Association between oral and fecal microbiome dysbiosis and treatment complications in pediatric patients undergoing allogeneic hematopoietic stem cell transplantation.

The oral and gastrointestinal mucosae represent the main targets of the toxic effect of chemo and/or radiotherapy administered during the conditioning regimen before hematopoietic stem cell transplant (HSCT). These harmful consequences and the immunological complications that may occur after the transplant (such as Graft versus Host Disease, GvHD) are responsible for the clinical symptoms associated with mucositis during the aplasia phase, like pain, nausea, vomiting, and diarrhea. These toxicities could play a critical role in the oral and gastrointestinal microbiomes during the post-transplant phase, and the degree of microbial dysbiosis and dysregulation among different bacterial species could also be crucial in intestinal mucosa homeostasis, altering the host's innate and adaptive immune responses and favoring abnormal immune responses responsible for the occurrence of GvHD. This prospective pediatric study aims to analyze longitudinally oral and gut microbiomes in 17 pediatric patients who received allogeneic HSCT for malignant and non-malignant diseases. The oral mucositis was mainly associated with an increased relative abundance of Fusobacteria, and Prevotella species, while Streptococcus descendants showed a negative correlation. The fecal microbiome of subjects affected by cutaneous acute GvHD (aGvHD) correlated with Proteobacteria. Oral mucosal microbiota undergoes changes after HSCT, Fusobacteria, and Prevotella represent bacterial species associated with mucositis and they could be the target for future therapeutic approaches, while fecal microbiome in patients with acute GvHD (aGvHD) revealed an increase of different class of Proteobacteria (Alphaproteobacteria and Deltaproteobacteria) and a negative correlation with the class of Gammaproteobacteria.

Complex odontoma at the upper right maxilla: Surgical management and histomorphological profile.

BACKGROUND: Odontomas are hamartomatous developmental malformations of the dental tissues. Usually asymptomatic, their presence is often revealed on routine radiographs. The study aimed to establish the efficacy of this conventional approach in treating odontomas, analysing clinical outcome, follow-up, and histomorphological profile. CASE REPORT: A case is presented with a review of the international literature. The patient, aged 8 years, had a complex odontoma localised on the front upper jaw. She was treated following the conventional surgical procedure. Post-operative course and healing were uneventful. Orthodontic treatment was necessary to realign the teeth. At the 12-month follow-up there was no recurrence or failure. Healing was excellent. CONCLUSION: Variations in normal tooth eruption are a common finding, but significant deviations from established norms should alert the clinician to further investigate the patient's health and development.

Clinical, histomorphological and therapeutic features of the Van der Woude Syndrome: literature review and presentation of an unusual case.

BACKGROUND: Van der Woude syndrome (VWS), an autosomal dominant condition associated with lower lip pits and/or cleft palate, is caused by mutations in the interferon regulatory factor 6 gene (lRF6 gene). The genetic alterations identified to date that contribute to expression of the syndrome are chiefly mutations located on chromosome 1 (the largest of our chromosomes), mutations at p36 that codifies the gene GRHL (grainy-head transcriptor factor) and mutations involving IRF6 (interferon regulatory factor). With frequency ranging from 1:35,000 to 1:100,000, depending on ethnicity, gender, and socio-economic status, the syndrome accounts for about 2% of orofacial clefts. The clinical and histomorphological aspects of VWS are studied, and a case of heterozygous female twins of whom only one was affected with VWS is reported. CONCLUSION: This very rare case (no similar case has been reported to date) contributes further evidence on modifying factors in the expression of this condition.

Space-maintaining management in maxillary sinus lifting: a novel technique using a resorbable polymeric thermo-reversible gel.

Several techniques have been proposed to achieve sinus floor elevation and the formation of new bone through the grafting of autologous, heterologous, or alloplastic materials. The grafted materials act as a scaffold for bone formation inside the maxillary sinus. This study investigated a non-graft sinus lifting procedure using a resorbable polymeric thermo-reversible gel. A space-maintaining approach to sinus lifting, using a resorbable polymeric thermo-reversible gel, was applied in 11 patients undergoing implant treatment in the atrophic posterior maxilla. After a healing period of 6 months, a total of 14 implants were placed; biopsies were taken and evaluated histologically and histomorphometrically. The parameters evaluated included the percentages of new bone formation, residual gel, and fibrous tissue. Histological examination showed the formation of new bone with no fibrous tissue or severe inflammatory cellular infiltration. The percentage of newly formed bone was in the range of 54-60%; this consisted of both lamellar and woven bone. No foreign-body reaction was observed. The mean quantities of both residual gel and connective tissue were small. This non-graft sinus lifting procedure using a space-maintaining gel appears to stimulate predictable bone formation; it is thus a useful technique for promoting bone formation in the sinus.

Tongue-tie assessment: clinical aspects and a new diode laser technique for its management.

BACKGROUND Often breastfeeding problems experienced by mothers and their babies may be attributed to the abnormal attachment of the infant's tongue (ankyloglossia) and/or maxillary lip-tie. Proper breastfeeding depends upon an infant's ability to correctly latch onto its mother's breast. If born with oral soft tissue abnormalities such as tongue-tie or lip-tie, it may be almost impossible for the infant to breastfeed. During the oral evaluation of an infant presenting with breastfeeding problems, one factor that is often overlooked and undiagnosed - and thus untreated - is the attachment of the upper lip to the maxillary gingival tissue. CASE REPORT: The case is reported of tongue-tie and breastfeeding difficulties, treated with a novel technique: the diode laser (980 nm).

Oral manifestations of gastrointestinal diseases in children. Part 3: Ulcerative colitis and gastro-oesophageal reflux disease.

BACKGROUND: Alterations of the oral cavity are common in children: 22% of children aged less than 4 years and 44% of those aged more than 12 develop dental erosion, 9-95% of children in Europe and in North America develop gingivitis, with adolescents showing a prevalence of more than 60% [Italian Ministry of Health, Guidelines 2013]. Alterations within the oral cavity can be the first sign of systemic diseases and may thus allow for an early diagnosis and treatment. In particular, being the oral cavity a part of the gastrointestinal system, oral alterations can be an expression of a gastrointestinal disease. Pyostomatitis vegetans can be a sign of ulcerative colitis. Dental erosion with enamel loss in facial, occlusal, and lingual surfaces, and an increased risk of dental caries have been reported in children and adolescents with gastro-oesophageal reflux with varying prevalence. A prompt recognition of systemic diseases through a careful examination of the oral cavity could allow proper investigations and management in a timely fashion.

Oral manifestations of gastrointestinal diseases in children. Part 4: Coeliac disease.

Alterations within the oral cavity can be the first sign of systemic diseases and may thus allow for an early diagnosis and treatment. In particular, being the oral cavity part of the gastrointestinal system, oral alterations can be an expression of a gastrointestinal disease. Dental enamel hypoplasia and aphthous ulcers have been found to be more common in children with coeliac disease compared with the general population and to regress after the patient is started on a gluten free-diet. A prompt recognition of systemic diseases through a careful examination of the oral cavity could allow the child to have appropriate investigations and to be treated in a timely fashion.

Allgrove syndrome: a report of a unique case characterised by peculiar dental findings resembling those of ectodermal dysplasia.

BACKGROUND: Triple A or Allgrove Syndrome (OMIM#231550) is a rare, autosomal recessive genetic disorder in which patients typically suffer from chronic adrenal insufficiency due to resistance to ACTH (Addison's disease), esophageal achalasia, and defective tear formation (alacrima). The syndrome is caused by mutations in the AAAS gene on chromosome 12q13 encoding a 546 aminoacid protein named alacrimia-achalasia-adrenal insufficiency neurologic disorder (ALADIN), a constituent of eukaryotic nuclear pore complexes. CASE REPORT: We describe a case of Allgrove Syndrome presenting with anhidrosis and peculiar dental features resembling those of Ectodermal Dysplasia (ED). CONCLUSION: Given the clinical findings in this case we suggest the hypothesis that the pathogenetic mechanism in Allgrove syndrome is related to the ED.

Mucocele of the minor salivary glands in an infant: treatment with diode laser.

BACKGROUND: Mucoceles are benign lesions that develop as a result of retention or extravasation of mucous material from minor salivary glands. Very uncommon in newborns and infants, they rarely may interfere with breastfeeding and compromise the respiratory function. CASE REPORT: We report a case of mucocele in a three-month-old infant in the right labial commissure excised by diode laser of different wavelengths (635-980 nm), with an average power of 1.8 W, in continuous wave mode, using 300 to 320 micron optical fibers. The healing occurred in 10 days. There were no adverse effects and the patient was carefully followed-up until complete healing. CONCLUSION: T he diode laser is not only a valuable tool for mucocele eradication but it also reduces relapses, thanks to the characteristics of the laser light.

Tooth developmental anomalies in severe combined immunodeficiency disease and juvenile myelomonocytic leukemia: common clinical features and treatment outcomes.

AIM: Human Severe Combined Immunodeficiency (SCID) is a prenatal disorder of T lymphocyte development that depends on the expression of numerous genes. Juvenile myelomonocytic leukemia (JMML), previously known as juvenile chronic myeloid leukemia (JCML), is a rare, myelodysplastic/myeloproliferative disease typically presenting in early childhood. CASE REPORTS: Two cases are described of immunodeficiency disorders, both treated with chemotherapeutic drugs (Busulfan plus cyclophosphamide) before bone marrow transplantation. After treatment, these two different cases showed several similar oral lesions: microdontia, root alterations, numerous tooth ageneses, incomplete calcification, enamel hypoplasia, premature apexification and hypodontia. Both subjects underwent dental and orthodontic treatment. The first phase comprised orthopaedic treatment using a removable appliance (Interim-G®) followed by rapid palatal expansion; in the second phase patients underwent tooth extraction and were treated using fixed appliances for 19 and 26 months, respectively (mean 2 years) to obtain final alignment and maximum intercuspation. In the third and final phase, reconstruction of malformed teeth was completed, and implant-supported protheses were applied. CONCLUSION: The difficulties of managing and treating these diseases are discussed, with particular focus on tooth anomalies and malocclusion disorders. Collaboration between dentist and paediatrician in dealing with patients with a variety of oral lesions and tooth anomalies is important in order to prevent any other possible tooth lesions and ensure correct jaw development.

Do changes in spheno-occipital synchondrosis after rapid maxillary expansion affect the maxillomandibular complex?

AIM: This was to evaluate changes in spheno-occipital synchondrosis one year after rapid maxillary expansion (RME), in order to assess the influence that any change might have on sagittal and vertical skeletal cephalometric variables. MATERIALS AND METHODS: Patients were selected consecutively and grouped into: Group 1 comprised 30 Caucasian patients (13 m; 17 f) undergoing RME therapy; after active expansion therapy, the Haas expander was worn as passive retainer for an average of 7 months. Group 2 as control included 14 untreated subjects (6 m, 8 f), matched by age, sex and vertebral skeletal maturity (CVM method, stages 1-3). Six cephalometric variables concerning spheno-occipital synchondrosis were studied: N-S-Ba; SOS-Ba; SOS-S; S-Ba; Ba-N; S-N; nine skeletal variables for sagittal and vertical evaluation were also checked. T-test was used for comparing the 2 groups data. RESULTS: A statistically-significant opening of the spheno-occipital synchondrosis and increase of the posterior cranial base length (Ba-SOS) were found between group 1 and 2. After 1 year, these modifications in spheno-occipital syncondrosis produced no change in the anteroposterior or vertical skeletal parameters examined. CONCLUSION: After RME there were statistically significant effects on spheno-occipital synchondrosis length and cranial base angle; however, these changes in the mid-term did not affect the vertical or sagittal parameters of the skeletal maxillomandibular complex.

Autoptic and echocardiographic findings in seven foetuses with congenital heart anomalies, lung lobation defects and normal visceroatrial arrangement.

OBJECTIVES: Congenital heart defects may be associated with various extracardiac and chromosomal anomalies, and complex cardiac defects may occur in the presence of heterotaxy syndromes, in which both lungs are bilobate, in left isomerism, or both trilobate, in right isomerism. Lung lobation defects are otherwise very rare. Lung lobation is recognisable only at autopsy; however, its definition is fundamental for evaluation of the visceroatrial arrangement, together with other characteristic signs. METHOD: We report seven cases of congenital heart defects diagnosed prenatally at 14-31 weeks of gestation (wg), 5 females and 2 males, in which autopsy revealed lung lobation defects in the presence of normal visceroatrial arrangement, in association with other extracardiac anomalies or dysmorphism. RESULTS: Three foetuses had hypoplastic left heart syndrome, one had corrected transposition of great arteries, one had tricuspid atresia, one Ebstein's anomaly and one had ventricular septal defect in trisomy 21. In six cases, pregnancy had been terminated, while the foetus with Ebstein's anomaly died in utero at 32 wg for supraventricular tachycardia. Monolobate, bilobate, trilobate and quadrilobate lungs were found in these foetuses, together with other minor extracardiac anomalies or dysmorphism. CONCLUSIONS: Autoptic analysis in cases with prenatal diagnosis is needed to confirm echographic findings and reveal other minor anomalies, undetectable by ultrasound imaging that may complete the malformation pattern, which is useful to the couple for genetic counselling.

Decontamination of root canals with the gallium-aluminum-arsenide laser: an in vitro study.

OBJECTIVES: In this in vitro study, we investigated the bactericidal effects on root canals of 810-nm diode laser irradiation, alone or combined with sodium hypochlorite and citric acid irrigation. MATERIALS AND METHODS: One hundred sixteen single-rooted human teeth extracted for periodontal reasons were randomly divided into four experimental groups. The canal of each tooth was prepared with a conventional step-back technique and a pure culture of vancomycin-resistant Enterococcus faecalis grown in brain heart infusion broth was used to contaminate the root canal. The specimens were incubated at 37 degrees C for 15 d in a test tube filled with agar, adding fresh bacterial suspension every 48 h, and sent them for microbiological analysis and bacterial count. Subsequently they were divided into four groups: in group A, 29 teeth were irrigated with 2 mL of 10% citric acid solution; in group B, 29 teeth were irrigated with 2 mL 5.25% sodium hypochlorite (NaOCl) solution; in group C, 29 teeth were irradiated with 810-nm laser energy via a 200-microm optic fiber at 2.5 W power in pulsed mode (10 msec on and 10 msec off) for 5 sec; and in group D, 29 teeth were irrigated with NaOCl, irradiated with the laser, then irrigated with citric acid, and irradiated with the laser, followed again by NaOCl irrigation and laser irradiation. All the samples were again sent for microbiological analysis and bacterial count. RESULTS: Group A had a bactericidal effect of 0.041 log mean CFU, that of group B was 3.381 log mean CFU, and that of group C was 1.459 log mean CFU, whereas group D showed the best results, with a bactericidal effect of 7.178 log mean CFU. CONCLUSIONS: The use of NaOCl, citric acid, and diode laser energy together have a synergistic effect, increasing treatment efficacy and leading to significantly better decontamination of the root canal.

Granular cells tumour in the oral cavity: report of eleven cases treated with laser surgery.

AIM: The aim of this paper is to examine the clinical and histological feature of oral tumours of neural derivation and discuss their treatment. METHODS: Between 1999 and 2004, 11 patients (6 females, 5 males; age range 28-66 years) were treated for tumours classified as being of neural origin; they were all myoblastomas or granular cells tumours (ex Abrikossoff tumour). Eight were located on the dorsum of the tongue, 2 on the lateral margin and 1 on the right buccal mucosa. In all cases the mass appeared nodular, single and firm; patients were treated in a single session by excisional resection with 808 and 830 diode laser. RESULTS: During follow-up, which ranged from 5 months to 5 years, there was no indication of tumour recurrence. Histopathological analysis of haematoxylin and eosinstained sections showed all specimens to exhibit features typical of GCT; 6 tumours were well circumscribed, whereas 5 infiltrated adjacent connective tissue, muscle fibres and nerve bundles. In 10 of the 11 cases, the immunohistochemical S-100 protein stain was available and appeared positive. Pseudo-epitheliomatous hyperplasia of different degrees was present in 5 patients, along the overlying epithelium. CONCLUSIONS: Granular cell tumour is a benign neoplasm with a tendency to relapse if not completely removed. The usefulness of laser in the surgical treatment of these tumours particularly if small in size, is underlined.

Oral metastasis of ciliary body melanoma. A case report.

Metastases of melanoma rarely occur in the oral cavity, and very few reports have been published. They are chiefly localized in the tonsil, tongue and lip, regardless of the primary site of the neoplasm. A 76-year-old woman presented a brownish berry-shaped floating neoformation at the upper lip, for which she was hospitalized at the Maxillofacial Surgery Unit, Italian Stomatologic Institute, Milan. Medical history revealed that 6 years previously, in 1998, she underwent enucleation of the right eye due to the presence of a melanoma of the ciliary body. The labial neoplasm was removed and at histological examination it was found to be a spindle cell melanoma with numerous melanophages containing granules of melanin. Both the spindle cells and the melanophages were strongly positive for HMB-45 and for S-100. Thus, the presence of melanic neoplasia at an unusual site together with the medical history of melanoma at the ciliary body, removed 6 years previously, indicated a diagnosis of labial metastasis of melanoma of the ciliary body and the patient was therefore transferred to the Oncology Unit for appropriate treatment.

[Dental pulp metastasis from oral squamous cell carcinoma: a case report and a review of the literature]. / Metastasi alla polpa dentaria da carcinoma squamoso orale: presentazione di un caso e revisione della letteratura.

A 61-year-old man was seen in the Oral Pathology Department with a three-month history of right mandibular pain. Clinical examination revealed a 3 cm mass involving the body and the angle of the right site of the mandible and palpable masses in the neck. The dental panoramic radiography showed on the right a radiolucenT area surrounding the lower second molar, extending to the mandible angle. Additionally, chest radiograph and routine laboratory analysis results were unremarkable. An incisional biopsy of the area, revealed histologically a well differentiated squamous cell carcinoma and after few days, the patient received a right hemimandibulectomy with dissection of submandibular salivary gland and laterocervical lymph nodes on the same side. The histology of surgical material confirmed the diagnosis of a differentiated squamous cell carcinoma G1, homolateral diffusion in the mandible bone and metastasis to the dental pulp of the second lower molar tooth and to one of 18 lymph nodes dissected. In this report is described a very rare metastasis to the dental pulp and literature review, in which only fifteen other cases are reported.

Conduction system in cardiac amyloidosis: two cases succumbed to cardiac arrest.

The present article reports the histological study of the conduction system in 2 cases of cardiac amyloidosis. The discrepant anatomical and clinical evidence yet confirmed the need for accurate ECG controls in all cases. Indeed, while in the first case evident lesions of the conduction system were revealed by surface ECGs, the second case did not exhibit significant ECG abnormalities but the right atrium and the His bundle showed slight fibro-amyloid involvement, as potential forerunners of high-risk arrhythmias.