RESUMO
Alagille syndrome has been described as a multisystemic clinical spectrum caused by an autosomal dominant genetic disorder. Although it is estimated that there is 1 case per 100 000 live births, the prognosis for survival and quality of life for these patients is varied but tends to be negative. In Colombia, this condition is considered an orphan disease with difficult management due to the lack of specialized centers that have all the medical specialties and subspecialties. Some reports state that no more than 30 cases have been published in this country. Materials and methods: The authors report a case of a male baby who, at 8 days old, he was taken to the general practitioner's outpatient clinic for persistent jaundice. At 3 months of age, he was reviewed by the pediatric gastroenterology department, which requested liver and biliary tract scintigraphy, showing atresia of the biliary tract, hepatomegaly, and the absence of a gallbladder. Results: Liver transplantation is the definitive solution. However, in low- and middle-income countries, where there are no well-established organ transplantation programs, the prognosis for these patients is presumed to be worse. Conclusion: Alagille syndrome is a rare disease that requires an accurate and early diagnosis and timely multidisciplinary management to reduce the impact of multisystemic complications. It is necessary to advance in transplant programs in low- and middle-income countries, to provide a solution to cases where there are no other therapeutic alternatives, and to contribute to the quality of life of the affected patient.
RESUMO
BACKGROUND: The study aimed to establish the benefits of using chest tubes with negative pleural suction against trapped water in patients with penetrating or blunt chest trauma who underwent tube thoracostomy, in terms of the incidence of complications, such as persistent air leak, clotted hemothorax, empyema, and duration of stay. METHODS: Patients who underwent tube thoracostomy because of traumatic pneumothorax, hemothorax, or hemopneumothorax were randomly assigned into one of two groups: in Group 1, the three-bottle drainage system was connected to a negative suction; in Group 2, no suction was given. Patients who required mechanical ventilation or emergency surgery (thoracotomy or thoracoscopy) either at the time of admission to the institution or immediately after the tube thoracostomy, patients who had histories of thoracic procedures or chronic pulmonary diseases (chronic obstructive pulmonary disease, diffuse interstitial lung disease), and patients with multiple injuries with severe traumatic brain injury and a Glasgow Coma Scale (GCS) score less than 8 of 15 were excluded from the study. Hospital stay, duration of tube thoracostomy, prolonged fistula, and other clinical variables were compared. RESULTS: One hundred ten patients were included, 56 in the group with suction and 54 in the group without suction. There were no differences in the demographic characteristics of each group. There were no differences between the groups in terms of hospital stay (p = 0.22), duration of tube thoracostomy (p = 0.35) (3 days in each group), or complications. However, the probability of air leak presence in time was greater for the Group 1 patients with negative suction versus the Group 2 patients (p = 0.023). CONCLUSION: The use of negative pleural suction did not demonstrate advantages over the three-bottle chest drainage system without suction in patients with uncomplicated traumatic pneumothorax, hemothorax, or hemopneumothorax. LEVEL OF EVIDENCE: Therapeutic study, level II.
Assuntos
Tubos Torácicos , Sucção/métodos , Traumatismos Torácicos/terapia , Adulto , Tubos Torácicos/efeitos adversos , Feminino , Hemopneumotórax/terapia , Hemotórax/terapia , Humanos , Tempo de Internação , Masculino , Pneumotórax/terapia , Sucção/efeitos adversos , Toracoscopia , Toracotomia , Resultado do Tratamento , Ferimentos não Penetrantes/terapia , Ferimentos Penetrantes/terapiaRESUMO
Macrophages and dendritic cells are involved in the immune response to Mycobacterium tuberculosis (Mtb). Such a response, although extensively studied using animal models and cells from human blood, has not been characterized in cells from pulmonary hilar lymph nodes (PHLN). We characterized populations of myeloid APC from PHLN and determined their expression of CCR2, CCR5, CCR7, CD40, CD54, CD80, and CD86 as well as the cytokine/chemokine microenvironment before and after purified protein derivative (PPD) and mannosilated lipoarabinomannan (ManLAM) stimulation. Results show that there are at least three APC populations in PHLN, defined as CD14highHLA-DRlow/-, CD14dimHLA-DRdim, and CD14-HLA-DRhigh/dendritic cells (DC), with the largest number represented by CD14dimHLA-DRdim cells (where dim indicates intermediate levels). CD14-HLA-DRhigh/DC expressed higher levels of costimulatory molecules and lower levels of CCR2 and CCR5, but all cell populations showed similar CCR7 levels. PPD and ManLAM specifically down-regulated CCR2 expression but not that of CCR5 and CCR7, and such down-regulation was observed on all APC populations. Mtb Ag did not affect the expression of costimulatory molecules. PPD but not ManLAM specifically induced MCP-1/CCL2 production, which was likely associated with the induction of IFN-gamma because this cytokine was highly induced by PPD. We characterized, for the first time, different APC from human PHLN and show that Mtb Ag exert fine and specific regulation of molecules closely associated with the immune response to Mtb infection. Because knowledge of this response in secondary lymphoid tissues is still poorly understood in humans, such studies are necessary and important for a better understanding of lymphoid cell microenvironment and migrating capacities and their role in the immunopathogenesis of tuberculosis.
Assuntos
Células Apresentadoras de Antígenos/imunologia , Antígenos de Bactérias/imunologia , Quimiocina CCL2/metabolismo , Pulmão/imunologia , Linfócitos/imunologia , Receptores CCR2/metabolismo , Adulto , Apresentação de Antígeno , Células Apresentadoras de Antígenos/efeitos dos fármacos , Antígenos de Bactérias/farmacologia , Quimiocina CCL2/análise , Quimiocinas/metabolismo , Criança , Células Dendríticas/efeitos dos fármacos , Células Dendríticas/imunologia , Citometria de Fluxo , Humanos , Lipopolissacarídeos/imunologia , Lipopolissacarídeos/farmacologia , Linfonodos/imunologia , Linfócitos/efeitos dos fármacos , Macrófagos/imunologia , Masculino , Monócitos/imunologia , Receptores CCR2/análise , Receptores de Quimiocinas/metabolismo , Tuberculina/imunologia , Tuberculina/farmacologiaRESUMO
The majority of knowledge about the role of cytokines and chemokines in controlling Mycobacterium tuberculosis infection mainly derives from animal models. In humans, this knowledge is still mainly limited to the blood compartment or accessible lymphoid organs, such as tonsils. Here, we studied cytokine and chemokine production and their modulation by M. tuberculosis antigens in mononuclear cells from human blood, spleen and hilar lung lymph nodes. Results show that the kinetics and magnitude of cytokine and chemokine production varied according to the tissue of cell origin. Mycobacterium tuberculosis antigens enhanced cytokine and chemokine production in blood, but the enhancement was restricted in spleen and hilar lung lymph node cells. We show, for the first time in humans, differences in cytokine and chemokine microenvironments according to lymphoid tissues, and suggest that these differences may affect the way cells respond to M. tuberculosis infection.