Auswahl der wissenschaftlichen Literatur zum Thema „616.804 7572“

Single crystals of Ni-25.6 at.% Pt and Ni-87.8 at.% Pt were investigated by diffuse x-ray scattering for states of thermal equilibrium (923 K and 603 K, respectively). The separated short-range order scattering showed local maxima at 100 positions. Effective pair interaction parameters, as determined by the inverse Monte Carlo method, show a strong composition dependence of the nearest-neighbor interaction parameter. First-principles calculations are consistent with this finding and reveal a large contribution due to lattice strain. Based on values of the ordering energy, NiPt3with L12structure was considered as a plausible new intermetallic phase, with Monte Carlo simulations giving an order-disorder transition temperature of 650 K. A single crystal of Ni-75.2 at.% Pt, quenched from 1073 K and aged at 613 K, showed L12-type ordering, reaching a long-range order parameter of 0.50(4) after 800 h.

ImportanceImmune-related adverse events (irAEs) secondary to immune checkpoint inhibitor (ICI) therapy reportedly improve overall survival (OS) in patients with non–small cell lung cancer (NSCLC). However, studies have been small and the association between irAE severity and OS remains poorly defined.ObjectiveTo examine the association between irAEs and their severity with OS in patients with locally advanced or metastatic NSCLC receiving ICIs.Design, Setting, and ParticipantsThis retrospective observational cohort study included patients with NSCLC receiving ICIs between March 1, 2014, and November 30, 2021, with follow-up until March 31, 2023. Data analysis was completed April 26, 2023. The Alberta Immunotherapy Database, a provincial, multicenter cohort, was used to capture data from patients receiving ICIs in Alberta, Canada. Participants included 803 patients 18 years or older who received at least 1 cycle of ICI (alone or with chemotherapy), agnostic to treatment line.ExposureDeveloping an irAE mandating delay or discontinuation of ICI therapy and/or systematic corticosteroids for management of toxic effects (hereinafter referred to as clinically meaningful irAEs).Main Outcomes and MeasuresThe primary outcome was association between irAEs and OS according to Kaplan-Meier analysis. Clinically meaningful irAEs were identified. Patients with poor prognosis (survival <3 months) who may have died prior to irAE development were excluded from OS analysis, mitigating immortal time bias. Adjusted Cox proportional hazards regression analyses ascertained variables associated with OS.ResultsAmong the 803 patients included in the analysis, the median age of patients with irAEs was 69.7 (IQR, 63.1-75.2) years and the median age of those without irAEs was 67.5 (IQR, 60.4-73.3) years, with comparable sex distribution (139 of 295 men [47.1%] and 156 of 295 women [52.9%] with irAEs vs 254 of 505 men [50.3%] and 251 of 505 women [49.7%] without irAEs). Mitigating immortal time bias (n = 611), irAEs were associated with OS (median OS with irAEs, 23.7 [95% CI, 19.3-29.1] months; median OS without irAEs, 9.8 [95% CI, 8.7-11.4] months; P < .001). No OS difference was associated with treatment in hospital vs as outpatients for an irAE (median OS, 20.8 [95% CI, 11.7-30.6] vs 25.6 [95% CI, 20.1-29.8] months; P = .33). Developing irAEs remained associated with OS in the total cohort after Cox proportional hazards regression with known prognostic characteristics (hazard ratio, 0.53 [95% CI, 0.40-0.70]; P < .001).Conclusions and RelevanceIn this cohort study of 803 patients with locally advanced or metastatic NSCLC receiving ICIs, developing a clinically meaningful irAE was associated with improved OS. This association was not compromised by hospitalization for severe toxic effects. Whether and how ICI therapy resumption after an irAE is associated with OS warrants further study.

Mục tiêu: Nghiên cứu hình ảnh cắt lớp vi tính 256 dãy (CLVT-256) về đặc điểm vôi hóa và hẹp mạch vành trên các bệnh nhân (BN) tăng huyết áp (THA). Đối tượng và phương pháp: Nghiên cứu mô tả cắt ngang đặc điểm hình ảnh mạch vành trên CLVT-256 ở các BN THA tại Bệnh viện Hữu nghị Việt Đức từ tháng 02 - 7/2021. Kết quả: 269 BN (129 nam và 140 nữ), tuổi trung bình là 72,6 ± 10,5 (từ 41 - 90 tuổi). Vôi hóa mạch vành (VHMV) gặp ở 97 nam (75,2%) và 90 nữ (64,3%) (p = 0,05). Tỷ lệ VHMV ở BN > 60 tuổi là cao hơn các BN £ 60 tuổi (72,2% so với 51,4%; p = 0,01). Về đặc điểm hẹp mạch vành (HMV), tuổi trung vị của các BN có HMV < 50% có tuổi trung vị thấp hơn các BN có HMV ³ 50% (72 so với 76 tuổi; p < 0,01). Mặt khác, BN > 60 tuổi có nguy cơ mắc HMV ³ 50% cao hơn nhóm £ 60 (OR: 5,9; 95%CI: 1,4 - 25,6; p < 0,01). Điểm vôi hóa Agatston là thấp hơn ở các BN có HMV < 50% so với các trường hợp HMV ³ 50% (23 so với 391; p < 0,01). Các BN có VHMV có nguy cơ mắc HMV ³ 50% cao hơn so với nhóm không có VHMV (OR: 6,6; 95%CI: 2,5 - 17,1; p < 0,01), và các BN có VHMV nặng (Agatston > 400 điểm) có nguy cơ mắc HMV ³50% cao hơn các BN có vôi hóa ít hơn hoặc không vôi hóa (OR: 17,3; 95%CI: 8,4 - 51,9; p < 0,01). Đường cong ROC dự báo điểm vôi hóa Agatston với HMV ³ 50% có AUC = 0,824 và điểm cut-off là 104 điểm với Sn = 77% và Sp = 76%. Kết luận: Điểm vôi hóa Agatston cao và tuổi cao là những yếu tố có liên đến HMV nặng ở các người bệnh THA.

Background: Laryngoscopic manipulation and endotracheal intubation are always a matter of concernwhich capable of producing tachycardia, arrhythmias and hypertension which is generally well tolerated inhealthy patient. In Hypertensive patient cardiovascular response to laryngoscopy and intubation is exaggerated. Aims: To assess the effectiveness in attenuation of haemodynamic responses to laryngoscopy andendotracheal intubation with different doses of intravenous dexmedetomidine in controlled hypertensivepatients with no adverse effects. Methods: This prospective Randomized controlled trial was carried out with 60 patients belonging toAmerican Society of Anesthesiologists (ASA) Physical Status II posted for elective general anaesthesia.Patients were randomly divided into three groups with fixed card sampling, where, patients who receivedIV dexmedetomidine 0.5 μg/kg diluted to 50 ml with normal saline as infusion over 10 min was consideredas group A, patients who received IV dexmedetomidine 0.75 μg/kg diluted to 50 ml with normal salinewas considered as group B and patients who received IV dexmedetomidine 1 μg/kg diluted to 50 ml withnormal saline was considered as group C. The primary outcome measures were haemodynamic responseat 1, 3 and 5 min after intubation. The secondary outcome measures were to note down any adverseeffects associated with drugs. Result: The groups were well matched for their demographic data . Male to female ratio was 1:1 in allthree group. The mean height, weight and BMI were almost similar among three groups. In this studybaseline readings of SBP, DBP, MAP and HR were almost similar in all three groups and statistically notsignificant. Maximum intubation response was seen at 1 min post intubation in all the three groups. Themean SBP of group A varied from 144.8±8.4 mmHg to 118.5±4.4 mmHg that of group B varied from134.8±4.1 to 122.0±4.2 mmHg and then group C varied from 126.5±15.5 mmHg to 103.8±8.4 mmHgduring different evaluation period (p<0.05). The mean DBP of group A varied from 91.8±7.6 mmHg to72.4±5.8 mmHg that of group B varied from 81.3±5.2 to 70.3±2.5 mmHg and then group C varied from80.9±6.7 mmHg to 63.4±2.4 mmHg during different evaluation period (p<0.05). The mean MAP of groupA varied from 109.0±5.6 mmHg to 87.5±4.4 mmHg that of group B varied from 98.7±2.5 to 86.3±3.4mmHg and then group C varied from 95.5±9.2 mmHg to 76.5±3.4 mmHg during different evaluationperiod (p<0.05). The mean heart rate of group A varied from 94.5±12.7 bpm to 75.2±10.5 bpm that ofgroup B varied from 87.3±8.3 to 75.0±6.6 bpm and then group C varied from 81.1±7.2 bpm to 66.2±8.1bpm during different evaluation period (p<0.05). Conclusion: Dexmedetomidine in doses of 0.75 μg/kg was more effective compared to 0.05 μg/kg and1μg/kg in attenuating haemodynamic response to laryngoscopy and endotracheal intubation withoutproducing adverse effects in control hypertensive patients. JBSA 2020; 33(2): 69-77

Background:Upadacitinib (UPA), an oral Janus kinase inhibitor (JAKi), has demonstrated efficacy in the phase 3 SELECT clinical program, conducted across a range of patients (pts) with rheumatoid arthritis (RA).1–6 Real-world data for UPA, including in pts previously treated with a JAKi, have not yet been reported since global approvals beginning in 2019.Objectives:To assess the characteristics of US-based pts receiving UPA and its effectiveness in clinical practice at 3 months.Methods:This observational study included US-based pts from the United Rheumatology Normalized Integrated Community Evidence (UR-NICE) database who initiated UPA 15 mg once daily from FDA approval (August 2019) to July 31, 2020 and had ≥6-month pre-baseline data available. Effectiveness was assessed in pts with a reported Clinical Disease Activity Index (CDAI) score at 3 months after UPA initiation and included proportions of pts achieving CDAI remission (≤2.8), CDAI low disease activity (≤10), other disease activity measures, and pt-reported outcomes. A subgroup analysis assessed UPA effectiveness in pts with or without prior tofacitinib (TOFA) treatment.Results:This analysis included 252 pts treated with UPA 15 mg, of whom 98 (38.9%) received UPA monotherapy and 154 (61.1%) received UPA combined with conventional synthetic (cs) disease-modifying antirheumatic drugs (DMARDs). 64.3% of pts were from the Southern region of the USA. 86.1%, 72.2%, and 47.6% of pts had been previously treated with csDMARDs, biologic DMARDs, and JAKis, respectively. Baseline characteristics were largely similar between UPA monotherapy and combination therapy groups and those with or without prior TOFA treatment (Table 1). Pts with prior TOFA treatment had a longer duration of RA since diagnosis and higher steroid use versus those without. UPA 15 mg improved disease activity scores (including CDAI) and pt-reported outcomes (including physical function and pain) after 3 months of treatment (Figure 1). Similar effectiveness was observed with UPA 15 mg in pts with or without prior TOFA treatment.Conclusion:In the UR-NICE real-world database of US-based pts, improvements in clinical and pt-reported outcomes were observed at 3 months in UPA-treated pts with RA, including those with or without prior TOFA treatment, despite the treatment-refractory population included in this dataset.References:[1]Burmester GR, et al. Lancet 2018;391:2503–12.[2]Smolen JS, et al. Lancet 2019;393:2303–11.[3]Fleischmann R, et al. Arthritis Rheumatol 2019;71:1788–800.[4]Genovese MC, et al. Lancet 2018;391:2513–24.[5]van Vollenhoven R, et al. Arthritis Rheumatol 2020;72:1607–20.[6]Rubbert-Roth A, et al. N Engl J Med 2020;383:1511–21.Table 1.Baseline characteristicsn (%), unless otherwise statedFull analysis set(n=252)Pts with prior TOFA treatment(n=113)Pts without prior TOFA treatment (n=139)Mean (SD) exposure, days219.7 (112.1)215.7 (116.7)222.9 (108.5)Female199 (79.0)85 (75.2)114 (82.0)Age ≥65 years75 (29.8)34 (30.1)41 (29.5)Oral steroid use140 (55.6)70 (61.9)70 (50.4)Prior csDMARDs217 (86.1)102 (90.3)115 (82.7)Prior TOFA113 (44.8)113 (100.0)0Prior biologic DMARDs182 (72.2)86 (76.1)96 (69.1)Tumor necrosis factor inhibitor147 (58.3)66 (58.4)81 (58.3)Interleukin-6 receptor inhibitor87 (34.5)47 (41.6)40 (28.8)nMean (SD)nMean (SD)nMean (SD)Duration of RA diagnosis, years1884.0 (3.0)895.1 (2.9)993.1 (2.8)Methotrexate dose, mg/week8817.0 (5.1)2817.8 (5.0)6016.6 (5.2)SJC282394.8 (5.7)1084.5 (5.0)1315.0 (6.2)TJC282376.5 (6.7)1076.5 (6.8)1306.5 (6.6)CDAI22520.4 (13.4)10520.2 (13.5)12020.6 (13.3)Routine assessment of patient index data 31654.2 (2.3)724.2 (2.4)934.3 (2.2)Disease Activity Score in 28 joints based on C-reactive protein1673.9 (1.5)833.9 (1.5)843.9 (1.5)Health Assessment Questionnaire-Disability Index1702.5 (2.1)742.4 (2.2)962.5 (2.1)Pain(0–10)22956.5 (28.5)10456.9 (29.3)12556.1 (28.0)SD, standard deviation; S/TJC, swollen/tender joint countAcknowledgements:AbbVie funded this study; contributed to its design; participated in data collection, analysis, and interpretation of the data; and participated in the writing, review, and approval of the abstract. No honoraria or payments were made for authorship. Medical writing support was provided by Hilary Wong, PhD, of 2 the Nth (Cheshire, UK), and was funded by AbbVie.Disclosure of Interests:Allan Gibofsky Shareholder of: AbbVie, Amgen, Johnson & Johnson, and Pfizer, Consultant of: AbbVie, Celgene, Eli Lilly, Flexion, Pfizer, Relburn Pharma, and Samumed. Paid consultant with investment analysts on behalf of the Gerson Lehrman Group, Bhavna Dhillon Shareholder of: May own stock or options in United Rheumatology, Employee of: United Rheumatology, Mark E. Pearson Shareholder of: May own AbbVie stock or options, Namita Tundia Shareholder of: May own stock or options in AbbVie, Employee of: AbbVie, Yanna Song Shareholder of: May own stock or options in AbbVie, Employee of: AbbVie, Kendall Dunlap Shareholder of: May own stocks or shares in AbbVie, Employee of: AbbVie, Grace Wright Speakers bureau: AbbVie, Amgen, Bristol-Myers Squibb, Eli Lilly, Exagen, Myriad Autoimmune, Novartis, Sanofi/Regeneron, UCB, and Vindico, Consultant of: AbbVie, Amgen, Bristol-Myers Squibb, Eli Lilly, Exagen, Gilead, Janssen, Myriad Autoimmune, Novartis, Pfizer, Sanofi/Regeneron, and UCB, Employee of: President and Founder of the Association of Women in Rheumatology

Abstract Background Chemotherapy use closer to the end of life is a marker of poor-quality care. There are now multiple studies and local reviews addressing this issue. Understanding the practice locally will give valuable insight and opportunity for improvement. Methods The study is a retrospective chart review of patients on chemotherapy at the Windsor Regional Cancer Center who died between April 1st, 2016 to December 31st, 2018. Information on demographics, type of cancer, type, intent and route of chemotherapy, line of chemotherapy, referral to hospice and palliative care services was collected. Results A total of 681 patients on chemotherapy died between April 1st, 2016 to Dec 13th, 2018. Of these, 119 (17.4 %) died within 30 days following chemotherapy. Chemotherapy was parenteral (Intravenous and Subcutaneous) for the majority (75.2%) of the patients. Most (66.4%) of the patients died of disease progression. Intent for chemotherapy was palliative in 85% of patients, adjuvant/neoadjuvant in 6.6% and curative in 8.4% of the patients. Chemotherapy was 1st, 2nd, 3rd line or more in 67.4%, 21.3% and 11.3% of the patients respectively. The type of chemotherapy was conventional in 74.3% of patients and targeted/immunotherapy in 25.7% of patients. Of the variables studied, lack of palliative referral and having lung cancer or melanoma were significantly associated with higher risk of getting chemotherapy within the last 30 days of life. The odds of getting chemotherapy within the last 30 days of life was 0.35, 95% CI (0.24-0.53), P <0.001 for those who were referred to palliative care. On the other hand, the odds of getting chemotherapy were 4.18, 95% CI (1.17-13.71), P = 0.037 and 2.21, 95% CI (1.24-4.01), P = 0.037 for those with melanoma and lung cancer respectively. In addition, those with early referral to palliative care (90 days or more prior to death) were least likely to receive chemotherapy within the last 30 days of life. Conclusion Administration of chemotherapy within the last 30 days of life could cause unnecessary suffering to patients and cost to society. Early referral to palliative care was significantly associated with reduced risk of getting chemotherapy within the last 30 days of life in this study. Prospective study is recommended to further investigate the role of early palliative referral on use of chemotherapy during the last 30 days of life.

Background: Early interventions that improve vital organ perfusion will reduce the number of lives lost from blood loss injuries. We have shown that generating 10 second (~0.1 Hz) fluctuations or “oscillations” in arterial pressure and blood flow during simulated hemorrhage protects cerebral tissue oxygenation. Lower body negative pressure (LBNP) was used to both simulate hemorrhage, and induce the hemodynamic oscillations in these previous studies. However, the magnitude of cerebral tissue ischemia is limited to 20-30% with LBNP due to the onset of pre-syncopal symptoms. To examine the effect of 0.1 Hz hemodynamic oscillations on blood flow delivery and tissue oxygenation of severely ischemic tissues, we developed a limb ischemia model. Hypothesis: Oscillatory arterial pressure and blood flow will attenuate reductions in brachial artery blood flow and forearm tissue oxygenation in a severely ischemic limb. Methods: Nine healthy human subjects (5M, 4F; 27.2 ± 4.1 y) completed two experimental protocols separated by ≥48 h. In both conditions, ischemia of the forearm was induced with a pneumatic cuff on the upper arm to decrease brachial artery (BA) blood velocity by ~70-80% from baseline. In the oscillation condition (OSC), 0.1 Hz oscillations in mean arterial pressure (MAP) and BA blood flow were then induced by inflating and deflating bilateral thigh cuffs every 10 seconds (0.1 Hz) throughout the forearm ischemia period. In the control condition (CON), the thigh cuffs were in place, but were inactive throughout the forearm ischemia period. BA blood flow was measured via duplex ultrasound, forearm muscle tissue oxygenation (SmO2) was measured via near infrared spectroscopy, and arterial pressure was measured via finger photoplethysmography. Results: The magnitude of forearm ischemia, indexed by the reduction in BA blood velocity, was matched between protocols (CON: -75.2 ± 8.4 % vs. OSC: -78.3 ± 7.8 %, p=0.20). Power spectral density of 0.1 Hz oscillations in MAP (CON: 19.4 ± 22.8 mmHg2 vs. OSC: 716.8 ± 514.6 mmHg2; p<0.001) and BA blood velocity (CON: 0.7 ± 1.0 cm/s2 vs. OSC: 10.6 ± 7.1 cm/s2, p=0.02) were greater with oscillatory thigh cuff compression compared with the control condition. While oscillatory thigh cuff compression during forearm ischemia had no effect on absolute MAP (CON: 94.3 ± 6.6 mmHg vs. OSC: 94.4 ± 10.8 mmHg, p=0.99), BA blood flow (CON: 9.7 ± 5.8 ml/min vs. OSC: 9.5 ± 7.3 ml/min, p=0.82), or BA conductance (CON: 0.10 ± 0.06 ml/min/mmHg vs. OSC: 0.09 ± 0.06 ml/min/mmHg, p=0.39), the reduction in SmO2 was attenuated (CON: -38.7 ± 8.3 % vs. OSC: -28.4 ± 9.7 %; p=0.04). These data provide further evidence for the use of 0.1 Hz hemodynamic oscillations as a therapeutic intervention for conditions associated with severe vital organ ischemia such as hemorrhage, stroke, myocardial infarction, and sepsis. National Institutes of Health/National Institute on Aging T32 Ruth Kirschstein Institutional National Research Service Award (T32AG020494); American Heart Association Transformational Project Award (19TPA34910073) This is the full abstract presented at the American Physiology Summit 2023 meeting and is only available in HTML format. There are no additional versions or additional content available for this abstract. Physiology was not involved in the peer review process.

ABSTRACT Forty-three new U-Pb zircon ages from metasedimentary and igneous rock units throughout the Cobequid Highlands of northern mainland Nova Scotia, Canada, provide new insights into the Neoproterozoic evolution of this long-enigmatic part of Avalonia in the northern Appalachian orogen. Contrasts in ages and rock types resulted in the identification of fault-bounded Neoproterozoic assemblages of units forming the Bass River, Jeffers, and Mount Ephraim blocks. In the Bass River block, quartzite, metawacke, and minor calc-silicate rocks and marble (Gamble Brook Formation) with a maximum depositional age of 945 ± 12 Ma are associated with subaqueous mafic volcanic rocks, siltstone, and ironstone (Folly River Formation) and intruded by 615–600 Ma calc-alkalic subduction-related dioritic to granitic rocks of the Bass River plutonic suite. The contrasting Jeffers block forms most of the Cobequid Highlands and consists mainly of intermediate to felsic volcanic, epiclastic, and minor plutonic rocks. The western and eastern areas of that block yielded ages mainly ca. 607–592 Ma for both volcanic and plutonic rocks, whereas the central area has ages of ca. 630–625 Ma from both volcanic and plutonic rocks and inheritance in overlying Devonian conglomerate. The Mount Ephraim block forms the eastern part of the highlands and includes possible ca. 800 Ma quartzofeldspathic, semipelitic and pelitic gneiss and schist of the Mount Thom Formation, ca. 752 Ma volcanic arc rocks of the Dalhousie Mountain Formation and related 752–730 Ma gabbroic/dioritic to granitic plutons of the Mount Ephraim plutonic suite and Six Mile Brook pluton, as well as ca. 631 Ma granitoid rocks of the Gunshot Brook pluton. The pre–750 Ma high-grade regional metamorphism and deformation and 752–730 Ma subduction-related magmatism recorded in the Mount Ephraim block were previously unrecognized in Avalonia. Evidence from zircon inheritance and Sm-Nd isotopic data in igneous units suggests linkages among these now-separate areas, and comparison with other parts of Avalonia in the northern Appalachian orogen suggests similarity to southeastern New England.

Врожденная дисфункция надпочечников (ВДКН) - группа аутосомно-рецессивных заболеваний, при которых происходит дефект одного из ферментов или транспортных белков, принимающих участие в синтезе кортизола в коре надпочечников. В РФ неонатальный скрининг на выявление дефицита 21-гидроксилазы проводится в обязательном порядке, согласно приказу Минздравсоцразвития РФ от 22.03.2006 N 185. Приводим свое клиническое наблюдение. Клинический случай: пациентка С., 19 лет, обратилась с жалобами к гинекологу на нарушение менструального цикла, угревые высыпания на лице, груди, головные боли. Из анамнеза: менархе наступили в 14 лет, менструальный цикл до 16 лет был регулярный. В последующем появилась нерегулярность цикла (опсоменарея), акне, чему пациентка не предала значения. В 2021 году обратилась за медицинской помощью. После осмотра гинеколога, который диагностировал гипертрофированный клитор и признаки генитального инфантилизма 2 ст. по УЗИ диагностике органов малого таза, пациентка направлена к эндокринологу. Объективно: на лице множественные акне, единичные волосы остевые на подбородке, усиленный рост волос на предплечьях, телосложение по женскому типу, рост - 154 см, вес - 51,5 кг, ИМТ - 22кг/м2 , костно - мышечный аппарат без видимых изменений, дыхание везикулярное, чд 16 в мин, АД 130/80 мм.рт.ст, пульс - 68 в мин., молочные железы - v степени развития по Таннеру. Щитовидная железа не увеличена, безболезненная, смещаемая, узлов нет. Половое оволосение по женскому типу. Лабораторные данные: ОАК: гемоглобин -158 г/л, эритроциты - 5,19×1012, лейкоформула без патологии, тромбоциты -289×109 . Биохимический анализ крови: глюкоза крови - 5,3 ммоль/л, калий - 3,7ммоль/л, натрий - 138 ммоль/л, общий белок - 75,2 г/л, креатинин - 66 ммоль/л. Гормональный профиль: ТТГ - 1,23мМЕ/л, Т4св - 10,1 пмоль/л, тестостерон общий - 15,09 нмоль/л, ДГЭА-S - 838,7 мкг/дл, тестостерон свободный - 0,329 нмоль/л, ФСГ - 7,6 МЕ/л, ЛГ - 2,3 МЕ/л, пролактин - 329,6 мМЕ/л., 17-OH-прогестерон - >35нмоль/л (26.11.2021), 17-OH-прогестерон - 1021 нмоль/л от (21.12.2021). По УЗИ почки и надпочечники без патологии. В связи с высоким уровнем 17-OH-прогестерона (соответственно клиническим рекомендациям), генетическое исследование не проведено. Диагностирована ВДКН, неклассическая форма и назначена терапия преднизолоном в дозе 5 мг/сут. Через месяц терапии уменьшилось количество акне, уровень 17-OH-прогестерона - 500 нмоль/л. С 21.07.22. по 26.07.22 г находилась на стационарном лечении в эндокринном отделении ГАУЗ НГКБ №1. Выявлено снижение уровня кортизола до 40 нмоль/л. В связи с высокими показателями 17-OH-прогестерона и диагностированной ятрогенной надпочечниковой недостаточностью, рекомендовано повышение преднизолона до 10 мг вечером, с подключением кортефа 10 мг утром. На данной терапии стабилизировался менструальный цикл, прекратился рост волос на лице, исчезли акне. Уровень 17-OH-прогестерона - 4,03 нмоль/л (25.08.2022). Заключение: в данном клиническом случае показана поздняя диагностика заболевания, которая и привела к развитию выраженной клинической картины.