Subcutaneous Immunoglobulin (SCIG) Therapy during Pregnancy in a Woman with Hyper-IgM (HIGM) Syndrome
نویسندگان
چکیده
منابع مشابه
Molecular analysis of a large cohort of patients with the hyper immunoglobulin M (IgM) syndrome.
The hyper immunoglobulin M (IgM) syndrome (HIGM), characterized by recurrent infections, low serum IgG and IgA, normal or elevated IgM, and defective class switch recombination and somatic hypermutation, is a heterogenous disorder with at least 5 distinct molecular defects, including mutations of the genes coding for the CD40 ligand (CD40L) and IKK-gamma (NEMO) genes, both X-linked; and mutatio...
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Background: Defects in B cell class switch recombination (CSR) are a heterogeneous and yet very uncommon group of disorders which all have a genetic basis uniformly leading to hyper IgM (HIgM) syndrome. Due to the rare frequency of these conditions, a very small number of case series have been conducted on the affected patients. Objective: To shed some light on the morbidity and mortality regar...
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Severe hyperbilirubinemia in a pregnant mother suffering from Crigler-Najjar syndrome type II is a threat to the unborn child and may result in brain injury. We report the case of a Gly71-->Arg/Tyr486-->Asp homozygous mother of East Asian descent, who was treated with phototherapy during embryogenesis and with phenobarbital during the rest of the pregnancy. This resulted in significantly reduce...
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Hyperimmunoglobulin M syndrome is a rare primary immunodeficiency disorder. We report a case of a 6-month-old boy who suffered from developmental delays, frequent respiratory tract infection, and unusual fungal and bacterial infection. X-linked hyperimmunoglobulin M syndrome was ultimately diagnosed with decreasing immunoglobulin-G, A, and E (immunoglobulin G = 51.3 mg/dL, immunoglobulin A = 8....
متن کاملBiased immunoglobulin G (IgG) subclass production in a case of hyper-IgM syndrome.
Hyper-immunoglobulin M (IgM) syndrome (HIGM) is a rare heterogeneous primary immune deficiency. We describe a patient with HIGM characterized by skewed production of serum IgG subclasses and normal somatic hypermutation. This case may represent a subgroup of HIGM type 4 that is characterized by a biased switching to the V-region proximal constant regions.
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ژورنال
عنوان ژورنال: Journal of Allergy and Clinical Immunology
سال: 2017
ISSN: 0091-6749
DOI: 10.1016/j.jaci.2016.12.705