All Stories

  1. Methotrexate Therapy in Juvenile Idiopathic Arthritis: No Clinically Relevant Pulmonary Impairment but Frequent Transient Liver Enzyme Elevations in a Longitudinal Single‐Center Pediatric Cohort of 274 Children Over 30 Years
  2. Type-Specific Impacts of Protein Defects in Pathogenic NFKB2 Variants: Novel Clinical Findings From 138 Patients
  3. Infants with biliary atresia exhibit an altered amino acid profile in their newborn screening
  4. Evaluation of Anti-CAR Linker mAbs for CAR T Monitoring after BiTEs/bsAbs and CAR T-Cell Pretreatment
  5. Diagnostic evaluation of paediatric autoimmune lymphoproliferative immunodeficiencies (ALPID): a prospective cohort study
  6. Identification of disease-specific TCRs maintaining pathogenic T helper cell responses in postinfectious Lyme Arthritis
  7. Abnormal biomarkers predict complex FAS or FADD defects missed by exome sequencing
  8. Kinderrheumatologie
  9. Activated phosphoinositide 3-kinase δ syndrome: Update from the ESID Registry and comparison with other autoimmune-lymphoproliferative inborn errors of immunity
  10. MAGT1 Deficiency Dysregulates Platelet Cation Homeostasis and Accelerates Arterial Thrombosis and Ischemic Stroke in Mice
  11. Wichtige angeborenen Störungen des Immunsystems: CTLA-4 Defizienz
  12. Monogenic early-onset lymphoproliferation and autoimmunity: Natural history of STAT3 gain-of-function syndrome
  13. Prospective Newborn Screening for SCID in Germany: A First Analysis by the Pediatric Immunology Working Group (API)
  14. Systematic genetic analysis of pediatric patients with autoinflammatory diseases
  15. Prospective Newborn Screening for SCID in Germany: A first  analysis by the Pediatric Immunology Working Group (API)
  16. Phenotypic spectrum in recessive STING-associated vasculopathy with onset in infancy: Four novel cases and analysis of previously reported cases
  17. Fokale Krampfanfälle und Posteriores Reversibles Enzephalopathie-Syndrom (PRES) als Erstmanifestation einer IgA-Vaskulitis
  18. Case Report: Rubella Virus-Induced Cutaneous Granulomas in Two Pediatric Patients With DNA Double Strand Breakage Repair Disorders – Outcome After Hematopoietic Stem Cell Transplantation
  19. Erratum zu: Warnzeichen für und moderne Diagnostik von angeborenen Störungen des Immunsystems
  20. Warnzeichen für und moderne Diagnostik von angeborenen Störungen des Immunsystems
  21. Chronisch-entzündliche Darmerkrankung
  22. Genetics in inborn errors of immunity: pediatric autoinflammatory phenotypes and the underlying genetic causes in 125 families
  23. Therapeutic options for CTLA-4 insufficiency
  24. The First 4 Years – Outcome of Children Identified by Newborn Screening for CF in Germany
  25. Focal Seizures and Posterior Reversible Encephalopathy Syndrome as Presenting Signs of IgA Vasculitis/Henoch-Schoenlein Purpura—An Educative Case and Systematic Review of the Literature
  26. Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations
  27. Biomarkers of DNA Damage Response Enable Flow Cytometry-Based Diagnostic to Identify Inborn DNA Repair Defects in Primary Immunodeficiencies
  28. Lack of gamma delta T cells ameliorates inflammatory response after acute intestinal ischemia reperfusion in mice
  29. Differential DNA Damage Response of Peripheral Blood Lymphocyte Populations
  30. Multisystem inflammation and susceptibility to viral infections in human ZNFX1 deficiency
  31. The first 4 years - Outcome of children identified by newborn screening for CF in Germany
  32. Activation-induced deaminase is critical for the establishment of DNA methylation patterns prior to the germinal center reaction
  33. Growth Factors Assessed during Kasai Procedure in Liver and Serum Are Not Predictive for the Postoperative Liver Deterioration in Infants with Biliary Atresia
  34. Shared genetic etiology between Parkinson’s disease and blood levels of specific lipids
  35. The transcription factor Bcl11b promotes both canonical and adaptive NK cell differentiation
  36. A distinct CD38+CD45RA+ population of CD4+, CD8+, and double-negative T cells is controlled by FAS
  37. Synthetic retinoid AM80 inhibits IL‐17 production of gamma delta T cells and ameliorates biliary atresia in mice
  38. Synonymous GATA2 mutations result in selective loss of mutated RNA and are common in patients with GATA2 deficiency
  39. Incidence of SCID in Germany from 2014 to 2015 an ESPED* Survey on Behalf of the API*** Erhebungseinheit für Seltene Pädiatrische Erkrankungen in Deutschland (German Paediatric Surveillance Unit) ** Arbeitsgemeinschaft Pädiatrische Immunologie
  40. Liposomal Delivery Improves the Efficacy of Prednisolone to Attenuate Renal Inflammation in a Mouse Model of Acute Renal Allograft Rejection
  41. A novel NFKBIA variant substituting serine 36 of IκBα causes immunodeficiency with warts, bronchiectasis and juvenile rheumatoid arthritis in the absence of ectodermal dysplasia
  42. Distinct molecular response patterns of activating STAT3 mutations associate with penetrance of lymphoproliferation and autoimmunity
  43. Functional flow cytometry of monocytes for routine diagnosis of innate primary immunodeficiencies
  44. Common pre-diagnostic features in individuals with different rare diseases represent a key for diagnostic support with computerized pattern recognition?
  45. Activation-induced deaminase is critical for the establishment of DNA methylation patterns prior to the germinal center reaction
  46. Non-CF Bronchiectasis as a Possible Indicator of a Primary Immunodeficiency: Diagnosis, Clinical Course, and Quality of Life in a Pediatric Cohort
  47. The German National Registry of Primary Immunodeficiencies (2012–2017)
  48. Hepatocyte growth factor levels in livers and serum at Kasai-portoenterostomy are not predictive of clinical outcome in infants with biliary atresia
  49. Clinical and Immunological Phenotype of Patients With Primary Immunodeficiency Due to Damaging Mutations in NFKB2
  50. Phenotype, penetrance, and treatment of 133 cytotoxic T-lymphocyte antigen 4–insufficient subjects
  51. Long-term outcome and necessity of liver transplantation in infants with biliary atresia are independent of cytokine milieu in native liver and serum
  52. Enhanced activation of interleukin-10, heme oxygenase-1, and AKT in C5aR2-deficient mice is associated with protection from ischemia reperfusion injury–induced inflammation and fibrosis
  53. CD59 deficiency presenting as polyneuropathy and Moyamoya syndrome with endothelial abnormalities of small brain vessels
  54. Is an infectious trigger always required for primary hemophagocytic lymphohistiocytosis? Lessons from in utero and neonatal disease
  55. CD57 identifies T cells with functional senescence before terminal differentiation and relative telomere shortening in patients with activated PI3 kinase delta syndrome
  56. Prevalence and clinical challenges among adults with primary immunodeficiency and recombination-activating gene deficiency
  57. Targeted Gene Panel Sequencing for Early-onset Inflammatory Bowel Disease and Chronic Diarrhea
  58. Hemophagocytic lymphohistiocytosis as presenting manifestation of profound combined immunodeficiency due to an ORAI1 mutation
  59. Effective Immunological Guidance of Genetic Analyses Including Exome Sequencing in Patients Evaluated for Hemophagocytic Lymphohistiocytosis
  60. Dextran sodium sulfate (DSS) induces necrotizing enterocolitis-like lesions in neonatal mice
  61. Inflammatory bowel disease caused by primary immunodeficiencies-Clinical presentations, review of literature, and proposal of a rational diagnostic algorithm
  62. Conventional alpha beta (αβ) T cells do not contribute to acute intestinal ischemia-reperfusion injury in mice
  63. IL-17A blockade or deficiency does not affect progressive renal fibrosis following renal ischaemia reperfusion injury in mice
  64. Patient’s Experience in Pediatric Primary Immunodeficiency Disorders: Computerized Classification of Questionnaires
  65. Diagnostic needs for rare diseases and shared prediagnostic phenomena: Results of a German-wide expert Delphi survey
  66. Primary and secondary hemophagocytic lymphohistiocytosis have different patterns of T-cell activation, differentiation and repertoire
  67. Evolution of disease activity and biomarkers on and off rapamycin in 28 patients with autoimmune lymphoproliferative syndrome
  68. DIVERGENT CELLULAR AND CLINICAL PHENOTYPE IN A PATIENT WITH CORONIN1A DEFICIENCY
  69. Reply
  70. Review 2016 - CD26/DPP4 in the immune system
  71. Hyperactive mTOR pathway promotes lymphoproliferation and abnormal differentiation in autoimmune lymphoproliferative syndrome
  72. Unravelling the immunological roles of dipeptidyl peptidase 4 (DPP4) activity and/or structure homologue (DASH) proteins
  73. Transplantation from a symptomatic carrier sister restores host defenses but does not prevent colitis in NEMO deficiency
  74. Interleukin 17, Produced by γδ T Cells, Contributes to Hepatic Inflammation in a Mouse Model of Biliary Atresia and Is Increased in Livers of Patients
  75. Immundefekte als Ursache von chronisch-entzündlichen Erkrankungen des Darmes
  76. The viral dsRNA analogue poly (I:C) induces necrotizing enterocolitis in neonatal mice
  77. FP197γδ T-CELL AND IL-17A DEFICIENCY IN HYPOXIA INDUCED ACUTE KIDNEY INJURY (AKI)
  78. Neutralization of interleukin-17 produced by gamma delta T cells constrains inflammation in experimental biliary atresia
  79. Increased Inflammatory Reaction to Intestinal Ischemia-Reperfusion in Neonatal versus Adult Mice
  80. Renal PKC-  deficiency attenuates acute kidney injury and ischemic allograft injury via TNF- -dependent inhibition of apoptosis and inflammation
  81. Rotavirus particles in the extrahepatic bile duct in experimental biliary atresia
  82. Effects of dipeptidyl peptidase-4 inhibition in an animal model of experimental asthma: a matter of dose, route, and time
  83. Reduced airway inflammation in CD26/DPP4-deficient F344 rats is associated with altered recruitment patterns of regulatory T cells and expression of pulmonary surfactant proteins
  84. Synthetic Retinoid AM80 Inhibits Th17 Cells and Ameliorates Experimental Autoimmune Encephalomyelitis
  85. CD26/dipeptidyl peptidase 4-deficiency alters thymic emigration patterns and leukcocyte subsets in F344-rats age-dependently
  86. Retinoid signals and Th17-mediated pathology
  87. Phenotyping of congenic dipeptidyl peptidase 4 (DP4) deficient Dark Agouti (DA) rats suggests involvement of DP4 in neuro-, endocrine, and immune functions
  88. Regulation of Expression and Function of Dipeptidyl Peptidase 4 (DP4), DP8/9, and DP10 in Allergic Responses of the Lung in Rats