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1.
Arch. argent. pediatr ; 120(1): e17-e20, feb 2022. tab, ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1353739

ABSTRACT

La hipertensión portopulmonar (HTPP) es una complicación infrecuente de la hipertensión portal, que sigue un curso progresivo con un pronóstico sombrío. Los reportes en pacientes pediátricos son escasos y con períodos de seguimiento cortos. Se describe una paciente con cirrosis descompensada que desarrolló HTPP resuelta mediante trasplante hepático, que permanece asintomática tras diez años de seguimiento.


Portopulmonary hypertension is an uncommon complication of portal hypertension, running a progressive course with a negative prognosis. Reports in pediatric patients are scarce with short follow up. We describe the case of decompensated cirrhosis who developed PoPH and resolved with liver transplantation, remaining asymptomatic after ten years of follow up.


Subject(s)
Humans , Female , Child , Liver Transplantation , Hepatitis, Autoimmune/complications , Hypertension, Portal/complications , Hypertension, Pulmonary/etiology , Liver Cirrhosis/complications
2.
Arch. cardiol. Méx ; 90(2): 154-162, Apr.-Jun. 2020. tab, graf
Article in English | LILACS | ID: biblio-1131025

ABSTRACT

Abstract Cirrhotic cardiomyopathy is characterized by the presence of structural and functional cardiac alterations in patients suffering from hepatic cirrhosis, without previously known cardiac causes that may explain it. Clinically, it is characterized by the presence of variable grades of diastolic and systolic dysfunction (SD), alterations in the electric conductance (elongation of corrected QT interval) and inadequate chronotropic response. This pathology has been related to substandard response in the management of patients with portal hypertension and poor outcome after transplant. Even when the first description of this pathology dates back from 1953, it remains a poorly studied and frequently underdiagnosed entity. Echocardiography prevails as a practical diagnostic tool for this pathology since simple measurements as the E/A index can show diastolic dysfunction. SD discloses as a diminished ejection fraction of the left ventricle and the latent forms are detected by echocardiography studies with pharmacological stress. In recent years, new techniques such as the longitudinal strain have been studied and they seem promising for the detection of early alterations.


Resumen La miocardiopatía cirrótica se caracteriza por la presencia de alteraciones cardiacas estructurales y funcionales en pacientes con cirrosis hepática, sin que existan otras causas de enfermedad cardiaca. Clínicamente se caracteriza por la presencia de grados variables de disfunción diastólica y sistólica, alteraciones de la conducción eléctrica (prolongación del intervalo QT) y respuesta cronotrópica inapropiada. Esta patología se ha relacionado con desenlaces clínicos adversos, mala respuesta en el manejo de la hipertensión portal y resultados desfavorables posterior a trasplante hepático ortotópico. A pesar de que las primeras descripciones datan de 1953, es una entidad poco estudiada y frecuentemente subdiagnosticada. El ecocardiograma es una herramienta de diagnóstico importante en esta entidad. Mediciones simples como el índice E/A pueden traducir disfunción diastólica. La disfunción sistólica se manifiesta con disminución de la fracción de eyección del ventrículo izquierdo y las formas latentes se detectan mediante estudios de ecocardiografía con estrés farmacológico; en los últimos años se han estudiado otras técnicas como el strain longitudinal, que parecen prometedoras en la detección de alteraciones tempranas.


Subject(s)
Humans , Echocardiography/methods , Liver Cirrhosis/complications , Cardiomyopathies/etiology , Liver Transplantation , Electrocardiography , Hypertension, Portal/complications , Hypertension, Portal/therapy , Liver Cirrhosis/therapy , Cardiomyopathies/diagnosis , Cardiomyopathies/physiopathology
3.
Rev. chil. pediatr ; 91(2): 251-254, abr. 2020. graf
Article in Spanish | LILACS | ID: biblio-1098899

ABSTRACT

Resumen: Introducción: La hemorragia digestiva por hipertensión portal, sin alternativa de tratamiento endos- cópico o quirúrgico por localizaciones ectópicas, no identificadas del sitio de sangrado o caracterís ticas anatómicas, constituye un desafío terapéutico en Pediatría. El tratamiento habitual incluye la infusión de octreótido endovenoso. En los últimos años, la presentación de octreótido de liberación prolongada (OCT-LAR) para administración mensual intramuscular, resulta una alternativa tera péutica atractiva. Objetivo: Reportar el caso de un lactante con hemorragia digestiva por hiperten sión portal que recibió tratamiento exitoso con OCT-LAR. Caso Clínico: Paciente de 8 meses de vida, con malformación de vena porta extrahepática y episodios reiterados de sangrados digestivos con re querimientos transfusionales e infusiones de octréotido, sin posibilidad de tratamiento endoscópico o quirúrgico. Indicamos OCT-LAR intramuscular mensualmente. Después de diez meses de iniciado el tratamiento, el paciente no repitió sangrados digestivos y no presentó efectos adversos relacionados a la medicación. Conclusión: Consideramos que el reporte de este caso puede resultar de utilidad al presentar una nueva alternativa para el tratamiento de pacientes pediátricos con sangrado digestivo por hipertensión portal sin posibilidades terapéuticas convencionales.


Abstract: Introduction: Upper gastrointestinal bleeding (UGIB) secondary to portal hypertension (PHT), without endoscopic or surgical treatment options due to an ectopic or unidentified bleeding site or the patient's anatomic characteristics, is challenging in pediatric hepatology. The usual treatment in these cases includes intravenous Octreotide. Recently, the availability of long-acting release Octreo tide (OCT-LAR) for monthly intramuscular administration has become an interesting therapeutic alternative. Objective: To report the case of an infant with UGIB due to PHT who was successfully treated with OCT-LAR. Clinical Case: Eight-month-old patient with repeated episodes of UGIB due to extrahepatic portal vein malformation, requiring blood transfusions, and intravenous octreotide infusions. As neither endoscopic nor surgical treatment were feasible, we decided to start IM OCT- LAR monthly. After ten months of treatment, the patient did not present bleeding episodes. No medication-related events were observed. Conclusion: We consider that this report could help in the management of similar pediatric patients with UGIB due to PHT without conventional therapeutic possibilities.


Subject(s)
Humans , Male , Infant , Gastrointestinal Agents/administration & dosage , Octreotide/administration & dosage , Duodenal Diseases/drug therapy , Gastrointestinal Hemorrhage/drug therapy , Hypertension, Portal/complications , Gastrointestinal Agents/therapeutic use , Octreotide/therapeutic use , Delayed-Action Preparations , Duodenal Diseases/etiology , Gastrointestinal Hemorrhage/etiology , Injections, Intramuscular
4.
ABCD arq. bras. cir. dig ; 33(3): e1525, 2020. tab, graf
Article in English | LILACS | ID: biblio-1141906

ABSTRACT

ABSTRACT Background: Portal hypertension (PH) can be measured indirectly through a hepatic vein pressure gradient greater than 5 mmHg. Cirrhosis is the leading cause for PH and can present as complications ascites, hepatic dysfunction, renal dysfunction, and esophagogastric varices, characterizing gastropathy. Aim: To evaluate the use of carvedilol as primary prophylaxis in the development of collateral circulation in rats submitted to the partial portal vein ligament (PPVL) model. Method: This is a combined qualitative and quantitative experimental study in which 32 Wistar rats were divided into four groups (8 animals in each): group I - cirrhosis + carvedilol (PPVL + C); group II - cirrhosis + vehicle (PPVL); group III - control + carvedilol (SO-sham-operated + C); group IV - control + vehicle (SO-sham-operated). After seven days of the surgical procedure (PPVL or sham), carvedilol (10 mg/kg) or vehicle (1 mL normal saline) were administered to the respective groups daily for seven days. Results: The histological analysis showed no hepatic alteration in any group and a decrease in edema and vasodilatation in the PPVL + C group. The laboratory evaluation of liver function did not show a statistically significant change between the groups. Conclusion: Carvedilol was shown to have a positive effect on gastric varices without significant adverse effects.


RESUMO Racional: A hipertensão portal (HP), medida indiretamente através do gradiente pressórico da veia hepática >5 mmHg, tem como principal causa etiológica a cirrose. Possui como complicações a ascite, disfunção hepática, disfunção renal e varizes esofagogástricas, que caracterizam o quadro de gastropatia. Objetivo: Avaliar o uso do carvedilol como profilaxia primária no desenvolvimento da circulação colateral em ratos submetidos ao modelo de ligadura parcial de veia porta (LPVP). Método: Estudo experimental qualitativo e quantitativo no qual foram utilizados 32 ratos Wistar, divididos em quatro grupos (n=8): grupo I - cirrose + carvedilol (LPVP+C); grupo II - cirrose + veículo (LPVP); grupo III - controle + carvedilol (SO - sham-operated+C); grupo IV - controle + veículo (SO - sham-operated). Após transcorridos sete dias do procedimento cirúrgico, foi administrado carvedilol (10 mg/kg) e veículo (1mL) para os respectivos grupos por sete dias consecutivos. Resultados: A análise histológica não mostrou alteração hepática em nenhum grupo e diminuição de edema e vasodilatação no grupo LPVP+C. A avaliação laboratorial da função hepática não mostrou alteração com significância estatística entre os grupos. Conclusão: Carvedilol mostrou ser fármaco com efeito positivo no sangramento das varizes gástricas e sem efeitos adversos significantes.


Subject(s)
Animals , Rats , Adrenergic beta-Agonists/administration & dosage , Carvedilol/administration & dosage , Gastrointestinal Hemorrhage/prevention & control , Hypertension, Portal/complications , Antihypertensive Agents/administration & dosage , Esophageal and Gastric Varices/complications , Esophageal and Gastric Varices/prevention & control , Rats, Wistar , Gastrointestinal Hemorrhage/etiology
5.
Arq. gastroenterol ; 55(4): 324-328, Oct.-Dec. 2018. tab, graf
Article in English | LILACS | ID: biblio-983843

ABSTRACT

ABSTRACT The disease and the case reported here are relevant especially because of their varied clinical presentation, possibility of being associated with other disorders affecting several organs and possible differential diagnoses. Congenital Hepatic Fibrosis is an autosomal recessive disease due to mutation in the PKHD1 gene, which encodes the fibrocystin/polyductine protein. It is a cholangiopathy, characterized by varying degrees of periportal fibrosis and irregular proliferation of bile ducts. Affected patients are typically diagnosed in childhood, but in some cases the disease may remain asymptomatic for many years. The exact prevalence and incidence of the disease are not known, but it is consider a rare disease, with a few hundred cases described worldwide. It can affect all ethnic groups and occur associated with various hereditary and non-hereditary disorders. The clinical presentation is quite variable, with melena and hematemesis being initial symptoms in 30%-70% of the cases. More rarely, they may present episodes of cholangitis. The disease has been classified into four types: portal hypertension, cholestasis / cholangitis, mixed and latent. Diagnosis begins with imaging tests, but the definition is made by the histopathological sample. So far, there is no specific therapy that can stop or reverse the pathological process. Currently, the therapeutic strategy is to treat the complications of the disease.


RESUMO A patologia e o caso aqui reportados são relevantes especialmente devido sua variada apresentação clínica, possibilidade de estar associada com outras desordens acometendo diversos órgãos e pelos possíveis diagnósticos diferenciais. A fibrose hepática congênita é uma doença autossômica recessiva, devido mutação no gene PKHD1, que codifica a proteína fibrocistina/poliductina. É uma colangiopatia, caracterizada por variados graus de fibrose periportal e proliferação irregular de ductos biliares. Os pacientes acometidos são tipicamente diagnosticados na infância, mas em alguns casos a doença pode permanecer assintomática por muitos anos. Exatas prevalência e incidência da doença não são conhecidas, mas sabe-se que é uma doença bastante rara, com algumas centenas de casos descritos no mundo. Pode afetar todos grupos étnicos e ocorrer associada com diversas desordens hereditárias e não-hereditárias. A apresentação clínica é bastante variável, com melena e hematêmese sendo sintomas iniciais em 30%-70% dos casos. Mais raramente, podem apresentar episódios de colangite. A doença tem sido classificada em quatro tipos: hipertensão portal, colestática/colangite, mista e latente. O diagnóstico inicia com exames de imagem, mas a definição é feita pela amostra histopatológica. Até o momento, não há terapia específica que possa parar ou reverter o processo patológico e a estratégia terapêutica atual é tratar as complicações da doença.


Subject(s)
Humans , Male , Female , Genetic Diseases, Inborn/diagnosis , Hypertension, Portal/diagnosis , Liver Cirrhosis/diagnosis , Incidental Findings , Asymptomatic Diseases , Genetic Diseases, Inborn/complications , Genetic Diseases, Inborn/therapy , Hypertension, Portal/complications , Hypertension, Portal/therapy , Polycystic Kidney Diseases/complications , Polycystic Kidney Diseases/diagnosis , Polycystic Kidney Diseases/therapy , Liver Cirrhosis/complications , Liver Cirrhosis/congenital , Liver Cirrhosis/therapy , Middle Aged
7.
Arch. cardiol. Méx ; 88(1): 25-38, ene.-mar. 2018. tab
Article in Spanish | LILACS | ID: biblio-1054985

ABSTRACT

Resumen: La hipertensión portopulmonar (HPP) es una entidad poco frecuente a nivel mundial, aunque se desconocen los datos epidemiológicos en México. Sin embargo, las enfermedades crónicas del hígado son muy prevalentes en mexicanos. La HPP es el 4.◦ subtipo en frecuencia del grupo de la hipertensión arterial pulmonar. Su diagnóstico está dentro de 2 escenarios: los pacientes con sospecha de hipertensión pulmonar y los candidatos a trasplante hepático ortotópico (THO). Tanto el ecocardiograma como el cateterismo cardiaco derecho son determinantes para el diagnóstico en ambos escenarios. La HPP es un reto para el THO, pues aumenta la mortalidad perioperatoria de manera importante. El uso de terapia específica es la piedra angular de este padecimiento, como una medida para poder mejorar el desenlace de los que llegan a ser candidatos a un THO con HPP moderada a grave. Es importante reconocer que la HPP puede llegar a ser una contraindicación para el THO. Hasta el momento el papel del trasplante combinado pulmón-hígado o corazón-pulmón-hígado como una medida de curación de la enfermedad vascular pulmonar en pacientes con HPP es incierto. © 2016 Instituto Nacional de Cardiolog´ıa Ignacio Cha´vez. Publicado por Masson Doyma Me´xico S.A. Este es un art´ıculo Open Access bajo la licencia CC BY-NC-ND (https://creativecommons.org/licenses/by-nc-nd/4.0/).


Abstract: Portopulmonary hypertension (PPH) is a rare condition worldwide, although epidemiological data are unknown in Mexico. However, chronic liver diseases are very prevalent in Mexico. PPH is the 4th subtype in frequency in the group of pulmonary arterial hypertension. Its diagnosis is made within 2 scenarios: patients with suspected pulmonary hypertension and candidates for orthotopic liver transplantation (OLT). Both echocardiogram and a right cardiac catheterisation are crucial for diagnosis in both cases. PPH is a challenge for OLT, since it can significantly increase perioperative mortality. The use of specific therapy is the cornerstone of this disease, as a measure to improve the outcome of those who become candidates for OLT with moderate to severe PPH. It is important to recognise that PPH can be a contraindication to OLT. The role of lung-liver transplantation or heart-lung-liver transplantation as a measure to heal pulmonary vascular disease in patients with PPH is still uncertain.© 2016 Instituto Nacional de Cardiolog´ıa Ignacio Cha´vez. Published by Masson Doyma Me´xico S.A. This is an open access article under the CC BY-NC-ND license (http://creativecommons. org/licenses/by-nc-nd/4.0/).


Subject(s)
Humans , Hypertension, Portal/complications , Hypertension, Pulmonary/complications , Liver Transplantation , Hypertension, Portal/diagnosis , Hypertension, Portal/therapy , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/therapy
8.
Rev. cuba. angiol. cir. vasc ; 18(2): 218-225, jul.-dic. 2017. ilus
Article in Spanish | LILACS | ID: biblio-844821

ABSTRACT

El shunt portosistémico intrahepático transyugular es un tratamiento aceptado y efectivo para el manejo de las complicaciones de la hipertensión portal en el paciente con cirrosis hepática. La colocación de este shunt es una técnica segura que mejora la calidad de vida de estos pacientes y de aquellos con ascitis refractaria o sangrados gastrointestinales por varices esofágicas. Esta técnica surgió hace más de 20 años, aun así, no existen publicaciones en la literatura médica sobre la implementación de este proceder en Cuba. Se describe la colocación de un stent-graft portosistémico recubierto tipo Viatorr realizada en el 2015 en un paciente masculino de 52 años de edad que debuta con un cuadro de sangrado digestivo alto como consecuencia de una cirrosis enólica diagnosticada 6 años antes. El proceder se llevó a cabo sin complicaciones inmediatas y con una reducción instantánea de la presión portal de 24 a 12 por ciento. Podemos señalar que el proceso de implantación del shunt portosistémico intrahepático transyugular es técnicamente realizable por el radiólogo intervencionista. Los resultados dependerán en gran medida de la experiencia del operador. Este proceder que gana cada día más confiabilidad entre los médicos y los pacientes, por su alta tasa de éxito y capacidad resolutiva, puede ser implementado en Cuba de manera sistemática y, a partir de esta experiencia inicial, generalizar su proceder a otros centros hospitalarios del país para beneficio de los pacientes(AU)


Transjugular intrahepatic portosystemic shunt is an effective accepted treatment in the management of portal hypertension complications in a hepatic cirrhosis patient. Transjugular intrahepatic portosystemic shunt placement is a safe technique that improves the quality of life of these patients and of those suffering from refractory ascites and gastrointestinal bleedings. This procedure emerged over 20 years ago; however no reports on Cuban literature about the implementation of this technique are found. This article described the placement of a covered portosystemic stent graft performed in a 52 years-old male patient who presented with upper digestive bleeding resulting from enolic cirrhosis diagnosed 6 years before. The procedure was performed with no immediate complicatios and instant reduction of portal pressure from 24 to 12 percent. We consider that the transjugular intrahepatic portosystemic shunt could be technically placed by the interventionist radiologist. The results will depend to a great extent on the experience of the staff. This procedure, which gains more reliability among physicians and patients because of its high rate of success and resolute capacity, may be implemented in Cuba on a systematic basis. Taking this initial experience as a starting point, the procedure may be extended to other hospitals in the country for the benefit of the patients(AU)


Subject(s)
Humans , Hypertension, Portal/complications , Liver Cirrhosis/complications , Stents
9.
Article in Spanish | LILACS, BNUY, UY-BNMED | ID: biblio-1253741

ABSTRACT

Se describe el caso clínico de una mujer de 62 años en la que se diagnostica una estenosis del colédoco en el contexto de hipertensión portal prehepática (cavernomatosis portal).


We report the case of a 62 years old woman with a choledochal stricture and chronic obstruction of the portal vein (portal cavernomatosis).


Relatamos o caso de uma mulher de 62 anos com diagnóstico de estenose do colédoco no contexto da hipertensão portal pré-hepática (cavernomatose portal).


Subject(s)
Humans , Female , Middle Aged , Cholestasis, Intrahepatic/etiology , Cholestasis, Intrahepatic/diagnostic imaging , Hypertension, Portal/complications , Hypertension, Portal/diagnostic imaging , Bile Ducts, Intrahepatic/pathology , Abdominal Pain/etiology , Common Bile Duct/pathology , Constriction, Pathologic , Gallbladder/pathology
10.
Arq. gastroenterol ; 54(1): 21-26, Jan.-Mar. 2017. tab
Article in English | LILACS | ID: biblio-838826

ABSTRACT

ABSTRACT BACKGROUND Bleeding of esophageal varices is the main cause of morbidity and mortality in children and adults with portal hypertension and there are few studies involving secondary prophylaxis in children and adolescents. OBJECTIVE To evaluate the efficacy of endoscopic secondary prophylaxis in prevention of upper gastrointestinal bleeding in children and adolescents with esophageal varices. METHODS This is a prospective analysis of 85 patients less than 18 years of age with or without cirrhosis, with portal hypertension. Participants underwent endoscopic secondary prophylaxis with sclerotherapy or band ligation. Eradication of varices, incidence of rebleeding, number of endoscopic sessions required for eradication, incidence of developing gastric fundus varices and portal hypertensive gastropathy were evaluated. RESULTS Band ligation was performed in 34 (40%) patients and sclerotherapy in 51 (60%) patients. Esophageal varices were eradicated in 81.2%, after a median of four endoscopic sessions. Varices relapsed in 38 (55.1%) patients. Thirty-six (42.3%) patients experienced rebleeding, and it was more prevalent in the group that received sclerotherapy. Gastric varices and portal hypertensive gastropathy developed in 38.7% and 57.9% of patients, respectively. Patients undergoing band ligation showed lower rebleeding rates (26.5% vs 52.9%) and fewer sessions required for eradication of esophageal varices (3.5 vs 5). CONCLUSION Secondary prophylaxis was effective in eradicating esophageal varices and controlling new upper gastrointestinal bleeding episodes due to the rupture of esophageal varices. Band ligation seems that resulted in lower rebleeding rates and fewer sessions required to eradicate varices than did sclerotherapy.


RESUMO CONTEXTO Os episódios de sangramento das varizes esofágicas são a principal causa de morbidade e mortalidade em crianças e adultos com hipertensão porta e poucos são os estudos envolvendo a profilaxia secundária em crianças e adolescentes. OBJETIVO Avaliar a eficácia da profilaxia endoscópica secundária na prevenção de hemorragia digestiva alta em crianças e adolescentes com varizes de esôfago. MÉTODOS Estudo prospectivo com 85 pacientes menores de 18 anos com hipertensão porta, cirróticos e não cirróticos. A profilaxia secundária endoscópica foi realizada através de ligadura elástica ou escleroterapia. Foram avaliadas erradicação de varizes, incidência de ressangramento, número de sessões endoscópicas necessárias para a erradicação, incidência de surgimento de varizes gástricas e da gastropatia da hipertensão porta. RESULTADOS Ligadura elástica foi realizada em 34 (40%) pacientes e escleroterapia em 51 (60%). As varizes de esôfago foram erradicadas em 81,2% após mediana de quatro sessões endoscópicas. Foi observada recidiva de varizes de esôfago em 38 (55,1%) pacientes. Ressangramento por ruptura de varizes de esôfago ocorreu em 36 (42,3%) pacientes e foi mais prevalente no grupo submetido à escleroterapia. O surgimento de varizes gástricas e gastropatia da hipertensão porta ocorreram em 38,7% e 57,9% respectivamente. Os pacientes submetidos à ligadura elástica apresentaram taxas menores de ressangramento (26,5% vs 52,9%) e número menor de sessões necessárias para erradicação das varizes de esôfago (3,5 vs 5). CONCLUSÃO A profilaxia secundária endoscópica mostrou-se eficaz para erradicação de varizes de esôfago e evitar novos episódios de hemorragia digestiva alta secundária à ruptura de varizes de esôfago. A ligadura elástica endoscópica provavelmente apresenta menores taxas de ressangramento e número menor de sessões necessárias para erradicação das varizes de esôfago, quando comparada à escleroterapia.


Subject(s)
Humans , Male , Female , Child , Adolescent , Esophageal and Gastric Varices/therapy , Esophagoscopy , Secondary Prevention , Gastrointestinal Hemorrhage/prevention & control , Hypertension, Portal/complications , Recurrence , Esophageal and Gastric Varices/complications , Sclerotherapy , Prospective Studies , Follow-Up Studies , Treatment Outcome , Ligation
11.
Rev. méd. Chile ; 144(7): 879-885, jul. 2016. tab
Article in Spanish | LILACS | ID: lil-794001

ABSTRACT

Background: There is a paucity of good quality research about the diagnosis of esophageal varices and the prophylaxis and treatment of variceal bleeding in pediatric patients with portal hypertension There is little consensus and practically no evidence-based approach about the management of these patients. Aim: To describe the behavior and preferences of pediatric gastroenterologists in Chile in the management of portal hypertension in children. Material and Methods: An online survey was sent to Chilean pediatric gastroenterologists, with questions evaluating the physicians’ approaches to screening of esophageal varices in children with portal hypertension, and their preferred methods of prophylaxis and initial management of variceal bleeding. Results: Thirty five of 69 contacted physicians answered the survey (51%). Twenty nine pediatric gastroenterologists (83%) screen for esophageal varices in patients with clinical evidence of portal hypertension, and 12 (34%) in every patient with chronic liver disease. Twenty eight respondents (80%) use primary prophylaxis, mainly beta blockers. Octreotide, proton pump inhibitors and endoscopy are the most common practices in the initial management of an esophageal varix bleed. The methods mostly used as secondary prophylaxis are band ligation and beta blockers. In the case of recurrent hemorrhage, besides band ligation, management with Transjugular Intrahepatic Portosystemic Shunt (TIPS) and hepatic transplantation are more likely. Conclusions: Even though most pediatric gastroenterologists in this survey are inclined to offer endoscopic screening of esophageal varices and prophylaxis to patients with portal hypertension, this is not a universal behavior. There are different approaches mainly in the election of secondary prophylaxis and the initial management of variceal bleeding.


Subject(s)
Humans , Child , Practice Patterns, Physicians'/statistics & numerical data , Esophageal and Gastric Varices/therapy , Gastrointestinal Hemorrhage/therapy , Hypertension, Portal/complications , Hypertension, Portal/therapy , Esophageal and Gastric Varices/complications , Gastrointestinal Hemorrhage/etiology , Gastrointestinal Hemorrhage/prevention & control
12.
Gastroenterol. latinoam ; 27(2): 114-118, 2016. ilus, tab
Article in Spanish | LILACS | ID: biblio-907623

ABSTRACT

Patients with cirrhosis may present with portal hypertension (PHT), which can lead to various complications. The most common areas cites, variceal bleeding, and hepatic encephalopathy. However, there is another entity o flow prevalence but high morbidity and mortality: the hepato pulmonary syndrome (HPS). We report the case of a 25 year-old woman with cirrhosis secondary to autoimmunehepatitis. She was admitted with respiratory symptoms suggestive of viral etiology, but evolved unfavorably. Various diagnoses of cardiovascular and respiratory conditions were successively ruled out, persisting with severe hypoxemia. Concomitantly, she developed progressive pain in the left hypochondrium area, and imaging studies show edsplenic and perisplenic form of manifestation of PHT. In context fHTP and hypoxemia, the diagnosis of HPS vs portopulmonary syndrome was considered, with the bubble test echocardiogramas a key study for such dilemma.


Los pacientes con daño hepático crónico (DHC) pueden cursar con hipertensión portal (HTP), que puede generar diversas complicaciones. Las más frecuentes son la ascitis, hemorragia variceal y encefalopatía hepática. Sin embargo, existe otra entidad de baja prevalencia, pero elevada morbimortalidad: el síndrome hepatopulmonar (SHP). Reportamos el caso de una mujer de 25 años con DHC secundario a hepatitis autoinmune. Ella ingresó con un cuadro sugerente de infección respiratoria alta de etiología viral, pero evolucionó tórpidamente. Se descartaron sucesivamente diversos diagnósticos de la esfera cardiovascular y respiratoria, persistiendo con hipoxemia grave. En forma concomitante desarrolló dolor en hipocondrio izquierdo de carácter progresivo, y las imágenes evidenciaron manifestaciones de HTP de predominio esplénico y periesplénico. En contexto de HTP e hipoxemia se consideró el diagnóstico de síndrome hepatopulmonar vs síndrome porto pulmonar, siendo el ecocardiograma con test de burbujas un estudio clave para el diagnóstico definitivo.


Subject(s)
Female , Humans , Adult , Hypoxia , Hepatopulmonary Syndrome/diagnosis , Hypertension, Portal/complications
13.
Article in English | WPRIM | ID: wpr-188157

ABSTRACT

According to the increasing need for accurate staging of hepatic fibrosis, the ultrasound (US) elastography techniques have evolved significantly over the past two decades. Currently, US elastography is increasingly used in clinical practice. Previously published studies have demonstrated the excellent diagnostic performance of US elastography for the detection and staging of liver fibrosis. Although US elastography may seem easy to perform and interpret, there are many technical and clinical factors which can affect the results of US elastography. Therefore, clinicians who are involved with US elastography should be aware of these factors. The purpose of this article is to present a brief overview of US techniques with the relevant technology, the clinical indications, diagnostic performance, and technical and biological factors which should be considered in order to avoid misinterpretation of US elastography results.


Subject(s)
Disease Progression , Elasticity Imaging Techniques/instrumentation , Fatty Liver/complications , Humans , Hypertension, Portal/complications , Liver/diagnostic imaging , Liver Cirrhosis/diagnostic imaging
14.
Article in English | WPRIM | ID: wpr-44154

ABSTRACT

OBJECTIVE: To evaluate the feasibility, safety, and clinical outcomes of plug-assisted retrograde transvenous obliteration (PARTO) to treat gastric variceal hemorrhage in patients with portal hypertension. MATERIALS AND METHODS: From May 2012 to June 2014, 19 patients (11 men and 8 women, median age; 61, with history of gastric variceal hemorrhage; 17, active bleeding; 2) who underwent PARTO using a vascular plug and a gelfoam pledget were retrospectively analyzed. Clinical and laboratory data were examined to evaluate primary (technical and clinical success, complications) and secondary (worsening of esophageal varix [EV], change in liver function) end points. Median follow-up duration was 11 months, from 6.5 to 18 months. The Wilcoxon signed-rank test was used to compare laboratory data before and after the procedure. RESULTS: Technical success (complete occlusion of the efferent shunt and complete filling of gastric varix [GV] with a gelfoam slurry) was achieved in 18 of 19 (94.7%) patients. The embolic materials could not reach the GV in 1 patient who had endoscopic glue injection before our procedure. The clinical success rate (no recurrence of gastric variceal bleeding) was the same because the technically failed patient showed recurrent bleeding later. Acute complications included fever (n = 2), fever and hypotension (n = 2; one diagnosed adrenal insufficiency), and transient microscopic hematuria (n = 3). Ten patients underwent follow-up endoscopy; all exhibited GV improvement, except 2 without endoscopic change. Five patients exhibited aggravated EV, and 2 of them had a bleeding event. Laboratory findings were significantly improved after PARTO. CONCLUSION: PARTO is technically feasible, safe, and effective for gastric variceal hemorrhage in patients with portal hypertension.


Subject(s)
Aged , Balloon Occlusion , Embolization, Therapeutic , Endoscopy, Digestive System , Esophageal and Gastric Varices/complications , Female , Gastrointestinal Hemorrhage/therapy , Gelatin Sponge, Absorbable/chemistry , Humans , Hypertension, Portal/complications , Male , Middle Aged , Retrospective Studies , Tomography, X-Ray Computed
15.
Rev. paul. pediatr ; 33(2): 246-250, Apr-Jun/2015. graf
Article in English | LILACS | ID: lil-750794

ABSTRACT

OBJECTIVE: To alert the pediatrician who is following up HIV-infected patients about the possibility of non-cirrhotic portal hypertension (NCPH) in this period of life, in order to avoid the catastrophic consequences of this disease as bleeding esophageal varices. CASE DESCRIPTION: A 13 years old HIV-infected patient by vertical route was receiving didanosine (ddI) for 12 years. Although the HIV viral load had been undetectable for 12 years, this patient showed gradual decrease of CD4+ T cells, prolonged thrombocytopenia and high alkaline phosphatase. Physical examination detected splenomegaly, which triggered the investigation that led to the diagnosis of severe liver fibrosis by transient elastography, probably due to hepatic toxicity by prolonged use of ddI. COMMENTS: This is the first case of NCPH in HIV-infected adolescent described in Brazil. Although, the NCPH is a rare disease entity in seropositive patients in the pediatric age group, it should be investigated in patients on long-term ddI or presenting clinical and laboratories indicators of portal hypertension, as splenomegaly, thrombocytopenia and increased alkaline phosphatase.


OBJETIVO: Alertar o pediatra sobre a ocorrência de hipertensão portal não cirrótica (HPNC) na faixa etária pediátrica, no sentido de evitar as consequências catastróficas dessa doença, como o sangramento de varizes de esôfago. DESCRIÇÃO DO CASO: Paciente de 13 anos, infectado pelo HIV por via vertical, recebia esquema antirretroviral com didanosina (ddI) havia 12 anos. Apesar do controle adequado da replicação viral, com carga viral do HIV indetectável havia 12 anos, passou a apresentar diminuição gradativa dos linfócitos TCD4+, trombocitopenia prolongada e fosfatase alcalina elevada. O exame físico detectou esplenomegalia, que desencadeou o processo de investigação e culminou no diagnóstico de fibrose hepática acentuada pela elastografia, por provável toxicidade hepática devido ao uso prolongado de ddI. COMENTÁRIOS: Este é o primeiro caso de HPNC em adolescente infectado pelo HIV descrito no Brasil. Embora seja entidade mórbida rara em pacientes soropositivos para o HIV na faixa etária pediátrica, deve ser investigada nos pacientes em uso prolongado de ddI ou que apresentem indicadores clínicos e/ou laboratoriais de hipertensão portal, como esplenomegalia, trombocitopenia e aumento de fosfatase alcalina.


Subject(s)
Humans , Male , Adolescent , Didanosine/adverse effects , Hypertension, Portal/complications , Liver Cirrhosis , Acquired Immunodeficiency Syndrome/complications
16.
Article in English | WPRIM | ID: wpr-157209

ABSTRACT

Owing to the high prevalence of various chronic liver diseases, cirrhosis is one of the leading causes of morbidity and mortality worldwide. In recent years, the development of non-invasive tests of fibrosis allows accurate diagnosis of cirrhosis and reduces the need for liver biopsy. In this review, we discuss the application of these non-invasive tests beyond the diagnosis of cirrhosis. In particular, their role in the selection of patients for hepatocellular carcinoma surveillance and varices screening is highlighted.


Subject(s)
Biomarkers/blood , Carcinoma, Hepatocellular/diagnosis , Elasticity Imaging Techniques , Endoscopy, Digestive System , Humans , Hypertension, Portal/complications , Liver Cirrhosis/complications , Liver Neoplasms/diagnosis , Risk Factors
17.
Rev. gastroenterol. Perú ; 34(1): 23-28, ene. 2014. ilus, tab
Article in Spanish | LILACS, LIPECS | ID: lil-717354

ABSTRACT

Objetivo: Evaluar la exactitud diagnóstica de la gradiente albúmina sangre/ascitis (GASA), proteínas totales en líquido ascítico (PTLA), albúmina en líquido ascítico (CAA) e índice de proteínas ascitis/suero (IPAS) para el diagnóstico de ascitis por hipertensión portal. Materiales y métodos: Se realizó un estudio, observacional, retrospectivo, de validez de pruebas diagnósticas. La población estudiada fueron pacientes mayores de 15 años con diagnóstico de ascitis a los cuales se les tomó una muestra para estudio del líquido ascítico mediante la técnica estándar de paracentesis, analizando proteínas totales y albúmina, además de estudio de proteínas totales y albúmina en sangre en el Hospital de Salud Pública Nacional Daniel Alcides Carrión del Callao, Perú (HNDCA), durante el periodo de enero a diciembre del 2012. Se obtuvo la exactitud diagnóstica, sensibilidad, especificidad, VPP y VPN de la gradiente albumina sangre/ascitis (GASA), proteínas totales en líquido ascítico (PTLA), albúmina en líquido ascítico (CAA) e índice de proteínas ascitis/suero (IPAS) para el diagnóstico de ascitis por hipertensión portal o no HTP. Para determinar ascitis por HTP según las pruebas diagnósticas se tomo en cuentas: GASA≥1,1, PTLA<2,5, CAA<1,1 o IPAS<0,5. Resultados: se obtuvieron 126 pacientes con diagnóstico de ascitis a los cuales se excluyó 10 pacientes por tener datos incompletos. De los 116 pacientes finales la edad promedio fue de 53,03 ± 15,73 años, pacientes de sexo masculino fueron 65 (56%) y femenino 51 (44%). Se encontró 61 (52%) líquidos ascíticos debido a HTP por cirrosis hepática, y 55 (48%) de ascitis por NO HTP. La sensibilidad y especificidad para el GASA fue de 93% y 47% respectivamente, para PTLA fue de 80% y 89% respectivamente, para CAA fue de 85% y 87% respectivamente y para el IPAS fue de 83% y 80% respectivamente. El área bajo la curva ROC para el GASA fue de 0,70, de las PTLA fue de 0,84, del IPAS fue de 0,81 y de la CAA fue de 0,86; encontrándose diferencia estadísticamente significativa entre el GASA comparado con los otros tres parámetros (p<0,01). Conclusión: La exactitud diagnóstica de la CAA, PTLA y IPAS es superior a la del GASA para discriminar entre ascitis por HTP o NO HTP, por lo que podrían ser usados en la práctica clínica de forma aislada, o en conjunto para lograr una aproximación diagnóstica más acertada.


Objective: To evaluate the diagnostic accuracy of the Serum-Ascites Albumin Gradient (GASA), Protein Concentration in the Ascitic Fluid (PTLA), Albumin Concentration in the ascitic fluid (CAA) and the Protein Ascites/Serum Ratio (IPAS) for the diagnosis of ascites due to portal hypertension. Materials and methods: it was an observational and retrospective study of validation of diagnostic tests. The study population was patients from a National Public Health Hospital Daniel Alcides Carrion of Callao, Peru, during the period January to December of 2012, patients over 15 years old with a diagnosis of ascites which samples were taken for study by paracentesis with an standard technique, it was analyzed total protein and albumin, as well as study of total protein and albumin in blood. We obtained the diagnostic accuracy, sensitivity, specificity, PPV and NPV of the Serum-Ascites Albumin Gradient (GASA), Protein Concentration in the Ascitic Fluid (PTLA), Albumin Concentration in the ascitic fluid (CAA) and the Protein Ascites/Serum Ratio (IPAS) for the diagnosis of ascites due to portal hypertension. To determine ascites by HTP as diagnostic tests we took into account: GASA ≥ 1.1, PTLA <2.5, CAA <1.1 or IPAS< 0.5. Results: There were 126 patients diagnosed with ascites, 10 patients was excluded for having incomplete data. Of the 116 patients, the average age was 53.03 +/- 15.73 years old, male 65 (56%) and female 51 (44%). 61 (52%) had ascites due to portal hypertension from liver cirrhosis, and 55 (48%) of ascites due to NO HTP. The sensitivity and specificity for GASA was 93% and 47% respectively, for PTLA was 80% and 89% respectively, for CAA was 85% and 87% respectively and for the IPAS was 83% and 80% respectively. The area under the ROC curve for GASA was 0.70, ATPL was 0.84, IPAS was 0.81 and CAA was 0.86, we found statistically significant differences between GASA compared to the other three parameters (p<0.01 ). Conclusion: The diagnostic accuracy of CAA, ATPL and IPAS is higher than the GASA to discriminate between ascites due to HTP or NO HTP, so that they could be used in clinical practice alone or together to achieve a diagnostic approach more successful.


Subject(s)
Female , Humans , Male , Middle Aged , Albumins/analysis , Ascites/diagnosis , Ascites/etiology , Ascitic Fluid/chemistry , Hypertension, Portal/complications , Serum Albumin/analysis , Ascites/metabolism , Hypertension, Portal/metabolism , Predictive Value of Tests , Proteins/analysis , Retrospective Studies , Sensitivity and Specificity
18.
Article in English | WPRIM | ID: wpr-112007

ABSTRACT

Tetrahydrobiopterin (BH4) is an essential cofactor in NO synthesis by endothelial nitric oxide synthase (eNOS) enzymes. It has been previously suggested that reduced intrahepatic BH4 results in a decrease in intrahepatic NO and contributes to increased hepatic vascular resistance and portal pressure in animal models of cirrhosis. The main aim of the present study was to evaluate the relationship between BH4 and portal hypertension (PHT). One hundred ninety-three consecutive patients with chronic liver disease were included in the study. Liver biopsy, measurement of BH4 and hepatic venous pressure gradient (HVPG) were performed. Hepatic fibrosis was classified using the Laennec fibrosis scoring system. BH4 levels were determined in homogenized liver tissues of patients using a high performance liquid chromatography (HPLC) system. Statistical analysis was performed to evaluate the relationship between BH4 and HVPG, grade of hepatic fibrosis, clinical stage of cirrhosis, Child-Pugh class. A positive relationship between HVPG and hepatic fibrosis grade, clinical stage of cirrhosis and Child-Pugh class was observed. However, the BH4 level showed no significant correlation with HVPG or clinical features of cirrhosis. BH4 concentration in liver tissue has little relation to the severity of portal hypertension in patients with chronic liver disease.


Subject(s)
Adult , Aged , Biopterin/analogs & derivatives , Chromatography, High Pressure Liquid , Chronic Disease , Elasticity Imaging Techniques , Female , Hepatic Veins/physiology , Humans , Hypertension, Portal/complications , Liver/pathology , Liver Cirrhosis/diagnostic imaging , Liver Diseases/complications , Male , Middle Aged , Nitric Oxide/metabolism , Portal Pressure , Regression Analysis , Severity of Illness Index
19.
Article in English | WPRIM | ID: wpr-85681

ABSTRACT

BACKGROUND/AIMS: Angiotensin receptor blockers (ARBs) inhibit activated hepatic stellate cell contraction and are thought to reduce the dynamic portion of intrahepatic resistance. This study compared the effects of combined treatment using the ARB candesartan and propranolol versus propranolol monotherapy on portal pressure in patients with cirrhosis in a prospective, randomized controlled trial. METHODS: Between January 2008 and July 2009, 53 cirrhotic patients with clinically significant portal hypertension were randomized to receive either candesartan and propranolol combination therapy (26 patients) or propranolol monotherapy (27 patients). Before and 3 months after the administration of the planned medication, the hepatic venous pressure gradient (HVPG) was assessed in both groups. The dose of propranolol was subsequently increased from 20 mg bid until the target heart rate was reached, and the candesartan dose was fixed at 8 mg qd. The primary endpoint was the HVPG response rate; patients with an HVPG reduction of >20% of the baseline value or to <12 mmHg were defined as responders. RESULTS: The mean portal pressure declined significantly in both groups, from 16 mmHg (range, 12-28 mmHg) to 13.5 mmHg (range, 6-20 mmHg) in the combination group (P<0.05), and from 17 mmHg (range, 12-27 mmHg) to 14 mmHg (range, 7-25 mmHg) in the propranolol monotherapy group (P<0.05). However, the medication-induced pressure reduction did not differ significantly between the two groups [3.5 mmHg (range, -3-11 mmHg) vs. 3 mmHg (range, -8-10 mmHg), P=0.674]. The response rate (55.6% vs. 61.5%, P=0.435) and the reductions in mean blood pressure or heart rate also did not differ significantly between the combination and monotherapy groups. CONCLUSIONS: The addition of candesartan (an ARB) to propranolol confers no benefit relative to classical propranolol monotherapy for the treatment of portal hypertension, and is thus not recommended.


Subject(s)
Adolescent , Adult , Aged , Antihypertensive Agents/therapeutic use , Benzimidazoles/therapeutic use , Blood Pressure , Drug Therapy, Combination , Female , Humans , Hypertension, Portal/complications , Liver Cirrhosis/complications , Male , Middle Aged , Propranolol/therapeutic use , Prospective Studies , Tetrazoles/therapeutic use , Treatment Outcome , Young Adult
20.
Article in English | WPRIM | ID: wpr-18382

ABSTRACT

A major cause of cirrhosis related morbidity and mortality is the development of variceal bleeding, a direct consequence of portal hypertension. Less common causes of gastrointestinal bleeding are peptic ulcers, malignancy, angiodysplasia, etc. Upper gastrointestinal bleeding has been classified according to the presence of a variceal or non-variceal bleeding. Although non-variceal gastrointestinal bleeding is not common in cirrhotic patients, gastroduodenal ulcers may develop as often as non-cirrhotic patients. Ulcers in cirrhotic patients may be more severe and less frequently associated with chronic intake of non-steroidal anti-inflammatory drugs, and may require more frequently endoscopic treatment. Portal hypertensive gastropathy (PHG) refers to changes in the mucosa of the stomach in patients with portal hypertension. Patients with portal hypertension may experience bleeding from the stomach, and pharmacologic or radiologic interventional procedure may be useful in preventing re-bleeding from PHG. Gastric antral vascular ectasia (GAVE) seems to be different disease entity from PHG, and endoscopic ablation can be the first-line treatment.


Subject(s)
Gastric Antral Vascular Ectasia/complications , Gastric Mucosa/pathology , Gastrointestinal Hemorrhage/etiology , Humans , Hypertension, Portal/complications , Liver Cirrhosis/complications , Peptic Ulcer/complications
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