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Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome is an inflammatory disorder of childhood classically characterized by recurrent fevers, pharyngitis, stomatitis, cervical adenitis, and leukocytosis. While the mechanism is unclear, previous studies have shown that tonsillectomy can be a therapeutic option with improvement in quality of life in many patients with PFAPA, but the mechanisms behind surgical success remain unknown. In addition, long-term clinical follow-up is lacking. In our tertiary care center cohort, 62 patients with PFAPA syndrome had complete resolution of symptoms after surgery (95.3%). Flow cytometric evaluation demonstrates an inflammatory cell population, distinct from patients with infectious pharyngitis, with increased numbers of CD8+ T cells (5.9% vs. 3.8%, p < 0.01), CD19+ B cells (51% vs. 35%, p < 0.05), and CD19+CD20+CD27+CD38-memory B cells (14% vs. 7.7%, p < 0.01). Cells are primed at baseline with increased percentage of IL-1β positive cells compared to control tonsil-derived cells, which require exogenous LPS stimulation. Gene expression analysis demonstrates a fivefold upregulation in IL1RN and TNF expression in whole tonsil compared to control tonsils, with persistent activation of the NF-κB signaling pathway, and differential microbial signatures, even in the afebrile period. Our data indicates that PFAPA patient tonsils have localized, persistent inflammation, in the absence of clinical symptoms, which may explain the success of tonsillectomy as an effective surgical treatment option. The differential expression of several genes and microbial signatures suggests the potential for a diagnostic biomarker for PFAPA syndrome. [ABSTRACT FROM AUTHOR]

Introduction: Recurrent salivary gland swelling of the parotid and submandibular glands results in painful swelling in the pediatric population. There is no defined algorithm for workup and treatment of these disorders, resulting in wide heterogeneity and in some cases overuse of computed tomography (CT) imaging. Sialendoscopy (SE) is an interventional option for recurrent swelling of both glands; however, its effectiveness in the pediatric population is still being determined. Objectives: To assess preoperative imaging utilization and benefit in the workup of recurrent pediatric sialadenitis, intraoperative SE findings, and postoperative outcomes after intervention with SE. Methods: Case-series with a 5-year retrospective chart review on children undergoing SE for recurrent sialadenitis. Results: Forty-nine SE procedures were performed on 38 parotid glands (PG) and 11 submandibular glands (SMGs) in 29 children. CT imaging findings were useful for identifying a stone or stricture and guiding surgical management in 45.5% of SMGs versus 2.6% of PGs (P < .001). A stone was found in 45.5% of SMGs and none in PG (P < .001). SE intervention such as balloon dilation or stone removal was performed in 54.6% of SMGs and 5.3% of PGs (P < .001). 74% of parotid patients undergoing SE responded to 1 intervention with a cessation of recurrent gland swelling, while 26% required additional interventions. One hundred percent of SMG patients responded to first intervention. There was no improvement in the beneficial effect of SE with steroid injection (P = .897) regardless of steroid used (P = .082). Conclusion: CT findings were found to be low yield for recurrent parotid swelling, and ultrasound is a recommended first-line step for PG pathology. SE is a recommended first-line intervention for SMG and parotid sialadenitis as demonstrated by 100% and 74% response rate to initial SE, respectively. [ABSTRACT FROM AUTHOR]

Recurrent respiratory papillomatosis (RRP) is mainly caused by human papillomavirus (HPV) 6 and 11. While various adjuvant therapies have been reported, no effective therapy has been documented to universally "cure" this disease. In the era of precision medicine, it would be valuable to identify effective intervention based on drug sensitivity testing and/or molecular analysis. It is essential to be able to successfully carry out in vitro culture and expand tumor cells directly from patients to accomplish this goal. Here we report the result of successful culture of HPV-infected cell lines (success rate 70%, 9/13) that express the E6/E7 RNA transcript, using pathologic tissue biopsies from patients treated at our institution. The availability of such a system would enable ex vivo therapeutic testing and disease modeling. [ABSTRACT FROM AUTHOR]

Recurrent respiratory papillomatosis (RRP) is a potentially lethal disease caused by the human papillomavirus (HPV) and characterized by recurrent papillomas in the aerodigestive tract. BEST PRACTICE SUMMARY Administration of systemic bevacizumab earlier in the RRP disease process may provide the benefit of decreased disease progression in addition to minimizing surgical sequelae. Currently, the mainstay of RRP treatment is via surgical intervention, with a minority of patients requiring adjuvant medical therapies during their disease. [Extracted from the article]

The article lists people who contributed to the reviewing of articles for the 2013 issue of the periodical "The Laryngoscope" including Mona Abaza, Jean Abitbol and Oliver Adunka.

Objectives/Hypothesis: To develop a national database of cases of juvenile onset recurrent respiratory papillomatosis (JoRRP) in Canada, to calculate trends in incidence and prevalence of JoRRP from January 1994 to December 2007 at the national and regional level, and to mathematically model the natural history of JoRRP. Study Design: Retrospective, multicenter study. Methods: Patient demographics, clinical presentation, human papillomavirus status, method and timing of treatment, and indicators of disease severity were captured with a standardized case report form. Operative records were retrospectively scored using the Derkay-Coltrera staging system for each operative intervention. Trends in incidence and prevalence of JoRRP from 1994 to 2007 were calculated at a national and regional level using national population census data. A multivariable mixed effects linear model was used to explore the effect of surgery-specific variables on the intersurgical interval. Nonlinear least-squares regression was used to model the natural history of JoRRP. Results: Development of a national database of children with JoRRP identified 243 cases who underwent 3,021 surgical procedures. The national incidence of JoRRP from 1994 to 2007 was 0.24 per 100,000 children aged 14 years and younger. The prevalence was 1.11 per 100,000 children. The natural history of JoRRP followed a nonlinear time course with 64% of cases having a decreasing annual rate of surgery over time. Conclusions: A Canadian national database of children with JoRRP was successfully developed. Modeling of the natural history of JoRRP may have important clinical and research implications. Laryngoscope, 2010 [ABSTRACT FROM AUTHOR]

Objectives: Recurrent respiratory papillomatosis is currently the most common lesion of the larynx in children. The course of the disease is variable and often requires repetitive surgical interventions to maintain the airway. The predominant concern for disease progression is the possibility of spread to the tracheobronchial tree, as this increases the rates of morbidity and mortality. Cidofovir is an antiviral drug with activity against members of the DNA virus family. Development of local malignant change secondary to use of cidofovir has been a concern. The histopathologic findings from biopsy specimens from children treated with cidofovir have not been previously reported. Methods: We performed a retrospective review of pediatric operative histologic biopsies and charts of patients treated with intralesional cidofovir and untreated study controls from January 1, 1995, through November 1, 2001. Results: Ninety-six specimens were evaluated by 2 blinded pathologists. No cases of dysplasia were identified. The most commonly identified finding was an increased nucleus-to-cytoplasm ratio in 8 of 95 cases (8.4%). No cases of abnormal mitoses, prominent nucleoli, or cellular or nuclear enlargement were found. Conclusions: This is the first report of pathologically evaluated recurrent respiratory papillomatosis specimens taken before and after treatment with intralesional cidofovir. No dysplasia was identified, and there were no significant dysplastic changes in the specimens analyzed. [ABSTRACT FROM AUTHOR]

Objective: To report preliminary results regarding the safety and efficacy of the 585-nm pulsed-dye laser (PDL) for the treatment of juvenile-onset recurrent respiratory papillomatosis (JORRP) in the pediatric population. Design: Prospective longitudinal cohort study. Setting: Two pediatric otolaryngology referral centers. Patients: Twenty-three pediatric patients ranging in age from 6 months to 17 years. Interventions: The 585-nm PDL was used for at least 1 treatment on each of these patients to treat JORRP of the true vocal folds or anterior commissure. Main Outcome Measures: Complications from the use of the 585-nm PDL in the treatment of JORRP. Results: There was no evidence of anterior commissure webbing or true vocal fold scarring in this group of 23 patients followed up for 3 months to 1 year. Conclusions: The 585-nm PDL seems to be a safe instrument for treatment of JORRP. There is the potential that improved voice outcomes may be apparent when compared with traditional therapies because the vocal fold epithelium seems to be unharmed when treated with this method. Furthermore, the lack of epithelial damage incurred by the 585-nm PDL should enable more aggressive surgical excision of anterior commissure disease. Further prospective longitudinal studies examining voice outcomes are needed. [ABSTRACT FROM AUTHOR]

Background: Circumscribed oral melanotic macules in adults are a well-defined entity. Congenital oral melanotic macules, however, are rare and not well characterized. We report 5 cases of congenital lingual melanotic lesions with consistent clinical and histologic features. Observations: Congenital lingual melanotic macules presented in all cases at birth, followed by proportional growth. Clinical findings were well-circumscribed, brown, single or multiple macules on the tongue, 2 to 5 mm in size, without history of bleeding, ulceration, or trauma. Family history was negative for similar pigmented lesions. A literature review showed only 2 previous clinical case reports with similar features. Histopathologic examination showed increased melanin pigmentation in the basal epidermal layer with varying degrees of overlying hyperkeratosis and subepidermal pigment-laden macrophages. No appreciable increase in melanocyte number, junctional nests of melanocytes, or cell atypia was noted. Two retrospective histopathology reviews mention 4 corresponding cases but report no clinical data. Conclusion: The congenital lingual melanotic macule represents a clinically distinct, benign, pigmented oral melanotic lesion, and may be more common than the literature suggests.s [ABSTRACT FROM AUTHOR]

Objective: To describe and to determine the robustness of our study evaluating the efficacy of OK-432 (Picibanil) as a therapeutic modality for lymphangiomas. Design and Setting: Prospective, randomized trial and parallel-case series at 13 US tertiary care referral centers. Subjects: Thirty patients diagnosed as having lymphangioma. Ages in 25 ranged from 6 months to 18 years. Twenty-nine had lesions located in the head-and-neck area. Intervention: Every patient received a 4-dose injection series of OK-432 scheduled 6 to 8 weeks apart unless a contraindication existed or a complete response was observed before completion of all injections. A control group was observed for 6 months. Outcome Measures: Successful outcome of therapy was defined as a complete or a substantial (>60%) reduction in lymphangioma size as determined by calculated lesion volumes on computed tomographic or magnetic resonance imaging scans. Results: Overall, 19 (86%) of the 22 patients with predominantly macrocystic lymphangiomas had a successful outcome. Conclusions: OK-432 should be efficacious in the treatment of lymphangiomas. Our study design is well structured to clearly define the role of this treatment agent. [ABSTRACT FROM AUTHOR]

The article presents a discussion among physicians concerning thermometry in special pediatric populations. The panel included, Dr. Barbara Kelly, Clinical Assistant Professor of Pediatrics at the University of Pennsylvania and Dr. Seth Pransky, Assistant Clinical Professor of the Department of Surgery at the UCSD Medical Center among others. A study by Dr. Weiss regarding rectal temperatures in newborns, Weiss highlighted that in many nurseries, it is standard to take the initial temperature through the rectum, in part, to rule out an obstruction of the anal area. There was some suggestion after several studies, that perhaps in the pre-term population, doctors could evaluate babies who had central lines to figure out some way of getting a central measurement on those babies and perhaps obtain better data.

People who the author would like to thank for their assistance in the creation of the periodical "Pediatric Dermatology" are mentioned.