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Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins)

Primary lymphedema is a rare chronic pathology associated with constitutional abnormalities of the lymphatic system. The objective of this French National Diagnosis and Care Protocol (Protocole National de Diagnostic et de Soins; PNDS), based on a critical literature review and multidisciplinary exp...

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Autores principales: Vignes, Stéphane, Albuisson, Juliette, Champion, Laurence, Constans, Joël, Tauveron, Valérie, Malloizel, Julie, Quéré, Isabelle, Simon, Laura, Arrault, Maria, Trévidic, Patrick, Azria, Philippe, Maruani, Annabel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7789008/
https://www.ncbi.nlm.nih.gov/pubmed/33407666
http://dx.doi.org/10.1186/s13023-020-01652-w
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author Vignes, Stéphane
Albuisson, Juliette
Champion, Laurence
Constans, Joël
Tauveron, Valérie
Malloizel, Julie
Quéré, Isabelle
Simon, Laura
Arrault, Maria
Trévidic, Patrick
Azria, Philippe
Maruani, Annabel
author_facet Vignes, Stéphane
Albuisson, Juliette
Champion, Laurence
Constans, Joël
Tauveron, Valérie
Malloizel, Julie
Quéré, Isabelle
Simon, Laura
Arrault, Maria
Trévidic, Patrick
Azria, Philippe
Maruani, Annabel
author_sort Vignes, Stéphane
collection PubMed
description Primary lymphedema is a rare chronic pathology associated with constitutional abnormalities of the lymphatic system. The objective of this French National Diagnosis and Care Protocol (Protocole National de Diagnostic et de Soins; PNDS), based on a critical literature review and multidisciplinary expert consensus, is to provide health professionals with an explanation of the optimal management and care of patients with primary lymphedema. This PNDS, written by consultants at the French National Referral Center for Primary Lymphedema, was published in 2019 (https://has-sante.fr/upload/docs/application/pdf/2019-02/pnds_lymphoedeme_primaire_final_has.pdf). Primary lymphedema can be isolated or syndromic (whose manifestations are more complex with a group of symptoms) and mainly affects the lower limbs, or, much more rarely, upper limbs or external genitalia. Women are more frequently affected than men, preferentially young. The diagnosis is clinical, associating mild or non-pitting edema and skin thickening, as confirmed by the Stemmer’s sign (impossibility to pinch the skin on the dorsal side or the base of the second toe), which is pathognomonic of lymphedema. Limb lymphoscintigraphy is useful to confirm the diagnosis. Other causes of swelling or edema of the lower limbs must be ruled out, such as lipedema. The main acute lymphedema complication is cellulitis (erysipelas). Functional and psychological repercussions can be major, deteriorating the patient’s quality of life. Treatment aims to prevent those complications, reduce the volume with low-stretch bandages, then stabilize it over the long term by exercises and wearing a compression garment. Patient education (or parents of a child) is essential to improve observance.
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spelling pubmed-77890082021-01-07 Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins) Vignes, Stéphane Albuisson, Juliette Champion, Laurence Constans, Joël Tauveron, Valérie Malloizel, Julie Quéré, Isabelle Simon, Laura Arrault, Maria Trévidic, Patrick Azria, Philippe Maruani, Annabel Orphanet J Rare Dis Position Statement Primary lymphedema is a rare chronic pathology associated with constitutional abnormalities of the lymphatic system. The objective of this French National Diagnosis and Care Protocol (Protocole National de Diagnostic et de Soins; PNDS), based on a critical literature review and multidisciplinary expert consensus, is to provide health professionals with an explanation of the optimal management and care of patients with primary lymphedema. This PNDS, written by consultants at the French National Referral Center for Primary Lymphedema, was published in 2019 (https://has-sante.fr/upload/docs/application/pdf/2019-02/pnds_lymphoedeme_primaire_final_has.pdf). Primary lymphedema can be isolated or syndromic (whose manifestations are more complex with a group of symptoms) and mainly affects the lower limbs, or, much more rarely, upper limbs or external genitalia. Women are more frequently affected than men, preferentially young. The diagnosis is clinical, associating mild or non-pitting edema and skin thickening, as confirmed by the Stemmer’s sign (impossibility to pinch the skin on the dorsal side or the base of the second toe), which is pathognomonic of lymphedema. Limb lymphoscintigraphy is useful to confirm the diagnosis. Other causes of swelling or edema of the lower limbs must be ruled out, such as lipedema. The main acute lymphedema complication is cellulitis (erysipelas). Functional and psychological repercussions can be major, deteriorating the patient’s quality of life. Treatment aims to prevent those complications, reduce the volume with low-stretch bandages, then stabilize it over the long term by exercises and wearing a compression garment. Patient education (or parents of a child) is essential to improve observance. BioMed Central 2021-01-06 /pmc/articles/PMC7789008/ /pubmed/33407666 http://dx.doi.org/10.1186/s13023-020-01652-w Text en © The Author(s) 2021 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Position Statement
Vignes, Stéphane
Albuisson, Juliette
Champion, Laurence
Constans, Joël
Tauveron, Valérie
Malloizel, Julie
Quéré, Isabelle
Simon, Laura
Arrault, Maria
Trévidic, Patrick
Azria, Philippe
Maruani, Annabel
Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins)
title Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins)
title_full Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins)
title_fullStr Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins)
title_full_unstemmed Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins)
title_short Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins)
title_sort primary lymphedema french national diagnosis and care protocol (pnds; protocole national de diagnostic et de soins)
topic Position Statement
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7789008/
https://www.ncbi.nlm.nih.gov/pubmed/33407666
http://dx.doi.org/10.1186/s13023-020-01652-w
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