Marco Romagnoli

Effectiveness of adenotonsillectomy in PFAPA syndrome: a randomized study.

OBJECTIVE To evaluate whether adenotonsillectomy leads to complete resolution in children with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome. STUDY DESIGN Thirty-nine children with PFAPA syndrome were randomized to either adenotonsillectomy (surgery group; n = 19) or expectant management (control group; n = 20). All patients were then invited prospectively to record all PFAPA episodes, and were evaluated clinically every 3 months for 18 months after randomization. RESULTS The proportion of patients experiencing complete resolution was 63% in the surgery group and 5% in the control group (P < .001). The mean (+/- standard deviation) number of episodes recorded during the study period was 0.7 +/- 1.2 in the surgery group and 8.1 +/- 3.9 in the control group (P < .001). The episodes were less severe in the surgery group. CONCLUSIONS Adenotonsillectomy is an effective treatment strategy for children with PFAPA syndrome.

Hypersaline nasal irrigation in children with symptomatic seasonal allergic rhinitis: a randomized study.

Recent evidence suggests that nasal irrigation with hypertonic saline may be useful as an adjunctive treatment modality in the management of many sinonasal diseases. However, no previous studies have investigated the efficacy of this regimen in the prevention of seasonal allergic rhinitis‐related symptoms in the pediatric patient. Twenty children with seasonal allergic rhinitis to Parietaria were enrolled in the study. Ten children were randomized to receive three‐times daily nasal irrigation with hypertonic saline for the entire pollen season, which had lasted 6 weeks. Ten patients were allocated to receive no nasal irrigation and were used as controls. A mean daily rhinitis score based on the presence of nasal itching, rhinorrea, nasal obstruction and sneezing was calculated for each week of the pollen season. Moreover, patients were allowed to use oral antihistamines when required and the mean number of drug assumption per week was also calculated. In patients allocated to nasal irrigation, the mean daily rhinitis score was reduced during 5 weeks of the study period. This reduction was statistically significantly different in the 3th, 4th and 5th week of therapy. Moreover, a decreased consumption of oral antihistamines was observed in these patients. This effect became evident after the second week of treatment and resulted in statistically significant differences during the 3th, 4th and 6th week. This study supports the use of nasal irrigation with hypertonic saline in the pediatric patient with seasonal allergic rhinitis during the pollen season. This treatment was tolerable, inexpensive and effective.

Nasal Rinsing with Hypertonic Solution: An Adjunctive Treatment for Pediatric Seasonal Allergic Rhinoconjunctivitis

Background: Recent but limited evidence suggests that nasal lavage with hypertonic saline may be useful as an adjunctive treatment modality in the management of pediatric allergic rhinitis. The aim of this study was to clarify whether nasal irrigation with hypertonic solution should be routinely recommended to children with seasonal grass pollen rhinoconjunctivitis. Methods: Fourty-four children with seasonal grass pollen rhinoconjunctivitis were recruited. Twenty-two patients were random ized to receive three-times daily nasal rinsing with hypertonic saline during the pollen season, which lasted 7 weeks. Twenty-two patients were allocated to receive no nasal irrigation and were used as controls. Twenty patients per group completed the study. A mean daily rhinoconjunctivitis score based on the presence of nasal discharge and obstruction as well as ocular symptoms as reddening and itching were calculated for each week of the pollen season. Patients were allowed to use oral antihistamines when required and the mean number of drugs taken per week was also calculated. Results: The mean weekly rhinoconjunctivitis score in the active group was reduced during the whole pollen period. This difference was statistically significant in week 6 and 7 of therapy. A markedly reduced intake of oral antihistamines was also observed in patients allocated to nasal rinsing, being statistically significant in 5 of the 7 weeks. No adverse effect was reported in the active group. Conclusions: This study supports the use of nasal rinsing with hypertonic saline in the pediatric patient with seasonal allergic rhinoconjunctivitis. This treatment proved to be tolerable, inexpensive and effective.

Incidence of Unexpected Malignancies in Routine Tonsillectomy Specimens in Children

Objectives: Controversy continues to exist regarding the necessity to routinely send for histologic examination those specimens obtained after tonsillectomy with or without adenoidectomy in children. Otolaryngologists fear missing an unsuspected diagnosis, such as a tonsil malignancy. However, given the rare incidence of this event, the cost‐effectiveness ratio of routine microscopic analysis is questionable. The purpose of this study was to assess the incidence of clinically relevant unexpected diagnosis among children who underwent tonsillectomy with or without adenoidectomy in our units and to review current available series on this topic.

Enzymatic replacement therapy for Hunter disease: Up to 9 years experience with 17 patients

Hunter disease is an X-linked lysosomal storage disorder characterized by progressive storage of glycosaminoglycans (GAGs) and multi-organ impairment. The central nervous system (CNS) is involved in at least 50% of cases. Since 2006, the enzymatic replacement therapy (ERT) is available but with no effect on the cognitive impairment, as the present formulation does not cross the blood–brain barrier. Here we report the outcome of 17 Hunter patients treated in a single center. Most of them (11) started ERT in 2006, 3 had started it earlier in 2004, enrolled in the phase III trial, and 3 after 2006, as soon as the diagnosis was made. The liver and spleen sizes and urinary GAGs significantly decreased and normalized throughout the treatment. Heart parameters improved, in particular the left ventricular mass index/m2 decreased significantly. Amelioration of hearing was seen in many patients. Joint range of motion improved in all patients. However, no improvement on respiratory function, eye, skeletal and CNS disease was found. The developmental quotient of patients with a CNS involvement showed a fast decline. These patients were no more testable after 6 years of age and, albeit the benefits drawn from ERT, their quality of life worsened throughout the years. The whole group of patients showed a consistent residual disease burden mainly represented by persistent skeletal disease and frequent need of surgery. This study suggests that early diagnosis and treatment and other different therapies which are able to cross the blood–brain barrier, might in the future improve the MPS II outcome.

The intriguing association between prematurity and neonatal isolated submandibular suppurative sialadenitis.

Submandibular suppurative sialadenitis occurring as an isolated lesion in the neonatal period is extremely rare. A case of a preterm newborn, who developed an isolated submandibular suppurative sialadenitis is described. A literature review highlighting salient points on this topic is also presented. In particular, an important role of prematurity in the etiology of this pathological condition is shown and discussed.

Lymphomas of head and neck in pediatric patients

Cancer among children is relatively uncommon, with approximately 1 in 7,000 children 0 to 14 years of age being newly diagnosed each year in the United States, and Hodgkin and non-Hodgkins lymphomas constitute 10-15% of all childhood cancers in the more developed countries, after acute leukemias and brain tumors. The diagnosis of lymphoma frequently involves otolaryngologists that play also an important role in the its management. A high index of suspicion for lymphoma as a cause of complaints in the head and neck region can lead an early diagnosis and improved outcome for lymphomas. This article reviews the epidemiology, presentation, diagnosis, staging, treatment and prognosis of Hodgkin and non-Hodgkins lymphomas in children.

Expression of cell cycle regulatory proteins and analysis of apoptosis in normal nasal mucosa and in nasal polyps

Background The etiopathogenesis of nasal polyps still is to be clarified. Although hyperplasia is a typical feature of these pathological processes, little attention has been paid to specific aspects of cellular growth in polyps. We have evaluated the expression and localization of some of the regulatory proteins that direct the cell through the specific sequence of events culminating in mitosis or apoptosis in nasal polyps. Methods Twenty samples of nasal polyps and 20 samples of normal nasal mucosa have been analyzed for apoptotic index by detecting the DNA 3’ OH ends deriving from DNA fragmentation. Moreover, they have been evaluated by immunohistochemical staining for expression of Ki-67, cyclins A and B1, p53, p21, p27, murine double minute clone 2, and Bcl-2. Results We have identified a greater proportion of proliferating cells in the lining epithelial cells of the polyps when compared with the normal mucosa as stained with anti–Ki-67 antibodies. An overexpression of p53, MDM2, and Bcl-2 and an increased apoptosis were observed in nasal polyps compared with the normal mucosa, whereas no variation of p27 expression was observed. The p21 and cyclins A and B1 were rarely expressed in both pathological and normal tissue. Conclusion The p53-based control system of cell cycle progression appears to be altered in nasal polyps, potentially leading to an abrogation of the DNA damage checkpoint. Evaluation of the expression of the regulatory proteins that direct the cells throughout their cycle in nasal polyps may allow a better understanding of the biological behavior and clinical outcome of these benign pathological entities.

Adenoidal hypertrophy and allergic rhinitis

Editor, We read with interest the review article by Scadding G. entitled ‘Non-surgical treatment of adenoidal hypertrophy: The role of treating IgE-mediated inflammation’ (1). The association between adenoidal hypertrophy (AH) and allergic rhinitis cannot be debated, and we agree that the use of intranasal corticosteroids (INS) should be the first line treatment for AH and ought to be recommended before the surgical option. As reported, INS suppress inflammatory mediators involved in the allergic reaction; for this reason it is of primary importance to discover the allergen/s responsible for the allergic inflammation (the etiologic agent). For many years, it has been well known that house dust mites are the main cause of intermittent allergic rhinopathy (2), however the article in question does not mention the relevance of hypersensitivity to mites in children. The author hypothesized the importance of allergic reaction to mold even if Alternaria and other molds are responsible for only few cases of allergic rhinitis (AR) in children (3). Today, the role of mites in AR is probably underestimated since symptoms are often much less evident than those caused by hypersensitivity to pollens. Nevertheless, the minimal persistent inflammation present in patients without symptoms, who are sensitized to mites and continuously exposed to the natural allergens, provokes long-term complications such as AH. Moreover, skin prick tests using commercially available allergen extracts often give a false negative result. A recent publication reported that immunochemical analysis of mite extracts produced by different manufacturers showed a heterogeneous amount of component/s. For this reason, the Authors suggested that the absence of relevant allergens could strongly affect diagnosis in some of the patients tested (4). In order to avoid this problem, in our clinical practice, patients affected by AH are firstly treated with nasal rinsing (hypertonic solution), INS and oral antihistamines. After 3 weeks, the majority of patients show a reduction in nasopharyngeal cavity volume obstruction (an example of rhinofibroscopy before and after treatment is reported in Fig 1) and a decrease in clinical symptoms of AH. These patients subsequently undergo first level allergic tests (skin test and serum specific IgE production test). If these prove to be negative, a second-line diagnosis is carried out with a specific nasal provocation test (5) in order to evaluate a possible association between AH and allergy. Treatment for allergic diseases includes allergen avoidance, pharmacotherapy, allergen specific immunotherapy and patient education. Therefore, we might add to the conclusion of the review article that it is very important to define the responsible allergens and to consider specific immunotherapy in order to change the natural history of the disease, in particular when mites are the causative agent. Treatment of allergic disease should be considered in the non-surgical treatment of AH options to avoid prolonged treatment with INS.