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  1. Article: Periodic Fever syndromes.

    Wurster, Victoria M / Carlucci, James G / Edwards, Kathryn M

    Pediatric annals

    2011  Volume 40, Issue 1, Page(s) 48–54

    MeSH term(s) Anti-Bacterial Agents/therapeutic use ; Anti-Inflammatory Agents/therapeutic use ; Anti-Inflammatory Agents, Non-Steroidal/therapeutic use ; Antibodies, Monoclonal/therapeutic use ; Antirheumatic Agents/therapeutic use ; Child ; Colchicine/therapeutic use ; Cryopyrin-Associated Periodic Syndromes/diagnosis ; Cryopyrin-Associated Periodic Syndromes/drug therapy ; Cytokines/therapeutic use ; Diagnosis, Differential ; Familial Mediterranean Fever/diagnosis ; Familial Mediterranean Fever/drug therapy ; Fever/diagnosis ; Fever/drug therapy ; Hereditary Autoinflammatory Diseases/diagnosis ; Hereditary Autoinflammatory Diseases/drug therapy ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use ; Immunosuppressive Agents/therapeutic use ; Lymphadenitis/diagnosis ; Lymphadenitis/drug therapy ; Mevalonate Kinase Deficiency/diagnosis ; Mevalonate Kinase Deficiency/drug therapy ; Neutropenia/diagnosis ; Neutropenia/drug therapy ; Pharyngitis/diagnosis ; Pharyngitis/drug therapy ; Prednisone/therapeutic use ; Recombinant Fusion Proteins/therapeutic use ; Recurrence ; Simvastatin/therapeutic use ; Stomatitis, Aphthous/diagnosis ; Stomatitis, Aphthous/drug therapy ; Syndrome
    Chemical Substances Anti-Bacterial Agents ; Anti-Inflammatory Agents ; Anti-Inflammatory Agents, Non-Steroidal ; Antibodies, Monoclonal ; Antirheumatic Agents ; Cytokines ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Immunosuppressive Agents ; Recombinant Fusion Proteins ; canakinumab (37CQ2C7X93) ; rilonacept (8K80YB5GMG) ; Simvastatin (AGG2FN16EV) ; Colchicine (SML2Y3J35T) ; Prednisone (VB0R961HZT)
    Language English
    Publishing date 2011-01
    Publishing country United States
    Document type Journal Article ; Research Support, U.S. Gov't, P.H.S. ; Review
    ZDB-ID 195430-1
    ISSN 0090-4481
    ISSN 0090-4481
    DOI 10.3928/00904481-20101214-10
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Long-term follow-up of children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.

    Wurster, Victoria M / Carlucci, James G / Feder, Henry M / Edwards, Kathryn M

    The Journal of pediatrics

    2011  Volume 159, Issue 6, Page(s) 958–964

    Abstract: Objective: To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.: Study design: Patients enrolled in a PFAPA registry were contacted and surveyed.: Results: ... ...

    Abstract Objective: To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.
    Study design: Patients enrolled in a PFAPA registry were contacted and surveyed.
    Results: Patients in the registry (n = 59) were surveyed with a follow-up time ranging from 12 to 21 years. Fifty patients had complete symptom resolution, with mean symptom duration of 6.3 years (95% CI, 5.4-7.3), and no sequelae developed. Nine patients continued to have persistent symptoms for a mean duration of 18.1 years (95% CI, 17.4-18.8). There were no differences in initial presentation between subjects with resolved PFAPA and subjects with persistent PFAPA. In subjects with persistent PFAPA, the mean duration of fever >38.3°C decreased from 3.6 days at onset to 1.8 days at follow-up (P = .01), and the mean symptom-free interval between episodes increased from 29 to 159 days (P < .005). Thirty-seven of 44 patients treated with corticosteroids reported prompt symptom resolution. Twelve patients underwent tonsillectomy or adenotonsillectomy; 9 of these patients experienced markedly reduced symptoms, and 6 patients had resolution of symptoms. Two subjects received other diagnoses.
    Conclusions: In long-term follow-up, most patients with PFAPA experienced spontaneous symptom resolution without sequelae. Patients with persistent symptoms had episodes of shorter duration and reduced frequency.
    MeSH term(s) Child, Preschool ; Female ; Fever ; Follow-Up Studies ; Humans ; Infant ; Lymphadenitis/diagnosis ; Lymphadenitis/therapy ; Male ; Neck ; Pharyngitis/diagnosis ; Pharyngitis/therapy ; Stomatitis, Aphthous/diagnosis ; Stomatitis, Aphthous/therapy ; Syndrome ; Time Factors ; Young Adult
    Language English
    Publishing date 2011-12
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 3102-1
    ISSN 1097-6833 ; 0022-3476
    ISSN (online) 1097-6833
    ISSN 0022-3476
    DOI 10.1016/j.jpeds.2011.06.004
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: Long-Term Follow-Up of Children with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome

    Wurster, Victoria M / Carlucci, James G / Feder, Henry M., Jr / Edwards, Kathryn M

    journal of pediatrics. 2011 Dec., v. 159, no. 6

    2011  

    Abstract: OBJECTIVE: To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. STUDY DESIGN: Patients enrolled in a PFAPA registry were contacted and surveyed. RESULTS: Patients in ... ...

    Abstract OBJECTIVE: To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. STUDY DESIGN: Patients enrolled in a PFAPA registry were contacted and surveyed. RESULTS: Patients in the registry (n = 59) were surveyed with a follow-up time ranging from 12 to 21 years. Fifty patients had complete symptom resolution, with mean symptom duration of 6.3 years (95% CI, 5.4-7.3), and no sequelae developed. Nine patients continued to have persistent symptoms for a mean duration of 18.1 years (95% CI, 17.4-18.8). There were no differences in initial presentation between subjects with resolved PFAPA and subjects with persistent PFAPA. In subjects with persistent PFAPA, the mean duration of fever >38.3°C decreased from 3.6 days at onset to 1.8 days at follow-up (P = .01), and the mean symptom-free interval between episodes increased from 29 to 159 days (P < .005). Thirty-seven of 44 patients treated with corticosteroids reported prompt symptom resolution. Twelve patients underwent tonsillectomy or adenotonsillectomy; 9 of these patients experienced markedly reduced symptoms, and 6 patients had resolution of symptoms. Two subjects received other diagnoses. CONCLUSIONS: In long-term follow-up, most patients with PFAPA experienced spontaneous symptom resolution without sequelae. Patients with persistent symptoms had episodes of shorter duration and reduced frequency.
    Keywords adrenal cortex hormones ; children ; complications (disease) ; fever ; patients ; pharyngitis
    Language English
    Dates of publication 2011-12
    Size p. 958-964.
    Publishing place Mosby, Inc.
    Document type Article
    ZDB-ID 3102-1
    ISSN 1097-6833 ; 0022-3476
    ISSN (online) 1097-6833
    ISSN 0022-3476
    DOI 10.1016/j.jpeds.2011.06.004
    Database NAL-Catalogue (AGRICOLA)

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  4. Article: Long-Term Follow-Up of Children with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome

    Wurster, Victoria M. / Carlucci, James G. / Feder, Henry M., Jr. / Edwards, Kathryn M.

    journal of pediatrics

    Volume v. 159,, Issue no. 6

    Abstract: OBJECTIVE: To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. STUDY DESIGN: Patients enrolled in a PFAPA registry were contacted and surveyed. RESULTS: Patients in ... ...

    Abstract OBJECTIVE: To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. STUDY DESIGN: Patients enrolled in a PFAPA registry were contacted and surveyed. RESULTS: Patients in the registry (n = 59) were surveyed with a follow-up time ranging from 12 to 21 years. Fifty patients had complete symptom resolution, with mean symptom duration of 6.3 years (95% CI, 5.4-7.3), and no sequelae developed. Nine patients continued to have persistent symptoms for a mean duration of 18.1 years (95% CI, 17.4-18.8). There were no differences in initial presentation between subjects with resolved PFAPA and subjects with persistent PFAPA. In subjects with persistent PFAPA, the mean duration of fever >38.3°C decreased from 3.6 days at onset to 1.8 days at follow-up (P = .01), and the mean symptom-free interval between episodes increased from 29 to 159 days (P < .005). Thirty-seven of 44 patients treated with corticosteroids reported prompt symptom resolution. Twelve patients underwent tonsillectomy or adenotonsillectomy; 9 of these patients experienced markedly reduced symptoms, and 6 patients had resolution of symptoms. Two subjects received other diagnoses. CONCLUSIONS: In long-term follow-up, most patients with PFAPA experienced spontaneous symptom resolution without sequelae. Patients with persistent symptoms had episodes of shorter duration and reduced frequency.
    Keywords patients ; pharyngitis ; children ; adrenal cortex hormones ; fever ; complications (disease)
    Language English
    Document type Article
    ISSN 0022-3476
    Database AGRIS - International Information System for the Agricultural Sciences and Technology

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