин старше 50 лет (A1). Рекомендуется устанавливать диагноз и назначать терапию остеопороза лицам с низкотравматическим переломом крупных костей скелета, и/или с высокой индивидуальной вероятностью основных низкотравматических переломов (FRAX), и/или при выявленном снижении МПК до -2,5 SD по Т-критерию в шейке бедра и/или поясничных позвонках (А1). Пациентам с болевым синдромом в спине, снижением роста на 4 см в течение жизни или на 2 см при регулярном медицинском контроле, принимающим глюкокортикоиды, больным с длительно некомпенсированным сахарным диабетом 2-го типа или на инсулинотерапии, а также c диагностированными переломами другой локализации рекомендовано проведение стандартного рентгенологического исследования позвоночника (Th4-L5) в боковой проекции для выявления компрессионных переломов тел позвонков (B1). Проведение двухэнергетической рентгеновской денситометрии (DXA) рекомендовано лицам с индивидуальной 10-летней вероятностью переломов (FRAX) в интервале между низкой и высокой вероятностью переломов (B1). Результат трабекулярного костного индекса (ТКИ), полученный в ходе стандартной рентгеновской денситометрии, рекомендуется включать в алгоритм FRAX с целью повышения чувствительности метода (B1). Лабораторные методы исследований рекомендуется проводить всем пациентам с впервые установленным диагнозом остеопороза, а также при неэффективности ранее назначенной терапии с целью дифференциальной диагностики с другими причинами повышенной хрупкости скелета (B1). Для предупреждения низкотравматических переломов и повышения МПК у пациентов с остеопорозом в равной степени рекомендованы бисфосфонаты (БФ), или антитела к лиганду рецептора-активатора ядерного фактора каппа-бета (деносумаб), или аналоги паратгормона (терипаратид) (A1). Деносумаб также рекомендован для предупреждения потери костной массы и переломов при отсутствии костных метастазов у женщин, получающих терапию ингибиторами ароматазы по поводу рака молочной железы, и у мужчин с раком предстательной железы, получающих гормон-депривационную терапию (A1). Ввиду анаболического эффекта терипаратида рекомендуется его использовать в качестве первой линии терапии у пациентов с тяжелым остеопорозом, с уже имеющимися переломами тел позвонков в анамнезе, у лиц с крайне высоким риском низкотравматических переломов, пациентов с неэффективностью предшествующей терапии, а также непереносимостью альтернативного лечения (B1). Все препараты для лечения остеопороза рекомендуется назначать в сочетании с препаратами кальция и витамина D (A1)