Histiocitosis de células de Langerhans con implicación oral. A propósito de un caso / Langerhans cell histiocytosis with oral implication. A case report

La proliferación desordenada de histiocitos muy similares a células de Langerhans da lugar a un tipo de patología que se conoce como histiocitosis de células de Langerhans. La histiocitosis de células de Langerhans es una enfermedad rara, poco frecuente, de etiología no muy clara y que se caracteriza por manifestaciones de variable presentación: desde una afectación de varios órganos o sistemas y con una mortalidad muy elevada, hasta una lesión única, bien sea con compromiso óseo o con patología pulmonar, de favorable evolución, incluso hasta la regresión espontánea. Es frecuente que exista sólo patología oral o que ésta acompañe al resto de la sintomatología general. A nivel bucal, puede presentar lesiones óseas líticas en un único o en varios puntos; se pueden ver, así mismo, fracturas patológicas de la mandíbula, dolor, úlceras bucales, compromiso periodontal con bolsas periodontales marcadas, acusada movilidad de piezas dentales con pérdida prematura de dientes, erupción precoz de dientes, etc (AU)

Langerhans cell histiocytosis (LCH) is a rare disorder of unknown etiology, characterized by disorganized proliferation of histiocytes similar to Langerhans cells. Variable clinical manifestations are observed in this entity: from acute multisistemic disease, associated with high mortality, to bone or lung lesions of favorable prognosis. Oral involvement may be present with or without other clinical signs. Osteolytic bone lesions, isolated or multiple areas of the maxilla, pathological mandibular fractures, pain, eritematous ulcerations, periodontal disease that may lead to periodontal deep pockets and alveolar bone loss, dental mobility, premature tooth eruption and premature dental loss may exist as oral manifestations of the disease (AU)

Asimetrías faciales y maloclusiones en pacientes con tortícolis muscular congénita. Una revisión sistemática / Facial asymmetries and dental malocclusions in patients with congenital

Las deformidades dentofaciales generan déficits funcionales y estéticos. Cuando la tortícolis muscular congénita (TMC) no se detecta precozmente y, por tanto, no se trata, pueden establecerse alteraciones craneofaciales (asimetrías, desviación lateral mandibular, deformación plagiocefálica del frontal, retracción del cigoma, distopia orbitaria, disminución de la dimensión vertical del lado afectado, desplazamiento posterior del oído del lado afectado, inclinación del plano comisural) y oclusodentales (inclinación del plano oclusal, mordida cruzada unilateral en el lado afecto, desviación de la línea media dentaria hacia el lado afectado). Es preciso, por tanto, un diagnóstico precoz, durante las primeras semanas de vida, para que esta anomalía pueda corregirse instaurando un tratamiento conservador mediante fisioterapia. Los casos que no responden a este tipo de tratamiento o que han tardado en diagnosticarse muy probablemente precisaran tratamiento quirúrgico. El propósito de este artículo es hacer una revisión sistemática de la tortícolis muscular congénita, centrándose fundamentalmente en la importancia del diagnóstico temprano y en las anomalías craneofaciales fundamentalmente, y oclusales que se producen si no se corrige

Dentofacial deformities generate functional and aesthetic deficits. When congenital muscular torticollisis not detected, craniofacial alterations (asymmetries, mandible lateral deviation, frontal deformational plagiocephaly, recession of the ipsilateral zygoma, orbital dystopia, reduction of vertical facial height on the affected side, posterior displacement of the ipsilateral ear, commissural canting) and dental malocclusions (occlusal plane canting, unilateral cross bite in the affected side, deviation of the lower center line to the affected side) can be established. Early diagnosis is necessary during the first weeks of life, to correct this anomaly by a conservative treatment with physical therapy. Cases that do not respond to this treatment or who have been diagnosed later will probably require surgical treatment. The aim of this article is to make a systematic review of the congenital muscular torticollis, focusing on the importance of early diagnosis and in the craniofacial and oclusal anomalies that occur if the congenital muscular torticollis is untreated

Quantifying esophagogastric junction contractility with a novel HRM topographic metric, the EGJ-Contractile Integral: normative values and preliminary evaluation in PPI non-responders.

BACKGROUND: Despite its obvious pathophysiological relevance, the clinical utility of measures of esophagogastric junction (EGJ) contractility is unsubstantiated. High-resolution manometry (HRM) may improve upon this with its inherent ability to integrate the magnitude of contractility over time and length of the EGJ. This study aimed to develop a novel HRM metric summarizing EGJ contractility and test its ability distinguish among subgroups of proton pump inhibitor non-responders (PPI-NRs). METHODS: 75 normal controls and 88 PPI-NRs were studied. All underwent HRM. PPI-NRs underwent pH-impedance monitoring on PPI therapy scored in terms of acid exposure, number of reflux events, and reflux-symptom correlation and grouped as meeting all criteria, some criteria, or no criteria of abnormality. Control HRM studies were used to establish normal values for candidate EGJ contractility metrics, which were then compared in their ability to differentiate among PPI-NR subgroups. KEY RESULTS: The EGJ contractile integral (EGJ-CI), a metric integrating contractility across the EGJ for three respiratory cycles, best distinguished the All Criteria PPI-NR subgroup from controls and other PPI-NR subgroups. Normal values (median, [IQR]) for this measure were 39 mmHg-cm [25-55 mmHg-cm]. The correlation between the EGJ-CI and a previously proposed metric, the lower esophageal sphincter-pressure integral, that used a fixed 10 s time frame and an atmospheric as opposed to gastric pressure reference was weak. CONCLUSIONS & INFERENCES: Among HRM metrics tested, the EGJ-CI was best in distinguishing PPI-NRs meeting all criteria of abnormality on pH-impedance testing. Future prospective studies are required to explore its utility in management of broader groups of gastroesophageal reflux disease patients.

Epidermólisis ampollosa de la unión: implicación oral. A propósito de un caso / Junctional epidermolysis bullosa: oral implication. A case report

Se entiende por epidermólisis ampollosa un grupo de dermatosis no inflamatorias, mecano ampollosas, enfermedades clínicamente similares con afectación cutáneo mucosa, que tienen carácter hereditario, en general, y que se caracterizan por una excesiva fragilidad de la piel y mucosas ante pequeños o mínimos traumas mecánicos, dando lugar a la aparición de vesículas, ampollas, erosiones y escaras. Existe una gran variabilidad fenotípica y considerable morbimortalidad (AU)

Epidermolysis bullosa is the name given to a group of rare genetically determined diseases of de skin and mucosal membranes, characterized by the development of blisters and vesicles in response to minor trauma. Is characterized by extensive phenotypic variability with considerable morbidity and mortality. Epidermolysis bullosa is classified into distinct subtypes depending on the location of blistering within the cutaneous dermoepidermal basement membrane zone (AU)