scholarly journals Guidelines for management of hereditary angioedema: What is new? What is missing?

2022 ◽  
Vol 43 (1) ◽  
pp. 12-19 ◽  
Author(s):  
Cristine Radojicic
Keyword(s):  
Hereditary Angioedema ◽  
Treatment Guidelines ◽  
Task Force ◽  
Burden Of Illness ◽  
Continuous Assessment ◽  
Practice Parameters ◽  
Key Points ◽  
Dominant Disease ◽  
Subcutaneous Tissues ◽  
Evidence Based Guidelines

Background: Hereditary angioedema is an autosomal dominant disease that presents with recurrent episodic swelling of the submucosal and/or subcutaneous tissues of the cutaneous, gastrointestinal, and respiratory systems. Evaluation and treatment guidelines have been published nationally and internationally to aid the treating provider. Methods: A review of the most cited and most recent updated guidelines was undertaken to review key points and to explore real-world feasibility of incorporating them into clinical practice. The International World Allergy Organization/European Academy of Allergy and Clinical Immunology (WAO/EAACI) Guideline for the Management of Angioedema - The 2017 Revision and Update, and the consensus reports from the Hereditary Angioedema International Working Group, the Joint Task Force on Practice Parameters focused practice parameter update, and the most recently updated US HAEA Medical Advisory Board 2020 Guidelines for the Management of Hereditary Angioedema were reviewed and summarized. Results: Key points that have been consistent throughout the guidelines include recommendations for evaluation and classification of hereditary angioedema as well as evidence-based guidelines for treatment. Further attention is required on the evaluation and continuous assessment of the burden of illness and quality of life (QoL). Conclusion: The guidelines for management of hereditary angioedema provide a framework for the clinician. However, the physician-patient dialog with regard to the patient disease experience, which includes attack frequency, severity, and Qol, must be continually assessed.

Assessment on hereditary angioedema burden of illness in Brazil: A patient perspective

2019 ◽  
Vol 40 (3) ◽  
pp. 193-197 ◽  
Author(s):  
Anderson Abdon Barbosa ◽  
Raquel de Oliveira Martins ◽  
Renata Martins ◽  
Anete Sevciovic Grumach
Keyword(s):  
Hereditary Angioedema ◽  
Burden Of Illness ◽  
Family Members ◽  
The United States ◽  
Consent Form ◽  
South American ◽  
Similar Data ◽  
Autosomal Dominant Disorder ◽  
Life Threatening ◽  
Subcutaneous Tissues

Background: Hereditary angioedema (HAE) is an autosomal dominant disorder that affects ∼1 in 50,000 individuals. The disorder is characterized by spontaneous and gradual episodes of edema in subcutaneous tissues or mucosal membrane that may endanger the patient's life. Previous studies that concern HAE treatment and burden of illness in the United States and Europe suggest an improvement in the control of HAE. However, no similar data are available in South American patients. The purpose of the present study was to evaluate the burden of disease and compliance to therapy of patients with HAE in Brazil. Methods: We developed a structured questionnaire to assess the variables that influence the diagnosis and treatment of Brazilian patients with HAE. The questionnaire was evaluated by allergists with expertise in HAE and was reformatted before applying it. The consent form was included before survey access; approval from the ethic committee of Faculdade de Medicina do ABC (Santo André, São Paulo, Brazil) was ensured. The Brazilian association of patients with HAE distributed the survey by e-mail to associated patients all over the country, and responses were accepted within a period of 3 months after signing a consent form. Results: Ninety patients (67 women, 23 men; average age, 36.5 years) responded; the main findings were the following: 53 of 90 (64%) had no need of visiting emergency departments in the past 6 months; 71 of 90 (79%) reported life-threatening fear due to their diagnosis; 73 of 90 (83%) had other family members affected, but 54 of 73 of these affected relatives did not look for specific HAE treatment; and 26 of 90 (29%) reported death due to asphyxia or throat swelling in family members. Conclusion: Patients with HAE report understanding how severe their diagnosis represent, but they did not ponder how important their commitment to treatment may decrease the constant fear brought by the disease in its possible swelling crisis. Family data supported this conclusion.

scholarly journals Respiratory Syncytial Virus Immunoprophylaxis with Palivizumab: 12-Year Observational Study of Usage and Outcomes in Canada

2021 ◽  
Author(s):  
Ian Mitchell ◽  
Abby Li ◽  
Candice L. Bjornson ◽  
Krista L. Lanctot ◽  
Bosco A. Paes ◽  
...  
Keyword(s):  
Risk Factors ◽  
Respiratory Syncytial Virus ◽  
Burden Of Illness ◽  
Respiratory Illness ◽  
Smoking Exposure ◽  
Key Points ◽  
Rsv Infection ◽  
History Of ◽  
Syncytial Virus ◽  
Airway Anomalies

Objective This study aimed to evaluate palivizumab (PVZ) use, trends in indications, and outcomes of respiratory illness hospitalizations (RIH) and respiratory syncytial virus hospitalizations (RSVH). Study Design It involves a large, Canadian prospective (2005–2017) observational multicenter study of children at high risk for RSV infection. Results A total of 25,003 infants (56.3% male) were enrolled at 32 sites; 109,579 PVZ injections were administered. Indications included: prematurity (63.3%); “miscellaneous” (17.8%); hemodynamically significant congenital heart disease (10.5%); bronchopulmonary dysplasia/chronic lung disease (8.4%). The “miscellaneous” group increased over time (4.4% in 2005–2006 to 22.5% in 2016–2017) and included: trisomy 21, airway anomalies, pulmonary disorders, cystic fibrosis, neurological impairments, immunocompromised, cardiac aged >2 years, multiple conditions, and a residual “unclassified” group. Adherence measured by expected versus actual doses plus correct interdose interval was 64.7%. A total of 2,054 RIH occurred (6.9%); 198 (9.6%) required intubation. Three hundred thirty-seven hospitalized children were RSV-positive (overall RSVH 1.6%). Risk factors for RSVH included having siblings, attending daycare, family history of atopy, smoking exposure, and crowded household. Infants with 5 risk factors were 9.0 times (95% CI or confidence interval 4.4–18.2; p < 0.0005) more likely to have RSVH than infants without risk factors. Three adverse events occurred; none were fatal. Conclusion Results are relevant to both clinicians and decision-makers. We confirmed the safety of PVZ. Use of PVZ increased steadily for children with miscellaneous conditions and medical complexity. Medical and social factors pose a risk for severe RIH and RSVH with accompanying burden of illness. A vaccine that protects against RSV is urgently required. Key Points

scholarly journals Clinical Practice Guidelines for the Management of Patients with Histoplasmosis: 2007 Update by the Infectious Diseases Society of America

2007 ◽  
Vol 45 (7) ◽  
pp. 807-825 ◽  
Author(s):  
L. Joseph Wheat ◽  
Alison G. Freifeld ◽  
Martin B. Kleiman ◽  
John W. Baddley ◽  
David S. McKinsey ◽  
...  
Keyword(s):  
Infectious Diseases ◽  
Health Care Providers ◽  
Antifungal Agents ◽  
Treatment Guidelines ◽  
Immunosuppressive Treatment ◽  
Case Series ◽  
Previous Treatment ◽  
Care Providers ◽  
New Information ◽  
Evidence Based Guidelines

Abstract Evidence-based guidelines for the management of patients with histoplasmosis were prepared by an Expert Panel of the Infectious Diseases Society of America. These updated guidelines replace the previous treatment guidelines published in 2000 (Clin Infect Dis 2000; 30:688–95). The guidelines are intended for use by health care providers who care for patients who either have these infections or may be at risk for them. Since 2000, several new antifungal agents have become available, and clinical trials and case series have increased our understanding of the management of histoplasmosis. Advances in immunosuppressive treatment for inflammatory disorders have created new questions about the approach to prevention and treatment of histoplasmosis. New information, based on publications from the period 1999–2006, are incorporated into this guideline document. In addition, the panel added recommendations for management of histoplasmosis in children for those aspects that differ from aspects in adults.

HEREDITARY ANGIOEDEMA – A PROBLEM AT THE INTERSECTION OF IMMUNOLOGY AND ALLERGOLOGY: ANALYSIS OF LITERATURE DATA AND DESCRIPTION OF A SERIES OF 34 CASES

2021 ◽  
Vol 100 (2) ◽  
pp. 49-57
Author(s):  
M.N. Guseva ◽  
◽  
E.I. Zinina ◽  
E.N. Suspitsyn ◽  
M.M. Kostik ◽  
...  
Keyword(s):  
Respiratory Tract ◽  
Hereditary Angioedema ◽  
Positive Family History ◽  
Clinical Manifestations ◽  
Upper Respiratory Tract ◽  
Upper Airways ◽  
Peripheral Edema ◽  
Dominant Disease ◽  
Characteristic Features ◽  
Head Neck

Hereditary angioedema (HAE) is a rare autosomal dominant disease characterized by isolated recurrent angioedema (AE) of various localizations: skin, the submucosa in the gastrointestinale tract, the respiratory tract. The characteristic features of edema in HAE are the absence of itching, skin flushing, accompanying urticaria, as well as the absence of pronounced effect of systemic corticosteroid therapy, systemic antihistamines therapy and adrenaline. The aim of our study was to present a descriptive characteristics of the group of patients with HAE and to access efficacy and safety of treatment. Materials and methods: the retrospective multicenter continuous nonrandomized uncontrolled study included data on 34 patients: 19 men (56%) and 15 women (44%) with HAE. The diagnosis of HAE was based on clinical and laboratorial data, according to the clinical guidelines of the Russian Association of allergologists and clinical immunologists on HAE (2014). Results The age range of patients at the time of inclusion in the study was 5–82 years, median – 38,8 (20,6; 46,9) years. Median age of first HAE episode was 12,5 (5,8; 16,3) years, ranged from 2 to 40, 22/28 (79%) of the patients manifested before 18 years. HAE was diagnosed at the median age of 30 years (17,5; 44,8) years, in 14,0 (9,0; 25,0) years after onset of the clinical manifestations. Most of the observations described have a positive family history of HAE (91%), in total 9 families were described. In 67% of families the disease manifested itself in two generations and in 33% of families – in three generations. At the onset of HAE peripheral edema was in 29/31 (94%) of the patients, and 22/31 (71%) had edema confined to the distal extremities. 7/31 (29%) patients had peripheral edema combined with edema of other locations. Abdominal attacks were in 6/31 (19%), head, neck and upper airways edema were in 7/31 (23%). The frequency of swelling of head, neck, upper respiratory tract increased statistically significantly from 23% (7/31) to 35% (7/20, p=0,003). 24 patients have recieved Icatibant treatment. No deaths were recorded against the background of this treatment. Conclusions HAE is diagnosed with significant time delays. Icatibant treatment is safe and effective.

Practice Guidelines for the Prevention, Detection, and Management of Respiratory Depression Associated with Neuraxial Opioid Administration

2016 ◽  
Vol 124 (3) ◽  
pp. 535-552 ◽  
Keyword(s):  
Respiratory Depression ◽  
Regional Anesthesia ◽  
Practice Guidelines ◽  
Task Force ◽  
Digital Content ◽  
Pain Medicine ◽  
Practice Parameters ◽  
Opioid Administration ◽  
American Society ◽  
American Society Of Anesthesiologists

Abstract The American Society of Anesthesiologists Committee on Standards and Practice Parameters and the Task Force on Neuraxial Opioids and the American Society of Regional Anesthesia and Pain Medicine present an updated report of the Practice Guidelines for the Prevention, Detection, and Management of Respiratory Depression Associated with Neuraxial Opioid Administration. Supplemental Digital Content is available in the text.

Practice Guidelines for Obstetric Anesthesia

2016 ◽  
Vol 124 (2) ◽  
pp. 270-300 ◽  
Keyword(s):  
Practice Guidelines ◽  
Task Force ◽  
Obstetric Anesthesia ◽  
Supplemental Digital Content ◽  
Digital Content ◽  
Practice Parameters ◽  
American Society ◽  
American Society Of Anesthesiologists

Abstract The American Society of Anesthesiologists Committee on Standards and Practice Parameters and the Task Force on Obstetric Anesthesia and the Society for Obstetric Anesthesia and Perinatology present an updated report of the Practice Guidelines for Obstetric Anesthesia. Supplemental Digital Content is available in the text.

scholarly journals Hereditary angioedema caused by C1-esterase inhibitor deficiency: a case report with literature-based analysis

2019 ◽  
Vol 8 (2) ◽  
pp. 741
Author(s):  
Sufia Athar ◽  
Noureddine Korichi ◽  
Yousra Shehada Siam
Keyword(s):  
Case Report ◽  
Hereditary Angioedema ◽  
Fresh Frozen Plasma ◽  
Pregnancy And Childbirth ◽  
C1 Esterase Inhibitor ◽  
Close Observation ◽  
Fresh Frozen ◽  
Dominant Disease ◽  
Frozen Plasma ◽  
Esterase Inhibitor

Hereditary angioedema (HAE) caused by C1-esterase inhibitor deficiency is an autosomal-dominant disease caused by a mutation in the C1-inhibitor gene. It is a rare disease that is often worsened during pregnancy and childbirth. HAE, though uncommon but if untreated it may lead to maternal death.  The case report presents the successful management of a 24 years old, G2P1, with hereditary angioedema caused by C1-esterase inhibitor deficiency. This patient was managed with a multidisciplinary approach by an obstetrician, an immunologist, an anaesthesiologist and a pediatrician. She had an uneventful antenatal period, labor was induced. She had precipitate delivery and soon after delivery had a flare up of the disease. It was successfully managed with fresh frozen plasma and close observation. 

Practice Advisory on Anesthetic Care for Magnetic Resonance Imaging

2015 ◽  
Vol 122 (3) ◽  
pp. 495-520 ◽  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Task Force ◽  
Digital Content ◽  
Resonance Imaging ◽  
Anesthetic Care ◽  
Practice Parameters ◽  
Practice Advisory ◽  
American Society ◽  
American Society Of Anesthesiologists

Abstract The American Society of Anesthesiologists Committee on Standards and Practice Parameters and the Task Force on Anesthetic Care for Magnetic Resonance Imaging presents an updated report of the Practice Advisory on Anesthetic Care for Magnetic Resonance Imaging. Supplemental Digital Content is available in the text.

scholarly journals Chronic pancreatitis.Evidence based management guidelines of the Hungarian Pancreatic Study Group

2015 ◽  
Vol 156 (7) ◽  
pp. 262-288 ◽  
Author(s):  
Tamás Takács ◽  
László Czakó ◽  
Zsolt Dubravcsik ◽  
Gyula Farkas ◽  
Péter Hegyi ◽  
...  
Keyword(s):  
Chronic Pancreatitis ◽  
Treatment Guidelines ◽  
Task Force ◽  
Correct Diagnosis ◽  
Study Group ◽  
Evidence Based ◽  
International Guidelines ◽  
Basic Reference ◽  
Evidence Based Guideline

Chronic pancreatitis is an inflammatory disease associated with structural and functional damage of the pancreas. In most cases pain, maldigestion and weight loss are the leading sympthoms, which significantly worsen the quality of life. Correct diagnosis and differential diagnosis of chronic pancreatitis and treatment of these patients requires up-to-date and evidence based treatment guidelines. The Hungarian Pancreatic Study Group proposed to prepare an evidence based guideline based on the available international guidelines and evidence. The preparatory and consultation task force appointed by the Hungarian Pancreatic Study Group translated and complemented and/or modified the international guidelines if it was necessary. 123 relevant clinical questions in 11 topics were defined. Evidence was classified according to the UpToDate®grading system. The draft of the guidelines were presented and discussed at the consensus meeting in September 12, 2014. All clinial questions were accepted with total or strong agreement. The present guideline is the first evidence based chronic pancreatitis guideline in Hungary. This guideline provides very important and helpful data for tuition, everyday practice and proper financing of chronic pancreatitis. Therefore, the authors believe that these guidelines will widely become a basic reference in Hungary. Orv. Hetil., 2015, 156(7), 262–288.

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