ADULT syndrome: dental features of a very rare condition.

The Acro-Dermato-Ungual-Lacrimal-Tooth syndrome (ADULT syndrome) is one of the rarest ectodermal dysplasias and it is associated with several malformations involving especially the limbs. The most clinical features are the presence of ectrodactyly, syndactyly, hypermelanosis or multiple lentigines, onhycodysplasia, abnormalities in the lacrimal duct, recurrent conjuntivitis, photophobia, mammarian hypoplasia, hypotrichosis and frontal alopecia, hypohydrosis, cutaneous photosensitivity, nasal bridge prominence, exfoliative dermatitis and xerosis. The ectodermal dysfunction expresses itself with conoid teeth, enamel hypoplasia, dentinal dysplasia and especially hypodontia, with following functional and aesthetic defects. We report the case of an 11-year-old Caucasian girl affected by ADULT syndrome.

The “elfin face”: craniofacial and dental aspects of the Williams-Beuren syndrome.

Williams Syndrome is a rare congenital disorder characterized by supravalvular aortic stenosis, peripheral pulmonary artery stenosis, mental retard and dysmorfic facial features. As regards the dental aspects of the syndrome, the deletion of the elastin gene induced clinicians to suspect periodontal alterations with a greater frequency of gingivo-periodontitis, but on the contrary no association between the syndrome and periodontal diseases have been found. Furthermore, patients show a higher frequency of teeth hypoplasia, an abnormal tooth morphology during primary dentition (12.5%) and during permanent dentition. We present a case report of a 12-year-old Caucasian boy affected by Williams-Beuren Syndrome who visited our hospital for a dental and orthodontic evaluation.

Oral manifestations of selective IgA-deficiency: review and case-report.

Immunoglobulin A deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA (less than 7 mg/dl) in the presence of normal levels of other immunoglobulin isotypes. Most individuals with IgA deficiency are asymptomatic and identified coincidentally. However, some patients may present with recurrent infections, allergic disorders and autoimmune manifestations, such as diabetes mellitus, Graves disease and celiac disease. The international literature has not produced any kind of review yet about intra-oral manifestations of selective IgA-deficiency. L.S., a 7-year-old Caucasian girl, was examined at our hospital. After she had undergone a professional dental cleaning, a symmetric, bilateral ulcerative gingivitis developed nearby the upper second primary molars. The gingival ulcers were persistent and did not disappear in the following 3 weeks. In the meantime, the young patient reported the presence of gastrointestinal symptoms. IgA serum level was 4.5 mg/dl, while the other isotypes levels were in the common range. The diagnosis of selective IgA-deficiency was formulated and the girl underwent further examination for the specific IgG autoantibodies in celiac disease, which were not present. Consequently, a full prevention program was planned. This case report emphasizes the role of the paediatric dentist in the early detection of systemic disorder, such as the immunological diseases. The oral cavity often reveals to be the first site of manifestation of important systemic diseases. Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency and is defined as a decrease in serum IgA levels in the presence of normal levels of other immunoglobulin isotypes (1). Serum IgA deficiency was first described in children with ataxia-telangiectasia (2) and has since been identified in other patients, including normal patients. The prevalence of IgA deficiency ranges from 1:223 to 1:1000 in community studies and from 1:400 to 1:3000 in healthy blood donors (3).

Thin Film Nanocrystalline TiO2 Electrodes: Dependence of Flat Band Potential on pH and Anion Adsorption.

Thin nanocrystalline TiO2 films were produced on ITO conductive glass by dip-coating of a sol-gel TiO2 precursor. The transparent films were characterized from the optical and structural point of view with UV-Vis, Spectroscopic Ellipsometry, Raman and X-ray photoelectron spectroscopies, the roughness of the coating by AFM. The changes in the electrochemical properties features of ITO/TiO2 electrodes were evaluated in the presence of different electrolytes (KCI, Na2SO4 and phosphate buffer) with the aim to clarify the role of the ion adsorption on the structure of the electrical double layer. Electrochemical tests (Cyclic Voltammetry, CV, and Impedance Electrochemical Spectroscopy, EIS) showed a strong influence of the electrolyte properties on the semiconductor band edge position in the electrochemical scale and on band bending. The CV profiles recorded can be explained by considering that the interface capacity is due to the charging of surface states (e.g., Ti(IV) surface sites coordinated by oxygen atoms, ≡Ti-OH or Ti-O-Ti). The surface charge is strongly affected also by the density and nature of adsorbed ions and by dissociation of surficial OH. Of interest the fact that for the produced nanocrystalline electrodes the flat band potential, measured from the Mott-Schottky analysis of the space charge layer capacity obtained with EIS, showed a non Nernstian behavior with the pH probably caused by a change in the surface acidity as a consequence of specific anion adsorption. The modulation of flat band potential with adsorbed ions is of interest for many applications, in particular for photocatalysis (change in the redox potential of photogenerated carriers) and for photovoltaic applications like DSSC (change in the photopotentials).

Alternative Augmentative Communication in paediatric dentistry: a mighty tool to amplify mother tongue's power.

AIM: The role of a child's mother tongue in shaping his/her identity and emotional development is crucial. In the context of paediatric dentistry, this principle should always be reminded. The child's right to effective communication, even when a language barrier exists, is a fundamental principle, as recently stated in the 'Rights from the Start' rights fact sheet. Preserving a child's native language in the dental setting, especially in the context of an increasingly diverse society with a significant number of refugee children, is essential. Augmentative and Alternative Communication (AAC) strategies can assist in bridging language gaps and improving treatment outcomes, blending with the traditional approaches used in paediatric dentistry. The article promotes flexibility, innovation, and empathy in paediatric dentistry to provide optimal care and ensure that every child's rights are respected. CONCLUSION: • The relationship with one's mother language plays a central role in children's growth and in the relationship they can develop with the world, "motherised" by the words of the caregiver. • The interaction between immigrant children from different linguistic and cultural backgrounds and dentists providing their care requires the integration of traditional paediatric dental techniques with AAC strategies that can compensate for deficient oral communication. • Dentists treating immigrant children should follow the suggestions proposed in this article to establish the best and most tailored paediatric setting for the child's specific needs.

Controlled Periodic Illumination Enhances Hydrogen Production by over 50% on Pt/TiO2.

Efficient solar water photosplitting is plagued by large overpotentials of the HER and OER. Even with a noble metal catalyst, the hydrogen evolution reaction can be limited by the strong M-H bonding over some metals, such as Pt, Pd, and Rh, inhibiting hydrogen desorption. H absorption is regulated by the potential at the metal nanoparticles. Through controlled periodic illumination of a Pt/TiO2 suspension, we hypothesized a fast variation of the photopotential that induced catalytic surface resonance on the metal, resulting in more than a 50% increase of the efficiency at frequencies higher than 80 Hz.

Diagnostic Salivary Tests for SARS-CoV-2.

The diagnosis of Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection relies on the detection of viral RNA by real-time reverse transcription polymerase chain reaction (rRT-PCR) performed with respiratory specimens, especially nasopharyngeal swabs. However, this procedure requires specialized medical personnel, centralized laboratory facilities, and time to provide results (from several hours up to 1 d). In addition, there is a non-negligible risk of viral transmission for the operator who performs the procedure. For these reasons, several studies have suggested the use of other body fluids, including saliva, for the detection of SARS-CoV-2. The use of saliva as a diagnostic specimen has numerous advantages: it is easily self-collected by the patient with almost no discomfort, it does not require specialized health care personnel for its management, and it reduces the risks for the operator. In the past few months, several scientific papers, media, and companies have announced the development of new salivary tests to detect SARS-CoV-2 infection. Posterior oropharyngeal saliva should be distinguished from oral saliva, since the former is a part of respiratory secretions, while the latter is produced by the salivary glands, which are outside the respiratory tract. Saliva can be analyzed through standard (rRT-PCR) or rapid molecular biology tests (direct rRT-PCR without extraction), although, in a hospital setting, these procedures may be performed only in addition to nasopharyngeal swabs to minimize the incidence of false-negative results. Conversely, the promising role of saliva in the diagnosis of SARS-CoV-2 infection is highlighted by the emergence of point-of-care technologies and, most important, point-of-need devices. Indeed, these devices can be directly used in workplaces, airports, schools, cinemas, and shopping centers. An example is the recently described Rapid Salivary Test, an antigen test based on the lateral flow assay, which detects the presence of the virus by identifying the spike protein in the saliva within a few minutes.

Primordial odontogenic tumour: A systematic review of the common but also unusual features of this novel entity.

BACKGROUND: Primordial odontogenic tumour (POT) is a novel entity that was described in 2014 and that is included in the group of benign mixed epithelial and mesenchymal odontogenic tumours. In recent years, several papers have added new cases with some clinical and histopathological aspects that slightly differ from those described in the original report. The aim of this systematic review is to update all available data on POT published in the literature and to identify those features of the neoplasm that require further investigation. MATERIALS AND METHODS: A systematic review of literature was conducted using PubMed, Embase, Web of Science and Scopus. Additional sources were also checked. Publications reporting cases with enough clinicopathological information were included, without any time or language restrictions. Histopathological or radiological studies were considered for qualitative analysis. RESULTS: A total of 30 publications were included. Seventeen papers were used for quantitative analysis while 13 papers were used only for qualitative analysis. A total of 18 cases of POT were identified. Some clinical, radiographic, histopathological and therapeutic features were common in all reported cases, while other aspects of the neoplasm were inconsistent through published cases. This inconsistency was particularly remarkable when dealing with the histopathological features of the neoplasm. DISCUSSION: Some issues about POT remain unclear and deserve to be clarified by future reports. The description of the odontogenic epithelium covering the ectomesenchyme is often contradictory, while it remains debatable whether peripheral ameloblastic epithelial islands or hard dental tissue deposition can occasionally occur within the tumour.

Biochemical control systems for small molecule damage in plants.

As a system, plant metabolism is far from perfect: small molecules (metabolites, cofactors, coenzymes, and inorganic molecules) are frequently damaged by unwanted enzymatic or spontaneous reactions. Here, we discuss the emerging principles in small molecule damage biology. We propose that plants evolved at least three distinct systems to control small molecule damage: (i) repair, which returns a damaged molecule to its original state; (ii) scavenging, which converts reactive molecules to harmless products; and (iii) steering, in which the possible formation of a damaged molecule is suppressed. We illustrate the concept of small molecule damage control in plants by describing specific examples for each of these three categories. We highlight interesting insights that we expect future research will provide on those systems, and we discuss promising strategies to discover new small molecule damage-control systems in plants.

Cataract Surgery in Patients with Keratoconus: Pearls and Pitfalls.

BACKGROUND: Keratoconus (KC) is a common ectatic disorder resulting in progressive corneal thinning and irregular astigmatism. It has been observed that patients affected by KC are more likely to develop lens opacities earlier compared to non-keratoconic patients. OBJECTIVE: Intraocular lens (IOL) selection and refractive outcome prediction are among a number of factors that can make cataract surgery in keratoconic patients challenging. Accurate biometry is often difficult to obtain due to unreliable K measurements and lack of dedicated biometric formulae. The use of toric IOLs has also been investigated. CONCLUSIONS: Determining the stage of KC, pre-operative patient counselling and the preferred method of refractive correction are all crucial to obtain successful postoperative outcomes and good patient satisfaction. The use of toric IOLs can achieve good results only in selected low-grade keratoconic eyes.

Single bubble sonochemistry: decomposition of alkyl bromide and the isomerization reaction of maleic acid.

Single bubble cavitation offers an unique opportunity to evaluate the effect of bubble activity in promoting chemical reactions. In this paper we study the isomerization reaction of maleic acid into fumaric acid using an aqueous solution of maleic acid saturated with CH2Br2. The Br* radicals are generated at the bubble surface and a whitish thread forms. For comparison, the same reaction was conducted in a sonochemical bath. A possible scheme of the reactions activated at bubble surface after the decomposition of organic brominated substances is proposed.

Outcomes of femtosecond laser-assisted mushroom-configuration keratoplasty in advanced keratoconus.

PURPOSE: To evaluate the long-term outcomes after femtosecond laser (FSL)-assisted mushroom-configuration keratoplasty in advanced keratoconus. PATIENTS AND METHODS: Thirteen eyes with Amsler-Krumeich stage IV keratoconus underwent FSL-assisted mushroom-configuration penetrating keratoplasty (PKP) and deep anterior lamellar keratoplasty (DALK) at a tertiary referral centre. Preoperative risk factors included low orneal thickness, high keratometry measurements, previous hydrops, and central stromal scarring. Main outcome measures were visual acuity and refractive outcome. RESULTS: The median follow-up was 33 months (range: 4-43). Preoperatively, the mean corrected distance visual acuity (CDVA) was 1.22±0.47 LogMAR (range: 0.5-1.9 LogMAR), mean minimum corneal thickness was 282±100.8 µm (range: 147-478 µm), and mean average keratometric (K) value was 63.4±7.63 dioptre (D; range: 57.0-75.7 D). Four patients underwent PKP and nine underwent DALK (two converted to PKP). Five patients subsequently underwent a modified arcuate mushroom interface dissection (AMID) procedure for astigmatic correction. At the final follow-up, the mean CDVA was 0.05±0.13 LogMAR (range: -0.10 to 0.20 LogMAR), mean spherical equivalent was -3.21±3.21D, mean cylindrical refractive error was 3.23±2.20 D, and mean average K was 43.1±1.53 D. Complications included early graft dehiscence, corneal vascularisation, stromal rejection, and sclerokeratitis. Sutures were completely removed at the mean 18.4 months for PKP and 9.1 months for DALK postoperatively. CONCLUSION: FSL-assisted mushroom-configuration keratoplasty is feasible and safe in patients with stage IV keratoconus. AMID could further enhance the refractive outcome safely.

Different photocatalytic fate of amido nitrogen in formamide and urea.

In the comparison of formamide and urea photocatalytic degradation, despite their similar structures, the final fate of bound nitrogen under illumination with TiO2 has shown a different behaviour; both the rate and the ratio of NH4+ and NO3- ion evolution seem not to be linked to the initial nitrogen oxidation state, but to the carbon oxidation state.

A further observation of corneal dystrophy and perceptive deafness in two siblings.

We studied two siblings with the rare association of corneal dystrophy and perceptive deafness (Harboyan syndrome). To our knowledge, this is the third description of this hereditary disorder. The results of the clinical, genetic, audiometric, and ocular examination of the two siblings and the type of inheritance, which agree with the previous description of the syndrome, are reported. Various hereditary syndromes associated with corneal dystrophy are reviewed.

Risk factors for development of post-trabeculectomy endophthalmitis.

BACKGROUND/AIMS: Although adjunctive use of antiproliferative agents improves the success rate of glaucoma filtration surgery it profoundly alters the morphology of the filtering bleb. In view of these structural changes, which have been suggested to predispose to bleb infection, the relative importance of potential risk factors in the development of post-trabeculectomy endophthalmitis was investigated. METHODS: A case-control study was performed on patients with post-trabeculectomy endophthalmitis presenting to a single academic centre over a 6 1/2 year period. Cases were diagnosed by the combination of vitreous and aqueous inflammation occurring 4 or more weeks postoperatively with control patients chosen by selecting the three patients undergoing trabeculectomy immediately following each index case. RESULTS: Analysis of these data, derived from 23 cases and 69 controls, demonstrated that an episode of blebitis and the presence of diabetes mellitus were statistically significantly associated with subsequent endophthalmitis (odds ratios (OR) 11.8, 95% CI: 2.21-88.31, p = 0.003 and OR 4.51, 95 % CI 1.02-20.29, p = 0.04 respectively). The data also suggest an association exists between antiproliferative use and endophthalmitis (OR 3.3, 95% CI 0.95-15.19, p = 0.07) as the time interval between filtration surgery and development of endophthalmitis was significantly shorter in patients treated with antiproliferative agents (p = 0.001). CONCLUSIONS: These results provide strong evidence of an increased risk of late endophthalmitis in patients who have diabetes mellitus or have had an episode of blebitis and suggest antiproliferative agents may also have an important role.

New strategies for the determination of phenylurea pesticides by gas chromatography with hot splitless inlet systems.

Direct gas chromatographic methods to analyse phenylurea pesticides are discouraged by the thermal instability of these compounds, that in conventional hot splitless inlet systems leads to extensive and irreproducible formation of isocyanates and amines. However a careful control of the operating conditions, like the inlet temperature, the pressure and the presence of suitable chemical additives (as acetic acid, low-molecular-mass amines, organic anhydrides) can either: (i) minimise the thermal decomposition enabling the direct GC-MS analysis of phenylureas, or (ii) lead to reproducible conversion to isocyanates. Experimental design was employed to study the effect of the experimental variables on the thermal transformation of phenylurea pesticides in splitless inlet system. Two strategies were alternatively optimised: (i) the minimisation of degradation reactions to increase the signal of phenylureas; (ii) the maximisation of the degradation to isocyanates that are in turn determined. The maximal yields in isocyanate were obtained with high inlet temperatures, low carrier flows in the injection phase and the presence of acetic anhydride. By contrast, the use of relatively low inlet temperatures, high carrier flows during the injection and the presence of an amine maximise the response of the parent compounds.

Phenol photonitration upon UV irradiation of nitrite in aqueous solution I: effects of oxygen and 2-propanol.

Nitrophenols are formed in aqueous solution upon UV irradiation of phenol and nitrite. The formation of nitrophenols is enhanced by dissolved oxygen and inhibited by the addition of 2-propanol. The mechanism of phenol photonitration involves both .NO2 (or N2O4, reacting with phenol, and 4-nitrosophenol, which is oxidised to 4-nitrophenol. A reaction scheme is proposed based on experimental results.

Phenol photonitration upon UV irradiation of nitrite in aqueous solution II: effects of pH and TiO2.

Phenol photonitration and photonitrosation were studied both in homogeneous and in heterogeneous phase in the presence of TiO2 particles. The effect of pH as well as of the semiconductor particles on the kinetics and products of the reaction was observed. Formation of nitrophenols is enhanced at acidic pH, due to thermal processes initiated by nitrous acid, as well as in the presence of TiO2, due to the photocatalytic oxidation of nitrite.

Formation of nitrophenols upon UV irradiation of phenol and nitrate in aqueous solutions and in TiO2 aqueous suspensions.

The formation of nitrophenols was studied as a consequence of ultra violet (UV) irradiation of aqueous solutions of phenol and nitrate in the range of pH 1-12. The study was performed both in homogeneous phase and in the presence of water-suspended TiO2. The effects of pH, dissolved oxygen and 2-propanol as .OH scavenger have been evaluated. A reaction mechanism is proposed, based on the experimental results.

What is the role of botulinum toxin in the treatment of dysthyroid strabismus?

BACKGROUND: Botulinum toxin A has been used in the treatment of dysthyroid strabismus primarily as a temporary measure during the active phase of the disease. We report on our experience with 65 patients. METHOD: We review the records of 65 patients with dysthyroid strabismus who were treated with botulinum toxin A at Moorfields Eye Hospital between 1984 and 1996. CONCLUSIONS: Patients with a short duration of relatively mild dysthyroid strabismus have a chance of long-term benefit with botulinum toxin A. There is little use for botulinum toxin A in cases of severe dysthyroid disease.