Radiocirugía estereotáxica por gamma knife en un paciente con cavernoma cerebral y síndrome de Evans

El cavernoma cerebral es una malformación vascular de diagnóstico infrecuente. Se define como una malformación a nivel de la vasculatura microcerebral que, dependiendo a la ubicación y si existe la posibilidad de ruptura, conlleva a una emergencia que puede terminar en la muerte del paciente. En esta oportunidad se reporta el caso de un paciente con cavernoma cerebral asociado al síndrome de Evans. Se decide manejo quirúrgico de la lesión por aumento de intensidad de cefalea e intolerancia oral. Dada la coexistencia del Síndrome de Evans y la alta tasa de morbimortalidad es que se decide manejo quirúrgico mediante radiocirugía estereotáxica con gamma knife. El uso de dosis de margen bajo para tratamiento con gamma knife para uso en cavernomas cerebrales produce un manejo controlado para sintomatología de convulsiones y mejor expectativa de calidad de vida.

Cerebral cavernoma is an infrequently diagnosed vascular malformation. It is defined as a malformation at the level of the microcerebral vasculature that, depending on the location and if there is a possibility of rupture, leads to an emergency that can end in the death of the patient. On this occasion, we report a case of a patient with cerebral cavernoma associated with Evans syndrome. Surgical management of the lesion was decided due to increased intensity of headache and oral intolerance. Given the coexistence of Evans Syndrome and the high rate of morbidity and mortality, surgical management was decided by stereotaxic radiosurgery with a gamma knife. The use of low-margin doses for treatment with gamma knife for use in brain cavernomas produces controlled management for seizure symptoms and better quality of life expectancy.

Pediatric Evans Syndrome: A 20-year experience from a tertiary center in Brazil

Abstract Introduction The Evans syndrome (ES) is a rare, often chronic, relapsing and treatment-refractory hematological disorder. We described the clinical features, diagnostic workup, treatment and outcome in patients with ES. Method We performed a retrospective chart review of patients aged < 18 years with ES admitted to a tertiary center in Brazil from 2001 to 2021. The analysis of the data was primarily descriptive, using median, interquartile range and categorical variables presented in absolute frequencies. Main results Twenty patients (12 female, 8 male) were evaluated in this study. The median age at the initial cytopenia was 4.98 years (1.30-12.57). The ES was secondary in nine cases (45%), of which six patients (30%) showed autoimmune disease (AID) or primary immunodeficiencies (PID) and one presented a spontaneous recovery. Steroids and intravenous immunoglobulin were first-line therapy in 19 cases. Twelve patients (63%) required second-line treatments (rituximab, cyclosporine, splenectomy, sirolimus, cyclophosphamide, mycophenolate mofetil, azathioprine and eltrombopag). The median follow-up period was 2.41 years (1.4 -7.52). One patient (5%) died of underlying neuroblastoma, one case (5%) was lost to follow-up and four patients (20%) received a medical discharge. The median age for the 14 remaining cases was 12.6 years. Twelve patients (85.7%) were in complete response (CR) with no therapies. Two patients (14.3%) were in CR with chronic therapy. Conclusion As ES may be a symptom of AID and PID, a thorough rheumatological, immunologic and genetic workup and a careful follow-up are essential. The second-line treatment remains a dilemma. Further prospective studies are needed to address the optimal therapeutic combinations, morbidity and mortality in this disorder.

Impact of baseline fluorescent antinuclear antibody positivity on the clinical outcome of patients with primary autoimmune hemolytic anemia

Abstract Introduction Autoimmune haemolytic anaemia (AIHA) is an autoimmune disorder that can present in primary or secondary forms. The literature looking at impact of baseline fluorescent antinuclear antibody (FANA) positivity on outcomes of AIHA patients is infrequent. Objective To study the impact of baseline FANA positivity in patients with primary AIHA. Method A prospective cohort study involving 29 consecutive primary AIHA patients presenting to the Haematology department from 2013 to 2015 was analysed. After recording baseline investigations including fluorescent ANA, all patients were treated as per the standard therapeutic protocols. Clinical remission, disease free survival, relapse, mortality were compared between the FANA positive and FANA Negative AIHA groups. Results Baseline FANA positivity was found in 17 patients (58.62%). Both the groups were comparable in terms of age, sex, Hemoglobin, LDH at presentation, number of lines of treatment needed and duration of follow up. Evan's syndrome was seen in six of FANA positive patients which was statistically significant (0 v/s 6, p= 0.023). FANA positive patients had significantly higher rates of relapse per patient month follow up (1.22 v/s 3.57, p= 0.023) and lower rates of complete response (83.33% v/s 35.29%, p= 0.0118) and relapse free survival at five years. Morbidity and mortality were numerically higher in FANA positive patients. Conclusion Baseline FANA positivity among AIHA patients was found to be associated with lower complete response rates and higher relapse rates with possible higher rates of morbidity. Presence of FANA will give us prognostic value and help us in deciding the treatment options.

Síndrome de Evans asociada a infección por citomegalovirus en una niña de 13 meses / Evans Syndrome Associated With Cytomegalovirus Infection In A 13-Month-Old Girl

Introducción: El síndrome de Evans es un desorden autoinmune poco frecuente, caracterizado por el descenso de al menos dos líneas celulares hemáticas. Las publicaciones del síndrome de Evans e infección por citomegalovirus resultan escasas. Objetivo: Examinar el caso de una niña con síndrome de Evans e infección activa por citomegalovirus que respondió favorablemente a la terapia antiviral. Presentación del caso: Niña de 13 meses con antecedentes de prematuridad y bajo peso al nacer, que acudió a consulta por presentar palidez y equimosis en tórax, abdomen y extremidades. En los exámenes de laboratorio se encontró trombocitopenia y anemia severa con prueba de Coombs directo positiva. Recibió pulsos de metilprednisolona con respuesta desfavorable. La carga viral resultó positiva para citomegalovirus (4019 copias de ADN) y recibió valganciclovir con evolución favorable en el seguimiento. Conclusiones: El síndrome de Evans asociado a infección por CMV es infrecuente. El tratamiento con valganciclovir podría ser beneficioso para cierto grupo de pacientes; sin embargo, hacen falta más estudios que demuestren la eficacia y seguridad de este tratamiento en este síndrome; más aún si está asociado a una elevada carga viral(AU)

Introduction: Evans syndrome is a rare autoimmune disorder, characterized by the descent of at least two blood cell lines. Publications of Evans syndrome and cytomegalovirus infection are scarce. Objective: To examine the case of a girl with Evans syndrome and active cytomegalovirus infection who responded favorably to antiviral therapy. Case presentation: A 13-month-old girl with a history of prematurity and low birth weight, who attended the consultation for presenting pallor and ecchymosis in the thorax, abdomen and extremities. Laboratory tests found thrombocytopenia and severe anemia after a positive direct Coombs test. She received pulses of methylprednisolone with unfavorable response. The viral load was positive for cytomegalovirus (4019 copies of DNA) and received valganciclovir with favorable evolution at follow-up. Conclusions: Evans syndrome associated with CMV infection is uncommon. Treatment with valganciclovir may be beneficial for a certain group of patients. However, more studies are needed to demonstrate the efficacy and safety of this treatment in this syndrome; even more so if it is associated with a high viral load(AU)

Technique optimization of intravenous or intraperitoneal injection of Evans blue combined with retinal preparations in mice / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To optimize the technique of intravenous injection of Evans blue and retinal preparations in mice, improving the accuracy and repeatability of staining experiment of retinal preparations.METHODS: C57BL/6 male mice were intravenous injected with 10g/L(1%)Evans Blue 0.3mL and circulated in vivo for 10 or 20min, and the eyes were removed after sacrificed and fixed in 4% paraformaldehyde for 20, 40 or 60min. When failure of intravenous injection, the experiment was remediated by intraperitoneal injection of 1% Evans Blue 0.3mL, circulated in vivo for 3h and fixed for 60min to observe morphology, distribution and leakage of the retinal vessels. Besides, we compared the morphology, distribution and leakage of the retinal vessels after intravenous injection with those after intraperitoneal injection to determine the optimal conditions for in vivo circulation time and retinal preparations.RESULTS: After intravenous injection, compared to the retinal vascular condition under 20min in vivo circulation time of Evans blue and 20 or 40min of fixation, with 10min of in vivo Evans blue circulation and 60min of fixation, the morphology of retinal vascular was more intact with less retinal vascular leakage, and the vascular branches are clear. When intravenous injection failed, remediated results from intraperitoneal injection showed that the morphology and distribution of retinal vessels were intact. There was no significant difference in morphology, distribution and leakage of the retinal vessels after 3h of intraperitoneal Evans blue circulation compared to 10min intravenous Evans blue circulation.CONCLUSION: This experiment optimizes the protocol, improves the accuracy and reproducibility of retinal preparations, and provides a reference for the study of related retinal vascular diseases.

Síndrome de Evans y embarazo: reporte de un caso / Evans syndrome and pregnancy: a case report

Resumen El síndrome de Evans es una enfermedad conformada por la presencia simultánea o secuencial de trombocitopenia inmunitaria y anemia hemolítica autoinmunitaria, que puede ser primaria o secundaria a otra patología. Es una afección poco frecuente, por lo que es necesario tener una alta sospecha, y descartar otras patologías que cursan con dichas alteraciones hematológicas, para hacer el diagnóstico. Su manejo representa un desafío terapéutico dado su curso crónico y recidivante. La presentación durante el embarazo se asocia a morbilidad materna y fetal. A continuación presentamos el caso de una gestante en quien se pesquisó trombocitopenia severa aislada al ingreso al control prenatal, y que en el curso del embarazo desarrolló AHAI conformando un síndrome de Evans, que se consideró secundario a LES incompleto al realizar el estudio reumatológico. Debido a la pobre respuesta al tratamiento médico con corticoides e inmunosupresores, la mayor parte del embarazo se mantuvo hospitalizada para observación, ajuste y cambio de terapia, siendo necesario recurrir a manejo quirúrgico con esplenectomía.

Abstract Evans syndrome is a rare entity formed by the simultaneous or sequential presence of immune thrombocytopenia and autoimmune hemolytic anemia, which can be primary or secondary to another pathology. The presentation of this disease during pregnancy is associated with maternal and fetal morbidity. The syndrome's diagnosis requires a high suspicion and the ruling out of other pathologies that can happen with the same hematological alterations. The management represents a therapeutic challenge because of its chronic and recurrent course. Below we present the case of a pregnant woman in whom isolated severe thrombocytopenia was detected at admission for prenatal control, and who developed AIHA during the pregnancy, forming Evans syndrome, which was considered secondary to incomplete SLE when performing the rheumatological study. Due to the poor response to medical treatment with corticosteroids and immunosuppressants, the patient was hospitalized for most of her pregnancy for observation, adjustment and change of therapy, and even it was necessary resort to surgical management with splenectomy.

Esplenectomía laparoscópica en paciente con síndrome de Evans / Laparoscopic splenectomy in patient with Evans syndrome

Resumen: El síndrome de Evans es una entidad rara que se presenta aproximadamente en 3.6 por cada millón de habitantes; siendo más común en el sexo femenino. Éste se caracteriza por la presencia de anemia hemolítica autoinmunitaria idiopática y púrpura trombocitopénica idiopática. Se presenta el caso de paciente femenino de 42 años de edad sin mejora en su nivel de plaquetas, por lo que se decide realizar esplenectomía mediante laparoscopía. La paciente, previo a la intervención quirúrgica, presenta nivel de plaquetas consideradas límites, 53,000/μL, por lo que se decide transfundir plaquetas previo al procedimiento. Durante la evaluación preanestésica se consignaron múltiples factores de riesgo para considerar una vía aérea difícil. Se optimizó el nivel de plaquetas y se mantuvieron esteroides perioperatorios. Se decidió dar anestesia general endotraqueal, con resultados satisfactorios durante el procedimiento quirúrgico.

Abstract: Evans syndrome is a rare syndrome that occurs in approximately 3.6 per million inhabitants; being more common in women. Its characteristics are the presence of idiopathic autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura. We present a case of a 42-year-old patient with no improvement in her platelet level, so it was programmed to perform a laparoscopic splenectomy. The patient before the surgical intervention presented a level of platelets considered limit, 53,000/μL, so it was decided to transfuse platelets before surgery. During the pre-anesthesia evaluation, multiple risk factors were recorded to be considered as a difficult airways. Thrombocytopenia was improved and perioperative steroids were maintained. It was decided to administer general endotracheal anesthesia, obtaining satisfactory anesthesia during the surgical procedure.

Síndrome de Evans como manifestación inicial de lupus eritematoso sistémico / Evans syndrome as an initial manifestation of systemic lupus erythematosus

El síndrome de Evans se caracteriza por la presentación simultánea de anemia hemolítica autoinmune y púrpura trombocitopénica inmune, puede presentarse como una patología aislada o como manifestación de una enfermedad sistémica. Caso Clínico: Preescolar masculino de 3 años, diagnosticado de síndrome de Evans, requirió tratamiento con corticoides e inmunoglobulina por mala respuesta inmunológica, tres meses después de su diagnóstico inicial presento afectación renal, además de presentar autoanticuerpos positivos, por lo que se estableció diagnóstico de lupus eritematoso sistémico. Conclusión: El síndrome de Evans es una entidad nosológica poco frecuente, ante su diagnóstico se debe descartar enfermedad sistémica subyacente.

Evans syndrome is characterized by the simultaneous presentation of autoimmune hemolytic anemia and immune thrombocytopenic purpura; it can be manifested as an isolated pathology or as a manifestation of a systemic disease. Clinical Case: 3-year-old preschool male, diagnosed with Evans syndrome, that required corticosteroids and immunoglobulin intravenous treatment due to poor immune response. Three months after his initial diagnosis he presents kidney affectation in addition to presenting positive auto-antibodies, with which it was established the diagnosis of systemic lupus erythematosus. Conclusion: Evans syndrome is a rare nosological entity, when the diagnosis is made an underlying systemic disease must be ruled out.

Síndrome de Evans y desregulación inmune: complejidades moleculares y etiopatogénicas / Evans syndrome and immune dysregulation: molecular and etiopathogenic complexities

Introducción: El síndrome de Evans se define como la presencia de citopenias inmunes que afectan dos o más líneas celulares simultánea o secuencialmente. Generalmente se refiere a la combinación de anemia hemolítica autoinmune con trombocitopenia inmune primaria, pero puede incluir también neutropenia autoinmune. Su etiología se atribuye a la producción de autoanticuerpos patológicos contra las células sanguíneas pero su causa real se desconoce. Objetivo: Explicar la relación del síndrome de Evans con la desregulación del sistema inmune. Método: Se realizó una revisión de la literatura en inglés y español a través del sitio web PubMed y el motor de búsqueda Google académico, de artículos publicados sobre el tema. El 69,73 por ciento correspondieron a los últimos 5 años. Conclusiones: La inmunopatología del síndrome de Evans se puede atribuir a una alteración en el desarrollo o la función de los linfocitos, de manera que el equilibrio inmunológico se inclina hacia la autorreactividad(AU)

Introduction: Evans syndrome is defined as the presence of autoimmune cytopenias affecting two or more blood cell lines, either simultaneously or sequentially. Most often, this refers to the combination of autoimmune hemolytic anemia and immune thrombocytopenia but can include autoimmune neutropenia as well. The etiology of Evans syndrome has been attributed to pathologic autoantibody production against the blood cells, but the true underlying cause remaining unknown. Objective: to explain the relationship of Evans syndrome with dysregulation of the immune system. Method: a review of the literature in English and Spanish was carried out through the PubMed website and the academic Google search engine for articles published on the subject. 69,73 percent corresponded to the last 5 years. Conclusions: the immunopathology of Evans syndrome can be attributed to an alteration in the development or function of lymphocytes, such that the immune balance is inclined towards self-reactivity(AU)

Síndrome de Evans y artritis reumatoidea como manifestaciones autoinmunes de la leucemia de células T grandes granulares / Evans syndrome and rheumatoid arthritis as autoimmune manifestations of large granular T-cell leukemia

Resumen La leucemia de células T grandes granulares es un trastorno poco frecuente de linfocitos citotóxicos. Estas células juegan un rol integral en el sistema inmunológico y se dividen en 2 linajes: T CD3 positivas y natural killer. Su proliferación y citotoxicidad descontrolada puede generar autoinmunidad o malignidad. La artritis reumatoide es la enfermedad autoinmune más común en individuos con este tipo de leucemia, sin embargo, se ha asociado a un amplio espectro de otras enfermedades autoinmunes y afecciones hematológicas incluyendo anemia hemolítica, aplasia pura de glóbulos rojos y neutropenia, que conducen a infecciones bacterianas recurrentes. Se presenta a continuación una paciente de 72 años con antecedentes de leucemia de células T grandes granulares y manifestaciones compatibles con artritis reumatoidea, que intercurre con un Síndrome de Evans grave con buena respuesta inicial y sostenida a gammaglobulina, corticoterapia, y rituximab.

Abstract Large granular T-cell leukemia is a rare cytotoxic lymphocyte disorder. These cells play an integral role in the immune system and are divided into 2 lineages: CD3 T positive and natural killer. Its proliferation and uncontrolled cytotoxicity can generate autoimmunity or malignancy. Rheumatoid arthritis is the most common autoimmune disease in individuals with this type of leukemia, however, it has been associated with a wide spectrum of other autoimmune diseases and hematological conditions including hemolytic anemia, pure red blood cell aplasia, and neutropenia, leading to recurring bacterial infections. The following is a case of a 72-year-old female with a history of large granular T-cell leukemia and manifestations compatible with rheuma toid arthritis, which occurs with a severe Evans syndrome with a good initial and sustained response to gamma globulin, corticosteroid therapy, and rituximab.

Síndrome de Evans Fisher / Evans fisher syndrome

Paciente femenina de 35 años de edad con color de la piel blanca, con antecedentes de cefalea migrañosa. Comenzó con manifestaciones purpúreas en forma de petequias y equimosis diseminadas por todo el cuerpo; palidez cutánea mucosa e íctero en piel y esclera, además de coluria. Se realizaron estudios hematológicos, humorales e inmunológicos. Es atendida en la sala de Medicina Interna del Hospital General Docente Comandante Pinares. Con evolución satisfactoria al tratamiento con esteroides y la inmunoglobulina intravenosa. Se realizó la discusión diagnóstica del caso confirmándose el Síndrome de Evans-Fisher, se revisa la bibliografía actualizada sobre la enfermedad(AU)

We report a case of a 35-year-old female patient, white skin color, with a history of migraine headache. She began with purple manifestations in the form of petechiae and ecchymoses spread throughout the body; mucous skin pallor and icterus in skin and sclera, as well as coluria. Hematological, humoral and immunological studies were carried out. She was treated in the Internal Medicine ward at Comandante Pinares General Teaching Hospital. She evolved satisfactorily to treatment with steroids and intravenous immunoglobulin. The diagnostic discussion of this case was carried out, confirming the Evans-Fisher Syndrome, the updated bibliography on the disease was reviewed(AU)

The spectrum of Evans syndrome: a literature review

Evans syndrome (ES) is an autoimmune disorder characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) and/or immune neutropenia in the absence of any underlying cause. Evans syndrome is a rare disorder although the exact frequency is unknown. No sex predilection is known and Evans syndrome has been described in all ethnic groups and at all ages. Classification of ES includes primary, with this being an exclusion diagnosis with no underlying condition, and secondary in the presence of an underlying disease. Clinical features are associated with anemia and thrombocytopenia including pallor, weakness, fatigue, jaundice, petechiae, ecchymosis, gingivorrhagia and epistaxis. First, a detailed history must be taken from the patient to determine the risk factors for developing ES then a family history of immune disorders along with a thorough physical examination. The management of Evans syndrome remains a challenge. Steroids with and without IVIG are recommended as front-line therapy. Red blood cell/platelet transfusion is indicated only in severe symptomatic patients due to the risk of exacerbations. Splenectomy may also be considered a second-line treatment.

Evans’ Index In Healthy North Indian Population: A Computed Tomographic Study

Background: As the humans age, there is decrease in size of brain tissue, increase in cerebrospinal fluid volumeand enlargement of ventricles. Brain ventricles can be studied by taking linear, planimetric or volumetricmeasurements. Linear ratios of width of ventricles to the width of skull or brain are considered to be an easy andreproducible measurement for assessment of ventricles. Evans’ index is one such linear ratio; it is the ratio ofmaximum width of frontal horns and maximum transverse internal diameter of skull in the same plane.Purpose of study: The purpose of our study was to obtain a baseline data of reference values of Evans index, inhealthy north Indian population.Materials and Methods: This study was jointly conducted by Department of Anatomy and Department ofRadiodiagnosis, King George’s Medical University, U.P, Lucknow. Axial CT scans of head region reportedradiologically normal, belonging to 100 individuals of different age groups were retrospectively collected andthoroughly analyzed using Radiant DICOM Viewer Software. Study subjects were categorized into V groups as perage: 18-30years, 31-40years, 41-50years, 51-60years and above 60years. Evans’ index was calculated.Results: Among study subjects, Evans’index ranged from 0.167 to 0.29 with a mean value of 0.23±0.02. Nosignificant association was observed between age and mean Evans’ Index (p>0.05). Mean Evans’ index was equalfor male and female and was not found to change with age.Conclusion: Study provided values for normal range of Evans’ index and proposes age dependent values of thesame for healthy adult males and females. The data could be utilized in routine radiological practice and byothers where required

Evans lateral lengthening calcaneal osteotomy in treatment of talocalcaneal coalition with hindfoot valgus deformity / 中国修复重建外科杂志

Objective: To investigate the effectiveness of the Evans lateral lengthening calcaneal osteotomy (E-LLCOT) in treatment of talocalcaneal coalition (TCC) with hindfoot valgus deformity. Methods: Between January 2014 and October 2017, 10 patients (13 feet) of TCC with hindfoot valgus deformities underwent E-LLCOTs. There were 6 males (8 feet) and 4 females (5 feet) with an age of 13-18 years (mean, 15.8 years). The disease duration was 10-14 months (mean, 11.5 months). The foot deformity was characterized by hindfoot valgus, forefoot abduction, and collapse of the medial arch. Pain site was the tarsal sinus in 4 feet, TCC in 5 feet, and ankle joint in 4 feet. There were tightness of the gastrocnemius in 3 cases (4 feet) and Achilles tendon in 7 cases (9 feet) on Silverskiold test. The preoperative American Orthopaedic Foot and Ankle Society (AOFAS) ankle-hindfoot score was 46.54±9.08 and visual analogue scale (VAS) score was 6.54±0.88 after walking 1 kilometer. The AOFAS ankle-hindfoot score and VAS score were adopted to evaluate the postoperative function of the foot. The talar-first metatarsal angle (T1MT), talonavicular coverage angle (TCA), talar-horizontal angle (TH), calcaneal pitch angle (CP), and heel valgus angle (HV) were measured after operation. Results: All incisions healed by first intention. All patients were followed up 12-30 months (mean, 18 months). At last follow-up, the AOFAS ankle-hindfoot score and VAS score were 90.70±6.75 and 1.85±0.90, respectively, showing significant differences when compared with preoperative scores ( t=-23.380, P=0.000; t=35.218, P=0.000). X-ray films showed that the osteotomy healed at 2-4 months (mean, 3 months) after operation. At last follow-up, the T1MT, TCA, TH, and HV were significantly lower than preoperative ones ( P<0.05), and the CP was significantly higher than preoperative one ( P<0.05). During the follow-up, the pain did not relieve obviously in 1 patient (1 foot), and the cutaneous branch of the sural nerve injured in 1 patient (1 foot). Conclusion: For TCC with severe hindfoot valgus deformity, E-LLCOT can effectively correct deformity and relieve pain.

Multivariate statistical analysis of Evans blue exudation spots and acupoint sensitization distribution in rats with acute intestinal mucosal injury / 针刺研究

OBJECTIVE: To observe the distribution characters of Evans Blue (EB) exudation spots in the abdominal area in acute intestinal mucosal injury (AIMI) rats by using latitude and longitude grid counting and multivariate statistical analysis and to explore the feasibility of these methods. METHODS: Twenty-four SD rats were randomly divided into blank control, 7.5%, 10%, and 12.5% mustard oil groups (n＝6 rats in each group). The AIMI model was established by mustard oil enema, followed by injection of EB (0.1 mL/100g) into the tail vein. At 5, 10, 15, 20 and 25 h after EB injection, the rats under anesthesia were fixed in supine position for observing and photographing the abdominal subcutaneous EB exudation spots. The H．E. staining was used to observe histopathological changes of colonic mucosa. The longitude and latitude grids of the abdominal region were constructed (by taking the midpoint of the superior sternum as the origin) to determine the position of the blue spots. That the coordinate grids of the two regions can be connected geometrically is termed as "characteristic region". The data were processed by using multivariate statistical analysis. RESULTS: ① H．E. staining showed edema and inflammatory cell infiltration after colonic enema of different concentrations of mustard oil. ② Clustering analysis indicated that the distribution of exudation points in the "characteristic grid" had no temporal variation trend, and was not related with the concentration of mustard oil (P>0.05). ③ Factor analysis and contour analysis about the exudation spots of EB at 7.5% concentration showed that the "characteristic region Ⅱ" of different factors presented a tendency of time-dependent exudation, i．e. reduction of exudation degree along with time (P<0.05), and it is located near "Tianshu" acupoint. ④At 5 h after injection of EB, the 8 "characteristic regions" presented an EB-concentration-dependent tendency (reduction in exudation degree along with the increase of EB-concentration), among which the exudation degree of region C (near "Tianshu" acupoint) of the abdomen was higher (P<0.05). CONCLUSION: The multivariate statistical analysis method can be used to identify the abdominal "characteristic regions" of exudation spots of EB in rats with AIMI, and the characteristic region has acupoint sensitization characteristics related to the time and severity of mucosal injury.

Maternal and perinatal outcome of Evan’s syndrome: a 5 years study in a tertiary care centre

Background: Evans syndrome is a rare autoimmune disorder characterized by simultaneous or sequential presence of a positive antiglobulin test, autoimmune haemolytic anemia (AIHA), and immune thrombocytopenia (ITP). It is characterised by frequent exacerbations and remissions within a chronic course. It was first described by Robert Evans in 1951.  Incidence of AIHA is 1 per 75 - 80,000 and ITP is 5.5 /100000 per general adult population.  Incidence of Evans syndrome is 1.8% to 10% of patients with ITP. Objective was to study the maternal and perinatal outcome of women with Evans syndrome (E).Methods: About 4 antenatal mothers were identified with Evans syndrome at St. Johns medical college and hospital, Bengaluru during the study period of 5 years from July 2013-July 2017. They were followed up during their antenatal, intra natal and postnatal period and outcomes were studied. All patients included in the study fulfilled the criteria for Evans syndrome.Results: There were 4 cases of Evans syndrome, with a total number of deliveries of 11859, during this 5 year study. Incidence was 0.09 per 1000 births. All patients presented with bleeding manifestations ranging from mucosal haemorrhage to subarachnoid haemorrhage (SAH) at the time of diagnosis. All patients were on treatment with either 1st or 2nd line of management with corticosteroids/ azathioprine. None had bleeding during pregnancy after the initiation of treatment. Patients had antenatal complications like preeclampsia 25%, IUGR 25%, oligohydraminos 50%, IUD 25%. 2 patients received platelet transfusions intrapartum. None had intrapartum or postpartum haemorrhage. There were no maternal and neonatal mortality.Conclusions: Evans syndrome in pregnancy is a rare condition and requires multi disciplinary approach involving specialists from obstetrics, neonatology, and hematology. Close maternal and fetal surveillance and management during pregnancy is essential to increase the possibility of a favourable pregnancy outcome in these women.

Morphometric Study Of Ventricular Indices In Human Brain Using Computed Tomography Scans In Indian Population

Introduction: Ventricular enlargement is a characteristic physical change that is present frequently in a numberof cerebral disorders encountered in neurologic and psychiatric practice. Quantitative assessment of neuroimagesfor ventricular size is an effective approach to reveal structural changes in conditions such as Alzheimer’sDisease, schizophrenia, Huntington’s disease, hydrocephalus and many other neurological and psychiatricdisorders.Aim: The present study is being done with the aim to establish normal ventricular indices of brain in both sexesand to find the correlation between these indices in different age groups in Indian population.Materials and Methods: The data for the present study are 300 CT scan which are collected from department ofRadiology, Ramaiah hospitals. The following indices such as Evans index, bifrontal index, bicaudate index, cellamedia index, third ventricular index , Huckman’s index and ventricular index were calculated. The parameterswere tabulated and statistically analyzed.Results: The ventricular indices showed statistically significant difference between males and females in all theindices except for cella media index. There was positive correlation coefficient between age and indices whichwas statistically significant.Conclusion: The knowledge of normal anatomy of ventricular system of brain helpful for clinicians, neurosurgeonsand radiologists in day to day clinical practice

Paludismo falciparum y trombocitopenia severa / Falciparum malaria and severe thrombocytopenia

La púrpura trombocitopénica idiopática inmune es la enfermedad hemorrágica más frecuente en niños. Entre las trombocitopenias asociadas a parásitos, sin embargo, la inducida por la malaria es prevalente. Se presenta el caso de un niño de tres años de edad, procedente de una zona endémica de paludismo en Colombia, con una malaria por Plasmodium falciparum cuyo tratamiento inicial fue desconocido, quien consultó por recaída de su cuadro clínico, el cual se caracterizó por anemia, bajas parasitemias, persistencia de gametocitos, sangrado y reticulocitosis. Las manifestaciones de la enfermedad fueron variadas, pero sobresalió la trombocitopenia profunda. Luego de diez días de estancia hospitalaria se obtuvo una mejoría notable, confirmada mediante la negativización del hemoparásito y la normalización de los valores de hemoglobina y de plaquetas.

Idiopathic immune thrombocytopenic purpura is the most frequent hemorrhagic disease in children. Among the thrombocytopenias associated with parasites, however, that induced by malaria is prevalent. We present the case of a three-year-old boy from an endemic area of malaria in Colombia, with Plasmodium falciparum malaria whose initial treatment was unknown, who consulted for relapse of his clinical picture, which was characterized by anemia, low parasitemia, persistence of gametocytes, bleeding and reticulocytosis. The manifestations of the disease were varied, but deep thrombocytopenia stood out. After ten days of hospital stay, a marked improvement was obtained, confirmed by the negativization of the hemoparasite and normalization of hemoglobin and platelet values.

β₂-Adrenoceptor Blockade Deteriorates Systemic Anaphylaxis by Enhancing Hyperpermeability in Anesthetized Mice

PURPOSE: Patients treated with propranolol, a nonselective β-adrenoceptor antagonist, develop severe anaphylaxis, but the mechanism remains unknown. We determined effects of β₁- and β₂-adrenoceptor antagonists on the anaphylaxis-induced increase in vascular permeability in mice. METHODS: In anesthetized ovalbumin-sensitized C57BL mice, mean arterial blood pressure (MBP) was measured, and Evans blue dye extravasation and hematocrit (Hct) were assessed at 20 minutes after antigen injection. The following pretreatment groups (n=7/group) were studied: (1) sensitized control (non-pretreatment), (2) propranolol, (3) the selective β₂-adrenoceptor antagonist ICI 118,551, (4) the selective β₁-adrenoceptor antagonist atenolol, (5) adrenalectomy, (6) the selective β₂-adrenoceptor agonist terbutaline, and (7) non-sensitized groups. RESULTS: The antigen injection decreased MBP, and increased Hct and vascular permeability in the kidney, lung, mesentery, and intestine, but not in the liver or spleen. Pretreatment with ICI 118,551, propranolol and adrenalectomy, but not atenolol, reduced the survival rate and augmented the increases in Hct and vascular permeability in the kidney, intestine, and lung as compared with the sensitized control group. Pretreatment with terbutaline abolished the antigen-induced alterations. Plasma epinephrine levels were increased significantly in the sensitize control mice. CONCLUSIONS: Blockade of β₂-adrenoceptor can deteriorate systemic anaphylaxis by augmenting hyperpermeability-induced increase in plasma extravasation by inhibiting beneficial effects of epinephrine released from the adrenal glands in anesthetized mice.

I-Evans blue: evaluation of necrosis targeting property and preliminary assessment of the mechanism in animal models

Necrosis is a form of cell death, which is related to various serious diseases such as cardiovascular disease, cancer, and neurodegeneration. Necrosis-avid agents (NAAs) selectively accumulated in the necrotic tissues can be used for imaging and/or therapy of related diseases. The aim of this study was to preliminarily investigate necrosis avidity of I-evans blue (I-EB) and its mechanism. The biodistribution of I-EB at 24 h after intravenous administration showed that the radioactivity ratio of necrotic to viable tissue was 3.41 in the liver and 11.82 in the muscle as determined by counting in model rats. Autoradiography and histological staining displayed preferential uptake of I-EB in necrotic tissues. nuclear extracts from necrotic cells exhibited 82.3% of the uptake in nuclei at 15 min, as well as 79.2% of the uptake at 2 h after I-EB incubation. The DNA binding study demonstrated that evans blue (EB) has strong binding affinity with calf-thymus DNA (CT-DNA) (=5.08×10 L/(mol/L)). Furthermore, the accumulation of I-EB in necrotic muscle was efficiently blocked by an excess amount of unlabeled EB. In conclusion, I-EB can not only detect necrosis by binding the DNA released from necrotic cells, but also image necrotic tissues generated from the disease clinically.