immunoglobulin levels
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2021 ◽  
Vol 12 ◽  
Author(s):  
Jessica Rojas-Restrepo ◽  
Andrés Caballero-Oteyza ◽  
Katrin Huebscher ◽  
Hanna Haberstroh ◽  
Manfred Fliegauf ◽  
...  

Predominantly antibody deficiencies (PAD) are a heterogeneous group of disorders characterized by dysfunctional antibody production, low immunoglobulin levels in serum and impaired vaccine responses. The clinical picture is variable, ranging from mild symptoms to severe complications, which may include autoimmunity, gastrointestinal disease, allergy, and malignancies. If left untreated, PAD patients are at risk of enduring disease progression, irreversible organ damage, and reduced life expectancy. A timely diagnosis has been shown to significantly improve disease prognosis. Here, we report on our experience using targeted gene panel sequencing by employing Agilent’s HaloPlex or SureSelect and Illumina’s MiSeq technologies in a cohort of 291 individuals who presented with low or absent immunoglobulin levels in combination with or without other clinical features. In total, we have detected over 57 novel or previously reported relevant mutations in ADA, ADA2, BTK, CTLA4, LRBA, NFKB1, NFKB2, PIK3CD, STAT3, and TNFRSF13B. Overall, a genetic diagnosis could be made in 24.7% of the investigated patients. The percentage of coverage for the targeted regions ranged from 90% to 98% in this study. Moreover, functional assays were performed on a defined group of the patients carrying candidate variants in CTLA4, LRBA, NFKB1 and BTK, which confirmed their deleterious effect on protein expression and/or function. This study reiterates that the immunological heterogeneity of predominantly antibody deficiencies may have a diverse genetic origin, although certain clinical features may hint towards a specific group of defects. Employing targeted sequencing panels proves to be a very time- and cost-efficient, yet reliable, method for the establishment of a genetic diagnosis in individuals with PAD. However, in case of negative panel results, or if functional testing reveals inconspicuous observations in patients with a clear indication for genetic testing, further work-up including whole exome or whole genome sequencing should be considered.


2021 ◽  
Vol 9 (3) ◽  
pp. 249-254
Author(s):  
Burçin BEKEN ◽  
Velat ÇELİK ◽  
Pınar GÖKMİRZA ÖZDEMİR ◽  
Mehtap YAZICIOĞLU

Metabolites ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 817
Author(s):  
So-Yun Jhang ◽  
Sung-Hyen Lee ◽  
Eun-Byeol Lee ◽  
Ji-Hye Choi ◽  
Sohyun Bang ◽  
...  

Platycodon grandiflorum (PG) is a perennial plant that has been used as a traditional remedy to control immune-related diseases. PG was steamed and dried to improve its taste (PGS). The aim of the study was to investigate the effects of PG and PGS (PG-diets) on the gut microbiome and immune system. We treated PG-diets to immunosuppressed mice via cyclophosphamide (CPA) injection. After two weeks of the supplement, we evaluated specific genera related to body weight and serum immunoglobulin levels and analyzed 16S rRNA sequencing and metagenomics statistical analysis. PG-diets groups showed an increased abundance of microorganisms in immunodeficient mice compared to the control group (NC). Moreover, Akkermansia significantly decreased in response to the CPA in the NC group at the genus level, whereas its abundance increased in the PG-diets groups. We also found that the modulation of the gut microbiome by PG-diets was correlated with body weight, IgA, and IgM levels. The results demonstrate that PG-diets may improve the health benefits of immunosuppressed mice by altering the gut microbiome, though not much difference was found between PG and PGS treatments. Finally, this is the first study showing the effects of PGS-diets on the gut microbiome and immune system as a potential nourishing immunity supplement.


2021 ◽  
Vol 67 (3) ◽  
pp. 125-128
Author(s):  
Cui Liu ◽  
Qian Luo ◽  
Yan Tang ◽  
Gaoxin Yu ◽  
Kuai Liang ◽  
...  

Nasal polyps are the most common benign nasal tumors that can lead to nasal obstruction and other annoying problems for the patient. Several hypotheses have been proposed as the basic mechanism of nasal polyps. In order to investigate one of the possible causes that can be a disorder in the regulation of systemic immune responses, the present study was designed to investigate the relationship between plasma cyclin-dependent kinase 5 (CDK5) levels and local immunoglobulin levels in patients with nasal polyps. A cross-section study was used to evaluate concentrations of local immunoglobulin levels (IgE, IgM, IgA, and IgG) on blood and polyp specimens from 60 patients with nasal polyps, and 60 control groups. Western Blot Analysis was done for CDK5 in plasma cells. IgA, IgG and IgE concentrations were significantly higher in polyp tissue specimens, but not in blood, of nasal polyp patients compared to the control group. Furthermore, plasma CDK5 levels were significantly higher in nasal polyp tissue compared with control. The difference in IgA, IgE and IgG expression between nasal polyp tissue and blood, supported by increased numbers of plasma cells, suggests a local production of these local immunoglobulins in nasal polyps in response to chronic antigens. Among local immunoglobulins, only there was a significant correlation between CDK5 with IgG (positive correlation) and IgE (negative correlation). The exact explanation for the relationship between plasma CDK5 and local immunoglobulins in nasal polyps needs further studies.


2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Jizhang Liu ◽  
Yuxia Zhong ◽  
Liangduan Ding ◽  
Ayinuer Tuluhong ◽  
Burebi Maihemuti ◽  
...  

Backgrounds. The pathogenesis of nephrotic syndrome (NS) is complex, and there are differences between regions. This study attempted to collect clinicopathological data of patients diagnosed with NS in Xinjiang and Heilongjiang in the past 2 years, so as to explore the onset features of NS and treatment and prognosis of patients in the two regions. Methods. Clinical data of 375 patients diagnosed with NS using renal biopsy in Xinjiang and Heilongjiang from March 2019 to March 2021 were collected. Clinical data of patients before treatment were collected, and the chi-square test was utilized to compare the differences in the sex distribution of two groups. The U test was utilized to compare abnormal distribution continuous data between two groups, such as age, hemoglobin, plasma albumin, proteinuria, and triglycerides. Independent sample t -test was utilized to compare normal distribution continuous data between two groups, such as serum total protein, serum creatinine, blood urea nitrogen, glomerular filtration rate, and total cholesterol. The independent sample t -test was also used to compare the immunoglobulin levels and complement levels between the two groups after treatment, including IgA, IgG, IgM, C3, and C4. Kaplan-Meier method was used to analyze and plot the cumulative curves of complete remission rate and partial remission rate. Results. For 275 NS patients from Xinjiang, the male-to-female ratio was 0.81 : 1. For 84 patients from Heilongjiang, the male-to-female ratio was 1.05 : 1. The onset ages of patients in Xinjiang and Heilongjiang were 22-45 years old and 22-47 years old, respectively. Respectively, there were 221 cases (80.36%) and 66 cases (78.57%) of primary NS in Xinjiang and Heilongjiang. There were 54 cases (19.64%) and 18 cases (21.43%) of secondary NS in Xinjiang and Heilongjiang, respectively. There was no statistically significant difference in cause distribution between the two regions ( p = 0.756 ). After treatment, immunoglobulin levels (IgA ( p = 0.009 ), IgG ( p = 0.002 ), IgM ( p < 0.001 )) and complement C3 ( p < 0.001 ) and C4 ( p < 0.001 ) levels in Xinjiang and Heilongjiang were statistically significant. 129 cases in Xinjiang (46.91%) and 55 cases in Heilongjiang (65.48%) were treated with glucocorticoid (GC) combined with immunosuppressive therapy, respectively. After receiving treatment, 67 (24.36%) of 275 patients in Xinjiang achieved complete remission, 166 (60.36%) achieved partial remission, 22 (26.19%) of 84 patients in Heilongjiang achieved complete remission, and 56 (66.67%) achieved partial remission, and there was no statistically significant difference in remission rate between the two regions ( p = 0.159 ). Patients in Xinjiang and Heilongjiang achieved complete remission at an average of 10.34 weeks (9.98-10.70) and 9.95 weeks (9.26-10.65), respectively. There was no significant difference in complete remission rates between the two regions ( p = 0.663 ). Patients in Xinjiang and Heilongjiang achieved partial remission at an average of 8.76 weeks (8.38-9.14) and 7.99 weeks (7.33-8.65), respectively. There was no significant difference in the partial remission rate between the two regions ( p = 0.065 ). Conclusion. The causes of NS in Xinjiang and Heilongjiang were similar. After treatment, there were differences in immunoglobulin levels (IgA, IgG, IgM) and complement levels (C3, C4) in the two regions. The main treatment methods used in the two regions were GC combined with immunosuppressive therapy. The prognosis of patients in the two regions was similar. The complete remission rate and partial remission rate after treatment in the two regions were similar, and the average time required to achieve complete remission and partial remission was also similar.


2021 ◽  
Vol 49 (6) ◽  
pp. 63-66
Author(s):  
Molood Safarirad ◽  
Ali Abbaszadeh Ganji ◽  
Farzad Nazari ◽  
Reza Yazdani ◽  
Hassan Abolhassani ◽  
...  

Background: The coronavirus disease 2019 (COVID-19) pandemic has affected millions of people around the world. This zoonotic-enveloped virus is primarily transmitted through inhalation. Infected people are commonly asymptomatic or manifest mild symptoms, including fever, cough, diarrhea, and fatigue. However, it may lead to severe patterns associated with multiple organ failure in individuals with an impaired immune system. Objective: Here we report a 7-year-old girl with hyper-immunoglobulin M (IgM) (HIgM) phenotype, admitted to the hospital emergency department with fever, cough, and pneumonia symptoms because of the COVID-19 infection. Coronavirus infection was confirmed by a positive real-time polymerase chain reaction test. Surprisingly, serum levels of both IgG and IgA of the patient were transiently normalized during the COVID-19 infection when tested prior to the monthly injection of intravenous immunoglobulin. After she recovered from the COVID infection, her immunoglobulin levels returned to the primary stage and she demonstrated HIgM phenotype. Conclusion: Since this transient increase in the levels of immunoglobulins was solely observed during the COVID-19 infection, and no other infectious episodes were diagnosed in the patient, clarifying the exact cause would help to understand in a better manner the implications and specification of humoral immunity in patients with primary antibody deficiencies.


2021 ◽  
Vol 7 (5) ◽  
pp. 3364-3373
Author(s):  
Teng Wang ◽  
Ping Zheng ◽  
Qian Jiang ◽  
Qianqian Sun

Mycoplasma pneumoniae pneumonia is a problem that has attracted the attention of children’s respiratory department in recent years. The relationship between vitamin A deficiency (VA) and the severity of Mycoplasma disease (MPP) in children was studied. In this paper, the hospitalized children with Mycoplasma pneumoniae pneumonia (MPP) in our hospital were selected as the research objects, and they were divided into common type and refractory type (RMPP) for comparative experiment. The VA and immunoglobulin levels of the two groups were compared. Attention should be paid to the normal ratio of VA deficiency (CVAD) and sub deficiency (svad) in the two groups. In this paper, the relationship between infection, immunoglobulin level and VA level was analyzed. The results showed that CVAD was 27.27% in MPP group and 63.75% in RMPP group. Comparison of immunoglobulin levels between the two groups: the levels of IgM, IgA and IgG in the normal MPP group were significantly lower than those in the RMPP group. On the other hand, the detection rate of CVAD was 81.48% in RMPP with infection and 54.72% in RMPP without infection. The detection rate of CVAD in patients with infection was significantly higher than that in patients without infection. There was a correlation between VA Deficiency and MPP classification.


2021 ◽  
Author(s):  
Stefanie J.G. Veenhuis ◽  
Nienke J.H. Os ◽  
Anjo J.W.M. Janssen ◽  
Marjo H.J.C. Gerven ◽  
Karlien L.M. Coene ◽  
...  

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