scholarly journals Hereditary angioedema. Clinical guidelines. (D84.1)

2021 ◽  
Vol 18 (2) ◽  
pp. 77-114
Author(s):  
Elena A. Bliznetz ◽  
Ekaterina A. Viktorova ◽  
Elena A. Vishneva ◽  
Eliso M. Dzhobava ◽  
Natalya I. Il'ina ◽  
...  

Hereditary angioedema is a rare, potentially life-threatening genetic disease that manifests with skin and mucous/submucosal swelling that occurs under the influence of bradykinin. The clinical guidelines for hereditary angioedema (code for the International Statistical Classification of Diseases and Related Health Problems D84.1) were developed in December 2020 by the Russian Association of Allergology and Clinical Immunology, Association of Medical Geneticists, National Association of Experts in the Field of Primary Immunodeficiencies, Union of Pediatricians of Russia. In December 2020, the project was approved at a meeting of the Scientific and Practical Council of the Ministry of Health of the Russian Federation (December 10, 2020 No. 743/12). The clinical guidelines are devoted to optimizing the clinical care of patients with hereditary angioedema. The topic is relevant owing to the under-diagnosis of hereditary angioedema and insufficient awareness of doctors of various specialties about this problem, as well as the difficulties in managing this category of patients. The clinical guidelines contain information about the epidemiology, etiology, classification, pathogenesis, and common clinical manifestations of hereditary angioedema. Thus, the paper described actual diagnostic approaches and differential diagnosis of hereditary angioedema and established its diagnostic criteria. Medical care algorithms to patients with hereditary angioedema at the prehospital and hospital stages are also described. The principles of pharmacotherapy, including long-term prophylaxis, short-term prophylaxis, and acute therapy, for hereditary angioedema are also presented. Treatment regimens are stratified according to patients age and gender. Non-drug measures for the prevention hereditary angioedema attacks are considered. Sections were also added in the edition of the new clinical guidelines of hereditary angioedema. Clinical guidelines for hereditary angioedema are intended for practicing doctors of all specialties, students and teachers of medical universities, residents, graduate students of medical universities.

2021 ◽  
Vol 18 (1) ◽  
pp. 56-72
Author(s):  
Natalya G. Astafieva ◽  
Ainagul Zh. Bayalieva ◽  
Igor B. Zabolotskikh ◽  
Natalya I. Ilyina ◽  
Alexander V. Kulikov ◽  
...  

Anaphylactic shock is a severe life-threatening condition caused by medicines, food, hymenoptera, animal poisons, etc. Therefore, anaphylactic shock (anaphylaxis) can develop both in medical institution and outside it, and physicians of any specialties should be ready to treat this condition. In this regard, clinical guidelines for anaphylactic shock have been developed. These recommendations were approved by the Russian Association of Allergy and Clinical Immunology (Protocol No. 743/12 dated December 30, 2020) and the Federation of Anesthesiologists and Resuscitators (Protocol No. 1 dated January 13, 2021), approved at a meeting of the Scientific and Practical Council of the Ministry of Health of the Russian Federation (Protocol No. 743/12 dated December 10, 2020). Guidelines covered a number of nosologies corresponding to codes of the International Statistical Classification of Diseases and Related Health Problems: T 78.0; T 78.2; T 80.5; T 88.6. The clinical guidelines systematize modern data on etiology, epidemiology, classification, clinical picture, diagnosis, treatment, and prevention of anaphylactic shock. In addition, an algorithm for managing a patient with anaphylactic shock has been developed. Clinical guidelines for anaphylactic shock are intended for practitioners of all specialties, students, and teachers of medical universities, residents, and graduate students.


Author(s):  
Kimberly Chow ◽  
Lauren Koranteng

Constipation, diarrhea, and bowel obstruction are three common and distressing symptoms in palliative care patients. These symptoms rarely occur without other clinical manifestations of disease, and their management must be considered in light of comorbid disease. Constipation, diarrhea, and bowel obstruction affect a person’s quality of life, eligibility for certain therapies, and may even be life-threatening. This chapter discusses the advanced practice registered nurse (APRN)’s role in the assessment, prevention, and management of these common bowel symptoms. as well as the global issues surrounding management. The causes and pharmacological treatment of these three bowel symptoms are discussed and detailed medication treatment regimens are presented in tabular form.


2011 ◽  
Vol 25 (6) ◽  
pp. 379-382 ◽  
Author(s):  
Sandra C. Christiansen ◽  
Bruce L. Zuraw

Background Hereditary angioedema (HAE) patients suffering from laryngeal attacks in the United States faced severely limited treatment options until 2008. These potentially life-threatening episodes occur in over one-half of the patients affected by HAE during their lifetimes. Acute therapy had been relegated to supportive care, intubation, and consideration of fresh frozen plasma (FFP)–-the latter with the potential for actually accelerating the speed and severity of the swelling. Methods In this article we will review the recently approved and emerging HAE treatments that have evolved from the recognition that bradykinin generation is the fundamental abnormality leading to attacks of angioedema. Results Acute therapy for laryngeal attacks will be discussed including purified plasma–derived C1 inhibitor (C1INH), recombinant C1INH, an inhibitor of plasma kallikrein (ecallantide), and a B2 receptor antagonist (icatibant). Prophylactic care has also been transformed from a reliance on attenuated androgens with their attendant side effects to C1INH replacement. Conclusion The arrival of these novel therapies promises to transform the future management of HAE.


2020 ◽  
Vol 166 (6) ◽  
pp. 382-386 ◽  
Author(s):  
H J Sawford ◽  
M B Smith

IntroductionThis paper presents the burden of mental health cases throughout UK military exercise SAIF SAREEA 3 (SS3), a low-tempo armoured brigade exercise in Oman from June to November 2018, and aims to discuss ways that mental health may be better managed on future large exercises.MethodsA retrospective review of all attendances at army medical facilities and relevant computerised medical records was undertaken.Results14 mental health cases were identified, which required 51 follow-up presentations throughout the duration of SS3. This represented 1.2% of all first patient presentations, and 6.3% of all follow-up work. 64% had diagnoses which predated deployment and could all be classified within 10th revision of International Statistical Classification of Diseases and Related Health Problems as either F30–F39 mood (affective) disorders, or F40–F48 neurotic, stress-related and somatoform disorders; all new diagnoses made while deployed were adjustment disorders. The medical officer spent an average of 147 min total clinical care time per patient. Six patients were aeromedically evacuated (AE), which represented 26% of all AE cases from SS3.ConclusionsPresentations were low, but time consuming and with poor disposal outcomes. Most conditions predated the exercise, and could have been predicted to worsen through the deployment. Given the disproportionate burden that mental health cases afforded during SS3, future brigade-sized deployments should include deployed mental health professionals in order to offer evidence-based therapy which should lead to improved disposal outcomes and a reduced AE burden.


2015 ◽  
Vol 78 (3) ◽  
pp. 309-316
Author(s):  
Magdalena Pietrzyńska ◽  
Barbara Stawińska-Witoszyńska ◽  
Alicja Krzyżaniak ◽  
Magdalena Łańczak-Trzaskowska ◽  
Kamila Nowak

Abstract Patients aged above 60 represent a very diversified population group with respect to their health condition. This may result from multimorbidity. In the rehabilitation process of elderly patients it is especially crucial to identify not only the underlying diseases which constituted the grounds for referral to the rehabilitation clinic, but also the comorbidities that have to be taken into consideration while planning their rehabilitation. The aim of the present paper is the assessment of comorbidities in patients of the rehabilitation clinic. The study population included 1616 patients (447 man and 1169 women) treated at the rehabilitation clinic. The factors put through analysis were the age and gender of the patient, the main diagnosed (underlying) illness subject to rehabilitation treatment, as well as comorbidities. All diseases, both the underlying conditions and the comorbidities have been classified according to the International Statistical Classification of Diseases and Related Health Problems (10th revised edition). The main reasons for the treatment at the rehabilitation outpatient clinic were arthrosis of the spine, knee and hip joints, polyarthritis, osteoporosis, diseases of the central nervous system diseases and paralytic syndromes as consequences of strokes, hypertension or atherosclerosis, as well as post-traumatic conditions. The most frequent comorbidities occurring in patients of the rehabilitation clinic were cardiovascular diseases (irrespective of the age group and the underlying disease). In the age group of 60-64, the subsequent comorbidities were gastrointestinal and cancers, and in the age of 65-74, neoplasms were the most frequently occurring comorbidities. The performed analysis resulted in the following conclusions: The majority of patients diagnosed and treated at the rehabilitation centre suffered from irregularities in the muscular, articular and skeletal system or the connective tissue (those were mainly the arthrosis of the spine, hip and knee joints as well as osteoporosis). The most frequently observed comorbidities in patients of the rehabilitation clinic were cardiovascular and gastrointestinal diseases, as well as neoplasms.


2021 ◽  
Vol 13 (1) ◽  
pp. 13-20
Author(s):  
Yu. V. Lobzin ◽  
A. A. Vilnits ◽  
M. M. Kostik ◽  
M. K. Bekhtereva ◽  
A. N. Uskov ◽  
...  

Since March 2020, the first reports have appeared about the increasing, almost everywhere, number of children who have undergone a new coronovirus infection caused by SARS-Cov-2 with a symptom complex resembling the manifestations of Kawasaki disease. A special feature of the clinical manifestations of this syndrome, which is called “Pediatric multisystem inflammatory syndrome associated with COVID-19”, is the high incidence of life-threatening conditions caused by the sharp development of arterial hypotension against the background of cardiogenic or vasogenic shock.In St. Petersburg, since the end of November 2020, there has been a sharp surge in admissions of children to the ICU of various hospitals with the clinic of Pediatric multisystem inflammatory syndrome, who have laboratory confirmation of the transferred COVID-19.The purpose of this article is to attract the attention of doctors of various profiles, to combine efforts to study this pathology, to determine the criteria for verifying the diagnosis, optimal treatment regimens and dispensary monitoring of patients who have been ill.


2016 ◽  
Vol 6 (23) ◽  
pp. 133-138
Author(s):  
Alexis Vuzitas ◽  
Codrut Sarafoleanu

Abstract Hereditary angioedema (HAE) is a rare autosomal dominant disease consisting of recurrent angioedema attacks, varying in severity, possibly life-threatening and with frequent involvement of the head and neck areas. The patophysiology of HAE differs from histamine-mediated allergic angioedema. Three types of reduced quality or quantity in various complement or coagulation factors, leading to massive release of bradykinin, increase vascular permeability and produce capilary leakage. Clinical manifestations of HAE include swelling located predominantly in the head and neck area, hands, feet and urogenital area and abdominal pain caused by edema of the gastrointestinal tract mucosa. Diagnosis requires laboratory tests for complement components and genetic tests. A timely and correct diagnosis in the emergency room is of utmost importance, the medical treatment of HAE being substantially different from that of allergic angioedema. Although new therapies are available and in development, airway intervention and surgery are still life-saving procedures and the ENT surgeon is an important part of the multidisciplinary team managing an HAE attack.


Author(s):  
Carolin Szász-Janocha ◽  
Eva Vonderlin ◽  
Katajun Lindenberg

Zusammenfassung. Fragestellung: Das junge Störungsbild der Computerspiel- und Internetabhängigkeit hat in den vergangenen Jahren in der Forschung zunehmend an Aufmerksamkeit gewonnen. Durch die Aufnahme der „Gaming Disorder“ in die ICD-11 (International Statistical Classification of Diseases and Related Health Problems) wurde die Notwendigkeit von evidenzbasierten und wirksamen Interventionen avanciert. PROTECT+ ist ein kognitiv-verhaltenstherapeutisches Gruppentherapieprogramm für Jugendliche mit Symptomen der Computerspiel- und Internetabhängigkeit. Die vorliegende Studie zielt auf die Evaluation der mittelfristigen Effekte nach 4 Monaten ab. Methodik: N = 54 Patientinnen und Patienten im Alter von 9 bis 19 Jahren (M = 13.48; SD = 1.72) nahmen an der Frühinterventionsstudie zwischen April 2016 und Dezember 2017 in Heidelberg teil. Die Symptomschwere wurde zu Beginn, zum Abschluss der Gruppentherapie sowie nach 4 Monaten anhand von standardisierten Diagnostikinstrumenten erfasst. Ergebnisse: Mehrebenenanalysen zeigten eine signifikante Reduktion der Symptomschwere anhand der Computerspielabhängigkeitsskala (CSAS) nach 4 Monaten. Im Selbstbeurteilungsbogen zeigte sich ein kleiner Effekt (d = 0.35), im Elternurteil ein mittlerer Effekt (d = 0.77). Der Reliable Change Index, der anhand der Compulsive Internet Use Scale (CIUS) berechnet wurde, deutete auf eine starke Heterogenität im individuellen Symptomverlauf hin. Die Patientinnen und Patienten bewerteten das Programm zu beiden Follow-Up-Messzeitpunkten mit einer hohen Zufriedenheit. Schlussfolgerungen: Die vorliegende Arbeit stellt international eine der wenigen Studien dar, die eine Reduktion der Symptome von Computerspiel- und Internetabhängigkeit im Jugendalter über 4 Monate belegen konnte.


Author(s):  
Andrea Zanichelli ◽  
Henriette Farkas ◽  
Laurance Bouillet ◽  
Noemi Bara ◽  
Anastasios E. Germenis ◽  
...  

AbstractHereditary angioedema (HAE) is a rare condition, mostly due to genetic deficiency of complement C1 inhibitor (C1-INH). The rarity of HAE impedes extensive data collection and assessment of the impact of certain factors known to affect the course of this disabling and life-threatening disease. Establishing a global registry could assist to overcome such issues and provides valuable patient data from different countries. The HAE Global Registry is a disease-specific registry, with web-based electronic support, where data are provided by physicians and patients through a dedicated application. We collected data between January 1, 2018, and August 31, 2020. Data on 1297 patients from 29 centers in 5 European countries were collected. At least one attack was recorded for 497 patients during the study period. Overall, 1182 patients were diagnosed with HAE type 1 and 115 with type 2. At the time of database lock, 389 patients were taking long-term prophylactic medication, 217 of which were on danazol. Most recorded attacks affected the abdomen, were generally moderate in severity, and occurred in patients who were not on prophylactic treatment (70.6%, 6244/8848). The median duration of attacks was 780 min (IQR 290–1740) in patients on prophylactic medication and 780 min (IQR 300–1920) in patients not on continuous prophylactic medication. In conclusion, the establishment of a registry for C1-INH-HAE allowed collection of a large amount of data that may help to better understand the clinical characteristics of this disease. This information may enhance patient care and guide future therapeutic decisions.


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