[Right postpneumonectomy syndrome: case report]. / Síndrome postneumonectomía derecha: informe de un caso.

Postpneumonectomy syndrome is a rare entity in children. We report a case secondary to pneumonectomy performed due to an intrathoracic tumor. A 4-year-old boy with a history of right pneumonectomy and progressive respiratory distress, with extrinsic airway obstruction due to displaced mediastinal structures. There were failure to intubate the airway with endoluminal devices and impossibility of withdrawing from mechanical ventilation. As mediastinal repositioning was not possible, an aortic bypass with a prosthetic tube graft was performed, with favorable outcome and hospital discharge.

Síndrome postneumonectomía derecha: informe de un caso / Right postpneumonectomy syndrome: Case report

El síndrome postneumonectomía es una entidad poco frecuente en pediatría. Comunicamos un caso secundario a una neumonectomía realizada por un tumor intratorácico. Niño de 4 años con antecedente de exéresis pulmonar derecha y dificultad respiratoria progresiva, con obstrucción extrínseca de la vía aérea por estructuras mediastínicas desplazadas. Hubo intentos frustros de tutorización con dispositivos endoluminales de la vía aérea, e imposibilidad de retiro de la asistencia ventilatoria mecánica. Ante la imposibilidad de reposicionamiento mediastinal, se realiza puente (bypass) aórtico con tubo protésico, con evolución favorable y egreso hospitalario.

Postpneumonectomy syndrome is a rare entity in children. We report a case secondary to pneumonectomy performed due to an intrathoracic tumor. A 4-year-old boy with a history of right pneumonectomy and progressive respiratory distress, with extrinsic airway obstruction due to displaced mediastinal structures. There were failure to intubate the airway with endoluminal devices and impossibility of withdrawing from mechanical ventilation. As mediastinal repositioning was not possible, an aortic bypass with a prosthetic tube graft was performed, with favorable outcome and hospital discharge.

Síndrome postneumonectomía derecha: informe de un caso / Right postpneumonectomy syndrome: Case report

El síndrome postneumonectomía es una entidad poco frecuente en pediatría. Comunicamos un caso secundario a una neumonectomía realizada por un tumor intratorácico. Niño de 4 años con antecedente de exéresis pulmonar derecha y dificultad respiratoria progresiva, con obstrucción extrínseca de la vía aérea por estructuras mediastínicas desplazadas. Hubo intentos frustros de tutorización con dispositivos endoluminales de la vía aérea, e imposibilidad de retiro de la asistencia ventilatoria mecánica. Ante la imposibilidad de reposicionamiento mediastinal, se realiza puente (bypass) aórtico con tubo protésico, con evolución favorable y egreso hospitalario.(AU)

Postpneumonectomy syndrome is a rare entity in children. We report a case secondary to pneumonectomy performed due to an intrathoracic tumor. A 4-year-old boy with a history of right pneumonectomy and progressive respiratory distress, with extrinsic airway obstruction due to displaced mediastinal structures. There were failure to intubate the airway with endoluminal devices and impossibility of withdrawing from mechanical ventilation. As mediastinal repositioning was not possible, an aortic bypass with a prosthetic tube graft was performed, with favorable outcome and hospital discharge.(AU)

[Effectiveness analysis of high-frequency oscillatory ventilation in pediatrics patients with acute respiratory failure in a tertiary hospital]. / Análisis de efectividad de la ventilación de alta frecuencia oscilatoria en pacientes pediátricos con insufciencia respiratoria aguda en un centro de alta complejidad.

Introduction. High frequency oscillatory ventilation (HFOV) is a rescue therapy for hypoxemic patients who deteriorate in conventional mechanical ventilation and/or for the air-leak syndrome treatment. A recent meta analysis showed that HFOV might have reduced mortality in pediatric and adult patients compared with conventional ventilation. In this context it's important to evaluate the effectiveness of this method in everyday use. Objectives. Main: To analize the effectiveness of HFOV in everyday practice in a center without extra corporeal membrane oxygenation (ECMO) capabilities. Secondary: To describe demographics and causes of severe respiratory failure of patients requiring HFOV. To assess the relationship between potential predictors and the occurrence of mortality. Population and methods. Retrospective study analyzing medical records of all patients that required HFOV in a tertiary care pediatric hospital pediatric intensive care units between march 1st 2008 and july 1st 2010. Results. Sixty-nine patients received 76 HFOV treatments. Eighty percent were diagnosed with acute lower respiratory infection or sepsis and 62.3% (n= 43) had preexisting chronic co-morbidities. The majority of HFOV treatments were because refractory hypoxemia (93.4%). Non survivors patients had worse clinical status at PICU admission, higher multiorgan failure, worse oxygenation and pulmonary condition. Conclusions. Everyday use of HFOV in a population with high incidence of chronic, oncologic and/or immunocompromised patients was associated with a survival of 33.4%. More prognostic studies are needed for a more effective selection of HFOV candidates.

Análisis de efectividad de la ventilación de alta frecuencia oscilatoria en pacientes pediátricos con insufciencia respiratoria aguda en un centro de alta complejidad / Effectiveness analysis of high-frequency oscillatory ventilation in pediatrics patients with acute respiratory failure in a tertiary hospital

Introducción. La ventilación de alta frecuencia oscilatoria (VAFO) es un recurso terapéutico ante la hipoxemia refractaria a la asistencia respiratoria mecánica convencional (ARMc), y en el tratamiento del síndrome de escape aéreo. Un metaanálisis reciente concluyó que la VAFO parece disminuir la mortalidad en niños posneonatales y adultos. En este contexto, es importante la evaluación de los resultados en el uso rutinario de esta tecnología. Objetivos. Principal: Analizar la efectividad de la VAFO en la práctica rutinaria en un centro que no dispone de oxigenación por membrana de circulación extracorpórea. Secundarios: Describir los datos demográfcos y las causas de insufciencia respiratoria grave de los pacientes que requirieron VAFO. Examinar la relación entre factores predictores potenciales y la ocurrencia de mortalidad. Población y métodos. Estudio retrospectivo en el cual se analizaron las historias clínicas de los pacientes que requirieron VAFO en las Unidades de Cuidados Intensivos de un hospital pediátrico terciario en el período 01/01/2008 - 01/07/2010. Resultados. Se analizaron 76 ingresos a VAFO en 69 pacientes. El 80% de tales ingresos tenían diagnóstico de infección respiratoria aguda baja o sepsis. El 62,3% (n= 43) de los casos presentaban enfermedad crónica subyacente. La mayoría de los ingresos fueron por hipoxemia refractaria (93,4%). Se halló que los pacientes fallecidos presentaban peores condiciones clínicas al ingreso, mayor compromiso multiorgánico, peor oxigenación y compromiso pulmonar. Conclusión. En una población con alta prevalencia de enfermos crónicos, oncológicos e inmunodeprimidos, el uso rutinario de la VAFO se asoció con un 33,4% de supervivencia. Se necesitan más estudios pronósticos que permitan una selección más efcaz de los pacientes candidatos a esta tecnología.

Introduction. High frequency oscillatory ventilation (HFOV) is a rescue therapy for hypoxemic patients who deteriorate in conventional mechanical ventilation and/or for the air-leak syndrome treatment. A recent meta analysis showed that HFOV might have reduced mortality in pediatric and adult patients compared with conventional ventilation. In this context it's important to evaluate the effectiveness of this method in everyday use. Objectives. Main: To analize the effectiveness of HFOV in everyday practice in a center without extra corporeal membrane oxygenation (ECMO) capabilities. Secondary: To describe demographics and causes of severe respiratory failure of patients requiring HFOV. To assess the relationship between potential predictors and the occurrence of mortality. Population and methods. Retrospective study analyzing medical records of all patients that required HFOV in a tertiary care pediatric hospital pediatric intensive care units between march 1st 2008 and july 1st 2010. Results. Sixty-nine patients received 76 HFOV treatments. Eighty percent were diagnosed with acute lower respiratory infection or sepsis and 62.3% (n= 43) had preexisting chronic co-morbidities. The majority of HFOV treatments were because refractory hypoxemia (93.4%). Non survivors patients had worse clinical status at PICU admission, higher multiorgan failure, worse oxygenation and pulmonary condition. Conclusions. Everyday use of HFOV in a population with high incidence of chronic, oncologic and/or immunocompromised patients was associated with a survival of 33.4%. More prognostic studies are needed for a more effective selection of HFOV candidates.

Análisis de efectividad de la ventilación de alta frecuencia oscilatoria en pacientes pediátricos con insufciencia respiratoria aguda en un centro de alta complejidad / Effectiveness analysis of high-frequency oscillatory ventilation in pediatrics patients with acute respiratory failure in a tertiary hospital

Introducción. La ventilación de alta frecuencia oscilatoria (VAFO) es un recurso terapéutico ante la hipoxemia refractaria a la asistencia respiratoria mecánica convencional (ARMc), y en el tratamiento del síndrome de escape aéreo. Un metaanálisis reciente concluyó que la VAFO parece disminuir la mortalidad en niños posneonatales y adultos. En este contexto, es importante la evaluación de los resultados en el uso rutinario de esta tecnología. Objetivos. Principal: Analizar la efectividad de la VAFO en la práctica rutinaria en un centro que no dispone de oxigenación por membrana de circulación extracorpórea. Secundarios: Describir los datos demográfcos y las causas de insufciencia respiratoria grave de los pacientes que requirieron VAFO. Examinar la relación entre factores predictores potenciales y la ocurrencia de mortalidad. Población y métodos. Estudio retrospectivo en el cual se analizaron las historias clínicas de los pacientes que requirieron VAFO en las Unidades de Cuidados Intensivos de un hospital pediátrico terciario en el período 01/01/2008 - 01/07/2010. Resultados. Se analizaron 76 ingresos a VAFO en 69 pacientes. El 80% de tales ingresos tenían diagnóstico de infección respiratoria aguda baja o sepsis. El 62,3% (n= 43) de los casos presentaban enfermedad crónica subyacente. La mayoría de los ingresos fueron por hipoxemia refractaria (93,4%). Se halló que los pacientes fallecidos presentaban peores condiciones clínicas al ingreso, mayor compromiso multiorgánico, peor oxigenación y compromiso pulmonar. Conclusión. En una población con alta prevalencia de enfermos crónicos, oncológicos e inmunodeprimidos, el uso rutinario de la VAFO se asoció con un 33,4% de supervivencia. Se necesitan más estudios pronósticos que permitan una selección más efcaz de los pacientes candidatos a esta tecnología.(AU)

Introduction. High frequency oscillatory ventilation (HFOV) is a rescue therapy for hypoxemic patients who deteriorate in conventional mechanical ventilation and/or for the air-leak syndrome treatment. A recent meta analysis showed that HFOV might have reduced mortality in pediatric and adult patients compared with conventional ventilation. In this context its important to evaluate the effectiveness of this method in everyday use. Objectives. Main: To analize the effectiveness of HFOV in everyday practice in a center without extra corporeal membrane oxygenation (ECMO) capabilities. Secondary: To describe demographics and causes of severe respiratory failure of patients requiring HFOV. To assess the relationship between potential predictors and the occurrence of mortality. Population and methods. Retrospective study analyzing medical records of all patients that required HFOV in a tertiary care pediatric hospital pediatric intensive care units between march 1st 2008 and july 1st 2010. Results. Sixty-nine patients received 76 HFOV treatments. Eighty percent were diagnosed with acute lower respiratory infection or sepsis and 62.3% (n= 43) had preexisting chronic co-morbidities. The majority of HFOV treatments were because refractory hypoxemia (93.4%). Non survivors patients had worse clinical status at PICU admission, higher multiorgan failure, worse oxygenation and pulmonary condition. Conclusions. Everyday use of HFOV in a population with high incidence of chronic, oncologic and/or immunocompromised patients was associated with a survival of 33.4%. More prognostic studies are needed for a more effective selection of HFOV candidates.(AU)

Análisis de efectividad de la ventilación de alta frecuencia oscilatoria en pacientes pediátricos con insufciencia respiratoria aguda en un centro de alta complejidad / Effectiveness analysis of high-frequency oscillatory ventilation in pediatrics patients with acute respiratory failure in a tertiary hospital

Introducción. La ventilación de alta frecuencia oscilatoria (VAFO) es un recurso terapéutico ante la hipoxemia refractaria a la asistencia respiratoria mecánica convencional (ARMc), y en el tratamiento del síndrome de escape aéreo. Un metaanálisis reciente concluyó que la VAFO parece disminuir la mortalidad en niños posneonatales y adultos. En este contexto, es importante la evaluación de los resultados en el uso rutinario de esta tecnología. Objetivos. Principal: Analizar la efectividad de la VAFO en la práctica rutinaria en un centro que no dispone de oxigenación por membrana de circulación extracorpórea. Secundarios: Describir los datos demográfcos y las causas de insufciencia respiratoria grave de los pacientes que requirieron VAFO. Examinar la relación entre factores predictores potenciales y la ocurrencia de mortalidad. Población y métodos. Estudio retrospectivo en el cual se analizaron las historias clínicas de los pacientes que requirieron VAFO en las Unidades de Cuidados Intensivos de un hospital pediátrico terciario en el período 01/01/2008 - 01/07/2010. Resultados. Se analizaron 76 ingresos a VAFO en 69 pacientes. El 80% de tales ingresos tenían diagnóstico de infección respiratoria aguda baja o sepsis. El 62,3% (n= 43) de los casos presentaban enfermedad crónica subyacente. La mayoría de los ingresos fueron por hipoxemia refractaria (93,4%). Se halló que los pacientes fallecidos presentaban peores condiciones clínicas al ingreso, mayor compromiso multiorgánico, peor oxigenación y compromiso pulmonar. Conclusión. En una población con alta prevalencia de enfermos crónicos, oncológicos e inmunodeprimidos, el uso rutinario de la VAFO se asoció con un 33,4% de supervivencia. Se necesitan más estudios pronósticos que permitan una selección más efcaz de los pacientes candidatos a esta tecnología.(AU)

Introduction. High frequency oscillatory ventilation (HFOV) is a rescue therapy for hypoxemic patients who deteriorate in conventional mechanical ventilation and/or for the air-leak syndrome treatment. A recent meta analysis showed that HFOV might have reduced mortality in pediatric and adult patients compared with conventional ventilation. In this context its important to evaluate the effectiveness of this method in everyday use. Objectives. Main: To analize the effectiveness of HFOV in everyday practice in a center without extra corporeal membrane oxygenation (ECMO) capabilities. Secondary: To describe demographics and causes of severe respiratory failure of patients requiring HFOV. To assess the relationship between potential predictors and the occurrence of mortality. Population and methods. Retrospective study analyzing medical records of all patients that required HFOV in a tertiary care pediatric hospital pediatric intensive care units between march 1st 2008 and july 1st 2010. Results. Sixty-nine patients received 76 HFOV treatments. Eighty percent were diagnosed with acute lower respiratory infection or sepsis and 62.3% (n= 43) had preexisting chronic co-morbidities. The majority of HFOV treatments were because refractory hypoxemia (93.4%). Non survivors patients had worse clinical status at PICU admission, higher multiorgan failure, worse oxygenation and pulmonary condition. Conclusions. Everyday use of HFOV in a population with high incidence of chronic, oncologic and/or immunocompromised patients was associated with a survival of 33.4%. More prognostic studies are needed for a more effective selection of HFOV candidates.(AU)

Influenza A (pH1N1) infection in children admitted to a pediatric intensive care unit: differences with other respiratory viruses.

OBJECTIVES: To describe the clinical features of patients admitted to the pediatric intensive care unit (PICU) with acute lower respiratory tract infection (LRTI) attributable to influenza A pH1N1 virus and compare them with those admitted with LRTI due to other viral pathogens. DESIGN: Retrospective, observational, comparative study. SETTING: PICU in a university-affiliated, tertiary-care, pediatric hospital. PATIENTS: Patients aged >1 month with acute viral LRTI admitted to the PICU who met the following criteria: 1) influenza A pH1N1 virus infection detected between June 1, 2009 and July 16, 2009; and 2) patients with LRTI due to other viral pathogens infection detected in the same period of the year 2008 plus patients with 2009 influenza A non-pH1N1 infection. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We evaluated age, weight, gender, length of hospital stay before PICU admission, length of PICU stay, underlying disease, clinical diagnosis, severity, complications, treatment, risk of death, mortality, and cause of death. We identified 30 patients with acute LRTI due to influenza A pH1N1 virus (group 1) and 62 patients with LRTI due to other viral pathogens (group 2). Relevant characteristics in group 1: 59% of patients were aged >2 yrs. Median length of PICU stay was 15 days. An underlying condition was present in 83% of the patients. None of them had bronchiolitis on PICU admission; 12 (40%) had bilateral pneumonia; 15 (50%) had acute respiratory distress syndrome; and nine (30%) had shock. Twenty-nine patients required mechanical ventilation (96.6%); only one required extracorporeal membrane oxygenation; and 19 (63%) had respiratory complications. Six (20%) children died. When both groups were compared, patients with influenza A pH1N1 infection were older; pneumonia, acute respiratory distress syndrome, and shock were more frequently seen at the time of PICU admission, although length of stay and mortality were similar. CONCLUSIONS: Patients with influenza A pH1N1 respiratory virus infection presented with more severe illness. Outcomes were similar in both groups.

Clinical implications of hepatitis A virus ribonucleic acid detection and genotyping in acute liver failure in children in Argentina.

OBJECTIVES: To investigate the detection of hepatitis A virus ribonucleic acid in patients with acute liver failure and to assess if the results have any clinical implications for the evolution of acute liver failure in children. Hepatitis A infection, a vaccine-preventable disease, is an important cause of acute liver failure in children in Argentina. Universal vaccination in 1-yr-old children was implemented in June 2005. DESIGN: Observational study in which patients were divided into Group 1 consisting of positive hepatitis A virus ribonucleic acid and Group 2 consisting of negative hepatitis A virus ribonucleic acid. SETTING: Pediatric intensive care unit in National Pediatric Hospital "Dr. J. P. Garrahan," Buenos Aires, Argentina. PATIENTS: Thirty-three patients with the diagnosis of acute liver failure secondary to hepatitis A virus infection and admitted to the Garrahan Pediatric Hospital between September 2003 and September 2005 were enrolled in the study. Twenty of these children were admitted to the pediatric intensive care unit. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Samples for total ribonucleic acid detection and genotyping were obtained from serum and/or stools on admission. We found positive hepatitis A virus ribonucleic acid in 13 patients and negative hepatitis A virus ribonucleic acid in 20 patients. The following clinical variables were evaluated: time of evolution, hospital stay, admission to the pediatric intensive care unit, pediatric intensive care unit stay, time on mechanical ventilation, criteria for orthotopic liver transplantation, and mortality. Characterization of the isolates did not reveal differences related to genotype; all cases were IA. No statistical significance was found as to the variables. However, positive hepatitis A virus ribonucleic acid showed lower percentages of pediatric intensive care unit admissions, criteria for orthotopic liver transplantation, number of orthotopic liver transplantation, and mortality than the group of patients with negative hepatitis A virus ribonucleic acid. CONCLUSIONS: Hepatitis A virus genotyping studies did not show any particularities, all cases were IA and, thus, apparent associations between genotype and the clinical presentation of acute liver failure could not be found.

Molecular characterization of hepatitis A virus in children with fulminant hepatic failure in Argentina.

BACKGROUND: Hepatitis A infection, a vaccine-preventable disease, is an important cause of fulminant hepatic failure (FHF) in children in Argentina. Universal vaccination in 1-year-old children was implemented in June 2005. The limited studies about the correlation between the characteristics of the hepatitis A virus (HAV) and FHF have been carried out in adults. METHODS: Samples from 41 children with FHF were studied from September 2003 to January 2006 and HAV RNA was detected, sequenced and analysed in the 5' non-coding region and VP1/2A region. RESULTS: Eighteen HAV strains were characterized and found to be different at the nucleotide level from the self-limited acute infection strains that have been circulating in Argentina with no temporal or geographical pattern. They did not form a genetic cluster, but some of them were identical in the largest fragment characterized and some of them seemed to be more closely related in time and/or geographically. CONCLUSION: Our results suggest that viral factors could be involved in the severity of the clinical presentation of HAV infection in children in Argentina.

[Molecular characterization of hepatitis E virus in three acute liver failure cases in children in Argentina]. / Caracterización molecular del virus de hepatitis E en tres casos de falla hepática fulminante en niños de Argentina.

Strains of hepatitis E virus (HEV) isolated from Argentinian patients with sporadic hepatitis, as well as from swine from Argentina, belong to genotype 3. HEV genotype 3 variants have been described associated with acute liver failure (ALF) in adults from Japan and the United Kingdom. In Argentina, 30% of ALF in adults and children are of unknown aetiology. To study if HEV could be an aetiological agent associated with ALF in children, serum and/or fecal samples fJom 35 children (mean age: 6 years, 20 female, 15 male) were analyzed during 2003 and 2004. HEV RNA was detected by RT-nested PCR with primers designed within ORF 1 and ORF 2 regions. HEV RNA could be detected in three cases. Two were 12-year-old boys fom Buenos Aires province and the third was a 3-year-old girl from Corrientes province. Sequence analysis indicates that the three isolates are distinct from each other but all belong to genotype 3, exhibiting a close relationship with swine and human strains fJom sporadic cases of HEV, previously reported in Argentina. This data suggests a potential link between ALF and HEVin children in Argentina and indicates the need for the determination of HEV status in the differential diagnosis in ALE Further studies would aid in determining the true impact of this infection in Argentina and the potential benefits of a vaccine against HEV presently in phase III trials.

Caracterización molecular del virus de hepatitis E en tres casos de falla hepática fulminante en niños de Argentina / Molecular characterization of hepatitis E virus in three acute liver failure cases in children in Argentina

Las cepas de virus de hepatitis E (HEV) encontradas en casos esporádicos humanos y en cerdos en Argentina corresponden al genotipo 3. Se han descripto variantes de este genotipo asociadas a fallas hepáticas fulminantes (FHF) en adultos de Japón e Inglaterra. En Argentina el 30% de las FHF en adultos y en niños es de etiología desconocida. Para estudiar si el HEV podría ser el agente etiológico asociado a FHF en niños se analizaron el suero y/o la materia fecal de 35 niños (edad media 6 años, 20 mujeres, 15 varones) durante 2003 y 2004. El HEV RNA fue detectado por RT-nested PCR con cebadores dirigidos a las regiones ORF 1 y ORF 2. El HEV RNA pudo detectarse en 3 casos. Dos eran varones de 12 años residentes en la provincia de Buenos Aires y el tercero, una niña de 3 años de la provincia de Corrientes. El análisis de las secuencias muestra que las 3 variantes son distintas, pero pertenecen todas al genotipo 3 y están muy relacionadas a las cepas encontradas previamente en casos esporádicos en humanos y en cerdos de Argentina. Estos datos sugieren una posible relación entre FHF y HEV en niños de Argentina e indican la necesidad de considerar la infección con HEV en el diagnóstico diferencial de las FHF. Se necesitan más estudios que demuestren el verdadero impacto de esta infección y el beneficio potencial de na vacuna para HEV, actualmente en fase III.

Strains of hepatitis E virus (HEV) isolated from Argentinian patients with sporadic hepatitis, as well as from swine from Argentina, belong to genotype 3. HEV genotype 3 variants have been described associated with acute liver failure (ALF) in adults from Japan and the United Kingdom. In Argentina, 30% of ALF in adults and children are of unknown aetiology. To study if HEV could be an aetiological agent associated with ALF in children, serum and/or fecal samples from 35 children (mean age: 6 years, 20 female, 15 male) were analyzed during 2003 and 2004. HEV RNA was detected by RT-nested PCR with primers designed within ORF 1 and ORF 2 regions. HEV RNA could be detected in three cases. Two were 12-year-old boys from Buenos Aires province and the third was a 3- year-old girl from Corrientes province. Sequence analysis indicates that the three isolates are distinct from each other but all belong to genotype 3, exhibiting a close relationship with swine and human strains from sporadic cases of HEV, previously reported in Argentina. This data suggests a potential link between ALF and HEV in children in Argentina and indicates the need for the determination of HEV status in the differential diagnosis in ALF. Further studies would aid in determining the true impact of this infection in Argentina and the potential benefits of a vaccine against HEV, presently in phase III trials.

Caracterización molecular del virus de hepatitis E en tres casos de falla hepática fulminante en niños de Argentina / Molecular characterization of hepatitis E virus in three acute liver failure cases in children in Argentina

Las cepas de virus de hepatitis E (HEV) encontradas en casos esporádicos humanos y en cerdos en Argentina corresponden al genotipo 3. Se han descripto variantes de este genotipo asociadas a fallas hepáticas fulminantes (FHF) en adultos de Japón e Inglaterra. En Argentina el 30% de las FHF en adultos y en niños es de etiología desconocida. Para estudiar si el HEV podría ser el agente etiológico asociado a FHF en niños se analizaron el suero y/o la materia fecal de 35 niños (edad media 6 años, 20 mujeres, 15 varones) durante 2003 y 2004. El HEV RNA fue detectado por RT-nested PCR con cebadores dirigidos a las regiones ORF 1 y ORF 2. El HEV RNA pudo detectarse en 3 casos. Dos eran varones de 12 años residentes en la provincia de Buenos Aires y el tercero, una niña de 3 años de la provincia de Corrientes. El análisis de las secuencias muestra que las 3 variantes son distintas, pero pertenecen todas al genotipo 3 y están muy relacionadas a las cepas encontradas previamente en casos esporádicos en humanos y en cerdos de Argentina. Estos datos sugieren una posible relación entre FHF y HEV en niños de Argentina e indican la necesidad de considerar la infección con HEV en el diagnóstico diferencial de las FHF. Se necesitan más estudios que demuestren el verdadero impacto de esta infección y el beneficio potencial de na vacuna para HEV, actualmente en fase III.(AU)

Strains of hepatitis E virus (HEV) isolated from Argentinian patients with sporadic hepatitis, as well as from swine from Argentina, belong to genotype 3. HEV genotype 3 variants have been described associated with acute liver failure (ALF) in adults from Japan and the United Kingdom. In Argentina, 30% of ALF in adults and children are of unknown aetiology. To study if HEV could be an aetiological agent associated with ALF in children, serum and/or fecal samples from 35 children (mean age: 6 years, 20 female, 15 male) were analyzed during 2003 and 2004. HEV RNA was detected by RT-nested PCR with primers designed within ORF 1 and ORF 2 regions. HEV RNA could be detected in three cases. Two were 12-year-old boys from Buenos Aires province and the third was a 3- year-old girl from Corrientes province. Sequence analysis indicates that the three isolates are distinct from each other but all belong to genotype 3, exhibiting a close relationship with swine and human strains from sporadic cases of HEV, previously reported in Argentina. This data suggests a potential link between ALF and HEV in children in Argentina and indicates the need for the determination of HEV status in the differential diagnosis in ALF. Further studies would aid in determining the true impact of this infection in Argentina and the potential benefits of a vaccine against HEV, presently in phase III trials.(AU)

[Evolution of children one year post liver transplant]. / Evolución de niños post-trasplante hepático luego del primer año de sobrevida.

Orthotopic liver transplantation is the only definitive mode of therapy for children with end-stage liver disease. However, it remains challenging because of the necessity to prevent long-term complications. The aim of this study was to analyze the evolution of transplanted patients with more than one year of follow up. Between November 1992 and November 2001, 238 patients underwent 264 liver transplantations. A total of 143 patients with more than one year of follow up were included. The median age of patients +/- SD was 5.41 years +/- 5.26 (r: 0.58-21.7 years). All children received primary immunosuppression with cyclosporine. The indications for liver replacement were: fulminant hepatic failure (n: 50), biliary atresia (n: 38), cirrhosis (n: 37), chronic cholestasis (n: 13) and miscellaneous (n: 5). The indications for liver re-transplantation were: biliary cirrhosis (n: 7), hepatic artery thrombosis (n: 4) and chronic rejection (n: 3). Reduced-size liver allografts were used in 73/157 liver transplants, 14 of them were from living-related donors and 11 were split-livers. Patient and graft survival rates were 93% and 86% respectively. Death risk was statistically higher in retransplanted and reduced-size grafted patients. Growth retardation and low bone density were recovered before the first 3 years post-transplant. The incidence of lymphoproliferative disease was 7.69%. De novo hepatitis B was diagnosed in 7 patients (4.8%). Social risk did not affect the outcome of our population. The prevention, detection and early treatment of complications in the long-term follow up contributed to improve the outcome.

Liver support for fulminant hepatic failure: is it time to use the molecular adsorbents recycling system in children?

OBJECTIVE: To describe the main liver support devices used for fulminant hepatic failure (FHF) and to review data on the Molecular Adsorbents Recycling System (MARS) and assess its efficiency in children. DATA SOURCE: Studies were identified through selected readings and a MEDLINE search from 1975 and 2004 using fulminant hepatic failure, acute liver failure, primary graft dysfunction, liver support, MARS, and extracorporeal liver assist device as key words. STUDY SELECTION: All original studies, including case reports, relating to the use of the MARS or albumin dialysis system were included. Additional attention was put on prognosis criteria of FHF severity in children. DATA EXTRACTION: Study design, numbers and diagnoses of patients, definite or bridging treatment, outcome measures, and complications were extracted and compiled. Results of individual trials were combined on the risk ratio scale. DATA SYNTHESIS: Nine randomized trials including 354 patients were identified. However, liver support failed to significantly affect mortality when compared with standard medical therapy. Albumin dialysis, and particularly MARS, emerges as an easily applicable technique for temporary liver support. Some well-designed studies have characterized its efficiency in a few indications, such as in intractable pruritus in chronic liver disease, in acute or chronic liver diseases, and in decompensated cirrhosis with hepatorenal syndrome. In adults and children with FHF, anecdotal reports suggest that MARS may stabilize the patient. However, no randomized controlled study has validated its use in this indication. A randomized controlled study is ongoing in adults with FHF. Such a trial seems to be unfeasible in children for several methodologic reasons. CONCLUSIONS: Although promising preliminary results suggest that MARS may have a significant position in the therapeutic arsenal for FHF, no sufficient data exist to justify its use in children. For as long as the results of the ongoing adult trial are not available, the indications of this expensive technique in children with FHF are limited.

Evolución de niños post-trasplante hepático luego del primer año de sobrevida. / [Evolution of children one year post liver transplant]

Orthotopic liver transplantation is the only definitive mode of therapy for children with end-stage liver disease. However, it remains challenging because of the necessity to prevent long-term complications. The aim of this study was to analyze the evolution of transplanted patients with more than one year of follow up. Between November 1992 and November 2001, 238 patients underwent 264 liver transplantations. A total of 143 patients with more than one year of follow up were included. The median age of patients +/- SD was 5.41 years +/- 5.26 (r: 0.58-21.7 years). All children received primary immunosuppression with cyclosporine. The indications for liver replacement were: fulminant hepatic failure (n: 50), biliary atresia (n: 38), cirrhosis (n: 37), chronic cholestasis (n: 13) and miscellaneous (n: 5). The indications for liver re-transplantation were: biliary cirrhosis (n: 7), hepatic artery thrombosis (n: 4) and chronic rejection (n: 3). Reduced-size liver allografts were used in 73/157 liver transplants, 14 of them were from living-related donors and 11 were split-livers. Patient and graft survival rates were 93

and 86

respectively. Death risk was statistically higher in retransplanted and reduced-size grafted patients. Growth retardation and low bone density were recovered before the first 3 years post-transplant. The incidence of lymphoproliferative disease was 7.69

. De novo hepatitis B was diagnosed in 7 patients (4.8

). Social risk did not affect the outcome of our population. The prevention, detection and early treatment of complications in the long-term follow up contributed to improve the outcome.