Oral health related quality of life in cleft lip and palate patients rehabilitated with conventional prostheses or dental implants

Cleft lip and/or palate (CLP) is the most common congenital craniofacial abnormality, with a prevalence of 9.92 per 10,000 live births. In treating patients with CLP, oral rehabilitation is definitely a very important phase of the treatment in order to improve the patient's oral health related quality of life (OH-QoL). The aim of this retrospective study is to assess the OH-QoL in patients rehabilitated with different prosthetic options, thus comparing the conventional treatments, which include removable partial dentures and fixed partial dentures, with the implant-supported prostheses

Medication-related osteonecrosis of the jaw

In 2014, the nomenclature of bisphosphonate-related osteonecrosis of the jaw (BRONJ) was changed in medication-related osteonecrosis of the jaw (MRONJ) to include osteonecrosis of the jaw caused by non-bisphosphonates (BPs) drugs. MRONJs are a rare drug adverse reaction associated with BPs and other antiresorptive (denosumab) and antiangiogenetic therapies. MRONJ pathophysiology is not completely elucidated, and three risk factors should be considered: Local factors, underlying disease and kind of medication. MRONJ aff ects considerably patient’s quality of life, so it is important to know pathology and risk factor in order to prevent or treat immediately the disease. Various BRONJ staging systems are used by clinicians: In 2006 Ruggero at al. proposed a clinical staging system with three diff erent levels based on signs and symthoms; in 2009 American Association of Oral and Maxillofacial Surgeons implemented it with Stage 0. Marx in 2007 was the only one who divided the stages on the basis of the lesion’s size. Bedogni in 2012 proposed a clinical-radiological staging system. The aim of this review is to summarize the current diagnosis, prevention and treatment strategies

Survival of short dental implants ≤7 mm: A review

The first long-term successful outcome of short dental implants was demonstrated by Frieberg et al. in 1991, however, the definition of “short” implants is still controversial and without uniform consensus nowadays. The specific aim of this review was to evaluate and to compare cumulative survival rate (CSR) of short dental implants of the two groups. The survival rate of short dental implants was the primary outcome variable to be extracted and analyzed. An electronic search was conducted through the Medline (PubMed) database of the National Library of Medicine, and EMBASE to find all relevant articles published between January 1, 1990, and April 30, 2015. The electronic search identified 347 publications, which were all carefully screened by title and abstract. About 65 articles qualified for a thorough full-text analysis: 35 studies were excluded because CSR% was not calculable. Finally, 30 studies with relevant data on CSR were selected to be included in this review. Articles were divided into two groups: All relevant articles published between 1991 and 2000 as Group 1 and between 2001 and 2015 as Group 2. In Group 1 CSR was 83.53% ± 19.46%, a considerable statistically significant difference compared to 93.65% ± 7.94% of Group 2. This review further identified the causes of failure: In Group 1 the majority of short implant failures occurred early, within the first 4 months, for an insufficient quantity of bone tissue. In Group 2, causes of early failures considered were low bone quality while prosthetic reasons were responsible for delayed failures

DiGeorge-like Syndrome in a Child with a 3p12.3 Deletion Involving MIR4273 Gene Born to a Mother with Gestational Diabetes Mellitus

Chromosome 22q11.2 deletion is the most common chromosomal alteration associated with DiGeorge syndrome (DGS), even though this is not the only underlying cause of DGS. In rare patients, mutations in a single gene, TBX1, have been described resulting in a DGS phenotype. Recently, it has been reported that at least part of the TBX1 mutant phenotype is due to excessive bone morphogenetic proteins (BMP) signaling. Evidence suggests that miRNA may modulate the expression of critical T-box transcriptional regulators during midface development and Bmp-signaling. We report on a 7-year-old Caucasian male born to a mother affected with gestational diabetes (GDM) who had a 371Kb-interstitial deletion of 3p12.3 identified by array CGH, involving the ZNF717, MIR1243 and 4273 genes. The child presented with a DiGeorge anomaly (DGA) associated with unilateral renal agenesis and language delay. The immunological evaluation revealed a severe reduction and impairment of T lymphocytes. FISH analysis and TBX1 sequencing were negative. Among the miRNA-4273 predicted target genes, we found BMP3, which is involved in several steps of embryogenesisincluding kidney and lung organogenesis and in insulin gene expression. Since DGA is not commonly found in newborns of diabetic mothers, we hypothesize that the pathogenesis of DGA associated with GDM is multifactorial, involving both genetic and/or epigenetic cofactors

The Evolutionary Scenario of Pediatric Unclassified Primary Antibody Deficiency to Adulthood

background: unclassified primary antibody deficiency (unPAD) is a relatively novel inborn error of immunity (IEI) condition that can vary with time to more defined entities. since long-term follow-up (FU) studies are scarce, we aimed to provide insight into the evolutionary clinical and immunological scenario of unPAD children to adulthood and identification of biomarkers of primary immune deficiency (PID) persistence. methods: a total of 23 pediatric unPAD patients underwent clinical and immunological FU for a mean time of 14 years (range 3-32 years, median 16 years). results: UnPAD diagnosis may change over time. at the last FU, 10/23 (44%) children matched the diagnosis of transient hypogammaglobulinemia of infancy and 13/23 (56%) suffered from a persistent PID. In detail, an unPAD condition was confirmed in 7/23 (30%) patients, whereas 3/23 (13%), 2/23 (9%), and 1/23 (4%) were reclassified as common variable immunodeficiency, selective IgA deficiency, and isolated IgM deficiency, respectively. Low IgA, low specific antibody response to pneumococcus, and lower respiratory tract infections at diagnosis were independently associated with IEI persistence. conclusions: long-term monitoring of unPAD patients is required to define their outcome and possible evolution towards a definitive IEI diagnosis

The Inborn Errors of Immunity—Virtual Consultation System Platform in Service for the Italian Primary Immunodeficiency Network: Results from the Validation Phase

purposeInborn errors of immunity (IEI) represent a heterogeneous group of rare genetically determined diseases. In some cases, patients present with complex or atypical phenotypes, not fulfilling the accepted diagnostic criteria for IEI and, thus, at high risk of misdiagnosis or diagnostic delay. this study aimed to validate a platform that, through the opinion of immunologist experts, improves the diagnostic process and the level of care of patients with atypical/complex IEI.MethodsHere, we describe the functioning of the IEI-Virtual consultation system (VCS), an innovative platform created by the italian Immunodeficiency network (IPINet). resultsIn the validation phase, from January 2020 to June 2021, 68 cases were entered on the IEI-VCS platform. a final diagnosis was achieved in 35/68 cases (51%, 95% CI 38.7 to 64.2). In 22 out of 35 solved cases, the diagnosis was confirmed by genetic analysis. In 3/35 cases, a diagnosis of secondary immunodeficiency was made. In the remaining 10 cases, an unequivocal clinical and immunological diagnosis was obtained, even though not substantiated by genetic analysis. conclusionFrom our preliminary study, the VCS represents an innovative and useful system to improve the diagnostic process of patients with complex unsolved IEI disorders, with benefits both in terms of reduction of time of diagnosis and access to the required therapies. these results may help the functioning of other international platforms for the management of complex cases

Surgical approach to malformation of maxillary central incisor following trauma to its predecessor. Two case reports

In the case reports, two different approaches have been described to treat the developmental disturbances in the maxillary central incisors due to trauma to its predecessor. The treatment plan was chosen according to the type and severity of the malformations, the exact location and the morphology of the involved teeth. In the first case, the disimpaction of the maxillary right central incisor was achieved with the combined of surgical and orthodontic therapy, that was planned in two consecutive stages. In the second case the severe root angulation and the failure of the previous orthodontic traction made impossible the repositioning of the upper right central incisor, which was surgical removed