Category Ranking

98%

Total Visits

921

Avg Visit Duration

2 minutes

Citations

20

Article Abstract

This paper describes a rare case of Turner's syndrome associated with Juvenile Haemochromatosis and severe lymphopenia, followed-up for a period of 5 years. Because of the indication for treatment with growth hormone (GH), this case was observed as a model to analyse the effects of GH on growth, iron mobilization and lymphocyte reconstitution. For this purpose, a serial study of the T lymphocyte subpopulations CD4+, CD8+, CD8+ CD28+ and CD8+ CD28- was performed by immunophenotyping during the follow-up period. Besides the impact of both phlebotomy treatment and GH on the rapid growth and mobilization of 20.8 g of iron in 136 weeks, the most relevant observation was the finding of a significant expansion of CD8+ T lymphocytes expressing the costimulatory marker CD28 in the setting of the severe lymphopenia. These findings constitute new clinical evidence supporting the notion that the GH/IGF-1 system has an important role on the maintenance of T cell homeostasis in vivo, and that GH may be regarded as a putative therapeutic agent in T lymphocyte reconstitution.

Download full-text PDF

Source
http://dx.doi.org/10.1111/j.1365-2265.2004.02069.xDOI Listing

Publication Analysis

Top Keywords

severe lymphopenia
12
growth hormone
8
cd8+ cd28+
8
rare case
8
associated juvenile
8
juvenile haemochromatosis
8
turner's syndrome
8
lymphocyte reconstitution
8
cd8+
5
growth
4

Similar Publications

Mapping the infectious burden in VEXAS syndrome: a systematic review and rationale for prevention.

Lancet Rheumatol

September 2025

Service de Médecine interne et polyvalente, Centre Hospitalier du Haut-Anjou, Château-Gontier, France; Université d'Angers, Inserm, CNRS, MITOVASC, Equipe MitoLab, SFR ICAT, F-49000 Angers, France. Electronic address:

Infections are increasingly recognised as a major cause of morbidity and mortality in patients with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome. We conducted a systematic review to characterise the infectious burden of VEXAS syndrome and propose preventive strategies. We included 57 studies (813 patients) showing that infections in patients with VEXAS syndrome were frequent, severe in 40-60% of cases, and fatal in 6-15% of cases.

View Article and Find Full Text PDF

Serological clusters in systemic lupus erythematosus and its clinical and prognostic implications: A longitudinal cohort study.

Semin Arthritis Rheum

August 2025

Grupo IRIDIS (Investigation in Rheumatology and Immune-Diseases), Instituto de Investigación Sanitaria Galicia Sur, (IISGS), Hospital Universitario Vigo, Vigo, Spain.

Introduction: Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disorder characterized by multi-organ involvement and variable clinical manifestations. Recurrent clinical patterns suggest distinct phenotypes, where cluster analysis of autoantibodies could identify prognostic subtypes.

Objectives: To define and describe serological clusters and their clinical-epidemiological characteristics, as well as their association with comorbidities, disease activity measures, severity, and damage.

View Article and Find Full Text PDF

Introduction: Zeta-chain-associated protein kinase 70 () is a tyrosine kinase that plays a crucial role in T-cell activation via the T-cell receptor/CD3 complex and contributes to B-cell signaling. variants can cause a range of immunodeficiencies with variable clinical presentations, including infections and malignancies.

Case Presentation: A 4-year-old boy presented with chronic cough, dyspnea, recurrent chest infections, and failure to thrive.

View Article and Find Full Text PDF

Associations Between Lymphopenia and the Development/Recovery of Acute Kidney Injury Among Patients Hospitalized With Respiratory Virus Infections.

Kidney Med

September 2025

National Center for Respiratory Medicine; State Key Laboratory of Respiratory Health and Multimorbidity; National Clinical Research Center for Respiratory Diseases; Institute of Respiratory Medicine, Chinese Academy of Medical Sciences; Department of Clinical research and Data management, Center of

Rationale & Objective: Lymphopenia may have a potential mechanism on the development of acute kidney injury (AKI) after respiratory virus infection but has never been revealed. We aimed to investigate the relationship between lymphopenia and AKI in patients hospitalized with respiratory virus infections.

Study & Design: A single-center and retrospective cohort study.

View Article and Find Full Text PDF

Background: Blau Syndrome is a rare monogenic disorder characterized by granulomatous polyarthritis, dermatitis, and uveitis. The diagnosis can be challenging as symptoms may not always align with the classic triad.

Case Description: An 8-year-old girl presented with fluctuant swellings in the wrists and ankles, strength reduction and stiffness.

View Article and Find Full Text PDF