The efficacy of long-term psoralen plus ultraviolet A and low-dose interferon-a combination therapy in mycosis fungoides: A literature review.

BACKGROUND/PURPOSE: Interferon (IFN)-a is often used in combination with psoralen plus ultraviolet A (PUVA) in patients with mycosis fungoides (MF) refractory to skin-targeted therapies in early or advanced stages. The main objective is to evaluate the effectiveness of combined PUVA and low-dose IFN-α-2a therapy in patients with early- and advanced-stage MF. METHODS: Sixty-eight patients who received a combination of PUVA twice or thrice a week and INF-a 3 MU thrice a week for at least 3 months were reviewed retrospectively. The treatment response was evaluated as complete remission (CR), partial remission, stable disease, or progression. RESULTS: At the initiation, the majority of patients (66.2%) had early-stage disease. In 27.9% of cases, this was the initial treatment administered following the diagnosis of MF. The median duration of combination therapy was 11 months. Complete remission was achieved in 45.6% of the patients with an overall response rate of 60.3%. The mean duration of response was 5 months. Complete remission was statistically significantly higher in early-stage patients (p < .05). No statistically significant correlation was observed between CR and gender, histopathological features, or laboratory parameters. In patients with CR, 80% experienced relapse, significantly higher in early-stage patients (p < .05). However, there was no significant difference in disease-free survival between early and advanced stages (p > .05). CONCLUSIONS: The study results indicated that PUVA + low-dose INF-a combination therapy was more effective in the early stage than in the advanced stage. Additionally, there was a high relapse rate after the cessation of treatment in patients who achieved CR.

Diagnostic Challenges in a Family with Dominant Dystrophic Epidermolysis Bullosa and Isolated Hereditary Nail Disorder: Paternal Gonosomal Mosaicism for COL7A1 Mutation and Maternal RSPO4 Variant.

Dystrophic epidermolysis bullosa (DEB) has long been recognized as a well-known genodermatosis, caused by COL7A1 gene pathogenic variants. Isolated anonychia associated with RSPO4 gene mutation is a recently described non-syndromic autosomal recessive condition. In this family, a 13-year-old girl presented with severe epidermolysis bullosa symptoms. Family history revealed milder but similar complaints in the siblings, and anonychia affecting all nails in the mother and maternal relatives. The father did not have any signs of DEB. Genetic testing revealed heterozygous c.6127G>A (p.Gly2043Arg) variant in the COL7A1 (NM_000094.4) gene, in the proband and her affected siblings. The variant was not detected in the mother or father, prompting investigation into parental mosaicism. Detection of the variant in sperm sample suggested paternal mosaicism. Additionally, RSPO4 gene (NM_001029871.4) was sequenced in the mother and two of her affected sisters for suspected non-syndromic isolated anonychia, revealing homozygous c.79+1G>A variant. Isolated nail disease in the mother was initially thought to be the result of DDEB nails-only subtype and the DEB in the children was inherited from the mother. However, further clinical and genetic investigation showed that the condition in the patient and her siblings arose from gonosomal mosaicism in the father and the nail phenotype in the mother is a separate coincidental condition. This report aims to serve as an example for similar cases and highlight the importance of detailed genetic analysis guided by comprehensive medical history in reaching a diagnosis.

EVALUATION OF MANDIBULAR TRABECULAR AND CORTICAL STRUCTURE BY FRACTAL ANALYSIS IN HYPERLIPIDEMIA AND HYPERTENSION PATIENTS.

INTRODUCTION: This study aimed to evaluate the mandibular trabecular and cortical changes in patients with hyperlipidemia (HL) and/or hypertension (HT) using fractal dimension (FD) analysis, mandibular cortical width (MCW), panoramic mandibular index (PMI) and mandibular cortical index (MCI). MATERIALS AND METHODS: Panoramic radiographs of 100 patients were evaluated. FD measurement of three region of interest (ROI) including the angulus, corpus and interdental bone area were made. MCW, PMI and MCI were also measured and noted. RESULTS: Angulus, corpus and interdental FD values were significantly lower in three disease groups than the control group. Angulus, corpus, and interdental FD values were significantly lower in the HL+HT group than in the HL group and HT group. MCW value was significantly lower in the HL group, HT group, and HL+HT group than the control group. The cortical index C1 was more common in the control group while C2 was more common in the HT, HL and HL+HT group. CONCLUSION: The fact that FD was significantly lower in the HL+HT group compared to the HL and HT groups indicates the positive effect of their association on bone loss and quality. FD measurements on images obtained using a direct digital panoramic system can be used for treatment planning and follow-up of patients with HL and/or HT.

The Relationship between an Accessory Maxillary Ostium and Variations in Structures Adjacent to the Maxillary Sinus without Polyps

Abstract Introduction The maxillary sinus and its variations are very important to dentistry and rhinology. Objective To investigate the effect of the accessory maxillary ostium (AMO) on the variations of adjacent structures of the maxillary sinus. Methods The computed tomography (CT) images of 400 patients were retrospectively evaluated. The prevalence of AMO was calculated. The relationship between morphological variations of adjacent structures of maxillary sinus such as agger nasi cell (ANC), Haller cell (HC), nasal septum deviation (NSD), hypertrophy of inferior concha (HIC), pneumatization of middle concha (PMC), mucus retention cyst (MRC), mucosal thickening (MT), and maxillary sinusitis (MS), as well as the presence of AMO, were investigated. Results Presence of AMO was diagnosed in 42 patients (10.5%), having been found in 4.5% of the patients only on the right side, in 1.25% of the patients only on the left side, and in 4.75% of the patients on both sides. There is an increasing incidence of ANC, HC, NSD, HIC, and PMC in the presence of AMO and MS. There is a decreasing incidence of MRC in the presence of AMO. Furthermore, AMO does not affect the incidence of MT. Conclusion This study showed that most parameters, except for MRC and MT, had increasing incidence in the presence of AMO. It is important for radiologists and rhinologists to have knowledge about the location of AMO and the presence of variations of MS adjacent structures to avoid surgical complications.

Evaluation of the prevalence of bifid mandibular condyle detected on cone beam computed tomography images in a Turkish population / Evaluación de la prevalencia de cóndilo mandibular bífido detectado en tomografía computadorizada cone-beam en una población turca

The aim of this study was to assess the frequency of the BMC phenomenon in a Turkish patient population. Cone beam computed tomography (CBCT) images of 2634 consecutive patients were retrospectively reviewed. The Chi-squared test was used to determine potential differences in the distribution of BMCs when stratified by sex and side. Among the 2634 patients, 42 (1.7%) patients were found to have BMC. Of these 42 patients, 22 were female (0.8%) and 20 were male (0.7%) with age ranging from 29 to 68 years (mean age 47.47). Among the 42 patients, 39 (92.8%) of the BMCs were unilateral and three (7.1%) were bilateral. Approximately 24 cases (53.3%) were on the right side, and 21 cases (46.6%) were on the left side. All of the BMCs showed a mediolateral orientation. The mean depth of the BMC was 2.55 mm in males and 2.68 mm in females. 2 patients have symptoms whereas the other patients were atraumatic and asymptomatic. BMC is a rare condition that might be more prevalent in the Turkish population. Greater detailed information regarding BMC could be obtained by the widespread use of CBCT in epidemiological studies.

El objetivo de este estudio fue evaluar la frecuencia del fenómeno CMB en una población de pacientes de Turquía. Se revisaron imágenes consecutivas de tomografía computarizada (CBCT) de 2.634 pacientes retrospectivamente. Se utilizó la prueba de Chi-cuadrado para determinar las posibles diferencias en la distribución de CMB estratificado por sexo y lado. Entre los 2.634 pacientes, se encontró que 42 (1,7%) pacientes tenían CMB. De estos 42 pacientes, 22 eran mujeres (0,8%) y 20 eran varones (0,7%), con edades entre 29 a 68 años (promedio edad 47,47). Entre los 42 pacientes, 39 (92,8%) del CMB fueron unilaterales y tres (7,1%) fueron bilaterales. Aproximadamente 24 casos (53,3%) estaban en el lado derecho, y 21 casos (46,6%) estaban en el lado izquierdo. Todas las CMB mostraron una orientación mediolateral. La profundidad media de la CMB era 2,55 mm en los hombres y 2,68 mm en las mujeres. Dos de los pacientes presentaron síntomas, mientras que en el resto de los pacientes no presentó trauma ni síntomas. CMB es una afección poco común que podría ser más frecuente en la población turca. Mayor información y detalle sobre CMB se podría obtener en estudios epidemiológicos con el uso generalizado de CBCT.

Bilateral bifid condylar process / Proceso condilar bífido bilateral

The bifid condylar process is a rare alteration that is usually diagnosed as an incidental finding in a panoramic radiograph. The etiology of bifid condylar process is unknown, although the most tenable theory is that of traumatic origin. A 46-year-old woman was referred to our dental department with a complaint of pain and clicking on her temporomandibular joints. A panoramic radiograph showed bilateral bifid condyles Described with bifid condylar process varies from case to case, however in most cases are asymptomatic. The most common and predominant symptoms are temporomandibular joints sounds. The diagnosis of bifid condylar process is based on its radiographic appearance. Some researchers have indicated the necessity of imaging the anomaly by a second imaging method obtained in a different plane, in addition to orthopantomograph, and they have employed different techniques such as CT and magnetic resonance imaging. The bifid condylar process, and particularly the bilateral bifid condylar process, is a rare finding, the etiology of which remains uncertain. It is predominately an asymptomatic condition, discovered by chance on routine radiographic survey. The diagnosis is based on the radiographic manifestations and the treatment is usually conservative.

El proceso condilar bífido es una alteración rara que usualmente se diagnostica como un hallazgo incidental en una radiografía panorámica. La etiología de proceso condilar bífido es desconocida, aunque la teoría más aceptable es la de origen traumático. Una mujer de 46 años de edad, fue remitida a nuestro servicio dental con presencia de dolor y clic en sus articulaciones temporomandibulares. Una radiografía panorámica mostró procesos condilares bífidos bilaterales. La descripción del proceso condilar mandibular bífido varía de caso a caso, sin embargo en la mayoría de los casos son asintomáticos. Los síntomas más comunes y predominantes son los sonidos en las articulaciones temporomandibulares. El diagnóstico de proceso condilar bífido se basa en su aspecto radiográfico. Algunos investigadores han indicado la necesidad de proyección de imagen de la anomalía por un segundo método de imagen obtenida en un plano diferente, además de ortopantomografía, y han empleado diferentes técnicas como la tomografía computarizada y resonancia magnética. El proceso condilar bífido, y en particular bilateral, es un hallazgo poco frecuente, cuya etiología sigue siendo incierta. Es predominantemente una enfermedad asintomática, descubierta por casualidad en examen radiográfico de rutina. El diagnóstico se basa en las manifestaciones radiográficas y el tratamiento suele ser conservador.

Florid cemento-osseous dysplasia: a case report

Florid cemento-osseous dysplasia (FCOD) has been described as a condition that characteristically affects the jaws of middle-aged black women. It usually exhibits as multiple radiopaque cemetum-like masses distributed throughout the jaws. Radiographically, FCOD appears as dense, lobulated masses, often symmetrically located in various regions of the jaws. Computed tomography, because of its ability to give axial, sagittal, and frontal views, is useful in the evaluation of these lesions. This paper presents the case of a patient who was diagnosed with FCOD on the basis of clinical and radiographic findings.

A displasia cemento-óssea florida (DCOF) tem sido descrita como uma condição que afeta tipicamente os maxilares de mulheres negras de meia idade, geralmente exibindo massas radiopacas semelhantes ao cemento, distribuídas nos ossos maxilares. Radiograficamente, a DCOF apresenta-se como densas masas lobuladas, frequentemente distribuídas simetricamente em diversas regiões dos maxilares. A tomografia computadorizada, que oferece vistas axial, sagital e frontal, é útil na avaliação dessas lesões. Este artigo apresenta o caso de uma paciente em que a DCOF foi diagnoasticada com base nos achados clínicos e radiográficos.

A retrospective study of the prevalence and characteristics of dens invaginatus in a sample of the Turkish population

Objective: The purpose of this study was to assess the prevalence of dens invaginatus and to classify the types of dens invaginatus in a sample of the Turkish population. Study Design: A retrospective study was performed using periapical and panoramic radiographs of 5355 patients who presented to the Department of Oral Diagnosis and Radiology at the Ondokuz Mayýs University Dentistry Faculty between January 2009 and December 2010. Maxillary and mandibular anterior teeth were evaluated for the presence and characteristics of dens invaginatus. Statistical evaluation of the presence of dens invaginatus related to gender was performed by the Pearson chi-squared test. Results: Dens invaginatus was observed in 116 of 4556 subjects, with a frequency of 2.5%. There was only one periapical lesion in teeth with type I dens invaginatus, but 8.1% of patients with type II and 87.5% of patients with type III dens invaginatus had apical periodontitis at the time of referral. There were 116 (72%) females and 32 (27%) males with dens invaginatus. Conclusion: This data represents the only study carried out in a large population in Turkey, and no dens invaginatus was found in mandibular teeth. The most commonly observed type of dens invaginatus was type I (69.8%) (AU)

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Prevalence and distribution of odontogenic and nonodontogenic cysts in a Turkish Population

Objective : To determine the relative frequency and distribution of odontogenic and nonodontogenic cysts in alarge Turkish population. Study Design A retrospective survey of jaw cysts was undertaken at the Oral Diagnosis and Radiology and Oral and Maxillofacial Surgery Department, Ondokuz Mayýs University Dental School, Samsun, Turkey . Data were retrieved from clinical files, imaging, and histopathology reports from 2000 to 2008; a total of 12,350 patients were included . In each case, we analyzed age, gender, type and number of cysts, and cyst location . Imaging pat -terns and pathologies associated with cystic lesions were also determined .Results : The prevalence of odontogenic and nonodontogenic cysts was 3.51%; males were affected more frequently than females . There were 452 odontogenic cysts (98.5%) and seven nonodontogenic cysts (1.5%). The most frequent odontogenic cyst was radicular (54.7%), followed by dentigerous (26.6%), residual (13.7%), odontogenic keratocyst (3.3%), and lateral periodontal cyst (0.2%). Nasopalatine duct cyst (1.5%) was the only nonodontogenic cyst . By age, cysts peaked in the third decade (24.2%). Concerning location, no statistically significant difference was found between the maxilla and mandible (p>0.05). The most frequent radiological feature of these lesions was unilocular cyst (93.7%). Pathologies associated with cystic lesions occurred in 14.7%.Conclusion : The prevalence of both odontogenic and nonodontogenic cysts were lower than that reported in many other studies . In our study population, cysts were mainly inflammatory in origin (AU)

An unusual case of talon cusp on a geminated tooth

Talon cusp is a developmental anomaly seen on the lingual surface of anterior teeth. Gemination is an anomaly caused by a single tooth germ that attempted to divide during its development. These developmental anomalies may cause clinical problems including esthetic impairment, pain, caries and tooth crowding. This paper presents an unusual case of gemination accompanied by talon cusp in permanent dentition. The clinical and radiographic findings are described.

Cúspide em garra (talon cusp) é uma anomalia de desenvolvimento que occore na face lingual de dentes anteriores. Geminação é uma anomalia causada quando um germe dental tenta se dividir durante a fase de desnvolvimento. Estas anomalias de desenvolvimento podem causar problemas clínicos tais como comprometimento estético, dor, cárie e apinhamento dental. Este artigo relata um caso incomum de geminação acompanhado de cúspide em garra (talon cusp) na dentição permemente. Os achados clínicos e radiográficos são descritos.

Benign cementoblastoma: a case report

In the past the benign cementoblastoma was recognized in the World Health Organization’s classification of odontogenic tumours as one of the cementoma neoplasias. Recently the benign cementoblastoma is included into ‘Mesenchyme and/or odontogenic ectomesenchyme, with or without odontogenic epithelium’ odontogenic tumours. Benign cementoblastoma has characteristic radiologic and microscopic features and it appears to be fused to the tooth roots. Symptoms may be totally absent, and when they do occur, pain and swelling are frequent findings. The final diagnosis is usually made histopathologically, but the clinical diagnosis is comparatively easy if it is examined radiographically. The tumour has unlimited growth potential. Most frequently tends to be associated with an erupted permanent tooth, most often the first molar: rarely has an association with an impacted or partial impacted tooth been reported. This case represents a case of benign cementoblastoma associated with a partially impacted mandibular third molar

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Regional odontodysplasia of the deciduous and permanent teeth associated with eruption disorders: A case report

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Regional odontodysplasia (RO) is an unusual, non-hereditary anomaly of the dental hard tissues with characteristic clinical, radiographic and histological findings. Clinically, RO affects the primary and permanent dentition in the maxilla and mandible or both jaws. Radiographically, there is a lack of contrast between the enamel dentin, both of which are less radiopaque than unaffected counterparts. Additionally, enamel and dentin layers are thin, giving theteeth a “ghost-like” appearance. Histologically, areas of hypocalcified enamel are visible and enamel prisms appear irregular in direction. Coronal dentin is fibrous, consisting of clefts and a reduced number of dentinal tubules; radicular dentin is generally more normal in structure and calcification.The RO etiology is uncertain; numerous factors have been suggested and considered as local trauma, irradiation,hypophosphatasia, hypocalcemia, hyperpyrexia. The treatment of RO has given rise to controversy. These cases require a continuous and multidisciplinary approach. Most clinicians advocate extracting the affected teeth as soon as possible and inserting a prosthetic replacement. Other clinicians prefer restorative procedures, if possible, to protectthe affected erupted teeth.A case of RO in an 8 year-old male whose chief complaint was the absence of eruption of permanent teeth is presented. Clinical, radiographic and histological findings are described (AU)