Orofacial Anomalies in Kindler Epidermolysis Bullosa.

Importance: Kindler epidermolysis bullosa is a genetic skin-blistering disease associated with recessive inherited pathogenic variants in FERMT1, which encodes kindlin-1. Severe orofacial manifestations of Kindler epidermolysis bullosa, including early oral squamous cell carcinoma, have been reported.

Objective: To determine whether hypoplastic pitted amelogenesis imperfecta is a feature of Kindler epidermolysis bullosa.

Clinical practice guidelines: Oral health care for children and adults living with epidermolysis bullosa.

Background: Inherited epidermolysis bullosa (EB) is a genetic disorder characterized by skin fragility and unique oral features.

Aims: To provide (a) a complete review of the oral manifestations in those living with each type of inherited EB, (b) the current best practices for managing oral health care of people living with EB, (c) the current best practices on dental implant-based oral rehabilitation for patients with recessive dystrophic EB (RDEB), and (d) the current best practice for managing local anesthesia, principles of sedation, and general anesthesia for children and adults with EB undergoing dental treatment.

Methods: Systematic literature search, panel discussion including clinical experts and patient representatives from different centers around the world, external review, and guideline piloting.

Knowledge about hand hygiene in the Generation Z: A questionnaire-based survey among dental students, trainee nurses and medical technical assistants in training.

Alcohol-based hand disinfection is the key measure to prevent healthcare-associated infections and nosocomial transmission of pathogens. We conducted a survey among dental students, trainee nurses and medical technical assistants in training from the Generation Z to determine their knowledge regarding hand hygiene and to optimize education. Overall knowledge was worthy of improvement.

Variable disease progression after successful stem cell transplantation: prospective follow-up investigations in eight patients with Hurler syndrome.

We report the results of a prospective, standardized follow-up programme of eight children (median age at SCT 1.2 yr) with mucopolysaccharidosis (MPS1H, M. Hurler) transplanted using a fludarabine-based SCT.

Shear bond strength of self-etch adhesives to enamel with additional phosphoric acid etching.

This study evaluated the shear bond strength of self-etch adhesives to enamel and the effect of additional phosphoric acid etching. Seventy sound human molars were randomly divided into three test groups and one control group. The enamel surfaces of the control group (n=10) were treated with Syntac Classic (SC).

Identification of a homozygous deletion in the AP3B1 gene causing Hermansky-Pudlak syndrome, type 2.

We report on the molecular etiology of an unusual clinical phenotype associating congenital neutropenia, thrombocytopenia, developmental delay, and hypopigmentation. Using genetic linkage analysis and targeted gene sequencing, we defined a homozygous genomic deletion in AP3B1, the gene encoding the beta chain of the adaptor protein-3 (AP-3) complex. The mutation leads to in-frame skipping of exon 15 and thus perturbs proper assembly of the heterotetrameric AP-3 complex.

Effect of different splinting methods and fixation periods on root development of autotransplanted immature third molars.

Purpose: The aim of the present study was to assess the influence of suture splinting for 1 week or rigid fixation for 4 weeks on final root length, root length increment, and mobility of autotransplanted immature third molars.

Patients And Methods: The sample consisted of 63 patients with a total of 65 transplanted immature third molars. All transplants had reached one-half (n = 29) to three-fourths (n = 36) of their expected root length.

Autotransplantation of immature third molars: influence of different splinting methods and fixation periods.

Different data were published documenting the influence of fixation methods and periods on the outcome of autotransplantations of teeth. Besides studies reporting increased ankylosis and disturbances of pulpal revascularization following rigid or extended fixation, there are studies revealing no connection in this matter. The clinical and radiological results of 76 transplanted germs of third molars were to be assessed after a rigid fixation for 4 weeks or after a suture splinting for 7 days and compared with each other.