The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update

Hereditary angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process. The goal of these recommendations and guideline is to help physicians and their patients in making rational decisions in the management of HAE with deficient C1 inhibitor (type 1) and HAE with dysfunctional C1 inhibitor (type 2), by providing guidance on common and important clinical issues, such as: (1) How should HAE be diagnosed? (2) When should HAE patients receive prophylactic on top of on-demand treatment and what treatments should be used? (3) What are the goals of treatment? (4) Should HAE management be different for special HAE patient groups such as children or pregnant/breast-feeding women? and (5) How should HAE patients monitor their disease activity, impact, and control? It is also the intention of this guideline to help establish global standards for the management of HAE and to encourage and facilitate the use of recommended diagnostics and therapies for all patients

The international WAO/EAACI guideline for the management of hereditary angioedema - The 2021 revision and update.

Hereditary Angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process. The goal of these recommendations and guideline is to help physicians and their patients in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2), by providing guidance on common and important clinical issues, such as: 1) How should HAE be diagnosed? 2) When should HAE patients receive prophylactic on top of on-demand treatment and what treatments should be used? 3) What are the goals of treatment? 4) Should HAE management be different for special HAE patient groups such as children or pregnant/breast feeding women? 5) How should HAE patients monitor their disease activity, impact, and control? It is also the intention of this guideline to help establish global standards for the management of HAE and to encourage and facilitate the use of recommended diagnostics and therapies for all patients

Laboratory X-ray tomography for metal additive manufacturing: Round robin test

This paper reports on the results of a round robin test conducted by ten X-ray micro computed tomography (micro-CT) laboratories with the same three selected titanium alloy (Ti6Al4V) laser powder bed fusion (L-PBF) test parts. These parts were a 10-mm cube, a 60-mm long and 40-mm high complex-shaped bracket, and a 15-mm diameter rod. Previously developed protocols for micro-CT analysis of these parts were provided to all participants, including suggested scanning parameters and image analysis steps. No further information on the samples were provided, and they were selected from a variety of parts from a previous different type of round robin study where various L-PBF laboratories provided identical parts for micro-CT analysis at one laboratory. In this new micro-CT round robin test which involves various micro-CT laboratories, parts from the previous work were selected such that each part had a different characteristic flaw type, and all laboratories involved in the study analyzed the same set of parts. The 10-mm cube contained subsurface pores just under its top surface (relative to build direction), and all participants could positively identify this. The complex bracket had contour pores around its outer vertical sides, and was warped with two arms deflected towards one another. Both of these features were positively identified by all participants. The 15-mm diameter rod had a layered stop/start flaw, which was also positively identified by all participants. Differences were found among participants for quantitative evaluations, ranging from no quantitative measurement made, to under and overestimation of the values in all analyses attempted. This round robin provides the opportunity to highlight typical causes of errors in micro-CT scanning and image analysis as applied to additively manufactured parts. Some workflow variations, sources of error and ways to increase the reproducibility of such analysis workflows are discussed. The ultimate aim of this work is to advance the efficient use of micro-CT facilities for process optimization and quality inspections for additively manufactured products. The results provide confidence in the use of laboratory micro-CT but also indicate the need for further development of standards, protocols and image analysis workflows for quantitative assessment, especially for direct and quantitative comparisons between different laboratories.status: Published onlin

Laboratory X-ray tomography for metal additive manufacturing: Round robin test

This paper reports on the results of a round robin test conducted by ten X-ray micro computed tomography (micro-CT) laboratories with the same three selected titanium alloy (Ti6Al4V) laser powder bed fusion (L-PBF) test parts. These parts were a 10-mm cube, a 60-mm long and 40-mm high complex-shaped bracket, and a 15-mm diameter rod. Previously developed protocols for micro-CT analysis of these parts were provided to all participants, including suggested scanning parameters and image analysis steps. No further information on the samples were provided, and they were selected from a variety of parts from a previous different type of round robin study where various L-PBF laboratories provided identical parts for micro-CT analysis at one laboratory. In this new micro-CT round robin test which involves various micro-CT laboratories, parts from the previous work were selected such that each part had a different characteristic flaw type, and all laboratories involved in the study analyzed the same set of parts. The 10-mm cube contained subsurface pores just under its top surface (relative to build direction), and all participants could positively identify this. The complex bracket had contour pores around its outer vertical sides, and was warped with two arms deflected towards one another. Both of these features were positively identified by all participants. The 15-mm diameter rod had a layered stop/start flaw, which was also positively identified by all participants. Differences were found among participants for quantitative evaluations, ranging from no quantitative measurement made, to under and overestimation of the values in all analyses attempted. This round robin provides the opportunity to highlight typical causes of errors in micro-CT scanning and image analysis as applied to additively manufactured parts. Some workflow variations, sources of error and ways to increase the reproducibility of such analysis workflows are discussed. The ultimate aim of this work is to advance the efficient use of micro-CT facilities for process optimization and quality inspections for additively manufactured products. The results provide confidence in the use of laboratory micro-CT but also indicate the need for further development of standards, protocols and image analysis workflows for quantitative assessment, especially for direct and quantitative comparisons between different laboratories

The international WAO/EAACI guideline for the management of hereditary angioedema - The 2021 revision and update.

Hereditary Angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process. The goal of these recommendations and guideline is to help physicians and their patients in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2), by providing guidance on common and important clinical issues, such as: 1) How should HAE be diagnosed? 2) When should HAE patients receive prophylactic on top of on-demand treatment and what treatments should be used? 3) What are the goals of treatment? 4) Should HAE management be different for special HAE patient groups such as children or pregnant/breast feeding women? 5) How should HAE patients monitor their disease activity, impact, and control? It is also the intention of this guideline to help establish global standards for the management of HAE and to encourage and facilitate the use of recommended diagnostics and therapies for all patients

The international WAO/EAACI guideline for the management of hereditary angioedema – The 2021 revision and update

© 2022 The Author(s)Hereditary Angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process. The goal of these recommendations and guideline is to help physicians and their patients in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2), by providing guidance on common and important clinical issues, such as: 1) How should HAE be diagnosed? 2) When should HAE patients receive prophylactic on top of on-demand treatment and what treatments should be used? 3) What are the goals of treatment? 4) Should HAE management be different for special HAE patient groups such as children or pregnant/breast feeding women? 5) How should HAE patients monitor their disease activity, impact, and control? It is also the intention of this guideline to help establish global standards for the management of HAE and to encourage and facilitate the use of recommended diagnostics and therapies for all patients.N

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update

Hereditary angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process. The goal of these recommendations and guideline is to help physicians and their patients in making rational decisions in the management of HAE with deficient C1 inhibitor (type 1) and HAE with dysfunctional C1 inhibitor (type 2), by providing guidance on common and important clinical issues, such as: (1) How should HAE be diagnosed? (2) When should HAE patients receive prophylactic on top of on-demand treatment and what treatments should be used? (3) What are the goals of treatment? (4) Should HAE management be different for special HAE patient groups such as children or pregnant/breast-feeding women? and (5) How should HAE patients monitor their disease activity, impact, and control? It is also the intention of this guideline to help establish global standards for the management of HAE and to encourage and facilitate the use of recommended diagnostics and therapies for all patients