Titre :

Metabolic and immunological phenotype of rare lipomatoses: Dercum's disease and Roch-Leri mesosomatic lipomatosis.

Auteur(s) :

Lemaitre, Madleen [Auteur]
Evaluation des technologies de santé et des pratiques médicales - ULR 2694 [METRICS]
Service Endocrinologie, diabétologie, maladies métaboliques et nutrition [LILLE - Endocrino]
Chevalier, Benjamin [Auteur]
Service Endocrinologie, diabétologie, maladies métaboliques et nutrition [LILLE - Endocrino]
Jannin, Arnaud [Auteur]
Service Endocrinologie, diabétologie, maladies métaboliques et nutrition [LILLE - Endocrino]
Le Mapihan, Kristell [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Boury, Samuel [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Lion, Georges [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Labalette, Myriam [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Vantyghem, Marie-Christine [Auteur]
Recherche translationnelle sur le diabète (RTD) - U1190

Titre de la revue :

Orphanet Journal of Rare Diseases

Nom court de la revue :

Orphanet J Rare Dis

Numéro :

16

Pagination :

290

Date de publication :

2021-07-02

ISSN :

1750-1172

Mot(s)-clé(s) en anglais :

Lipodystrophy
Dercum's disease
Roch-Leri
Lipomatosis
Basophil
Natural killer
T lymphocyte
CD4 (cluster differentiation 4)
CD3
CD8

Discipline(s) HAL :

Sciences du Vivant [q-bio]

Résumé en anglais : [en]

Context Dercum’s disease (DD) and Roch-Leri mesosomatic lipomatosis (LMS) are rare and poorly characterized diseases. The clinical presentation combines multiple lipomas, painful in DD in contrast with LMS, without ...
Lire la suite >Context Dercum’s disease (DD) and Roch-Leri mesosomatic lipomatosis (LMS) are rare and poorly characterized diseases. The clinical presentation combines multiple lipomas, painful in DD in contrast with LMS, without lipoatrophy. Objective To identify any specific metabolic and immune phenotype of DD and LMS. Design and patients This monocentric retrospective study included 46 patients: 9 DD, 11 LMS, 18 lean and 8 obese controls. Metabolic and immunohematological characteristics of each group were compared. Results The median age of the patients was similar in the 3 groups (31 years). The number of women, and of basophils, and CD3+, CD4+ and CD8+ T lymphocytes was significantly higher in the DD versus the LMS group, without any difference of the metabolic parameters. Weight, BMI, blood pressure, gamma-GT, leptin, fasting insulin and C-peptide levels, fat mass percentage, and intra/total abdominal fat ratio were significantly higher in each lipomatosis group compared with the lean group. Compared with the lean group, the DD group had significantly higher fasting blood glucose, LDL-cholesterol, platelets, leukocytes, basophils, and a lower NK cell count, whereas the LMS group had a significantly lower rate of CD3, CD4, and CD8 lymphocytes. Compared with the obese controls, basophils remained higher in DD and T lymphocytes subpopulations lower in LMS groups. Conclusion DD and LMS show a common background of obesity and metabolic phenotype, but a distinct immunohematological profile characterized by a higher number of basophils in DD patients, an inflammatory profile that could contribute to pain. T lymphocyte depletion was present in LMS. These findings could offer specific therapeutic opportunities, especially for painful DD.Lire moins >

Langue :

Anglais

Comité de lecture :

Oui

Audience :

Internationale

Vulgarisation :

Non

Établissement(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
• METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694
• Recherche translationnelle sur le diabète (RTD) - U1190

Date de dépôt :

2024-01-12T06:32:17Z
2024-02-28T14:12:19Z