Desordens hiperostóticas em American Bully / Hiperostotic disorders in american bully

O complexo de desordens hiperostóticas é uma condição rara e autolimitante, que tem as mesmas características histopatológicas, que cursa com proliferação óssea de caráter não neoplásico. Acomete cães jovens de raças distintas, com variabilidade quanto ao tipo de proliferação óssea e quanto aos ossos acometidos. O complexo é composto pela osteopatia craniomandibular, hiperostose da calota craniana e osteodistrofia hipertrófica. Podendo estar presente nos ossos da calota craniana, mandíbulas, coluna cervical e esqueleto apendicular. O presente relato, descreveu o quadro de uma cadela, da raça American Bully, não castrada, três meses de idade, que foi atendida com queixa de aumento de volume doloroso das mandíbulas, hiporexia e sialorreia há 15 dias, apresentando ao exame físico, amplitude de movimento diminuída e sensibilidade dolorosa da articulação temporomandibular, espessamento firme bilateral do crânio em região de fossa temporal, espessamento palpável de consistência firme das mandíbulas e crepitação respiratória. Após avaliação clínica e realização de exames complementares, chegou-se ao diagnóstico presuntivo, de complexo de desordens hiperostóticas. Foi instituído como conduta terapêutica o suporte analgésico, sendo eficaz para a manutenção das necessidades fisiológicas até a paciente alcançar a fase adulta. O prognóstico para esta paciente foi considerado bom, uma vez que não havia indícios de anquilose da articulação temporomandibular e/ou manifestações neurológicas.

The complex of hyperostotic disorders is a rare and self-limiting condition, which has the same histophatological characteristics, which courses with non-neoplastic bone proliferations. It affects young dogs of different breeds, with variability the bones affected. The complex is composed of craniomandibular osteopathy, calvarial hyperostotic syndrome and hypertrophic osteodystrophy. It may be present in the bones of the skullcap, jaws, cervical spine and appendicular skeleton. The present report describes the condition of a female dog, American Bully breed, entire, three months old, with a complaint of painful swelling of the jaws, hyporexia and drooling for 15 days, presenting on physical examination, reduced amplitude and pain of the temporomandibular joint, bilateral firm thickening of the skull in the temporal fossa region, palpable firm-consistent thickening of the mandibles and respiratory crackle. After clinical evaluation and complementary tests, a presumptive diagnosis of hyperostotic disorders complex was reached. It was instituted pain management as a treatment, being effective for the maintenance of physiological needs until the patient reaches the adulthood. The prognosis for this patient was considered good, since there was no evidence of temporomandibular joint ankylosis and/or neurological manifestations.

Estudio anatómico de la hiperostosis porótica en órbitas oculares: cribra orbitalia en la población arqueológica de Tutuquén, región del Maule, Chile / Anatomical study of porotic hyperostosis in the eye orbits: cribra orbitalia in the archaeological population of Tutuquén, Maule region, Chile

RESUMEN: La cribra orbitalia o hiperostosis porótica del techo orbitario es considerada una manifestación ósea microperforativa de condiciones patológicas hematológicas, especialmente la anemia ferropénica. Este hallazgo, se enmarca de manera casi exclusiva a estudios en poblaciones arqueológicas. El propósito de este estudio fue describir la expresión de cribra orbitalia en una población arqueológica de Chile central. Restos óseos de 32 individuos fueron analizados, los cuales fueron obtenidos de la población del Monumento Arqueológico Cementerio Tutuquén, los que están depositados en el Museo Regional de Rancagua. En cada una de la muestras incluidas, se evaluaron períodos de datación, sexo, rango etáreo, presencia v/s ausencia de cribra orbitalia y en los casos en que ésta estuvo presente, se determinó su intensidad mediante visión directa con lente de aumento. Un 59,38 % de los individuos presentó cribra orbitalia. De éstos, 26,32 % fueron de sexo femenino, 31,58 % de sexo masculino y 42,10 % indeterminado. De los individuos del período 10.000 AP, 33,33 % presentó cribra orbitalia; de los individuos del período 7.000 AP un 50 % y de los individuos pertenecientes al período 1.000 AP, un 68,4 %. Al clasificar la severidad de la cribra orbitalia se observó que 31,57 % de los individuos presentaron Cribra orbitalia tipo I; 36,84 % tipo II; 10,52 % tipo III; 10,52 % tipo IV y 10,52 % tipo V. Los datos aportados complementarán el conocimiento morfopatológico de la órbita ocular humana desde el estudio de poblaciones arqueológicas.

SUMMARY: The cribra orbitalia or porotic hyperostosis of the orbital roof is considered a microperforative bone manifestation of hematological pathological conditions, especially iron deficiency anemia. This finding is almost exclusively part of studies in archaeological populations. The purpose of this study was to describe the expression of cribra orbitalia in an archaeological population of central Chile. Bone remains of 32 individuals were analyzed, which were obtained from the population of the Tutuquén Cemetery Archaeological Monument, which are deposited in the Regional Museum of Rancagua. In each of the included samples, dating periods, sex, age range, presence v / s absence of cribra orbitalia were evaluated and in the cases in which it was present, its intensity was determined by direct vision with a magnifying lens. The 59.38 % of the individuals presented cribra orbitalia. Of these, 26.32 % were female, 31.58 % male, and 42.10 % undetermined. Of the individuals in the period 10,000 BP, 33.33 % presented cribra orbitalia; of the individuals of the period 7,000 AP, 50% and of the individuals belonging to the period 1,000 AP, 68.4 %. When classifying the severity of the cribra orbitalia, it was observed that 31.57 % of the individuals had type I; 36.84 % type II; 10.52 % type III; 10.52 % type IV and 10.52 % type V. The data provided will complement the morpho-pathological knowledge of the human eye orbit from the study of archaeological populations.

Presentación atípica de síndrome de CamuratiEngelmann en una paciente femenina colombiana. Reporte de caso / Atypical presentation of Camurati-Engelmann syndrome in a colombian female patient. Case report

Resumen El síndrome de Camurati-Engelmann, también conocido como displasia diafisaria progresiva, es una enfermedad rara, autosómica dominante y con una prevalencia de uno por cada millón de habitantes. Genera mutaciones del factor de crecimiento transformante beta, que participa en la proliferación ósea. Son frecuentes las manifestaciones osteomusculares y neurológicas, con escasas expresiones de laboratorio. El diagnóstico se basa en la clínica, los hallazgos radiológicos y la confirmación genética; el tratamiento se dirige al control sintomático y el pronóstico es incierto. La presente publicación tiene como objetivo compartir con la comunidad médica el tercer caso de síndrome de Camurati-Engelmann conocido en Colombia. Se trata de una paciente femenina de 33 años con cuadro clínico de distonías intensas y signos y síntomas característicos de este síndrome, cuyo diagnóstico fue confirmado por prueba molecular, encontrando la presencia de la variante patogénica p.Arg156Cys en el gen TGF-β1, con presentación de novo. MÉD.UIS.2021;34(1): 119-27.

Abstract Camurati-Engelmann syndrome, also known as progressive diaphyseal dysplasia, is a rare, autosomal dominant disease with a prevalence of one per million inhabitants. It generates mutations of the transforming growth factor beta, which participates in bone proliferation. Osteomuscular and neurological manifestations are frequent, with few laboratory expressions. The diagnosis is based on the clinic, radiological findings, and genetic confirmation, treatment is aimed at symptom control and prognosis is uncertain. The objective of this publication is to share with the medical community the third case of Camurati-Engelmann syndrome known in Colombia. This is a 33-year- old female patient with a clinical picture of intense dystonia and characteristic signs and symptoms of this syndrome, whose diagnosis was confirmed by molecular testing, finding the presence of the pathogenic variant p.Arg156Cys in the TGF-β1 gene, with de novo presentation. MÉD.UIS.2021;34(1): 119-27.

The presence of hyperostosis in Atlantic moonfish, Selene setapinnis (Mitchill, 1815) in the Brazilian Coast - case report / [A presença de hiperostoses no peixe-galo-sem-penacho, Selene setapinnis (Mitchill, 1815) na costa brasileira - relato de caso]

The occurrence of hyperostotic bones is common in marine fish, especially in the Carangidae family, despite few records of this condition in fishes from Brazilian waters. The present study describes the occurrence of hyperostosis in Atlantic moonfish (Selene setapinnis) in, Rio de Janeiro State, Brazil. Radiographs of the fish specimens were taken and all of them presented hyperostosis in at least three different regions: cleithrum, pterygiophores and pleural ribs. The observed pattern is different from previously described for other species of the same genus. It is the first record for the species and a valuable contribution to the study of bone diseases in fishes from the Brazilian coast.(AU)

A ocorrência de ossos hipertostóticos é comum em peixes marinhos, especialmente na família Carangidae, apesar de poucos relatos dessa condição em peixes de águas brasileiras. O presente estudo descreve a ocorrência de hiperostose no peixe-galo-sem-penacho (Selene setapinnis) no estado do Rio de Janeiro, Brasil. Radiografias dos espécimes de peixes foram feitas e todos os exemplares apresentaram hiperostoses em pelo menos três regiões diferentes: cleithrum, pterigióforos e costelas pleurais. O padrão observado é diferente do descrito previamente para outras espécies do mesmo gênero. Este é o primeiro relato para a espécie e é uma contribuição valiosa para o estudo de doenças ósseas em peixes da costa brasileira.(AU)

Research Advances of Human Homologue of Mouse Progressive Ankylosis Protein and Bone and Joint Diseases / 中国医学科学院学报

The human homologue of mouse progressive ankylosis protein(ANKH)is an inorganic pyrophosphate transport regulator,which regulates tissue mineralization by controlling the level of inorganic pyrophosphate.It plays an important role in the pathogenesis and development of bone and joint diseases,such as ankylosing spondylitis,craniometaphyseal dysplasia,and articular cartilage calcification.This review summarizes the progress of research on ANKH and the above-mentioned diseases.

Enfoque radiológico de la enfermedad de Forestier-Rotes-Querol. Presentación de caso / Radiological approach to Forestier Rotes Querol disease. A case report

Se presenta el caso de una paciente femenina de 64 años de edad, con comorbilidades asociadas, quien acude al Instituto Hondureño de Seguridad Social por dolor y rigidez cervical de evolución crónica. En estudios de resonancia magnética se observa protrusión ósea con formación de puentes anteriores a los cuerpos vertebrales, que concuerda con las alteraciones halladas en tomografía y radiografía cervical. La hiperostosis esquelética difusa idiopática, o enfermedad de Forestier-Rotes-Querol, es una patología de la columna poco reconocida que puede cursar asintomática y desapercibida, pero ante dolor cervical la tomografía de columna es el método de estudio inicial e ideal para la valoración ósea en caso de patologías osificantes y degenerativas, que al complementar con resonancia magnética ayuda a realizar un diagnóstico preciso, para decidir entre un tratamiento quirúrgico o un manejo conservador.

The case of a 64-year-old female with associated comorbidities is presented, who attends the Honduran social security institute due to chronic cervical pain and stiffness. Magnetic resonance studies show bone protrusion with formation of anterior bridges of vertebral bodies that is consistent with the alterations visualized in tomography and cervical radiography. Idiopathic diffuse skeletal hyperostosis is a poorly recognized pathology of the spine that can be asymptomatic and unnoticed. In case of cervical pain, spinal tomography is the initial and ideal study method for bone assessment in case of ossifying and degenerative pathologies that, when complemented with magnetic resonance imaging, helps to make an accurate diagnosis and thus evaluate and decide between surgical treatment or conservative management

Does the reduction of inferior turbinate affect lower airway functions? / A redução da concha nasal inferior afeta as funções das vias aéreas inferiores?

Abstract Introduction: Although the nose and lungs are separate organs, numerous studies have reported that the entire respiratory system can be considered as a single anatomical and functional unit. The upper and lower airways affect each other either directly or through reflex mechanisms. Objective: In this study, we aimed to evaluate the effects of the radiofrequency ablation of persistent inferior turbinate hypertrophy on nasal and pulmonary function. Methods: Twenty-seven patients with bilateral persistent inferior turbinate hypertrophy without septal deviation were included in this study. All of the patients were evaluated using anterior rhinoscopy, nasal endoscopy, acoustic rhinometry, a visual analogue scale, and flow-sensitive spirometry on the day before and 4 months after the radiofrequency ablation procedure. Results: The post-ablation measurements revealed that the inferior turbinate ablation caused an increase in the mean cross-sectional area and volume of the nose, as well as in the forced expiratory volume in 1 s, forced vital capacity, and peak expiratory flow of the patients. These differences between the pre- and post-ablation results were statistically significant. The post-ablation visual analogue scale scores were lower when compared with the pre-ablation scores, and this difference was also statistically significant. Conclusion: This study demonstrated that the widening of the nasal passage after the reduction of the inferior turbinate size had a favorable effect on the pulmonary function tests.

Resumo Introdução: Embora o nariz e os pulmões sejam órgãos separados, numerosos estudos relataram que todo o sistema respiratório pode ser considerado como uma única unidade anatômica e funcional. As vias aéreas superiores e inferiores afetam uma à outra diretamente ou através de mecanismos reflexos. Objetivo: Avaliar os efeitos da ablação por radiofrequência em conchas nasais inferiores com hipertrofia persistente sobre a função nasal e pulmonar. Método: Foram incluídos neste estudo 27 pacientes com hipertrofia persistente bilateral de conchas inferiores sem desvio septal. Todos os pacientes foram avaliados com rinoscopia anterior, endoscopia nasal, rinometria acústica, escala visual analógica e espirometria sensível ao fluxo no dia anterior e quatro meses após o procedimento de ablação por radiofrequência. Resultados: As medidas pós-ablação demonstraram que a ablação das conchas nasais inferiores resultou em um aumento da área transversal média e do volume do nariz, bem como do volume expiratório forçado em um segundo, da capacidade vital forçada e do fluxo expiratório máximo dos pacientes. Essas diferenças entre os resultados pré e pós-ablação foram estatisticamente significantes. Os escores da escala visual analógica pós-ablação foram menores quando comparados com os escores pré-ablação e essa diferença também foi estatisticamente significante. Conclusão: O alargamento da passagem nasal após a redução do tamanho das conchas nasais inferiores teve efeito favorável nos testes de função pulmonar.

Síndrome de Klippel-Trenaunay en un recién nacido / Klippel-Trenaunay Syndrome in a newborn

Se describe el caso clínico de un recién nacido, asistido en el Hospital General Docente Dr Juan Bruno Zayas Alfonso de Santiago de Cuba, sin antecedentes patológicos maternos ni familiares, quien presentaba macrocefalia, asimetría de los miembros inferiores, marcada hipertrofia en el miembro inferior izquierdo y macrodactilia, así como angiomas cutáneos en el área inferior del tronco, en el abdomen, la región glútea y las piernas. Luego de una evaluación detallada por parte de un equipo multidisciplinario, que se basó en el cuadro clínico y el estudio radiográfico, se diagnosticó el síndrome de Klippel-Trenaunay. El paciente ha mantenido una evolución favorable hasta el momento actual y se continúa un estricto seguimiento médico.

The case report of a newborn, assisted in Dr Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba is described, without maternal or family pathological history who presented macrocephalic asymmetry of the lower members, marked hypertrophy in the left low member and macrodactilia, as well as cutaneous angiomas in the lower area of the trunk, abdomen, buttocks region and legs. After a detailed evaluation by a multidisciplinary team which was based on the clinical pattern and radiographical study, the Klippel-Trenaunay syndrome was diagnosed. The patient has maintained a favorable clinical course up to now and a strict medical follow-up is carried out.

A Case of SAPHO Syndrome Associated with Lytic Bone Lesions Resembling Metastases / 대한피부과학회지

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a variety of inflammatory bone disorders associated with dermatologic pathology. A 57-year-old female presented with pustulosis on both hands that had persisted for several months. She also had lower back pain without trauma history. On physical examination, tenderness on her lower back and left anterior chest wall pain were found, and claudication was observed. Radiological studies including computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET)-CT showed endplate lytic changes in her spine, a focal hypermetabolic lesion in a left rib, and costochondral junction. These findings raised doubt on the presence of metastatic bone lesions, and there was no indication for primary cancer after a complete medical checkup. Palmoplantar pustulosis was well controlled by treatment with acitretin. The osteitis associated with SAPHO syndrome usually presents as osteosclerosis, while reports on osteolytic lesions are rare. We report herein a rare case of SAPHO syndrome associated with bone lesions resembling bone metastasis.

A Rare Case of Lethal Prenatal-Onset Infantile Cortical Hyperostosis

Infantile cortical hyperostosis, or Caffey's disease, usually presents with typical radiological features of soft tissue swelling and cortical thickening of the underlying bone. The disease can be fatal when it presents antenatally, especially before a gestational age of 35 weeks. This fatal, premature form of the disease is known to occur in various ethnic groups around the globe, and approximately 30 cases have been reported in English literature. This paper is unique in that it is the first paper to report a lethal form of prenatal-type infantile cortical hyperostosis diagnosed in South Korea. Born at gestational age of 27 weeks and 4 days, the patient had typical features of polyhydramnios, anasarca, hyperostosis of multiple bones, micrognathia, pulmonary hypoplasia, and hepatomegaly. The patient was hypotonic, and due to pulmonary hypoplasia and persistent pulmonary hypertension, had to be supported with high frequency ventilation throughout the entire hospital course. Due to the disease entity itself, as well as prolonged parenteral nutrition, liver failure progressed, and the patient expired on day 38 when uncontrolled septic shock was superimposed. The chromosome karyotype of the patient was normal, 46, XX, and COL1A1 gene mutation was not detected.

Síndrome de Goldbloom: síndrome febril prolongado con disproteinemia. Caso clínico / Goldboom's syndrome: prolonged febrile syndrome with dysproteinemia. Case report

El síndrome de Goldbloom es una rara entidad clínica de etiología desconocida que ocurre casi exclusivamente en pediatría. Consiste en un síndrome febril prolongado con hiperostosis perióstica y disproteinemia, que, con frecuencia, simula una patología hematooncológica o linfoproliferativa. El diagnóstico se hace por exclusión de las diferentes causas de dolor de los huesos y se asocia a hipergammaglobulinemia, hipoalbuminemia, eritrosedimentación acelerada e imágenes radiológicas de periostitis. La sintomatología, la radiología y los parámetros de laboratorio remiten en un tiempo variable, que va, habitualmente, de los 3 a los 12 meses. Se presenta a un paciente de 6 años con dolores óseos difusos, hiperostosis perióstica, síndrome febril prolongado de 8 meses de evolución, pérdida de peso y reactantes de fase aguda elevados con disproteinemia (hipergammaglobulinemia e hipoalbuminemia). Debe considerarse el síndrome de Goldbloom en un paciente con las manifestaciones descritas luego de la exclusión de la patología infecciosa, hematooncológica e inflamatoria de otra causa.

Goldbloom syndrome is a rare clinical entity, of unknown etiology that happens almost exclusively in pediatric population. It is a prolonged febrile syndrome with periosteal hyperostosis and dysproteinemia, and often simulates an hematooncology or lymphoproliferative disease. The diagnosis is to rule out the different causes of bone pain associated with hypergammaglobulinemia, hypoalbuminemia, high erythrocyte sedimentation rate and periostitis at the radiographies. Symptomatology, radiology and laboratory parameters refer in a variable time, usually from 3 to 12 months. We report the case of a six-year-old boy with diffuse bone pain, prolonged febrile syndrome (of 8 months of evolution), weight loss and elevated acute phase reactants with dysproteinemia (hypergammaglobulinemia and hypoalbuminemia). Goldbloom syndrome should be considered in patients with prolonged febrile syndrome and cortical hyperostosis after the exclusion of infectious, lymphoproliferative or inflammatory disease.

Risk Factors for Cement Loosening after Vertebroplasty for Osteoporotic Vertebral Fracture with Intravertebral Cleft: A Retrospective Analysis

STUDY DESIGN: Retrospective case-control study. PURPOSE: To evaluate the primary outcomes and radiographic results of percutaneous vertebroplasty (PVP) in patients with singlelevel osteoporotic vertebral fracture (OVF) with intravertebral cleft (IVC) to identify the risk factors for cement loosening after PVP. OVERVIEW OF LITERATURE: PVP is a widely accepted method for managing painful OVF; however, cement loosening occasionally occurs with poor outcomes. METHODS: This retrospective study involved 195 patients treated with PVP for single-level OVF with IVC. Six months thereafter, the primary outcomes were evaluated using the Visual Analog Scale (VAS) for back pain and the modified Oswestry Disability Index. Computed tomography was conducted to detect cement loosening. Possible risk factors, such as age, sex, wedging angle, intravertebral instability, Parkinson’s disease, spinous process fracture, ankylosing spinal hyperostosis, split vertebrae, and adjacent intervertebral vacuum, were assessed. RESULTS: Forty-nine patients (25%) experienced cement loosening 6 months after PVP. The mean VAS scores were significantly higher in patients with cement loosening than in those without (50 vs. 26 mm, respectively; p < 0.01). Cement loosening was closely associated with intravertebral instability (odds ratio [OR], 1.20; 95% confidence interval [CI], 1.04–1.40; p =0.015), Parkinson’s disease (OR, 54.31; 95% CI, 4.47–659.53; p =0.002), spinous process fracture (OR, 7.11; 95% CI, 1.65–30.60; p =0.009), and split vertebrae (OR, 11.59; 95% CI, 1.64–82.02; p =0.014). CONCLUSIONS: Patients with cement loosening experienced worse back pain than those without cement loosening. The important risk factors that influenced cement loosening after PVP were high intravertebral instability, Parkinson’s disease, spinous process fracture, and split vertebrae.

Parosteal Lipoma Associated with Underlying Recurrent Bizarre Parosteal Osteochondromatous Proliferation (Nora's Lesion) of the Hand / 대한정형외과학회잡지

Parosteal lipoma is a benign tumor of the mature adipose tissue that contacts the periosteum of the underlying bone directly. The tumor commonly arises in the long bones, such as the femur, radius or tibia, and often exhibits underlying osseous changes, such as a cortical hyperostosis or erosion. Parosteal lipoma arising in a finger is rare. Furthermore, there are no reports of parosteal lipoma associated with underlying bizarre parosteal osteochondromatous proliferation. The authors present a rare case of parosteal lipoma of the proximal phalanx of the little finger accompanied by recurrent bizarre paroteal osteochondromatous proliferation in a 64-year-old male patient who had previously undergone an excisional biopsy at the same location 8 years earlier.

Prevalence of Diffuse Idiopathic Skeletal Hyperostosis Diagnosed by Whole Spine Computed Tomography: A Preliminary Study

BACKGROUND: Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by ossification of the enthesis. The diagnosis has been mainly based on the chest or whole spine lateral plain film. Recently, chest or thoracolumbar computed tomography (CT) has been reported to be more reliable for the diagnosis of DISH. The purposes of this study were to investigate the prevalence and location of DISH and evaluate the prevalence of comorbidities, such as ossification of the posterior longitudinal ligament (OPLL) and ossification of the ligamentum flavum (OLF), using whole spine CT. METHODS: Whole spine CT scans of patients over 16 years of age who were examined at Cheju Halla General Hospital between February 2011 and December 2016 were reviewed for this study. The diagnosis of DISH was made according to the modified Resnick criteria. The prevalence of DISH in each age decade and its location were evaluated. Also, the prevalence of OPLL and OLF in DISH patients was investigated. RESULTS: The overall incidence of DISH was 24.4% (40 of 164 cases). There was no case of DISH in patients in their 40s and younger. The percentile incidences of DISH in patients in their fifth, sixth, seventh, eighth, and ninth decades were 20.0% (4 of 20 cases), 32.3% (10 of 31 cases), 40.0% (10 of 25 cases), 34.5% (10 of 29 cases), and 27.3% (6 of 22 cases), respectively. A strong positive correlation between the age decade and the incidence of DISH was noted (r = 0.853, p = 0.007). DISH patients had higher incidences of OLF (22.5%) and OPLL (37.5%). The most common location of DISH was the middle thoracic spine (90.0%) followed by the lower thoracic spine (87.5%). There was one case of DISH involving only the cervical spine. CONCLUSIONS: The incidence of DISH diagnosed by CT was higher than we expected. Whole spine CT can be a valuable modality to evaluate the location of DISH in the cervical and lumbar spine and the comorbidity rates of OLF and OPLL.

SAPHO. Puesta al día / SAPHO: Update

SAPHO es un acrónimo en el que se incluye sinovitis, acné, pustulosis, hiperostosis y osteítis, corresponde a una entidad clínica que abarca varias manifestaciones osteoarticulares y dermatológicas que pueden aparecer en el mismo paciente de forma simultánea o sucesiva a lo largo de su vida; tiene características radiológicas importantes al momento de establecer el diagnóstico y su origen es multifactorial interviniendo factores genéticos, sobre todo en los genes LPNI2 y NOD2 ubicados en el cromosoma 18, infecciosos e inmunológicos. Dentro de los aspectos clínicos relevantes destaca la afectación de la pared anterior del tórax que puede ser tan notable como para llegar a producir compresión de las venas subclavia y cava superiores, lo cual puede conducir a trombosis vascular debido a la hiperostosis y a la compresión extrínseca por una masa de partes blandas en el mediastino. Desde su identificación, la primera línea de tratamiento ha sido el uso de AINE; sin embargo, muchos medicamentos inmunosupresores se han desarrollado para su tratamiento, el uso de anti-TNF ha mostrado eficacia para reducir las manifestaciones articulares, óseas y de piel y, por tanto, se ha convertido en una excelente opción para el tratamiento

Craniometaphyseal dysplasia: Report of 2 cases with an emphasis on panoramic imaging features

Craniometaphyseal dysplasia (CMD) is a rare hereditary disorder characterized by hyperostosis of the craniofacial bones and flared metaphyses of the long bones. Although some reports have described the dentomaxillofacial characteristics of CMD, including increased density of the jaw, malocclusion, and delayed eruption of the permanent teeth, only a few studies have reported the distinct imaging features of CMD on panoramic radiography. This report presents 2 cases of confirmed CMD patients with an emphasis on panoramic imaging features. The patients' images revealed hyperostosis and sclerosis of the maxilla and mandibular alveolar bone, but there was no change in the mandibular basal bone. In both cases, the mandibular condyle heads exhibited a short clubbed shape with hyperplasia of the coronoid process. For patients without clear otorhinolaryngological symptoms, common radiologic features of CMD could be visualized by routinely-taken panoramic radiographs, and further medical examinations and treatment can be recommended.

Recurrent Aspiration Pneumonia due to Anterior Cervical Osteophyte / 대한구급학회지

A 74-year-old man presented with recurrent vomiting and aspiration pneumonia in the left lower lobe. He entered the intensive care unit to manage the pneumonia and septic shock. Although a percutaneous endoscopic gastrostomy tube was implanted for recurrent vomiting, vomiting and aspiration recurred frequently during admission. Subsequently, he complained of neck pain when in an upright position. A videofluoroscopic swallowing study showed compression of the esophagus by cervical osteophytes and tracheal aspiration caused by an abnormality at the laryngeal inlet. Cervical spine X-rays and computed tomography showed anterior cervical osteophytes at the C3-6 levels. Surgical decompression was scheduled, but was cancelled due to his frailty. Unfortunately, further recurrent vomiting and aspiration resulted in respiratory arrest leading to hypoxic brain damage and death. Physicians should consider cervical spine disease, such as diffuse skeletal hyperostosis as an uncommon cause of recurrent aspiration pneumonia.

Recurrent Aspiration Pneumonia due to Anterior Cervical Osteophyte / 대한중환자의학회지

A 74-year-old man presented with recurrent vomiting and aspiration pneumonia in the left lower lobe. He entered the intensive care unit to manage the pneumonia and septic shock. Although a percutaneous endoscopic gastrostomy tube was implanted for recurrent vomiting, vomiting and aspiration recurred frequently during admission. Subsequently, he complained of neck pain when in an upright position. A videofluoroscopic swallowing study showed compression of the esophagus by cervical osteophytes and tracheal aspiration caused by an abnormality at the laryngeal inlet. Cervical spine X-rays and computed tomography showed anterior cervical osteophytes at the C3-6 levels. Surgical decompression was scheduled, but was cancelled due to his frailty. Unfortunately, further recurrent vomiting and aspiration resulted in respiratory arrest leading to hypoxic brain damage and death. Physicians should consider cervical spine disease, such as diffuse skeletal hyperostosis as an uncommon cause of recurrent aspiration pneumonia.

Infliximab for Treatment of Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis Syndrome: A Case Report

No abstract available.

Enfermedad de Camurati-Engelmann: reporte de un caso y revisión de la literatura / Camurati-Engelmann disease: case report and review of literature

La enfermedad de Camurati-Engelmann es una entidad poco común debida a mutaciones en el gen que codifica el TGF-(3. Se caracteriza por hiperostosis de huesos largos y cráneo, acompañada de dolor óseo intenso, ocasionalmente debilidad muscular, marcha de pato. El tratamiento se basa en el uso de glucocorticoides en dosis altas y en casos severos la descompresión quirúrgica está indicada. Desde nuestro conocimiento este es el primer caso reportado en Colombia

Camurati-Engelmann disease is a rare entity due to mutations in the gene encoding the TGF-(3. It is characterised by hyperostosis of long bones and skull, accompanied by severe bone pain, and occasionally muscular weakness and a waddling gait. The treatment is based on the use of high doses of glucocorticoids, and in severe cases surgical decompression is indicated. As far as we know, this is the first case reported in Colombia