Post-Transplant Lymphoproliferative Disorder (PTLD) is a life threatening complication in organ t... more Post-Transplant Lymphoproliferative Disorder (PTLD) is a life threatening complication in organ transplant recipients. Risk factors include primary Epstein-Barr virus infection, intensity of immunosupression and cytomegalovirus infection. To evaluate the incidence, clinical presentation, risk factors, histopathologic appearance and outcome of pediatric liver recipients with PTLD at our institution. Retrospective, descriptive and observational analysis. Between November 1992 and December 2005, 383 liver transplants were performed. The diagnosis of PTLD was based on clinical history and physical examination and confirmed by histologic appearance and immunohistologic staining. Knowles' classification was used for histopathologic diagnosis. The incidence of PTLD was 5.7% (n: 22p). The average onset after tansplantation (OLT) was 24.9 months. Clinical manifestations were malaise, anorexia, fever of more than 3 days, peripheral adenopathy, tonsillar hypertrophy, abdominal mass, hepatosplenomegaly, snoring, interstitial pulmonary infiltrate, G.T.-tract bleeding, rash, submaxilar mass. Histopathologic diagnosis were Plasmocytic Hyperplasia (n: 10), Polymorphic Lymphoproliferative Disorder (n: 8), Non-Hodgkin Lymphoma (n: 4). Mortality was 18%. The clinical presentations were protean and not specific. A high index of suspicion is important for early diagnosis as it correlates with more benign lesions and more favorable outcume. The lower mortality rate in our series is concordant with that reported in more recent articles.
Invasive fungal infections are a significant cause of morbidity and mortality in children. Caspof... more Invasive fungal infections are a significant cause of morbidity and mortality in children. Caspofungin is an echinocandin used as an alternative treatment in the prevention and/or treatment of certain invasive fungal infections in children, although compared to the standard treatment there is little evidence on its efficacy and safety. Objectives. To evaluate the efficacy and safety of caspofungin compared with other antifungal drugs for the prevention and/or treatment of invasive fungal infections in children. Material and methods. The objective of the initial search strategy was to identify randomized controlled studies of acceptable methodological quality (Jadad scale >3), through the key word "caspofungin", conducted in patients with an age range from 0 to 18 years old. Results. Only 3 publications met the inclusion criteria. Two of them were studies conducted in children and one in newborn infants. A higher incidence of adverse events was not documented for caspofungin and its efficacy was not different from that of other antifungal drugs (typical RR 1.47; CI 95%: 0.78-2.79). Conclusions. This systematic review suggests that caspofungin could be considered as an alternative drug in children for the prevention and treatment of invasive fungal infections. However, given the small number of existing publications, more studies are required to reach definite conclusions.
Resumo: La aparición de ascitis es una complicación importante en la evolución de la enfermedad h... more Resumo: La aparición de ascitis es una complicación importante en la evolución de la enfermedad hepática crónica y ha sido propuesta como un indicador para evaluar la necesidad de transplante hepático. Su tratamiento consiste en dieta hiposódica estricta y ...
Eficacia y seguridad de la caspofungina en pediatría: revisión sistemática y metaanálisis Efficac... more Eficacia y seguridad de la caspofungina en pediatría: revisión sistemática y metaanálisis Efficacy and safety of caspofungin in children: systematic review and meta-analysis a.
Voriconazole is a second-generation triazole. It has excellent bioavailability and broad antifung... more Voriconazole is a second-generation triazole. It has excellent bioavailability and broad antifungal spectrum; thus, it is an attractive option for patients at high risk of invasive fungal infections (IFIs). Comparing efficacy and safety of voriconazole with other antifungals in prophylaxis or treatment of IFIs would be useful to draw conclusions regarding prevention and therapeutics of these infections. To assess efficacy and safety of voriconazole compared with other options as prophylaxis or treatment of IFIs in haematology-oncology patients. A literature search was performed in MEDLINE database using the search term 'voriconazole' and completed with manual search. Randomized controlled trials (RCTs) comparing voriconazole with other antifungal agents or placebo. Seven studies fulfilled the eligibility criteria. Five studies compared voriconazole to another comparator as prophylaxis of IFIs and two as treatment. Pooled results showed that voriconazole was more effective than the comparator (RR = 1.17; 95%CI = 1.01-1.34), but heterogeneity was significant (Q test 32.7; p = .00001). Sub-analysis according to prophylaxis showed RR = 1.17; 95%CI = 1.00-1.37; while as treatment, RR = 1.23; 95%CI = 0.68-2.22. Risk of adverse events was not different from that observed for the comparator (RR = 1.06, 95%CI = 0.66-1.72) though significant heterogeneity was detected (p < .01). Voriconazole was as effective and safe as comparators, probably better as prophylaxis than as treatment, but limitations due to variability in the sample size of studies, differences in the age of patients, and heterogeneity between studies' outcome measures indicate the need for further research.
Manejo de la ascitis secundaria a cirrosis en el paciente pediátrico. Revisión y recomendaciones ... more Manejo de la ascitis secundaria a cirrosis en el paciente pediátrico. Revisión y recomendaciones Management of cirrhotic ascites in children. Review and recommendations
El sodio es el cation con mayor concentracion y el soluto mas importante determinante de la osmol... more El sodio es el cation con mayor concentracion y el soluto mas importante determinante de la osmolaridad y el volumen del compartimiento extracelular. La hiponatremia se presenta cuando hay un exceso de agua relativa al sodio. Esta situacion ocurre cuando se produce una mayor perdida de sodio que de agua, cuando hay un incremento del agua corporal total o cuando hay un aumento de ambos, siendo mayor el aumento de agua corporal total. La hiponatremia es importante porque puede provocar dano neurologico severo y muerte. A su vez, su tratamiento puede estar asociado a morbilidad del sistema nervioso central y muerte. Esta alteracion hidroelectrolitica debe ser diagnosticada y tratada en el contexto del nino que la presenta y no como un desequilibrio de la natremia aislado. La evaluacion inicial de la hiponatremia en un paciente debe tener en cuenta la valoracion clinica del compartimiento extracelular y la excrecion renal de sodio. El tratamiento dependera de la presencia de compromiso neurologico en el paciente, del tiempo de evolucion y de los niveles sericos de sodio. No hay consenso aun sobre cual es la mejor solucion hidroelectrolitica de mantenimiento endovenoso en el paciente hospitalizado. Sin embargo se sugiere cautela con la administracion de aportes altos, en especial si las soluciones son hipotonicas. Una adecuada comprension del metabolismo hidrosalino puede ayudar al pediatra a prevenir la hiponatremia, a estar alerta de signos incipientes cuendo esta ocurre y tambien sera beneficioso para un tratamiento apropiado de la misma
There are few cases reported of autoimmune hepatitis (AIH) type 2 presenting as fulminant hepatic... more There are few cases reported of autoimmune hepatitis (AIH) type 2 presenting as fulminant hepatic failure (FHF) in children. The purpose of this study was to report three girls with AIH type 2 that presented as FHF. Over a period of 12 years, 123 patients with AIH diagnosed based on international criteria, 9 (7%) were type 2.3 of them presented as FHF. Other etiologies (viral, metabolic and toxic) were ruled out. The treatment was started with prednisone (2 mg-kg-day) and azathioprine (2 mg-kg-day). Patients 1 and 3 died while waiting for liver transplant (LT) at 72 and 48 hours after initiating medical treatment. Patient 2 underwent LT3 days after starting treatment, with excellent evolution at 3 years and 7 months of follow up. 1--AIH type 2 was very infrequent in our group. 2--33% of cases had initial presentation as FHF. 3--The course of the disease was aggressive, not responding to immunosuppressive therapy. The evolution was unfavorable in all patients. 4--LT is an alternative...
Chronic hyponatremia, unless associated with extracellular fluid volume expansion, is an infreque... more Chronic hyponatremia, unless associated with extracellular fluid volume expansion, is an infrequent electrolyte imbalance in pediatrics. We report an infant with chronic hyponatremia suggestive of a syndrome of inappropriate secretion of antidiuretic hormone (SIADH), in the absence of ADH secretion. A mutation was found in the same codon of the gene that results in a loss-offunction of arginine vasopressin receptor 2 (AVPR2) observed in congenital nephrogenic diabetes insipidus. In this case, a gain-of-function of AVPR 2 was found to be responsible for a SIADH-like state. Keywords Seizure. Syndrome of inappropriate secretion of antidiuretic hormone. AVPR2. Water excretion. Nephrogenic syndrome of inappropriate antidiuresis. Hypo-osmolarity syndrome of inappropriate antidiuresis
Background: The aim of this study was to assess whether daptomycin is safer and more efficacious ... more Background: The aim of this study was to assess whether daptomycin is safer and more efficacious than comparators for the treatment of serious infection caused by gram-positive microorganisms. Methods: Electronic databases (Medline, EMBASE, the Cochrane Central Register of Controlled Trials and clinical registered trials) were searched to identify randomized controlled trials (RCTs) that assessed the efficacy and safety of daptomycin versus therapy with any other antibiotic comparator. Two reviewers independently applied selection criteria, performed a quality assessment and extracted the data. Heterogeneity was assessed, and a random-effects or fixed-effects model, when appropriate, was used for estimates of risk ratio (RR). The primary outcome assessed was the risk of clinical treatment failure among the intention-to-treat population and the presence of any treatment related adverse event (AEs). Results: A total of seven trials fulfilled the inclusion criteria. Daptomycin treatmen...
Voriconazole is a second-generation triazole. It has excellent bioavailability and broad antifung... more Voriconazole is a second-generation triazole. It has excellent bioavailability and broad antifungal spectrum; thus, it is an attractive option for patients at high risk of invasive fungal infections (IFIs). Comparing efficacy and safety of voriconazole with other antifungals in prophylaxis or treatment of IFIs would be useful to draw conclusions regarding prevention and therapeutics of these infections. To assess efficacy and safety of voriconazole compared with other options as prophylaxis or treatment of IFIs in haematology-oncology patients. A literature search was performed in MEDLINE database using the search term 'voriconazole' and completed with manual search. Randomized controlled trials (RCTs) comparing voriconazole with other antifungal agents or placebo. Seven studies fulfilled the eligibility criteria. Five studies compared voriconazole to another comparator as prophylaxis of IFIs and two as treatment. Pooled results showed that voriconazole was more effective th...
Ascites is a major complication of cirrhosis. There are several evidence-based articles and guide... more Ascites is a major complication of cirrhosis. There are several evidence-based articles and guidelines for the management of adults, but few data have been published in relation to children. In the case of pediatric patients with cirrhotic ascites (PPCA), the following questions are raised: How are the clinical assessment and ancillary tests performed? When is ascites considered refractory? How is it treated? Should fresh plasma and platelets be infused before abdominal paracentesis to prevent bleeding? What are the hospitalization criteria? What are the indicated treatments? What complications can patients develop? When and how should hyponatremia be treated? What are the diagnostic criteria for spontaneous bacterial peritonitis? How is it treated? What is hepatorenal syndrome? How is it treated? When should albumin be infused? When should fluid intake be restricted? The recommendations made here are based on pathophysiology and suggest the preferred approach to its diagnostic and ...
Invasive fungal infections are a significant cause of morbidity and mortality in children. Caspof... more Invasive fungal infections are a significant cause of morbidity and mortality in children. Caspofungin is an echinocandin used as an alternative treatment in the prevention and/or treatment of certain invasive fungal infections in children, although compared to the standard treatment there is little evidence on its efficacy and safety. To evaluate the efficacy and safety of caspofungin compared with other antifungal drugs for the prevention and/or treatment of invasive fungal infections in children. The objective of the initial search strategy was to identify randomized controlled studies of acceptable methodological quality (Jadad scale >3), through the key word "caspofungin", conducted in patients with an age range from 0 to 18 years old. The objective of the initial search strategy was to identify randomized controlled studies of acceptable methodological quality (Jadad scale >3), through the key word "caspofungin", conducted in patients with an age rang...
Resumen El trasplante hepático constituye la única alternativa terapéutica para numerosas enferme... more Resumen El trasplante hepático constituye la única alternativa terapéutica para numerosas enfermedades hepáticas avanzadas. En el seguimiento post-trasplante pueden observarse complicaciones de diversa gravedad. El objetivo de este estudio fue analizar la evolución a largo plazo de los pacientes trasplantados con un seguimiento mayor de un año post quirúrgico. En el período 11/92-11/01 se realizaron 264 trasplantes en 238 pacientes; incluyéndose en este trabajo 143 niños con más de un año post-trasplante. La mediana de edad ± DS fue de 5.41 ± 5.26 años (r: 0.58-21.7 años). Catorce (9.79%) fueron retrasplantados. Todos los pacientes recibieron ciclosporina (inmunosupresión primaria). Las indicaciones de trasplante fueron: falla hepática fulminante (n: 50); atresia de vías biliares (n: 38); cirrosis (n: 37); colestasis crónica (n: 13) y otras (n: 5). Las indicaciones de retrasplante fueron: cirrosis biliar (n: 7); trombosis de la arteria hepática (n: 4) y rechazo crónico (n: 3). Fueron utilizados injertos reducidos en 73/157 trasplantes (14 donantes vivos relacionados y 11 biparticiones hepáticas). La sobrevida global fue de 93%, paciente y 86%, injerto. El retrasplante y el injerto reducido fueron las variables de mayor significación para el aumento del riesgo de muerte. El déficit de talla y masa ósea se recuperó antes de los 3 años post-trasplante. La incidencia de síndrome linfoproliferativo fue del 7.69%. Se diagnosticó hepatitis B de novo en 7 pacientes (4.8%). El riesgo social no afectó la sobrevida. La prevención, detección y tratamiento precoz de las complicaciones en el seguimiento a largo plazo permitió mejorar la evolución de los pacientes.
Paracoccidioidomycosis is the most frequent systemic mycosis in Latin America, caused by the dimo... more Paracoccidioidomycosis is the most frequent systemic mycosis in Latin America, caused by the dimorphic fungus Paracoccidioides. Paracoccidioidomycosis in children is uncommon. Our aim is to describe clinical features of patients who had a confirmed diagnosis of paracoccidioidomycosis in our hospital in the last 10 years. We describe 4 cases of paracoccidioidomycosis in previously healthy children from the north of our country. Diagnoses were made by biopsy or culture. The diagnosis of paracoccidioidomycosis should be considered in a patient coming from regions where Paracoccidioides is endemic, and presenting with a lymphoproliferative syndrome, anemia, eosinophilia and hypergammaglobulinemia.
Resumo: La aparición de ascitis es una complicación importante en la evolución de la enfermedad h... more Resumo: La aparición de ascitis es una complicación importante en la evolución de la enfermedad hepática crónica y ha sido propuesta como un indicador para evaluar la necesidad de transplante hepático. Su tratamiento consiste en dieta hiposódica estricta y ...
RESUMEN El sodio es el catión con mayor concentración y el soluto más importante determinante de ... more RESUMEN El sodio es el catión con mayor concentración y el soluto más importante determinante de la osmola-lidad y del volumen del compartimiento extracelular. La hiponatremia se presenta cuando hay un exceso de agua relativa al sodio. Esta situación ocurre cuando se produce una mayor pérdida de sodio que de agua, cuando hay un incremento del agua corporal total o cuando hay un aumento de ambos, siendo mayor el aumento del agua corporal total. La hiponatremia es importante porque puede provocar daño neurológi-co severo y muerte. A su vez, su tratamiento también puede estar asociado a morbilidad del sistema nervio-so central y a muerte. Esta alteración hidroelectrolítica debe ser diagnosticada y tratada en el contexto del(la) niño(a) que la presenta y no como un desequilibrio de la natremia aislado. La evaluación inicial de la hipona-tremia en un paciente pediátrico debe tener en cuenta la valoración clínica del compartimiento extracelular y la excreción renal de sodio. El tratamient...
Las infecciones fúngicas han aumentado últimamente y el espectro de especies se ha modificado, en... more Las infecciones fúngicas han aumentado últimamente y el espectro de especies se ha modificado, en especial por el aumento del número de pacientes inmunocomprometidos. La anfotericina B es un antimicótico de amplio espectro; ha sido, durante los últimos 50 años, el fármaco de elección para el tratamiento antifúngico empírico y frente al aislamiento de hongos sensibles. Sin embargo, su uso se asocia con efectos adversos como fiebre, escalofríos, náuseas y vómitos (dependientes de la infusión) y, más significativamente, con nefrotoxicidad. Existen en la actualidad tres presentaciones farmacéuticas con lípidos que aumentan el índice terapéutico. A pesar de su uso extenso, existen aún interrogantes acerca de las dosis óptimas y costos que justifiquen su empleo en la edad pediátrica. No hay trabajos de alta calidad de evidencia sobre infecciones fúngicas documentadas en pediatría que comparen las cuatro presentaciones farmacéuticas disponibles. No obstante, pareciera que todas tienen similar eficacia. Los elevados costos de las anfotericinas asociadas a lípidos limitan su uso; sin embargo, la reducción de costos asociados a la menor nefrotoxicidad debería considerarse cuando se analizan los aspectos económicos de su indicación.
Post-Transplant Lymphoproliferative Disorder (PTLD) is a life threatening complication in organ t... more Post-Transplant Lymphoproliferative Disorder (PTLD) is a life threatening complication in organ transplant recipients. Risk factors include primary Epstein-Barr virus infection, intensity of immunosupression and cytomegalovirus infection. To evaluate the incidence, clinical presentation, risk factors, histopathologic appearance and outcome of pediatric liver recipients with PTLD at our institution. Retrospective, descriptive and observational analysis. Between November 1992 and December 2005, 383 liver transplants were performed. The diagnosis of PTLD was based on clinical history and physical examination and confirmed by histologic appearance and immunohistologic staining. Knowles' classification was used for histopathologic diagnosis. The incidence of PTLD was 5.7% (n: 22p). The average onset after tansplantation (OLT) was 24.9 months. Clinical manifestations were malaise, anorexia, fever of more than 3 days, peripheral adenopathy, tonsillar hypertrophy, abdominal mass, hepatosplenomegaly, snoring, interstitial pulmonary infiltrate, G.T.-tract bleeding, rash, submaxilar mass. Histopathologic diagnosis were Plasmocytic Hyperplasia (n: 10), Polymorphic Lymphoproliferative Disorder (n: 8), Non-Hodgkin Lymphoma (n: 4). Mortality was 18%. The clinical presentations were protean and not specific. A high index of suspicion is important for early diagnosis as it correlates with more benign lesions and more favorable outcume. The lower mortality rate in our series is concordant with that reported in more recent articles.
Invasive fungal infections are a significant cause of morbidity and mortality in children. Caspof... more Invasive fungal infections are a significant cause of morbidity and mortality in children. Caspofungin is an echinocandin used as an alternative treatment in the prevention and/or treatment of certain invasive fungal infections in children, although compared to the standard treatment there is little evidence on its efficacy and safety. Objectives. To evaluate the efficacy and safety of caspofungin compared with other antifungal drugs for the prevention and/or treatment of invasive fungal infections in children. Material and methods. The objective of the initial search strategy was to identify randomized controlled studies of acceptable methodological quality (Jadad scale >3), through the key word "caspofungin", conducted in patients with an age range from 0 to 18 years old. Results. Only 3 publications met the inclusion criteria. Two of them were studies conducted in children and one in newborn infants. A higher incidence of adverse events was not documented for caspofungin and its efficacy was not different from that of other antifungal drugs (typical RR 1.47; CI 95%: 0.78-2.79). Conclusions. This systematic review suggests that caspofungin could be considered as an alternative drug in children for the prevention and treatment of invasive fungal infections. However, given the small number of existing publications, more studies are required to reach definite conclusions.
Resumo: La aparición de ascitis es una complicación importante en la evolución de la enfermedad h... more Resumo: La aparición de ascitis es una complicación importante en la evolución de la enfermedad hepática crónica y ha sido propuesta como un indicador para evaluar la necesidad de transplante hepático. Su tratamiento consiste en dieta hiposódica estricta y ...
Eficacia y seguridad de la caspofungina en pediatría: revisión sistemática y metaanálisis Efficac... more Eficacia y seguridad de la caspofungina en pediatría: revisión sistemática y metaanálisis Efficacy and safety of caspofungin in children: systematic review and meta-analysis a.
Voriconazole is a second-generation triazole. It has excellent bioavailability and broad antifung... more Voriconazole is a second-generation triazole. It has excellent bioavailability and broad antifungal spectrum; thus, it is an attractive option for patients at high risk of invasive fungal infections (IFIs). Comparing efficacy and safety of voriconazole with other antifungals in prophylaxis or treatment of IFIs would be useful to draw conclusions regarding prevention and therapeutics of these infections. To assess efficacy and safety of voriconazole compared with other options as prophylaxis or treatment of IFIs in haematology-oncology patients. A literature search was performed in MEDLINE database using the search term 'voriconazole' and completed with manual search. Randomized controlled trials (RCTs) comparing voriconazole with other antifungal agents or placebo. Seven studies fulfilled the eligibility criteria. Five studies compared voriconazole to another comparator as prophylaxis of IFIs and two as treatment. Pooled results showed that voriconazole was more effective than the comparator (RR = 1.17; 95%CI = 1.01-1.34), but heterogeneity was significant (Q test 32.7; p = .00001). Sub-analysis according to prophylaxis showed RR = 1.17; 95%CI = 1.00-1.37; while as treatment, RR = 1.23; 95%CI = 0.68-2.22. Risk of adverse events was not different from that observed for the comparator (RR = 1.06, 95%CI = 0.66-1.72) though significant heterogeneity was detected (p < .01). Voriconazole was as effective and safe as comparators, probably better as prophylaxis than as treatment, but limitations due to variability in the sample size of studies, differences in the age of patients, and heterogeneity between studies' outcome measures indicate the need for further research.
Manejo de la ascitis secundaria a cirrosis en el paciente pediátrico. Revisión y recomendaciones ... more Manejo de la ascitis secundaria a cirrosis en el paciente pediátrico. Revisión y recomendaciones Management of cirrhotic ascites in children. Review and recommendations
El sodio es el cation con mayor concentracion y el soluto mas importante determinante de la osmol... more El sodio es el cation con mayor concentracion y el soluto mas importante determinante de la osmolaridad y el volumen del compartimiento extracelular. La hiponatremia se presenta cuando hay un exceso de agua relativa al sodio. Esta situacion ocurre cuando se produce una mayor perdida de sodio que de agua, cuando hay un incremento del agua corporal total o cuando hay un aumento de ambos, siendo mayor el aumento de agua corporal total. La hiponatremia es importante porque puede provocar dano neurologico severo y muerte. A su vez, su tratamiento puede estar asociado a morbilidad del sistema nervioso central y muerte. Esta alteracion hidroelectrolitica debe ser diagnosticada y tratada en el contexto del nino que la presenta y no como un desequilibrio de la natremia aislado. La evaluacion inicial de la hiponatremia en un paciente debe tener en cuenta la valoracion clinica del compartimiento extracelular y la excrecion renal de sodio. El tratamiento dependera de la presencia de compromiso neurologico en el paciente, del tiempo de evolucion y de los niveles sericos de sodio. No hay consenso aun sobre cual es la mejor solucion hidroelectrolitica de mantenimiento endovenoso en el paciente hospitalizado. Sin embargo se sugiere cautela con la administracion de aportes altos, en especial si las soluciones son hipotonicas. Una adecuada comprension del metabolismo hidrosalino puede ayudar al pediatra a prevenir la hiponatremia, a estar alerta de signos incipientes cuendo esta ocurre y tambien sera beneficioso para un tratamiento apropiado de la misma
There are few cases reported of autoimmune hepatitis (AIH) type 2 presenting as fulminant hepatic... more There are few cases reported of autoimmune hepatitis (AIH) type 2 presenting as fulminant hepatic failure (FHF) in children. The purpose of this study was to report three girls with AIH type 2 that presented as FHF. Over a period of 12 years, 123 patients with AIH diagnosed based on international criteria, 9 (7%) were type 2.3 of them presented as FHF. Other etiologies (viral, metabolic and toxic) were ruled out. The treatment was started with prednisone (2 mg-kg-day) and azathioprine (2 mg-kg-day). Patients 1 and 3 died while waiting for liver transplant (LT) at 72 and 48 hours after initiating medical treatment. Patient 2 underwent LT3 days after starting treatment, with excellent evolution at 3 years and 7 months of follow up. 1--AIH type 2 was very infrequent in our group. 2--33% of cases had initial presentation as FHF. 3--The course of the disease was aggressive, not responding to immunosuppressive therapy. The evolution was unfavorable in all patients. 4--LT is an alternative...
Chronic hyponatremia, unless associated with extracellular fluid volume expansion, is an infreque... more Chronic hyponatremia, unless associated with extracellular fluid volume expansion, is an infrequent electrolyte imbalance in pediatrics. We report an infant with chronic hyponatremia suggestive of a syndrome of inappropriate secretion of antidiuretic hormone (SIADH), in the absence of ADH secretion. A mutation was found in the same codon of the gene that results in a loss-offunction of arginine vasopressin receptor 2 (AVPR2) observed in congenital nephrogenic diabetes insipidus. In this case, a gain-of-function of AVPR 2 was found to be responsible for a SIADH-like state. Keywords Seizure. Syndrome of inappropriate secretion of antidiuretic hormone. AVPR2. Water excretion. Nephrogenic syndrome of inappropriate antidiuresis. Hypo-osmolarity syndrome of inappropriate antidiuresis
Background: The aim of this study was to assess whether daptomycin is safer and more efficacious ... more Background: The aim of this study was to assess whether daptomycin is safer and more efficacious than comparators for the treatment of serious infection caused by gram-positive microorganisms. Methods: Electronic databases (Medline, EMBASE, the Cochrane Central Register of Controlled Trials and clinical registered trials) were searched to identify randomized controlled trials (RCTs) that assessed the efficacy and safety of daptomycin versus therapy with any other antibiotic comparator. Two reviewers independently applied selection criteria, performed a quality assessment and extracted the data. Heterogeneity was assessed, and a random-effects or fixed-effects model, when appropriate, was used for estimates of risk ratio (RR). The primary outcome assessed was the risk of clinical treatment failure among the intention-to-treat population and the presence of any treatment related adverse event (AEs). Results: A total of seven trials fulfilled the inclusion criteria. Daptomycin treatmen...
Voriconazole is a second-generation triazole. It has excellent bioavailability and broad antifung... more Voriconazole is a second-generation triazole. It has excellent bioavailability and broad antifungal spectrum; thus, it is an attractive option for patients at high risk of invasive fungal infections (IFIs). Comparing efficacy and safety of voriconazole with other antifungals in prophylaxis or treatment of IFIs would be useful to draw conclusions regarding prevention and therapeutics of these infections. To assess efficacy and safety of voriconazole compared with other options as prophylaxis or treatment of IFIs in haematology-oncology patients. A literature search was performed in MEDLINE database using the search term 'voriconazole' and completed with manual search. Randomized controlled trials (RCTs) comparing voriconazole with other antifungal agents or placebo. Seven studies fulfilled the eligibility criteria. Five studies compared voriconazole to another comparator as prophylaxis of IFIs and two as treatment. Pooled results showed that voriconazole was more effective th...
Ascites is a major complication of cirrhosis. There are several evidence-based articles and guide... more Ascites is a major complication of cirrhosis. There are several evidence-based articles and guidelines for the management of adults, but few data have been published in relation to children. In the case of pediatric patients with cirrhotic ascites (PPCA), the following questions are raised: How are the clinical assessment and ancillary tests performed? When is ascites considered refractory? How is it treated? Should fresh plasma and platelets be infused before abdominal paracentesis to prevent bleeding? What are the hospitalization criteria? What are the indicated treatments? What complications can patients develop? When and how should hyponatremia be treated? What are the diagnostic criteria for spontaneous bacterial peritonitis? How is it treated? What is hepatorenal syndrome? How is it treated? When should albumin be infused? When should fluid intake be restricted? The recommendations made here are based on pathophysiology and suggest the preferred approach to its diagnostic and ...
Invasive fungal infections are a significant cause of morbidity and mortality in children. Caspof... more Invasive fungal infections are a significant cause of morbidity and mortality in children. Caspofungin is an echinocandin used as an alternative treatment in the prevention and/or treatment of certain invasive fungal infections in children, although compared to the standard treatment there is little evidence on its efficacy and safety. To evaluate the efficacy and safety of caspofungin compared with other antifungal drugs for the prevention and/or treatment of invasive fungal infections in children. The objective of the initial search strategy was to identify randomized controlled studies of acceptable methodological quality (Jadad scale >3), through the key word "caspofungin", conducted in patients with an age range from 0 to 18 years old. The objective of the initial search strategy was to identify randomized controlled studies of acceptable methodological quality (Jadad scale >3), through the key word "caspofungin", conducted in patients with an age rang...
Resumen El trasplante hepático constituye la única alternativa terapéutica para numerosas enferme... more Resumen El trasplante hepático constituye la única alternativa terapéutica para numerosas enfermedades hepáticas avanzadas. En el seguimiento post-trasplante pueden observarse complicaciones de diversa gravedad. El objetivo de este estudio fue analizar la evolución a largo plazo de los pacientes trasplantados con un seguimiento mayor de un año post quirúrgico. En el período 11/92-11/01 se realizaron 264 trasplantes en 238 pacientes; incluyéndose en este trabajo 143 niños con más de un año post-trasplante. La mediana de edad ± DS fue de 5.41 ± 5.26 años (r: 0.58-21.7 años). Catorce (9.79%) fueron retrasplantados. Todos los pacientes recibieron ciclosporina (inmunosupresión primaria). Las indicaciones de trasplante fueron: falla hepática fulminante (n: 50); atresia de vías biliares (n: 38); cirrosis (n: 37); colestasis crónica (n: 13) y otras (n: 5). Las indicaciones de retrasplante fueron: cirrosis biliar (n: 7); trombosis de la arteria hepática (n: 4) y rechazo crónico (n: 3). Fueron utilizados injertos reducidos en 73/157 trasplantes (14 donantes vivos relacionados y 11 biparticiones hepáticas). La sobrevida global fue de 93%, paciente y 86%, injerto. El retrasplante y el injerto reducido fueron las variables de mayor significación para el aumento del riesgo de muerte. El déficit de talla y masa ósea se recuperó antes de los 3 años post-trasplante. La incidencia de síndrome linfoproliferativo fue del 7.69%. Se diagnosticó hepatitis B de novo en 7 pacientes (4.8%). El riesgo social no afectó la sobrevida. La prevención, detección y tratamiento precoz de las complicaciones en el seguimiento a largo plazo permitió mejorar la evolución de los pacientes.
Paracoccidioidomycosis is the most frequent systemic mycosis in Latin America, caused by the dimo... more Paracoccidioidomycosis is the most frequent systemic mycosis in Latin America, caused by the dimorphic fungus Paracoccidioides. Paracoccidioidomycosis in children is uncommon. Our aim is to describe clinical features of patients who had a confirmed diagnosis of paracoccidioidomycosis in our hospital in the last 10 years. We describe 4 cases of paracoccidioidomycosis in previously healthy children from the north of our country. Diagnoses were made by biopsy or culture. The diagnosis of paracoccidioidomycosis should be considered in a patient coming from regions where Paracoccidioides is endemic, and presenting with a lymphoproliferative syndrome, anemia, eosinophilia and hypergammaglobulinemia.
Resumo: La aparición de ascitis es una complicación importante en la evolución de la enfermedad h... more Resumo: La aparición de ascitis es una complicación importante en la evolución de la enfermedad hepática crónica y ha sido propuesta como un indicador para evaluar la necesidad de transplante hepático. Su tratamiento consiste en dieta hiposódica estricta y ...
RESUMEN El sodio es el catión con mayor concentración y el soluto más importante determinante de ... more RESUMEN El sodio es el catión con mayor concentración y el soluto más importante determinante de la osmola-lidad y del volumen del compartimiento extracelular. La hiponatremia se presenta cuando hay un exceso de agua relativa al sodio. Esta situación ocurre cuando se produce una mayor pérdida de sodio que de agua, cuando hay un incremento del agua corporal total o cuando hay un aumento de ambos, siendo mayor el aumento del agua corporal total. La hiponatremia es importante porque puede provocar daño neurológi-co severo y muerte. A su vez, su tratamiento también puede estar asociado a morbilidad del sistema nervio-so central y a muerte. Esta alteración hidroelectrolítica debe ser diagnosticada y tratada en el contexto del(la) niño(a) que la presenta y no como un desequilibrio de la natremia aislado. La evaluación inicial de la hipona-tremia en un paciente pediátrico debe tener en cuenta la valoración clínica del compartimiento extracelular y la excreción renal de sodio. El tratamient...
Las infecciones fúngicas han aumentado últimamente y el espectro de especies se ha modificado, en... more Las infecciones fúngicas han aumentado últimamente y el espectro de especies se ha modificado, en especial por el aumento del número de pacientes inmunocomprometidos. La anfotericina B es un antimicótico de amplio espectro; ha sido, durante los últimos 50 años, el fármaco de elección para el tratamiento antifúngico empírico y frente al aislamiento de hongos sensibles. Sin embargo, su uso se asocia con efectos adversos como fiebre, escalofríos, náuseas y vómitos (dependientes de la infusión) y, más significativamente, con nefrotoxicidad. Existen en la actualidad tres presentaciones farmacéuticas con lípidos que aumentan el índice terapéutico. A pesar de su uso extenso, existen aún interrogantes acerca de las dosis óptimas y costos que justifiquen su empleo en la edad pediátrica. No hay trabajos de alta calidad de evidencia sobre infecciones fúngicas documentadas en pediatría que comparen las cuatro presentaciones farmacéuticas disponibles. No obstante, pareciera que todas tienen similar eficacia. Los elevados costos de las anfotericinas asociadas a lípidos limitan su uso; sin embargo, la reducción de costos asociados a la menor nefrotoxicidad debería considerarse cuando se analizan los aspectos económicos de su indicación.
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