Recent Research Advance to Differentiate Portal Hypertension Associated with Primary Myelofibrosis and Cirrhosis --Review / 中国实验血液学杂志

Primary myelofibrosis (PMF) is easily confused with cirrhosis, due to its main clinical manifestations of splenomegaly and the blood cytopenia. This review focuses on clinical studies to identify primary myelofibrosis and cirrhosis related portal hypertension, to analyze the differences between the two diseases, in order to distinguish PMF and cirrhosis from the pathogenesis, clinical manifestations, laboratory examinations and treatment principles, and simultaneously improve clinicians' understanding of PMF, which is a reference for exploring the early screening or diagnostic indicators of PMF, also provides a clinical basis for the application of new targeted drugs such as ruxolitinib.

Study on the comparative analysis of the efficacy of transmesenteric vein extrahepatic portosystemic shunt and transjugular intrahepatic portosystemic shunt in the treatment of cavernous transformation of portal vein / 中华肝脏病杂志

Objective: To compare the safety and efficacy of transmesenteric vein extrahepatic portosystemic shunt (TEPS) and transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of cavernous transformation of the portal vein (CTPV). Methods: The clinical data of CTPV patients with patency or partial patency of the superior mesenteric vein treated with TIPS or TEPS treatment in the Department of Vascular Surgery of Henan Provincial People's Hospital from January 2019 to December 2021 were selected. The differences in baseline data, surgical success rate, complication rate, incidence rate of hepatic encephalopathy, and other related indicators between TIPS and TEPS group were statistically analyzed by independent sample t-test, Mann-Whitney U test, and Chi-square test. Kaplan-Meier survival curve was used to calculate the cumulative patency rate of the shunt and the recurrence rate of postoperative portal hypertension symptoms in both groups. Results: The surgical success rate (100% vs. 65.52%), surgical complication rate (6.67% vs. 36.84%), cumulative shunt patency rate (100% vs. 70.70%), and cumulative symptom recurrence rate (0% vs. 25.71%) of the TEPS group and TIPS group were statistically significantly different (P < 0.05). The time of establishing the shunt [28 (2141) min vs. 82 (51206) min], the number of stents used [1 (12) vs. 2 (15)], and the length of the shunt [10 (912) cm vs. 16 (1220) cm] were statistically significant between the two groups (t = -3.764, -4.059, -1.765, P < 0.05). The incidence of postoperative hepatic encephalopathy in the TEPS group and TIPS group was 6.67% and 15.79% respectively, with no statistically significant difference (Fisher's exact probability method, P = 0.613). The pressure of superior mesenteric vein decreased from (29.33 ± 1.99) mmHg to (14.60 ± 2.80) mmHg in the TEPS group and from (29.68 ± 2.31) mmHg to (15.79 ± 3.01) mmHg in TIPS group after surgery, and the difference was statistically significant (t = 16.625, 15.959, P < 0.01). Conclusion: The best indication of TEPS is in CTPV patients with patency or partial patency of the superior mesenteric vein. TEPS improves the accuracy and success rate of surgery and reduces the incidence of complications.

Remisión a largo plazo de la hipertensión portopulmonar mediante trasplante hepático en paciente con cirrosis asociada a hepatitis autoinmune / Long term remission of portopulmonary hypertension with liver transplantation in a patient with cirrhosis associated to autoimmune hepatitis

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.

Effects of primary preventive treatment under endoscope for esophageal and gastric varices on bleeding rate and its relevant factors / 中华肝脏病杂志

Objective: To investigate the effects of primary preventive treatment under endoscope for esophageal and gastric varices on bleeding rate and its relevant factors. Methods: 127 cases with liver cirrhosis accompanied with esophageal and gastric varices without bleeding history were included in the endoscopic and non-endoscopic treatment group, respectively. Informed consent was obtained from both groups. Gastric varices (Lgf) and esophageal varices (Leg) were diagnosed according to LDRf classification criteria, and the corresponding treatment scheme was selected according to the recommended principle of this method.The incidence rate of bleeding from ruptured esophageal varices were observed at 3, 6 months, and 1, and 2 years in the treated and the untreated group, and the patients with different Child-Pugh scores were followed-up for 2 years. Gender, age, etiology, varicose degree, Child-Pugh grade, platelet count, prothrombin activity, portal vein thrombosis, collateral circulation, portal vein width and other factors affecting the bleeding rate were assessed. Measurement data were described as mean ± standard deviation (x¯±s), and qualitative data of categorical variables were expressed as percentage (%), and χ2 test was used. Results: 127 cases were followed up for 2 years. There were 55 cases in the endoscopic treatment group (18 cases underwent band ligation, 2 cases underwent band ligation combined with tissue adhesive embolization, 28 cases underwent sclerotherapy, and 7 cases underwent sclerotherapy combined with tissue adhesive embolization). Recurrent bleeding and hemorrhage was occurred in 5 (9.1%) and 28 cases (38.9%), respectively (P<0.05). In addition, there were 72 cases in the untreated group (P<0.05). Severe varicose veins proportions in treated and untreated group were 91.1% and 85.1%, respectively (P>0.05). There was no statistically significant difference in liver cirrhosis-related medication and β-blocker therapy between the treated and untreated group (P>0.05). There was no statistically significant difference in the bleeding rate between the different treated groups (P>0.05). The bleeding rates at 3, 6 months, 1, and 2 years in endoscopic treated and untreated group were 2.00% vs. 2.59% (P>0.05), 2.30% vs. 5.88% (P>0.05), 3.10% vs. 7.55% (P>0.05) and 4.00% vs. 21.62% (P<0.05), respectively. All patients with Child-Pugh grade A, B and C in the treated and the untreated group were followed-up for 2 years, and the bleeding rates were 1.8% vs. 8.1% (P<0.05), 1.1% vs. 9.4% (P<0.05) and 9.1% vs. 10.1% (P>0.05), respectively. There were statistically significant differences in the rupture and bleeding of esophageal and gastric varices, varices degree, Child-Pugh grade and presence or absence of thrombosis formation in portal vein (P<0.05); however, no statistically significant differences in gender, age, etiology, platelet count, prothrombin activity, collateral circulation and portal vein width (P>0.05). There was no intraoperative bleeding and postoperative related serious complications in the treated group. Conclusion: The risk of initial episodes of bleeding from esophageal and gastric varices is significantly correlated with the varices degree, Child-Pugh grade, and portal vein thrombosis. Primary preventive treatment under endoscope is safe and effective for reducing the long-term variceal bleeding risk from esophageal and gastric varices.

Physiopathological and diagnostic aspects of cirrhotic cardiomyopathy / Aspectos fisiopatológicos y diagnósticos de la miocardiopatía cirrótica

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.

Octreótido de liberación prolongada para el tratamiento de hemorragia digestiva alta en Pediatría / Long-acting Release Octreotide for Pediatric Upper Gastrointestinal Bleeding

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.

Carvedilol as primary prophylaxis for gastric variceal bleeding in portal hypertension model in rats / Carvedilol como profilaxia de sangramento em varizes gástricas em modelo de hipertensão portal em ratos

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.

Hidrotórax hepático: presentación de 3 casos con diferentes enfoques terapéuticos / Hepatic hydrothorax: presentation of 3 cases with different therapeutic approaches

El hidrotórax hepático (HH) se define como un derrame pleural mayor de 500 ml en pacientes con cirrosis e hipertensión portal. Representa una complicación infrecuente por lo general asociada con ascitis y su origen se relaciona con el paso de líquido ascítico a través de pequeños defectos en el diafragma de predominio en el hemitórax derecho. Una vez establecido el diagnóstico por imágenes, la toracentesis diagnostica permite confirmar un trasudado. La terapia inicial está basada en la restricción de sodio y el uso combinado de diuréticos. El 20-25% de los pacientes desarrolla un HH refractario, el cual requiere intervenciones invasivas tales como la derivación percutánea portosistémica intrahepática (DPPI), la reparación de los defectos diafragmáticos por videotoracoscopia asistida asociada a pleurodésis química y el uso de un catéter pleural tunelizado. No se recomienda la inserción de un tubo de tórax por su elevada morbilidad y mortalidad. El tratamiento definitivo del HH es el trasplante hepático el cual alcanza una excelente sobrevida. Presentamos tres casos de hidrotórax hepático con diferentes enfoques terapéuticos que incluyeron el manejo conservador con dieta y diuréticos, la inserción fallida de un tubo de tórax con pleurodesis y una DPPI.

Hepatic hydrothorax is uncommon transudative pleural effusion greater than 500 ml in association with cirrhosis and portal hypertension. Ascites is also present in most of the patients and the pathophysiology include the passage of ascites fluid through small diaphragmatic defects. After diagnostic thoracentesis studies, the first line management is restricting sodium intake and diuretics combination including stepwise dose of spironolactone plus furosemide. Therapeutic thoracentesis is a simple and effective procedure to relief dyspnea. Hepatic hydrothorax is refractory in approximately 20-25% and treatments options include repeated thoracentesis, transjugular intrahepatic portosystemic shunts (TIPS) placement, chemical pleurodesis with repair diaphragmatic defects using video-assisted thoracoscopy surgery (VATS), and insertion of an indwelling pleural catheter. Chest tube insertion carries significant morbidity and mortality with questionable benefit. Hepatic transplantation remains the best treatment option with long term survival. We present three cases of hepatic hydrothorax with different therapeutic approach including first line management, failed chest tube insertion and TIPS placement.

Fibrose hepática congênita e venopatia portal obliterativa sem hipertensão portal - revisão da literatura com base em um caso assintomático / Congenital hepatic fibrosis and obliterative portal venopathy without portal hypertension - a review of literature based on an asymptomatic case

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.

Cavernoma portal complicada con biliopatía secundario a enfermedad de Gaucher tipo 1: reporte de un caso peruano / Cavernoma complicated with biliopatia secondary to type 1 Gaucher disease: report of a Peruvian case

La enfermedad de Gaucher, es un trastorno autosómico recesivo de depósito lisosomal que se caracteriza por deficiencia de la beta-glucocerebrosidasa que lleva a la acumulación de glucosilceramida principalmente en células del sistema fagocítico mononuclear causando afectaciones sistémicas. Se presenta paciente varón de 20 años que cursa con dolor crónico en hipocondrio izquierdo con episodios de sangrados desde hace 3 años y sensación de alza térmica, al examen físico se identificó ictericia y esplenomegalia masiva, sin afectación neurológica. Como apoyo al diagnóstico se mostró osteoporosis severa, pancitopenia y como hallazgo inesperado la presencia de trombosis de vena porta con transformación cavernomatosa complicada con biliopatía portal simulando un tumor de klatskin, los estudios de médula y enzimáticos eran compatibles con enfermedad de Gaucher, por lo cual recibió tratamiento con imiglucerasa realizando seguimiento. Es un caso poco frecuente, de gran interés, heterogeneidad en sus manifestaciones clínicas e inéditas por su complicación, constituyendo un desafío llegar a su diagnóstico de esta enfermedad huérfana.

Gaucher disease is an autosomal recessive lysosomal storage disorder characterized by deficiency of beta-glucosidase that would lead to the accumulation of glucosylceramide mainly in cells of the mononuclear phagocytic system causing systemic effectations. We present a patient of twenty years who is suffering from chronic pain in the left hypochondrium with episodes of bleeding for 3 years and sensation of thermal rise, physical examination revealed jaundice and massive splenomegaly, without neurological involvement. Severe osteoporosis, pancytopenia, and the presence of portal vein thrombosis with cavernomatous transformation complicated by portal biliopathy simulating a klatskin tumor, marrow and enzymatic studies were compatible with Gaucher disease, were shown as unexpected findings. he received treatment with imiglucerase, following up. It is a rare case, of great interest, heterogeneity in its clinical manifestations and unpublished by its complication, constituting a challenge to reach its diagnosis of this orphan disease.

Hipertensión portopulmonar: Revisión actualizada / Portopulmonary hypertension: Updated review

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/).

Colangiopatía portal: complicación poco frecuente de la hipertensión portal / Portal cholangiopathy: infrequent portal hypertension complication / Colangiopatia do portal: complicação incomum da hipertensão portal

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).

Colocación de un dispositivo de derivación porto sistémica en el tratamiento del paciente con hipertensión portal / Transjugular intrahepatic portosystemic shunt in the management of portal hypertension

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)

Evaluation of endoscopic secondary prophylaxis in children and adolescents with esophageal varices / Avaliação da profilaxia secundária endoscópica em crianças e adolescentes com varizes de esôfago

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.

Características clínicas y laboratoriales de pacientes hospitalizados con hipertensión portal y complicaciones vasculares pulmonares en el Instituto Nacional de Salud del Niño / Clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño

El síndrome hepatopulmonar (SHP) y la hipertensión portopulmonar (HTPP) son distintas complicaciones vasculares pulmonares de la hipertensión portal (HTP) y se asocian con una mayor morbilidad y mortalidad. Objetivos: Describir las Características Clínicas y Laboratoriales de los pacientes con HTP y complicaciones vasculares pulmonares hospitalizados en el Instituto Nacional de Salud del Niño. Materiales y métodos: se incluyeron los pacientes con HTP hospitalizados desde enero del 2012 hasta junio del 2013 y que durante su evolución cursaron con SHP o HTPP. Para el análisis se les dividió en un primer grupo de pacientes con cirrosis hepática y un segundo grupo con obstrucción extra hepática de vena porta. Resultados: De 22 pacientes con HTP el 45,5% fueron varones y el rango de edad fue entre 1 mes y 17 años. La etiología en el grupo de cirrosis (n=14) fue: hepatitis autoinmune (35,7%), cirrosis criptogénica (35,7%), error innato del metabolismo (14,3%), hepatitis viral crónica por virus C (7,15%) y atresia de vías biliares extra hepática (7,15%). Las complicaciones vasculares pulmonares, se presentaron más frecuentemente en los pacientes con cirrosis hepática (1 caso de síndrome hepatopulmonar y un caso de hipertensión portopulmonar). En ellos se encontró más frecuentemente disnea, astenia, edema, desnutrición, ascitis, hiperesplenismo y hemorragia digestiva por várices esofágicas, además de valores elevados de ALT, fosfatasa alcalina y menores niveles de albúmina sérica. Conclusiones: En niños con HTP, las complicaciones vasculares pulmonares son muy infrecuentes. En la evaluación de estos pacientes debería incluirse la oximetría de pulso para detectar hipoxemia y posteriormente, de ser necesario una ecocardiografía Doppler y de contraste. Ante el hallazgo de hipertensión sistólica pulmonar es necesario realizar un cateterismo cardiaco derecho.

The hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHN) are distinct pulmonary vascular complications of portal hypertension (PHT) and are associated with increased morbidity and mortality. Objectives: To describe the clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño. Materials and methods: We included patients with HTP hospitalized from January 2012 to June 2013 and that during its evolution progressed with SHP or HTPP. For analysis, they were divided into a first group of patients with liver cirrhosis and a second group with extrahepatic portal vein obstruction. Results: Of 22 patients with HPT 45.5% were male and the age range was between 1 month and 17 years. The etiology in the group of cirrhosis (n=14) was: autoimmune hepatitis (35.7%), cryptogenic cirrhosis (35.7%), inborn error of metabolism (14.3%), chronic viral hepatitis C (7.15%) virus and atresia extra-hepatic bile ducts (7.15%). Pulmonary vascular complications more frequently occurred in patients with liver cirrhosis (1 case of HPS and a case of PPHTN). They most often dyspnea, asthenia, edema, malnutrition, ascites, hypersplenism and gastrointestinal bleeding from esophageal varices was found. Also, they had elevated ALT values, alkaline phosphatase and serum albumin values decreased. Conclusions: In children with pulmonary hypertension, pulmonary vascular complications are rare. In the evaluation of these patients pulse oximetry should be included to detect hypoxemia and subsequently a Doppler echocardiography and contrast echocardiography necessary. Dueto the finding of systolic pulmonary hypertension it is necessary to perform right heart catheterization.

Caput Medusae due to portal hypertension in schistosomiasis mansoni / Cabeza de Medusa debida a la hipertensión portal en esquistosomiasis mansoni

A 62-year-old Brazilian man who lived in endemic areas of tropical diseases had an episode of hematemesis associated with portal hypertension. He used to swim in natural ponds during childhood and developed the hepatosplenic form of schistossomiasis with moderate ascites, in addition to the characteristic features of abdominal Caput Medusae. The aim of the report is highlight the role of chronic liver disease and schistossomiasis

Un hombre natural de Brasil de 62 años de edad que vivía en zonas endémicas de enfermedades tropicales presentó un episodio de hematemesis asociada con hipertensión portal. Frecuentemente se bañaba en los estanques naturales durante la infancia y desarrolló la forma hepatosplénica de la esquistosomiasis con ascitis moderada, además de los rasgos abdominales característicos de la Cabeza de Medusa. El objetivo del informe es poner de relieve el papel de la enfermedad hepática crónica y de la esquistosomiasis

Enfrentamiento de pacientes pediátricos con várices esofágicas / Management of pediatric patients with esophageal varices

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.

Síndrome hepatopulmonar: poco común, pero mortal / Hepatopulmonary syndrome: uncommon, but deadly

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.

Plug-Assisted Retrograde Transvenous Obliteration for the Treatment of Gastric Variceal Hemorrhage

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.