Zentrum für Primäre Immundefekte und Autoinflammatorische Erkrankungen am Universitätsklinikum Würzburg
Description of facility
Director / Spokesperson
PD Dr. Henner MorbachInformation
Care facility for adults and childrenDescription
Das Zentrum für Primäre Immundefekte und Autoinflammatorische Erkrankungen betreut ca. 110 Patienten mit primären Immundefekten. Die Versorgung der Kinder und Jugendlichen erfolgt federführend an der Universitäts-Kinderklinik. Das Zentrum verfügt über ein Speziallabor zur Diagnostik von Immunstörungen und forscht intensiv sowohl an den Ursachen der Erkrankungen als auch an deren Auswirkungen.
Consultation hours
nach Vereinbarung.
Care provisions
This facility offers the following
-
Participation in registries
Europäisches Patientenregister für primäre Immundefekte (ESID registry) -
Social / legal advice
- Genetic counselling
-
Clinical studies / research
A prospective outcome study on patients with profound combined immunodeficiency Study of interstitial lung disease in Primary antibody deficiency - Diagnostic
- Therapy
-
Contact person for patients with an unclear diagnosis
Anfragen über das Zentrum für Seltene Erkrankungen - Referenzzentrum Nordbayern, siehe www.zese.ukw.de -
Contact with support groups
Deutsche Selbsthilfe Angeborene Immundefekte e.V. (DSAI)
Contact
PD Dr. Henner Morbach
0931 20127728
morbach_h@ukw.de
Website
https://www.ukw.de/behandlungszentren/zentrum-fuer-primaere-immundefekte-und-autoinflammatorische-erkrankungen/startseite/
Languages
Deutsch
Englisch
Italienisch
European Reference Network 1
Mentioned by the following facilities 1
Preview of the assigned diseases 4
Dyskeratosis congenita
Rare chromosomal anomaly
Leukocyte adhesion deficiency type I
Severe combined immunodeficiency due to DCLRE1C deficiency
Autoimmune lymphoproliferative syndrome with recurrent viral infections
T-B+ severe combined immunodeficiency due to gamma chain deficiency
Leukocyte adhesion deficiency type III
Severe combined immunodeficiency due to adenosine deaminase deficiency
Diffuse large B-cell lymphoma with chronic inflammation
Leukocyte adhesion deficiency type II
Primary immunodeficiency syndrome due to LAMTOR2 deficiency
Autosomal recessive mendelian susceptibility to mycobacterial diseases due to partial IFNgammaR2 deficiency
Agammaglobulinemia-microcephaly-craniosynostosis-severe dermatitis syndrome
Autosomal recessive mendelian susceptibility to mycobacterial diseases due to partial IFNgammaR1 deficiency
Susceptibility to viral and mycobacterial infections due to STAT1 deficiency
Facial dysmorphism-immunodeficiency-livedo-short stature syndrome
Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood
Combined immunodeficiency with granulomatosis
Nodular lymphocyte predominant Hodgkin lymphoma
Familial isolated congenital asplenia
Autosomal dominant mendelian susceptibility to mycobacterial diseases due to partial IFNgammaR1 deficiency
Activated PI3K-delta syndrome
Transient hypogammaglobulinemia of infancy
Felty syndrome
Autosomal recessive severe congenital neutropenia due to G6PC3 deficiency
Lung fibrosis-immunodeficiency-46,XX gonadal dysgenesis syndrome
Glycogen storage disease due to glucose-6-phosphatase deficiency type Ib
Recurrent infection due to specific granule deficiency
Hydroa vacciniforme-like lymphoma
Hyperzincemia and hypercalprotectinemia
Pearson syndrome
Dianzani autoimmune lymphoproliferative disease
Hepatic veno-occlusive disease-immunodeficiency syndrome
Immunodeficiency due to a classical component pathway complement deficiency
Autosomal dominant mendelian susceptibility to mycobacterial diseases due to partial IFNgammaR2 deficiency
Herpes simplex virus encephalitis
Constitutional neutropenia with extra-hematopoietic manifestations
PLCG2-associated antibody deficiency and immune dysregulation
22q11.2 deletion syndrome
X-linked hyper-IgM syndrome
Hyper-IgM syndrome type 4
Hyper-IgM syndrome type 3
Hyper-IgM syndrome type 2
T-B+ severe combined immunodeficiency due to JAK3 deficiency
Immunodeficiency by defective expression of MHC class II
Immunodeficiency due to a late component of complement deficiency
Hyper-IgM syndrome type 5
Immunodeficiency due to ficolin3 deficiency
T-B+ severe combined immunodeficiency
Nijmegen breakage syndrome-like disorder
Acquired immunodeficiency
Epidermodysplasia verruciformis
Combined immunodeficiency due to STK4 deficiency
Muckle-Wells syndrome
Chédiak-Higashi syndrome
Mendelian susceptibility to mycobacterial diseases due to partial IRF8 deficiency
Other immunodeficiency syndromes due to defects in innate immunity
T-B+ severe combined immunodeficiency due to IL-7Ralpha deficiency
Isolated agammaglobulinemia
Immunodeficiency due to MASP-2 deficiency
T-B+ severe combined immunodeficiency due to CD3delta/CD3epsilon/CD3zeta
Combined immunodeficiency due to CARD11 deficiency
Omenn syndrome
Mendelian susceptibility to mycobacterial diseases due to partial STAT1 deficiency
T-B+ severe combined immunodeficiency due to CD45 deficiency
Primary central nervous system lymphoma
X-linked mendelian susceptibility to mycobacterial diseases
Syndromic agammaglobulinemia
Autoimmune enteropathy and endocrinopathy-susceptibility to chronic infections syndrome
Cartilage-hair hypoplasia
Severe combined immunodeficiency due to DNA-PKcs deficiency
T-B- severe combined immunodeficiency
Autoimmune polyendocrinopathy type 1
X-linked severe congenital neutropenia
Specific antibody deficiency with normal immunoglobulin concentrations and normal numbers of B cells
X-linked agammaglobulinemia
Kostmann syndrome
Severe congenital neutropenia
Hoyeraal-Hreidarsson syndrome
Roifman syndrome
Blau syndrome
Combined immunodeficiency due to ORAI1 deficiency
X-linked mendelian susceptibility to mycobacterial diseases due to IKBKG deficiency
Acquired neutropenia
Combined immunodeficiency with facio-oculo-skeletal anomalies
Combined immunodeficiency due to STIM1 deficiency
Severe combined immunodeficiency due to complete RAG1/2 deficiency
Laron syndrome with immunodeficiency
Syndrome with combined immunodeficiency
X-linked mendelian susceptibility to mycobacterial diseases due to CYBB deficiency
Post-transplant lymphoproliferative disease
Cohen syndrome
Schimke immuno-osseous dysplasia
Autoinflammatory syndrome with immune deficiency
Immunodeficiency with factor I anomaly
Deficiency in anterior pituitary function-variable immunodeficiency syndrome
Primary immunodeficiency due to a defect in adaptive immunity
Mendelian susceptibility to mycobacterial diseases due to complete IFNgammaR1 deficiency
Immunodeficiency with factor H anomaly
Susceptibility to infection due to TYK2 deficiency
Mendelian susceptibility to mycobacterial diseases
Immunodeficiency due to absence of thymus
Hypohidrotic ectodermal dysplasia with immunodeficiency
Recurrent Neisseria infections due to factor D deficiency
Familial Mediterranean fever
Hyper-IgE syndrome
Hyperimmunoglobulinemia D with periodic fever
Lichtenstein syndrome
Sterile multifocal osteomyelitis with periostitis and pustulosis
Common variable immunodeficiency
Selective IgM deficiency
Anhidrotic ectodermal dysplasia-immunodeficiency-osteopetrosis-lymphedema syndrome
Severe combined immunodeficiency
Reticular dysgenesis
Severe combined immunodeficiency due to IKK2 deficiency
Immunodeficiency due to interleukin-1 receptor-associated kinase-4 deficiency
Autosomal dominant severe congenital neutropenia
Immunodeficiency with isotype or light chain deficiencies with normal number of B-cells
Purine nucleoside phosphorylase deficiency
Immunodeficiency due to selective anti-polysaccharide antibody deficiency
WHIM syndrome
Immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome
Epstein-Barr virus-associated malignant lymphoproliferative disorder
Hyper-IgM syndrome with susceptibility to opportunistic infections
Spondyloenchondrodysplasia
T+ B+ severe combined immunodeficiency
Other immunodeficiency syndrome with predominantly antibody defects
FADD-related immunodeficiency
Agammaglobulinemia
Hyper-IgM syndrome without susceptibility to opportunistic infections
Immunodeficiency by defective expression of MHC class I
ICF syndrome
DNA repair defect other than combined T-cell and B-cell immunodeficiencies
Short stature due to isolated growth hormone deficiency with X-linked hypogammaglobulinemia
Immunodeficiency with severe reduction in serum IgG and IgA with normal/elevated IgM and normal number of B-cells
Absent thumb-short stature-immunodeficiency syndrome
CINCA syndrome
PFAPA syndrome
Idiopathic CD4 lymphocytopenia
T-cell immunodeficiency with epidermodysplasia verruciformis
Wiskott-Aldrich syndrome
Combined T and B cell immunodeficiency
Cernunnos-XLF deficiency
Tumor necrosis factor receptor 1 associated periodic syndrome
X-linked immunodeficiency with magnesium defect, Epstein-Barr virus infection and neoplasia
Congenital neutropenia-myelofibrosis-nephromegaly syndrome
Immuno-osseous dysplasia
Immunodeficiency syndrome with autoimmunity
Immunodeficiency predominantly affecting antibody production
Combined immunodeficiency due to ZAP70 deficiency
Hermansky-Pudlak syndrome due to AP-3 deficiency
Hodgkin lymphoma
Combined immunodeficiency due to CD27 deficiency
Severe combined immunodeficiency due to CORO1A deficiency
Cyclic neutropenia
Immunodeficiency syndrome with hypopigmentation
Combined immunodeficiency due to CD3gamma deficiency
Poikiloderma with neutropenia
Recurrent infections associated with rare immunoglobulin isotypes deficiency
Pancytopenia due to IKZF1 mutations
Functional neutrophil defect
Griscelli syndrome type 2
Adult idiopathic neutropenia
Immune dysregulation disease with immunodeficiency
Griscelli syndrome type 1
Lymphoproliferative syndrome
Susceptibility to respiratory infections associated with CD8alpha chain mutation
Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly
Neutropenia-monocytopenia-deafness syndrome
Majeed syndrome
Primary hemophagocytic lymphohistiocytosis
Griscelli syndrome type 3
Adult-onset immunodeficiency with anti-interferon-gamma autoantibodies
Ataxia-telangiectasia
Nijmegen breakage syndrome
Properdin deficiency
Primary immunodeficiency with natural-killer cell deficiency and adrenal insufficiency
Primary immunodeficiency due to a defect in innate immunity
Severe dermatitis-multiple allergies-metabolic wasting syndrome
Constitutional neutropenia
Combined immunodeficiency due to CRAC channel dysfunction
Autosomal agammaglobulinemia
Leukocyte adhesion deficiency
Combined immunodeficiency due to partial RAG1 deficiency
NLRP3-associated autoinflammatory disease
Severe combined immunodeficiency due to FOXN1 deficiency
Syndromic multisystem autoimmune disease due to Itch deficiency
Chronic granulomatous disease
Immunodeficiency due to a complement cascade protein anomaly
Chronic mucocutaneous candidiasis
RAS-associated autoimmune leukoproliferative disease
Autosomal recessive mendelian susceptibility to mycobacterial diseases due to a partial deficiency
Monocytopenia with susceptibility to infections
Combined immunodeficiency due to MALT1 deficiency
Griscelli syndrome
LIG4 syndrome
TCR-alpha-beta-positive T-cell deficiency
Immunodeficiency due to CD25 deficiency
Congenital sideroblastic anemia-B-cell immunodeficiency-periodic fever-developmental delay syndrome
Autosomal recessive mendelian susceptibility to mycobacterial diseases due to a complete deficiency
Barth syndrome
PAPA syndrome
Plasmablastic lymphoma
Good syndrome
Mendelian susceptibility to mycobacterial diseases due to complete IFNgammaR2 deficiency
Primary immunodeficiency
Ataxia-telangiectasia-like disorder
Combined immunodeficiency due to IL21R deficiency
X-linked immunoneurologic disorder
Short-limb skeletal dysplasia with severe combined immunodeficiency
Classic Hodgkin lymphoma
Autosomal dominant mendelian susceptibility to mycobacterial diseases due to a partial deficiency
Combined immunodeficiency due to DOCK8 deficiency
Neutrophil immunodeficiency syndrome
X-linked lymphoproliferative disease
Immunoglobulin heavy chain deficiency
Autoimmune lymphoproliferative syndrome
Bloom syndrome
Lymphomatoid granulomatosis
Complement component 3 deficiency
T-cell large granular lymphocyte leukemia
Familial cold urticaria
Mendelian susceptibility to mycobacterial diseases due to complete IL12RB1 deficiency
Osteopetrosis-hypogammaglobulinemia syndrome
Vici syndrome
Genetic susceptibility to infections due to particular pathogens
Shwachman-Diamond syndrome
Mendelian susceptibility to mycobacterial diseases due to complete ISG15 deficiency
Familial hemophagocytic lymphohistiocytosis
Say-Barber-Miller syndrome
Primary effusion lymphoma
Autosomal dominant hyper-IgE syndrome due to STAT3 deficiency
Myeloperoxidase deficiency
Bacterial susceptibility due to TLR signaling pathway deficiency
Papillon-Lefèvre syndrome
Burkitt lymphoma
Mendelian susceptibility to mycobacterial diseases due to complete IL12B deficiency
Severe combined immunodeficiency due to LCK deficiency
Extranodal nasal NK/T cell lymphoma
T-cell immunodeficiency with thymic aplasia
9.953800749.8007685Zentrum für Primäre Immundefekte und Autoinflammatorische Erkrankungen am Universitätsklinikum Würzburg
Last updated:
22.03.2023