Prevalence of ankylosing spondylitis in Spain: EPISER2016 Study.

Objective: The aim of this study was to estimate the prevalence of ankylosing spondylitis (AS) in Spain.Method: This is a cross-sectional, population-based study of people aged 20 years or older in Spain. Randomly selected individuals were contacted by telephone and rheumatic disease screening was performed. If the first screening was positive, medical records were then reviewed and/or a telephone questionnaire was conducted by a rheumatologist, followed by an appointment if necessary. Cases had to fulfil the modified New York (mNY) criteria.Results: In total, 4916 individuals were included, of whom 355 had a positive screening result for AS. Of these, 11 were classified as AS. An additional individual who reported a prior diagnosis of rheumatoid arthritis had a diagnosis of AS confirmed on review of the medical records. Estimated prevalence was 0.26% (95% CI 0.14-0.49).Conclusion: EPISER2016 is the first population-based study to estimate the prevalence of AS in Spain, which has been estimated as being similar to that in other European countries.

Systemic blockade of TNF-α does not improve insulin resistance in humans.

The purpose of this study was to determine whether long-term modulation of inflammatory activity by tumor necrosis factor (TNF)-α inhibitors has some influence on insulin resistance (IR). 16 active rheumatoid arthritis (RA) patients without CV risk factors treated with anti-TNF-α agents were included in this study. RA activity by disease activity score 28, IR by HOMA2-IR, body composition by impedance analysis, physical activity by accelerometry, abdominal fat distribution by magnetic resonance imaging, and serum level of key adipokines by ELISA were measured at baseline and during a 1-year follow-up period. Patient body mass index increased significantly (26.94 ± 3.88 vs. 28.06 ± 4.57 kg/m2, p=0.02) after 1 year of treatment. Body composition, in terms of fat and fat-free mass, remained unchanged except for a significant elevation in body cell mass (25.50 ± 4.60 vs. 26.60 ± 3.17 kg, p=0.02). Basal levels of IR in the RA patients included in this study were significantly higher than healthy controls (1.6 ± 0.8 vs. 1.11 ± 0.56, p=0.011) but did not change during the follow-up. Nor did basal concentrations of adiponectin, visfatin, leptin, ghrelin, resistin, and apelin in response to anti-TNF-α treatment; only retinol-binding protein 4, showed a significant increase (51.7 ± 32.7 vs. 64.9 ± 28.4 µg/ml, p=0.03) at the end of the study. IR, adiposity distribution, and serum levels of most adipokines are not significantly affected by long-term inhibition of TNF-α in RA patients. Our data suggest that although systemic blockade of TNF-α exerts an anticachectic effect in RA patients, it does not seem to play a major role in IR.

Distracción osteogénica vertical de injerto microvascularizado de peroné para reconstrucción mandibular y optimización de la rehabilitación implantológica / Vertical distraction osteogenesis of microvascularised fibular grafts for mandibular reconstruction and optimisation of rehabilitation implantology

Introducción: En la actualidad, se considera que el injerto microvascularizado de peroné es la mejor opción de tratamiento para las reconstrucciones mandibulares de defectos óseos mayores de 6 cm. No obstante, debido a su limitada altura, presenta una importante desventaja en cuanto a la rehabilitación protésica, particularmente en aquellos casos de resecciones mandibulares parciales con una hemimandíbula contralateral dentada. Material y métodos: Presentamos un caso clínico de mixoma mandibular tratado mediante resección quirúrgica y reconstrucción mandibular primaria mediante injerto microvascularizado de peroné, al que se sometió a distracción osteogénica vertical para optimizar la posterior rehabilitación implantológica. Resultados: La discrepancia de altura se solucionó completamente y se colocaron tres implantes osteointegrados en el área distraída, lo cual permitió una rehabilitación protésica muy satisfactoria, tanto estética como funcionalmente. Conclusiones: La distracción osteogénica vertical de peroné constituye una excelente opción terapéutica para igualar la altura del peroné a la del reborde alveolar de la hemimandíbula dentada contralateral, tras la reconstrucción de defectos mandibulares extensos, de cara a una posterior rehabilitación implantológica, lo que permite llevar a cabo ésta con resultados, tanto funcionales como estéticos, muy satisfactorios. Además, no presenta los inconvenientes de otras técnicas empleadas en estos pacientes, como el peroné en doble barra (mayor dificultad técnica y mayor riesgo de trombosis en el pedículo) y el empleo de injertos “onlay” (morbilidad añadida en la zona de toma del injerto)(AU)

Introduction: Free vascularized fibular flap is considered the treatment of choice in mandibular reconstruction for extensive bone defects. But, when the reconstruction involves a dentate mandible, the fibula has the limit as it doesn’t offer sufficient bone height to restore the alveolar arch up to the occlusal plane. Therefore, the deficiency in bone height makes implant placement impractical. Materials and methods: We report a case of vertical distraction osteogenesis of a free vascularized fibula flap used to reconstruct a hemimandible after resection of an odontogenic myxoma. The distraction device was applied intraorally. Distraction of 0.5 mm per day was performed followed by 3 months of consolidation period. Results: The vertical discrepancy between the fibula and the native hemimandible was corrected. The increase of vertical bone height was stable and enabled placement of dental implants without any complications. Conclusions: We believe that vertical distraction osteogenesis of free vascularized flaps is a reliable technique that optimizes implant positioning for ideal prosthetic rehabilitation, after mandibular reconstruction following tumour surgery(AU)

Anquilosis mandibular: una frecuente secuela por Noma / Mandibular ankylosis: a Noma frequent sequel

Conocemos poco sobre el Noma o Enfermedad Cancrum Oris. Esta entidad afecta a niños con malnutrición crónica, poca higiene oral, poco desarrollo sanitario y enfermedades concurrentes debilitantes. Existe un consenso general sobre que el Noma empieza como una gingivitis; en su fase aguda, puede haber respuesta al tratamiento antibiótico, no obstante, las secuelas tras su curación incluyen daños estéticos y funcionales variables, que pueden requerir cirugía reconstructiva. Presentamos 3 casos de anquilosis mandibular que en el examen preoperatorio presentaron dimorfismo mandibular, fusión de hueso maxilar y mandibular y anquilosis dela articulación témporomandibular. La ortopantografía y la Tomografía Axial Computarizada (TAC) fueron muy útiles en la planificación de la cirugía. El tratamiento quirúrgico consistió en todos los casos en la resección del hueso anquilótico y la reconstrucción con injerto condrocostal en uno de los casos. En el postoperatorio se siguió fisioterapia intensiva. Clínicamente todos los pacientes mostraron una gran mejoría en la masticación, la alimentación y el habla, obviamente debido a una buena oclusión. El problema más común fue la baja colaboración en la rehabilitación mandibular por parte de los pacientes (AU)

Little is known about Noma or Cancrum Oris Disease. This entity affects to children with chronic malnutrition, poor oral hygiene, poor environmental sanitation and debilitating concurrent illness. There is general consensus that nomad starts as gingivitis. The acute stage responds readily to antibiotic treatment. The sequelae after healing include variable functional and aesthetic impairments, which require reconstructivesurgery. We report 3 cases of mandibular ankylosis. Preoperative examination revealed mandibular dismorphism, fusion of maxilla and mandible and ankylosis of the temporomandibular joint. Orthopantography and Computed Thomography scan (TCS) were very useful in planning surgery. The surgical treatment performed consisted in ankylotic bone resection in all cases, accompanied with condrocostal graft reconstruction in one case. All patients continued postoperatively with an intensive physiotherapy. Clinically all patients showed an improvement in eating, chewing and speaking, obviously due to good occlusion. Low collaboration with the mandibular rehabilitation was the most common problem (AU)

Síndrome de Gorlin-Goltz: serie de 7 casos / Gorlin-Goltz Syndrome. A 7 cases serie

El Síndrome Névico Basocelular (SNBC) o Síndrome de Gorlin-Goltz es un trastorno autosómico dominante, caracterizado principalmentepor carcinomas basocelulares, múltiples queratoquistes y anomalías esqueléticas.El presente trabajo revisa a este desconocido síndrome dada la importanciaque tiene para nosotros como especialistas. Presentamos un total desiete casos recogidos por el Servicio Cirugía Oral y Maxilofacial desde 1992al 2008, con seguimiento medio de 10 años, determinamos la frecuencia delas características clínicas en nuestra serie de SNBC y el manejo terapéuticode las mismas(AU)

Nevoid Basal Cell Carcinoma Syndrome (NBCSS) or Gorlin-Goltz Syndrome is an autosomal dominant disorder principallycharacterized by cutaneous basal cell carcinomas, multiplekeratocysts and skeletal anomalies. This report reviews currentknowledge of this disorder that is important to us as specialists. Theauthors reviewed seven case files from the Department of Oral andMaxillofacial Surgery of H. U. La Princesa from 1992-2008. Theaverage follow up was 10 years; we determine the frequency of theclinical features and treatment in our series of NBCCS(AU)

Hipertrofia bilateral de apófisis coronoides / Bilateral Hypertrophy of the coronoid process

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An open-label pilot study of infliximab therapy in diffuse cutaneous systemic sclerosis.

AIM: The safety and potential efficacy of a chimaeric anti-tumour necrosis factor alpha monoclonal antibody (infliximab) were examined in diffuse cutaneous systemic sclerosis (dcSSc). METHODS: A 26-week open-label pilot study in which 16 cases of dcSSc received five infusions of infliximab (5 mg/kg). Clinical assessment included skin sclerosis score, scleroderma health assessment questionnaire, self-reported functional score and physician global visual analogue scale. Collagen turnover, skin biopsy analysis and full safety evaluation were performed. RESULTS: There was no significant change in skin score at 26 weeks but a trend for lower modified Rodnan skin score at 22 weeks (OR 17, 95% CI 6 to 46) compared with peak value (OR 29, 95% CI 11 to 44; p = 0.10). Serum aminoterminal propeptide of type III collagen level was significantly lower at week 26 compared with baseline (p = 0.03). Secretion of type I collagen by dermal fibroblasts was reduced at 26 weeks compared with baseline (p = 0.02). There were no deaths during the study and no suspected unexpected serious adverse reactions. 21 serious adverse events (AE) occurred in seven subjects, mostly attributable to dcSSc. 127 distinct AE occurred in 16 subjects. Of these, 19 AE (15%) were probably or definitely related to infliximab treatment. Eight (50%) patients prematurely discontinued infliximab. Anti-infliximab antibodies developed during the study in five subjects and were significantly associated with suspected infusion reactions (p = 0.025). CONCLUSION: In dcSSc infliximab did not show clear benefit at 26 weeks but was associated with clinical stabilisation and a fall in two laboratory markers of collagen synthesis. The frequency of suspected infusion reactions may warrant additional immunosuppression in any future studies in systemic sclerosis.

Metástasis cervical contralateral en el carcinoma epidermoide de la cavidad oral. Estudio clínico analítico retrospectivo en 315 pacientes primariamente tratados con cirugía / Contralateral neck metastasis in squamous cell carcinoma of the oral cavity. An analytical retrospective clinical study of 315 patients primarily treated with surgery

Objetivos. Existen en la literatura numerosos estudios en relacióncon los factores pronósticos implicados en la aparición de metástasis cervicalesipsilaterales en el carcinoma epidermoide de cavidad oral. Sin embargo,no existen estudios clínicos amplios acerca de la asociación de factoresclínico-patológicos y la aparición de metástasis cervicales contralaterales trasla resección quirúrgica del tumor primario. El propósito de este estudio es elanálisis de los factores implicados en la aparición de metástasis cervicalcontralateral en pacientes con carcinoma epidermoide de cavidad oral primariamentetratados con cirugía.Pacientes y métodos. Se analizó una serie de 315 pacientes consecutivos concarcinoma epidermoide de la cavidad oral no tratados previamente. Serealizó un estudio complementario del subgrupo de 203 pacientes concarcinoma epidermoide del borde lateral de la lengua libre, por tratarse deun grupo muy prevalente en la serie analizada. Todos los pacientes recibierontratamiento quirúrgico con o sin tratamiento radioterápico adyuvante.Varias fueron las variables clínicas e histopatológicas analizadas, como son:las características clínicas del tumor, el estadio tumoral, el grado de diferenciaciónhistológica, el tipo de disección cervical, la supervivencia enfermedad-específica, los márgenes quirúrgicos en la pieza resecada, la extensiónganglionar extracapsular, la diseminación perineural y la afectación ósea.Resultados. La duración media del seguimiento de los pacientes que sobrevivieronfue de 70,9 ± 49,6 meses. Ochenta y tres pacientes murieron a causade la enfermedad a lo largo del seguimiento. Cuarenta y siete de estos pertenecíanal subgrupo de pacientes con carcinoma epidermoide de lengua.Un total de 147 pacientes permanecía vivo sin evidencias de recurrencia dela enfermedad al final del periodo de seguimiento, 116 de los cuales correspondíanal subgrupo de pacientes con carcinoma epidermoide de lengua...(AU)

Objectives. There are numerous studies in the literatureon the prognostic factors involved in the appearance of ipsilateralneck metastasis in squamous cell carcinoma of the oral cavity.However, there are no extensive clinical studies on the associationof clinicopathological factors and the appearance of contralateralneck metastasis after the surgical resection of the primary tumor.The object of this study is to analyze the factors implied in theappearance of contralateral neck metastasis in patients withsquamous cell carcinoma of the oral cavity treated primarily withsurgery.Patients and methods. A series of 315 consecutive patients withsquamous cell carcinoma of the oral cavity, who had not beentreated previously, were analyzed. A complementary study of asubgroup of 203 patients with squamous cell carcinoma of thelateral border of the mobile tongue was carried out, as this was avery prevalent group in the series analyzed. The patients all receivedsurgical treatment with or without adjuvant radiotherapy treatment.Various clinical and histopathological variables were analyzed, suchas the clinical characteristics of the tumor, tumor stage, degree ofhistological differentiation, type of neck dissection, disease-specificsurvival, surgical margins of the resected specimen, extracapsularlymph node extension, perineural dissemination and boneinvolvement.Results. The mean follow-up of patients who survived was 70.9 ±49.6 months. Eighty-three patients died as a result of the diseaseduring the follow-up period. Forty-six of these belonged to thesubgroup of patients with squamous cell carcinoma of the tongue.A total of 147 patients remained alive with no evidence of diseaserecurrence at the end of the follow-up period, 116 of whomcorresponded to the subgroup of patients with squamous cellcarcinoma of the tongue...(AU)

Permanent neonatal diabetes caused by a homozygous nonsense mutation in the glucokinase gene.

Glucokinase deficiency is an unfrequent cause of permanent neonatal diabetes (PND), as only seven patients have been reported, either homozygous for a missense or frameshift mutation or compound heterozygous for both of them. We report here the first known case caused by a homozygous nonsense mutation (Y61X) in the glucokinase gene (GCK) that introduces a premature stop codon, generating a truncated protein that is predicted to be completely inactive as it lacks both the glucose- and the adenosine triphosphate-binding sites. The proband, born to consanguineous parents, was a full-term, intra-uterine growth-retarded male newborn who presented with a glycaemia of 129 mg/dL (7.16 mmol/L) on his second day of life, increasing thereafter up to 288 mg/dL (15.98 mmol/L) and 530 mg/dL (29.41 mmol/L) over the next 24 h, in the face of low serum insulin (<3 muIU/mL; <20.83 pmol/L). He was put on insulin on the third day of life. Insulin has never been discontinued since then. The patient was tested negative for anti-insulin and islet cell antibodies at age 5 months. His father had non-progressive, impaired fasting glucose for several years. The mother was found to be mildly hyperglycaemic only when her glucose was checked after the child was diagnosed. In conclusion, biallelic GCK loss should be considered as a potential cause of PND in children born to consanguineous parents, even if they are not known to be diabetic at the time of PND presentation.

Injerto libre braquial medial / Free medial arm graft

Introducción. Entre las reconstrucciones de defectos titularesde cabeza y cuello, el injerto libre microvascularizado braquial medial noha adquirido mucha popularidad debido a las variaciones anatómicas quese reflejan en la vascularización de éste. Nuestro objetivo es realizar unadescripción de la anatomía y técnica quirúrgica, así como una revisión dela literatura describiendo las ventajas y desventajas de este tipo de injerto.Material y método. Presentamos el caso de una paciente con carcinomaepidermoide de mucosa yugal izquierda con afectación ganglionar ipsilateral.Se procedió a su resección con márgenes más disección cervical funcional.La reconstrucción del defecto se llevó a cabo mediante un injertolibre microvascularizado braquial medial de brazo izquierdo.Discusión. Pensamos que el injerto libre braquial medial de brazo se tratade una opción más segura a la hora de la reconstrucción de defectos cervicofaciales,aportando una serie de ventajas entre las que destacan: nosacrificio de una arteria terminal, cierre primario de la zona donante, mínimodefecto estético, y poseer una piel fina, elástica y sin vello(AU)

Introduction. Free medial microvascularized arm graftshave not become very popular for the reconstruction of head andneck defects due to anatomic variations in their vascularization.Our objective was to describe the anatomy and surgical techniqueand to review the literature on the advantages and disadvantagesof free medial arm grafts.Material and methods. We report the case of a patient withsquamous cell carcinoma of the left jugal mucosa with same-sidelymph node involvement. The tumor was resected with margins anda functional cervical dissection was performed. The defect wasreconstructed using a free medial microvascularized graft from theleft arm.Discussion. We believe that free medial arm grafts are a safer optionfor the reconstruction of cervicofacial defects and that they offeradvantages, among which can be highlighted: not sacrificing aterminal artery, primary closure of the donor zone, minimal estheticdefect, and yielding fine, elastic, hairless skin(AU)

Tumor hinchado de Pott. Recidiva tras 10 años asintomático / Pott’s Puffy tumor. Recurrence after being asymptomatic for 10 years

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Contralateral lymph neck node metastasis of primary squamous cell carcinoma of the tongue: a retrospective analytic study of 203 patients.

In primary squamous cell carcinoma (SCC) of the oral cavity, many clinical and histopathological factors have been described as predictive for cervical lymph-node metastasis, but there are no data available on this association for surgical resection of lateral tongue primary SCC. The aim of this study was to analyse factors related to contralateral neck relapse in a series of 203 consecutive patients with SCC of the lateral aspect of the tongue treated by surgery with or without adjuvant radiotherapy. Several clinical features were analyzed. Histological study included pTNM classification, tumour size, surgical margins, extracapsular spread of lymph-node metastasis, perineural infiltration, peritumoral inflammation and bone involvement. The mean duration of follow up for surviving patients was 70.9+/-49.6 months; 47 patients eventually died of the disease and 116 patients are alive with no evidence of recurrence. The mean disease-specific survival time was 149+/-7 months. Twenty (9.8%) patients developed ipsilateral and nine (4.4%) contralateral neck recurrence. The mean period of time from surgery to contralateral neck recurrence was 11.4 months (range 3-27 months). Fourteen of the 20 ipsilateral and 8 of the 9 contralateral neck relapse patients eventually died of the disease. Histopathological grading and peritumoral inflammation were found to be statistically significant (P<0.05). Clinical and pathological lymph neck node status was not found to be associated with the appearance of contralateral lymph neck node relapse. Due to the increased risk of contralateral neck relapse within the first 2 years of surgery, close surveillance is mandatory at this time.

Radial forearm free flap for reconstruction of the oral cavity: clinical experience in 55 cases.

OBJECTIVE: Since the advent of modern microvascular techniques, the radial forearm free flap (RFFF) has become a reliable method for reconstruction of defects within the oral cavity. The purpose of the present study was to evaluate our experience with the use of the RFFF for the reconstruction of oral cavity defects after tumor resection. STUDY DESIGN: During a 9-year period, 103 consecutive patients were treated in our department for the reconstruction of oral defects after tumoral ablation by means of microvascularized free flaps. Fifty-five patients were reconstructed by means of the RFFF. Patients were treated for benign (n = 1) and malignant (n = 54) entities. All the patients underwent an abdominal split-thickness skin graft for the closure of the donor site. RESULTS: Fifty-five patients underwent reconstruction by means of the RFFF after resection of the oral cavity. Squamous cell carcinoma was present in 54 patients. A mean age of 55.5 years was observed (range 16-78). Thirty-nine patients (70.9%) were men and 16 (29.1%) women. Primary reconstruction was achieved in 52 patients (96.3%). A fasciocutaneous graft was used in all of the cases, with a mean size of 7.39 x 5.17 cm. The mean flap ischemic time was 56.02 minutes. During the immediate follow-up period, revision of the vascular anastomosis was necessary in 18.9% of the cases owing to flap ischemia. CONCLUSION: Our results revealed that the RFFF is a reliable method for reconstructing a wide range of oral cavity defects with an acceptable low morbidity rate. It provides adequate bulkiness and pliability, resulting in adequate reconstruction of a wide variety of defects within the oral cavity.

Carcinoma neuroendocrino de células pequeñas de la región nasosinusal / Small cell neuroendocrine carcinoma of the sinonasal region

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Trombosis de malformación vascular cervical en paciente con neurofibromatosis de tipo 1 / Thrombosis of a cervical vascular malformation in a patient with neurofibromatosis Type 1

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Candidiasis hiperplásica crónica de la mucosa oral / Chronic hyperplastic candidosis of the oral mucosa

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Carcinoma mucoepidermoide de base de lengua. Abordaje quirúrgico mediante labiomandibulotomía media / Mucoepidermoid carcinoma of the base of the tongue. A surgical approach by means of the median labiomandibulotomy

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Factores pronósticos en neoplasias malignas primarias de glándulas salivares. Estudio retrospectivo de 20 años / Prognostic factors in primary malignant salivary gland neoplasms. A 20-year retrospective study

Objetivo. Analizar los posibles factores pronósticos que pueden influir en la supervivencia y el desarrollo de recurrencias en nuestra serie de pacientes con neoplasias malignas primarias de glándulas salivares. Diseño del estudio. Se realiza un estudio retrospectivo de 75 pacientes con carcinomas glandulares tratados entre 1980 y el 2003. En todos los casos el tratamientorealizado ha sido la cirugía. Se administró radioterapia postoperatoria en un 52% de los pacientes. Se emplea el modelo de Kaplan-Meier para el análisis de supervivencia y el periodo libre de enfermedad. Así mismo, se analiza la posible asociación entre los diversos factores pronósticos y estas variables mediante el test Log Rank para el estudio univariante y el modelo de Cox parael multivariante. Resultado. La supervivencia global y causa-específica de la serie a 5 y 10 años ha sido del 74,9 y 63 y del 77,7 y 65,3% respectivamente. El periodo libre de enfermedad ha sido de 51,8 y 43,2% a 5 y 10 años. De los factores analizados, la localización tumoral, el sexo, el estadio patológico, el tamaño tumoral, la parálisis facial, la infiltración perineural y la infiltración vascular han mostrado una influencia estadísticamente significativa en la supervivenciay/o recurrencias (p<0,05), siendo factores independientes la parálisis facial y la infiltración vascular. Conclusiones. En la serie de pacientes presentada, los factores pronósticos más relevantes han sido la parálisis facial al diagnóstico y la infiltración vascular tumoral. La radioterapia postoperatoria complementaria no parece influir en la evolución de nuestros pacientes

Objective. To analyze the possible prognostic factors forsurvival and disease-free survival in a group of patients with primary malignant salivary gland tumors. Design. Seventy-five patients with salivary gland carcinomas were studied retrospectively from1980 to 2003. All cases were initially treated with surgery. Postoperative radiotherapy was applied in 52% of the patients. Survival and disease-free survival were analyzed with the Kaplan-Meier method. The association between the different prognostic factorsand survival was studied with the Log Rank test for univariate analysis and the Cox proportional model for multivariate analysis. Results. The 5-year and 10-year crude survival rates were 74.9% and 63% and cause-specific survival rates were 77.7% and 65.3% respectively. The 5-year and 10-year disease-free survival rates were 51.8% and 43.2%. Tumor location, sex, pathologic stage, tumor size, facial palsy, perineural spread and vascular spread showed statistical significance in survival and recurrence (p<0.05). Facial palsy and vascular spread were prognostic factors in the multivariate analysis. Conclusion. In our group of patients, facial palsy and vascular spread are the most important factors for survival and recurrence. Postoperative radiotherapy does not appear to improve the prognosis of our patients