ABSTRACT
El trasplante hepático pediátrico (THP) es la única alternativa de tratamiento para niños que padecen enfermedades hepáticas terminales, ya sean éstas agudas o crónicas. En Chile el THP representa aproximadamente un 20 por ciento del total de trasplantes de hígado realizados en nuestro país. Objetivo: Exponer las indicaciones, procedimientos y principales complicaciones del THP desde el punto de vista teórico y realizar un estudio descriptivo de la experiencia recogida por el grupo de trasplante pediátrico de Clínica Las Condes y Hospital Luis Calvo Mackenna desde 1994 a 2009 en esta materia. Material y Métodos: Se recolectaron los datos de 209 trasplantes hepáticos realizados a 173 pacientes menores de 18 entre 1994 y 2009 en ambos centros, realizando estadística descriptiva y curvas de sobrevida de Kaplan y Meier. Resultados: Las principales causas de trasplante fueron Atresia de Vías Biliares (48 por ciento) y Falla hepática aguda (28 por ciento). Cuarenta y un trasplantes se realizaron en niños menores de 10 kilos. Se trasplantaron con donante vivo 27 por ciento de los casos. Las complicaciones vasculares se presentaron en 13 por ciento de los casos y las biliares en 14 por ciento. Rechazos moderados o severos se diagnosticaron en 36 por ciento de los trasplantes. Enfermedad por Citomegalovirus se detectó en 30 por ciento de los pacientes dentro del primer año post trasplante y síndrome linfoproliferativo en 5 por ciento. La sobrevida actuarial de pacientes a 1 año ha sido de 76 por ciento, a 5 años de 67 por ciento y a 10 años de 65 por ciento. Conclusión: Los resultados del THP en términos de sobrevida y complicaciones es similar a la experiencia de centros extranjeros, la cual es superior a las expectativas y calidad de vida de los pacientes sin trasplante. Las áreas de mayor desarrollo de nuestro centro y que presentan los mayores desafíos son el trasplante hepático en niños menores de 10 kilos, en falla hepática aguda, y el uso de donante vivo.
Pediatric liver transplantation (PLT) is the only therapeutic option for children with acute or chronic end stage liver disease. In Chile PLT represents approximately 20 percent of all liver transplants performed. Objective: To present indications, procedures and main complications of PLT from an theoretic point of view and describe the experience achieved by the pediatric liver transplant team of Clínica Las Condes and Hospital Luis Calvo Mackenna from 1994 to 2009 in this field. Methods: Data from 209 liver transplants performed in 173 patients younger than 18 years old, between 1994 and 2009 were analyzed with descriptive statistics. Kaplan-Meiersactuarial survival graphs were calculated. Results: The main liver diseases leading to PLT were Biliary Atresia (48 percent) and Acute Liver Failure (28 percent). Forty-one transplants were performed in children weighting b 10 kilograms. Living donor was used in 27 percent of the cases. Vascular complications occurred in 13 percent and biliary reoperations performed in 14 percent. Moderate and severe acute graft rejection was diagnosed in 36 percent of the transplants. Cytomegalovirus disease occurred in 30 percent of the cases and post transplant lymphoproliferative disease in 5 percent. Actuarial patients survival at 1 year has been 76 percent, at 5 years 67 percent and at 10 years 65 percent. Conclusion: Results of PLT in terms of survival and complications is comparable to foreign transplant centers, which is superior to life expectancy and quality of life of these patients without liver transplant. The areas that have exhibit major development in our center and represent the biggest challenge are liver transplant in small children, in patients with acute liver failure, and the use of living donors.
Subject(s)
Humans , Adolescent , Child , Liver Failure/epidemiology , Graft Rejection/epidemiology , Liver Transplantation/statistics & numerical data , Chile , Epidemiology, Descriptive , Monitoring, Physiologic , Patient Selection , Quality of Life , Survival Analysis , Liver Transplantation/adverse effects , Liver Transplantation/mortality , Waiting ListsABSTRACT
Cytomegalovirus (CMV) infection and disease in transplant (Tx) recipients may severely complícate the patients outcome. Aim: To determine the incidence, clinical characteristics and risk factors for CMV infection and disease in liver and kidney transplant recipients in a tertiary care children's hospital. Method: A clinical and laboratory evaluation was prospectively performed in 44 and 20 children receiving a renal and liver Tx respectively in the Hospital Luis Calvo Mackenna between 2004 and 2006. Results: At the time of the organ Tx 20.3 percent (13/64) children were seronegative for CMV. Thirty six per cent (23/64) patients were infected with CMV, of whom 32 percent (14/44) received kidney Tx and 9/20 (45 percent) received liver Tx. CMV disease occurred in 52 percent (12/23) of infected patients. CMV disease was characterized by fever (100 percent), anemia (50 percent), leucopenia (16.6 percent) and specific organ involvement (renal graft 60 percent liver graft 57.1 percent, lung 25 percent, intestine 16.6 percent). Variables significantly associated with infection were a CMV seronegative status (p = 0.035) and lower age 5.5 + 3.7 years oíd vs 8.3 + 4.4 years oíd (p = 0.01). Conclusions: Incidence of CMV infection was high in children receiving a solid organ transplant in our institution and near half of infected children developed CMV-associated disease.
La infección y enfermedad por citomegalovirus (CMV) en pacientes sometidos a trasplantes (Tx) es una complicación que condiciona la evolución del injerto y la sobrevida del paciente. Objetivos: Determinar la incidencia de infección y enfermedad por CMV durante los primeros seis meses de efectuados Tx hepático y renal. Caracterizar la enfermedad, e identificar factores de riesgo asociados a infección. Metodología: Análisis prospectivo en 64 pacientes pediátricos sometidos a Tx renal (n: 44) o hepático (n: 20) en el Hospital Luis Calvo Mackenna entre 2004 y 2006. Resultados: Al trasplante, 23,1 por ciento (13/64) eran receptores IgG CMV (-). Cumplieron criterio de infección 36 por cientoo (23/64) de los pacientes, con Tx renal 32 por ciento (14/44) y con Tx hepático 45 por ciento (9/20). Desarrolló enfermedad el 52 por ciento) (12/23) la que se caracterizó porfiebre (100 por cientoo), anemia (50 por cientoo), leucopenia (16,6 por cientoo), disfunción del órgano trasplantado 60 por cientoo en Tx renal, hepático 57, l por cientoo, compromiso pulmonar en 25 por cientoo e intestinal en 16,6 por cientoo del total de pacientes. Variables asociadas a infección fueron: ser receptor IgG CMV (-)pre-Tx (p=0,035) y una menor edad del paciente 5,5 +3,7 vs 8,3 + 4,4 (p= 0,01). Conclusiones: Hay una elevada tasa de infección por CMV en la población de pacientes con Tx renal y hepática en nuestro medio, la mitad de ellos desarrolló enfermedad amenazando la función del injerto.
Subject(s)
Adolescent , Child , Child, Preschool , Humans , Infant , Cytomegalovirus Infections/etiology , Kidney Transplantation , Liver Transplantation , Antiviral Agents/therapeutic use , Cytomegalovirus Infections/drug therapy , Cytomegalovirus Infections/immunology , Ganciclovir/therapeutic use , Immunocompromised Host , Incidence , Prospective Studies , Risk FactorsABSTRACT
Background: Ample use of serological markers of high sensitivity and specificity led to relevant changes in the epidemiology of celiac disease. The impact of these changes in our country is poorly known. Aim: To assess the diagnostic procedures, clinical presentations and follow up of celiac disease as conducted in current pediatric practice. Material and methods: A multicentric retrospective study of patients diagnosed between 2000 and 2005 in five pediatric hospitals in Santiago, Chile. Data was obtained from clinical records, recorded in electronic spreadsheets and analyzed by descriptive statistics. Results: Seventy four of 83 identified patients fulfilled the inclusion criteria and were analyzed. Mean time to reach the diagnosis was 2.1 years. Cases younger than 10 years presented digestive manifestations such as chronic diarrhea and abdominal distension. Twenty one percent of older patients had atypical presentations (mainly short stature, refractory anaemia). Ten percent of cases were screened because a first degree relative had celiac disease. All patients had significant duodenal/jejunal lesion. IgA-antiendomysial antibodies (n =65) and IgA-antigliadin antibodies (n =23) were the most commonly used screening tests used but often, they were not available for follow up. A second biopsy was planned in all patients but only 26 had it due to repeated dietary transgressions, often due to unnoticed consumption of gluten in poorly labeled products. Conclusions: Digestive manifestations were the main presentation form for celiac disease among patients under 10 years of age. Atypical symptoms become relevant in patients older than 10 years. Antiendomysial and antitransglutaminase antibody measurement should be incorporated for routine screening and follow up of celiac disease in public hospitals. To improve food labeling about their gluten content is needed.
Subject(s)
Adolescent , Child , Child, Preschool , Female , Humans , Male , Celiac Disease/diagnosis , Celiac Disease/diet therapy , Diet, Gluten-Free , Biomarkers/blood , Biopsy , Celiac Disease/blood , Diagnosis, Differential , Feeding Behavior , Gliadin/immunology , Immunoglobulin A/blood , Immunologic Factors/blood , Intestines/pathology , Retrospective Studies , Transglutaminases/immunologyABSTRACT
Background: Celiac patients are at high risk of developing insulin-dependent diabetes mellitus, a condition that has a long pre-diabetic period. During this lapse, anti-islet cell antibodies serve as markers for future disease. This may be related with the duration of the exposure to gluten. Aim: To test the hypothesis that long term adherence to a gluten free diet decreases the frequency of risk markers for insulin dependent diabetes mellitus during adolescence and early adulthood. Patients and methods: 158 celiac patients were classified as: G1, (n=30 patients) studied at the time of diagnosis; G2 (n=97 patients) exposed to gluten as a result of non compliance with the gluten free diet and, G3 (n=31 patients) who had maintained a long term, strict gluten free diet. Isotype IgG anti-islet cell antibodies were detected by indirect immunofluorescence using monkey pancreas; results were reported in Juvenile Diabetes Foundation (JDF) units. Results: Celiac patients exposed to a gluten containing diet had a significantly higher prevalence of anti-islet cell antibodies than those who had been exposed only briefly (p <0.017). In addition, a significantly higher prevalence of anti-islet cell antibodies was observed in those patients whose exposure to gluten was longer than 5 years than in those whose exposure was shorter (p <0.02). Conclusions: Celiac patients long exposed to gluten have a significantly higher prevalence of anti-islet cell antibodies than those exposed for a short period. This fact supports the hypothesis that the development of these antibodies is associated with the length of the exposure to gluten (Rev Méd Chile 2004; 132: 979-84).
Subject(s)
Adolescent , Adult , Male , Humans , Female , Infant, Newborn , Infant , Child, Preschool , Child , Diabetes Mellitus, Type 1 , Celiac Disease/immunology , Glutens/administration & dosage , Glutens/adverse effects , Islets of Langerhans/immunology , Diet , Risk Factors , Time FactorsSubject(s)
Humans , Male , Female , Infant , Child, Preschool , Adolescent , Adult , Middle Aged , Cystic Fibrosis/genetics , Cystic Fibrosis Transmembrane Conductance Regulator , Chlorides , Iontophoresis , Polymerase Chain Reaction , Genotype , Genetic Counseling , Cystic Fibrosis/etiology , Mutation/genetics , Cystic Fibrosis Transmembrane Conductance RegulatorABSTRACT
El síndrome de la úlcera solitaria del recto (SUSR) es una entidad poco frecuente que consiste en una anormalidad recto-anal, causada por un esfuerzo defecatorio excesivo y que tiene características específicas. Se presenta con mayor frecuencia entre la tercera y la cuarta década, pero existen casos publicados en pediatría. Se presentan 5 pacientes (4 varones). Todos tuvieron rectorragia moderada repetida, esfuerzo defecatorio excesivo, pujo, tenesmo y abundantes mucosidades en las deposiciones. Un niño tuvo prolapso rectal completo, lo que fue comprobado endoscópicamente. La defecografía mostró en todos una falta de relajación del músculo puborrectal durante la evacuación. La endoscopía mostró en cuatro pacientes la existencia de una o múltiples úlceras y en el quinto una lesión nodular fibrótica. El examen histopatológico de la mucosa rectal demostró en todos los casos una fibrosis de la "lámina propria", aumento de las fibras musculares lisas con acentuado engrosamiento de la "muscularis mucosae". El tratamiento médico fue suficiente en cuatro niños, pero uno solo alivió sus síntomas después de una sacro-promonto-pexia. Esta enfermedad raramente se diagnostica en niños. Sin embargo, creemos que su búsqueda adecuada permitirá encontrar más pacientes en el futuro. No existe uniformidad en el tratamiento médico, pero generalmente se consigue aliviar la sintomatología. La cirugía debe reservarse para los cuadros clínicos severos, en los cuales el prolapso rectal fue comprobado