Use of an Orthodontic and Otolaryngological Approach in an Infant with Holoprosencephaly.

Holoprosencephaly is a complex human brain malformation resulting from incomplete cleavage of the prosencephalon into both hemispheres. Congenital nasal pyriform aperture stenosis (CNPAS) is sometimes found in patients with mild forms of holoprosencephaly. Surgical treatment is required.

Temporomandibular Joint and Otitis Media: A Narrative Review of Implications in Etiopathogenesis and Treatment.

Otitis media (OM) and its recurring (rAOM), effusive (OME), and chronic forms, represent a frequent clinical challenge. The middle ear, the mandible, and the temporomandibular joint (TMJ) share several embryological and anatomical connections. Despite that, the role of mandibular malposition and TMJ dysfunction is frequently overlooked in the management of otitis media.

Association between upper airway obstruction and malocclusion in mouth-breathing children.

Objectives: To evaluate the association between upper airway obstruction and occlusal anomalies in mouth-breathing children.

Methods: 356 mouth-breathing children were evaluated by ENT physicians and specialists in orthodontics. ENT examination included nasal endoscopy to assess the adenoidal hypertrophy, tonsillar grading and presence of nasal septum deviation.

Balloon dilation and rapid maxillary expansion: a novel combination treatment for congenital nasal pyriform aperture stenosis in an infant.

Background: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare condition that may occur alone or as part of a multi-formative syndrome. Management remains difficult. There is no specific treatment protocol.

Needle aspiration of lateral neck abscess in children: A simple and effective treatment.

Background: Cervical abscesses frequently occurred in pediatric patients. Surgical drainage is currently recommended in the cases not responding to medical therapy. Needle aspiration may represent a simple, effective and minimally invasive treatment for acute neck suppuration in selected cases.

The Interplay between CD27 and CD27 B Cells Ensures the Flexibility, Stability, and Resilience of Human B Cell Memory.

Memory B cells (MBCs) epitomize the adaptation of the immune system to the environment. We identify two MBC subsets in peripheral blood, CD27 and CD27 MBCs, whose frequency changes with age. Heavy chain variable region (VH) usage, somatic mutation frequency replacement-to-silent ratio, and CDR3 property changes, reflecting consecutive selection of highly antigen-specific, low cross-reactive antibody variants, all demonstrate that CD27 and CD27 MBCs represent sequential MBC developmental stages, and stringent antigen-driven pressure selects CD27 into the CD27 MBC pool.

Deep Neck Abscesses in Children: An Italian Retrospective Study.

Background: Retropharyngeal and parapharyngeal abscesses (RPAs, PPAs) usually affect young children. Surgical drainage and/or antibiotic therapy are treatment of choice, but no specific guidelines exist. In order to reduce the risk of severe complications, appropriate diagnosis and therapy are necessary.

Juvenile recurrent parotitis: Diagnostic and therapeutic effectiveness of sialography. Retrospective study on 110 children.

Introduction: Juvenile recurrent parotitis (JRP) is characterized by recurrent episodes of painful parotid swelling, generally associated with non-obstructive sialectasia of the parotid gland. The aim of this study was to evaluate the diagnostic and therapeutic effectiveness of sialography in children affected by JRP.

Methods: Clinical records of 110 outpatients with a diagnosis of JRP followed up from 2008 to 2017 at the Unit of Paediatric Otorhinolaryngology, Surgery Department of the Bambino Gesù Children's Hospital of Rome, were retrospectively reviewed.

Dysregulated miR-155 and miR-125b Are Related to Impaired B-cell Responses in Down Syndrome.

Children with Down Syndrome (DS) suffer from immune deficiency with a severe reduction in switched memory B cells (MBCs) and poor response to vaccination. Chromosome 21 (HSA21) encodes two microRNAs (miRs), miR-125b, and miR-155, that regulate B-cell responses. We studied B- and T- cell subpopulations in tonsils of DS and age-matched healthy donors (HD) and found that the germinal center (GC) reaction was impaired in DS.

Audiological findings in a de novo mutation of ANKRD11 gene in KBG syndrome: Report of a case and review of the literature.

KBG syndrome is a rare genetic disorder, due to a mutation of ANKRD11, characterized by specific craniofacial dysmorphism, short stature and macrodontia of upper central incisors, intellectual disability and skeletal anomalies. We report a de novo mutation of ANKRD11 gene in a 7-years old girl, affected by KBG syndrome with bilateral conductive hearing loss. The aim of this article was to review the audiological findings of this syndrome.

Head and neck vascular anomalies in children.

Vascular lesions are the most common congenital and neonatal abnormalities. The aim of this work is to point out differences between various vascular anomalies, in order to define accurate diagnosis, and to present different therapeutic options now used for the treatment of the vascular lesions in children. According to biological classification described by the work of Mulliken and Glowacki there are two major types of vascular abnormality: haemangioma and vascular malformation.

Otolaryngologic manifestations of pediatric immunodeficiency.

Many adults and children with an underlying immunodeficiency can frequently present to ear, nose and throat (ENT) surgeons. This work deals with the presentation, investigation and management of immuno-compromised children in ENT practise. Both primary immunodeficiencies (PID) and secondary or acquired immunodeficiencies such as human immunodeficiency virus (HIV) infection are here discussed.

Relapsing polychondritis: new therapeutic strategies with biological agents.

Relapsing polychondritis (RP) is a rare disease of unknown etiology characterized by recurrent episodes of inflammation resulting in the destruction of cartilaginous tissues. We describe a young girl with RP unresponsive to conventional therapy.