Pavlina Kralickova

Scopus Publications

Oral deucrictibant for on-demand treatment of hereditary angioedema attacks (RAPIDe-1): a randomised, double-blind, placebo-controlled, phase 2 trial
Marcus Maurer, Marcin Stobiecki, Anna Valerieva, Roman Hakl, Maria T Staevska, et al.
Lancet Haematology, 2026
Epidemiology and management of malignancies in patients with inborn errors of immunity—An ESID registry study of 19,959 patients
Delfien J.A. Bogaert, Christina H. Wolfsberger, Andishe Attarbaschi, Jonathan Gathmann, Klaus Warnatz, et al.
Journal of Allergy and Clinical Immunology, 2026
Machine learning–assisted diagnosis classification of primary immune dysregulation using IDDA2.1 phenotype profiling
Malte Schwitzkowski, Sai Pavan Kumar Veeranki, Benedikt N. Seidel, Gerhard Kindle, Stephan Rusch, et al.
Journal of Allergy and Clinical Immunology, 2026
International Guideline on the Diagnosis and Management of Pediatric Patients With Hereditary Angioedema
Henriette Farkas, Inmaculada Martinez‐Saguer, Konrad Bork, Anastasios E. Germenis, Anete S. Grumach, et al.
Allergy European Journal of Allergy and Clinical Immunology, 2026
Hereditary angioedema (HAE) with C1 inhibitor deficiency is a rare disease characterized by unpredictable episodes of tissue swelling (angioedema), which, in most cases, occur first under the age of 18 years, and entail a significant burden of disease not only for the patients but also for their families. Clinical symptoms of HAE are not specific, which may cause difficulties in differential diagnosis. Additionally, if not appropriately treated, HAE attacks can be life‐threatening. The international HAE guidelines published so far have focused mainly on adults. A guideline that refers to the age‐specific characteristics of pediatric patients, both in terms of diagnosis and management, was therefore needed. The International Steering Committee and Taskforce developed recommendations and provided evidence‐based grading based on expert opinion and strength of evidence. Recommendations were presented to, discussed, and electronically voted by healthcare professionals during the 14th C1 Inhibitor Deficiency and Angioedema Workshop in Budapest, Hungary, 2025. This international guideline will ensure early diagnosis, standardized and up‐to‐date treatment, and promote the availability of effective therapies for all pediatric patients affected with this rare disease. It also draws attention to the importance of establishing HAE centers and registries, which solicit specialist care and research of the disease.
Bronchiectasis, Low IgG Levels and Lack of Vaccination are Risk Factors for Covid-19 Hospitalization in X-linked Agammaglobulinemia – A Retrospective Multicenter Study
Caroline Stenlander, Hannes Lindahl, Emelie Wahren-Borgström, Christoph B. Geier, Anna Sediva, et al.
Journal of Clinical Immunology, 2025
X-linked agammaglobulinemia (XLA) is caused by loss-of-function variants in Bruton’s tyrosine kinase, leading to absence of circulating B lymphocytes and inability to produce antibodies. Despite the fear that patients with XLA would be at high risk for severe infection when the novel virus SARS-CoV-2 emerged in the society with low pre-existing immunity, most patients with XLA did not suffer from severe disease. However, some patients were critically affected. Factors associated with hospitalization in patients with XLA remain poorly described. Thus, we designed a study to determine risk factors associated with hospitalization due to Covid-19 in patients with XLA. Data was collected from 17 sites in Europe and the US, comprising n = 81 patients, with hospitalization due to SARS-CoV-2 infection in 14 patients. Nearly 17% of patients with XLA required hospitalization due to Covid-19, but only 3 patients had ventilatory support. After correcting for the effect of the date of infection during the early pandemic, univariate and multiple logistic regression analysis showed that preexisting bronchiectasis and lower IgG serum trough levels (< 8 g/L) before infection were associated with an increased risk for hospitalization, with a high rate of superinfection. The lack of vaccination seemed to contribute to this risk, and ambulatory patients had higher amounts of CD4 + T cells before infection compared to hospitalized patients. Thus, our data suggests a need for IgG trough levels above 8 g/L, especially in patients with bronchiectasis, to protect patients with XLA during viral infections such as Covid-19 and reduce morbidity due to superinfections.
Correction to: Self-reported Clinical Outcomes and Quality of Life in Agammaglobulinemia: the Importance of an Early Diagnosis (Journal of Clinical Immunology, (2025), 45, 1, (125), 10.1007/s10875-025-01904-z)
Maartje Blom, Annelotte J. Duintjer, Mahnaz Jamee, Melanie de Gier, Markéta Bloomfield, et al.
Journal of Clinical Immunology, 2025
Self-reported Clinical Outcomes and Quality of Life in Agammaglobulinemia: the Importance of an Early Diagnosis
Maartje Blom, Annelotte J. Duintjer, Mahnaz Jamee, Melanie de Gier, Markéta Bloomfield, et al.
Journal of Clinical Immunology, 2025
Purpose Patients with (X-linked) agammaglobulinemia (XLA) suffer from severe, recurrent infections potentially leading to life-threatening complications such as sepsis, meningoencephalitis and chronic lung disease. Early diagnosis and timely treatment can prevent infections and secondary complications, emphasizing a role for early detection of XLA via newborn screening (NBS). Our international multicenter survey study aimed to evaluate self-reported outcomes and parental perspectives in XLA patients to determine whether an early diagnosis is associated with better quality of life (QoL). Methods QoL-questionnaires included the PedsQL for children and SF-36, CVID_QOL, PADQOL-16 for adults. A new questionnaire was specifically developed for parents about an early diagnosis of XLA. Results In total, 88 adult and 65 pediatric XLA patients, and 69 parents from 14 countries completed the survey. Patients with an early diagnosis reported less severe, recurrent infections and less hospitalization (p < 0.05). QoL was significantly lower in multiple health domains for pediatric and adult patients with a late diagnosis compared to the general population. Patients with an early diagnosis reported similar QoL outcomes compared to the general population. Parents showed immense support for NBS for XLA stating that an early diagnosis prevents emotional insecurity, health damage, unnecessary diagnostics and allows early access to medical care and informed family planning. Conclusion Our study has shown supportive evidence to pursue an early diagnosis of XLA from both a self-reported clinical, health related QoL and parental perspective. The main plea from patients and parents is to achieve an early diagnosis for XLA and severe B-lymphocyte deficiencies with NBS.
Recommendations for Transitioning Young People with Primary Immunodeficiency Disorders and Autoinflammatory Diseases to Adult Care
Muskan Israni, Eliska Alderson, Nizar Mahlaoui, Laura Obici, Linda Rossi-Semerano, et al.
Journal of Clinical Immunology, 2025
Purpose Significant improvements in the prognosis for young patients with Primary Immunodeficiency Diseases (PID) and Autoinflammatory Disorders (AID), which together make up the majority of Inborn Errors of Immunity (IEI), have resulted in the need for optimisation of transition and transfer of care to adult services. Effective transition is crucial to improve health outcomes and treatment compliance among patients. Evaluations of existing transition programmes in European health centres identified the absence of disease-specific transition guidelines for PID and AID, as a challenge to the transition process. This research aimed to establish expert consensus statements for the transition of young patients with PID and AID to adult services. Methods This project used the Delphi method to establish mutual agreement for the proposed recommendations. A draft set of statements was developed following a literature review of existing transition programmes. Then the ERN RITA Transition Working Group convened to review the drafted recommendations and develop them into a survey. This survey was circulated among healthcare professionals to determine consensus using a five-point Likert scale, with the level of agreement set to 80% or greater. Statements that did not reach consensus were revised by the Working Group and recirculated among respondents. Results The initial survey received 93 responses from 68 centres across 23 countries, while the following survey outlining revised recommendations received 66 responses. The respondents agreed upon recommendations detailing the structure and administration of transition programmes, collaborative working with social systems, and contraindications to transfer of care. Conclusion This paper sets out a comprehensive set of recommendations to optimise transitional care for PID and AID.
Gastrointestinal disorders in patients with common variable immunodeficiency
Pavlína Králíčková, Štěpán Šembera, Kateřina Kamarádová, Ilja Tachecí
Gastroenterologie A Hepatologie, 2025
Summary: Common variable immunodefi ciency (CVID) is a group of inherited antibody immune disorders. Patients often suff er from recurrent bacterial infections, autoimmune or dysregulatory complications, and have an increased risk of developing cancer (e. g., lymphoma, gastric cancer). The most common gastrointestinal pathogens include Helicobacter pylori, Campylobacter jejuni, and Giardia lamblia; in more severe cases, norovirus or cytomegalovirus (CMV) may cause signifi cant diarrhoea. Bacterial overgrowth syndrome is also common. CVID is associated with alterations in the microbio me, which contributes to complications in distant organs such as the lungs and liver. Non-infectious complications can aff ect the entire gastrointestinal tract. Notable examples include intestinal metaplasia of the gastric mucosa with a risk of carcinoma, enteropathy mimicking celiac dis ease, infl ammatory bowel dis ease-like colitis, nodular regenerative hyperplasia, and pre-portal hypertension. These complications typically develop slowly and subtly over time. Once malnutrition sets in, irreversible changes to the intestinal mucosa are often present. Managing CVID requires a multidisciplinary approach and regular screening. Endoscopic examinations, including repeated and multiple bio psies, are essential for accurate dia gnosis. Elastography is suitable for detecting hepatopathies. Serological blood tests are generally not helpful. Treatment is challenging and should be individualized. Immunoglobulin replacement therapy reduces the frequency of infections only. In cases of non-infectious complications, immunomodulatory therapy is required and, tailored to the patient’s genetic background, if possible. Key words: common variable immunodeficiency – endoscopy – intestinal dis eases – malabsorption syndrome – liver dis ease – microbio ta – immunomodulation
First report from the Czech national registry of inborn errors of immunity (2012–2025)
Zita Chovancova, Eva Hlavackova, Roman Hakl, Tomas Milota, Pavlina Kralickova, et al.
Frontiers in Immunology, 2025
IntroductionCongenital immune system defects represent an ever-growing group of diseases characterized by increased susceptibility to infections and association with autoimmune, autoinflammatory, allergic and malignant complications. Here, we provide the first comprehensive report on inborn errors of immunity (IEIs) in Czechia based on the analysis of patient data from the Czech national registry (CzNR) of IEIs.Material and methodsThe online platform of CzNR of IEIs was established in 2012, compiling data about epidemiology, type of diagnosis, clinical and laboratory parameters, as well as the treatment of patients diagnosed with IEIs since 1981.ResultsThe total of 1,443 registered patients includes 697 males (48.3%) and 746 females (51.7%). The median age at diagnosis was 21.0 (0–86) years. The most represented group of patients was those with antibody deficiencies (788 patients; 54.6%). This was followed by complement deficiencies (242; 16.8%), combined immunodeficiencies with syndromic features (250; 17.3%), combined immunodeficiencies (55; 3.8%), congenital defects of phagocyte number, function, or both (31; 2.1%), autoinflammatory disorders (28; 1.9%), immune dysregulation diseases (24; 1.7%), intrinsic and innate immunity defects (21; 1.5%), primary immunodeficiency phenocopies (3; 0.2%), and bone marrow failure disorders (1; 0.1%). Common variable immunodeficiency (504; 34.9%), hereditary angioedema (222; 15.4%), and DiGeorge syndrome (182; 12.6%) were the most frequent diagnoses.ConclusionIn this article, we report the epidemiology of IEIs in the Czech Republic for the first time based on the CzNR of IEIs data. The prevalence of IEIs is approximately 13.2 patients per 100000 inhabitants of the Czech Republic.
Clinical efficacy of SARS-CoV-2 Omicron-neutralizing antibodies in immunoglobulin preparations for the treatment of agammaglobulinemia in patients with primary antibody deficiency
Michael Karbiener, Gerhard Kindle, Isabelle Meyts, Mikko R. J. Seppänen, Fabio Candotti, et al.
Journal of Medical Virology, 2024
Systematic Approach Revealed SERPING1 Splicing-Affecting Variants to be Highly Represented in the Czech National HAE Cohort
Hana Grombirikova, Viktor Bily, Premysl Soucek, Michal Kramarek, Roman Hakl, et al.
Journal of Clinical Immunology, 2023
Activated phosphoinositide 3-kinase δ syndrome: Update from the ESID Registry and comparison with other autoimmune-lymphoproliferative inborn errors of immunity
Maria Elena Maccari, Martin Wolkewitz, Charlotte Schwab, Tiziana Lorenzini, Jennifer W. Leiding, et al.
Journal of Allergy and Clinical Immunology, 2023
Subsets of Eosinophils in Asthma, a Challenge for Precise Treatment
Jakub Novosad, Irena Krčmová, Ondřej Souček, Marcela Drahošová, Vratislav Sedlák, et al.
International Journal of Molecular Sciences, 2023
Severe combined immunodeficiencies
Dita Záveská, Ilona Pospíšilová, Karin Bánszka, Tomáš Freiberger, Hana Grombiříková, et al.
Cesko Slovenska Pediatrie, 2023
Myeloid lineage cells evince distinct steady-state level of certain gene groups in dependence on hereditary angioedema severity
Lucie Ballonová, Přemysl Souček, Peter Slanina, Kamila Réblová, Ondřej Zapletal, et al.
Frontiers in Genetics, 2023
Immunogenicity and Safety of the Spikevax® (Moderna) mRNA SARS-CoV-2 Vaccine in Patients with Primary Humoral Immunodeficiency
Pavlina Kralickova, Karolina Jankovicova, Ilona Sejkorova, Ondrej Soucek, Katerina Koprivova, et al.
International Archives of Allergy and Immunology, 2022
Registry-based analysis of Icatibant and C1-inhibitor use in treatment of laryngeal attacks of hereditary angioedema
Roman Hakl, Pavel Kuklínek, Marta Sobotková, Irena Krčmová, Pavlína Králíčková, et al.
Clinical and Experimental Allergy, 2022
A Multi‑Center, Open‑Label, Single‑Arm Trial to Evaluate the Efficacy, Pharmacokinetics, and Safety and Tolerability of IGSC 20% in Subjects with Primary Immunodeficiency
Manuel Santamaria, Olaf Neth, Jo A. Douglass, Gergely Krivan, Robin Kobbe, et al.
Journal of Clinical Immunology, 2022
Correction to: A Multi-center, Open-Label, Single-Arm Trial to Evaluate the Efficacy, Pharmacokinetics, and Safety and Tolerability of IGSC 20% in Subjects with Primary Immunodeficiency (Journal of Clinical Immunology, (2022), 42, 3, (500-511), 10.1007/s10875-021-01181-6)
Manuel Santamaria, Olaf Neth, Jo A. Douglass, Gergely Krivan, Robin Kobbe, et al.
Journal of Clinical Immunology, 2022
Risk Factors for Severe COVID-19 and Hospital Admission in Patients With Inborn Errors of Immunity - Results From a Multicenter Nationwide Study
Tomas Milota, Marta Sobotkova, Jitka Smetanova, Marketa Bloomfield, Jana Vydlakova, et al.
Frontiers in Immunology, 2022
Therapeutic options for CTLA-4 insufficiency
David Egg, Ina Caroline Rump, Noriko Mitsuiki, Jessica Rojas-Restrepo, Maria-Elena Maccari, et al.
Journal of Allergy and Clinical Immunology, 2022
Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients
Marta Sobotkova, Radana Zachova, Roman Hakl, Pavel Kuklinek, Pavlina Kralickova, et al.
International Archives of Allergy and Immunology, 2021
Gut microbiota and nafld: Pathogenetic mechanisms, microbiota signatures, and therapeutic interventions
Tomas Hrncir, Lucia Hrncirova, Miloslav Kverka, Robert Hromadka, Vladimira Machova, et al.
Microorganisms, 2021
Primární imunodeficience - diagnostika a lécba
Pediatrie Pro Praxi, 2021
Treatment of allergic rhinoconjuctivitis in children
Pavlína Králíčková, Dita Záveská
Pediatrie Pro Praxi, 2021
Vrozený deficit C2 složky komplementu - variabilní klinická manifestace (kazuistiky a přehled problematiky)
Vnitrni Lekarstvi, 2020
Inherited c2-complement deficiency: Variable clinical manifestation (case reports and review)
Vnitrni Lekarstvi, 2020
Czech Hizentra Noninterventional Study With Rapid Push: Efficacy, Safety, Tolerability, and Convenience of Therapy With 20% Subcutaneous Immunoglobulin
Tomas Milota, Marketa Bloomfield, Pavlina Kralickova, Dalibor Jilek, Vitezslav Novak, et al.
Clinical Therapeutics, 2019
Challenges in investigating patients with isolated decreased serum IgM: The SIMcal study
Lisanne M. A. Janssen, Roeland W. N. M. Hout, Esther de Vries, Claudio Pignata, Emilia Cirillo, et al.
Scandinavian Journal of Immunology, 2019
Vaccination as diagnostic and therapeutic approach in primary and secondary humoral immunodeficiencies
Vakcinologie, 2019
Bacterial but not fungal gut microbiota alterations are associated with common variable immunodeficiency (CVID) phenotype
Kristýna Fiedorová, Matěj Radvanský, Juraj Bosák, Hana Grombiříková, Eva Němcová, et al.
Frontiers in Immunology, 2019
CVID-associated tumors: Czech nationwide study focused on epidemiology, immunology, and genetic background in a cohort of patients with CVID
Pavlina Kralickova, Tomas Milota, Jiri Litzman, Ivana Malkusova, Dalibor Jilek, et al.
Frontiers in Immunology, 2019
Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy
Roman Hakl, Pavel Kuklínek, Irena Krčmová, Pavlína Králíčková, Tomáš Freiberger, et al.
Journal of Clinical Immunology, 2018
Successful rituximab treatment of granulomatous/lymphocytic interstitial lung disease in common variable immunodeficiency
Epidemiologie Mikrobiologie Imunologie, 2018
Selective IgM Deficiency: Clinical and Laboratory Features of 17 Patients and a Review of the Literature
Zita Chovancova, Pavlina Kralickova, Alena Pejchalova, Marketa Bloomfield, Jana Nechvatalova, et al.
Journal of Clinical Immunology, 2017
Prevention of hereditary angioedema attacks with a subcutaneous C1 inhibitor
Hilary Longhurst, Marco Cicardi, Timothy Craig, Konrad Bork, Clive Grattan, et al.
New England Journal of Medicine, 2017
Erratum: Common Variable Immunodeficiency patients with a phenotypic profile of immunosenescence present with thrombocytopenia
Jan Stuchlý, Veronika Kanderová, Marcela Vlková, Ivana Heřmanová, Lucie Slámová, et al.
Scientific Reports, 2017
Common Variable Immunodeficiency patients with a phenotypic profile of immunosenescence present with thrombocytopenia
Jan Stuchlý, Veronika Kanderová, Marcela Vlková, Ivana Heřmanová, Lucie Slámová, et al.
Scientific Reports, 2017
Antibodies against Pneumococcal Capsular Polysaccharides and Natural Anti-Galactosyl (Alpha-Gal) in Patients with Humoral Immunodeficiencies
P. Kralickova, J. Kuhnova, O. Soucek, P. Vodarek, P. Zak, et al.
Journal of Immunology Research, 2017
Peripheral blood leukocyte populations and urinary neopterin during chemotherapy in patients with breast cancer
Pavlína Králíčková, Doris Vokurková, Jiří Haviger, Vladimíra Řezáčová, Dagmar Solichová, et al.
Pteridines, 2016
Negativity for Specific Autoantibodies in Patients with Type 1 Diabetes That Developed on a Background of Common Variable Immunodeficiency
Tomáš Milota, Zdeněk Šumník, Barbora Obermannová, Pavlína Králíčková, Karel Vondrák, et al.
International Archives of Allergy and Immunology, 2016
Current forms of immunoglobulin substitution therapy
Alergie, 2016
Principles of rational antibiotic use (part II)
Intervencni A Akutni Kardiologie, 2016
Principles of rational antibiotic use (part I)
Intervencni A Akutni Kardiologie, 2016
Recurrent meningitis and inherited complement deficiency
Epidemiologie Mikrobiologie Imunologie, 2016
Pregnancy Outcome in Patients with Common Variable Immunodeficiency
Pavlina Kralickova, Barbora Kurecova, Ctirad Andrys, Irena Krcmova, Dalibor Jilek, et al.
Journal of Clinical Immunology, 2015
Spectrum and Management of Complement Immunodeficiencies (Excluding Hereditary Angioedema) Across Europe
A. J. Turley, B. Gathmann, C. Bangs, M. Bradbury, S. Seneviratne, et al.
Journal of Clinical Immunology, 2015
Secondary humoral immunodeficiency in patiens with systemic lupus erythematosus
Vnitrni Lekarstvi, 2015
Urinary neopterin concentrations during combination therapy with cetuximab in previously treated patients with metastatic colorectal carcinoma
In Vivo, 2014
Immunoglobulin substitution therapy in patients with primary immunodeficiency
Prakticky Lekar, 2014
Cytomegalovirus disease in patients with common variable immunodeficiency: Three case reports
Pavlina Kralickova, Eva Mala, Doris Vokurkova, Irena Krcmova, Lenka Pliskova, et al.
International Archives of Allergy and Immunology, 2014
Nocardia abscessus-Unusual source of nasal inflammation
Otorinolaryngologie A Foniatrie, 2013
Primary immunodeficiencies in children, current therapeutic options
Pediatrie Pro Praxi, 2012
Good's syndrome (thymoma associated hypogammalobulinaemia) - 2 case reports
Vnitrni Lekarstvi, 2012
Hypomagnesaemia in patients with metastatic colorectal carcinoma treated with cetuximab
Hepato Gastroenterology, 2012
Biological therapy in oncology II - Monoclonal antibodies, cytokines
Prakticky Lekar, 2011
Biological therapy in oncology (I)
Prakticky Lekar, 2011
Unusual coincidence of the hereditary angioedema, systemic lupus erythematodes and tongue carcinoma
Otorinolaryngologie A Foniatrie, 2011
Correlation of peripheral blood CD14+CD16+ monocytes, urinary neopterin and the risk factors of atherosclerosis in patients with breast carcinoma
Pavlína Králíčková, Hana Kalábová, Lenka Krčmová, Markéta Kašparová, Jiří Plíšek, et al.
Pteridines, 2011
Hereditary angioedema - Neglected diagnosis
Vnitrni Lekarstvi, 2010
Diffuse large B-cell lymphoma in a patient with hyper-IgE syndrome: Successful treatment with risk-adapted rituximab-based immunochemotherapy
David Belada, Lukáš Smolej, Pavla Štěpánková, Pavlína Králíčková, Tomáš Freiberger
Leukemia Research, 2010
Renal tubular dysfunction and urinary zinc excretion in breast cancer patients treated with anthracycline-based combination chemotherapy
Journal of Experimental and Clinical Cancer Research, 2004
CD4+ T-lymphocytopenia and systemic immune activation in patients with primary and secondary liver tumours
B. Melichar, M. Tousková, D. Solichová, P. Králicková, O. Kopecký
Scandinavian Journal of Clinical and Laboratory Investigation, 2001
Intestinal permeability in patients with chemotherapy-induced stomatitis
Bohuslav Melichar, Pavel Kohout, Miluše Brátová, Dagmar Solichová, Pavlína Králíčkov&#, et al.
Journal of Cancer Research and Clinical Oncology Supplement, 2001

Pavlina Kralickova

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Scopus Publications