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| 1 | Neither hereditary periodic fever nor periodic fever, aphthae, pharingitis, adenitis: Undifferentiated periodic fever in a tertiary pediatric center显示文摘AIM To describe the frequency and clinical characteristics of patients with undifferentiated periodic fever(UPF) and to investigate whether a clinical classification of UPF based on the PRINTO-Eurofever score can help predicting the response to treatment and the outcome at follow-up.METHODS Clinical and therapeutic information of patients with recurrent fever who presented at a single pediatric rheumatology center from January 2006 through April 2016 were retrospectively collected. Patients with a clinical suspicion of hereditary periodic fever(HPF) syndrome and patients with clinical picture of periodic fever, aphthae, pharingitis, adenitis(PFAPA) who were refractory to tonsillectomy underwent molecular analysis of five HPF-related genes: MEFV(NM_000243.2), MVK(NM_000431.3), TNFRSF1 A(NM_001065.3), NLRP3(NM_001079821.2), NLRP12(NM_001277126.1). All patients who had a negative genetic result were defined as UPF and further investigated. PRINTO-Eurofever score for clinical diagnosis of HPF was calculated in all cases. RESULTS Of the 221 patients evaluated for periodic fever, twelve subjects with a clinical picture of PFAPA who were refractory to tonsillectomy and 22 subjects with a clinical suspicion of HPF underwent genetic analysis. Twenty-three patients(10.4%) resulted negative and were classified as UPF. The median age at presentation of patients with UPF was 9.5 mo(IQR 4-24). Patients with UPF had a higher frequency of aphthae(52.2% vs 0%, P = 0.0026) and musculoskeletal pain(65.2% vs 18.2%, P = 0.0255) than patients with genetic confirmed HPF. Also, patients with UPF had a higher frequency of aphthous stomatitis(52.2% vs 10.7%, P < 0.0001), musculoskeletal pain(65.2% vs 8,0%, P < 0.0001), and abdominal pain(52.2% vs 4.8%, P < 0.0001) and a lower frequency of pharyngitis(56.6% vs 81.3%, P = 0.0127) compared with typical PFAPA in the same cohort. Twenty-one of 23 patients with UPF(91.3%) received steroids, being effective in 16; 13(56.2%) were given colchicine, which was effective in 6. Symptoms resolution occurred in 2 patients with UPF at last follow-up. Classification according to the PRINTOEurofever score did not correlate with treatment response and prognosis. CONCLUSION UPF is not a rare diagnosis among patients with periodic fever. Clinical presentation place UPF half way on a clinical spectrum between PFAPA and HPF. The PRINTOEurofever score is not useful to predict clinical outcome and treatment response in these patients. | Silvia De Pauli Sara Lega Serena Pastore Domenico Leonardo Grasso Anna Monica Rosaria Bianco Giovanni Maria Severini Alberto Tommasini Andrea Taddio | 2018 | World Journal of Clinical Pediatrics2018，7，1： | 2 |
| 2 | Solid-papillary tumors of the pancreas:histopathology JOP显示文摘 | Santini D Poli F Lega S | 2006 | J Pancreas2006，7，1： | 1 |
| 3 | A patient with thoracicintradural disc herniation显示文摘 | Whitmore RG Williams BJ Lega BC | 2011 | J Clin Neurosci2011，18，12： | 1 |
| 4 | Quantitative determination of organic priority pollutants in sewage sludge by GC/MS显示文摘 | Lega R Ladwig G Meres O | 1997 | Chemosphere1997，34，8： | 1 |
| 5 | Deep brain stimulation in the treatment of refractory epilepsy:update on current data and future directions显示文摘 | Lega BC Halpem CH Jagse JL | 2010 | Neurobiol Dis2010，38，： | 1 |
| 6 | Signal modeling for low-coherence height-scanning interference microsopy显示文摘 | de Groot P de Lega X C | 2004 | Appl Opt2004，43，： | 1 |
| 7 | Association between metformin therapy and mortality after breast cancer: a population-based study显示文摘 | Lega IC Austin PC Gruneir A | 2013 | Diabetes Care2013，36，10： | 1 |
| 8 | Primary non-Hodgkin's lymphoma of the uterine cervix successfully treated by neoadjuvant chemotherapy:Case report显示文摘 | Szánthó A Bálega J J Csapó Z | 2003 | Gynecol Oncol2003，89，1： | 1 |
| 9 | Quantitative determination of organic priority pollutants in sewage sludge by GC/MS显示文摘 | Lega R Ladwig G Mercsz O | 1997 | Chemosphere1997，34，： | 1 |
| 10 | Natriuretic peptides and tro- ponins in pulmonary embolism: a meta-analysis 显示文摘 | Lega J C Lacasse Y Lakhal L | 2008 | Am J Respir Crit Care Med2008，178，8： | 1 |
| 11 | Solid - papillary tumors of the pancreas: histopathology显示文摘 | Santini D Poli F Lega S | 2006 | J Pancreas2006，7，1： | 1 |
| 12 | Quantitative determination of organic priority pollutants in sewage sludge by GC/MS 显示文摘 | Lega R | 1997 | Chemosphere1997，34，8： | 1 |
| 13 | Signal modeling for low-coherence height-scanning interference microscopy显示文摘 | Peter de Groot Xzvier Colonna de Lega | 2004 | Applied Optics2004，43，25： | 1 |
| 14 | Association Between Metformin Therapy and Mortality After Breast Cancer显示文摘 | Iliana C. Lega Peter C. Austin Andrea Gruneir Pamela J. Goodwin Paula A. Rochon Lorraine L. Lipscombe | 2013 | Diabetes Care2013，，10： | 1 |
| 15 | Severe neonatal hy- perbilirubinemia and UGT1A1 promoter polymorphism 显示文摘 | Travan L Lega S Crovella S | 2014 | J Pediatr2014，165，1： | 1 |
| 16 | Determination of fringe order in white light interference micrOscopy 显示文摘 | de Groot P de Lega X C Kramer J | 2002 | Applied Opties(S1559-128X)2002，41，22： | 1 |
| 17 | Signal modeling for low coherence height scanning interference microscopy 显示文摘 | de Groot P de Lega X C | 2004 | Applied Optics (S1559-128X)2004，43，25： | 1 |
| 18 | Characteristics, prognosis,and outcomes of cutaneous ischemia and gangrene in systemic ne-crotizing vasculitides: A retrospective multicenter study 显示文摘 | Lega JC Seror R Fassier T | 2014 | Se-min Arthritis Rheu2014，4，3： | 1 |
| 19 | HBxAg in the liver from carrier patients with chronic hepatitis and cirrhosis显示文摘 | Wang WL London WT Lega L | 1991 | Hepatology1991，14，1： | 1 |
| 20 | The Italian SEME surveillance system of severe mental disorders presenting to community mental health services显示文摘 | Gigantesco A Lega I Picardi A | | 0，，： | 1 |
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