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| 2. |
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| 3. |
- Berg, Stefan, 1959, et al.
(författare)
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Autoinflammatory disorders
- 2008
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Ingår i: Primary Immunodeficiency Diseases Definition, Diagnosis and Management. - Berlin, Heidelberg : Springer-Verlag. - 9783540785378
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Bokkapitel (övrigt vetenskapligt/konstnärligt)
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| 4. |
- Berg, Stefan, 1959, et al.
(författare)
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Autoinflammatory Disorders
- 2016
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Ingår i: Primary Immunodeficiency Diseases. - Berlin, Heidelberg : Springer. - 9783662529096 ; , s. 393-435
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Bokkapitel (refereegranskat)
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| 5. |
- Berg, Stefan, 1959, et al.
(författare)
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Autoinflammatory disorders
- 2017
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Ingår i: Primary Immunodeficiency Diseases. Definition, Diagnosis, and Management, 2nd ed.. - Berlin, Germany : Springer. - 9783662529072
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Bokkapitel (övrigt vetenskapligt/konstnärligt)
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| 6. |
- Berg, Stefan, 1959, et al.
(författare)
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Irregular Recurrent Fever : Chapter 113
- 2019
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Ingår i: Pediatric Immunology. A Case-Based Collection with MCQs. Nima Rezaei (red.). - Cham : Springer Nature Switzerland AG. - 9783030212629 ; , s. 617-621
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Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- Non-classifiable periodic fever syndromes are common Patients may have recurrent fevers or continuous chronic inflammation, together with different combinations of arthralgia/arthritis, mouth ulcers, lymphadenopathies, conjunctivitis, rashes, pleuritic pain, splenomegaly, hepatomegaly and abdominal pain Inheritance varies from no apparent pattern to autosomal dominant inheritance Many patients respond to colchicine as a reasonable first-line treatment
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| 7. |
- Berg, Stefan, 1959, et al.
(författare)
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Long Episodes of Rash and Fever : Chapter 100
- 2019
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Ingår i: Pediatric Immunology : A Case-Based Collection with MCQs, Volume 2. Rezaei, N. (red.). - Switzerland AG : Springer Nature. - 9783030212629 ; , s. 527-531
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Bokkapitel (övrigt vetenskapligt/konstnärligt)
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| 8. |
- Berg, Stefan, 1959, et al.
(författare)
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Rash and Fever since Two Weeks of Age : Chapter 102
- 2019
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Ingår i: Pediatric Immunology. A Case-Based Collection with MCQs. Rezaei, N. (red.). - Cham : Springer Nature. - 9783030212629 ; , s. 539-543
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Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- Cryopyrin-associated periodic syndrome (CAPS) is an umbrella term today used for three formerly described conditions in order of increasing severity familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and chronic infantile neurologic cutaneous and articular syndrome (CINCA), also known as neonatal-onset multisystem inflammatory disease (NOMID) CAPS is an autosomal dominant disease that starts early in life Mutations in CAPS give rise to a gain-of-function in the NLRP3 inflammasome Somatic mosaicism should be considered in patients with clinical CAPS and no mutation detected in the NLRP3 with Sanger sequencing CAPS is characterized by a varying degree of systemic inflammation, urticaria-like rash, musculoskeletal symptoms, and a risk of amyloidosis and neurologic sequelae Treatment with IL-1 blockade is generally very effective in CAPS
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| 9. |
- Berg, Stefan, 1959, et al.
(författare)
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Sudden Dizziness, Somnolence and Diplopia : Chapter 111
- 2019
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Ingår i: Pediatric Immunology. A Case-Based Collection with MCQs.. - Cham : Springer Nature. - 9783030212629 ; , s. 603-609
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Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- Deficiency of adenosine deaminase 2 (DADA2) is a autosomal recessive disease caused by mutations in CECR1 Mutations in CECR1 cause a deficiency of the enzyme adenosine deaminase type 2 (ADA2) DADA2 phenotype has a wide spectrum and is characterized by the presence of three main features: (1) vascular inflammation, (2) immunodeficiency, and (3) coagulopathy, that may or may not overlap in the individual patient The vascular-inflammatory manifestations include livedo reticularis/racemosa, stroke, vasculitis, recurrent fever episodes and increased inflammatory markers The risk for stroke is high in DADA2 The phenotype may be almost indistinguishable to polyarteritis nodosa (PAN) TNF-blockade is an effective treatment for the vasculitis and inflammatory manifestations Patients with severe disease especially with hematological manifestations and immunodeficiency may benefit from HSCT
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