scholarly journals Resolution on the results of the Council of Experts on the Long-term Prophylaxis of Hereditary Angioedema in Russia

Author(s):  
Elena Latysheva ◽  
Tatiana V. Latysheva ◽  
O S Bodnya ◽  
Darya V Demina ◽  
Natalia B. Kuzmenko ◽  
...  

Hereditary angioedema (HAE) is a rare (orphan) disease associated with the development of angioedema of various localization caused by the action of bradykinin. The main symptoms of the disease are peripheral angioedema, abdominal attacks (accompanied by severe pain syndrome), edema of the upper respiratory tract, which may lead to asphyxia and death of the patient. Peripheral edema disrupts social and professional activity, the occurrence of facial edema complicates social adaptation, the unpredictability and potential threat to life of edema in the larynx, as well as the lack of effect from standard (systemic glucocorticoid and antihistamines) therapy regimens lead to a low quality of life of patients, a large number of days of disability, hospitalizations, in some cases, premature death of patients. According to international studies, "on-demand" therapy does not reduce the burden of the disease, and therefore international guidelines emphasize the importance of timely and individualized selection of long-term prevention for patients with HAE. Taking into account the appearance of a new drug for long-term prevention in Russia, an Expert Council was held to assess the burden of the disease in Russian patients, determine the proportion of patients who need long-term prevention, criteria for choosing a medication for prophylaxis and the place of Lanadelumab in it.

2018 ◽  
Vol 20 (4) ◽  
pp. 26-31
Author(s):  
A A Myasnikov ◽  
E V Eficenko ◽  
D P Zverev ◽  
I R Klenkov

Clinical pattern of the chronic decompression sickness is non-specific and there is objective difficulty to reveal the cause-and-effect relationship between the diver’s health condition and his professional activity in each case. This problem is not only obstructing necessary medical and social aid to the patient but also provides the light-minded relation to the preventive measures organization before diving long-term adverse health effects. A clinical case of professional pathology diagnosed in the diver. The patient served in 1993-2000 in Armed Forces of the Russian Federation as diver-welder, during this period no active complains were demonstrated, as he told, for saving diver’s qualification. In 2000 the patient retired for social and economic reasons and was accepted as a healthy person by a military medical expert commission. Until 2007 he was working by speciality not related to increased pressure terms. In 2007 after medical expert commission examination, the patient continued his work as a civilian personnel diver of the Russian Federation Ministry of Defense, during this time the clear relation between pain syndrome and diving episodes was detected. The patient was self-treated with analgesics and heat physical therapy with unstable effect. In 2014 because of increased lower back pain syndrome, he turned to medical aid. In2015 the diagnosis of chronic decompression sickness was established. Pathogenesis of chronic decompression sickness in not enough investigated, including methodical reasons. Prophylactic measures existing in routine medical diving practice are aimed only for acute non-specific and specific diver’s pathologies and this fact doesn’t favour special attention of the medicals for long-term post-diving effects. It provides to the low efficiency of measures for saving divers, aquanauts and caisson workers professional suitability despite known cases of their health disorders during work experience.


Author(s):  
Anton Yarikov ◽  
Maxim Shpagin ◽  
Iliya Nazmeev ◽  
Sergey Gorelov ◽  
Olga Perlmutter

The immediate and long-term results of treatment of 30 patients with severe pain syndrome of the lumbar region, who underwent operations on denervation of DOS, were studied. The aim of the study was to evaluate the effectiveness of minimally invasive technologies for the treatment of pain in the lumbar region (denervation of DOS), to study the near and distant results of these treatment methods. Denervation DOS is an effective minimally invasive method for the treatment of facet syndrome caused by spondylarthrosis. It allows in the early and distant postoperative periods to significantly reduce the pain syndrome and improve the quality of life of patients.


2021 ◽  
Vol 11 (4) ◽  
Author(s):  
Henriette Farkas ◽  
Marcin Stobiecki ◽  
Jonny Peter ◽  
Tamar Kinaciyan ◽  
Marcus Maurer ◽  
...  

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Konrad Bork ◽  
John T. Anderson ◽  
Teresa Caballero ◽  
Timothy Craig ◽  
Douglas T. Johnston ◽  
...  

Abstract Background Hereditary angioedema (HAE) is a rare disease characterized by unpredictable, potentially life-threatening attacks, resulting in significant physical and emotional burdens for patients and families. To optimize care for patients with HAE, an individualized management plan should be considered in partnership with the physician, requiring comprehensive assessment of the patient’s frequency and severity of attacks, disease burden, and therapeutic control. Although several guidelines and consensus papers have been published concerning the diagnosis and treatment of HAE, there has been limited specific clinical guidance on the assessment of disease burden and quality of life (QoL) in this patient population. Practical guidance is critical in supporting effective long-term clinical management of HAE and improving patient outcomes. The objective of this review is to provide evidence-based guidelines for an individualized assessment of disease burden and QoL in patients with HAE. Methods A consensus meeting was held on February 29, 2020, consisting of 9 HAE experts from the United States and Europe with extensive clinical experience in the treatment of HAE. Consensus statements were developed based on a preliminary literature review and discussions from the consensus meeting. Results Final statements reflect the consensus of the expert panel and include the assessment of attack severity, evaluation of disease burden, and long-term clinical management of HAE caused by C1-esterase inhibitor deficiency. Patient-reported outcome measures for assessing HAE attack severity and frequency are available and valuable tools; however, attack frequency and severity are insufficient markers of disease severity unless they are evaluated in the broader context of the effect on an individual patient’s QoL. QoL assessments should be individualized for each patient and minimally, they should address the interference of HAE with work, school, social, family, and physical activity, along with access to and burden of HAE treatment. Advances in HAE therapies offer the opportunity for comprehensive, individualized treatment plans, allowing patients to achieve minimal attack burden with reduced disease and treatment burden. Conclusion This consensus report builds on existing guidelines by expanding the assessment of disease burden and QoL measures for patients with HAE.


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