RESUMO
BACKGROUND: Mesial temporal lobe epilepsy (MTLE) is a symptomatic epilepsy syndrome clinically characterized by high prevalence, pharmacoresistance, good surgical prognosis and hippocampal sclerosis (HS); however, no singular criteria can be considered sufficient for the MTLE-HS diagnosis. MicroRNAs (miRNAs) are small non-coding molecules that act as important gene-expression regulators at post-transcriptional level. Evidences on the involvement of miRNAs in epilepsy pathogenesis as well as their potential to be employed as biomarkers claim for investigations on miRNAs' applicability as epilepsy diagnosis and prognosis biomarkers. Consequently, the present study aimed to evaluate the applicability of three specific miRNAs as biomarkers of diagnosis and surgical outcomes in adult patients with MTLE-HS. METHOD: Hippocampus, amygdala and blood samples from 20 patients with MTLE-HS were analyzed, 10 with favorable surgical prognosis (Engel I) and 10 with unfavorable surgical prognosis (Engel III-IV). For the control groups, hippocampus and amygdala from necropsy and blood samples from healthy individuals were adopted. The miRNAs expression analysis was performed using Real-Time Quantitative Polymerase Chain Reaction for miRNAs highlighted from microarray as being involved in GABAergic neurotransmission. RESULTS: The miRNAs miR-629-3p, miR-1202 and miR-1225-5p were found to be hyper-expressed in MTLE-HS patients' blood. CONCLUSIONS: Our data suggest the existence of three circulating miRNAs (miR-629-3p, miR-1202 and miR-1225-5p) that could possibly act as additional tools in the set of factors that contribute to MTLE-HS diagnose.
Assuntos
Epilepsia do Lobo Temporal , MicroRNAs , Adulto , Humanos , Epilepsia do Lobo Temporal/diagnóstico , Epilepsia do Lobo Temporal/genética , Epilepsia do Lobo Temporal/cirurgia , Esclerose/diagnóstico , Esclerose/metabolismo , Esclerose/patologia , Hipocampo/cirurgia , Hipocampo/metabolismo , Hipocampo/patologia , MicroRNAs/genética , MicroRNAs/metabolismo , BiomarcadoresRESUMO
Myopathies secondary to collagen VI mutations (COLVI-M) are the most frequent in the northern hemisphere, affecting the adult and pediatric population. There are no data on its prevalence in Latin America. They are characterized by a great clinical variability, from severe phenotypes, such as Ullrich congenital muscular dystrophy (UCMD), to intermediate and mild ones such as Bethlem myopathy (BM). Its onset is also variable and extends from the neonatal period to adulthood. Given the presence of joint hypermobility, the differential diagnosis should be made with various connective tissue diseases. The classical diagnostic algorithm in many patients has been insufficient to guide the genetic study in an adequate way, and from this the muscular magnetic resonance imaging has emerged as a very useful tool for a better diagnostic approach of this and other muscular pathologies. This ob jective of this review is to study the forms of presentation, clinical characteristics, specific diagnostic study, differential diagnosis and management of one of the most frequent hereditary muscular patho logies, with emphasis on the contribution of muscle magnetic resonance imaging.
Assuntos
Colágeno Tipo VI/genética , Contratura/diagnóstico , Distrofias Musculares/congênito , Esclerose/diagnóstico , Contratura/genética , Contratura/terapia , Diagnóstico Diferencial , Marcadores Genéticos , Testes Genéticos , Humanos , Imageamento por Ressonância Magnética , Distrofias Musculares/diagnóstico , Distrofias Musculares/genética , Distrofias Musculares/terapia , Mutação , Exame Físico , Esclerose/genética , Esclerose/terapiaRESUMO
Resumen: Las miopatías secundarias a mutaciones en el colágeno VI (M-COLVI) son las más frecuentes en el hemisferio norte, afectando población adulta y pediátrica. No existen datos de su prevalencia en Latinoamérica. Se caracterizan por presentar una gran variabilidad clínica, desde fenotipos severos, como la distrofia muscular congénita de Ullrich (DMCU), a intermedios y leves como la Miopatía de Bethlem (MB). Su inicio también es variable y se extiende desde el período de recién nacido hasta la vida adulta. Dada la presencia de hiperlaxitud articular, el diagnóstico diferencial se debe realizar con diversas enfermedades del tejido conectivo. El algoritmo diagnóstico clásico en muchos pacientes ha sido insuficiente para orientar el estudio genético de forma adecuada, y a partir de esto la resonancia magnética muscular ha emergido como una herramienta de gran utilidad para una mejor aproxima ción diagnóstica de ésta y otras patologías musculares. Esta revisión tiene como objetivo examinar las formas de presentación, características clínicas, estudio diagnóstico específico, diagnóstico dife rencial y manejo de una de las patologías musculares herediatarias más frecuentes, con énfasis en el aporte de la resonancia magnética muscular.
Abstract: Myopathies secondary to collagen VI mutations (COLVI-M) are the most frequent in the northern hemisphere, affecting the adult and pediatric population. There are no data on its prevalence in Latin America. They are characterized by a great clinical variability, from severe phenotypes, such as Ullrich congenital muscular dystrophy (UCMD), to intermediate and mild ones such as Bethlem myopathy (BM). Its onset is also variable and extends from the neonatal period to adulthood. Given the presence of joint hypermobility, the differential diagnosis should be made with various connective tissue diseases. The classical diagnostic algorithm in many patients has been insufficient to guide the genetic study in an adequate way, and from this the muscular magnetic resonance imaging has emerged as a very useful tool for a better diagnostic approach of this and other muscular pathologies. This ob jective of this review is to study the forms of presentation, clinical characteristics, specific diagnostic study, differential diagnosis and management of one of the most frequent hereditary muscular patho logies, with emphasis on the contribution of muscle magnetic resonance imaging.
Assuntos
Humanos , Esclerose/diagnóstico , Contratura/diagnóstico , Colágeno Tipo VI/genética , Distrofias Musculares/congênito , Exame Físico , Esclerose/genética , Esclerose/terapia , Imageamento por Ressonância Magnética , Marcadores Genéticos , Testes Genéticos , Contratura/genética , Contratura/terapia , Diagnóstico Diferencial , Distrofias Musculares/diagnóstico , Distrofias Musculares/genética , Distrofias Musculares/terapia , MutaçãoRESUMO
OBJECTIVE: We evaluated extratemporal metabolic changes with phosphorus magnetic resonance spectroscopy (31P-MRS) in patients with unilateral mesial temporal sclerosis (MTS). METHOD: 31P-MRS of 33 patients with unilateral MTS was compared with 31 controls. The voxels were selected in the anterior, posterior insula-basal ganglia (AIBG, PIBG) and frontal lobes (FL). Relative values of phosphodiesters- PDE, phosphomonoesters-PME, inorganic phosphate - Pi, phosphocreatine- PCr, total adenosine triphosphate [ATPt = γ- + a- + b-ATP] and the ratios PCr/ATPt, PCr/γ-ATP, PCr/Pi and PME/PDE were obtained. RESULTS: We found energetic abnormalities in the MTS patients compared to the controls with Pi reduction bilaterally in the AIBG and ipsilaterally in the PIBG and the contralateral FL; there was also decreased PCr/γ-ATP in the ipsilateral AIBG and PIBG. Increased ATPT in the contralateral AIBG and increased γ-ATP in the ipsilateral PIBG were detected. CONCLUSION: Widespread energy dysfunction was detected in patients with unilateral MTS.
Assuntos
Espectroscopia de Ressonância Magnética/métodos , Fósforo/metabolismo , Lobo Temporal/patologia , Adulto , Estudos de Casos e Controles , Epilepsia do Lobo Temporal/metabolismo , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose/diagnóstico , Esclerose/metabolismo , Lobo Temporal/metabolismo , Adulto JovemRESUMO
ABSTRACT Objective We evaluated extratemporal metabolic changes with phosphorus magnetic resonance spectroscopy (31P-MRS) in patients with unilateral mesial temporal sclerosis (MTS). Method 31P-MRS of 33 patients with unilateral MTS was compared with 31 controls. The voxels were selected in the anterior, posterior insula-basal ganglia (AIBG, PIBG) and frontal lobes (FL). Relative values of phosphodiesters- PDE, phosphomonoesters-PME, inorganic phosphate - Pi, phosphocreatine- PCr, total adenosine triphosphate [ATPt = γ- + a- + b-ATP] and the ratios PCr/ATPt, PCr/γ-ATP, PCr/Pi and PME/PDE were obtained. Results We found energetic abnormalities in the MTS patients compared to the controls with Pi reduction bilaterally in the AIBG and ipsilaterally in the PIBG and the contralateral FL; there was also decreased PCr/γ-ATP in the ipsilateral AIBG and PIBG. Increased ATPT in the contralateral AIBG and increased γ-ATP in the ipsilateral PIBG were detected. Conclusion Widespread energy dysfunction was detected in patients with unilateral MTS.
RESUMO Objetivo Nós avaliamos as alterações metabóblicas através da espectroscopia de fósforo por ressonância magnética (31P-MRS) em pacientes com esclerose mesial temporal (EMT) unilateral. Método 31P-MRS de 33 pacientes com EMT unilateral foram comparadas aos de 31 controles. Foram selecionados os voxels nas regiões insulonuclear anterior e posterior (RINA e RINP) e frontal (RF). Os valores relativos de fosfodiésteres – PDE, fosfomonoésteres- PME, fosfato inorgânico- Pi, fosfocreatina –PCr, adenosina trifosfato total [ATPt = γ- + a- + b-ATP] e as razões PCr/ATPt, PCr/γ-ATP, PCr/Pi e PME/PDE foram obtidas. Resultados Nós encontramos anormalidades em pacientes com EMT em comparação aos controles. Redução de Pi nas RINA bilateralmente, RINP ipsilateral e RF contralateral, redução de PCr/γ-ATP nas RINA e RINP ipsilaterais foram detectadas. Aumentos de ATPT na RINA contralateral e aumento de γ-ATP na RINP ipsilateral também foram encontradas. Conclusão Disfunção energética difusa foi encontrada em pacientes com EMT unilateral.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Adulto Jovem , Fósforo/metabolismo , Lobo Temporal/patologia , Espectroscopia de Ressonância Magnética/métodos , Esclerose/diagnóstico , Esclerose/metabolismo , Lobo Temporal/metabolismo , Estudos de Casos e Controles , Epilepsia do Lobo Temporal/metabolismoRESUMO
The objectives of this study were to assess religiosity aspects in patients with epilepsy (PWEs) and controls and to determine whether such aspects were related to the samples' clinical, sociodemographic, and QOL-31 data. The Duke Religion Index was administered to 159 adult PWEs and 50 controls. The relationships between the Duke Religion Index and the study variables of the two groups were compared. Intrinsic religiosity (IR) and nonorganizational religiosity (NOR) were higher in PWEs than in controls. Logistic regression showed that being female (p=0.022) and having mesial temporal lobe epilepsy with hippocampus sclerosis (MTLE-HS) (p=0.003) were predictors of high organizational religiosity (OR) and that high NOR was associated with MTLE-HS (p=0.026) and controlled seizures. Further, only MTLE-HS (p=0.002) was predictive of high IR. The Duke Religion Index and QOLIE-31 scores were not related. Different forms of interictal religiosity are related to clinical aspects of epilepsy.
Assuntos
Epilepsia do Lobo Temporal/diagnóstico , Epilepsia do Lobo Temporal/psicologia , Religião e Psicologia , Adulto , Feminino , Hipocampo/patologia , Humanos , Entrevista Psicológica/métodos , Masculino , Pessoa de Meia-Idade , Esclerose/diagnóstico , Esclerose/psicologia , Convulsões/diagnóstico , Convulsões/psicologiaRESUMO
PURPOSE: Do epilepsy and spirituality interact? This study aimed to determine whether an easy-to-administer scale, such as the spirituality self-rating scale (SSRS), could detect increased religiousness in people with epilepsy and verify how epilepsy influences spirituality. METHODS: A total of 196 consecutive patients with epilepsy (epilepsy group, EG) with a mean age and standard deviation of 46.5 ± 14.8 years and 66 subjects with no history of neurological or other chronic disorders (control group, CG) were assessed by the SSRS and neurologically. RESULTS: The SSRS scores of the EG and CG did not differ significantly (22.8 ± 5.1 and 22.0 ± 5.7, respectively). Patients with mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) had significantly higher SSRS scores than those with other epileptic syndromes and, than in individuals of the CG. Multiple regression showed that the factors significantly associated with greater spirituality (greater SSRS score) for the EG, were lower education level, abnormal background EEG activity, and MTLE-HS. Other relationships with the clinical features of epilepsy and with the presence of psychiatric co-morbidity were not found. CONCLUSION: The present findings do not confirm a specific role of epilepsy in spirituality or of "epileptic hyperreligiosity," but suggest that spirituality in people with epilepsy is influenced by education level, and may also stem from epilepsy-related factors such as abnormal background EEG activity and the presence of MTLE-HS.
Assuntos
Epilepsia/fisiopatologia , Epilepsia/psicologia , Autorrelato , Espiritualidade , Adulto , Eletroencefalografia/métodos , Eletroencefalografia/psicologia , Epilepsia/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose/diagnóstico , Esclerose/fisiopatologia , Esclerose/psicologiaRESUMO
Sclerosing peritonitis is a complication described in different clinical situations, such as patients that underwent prolonged peritoneal dialysis or renal transplantation with previous history of peritoneal dialysis. The origin of this entity is unclear so far and it is believed that several mechanisms may contribute to its development. The hallmark of sclerosing peritonitis is the continuous accumulation of fibrocollagenous deposits in the intestinal wall and mesenteries causing progressive adhesion of the intestinal loops and mesenteric retraction resulting in intestinal obstruction. Also, it has been described as a rare complication after intestinal transplant that might lead to graft failure. In this report, we describe a case of sclerosing peritonitis after intestinal transplantation that was successfully treated with modifications in the immunosuppressive regime allowing restitution of gastrointestinal transit and intestinal autonomy.
Assuntos
Terapia de Imunossupressão/métodos , Intestinos/transplante , Peritonite/etiologia , Esclerose/etiologia , Biópsia , Criança , Doença de Hirschsprung/terapia , Humanos , Imunoglobulina E/sangue , Imunossupressores/uso terapêutico , Obstrução Intestinal/diagnóstico , Obstrução Intestinal/etiologia , Masculino , Peritonite/diagnóstico , Complicações Pós-Operatórias , Esclerose/diagnóstico , Transplante/efeitos adversos , Resultado do TratamentoRESUMO
This review highlights the role of delayed enhancement magnetic resonance imaging for the diagnosis of patients with nonischemic myocardial disease. The authors discuss the use of delayed enhancement for differentiation between ischemic and nonischemic myocardial disease and for narrowing the differential diagnosis when nonischemic etiologies are suspected. In addition, special focus is given to the prognostic applications of delayed enhancement magnetic resonance imaging.
Assuntos
Cardiomiopatias/diagnóstico , Aumento da Imagem/métodos , Imageamento por Ressonância Magnética/métodos , Amiloidose/diagnóstico , Arritmias Cardíacas/diagnóstico , Cardiomiopatia Dilatada/diagnóstico , Diagnóstico Diferencial , Gadolínio , Cardiopatias/diagnóstico , Humanos , Processamento de Imagem Assistida por Computador , Isquemia Miocárdica/diagnóstico , Miocardite/diagnóstico , Prognóstico , Esclerose/diagnósticoRESUMO
We present a rare case of thymoma in a 36-year old woman, who was initially diagnosed with severe myasthenia gravis and subsequently undergone surgical resection. During surgery tumor was found at the anterior mediastinum, tightly attached to the phrenic nerve, pleura and pericardium. Histological assessment showed large areas of sclerosis and fibrous collagenous tissue as well as islands of epithelial and lymphoid cells. Sclerosing thymoma, which is a rare subtype of thymoma (< 1%), was diagnosed, thus confirming the first report in Brazil. The patient showed partial improvement of symptoms associated with myasthenia gravis.
Relatamos um caso raro de timoma em uma mulher de 36 anos de idade, com clínica e diagnóstico de miastenia gravis de difícil controle clínico, submetida à ressecção cirúrgica. No intraoperatório, observou-se tumor no mediastino anterior, firmemente aderido ao nervo frênico, à pleura e ao pericárdio. Ao exame histológico, foram evidenciadas extensas áreas de tecido fibrocolagenoso e esclerose, assim como ilhas de células epiteliais e células linfoides. Diagnosticado timoma esclerosante, subtipo raro de timoma (< 1%), sendo este o primeiro caso relatado no Brasil. A paciente apresentou melhora parcial dos sintomas associados à miastenia gravis.
Assuntos
Humanos , Feminino , Adulto , Esclerose/diagnóstico , Miastenia Gravis/complicações , Timoma/classificação , Timoma/diagnósticoRESUMO
PURPOSE: Video electroencephalography (vEEG) monitoring of patients with unilateral mesial temporal sclerosis (uMTS) may show concordant or discordant seizure onset in relation to magnetic resonance imaging (MRI) evidence of MTS. Contralateral seizure usually leads to an indication of invasive monitoring. Contralateral seizure onset on invasive monitoring may contraindicate surgery. We evaluated long-term outcome after anteromesial temporal lobectomy (AMTL) in a consecutive series of uMTS patients with concordant and discordant vEEG findings, uniformly submitted to AMTL on the MRI evidence of MTS side without invasive monitoring. METHODS: We compared surgical outcome of all uMTS patients undergoing vEEG monitoring between January 1999 and April 2005 in our service. Discordant cases were defined by at least one seizure onset contralateral to the MRI evidence of MTS. Good surgical outcome was considered as Engel's class I. We also evaluated ictal SPECT concordance to ictal EEG and surgical outcome. RESULTS: Fifty-four patients had concordant (C) and 22 had discordant (D) scalp EEG and MRI. Surgical outcome was similar in both groups (C = 74% versus D = 86%). Duration of follow-up was comparable in both groups: C = 56.1 +/- 20.7 months versus D = 59.8 +/- 21.2 months (p = 0.83, nonsignificant). Discordant single-photon emission computed tomography (SPECT) results did not influence surgical outcome. DISCUSSION: Surgical outcome was not influenced by contralateral vEEG seizure onset or contralateral increased flow on ictal SPECT. Although vEEG monitoring should still be performed in these patients, to rule out psychogenic seizures and extratemporal seizure onset, a potentially risky procedure such as invasive monitoring may not only not be indicated in this patient population, but may also lead to patients erroneously being denied surgery.
Assuntos
Eletroencefalografia , Epilepsia do Lobo Temporal , Esclerose , Lobo Temporal/patologia , Lobo Temporal/cirurgia , Cirurgia Vídeoassistida/métodos , Adolescente , Adulto , Idoso , Lobectomia Temporal Anterior/métodos , Criança , Epilepsia do Lobo Temporal/diagnóstico , Epilepsia do Lobo Temporal/etiologia , Epilepsia do Lobo Temporal/cirurgia , Feminino , Seguimentos , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Esclerose/complicações , Esclerose/diagnóstico , Esclerose/cirurgia , Lobo Temporal/diagnóstico por imagem , Tomografia Computadorizada de Emissão de Fóton Único , Resultado do TratamentoRESUMO
A mediastinite esclerosante é uma doença rara, caracterizada por tecido fibrótico mediastinal extenso, que mimetiza uma neoplasia devido à compressão ou invasão das estruturas mediastinais. Apresentamos três casos de síndrome de veia cava superior em que se comprovou a mediastinite esclerosante. A fisiopatologia relaciona-se a adenomegalias mediastinais, proliferação de fibroblastos e deposição de colágeno. As principais causas são histoplasmose e tuberculose, doenças prevalentes em nosso meio. O diagnóstico histopatológico geralmente é difícil por métodos pouco invasivos. Necessita-se de exploração cirúrgica para o diagnóstico definitivo e resolução da obstrução das vias aerodigestiva e vascular, uma vez que não há tratamento clínico efetivo para esta afecção.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Neoplasias do Mediastino/diagnóstico , Mediastinite/diagnóstico , Síndrome da Veia Cava Superior/diagnóstico , Diagnóstico Diferencial , Mediastinite/complicações , Mediastinite/patologia , Mediastinite/cirurgia , Esclerose/complicações , Esclerose/diagnóstico , Síndrome da Veia Cava Superior/etiologia , Síndrome da Veia Cava Superior/cirurgiaRESUMO
OBJECT: The authors conducted a study to assess the efficacy of surgery in patients who underwent magnetic resonance (MR) imaging alone for localization of foci in temporal lobe epilepsy (TLE). METHODS: One hundred patients (43 men, 57 women) with a clinical diagnosis of TLE were prospectively studied (mean age 28 +/- 9 years [+/- standard deviation {SD}]). All patients underwent high-resolution MR imaging, and in all unilateral mesial temporal sclerosis (MTS) was diagnosed by visual inspection. All patients underwent interictal preoperative electroencephalography (EEG) and in 87 patients pre- and 1-year postoperative neuropsychological testing was performed. Both EEG and neuropsychological examinations were conducted in a blinded fashion, and these data were not taken into account during the surgery-related decision-making process. All patients underwent a corticoamygdalohippocampectomy at the side of the MTS. Surgery-related outcome was rated as Class I (seizure free or simple partial seizures only) or Class II (> or = 90% improvement). The follow-up period ranged from 18 to 48 months (mean 24 +/- 5 months [+/-SD]). No patient underwent prolonged video-EEG monitoring, Wada testing, positron emission tomography, or single-photon emission computerized tomography. In eighty-nine patients Class I results were achieved, and 11 Class II results were achieved postoperatively. There was no mortality in this series. Except for in two patients who underwent surgery in the dominant temporal lobe, there was no postoperatively cognitive decline. In these two patients verbal memory decline occurred, which was associated with posterior temporal cortical damage, demonstrated postoperatively on MR imaging. Twenty-five percent of the patients experienced improved memory function related to the nonoperated side, and 54% experienced a 10% gain in general intelligence quotient status. CONCLUSIONS: In patients with clinically suspected TLE, MR imaging alone is able to localize temporal lobe foci correctly. Ruling out pseudoseizures remains the only indication for prolonged video-EEG recordings in this group of patients.
Assuntos
Epilepsia do Lobo Temporal/diagnóstico , Epilepsia do Lobo Temporal/cirurgia , Lobo Temporal/patologia , Lobo Temporal/cirurgia , Adulto , Tonsila do Cerebelo/cirurgia , Córtex Cerebral/cirurgia , Feminino , Hipocampo/cirurgia , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Procedimentos Neurocirúrgicos/métodos , Estudos Prospectivos , Esclerose/diagnóstico , Esclerose/cirurgia , Convulsões/diagnóstico , Convulsões/cirurgia , Resultado do TratamentoRESUMO
Objetive: To assess the frecuency and clinical picture of Hepatoportal Sclerosis in a population of Mexican children of the Instituto Nacional de Pediatría, México City. Background: Hepatoportal Sclerosis is a disease of unknown etiology. It's diagnosis is difficut. The main clinical presentation is splenomegaly with or without hematemesis (portal hypertension). Splenoportography and liver histology study are the best procedures for diagnosis and must be performed by experts. Methods: We studied 7/106 children with portal hypertension during a period of 10 years, who were seen at the Instituto Nacional de Pediatría, México city. Inclusion criteria were specifical findings of splenoportography and histologic changes in liver biopsy. Results: We found 7/106 children. The main clinical manifestation were splenomegaly and hematemesis. We did not find any previous history of contact with arsenisc, vinyl chloride or copper sulfate. In 6/7 children a porto-systemic shunt was performed. Only one received propranolol and sclerotheraphy. At the time of this report all children have shown a good clinical course.
Assuntos
Criança , Feminino , Humanos , Adolescente , Sistema Porta/patologia , Incidência , México/epidemiologia , Portografia , Estudos Retrospectivos , Esclerose/diagnóstico , Esclerose/epidemiologiaRESUMO
Objetive: To assess the frecuency and clinical picture of Hepatoportal Sclerosis in a population of Mexican children of the Instituto Nacional de Pediatría, México City. Background: Hepatoportal Sclerosis is a disease of unknown etiology. Its diagnosis is difficut. The main clinical presentation is splenomegaly with or without hematemesis (portal hypertension). Splenoportography and liver histology study are the best procedures for diagnosis and must be performed by experts. Methods: We studied 7/106 children with portal hypertension during a period of 10 years, who were seen at the Instituto Nacional de Pediatría, México city. Inclusion criteria were specifical findings of splenoportography and histologic changes in liver biopsy. Results: We found 7/106 children. The main clinical manifestation were splenomegaly and hematemesis. We did not find any previous history of contact with arsenisc, vinyl chloride or copper sulfate. In 6/7 children a porto-systemic shunt was performed. Only one received propranolol and sclerotheraphy. At the time of this report all children have shown a good clinical course. (AU)