| 1. |
- Berg, Stefan, 1959, et al.
(författare)
-
Irregular Recurrent Fever : Chapter 113
- 2019
-
Ingår i: Pediatric Immunology. A Case-Based Collection with MCQs. Nima Rezaei (red.). - Cham : Springer Nature Switzerland AG. - 9783030212629 ; , s. 617-621
-
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
|
|
| 2. |
- Berg, Stefan, 1959, et al.
(författare)
-
Long Episodes of Rash and Fever : Chapter 100
- 2019
-
Ingår i: Pediatric Immunology : A Case-Based Collection with MCQs, Volume 2. Rezaei, N. (red.). - Switzerland AG : Springer Nature. - 9783030212629 ; , s. 527-531
-
Bokkapitel (övrigt vetenskapligt/konstnärligt)
|
|
| 3. |
- Berg, Stefan, 1959, et al.
(författare)
-
Rash and Fever since Two Weeks of Age : Chapter 102
- 2019
-
Ingår i: Pediatric Immunology. A Case-Based Collection with MCQs. Rezaei, N. (red.). - Cham : Springer Nature. - 9783030212629 ; , s. 539-543
-
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
|
|
| 4. |
- Berg, Stefan, 1959, et al.
(författare)
-
Sudden Dizziness, Somnolence and Diplopia : Chapter 111
- 2019
-
Ingår i: Pediatric Immunology. A Case-Based Collection with MCQs.. - Cham : Springer Nature. - 9783030212629 ; , s. 603-609
-
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
|
|
| 5. |
- Wekell, Per, et al.
(författare)
-
Prolonged Fever and Swollen Joints : Chapter 106
- 2019
-
Ingår i: Pediatric Immunology A Case-Based Collection with MCQs. Nima Rezaei (red.). - Cham : Springer Nature Switzerland AG. - 9783030212629 ; , s. 565-573
-
Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- The diagnosis of systemic onset juvenile idiopathic arthritis (SoJIA) is based on clinical features including: prolonged quotidian fever, erythematous rash during fever spikes, generalized lymph node enlargement, arthralgia/arthritis and splenomegaly Glucocorticoids are the first-line treatment of SoJIA Approximately 10% of patients with SoJIA develop macrophage activation syndrome (MAS) associated with significant mortality The main complication of SoJIA is MAS characterised by: Clinical features such as continuous fever, petechial rash, hepatosplenomegaly and compromised circulation, laboratory findings such as cytopenias in two or more cell lines, increased ferritin, liver transaminases, lactate dehydrogenase and triglycerides There are no evidence-based treatment guidelines for MAS in SoJIA. Early expert advice should be sought. High-dose intravenous methylprednisolone is recommended as a first-line treatment.
|
|
| 6. |
- Wekell, Per, et al.
(författare)
-
Recurrent Chest Pain : Chapter 112
- 2019
-
Ingår i: Pediatric Immunology. A Case-Based Collection with MCQs. Nima Rezaei (red.). - Cham : Springer Nature Switzerland AG. - 9783030212629 ; , s. 611-616
-
Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- Recurrent pericarditis is diagnosed in a patient with acute pericarditis relapse after a symptom-free interval of 4–6 weeks or longer First-line treatment for recurrent pericarditis is cycloxygenase-inhibitors and colchicine Corticosteroid should be avoided in children due to their adverse side-effects, increased risk of relapse and effects on growth Long-term prognosis for children with recurrent pericarditis is generally good
|
|
| 7. |
- Wekell, Per, et al.
(författare)
-
Skeletal Pain in Knee and Clavicle : Chapter 107
- 2019
-
Ingår i: Pediatric Immunology. A Case-Based Collection with MCQs. Nima Rezaei (red.). - Cham : Springer Nature Switzerland AG. - 9783030212629 ; , s. 575-581
-
Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- Chronic non-bacterial osteomyelitis (CNO) or chronic recurrent multifocal osteomyelitis (CRMO) is characterized by sterile bone inflammation commonly located in the metaphysis of long bones, the clavicle, spine, and the pelvisBone lesions can be both symptomatic and asymptomatic CNO/CRMO is a diagnosis of exclusion and a bone biopsy is often needed to confirm the diagnosis and exclude other disorders Empirical treatment options include; Cox-inhibitors (NSAIDs), glucocorticoids, anti-TNF agents and bisphosphonates
|
|
