Hemifacial microsomia: treatment alternatives-a systematic review of literature.

OBJECTIVE: To systematically review literature on therapeutic options for treating hemifacial microsomia (HFM), in young patients with growth potential, classifying and comparing the different dentofacial treatment methods. STUDY DESIGN: An independent review of databases (Scopus, Embase, Ovid, Cochrane Library and PubMed) following the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA), conducted by four evaluators. The protocol of this study was registered in International prospective register of systematic reviews (PROSPERO), under the number CRD42021293076. RESULTS: Between 1970-2021, a total number of 1137 articles were published of which 27 were included in this study according to the selection criteria: one randomized multicentric trial, two case-control studies, three case series and 21 case reports. CONCLUSIONS: The most common orthopedic treatments provide vertical stimulation of the maxillary process in the affected side. Orthodontic approaches are mainly applied for vertical correction and stabilization of the occlusal plane. Other treatment options include orthognathic surgery, osteogenic distraction, temporomandibular reconstruction and grafting. It is recommended that prospective clinical randomized controlled studies be conducted using homogeneous pediatric groups with long-term follow-up, to establish recommended evidence-based methods for treating each set of hemifacial microsomia symptoms.

Structural and immunological characterization of sulphatides: relevance of sulphate moieties in Trypanosoma cruzi glycoconjugates.

Sulphoglycosphingolipids, present on the surface of diverse cells, participate in the regulation of various cellular events. However, little is known about the structure and the role of sulphoglycosphingolipids in trypanosomatids. Herein, sulphated dihexosylceramide structures - composed mainly of sphingosine as the long chain base acylated with stearic acid - have been determined for the first time in Trypanosoma cruzi epimastigotes by UV-MALDI-TOF-MS analysis. Interestingly, inhibition ELISA assays using cruzipain as antigen and polyclonal rabbit antibodies specific for cruzipain, the major cysteine proteinase of T. cruzi, or for its C-terminal domain, have demonstrated (i) that sulphate epitopes are shared between cruzipain and sulphatides of T. cruzi, (ii) that cross-reactivity maps to the C-terminal domain and (iii) the existence of other antigenic determinants in the glycolipidic structures. These features provide evidence that sulphate groups are antigenic in sulphate-containing parasite glycoconjugates. Furthermore, IgG2 antibody levels inversely correlate with disease severity in chronic Chagas disease patients, suggesting that IgG2 antibodies specific for sulphated epitopes might be associated with protective immunity and might be considered as potential surrogates of the course of chronic Chagas disease.

A striking common O-linked N-acetylglucosaminyl moiety between cruzipain and myosin.

Single units of O-linked N-acetylglucosamine (GlcNAc), usually components of nuclear and cytoplasmatic proteins, are present at the C-terminal domain of cruzipain (Cz), a lysosomal major antigen from Trypanosoma cruzi. On the other hand, antibodies directed against some self-antigens like myosin are associated with Chagas heart disease. The participation of O-GlcNAc moieties in the molecular antigenicity of Cz was determined using GlcNAc linked to aprotinin by ELISA. The immune cross-reactivity between Cz and myosin is mainly focused in the C-T domain. ELISA inhibition assays using rabbit sera specific for Cz and C-T in conjunction with immune-gold electron microscopy analysis of heart tissues from mice immunized with C-T confronted with polyclonal rabbit sera specific for Cz and C-T prior and after myosin adsorption provided evidence which indicates that O-GlcNAc moieties constitute a common epitope between Cz and either myosin or other cardiac O-GlcNAc-containing proteins, showing a new insight into the molecular immune pathogenesis of Chagas heart disease.

MALDI-TOF MS analysis of labile Lolium perenne major allergens in mixes.

BACKGROUND: It is well known that allergen extracts used for specific therapy of allergic disorders are commonly stored as mixtures, causing an alteration of its stability. OBJECTIVE: The aim of this report is to identify pollen allergens susceptible to degradation during storage of mixtures containing different sources of proteases in the absence of glycerol as a preserving agent. METHODS: Mixes containing Lolium perenne (Lol p) pollen extract with either Aspergillus fumigatus or Periplaneta americana extracts were prepared and co-incubated for 90 days at 4 degrees C. Samples were taken off at fixed times and comparatively tested by in vitro and in vivo assays with atopic patients. Selected pollinic allergens were subjected to MALDI-TOF MS analysis. RESULTS: ELISA inhibition evidenced the loss of potency from ryegrass extract, and immunoblotting assays showed the degradation of specific pollinic allergens during storage of mixtures containing protease-rich sources. An in vivo intradermal skin assay confirmed the gradual loss of the biological activity of L. perenne pollen extract co-incubated with non-related protease-rich extracts in comparison with that of the control pollen extract. MALDI-TOF MS analysis allowed us to determine that Lol p 1 and Lol p 5 are susceptible to proteolysis whereas Lol p 4 was found to be resistant to degradation during storage. CONCLUSIONS: Lol p 1 and Lol p 5 degradation is responsible for the loss of the biological activity of L. perenne pollen extract when co-incubated with protease-rich fungal and cockroach extracts in the same vial for months in the absence of glycerol as a preserving agent. The integrity of these major allergens must be preserved to increase the vaccine stability and to assure efficacy when mixes are used for immunotherapy.

[Anesthetic management during removal of a giant intrathoracic mass]. / Manejo anestésico en la exéresis de un tumor intratorácico gigante.

We report the case of a young woman with a giant intrathoracic angiomyolipoma accounting for 10% of her weight and occupying 75% of the right hemithorax and 30% of the left. Before anesthetic induction, an arterial line and a central venous catheter were applied for monitoring; neck and thoracic punctures were avoided. The trachea was intubated with a double lumen tube after provision of sedation and analgesia with remifentanil-midazolam and topical anesthesia of the larynx. A rigid bronchoscope and extracorporeal circulation were available at all times and muscle relaxants were avoided. Ventilation was maintained with pressure support until the mass effect was resolved. The patient was transferred to the intensive care unit, extubated after 24 hours, and discharged 5 days after surgery. We describe the recommendations for perioperative management in cases involving this type of tumor and the complications that can develop. Recent symptoms, diagnostic images, and the results of lung function tests provide information for guiding the anesthetic approach. The obstructive ventilatory compromise caused by a giant mass depends more on location than size. Extracorporeal circulation or rigid bronchoscopy might be needed at any time during surgery.

Manejo anestésico en la exéresis de un tumor intratorácico gigante / Anesthetic management during removal of a giant intrathoracic mass

Se describe el caso de una mujer joven con un angiomiolipomagigante intratorácico correspondiente al 10%de su peso, que ocupaba 75% del hemitórax derecho y30% del izquierdo. Previo a la inducción anestésica semonitorizó a la paciente de forma invasiva, evitando losaccesos centrales en cuello o tórax. La intubación se realizócon tubo de doble luz izquierdo, previa sedaciónanalgesiacon remifentanilo-midazolam y anestesia tópicasobre las estructuras laríngeas. Se mantuvodisponibilidad permanente de broncoscopio rígido y circulaciónextracorpórea y se evitó el uso de relajantesmusculares manteniendo la ventilación con soporte depresión hasta que el efecto de masa fue resuelto. Lapaciente fue trasladada a UCI, extubada a las 24 horas ydada de alta a los 5 días del postoperatorio. Se describenlas recomendaciones perioperatorias en este tipo detumores y los posibles tipos de complicaciones.Al enfrentarnos a este tipo de pacientes, la sintomatologíareciente y la evaluación mediante imágenes y pruebasfuncionales nos guiarán en el enfoque anestésico. Elcompromiso obstructivo generado por estas masasgigantes depende principalmente de su localización másque de su tamaño. Cualquier momento del intraoperatoriopuede requerir la necesidad de circulación extracorpóreao broncoscopia rígida

We report the case of a young woman with a giantintrathoracic angiomyolipoma accounting for 10% of herweight and occupying 75% of the right hemithorax and30% of the left. Before anesthetic induction, an arterialline and a central venous catheter were applied for monitoring;neck and thoracic punctures were avoided. Thetrachea was intubated with a double lumen tube afterprovision of sedation and analgesia with remifentanilmidazolamand topical anesthesia of the larynx. A rigidbronchoscope and extracorporeal circulation were availableat all times and muscle relaxants were avoided. Ventilationwas maintained with pressure support until themass effect was resolved. The patient was transferred tothe intensive care unit, extubated after 24 hours, and discharged5 days after surgery. We describe the recommendationsfor perioperative management in cases involvingthis type of tumor and the complications that can develop.Recent symptoms, diagnostic images, and the resultsof lung function tests provide information for guiding theanesthetic approach. The obstructive ventilatory compromisecaused by a giant mass depends more on locationthan size. Extracorporeal circulation or rigid bronchoscopymight be needed at any time during surgery