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Common variable immune deficiency, central diabetes insipidus, and anemia
Central European Journal of Immunology ( IF 1.3 ) Pub Date : 2020-11-01 , DOI: 10.5114/ceji.2020.101267
Marta Baleva , Spaska Lesichkova , Nevena Gesheva , Snejina Mihailova , Vanya Gerova , Borislav Vladimirov , Plamen Penchev , Milena Nikolova-Vlahova , Elissaveta Naumova

Common variable immune deficiency (CVID) accounts for approximately 20% of all cases of primary immune deficiencies, and is characterized by low serum levels of IgG, IgA, and/or IgM. The diagnosis is usually made between 20 and 40 years of age, sometimes earlier. CVID patients are divided into two major groups based on complications observed: 1 group consists of patients with predominant infections, and 2 group includes patients with inflammatory and/or hematological complications, such as lymphadenopathy, splenomegaly, autoimmune cytopenia, enteropathy, and/or granulomatous conditions. The most prevalent gastrointestinal symptom is transitory or persistent diarrhea. Central diabetes insipidus (CDI) is a rare disease associated with decreased synthesis or release of antidiuretic hormone that leads to an excessive production of diluted urine (polyuria). Different factors can lead to the development of CDI, including autoantibodies to arginine vasopressin-producing cells. Celiac disease is an autoimmune condition affecting small intestine in genetically predisposed individuals, which can be associated with endocrinopathies. Here, we describe a patient with CVID, CDI, gluten-sensitive diarrhea, and anemia of combined type (thalassemia minor and B12-deficiency anemia).

中文翻译:

常见的可变免疫缺陷,中枢性尿崩症和贫血

普通可变免疫缺陷症(CVID)约占所有原发性免疫缺陷病例的20%,其特点是血清IgG,IgA和/或IgM水平较低。诊断通常在20至40岁之间,有时更早。根据观察到的并发症,CVID患者分为两大类:一类包括主要感染的患者,二类包括具有炎症和/或血液学并发症(例如淋巴结病,脾肿大,自身免疫性血细胞减少,肠病和/或肉芽肿)的患者条件。最普遍的胃肠道症状是暂时性或持续性腹泻。中枢尿崩症(CDI)是一种罕见的疾病,与抗利尿激素的合成或释放下降有关,导致尿液稀释产生过多(多尿)。不同的因素可以导致CDI的发展,包括对产生精氨酸加压素的细胞的自身抗体。乳糜泻是一种自身免疫疾病,会影响遗传易感个体中的小肠,这可能与内分泌病相关。在这里,我们描述了CVID,CDI,面筋敏感性腹泻和合并型贫血(轻度地中海贫血和B12缺乏性贫血)的患者。可能与内分泌病变有关。在这里,我们描述了CVID,CDI,面筋敏感性腹泻和合并型贫血(轻度地中海贫血和B12缺乏性贫血)的患者。可能与内分泌病变有关。在这里,我们描述了CVID,CDI,面筋敏感性腹泻和合并型贫血(轻度地中海贫血和B12缺乏性贫血)的患者。
更新日期:2020-12-01
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