A c.3037G>A mutation in FBN1 gene causing Marfan syndrome with an atypically severe phenotype.

Marfan syndrome is a pleiotropic connective tissue disease inherited as an autosomal dominant trait, mostly caused by mutations in the FBN1 gene, which is located on chromosome 15q21.1 and encoding fibrillin 1. We report a case of Marfan syndrome presenting with severe ocular and systemic manifestations, such as cardiac congenital anomalies. The patient underwent a multidisciplinary approach and his clinical diagnosis was associated with a c.3037G>A mutation in the FBN1 gene. Identification of this genetic alteration should instigate a prompt multidisciplinary assessment and monitoring, in order to prevent devastating consequences such as cardiac and ocular phenotype. Molecular modeling of the mutation highlighted the importance of the preservation of the calcium-dependent structure of an epidermal- growth-factor-like domain of fibrillin-1 and consequently the microfibrillar formation process. This report aims to highlight the importance of an early clinical and molecular diagnosis and once more, the importance of the multidisciplinary approach of this genetic entity.

An evaluation of clinical, radiological and three-dimensional dental tomography findings in ectodermal dysplasia cases.

BACKGROUND: This study aimed to review the results related to head and jaw disorders in cases of ectodermal dysplasia. The evaluation of ectodermal dysplasia cases was made by clinical examination and examination of the jaw and facial areas radiologically and on cone-beam 3-dimensional dental tomography (CBCT) images. MATERIAL AND METHODS: In the 36 cases evaluated in the study, typical clinical findings of pure hypohidrotic ectodermal displasia (HED) were seen, such as missing teeth, dry skin, hair and nail disorders. CBCT images were obtained from 12 of the 36 cases, aged 1.5- 45 years, and orthodontic analyses were made on these images. RESULTS: The clinical and radiological evaluations determined, hypodontia or oligodontia, breathing problems, sweating problems, a history of fever, sparse hair, saddle nose, skin peeling, hypopigmentation, hyperpigmentation, finger and nail deformities, conical teeth anomalies, abnormal tooth root formation, tooth resorption in the root, gingivitis, history of epilepsy, absent lachrymal canals and vision problems in the cases which included to the study. CONCLUSIONS: Ectodermal dysplasia cases have a particular place in dentistry and require a professional, multi-disciplinary approach in respect of the chewing function, orthognathic problems, growth, oral and dental health. It has been understood that with data obtained from modern technologies such as three-dimensional dental tomography and the treatments applied, the quality of life of these cases can be improved.

Anti-Inflammatory, Antioxidant, and Anti-Atherosclerotic Effects of Natural Supplements on Patients with FMF-Related AA Amyloidosis: A Non-Randomized 24-Week Open-Label Interventional Study.

We aimed to evaluate the effect of a combination of natural products on parameters related to inflammation, endothelial dysfunction, and oxidative stress in a cohort of familial Mediterranean fever (FMF) patients with Serum Amyloid A amyloidosis, in a non-randomized, 24-week open-label interventional study. Morinda citrifolia (anti-atherosclerotic-AAL), omega-3 (anti-inflammatory-AIC), and extract with Alaskan blueberry (antioxidant-AOL) were given to patients with FMF-related biopsy-proven AA amyloidosis. Patients were >18 years and had proteinuria (>3500 mg/day) but a normal estimated glomerular filtration rate (eGFR). Arterial flow-mediated dilatation (FMD), carotid intima media thickness (CIMT), and serum biomarkers asymmetric dimethylarginine (ADMA), high sensitivity C-reactive protein (hs-CRP), pentraxin (PTX3), malondialdehyde (MDA), Cu/Zn-superoxide dismutase (Cu/Zn-SOD), and glutathione peroxidase (GSH-Px) were studied at baseline and after 24 weeks of treatment. A total of 67 FMF-related amyloidosis patients (52 male (77.6%); median age 36 years (range 21−66)) were enrolled. At the end of a 24-week treatment period with AAL, AIC, and AOL combination therapy, ADMA, MDA, PTX3, hsCRP, cholesterol, and proteinuria were significantly decreased compared to baseline, while CuZn-SOD, GSH-Px, and FMD levels were significantly increased. Changes in inflammatory markers PTX3, and hsCRP were negatively correlated with FMD change, and positively correlated with decreases in proteinuria, ADMA, MDA, cholesterol, and CIMT. Treatment with AAL, AIC and AOL combination for 24 weeks were significantly associated with reduction in inflammatory markers, improved endothelial functions, and oxidative state. Efficient control of these three mechanisms can have long term cardiovascular and renal benefits for patients with AA amyloidosis.

Cone-beam dental computerized tomography for evaluating changes of aging in the dimensions central superior incisor root canals.

OBJECTIVES: Cone-beam dental computerize tomography (CBCT), a noninvasive imaging method, was used to image the pulp-dentin complex. Pulp changes histologically with aging. While many studies have investigated the histological changes in pulp, few studies have focused on the changes in the shape of the root canals. This study evaluated the changes in the root canals with aging of central superior teeth using CBCT. STUDY DESIGN: The study examined 100 non-carious maxillary central teeth. These teeth were divided into five groups according to the age of the patients: Group A:15-24, Group B: 25-34, Group C: 35-44, Group D: 45-54 and Group E: 55 years and older. CBCT was used to determine root length, and pulp width at the cervical, apical 1/2, and apical 1/3. RESULTS: On comparing the groups using one-way analysis of variance (ANOVA), the root length did not differ (P>0.05), while the pulp width at the cervical, apical 1/2, and apical 1/3 differed between the groups (P<0.001). CONCLUSIONS: The pulp length did not change with aging, while the pulp width did change. CBCT can be used to determine the precise root length and width, to prevent iatrogenic exposure of the apex, complementing existing methods. This will improve the prediction of the prognosis of root canal treatment.

Differences in ischemia-modified albumin levels between end stage renal disease patients and the normal population.

INTRODUCTION: Conventional biomarkers suffer from the drawback of being elevated in chronic renal failure even in the absence of myocardial ischemia. Ischemia-modified albumin (IMA) is a new biomarker proposed for the diagnosis of myocardial ischemia. This study was performed with the primary aim of determining IMA levels in patients with end stage renal disease (ESRD). The secondary aim of the study was to determine the impact of hemodialysis (HD), HD speed, and hemoglobin (Hb) levels on IMA levels. METHODS: The study was conducted with 108 ESRD patients entering HD and 30 healthy volunteers. The serum IMA levels of ESRD patients were compared with the post-HD levels and also with healthy individuals. The interaction between Hb levels and HD treatment and the IMA levels were tested by using the Generalized Linear Model for repeated measurements. RESULTS: The IMA levels of ESRD patients, both pre- and post-HD, were significantly higher than those of the control group. The baseline IMA levels of "low" and "high Hb groups" were not significantly different. Hb level modifies the effect of HD treatment on IMA concentration in ESRD patients. Furthermore, post-HD levels of IMA were increased at a lower dialysis speed. CONCLUSIONS: Both pre- and post-dialysis IMA levels are higher in ESRD patients entering HD than in healthy individuals. Anemia is an effect-modifier for the effect of HD treatment on IMA levels in ESRD patients.

Comparison of Bacterial Load Parameters in Subgingival Plaque during Peri-implantitis and Periodontitis Using the RT-PCR Method.

OBJECTIVE: To estimate the actual parameters of bacterial load in subgingival plaque during periodontitis and peri-implantitis pathologies using the RT-PCR (real-time polymerase chain reaction) method and evaluate their associations with clinical periodontal indicators. MATERIALS AND METHODS: Five different groups of subjects were selected according to a formulated design of the study: with mild/moderate periodontitis, with severe periodontitis, with peri-implantitis, healthy periodontal group and healthy peri-implant group. Subgingival plaque samples were formed with paper points inserted in the pocket/sulcus area for 30 seconds. A standardized test the "ParodontoScreen" was provided for identification of target opportunistic pathogens (A. actinomycetemcomitans, P. gingivalis, T. forsythia, P. intermedia, T. denticola) by the RT-PCR. RESULTS: Bacterial load parameters demonstrated a significant tendency towards an increase within periodontitis progression and during the presence of peri-implantitis pathology. Each targeted mean bacterial load level was statistically associated with periodontitis or peri-implantitis pathology (p < 0, 05) according to the provided univariate analyses and upon condition that bacterial load parameters of healthy sites were used as reference for equiparation. The highest correlation values were found between periodontal probing depth and bacterial load parameters of A. actinomycetemcomitans (r=0, 37; p < 0, 05) and P. gingivalis (r=0, 28; p < 0, 05); and also between clinical attachment loss and bacterial load values of A. actinomycetemcomitans (r=0, 38; p < 0, 05) and P. gingivalis (r=0, 24; p < 0, 05). CONCLUSIONS: Periodontitis and peri-implantitis are associated with the same microbial pathogens even though the distribution pattern of their bacterial load and detection frequency parameters registered with RT-PCR could be distinct and linked to the individual patient-related conditions and the severity stage of pathology.

Coronary artery bypass surgery in patients with malignancy: a single-center study with comparison to patients without malignancy.

BACKGROUND: There is debate on the timing and outcome of coronary artery bypass surgery in patients with coincident malignancy. In this study, we compared the outcome of coronary artery bypass graft (CABG) in such patients with those without malignancy. METHODS: The patients were selected from those who had undergone coronary artery bypass surgery in the last decade. The study group (group I) included the patients with malignancy in remission. The control group comprised those patients who were selected randomly from those without any malignancy. The patients were retospectively examined with regard to preoperative, operative, and postoperative data from personal files, computerized recording system, and operation reports. RESULTS: Group I included 48 patients (age 48 to 69; 29 male) while group II included 50 patients (age = 38 to 73; 35 male). In group I, comorbidity rates were: renal dysfunction in 12 (25%), obstructive lung disease 10 (21%), congestive failure in four (8%) patients. The malignancy rates were: lung in 15 (31%), breast in 10 (21%), stomach in five (10%), colon in four (8%), renal in one (2%), Hodgkin's lyphoma in three (6%), leukemia in two (4%), ovarian in three (6%), and prostate in five (10%) patients. In group II, the comorbidity rates were: diabetes mellitus 18 (36%), renal dysfunction in five (10%) and obstructive lung disease in 13 (26%) patients. In group I, chemotherapy and radiotherapy were performed in 38 and 34 patients, respectively. In groups I and II, the CABG was elective in 47 (98%) and in 45 patients (90%); the off-pump surgery was performed in 27 (56%) and 12 (24%) patients, respectively. The total duration of bypass was 37 +/- 6 minutes and 44 +/- 5 minutes; the duration of aortic clamp was 26 +/- 4 and 29 +/- 7 minutes, respectively, in groups I and II. Posoperative complication rates were: infection in 12 (25%), bleeding in eight (17%), acute renal insufficiency in eight (17%), prolonged air escape in five (10%), and prolonged entubation in 17 (35%) patients in group I and atrial fibrillation in 11 (22%) patients in group II. Mortality rates in both groups were two (4%). CONCLUSION: CABG in patients with comorbid malignancy is as safe as the other patients. In patients with full remission of malignancy, the surgeons should be encouraged about the safety of CABG.

[Isotretinoin embryopathy: An entity that can be avoided]. / Embriopatía por isotretinoína: Una entidad que puede evitarse.

Isotretinoin is the most effective drug in the treatment of severe recalcitrant nodulocystic acne. However, treatment with this drug is associated with adverse effects, the most severe being teratogenesis. It has been estimated that 40% of pregnancies exposed to isotretinoin present spontaneous abortion and 35% develop embryopathy. We present the case of a newborn with a history of prenatal exposure to isotretinoin, a clinical entity that can be avoided, with severe congenital defects in the central nervous system and important facial dysmorphisms, with unfavorable clinical course.

La isotretinoína es el medicamento más efectivo en el tratamiento del acné noduloquístico recalcitrante grave. Sin embargo, el tratamiento con este fármaco se encuentra asociado con efectos adversos, y el más grave es la teratogénesis. Se ha estimado que 40% de los embarazos expuestos a isotretinoína presenta un aborto espontáneo y 35% desarrolla embriopatía. Se presenta el caso de un recién nacido con antecedente de exposición prenatal a isotretinoína, una entidad clínica que puede evitarse, con graves defectos congénitos en el sistema nervioso central e importantes dismorfias faciales, con evolución clínica desfavorable.

The Evaluation of Root Fracture with Cone Beam Computed Tomography (CBCT): An Epidemiological Study.

BACKGROUND: The aim of this study was evaluation of the cone-beam computed tomography (CBCT) image of 50 patients at the ages of 8-15 suspecting root fracture and root fracture occurred, exposed to dental traumatic. In additionally, this study was showed effect of crown fracture on root fracture healing. MATERIAL AND METHODS: All of the individuals included in the study were obtained images with the cone-beam computed tomography range of 0,3 voxel and 8.9 seconds.(i-CAT®, Model 17-19, Imaging SciencesInternational, Hatfield, Pa USA).The information obtained from the history and CBCT images of patients were evaluated using chi-square test statistical method the mean and the distribution of the independent variables. RESULTS: 50 children, have been exposed to trauma, was detected root fracture injury in 97 teeth. Horizontal root fracture 63.9% of the 97 tooth, the oblique in 31.9%, both the horizontal and oblique in 1.03%, partial fracture in 2.06% ,and both horizontally and vertical in 1.03% was observed.The most affected teeth, respectively of, are the maxillary central incisor (41.23% left, right, 37.11%), maxillary left lateral incisor (9.27%), maxillary right lateral incisor (11.34%), and mandibular central incisor (1.03%). CONCLUSIONS: Crown fractures have negative effects on spontaneous healing of root fractures. CBCT are used selected as an alternative to with conventional radiography for diagnosis of root fractures. In particular, it's cross-sectional image is quite useful and has been provided more conveniences seeing the results of diagnosis and treatment for clinician. Key words:Root fracture, CBCT, Epidemiolog.

[Clinical and molecular study in a family with cleidocranial dysplasia]. / Estudio clínico y molecular en una familia con displasia cleidocraneal.

Cleidocranial dysplasia is an uncommon bone dysplasia with an autosomal dominant inheritance pattern characterized by short stature, large fontanels, midface hypoplasia, absence or hypoplasia of clavicles and orodental alterations. This is Estudio clínico y molecular en una familia con displasia cleidocraneal Clinical and molecular study in a family with cleidocranial dysplasia produced by mutations in the RUNX2 gene located at 6p21.1. We report two male adolescents (cousins), with cleidocranial dysplasia who presented a heterozygous missense mutation (c.674G> A, p.R225Q) in the RUNX2 gene, characterized by severe phenotype, such as absent clavicles, but with variation in the delayed fontanel closure, dental abnormalities (anomalies in shape and number) and scoliosis, thus demonstrating intrafamilial variation in these patients with the same genotype.

La displasia cleidocraneal es una displasia ósea infrecuente con patrón de herencia autosómico dominante, que se caracteriza por presentar talla baja, fontanelas amplias, hipoplasia mediofacial, ausencia o hipoplasia de clavículas y alteraciones orodentales. Es producida por mutaciones en el gen RUNX2 localizado en 6p21.1. Se presentan dos adolescentes masculinos (primos hermanos) con displasia cleidocraneal, los cuales mostraron mutación heterocigota, cambio de sentido (c.674G>A, p.R225Q) en el gen RUNX2, caracterizados por presentar fenotipo grave, como ausencia de clavículas, pero con variación en el retardo en el cierre de fontanelas, alteraciones dentales (anomalías en forma y número) y escoliosis, por lo que se demuestra la variación intrafamiliar en estos pacientes con el mismo genotipo.

[Clinical and molecular study in a family with autosomal dominant hypohidrotic ectodermal dysplasia]. / Estudio clínico y molecular en una familia con displasia ectodérmica hipohidrótica autosómica dominante.

Hypohidrotic ectodermal dysplasia (HED) is a rare disease characterized by deficiency in development of structure derived from the ectoderm and is caused by mutations in the genes EDA, EDAR, or EDARADD. Phenotypes caused by mutations in these three may exhibit similar clinical features, explained by a common signaling pathway. Mutations in EDA gene cause X linked HED, which is the most common form. Mutations in EDAR and EDARADD genes cause autosomal dominant and recessive form of HED. The most striking clinical findings in HED are hypodontia, hypotrichosis and hypohidrosis that can lead to episodes of hyperthermia. We report on clinical findings in a child with HED with autosomal dominant inheritance pattern with a heterozygous mutation c.1072C>T (p.Arg358X) in the EDAR gene. A review of the literature with regard to other cases presenting the same mutation has been carried out and discussed.

La displasia ectodérmica hipohidrótica (DEH) es una entidad infrecuente caracterizada por deficiencia en el desarrollo de estructuras derivadas del ectodermo y es causada por mutaciones en los genes EDA, EDAR o EDARADD, que pueden exhibir hallazgos clínicos similares, debido a una vía de señalización común. Las mutaciones en el gen EDA causan la DEH ligada al X, que es la forma más frecuente. Por su parte, las mutaciones en los genes EDAR y EDARADD causan la DEH con patrón de herencia autosómica dominante y recesiva. Los hallazgos clínicos más resaltantes son hipodoncia, hipotricosis e hipohidrosis, que pueden llevar a episodios de hipertermia. Se presentan los hallazgos clínicos en un niño con DEH con patrón de herencia autosómica dominante, cuyo análisis molecular demostró mutación heterocigótica c.1072C>T(p.Arg358X) en el gen EDAR, y se discuten los diferentes aspectos clínicos encontrados en esta mutación en los casos descritos en la literatura.

Ectodermal dysplasia: Retrospective study of fifteen cases.

The aim of this article is to review possible cranio-maxillofacial deformative consequences associated with hypohidrotic ectodermal dysplasia and embryonic malformations, which include dental ageneses, and describe the oral habilitation. Hypohidrotic ectodermal dysplasia patients had a clinical examination and underwent radiographic and Steiner's analyses and a respiratory capability test before assessment and treatment. Fifteen patients (eight males and seven females, aged 5-45 years) had tooth ageneses (from hypodontia to anodontia) associated with cutaneous dyshydrosis and hair and nail dystrophy. Most patients had sparse or absent hair, a short face with an unusual facial concavity, a maxillary retrusion and a relative mandibular protrusion. Dentists must conduct a comprehensive and multidisciplinary approach to these patients in order to improve their dental, masticatory, growth and orthognathic conditions.

Evaluation of ectodermal dysplasia.

This case series report outlines possible cranio-maxillofacial deformation consequences associated with ectodermal dysplasia (ED) and embryonic malformations, including dental agenesis. Also described are the oral aspects and rehabilitation. A total of 14 ED patients (7 males and 7 females, aged 5-45 years) underwent clinical examination before assessment and treatment. Lateral cephalometric radiography, Steiner's analysis, and respiratory capacity tests were performed. Most of the patients had sparse or absent hair, a short face with an unusual facial concavity, a maxillary retrusion, and a relative mandible protrusion. Depending on age and orthopedic abnormalities, patients were treated with prosthodontic and orthodontic approaches or implant treatment. Therapists should take a comprehensive and multidisciplinary approach with these patients to improve their dental, masticatory, growth, and orthognathic conditions, as well as esthetic appearance.

Posttransplant symptomatic lymphocele treatment with percutaneous drainage and ethanol sclerosis: long-term follow-up.

PURPOSE: Lymphocele following kidney transplantation is a well-known complication. We present here the long-term results of percutaneous drainage and ethanol sclerosis in posttransplant lymphoceles. PATIENTS AND METHODS: Between October 1988 and September 2003, 18 patients with symptomatic lymphoceles were treated with percutaneous transcatheter ethanol sclerosis among 300 renal transplants followed up. Catheters were positioned under ultrasonography (US) guidance. Cavity dimensions and extravasation were evaluated by pouch graph. The day after catheter insertion, sclerotherapy with ethanol was applied. Daily output and probable complications were followed up on periodic controls, and the alcohol was instilled when required. RESULT: The mean initial size of the lymphoceles was 6 x 8 x 10 cm (range 3 x 4 x 6 to 7 x 12 x 21 cm), with a mean initial volume of 230 ml (range 40-1340 ml). Incomplete resolution was achieved in all patients after sclerotherapy in the first week (100%). The mean duration of therapy was 17 days (range 4-45 days). The mean therapy sitting was 12 (4-21), and the average alcohol volume in one sitting was 30 ml. One recurrence and one graft lost were seen in this series. Minor complications like discomfort and mild temperature elevation were determined in 10 patients. In one case, temporary double-J stent was required to alleviate the increased pressure in collecting system of transplanted kidney. No late complication was occurred. CONCLUSION: As a less invasive method, percutaneous transcatheter ethanol sclerosis and drainage in the treatment of symptomatic lymphocele is a simple, safe and cost-effective method. Other treatment choices, like open or laparoscopic fenestration, can be used in case of failure.

[Clinical and molecular study in a child with X-linked hypohidrotic ectodermal dysplasia]. / Estudio clínico y molecular en un escolar con displasia ectodérmica hipohidrótica ligada al X.

Ectodermal dysplasia encompasses more than 200 clinically distinct entities, which affect at least two structures derived from the ectoderm, including the skin, hair, nails, teeth, sweat glands, and sebaceous glands. X-linked hypohidrotic ectodermal dysplasia is the most common type and is caused by mutation of the EDA gene that encodes Ectodysplasin-A. It occurs in less than 1 in 100 000 individuals and is clinically characterized by hypodontia, hypohidrosis, hypotrichosis, and eye dis orders. We present a child evaluated in a multidisciplinary manner with clinical and molecular diagnosis of X-linked hypohidrotic ectodermal dysplasia with type missense mutation c.1133C> T; p.T378M in EDA gene.

Minimal change disease in a patient receiving IFN-alpha therapy for chronic hepatitis C virus infection.

Chronic hepatitis C virus (HCV) infection is associated with several extrahepatic syndromes. The principal types of renal disorders associated with chronic HCV infection are cryoglobulinemia or noncryoglobulinemic membranoproliferative glomerulonephritis (MPGN). Interferon-alpha (IFN-alpha) may precipitate or exacerbate the occurrence of MPGN. Our patient was a 32-year-old man who tested positive for HCV in July 1997. The patient was treated with IFN-alpha in another medical center for 6 months because his liver biopsy showed chronic active hepatitis. In December 1998, he applied to our clinic for a follow-up examination. The level of aspartate aminotransferase (AST) was 44 U/L, and that of alanine aminotransferase (ALT) was 69 U/L. HCV RNA was positive in serum, and chronic HCV infection was detected by liver biopsy. IFN-alpha therapy (5 million U/day) was administered for 6 months longer. In May 1999, the patient came to our polyclinic with edema of the feet and legs. We detected proteinuria, serum cholesterol of 269 mg/dl, AST of 50 U/L, ALT of 41 U/L, serum total protein of 3.4 g/dl, serum albumin of 1.2 g/dl, positive cryoglobulin, and urine protein of 9.84 g/day. Cryoglobulinemic MPGN was suspected and kidney biopsy was performed, resulting in a diagnosis of minimal change disease (MCD).

Ophthalmic manifestations in patients with ectodermal dysplasia syndromes.

BACKGROUND: Ectodermal dysplasia (ED) is a disorder that results from abnormal formation of at least two of the four major ectodermal derivatives in the developing embryo. The ectoderm of the embryo forms the skin, teeth, hair and nails, sweat glands and part of the eyes. OBJECTIVES: The aim of this article is to reveal ophthalmologic symptoms and signs as multidisciplinary, reliable criteria for ectodermal dysplasia. MATERIAL AND METHODS: In this retrospective study, 24 patients with ED were analyzed from the recorded data. Ophthalmological examination of the patients, who had previously received the diagnosis of ED in the dental department, was done. During the examination, ocular symptoms related to tear film, corneal changes, lacrimal duct, periorbital hyperpigmentation, alteration lashes and eyebrows were evaluated. RESULTS: The age ranged between 3-45, and the mean and standard deviation (Mean ± SD) was 15.8 ± 7.4 years. The number of males was 13 (54.2%) and females, 11 (45.8%). Eighteen patients (75.0%) suffered from ocular complaints related to the ocular surface. In 11 of the patients with ED, there were dry eye symptoms. While the mean age of cases with eye involvement was 17.5, it was 23.1 in cases with dry eye symptoms. CONCLUSIONS: In the study, it was observed that, in patients with ED, ocular complaints, particularly dry eye symptoms, may increase as age advances.

Association Between Saliva Quantity and Content Parameters with Caries Intensity Levels: A Cross-Sectional Study Among Subcarpathian Children

Abstract Objective: To evaluate saliva quantity and content parameters among children of 7 and 12 years old, who permanently living on the territory of Subcarpathia with the registered territory-associated fluoride deficiency in the water, and their association with the caries status of pediatric patients. Material and Methods: The study sample was formed of 48 children (22 of 7 years old and 26 of 12 years old). The content of calcium in the oral liquid was determined by the o-cresolphthalein complexone method. Estimation of concentration rate and fluoride activity in the oral liquid was carried out by using the ion-selective electrode ELIS-131 F and ionometer EV-74. The content of inorganic phosphorus in saliva was determined using the phosphorus reaction with molybdic acid Results: Among all study samples, 18.8% were registered with low caries intensity level (DMF = 1.55 ± 0.16), 33.3% with moderate caries intensity level (DMF = 3.94 ± 0.29), and 47.9% with high caries intensity level (DMF = 9.05 ± 1.11). During the comparison of calcium content and mineralization coefficient values between children with low and high caries intensity levels registered difference was statistically significant (p<0.05), while for salivary flow rate parameter such difference was no significant (p>0.05). Between children with normal salivary flow rate, and children with a lowered salivary flow rate there was no statistical difference in such parameters as fluoride concentration, calcium content, phosphorus content and calcium-phosphorus balance (p>0.05) Conclusion: Caries intensity levels were more statistically associated with parameters of calcium content in saliva and related mineralization coefficient, rather than with the average salivary flow rate.

A case of cleidocranial dysplasia with peculiar dental features: pathogenetic role of the RUNX2 mutation and long term follow-up.

This report deals with a case of Cleidocranial Dysplasia (CCD) associated to a rare mutation of the RUNX2 gene and a peculiar dental phenotype, namely no supernumerary teeth. The aim consists in evaluating the long-term follow-up after treatment and discussing the pathogenetic mechanism of the mutation. We have carried out a clinical evaluation after treatment and attempted to analyze the potential pathogenetic effect of the mutation, based upon the available experimental structure of RUNX family domain and the highly conserved homology of RUNX1-3. Clinically the treatment has led to tooth development in crowns an roots, correction of cross-bite and eruption of the central maxillary incisor. The structural analysis has pointed out impairment in the DNA binding capability of the mutant protein. The described mutation, c.391C>T (p.R131C) appears to influence both structure and function of the protein by hampering the interaction of RUNX2 with DNA. The impaired function could explain the peculiar reported CCD phenotype. The dental condition of our patient has largely improved after treatment.

A new biological approach to guided bone and tissue regeneration.

The purpose of this study was to determine the potential of platelet-rich fibrin (PRF) membranes used for guided bone and tissue regeneration. A patient with insufficient alveolar ridge width in aesthetic zone was enrolled. The patient's blood was centrifuged to obtain PRF membranes. Autogenous bone graft was mixed with bovine hydroxyapatite, PRF particles and applied to fill the defect. Five PRF membranes were placed over the bone mix. After 4 months a cone-beam CT was performed to evaluate bone regeneration. The use of PRF as cover membrane permitted a rapid epithelisation and represented an effective barrier versus epithelial cell penetration. After 4 months the site appeared precociously healed and the bone volume increased. This new approach represents a predictable method of augmenting deficient alveolar ridges. Guided bone regeneration with PRF showed limitation compared with guided bone regeneration using collagen membrane in terms of bone gain. The association of collagen membrane and PRF could be a good association.