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Common Variable Immunodeficiency Associated With Noninfectious Pulmonary Complications and Its Treatment: Beyond Immunoglobulin Therapy.
Pulm Circ
January 2025
Division of Pulmonary Medicine, Henry Ford Hospital Detroit Michigan USA.
Common variable immunodeficiency (CVID) is a type of primary immunodeficiency that presents as a heterogenous disorder characterized by hypogammaglobinemia, poor response to vaccines, recurrent sinopulmonary infections, and can have noninfectious systemic manifestations. We performed a single-center, retrospective, observational study of five patients with noninfectious complications of CVID. All patients had CVID as defined by the European Society of Immunodeficiencies criteria and had received intravenous immunoglobulin therapy.
View Article and Find Full Text PDF[Clinical variables and management of disseminated granuloma annulare - monocentric retrospective analysis of 33 cases between 2021 and 2023].
Dermatologie (Heidelb)
January 2025
Klinik und Poliklinik für Dermatologie und Allergologie, Universitätsklinikum Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Deutschland.
Article Synopsis
- Granuloma annulare (GA) is a non-infectious skin condition that can become widespread in some patients; this study focused on those with disseminated GA in a German hospital.
- A total of 239 patients were studied, with 33 having confirmed disseminated GA, predominantly affecting women around the age of 57, and many reporting little to no symptoms.
- Treatment options included glucocorticoids and phototherapy, but only a small percentage achieved remission, highlighting the need for more effective clinical trials for new therapies.
Patterns and Outcomes of Pediatric Uveitis in a University-Based Tertiary Referral Center in Riyadh, Saudi Arabia.
Ocul Immunol Inflamm
December 2024
Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Article Synopsis
- The study aimed to analyze the patterns and outcomes of uveitis in children through a retrospective patient review.
- A total of 184 pediatric patients were examined, revealing that panuveitis was the most common type, with a significant number displaying non-granulomatous and non-infectious uveitis.
- After one year, most patients (85.4%) had improved vision, achieving at least 20/40 visual acuity, and Vogt-Koyanagi-Harada disease was identified as the most prevalent specific diagnosis.
Two distinct clinical progressions of P67phox-deficient CGD, both commencing with cervical lymphadenitis.
Ital J Pediatr
November 2024
Respiratory Department II, National Clinical Research Center for Respiratory Diseases, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, NO.56, Nanlishi Road, 100045, China.
We present two rare cases of p67phox-deficient chronic granulomatous disease (CGD) caused by compound heterozygous mutations in the NCF2 gene. They developed cervical lymphadenitis as the initial manifestation of CGD but had distinct clinical progressions. Patient 1 presented with aspergillous meningitis, an extremely rare manifestation of neurological involvement in CGD, which has not been reported before.
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