Pyostomatitis vegetans in a pediatric patient with ulcerative colitis: case report of a rare pediatric inflammatory bowel disease extraintestinal manifestation and review of the literature.

Inflammatory bowel diseases can occur with a wide range of extraintestinal symptoms, among which pyostomatitis vegetans, that is a rare but almost pathognomonic finding. We report the case of a 9-year-old female patient affected by ulcerative colitis and recently treated for an oral candidiasis, who experienced a relapse in her ulcerative colitis (PUCAI 50), preceded by the return of whitish lesions in the oral cavity, initially misdiagnosed and unsuccessfully treated as candidiasis and then recognized as pyostomatitis vegetans. Bloody diarrhea was treated with oral beclomethasone, with rapid remission of ulcerative colitis and disappearance of pyostomatitis vegetans. After 2 years, ulcerative colitis is in sustained remission with oral mesalamine and pyostomatitis vegetans has not recurred. Pyostomatitis vegetans is considered a marker of ulcerative colitis relapse among adult population, and although very few pediatric cases are described, it is very important to remember it in the differential diagnosis of the oral manifestations and comorbidities of inflammatory bowel diseases.

Airway Clearance Management with Vaküm Technology in Subjects with Ineffective Cough: A Pilot Study on the Efficacy, Acceptability Evaluation, and Perception in Children with Cerebral Palsy.

Background: In the medical complexity of a children with cerebral palsy, impaired airway clearance represents a major problem, leading to significative respiratory morbidity and mortality. Its management is difficult because of limited cooperation and poor tolerance to invasive treatments. Free Aspire® (FA) is a device designed to remove bronchial secretions noninvasively, without generating cough. Methods: The aim of our pilot prospective study is to assess the efficacy and acceptability of FA in removing airway obstruction in subjects with cerebral palsy and ineffective cough. We enrolled 11 subjects. At enrollment and after 3, 6, and 12 months, we collected data regarding health care resources use for respiratory exacerbations, perceived efficacy, and acceptability of treatment. Results: We observed a reduction in emergency room (ER) accesses, home pharmacological treatment, hospitalizations number, and hospital stay length. In particular after 12 months we observed a reduction of 74% in ER accesses and home pharmacological treatment, 38% in hospitalizations number, and 17% in hospital stay length for respiratory exacerbations. The 100% of caregivers considered the treatment effective and simple to use and noted an improvement in subjects' general condition. They also reported good treatment tolerance of subjects, with an overall good compliance. Conclusion: The study demonstrates that FA is an effective device for the removal of bronchial secretions, with a positive caregivers' perception, that favored a good long-term compliance.

A New Formulation of an Old Drug: A Potential New Therapy in the Management of Oral cGvHD.

Oral chronic graft versus host disease (cGVHD) is often refractory to systemic therapies. Additional topical treatment is commonly required. The potency of the agent, the vehicle and formulation in which it is delivered are all critical factors in determining the effectiveness of topical therapies. High potency of budesonide, combined with its very low bioavailability when absorbed through mucosal surfaces, increased the potential role in topical application for oral cGVHD. Viscous formulation increases mucosal contact time resulting in a greater decrease in mucosal inflammation. This short communication suggests that oral viscous budesonide should be considered as a potential new therapy in the management of oral cGVHD.