[Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) in adults]. / Periodisches Fieber, aphthöse Stomatitis, Pharyngitis und Lymphadenitis (PFAPA-Syndrom) im Erwachsenenalter.

BACKGROUND AND OBJECTIVE: The PFAPA syndrome is characterized by a regular appearance of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis and is associated with a distinctive poor general condition of the patients. Since its first description in 1987 the syndrome has usually been described in children. But we here report its diagnosis in three adult patients. METHODS: At the Department of Otorhinolaryngology/Head and Neck Surgery of the University Medical Center Freiburg, data on 36 patients with PFAPA syndrome were collected between 2004 and 2010.  Data analysis was based on structured questionnaires, medical files of patients and a systematic assessment of the relevant international literature up to April 2011. RESULTS: The average age of the initial occurrence of PFAPA syndrome in the three patients was in the first few months after birth (month 2, 6 and 7). A tonsillectomy had been performed on all three patients in early childhood. As a result two patients had remission of symptoms for several years (4.5 and 12 years) with recurrence of PFAPA attacks while the third patient had no changes in symptoms. CONCLUSION: The PFAPA syndrome is an illness that first manifests itself in children. However, it can persist until adulthood or reappear after phases of remission. A knowledge of this periodic fever syndrome and extensive and close cooperation of general practitioners, paediatricians, internists and ENT specialists will be helpful in early diagnosis of this disorder.

Human islet distribution program for basic research at a single center.

The Clinical Islet Laboratory at the University of Alberta/Alberta Health Services distributes human islets for basic research when islet preparations fail to meet defined release criteria for transplantation. This report highlights our islet distribution activity for diabetes research over a 3-year period. Shipments of the acinar-enriched fraction for research were not included in this report. In 2010, we distributed 6.3 million islet equivalents (IEQs) of islets through 127 shipments to 8 researchers, locally, nationally, and internationally. The number of preparations for research use was stable over the 3-year period (26, 23, and 29 preparations in 2008, 2009, and 2010, respectively). Islet yield distributed for research per isolation was 201, 212, and 218 × 10(3) IEQs, respectively. The number of basic researchers was stable as well, although there were only 2 researchers before 2007. Recently, each researcher has received fewer islets per shipment (49,820 IEQs in 2010 vs 75,635 IEQs in 2008) but more frequently (21.5 in 2010 vs 11.2 times per year in 2008). This paradigm shift would be desirable for researchers, because in our experience, most require <30,000 IEQs per shipment, and more frequent islet shipments results in a larger sample size for experimentation. After an initial expansion in the number of researchers requesting islets, our islet distribution activity has remained stable over the years in terms of total productivity of islets utilized for research. The current supply-versus-demand ratio in our program appears to be appropriate.

[Benefits and importance of tonsillectomy in children and youth with PFAPA syndrome]. / Nutzen und Wertigkeit der Adeno-Tonsillektomie bei Kindern und Jugendlichen mit PFAPA-Syndrom.

BACKGROUND: PFAPA syndrome is characterized by periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. Tonsillectomy and adenotonsillectomy are surgical treatment options for this periodic fever syndrome, the significance of which will be discussed. MATERIAL AND METHODS: Between 2004 and 2010 we collected data of 36 patients with the diagnosis of PFAPA syndrome. Data analysis was carried out on the basis of structured questionnairs, patients' files as well as a systematic evaluation of international literature up to April 2011. RESULTS: The average age for the appearance of PFAPA episodes was 22 months and they recurred for an average duration of 3-5 days every 14-33 days. During a PFAPA attack, aphthous stomatitis was present in 70% of the patients, pharyngitis was present in 93% and cervical adenitis in 96%. The family history for recurrent fever was positive in 4 of the patients. In 85% cortikosteroids were the only effective medicative treatment with no further symptoms until the next attack. Surgery (tonsillectomy±adenoidectomy) aborted the PFAPA episodes in 10 of 16 patients, in 2 patients the frequency of episodes decreased, 3 patients had no noticeable change and 1 patient died as a result of postsurgical bleeding at another institution. After an average duration of illness of 4 years the PFAPA syndrome was in spontaneous remission in 8 patients. CONCLUSION: Tonsillectomy is an effective treatment option for the PFAPA syndrome. Contrary to a general indication the decision should be personalized considering the benefit vs. the risk of operation with the advice of an ENT specialist.