Tonsillotomy for Periodic Fever Syndrome: A Randomized and Controlled Trial.

OBJECTIVE: Tonsillectomy is an effective treatment for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Tonsillotomy has a milder operative risk profile and postoperative morbidity in children than tonsillectomy. We aimed to compare the efficacy of tonsillotomy to observation-only in children with PFAPA syndrome at a 3-month follow-up. METHODS: This was a randomized multicenter trial with sequential design. Participants were randomized into a tonsillotomy group and a control group that was only observed. The trial started in 1/2017 and was accomplished in 12/2021 with 16 patients (10 boys, six girls, the mean age 4.2 years). The symptoms were monitored with daily symptom diaries. RESULTS: After the 3-month follow-up, 7/8 patients (87.5%) in the tonsillotomy group and 2/8 (25%) patients in the control group were free from PFAPA symptoms (95% CI 13% to 87%; p = 0.0021). The mean number of days with fever was 2.6 (SD 3.7) in the tonsillotomy group and 8.0 (SD 6.5) days in the control group (n = 8) (p = 0.06). Mean number of fever days compatible with PFAPA syndrome was 0.8 (SD 1.4) in the tonsillotomy group and 6.5 (SD 6.0) in the control group (95%CI -10% to -1%; p = 0.007). Rescue tonsillectomy was needed for all patients in the control group and none of the patients in the tonsillotomy group. CONCLUSIONS: Tonsillotomy might be an effective treatment option for children with PFAPA syndrome. Further studies are needed to clarify the long-term efficacy of tonsillotomy for treating PFAPA. LEVEL OF EVIDENCE: 2 Laryngoscope, 134:968-972, 2024.

Management of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis with Intracapsular Tonsillectomy.

OBJECTIVE: This study aimed to present 2 children clinically diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and treated with intracapsular tonsillectomy with adenoidectomy (ITA). METHODS: We conducted a retrospective analysis of 2 children who were referred for an otolaryngology consultation between 2019 and 2022 for surgical treatment of PFAPA syndrome. Both patients had symptoms strongly suggestive of PFAPA and were at risk for total tonsillectomy (TT) complications. ITA was performed using a microdebrider. Both patients were followed up postoperatively to assess for symptomatic resolution and complications. RESULTS: Two children exhibited recurrent febrile episodes prior to ITA. The procedure was efficacious in both patients, with neither experiencing postoperative complications or recurring PFAPA symptoms for over 1 year after surgery. CONCLUSION: Our study reported on the use of ITA as a surgical treatment option for PFAPA. We showed that ITA eliminated febrile attacks and was safely performed without postoperative complications in 2 pediatric patients after 1-year follow-up. Future studies involving larger cohorts of PFAPA patients and lengthier follow-ups will need to be conducted to further evaluate ITA as a surgical option. Laryngoscope, 134:1967-1969, 2024.

Tonsillectomy reduces the family impact of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome and improves health-related quality of life in affected children.

BACKGROUND: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is an autoinflammatory disorder that primarily affects young children, and typically gives rise to fever episodes that recur monthly for several years. This study investigated the impact of PFAPA syndrome on the families of affected children, the health-related quality of life (HRQOL) of children with the syndrome, and how these factors were influenced by tonsillectomy. METHODS: This prospective cohort study included 24 children with typical PFAPA syndrome that were referred for tonsillectomy, of whom 20 underwent the procedure. The control group consisted of randomly selected children from the general population. Family impact and HRQOL were measured using the standardized, validated questionnaires Pediatric Quality of Life Inventory™ (PedsQL™) Family Impact Module (FIM) and PedsQL™ 4.0 Generic Core Scales (GCS). Parents to children with PFAPA completed the questionnaires before and 6 months after their child underwent tonsillectomy, and HRQOL was measured both between and during PFAPA episodes. The Wilcoxon signed-rank test was used to compare data before and after tonsillectomy in the patient group, while the Mann-Whitney test was used for comparison of the patient and control groups. RESULTS: Before tonsillectomy, children with PFAPA had significantly lower scores than the control group on the PedsQL™ FIM and the PedsQL™ 4.0 GCS during fever episodes. After tonsillectomy, all patients improved with diminished febrile episodes, which resulted in significantly higher scores regarding both family impact and HRQOL at the time of follow-up. HRQOL of in children with PFAPA improved after tonsillectomy even when compared to afebrile intervals before the procedure. The differences between PFAPA patients and controls were eliminated after tonsillectomy. CONCLUSION: PFAPA syndrome has a profound negative impact on the families of affected children. Tonsillectomy that leads to cessation or reduction of fever episodes eases the impact of the disease on the family. HRQOL in children with PFAPA is low during febrile episodes and similar to healthy controls in between episodes. The improvement of HRQOL in patients with PFAPA after tonsillectomy compared to the afebrile intervals before tonsillectomy highlights that the constantly recurring fevers may affect the children's well-being even between fever episodes.

Long-term efficacy of tonsillectomy/adenotonsillectomy in patients with periodic fever aphthous stomatitis pharyngitis adenitis syndrome with special emphasis on co-existence of familial Mediterranean fever.

Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome is one of the most common autoinflammatory fever disorders in the childhood which may co-exists with familial Mediterranean fever (FMF) causing treatment complexity. As the role of surgery in PFAPA syndrome is still controversial, in this paper, our aim is to present our results of tonsillectomy/adenotonsillectomy in the treatment of PFAPA syndrome. Archives of a tertiary care hospital were investigated for patients who underwent tonsillectomy or adenotonsillectomy due to PFAPA Syndrome between 2010 and 2020. 344 patients were found but only 281 of them were accessible. Through phone call interview and chart review methods, preoperative and postoperative the number and severity of the attacks and general satisfaction after the operation were recorded and analyzed. Also, patients with concomitant FMF were analyzed separately. A total of 281 patients were included in the study. There was no improvement in 10 (3.55%) patients. Eight (2.84%) patients showed mild improvement, 29 (10.32%) patients had moderate improvement and 234 (83.27%) patients had full recovery after tonsillectomy. There were 266 PFAPA patients without FMF. No improvement, mild improvement, moderate improvement, and full recovery in this patient group were 5 (1.9%), 6 (2.3%), 25 (9.4%) and 230 (86.5%), respectively. FMF was present in 5.33% (15/281) of the patients. In PFAPA + FMF group 5 patients had no improvement (33.3%), 2 had mild improvement (13.3%), 4 had moderate improvement (26.7%) and 4 had full recovery (26.7%). Benefit of tonsillectomy was significantly lower in the patients with concomitant FMF when compared to the patients who did not have FMF (p < 0.001). Age of diagnosis, age of operation, severity of the disease, type of operation, and gender were found to have no significant relationship with the benefit from surgery (p < 0.05). According to the findings of this study, tonsillectomy is an effective long-term treatment for PFAPA syndrome with success rate of 83.27%. Also, preoperatively FMF should be considered in these patients, which dramatically reduces surgical efficacy.

Factors Associated With Noninfectious Fever After Endovascular Aortic Aneurysm Repair.

PURPOSE: The post-endovascular abdominal aortic aneurysm repair (EVAR) inflammatory response, which is very often associated with fever, has been ascribed to a wide range of proinflammatory mediators and operative events. The aim of this study was to evaluate the impact of such factors in the development of fever of noninfectious origin after elective EVAR. MATERIALS AND METHODS: A retrospective analysis of prospectively collected data of patients treated with standard elective EVAR between February 2017 and December 2020 was undertaken. The database included patients' demographics and comorbidities, as well as laboratory inflammatory markers (white blood cell count, neutrophils, and C-reactive protein [CRP]) and anatomical characteristics (sac diameter, inferior mesenteric artery [IMA] patency and diameter, number of patent lumbar arteries, internal iliac artery [IIA] patency or occlusion). Intraoperative details, such as type of stent graft material and IIA overstenting, were also analyzed. Patients with infectious postoperative complications or previously receiving systemic anti-inflammatory medication were excluded. Statistical analysis was performed by SPSS 22.0 for Windows software (IBM Corp, Armonk, New York). RESULTS: From 332 patients treated with elective EVAR between 2017 and 2020, 268 patients (all men) were included in the analysis. The mean age was 72.1±7.5 years and the mean aneurysm diameter was 59.1±12.1 mm. Seventeen patients were excluded due to a known infection site. From the study cohort, 114 (42.5%) patients presented with fever. Multivariate regression analysis confirmed that the occlusion of IMA ≥5 mm (p<0.008) and higher CRP (p<0.001) were independent factors associated with postoperative fever. A subanalysis was performed only on patients with patent IMA before EVAR. In the multivariate regression analysis of this subgroup, IMA ≥5 mm (p=0.008), presence of dyslipidemia (p=0.037), and higher CRP (p<0.001) were related to fever. CONCLUSION: Occlusion of an existing wide (≥5 mm) and patent IMA prior to EVAR may contribute to the development of post-EVAR pyrexia. The CRP is a reliable marker for post-EVAR fever. Further prospective studies are needed to corroborate these findings.

Pre- and postoperative quality of life and emotional/behavioural problems in children with PFAPA.

OBJECTIVE: This study aimed to evaluate the effects of surgical intervention on quality of life, emotional/behavioural problems and school absenteeism in children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA.) METHOD: A total of 56 children aged between 4 and 12 years, diagnosed with PFAPA and undergoing tonsillectomy with/without adenoidectomy at a tertiary hospital were included in the study. The Questionnaire for Quality of Life Assessment for Children and Adolescents Parent Form (Kid-KINDL) and the Strengths and Difficulties Questionnaires (SDQ) were used to evaluate quality of life and emotional/behavioural problems, respectively. The cases were assessed before and three months after surgery. RESULTS: The mean (SD) age of the children was 6.64 (3.03) years, and 58.9% (n = 33) were boys. The numbers of pre- and post-operative periodic fever episodes were 3.0 (1.7) and 0.6 (0.9) (p < 0.001), those for school absenteeism were 10.28 (5.53) and 2.85 (2.95) days (p < 0.001) and those for hospital presentations were 3.8 (2.5) and 1.1 (1.1) (p < 0.001), respectively, all of which were significantly lower in children with PFAPA at three months after the operation. The surgical procedures were effective in improving quality of life and emotional/behavioural problems, as evidenced by the significant differences between the pre- and post-treatment scores in outcome measures, including SDQ and Kid-KINDL (p < 0.001). CONCLUSIONS: This study demonstrated that the surgical approach exhibited positive effects in terms of improving quality of life and reducing emotional/behavioural problems and school absenteeism in children with PFAPA.

Surgical outcomes and unique histological features of tonsils after tonsillectomy in adults with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.

OBJECTIVES: Data on the adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome are scarce. European studies reported that unlike pediatric-onset PFAPA, tonsillectomy is ineffective for adult-onset PFAPA. The aims of this study were (1) to assess the response to tonsillectomy in a cohort of Japanese adult-onset PFAPA patients and (2) to evaluate the histologic appearance of tonsils in adult-onset PFAPA patients and to compare them with those of tonsils from age- and sex-matched controls with chronic tonsillitis. METHODS: In this retrospective cohort study, 5 adults with PFAPA and 15 controls who had undergone tonsillectomy were recruited. The size of the tonsil germinal centers was measured by hematoxylin and eosin staining, and the number and density of B and T lymphocytes in germinal centers were measured by immunohistochemistry, using CD3, CD4 and CD8 as T cell markers and CD20 as B cell marker. RESULTS: All patients had complete remission of the symptoms after surgery. PFAPA patients had significantly smaller germinal center areas than controls. The number and density of CD8+ cells in germinal centers were significantly lower in tonsils from PFAPA compared with controls. No differences were found between the two groups in CD3+, CD4+, and CD20+ cells. These results are compatible with the tonsillar features of pediatric-onset PFAPA. CONCLUSION: Our report demonstrates that tonsillectomy might be effective for adult-onset PFAPA and that tonsils of adult- and pediatric-onset PFAPA share the same histological features. These results suggest that the pathogenic mechanisms of adult- and pediatric-onset PFAPA are identical.

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA).

BACKGROUND: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a rare clinical syndrome of unknown cause usually identified in children. Tonsillectomy is considered a potential treatment option for this syndrome. This is an update of a Cochrane Review first published in 2010 and previously updated in 2014. OBJECTIVES: To assess the effectiveness and safety of tonsillectomy (with or without adenoidectomy) compared with non-surgical treatment in the management of children with PFAPA. SEARCH METHODS: The Cochrane ENT Information Specialist searched the Cochrane ENT Trials Register; Central Register of Controlled Trials (CENTRAL 2019, Issue 4); PubMed; Ovid Embase; CINAHL; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The date of the search was 15 October 2019. SELECTION CRITERIA: Randomised controlled trials comparing tonsillectomy (with or without adenoidectomy) with non-surgical treatment in children with PFAPA. DATA COLLECTION AND ANALYSIS: We used the standard methodological procedures expected by Cochrane. The primary outcomes were the proportion of children whose symptoms have completely resolved and complications of surgery (haemorrhage and number of days of postoperative pain). Secondary outcomes were: number of episodes of fever and the associated symptoms; severity of episodes; use of corticosteroids; absence or time off school; quality of life. We used GRADE to assess the certainty of the evidence for each outcome. MAIN RESULTS: Two trials were included with a total of 67 children randomised (65 analysed); we judged both to be at low risk of bias. One trial of 39 participants recruited children with PFAPA syndrome diagnosed according to rigid, standard criteria. The trial compared adenotonsillectomy to watchful waiting and followed up patients for 18 months. A smaller trial of 28 children applied less stringent criteria for diagnosing PFAPA and probably also included participants with alternative types of recurrent pharyngitis. This trial compared tonsillectomy alone to no treatment and followed up patients for six months. Combining the trial results suggests that patients with PFAPA likely experience less fever and less severe episodes after surgery compared to those receiving no surgery. The risk ratio (RR) for immediate resolution of symptoms after surgery that persisted until the end of follow-up was 4.38 (95% confidence interval (CI) 0.64 to 30.11); number needed to treat to benefit (NNTB) = 2, calculated based on an estimate that 156 in 1000 untreated children have a resolution) (moderate-certainty evidence). Both trials reported that there were no complications of surgery. However, the numbers of patients randomly allocated to surgery (19 and 14 patients respectively) were too small to detect potentially important complications such as haemorrhage. Surgery probably results in a large overall reduction in the average number of episodes over the total length of follow-up (rate ratio 0.08, 95% CI 0.05 to 0.13), reducing the average frequency of PFAPA episodes from one every two months to slightly less than one every two years (moderate-certainty evidence). Surgery also likely reduces severity, as indicated by the length of PFAPA symptoms during these episodes. One study reported that the average number of days per PFAPA episode was 1.7 days after receiving surgery, compared to 3.5 days in the control group (moderate-certainty evidence). The evidence suggests that the proportion of patients requiring corticosteroids was also lower in the surgery group compared to those receiving no surgery (RR 0.58, 95% CI 0.37 to 0.92) (low-certainty evidence). Other outcomes such as absence from school and quality of life were not measured or reported. AUTHORS' CONCLUSIONS: The evidence for the effectiveness of tonsillectomy in children with PFAPA syndrome is derived from two small randomised controlled trials. These trials reported significant beneficial effects of surgery compared to no surgery on immediate and complete symptom resolution (NNTB = 2) and a substantial reduction in the frequency and severity (length of episode) of any further symptoms experienced. However, the evidence is of moderate certainty (further research is likely to have an important impact on our confidence in the estimate of effect and may change the estimate) due to the relatively small sample sizes of the studies and some concerns about the applicability of the results. Therefore, the parents and carers of children with PFAPA syndrome must weigh the risks and consequences of surgery against the alternative of using medications. It is well established that children with PFAPA syndrome recover spontaneously and medication can be administered to try and reduce the severity of individual episodes. It is uncertain whether adenoidectomy combined with tonsillectomy adds any additional benefit to tonsillectomy alone.

Clinical Characteristics and Prognostic Factors of Patients with Intrahepatic Cholangiocarcinoma with Fever: A Propensity Score Matching Analysis.

BACKGROUND: Patients with intrahepatic cholangiocarcinoma (ICC) rarely present fever as the initial symptom. We aimed to identify clinical characteristics and prognostic factors for these feverish patients. SUBJECTS, MATERIALS, AND METHODS: This study retrospectively reviewed 31 patients with ICC with fever (≥38.0°C) treated at our hospital between January 2002 and December 2014. A propensity score was used to match patients with and without fever at a ratio of 1:2. RESULTS: Patients with ICC with fever had higher serum γ-glutamyl transferase and carcinoembryonic antigen levels, larger tumors, poorer tumor differentiation, and worse prognosis (all p < .05) than those without fever. This was supported by propensity score matching (PSM) analysis. Univariate and multivariate analyses indicated that microvascular invasion, hilar lymph node metastasis, and temperature ≥ 38.6°C were related to prognosis. Patients with ICC with fever had higher levels of leucocytes, neutrophils, neutrophil-to-lymphocyte ratio (NLR), and platelet-to-lymphocyte ratio (PLR) in peripheral blood before and after PSM analysis. Body temperature positively correlated with leucocytes (r = 0.599, p < .001), neutrophils (r = 0.644, p < .001), NLR (r = 0.681, p < .001), and PLR (r = 0.457, p = .010). CONCLUSION: Patients with ICC with fever ≥38.0°C and ≥38.6°C had poor and extremely poor prognosis, respectively. Radical surgical treatment may improve the prognosis of patients with ICC with fever <38.6°C. However, systemic therapy (e.g., anti-inflammatory and immune therapy) may be preferable to surgery for these patients with fever ≥38.6°C. IMPLICATIONS FOR PRACTICE: Patients with intrahepatic cholangiocarcinoma (ICC) with fever (≥38.0°C) as the initial symptom are extremely rare. Because their symptoms are similar to those of liver abscess, diagnosis is challenging, and most of these patients are already at an advanced stage at the time of diagnosis. Patients with ICC with fever had different clinical characteristics and worse prognosis than those without fever. The prognosis of those with temperature <38.6°C would be improved by timely surgical intervention. Those with fever ≥38.6°C had an extremely dismal outcome, although they all received radical surgical treatment. New therapeutic strategies are needed to improve survival for patients with ICC with temperature ≥38.6°C.

Long-term follow-up of tonsillectomy efficacy in children with PFAPA syndrome / Acompanhamento em longo prazo da eficácia da tonsilectomia em crianças com síndrome de PFAPA

Abstract Introduction: The role of tonsillectomy in the periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome, is controversial. Although some studies reported high success rates with tonsillectomy, further investigations are needed with larger numbers of patients. Objective: To seek the long-term outcomes of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. Methods: Case series; multi-center study. The study comprised 23 patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome who underwent surgery (tonsillectomy with or without adenoidectomy) between January 2009 and November 2014. Results: 21 (91%) of 23 patients had complete resolution immediately after surgery. One patient had an attack 24 h after surgery, but has had no further attacks. One patient had three attacks with various intervals, and complete remission was observed after 3 months. Conclusions: Tonsillectomy is a good option for the treatment of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.

Resumo Introdução: O papel da tonsilectomia na síndrome da febre periódica, estomatite aftosa, faringite e adenite é controverso. Embora alguns estudos tenham relatado altas taxas de sucesso com a tonsilectomia, são necessárias mais pesquisas com um número maior de pacientes. Objetivo: Avaliar os resultados em longo prazo da tonsilectomia na síndrome de febre periódica, estomatite aftosa, faringite e adenite. Método: Série de casos; estudo multicêntrico. O estudo avaliou 23 pacientes com síndrome de febre periódica, estomatite aftosa, faringite e adenite submetidos a cirurgia (tonsilectomia com ou sem adenoidectomia) entre janeiro de 2009 e novembro de 2014. Resultados: Dos 23 pacientes, 21 (91%) apresentaram resolução completa imediatamente após a cirurgia. Um paciente apresentou um episódio 24 horas após a cirurgia, mas sem recorrência posterior. Um paciente teve três episódios com vários intervalos e a remissão completa foi observada após 3 meses. Conclusões: A tonsilectomia é uma boa opção para o tratamento da síndrome de febre periódica, estomatite aftosa, faringite e adenite.

Long-term follow-up of tonsillectomy efficacy in children with PFAPA syndrome.

INTRODUCTION: The role of tonsillectomy in the periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome, is controversial. Although some studies reported high success rates with tonsillectomy, further investigations are needed with larger numbers of patients. OBJECTIVE: To seek the long-term outcomes of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. METHODS: Case series; multi-center study. The study comprised 23 patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome who underwent surgery (tonsillectomy with or without adenoidectomy) between January 2009 and November 2014. RESULTS: 21 (91%) of 23 patients had complete resolution immediately after surgery. One patient had an attack 24h after surgery, but has had no further attacks. One patient had three attacks with various intervals, and complete remission was observed after 3 months. CONCLUSIONS: Tonsillectomy is a good option for the treatment of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.

Incidence of indications for tonsillectomy and frequency of evidence-based surgery: a 12-year retrospective cohort study of primary care electronic records.

BACKGROUND: Neither the incidence of indications for childhood tonsillectomy nor the proportion of tonsillectomies that are evidence-based is known. AIM: To determine the incidence of indications for tonsillectomy in UK children, and the proportion of tonsillectomies meeting evidence-based criteria. DESIGN AND SETTING: A retrospective cohort study of electronic medical records of children aged 0-15 years registered with 739 UK general practices contributing to a research database. METHOD: Children with recorded indications for tonsillectomy were identified from electronic medical records. Evidence-based indications included documented sore throats of sufficient frequency and severity (Paradise criteria); periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA); or tonsillar tumour. Other indications were considered non-evidence-based. The numbers of children subsequently undergoing tonsillectomy was then identified. The numbers with evidence-based and non-evidence-based indications for surgery among children who had undergone tonsillectomy were determined. RESULTS: The authors included 1 630 807 children followed up for 7 200 159 person-years between 2005 and 2016. Incidence of evidence-based indications for tonsillectomy was 4.2 per 1000 person years; 13.6% (2144/15 760) underwent tonsillectomy. Incidence of childhood tonsillectomy was 2.5 per 1000 person years; 11.7% (2144/18 281) had evidence-based indications, almost all with Paradise criteria. The proportion of evidence-based tonsillectomies was unchanged over 12 years. Most childhood tonsillectomies followed non-evidence-based indications: five to six sore throats (12.4%) in 1 year, two to four sore throats (44.6%) in 1 year, sleep disordered breathing (12.3%), or obstructive sleep apnoea (3.9%). CONCLUSION: In the UK, few children with evidence-based indications undergo tonsillectomy and seven in eight of those who do (32 500 of 37 000 annually) are unlikely to benefit.

Comorbidity of PFAPA (periodic fever, aphthous stomatitis, pharyngitis and adenitis) patients: a case control study.

OBJECTIVES: To compare the long-term morbidity of patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome treated by tonsillectomy (TE) in childhood to that of matched controls. METHODS: We identified 132 PFAPA patients from the medical records treated by TE in 1987-2007 in Oulu University Hospital, Finland. Altogether 119 patients participated the follow-up study and 94 were clinically examined on average 9.0 years after TE. The controls consisted of 230 randomly selected age-, sex-, and birth place-matched individuals from the Population Register Center of Finland. The patients and controls completed a detailed questionnaire about their current health and the data were compared. RESULTS: Self-estimated general health was good and growth was normal among PFAPA patients and controls at long-term follow-up. There were no between-group differences in the occurrence of autoimmune or other chronic diseases. Thirty percent of the PFAPA patients and 13% of the controls reported infections as causes of hospital visits during their lifetime (p<0.001). Usage of antibiotics during lifetime was reported by 99% of the PFAPA patients and by 88% of the controls (p= 0.009). Twelve percent of PFAPA patients and 0.4% of the controls reported oral thrush in their history (p=0.003). CONCLUSIONS: The health of the PFAPA patients was as good as that of healthy matched controls. Autoimmune or other chronic diseases were not more prevalent among PFAPA patients treated with TE in childhood than among controls. Respiratory infections and oral thrush were more common among the PFAPA patients than controls.

Aortofemoral Reconstruction for an Infected Graft Using Thrombosed Femoral Veins.

INTRODUCTION: Treatment of an infected aortic prosthesis is difficult and the ideal graft material is subject to debate. REPORT: A case of infected aortic prosthesis treated using bilateral thrombosed superficial femoral veins (SFVs) is presented. Bilateral reversed SFVs were cut longitudinally at both proximal ends about 3-4 cm and were sutured side by side. The operating time was 5 h. No sign of recurrent infection was observed when the patient suffered a myocardial infarction and died 6 months post-operatively. DISCUSSION: Thrombosed SFVs may be considered as a therapeutic option for infected aortic graft replacement.

Noma as a complication of false teeth (Ebiino) extraction: a case report.

BACKGROUND: Ebiino, also known as false tooth extraction, is a traditional practice done mainly in the remote areas of African countries, including Uganda. It involves the extraction of tooth buds in babies with common childhood illnesses such as fever, cough, and diarrhea. It is thought that the tooth buds are responsible for the ailments seen in these infants. The practice is performed by traditional healers using unsterile instruments. The complications associated with this dangerous practice have been mentioned in the literature and include anemia and septicemia, among others. This case report describes a baby with noma, an orofacial gangrenous infection. CASE PRESENTATION: A 16-month-old girl from western Uganda belonging to the Banyankole ethnic group was admitted to Mbarara University Teaching Hospital with a 5-day history of a dark lesion on the left cheek. The lesion had started from the left upper gum at the site where a tooth bud had been extracted 1 week prior to admission. The child had experienced occasional cough and fever and also had erupting tooth buds. These tooth buds had been seen as the cause of the cough and fever by the traditional herbalist; hence, they were extracted. An unsterile instrument had been used for the procedure. At the hospital, a local examination showed necrotic tissue involving the left cheek and extending into the left upper gingival area of the girl's mouth. A clinical diagnosis of orofacial gangrene (noma) was then made. CONCLUSIONS: Ebiino, or false tooth extraction, is still practiced in some remote areas of Uganda. Noma has been mentioned as a possible complication of this traditional practice; however, case reports in the literature are scant. Public awareness of the dangers of this practice is therefore still required to prevent this dangerous complication.

Long-Term Outcome of Classic and Incomplete PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) Syndrome after Tonsillectomy.

OBJECTIVE: To compare the effectiveness of tonsillectomy and the long-term outcome of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome in patients fulfilling the classic diagnostic criteria and in those with regularly recurring fever as the only symptom or with onset of symptoms after age 5 years or both. STUDY DESIGN: We reviewed the medical records of 3852 children who underwent tonsillectomy between 1990 and 2007 and identified 108 children who did so because of regularly recurring fevers. The patients were invited to an outpatient visit and were classified into 2 groups: those who met (N = 58) and those who did not meet (N = 50) Thomas diagnostic criteria. We then compared the clinical profile and outcome of PFAPA symptoms after tonsillectomy between the 2 groups. RESULTS: In the group that met Thomas criteria, 97% (56/58) had complete resolution of fever episodes after tonsillectomy; in the group that did not meet Thomas criteria (50/50) had complete resolution of fever episodes after tonsillectomy (P = .25). The clinical profile of the periodic fevers and the occurrence of other illnesses during follow-up were similar in both groups. Thomas criteria identified 56 of 106 patients responding to tonsillectomy. CONCLUSIONS: Tonsillectomy was an effective treatment for patients with regularly recurring fever episodes who failed to meet the classic Thomas criteria. We suggest that PFAPA syndrome should be suspected and tonsillectomy considered in children with a late onset of symptoms (>5 years of age) or when fever is the only symptom during the episodes.

Microbiological analysis of bile in patients with benign and malignant biliopancreatic diseases and its consequences / Análise microbiológica da bile em pacientes com doenças biliopancreáticas benignas e malignas e suas consequências

ABSTRACT Background Bactibilia has several consequences to human health. Objetive Assessing the bile microbiology of patients with biliopancreatic diseases in order to identify bacteria and their possible infectious complications. Methods Retrospective study of 30 bile culture samples from patients with benign and malignant biliopancreatic diseases. The samples were assessed to set the bile microbiological flora and to search for its possible link with comorbidity, carcinogenesis and postoperative infectious complications. Results Thirty bile samples from patients at mean age ≈57.7 years, mostly female (n=18), were assessed. Bactibilia was found in 12 cases, mostly in patients with benign diseases (n=8), older than 50 years (n=23) and female (n=10). Adenocarcinoma of the duodenal papilla (n=9) and cholelithiasis (n=8) were the most common diseases. Escherichia coli (n=5) and Klebsiella sp (n=3) were predominantly found in patients with benign diseases; and Klebsiella sp (n=2) and Streptococcus sp (n=2) were prevalent in cancer patients. There were postoperative infectious complications in seven cases, five of them in bactibilia-associated patients (P=0.084). Conclusion Bactibilia was found in 12 samples and Escherichia coli and Klebsiella sp were most often identified in patients with benign diseases, as well as Streptococcus sp and Klebsiella sp in cancer patients. There was a trend of higher postoperative infectious complication incidence in patients with bactibilia.

RESUMO Contexto Bacteriobilia pode produzir várias consequências para a saúde humana. Objetivo Avaliar a microbiologia da bile de pacientes com doenças biliopancreáticas para identificar bactérias e possíveis consequências. Métodos Estudo retrospectivo microbiológico. Trinta amostras de bile de pacientes com doenças biliopancreáticas benignas e malignas foram avaliadas para determinar a flora microbiológica da bile e procurar alguma possível relação dessa com comorbidades, carcinogênese e complicações infecciosas pós-operatórias. Resultados As amostras de bile foram avaliadas em pacientes, com idade média ≈57,7 anos, a maioria mulheres (n=18). Evidenciou-se bacteriobilia em 12 casos, a maioria em pacientes com doenças benignas (n=8); pacientes com mais de 50 anos (n=23) e mulheres (n=10). As doenças mais comuns foram o adenocarcinoma de papila duodenal (n=9) e a colelitíase (n=8). Escherichia coli (n=5) e Klebsiella sp (n=5) foram as bactérias mais identificadas em pacientes com doenças benignas; sendo a Klebsiella sp (n=2) e o Streptococcus sp (n=2) as que predominaram nos pacientes com cânceres. As complicações pós-operatórias exclusivamente infecciosas ocorreram em sete casos, sendo em cinco desses associados à bacteriobilia (P=0,084). Conclusão Bacteriobilia foi evidenciada em 12 amostras, sendo as bactérias mais identificadas Escherichia coli e Klebsiella sp em pacientes com doenças benignas; e Streptococcus sp e Klebsiella sp nos pacientes com câncer. Existiu uma tendência a maior incidência de complicações infecciosas pós-operatórias em pacientes com bacteriobilia.

MICROBIOLOGICAL ANALYSIS OF BILE IN PATIENTS WITH BENIGN AND MALIGNANT BILIOPANCREATIC DISEASES AND ITS CONSEQUENCES.

BACKGROUND: Bactibilia has several consequences to human health. OBJETIVE: Assessing the bile microbiology of patients with biliopancreatic diseases in order to identify bacteria and their possible infectious complications. METHODS: Retrospective study of 30 bile culture samples from patients with benign and malignant biliopancreatic diseases. The samples were assessed to set the bile microbiological flora and to search for its possible link with comorbidity, carcinogenesis and postoperative infectious complications. RESULTS: Thirty bile samples from patients at mean age ≈57.7 years, mostly female (n=18), were assessed. Bactibilia was found in 12 cases, mostly in patients with benign diseases (n=8), older than 50 years (n=23) and female (n=10). Adenocarcinoma of the duodenal papilla (n=9) and cholelithiasis (n=8) were the most common diseases. Escherichia coli (n=5) and Klebsiella sp (n=3) were predominantly found in patients with benign diseases; and Klebsiella sp (n=2) and Streptococcus sp (n=2) were prevalent in cancer patients. There were postoperative infectious complications in seven cases, five of them in bactibilia-associated patients (P=0.084). CONCLUSION: Bactibilia was found in 12 samples and Escherichia coli and Klebsiella sp were most often identified in patients with benign diseases, as well as Streptococcus sp and Klebsiella sp in cancer patients. There was a trend of higher postoperative infectious complication incidence in patients with bactibilia.

Guidelines for the management and treatment of periodic fever syndromes: periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome.

OBJECTIVE: To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. DESCRIPTION OF THE EVIDENCE COLLECTION METHOD: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. RESULTS: 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. RECOMMENDATIONS: 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development. 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes. 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients. 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA. 5. Despite promising results obtained in studies with IL-1ß inhibitors, such studies are limited to a few case reports.