Diagnosis and management of acquired aplastic anemia in childhood. Guidelines from the Marrow Failure Study Group of the Pediatric Haemato-Oncology Italian Association (AIEOP).

Acquired aplastic anemia (AA) is a rare heterogeneous disorder characterized by pancytopenia and hypoplastic bone marrow. The incidence is 2-3 per million population per year in the Western world, but 3 times higher in East Asia. Survival in severe aplastic anemia (SAA) has improved significantly due to advances in hematopoietic stem cell transplantation (HSCT), immunosuppressive therapy, biologic agents, and supportive care. In SAA, HSCT from a matched sibling donor (MSD) is the first-line treatment. If a MSD is not available, options include immunosuppressive therapy (IST), matched unrelated donor, or haploidentical HSCT. The purpose of this guideline is to provide health care professionals with clear guidance on the diagnosis and management of pediatric patients with AA. A preliminary evidence-based document prepared by a group of pediatric hematologists of the Bone Marrow Failure Study Group of the Italian Association of Pediatric Hemato-Oncology (AIEOP) was discussed, modified and approved during a series of consensus conferences that started online during COVID 19 and continued in the following years, according to procedures previously validated by the AIEOP Board of Directors.

Impact of mother donor, peripheral blood stem cells and measurable residual disease on outcomes after haploidentical hematopoietic cell transplantation with post-transplant cyclophosphamide in children with acute leukaemia.

Haploidentical hematopoietic-cell transplantation using post-transplant cyclophosphamide(Haplo-PTCy) is a feasible procedure in children with haematologic malignancies. However, data of a large series of children with acute leukaemia(AL) in this setting is missing. We analysed 144 AL Haplo-PTCy paediatric recipients; median age was 10 years. Patients had acute lymphoblastic(ALL; n = 86) or myeloblastic leukaemia(AML; n = 58) and were transplanted in remission(CR1: n = 40; CR2: n = 57; CR3+: n = 27) or relapse (n = 20). Bone marrow was the graft source in 57%; donors were father (54%), mother (35%), or sibling (11%). Myeloablative conditioning was used in 87%. Median follow-up was 31 months. At day +100, cumulative incidence (CI) of neutrophil recovery and acute GVHD (II-IV) were 94% and 40%, respectively. At 2-years, CI of chronic GVHD and relapse, were 31%, 40%, and estimated 2-year overall survival (OS), leukaemia-free survival (LFS) and graft-versus-host-relapse-free survival (GRFS) were 52%, 44% and 34% respectively. For patients transplanted in remission, positive measurable residual disease (MRD) prior to transplant was associated with decreased LFS (p = 0.05) and GRFS (p = 0.003) and increased risk of relapse (p = 0.02). Mother donor was associated with increased risk of chronic GVHD (p = 0.001), decreased OS (p = 0.03) and GRFS (p = 0.004). Use of PBSC was associated with increased risk of chronic GVHD (p = 0.04). In conclusion, achieving MRD negativity pre-transplant, avoiding use of mother donors and PBSC as graft source may improve outcomes of Haplo-PTCy in children with AL.

Guided endodontic microsurgery in apicoectomy: a review.

The purpose of this review was twofold. The first aim was to show predictable protocols of guided endodontic microsurgery (EMS) and to describe its clinical outcomes. The second aim was to verify the accuracy of this technique compared to the traditional pathway. An electronic search of publications was established from two electronic databases, Cochrane and PubMed, by two independent researchers. The search strategy used a combination of controlled vocabulary and free-text words. Inclusion and exclusion criteria were defined by the authors before the start of the study. Inclusion criteria were: all studies published in English language; studies in vivo on humans; human cadaver studies; studies analyzing guided surgery in apicoectomy. The initial search yielded 67 citations, of which 10 were included. The studies included in this review analysis have shown two different technique both for digitization of dental arch and for surgical procedures too. Guided endodontic micro-surgery is a more predictable technique with less intra-operative and post-operative complications compared to the "freehand" technique and this procedure has shown a greater accuracy.

Different renal manifestations associated with very early onset pediatric inflammatory bowel disease: case report and review of literature.

BACKGROUND: Inflammatory bowel diseases are characterized by chronic inflammation of the gastrointestinal tract. In particular, Crohn disease and ulcerative colitis represent the two most common types of clinical manifestations. Extraintestinal manifestations of inflammatory bowel diseases represent a common complications, probably reflecting the systemic inflammation. Renal involvement is reported in 4-23% of cases. However, available data are limited to few case series and retrospective analysis, therefore the real impact of renal involvement is not well defined. CASE PRESENTATION: We report the case of a 10-years old male affected by very early onset unclassified-Inflammatory bowel diseases since he was 1-year old, presenting with a flare of inflammatory bowel diseases associated with acute kidney injury due to granulomatous interstitial nephritis. Of interest, at 7-year-old, he was treated for IgA nephropathy. To our knowledge, no previous reports have described a relapse of renal manifestation in inflammatory bowel diseases, characterized by two different clinical and histological phenotypes. CONCLUSIONS: The link between the onset of kidney injuries with flares of intestinal inflammation suggest that nephritis maybe considered an extra-intestinal manifestation correlated with active inflammatory bowel disease. However, if granulomatous interstitial nephritis represents a cell-mediated hypersensitivity reaction than a true extraintestinal manifestation of inflammatory bowel diseases is still not clarified. We suggest as these renal manifestations here described may be interpreted as extraintestinal disorder and also considered as systemic signal of under treatment of the intestinal disease.

The effectiveness of subgingival irrigant ozone-based as adjuvant for non-surgical periodontal therapy in the treatment of chronic periodontitis: a review.

The aim of this review was to establish the efficacy of Ozone therapy (OT) in Periodontal disease's (PD) tratment. An electronic search of publications was established from two electronic databases: PubMed and Web of Science. The search strategy used a combination of controlled vocabulary and freetext words. Inclusion and exclusion criteria were defined by the authors before the start of the study. The inclusion criteria were: all studies published in English language; studies in vivo on humans; studies analyzing the correlation between PD and OT. The search resulted in 102 titles. Only 9 articles were included in the quantitative analysis. By the analyzed studies a strictly connection is still not evincible. More clinical trials are recommended to investigate the role of ozone, in its various types of topical administration, as a valid subgingival irrigant to achieve the goal of periodontal tissue health.

Rehabilitation of a severe mandibular atrophy with four millimeter extra-short implant and guided bone regeneration (GBR): case report with 7-years follow-up.

The aim of this study was to report the use of extra-short and short implants in combination with Guided Bone Regeneration (GBR) to rehabilitate a case of severe mandibular reabsorption. A 55-yearold female patient asked for a fixed implant supported rehabilitation in 4.5 - 4.6 zone which showed severe atrophy. It was decided to use an extra-short 4 mm length and 4.1 mm Ø, Roxolid and SLActive implant (Straumann Standard Plus Regular Neck, Institut Straumann AG) in 4.5 position and a short 6 mm length and 4.8 mm Ø, Roxolid and SLActive implant (Straumann Standard Plus Wide Neck, Institut Straumann AG) in 4.6 position. The implants supported cemented fixed prosthesis. Examinations were performed at the day of the surgery and up to 7-years in function to evaluate implant stability and periapical radiography. One extra-short implant and one short implant were placed. After 7 years in function no biological or prosthetic complication were recorded. Within the limitation of this case report, the use of short and extra-short implants to support fixed prosthesis seem to be a feasible treatment alternative in severe mandibular atrophy.

Comparison of the accuracy of intraoral scanning and conventional impression techniques on implants: a review.

The aim of this review was to collect available evidence and evaluate accuracy outcomes of dental implant impression techniques, and to compare the accuracy of conventional implant impression versus digital implant impression. Inclusion and exclusion criteria were defined by the authors before the start of the study. The inclusion criteria were: all studies published in English language; studies no older than five years; analyzing the accuracy of digital vs conventional technique impression on implants. The exclusion criteria were: publications that reported the same data as later publications by the same authors and systematic reviews; commentaries and letters to the editor; case report and case series. The search resulted in 106 titles. Following the first stage of screening, after the records identification through database manual searching, 112 potentially relevant studies were identified. After the second stage screening, 33 full text publications were obtained and analyzed and 17 were excluded. Afterwards, 18 articles resulted eligible after full text reading and a cross search of the articles' references was accomplished; 3 articles were consequently added. At the end only 7 articles were included in the quantitative analysis. Within the limitations of this systematic review, digital impression on dental implants offers a comparable accuracy compared with conventional impressions technique. More clinical trials are recommended to investigate the accuracy of these scanners and their validity in clinical use.

Fit evaluation of cad/cam fabricated all-ceramic restorations based on direct and indirect digitalization in vivo: a systematic review.

The aim of this study was to compare the fit of all-ceramic restorations on natural teeth fabricated through a direct digital workflow or an indirect digital workflow. An electronic search of publications was established from three electronic databases: Cochrane, PubMed and Web of Science. The search strategy used a combination of controlled vocabulary and free-text words. The detailed search design and strategies, including keywords, are presented below. The authors used two filters to follow data for the research: papers written in English and published in the last 5 years. The search resulted in 3042 titles. Following the first stage of screening, after the records identification through database manual searching, 3047 potentially relevant studies were identified. After the second stage screening, 38 full text publications were obtained and analyzed and 17 were excluded. Afterwards, 22 articles resulted eligible after full text reading and a cross search of the articles references was accomplished and 5 articles were consequently added. At last, 6 articles were included in the quantitative analysis. This study was designed to compare the fit of restorations obtained by means of a direct or indirect digital workflow. The values reported on the maximum acceptable gap in scientific literature range from 50 to 200 µm, so there does not seem to be an objective limit based on scientific evidence. According to the most accepted marginal discrepancy in the literature, most of the values of the studies examined are in the 200 µm acceptability range. Within the limitations of this systematic review, computer-aided design and computer-aided manufacturing (CAD/CAM) fabricated restorations obtained by means of an intraoral scanner (IOS) showed better marginal and internal fit than restorations obtained through conventional impression and subsequent laboratory scanning. According to the results of this systematic review, the direct digital workflow resulted as a valid alternative to the indirect digital workflow to produce CAD/CAM all-ceramic restorations.

Radiosurgery in dentistry: a review.

The aim of the present study was to analyse the clinical potential of radiosurgery applied to modern dentistry through a revision of the indexed literature. Radiosurgery represents a recent form of electrosurgery working at a frequency of 3.0-4.0 MHz. The depth of penetration of the waves is inversely proportional to the frequency. Radiowaves working at high frequency express a lower penetration with a consequent lower tissue alteration. The authors analyzed the literature in order to give to the scientific community an overview of the possible applications of radiosurgery in the clinical dental practice and their effectiveness. Radiosurgery can be used in prosthodontics, in periodontal and implant surgery, in the treatment of trigeminal neuralgia, in the treatment of obstructive sleep apnea syndromes (OSAS) and in endodontics. Our analysis shows that although there are few articles in indixed literature on the applications of radiosurgery in dentistry, thanks to the benefits that derive from it, radiosurgery will spread more in the daily clinical practice of the dentists.

Influence of implant scanbody material and operator on scanning fluency and polygonal mesh numbers of digital impression: an in vitro study.

The purpose of this study is to evaluate the influence of implant scanbody (ISB) material and operator on scanning fluency and polygonal mesh numbers of a confocal microscopy intraoral scanning (IOS). An edentulous maxillary master model with 6 implant analogues was assembled. Thereafter 3 ISBs featured with same geometry but different materials (polyetheretherketone (PK), titanium (T) and PK with a titanium base (PKT)) were produce and scanned according to a randomized sequence by three different operators. The confocal microscopy IOS resulted in 45 STL test files that were processed to a dedicate software to obtain the request data. The overall analysis of fluency (imm/sec) showed the following results: mean 11.997 imm/sec, SD 2.355. The multivariate analysis showed statistical significance of material (p<.0001) and operator (p<.0001) influence. The univariate analysis referred to polygonal mesh numbers expressed the following results: mean 30327.8, SD 2432.5. The multivariate analysis stressed how there is a related effect to materials (p<.0001) and operator (p=0.0205). PKT represented the material with the best results for both. Analyzing the overall distribution histogram for scanning fluency, the IOS device seems to work with higher frequency at level of 13 imm/sec. The distribution of the measurements in the reference histograms showed an effect related to both variables referring to scanning fluency as well as to polygonal mesh numbers. Operator can be considered as a random effect; regarding the materials, the difference between PKT and PK can be related to the different base material. The dissimilar optical properties of T justify the different behavior.

Radiosurgery-assisted gingival displacement: a proof of concept technique to enhance the efficiency of intraoral digital impression.

Nowadays, an increasing number of dentists are using intraoral scanners (IOS) in their daily practice as an alternative to conventional impression taking. One of the main concerns is related to the capability of scanning the subgingival anatomy of the die, usually very challenging due to the limited operative field and the presence of oral fluids. The radiosurgery assisted gingival displacement technique (RAGD) may enhance the intraoral optical scanning of the finish line and neighbor tooth anatomy. The contour of the interim prosthesis is used to drive the tip of radiosurgery electrode along the tooth surface and open selectively the gingival crevice with a prosthetically-driven and minimally invasive approach. The clinical implication of this technique is related to increasing the efficiency and accuracy of the digital impression technique in the critical zone of the prosthetic shoulder.

Marfan Syndrome: oral implication and management.

Marfan's Syndrome is a multisistemic pathology of connective tissues, a dominant autosomal transmission, first discovered by a French pediatrician, Antoine Bernard-Jean Marfan, who first found in some of his patients a disproportionate alteration of inferior infertility. This alteration was caused by the mutation of the FBN1 gene, located on the long arm of the chromosome 15, which encodes for an extracellular matrix protein, fibrin-1. Later it was discovered that the disease could occasionally be due also to the mutation of the TGFBR2 gene, which encodes for a TGF-beta receptor 1. The estimated incidence of the disease is 2-3 subjects affected every 10,000, in the absence of predilection ratial, ethnic, geographic and gender. It is believed that some 15,000 people in Italy suffer from Marfan Syndrome. The disease is characterized by a wide range of clinical manifestations that affect different organs. The study evaluates through a literature review the manifestations in the oral cavity of the marfan syndrome and the correct management of the patient during dental maneuvers.

In vitro evaluation of the post-space depth reading with an intraoral scanner (IOS) compared to a traditional silicon impression.

OBJECTIVES: The aim of the study was to assess the depth and quality of the post-space reading, using an IOS without scan-post, compared to a traditional silicon technique. METHODS: Six extracted bicuspids were decoronated and endodontically treated. After having prepared the space for the posts, a structure in pink acrylic resin was created with two resin elements. At the center of the structure one sample was put at a time. Digital and traditional impressions were taken for each sample.Digital impressions were developed through the Computer-aided design (CAD) software in order to integrate the scanner results into a three-dimensional grid to make the measurements. A K-file was used to measure the length of the post-space of each sample obtained through the traditional silicon impression and subsequently the measurement results were reported on a millimeter gauge. Furthermore, an assessment of the width of the entrances of the post-spaces was carried out. RESULTS: The mean reading depth discrepancy expressed in percentages (19.58%) indicates that the digital impression with current technologies fails to impress clearly the post-space. Standard deviation of the data expressed in percentage is 13.89, suggesting that the values were not similar to each other. In two cases the digital technique has achieved less than 10% difference compared to the traditional technique, but there have been also cases in which the variation in depth has reached almost 40%.The samples that showed the minor discrepancy between the two techniques expressed the widest post-space entrance. CONCLUSIONS: In this in vitro study, the application of the IOS for the post-space reading in order to deliver an anatomic post has been proven to be still not reliable, as there are still depth reading limitations for the narrow root channels. In fact, in this type of channels it is difficult to reach with the light beam of the IOS the deepest areas of the post-space, with a consequent incomplete post-space reading.

Smart fusion vs. double scan: a comparison between two data-matching protocols for a computer guided implant planning.

AIM: To make a comparison between two different approaches of data matching during the preparation of a computer guided implant planning. MATERIAL AND METHODS: Electronic and manual literature searches were performed to collect information about the double-scan protocol and the smart fusion protocol. The two systems were then tested to identify or confirm their advantages and disadvantages. RESULTS: The double scan protocol is a reliable and well-known process to obtain a fusion between the anatomical data (CBCT) and the prosthetic data (radiographic template). It is possible to use this protocol both in dentate and edentulous patients. The newly developed smart fusion technique offers the possibility to superimpose the anatomical data (CBCT) onto the prosthetic data (cast + wax-up scan) without the production of a radiographic template. This system is still being tested by the clinicians even though dental manufacturers already consider it as the best solution for the dentate patients selected for a static computer guided implant surgery. CONCLUSIONS: Smart fusion protocol offers a reasonable time and cost reduction even though its application is limited to dentate patients. A noticeable drawback of the workflow is the matching step: often a manual intervention is necessary to obtain a correct alignment of the CBCT data with the lab scan of the cast. This issue is partially due to the use of non-volume stable materials during the preparation of the cast. Future improvements could be made by combining CBCT data with direct optical scans of patient dental arches in order to create the so-called 'virtual patient'.

A novel workflow for computer guided implant surgery matching digital dental casts and CBCT scan.

Nowadays computer-guided "flap-less" surgery for implant placement using stereolithographic tem-plates is gaining popularity among clinicians and patients. The advantages of this surgical protocol are its minimally invasive nature, accuracy of implant placement, predictability, less post-surgical discomfort and reduced time required for definitive rehabilitation. Aim of this work is to describe a new protocol (Smart Fusion by Nobel Biocare), thanks to which is now possible to do a mini-invasive static guided implant surgery, in partially edentulous patients with at least 6 remaining teeth, without the use of a radiographic guide. This is possible thanks to a procedure named surface mapping based on the matching between numerous points on the surface of patient's dental casts and the corresponding anatomical surface points in the CBCT data. The full protocol is examined focusing the attention on the clinical and laboratory procedures. CONCLUSIONS: Also with some critical points and needing an adequate learning curve, this protocol allows to select the ideal implant position in depth, inclination and mesio-distal distance between natural teeth and or other implants enabling a very safe and predictable rehabilitation compared with conventional surgery. It represents a good tool for the best compromise between anatomy, function and aesthetic, able to guarantee better results in all clinical situations.

Oral complications of head and neck radiotherapy: prevalence and management.

AIM: The aim of the study was to evaluate the short-term and long-term toxicity caused by radiation treatment in the head and neck with the technique of intensity-modulated radiotherapy (IMRT). METHODS: We selected 20 patients, 18 men and 2 women aged between 21 and 71 years, undergoing radiation therapy (IMRT) in head and neck. Patients were visited during radiotherapy and followed for six months after the end of the therapy. We assessed the presence of: mucositis, xerostomia, dysgeusia, dysphagia, pain, trismus and, in the case of late-onset complications, radiation cavities. RESULTS: Acute toxicity: in 20 patients, 18 reported mucositis, 19 xerostomia, 17, dysgeusia, 15 dysphagia, 18 had pain and 3 patients had trismus. Tardive toxicity: in 14 patients, 5 reported mucositis, 11 xerostomia, 6 dysgeusia, 2 dysphagia, 3 had pain, 4 trismus and in 4 patients were found radiation cavities. CONCLUSION: Acute complications with higher prevalence were xerostomia (19 of 20 patients), dysgeusia of 2nd grade (11 patients of 20), mucositis of 1st grade and pain of 1st grade (10 patients of 20). Among the late complications it was noted a maintenance of the high prevalence of xerostomia (11 patients of 14) and an increase in prevalence of trismus (4 patients of 14) against a reduction of all other complications. The presence of radiation cavities in 4 patients of 14 was also recorded.

Combined orthodontic-surgical approach in the treatment of impacted maxillary canines: three clinical cases.

Impaction of maxillary canine is a relatively frequent orthodontic anomaly which could represent fuctional and aesthetic problems for patients. Nowadays, the conventional technique to impacted canines consists of a combined orthodontic and surgical approach, aimed to guide cuspids at the center of the alveolar ridge in a stable position and surrounded by healthy hard and soft tissues. This article presents three cases studies with different combined surgical-orthodontic approaches for the treatment of infraosseous impacted canines. An impacted maxillary canine could be guided, after adequate space is created orthodontically, to the center of the ridge through an orthodontic traction directly applied to the crown of impacted cuspid. Several surgical techniques have been proposed to expose the crown of impacted tooth. Location (buccal or palatal side) of impactation and depth influence surgical approach in order to obtain best aesthetic and functional results.

Dental photography today. Part 1: basic concepts.

This paper is the first article in a new series on digital dental photography. Part 1 defines the aims and objectives of dental photography for examination, diagnosis and treatment planning, legal and forensic documentation, publishing, education, marketing and communication with patients, dental team members, colleagues and dental laboratory.