Liver stiffness assessment by transient elastography suggests high prevalence of liver involvement in common variable immunodeficiency

2019 ◽  
Vol 51 (11) ◽  
pp. 1599-1603 ◽  
Author(s):  
Ludovica Crescenzi ◽  
Antonio Pecoraro ◽  
Andrea Fiorentino ◽  
Remo Poto ◽  
Gilda Varricchi ◽  
...  
2017 ◽  
Vol 55 (3) ◽  
pp. 340-351 ◽  
Author(s):  
Junmin Song ◽  
Ana Lleo ◽  
Guo Xiang Yang ◽  
Weici Zhang ◽  
Christopher L. Bowlus ◽  
...  

2019 ◽  
Vol 114 (4) ◽  
pp. 648-655 ◽  
Author(s):  
Sampsa Pikkarainen ◽  
Timi Martelius ◽  
Ari Ristimäki ◽  
Sanna Siitonen ◽  
Mikko R.J. Seppänen ◽  
...  

2017 ◽  
Vol 8 ◽  
Author(s):  
Jannica S. Selenius ◽  
Timi Martelius ◽  
Sampsa Pikkarainen ◽  
Sanna Siitonen ◽  
Eero Mattila ◽  
...  

2021 ◽  
Author(s):  
Tomas Milota ◽  
Kotaska Karel ◽  
Lastuvka Petr ◽  
Smetanova Jitka ◽  
Bloomfield Marketa ◽  
...  

Abstract Background: Common variable immunodeficiency (CVID) is an inborn error of immunity characterized by disturbed immunoglobulin production. Despite of the terrain with severe antibody deficiency, autoantibody-mediated autoimmune phenomena belong to the most frequent autoimmune manifestation. However, many unresolved issues such as prevalence, clinical relevance and origin of autoantibodies detected in CVID patients receiving immunoglobulin replacement therapy (IRT) make the diagnostics of autoimmune complications difficult.Methods: A prospective observational study evaluating the spectrum of 23 different autoantibodies in 38 CVID patients receiving IRT, and in the immunoglobulin solutions used for IRT. Results: The study reveals a high prevalence of anti-GAD (55.3%) and anti-TPO (68.4%) autoantibodes in the cohort of 38 CVID patients on regular IRT. However, the titers of anti-GAD (3.22 vs. 22 kU/L, p≤0.0001) and anti-TPO (109.7 vs. 713 kU/L, p≤0.0001) were significantly lower compared to the newly diagnosed T1D and AIT patients. Moreover, none of the CVID patients with detectable antibodies manifested with T1D and only three patients became suspected of having AIT. A high quantity of anti-GAD (3.24-24.48 kU/L) and anti-TPO (123.6-156.55 kU/L) autoantibodies was found in immunoglobulin solutions for IRT. Conclusions: The study finds a very high prevalence of anti-GAD and anti-TPO autoantibodies in CVID patients receiving regular IRT. Nevertheless, the presence of anti-GAD and anti-TPO is not associated with the manifestation of the respective autoimmune disease. As the high titers of both anti-GAD and anti-TPO were also found in the therapeutics used for IRT, we suggest that the therapeutic immunoglobulins are the source of this false positivity.


2019 ◽  
Vol 7 (7) ◽  
pp. 2507-2508 ◽  
Author(s):  
Antonio Pecoraro ◽  
Ludovica Crescenzi ◽  
Andrea Fiorentino ◽  
Filomena Morisco ◽  
Giuseppe Spadaro

Praxis ◽  
2007 ◽  
Vol 96 (1) ◽  
pp. 3-11
Author(s):  
Suter-Meyer ◽  
Nigg ◽  
Kolyvanos Naumann ◽  
Käser ◽  
Vetter

Author(s):  
Anders Batman Mjelle ◽  
Anesa Mulabecirovic ◽  
Roald Flesland Havre ◽  
Edda Jonina Olafsdottir ◽  
Odd Helge Gilja ◽  
...  

Abstract Purpose Liver elastography is increasingly being applied in screening for and follow-up of pediatric liver disease, and has been shown to correlate well with fibrosis staging through liver biopsy. Because time is of the essence when examining children, we wanted to evaluate if a reliable result can be achieved with fewer acquisitions. Materials and Methods 243 healthy children aged 4–17 years were examined after three hours of fasting. Participants were divided into four age groups: 4–7 years; 8–11 years; 12–14 years and 15–17 years. Both two-dimensional shear wave elastography (2D-SWE; GE Logiq E9) and point shear wave elastography (pSWE; Samsung RS80A with Prestige) were performed in all participants, while transient elastography (TE, Fibroscan) was performed in a subset of 87 children aged 8–17 years. Median liver stiffness measurement (LSM) values of 3, 4, 5, 6, 7, and 8 acquisitions were compared with the median value of 10 acquisitions (reference standard). Comparison was performed for all participants together as well as within every specific age group. We investigated both the intraclass correlation coefficient (ICC) with absolute agreement and all outliers more than 10 %, 20 % or ≥ 0.5 or 1.0 kPa from the median of 10 acquisitions. Results For all three systems there was no significant difference between three and ten acquisitions, with ICCs ≥ 0.97. All systems needed 4 acquisitions to achieve no LSM deviating ≥ 1.0 kPa of a median of ten. To achieve no LSM deviating ≥ 20 % of a median of ten acquisitions, pSWE and TE needed 4 acquisitions, while 2D-SWE required 6 acquisitions. Conclusion Our results contradict recommendations of 10 acquisitions for pSWE and TE and only 3 for 2D-SWE.


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