Diaphragmatic endometriosis and thoracic endometriosis syndrome: a review on diagnosis and treatment.

Endometriosis of the diaphragm has been gaining more attention in the practice of gynecologists and thoracic surgeons in recent years. Understanding related symptoms and developing imaging methods have improved their approach. A review of the literature was performed with the aim to report on incidence, diagnosis, treatment and prognosis of diaphragmatic endometriosis. We also cover the issue of the Thoracic Endometriosis Syndrome (TES). Complaints of cyclic chest pain in patients of childbearing age should have as differential diagnosis the presence of thoracic endometriosis. Catamenial pneumothorax is the main manifestation of diaphragmatic endometriosis and Thoracic Endometriosis Syndrome. Other possible manifestations are hemothorax, pulmonary nodules, and diaphragmatic hernia. Despite the possibility of drug treatment, many patients will be submitted to surgical treatment. The minimally invasive approach should be the one of choice. The robotic pathway allows for an easier approach due to its ability to articulate robotic arms, allowing the treatment of lesions in hard-to-reach locations, such as the posterior part of the diaphragm. Multidisciplinary treatment should be used in most cases, as only abdominal approach is not sufficient for the diagnosis and treatment of lesions in the thoracic cavity. The approach of endometriosis of the diaphragm and Thoracic Endometriosis Syndrome should be multidisciplinary, allowing the improvement of quality of life in most patients.

Recambio por aféresis de glóbulos rojos. Primer registro de un hospital pediátrico en Quito-Ecuador / Red blood cell exchange. First report in a pediatric Hospital in Quito-Ecuador

Introducción: La anemia falciforme es la hemoglobinopatía estructural más frecuente en todo el mundo y es causada por la producción de hemoglobina S (HbS) a consecuencia de una mutación puntual en el gen de la beta globina.Objetivo: Mostrar los beneficios del recambio de glóbulos rojos por aféresis en la presentación del síndrome torácico agu-do, causado por la anemia drepanocítica.Presentación del caso: Se describe el manejo de una paciente de trece años de edad, con anemia drepanocítica que, al momento de su ingreso al hospital, presentó crisis vaso- oclusiva secundaria a su patología de base. Al segundo día presen-tó síndrome de tórax agudo, por lo que se solicitó al Servicio de Medicina Transfusional, el recambio eritrocitario. Analizado el caso, se realizó el cálculo de la volemia total de la paciente, se prepararon concentrados de glóbulos rojos (CGRs) com-patibles con la paciente: se filtraron; y se les cuantificó el hematocrito. El procedimiento se realizó con el equipo de aféresis COM.TEC. en el que se recambió 1.200 mililitros de eritrocitos totales. Discusión: El recambio eritrocitario por aféresis aportó una notable y visible mejoría clínica y laboratorial. Por lo que en nuestra experiencia consideramos que el procedimiento fue eficiente. Conclusiones: El recambio de eritrocitos por aféresis en el síndrome torácico agudo en crisis drepanocítica es un procedi-miento que se puede utilizar en pacientes que no responden a otras terapias por su mínima alteración de la viscosidad y volumen sanguíneo en el paciente, y disminuir la concentración de hemoglobina S.

Introduction: Sickle-cell anemia is the most common structural hemoglobinopathy worldwide and is caused by the pro-duction of hemoglobin S (HbS) as a result of a point mutation in the beta globin gene.Objective: Show the benefits of red blood cell replacement by apheresis in the presentation of acute chest syndrome, cau-sed by sickle-cell anemia.Case Presentation: We describe the management of a thirteen-year-old patient with sickle-cell anemia, who presented, at admission to the hospital, an occlusive vessel crisis, secondary to her underlying pathology. On the second day of admission, she presented acute chest syndrome. Erythrocyte replacement was requested to the hospital blood service. After analyzing the case, the total blood volume of the patient was calculated, red blood cell concentrates (RBCs) compatible with the pa-tient were prepared, all RBCs were filtered, and the hematocrit was quantified in all RBCs. The procedure was performed with the apheresis equipment COM.TEC. in which a total of 1,200 milliliters of erythrocytes was replaced. Discussion: The erythrocyte replacement by apheresis contributed a remarkable and visible clinical and laboratory impro-vement. In our view, we consider that the procedure was efficient.Conclusions: The replacement of erythrocytes by apheresis in the acute thoracic syndrome in sickle cell crisis is a procedure that may be used in patients who do not respond to other therapies, benefiting from minimal alteration of the viscosity and blood volume in the patient, as well as concomitant decrease of hemoglobin S concentration.

[Case study of acute thoracic syndrome of unknown etiology in a pregnant woman]. / Syndrome thoracique aigu chez une gestante AS méconnue: à propos d'un cas.

We report a case of acute thoracic syndrome diagnosed in a Congolese pregnant woman in whom sickle-cell trait was detected after admission to hospital for fever and respiratory symptoms. Paraclinical examinations performed to detect the cause of the disease were unhelpful. Chest X-ray showed infiltrates at the base of the lungs. Sickle SCAN test kit confirmed by liquid chromatography associated with mass spectrometry showed that the patient had sickle cell trait Symptoms were quite refractory to treatment. A favorable outcome was observed after expulsion of the fetus. Clinicians should suspect acute thoracic syndrome in pregnant women with sickle-cell trait because outcome can be favorable in the postpartum period.

[Multiorgan failure after sickle cell vaso occlusive attack: integrated clinical and biological emergency]. / Complications en cascade avec défaillance multiviscérale au cours d'une drépanocytose : gestion coordonnée de l'urgence au laboratoire et au service de réanimation médicale.

We describe the case of a 30-year-old patient, suffering from composite S/ß + sickle cell disease. He was hospitalized following a vaso-occlusive attack with acute bone pains. Despite an analgesic treatment and transfusion of three units of red blood cells, a non-regenerative anemia appeared within 24 hours. One day later an acute chest syndrome with atelectasis of the left lung and desaturation and multi-organ failure occurred and necessitated the patient's intubation and required him to be placed in an artificial coma. A bronchoalveolar lavage was performed, which eliminated pneumonia but proved, after staining with oil red O, many neutral fatty acid microvacuoles in more than 80% of macrophages, suggesting a pulmonary fat embolism. The hypothesis of a bone marrow necrosis causing a pulmonary fat embolism was discussed and confirmed the next day by the characteristic appearance of the bone marrow. A therapeutic protocol associating iteratively bleeding and red blood cells transfusion was administered on the second day with the objective of maintaining haemoglobin S at less than 20% rate. Successive haemoglobin S assay was applied using a high performance liquid chromatography (HPLC) technique with a quick response within one hour after transfusion or bleeding. This protocol resulted in an improvement in the patient's condition, with a gradual normalization of vital signs and extubation twelve days later and discharge without sequelae twenty-five days later. The succession of rare but serious sickle cell complications anaemia which occurred in this patient could be controlled by adapting the laboratory for the clinical emergency.

Progressive Brachial Plexus Palsy after Osteosynthesis of an Inveterate Clavicular Fracture.

INTRODUCTION: The thoracic outlet syndrome (TOS) is a rare complication of clavicular fracture, occurring in 0.5-9% of cases. In the literature from 1965 - 2010, 425 cases of TOS complicating a claviclular fracture were described. However, only 5 were observed after a surgical procedure of reduction and fixation. The causes of this complication were due to the presence of an exuberant callus, to technical surgery errors or to vascular lesions. In this paper we describe a case of brachial plexus plasy after osteosynthesis of clavicle fracture. CASE REPORT: A 48 year old female, presented to us with inveterate middle third clavicle fracture of 2 months duration. She was an alcoholic, smoker with an history of opiate abuse and was HCV positive. At two month the fracture was displaced with no signs of union and open rigid fixation with plate was done. The immediate postoperative patient had signs of neurologic injury. Five days after surgery showed paralysis of the ulnar nerve, at 10 days paralysis of the median nerve, radial and ulnar paresthesias in the territory of the C5-C6-C7-C8 roots. She was treated with rest, steroids and neurotrophic drugs. One month after surgery the patient had signs of complete denervation around the brachial plexus. Implant removal was done and in a month ulnar and median nerve functions recovered. At three months post implant removal the neurological picture returned to normal. CONCLUSION: We can say that TOS can be seen as arising secondary to an "iatrogenic compartment syndrome" justified by the particular anatomy of the space cost joint. The appropriateness of the intervention for removal of fixation devices is demonstrated by the fact that the patient has returned to her daily activities in the absence of symptoms and good functional recovery in about three months, despite fracture nonunion.

Hidroxiurea en el tratamiento de niños y adolescentes con depranocitosis / Hydroxyurea in the treatment of children and adolescents with sickle cell anemia

Se realizó un estudio de intervención terapéutica, a fin de determinar la mejoría clínica y de los resultados de laboratorio en 163 niños afectados por drepanocitosis y tratados con hidroxiurea, los cuales fueron atendidos en el Hospital Infantil Sur de Santiago de Cuba desde septiembre de 2009 hasta igual mes de 2010. Para ello se seleccionó una muestra intencional de 22 pacientes, teniendo en cuenta criterios de inclusión y exclusión, número de eventos clínicos, parámetros de laboratorio, transfusiones e ingresos al año con el uso del medicamento; meses después de iniciado el tratamiento, fueron comparados con los mismos indicadores. Al finalizar la investigación se constató que la hidroxiurea había aumentado los niveles de hemoglobina fetal y disminuido la aparición de eventos clínicos, entre los cuales se encontraban: crisis vasooclusivas y del sistema nervioso central, síndrome torácico agudo e infecciones.

A study of therapeutic intervention was carried out, in order to determine the clinical improvement and laboratory results in 163 children affected by sickle cell anemia and who were treated with hydroxyurea, and who were assisted in the Southern Children Hospital in Santiago de Cuba from September, 2009 to the same month of 2010. For this, an intentional sample of 22 patients was selected, taking into account the inclusion and exclusion criteria, number of clinical events, laboratory parameters, transfusions and admissions in a year with the use of the medication. After some months of initiating the treatment, they were compared with the same indicators. When concluding the investigation it was verified that hydroxyurea had increased the fetal hemoglobin levels and decreased the occurrence of clinical events, among which there were: vasoocclusive and central nervous system crisis, acute thoracic syndrome and infections.

La drepanocitosis en Cuba. Estudio en niños / Drepanocytosis in Cuba: Study in children

La drepanocitosis es la anemia hemolítica determinada genéticamente más frecuente en el mundo. En Cuba, la frecuencia del estado de portador es del 3,08 por ciento en la población general. La fisiopatología de la oclusión vascular es muy compleja; involucra la polimerización de la Hb S, las alteraciones de la membrana del hematíe, las moléculas de adhesión, las citocinas inflamatorias, los factores de la coagulación y lesiones del endotelio vascular. Las manifestaciones clínicas más frecuentes son: las crisis vasooclusivas dolorosas, el síndrome torácico agudo, la crisis de secuestro esplénico, la crisis aplástica, la necrosis aséptica de la cabeza del fémur y la úlcera maleolar. El cuadro clínico es muy variable: desde niños que mueren temprano en la vida hasta pacientes que alcanzan la sexta década de la vida. En el Instituto de Hematología e Inmunología existe un Programa de Atención Integral que incluye: seguimiento sistemático desde temprano en la vida en una consulta especializada, la administración de ácido fólico de forma permanente y de penicilina oral profiláctica los primeros 5 años de la vida; así como la educación del niño y de sus padres. Desde 1986 se realiza esplenectomía parcial en la crisis de secuestro esplénico con excelentes resultados. Entre 2004-2008 fallecieron solamente 16 enfermos en todo el país y en 397 adultos la sobrevida fue de 53 años en la anemia drepanocítica y de 58 en la hemoglobinopatía SC. Como resultado de este programa, en los últimos años la sobrevida ha aumentado, la calidad de vida del paciente ha mejorado y han disminuido los costos invertidos en el tratamiento de las complicaciones

Drepanocytosis is the hemolytic anemia more frequent genetically determined in the world. In Cuba, the frequency of carrier status is of 3,08 percent in general population. The pathophysiology of vascular occlusion is very complex; includes the polymerization of the Hb S, the alterations of red-blood cells, the adhesion molecules, the inflammatory cytokines, the coagulation factors and the lesions of the vascular endothelium. The more frequent clinical manifestations are: painful vaso-occlusive crises, the acute thoracic syndrome, the splenic sequestration crisis, the aplastic crisis, the aseptic necrosis of femur head and malleolar ulcer. The clinical picture is very variable: from children dying early in life up to patients achieve the sixth decade of life. In the Institute of Hematology and Immunology there is an Integral Care Program including: systematic follow-up from early in life in a specialized consultation, permanent administration of folic acid and of prophylactic oral penicillin during the first 5 years of age; as well as the child education and of parents. From 1986 it is carried out the partial splenectomy in crises of splenic sequestration with excellent results. Between 2004-2008 in all the country deceased only 16 patients and in 397 adults the survival rate was of 53 years in the drepanocythemia and of 58 in the SC hemoglobinopathy. As result of this program, in past years the survival has increased, the quality of life of patient improved and the costs spent in treatment of complications has decrease

La drepanocitosis en Cuba. Estudio en niños / Drepanocytosis in Cuba: Study in children

La drepanocitosis es la anemia hemolítica determinada genéticamente más frecuente en el mundo. En Cuba, la frecuencia del estado de portador es del 3,08 por ciento en la población general. La fisiopatología de la oclusión vascular es muy compleja; involucra la polimerización de la Hb S, las alteraciones de la membrana del hematíe, las moléculas de adhesión, las citocinas inflamatorias, los factores de la coagulación y lesiones del endotelio vascular. Las manifestaciones clínicas más frecuentes son: las crisis vasooclusivas dolorosas, el síndrome torácico agudo, la crisis de secuestro esplénico, la crisis aplástica, la necrosis aséptica de la cabeza del fémur y la úlcera maleolar. El cuadro clínico es muy variable: desde niños que mueren temprano en la vida hasta pacientes que alcanzan la sexta década de la vida. En el Instituto de Hematología e Inmunología existe un Programa de Atención Integral que incluye: seguimiento sistemático desde temprano en la vida en una consulta especializada, la administración de ácido fólico de forma permanente y de penicilina oral profiláctica los primeros 5 años de la vida; así como la educación del niño y de sus padres. Desde 1986 se realiza esplenectomía parcial en la crisis de secuestro esplénico con excelentes resultados. Entre 2004-2008 fallecieron solamente 16 enfermos en todo el país y en 397 adultos la sobrevida fue de 53 años en la anemia drepanocítica y de 58 en la hemoglobinopatía SC. Como resultado de este programa, en los últimos años la sobrevida ha aumentado, la calidad de vida del paciente ha mejorado y han disminuido los costos invertidos en el tratamiento de las complicaciones(AU)

Drepanocytosis is the hemolytic anemia more frequent genetically determined in the world. In Cuba, the frequency of carrier status is of 3,08 percent in general population. The pathophysiology of vascular occlusion is very complex; includes the polymerization of the Hb S, the alterations of red-blood cells, the adhesion molecules, the inflammatory cytokines, the coagulation factors and the lesions of the vascular endothelium. The more frequent clinical manifestations are: painful vaso-occlusive crises, the acute thoracic syndrome, the splenic sequestration crisis, the aplastic crisis, the aseptic necrosis of femur head and malleolar ulcer. The clinical picture is very variable: from children dying early in life up to patients achieve the sixth decade of life. In the Institute of Hematology and Immunology there is an Integral Care Program including: systematic follow-up from early in life in a specialized consultation, permanent administration of folic acid and of prophylactic oral penicillin during the first 5 years of age; as well as the child education and of parents. From 1986 it is carried out the partial splenectomy in crises of splenic sequestration with excellent results. Between 2004-2008 in all the country deceased only 16 patients and in 397 adults the survival rate was of 53 years in the drepanocythemia and of 58 in the SC hemoglobinopathy. As result of this program, in past years the survival has increased, the quality of life of patient improved and the costs spent in treatment of complications has decrease(AU)