Deficiencia de lipasa ácida lisosomal, una patología infrecuente.

Lysosomal acid lipase deficiency is a genetic disease with a low prevalence and high morbidity and mortality in children and adults. It is characterized by an alteration of lipid metabolism, which generates cholesterol and triglyceride esters deposits in the body. Its clinical presentation depends on enzymatic activity. This condition should be suspected in patients with lipid or liver alterations after ruling out other diagnoses. Currently, there is the option of using a recombinant enzyme, which can improve lipid and liver parameters, as well as disease progression. Establishing a timely diagnosis in order to initiate specific treatment early is imperative for the prevention of morbidity and mortality. The purpose of this work is to perform a review of the literature about lysosomal acid lipase deficiency and to guide about its pathophysiology, clinical manifestations, diagnosis and treatment.

El déficit de lipasa ácida lisosomal es una enfermedad genética poco prevalente, con alta morbimortalidad en niños y adultos. Se caracteriza por alteración del metabolismo lipídico que genera depósitos de ésteres de colesterol y triglicéridos en el organismo. La presentación clínica depende de la actividad enzimática. Se debe sospechar en pacientes con alteraciones lipídicas o alteraciones hepáticas después de descartar otros diagnósticos. Actualmente existe la opción de utilizar enzima recombinante, la cual puede mejorar los parámetros lipídicos y hepáticos, así como detener la progresión de la enfermedad. Es imperioso realizar el diagnóstico oportuno para iniciar de forma temprana el tratamiento específico, con el fin de prevenir la morbimortalidad. Se llevó a cabo revisión de la literatura en torno del déficit de lipasa ácida lisosomal, para orientar acerca de su fisiopatología, manifestaciones clínicas, diagnóstico y tratamiento.

Deficiencia de lipasa ácida lisosomal, una patología infrecuente / Lysosomal acid lipase deficiency, a rare pathology

Resumen El déficit de lipasa ácida lisosomal es una enfermedad genética poco prevalente, con alta morbimortalidad en niños y adultos. Se caracteriza por alteración del metabolismo lipídico que genera depósitos de ésteres de colesterol y triglicéridos en el organismo. La presentación clínica depende de la actividad enzimática. Se debe sospechar en pacientes con alteraciones lipídicas o alteraciones hepáticas después de descartar otros diagnósticos. Actualmente existe la opción de utilizar enzima recombinante, la cual puede mejorar los parámetros lipídicos y hepáticos, así como detener la progresión de la enfermedad. Es imperioso realizar el diagnóstico oportuno para iniciar de forma temprana el tratamiento específico, con el fin de prevenir la morbimortalidad. Se llevó a cabo revisión de la literatura en torno del déficit de lipasa ácida lisosomal, para orientar acerca de su fisiopatología, manifestaciones clínicas, diagnóstico y tratamiento.

Abstract Lysosomal acid lipase deficiency is a genetic disease with a low prevalence and high morbidity and mortality in children and adults. It is characterized by an alteration of lipid metabolism, which generates cholesterol and triglyceride esters deposits in the body. Its clinical presentation depends on enzymatic activity. This condition should be suspected in patients with lipid or liver alterations after ruling out other diagnoses. Currently, there is the option of using a recombinant enzyme, which can improve lipid and liver parameters, as well as disease progression. Establishing a timely diagnosis in order to initiate specific treatment early is imperative for the prevention of morbidity and mortality. The purpose of this work is to perform a review of the literature about lysosomal acid lipase deficiency and to guide about its pathophysiology, clinical manifestations, diagnosis and treatment.

Tendencias de las publicaciones especializadas en el campo de la educomunicación y alfabetización mediática en Latinoamérica / Research trends in specialist publications from the field of educommunication and media literacy in Latin America / Tendências das publicações especializadas no campo da educomunicação e alfabetização midiática na América Latina

Se analizan tendencias recientes de investigación en comunicación publicadas en revistas de comunicación latinoamericanas más visibles en bases de datos de referencia y citación. La revisión se centra en los artículos de investigación publicados entre 2013 y 2017 en las nueve revistas latinoamericanas de comunicación incluidas en Scopus. La metodología se basa en la consulta de bases de datos, su sistematización a través de gestores bibliográficos y el análisis de contenido cualitativo de los textos recopilados, apoyado con el software de QDA NVIVO11. Se recopilan 167 artículos que abordan temáticas de educomunicación, alfabetización mediática y narrativas transmedia. Predomina la autoría individual y la afiliación a un solo país e institución, entre los cuales destacan Brasil y España. En relación con el contenido, predomina un enfoque temático sobre los medios y los entornos digitales y un abordaje cualitativo de las investigaciones.

This study analyzed recent research trends in communication research published in nine leading communication journals in Latin America. A literature review of scientific articles published between 2013 and 2017 was conducted using the Scopus database. A database query was run, together with the systematization of data using bibliographic management software and qualitative content analysis of selective texts using the QDA software NVivo 11. A total of 167 articles addressing the themes educommunication, media literacy, and transmedia narrative were analyzed. The majority of articles were written by individual authors belonging to a single institution and country, with emphasis on Brazil and Spain. With respect to content, the predominant themes were media and digital environments and the majority of studies used a qualitative approach.

Analisam-se as tendências recentes de pesquisa em comunicação publicadas nas nove revistas de comunicação mais significativas da América Latina. A pesquisa se centra na revisão dos artigos científicos publicados entre 2013 e 2017 em Scopus. A metodologia se baseia na consulta de bases de dados, sua sistematização por meio de gestores bibliográficos e a analise do conteúdo qualitativo dos textos selecionados, com o uso do software QDA NVIVO11. Estudam-se 167 artigos que abrangem temáticas de educomunicação, alfabetização midiática e narrativas transmídia. Pode-se identificar o predomínio da autoria individual, assim como a filiação a uma só instituição e a um só país, com destaque para Brasil e Espanha. A respeito do conteúdo, predomina o foco temático sobre mídia e os entornos digitais, assim como uma abordagem qualitativa das pesquisas.

Aspergillus hepatic artery thrombosis in liver transplantation.

We present the first cirrhotic patient who underwent liver transplantation (LT) and presented a hepatic artery thrombosis of the graft due to Aspergillus fumigatus, within the first month of LT. This culminated in graft loss, re-transplant with multiple biliary and infectious complications. To our knowledge, this is a case report of an early hepatic artery thrombosis due to Aspergillus fumigatus in an infection-free patient.

Lysosomal Acid Lipase Deficiency, a Rare Pathology: The First Pediatric Patient Reported in Colombia.

BACKGROUND Lysosomal acid lipase deficiency is a rare genetic metabolic lipid storage disease, with a high morbidity, and mortality, in children and adults. It is characterized by a mutation in the LIPA gene that causes an alteration of lipid metabolism, resulting in deposits of cholesterol esters and triglycerides in organs such as the liver, blood vessels, and gastrointestinal tract. Lysosomal acid lipase deficiency is predominantly caused by the mutation c.894G>A, seen in approximately 50-70% of patients. Our objective is to report the first pediatric case of lysosomal acid lipase deficiency in a pediatric patient in Colombia. CASE REPORT The patient is a 14-year-old boy with isolated hepatomegaly since 6 years of age without a family history of dyslipidemia. In the pediatric control, laboratory exams revealed dyslipidemia, and a hepatic biopsy was performed, revealing severe fibrosis with septation and grade 3 microvesicular steatosis (>75%). He was referred to our center and was suspected to have lysosomal acid lipase deficiency. Enzymatic activity was measured, showing absent activity. Confirmatory diagnosis with genetic sequencing showed a pathological homozygous mutation of c.894G>A. CONCLUSIONS Lysosomal acid lipase deficiency can manifest as early- or late-onset, with variable and severe signs and symptoms. The late-onset form has a broad spectrum of manifestations with mild symptoms, leading to under-diagnosis, which increases the actual disease burden. Early diagnosis is essential to initiate enzyme replacement therapy, since the natural disease course can be changed. More studies should be conducted in Latin America to evaluate the prevalence of the disease.

[Invasive pulmonary aspergillosis in children with hepatic transplant: a survivor]. / Aspergilosis pulmonar invasora en pacientes pediátricos con trasplante hepático, a propósito de una sobreviviente.

INTRODUCTION: Mycotic infections due to Aspergillus spp, are the main mycotic associated infections in liver transplant patients, with mortality rates up to 90% of the cases. Almost 50% of patients will de velop an infection during the first months after transplantation, of which 10% are associated with op portunistic agents. OBJECTIVE: To describe the diagnosis and management of an Invasive Pulmonary Aspergillosis (IPA) episode in a liver transplant patient. CASE REPORT: 11-months-old patient with liver transplant due to a biliary atresia who developed severe pneumonia associated with mechanical ventilation. The bronchoalveolar lavage showed high levels of galactomannan and positive culture for Aspergillus fumigatus leading to an IPA diagnosis. This episode was treated with antifungal with a favorable outcome. CONCLUSION: The IPA is an opportunistic infection in liver transplant patients, with high mortality rates, that must be suspected in this group of patients since an early diagnosis and treatment reduce mortality.

Aspergilosis pulmonar invasora en pacientes pediátricos con trasplante hepático, a propósito de una sobreviviente / Invasive pulmonary aspergillosis in children with hepatic transplant: a survivor

INTRODUCCIÓN: Las infecciones por Aspergillus spp son la principal infección micótica por hongos en pacientes con trasplante hepático, con una mortalidad reportada de hasta un 90% de los casos. En los pacientes trasplantados de hígado se espera que hasta un 50% desarrollen un episodio infeccioso en sus primeros meses postrasplante, de los cuales un 10% se asocian con agentes oportunistas. OBJETIVO: Describir el diagnóstico y manejo de un episodio de Aspergilosis Pulmonar Invasora (API) en una paciente con un trasplante hepático CASO CLÍNICO: Paciente de 11 meses de vida, con trasplante hepático secundario a atresia de vías biliares. En el periodo post-trasplante inmediato evolucionó con una neumonía grave asociada a ventilación mecánica. El lavado broncoalveolar presentó niveles altos de galactomanano y cultivo positivo para Aspergillus fumigatus, diagnosticándose una API. Este episodio se trató con un esquema de antifúngico con un resultado clínico favorable. CONCLUSIÓN: La API es una infección oportunista en pacientes con trasplante hepático, que debe ser sospechada en este grupo de pacientes, ya que el diagnóstico y tratamiento oportuno impacta directamente en la resolución de la infección por Aspergillus fumigatus.

INTRODUCTION: Mycotic infections due to Aspergillus spp, are the main mycotic associated infections in liver transplant patients, with mortality rates up to 90% of the cases. Almost 50% of patients will de velop an infection during the first months after transplantation, of which 10% are associated with op portunistic agents. OBJECTIVE: To describe the diagnosis and management of an Invasive Pulmonary Aspergillosis (IPA) episode in a liver transplant patient. CASE-REPORT: 11-months-old patient with liver transplant due to a biliary atresia who developed severe pneumonia associated with mechanical ventilation. The bronchoalveolar lavage showed high levels of galactomannan and positive culture for Aspergillus fumigatus leading to an IPA diagnosis. This episode was treated with antifungal with a favorable outcome. CONCLUSION: The IPA is an opportunistic infection in liver transplant patients, with high mortality rates, that must be suspected in this group of patients since an early diagnosis and treatment reduce mortality.

Principales causas de negativa familiar a la donación de órganos y tejidos: 10 años de experiencia en un centro latinoamericano / Main causes of family refusal to organ and tissue donation: 10 years of experience in a latin american center

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Impact of the new kidney allocation system A2/A2B → B policy on access to transplantation among minority candidates.

Blood group B candidates, many of whom represent ethnic minorities, have historically had diminished access to deceased donor kidney transplantation (DDKT). The new national kidney allocation system (KAS) preferentially allocates blood group A2/A2B deceased donor kidneys to B recipients to address this ethnic and blood group disparity. No study has yet examined the impact of KAS on A2 incompatible (A2i) DDKT for blood group B recipients overall or among minorities. A case-control study of adult blood group B DDKT recipients from 2013 to 2017 was performed, as reported to the Scientific Registry of Transplant Recipients. Cases were defined as recipients of A2/A2B kidneys, whereas controls were all remaining recipients of non-A2/A2B kidneys. A2i DDKT trends were compared from the pre-KAS (1/1/2013-12/3/2014) to the post-KAS period (12/4/2014-2/28/2017) using multivariable logistic regression. Post-KAS, there was a 4.9-fold increase in the likelihood of A2i DDKT, compared to the pre-KAS period (odds ratio [OR] 4.92, 95% confidence interval [CI] 3.67-6.60). However, compared to whites, there was no difference in the likelihood of A2i DDKT among minorities post-KAS. Although KAS resulted in increasing A2/A2B→B DDKT, the likelihood of A2i DDKT among minorities, relative to whites, was not improved. Further discussion regarding A2/A2B→B policy revisions aiming to improve DDKT access for minorities is warranted.

Hepatitis A, cardiomyopathy, aplastic anemia, and acute liver failure: A devastating scenario.

Hepatitis A virus (HAV) causes an acute infection and is usually asymptomatic in children. When clinical manifestations appear, these include choluria, jaundice, and abdominal pain. Although infrequent, extra-hepatic manifestations related to HAV have been described, affecting the heart, bone marrow, blood vessels, and other tissues.A 10-year-old boy from a rural area presented with a 15-day history of malaise, fever, and jaundice; laboratory examinations were compatible with HAV infection. The patient turned encephalopathic and was remitted to our center, where laboratory examinations showed a medullary aplasia and fulminant hepatitis requiring a liver transplant that was performed 72 hours after admission. At 24 hours post transplant, the patient developed a cardiomyopathy secondary to HAV, and intravenous immunoglobulin was administered. The patient is still alive and attending his medical check-ups.Although rare, extra-hepatic manifestations of HAV infection have been described in 14% of cases. The groups of patients affected are usually aged and present with high bilirubin levels. Acquired aplastic anemia and myocarditis caused by HAV are uncommon, and its pathophysiology has not yet been elucidated.HAV infection is usually asymptomatic in children, although extra-hepatic manifestations can appear requiring early detection and management.

Donantes de órganos en muerte cerebral por herida por proyectil de arma de fuego en cráneo: una trágica realidad que beneficia receptores de órganos en Colombia / Fire arm wounded cerebral death organ donors: a tragic reality that benefits organ receptors in Colombia

Antecedentes y objetivo. Los pacientes con muerte encefálica por trauma craneoencefálico secundario a herida por proyectil de arma de fuego ayudan a mitigar la necesidad de donantes de órganos y tejidos. En Colombia, el 78 % de los homicidios son por arma de fuego. Se buscaba describir la experiencia con donantes de órganos y tejidos con muerte encefálica secundaria a trauma craneoencefálico por herida por proyectil de arma de fuego en un centro colombiano de trasplantes. Materiales y métodos. Se trató de un estudio de cohorte histórica retrospectiva. Se caracterizaron 169 donantes con diagnóstico de muerte encefálica secundaria a trauma craneoencefálico por herida con proyectil de arma de fuego en la institución, entre 2010 y 2016. Las variables cualitativas se evaluaron mediante proporciones, las variables cuantitativas continuas, con medidas de tendencia central, y la supervivencia, con el método de Kaplan-Meier. Resultados. La mayoría de los pacientes eran de sexo masculino (93 %), con una mediana de edad de 21 años (rango intercuartílico, RIQ=17-27) y el 32 % tenía antecedentes de consumo de sustancias psicoactivas. El 12 % de los pacientes necesitó manejo con hemoderivados y el 62 % requirió dosis altas de vasopresores. Se trasplantaron 338 órganos sólidos. La supervivencia del injerto renal y hepático a un año fue de 90 % y 85 %, respectivamente, y a 5 años, de 87 % y 76 %. Conclusiones. Según este estudio, la mayoría de estos pacientes eran hombres jóvenes con órganos potencialmente trasplantables que mostraron excelentes resultados en la supervivencia de los injertos. Consideramos que estos donantes son una buena alternativa para aumentar el número de donantes de órganos y tejidos

Background and objectives: Patients with brain death (BD) due to traumatic brain injury (TBI) secondary to a gunshot (GS) wound in the skull contribute to mitigate the demand for organ and tissue donors. In Colombia, 78% of the homicides are by GS. We describe our experience with BD secondary to TBI by GS organ and tissue donors at a transplant center in Cali, Colombia. Materials and methods: This is a retrospective historical cohort study for characterization of donors diagnosed with BD secondary to TBI by GS at our institution in the period 2010-2016 (n=169). Qualitative variables were assessed by proportions, continuous quantitative variables with measures of central tendency, and survival with Kaplan Meier. Results: The majority of the patients were male (93%), with a median age of 21 years (RIQ 17-27), 32% had history of psychoactive substance use. 12% of the patient's required management with blood products and 62% with high doses of vasopressors. 338 solid organs were transplanted. Survival of the renal and hepatic graft at one year was 90% and 85% respectively and at five years of 87% and 76%. Conclusions: According to our study, the majority of these patients are young men with potentially transplantable organs, showing excellent results in the survival of the grafts. We consider these donors to be a good alternative to increase the number of organ and tissue donors

Seguimiento inmunológico con anticuerpos anti-HLA por tecnología Luminex™, ¿se puede prevenir el rechazo agudo celular y humoral en trasplante de intestino? / Anti-HLA antibody immunological follow up by the Luminex TM. Is it possible to prevent the acute cell and humoral immune rejection in small bowel transplant? Report of one case

Introducción. El trasplante de intestino mejora la supervivencia de pacientes con falla intestinal secundaria al síndrome de intestino corto. Estos receptores tienen gran riesgo de rechazo agudo, por lo cual, de manera protocolaria y como método de referencia, se practican biopsias intestinales. En este reporte de caso se hizo el seguimiento inmunológico de anticuerpos anti-HLA por tecnología Luminex™ (LSA) de un paciente con trasplante de intestino más biopsias por protocolo para un diagnóstico temprano, y una adecuada correlación histológica. Presentación del caso. Se trata de un paciente de 20 años de edad con síndrome de intestino corto, que ingresó a la Fundación Valle del Lili (Cali, Colombia) y requirió un trasplante aislado de intestino. El seguimiento inmunológico se hizo con tecnología Luminex™ y biopsias intestinales mensuales. Según la tamización contemplada en el protocolo previo al trasplante, el paciente tuvo anticuerpos anti-HLA (PRA de clase I y II) negativos; y a los 11 meses después del trasplante, los anticuerpos anti-HLA de clase I y II fueron positivos. Con la prueba de LSA se detectó un anticuerpo específico contra donantes (Donor Specific Antibodies, DSA) y varios anticuerpos contra otros subtipos moleculares. Se tomó una biopsia que mostró un leve rechazo celular agudo y se inició tratamiento con plasmaféresis. Hasta 21 meses después del trasplante, el paciente no ha presentado rechazos clínicos y ha tenido una adecuada evolución clínica y paraclínica Conclusión. Este es el primer trasplante de intestino en nuestro centro, en el que se hace seguimiento inmunológico con tecnología Luminex™. Consideramos que la detección con DSA es un buen marcador de rechazo agudo humoral, que permitiría una aproximación diagnóstica y una intervención oportuna

Background: Small bowel transplant improves survival of the recipients that have intestinal failure secondary to short bowel syndrome. These patients have a high risk of acute rejection; for this reason bowel biopsies are performed as protocol and is the gold standard. Immunological follow-up of anti-HLA antibodies with Luminex® technology (LSA) was carried out in a patient with intestinal transplant and biopsies were performed to achieve an early diagnosis and a suitable histological correlation. Case report: A 20-year-old patient with short bowel syndrome secondary to extensive intestinal resection due to a complicated appendicitis underwent isolated bowel transplantation. The post-transplant immunological follow-up was performed with LSA and monthly intestinal biopsies. Antibodies with mean fluorescence intensity greater than 1500 were positive. During the pre-transplant protocol, the patient was screened for anti-HLA antibodies with negative results. Eleven months post-transplant, the screening test for anti-HLA Class I and II antibodies was positive; the specificity of the LSA test detected one specific donor antibody (DSA) and several antibodies against other molecular subtypes. The biopsy result was a mild acute cellular rejection and plasmapheresis therapy was started. The patient has not presented a clinical rejection, and at 21 months post-transplantation exhibits an adequate clinical and paraclinical evolution. Conclusions: This is the first small bowel transplant where immunological follow-up is done with LSA. We believe that the detection of DSA is a marker of acute humoral rejection that allows a diagnostic approach and a timely intervention

Hepatoblastoma: Transplant Versus Resection Experience in a Latin American Transplant Center.

BACKGROUND: Hepatoblastoma is the most common primary malignant liver tumor in children and is usually diagnosed during the first 3 years of life. Overall survival has increased 50% due to chemotherapeutic schemes, expertise surgery centers, and liver transplantation. METHODS: A retrospective collection of data was performed from pediatric patients with diagnosis of hepatoblastoma. Variables included demographic, diagnostic tools and histological classification; chemotherapy and surgical treatment; and outcomes and patient survival. The PRETEXT classification was applied, which included the risk evaluation, and according to the medical criterion in an individualized way, underwent resection or transplant. The morbidity of patients was evaluated by the Clavien-Dindo classification. Statistical analysis was performed according to the distribution of data and the survival analysis was carried out using the Kaplan-Meier method. RESULTS: The patients (n = 16) were divided in a resection group (n = 8) and a transplant group (n = 8). The median age at the time of diagnosis was 13.5 months. The motive for the initial consultation was the discovery of a mass; all patients had high levels of α-fetoprotein and an imaging study. Ten of 16 patients required chemotherapy before the surgical procedure. In the resection group, 5 of 8 patients were classified as Clavien I and 4 of 8 patients of the transplant group were classified as Clavien II. Patient survival at 30 months was 100% in the resection group and 65% in the liver transplantation group. CONCLUSIONS: To our knowledge, this is the first case report of pediatric patients with hepatoblastoma and liver resection or transplant in Colombia and Latin America. Our results are comparable with the series worldwide, showing that resection and transplant increase the survival of the pediatric patients with hepatoblastoma. It is important to advocate for an increase of reporting in the scientific literature in Latin America.

Experiencia de trasplante renal de donantes y receptores mayores de 60 años en la Fundación Valle del Lili en Cali, Colombia, del año 2002 al 2016 / Kidney transplantation in donors and recipients over 60 at Fundación Valle del Lili in Cali, Colombia, from 2002 to 2016

INTRODUCCIÓN: El trasplante renal es el tratamiento de elección para los pacientes con insuficiencia renal terminal. Los pacientes mayores de sesenta años representan la población de mayor crecimiento con esta patología. Sin embargo, no se realizan los trasplantes de manera oportuna y la mayoría permanecen en diálisis con una menor sobrevida y calidad de vida. En este estudio se exponen los desenlaces de los trasplantes renales anciano-para-anciano realizados en una clínica de alta complejidad en Cali, Colombia. MATERIAL Y MÉTODOS: Estudio de cohorte, descriptivo de 31 trasplantes renales con donantes y receptores mayor de 60 años, realizados en la Fundación Valle del Lili en Cali, Colombia, desde enero del 2002 a marzo de 2016. RESULTADOS: De los 31 pacientes trasplantados renales, el 16% presentaron enfermedad cardiovascular post-trasplante, el 6,4% enfermedad cerebrovascular y el 22,6% malignidad. Se presentaron 12 (38,7%) infecciones oportunistas. Cinco pacientes (16%) presentaron disfunción crónica del injerto y tres (9,6%) pérdida del injerto. Nueve pacientes (29%) fallecieron con injerto funcionante. CONCLUSIÓN: La supervivencia de los pacientes trasplantados anciano para anciano en la Fundación Valle del Lili, es equiparable con los resultados en la literatura mundial. Las principales complicaciones asociadas a este tipo de trasplantes son malignidad, infecciones y patologías cardiovasculares. Debido a la alta complejidad y complicaciones de este tipo de trasplantes, los pacientes deben ser cuidadosamente seleccionados

INTRODUCTION: Kidney transplant is the first-line therapy for end-stage renal disease. Patients over 60 constitute a population which is increasingly affected by this disease. However, they do not receive timely transplantation and most of them stay on dialysis treatment with a reduction of their survival time and life quality. In this study we show the results of the kidney transplants between elderly patients performed at a private tertiary care hospital in Cali, Colombia. METHODS: This descriptive, cohort study includes 31 kidney transplants with donors and recipients over 60, which were carried out at Fundación Valle del Lili in Cali, Colombia, from January 2002 to March 2016. RESULTS: The average ages were 66 for recipients and 65 for donors. In most cases (90%) deceased donors were involved. The main cause of renal disease was diabetic nephropathy. CONCLUSION: The survival rate for the patients who underwent this procedure at the center mentioned above is similar to the results shown in the literature all over the world. The most common complications associated with this kind of operation are malignancy, infections and cardiovascular pathologies. Candidates for this transplantation should be carefully chosen given its complexity and related complications

Effects of dobutamine on intestinal microvascular blood flow heterogeneity and O2 extraction during septic shock.

Derangements of microvascular blood flow distribution might contribute to disturbing O2 extraction by peripheral tissues. We evaluated the dynamic relationships between the mesenteric O2 extraction ratio ([Formula: see text]) and the heterogeneity of microvascular blood flow at the gut and sublingual mucosa during the development and resuscitation of septic shock in a swine model of fecal peritonitis. Jejunal-villi and sublingual microcirculation were evaluated using a portable intravital-microscopy technique. Simultaneously, we obtained arterial, mixed-venous, and mesenteric blood gases, and jejunal-tonometric measurements. During resuscitation, pigs were randomly allocated to a fixed dose of dobutamine (5 µg·kg-1·min-1) or placebo while three sham models with identical monitoring served as controls. At the time of shock, we observed a significant decreased proportion of perfused intestinal-villi (villi-PPV) and sublingual percentage of perfused small vessels (SL-PPV), paralleling an increase in [Formula: see text] in both dobutamine and placebo groups. After starting resuscitation, villi-PPV and SL-PPV significantly increased in the dobutamine group with subsequent improvement of functional capillary density, whereas [Formula: see text] exhibited a corresponding significant decrease (repeated-measures ANOVA, P = 0.02 and P = 0.04 for time × group interactions and intergroup differences for villi-PPV and [Formula: see text], respectively). Variations in villi-PPV were paralleled by variations in [Formula: see text] (R2 = 0.88, P < 0.001) and these, in turn, by mesenteric lactate changes (R2 = 0.86, P < 0.001). There were no significant differences in cardiac output and systemic O2 delivery throughout the experiment. In conclusion, dynamic changes in microvascular blood flow heterogeneity at jejunal mucosa are closely related to the mesenteric O2 extraction ratio, suggesting a crucial role for microvascular blood flow distribution on O2 uptake during development and resuscitation from septic shock.NEW & NOTEWORTHY Our observations suggest that dynamic changes in the heterogeneity of microvascular blood flow at the gut mucosa are closely related to mesenteric O2 extraction, thus supporting the role of decreasing functional capillary density and increased intercapillary distances on alterations of O2 uptake during development and resuscitation from septic shock. Addition of a low-fixed dose of dobutamine might reverse such flow heterogeneity, improving microcirculatory flow distribution and tissue O2 consumption.

Sclerosing Encapsulated Peritonitis: A devastating and infrequent disease complicating kidney transplantation, case report and literature review.

INTRODUCTION: Sclerosing Encapsulating Peritonitis (SEP) is a rare condition with an incidence of up to 3% and a mortality of up to 51% among peritoneal dialysis (PD) patients (Brown et al., Korte et al. and Kawanishi et al.). In the last ten years, the incidence of SEP in kidney transplant recipients has increased (Nakamoto, de Sousa et al. and Korte et al.). PRESENTATION OF CASE: A 31-year old male with a 15 years history of PD and later kidney retransplantation was admitted to the emergency service after experiencing several weeks of diffuse abdominal pain which had escalated to include vomiting and diarrhea during the 24h previous to admission. The patient underwent an exploratory laparotomy where severe peritoneal thickening was found, in addition to signs of chronic inflammation and blocked intestinal loops. Histopathologic findings were suggestive of sclerosing peritonitis. After two months of treatment in hospital, the patient presented an obstructed intestine, with a rigid and thickened peritoneum compromising all the intestinal loops. DISCUSSION: Despite being rare, SEP, represents a significant complication due to its high mortality and recurrence. It is insidious in its early stages and culminates in an intestinal obstruction (Fieren). Risk factors for its development in kidney transplant recipients include a history of prolonged treatment with PD and the use of calcineurin inhibitors as an immunosuppressive treatment (Korte et al.). CONCLUSION: Given the increase in the incidence of SEP in kidney transplant recipients, the clinician should be alert to the presence of this complication. A greater number of multi-centre studies are required to identify the risk factors for SEP that are inherent in renal transplant recipients.

Insuficiencia renal y trasplante post donación / Renal failure and transplant in a former donor

El trasplante renal es el tratamiento de elección para los pacientes con enfermedad renal terminal. El trasplante con donante vivo, es la mejor opción para los receptores al implicar menor morbi-mortalidad y disminución del tiempo en lista activa. A pesar que el riesgo de ser donante vivo ha sido determinado y es bajo, se debe realizar una evaluación médica a los posibles donantes para identificar factores de riesgo para desarrollar insuficiencia renal crónica. En este reporte se describe un paciente quien fue donante y 21 años después desarrolló insuficiencia renal crónica (IRC) avanzada secundaria a hipertensión arterial no tratada por lo que fue trasplantado

Kidney transplant is the first-line therapy for end-stage renal disease. Living-donor transplant is the best choice for recipients as it reduces morbidity and mortality and the time spent on the active waitlist. Although it is known that the risk of being a living donor is low, a medical evaluation must be performed in order to identify risk factors for the development of chronic kidney disease. We report a case of a patient who was a donor and 21 years later presented advanced chronic kidney disease (CKD) following untreated high blood pressure. For this reason, the patient underwent a transplant

En-Bloc Transplant of the Liver, Kidney and Pancreas: Experience from a Latin American Transplant Center.

BACKGROUND En-bloc transplantation is a surgical procedure in which multiple organs are transplanted simultaneously. It has some similarities with multi-organ transplantation but offers certain advantages. This report highlights the experience of our interdisciplinary group regarding the treatment and follow-up of patients who received en-bloc transplantation, with the aim of encouraging the development of this surgical technique. CASE REPORT The first case is a 38-year-old patient with type 1 diabetes mellitus, liver cirrhosis, and chronic kidney failure who received an en-bloc transplant of the liver, pancreas, and kidney with no intraoperative complications. He had a prolonged hospital stay due to anemia and systemic inflammatory response syndrome, which were resolved successfully. At follow-up, he had no requirement for insulin or for dialysis, or for new interventions. The second case describes a 48-year-old patient with type 2 diabetes mellitus, renal failure, and liver cirrhosis who received an en-bloc transplant of the liver, pancreas, and kidney with no complications. During the postoperative period, the patient suffered a possible episode of acute tubular necrosis, which evolved towards improvement, with a tendency to normal metabolic and renal functioning, with no additional events. The patient is currently in follow-up and is insulin-independent. CONCLUSIONS En-bloc transplantation is a safe procedure, which is technically simple and which achieves excellent results. This procedure is indicated in patients with end-stage renal disease, cirrhosis, and diabetes mellitus that is difficult to control.

Clinical xenotransplantation, a closer reality: Literature review. / Xenotrasplantes, una realidad cercana en la práctica clínica: revisión de la literatura.

Xenotransplantation could provide an unlimited supply of organs and solve the current shortage of organs for transplantation. To become a reality in clinical practice, the immunological and physiological barriers and the risk of xenozoonosis that they possess should be resolved. From the immunological point of view, in the last 30 years a significant progress in the production of transgenic pigs has prevented the hyperacute rejection. About xenozoonosis, attention has been focused on the risk of transmission of porcine endogenous retroviruses; however, today, it is considered that the risk is very low and the inevitable transmission should not prevent the clinical xenotransplantation. Regarding the physiological barriers, encouraging results have been obtained and it's expected that the barriers that still need to be corrected can be solved in the future through genetic modifications.