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A Lorentzian manifold, N, endowed with a time function, tau, can be converted into a metric space using the null distance, d circumflex expressionccent (tau), defined by Sormani and Vega [Classical Quant. Grav. 33(8), 085001 (2016)]. We show that if the time function is a regular cosmological time function as studied by Andersson, Galloway, and Howard [Classical Quant. Grav. 15(2), 309-322 (1998)], and also by Wald and Yip [J. Math. Phys. 22, 2659-2665 (1981)], or if, more generally, it satisfies the anti-Lipschitz condition of Chrusciel, Grant, and Minguzzi [Ann. Henri Poincare 17(10), 2801-2824 (2016)], then the causal structure is encoded by the null distance in the following sense: for any p is an element of N, there is an open neighborhood U-p such that for any q is an element of U-p, we have d circumflex expressionccent (tau)(p, q) = tau(q) - tau(p) if and only if q lies in the causal future of p. The local encoding of causality can be applied to prove the global encoding of causality in a variety of settings, including spacetimes N where T is a proper function. As a consequence, in dimension n + 1, n >= 2, we prove that if there is a bijective map between two such spacetimes, F : M-1 -> M-2, which preserves the cosmological time function, tau(2)(F(p)) = tau(1)(p) for any p is an element of M-1, and preserves the null distance, d circumflex expressionccent tau(2) (F(p), F(q)) = d circumflex expressionccent tau(1) (p, q) for any p, q is an element of M-1, then there is a Lorentzian isometry between them, F*g(1) = g(2). This yields a canonical procedure allowing us to convert large classes of spacetimes into unique metric spaces with causal structures and time functions. This will be applied in our upcoming work to define spacetime intrinsic flat convergence.

A Lorentzian manifold, N, endowed with a time function, tau, can be converted into a metric space using the null distance, d circumflex expressionccent (tau), defined by Sormani and Vega [Classical Quant. Grav. 33(8), 085001 (2016)]. We show that if the time function is a regular cosmological time function as studied by Andersson, Galloway, and Howard [Classical Quant. Grav. 15(2), 309-322 (1998)], and also by Wald and Yip [J. Math. Phys. 22, 2659-2665 (1981)], or if, more generally, it satisfies the anti-Lipschitz condition of Chrusciel, Grant, and Minguzzi [Ann. Henri Poincare 17(10), 2801-2824 (2016)], then the causal structure is encoded by the null distance in the following sense: for any p is an element of N, there is an open neighborhood U-p such that for any q is an element of U-p, we have d circumflex expressionccent (tau)(p, q) = tau(q) - tau(p) if and only if q lies in the causal future of p. The local encoding of causality can be applied to prove the global encoding of causality in a variety of settings, including spacetimes N where T is a proper function. As a consequence, in dimension n + 1, n >= 2, we prove that if there is a bijective map between two such spacetimes, F : M-1 -> M-2, which preserves the cosmological time function, tau(2)(F(p)) = tau(1)(p) for any p is an element of M-1, and preserves the null distance, d circumflex expressionccent tau(2) (F(p), F(q)) = d circumflex expressionccent tau(1) (p, q) for any p, q is an element of M-1, then there is a Lorentzian isometry between them, F*g(1) = g(2). This yields a canonical procedure allowing us to convert large classes of spacetimes into unique metric spaces with causal structures and time functions. This will be applied in our upcoming work to define spacetime intrinsic flat convergence.

A Lorentzian manifold, N, endowed with a time function, tau, can be converted into a metric space using the null distance, d circumflex expressionccent (tau), defined by Sormani and Vega [Classical Quant. Grav. 33(8), 085001 (2016)]. We show that if the time function is a regular cosmological time function as studied by Andersson, Galloway, and Howard [Classical Quant. Grav. 15(2), 309-322 (1998)], and also by Wald and Yip [J. Math. Phys. 22, 2659-2665 (1981)], or if, more generally, it satisfies the anti-Lipschitz condition of Chrusciel, Grant, and Minguzzi [Ann. Henri Poincare 17(10), 2801-2824 (2016)], then the causal structure is encoded by the null distance in the following sense: for any p is an element of N, there is an open neighborhood U-p such that for any q is an element of U-p, we have d circumflex expressionccent (tau)(p, q) = tau(q) - tau(p) if and only if q lies in the causal future of p. The local encoding of causality can be applied to prove the global encoding of causality in a variety of settings, including spacetimes N where T is a proper function. As a consequence, in dimension n + 1, n >= 2, we prove that if there is a bijective map between two such spacetimes, F : M-1 -> M-2, which preserves the cosmological time function, tau(2)(F(p)) = tau(1)(p) for any p is an element of M-1, and preserves the null distance, d circumflex expressionccent tau(2) (F(p), F(q)) = d circumflex expressionccent tau(1) (p, q) for any p, q is an element of M-1, then there is a Lorentzian isometry between them, F*g(1) = g(2). This yields a canonical procedure allowing us to convert large classes of spacetimes into unique metric spaces with causal structures and time functions. This will be applied in our upcoming work to define spacetime intrinsic flat convergence.

A Lorentzian manifold, N, endowed with a time function, tau, can be converted into a metric space using the null distance, d circumflex expressionccent (tau), defined by Sormani and Vega [Classical Quant. Grav. 33(8), 085001 (2016)]. We show that if the time function is a regular cosmological time function as studied by Andersson, Galloway, and Howard [Classical Quant. Grav. 15(2), 309-322 (1998)], and also by Wald and Yip [J. Math. Phys. 22, 2659-2665 (1981)], or if, more generally, it satisfies the anti-Lipschitz condition of Chrusciel, Grant, and Minguzzi [Ann. Henri Poincare 17(10), 2801-2824 (2016)], then the causal structure is encoded by the null distance in the following sense: for any p is an element of N, there is an open neighborhood U-p such that for any q is an element of U-p, we have d circumflex expressionccent (tau)(p, q) = tau(q) - tau(p) if and only if q lies in the causal future of p. The local encoding of causality can be applied to prove the global encoding of causality in a variety of settings, including spacetimes N where T is a proper function. As a consequence, in dimension n + 1, n >= 2, we prove that if there is a bijective map between two such spacetimes, F : M-1 -> M-2, which preserves the cosmological time function, tau(2)(F(p)) = tau(1)(p) for any p is an element of M-1, and preserves the null distance, d circumflex expressionccent tau(2) (F(p), F(q)) = d circumflex expressionccent tau(1) (p, q) for any p, q is an element of M-1, then there is a Lorentzian isometry between them, F*g(1) = g(2). This yields a canonical procedure allowing us to convert large classes of spacetimes into unique metric spaces with causal structures and time functions. This will be applied in our upcoming work to define spacetime intrinsic flat convergence.

A Lorentzian manifold, N, endowed with a time function, tau, can be converted into a metric space using the null distance, d circumflex expressionccent (tau), defined by Sormani and Vega [Classical Quant. Grav. 33(8), 085001 (2016)]. We show that if the time function is a regular cosmological time function as studied by Andersson, Galloway, and Howard [Classical Quant. Grav. 15(2), 309-322 (1998)], and also by Wald and Yip [J. Math. Phys. 22, 2659-2665 (1981)], or if, more generally, it satisfies the anti-Lipschitz condition of Chrusciel, Grant, and Minguzzi [Ann. Henri Poincare 17(10), 2801-2824 (2016)], then the causal structure is encoded by the null distance in the following sense: for any p is an element of N, there is an open neighborhood U-p such that for any q is an element of U-p, we have d circumflex expressionccent (tau)(p, q) = tau(q) - tau(p) if and only if q lies in the causal future of p. The local encoding of causality can be applied to prove the global encoding of causality in a variety of settings, including spacetimes N where T is a proper function. As a consequence, in dimension n + 1, n >= 2, we prove that if there is a bijective map between two such spacetimes, F : M-1 -> M-2, which preserves the cosmological time function, tau(2)(F(p)) = tau(1)(p) for any p is an element of M-1, and preserves the null distance, d circumflex expressionccent tau(2) (F(p), F(q)) = d circumflex expressionccent tau(1) (p, q) for any p, q is an element of M-1, then there is a Lorentzian isometry between them, F*g(1) = g(2). This yields a canonical procedure allowing us to convert large classes of spacetimes into unique metric spaces with causal structures and time functions. This will be applied in our upcoming work to define spacetime intrinsic flat convergence.

Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome (WS) is a combined immunodeficiency caused by gain-of-function mutations in the C-X-C chemokine receptor type 4 (CXCR4) gene. We characterize a unique international cohort of 66 patients, including 57 (86%) cases previously unreported, with variable clinical phenotypes. Of 17 distinct CXCR4 genetic variants within our cohort, 11 were novel pathogenic variants affecting 15 individuals (23%). All variants affect the same CXCR4 region and impair CXCR4 internalization resulting in hyperactive signaling. The median age of diagnosis in our cohort (5.5 years) indicates WHIM syndrome can commonly present in childhood, although some patients are not diagnosed until adulthood. The prevalence and mean age of recognition and/or onset of clinical manifestations within our cohort were infections 88%/1.6 years, neutropenia 98%/3.8 years, lymphopenia 88%/5.0 years, and warts 40%/12.1 years. However, we report greater prevalence and variety of autoimmune complications of WHIM syndrome (21.2%) than reported previously. Patients with versus without family history of WHIM syndrome were diagnosed earlier (22%, average age 1.3 years versus 78%, average age 5 years, respectively). Patients with a family history of WHIM syndrome also received earlier treatment, experienced less hospitalization, and had less end-organ damage. This observation reinforces previous reports that early treatment for WHIM syndrome improves outcomes. Only one patient died; death was attributed to complications of hematopoietic stem cell transplantation. The variable expressivity of WHIM syndrome in pediatric patients delays their diagnosis and therapy. Early-onset bacterial infections with severe neutropenia and/or lymphopenia should prompt genetic testing for WHIM syndrome, even in the absence of warts.

When working with asymptotically hyperbolic initial data sets for general relativity it is convenient to assume certain simplifying properties. We prove that the subset of initial data sets with such properties is dense in the set of physically reasonable asymptotically hyperbolic initial data sets. More specifically, we show that an asymptotically hyperbolic initial data set with non-negative local energy density can be approximated by an initial data set with strictly positive local energy density and a simple structure at infinity, while changing the mass arbitrarily little. This is achieved by suitably modifying the argument used by Eichmair, Huang, Lee and Schoen in the asymptotically Euclidean case.

When working with asymptotically hyperbolic initial data sets for general relativity it is convenient to assume certain simplifying properties. We prove that the subset of initial data sets with such properties is dense in the set of physically reasonable asymptotically hyperbolic initial data sets. More specifically, we show that an asymptotically hyperbolic initial data set with non-negative local energy density can be approximated by an initial data set with strictly positive local energy density and a simple structure at infinity, while changing the mass arbitrarily little. This is achieved by suitably modifying the argument used by Eichmair, Huang, Lee and Schoen in the asymptotically Euclidean case., QC 20220304

Nijmegen breakage syndrome (NBS) is a DNA repair disorder characterized by combined immunodeficiency and a high predisposition to lymphoid malignancies. The majority of NBS patients are identified with a homozygous five base pair deletion in the Nibrin (NBN) gene (c.657_661del5, p.K219fsX19) with a founder effect observed in Caucasian European populations, especially of Slavic origin. We present here an analysis of a cohort of 136 NBS patients of Eastern Slav origin across Belarus, Ukraine, Russia, and Latvia with a focus on understanding the geographic distribution, incidence of malignancy, and treatment outcomes of this cohort. Our analysis shows that Belarus had the highest prevalence of NBS (2.3 per 1,000,000), followed by Ukraine (1.3 per 1,000,000), and Russia (0.7 per 1,000,000). Of note, the highest concentration of NBS cases was observed in the western regions of Belarus and Ukraine, where NBS prevalence exceeds 20 cases per 1,000,000 people, suggesting the presence of an “Eastern Slavic NBS hot spot.” The median age at diagnosis of this cohort ranged from 4 to 5 years, and delay in diagnosis was more pervasive in smaller cities and rural regions. A total of 62 (45%) patients developed malignancies, more commonly in males than females (55.2 vs. 34.2%; p=0.017). In 27 patients, NBS was diagnosed following the onset of malignancies (mean age: 8 years). Malignancies were mostly of lymphoid origin and predominantly non-Hodgkin lymphoma (NHL) (n=42, 68%); 38% of patients had diffuse large B-cell lymphoma. The 20-year overall survival rate of patients with malignancy was 24%. However, females with cancer experienced poorer event-free survival rates than males (16.6% vs. 46.8%, p=0.036). Of 136 NBS patients, 13 underwent hematopoietic stem cell transplantation (HSCT) with an overall survival of 3.5 years following treatment (range: 1 to 14 years). Indications for HSCT included malignancy (n=7) and immunodeficiency (n=6). Overall, 9% of patients in this cohort reac

We propose a new foliation of asymptotically Euclidean initial data sets by 2-spheres of constant spacetime mean curvature (STCMC). The leaves of the foliation have the STCMC-property regardless of the initial data set in which the foliation is constructed which asserts that there is a plethora of STCMC 2-spheres in a neighborhood of spatial infinity of any asymptotically flat spacetime. The STCMC-foliation can be understood as a equivariant relativistic generalization of the CMC-foliation suggested by Huisken and Yau (Invent Math 124:281-311, 1996). We show that a unique STCMC-foliation exists near infinity of any asymptotically Euclidean initial data set with non-vanishing energy which allows for the definition of a new notion of total center of mass for isolated systems. This STCMC-center of mass transforms equivariantly under the asymptotic Poincare group of the ambient spacetime and in particular evolves under the Einstein evolution equations like a point particle in Special Relativity. The new definition also remedies subtle deficiencies in the CMC-approach to defining the total center of mass suggested by Huisken and Yau (Invent Math 124:281-311, 1996) which were described by Cederbaum and Nerz (Ann Henri Poincare 16:1609-1631, 2015).

We solve the Jang equation with respect to asymptotically hyperbolic "hyperboloidal" initial data. The results are applied to give a non-spinor proof of the positive mass theorem in the asymptotically hyperbolic setting. This work focuses on the case when the spatial dimension is equal to three.

We propose a new foliation of asymptotically Euclidean initial data sets by 2-spheres of constant spacetime mean curvature (STCMC). The leaves of the foliation have the STCMC-property regardless of the initial data set in which the foliation is constructed which asserts that there is a plethora of STCMC 2-spheres in a neighborhood of spatial infinity of any asymptotically flat spacetime. The STCMC-foliation can be understood as a equivariant relativistic generalization of the CMC-foliation suggested by Huisken and Yau (Invent Math 124:281-311, 1996). We show that a unique STCMC-foliation exists near infinity of any asymptotically Euclidean initial data set with non-vanishing energy which allows for the definition of a new notion of total center of mass for isolated systems. This STCMC-center of mass transforms equivariantly under the asymptotic Poincare group of the ambient spacetime and in particular evolves under the Einstein evolution equations like a point particle in Special Relativity. The new definition also remedies subtle deficiencies in the CMC-approach to defining the total center of mass suggested by Huisken and Yau (Invent Math 124:281-311, 1996) which were described by Cederbaum and Nerz (Ann Henri Poincare 16:1609-1631, 2015).

We solve the Jang equation with respect to asymptotically hyperbolic "hyperboloidal" initial data. The results are applied to give a non-spinor proof of the positive mass theorem in the asymptotically hyperbolic setting. This work focuses on the case when the spatial dimension is equal to three.

We solve the Jang equation with respect to asymptotically hyperbolic "hyperboloidal" initial data. The results are applied to give a non-spinor proof of the positive mass theorem in the asymptotically hyperbolic setting. This work focuses on the case when the spatial dimension is equal to three.

We propose a new foliation of asymptotically Euclidean initial data sets by 2-spheres of constant spacetime mean curvature (STCMC). The leaves of the foliation have the STCMC-property regardless of the initial data set in which the foliation is constructed which asserts that there is a plethora of STCMC 2-spheres in a neighborhood of spatial infinity of any asymptotically flat spacetime. The STCMC-foliation can be understood as a equivariant relativistic generalization of the CMC-foliation suggested by Huisken and Yau (Invent Math 124:281-311, 1996). We show that a unique STCMC-foliation exists near infinity of any asymptotically Euclidean initial data set with non-vanishing energy which allows for the definition of a new notion of total center of mass for isolated systems. This STCMC-center of mass transforms equivariantly under the asymptotic Poincare group of the ambient spacetime and in particular evolves under the Einstein evolution equations like a point particle in Special Relativity. The new definition also remedies subtle deficiencies in the CMC-approach to defining the total center of mass suggested by Huisken and Yau (Invent Math 124:281-311, 1996) which were described by Cederbaum and Nerz (Ann Henri Poincare 16:1609-1631, 2015).

We solve the Jang equation with respect to asymptotically hyperbolic "hyperboloidal" initial data. The results are applied to give a non-spinor proof of the positive mass theorem in the asymptotically hyperbolic setting. This work focuses on the case when the spatial dimension is equal to three.

We propose a new foliation of asymptotically Euclidean initial data sets by 2-spheres of constant spacetime mean curvature (STCMC). The leaves of the foliation have the STCMC-property regardless of the initial data set in which the foliation is constructed which asserts that there is a plethora of STCMC 2-spheres in a neighborhood of spatial infinity of any asymptotically flat spacetime. The STCMC-foliation can be understood as a equivariant relativistic generalization of the CMC-foliation suggested by Huisken and Yau (Invent Math 124:281-311, 1996). We show that a unique STCMC-foliation exists near infinity of any asymptotically Euclidean initial data set with non-vanishing energy which allows for the definition of a new notion of total center of mass for isolated systems. This STCMC-center of mass transforms equivariantly under the asymptotic Poincare group of the ambient spacetime and in particular evolves under the Einstein evolution equations like a point particle in Special Relativity. The new definition also remedies subtle deficiencies in the CMC-approach to defining the total center of mass suggested by Huisken and Yau (Invent Math 124:281-311, 1996) which were described by Cederbaum and Nerz (Ann Henri Poincare 16:1609-1631, 2015).

We solve the Jang equation with respect to asymptotically hyperbolic "hyperboloidal" initial data. The results are applied to give a non-spinor proof of the positive mass theorem in the asymptotically hyperbolic setting. This work focuses on the case when the spatial dimension is equal to three.

We propose a new foliation of asymptotically Euclidean initial data sets by 2-spheres of constant spacetime mean curvature (STCMC). The leaves of the foliation have the STCMC-property regardless of the initial data set in which the foliation is constructed which asserts that there is a plethora of STCMC 2-spheres in a neighborhood of spatial infinity of any asymptotically flat spacetime. The STCMC-foliation can be understood as a equivariant relativistic generalization of the CMC-foliation suggested by Huisken and Yau (Invent Math 124:281-311, 1996). We show that a unique STCMC-foliation exists near infinity of any asymptotically Euclidean initial data set with non-vanishing energy which allows for the definition of a new notion of total center of mass for isolated systems. This STCMC-center of mass transforms equivariantly under the asymptotic Poincare group of the ambient spacetime and in particular evolves under the Einstein evolution equations like a point particle in Special Relativity. The new definition also remedies subtle deficiencies in the CMC-approach to defining the total center of mass suggested by Huisken and Yau (Invent Math 124:281-311, 1996) which were described by Cederbaum and Nerz (Ann Henri Poincare 16:1609-1631, 2015).

Актуальность. Данная работа представляет собой исследование и анализ влияния острого гнойного синусита на состояние пациента по данным визуально-аналоговой шкалы. При этом изучалось как общее влияние заболевания, так и вклад отдельных симптомов в общую картину. Актуальность проблемы обусловлена тем, что в периодической литературе преимущественно встречаются работы, посвященные хроническому риносинуситу. При этом острому гнойному риносинуситу посвящено относительно мало работ. Цель исследования: при помощи визуально-аналоговой шкалы оценить клиническую картину острого гнойного риносинусита с учетом отдельных симптомов (боль, заложенность носа, выделения из носа, снижение обоняния), влияния пола, возраста, количества пазух с воспалением, нозологии. Материалы и методы. 172 взрослых пациента с подтвержденным (в том числе пункциями околоносовых пазух) гнойным риносинуситом. Результаты. Острый гнойный риносинусит протекает со значимым снижением качества жизни, при этом доминирующее значение среди симптомов имеют заложенность носа и ринорея. Общее состояние оценивается пациентами тяжелее, чем каждый отдельный симптом, что может говорить о недостаточной оценке интоксикационного компонента заболевания или о взаимном усилении отдельных симптомов острого синусита. Выводы. Не обнаружены достоверные корреляции между отдельными симптомами риносинусита и количеством воспаленных околоносовых пазух, возрастом и полом практически для всех форм риносинусита. Лишь для фронтита выявлена корреляция между количеством воспаленных пазух и болью. Не обнаружено различий в оценке состояния между мужчинами и женщинами. Четыре пятых от количества пациентов (85,9 %) оценили свое состояние как тяжелое или средней тяжести, при этом более половины (59,3 %) имели непосредственное отрицательное влияние заболевания на качество жизни. Корреляционные взаимосвязи между оценкой общего состояния и отдельными симптомами были сопоставимы., Background. This work is a study and analysis of the effect of acute purulent sinusitis on the patient’s condition accor-ding to the visual analogue scale. Whereby, both the general effect of the disease and the contribution of individual symptoms to the clinical picture were studied. Background of the problem is due to the fact that in the periodical literature, there are mainly works on the chronic rhinosinusitis. At the same time, relatively few works are dealing with acute purulent rhinosinusitis. Objective: using a visual analogue scale to assess the clinical picture of acute purulent rhinosinusitis, taking into account individual symptoms (pain, nasal congestion, nasal discharge, decreased sense of smell), effects of sex, age, number of inflamed sinuses, nosology. Materials and methods. One hundred and seventy-two adult patients with confirmed (including punctures of the paranasal sinuses) purulent rhinosinusitis were examined. Results. Acute purulent rhinosinusitis occurs with a significant decrease in the quality of life, with nasal congestion and rhinorrhea being dominant among the symptoms. The general condition is assessed by patients as more severe than each individual symptom, which may indicate an insufficient assessment of the intoxication component of the disease or mutual reinforcement of some symptoms of acute sinusitis. Conclusions. No significant correlations were found between the individual symptoms of rhinosinusitis and the number of inflamed paranasal sinuses, age and sex for almost all forms of rhinosinusitis. Only in frontal sinusitis, a correlation was revealed between the number of inflamed sinuses and pain. No differences were found when in the assessment of the state between men and women. Four fifths of patients (85.9 %) rated their condition as severe or moderate-to-severe, with more than half (59.3 %) having a direct negative effect of the disease on quality of life. The correlations between the assessment of the general condition an, Актуальність. Дана робота являє собою дослідження й аналіз впливу гострого гнійного синуситу на стан пацієнта за даними візуально-аналогової шкали. При цьому вивчали як загальний вплив захворювання, так і внесок окремих симптомів у загальну картину. Актуальність проблеми обумовлена тим, що в періодичній літературі переважно зустрічаються роботи, присвячені хронічному риносинуситу. При цьому гострому гнійному риносинуситу присвячено відносно мало робіт. Мета дослідження: за допомогою візуально-аналогової шкали оцінити клінічну картину гострого гнійного риносинуситу з урахуванням окремих симптомів (біль, закладеність носа, виділення з носа, зниження нюху), впливу статі, віку, кількості пазух з запаленням, нозології. Матеріали та методи. 172 дорослих пацієнти з підтвердженим (у тому числі пункціями навколоносових пазух) гнійним риносинуситом. Результати. Гострий гнійний риносинусит перебігає зі значним зниженням якості життя, при цьому домінуюче значення серед симптомів мають закладеність носа і ринорея. Загальний стан оцінюється пацієнтами тяжче, ніж кожен окремий симптом, що може свідчити про недостатню оцінку інтоксикаційного компонента захворювання або про взаємне посилення окремих симптомів гострого синуситу. Висновки. Не виявлені вірогідні кореляції між окремими симптомами риносинуситу і кількістю запалених навколоносових пазух, віком і статтю практично для всіх форм риносинуситу. Лише для фронтиту виявлено кореляцію між кількістю запалених пазух і болем. Не визначено відмінностей в оцінці стану між чоловіками і жінками. Чотири п’ятих від кількості пацієнтів (85,9 %) оцінили свій стан як тяжкий або середньої тяжкості, при цьому більше половини (59,3 %) мали безпосередній негативний вплив захворювання на якість життя. Кореляційні взаємозв’язки між оцінкою загального стану і окремими симптомами можна було порівняти.

FOXN1 is the master regulatory gene of thymic epithelium development. FOXN1 deficiency leads to thymic aplasia, alopecia, and nail dystrophy, accounting for the nude/severe combined immunodeficiency (nu/SCID) phenotype in humans and mice. We identified several newborns with low levels of T cell receptor excision circles (TRECs) and T cell lymphopenia at birth, who carried heterozygous loss-of-function FOXN1 variants. Longitudinal analysis showed persistent T cell lymphopenia during infancy, often associated with nail dystrophy. Adult individuals with heterozygous FOXN1 variants had in most cases normal CD4+ but lower than normal CD8+ cell counts. We hypothesized a FOXN1 gene dosage effect on the function of thymic epithelial cells (TECs) and thymopoiesis and postulated that these effects would be more prominent early in life. To test this hypothesis, we analyzed TEC subset frequency and phenotype, early thymic progenitor (ETP) cell count, and expression of FOXN1 target genes (Ccl25, Cxcl12, Dll4, Scf, Psmb11, Prss16, and Cd83) in Foxn1nu/+ (nu/+) mice and age-matched wild-type (+/+) littermate controls. Both the frequency and the absolute count of ETP were significantly reduced in nu/+ mice up to 3 weeks of age. Analysis of the TEC compartment showed reduced expression of FOXN1 target genes and delayed maturation of the medullary TEC compartment in nu/+ mice. These observations establish a FOXN1 gene dosage effect on thymic function and identify FOXN1 haploinsufficiency as an important genetic determinant of T cell lymphopenia at birth.

The paper deals with the mathematical modeling of the controlled motion of an automatic underwater vehicle under conditions of inaccuracy and uncertainty of information support. Methodological and theoretical approaches based on the application of the principle of complexity and fuzzy logic are proposed.

The history of socio-statistical studying of manpower resources of Belarussian village are considered and presented in article. The stages of their study are highlighted and a brief description of each stage is presented. The necessity of socio-statistical studying of manpower resources of Belarussian village at the present stage is proved., В статье рассмотрена и проанализирована история социолого-статистического изучения трудовых ресурсов белорусского села. Выделены этапы их изучения и представлена краткая характеристика каждого этапа. Обосновывается необходимость социолого-статистического изучения трудовых ресурсов белорусского села на современном этапе.

Improving the efficiency of communication systems in the modern conditions of their development is one of the most urgent scientific and practical problems. Particular attention is paid to the problem of quantifying the effectiveness of the functioning of these systems, and not on individual indicators, but in a complex way, taking into account the influence of all indicators of quality on the result of the system's intended use. The purpose of the publication is to formalize the process of comprehensive assessment of the effectiveness of the communication system and to demonstrate, by way of example, the observance of the requirements for reliability and readiness, the opportunity to streamline its evaluation activities with the calculation of probabilistic quality indicators.Evaluation of the efficiency of the communication system is a multi-criteria problem of determining the real state of various components of the quality of its functioning. The formalization of the complex estimation of efficiency is proposed, taking into account the influence on its value of separate quality indicators. Scientific novelty of the work consists in quantitative estimation of the value of the probabilistic indices of individual components and a comprehensive assessment of the quality of the functioning of the communication system in general. The results obtained should be used both in assessing the effectiveness of existing and well-founded choices for the development of promising communication systems., Повышение эфективности функционирования систем связи в современых условиях их розвитие есть одной из актуальных научно-практических проблем. Особое внимание в решении этой проблемы уделяется вопросу количественной оценки эфективности функционирования определённых систем, причём не по отдельными показателям, а комплексно с учётом влияния всех показателей качества на результат применения системы по назначению. Публикация имеет цель формализации процесса комплексной оценки ефективности системи связи и показать на примере соблюдение требований по надёжности и готовности возможности упорядочить действия по её оценке с расчётом вероятностных показателей качества.Оценка эфективности системы связи – многокритериальная задача опредиления реального состояния различных составляющих качества её функционирования. Предложеная формализация комплексной оценки ефективности с учётом влияния на её значения отдельных показателей качества. Научная новизна роботы состоит в количественной оценке значения вероятностных показателей отдельных состовляющих и комплексной оценки качества функционирования системы связи в целом. Полученые результати целесообразно использовать как во время оценки ефективности существующих, так и при обоснованому вибору варианта розвития перспективных систем связи., Підвищення ефективності функціонування систем зв’язку в сучасних умовах їх розвитку є однією з актуальних науково-практичних проблем. Особлива увага у вирішенні цієї проблеми приділяється питанням кількісного оцінювання ефективності функціонування зазначених систем, причому не за окремими показниками, а комплексно з врахуванням впливу всіх показників якості на результат використання системи за призначенням. Публікація має за мету формалізацію процесу комплексної оцінки ефективності системи зв’язку та показати на прикладі дотримання вимог до надійності та готовності можливість упорядкування дій щодо її оцінки з розрахунком імовірнісних показників якості.Оцінка ефективності системи зв’язку – багатокритеріальна задача визначення реального стану різноманітних складових якості її функціонування. Запропоновано формалізацію комплексної оцінки ефективності з урахуванням впливу на її значення окремих показників якості. Наукова новизна роботи полягає у кількісній оцінці значення імовірнісних показників окремих складових і комплексного оцінювання якості функціонування системи зв’язку в цілому. Отримані результати доцільно використовувати як під час оцінки ефективності існуючих, так і при обґрунтованому виборі варіанту розвитку перспективних систем зв’язку.

We use the notion of intrinsic flat distance to address the almost rigidity of the positive mass theorem for asymptotically hyperbolic manifolds. In particular, we prove that a sequence of spherically symmetric asymptotically hyperbolic manifolds satisfying the conditions of the positive mass theorem converges to hyperbolic space in the intrinsic flat sense, if the limit of the mass along the sequence is zero.

We use the notion of intrinsic flat distance to address the almost rigidity of the positive mass theorem for asymptotically hyperbolic manifolds. In particular, we prove that a sequence of spherically symmetric asymptotically hyperbolic manifolds satisfying the conditions of the positive mass theorem converges to hyperbolic space in the intrinsic flat sense, if the limit of the mass along the sequence is zero.

We use the notion of intrinsic flat distance to address the almost rigidity of the positive mass theorem for asymptotically hyperbolic manifolds. In particular, we prove that a sequence of spherically symmetric asymptotically hyperbolic manifolds satisfying the conditions of the positive mass theorem converges to hyperbolic space in the intrinsic flat sense, if the limit of the mass along the sequence is zero.

The purpose of our study was to determine the capabilities of 18F-FDG PET/CT (fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography) for the lateralization and localization of the focus in temporal lobe epilepsy and mapping of eloquent areas of the brain in these patients. Materials and methods. 18F-FDG PET/CT was implemented on the basis of statistical analysis by means of three-dimensional stereotactic surface projection (3D-SSP) on the workstation ADW4.6 using the software package CortexID. At the same time, we have analyzed the clinical data, magnetic resonance imaging (MRI) and electroencephalography (EEG) results. Forty two patients (25 men and 17 women aged 33 ± 11 years) suffering from epilepsy with pharmacoresistant course of disease were examined using the following methods: brain MRI, 18F-FDG PET/CT, EEG and EEG-mapping. The time of the PET/CT since the last attack ranged from 3 to 14 days. Results. In 21 patients out of 42 (50 %), foci of hypometabolism were detected in the temporal lobe (mostly), and also parietal and frontal lobes of the cerebral hemispheres visually. The identified foci and areas of hypometabolism, as a rule, correspon­ded to the epileptogenic focus discovered by EEG-mapping. When comparing hypometabolic foci and areas identified visually and statistically on 18F-FDG PET/CT, the localization of epileptic focus according to the routine EEG was comparable in 16 of 42 patients and according to EEG-mapping in 20 of 42 patients. When evaluating 18F-FDG PET/CT using the software package ID Cortex, we have also detected hypometabolism and areas of metabolic activity asymmetry in 8 of 42 patients (19 %) that were not identified during standard visual assessment. In 31 % of cases (13 of 42 patients), there were no significant signs of pathological metabolic activity in the brain. Conclusions. 18F-FDG PET/CT of the brain is an important component of diagnostic algorithms in patients with epilepsy. It is important to no, Целью нашего исследования были определение возможностей 18F-ФДГ-ПЭТ/КТ (позитронно-эмиссионная томография, совмещенная с компьютерной томографией, с применением 18F-фтордезоксиглюкозы) для латерализации и локализации очага при височной и вневисочной эпилепсии и картирование функционально значимых зон мозга у данной категории пациентов.Материалы и методы. 18F-ФДГ-ПЭТ/КТ реализовывалась на основе статистического анализа с использованием трехмерной стереотаксической проекции поверхности (3D-SSP) на рабочей станции ADW4.6 с применением программного пакета CortexID. Одновременно анализировались клинические данные, результаты магнитно-резонансной томографии и электроэнцефалографии (ЭЭГ). Обследовано 42 пациента (25 мужчин и 17 женщин, возраст которых составил 33 ± 11 лет), страдающих эпилепсией с фармакорезистентным течением, с применением следующих методов: магнитно-резонансной томографии головного мозга, 18F-ФДГ-ПЭТ/КТ, ЭЭГ и ЭЭГ-картирования. Время проведения ПЭТ/КТ с момента последнего приступа составляло от 3 до 14 дней. Результаты. У 21 пациента из 42 (в 50 % случаев) визуально обнаружены очаги гипометаболизма в височной (преимущественно), а также теменной и лобной долях полушарий головного мозга. Выявленные фокусы и зоны гипометаболизма, как правило, соответствовали эпилептогенному очагу, выявляемому по данным ЭЭГ-картирования. При сравнении гипометаболических фокусов и областей, визуально и статистически выявленных на 18F-ФДГ-ПЭТ/КТ, локализация эпилептического очага по данным рутинной ЭЭГ была сопоставимой у 16 из 42 пациентов, а по данным ЭЭГ-картирования — у 20 из 42 пациентов. При оценке 18F-ФДГ-ПЭТ/КТ при помощи программного пакета CortexID дополнительно констатировались гипометаболизм и зоны асимметрии метаболической активности у 8 из 42 пациентов (в 19 % случаев), которые не были выявлены при стандартной визуальной оценке. В 31 % случаев (у 13 из 42 пациентов) отсутствовали достоверные признаки патологической метаболической активности головного мозга. Вывод, Метою нашого дослідження були визначення можливостей 18F-ФДГ-ПЕТ/КТ (позитронно-емісійна томографія, поєднана з комп’ютерною томографією, із застосуванням 18F-фтордезоксиглюкози) для латералізації та локалізації вогнища при скроневій і позаскроневій епілепсії і картування функціонально значущих зон мозку в даній категорії пацієнтів. Матеріали та методи. 18F-ФДГ-ПЕТ/КТ реалізовувалася на основі статистичного аналізу з використанням тривимірної стереотаксичної проекції поверхні (3D-SSP) на робочій станції ADW4.6 із застосуванням програмного пакета CortexID. Одночасно аналізувалися клінічні дані, результати магнітно-резонансної томографії та електроенцефалографії (ЕЕГ). Обстежено 42 пацієнти (25 чоловіків і 17 жінок, вік яких становив 33 ± 11 років), які страждають від епілепсії з фармакорезистентним перебігом, із застосуванням таких методів: магнітно-резонансної томографії головного мозку, 18F-ФДГ-ПЕТ/КТ, ЕЕГ і ЕЕГ-картування. Час проведення ПЕТ/КТ із моменту останнього нападу становив від 3 до 14 днів. Результати. У 21 пацієнта з 42 (у 50 % випадків) візуально виявлені вогнища гіпометаболізму в скроневій (переважно), а також тім’яній і лобовій частках півкуль головного мозку. Виявлені фокуси і зони гіпометаболізму, як правило, відповідали епілептогенному вогнищу, що встановлене за даними ЕЕГ-картування. При порівнянні гіпометаболічних фокусів і ділянок, візуально і статистично виявлених на 18F-ФДГ-ПЕТ/КТ, локалізація епілептичного вогнища за даними рутинної ЕЕГ була порівнянною в 16 з 42 пацієнтів, а за даними ЕЕГ-картування — у 20 з 42 пацієнтів. При оцінці 18F-ФДГ-ПЕТ/КТ за допомогою програмного пакета CortexID додатково констатувалися гіпометаболізм і зони асиметрії метаболічної активності у 8 з 42 пацієнтів (в 19 % випадків), що не були виявлені при стандартній візуальній оцінці. У 31 % випадків (у 13 з 42 пацієнтів) були відсутні вірогідні ознаки патологічної метаболічної активності головного мозку. Висновки. 18F-ФДГ-ПЕТ/КТ головного мозку є важливим компонентом

We use the notion of intrinsic flat distance to address the almost rigidity of the positive mass theorem for asymptotically hyperbolic manifolds. In particular, we prove that a sequence of spherically symmetric asymptotically hyperbolic manifolds satisfying the conditions of the positive mass theorem converges to hyperbolic space in the intrinsic flat sense, if the limit of the mass along the sequence is zero.

We use the notion of intrinsic flat distance to address the almost rigidity of the positive mass theorem for asymptotically hyperbolic manifolds. In particular, we prove that a sequence of spherically symmetric asymptotically hyperbolic manifolds satisfying the conditions of the positive mass theorem converges to hyperbolic space in the intrinsic flat sense, if the limit of the mass along the sequence is zero.

The article defines the purposes of monitoring; the essence of monitoring in terms of its effectiveness. It determines technique of carrying out monitoring. The author suggests the way to improve performance of monitoring.

Objective. The aim of our study was the comparison of the microstructure and neurometabolic disorders of the brain, as well as neuropathophysiological features of patients suffering from epilepsy and migraine. Methods. We investigated 60 patients suffering from migraine, aged 16–42 years (mean age was 29.30 ± 0.52 years) and 60 — with epilepsy, aged 18–51 years (mean age was 28.20 ± 0.98 years). 60 patients with migraine and 60 with epilepsy underwent diffusion tensor magnetic resonance imaging. 18 migraine and 28 epilepsy patients underwent proton nuclear magnetic resonance spectroscopy. All patients were examined using the computer electroencephalography. Migraine and epilepsy were diagnosed according to international guidelines. Results. The obtained data indicate not only a neuron tissue lesion in patients suffering from migraine and epilepsy but, probably, do not exclude a feedback in this event hierarchy and the deafferentation probability conditioned by a loss of hippocampal bonds. This state is confirmed by decreasing tracts in limbic zones that also plays an important role in the damage of brain extrahippocampal parts.Thus, hippocampus is one of the main structures in disintegrating neuronal combinatorics and forms peculiarities of clinical manifestations of paroxysmal states, involving these or those brain areas at these two diseases., Цель. Целью нашего исследования являлось сопоставление микроструктурных и нейрометаболических нарушений мозга, а также нейропатофизиологических особенностей у пациентов, страдающих от эпилепсии и мигрени. Методы. Нами обследовано 60 пациентов с мигренью в возрасте 16–42 лет (средний возраст составил 29,30 ± 0,52 года) и 60 — с эпилепсией в возрасте 18–51 года (средний возраст 28,20 ± 0,98 года). Шестидесяти пациентам с мигренью и 60 — с эпилепсией проведена диффузионная тензорная магнитно-резонансная томография. Протонная магнитно-резонансная спектроскопия выполнена 18 пациентам с мигренью и 28 — с эпилепсией. Все пациенты обследованы с использованием компьютерной электроэнцефалографии. Диагноз мигрени и эпилепсии был выставлен согласно международным рекомендациям. Результаты. Полученные данные не только указывают на повреждение нейрональной ткани у пациентов с мигренью и эпилепсией, но и, возможно, не исключают обратную связь в этой иерархии событий и вероятность деафферентации, обусловленной потерей гиппокампальных связей. Данное обстоятельство подтверждается уменьшением трактов в лимбических зонах, что играет важную роль также в повреждении экстрагиппокампальных отделов мозга.Таким образом, гиппокамп является одной из главных структур в дезинтеграции нейрональной комбинаторики, а включение тех или иных областей мозга обусловливает особенности клинических проявлений пароксизмальных состояний при этих двух заболеваниях., Мета. Метою нашого дослідження було порівняння мікроструктурних і нейрометаболічних порушень мозку, а також нейропатофізіологічних особливостей у пацієнтів, які страждають від епілепсії і мігрені. Методи. Нами обстежено 60 пацієнтів iз мігренню віком 16–42 роки (середній вік становив 29,30 ± 0,52 року) і 60 — з епілепсією віком 18–51 рік (середній вік 28,20 ± 0,98 року). Шістдесяти пацієнтам iз мігренню і 60 — з епілепсією проведена дифузійна тензорна магнітно-резонансна томографія. Протонна магнітно-резонансна спектроскопія виконана 18 пацієнтам iз мігренню і 28 — з епілепсією. Усі пацієнти обстежені з використанням комп’ютерної електроенцефалографії. Діагноз мігрені і епілепсії був виставлений згідно з міжнародними рекомендаціями. Результати. Отримані дані не тільки вказують на пошкодження нейрональної тканини у пацієнтів iз мігренню і епілепсією, а й, можливо, не виключають зворотний зв’язок у цій ієрархії подій і ймовірність деаферентації, обумовленої втратою гіпокампальних зв’язків. Ця обставина підтверджується зменшенням трактів у лімбічних зонах, що відіграє важливу роль також у пошкодженні екстрагіпокампальних відділів мозку.Таким чином, гіпокамп є однією з головних структур у дезінтеграції нейрональної комбінаторики, а включення тих чи інших областей мозку обумовлює особливості клінічних проявів пароксизмальних станів при цих двох захворюваннях.

Интенсивная терапия огнестрельных и взрывных ранений является актуальной проблемой военно-полевой хирургии и хирургии повреждений. Наличие при тяжелой огнестрельной травме синдрома микроциркуляторных нарушений с исходом в гипоксию является показанием для включения в комплекс лечения гипербарической оксигенации. Гипербарическая оксигенация может быть начата только после выполнения неотложных противошоковых и хирургических мероприятий.Цель исследования: изучение возможностей гипербарической оксигенации в комплексе послеоперационной интенсивной терапии огнестрельных и взрывных ранений. В отделении интенсивной терапии гипербарической оксигенации пролечено 56 пострадавших: абдоминальные ранения — 12, ранения грудной клетки — 5, осколочные ранения верхних и нижних конечностей — 39. Курс гипербарической оксигенации начинали через 3–20 часов после хирургической коррекции, он составлял 5–15 сеансов на режимах 1,5–2 АТА с экспозицией 40–60 мин. Выявлены особенности эффективности гипербарической оксигенации в зависимости от вида травмы., Інтенсивна терапія вогнепальних і вибухових поранень є актуальною проблемою військово-польової хірургії та хірургії ушкоджень. Наявність при тяжкій вогнепальній травмі синдрому мікроциркуляторних порушень з виходом у гіпоксію є показанням для включення в комплекс лікування гіпербаричної оксигенації. Гіпербарична оксигенація може бути почата тільки після виконання невідкладних протишокових і хірургічних заходів.Мета дослідження: вивчення можливостей гіпербаричної оксигенації в комплексі післяопераційної інтенсивної терапії вогнепальних і вибухових поранень.У відділенні інтенсивної терапії гіпербаричної оксигенації проліковано 56 постраждалих: абдомінальні поранення — 12, поранення грудної клітки — 5, осколкові поранення верхніх і нижніх кінцівок — 39. Курс гіпербаричної оксигенації починали через 3–20 годин після хірургічної корекції, він становив 5–15 сеансів на режимах 1,5–2 АТА з експозицією 40–60 хв. Виявлено особливості ефективності гіпербаричної оксигенації залежно від виду травми., Intensive care of gunshot wounds and blast injuries is an urgent problem of military surgery and surgery of injuries. Presence of microcirculatory disturbance syndrome with outcome to hypoxia in a severe gunshot wound is an indication to include hyperbaric oxygenation to treatment course. Hyperbaric oxygenation can be started only after completing urgent antishock and surgical interventions.Research objective: to study capabilities of hyperbaric oxygenation as a part of postoperative intensive care for gunshot wounds and blast injuries.56 wounded persons were treated in intensive care and hyperbaric oxygenation unit: with abdominal wounds — 12, chest wounds — 5, shrapnel wounds of upper and lower limbs — 39. Hyperbaric oxygenation regimen was started in 3–20 hours after surgical correction, it comprised 5–15 sessions in 1.5–2 ATA modes with 40–60 min exposures. There were identified features of hyperbaric oxygenation efficiency depending on type of wounds.

Запропонована формалізація процесу розробки діагностичного забезпечення ремонту техніки зв’язку з різним ступенем пошкодження і реалізацією видів групового пошуку дефектів., Предложена формализация процесса разработки диагностического обеспечения ремонта техники связи с разной степенью повреждения и реализацией видов группового поиска дефектов., Formalization of the process of development of the diagnostics support of telecommunications equipment repair with different defects level and realization of the of group defects search types is suggested.

BACKGROUND: Unruptured saccular intracranial aneurysm (SIA) is associated with an increased prevalence of migraine, but it is unclear whether this is altered by clipping of the aneurysm. The aim of our study was to determine whether remission rate of migraine and other recurrent headaches was greater in patients with SIA after clipping than in controls.METHODS: We prospectively studied 87 SIA patients with migraine or other recurrent headaches. They were interviewed about headaches in the preceding year before and 1 year after clipping using a validated semi-structured neurologist conducted interview. The remission rates of migraine and tension-type headache (TTH) in these patients were compared to 92 patients from a headache center. Diagnoses were made according to the ICHD-2.RESULTS: During 1 year preceding rupture 51 patients with SIA had migraine. During the year after clipping, this was reduced by 74.5% (P < 0.0001). At first encounter, 47 control patients had migraine during the preceding year, and during 1 year of treatment, it was 41, a reduction 12.8% (P > 0.5). The decrease of migraine in SIA patients was significantly higher than in controls: 74.5% vs 12.8% (P < 0.001). A history of TTH was given by 33 patients with SIA during the year preceding rupture and by 44 during 1 year after clipping (P > 0.75). Forty-one control patients had TTH, 27 after 1 year of treatment, a reduction 34.1% (P < 0.05). No factors except clipping of the aneurysm could explain the remission of migraine.CONCLUSIONS: Migraine prevalence in patients with SIA decreases significantly after clipping. Further comparative studies of migraine after coiling vs clipping in SIA patients are needed.

For asymptotically hyperbolic manifolds of dimension n with scalar curvature at least equal to -n(n - 1) the conjectured positive mass theorem states that the mass is non-negative, and vanishes only if the manifold is isometric to hyperbolic space. In this paper we study asymptotically hyperbolic manifolds which are also conformally hyperbolic outside a ball of fixed radius, and for which the positive mass theorem holds. For such manifolds we show that the conformal factor tends to one as the mass tends to zero., QC 20140205. Updated from manuscript to article in journal.

Эпилепсия является достаточно распространенным заболеванием, находящимся на стыке неврологии и психиатрии. Разработка и применение новых противоэпилептических средств позволили не только улучшить качество жизни пациентов благодаря контролю над приступами, но и повлиять на их социальное функционирование. Однако важной проблемой остаются те или иные психопатологические расстройства, особенно при многолетнем течении болезни. Они могут проявляться в форме аффективных симптомов, поведенческих реакций, транзиторных продуктивных психопатологических расстройств, а в ряде случаев — в виде дефицитарных нарушений, включая деменцию.

In this paper, we study asymptotically hyperbolic manifolds given as graphs of asymptotically constant functions over hyperbolic space H-n. The graphs are considered as unbounded hypersurfaces of Hn+1 which carry the induced metric and have an interior boundary. For such manifolds, the scalar curvature appears in the divergence of a 1-form involving the integrand for the asymptotically hyperbolic mass. Integrating this divergence, we estimate the mass by an integral over the inner boundary. In case the inner boundary satisfies a convexity condition, this can in turn be estimated in terms of the area of the inner boundary. The resulting estimates are similar to the conjectured Penrose inequality for asymptotically hyperbolic manifolds. The work presented here is inspired by Lam's article (The graph cases of the Riemannian positive mass and Penrose inequalities in all dimensions. http://arxiv.org/abs/1010.4256, 2010) concerning the asymptotically Euclidean case. Using ideas developed by Huang and Wu (The equality case of the penrose inequality for asymptotically flat graphs. http://arxiv.org/abs/1205.2061, 2012), we can in certain cases prove that equality is only attained for the anti-de Sitter Schwarzschild metric., QC 20130712