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Bernard–Soulier Syndrome (BSS) & tuberculosis: A case report

Pediatric Respiratory Disease Research Centre, NRITLD, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Bernard–Soulier Syndrome (BSS) is a rare congenital bleeding disorder characterized by thrombocytopenia with giant platelets [1,2]. This syndrome is caused by a quantitative or qualitative defect in the receptor complex of 1b/1X glycoprotein-located

Extra-pulmonary Pneumocystis jiroveci infection: a case report

Pediatric Respiratory Disease Research Centre, NRITLD, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
IntroductionA 46-year old man complaining of dyspnea and abdominalpain since 10 days ago that exacerbated in last 4 days ispresented. He mentioned prolonged fever of 39◦C, 0.5–1 heach night during the last 6 months. The patient was

IgG anti-IgA antibodies in paediatric antibody-deficient patients receiving intravenous immunoglobulin

Pediatric Respiratory Disease Research Centre, NRITLD, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Predominantly antibody deficiencies (PAD) are the most common types of primary immunodeficiency disorders. Patients with PAD have increased proneness to recurrent infections and consequent morbidity and mortality due to defect in humoral immunity.

Bronchoalveolar galactomannan in invasive pulmonary aspergillosis: a prospective study in pediatric patients

Pediatric Respiratory Disease Research Centre, NRITLD, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Invasive pulmonary aspergillosis (IPA) represents frequent and serious complications in immunocompromised patients [1]. Children with hematological malignancies, solid organ or hematopoietic stem cell transplantation, severe congenital

Human intracellular ISG15 prevents interferon-α/β over-amplification and auto-inflammation

Pediatric Respiratory Disease Research Centre, NRITLD, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Calcification of the cerebral basal ganglia during childhood is an important radiological sign associated with a range of genetic and non-genetic states12,13. In Fahr’s disease, also known as idiopathic basal ganglia calcification

Human TYK2 deficiency: Mycobacterial and viral infections without hyper-IgE syndrome

Pediatric Respiratory Disease Research Centre, NRITLD, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
The first TYK2-deficient patient (P1) to be identified was reported in 2006 (Minegishi et al., 2006; Casanova et al., 2012). This patient was Japanese and displayed the triad of signs characteristic of hyper-IgE syndrome (HIES): atopic dermatitis

Monogenic mutations differentially affect the quantity and quality of T follicular helper cells in patients with human primary immunodeficiencies

Pediatric Respiratory Disease Research Centre, NRITLD, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Naive CD41 T cells differentiate into distinct populations of effector cells with specialized functions. Such fine tuning ensures the generation of appropriate immune responses that efficiently clear pathogens and generate long-term protective

 

RAD50 Single-Nucleotide Polymorphism in Predominantly Antibody Deficiency

Pediatric Respiratory Disease Research Centre, NRITLD, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Common variable immunodeficiency (CVID) and selective IgA deficiency (SIgAD) constitute 2 important predominantly antibody deficiencies. Patients with CVID usually have reduced levels of at least 2 immunoglobulin isotypes, predisposing

 

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